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A 74-year-old man was admitted to the cardiology department of our institution with a history of chest pain and dyspnea on exertion. His functional capacity was class II according to the New York Heart Association (NYHA) classification. He had a history of stent implantation in the proximal left anterior descending coronary artery (LAD) two years ago, in another facility. Physical examination was unremarkable except for the hypotension (90/50 mmHg) and bradycardia (57 beats per minute). A chest X-ray revealed moderate cardiomegaly. The patient's complete blood count, biochemical analyses, and the coagulation tests were normal. An electrocardiogram (ECG) showed sinus rhythm and nonspecific ST-T segment changes. Transthoracic echocardiography images revealed hyperdynamic left ventricular systolic function, grade I diastolic dysfunction, left ventricular regional wall motion abnormality (mild anterior wall hypokinesia), and mild tricuspid and mitral regurgitation. In order to investigate the etiology of the chest pain, dobutamine stress echocardiography was performed, revealing worsening hypokinesia of the anterior wall. Due to positive dobutamine stress echocardiography and recurring angina, coronary angiography was performed. It revealed nonsignificant atherosclerotic plaques in the circumflex artery and the right coronary artery, a patent stent in the proximal LAD, and myocardial bridges causing severe compression and near complete occlusion of the middle and distal LAD during systole, with pulsatile contrast hanging or a “milking effect.” Despite medical therapy with acetylsalicylic acid, a β-blocker, and a calcium antagonist, the patient continued to experience dyspnea and chest pain during exercise; therefore PCI for the treatment of the bridged segments in the LAD was planned. After administration of a 600 mg loading dose of clopidogrel and an intravenous bolus of 10.000 units heparin, left main coronary artery was cannulated with a 7F JL4 guiding catheter and a 0.014′′ floppy guide wire was advanced. The middle segment of the LAD which was almost totally compressed by the myocardial bridge was stented using a 2.75 × 18 mm drug eluting stent (DES) deployed at 16 atmospheres. There was no residual stenosis, dissection, or evidence of compression by the myocardial bridge at the end of this procedure. During stenting of the bridged segment in the distal portion of the LAD with a 2.75 × 14 mm DES at 18 atmospheres, CP occurred and angiographic images showed massive and pulsatile extravasation from the LAD into the pericardial space (). An immediate drop in blood pressure and heart rate was noted, and the patient complained of severe chest pain. Bigeminal ventricular premature complexes and slight ST segment depression in the anterior precordial leads were noted on the ECG. A stent-graft 19 mm in length connected with a 3 mm balloon was implanted immediately in the site of the rupture, with subsequent complete restitution of blood flow in the LAD and termination of extravasation into the pericardial space. The chest pain abated and the ECG was normalized. Protamine sulfate (50 mg) was administered intravenously to reverse the effect of heparin after intervention. Ibuprofen (800 mg 1 × 2 p.o.) was prescribed as an analgesic and anti-inflammatory medication. Transthoracic echocardiography in the catheter laboratory and one day after the PCI procedure showed minimal intrapericardial fluid but no progression was observed. The patient was followed up for 2 days in the coronary care unit and was discharged from the hospital 1 week later. At the follow-up visits at 30 days and 2 months, the patient's NYHA class was improved and chest pain was alleviated. The patient is currently on follow-up with medical treatment for 4 months and long-term dual antiplatelet therapy for at least 1 year has been planned.
We present a 50-year-old female with cirrhosis secondary to chronic hepatitis C and alcohol abuse in remission who was admitted to our hospital with a three-day history of worsening abdominal pain and confusion. She was prescribed lactulose for the hepatic encephalopathy, which kept her ammonia in the normal range but had missed several doses over the preceding three days in addition to a decrease in the number of bowel movements. On initial exam the patient was normotensive and oxygenating well on room air with mild encephalopathy. A chronic ventral hernia displayed no signs of peritonitis. Labs on admission were significant for an ammonia level of 87 μmol/L (18–72 μmol/L), platelet level of 96 k/μL, sodium of 132 mmol/L, and international normalized ratio (INR) of 1.6. Computed tomography of her abdomen and pelvis with intravenous contrast revealed that the hernia contained dilated small bowel but no definitive obstruction.\nOvernight her mental status declined and by the next morning she was difficult to arouse and unable to follow commands. Vitals were within normal limits and morning labs were significant for a rise in plasma ammonia to 277 μmol/L. She was electively intubated for airway protection but was not hypoxic. The following morning, on hospital day 3, she developed rhythmic clonic facial movements with eyelid fluttering, sustained tonic stiffening of the extremities, and intermittent right upper extremity jerks. The neurology service was consulted and continuous electroencephalogram (EEG) was recommended. During two hours of recording, prior to MRI imaging, there was focal status epilepticus in the left temporal region that spread to the left central and right temporal regions. 5 mg of intravenous lorazepam was administered and 1 gm levetiracetam twice daily was started, but the EEG continued to show persistent SE. She was paralyzed with vecuronium to obtain MRI of the brain with gadolinium contrast. This revealed extensive CDR involving the bilateral supratentorial cortex, with relative sparing of the occipital lobes, thalami, and deep gray matter. There was a corresponding decrease in apparent diffusion coefficient values, consistent with cytotoxic edema (). Continuous neuromuscular blockade with intravenous cisatracurium was required to control tachypnea and burst suppression on EEG was achieved with high-dose propofol infusion.\nOver the next two days, on hospital days 4 and 5, the patient developed febrile sepsis but was not significantly hypotensive or hypoxic. On hospital day 6, despite the cessation of cisatracurium and propofol, she remained comatose with sluggishly reactive pupils and a weak gag response. EEG showed prolonged generalized suppression alternating with brief periods of low amplitude slow wave activity, ultimately progressing to diffuse attenuation. On hospital day 7, her exam remained unchanged and a repeat MRI brain demonstrated cerebral edema with bilateral uncal herniation and the extensive CDR seen on the first MRI, but there was spread of the CDR to the occipital cortices, the bilateral thalami, and basal ganglia. The prior areas of CDR were now bright on T2 and ADC, consistent with an evolution to extracellular cerebral edema, while the new areas of CDR were dark on ADC, suggesting a more recent insult still in the phase of cytotoxic edema (). After discussion of the patient's prognosis with her husband, supportive care was withdrawn and the patient died shortly thereafter.
A 58-year old male patient reported to the Department of Oral Medicine and Radiology of PMS College of Dental Science and Research (Kerala, India) with a slow growing painless swelling on the palate that had been present for almost 4 years. The patient was a well-controlled diabetic with no other medical problems. Extra oral examination revealed no abnormalities. On intraoral examination, a smooth surfaced sessile swelling was seen at the junction of hard and soft palate, 2 cm from the midline and 1 cm away from the crest of the edentulous alveolar ridge near to the left maxillary tuberosity region. The lesion was slightly yellowish, well circumscribed, and non-tender with a soft consistency. It measured approximately 1×1.5 cm. There was no evidence of superficial vascularity as the lesion did not blanch on palpation. The swelling appeared to slip under the mucosal surface on palpation (). Radiographic evaluation did not reveal any bone involvement. The patient was largely edentulous except for the three standing teeth (upper right maxillary central and lateral incisors and maxillary right upper canine). Routine hematological and urine analysis and chest X-ray was normal. ELISA for HIV was non reactive.\nThe clinical differential diagnosis of a slow growing, soft, non tender, non ulcerated smooth surfaced sessile mass at the junction of hard and soft palate includes a list of lesions comprising of commonly occurring palatal abscess, cysts both odontogenic and non-odontogenic; soft tissue neoplasms like fibroma, lipoma, neurofibroma, schwannnoma, leiomyoma and also minor salivary gland neoplasms. Palatal abscess was eliminated from the list due to lack of infectious foci. Reactive lesions like pyogenic granuloma was not considered in the differential diagnosis as there was no recognizable irritant like dentures, dental plaque etc. Soft and slippery consistency of the lesion helped in eliminating hard tissue tumors; hard tissue and vascular malformations; and lesions like fibroma, irritation fibroma and ossifying fibroma. While preparing the diagnostic list, several soft tissue tumors of connective tissue (muscle, neural, adipocytic), and salivary gland origin had to be considered, as they have similar clinical features especially when occurring in the palate. The lesion being a slow growing one, high-grade malignancies were not considered in the differential diagnosis. Benign muscle tumor like leiomyoma was considered in the differential diagnosis taking into account the age of the patient and the location, but granular cell myoblastoma was not considered as it was more common in the tongue than in the palate. Benign nerve tissue tumors like neurofibroma and neurilemmoma had several features similar to this case. Out of the two, neurofibroma was as a more probable option considering its relatively higher frequency on the palate. The color, the size and the feel of the lesion also suggested the possibility of lipoma, in spite of the fact that classical lipomas occur rarely on the palate. Some literature describes sialolipoma, a relatively rare lesion with more incidences in the palate than classical lipoma, to be having certain clinical features similar to this case. Considering the frequency of occurrence in the palate and the softness of the lesion, benign salivary gland tumors had to be strongly considered probably before any of the muscle or nerve tissue tumors in the panel of diagnostic hypothesis. Of the benign salivary gland tumors occurring in the palate, pleomorphic adenoma was the most probable and other tumors like basal cell adenoma, myoepithelioma, and cyst adenoma further down in the differential diagnosis list. The long duration (4 years), the lesion took to grow up to the present size helped in ruling out the possibility of malignant salivary gland neoplasms, however slow growing polymorphous low grade adenocarcinoma and low grade mucoepidermoid carcinoma could be considered. Since palatal involvement of lesions like non-Hodgkin lymphoma is usually associated with human immunodeficiency virus infection, it was not considered as the patient was relatively healthy and free from HIV infection. Even though rare in the palate, possibility of fibrous histiocytoma was kept in mind as the lesion presented as a painless submucosal nodule in an elderly adult.\nThus the clinical differential diagnosis list was formulated considering the clinical features of the patient’s tumor, prevalence of previously described lesions, and demographic data. The list of lesions suggested had salivary gland tumors like pleomorphic adenoma, basal cell adenoma, myoepithelioma, cyst adenoma, low grade mucoepidermoid carcinoma, polymorphous low grade adenocarcinoma occupying the top slots in the list followed by other lesions like lipoma, neurofibroma, neurilemmoma, leiomyoma and benign fibrous histiocytoma. However, the diagnosis of any soft tissue tumor can only be confirmed by histopathological investigation like fine needle aspiration cytology or incisional/excisional biopsy.\nThe surgical excision of the mass was done and sent for histopathological examination. The postoperative period was uneventful and the patient is asymptomatic till date. The excised tissue was fixed in 10% formaldehyde for 24 h. Routine laboratory procedures were followed by paraffin embedding of the tissue. Five μm thick sections were obtained and stained with hematoxylin and eosin (H&E) for histological analysis ().\nMicroscopically, it was a thinly encapsulated tumor composed mainly of sheets of clear cells mixed with cells having eosinophilic cytoplasm. The cells showed small round to oval, bland nuclei that was eccentrically placed. In between these cells hyalinized fibrous septae were seen. Congested blood vessels and areas of hemorrhage were also noted.\nHistopathology revealed the possibility of a clear cell neoplasm. The differential diagnoses of clear cell tumors that can possibly occur in this region include benign and malignant salivary gland neoplasms and metastatic clear cell lesions especially from kidney and thyroid. Malignant primary salivary gland neoplasms include clear cell variant of mucoepidermoid carcinoma, acinic cell carcinoma showing clear cell changes, epithelial myoepithelial carcinoma, clear cell carcinoma, clear cell myoepithelial carcinoma and sebaceous carcinoma. In contrast to carcinomas, myoepitheliomas have a non-infiltrative, well-circumscribed periphery. Malignant primary and metastatic lesions were ruled out as there was no infiltrative growth pattern, cytologic pleomorphism, high mitotic rate, coagulative necrosis or lack of encapsulation.\nThe benign clear cell tumors of salivary gland include pleomorphic adenoma, myoepithelioma, oncocytoma, oncocytic hyperplasia and sebaceous adenoma. Absence of glanduloductal differentiation and chondromyxoid or chondroid foci made us delete pleomorphic adenoma from the differential diagnosis. Oncocytoma and oncocytic hyperplasia show the characteristic appearance of oncocytes. In the clear cell variant of oncocytoma, in which the clear cells are a dominant or partial component, the sparse granularity of the typical oncocyte will still be evident. Sebaceous adenoma is composed of irregularly sized and shaped nests of sebaceous cells without cytologic atypia.\nWhen pleomorphic adenoma, oncytoma, oncocytic hyperplasia and sebaceous adenoma were excluded from the histological diagnosis, we came to the conclusion that our case is a clear cell myoepithelioma. Conflicting reports are observed on immunostains of myoepitheliomas. The most consistent was the positive staining for cytokeratin, S100 and SMA whereas vimentin and GFAP expression vary. Among cytokeratins CK7 and CK14 are found usually positive. Pan cytokeratin and myoepithial markers like calponin, S-100, p63 are also generally positive for myoepitheliomas. In our case, immunohistochemical markers pan cytokeratin, S-100 and SMA was done and most of the tumor cells showed positivity for all the three markers ().\nSo a final diagnosis of clear cell myoepithelioma of palate was given.
A 16-year-old male patient, without any relevant aspects in his medical history except a Non-Hodgkin lymphoma of his father, which is currently being treated, and a breast cancer of his mother, also in treatment. The patient was derived from the Hematology Unit for evaluation and definitive treatment of an osteolytic lesion in the left maxillary region with an evolution of nearly 2 months. Thirty days before, the patient had consulted at a dental service for a routine dental exam in which a radiolucent image appeared in the radiographic study. The patient underwent a biopsy of an asymptomatic osteolytic lesion located at apical level of the first upper left molar.\nIn his admission to our service, the oral and maxillofacial clinical examination did not show facial asymmetry, swelling or solution of continuity in the left maxillary alveolar ridge. The patient only had a slight and generalized gingival inflammation, and no other findings.\nHe brought images of a recent dental study (panoramic radiography) and a report of a biopsy performed 30 days before. The imaging study reported an osteolytic lesion in the left maxillary ridge, extending between the first and second upper left molars, with projection to the maxillary sinus [Fig. ]. The biopsy report concluded that the sample was consistent with bone monostotic Eosinophilic granuloma (Langerhans histiocytosis). The histophatological findings of the performed biopsy, compatible with Eosinophilic granuloma, are showed in the Fig. and Fig. .\nTo complete the case study, additionally to the routine preoperative laboratory tests (blood count, biochemical profile and coagulation tests), a cone-beam CT (CBCT) was requested, and to rule out compromise of other bones, a scintigraphy was requested. Previously performed paraffin embedded samples of biopsy were also requested.\nLaboratory tests were normal. The new CBCT [Fig. ] showed inflammatory changes in relation to the left maxillary sinus with great opacification, an area of osteolysis in connection with the roots of the upper left first molar, causing soil discontinuity of the ipsilateral sinus; and extensive loss of continuity of the oral bone plate, compatible with post-surgical biopsy changes. Bone scintigraphy showed an increase in osteoblast activity in the left maxillary bone, related with the known bone injury. There were no other findings of pathological significance in the rest of the study [Fig. ].\nGiven these images of sinus compromise, nasal congestion and subsequent discharge reported by the patient, it was decided to complete the study with computed tomography (CT) of the sinuses before the complete surgical removal of the lesion [Fig. ], and refer the patient to the assessment and care of an otolaryngologist.\nThe patient came back to us 3 months after his first examination to schedule his surgical procedure, which prompted new imaging study to redefine treatment [Fig. ].\nLater studies showed recovery of the left maxillary sinus transparency in spite of the persistence of mucosal thickening of the sinus floor. Newly formed bone in the periphery and between the roots was compromised by the initial injury, and a decrease of the hypodense areas found in the previous examination was also observed [Fig. ].\nThese findings were consistent with spontaneous remission of the osteolytic lesion after the biopsy. The patient underwent regular checks to ensure no recurrence of his original injury; the last check was 9 months after the initial biopsy. Figures and show the grade of bone repair in the originally affected area. Since the EG is a benign lesion, which developed a spontaneous remission in this case, our patient’s prognosis is excellent, as long he follows his regular checks in order to detect any early recurrence or new outbreaks for at least 5 years.
A 38-year-old white male was found down in the field after he reportedly grabbed a “live” electrical wire. On the way to the local hospital, he regained consciousness but remained confused thereafter. He had sustained second-degree burns at the entry wound in the right palm and had a small exit wound in the right foot. Ecchymosis was also noted on right side of the body over the knee, thigh, and shoulder. A pan CT scan of the body did not reveal any major abnormality, including fractures. He remained hemodynamically stable but was pleasantly confused with a nonfocal neurological examination. A CT scan of the brain showed multiple hypodensities in the left parietal region and frontal region bilaterally. All serology labs were normal except total creatinine kinase levels, which peaked at 1100 U/L. Hence, he was transferred to the burn unit in our hospital for further management of burns with rhabdomyolysis. On examination, he was a pleasant gentleman with complete amnesia of the inciting event. He was alert but disoriented to all spheres. Most of the responses were limited to either head nodding or a few words associated with tangentiality and paraphasia. Repetition was impaired, but comprehension was preserved for simple commands. The cranial nerves and motor and sensory systems were clinically normal except limitation of the right arm's movement due to pain. MRI of the brain using 1.5 Tesla MRI machine showed multiple areas of diffusion restriction in the bilateral medial frontal lobe and bilateral basal ganglia along with watershed areas in the left frontal and temporal regions (). Susceptibility-weighted images showed hemorrhage in the left basal ganglia and insula (). T2-weighted images showed hyperintensity in the corresponding regions. MR angiogram did not reveal any filling defect or obvious vasospasm but showed aplastic A1 segment of the right anterior cerebral artery (). Based on these findings, acute stroke was diagnosed, likely due to vasospasm of the left anterior cerebral circulation due to electrocution. As the patient had presented 3 days after the injury and had shown clinical improvement, it was hypothesized that vasospasm of cerebral vessels might have been resolved when MR angiogram was performed. No hypotension in the field was reported by the first responders and no hypotension was documented during the initial intensive care unit stay at the outside hospital and telemetry monitoring at our center. Also, there was no evidence of end-organ injury due to hypotension induced hypoperfusion arguing against hypotension induced watershed cerebral infarction. Due to aplasia of the right A1 segment, both frontal lobes were supplied by the anterior cerebral artery and vasospasm of the left anterior cerebral circulation might have led to both frontal lobe infarcts along with watershed infarcts of the left middle cerebral artery territory.\nAn extensive work-up did not reveal any other risk factors for stroke. Transthoracic echocardiogram showed normal structural and functional heart without any intracardiac thrombus; vegetation and bubble study did not show any right to left shunt. Telemetry cardiac monitoring also failed to detect any cardiac arrhythmia. MRA neck did not show any evidence of carotid or vertebral artery disease. The patient's family denied that the patient had any preexisting medical or surgical conditions, including a substance abuse problem. He was an anthropology graduate working as a stunt man for movies and reported being healthy before this event. Despite extensive interviews, the circumstances of the electrocution could not be elucidated, except that he was barefoot at the time of the accident and it was a high-voltage electrical wire. People visiting a local church found him confused on the ground and called for emergency medical services. With supportive treatment, the patient's overall condition improved with some residual language defects. The mild rhabdomyolysis improved after hydration. The entry and exit electric burn wounds responded to local wound care. He was transferred to the neurorehabilitation center for further management and is reported to be recovering well.
A 69-year-old woman presented to the Rheumatology Clinic in January 2010 for evaluation of generalized morphea/localized scleroderma diagnosed 7 months after receiving local radiation to right breast for treatment of breast cancer. In September 2008, breast cancer was diagnosed by needle biopsy when an abnormal cluster of calcifications was identified by a screening mammography in September 2008. After consultation with her surgeon, oncologist, and radiation therapist, she underwent lumpectomy and sentinel lymph node biopsy. The cancer was estrogen receptor negative, and the sampled lymph nodes were negative. The tumor was estrogen and progesterone receptor positive. She opted not to take tamoxifen or other antiestrogen therapy. To prevent cancer recurrence, external beam radiation to right breast was completed (October 2008). She experienced local radiation side effects of erythema and superficial blister formation that resolved with local wound care.\nApproximately 7 months after completing radiation therapy, she developed two painful blisters in the inframammary fold of the right breast. On examination she was found to have generalized induration of the right breast and right axilla attributed to postradiation fibrosis. The breast and axilla were warm to touch and painful to palpation. That was with sparing of the areola. Additional lesions distant from the radiation field were also noted. These included hyperpigmentation of the skin at the waistline, induration of the skin in the left upper inner thigh and left groin, and a shiny patch of thickened skin on the anterior left shin. The lesions not involving the breast and axilla were not painful.\nIn May 2009, a punch biopsy of the left groin lesion diagnosed localized scleroderma (morphea). On microscopy, the skin surface was thin with a slightly keratotic epidermis. The papillary dermis was edematous and homogenous. The reticular dermis had dense collagen in bundles with intervening mild lymphoid and plasma cellular inflammation present around the adnexal structures. Mild chronic inflammation in the subcutaneous fat was noted at the base of the biopsy. For treatment of the right breast and axillary lesions, topical calcipotriol and topical betamethasone dipropionate were prescribed without benefit. Painful ulcers developed in the area of application in the right axilla which healed slowly when the topical medications were discontinued. The area under the right breast remained painful with chronic superficial ulceration. Application of topical silver impregnated dressings was of no benefit.\nIn addition to the skin lesions, she described fatigue and generalized arthralgias. She denied more specific symptoms of systemic connective tissue disease, that is, no history of Raynaud's, esophageal reflux, shortness of breath, cough, joint swelling, morning stiffness, sicca symptoms, pleuritis, or serositis. Prior to diagnosis of breast cancer, she had no significant past medical or psychiatric history and was on no prescribed medications. Family history was negative for connective tissue disease. Physical examination confirmed circumferential induration of the right breast with sparing of the areola. Induration extended into the right axilla. Superficial erosion of the epidermis was noted in the inframammary fold of the right breast. A band of hyperpigmented, thickened skin was noted along the anterior waistline. A hypopigmented indurated patch was noted in the left groin with a healed punch biopsy site. Thinning of the skin with hypopigmentation and superficial telangiectasias was noted in the upper left medial thigh. A 3 cm patch with central hypopigmentation was present on the left anterior mid tibia. She had no sclerodactyly. Nail fold examination for capillary changes was normal. Examination of the heart, lungs, and abdomen was normal. Laboratory testing was normal, including complete blood count, chemistry panel, thyroid function tests, and urine analysis. Sedimentation rate and C-reactive protein were normal. Lyme titer was negative. Thyroid autoantibody testing was negative. ANA was interpreted as borderline positive, with titer of 1 : 160 in a speckled pattern. Further testing for ANA subtypes was negative for anti-topoisomerase, anti-RNP, anti-Smith, anti-ds DNA, anti-SSA, and anti-SSB.\nShe was prescribed minocycline 100 mg twice a day, and, after three days of therapy, she reported dramatic improvement in generalized pain and fatigue. The superficial erosions in the inframammary fold healed. However, despite the initial perceived benefit, the right breast remained warm and tender to palpation. New lesions occurred on the right lower extremity. In August 2010, methotrexate 7.5 mg weekly and titrated to 12.5 mg was prescribed. Folic acid 1 mg per day was prescribed to prevent methotrexate side effects. After the addition of methotrexate, no new lesions occurred. Signs of inflammation in the irradiated right breast resolved though skin thickening remained unchanged. The lesions on bilateral lower extremities softened. Minocycline was discontinued in March 2011 when abnormal pigmentation developed as a side effect. She remained without signs or symptoms of a systemic connective tissue disease.
A previously healthy 6-year-old boy presented to a pediatric hospital with a 3-week history of torticollis. He had symptoms of an upper respiratory tract infection four weeks prior and had 2 days of documented fever at home during that time. He had been treated with a 7-day course of amoxicillin by the primary care physician for suspected streptococcal pharyngitis. Four days into the course of antibiotics, he woke up from sleep with pain on the left side of his neck. Despite taking ibuprofen and acetaminophen, he presented to the Emergency Department 3 weeks later due to persisting torticollis. Pain was worse with movement. There was no history of head/neck trauma. At the time of presentation, the infectious symptoms had resolved. Some fatigue was noted but he remained generally active, continuing to play hockey. There was no history of rash, peripheral joint pain, or weight loss. Past medical history and family history were unremarkable.\nOn examination, the patient was afebrile with normal blood pressure for age and a maximum heart rate of 110 beats per minute. The patient's head was tilted to the right with chin rotation to the left. No lymphadenopathy or masses were noted on palpation of the neck. There was no tenderness to palpation of bilateral sternocleidomastoid muscles. There was a limited range of motion in all planes of rotation of the neck secondary to pain, particularly in lateral flexion. Bilateral injected conjunctivas were present. The oropharynx was normal with no erythema or mucus membrane changes. Cardiovascular exam revealed normal peripheral pulses, a quiet precordium with normal heart sounds, and no murmur. Respiratory exam was normal. The abdomen was soft with no distension, tenderness, or hepatosplenomegaly. There were no bruits heard on auscultation of major vessel regions. There were no rashes or desquamation of the skin. Neurological exam was normal.\nAt the time of presentation, laboratory investigations revealed an elevated white blood cell count of 17.4 × 109/L with a neutrophil count of 14.1 × 109/L. Hemoglobin was normal for age at 110 g/L. Inflammatory markers were elevated including platelet count of 860 × 109/L and CRP of 38.5 mg/L. Renal function (BUN and creatinine) and liver function (ALP and ALT) were normal for age. Because of the unexplained elevated white blood cell count and evidence of inflammation, a chest X-ray was performed which revealed normal lung fields but an enlarged cardiac silhouette. X-ray of the cervical spine was normal with no atlantoaxial rotary subluxation demonstrated. Ultrasound of the neck revealed mild thickening of the left sternocleidomastoid muscle and no lymphadenopathy. Abdominal ultrasound with Doppler was normal.\nAdditional investigations included a normal throat swab for group A streptococci and a negative anti-streptolysin O antibody titer. High-sensitivity troponin was elevated to 176 ng/L. Creatinine kinase was normal. ANCA was normal. Electrocardiogram showed normal sinus rhythms without evidence of chamber hypertrophy. The patient underwent an echocardiogram to further characterize the enlarged cardiac silhouette identified on the chest X-ray. This revealed massive ectasia and aneurysmal dilatation of the right coronary artery, left main artery, left anterior descending artery, and circumflex arteries, as seen in . Left ventricular function was normal. The aortic arch was normal as were the proximal neck vessels.\nBecause of the dilated coronary aneurysms, the patient was diagnosed with KD. Despite lack of fever, given the evidence of ongoing inflammation and initial presence of bilateral nonsuppurative conjunctivitis, in addition to the coronary artery changes, the patient was treated with high-dose IVIG (2 g/kg) and started on daily low-dose aspirin. Low-molecular-weight heparin was started as antithrombotic therapy and once stabilized, daily atenolol was initiated. Activity was restricted as much as possible.\nInflammatory markers were followed. Platelets revealed a peak of 952 × 109/L and CRP a peak of 54.6 mg/L. After treatment, both platelet and CRP levels normalized.\nThe patient's neck pain and the limited range of movement resolved immediately after treatment, as did the bilateral conjunctivitis. The patient was stable and appeared well at time of discharge. His aspirin, low-molecular-weight heparin, and atenolol were continued. The CT angiogram performed after discharge revealed massively dilated and aneurysmal coronary arteries, as shown in .\nIn follow-up cardiology and rheumatology clinics, he has been doing well with no further neck pain or stiffness. He did not develop desquamation during follow-up, and the repeat echocardiogram one month after discharge was unchanged. He will continue long-term anticoagulation therapy with low-dose heparin with a target level greater than 0.5 IU/ml. He will also continue low dose aspirin and atenolol. His family was advised to have the annual influenza vaccine.
A 75-year-old man visited our hospital because of exacerbation of lower extremity edema that he had noticed 3 months before admission. He had received annual medical checks and had no specific medical history. His body weight had increased by 10 kg over the past 5 months, and his systemic edema was remarkable. Urinary protein was 9.9 g/day, no microscopic hematuria was detected, and his serum creatinine was 1.15 mg/dL on admission. The serum protein fractions showed an M peak at a high level and serum IgM was 1657 mg/dL. Protein electrophoresis showed IgM κ type M proteinemia. Although his monoclonal IgM was increased, bone marrow biopsy showed normal findings, with no infiltration of abnormal lymphocytes, in plasma cells, or chromosomal abnormalities, suggesting that this was a case of IgM monoclonal gammopathy rather than primary macroglobulinemia.\nBecause the patient did not agree to our proposal initially, a renal biopsy was performed 3 months after his first admission to elucidate the cause of his nephrotic syndrome. Thus, the etiology of his nephrosis was initially unknown due to the lack of renal biopsy. Considering his background, the possibility of membranous nephropathy was high; consequently, we administered 30 mg of oral prednisolone first. Three months after his first admission, the patient agreed to receive a renal biopsy.\nUnder light microscopy, the biopsy sample largely consisted of renal cortex and contained 34 glomeruli. Marked enlargement of the mesangial region and strong periodic acid-Schiff (PAS)-positive deposits were observed on the glomerular basement membrane and mesangial matrix (Fig. a). Periodic acid-methenamine-silver (PAM) staining lead to strong silver staining of the spicula in the glomerular basement membrane and mesangial matrix area (Fig. b), but only weak DFS staining and scarce positive blue-green birefringence under polarized light microscope was observed (Fig. c). The immunofluorescence analysis showed that IgM and κ chains were clearly deposited on the glomerulus, and mild deposits were found in the mesangial region. No significant deposition of other globulins was observed; we considered this to be a case of monoclonal deposition of IgM-κ chain (Fig. ).\nElectron microscopy showed deposition of fibrillar materials in the subepithelial and subepithelial regions of the mesangial and glomerular basement membranes. Deposition of fibrillar substances approximately 20 nm in diameter was also observed in the subendothelial and subepithelial layers of the mesangial and glomerular basement membrane by electron microscopy (Fig. ). Based on the results of DFS staining and electron microscopy, the patient was diagnosed with FGN. This diagnosis was confirmed 6 months after his first admission because renal biopsy could not be performed at first admission. Further, it took approximately 2 months to receive the electron microscopy result. Since urinary protein did not improve after administration of oral prednisolone only, 150 mg of mizoribine was added as a drug for nephrosis. Temporary urinary protein decreased to approximately 4 g/gCr, but a significant increase in serum creatinine subsequently occurred. A daily dose of 100 mg of oral cyclophosphamide was started on the 180th day to halt disease progression of FGN and suppress the production of monoclonal IgM, resulting in a reduction of the increase in serum creatinine and a decrease in urinary protein. One month after initiation, the daily dose of cyclophosphamide was decreased to 50 mg and continued for 2 weeks. Soon after, the patient decided to cease administration of oral cyclophosphamide and oral prednisolone; the cumulative dose of cyclophosphamide was approximately 3.7 g. Although cyclophosphamide might have had a positive effect on renal prognosis, the progression of renal dysfunction could not be halted, and hemodialysis was initiated on the 230th day. The patient had melena caused by intestinal bleeding, the etiology of which was amyloidosis, but a diagnosis of AHL was not confirmed in the intestinal tissue. There was no symptom of nerve conduction from amyloidosis. The melena was not observed at the time of hemodialysis initiation, but occurred 2 years after hemodialysis initiation. In addition, the patient contracted liver cirrhosis and was troubled by ascites. Due to the severe intestinal bleeding and liver cirrhosis, his blood pressure decreased and finally he could not receive hemodialysis. The patient died from multiple organ failure at another hospital approximately 2 years after hemodialysis initiation. A cardiac echocardiogram performed 1 month before his death did not show evidence of cardiac amyloidosis, such as shaggy heart and cardiac wall thickening. Therefore, multiple organ failure was presumed to be mainly caused by the liver dysfunction.\nBased on the several new insights into FGN recently reported, we decided to reassess the renal biopsy. Even though DFS staining was weak positive, Congo red staining was positive, leading us to believe that this was a case of congophilic FGN. To confirm the diagnosis, the renal tissue was evaluated by LMD/MS and the Scaffold database, and a minimum of 4 mass spectra as described previously []. Notably, the presence of massive monoclonal heavy chain components (constant region), light chain components (constant region), serum amyloid P components, and apolipoprotein E (Apo E) components indicated that this was a case of heavy-and-light-chain amyloidosis (Fig. ). No DNAJB9 component was detected. Although a blue-green birefringence under polarized light microscope in DFS staining was scarce (Fig. c), that in Congo red staining was positive (Fig. d).
The patient was a 60-year-old man. Abdominal ultrasonography indicated that he had an abdominal mass. He reported having perceived lower abdominal pain 7 months before the initial examination, but it spontaneously remitted. The patient had no fever nor any history of conditions such as hypertension. A blood test revealed that he did not have anemia, thrombocytopenia, or coagulation abnormalities. The patient demonstrated slightly elevated levels of carcinoembryonic antigen (4.7 ng/ml) and soluble interleukin-2 receptor (603 U/ml), tumor markers that are elevated in gastrointestinal cancers and malignant lymphoma. Contrast-enhanced CT showed a 33× 31 mm abdominal aortic aneurysm and a 106 × 81 mm mass peripheral to the abdominal aorta. The mass did not feature a contrast effect, and its margin was enhanced in the venous phase (Fig. ). Sigmoid wall thickening and inferior vena cava thrombosis were also observed (Fig. ). We suspected the patient had a neoplastic disease, such as a malignant lymphoma or sigmoid colon cancer. Detailed examinations were consequently performed, and direct oral anticoagulant therapy was initiated to treat the inferior vena cava thrombosis.\nThe FDG-PET revealed an abnormal accumulation of FDG in the margin of the mass and the sigmoid colon, but not in any other organs. Colonoscopy showed only mild inflammation in the sigmoid colon, and a sigmoid colon biopsy revealed no significant findings. Chronic inflamed fibrous connective tissue was harvested from the periaortic mass using CT-guided biopsy. There were no findings indicative of infection or neoplastic disease, thus precluding a pathological diagnosis.\nAt 1 month after the initial examination, intermittent claudication occurred, prompting the performance of follow-up contrast-enhanced CT scanning. On CT scanning, we found an arterial occlusion from the infrarenal abdominal aorta to the bilateral common iliac arteries, the right external iliac artery, and the right internal iliac artery (Fig. ). These findings indicated the potential progress of the tumor to the aorta. Via a midline incision, biopsies of the mass and the lymph nodes peripheral to the sigmoid colon were obtained. However, these biopsies showed only connective tissue with inflammation and fibrosis and did not yield any significant findings. Since the developments, we elected to resect the mass and perform bypass surgery for definitive diagnosis and treatment after 3 weeks from biopsy.\nSurgery was performed using a transperitoneal approach via a midline incision. Adhesion was observed peripheral to the abdominal aorta, the bilateral iliac arteries, and the retroperitoneal mass. The abdominal aortic lumen was completely occluded by an old thrombus and plaque. Removal of the thrombus revealed a 35-mm intimal defect consistent with the direction of the retroperitoneal mass (Fig. ). Communication was observed between the intimal defect site and the retroperitoneal mass, which was filled with a partially organized old thrombus. During the intraoperative rapid pathological examination, the tissue inside the mass was diagnosed as a fibrin clot. The tumor tissue was not assessed. The area from the infrarenal abdominal aorta to the right common femoral artery and the left common iliac artery was bypassed using a Y-shaped woven Dacron graft. Pathologic examination showed that the mass tissue was a partially organized fibrin clot, thus agreeing with our intraoperative rapid diagnosis. On the basis of the intraoperative and pathologic findings, the patient was diagnosed with CCR-AAA with abdominal aortic occlusion. His intermittent claudication improved, and he was discharged on day 19 after surgery. Six months after the operation, the retroperitoneal mass disappeared on CT.
A 60-year-old Egyptian male originally presented with syncope and acute -onset, sharp left flank, and left upper quadrant abdominal pain for four days associated with a three-hour loss of consciousness. The patient endorsed syncope upon rising from a chair at which point he slowly collapsed to the ground under the support of a nearby friend. There was no history of fever, hematuria, dysuria, frequency, urgency, or incomplete emptying. There was no association of chest pain or palpitations prior to the syncopal episode, and the patient had reported a few episodes of presyncope in the past associated with stress or sleep deprivation. Vitals upon admission were all within normal limits with a blood pressure of 126/80 mmHg, pulse 89 beats per minute, respiratory rate 22 breaths per minute, and temperature 98.1 °F. A review of systems was positive for a 20lb unintentional weight loss over several months. He was a lifetime nonsmoker with no personal history of cancer and only significant family history of a brother with prostate cancer. His original creatinine obtains on admission was 1.0 mg/dL. A non-contrast CT of the head was obtained while in the ED and was negative for any intracranial abnormality. A contrast-enhanced CT of the abdomen and pelvis was obtained and demonstrated a 5 mm left lower lobe pulmonary nodule, an irregular soft tissue density within the gallbladder suggestive of cholelithiasis vs a possible mass, and a large mass replacing most of the left kidney measuring 13.8 x 11.9 x 10.3 cm with scattered areas of calcification appearing both solid and cystic. No definite left renal vein extension and a grossly normal-appearing right kidney were appreciated as seen in Figure . An MRI of the abdomen was subsequently obtained which confirmed the absence of renal vein involvement.\nCardiology was initially consulted to evaluate for potential causes of the syncope and an echocardiogram was ordered in addition to placing the patient on telemetry. The echocardiogram revealed a normal-sized left and right ventricle with an estimated ejection fraction in the range of 55-60%. Additionally, the electrocardiogram (EKG) and telemetry strips demonstrated normal sinus rhythm suggesting the most likely cause of syncope to be orthostatic in nature and did not require a formal neurology consultation as there was no recurrence or enduring effects of this episode. At no point during his hospital stay did the patient experience any sustained and significant vital sign derangements. Four days after the original presentation and after a complete evaluation by urology, the patient was taken for an open left radical nephrectomy and cholecystectomy. Intraoperative findings demonstrated a large multilobulated left renal mass with extremely large serpiginous vessels extending down to the inguinal canal and up the left paracolic gutter. Frozen sections revealed negative surgical margins and a gallbladder positive for metastatic ccRCC. He was referred to medical oncology for stage IV, pT3aNxM1, with imaging negative for brain or bony metastasis. With negative margins and no evidence of disease, the patient elected for surveillance and did not pursue any additional adjuvant therapy. There were no additional significant findings at his initial postoperative visit. His postoperative creatinine remained relatively unchanged at 1.3 mg/dL. His follow-up was arranged on an as-needed basis with urology as he continued his primary surveillance with oncology.\nSubsequent surveillance imaging did not demonstrate any clinically significant change in his pulmonary nodule, nor did he demonstrate any recurrence of the disease. At almost three years postoperatively, the patient was noted to have a 2.5 x 1.7 cm exophytic right mid pole posterior renal mass as seen in Figure . The patient then underwent CT-guided right cryoablation of the renal mass three years from his original presentation. Since his ablation, he has been recurrence-free.
The patient was a 31-year-old healthy male who is self-employed in the shipping industry as a repairman of fiberglass boats. After consuming half a bottle of locally produced arrack (alcohol 38 % W/w) he ingested an estimated volume of 150 ml of MEKP containing solvent (60 % MEKP in phthalate solution) as an act of deliberate self-harm. He was unaware of the level of toxicity of the substance.\nThe patient developed severe throat and epigastric discomfort soon after ingestion. He was admitted to a regional hospital where he was given activated charcoal. A nasogastric tube was inserted in anticipation of erosive complications. Over the next 24 hours, his symptoms progressed with worsening irritation of the throat, development of abdominal pain, difficulty in breathing and noticeably reduced urine output. He was transferred to our tertiary care unit for further management of progressive multi organ involvement.\nOn general examination he was alert and coherent with Glasgow coma scale score of 15. There were multiple ulcerations in his mouth and oropharyngeal region. Nasogastric tube contents revealed coffee ground aspirate. Respiratory examination revealed rapid shallow breathing with a respiratory rate of 40 per minute. Auscultation of the lungs was unremarkable and oxygen saturation was 99 % on room air on pulse oxymetry. He was tachycardic (heart rate:104 beats per minute) with normal blood pressure and adequate peripheral perfusion. Severe guarding and tenderness over the epigastrium was present in examination of the abdomen. Bowel sounds were sluggish.\nPreliminary investigations revealed progressively rising serum creatinine and blood urea. He had a neutrophil leukocytosis (Table ). Arterial blood gas analysis showed a high anion gap metabolic acidosis with a pH of 7.21. Serum potassium was elevated at 6.4 mmol/L, which persisted through the first week of hospital stay. Ultrasound scan of the abdomen showed normal sized kidneys with evidence of acute renal failure. Subsequent complete blood counts showed a reducing trend in hemoglobin and platelets. Bite cells, Heinz bodies and fragmented red cells were also seen. Serum lactate dehydrogenase level was elevated. Direct Coombs test was negative. The reticulocyte count was 1.5 % showing an inadequate bone marrow response. Hemolysis settled by the second week of the illness.\nThe patient was managed in the ward. Acute management included intravenous sodium bicarbonate to correct acidosis and medical management of hyperkalemia. Urgent hemodialysis was followed by regular renal replacement therapy for the next four weeks. Repeated blood transfusions (8 units during the first week) were required to maintain hemoglobin levels.\nGastric protection was instituted with intravenous proton pump inhibitors. Parenteral feeding was initiated due to intolerance of oral feeds. On third day the patient developed large volume coffee ground aspirate through the NG tube, which later became bilious. This heralded distal duodenal obstruction, which was confirmed by a gastrografin study. This resolved after 2 weeks and liquid feeds were gradually started as tolerated. However, he had persistent odynophagia. Upper gastrointestinal endoscopy performed on day 28 revealed inflammatory lesions extending from upper esophagus to duodenum. There were slough, exudate and fibrotic bands but significant stricture formation was not seen (Fig. ).\nThe patient was discharged on the 29th day. He was tolerating semisolid diet despite having persisting odynophagia. He had lost 4.7 kg during his hospital stay. His follow up plan included a repeat endoscopic assessment in 3 months time, psychiatric assessment and follow up with the medical unit. He was also referred to the dietitian for nutritional support and supervision.\nBy his first review in 2 weeks the renal functions had returned to baseline. First follow up endoscopy at 3 months revealed slough, which was cleared by the passage of the endoscope. This resulted in temporary relief of odynophagia. However it recurred and the odynophagia persisted for six months. At 6-month review he was tolerating normal feeds with only slight discomfort. He has lost further 7 kg (weight on admission 68 kg) but features of other nutritional deficiencies were not present. One year after ingestion, he was asymptomatic and was gaining weight.
A 47-year-old male visited an otorhinolaryngologist with a chief complaint of bilateral hearing loss since July 2019, which was gradual in onset. In November 2019, the treating otorhinolaryngologist after managing conservatively and reporting no relief in symptoms referred the case to the medical oncology department of a private hospital in New Delhi. On examination by a medical oncologist, the patient reported the loss of hearing and pain. He complained of pain over the preauricular region, which was described as dull, aching, and bilateral. Pain which used to be on and off and started as mild but gradually increased on rare occasions which got relieved by taking tablet tramadol 50 mg. At the clinic, the patient got investigated, and one small growth was found at the posterior nasopharyngeal wall which was confirmed to be NPC on biopsy. PET scan report shows that the disease is confined to its anatomical site of origin and is not metastasized yet. He was given six cycles of chemotherapy (CT) over a span of 9 weeks. He responded to the treatment and planned for further therapies. Then, the patient was given concurrent chemoradiation. The patient got the last radiotherapy on April 10 and chemotherapy on April 8.\nOne week later, he developed a low-grade fever which was considered as a complication of chemotherapy. However, when fever continued, he was suspected of corona infection, so he was sent to the COVID-19 testing center where he came negative. Then, the patient developed urinary retention the next day and found to have some growth in urinary bladder and bladder was overflowing according to CT scan. Urinary catheterization tried but failed, so the patient was planned for suprapubic catheterization. Before the procedure, he was again tested for COVID-19, and the report came out to be positive. Plan for suprapubic catheterization was changed and urinary catheterization tried which was successful this time with smaller size catheter (10 F). Then, the patient was sent to RML Hospital, New Delhi, on April 28, and from there, he was referred to the COVID care facility at NCI-AIIMS Jhajjar.\nThe patient is asymptomatic given COVID-19 infection and being managed conservatively. His vitals are stable; oxygen saturation is well maintained above 98% on room air. He has been tested thrice for COVID-19. He is still getting reverse transcription polymerase chain reaction (RT-PCR) positive for a month. As the patient got COVID-19 infection just after his therapies for NPC, immunosuppression can be the reason. This shows that cancer patients with ongoing treatments have a higher risk.\nHe is not able to communicate properly with health-care workers and his family members on the phone as he has hearing difficulty. Due to a lack of communication, he feels lonely and unattended. His father is the main caregiver, and his family has been quarantined so he is unable to get a few items which he needs from home. As the father is unable to come to meet his son, he is also feeling guilty for not being able to fulfill his needs.\nHis first report was negative then subsequent 3 RT-PCR test reports came positive. He is regularly asking about his report and doubting the results. He is worried and in doubt that why others are getting negative after 2 tests, but he is not. He does not trust the report. His father is also repeatedly calling to ask why his report is coming positive.\nHe has this fear that his condition will deteriorate as he is getting repeated positive reports. He is afraid that his cancer treatment plans will get delayed and will affect his outcome.\nDue to the overwhelming number of people seeking medical care and the burden that COVID-19 is placing on health-care providers, it has become more difficult to access regular cancer treatments during this time. Further treatment of cancer and follow-up is getting interrupted due to COVID-19 infection. Delay in the treatment is making the patient more anxious and irritable. His father believes that his cancer will get cured if he gets further treatment. But due to COVID-19, his treatment is getting affected.\nThe role of a caregiver during this time is to provide support and stability. Recommend patient or family members to avoid watching the news if it causes them anxiety or concern. Reassure patient that you will always be available by phone or video call. Continue to remain in contact with your patient. As a caregiver, he should be the pillar of security and comfort. Preparing a safe environment and providing the right resources will ensure the patient.
An otherwise healthy and physically active 67-year-old man presented with severe lower back pain and bilateral leg pain more so in the right leg than left. His leg pain worsened with walking and improved with sitting consistently with neurogenic claudication. He also had diffuse tenderness to palpation along the midline of his lumbar back. At the time, the patient had been using a cane due to difficulty walking. He had a past surgical history of left ankle fusion with a leg-length discrepancy. Additionally, he was undergoing evaluation for bilateral knee replacements. Examination revealed the patient had a forward-leaning posture, bowed legs, pain with lumbar extension, and inability to stand on his toes and heels due to ankle fusion. His leg strength was normal and his sensation was grossly intact to light touch. X-ray imaging of the lumbar spine revealed straightening of the normal lumbar lordotic curvature with mild rotoscoliosis, but severe degenerative changes in the lumbar spine. The patient had Baastrup’s disease at the L3-4 and L4-5 levels that contributed to his reduced range of motion on extension imaging.\nStanding preoperative sagittal scoliosis film revealed a coronal vertical axis of +51 mm and a sagittal vertical axis of +82 mm with the following pelvic parameters: 29 degrees of pelvic incidence (PI), 15 degrees of sacral slope (SS), and a pelvic tilt (PT) of 13 degrees. Further, there appeared to be a reversal of lumbar lordosis centered around L2-L3. Lumbar lordosis measured approximately -2 degrees from the superior endplate of L1 to the inferior endplate of L5. There was also a straightening of the thoracic kyphosis with approximately 26 degrees from the superior endplate of T1 to the inferior endplate of T12 (Figure ).\nMRI without contrast of the lumbar spine displayed multilevel degenerative disease with severe stenosis from L2-L5. Interestingly, his alignment changed when lying supine for the MRI and he appeared to regain some lumbar lordosis (Figures -).\nIntervention and outcome\nGiven the patient’s loss of lumbar lordosis and positive sagittal alignment, deformity correction surgery was considered; however, his symptoms were felt to be mostly neurogenic claudication related to his severe multilevel stenosis and less from his spinal deformity. We felt that his loss of lumbar lordosis was from his Baastrup’s disease at L3 and L4 since putting his lumbar spine into kyphosis increased the interspinous distance. Open full decompressive lumbar laminectomies with bilateral medial facetectomies and foraminotomies from L3-L5 were recommended in order to address his multilevel stenosis and resect his spinous processes causing Baastrup’s disease.\nThe patient tolerated the procedure well and was discharged home on the same day. The postoperative course was uncomplicated and involved physical therapy to work on core strengthening exercises to improve his overall spinal alignment.\nOn follow-up, the patient has a resolution of his back pain and radicular leg pain. Likewise, he reported feeling “much straighter” while walking as he no longer had pain when leaning backward. Follow-up standing scoliosis survey films two months and six months postoperatively demonstrated significantly improved sagittal forward balance and improvement in both his lumbar lordosis and thoracic kyphosis (Figures , ), as well as improvement in his pelvic parameters (Table ).
