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A 61-year-old Chinese lady presented with a 6-month history of a localised abdominal swelling and distention one and a half years post-midline laparotomy for a bleeding peptic ulcer. Her co-morbidities include a mild hiatus hernia and ischaemic heart disease for which she was on a low-molecular-weight heparin and a platelet inhibitor. She is American Society of Anaesthesiologists (ASA) Grade II and is not on regular non-steroidal, anti-inflammatory medications. On examination, an incisional hernia was found at the previous laparotomy site. It had a smooth surface and a positive cough impulse.\nThe patient underwent a laparoscopic intraperitoneal placement onlay mesh repair for her hernia. An Optic view port (12 mm) was placed at the left subcostal region as primary port entry with two 5 mm ports at the left flank. Dense abdominal adhesions were found with small bowel adherent to the anterior abdominal wall within the hernia sac. These were localised to the hernia site.\nA laparoscopic adhesiolysis was done dissecting the adherent small bowel off the anterior abdominal wall. The hernia defect measured 16 cm by 20 cm. An 20.32 cm by 25.4 cm sized polypropylene mesh coated with omega 3 fatty acids (fish oil) was placed tension-free with no primary suturing and fixed with two rows of ProTack™ 5 mm titanium helical fasteners to restore the integrity of the wall. The hernia sac was not excised. A standard double crown technique with stay sutures was employed. There were no iatrogenic injuries during adhesiolysis. The patient recovered and the immediate post-operative period was uneventful.\nDuring a routine follow-up visit 3 months after the procedure, the patient complained of mild dyspepsia, intermittent abdominal pain. There was no discharge from the incision site. On examination, there was slight tenderness over the incisional hernia site but no obvious recurrence. The patient was reassured and managed conservatively.\nThe patient’s dyspepsia and abdominal pain got progressively worse and a hard tender mass located in the epigastrium was palpated 10 months post-procedure. Computed tomography imaging of the abdomen showed thickening of the body and pylorus of the stomach and a thin soft tissue plane separating the stomach lesion and the pancreas (Fig. , ).\nGastroscopy revealed a thickened gastric antrum. There was no gross evidence of acute recurrence of her ulcer disease. Histopathological examination of biopsies taken from the gastric antrum showed chronic inflammatory granulation and fibrosis with no evidence of malignancy.\nThe patient subsequently underwent a laparoscopy. Dense intrabdominal adhesions were found intra-operatively. There was an adhesion of the small bowel to the fish oil mesh with fine inflammatory exudates. It was converted to an open procedure. Adhesiolysis, explantation of fish oil coated mesh and ProTack™, partial gastrectomy, gastrojejunostomy and jejuno-jejunostomy were performed. There was no evidence for a recurrence of the incisional hernia. There was no evidence of fistula or chronic sepsis. A sutured repair was undertaken. The condition of the patient improved a few days after the surgery.\nTwo samples were sent for histopathological analysis: the mesh sample and the partial gastrectomy. Macroscopic analysis of the mesh sample revealed the sample to be a torn piece of plastic mesh embedded in fibrous tissue secured by multiple metal coils. Microscopically, sections of the sample showed partly hyalinised fibrofatty tissue exhibiting an intense foreign-body granulomatous inflammatory reaction towards an amorphous translucent foreign material (Fig. , ).\nThe partial gastrectomy specimen had thick haemorrhagic fibrous adhesions. The mucosa showed loss of rugae and there appeared to be superficial ulcerations in the mucosa. Microscopically, the gastric specimens revealed submucosal oedema, inflammed granulation and foci of suppurative inflammatory necrosis with extension of the inflammation across the muscularis into the serosa.\nThe patient had an unremarkable post-operative recovery and has had no further sequelae in subsequent follow-up visits. |
A 64-year-old male patient underwent cardiac resynchronization therapy (CRT) device with defibrillator implantation for dilated cardiomyopathy and recurrent ventricular tachycardia. During the implantation procedure, it was difficult to determine the location of the left axillary vein as although the left subclavian vein was punctured and a guide wire could be inserted, we could not insert a peel-away introducer across the costoclavicular junction and handle the inserted right ventricular lead due to mechanical resistance at the costoclavicular junction. To avoid the risk of difficult lead handling and future subclavian crush syndrome, a second puncture was performed at a more lateral site approximately two fingers away from the initial puncture point. Although a puncture needle should be inserted almost vertically in order to reach the lateral axillary vein near the junction of the cephalic and brachial veins, we were able to insert peel-away introducers and the left ventricle (LV) and right atrium (RA) leads without any difficulty. The LV and RA leads were positioned where the optimal values of sensing and pacing parameters were obtained. After starting biventricular pacing, the episodes of ventricular tachycardia decreased remarkably, suggesting successful electrical remodeling. The patient was discharged without overt complications.\nHowever, two weeks after the implantation of the CRT device, the patient started complaining of "electric shock-like" pain in the left axillary area radiating to the medial border of the left arm. The patient described the pain as being usually triggered by active shoulder movements, especially when pulling up his pants. During physical examination, typical pain in the left axillary area was reproduced whenever his left shoulder was passively abducted more than 60 degrees. However, there were no objective sensory changes or motor weakness. The patient's symptoms and signs suggested irritation of the left brachial plexus. Chest computed tomography scans showed the LV and RA leads running together into the lateral axillary vein along the lateral side of the pectoralis minor muscle causing a curvature with an acute angle (). Fluoroscopic examination in the supine position showed that the LV and RA leads were positioned at an acute angle directing towards the left brachial plexus whenever the patient's shoulder was passively abducted more than 60 degrees (; ). Severe left axillary and radiating arm pain recurred whenever the LV and RA leads formed such an acute angulation on fluoroscopic examination. Brachial plexus irritation by the angulated CRT device leads was strongly suspected. Analgesics and antibiotics were prescribed to control the pain and to treat the possible subclinical device-related infection. However, the patient complained of gradual worsening of the pain despite continued administration of high-dose pain killers. Six months after implantation of the CRT device, the patient was readmitted for adjustment of lead angulation due to worsening of the left axillary and radiating arm pain which impeded the patient's daily physical activities.\nDue to the fact that the LV and RA leads were inserted across the pectoralis major and minor muscles into the lateral axillary vein, which was located deep in the patient's chest, correction of the lead angulation by generator repositioning was technically impossible. Operators had to move the entry site of the LV and RA leads from the distal to the proximal axillary vein using the cut-down method. Under general anesthesia, the pectoralis minor muscle was cut and the axillary vein was exposed. The vein was clipped and incised transversely at a proximal site (). After disconnection of the LV and RA leads from the generator, we attempted to manually extract the disconnected leads through the transverse incision line. However, the leads were tightly adhered to the axillary venous wall, and the possibility of vascular injury and LV lead malpositioning due to extraction force was considered. Finally, we had to open the left axillary vein to separate the leads safely. The left axillary vein was incised longitudinally from the initial insertion site of the LV and RA leads to the more proximal site which was 3 cm away ( and ). The adhesions between the leads and vessel wall were then dissected carefully. Separated leads were moved to the proximal site through the longitudinal incision line and the remaining incision lines were closed. After adjusting the LV and RA lead insertion site, acute angulation was not observed during passive shoulder abduction more than 60 degrees on fluoroscopic examination (). After confirming successful lead repositioning, all of the procedures were completed without complications. Although curvatures of the LV and RA leads were slightly modified after the correction surgery, there was no significant change in the LV lead tip location and LV capture threshold. Neuropathic pain decreased remarkably two weeks later and analgesic agents could be withheld two months later. There were no symptoms and signs of left subclavian vein obstruction. The ranges of passive and active shoulder movements increased gradually but were nearly completely normalized at six months after the surgery. |
Mr. S is a 27-year-old male from middle socio-economic status. The patient was referred to psychiatry outpatient department from the plastic surgery department for clearance for rhinoplasty surgery. Patient was interviewed in detail and his history dated back to 17 years of age when he was in his final year of schooling. He started noticing pimples and acne on his face, which embarrassed him to face his peers. He observed that few of the acne cleared, but few on the face left black marks and those on the nose turned into comedones. Most of them cleared with the treatment from a dermatologist. But the patient's concern with the healed scars on the face and nose started increasing and he started becoming preoccupied with them. He felt embarrassed to face his classmates and would feel shy to face the public or relatives visiting his house.\nThe patient adopted measures like covering his nose with his hands while speaking to others or while listening to the lectures in the class so as to hide the marks. Over a period of time, he started developing rituals such as repeatedly watching his nose in the mirror, frequently washing his face after he returns back home from outside, applying powder over the nose to cover up the imagined area of scars on the nose, and trying to reshape his nose on his own using a stone, which resulted in bleeding and worsening of the condition.\nThe patient developed anxiety in social situations because of his referential thinking involving his imagined ugly appearance. He also developed misinterpretations of other people's behavior and comments linking them to his facial disfigurement. He spent enormous amount of time and money in reshaping his nose, at times resorting to stealing money from home. He developed depressed mood, death wishes, and suicidal thoughts as a result of lack of improvement with treatment from various doctors. Patient had frequent change of jobs giving the reason as his inability to cope up with works involving social contact as a result of his ugly nose. He also started attributing his failures in academics and professional life to the imagined deformity in the nose. The patient did have recurrent suicidal ideation secondary to preoccupation with imagined deformity and occupational dysfunction, but there was no active attempt.\nThe patient did have history of stammering since childhood, which exacerbated after his social anxiety increased. He has a history of alcohol and tobacco abuse. He is the first among his five siblings. His mother used to be very critical of his appearance from his childhood. There is family history of stammering, but there was no other significant mental illness in the family. He had difficult temperament from his childhood, although adequate information could not be got.\nAt the time of presentation to us, the patient had non pervasive sad mood, decreased concentration in his work, significant social anxiety, and avoidance of social situations due to referential thinking, stammering, strong beliefs that his nose is ugly and deformed (amounting to delusional level) with absent insight, and he attributed all his failures to the physical problem. There was significant impairment in social, occupational, and academic functioning. There were obsessions related to contamination and compulsive washing, checking, and obsessive images, blasphemous thoughts, and sexual obsessions.\nThe patient was diagnosed to have BDD-delusional variant with obsessive compulsive disorder mixed type, social anxiety disorder, dysthymia with mild depressive episode without somatic complaints, and stammering, according to DSM-IV criteria. |
A 65-year-old man who was on maintenance HD for 27 years presented with gross hematuria for which subsequent imaging was highly suggestive of renal malignancy. He underwent left radical nephrectomy as it was a local disease and pathology confirmed RCC. Two years after the nephrectomy, he was unfortunately diagnosed with metastatic RCC. His past history was significant for Child-Pugh class B hepatitis C (HCV) related liver cirrhosis (albumin 28–35 g/L, Bilirubin <34 μmol/L, INR <1.7, presence of small amount of ascites, one past episode of grade 2 encephalopathy). The only episode of hepatic encephalopathy occurred 15 months before the diagnosis of metastatic RCC and was managed successfully with lactulose; he was subsequently started on rifaximin and had remained well since with no recurrence of encephalopathy. After deliberation of the various approaches for treatment, his oncologist considered TKI to be the best treatment option and started him on sunitinib 25 mg daily.\nOur patient was brought to the emergency department (ED) with confusion and bilateral asterixis 44 days after initiation of sunitinib (patient was maintained on 25 mg daily throughout this period). After appropriate investigations including computed tomography (CT) of the brain had ruled out other causes, he was managed as for metabolic encephalopathy (grade 2). His serum ammonia level was 170.5 μg/dL at presentation. His symptoms resolved 4 days later (with discontinuation of sunitinib). His serum ammonia was reduced to 48.7 μg/dL. He received the same dialysis schedule during his hospitalization and was discharged to continue with his usual HD regimen (3×/wk and 3.5 h/session).\nHe was later seen by his oncologist and was started at a lower dose of 12.5 mg sunitinib daily 2 weeks after his discharge, increasing to 25 mg daily 4 weeks after being stable on the lower dose with no complaints. He presented to ED again with similar presentation of confusion and asterixis 28 days later after the increase in dose. His serum ammonia level was significantly elevated again at 176.1 μg/dL pre-dialysis, declining to 68.2 μg/dL post-dialysis. He was dialyzed with a larger surface area high-flux filter (polysulfone 1.6 m2) using blood flow rate of 280 mL/min and dialysate flow rate of 500 mL/min for 4 hours. His body weight was 54.5 kg. His confusion improved significantly post dialysis and he recovered the following day. He was maintained on sunitinib 25 mg daily and discharged well with a schedule for more intensive dialysis (higher frequency 4×/wk and with better clearance achieved by increased blood flow, longer duration, and larger filter). He was successfully kept out of hospital with no recurrence of encephalopathy with that dialysis schedule. There were no adverse events and episodes of confusion.\nExcept for the elevation in serum ammonia level, there were no changes in coagulation profile, bilirubin, or liver enzymes during each of these 2 admissions for encephalopathy. There were also no other precipitating factors for hepatic encephalopathy that was evident in these admissions. He did not experience constipation and was compliant with lactulose and rifaximin. CT brain done during his admission did not reveal structural brain pathology. He does not consume alcohol and did not take traditional Chinese medicine. He was not given any medication that can inhibit CYP3A4. He was also not taking any therapeutic agent that has reported association with hyperammonemia. Patient was jointly managed by a multi-disciplinary team (Liver, Medical Oncology and Nephrology). All management decisions were discussed and made jointly by consensus. |
The patient is a 7-month-old twin boy who presented to our institution's emergency department with increased work of breathing and desaturations (70 s). He was born at 33 weeks gestational age with Down syndrome, developed chronic lung disease (CLD) of prematurity, and was also found to have a moderate size secundum atrial septal defect (ASD) as a newborn. Prior to the current illness, he had been in the hospital multiple times for failure to thrive and respiratory distress, requiring mechanical ventilation with high amount of supplemental O2 and inhaled nitric oxide (iNO) as he developed pulmonary hypertension (PH). Echocardiography showed progressive enlargement and hypertrophy of his right ventricle and at times bidirectional shunting across his ASD. A diagnostic cardiac catheterization as a preoperative evaluation was performed, which showed elevated pulmonary vascular resistance indexed (PVRi) at baseline (8.8 WU·m2), which decreased with inhaled oxygen alone and iNO (3.8 WU·m2). Additional catheterization data at baseline condition showed a right atrial mean pressure of 6 mmHg, right ventricular end diastolic pressure of 6 mmHg, and pulmonary artery pressure 51/19 mmHg with mean 32 mmHg. The patient was started on home O2 therapy with nasal cannula. The current hospitalization occurred prior to a planned fenestrated patch repair of his ASD.\nHe was initially admitted to the general ward and soon transferred to the pediatric ICU for severe hypoxemic respiratory failure requiring mechanical ventilation. Respiratory syncytial virus (RSV) infection was diagnosed with the positive antigen test. He continued to have paroxysmal severe hypoxic events compatible with PH crisis. He was treated with sedation and neuromuscular paralysis, increased FiO2, optimization of O2 carrying capacity with packed red blood cells transfusions, and iNO. Milrinone infusion was added as the right ventricular function was depressed on echocardiogram (TAPSE 6 mm, Z-score −4), which demonstrated evidence of systemic to suprasystemic right ventricular pressure and bidirectional shunting across the ASD (Figures and ). No other cardiovascular intravenous drips were given during the ICU stay. Sildenafil was initiated enterally and escalated to maximal dose (2 mg/kg/day) without hemodynamic compromise. He was on diuretic therapy (bumetanide infusion up to 10 mcg/kg/hr) as chest X-ray demonstrated evidence of bilateral interstitial edema with bilateral pleural effusions on admission () and confirmed by chest ultrasound. Bilateral chest tubes were placed after failure of diuretic therapy to reduce effusions on hospital day #6. The drained fluid was milky in appearance bilaterally, with a white blood cell of 1,004/mm3 with lymphocyte predominance (88%) and elevated triglycerides (1008 mg/dl), and hence a diagnosis of chylothorax was made. Low IgG level (249 mg/dl) and hypoalbuminemia (2.5 g/dl) were noted at the time of pleural effusion drainage. Intravenous immunoglobulin and 25% albumin solution were administered. His feeding formula was changed to medium-chain triglyceride formula. The milky drainage became serous; however, the volume of chest tube drainage remained unchanged. Enteral feeding was discontinued and total parenteral nutrition was initiated, which decreased the volume of pleural effluent but small to moderate amount of pleural effusion was intermittently observed by chest X-ray for over sixty three days until the patient's death. Venous Doppler ultrasound of the upper extremities and the neck was performed on hospital day #7 and 4 weeks later, and compression, thrombosis or obstruction of the superior vena cava, and upper extremity were ruled out. A central venous catheter was placed in the right jugular vein soon after admission and was removed on hospital day #7 and replaced by a peripherally inserted central line. The patient required chest tubes for drainage until hospital day #22. Since then, intermittently small to moderate pleural effusion was observed by chest X-ray, but chest tubes were not placed.\nHe continued to be critically ill with persistent hypoxemic respiratory failure without improvement in PH with several PH crisis episodes. Therapy with an endothelin (ET) receptor antagonist (Bosentan) was added. The hospital course was complicated by bacterial tracheitis from Pseudomonas and E. coli. The patient remained on mechanical ventilator support for 6 weeks due to failed weaning of ventilator support from hypoxemia despite high levels of supplementary FiO2 and iNO. Cardiac catheterization performed 6 weeks after admission showed PVRi of 7 WU m2 on 100% FiO2 and 20 ppm of iNO under general anesthesia, pulmonary venous desaturation, and bidirectional shunting through ASD. Additionally, interval increases in right atrial pressure (mean 13 mmHg), right ventricular end diastolic pressure (12 mmHg), and pulmonary artery pressure (52/24 mean 36 mmHg) were noted. Given his severe and irreversible lung injury from mechanical ventilation in addition to baseline chronic lung disease, he was deemed not a candidate for lung transplant. Considering that the patient had Eisenmenger physiology due to severe PH and poor prognosis, the palliative care team was also consulted. Weaning from the mechanical ventilator was tried multiple times, but failed. At 9 weeks of his ICU hospitalization, he developed severe hypoxemia unresponsive to medical therapy that ultimately caused his death.\nAn autopsy showed bilateral small straw-colored pleural effusions (right 17 ml and left 10 ml), and the lung parenchyma was red-brown, poorly aerated, and diffusely congested with focal consolidation. The heart had an ASD (0.8 × 1.2 cm) with right ventricular hypertrophy secondary to PH. Microscopically, both lungs showed subpleural cysts lined by pneumocytes and containing macrophages, sloughed pneumocytes, and neutrophils. Acute multifocal bronchopneumonia was present with neutrophils in the bronchioles and alveoli. Chronic interstitial lung disease is diffusely present with alveolar septal thickening, capillary disorganization, and hemosiderosis. Small pulmonary arterial branches demonstrate moderate to marked medial smooth muscle hypertrophy with lumen narrowing, while large pulmonary arteries were normal with minimal changes. No lymphatic dilatation was observed on H&E or D2-40 immunostained slides; therefore, lymphangiectasia was ruled out (). From the autopsy results, hypoxia due to progressive PH was considered as a cause of death. |
A 42-year-old male visited the emergency department complaining of chest pain. He had previously been admitted 3 months ago due to similar symptoms, and was diagnosed with acute pericarditis according to the features of typical chest pain and findings of electrocardiography (ECG) and echocardiography (). Ibuprofen and colchicine were administered and the symptoms improved within 24 hours. At the time, he was discharged after 5 days, and he had stopped taking the medications by himself after 2 weeks as he did not feel any continuing symptoms.\nAt admission, he complained of severe chest pain which had begun 4 hours prior to admission. The pain increased with deep breathing and position change. He also complained of palpitation which had been present prior to the development of chest pain. The patient had no relevant abnormal medical history and no current medication, and reported to have lost 2 kg during the past 2 months. He was a 14-pack-year smoker, but denied any alcohol use.\nUpon general examination, he appeared acutely ill. His vital signs in the emergency department were as follows: body temperature 36.9℃, pulse rate of 121 beats per minute, and blood pressure 140/89 mm Hg. The heartbeat was regular and no cardiac murmur including precordial friction rub could be identified. In addition, the lung sound was clear. There was no thyroid enlargement or neck vein engorgement.\nLaboratory results including complete blood count, biochemistry profile, and cardiac enzymes were within normal limit. The chest radiograph finding was normal. ECG showed sinus tachycardia and diffuse concave ST-segment elevation in all leads except aVR and V1. PR segment was normal (). Transthoracic echocardiography showed normal systolic and diastolic function of the left ventricle (LV) and a scanty amount of pericardial effusion at the LV posterior side (). Echocardiography that had been performed at the previous admission had shown constrictive physiology, but no such finding was visible this time. Cardiac magnetic resonance imaging showed a slightly thickened pericardium with small amounts of pericardial effusion (). Autoimmune antibodies (ANA and Anti-ds-DNA) showed normal results. According to the characteristics of the chest pain, which was sharp and pleuritic in nature, and the diffuse concave ST elevation without relevance to any certain coronary artery which was out of proportion to the pain intensity, the patient was diagnosed as acute pericarditis rather than coronary spasm-related angina.\nThe patient was given ibuprofen and colchicine. Due to a history of palpitation and weight loss, tests for thyroid function were done on hospital day 3. Serum thyroid hormone examination showed evidence of hyperthyroidism with a T4 level of 14.2 µg/mL, T3 level of 182 ng/dL, and TSH level of 0.06 µIU/mL (normal value T4 4.7-12.5 µg/mL, T3 76-190 ng/dL, TSH 0.3-6 µIU/mL). Thyroid autoantibody evaluation and a thyroid scan were performed. TSH receptor antibody was 5.4 IU/L (normal 0-1 IU/L), thyroglobulin antibody 89 U/mL (normal 0-60 U/mL), and microsomal antibody 136 U/mL (normal 0-60 U/mL) all showed elevated levels. Thyroid scan showed 17% (normal 5 to 15%) uptake and the patient was diagnosed with Graves' disease, after which methimazole 10 mg bid was prescribed. Chest pain subsided within 24 hours, and the patient showed marked clinical improvement within 7 days with improvement of palpitation and general weakness. Elevated ST-segment was improved. The patient was discharged with medications for anti-hyperthyroidism and colchicine. No clinical evidence of recurrence was found during the following 3 months. The ST-segment eventually normalized on the follow up electrocardiogram (). |
A 52-year-old woman presented at a local hospital’s emergency department after being discovered by her family with sudden-onset vomiting and loss of consciousness. Head CT showed bilateral basal ganglia hemorrhages (data not obtained). The patient was immediately transferred to our hospital. Vital signs on arrival were unremarkable except for markedly elevated blood pressure (180/100 mm Hg). The patient had a 2-year history of hypertension without any history of diabetes, trauma, surgery, or taking oral anticoagulant drugs. Her Glasgow Coma Scale (GCS) score was 6 (E1V1M4). Neurologic examinations revealed coma, quadriplegia, and bilateral positive Babinski sign. Bilateral pupils were 3 mm but sluggish to light. Head CT showed bilateral basal ganglia hemorrhages of about 18 mL on the right and 27 mL on the left side (). Hematoma volume was calculated using the ABC/2 method. Chest CT showed bilateral mild pleural effusion and infection in bilateral lungs. Laboratory tests were within normal ranges including complete blood cell counts, bleeding time, activated partial thromboplastin time, prothrombin time, liver and renal function, and blood glucose level. We decided to remove the hematomas by aspiration to reduce intracranial pressure (ICP). We pasted CT markers on the frontal and temporal areas of the patient’s head for head CT to indicate the puncture points for aspiration (). The patient later underwent MIS for ICH under general anesthesia. The neurosurgeon performed hematoma catheter injections using a standard sterile technique. The catheter diameter was 4.8 mm. Frontal and temporal catheters were placed, and 10 and 5 mL of blood clots were aspirated from the left and right sides, respectively. Immediate postoperative head CT confirmed the catheter track and residual hematomas ( and ). The patient was sedated and transferred with an endotracheal tube and mechanical ventilation to the Neurosurgical Intensive Care Unit.\nUrokinase injection and clot aspiration were performed for the residual hematomas. On postoperative day 1, the 2 catheters were clamped after infusion of urokinase (20,000 U dissolved in 3 mL normal saline) for 2 h. However, at 1.5 h, the patient exhibited the Cushing reflex and bilateral pupils were 5 mm and unresponsive to light. We opened the catheter valve, which alleviated the patient’s signs and symptoms. GCS score improved, and the patient started responding to commands starting from postoperative day 2. We infused urokinase every 12 h up to 4 doses instead of performing a single injection, and the patient was stable during the process. On postoperative day 3, head CT showed a residual clot (10 mL on the right and 2 mL on the left side) and the patient was extubated ( and ). Human albumin and furosemide were administered to control ICP and cerebral edema. The patient’s pneumonia continued to worsen after the operation, requiring assisted ventilation. She underwent tracheotomy by general anesthesia on postoperative day 10. On postoperative day 19, the ventilator was removed and head CT revealed absorption of the intracranial hematoma ( and ). Over the 2 years of follow-up, there was no recurrence of ICH. The non-fluent aphasia improved but speech did not return to normal. The patient suffered mild disorder of the left and right limb muscles, but she was able to walk unassisted. The patient’s modified Rankin Scale score was 3 on postoperative day 90 and 2 at the 2-year follow-up. This case was approved by the medical ethics committee of the First Affiliated Hospital of China Medical University to publish the case details. Written, informed consent was obtained from the patient for publication of case details and accompanying images. |
A 73-year-old woman with a history of well-controlled type 2 diabetes mellitus and hypertension presented to the clinic with visual loss in both eyes in July 2008. The vision was 20 / 200 in both eyes. There were no other abnormalities in the anterior segments of the eyes, other than visually significant cataracts, bilaterally. The fundus examination with the indirect ophthalmoscope revealed the presence of a moderate nonproliferative diabetic retinopathy and macular edema in both eyes. Optical coherence tomography showed macular thickening in both eyes with a taut posterior hyaloid membrane of the macula in the right eye. In July of 2008, phacoemulsification and intraocular lens implantation were performed, as well as vitrectomy with removal of the posterior hyaloid and internal limiting membranes, as well as endolaser treatment of the right eye. At the end of the vitrectomy, IVTA (4 mg) was administered. One week later, phacoemulsification, intraocular lens implantation, and IVTA administration (4 mg) were performed on the left eye. The postoperative clinical course was fair. In August of 2008, the vision improved slightly to 20 / 100 with improvement of the macular edema in both eyes.\nThree-months later, in October of 2008, the patient presented with 'cloudy vision' in both eyes that developed two weeks previously. The visual acuity was 20 / 200 in the right eye and counting fingers at 30 cm in the left eye. The intraocular pressure (IOP) was 30 mmHg in the right eye and 42 mmHg in the left eye. The slit lamp examination demonstrated fine keratic precipitates of the corneal endothelium, 3+ inflammatory cells, 2+ flare in the anterior chamber, and moderate vitreous haze in both eyes. The fundus examination revealed white-yellow, necrotic peripheral retinal lesions in the superotemporal quadrant of both eyes ().\nAs bilateral acute retinal necrosis was suspected, starting intravenous treatment with acyclovir was considered. However, the patient refused hospital admission and further treatment. Moreover, azotemia resulting from diabetic nephropathy limited the use of acyclovir. Therefore, antiviral treatment was not started and the patient was closely monitored. A diagnostic aqueous humor sample for polymerase chain reaction (PCR) analysis was obtained. PCR was carried out to test for varicella zoster virus, herpes simplex virus type 1 and 2, and CMV. Topical anti-glaucomatous and steroid medications were used for treatment.\nThe PCR results, available seven days later, confirmed the presence of CMV. The infectious disease consultant reported that systemic CMV infection was not possible and recommended antiviral therapy with ganciclovir for the CMV retinitis. However, one week had elapsed and the IOP and inflammatory reaction of the anterior chamber and vitreous had decreased, the peripheral retinal lesion did not increase, and the macula was not threatened in either eye ().\nThe CMV retinitis was likely caused by the local (intraocular) immunosuppression caused by the IVTA and the immunosuppression might have reduced over time (already 3 months since the IVTA was administered in both eyes). The CMV retinitis did not threaten the macula. Since starting treatment with ganciclovir was not urgent, the patient was closely followed without any anti-CMV medications. The plan was close observation while maintaining the topical anti-glaucomatous and steroid therapy.\nIn November of 2008, two months after the initial presentation, the inflammatory reaction of the anterior chamber appeared to be resolved and the vitreous haze was much improved in both eyes. The IOP was 24 mmHg in the right eye and 28 mmHg in the left eye with topical application of timolol and dorzolamide. The necrotic retinal lesion regressed slightly without any progression of the margin of the lesion in both eyes.\nIn April of 2009, six months after initial presentation, the visual acuity was 40 / 200 in the right eye and 20 / 200 in the left eye. The anterior chamber reactions had resolved and the inflammatory retinal lesions regressed completely with the subsequent development of retinal pigmentary changes in both eyes. A mild vitreous haze was still present in the left eye (). |
A 19-year-old Japanese woman underwent root canal treatment on tooth #47 by a general practitioner in 2005, but dull pain persisted after. Tooth #48, which was horizontally embedded, was extracted in August 2010, but the pain continued. In August 2015, she presented at the Department of Oral Surgery of another hospital with a purulent discharge from the pocket of tooth #47. Intraoral radiography showed insufficient root canal filling in the distal root and a foreign body, suspected to be extruded gutta-percha, outside the apex of the mesial root of tooth #47, accompanied by bone absorption around both roots (Fig. ). Two months later, she underwent extraction of tooth #47, removal of the foreign body, and curettage of the periapical lesion, resulting in the disappearance of the pain. However, she noted expansion of the right posterior mandibular bone in March 2016 and was thus referred in May 2016 to the Department of Oral and Maxillofacial Surgery, Tokyo Medical and Dental University, Japan. Her health and nutritional status were good in spite of a low body mass index (17.0). Both a blood test and a chest X-ray showed normal findings. She had neither lymphadenopathy nor paralysis of the mental/lingual nerve. The right inferior border of the mandibular bone slightly bulged. The socket of tooth #47 was epithelialized, and tooth #46 was vital, with a pocket depth of 3 mm. There was no sinus tract. Panoramic radiography revealed new bone formation at the equivalent sites of teeth #48 and #47 and a radiolucent region around the distal root of tooth #46 (Fig. ). Computed tomography showed continuous absorption from the alveolar bone of the distal root of tooth #46 to the lingual cortical bone at the equivalent of tooth #47 and six granulated hard tissues (Fig. , ). On magnetic resonance imaging, the right lower molar region was of low intensity on an enhanced T1-weighted image and high intensity on an enhanced T2-weighted image (Fig. ). This same region was enhanced heterogeneously by gadolinium, as was the soft tissue located between interior to the mandibular angle and anterior to the submandibular gland (Fig. ). She was diagnosed clinically as having osteomyelitis of the right mandible.\nSurgical debridement under general anesthesia was performed. Via the submandibular region, an incision was made in the thickened periosteum at the inferior border of the mandible, after which the lingual periosteum was separated from the mandible (Fig. ). Tooth #46 was then extracted to achieve complete primary closure, and the gingiva incised (Fig. ) to enucleate the soft tissue including some of the hard tissues accompanying the periapical lesion of tooth #46 (Fig. ). There was no sequestrum and the intraoral wound was closed primarily. Our patient’s postoperative course was uneventful, with no exacerbation of the inflammation for more than 20 months.\nOn histopathologic examination, a large epithelioid cell granuloma with central caseating necrosis was observed in the dense fibrous tissue (Fig. ). The granuloma contained Langhans multinucleated giant cells. Acid-fast bacilli were not detected by either Ziehl–Neelsen staining or immunohistochemical staining using anti-BCG and anti-TB1 antibodies. Grocott staining revealed a slight presence of fungi. The pathological diagnosis was epithelioid cell granuloma including caseating necrosis. |
A 19-year-old man without significant past medical history was brought to the Emergency Department having been found unconscious at home, after an explosion of unclear origin was noticed in the basement of the family home. Upon admission, the patient was fully conscious and stated, that he had been handling a self-made pipe bomb that exploded unexpectedly, severely injuring his left hand and “somehow hitting his right side of the face.” At the scene, he complained about a headache and the inability to see with his right eye, which was blood crusted. The patient also reported that he could not feel his left hand, which was severely injured. On presentation to the ER (trauma level 1 institution), he was stable and conversant with a GCS of 15 with minimal residual bleeding from his face and a profusely bruised and swollen right orbit []. His left hand was partially amputated []. The patient subsequently underwent primary and secondary surveys according to ATLS protocol and was sent for imaging studies. An ophthalmology consult was obtained in the ER, which revealed gross destruction of the right eye with globe rupture and no residual vision. His contralateral left globe was fully intact the only neurological abnormality was progressive drowsiness, which led to expeditious imaging work-up.\nInitially a noncontrast CT scan of the head was performed emergently and revealed an intracranial irregularly shaped metal fragment measuring approximately 5 × 1.5 × 0.2 cm as seen on the tomogram scout films []. The object was lodged in the back of the right orbit, reached through the superior orbital fissure into the middle cranial fossa and transversed the right temporal lobe. It was comparable to a near complete blow-out fracture of the right orbit with adjacent ethmoid- and sphenoid-fractures before the fragment penetrated posteriorly into the floor of the middle cranial fossa formed by the petrous portion of the right temporal bone and skull base []. For further analysis, a CT angiography was performed revealing the tip of the metal fragment had penetrated the petrous bone deeply reaching the right internal carotid canal [].\nWhile examinations were ongoing, the patient became progressively restless and drowsy. He was emergently intubated in the ER and taken to the OR for neurosurgical exploration in conjunction with an orthopedic trauma team who simultaneously took care of the patient’s left hand. The patient received intravenous antibiotics (vancomycin 1 g, flagyl 500 mg, gentamycin 80 mg) and a tetanus vaccination. For brain relaxation 100 g I/V Mannitol and 20 mg I/V lasix with mild hyperventilation (pCO2 = 30) was administered along with 10 mg of I/V Decadron for a possible temporal lobectomy. Two units of fresh frozen plasma were infused to prevent DIC and other blood products were readily available.\nSurgical exposure was accomplished via a right fronto-temporal craniotomy and simultaneous right neck exposure of the right common carotid artery, to allow for proximal control in case of significant intracranial hemorrhage. A right temporal lobectomy was carefully performed around the retained foreign body and the skull base was explored. The copper pipe fragment [] which was firmly lodged into the petrous bone and sphenoid wing was exposed. The part of the metal fragment in the petrous bone was safely loosened from the intracranial side. An occuloplastics consult was performed intraoperatively. As the eye was severely and irrepairably injured, a right-sided enucleation was performed. This procedure also facilitated further transorbital exploration and allowed to mobilize the metal fragment which was lodged in the sphenoid. After drilling down parts of the orbital apex and sphenoid wing around the metal fragment, which reached the floor of the middle cranial fossa, it was possible to mobilize and retrieve the entire foreign body in one single piece via the transorbital route[] []. Simultaneous packing of the petrous portion of the temporal bone was performed. As a precaution, we had exposed and slinged the ICA at the level of the CCA bifurcation to allow for possible ICA occlusion in the neck, should the packing be unsuccessful. During careful exploration, no laceration of the intracanalicular ICA was evident and the skull base defect was closed. The floor of the middle cranial fossa and the retro-orbital wall was closed with a titanium mesh (Synthes) and an onlay duroplasty allograft (Duragen; Integra). Due to the severity of the injury, we felt safer to leave the patient with a decompressive hemicraniectomy.\nWhen the neurosurgery team had completed the critical portion of this procedure, the left hand was explored, surgically debrided and initially reconstructed as a claw with Steinman pins. Meanwhile, a spherical implant was placed by occuloplastics to reconstruct the orbital soft tissue following the enucleation. The postoperative CT scan of the head demonstrated normal post-surgical findings without complications []. The patient was then transferred to the ICU in stable condition. He was extubated on hospital day two, transferred to the floor the same day, and fully mobilized on hospital day three. The patient did not show any focal neurological deficit other than loss of vision on the right side, mild ipsilateral periorbital numbness, and left hand impairment. Due to extensive orthopedic wound care requirements, he was discharged from the neurosurgical service on postoperative day nine. The patient was scheduled to follow-up with outpatient care for planning of his further elective interventions: cranioplasty and staged repair for his upper left extremity and right eye. Of note, the 19-year-old patient was able to attend high-school graduation only 14 days after this incident [].\nAn uneventful cranioplasty was performed eight weeks after the initial bomb explosion. Outpatient follow up scans have been unremarkable for three years without signs of delayed complications. Clinically the patient remains unchanged. Due to the severe and destructive hand injury, the initially reconstructed claw-fingers had to be amputated at the proximal metacarpal line several weeks later to create a stump with palmar tissue only.\nNo additional neurological deficits occurred and the patient enrolled to college the year after his accident and successfully graduated in 2009 []. The patient also was able to master physical labor as field apprentice in a construction firm. |
A 13-year-old girl with no prior medical history was admitted to the hospital after resuscitation from cardiac arrest. The patient had collapsed during physical exercise in school, and cardiopulmonary resuscitation (CPR) was initiated immediately by school personnel. Pulseless electrical activity was the first observed prehospital rhythm. The patient was intubated by the ambulance staff, and return of spontaneous circulation was achieved after 35 minutes through CPR and adrenaline administration.\nUpon arrival to our emergency department, the patient was hemodynamically unstable with a mean arterial pressure (MAP) of 60–70 mmHg and heart rate of 120 bpm. Arterial blood gases showed a severe combined metabolic and respiratory acidosis with a pH of 6.7, PaCO2 of 83 mmHg (11 kPa), and a blood lactate of 15 mmol/L on mechanical ventilation. Bedside echocardiography revealed a dilated right ventricle and a mass in the right ventricular outflow tract suggesting a venous thrombus. Computed tomography (CT) with pulmonary angiography showed multiple peripheral emboli in both lungs, and CT of the abdomen and pelvis revealed an aneurysm of the inferior vena cava as the likely source of the emboli. CT of the brain showed no evidence of cerebral edema or infarction (). However, based on the prolonged resuscitation and decreased level of consciousness, an ICP transducer was inserted to enable detection of cerebral edema during the impending deep sedation. Initial ICP readings were normal (4 mmHg).\nThe patient was transferred to the cardiothoracic intensive care unit (ICU) and a cerebral perfusion pressure >60 mmHg was maintained with infusion of adrenaline and noradrenaline. Targeted temperature management aiming at 36°C for 24 hours was initiated, and the patient was started on high-dose unfractionated heparin due to the pulmonary emboli. Failed attempts to cannulate both femoral arteries resulted in bilateral hematoma formation and continued bleeding from the right femoral artery despite compression necessitated surgical exploration with repair of the artery and fasciotomy.\nThe day after admission, the patient had been hemodynamically stabilized and weaned off vasopressors. Sedation was gradually diminished and finally turned off. The patient demonstrated eye opening upon stimulation, pupils that were equal and reactive to light, spontaneous breathing, and a normal swallowing reflex. No spontaneous movements were observed. ICP was slightly elevated at 10–17 mmHg depending on stimulation and closely related to MAP. Laboratory analyses revealed rising levels of creatinine, urea, and potassium, and diuresis was low despite stimulation with furosemide and metolazone. Myoglobin and creatine kinase were also significantly elevated, suggesting rhabdomyolysis due to hypoxia, compartment syndrome of the right thigh, or both.\nOn the evening of day 3, the patient had a P-creatinine of 5.17 mg/dL (457 μmol/L), P-urea of 99 mg/dL (35.4 mmol/L), and a P-potassium of 6.0 mmol/L despite infusion of glucose and insulin. The patient was visibly hypervolemic with an estimated cumulated fluid balance of +12.5 liters. ICP was stable but slightly elevated at 16–19 mmHg, and the patient was lightly sedated with remifentanil infusion. It was decided to initiate continuous renal replacement therapy (CRRT), and a double lumen dialysis catheter was placed in the right internal jugular vein. The following dialysis settings were used: Continuous venovenous hemodiafiltration, ST100 dialyzer (Gambro; surface area 1 m2, KUF 25 mL/(h·mmHg)), blood flow 120 mL/min, predilution flow 1000 mL/h (Prismocitrate, Gambro), dialysate flow 1000 mL/h (Prism0cal B22, Gambro), and postdilution flow 200 mL/h (Phoxillium, Gambro).\nApproximately seven hours after start of CRRT, ICP had increased to 38 mmHg (); the patient had developed diverging eye axes and become unresponsive to pain (Glasgow Coma Score 3). On suspicion of cerebral edema or infarction, CRRT was stopped, 50 mL of hypertonic saline (1 mmol/mL) was administered, and the patient was sedated with propofol. CT of the brain was performed and revealed diffuse cerebral edema (). ICP decreased to 20 mmHg within a few hours. Due to the neurological deterioration and unstable ICP, the patient was transferred to the neurological ICU (NICU) and sedated with thiopentone, midazolam, and fentanyl.\nOn day 4, an external ventricular drain (EVD) was inserted stereotaxically based on a predicted need for further hemodialysis, upon which ICP decreased from 10 to 3 mmHg. A window of cardiovascular stability and low ICP was used as an opportunity to start careful intermittent hemodialysis. Hemodialysis was chosen over peritoneal dialysis due to hyperkalemia and the need for removal of large volumes of fluid, as well as the possibility of separating ultrafiltration and dialysis. P-creatinine was 5.92 mg/dL (523 μmol/L) and P-urea 95 mg/dL (34.1 mmol/L) before start of dialysis. The patient completed 3.5 hours of isolated ultrafiltration, with ultrafiltration rate gradually increasing from 250 mL/h to 1000 mL/h and a total fluid removal of 2.5 L. Subsequently, the patient underwent 1 hour of hemodialysis without complications and with no clinically significant changes in ICP. Dialysis settings were modified to reduce the dialysis dose in an attempt to prevent DDS and were as follows: H6 dialyzer (Gambro; surface area 0.6 m2, KUF 33 mL/(h·mmHg)), dialysate sodium 148 mmol/L, potassium 2 mmol/L, bicarbonate 38 mmol/L, calcium 1.5 mmol/L, magnesium 0.5 mmol/L, and low dialysate flow at 300 mL/min and blood flow 150 mL/min with concurrent flows to reduce dialysis efficiency. On day 5, ultrafiltration and hemodialysis were repeated with similar settings. However, ICP gradually increased from 0 to 10 mmHg and the treatment was stopped prematurely. Similar problems were encountered on the following days, necessitating frequent pauses or early cessation of hemodialysis.\nOn day 8, an inferior vena cava filter was placed in order to prevent future episodes of pulmonary embolization. From day 10 and onwards, the patient was able to tolerate hemodialysis without increases in ICP. Hemodialysis was discontinued on day 21, as kidney function was rapidly returning. The patient was gradually weaned off sedation and mechanical ventilation; autonomic dysfunction ensued and was treated with baclofen and propranolol. On day 51 the patient was in a minimally conscious state (MCS) and was discharged from the NICU and transferred to a neurorehabilitation facility. Upon follow-up three months after discharge, she remained in MCS with signs of slow improvement. |
A 75-year-old man presented at the emergency room shortly after he sustained an injury from a horse fall. When he fell, the horse lost balance and fell on him, crushing his groin. His past history showed he was diabetic. His heart rate (HR) was 90 beats/min and blood pressure (BP) was 50/0 mmHg. Bruising, swelling, and distention were seen in the right groin on inspection. A large pulsatile hematoma in the right groin was palpated. There was a right lower quadrant tenderness, and no rebound tenderness or guarding (). The ankle pulses were palpable and there were no edema and ischemic changes in the lower extremities. His hemoglobin level was 10.2 g/dL on admission but decreased 1 hour later to 6.7 g/dL. After rapid transfusion of five units of packed red blood cells (RBCs) and five units of fresh frozen plasma (FFP) through a central venous catheter, his vital signs stabilized. His BP was 95/50 mmHg and his HR was 90 beats/min. An emergency computed tomography angiogram showed a hematoma in the right inguinal and femoral region, and active bleeding of the iliac or femoral artery was confirmed. There were no accompanying fractures of the pelvis or lower extremities ().\nWe determined that it would be difficult to access the injury site and secure the field during open surgery due to severe swelling and hematoma. If it took a lot of time to access the surgical site, the patient’s chances of resuscitation would be expected low. Therefore, we decided to perform an emergent endovascular procedure for rapid hemostasis under general anesthesia with C-arm. We directly punctured the left CFA and inserted a catheter to the right external iliac artery in a crossover approach. Active bleeding of the iliac artery was not seen on the intraoperative angiogram, but extravasation of the contrast media was confirmed in the femoral artery. The rupture site was the EPA, below the inguinal ligament, and quite above the bifurcation of superficial femoral artery and deep femoral artery. The rupture site was covered with an 8×50 mm Viabahn® stent-graft (W.L. Gore & Associates, Inc., Flagstaff, AZ, USA) (). The final angiography confirmed that there was no more bleeding from the artery, and blood flow to the right lower extremity was not disrupted.\nAfter removing of the endovascular device and compressing the puncture site for hemostasis, BP decreased to 70/35 mmHg and did not rise thereafter. After rapid transfusion of 3 units of RBCs and 1 unit of FFP through the Fluid Management System (Model 2516 universal 3-Spike disposable set; Belmont Inst., Billerica, MA, USA), BP rose to 110/50 mmHg. Bleeding from other sites was suspected; therefore, we performed a longitudinal incision and explored the right inguinal area. A 3-mm defect was found on the anterior wall of EIV above the right inguinal ligament. And a primary repair was performed with a prolene suture 5-0 (). Minor bleeding was controlled, a 200 mL Jackson-Pratt drain was inserted, and the incision was closed. The patient was then moved to the intensive care unit. Dual antiplatelet therapy (aspirin and clopidogrel) were started on the 10th day postoperatively, when the bleeding tendency decreased and disseminate intravascular coagulation improved.\nDuring the second postoperative month, pulmonary edema developed, and a non-ST-elevation myocardial infarction was diagnosed. A percutaneous coronary intervention was done, and a coronary stent was placed. There was repeated surgical site infection by staphylococcus epidermidis, a skin flora at the groin, and it was improved by intravenous administration of vancomycin. Wound debridement and skin grafts were performed twice (). As the inguinal wound improved, the patient was discharged 94 days after surgery. He has been continually monitored through the outpatient clinic, and 71 months after the deployment of the Viabahn® stent-graft, it is still patent (). |