A 31-year-old male patient, carpenter by occupation was admitted to the hospital on 25-01-2012 for instability of gait since 1 month duration. There was also slurring of speech since then. There was a history of tingling and numbness of both lower limbs, which had gradually ascended up to the knees for the past 1 month. The footwear used to slip off his feet while walking. Because of the numbness of the feet and the in coordination he was dependent on the family members for most of his daily activities. He experienced clumsiness while buttoning his shirt and mixing the food was also observed. Tremulous and overshooting of hand while eating, food was observed.\nThe patient had a generalized tonic-clonic seizure in the hospital, on the day of admission. There was a past history of 3 seizures 3 weeks earlier, on 15-12-2011 however, he was not evaluated at that time and was not on any antiepileptic medication at the time of admission. He was not a known epileptic before starting the anti-amoebic medication.\nThere was a history of weight loss of 11 kg in the past 4 months. He was admitted to the gastroenterology unit of the hospital 4 months ago, where he was diagnosed to be suffering amoebic liver abscess. The liver abscess was drained twice in 15 days’ time and he was administered metronidazole infusion in the hospital at that time and he was asked to stop alcohol. As the abscess was resolving, he was advised to continue metronidazole tablets 400 mg 5 tablets daily for a long period for another 2 weeks. Patient came for review after nearly 3 months with diarrhea. The patient was taking metronidazole for nearly 4 months before admission. In the last 1 month, Ornidazole 250 mg + Diloxanidee furoate 200 mg twice daily were added for control of diarrhea (without the knowledge that he was also taking metronidazole), The latter combination was taken for 3 weeks and discontinued, thereafter since the patient could not afford it.\nOn examination, he was alert and co-operative; there were no cranial nerve deficits. Speech was slurred with a scanning character. There was hypotonia in all 4 limbs. The muscle power on medical research council (MRC) grading scale was normal except minimal weakness of toe extensors bilaterally. The plantar reflexer were flexor, deep tendon reflexes were normal. There was intention tremor, dysmetria, dysdiadochokinesia in both upper limbs. In-coordination was noted in both upper and lower limbs. He was unable to stand on a narrow base. The gait was ataxic and he was unable to walk tandem. There was impairment of all modalities of sensation in both lower limbs below the knees.\nThe patient was investigated for his complaints. The laboratory work-up showed total leucocyte count of 8600/Cu mm with normal differential count. Platelets were adequate on smear and the count was 230,000/Cu mm. Random blood glucose was 143 mg/dl, blood urea was 37 mg/dl, and serum creatinine was 1.2 mg/dl. Liver function tests were normal. An ultrasound examination of the abdomen was normal, and the abscess had resolved. Nerve conduction study of the limbs suggested a length dependent axonal sensorimotor neuropathy. X-ray chest and electrocardiogram ruled out any cardiac involvement. Magnetic resonance imaging (MRI) scanning of the brain showed faint hyper-intense signals on T2 and FLAIR imaging from the dentate nucleus bilaterally more prominent on the left side [Figures -]. There was also hyper-intense signal in the splenium of corpus callosum.\nOn the day of admission, the seizures were controlled with diazepam. He was started on an anti-epileptic drug, levetiracetam. A diagnosis of post-infectious immune mediated disease like acute disseminated encephalomyelitis (ADEM) was considered initially as the seizures and ataxia followed a diarrheal illness. He was given one bolus of high-dose methylprednisolone as part of treatment for ADEM. When the MRI did not reveal typical lesions of ADEM, the history and clinical findings were reviewed. Metronidazole was suspected to cause the adverse reaction and was discontinued.\nThe patient was discharged after 7 days and followed-up after 10 days. Dysarthria and ataxia had resolved. There were no more seizures. Levetiracetam was also discontinued. The sensory symptoms in the lower limbs were persisting; however, he was able to walk independently.
A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine (basically removed); intestinal histopathology (cecum, biopsy) showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen (Fig. A) revealed a more homogeneous thickening of the gastric wall in the antrum. Round unenhanced low-density foci with a diameter of 4.6 cm was seen in the S7 segment of the liver. Nodular calcifications were also observed. No significant dilatation was noted in the intrahepatic and extrahepatic bile ducts. The size and shape of the gallbladder were normal, the wall was not thick, and no significant abnormal density was observed in the cavity. The pancreas, spleen, and adrenal glands showed no significant abnormalities. Test results from computed tomography indicated liver cyst and intrahepatic calcifications. Contrast-enhanced magnetic resonance imaging of the liver and gallbladder (Fig. B, ) revealed a normal size and shape of the liver and proportion of each lobe, and the intrahepatic and extrahepatic bile ducts and flow vessels ran naturally. A long T1 and long T2 cystic signal with a diameter of about 4.7 cm was observed in the right lobe of the liver, with liquid level, short T1 high signal intensity in the lower layer, high signal intensity on diffusion-weighted imaging sequence, and enhancement of the cyst wall on the enhanced scan. Test results from magnetic resonance imaging showed that the space-occupying lesion of the right lobe of the liver had been considered to be more likely a hepatic hydatid cyst. Preoperatively, three-dimensional reconstruction demonstrated the location of the tumor and its relationship with the surrounding vessels (Fig. A).\nThe patient underwent laparoscopic S7 segmentectomy. Intraoperative findings showed that the tumor was located at the S7 segment of the liver and was about 5 × 4 cm in size and partially protruding from the surface of the liver, with an intact capsule and clear boundary with normal liver tissue. The tumor compressed the right hepatic vein and its tributaries and densely adhered to the right hepatic vein (Fig. B). On postoperative pathology, a mass was observed immediately adjacent to the liver capsule, with a volume of 5 × 5 × 4.5 cm. The section surface showed a brown turbid fluid, a smooth inner wall, and greyish red, greyish yellow, and soft section surfaces of other liver tissues. The pathological section showed a low-grade mucinous cystic neoplasm (volume 5 × 5 × 4.5 cm) in the S7 segment of the liver, with steatosis in the surrounding hepatic tissue area, chronic inflammatory cell infiltration in the portal area, and no tumor cell involvement in the margin of the liver resection.\nImmunohistochemistry demonstrated tumour cells CK7 (+), CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin (a small amount +), vimentin (+), desmin (+), and actin (+) (Fig. A–C). This study was approved by the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University.
Our patient was a 74-year-old man who had received dialysis for 8 years due to end-stage renal disease. His hiccups started 1 year after the beginning of dialysis and occurred 2-3 times per week, typically lasting 1-2 hours each time. A magnetic resonance image (MRI) scan of his brain did not detect any problems, apart from for a lacunar infarct of the central pontine. He was treated with drugs in the department of internal medicine with no apparent improvement in symptoms. The patient had undergone coronary artery bypass graft surgery 6 years earlier due to myocardial infarction; subsequently, his hiccups worsened, appearing very frequently, especially when he finished eating in the afternoon or tried to sleep at night. He usually suffered 3-4 hours once the hiccups started. To treat his aggravated symptoms, 5 mg of baclofen and 100 mg of gabapentin were given twice a day, making him somewhat better. However, he was not able to increase the dose, as he showed symptoms of drowsiness and ataxia at a higher dose. Eventually, the patient was referred to our pain clinic, complaining of fatigue, insomnia, and weight loss.\nOur first action for the patient was blocking the right phrenic nerve guided by ultrasound using 4 ml of 0.2% ropivacaine and 10 mg of triamcinolone (). This procedure decreased the frequency of the hiccups for about one week, but afterward, his symptoms returned to their previous state. Three weeks later, we attempted to perform the same procedure on the left phrenic nerve, only to have the same outcome: a relapse after approximately 1 week.\nConsequently, we decided to perform PRFL, targeting both phrenic nerves with the use of ultrasound. After explaining the procedure, efficacy and possible side effects of the pulsed RF treatment, we put the patient in a supine position and turned his face to the left in order to perform the procedure, aiming at the right phrenic nerve. We prepped the skin above with betadine and aseptically draped and placed a 10 MHz, linear probe at a position 2 cm cephalad from the top of the clavicle to observe the anatomical structures of his neck through ultrasound (Xario, Toshiba, USA). After discovering the phrenic nerves situated in the middle, anterior margin of anterior scalene (), the 10 cm RF electrode (Neurotherm, Medipoint, Germany) with a 5 mm active tip was advanced as close as possible to the nerves by using the ultrasound. Nerve stimulation using the RF lesion generator (Neurotherm, Morgan Automation LTD, UK) showed concordant movement of 120 times per minutes in upper abdomen at 2 Hz and 0.5 mA which confirmed proper localization of the RF electrode. After clarifying the location of the target nerves once again, we performed PRFL twice for a total of 120 seconds at 42℃ and at stimulation intervals of 20 ms/sec. Following this procedure, the same method was applied to the left phrenic nerve.\nThroughout the treatment, the patient did not show any problem; he went back home after taking a rest for 1 hour. The frequency of his hiccups gradually decreased; they were at half of the previous level 1 month after the treatment, when he visited our hospital as an outpatient. In addition, the number of days he was completely free from hiccups were increased. To improve the effectiveness, we repeated the same treatment after 2 and 4 months and confirmed that he was completely healed from hiccups according to the follow up by asking the patient by telephone call 1 month after he received the last treatment.
A 72-year-old male presented to our institution for orthopedic consultation following a visit to his primary care physician with a complaint of approximately five weeks of progressive bilateral lower back pain without a history of trauma. The pain was described as sharp with movement and constantly dull at rest with a rating of 4/10 on the VAS (Visual Analog Scale) pain scale. On physical examination, there was tenderness over L1 and L2, but no deficits in lower extremity sensation, motor strength, or reflexes. Computed tomography (CT) of the lumbar spine without contrast was performed and mild compression fractures of L2 and L3 were identified, likely subacute and non-healed (Figure ). No soft tissue masses were noted. Abnormal marrow signal intensity was also seen compatible with an infiltrative marrow process such as MM. One week later, a follow-up magnetic resonance imaging (MRI) of the thoracic spine with and without contrast showed a 1.5 cm enhancing lesion in the right pedicle of T8, as well as a diffuse infiltrative process of the marrow. These results prompted a referral to hematology to confirm the diagnosis of MM.\nLab tests were performed by the hematology consultant to evaluate for the presence of MM. A serum protein electrophoresis showed gamma globulins reduced to 0.9% with an abnormal protein of 0.4 g%. The immunoglobulin studies showed a 0.34 g% monoclonal component of lambda light chains. Two samples of kappa and lambda light chains were elevated. One sample showed elevated lambda light chains to 335 mg/dL, giving a ratio of 0.6. The other sample showed elevated lambda light chains to 11,463 mg/dL. Chemistries were abnormal, with an elevated BUN (blood urea nitrogen) at 42 and creatinine at 1.98. Quantitative immunoglobulin studies were normal.\nDue to the concerning results of the blood work, a radiographic bone survey was performed. It revealed innumerable small lytic lesions scattered throughout the axial and appendicular skeleton, all likely secondary to MM. The patient was subsequently put on a combination of bortezomib (Velcade) and dexamethasone for treatment, which worked well, decreasing lambda light chains from 11,463 mg/dL to 49.16 mg/dL on repeat lab work approximately 10 weeks later.\nThe patient was admitted approximately three months after the myeloma diagnosis for extreme right hip pain and CT scan of the right hip, abdomen, and pelvis was performed. The study showed a right total hip arthroplasty and an osseous mass extending from the right acetabulum and right iliac crest. The mass had a “sunburst” pattern (Figure ). The mass was partially ossified with soft tissue components (Figure -).\nOver the next few weeks, in addition to persistent severe right hip pain, the patient developed progressive shortness of breath, cough, and fever. Multiple CT scans of the chest over several weeks showed progressively worsening infiltrates, effusions, and mid and lower lung nodules, with most nodules showing calcifications (Figures -). Metastatic OS from the right pelvis was suspected. Wedge resections of the left lower lobe and left upper lobe were obtained and histopathologic assessment was performed on the obtained tissue.\nThe resected tumors revealed identical histopathology. Figure demonstrates areas of hemorrhagic necrosis and sheets of viable tumor cells that display irregularly shaped, variably sized, hyperchromatic, and sometimes vesicular nuclei. Varying amounts of pale eosinophilic cytoplasm without brown granular pigment, mucinous vacuoles, or cytoplasmic keratinization are also visualized (Figure ). Architecturally, the tumor cells do not form glands, cysts, papillary fronds or any formed patterns. There are also curved or irregularly shaped deposits of eosinophilic extracellular matrix consistent with malignant osteoid (Figure ). Immunohistochemical stains showed no immunoreactivity to four keratin markers (AE1, Cam 5.2, 34betaE12, CK5/6), S100, SOX10, calretinin, Wilms tumor-1, carcinoembryonic antigen (CEA), thyroid transcription factor-1 (TTF-1), or MOC-31. These overall findings were diagnostic of metastatic osteogenic sarcoma.
This study presented a case of a 78-year-old female with an 18-month history of painless progressive swelling and erythema on the left forehead and right upper eyelid. The lesions were slightly developed and involved all of the scalp surface, eyelids, and face; however, there was no other abnormality. The chief complaint of the patient was the mass on the right eyelid. She had a proptosis of the right eye and an orbital mass; however, there were no signs of headache, nausea, vomiting, or double vision. The medical history of the patient demonstrated that she had hypertension and coronary artery disease, and had undergone angioplasty. However, she had no history of undergoing radiotherapy or chemotherapy. The result of the brain MRI in May 2015 demonstrated a mucosal thickening in the right maxillary sinus. A soft tissue with a slightly lobulated surface was observed using magnetic resonance imaging (MRI). The ventricles, subarachnoid space, and brain stem demonstrated normal appearance, and there was no evidence of mass lesion or midline shift. The MRI with and without contrast, performed 2 months later, revealed a tissue measuring 5 × 3 × 1 cm and weighing 12 g in the right orbital mass. The microscopic findings demonstrated a benign lesion composed of a mixture of angiomatous tissue and lobulated fatty tissue connected with skeletal muscle tissue, which was suggestive of hemangioma. The brain computed tomography (CT) findings showed that there were no abnormalities in the brain structure. There was diffused skin and subcutaneous soft tissue edema in the cranial area, which was more pronounced in the radiation therapy site. The ultrasound reviewed an inflammatory soft tissue in the right frontotemporal region progressing to the ear. The patient used atenolol 50 mg (twice daily), triamterene-h (half a pill every 12 h), acetylsalicylic acid (daily), and atorvastatin 20 mg (daily). In July 2016, she was hospitalized in the infectious diseases ward with a diagnosis of cellulitis and received oral antibiotic therapy (cephalexin) for 2 weeks. Owing to the lack of response to the treatment, she was then referred to the dermatology department. Swelling and slightly warm soft tissue along with erythema was observed throughout the facial surface and scalp. The swelling of the eyelids resulted in complete closure of the eyelids on both sides, which impaired the patient’s vision; however, the patient’s eye exam was normal (Fig. ). The complete blood count test was done for the patient (hemoglobin level: 14.7 g/dl, white blood cells: 5950 cells/mm3, neutrophils: 50%, lymphocytes: 35%, monocytes: 10%, eosinophils: 1%, basophils: 3%, sodium: 141 mEq/L, potassium: 3.8 mmol/L, blood urea nitrogen: 53 mg/dL, creatinine: 1.2 mg/dL, blood sugar: 163 mg/dL). Furthermore, the urinalysis and liver enzyme test demonstrated normal results. Based on the CT scan with and without contrast, no anomalies or bone lesions were reported. The brain MRI showed that there was an area of increased signal intensity on the axial T2-weighted images in the skin and subcutaneous tissue of the right cheek. Five months later, an elliptical biopsy at the margin of the lesion was done and the microscopic examination revealed a malignant vascular neoplasm composed of a poorly circumscribed dermal and subcutaneous tumor with an infiltrative growth pattern. In some areas, there were proliferations of solid epithelioid cells with vesicular nuclei, eosinophilic cytoplasm, and intracytoplasmic lumina, which intermixed through vascular channels with retiform architecture lined by plump hyperchromatic endothelial cells with a hobnail appearance. The lining of endothelial cells in some areas showed multi-layering plump and occasionally pleomorphic nuclei with scattered mitotic figures (Figs and ). The diagnosis was a low-grade malignant vascular neoplasm compatible with CHE. The patient was referred to undergo the immunohistochemistry (IHC) test. The IHC test demonstrated negative results regarding the tumor cell in MNF 116 and SMA; however, it showed positive results for CD31 and CD34 (Fig. ). Furthermore, Ki67 was observed to be positive in only 8% of the tumor cells. Based on the combination of histopathological findings and IHC results, the patient was diagnosed with CHE; therefore, she was recommended to undergo a complete lesion excision due to the involved cervical lymph node. The patient underwent partial excision of lesions and skin grafting. Post-surgery, she received chemotherapy by 100 mg thalidomide (daily). Following the thalidomide treatment, the erythema and swelling regressed, and she was able to open the eyelids.
A 37-year-old woman was presented to the emergency room with abrupt severe right upper abdominal pain. The pain was squeezing and started suddenly after breakfast. The pain was not accompanied by vomiting or fever. The patient had previously been treated at the Department of Medicine in Samsung Medical Center with a diagnosis of EGPA. After successful suppression of blood hypereosinophilia with combination of prednisolone and azathioprine in recent months, she had been in the process of tapering prednisolone down to 15 mg per day. However, after dose reduction, her eosinophil counts slowly increased to 1989 cells/µL and levels of aspartate aminotransferase and alanine aminotransferase were elevated from normal to more than 300 U/L with mild abdominal pain. Consequently, her dose of prednisolone was raised back to 60 mg per day. While waiting for admission for further evaluation, abdominal pain brought her to emergency room.\nFive years ago, her first visit was prompted by symptoms of sinusitis, rash and eosinophilia. Three months later, numbness and cutting pain in both upper and lower extremities with fever, myalgia, and asthma developed. A nerve conduction study suggested multifocal sensorimotor neuropathy. Biopsy of the sural nerve revealed ischemic change resulting from vasculitis with prominent eosinophilic infiltration. Although perinuclear anti-neutrophil cytoplasmic antibodies were not detected, the patient was diagnosed as EGPA. After repeated intravenous cyclophosphamide pulse therapy with a high dose of systemic steroids, most symptoms were resolved. Sinusitis, presented as an initial symptom, improved after treatment at an early stage and otolaryngologic complication did not appear afterwards.\nHowever, whenever the tapering a dose of steroids was attempted, she developed new symptoms such as myalgia and arthralgia. She even experienced dry gangrene in the fingertips of both hands because of arterial thrombosis when her eosinophil count increased up to 6000 cells/µL (Fig. ). Duplex scan for arteries and veins indicated the total occlusion of the right distal ulnar artery and left medial forearm ulnar artery. The study also revealed a total occlusion of both mid-anterior tibial arteries, whereas toes of both feet were intact as other vessels of lower extremities were not involved. Hypercoagulability was thought to be caused either directly or indirectly by hypereosinophilia. We recommended mepolizumab, a monoclonal antibody to interleukin-5 used to interrupt eosinophil production, to reduce eosinophil count. But the patient refused it because of cost. We added aspirin and calcium channel blockers after cardiologic consultation. After the affected fingers were dried up, necrotized fingertips were amputated by orthopedic surgeon.\nInitial vital signs in the ER showed a blood pressure of 85/45 mmHg, a heart rate of 98 beats per minute with a temperature of 36.1’C, and a respiratory rate of 16 breaths per minute. Physical examination revealed mild tenderness in the right upper abdomen without rebound tenderness. The complete blood count revealed leukocytosis of 17,660 white blood cells/µL. Absolute eosinophil count was 1120 cells/µL. The erythrocyte sedimentation rate was 29 mm/hr, CRP was 3.7 mg/dL and total immunoglobulin E was 107 kU/L.\nAn abdomen-pelvis CT demonstrated subcapsular hematoma in the right hepatic lobe (Fig. ). Hemoperitoneum probably caused by multifocal hepatic capsular arterial bleeding was also detected. There was no evidence of bowel perforation. In a previous CT scan taken 1 year before, there had been no evidence of any aneurysm changes in the hepatic vessels. A celiac angiogram demonstrated multiple, various sized hepatic aneurysms in both lobes (Fig. ). The small aneurysms resembled the appearance of a string of beads, characteristically observed in polyarteritis nodosa (PAN). Huge aneurysms larger than 1 cm in size were noticed in S7 and S8. Emergency embolization was performed. The huge aneurysm and the aneurysms with bead-like appearance in S7 and S8 were successfully occluded. Although several small aneurysms in S5, S6 and the left lobe still remained, no further embolization was performed to conserve hepatic function.\nMeticulous review of the abdomen-pelvis CT did not reveal evidence of aneurysms in other abdominal organs. After emergency intervention, vital signs became stable and numbness of both hands and feet improved. Based on her clinical course, we strongly recommended to add mepolizumab to control hypereosinophilia and reduce the dose of steroid. After administration of mepolizumab in addition to the previous steroid and cyclophosphamide therapy, eosinophil count became stable and was reduced to a near zero percentage of total white blood cell count.
A 50-year-old male patient was referred to our institution for evaluation of a known spinal cord mass. He presented with left leg weakness and low back pain for the previous 6 months. On the lumbar spine magnetic resonance (MR) images taken with a 1.5 Tesla (T) MR scanner and in the hospital that referred the patient to us, about a 2 cm sized mass that arose from the conus medullaris was found at the level of the T12 to L1 vertebrae. Compared with the adjacent muscles, the signal intensity of the mass was heterogeneously hyperintense on the fat suppressed T2-weighted image (T2WI) () and it was isoinstense on the T1-weighted image (T1WI) (). The mass showed multifocal nodular enhancement on the fat-suppressed T1WI obtained after the intravenous injection of gadolinium contrast agent (). Although they were overlooked during the initial assessment of these MR images from the referring hospital, multiple bony lesions were present in the thoracolumbar vertebral bodies and sacrum. Compared with the adjacent muscle, the lesions were heterogeneously hyperintense on the fat suppressed T2WI () and they were hypointense on the T1WI (). The bony lesions showed heterogeneous enhancement on the fat suppressed T1WI obtained after the intravenous injection of gadolinium compound (CE T1WI) (). Eight days after the first MR imaging, the intramedullary mass was partially removed for the purpose of pathologic confirmation and to halt further neurologic impairment. The final pathologic examination revealed the anaplastic astrocytoma (). For the remaining primary mass, the patient received radiation therapy to the spinal canal at the level of the T12 through the L1 vertebral bodies with a dosage of 5400 cGy.\nThe follow-up MR imaging was performed 60 days after the surgery to assess the postsurgical and radiotherapy status of the spinal cord. The MR images revealed a shrunken primary mass of the spinal cord. However, the bony lesions of the entire vertebral column were found to be worsened and they had become heterogeneous, and bone metastasis was considered to have already happened at that time (). Subsequently, computed tomography (CT) of the chest, abdomen and pelvis was performed right after the follow-up MR imaging for the further evaluation of distant metastasis. Matching all the bony lesions depicted on the MR images with those seen on the CT images, the lesions demonstrated hyperdense attenuation, suggesting osteoblastic metastasis (). A whole body bone scan and PET-CT (positron emission tomography-computed tomography) were also sequentially performed within the next two days after the CT examinations. On the whole body bone scan images obtained from a dual head gamma camera (Millennium VG, GE Medical Systems, Milwaukee, WI), multiple foci of heterogeneously increased bony uptakes were detected in the thoracolumbar spine, the bilateral rib cages and the pelvic bones, which were thought to be consistent with the multiple bony lesions noted on CT and MRI (). On the whole body 18F-fluorodeoxyglucose (FDG) PET-CT using a Biograph 40 (Siemens/CTIMI, Knoxville, TN), among all the osteoblastic lesions, focally increased FDG uptake was noted only on the T11 vertebral body (the SUV 2.19). No extraskeletal metastatic focus was observed on PET-CT (). For an accurate pathologic diagnosis of the bony lesions, CT-guided percutaneous biopsy was performed with targeting the lesion of the T11 vertebral body based on the findings of PET-CT. However, the biopsy revealed a non-satisfactory result because the amount of specimen was not sufficient for making a confirmative diagnosis. Hence, CT-guided localization was carried out using a guide pin and then a subsequent open bone biopsy was done, instead of a percutaneous biopsy, for the iliac bone (). A bone block measuring 4 × 1 × 2 cm was obtained, and the pathologic result from the specimen revealed metastatic anaplastic astrocytoma ().\nThe patient had undergone 2 cycles of chemotherapy with the use of carboplatin and vincristine before he was found to have bone metastasis. However, after the diagnosis of bone metastasis, the chemotherapy regimen was changed into temozolomide, and cisplatin was also administered to the patient during one of the cycles. After the last session of chemotherapy, the patient was lost to follow up.
A 77-year-old woman was referred for the treatment of her severely worn dentition. Her chief complaint was that she could not eat anything because her teeth were worn too much. The patient had anticoagulant and analgesic agent due to hypertension and idiopathic headache. Intraoral examination revealed a generalized loss of dental substance that was greater in the maxillary left incisors and the mandibular right incisors. Maxillary left canine and mandibular right canine were worn to the gingival level, and had got root canal treatments (). The anterior teeth had sharp enamel edges, dentinal craters, and attritional wear due to the loss of posterior support. The mandibular posterior teeth were missing, but she said that she did not use her old removable partial denture (RPD) as it was not comfortable. The mandibular RPD lost its retention and support because the regions that functioned as the rest and retentive undercut of abutment teeth had been fractured. The facial type of patient was square and her lip seemed to be under strong tension. The patient did not have temporomandibular disorder history and soreness of the mastication muscles, but the discrepancy between centric occlusion (CO) and maximum intercuspal position (MIP) was found when she was guided to CR with bimanual technique. The transcranial view was taken to determine whether a temporomandibular problem exists. The right mandibular condyle was flatter than the left one, but any specific disorder was not found ().\nTo determine whether VDO had been altered, the following aspects were investigated:,,\nLoss of posterior support: mandibular posterior teeth were missing, and the patient did not use the mandibular partial denture. Posterior collapse resulted in excessive wear and fracture of anterior teeth. History of wear: Physiologic wear can be compensated by tooth eruption in general, but the accelerated wear may exceed the rate of eruption. The patient liked vegetables and acidic fruits. Her favorite food was tough and fibrous. In addition, maxillary posterior base metal prostheses might accelerate the wear of mandibular acrylic resin teeth and unbalance of wear rate. Phonetic evaluation: If the distance between the incisal edge of the mandibular incisors and lingual surface of the maxillary incisors is about 1 mm, it makes normal /s/ sound. The patient's increased space altered /s/ sound to /∫/. Interocclusal rest space: The patient's interocclusal rest space that was measured between nose tip and chin tip was 5 - 6 mm that was greater than the normal value, 2 - 4 mm. Facial appearance: Wrinkles and drooping commissures around mouth were observed.\nThe possible causes of patient's worn dentition that might include posterior interferences, parafunction, eating habit, and dental ignorance were explained to the patient. And the options of treatment plan were restoring mandibular edentulous posterior region with implants or removable partial denture, full mouth rehabilitation with metal ceramic restoration with or without crown lengthening procedure. The patient was scared of implant surgery, so the option of implant installation was excluded. Also the patient did not want to get multiple crown lengthening procedures and endodontic treatments to restore the worn teeth in the insufficient space. As there was clinical evaluation of reduced VDO, full mouth rehabilitation with increasing VDO was planned. Only severely worn teeth which were maxillary left canine and mandibular right canine were undergone crown lengthening procedures to obtain a sufficient clinical crown length and ferrule effect.\nThe patient's casts were mounted on a semi-adjustable articulator (Hanau™ Modular Articulator; Whip Mix Corp., Louisville, USA) using a face-bow record and an interocclusal record that was made with the aid of a Lucia jig and polyvinylsiloxane occlusal registration material (EXABITE II; GC Corp., Tokyo, Japan). The new VDO was set by 5 mm increase in the incisal guidance pin of the articulator. Because the patient's interocclusal rest space was 2 - 3 mm larger on the premolar area than normal distance, the actual increase were determined 3 mm in the anterior teeth and 1 - 2 mm in the posterior teeth. The splint was designed to offer bilateral contacts of all posterior teeth in centric relation and guides of the anterior teeth in excursive movement (). The anterior guidance disoccluded the posterior teeth in all jaw position except centric relation.\nThe adaptation of patient to the increased VDO was evaluated during 1-month trial period. No muscle tenderness and temporomandibular discomfort was found. The method of increasing VDO with the splint was used to determine desirable VDO of the fixed interim prostheses. After taking CR record using Lucia jig and wax-rim, diagnostic wax-up was performed (). Autopolymerizing acrylic resin (ALIKE™; GC America, ALSIP, USA) provisional crowns were fabricated using a vacuum formed matrix (Drufolen H; Dreve Dentamid GmbH, Unna, Germany) that was produced from the diagnostic wax-up, and mandibular provisional RPD was made to fit provisional crowns (). The provisional fixed restorations were cemented with temporary cement (FREEGENOL TEMPORARY PACK; GC Corp., Tokyo, Japan), and the patient's adaptation was monitored.\nFor three months, interim restorations were adjusted, and used as a guide for the definitive oral rehabilitation. During this period, the patient's condition and functions, such as muscle tenderness, discomfort of TMJ, mastication, range of the mandibular movements, swallowing, and speech, were evaluated. Improvement in mastication, speech, and facial esthetics confirmed the patient's tolerance to the new mandibular position with the restored VDO. The anterior guidance and posterior disclusion on excursive movement were established. Adjusted occlusion was transferred to customized anterior guide table, which was made with acrylic resin (PATTERN RESIN; GC Corp, Tokyo, Japan)().\nFinal preparation was performed, and definitive impressions were made with polyvinylsiloxane impression material (Extrude; Kerr Corp., Romulus, Germany). Bite registration was taken using provisional crown and occlusal registration material (StoneBite; Dreve Dentamid GmbH, Unna, Germany) by half and half (). Porcelain fused to metal restorations were made using customized anterior guide table and cemented with resin modified glass ionomer cement (FujiCEM; GC America, Alsip, USA). Because the patient's anterior guidance table was used in the production of definitie restoration, the amount of occlusal adjustment on the lingual surface of maxillary anterior teeth was minimal. Individual tray with polyvinylsiloxane (EXAMIXFINE; GC Corp., Tokyo, Japan) was used for the impression of mandibular RPD. The impression on posterior alveolar ridge was taken once more with the individual tray which is attached to the RPD framework, and the altered cast was made. After the adaptation of RPD framework and the trial of wax denture were done, the definitive mandibular RPD was fabricated and delivered with minor occlusal adjustment (). The prostheses were designed using mutually protected occlusion. The anterior teeth protected the posterior teeth from excursive force and wear, and posterior teeth supported the bite force. Oral hygiene instruction and regular check-up were administered.
A 50-year-old woman presented with back pain. A gastric tumor was suspected by upper gastrointestinal series at a local clinic, and she was referred to our hospital. Upper gastrointestinal endoscopy showed a submucosal tumor in the posterior wall of the stomach body (Fig. ). Contrast-enhanced computed tomography (CT) showed a well-demarcated inhomogeneously enhanced solid mass in the posterior wall of the stomach (Fig. ). CT also showed a slightly enhanced lesion on the right side of the uterus (Fig. a). T2-weighted magnetic resonance imaging (MRI) showed a mass around the uterus (Fig. b). Dynamic MRI showed a gradually enhanced mass (Fig. c). Based on these results, a preoperative diagnosis of GIST accompanied by an ovarian tumor, leiomyoma, lymphoma, or disseminated lesion from GIST was made. Partial resection of the stomach and extended hysterectomy after exploratory laparoscopy was scheduled. The laparoscopy demonstrated a protruded tumor from the posterior wall of the stomach in the omental bursa. A soft solid tumor was observed in the broad ligament, and multiple small masses were found around the uterus. No disseminated lesion was observed near the gastric tumor in the upper abdominal space including the omental bursa. Considering these findings, we concluded that the pelvic tumors were not disseminated lesions from GIST. A tentative diagnosis of gastric GIST and ovarian tumor with disseminations was made. Intraoperative frozen sectioning of the tumor in the pelvic cavity showed spindle cells, suggesting the presence of mesenchymal tumor such as leiomyoma. However, it was difficult to rule out the presence of disseminated lesions from gastric GIST. Partial resection of the stomach was performed; thereafter, the tumors in the pelvic cavity were removed by total hysterectomy and bilateral salpingo-oophorectomy under laparotomy.\nThe resected gastric specimen showed an encapsulated solid tumor measuring 45 mm in the greatest dimension (Fig. a). Microscopically, the gastric tumor was composed of spindle cells forming bundles in an interlacing pattern (Fig. b). Upon immunohistochemical analysis, the tumor cells showed positivity for c-KIT and CD34 (Fig. c, d) and very low positivity for Ki-67 and negativity for actin, desmin, HHF-35, S-100 protein, estrogen receptor (ER), and progesterone receptor (PgR) (Table ). These findings were identical for GIST. Meanwhile, the tumor of the broad ligament showed ill-defined or fused small nodules (Fig. a). Microscopically, the nodules of the broad ligament, uterus, and its surrounding peritoneum were composed of spindle cells resembling smooth muscle cells (Fig. b). Immunohistochemically, they were positive for smooth muscle actin, desmin, HHF-35, ER, and PgR (Fig. c, d), but negative for S-100 protein, CD34, and c-KIT (Table ). The pelvic tumors were diagnosed as DPL based on these immunohistopathological results. The risk grade of GIST was low because the mitotic counts of GIST were < 5 per 50 HPF. The postoperative course was uneventful. The patient had no complications and was discharged on the 14th day after surgery. Subsequently, the patient was followed up at 6-month intervals for the first 5 years and then annually for another 5 years. She has neither had any recurrence nor received any drug therapy for 10 years.
This 79-years-old gentleman was admitted from the Infectious Diseases clinic, in April 2011. He presented to the clinic with night sweats, intermittent fevers, rigors, lethargy, weight loss, poor appetite, and generalised arthralgias. He described a change in the bowel habit over the previous few months with constipation and mild abdominal discomfort. His C-reactive protein was 58. This was his fourth follow-up clinic appointment following a recent hospital discharge.\nHis past medical history included the following: August 2010 admission for relapsed septic arthritis of a right native knee, joint fluid aspirated at that time isolated Pseudomonas aeruginosa and blood cultures repeatedly isolated Pseudomonas aeruginosa and Enterobacter; July 2010 admission for probable recurrent septic arthritis, blood cultures isolated Streptococcus constellatus and Aerococcus; December 2009 admission for a primary septic arthritis, culture of synovial fluid isolated Streptococcus constellatus. In 1993 he had undergone an aortic aneurysm repair with insertion of an aorto-bi-iliac Dacron surgical graft.\nOn his admission in December 2009, because of the in situ aortic graft, he had undergone a CT abdomen and pelvis which showed normal appearances of the aorto-bi-iliac graft. The scan was repeated on each subsequent admission, in July 2010 and August 2010, each time showing normal appearances of the graft and no evidence of a fluid or gas collection around the abdominal aorta or iliac arteries. On admission from clinic, he underwent investigation for PUO in which he had multiple sets of blood cultures collected and 3 sets of isolated lactobacillus ().\nHe underwent a whole body scan showing a pool of activity in the right knee suggestive of chronic low-grade persistent infection. A transthoracic echocardiogram and a colonoscopy to investigate the altered bowel habit were carried out and were both negative. At this time a fourth repeat CT abdomen and pelvis was requested. This showed small extramural pockets of gas at the level of the graft bifurcation and a further possible tiny pocket of gas at the cranial aspect of the graft, appearances which would be suspicious for sepsis related to the graft.\nAt this point, there was multidisciplinary team input from vascular surgeons, vascular radiologists, infectious diseases and microbiology and appropriate management options were discussed. The two available options were (1) explantation of the graft with extra-anatomical bypass and (2) long-term suppressive antibiotics with serial CRP and imaging. In view of his age, that the WCC was within normal limits and the CRP was falling, the equivalence of the CT finding, and the fact that the surgery to remove the graft would carry a significant mortality and morbidity risk, the decision was made to start IV antibiotics for a minimum of 6 wks. The caveat to this approach was that in the event of worsening sepsis despite antibiotics and/or CT evidence of worsening perigraft infection or development of aortoenteric fistula, then surgery would be carried out.\nIV Daptomycin (6 mg/kg) (plus gentamicin initially) was commenced after confirmatory MIC testing of the lactobacillus to Daptomycin; this was the most recently and most persistently isolated pathogen. Daptomycin was well tolerated by the patient. At 6 weeks, based on good clinical response, this was changed to oral amoxicillin plus clindamycin, and the patient was discharged home. Within 48 hrs of discharge he represented with rigors and fevers. He was readmitted and a repeat CT showed increasing air encircling the right most anterior limb of the aortic graft. At this point the decision was made to carry out surgery to remove the graft.\nIn July 2011, the infected graft was explanted, the space washed out, and bilateral axillofemoral bypass graft inserted. Intraoperatively, a perforated duodenum was reported, which was the likely source of the multiple bacteraemias isolating the variety of gut organisms seen. This was repaired involving a retrocolic gastrojejunostomy and feeding jejunostomy, in an operation lasting more than 10 hours ().\nTo date, the patient remains well at home, the graft remains patent and perfusing the leg, and he has chosen not to continue oral suppressive antibiotics.
The patient is a 74-year-old male with comorbidities of coronary artery disease, congestive heart failure, hypertension, and renal insufficiency who presented to an outside hospital with back pain and left upper extremity weakness associated with high fevers and urinary incontinence. Outside spinal imaging showed a large spinal abscess; therefore, he was transferred to our institution for a higher level of care. On presentation, the patient was in septic shock requiring fluid boluses and inotropic agents to stabilize him. Further history was obtained from the family as follows:On November 2016, he underwent an elective right TKA complicated by an early MRSA PJI associated with bacteremia On May 0f 2017, he underwent resection of the hardware in the knee, incision and drainage, followed by 12 weeks of daptomycin therapy Five months later, on October of 2017, due to relapse, he had a second debridement of the right knee for source control as well as left ankle incision and debridement followed by another 8 weeks of daptomycin for this relapse of infection A daptomycin-susceptible, vancomycin-susceptible MRSA was isolated from blood and both surgical sites, knee and ankle, on both occasions He had ongoing thoracic back pain since 2016 which was monitored radiographically by his local providers, until the development of spinal epidural abscess with upper extremity weakness, which prompted his current hospitalization in January of 2018\nReview of systems on presenting to our institution was significant for general weakness and malaise, right shoulder and thoracic back pain, and constipation from narcotics. He was hemodynamically unstable requiring inotropic support. He was awake and oriented, following commands with intact speech. There were no cranial nerve deficits. On motor testing, he had normal muscle bulk with generalized hypotonia. There was no movement of his left upper extremity. He had 2/5 strength on his right upper extremity and 2/5 strength on his bilateral lower extremity. There was decreased sensation to light touch on his left side. Reflexes were globally decreased with negative Hoffman and Babinski signs. The white blood cell count was 30,000/L, and procalcitonin was 4.88 ng/ml. Blood cultures grew MRSA rapidly. Repeat imaging of the brain and spine at our institution showed extensive epidural phlegmon throughout the cervical, thoracic, and lumbar spine with intracranial expansion into the posterior fossa beneath the cerebellum with pockets of possible early organizing abscess within the phlegmon (). Brain imaging identified no discrete abscess or leptomeningeal enhancement.\nNeurosurgery immediately evaluated the patient and promptly performed a cervical spine decompression of C1–C7 and thoracic spine decompression of T5–T7. Operatively, a large epidural abscess was found, drained, and washed out. He was started on vancomycin every 12 hours with trough vancomycin levels being therapeutic. The patient subsequently underwent irrigation and debridement of the right knee, left ankle, and left great toe as well at our institution; all surgical sites grew MRSA with vancomycin MIC of 1 mcg/ml. Despite attempts at source control and optimal pharmacokinetic dosing of vancomycin with a trough level of 20.5 mcg/ml on day 5, he had refractory MRSA bacteremia. Infectious disease deemed he had failed daptomycin therapy; therefore, ceftaroline 600 mg every 8 hours (MIC of 0.38 mcg/ml) was added to vancomycin. Repeat blood cultures showed clearance of bacteremia after 48 hours of initiation of the combination therapy. His left ankle and right knee continued to yield MRSA. Due to his multiple comorbidities and need for more aggressive source control of his infection, i.e., amputation of the leg, palliative care was sought by the family, and he died a few days later.
An 86-year-old woman was admitted from an outside institution to our neurological intensive care unit with fluctuating consciousness after hitting her head during a fall. Four weeks before admission, she was in normal health for her age with a history of arterial hypertension. Because of atrial fibrillation, she was treated with rivaroxaban 20 mg once daily. Two weeks before hospitalization, she experienced pain in her lower abdomen accompanied by a feeling of illness and fatigue which she self-medicated with aspirin. She experienced nose bleeding but continued to take aspirin. She was then admitted to an external clinic after falling. During the first in-hospital night, she fell out of bed and struck her head. Thereafter, consciousness decreased and she was transferred to the neurocritical care unit of our institution. She presented with dysarthria and mild motor aphasia, but language comprehension was fully preserved. In addition, mild right-sided hemiparesis was noted. Most of the time, she was awake but intermittently somnolent. Body temperature was 37.6 °C. Routine laboratory tests revealed prolonged prothrombin time, increased international normalized ratio (INR), increased CRP, leukocytosis and corresponding signs of a urinary tract infection. She was treated with prothrombin complex concentrate and antibiotics.\nThe initial computed tomography (CT) on day 0 showed contusions in the left frontal and temporal lobes and tSAH. Figure a shows this first CT scan with subarachnoid blood in two sulci of the left frontal cortex. A contre-coup injury was found in the right posterior cranial fossa with an epidural hematoma and corresponding tSAH. In addition, a small intra-parenchymal hemorrhage was observed in the right basal ganglia. Arterial aneurysms or arteriovenous malformation were ruled out using CT angiography (CTA). Blood was also detected in the fourth ventricle, but signs of disturbed cerebrospinal fluid circulation were not seen. Accordingly, the patient did not receive external ventricular drainage. Further CT scans on days 1, 3 and 5 showed neither increase of the epidural hematoma nor development of an occlusive hydrocephalus. They revealed only the progressive decrease of Hounsfield units within the blood-related hyperdensities. Nimodipine was not given because there is currently no recommendation for its use after tSAH [].\nThe patient was alert and in an improved general condition when she was transferred to the normal ward on day 7. On day 8, however, the patient experienced a secondary deterioration of her neurological status, as characterized by reduced consciousness and global aphasia, with loss of ability to speak or understand. Whereas the CT scan on day 9 was still unremarkable, the MRI scan on day 10 revealed new cortical laminar infarcts adjacent to the sulcal clots (Fig. c and d). The infarcts included both Broca and Wernicke areas. Transcranial Doppler sonography (TCD) on day 10 showed normal mean velocities of the posterior cerebral arteries (right PCA 29 cm/s, left PCA 23 cm/s) and normal pulsatility indices. The bone window was not sufficient to assess velocities of the middle cerebral arteries (MCA). Neither MR angiography (MRA) on day 10 nor CTA on day 14 showed any signs of proximal vasospasm (Fig. e and f). However, the CT scan on day 14 revealed the same infarcts as the MRI scan on day 10 (Fig. b). Over the following week, the patient improved again. She was able to respond to simple commands when she was transferred to clinical rehabilitation.
A 32-month-old Middle Eastern boy was born full term at a community hospital in Michigan with birth weight of 3135 g (15.0 percentile). He had normal prenatal ultrasounds. He passed meconium at birth and had no other complications including prolong neonatal jaundice or dehydration. His CF NBS showed serum IRT 139 ng/ml and was negative for the 40 gene mutations panel. At 1 month of age, he developed a wet cough without any other symptoms. He was followed by his primary care provider (PCP), and no treatment was given at the time. His symptoms continued on and off until 1 year of age. At 1 year, the mother noticed increased frequency of productive cough, lack of appetite, and poor weight gain. His weight-for-age percentile ranged from 0.3 to 5.0. His stools were reportedly normal. He had no excessive sweating. He was referred to an outside asthma/allergy specialist for evaluation of asthma. He was prescribed budesonide without any improvement. He had frequent pharyngitis and otitis media that were treated with oral antibiotics that reportedly helped treat acute infection, but the cough persisted. He was also prescribed a H2 blocker for possible gastroesophageal reflux disease, but no improvement in symptoms was noted. Family history was negative for CF.\nAt 30 months of age, he was seen by his PCP for one week of cough and fever. He was treated with amoxicillin. His symptoms continued to worsen despite oral antibiotics, and he had two episodes of small-volume hemoptysis. He was subsequently admitted for community-acquired pneumonia and influenza B. Chest X-ray showed diffuse ill-defined opacities in the perihilar area and diffuse bronchiectasis. During the hospitalization, pediatric pulmonary consult was obtained. Given the negative NBS, it was stated that CF was unlikely and no sweat chloride test was recommended. He had a normal videofluoroscopic swallow study. Immunodeficiency workup revealed elevated immunoglobulin levels, protective vaccine titers, and normal lymphocyte counts and response to phytohaemagglutinin, concanavalin A, and pokeweed mitogen. HIV test was negative. Pediatric gastroenterology was consulted for failure to thrive and recommended to continue high-calorie diet. He was discharged home on augmentin.\nTen days following discharge, he was seen at the immunology clinic. He was noted to have digital clubbing, worsening tachypnea, and crackles. With the concerning physical exam findings, a sweat chloride test was done with a result of 90 mmol/L (normal 0–29 mmol/L; intermediate 30–59 mmol/L; abnormal ≥60 mmol/L) []. He was referred to pediatric pulmonary clinic the same day. He was then admitted and treated for a CF exacerbation. Throat culture grew Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus (MSSA). Fecal elastase-1 was <50 mcg E/g stool (normal >200 mcg E/g stool). Lab results including comprehensive metabolic panel and vitamin A and E levels were normal. He completed two weeks of cefepime and tobramycin.\nAfter notifying MDHHS with the false-negative NBS results, the blood spot that was available at the NBS lab was retested using the new and expanded mutation panel (60 mutations). He was found to be homozygous for R1066C (c.3196C > T; p.Arg1066Cys) mutation. His care was transferred to our CF center, as per parents' request. Two weeks later, he was admitted for worsening respiratory symptoms and treated for a CF exacerbation. Vitamin D level was low at 25 ng/ml (normal ≥30 ng/ml). High-resolution computed tomography of the chest showed diffuse bilateral bronchiectasis (). Flexible bronchoscopy showed airway erythema and significant thick green secretions () that was positive for MSSA.
A 54-year-old woman was admitted with fever and dull abdominal pain. She had been postmenopausal for 18 months and had no abnormal vaginal bleeding. A physical examination confirmed the presence of a cystic mass 10 cm in diameter on the right posterior of the uterus with clear boundaries, poor activity and no tenderness. The serum levels of CA125 and CA199 were 88.8 U/ml and 40.4 U/ml, respectively. Vaginal ultrasonography revealed a cystic mass full of fine spots that was located in the right ovarian area; the mass had clear boundaries, irregular low echo protrusions on the wall and no blood flow signals on CDFI. A solid mass with clear boundaries and a diameter of 7.35.96.5 cm was located below the former site of the cystic mass, and abundant arteriovenous blood flow signals could be seen inside and around the mass.\nHer medical history included 30 years of dysmenorrhea and 18 years of endometriosis and adenomyosis.\nWe performed an exploratory laparotomy, and during the operation, a small amount of bloody ascites was observed. We observed that the size of the uterus was approximately equal to that at 6 weeks of pregnancy, and no obvious abnormality was seen in the left ovarian area. A cystic-solid mass with an approximate diameter of 10*15 cm was seen in the right ovarian area. After 600 ml of thin, chocolate-like intracapsular fluid was removed, we observed that the mass was densely adherent to the surrounding pelvic peritoneum and that the rectal pouch was completely closed. The source of the mass was found to be the left accessory ovary, and thus, the left adnexa was completely removed. After the mass was cut open, the contents appeared pale white, crisp and vortex-free and contained a chocolate-like liquid. The intraoperative rapid pathology report described a right ovarian spindle cell tumor with a large necrotic area, which was considered a sex cord-stromal tumor, but a malignant tumor was not excluded. After communicating with the patient’s family, they wanted to wait for the final pathology results before proceeding with additional surgery. Total hysterectomy and bilateral adnexectomy were then performed.\nHowever, 10 days later, the final pathologic diagnosis was fibrosarcoma of the right ovary, with extensive necrosis. The mitotic counts were evaluated > 40 times/10 HPFs. Immunohistochemical analysis showed the following: CD31 (+), Ki-67 (+ 70%), SMA (+), AE1/AE3 (-), CD34 (-), CD117 (-), ER (-), PR (-), Desmin (-). No tumor cells were found during peritoneal washing.\nAfter surgery, the serum levels of CA125 and CA19-9 were 109.0 U/ml and 5.6 U/ml, respectively. The patient was given 1 cycle of systemic chemotherapy consisting of Cisplatin + Epirubicin + Ifosfamide (PEI). After 1 course of chemotherapy, the serum levels of CA125 and CA19-9 were 24.9 U/ml and 4.3 U/ml, respectively.\nWe then performed a second laparoscopic exploration, adhesion decompression and partial removal of the pelvic mass 5 weeks after the first surgery and 3 weeks after chemotherapy. During the surgery, a hard, solid mass approximately 5 cm in diameter was palpable at the top of the stump and was located below the adhesion of the bladder and rectum. The surface of the mass was not visible. No obvious tumor lesions were seen on the pelvic peritoneal surfaces and visceral surfaces. Due to the tight adhesion, separation was extremely difficult, and the mass did not have a capsule or boundaries. Only 2/3 of the tumor was removed, and after the mass was opened, solid, brittle gray material was present.\nThe pathology report still described a fibrosarcoma. One week after the second surgery, the serum level of CA125 was 35.1 U/ml, and she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin (TC). After 1 course of TC, the serum level of CA125 was 32.9 U/ml, after which the chemotherapy ended, and the patient died within 1 year after surgery (Table ).
The patient was a 29-year-old woman who had been in good health until 1995, at which time she was diagnosed at 10 years of age with type 1 diabetes with a positive glutamate decarboxylase (GAD) antibody, and insulin treatment was started. Although her mother had a history of migraines, no history of diabetes in her family was observed.\nBecause she was bullied in elementary school due to diabetes, she developed overeating behaviors and her blood glucose levels became unstable; frequent hospitalizations were required and became pivotal to her daily life. Unfortunately, the patient continued to experience bullying even in junior high school, and her mother was often absent from home due to work; so the patient began overeating due to loneliness and anxiety. She was then allowed admission to a pediatric long-term care facility where she was also bullied by other patients. In addition, she was repeatedly hospitalized due to high plasma glucose levels. Ultimately, she entered a nursing-high school at the local hospital and graduated from junior high school. Following admission to the high school, to perform education through distance learning, she graduated at 20 years of age. During this period, she was hospitalized for a psychosomatic illness when she was 17 years old. She was transferred from the local hospital to another hospital’s pediatric unit. Furthermore, she began to display signs of retinopathy and overt proteinuria.\nIn September 2007, at 23 years old, the patient first visited our hospital through referral from the pediatrician at the previous hospital as she had become an adult and glycemic control was becoming increasingly difficult.\nIn December 2007, the patient, due to vomiting in response to stressful events, was first admitted to our hospital. On physical examination, her height was 158 cm, weight was 56.9 kg, BMI was 22.8 kg/m2, body temperature was 38.4 °C, blood pressure was 146/91 mmHg, and pulse was 117 bpm and regular. Her cardiopulmonary examination was normal. She had no abnormal abdominal findings. Her bilateral Achilles tendon reflexes were absent and bilateral lower extremity vibration sensation was also absent. On ophthalmologic examination, she had diabetic microangiopathies and growth arrest retinopathy. She demonstrated peripheral and autonomic neuropathy and had evidence of diabetic nephropathy of the third stage, i.e., overt proteinuria had been diagnosed, and her estimated glomerular filtration rate was 139.7 mL/min/1.73m2. She had no evidence of chronic thyroiditis (anti-thyroglobulin antibodies 11 IU/mL and anti-thyroid peroxidase antibodies 7 IU/mL; normal range: ≤ 28 IU/mL and ≤ 16 IU/mL, respectively).\nDue to financial difficulties, she was only able to afford outpatient visits once every 2 months. Her HbA1c at this time remained at 13 %. In April 2009, she was repeatedly hospitalized with dehydration and diabetic ketoacidosis (DKA) due to the interruption of insulin injections because of frequent vomiting that had appeared in response to stressful events. At that time, vomiting episodes occurred more than 30 times a day, and hematemesis was also observed. Nausea and increased salivation always persisted. Episodes were so severe that the patient was often found lying on the floor unresponsive and nearly unconscious. In addition, both fever and elevation of blood pressure were observed. For antiemetic therapy, intravenous administration of both diazepam and metoclopramide were ineffective, but intravenous administration of haloperidol was slightly effective. From around the 4th or 5th day after admission, vomiting gradually improved. During the period of vomiting, excessive secretion of both ACTH and ADH were observed, but they promptly improved during remission (Table ). As a result of psychological intervention (behavioral therapy to include a combination of counseling and autogenic training, etc.) by a clinical psychologist, glycemic control showed temporary improvement (Fig. ). However, in December of the same year, we began CSII treatment during the vomiting phase because self-injection of insulin was interrupted and glycemic control had become more difficult with her HbA1c showing another upward trend (Fig. ). As a result, she no longer developed DKA during the periods of vomiting and was capable of maintaining her activities of daily living, while repeated cycles of vomiting remitted and glycemic control also showed a trend toward improvement (Fig. ).\nSubsequently, around March 2011, the patient’s vomiting episodes worsened and were more prolonged. She had a prolonged period of hospitalization, and her daily life again became difficult. Although her nutrition was managed by nasogastric tube feedings and central venous nutrition, her body weight decreased significantly to 38.0 kg with a body mass index (BMI) of 15.2 kg/m2.\nIn February 2012, we started hypnotherapy. The treatment was limited by poor language comprehension (Wechsler Adult Intelligence Scale-Third Edition [WEIS-III] score was 60 points), and a developmental disorder was inferred. Even so, the patient was able to be discharged because vomiting frequency was reduced by a combination of hypnotherapy and sandplay therapy (Fig. ). Today, her episodes of vomiting have not completely remitted, but it was possible to shift her to outpatient management, and her HbA1c has also been maintained at 7 %.
A 67-year-old man presented after being struck by a vehicle while riding his motorcycle with complaints of pain in his neck, pelvis, and left upper extremity. There was no loss of consciousness. Urinary function could not be assessed at initial presentation due to the presence of a Foley catheter, but there was no blood at the urethral meatus. Medical history was significant only for hypertension. On examination, he was conscious, oriented, and hypotensive (103/55 mm Hg). There was hypogastric tenderness and exposed bone through an open wound near his left elbow. Neurovascular status was preserved in both the upper and lower extremities. There was no damage to the spinal cord elements. Anteroposterior pelvic radiograph revealed pubic diastasis of 4.7 cm and a midline fracture through the sacral body with 1.0 cm of displacement (). A pelvic binder was placed to decrease intrapelvic hemorrhage and repeat plain films demonstrated acceptable closure of his pelvic diastasis (). After binder application and the placement of two large-bore intravenous cannulas administering crystalloid isotonic solution, hemodynamic stability was achieved and the patient was deemed stable a contrast-enhanced computed tomography CT scan revealed hemoperitoneum, 1.3 cm of pubic diastasis, and a complete, midline sacral fracture through the spinal canal and posterior sacral elements extending from S1 to the coccyx (). To rule out urologic injury, a CT cystogram was obtained, which revealed no evidence of bladder or urethral rupture. An urgent irrigation and debridement with surgical fixation of his left open ulnar shaft fracture were performed. Great care was taken to monitor the patient’s skin condition beneath the binder to assure he did not experience any areas of skin necrosis or compromise. 2 days after hospital admission, he underwent open reduction internal fixation of the pubic diastasis using a pelvic reduction clamp and a 6-hole non-locking symphyseal plate by Pfannenstiel approach (). During reduction and fixation of the anterior pelvis, intraoperative fluoroscopic assessment revealed increased widening of the midline longitudinal sacral fracture. Due to the fracture’s involvement of the posterior sacral elements and subsequent axial plane instability, fixation of the sacral fracture was deemed necessary to prevent further displacement and potential symptomatic deformity. Under fluoroscopic guidance, transsacral-transiliac guide pins were then used to insert 7.3mm fully threaded screws into the S1 and S2 body, measuring 150 mm and 140 mm, respectively. The screws were inserted posterior to the iliaccortical density so as not to injure the L5 nerve root. Using lag-screw fixation technique principles, these screws closed down the midline sacral fracture diastasis and anatomic reduction was achieved. Postoperatively, the patient was mobilized to weight bear for transfers. At 10weeks, he began weight-bearing as tolerated. The patient developed post-operative urinary retention and a Foleycatheter remained in place on hospital discharge. The urology team followed him as an outpatient and the catheter was removed at 1month, at which time he was able to pass urine normally. The patient received routine follow-up evaluations. Sacral fracture union was evident at 5 months with only mild loss of reduction at the pubic symphysis (1.3 cm of diastasis) (). He had persistent impotence with otherwise normal urologic function and no neurologic deficits.
A 52-year-old female patient with no obvious abdominal symptoms was admitted for liver mass found by ultrasound examination. A hypoechoic solid mass ranged 5.7 * 5.6 * 5.0 cm was detected in the left lobe, with a clear outline and abundant blood flow inside the tumor. Physical examination showed no jaundice in the skin and sclera, liver palms, and any spider angioma. The abdomen was flat and soft, with no varicose veins. No tenderness or rebound pain was induced by palpation. All investigations of chest and limbs were normal. Laboratory tests showed normal blood cell count and liver functions except a slightly increase of total bilirubin to 21.3 μmol/L. Liver tumor related cell markers, including alpha feto-protein, carcino-embryonic antigen, and carbohydrate antigen 199 were all within normal values. Hepatitis serological tests were all negative. No family or personal history of malignancy disease either.\nBoth CT and MRI examinations revealed the mass in the IVth segment of the liver with a multi-lobular appearance, clear boundary, and internal separations. The solid mass can be enhanced in the CT scan from 20Hu to 30Hu. Long T1 and T2 signal were showed in MRI, with only a weak strengthening in the delayed phase, suggesting the high probability of a benign hepatic tumor (Fig. ).\nPreoperative biopsy was suggested but denied by the patient and an open abdominal surgery was performed to exclude any risk of malignancy. The tumor was identified on the diaphragm side of the liver, with a size of 6 * 5 * 5 cm and white-yellow color, in an exophytic growth pattern (Fig. ).\nThe tumor was resected, and no intraoperative frozen sections were taken because the mass was recognized as a benign lesion. A smooth capsule with a clear boundary can be visualized outside the solid-cystic cortex, containing yellow colored jelly-like substance in the central portion with white fibrous septa (Fig. ).\nMicroscopy findings showed that the tumor was composed with different proportions of loose fibroelastic connective tissue, hypocellular mucus-containing stroma, and scattered bile duct epithelium, associated with partial hemorrhage (Fig. ).\nImmunohistochemistry (IHC) showed staining of Vimentin (Fig. ), SMA, CK, CK7, CD34, < 2% Ki67, but no CD117 and S− 100. Pathological diagnosis was collectively determined as the hepatic mesenchymal hamartoma by three pathological experts. According to AJCC classification, this mass belongs to T2bN0M0/IIb. Based on FNCLCC system, the hepatic tumor belongs to Grade 1 and the nodules of omentum metastasis belongs to Grade 2.\nThree years after the surgery, the patient was readmitted because of massive ascites. Severe peritoneal effusions and omental thickening were detected by both ultrasound and enhanced CT scan. An ultrasound-guided percutaneous biopsy of the omentum was performed and followed by H.E. staining. It was found that spindle-shaped cells were distributed in a bundled or interlaced pattern with a relative high density. Multi-sized nuclei were slightly enlarged and eosinohilic cytoplasms were transparent but lack clear boundaries with surrounding myxoid stroma (Fig. ).\nIHC staining of the biopsy specimen showed positive of vimentin, calretinin, and D2–40, < 5% Ki67, but weak for CK and negative for CK7, CK20, SMA, CEA, CA125, GLUT-1, WT1, CD34, and S− 100. Combination of the clinical progress with histopathological findings indicated that these metastatic lesions were developed from a malignant tumor with a mesenchymal origin, thus considered as low-grade MFS. Pathological consultation was carried out and a historical review of the original liver tumor slices indicated a high homology between these two lesions. The second onset was therefore considered as peritoneal metastasis of the primary liver tumor. This patient passed away 4 months later due to multiple organ failure.
A 69-year-old man presented a 3-month history of persistent dull pain in the left lower abdomen. The patient experienced occasional diarrhea, slightly black stools, a poor appetite, fatigue, and 20-lb weight loss. There was no nausea, vomiting, fever, or night sweats. The patient denied any personal or family history of cancer. Before being transferred to our hospital, the patient underwent a gastrointestinal endoscopy that showed no abnormalities. Abdominal CT revealed a large, irregular mass in the pelvic cavity () that was considered as a intestine diverticulum with an abscess. He was suspected to have an inflammation and was treated with a two-week course of antibiotics in another medical facility. However, the antibiotics did not relieve his symptoms. His hemoglobin level was 111 g/L (standard 135–170 g/L), and occult blood (OB) test was positive. Biochemical infection screening and tumor markers (CEA, AFP, CA199, CA724, CA125) were all normal on admission. On physical examination, he was found to have a well-defined soft mass on the left lower abdomen, poor mobility, slight tenderness. Ultrasonography revealed a 6.1 3.8 × 4.2 cm irregular, heterogeneous hypoechoic mass in the left lower abdomen (\n). The tumor contained multiple necrotic areas and close contact with the bladder wall. Heterogeneous enhancement, and wash-out time of 54 s on CEUS ().\nThe PET–CT was performed revealing a highly metabolic mass in the lower abdomen and multiple metabolic nodules in the mesentery (). This was considered a small intestinal stromal tumor with lymph node metastasis. The patient did not show symptoms of lymphoma, and no abnormalities were found in other organs. The consensus of the attending radiologists and surgeons was that it was a malignant tumor rather than an inflammatory process. A core needle biopsy was rejected because of the broad area of necrotic tissue; there was also concern that adequate tumor tissue would not be obtained while risking intestinal perforation or tumor dissemination. Finally the patient underwent surgery to remove the lesion. A 5× 6 cm brown cauliflower-like mass was resected from the ileum, 50 cm away from the ileocecal junction and the surrounding lymph nodes. This mass invaded the serosal layer at the inferior portion of the bladder.\nGross pathology showed the tumor to be friable and having multiple ulcerations on the surface (). H&E sections revealed a small, blue, round tumor (). Histopathological examination showed positive CD99, CK (pan), Ki67 (70%+), Fil-1, and CD34 levels (). Molecular analysis revealed positive EWSR1 fusion gene transcripts, as shown by RT-PCR (). Based on morphology and immunohistochemistry, the tumor was diagnosed as EES/PNET. The patient refused to receive chemotherapy after surgery. He was requested to come to the hospital for examination every month for the first half year, but he did not follow the advice. He came to the hospital because a soybean-like mass was on his forehead, and denied any other symptoms. An MRI was performed revealing a 2.3 × 2.1 × 2.3 cm lesion on the right side of the frontal bone (). The mass extended to the skull and invaded the brain tissue. A second surgery was performed to remove the lesion and adjacent erosive bone. Post-operative pathologic diagnosis revealed the same histology, i.e. the mass metastasized from the primary small intestinal tumor. We performed a telephone follow-up with this patient every two months but learned that he did not undergo any further treatment after the second surgery and died 6 months later; there was no more information about his death.
A 24-year-old Egyptian female presents after a syncopal fall and head trauma. She reports having multiple falls for the past month prior to admission. Upon evaluation, the patient reported neurological symptoms including left-sided weakness in the upper and lower extremities, diplopia, and ataxia along with dysphagia. Most pertinent neurological exam findings were left abducens (CN VI) palsy, bilateral upper and lower extremity weakness of 2/5, and inability to stand due to pain with a 1 cm palpable left soft tissue pelvic mass.\nInitial lab work revealed an elevated lactate dehydrogenase 375 U/L. The CBC and CMP findings were otherwise normal. Previous imaging from Egypt showed a fracture of her mandible with bilateral plate placement on X-ray. On our imaging, CT brain and maxillofacial showed several soft tissue masses present with contiguous bony erosion and destruction involving the central skull base and mandible (). At the skull base, the soft tissue mass eroded the basisphenoid and bordering maxilla, as well as the left greater wing of the sphenoid into the middle cranial fossa with infiltration of the left lateral rectus in the left orbit. CT of the spine showed paraspinal and epidural masses with bordering vertebral erosion (). CT of the chest, abdomen, and pelvis with contrast showed multiple lesions, with soft tissue infiltrating the chest wall, and left hemipelvis straddling the sacrum to involve the left gluteus musculature. These were superimposed on diffusely lytic lesions throughout thoracolumbar spine, some associated with mild compression fractures. MRI of the brain, cervical, thoracic, and sacral spine showed decreased T1 and T2 along with hyperintensive signals on diffusion of the calvarium, skull base, and facial bones with lytic lesions. Large expansile lesion involves L lateral orbital bony margin and zygoma 11 mm × 5 mm × 1.2 cm concerning for neoplasm. No new ischemic changes were observed in the brain. Cervical spine shows irregular T1 enhancement in clivus, C1, and C2, diffusely throughout C3-T1, and there was no central canal or foraminal compromise. Thoracic spine with extensive metastatic lesions with multiple compressions most pronounced at T4, T6, T8, and T11 and leptomeningeal involvement. Lumbar spine showing compression upon the cauda equina at the levels of L3-L4 and L4-L5 soft tissue in the left L3-L4, L4-L5, and L5-S1 neural foramina, encasing/compressing the left L3, L4, and L5 nerve root and bulky bilateral sacral masses.\nAt the time of initial evaluation, the main differential diagnosis was metastatic solid tumor malignancy, atypical neuroblastomas, and lymphoma. Decadron 4 mg every 6 hours was given for empiric treatment to reduce paraspinal inflammation. A fine needle aspiration/biopsy was done via CT guidance of a soft tissue sternal mass.\nFlow cytometry on the biopsied specimen revealed an immunophenotype of monotypic plasma cells (11% of cells) positive for cytoplasmic lambda, CD38, CD56, dim CD45, and CD117 and negative for CD19 and CD20 with morphology showing atypical plasma cells with high nucleus-to-cytoplasm ratio, eccentric and irregular nucleuses, and frequent mitosis (Figures and ). Cytopathology immunohistochemical (CD20, CD117, cyclinD1, c-MYC, and p53) and in situ hybridization (EBR) stains showed neoplastic cells positive for c-MYC, CD56 and CD117 and negative for CD19, CD 20, cyclinD1, p53, and EBER. FISH using probe and location MYC (8q24) was negative for MYC rearrangement. A full multiple myeloma workup was then pursued. Serum free light chain analysis showed kappa light chain of 0.04 mg/dL, lambda light chain of 213 mg/dL, high IgG levels of 1840 mg/dL with an IgG lambda monoclonal band on urine and serum immunofixation, and low beta-2 microglobulin 0.5 mg/L.\nAccording to the 2016 International Myeloma Working Group criteria, this patient met criteria for multiple myeloma with a biopsy proving soft tissue extramedullary plasmacytoma and multiple myeloma defining events with osteolytic lesions shown on imaging and a free light lambda chain ratio >100 mg/L and elevated LDH. The patient was diagnosed with stage III plasma cell IgG lambda multiple myeloma [, ]. The patient clinically had acute disease progression and severely worsening neurological symptoms; a bone marrow biopsy was deferred. A lumbar puncture was done and showed no malignant plasma cells in the CSF. She received palliative radiation therapy to the skull base, thoracic spine, and sacrum, and systemic treatment was started.\nCyclophosphamide 600 mg/m2, bortezomib 1.3 mg/m2, and dexamethasone 40 mg (CyBorD) regimen was initially given [, ]. An attenuated dose of cyclophosphamide was used with an attempt to quickly debulk the disease. Unfortunately, the disease continued to progress, the patient had worsening jaw pain, within 18 days after starting CyBorD. Repeat CT of the maxillofacial area revealed enlargement of the left masticator space lesion. Her treatment regimen was changed to second-line therapy with dexamethasone 40 mg, cyclophosphamide 400 mg/m2, etoposide 40 mg/m2, and cisplatin 10 mg/m2(DCEP) []. Seven days later, with symptoms of dysphagia and lower extremity weakness, the decision was made to add daratumumab 16 mg/kg for primary refractory myeloma [, ].\nAfter addition of daratumumab, she began to clinically improve. Subsequent CT of the maxillofacial showed a decrease in previously seen masticator space masses (). Furthermore, there were decreased size paraspinal and epidural lesions on CT lumbar and thoracic spine (). The Chest, abdomen, and pelvis CT scan also demonstrated substantial interval decreased size of right chest wall and left hemisacrum soft tissue masses. Based on the International Myeloma Workshop Group criteria [], the patient had a partial response to one dose of daratumumab, with CT imaging showing ≥50% reduction in size of soft tissue plasmacytoma and marked reduction in lambda light chain levels from 213 to 3.02 mg/dL. The main toxicity during the hospital course was neutropenia with fever and a nadir ANC 500. Moreover, repeat MRI brain and spine 17 days later significantly decrease in enhancing soft tissue in the lumbar extradural space, with the resolution of compression upon the cauda equina at the levels of L3-L4 and L4-L5 as described above. Persistent soft tissue in the left L3-L4, L4-L5, and L5-S1 neural foramina, encasing/compressing the left L3, L4, and L5 nerve roots was found. Slight improvement of large bulky bilateral sacral masses was observed, which extends to the left presacral and left sciatic foramen regions. Persistent obliteration of the left sacral foramina was observed. Stable mild superior endplate pathologic compression deformities at T12, L4 and L5 were also observed. In the brain MRI, the bony lesions overall appears smaller in size and less bulky, most consistent with a favorable response to treatment. No evidence for brain parenchymal metastatic disease, acute infarct, or acute hemorrhage. A repeat flow cytometry was done 30 days into her admission of her left iliac, but cytopathology analysis was done and showed from the core biopsy with paucicellular and a few plasma cells and rare plasmacytoid cells of uncertain lineage (erythroid versus plasma cellular), amidst scattered bone marrow elements, fibrous tissue, and abundant blood. Immunostains were performed and show rare CD138 staining plasma cells, abundant CD3 staining T cells. Kappa and lambda are noncontributory due to depletion of the tissue upon deeper sectioning. Cam 5.2 is negative and excludes any epithelial cells. She continued to improve, and her ANC rose above 1000 over the next few days. She began moving her lower extremities and was able to walk with crutches at the time of discharge.
A 29-year-old man presented with left upper eyelid swelling, which gradually increased in size over a few weeks before his first visit to the hospital. This non-tender swelling occurred 2 months before the first visit to the hospital for no conspicuous reason. The initial CT and MRI revealed a soft tissue mass (2.7×1.7 cm2) with lobulated margin in the left lacrimal fossa (). Mass adhesion to the superior and lateral rectus muscle was suspected, and an irregular bony erosion on the adjacent lateral orbital rim was observed. Suspecting the possibilities of benign or malignant tumor, the patient underwent lacrimal mass excision and partial orbitectomy of the eroded lateral orbital wall while preserving the surrounding soft tissue for both diagnosis and treatment. The tumor already invaded the orbit intraoperatively; and upon biopsy, the patient was diagnosed with solid-type ACC in the lacrimal gland. Per patient’s request, he was transferred to our hospital for further treatment.\nThe patient’s follow-up CT images after the first operation showed a diffuse and enhanced soft tissue mass remaining in the superolateral orbit, extending to the superior orbital fissure without intracranial extension (). Decided by the ophthalmological department, the patient underwent radical tumor excision including exenteration of the globe and orbitectomy of the lateral orbital wall with safety margin cleared under frozen biopsies. The patient received 6,400 cGy of adjuvant radiotherapy postoperatively. No evidence of residual or recurrent tumor was observed on the patient’s postoperative orbit CT and MRI scans at 4 and 8 months postoperatively.\nEleven months after the second surgery, on the regular follow-up appointment, the patient complained of swelling in his left forehead. MRI and CT scans showed an enhancement of the orbital apex, dural thickening in the right frontal to the left frontotemporal convexity and anterior falx, and suspicious tumor invasion in the anterior superior sagittal sinus without abnormal findings in the brain parenchyma and cerebrospinal fluid space (). Furthermore, lateral sphenoid bone involvement was observed. Considering the patient’s medical history, recurrence of ACC was suspected and was confirmed by an incisional biopsy.\nPer the multidisciplinary committee’s decision, the patient underwent a third surgery, a second radical excision of the tumor including frontotemporoparietal craniotomy, and excision of the dura with tumor firmly attached on its surface (). The soft tissue around the orbital apex, surrounding the skin of the patient’s left forehead and upper and lower lids, were excised, and an additional orbitectomy including total resection of the greater wing of the sphenoid bone was performed. After securing a clear margin with frozen biopsies, duraplasty and cranioplasty with a titanium mesh plate were performed. Anterolateral thigh chimeric flap containing a portion of the vastus lateralis muscle was used for reconstruction. The vastus lateralis muscle was used to fill the space around the orbital apex. No specific postoperative complications were observed (). An additional 4,000 cGy of radiotherapy was administered 1 month postoperatively.\nRegular follow-up imaging studies showed no residual tumor until 1 year after the third surgery, where MRI scan showed enhanced nodular mass lesion in the anterior falx and right temporal convexity (, ). Multiple pulmonary nodules (measuring up to 4 cm) on both hemithoraces were also observed in the patient’s follow-up CT scans. Therefore, the patient was diagnosed with tumor recurrence and metastasis. Debulking surgery was performed including excision of the brain tumor and wedge resection of the lung.\nTwo and a half years after the first recurrence of the tumor, the patient is currently undergoing palliative care including chemotherapy with vinorelbine, methotrexate, and bleomycin and has underwent an additional debulking surgery of the brain tumor as symptomatic treatment for headache and seizures.
A 52-year-old male was brought to the trauma center after a high speed motor cycle collision. The patient was not wearing a helmet and had altered mental status on the scene requiring emergent intubation with a King laryngeal tube (Ambu Inc., MD, USA). On arrival at the trauma center his airway was secured with an endotracheal tube and a cervical collar was placed. He had bilateral breath sounds and his oxygen saturation was 100%. His distal pulses were 2+, blood pressure 98/60 mm Hg and pulse rate was 89 beats per minute (bpm). He had a negative focused abdominal examination for trauma (FAST) exam. His Glasgow Coma Score (GCS) was three with pupils two mm bilaterally and reactive. On log roll there was no rectal tone, but the patient had received succinylcholine 100 milligram (mg) for the endotracheal intubation. A foley catheter was placed and no hematuria noted, his pelvis was stable. The patient’s repeat blood pressure was 70/49 mmHg and pulse 85 bpm with a weak thready radial pulse and warm extremities. He was given two units of packed red cells and two units of fresh frozen plasma. His repeat blood pressure was 79/62 mm Hg as measured by a right femoral arterial line. A massive transfusion protocol was initiated with the patient receiving another five units of packed red cells, four units of fresh frozen plasma, one pack of platelets, and one gram of Tranexamic acid. Simultaneously the right femoral arterial line was exchanged for the 7 French introducer catheter and connected to the arterial line with good waveform. The REBOA catheter was placed at 42 centimeter (cm) into zone one. Inflation was done with 2 milliliters (ml), then 4 ml, then 6 ml of saline for a total of 6 ml of saline with improvement in the blood pressure to 112/62 mm Hg. The REBOA catheter was locked and sutured in place. His abdominal X-ray (Figure ) showed good placement and the catheter was placed to continuous invasive monitoring. His repeat FAST and chest X-ray were negative for intrathoracic effusion or intraabdominal free fluid. Norepinephrine was added for suspected spinal shock given his warm extremities with dilated peripheral veins. Prior to leaving the trauma resuscitation area his systole blood pressure was 125 mm Hg and went up to 195 mm Hg on arrival at the CT scan suite. The REBOA balloon was deflated slowly 30 minutes after inflation and prior to the completion of computed tomography (CT) scans. He was also weaned off the norepinephrine prior to the completion of CT scans, and 0.6 mg of atropine was given for bradycardia in the 40s. He was brought to the intensive care unit for further resuscitation. The patient sustained diffuse axonal injury and subarachnoid hemorrhage. The patient's post resuscitation GCS was 11T. The patient had neurogenic shock secondary to C5 level comminuted fractures involving lateral masses and lamina bilaterally, C6 level comminuted distracted fracture of the spinous process, C7 level fracture of the left lateral mass and left lamina and 5 mm anterolisthesis of C5 on C6 with cord contusion, facet joints causing a perched right C5 facet. The patient also had T1, T2, T3, T5, T7, T8, and T9 level vertebral body fractures and contusion. The patient also had bilateral mandible fracture, avulsion of the right earlobe, and right first and second ribs fracture.\nThe neurogenic shock resolved and the REBOA catheter was removed 78 minutes after initial placement. The patient did not require further transfusion of blood products or vasopressors. Lovenox for venous thromboembolism prophylaxis was started on hospital day 2. The patient underwent stabilization of his cervical spine and fixation of his mandibular fractures, and reattachment of the right earlobe. At the six-month follow-up the patient was independent of the ventilator, masticated with assistance, and was participating in spinal cord injury rehabilitation.
A 62-year-old Indian male who presented to our clinic for evaluation of a persistent intraoral lesion. The lesion progressively increased in size for 9 months and became painful. Patient denied any weight loss, dysphagia, or facial swelling. He has no significant medical or surgical history. He chewed betel quid for twenty years but quit seven years ago.\nOn physical examination, his neck was soft and non-tender with a mobile 2-cm node at the left submandibular region. Intraoral assessment confirmed a tongue with full range of motion and a 3 × 3 cm exophytic ulcerative lesion of the left buccal mucosa with no bony invasion (). The CT neck demonstrated hyperdensity along the left buccal mucosa ridge measuring 2.3 × 1.9 × 2.3 cm as well as a centrally necrotic ipsilateral lymph node at level 1B (). The CT chest was unremarkable and the CTA of lower extremities showed severe peripheral vascular disease. The histological study of the incisional biopsy revealed invasive keratinizing moderately differentiated squamous cell carcinoma. Finally, the case was examined by the institutional tumor board and classified as a cT4aN1M0. Based on findings, the tumor board recommended surgery as primary treatment. Accordingly, three-dimensional (3D) printed models, a pre-bent reconstruction plate and surgical cutting guides for the mandible resection were obtained prior to the procedure.\nThe surgery began with a standardized tracheostomy. Because an 8-cm skin defect at the left cheek was planned as part of the oncological resection, the surgery continued with the elevation of a cervicothoracic rotation flap. We utilized a medially based skin flap. The incision was made from the margin of the cheek skin resection (near to the left commissure) toward the tragus. Then, the incision continued a preauricular trajectory followed by a course around the ear lobe. Subsequently, the incision extended downward just anterior to the hairline and then along the anterior edge of the trapezius muscle and the deltopectoral groove. At the anterior border of the axilla, the incision turned inward in 90 degrees (back-cut) and continued in a medial direction, above the areola, until the lateral border of the sternum (). The flap was elevated in a sub-SMAS, subplatysmal and subfascial approach, respectively (). The left modified radical neck dissection was then performed including the levels 3–5 of lymphoareolar tissues. The external jugular vein was identified and ligated. Next, the anterior mandibular osteotomy was performed using reciprocating saw and cutting guide. Afterward, the mandible was displaced laterally to allow the dissection of the tumor and preserve 1.5-cm safety margins from surrounding mucosa. The left portion of the floor of the mouth, the exophytic lesion, the nearby buccal mucosa and muscular plane as well as the overlying cheek skin were all excised and incorporated to the specimen (). Subsequently, the distal mandibular osteotomy was carried out with reciprocating saw from the mandibular notch (to include the coronoid process), along the ramus, up to the inferior border of the mandible. The insertion of the temporal muscle was stripped off from the coronoid process to complete the release of the specimen (). Finally, extraction of all remaining teeth was performed.\nSimultaneously, a second surgical team harvested a left osteocutaneous radial forearm free flap. Briefly, centered on the radial artery trajectory, a 5 × 8 cm skin paddle was elevated in standard fashion from the medial edge up to the radial side of the flexor carpi radialis (FCR) tendon. The dissection continued downward through the muscle bellies of flexor digitorum superficialis (FDS) and flexor pollicis longus (FPL) until the radius was encountered behind the radial vessels. Thereafter, the lateral edge of the flap was dissected medially to the brachioradialis tendon and protecting the lateral antebrachial cutaneous nerve. Osteotomy of the anterolateral half of the radius with oscillating saw. This step was accomplished without cutting guides. A 9-cm bony segment was incorporated to the flap. The radial artery and its venae comitantes were released up to the antecubital fossa. Once the flap was brought to the left aspect of the face, microsurgical anastomoses were performed under the microscope: end-to-end arterial anastomosis with left facial artery using 9-0 nylon sutures and end-to-end venous anastomosis with facial vein using 2.5-mm coupler. The vascularized segment of radius was subsequently used to reconstruct the mandible with a pre-bent 2.4-mm reconstruction plate and 6-mm screws. The plate was then fixed to the remaining mandible with bicortical screws. Afterward, the intraoral lining was re-established by suturing the skin paddle to the surrounding mucosa with multiple 2-0 vicryl stitches. Finally, the cervicothoracic flap was advanced and rotated to close the cheek defect and sutured in layers with multiple stitches of 2-0 vicryl and 5-0 nylon ().\nAt the left forearm, to prevent stress fractures, a 14-cm reconstruction plate was placed over the bony defect. More specifically, the plate was partially placed over the bony defect and partially over the more lateral aspect of the intact radius. This practice facilitates the coverage of a thick and wide reconstruction plate with the nearby skin flap while maintaining the protection over the radius defect. Following our protocol, the plate length allowed two bicortical screws distally and three bicortical screws proximally to the radius bony defect (). The incision leading to the antecubital fossa was closed over a Blake drain with 3-0 vicryl stitches at the deep dermal layer followed by 4-0 nylon sutures at the skin. Even though peritendons were preserved, we buried exposed tendons inside the surrounding muscles or cover them by advancing the skin edges. The remaining open area (skin paddle harvest) was covered with 0.012-inch split thickness skin graft, meshed at 1:1.5 ratio. We squeezed the meshed skin graft in to almost occlude the holes of the 1:1.5 expansion. This maneuver secured the full coverage of the open area, allowed the interstitial fluid leakage and minimized the resulting scar (). The donor site was protected during the immediate postoperative period with a dorsal splint in 30 degrees of wrist flexion that was reversed with progressive splint adjustments and physical therapy.\nThe postoperative period was satisfactory without complications. Patient was discharged on postoperative day 8 without tracheostomy. Following tumor board’s recommendation, patient underwent external beam radiation therapy. New PET/CT scan was obtained 3 months after completing radiation without evidence of residual or recurrent disease. Fourteen months after surgery, the patient continues an uneventful course, keeps regular cancer screening follow ups, and has healed well. He has adequate mandibular motion, no local pain, no trismus or hardware exposure ().
A 3.5-year-old boy with no relevant medical history was referred to the Pediatric Dentistry Unit of Policlinico Umberto I of Rome. Parents reported that the boy suffered a dental injury from a fall while playing at home. The child did not lose consciousness or vomit after the injury. Once informed consent from his parents was obtained, a medical anamnestic chart was filled, and a clinical exam was performed within 1 h of the trauma. During extraoral examination, no abnormalities of the temporomandibular joint were reported. Soft tissue lacerations with hematoma and a displacement of 3 mm along the axis of the deciduous maxillary right central incisor (5.1) were observed during intraoral examination. These resulted in a diagnosis of extrusion (a). Furthermore, occlusal interference due to extrusion of the incisor prevented the molars from a proper occlusion. A periapical radiograph of the anterior teeth did not show altered periodontal ligament space, but horizontal root fracture was diagnosed (b).\nAccording to the protocols proposed by the IADT [,], the degrees of displacement and mobility, the root formation, the alteration of PDL space, and the child’s ability to deal with an emergency should be considered when planning the treatment. In cases of extrusion equal to or less than 3 mm, the protocol suggests careful repositioning or waiting for spontaneous repositioning. In cases of extrusion more than 4 mm, the tooth should be extracted. Furthermore, for root fracture with increased mobility and displacement, the protocol also suggests placement of orthodontic splinting according to the degree of mobility and compliance of the patient, in addition to periodic follow-up appointments.\nTherefore, in the reported case, a thorough evaluation was carried out, and treatment was planned. The oral cavity was rinsed with 0.12% chlorhexidine solution, and local anesthesia was performed. An orthodontic flexible splint was prepared to be attached to the adjacent unaffected teeth (a); then, the tooth was gently repositioned (b), in order to avoid occlusal interference, and stabilized through the splint (c,d).\nA good prognosis following an oral trauma depends on effective oral hygiene; therefore, parents were instructed on how to take care of the injured tooth and prevent further injury by supervising potentially hazardous activities. They were instructed not to further traumatize the injured tooth when eating; nonetheless, a return to normal function as soon as possible was encouraged. Parents were advised to use a soft brush or cotton swab combined with alcohol-free chlorhexidine gluconate (0.12%) applied topically twice a day, in order to prevent accumulation of debris and to reduce the bacterial load. They were advised about possible complications that might occur, such as increased mobility, swelling, or a sinus tract; children might not complain about pain, but infection might still occur. If that happens, the child should be taken to a dentist for treatment.\nIn the reported case, a follow-up clinical examination was made 1 week after the injury, and the tooth was asymptomatic; there was normal color of the crown and lack of any signs of pulp necrosis and infection. The same follow-up appointment was planned for 4 weeks after the trauma for splint removal, if supported by healing signs. Aspects to be examined when carrying out a splint removal are color, symptoms, and mobility of the tooth, as well as healthy surrounding tissues. In this case, element 5.1 was asymptomatic; realignment of the root-fractured tooth was observed, mobility was physiological, and crown had a normal color. Supporting tissues were clinically healthy, and no signs of pulp necrosis or infection were observed. Thus, the splint was removed, and another follow-up appointment was planned for 8 weeks after the trauma. The same conditions and signs were observed at the subsequent checkups.\nAt the 1-year follow-up appointment, the deciduous tooth showed no sensitivity to percussion nor pulp alterations. Tooth-supporting soft tissue was intact, and no pain or discomfort was reported. The crown showed no signs of alteration in color (a). An anterior periapical radiograph was taken, and early signs leading to pulp canal obliteration (PCO) were present; PCO often occurs after dental injuries and can be associated or not with crown discoloration. No signs of pulp necrosis were present. Another aspect that can be observed in the periapical radiograph (b) is the atypical resorption of the apical fragment, where—even if different from the physiological one–its characteristics are also different from pathologic root resorption.\nFrequent follow-ups were scheduled until the eruption of the permanent teeth; no abnormal changes were observed. After 3 years, when the child was 6.5, the deciduous maxillary right central incisor exfoliated and was physiologically replaced by the permanent right maxillary incisor (element 1.1). In the subsequent checkups, normal development of element 1.1 was observed, with no alteration in color or shape, demonstrating effective management of the trauma. The panoramic view in a shows the follow-up at age 10, while b,c show the clinical view at age 12.
A 73-year-old man with past medical history of stage IV bladder and prostate cancer, status post cystoprostatectomy with ileal conduit, left orchiectomy, and subsequent left radical nephrectomy (for recurrent left hydronephrosis and pyelonephritis), presented with worsening right leg edema and pain for 2–3 weeks which confined him to bedrest. On examination, there was severe pitting edema of the right leg associated with erythema and warmth.\nDuplex ultrasound examination of the lower extremities revealed a nonocclusive thrombus extending from the right external iliac to the central (cranial) segment of the femoral vein and an occlusive thrombus in the central segment of the deep femoral (profunda femoris) vein. A non-contrast CT scan of the abdomen and pelvis revealed an increase in the size of a pelvic retroperitoneal nodal mass inseparable from the right common iliac artery and right ureter, with no clear visualization of the right iliac venous system (Fig. ). The patient was placed on therapeutic low molecular weight heparin in preparation for right lower extremity catheter-directed thrombolysis and stenting.\nPre-procedural ultrasound examination of the access site confirmed findings seen on lower extremity Doppler examination and revealed extension of the venous thrombosis into the femoral and deep femoral vein precluding femoral vein access. The patient was then placed in prone position under general anesthesia and the right popliteal vein was accessed. Venography showed extensive thrombosis and strictures of the right common iliac, right external iliac, and right femoral veins with collateralization (Fig. ). Pharmacomechanical thrombolysis using the Trellis Thrombectomy System (Formerly Covidien, Boston, MA, now discontinued) was performed in the right femoral vein through the popliteal vein access to clear the acute thrombus. Repeat venography showed resolution of the acute DVT in the right femoral and external iliac veins with minimal residual stenosis. There was however no identifiable connection between the right common iliac vein and the inferior vena cava (IVC). Unsuccessful attempts were made using multiple wire/catheter combinations to recanalize the right common iliac vein. Initial attempts to cross the obstruction using an angled catheter in combination with soft and stiff hydrophilic wires were unsuccessful. Furthermore, attempts were made using a crossing Rubicon Support Catheter (Boston Scientific, Marlborough MA) in combination with hydrophilic wires and the stiff (back) end of an Amplatz wire. However, there was inadequate support with this combination. The left popliteal vein was then accessed using a micro puncture set and venography demonstrated a narrow, but patent left common iliac vein and patent IVC without identifiable inflow from the right common iliac vein.\nThe patient was subsequently turned supine. A wire was advanced through a right common femoral artery access into the lower aorta to provide a visual safeguard for preventing arterial injury during sharp venous recanalization. An 18 mm Atlas balloon (Bard, Murray Hill, NJ) placed through a right internal jugular access was inflated across the IVC/left common iliac vein confluence to provide a central target. A 10 French right femoral venous sheath was inserted and the metallic stiffening cannula/catheter combination from a Rösch-Uchida Transjugular Liver Access Set was advanced into the peripheral (caudal) segment of the right common iliac vein stump. The metal cannula was then progressively advanced towards the target balloon in the lower IVC. A 21-gauge Chiba needle was advanced through the cannula (Fig. ). The needle and a 0.018-in. guidewire were used to create a channel through the encasing tumor into the caudal segment of the IVC with one pass (Fig. ).\nAfter gaining access into the patent IVC, balloon angioplasty was performed and self-expanding kissing [16 mm diameter × 90 mm length] Wallstents (Boston Scientific, Marlborough MA) were placed in both common iliac veins. Three additional overlapping stents [one 16 mm × 90 mm Wallstent; two 14 mm × 60 mm S.M.A.R.T stents (Cordes, Fremont, CA)] were placed from the right common iliac to the right common femoral vein followed by balloon angioplasty (Fig. ). Initial post-stenting venography demonstrated thrombosis of the central stents, likely due to inadequate inflow. Pharmacomechanical thrombolysis using the Trellis Thrombectomy System was performed to successfully remove the acute thrombus from the central stents. The stents were extended below the femoral head to ensure adequate inflow using an additional 10 mm × 40 mm EV3 Protégé (Medtronic, Minneapolis MN) stent. Completion venography demonstrated widely patent stents with contrast flowing into the IVC (Fig. ). The catheters were removed, and hemostasis achieved. Over the next several days, there was significant improvement of the pain and swelling and the patient was discharged to hospice care. The patient passed away after 2 months due to progression of the metastatic disease.
A 32-year-old Japanese women, gravida 0, para 0, had been infertile for 3 years. The cause of the infertility had been investigated extensively, but no clear reason was found. Pap smear tests for cervical and endometrial cancers performed at 6 months before the pregnancy were negative. Pregnancy was established by repeated embryonal transfer.\nIrregular vaginal bleeding was sometimes noted even during the first trimester. A Pap smear test performed at 10 gestational weeks was negative for intraepithelial lesions or malignancy (NILM). The patient was hospitalized at 26 gestational weeks due to increasing uterine contractions, genital bleeding, and shortening of the uterine cervix (2.3 cm). Magnesium sulfate and ritodrine chloride were administered, but the disease progressed rapidly. Because of the transverse presentation of the baby and a bulge in the membrane, a cesarean section was performed at 29 gestational weeks. The baby weighed 1124 g with an Apgar score of 4/6. Pathological examination of the placenta showed grade 3 chorioamnionitis.\nThe patient was discharged from the hospital on the7th day after the surgery. At that time, ultrasound showed that the uterus had shrunk to 12-week size and the endometrium was thin. An obstetrical check performed one month postpartum indicated that the uterus was atrophic, and no unusual findings were detected upon pelvic ultrasound ().\nAt 2 months after childbirth, the left abdominal had worsened, so the patient visited us. A pelvic examination revealed multiple pelvic masses and a diffuse vaginal tumor causing stenosis. An ultrasound scan of the pelvis revealed a slightly hyperechogenic uterus of 6-week size, with multiple peritoneal nodules and ascites, while the endometrium was still thin. Pap smear of the uterine cervix showed NILM again.\nA CT scan showed multiple nodules in the lungs and on the peritoneum. The uterus was enlarged and irregularly stained in contrast-enhanced CT (). The pancreas was diffusely swollen with small amount of effusion surrounding it.\nT2-weighted MRI revealed that the endometrium was atrophic, but the myometrium was hypertrophic and the junctional zone had disappeared. The uterine cervix appeared normal. The vaginal thickness was observed in its lower segment. Diffusion-weighted MRI of the uterus and the vagina suggested that the entire uterus might have been replaced by malignant tissue, which had reached deep into the lower segment of the vagina ().\nIn PET-CT, abnormal accumulations of fluorine 18 fluorodeoxyglucose (FDG) were observed throughout the uterus, vagina, and pancreas and parts of the vulva, ovary, bilateral ischial tuberosities, peritoneal nodules, lungs, and mediastinal and pelvic lymph nodes ().\nVaginal tumors were biopsied and subjected to histological examination via hematoxylin and eosin staining. Results showed undifferentiated carcinoma (). Tumors were further investigated by immunostains and fluorescence in situ hybridizations. Percentages of cell staining were recorded: negative (0-5%), 1+ (6-25%), 2+ (26-50%), 3+ (51-75%), and 4+ (>75%). The staining results are summarized in . Despite these results, however, the origin of the cancers remained uncertain. The pathological diagnosis was finally determined as undifferentiated carcinoma of unknown origin with possible adenocarcinoma.\nSerum levels of CA125, CA199, and CEA reached 324.8 IU/ml, 123.2 IU/ml, and 6.0 mg/dl, respectively. Given the well-preserved structure of the uterine cervix, repeated negative cytology from the uterine cervix, and irregularly enlarged uterine body, we finally diagnosed the patient with an endometrial carcinoma of FIGO stage 4b.\nRegarding the abnormal FDG accumulation in the pancreas, hypercalcemia-induced edematous pancreatitis was conceived. Urinastatin administration rapidly diminished serum amylase and lipase, and the pancreatitis was completely cured within a month.\nChemotherapy with paclitaxel and carboplatin was repeatedly performed, but the patient died 6 months after childbirth.
The patient was a 53 year old Caucasian woman, without any previous major medical problems, who was recently diagnosed with synchronous bilateral breast cancers and who underwent a right modified radical mastectomy and a left total mastectomy and left axillary sentinel lymph node biopsy for a pT2, pN1, estrogen receptor positive, progesterone receptor positive, HER-2/neu negative invasive lobular carcinoma of the right breast and a pT1b, pN0, estrogen receptor positive, progesterone receptor positive, HER-2/neu negative invasive ductal carcinoma of the left breast, respectively. The patient was subsequently recommended for placement of a subcutaneous implanted port for administration of postoperative adjuvant systemic chemotherapy.\nTherefore, the patient was taken to the operating room by the surgeon for subcutaneous port placement. At the request of the patient, this procedure was done under general anesthesia. The left side was selected, as it represented the side of her earlier-stage breast cancer. A left cephalic vein cutdown approach was undertaken in the left lateral infraclavicular region, by the methodology as previously described by Povoski []. Upon creating a transverse venotomy in the anterior wall of the left cephalic vein and passing a 9.6 French single lumen silicone catheter centrally, it was noted on real-time intraoperative fluoroscopy of the thoracic region that the 9.6 French single lumen silicone catheter eventually advanced downward in a craniocaudal fashion along the left paramediastinal border. As a result of this finding, intraoperative venography (Figure ) was undertaken by the surgeon in a non-digital subtraction fashion through the 9.6 French single lumen silicone catheter and at several distances from the entry point into the left cephalic vein, as the 9.6 French single lumen silicone catheter was sequentially advanced from approximately the 8 cm mark to the 15 cm mark. A total of approximately 50 milliliters of iohexol injectable contrast (300 mg/mL) was utilized during intraoperative venography. With the tip of the 9.6 French single lumen silicone catheter first positioned in the region of the mid-portion of the left subclavian vein, but at a point at which some resistant to further advancement of the 9.6 French single lumen silicone catheter was noted, intraoperative venography performed through the 9.6 French single lumen silicone catheter (Figure ) revealed a small (3 to 4 mm) venous branch off of the left subclavian vein that was first directed horizontally for approximately 3 to 4 cm and then was re-directed cephalad in a rightward direction across the upper thorax/lower neck region. Just before the transition from the horizontal to cephalad portion of this small (3 to 4 mm) venous branch off of the left subclavian vein, a tiny (1 to 2 mm) venous tributary was seen to originate off of the small (3 to 4 mm) venous branch. This tiny (1 to 2 mm) venous tributary was noted to meander in a generalized horizontal fashion across the midline of the upper thorax region and into the contralateral right hemi-thorax region. Subsequently, after repositioning of the 9.6 French single lumen silicone catheter and overcoming the previous resistence to catheter advancement, and with the tip of the 9.6 French single lumen silicone catheter now positioned more centrally (but still horizontally) in the region of the left subclavian vein (Figure ), and then with further catheter advancement with the tip of the 9.6 French single lumen silicone catheter then positioned even more centrally in a craniocaudal fashion in the upper left paramediastinal border region (Figure ), intraoperative venography revealed the presence of a relatively large diameter craniocaudally-oriented venous structure located to the left side of the midline in the medial left hemi-thorax region in a location adjacent to the cardiomediastinal silhouette and which appeared to eventually drain into the cardiac silhouette. There was absence of visualization of an identifiable left innominate vein on intraoperative venography. This relatively large diameter craniocaudally-oriented venous structure coursing downwards on the left side of the midline in the medial left hemi-thorax region was intraoperatively suspected by the surgeon to represent a PLSVC.\nSame-day consultation with the interventional radiologist revealed a similar opinion. However, based upon the intrinsic limitations of the non-digital subtraction intraoperative venography procedure performed, an accurate assessment of the point of insertion of the PLSVC into the venous return of the heart and the anatomy of the contralateral right-sided central venous system could not be adequately determined. Therefore, subsequent standard digital subtraction venography was recommended by the interventional radiologist.\nThe subcutaneous port placement procedure was uneventfully completed by the surgeon by placing the tip of the 9.6 French single lumen silicone catheter to approximately the 15 cm mark within the recognized PLSVC and attaching the 9.6 French single lumen silicone catheter to an implantable port (Titanium Bard PowerPort, C. R. Bard, Inc., Salt Lake City, UT) and closing the port insertion surgical skin incision site that was located in the left lateral infraclavicular region.\nA subsequent posterioranterior and lateral chest x-ray (Figure ) was performed and demonstrated the implanted left-sided subcutaneous port and the attached 9.6 French single lumen silicone catheter and its course along the medial left hemi-thorax region in a location adjacent to the cardiomediastinal silhouette, consistent with PLSVC.\nIn the subsequent weeks after left-sided subcutaneous port placement, digital subtraction venography of the left-sided central venous system (by way of the left-sided subcutaneous port) (Figure ) and digital subtraction venography of the right upper extremity veins and right-sided central venous system (by way of a peripheral vein in the dorsum of the right hand) (Figure ) were both performed by the interventional radiologist within the interventional radiology suite.\nAt approximately two weeks after left-sided subcutaneous port placement, the patient underwent digital subtraction venography of the left-sided central venous system by way of the left-sided subcutaneous port (Figure ), in order to fully characterize the central venous drainage pathway of the PLSVC (i.e., the point of confluence of the PLSVC with the venous return of the heart). The left-sided subcutaneous port reservoir was accessed in a sterile fashion using an 18-gauge Huber needle. Power injections were performed at 5 mL/second of iodixanol injectable contrast (320 mg/mL), with maximum injection pressure set at 300 PSI. Digital subtraction imaging was performed at 6 frames/second during the power injection. Digital subtraction venography confirmed that the point of confluence of the PLSVC with the venous return of the heart was at the right coronary sinus and into an atrial structure within the cardiac silhouette (Figure ). Delayed digital subtraction images demonstrated that the atrial structure then drained into the right ventricle and subsequently into the pulmonary arteries (Figure ), confirming that the atrial chamber receiving the venous return from the PLSVC was indeed the right atrium. Further later delayed digital subtraction images demonstrated the pulmonary venous return and the filling of the left side of the heart and subsequent aortic outflow (Figure ). There was no evidence of early arterial filling. There was no evidence of right to left shunting on the early images, nor was there evidence of left to right shunting on the delayed images.\nApproximately four weeks later, the patient underwent digital subtraction venography of the right upper extremity veins and the right-sided central venous system by way of a peripheral vein in the dorsum of the right hand (Figure ), in order to fully characterize the right-sided peripheral and central venous anatomy. A vein in the dorsum of the right hand was accessed in a sterile fashion using an 18-gauge angiocatheter. Power injections were performed at 3 mL/second of iodixanol injectable contrast (320 mg/mL), with maximum injection pressure set at 600 PSI. Digital subtraction imaging was performed at 3 frames/second during the power injection. Digital subtraction venography demonstrated normal venous anatomy within the right forearm and right upper arm regions. The more central right-sided veins, including the right axillary vein and right subclavian vein were also normal in appearance. Incidentally, there was partial fenestration of a portion of the right subclavian vein, a commonly encountered venous entity, which is usually of no clinical significance. Her right superior vena cava (SVC) was somewhat smaller in caliber than is usually seen in someone without a co-existing PLSVC. However, her right SVC was approximately of the same size as her PLSVC that was seen on her prior venography imaging. The right SVC venous return to the heart was into the right atrium, and without venographic evidence of right-to-left shunting or left-to-right shunting. The venous flow from the right atrium was identical to that seen during the previous digital subtraction venogram of the left-sided central venous system performed by way of the left-sided subcutaneous port.\nThereafter, the patient was allowed to use her left-sided subcutaneous port for continued administration of postoperative adjuvant systemic chemotherapy, blood draws, and all necessary subsequent contrast-based imaging. The patient had no detectable problems during the utilization of her left-sided subcutaneous port and had no resultant complications. The patient's left-sided subcutaneous port was eventually removed after she completed her postoperative adjuvant systemic chemotherapy, some seven months after its original placement.