A 12-year-old boy was referred to the children and adolescent psychiatric outpatient clinic with suspicion of ADHD. It is the third time reference with the same suspicion. First time reference was in 2011 and second time was 2014 where he did not meet the criteria for ADHD diagnosis. The third reference to the children and adolescent psychiatric outpatient clinic was in 2018. The reference from the patient's family doctor was described concentration difficulties, difficulties with emotion regulation, impulse control, and regulation of activity and sleep. The patient met the criteria for the diagnosis of ADHD at third control.\nThe patient was placed in a foster home when he was 8 months old. When he moved to his foster family, he was malnourished, dehydrated, and slept 18 hours a day. Based on the previous examination in the outpatient clinic, it appears that the boy has struggled with anxiety, concentration, and impulsivity since then. Some of his symptoms lead that the patient getting tired quickly and may fall asleep suddenly at school or in activity. At a younger age, he could suddenly fall asleep while sitting and playing in the sandbox. When he gets tired, he often gets headaches and visual disturbances. The last mentioned can disappear after getting some rest. His impulsivity shows by suddenly pushing a child he accidentally passes. He has difficulties to perceive messages and follow instructions. He strives to regulate the emotions, and the emotions fluctuate quickly and become unusually intense. When he gets angry, he gets very angry and apparently loses control of what he does. The patient was previously examined for sleep disorders. No suspicion of hypersomnia such as Kleine-Levin or narcolepsy was detected based on history taking (anamnesis). The patient had no signs or symptoms that could be related to obstructive sleep apnea syndrome (OSAS) either. He had problems with insomnia and poor sleep quality, no hypersomnia, snoring, or constant fatigue during the day.\nDuring the consultation at the outpatient clinic, we did the following tests: Ability Test (WISC-IV), Kiddie SADS, Playing observation, BRIEF, and Barkley. A summary of these tests is shown in . It was taken a complete blood sample of the patient as a part of routine examination which was normal. The summary of laboratory test results is shown in .\nGeneral physical examination is the following: normal findings of the cardiac, pulmonary, and abdominal examination. Diadochokinesis test is the following: jerky and slow movements on both sides and scores 5 points. Finger opposition test is the following: slow with slightly uneven left-sided mirror movements and scores 5 points. Walk on the lateral side of the foot is the following: relaxed extremity bilaterally and scores 2 points. Total score is 12 points (6 points more than 15 percentile). Otherwise, normal findings of the neurological examination are examined.\nBecause of intense agitation and sugar cravings, the patient was referred to EEG. Generally, minor neurological abnormalities can be observed in children with, for example, hyperkinetic disorders/learning difficulties, or cognitive dysfunctions. The EEG from November 2018 shows pathological activity with bilatero-temporal to central epileptiform activity, not synchronized (). There was no information about seizures, sudden uncontrolled twitching, or observations of collapse or fall down. The patient cannot remember things after the incident.\nAfter pathological EEG findings, the patient started treatment with Lamotrigine. Great improvement when it comes to agitation, moodiness, and reduction of sugar craving after starting with Lamotrigine. However, concentration difficulties and hyperactivity are unchanged; this may be due to ADHD. CNS stimulants are scheduled to start soon. |
A 56-year-old man was admitted to a local hospital for transient (10 min) left limb numbness when he rested on the sofa. Magnetic resonance angiography revealed that the right ICA was occluded from its origin to the intracranial segment. The patient reported taking aspirin, clopidogrel and atorvastatin, and was referred to the authors' center 2 weeks later. Physical examination revealed no obvious positive findings. Although a 2-month history of diabetes was recorded, there was no history of hypertension, hyperlipidemia, or smoking.\nThe initial CDFI was performed on day 1 of hospitalization, which revealed a patent right ICA with normal blood flow velocity (). However, CTA on day 2 indicated that the right ICA was occluded (). A repeat CDFI scan on day 4 of hospitalization revealed a hypoechoic mass [thickness 2.8 mm (suspected thrombus)] was attached to the anterior wall of the initial segment of the right ICA (). Magnetic resonance imaging (MRI) was performed on day 6; T1-weighted imaging showed that the intracranial segment of the right ICA was invisible, indicating that the right ICA was occluded (). However, digital subtraction angiography (DSA) performed on the same day revealed that the right ICA was normal, with no significant stenosis in any segment ().\nCDFI findings were consistent with those of DSA, but nevertheless inconsistent with CTA and MRI. It was not advisable for clinicians to make a definitive diagnosis pertaining to the vascular lesions of the patient. Given the generally good condition of the patient and no significant discomfort, he continued taking aspirin 100 mg/day, clopidogrel 75 mg/day, and atorvastatin 20 mg/day after discharge. Follow-up CDFI 1 and 3 months after discharge did not detect the hypoechoic mass that was attached to the anterior wall of the initial segment of the right ICA, and complete patency and normal blood flow velocity were apparently restored.\nFive months later, however, the patient was re-admitted to the authors' hospital again due to transient (~10 min) left limb weakness without obvious cause. Repeat CTA revealed severe stenosis of the right ICA, and the sagittal image revealed the right styloid process compressing the right ICA horizontally at the C2-3 intervertebral disc (). Subsequent 3D computed tomography reconstruction of the cervical spine revealed a bilateral overgrown styloid processes, which was ~6.3 cm in length on the right side and 6.1 cm on the left side ().\nSubsequently, CDFI depicted a long hyperechoic bony structure on the right side of the neck (), located between the base of skull and the ICA compressing the ipsilateral ICA causing visible artery stenosis (). Blood flow velocity in the ICA dramatically increased when the patient slowly turned his head to the right (), which was significantly altered compared with the normal position ().\nTCD was then used to monitor hemodynamic changes of the bilateral middle cerebral artery (MCA) in real time. Even when the patient was in a normal position, blood flow velocity in the right MCA was lower than in the left, but, nevertheless, remained in the normal range (). When the patient turned his head to the right, blood flow velocity in right MCA was significantly decreased (), but increased significantly when he turned his head back to the starting position (). Blood flow velocity in the left MCA did not change markedly during these maneuvers (). When the patient turned his head slowly and continuously, blood flow velocity in the right MCA changed dramatically, with no significant change on the left side ().\nThe patient underwent right styloidectomy. During the operation, the elongated styloid process could be visualized on the front of the right ICA. The middle and lower part of the overgrown styloid process was removed; the length of the specimen was approximately 4.2 cm (). Postoperatively, the right styloid process stump was detected and patency in the right ICA was restored (). TCD was repeated to monitor bilateral MCA in real time; there was no significant change on either side when the patient's head was rotated. |
A 22-year-old nulliparous lady with no previous medical illness presented with a 6-month history of a right breast lump. The lump was described as slow growing, but rapidly enlarged 3 weeks before presentation to the hospital. She denied having nipple discharge or fever. There was no family history of breast carcinoma.\nOn physical examination, the right breast was markedly swollen and tender. The overlying skin was warm and erythematous. A large firm mass was felt predominantly in the lower quadrants of the right breast. It was fixed to the superficial skin, nipple and underlying muscle. Several right axillary lymph nodes were palpable. The left breast and the left axilla were normal. She was afebrile with stable vital signs. Examination of the other systems was unremarkable. Blood investigation revealed normal parameters. She was immediately referred for a breast ultrasonography after a core biopsy of the mass was performed in the breast clinic. Ultrasound examination of the right breast revealed a large lobulated hypoechoic solid lesion occupying the lower quadrants and extending to the retroareolar region. The nipple was retracted towards the mass. The mass measured approximately 8.0 cm in width and 3.9 cm in height and contained cystic areas and few hyperechoic foci. The margin of the mass was partially well-delineated and mildly lobulated. Neither posterior acoustic enhancement nor shadowing was present. The surrounding breast parenchyma was heterogeneous and edematous. There was no satellite nodule. Color-Doppler study of the lesion showed minimal flow signal seen only at the medial side of the periphery of the mass. The rest of the mass was generally avascular. No penetrating vessel into the mass was seen. A moderate amount of flow signal was documented in the stroma surrounding the mass (). Multiple enlarged right axillary lymph nodes with loss of the fatty hilum were noted with the largest measuring 1.9 × 1.2 cm. Ultrasound of the left breast and axilla was normal.\nHistopathological examination of the core tissue revealed partly necrotic, focally crushed fragments of fibrofatty tissue exhibiting clusters and trabeculae of malignant epithelial cells surrounded by desmoplastic stroma. There was no obvious tubule or gland formation. The tumor cells exhibit moderately pleomorphic hyperchromatic nuclei, scanty cytoplasm and a high mitotic activity (10/10 hpf) (). Numerous apoptotic bodies were noted. Immunohisto chemically, the tumor cells were immune positive for synaptophysin, chromogranin and MNF116. Interpretation of the result was infiltrating neuroendocrine carcinoma of the breast. The patient initially refused further imaging or treatment, but returned to the clinic a month later. This time the breast mass had grown larger associated with skin ulceration. The previously palpable right axillary nodes had also become larger and fixed. A contrast enhanced computed tomography (CT) examination of the thorax, abdomen and pelvis revealed a heterogeneously enhancing mass 5.4 × 13.8 × 11.0 cm in size, occupying the whole of the right breast. The mass infiltrated the underlying pectoralis major muscle and a part of the underlying chest wall. Multiple enhancing enlarged right axillary lymph nodes were present (). The left breast and axilla were normal. There was no significant abnormality detected in the rest of the thorax, abdomen or pelvis and including the visualized spine bones.\nA combination of cisplatin and etoposide (100 mg/m2) neoadjuvant chemotherapy based on the protocol for small cell lung carcinoma was started immediately. Upon completion of the 4th cycle of chemotherapy, she developed bilateral lower limb weakness with urinary incontinence and sensory loss up to T4 level. Magnetic resonance imaging (MRI) of the spine (GE 1.5T) demonstrated metastases to the vertebral bodies of T2 to T4 with paraspinal and epidural soft tissue components that caused spinal canal stenosis and spinal cord compression at the T3 level. The paraspinal component was also indenting the posterior wall of the trachea and displacing the esophagus anteriorly (). Emergency decompression laminectomy and debulking of the paraspinal tumor was performed. After the surgery, she regained sensation with slight improvement of the power of both lower limbs, but on the 9th post surgical day, she suddenly developed chest discomfort and soon went into cardio-circulatory collapse. Attempts to revive her failed. Clinically, she was suspected of having acute pulmonary embolism. Post mortem autopsy was not performed on her as family members did not agree. |
A 55-year-old female with a history of renal cell carcinoma of the left kidney metastatic to the bony pelvis, lungs, mediastinum, and spleen presented to the emergency department with shortness of breath, pleuritic chest pain, and left scapular pain. She presented to the same emergency department one week prior with pleuritic chest pain but was discharged home after pulmonary embolism was ruled out.\nShe was diagnosed with renal cell carcinoma of the left kidney five years prior after presenting with gross hematuria. At that time, she underwent left radical nephrectomy. One year later, she developed a metastatic lesion in the bony pelvis for which she underwent radiation therapy. She as treated with pazopanib for two years with stable disease but stopped due to gastro-intestinal toxicity. Therapy was switched to nivolumab, which was discontinued after six months due to grade four pancreatitis and grade two rash. Eight months prior to her current presentation, she underwent radiation treatment to metastatic lesions in the left pubic symphysis and spleen. The patient initiated therapy with cabozantinib, a tyrosine-kinase-inhibitor used to treat renal cell carcinoma, three months prior to her current presentation.\nOn physical examination, she was wheezing in all lung fields and hypoxemic requiring supplemental oxygen. She had prior 12-pack-year smoking history but no formal diagnosis of chronic obstructive pulmonary disease (COPD). A chest x-ray revealed a small left pleural effusion and left basilar atelectasis. Laboratory workup, including complete blood count, renal and hepatic panels, and troponin, was unremarkable. An electrocardiogram (ECG) revealed sinus tachycardia without signs of ischemia. CT was not repeated due to her negative CT angiogram one-week prior. Given radicular and left scapular pain, an MRI of the spine was done, which revealed no pathologic metastases in the thoracic or lumbar spine but did reveal a new sacral lesion. Given her progressive stridor, she underwent laryngoscopy, which revealed a normal upper airway. A bronchoscopy showed significant trachea-bronchomalacia and thick purulent secretions in the left upper lobe, lingula, and right upper lobe.\nTwo days after admission, repeat chest X-ray revealed near complete opacification of left lung and large pleural effusion, a remarkably different radiograph from admission (Figure ).\nSubsequent CT chest revealed a large left pleural effusion with partial loculation as well as partial atelectasis of the left upper lobe and complete atelectasis of the left lower lobe. A right perihilar metastasis and perisplenic metastases were reported. The study was negative for pulmonary thromboembolism.\nThoracentesis revealed cloudy straw colored exudative effusion. A four French pigtail catheter was placed. Approximately 400 milliliters of yellow-green fluid was immediately drained. Pleural fluid studies revealed a white blood cell count of 33,000/μL (97% neutrophils), pH of 6.44, LDH of 4760 U/L, and an amylase of 394 U/L. She was started on vancomycin, cefepime, and metronidazole for presumed empyema. Pleural fluid cultures showed heavy growth of lactobacillus species, heavy growth of anaerobic gram negative cocci, and moderate growth of Candida krusei. Antimicrobial therapy was subsequently narrowed to ertapenem and anidulafungin. Given lack of improvement and continued significant chest tube output over the following week, further CT imaging was obtained, revealing a gastro-pleural fistula (via the left diaphragm and superior posterolateral stomach) with associated complex pleural effusion containing contrast material and gas (Figure ). This process abutted the known splenic metastases.\nAn esophagogastroduodenoscopy (EGD) revealed a 1.5-cm fistula in the posterolateral stomach that opened to the pleural space (Figure ).\nEndoscopic suturing was attempted to close the fistula with limited success (partial closure noted on imaging, with methylene blue dye taken via mouth visualized in the chest tube drainage catheter on water seal; Figure ).\nFor complete closure, the authors attempted a novel approach utilizing a venting gastrostomy tube and chest tube to water seal to facilitate closure of the fistula over the ensuing six weeks. Enteral feeding via jejunostomy tube to aid closure of the fistula was employed. The patient was continued on ertapenem and anidulafungin. She was also initiated on a proton pump inhibitor. She was discharged to a rehabilitation facility with plans to repeat imaging and methylene blue swallow in six weeks.\nUnfortunately, CT scans after six weeks showed that the fistula remained patent. A second attempt was made at endoscopic closure, which was again unsuccessful. One month later, during a hospitalization for electrolyte abnormalities, the patient decided to pursue elective surgical repair of the fistula in hopes of regaining the ability to resume normal oral intake. Four months after her initial presentation, she underwent laparoscopic surgery for fistula repair. The surgeon visualized extensive radiation fibrosis involving the stomach, spleen and retro-peritoneum. Given these findings and to avoid splenic bleeding, they pursued a conservative surgery whereby they stapled the stomach to ligate the gastro-pleural fistula anatomically. This approach is novel and was successful in our patient. A fluoroscopic upper GI series with oral contrast three days after surgery demonstrated no leakage of contrast outside of the GI tract or into the pleural space, and CT five days after surgery revealed no evidence of communication between the stomach and pleural space (Figure ).\nShe tolerated an oral diet. Gastrostomy tube, jejunostomy tube, and chest tube were removed without complication. |
A 25-year-old Sri Lankan female presented with weakness of all four limbs in January 2014. She had a similar illness 12 years back.\nIn 2002, at the age of 13 years, patient had noticed tingling sensation of distal upper and lower limbs followed by weakness of lower limbs, involving both distal and proximal muscle groups. Weakness was progressive and ascending, involving both upper limbs and neck muscles by day 6 of the illness. There was no dysphagia, dysphonia, respiratory difficulty or bladder/bowel involvement. There was no significant medical history suggestive of preceding infection, toxin ingestion or similar disease in the past. On examination, there was flaccid quadriparesis (muscle power grade - 3/5) with areflexia in all 4 limbs. There were no cranial nerve palsies or features suggestive of autonomic involvement. Sensory system examination was normal. Full blood count, blood picture, serum electrolytes, blood urea and liver enzymes profile were normal. Her erythrocyte sedimentation rate was 13mm during 1st hour. Nerve conduction studies performed on day 4 of the illness showed focal segmental demyelinating type sensory and motor neuropathy with conduction blocks (Table ), suggestive of acute inflammatory demyelinating polyneuropathy (AIDP). EMG studies were not performed during this episode. CSF analysis on day 14 of the illness revealed normal results with proteins – 30 g/dl (normal 15–40 g/dl), white cells 2 with 100 % lymphocytes and normal CSF glucose levels compared to plasma values. She was treated with intravenous immunoglobulin for 5 consecutive days in addition to physiotherapy and discharged on day 18. She made a complete recovery in 3 months based on normal tone, power and deep tendon reflexes on neurological examination. Follow-up NCS was not performed after clinical recovery.\nIn 2014, patient readmitted with numbness and progressive ascending weakness of all four limbs for 3 days duration and had developed poor cough response, dysphagia and difficulty in breathing during the following 2 days. This episode was preceded by an upper respiratory tract infection two weeks back. There was no similar illness noted in any of her family members. On examination, flaccid quadriparesis with generalized areflexia was noted with a muscle power of 1/5 in lower limbs, 2/5 in upper limbs and 2/5 in neck muscles. Weakness progressed to involve bilateral seventh cranial nerves and bulbar muscle without ophthalmoplegia. Rest of the neurological examination including sensory system and other organ system examination were normal except for a resting tachycardia of 130 beats/min without any significant blood pressure fluctuations. Respiratory rate was 30 cycles/min with oxygen saturation of 92 % on air. Nerve conduction studies done on day 10 of the illness revealed focal segmental demyelinating type sensory and motor neuropathy with prolonged distal motor latency, delayed F-wave and conduction blocks, concluding as AIDP variant of GBS (Table ). Electromyogram done on day 10 of the illness did not show any evidence of denervation but noted fibrillation potentials of positive sharp waves. CSF analysis on day 10 and a repeat study on day 24 showed normal results without cyto-protein dissociation or pleocytosis (Table ) Full blood count, blood picture, serum electrolytes, erythrocyte sedimentation rate, blood urea and liver enzymes profile were normal. Serology for Mycoplasma, Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, hepatitis B & C and retroviral studies were negative. Autoimmune panel including anti-nuclear factor was normal. Due to the rapid progressive nature of weakness and respiratory distress, patient was mechanically ventilated for 12 days. Diagnosis of GBS was made and intravenous immunoglobulin 0.4 g/kg/day was administered for 5 days. In addition, she received physiotherapy to support her recover in motor function of limbs and speech therapy following extubation. She made a good clinical recovery assessed subjectively as well as objectively and was discharged home on day 28 with a muscle power of 4/5 and deep tendon reflexes of +1.\nOn follow-up, patient had normal neurological examination findings and subsequent nerve conduction study after 4 months revealed normal results (Table ). |
A 29-year-old gravida 4, para 3 female with a past medical history notable for type 2 diabetes and elevated HgbA1c at 7.5% was first noted to have a fetal abnormality by nuchal translucency ultrasound (US) at an outside institution, with nuchal translucency measuring 3.8 mm. She underwent genetic counseling but declined further invasive testing. Cell-free DNA screening was performed and results were low risk for Down syndrome, trisomy 13 or 18. Given these abnormalities, however, she underwent further fetal ultrasound at 16 weeks and was noted to have ventriculomegaly with a suspected posterior fossa mass. She presented to our institution at 20 weeks 6 days gestation, and fetal US and brain MRI were performed. Obstetric ultrasound demonstrated symmetric bilateral enlargement of echogenic choroid plexus with associated ventriculomegaly and enlarged extra-axial fluid-filled spaces (Figure ), with differential diagnosis including intraventricular hemorrhage, choroid plexus hyperplasia or choroid plexus papilloma. Head size was larger than expected for gestational age (GA). Biparietal diameter measured 6.7 cm (corresponding with 27 weeks 2 days GA) and head circumference measured 24.2 cm (corresponding with 26 weeks 1 day GA).\nAn MRI scan of the fetal brain performed on the same day demonstrated bilateral ventriculomegaly (Figure ). The lateral ventricles measured up to 14 mm in atrial diameter, with normal morphology of the third and fourth ventricles. There was also marked enlargement of the supratentorial and infratentorial extra-axial subarachnoid spaces. The choroid plexus appeared enlarged bilaterally without definite hemorrhage on echo-planar imaging. Additionally, there was no susceptibility or T2 hypointensity outlining the ventricles or brainstem, suggesting that there was no prior intracranial hemorrhage.\nBased on these MRI findings, targeted Doppler US was performed and demonstrated increased flow within the choroid plexus bilaterally. Thus, the US findings suggested enlarged vascularized choroid, rather than intraventricular hemorrhage. No other fetal morphologic abnormality was noted. Mild parenchymal thinning was present, likely secondary to ventriculomegaly. The findings on prenatal US and fetal MRI suggested bilateral choroid plexus papillomas or hyperplasia with increased cerebrospinal fluid (CSF) production leading to hydrocephalus and enlarged subarachnoid spaces. The differential diagnosis included possible sequelae of dural venous sinus atresia, leading to decreased CSF absorption and engorgement of the choroid plexuses, and a follow-up study was recommended.\nFollow-up sonogram at 22 weeks 6 days gestation demonstrated marked macrocephaly (head circumference 30.4 cm corresponding with 33 weeks 1 day GA) with further increase in the size of bilateral choroid plexuses and of extra-axial CSF volume. Prominent flow was again shown by Doppler US within the enlarged choroid plexus (Figure ), making bilateral choroid plexus papillomas or hyperplasia the most likely diagnoses. Choroid plexus papilloma was favored, due to the relative rarity of choroid plexus hyperplasia [].\nThe fetal head size continued to enlarge. On obstetric sonogram at 31 weeks 3 days, the biparietal diameter measured 22.6 cm and head circumference measured 75.3 cm, both significantly beyond standard measurements of fetal head size.\nThe patient presented in preterm labor at 31 weeks 6 days. Due to marked macrocephaly, fetal cephalocentesis was performed at the time of delivery. Under ultrasound guidance, a 22-gauge spinal needle was introduced through the uterine wall, through the fetal scalp and into the enlarged extra-axial CSF space of the fetal head. The spinal needle was connected to a vacuum bottle, with controlled drainage of 3.5 L of xanthochromic CSF. Post-procedural ultrasound demonstrated no evidence of intracranial hemorrhage. The fetus was subsequently delivered by cesarean section, but demised shortly after delivery, despite efforts at resuscitation. Postnatal pathology confirmed atypical choroid plexus papillomas with ependymal differentiation (WHO grade II; Figure ). |
A 9-year-old, previously healthy female was admitted to The First Affiliated Hospital of Wenzhou Medical University (Wenzhou, China), and presented with abdominal pain and fever. Upon examination, the patient’s temperature was 38°C, the pulse was 86 beats per minute, the blood pressure was 103/53 mmHg and the respiratory rate was 20 breaths per minute. Abdominal tenderness was identified in the right upper quadrant without rebound tenderness. The remainder of the examination was normal and the results are shown in . Abdominal CT scan revealed a cystic and solid mass with a size of 11.5×8.3×6.7 cm3 and a well-defined border, which involved the right lobe of the liver and had a non-homogeneous density (). The density was marginally and heterogeneously increased in the enhanced CT scan (). The clinical impression was that this mass represented a hepatoblastoma.\nSurgery was performed to resect the tumor. A right subcostal incision was made and, following entry into the abdomen, the ligamentum teres hepatis and falciform were incised. Examination of the future liver remnant and abdominal cavity revealed no evidence of tumor metastasis. The tumor had ruptured at the diaphragmatic surface and had adhered to the diaphragm. The surgical margin distance from the tumor was at ≥1 cm. The tumor was resected following the margins, until it was completely removed. A drainage tube was inserted under the right diaphragm and exited the body through the right abdominal wall. The abdominal wall was closed using standard procedures. During surgery, the patient had 300 ml bleeding and was transfused with 1.5 units of red blood cells.\nHistological examination revealed pleomorphically shaped tumor cells with marked nuclei and clear to the eosinophilic cytoplasm (). All the primary antibodies used in the present study are shown in . Immunohistochemical staining of the tumor cells showed positive expression of caldesmon (), cluster of differentiation (CD) 68 (), Ki67 () and vimentin (), but negative expression of α-fetoprotein (AFP), creatine kinase (CK), desmin, hepatocyte, muscle specific actin (MSA), myogenic differentiation (myod) 1, myoglobin and smooth muscle actin (SMA). On the basis of these findings, the patient was diagnosed with UESL, which was confirmed by two surgical pathologists.\nThe patient was recommended for further treatment, which was refused due to socioeconomic and psychological reasons. Thus, adjuvant chemotherapy was not initiated. Following complete tumor resection, the patient was regularly examined. Abdominal CT scans and US were performed every 3 months to monitor tumor recurrence. At the 6-month follow-up, the patient was surviving free of disease; however, at the 9-month follow-up, unenhanced CT revealed a right, low-density prerenal recurrent mass with a size of ~2.7×2.4 cm2 and a well-defined border (). Enhanced CT showed a marginally and heterogeneously increased density (). The patient did not accept any further treatment. Two months later, CT scan demonstrated that the mass had enlarged to ~3.3×3.4 cm2. A right prerenal dissemination of UESL was suspected. Based on the patient’s condition, a second surgical procedure was required.\nUpon second admission, the patient was comfortable and physical examination revealed there was no abnormal condition. The laboratory results are shown in . Complete prerenal tumor resection was performed. There was tumor hemorrhage inside. Microscopic analysis showed that the tumor was evidence of undifferentiated embryonal sarcoma recurrence. However, the patient was comfortable and physical examination revealed no abnormal conditions. In addition, the laboratory results were normal. Abdominal computed tomography scan and ultrasound were performed every 3 months to monitor the tumor recurrence. At the time of writing, it has been 6 months after the second surgical procedure and there has been no appearence of abnormalities. |
A previously well 87-year-old Caucasian woman living in a senior assisted care center presented to the neurology clinic with complaints of six months of slowly progressing left sided weakness. Initial difficulty in ambulating and using the stairs progressed to being wheelchair bound. Neurologic exam revealed diffuse 3/5 left sided weakness, left leg drift, and left facial droop. Brain magnetic resonance imaging (MRI) revealed a large confluent white matter T2-hyperintensity in the right frontal lobe with multifocal nodular enhancement of the left cerebral hemisphere (). Foci of enhancement were also identified in the cerebellum and leptomeninges. The radiologic differential diagnosis included vasculitis, lymphoma, and CNS sarcoidosis as the most probable causes of the multifocal disease process, with glial neoplasm, demyelination, and metastases considered less likely.\nAll sample analysis described below were performed on material obtained by brain biopsy as part of clinical care. All samples were obtained with appropriate consent.\nA biopsy of the mass was performed and revealed extensive parenchymal lakes and vascular and perivascular deposition of amorphous, amyloid like material (). Congo-red positive staining and apple-green birefringence (not shown) of the amorphous material upon polarization confirmed that the amorphous material was amyloid (). Also present in the resected tissue were a number of small intraparenchymal blood vessels with perivascular lymphoplasmacytic infiltrates (). The initial histologic differential diagnoses included cerebral amyloid angiopathy-inflammatory type (CAA-I) and lymphoma associated amyloidoma. To identify the underlying etiology of the amyloid accumulation, a number of additional analyses were performed.\nLiquid chromatography tandem mass spectroscopic analysis identified the amyloid as AL λ-type and not β amyloid or an amyloid associated with a hereditary amyloidosis. Further analysis of the perivascular lymphoid populations was undertaken. Histologically, the monotonous populations of perivascular lymphoid cells demonstrated a lymphoplasmacytic appearance (). Immunohistochemical analysis demonstrated that the lymphoid cells were CD20 positive (). Tumor cells were negative for CD3, CD5, BCL1, and CD23. The tumor Ki67 proliferation index was low (3%). The more plasmacytoid appearing cells were CD138 positive and were shown to be lambda light chain restricted by kappa and lambda chromogenic in situ analysis (Figures and ). An immunoglobulin heavy chain (IgH) gene rearrangement analysis of the brain tissue from this case was positive for a clonal process with a 253-base pair peak in the FR2 region. A MYD88 L265P mutation analysis by PCR-based pyrosequencing on the brain tissue from this case was negative. A diagnosis of a low grade, lymphoplasmacytic lymphoma (LPL) was rendered. The identification of this CNS low grade lymphoplasmacytic lymphoma confirmed the cause of the amyloidoma to be a lambda light chain producing lymphoplasmacytic lymphoma.\nTo determine if an extracranial/systemic lymphoplasmacytic lymphoma was the source of the CNS neoplasm, a bone marrow biopsy was performed. The bone marrow biopsy showed normal trilineage hematopoiesis and no evidence of lymphoma, myeloma, or amyloidosis. Cytogenetics and fluorescent in situ hybridization studies on the bone marrow were negative for genetic aberrations. Urine protein and serum immunoglobulin levels were within normal limits. A biopsy of subcutaneous abdominal adipose tissue was negative for amyloid, demonstrating lack of evidence of systemic amyloid deposition. Interestingly, an IgH gene rearrangement analysis on the bone marrow was positive for a clonal gene rearrangement with two peaks: a 282-base pair peak in FR2 region and a 120-base pair peak in FR3 region in a polyclonal background, which importantly were markedly different from the IgH gene rearrangement identified in the CNS lymphoplasmacytic lymphoma. Since the two-small bone marrow clonal peaks are present in a polyclonal background, their significance is uncertain and may be age related.\nOur patient received one cycle of chemotherapy with Rituximab for Primary CNS lymphoplasmacytic lymphoma. Two months after diagnosis, she developed a hemorrhagic infarct on the left frontal white matter and was transferred to hospice care. |
A 30-year-old woman visited the hospital due to right lower quadrant pain over the period of 1 week. A laparoscopic myomectomy was performed 4 years ago because of a broad ligament leiomyoma, which was about 10 cm in size. Laboratory findings included a routine blood examination, and a C-Reactive Protein (CRP) test, with tumor markers all found to be within normal ranges. The pelvic Magnetic Resonance Imaging (MRI) scan showed some nodules at the posterior margin of the bladder that were considered to be endometriosis, and some pelvic effusion that was significant on the right side. It was also noted that there was a mass shadow at the lower right ureter (medial to the iliopsoas muscle), with distention of the lower right ureter (Fig. ). The patient also underwent computed tomography (CT) scans to enable the practitioners to observe the size of the abdominal mass and its surroundings. The CT images revealed a region occupying the middle right ureter that was considered to be a retroperitoneal aggressive fibroma, which led to severe hydronephrosis on the right kidney and upper ureter, and a right pelvic effusion (Fig. ). After more detailed examinations were conducted, there were no obvious abnormalities detected in the brain, heart, liver, gallbladder, spleen, pancreas or blood. The color Doppler ultrasound demonstrated that there was a hypoechoic mass next to the right iliac vessels that was closely related to the adjacent ureter. This resulted in severe hydronephrosis of the right kidney and a right upper ureteral dilatation (Fig. ). Ndzengue et al. [] reported a case of a pelvic desmoid tumor simulating a uterine leiomyoma recurrence. The patient that presented at our hospital had a history of uterine leiomyoma. We subsequently organized a multidisciplinary consultation to determine the next stage of her treatment plan. According to the patient’s surgical history, the next step would be determined after reviewing the results of the last surgical pathological wax, because the pathological nature of the retroperitoneal mass was uncertain.\nConsequently, a laparoscopic pelvic tumor resection with assistance from a Da Vinci robot was successfully conducted. A local peritoneal protuberance was observed in the right iliac vascular area. The parietal peritoneum was incised above the mass and it was carefully freed along the edge of the mass. The size of the tumor was about 6.0 cm × 5.0 cm × 3.0 cm. It had invasively grown and it was wrapped around the right ureter and the right ovarian arteries and veins. It was stuck to the psoas muscle and the iliac vessels. According to preoperative preparations and intraoperative conditions, a decision was made to cut off the right ureter, the right ovarian arteries and veins, and completely remove the tumor and the two masses that were located in front of the broad ligament on the right hand side of the uterus. The sizes of the masses were approximately 1.5 cm × 1.0 cm × 1.0 cm. The right ureter was anastomosed and put into a double J tube. A pathological diagnosis of an intraoperative frozen sample determined that it was a spindle cell soft tissue tumor, and the two masses were leiomyomas. Postoperative pathology tests of the pelvic mass determined that it was aggressive fibromatosis that had invaded the ureteral wall (Fig. ). The uterine surface nodules were also leiomyomas. Microscopically, the tumor cells were arranged sparsely in a spindle shape with blood vessels of different sizes found in the interstitial tissue. Immunohistochemical findings were found to be partially positive for smooth muscle actin (SMA) and desmin, and less than 5% ki-67 of positive cells were seen in the lesion. A detailed re-examination was performed 3 months after the initial surgery, to determine the structure and function of the ovaries. The transvaginal color Doppler ultrasonography was able to determine that the ovaries were normal in size with several follicular echoes. The blood supply to the right ovary was good. There were no obvious abnormalities in the uterus or pelvic cavity (Fig. ). The pelvic MRI and the CT scan of the whole abdomen determined that there were no abnormal lesions in the pelvis. Simultaneously, the endocrine function of the patient’s ovaries was found to be normal, and she was able to self-maturate after removing the double J tube. |
An 82-year-old man, who had previously undergone the first successful endovascular repair of a ruptured chronic type B dissection, presented to us again five years after his first procedure [].\nHis original diagnosis of a Stanford type B aortic dissection had been made in 1994. He was initially managed medically with antihypertensive medication alone, however seven years later, he suffered sudden collapse and chest pain. A ruptured false lumen thoracic aneurysm was diagnosed by spiral computed tomographic angiography (SCTA). The aortic dissection extended distally from the left subclavian artery to the left common iliac artery. The coeliac axis appeared to have a common origin from both the true and false lumens, whereas the left renal and inferior mesenteric arteries originated from the false lumen.\nHe was deemed to be unsuitable for open surgery due to significant medical co-morbidity, including atrial fibrillation, ischaemic heart disease and chronic obstructive airways disease so therefore endovascular treatment of his condition was undertaken []. The challenge was to exclude the rupture while maintaining perfusion of his gut and kidneys. This was undertaken using a total of four Gore Excluder endografts (WL Gore & Associates, Flagstaff, Ariz.). This has been previously described [].\nAfter a stormy post-operative course, he was discharged home with regular clinical and radiological follow-up, but after two years he declined to attend further review.\nFour years after his original procedure, he re-presented to a nearby hospital with a one-month history of increasing chest and back pain associated with shortness of breath. Chest X-Ray showed left lower zone shadowing and he was treated for pneumonia. His respiratory symptoms improved, but his chest and back pain continued. Further laboratory investigations revealed that he was hypercalcaemic with a corrected calcium of 2.77 mmol/l. SCTA revealed a haematoma in the left mid-thoracic cavity associated with vertebral body erosion. The hypercalcaemia was attributed to this bony erosion and it was postulated that this had been caused by the pulsatile haematoma giving rise to his symptoms of chest & back pain. The patient was transferred to the vascular unit at our institution. Three dimensional SCTA reconstruction revealed a probable defect at the junction between the middle two of the four stents (adjacent to the haematoma), which appeared to have only 5 mm of overlap. Above the level of the haematoma, an apparent perforation of the second thoracic stent was seen. The haematoma was thought to be originating from either or both of these structural defects. These defects therefore constituted a type III endoleak [Figure , ]\nThe initial plan was to perform re-stenting on an urgent basis meanwhile optimising his general medical condition. However, on the third day of admission his condition deteriorated suddenly as he became hypotensive and confused. A decision was made with the patient & his family to perform immediate re-stenting of the thoracic aorta as an emergency.\nUnder general anaesthesia, the right brachial and right common femoral arteries were exposed and cannulated. A standard guidewire was passed from the brachial artery to the right common femoral artery using a snare device from below ensuring that the true lumen was entered and avoiding entering either of the two false lumens in the abdominal aorta. Endovascular repair was then carried out in the standard fashion through the right common femoral artery. A 42 × 200 mm Medtronic Talent endograft (Medtronic, Santa Rosa, Calif.) was deployed across the site of blowout and the presumed defective junction. A further 42 × 150 mm Medtronic Talent endograft was used to overlap and extend distally. SCTA performed the following day demonstrated good stent position with no evidence of endoleak [Figure ].\nThe patient's postoperative recovery was complicated by postoperative pneumonia requiring intravenous antibiotics. Despite supportive treatment he eventually succumbed on the ninth postoperative day to this respiratory complication. |
A 30-year-old woman sought medical attention in our department for a recurrent iliac tumor. Ten years previously, she had felt a growing lump and pain in her right iliac bone that intensified with walking. She was admitted to a local hospital, where the surgeons removed an osseous tumor from her iliac bone, and the subsequent pathological studies showed that it was a low-grade chondrosarcoma. Three years before presentation, the patient felt pain while walking and noticed a similar lump in the previously operated area. Fifteen months before presentation, she visited her local hospital because of increasing pain and growth of the lump in her right iliac bone. Pathological studies following fine needle aspiration showed the same diagnosis of low-grade chondrosarcoma. Three months later, the patient visited our department for more specialized surgical treatment. Physical examination after admission showed a 4- × 4- × 3-cm lump with a clear boundary and no mobility. The patient reported mild tenderness when placing pressure on the lump. The patient had no active infection or skin impairment that could prevent us from scheduling a surgery, no sensory deficiency, and no muscle atrophy. No abnormalities were found in the muscle strength, sensory function, or circulation of the lower extremities. Computed tomography (CT) scans of the pelvic bone showed osteolytic damage in the right iliac body and iliac wing with a large soft tissue mass (). A CT-guided biopsy suggested grade I chondrosarcoma.\nBecause chondrosarcoma is not sensitive to chemotherapy, and considering the availability of a safe and mature technique with which to remove the lesion in a one-step surgical procedure, we considered one-step surgery to be the optimal treatment regimen. Any contraindications for surgical intervention were ruled out with a comprehensive blood test and examinations of the patient’s circulatory, respiratory, urinary, and gastrointestinal systems. Magnetic resonance imaging was performed to exclude early tumor penetration of the pelvic bone outside the large bone mass. After completion of all preoperative evaluations, surgery was scheduled with the consent of the patient and her family. The 3D printed tantalum implant was prepared by reconstruction of the patient’s CT scan with a spatial resolution of 20 lp/cm and density resolution of 0.67 mm ().\nImages of the patient’s pelvic bone and chondrosarcoma were obtained by a CT scanner (Siemens Healthineers, Erlangen, Germany), and the scan data from the Picture Archiving and Communication System (PACS) were reconstructed by Mimics 17.0 software (Materialise NV, Leuven, Belgium). The extent of the lesion was outlined, and the preoperative surgical plan was established using the 3D model. The 3D data including the surgical plan was stored in STL format and sent to an engineer for implant printing. The implant was designed by an engineer according to the surgical plan, bone quality, and patient’s overall condition (e.g., weight and height) under the parameters of “Personalized Additive Manufactured Medical Device Technical Censoring Guidelines (February, 2018)” by the Center for Medical Device Evaluation of China (). Finite element analysis was carried out to evaluate the probable biomechanical stress distribution of the implant. A porous structure was used in the 3D painted implant to avoid bone resorption and implant loosening from stress shielding. The implants were 3D printed using tantalum powder by Xi’an Sailong Metal Materials Co., Ltd. (Xi’an, China). The density of the implant was 2.7 g/m3, the diameter of each aperture was 200 µm, and the extent of porosity was 40%. The muscle and ligament suturing points were designed according to the pelvic CT and magnetic resonance imaging scans. Tumor recession guidance plates were designed to cut the tumor 2 cm beyond tumor border, and a 3-mm-diameter hole was made in the middle of the plate to fix it to the bone with a 2.5-mm-diameter Kirschner wire (). |
A 68-year-old male presented to the head and neck clinic with history of a progressive midline lower neck swelling which appeared suddenly, increased in size progressively and became prominent over 6 weeks. On examination, the swelling was about 5 × 2 cm in size, mobile and not adherent to the skin or underlying structures. The swelling was not prominent on tongue protuberance. Thyroid was impalpable and patient was clinically euthyroid with no evident Grave's disease features. It was neither associated with fever nor any other constitutional symptoms. His pressure symptom includes mild dysphagia to solids which compelled him to visit our hospital, but he did not encounter any respiratory complaint.\nPatient was not on any anticoagulants, but he was taking regular medications for diabetes and hypertension for many years.\nFor treatment decision, the patient was referred to radiology department. Ultrasound neck showed a large well defined thin-walled cystic neck lesion measuring about 5.1 × 2.6 cm (transverse x anteroposterior). There was no appreciable internal septation or solid tissue in it (). No hypervascularity was noted within or periphery of the lesion (). The internal content of the cyst was of 2 types, basal part was homogenously low echogenic with curved upper border while rest of the part was filled up with anechoic fluid (). Few tiny echogenic foci were also seen in its low echogenic part which were not giving any comet tail artifact to be confidently called as colloid content (). Thyroid gland was not easily visible due to lateral push by this large cyst. Thyroid isthmus was hardly visible clearly. Lobes were found to be normal, but their echotexture was heterogenous and micronodular with normal vascularity and intact echogenicity. According to the location and content of cyst, the closest sonographic differential in our patient was thyroglossal cyst, but it is usually smaller in size, moves with tongue protrusion and more cranial in position. Other differentials could be hemorrhagic or fourth branchial cleft cyst.\nLeft lobe was having a solid well-defined isoechoic nodule measuring around 2.5 × 2.0 × 1.5 cm with some internal vascularity on doppler ultrasound ().\nConsidering the differentials and ambiguous site of mass origin, referring team discussed the case with radiologist and planned to proceed for contrast enhanced computed tomography neck. Computed tomography (CT) showed a non-enhancing midline cyst in the region of isthmus measuring around 5.1 × 3.5 × 5.6 cm (transverse x anteroposterior x craniocaudal). It was pushing both lobes of the thyroid gland inferolaterally and strap muscles anterolaterally with no signs of aggression. The craniocaudal extension of the cyst was from the level of the thyroid cartilage to just above the clavicles (). The attenuation of the fluid content was varying between 27-51 Hounsfield Unit. No calcification was appreciated (). No worth noting mass effect or its connection with the airway was noted. On careful inspection of images, isthmus was unappreciable and we were able to see a “claw sign” in the thyroid lobes which helped us to discern the organ of origin (). The differentials of non-inflammatory thyroid cysts including atypical colloid and hemorrhagic cyst were given on CT scan with a suggestion of cyst aspiration to have definitive diagnosis. 3D volume rendered images for skin demonstrated clinically visible swelling (). 3D volume rendered airway images clearly showed no mass effect (). After observation of “claw sign” on CT scan we ruled out thyroglossal and branchial cysts from our differential list.\nLab results showed strongly positive antithyroid peroxidase antibodies (TPO), while thyroid stimulating hormone, triiodothyronine (T3) and thyroxine (T4) were normal. Rest of the blood examination findings were unremarkable.\nPathology assessment of the diagnostic aspirate showed, 18 ml of fluid having abundant colloid, few scattered foamy macrophages and hemosiderin. It was consistent with benign cystic colloid nodule with hemorrhagic changes, Bethesda Category II. It was found negative for malignancy. Fine needle aspiration (FNA) of the left thyroid nodule showed small groups as well as isolated follicular cells with focal Hurthle cell changes and rare collection of foamy macrophages in the background of thick colloid. Few clusters showed follicular epithelium with focal nuclear atypia including nuclear overlapping and grooving, putting it under Bethesda category III with further advise to have follow up and repeat FNA at appropriate interval.\nDiagnostic aspiration was later followed by therapeutic colloid aspiration. On follow up visit, symptom of dysphagia was resolved. Due to atypical cells on histopathology and high-risk age group for thyroid malignancy, left hemithyroidectomy was done on subsequent visit. Patient did not report further complaints. |
A 22-year-old gentleman presented to our clinic reporting that he was struck in his left eye with a tree branch two days previously. Immediately after the injury he vigorously flushed his eye out with tap water, but the next day he continued to have eye pain and poor vision and reported to the local urgent care facility. He was diagnosed with a corneal abrasion, started on sulfacetamide drops every two hours, and advised to follow-up with an ophthalmologist. He was seen in our clinic for a follow-up evaluation the next day. Upon examination he was found to have a best-corrected visual acuity (BCVA) of 20/200 and a 2 × 3 mm epithelial defect just below the corneal apex. The defect was noteworthy because it was associated with a very prominent infiltrate of the anterior corneal stroma. Cultures of the cornea were performed for bacterial, viral and fungal organisms, and the patient was started on moxifloxacin eye drops every hour with directions to follow-up in 24 hours.\nThe next day there was no significant change in the patient's condition. He was seen again 48 hours later and the epithelial defect was almost healed except for the appearance of moderate punctate epithelial erosions in the distribution of the original abrasion as well as the dense stromal cellular infiltrate. Although the BCVA had improved to 20/30, the stromal infiltrate persisted and involved the anterior one-third of the cornea. Cultures had become positive for germ tube-negative yeast (fig. ). There was no consolidation of the infiltrate, abscess formation or ulceration. Furthermore, the deep cornea remained uninvolved and there was no anterior chamber reaction. The patient was immediately started on voriconazole 1% drops every hour. Seventy-two hours later, eight days after the initial injury, the patient was examined again and his vision had improved to 20/20 (BCVA) and the epithelial defect had healed. Still, a cellular infiltrate remained in the anterior one-third of the corneal stroma, though it was concentrated more centrally with peripheral clearing (fig. ). The patient was sent for confocal microscopy. Confocal images (fig. ) revealed both round and dumbbell shaped structures suggestive of budding yeast forms as well as extensive branching structures resembling hyphae or pseudohyphae structures. Twelve days after the initial injury the patient was seen again and the infiltrate seemed to have improved and vision remained stable. By this visit the fungal culture was identified as R. mucilaginosa by DNA sequencing, and after a review of the literature suggested that amphotericin B would provide good coverage [], topical amphotericin B 0.15% was added to his treatment schedule. One week later the patient returned and the infiltrate seemed to be nearly resolved, but a residual haze of the anterior corneal stroma persisted. One month after the initial injury only a faint stromal haze remained. Repeat confocal microscopy at this time (fig. ) demonstrated that the inflammatory process had impressively resolved leaving what appeared to be residual fragments of the previously seen branching structures that had lost their tubular architecture. The patient was continued on amphotericin and voriconazole for a total course of three months at which time medications were discontinued with apparent full resolution of the infectious process. A full three months of treatment was administered because it is known that fungal infections require prolonged therapy []. The patient has continued to do well. |
An 11-year-old African-American male presented to our care in January 2011 because of excessive daytime sleepiness and episodes of losing muscle tone upon experiencing strong emotional stimuli. Before seen by us, the patient was evaluated by a psychiatrist in March 2010 at a request of his mother. Clinician records suggest that the patient was being seen for not being able to express himself, labile mood, lack of focus and concentration at school, anxiety, and lack of self-esteem. At the end of the evaluation by the psychiatrist, the patient was diagnosed with Adjustment disorder of childhood with mixed emotions, depressed feelings, and attention deficit hyperactivity disorder primarily inattentive type. Furthermore, the patient scored 55 (normal = 91-100) on the global assessment of functioning (GAF) score. It is a numerical scale utilized by mental health clinicians to determine the patient's day to day functionality. Specifically, it allows the clinicians to score and determine their social, occupational and psychological functioning [].\nAfter presenting to our care, the patient was sent for further workup including the Epworth Sleepiness Scale (ESS), a nocturnal polysomnogram (NPSG), and a multiple sleep latency test (MSLT). The ESS is a subjective test utilized to measure the patient’s sleepiness []. It includes eight scenarios in which the patient rates their tendency to become sleepy. The scale ranges from 0 (no chance of dozing) to 3 (high chance of dozing). These eight scenarios are sitting and reading, watching television, sitting inactive in a public place, as a passenger in a car for an hour without a break, in a car while stopped for a few minutes in traffic, lying down to rest in the afternoon, sitting and talking to someone, and sitting quietly after a lunch without alcohol [, ]. Our patient scored 17 which is highly associated with pathologic sleepiness because a score of greater than 15 suggests that the patient is excessively sleepy and should be treated.\nNPSG is a sleep study which records brain waves, oxygen levels, heart rate and breathing patterns along with eye and extremity movement as the patient sleeps []. According to the results of NPSG, the patient slept 367.00 minutes out of 440.7 minutes in bed for a sleep efficiency of 83.3%. The patient spent 66.4% of total sleep time in supine position. In addition, the patient spent 16.9%, 38.3%, 27.5% of sleep time in stage 1, 2 and 3, respectively. The study identified five REM sleep periods with REM stage lasting for 17.3% of sleep time. REM latency was recorded as normal at 87.5 minutes. Furthermore, the sleep study noted sleep latency (SL) to be 3.2 minutes. Overall Apnea-Hypopnea Index was 0.5 events/hour. The REM specific index was 1.9 events per hour. During the study, one obstructive hypopnea was with a mean duration of 14.3 seconds. There were two mixed apneas with a mean duration of 10.7 seconds along with 0 central apneas. Laboratory workup for HLA-DR15 and DQ0602 was positive.\nMSLT is the primary diagnostic tool for narcolepsy and is typically performed following an NPSG to measure sleep latency []. Sleep latency can be described as the amount of time it takes to go from wakefulness to entering sleep. Also, it measures sleep onset REM periods (SOREMPs) which illustrates how quickly the patient enters REM sleep []. This test typically includes four or five naps lasting for 20 minutes each. Results of the MSLT consisted of five nap trials with a sleep latency of 1.8, 3.4, 4.7, 2.9, 6.4 minutes, respectively. In addition, REM latency during five nap trials was recorded to be at 2.5, 1.0, 1.5, 0.5 and 2.5 minutes, respectively. The result shows that the patient slept during each nap trial with mean sleep latency (MSL) to be at 3.8 minutes. Lastly, the study recorded five SOREMPs with a mean REM latency of 1.6 minutes. Based on the workup, the patient was diagnosed with narcolepsy which has been successfully managed with stimulant therapy and sodium oxybate. |
The patient was a 2-year-old boy from Russian republic of Ingushetia. Both parents and younger sibling (female) were asymptomatic. Family history is not burdened. Parents may be related, as the mother of the child and the grandmother (father's line) are from neighboring villages of Malgobek region of Ingushetia.\nThe patient is the first child of healthy parents. Pregnancy proceeded against the background of gestosis in the first and second trimester, threats of interruption for a period of 12 weeks, anemia, and lack of hydration. The child was born as a result of fast unassisted childbirth. His birth weight was 3570 g and height was 55 cm. Apgar scores were 6/7. Early motor development slightly delays: He attained head holding at 3 months, sitting without support at 6 months, by the year the boy got up and walked with support. From the age of 8 months, the baby had a weight loss of 2 kg for 2 months with episodes of repeated vomiting. At the age of 1 year 2 months against a background of acute respiratory viral infection, there was a regress in development—the boy stopped walking. Subsequently, rachitic skeletal changes, an increase in the volume of the abdomen appeared, and a loss of motor skills progressed. Magnetic resonance imaging (MRI) of brain revealed a mildly expressed leukopathy in white matter of parietal lobes. Proteinuria and glucosuria appeared at the age of 1 year 8 months.\nAt the age of 2 years 2 months, he was admitted to Russian children's clinical hospital with psychomotor retardation and treatment resistance. On physical examination, he was observed to have a pronounced delay in psychomotor development (he did not get up, walk, crawl; he could only keep his head and turn over). Neurological examination revealed muscular hypotrophy, tendency to hypotension in axial musculature, and uniform decrease in tendon reflexes from hands and feet. MRI of brain revealed subatrophic changes, moderate expansion of the lateral ventricles within the subatrophy with compensated liquorodynamics, and the presence of hypomyelinization zones in the periventricular white matter of the posterolateral regions.\nClinical exome sequencing was carried out by Genotek Ltd. Genomic DNA from peripheral blood sample was extracted using QIAamp DNA Mini Kit (Qiagen, Hilden, Germany). DNA libraries were prepared using NEBNext Ultra DNA Library Prep Kit for Illumina (New England Biolabs, Ipswich, Massachusetts, USA) with adapters for sequencing on Illumina platform. Double barcoding was performed by PCR with NEBNext Multiplex Oligos for Illumina kit. Quality control of DNA libraries was carried out using Bioanalyzer 2100 (Agilent Technologies, Santa Clara, California, USA). We used SureSelect XT2 (Agilent Technologies, Santa Clara, California, USA) for target enrichment. Enriched samples were sequenced on Illumina HiSeq 2500 using pair-end 100 base pairs reads. After sequencing, we trimmed 3′-nucleotides with read quality below 10 using Cutadapt. Raw reads were aligned to reference genome hg19 (GRCh37.p13) using BWA MEM. Deduplication of reads was carried out using SAMtools rmdup. FastQC was used for data quality control. We called short variants using GATK HaplotypeCaller according to GATK Best Practices DNA-seq. The effect of each mutation was assessed using snpEff. To assess pathogenicity and conservatism, the data were extracted from the dbNSFP, ClinVar, OMIM database, and HGMD, as well as using the SIFT and PolyPhen-2 utilities to predict pathogenicity of the mutation. Information on the frequency of mutations was taken from 1000 Genomes project, ExAC, and Genotek frequency data. Description of mutations and their pathogenicity was predicted according to the Standards and Guidelines developed by ACMG (American College of Medical Genetics and Genomics), AMP (Association for Molecular Pathology), and CAP (College of American Pathologists). Copy number alterations were determined using CNVkit. CTNS variant identified by exome sequencing was confirmed by Sanger sequencing of patient, parental, and sibling DNA samples.\nAfter exome sequencing, on admission in Veltischchev Research and Clinical Institute for Pediatrics, patient was 9 kg in weight and 80 cm in height, with low weight-for-height proportion and disharmonious physical development. Deficiency of subcutaneous fat was noted. Attention was paid to rachitic changes in skeleton: expansion of metaphyses, keel-like deformation of thorax, rachitic “rosary,” varus deformity of lower limbs. Hepatosplenomegaly was noted: liver and spleen protruded from under edge of the costal arch by 2.5 cm. Clinical signs include polyuria and polydipsia (consumed liquid per day 1300 mL, daily diuresis—1300 mL). Blood tests showed mild anemia, hypokalemia, decrease in uric acid, increase in alkaline phosphatase activity, hypophosphatemia, increase in triglycerides, and creatinine level at the lower limit of the norm. The study of equilibrium of acids and bases of blood revealed metabolic acidosis. Test of thyroid gland hormonal profile showed subclinical hypothyroidism. Level of cystine in leukocytes was normal. Glomerular filtration rate (GFR) (97 mL/min) was within the normal range (80-120 mL/min). Urine test showed phosphaturia, glucosuria, and low molecular weight proteinuria. Cystine was detected in urine test. Ultrasound examination showed hepatosplenomegaly, multiple foci of increased echogenicity in liver, nephromegaly with thickening, and diffuse changes in renal parenchyma. Radiologic examination revealed pronounced osteoporosis, lagging behind bone age. Ophthalmoscopy revealed cystine crystals in the cornea. Thus, the child has signs of infantile form of nephrotic cystinosis with preserved kidney function.\nThe patient was commenced on cysteamine (Cystagon), initially 50 mg orally four times daily, to increase gradually to 100 mg four times daily (0.8 g/m2/d). Also he was commenced on ophthalmic solution Cystadrops (one drop six times daily) and potassium, phosphorus, vitamin D, energotropic agents. After 6-month therapy, the patient's condition remains stable and serious with slight positive dynamics. The child gained 1 kg in weight and grew by 0.5 cm. Polydipsia/polyuria decreased to 1000-1200 mL. Hemoglobin and red blood cell levels, and thyroid-stimulating hormone and T4 levels were normalized. Minimal phenomena of metabolic acidosis persist. Also, filtration function of the kidneys was preserved, and no signs of nephrocalcinosis were detected.\nAt the last examination, patient had elevated cystine level in the granulocytes (2.32 nmol) and nephrocalcinosis of both kidneys. Cysteamine dose was increased to 1.25 g/m2/d. |
A 48-year-old married woman presented to our hospital with intermittent gross hematuria for 3 months. She had regular menstrual cycle of 28 days without pain around her waist line or abdominal pain and no history of gynecologic surgery. She denied a family history of cancer, endometriosis, genetic and psychosocial diseases.\nThere was no obvious mass on the palpation of the bilateral kidney area, and no percussion pain. An ultrasound of the urinary system was recommended first. Conventional ultrasound examination was performed under standardized settings using Toshiba ultrasound systems (Aplio500, Tokyo, Japan) (ultrasound transducer: PVT-375BT, frequency 3.5 MHz). Ultrasonography showed a cystic mass in the lower pole of the right kidney with septa and well-circumscribed. The thickness of the cystic mass wall is about 4 mm. Doppler color flow imaging showed a small signal in the wall of the mass, but no blood flow was detected in the cystic part. Contrast-enhanced computed tomography (CT) of the kidney was then performed. It showed a heterogeneous hypodense mass in the lower pole of the right kidney, measuring ~1.8 × 1.5 × 1.4 cm (). The mass was polycystic, with slight enhancement of the wall and septa and no enhancement of the cystic area. Contrast filling of the mass was not observed during the excretory phase. The right lower pole of the kidney was partially depressed, and there was some local thinning of the renal cortex. Given the slight enhancement of the mass, the possibility of cystic renal tumor could not be ruled out, and surgical resection was planned. Laparoscopic right renal mass resection was subsequently performed at our hospital on the 7th day after her menstruation. After successful anesthesia, the surgeon opened the lateral retroperitoneum under laparoscopy, pushed the colon away and opened the Gerota's fascia, then carefully dissociated the right kidney. The right lower pole of the kidney was partially irregular and contracted. A well-demarcated cystic tumor with the size of 2.5 × 2.0 × 2.0 cm could be seen at the lower pole of the right kidney after surgical incision, which involved the renal parenchyma but had clear boundaries, and the mass was easily peeled off. The mass composed of brown central area showed several branching cavities, with 5 mm wide homogenous light-yellow zone at its periphery. Perioperatively, the tumor was confined to the right kidney without extracapsular invasion, which did not encroach on the perirenal fat sac or surrounding organs. The mass was completely resected, with an ~0.5 cm margin of normal renal tissue. The resected mass had a diameter of ~1.5 cm. There were several capsular spaces containing brown fluid, and the cut surface of the mass was yellowish. Microscopic examination confirmed the diagnosis of renal endometriosis characterized by endometrial glands and embedded stromal cells (). Immunohistochemical analysis revealed that the stromal cells and epithelial cells were positive for estrogen receptor (ER), progestin receptor (PR), and vimentin, which further supported the diagnosis of renal endometriosis. Histopathology was reviewed by two senior pathologists separately, without any atypia. The patient recovered well after the surgery, and there were no obvious space-occupying lesions by ultrasonography in the kidney at the 3 months follow-up. The patient is very satisfied with the treatment of this disease. |
The patient was a 70-year-old female whose past medical history was significant for arthritis and a right total hip arthroplasty approximately 9 years ago. A laparoscopic cholecystectomy (LC) for acute cholecystitis was performed approximately at another hospital approximately two months prior to presentation. She developed a surgical site infection with Escherichia coli (E. coli) bacteremia following her LC and was treated successfully with intravenous (IV) antibiotics. The postoperative course was also complicated by choledocholithiasis requiring an endoscopic retrograde cholangiopancreatography (ERCP) with stone pulverization and placement of two plastic 10F × 12 cm biliary stents. Two days prior to admission, she was hospitalized at an outside facility in septic shock with fevers, chills, lethargy, altered mental status, and blood and urine cultures positive for E. coli. At that time, she endorsed right hip pain and an inability to move her hip or leg. A computed tomography (CT) scan of her right hip revealed two partly calcified soft tissue masses associated with the right iliopsoas and obturator internus muscles (). A CT-guided fine needle biopsy of the right hip and psoas locules aspirated 100 mL of frank pus notable for a nucleated cell count of 344,000 (98% PMNs) with growth of E. coli. As a result, the patient was transferred to our institution with concerns for an iliopsoas abscess and a periprosthetic infection.\nOn admission, she was febrile to 102.7 F without any significant distress. Her physical examination was remarkable for a well-healed, right lateral hip incision with no erythema or drainage. She experienced pain with right hip flexion and internal rotation. Laboratory studies showed WBC, hemoglobin and hematocrit, basic metabolic profile, and liver function tests all within normal limits. A 3 cm hepatic abscess was identified on CT scan of the abdomen and pelvis. An MRI of the right hip showed a large air- and fluid-filled collection tracking along the iliopsoas bursa and psoas musculature into the pelvis (). This collection communicated with the hip prosthesis, and on CT imaging, the acetabulum component appeared to be medialized beyond the medial wall of the acetabulum as depicted in . Regarding the hepatic abscess, the patient was managed with IV antibiotics and interventional radiology (IR) placed drainage catheter.\nTo address the hip periprosthetic infection, the patient was managed in multiple surgical stages. In the first stage, an irrigation and debridement of the right hip and explantation of components were performed through an anterior approach. The femoral and acetabular components were explanted. Purulent material was seen draining from the pelvis through a medial acetabular wall defect into the hip joint. Approximately 1 liter of pus was evacuated from the hip joint. Multiple irregularly shaped granulated pea-sized pieces of hard brown substance were found deep in the acetabulum. A handful of this material was removed which suggested that these were spilled gallstones from the patient's recent LC.\nTemporary components were replanted with an antibiotic impregnated cement spacer system [] (Figures and ). An antibiotic cement spacer with gentamicin was placed in the acetabulum defect, and a loosely fitted antibiotic-cemented stem was placed in the proximal femur.\nThe intrapelvic iliopsoas collection could not be fully debrided through the anterior approach, and IR was consulted for drain placement and serial debridements, which were conducted with a rotating basket Trerotola device over the course of the next four weeks. In , contrast dye can be seen tracking from within the iliopsoas abscess into the hip joint.\nFour weeks following her explantation, the patient returned to the operating room for placement of a second temporary weight bearing custom-fitted prosthesis made from methyl methacrylate with gentamicin-impregnated antibiotics [] (Figures and ). For the acetabulum, methyl methacrylate was molded in its doughy state into the cavities and deformities of the acetabulum, and a polyethylene acetabulum was pressed into the cement. The femur was reamed to size of a large diameter chest tube. A femoral stem and a reinforcing wire were cemented into the chest tube, and once the cement was hardened, the femoral stem encased in a solid cylinder of bone cement was removed from the chest tube and malleted into the proximal femur.\nPostoperatively, the patient did well. She was made weight bearing as tolerated, ambulated with physical therapy and elected to delay placement of permanent components. She was eventually discharged to a short-term rehabilitation facility with a 6-week course of IV antibiotics.\nApproximately 18 months later, she presented to our clinic complaining of hip and thigh pain with ambulation. She was followed by infectious disease as an outpatient with multiple hip aspirations which had negative cultures. X-rays revealed her temporary prosthesis to be stable, but with radiolucencies primarily around the femoral component (). The patient was taken to the operating room, the temporary prosthesis was removed, and a long porous coated system was inserted (). Intraoperative cultures grew vancomycin-resistant enterococcus (VRE), and the patient was eventually discharged to home with a 12-week course of daptomycin and outpatient physical therapy. Now, two years from her initial explantation, she continues to follow up monthly in our clinic and states that she is doing well. Her final construct is shown in . She continues to have a moderate limp but ambulates without assistive devices. Her pain is much improved and she is no longer on chronic antibiotic suppression with no clinical signs or symptoms of recurrent infection. |
Patient is a 45-year-old female patient, with past medical history of gastritis. Six months before she sought medical attention, she was complaining of mild upper abdominal pain, however, due to her previous condition she was medicated with proton pump inhibitors assuming that the pain was due to her gastritis. In the last 3 months, she noticed a mass in her upper abdomen and the pain became more severe and started to affect her daily activities, thus she presented to a routine physical exam. No weight loss or other constitutional symptoms were noticed. Also no history of malignancy in her family was mentioned. On clinical examination, a 12 × 15 cm2 mass was discovered on her upper abdomen, mild pain was found on palpation but no tenderness was present. An abdominal ultrasonography revealed a 15 × 15 cm2 septate thin-walled giant cystic mass, it was anechoic and had a volume of ~735 ml. The cyst occupied most of the hepatorenal space and it was in intimate contact with the liver, right kidney and pancreas (Fig. A). Due to the size of the mass, CT was requested, it revealed well-defined 19.7 × 15 × 10 cm3 giant cyst, the cyst was located within the small bowel mesentery and it was in intimate contact with the liver, gallbladder, pancreas and right kidney (Fig. A). Nevertheless, the tomography could not rule out that the mass had some sort of communication with the bile duct or the pancreas. This is why a magnetic resonance cholangiopancreatography found that the cyst was attached but had no communication with the bile duct or the pancreas (Fig. A). CBC and blood work was normal.\nDue to the size of the cyst and clinical condition surgery was planned. At laparotomy, a 19 × 15 × 10 cm3 mesenteric cyst was discovered (Fig. B), the cyst was near the root of the small bowel mesentery but did not compromise its main vessels, it had a yellowish appearance (Fig. B), and clear fluid was found within the cyst. Multiple adhesion were identified between the cyst wall and the transverse colon, liver, third portion of the duodenum and gallbladder. With these findings surgery was straightforward, complete resection of the cystic mass was performed, however, the cyst wall was completely attached to the body of the gallbladder and could not be resected without compromising it (Fig. B), so a cholecystectomy was performed as well. After this, the remainder of the procedure continued without any complication.\nPathology reported a giant primary mesenteric cyst, it was lined with a single layer of cuboidal epithelial cells and partially covered by adipose tissue and prominent vasculature, it had a yellowish wall and its thickness varied between 0.1 and 0.3 cm (Fig. C). Its interior had a white trabecular internal surface with brown patches it did not have any communication with other organs, and no areas of malignancy were found (Fig. C).\nIn the early postoperative period, a tachycardic patient with severe abdominal pain and tenderness was encountered, she became hypotensive an oliguric, thus an emergency re-laparotomy was performed. The 1000 ml of hemoperitoneum was encountered and after extensive washing of the abdominal cavity, an arterial vessel from the resected gallbladder was discovered and successfully managed. After this surgery patient fully recovered. Sips of liquids were initiated on the third postoperative day, and full diet was initiated a day after. She was discharged without any complications and, on follow up controls patient is doing well. |
A 73-year-old woman presented with persistent mid and lower abdominal pain. She is known to have IBS, and blood investigations showed iron deficiency anemia. At the time of presentation, the patient had no relevant history of medical comorbidities nor history of smoking; National Early Warning Score (NEWS) was 1 (blood tests outlined in ). She was referred for abdominopelvic ultrasonography which revealed left renal mass around 18 cm with a mixed solid and cystic component; also, hypoechoic foci were noted within the pancreas (Figures and ).\nSubsequent chest CT and CT urogram were done and confirmed a large left renal mass of 18 cm × 10 cm × 12 cm with central necrosis and peripheral hypervascularity (). The left renal vein was patent. The adrenal gland looked separable from the mass. Multiple hypervascular foci were noted within the pancreas. Small lung nodules were also noted. Multidisciplinary team (MDT) decision was for cytoreductive nephrectomy and to consider systemic therapy.\nA month later, the patient had an open left nephrectomy. Renal cell carcinoma, clear cell type grade II, with no lymphovascular invasion was evident in pathological assessment; surgical margins were clear; and the tumor was classified as grade II pT3a clear cell renal cancer with evident renal vein involvement and no involvement of Gerota's fascia, adrenal gland, perinephric fat, or adjacent organs.\nShe had evidence of a small volume of metastatic disease in her preoperative imaging. Follow-up every 3 months was scheduled; interval scans in between guided by the clinical examination were done.\nInitially, the patient had shown stable minimal progress of the metastatic disease for which systemic therapy was not indicated at that time. 16-month postnephrectomy, CT follow-up revealed stable chest disease with new hepatic metastasis for which she started sunitinib treatment (). Three months later, brain CT showed brain metastasis after she got an episode of confusion (). So full head radiotherapy was started. Further progression of the metastatic disease and recurrence of the primary with new deposits in the spine were noted for which radiotherapy was indicated.\nAt the same time, the patient was referred on red flag breast clinic for assessment of a newly felt lump in her right breast. On examination, the lump was in the upper part of the right breast 1-2 cm, hard, and poorly mobile with no signs of fixation to the covering skin or the underlying muscle. There was fullness in the axillae but no discrete palpable nodes. The patient stated that she had a positive family history of breast cancer.\nBilateral mammography showed a dense new mass lesion as compared to her previous mammogram done 2 years earlier (Figures and ). The mass was seen in the upper half of the right breast without microcalcifications. Since it was a new finding, M4 score was given. Breast ultrasound (US) confirmed a rather well-defined hypoechoic solid mass lesion in the right breast at 1 o'clock measuring 20 mm × 13 mm with tiny cystic changes. Axilla was normal. It was reported as U5 in ultrasound. US-guided core biopsy was done in the same clinic ().\nEven though the patient had widespread RCC metastatic disease, it was important to clarify if this lesion represented a primary breast tumor which should indicate endocrine therapy (if ER-positive) or a secondary from the RCC which should be managed conservatively unless getting symptomatic.\nPathology revealed breast tissue infiltrated by tumor with a nested pattern composed of clear cells. A panel of immunohistochemical markers reveals positive staining with the renal markers PAX 8 and carbonic anhydrase (Figures –). CK7 is negative as are the breast markers GATA 3 and mammaglobin. Given the clinical history, the features were those of metastatic renal cell carcinoma. |
A 46-year-old nondiabetic woman from a middle-class family, presented with discharging sinuses over the lower anterior abdominal wall for the past 2 years. There was no rise of temperature or weight loss. Her incision wound from laparoscopic appendectomy in 1998 did not heal properly and the surgical site was reopened after 1 month. There was no healing of the incision wound after reexcision. After that the whole area started to become erythematous and indurated and later on a keloid started developing over the scar. A large sinus developed over the operation scar and the other stitch sites also developed into sinuses discharging pus. However, there was no history of cough, haemoptysis, breathlessness, and anorexia. There was no history of intake of any immunosuppressive drugs/corticosteroids, any local trauma/injury, or tuberculosis. She had been treated off and on without having any definitive microbiological diagnosis with multiple injectable and oral antibiotics before consultation at this hospital. She did not have any contact with tuberculosis patients.\nOn examination, a previous-surgical scar was present over which a large keloid had formed, on the lower anterior abdominal wall in the midline (although a little more towards the right). There was no apparent mass over the abdomen, but 6 sinus openings were noted over the lower anterior abdominal wall (). At the time of presentation a single sinus was discharging pus. On palpation, tenderness was noted over and around the sinuses. The sinus opening was bluish, wide, and undermined and it was discharging a serosanguinous exudate admixed with pus. Complete haemogram and clinical chemistry were within normal limits including blood sugar. HIV ELISA negative by two kits, C-reactive protein normal and the ESR, was peaked at 22 mm. Pus from the discharging sinus was collected and processed using standard procedures. Gram stained smear showed plenty of pus cells with Gram variable bacilli (many of which were Gram positive) and on Ziehl–Neelsen staining numerous acid fast bacilli could be observed. The discharge was cultured on blood agar, MacConkey medium, and Lowenstein-Jensen medium and inoculated into a BacT/ALERT MP bottle, which was incubated in the BacT/ALERT 3D system (bioMerieux, USA). The blood agar and MacConkey medium were incubated under aerobic conditions at 37°C. The blood agar plate showed numerous dry, small nonhemolytic colonies after 72 hours but there was no growth on the MacConkey medium. The BacT/ALERT MP showed positive growth in 5 days for acid fast bacilli, which on further subculture onto L-J medium gave dry, rough, pigmented, and yellow to orange colonies after one-week period (). They were found to be scotochromogenic at 37°C. Speciation for the isolate was confirmed by the GenoType Mycobacterium CM/AS assay based on Mycobacterium DNA strip technology (Hain Lifescience, Nehren, Germany), [] following manufacturer's instructions. It showed a mixed infection caused by two NTM species, that is, Mycobacterium szulgai and M. intermedium. To confirm microbiological result, culture from repeat sample after one week showed the same dual infection caused by Mycobacterium szulgai and M. intermedium by the GenoType Mycobacterium CM/AS assay. Drug susceptibility testing (DST) was performed by standard disk diffusion method over Middlebrook 7H10 agar supplemented with OADC (oleic acid, dextrose, and citrate) and the following results were obtained: sensitive to linezolid, levofloxacin, clarithromycin, amikacin, and ciprofloxacin and found to be resistant to cotrimoxazole. DST for 1st and 2nd line antitubercular drugs were done by the GenoType MTBDRplus and MTBDRsl assay (Hain Lifescience, Nehren, Germany) which showed resistance to isoniazid and sensitive to rifampicin, fluoroquinolones, aminoglycosides/cyclic peptides, and ethambutol.\nThe patient was advised a combination of rifampicin 600 mg once daily, ethambutol 600 mg once daily, and clarithromycin 500 mg twice daily to be taken along with periodic follow-up based upon clinical response as well as microbiological response.\nShe was advised to continue the therapy and when she presented to the hospital after one month, there was no pus discharge from the sinus. She was advised to continue the therapy and report to hospital after another month. Again after one month of therapy, there was no discharge present and the sinus was almost completely healed. Thereafter, she was advised to continue the same therapy for another two months. Thereafter, patient was completely all right with no such discharge after completion of two-month antibiotic therapy. |
A 58-year-old male presented with decreased vision in his left eye, without redness or floaters, which had persisted for three days. His medical history detailed a diagnosis of nephrotic syndrome (membranous nephropathy), which had been treated with prednisone for a period of four months. Drug-induced diabetes was subsequently detected one month after the onset of corticosteroid treatment. The patient developed productive cough, pyrexia, and night sweats, and was further diagnosed with pneumonia three weeks before the admission. His medications at the time included prednisone 20 mg daily, insulin and oral cefdinir. He had no history of ocular trauma or surgery.\nAn ophthalmic examination revealed visual acuities of 20/20 in the right eye and counting fingers at 2 ft in the left. Pupils were 3 mm and reactive in each eye without relative afferent pupillary defect. The right eye was normal. Slit lamp examination of the left eye was unremarkable. Fundus examination showed clear media and a creamy-white and raised subretinal lesion located at the posterior pole, with the lesion being five disc diameters in size. Additionally, multiple retinal hemorrhages were seen in the overlying retina (Fig. ). The intraocular pressures were normal in both eyes. Optical coherence tomography (OCT) demonstrated a hyperreflective substance located in the subretinal space, between the outer retina and retinal pigment epithelium (Fig. ). Although a further diagnostic workup was requested on the follow day of presentation, the patient was lost to follow-up and presented five days later with reduced vision in the affected eye. On examination, slit lamp examination of the left eye showed mild conjunctival injection and anterior chamber cells of 2+. Fundus examination revealed mild vitritis and further, the area of the retinal lesion had expanded over the superior and inferior arcades (Fig. ).\nThe patient was admitted for further workup. All vital signs were stable and within normal range. The white cell count was 11.48 × 109/l, with 75.8% neutrophils, and the blood level of C-reactive protein was 59.9 mg/liter. Serum markers of human immunodeficiency virus (HIV), syphilis and tuberculosis were all negative. A chest computed tomography (CT) scan showed multiple bilateral nodules, some of them cavitated in the lungs, which suggested of multiple abscesses (Fig. ). In the context of his lung abscess, a presumptive diagnosis of endogenous endophthalmitis with subretinal abscess was made.\nA vitreous tap along with empirical intravitreal injections of a combination of vancomycin (1 mg/0.1 ml) and ceftazidime (2 mg/0.1 ml) were performed on the day of hospitalization. The vitreous samples were sent for bacterial and fungal cultures, as well as bacterial, fungal and viral polymerase chain reaction (PCR). Blood and sputum cultures were also performed. Topical levofloxacin 0.5% and prednisolone acetate 1% eye drops were administrated four times daily, and in addition, oral prednisone and cefdinir were continued. Three days after initial intravitreal injections, the subretinal abscess had improved significantly (Fig. ) and a second injection was administered. The subretinal abscess gradually improved, leaving only a small inferior lesion a week later (Fig. ). Vitreous cultures and PCR were all negative, as were blood cultures. Nocardia, Heamophilus and Candida albicans were identified in sputum cultures. Based on the clinical data and the characteristic appearance of the ocular lesion, it was deduced that the causative organism was most likely Nocardia. As a result, a combination of oral trimethoprim and sulfamethoxazole (TMP-SMX) was administrated. Ten days after hospitalization, the third intravitreal injections were repeated, with the patient then being discharged for follow-up as an outpatient with directions to continue with oral prednisone 20 mg daily and oral TMP-SMX for an further month. The patient remained in stable condition over the following four weeks after discharge. Further fundus examination revealed the subretinal abscess had resolved, leaving some yellowish subretinal precipitates and chorioretinal scarring, with visual acuity of 1/10 (Fig. ).\nSeven weeks after discharge, that is three weeks after cessation of TMP-SMX therapy, this patient was admitted to another hospital due to right extremity weakness and a lack of ability to walk or stand on his own for ten days. Magnetic resonance imaging (MRI) of the brain showed multiple cystic-enhancing lesions with surrounding edema over both cerebral hemispheres, suggestive of multiple abscesses, whilst the retina remained quiescent (Fig. ). A chest CT scan showed multiple lung abscesses. Blood cultures were positive for Nocardia, and as such disseminated nocardiosis was diagnosed. Based on sensitivity data, the patient was administered with intravenous imipenem/cilastatin and oral TMP-SMX, with intravenous therapy being continued for three weeks, followed by TMP-SMX monotherapy. The patient was discharged due to improvement in his systemic condition with the recommendation of close follow-up by an infectious disease specialist. Long-term oral TMP-SMX was also prescribed for the patient. Within follow-up chest CT and brain MRI carried out three months later, it was found that the previously detected foci had become smaller or had completely disappeared. A six-month follow-up indicated that the lung abscesses disappeared, and a brain MRI showed the previous residual lesions had almost completely resolved. |
A 67-year-old male patient reported to the Department of Prosthodontics with the chief complaint of difficulty in mastication due to dryness of mouth and absence of teeth. Medical history revealed that the patient had undergone RT 2 years back for the moderately differentiated squamous cell carcinoma involving posterior 1/3rd of left lateral border of the tongue.\nExtraoral examination revealed reduced lower facial height and unsupported lips. Intraoral examination revealed maxillary and mandibular edentulous residual ridges and decreased salivary flow [Figure and ]. A diagnosis of xerostomia secondary to RT was made after calculating unstimulated whole saliva flow rate by absorbent (swab) method (whole saliva flow rate was < 0.1 ml/min).\nTreatment plan included either (1) fabrication of implant supported or conventional upper and lower complete dentures with external application of salivary substitute in the oral cavity or (2) fabrication of lower complete denture with artificial saliva reservoir in it and upper conventional complete denture. It was discussed with the patient and informed consent was obtained for the agreed treatment, which included rehabilitation of the upper arch with conventional complete denture and reservoir denture in the lower arch.\nPrimary impressions were recorded in irreversible hydrocolloid impression material. After border molding, final impressions were made in medium body elastomeric impression material [Figure and ] Jaw relation was recorded with wax occlusal rims fabricated on self-cure acrylic denture base. The vertical height of the lower rim was measured. Accordingly, teeth selection was carried out and size of the reservoir was determined, so that maximum space for the reservoir is gained while maintaining 2 mm thickness of the reservoir wall. Then using face bow, only upper cast was mounted on the semi-adjustable articulator. Upper and lower wax rims with the centric records were preserved and set aside for later use Shellac base plate was adapted on the lower final cast. According to the predetermined dimensions, putty type of elastomeric material was placed in the center of the base plate and modeling wax (modeling wax, DPI, Mumbai, India) was added at the periphery to create the walls of the reservoir []. Space for reservoir was created by removing putty from the center []. Five stainless steel rods (3 mm in length and 2 mm in diameter) were positioned parallel to each other in the wax []. The two parts of the reservoir lower denture would be attached to each other using these rods. A bevel was carved 1-1.5 mm above the inferior border at the periphery of the base Then flasking and dewaxing was carried out as a routine procedure. Clear heat cure polymerized resin was used for packing the mold space (Trevalon HI, Dentsply India Ltd., Gurgaon, India) and after curing a permanent clear acrylic base was obtained with five stainless steel rods embedded in it and having a reservoir in the center. Thus, the tissue bearing plate of the two piece reservoir denture was fabricated [] Next, the lower cast was retrieved from the flask and with the preserved wax occlusal records it was mounted on the semi-adjustable articulator. After this, the lower temporary record base was replaced with the permanent clear acrylic base [] Teeth arrangement was carried out on the semi-adjustable articulator with putty maintaining the reservoir space []. Extra bulk of wax was added to the mandibular trial denture to gain optimum thickness. Then trial dentures were finished and tried in the patient's mouth to check for retention, stability, esthetics, phonetics and comfort [] Flasking followed by dewaxing of the lower trial denture was carried out to obtain base portion of the flask containing permanent denture base [] and a counter portion containing the teeth [] Aluminum foil was adapted on the denture base for easy separation of the two halves of the mandibular denture []. Packing was carried out with pink heat cure acrylic resin (Trevalon HI, Dentsply India Ltd., Gurgaon, India) and processed to obtain the teeth bearing plate of the reservoir denture [] After careful deflasking aluminum foil was removed. The teeth bearing plate of the mandibular denture rested on the bevel created on the tissue bearing plate. Finishing and polishing was carried out with the segments together to ensure a flush smooth finish, without any damage to the edges [] Two outlet holes were drilled for the salivary substitute on each side in the retromylohyoid region of the teeth bearing plate and were adjusted in size similar to the thickness of a 23 gauge orthodontic wire [] Artificial saliva substitute (Wet mouth, ICPA Health Products Ltd., India) was then filled in the reservoir space of the lower denture with a capacity of 3.8 ml The upper denture was fabricated in the conventional manner Denture insertion was carried out and post-insertion instructions were given to the patient. Furthermore, he was taught how to open and close the reservoir denture for filling the artificial salivary substitute and cleaning the reservoir space. In addition, fine orthodontic wire was provided to him for cleaning the drainage holes if they get blocked Recall appointments were scheduled after 24 h, 1 week, 4 weeks and every 6 months. At recall appointments, good retention was observed and relief from the symptoms of dry mouth and improvement in dietary intake was reported by patient. Patient found it easy to clean the dentures as taught and informed that once filled saliva substitute lasts for 4-5 h. Patient has now been successfully wearing the dentures for over 2 years. |
A male patient, aged 20 years, reported to the Department of Periodontology and Oral Implantology with the chief complaint of replacement of missing right lower back tooth. Patient wanted the fixed replacement of tooth (#46), but without getting the adjacent teeth grinded. Therefore, treatment option of implant with its benefits and risks was explained to the patient and an informed written consent was procured from him.\nA thorough medical, dental and personal history of the patient was recorded. Patient was found to be systemically and periodontally healthy []. The patient revealed that he was an off and on social drinker. The adverse effects of alcohol on healing were explained and the patient was asked to abstain from alcohol during the treatment and healing period.\nDiagnostic casts were made along with radiographic assessment of the implant site. A computed tomographic (CT) scan of the site was also obtained to accurately assess the dimensions of the site as well as the bone density. The mesiodistal dimension of the implant site was 8 mm and bucco-lingual dimension was 5 mm. The distance of alveolar crest from the anatomical structures like mental foramen and infra-alveolar canal was 16 mm. All the necessary preoperative hematological investigations were carried out before implant placement. Implant surgery was planned for the placement of one piece immediate loading implant (TRX-OP) with of diameter 2.8 mm and length of 13 mm.\nA mid crestal incision was given using number 11 blade. Full-thickness bucco-lingual mucoperiosteal flaps were elevated to expose the alveolar bone using molt periosteal elevator number 9. Preparation of osteotomy site was done according to the manufacturer's instructions. Drilling was done at a speed of 1000–1500 rpm with normal saline irrigation. An initial indentation at the center of alveolar crest was made using a round bur, followed by a 2-mm pilot drill up to the desired length. Further sequential drilling was done up to the pre-determined length. After each sequence of bone drilling, the paralleling tool was used to check the angulation of the prepared osteotomy site. Then, the implant of desired dimension (diameter of 2.8 mm and length of 13 mm) was placed with the hand wrench into the prepared osteotomy site. Torque on the hand wrench was adjusted to 40 N cm. After implant insertion, suturing was done with 4-0 black silk sutures to achieve primary closure []. Post-insertion clinical examination revealed no significant findings. An immediate post-insertion radiograph revealed desired direction of the implant []. Patient was discharged after thoroughly explaining the postoperative instructions.\nSeven days postoperatively, the patient reported to the department for checkup and suture removal. On removal of periodontal dressing, pale white bone devoid of gingiva was observed around the implant on the buccal aspect []. Clinical examination revealed a fibrotic thick gingiva around the exposed bone and on the lingual aspect. Also, the bone was devoid of periosteum and was showing changes toward necrosis. Lingually, a probing depth of 5–6 mm was noticed although the stability of the implant was maintained at the same level as that at the time of insertion. Radiographic examination revealed mild to moderate lytic changes in the bone around the implant [].\nOn taking history, the patient disclosed intake of alcohol for 1 day after implant placement.\nPatient was motivated for strict abstinence from alcohol until the healing period of implant. Open flap debridement of the affected site with excision of fibrous lateral pocket wall was carried out. Also, buccal osteoplasty was carried out to induce bleeding points on buccal surface of the alveolar bone. All the necessary precautions were taken care not to expose the implant bone contact area. The operated area was thoroughly debrided and irrigated with betadine and sutures were placed. The remaining exposed area was covered with sterile tin foil followed by periodontal pack application. Follow-up was done every 10 days for 9 months. Periodontal dressing was changed at every appointment after thorough irrigation of the operated site. Also, the patient was put on strict oral hygiene maintenance except for the operated site for which 0.2% chlorhexidene mouth wash twice daily was recommended.\nClinical examination at 20 weeks revealed almost 70% of the attachment gain around the implant with growth of healthy gingiva over the affected bone []. On radiographic assessment, an increased bone height was observed, more at the mesial aspect than distal.\nFollow-up at 40 weeks demonstrated complete healthy soft tissue coverage as well as normal radiographic features at the operated site. Also, radiograph revealed resolution of lytic changes with radiopaque flecks of bone around the implant on the mesial as well as distal aspects [Figures and ]. |
A 50-year-old Chinese man was initially admitted to our department for the sensorimotor dysfunction in left forearm, wrist, and hand. Before that, the patient had visited a clinic and been treated with oral neurotrophic drugs. Conservative treatment was ineffective, and the symptoms gradually worsened. Tracing his past history, we found that he was accustomed to resting in the left lateral position, with the left upper limb pressed under his body. After an episode of drunkenness 2 months ago, he rested in the left lateral position, with the left upper limb pressed under his head for more than 6 hours. After waking, he showed dysfunction when extending the left wrist and fingers, weakness of the left upper limb and inability to grasp.\nPhysical examination revealed that the fingers and wrist of his left hand were dropped and presented as a “claw-shaped hand” deformity. The left forearm flexor muscles group, the major and minor thenar, was characterized as muscle atrophy (Fig. ). The sensory function of the dorsal side of the left forearm, the major and minor thenar area and the tiger's mouth area was decreased. Moreover, the motorial function of the left thumb against the palm was absent. In addition, the muscle strength of the left upper limb was apparently decreased, the left brachioradialis muscle strength was grade 0, the ulnar wrist flexor muscle strength was grade 3, the wrist and finger flexor muscle strength were grade 4, and the wrist and finger extensor muscle strength was grade 0. The clipping paper test of the left thumb and index finger was positive, while the Hoffmann test was negative.\nElectromyography (EMG) and nerve conduction study (NCS) were rapidly performed after admission, indicating peripheral nerve injury of the left upper limb. EMG showed the fibrillation potential in the left short abductor muscle of the thumb at rest. The amplitude and latent period of the action potential were normal in light contraction, and presented as mixed phase in heavy contraction. NCS revealed the decreased amplitude of sensory nerve action potential in both the left radial nerve, median nerve and ulnar nerve, and sensory nerve conduction velocity slowed down apparently. Meanwhile, the cranium and cervical vertebra magnetic resonance imaging (MRI) was performed to exclude intracranial and cervical lesions, and the results were normal. Consequently, the patient was diagnosed with radial nerve, median nerve, and ulnar nerve injuries in the left upper arm caused by autogenous compression after drunkenness.\nAfter definite diagnosis, the patient underwent exploratory neurolysis surgery of the radial nerve, median nerve, and ulnar nerve in the left upper limb. Under brachial plexus block anesthesia, the exploration incision of the radial nerve, median nerve, and ulnar nerve in the left upper arm was selected respectively according to the injured site. In the operation, 3 nerves were observed to be whitened, and have edema and adhesion (Fig. ). The adhesions were released separately. Postoperative oral neurotrophic drugs were continued and the patient was guided to perform passive thumb and finger extension exercises. During 3 years of follow-up, the left upper limb sensorimotor function recovered well. The left brachioradialis muscle strength was grade 5, the ulnar wrist flexor muscle strength was grade 5, the wrist and finger flexor muscle strength were grade 4, and the wrist and finger extensor muscle strength was grade 4. Moreover, the motorial function of the left thumb against the palm was restored, and the clipping paper test of the left thumb and index finger was negative. |
A 51-year-old man with liver cirrhosis was admitted to our hospital due to loss of appetite, recurrent abdominal distension for 1 year, and hematemesis and melena for 3 months. Upper endoscopy showed variceal veins in the fundus of the stomach, and 4 variceal veins 25 cm from the incisor tooth that extended down to the cardia (Fig. ). Polyglycol and tissue glue were injected intravenously, and endoscopic variceal ligation was performed (Fig. ). During the ligation procedure, the patient developed significant bleeding when the seventh elastic ring came loose.\nAn SB tube was inserted in an attempt to stop the bleeding; however, placement of the tube was difficult due to the 6 elastic rings that had already been placed. The patient soon developed dyspnea and subcutaneous emphysema, and the SB tube was withdrawn. The patient became hypotensive and tachycardia with a blood pressure of 80/60 mm Hg and a heart rate of 104 beats/min. An emergency splenectomy and pericardial devascularization was performed under general endotracheal anesthesia. The operation was successful, and the patient was transferred intubated to the intensive care unit. The cardiothoracic surgery team was consulted regarding the patient's subcutaneous emphysema, and a large-bore needle was used to puncture the tissues and vent the gas. However, the subcutaneous emphysema worsened with the use of positive pressure ventilation, developing on both sides of the patient's neck and chest wall. The needle puncture was again performed, but the improvement was minimal. Therefore, a subcutaneous indwelling catheter was placed to relieve the gas accumulation.\nComputed tomography revealed that gas had accumulated on both sides of the neck as well as the skull base, mediastinum, abdominal cavity, scrotum, and beneath the thoracic and abdominal wall (Fig. ). At this point, it was suspected that the subcutaneous emphysema was due to a tracheal injury and possibly an esophageal injury. Fiberoptic bronchoscopy revealed an inward protrusion of the trachea that appeared during expiration and disappeared during inspiration. This protrusion resulted in severe airway obstruction, but no tracheoesophageal fistula was visible (see Video, Supplemental Video, which demonstrates the protrusion of the trachea that appeared during expiration and disappeared during inspiration, resulting in severe airway obstruction). Upper endoscopy indicated no obvious bleeding and no visible esophageal fistula. Fiberoptic bronchoscopy was repeated to adjust the position of the endotracheal tube to relieve the airway obstruction.\nDuring the following weeks, the patient developed a severe productive cough. We adjusted the position of the endotracheal tube multiple times to reduce the airway obstruction. However, after a multidisciplinary consultation, the decision was made to place an 8-cm × 1.8-cm-covered tracheal stent by fiberoptic bronchoscopy (Fig. ). Once the stent was placed and the patient's vital signs had stabilized, the patient was extubated, given oxygen through a nasal cannula, and discharged from the intensive care unit to a regular ward. A sputum culture showed a Staphylococcus aureus infection that was sensitive to rinathiazoleamine and vancomycin, and the patient was started on rinathiazoleamine. The patient ultimately recovered and was discharged on hospital day 26.\nFour weeks later, the patient was rehospitalized with throat pain and hemoptysis. Laryngoscopy was performed at a local hospital, which revealed a metal stent in the hypolarynx that prevented closure of the glottis. A computed tomography showed a small amount of gas around the trachea (Fig. ). The patient was transferred to our hospital, the tracheal stent was removed (Fig. ), and the patient was discharged 2 days later.\nTo date, the patient has been followed up for 10 months since his first admission in our hospital. Recently, he has no hematemesis or melena. Furthermore, no respiratory discomfort has been shown after removing the stent. |