A 53-year-old female presented with an asymptomatic gastric tumor found incidentally during screening upper gastrointestinal endoscopy. The lesion appeared as a flat protrusion in the lesser curvature of the lower third of the stomach (Fig. ). Endoscopic ultrasound (EUS) showed a 23 × 12-mm, hypovascular, heterogeneous lesion thickening the second and third gastric layers. The findings strongly suggested scirrhous gastric cancer (Fig. ). Although the lesion biopsy revealed no evidence of malignancy, our suspicion of scirrhous gastric carcinoma persisted due to the configuration of the tumor and our EUS findings. We performed an endoscopic open biopsy to make an accurate diagnosis. However, evaluation of the specimen revealed only slight chronic inflammatory cell invasion. Computed tomography (CT) scanning showed only gastric wall thickening at the angle of the stomach (Fig. ). Positron emission tomography-CT showed no evidence of metastasis or abnormal uptake in the tumor (Fig. ). The laboratory findings were normal as were levels of tumor markers (CEA, 1.6 ng/ml; CA19-9, 12 U/ml).\nIn spite of these findings, given our high level of suspicion, we decided to perform surgery to obtain a definitive diagnosis and treat the tumor. We chose gastric wedge resection using a combined laparoscopic and endoscopic method for several reasons. Firstly, the biopsy did not show cancer, but the tumor configuration strongly suggested malignancy; therefore, we selected a non-exposed method to prevent interoperative dissemination of tumor cells. Secondly, the tumor appeared to be a submucosal tumor (SMT), and gastric wedge resection using a combined laparoscopic and endoscopic method is among the safest procedures for resection of gastric SMTs [, ]. Lastly, we chose a wedge resection because if the tumor was not malignant, a distal gastrectomy could be considered excessive. We obtained an interoperative pathological diagnosis, and we planned to perform partial gastric resection in the absence of malignancy and laparoscopic distal gastrectomy with lymph node dissection if cancer was identified.\nThe first port was inserted through the umbilicus using an open technique. Four additional ports were inserted: the second in the subcoastal arch, the third at the mid-point between the camera port and the second port, and the fourth port and fifth port symmetrically. The first, second, and third ports were 12 mm. The fourth and fifth ports were 5 mm.\nThe lesser curvature gastric tumor was easily recognized laparoscopically. Our intraabdominal examination revealed a scar-like tumor with twitch. After preparation of lesser curvature vessels, the tumor periphery was viewed endoscopically. The entire circumference of the tumor was marked to ensure an approximately 0.5-cm margin from the tumor edge (Fig. ). We then injected indigo carmine into the gastric submucosal layer with an endoscopic needle. The seromuscular dissection was performed using a laparoscopic electrocautery scalpel (Fig. ). The specimen was pulled up along with the surrounding mucosa. A full-layer resection including the specimen was achieved using a laparoscopic stapling device (Fig. ). We used a hand-sewn technique for seromuscular suturing so the staple line would not be exposed (Fig. ). Finally, we inserted the endoscope into the duodenum to ensure that there was no gastric stenosis.\nThe patient had gastric hypoperistalsis on postoperative day (POD) 1. We inserted a gastric tube; however, we planned for an extended period with no oral intake in case of persistent hypoperistalsis. On POD 4, a W-elemental diet (W-ED) tube was inserted to drain the stomach and provide nutrition. We started the patient on continuous low-dose erythromycin, mosapride citrate, and Rikkunshi-Tou to treat her hypoperistalsis. The W-ED tube was removed on POD 11 after an oral contrast study confirmed gastric motility. On the same day, oral intake was initiated. The patient was discharged from the hospital on POD 18.\nThe submucosa tumor could be seen in the resected specimen. The tumor had 20 mm × 20 mm × 13 mm size, including spindle cells with myxoid changes and collagen fibers. There was no evidence of adenocarcinoma. In this findings, fibromatosis can be a differential diagnosis.\nSpindle tumor cells were identified in the gastric wall from the submucosa to the serosa accompanied by myxoid changes and collagen fibers in the stroma (Fig. and ). The spindle cells were strongly immunopositive for alpha-smooth muscle actin, and anaplastic lymphoma kinase (ALK), but immunonegative for c-kit, desmin, and S-100. CD34, bcl-2, beta-catenin, CD31, pankeratin, platelet-derived growth factor-A, and DOG1 were almost immunonegative (Fig. and ). Based on these findings, the tumor was diagnosed as gastric IMT.
A 77-year-old woman underwent an annual medical checkup and was diagnosed with a cystic tumor in the pancreas by computed tomography (CT) of the abdomen. The patient was referred to our hospital for further examination. Her medical history included a radical parotidectomy for a parotid gland tumor and a total knee replacement for the right leg. In addition, she was receiving treatment for hypertension and osteoporosis. On admission, she had no clinical symptoms. Her height was 154 cm and her body weight was 61 kg. There was no superficial lymphadenopathy or palpable mass in the abdomen. Her serum amylase level was 211 U/L (normal range; 30–120 U/L), and other biochemical data, including tumor marker levels, fasting plasma glucose, and hemoglobin A1c, were within normal ranges. An upper gastrointestinal endoscopy showed esophageal hiatal hernia and short-segment Barrett’s esophagus. Colonoscopy showed diverticula in the sigmoid colon. Contrast-enhanced abdominal CT scanning demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst (Fig. ). Magnetic resonance cholangiopancreatography (MRCP) revealed a cystic tumor in the head of the pancreas along with a normal, non-dilated dorsal pancreatic duct throughout the pancreas (Fig. ). The presence of a connection between the cystic lesion and the main pancreatic duct was unclear. Endoscopic retrograde pancreatography (ERP) via the major duodenal papilla showed a cystic tumor and a slightly dilated main pancreatic duct, but the main pancreatic duct was abruptly interrupted at the head of the pancreas (Fig. ). The major duodenal papilla was enlarged and the orifice was filled with abundant mucin (Fig. ). The minor duodenal papilla was normal in size and ERP via the minor papilla was not possible. The diagnosis based on pancreatic juice cytology was “highly suspicious for adenocarcinoma,” suggestive of an intraductal papillary mucinous carcinoma (IPMC) arising in the ventral pancreas of pancreas divisum. The patient underwent a pylorus-preserving pancreaticoduodenectomy (PPPD) with regional lymphadenectomy. The postoperative course was uneventful, except for a Grade A pancreatic fistula (staged according to the International Study Group on Pancreatic Fistula clinical criteria []), and the patient was discharged on postoperative day 29.\nA pancreatography via the major and minor duodenal papillae on the surgical specimen revealed no connection between the ventral and dorsal pancreatic duct systems (Fig. ). Macroscopically, a multilocular cystic tumor, 40 × 35 × 25 mm in size, with abundant accumulation of mucin was identified in the ventral pancreas. Microscopically, the tumor was composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (Fig. ). They formed prominent papillary structures. The Mib-1 index was up to 80 %. The tumor cells slightly progressed into the main pancreatic duct. Finally, the tumor was determined to be a mixed type IPMC (well-differentiated adenocarcinoma) with partial microinvasion. The pancreas bearing the tumor was drained by the pancreatic duct, which opened into the major papilla, suggesting that it was the ventral pancreas. The region surrounding the IPMC was mainly composed of fibrous tissue, which was clearly distinguished from the normal pancreas (Fig. ). The normal pancreas was relatively rich in adipose tissue and the islets of Langerhans were typically oval in shape, features consistent with the dorsal pancreas. Furthermore, the pancreatic duct in the region of the normal pancreas was linked to the minor papilla, also suggesting a dorsal pancreas origin.
A 27-year-old lady at 33 weeks of gestation, with a history of normal pregnancy and an abortion at 32 weeks' gestation without apparent cause. The patient did not have family history of complications of megacolon; pregnancy was going on with medical monitoring and had no abnormalities.\nShe was admitted to the emergency room with complains of abdominal pain over the last three days, with progressive worsening despite the use of antispasmodics. On physical examination, the patient was confused, dehydrated, with severe respiratory distress, fever, reduced peripheral perfusion, and hypotension. The abdomen showed asymmetric distention more prominent on left flanks. Bowel sounds were absent. During the obstetric examination, it was not possible to detect fetal movements and fetal heart sounds were absent, suggesting intrauterine fetal death. On vaginal examination, the cervix was located in a mid-posterior position, without dilatation. Routine laboratory examination results were normal except for an elevated white blood cell count of 18.1 × 103/μL. Abdominal radiographs revealed an abnormal gas pattern, with a dilated colon in the upper abdomen and air fluid levels. The colon showed significant dilation with the image in “horseshoe” suggesting the presence of SV. There were no signs of pneumoperitoneum. Chest radiography showed a significant elevation of the diaphragm, restricting pulmonary function (). Ultrasonography of the abdomen and pelvis confirmed fetal death and showed the presence of moderate amounts of free fluid in the abdominal cavity.\nShe was submitted to initial resuscitation with IV fluids, nasogastric suction, and bladder tube. The urinary catheter showed no urine output even after replacement of 2 L of saline. Despite having been kept since admission with oxygen mask, the patient developed an acute respiratory distress syndrome that required orotracheal intubation and mechanical ventilation. After initial resuscitation, with suspected bowel obstruction complicated by SV and intrauterine fetal death, the patient was taken emergently for exploratory laparotomy under general anesthesia. The abdominal cavity was accessed by laparotomy and after opening the peritoneum it was noted leaking from hemorrhagic, lots of foul smell and intense distension of loops of small intestine and colon. At laparotomy, an enormously distended sigmoid loop, with gangrenous changes extending from the transverse to extraperitoneal rectum, was found. The necrosis of the colon resulted from the presence of an SV, due to double twist on sigmoid mesocolon. The necrotic colon was posteriorly displaced by the pregnant uterus, without signs of perforation (Figures and ).\nAs there was no response to induction via vaginal labor and the patient conditions were critical, it was decided to carry out a concomitnt cesarean section. However, after removal of the dead fetus, the patient showed a uterine atony with severe bleeding, about 800 mL of blood, and it was refractory to standard clinical measures. Unfortunately, she required a concomitant total abdominal hysterectomy.\nFaced with irreversible colon necrosis, resection of sigmoid colon and descending colon and proximal colostomy was performed. The closure of the rectal stump, below the peritoneal reflection, was performed with mechanical suture. As there was no response to induction via vaginal labor and the patient conditions were critical, it was decided to carry out a postmortem cesarean section. However, after removal of the dead fetus, the patient showed a uterine atony by severe bleeding, about 800 mL of blood, and it was refractory to standard clinical measures. Unfortunately, she required a concomitant total abdominal hysterectomy. After cesarean section, the peritoneal cavity was washing with saline and the abdominal wall was closing by planes. As the patient remained shocked and with respiratory distress syndrome during the surgical procedure, and she had received 4 UI of the plasma and 1 UI of the red blood cell concentration, the postoperative was followed at unit of intensive care. She remained for four days on mechanical ventilation and broad-spectrum antibiotic therapy. The renal and respiratory failure was improving gradually being transferred on the 7th day to the infirmary. With progressive improvement in general condition and normalization of pulmonary and renal function, she was discharged on 15th postoperative days. Colostomy was closed after three months and colorectal anastomosis was done.
A 28-year-old female had experienced intermittent abdominal pain for 3 months and visited the Asan Medical Center, Seoul, South Korea, for further evaluation and treatment. An abdominal-pelvic computed tomography scan taken in January 2010 at the Asan Medical Center revealed a huge mass involving most of the lower abdomen and pelvic cavity with multiple septations and areas of enhancement (). The provisional diagnosis on the basis of the image findings was either borderline or malignant ovarian neoplasm. Neither ascites nor metastatic lymphadenopathy was found.\nThe patient had a history of radical nephrectomy for Wilms' tumor of the right kidney 25 years previously, at the age of 3 years, in April 1985 at an outside hospital. After surgery, she had been treated with intravenous injection of dactinomycin 25 mcg for 5 days and vincristine 1 mg on the 7th day after the operation. Since then, she had been healthy with no evidence of recurrence or metastasis until she noticed abdominal pain in September 2009.\nUnder the clinical diagnosis of an ovarian tumor, the pelvic mass was resected in February 2010. During surgery, the mass was noted to occupy the entire lower abdominal cavity without involving both adnexa. Intraoperative consultation was made and the frozen section diagnosis was malignant gastrointestinal stromal tumor or other malignant mesenchymal tumor such as leiomyosarcoma with pending evaluation of permanent sections.\nThe original slides of the Wilms' tumor, resected 25 years ago, could not be reviewed because the slides were not available in the file of the outside hospital. However, the outside pathology report stated that the size of the tumor was 12 cm and it was located at the right kidney. The tumor was abutting the head of the pancreas, and in the operation record it was written that the tumor was not completely excised. Microscopically, the tumor was shown to be a classic Wilms' tumor with predominant epithelial tubular elements and minor blastemal and mesenchymal components. No anaplasia or lymphovascular invasion was recorded, and the mitotic count was stated as scant. The final diagnosis of the nephrectomy specimen was Wilms' tumor, stage III.\nGrossly, the resected tumor in February 2010 was a 30-cm, previously ruptured, solid and cystic mass. On the cut surface, the tumor was heterogeneous, pinkish tan to yellow, and had both solid and friable areas. Multifocal hemorrhagic, cystic, and necrotic changes were also noted (). Microscopically, the tumor was composed of three different cell types, epithelial, mesenchymal, and blastemal components (). Areas of tumor necrosis were seen (). The mesenchymal tumor cells were short, spindle-shaped to round cells with scanty cytoplasm in a myxoid background (). The epithelial cell component formed tumor cell nests with glandular architecture (). The majority of the tumor compartment was an undifferentiated blastemal cell component. Mitoses were frequently observed up to 30/10 HPFs, particularly in the blastemal component. Hyperchromatism was also observed, but significant pleomorphism was not obvious (). Immunohistochemical staining was done and the results were as follows: focal positive for cytokeratin in the epithelial component but negative in the blastemal and mesenchymal components (). The Ki-67 labeling index was positive in about 50% of the tumor cells (), and p53 staining was positive in about one third of the tumor cells in all three elements. The immunohistochemical staining for WT-1, S-100 protein, SMA, desmin, myogenin, myoglobin, CD117, and CD34 was negative in all three components.\nOn the basis of the histologic findings in conjunction with the immunohistochemical findings, the tumor was diagnosed as a recurrent classic Wilms' tumor without anaplastic changes.
A 56-year-old, nulliparous woman, with previous surgical history of an open paraumbilical hernia repair without the use of prosthetic mesh a year ago was referred due to diffuse abdominal pain. The patient was on antipsychotic medication for schizophrenia, a heavy current smoker (30 pack/years) and morbidly obese (body mass index 35). Patient's mental capacity was assessed using the legally relevant criteria, and she was found to be incompetent. Therefore, her brother acted as decision maker and consented for further investigation and treatment.\nThe patient had normal pulse (85/min) and arterial blood pressure (130/82 mmHg), remaining apyrexial. She was initially assessed by the surgical team, and she was found to have an approximately 4-cm irreducible incisional paraumbilical hernia. The content and texture of the hernia sac were round and firm clinically. Tenderness was elicited with deep palpation, and an incarcerated incisional hernia was the initial working diagnosis. Routine hematological and biochemical laboratory investigations were unremarkable, and abdominal X-ray did not show any signs of small bowel obstruction. Lactate levels were within normal range.\nDigital rectal examination identified a large painful pelvic mass and no signs of bleeding. A gynecological examination was requested, but there were limitations for further assessment as the patient was virgin, and she could not undergo a per-vagina examination. Considering all the above findings and body habitus, an urgent abdominal CT scan was performed. The latter showed a 15 cm solid pelvic mass with scattered calcifications originating from the uterus or adnexa suggesting a large uterine leiomyoma. The mass was extending up to the level of a sizable lower abdominal midline incisional hernia. The hernia sac was filled with small bowel loops on imaging. No free fluid or pneumoperitoneum was noted. The patient was discussed in our MDT with gynecological input, and a decision was taken to proceed with an exploratory laparotomy.\nOf note, she was postmenopausal and nulliparous, and a decision had to be made regarding the potential scenario of hysterectomy. Following an extensive discussion with her brother, consent was obtained to proceed with hysterectomy if needed.\nAs a joint care with the Gynecologists, surgical exploration was decided and performed via a midline laparotomy incision along the previous abdominal wall scar. Cefuroxime 1.5 g was given at anesthetic induction for antimicrobial prophylaxis.\nSurprisingly, and against CT scan findings, a large incarcerated solid mass was seen within the hernia sac (Fig. ). There was no small bowel protruding through the abdominal wall defect. The large mass lesion was almost completely into the hernia sac and incarcerated, while the stalk of the tumor was occupying the neck of the hernia defect (Fig. ). Further exploration revealed the origin of this lesion being the uterus, and therefore, total abdominal hysterectomy and bilateral salpingoophorectomy were decided and performed by a Consultant Gynecologist. The incisional hernia was primarily repaired by the Consultant General Surgeon, using no prosthetic mesh, due to the emergent operative setting and infective contamination concerns. A negative pressure drain was inserted above the repaired hernia defect to prevent a seroma formation.\nFinal histopathology of the tumor showed a 19 × 14 × 9 cm uterine leiomyoma with ischemic and hemorrhagic necrosis, calcifications, and hyalinization with no evidence of dysplasia or malignancy (Fig. ).\nThe patient had an uneventful postoperative course, and standard antipsychotic medication was promptly restarted. On the second postoperative day, she accidentally pulled her negative pressure drain off, and a small seroma was formed, which was gradually absorbed. She was discharged on the 7th postop day.
A 71-year-old female visited the outpatient department with a 2-week history of coughing with sputum and dyspnea on exertion. She had no other medical history with the exception of pulmonary tuberculosis at the age of 30 years. After administration of anti-tuberculosis medication, she was completely cured.\nA chest X-ray performed during the patient's first visit to the outpatient department showed consolidation in the right lower lung field and focal consolidation in the left lower lobe. Sputum acid-fast bacteria (AFB) staining was negative. On bronchoscopy, the inlet of the right lower lobe was crushed out, especially the superior segment of the lower lobe. However, intraluminal lesions were not found. Based on these results, the patient was diagnosed with community-acquired pneumonia and was prescribed clarithromycin for 7 days. She was scheduled for a follow-up chest X-ray after finishing the medication, but she did not return to the hospital.\nThe patient returned to the hospital after 6 months because of dyspnea on exertion (American Thoracic Society grade 2). A chest X-ray was again performed (). The right middle lobe and lower lobe showed increased haziness and density together with linear consolidation in the left lung. A chest CT scan was then performed (). Consolidation was seen in all right lung zones with the exception of the superior segment of the right upper lobe. The right middle and lower lobes were mainly collapsed. Both the upper lobe and left lower lobe had many nodules. They also showed GGOs. Compared with the first chest X-ray, increases in the extent and haziness of the lesions were observed. Therefore, due to suspicion of pneumonia aggravation, we administered cefepime and clindamycin after performing a culture. However, we could not rule out the possibility of pulmonary tuberculosis reactivation or a cancerous mimic of pneumonia. Therefore, sputum AFB staining and cytology were performed, followed by bronchoscopy. Transbronchial lung biopsy was attempted, but failed. The AFB stain and cytology tests were all negative, and there was no endobronchial mucosal lesion. Upon admission, vital signs were as follows: blood pressure 100/60 mm Hg, heart rate 114 beats per minute, respiratory rate 20/min, and body temperature 39.1℃. The patient was alert and oriented, but showed an acutely ill-looking appearance, and there were rales in the right lower lobe zone with a rough respiratory sound.\nPeripheral blood test results were as follows: white blood cells 25,200/µL (neutrophils 86%, lymphocytes 7.3%, monocytes 6.1%, eosinophils 0.5%, and basophils 0.3%), hemoglobin 14.6 g/dL, and platelets 205,000/µL. In addition, the C-reactive protein level increased to 6.6 mg/dL. Chemistry test results were as follows: aspartate aminotransferase 20 IU/L, alanine aminotransferase 15 IU/L, total bilirubin 1.4 mg/dL, lactate dehydrogenase 440 IU/L, total protein 7.1 g/dL, albumin 3.8 g/dL, blood urea nitrogen 12 mg/dL, and creatinine 0.6 mg/dL. The results of arterial blood gas analysis at room temperature were as follows: pH 7.46, pCO2 31 mm Hg, pO2 67 mm Hg, HCO3 22 mmol/L, and saturation 94%. There was no improvement on chest X-ray after finishing the antibiotics or after changing them. The bronchoscopic washing cytology test results obtained before admission were suspicious for malignancy. Thus, a peripheral blood carcinoembryonic antigen level was obtained, and the result was 0.8 ng/mL.\nFor a definitive diagnosis, an open lung biopsy was performed in the basal segment of the right lower lobe. The lesion in the right lobe showed a homogenous nodular pattern during the surgery, and the biopsy was extracted from the right basal segment. A 2.5×1.7-cm section of tissue was extracted for frozen biopsy and was diagnosed as AAH (). Regular biopsy using the rest of the tissue after frozen biopsy revealed AAH with a negative epidermal growth factor receptor (EGFR) mutation test. The blood and sputum cultures upon admission were both negative, as was the AFB culture of sputum. The patient's fever and other symptoms showed improvement after 26 days of hospitalization with supportive care. The patient was then finally discharged.\nAfter 4 months, the patient was admitted again due to aggravated dyspnea on exertion. Both a chest X-ray () and CT scan () were performed. On the chest CT scans, the right lung consolidation was improved, but the extent of the GGO had increased. In the lateral segment and posterior basal segment of the left lower lobe, the GGO pattern as well as the extent and haziness of the consolidation had increased. Moreover, the size of the multiple nodules with GGO increased. Thus, the patient was diagnosed with AAH in progression. According to these findings, positron emission tomography-CT (PET-CT) was performed to rule out malignancy and in preparation for possible chemotherapy. PET-CT was performed on the first day. The images in show that there was some glucose uptake in the lesions in the collapsed part of the right lower lobe, but no uptake in the regional lymph nodes. These observations were believed to be secondary to an infection rather than a cancerous lesion. The biopsy results were consistent with AAH. Gefitinib was administered to the patient after 7 days of hospitalization. After 17 days of treatment with gefitinib, a follow-up chest X-ray was performed. shows that the right lung and hazy left lower lobe consolidation had improved compared with the findings before gefitinib treatment.
A 69-year old woman was referred to our clinic with chronic left knee pain. Her walking distance was limited and she complained of joint stiffness.\nAfter a sports trauma 40 years ago, the patient underwent a lateral meniscectomy of the left knee. Two loose tissue parts were removed; histological analysis showed synovial tissue with chronic inflammation possibly indicating rheumatoid arthritis (RA). The following decades she had internal and rheumatological examinations for multiple joint pain and general body weakness. Multiple joint osteoarthritis and rheumatoid factor (RF)-negative RA were diagnosed. In 1997 a total knee arthroplasty was performed on the right side. In 2006 the patient consulted a rheumatologist for chronic fatigue, pain in the left knee, elbow and both wrists and feet. Further laboratory and radiographic investigation yielded no other diagnosis than RF-negative RA.\nA vital woman was seen with a normal hip function. There was a correctable valgus deformity of the left leg with slight effusion of the knee and tenderness on palpation of the lateral joint space. No inflammation was seen and range of motion was normal. A conventional X-ray showed severe lateral osteoarthritis of the knee with loss of height of the lateral tibial plateau (Figure\nA,B).\nWith informed consent of the patient we decided to proceed to a total knee replacement. Intraoperatively, pigmented vitreous synovial tissue was seen in the subcutaneous tissue, which was resected and sent to the pathology department for further analysis. A total knee replacement could be performed without any complications. The patient recovered well and was discharged five days postoperatively.\nImmunohistological analysis of the resected soft tissue showed a large cell lymphoid proliferation under the synovial tissue surface with expression of B-cell antigen CD-20 (Figure\nA-C). This finding matches the localization of a B-cell NHL, WHO 2008 classified as diffuse large B-cell lymphoma– not otherwise specified (DLBCL-NOS). WHO guidelines suggest an Epstein Bar Virus-negative B-cell lymphoma that develops around chronically inflamed joints in a patient with RA, as in the present case, to be classified in this category.\nAnamnesis and physical examination by an oncologist did not reveal any clues for malignancy. But a PET-CT scan showed pathologic enlarged lymph nodes along the aorta and the left iliac and inguinal vessels. Lungs and liver were clean in the scan and a bone marrow biopsy did not show signs of tumor activity. The B-cell lymphoma was staged level 2-E, therapy was started with rituximab, cyclofosfamide, vincristine, doxorubicin and prednisone (R-CHOP) combination chemotherapy in six doses.\nIn 5-25% of the patients with NHL, the bone is involved, sometimes resulting in joint pain\n[-]. When this is the primary symptom, imaging techniques can raise suspicion of a malignancy. Also, bone surfaces during surgery can be suspect. A NHL was once reported in the cutting surface of the femur in a total knee arthroplasty\n[]. A routine analysis of 852 retrieved femoral heads in hip arthroplasty confirms the possibility of bone involvement\n[]. In 14 femoral heads a low-grade B-cell lymphoma was detected. Systemic disease was found in only two of these patients.\nThe diagnosis in this case report was based on synovial tissue analysis. This coincidental finding was reported twice before, though this was in elbow and shoulder surgery\n[,]. In a literature overview till 2006, 13 cases of intra-articular synovial manifestation of NHL were presented, of which 11 cases concerned the knee\n[]. All patients presented with inflammation of the knee joint, sometimes clinically simulating RA\n[,]. In later literature two more patients were strikingly described discovering NHL with arthroscopy\n[,]. Both patients were planned for a partial meniscectomy. One patient (51 years old) had a history of gonarthritis deformans, without signs of inflammation of the knee.\n[] The other patient (31 years old) had a constant knee pain and swelling\n[]. Arthroscopically obtained atypical synovial tissue appeared to be a B-cell lymphoma in both cases.\nImportant in this case report is that patients with active RA have an increased risk of developing lymphoma\n[,-]. A recent review showed that aggressive B-cell lymphomas, particularly the diffuse large B-cell lymphoma in the present case, are stronger associated with autoimmune rheumatic diseases than more indolent lymphomas. Although the presence of NHL was less associated with RA than with Sjögren’s syndrome and systemic lupus erythematosus\n[], a 28-fold increased risk of NHL in patients with RA was found when severe damage in the knee existed in the year prior to lymphoma diagnosis\n[]. Most lymphomas in this population are diffuse large B-cell lymphomas, which form an aggressive subtype of NHLs. Though with new treatment methods survival has significantly increased over the last decade\n[].\nThe main pathophysiological mechanisms for NHL are B-cell hyperactivity and chronic inflammation\n[]. Nevertheless, anti-inflammatory drugs are no treatment option for NHL\n[]. Probably through its malignant character, NHL will be persistent to anti-inflammatory drugs, also in case of musculoskeletal involvement. In relation with RA, the rheumatic disease itself appears to have a larger effect on the development of lymphoma than its therapy\n[]. As the presented patient had used immunosuppressive drugs (salazopyrine and leflunomide) for only two years, the contribution of drugs can be expected to be minimal. Part of the patients with a lymphoma is infected with the Epstein-Barr Virus (EBV)\n[]. The presence of EBV in the lymphoma can have therapeutic consequences. Though, in the presented case no EBV-encoded RNA was found with in-situ hybridisation.
A 21-day-old boy with vomiting, abdominal distention and feeding intolerance presented to our institution. He was born at 36 weeks and 6 days of gestation, weighing 2220 g, with Apgar scores of 8 at 1 min and 8 at 5 min. For the first 4 days of life, he was both breast- and formula-fed. After hospital discharge, he was exclusively breast-fed. He experienced occasional vomiting until 19 days of age, when he developed frequent vomiting. When he was 20 days old, he was taken to the doctor for several days of watery stools, a single episode of bilious vomiting, and feeding intolerance. He was admitted to a local hospital at a weight of 2685 g. Abdominal radiography showed partially dilated loops of bowel with intestinal gas (Fig. a), and a gastric tube was inserted for frequent vomiting. At the age of 21 days, he was transferred to our hospital for further examination. A gastrointestinal X-ray series and an enema revealed gastric volvulus and gastroesophageal reflux, without intestinal malrotation or a change in intestinal caliber. We admitted the patient for observation. The following day, an abdominal radiograph showed complete passage of contrast, which indicated the absence of intestinal atresia or obstruction. However, the volume of bile discharged through the gastric tube was gradually increasing, and he had little passage of feces, even with a glycerin enema. We decided to re-evaluate for intestinal obstruction and injected contrast into the gastric tube. Follow-up abdominal radiography showed obvious intestinal dilation with gas and retention of the contrast medium (Fig. b and c). Based on his clinical course and radiological findings, we suspected distal intestinal obstruction. When the patient was 24 days of age, we performed laparotomy, which revealed a caliber change in the ileum with a stricture ~10 cm proximal to the ileocecal valve (Fig. a). A 6-cm length of bowel around the stricture site was resected, and an end-to-end anastomosis was performed. On gross findings of the resected specimen, the lesion was noted to be 1.5 cm in length, featuring a stricture and erosion/ulceration (Fig. b and c). On microscopic examination, the margins of resection showed <20 eosinophils per high-power field (HPF) (×400) (Fig. a). However, mucosal eosinophilia was recognized in distant position from the ulcer (Fig. b and c). The presence of eosinophil accumulation in the lesions presented more than 20 eosinophils per high-power field (HPF) (×400). The eosinophils were oriented towards the epithelium and diffusely distributed throughout the tissue. In the stenotic portion of the specimen, the layers between the mucosa and the muscularis propria were notably absent, and granulation and fibrotic tissues were found (Fig. d).\nBased on these findings, the suspected diagnosis was eosinophilic enteritis. However, laboratory data on admission showed no hypereosinophilia (white blood cell count, 4800/μL with 1.4% eosinophils). Serum allergy investigation revealed no remarkable elevation in non-specific IgE, although the C-reactive protein level was slightly elevated (Table ). Nevertheless, we strongly suspected a food protein-induced allergy to cow’s milk or breast milk. An allergen-specific lymphocyte stimulation test was performed for kappa (κ)-casein, lactoferrin and human alpha (α)-lactalbumin (outsourced to Bio Medical Laboratories [BML, Inc, Tokyo, Japan]) []. The lymphocyte response to lactoferrin was markedly increased (7489 counts per minute; stimulation index, 10.11; cutoff index, 3.9) (Table ). He was finally diagnosed as eosinophilic enteritis with intestinal stricture caused by an allergy to either cow’s milk or breast milk. After surgery, the patient was fed with a pediatric elemental formula. His subsequent hospital course was uneventful, and he was discharged with no symptoms and good weight gain on the 19th postoperative day. At 5 months of age, he was able to eat baby food with no recurrence of symptoms. At 1 year of age, favorable growth and development were confirmed.
A 35-year-old male patient reported to the outpatient department of our institute, Maratha Mandal's Nathajirao G. Halgekar Institute of Dental Sciences and Research Centre, Belagavi, on July 7, 2019, with a chief complaint of pain in the left and right lower jaw area for 5 months while having food. On detailed history, the patient informed that he experiences acute pain (10/10 on Numerical Rating Scale) on having the first bite of meal and gradually subsides on subsequent intake of food and subsides completely in few minutes after completion of meal. The pain was described as sharp and stabbing type. He experiences maximum pain while having the first bite of his food in the morning, and subsequently, with every first bite of the meal in a day, he experienced pain, but the intensity of pain reduced from morning to evening. During dinner, the intensity of pain experienced on the first bite of the meal was least. Because of pain during eating, the patient developed aversion for food which led to his weight loss.\nPertaining to medical history, the patient had met with an accident in 2014 and was hospitalized for 2 months and recovery was uneventful. Later in October 2018, he had a head injury following a fight where he was hit with a rod on the head, which was sutured, followed by which he was treated as a day-care patient. During this time, the patient was diagnosed with diabetes. Since then, he is on medication for diabetes (Glycomet 250 mg). The patient's blood glucose level was in normal limits with controlled level of glycosylated hemoglobin. Later in January 2019, he started experiencing pain in the parotid region while having food.\nHe initially visited an ENT specialist for the same, where he was diagnosed to have parotitis and was prescribed with medication which did not give any permanent relief. The patient is unaware of the medicine prescribed. Later, he visited another two ENT specialists. He was advised for scanning. Ultrasonographies of both right and left parotid glands were taken []. A computed tomography scan was taken from the base of the skull to the lung apex. No relevant findings or abnormalities were noted except for a slight enlargement of the glands []. He was asked to continue with the same medication, which did not give him any permanent relief. Hence, he was referred to dental institute.\nOn examination, bilateral swelling was seen on the angle of mandible with raised earlobe []. On palpation, tenderness was elicited on the left side. On milking of parotid glands, clear but scanty fluid was noted. On intraoral and extraoral examination, no other abnormalities were detected.\nBased on the history, symptoms observed and records of imaging, a final diagnosis of FBS was arrived at.\nFrom July, the patient was prescribed with drugs for neuropathic pain that is Pregeb M 75, one tablet once daily, and Teedal, one tablet twice daily. The patient was called for weekly follow-up. After 5 months patient showed 80% reduction in pain and rated 2/10 on pain scale for pain. The patient is still under follow-up.\nOn literature search, we found six published reports (one case series and five case reports) of IFBS, out of which two case reports were in Japanese language and abstract was available in English; hence, it was also included in review [].\nThe first case was reported in the year 2010 by Kurokawa et al. of a patient with a 6-month history of pain in the parotid region that occurred after the first bite of every meal. Pain decreased after 6 repeats of stellate ganglion blocks (SGBs) and completely disappeared after 16 repeats of SGB.[] The patient had no history of trauma or surgery.\nWemyss et al. reported a case of the patient who had severe, sharp unilateral pain on the first bite of eating. No abnormalities were detected with respect to TMJ, PPS, trigeminal nerve or parotid gland. FBS was suspected, and the patient was immediately started with carbamazepine 200 mg, three times daily. It was proved to be successful in treating, and in 4 months, symptoms resolved completely.[]\nA similar case with symptoms consistent with FBS was reported by Stoopler et al. Medical therapy of the patient included oxcarbazepine and gabapentin without benefit. Later, patient was given botulinum toxin A injections to the parotid gland and patient reported improvement in pain with the treatment.[]\nChiba et al. reported case series of 14 patients with idiopathic parotid pain (IPP), and they opined that clinical picture and pathogenesis of IFBS remains unknown and also only a few cases have been reported in the literature, where the difference in clinical presentation of IPP and IFBS has not been clearly described. Hence, they referred their cases as IPP instead of IFBS. Out of 14 cases analyzed retrospectively by checking the medical records, they considered only 9 cases that were diagnosed with diabetes as IPP and the rest 5 cases were not considered as they had no history of diabetes. Their main aim was to find if there was any association of diabetic neuropathy with FBS. In this study, the authors ruled out various pathogeneses reported earlier such as the loss of sympathetic innervations to the parotid gland and gustatory stimuli which evoked facial pain. Thus, the pathogenesis of IPP in the study remained to be elucidated. They believed that there might be an association between diabetic neuropathy and pathogenesis of IPP, but it is unlikely that the diabetic neuropathy alone contributed to the development of IPP. Thus, the relationship between IPP and diabetes was speculated and required further studies.[]\nHayashi et al. reported a case of IFBS treated with rikkosan. Here, the treatment with carbamazepine was ineffective and was, in turn, treated with the Kampo medicine rikkosan gargles along with dietary strategy. In this case, in 1 year, IFBS improved with no recurrence.[]\nThe literature review provided with total of five individual case reports of IFBS and one case series of five cases without diabetes diagnosed as IFBS and nine cases with diabetes. We report an additional case of IFBS with diabetes.
A 30-year-old Brazilian male was brought to our ED from a local jail because of agitation. He had been arrested by Swiss authorities because of suspected internal concealment of drugs of abuse (body packing), which had been confirmed by abdominal CT scan in our hospital approximately 12 h before (Fig. ). During this first visit, the patient had presented asymptomatic and had admitted to carrying cocaine-containing body packets. In addition, he had reported recreational use of marijuana and cocaine. It was also noted that the patient was transsexual; he had breast implants. The patient’s past medical history was unknown. Standard operating procedure in such a case is to perform a CT scan without a consultation at the ED. During the second visit approximately 12 h later, the patient presented with psychomotor agitation, mydriasis and tachycardia. His heart rate was 116 bpm, blood pressure was 116/68 mmHg, respiratory rate was 40/min, oxygen saturation by pulse oximetry was 99% on ambient air and auricular temperature was 38.0° Celsius. Communication with the patient was impossible because of his altered mental status and because he spoke a foreign language. The physical examination of heart, lungs and abdomen revealed no pathologies. Neurological examination showed symmetrical spontaneous movement of all extremities and symmetrical gaze to both sides prompted by speech or touch. Glasgow coma scale was 11. An electrocardiogram showed sinus tachycardia without signs of ischemia. Due to our knowledge of the ingested body packets, we first suspected cocaine intoxication because of package rupture. We treated the patient with repeated doses of intravenous midazolam and performed an emergency abdominal CT scan to guide potential emergency surgical decontamination. The CT scan showed 60–70 packets in the gastrointestinal tract without signs of gastrointestinal obstruction or perforation. Laboratory results showed an increased C-reactive protein (CRP) level (231 mg/l, norm < 5 mg/l), an increased creatinine level (181 mcmol/l, norm 62–106 mcmol/l), an increased creatine kinase (CK) level (1000 U/l, norm < 190 U/l), a slightly increased troponin level (16 ng/l, norm < 14 ng/l), hypoglycemia (2.8 mmol/l), mild hyponatremia (128 mmol/l, norm 136–145 mmol/l), hyperkalemia (5.8 mmol/l, norm 3.3–4.5 mmol/l) and hyperphosphatemia (1.68 mmol/l, norm 0.87–1.45 mmol/l) (Table ). Urine analysis showed no evidence of urinary tract infection. A qualitative urine toxicological test was positive for cocaine and benzodiazepines.\nGiven the clinical presentation of our patient with remarkable hypotension despite cocaine intoxication and elevated inflammatory markers, we suspected infection rather than massive cocaine intoxication to be the main problem. Thorough review of the abdominal CT scans revealed rapidly progressive pulmonary infiltrates in the lower left lobe and lingula, diffuse lymphadenopathy and hepatosplenomegaly (Fig. ). To assess the extent and morphology of the pulmonary infiltrates and to investigate for thoracic lymphadenopathy or solid tumors, we performed a thoracic CT scan. Compared to the abdominal CT scan 4 h before, it showed progression of the pulmonary infiltrates in the lower left lobe and lingula, multiple nodular pulmonary consolidations in both upper lobes compatible with septic emboli or tuberculous foci as well as diffuse cervical, mediastinal and axillary lymphadenopathy.\nDue to the above named findings, we withheld emergency laparotomy and started broad-spectrum antibacterial treatment with ceftriaxone and clarithromycin according to our hospital’s guidelines for severe pneumonia. Given the rapid progression of the pulmonary infiltrates in the course of few hours on the thoracic CT scan, we considered the possibility of toxic rather than infectious infiltrates. Because of the patient’s poor general condition we therefore decided to broaden the antibacterial treatment as for sepsis with unknown focus – especially taking into account an abdominal source – to piperacillin/tazobactam instead of ceftriaxone according to our hospital’s guidelines. Based on the elevated creatinine level we diagnosed acute kidney injury, most probably caused by dehydration precipitated by infection and cocaine intoxication. The patient also had an elevated CK level. Cocaine-induced rhabdomyolysis could therefore be an additional cause of the acute kidney injury. Therefore we treated the patient with intravenous fluids.\nThe patient was admitted to our intensive care unit and isolated for possible pulmonary tuberculosis. In the course of his hospitalization, further diagnostics were available: Pneumococcal urinary antigen test was positive. Screening and confirmatory test for HIV-1 were positive and CD4+ T-cell count was 144/μl, so we diagnosed CDC stage A3 HIV infection. Blood and urine cultures didn’t show any bacterial growth. The diagnosis of pneumococcal pneumonia and sepsis could thus be confirmed, with underlying untreated HIV infection as risk factor. Active pulmonary tuberculosis was excluded by three negative sputum smears and three negative sputum cultures.\nWith the aid of an interpreter, the patient told us in the course of the hospitalization that one of the drug packages had ruptured in his mouth during swallowing, but that he had been able to spit out most of the cocaine. The exact time of this event remained uncertain.\nUpon diagnosis of pneumococcal pneumonia, the antibacterial therapy was changed from piperacillin/tazobactam back to ceftriaxone. As beta-lactam resistant pneumococci were a concern because of the patient’s geographical origin, vancomycin was added to ceftriaxone. This regimen was continued for 7 days. Clarithromycin was stopped after diagnosis of pneumococcal pneumonia. After 9 days, the patient was discharged into custody of the Swiss authorities. Antiretroviral therapy was established in an outpatient setting after ruling out active pulmonary tuberculosis. The patient recovered uneventfully.\nFigure shows the key diagnostic findings and interventions on a timeline.
A 21-year-old female presented to the Case Western Reserve University School of Dental Medicine Clinic in Cleveland, Ohio. Her chief complaint was a slowly growing painless swelling involving the right side of the mandible that had started one year previously. Her past medical history revealed no previous surgeries or diseases. Her vital signs were recorded as 120/80 mmHg blood pressure, a pulse of 103 beats per minute (bpm), 15 respirations per minute, height - 5.1 ft, weight - 165 lb, and a calculated body mass index (BMI) of 23.71. No other medical conditions were identified, and the patient did not report taking any medications. Clinical examination revealed an intraoral swelling involving the posterior part of the body of the mandible and extending to the ramus on the right side. On palpation, the lesion was hard in consistency, except for select areas which exhibited a softer texture.\nThe patient was referred to a private dental imaging center for a CBCT scan to evaluate the extent of the lesion. A board-certified oral and maxillofacial radiologist performed the radiographic interpretation of the CBCT scan. The scan revealed a well-defined radiolucent lesion ranging from the interdental bone in between the second and third right molars and extending to the ramus of the mandible posteriorly in the anteroposterior direction. The lesion extended from the alveolar crest to the inferior border of the mandible in the superior-inferior direction (Figure ).\nOsteolytic changes of the alveolar crest margin distal to the third molar were noted and displaced the roots of the third molar more distally. The sagittal cut showed that the lesion had a multilocular appearance with an incomplete internal septal structure, demonstrating wispy-like septations. The inferior border of the mandible showed some resorption with undulating borders (Figure ). The lesion showed expansion of the alveolar crest. In axial cuts, the lesion involved the ramus of the mandible, and expansion and thinning of the inner cortical plate were noted.\nExtending more medially, the borders of the lesion showed fine wispy-like septations with undulating borders (Figure ). Coronal slices demonstrated the resorption of the outer cortical boundary, a multilocular appearance, an expansible nature, and thinning and resorption of the inner cortical boundary. The presence of an intact (albeit thinning) border, wispy septations, and expansion led to a provisional diagnosis of an aggressive benign tumor. Central giant cell granuloma, ameloblastoma, and keratocystic odontogenic tumor (KOT) were considered in the differential. However, the presence of fine, wispy septations and undulating borders favored a CGCG diagnosis.\nAn incisional biopsy was performed, and the histopathological report for the lesion revealed a giant cell reparative granuloma formed of proliferating spindle cells, multinucleated giant cells, extravasated red blood cells (RBCs), and mononuclear cells. No specific inflammatory granuloma or significant nuclear anaplasia and mitosis or other evidence of malignancy were noticed. This information, along with clinical and radiological features, was suggestive for CGCG and hyperparathyroidism (because of similar histopathological features). Laboratory investigations showed that the parathyroid hormone, alkaline phosphates, and calcium levels were all within normal limits. This excluded hyperparathyroidism as a possible diagnosis. The patient underwent surgical resection of the lesion. The surgery and recovery were uneventful.
A 65-year-old Caucasian female with a past medical history of hypertension, gastroesophageal reflux disease, iron deficiency anemia, and rheumatoid arthritis was referred for exposed left mandibular bone and a persistent neck fistula 3 weeks after extraction of tooth #20. She had a concomitant extraoral incision and drainage for a presumed submandibular abscess by an outside practitioner. The patient reported a history of being on bisphosphonates for osteoporosis. She was on Risedronate (Actonel) for a total of 4 years and was then switched to yearly Zoledronic Acid (Reclast) injections for a period of two years with the last dose being roughly 1 year prior to the extraction. She was however started on Denosumab (Prolia) subcutaneously roughly 1 week prior to her extraction.\nOn physical exam, the patient had a 1 cm cutaneous fistula in left submandibular region. Intraorally there was an area of exposed bone roughly 1 cm in size on the buccal aspect of the mandible in the area of tooth #20; there was a separate area of exposed bone on the lingual aspect. There was minimal amount of thin purulent drainage intraorally and on the neck bandage. A diagnosis of MRONJ was made based on her clinical presentation, and she was scheduled for follow-up with imaging to discuss her treatment options. At follow-up 10 days later, the patient's lesions had increased in size and the two areas of exposed bone were confluent. There was another 5 mm area of exposed bone in the symphysis region and another 5 mm area in the right retromolar pad area. The neck fistula slightly increased in size. CT scan showed sclerotic changes involving the mandible diffusely. Given the diffuse nature of her bony involvement and continued progression of bony exposure, we have elected to watch and wait and allow the necrotic bone to declare itself prior to proceeding with surgical resection and microvascular reconstruction.\nA week later, the patient was transferred from an outside hospital to our intensive care unit with a diagnosis of sepsis. Her left neck fistula had doubled in size and now had a necrotic appearance. The inferior border of the mandible was visible in the wound (). Intraorally, the area of bony exposure increased in size to 2.5–3 cm area in the left posterior mandible. The symphysis and right posterior mandible areas of bony exposure also increased in size (). Moreover the right side of her soft palate appeared to have undergone spontaneous necrosis, forming a through and through 2 cm defect into the nasopharynx. This fistula did not communicate with any areas of bony necrosis on exam and endoscopy (). The patient was started on broad spectrum antibiotics including Vancomycin, Levofloxacin, and Meropenem and ID was consulted. The patient was afebrile with a white cell count of 10.5 × 103/mm3. She suffered from tachycardia and was tachypneic and her chest X-ray showed findings suspicious of Acute Respiratory Distress Syndrome (ARDS) (). She required supplemental oxygen but no mechanical ventilation. She also received a blood transfusion at the outside hospital for hemoglobin of 5.0 g/dL and was transfused again in our facility for hemoglobin of 7.1 g/dL. She received multiple platelet transfusions for thrombocytopenia with a count 63 × 103/mm3 on arrival and a nadir of 16 × 103/mm3 (unit) during her 10-day hospital stay. The differential diagnosis by hematology was autoimmune causes due to her sepsis such as idiopathic thrombocytopenic purpura (ITP) versus drug related causes. The patient also had anion gap metabolic acidosis and was managed with fluids, insulin, dextrose, and bicarbonate and took several days to stabilize. Blood cultures eventually grew out Escherichia coli which was sensitive to the antibiotics administered. A repeat CT scan showed no abscess but showed some air in the marrow on left side of the mandible (). It also showed air in the epidural space in cervical region (). Neurosurgery recommended treatment with antibiotics.\nAs the patient progressed in her hospital course, she started to improve. Her anion gap was corrected, her counts improved, and her intra- and extraoral wounds stabilized. A PICC line was placed and the plan was to have the patient complete a 4-week course of Unasyn (Ampicillin/Sulbactam) after discharge followed by oral Augmentin (Amoxicillin/Clavulanate). Due to her generalized weakness and diffuse involvement of her mandible, the patient was deemed not a good surgical candidate at the time. There was also a concern with regard to soft tissue healing after surgery given her soft tissue wounds.\nAt follow-up 3 months after discharge, the palatal fistula was found to have completely healed. The neck wound had nearly completely healed with a pin point fistula with minimal drainage remaining. The 2 small areas of bony exposure in the symphysis and right posterior mandible had resolved. The left mandibular bony exposure was stable without progression. The patient continued to show progressive improvement on periodic follow-up. At 1 year, the soft palate and neck wounds were completely healed, and the intraoral wound had dramatically decreased in size with no purulence (). At 18 months the patient continued to do well. She essentially had near complete recovery with no surgical intervention. The patient was content with area of exposed bone in her oral cavity and was not interested in any conservative surgical debridement.
A 44-year-old woman (height: 5′4′′ [1.6 m]; weight: 185 lbs [83.9 kg]; body mass index: 31.75) presented to the Emergency Department with complaints of severe low back pain and bilateral lower extremity pain and weakness. She stated that the symptoms initiated one day earlier with muscle cramping of the bilateral posterior thighs and had gradually progressed to an inability to walk or lift her legs. The patient initially attributed her symptoms to dehydration as she had worked several 12-h shifts in a row as a registered nurse. She reported some urinary hesitancy although denied urinary or bowel incontinence, any sensory deficits of the lower extremities, or severe headaches. The patient mentioned several stressors, including marriage in the next week to a man to whom she had been previously married, working overtime, minimal sleeping at night, and long commute to work. Past medical history was significant for anxiety, depression, and migraines. The patient denied a history of sexually transmitted diseases. Two years earlier, the patient was hospitalized for 24 h following complaints of right hemiparesis and chest pain. She was diagnosed with conversion disorder due to a psychological stressor.\nThe physical examination revealed 1/5 power in the lower extremities bilaterally, with distal strength greater than proximal. Sensation, deep tendon reflexes, and muscle tone of the lower extremities were normal. There was an absence of atrophy or hypertrophy of the muscles, rigidity or spasticity, and tremors or abnormal movements. Serial cervical, thoracic, and lumbar MRI scans with and without gadolinium contrast demonstrated no significant stenosis, neural compression, or other etiologies of her symptoms. The brain MRI with and without gadolinium contrast was normal. The initial impression was conversion disorder due to the patient's multiple work and familial stressors. She was hospitalized and treated with methylprednisolone and hydromorphone.\nA lumbar puncture and a CSF pathogen panel were performed, the latter utilizing a multiplex nested PCR followed by a melting analysis to detect nucleic acid sequences. The sensitivity was greater than 95%, and the specificity was greater than 99.6%. The panel detected HSV-2 and was negative for crypococcal antigen. The following findings were also observed in the CSF: glucose 90 mg/dL (40–70 mg/dL), protein 55 mg/dL (12–60 mg/dL), WBC 434/μL (0–5/μL), RBC 9/μL (0–2/μL), lymphocytes 94% (40–80%), and monocytes 6% (15–45%). The culture CSF/Gram stain revealed rare WBCs and no organisms, and the HIV-1 and -2 antibody/antigen combination was nonreactive. Borrelia burgdorferi antibodies (Lyme disease), syphilis total antibodies, hepatitis B core antibodies and surface antigens, Chlamydia trachomatis, and Neisseria gonorrhoeae were all negative. The patient was diagnosed with radiculomyelitis due to HSV-2 marked by back pain and weakness of the lower extremities with evidence of lymphocytic pleocytosis. She initiated treatment with acyclovir 650 mg infused every 8 h for 3 weeks and was subsequently prescribed valacyclovir.\nThe patient had attained no improvement of her lower extremity pain when evaluated by a neurologist 5 and 8 months following the start of her symptoms. Additionally, CSF findings demonstrated a WBC count 6/μL, protein 33 mg/dL, and glucose 57 mg/dL 5 months after her initial lumbar puncture, respectively. There were greater than 4 oligoclonal bands detected in the CSF with no corresponding bands detected in the serum, and the IgG index was 0.9 [0.3–0.7]. These positive findings indicated an inflammatory process. The aquaporin 4 (AQP4) receptor antibody was negative. The patient was evaluated by a neurologist 17 months after her symptoms initiated, at which time she had significant improvement in the range of motion strength in her lower extremities. Her minimal residual pain was controlled with gabapentin, baclofen, and alprazolam.
A 25-year-old married woman was brought by the mother to psychiatry outpatient department with the complaints of withdrawn behavior, headache, abnormal posturing, and reduced sleep since around 15 days and sudden onset altered behavior a night before during sleep, when patient woke up, started screaming, went to wash room and started inducing the vomiting. She was fearful and not recognizing the family members and couldn't sleep. The episode lasted for the whole night and the next day she was brought to the hospital. Family members denied any stressor or organic antecedents before the start of illness. There was no sociooccupational impairment according to family members though the patient was symptomatic in last 15 days. Patient did not report any depressive features, delusions, or hallucinations. Mother also reported doubtful history of abnormal jerky movements of lower limbs in sleep with altered sensorium, only one episode that lasted for 4-5 min few months back. On examination in the outpatient department, patient was found emaciated, not interacting, not maintaining eye contact, maintaining abnormal posture, was starring and bit drowsy. She was minimally responding even to the painful stimuli. Patient was urgently referred to Medicine Department, CT scan brain was done and the patient was admitted to medicine intensive care unit (MICU). The radiological findings showed bilateral symmetrical dense/amorphous BGC with white and grey matter edema in the left parietal and occipital region []. Based on the clinical features and radiological signs, differential diagnoses were considered as acute confusional state and cerebral edema with the possibility of tubercular meningitis, cerebral malaria and viral meningitis. Patient was investigated further for above conditions and meanwhile neurologist was consulted who advised magnetic resonant imaging (MRI) and electro-encephalogram. Patient was started on antibiotics and phenytoin. Patient remained in MICU for around 10 days and was persistently confused, agitated, talking irrelevantly, disoriented, sometimes not communicating at all and maintaining abnormal posture. For behavioral control, she was started on oral risperidone 0.5 mg and lorazepam 1 mg. Her cerebrospinal fluid examination revealed normal study, patient was negative for malarial parasite, and serum electrolytes were within normal range with normal liver and kidney function tests. Serum calcium and magnesium levels were within normal limits. MRI findings were suggestive of inherited/acquired leukoencephalopathy with BGC and associated mass effects on the left side of brain with grey matter hypointensity. She was negative for HIV and hepatitis B virus testing. Her hormonal study revealed normal thyroid status with low level of serum parathyroid hormone. Slowly there was an improvement in the features of delirium and when she became medically stable, was transferred to a psychiatry ward. In psychiatry ward patient was found continuously pacing around, minimally interacting, had poor self-care and disorganized behavior (e.g. handling the feces), irritable and anxious. But she was oriented in time, place and person. She was inconsistent in her verbal responses with rapid and unclear speech (dysarthric speech) and sometimes words could not be understood. Her judgment, immediate and recent memory was impaired and was not co-operative for remote memory testing. Doses of risperidone were increased. In view of low parathyroid level, her ionic calcium levels were done which came out to be within normal range (1.16 mmol/l). Endocrinologist's opinion was taken and started on treatment for hypoparathyroidism. Her past history revealed one episodes of vomiting with abnormal jerky movements of limbs around 4 years back. The details of treatment were not available. Her family history was not significant. She was educated up to HSC, was average in studies, married for around 8 years and had a history of spontaneous abortion around 2 years back. Her menstrual cycles were regular. Based on above history and investigations, diagnosis of Fahr's syndrome (bilateral BGC, dysarthria and neuropsychiatric symptoms) probably due to hypoparathyroidism was kept. Patient showed partial improvement in behavioral symptoms; hence, oxcarbazepine was added in a dose of 300 mg. Patient responded (with risperidone, oxcarbazapine, lorazepam, and memantine and treatment for hypo-parathyroidism) in next 20 days and was discharged.
A 74-yr-old woman was transferred to our emergency department from the local general hospital with an initial diagnosis of brain stem infarct on January 17, 2009. The patient was found unconscious and in a bed-ridden state during the early morning on the presenting day. The patient's breathing was shallow and irregular. The patient was unable to walk well due to a coccyx fracture two years ago, but she was able to perform daily life activities with minor help. One week ago, her facial edema started and she complained dry cough and general weakness three days ago. No other medical, familial, or social history was reported, and the patient had not taken any medication. There was also no evidence of intoxication.\nAccording to the records of the local general hospital, the patient had an impending respiratory arrest and comatose mental status. Her blood pressure was 71/48 mmHg, her body temperature was 36.0℃, and her pulse rate was 99 beats/min with a sinus rhythm by electrocardiography (ECG). Neurologic examination revealed pinpoint pupils and a lack of brain stem reflexes, including the light reflex, corneal reflex, gag reflex, and doll's-eye phenomenon. She also showed decerebrated rigidity in response to painful external stimuli. The patient was assigned a score of 5 on the Glasgow Coma Scale, and the Babinski sign was detected at both feet. Brain computed tomography (CT) produced unremarkable results. Endotracheal intubation was performed for protection of the patient's airway, assisted ventilation was initiated, and an inotropic agent was injected to support the patient's state of shock, which did not respond to fluid therapy (1 L of normal saline). Prior to transfer, the patient's blood pressure was increased, but her neurologic findings were not improved. She was transferred to our hospital for further evaluation and treatment of a brain stem infarct.\nOn arrival, the patient's blood pressure was 70/40 mmHg, her pulse rate was 59 beats/min, and an intravenous infusion of premixed dopamine was administered (500 mL/800 mg, 20 µg/kg/min). The patient's body temperature was 34.8℃ according to a tympanic membrane thermometer, and her self-respiration was slow and shallow. The patient's lungs on auscultation were bilaterally clear, and cardiac examination showed a regular rate and rhythm without murmurs, gallops, or rubs. The patient's abdomen was soft without signs of guarding, tenderness, or rebound tenderness. A rectal examination revealed no masses, and the patient's stool was guaiac negative. No soiling due to urine or feces was noted on her undergarments.\nThe patient's face and extremities were edematous, and she presented with non-pitting edema. Her skin was dry, cool, pale, and slightly mottled and desquamated; however, there was no evidence of petechiae or purpura ().\nThe patient's level of consciousness was stupor to semi-coma. A neurologic examination revealed intact cranial nerves and she didn't show lateralizing signs. The patient's pupils were 3 mm across and briskly reactive. It was impossible to check for visual abnormalities and focal cerebellar findings. The results of the Babinski reflex test were negative.\nThe patient's laboratory values were as follows: WBC count 6,000/µL (86% neutrophils), hemoglobin 11.8 g/dL, sodium 121 mEq/L, potassium 4.2 mEq/L, chloride 87 mEq/L, BUN 1.3 mg/dL, creatinine 0.6 mg/dL, glucose 104 mg/dL, ALT 113 U/L, AST 74 U/L, albumin 3.3 g/dL, CK 499 IU/L, CK-MB 21.4 ng/mL, troponin-I <0.02 ng/mL, lactate 1.2 mM/L, urine RBCs 1-4/HPF, and urine WBCs 0-1.5/HPF. Arterial blood gas analysis (FiO2 1.0) revealed a pH of 7.486, a pCO2 of 30.6 mmHg, a pO2 of 36.4 mmHg, a HCO3- concentration of 22.9 mM/L, and an oxygen saturation of 77%.\nA portable chest radiography revealed infiltration of the right lower lung zone and cardiomegaly. ECG revealed sinus bradycardia with no evidence of ischemia; the intervals and axis were normal except for a prolonged QTc interval. Echocardiography revealed a small amount of pericardial effusion, but it did not affect the patient's hemodynamic status.\nDuring our resuscitative efforts, the patient's blood pressure didn't respond and additional laboratory test results confirmed hypothyroidism. The patient's free thyroxine was 0.05 ng/dL (0.93-1.7) and her TSH was 30.12 µIU/mL (0.27-5.0). A diagnosis of myxedema coma exacerbated by community-acquired pneumonia was thus assigned.\nThe patient was referred to the Department of Endocrinology and admitted to the intensive care unit for treatment and ventilatory support. The patient received 1,000 µg of levothyroxine orally for two days, followed by 300 µg/day for an additional three days and finally maintained on levothyroxine 0.1 mg orally each day. Following the administration of synthetic thyroid hormones for hypothyroidism and antibiotics for pneumonia, the patient regained consciousness and respiration. She was transferred to a general ward after two weeks in the intensive care unit and discharged from the hospital one week later.
A 28-year old native female patient complained about recurrent pain in the right hip joint without previous trauma progressing by physical strain starting about 1.5 years before. A MRI of the pelvis performed 3 months before admittance showed a swelling of the capsule and reduction of the cartilage in the right hip joint which was considered as an early arthritis or coxitis. After treatment with physiotherapy and oral non-steroidal antirheumatic medication the symptoms were regressive and no further diagnostic procedures were performed.\nCaused by recently aggravation of pain in the right hip joint within the days before, the patient was not able to stand and walk and the range of motion (ROM) was painfully reduced. Blood samples were normal except slightly increased CRP of 4.2mg/l (reference: <0.5mg/l) without any local signs of inflammation in the pelvic region. X-ray of the right pelvis showed a slight joint space narrowing with affection of the acetabulum and the femoral head (Fig. ). An effusion was detected by ultrasound, aspiration of the hip joint led to no further diagnosis as aerobic and anaerobic cultures were sterile. The MRI showed a joint space narrowing in the cranial part of the acetabulum with increased subchondral sclerosis as well as a synovial enhancement with bone marrow edema in the femoral head and the corresponding acetabulum.\nThe further clinical examination with detailed reconstruction of patient´s history revealed a surgical procedure with extirpation of a lymph node from the left supraclavicular region two years before. The histological result was suspicious for tuberculosis with marked granulomatous lymphadenitis and caseous degenerated confluent necrosis, but tuberculin test and sputum sample examinations by microscopy and culture medium were negative. A CT without any proof of lymphadenopathy and pulmonary infiltration could not confirm this suspicion. In addition there were no other risk factors except her profession as a veterinarian, especially as she was neither an immigrant nor had long-termed stays in foreign countries. So no further treatment had been started at that time.\nAlthough the initial microbiological examination of the first aspirate being inconspicuous, a current Quantiferon-Test [] showed a positive reaction as an indication of a latent or active infection with Mycobacterium tuberculosis (M. tbc), Mycobacterium Kansasii, Mycobacterium Szulgai or Mycobacterium Marinum. X-ray of the chest showed an enhanced parenchymal change in both apical segments. A consequently performed CT of the chest and abdomen revealed the clinical sign of reactivated tuberculosis in the apical and posterior bronchopulmonary segments with nodular peribronchiovascular focus as a sign of open lung tuberculosis. Furthermore a second aspiration of the right hip joint confirmed M. tbc by microscopical analysis and bacterial culture. Gastric secretion examined by polymerase chain reaction (PCR) as well as bacterial culture and microscopy of the sputum showed an amount of M. tbc.\nThereafter by confirming TBC and open lung tuberculosis, the patient was treated with combination of oral Isoniazid, Rifampicin, Ethambutol and Pyrazinamid. She received physiotherapy with partial weight bearing of 10 kg.\nThree months later X-ray showed an increasing patchy transparency in the femoral head and neck as well as in the greater trochanter and acetabulum with clear progressive joint space narrowing in the corresponding parts of the hip joint (Fig. ). A complementary MRI with contrast medium five months after starting therapy showed active arthritis with progressive destruction of the right hip joint and a new appearance of liquid retention in the right acetabulum as a sign of tuberculous osteomyelitis (Fig. ).\nAfter seven months the microbiological examination of gastric secretion and sputum analysis did not detect open lung tuberculosis, so the oral therapy was reduced to double combination with Isoniazid and Rifampicin. In contrast to X-ray and MRI results with progressive destruction, the ROM of the right hip joint improved. The patient underwent reconstructive surgery by extirpation of the granuloma from the acetabulum and augmentation with autologous spongiosaplasty from the ipsilateral iliac crest. Microbiological and microscopic examination of intraoperative swabs and surface biopsies did not detect M. tbc or other pathological results.\nSix weeks after surgery, continuing physiotherapy and oral double combination, the patient was almost free of pain and ROM was improved. X-ray of the right pelvis revealed an unchanged result compared to the examination after operation without any progression of destruction and regular positioned autologous spongiosaplasty without dislocation. The physical load for the right leg was gradually increased and ROM was continuously improved.\nTen months after starting oral therapy, a CT of the thorax detected scars without nodular pathologic infiltrates or pleural effusion in both apical parts of the lungs.\nThe ROM of the hip joint in spite of the radiological findings with progressive sclerosis in the acetabulum and joint space narrowing (Fig. ) was satisfactory measured with 125° for flexion, 35° for abduction, 20° for adduction, 5° for internal rotation and 30° for external rotation. The patient was able to walk without crutches suffering only minor pain.
A 9-year-old Italian girl was referred by her pediatrician because her lower first permanent left molar was not present in her dental arch. Before coming to our attention a general dentist had suggested extraction of the element, after viewing the orthopantomograph (Figure ) in which the apical third of the roots showed a marked angulation with respect to the long axis of the root itself (Figure ). However, her pediatrician, consulted for a medical visit by her mother, hearing the proposal to extract a permanent tooth, advised her to consult an orthodontist, a dentist who specializes in orthodontics before the surgical extraction.\nShe was in good health, and her dental and medical history was unremarkable with only the usual childhood maladies.\nAn extraoral clinical examination disclosed a symmetrical face with balanced vertical thirds.She has a dental-skeletal class I with mandibular normal divergence, with no bad habits; her cephalometric values are all normal, even her lower and upper incisors are normal-inclined. Her profile was standard for Italian people. An intraoral examination revealed that her dental midlines were not concordant with each other and with her face and no mandibular shift was detected on closure. Except for some lower incisor crowding and deep-bite, the overall occlusion was fair with acceptable overjet (Figure ). The molar relationship was Class I at right and left sides. We performed an orthopantomogram (OPT) 1 year after her first visit to the other dentist and we noticed that the situation was even worse; the roots were all sizes and showed closed apexes, and the presence of bone above was increased with respect to the first evaluation (Figure ). After careful evaluation of this case, we decided to treat this impacted tooth orthodontically. Full mouth orthodontic treatment was suggested. Before the beginning of the therapy, the patient and her parents were informed about the difficulties of the treatment and the recovery of tooth. It was our goal to treat this case with a non-extraction orthodontic approach using upper and lower orthodontic appliances, while doing our best to correct the impacted tooth, maintaining the profile and reaching as good a final occlusion as possible.\nThe objectives of orthodontic treatment for this patient were to bring the impacted mandibular left first molar into her dental arch, level and align her arches, maintain her normal overjet, improve her overbite, and achieve a bilateral Class I canine and molar occlusion.\nAs the first step, a lingual arch was designed and cemented on her lower left deciduous molar and lower right first molar; the lingual arch shows an extension distal to the deciduous molar, to allow the extrusion of the retained first molar. The upper arch was prepared using a Schwarz plate with expansion to improve the dental arch contraction (Figure ). Before the bonding of the arch, surgical intervention was performed to place the orthodontic bracket on the impacted molar crown (Figure a). After the opening session, two buttons were bonded on the molar crown (Figure b). Three months after surgery, an OPT was taken as a control and this confirmed that the tooth was moving, as evidenced by the increase in the distance between the lower edge of her jaw and the roots of the impacted tooth (Figure ). Then, as the second molar was erupting, and the first molar was impacting against it, it was decided to wait for the eruption of the second molar before continuing the treatment. So the lingual arch was eliminated to avoid the mesialization of the second molar (Figure ). When the lingual arch was removed, the functional therapy in the upper arch was continued. After a year, we bonded the lower dental arch. We then positioned an open coil spring to increase the space for the molar. The molar was then ligated directly to the archwire (Figure ). Then, the molar was tied directly to the archwire with constant and light force. We used pre-informed brackets and straight archwires. In the first session we used a NiTi (0.014 inch) archwire; we then replaced it with a (0.016×0.022 inch) NiTi archwire. Approximately 6 months after treatment the tooth appeared in the dental arch (Figure ), and some weeks later perfectly extruded (Figure ). Criss-cross elastics were used to improve the intercuspidation. After approximately a year, the appliance was removed (Figure ). The total treatment time for this patient was 24 months. Retention was established with a removable plate. The post-treatment panoramic view showed that the roots of her teeth were well angulated and aligned. No apical root resorption was evident on a radiograph (Figure ). The orthodontic treatment allowed the correct placement of midline, overjet and overbite. The radicular anomalies of the impacted first molar could be due to the impact of the tooth near the lower contour of her mandible. Perhaps, if the treatment was made a year before, this anomaly could be less evident. The periodontium of the tooth during treatment showed some alterations of an inflammatory nature, which were treated with sessions of hygiene and good oral home health. One year post-treatment follow-up there was no obvious relapse (Figure ). No tooth morbidity is evident. Her gums are healthy, and the gingival attachment in her molar region is intact. She is satisfied with the treatment results [].
A 78-year-old socially isolated male with history of non-compliance to medical advice presented in a hospital after recent multiple falls at home. At the hospital, he was found to have a huge, ulcerative and bleeding mass disfiguring the right side of his face (). The patient stated that he had the mass on the right side of his face for many years, had a biopsy on the mass showing cSCC three years ago at the other medical facility but declined surgery or any treatments at that time (data was not available). Subsequently, a surgery consultation was made for biopsy and to get a surgical opinion to manage this neglected facial lesion. Biopsy confirmed cSCC. Computed tomography (CT) scan showed an 11×10×3 cm soft tissue mass arising from the right side of his face and temporal area. The mass invaded the myofascial plane of the right temporalis muscle, but not the facial bone (). There were several enlarged right submandibular and upper cervical neck lymph nodes. Positron emission tomography (PET) scan demonstrated hypermetabolic masses involving the right face and frontotemporal scalp with maximum standardized uptake values (SUVs) of 18 () and right neck level I–II lymph nodal station with maximum SUVs of 4.9 (). The patient again declined to receive extensive surgeries for this neglected cSCC on the right face. Then, he was referred for RT.\nDuring the radiation oncology consultation, a huge mass on the right side of his face with a foul-odor, necrotic and bleeding surface extending into the right-side eyelid, ear lobe and temporal scalp was noticed. He also presented weakness and trismus in the right face (). Motion and sensation in the left face were grossly intact. There was no palpable lymphadenopathy in the neck. He refused to make weeks of daily trips for definitive RT but would consider less than two trips. Thus, a radiation oncologist suggested cyclical hypofractionated RT also known as QUAD Shot to cSCC on the face and hypermetabolic nodes in the neck for local disease control and symptom palliation. To receive a QUAD Shot, the patient would need to visit the radiation oncology clinic for 2 consecutive days, which could be repeated every 3–4 weeks for at least three times depending on the tumor response and patient’s tolerability. Patient agreed to try the first QUAD Shot and wanted to decide further treatments thereafter.\nThe first QUAD Shot consisted of 14 Gy in 4 fractions, given twice a day, 6 hours apart, for 2 consecutive days, to hypermetabolic gross diseases in the right face and neck including right eyelid, right parotid gland, involved right masticate space, and right trigeminal nerve and facial nerve tracts to the skull base using an intensity-modulated RT. A 1.0-cm bolus was applied over cSCC on the right face to ensure the surface of the tumor would get the full prescription dose. Planning objectives required the planning target volume coverage of 95% to 110%. Radiation dose to the spinal cord, brain stem, and optic nerve/chiasm were limited to 9 Gy per QUAD Shot. For other organs at risk, the constraints were ‘as low as reasonably achievable’ ().\nTwo weeks after the first QUAD Shot, the patient noticed his cSCC on the right face became significantly decreased in size and bled less (), which motivated him to pursue additional RT. The subsequent QUAD Shots were planned on new computed tomography (CT)-simulation scans and were given to gross residual diseases. He successfully completed the second and third QUAD Shot every 3–4 weeks resulting in continuous shrinkage of cSCC on the face ( and , respectively). Two weeks after the third QUAD Shot, he returned to the radiation oncology clinic for follow-up. While there was mild ptosis, he was able to open his right eye. Vison and eyeball movements were grossly intact. Improved trismus and weakness in the right face were also observed. Patient experienced slightly decreased salivation and hair loss in the right lateral scalp, eye brow, and jaw. Although the previous three cycles of QUAD (QUAD Shot × 3) Shots substantially decreased the neglected cSCC on the right side of his face, there was still residual painful induration with focal necrosis and foul-odor around the posterior and inferior parts of the tumor bed (). An additional QUAD Shot was requested by the patient to alleviate the residual painful induration in the tumor bed.\nBefore considering further local treatment, he underwent repeat PET-CT scan revealing a significant decrease in size and metabolic activity (SUV of 4) in the right facial tumor (), no metabolic changes in the right neck lymph node and no disease elsewhere. Subsequently, a surgical option for the residual disease was sought. However, surgeons considered him to be unfit for extensive surgery, then recommended him for further local RT if possible. After discussions regarding the risks and benefits from additional RT, he elected to pursue the fourth QUAD Shot.\nThe fourth QUAD Shot was planned on new CT-simulation scan with 9 MeV-electron with 1.0 cm bolus covering the surface of the tumor bed (). The accumulated doses to the optic nerve, optic chiasm, brain, and spinal cord were less than 36 Gy from all QUAD × 4. He successfully completed the fourth QUAD Shot. One month after the last QUAD Shot, he reported no pain or foul-odor in the tumor bed, which positively affected on his quality of life and motivated him to pursue a palliative maintenance immunotherapy (cemiplimab at a medical oncologist’s discretion). On his last clinic visit 12 months after the first QUAD Shot, the patient demonstrated a restored face with minimum facial weakness and regenerating skin around the tumor bed () without local disease progression in repeated PET-CT scan (, ).\nInstitutional Review Board waived a board review on the case report. The patient has given signed consent for reporting this report.
A 53-year-old nondiabetic and nonhypertensive female patient who was having an insidious onset and gradually progressive weakness of all four limbs for past two months along with decreased sensation in both lower limbs and tingling sensation in all four limbs now presented to us with development of retention of urine for the last four days. Two months back she had a preceding history of low grade fever for about one week which subsided spontaneously but she developed a persistent dull aching neck pain along with a gradually progressive quadriparesis. She had no history of anorexia, weight loss, cough with expectoration, hemoptysis, or any lump elsewhere in body. Examination of general survey was unremarkable except for the presence of mild pallor. Neurological examination revealed grade 3 power in both lower limbs and grade 4 power in both upper limbs, spasticity of all four limbs with exaggerated deep tendon reflexes in all 4 limbs, and bilateral extensor type of plantar response and hypoesthesia of both lower limbs but there was no definite sensory level. There was mild hepatosplenomegaly.\nHer routine blood investigation revealed a hemoglobin level of 8.1 g/dL, total leucocyte count was 8,000/mm3, and ESR (erythrocyte sedimentation rate) was 42 mm in 1st hour. Blood sugar, urea, creatinine, and liver function test were within normal limit. She was found to be HIV (human immunodeficiency virus) negative. Her chest X-ray showed an expansile lesion involving right 4th rib (). MRI of spine revealed altered marrow signal intensity with erosion of C5 to D1 vertebrae. There was intraspinal extension of the disease process with elevation of posterior longitudinal ligament between C5 and D1 levels causing compression of thecal sac and spinal cord. The intervertebral discs were of normal height and signal intensity (). MRI of dorsal and lumbar spine showed altered marrow signal intensity with abnormal soft tissue lesion involving D6, D7, D9, D10, and L2 to L5 vertebrae and sacrum. Pedicles at D10 and L5 level are also involved. Intraspinal extension with thecal sac compression is seen at the level of D6 vertebrae and in sacrum. Large pre- and paravertebral soft tissue element are seen at dorsal level and presacral space (Figures and ).\nAt this time we had a differential diagnosis of some metastatic disease process or tuberculosis. We examined the patient once again and this time we found a small breast lump involving upper outer quadrant of right breast which was painless, mobile, and firm in consistency which was unnoticed by the patient. This raised the possibility of carcinoma of breast with metastasis. We went for USG (ultrasonography) of breast which showed a hypoechoic, heterogeneous mass lesion involving right breast measuring 3.5 × 2.3 cm () with echogenic foci within it. Few enlarged axillary lymph nodes are noted on right side, the largest of which was 2 × 0.8 cm USG of left breast was normal. Next, we went for CT (computed tomography) guided FNAC (fine needle aspiration cytology) from paravertebral collection and USG guided FNAC from right breast lesion () both of which showed degenerated inflammatory cells in a necrotic background without any presence of malignant cell or granuloma. However, ZN (Ziehl-Neelsen) stain of both lesions showed presence of acid fast bacilli () which was suggestive of tubercular lesion.\nPatient was put on antitubercular drug for 1 year (initial 2 months, rifampicin, isoniazid, pyrazinamide, and ethambutol, followed by rifampicin and isoniazid for the next 10 months). After treatment her quadriparesis improved completely with resolution of breast lump and rib lesion.
Our patient, an 18-year-old female, was referred to neurology with a diagnosis of schizoaffective disorder after a possible first-time seizure. She had experienced previous mood lability and attentional impairments as a teenager and had been treated for both during that time. However, she had been off medications for 5 years and had been doing well in the recent past. Grades and athletic functioning were above average, and she played three sports in high school and was in mainstream classes. Her family history was notable for a history of maternal depression, psychogenic nonepileptic seizures, and anxiety, as well as a maternal grandmother with a reported history of schizophrenia and bipolar disorder. Eight months prior to the patient's referral to neurology, she experienced a self-limited illness characterized by diarrhea, headache, and vomiting. This was managed at home, although she was seen by her primary care provider and diagnosed with a viral gastroenteritis. Approximately one month later, she began to experience insomnia, mood lability, and weight loss. Memory issues ensued, and the parents reported that she began to frequently lose objects and had difficulty remembering bus routes. Personal hygiene suffered, and after 4 months of worsening symptoms, she began to experience weekly episodes of urinary incontinence. There were no other autonomic manifestations. Throughout the course of the subacute illness, she experienced a “tremor” of the left upper extremity when attempting to manipulate or reach for objects, as well as dragging her left foot when ambulating. She received ongoing psychiatric treatment and monitoring, and her movement phenomenon was attributed to a psychogenic cause, and a diagnosis of likely schizoaffective disorder was made. After 7 months of symptoms, she experienced a spell, characterized by clonic movements of the left hand, followed by tonic stiffening of her entire body, with right sided head and neck deviation, lasting 30 seconds, followed by confusion and sedation. She recovered to baseline after 30 minutes. After this episode, she was referred to neurology. On initial presentation to our service, her spell was attributed to a first-time seizure by history. Mental status examination demonstrated a distracted and labile demeanor. Cognition was impaired on both bedside and formal neuropsychological testing with impairments in memory, attention, and reasoning. Neurologic examination demonstrated left sided dysmetria as well as gait disturbance arising from left lower extremity coordination impairment. MRI of the brain revealed increased FLAIR signal of the left hippocampus. No cerebellar or contralateral brain findings were identified as potential substrate for her unilateral ataxia. Electroencephalogram was normal. The combination of her ataxia, cognitive decline, and incontinence prompted an extensive laboratory evaluation for systemic conditions as well as inborn errors of metabolism. Spinal tap was performed, with testing for autoimmune and inflammatory conditions. Testing was positive only for the presence of NMDA receptor antibodies in the spinal fluid (NMDA-R Ab IF Titer Assay, CSF Positive 1 : 4 and reference < 1 : 2, Mayo Clinic Laboratories) with no evidence of active infection or inflammation. Extensive evaluation for a neoplastic substrate, specifically ovarian teratoma, was negative. Because of the extended course experienced by the patient she was started on an aggressive regimen of immune modulation, including concurrent intravenous immunoglobulin, IV methylprednisolone, and rituximab. Although her parents report that psychiatric symptoms were improving after 3 months of treatment, she continues to require medical treatment for her psychiatric manifestations and has been unable to return to school or employment. Additionally, her movement abnormalities persist.
A 60 year old male, who had had an open fracture of the right lower leg accompanied with the vascular injuries of the anterior tibial, posterior tibial, and peroneal artery by a traffic accident. Mangled Extremity Severity Score at the time of presentation was eight points. Although the posterior tibial artery that was repaired primarily resulted in the obstruction with thrombus, the tissue distal to the injury survived by the collateral vasculature [Figure and ]. After radical debridement of the necrosed tissue, there was a large open defect with a certain loss of bone, including the plafond in lower third of the lower leg []. Continuity of the tibial nerve was identified with severe attenuation, and both the superficial and deep peroneal nerves were lost. Since there was severe soft tissue injury proximal to the popliteal fossa for exploration of the proximal parts of the major arteries, secondary reconstruction of major arteries using arteriovenous loop was impossible and avoided. After explanation to the patient regarding the respective results and complications that could be expected with below knee amputation (BKA), and with difficult reconstruction for preservation respectively, he desired to retain the injured lower leg.\nAs the first step for reconstruction, the defect was augmented by a cross leg free rectus abdominis musculocutaneous flap as to achieve healthy recipient site for subsequent bone graft. The deep inferior epigastric artery and its venae comitantes were anastomosed to the posterior tibial artery and its venae comitantes of the normal opposite limb. The legs were immobilized by external fixator []. Three weeks later, the pedicle was severed and then external fixator for immobilization of the both legs was removed.\nAfter 8 weeks for rehabilitation to the both legs, the distally pedicled fibula osteocutaneous cross leg flap was planned. Prior to flap harvest, vascular delay to the peroneal artery at the bifurcation from the posterior tibial artery was administered []. Two weeks later, contralateral distally pedicled fibula osteocutaneous flap was elevated. The fibula was divided into two parts, and then the double-barreled fibula 12 cm in length each inserted into the bone defect between the proximal remnant of the tibia and the talus [Figure and ]. The both legs were immobilized by external fixator again. Three weeks later, the vascular pedicle was divided and then the external fixator was removed. Because the cutaneous flap put between both legs during the immobilization period resulted in necrosis due to excessive pressure, flap coverage of the grafted fibula with 3rd cross leg flap was mandatory.\nFour weeks after previous operation, the exposed bone was covered with the proximal based medial sural cross leg flap [Figure and ]. The both legs had been immobilized by external fixator for 3 weeks, and then the pedicle was divided and the external fixator was removed []. After a course of consecutive three cross leg flaps, neither contralateral knee nor ankle contracture remained due to an adequate range of motion (ROM) training in the intervals of each immobilization periods [Figure and ].\nOver 3 years after injury, including almost a year of the total period of substantive treatment, the patient can walk on crutches with patella tendon weight bearing orthosis [Figure and ]. No limitation of ROM was identified on both limbs except the fused ankle of the affected side. Deep sensation of the foot were preserved. Bone and functional results using Association for the Study and Application of the Method of Ilizarov scoring system were both good.
The patient, a 40-year-old male, presented to our clinic with a history of right knee pain predominately along the medial aspect. His medical history includes prior examinations and treatments by another physician for right knee pain which provided minimal relief. The patient’s pain originated 2 months prior, wherein he experienced moderate pain that became sharp when squatting and bending, as well as stiffness while sleeping. The pain subsided with extension of the extremity, but loading of the joint re-aggravated the pain. Physical examination showed good range of motion (ROM), slight patellofemoral crepitus, and no meniscal signs along the medial joint line.\nMRI with no contrast was obtained, which demonstrated full-thickness chondral fissuring of the lateral patellar facet, mild abnormal signals of the proximal patellar tendon and Hoffa’s fat pad, and intact ACL and posterior cruciate ligament (PCL). The patient was treated with an ultrasound-guided injection of 2 cm3 of 1% lidocaine without epinephrine and 1 cm3 of Kenalog-40. He experienced immediate pain relief in his knee and was scheduled for clinical follow-up in 6 weeks.\nThe patient first presented to our office approximately 6 weeks following last examination and treatment from previous physician. He presented with medial pain of the right knee that was sharp but minor in nature. The pain was predominately activity-related and the patient was able to ambulate on his own. The patient stated that he was active as a bodybuilder and was a professional fighter for the prior 12 years. Our physical examination showed an antalgic gait with the patient favoring the affected extremity. The patient also exhibited minimal focal tenderness below the medial joint line and a negative McMurray test. Patient’s ROM was 0–120°+ and the knee was stable in reverse pivot and varus and valgus stress. When positioned in deep flexion, the patient experienced pain along the medial side of the knee. I personally reviewed the previous MRI the patient received while under previous physician’s care. Upon examination and imaging review, the patient was prescribed a lidocaine cream for pain management, and Pilates for stretching exercises were recommended. The patient was subsequently scheduled for follow-up in 6 weeks.\nThe patient returned to our office for 6-week follow-up visit. Approximately 2 weeks prior, he had experienced an injury to his right knee while teaching a kickboxing class, which had caused severe pain. The pain was severe enough in nature to cause difficulty and discomfort while sleeping. He had applied the previously prescribed lidocaine for the pain, but it offered only little relief to the knee. A physical examination was performed, which resulted in similar results as the previous examination—antalgic gait, minimal tenderness along medial joint line, medial pain in deep flexion, and no pain when in varus or valgus. We discussed the patient’s injury history and it was our recommendation to perform an in-office diagnostic arthroscopy (mi-eye 2™) due to the continued pain and discomfort with a negative MRI. The patient agreed with this recommendation and was scheduled to return for mi-eye 2 procedure in approximately 2 weeks with the goal to visualize pathology and develop a treatment plan.\nUpon return for the in-office arthroscopy, the patient was positioned lying down with a “bump” placed under the right knee for flexion (). The procedure was performed in a sterile environment, and the knee portals were aseptically prepared. The patient was given an analgesic injection of 1% lidocaine (5 cm3). The mi-eye 2 was inserted into the right knee and immediately—within approximately 20 s—the intact ACL was visualized () and a tear of the mid-body of the medial meniscus was identified ( and ). Through the duration of the procedure, minimal saline was needed—approximately 4 cm3. Following visualization, we discussed the treatment options with the patient, who ultimately opted for surgical intervention.\nThe patient was scheduled for surgery 2 days later to address the meniscus tear visualized with mi-eye 2. In addition to the mid-body tear of the medial meniscus, surgical findings included grade II changes to the medial and lateral edges of the patella and radial tear of the posteromedial aspect of the medial meniscus. The mid-body meniscal tear previously visualized using mi-eye 2 was confirmed ( and ) and the meniscus was debrided to a stable rim (). The patient was discharged following recovery from anesthesia and allowed for weight-bearing on his right knee as tolerated. He was scheduled for follow-up 3 weeks post-operatively.\nFollowing 2 weeks of surgery, the patient reported good functional motility and resolution of medial-sided joint pain.
This is a 26-year-old man who experienced right lower limb weakness for 2 years and the weakness exacerbated in last half year. He visited the second affiliate hospital of the Zhejiang University School of Medicine. A physical examination indicated the result of the straight leg raising test was positive and also muscle atrophy. The muscle power of the right lower limb had decreased to grade 3. There was no sensory impairment. Magnetic resonance imaging revealed an intraspinal extradural tumor over T10 to L3 (Fig. ). It appeared to be a spinal extradural arachnoid cyst (SEAC). To confirm whether the fistula existed between the subarachnoid space and arachnoid cyst, and to localize the position of the fistula, we performed a real-time technique. First, we injected contrast medium into the cyst under fluoroscopy. After 1 h the follow-up computed tomography (CT) was administered, and it revealed there was no contrast-infiltration into the subarachnoid space (Fig. ). We then extracted about 20 mL of fluid from the cyst. The follow-up magnetic resonance imaging on the same day indicated the cyst did not become smaller (Fig. ). A “one-way valve” fistula was suspected such that cerebral spinal fluid could pass into the cystic space from the subarachnoid space but could not flow in the opposite manner. Therefore we designed a procedure to localize the fistula. We penetrated two needles into the cyst and subarachnoid space separately in the L3/L4 level under digital subtraction angiography (Fig. ). Pending the fluid drained through those two needles, we injected 10 mL of contrast medium slowly into the subarachnoid space and a little contrast medium infiltrated into the cystic space horizontally at the T12/L1 level. We then administered high resolution computed tomography (HRCT) immediately to confirm the position in the axial plane. The HRCT revealed a funnel-shaped enhancement between the lower edges of the T12 body and the left nerve root, and this is the accurate position of the fistula (Fig. ).\nAfter general anesthesia and endotracheal intubation, the patient was placed in prone position. A fluoroscopy was used to determine the correct operative level, and a longitudinal incision was made 5 cm off midline at the T12 level. Muscle was dissected layer by layer, and a spinal process and left lamina were exposed. The left lamina was partially ground by cutting burr and then removed along with ligamentum flavum by Kerrison punch. After that, the cyst was exposed. Under a microscope, the cystic wall was fenestrated and it then drained off clear cystic fluid (cerebral spinal fluid). After partial excision of the cyst and evacuation of cystic fluid, a spinal endoscope (SPINENDOS, Germany) was maneuvered into the space and the fistula was detected (Figs. , ). The fistula was detached from the arachnoid membrane and was ligated with a 7–0 Vicryl purse string suture. Regional leakage from the repaired site was noted. An anastoclip was then used to close the fistula (Fig. ). Pulmonary pressure was elevated by ventilator (valsalva maneuver) to check the leakage and there was no more leakage. Hemostatic matrix and gel [Fibrin sealant kit (human), (Shanghai RAAS Blood Products Co, Ltd., Shanghai, China)] were utilized at the local region. The postoperative diagnosis was a thoracolumbar extradural arachnoid cyst. The pathological report revealed an arachnoid cyst (Fig. ). The symptoms improved on postoperative day 2. His lower limbs regained strength with limited dorsiflexion of the right foot.\nThrough the 3-month outpatient department follow-up, there was no more numbness or weakness of his right lower limb. The motion of dorsiflexion also improved. The 3-month follow-up magnetic resonance imaging revealed no recurrence of the previous lesion and no spinal cord compression (Fig. ).
A 39-year-old woman (G10P2) whose last delivery was by cesarean section at full term in August 2016 was admitted to our emergency room complaining of sudden abdominal pain and irregular vaginal spotting in November 2018. In 2014, this patient presented with an invasive molar pregnancy (stage I: risk score of 5) and was treated with two cycles of 5-day single-agent methotrexate. The HCG level was decreased to 16 mIU/mL after which the patient was lost to follow-up.\nHer abnormal uterine bleeding had lasted for almost 3 months. She occasionally had lower abdominal pain for 7 days before admission and was urgently hospitalized in November 2018 with severe left abdominal pain, nausea, and transient syncope without a history of trauma. She was not using any effective contraception. On a physical examination, her vital signs were normal, except for a heart rate of 108 beats/minute. She was pale and had generalized tenderness of her abdomen with guarding. The tenderness was so severe that the uterus and adnexa were not clearly felt on a pelvic examination. Blood tests showed anemia. Her β-HCG level was >15,000 mIU/mL, which was the upper limit of the assay used in the emergency laboratory. Ultrasonography revealed a large amount of free fluid in the abdomen and rupture of a 6.75 × 7.27-cm mass on the surface of the spleen was suspected Several smaller solid masses in the liver, and a normal adnexa and uterus were observed (). An emergency computed tomography (CT) scan showed the same findings. Culdocentesis was performed immediately and yielded nonclotted blood. The differential diagnoses were considered as rupture of splenic metastases secondary to choriocarcinoma and rupture of an ectopic pregnancy.\nIn view of the life-threatening intraperitoneal hemorrhage, laparoscopic exploration was performed in the Emergency Department. There had been spontaneous rupture of the upper splenic mass with active bleeding and approximately 3000 mL of free blood was aspirated from the peritoneum. Conversion to open splenectomy was performed to control the bleeding more quickly.\nA 5-cm-long split covered by the omentum was found on the surface of the upper pole of the spleen. Scattered tumor metastases were observed on the omentum. Soft lesions with sizes of 1 and 2 cm were palpated in the right lobe of the liver. No abnormality was found in the uterus or adnexae. We decided to perform splenectomy and resection of omental metastases. A histological examination showed metastasis of the spleen and omentum secondary to choriocarcinoma ().\nOn the 3rd, 6th, and 10th days after surgery, β-HCG levels were 177,400 mIU/mL, 231,000 mIU/mL, and 1,210,630 mIU/mL, respectively. The patient had an uneventful recovery, and her lung CT and brain magnetic resonance imaging findings were normal ().\nAccording to the International Federation of Obstetrics and Gynecology (FIGO) 2000 scoring system, her condition was diagnosed as stage IV:20 choriocarcinoma, which predicted an ultra-high–risk for mortality and chemoresistance. She received two lines of multi-agent chemotherapy. The first regimen was floxuridine, actinomycin D, etoposide, and vincristine (FAEV). The patient received seven cycles with a rise in β-HCG levels. Second-line multi-agent chemotherapy was then provided with five cycles of etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine (EMA/CO) therapy.\nPartial liver resection was performed after two cycles of EMA/CO chemotherapy because follow-up CT showed no significant reduction in the size of the tumor in the right lobe of the liver. EMA/CO chemotherapy was continued for three more cycles and then stopped owing to the patient’s rejection at which time β-HCG levels had dropped to <5 mIU/mL three times. However, 2 months after completion of EMA/CO therapy, β-HCG levels began to rise again. With a subsequent rise in β-hCG levels, the patient received immunotherapy (programmed cell death protein 1 inhibitor, camrelizumab) and targeted therapy (apatinib). In the 20th month of follow-up, a laparoscopic partial lung resection was performed for new lung metastases. The serum β-HCG level at this time was 250 mIU/mL. The pathology of the liver and lung lesions was consistent with metastasis of choriocarcinoma. The patient received two lines of multi-agent chemotherapy, underwent surgery of the liver and lungs, and received immunotherapy and targeted therapy. Up to the time of writing this report, the patient had been receiving HCG administration of approximately 1 mIU/mL for 9 months ().
The patient was a 12-year-old male who had his first seizure attack at the age of 10, and had four types of epileptic seizures. The first type of seizure was tonic axial seizures characterized by flexion of the neck and body and the extension of four extremities for several seconds. The tonic seizures could last several seconds. This type of seizure occurred predominantly at night and the frequency was about 4–5 times per week. The second type was atypical absence seizures, which manifested as a sudden loss of consciousness and the resuming of normal activity right after the seizure. This type of seizure could last about 15 s and the frequency was 4–5 times per week. The third type was the myoclonic seizure, presented as prominent myoclonic jerks of bilateral upper limbs. The frequency of this type of seizures was 2–3 times per day. The last type of seizure was the generalized tonic-clonic seizure, which could last about 5 min. The mean frequency of this type of seizure was <1 time per week.\nThe patient was a full-term infant with no history of perinatal asphyxia, head injury, encephalitis, and febrile convulsions. His family history was unremarkable. He had a mild degree of intellectual impairment and learning disability after the onset of the disease. The physical examinations were normal. Auxiliary examinations, including blood routine examination, serum biochemical examination, thyroid function, autoimmunity antibody, and blood ammonia, were normal. The electrocardiogram was normal and the QT interval (QTc) was 372 ms. There was no lesion on the brain MRI. The Wechsler Intelligence Scale showed a borderline cognition impairment. The electroencephalogram (EEG) before treatment showed that there was a large number of 3 to 5 Hz slow waves with middle and high amplitude in the anterior region in the background. Fast rhythms bursts of 16 to 20 Hz and multiple-spike-and-slow-waves of 0.5 to 1 Hz were observed during the sleep period. Slow spike-and-slow-waves of 1.5 to 2.5 Hz were observed during awake time ().\nThe patient was diagnosed with Lennox-Gastaut syndrome after considering his multiple types of epileptic seizures, mental retardation, and typical electroencephalographic features. He was refractory to a multiple anti-epileptic drugs treatment, including sodium valproate (8 mg/kg/day), levetiracetam (50 mg/kg/day), clonazepam (0.0375 mg/kg/day), topiramate (3.75 mg/kg/day), and lamotrigine (2.5 mg/kg/day). Whole exome sequencing (WES) identified a novel heterozygous KCNT1 mutation (chr9:138649026; c.625C>T; p.Arg209Cys) inherited from his father. This missense mutation was highly likely to cause the dysfunction of the KCNT1 channel and led to a gain-of-function phenotype. This alteration had not been previously reported and was not found in the ExAC database (), and was predicted to be likely pathogenic.\nThis study was approved by the human research ethic committees of Xuanwu hospital capital medical university. Written informed consent was obtained from all participants and guardians of minors for the quinidine therapy and the publication of this study. Additive quinidine therapy to our patient was initiated at 12 years of age. The doses of the above anticonvulsants remained unchanged. In the month before quinidine therapy, the patient had 16 tonic seizures, 12 atypical absence seizures, 10 myoclonic seizures, and 1 generalized tonic-clonic seizures.\nAfter admission, the quinidine therapy was initiated with 5 mg/kg/day in 3 divided doses under electrocardiographic (ECG) monitoring. The QTc ranged from 361 to 415 ms, with an average of 378 ms (the normal limit of QTc is within 450 ms). After 1 month of treatment, the dose of quinidine was titrated to 10 mg/kg/day and he had 13 tonic seizures during this month. The dose was maintained during the following 2 months. QTc was in normal range (391–436 ms). There was also no other adverse effect of quinidine. At the fourth month, the dose of quinidine was increased to 13.75 mg/kg/day in 3 divided doses. The frequency of tonic seizures ranged between 4 and 6 times per month. The mean QTc interval was 383 ms. As no adverse effects were experienced, the dose of quinidine was maintained to 13.75 mg/kg/day during the following 4 months. The patient had 4 tonic seizures per month. The frequency of tonic seizures subsided by 75% (), whereas the frequency of the other types of seizures was not reduced significantly.\nVideo EEG (VEEG) was performed regularly during treatment and the number of epileptic discharges was counted by four expert technicians who had not seen the patient's clinical information. One slow spike-and-wave complex or one episode of paroxysmal fast rhythms in EEGs were counted as one epileptiform discharge. 1323 epileptiform discharges were recorded during a 24-h video EEG before the treatment of quinidine. We subsequently applied the 24-h VEEG every 3 months to evaluated the efficacy of the quinidine treatment. The total number of epileptic discharges was 512, 652, and 598 in the next 3, 6, and 9 months, respectively. The epileptiform discharges decreased by 54.80% ().