A 74-year-old male patient underwent left total knee arthroplasty (TKA) at our institution for degenerative knee osteoarthrosis. He had an uneventful postoperative course with no history of delayed wound healing or persistent drainage. Four years later, he presented with a 2-month history of a gradually developing painless swelling over the anterior aspect of the operated knee; the swelling was associated with a small sinus that was extruding a straw-coloured fluid. He had no history of fever, decreased appetite, or weight loss. He had no other musculoskeletal, respiratory, or systemic symptoms of note. He had no history of antecedent trauma, recent travel, or contact with infectious diseases. The patient is a known hypertensive, but the blood pressure was well controlled with treatment, and he is otherwise healthy. He is a retired teacher with no history of involvement in activities requiring excessive kneeling. He is ambulatory in his community and can walk comfortably with the assistance of a cane.\nThe patient's general physical examination results were within normal limits; positive physical findings were limited to the involved knee. There was an anterior knee swelling involving mainly the prepatellar area, approximately 7 cm in diameter, fluctuant, and not tender to palpation, with minimal surrounding erythema; the erythema was present mainly at the punctum. The punctum was draining a yellowish discharge on pressure (). There was no bony tenderness at the patella, distal femur, or proximal tibia. There was no detectable knee effusion, instability, or crepitus. The range of motion was well preserved (5–110°), as it was a prosthetic knee. It was only painful at the end of flexion as this movement compressed the prepatellar bursa.\nPlain radiographs of the knee showed a prepatellar soft tissue swelling (). There were no obvious bony changes, osteolysis, or loosening at the bone-prosthesis interface. Needle aspiration of the prepatellar bursa yielded 50 mL of slightly turbid straw-coloured yellowish fluid that was sent as per our protocol for cell count determination, microcrystal analysis, Gram staining, and Ziehl–Neelsen (ZN) staining for acid-fast bacilli (). Cultures for aerobic and anaerobic bacteria, Mycobacterium tuberculosis, Brucella, and fungi were requested.\nConsidering the patient's age, chronic presentation, and the presence of sinus, he was admitted with a diagnosis of infected prepatellar bursitis. The primary aims of admission were wound dressing, awaiting aspiration results, and further work-up. Aspiration results revealed normal cell counts, no crystals, and no organisms on Gram stain. To our surprise, ZN stain revealed acid-fast bacilli consistent with typical tuberculous infection; the bacterium was confirmed on culture 6 weeks later as Mycobacterium tuberculosis-sensitive to rifampicin, isoniazid, ethambutol, and streptomycin. Cultures for bacteria, Brucella, and fungi were negative. Aspiration was repeated and yielded similar results. The blood work-up showed normal total and differential white blood cell count and slight elevation of both erythrocyte sedimentation rate (ESR) (80 mm/hr; normal: 30–70 mm/hr) and C-reactive protein (CRP) (8 mg/L; normal: 0–4 mg/L). Tuberculin skin test revealed a negative result (<5 mm induration at 72 hrs), and further work-up including chest radiograph and echocardiogram revealed no evidence of systemic disease. A triple three-phase bone scan displayed normal uptake both at the bone-prosthesis interface and at the patella. An infectious disease consult was obtained. The patient was started on rifampicin (600 mg/day), isoniazid (300 mg/day), pyrazinamide (1500 mg/day), and ethambutol (800 mg/day) for 2 months and continued on the same dosage of rifampicin and isoniazid to complete a 6-month course.\nThree weeks later, the swelling significantly subsided in size and the sinus healed; therefore, the patient was discharged. He was reviewed at 6-week intervals at both the orthopaedic and infectious disease clinics for clinical progression and for any side effects of the medication. At the most recent 6-year follow-up, he was doing well with no evidence of local recurrence or prosthetic loosening (). |
A 25-year-old female with no known co-morbidities or history of previous illness or trauma presented to the neurosurgery outpatient department. According to her, she was well three months back, when she suddenly developed episodes of severe left frontal headache which were continuous for hours to days. It was throbbing in character not associated with symptoms of nausea, vomiting, dizziness or fits. At many occasions, she complained of double vision with severe headache. For the same length of time, she also noticed that her left eye was bulging outwards along with pulsation in the same protruded eye which gradually increased and since one month it had been so obvious that anyone looking at her could appreciate it.\nHer general physical examination was unremarkable. She was conscious and well oriented to time, place and person. Her higher mental functions, gross motor, fine motor and sensory functions were intact. On local examination of the left eye, there was non-axial pulsatile proptosis with outward depression of the left eyeball. On visual acuity, she was assessed to be emmetropic with diplopia in her left eye. Ocular movements in both eyes were normal. There was no tenderness on palpation but pulsations were felt on left eye. No bruit was heard on auscultation.\nComputed tomography (CT) scan of the brain with contrast study showed deficient bone on left orbital roof and floor with left frontal gliotic brain compressing the eyeball causing it to move out and down (Figure ). Her magnetic resonance imaging (MRI) brain and orbit with contrast study showed an asymmetrical deformity of the skull and left cerebral hemisphere which was bulging towards the left orbit, causing extrinsic pressure resulting in proptosis of the eyeball (Figure ). It also showed widened cortical sulci due to decreased volume. There was indirect evidence of widening of left lateral ventricle and Sylvian fissure due to hemi-atrophic changes. Contrast studies did not elucidate any abnormal enhancement. Right orbit, right hemisphere, brainstem, and cerebellum appeared normal. There was no evidence of demyelination or any intracranial mass. The affected orbit was larger and downwards than normal. There was an absence of floor and roof of the left orbit.\nWith this information, a working diagnosis of orbital meningoencephalocele was made and a maxillofacial consult was taken. The patient and her family were counseled regarding the condition and about our plan of surgical intervention that included a two-staged procedure. Left frontal craniotomy was done as a part of the first stage. Per-operatively after opening the dura, frontal lobe was retracted without violation of the arachnoid. The retraction assisted in determining the bony defect. Then a titanium mesh was used to reconstruct the orbital roof and was fixed with screws over the inner surface of superior orbital rim. Duroplasty was performed and the frontal bone flap was secured. A subgaleal drain was then placed and the wound was closed in layers. The drain was removed at the first post-operative day and she was mobilized and discharged on third post-operative day. Her post-operative CT scan with three-dimensional (3-D) reconstruction confirmed the appropriate placement of metallic prosthesis covering the bony defect in the roof of the left orbit (Figures -).\nAt her two-week follow-up, her diplopia and headache had considerably improved and the proptosis had resolved with no visible pulsations. She is planned for second stage surgery after three months in which we plan to reconstruct the orbital floor. |
A 2.8-kg-one-day old female baby was brought to the hospital with respiratory distress and multiple growth protruding from oral cavity []. The child had macrostomia, cleft palate, bifid tongue, and two oral growths one measuring approximately 25 × 20 × 15 mm attached to hard palate and other in the sublingual region measuring approximately 12 × 10 × 15 mm. Computed tomography (CT) finding showed large well defined cystic lesion of size 23 × 17 × 12 mm in left parasagittal palatal region with internal fat fluid density and calcified densities (Hypertrophied palatal bones). Similar small cystic lesion of size approximate 11 × 8 × 13 mm is seen within oral cavity at sublingual region. On examination, the child was vigorous, tachypneic, and there was flaring of ala nasae. Mallampati grading could not be assessed.\nExcision of the tumor was scheduled on the day 3rd of life of the baby.\nOn arrival in operating room, routine monitoring (electrocardiogram, pulse oximeter, noninvasive blood pressure, and temperature) were attached. Difficult intubation cart and pediatric bronchoscope were kept ready to encounter any difficulty in intubation, and even surgical team were also standby to perform emergency tracheostomy if required. Oxymetazoline nasal drop was put in both nostril and patency of nare was checked by nasogastric feeding tube to rule out any extension of mass. Injection atropine 0.01 mg/kg was given through already secured intravenous access to limit secretions. For preoxygenation, a bigger size transparent circular silicon face mask was selected along with surgical pads to form a seal around the nose, lower jaw, and the mass to ensure proper bag and mask ventilation without causing any trauma to mass. Preoxygenation was done for 5 min. Once it was established that mask ventilation was possible, anesthesia was induced by sevoflurane and oxygen in gradually increasing concentration. When the patient was anesthetized, the mass was covered with surgical pads to avoid any injury to it and a gentle check laryngoscopy was done to visualize the glottis with Miller's blade 1.5; as glottis was visible, injection succinylcholine 1.5 mg/kg was given. After adequately lubricating the endotracheal tube (ET) with 2% lignocaine jelly, right nasal intubation was done with 3 mm internal diameter uncuffed ET by using Magill's forceps. Tube was fixed on the anterior surface of the nose. Injection fentanyl 2 μg/kg and injection atracurium 0.5 mg/kg were given. Anesthesia was maintained with oxygen, nitrous oxide, and sevoflurane. Pharyngeal packing was done by the surgeon before performing procedure. Sublingual mass was excised completely while mass at hard palate could be excised in piecemeal []. There was not significant blood loss and hemostasis was done. The duration of surgery was 40 min. At the end of surgery, neuromuscular blockade was reversed by injection neostigmine 0.07 mg/kg and injection glycopyrrolate 0.02 mg/kg and extubation could be done smoothly []. Patient was shifted to postoperative care unit. Nasogastric feeding was started on 2nd day and on fourth postoperative day, oral feeding was started and patient was discharged on fifth postoperative day. |
Case #1 - Twelve-year-old Caucasian boy sustained a traumatic dog bite to the right elbow by a large stray dog and was brought to an outside hospital. X-rays were performed and reported no fracture, dislocation, or intra-articular air ().\nThe elbow wounds were irrigated with normal saline and bathed in a normal saline/betadine mixed solution for 15 min at this outside facility. The patient was given Augmentin as well as rabies immunoglobulin and vaccine due to the unknown canine vaccination history. He was then transferred to our tertiary children’s hospital for further orthopedic surgery evaluation. Evaluation in the emergency department revealed five 1 cm or smaller lacerations over the lateral elbow and another 1 cm laceration to the volar and proximal forearm, just distal to the antecubital fossa ().\nThe patient had near full range of motion, falling about 10 degrees shy of full extension and 10 degrees short of full supination, limited by pain. He was also found to be grossly neurovascularly intact of the median, radial, and ulnar nerves to motor and sensation with strong radial and ulnar pulses. A SLT was performed through the posterior arthroscopic portal site into the olecranon fossa []. Fluid was seen extravasating from the lateral elbow wounds once 20cc of normal saline was injected into the joint, thus a positive test. He was admitted overnight for continued antibiotics (Augmentin 875–125 mg every 8 h) and surgical arthroscopic irrigation and debridement the next morning. For the procedure, the patient was positioned supine with the elbow across the chest. The posterior arthroscopic portal was established as well as the anterolateral arthroscopic portal with 2.7 mm cannulas []. Three liters of normal saline irrigation were run through the joint and all the wounds with gentle curettage debridement. The wounds were then loosely closed, and a sterile dressing with a long arm splint was applied without a drain to allow soft tissue rest. He was discharged with Augmentin 400/57mg/5 mL BID prophylactically for 7 days. The patient followed up in the office 1-week and 4-week post-operatively. He was without any erythema, drainage, or signs of infection; however, mild elbow stiffness in extension had improved.\nCase #2 - Seven-year-old African American boy was in a bicycle accident and sustained a large laceration over the proximal ulna/posterior elbow. Evaluation in the emergency department revealed an approximately 15 by 4 cm “U” like laceration with a large flap over the posterior elbow/proximal ulna ().\nSubcutaneous tissue, muscle, and triceps tendon were visualized, however, no obvious joint capsule violation. The patient had near full range of motion, about 10 degrees shy of full flexion and extension limited by pain. He was grossly neurovascularly intact of the median, radial, and ulnar nerves to motor and sensation with strong radial and ulnar pulses. X-ray revealed posterior elbow soft tissue injury and no intra-articular air ().\nA small bony fragment was displaced from the posterior aspect of the proximal ulna. A SLT was performed through the anterolateral “soft spot” arthroscopic portal site4, and fluid was seen extravasating from the wound after 30cc of normal saline was injected ().\nHe was admitted overnight for continued antibiotics (IV Ancef 40 mg/mL every 8 h) and surgical arthroscopic irrigation and debridement the next morning. For the procedure, the patient was positioned supine with the elbow across the chest. The posterior arthroscopic portal was established as well as the anterolateral arthroscopic portal with 2.7 mm cannulas []. Three liters of normal saline irrigation were run through the joint and the wound with gentle curettage debridement. The small bony fragment was deemed non-viable and excised. The wound was then loosely closed, and a sterile dressing with a long arm splint was applied without a drain to allow soft tissue rest. He was discharged home with Cephalexin 500 mg BID prophylactically for 7 days. The patient followed up in the office 1-week and 4-week post-operatively without any erythema, drainage, or signs of infection and had near full elbow range of motion. |
A 50 year old female patient previously healthy has undergone an abdominal ultrasound demanded by her primary care physician when her routine checkup blood test showed a slightly elevated level of liver enzymes with no other lab abnormalities. This ultrasound showed right adrenal lesion of 9 cm of diameter. An Abdominal MRI was then done and revealed a soft tissue necrotic encapsulated mass of 10 × 9 cm of right adrenal gland origin. She was completely asymptomatic and the physical exam was strictly normal. Endocrinological evaluation was done with normal hypothalamic-pituitary-adrenal axis function and no hyper secretion of catecholamines. The patient was considered to have a non-secreting right adrenal mass for which an adrenal scan was done and showed a well encapsulated 10 × 9 × 7 cm heterogeneous right adrenal mass with areas of necrosis and calcifications without local invasion (). The decision of right laparoscopic trans peritoneal adrenalectomy was taken with the patient. The surgery was done under general anesthesia after central and arterial lines insertion, the patient was on left decubitus position. 5 trocars were inserted as follows: The first 12-mm port was inserted at the lateral border of the rectus abdominis muscle just above the level of the umbilicus to accommodate the camera. Two subcostal 11 mm ports were also placed; one in the midclavicular line and the other in the lateral border of the rectus abdominis muscle. The forth 5-mm subcostal trocar was inserted in the anterior axillary line to retract the liver and the fifth 5 mm one was inserted in the epigastrium and used specially for aspiration and irrigation. After liver retraction, the peritoneum along the lateral aspect of the IVC was incised to expose the IVC just below its intrahepatic course. The duodenum which was diverted by the mass was mobilized. Dissection was next carried inferiorly by incising the peritoneum along the lateral edge of the vena cava to the superior edge of the renal vein. Dissection of the mass was subsequently carried out with special care at the medial aspect where we found that the wall of the IVC and the renal vein were very adherent to the mass which had a lot of small vessels that were oozing during all the time of the surgery (). In addition, the mass was extended posterior to the vena cava and we could not do a medial retraction of the IVC since the tumor was adherent to it. In front of these facts, we decided to convert to open surgery by a sub-costal incision (between two trocars) that allowed us to remove the mass safely (). A drain was put in the retro peritoneum at the end of the surgery, the operative time was 200 min, the blood loss was 850 cc and no transfusion was done. The drain was removed at the second post-operative day and the patient was discharged uneventfully on the sixth day after surgery.\nHistologically, the tumor consisted of spindle cells with alternating areas of compact hypercellularity with irregular streams and without atypia or mitosis (). This tumor was completely compressing and reducing the adrenal gland that was laminated but intact without histological abnormalities (). Immunohistochemical analysis demonstrated negative CKAE1-AE3, synaptophysine and chromogranine. In contrast to these results, S-100 and CD68 (PGM1) staining were diffusely positive across the tumor (). Thus, the evidences corresponded to a benign schwannoma (). |
A 40-year-old Asian-Indian male presented to our clinic with the complaints of sudden onset diplopia of 5 days duration associated with headache and vomiting. He was a known diabetic and was on oral hypoglycemic agents. On examination, his best-corrected visual acuity was OU 6/6, N6. On further examination, it was evident that he had a left pupil sparing oculomotor nerve palsy and loss of corneal sensations along with a firm mass palpable in the left temporal fossa. On questioning, patient gave history of presence of “painless progressively increasing bump” on his left temple for 35 years []. Magnetic resonance imaging (MRI) brain revealed a heterogeneously enhancing mixed intensity dumb-bell shaped lesion measuring 5.64 × 3.12 cm, with fat-fluid level noticed in the left orbit, along the superolateral aspect with the medial end extending up to the superior orbital fissure and anterior cavernous sinus. The lateral end of the cyst was extending into the infratemporal fossa []. In the anterior cavernous sinus, the lesion was compressing the oculomotor and the ophthalmic division of the trigeminal nerve. On the basis of the findings, a clinico-radiological diagnosis of frontotemporal intraosseous dermoid cyst was made.\nPatient underwent total excision of the dermoid cyst using a left infratemporal extradural approach. A formal temporal craniotomy was not performed; instead the lesion was approached through the left temporal bony defect due to erosion caused by the tumor itself. After excising the superficial part of the lesion with capsule, the microscope was used to further navigate and drill the bone to excise the lesion in toto. Under the microscope, left sphenoid bone was drilled up to the superior orbital fissure. The dermoid was extending to the left superior orbital fissure and reaching the left anterior cavernous sinus region where it was compressing the nerves in the lateral wall of the sinus. The dermoid cyst was soft yellowish with semi-solid cheese-like consistency with a thin-walled capsule. The capsule was radically excised and also scrapped off along with the periosteal layer of the bone. In the immediate postoperative period, his ophthalmoplegia and ptosis started improving.\nTwo months postoperatively, patient recovered well with marked improvement in diplopia and ptosis [] Follow-up MRI study showed postoperative changes with granulation tissue at the lateral wall of the cavernous sinus wall, without evidence of any residual mass [].\nFrontotemporal dermoids are the commonest group in the orbitofacial region accounting for 64% of cases in one of the largest series.[] They usually present as discrete slow growing masses, located superficially with no deep extension and can be managed by direct excision biopsy via an upper eyelid incision.[] Deep orbital dermoids may occasionally extend through the lateral orbital wall to secondarily involve the temporal fossa, sometimes in a “dumb-bell” or “collar-stud” pattern. In these cases, the intraorbital component of the dermoid usually predominates relative to the extraorbital component, and ophthalmic manifestations such as proptosis or ocular motility disturbances or both typically are present. Intracranial dermoid cysts typically increase in volume slowly over time and tend to grow in intracranial subarachnoid spaces that offer minimal resistance, such as cisterns, sulci or fissures.[] The growth rate of the dermoid cyst significantly affects the clinical presentation. Primary cavernous region dermoids present with oculomotor nerve palsy early in the course of the disease.[]\nHowever, our patient had the temporal swelling for many years preceding the onset of oculomotor paresis and is therefore a case of primary temporal dermoid extending to the orbit, superior orbital fissure and then anterior cavernous sinus; with MRI revealing the characteristic dumb-bell shaped tumor entering the orbit through a small bony window []. In our patient, the oculomotor nerve and the ophthalmic division of the trigeminal nerve were involved and symptoms were present only for 5 days prior to presentation suggestive of progressive growth and enlargement of a primary temporal dermoid. To the best of our knowledge this unique presentation of primary temporal dermoid has not been described so far in the literature and reiterates the continuous slow growing nature of the tumor.\nMagnetic resonance imaging permits the recognition of these lesions with their typical appearance as hyperintense signals on T1-weighted MRI and hypointense to mixed signals on T2-weighted MRI. Absence of peripheral edema due to slow growth with signal characteristics on T1-weighted and T2-weigted sequences helps in the differential diagnosis of dermoids from cystic glioma, arachnoid cysts or other lesions.[]\nExcision of temporal dermoids can be complicated by factors such as thin capsular wall, liquefied cyst contents, large size, rupture or leakage, adhesions to surrounding tissues, and variable involvement of bone with potential extension to the intraorbital or intracranial compartments. Where the cyst is attached to the bone, careful subperiosteal dissection is prudent as intraoperative rupture is common at these sites.[] If a portion of the dermoid is found to extend through a small bony aperture with pronounced expansion within the intraorbital or intracranial cavity, like in our patient, it is necessary to facilitate exposure by enlarging the bony opening to allow continuing dissection from the original approach. This was performed in our patient. Some surgeons prefer to open the second compartment by a formal lateral orbitotomy or craniotomy. |
An 84-year old male presented with shortness of breath during ordinary daily physical activity {New York Heart Association (NYHA) functional classification class III}. He also experienced syncope 2 months prior to admission to our hospital and intermittent chest pain for 2 weeks leading up to admission. The patient had a past medical history of hypertension and chronic obstructive pulmonary disease. He had been diagnosed with moderate AS 4 years ago and followed up regularly with checkups in the outpatient clinic, and the patient had been prescribed irbesartan, amlodipine, hydrochlorothiazide, atorvastatin. On admission his blood pressure and pulse rate were 150/70 mm Hg and 78/min, while body weight and height were 53 kg and 172 cm. The 12-lead electrocardiogram (ECG) on admission showed sinus rhythm with intermittent premature atrial complexes and findings suggestive of left ventricular hypertrophy with strain signs on lateral leads. Cardiomegaly was noted on the chest X-ray. Transthoracic and transesophageal echocardiography revealed severe degenerative change of aortic valve with heavy calcification (). The aortic valve area was 0.76 cm2 with a mean systolic pressure gradient of 43 mm Hg and peak systolic pressure gradient of 79 mm Hg compatible with severe AS. The aorta diameter was 21 mm at the aortic annulus, 32 mm at the sinus of valsalva, and 36 mm in the ascending aorta. Left ventricular end diastolic diameter was 52 mm and ejection fraction was 67%. There was mild pulmonary hypertension with an estimated right ventricular systolic pressure of 38 mm Hg. Coronary angiography showed mild coronary artery disease with 50% stenosis at the mid left anterior descending segment. Computed tomography showed diffuse atherosclerotic change of aorta and both external iliac arteries with calcification. The minimal lumen diameter of right and left external iliac arteries was 6 mm and 7 mm, respectively. A pulmonary function test revealed mild obstructive airway disease. The logistic Euroscore was calculated to be 36.1%.\nThe TAVI procedure was performed under general anesthesia. The vascular access for CoreValve delivery catheter was obtained at the right common femoral artery (CFA) with standard percutaneous access techniques. An 18 Fr introducer sheath was inserted through the right CFA after preparing percutaneous closure using two pre-loaded suture devices (Perclose Proglide, Abbott Vascular, Abbott Park, IL, USA). A pig tail catheter was inserted through the left CFA and positioned at the aortic root for the aortography during the procedure. A temporary pacemaker was placed in the right ventricle via the left femoral vein. A 0.035 inch Amplatz Super Stiff wire (Boston Scientific, Natick, MA, USA) was inserted into the left ventricle through the 18 Fr sheath. Balloon dilation of the stenotic aortic valve was performed with a balloon (diameter 25 mm, length 40 mm, Z-med, NuMED Inc., Hopkinton, NY, USA) under rapid pacing using a temporary pacemaker. Then, a 29 mm CoreValve was deployed at the aortic annulus under angiographic guidance (). An immediate post-procedural aortogram showed good position of the CoreValve with mild aortic regurgitation (). Post-procedural transthoracic echocardiography demonstrated a well-functioning CoreValve with a mean systolic pressure gradient of 7 mm Hg and peak systolic pressure gradient of 16 mm Hg (). The right ventricular systolic pressure decreased from 38 to 31 mm Hg, accompanied by moderate paravalvular aortic regurgitation (grade II/IV). After the procedure, the patient was observed in an intensive care unit for 72 hours with continuous ECG monitoring and temporary pacemaker back-up. No significant conduction abnormalities were observed except for transient nonspecific intraventricular conduction delay with incomplete left posterior hemifascicular block. The patient's symptoms subsequently improved from NYHA class III to class I and was discharged from the hospital at day 8 post procedure without any significant complication. A 3-month follow-up echocardiography revealed no significant interval change from the immediate post procedure echocardiographic findings. The patient remained free from any symptom or any major cardiovascular event for a total follow-up period of 6 months. |
We report a case of a 40-year-old Hispanic female who was evaluated in the emergency room (ER) for worsening abdominal pain and distension. She had been in her usual state of health until 2 months before this admission. She was initially evaluated by her primary care physician (PCP) for abdominal distension. The initial workup form PCP office showed mild anemia, hepatomegaly secondary to fatty infiltration and ascites on ultrasound of abdomen. She was referred to the outpatient gastroenterology clinic by PCP; however patient presented to ER due to worsening abdominal pain.\nOn admission to this hospital, she reported that her abdominal pain and distension have been worsening for the past 3 weeks. She described the pain to be sharp, diffuse, non-radiating, rated 10 on a scale of 0 to 10, with 10 indicating the most severe pain. There was no nausea, vomiting, diarrhea, constipation, weight loss or change in appetite. She had regular bowel movements but had noticed several episodes of blood while wiping herself a few weeks ago. Two weeks before this presentation she had dysuria, which had resolved spontaneously in 1 day. However, she did not have flank pain, foul-smelling urine, vaginal discharge or fever during that episode. She had not traveled outside the United States (US) recently and had not contacted any sick person. Her menstrual periods occurred in regular 30-day cycles, and her last menstrual period was 1 week before this presentation.\nShe had a history of beta thalassemia trait, iron deficiency anemia. She underwent tubal ligation 13 years ago. She had no known drug allergies. She was an active smoker with a 10-pack-year history of smoking. She consumed alcohol socially and reported using marijuana on a regular basis. Her active medications include omeprazole, acetaminophen, naproxen (as needed), and iron supplements. She was born and raised in the US. She lived with her husband and was sexually active. Her father had hypertension, and her aunt had breast cancer.\nOn initial evaluation, the temperature was 36.9 °C; the heart rate was 83 beats per minute, the blood pressure 111/59 mm Hg, the respiratory rate was 14 breaths per minute, and she was saturating 100% on room air. She was in mild distress due to pain and distension of abdomen. She was well developed and appeared in regular nutritional status. Abdominal examination revealed a distended abdomen with protruding umbilicus and positive shifting dullness suggestive of intra-abdominal fluid. Laboratory tests showed anemia, with hemoglobin of 11.3 g/dL. Her liver and renal function tests were within reasonable limits. Alpha1 anti-trypsin was marginally elevated (207 mg/dL). She was tested negative for viral hepatitis markers (). An echocardiogram was performed which did not show any signs of heart failure. Computed tomography (CT) of the abdomen with intravenous contrast revealed massive complex ascites with irregular nodular enhancement of the peritoneal wall (omental caking) suspicious of a malignant process (). She subsequently underwent CT-guided peritoneal tap which yielded 300 mL of serosanguinous fluid. Biochemical evaluation of the fluid showed serum ascites albumin gradient (SAAG) of 1.1 g/dL with a total protein of 5.2 g/dL pointing towards a differential diagnosis of carcinomatosis and tuberculosis (). Cytological examination of the fluid did not reveal any malignant cells. Considering lower yield of fluid cytology, patient was evaluated by the surgical team and underwent laparoscopic peritoneal biopsy which yielded epithelioid type malignant mesothelioma (-). The patient was referred to a tertiary care oncology center for further management, where chemotherapy with cisplatin and pemetrexed was initiated. The patient has been healthy on the follow-up visits and continues to follow there for continued care. |
A 21-year-old woman was consulted in February 2015 for bleeding gingival enlargement evolving for 12 months. She complained of esthetics, discomfort, and difficulties of plaque control. According to medical history, the patient had received a kidney transplantation 2 years earlier (2013). She has been administrating a daily immune suppressor treatment based on cyclosporin A 125 mg, prednisolone 5 mg, and mycophenolate mofetil 500 mg per day as a prophylaxis against organ transplant rejection.\nThe patient had a very poor oral plaque control; the plaque index PI [] and gingival index GI scores [] were high which were, respectively, 2 and 2.75.\nThe clinical examination revealed an erythematous, edematous gingival overgrowth localized at the buccal and lingual side of the anterior teeth. The gingival overgrowth appeared as localized nodular enlargement of the interdental papilla (Figures –).\nThe amount of the gingival overgrowth was obtained according to the GO score of Seymour et al. [].\nA GO score was assigned to each buccal and lingual interdental papilla (gingival unit) of the six anterior upper and lower teeth. Then the sum of the horizontal and the vertical enlargement components was made.\nThe first component measured the degree of gingival thickening (horizontal enlargement) labially and lingually by means of a three-point scale (0 = normal width, 1 = thickening up to 2 mm, and 2 = thickening of more than 2 mm). The second component measured the extent of encroachment (vertical enlargement) of the gingival tissues on the labial and lingual aspects of adjacent tooth crown; it ranged from 0 to 3 (from no clinical evidence of overgrowth to an overgrowth covering three-fourths of the tooth crown). Likewise, a total of 20 papillae are examined, presenting a potential maximum GO score of 100, which could be expressed as a percentage [].\nThe gingival overgrowth is considered as clinically significant if the GO score is ≥30% [].\nIn the present case report, the GO score was 30.5%, so that it was classified as clinically significant gingival overgrowth.\nA suitable probing revealed deep pockets with negative recessions, due to the gingival overgrowth (indicating coverage of clinical crowns ≥ 2 mm). Underlying calculus was localized mainly at the anterior teeth. The pocket values and clinical attachment loss varied from 5 to 7 mm and from 2 to 3 mm, respectively.\nX-ray examination showed a marginal (coronal third) horizontal alveolar bone loss which was more pronounced at the lower incisors (). So the patient had a periodontitis beside the gingival enlargement.\nThe final diagnosis was CsA-induced gingival overgrowth with underlying localized moderate periodontitis stage II grade B. The periodontitis was classified according to the new classification system of periodontal diseases and conditions from the American Academy of Periodontology and the European Federation of Periodontology 2018 [] (Tables and ).\nThe management strategy consisted of a nonsurgical periodontal therapy based, initially, on oral hygiene instruction. On the second-time round, a full-mouth scaling and root planning were performed a week later as well as polishing of all the rough dental surfaces. Extraction of the remaining root of tooth #26 was done at the same appointment.\nThe treatment was conducted under appropriate antibiotic prophylaxis based on amoxicillin plus clavulanic acid 1 g (intraoral) 2 times per day for 8 days as suggested by the patient's nephrologist. The antibiotic prophylaxis was performed in order to cover the infectious risk related to the systemic health status.\nTwo months after the periodontal treatment (hygienic phase), the clinical evaluation showed a successful regression of the inflammation and improvement of periodontal parameters. We have noted a reduction of pockets' depth and plaque and gingival index scores which become, respectively, PI: 0.5 and GI: 0.8.\nThus, a supportive therapy was established including the reinforcement of oral hygiene instruction and full-mouth scaling every 2 months. The whole treatment resulted in the total disappearance of gingival overgrowth without any surgical procedure. The last clinical and X-ray evaluation after 2 years of regular follow-up shows the good stability of the results (Figures –). |
Our patient is a 58-year-old African American woman who initially presented to an outside facility of difficulty swallowing, neck pain, fatigue, and shortness of breath for 3 months. She had bilateral neck swelling, greater on the left side, that started about a year prior to her presentation. This patient also had a 4-day history of productive cough and nasal drainage. She had a 30-pack year history and consumed approximately 12 beers daily for years.\nShe reported a history of CLL and having been treated with chemotherapy, but she was unsure of the regimen. Examination findings and computed tomography (CT) scan of neck at the outside facility were concerning for impending airway compromise, and she was transferred to our hospital to be evaluated by an otorhinolaryngology or oral and maxillofacial surgery service.\nDuring her evaluation in our emergency department, she was noted to have a muffled voice with mild gurgling of secretions with speech. Large, fixed cervical and submandibular lymph node, and a large left neck mass were present. Enlarged axillary lymph nodes were palpated bilaterally. The uvula and posterior oropharynx were not visible due to her tongue and left neck mass. White blood cell (WBC) count was 83 600/mm3. She was started on dexamethasone to reduce the compression of the neck mass on the airway. The oral and maxillofacial surgery service team evaluated her and determined that a definitive airway was not indicated. She was admitted to the medical intensive care unit for close airway monitoring.\nThe oncology team became involved at this time. Her CLL was the suspected etiology of the neck mass and lymphadenopathy, and intravenous fluids and allopurinol were started to prevent tumor lysis syndrome. Further laboratory workup showed the predominantly lymphocytic, elevated WBC count as before and a normocytic anemia, hemoglobin 10.6 g/dL. Smudge cells were present on the peripheral blood smear. Iron studies, B12, and folate were normal. Flow cytometry was pending ( and ).\nCT chest showed lymphadenopathy of the mediastinum, right hilum, bilateral axilla, submental, and supraclavicular lymph nodes. Consolidative and ground glass opacities were also present in the bilateral lung fields ().\nPartially confluent retroperitoneal, mesenteric, pelvic, and inguinal lymphadenopathy were noted on CT abdomen and pelvis. These findings were consistent with known CLL.\nIn addition to CLL, diagnoses considered included Richter transformation. We proceeded to obtain biopsies of the left neck mass.\nAround this time outside records were received. Our patient had been treated with rituximab and chlorambucil 5 months prior after presenting with similar symptoms. She had been incidentally found with a WBC count of 40 000/mm3 several years before. She was lost to follow-up after both occasions. We started her on rituximab for treating CLL with possible Richter transformation. The tentative plan was to treat with fludarabine, cyclophosphamide, rituximab (FCR) if CLL or rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP) if Richter transformation had occurred.\nFlow cytometry showed a CD5+ mature B-cell lymphoma consistent with CLL, 13q deletion, and mild CD38 positivity. Over the next few days, she had improvement in her neck pain. The mass and lymph nodes decreased in size. The WBC count decreased to 40 000/mm3. Biopsy of the neck mass showed squamous cell carcinoma (SCC) with extensive necrosis involving lymphoid tissue.\nWith evidence of a second malignancy with possible significant oropharyngeal involvement, direct laryngoscopy with biopsy was pursued ( and ).\nDirect laryngoscopy was performed and showed a large mass involving the right tonsil extending to midline of the posterior oropharynx. Biopsies were obtained. Posterior oropharynx, right tonsil, and right nasopharynx biopsy contained invasive keratinizing SCC, moderately differentiated, with basaloid features. The nasopharyngeal biopsy also included atypical lymphoid infiltrate consistent with involvement by CLL.\nThis patient’s case was discussed at the tumor board. Given the widespread involvement of her disease, she was diagnosed with Stage IV oropharyngeal SCC. She was not at candidate for resection and was to be treated with high-dose cisplatin (25 mg/m2) weekly for 6 cycles and radiotherapy. Treatment was to be delayed for her Rai Stage III CLL out of priority since her airway compromise was attributed to the SCC and the CLL component had been partially treated with rituximab.\nAt time of discharge, she had continued improvement of her symptoms. The plan was for her to follow with our medical oncology and radiation oncology teams as an outpatient. However, due to long distance from home, she established care at an outside facility. |
A 6-day-old (46 kg) male Belgian Blue-Holstein crossbred calf was submitted for clinical investigation because of ulcerations of the skin and nasal planum since short time after birth. The animal was delivered after a gestation period of 287 days.\nThe cutaneous lesions were characterized by widespread irregular erosions and ulcerations of various sizes on most parts of the body (Figure ), but in particular on the limbs (Figure ) and over bony prominences. Upon handling, the epidermis easily separated leaving a blister with a black colored, nonhemorrhagic base indicating a separation superficial to the stratum basale. Peracute blister were easily induced by rubbing the skin by an eraser after having cut the hair locally. Older lesions consisted of ulcerations covered by crusts and occasional acute hemorrhage. On the nasal platum, lips and nares extensive ulcerations were present; the calf also showed a purulent nasal discharge (Figure ). Moreover, the animal seemed to be in pain when walking on a hard surface. The aspect resembled EB and therefore was further referred to the Danish surveillance program for genetic diseases in cattle for further examination. Because of the poor prognosis and the painful situation, the calf was euthanized for welfare reasons by IV administration of an overdose of pentobarbital. In addition to the skin lesions, gross pathologic examination revealed erosions in the oral cavity, pharynx, proximal esophagus, and rumen. The epithelium on the dorsal surface of the tongue was thickened and with furrows (Figure ). The incisor teeth were disorganized and not completely erupted and the surrounding parts of the mandibles appeared thickened and cystic. The hoofs seemed intact, yet when sawed longitudinally, the capsule was partly separated from the dermal lamella with hemorrhage in the interface.\nImmediately before euthanasia, the skin covering the dorsal part of the pelvis was gently scrubbed with an eraser with blister formation. Skin biopsies from this area and from other representative cutaneous lesions were taken immediately after euthanasia for histological analysis, whereas the necropsy was completed at the university a few hours later. Additional specimens for histological analysis were then collected, including the oral mucosa, pharynx, rumen, reticulum, and major internal organs. All collected samples were fixed in 10% neutral buffered formalin, trimmed, processed, embedded in paraffin wax, sectioned at 4 to 5 μm, and stained by hematoxylin and eosin (HE). Histologically, the peracute lesions induced by rubbing before euthanasia displayed a very striking, multifocal to coalescing dermal-epidermal separation at the level of the basal layer, which extended into the wall of the hair follicle infundibula (Figure ). The spontaneously occurring, chronic lesions present in the nasal planum and in the distal limbs displayed a multifocal to coalescing epithelial loss with consequent severe ulceration and underlying neutrophilic infiltration, replacement of the papillary dermis by granulation tissue, and re-epithelialization. A multifocal dermal-epidermal separation at the level of the basal cell layer with multifocal underlying accumulation of free erythrocytes and fibrin exudation was occasionally visible at the border of the ulcerated areas.\nIn the tongue, the mucosa of the dorsal surface showed a marked parakeratotic hyperkeratosis. At the lateral borders, where the epithelium had a normal thickness, areas with complete loss of mucosa were observed. The superficial layer of the submucosal connective tissue had a necrotic surface, intense hyperemia, and infiltration with neutrophils. Mucosa cleavage in the adjacent areas was not observed, but the height of the epithelium gradually decreased. The stratum spinosum showed ballooning degeneration and in these areas, the stratum corneum was not present. In the pharyngeal lining, multiple intensely inflamed ulcers covered by a debris of fibrin, degenerated neutrophils, erythrocytes, bacterial colonies were present.\nThe epithelium of the rumen and reticulum was normally developed but an acute suppurative multifocal rumenitis was present. Separation of the epithelium from the underlying submucosa was seen in some areas but considered as a post mortem artifact. Additional findings included suppurative periportal hepatitis and multifocal intense pulmonary hyperemia associated with fibrin in the alveoli. Other tissues were unremarkable. The histopathological findings in the skin and pharyngeal linings resembled EBS.\nAdditionally, WGS using the Illumina NovaSeq6000 was performed on DNA extracted from ethylenediaminetetraacetic acid (EDTA) blood of the calf. The sequenced reads were mapped to the ARS-UCD1.2 reference genome resulting in an average read depth of approximately 19×, and single-nucleotide variants and small indel variants were called. The applied software and steps to process fastq files into binary alignment map and genomic variant call format files were in accordance with the 1000 Bull Genomes Project processing guidelines of run 7 (), except for the trimming, which was performed using fastp. Further preparation of the genomic data had been done according to Häfliger et al. In order to find private variants, we compared the genotypes of the affected calf with 493 cattle genomes of various breeds that had been sequenced in the course of other ongoing studies and that are publicly available (Table ) in the European Nucleotide Archive (SAMEA6528898 is the sample accession number of the case; ). Integrative Genomics Viewer (IGV) software was used for visual inspection of candidate variants. A total of 115 private protein-changing variants with a moderate or high predicted impact on the encoded protein, located within 108 different genes or loci, were identified. These variants were further checked for their occurrence in a global control cohort of 3103 genomes of a variety of breeds (1000 Bull Genomes Project run 7; ), which revealed 26 protein-changing variants exclusively present heterozygous in the genome of the EBS-affected calf. These 26 variants located within 25 different genes or loci (Table ) were subsequently visually inspected using IGV software confirming all as true variants. Of all these 26 remaining private variants, only 1 occurred in a candidate for EB: keratin 5 (KRT5). The variant was a heterozygous disruptive in-frame deletion variant on chromosome 5: 27367604delCAA (NM_001008663.1:c.534_536delCAA), leading to a loss of an asparagine amino acid at residue 178 of the encoded KRT5 protein (NP_001008663.1:p.Asn178del). This variant affecting an EB candidate gene was further investigated as likely causal mutation for the observed phenotype.\nTo confirm that the c.534_536delCAA variant in KRT5 was a de novo mutation, the affected genomic region was amplified by polymerase chain reaction (PCR) and Sanger sequenced in the affected calf, its Belgian Blue sire and Holstein dam based on DNA extracted from EDTA blood of the dam, and from both EDTA blood and semen of the sire. PCR products were amplified using flanking primers for the KRT5 exon 1 deletion with 5′-AGGCATCCAAGAGGTCACCG-3′ (forward primer) and 5′-TAGCACATATCCCACACTCATGG-3′ (reverse primer). Sequence data were analyzed using Sequencher 5.1 (GeneCodes). Analyzing the sequencing data, we concluded that only the EBS-affected calf was heterozygous for the KRT5 variant and the dam and sire were both homozygous for the wild type allele in all analyzed samples including both semen and blood of the sire (Figure ). |
A 41-year-old man had been diagnosed as having a middle esophageal diverticulum based on an upper gastrointestinal contrast examination performed when he was 30 years old. He had not received treatment because he was asymptomatic. Eight months earlier, he experienced chest discomfort after eating and visited our hospital. His past history is pediatric asthma. The diameter of his middle esophageal diverticulum was 47 mm, and the accumulation of contrast medium was observed in the diverticulum. A gastrointestinal endoscopy revealed a diverticulum in the right wall located 30 cm from the incisor row (Fig. ), and the diverticulum mucous membrane was partially covered by adherent white matter that was unstained with iodine (Fig. ), and narrowband imaging which revealed type A. We performed a biopsy, and the pathological findings of the endoscopic biopsy were atypical epithelium and no malignant findings. We confirmed the function of the lower esophageal sphincter, and the esophageal body peristaltic wave was observed to be normal using high-resolution manometry. We decided to perform a thoracoscopic diverticulectomy based on his symptoms and the possibility of malignancy suggested by the atypical epithelium. Surgery was performed with the patient placed in the prone position and four trocars inserted into the right thoracic cavity. A 12-mm trocar was inserted into the fifth intercostal space on the posterior axillary line. Only the left lung was ventilated, and a pneumothorax in the right chest was created using CO2 gas at 6 mm. Twelve-mm trocars were inserted into the seventh and ninth intercostal spaces at the level of the inferior scapular angle. A 5-mm trocar was then inserted into the seventh intercostal space on the posterior axillary line. The thoracoscope was inserted via the 12-mm port in the ninth intercostal space at the level of the inferior scapular angle. The operator used the 12-mm port in the seventh intercostal space on the inferior scapular angle line and the 5 mm port in the seventh intercostal space on the posterior axillary line. The assistant used the 12-mm port in the fifth intercostal space on the posterior axillary line. The endoscope was inserted into the lumen of the upper thoracic esophagus before the patient was placed in the prone position and kept in the esophagus throughout the surgery. Confirmation of the middle esophageal diverticulum was easily achieved, but the area was difficult to exfoliate because the diverticular wall had adhered to the lymph nodes of the trachea bifurcation. To achieve a complete resection of the diverticulum, threads were placed on the oral and anal sides of the diverticulum, the threads were pulled. A thoracoscope was inserted via the 12-mm port in the fifth intercostal space on the posterior axillary line and the surgical stapler was inserted via the 12-mm port placed in the ninth intercostal spaces at the level of the inferior scapular angle, and the diverticulum was resected using two sets of the Tri-Stapler (Fig. ). A postoperative upper gastrointestinal contrast examination revealed no abnormalities (Fig. ). Oral intake was initiated on postoperative day 7, and he was discharged on postoperative day 12. The histopathological findings were a true diverticulum with a muscular layer and a highly inflamed mucosa. No malignant findings were seen (Fig. ). Postoperatively, the patient visited the hospital at 1 month, 3 months, 6 months, and 1 year after the surgery. He underwent gastrointestinal endoscopy and upper gastrointestinal contrast examination 1 year after the surgery. We found no evidence of recurrence of the esophageal diverticulum. We are planning to repeat the upper gastrointestinal endoscopy at 2 years after the surgery. |
A 67-year-old Japanese man gave a history of being diagnosed with a cystic lesion in the right third molar region of the mandible by X-rays 11 years earlier. Subsequently, a tooth extraction had been performed. However, cyst enucleation and histopathological examination had not been carried out at that time. Eight years after the tooth extraction, he noticed a gingival swelling which lasted for three years. The medical history was only prostatic hypertrophy. On examination, a slight swelling was palpable in the gingiva of the right third molar region of the mandible. There was no fistula but a part of the bone had a defect. A panoramic radiograph revealed a radiolucent cystic lesion, measuring 10 × 12 mm in the same area (Fig. a, yellow arrows). A computed tomography (CT) showed an unilocular radiolucent lesion along with cortical bone resorption of the mandible on the lingual side (Fig. b). On the basis of clinical and radiological findings, a presumptive diagnosis of an odontogenic cyst was made and a biopsy was performed (Fig. c). The incisional biopsy resulted in a diagnosis of central MEC arising from a GOC as described below. Chest and abdominal CT findings were within normal limits. A magnetic resonance imaging (MRI) revealed a contrast defect in the same area (Fig. d). Cervical lymph node metastasis was absent on MRI. Due to the malignant nature of the tumor, as well as a history of previous surgeries, a partial mandibulectomy was performed to remove the lesion with a sufficient surgical margin and the tumor was surgically excised under general anesthesia. Following a final diagnosis of central MEC, the patient made an uneventful recovery and demonstrated no clinical evidence of recurrence in the two years following the surgery.\nMicroscopic examination of the biopsy material showed an enlarged unilocular cyst (Fig. a). The cystic lumen was lined by epithelial cells and was surrounded by thick fibrous connective tissue. Additionally, a solid polycystic lesion was also seen on one side (Fig. a, black arrows). Numerous microcysts and mucous goblet cells were observed in the lining epithelium (Fig. b). The intraepithelial mucin in the mucous goblet cells was positive for mucicarmine staining (Fig. c). Eosinophilic cuboidal cells (Fig. d) and ciliated cells (Fig. e) were scattered within the non-keratinized squamous epithelial cells. These histopathological findings were suggestive of a GOC. In addition to the cyst wall consisting of fibrous connective tissue and the above-mentioned non-keratinized squamous epithelium coating the fibrous stroma (Fig. a, black square and 2f), the proliferation of many cystic nests containing mucous materials was observed in another part of the cyst wall (Fig. a, yellow square and 2 g). The lining epithelium inside several cysts consisted of a mixture of epidermoid, mucous, and intermediate cells (Fig. g). These findings served to confirm the diagnosis of central MEC arising from a GOC.\nWe evaluated the cytokeratin (CK) profile by immunostaining to compare the CK expression patterns between central MEC and GOC in the biopsy specimen. The lining epithelium comprising non-keratinized squamous cells in the GOC part (Fig. f) showed immunopositivity for CK 7 (Fig. a), CK13 (Fig. c), CK14 (Fig. e), CK18 (Fig. g), and CK19 (Fig. i). On the other hand, the central MEC part was positively stained for CK7 (Fig. b), CK14 (Fig. f), CK18 (Fig. h), and CK19 (Fig. j), whereas immunoreactivity for CK13 was not detected (Fig. d). In the final surgical specimen obtained after mandibular partial resection, the tumor with several cystic spaces could be seen to expand into the submucosal area under the gingival mucosa from the mandibular bone in the cut surface (Fig. a). The resected specimen contained only central MEC (Fig. b). The keratin immunohistochemical profiles of CKs were similar to those of central MEC in the biopsy specimen, which was not positive for CK13 (Fig. c) but was positive for CK18 (Fig. d). The histopathological examination of the final surgical specimen confirmed the presence of central MEC arising from a previous GOC after consideration of the histopathological findings of the biopsy specimen.\nWe sought to clarify the relationship of GOC to central MEC by performing MAML-2 molecular analysis of the lesion. Break-apart fluorescence in situ hybridization (FISH) for MAML-2 was performed. The component of central MEC in the biopsy specimen exhibited the MAML-2 rearrangement (Fig. a). In cystic areas of the GOC, the MAML-2-split was also present (Fig. b). Additionally, MAML-2 rearrangement was also detected in central MEC of the final surgical specimen (Fig. c). |