A 65-year-old male presented with a three month history of progressive left hip pain. His past medical history was noteworthy for a non-ischemic cardiomyopathy for which he underwent a heart transplant ten years prior to presentation. He had a history of osteoarthritis for which he had undergone bilateral hip replacements complicated by a left prosthetic hip infection with Cutibacterium acnes. His past history was also positive for rheumatoid arthritis, hypertension, and type 2 diabetes.\nThe first stage of his left hip revision after his first episode of prosthetic joint infection with C. acnes occurred pre-heart transplantation and he was treated with 6 weeks of vancomycin following hardware explantation. His second-stage hip revision occurred over a year later, following his heart transplant. His post-transplant course was otherwise uncomplicated, without any episodes of graft dysfunction, rejection, or infectious complications over the previous nine years. He was maintained on tacrolimus and mycophenolate mofetil since his transplant without recent corticosteroid exposure.\nWith regard to his present symptoms, along with the pain, he noted occasional night sweats. He reported receiving 2 hip injections for the pain (one into his hip joint and another into his trochanteric bursa, both on his left side) by his outpatient orthopedic doctor about 2 weeks prior to presentation that provided temporary relief. Sterile precautions were reportedly observed during in-clinic procedures. Following the injections, the patient noticed a progressive fluctuant area swelling of his left lateral thigh and hip. Aspiration of that area 4 days prior by his outpatient orthopedist yielded purulent-appearing fluid that was Gram stain negative for organisms, with no growth seen either on aerobic or anaerobic cultures. Three days prior to admission, he underwent hip arthrocentesis that showed cloudy fluid with 65,000 white blood cells per microliter and a differential of 95% polymorphonuclear neutrophils. Gram stain was negative, and no crystals were seen. Culture of the synovial fluid was later finalized as no growth. However, prosthetic joint infection was suspected and so the patient was admitted for surgery.\nThe patient was an active dog breeder (Yorkies, Maltese, and Morkies), with over 10 dogs at home at any one time, and had recently participated in the birth of several puppies. There was no history of illicit or injection drug use. He also noted being scratched on his legs by the dogs in the past, but could not recall any recent scratches or bites recently.\nOn admission, he was well appearing and comfortable at rest. All vital signs were normal and he was afebrile. He noted mild pain with passive and active movement of his left hip, however, he was able to ambulate. Oropharyngeal exam was unremarkable with good dentition. His bilateral shins had evidence of previously-healed scratches. On the lateral aspect of his thigh was a tender egg-sized swollen area of fluctuance and erythema, without increased warmth or obvious drainage or sinus tract.\nLaboratory evaluation was notable for a peripheral white blood cell count of 6300 per microliter. Erythrocyte sedimentation rate and C-reactive protein levels were 63 mm/hour and 5.4 mg/dL, respectively. Hip X-ray did not show loosening or disruption of his prosthesis. He did not receive any antibiotics prior to surgery. Two sets of pre-operative blood cultures were drawn and finalized as no growth. Medications at the time of presentation included methotrexate (2.5 mg daily), mycophenolate mofetil (250 mg twice daily), and tacrolimus (1 mg twice daily). His diabetes mellitus was well-controlled with dietary changes and his most recent hemoglobin A1c measurement was 6.2%.\nThe area of the suspected abscess the lateral thigh was incised and drained. The proximal prosthetic body, femoral head, and acetabular liner were all removed, the joint was irrigated and debrided, and hardware was replaced in a one-stage fashion. Vancomycin and tobramycin-containing beads were placed. Intraoperatively, frank purulence within the joint was not observed. Five separate tissue specimens were sent for bacterial, fungal, and mycobacterial culture. Post-operatively, vancomycin and ceftriaxone were begun empirically pending further culture data.\nApproximately 2-3 mm of each tissue submitted was embedded whole in fungal media. The remaining tissue from each site was ground in 1 mL Tryptic Soy Broth. Mycobacterial, fungal, and bacterial growth media were inoculated with ground tissue suspension. Gram, Acid Fast, and Calcofluor White stains were prepared from touch preps of intact tissue and ground tissue suspensions. All stains of tissue were negative for organisms. Bacterial cultures were incubated at 35 °C in a 5–10% CO2 incubator for aerobic cultures and in the BD BBL GasPak anaerobic pouch for anaerobic cultures. After four days of incubation, tissue cultures collected from the left hip (one of 5 total submitted) grew small, translucent, spreading colonies from the anaerobic blood agar incubated under anaerobic conditions. Gram stain of the colonies demonstrated spiral-shaped gram-negative rods (Fig. ). Matrix-assisted laser desorption time of flight (MALDI-TOF) mass spectrometry yielded and identification of Anaerobiospirillum succiniciproducens, which is currently unclaimed in the bioMerieux Vitek-MS database. Therefore, 16 s ribosomal RNA gene sequencing was performed, with the best matches using NCBI Blast Nucleotide Sequence Database to Anaerobiospirillum succiniciproduces and Anaerobiospirillum species with > 99% query coverage and identity. Ultimately, the laboratory report was released as “most closely resembles Anaerobiospirillum species; Identified by DNA sequencing.” 16S sequencing was not performed on any other samples including blood.\nMycophenolate was held temporarily in the setting of infection. Following identification of the organism, vancomycin was discontinued and ceftriaxone (2 g daily) was continued to complete 6 weeks total of antibiotic therapy. He tolerated treatment well to date, without evidence of recurrent infection.\nSaliva and perirectal anaerobic cultures of two of the patient’s dogs (both Maltese) were performed using flocked swabs submitted in anaerobic agar gel transport media, and cultured on standard anaerobic media. No organisms resembling Anaerobiospirillum spp. were isolated.\nFollow up 2 months later was unremarkable for relapse of infection, after completion of intravenous antibiotics,.
The patient was a healthy 25-year-old man with a one-year history of right ankle pain following trauma.\nHe had met a car accident while walking. One year earlier, he had undergone open reduction and internal fixation on his right ankle for fracture at another hospital. A tibia diaphysis spiral fracture was fixed by the anterograde intramedullary nail with infra-patellar approach. An ankle malleolar fracture was fixed by the locking plate and cannulated cancellous screws with direct lateral and medial approach. He had finally consulted us because of worsening ankle pain while walking. On physical examination, there was tenderness in the anteromedial joint space of the right ankle. Slight ankle swelling was noted. Dorsiflexion of the right ankle was 10°, similar to that of the left ankle, but plantarflexion was restricted to 38°, compared with 60° on the left, but the ankle instability test was negative.\nThe first three months, even though we performed intra-articular injections, arthroscopic synovectomy for osteoarthritis, and the fixation implant removal in order to release implant irritation, his ankle pain persisted.\nThe AOFAS ankle score at that point in time was 50 . Radiographs showed moderate narrowing of the ankle joint and forward displacement of the talus (-A, B). Computed tomography of the right ankle showed an osteochondral defect on the anterolateral surface of the distal tibial plafond (). This was diagnosed as progressive osteoarthritis caused by an osteochondral defect on the anterolateral surface of the distal tibial plafond, and surgical repair of the osteochondral defect was recommended. Three months later, the osteochondral graft was performed on the patient's right ankle. The patient was placed in the supine position under general anesthesia. The lower extremity was prepared and draped in the standard sterile fashion. We inserted the 2.0 mm K-wire in his right calcaneus, then skeletal traction was done in order to open his right ankle joint space if necessary. Next, 10 mL of fluid was injected intra-articularly to distend the ankle. The anterolateral ankle arthroscopy portal was established in the routine fashion, and global arthroscopy was performed. The osteochondral defect was identified about the anterolateral plafond which site was enthesis of the anterior tibiofibular ligament (), and osteochondral lesions of the tibia and talus were identified. An anteromedial portal was established. The osteochondral lesions were probed, and the osteochondral lesions were diagnosed as grade 2 according to the classification of the International Cartilage Repair Society . We performed multiple OAT in the anterolateral plafond at the anterolateral wall with extension of the anterolateral portal. The anterolateral osteochondral defect was measured 9 mm × 20 mm (). The plug donor site was the lateral corner of the patellar groove in the right knee joint. Under direct vision, we harvested 2 bone plugs from the normal bone deeper than the osteochondral defect for the preparation of the recipient site in order to accept the osteochondral plugs from the donor site in maximum plantarflexion. Then, the dilator was interpositioned into the recipient site, and a radiograph was taken to calculate the inclination angle between the dilator and tibial plafond. Therefore, we thought that the smooth surface was obtained by the inclination of the grafted plugs. According to the angle required, two 8-mm osteochondral autograft plugs ramped 35° and 45° were harvested from the donor site (), and transferred into the anterolateral plafond at an angle to re-contour the articular surface and make it smooth. Both harvest and transplantation of osteochondral autograft plugs were performed by open approaches for transplantation in the exact slope. After this procedure, the surface of the anterolateral wall of plafond was almost completely smooth (). The depth of these plugs was 20 mm, deeper than the osteochondral defect. It was confirmed that the deepest area of the plugs consisted of a macroscopically normal bone. We used press-fit technique in order to stabilize the osteochondral plugs. Stabilization of the osteochondral fragments relied on tight interference with the host bone. In case of difficulty with press-fit technique, a screw made of polylactic acid and hydroxyapatite was prepared, but it was never used.\nPostoperatively, the foot was placed in a short leg cast, and the patient was advised to ambulate non-weightbearing for a 4-week period. The patient had an uneventful postoperative course, and a range of motion exercises were initiated immediately after cast removal at 4 weeks, with partial progressive weightbearing allowed during the next 4 weeks. The patient was able to return to standing work 10 months after surgery. The most recent magnetic resonance imaging scans at 2 years after surgery showed that the cartilage in the anterolateral joint surface was almost intact, and there was no osteonecrosis (). At 3 years the post-operative X-rays of the ankle joint depicted a larger osteophyte, compared with the pre-operative situation, in the anterolateral corner of the right tibial plafond. No forward displacement of the talus was evident on the lateral view of the right ankle joint, while the respective joint space was preserved (). On the latest physical examination, the patient complained of mild pain in his right ankle while walking, but could walk for 30 min. Dorsiflexion of the right ankle was 0°, compared with 10° on the left, and plantarflexion was 30°, compared with 60° on the left. The postoperative AOFAS ankle score was 80. The patient remained asymptomatic with respect to the donor sites of the right knee. He was able to return to carpenter's work on a five-day week, but remained with minor ankle pain on standing up and walking for more than half an hour over the 3-year observation period.
A 25-year-old man was referred to vascular medicine clinic because of pain in the left calf while running, which had been occurring for the past 4 years.\nThis patient practiced almost daily physical activity that involved both combative and running (over 8 h per week), and he competed at a regional level (with a personal best of running of 36min12s/10000 m). In 2012, the patient had reported left calf pain when running, forcing him to stop. He described a calf swelling sensation that gradually increased with the intensity of running. While in the past he could easily run 15–20 km, the patient reported at that date that he could not run more than 2 km before having to stop. After stopping, the pain was deep and persisted for several minutes; it was also associated with swelling of the calf. This pain had led him to change his occupation to a less physically demanding job (military officer in the past, and now security officer). The consequences were a weight gain of 8 kg, and a cessation of all physical activity (running, fight sports). The patient also reported a loss of self-esteem.\nOver a period of 4 years, the patient was seen by two general physicians, a rheumatologist, a neurologist, a sports physician and a vascular physician. Many explorations were carried out (spinal and pelvic x-ray, pelvic and lumbar spine scan, venous duplex scan, arterial duplex scan, and electromyography), all of which revealed no sign of dysfunction. The sports physician raised the possibility of diagnosis of a compartment syndrome, but the patient refused to submit to investigation of intramuscular pressure taken at rest and in immediately post exercise. Different therapies were proposed, including anti-inflammatory drugs, paracetamol and compression stockings (Class 2).\nThe patient interview revealed that a proximal deep vein thrombosis DVT (thrombosis in the iliac, femoral and popliteal veins) of the left lower limb had been diagnosed in 2012 and treated for 6 months by direct oral anticoagulant therapy (Rivaroxaban, Xarelto 20 mg per day) combined with compression stockings french class 3 worn (20–36 mmHg) for 6 months and then class 2 compression socks (15–20 mmHg) thereafter. This episode of DVT occurred spontaneously without major or minor contributing factors. There was no family history of venous thromboembolism disease. Clinical and laboratory tests had found no neoplasia. The biological thrombophilia test at distance of anticoagulation was negative.\nThe patient was 1.78 m and 79 kg (body mass index = 24.9 kg/m2). He was afebrile, and his brachial blood pressure was 120/70 mmHg. Upon examination the legs were hot and there was no trophic disorder. There was a difference in the circumference of the calves (left calf 2 cm > right calf). There were no visible or palpable varicose veins. Peripheral pulses were present and there was no sign of Lassegue. Palpation of the major lower limb joints (ankles, knees, hips) was not painful. Calves were flexible, painless, with good trophicity. No lymphadenopathy was found and no local inflammatory signs. When examined by auscultation, there were no vascular sounds along the vascular axes.\nAll Doppler ultrasound measurements were performed with a high-resolution ultrasound machine (Affiniti 70, Philips, Amsterdam, The Netherlands). The deep venous system was free and compressible when compressed by the ultrasound probe at calf level (posterior tibial veins, peroneal, gastrocnemius and soleus), at femoral popliteal level (popliteal veins, femoral and common femoral) and at ilio-cava level with no sign of recent deep vein thrombosis. There was wall thickening at the left common femoral vein CFV (anterior posterior diameter APD under compression = 3.9 mm) and at the left external iliac vein (APD under compression = 3.6 mm); this finding had already been highlighted when vascular physician examined the patient in 2013.\nThe Doppler examination showed a clear asymmetry of the respiratory modulations of blood velocity between right and left CFV. In the left CVT, respiratory modulations were almost absent and blood volume flow was not as high as on the right side. We found a reflux into the left internal iliac vein and dilation of the left gonadal vein. While standing, there was no significant deep venous reflux (> 1 s) at the popliteal vein (functional venous valves). The superficial venous system (great and small saphenous veins) was compressible and continent.\nThe diagnostic hypothesis of MTS was issued at the end of this first consultation, taking into account the following clinical and hemodynamic arguments:▪ History of the left proximal DVT without predisposing factor. ▪ Symptoms of left calf venous claudication due to effort (+/− associated with unilateral edema reported by the patient). ▪ Asymmetry respiratory modulation and venous blood volume flow at the left proximal deep vein (as a result of a downstream obstacle).\nAdditional morphological exploration was requested computed tomography angiography (CTA) and a surgical opinion was recommended and requested.\nThe CTA revealed compression of the left common iliac vein (CIV) between the right common iliac artery (CIA) and lumbar spine (Fig. ) and intraluminal spurs. There was also dissimilar venous systems collatorally with that was more highly developed on the left iliac axis relative to the right iliac axis, reflecting the need to create a system of substitution.\nIn this context of symptomatic MTS with compatible imaging, venography was performed in order to confirm the diagnosis and to treat the MTS at the same time. Venography showed patency of the left venous axis iliac featuring endoluminal spurs and a footprint at the terminal portion of the iliac vein (Fig. ).\nEndovascular support was carried out with introduction of two Wallstent endo prostheses (Boston Scientific) into the common iliac and external iliac veins, associated with angioplasty intra stent to modulate the different areas of overlap, the diameters and lengths to achieve a harmonious assembly. The phlebographic control carried out at the end of the procedure was satisfactory.\nAt 3 and 6 months, the patient reported an improvement in symptoms. The patient restarted running, and initially venous claudication was not felt and the patient was once more able to run more than 10 km. He currently wears a compression class III especially during physical activity, as advised.\nAngiographic assessment found normal stent patency at the iliac vein, with symmetrical respiratory modulations. It was also decided to carry out a duplex ultrasound scan at 3 months. At this scanning session the stents were in place and permeable. Pharmacological treatment in the form of an anti platelet (Clopidogrel 75 mg) was prescribed.
The patient, now an eighteen year old man, was born after normal pregnancy and delivery and had normal early psychomotor development. From the age of three years he experienced episodic migraine-like headache, which resolved spontaneously at the age of sixteen. From the age of nine he noticed slowly progressive difficulties with handwriting due to focal stiffness and abnormal postures of his right hand. There was no infection or vaccination prior to the onset of symptoms. Subsequently, he developed abnormal postures and involuntary movements in his whole right upper limb described as slow, strained abduction and elevation of the shoulder and flexion of the elbow, wrist and fingers. The motor symptoms gradually progressed and, over a period of approximately one year, involved both upper limbs, the neck and face, truncal muscles and the lower limbs. From the age of twelve years he lost unsupported locomotion. He is now eighteen years old and wheel-chair dependent with severe generalized dystonia. He has completed his primary and secondary education and shows no evidence of cognitive or psychiatric dysfunction.\nPhysical examination by the authors at the age of fifteen revealed severe generalized dystonia with predominantly tonic features, axial and appendicular rigidity and abnormal posturing involving the neck, trunk and all four extremities (Additional file ). He had facial hypomimia and dystonia, and oromandibular dystonia with severe dysarthria, but no dysphagia or dysphonia. Sensory and cerebellar functions were normal and his cognitive function was unremarkable.\nMRI of the brain at the age of nine showed bilateral, high T2 signal lesions of the posterior putamen, which were more pronounced on the left side. Subsequent examinations at the ages of ten, fourteen, seventeen and eighteen years showed progression of the lesions in a posteroanterior fashion gradually involving all of the putamen and extending into the left caudate nucleus (Figure ). The putaminal lesions appeared cavitated on T1 and FLAIR sequences and showed high apparent diffusion coefficient (ADC) on diffusion weighted imaging (DWI). The left caudate lesion appeared oedematous with high T2 signal and heterogeneous water diffusion with low ADC corresponding to the newest parts of the lesion (Figure ). The nucleus accumbens and pallidum were spared and no other abnormalities were seen in the brain. There was no contrast enhancement on repeated examinations.\nWhole body positron emission tomography (PET) imaging with fluor-18-deoxy-glucose (FDG) was performed at the age of eighteen on a Siemens Biograph 40 PET-CT. FDG-PET of the brain showed glucose hypermetabolism in the frontal cortex and hypometabolism in the cerebellum. There was no tracer uptake in the putamen and reduced uptake in the left caudate (Figure ). PET-CT of the torso showed no abnormalities and ultrasound of the testes revealed no signs of teratomas or other neoplasms.\nCSF analysis on several occasions showed normal cell count and mildly elevated protein (0.52-0.53 g/L), but no oligoclonal bands. Antibodies against the NR1 subunit of the NMDA receptor were detected using transfected cells produced by Euroimmun Medizinische Labordiagnostika AG (D-2356 Lübeck) in the patient’s serum and CSF. Screening for other autoantibodies to nervous system antigens was negative including anti-Hu, Ri, Yo, Tr, MAG, myelin, Ma/Ta, GAD, amphiphysin, AMPA, GABA-b receptor, LGI1, CASPR2 and glycine receptors.\nHe had normal metabolic screening in blood, CSF and urine. Wilson’s and Leigh disease due to mitochondrial DNA or SURF1 mutations were excluded. He was thoroughly evaluated for mitochondrial disease including a muscle biopsy and qualitative/quantitative mitochondrial DNA analysis with normal findings. Analysis of DYT1, RRM2B, SUCLA2 and OPA1 genes was normal.\nThe patient received five intravenous immunoglobulin (IVIg) infusions (Kiovig 0.4 g/day) followed by two more infusions a month later and a new screening for anti-NMDAR antibodies one month later (2 months after treatment start) was negative in serum and CSF. Three months after the first IVIg infusion his clinical status and MRI findings, including lesional ADC, are stable but there have not been signs of clinical improvement. He receives regular intramuscular injections of Incobotulinum toxin A in the upper limbs resulting in moderate improvement in motor function.\nThis work has been deemed quality control by our local ethical committee (Regional Committee for Medical Research Ethics in Western Norway).
A 68-year-old Asian man presented to our out-patient department with lower back and bilateral leg pain for 1 year. The symptom was described as a “tingling sensation,” and the pain in both his thighs was aggravated by walking for more than 20 minutes. The diagnosis was neurogenic intermittent claudication, which was not relieved by analgesics, muscle relaxants, and other medications. Lumbar spine magnetic resonance imaging showed degenerative spondylolisthesis and severe degenerative central canal and bilateral foraminal stenosis (Fig. ).\nAt first, we performed a transforaminal nerve root block, but his symptoms did not improve. We discussed his condition with a specialist from our anesthesiology department to formulate a plan for managing his symptoms. We decided to perform percutaneous epidural neuroplasty with a Racz catheter. If his symptoms and signs were not relieved with neuroplasty, a surgical procedure would be considered.\nAfter obtaining our patient’s informed consent, we transferred him to our operation room, and he was placed in a prone position. Following sterile preparation and draping, we inserted a 16-gauge RX Coudé needle through his sacral hiatus. We performed epidurography with 10 ml of water-soluble contrast media. Before the epidural catheter was inserted, we checked its integrity by injecting 1 ml of sodium chloride to flush the catheter. The Racz neuroplasty catheter was inserted through the needle under continuous fluoroscopy. Undue resistance or abnormality did not occur during the insertion of the catheter and injection of materials. We injected 1500 units of hyaluronidase in 10 ml of normal saline. In addition, 9 ml of 0.125 % bupivacaine and 4 mg of dexamethasone were injected. After the injections, the anesthesiologist tried to pull out the catheter in the epidural space. During the procedure, our patient experienced severe burning pain on both buttock areas and suddenly moved his back and legs on the surgical table. We made him relax by injecting analgesics. Following this event, our patient complained of radiating pain on his left buttock. The anesthesiologist felt resistance while removing the catheter. Under fluoroscopy, the catheter could not be removed because it was trapped in the left foraminal portion of the vertebra. The distal tip of the sheared catheter was exposed and was palpable in the subcutaneous layer of the puncture site. As the anesthesiologist pulled the end portion of the catheter, the wire of the epidural catheter stretched out of shape and was not removed.\nDuring the procedure, the epidural catheter was finally broken. The anesthesiologist continuously tried to remove the broken catheter in the coccyx but was unsuccessful. Subsequently, our patient experienced more severe radiating pain in his left leg. Lumbar spine three-dimensional computed tomography (CT) revealed that the epidural catheter tip was located at the left neural foraminal inlet at the lumbar vertebral body 5 (L5) to sacral vertebral body 1 (S1) level (Fig. ). We explained the errant issue and its possible complications to our patient. We decided to perform surgical removal of the broken neuroplasty catheter with his consent. He was moved to our operating room and placed in a prone position under general anesthesia.\nA total punch laminectomy was performed, starting from the lower margin of the lamina of lumbar vertebral body 4 (L4). The catheter was confirmed to be present in the inlet area of the left L5 to S1 facet and was removed by retraction. The size of the retained catheter was 12 cm (Fig. ). After identifying the nerve root of L4, we performed foraminotomy. The nerve root of L5 was trapped in the bony structure. The facet joints on L4 to L5 were totally removed. Subsequently, both L4 nerves were released. While the thecal sac and nerve root were retracted, discectomy was performed at the L4 to L5 level. We performed lumbar interbody fusion with cages and transpedicular screw fixations on L4 to L5 and then closed the surgical wound. Our patient’s symptoms subsided after the surgery. There was no evidence of infection at the operation site. During a 12-month postsurgical follow-up period, our patient experienced no symptoms and no neurological deficits.
A 16-year-old Japanese girl with no past medical history or previous injuries presented to a nearby clinic complaining of chronic anterior left ankle pain for the past 2 years. She was diagnosed with anterior ankle impingement syndrome by ultrasonographic findings of synovial hyperplasia in the anterior aspect of the ankle joint and referred to our hospital for further treatment. A physical examination revealed tenderness and swelling across the anterior aspect of the left ankle but no local heat or redness of the overlying skin. She played badminton three times a week and complained of increasing pain with sports activities. Careful interviewing revealed that she also had pain at rest which increased with motion in the morning which was strong especially immediately after awakening.\nThe range of motion of the left ankle was normal, and marked pain was observed with forced dorsiflexion. There were no signs of ankle instability. A blood test showed that her white blood cell count, C-reactive protein level, and matrix metalloproteinase-3 level were within normal limits. Rheumatoid factor and anticyclic citrullinated peptide antibody tests were negative. Plain radiographs showed a small exostotic bony bulge on the talar neck which resembled a traction spur and a recess on the talar neck (). Computed tomography (CT) showed an 8 mm radiolucent lesion with marginal sclerosis and central calcification in the talar neck (). Magnetic resonance imaging (MRI) revealed a bone lesion in the talar neck with surrounding bone marrow edema, synovial thickening in front of the bone lesion, and joint effusion ().\nWe initially considered two pathologies as differential diagnoses: one was anterior ankle impingement syndrome considering the bone lesion as a recess, flake, and spur caused by impaction of the distal tibia against the talar neck; and the other was OO in the talar neck considering the bone lesion in CT and MRI as a nidus with secondary synovitis in the ankle joint. Activity modification and daily oral aspirin therapy slightly reduced but did not eliminate her symptoms. An intra-articular steroid injection provided some pain relief; however, the effect lasted only 1 week. Persistent synovitis unresponsive to conservative treatments for several months prompted us to narrow down the differential diagnosis to OO. The patient opted for conservative treatment consisting of oral aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs); however, her symptoms persisted and she finally decided to undergo surgical resection 14 months after the first visit.\nWe performed arthroscopic surgery using anteromedial and anterolateral portals. The patient was placed in the supine position on a radiolucent carbon fiber table with the ankle manually distracted. Arthroscopy revealed capillary hyperemia and synovial hyperplasia in the anterior aspect of the ankle joint (). We performed a thorough synovectomy with a shaver and radiofrequency probe to obtain a clear and larger operative field. The surface of the OO lesion was suspected after exposure because the overlying cortex was irregular and too thin to exhibit evidence of denting under gentle pressure with a probe (). We then used 3D C-arm-based imaging (ARCADIS Orbic 3D, Siemens Medical Solutions, Erlangen, Germany) to verify the lesion's exact location and visualize the exact extent of the nidus without removal of large part of cortex () and used grasping forceps and curettes to remove it. The entire shell of the lesion could not be visualized arthroscopically after thorough resection; therefore, we checked the lesion with another 3D scan. Unexpectedly, a remnant lesion was confirmed in a dead angle of the arthroscope. We resected the residual nidus and marginal sclerotic bone using curettes and a radiofrequency probe. A final 3D scan was performed to confirm that the nidus was completely removed (). The cavity of the excised tumor was left empty without any augmentation. The excised nidus and obtained synovium were sent separately for histopathological examination. The histopathology of the synovium was consistent with inflammatory synovitis, while the excised nidus showed randomly interconnecting trabeculae of the osteoid in a fibrovascular stroma rimmed by osteoblasts which was consistent with OO.\nPostoperatively, the patient reported immediate relief of her ankle pain. She was followed up for 12 months without lesion recurrence. Symptoms of anterior ankle impingement disappeared with no functional disability at the latest follow-up.
A 53-year-old African-American woman with ESRD was transferred from dialysis clinic to the emergency room (ER) for evaluation of non-radiating and dull epigastric pain for two weeks associated with fever and chills during hemodialysis (HD). Three months ago, she was hospitalized and treated for Streptococcus pneumoniae and Enterobacter cloacae bacteremia. A year ago she was treated for S. maltophilia bacteremia secondary to an infected dialysis catheter. Past medical history was also significant for hypertension, atherosclerotic vascular disease pending elective coronary artery bypass graft (CABG) surgery, and sudden cardiac arrest followed by ICD placement. With the current presentation, both blood and catheter cultures obtained at the dialysis clinic were positive for S. maltophilia, prompting her subsequent arrival to the emergency room. She presented with a continuation of fever and chills, as well as tachycardia and episodic hypotension. She was noted to have mild epigastric tenderness. There was no surrounding erythema, discharge, or tenderness noted around the tunneled dialysis catheter on the right anterior chest. Initial workup showed elevated troponin and procalcitonin. Chest X-ray findings were suggestive for left lower lobe pneumonia. Within the ER, the patient's hypotension resolved with fluid resuscitation and intravenous levofloxacin therapy was started with blood cultures drawn.\nCardiology was consulted for persistent elevation of troponins and it was presumed secondary to impaired clearance in ESRD. Transthoracic echocardiography (TTE) was done to evaluate for endocarditis given the presentation of bacteremia and fevers. TTE revealed artifact noted on an abandoned ICD lead in the right heart concerning for possible vegetation. Repeat blood cultures were positive for S. maltophilia and the patient was continued on levofloxacin. Infectious Diseases was consulted and as per their recommendation the infected tunneled dialysis catheter was removed on the 2nd day of the hospitalization (DOH). The patient continued to be febrile despite levofloxacin therapy and a transesophageal echocardiogram (TEE) was performed to look for a cardiac source. Subsequent blood cultures were negative at this time but the patient continued to be symptomatic. The TEE was done on the 4th DOH and showed a 1 x 0.5 cm echodensity attached to an abandoned right ventricular (RV) ICD lead in the superior vena cava (SVC) as it entered into the right atrium (RA). The echodensity was concerning for a vegetation due to IE. ID consultation recommended removal of the abandoned lead with culture of the probable vegetation that may have served as a nidus for recurrent bacteremia. Levofloxacin therapy was continued and a new dialysis catheter was placed on the 5th DOH with HD restarted. At this time, the patient became afebrile and reported symptomatic improvement. Despite a strong suspicion for IE by Duke criteria, fluorodeoxyglucose positron emission tomography (FDG PET) on the 6th DOH was non-diagnostic for differentiating infective etiology from thrombotic. Cardiothoracic surgery was consulted for removal of the abandoned lead for culture and planned to do so in coordination with her pending elective CABG procedure. In the interim, repeat blood cultures remained negative on the 7th DOH and the patient was stable and discharged on the 12th DOH with instructions to transition to oral levofloxacin until her CABG procedure, scheduled 18 days from discharge.\nUnfortunately, the patient expired due to complications from cardiac arrest secondary to severe hypokalemia in the postoperative period after removal of the infected ICD lead and successful CABG. No vegetation could be appreciated on gross inspection of the removed ICD-lead and subsequent culture was negative for any growth, indicating a resolution of the IE over the one-month course of levofloxacin treatment since presentation.
A 46-year old male, with the history of injury 3 months back in the left cornea with sparks of fire while wielding at work gave a history of occasional redness, glare, and watering initially, which the patient ignored as it resolved spontaneously but later noticed a white spot in that eye. With no treatment, the lesion showed a gradual increase in size, and so he visited us. He was a known diabetic for 3 years before the incident. At presentation to us, his distant-corrected visual acuity in the right eye was 20/80 and left eye was 20/40. On the clinical and slit-lamp examination, his right eye was normal and the left eye was mildly congested, but no discharge was noted. The cornea showed a single, dense cheesy white, full thickness, 5 sq mm infiltrate with serrated margins that were well delineated and had a 3 sq mm central epithelial defect with 20% thinning []. The infiltrate was partly involving the pupillary area []. There was no anterior chamber reaction, and dense lens changes were noted []. Intraocular pressure was 18 mmHg in the right eye and normal digital tension in the left eye. Both eyes' dilated fundus examination was normal. Corneal scraping was collected from the left eye and was processed for microbiological investigations including direct smear and culture. Fungal elements were not seen in the KOH/Calcofluor stained smear, and bacteria or yeasts were not seen in the Gram-stained smear. With the clinical suspicion of an indolent fungal keratitis, the patient was started on intensive topical antifungals of 1% voriconazole and 5% natamycin hourly, along with suitable adjuvant treatment and strict control of diabetes. Culture for fungus was positive at the end of 10 days of incubation only from liquid medium (brain–heart infusion medium), and on subculturing onto solid medium, the growth was identified as Gram-positive spherical yeast cells []. Based on the morphology, suspecting it to be an unusual yeast, it was subjected to identification byVitek 2 system and was identified as Prototheca wickerhamii.\nSince there was no clinical improvement for more than a month of medical management, the patient underwent therapeutic penetrating keratoplasty, with 8 mm diameter graft to make sure a minimum of 2 mm clear zone to encompass the infiltrate was left. Graft was anchored with 16 10-0 nylon interrupted sutures. Postoperatively, the patient was on continued intensive topical antifungals; there was no sign of recurrence. The corneal button was subjected to microbiological investigations, and the culture confirmed the algae which was identified by automated Vitek 2 system as P. wickerhamii. Corneal button subjected to histopathology showed focal defects in epithelial and Bowman's layers with no cellular infiltration of stroma with normal Descemet's membrane and sparse endothelium. Gomori's Methenamine Silver (GMS) stain was negative. Postoperatively, patient continued topical voriconazole for 1 month, and steroid was started after 3 weeks and he is maintaining clear graft, and the eye is uninflammed with no recurrence of infection for a period of 2 months of postoperative follow-up.\nTo confirm the identification of P. wickerhamii, we used primer set specific for P. wickerhamii targeting 18Sr RNA gene, forward primer 5′-TCA AAA AGT CCC GGC TAA TCT CGT GC-3′, reverse primer 5′-CGC TTT CGT GCC TCA ATG TCA GTG TT-3′, with the annealing temperature of 58°C, 35 cycles as described by Hariprasad et al.[] However, the polymerase chain reaction (PCR) profile was optimized by us with the denaturation at 94°C annealing at 58°C, and extension at 72°C, for 35 cycles and the expected product was 319 base pairs (bp). The PCR performed yielded 319 bp specific product [] and further DNA sequencing performed with the amplified product also confirmed it to be P. wickerhamii. The sequences were also deposited in the Genbank (Genbank accession number for SUB3006650 seq1 MF796662).
A 44-year-old woman injured her right ankle joint after falling from a bicycle in January 2013. One week later, ORIF was performed at the initial emergency hospital. One month postoperatively, the patient was permitted to walk with weight-bearing but was unable to do so due to sustained ankle pain on the lateral and posterior aspect. After follow-up, including rehabilitation for 7 months, all screws were removed under the assumption that bone union had been obtained. However, ankle pain remained and the patient still could not walk with weight-bearing and was referred to our hospital in December 2013.\nOn initial plain radiographs, Weber type B and Lauge-Hansen classification SE type stage 4 fractures were revealed (). The medial and lateral malleoli were fixed using Acutrak 2 screws (Acumed, Hillsboro, OR) at the time of initial operation in February 2013 (). After the removal of those screws, a medial clear space was opened (). Computed tomography at our hospital revealed bone union of the posterior and medial malleoli but malunion of the lateral malleolus ().\nAnkle arthrodesis is considered one form of salvage when a long time has elapsed since the first operation. However, reconstructive osteotomy was selected in this case because of the young age of the patient and the relatively smooth joint surface on radiography. In this case, shortening of the fibula because of posterolateral rotation of the distal fragment and widening of the ankle fork because of concomitant syndesmotic injury was present. As a result, the surgical plan involved fibular osteotomy through the initial fracture plane with fibular lengthening and internal rotation of the distal fragment until the ankle fork had been reconstructed anatomically. In addition, syndesmotic stabilization was planned by screw fixation.\nWe also evaluated preoperative foot function before reconstructive osteotomy using the self-administered foot evaluation questionnaire (SAFE-Q) () []. Despite poor results, ankle range of motion was relatively well maintained from 10° of dorsiflexion to 30° of plantar flexion.\nRevision was performed in February 2014, almost 1 year after the first operation. At first, the malunited part of the fibula was exposed and scar tissue was removed. Partially united bone was recut with a chisel, and distal and proximal bone fragments were completely separated. Corticated surface of bone fragments was decorticated with a 1.2 mm K-wire. The bone fragments were reconstructed as close as possible to the normal side under fluoroscopic imaging; then a bone gap was created (). Autologous iliac cancellous bone was grafted into this gap and bone fragments were fixed with a locking plate. Next, a syndesmotic compression screw was inserted to fix the tibiofibular joint. Confirming the medial side, the medial triangle ligaments were loose and were therefore corrected by resuturing firmly. The operating time was 2 h 36 min, and blood loss was minimal.\nPostoperatively, a short leg cast was applied and maintained for 4 weeks and then changed to a half cast and range of motion exercises for the ankle were started. The compression screw was removed at 6 weeks postoperatively, and partial weight-bearing walking was started. Full weight-bearing with an ankle support was achieved 12 weeks postoperatively. Radiography after 5 months revealed that the medial clear space had been properly maintained. With regard to the fibula, grafted bone was not absorbed and alignment was unchanged after the second operation; bone union was considered to have been achieved ().\nSAFE-Q was also administered at 5 months postoperatively, showing significant improvements in all subscales (). Postoperative range of motion was 10° of dorsiflexion and 40° of plantar flexion. Compared with the range of motion before the second operation, little change was evident.
A 56-year-old female was referred to our department from another facility in the patient’s area for a rapidly progressing tumor in the gallbladder and liver area. The patient reported several-month right upper quadrant pain and 4-kg weight loss over the past year. There was no laboratory sign of obstructive jaundice at the day of admission. Preoperative CT and MR scan (Figs. and ) of the liver was performed, and the patient was diagnosed with a tumor in the gallbladder area with a relatively massive infiltration of the S5 and S6 liver segments and extensive regions of necrosis. Given the potentially resectable lesion according to preoperative imaging, exploratory laparotomy was indicated to attempt radical resection. During the exploration, a voluminous tumor was found attached to the peritoneum. Intraoperative ultrasound was performed and revealed a tumor originating from the gallbladder bed area and reaching up to the area of the hepatic hilum and extensive involvement of the hepatoduodenal ligament by the tumor through the lymph nodes. The tumor was classified as inoperable due to this finding. But during the exploration, however, a rupture of the fragile tumor occurred with massive eruption of the necrotic mass and the gallbladder content into the abdominal cavity, accompanied by bleeding of the liver parenchyma. We decided that the condition could only be managed by attempting modified resection. We performed cholecystectomy and non-anatomical resection of hepatic segments S5 and S6 and partial resection of S4 without lymphadenectomy as a debulking operation (Fig. ). The course of hospitalization was uncomplicated, and the patient was discharged to home care on postoperative day 9. Histologically, the tumor was confirmed as MINEN of gallbladder (Figs. , , and ), and its non-neuroendocrine component had the character of moderately differentiated tubular gall bladder adenocarcinoma, while the neuroendocrine component had the appearance of small cell carcinoma and was dominant, accounting for more than 65% of the viable tumor. The neuroendocrine component contained extensive necrosis, with mitotic index 64/10 HPF and a proliferation index of 70% (Fig. ). It was therefore obvious that the prognosis and the subsequent biological behavior would be influenced in particular by the neuroendocrine carcinoma component. Six weeks after the discharge, the patient underwent a follow-up CT scan prior to the initiation of systemic therapy, which revealed a large recurrence of the disease at the resection surface of the liver accompanied by hilar lymphadenopathy. The patient was started on systemic therapy with etoposide and carboplatin in combination with somatostatin analogues with very good radiological effect. We use this regimen as a standard in patients with MINEN of gastrointestinal tract with dominant neuroendocrine component, even with no somatostatin receptors staining available. Now the patient is almost a year after being diagnosed with a tumor, after completion of 6 cycles of adjuvant chemotherapy (carboplatin + etoposide) in combination with biological therapy, the long-acting somatostatin analogues. The patient is in good clinical condition, and while a recently performed PET/MRI scan revealed a hepatic lesion and hilar lymphadenopathy in full regression, there was a spread of small peritoneal and pleural metastases, with a solitary metastasis in Th9. The condition was evaluated as disease progression stage according to RECIST criteria, the patient remains in the follow-up care, and it is now 13 months after surgery (Figs. , , and ).
A 49-year old perimenopausal Caucasian woman, gravida 3 para 3, was referred to our institution for a suspicious adnexal lesion. She complained of an intermittent pelvic pain over the preceding 3 months accompanied by weight loss. No fever was reported, neither bloody nor purulent vaginal discharge. Physical examination showed a mild tenderness at the lower quadrants without a palpable adnexal mass. Gynecological exam did not find signs of inflammation in the vaginal or cervical mucosa. No cervical motion tenderness was found. Patient’s medical history included the insertion of a copper IUD 20 years ago. The IUD has not been changed until its removal 1 month prior to the admission. No other relevant medical or surgical history was found besides smoking. Laboratory findings showed an increased C-reactive protein level (203 mg/l), leukocytosis (13,000/mm3) and microcytic anemia (hemoglobin level: 9.2 g/dl). The tumor markers were normal except the CA-125 which reached 209 mIU/l. Pelvic and abdominal computed tomography (CT) and MRI were performed and revealed bilateral ovarian multiloculated cystic lesions with multiple anterior pelvic implants in the utero-vesical space evoking peritoneal carcinomatosis. The largest implant above the bladder dome measured 2 cm, while the ovarian lesions reached 4 cm in diameter (). Pelvic lymphadenopathies were also seen on the CT scan.\nThe case was discussed in a multidisciplinary meeting at our institution and an exploratory laparoscopy with biopsies was recommended. The procedure showed severe adhesions in the lower abdomen with frozen pelvis and ascites. Due to the fear of spreading eventual malignant cells, the pelvic magma was not dissected or ruptured laparoscopically. In order to rule out malignancy, peritoneal biopsies were taken and ascites fluid was collected for cytological examination. Histological examination demonstrated that the peritoneal nodules were fibrosing and inflammatory. No malignant features were found. The patient was; therefore, discharged empirically on ciprofloxacin. Thus, the patient described a persistent pelvic pain and pressure resistant to analgesics 6 weeks later. We therefore, decided to undergo a surgical treatment due to the patient’s discomfort and the absence of bacterial growth on samples collected during the laparoscopy. An exploratory laparotomy was performed: an indurated nodule was seen in the vesico-uterine space and was invading the posterior bladder wall. Posteriorly, the adnexal magma was adherent to the ileocecal valve and infiltrated the sigmoid. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, associated with partial cystectomy, cecal resection, sigmoidal wedge resection and partial omentectomy. Visceral resection was limited due to the atypical inflammatory aspect that was seen macroscopically; though, it was performed in order to secure clear margins away from any potential malignant tissue. The bladder and sigmoidal defects were closed using continuous resorbable suturing. On macroscopic examination, thick yellowish purulent material was filling both ovaries. The histopathological examination revealed no evidence of malignancy; the ovaries showed foci of extensive suppurative granulomatous inflammation with no caseous necrosis, focally containing clumps of basophilic filamentous bacteria that were surrounded by acute inflammation and were positive for Gram, periodic acid-Schiff (PAS) and grocott methenamine silver (GMS) stains, consistent with actinomycosis (). The same inflammatory foci were seen in the bladder wall, containing the clumps of filamentous bacteria. The endometrium and the fallopian tubes showed on the other hand signs of chronic endometritis/salpingitis with lymphocytic infiltrate.\nThe postoperative period was uncomplicated: a urinary catheter was left in place for 2 weeks and bowel movements returned on day 5 with progressive diet initiated thereafter. The patient received intravenous ampicillin during her hospital stay and was discharged on ampicillin 1 g daily for 6 months. Retrograde cystourethrogram returned normal 2 weeks after the surgery. The patient was followed-up afterward for 2 years with no recurrences detected so far.
A 65 year-old woman was injured when an automobile pushed her against a concrete wall. Vital signs were normal but a pelvic fracture was suspected because of severe pain in the lumbar region and she was brought to the critical care center.\nHer consciousness was clear and hemodynamics stable. Severe pressure pain was present on the anterior surface of the pubic bone and near the right sacrum. Macroscopic hematuria was found when a urethral catheter was inserted. A contrast enhanced CT (CECT) was performed on admission but the CT did not capture contrast leakage from the bladder. However, a pelvic X-ray post CT showed contrast leakage in the extraperitoneal space at the anterior of the bladder. An extraperitoneal bladder rupture was diagnosed. The open-book type pelvic fracture was treated with Hoffman's external fixation. Macroscopic hematuria was gradually improved and disappeared on day2.\nOn day 4, retrograde cystography (Fig. ) and CT cystography were performed. An urinoma was found to extend from the anterior surface of the pubic bone to the medial side of the left thigh. In a sagittal reconstruction CT image, a leakage site of the contrast medium was observed at the lower anterior surface of the bladder. The base of the bladder had descended to the inferior margin of the pubic symphysis. The patient's general condition was very good and no symptoms of infections were found. Urine drainage through a urethral catheter was good. Therefore, we though that this urinoma was spontaneously discharged through a urethral catheter, and the bladder laceration to extraperitoneum was spontaneously healed. Non-surgical management was selected. CT cystography were performed on day 21. As shown in Fig. , the urinoma had become enlarged. The base of the bladder had descended to lower than the CT on day 4. Surgery was performed.\nA midline incision of the lower abdomen was made and the incision was extended through a front of the pubic symphysis to the left thigh. Surgical findings (Fig. ) revealed that the bladder was not anchored because of severe lacerations in the urogenital diaphragm, and had a laceration of about 3 cm in the anterior surface of the bladder neck. The findings also showed a partial laceration of the left femoral adductor muscle at the pubic insertion and an urinoma cavity extending from the anterior surface of the pubic bone to the left thigh. The wall of the urinoma consisted of membranous tissue with a flat smooth surface. The laceration of the bladder was closed by sutures. The membranous tissue of the urinoma wall was partially resected and the remaining membranous tissue was extensively coagulated. Four suction drains were placed and the urinoma cavity was closed. The retropubic suspension procedure was also performed to prevent postoperative urinary incontinence. Sutures were placed on either side of the bladder neck, taking bites through the paravesical fascia and anterior vaginal wall. The most distal suture is placed at the level of the bladder neck. Each suture was then passed into an appropriate site in the cartilaginous portion of the symphysis.\nPathological findings of the urinoma wall showed histology with severe hyperplasia of collagen fiber and fibroblasts.\nThe postoperative course was uneventful. When cystography was performed on postoperative day 16, no leakage of the contrast medium was found. The urethral drain was removed 3 weeks postoperatively and the Hoffman's external fixation was removed on day 55. Urgent incontinence was seen one time just after the catheter removal and urinary incontinence was not seen thereafter. She was discharged on foot on day 65. Neurogenic bladder did not occur during follow-up period.