A 65-year-old female presented to an outside institution with several weeks of headaches and acute worsening of progressive numbness in her right lower extremity. Her past medical history was only significant for basal cell carcinoma of the left nasal ala, resected 5 years prior. Her family history was notable for breast cancer of middle-aged onset in her sister, mother, and grandmother but no prior diagnosis of a familial cancer predisposition syndrome. Her symptoms prompted magnetic resonance imaging (MRI) of the brain, which revealed a predominantly non-enhancing right parietal lesion with patchy enhancing foci extending to the splenium of the corpus callosum (Fig. ). Further imaging with computed tomography (CT) of the chest, abdomen, and pelvis did not reveal a primary systemic tumor to suggest a diagnosis of brain metastasis. She subsequently underwent stereotactic biopsy of her lesion with pathology demonstrating a glial neoplasm, consistent with World Health Organization (WHO) grade II astrocytoma (Fig. ). The Ki-67 index was 1–3% and immunohistochemical staining for TP53 was positive. Further molecular testing was negative for the EGFR variant III, IDH1 mutation, and MGMT promoter methylation. She was subsequently referred to our institution for further adjuvant treatment, where she underwent adjuvant radiation therapy and temozolomide treatment, as well as use of tumor-treating fields (TTF) with the Optune device (Novocure, Jersey, U.K.). A brain MRI obtained several months after completion of chemoradiation and TTF showed radiographic response with stable small foci of enhancement and drastic reduction in perilesional edema (Fig. ). Clinically, the patient was stable with moderate cognitive deficits and requirement of mild assistance with activities of daily living.\nSix months after completion of chemotherapy, she presented with increasing gait instability of two weeks duration and worsening thoracic back pain, localized between her shoulder blades. MRI of her thoracic and lumbar spine was obtained, demonstrating a homogenously enhancing dural-based, extramedullary lesion anterior to the spinal cord at T4 with moderate cord compression, causing edema extending from T2 to T7 (Fig. ). She was neurologically intact on examination other than brisk lower extremity reflexes, allodynia at the T4 level, and baseline right-sided hemibody numbness. Imaging findings were consistent with spinal meningioma, and the patient was admitted with plans for elective, inpatient surgery. However, two days after admission, she developed acute weakness in her bilateral lower extremities and subsequently was taken to the operating room for urgent resection of her tumor, which was removed in its entirety. Intra-operatively, the tumor was noted to be dural-based with a well-defined plane between tumor and the dura as well as the pia of the spinal cord. Motor evoked and somatosensory evoked potentials were improved at the end of the case, compared to baseline levels. Final pathology from the resected specimen was WHO IV gliosarcoma with biphasic tissue architecture, comprised of prominent GFAP-positive glial regions admixed with GFAP-negative spindled areas containing reticulin fibers (Fig. ). The Ki-67 index was elevated >10–15% and further routine molecular testing demonstrated MGMT promoter methylation but was otherwise negative for IDH1 mutation and ATRX loss.\nThe patient was subsequently discharged to rehabilitation and exhibited some improvement in lower extremity strength, albeit remained non-ambulatory and required significant assistance with activities of daily living. Further treatment with adjuvant chemoradiation was considered but given the unfavorable prognosis from new gliosarcoma diagnosis and poor performance status, she elected to undergo hospice care without further imaging or treatment and subsequently expired one month after surgery. |
A 70-year-old female with no significant past medical history, Eastern Cooperative Oncology Group performance status 0 and no prior incidence of hypersensitivity reactions, was incidentally found to have a small nodule in the proximal right posterolateral vaginal wall after presenting with post-menopausal bleeding. Subsequent biopsy demonstrated a 9 mm invasive melanoma and the patient underwent wide local excision with confirmed negative margins. 4 months later, she developed a right periclitoral mass. Positron emission tomography/CT scan at that time demonstrated focal uptake in this area but no regional/distant metastases (). Excision demonstrated a large submucosal mass of atypical epithelioid cells with evidence of melanin synthesis, consistent with malignant melanoma. Breslow depth was 9 mm (3 mitoses/mm2) with a positive deep margin, and there was no evidence of lymphatic invasion. Her case was presented at a multidisciplinary tumour board and either additional surgery or radiation therapy was recommended to the patient. Owing to the significant morbidity anticipated with additional surgery, the patient opted for radiation therapy. Given the high risk of both local and regional/distant failure, concurrent chemotherapy was proposed. Owing to the historically poor response rates with standard chemotherapy, an immune pathway targeted agent was considered. This non-standard approach was actually initially proposed by the patient. After being thoroughly explained the current standard of care, in addition to the pros and cons of pursuing concurrent radiation and immunotherapy, the patient chose to proceed with combination immunotherapy and radiation treatment. Several studies have demonstrated that local radiotherapy primes and/or enhances an immune response through cytotoxic T lymphocytes. Concurrent immunotherapy may then further enhance the activity and/or duration of the downstream immune response. Given the historically low efficacy of our current treatment paradigms in this disease, as well as the preclinical/clinical rationale to combine radiation and immunotherapy, a strategy of pursuing a combination of ipilimumab with radiation was felt to be reasonable.\nRadiation was planned with intensity-modulated radiation therapy (IMRT) to the vulva and vagina (no elective nodal radiation to the groin) to an initial dose of 45 Gy (1.8 Gy/fraction over 25 fractions), and was to be followed by an electron boost to a total dose of 63 Gy (1.8 Gy/fraction over 10 fractions) at the site of the positive margin (). A thermoluminescent dosimeter was placed on the vulva at the start of treatment to measure the skin dose and read as 1.78 Gy [95% confidence interval (1.66–1.90)], confirming that the planned dose was accurate on the skin. The patient received her first cycle of ipilimumab (3 mg kg−1) 7 days after the start of radiation, and the second cycle was delivered 3 weeks later when the patient was at a dose of 36 Gy. Around this time (3 weeks post ipilimumab cycle 1), she began to develop non-painful erythema in the vulvar and perianal area, as well as a pruritic, grade 2 cutaneous eruption that morphologically showed distinct erythematous papules that coalesced into thin plaques over the upper arms, chest, back and face/ears (all toxicities were graded using Common Terminology Criteria for Adverse Events version 4.03). She did not experience any fevers or other systemic symptoms. By 48.6 Gy dose (10 days post ipilimumab cycle 2), the patient developed a grade 3 skin reaction () that was characterized as a moist desquamation with significant oedema, erythema and pain in the vaginal/vulvar/perianal region and was restricted to the radiation field (). A timeline of these events is illustrated in . After proper consent, a 4 mm punch biopsy of the affected skin was performed and histopathological examination demonstrated spongiotic and interface dermatitis with a perivascular inflammatory infiltrate consisting of numerous eosinophils, consistent with a fixed drug eruption.\nRadiation and ipilimumab were held given the severity of the moist desquamation, and the patient was referred to a dermatologist for evaluation of the ipilimumab-associated cutaneous eruption. It is noted that the patient had no existing risk factors or hypersensitivities predisposing her to an enhanced skin toxicity. The patient was started on 0.1% topical triamcinolone cream along with a methylprednisone dosepak. Given only mild improvement after 1 week, she was started on prednisone 60 mg daily (tapered over 7 days) with oral diphenhydramine for pruritus as needed. This resulted in significant improvement in her cutaneous eruption and pruritus. She then received a third cycle of ipilimumab (4 weeks after cycle 2) and resumed her periclitoral radiation boost without further issues after a 1-month break from radiation treatment. She went on to receive a fourth cycle of ipilimumab after completion of radiation without any complications.\nAt follow-up 8 months post completion of radiotherapy, she had complete resolution of the in-field toxicity and improvement of her ipilimumab-associated cutaneous eruption (). Clinical examination and positron emission tomography/CT imaging 10 months after completion demonstrated no evidence of disease recurrence. Most recently, at her 15-month follow-up, she remains disease- and symptom-free ().\nAt each follow-up visit, the patient has repeatedly expressed satisfaction with her decision to pursue immunotherapy rather than standard chemotherapy to complement her radiation treatment. She admits the initial side effects and intensity of the treatment course were difficult, although she is very pleased with the outcomes and would make the same decision if asked to do so again. |
A 50-year-old female was referred to the Department of Oral and Maxillofacial Surgery for treatment of TMJ ankylosis. Ankylosis began when the patient was 12 years old. The patient had experienced a fall, but she could not remember the time and the specifics of the associated trauma accurately. She had experienced otitis media in her right ear more than 20 years previously. Clinical examination revealed facial asymmetry and limitation of the mouth opening (2 mm). On a panoramic radiograph, bony fusion of the right TMJ, a prominent right antegonial notch, and severe deviation of the mandible to the right were noted, along with multiple dental caries, retained roots, and generalized severe alveolar bone loss. A well-defined outline of the condyle was noted in the left TMJ (). CT images revealed bony ankylosis with a large mass in the right TMJ area, the loss of air cells in the temporal squama and mastoid area, and a thickened right zygomatic arch. The normal relationship of the articular fossa and the condyle with the articular space was revealed in the left TMJ (). Magnetic resonance imaging (MRI) showed a large fused bone mass with normal bone marrow in the right TMJ () and flattening of the condyle with a thin disk in the left TMJ. Irregularities of the cortical bone of the articular fossa and the condyle were also detected in the left TMJ ().\nA three-dimensional (3D) rapid prototyping (RP) model was fabricated using the CT images. The right condyle was fused with the posterior portion of the zygomatic arch. Abnormal bone formation was detected in and around the TMJ. Anatomical structures of the TMJ were not found (). A narrowed articular space between the condylar head and the articular fossa was detected in the left TMJ ().\nRadiographically, the differential diagnoses for the right TMJ included benign bone tumors such as osteochondroma, ostoma, chondroma, chondroblastoma, benign osteoblastoma, fibro-osteoma, fibrous dysplasia, myxoma, and giant cell tumor. However, careful history taking and radiological and histological examination for this patient would be useful for the differential diagnosis. On the basis of the clinical and radiological examinations, the patient was diagnosed with bony ankylosis in the right TMJ and suspected adhesion in the left TMJ.\nSurgery was performed under general anesthesia using laryngoscopy with nasotracheal intubation. A preauricular incision with a temporal extension on the right side was made. In order to create an approximately 15 mm wide gap, excision of the bony mass was carried out. Then, the mandible was mobilized manually, but it did not move. An intraoral incision was made on the left side, and the left coronoid process was removed. Another preauricular incision without a temporal extension was made on the left side. Surgical opening of the joint, discectomy, and high condylectomy were performed on the left TMJ. The right temporalis fascia was used as the interpositional graft material. An occlusal stop (height: 30 mm) was inserted between the dental arches and maxillomandibular fixation (MMF) and was maintained for 5 days. The maximum mouth opening (MMO) was 35 mm after surgery. Postoperative pain medications were administered, and vigorous postoperative physiotherapy was performed to preserve the range of motion and to prevent postoperative hypomobility due to recurrence.\nAfter the 3-month follow-up, the MMO was 25 mm. All teeth were extracted because of the severe dental problems. One month after extraction, the patient underwent dental implant surgery (). Four months after dental implant surgery, she had full mouth rehabilitation (). Two years after surgery, the MMO was approximately 20 mm, and the masticatory function was normal (). |
A 48-year-old female patient presented to our outpatient clinic two years ago with complaints of severe muscle weakness. She stated that muscle weakness had been troubling her for more than 10 years and she had presented to a hospital with muscle weakness, fatigue, and respiratory distress at the time. The epicrisis drafted for the patient back then showed the proximal muscle strength in both upper and lower extremities to be between ⅖ and ⅗ as identified in physical examination. No family history was indicated in the given report. According to the laboratory analyses, the patient had a creatine kinase (CK) level of 4,267 (29-200 U/L) and tested negative for antinuclear antibodies and extractable nuclear antigens. Electromyography findings were suggestive of inflammatory myopathy in the proximal muscles of both lower and upper extremities. Furthermore, muscle biopsy performed back then led to the observation of necrosis in the deltoid muscle fibers, which was considered to be suggestive of PM. Neither thoracic computed tomography nor electrocardiographic examination indicated pathologies. At the time, the patient was put on a 1,000 mg/day of methylprednisolone treatment for three days, and the treatment was continued with 1 mg/kg/day of methylprednisolone and 15 mg/week of methotrexate, and the dose of methylprednisolone was gradually reduced to 4 mg/day. The patient benefited from the treatment to a certain extent; however, she had to come back to the clinic within eight months because of recurrence of the previous complaints, upon which hydroxychloroquine was added to her treatment regimen. Nine years ago, the clinic shifted to 1 mg/kg/day of prednisolone and 3 mg/kg/day of azathioprine because of insufficient improvement in the patient’s condition. Seven years ago, subcutaneous administration of 15 mg/week of methotrexate was added to her treatment regimen, upon which the patient suffered further deterioration and muscle weakness and received a course of intravenous immunoglobulin, followed by 40 mg/day of leflunomide in the next years. She also received 1,000 mg of intravenous ritixumab twice with a two-week interval nearly a year ago. No positive effects were observed after these treatments, and the CK levels of the patient was identified to be above 1,000 U/L all the time, ranging between 1,000 and 4,000 U/L. A written informed consent was obtained from the patient.\nThe patient received a total hip replacement because of aseptic necrosis in the right femoral head that developed due to the use of steroids. Our physical examination indicated the muscle strength to be ⅕ in the proximal side of the bilateral upper extremities and ⅖ in the proximal side of the lower extremities. There were no marked findings of atrophy in the muscles. We found the body mass index to be 30 kg/m2. With a level of CK at 3,025 U/L, the patient was put on a monotherapy regimen of 125 mg/week of abatacept. After two months of treatment, muscle strength was found to be ⅗ in the upper extremities and ⅘ in the lower extremities, and the CK level dropped to 1,950 U/L (Figure 1). Muscle strength was found to be ⅗ in the upper extremities and ⅘ in the lower extremities in the fourth month of the treatment, with CK level coming down to 1,200 U/L. Her examinations at month eight showed a muscle strength of ⅘ in both upper and lower extremities and a CK level of 1,003 U/L. The patient was put on resistance exercises. The whole cycle of a 12-month treatment was completed in November 2018 with full muscle strength achieved and a CK level of 484 U/L. The patient, who is being regularly followed-up, is still receiving a monotherapy of 125 mg/week of abatacept, and her condition is stable. |
The patient was a 61-year-old man with multiple left rib fractures (1–6 ribs), left pneumothorax, left lung contusion, and left thoracic subcutaneous emphysema due to a fall injury. The examination showed a partial depression in the left front rib and abnormal breathing (see Fig. ).\nAdmission chest CT examination: 1–6 rib fractures on the left side (of which 3, 4 ribs are long comminuted fractures (see Fig. )); left pneumothorax, left traumatic wet lung; a small amount of liquid pneumothorax on the left side.\nPatient was given early chest straps, multiparametric monitoring, analgesia, and oxygen therapy. The chest pain was still severe. The visual analogue scale scored 7–8 points for the pain at rest and 9 points for the cough.\nPhysical examination revealed that the left chest wall was recessed and abnormally breathed. The CT scan of the rib showed a long comminuted fracture of 3 and 4 ribs. The key to successful operation was the reduction and fixation of these two rib fractures. A preoperative CT scan was performed to reconstruct the 3D model based on the scan results (see Fig. ), and 3D printing technology was used to prepare 3 and 4 rib models (see Fig. ). The three D print models of each fracture segment of the two ribs were adherently reconstructed.\nThe two rib metal plates were separately shaped according to the reconstruction model (see Figs. and ).\nThe patient is scheduled to have a open reduction and internal fixation of 3–6 rib fracture. After general anesthesia, right side lying position, small incision about 8 cm was performed under the edge of 4th rib underarm. The skin was sequentially incised and the subcutaneous tissue was freed layer by layer. The front of the latissimus dorsi muscle and the anterior serratus were exposed. The tunnel was established on the 3rd and 4th rib surfaces from the back of the chest and small muscles to the back of the scapula. The special long hooks lifted the scapula and exposed the scapular operation space. With assistance of endoscope, the electrocautery is useful to expose 3 cm outside the outermost fracture lines of the 3 and 4 ribs. The locking plate was molded on the surface of the third rib before operation, and the broken end of the non-fracture at the anterior and posterior portions of the third rib was well fitted. The distance between the two ends of the metal bone plate exceeded the fracture line to 3 nail holes distance. Under the thoracoscope, the metal plate and the ribs were temporarily fixed with long-angled forceps. The MIPO system was used to drill the holes. Two screws were implanted and locked at both ends to firmly fix the metal plate. In turn, each fracture segment was reset and drilled and secured to a metal plate. The fourth rib is fixed in the same way. Intraoperative image (Figs. , and ). 5, 6 rib fractures given to fix the ribs, not the content of this article, not elaborated. Sufficient to stop the bleeding, the wound was given to leave a negative pressure drainage tube. After a routine thoracoscopic probe of the chest cavity, a closed thoracic drainage tube was placed posterior to the 7th intercostal space and the incision was closed layer by layer. After the chest wall is well-shaped. Three days after surgery review the map (Fig. ). |
A 31-year-old woman, gravida 2 para 1, presented for a prenatal ultrasonographic examination at 36 gestational weeks owing to a suspicion of a fetal thoracic wall defect. Her personal history revealed a spontaneous abortion and no consanguinity. She underwent routine ultrasonographic examinations at 13, 22, and 30 gestational weeks at a regional hospital; however, at 35 gestational weeks, ultrasonography revealed an abnormal fetal thoracic wall.\nPrenatal ultrasonography revealed a fetal thoracoabdominal wall defect with partial displacement of the left ventricle and the liver associated with rotation and elongation of the heart and a high index of clinical suspicion for intracardiac malformations such as tricuspid atresia, a ventricular septal defect, and pulmonary artery hypoplasia (Figures –).\nBased on the aforementioned findings, she was admitted to the Obstetrics and Gynecology Clinic in Târgu Mure at 39 gestational weeks, where she underwent a cesarean section. The male newborn weighed 3,100 g with an APGAR score of 7. Clinically, he demonstrated a superior abdominal wall defect, a partial extrathoracic displacement of the heart, and a partially herniated liver (these structures being covered by a very thin skin layer), and also a diastasis of the sagittal suture (Figure ). The newborn was intubated, and we applied a saline-soaked gauze pad on the thoracoabdominal and cranial defects to maintain humidity.\nPostnatal echocardiography confirmed the prenatal diagnosis and also showed a partial extrathoracic and extra-abdominal displacement of the heart and liver, a large ventricular septal defect, great arteries originating from the left ventricle with the aorta situated anteriorly, a posterior deviation of the outlet septum causing severe subpulmonary stenosis, hypoplasia of the pulmonary artery, and a large hourglass-shaped left ventricle secondary to narrowing of the heart at the level of its extrathoracic displacement.\nWe also performed thoracoabdominal CT-angiography, which showed complex cardiac malformations consisting of large ventricular and atrial septal defects, an increased left ventricular volume, with apical extrathoracic aneurysmal dilatation below the xiphoid process at the level of the abdominal midline, hypoplasia of the right ventricle, and a reduced caliber of the pulmonary trunk artery. Abdominal CT revealed partial transparietal herniation of the left hepatic lobe adjacent to a left ventricular diverticulum, and an increase in the size of the right adrenal gland with hyperdense contents suggesting an adrenal hematoma. Cranial CT revealed a diastasis of the sagittal suture causing subcutaneous herniation of the venous sagittal sinus.\nFollowing admission to the Neonatal Intensive Care Unit, the newborn was administered ampicillin and amikacin, fluconazole, prostaglandin E, and phenobarbital (because he presented with multiple seizures), and also received daily dressing changes. During the first week of life, he showed multiple episodes of bradycardia and low oxygen saturation despite undergoing orotracheal intubation; therefore, surgical intervention was postponed until he was hemodynamically stable. He underwent surgical intervention at 14 days of age, consisting in the replacement of the heart inside the thorax via a systemico-pulmonary shunt procedure, with vascular prosthesis, the ligature of both persistent arterial canal and pulmonary artery trunk, and repair of the diaphragm defect. The abdominal wall defect was also sutured, but the thorax remained open. The surgical procedure was performed in extracorporeal circulation, and lasted 4 h and 15 min.\nPostoperatively, the newborn developed multiple episodes of tachyarrhythmia and low cardiac output suggesting an inability of the heart to adjust to the intrathoracic pressure. Unfortunately, the newborn died 5 h postoperatively secondary to progressive hemodynamic deterioration, metabolic acidosis, and hypoxia. |
We present a case of a 46-year-old man living in a remote rural area with a history of an old left-sided intracranial hemorrhage. The patient had lingering neurologic deficit from his previous stroke and presented with a report of intermittent back pain for the past 3 years. The pain first started as pressure-like in nature, 2 of 10 in intensity, not radiating, and occurring once every 2 to 4 weeks. Two weeks before presenting at our institution, the patient experienced gradual increase in his pain symptoms, the pain was continuous and pressure-like in nature (6 to 7 of 10), and radiating to the upper and lower spine without limb involvement; however, it was controlled with simple analgesia. One week later, the patient sought medical treatment in an outside facility where tramadol was needed to control the pain (dosages unknown). The patient denied any limb weakness, radicular pain or numbness, and urinary or stool incontinence at the time. The patient was referred to our institution's spine unit for assessment. His referral included an MRI (without contrast) and a medical report suggesting an osteogenic tumor.\nOn examination, the patient was vitally stable. He had a normal gait; however, the patient could not walk in a tandem gait or on tiptoes/heels alone. It was noted that the pain increases with axial rotation of the abdomen and lateral bending. Bilateral lower limb examination showed normal motor function and reflexes. Sensory examination revealed hypoesthesia to pinprick and intact vibration sense in the left lower until midthigh, all of which were present since his previous brain hemorrhage. The examination was otherwise normal.\nThe plan was to control the pain, monitor distal neurovascular status, rule out brucellosis by a brucella titer, and repeat erythrocytes sedimentation rate and C-reactive protein tests, in addition to doing an acid-fast bacilli test and culture for tuberculosis.\nHowever, a secondary review of his MRI by a senior musculoskeletal radiologist leaned toward a possible vascular etiology (T2-weighted sequences: the mass showed heterogeneous T2 signal with concentric layering and central signal void. T1-weighted sequence: the mass showed mild heterogeneity with intermediate to high T1 signal with pulsation artifact, Figure ). The findings were suggestive of a partially thrombosed pseudoaneurysm. Contrast studies were needed, but the decision was quickly forgone after further assessment because the patient had elevated creatinine (171 umol/L) and low estimated glomerular filtration rate (40 mL/min/1.73 m2).\nA color Doppler ultrasonography (US) study (Figure ) was recommended instead to confirm the presence of a pseudoaneurysm and if present, to proceed with CT angiography. The color Doppler US showed a bidirectional and pulsatile turbulent flow, further confirming the presence of a pseudoaneurysm.\nNephrology consultation was made because the patient was expected to undergo multiple contrast-enhanced studies and/or treatment modalities. After proper optimization and clearance, we proceeded with CT angiogram that showed a right paraspinal mass at the T9-T11 level (Figure ). It had contrast enhancement and flow inside, consistent with partially thrombosed pseudoaneurysm. The lesion was thought to be arising from the right T10 segmental or intercostal artery. Evidence of bone remodeling in the adjacent thoracic vertebrae was also noted (Figure ).\nThe procedure was discussed, along with its indication, alternatives, and potential complications, and informed consent was obtained. Using a standard technique, right femoral artery access was obtained. An aortic angiogram was performed, which showed the posterior intercostal arteries at the levels of T8, T9, and T10. A large aneurysm was noted, arising from the midpart of the right T10 segmental artery with a narrow neck, pointing toward the spinal column with an estimated sac of 3 to 4 cm. A tributary to the anterior spinal artery coming from the left T10 segmental artery was also noted. Embolization of the pseudoaneurysm was performed by using Onyx 18 (ONYX liquid embolic system, Medtronic) (Figure ). Postembolization contrast injection showed no refilling of the pseudoaneurysm.\nPostprocedure, the patient was stable and discharged the next day. His pain completely resolved postembolization. Patient was pain free due to the pressure relief that was pressing on his vertebra in association with the pain medications he was discharged on; the patient was discharged on tramadol, acetaminophen, omeprazole, and nifedipine. He was seen in clinic 14 days later with no new symptoms or reports. Follow-up imaging was planned for his 6-week visit, but he was lost to follow up. |
A previously healthy 9-year-old female presented to her primary care physician after developing left-sided facial weakness. She was referred for magnetic resonance imaging (MRI) which showed T2/FLAIR hyperintensity centered within and expanding the pons. The initial physical exam revealed several neurological abnormalities including a left 6th nerve palsy with bilateral nystagmus as well as an incomplete left facial palsy and left-sided dysmetria.\nThe patient was enrolled on a Children's Oncology Group trial with vorinostat and focal radiation therapy; the post-radiation MRI revealed improvement in the pontine lesion with decreased mass effect. However, seven months later the pontine glioma increased in size and two new metastases were simultaneously noted: 1) a large lesion of the septum pellucidum involving the frontal horns of the lateral ventricles and the undersurface of the anterior corpus callosum (labeled ‘SP’ metastatic point), and 2) a left posterior hippocampal lesion (labeled ‘PH’ lesion) (Figure , ).\nThe patient was next enrolled in a Pediatric Brain Tumor Consortium trial and received two doses of a telomerase inhibitor which was discontinued for reasons unrelated to the patient's clinical course, and she was noted to have subsequent progression of both the primary and metastatic lesions. After the patient died, an autopsy was performed within 10 hours of death, and fresh-frozen and formalin fixed tissue was obtained from the primary site and the disseminated lesions as well as the grossly normal brain.\nCoronal sections of the post-mortem brain showed the SP tumor: a large, ill-defined metastatic solid mass centered at the septum pellucidum and involving the corpus callosum, right internal capsule, and the frontal horns of the lateral ventricles. Autopsy also revealed the PH lesion: a smaller lesion centered in the left posterior hippocampus. The basis pontis was significantly expanded, while the cerebellum was grossly normal.\nHistological analysis was undertaken of multiple sites in both the primary and the two metastatic tumor sites, showing that the SP tumor, much like the primary site, was overall best classified as high-grade (WHO Grade IV, Figure ) while the PH tumor was lower grade; nevertheless, all sites of disease displayed focal necrosis and vascular proliferation. The metastatic tumors had scattered areas of small, round blue cells reminiscent of PNET, but were strongly diffusely positive for GFAP, negative for synaptophysin and were overall best classified as glioma. Metastatic disease in the SP tumor had an increased Ki67 proliferation index compared to the brainstem lesion, 30% vs. 8%, respectively. All tumor sites displayed positive staining for the histone 3 K27M (H3K27M) mutation with less evident histological staining for wild type histone 3 trimethylation (H3K27me3) (Figure ). The tumors were also positive for CD45 representing various degrees of infiltrating resident microglia and macrophages (Figure ).\nWe extracted mRNA from six brain locations to attempt to differentiate metastatic from primary tumor based on molecular signature, including three locations from the brainstem tumor and 3 sites from the SP tumor (right and left ventricular portions as well as the right frontal portion). No mRNA could be extracted from the PH lesion due to fixation issues. mRNA profiling was completed using the NanoString platform (Cancer Panel) and the differential mRNA expression pattern between the primary brainstem and metastatic tumors was assessed using Partek Genomic Suite software. mRNA profiles of different sites of the large SP tumor were variably similar to the brainstem tumor (Figure ). Ingenuity pathway analysis revealed that p53 signaling, cell cycle regulation, DNA damage response, growth arrest and DNA damage-inducible 45 (GADD45), and ATM signaling were common tumorigenic pathways between primary and all sites of the SP tumor. We identified differentially expressed mRNA species between the primary tumor compared to the ventricular portion (twenty-two species) or the frontal portion (eight species) of the metastatic SP tumor (fold change > 1.5; < -1.5; p < 0.05) (Table ). For example, expression of the fibroblast growth factor receptor, FGFR3, is up regulated in the brainstem compared to metastatic tumor samples. However, the overall mRNA profiles of these differentially expressed genes primarily exhibit dysfunction of cell cycle regulatory pathways (Table ). Given the complexities of histological staining for tumor assessment, these identified mRNA profiles may have clinical relevance in rapid identification of potential therapeutic targets. |
We present a case of a 51 year-old white female with a 5-year history of ulcerative colitis. She has been taking mesalamine 1.2 gram (2 tablets two times a day) for 1 year with no complications that were reported. No history of allergies and no history of smoking or alcohol abuse were present. Past medical history was only remarkable for ulcerative colitis and there was no significant past surgical or family history. Around March 2018, she started having increased watery diarrhea with occasional blood (10-12 bowel movements per day from a baseline of 1-2 bowel movements per day) as well as cramping abdominal pain. She went to see her gastroenterologist in clinic. On physical examination, she had diffuse tenderness to palpation of her abdomen. Stool studies including stool cultures, stool ova, and parasites were sent which were negative. ESR and CRP levels were elevated. Therefore, she was thought to be in a moderate to severe ulcerative colitis flare based on the current criteria and was prescribed budesonide multimatrix (MMX) 9 mg once daily. Her abdominal pain improved but the diarrhea persisted. She then received a course of oral prednisone 40 mg daily for one month without any improvement of her symptoms and was subsequently started on infliximab therapy. Prior to initiating infliximab therapy, an interferon gamma release assay, hepatitis panel, varicella zoster antibody, and HIV tests were done which were negative. On 4/13/2018, she received her first dose of infliximab 500 mg based on her weight of 100 kg (5mg/kg). Her symptoms got better during the first week after the infusion; however during her second week, she complained of nonradiating chest pain located at the midsternal region, shortness of breath, and worsening fatigue. She went to a hospital where she was admitted. Her initial vitals were significant for low to normal blood pressure and a persistent tachycardia of up to 110. EKG was negative for any acute changes and a CT-PE was also negative for pulmonary embolism but showed a moderate size pericardial effusion. She was given fluids with no change in the blood pressure, and she continued to remain hypotensive and tachycardic and was eventually transferred to another hospital for concerns of a cardiac tamponade. At the other hospital, a transthoracic echocardiogram was done that showed an ejection fraction of 65-70% and a small to moderate size pericardial effusion that was present more anteriorly and less prominently on the apical, inferior, and subcostal views (Figures and ). There were no echocardiographic criteria for cardiac tamponade. Based on the difficult anatomical location of the effusion, decision was made to medically manage the patient.\nShe underwent extensive workup to evaluate the etiology of her pericardial effusion. Viral causes including HIV, monospot test were negative. T-spot was also negative. Due to concerns for a drug-induced lupus from infliximab, ANA and ds-DNA were checked, which were negative. Antihistone Abs were 1.9 (positive). ESR was 70 and CRP was more than 190. There were no signs of serositis, oral ulcers, photosensitivity, blood disorders (leukopenia, anemia, and thrombocytopenia), neurologic disorder, or rash (malar or discoid).\nThe clinical presentation was not compatible with any other pathology and based on the specified time frame of the presentation, a diagnosis of infliximab induced lupus was made and patient was taken off infliximab therapy.\nHer infectious workup for diarrhea including stool culture, stool ova and parasite, and clostridium difficile were negative. A procalcitonin level was also negative. She was in a moderate to severe ulcerative colitis flare and was therefore started on IV methylprednisolone 60 mg daily for 3 days and then transitioned to PO prednisone 40 mg daily. Her shortness of breath and fatigue got better and, after discharge, her diarrhea frequency went back to baseline. After she completed her prednisone taper, she was planned for vedolizumab (antagonist to α4β7 integrin) therapy for her ulcerative colitis. Vedolizumab is not shown to be associated with drug induced lupus [] and that is why it was chosen for our patient. She got induction therapy with IV vedolizumab 300 mg at weeks 0, 2, and 6 and was then continued on maintenance therapy with IV vedolizumab 300 mg every 8 weeks. She did not receive infliximab therapy in the future. Post discharge, serum anti-TNF alpha antibodies were checked which were negative. |
A 68-year-old woman was admitted to our hospital in September 2016 because of a history of back pain and progressive bilateral lower extremity weakness for 2 months. The patient had no trauma history and her significant medical history included non-insulin-dependent diabetes mellitus and hypertension. On examination, tenderness in the thoracic spine region was positive. Neurologic examination demonstrated bilateral lower limb spasticity with an asymmetric pyramidal pattern of weakness (right > left, Medical Research Council grade 4 and 3, respectively). No reflex deficits were identified, and there was no evidence of bladder or bowel dysfunction. No further neurological deficits were found. Magnetic resonance imaging (MRI) of the spine revealed a diffusely contrast-enhancing epidural mass extending from vertebral body T6 to T8 with compression of the spinal cord (Fig. A, B, G). The epidural mass extended through the right intervertebral foramen of T6–7, forming a paravertebral tumor of similar size. No hemorrhage or infiltration of bony structures was noted. The possibility of inflammatory spondylitis with epidural abscess was considered while tumor should be excluded. Laboratory investigations including tumor markers did not show any abnormal findings.\nTo achieve early decompression of the spinal cord and establish a definite pathological diagnosis by samples from the epidural mass, a T6–8 laminectomy was performed. The dorsal mass was near-total resected with partial resection of the ventral and lateral tumor. The resected specimen appeared as a 4 × 2 cm, grayish, fleshy mass lesion of soft consistency. Complete resection was impossible because of significant adherence of the tumor to the dural sheath. After successful surgery procedure, the postoperative course was uneventful.\nPathological examination of the tumor samples revealed that the majority of the tumor cells were monocytoid small B-cells, which have small to medium sized nuclei and abundant pale cytoplasm, surrounding germinal centers. By immunochemical studies, most tumor cells were positive for CD20, CD21, CD45, CD79a, CD43, bcl-2 with Ki-67 labing index was 15%, but were negative for CD3, CD5, cyclin D1, BCL6, and CD23. Expression of CD3 and CD5 was restricted to reactive T lymphocytes. Given the nonspecific B-cell phenotype and the presence of reactive germinal centers, the pathology department reported that these findings were consistent with MALT lymphoma.\nFour months later, the patient visited our hospital again for her progressive asymmetric weakness of both lower limbs and increasing back pain with bowel and bladder dysfunction. The PET/CT was performed which indicated that the thoracic spine of T6–8 level (SUVmax = 8.74), right iliac wing (SUVmax = 9.05), and right liver (SUVmax = 8.35) presented abnormal 18-fluorodeoxyglucose (18-FDG) uptake (Fig. ). Taking together tumor and surgical destruction and distant metastases was considered. Unfortunately, the distant lesions did not undergo biopsy and surgical section. Then bone marrow biopsy and cerebrosppinal tapping were performed, which revealed no infiltration. Based on these findings, a diagnosis of stage IVE MALT lymphoma was made according to revised staging for primary nodal lymphoma and Lugano classification.[ In combination with the patient's intention who refused to radiotherapy. Systemic therapy with rituximab, methotrexate, cyclophosphamide, vincristine, and prednisolone (R-MAX-COP protocol) every 3 weeks and central nervous system prophylaxis (intrathecal cytarabine and dexamethasone) were carried for the patient. After the first cycle of chemotherapy (Fig. C and D), her back pain, motor weakness, and urinary dysfunction showed remarkable improvement. After 4 cycles, her MRI of spine confirmed continuing clinical remission of the primary lesion and right iliac wing (Fig. E, F, H, I). No new disease manifestations development until recent fellow-up. |
A 58-year-old male patient who had no past medical history presented with fatigue, subjective feeling of the left lower-extremity weakness for 1 month, and the left facial palsy for 3 days. Brain MRI was preformed after admission to the Department of Neurosurgery at the Konkuk University Medical Center. A brain tumor was revealed at corpus callosum and septum pellucidum. On the physical examination was observed was the central type left facial palsy without other neurological abnormalities. He underwent craniotomy and a tumor removal operation, and didn't show any changes in the neurological examination following the surgery. After the diagnosis of the diagnosed as diffuse large B-cell lymphoma on the tissue biopsy, the patient he was transferred to the Department of Internal Medicine for chemotherapy. He received one treatment of the systemic chemotherapy, with MTX, thiopeta, and vincristine, 16 days after the surgery. Five weeks after the surgery, the patient he underwent the IT chemotherapy 9 times, with MTX 15 mg, Ara-C 40 mg, and hydrocortisone 50 mg over a 4-week period, followed by the intracranial radiation therapy with 30 Gy doses. After 4 weeks from the initiation of the IT chemotherapy, he developed a gradual weakness of the bilateral lower extremities, and then dysuria 4 days later. The cerebrospinal fluid (CSF) study was normal with a pressure of 13.0 cm H2O, the protein level 78.1 mg/dL, and the glucose level more than 70% of blood sugar. There were no findings of white blood cells nor malignant cells in the CSF. The patient was transferred to the Department of Rehabilitation Medicine for the evaluation and treatment of the bilateral lower extremities weakness. He demonstrated a Medical Research Council (MRC) grade III weakness of the right hip flexor and knee extensor, MRC grade II weakness of the right hip extensor, knee flexor, ankle dorsiflexor and plantarflexor, and big toe extensor and flexor, and general MRC grade II weakness of the left lower extremity. There was hypesthesia to the pain, temperature, vibration, and proprioception at the dermatome below L2 level bilaterally. There were no responses of the bilateral knee and the ankle deep tendon reflexes, and the pathologic reflexes including Babinski sign and ankle clonus were absent. He received a Foley catheter insertion, as he did not have any urination sense and was not able to void at all. He also could not sense anal mucosa touch or deep anal pressure, and showed weakness of the anal sphincter muscle. There were no responses of the bilateral bulbocavernosus reflexes (BCR). However, the bilateral upper extremities and trunk had normal neurological findings. Electrodiagnostic examination was performed 5 weeks after the onset of the bilateral lower extremities weakness. It revealed a decreased compound muscle action potential (CMAP) amplitude of the right common peroneal nerve, measured from the extensor digitorum brevis (EDB) and the left tibial nerve from the abductor hallucis (AH) muscles. No CMAP response was detected from the left EDB muscle. There were normal sensory findings of the bilateral superficial peroneal and sural nerves. Late response studies showed the delayed F-wave latency of the left tibial nerve, with no F-wave of the left common peroneal nerve. The H-reflex was absent in the bilateral tibial nerves (). The motor-evoked potentials recorded from the bilateral AH muscles following the stimulation of the bilateral primary motor cortices showed delayed latencies, 46.00 ms in the right and 45.85 ms in the left side. However, the recordings of the bilateral upper extremities were normal. The somatosensory evoked potentials which were recorded on the bilateral somatosensory cortices had no responses when the bilateral tibial nerves were stimulated, but showed normal findings when the bilateral upper extremities were stimulated. The needle electromyogram revealed abnormal spontaneous activities in the bilateral iliopsoas, vastus medialis, tibialis anterior, peroneus longus, gastrocnemius, gluteus maximus, and the 1st sacral paraspinalis muscles, along with the polyphasic motor unit potentials in the left tibialis anterior and the peroneus longus muscles (). The electrodiagnostic study findings were consistent with the bilateral lumbosacral polyradiculopathy from the 2nd lumbar to the 1st sacral spinal nerve. The urodynamic study revealed the areflexic neurogenic bladder which may present the 2nd to the 4th sacral nerves dysfunction. He also presented a decreased sense of the anus and a weakness of the anal sphincter muscle. He was diagnosed with the cauda equina syndrome based on his clinical presentation and the results of the electrodiagnostic and urodynamic studies. The lumbar computed tomography (CT) did not show other lesions except the central spinal stenosis at the L4-5 intervertebral disc level to a mild degree, which was not significant enough to induce a nerve injury (). He received a rehabilitation program regimen including the wheelchair transfer training, strengthening of the upper extremities, activities of daily living training, and management of neurogenic bladder and bowel. There were no neurological improvements upon discharge and at 14 months follow-up visit after the onset. |
A 50-year-old male school administrator was diagnosed with DCMP with congestive heart failure. He received medical treatment since 1996 and his LVEF was 20% at the reported time.\nOn December 12, 2011, the patient was admitted to the emergency room due to a sudden collapse and mentality loss. At the time of initial evaluation, the patient was still unconscious and the electrocardiogram (ECG) showed a ventricular fibrillation. A sinus rhythm returned after 40 minutes of cardiopulmonary resuscitation with defibrillation and intravenous injections of epinephrine and amiodarone. The patient received post-resuscitation care including therapeutic hypothermia, mechanical ventilation, and a tracheostomy in an intensive care unit. After three months, the patient got his consciousness back but his LVEF was 14% only.\nOn April 8, 2012, the patient was transferred to the department of rehabilitation medicine where he was provided with CR. The cognitive function measured with the Mini-Mental State Evaluation (MMSE) score was declined to 7. The limbs were measured to be fair grade in a manual muscle test and the functional level was measured to be 71 at the Functional Independence Measure score. Thus, the patient needed moderate help in self-care, mobility, communication, and social cognition. The existing dysphagia persisted and a Levin tube was inserted therefore. Although the patient was capable to respire spontaneous the T-tube remained due to the dysphagia and the weak power for coughing and sputum expectoration. An ET monitored with ECG with 1.8 metabolic equivalents (METs) was started and the intensity was gradually increased. The first symptom limited exercise tolerance test (ETT) was conducted at the third week of CR (). The exercise intensity was increased by 0.5 METs every three minutes according to the manual protocol. After 14 minutes, the ETT was terminated by patient's request due to a dyspnea. At that time, the VO2max was 13.5 mL/kg/min and the exercise ECG showed frequent premature ventricular contractions. However, the hemodynamic response was acceptable without chest pain. The respiratory exchange ratio was 1.14. The patient participated in a CR program for 8 weeks in this hospital which included an ECG monitored ET for 50 minutes per session at five times per week (). Subsequently, a follow-up ETT was done using the same manual protocol after the completion of the CR program. The test was stopped after 28 minutes at the maximal heart rate (HR) of 178 bpm. The VO2max improved from 13.5 to 19.4 mL/kg/min (43% increase) and the myocardial oxygen demand at the submaximal level decreased from 21,710 (3.9 METs) to 12,669 (3.9 METs) mmHg.bpm. The peak myocardial oxygen demand was 19,936 (5.5 METs) mmHg.bpm. The Borg's scale of perceived exertion at 3.9 METs decreased from 15 to 9 (). A follow-up transthoracic echocardiogram (TTE) showed a slight improvement of the LVEF from 14% to 15% ().\nThe patient's MMSE improved from 7 to 13 and the activities of daily living could be independently performed almost. The dysphagia therapy and the swallowing functional electrical stimulation were continued. No aspiration or penetration was shown during a videofluoroscopic swallowing study and the patient could sufficiently expectorate by himself. L-tube and T-tube were successfully removed.\nThe patient performed an outpatient CR program with three times a week after the discharge from the department of rehabilitation. On December 26, 2012, a follow-up ETT was performed with the modified Bruce protocol for the first time. The test was stopped after 13 minutes due to patient's request at the maximal HR of 191 bpm and the VO2max was 19.5 mL/kg/min. The recent LVEF was 19%.\nThe patient received cardiac resynchronization therapy under the use of biventricular pacing according to the latest heart failure treatment guidelines [] and received also outpatient CR for additional 3 months. Together with his wife he is preparing to return to his previous job. |
A 46-year-old African-American man presented with an enlarging, tender, left-sided neck mass. The initial evaluation consisted of magnetic resonance and computed tomographic (CT) imaging that demonstrated a 7-cm mass with T1 signal and T2 signal contrast enhancement []. On the CT scan, the mass was revealed to be hyperdense. A presumptive diagnosis of nonsecretory paraganglioma was made after catecholaminergic products were absent in serum and urine analyses. Given the size and progressive growth of the mass and symptomatic status of the patient, resection was planned.\nAfter informed consent was obtained, digital subtraction angiography and balloon occlusion testing were performed. Angiography confirmed a 7-cm vascular tumor with very thin vascular channels arising directly from the walls of the common and internal carotid arteries. There was no pedicle large enough to be suitable for embolization []. This pattern was unusual because paragangliomas are extremely vascular with very well developed vascular channels that usually arise off the external carotid artery and have a pattern of arborization that is highly amenable to endovascular embolization. In this case there was the appearance that the vascular channels were direct offshoots of the vasa vasorum of the affected vessels. In addition, there was irregularity and dilatation in the region of the carotid bulb suggestive of carotid wall involvement, which is possible but rare in paragangliomas. The patient tolerated the balloon occlusion test; and because the tumor was considered to be invading the wall of the carotid artery, we elected to endovascularly sacrifice the internal carotid artery (ICA) in preparation for surgical resection. The purpose of this sacrifice was to starve the tumor of its blood supply and minimize the degree of intraoperative bleeding. The endovascular sacrifice of the ICA was accomplished without incident. One-week later, the patient was taken to the operating room for resection.\nSurgical exposure of the carotid sheath revealed a large mass in the region of the carotid bifurcation. Exposure from the skull base to the omohyoid muscle allowed sequential separation of the jugular vein and the vagus, accessory, and hypoglossal nerves from the mass. The entire carotid bifurcation was dilated without separation of planes between the internal and external carotid arteries, carotid bulb, and tumor. The common, internal, and external carotid arteries were subsequently ligated away from the tumor; and the tumor and neighboring arterial segments were resected en bloc and sent for pathologic examination. Four distinctive lymph nodes appreciated during neck dissection were additionally submitted for examination. After resection of the mass, the sympathetic chain ganglia below the carotid sheath became apparent and appeared uninvolved.\nThe patient woke up without deficits; however, on the second postoperative day, he developed mild hoarseness and swallowing difficulty with an enlarging neck mass. He was taken emergently for the evacuation of a neck hematoma. No active bleeding sites were noted. Thereafter, the patient made an uneventful recovery and was discharged home without evidence of cranial neuropathies.\nGross examination of the en bloc specimen revealed a segment of artery surrounded by a mass measuring 4.8 cm × 4.0 cm × 2.4 cm. The tumor had a tan, myxoid surface with yellow tissue, and hemorrhagic changes next to the thrombosed arterial segment. Microscopic examination revealed a large circumscribed cervical lymph node with a large elastic artery coursing through it. The lymph node showed distinctive features including follicles composed of concentric rings of mantle zone lymphocytes around germinal centers as well as prominent vascularity of the germinal centers, often showing a single penetrating arteriole [Figure and ]. Immunostaining patterns from the lymph node revealed CD23, cyclin D1, BCL2, CD20, CD3, CD138, CD56, kappa/lambda, CD30, and CD15, which excluded the diagnosis of lymphoma; and Congo red staining excluded amyloidosis. Additional adjacent benign lymph nodes submitted for microscopic examination showed some of the above-mentioned features of Castleman's lymphadenopathy. |