This patient is a 29-year-old white woman from the USA with a medical history significant for severe IBS-D (diagnosed at age 12) and anxiety disorder. In July 2015, she presented with severe bleeding hemorrhoids secondary to IBS, which required hemorrhoidectomy and anal sphincterotomy in August 2015. The week before the surgery she developed pharyngitis and was treated with azithromycin, which resulted in mucousy diarrhea and abdominal discomfort. She tested negative for C. difficile antigen and toxins at that time.\nA week after surgery, she developed a perirectal abscess that had formed at the site of the sphincterotomy and was prescribed orally administered ciprofloxacin. Despite moderate symptom improvement, in September 2015 she required an abscess incision and drainage procedure and Penrose drain insertion. Prior to the surgery she was given a single dose of clindamycin. An additional 2-week course of ciprofloxacin and metronidazole was then prescribed. In late September 2015 she was admitted to the hospital for two nights due to further complications related to the abscess and was then diagnosed as having a perianal fistula.\nIn November 2015, she was prescribed clindamycin for an episode of group C streptococcal-positive pharyngitis. In late November 2015, she was also diagnosed as having Ehlers–Danlos syndrome, which according to her medical record may partially explain the poor wound healing from the perirectal abscess. In December 2015, her fistula required an anus seton placement. She was treated with multiple courses of ciprofloxacin and metronidazole off and on from December 2015 to January 2016.\nIn January 2016, following up on her recurrent pharyngitis, she was diagnosed as having chronic tonsillitis which led to tonsillectomy. In February 2016, 2 weeks after the surgery she was prescribed clindamycin. At the beginning of March 2016, she was diagnosed as having bacterial vaginosis and was prescribed orally administered metronidazole. A week later she was diagnosed as having vaginal candidiasis and was prescribed orally administered fluconazole. In April 2016, she complained of dysuria and was prescribed ciprofloxacin. After 2 days, when urine analysis results came back negative, she was asked by her physician to stop the treatment.\nIn June 2016, she presented for follow-up with ongoing diarrhea and abdominal pain. She was diagnosed as having C. difficile diarrhea, her antigen and toxins laboratory results were indeterminate, and a toxigenic strain was confirmed by polymerase chain reaction (PCR). She was prescribed a 6-week course of orally administered vancomycin. After a week of treatment her symptoms worsened, and following discussion with her gastroenterologist her treatment was switched to a 2-week course of metronidazole. Hours later, she was admitted to the hospital for a 4-day period for colitis. Her C. difficile antigen and toxin test returned negative during her admission. She received intravenously administered metronidazole treatment during her hospitalization. Her symptoms improved during her hospital stay, with 1–2 soft bowel movements a day. At discharge her metronidazole course was stopped and she was again prescribed vancomycin, which she took for over a month. She continued to experience GI irregularity (3–5 bowel movements a day) beyond what she had experienced secondary to her IBS prior to her surgeries. In March 2017, she was prescribed rifaximin for 2 weeks to treat chronic diarrhea.\nIn November 2017, she was prescribed a series of clinical intestinal tests (SmartGut™, uBiome Inc., San Francisco, USA) with the instructions to administer the test at home whenever she was experiencing a noticeable change of GI symptoms, then follow-up with her health care provider to discuss the results. This sequencing-based test requires that patients use a sterile swab to transfer a small amount of fecal material from toilet paper into a vial containing a lysis and stabilization buffer that preserves the microbial DNA for transport by mail back to the laboratory for processing, which involves DNA extraction, 16S ribosomal RNA (rRNA) gene amplification, and sequencing []. She first used this test in November 2017, about a month after completing a 2-week course of rifaximin. The results revealed a number of microbial organisms that were outside the healthy reference ranges, but she was negative for all pathogenic organisms included in the test, including C. difficile (Fig. ).\nBetween November and December 2017, her GI symptoms worsened considerably; her daily bowel movements increased from 3–4 to 6–10, stool consistency became more mucous-like and gelatinous, and she was experiencing more pain with defecation. She re-tested with SmartGut™ test again in January 2018. Her results continued to reveal a number of microbial organisms outside the healthy range and, this time, her sample also indicated the presence of C. difficile (Fig. ). She immediately contacted her primary care provider, who re-tested her for C. difficile and confirmed indeterminate CDI by antigen and toxins A and B. Additional PCR testing at a regional laboratory confirmed the sample was positive for a toxigenic C. difficile strain. As a result of testing, her clinician started her on fidaxomicin; her symptoms improved rapidly. By April 2018, she had returned to her baseline in regard to her IBS-related GI symptoms with no blood in her stools.\nIn addition, the second SmartGut™ sample was tested for toxins A and B by sequencing at uBiome Inc. laboratory in San Francisco, USA, which resulted positive for both and confirmed the toxigenic nature of the C. difficile strain.
We analyzed a 60-year-old man who was admitted to our department complaining of a 1-year history of a loss of appetite with abdominal distension and an 8-month history of dizziness with numbness of the bilateral lower extremities. In addition, the patient had a history of type II diabetes mellitus for 14 years and hypertension for 25 years. The patient was diagnosed as diabetic peripheral neuropathy before he came to our hospital and the diagnosis on admission was (1) autonomic dysfunction; (2) type II diabetes mellitus; and (3) hypertension. Gradually, he suffered from aggravated dizziness when standing up from a squatting position. He also suffered from alternating bouts of diarrhea and constipation. We performed a physical examination, laboratory tests, brain magnetic resonance imaging (MRI), positron emission tomography/computed tomography (PET–CT), lumbar puncture, marrow puncture, and biopsy of the sural nerve and rectal membrane. The study protocol was approved by the local committee on human research, and the patient provided his informed consent.\nOn examination, the patient’s blood pressure was 120/80 mmHg in the supine position and 60/40 mmHg in the standing position. He had normal consciousness and orientation. Muscle strength, tone of the extremities, and coordinate movement were all normal. There was an absence of sensation, bilaterally below the knee. All of the tendon reflexes were normal, with no pathological reflexes. Increased sweating from the chest was noted.\nAn electromyogram showed mixed peripheral nerve damage predominately in the sensory nerve fibers of the right extensor digitorum muscle and the tibialis anterior muscle. Doppler echocardiography showed diffuse hypertrophy of the left ventricular wall; the thickness of the ventricular septum was 17 mm. Plain and enhanced brain MRI scanning revealed mild brain atrophy in the bilateral hemisphere. Brain PET–CT was normal. Holter monitoring showed a second-degree atrioventricular block (type I, 2:1 block). The patient’s heart rate reduced to 30 beats per minute, and he was transferred to the Department of Cardiology for the implantation of a permanent pacemaker. A lumbar puncture was performed to collect cerebrospinal fluid (CSF); CSF analysis was normal, although CSF protein concentration (1,680 mg/L) was elevated. Transthyretin (TTR) gene sequencing showed no causative mutation. The Bence Jones protein urine test was positive, and the serum electrophoresis blood test was weakly positive for Bence Jones protein. Urinary protein or micro-albumin excretion was only mildly elevated.\nSural nerve and rectal membrane biopsies were performed. Congo red staining was positive, and apple-green birefringence was visualized under polarized light microscopy. Deposition of amyloid most commonly identified around blood vessels in the mesenchyme of the intestinal mucosa (Figure ). Furthermore, endoneurial and perivascular amyloid deposition was detected in the sural nerve (Figure ). Bone marrow smears showed active bone marrow hyperplasia, while bone marrow puncture indicated that the proportion of abnormal plasma cells were 0.56% of the whole blood cells and 87.4% of the total number of plasma cells.\nThe patient’s symptoms appeared to recover following gamma globulin implosive therapy (0.4 g/kg/day) and dexamethasone (10 mg/day) management. The difference in systolic blood pressure between the supine and standing position reduced to 20 mmHg, and the patient could walk for approximately 10 min without assistance. The frequency of diarrhea was reduced. However, his symptoms worsened 1 week after the improvement. He could sit for approximately 20 min, but he still felt dizzy when standing. In his follow-up visit 3 months after hospital discharge, the systolic blood pressure difference between the supine and standing position was approximately 30 mmHg, and he could walk for 200 m with a supportive device. He reported that he had experienced an episode of ventricular fibrillation during his sleep, in which he experienced reduced blood pressure and oxygen concentration, but his symptoms resolved after emergency treatment. In terms of his drug regimen, dexamethasone was replaced with hydrocortisone. The patient refused to be transferred to the Department of Hematology for treatment and went back to his home town. Unfortunately, the patient died in March 2017 due to ventricular fibrillation and renal failure. There was no autopsy result because the relatives of the patient refused to perform autopsy after the patient died.
A 20-year-old right-hand-dominant and otherwise healthy female student presented with protrusion of the left upper back and left periscapular pain that occurred after sport activities. Ten months previously, the patient had been seated in the left rear passenger seat in a car that was hit in the left side by another car. Further details such as the posture and the arm position of the patient at the time of the accident were uncertain. At the time of the car accident, the patient visited an orthopedic clinic where a surgeon diagnosed left shoulder contusion without any abnormal radiographic findings. The left arm was kept in a sling for 2 months, as left arm elevation caused severe pain in the upper back. After sling removal, the patient returned to basketball, which generated continuous dull pain around the left scapula. She presented at our clinic because her mother had noticed the deformity of her back.\nThe patient had no relevant family or medical history. There was no neurological deficit in the left shoulder and arm. The left scapula was slightly higher than the contralateral scapula and exhibited atypical medial winging with the arm at the side. The distance between the spinal process and medial scapular border was shorter on the left side than the right side at the inferior angle level, but these distances were almost the same at the scapular spine level (). Contraction of the scapular stabilizing muscles was good. There was a palpable bony protuberance without tenderness on the ventral side of the ISA. The limitations of the active ranges of motion of the left shoulder compared with the right shoulder were 25° for total elevation, 15° for external rotation, and none for internal rotation and horizontal adduction; however, there were no limitations of the passive ranges of motion. The winged scapula became prominent at 0–45° of active flexion, while it disappeared when the patient flexed the left arm while consciously attempting to depress the scapula (). The winged scapula did not emerge when the patient pushed on a wall at chest level. Radiographs showed a small bony fragment in the ventral side of the ISA, with a narrow space between the fragment and the scapular body (). Computed tomography revealed a bony protrusion extending from the medial scapular border to the bony fragment, with a narrow gap between the protrusion and the fragment (Figures –).\nThe patient was instructed to avoid elevating the left arm for 2 months and then performed reinforcement exercises of the SA such as the scapular push-up and the bear hug using an elastic band for 2 months. At examination 4 months later, the periscapular pain and the winging of the scapula with the arm at the side and in active flexion had resolved. The push-on-the-wall test at waist level was negative, and the range of motion of the left arm was the same as the unaffected side, except for a 15° limitation in external rotation. Although the radiographic findings were the same as at the first visit, computed tomography demonstrated bony union (Figures and ). The patient was permitted to use the left arm without restrictions.\nAt the time of the final follow-up 10 years of postinjury, the patient reported that there was an occasional painless click and a sporadic floating feeling of the scapula with initial active flexion of the arm. However, there was no pain or any disturbance to the patient's activities of daily life and work as a physical therapist. The patient's colleague confirmed the disappearance of the winged scapula associated with shoulder movement. The DASH score was 0, and the Constant score ratio compared with the right shoulder was 100% [, ].
A 51-year-old female was admitted to our hospital with a 3-month history of tonic-clonic seizures lasting up to 20 minutes each, and left-sided limb weakness. Her family reported that she did not sustain any head injuries during her seizures, and always recovered completely without fever, headache, or cognitive dysfunction.\nPrior to admission to our hospital, she had been admitted to another hospital on several occasions. Following her first seizure, which was 20 minutes in duration, she was assessed at a clinic. At presentation, her vital signs were normal, and head CT, MRI, and electroencephalography were normal. She was diagnosed with epilepsy. As no abnormalities were detected on imaging studies and she had no previous history of seizures, she was not started on prophylactic anticonvulsive medication. She was asymptomatic until 25 days later, when she had a tonic-clonic seizure lasting approximately 10 minutes. She was assessed at the emergency department. Head CT showed a low-density lesion in the right frontal lobe, and MRI showed an area of abnormal signal in the right frontal lobe. However, magnetic resonance angiography of the head was normal. Full body positron emission tomography demonstrated decreased uptake of fluorodeoxyglucose in the right frontal lobe.\nShe was admitted to hospital. Her vital signs were normal. Serum electrolyte, blood urea nitrogen, creatinine, glucose, albumin, bilirubin, alkaline phosphatase, aspartate aminotransferase, cholesterol, triglyceride, and low-density lipoprotein cholesterol levels, and red blood cell and platelet counts, were normal. She was diagnosed with epilepsy and treated with intravenous fluids, mannitol, and anticonvulsant medication (sodium valproate). She improved significantly over the course of her admission. Although she still had occasional seizures, she was discharged home on the 7th day after admission on sodium valproate 0.2 mg, three times daily.\nFor the first 14 days after discharge, she was seizure-free, but gradually developed left-sided limb weakness. On the 15th day, she could no longer move her left arm and leg, and was readmitted to the same hospital. Her blood pressure was 142/98 mmHg (1 mmHg = 0.133 kPa) and her other vital signs were normal. Head CT showed enlargement of the right frontal lobe lesion. Brain MRI showed a right frontal lobe lesion with mixed low and intermediate signal intensity on T1-weighted images, high signal intensity on T2-weighted images, massive edema, insignificant compression of the right lateral ventricle, and no enhancement (Figures –). Lumbar puncture showed a normal cerebrospinal fluid (CSF) opening pressure. CSF analysis showed normal white blood cell count, red blood cell count, protein, glucose, herpes simplex virus and Epstein-Barr virus polymerase chain reaction, toxoplasma titer, and oligoclonal bands. She was still thought to have epilepsy. She was treated with intravenous fluids and mannitol, and sodium valproate was continued. After 7 days, her limb weakness had resolved and she could walk without assistance. She was discharged 15 days after admission. Five days after discharge, her left-sided hemiplegia returned, and she complained of a mild to moderate right-sided headache. She was readmitted to the same hospital. Head CT showed massive edema in the right frontal lobe, with nodular hemorrhage (Figures , ). Brain MRI showed enlargement of the right frontal lobe lesion, and also showed a new lesion with low signal intensity on both T1- and T2-weighted images and diffuse small hemorrhages in the juxtacortical areas, with significant compression of the right lateral ventricle and deviation of the midline to the left (Figures –). She was still thought to have epilepsy, and was treated with mannitol and sodium valproate. However, her symptoms did not resolve.\nApproximately 3 months after the first seizure, she was transferred to our hospital. On reviewing her past history, we learned that she was a farmer, and had a history of type II diabetes mellitus and hypertension, both diagnosed 5 years previously. Both her blood glucose level and blood pressure had been well controlled. She denied the use of tobacco, alcohol, or illicit drugs. Her last menstrual period was 2 years ago, and she did not use any contraceptive medication. She denied any similar symptoms in other family members.\nOn physical examination, her blood pressure was 150/90 mm Hg, and other vital signs were normal. She was alert and oriented, and her speech was fluent. Her pupils were equal, round, and reactive to light. Fundus examination revealed bilateral edema and hemorrhage. Examination of the other cranial nerves was normal. Muscle strength was grade 2 in the left arm and grade 0 in the left leg. She had a positive Babinski sign on the left side. The remainder of the neurological examination was within normal limits.\nThe differential diagnosis included primary angiitis of the central nervous system, tumor, infection, and venous sinus thrombosis. Autoimmune markers (erythrocyte sedimentation rate, rheumatoid factor, antineutrophil cytoplasmic antibody, antiphospholipid antibody, antinuclear antibody), tumor-related antibodies (carcinoembryonic antigen, CA-125, CA-199, CA-724), and coagulation protein levels (antithrombin III, protein C, protein S, lupus anticoagulant, factor V) were all normal. Serological tests for human immunodeficiency virus, Borrelia, and syphilis were negative.\nLumbar puncture showed a CSF opening pressure of 304 kPa. CSF analysis showed a total cell count of 10 × 106/L, white blood cell count of 6 × 106/L, and elevated protein level of 63 mg/dL (normal: 15–45 mg/dL). CSF glucose and chloride levels, oligoclonal bands, 24-hour IgG synthesis rate, and cytology were all normal, and CSF virus antibody testing was negative.\nElectrocardiography and echocardiography were normal, and brain magnetic resonance venography was normal. Our laboratory and imaging findings did not indicate a diagnosis of isolated cortical vein thrombosis.\nAfter the lumbar puncture and blood draw, she was treated with mannitol. Sodium valproate 0.2 mg, three times daily, was continued. There was no further deterioration. Two weeks after admission, surgical excision of the right frontal lobe lesion was performed under general anesthesia.\nPostoperative histological examination of the surgical specimen showed destruction of brain parenchyma with infiltration of macrophages and proliferation of reactive astrocytes and small vessels. There were foci of hemorrhage in the lesion (). Further examination found that a number of small vessels in both the subarachnoid space and brain parenchyma were filled with thrombus, some of which was organized. Elastic fiber staining showed that the obstructed vessels were veins.\nThe patient recovered gradually after surgery. She had residual mild left-sided hemiparesis and occasional tonic-clonic seizures. After 1 month of rehabilitation, she was able to live on her own. DSA at 2 weeks after surgery was normal. At her 9-month follow-up appointment, she still had mild left-sided hemiparesis. She continued taking sodium valproate (0.2 mg, three times daily) and had experienced one tonic-clonic seizure since discharge. All the routine tests that were performed during her initial presentation were repeated, and the results were normal. Brain MRI did not show any new lesions.
We present the unique case of a 28-year-old male patient displaying a complex clinical picture with mental retardation and various behavioural problems since birth. Symptoms of the autism spectrum comprising difficulties in social interaction and communication are reported since childhood. Additionally, he suffers from auto-aggressive tics in terms of beating himself with objects against his head and lower jaw, head movement tics and simple vocal tics. Striking dysmorphic features are not evident.\nExcept a one-time bleeding in week 20 of gestation, pregnancy had been without any complications. No infections, medication, smoking, or intake of alcohol or drugs during pregnancy was reported. The patient was delivered in week 40 of gestation with the help of a ventouse due to irregular cardiac activity. During delivery there were minor signs of birth asphyxia. Birth weight was 2.900 g (25th percentile), birth length 51 cm (50th percentile) and head circumference 33 cm (<3rd percentile). During infancy a prominent frontal fissure was conspicuous. A premature ossification of the sagittal fissure could not be detected.\nThe patient showed psychomotor retardation: he walked alone only by 26 months and was not able to sit alone falling over to one side without shoring up even at the age of 2. Furthermore, tics in terms of eye blinking as well as a muscular hypotonia were described. The patient’s parents reported early autistic features such as difficulties in social communication and interaction with avoiding eye contact and poor interest in social interaction. Development of speech was delayed (first words with 18 months). He refused body contact and demonstrated stereotypic patterns of behaviour such as filling bowls without showing any variations.\nWhen examined at the age of 27 months, the patient presented some special facial features such as synophrys, epicanthus, modelled ears, a deep joined thumb and microcephaly. His weight was 10 kg (3rd percentile), his length 88 cm (25th percentile) and his head circumference 46 cm (2 cm above the 3rd percentile). The patient only spoke a few words and never a whole sentence. Mostly, he only repeated the words he had heard before in terms of an echolalia. A considerable general delay of development with severe perceptual disturbance and autistic traits was diagnosed.\nSince the age of two years, the patient shows relevant aggressive symptoms such as throwing his head on the floor or biting into items. He needs extensive support concerning all activities of daily living and requires constant daily routines. During nursery school, auto-aggressive symptoms exacerbated in terms of head banging behaviour injuring his jaw and ears. At the age of 3 years, he started grinding his teeth. Later on, he presented head throwing movements against his left shoulder and banging of one row of his teeth against the other. There was a pattern of aggravation of the tic symptoms during stressful situations. He repeatedly showed refusal of meals and sleeping disturbances. At the age of 3 years, once there was a query febrile convulsion associated with an infection. Apart from that, there was no evidence for further seizures and clearly no epilepsy.\nAccording to ICD-10 and DSM-5 classification, the clinical features of the patient are consistent with early infantile autism as well as with Tourette syndrome.\nIn the clinical examination, the patient presented with cauliflower-ears as a result of his head banging behaviour and subsequent repetitive ear injuries as well as various injuries of all kinds and healing stages. Dysmorphic features or additional external malformations were not noted, there was no evidence of internal abnormalities (heart, eye, inner ear) either. The patient showed a preserved ability of speech comprehension with rare speech production.\nOur patient is the first son of non-consanguineous healthy German parents. His younger brother (24 years old) was diagnosed with an autism spectrum disorder (Asperger syndrome). There was no evidence of syndromal-secondary autism in the brother. He did not show intellectual impairment, dysmorphic signs or organic conditions such as a heart malformation or epilepsy. The mother’s paternal grandparents were cousins. They had five healthy children. In their further progeny three mentally retarded persons were reported, one granddaughter (died at the age of approximately 27 years) and two great-grandchildren (a boy and a girl). The exact diagnosis was unknown to our patient’s parents. Consanguinity of the retarded relatives’ parents was denied. One of the patient’s cousins (the son of his father’s brother) was said to suffer from clinically apparent dyslexia and perhaps an autism spectrum disorder. A disablement of a cousin of our patient’s father (son of his father’s sister) could not be specified further. An assessment of the father revealed no clinical characteristics, neither with respect to striking dysmorphic features, the occurrence of tics nor with respect to autistic symptoms. Further relatives with neurodevelopmental disorders were not reported on the father’s side of the family.\nConventional R-banded karyotyping of the patient was performed according to standard protocols with a resolution of approximately 500 bphs and revealed a structurally and numerically normal male karyotype (46,XY) in all 21 metaphases examined.\nFurthermore, the genomic DNA of the patient was examined by microarray-analysis (CytoSureTM Constitutional v3 Array 180 k, OGT (Oxford Gene Technology)) according to the manufacturer’s instructions. After hybridization, the array was scanned with the SureScan Microarray scanner (Agilent), the results were analyzed using CytoSure interpret software v.4.9 (OGT) against the Genome Reference Consortium human genome GRCh37 (hg19). Molecular karyotyping revealed a heterozygous deletion of approximately 719 kb (267 contiguous oligonucleotides) out of the chromosomal region 2q24.3 (karyotype according to ISCN (International System for Human Cytogenetic Nomenclature) (2016): arr[GRCh37] 2q24.3(165471418_166190427)× 1). The deletion encompasses the five genes GRB14 (exon 1 to intron 2–3), COBLL1, SLC38A11, SCN3A and SCN2A (exon 1 to intron 14–15) listed in OMIM (Online Mendelian Inheritance in Man) (Fig. ). With FISH (fluorescence in situ hybridization) analysis using probe RP11-150F4 (Empire Genomics) located at 2q24.3 the deletion could be confirmed. There is no evidence in literature that the microdeletion we detected is a common variant in the Caucasian population [, ]. “Orphanet” states a prevalence rate of a 2q24 microdeletion < 1/1000000 (worldwide) ().\nThe subsequently performed chromosome analysis of the parents, FISH with RP11-150F4 included, showed a normal female karyotype in the mother and a normal male karyotype in the father with no evidence of a deletion or rearrangement at 2q24.3. Therefore, it can be asserted that the deletion in the patient is a de novo mutation. The patient’s younger brother (suffering from Asperger syndrome) was shown to have a normal male karyotype without a deletion or rearrangement at 2q24.3.\nMagnetic resonance imaging conducted in 2014 showed no abnormalities of the patient’s brain. In 1991, a detailed investigation was performed with a metabolic screening of serum, urine and cerebrospinal fluid revealing normal results. In the EEG, there was a slow baseline activity without any epilepsy suspicious potentials. A proton-spectroscopy showed no abnormalities of N-acetyl-aspartate, choline and phosphor-creatinine levels. An ophthalmologic investigation exhibited no deviations. In 1997, gastrointestinal passage was unsuspicious, and in an X-ray of the brain no stenosis of the sagittal fissure was confirmed.
On 25 March 2020, a 31-year-old woman (gravida 1, para 0) was referred from another hospital at a gestational age of 23 + 3 weeks because of a suspicion of heart anomaly. No significant medical or family history was suggestive of any structural heart disease. A fetal echocardiogram performed at the gestation age of 23 + 3 weeks revealed dysplastic right ventricular cavity with focal thinning, and dilatation of the myocardium of the right ventricular free wall, with bulging of the interventricular septum into the left ventricular outflow tract (LVOT). The visceroatrial situs was solitus and the aortic arch was normal with no arch obstruction. The tricuspid valve was nearly atretic and had several small perforations with regurgitant flow. The main pulmonary artery (MPA) arose from the right ventricle (RV) normally and branched to both sides with good perfusion. The ductus arteriosus connected to the MPA with right to left shunt (A).\nAt 35 + 1 weeks of gestation, on 15 July 2020, fetal echocardiography showed a dilated RV that had a dysplastic cavity, with focal thinning of the right ventricular free wall and the interventricular septum bulged into the LVOT. There were no pulmonary valve leaflets with free pulmonary regurgitation. Other findings were similar to those observed in previous fetal echocardiograms (B).\nOn 29 July 2020, a male neonate was delivered by cesarean section. He weighed 3.14 kg (50~75 percentile), length was 46 cm (within the 25 percentile), and his Apgar scores at the first and fifth minutes were 3 and 5 points, respectively. Oxygen saturation in room air was below 80%. After birth, the neonate was severely dyspneic and was intubated immediately and placed on mechanical ventilation.\nChest radiography showed cardiomegaly and a cardiothoracic ratio of 0.75. A transthoracic echocardiogram () showed that the situs was solitus and the entire RV except the apical portion was aneurysmally dilated. Apical hypertrophy of the myocardium of the RV was observed. The tricuspid valve was atretic with no movement. The site of the tricuspid valve had several small holes with continuous severe regurgitant flow. The pulmonary annulus was normal in size (7.1 mm, Z-score −0.27), but there were no pulmonary valve leaflets. MPA (11.9 mm, Z-score 2.75), right PA (7.78 mm, Z-score 3.39), and left PA (6.76 mm, Z-score 2.88) had good blood flow. The mitral annulus was 16.4 mm (Z-score 2.45). The aortic arch was left-sided and the annulus was 10.1 mm (Z-score 4.38). There was a large patent ductus arteriosus with a left to right shunt.\nOn the eighth day of life, he underwent right modified Blalock-Taussig shunt with Gore-Tex tube graft, tricuspid valve and pulmonary valve obliteration, resection of the right ventricular aneurysmal free wall, and atrial septectomy. Histological examination of the right ventricle demonstrated the absence of the RV myocardium (). During the 3-month follow-up, the patient was awaiting a staged single-ventricle palliation involving a bidirectional Glenn procedure.
A 68-year-old man was admitted to our department of internal medicine in June 2010 because of fever, gradually worsening low back pain and difficulty in rising. These symptoms started approximately two weeks before his admission. The patient had no significant medical record and he did not receive any chronic medication. From the recent medical history he mentioned a restorative dental procedure two months ago and a right shoulder tendonitis three weeks ago for which nonsteroidal anti-inflammatory agents per os were administered.\nOn admission the patient was febrile up to 38.5°C and oligoanuric. He was subjected to laboratory tests () that revealed acute renal failure, normocytic normochromic anemia, leukocytosis with neutrophilia, and highly elevated markers of inflammation. Urine analysis detected considerable hematuria and albuminuria.\nThe patient was started on a continuous intravenous infusion of furosemide and human albumin solution which resulted in a satisfactory diuresis. Besides, a renal ultrasound was performed, which disclosed a well defined hypoechoic area 1.19 × 0.96 cm within the cortex of the left kidney with low-amplitude internal echoes enhancement and lack of vascularity on doppler imaging (). These findings were consistent with a renal abscess. Empiric therapy included meropenem 500 mg thrice daily iv (reduced dose on account of acute renal failure). Moreover, four blood and two urine cultures obtained after admission yielded methicillin-susceptible Staphylococcus aureus (MSSA). Endocarditis was excluded due to normal findings of transthoracic and transesophageal echocardiographs. One week later, de-escalation of antibiotic therapy was undertaken based on microbiological susceptibility data and teicoplanin 400 mg twice daily iv was initiated.\nThe patient became afebrile on the third week and the renal function was progressively getting improved. Nevertheless, the deterioration of his anemia that imposed consecutive transfusions of RBC plus the persistence of the elevated markers of inflammation (ESR = 135 mm/h; CRP = 12 IU/L) raised the need for further examinations. A complete skeletal X-ray was initially performed but no lytic lesions were apparent. Serum calcium was normal, beta-2 microglobulin was slightly elevated (b2m = 3.50 μg/mL) and urine albumin was 1.67 gr/d. Immunoglobulin levels were identified, showing an increase in IgG and normal IgA and IgM (). Serum electrophoresis showed a monoclonal component in the gamma region and the immunofixation electrophoresis (IFE) pattern demonstrated a typical paraprotein, IgG-lambda (). Bence Jones protein was not detected in urine analysis. The bone marrow aspiration revealed plasma cells to an extent of 6% and the bone marrow biopsy found only a few plasma cells (<10%). Lastly, flow cytometric immunophenotyping showed a normal population of T cells and a decrease of the B-cell population (2%, normal range 6–23%). Therefore, these results suggested most likely a MGUS rather than the likelihood of an underlying hematological malignancy.\nFour weeks after admission the patient was afebrile and a renal ultrasound manifested a complete resolution of the abscess. Still, the patient complained of deterioration in back pain and at the same time the laboratory markers of inflammation were steadily elevated. Complete serological tests, neoplastic markers, Gen Probe and Ziehl-Neelsen stain for mycobacteria, Widal, Wright and Wright-Coombs as well as testing for HBV, HCV and HIV were all negative. Soon after the normalization of the renal function, a computed tomography scan with iv contrast of the abdomen, chest and spine was performed. The findings suggested a spondylodiscitis and diagnosis was confirmed by magnetic resonance imaging, which revealed a three-level involvement of the spine; T7-T8, T11-T12 and L2-L3 ().\nThe patient was treated for another two months with antistaphylococcal agents. Over this period the markers of inflammation gradually decreased while a second check of serum immunoelectrophoresis detected a minor monoclonal component (). After the three-month treatment no paraprotein was identified and the patient was discharged (). During the 2-year follow-up period the patient fully recovered to his previous ambulatory status and the laboratory tests returned to normal.
A 52-year-old previously healthy man with an 11-year history of recurrent, bilateral carpal tunnel syndrome presented with a 22-month history of severe NP in his right hand. The same condition was present on the left side, although to a lesser degree.\nThe patient had undergone multiple decompressive surgeries (two on the left side, three on the right) during the 11 years prior to the presentation. After each operation, he experienced symptom relief lasting from 6 to 9 months, followed by gradual recurrence. His last operation on his right wrist, 30 months prior to presentation, resulted in roughly 8 months of pain relief. Further surgical intervention was not recommended on pain recurrence. Various treatments were tried without satisfactory pain relief (see ). The pain was eventually deemed chronic and treatment refractory.\nThe patient described the pain as constantly present, with intensity usually ranging between 5 and 8 on a 0–10 Numeric Rating Scale (NRS). The pain was located primarily distal to the wrists, in the area of median nerve innervation. Although there were both pain and sensory symptoms beyond this, both in the hand and lower forearm, he did not describe it as radiating. The pain was associated with numbness, tingling and prickling sensations. The most striking and clinically debilitating aspect of the patient’s pain was the degree to which it was aggravated by cold, in terms of both cold allodynia and the effect of ambient temperature. He scored 22/38 on the PainDETECT questionnaire, indicating >90% probability that the pain had a neuropathic component.\nMRI of the right wrist revealed scar tissue in close proximity to the median nerve. Neurological examination found cold and light touch allodynia as well as decreased sensation distal to the wrist scars in both hands. These findings included but also went beyond the distribution of the median nerves. Findings were similar on both sides, but more severe on the right. Neurography confirmed damage to the right median nerve at the level of the carpal tunnel (the left side was not tested). Neurological examination was otherwise normal.\nThe patient was referred to us for inclusion in the NoTOPain trial which tested EGFR-inhibition in patients with chronic, treatment-refractory NP. He was randomised to receive a single dose of blinded placebo first, followed by a single dose of blinded cetuximab and then one open-label cetuximab infusion (see ).\nThe patient’s pain scores (self-reported daily, on a 0–10 Numeric Rating Scale) showed a clinically significant decrease after blinded cetuximab, but not after placebo (see ). Only the pain scores for the right hand (the most severely affected) were registered in the NoTOPain trial, although improvement was analogous on the left side.\nThe patient described the pain relief he experienced after cetuximab as having completely transformed his quality of life. Specifically, he was able to sleep right through the night, without being woken up by pain that he otherwise experienced several times each night when his hands were exposed to cold as they slipped out from under the bed covers. Being well-rested improved his concentration and capacity to work. Ability to tolerate cold meant that he no longer required warm gloves and could work outdoors in all types of weather. He was more flexible and functional in general, able to do spontaneously things he enjoyed with family and friends, no longer having to consider pain and disability. He was able to stop taking all other pain medications and no longer needed to use the transcutaneous electrical nerve stimulation apparatus at bedtime.\nMechanical allodynia was assessed during the NoTOPain trial using standardised nylon monofilaments before and after the patient was treated with blinded cetuximab. He was asked to identify the area on his right hand with maximum pain. The monofilaments, in order of increasing size and target force, were then sequentially applied there, and he was asked to indicate when the filament triggered a painful response (using a non-painful area on the opposite side as a control). Just prior to the start of the cetuximab infusion, the patient scored 7 on the 0–10 NRS for ‘pain right now’. The first filament to provoke a pathological pain response at that time had a target force of 60 g (size 5.88). The test was repeated in the same place 2 hours after the cetuximab infusion, when his pain score was 1 on the same NRS (see ). At that time, none of the monofilaments provoked a pathological pain response (the largest filament tested having target force 300 g (size 6.65)). The test was repeated again the following day, with a pain score of 2 and again allodynia was not provoked by any of the monofilaments.\nThe patient experienced near-complete pain-relief after one of the two blinded study infusions which at study closure was revealed to be cetuximab. The open-label cetuximab infusion elicited the same response. He was, therefore, prescribed the oral EGFR-inhibitor erlotinib 150 mg daily, which he started taking once pain recurrence was well-established after his last cetuximab infusion. The patient described partial, transient improvement in NP after the first dose of erlotinib but felt that it never measured up to the dramatic improvement that he had experienced after the two intravenous cetuximab infusions in the trial.\nThe question of whether lack of efficacy of erlotinib could be a dosing question was raised so after 2 weeks taking 150 mg he increased to 300 mg daily. He took 300 mg daily for a total of 6 days but stopped because he was then convinced that erlotinib in fact had no effect at all, that is, he reported that the pain was back to the levels he had before start of the trial.\nAfter a further month of pain, treatment with the oral EGFR-inhibitor gefitinib was started. He took 250 mg gefitinib daily for 3 weeks without any change in NP severity.\nHaving failed both oral EGFR-Is that had shown benefit in several patients before, afatinib was prescribed, based on the hypotheses that a germline EGFR mutation may cause resistance to erlotinib and gefitinib, or that a broader inhibition (afatinib is a pan-HER inhibitor) may reproduce the effect he had experienced after the two cetuximab infusions in the NoTOPain trial. Therefore, 6 months after his last dose of cetuximab in the trial, the patient was prescribed afatinib 40 mg daily. He began noticing improvement in his NP on day 4 of this treatment. There was a gradual decrease in pain scores over the subsequent 2–3 weeks until a new plateau was reached (see ).\nAgain, pain relief was accompanied by improved function and enjoyment of life, as indicated by Brief Pain Inventory scores (see ).\nAfter approximately 3 months, the patient’s afatinib dose was reduced to 20 mg daily in order to test if that was a sufficient dose for pain control. The patient’s impression is that afatinib is not fully as effective as he remembers intravenous cetuximab but that 20 mg daily of afatinib is virtually as effective as 40 mg, with lesser side effects. During the treatment breaks, his pain typically recurs after 3 days.\nAfatinib may inhibit EGFRs with mutations that render them resistant to erlotinib and gefitinib. The patient‘s blood was therefore analysed for germline EGFR mutations with allele-specific PCR (Cobas EGFR mutation test v2, Roche), covering 42 different mutations in exons 18, 19, 20 and 21. None of these mutations were found.\nA year after starting afatinib, the patient was offered a trial of the HER1/HER2 inhibitor lapatinib 1250 mg daily in an attempt to reduce gastrointestinal and cutaneous side effects. He took this treatment for 7 days without noticing any improvement in his NP and therefore reverted to afatinib.\nThe patient has intermittently taken tetracycline 500 mg two times per day to treat acneiform rash while under treatment with the various EGFR-Is. He has had maximum grade 2 dry skin and mucositis. His most bothersome side effect has been grade 2 diarrhoea, for which he has taken loperamide as needed, with partial effect.
A 56-year-old man presented to our department with headache, vomiting, and gait disturbance (for 1 month). He had a past medical history of hepatitis type B virus infection and hepatic failure. He had been medically treated for hypertension and hepatitis for the previous 4 years. On admission, he had an impaired consciousness [Glasgow coma scale (GCS), 14]. Cerebellar ataxia and gait disturbance were evident. Diffusion weighted imaging (DWI) demonstrated multiple cerebellar infarctions at several intensities with perilesional edema of the left cerebellar hemisphere (Fig. ). Brain magnetic resonance imaging (MRI) did not reveal any prominent meningeal gadolinium enhancement or nodule (Fig. ). MR angiography revealed no abnormal findings. The main venous sinuses were confirmed to be patent by 3-dimensional reconstructions of MRI with gadolinium (Fig. ). Chest X-ray did not reveal any abnormal lesions, and the results of serum examination for infectious diseases, including human immunodeficiency virus (HIV), were negative except for hepatitis B virus surface antigen.\nSubsequently, the patient was diagnosed with subacute cerebellar infarction due to arteriosclerosis and was administered glycerol to control the intracranial pressure; however, 1 week after admission, his GCS decreased to 11. Computed tomography confirmed worsened cerebellar edema and hydrocephalus. External and internal decompression surgery were performed to control the intracranial pressure (Fig. ). A section of the swollen cerebellar hemisphere was removed and submitted as a surgical specimen. Additionally, external continuous ventricular drainage was performed to control hydrocephalus. Lumbar puncture to collect cerebral spinal fluid (CSF) was not performed until this time because of the risk of cerebral herniation. CSF from continuous ventricular drainage demonstrated mild inflammation (cell count, 36 /mm3; protein, 16 mg/dl; glucose, 113 mg/dl). C. neoformans was detected in CSF as well as in the surgical specimen of the cerebellum.\nHistopathologic examination of the surgical specimen revealed strong hyperplasia of the arachnoid mater (Fig. ). Fungi were mainly localized in the subarachnoid space and rarely in the parenchyma (Fig. ). Lymphocytes and multinucleated giant cells forming granulomata invaded the arachnoid and subarachnoid spaces and pia with heavy fibrosis (Fig. ). Small arteries were occasionally observed to be occluded with internal endothelial proliferation. While there were arteries in the sample, veins were rarely observed in the subarachnoid space (Fig. ). In addition, venules in the parenchyma were frequently observed to be congested. The patient was diagnosed with granulomatous meningitis due to C. neoformans and was immediately treated using liposomal amphotericin B and fluconazole; however, the ischemic lesion of the cerebellum continued to bilaterally worsen along with worsening perilesional edema (Fig. ). The patient’s course subsequently deteriorated. He developed renal failure and ultimately died 25 days after admission.\nAutopsy confirmed that the pathological changes were confined to the central nervous system and predominantly localized at the surface of the cerebellar hemisphere. Fungal bodies were widely spread along the surface and bilaterally into the deep sulcus of the cerebellum (Fig. ). Few fungi were observed in the supratentorial and intraparenchymal lesions. The lesion in the arachnoid mater of the cerebellum was roughly the same as that in the surgical specimen although it was more deeply spread into the peripheral sulcus and the granulomatous inflammation was not as severe (Fig. ).
A 51-year-old male was referred to our hospital with dysphagia and recurrent upper abdominal discomfort. Apart from arterial hypertension, no significant medical history was reported. Endoscopy detected an ulcerous lesion dorsal at the GEJ (figure ), however, biopsies did not prove malignant disease. Deep biopsies lead to the histopathological diagnosis of a GIST in the GEJ. High-resolution multislice computerized tomography (CT) showed a solid tumor measuring 7.6 cm extending from the distal esophagus to the gastric cardia and fundus with extension into of the left diaphragmatic muscular column and the splenic hilus (figure ). Surgery with curative intent at this stage would have required a multivisceral resection by an abdomino-thoracic approach, including resection of the left diaphragmatic muscle as well as splenectomy. After thorough discussion of the treatment options, the patient consented to try to downstage the tumor first by neoadjuvant treatment with imatinib mesylate followed by surgery after three to six months.\nLiver metastases were excluded by ultrasound and abdominal CT as were lung metastases by conventional chest x-ray and CT of the thorax. Before starting with drug treatment the patient underwent functional staging with 18F-FDG-PET demonstrating an increased tumor metabolism without signs of distant tumor spread. Imatinib mesylate at 400 mg per day was given orally. The patient suffered from mild diarrhoea and nausea during the treatment. The side effects were controlled by loperamide and metoclopramide. Follow-up 18FDG-PET examination two months after the beginning of the treatment showed a steep decline of 18FDG-uptake at the area of the tumor which by visual analysis of the PET could no longer be detected. This result documented response to treatment with imatinib mesylate which was continued for another four months. Follow-up CT at six months revealed a regression of the tumor diameter from 7.6 cm to 4.8 cm. The tumor margin showed a wash-out phenomenon with loss of contrast enhancement and no clear delineation (figure ). Resection of the residual tumor was felt to be possible now with preservation of the distal stomach and the spleen.\nIntraoperatively the tumor was found to be located dorsal of the GEJ. The diaphragm was incised and after mobilisation of the greater and lesser curvature and opening of the lesser sac, the tumor could be mobilized easily from the pancreas as well as from the splenic hilus. Through mobilisation of the distal esophagus the tumor was resected en-block by linear stapler technique together with the gastric fundus and cardia using the retroperitoneal fat and parts of the left column of the diaphragm for covering the residual mass and as resection margin. The postoperative specimen showed residues of the ulcerous lesion (figure ). For reconstruction of the food passage an isoperistaltic jejunal segment was inserted.\nHistopathological examination of the resection specimen confirmed a GIST with extensive regressive changes. The tumor originated from the submucosal layers and extended to the subserosa with a remaining diameter of 2.5 cm (figure ). Tumor cells were still positive for c-Kit, but the proliferation rate measured with Ki-67 expression was less than 10%. Oral and aboral resection margins were free of tumor cells as were eight perigastric lymph nodes. Molecular pathology of exon mutation analysis could not find a mutation in exons 9 and 11 of c-Kit nor in exon 18 of PDGF receptor alpha. Thus the case was classified as 'wildtype'.\nThe postoperative course was uneventful, the patient recovered quickly. He was allowed regular food intake from day four onward could be discharged from hospital at the 10th postoperative day. After recovery the patient continued antiproliverative therapy with imatinib mesylate at 400 mg per day. One and a half years later he is in an excellent physical condition and free from disease. The patient reports no restriction in the oral food uptake nor regurgitation or sourness. CT imaging and abdominal ultrasound did not show recurrent or metastatic tumor growth (figure ).
The case is of a 3.7-kg, 1-month-old male patient who presented at day 24 of life with severe aortic stenosis (AS) and coarctation of the aorta (CoA). He was born at term following an uncomplicated pregnancy. On day 5 of life, he was noted to be jaundiced and have a murmur. His jaundice resolved, and he was discharged home with cardiac follow-up. He then had progressive increase in his work of breathing and was not gaining weight. He had an echocardiogram on day 24 of life which showed a thickened and dysplastic bicuspid aortic valve with doming of the valve leaflets. The flow across the valve was measured on continuous wave doppler at 4 m/s. He was also found to have a discrete juxta ductal coarctation of the aorta with continuous doppler flow of 2.5 m/s with diastolic tail. His ventricular function remained preserved. He was started on intravenous prostaglandin and urgent surgery was scheduled.\nDue to the complexity of the case and the need for both aortic valve and aortic arch intervention, it was felt the best course of action would be a hybrid approach with surgical end-to-end anastomosis of the aortic arch via a thoracotomy followed by surgical carotid cut down for AoVP. This plan was made to avoid the need for a sternotomy on bypass to alleviate the aortic valve stenosis. The patient was taken to the hybrid catheter laboratory theater, where he was placed in the left lateral position, prepped and draped. He underwent the surgical end-to-end anastomosis via the thoracotomy via the third intercostal space, as planned. The arch, patent ductus arteriosus (PDA) and descending aorta were then dissected out. Clamps were applied to the arch and descending aorta. The coarctation segment was resected and PDA ligated. After this, an end to side anastomosis was performed between the aortic arch and the descending aorta. The pleura and chest were closed in layers.\nAt this point the patient was assessed and felt to be stable to proceed with the planned aortic balloon. The patient was turned from the lateral position to his back and the surgeon performed a carotid cutdown on the right common carotid artery. A 5/0 purse string suture was applied to the vessel, and through that a 4-Fr sheath was inserted in the right common carotid artery. Using a 4-Fr pigtail catheter, ascending aortic pressure was measured at 61/42 mmHg. An aortic angiogram was then performed and the aortic valve annulus measured 7.5 mm. The pigtail catheter was then replaced with a 4-Fr Judkins Right Coronary Catheter (JR). Using a Terumo 0.035″ guide wire the JR catheter was placed into the left ventricle (LV). The pressure in the LV was recorded at 110/13 mmHg, giving a peak to peak gradient of 49 mmHg. A 0.014 ChoICE PT extra support coronary wire (Boston Scientific) was then placed in the LV and the JR catheter removed. Next a NuMED Tyshak II 6 mm × 2 cm percutaneous transluminal valvuloplasty balloon catheter was chosen and delivered over the ChoICE wire to the aortic valve. The balloon was inflated to burst pressure of 4 atmospheres (atm) with waist seen within the balloon and dewaisting occurred. The balloon catheter was removed and the JR catheter re-introduced. The pressure was measured at 100/10 mmHg. Repeat angiogram at the aortic root at this stage did not demonstrate any significant aortic incompetence. The JR catheter and the Terumo wire were again used to recross the valve, and a 7 mm × 2 cm NuMED Tyshak II percutaneous transluminal valvuloplasty balloon catheter was chosen and delivered over the coronary guidewire in position. The balloon was inflated to 4 atm, a waist was again seen and dewaisting occurred. The balloon catheter was removed, and the JR catheter placed back into the LV. Pressure within the LV was now 90/10 mmHg with pullback peak to peak gradient between the LV and ascending aorta measuring 30 mmHg. The catheter and sheath were removed, the vessel was repaired by tightening the pursestring suture and the skin closed in layers.\nAn echocardiogram performed post procedure showed qualitatively normal ventricular function with no pericardial effusion. The arch was unobstructed with doppler flow velocity of 1.6 m/s. The aortic valve was bicuspid and obviously dysplastic. There was mild flow acceleration across the valve using continuous wave doppler of 3 m/s with mean doppler gradient of 15 mmHg. There was no obvious aortic incompetency seen.\nThe patient was transferred from the hybrid catheter laboratory to the pediatric intensive care unit (PICU). He remained cardiovascularly stable during his PICU admission. He was extubated but had to be reintubated within a few hours due to upper airway issues which prolonged his PICU stay. He was successfully extubated on day 3 post procedure and was transferred to the cardiac ward. His admission on the ward was uncomplicated and he was discharged on day 6 post intervention.\nAt 4 months of age, he was reviewed in the outpatient clinic. He was clinically asymptomatic with steady growth. His echocardiogram demonstrated qualitatively normal ventricular function, the aortic valve stenosis was mild to moderate with flow of 3.7 m/s on continuous wave doppler with mean gradient of 30 mmHg. There was evidence of mild aortic incompetence, his aortic arch remained unobstructed with flow velocity of 1.4 m/s. His most recent follow-up was at 8 months of age. He remained asymptomatic and well with his weight increasing to 9 kg. His echocardiogram again demonstrated qualitatively normal ventricular function, bicuspid aortic valve with flow of 3.2 m/s across the valve. There was mild aortic incompetence and the aortic arch remained unobstructed. There were no issues with his carotid artery access and there were no ongoing neurological issues.
A 71-year-old male with a history of chronically implanted ICD (6 years) was initially admitted to electrophysiology (EP) suite for an ICD lead extraction and replacement upgrade of the system to a biventricular functional system. During an ICD pulse generator replacement (Medtronic Viva XT CRT-D, model DTBA1D1) 2 months earlier the coronary sinus lead was noted to have a fracture due to insulation break so it was cut and capped at that time. The model of this particular implanted Left Ventricular Coronary sinus lead was Medtronic StarFix unipolar active fixation, model 4195 (implant date 6 years ago). Patient had history significant for class III congestive heart failure with underlying LBBB.\nCardiac fluoroscopy was performed to demonstrate normal position of the ICD pulse generator in the left subclavian area. The right atrial and ventricular leads tracked normally while the coronary sinus lead was noted to be fractured within the pocket with the lead remnant in the ICD pocket. Cardiac silhouette motion was normal and no evidence of pleural effusion was noted. Local anesthesia was infiltrated to the right and left groin. An 18 g arterial line was placed in the left femoral artery with good waveform. #7f and #9f venous lines were placed in the right femoral vein. Through the #9f, an intracardiac echocardiographic (ICE) probe was advanced to the level of the right atrium and ventricle. No evidence of vegetation or pericardial effusion was noted. The left subclavian area was prepped and draped. Local anesthesia was administered to the left anterior chest wall prior to opening the pocket. The pulse generator was removed from the pocket and leads were dissected to the level of the anchoring sleeves. There was a cap present over the abandoned lead remnant and this cap was removed. Tie-down sutures were removed. Leads were dissected to the level of the anchoring sleeves to free them up from posterior scar tissue. The anchoring sleeve of the coronary sinus lead was removed and gentle traction initiated; however, it was heavily scarred into position at the distal branch of the lateral branch of the coronary sinus. All 4 splines were deployed. The head of the lead was cut off and a Liberator locking stylet was advanced and locked at 1 cm from the tip of the lead. With continuous traction, a 12-French laser sheath was advanced over the lead to break up heavy fibrosis near the left brachiocephalic vein and down to the superior vena cava. The lead became dislodged situating itself at the proximal area of the coronary sinus. Continuous gentle traction of this area and laser at the ostium of the coronary sinus lead dislodged the lead in its entirety from the coronary sinus and it was removed without difficulty. There was a substantial amount of scarring at the level of the coronary sinus insertion site and around the splines before they became dislodged.\nAt this time, the patient's blood pressure became labile. Cardiac silhouette motion was decreased and ICE probe showed pericardial effusion consistent with pericardial tamponade. A subxiphoid pericardiocentesis needle was inserted and 60 cc of blood was aspirated. The patient's blood pressure responded and, however, subsequently started to decrease again. Cardiothoracic surgery was called to the EP suite, and patient was intubated and requiring massive hemodynamic support, which deteriorated into cardiac arrest requiring cardiopulmonary resuscitation. While in the EP suite, open chest sternotomy and evacuation of hematoma from the pericardium was performed. Large amount of clot was removed from behind the heart and hemodynamics stabilized despite continued bleeding. Patient was transferred to the operating room.\nIntraoperatively, the patient was immediately placed on cardiopulmonary bypass to explore cardiac structures and injury. Several injuries were noted including a large amount of bleeding from the posterior portion of the heart due to total disruption of the distal coronary sinus and large degloving injury of posterior portion of the heart with large first obtuse marginal branch bleed. In addition, the innominate vein had a large hematoma overlying a tear where it crossed the aorta. At least 2 puncture injuries to the heart were noted likely associated with pericardiocentesis.\nAfter the patient's status stabilized, electrophysiology recommended replacement of biventricular ICD pulse generator to his remaining right atrial and ventricular leads without any further manipulation of the leads with intention to eventually upgrade to a biventricular device. The procedure was successfully completed.