An 11-year-old girl was referred to our hospital in August 2010 with a lesion in the right cheek area which was progressively enlarging. The patient complained that her nose and mouth corner were crooked and that her face was swelling. In clinical examination, the patient had a slight asymmetry in the right midface as a result of buccal and palatal cortical expansion from the right maxillary canine to the molar region, resulting in depression of the nasal alar and mouth corner (Fig. ).\nA panoramic radiograph showed an increased bone density on the right maxilla and zygoma and obliteration of the right maxillary sinus. Computed tomography (CT) revealed a 4.5 × 4 × 4.5 cm, expansile ground-glass opacity lesion involving the right maxillary sinus, right maxillary alveolar process, zygoma, and hard palate. Bone scan revealed an irregularly shaped hot uptake in the right maxilla, and no abnormally increased uptake was observed at any other sites (Fig. ). The physical examination did not show any other lesions, and the patient had no history of pain, trauma, loosening of teeth, or trismus. Based on the typical radiologic findings, the patient was diagnosed with FD, and no additional biopsy was performed. The patient had regular follow-up every 6 months to monitor the lesion’s progress. At the 1-year follow-up, the development of tooth germ within the lesion was normal, and slight expansion of the lesion to the bucco-lingual side was observed. Because we thought the patient was still growing and increasing in height, we decided to conduct an ongoing progress observation.\nAbout 3 years later in December 2012, there were no significant changes of the FD lesion, but the distance from the mouth corner to the inner canthus was about 2 cm longer on the right side than on the left. Periodic observation was continued, and in August 2017, corrective surgery was planned because the maturation of the lesion was confirmed to be complete and there were no changes in the size of the lesion. At that time, the distance from the mouth corner to the inner canthus was 2.5 cm longer on the right side than on the left, and the distance from the occlusal plane to the outer canthus was 5 cm longer on the right side than on the left. Bone contouring surgery, the primary treatment for facial asymmetry and fibrotic bone lesions, was planned (Fig. ).\nThe patient wanted to improve asymmetrical facial appearance through the surgery. Therefore, we aimed not only to remove the FD lesion, but also to make the patient’s facial as symmetrical as possible. For this, direct visualization and surgical approach to the infraorbital rim and lateral area of zygoma were required, but the surgical approach through buccogingival incision had limited access to these areas. On the other hand, the midfacial degloving approach was expected to help reestablishing symmetric facial contour by allowing direct comparison of the lesion with the normal side. Moreover, this approach provides esthetically acceptable outcomes, leaving no scars and no functional disability. Therefore, we decided to perform the operation through the midfacial degloving approach.\nWith the patient under orotracheal anesthesia, the lesion was removed by the midfacial degloving surgical procedure. Local anesthesia with 2% lidocaine with epinephrine (1:100,000) was infiltrated into the maxillary vestibular mucosa and into the nose. The procedure is performed with a maxillary vestibular incision and three intranasal incisions to expose the entire midface skeleton that include (1) bilateral intercartilaginous, (2) complete transfixion, and (3) bilateral piriform aperture incisions (Fig. ).\nA buccogingival incision was made in the maxillary vestibule approximately 5 mm superior to the mucogingival junction and extended from the second molar to the contralateral second molar. Periosteal elevators were used to elevate the tissues in the subperiosteal plane fist over the anterior maxilla and then extending widely to encompass posterior tissues behind the zygomaticomaxillary buttress. The infraorbital neurovascular bundle was identified superiorly and dissected. Subperiosteal dissection along the piriform aperture stripped the attachments of the nasal labial muscularture to allow its complete release from the midface skeleton. The mucoperiosteal flap was elevated up to the piriform aperture.\nThe intercartilaginous incision divided the junction between the upper and lower lateral cartilages (Fig. b). An incision was made along the inferior border of the upper lateral cartilage, beginning at the lateral end and extending medially curved into the membranous septum anterior to meet transfixion incision (Fig. a). Laterally, the incision was sufficient that it extended to the piriform aperture. The lower lateral cartilage was eventually displaced superiorly during the degloving procedure, whereas the upper lateral cartilage remained attached to the midface skeleton. The transfixion incision was used to separate the membrane septum/columella from the cartilaginous septum. An incision was made along the caudal border of the septal cartilage from the medial end of the intercartilaginous incision toward the anterior spine (Fig. a). The intranasal incision was made by a full-thickness incision down through the periosteum of the piriform margin and the nasal floor.\nDissection through the intercarilaginous incision allowed access to the nasal dorsum and bones (Fig. b). Sharp subperichondrial dissection with a scalpel or a blunt dissection with scissors freed the soft tissues above the upper lateral cartilage as in a standard open rhinoplasty. The dissection should be within the subperichondrium plane to prevent injury to the overlying musculature and blood vessels of the nose. Elevation extended laterally to the nasomaxillary sutures and superiorly to the glabella. Retraction of the freed soft tissues allowed sharp incision to be made with a scalpel or with sharp periosteal elevators through the periosteum at the inferior edge of the nasal bones. Elevation of the soft tissue laterally to the piriform aperture was also performed so that the maxillary vestibular dissection was easily connected to this pocket.\nAfter the connection of the nasal and oral incisions, the midface was degloved. The midface skin was separated from the maxilla and the nasal pyramid. The upper lip and the intact nasal columella, nasal tip, and alar cartilages were then retracted by two Penrose drains introduced through the nostrils over the nose to the level of the inferior orbital rim. This approach provided visualization of the medial maxillary wall, pterygoid junction, nasofrontal suture, infraorbital rim, and laterally to the temporal process of the zygoma (Fig. d). Under direct visualization, the overgrowing bone lesion was then excised using osteotomes and saws. The right maxilla was drilled further at the orbital rim and laterally till zygomatic complex. The contour of the midface was reestablished using burr to give a cosmetically normal looking midfacial skeletal contour while protecting the infraorbital nerve (Fig. ). For the removed lesion, a biopsy was performed for the accurate diagnosis and histologically confirmed as FD. The soft tissues were then carefully redraped and the nasal tip brought back into position. The intranasal incisions were closed using 4-0 resorbable sutures (vicryl), and the transfixion sutures were precisely performed to determine the final position of the nasal tip and prevent vestibular stenosis. The cinch suture of alar base was used to prevent postoperative alar base widening. The intraoral incisions were closed using a 3-0 black silk. Nasal packing into the maxillary dead space with Vaseline gauze was done for 3 days in order to minimize the postoperative bleedings.\nThe patient’s postoperative course was generally uneventful. There was moderate nasal crusting for 3–4 days. Mild swelling with periorbital ecchymosis disappeared after 2 weeks, and transient paresthesia around the infraorbital nerve spontaneously resolved after 3 month. No postoperative complications such as epistaxis, vestibular stenosis, or esthetic problems of the nose were seen. Clinical and radiographic examinations obtained 4 months after surgery showed the anatomical structures were in a stable state without recurrence of FD (Fig. ). The esthetic result was satisfactory for the patient, and occlusal state was also well maintained (Fig. ). Therefore, no additional orthodontic treatment or orthognathic surgery was performed. |
A 59-year-old female patient with a long history of low back pain presented with swelling and distending pain in her lower extremities for 1 month; all her symptoms were aggravated after overexertion and improved with rest. Her lower limb symptoms were less severe in the morning and worse at night; however, since 1 week before the presentation, the swelling seldom resolved.\nOn physical examination, the patient showed stable vital features. During the walking test, the patient presented with intermittent claudication, which was more prominent on the left side. Her left thigh perimeter was 59 cm in the morning and 63 cm in the evening, while the right thigh perimeter was 55 to 59 cm. On examination of the spine, the patient had percussion pain and tenderness on the L4 and L5 spinous processes and in the corresponding paraspinal muscle. The distending pain in the calf and heaviness appeared after walking or standing for 200 m or for 5 minutes. In the lying position, there were no remarkable neurological symptoms, but there was a slight loss of key muscle force and pitting edema over the lower extremities. According to the visual analog scale (VAS), the patient scored 6 for her low back pain and 4 for her calf pain.\nLaboratory tests and electrocardiograph demonstrated normal renal and cardiac functioning, which excluded the probability of renal or cardiac edema. Lower extremity ultrasound showed no DVT or venous obstruction. To identify the etiology of her low back pain, lumbar magnetic resonance imaging (MRI) was conducted, which revealed lumbar disc degeneration (LDD) and anterior lumbar disc herniation (LDH) in the segment L4-5 (Fig. ). Furthermore, the narrowed IVC was visualized in the space between the anterior protrusion and right common iliac artery, both on the MRI and abdominal CT (Figs. C, D and 2A–C). An abdominal Doppler ultrasound indicated over 50% patency loss of the IVC due to the compression induced by the anterior LDH (Fig. D). Further examinations such as a venogram and CT with contrast were rejected due to the invasiveness and the patient's refusal of the contrast agent. Based on the current results, this patient was diagnosed with L4-5 lumbar disc anterior herniation, moderate IVC stenosis, and dMTS.\nFor surgery, we aimed the treatment at the anterior LDH instead of endovascular management because of the severe IVC compression of the anterior LDH and the evidence against DVT. RF was adopted instead of conventional intravenous stents. The patient was settled in a prone position, and the L4-5 disc and 2 puncture points were confirmed via C-arm fluoroscopy. After routine disinfection and sterile towel placement, local anesthesia was applied. Monitored by C-arm fluoroscopy, the puncture needles were inserted bilaterally and symmetrically until the needles reached the edge of the Kambin triangle (Fig. A). After confirming that the needles were in the correct direction, they were further inserted through the annulus fibrosus into the nucleus pulposus towards the midline, on the anteroposterior view (Fig. B). Then, the monopolar RF electrode was placed through the puncture needle to the central part of the disc complex. After confirming the absence of nerve irritation, thermocoagulation was applied at 50 °C, 60 °C, and 70 °C for 60 seconds each, followed by 80 °C and 90 °C for 90 seconds each without rest between temperature changes. During RF thermocoagulation, the patient reported only warmth at the site of her low back pain, and no burning feeling on either lower extremity. The total operation time was less than 30 minutes, and blood loss was minimal.\nAfter the procedure, the patient's swelling on both extremities started to relieve within 5 hours, and it completely diminished after 48 hours. Her VAS decreased to 1 and 0 at her lower back and calves, respectively. Postoperative abdominal ultrasound confirmed that the venous patency of the left common iliac vein increased to 70%, which represented mild venous stenosis (Fig. ). On follow up, only occasional slight lower back pain was reported, and her leg symptoms did not recur at 12 months after surgery. |
A 61 year-old man was admitted with a 103 ° F fever, confusion, weakness and slurred speech after hemodialysis. He had a history of viridans streptococcal mitral valve endocarditis, end stage renal disease on hemodialysis, atrial fibrillation not on anticoagulation due to GI bleeding, and monoclonal gammopathy of undetermined significance. He had a productive cough for a week without any identifiable sick contact. Physical examination was notable for an agitated edentulous man with a left central facial palsy, severe dysarthria, and a systolic murmur at the left lower sternal border. His lungs were clear to auscultation and there was no stigmata of endocarditis.\nThe patient was initially treated empirically for pneumonia and worked up for stroke. However, the treatment plan was quickly modified when a transthoracic echocardiogram on day two of admission revealed two echogenic structures consistent with vegetations: 0.4 × 0.4 cm on the anterior leaflet of the mitral valve, and the other 0.7 × 1.8 cm attached to left coronary cusp of the aortic valve (Fig. ). There was also thickening of the aortic root suggestive of abscess formation. Two sets of blood culture grew Gram-positive rods after 37.5 h incubating in anaerobic bottles (Fig. ), and after 86 h in aerobic bottles. The organism was identified as A. neuii by MALDI-TOF MS on day five of admission. Serial brain MRI scans revealed multiple bilateral infarcts on day two with increased number of infarcts and a small focus of hemorrhage on day five. The patient was diagnosed with infective endocarditis by A. neuii complicated by aortic root abscess and presumed cerebral septic emboli.\nThe patient was initially treated with vancomyin and piperacillin/tazobactam until A. neuii was identified. Subsequently, he was treated with ampicillin and gentamicin for two days, followed by ampicillin for the rest of his hospitalization. The choice of ampicillin was based on a large series that studied susceptibility to antibiotics of Actinomyces species [], and a previously successfully treated A. neuii endocarditis case []. Antibiotic susceptibility was not tested for our patient because he responded to the treatment well, and repeat blood cultures were all negative. A CT angiography of the brain and neck on day six ruled out mycotic aneurysm. It was concluded that the risk of further septic embolization outweighed the risk of intracranial hemorrhage, and the patient underwent aortic valve replacement, debridement of aortic root subannular abscess, mitral valve repair, and repair of a fistula between the aorta and left atrium on hospital day fourteen. A 2.5 × 0.6 cm vegetation on the aortic valve and a vegetation on the mitral chordae tendineae were removed. There was no microscopic evidence of bacterial elements on the aortic valve based on histopathology with Gram stain, and culture did not grow any organisms. The patient’s post-operative course was complicated by shock requiring intraaortic balloon pump, and a cardiac arrest from ventricular fibrillation 10 days after surgery. He recovered without further neurological deterioration, and was discharged to a nursing facility two months after heart surgery. He received 12 weeks of IV ampicillin followed by 11 months of oral doxycycline.\nOne year after the diagnosis of A. neuii endocarditis, while on chronic doxycycline, the patient had a fever and a bacteremia with coagulase negative Staphylococcus and group B Streptococcus. The bacteremia was sterilized after the initiation of antibiotic therapy and there was no growth from subsequent blood cultures. Transthoracic echocardiogram showed a small, mobile echogenic density on the non-coronary cusp of the bioprosthetic aortic valve. The patient refused to undergo transesophageal echocardiogram to further evaluate the prosthetic valve, so he was treated empirically for possible prosthetic valve endocarditis. The patient was cured from infection after two weeks of IV vancomycin and gentamicin, followed by four weeks of IV vancomycin. He had been taking oral doxycycline in addition to his IV antibiotics.\nThe patient eventually died of a sudden cardiac arrest after hemodialysis. This was 15 months after the diagnosis of A. neuii infective endocarditis, and four weeks after discontinuation of oral doxycycline. The family declined autopsy.\nPrimary infective endocarditis caused by Actinomyces spp. is rare. After PubMed (search term ((actinomyces spp) OR actinomyces) AND ((infective endocarditis) OR endocarditis)) and additional bibliographical search, we found 26 human cases dating back to 1939 (Table ), after excluding four reports, two with bacteria that have been subsequently reclassified to different genera [, ], one report with possible direct extension of pulmonary actinomycosis to the endocardium [], and one with primary IUD-associated actinomyosis and secondary endocarditis []. Cases were reported at all ages (6–87 years old). Two thirds of patients were men. The most commonly identified species were A. israelii (19%) and A. viscosus (15%). Twenty-two cases involved left-sided valves (mitral 9; aortic 5; prosthetic aortic 3; both mitral and aortic 4; undetermined 1). Risk factors included valvular disease (41%), poor dental hygiene or dental procedure (36%), and prosthesis (14%). All four right-sided cases were associated with intravenous drug use [, , , ].\nMost left-sided endocarditis patients had indolent courses. This did not seem to vary over time. However, the mortality and complications have improved significantly over time. Five of eight patients reported before 1990 died and five had embolic events (brain, spleen, kidneys, small bowel and skin), whereas only two of 14 cases reported after 1990 died, and only one had emboli to skin. Despite temporal courses of a subacute endocarditis, where stigmata of endocarditis are more common, only one report described Roth’s spots []. It is unclear whether this was related to virulence factors from Actinomyces spp., or simply the rarity of these complications []. Right-sided endocarditis cases had more acute and fulminant courses, and were all complicated by septic emboli to the lungs. Two (50%) of them had polymicrobial endocarditis [, ], which might have contributed to more complicated clinical courses. All four right-sides cases survived and all were reported after the year of 2000. Irrespective of the side of endocarditis, most patients were treated with a prolonged course of penicillin or β-lactam antibiotics. Four cases had surgery (three aortic valves [, , ] and one Eustachian valve [], an embryologic remnant of the valve of the inferior vena cava).\nTwo cases of infective endocarditis by A. neuii were previously reported [, ]. Both were in older men with preexisting aortic valvular anomalies (one had a bicuspid valve and the other a prosthetic valve). Both presented with subacute endocarditis, large aortic vegetations (2 cm) and root abscesses. The patient with a native valve underwent surgery []. Both patients were cured from the infection. One was initially treated with ampicillin, then ceftriaxone due to interstitial nephritis, and finally doxycycline for 9 months []. The other was treated with penicillin, followed by amoxicillin for 12 months []. |
A 53-year-old male presented to emergency department with complaints of acute onset, generalized rigidity and tremors of all limbs, difficulty speaking and swallowing along with slowness of movements, masked facies, and decreased blinking. He was recently discharged from medical ICU after being managed for episodes of vomiting and decreased food intake associated with confusion, lethargy, disorientation to time and place. Also he developed two episodes of generalized tonic clonic seizures. His serum Na+ was found to be very low (102 mEq/ml). He was also put on tab levetiracetam 1000 mg for seizure prophylaxis. At the time of discharge after 6 days of hospitalization his serum Na+ was 131 mEq/ml. His hyponatremia had been corrected rapidly (by 13 mEq/ml in initial 8 h). He had undergone neurosurgery for non-functional pituitary macroadenoma 10 years back. He was not taking any hormone supplements. It was hypothesized that he might have been having chronic hyponatremia due to hypopituitarism. Patient developed rigidity and associated symptoms after 6 days of sodium correction. He also underwent magnetic resonance imaging (MRI) of the brain which did not reveal any abnormality except for chronic ischemic changes in the bilateral centrum semiovale.\nDetailed assessment did not reveal psychiatric symptoms or any previous psychiatric illness nor was there any history of substance abuse. There was no family history of any psychiatric illness as well. From the history and review of medical records, a provisional diagnosis of CPEM was made. Neuroleptic malignant syndrome was ruled out as patient was not subjected to any antipsychotics in previous hospital as per records. Moreover, serum creatine phosphokinase (CPK) levels were normal. He was started on oral trihexyphenidyl (centrally acting anticholinergic) 1 mg TDS along with supportive treatment.\nMRI brain was repeated on day 4 of admission (after 5 days of onset of symptoms) which showed pontine hyperintensities and bilateral basal ganglia (caudate and putamen) hyperintensities on T2W1 and FLAIR sequences confirming the diagnosis of CPEM (secondary to rapid correction of hyponatremia) [Figures and ].\nPatient's symptoms started improving dramatically after the treatment was started. Hormone supplementation was started following reports of low Adrinocorticotrophic Hormone (ACTH), Thyroid Stimulating Hormone (TSH), Follicle Stimulating Hormone (FSH) and Leutinising Hormone (LH) levels. After 3 days of hospitalization, he developed behavioral symptoms for which psychiatric consultation was again taken. He was found to have disturbed sleep, irritability, suspiciousness toward a fellow patients in the ward and aggression toward treating resident doctors. He developed delusion of persecution and reference, claiming that a female patient on neighboring bed was passing insulting remarks to him indirectly when talking to others and was also teasing him through her gestures. He would appear restless and kept pacing in ward in anger. He was fully conscious and oriented with clear sensorium. No apparent mood symptoms could be noticed. He did not have any Schneiderian First rank symptoms. Possibility of any substance abuse in ward was ruled out. He was started on quetiapine 50 mg and dose was increased to 100 mg over a period of 3 days. He tolerated it well and his psychotic symptoms started improving over a period of 4 weeks. He was discharged from the ward after his extrapyramidal symptoms got completely resolved. During follow-up visits, trihexyphenidyl was tapered off over next 1 month and quetiapine was gradually tapered off over next 6 months without any new symptoms. The patient achieved complete remission and started attending office subsequently. |
We present the case of a 26-year-old Asian gentleman who presented to the Accident & Emergency (AnE) with three-week history of pain in the right foot. The pain was present all the time and there were no aggravating or relieving factors. He had no previous medical problems. There was no history of tobacco or alcohol use, and he was a car mechanic by profession. There was no history of trauma. He had no allergies and did not have any pets. There was no history of recent travel. Physical examination, vitals and an X-ray of right foot were normal and the patient was discharged home on simple analgesia. No blood tests were done on his first presentation.\nA referral to physiotherapy was made as well. He, however, represented a week later with similar symptoms. His D-dimers were elevated, however, right leg venous Doppler was negative for a deep vein thrombosis (DVT).\nHe presented to AnE for the third time and in addition to the foot pain, he now reported tingling sensations in his hands and weight loss of 1 stone in last three weeks. His general practitioner (GP) was suspecting a rheumatological problem and had already requested a computed tomography of chest/abdomen/pelvis (CT CAP) and a magnetic resonance imaging (MRI) of the right foot for him.\nHe was admitted to the hospital on this occasion. On examination his chest was clear with a central trachea and normal vesicular breathing. There was no clubbing and there were no palpable lymph nodes. Heart sounds were normal. Abdomen was soft, non-tender with a palpable liver edge 2 cm below the costal margin. Neurological exam revealed good power and tone in all limbs with no focal motor or sensory deficit. There were no cerebellar signs and he had a normal gait.\nHis blood eosinophils were raised at 1.1 with deranged liver function tests (LFTs). A chest X-ray on admission was normal.\nHe was referred to the Neurology team for his symptoms of pain and paraesthesias. A diagnosis of multiple sclerosis was ruled out by a normal MRI head and spine. A lumbar puncture (LP) was performed and cerebrospinal fluid (CSF) showed raised protein (1.24), normal opening pressure and no oligo-clonal bands with normal differential counts. Neurology team further advised nerve conduction studies (NCS) which showed “subacute demyelinating length dependent sensorimotor polyneuropathy with substantial axonal loss in the distal lower limbs”. Neurology suspected a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP), however, duration of his symptoms was short and inconsistent with this diagnosis. He received five days of IV immunoglobulins with little improvement. Neurology advice was to consider a course of methylprednisolone. He was booked in for outpatient neurology follow-up.\nMRI (Figure ) of his right foot showed stress fracture of fifth metatarsal and focal oedema in anterior aspect of calcaneum. Orthopedic team was called upon. They asked for an MRI of left foot which showed multiple stress-related focal changes/stress fractures in first, second, fourth and fifth metatarsals (Figure ). They advised “off loader boots” on both feet and mobilization as tolerated. Outpatient follow-up was arranged.\nRheumatology team reviewed him and advised that he most likely has marble bone disease. They advised outpatient rheumatology follow-up post discharge.\nHe was referred to hematology in view of young age, weight loss and an enlarged liver. After reviewing the case hematology team conducted a bone marrow biopsy which was normal.\nHis liver was imaged with an ultrasound in view of deranged LFTs and palpable liver and was noted to be “enlarged in size and hypoechoic”. An acute liver screen to look for viral, autoimmune, haemochromatosis and fatty liver disease was carried out and was negative.\nThe CT-CAP (Figure ) done for unexplained weight loss was reported as having “multilobar bilateral patchy subtle ground glass pulmonary opacification (GGO)”.\nThis prompted the involvement of the respiratory team. The respiratory team advised lung function testing (PFTs) (Table ) that showed a restrictive defect with reduced TLCO.\nT-Spot was negative. In view of the eosinophilia, GGO changes on CT and lung function test findings, a bronchoscopy with transbronchial lung biopsy (TBBx) was done and bronchoalveolar lavage (BAL) samples were taken for microbiology, cytology and histopathology. A differential count revealed mononuclear cells 60%, lymphocytes 35%, neutrophils 4%, and eosinophils 1%. Aura-mine, fungal and viral cultures were negative. Trans-bronchial lung biopsy showed “non-necrotising granulomatous inflammation” (Figure ).\nA number of differentials were considered before finally treating the patient as sarcoidosis. His bloods since his first presentation through to his follow-up are tabulated below (Tables , ) along with a list of other investigations and how they were used to rule out the considered differentials.\nThe biopsy results in conjunction with eosinophilia, high urinary calcium, restrictive lung disease on PFTs and high proteins on LP were consistent with a diagnosis of sarcoidosis.\nHe was started on oral prednisolone 50 mg by the respiratory team and discharged home with a planned multidisciplinary follow-up to ensure comprehensive care.\nHe has since been reviewed in the respiratory, rheumatology, neurology and orthopedic clinics. He reports his symptoms have shown significant improvement. His repeat MRI of feet (Figures , ) two months after his discharge showed that his stress fractures had improved. He also had a repeat CT thorax, three months after discharge which showed resolution of GGO. He remains under respiratory follow-up and continues to do well. |
Patient 1 is a 68-year-old married white British male with a diagnosis of TRS. He first presented with psychosis to psychiatric services in 1965 at age 17. Over the next four decades he was treated with a number of antipsychotic agents including sulpride, aripiprazole, haloperidol and olanzapine; all were at up to maximal British National Formulary recommended doses and a minimum of 12 weeks’ duration with compliance assured during inpatient episodes; he experienced a number of relapses over time. He did not achieve remission and was started on clozapine in 2002 (dose 400 mg daily). Following that, he was functional and relatively stable for 11 years, aside from some minor residual psychotic and anxiety symptoms.\nHis medical history at this time included ischaemic heart disease (IHD), cardiomyopathy with an ejection fraction of 35%, congestive heart failure (NYHA II) and bilateral pedal oedema. In 2014, following a decline in his cardiac function, his maintenance clozapine dose was reduced from 350 mg to 300 mg daily. Unfortunately, this was followed by a severe psychotic relapse characterized by prominent thought disorder, paranoid delusions, self-neglect and increasingly aggressive and challenging behaviour. His clozapine dose was increased back to 350 mg but his mental state continued to deteriorate and due to violence and aggression he was transferred to a psychiatric intensive care unit in March 2015.\nIn August 2016 he was admitted to the National Psychosis Unit, having been an inpatient for over 2 years. His symptoms at this time included persistent persecutory delusional beliefs, obsessive-compulsive traits, anxiety, mood lability, prominent difficulties in concentration and memory and irritability with outbursts of aggression. Psychotropic medication on admission included lurasidone 111 mg (which he had been treated with for over 1 year), sertraline 100 mg and diazepam 6 mg daily. On admission, he scored 115 on the PANSS total score and 29/30 on the Mini Mental State Exam (MMSE). An MRI brain scan was within normal limits; blood tests on admission were mostly within normal limits, except for a raised urea of 13.9 mmol/L (3.3–6.7) and an increased prolactin level of 894 mIU/L (100–410), attributed to lurasidone. The pharmacotherapeutic approaches considered included a trial of high-dose olanzapine, switching from sertraline to vortioxetine because of the added benefit in attention and cognitive flexibility, or adding either memantine or donepezil as adjuncts to aid cognition.\nIn August 2016 it was decided to increase his lurasidone dose to 148 mg daily. Sertraline was switched to adjunct vortioxetine 10 mg, which was gradually titrated to 20 mg over the next couple of weeks. By September 2016 there was noticeable improvement in symptoms, and by December 2016 he was in remission, scoring 44 on the PANSS total score. Pregabalin 25 mg BD was prescribed as an anxiolytic after stabilization on lurasidone and vortioxetine, and benzodiazepines were down-titrated and stopped. He was successfully discharged home in January 2017. Psychotropic medication on discharge included lurasidone 148 mg, vortioxetine 20 mg and pregabalin 25 mg BD. He described his wellbeing as 9 out of 10, compared to having been 1 out of 10 at admission. The family described his change as ‘miraculous, and that he had not been so well, even while being treated with clozapine’. Subsequent follow up confirmed a sustained functional and symptomatic recovery. |
A 30-year-old woman working as a clerical staff, requiring daily commuting by crowded trains, was suffering from left knee pain for last 6 years. Her difficulty in walking had worsened since last 6 months and did not respond to nonsurgical management. She was bedridden for 2 months before she presented to us. She also had a history of intermittent rectal bleeding.\nOn examination, there were cutaneous and soft tissue hemangiomas alongwith varicose veins throughout the affected left lower extremity from hip to toes, mainly on the gluteal region, posterior thigh, popliteal fossa, calf and foot [Figure -]. There were no visible salmon patches or portwine stains. Left lower extremity was not significantly enlarged compared to right lower extremity. There were no lesions over the anterior aspect of left knee. There was a 10° valgus alignment of the knee []. She had a flexion deformity of 20° with further flexion up to 40°. The movements were very painful in the available range of motion (ROM). The knee society pain score was 29 and her functional score was 0. There was no true leg length discrepancy. Dorsalis pedis and posterior tibial pulsations were palpable. The distal neurological status was normal. The right knee had full range of movements with no pain or deformity. There were no vascular lesions in right lower extremity. Bilateral hip examination was normal.\nRadiographs [] showed severe degenerative tricompartmental arthritis in the left knee joint with bone loss from the lateral femoral condyle and the lateral tibial plateau. There was subchondral sclerosis and osteophyte formation. Phleboliths in the vascular malformations were noticed on the X-rays.\nMagnetic resonance imaging and magnetic resonance venogram [] of the left lower limb with pelvis showed vascular malformation; mostly multifocal hemangiomas involving superficial and deep soft tissues of the entire left lower limb, pelvic region, rectum and left femur. There were no communications between the superficial and the deep lesions. Dual phase angiogram did not show any feeder artery or draining vein. As there were no feeder vessels, preoperative embolization was not done. Her coagulation profile was normal (bleeding time -5 s, clotting time -3 min, prothrombin time-13 s, international normalization ratio-1.1, activated partial thromboplastin time-28 s, Factor VIII-110% and Factor IX-100%). She did not have any systemic involvement. In the absence of limb hypertrophy, we could not label it as classical Klippel–Trenaunay syndrome, but it can be called as a variant of Klippel–Trenaunay syndrome.\nSurgery was done under spinal anesthesia with tourniquet and exsanguination of limb using Eshmarch bandage. Anterior midline incision with medial parapatellar approach was used. Computer-assisted navigated surgery was done due to intra medullary involvement of the lesions []. Ci navigation system (DePuy, Brainlab, Germany) was used. Two thin threaded pins with bicortical holds were used to fix the clamps carrying the tracker ball arrays. The pin hold was good and remained well till the end of the surgery as the bone quality was good on both femoral as well as tibial sides. The synovium was grey-black with an orange tinge of the remaining articular cartilage. Femoral condyles were squared. Femoral bone cuts also revealed vascular malformations in the bone which were curetted out [].\nCemented TKA using posterior cruciate substituting femoral prosthesis (PFC Sigma, Depuy, Johnson and Johnson) with all-poly tibial component [] was done. Bone wax was used to control bony bleeding from metaphyseal lesions at the femoral cuts. Complete hemostasis was achieved after release of tourniquet. Closure was done under re-inflated tourniquet as there was generalized ooze but no active bleeding. Total tourniquet time was 80 min. Intravenous (I.V) tranexamic acid was used (900 mg I.V. infusion, first dose 30 min before tourniquet inflation, second dose 3 h later, and additional third dose 6 h after second dose).\nPostoperatively, her hemoglobin dropped from 12.5 g% to 8.6 g% and she was transfused three units of packed cell volume. Intra-operative blood loss was 300 ml. Postoperative drain was kept for 48 h. The total collection in drain was 650 ml. Prophylactically oral rivoroxaban 10 mg (Xeralto - Bayer Zydus Pharma) was given 2 weeks postsurgery, because such patients have increased risk of thromboembolism. Subsequently, she was kept on oral aspirin 75 mg daily for a period of 4 weeks. Patient was kept on intermittent calf pumps for 3 days postsurgery. She was given DVT above knee stocking and encouraged to do active ankle-foot exercises. Aggressive mobilization was used under supervision of a physiotherapist. Continuous passive motion and full weight bearing walking with a walker were started from next day of surgery. She was walking with walker support and had 0–90° ROM by 7th day, when she was discharged. Her wound healed well, and sutures were removed on the 15th day postsurgery.\nAt 1-year followup, her knee society pain score improved to 97 and functional score improved to 100. She was walking normally without pain and had joined her job. She was also able to commute by crowded public trains without any support.\nHistopathological examination of the removed bone and synovial tissue showed vascular proliferation with lymphoplasmacytic exudates. Conglomerate of vessels with thick walls and red blood cells in slit like lumina with hemosiderophages were seen. These findings were consistent with the histological examination of hemangiomas of Klippel–Trenaunay Syndrome. |
A 30-year-old male presented to the ED via emergency medical services (EMS) with right knee pain and swelling after sustaining a fall. The patient stated that he was standing on a conveyor belt on his left lower extremity and then fell, landing with his right knee in varus stress. The fall happened from approximately 3 feet, and upon falling, he felt an immediate pop, accompanied by intense pain that radiated distally down to his foot. The patient reported the severity of pain at onset as 10/10 and was unable to bear weight on his right lower extremity. En route, EMS reported that the patient received 100 mcg of intravenous (IV) fentanyl, with improvement in pain from 10/10 to 2/10, as well as a 500 cc bolus of IV saline.\nThe patient had a temperature of 36.5°C, with a blood pressure of 135/85 mmHg and a pulse of 53 beats per minute. Electrocardiogram (ECG) showed slight sinus bradycardia but the patient was asymptomatic. Oxygen saturation was 95% on room air, and respiratory rate was 14 breaths per minute. Though otherwise healthy, the patient did admit to smoking a pack of cigarettes every day. Physical examination showed no abnormalities outside of the affected extremity. Examination of the right lower extremity showed moderate swelling over the right anterior knee with moderate tenderness to palpation over the right anterior and right lateral knee. There was moderate tenderness to the right proximal fibula, but the anterior compartments were soft, and skin was intact. The patient’s calf was not tender. His right foot was diffusely tender to palpation, but otherwise had no swelling, erythema, or ecchymosis. The patient was able to move all digits and his dorsalis pedis and posterior tibial pulses were equal bilaterally. On admission, the patient was agitated but otherwise alert and oriented.\nX-ray of the right femur showed right tibial and fibular fractures without femoral fracture. X-ray of the right foot showed no evidence of acute fracture or dislocation. X-ray of the right tibia fibula showed comminuted proximal tibial and fibular fractures with evidence of intra-articular extension, right knee joint effusion, and lipohemarthrosis (Figure ).\nA CT of the lower extremity without contrast yielded evidence of a complex comminuted depressed fracture of the proximal tibia, involving mainly the lateral plateau, as well as a fracture of the fibular head. Moderate lipohemarthrosis and soft tissue edema was also identified (Figure ).\nThe patient was diagnosed with a right proximal intra-articular tibial fracture (tibial plateau fracture), right knee effusion, right proximal fibular fracture, and lipohemarthrosis. Re-evaluations of the patient at first revealed no signs or symptoms of compartment syndrome, but by the fourth re-evaluation, the patient presented with anterior compartments of the right lower extremity warm and firm to touch. The extremity was placed in an increased level of elevation and was iced, and a CT without contrast was ordered following observed signs of early compartment syndrome. At the time of the fourth re-evaluation, the patient’s compartment pressure was measured at 62 mmHg. ED staff continued to assess the compartment pressures of the patient with a commercially available manometer (Table ).\nFollowing a diagnosis of early compartment syndrome in the patient, an emergency right lower extremity four-compartment fasciotomy was planned and the operating room (OR) team was called in. A multi-planar knee-spanning external fixator system was placed for soft tissue and bone stabilization. The patient returned to the OR on day 3 and 4 for repeat irrigation and debridement of the fasciotomies, as well as removal of the external fixator and open reduction internal fixation of the patient’s tibial plateau fracture and fasciotomy closure. Antibiotics were continued for 24 hours following the completion of the patient’s fasciotomies. The patient remained in the hospital for ten days improved pain control and rehabilitation. He was discharged home with home health care. |
A 20-year-old collegiate football lineman was blocking during practice, when he was forced from his left side over an extended right leg, creating an internal rotation and adduction moment of the hip. He felt a ‘sliding and crunching sensation’ as he was driven over his right hip. He fell to the ground, and immediately had pain in his right hip. Initial evaluation on the field by the training staff and physician revealed that the hip had full motion, but there was pain with rotation of hip and inability of the athlete to bear weight. The patient was transferred to the emergency room for radiographs and further evaluation.\nIn the emergency room, the athlete complained of further pain in the hip which was increased with hip rotation. Initial radiographs consisted of pelvis anteroposterior view and lateral view of right hip []. The radiographs did not reveal any fracture or dislocation. With his persistent symptoms and continued pain with rotation, magnetic resonance imaging (MRI) was obtained to rule out an occult fracture within three hours of the initial injury. The MRI revealed a small hemarthrosis and a posterior labral tear, but there was no iliofemoral ligament tear or bone edema in the acetabulum or femoral head []. The patient was discharged with instructions to not bear weight on the right hip, and use crutches.\nThe athlete was re-evaluated the next day and found to have continued tenderness with internal rotation and with stressing of the posterior capsule and labrum. He continued to feel a ‘sliding sensation as if (his) hip was popping out,’ although this was not reproducible on examination. A diagnosis of traumatic hip subluxation was made based on his original injury, continued feelings of slipping, and his examination findings. The athlete was placed on hip precautions (patient was instructed to limit internal rotation of the hip, limit adduction of hip, sleeping on back with pillows between the leg) and continued on non-weight bearing, with crutches, for six weeks.\nOver the course of this time, the athlete’s symptoms and capsular signs resolved. Radiographs (AP pelvis, AP and lateral hip films) at six weeks were unremarkable. An MRI was obtained to search for signs of avascular necrosis (AVN) and to determine the safety of a possible clearance for return-to-play. An increased signal was observed in a well-localized area in the superior anterior aspect of the femoral head near the fovea on the T2-weighted images []. The location was atypical for AVN, but the lack of edema in the same area immediately post injury made these changes worrisome. The differential diagnosis was atypical AVN (by location) or an atypical femoral head bone bruise (by the lack of edema on the initial MRI). On account of the uncertainty of diagnosis the patient was continued on toe touch weight bearing for a further six weeks, but allowed gentle range of motion exercises with the trainers. The patient reported compliance with all physician instructions.\nAn MRI was obtained three months after injury []. The lesion on T2 weighted images was still present in the same location and had increased in size and signal intensity. A CT scan was performed to better delineate this lesion and revealed a posterior lip acetabular fracture [].\nThe patient remained on restricted activities, but was allowed to start range of motion activities and non-impact conditioning under the supervision of the athletic trainers.\nSubsequent MRIs at five and 10 months post injury revealed progressive improvement with almost complete resolution of the abnormal signal intensity []. The athlete progressed with training under supervision after his five month MR, and continued, within two months, to unsupervised training for football without any episodes of instability, clicking or pain. He returned to competitive football the following season, 12 months after injury without incident. The final diagnosis was hip subluxation with bone bruise of the femoral head that was not seen on the acute MRI. The subject was informed that data concerning the case would be submitted for publication. |
A 90-year-old female was admitted to the chronic care hospital. The patient lived with her husband and was independent regarding cooking and feeding. She had a history of fracture of the right ankle for which she underwent an operation six years before. Though muscle of bilateral lower extremities was gradually getting weak since this episode, her indoor ambulation was sufficiently independent with the aid of a rehabilitation walker. However, she required support from either her husband or a wheelchair for ambulation at home due to the pain in the right knee related to gonarthrosis. She did not take any analgesic for the pain in the right knee. The patient had fallen while moving from a toilet to a wheelchair; thereafter, she was unable to transfer herself to the toilet due to anxiety about a repeated falling episode. As her ability to transfer to the toilet declined, incontinence recurred. She was, therefore, admitted to the hospital for rehabilitation therapy to regain independence regarding toileting and indoor ambulation. The patient had anemia, atrial fibrillation, and stomach cancer in the early stage. As the patient did not wish any treatment for the stomach cancer, the stomach cancer was under observation. She was taking iron supplementation, lansoprazole 15 mg, and verapamil 120 mg per day. On admission, the patient was examined by both occupational and physical therapists. Her height was 155 cm, and her body weight was 56 kg (body mass index = 23.3). She could not extend fully bilateral knees due to pain and muscle weakness and her back was kyphotic. However, the patient could stand still with that posture. She was able to walk only for approximately 6 m on parallel bars. After excretion in toilet, she could not lift up the slacks to the west. She had a score of 30 on the revised Hasegawa’s Dementia Scale, while the initial functional independence measure (FIM), motor, and cognitive subtotal scores were 59, 32, and 27 respectively (Table ); her Barthel Index (BI) on admission was 45. The scores regarding ambulation, grooming, and toilet activities were severely reduced (Table ). Rehabilitation therapy for ambulation and independence of toileting was initiated. A physical therapist trained the patient 60 minutes a day and an occupational therapist trained her 40 minutes a day. Rehabilitation therapy was performed four to five days a week. During rehabilitation therapy, the patient needed rest due to irregular pulse, but the symptom disappeared within a few minutes. Three weeks after the initiation of rehabilitation therapy, the patient was able to walk for approximately 24 m on parallel bars. As the standing position of the patient started to be stable, the patient was able to walk for several meters with the aid of a rehabilitation walker. She started also to succeed to lift the slacks to the west after excretion in toilet. The vital signs of the patient were almost stable as systolic blood pressure of 95-110 mmHg and pulse of 70-80 per minute. However, a cluster of COVID-19 infections occurred at the hospital. All infected patients were transferred to other hospitals, and although the patient was not infected, rehabilitation therapy was ceased under the public health administration to avoid the risk of spreading COVID-19 through rehabilitation therapists. During the period of therapy suspension, the patient could only transfer to the toilet and dining hall with the aid of a wheelchair, while continuing herself to exercise by lifting bilateral legs while lying in the bed several times a day. During the cessation period of rehabilitation therapy, the patient started complaining of muscle weakness of the lower extremities. Thus, the patient required a flip-up wheelchair when she moved from the bed. Rehabilitation therapy was resumed two weeks after the last COVID-19-positive patient was identified; therefore, she did not receive rehabilitation therapy for a total of 25 days. The patient was re-evaluated when rehabilitation therapy was reinitiated and FIM and BI were assessed. The patient was not able to stand stably. The patient was able to walk for less than 6 m on parallel bars. The patient reported lethargy during therapy, and required additional support to get up from the sitting position; she also mentioned shortness of breath and an irregular pulse during toilet activities. When she complained of these symptoms, systolic blood pressure was 110-120 mmHg and pulse was 79-106 per minute. These symptoms did not disappear for more than 5 minutes even with suspension of rehabilitation therapy, which was not observed in the patient before the cluster of COVID-19 infections had occurred. However, the patient did not complain of lethargy outside of rehabilitation therapy. The FIM, and motor and cognitive subtotal scores were 54, 29, and 25, respectively (Table ); the BI score was 40 (Table ). The transfer scores decreased in both assessments, while the scores for comprehension and memory in the FIM also slightly decreased. At the time of writing, the patient continued to undergo rehabilitation therapy. The patient demonstrated recovery and was able to walk for 21 m with gait training on parallel bars, three weeks after reinitiating rehabilitation therapy. Shortness of breath or any irregular pulse did not last for more than 5 minutes as the patient's performance ameliorated with rehabilitation therapy. However, she did not achieve the goal of rehabilitation therapy to be independent of toileting. |
The patient in question was a 45-year-old Hispanic female who had lived in Canada for the previous 11 years. She reported a long history of manual labour and subsisted on similar occupations since arriving in Canada. At the time of her presentation, her occupation required prolonged periods of standing. The patient's chief complaint was chronic low back pain. There was no antecedent trauma, bone surgery, or history of cancer. The onset of low back pain was described as insidious, with constant achy pain of at least two years duration which was progressively worsening. The pain remained localised to the central lower back in the area of the lumbosacral junction. The pain was rated at 3/10 (verbal scoring) at its best and 8/10 (verbal scoring) at its worst. An increase in pain was associated with an increased level of physical activity during the day, with the pain typically worse in the evening. During periods of increased pain, there were intermittent incidences of pain radiating to the right-sided posterior thigh and leg with "pins and needles" in the lateral toes of the right foot. Treatment to date had consisted of non-steroidal anti-inflammatory medication, which she felt had been of limited benefit. There had been no use of acupuncture, massage therapy, therapeutic exercise, or any manual therapies for this condition. The patient was referred to the department of Medical Imaging for evaluation of chronic lower back pain.\nThe patient reported that no spinal imaging had been performed in investigation of these complaints. This was confirmed by a review of the patient's records.\nImaging specialists typically do not examine patients who are referred into an imaging department for investigation. As such, no physical examination was performed in this case. Permission was granted after the images were interpreted only to interview the patient for this case report.\nA three-phase bone scan was performed with 99 mTc(Technetium)-MDP (methylene diphosphonate) including blood flow and blood pool imaging followed by a delayed whole-body scan. SPECT/CT imaging centered over the lumbar spine was subsequently performed on a Symbia T6 (Siemens), a dual-head gamma-camera incorporating a low-dose 6-slice non-contrast enhanced CT (12 mAs, 130 kVp, Effective Dose < 4 mSv). The CT scan duration was less than 1 min. Overall, the SPECT/CT scan duration was about 20 min. The SPECT/CT fused images were displayed on the e-soft 2007 workstation (Siemens) in axial, sagittal, and coronal slices.\nThe blood flow and pool images were unremarkable, suggesting no active inflammatory process. The delayed whole-body images showed degenerative changes in multiple sites in the axial and appendicular skeleton. Heterogeneous tracer uptake was noted at multiple spinal levels with marked increased focal tracer uptake in the right lateral aspect of L3 (Figure ).\nSPECT/CT images confirmed intense tracer uptake in the right L3/L4 facet joint (Figure ). In the absence of other osteoblastic or osteolytic pathology, this is most consistent with active degenerative osteoarthrosis. Mild tracer uptake is also noted in the right facet joint of L5/S1 in the presence of marked degenerative arthrosis, which is consistent with limited active bony pathology. The low-dose CT images from the SPECT/CT are not of diagnostic quality but they are adequate for anatomical localisation and gross tissue evaluation. In reviewing the axial CT images, there are many abnormalities to note. At the level of the L3/L4 right facet joint, there are only mild indicators of degenerative joint disease: focal joint space narrowing and early sclerosis (Figure ). In addition to marked degeneration of the right L5/S1 facet (Figure ), there is a left-sided discontinuity of the pars interarticularis and an incidental spina bifida occulta at L5 (Figure ). |