A 52-year-old man complained of nasal stuffiness along with bleeding, headache, and vomiting. He was referred to our department after magnetic resonance imaging (MRI) showed an enormous mass occupying the nasal and paranasal cavities and extending into the bilateral frontal base [Figure and ]. No neurological deficit other than anosmia was identified. The tumor spread beyond the nasal cavity and paranasal sinuses, and was therefore classified as stage C based on the modified Kadish clinical staging system [].[] Total removal of the tumor was achieved surgically, using a transnasal approach in combination with a bilateral front basal craniotomy [Figure and ]. The dura along the anterior skull base was opened partially and the cranial base was reconstructed using the pericranial flap. Histological examination of the tumor demonstrated proliferating tumor cells with large, oval nuclei containing prominent nucleoli. Scattered necrotic changes, nuclear fission, and Homer–Wright rosettes were identified. These findings were consistent with Hyams grade III ONB [].[] No neurological deficit other than olfactory analgesia was observed after surgery, however, the patient suffered from meningitis caused by cerebrospinal fluid rhinorrhea that developed 1 week postoperatively. Because complete eradication of the rhinorrhea and meningitis required 9 weeks of treatment, radiation therapy was started 10 weeks after the surgery. Large-field irradiation of the resected area, from the nasal cavity to the frontal lobe, was administered with a total of 60 Gy in 30 fractions. The patient was discharged 17 weeks after the surgery and was followed-up on an outpatient basis. At the time of discharge, whole-body enhanced computed tomography (CT) revealed no apparent distal metastasis or tumor recurrence.\nAt 24 weeks postoperatively, the patient noted numbness in both upper limbs, which gradually worsened. He was readmitted 25 weeks after the surgery because of rapidly deteriorating symptoms. Neurological findings on readmission revealed tetraparesis, hypesthesia, and hypoalgesia below C6, hyperreflexia of both legs, and urinary incontinence. CT and MRI revealed an intradural lesion compressing the spinal cord along its right ventral aspect at C5 through T2 [Figure –]; MRI of the head showed no evidence of tumor recurrence. In view of the diffuse nature of the disease, it was decided to surgically relieve the compression by the tumor to avoid complete tetraplegia. A laminectomy from C4 to T3 was performed. Intraoperative findings confirmed the absence of tumor in the epidural space. However, under the arachnoid membrane, the tumor compressed the spinal cord along its right ventral aspect. Because the tumor adhered to the spinal surface and was entangled in several nerve roots, it was removed but not completely. A dural plasty was then performed with a Gore-Tex® membrane to avoid restriction of the subdural space. The tumor was histologically diagnosed as ONB, consistent with the first operation [Figure and ]. After surgery, his urinary incontinence promptly improved. Paralysis of the right upper and both lower limbs remained but seemed to be improving. Residual tumor was seen on MRI performed 1 week after the surgery but the spinal cord was well decompressed [Figure –]. Whole-spine irradiation of 58 Gy in 29-Gy fractions was started 1 week postoperatively. MRI after irradiation no longer revealed residual spinal tumor, but shortly after spinal irradiation, the patient became progressively lethargic. Brain MRI showed multiple leptomeningeal enhanced lesions. General malaise and anorexia followed and the patient developed bilateral pneumonia, which caused his death 16 weeks after the surgery for ISM and 41 weeks after the first operation. During the time before his death, there had been no further deterioration in the motor weakness of his four extremities.
An 83-year-old woman presented with a growing solid right frontal scalp lesion over the past month that had not been present two months prior. Her past medical history was significant for diagnosis of a right frontal anaplastic astrocytoma, resected 18 years earlier, followed by adjuvant temozolomide and external radiation therapy (60 Gy in 30 fractions). She also underwent complete resection of stage IA lung adenocarcinoma four years prior. On examination, she was neurologically intact and cognitively at her baseline of mild dementia.\nMagnetic resonance imaging (MRI) of her brain demonstrated a bilobed homogeneously enhancing right frontal scalp, measuring up to 4.5 cm, with 2.8 cm of intracranial extension (). There were also expected encephalomalacic changes of the right frontal lobe with ex vacuo dilation of the frontal horn of the right lateral ventricle from prior astrocytoma resection. The differential diagnosis was felt to include a primary skin or soft tissue neoplasm, systemic metastasis, meningioma, or recurrence of a high-grade glioma with extracranial invasion. After consultation with neuro-oncology, surgical intervention was recommended to pursue a tissue-based diagnosis.\nThe tumor was gross totally resected via a right frontal craniectomy with mesh cranioplasty to replace the bony defect, followed by complex scalp closure that involved rotation of a vascularized forehead flap and inset of a harvested split-thickness skin graft from the thigh. Pathologic review of the specimen revealed a densely cellular spindle cell malignancy composed of highly pleomorphic cells in a partly fascicular or storiform pattern with areas of necrosis (Figures and ). The Ki-67 index was >75% (), and p53 nuclear expression was observed in >90% of cells (). Further immunohistochemical stains for vimentin were strongly positive, while those for S100, GFAP, EMA, and panCK were negative. Together these findings were suggestive of a high-grade pleomorphic sarcoma, French Federation of Cancer Centers Sarcoma Group (FNCLCC) grade 3. The patient recovered from surgery uneventfully. Her family declined further treatment, given her baseline poor performance status and age.\nA targeted cancer gene panel (Oncomine Comprehensive Assay v3, Thermo Fisher Scientific, Waltham, MA, USA) was performed on the surgical specimen and peripheral blood, the latter serving as a normal, germline control specimen. This assay examines tumor DNA for mutations and/or amplifications in 146 cancer-related genes, as well as tumor RNA for the presence of gene fusion transcripts involving 44 oncogenic driver genes. This assay revealed somatic variants within the tumor, predicted to be deleterious based on SIFT [] and PolyPhen [] prediction algorithms, of KIT (V603D) and TP53 (Y220C), as well as 7 copy number amplifications of PDGFRA and KIT ().\nThese results were cross-referenced with The Cancer Genome Atlas (TCGA) sarcoma cohort [], comprising 206 samples, including 44 cases of UPS. There were four total KIT variants predicted to be deleterious in this database in three patients, comprising two cases of leiomyosarcomas (patient 1: R804Q; patient 2: W557Gfs∗18, Q556Rfs∗8) and one malignant peripheral nerve sheath tumor (C906R). The KIT V603D mutation observed in our patient was located in the protein tyrosine kinase domain and plotted alongside data from the TCGA database with MutationMapper [, ] (). TP53 mutations were reported in 69 (33.5%) patients in the TCGA sarcoma database, including 18 (26.1%) cases of UPS. Regarding copy number alterations, the TCGA database reported three cases of sarcomas with copy number amplifications in KIT, comprising two UPS and one myxofibrosarcoma. Notably, these three comprised the majority of the four total cases in the database that harbored PDGFRA copy number amplification with the remaining case also being UPS.
A 24-year-old male with HA was admitted to our department with pain in multiple joints on May 23, 2011. The patient had a medical history of hemophilia A since the age of 3 and was intermittently treated with factor VIII. During these years, he sequentially developed left knee, left elbow, left hip, and right knee joint pain and swelling with limited activity and was soon diagnosed as HA. Initially, the joint manifestations could be largely relieved by factor VIII replacement therapy. Factor VIII inhibitor screening remained negative. Later, factor replacement therapy failed to achieve satisfactory effects, so in 2002 and 2006, he received left elbow synovectomy and left total hip arthroplasty, separately. In the subsequent years, the patient still suffered from the recurrent episodes of left elbow and bilateral knee joints hemorrhage, pain, and swelling. In recent 2 years, the frequency of joint hemorrhage had increased to approximately 2 times a week and only slightly relieved after factor VIII replacement therapy. Currently, the activity of those joints was limited to various degrees. Other medical history involved 2 cerebral hemorrhages 18 and 15 years ago, separately.\nOn physical examination, significant tenderness was noted in the left elbow joint with limited pronation and decreased grip strength. The preoperative Mayo elbow performance score (MEPS)[ was 55 for the left elbow. Moreover, knee valgus (left 20° and right 15°) was noted, and hyperextension, hyperflexion, and positive grinding test results were noted in both knee joints with a swollen and warm right knee. The preoperative Hospital for Special Surgery (HSS) knee scores[ were 58 for the left knee and 65 for the right knee.\nBilateral knee joints and left elbow joint exhibit advanced arthropathy on radiographs (Figs. A and 2A). These joints present narrowing of joint space, erosions of the articular facets, and bone deformation to various degrees.\nOur diagnosis was hemophilia A and HA of the left elbow joint, both knee joints, and left hip joint. The patient received left elbow synovectomy and left total hip arthroplasty, but the condition continued to deteriorate over time with worsening of the left elbow and both knee joints. Taking all of these factors into account, surgical methods were our top priority, and simultaneous total multi-joint replacement was indicated. Due to the complicated joint lesions and medical conditions, our preparations for this arthroplasty were far more sufficient than usual. Given that arthroplasty for patients with hemophilia A, particularly the simultaneous replacement of multiple joints, is challenging, the patient and his family were informed in detail of the possible benefits and risks of the surgery. We performed a full musculoskeletal assessment and thorough medical evaluation beforehand. Blood products were prepared for possible bleeding events. Then, our team performed bilateral total knee arthroplasty (Zimmer NexGen) and left total elbow arthroplasty (Zimmer) under tightly regulated factor VIII replacement therapy. Antibiotic prophylaxis was administered 30 minutes prior to surgery, and an additional dose was administered once during the operation. Local hemorrhage was carefully controlled to prevent secondary joint damage. Approximately 1800 mL blood was lost during the entire surgery. The patient received 900 mL blood by autotransfusion and 4 units of red blood cells plus 800 mL fresh frozen plasma by intraoperative infusion. During surgery, we observed hemarthrosis and villous synovial hypertrophy at the joints, and severe erosion of the articular surface and various degrees of bone deformation were noted. These findings confirmed the preoperative diagnosis and preoperative assessments. After surgery, hemostasis management, such as compressive bandage, factor VIII infusion, and rigorous monitoring of coagulation indicators, was performed. An early rehabilitation program was applied to achieve improved regain of function.\nWe managed factor VIII replacement therapy during perioperative period under the guidance of hematologists. On the day of surgery, 3000 U/12 h (the body weight of this patient is 63 kg) factor VIII (ADVATE) was administered intravenously followed by 2000 U/12 h on postoperative days 1 to 3 (POD 1–3). Then, on POD 4 to 6, a dose of 1500 U/12 h was administered followed by 1000 U/12 h over the following 6 weeks. Factor VIII inhibitor remained negative in perioperative tests.\nAt the follow-up, the patient's joints functioned well. The MEPS of the left elbow was 85, and the HSS score of knee joints were 71 (left) and 81 (right). On radiographs (3 months and 5 years after operation), the arthropathy of bilateral knee joints and left elbow joint was significantly relieved (Figs. B,C and 2B,C).
A 73-year-old female patient was admitted for further evaluation due to a suspected right lesser trochanter pathologic fracture on the hip X-ray and bone metastasis on the whole body bone scan. The patient was on medication for hypertension and hypothyroidism. She was also diagnosed with pulmonary tuberculosis 10 years ago and was cured after treatment. The patient had been taking antiplatelet agents for 9 years after intra-arterial stent insertion due to right internal carotid artery stenosis. SARS-CoV-2 RT-PCR was performed because the patient had a fever of 37.8°C one week before admission. A negative result was reported. On the second day of admission, a fever of 38°C or higher was observed; thus, SARS-CoV-2 RT-PCR was performed but negative results were confirmed. Chest radiography showed no specific findings other than the old pulmonary tuberculosis lesion in the right upper lung. Later, the fever subsided.\nOn the 4th day of admission, chest CT showed no specific findings except for the multifocal post-infectious old lesions in the right lung, and there was no change compared to the previous chest CT (). Therefore, on the 5th day of admission, an excisional biopsy of the right femur was performed. Considering that the possibility of COVID-19 infection was excluded, the patient was transferred to a room different from the previous one after the excisional biopsy was performed. After the biopsy, a fever of 37.5°C or higher was noted and the C-reactive protein was elevated. As a result of the biopsy, a lymphoma was suspected.\nOn the 9th day of admission, a right femoral shaft pathologic fracture occurred, and it was decided to perform a closed reduction of the fracture with internal fixation at the emergency on that same day. We considered that the patient had been confirmed to be negative for SARS-CoV-2 by RT-PCR 6 days before admission and on the 2nd day of admission, and there was no evidence of active infection on chest CT on the 4th day of admission. For the persistent fever, we consulted with the division of infectious disease and infection control department. The division of infectious disease and infection control department suggested that the patient's fever seemed to be a neoplastic fever caused by lymphoma. Therefore, we excluded the possibility of a SARS-CoV-2 infection in the patient and decided to perform the surgery with standard PPEs including surgical gloves, gown, eye shields, and N95 respirators. Since the patient was taking an antiplatelet agent (clopidogrel) due to internal carotid artery stenosis, we decided to administer a general anesthesia to the patient. After preoxygenation, propofol 2 mg/kg was administered for anesthesia induction, and rocuronium bromide 0.6mg/kg was administered. Tracheal intubation was performed using McGrath MAC (McGRATH, Aircraft Medical Ltd.) and anesthesia was maintained with 1.5–2.0 vol% sevoflurane and remifentanil infusion. Oxygen saturation was maintained at 100% during anesthesia, and blood pressure, heart rate, and mechanical ventilation were maintained stable.\nThe surgery lasted 85 min. Then, sugammadex 2 mg/kg was administered to reverse the neuromuscular blockade. Five minutes after administration of sugammadex, the neuromuscular blockade was completlely confirmed; therefore, tracheal tube extubation was performed. After that, the patient was transferred to the PACU for recovery. After an hour, the PAR score was checked at 9 points; therefore, the patient was moved to the hospital room.\nOne day after the surgery, the patient developed a fever of 38.4°C again, and another patient in the same hospital room was confirmed to be COVID-19 positive. Therefore, the chest CT and SARS-CoV-2 RT-PCR test were redone on the patient. On chest CT, bilateral pleural effusion was observed (), and the SARS-CoV-2 RT-PCR results were positive. As a result, the patient was confirmed to be COVID-19 positive and moved to the isolated room. After that, Lopinavir, ritonavir, and hydroxychloroquine were administered for COVID-19 treatment and antibiotics were administered in consideration of possible bacterial pneumonia.\nWe investigated healthcare workers who participated in the surgery. In the PACU, all healthcare workers were wearing standard PPEs, so there were no exposed healthcare workers, but confirmed that the anesthesiologist and nurse who participated in the surgery were exposed because they did not wear eye shields when they performed the anesthesia. Therefore, we ran SARS-CoV-2 RT-PCR on these two healthcare workers, and made them stay at home for two weeks. For the two tests, both showed negative results on SARS-CoV-2 RT-PCR. Fever and cough symptoms were not observed for two weeks. After two weeks, on the SARS-CoV-2 RT-PCR, both healthcare workers’ results were reported as negative.\nBy the way, the operating room was also exposed to the possibility of infection. Therefore, we performed surface disinfection, emptied the operating room for 24 h, and performed room air ventilation. A carbon dioxide absorber of the anesthesia machine was also replaced as we performed disinfection. 5 days after the surgery, a multifocal patchy ground-glass appearance (GGA) lesion in both lungs which were suspected to be viral pneumonia was reported on the chest CT scan (); therefore, antibiotics and hydroxychloroquine were administered. However, pneumonia had not improved, and the patient was given a ventilator care in the intensive care unit. Twenty-three days after surgery, pneumonia was aggravated and the patient was died.
A 5-year-old boy with a history of uncontrolled, moderate-persistent asthma presented to the pulmonology clinic for asthma management. The patient had a previously normal developmental history, up-to-date immunization record including Streptococcus pneumoniae and Haemophilus influenzae, and notable past medical history significant for recurrent otitis media due to poor compliance to oral antibiotics, which were subsequently successfully treated with parenteral antibiotics. Upon presentation, the patient recently had a left lower lobe community-acquired pneumonia that was treated with one dose of intramuscular ceftriaxone in the emergency department followed by a standard 10-day course of amoxicillin. The patient's family was instructed to open the capsule and mix the powder with food to improve compliance due to known history of poor palatability with previously prescribed antibiotics. The patient returned to the emergency department two weeks later with worsening of symptoms and was treated with oral cefdinir for unresolved pneumonia. He was subsequently seen in the pulmonology clinic four days later to establish care without acute complaint. He was started on fluticasone/salmeterol and a five-day course of oral prednisolone for management of an acute asthma exacerbation.\nOne month later, the patient presented to the emergency department with fever and increased cough and was diagnosed with right middle lobe pneumonia. He was treated with intramuscular ceftriaxone to be followed with a home course of oral antibiotics, which was reportedly completed. Two weeks later, the patient again presented to the emergency department with recurrent symptoms and diagnosed with right lower lobe pneumonia that failed outpatient treatment. The patient was subsequently admitted to the medical floor and managed with a single dose of intravenous ceftriaxone. He was discharged the following day in stable condition on cefdinir and azithromycin. The patient was to follow-up as an outpatient with his pulmonologist and primary care physician. Hospital laboratory work including CBC with differential, peripheral blood cultures, sweat chloride test, immunoglobulin assay, T cell count, and Aspergillus antibody was unremarkable. At the two-week follow-up, he had persistent fever, wheezing, daytime and nighttime cough, shortness of breath, exercise intolerance, and difficulty breathing. The patient was scheduled for further evaluation with high-resolution chest CT without contrast and flexible bronchoscopy with bronchoalveolar lavage and nasal cilia biopsy to evaluate for Primary Ciliary Dyskinesia (PCD). Repeat chest X-ray at that time revealed an improved right lower lobe consolidation without complete resolution, as expected. CT scan revealed a mild degree of bronchiectasis in the lower lobes, bilaterally, associated with consolidation on the right and linear atelectasis and scarring on the left with other nonspecific evidence of chronic inflammatory versus infectious process. Bronchoscopy identified multiple mucus plugs in the right bronchus intermedius (Figures –), right middle lobe (Figures and ), and right lower lobe (). Gross inspection of the bronchoscopy sample revealed a solid, rock-like mucus plug (). Airway clearance via a high-frequency chest wall oscillation vest along with nebulized therapies was initiated. The patient did not tolerate postoperative oral azithromycin and required mixing with grape juice.\nOne week following the procedure, he was readmitted for recurrent fever and a new rounded lucency on chest X-ray in the right lung base, suspicious for cavitation or pneumatocele. Chest CT demonstrated a 2.5 × 3.2 × 3.7 cm area of cavitation within a region of consolidation or bronchiectasis in the posterior aspect of the right lower lobe with mediastinal and right hilar adenopathy, suspicious for cavitary bacterial pneumonia or fungal disease (). Home treatment continued with the addition of intravenous ceftriaxone. Bronchoalveolar lavage culture from the previous week grew beta lactamase negative Haemophilus influenza and Moraxella catarrhalis. Fungal culture, mycobacterial culture, bronchial aspirate cell count, cytology, gastrin pepsin assay, and pathology report were all unremarkable. A PICC line was placed for 3 weeks of home antibiotics as a management for complicated cavitary pneumonia. Further workup included QuantiFERON-TB Gold, HIV assay, complement assay, tetanus antibody, diphtheria antibody, and pneumococcal antibody which were all unremarkable.\nAt 3-week follow-up post hospitalization, the patient was symptomatically improved without coughing or fever and he was reported to be back to his baseline. The mother reported adherence to antibiotic regimen through the PICC line. He continues maintenance therapy with fluticasone/salmeterol, montelukast, and vest therapy. He was started on oral azithromycin three times weekly. At the two-month follow-up, the patient remained asymptomatic, and repeat imaging at the nine-month follow-up revealed complete resolution of cavitating consolidation with residual bilateral mild bronchiectasis ().
A 15-year-old girl was admitted to the emergency department due to strongest headache, ataxia, vomiting, and progressive somnolence. The complaints had started 24 hours prior to admission, following a three-week period of abnormal fatigue. Four weeks before admission, the patient had started to take an oral contraceptive for family planning purposes, any other medication was denied.\nProgressive somnolence necessitated intubation and assisted ventilation. The initial cCT and successive cMRI scan revealed an extensive cerebral venous sinus thrombosis (CVT) of the straight sinus, left transverse sinus, and the occipital part of the superior sagittal sinus with subsequent congestive infarction of the basal ganglia and thalami (Figure ). During the immediate angiography, a 6 cm venous thrombus could be interventionally removed by aspiration. Mechanical thrombectomy was carried out under general anesthesia. A diagnostic catheter was placed to the left internal carotid artery via the right femoral artery. A diagnostic run was performed confirming CVT of the internal cerebral veins, the vein of Galen, the straight sinus and the proximal part of the left transverse sinus (Figure ). A therapeutic catheter was introduced via the right femoral vein to the left jugular bulb, and advanced to the level of the thrombotic occlusion of the left transverse sinus. Continued aspiration was carried out for 20 minutes within the transverse sinus and secondary within the straight sinus. After aspiration thrombectomy, flow was restored within the transverse sinus (Figure ). However, progressive congestive brain edema required implantation of an intracranial pressure monitor and subsequently an external cerebrospinal fluid drainage and surgical decompression by partial removal of calvarium bone. Intracranial pressure improved continuously, so that drainages and monitor could be removed after 2 weeks. After cessation of ventilation and anesthesia, the girl initially showed a left-sided hemiparesis and a motoric aphasia. She was able to communicate by movements with her eyes and head. A control cMRI 2 weeks post intervention revealed amelioration of basal ganglia congestive infarction alterations (Figure ). During the following rehabilitation, she fully recovered, and on examination 5 months later, she showed no neurological sequelae. The girl returned to school with normal performance.\nImmediately after diagnosis of cerebral venous sinus thrombosis, therapy with unfractionated heparin was initiated and changed to low molecular weight heparin 2 weeks later. After 5 months, anticoagulative treatment was shifted to phenprocoumon. The oral contraceptive had been discontinued instantly after initial hospital admission.\nAn extensive diagnostic work-up regarding thrombophilia including lipoprotein a, prothrombin G20210A and factor V Leiden mutations revealed no abnormal findings. Methylenetetrahydrofolate reductase (MTHFR) gene analysis showed the thermolabile variant c.655C>T (p.Ala222Val) in heterozygous state, but homocysteine in plasma was repeatedly normal. Proteins C, S, and factor XI were abnormal. Of note, AT was persistently low. Subsequently, SERPINC1/AT gene analysis was performed but revealed no abnormal findings. Further evaluation of causes of reduced AT activity revealed an abnormal glycosylation pattern in transferrin isoelectric focusing analysis, indicating a congenital disorder of glycosylation (CDG) type 1 (Figure ). Enzymatic analysis of mannose phosphate isomerase (MPI) in leukocytes displayed absent activity, proving MPI-CDG (Figure ). This was confirmed by MPI gene analysis showing compound heterozygous occurrence of the variants c.655C>T/p.Arg219Trp and c.1178G>C/p.Gly393Ala. The patient's mother carried the variant c.655C>T/p.Arg219Trp in heterozygous state and showed c.1178G as wildtype allele; DNA of the father, however, was not obtainable. Both variants have not been reported in the literature yet but showed an allele frequency of 0.002% and 0.02%, respectively, according to the dbSNP database (entries rs138891630 and rs201815588). They were therefore considered pathogenic. An extensive evaluation of the preceding medical history did not reveal any gastrointestinal, hepatic or endocrine symptoms during infancy, childhood and adolescence (Table ), as they have been frequently reported in other MPI-CDG patients.\nFollowing the diagnosis, a therapy with oral supplementation of D-mannose was initiated at a dose of 0.9 g/kgBW per day in 3 to 4 divided doses. AT, protein C, protein S and factor XI activities almost or fully normalized within 2 weeks (Figure ), and phenprocoumon was discontinued. However, the patient suffered from loose stools and independently reduced the dose to 0.6 g/kgBW per day. Under this regimen, AT activity decreased again, so that the mannose dose was stepwise increased to 0.75 g/kgBW per day divided into 5 to 6 doses. On this mannose dose, AT normalized again (Figure ), without any further gastroenterologic complaints.
A male patient, 25 years old, attended Yonsei University College of Dental Hospital complaining of pain in the maxillary right central incisor (tooth #21), which presented a history of trauma on tooth #21. After clinical and radiological examination, calcified canal was observed to be significant. Under a surgical microscope, localization of root canals was attempted twice but failed as patient continued to show clinical symptoms. Therefore, endodontic microsurgery was suggested, and patient consent was obtained. A CBCT scan was taken and apical radiolucency and completely blocked root canal with intact buccal bone were identified. Alginate impression was taken and poured up in yellow stone to obtain study cast. With conventional root-end resection, vast amount of buccal bone removal was inevitable and therefore, the use of minimally invasive guided endodontic microsurgery by using surgical template, cone-beam computed tomography and 3D printing technology was suggested as an alternative.\nThe study cast made by alginate impression was scanned and transformed to the digital data using tabletop scanner (Identica Blue; Medit Co., Seoul, Korea). Both CBCT data and digital data obtained from patient's study cast were imported to implant planning software (In2Guide; Cybermed Co., Seoul, Korea). Two sets of data were merged for the tooth image, so the surgical template was designed accordingly.\nThe guide template was designed to cover 5 teeth with maxillary left central incisor placed in the middle. Among various guide drills with different diameter contained within In2Guid Universal implant kit (Cybermed Co.), which is compatible with the guide template, anchor pin drill with 1.5 mm in diameter was selected for targeting root apex and sleeves to accommodate anchor pin drill was designed inside the template. The position and direction of sleeve were determined in consideration of approachable drill angulation as well as accessibility for minimum of 3 mm preparation depth. Finally, it was once more confirmed if the drill accurately aims for target root without damaging the adjacent root or surrounding vital structures. () Designed guide template was exported in forms of STereoLithography (STL) file and printed with 3D printer (Objet 260; Stratasys Co., MN, USA).\nLike conventional oral surgery, patient was prescribed amoxicillin (250 mg) and Ibuprofen (400 mg) 1 hour before surgery. Before starting microsurgery, surgical guide was applied to upper anterior area for checking its adaptation. Incisal opening of guide was helpful for fully adaptation between tooth and guide (). The Leubke-Oschenbein flap design was applied after 1 ample local anesthesia (2% lidocaine with epinephrine 1:80,000; Huons, Seongnam, Korea) for minimal invasion. All surgical procedures were performed with an operating microscope (OPMI PICO; Carl Zeiss, Göttingen, Germany) ().\nUnder the application of guide, surgical site was punched 3 mm depth with 2 mm straight rotary instrument ( and ). After removing guide template, punched buccal bone was checked. Only apical buccal bone was prepared again with a No.6 round rotary straight instrument (No. 6; Prima Classic Prima Dental Group, Gloucester, UK) under distilled irrigation (). Exposed root tip was confirmed with methylene blue. And then root tip was finally prepared and removed with diamond high speed bur (). After removing of all inflammatory tissues, the resected root surfaces were then stained with methylene blue and inspected with micro-mirrors (Obtura Spartan, Fenton, MO, USA) under ×20 magnification to detect the canal space. The root-end preparation was made with KIS ultrasonic tips (Obtura Spartan). ProRoot MTA (Dentsply Tulsa Dental, Tulsa, OK, USA) used with the root-end filling material. ProRoot MTA was incrementally placed under the bleeding control, after that, the wound site was closed and sutured with 5 × 0 monofilament sutures. A post-operative radiograph was taken and compared with pre-operative radiograph (). Only 30 minutes was taken for entire operation.\nAfter 1 month follow up, clinical symptom was completely resolved. No other signs of adverse effects around surrounding soft tissues were identified.
A 38-year-old Caucasian woman, 35 weeks into her first pregnancy, presented to the emergency department for acute right-sided hip pain which precluded weight-bearing. Her right leg was shortened and externally rotated - there was no bruising or evidence of trauma.\nThe patient’s history was significant for hereditary thrombophilia (Factor V Leiden) and secondary anemia. Hip radiography revealed an unstable, displaced, right-sided femoral neck fracture with no evidence of osteonecrosis (Figure ). The decision to administer radiography, in this case, was based on the American College of Radiology guidelines, which cite an absence of in-utero deterministic effects of ionizing radiation effects after 27 weeks of gestation. Unfortunately, it was not possible to evaluate the symptoms of the patient with MRI at this time due to the coronavirus disease pandemic-induced stress on the healthcare system of our country.\nThe patient denied falls or trauma during the pregnancy, nor was there any history of smoking, alcohol abuse, use of glucocorticoids, or presence of rheumatologic/oncologic disease. Additionally, the patient was not malnourished, she underwent routine antenatal care, and took multivitamins. Serologic tests for inflammatory markers, as lab tests for serum calcium, phosphate, alkaline phosphatase, parathyroid hormone, vitamin D, and D-dimer returned normal.\nDuring multidisciplinary rounds, it was decided that delaying surgery was the best course of action out of fear of causing either mechanical or fluoroscopy-induced damage to the fetus during total hip arthroplasty. Five days later the patient experienced premature rupture of membranes, which was managed with emergency cesarean section (C-section) - no complications were encountered and a healthy 2300 g female was successfully delivered. Three days later the patient was transferred to our orthopedic surgery department for the treatment of the fracture. The significant degree of displacement (grade IV) of the fracture lasting over one week precluded open reduction with internal fixation due to fears of femoral head necrosis. During our literature review, we encountered a similar case of femoral neck fracture with grade IV displacement that was treated with open reduction internal fixation - despite restoration of blood flow to the femoral head within 15 hours, the authors still encountered femoral head necrosis with collapse six months later []. Given the considerable delay between symptom presentation and treatment, we decided the case warrants total hip arthroplasty instead of native hip salvage. Hemiarthroplasty was considered but was ultimately discarded as the conversion rate to total hip arthroplasty in young patients remain relatively high and the fracture was subsequently treated with a total uncemented prosthesis (Figure ), consisting of a 50 mm cup with 32 mm ultra-high-molecular-weight polyethylene insert and a 32 mm head with a 4 mm ceramic insert (Link Inc., Hamburg, Germany). Postoperative radiography confirmed prosthesis placement (Figure ); antibiotic and anticoagulant prophylaxis was initiated with ampicillin/sulbactam and enoxaparin sodium, respectively.\nThree days after the intervention the patient developed moderate abdominal pain without fever and accelerated intestinal transit, which both worsened over the next four days. Given the clinical presentation, there was a high index of suspicion for infection with Clostridium difficile (C. diff), although the diagnosis was ambiguous as the enzyme immunoassay (EIA) for the C. diff-specific antigen glutamate dehydrogenase was positive, while EIA for exotoxin A and B were negative. Due to exacerbation of symptoms and development of moderate hypokalemia (2.9 mEq/L), empiric treatment with metronidazole was began and marked rapid improvement. The patient was discharged five days later.
A 31-year-old female reported with a history of developing a progressing mass 7 years ago on the left lateral wall of the nose with the symptoms of epistaxis, which further lead to the unilateral nasal obstruction which was not relieved with medication. She underwent four surgical interventions in a span of 6 years in various hospitals at Bengal, Bhopal, and Kolkata due to the recurrence of the tumor to a huge size of almost resembling an elephant trunk [Figure and ]. Under histopathological examination, the tumor was diagnosed as “HEMANGIOPERICYTOMA.” The lesion was surgically removed, and subsequently, the patient was made to undergo radiotherapy and chemotherapy. The extensive surgery left her with huge embarrassment and humiliation whenever she interacted.\nDuring the examination, the patient-related dissatisfaction with her appearance and was especially concerned about her facial disfigurement []. There were a large septal perforation and the bilateral absence of mucous blanket, which lead to mucosal atrophy []. Thus, there was a postoperative nasal breathing dysfunction. The only provision of breathing for the patient was through the large defect after excision of the tumor.\nThis defect was elliptical with obliterated edges measuring about 6 cm long and 2 cm wide with a depth of more than that 3 cm along the left side of the face []. The exposed structures of the defect were conchae and meatus with an intact nasal bone, lateral part of the septal cartilage, frontal process of maxilla, and medial wall of the orbit and the cribriform plate of the ethmoidal bone []. With the existing situation, the patient was extremely depressed psychologically, and hence, it was important to treat with an interdisciplinary approach of psychiatry, and ENT. Once the patient was got to a confidence level, a prosthodontic approach was made.\nVarious prosthetic treatment modalities ranging from acrylic resin partial facial mask to an implant-retained silicone prosthesis[] were explained and discussed with the patient. Due to economic constraints and remoteness of her area of residence, the patient denied to come for repeated recalls and reviews. Hence, the prosthesis made of polymethyl methacrylate (PMMA) resin was chosen to be made. There was absolutely no viable bone left over to plan for implant-supported Maxillofacialprosthesis (MFP). Hence, it was decided to use a spectacle glass frame for retaining the prosthesis.\nThe patient was draped, and the petroleum jelly was applied to the patient's eyebrows and eyelashes. Moist gauze was packed to prevent the flow of material into the undesired areas of the defect, and an impression was made of the defect and adjacent tissues using an alginate in a semi-upright position to minimize tissue bed distortion [] The impression was then poured with the type III dental stone (Kala Stone; Kala Bhai Pvt Ltd., Mumbai, India) to achieve a cast []. A model of the prosthesis was sculpted on the facial cast with No. 2 dental modeling wax (MDM Corporation; Delhi, India). Taking into account the patient's general appearance, the esthetic contours were developed. With repeated surgeries, the patient was suffering from nasal stenosis due to which she was unable to breathe through her nares instead the only possible way she could breathe was through the defect. Hence, closing the defect with the prosthesis would make her inconvenient to breathe. Therefore, it was planned to incorporate two slits on the facial prosthesis, to facilitate the passage of air to make her comfortable to breathe [Figure and ] In the wax pattern, two longitudinal vents were then provided on the patient's left lateral wall resembling part of the wax pattern as a provision for breathing for the patient [] The wax pattern adaptation on the patient's face was checked, especially in the border areas for the close adaptation [] Spectacles with a thicker frame with the power of the patient were chosen and adapted at the nasal bridge area of the wax pattern to increase the surface area and close adaptation of the spectacles to the prosthesis [] Once the fitting and approximation have been confirmed, further laboratory procedures of dewaxing and acrylization have been followed Care should be taken, that the vents should be blocked out with an elastomeric putty material. As the wax pattern to be processed had made contours and undercuts, a three-part mold pouring (multisectional mold)[] was necessary to achieve easy placement of acrylic. Investing the wax pattern in the lower half of a large flask was the first pour. Apply separating medium to the stone surface. Invest the posterior undercut surface of the wax pattern with dental stone. The walls of this section must cover toward the center of the flask and are considered as a second pour. The dental stone should be at least 0.5 cm thick in all areas. Apply separating medium to the stone surfaces. Invest the lower half of the wax pattern with the third pour of dental stone and close the lid, and further acrylization was carried out by short curing cycles Heat cure polymethyl-methacrylate material of three shades, namely tooth colored, pink, and clear acrylics were chosen. Shade matching was done by intrinsically staining the material with acrylic stains crylization of the prosthesis was carried out by short curing cycles. The final prosthesis was been tried on the patient, and shade matching was done by extrinsic staining The placement of the prosthesis was demonstrated to the patient and was then delivered. Detailed instructions regarding care and use were provided to the patient [].\nThe patient was scheduled for the first postinsertion adjustments, 1 day after the insertion to ensure the health of the tissues and to relieve the prosthesis for pressure areas on the tissues.\nAt the follow-up evaluation after 2 weeks, the prosthesis appeared to be functioning within normal limits. The patient was then asked to come for recall visit once in every 3 months for the evaluation of prosthesis and observation of any recurrence.
A 79-year-old man who was a current smoker was admitted to our hospital for hemoptysis in July 2011 (day 0). Before admission, pulmonary emphysema and multiple pulmonary bullae were detected on a chest computed tomography (CT) scan, and we also discovered that the patient had hypertension. He had a history of lobectomy of the left lower lobe for lung cancer in 1993, with no recurrence; two episodes of bacterial pneumonia; gastrectomy for gastric cancer in August 2010; right spontaneous pneumothorax that required tube drainage in November 2010; and hospitalization for dyspnea in February 2011, at which time chest radiographs showed left lung opacity and a chest CT scan showed consolidation with pleural effusion in the left lung. Several bacterial antibiotics failed to improve the opacity on chest radiographs. A sputum culture was negative for Aspergillus. The lack of efficacy of bacterial antibiotics and the presence of multiple bullae and precipitating antibody to Aspergillus in the serum (day -163) led to a presumptive diagnosis of chronic necrotizing pulmonary aspergillosis. Voriconazole with a loading dose of 800 mg a day followed by a maintenance dose of 400 mg a day was started on day -155. A switch from intravenous to oral administration of voriconazole at a dose of 400 mg a day was made on day -106. With administration of voriconazole, the opacity on chest radiographs had significantly improved by June 2011. The patient underwent transurethral resection of bladder cancer in March 2011. Oral voriconazole was continued at our outpatient clinic until his admission for hemoptysis. He had no hematologic neoplasm.\nOn admission, a chest radiograph (a) showed consolidation in the upper field of the left lung and an infiltration shadow in the middle and lower fields of the left lung. A chest CT scan (b) showed consolidation in the pulmonary bullae adjacent to the aortic arch. These shadows were considered to be due to hemorrhage. Bronchoscopy revealed slight bleeding from left B1+2 without evidence of a tumor. Laboratory tests showed a normal count of polymorphonuclear leukocytes. Serological human immunodeficiency virus (HIV) testing was not performed, but the patient had no obvious risk factors for HIV infection. Serum precipitating antibody to Aspergillus was positive on day 2. The amount of expectorated blood decreased with rest, but massive hemoptysis occurred on day 16. Despite bronchial artery embolization, a second massive hemoptysis occurred and the patient died on day 21. The final day of voriconazole administration was day 20.\nAt autopsy, the pulmonary bullae were filled with blood. A macroscopic examination showed a hole in the aortic wall into the bullae (). This hole was unlikely to have been a consequence of the autopsy procedure because a microscopic examination of the tissue around the hole showed extensive accumulation of inflammatory cells (a–c). This microscopic examination also revealed small fungal lesions in the pulmonary bullae adjacent to the aortic wall (a–c). The diameters of these lesions were approximately 4 mm. The fungal hyphae were 5-10 μm in width, with septae and a dichotomous pattern of branching at 45°. These features are consistent with those of Aspergillus species. Microscopically, extensive necrotic lesions with Aspergillus hyphae were found in the media of the aortic wall (a and b). Immunohistochemical analysis with an anti-Aspergillus antibody (rabbit polyclonal antibody, anti-Aspergillus antibody ab20419; Abcam plc, Cambridge, UK) identified Aspergillus hyphae in lesions in pulmonary bullae (a and b) and in the necrotic media of the aortic wall (a and b). This established the diagnosis of aspergillosis. There was no other histopathologic evidence for aspergillosis elsewhere in the body, but we note that autopsy was not performed on the brain. There was no histopathological evidence for recurrence of gastric cancer, lung cancer, or bladder cancer. Prostate cancer was also found at autopsy.
A 35-year-old female patient presented to the Department of Conservative Dentistry and Endodontics (Manubhai Patel Dental College and hospital, Vadodara, India) with a chief complaint of intermittent pain in the past 1 month and discoloration of the upper right and left central incisor (11 and 21). The patient gave a history of trauma approximately 20 years ago when she met with an accident; thereafter, she noticed a gradual change in the color of crown of both central incisors, which became dark yellowish. The medical history of the patient was noncontributory. Intraoral examination revealed discoloration of tooth 11 and 21 with tenderness to vertical percussion. Tooth 11 and 21 also showed a slight fracture of the incisal edges with slight rotation of teeth and anterior open bite indicative of the luxation injuries suffered due to trauma [Figure and ]. Thermal and electric pulp response was negative with tooth 11 and 21, whereas adjacent teeth showed normal response. Both teeth 11 and 21 did not show any signs of mobility.\nThe preoperative IOPA radiographic examination [] revealed an irregular, large radiolucent area in the cervical and middle third of the external root surface at the mesial aspect of 11 suggestive of ICR. Furthermore, 11 shows resorption in the apical third on the distal aspect of the root. Preoperative IOPA radiograph [] also showed pulp canal stenosis in 21. CBCT was performed to determine the extent and depth of the lesion in the three spatial levels. Based on the CBCT images and three-dimensional (3D) reconstructions [], a diagnosis of cervical external resorption of Heithersay[] Class IV and apical resorption in 11 was determined. Completely obliterated pulp canal with only pinpoint trace of canal and diagnosis of root canal stenosis was made in 21.\nThe treatment plan included endodontic treatment and surgical intervention for removal of the inflamed granulation tissue that occupied the lesion cavity and repair of the resorption defect with Biodentine in 11 and nonsurgical endodontic treatment for 21. Before planning for the surgical procedure, patient's platelet count (4 lakh/mm3), hemoglobin (14 g/dl), bleeding time (3 min), and clotting time (5.5 min) were assessed and found to be within the normal limits.\nAfter rubber dam isolation of tooth 21, access opening was done with the help of long neck taper fissure bur via palatal approach. Scouting of the root canal orifice was done using a DG 16 explorer and visualized under surgical operating microscope (Labomed Dental Microscope, Prima DNT, USA). A catch was identified in the center of the tooth and Size 8 K file (Mani, Tochigi, Japan) was introduced into the root canal. Copious amount of 17% EDTA (Glyde, Dentsply-Maillefer, Ballaigues, Switzerland) was introduced into the pulp chamber and size 8C + file (Dentsply, Tulsa, OK, USA) was used in watch-winding motion with minimal vertical pressure to reach till the apex. C + files have stronger buckling resistance compared with K files and provide easy negotiation and access to the apical third of the root. During instrumentation, frequent inspection of the file was done to identify any sign of fatigue, irregularity of the flutes or any other defects. Once size 8C + file became loose, size 10 k file (Mani, Tochigi, Japan) was introduced to gain apical patency and working length was measured using an electronic apex locator (Root ZX II, J Morita, Kyoto, Japan) and confirmed radiographically []. The root canal was prepared by the crown down approach using ProTaper Next rotary files to size X2 (Dentsply Maillefer, Ballaigues, Switzerland) with copious amount of 5.25% sodium hypochlorite (Chloraxid 5.25%, Cerkamed, Poland) as irrigant during instrumentation. Obturation was completed using ProTaper Next X2 gutta-percha (Dentsply Maillefer, Ballaigues, Switzerland) and AH Plus (Dentsply Maillefer Ballaigues, Switzerland) as endodontic sealer [].\nIn the next appointment, isolation of 11 was done with rubber dam and access cavity was prepared and the working length was checked on the radiograph []. There was profuse bleeding from the canal in 11 at this stage, so the surgical exposure and enucleation of the granulation tissue was initiated. After infiltration of local anesthesia buccally and palatally to the surgical site using Articaine (Septanest 1:100,000), a rectangular full-thickness flap was raised buccally. This revealed that part of the buccal bone plate was missing and exposed a small amount of inflammatory granulation tissue and the resorptive root surface [ and ]. Curettage of the resorptive lesion and the surrounding inflamed tissue both on the mesial aspect and apical third was performed together with curette and surgical round bur [].\nDue to the palatal extension of the resorptive defect, after giving sulcular incision, palatal flap was reflected, and complete curettage of the granulation tissue was done till a sound dentin base was revealed []. A small cotton pellet was then dipped in 90% trichloroacetic acid, and the excess was removed by dabbing it on a piece of gauze. The cotton pellet was then applied over the resorptive defect area with gentle pressure for about 1 min [].\nThe irregular borders of the defect were smoothed with a small round bur. Biodentine (Septodont, St. Maurdes Fossés, France) was mixed according to the manufacturer's instructions and was firmly condensed in the resorptive defect and root canal. Biodentine was contoured laterally and apically as per the external root anatomy [ and ]. Biodentine was left for 15 min to achieve the initial setting and relative hardness. After the setting of Biodentine was confirmed, the tissue flap was then repositioned and sutured with 3-0 black silk suture material, and the patient was instructed to report after a week for suture removal. Postoperative IOPA radiograph was taken [].\nAt 12 months follow-up, the patient was completely asymptomatic on presentation. Clinical and radiographic examinations for treated teeth [] revealed repair of resorption defect was successfully treated with Biodentine. The gingival attachment was still healthy (maximum probing depth of 3 mm), and tooth mobility was normal; the patient remained complaint-free. Further follow-up examinations were planned along with esthetic treatment.
A 6-year-old, female neutered Rhodesian Ridgeback was presented with a 1.5 year history of right forelimb lameness. No previous examinations had been performed. The dog showed a markedly shortened stance phase in its gait at a walk and trot. Significant muscle atrophy of the right shoulder muscles was present. Orthopaedic examination of the right front leg elicited repeatedly a strong pain response on flexion of the shoulder joint with simultaneously digital pressure medial to the greater tubercle in the region of the biceps brachii tendon, while flexing the shoulder the elbow was held in a ‘neutral standing angle’. No joint distention of the right elbow was present and no pain response was found during manipulation of the joint and palpation of radius and ulna. Neurological examination revealed no abnormalities.\nRadiographs of the shoulders revealed no abnormalities. The extended lateral view of the right elbow showed an abnormally shaped MCP: instead of the normal concave form, a more steep to convex delineation was present. The MCP was blurred and there was a radiolucent area at the level of the radial head surrounded by a heterogeneous zone. The ulnar notch showed sclerosis (Fig. ).\nThe dog was referred for diagnostic imaging to the Ghent University Veterinary Faculty, where additional radiographic projections of both elbow joints were made. The previous findings were confirmed and, based on the two radiographic examinations, the presumptive diagnosis of FCP was made.\nIn addition, CT examination of both elbows was performed. This revealed abnormally steep delineation of the MCP. The sub-trochlear notch showed sclerosis, and a small fissure was visible at the level of the MCP (Fig. a). On the more distal transverse CT images (Fig. b), demineralization of the MCP associated with several opacities was distinct. These findings were also visible on the sagittal and dorsal reformatted images (Fig. c, d).\nClinical and imaging findings suggested the presence of MCP disease, although some atypical findings were present: extensive demineralization associated with several areas with marked new bone formation. Due to these atypical findings, it was decided to conduct a histopathological examination in addition to surgical treatment of the coronoid lesions.\nFor surgery, a standard medial approach to the elbow joint was used []. Macroscopically, only a bone defect at the height of the MCP was noted. The cranial delineation of the MCP was no longer visible. The size of the defect was not measured, but a subtotal coronoidectomy was performed, extending distally 15 mm and caudally 6 mm. The osteotomy surface showed no visible abnormalities. The recovery was uneventful. Meloxicam (MetacamR, Boehringer Ingelheim) [0.1 mg/kg once a day per os (po)] was given post-operatively. At 2 weeks post-operatively the lameness substantially decreased. After explanation of the results of pathology at 6 weeks post-operatively the owner declined any follow up examinations and chose to continue the meloxicam as long as this medication could alleviate the pain sufficiently.\nFour specimens of the excised bone (15 × 6 mm) were sent for histopathological evaluation. The samples were fixed in 10 % neutral buffered formalin and transferred to a formic acid solution for decalcification. After 3 days of decalcification the tissue samples were routinely processed, paraffin embedded, sectioned at 5 μm and stained with hematoxylin and eosin (HE) for histologic evaluation by a board certified veterinary pathologist. Histopathological evaluation of the tissue samples of the proximal ulna at height of the MCP revealed a non-encapsulated, moderately demarcated, invasive mesenchymal neoplasm, extending through the cortex and through the bony trabeculae into the medulla. The neoplastic cells showed moderate anisocytosis and anisokaryosis, they were spindle-shaped with a small amount of eosinophilic cytoplasm, with distinct cell borders and the cells were arranged in streams. There was one mitotic figure in ten high-power fields (400× magnification). The nuclei were ovoid with a stippled chromatin pattern and contained a single, large, centrally located nucleolus. Multinucleated giant cells were scarce. The neoplastic intercellular matrix was composed of small irregular deposits of hyaline eosinophilic material consistent with osteoid (Fig. ) that multifocally revealed a moderate amount of mineralization. Also larger areas with cartilaginous matrix were present within the neoplasm.\nBased on previously published criteria [], the neoplasm was diagnosed as a chondroblastic OS. Based on grading criteria defined by Kirpensteijn et al. [], the dog was classified with low-grade II OS. The edges of the histopathological samples were not free of tumour cells.\nSeveral options—including amputation of the right forelimb, chemotherapy or radiation—were suggested to the owner, but were refused. The dog remained slightly lame in the right front leg during 9 months postoperatively despite daily administration of meloxicam. Obviously right forelimb lameness returned after 10 months. As the OS had not been completely excised based on histopathology margins, local recurrence of the tumour was suspected. On the medio-lateral and cranio-caudal radiographs of the right elbow, fulminant new bone formation around the area of the MCP was visible (Fig. ). This radiographic appearance differed from the expected post-operative images seen after subtotal coronoidectomy, namely a stump coronoid process with little or no bone reaction. The radiographic changes in this case were suggestive of a recurrence of the OS. Biopsy and further therapeutic options were refused by the owner. The dog was treated with daily meloxicam (MetacamR, 0.1 mg/kg once a day po) to relieve the clinical signs.\nTwo and a half years after tumour resection, the dog was presented with severe dyspnea and the right elbow was swollen and painful. Thoracic radiographs showed several large lesions in the lungs suggestive of metastases. The owner requested immediate euthanasia and declined any post-mortem examination.

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