We report the case of a 31-year-old Caucasian woman, gravida 3, para 1, who was referred after a second trimester fetal anatomy screening at 20 weeks gestational for a suspicion of a complex fetal cardiac malformation, for which several specialized opinions tried to reach consensus.\nThe obstetrical history of the patient includes a previous Caesarian section with a normal course of parturition and a spontaneous miscarriage. The current pregnancy presented a low risk for aneuploidy according to the performed cell-free fetal DNA test. The classical karyotype performed after the abortion did not reveal any chromosomal abnormalities.\nPrevious ultrasound evaluations were incongruent and reported the following findings:an isolated aortic arch anomaly (supposedly aneurysmal dilation from which the left common carotid artery emerges) and coarctation of the aorta with the anterograde flow; ventricular septal defect, coarctation of the aorta, and a vascular formation located superior from the aortic arch with the appearance of an arteriovenous fistula; aneurysmal dilation located above the pulmonary trunk bifurcation and a dilated left common carotid artery with a retrograde flow; minor ventricular septal defect with a normal ductus venosus triphasic flow.\nWe performed fetal echocardiography, which demonstrated a mild cardiomegaly with a left deviated 72-degree heart axis, normal aspect of the four-chamber view, a small membranous ventricular septal defect, and ductal aortic coarctation; the ductus venosus flow was normal (, and ). In addition, we identified an aneurysmal structure measuring 1.63/1.25/1.16 cm with turbulent Doppler flow, situated above the emergence of the pulmonary trunk and continued by a dilated vascular structure that bifurcates in the cervical region; the aneurysm seemed connected to the left pulmonary artery as well. A dilated left subclavian artery was also suspected (, and ).\nIn the context of complex cardio-vascular malformations, the patient requested the termination of the pregnancy by drug-induced abortion.\nThe hands-on dissection of the fetus revealed a set of abnormalities that could stand as an anatomical basis for what has been found during the ultrasound examination.\nThe first and the most pronounced aspect was the distention of the whole venous system of the neck and mediastinum. The specimen presented a linguo-facial vein that described a rather sinuous pathway alongside the inferior margin of the mandible (). Both the linguo-facial vein and the external jugular vein appeared with a markedly increased caliber, around 4–5 times larger than expected for this gestational age. Both left and right jugular veins and the right subclavian vein were assessed as three times larger than usual, respecting the normal relations to the neighboring structures ( and ).\nThe confluence between the left jugular and subclavian vein into the left brachiocephalic vein was observed to be very dilated to superior and inferior, extending above the superior margin of the omohyoid muscle as well as below the inferior concavity of the aortic arch. Moreover, on the inferior side of the enlarged brachiocephalic vein, a vessel could be observed descending lateral to the left vagus nerve and communicating with the left pulmonary artery. The left pulmonary artery was observed to be dilated as well, around twice as normal ().\nRegarding the great vessels of the heart, there are some anomalies to be discussed. A narrowing of the aortic arch was identified distally to the emergence of the left subclavian artery ( and ). A large, patent ductus arteriosus was found, ending right at the narrowing level observed in the aortic arch (ending right at the coarctation level) (). The left subclavian artery was dilated as well, sizing as much as the ascending aorta and the aortic arch, creating the illusion of a terminal branch rather than a lateral one ( and ).\nHeart analysis concluded no distinct changes in heart architectural formation for this gestational age. Atrioventricular and ventriculoarterial concordance was noted. Atria and ventricles were increased in relation to the mediastinum. Surprisingly for an aortic coarctation, the right atrium was not found to be enlarged. |
A 54-year-old female patient came to dental office to improve her smile. There were no relevant medical history and no contraindication to dental treatment.\nThe extraoral examination indicated a symmetrical and mesognathic facial pattern with a convex profile. At rest, there were about 2 mm of incisal edge visible with slightly incompetent lips.\nOn the intraoral objective examination, the patient presented an all-ceramic crown on the element 16, two crowns screwed onto implants at the sites of the elements 35 and 36, and Miller's I class gingival recessions on the teeth 11, 21, 37, 34, and 45. The patient's biotype was thick, and there were no periodontal problems (). The movements of laterality and protrusion had no working and nonworking precontacts. The overbite was 3.5 mm and the overjet 1 mm.\nThe patient presented a canine and molar 1st dental class. The lower midline coincided with the facial median while the upper midline was displaced 2 mm to the right. The smile line was medium, the incisal trend compared to the lower lip was convex, the lip was 1 mm apart, the dental exposure was 8 teeth (from 14 to 24), and the labial corridor was normal both on the right and on the left.\nFrom the esthetic analysis of the profile, an adequate support of the upper lip was observed for the correct inclination of the upper incisors. At the dental esthetic analysis, the inversion of the dental axis of teeth 12 and 22 was found. Problems were present at the level of the gingival margins and at the embrasures lines. There were diastemas between all maxillary teeth with the exception of the space between 11 and 21.\nA surface of wear was found on the incisal edge of tooth 21, and there was the presence of white spots on 12, 11, 21, 22, and 23.\nSubsequently, posterior radiographs were performed to assess the presence of interproximal carious lesions, photographs were taken, and dental impressions were taken in order to make a diagnostic wax-up. At the time of the reevaluation, the presence of carious and periodontal lesions was not found. The diagnosis included the presence of diastemas between the upper front teeth, the incorrect position of the elements 12 and 22, the presence of white spots, the nonideal proportion of the elements 11 and 21, and the noncoincidence of the facial midline with the upper dental midline.\nThe treatment of the white spots could have been performed using infiltrating resin (Icon, DMG) resin with a superficial [] or deep [] treatment. However, the patient wanted a change not only in the color and surface texture of his teeth but also in morphology.\nIt was decided to treat the upper anterior teeth with a minimally invasive multidisciplinary orthodontic-prosthetic approach. The proposed treatment plan was to move the upper teeth to redistribute the diastemas between 14 and 24, restore a correct surface texture, and restore the position of the upper dental midline and the proportion of the teeth using feldspathic ceramic veneers.\nOn the study models originated by preliminary impressions, the technician performed an initial wax-up () on which he fabricated a silicone template (Vestige 70 shore, Trayart) to print the preliminary mock-up in the patient's mouth.\nThe flowable composite resin is then dispensed in to the silicone template and positioned in the patient mouth (). The previsualization provided a true copy of the planned wax-up allowing for an immediate evaluation of shape, volume, occlusion, and relationship with the surrounding tissue. The patient thus accepted the treatment plan. Impressions in polyvinylsiloxane (Aquasil, Dentsply) were taken, and a teleradiography and an orthopantomography were performed. X-rays and impressions were sent to the Invisalign® (Align Technology) center together with the orthodontic treatment goals.\nThe cephalometric radiography study has allowed to appreciate the following: severely brachifacial growth type; 1st skeletal and dental class; poor sagittal development of the maxilla; and increased mandibular development.\nThe orthodontic treatment of the upper arch alone was carried out using transparent aligners (), in order to redistribute the diastemas present in the superior frontal group so as to uniform the spaces, center the medians, and facilitate the subsequent prosthetic rehabilitation of the same. In addition to aligning the upper frontal group from 13 to 23, derotation of the 23 and correction of the distal tipping were performed.\nAfter six months, the orthodontic treatment was completed (), alginate impressions of the dental situation with the redistributed diastemas were taken. The technician developed class IV plaster models and made the final wax-up on them (), making a silicone template for the definitive mock-up. The mock-up was printed in the patient mouth after performing computerized anesthesia. The preparation of teeth 13, 12, 11, 21, 22, and 23 was performed through the mock-up so that they could be minimally invasive and preserve the greatest possible amount of enamel () []. In the mesial parts of the elements 14 and 24, two direct composite resin restorations were performed. Once the teeth were prepared, the retractor fibers (Retraction Cord #00, Ultrapak™) were positioned, and an impression in polyether (Impregum Penta, 3M ESPE) was taken. The mock-up was printed again on the prepared teeth, and it was finished and left in the patient's mouth as a temporary (). The dental technician received the impressions, developed the model, and stratified sintered feldspathic veneers from tooth 13 to tooth 23.\nAfter 7 days, ceramic veneers were made, and the patient returned at the dental office. The feldspathic ceramic veneers were etched with 9% hydrofluoric acid from 60 to 90 seconds, rinsed, and then cleaned with pure alcohol for 5 minutes. The silane was placed on them, and then the adhesive was brushed on without light curing. The prepared teeth were etched with 35% orthophosphoric acid for 30 seconds and rinsed for 30 seconds, and then the primer and the bonding were applied on without light curing. Resinous composite cement (Variolink Esthetic, Ivoclar Vivadent) was applied, the veneers were positioned on the teeth, and the cement excesses were removed.\nEach veneer was light-cured for 120 seconds.\nFinishing was carried out with fine-grain diamond burs and decreasing abrasiveness rubber burs. The patient returned at the office every six months for maintenance calls, and 3 years after the cementation, the control photographs and radiographs were taken (). |
A 66-year-old male with a history of Hepatitis C treated in 2009 with Ledipasvir/Sofosbuvir (Harvoni®) initially presented to the Medical Oncology team in Hackensack University Medical Center, NJ with abdominal discomfort and distention for 45 months associated with an approximate 15 pounds of weight loss. He underwent CT scan of the abdomen with contrast and was found to have a large 15.0 × 11.0 × 13.0 cm heterogenous mass attached to the left lobe of the liver, with compression of surrounding intra-abdominal contents including the stomach and transverse colon, as well as the abdominal wall (Figure ). Subsequent percutaneous biopsy was performed for genetic testing, and results demonstrated hepatocellular carcinoma with extensive necrosis. He was then referred to the Surgical Oncology team for further workup and management. Follow up MRI demonstrated two additional satellite lesions consistent with multifocal hepatocellular carcinoma without evidence of invasion into surrounding vital structures. Surgical intervention was deemed necessary given the size of the mass and symptomology. A baseline AFP was 6.1, he was designated as a Child Pugh Score of 8 and he had a MELD Score of 9.\nIn the operating room, two large-bore intravenous catheters were placed but fluid was kept at a low rate along with placement of a foley catheter. A laparotomy was performed; making a midline sub-xiphoid incision, which was extended down to the pubic symphysis due to the size of the tumor (Figure ). Multiple friable blood vessels were noted at the umbilicus. The omentum as then resected, freeing the anterior abdominal wall. The transverse colon was then carefully separated away from the tumor using a GIA stapler. The transverse colon and its mesentery were preserved and protected from the dense mass. It was also noted that the distal stomach was adhered to the mass along the greater curvature. A sleeve gastrectomy was also completed, requiring three firings for completion.\nAt this time, the retroperitoneal attachment to the duodenum was carefully dissected, followed by separation of the pancreatic head. This allowed the tumor to be separated from the retroperitoneum and lifted out of the abdomen. A Pringle maneuver was performed using a vascular clamp, and the satellite lesions were transected using parenchymal ablation using the NeuWave Microwave Ablation system. The midline laparotomy was closed in the usual fashion.\nOn gross inspection, the resected specimen was a 21.0 × 20.0 × 10.7 cm, tan-yellow mass weighing 1948 g (Figure ). The tumor is partially adhered to a portion of the stomach and surrounded by adjacent background liver. Microscopically, the tumor shows solid and trabecular growth pattern with round nucleus, prominent nucleoli, abundant cytoplasm and distinct cell border typical for hepatocellular carcinoma (Figure ). Mitotic figures are frequent and tumor necrosis is also seen. Multiple satellite nodules are seen adjacent to the main tumor and extensive small vessel invasion is present (Figure ). The tumor also invades into the gastric wall. The background non-tumor liver shows well-formed cirrhotic liver with mild to moderate activity. The attached portion of the stomach demonstrated negative margins (R0). The specimen from the partial hepatectomy, which was the satellite lesion of segment 3, demonstrated a similar pattern.\nThe post-operative course was significant for urinary retention. Given his history of benign prostate hyperplasia (BPH), a foley catheter was reinserted. He had also developed an ileus requiring a nasogastric tube, which was removed four days later. The patient developed serosanguineous drainage from his incision site after a bout of heavy coughing. A Prevena VAC was placed but was discontinued one week later when the output was reduced to zero, and he was discharged from the hospital later in the day. |
An 81-year-old Japanese woman with a 2-week history of abdominal distension presented to our hospital for assessment. The patient did not have a past history of malignancy, with only a cesarean section as a relevant feature in her history. Endoscopic examination at a previous hospital revealed the presence of early carcinomas in the stomach and distal esophagus. The patient was referred to our hospital for endoscopic resection.\nLaboratory data, as well as serum carcinoembryonic antigen, squamous cell carcinoma antigen, and cytokeratin-19 fragment levels, were close to normal limits. Endoscopic examination revealed mild granular elevated lesions, with slightly depressed irregular mucosa, extending from the anterior wall to the right wall of the distal esophagus (Fig. ). This irregular mucosa further extended from the anterior wall to the left wall, with the boundary on the oral side being unclear (Fig. ). A superficial elevated tumor-like lesion was also observed in the lower body of the stomach, with a diameter of about 10 mm (Fig. ). Based on the endoscopic biopsy specimen, this gastric lesion was diagnosed as a well-differentiated tubular adenocarcinoma. On the other hand, the preoperative biopsy specimens of the esophageal tumor showed intraepithelial tumor cells, which were isolated or in clusters, and consisted of large clear cells with atypical nuclei and prominent nucleoli. No glandular structures and no obvious intracytoplasmic mucin were observed. These histological findings were consistent with a malignant melanoma, with a pagetoid spread of invasive adenocarcinoma or squamous cell carcinoma, and Paget’s disease as a differential diagnosis. Immunohistochemically, the tumor cells diffusely stained positive for CK7 and partially for CK20, with negative staining for S100 protein and HMB-47. On the basis of these results, a diagnosis of malignant melanoma was excluded. All human mucin core proteins examined (MUC2, MUC5AC, and HIK1083) were also negative. Furthermore, p53 overexpression was observed in all tumor cells. From these results, we diagnosed the tumor as Paget’s disease or a pagetoid spread of an esophageal carcinoma. On enhanced computed tomography (CT) and [18F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET)/CT imaging, no lymph node and distant metastases were identified (Fig. ). FDG uptake was observed only in the lower body of the stomach, with these lesions considered to reflect past endoscopic submucosal dissection (ESD) for early gastric cancer (Fig. ). Although we could not define the margin of the tumor, previous reports of esophageal Paget’s disease indicated a wide extension of Paget cells in the esophageal mucosa. On the basis of these findings, we planned ESD for the treatment of the gastric lesion, followed by a thoracoscopic esophagectomy (TE) and hand-assisted laparoscopic proximal gastrectomy (HALPG) for the treatment of esophagogastric Paget’s disease. Histological examination of the ESD specimen revealed a well-differentiated mucosal adenocarcinoma (11 mm × 8 mm) without lymphovascular involvement. The lateral and vertical margins of the resected tissue were free of tumor cells, and ESD was considered as a curative resection.\nTE and HALPG, with lymph node dissection, were performed at 43 days after the gastric ESD. Regional lymph nodes were dissected, with no metastatic invasion identified in the thoracic and abdominal lymph nodes. Reconstruction with a gastric tube was performed after esophagectomy, using a hand-assisted laparoscopy procedure via a post-sternal route.\nHistological examination of the surgically resected specimen was performed. Macroscopically, the mucosa of the lower thoracic and abdominal esophagus was slightly irregular and depressed, with submucosal capillary hyperplasia (Fig. ). No tumor mass or ulceration was observable in the resected material. With iodine staining, the mucosa of the lower esophagus, which was congruous with the irregular and depressed area, did not stain. Furthermore, isolated small iodine-stained foci were observed in the gastric mucosa adjacent to esophagogastric junction (Fig. ). Microscopically, these foci consisted of squamous metaplasia of the gastric mucosa. The sectioned tissues were stained with hematoxylin and eosin (HE) and periodic acid-Schiff (PAS)/Alcian blue. As well, immunohistochemical staining for CK5, CK7, CK20, CDX2, MUC2, MUC5AC, HIK1083, p53, p63, S100, and HMB-45 was performed. Microscopic examination revealed neoplastic cells, with a large atypical nucleus and pale-staining cytoplasm, in the lower part of the esophageal epithelium, occurring either singly or in clusters (Fig. ). Reserve cell hyperplasia (Fig. ) and squamous metaplasia (Fig. ) were observed in the gastric mucosa, adjacent to the esophagogastric junction, and an intraepithelial squamous cell carcinoma (SCC) was observed within the squamous metaplasia (Fig. ). Components of the intraepithelial squamous cell carcinoma were identified following the Paget cells in the esophageal squamous epithelium. Only a few Paget cells stained positively for PAS/Alcian blue. Immunohistochemically, negative staining for CK5 (Fig. ) and p63 was identified in Paget cells, with positive staining for CK7 (Fig. ). The Paget cells showed no reactivity for intestinal mucin (MUC2) and gastric foveolar mucin (MUC5AC), but a few Paget cells were positive for gastric gland mucin (HIK1083). On the other hand, the intraepithelial SCC showed positive reactivity for CK5 and p63, but no reactivity for CK7 and CK20. Overexpression of p53 was observed in both Paget cells (Fig. ) and the intraepithelial SCC. Histochemical and immunohistochemical results are summarized in Table , and schematic representation of the distribution of Paget cells and squamous cell carcinoma of the esophagogastric junction is shown in Fig. . Because there were any findings of Barrett’s esophagus neither endoscopically nor pathologically, macroscopic esophagogastric junction and pathological squamocolumnar junction were identical. Regional lymph node metastases were not identified on pathological assessment.\nAt the last follow-up, conducted 2 years and 8 months after surgery, the patient’s health status was fairly good, with no recurrence of the EMPD or carcinoma.\nEMPD was first described in a patient with urinary bladder carcinoma in 1889 []. Since this initial report, EMPD has been described in various sites of the body, most commonly the vulva, perianal region, scrotum, penis, and axilla []. EMPD is subdivided into primary and secondary types on the basis of the presence or absence of associated malignancies. Primary EMPD is thought to be derived from an underlying neoplastic transformation of the intraepidermal portion of a sweat gland, whereas secondary EMPD is caused by the intraepidermal spread of neoplastic cells, typically derived from an underlying adenocarcinoma [, ].\nEsophageal Paget’s disease is quite rare, with only a few cases having been reported [–]. Yates and Koss [] described esophageal Paget’s disease associated with a poorly differentiated squamous cell carcinoma of the distal esophagus, whereas Norihisa et al. [] reported a case of adenosquamous carcinoma of the esophagus with pagetoid extension of the adenocarcinoma component. Therefore, both of these cases were diagnosed as a pagetoid growth of an advanced esophageal carcinoma. On the other hand, Nonomura et al. [] and Matsukuma et al. [] reported esophageal Paget’s disease associated with an early underlying carcinoma, one being an intraepithelial carcinoma and the other, a minimally invasive adenocarcinoma of the esophagus. Ishihara et al. [] also reported a case of an early invasive carcinoma, which consisted of pagetoid squamous cell carcinoma in situ combined with early invasive components and choriocarcinoma at the metastatic site. Abraham et al. [] reported a close relationship between Paget cells in the esophagus and an underlying poorly differentiated adenocarcinoma in the esophagus or esophagogastric junction. From these reports, all previously reported cases of Paget’s disease of the esophagus were thought to be secondary to an underlying carcinoma, although the malignant component varied in each case. In our case, we identified an SCC component, with squamous metaplasia and reserve cell hyperplasia, in the gastric mucosa of the esophagogastric junction, which was followed by Paget cells. However, unlike typical Paget’s disease, only few Paget cells were positive for PAS/Alcian blue staining and immunohistochemically positive for gastric gland mucin, whereas a strong p53 overexpression was observed in both SCC component and Paget cells.\nReserve cells are small undifferentiated cells found as a single layer beneath the endocervical columnar epithelium. They have the capacity to transform into both endocervical columnar and squamous epithelium in the endocervix []. Reserve cell hyperplasia and epithelial dysplasia are frequently observed in the squamocolumnar junction of the cervix uteri, and squamous cell carcinoma of the cervix uteri is considered to be derived from these changes []. Reserve cell hyperplasia and squamous cell metaplasia of the gastric mucosa are rare phenomena. In 1981, Takubo [] reported the resemblance of squamous cell metaplasia to reserve cell hyperplasia in the cervix uteri and considered squamous metaplasia or reserve cell hyperplasia with atypical change as a precursor of SCC in the esophagogastric junction. However, this hypothesis has not been fully elucidated. From histological findings and immunohistochemical results in our case, we speculate that the Paget cells were derived from the squamous cell carcinoma, developing in the squamous metaplasia and reserve cell hyperplasia of the esophagogastric junction. The difference in the pattern of expression of cytokeratin and p63 might reflect the glandular differentiation of tumor cells.\nFDG-PET/CT imaging is currently accepted as the most accurate technique for exploring metastatic lesions of a solid tumor. The combination of metabolic and structural information provided by the PET and CT portions, respectively, has improved the accuracy of tumor staging, detection of recurrence, and therapeutic monitoring, having an enormous impact on patient management [, ]. In patients with EMPD, 18F-FDG PET/CT diagnosis of primary lesions is mainly dependent on the thickness of the lesions, whereas it is more sensitive for the diagnosis of lymph node and distant metastases []. In this case, thick primary lesions showed an intense uptake of 18F-FDG (SUVmax 14.9 and 7.5), whereas thin primary lesions showed only a mild 18F-FDG uptake (mean SUVmax 3.25 ± 0.24). Three of the 10 cases reported, however, showed no 18F-FDG uptake at primary site, as in our case. In 3 of these 10 cases with lymph node invasion and distant metastases of EMPD were upstaged by PET/CT, rather than conventional staging examination. To determine the appropriate treatment strategy for EMPD based on staging, PET/CT may play an important role, although some EMPD might be 18F-FDG negative.\nTraditionally, EMPD has been surgically managed, especially in the early stage of the disease. Achieving adequate margins for the primary lesions is an important factor in reducing the risk of recurrence. In patients unfit for radical surgery, radiotherapy is proposed as alternative treatment, as long as invasive disease has been excluded []. In the surgical treatment of esophageal cancer, thoracoscopic esophagectomy is generally regarded, and accepted, as a minimally invasive surgery []. Biere et al. reported on the short-term benefits of minimally invasive esophagectomy for patients with resectable esophageal cancer, with prevention of pulmonary infection being an important benefit. Furthermore, thoracoscopic esophagectomy with three-field lymphadenectomy, pursuing best loco-regional control by surgery, is a feasible and safe alternative treatment commonly performed in Japan []. Our case was diagnosed as early stage Paget’s disease of the esophagus by endoscopic, CT, and PET/CT findings. But because of the unclear and extensive proximal margin of the tumor, a thoracoscopic esophagectomy was performed to obtain a wide local excision of the EMPD. However, in the pathological diagnosis, Paget’s disease and squamous cell carcinoma were identified in the mucosal layer. Therefore, curative resection with ESD could have been possible. ESD is an effective treatment for superficial esophageal neoplasms. Funakawa et al. [] reported a success rate of 99.4% (164/165) for en bloc resection and 90.9% (150/165) for complete en bloc resection, with no instance of fatal complications. However, the reported incidence of esophageal strictures after ESD for near-circumferential or circumferential esophageal neoplasms is extremely high at 88–100% []. Post-ESD strictures seriously lower patients’ quality of life, being associated with several symptoms, including dysphagia, nausea, vomiting, weight loss, and even cachexia. In contrast, esophagogastric junction cancers have a high rate of submucosal invasion, irrespective of size, compared to non-junctional cancers []. Furthermore, the rates of positive lymphatic and/or venous invasion were remarkably higher in junctional cancers []. Therefore, when ESD is performed for near-circumferential junctional cancer as in our case, attention must be paid to the occurrence of esophageal stricture. It is important to evaluate the risk of recurrence by pathological diagnosis and to consider whether additional treatment, including surgical resection, should be performed. |
A 61-year-old woman was admitted to National Cancer Center with a persistent headache. Her headache started suddenly about one week before being admitted to the hospital, after a cough. Right frontal area headache was the only symptom, her vital signs were normal and the neurologic exam showed no abnormalities. She wasn't on any medications and had a past history of ovarian cancer (stage IIIc, histologic type: adenocarcinoma, mixed endometrioid/mucinous) that was diagnosed 9 years ago. She underwent an operation and multiple cycles of adjuvant chemotherapy for 2 years from diagnosis. During her follow-up, recurrence was found in the pelvic lymph nodes 2 years ago and she received radiotherapy for 3 months. Since then there was no evidence of disease and she was having regular check-ups on outpatient clinic basis.\nA computed tomography scan showed a lesion in the right frontal area with a 5 cm sized conglomerated high density lesion and a cresenteric low density lesion in the ipsilateral subdural area (). Magnetic resonance imaging revealed a lobulated, contoured, well-defined dural based mass with a central portion of iso-to-high signal intensity in T1-weighted image and low signal intensity in T2-weighted image suggesting acute- to subacute intratumoral hemorrhage (). The cresenteric subdural lesion appeared as a low signal intensity in T1-weighted image and high signal intensity in T2-weighted image which is consistent with a liquefied subdural hemorrhage. On gadolinium enhancement, the mass showed strong homogenous enhancement except for the central portion representing hemorrhage (). Our first impression from these findings was acute an intratumoral hemorrhage and subacute stage subdural hemorrhage in a dural metastatic tumor due to her past history and presentation of hemorrhages which occurs more frequently in malignant or metastatic tumors than in benign tumors []. Meningioma was also the differential diagnosis because of the tumor location and homogenous enhancement. We planned a curative resection of the tumor and adjuvant therapy was undetermined until we received pathology results.\nAn elective operation was planned and performed without any complications. Bicoronal incision was done and an enlarg-ed middle meningeal artery heading towards the tumor was made visible by opening the frontal lobectomy bone flap. Dura mater was incised and the subdural hemorrhage was evacuated (). The subdural hemorrhage was composed of mixed components ranging from a liquefied hemorrhage to a dense acute hematoma (). The surface of the brain contacting tumor was intact and not been invaded by the tumor. At the deepest portion of tumor, a capsule attached to pial surface and cortical vein was observed (). Intraoperative findings suggested that this cortical vein adhered to the tumor arachnoid capsule might be a focus of bleeding in this case. A frozen biopsy of the main mass turned out to be a meningioma but the histologic subtype was uncertain. The pathologist reported tumor involvement of the pia attached to cortical vein. Thus, the cortical vein and underlying pia mater was removed with negative margins. The subdural hemorrhage had a membranous structure and the frozen biopsy suggested tumor involvement (). Therefore, the membranes surrounding the hemorrhage were removed totally and all resection margins were tumor-free, indicating Simpson grade I operation. She experienced an immediate postoperative partial seizure which was controlled with 'add-on' anti-epileptics to preventive one. She recovered without any neurological deficit. Final pathology results revealed a conventional meningioma of fibrous type (World Health Organization grade I) with intratumoral hemorrhage revealed among the thin-walled dilated capillaries (). She was discharged without further event and is now being observed on an outpatient basis. |
A 43 year old female district nurse presented to the accident and emergency department with a 6 hour history of initially generalized abdominal pain which was localizing to the right. This was associated with nausea and loss of appetite, made worse by movement and not relieved with intramuscular morphine injection. There was no history of recent or past trauma to the chest or abdomen. On examination, she was tender in the right upper quadrant and right iliac fossa with rebound and guarding, with a Rovsing positive sign and normal bowel sounds. She was incidentally found to have some marfanoid features including the long span of upper limbs, a high arched palate and the very soft early diastolic murmur of aortic regurgitation. The patient had a positive family history of Marfan's syndrome but had never undergone genetic testing to confirm the diagnosis.\nA likely diagnosis of appendicitis was made on the clinical picture associated with a pyrexia and raised white cell count and C – reactive protein. Her abdominal X-ray at that time showed absence of gas in the right side of her bowel. Her chest X-ray did not show any obvious abnormality (Figure )\nShe was taken to theatre the same day for appendicectomy through a standard right iliac fossa incision. There was a small amount of free fluid but the right iliac fossa was filled with a normal looking gall bladder and small bowel only with no sign of the caecum, appendix, ascending or proximal transverse colon. The terminal ileum was found to run up under the right lobe of the liver towards the hilum. The right iliac fossa wound was closed and we proceeded to an exploratory laparoscopy.\nAt laparoscopy, the liver was retracted to see under the right liver lobe. The proximal transverse colon was found to be running up towards the liver hilum were it felt to be tethered. This suggested a possible herniation of the right colon into the chest. The procedure was abandoned and a CT thorax and abdomen were performed to define the anatomy involved.\nThis (Figure , ) demonstrated bilateral pleural effusions of moderate size with underlying unexpanded lungs. The liver had what appeared to be a large Reidl's lobe. Behind the right liver lobe, the right kidney was markedly elevated and the ileo-caecal junction appeared to lie between the liver and the kidney. The right side of the colon appeared to lie above the liver. Three dimensional reconstruction of the scans demonstrated absence of the right hemidiaphragm. (Figure , )\nThe symptoms and signs persisted and a laparotomy was performed. At laparotomy, there was some free fluid. There was definitely a large defect in the right hemidiaphragm. The right liver lobe had grown right up into the right intrathoracic space. There was a large hernial sac beneath the right lobe of the liver between the common bile duct, duodenum and liver, displacing the right kidney medially and containing transverse colon, terminal ileum, caecum, appendix and free fluid. The contents of this sac were successfully reduced and a perforated gangrenous appendix with pus was found within the intrathoracic hernial sac. A standard appendicectomy was performed. The right colon was formally mobilized, fully reduced and the caecum was fixed within the right iliac fossa.\nThe patient was admitted to the intensive care unit postoperatively where she made a good recovery. She had instant relief of her abdominal symptoms and post-operative contrast study demonstrated the presence of the right colon within the abdomen (Figure ). She was discharged a week post-operatively. |
A 40-year-old woman presented with a headache and was found to have a sinusitis-related headache pretreated with antibiotics. On admission, she was found to have random venous glucose of 33 mm/L and glycated hemoglobin (HbA1C) of 13.1%. (Table ). She had a significant family history of diabetes, and her father was diagnosed to have diabetes mellitus (DM) before the age of 40. Her weight at baseline was 48 kgs, with a calculated body mass index of 18.5. Detailed history revealed that she did have mild polyuria and polydipsia with the onset of sinusitis-related symptoms. Her vitals were stable, and the initial physical examination was unremarkable. She was started on oral hypoglycaemic agents on admission but soon failed despite adequate doses and was diagnosed with type 1 diabetes mellitus. She was continued on her antibiotics for the treatment of sinusitis and commenced on insulin glargine 16 units at night, glulisine five units three times before meals, and metformin. She was discharged promptly after three days as she recovered from her sinusitis and was competent with the self-administration of insulin.\nOn Day 4, she was readmitted, as she developed generalized swelling. It started off with lower limb swelling and progressed to develop discomfort over her groin and lower abdominal area due to swelling. This swelling swiftly progressed to involve the upper limbs, abdomen, and torso the following day. She remained ambulant and did her usual activities and was compliant with her medications. She was not taking any other herbs or over-the-counter medicines when she went home. Her vitals were stable and her weight increased to 55 kgs by this time. Physical examination revealed a generalized edematous woman with marked pitting edema extending over all four limbs and face. Her heart sounds were dual and lung sounds were clear. The examination of her abdomen was unremarkable, apart from the soft tissue swelling. The examination did not reveal a shifting dullness, organomegaly, or lymphadenopathy although the patient reported an increase in her abdominal girth. She was started on gentle diuresis and with the working diagnosis as fluid overload while investigations to rule out renal complications were underway. Investigations (Table ) revealed hypoalbuminemia on the first admission.\nHer renal function tests, liver transaminase, 24-hour urine protein, and thyroid function were all within acceptable ranges. Transthoracic echocardiogram demonstrated a normal left ventricular function with an ejection fraction of > 60%, without diastolic dysfunction and absent regional wall motion abnormalities (Figure ).\nHer chest X-ray showed a normal-sized heart without signs of fluid overload (Figure ).\nA contrasted computed tomographic (CT) scan of her abdomen and pelvis was performed to rule out an obstructive cause, as the swelling started in both her lower limbs. The CT scan revealed bilateral pleural effusions with a sliver of ascites and marked diffuse subcutaneous edema (Figure ). Notably, her liver was normal on imaging.\nOther blood tests for retroviral and autoimmune causes were all unremarkable. Her weight continued to rise to a maximum of 56 kgs (8 kgs above her baseline weight of 48 kgs) despite salt and fluid restriction, and she was subsequently started on low-dose spironolactone. By now, the results of the initial investigations were largely unyielding, hence, the possibility of insulin edema by a diagnosis of exclusion was considered. This led to withholding rapid-acting insulin and starting of both dapagliflozin and spironolactone.\n \nTwo days after stopping insulin, there was symptomatic relief and resolution of anasarca with her weight down-trending. She was discharged and reviewed in the outpatient clinic one week after with her weight returning to the baseline of 49 kgs and was asymptomatic. Her insulin was restarted and diuretics were discontinued after two weeks. She did not experience any recurrence of edema thereafter. |
A 69-year-old male presented with a long-standing history of nausea, vomiting, abdominal pain, and weight loss. Prior to this presentation, he was under the care of multiple gastrointestinal physicians for diet intolerance, bloating, and intermittent abdominal pain, which was attributed to diverticulosis of the small bowel. History also included intestinal malrotation and a wandering liver diagnosed from computed tomography (CT) scans. The liver on CT scans was demonstrated to be located to the left and right of the midline on different occasions (Fig. a–c). A diagnostic laparoscopy for presumed free intraperitoneal air 6 years prior also reported the presence of the liver on the left side. The intestinal malrotation was not addressed surgically at that point in time. The patient was transferred to our institution with a diagnosis of small bowel obstruction (SBO). An abdominal CT scan revealed distension of the stomach and small bowel with decompression of the mid-distal ileum and colon as well as a mesenteric volvulus. (Figure ) The liver and gallbladder were located in the left upper quadrant and medially displaced relative to its position (to the right) noted from a prior CT scan performed 2 years earlier.\nA decision was made to proceed with a diagnostic laparoscopy on the basis of a midgut volvulus, intestinal malrotation, and wandering liver on radiologic studies. Initial inspection revealed the liver and gallbladder to be located in the left upper quadrant in direct contact with the left abdominal wall. (Fig. a) Neither organ showed any obvious pathology. The stomach was found to be mildly dilated and located below the liver, with the pylorus oriented to the right upper quadrant. Dilatation of the proximal small bowel and multiple large diverticula arising from the duodenum was also evident. The transverse colon was located in the right upper quadrant, with the descending colon traversing down to the suprapubic area. The duodenum was also visualized in the right upper quadrant, containing numerous large diverticula without signs of active inflammation. The small bowel was run and rotated in a counterclockwise fashion. A volvulus of the mesentery was evident in the mid to distal small intestine, which was subsequently reduced. During counterclockwise rotation, the small bowel was placed in the right side of the abdomen with the large bowel moved to the left abdomen. A fibrotic tissue, corresponding to a Ladd’s band (Fig. b), was visualized crossing along a transverse plane in the lower abdomen and was taken down with ultrasonic scissors to the root of the mesenteric vessels. The small intestine was run again to verify the reduction of the volvulus and the absence of any twisting. The final procedure performed was an appendectomy.\nPostoperatively, the patient experienced an ileus and recovered uneventfully while regaining bowel function and tolerating diet by postoperative day 3. At 2 weeks of follow-up, resolution of symptoms was demonstrated.\nA wandering or hypermobile liver in both the adult and pediatric population is rare in nature and the reported literature. A total of 11 cases since 1960 have been published in medical journals. Various distinct pathologies are associated with this condition in volvulus and/or obstruction of the stomach and bowel due to a persistent ventral mesogastrium [, ]. To our knowledge, this is the first reported occurrence of a concurrent wandering liver and intestinal malrotation in an individual. The incident was additionally complicated by a midgut volvulus with diverticulosis in full length of the small bowel.\nThe hepatic suspensory apparatus, which suspends the liver from the diaphragm and maintains the liver in the right upper quadrant of the abdomen, is made up of the coronary, triangular, and falciform ligaments []. A persistent ventral mesentery is known to be associated with malrotation and volvulus; however, because the ligaments of the hepatic suspensory apparatus are derived from the ventral mesentery before it fuses with the peritoneum, it has been proposed that a persistent ventral mesentery could represent an arrest in the normal development of these ligaments, which predisposes them to laxity, allowing the liver to “wander” [, ].\nIntestinal malrotation diagnosed at adult age is a rather rare finding and typically asymptomatic. Authors Nichols et al. [] proposed an embryologic error that may result in the laxity or absence of both hepatic and colonic suspensory ligaments in the setting of colonic volvulus. We agree on this notion of developmental error; however, present literature does not offer any link of hepatic hypermobility to intestinal malrotation or volvulus. Nonetheless, malrotation is reported in 1:500 live births before one month of age with, essentially, no mention of a wandering liver []. The impression that a wandering liver is an independent entity as opposed to a clinical sign in a syndrome requires more reported incidences for epidemiologic investigation.\nIn adult life, intestinal malrotation is generally an incidental finding on CT [] and exceptionally infrequent in the setting of a wandering liver. This combination can be discovered during abdominal surgeries for different reasons or present as bowel obstruction. Recurrent episodes of bowel obstruction can occur with the potential of partial or complete volvulus. When symptomatic, prompt intervention (which could be performed laparoscopically) is indicated. Thus, a systemic approach to intestinal malrotation with a wandering liver is crucial in recognizing abnormal anatomy (i.e., duodenal obstruction) especially when approached laparoscopically.\nIn this case presentation, the preoperative CT scan demonstrated the liver to be midline of anterior abdomen. Upon insufflation and entering the abdomen laparoscopically, the liver was positioned in the left upper quadrant. With manipulation of the liver and positioning of the patient on the operating table, the unimpeded laxity of the hepatic ligaments was manifested within the upper abdominal cavity. This may indicate the competency of the liver to wander in abbreviated times as opposed to a long migratory process. Although not established, the short wandering time may increase predisposition to bowel obstruction in the presence of intestinal malrotation. Figure exhibits liver migration within years of each other. However, on basis of laparoscopic exploration, a wandering liver suggests the ability to migrate in varying locations within hours. Hepatic displacement appears to be independent of any intra-abdominal pathology, i.e., bowel obstructions.\nIn the treatment approach of concurrent intestinal malrotation and wandering liver, identifying Ladd’s bands is critical for long-term success. The Ladd procedure remains the initial and mainstay procedure for intestinal malrotation in reducing any volvulus, dividing mesenteric bands, and performing an appendectomy. Compared to the open approach, laparoscopic correction may result in a shorter hospital length of stay and incite fewer adhesions with improved outcomes in postoperative pain. |
A 52-year-old male with a 30-year history of chronic low back pain of unknown etiology presented to a rheumatology clinic. He noted low back pain associated with morning stiffness that worsened throughout the day. He also described increased difficulty with ambulation associated with bilateral paresthesia of the lower extremities, saddle paresthesia, and increased urinary urgency. His past medical history was significant for hypertension, hyperlipidemia, and tonsillectomy as a child. He had no known history of trauma or fractures. He smoked for more than 30 years but denied any alcohol or drug use. His physical examination revealed severely limited range of motion of the cervical and lumbar spine with a modified Schober’s test of 2.4 cm. He also had significant weakness in his hip flexors with decreased patellar reflexes. He was positive for human leukocyte antigen (HLA) B27. Radiographs of the lumbar spine showed sacroiliitis and syndesmophytes in the lumbar spine. He was diagnosed with ankylosing spondylitis based on these results. He was also clinically diagnosed with cauda equina syndrome though a lumbar spine magnetic resonance imaging (MRI) done later did not show any cord compression. The recommendation was to start treatment with adalimumab pending the results of tuberculosis screening.\nWhile awaiting initiation of therapy, the patient had worsening lower extremity weakness and was admitted three weeks after presentation to the rheumatology clinic. He was afebrile on admission. His initial physical exam showed bilateral lower extremity weakness that was worse in the right lower extremity with bilateral diminished reflexes. His initial labs showed an elevated C-reactive protein (CRP) of 48 mg/L and no leukocytosis. A repeat lumbar spine MRI in the hospital showed no evidence of cord compression. His thoracic spine MRI, however, demonstrated T7-T8 discitis with concern for osteomyelitis but no pathological fracture. He underwent emergent surgical decompression of the thoracic spine from T7 to T10 (Figures -). A swab of the surgical site showed rare Gram-positive cocci on Gram stain, and he was started on empiric vancomycin by the infectious disease specialist. His condition showed little improvement over the next few days, and the culture from his surgical specimen showed no growth. He then had interventional radiology (IR) aspiration of the T7-T8 disc space one week later that revealed rare Gram-negative rods on Gram stain. The infectious disease team started him on cefepime at that point and continued with vancomycin. The culture of the aspirated fluid again showed no growth on culture. Rheumatology was consulted and suggested that the etiology of the discitis could be inflammatory due to long-standing ankylosing spondylitis. This was supported by repeat lab evaluation two days after the IR aspiration which showed an increasing CRP of 129 mg/L despite antibiotic therapy as well as a low procalcitonin of 0.12 ng/mL. The hospitalist, infectious disease, and rheumatology teams all reviewed the case. The negative cultures and history of long-standing ankylosing spondylitis suggested sterile inflammatory discitis, but they could not definitively exclude an infectious etiology. The group then decided to complete a six-week course of vancomycin and cefepime prior to starting adalimumab due to the risks of starting immunosuppressive therapy in a patient with possible infectious discitis.\nThe patient was followed by infectious disease as an outpatient shortly after completing his six-week course of antibiotics. He showed a significant improvement in his lower extremity weakness and paresthesia, and his CRP had decreased to 35.6 mg/L. The infectious disease specialist then cleared him to start biological therapy. He started a course of adalimumab 40 mg subcutaneous every two weeks at his rheumatologist's office where he continued to show gradual improvement in both his ambulation and paresthesia. He will continue to be monitored over the next few months by his rheumatologist as he continues adalimumab therapy with repeat thoracic and lumbar spine MRIs in two months. |
A 19-year old female was referred to the Department of Internal Medicine for workup of anemia. She complained of having fever (up to 38 °C), night sweats, dyspnea, dry cough, and fatigue. Symptoms had been present for 4 weeks and had not improved after a course of antibiotics prescribed by her family physician. On physical examination, a palpable painless mass was noticed in her right abdomen, with enlarged inguinal lymph nodes as well as a palpable lump in the right breast with ipsilateral axillary lymphadenopathy. Abdominal ultrasound (US) examination revealed a multiloculated cystic mass with solid areas measuring 35 × 30 cm in her pelvis. The US examination also showed an enlarged spleen with enlarged para-aortal lymph nodes. Subsequently, a thoracic, abdominal and pelvic multi-slice computed tomography scan (MSCT) was performed and showed a well circumscribed, solid nodule, 4 cm in diameter, in the upper outer quadrant of right breast with enlarged ipsilateral axillary lymph nodes, along with generalized abdominal lymphadenopathy, an enlarged spleen with diffuse multiple nodal masses and a complex right pelvic tumor mass with amorphous calcification similar to tooth formation. Based on the MSCT scan, a tentative diagnosis of an immature teratoma was made. The patient proceeded to surgery for a unilateral right salpingo-oophorectomy, lymphadenectomy, splenectomy, omentectomy and right breast lumpectomy. Intraoperative peritoneal washing revealed a poorly differentiated malignant tumor on cytology.\nOn macroscopic examination, the right ovary was replaced by a cystic mass with a glistening surface, measuring 17 cm in diameter (Fig. ). On incision, the cyst was partly filled with fatty material and hair. The wall of the cyst was thickened with solid, grayish nodules. The spleen was diffusely infiltrated with grayish nodes as well, measuring up to 4.5 cm in diameter (Fig. ). There was a lobulated, well circumscribed solid, grayish nodule identified on lumpectomy of the breast. On histological examination, the cavity of the cyst was lined by skin with dermal appendages as well as respiratory type of epithelium, smooth muscle, mature adipose and bone tissue. This morphology was in agreement with a diagnosis of mature cystic teratoma of the ovary. Solid nodules within cyst walls were tumor tissue, with a diffuse growth pattern, composed of large polymorphic cells with vesicular nuclei, a high nuclear-cytoplasmic ratio, and one or more prominent nucleoli. Some cells had eccentrically positioned nuclei with a cytoplasmic halo resembling plasmablasts. The tumor tissue from the right breast and spleen had the same histological appearance. An extensive immunohistochemical evaluation was performed. Tumor cells were negative for panCK, AFP, PLAP, CD117, D2-40, CD30, MyoD1, HMB45, desmin and CD99, but positive for LCA, EMA and vimentin. Therefore, a diagnosis of NHL was made. Subsequent immunohistochemistry showed tumor cells to be CD79a, PAX5, MUM-1, CD38, CD138 positive and CD20, CD10, Bcl-6, ALK, cyclin D1, CD56 negative. Ki-67 was high, around 80% and EBER in situ hybridization was negative (Fig. ). According to the morphological and immunohistochemical findings a diagnosis of PBL was made. Based on the clinical findings, the patient was staged, Ann-Arbour IV PBL. Laboratory data showed anemia, normal platelet and leucocyte counts with elevated lactate dehydrogenase (LDH), aspartate aminotransferase (AST) and C-reactive protein (CRP). Serum alpha-1-fetoprotein and human chorionic gonadotropin were normal while carbohydrate antigen 125 was increased. Urine analysis and renal function were within normal limits. Serum immunoglobulin levels were within normal limits, without evidence of monoclonal gammopathy and serology was negative for HIV, HBV and HCV. The patient was transferred to the Hematology ward and treated with dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH) as well as intra-thecal EPOCH with each cycle [, ]. After 4 cycles of chemotherapy, the patient subjectively felt better however evaluation with positron emission tomography and computed tomography scans (PET-CT) showed high activity of disease. An autologous peripheral blood stem cell transplantation was planned and 1 cycle of salvage chemotherapy was given according to DHAP protocol (cisplatin, cytarabine and dexamethasone). Unfortunately, her underlying disease continued to progress and 4 cycles of alternating modified CODOX-M and IVAC chemotherapy (cyclophosphamide, vincristine, doxorubicin, high-dosemethotrexate/ifosfamide, etoposide, and high-dosecytarabine) were given. Autologous stem cell transplantation could no longer be performed due to patient’s clinical deterioration. After the fourth cycle of chemotherapy, PET-CT scan was performed again, and revealed profound disease progression. The patient also had developed treatment complications, such as secondary anemia, thrombocytopenia, sepsis, enterocolitis, ileus, pleural effusions and ascites. Due to the worsening of the patient’s physical condition, her goals of care were changed and only supportive therapy was given. The patient died of multiple organ failure approximately 11 months after initial diagnosis (Additional file ). |
A 47-year-old woman with no history of brain lesion experienced a blunt trauma to her head. A brain computed tomography scan established a diagnosis of traumatic subdural hematoma (SDH) in the left frontotemporal lobe with midline shifting (). The patient's mental state was comatose, and the Glasgow Coma Scale score at presentation was 3/15. She underwent an emergency surgery for removal of the hematoma with craniectomy to reduce increased intracranial pressure caused by cerebral edema and hematoma. However, in the intensive care unit after surgery, she unexpectedly developed left hemiplegia, despite the SDH being in the left frontotemporal lobe.\nShe had been under continuous rehabilitation therapy in the local inpatient clinic for the last 2 years; she presented to our hospital and was admitted for intensive rehabilitation treatment. In order to evaluate hemiplegia, on the manual muscle test, the patient's muscle strength was classified as Medical Research Council grade 2 in the left upper and lower extremities with normal strength on the right side. On the Fugl-Meyer Assessment, she scored 17 points for the upper extremities and 34 points for the lower extremities.\nDeep tendon reflexes were increased on the left side, and spasticity was evaluated as Modified Ashworth Scale (MAS) score 3. On neurological examination, she had a Korean Mini-Mental State Examination score of 30, and a Loewenstein Occupational Therapy Cognitive Assessment score of 111 out of 114; these scores indicated no prominent deterioration in the left hemisphere functions, such as memory, calculation, executive function, and language.\nBecause the ipsilateral symptoms did not correlate with the brain lesion, we considered 2 possibilities that could explain her clinical symptoms. The first was a missed diagnosis, such as diffuse axonal injury caused by countercoup or ischemic infarct followed by cerebral edema with midline shifting (Kernohan's notch phenomenon), and the second was an uncrossed CST.\nTo investigate the pathomechanism of the ipsilateral hemiplegia, conventional magnetic resonance imaging (MRI), including diffusion tensor imaging (DTI), was performed 3 years after the accident using a 3.0T MAGNETOM Skyra (Siemens AG, Medical Solutions, Erlangen, Germany). Fibers passing through 2 regions of interest at the upper and middle pons were selected as the CSTs, with a fractional anisotropy (FA) threshold >0.2 and an angle threshold <60°. Both CSTs originating from the cerebral cortex, including the primary motor cortex, descended to the medullary pyramid through the normal CST pathway. FA and apparent diffusion coefficient (ADC) values were obtained in the pons.\nThere was no pathological evidence that correlated with left hemiplegia, such as diffuse axonal injury or leukomalacia changes in conventional MRI (). However, the right CST was sharply narrowed at the lower pons level. In contrast, the left CST was relatively well preserved within normal limits despite the huge hematoma that had caused midline shifting (). Moreover, DTI revealed that the FA values of the right CST decreased and the ADC values of the right hemisphere increased when compared with those of the left hemisphere ().\nTo rule out the presence of uncrossed CST, motor evoked potentials (MEPs) were checked. However, magnetic stimulation of the hand area of the left cerebral motor cortex did not produce MEPs in the left thenar muscles.\nTo obtain somatosensory evoked potential (SEP), following electrical stimulation of the left median nerve, P9, P13, N19, and P23 were recorded bilaterally in the frontal region, and cortical responses were localized in the right central and parietal regions, which were contralateral to the side of stimulation. Right median nerve stimulation evoked a cortical response in the left frontal and parietal lobes. Electrodiagnostic examination was performed to rule out peripheral nerve lesions, including radiculopathy or brachial and/or lumbosacral plexopathy; the results revealed mild grade bilateral carpal tunnel syndrome on both sides. |
A 15-year-old male without any symptoms was referred to our hospital because he was noted as having an abnormal shadow on chest X-ray at a health checkup. No abnormal findings were observed on his hematological and biochemical examinations. On chest computed tomography (CT), a 40 × 33-mm wide tumor shadow with clear boundaries in the right pulmonary hilar area was found. The tumor was strongly enhanced in the early phase. Abnormal findings were not found in the lung field and mediastinum (Fig. a–c). Bronchoscopic examination was performed under topical anesthesia. The lateral segment of the lower lobe of the right bronchus was narrowed by compression of the tumor although the endobronchial mucosa was intact. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was performed but no specific findings were obtained from the cytological and histological evaluation. However, the patient was admitted to our department for surgery because 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed abnormal accumulation in only the tumor; SUV (standard uptake value) max was 4.4 (Fig. d), which suggested potential malignancy. Due to the possibility of a malignant tumor, right middle and lower lobectomy was necessary due to its localization, and depending on the intraoperative findings, it was also necessary to perform right pneumonectomy. We informed the patient and his mother of this before surgery and obtained their consent. However, from the imaging morphology of the tumor and lack of evidence of malignancy in EBUS-TBNA, we also kept in mind before surgery the possibility of benign tumors including Castleman’s disease. We decided to make a final decision on the procedure based on the findings of the intraoperative macroscopic findings and the intraoperative frozen section diagnosis.\nOn operative findings, the tumor existed between the middle and lower lobes of the right lung with no pleural involvement. The interlobar pulmonary artery was revealed on the back side of the tumor. We performed 18Ga needle biopsy for intraoperative frozen section diagnosis, which showed only chronic inflammation findings. Moreover, macroscopically, no tumor invasion into the pulmonary vessels, bronchi, and lung parenchyma was found. Therefore, only the tumor enucleation was performed (Fig. a). An intraoperative frozen section diagnosis of the removed tumor found suspected Castleman’s disease. Therefore, we decided not to do further resection.\nThe extirpated specimen was a 30 × 23-mm smooth and well-encapsulated tumor. Histologically, the lesion had a fibrous capsule with clear boundaries consisting of lymph follicle hyperplasia. There were clearly hyalinizing concentric fibrotic nests around the lymph follicle and vessel hyperplasia on the hyalinizing wall. There were almost no plasma cells outside the follicle. Histological diagnosis was that of HV type CD (Fig. b). The postoperative course was good, there were no complications, and he was discharged home on the fifth postoperative day. The patient is now under follow-up observation with no recurrence seven years after the operation.\nHV type CD is common in young people. The most common sites of this disease are in the cervix, mediastinum, abdomen, and retroperitoneum [], but it is very rare in the pulmonary hilum. HV type CD is usually discovered by chance in regular health checkups with almost no accompanying specific symptoms. In radiological findings, enhanced CT shows a contrasted tumor with clear boundaries in general, especially in the HV type. High contrast is promptly recommended for vessel hyperplasia with hyalinizing inside the tumor []. In addition, some reports recently found that FDG-PET sometimes shows light-to-moderate accumulation [–]. However, in any case, the findings are not specific for CD, making it very difficult to reach the diagnosis by radiological findings alone. Moreover, from the reports so far, definitive diagnosis by preoperative biopsy seems difficult [–]. Similarly, the preoperative diagnosis of our case could not be made because of insufficient biopsy specimens for the pulmonary hilar mass.\nKeller et al. retrospectively examined six localized HV type CD patients who received partial resection, biopsy, or observation alone. They reported that disease progression was noted at four years after surgery in one patient and, in another patient, complete resection was performed eight years after an initial biopsy and observation following the onset of symptoms []. Moreover, Biçakçioğlu et al. reported that 17 of the 19 CD cases, including seven cases in the pulmonary hilum, in which surgery was performed were localized, and that 15 cases in which complete resection was performed had no recurrence []. Therefore, the recommended treatment strategy for localized HV type CD is complete resection. We found ten cases of resection of HV type CD in the pulmonary hilum in which detailed clinical information including surgical procedure could be obtained from the articles searched with the terms: Castleman’s disease AND pulmonary AND surgery in PubMed (Table ) [–, –]. One case reported by Luo et al. was described as “whole resection” and the details were unknown about surgical procedure []. Lobectomy or more extensive surgery was performed in seven of these nine cases. In only one of seven cases was intraoperative frozen section diagnosis performed []. In three of six cases, lobectomy was performed because malignancy of the tumor could not be ruled out [, , ]. Moreover, in the other case, there was no mention in the article why lobectomy was performed []. If intraoperative frozen section diagnosis was performed on these four cases with benign diseases as in this present case of CD, it may have been possible to select a procedure that would have preserved pulmonary function. Tumor enucleation was performed in two cases. In one of two cases, intraoperative frozen section diagnosis was performed []. We performed only tumor enucleation considering both the localized FDG-PET accumulation and the intraoperative frozen section diagnosis without major bleeding to carefully separate the tumor from surrounding tissue. The patient is now under follow-up observation with no recurrence seven years after the operation.`` |
A 60-year-old African American female was following up for her chronically elevated alkaline phosphatase levels. She had a history of hypertension, hyperlipidemia, type 2 diabetes mellitus, allergic rhinitis, and chronic lower back pain. Patient has a family history of arthritis, cardiovascular disease, and diabetes mellitus; she denies ever using alcohol or tobacco.\nWith the onset of elevated alkaline phosphatase level and vague abdominal pain in 2013, an abdominal ultrasound performed in December showed hepatic steatosis. Viral serologies for hepatitis during 2013 were negative, as a gastrointestinal consult was required to determine the need for a liver biopsy. A liver biopsy was subsequently performed, which showed focal mixed micro and macrovesicular steatosis. Portal tracts showed minimal focal chronic inflammation, no significant fibrosis, and no iron deposition.\nThe vague abdominal pain that she was experiencing waxed and waned for two years. Additionally, the patient experienced some vague chest pain and dyspnea that prompted an echocardiogram in February of 2015, which demonstrated a left ventricle ejection fraction of 44%. Consequently, a left heart catheterization in the following month showed no significant coronary disease with a dilated left ventricle with an ejection fraction of 50%. A 2-year follow-up in July of 2015 showed suspicious cirrhosis by Computed Tomography (CT) scan (), possibly due to granulomatous changes and chronic inflammation. A CT scan was determined to be necessary for our patient because of rising alkaline phosphatase without other explainable etiologies, in addition to the patient's appetite suppression and vague abdominal pains. Patient's weight during this time was 207 lbs (93.89 kg) and was advised to diet and exercise. After 4 months of continuous symptoms, especially with abdominal pain, a laparoscopic cholecystectomy was performed with a liver biopsy that showed subsequent granulomatous changes in September of 2015. The liver biopsy showed coalescing periportal nonnecrotizing epithelioid granulomas with associated multinucleated giant cells and chronic inflammation. The chronic and patchy inflammation is representative of the granulomatous hepatitis, despite not having elevated transaminases. The granulomatous changes suggested possible sarcoidosis (). The liver biopsy was not histologically suggestive of nonalcoholic steatohepatitis, with no steatosis noted. Chest X-ray at that time showed no significant findings.\nInflammatory bowel disease (IBD) was not explored in the patient because she never had clinical signs on past or present examinations, denying any symptoms of IBD including alternating bowel habits, predominant constipation, or diarrhea. Laboratory studies 2 years and 6 months since the onset, at the end of 2015 showed negative antinuclear antibodies (ANA), negative rheumatoid arthritis factor, negative cyclic citrullinated peptide (CCP) antibodies (IgG/IgA), elevated C-reactive protein of 8.9 mg/L, normal complement C3/C4, elevated B-type natriuretic peptide 688.6 pg/mL, and negative mitochondrial (M2) antibody. Subsequent office visits and additional laboratory results are shown in .\nAdditionally, patient experienced peak weight loss with a weight of 154 lbs. (69.85 kg) in 2016. During this time period, an echocardiogram showed a decreased ejection fraction of 26%. This result and a history of having ventricular tachycardia resulted in the patient having an automated implantable cardioverter-defibrillator (ICD) placed.\nUrinalysis in June 2017 showed RBC 0-5/hpf, WBC 0-5/hpf, bacteria 2+, and moderate calcium oxalate crystals. Additionally, patient's weight increased in 2017 to 190 lbs. (86.18 kg). |
A 56-year-old man with a history of non-insulin dependent diabetes mellitus was referred to the surgical department because of the onset of lower back pain and hematuria that occurred the previous day. The patient was not a smoker and denied alcohol consumption; his family history was unremarkable and there was no other complaint. The ultrasound of the left kidney revealed the presence of stones in the renal pelvis causing obstruction of the ureteral meatus and consequent dilatation of the upper calix. During the examination, the presence of a solid neoplasm was noticed that involved the renal medulla reaching the hilum area without signs of hilar infiltration. For further investigations, the patient underwent CT (computed tomography) and MR (magnetic resonance) that showed the slight enhancement and extensive necrotic areas of the lesion and the existence of a solid nodule with same features in the left lung, likely due to metastases (Figs. a-b). A lombotomic total nephrectomy was performed, and the patient had an uneventful post-operative recovery.\nGrossly, the kidney presented irregular profile due to a hilar lesion measuring 4 cm × 2.5 cm. On cut sections, it was highly hemorrhagic, soft, and reddish with infiltrating borders (Figs. c-d). The tumor was extensively sampled. Microscopic examination was performed on paraffin-embedded sections stained with hematoxylin and eosin (Figs. and ). Histopathological examination showed a proliferation of canalicular structures of various sizes, sometimes with some degree of cystic changes, lined by atypical cells with epithelioid features, deeply invading the renal parenchyma and the perinephric fat. Many of these structures contained red blood cells giving the idea that it was a vascular lesion. In some fields was observed an almost solid growth pattern with erythrocytes trapped in thin spaces between neoplastic cells. Marked cellular pleomorphism, enlarged and hyperchromic nuclei, irregular nuclear contour, prominent nucleoli, and frequent mitotic figures were also evident. In the context of this lesion, few fields showed a quite different morphology consisting of spindle cells arranged in palisading fashion without cellular atypia or invasive features. An immunohistochemical study was performed on formalin-fixed paraffin-embedded tissue block to define the histogenesis of the lesion. Pre-diluted antibodies produced by Ventana-Roche were used, directed against pan-cytokeratin (clone AE1/AE3/PCK26; mouse monoclonal), CD34 (clone QBEnd/10; mouse monoclonal), factor VIII (rabbit polyclonal), Ki67 (clone 30-9; mouse monoclonal), S100 (clone 4C4.9; mouse monoclonal), EMA (clone E29; mouse monoclonal), and CD10 (clone SP67; rabbit monoclonal). The malignant population was reactive for pan-cytokeratin, CD34, factor VIII, partially positive for Ki67 (positivity in about 40 % of neoplastic cells) and negative for S100, EMA and CD10, therefore was consistent with epithelioid angiosarcoma. Otherwise, the benign spindle cells population was positive for S100 and negative for Ki67 and epithelial and endothelial markers. It was so interpreted as residuals of pre-existent schwannoma. In view of these morphological and immunohistochemical findings, a diagnosis of primary renal epithelioid angiosarcoma probably arising in schwannoma was made. A CT-guided fine-needle aspiration cytology of the pulmonary lesion was subsequently performed that showed CD31-positive atypical epithelioid cells in the context of numerous erythrocytes, confirming the diagnosis of lung metastasis of angiosarcoma. During the post-operative period, the patient has not been subjected to antineoplastic therapy because of poor general health and he came to exitus a few months after diagnosis, because of a massive hemothorax caused by lung metastasis. |
On Feb. 3, 1982, a 33 year old man, an industrial worker, was admitted to the general surgery department of Korea University Hospital because of intermittent claudication and longstanding ulceration of the right leg, which developed following acupuncture on his right leg. The perfusion was very poor and the arterial pulses were not palpable over the femoral arteries and down either leg. An aorto-femoral angiogram revealed occlusion of the right femoral artery with patent anterior tibial, posterior tibial, and peroneal arteries, and the occlusion of the left internal iliac artery with collateral arteries.\nFor the purpose of limb salvage, embolectomy and right femoro-tibial saphenous vein bypass graft surgery was attempted. There was no evidence of atherosclerosis in the leg vessels. Twelve hours postoperatively, the bypass graft stopped functioning. Reoperation was done and thrombectomy was carried out, but the patient finally had to undergo above-knee amputation on the right leg at a later date. During this admission, the patient experienced multiple episodes of precordial chest pain and an episode of colicky left flank pain.\nAccording to the past medical history, he was first admitted to Korea University Hospital because of severe chest pain, which awakened him early in the morning of July 23, 1976. The electrocardiogram on admission showed changes compatible with an acute inferior wall myocardial infarction (), the peak SGOT was 230 IU L and the peak LDH was 1500 U ml (Wrobleski). The hospital course was uneventful except for the intermittent episodes of abdominal pain. He was discharged on Aug. 16, 1976.\nBetween Aug., 1976 and Feb., 1982, the patient had to be amitted to the hospital three times. The first time was for the sudden development of dizziness, the second time for abdominal pain due to ileus, and the third time because of chest tightness and abdominal pain.\nHe had had an appendectomy at the age of 13 and had contracted typhoid fever at 14. According to his history his father and younger brother had hypertension. He smoked about two packs of cigarettes per day and consumed a little alcohol.\nThe chest X-ray revealed mild cardiomegaly with a prominent left ventricle. Occasional premature ventricular contractions were noted on the twelve-lead electrocardiogram. The hemoglobin was 14.0 gm dl; hematocrit 42%: ESR 30 mm hr: WBC 12,250 with 50% of segs, 22% of lymphs, 10% of monos, and 12% of eosinophils. The platelet count was 258,000 mm3. The bleeding time and the coagulation time were normal. The serum cholesterol was 167 mg dl and the triglyceride was 55 mg dl. The lipoprotein electrophoresis revealed that α-lipoprotein was 22.7%, prebeta 28.6%, and beta 48.7%, the RA factor was negative. The protein electrophoresis revealed albumin 50.9%, α1 3.2%, α2 10.9%, β13.8%, and γ21.2%. The patient was discharged on Apr. 14, 1982.\nOn July 3, 1982, the patient was admitted to the hospital because of abdominal pain. The abdomen was tender and distended, and the bowel sound was diminished. Under the impression of mesenteric artery thromboembolism heparinization and subsequent coumadinization was done. To rule out the possibility of embolization from the heart two dimensional echocardiography was done. This reveled a large, pedunculated mass which had its base in the apex of the left ventricle and its end freely movable in the left ventricular outflow tract (). The mitral valve leaflets were seen striking the mass during its motion. The mass was 7×3 cm. The mass reflected echoes which were dense compared with the lighter echoes from the myocardium. The outline of the mass was rather well defined and several daughter masses were noted to be budding from the main mass. The left ventricle was only slightly enlarged in size, and the apex and the distal septum were noted to be akinetic. Under the impression of either a left ventricular thrombus or a myxoma open heart surgery was performed on July 9, 1982.\nIn the operative field, the left ventricular apex was noted to be fibrotic and moderately adhered to the regional pericardium in an area of about 3×5 cm2. The left ventricle was opened through the infarcted area. The organized thrombus -like mass, which was pedunculated from the inner surface of the apex and intermixed with the muscular trabeculae was removed en bolc with the central infarcted apical wall to preserve the total continuity of the mass. The left ventricular wound was closed with heavy, interrupted, cross suture and pledgeted dacron flet velour.\nThe gross specimen measured 7.0×4.5×2.7cm and weighed 35 gm (): On pathologic examination the mass was confirmed to be a thrombus. The surface of the mass was irregular and grayish-pink colored with multiple hemorrhagic foci. On the cut surface could be seen whitish-yellow-to-pinkish-brown, soft tissue with interlacing bundles. The microscopic examination revealed fibrinous lamellated eosinophilic material mixed with coagulated RBSs, WBCs, platelets, cholesterol clefts, and focal calcifications. At the periphery of the mass, fibroblastic proliferation was seen, sections from the right autricle and the left ventricle revealed the coagulative necrosis of myocardial fibers and fibroblastic proliferation.\nOn July 23, 1982, he developed impending gangrene of the left leg. Thromboembolectomy of the left femoral artery was done without success. Finally, he had to undergo the above knee amputation on the left leg. During this admission, the patient experienced squeezing precordial pain and intermittent abdominal pain.\nOn Aug. 30, 1982, factor VIII concentration was 200% and factor V 100% of the normal control. The euglobulin lysis time was 3 hours. FDP was less than 10 μg/dl. The fibrinogen was 407 mg/dl. The serum antithrombin III level was within normal range. The platelet aggregation test revealed increased aggregability with ADP (patient, 91.3%; control, 77.5%), epinephrine (patient, 82.5%; control, 72.5%), and collagen (patient, 81.3%; control, 70.0%).\nThe two-dimensional echocardiography repeated on Oct 6, 1982 revealed no evidence of recurrence of thrombus in the left ventricle. The patient was discharged on Now. 16, 1982. He has been followed in the outpatient clinic and has been doing reasonably well so far, except for occasional mild left precordial chest pain. A thallium-201 myocardial imaging was done on June 29, 1983, by injecting 2 mCi of thallium-201 intravenously following the intravenous injection of 7 mg of dipyridamole. It showed a perfusion defect of the inferior wall, the site of the previous myocardial infarction. The perfusion defect did not improve with the delayed images taken four hours later. |
A 45-year old woman presented with a huge left breast mass that had necrosis and a foul odor. She had a history of contralateral metaplastic carcinoma, and she had undergone a right modified radical mastectomy 15 years previously. She also presented with a 2-cm-sized painless palpable breast mass in her left breast two years before presentation for the case in this report, but she did not undergo any clinical work-up at that time. Then, approximately 2 or 3 months prior to presentation of this case, she noticed a mass extrusion of the outside skin with rapid growth. On physical examination, a huge necrotic mass 20 cm in diameter was seen in the entire left breast. The mass was extruded skin outside, and skin overlying the mass was thickened with edematous change (Figure ). The axilla showed multiple conglomerated lymph nodes (LNs) that were fixed to the chest wall.\nA chest computerized tomography (CT) showed a 22-cm-sized huge breast mass with necrotic changes and multiple LN enlargements in the left axilla, both supraclavicular and parasternal areas including the left internal mammary and paracardiac LNs (Figure a). On 18 F-FDG positron emission tomography (PET)/CT, a huge uneven hypermetabolic mass was seen in the left breast, and there was no evidence of distant organ metastasis except for extensive hypermetabolic LN metastasis (Figure b).\nUnder strong clinical suspicion of inflammatory breast cancer, we performed a core needle biopsy on the tumor itself. Core-cut biopsy revealed a biphasic neoplasm with mild nuclear atypia and low mitotic activity, accompanied by leaf-like processes protruding into dilated ductal spaces, consistent with a low-grade phyllodes tumor. We discussed the discrepancy about the clinical diagnosis and core biopsy results at the preoperative multidisciplinary meeting that included an oncologist, a breast surgeon, a pathologist and a radiologist. We concluded the case was a carcinoma associated with huge phyllodes tumor and decided not to do further preoperative biopsy. The patient underwent a palliative modified radical mastectomy with level II axillary LN dissection. Macroscopic examination of the resection specimen revealed a firm and well-demarcated mass with hemorrhage and necrosis, measuring approximately 24 cm in the largest dimension (Figure a). This mass was composed of two separated tumorous lesions: phyllodes tumor and invasive carcinoma of no special type. Histologically, about 70% of the tumor area represented a classical phyllodes tumor showing variable benign to malignant histologic characteristics. The malignant phyllodes tumor was composed of highly cellular stromal cells, moderate and variable nuclear atypia, and increased mitotic activity (>10 mitotic figures per 10 high-power fields) (Figure b). In addition, in almost one-third of the tumor, frankly invasive carcinoma of no special type (Figure c) with foci of pleomorphic carcinoma was observed (Figure d). The skin was thickened and showed pathognomonic dermal lymphatic tumor emboli, consistent with inflammatory carcinoma (Figure a). The areas of margin between the two tumor lesions were evaluated by further re-cutting and serial section. On serial section, there was no histologic evidence of transition from phyllodes tumor to invasive ductal carcinoma, so the lesion was thought to be a collision tumor with invasive carcinoma of no special type and malignant PT, rather than ductal carcinoma arising from PT (Figure b). Metastatic ductal adenocarcinoma cells were detected in 16 out of 16 dissected axillary lymph nodes. On immunohistochemistry, both epithelial and stromal compartment of the PT as well as the carcinoma cells showed no immunoreactivity for estrogen and progesterone receptors and HER2, except for p53 overexpression on the carcinoma cells.\nThe wound healed completely without complication. Chemotherapy based on anthracyclines and taxanes was started and radiotherapy was planned. Six months after the operation, and after completion of eight cycle’s chemotherapy, the patient developed left supraclavicular and upper chest wall swelling, which was consistent with regional aggravation of metastatic carcinoma and was supported by image findings. She underwent a session of radiation therapy to the chest wall and neck, and platinum-based chemotherapy will be pursued in this patient as further treatment. She is doing well with good compliance to the chemotherapy and radiation therapy. |
A 66-year-old Caucasian male patient was first examined in January 2020 in our memory clinic (). The patient is married and father of a daughter. He achieved an educational level of 8 years and was employed as a factory worker until retiring in 2017. His wife described a marked loss of memory functions about 3 years ago. After a subacute onset of cognitive decline, his memory problems failed to resolve, and were accompanied by depressive symptoms with insomnia over time. Symptoms of cognitive impairment were initially attributed to the affective disorder, and he was given mirtazapine at a daily dosage of 15 mg by a psychiatric consultant. The patient underwent MRI imaging of the neurocranium in November 2019, which revealed asymmetrical atrophy of the mesiotemporal region suggesting Alzheimer´s disease (A,B). His wife also reported a progressive speech disorder, a beginning disorientation, and behavioral symptoms in the last few months. Behavioral symptoms comprised minor signs of frontal disinhibition and impulse-control dysregulation. She also reported that he had begun using swear words more often, and had developed delusional symptoms: he became convinced he was being addressed by the hosts of a certain afternoon television show and seemed to converse with them bidirectionally. The presence of hallucinations in the context of psychotic symptoms could not be confirmed. His treatment at the time of first contact in our memory clinic consisted of mirtazapine at a daily dosage of 15 mg, acetylsalicylic acid, magnesium and Vitamin D supplements. His mother grew very old (88 years), but suffered from dementia of unknown etiology. The cause of his father’s death was unknown. His medical history comprised a myocardial infarction in 2018 and intermittent muscle cramps. There were no signs of substance-related abuse, or consumption of illegal psychoactive substances.\nInitial physical and neurological examination revealed no pathologies, in particular, no signs of cerebellar disorders like ataxia or oculomotor abnormalities. Routine and extended laboratory tests excluded Vitamin B1, B6, and B12 deficiency. Ceruloplasmin and cupper values in serum were normal. Thyroidal hormone levels were also normal, and screening for autoantibodies against thyroidal gland tissue was negative. Any neurological manifestation of borreliosis and lues was excluded. IgM-antibodies against borrelia burgdorferi in serum were ascribed to a persistent antibody response with no clinical relevance, as no specific borreliosis-antibodies or elevated cell count were detected in CSF. Routine CSF diagnostics continued to reveal normal values (2 cells/µL, protein 401 mg/L, oligoclonal bands negative, MRZ-VZV-HSV-AI were not elevated). Biomarkers implied no neurodegenerative disease (normal were total tau, 181pTau, ß-Amyloid 1-42, ß-Amyloid-Ratio). An anti-neural antigen immunofluorescence test exposed Anti-KCNA2 (voltage-gated potassium channel ß-subunit Kv1.2) (1:100) antibodies in serum only. No LGI1 or CASPR2 reactivity was identified ().\nHis MRI dataset was reviewed in the Department of Neuroradiology, confirming an asymmetrical atrophy of the mesiotemporal and right temporopolar brain regions and progressive leukoencephalopathy in white matter (A,B). MRI data were initially compared to those obtained in 2014 that had shown no atrophy and fewer white matter lesions. The mesiotemporal atrophy was interpreted as being compatible with a limbic encephalitis at later stages, which was recently reported in courses of limbic encephalitis by Wagner et al. [].\nPreliminary cognitive screening detected a significant result in MMSE (Mini Mental State Examination) (23/30). The clock-drawing test was normal, but comprehensive neuropsychological assessments including the CERAD (Consortium to Establish a Registry for Alzheimer’s Disease) test-plus battery (covering these cognitive domains: attention, language, visuoconstruction, memory and executive functions) and ROCFT (Rey-Osterrieth-Complex-Figure test) revealed marked deficits in figural and verbal memory parameters associated with an impaired delayed recall. Moreover, language impairments (Boston Naming Test and Semantic fluency) were moderate. His working memory was also moderately affected, whereas visuoconstructual/visuo-spatial abilities were unaffected. As our patient is not a native German-speaker, his verbal memory and language functions must be interpreted very carefully (). Nevertheless, in light of his relevant deficits in daily-living abilities, we diagnosed a dementia.\nSubsequently, the patient was admitted to the Department of Neurology in February 2020. EEG recordings revealed no abnormalities—neither epileptic potentials nor any focal slowing. Thorough electrophysiological examinations (EMG, neurography) revealed no signs of peripheral nerve hyperexcitability, nor acute or chronic denervations. Thus, Morvan´s syndrome was unlikely. Daily intravenous corticosteroids were applied over 5 days and well tolerated. His wife reported improved speech fluency and attentiveness. We screened for any malignancy, including a conventional X-ray of the thorax and ultrasound diagnostics of the abdomen. An undefined tumorous structure in the bladder and additional hyperdense lesion in the lung were identified. We recommended a further computer tomography of the thorax and abdomen and PET (positron emission tomography)-tumor-screening, but the patient refused. |
A 55-year-old Caucasian female with stage IV pancreatic cancer and without other significant medical history presented to the interventional pain clinic with increasing sacral, lower extremity, and pelvic pain. The patient was unable to ambulate secondary to pain and deconditioning. Magnetic resonance imaging without contrast and computed tomography (CT) with contrast just prior to her clinic visit showed a large pancreatic mass with liver infiltration, multiple pelvic and sacral metastatic lesions, and a significant collection of intra-abdominal fluid. Of note, the lumbar and sacral epidural space showed no abnormalities or lesions. Her oncology team determined she was no longer a candidate for any further treatment and recommended palliative measures only. Her prognosis was poor with a life expectancy of a few weeks. Her pain control regimen included fentanyl patch 200 mcg every 72 hours, hydrocodone/acetaminophen 10/325 mg every 6 hours as needed, duloxetine 60 mg daily, and gabapentin 300 mg three times a day; however, she continued to have uncontrolled pain with significant decrease in her quality of life.\nDue to the patient’s poor prognosis and short life expectancy, an implantable intrathecal pain pump was not pursued. It was decided that a tunneled multiorifice epidural catheter with an external infusion pump would be the best treatment option for her pain. The epidural catheter was placed at the L5-S1 level using standard fluoroscopic technique. A paramedian approach with the Tuohy bevel directed cephalad was used to advance the catheter to the L3–4 vertebral level. Then 2 mL of injected contrast showed spread along the right L5 and S1 nerve roots. There was no contralateral spread seen () and therefore the decision was made to inject a test dose of 6 mL of bupivacaine 0.25%. After waiting for 10 minutes, the patient was assessed and she had marked anesthesia and decreased pain on her right side, but normal pain on the left. To get full relief of her symptoms, it was decided to place a second tunneled epidural catheter using the same technique as the previous catheter placement along the left side of the epidural space at the same L5-S1 level in order to achieve bilateral analgesia. Then 2 mL of contrast was again injected through this catheter after placement, and it showed spread only along the left L5 and S1 nerve roots (). To achieve contrast spread bilaterally, injection through both epidural catheters was required. The placement of two epidural catheters was therefore performed in order to avoid the anticipated poor bilateral spread of infused local anesthetic and subsequent inadequate pain relief. The patient tolerated the procedure well and was placed on dual patient controlled epidural analgesia (PCEA) pumps with each pump delivering a basal dose of 3 mL of bupivacaine 0.2% per hour with a demand dose of 3 mL every 30 minutes. She received excellent pain control and her reported numeric pain score for her lower extremity and sacral pain went from a 10 to a 3. She was discharged to hospice and placed under the care of the palliative physician. On phone call follow-up, it was reported that she had no adverse effects associated with the epidural infusion and continued to have numeric pain scores in the 3 to 6 range. While in hospice she was continued on the same basal epidural infusion and with approximately five demand doses per day. The patient remained on the same topical and oral pain medication regimen, which included transdermal fentanyl and oral hydrocodone/acetaminophen. Unfortunately after 4 weeks, the patient passed away in her sleep with her husband at her bedside. Written informed consent was obtained from the patient’s next of kin (husband) to publish this case report and any accompanying images. |
A 49-year-old Caucasian woman, an active smoker (15 packs/year) with an otherwise silent past medical history, presented with a dry cough that worsened in the supine position. While the cough resolved with proton-pump inhibitor (PPI) therapy, suggesting a gastrointestinal nature of the symptom, the patient also underwent a routine chest radiograph. The imaging showed a nodule with a diameter of 3.5 cm in the right lower lobe. Thus, a chest CT scan was performed, which confirmed the presence of a solid lesion with well-defined margins, mild contrast enhancement, and a diameter of 34 × 26 mm in the anterior basal segment of the inferior right pulmonary lobe. The exam also revealed an enlarged axillary lymph node that was later confirmed to be inflammatory in nature. To further characterize the lesion, a PET-CT scan with an injection of 18F-fluorodeoxyglucose (FDG) was done; the images showed mild uptake at the level of the nodule in the right lower lobe (with a maximum SUV of 2.8) (, ), suggesting a possibly benign or locally invasive biological behavior. Fine-needle biopsy for typing of the lesion was attempted but ultimately not performed due to poor compliance of the patient during the CT-guided procedure. Therefore, wedge resection of the right inferior lobe and nodal sampling were performed with video-assisted thoracoscopic surgery. A frozen section procedure was performed during the operation, which gave unconclusive results. Hence, it was decided not to perform a completion lobectomy but to wait for the final histological results. The tissue specimen analyzed at histology showed richly vascularized intrapulmonary solid proliferation () that comprised two types of cells: epitheliomorphic and spindle-shaped cells. The former type of cells had large eosinophilic cytoplasm and moderately atypical nuclei (nucleolates) partially dispersed in a loose stroma crossed by ill-defined septa (); the latter cells, also called “sub-tentacular cells,” were interposed with moderate infiltrate of lympho-plasma cells (). No necrosis was found in the sections examined, and the mitotic index was <1 mitosis for 10 high-magnification fields (10 HPF, 40X). At immunocytochemistry, all cellular elements showed a strong positive reaction for synaptophysin (Syn) and neuron-specific enolase (), while there were only areas of positivity to the S-100 protein in correspondence of the sub-tentacular elements. The dissected lymph nodes were negative. The final diagnosis was a primary pulmonary paraganglioma. The postoperative course was uneventful, with the thoracic drainage removed on the third postoperative day (POD) and no signs of pneumothorax on the chest radiograph performed afterward. The patient was discharged from the hospital on the fourth POD. At the latest checkup, 1 month after the hospital discharge, she showed no sign of relapse on the chest radiograph. After multidisciplinary discussion with the Oncology, Radiology, and Pneumology Departments, it was decided to proceed with radiologic follow-up at 3-month intervals for the first semester with a chest CT scan, then in 6 months for the following year, and later maintain a 1-year radiologic follow-up either with chest radiographs or a CT scan. The main limitation on deciding the timing of the follow-up was the scarcity of available literature on both the treatment and the recurrence rates; however, the available reports seem to suggest an indolent nature of this tumor with an unlikely tendency to recur, which is what informed about our decision on not performing completion of a lobectomy. |
This 19-year-old female was the fifth child of first-cousin parents. One sibling passed away at 20 days of age because of pneumonia and another died at the age of 4 months due to kidney failure. There was also a full-term idiopathic stillborn. Unfortunately, none of them were seen at our institution and we did not have access to their charts. It is not possible to make any inference about the etiology of their diseases.\nThe patient was born uneventfully at 36 weeks of gestational age by caesarean section, had a birth weight of 2600 g (5th–15th percentile) and 46 cm birth length (5th percentile). Head circumference information was unavailable. There was no clinical sign or laboratory findings consistent with cholestasis at neonatal period or in her first 2 years of life. She had global developmental delay that was first recognized after 6 months of age. All domains were affected. She was not able to hold her head up and sit without support until 1 year old. She started walking with support at 6 years old and without support at 7 years old. She started speaking some words at 4 years old. She did not name colors, recognize numbers or letters, and she can only produce scribbles. She started using a spoon at 11 years old. At that moment, her functional age according to Denver II was gross motor 12–13 months, fine motor 15–16 months, language 18–19 months, and personal/social 7 months. A formal neuropsychological assessment was not conducted.\nShe had recurrent hypoglycemia associated with fasting from 6 months to 7 years of age without hyperinsulinism and was not responsive to glucagon. At the age of 10 months, she experienced a febrile seizure without hypoglycemia and at the age of 9 she started having focal motor seizures with impaired awareness and automatisms. The seizures were rare and easily controlled with carbamazepine.\nAn etiological investigation was initiated when she was admitted to our service at the age of 10 years old.\nAt this age, her weight was 14.5 kg (2.5th–10th percentile), her height was 100 cm (<2.5th percentile), and her head circumference was 52 cm (2.5th–50th percentile). She had subtle dysmorphic features, including frontal bossing, high forehead, down slanted palpebral fissures, and dental malocclusion (Figure ). She walked with an independent gait and had generalized hypotonia, as well as severe intellectual disability, being able to speak only isolated words. The laboratory investigation revealed intermittent increases of transaminases (up to AST 319 U/L, ALT 360 U/L) without liver failure or hyperbilirubinemia (total bilirubin level 0.56 mg/dL, direct bilirubin level 0.18 mg/dL). Methionine levels were elevated only once (1533 μmol/L; reference value 7–47 μmol/L) and became normal (33.96 μmol/L on her last visit) spontaneously, without any specific diet (Table ). Proline levels were also elevated (541 μmol/L; reference value 59–369 μmol/L); there were mildly elevated tyrosine and phenylalanine levels; diminished levels of L-aspartate, glutamic acid, citrulin, arginine and ornithine, and normal homocysteine levels at this age.\nBrain magnetic resonance imaging (MRI) and head and neck MR angiography (MRA) were done at the age of 10 to investigate the epilepsy. There were no brain lesions, but MRA demonstrated ectasia and tortuosity of both the internal carotid and vertebral arteries, particularly in the cervical segments (Figure ). The intracranial arteries were normal. Thoracoabdominal aorta computed tomography angiography was performed after the findings of the neck MRA and revealed normal results.\nAt the age of 12, 15 days after Achilles tendon tenotomy, she had a seizure followed by loss of language skills and strength at the right side of her body. She was admitted to another hospital and did not undergo brain imaging. At an office visit to our service 6 months later, on neurological exam she showed motor aphasia, right side hemiparesis with pyramidal signs. She could walk without assistance and with a hemiparetic gait.\nAnother ischemic stroke occurred at the age of 13, leading to a left hemiparesis, secondary to occlusion/sub occlusion of the distal intracranial right internal carotid artery, as demonstrated by brain MR/MRA (Figure ). It extended to the anterior and middle cerebral arteries, causing a large infarct in the area nourished by those vessels. Previous ischemic areas were also observed in the right internal globus pallidum and genu of the internal capsule, as well as in the left midbrain, pons, cerebellar hemisphere, and medial cerebellar peduncle. Laboratory screening for thrombophilia was normal, and her methionine plasma level was normal.\nADK deficiency was diagnosed at the age of 19 by whole exome sequencing, which revealed a homozygous missense variant in ADK [Hg19 Chr 10:76.468.145C>T, c.980C>T ENST00000372734, p.Ala327Val]. Segregation analysis was not performed.\nCurrently, at 19 years of age, she is wheelchair-bound with a profound intellectual disability. She is unable to sit unsupported and cannot reach or hold objects with her left hand. She speaks only isolated words and still wears diapers. Her current weight is 36 kg (<2.5th percentile), her height is 140 cm (<2.5th percentile), and her head circumference is 56 cm (50th–95th percentile). Neurological examination showed lack of head support due to muscular hypotonia, and bilateral pyramidal signs (mainly on the left side and lower limbs), contractures on her feet and hands, and hyperreflexia. Respiratory, cardiovascular, and gastrointestinal examinations were unremarkable. It is worth mentioning that there was no clinical, laboratory, or image signs of cholestasis or liver dysfunction. There was only mild elevation of ALT and ammonia. Methionine levels were normal. No new clinical or imaging events have been observed since the age of 13. |
A 79-year-old man with a history of controlled hypertension had been in a restaurant, where he had got into an altercation with another customer. He had then been hit in the chest and fell to the ground. Although he could not recall details of the situation probably due to mild alcohol intoxication, according to an eyewitness in a restaurant, the hit was a single strong one in the center of his chest. He started feeling nausea 30 min later and requested emergency services. He was brought to the emergency department of another hospital complaining of nausea. On arrival, he was alert and oriented with almost-normal vital signs except for a relatively low blood pressure (92/68 mmHg). He denied chest or back pain, or any other accompanying symptoms. No obvious wounds were evident on physical examination. Laboratory data showed mild elevation of D-dimer (2.7 µg/mL), but other parameters were almost within normal limits. Despite stable vital signs and no signs of chest injury, the physician decided to conduct computed tomography (CT) of the head and chest without contrast, based on the history of chest trauma and the unexplained symptom of nausea. No abnormalities were seen on head CT, but massive pericardial effusion was demonstrated on CT of the chest and dissection was suspected in the ascending aorta (). Aortic dissection was considered as a cause of pericardial effusion. The patient was transferred to our institute for further evaluation. Contrast-enhanced CT revealed an aortic dissection localized at the ascending aorta with massive pericardial effusion (). A three-dimensional (3D) reconstruction of preoperative CT demonstrated dissected and dilated ascending aorta with a maximal diameter of 44 mm (). Given these findings, an emergent operation was performed. After opening the pericardium, massive hemorrhagic pericardial effusion was evacuated and hematoma was revealed on the pulmonary artery side of the ascending aorta (). There was no obvious laceration on the adventitia; however, it can be contained rupture into aortopulmonary window in view of massive hemorrhagic pericardial effusion. Cardiopulmonary bypass (CPB) was initiated with an arterial cannula inserted into the undamaged right side of the ascending aorta under transesophageal echocardiography guidance according to CT finding of intact arch vessels and a venous cannula via the right atrium. In addition to an arterial cannula in the ascending aorta, one more arterial cannula was inserted into the left common femoral artery for flushing out of debris. Moderate hypothermia (bladder temperature, 28°C) was induced and the ascending aorta was opened without applying an aortic clamp. Retrograde cardioplegia was used for myocardial protection throughout the operation. For cerebral protection during circulatory arrest, antegrade cerebral perfusion was selected. Intimal tears were present on the noncoronary sinus of Valsalva and the distal part of ascending aorta located at the lesser curvature, and the ostia of the coronary arteries were intact (). All dissection sites were excluded and ascending aortic replacement with a single-branched Dacron® graft (J Graft 28/9 mm; Japan Lifeline, Tokyo, Japan) was performed. At first, distal anastomosis was performed under lower body circulatory arrest, followed by proximal anastomosis with lower body circulation using the side branch of graft connected to an arterial circuit. Weaning off CPB was uneventful and the chest was closed without difficulties.\nThe patient was transferred to the intensive care unit under stable condition. He was extubated on the day of operation and was transferred to the general ward on postoperative day (POD) 5. Postoperative contrast-enhanced CT revealed no abnormalities at sites of anastomosis, with no residual dissection. Pathological findings of the mid-part of ascending aortic wall showed hemorrhage and fibrosis in the adventitia, whereas no abnormality was seen in the media (). The postoperative course showed no complications, and the patient was discharged home on POD 24 after setting up in-home nursing care services. |
A 50-year-old Caucasian male with a medical history of gastroesophageal reflux disease (GERD), hypertension, and chronic smoking presented to the emergency department with chest discomfort. His vitals on presentation were a temperature of 98.4 F, blood pressure (BP) of 159/89 mmHg, pulse rate of 72/minute, respiratory rate of 22/minute, and oxygen saturation of 95% on room air. He did not report any significant family history of coronary artery disease or cardiomyopathies. His blood troponin levels were within normal limits and he did not have any new changes on his ECG. He was admitted to the hospital and an echocardiogram was done which showed normal left ventricular function with trace valvular insufficiencies and mildly dilated aortic root (). Acute coronary syndrome (ACS) was ruled out and the patient was referred for an outpatient exercise stress echocardiogram, which showed no evidence of ischemia. There were no changes to his medication regimen during this encounter.\nSix months later, he called emergency medical services (EMS) with episodes of stabbing chest pain associated with shortness of breath, lightheadedness, and dizziness. The patient was found to be in VF arrest en-route and was defibrillated by the EMS. On arrival, his mental status returned to baseline and the patient was conversant. His vital signs were within normal limits, and an ECG showed normal sinus rhythm but his blood work revealed elevated troponin levels that peaked at 11.74 ng/ml. An emergency cardiac catheterization was done which did not show any evidence of obstructive lesions, or focal or diffuse vascular spasms except for mild right coronary artery plaque. A computerized tomography (CT) angiogram of his chest did not show aortic aneurysm or dissection. He had frequent episodes of non–sustained ventricular tachycardia on telemetry as an inpatient (). A single chamber implantable cardiac defibrillator (ICD) was placed with settings at ventricular tachycardia (VT) monitor zone 160 and VFib zone 200 for secondary prevention of VF. He was discharged on diltiazem 180 mg daily and isosorbide mononitrate 60 mg daily.\nAbout one month later, he again presented to the hospital with spontaneous ICD shock following an episode of transient chest pain at rest, with no notable precipitating factors. An interrogation of his ICD was done which revealed VF episodes that led to ICD firing. Subsequently, he was started on sotalol and his diltiazem was switched to amlodipine 5 mg. His ICD was reprogrammed after evaluation by an electrophysiologist.\nSeven months after discharge, the patient came to the emergency department complaining of sudden substernal chest discomfort, which he rated at 10 out of 10 in intensity. His vital signs on presentation were temperature of 98.6 F, BP 166/93 mmHg, pulse rate of 69/min, respiratory rate of 48/min, and an oxygen saturation of 93% on room air. His chest discomfort improved with sublingual nitroglycerine and morphine. An ECG revealed ST elevations in lead I and aVL with reciprocal ST–T wave changes in inferior leads (). He underwent emergency cardiac catheterization with concerns for ACS, which revealed no focal coronary spasms, and non–occlusive disease with a left ventricular ejection fraction (EF) of 50–55% and mild hypokinesia in the anterolateral wall. His labs showed an elevated troponin level of 2.58 ng/ml (normal <0.03). A complete blood count, basic metabolic panel, serum electrolytes, and lipid panel were all within normal limits. A repeat ECG showed resolution of prior ECG changes (). The patient was discharged on aspirin 81 mg once a day, atorvastatin 40 mg once a day, increased amlodipine to 10 mg, sotalol 120 mg twice a day, and nitroglycerin 0.4 mg as needed for chest pain. Of note is his Imdur had been stopped a few days prior to his presentation as he had not had any recurrent` chest pain and had started using sildenafil citrate as needed for erectile dysfunction.\nOn follow up, the patient has not had any more hospitalization or further episodes of chest pain or ICD shocks on the current medication regimen. |
A 69-year-old male with known hypertension and poor dentition presented to the hospital with 5 months of dry cough, night sweats, and weight loss. Physical exam revealed a new 2/6 diastolic murmur. Investigative TEE showed a left aortic perivalvular abscess with vegetations and severe aortic insufficiency. The diagnosis of native valve subacute endocarditis was made, with blood cultures positive for Streptococcus anginosus. The patient was discharged on a 1-month course of IV Ceftriaxone and scheduled for a minimally invasive aortic valve replacement with possible aortic root reconstruction on completion of antibiotic therapy. On day 26/28 of antibiotic therapy, the patient presented to his local emergency room after having experienced a first-time seizure and loss of consciousness. He had no personal history or known family history of seizures or seizure disorders.\nOn admission, physical exam showed elevated blood pressure at 151/61 with a regular heart rate of 68, a faint diastolic murmur at the base, and obvious favoring of the right upper and lower extremities without pronator drift. Physical exam was otherwise unremarkable. The patient was given Levetiracetam 1000 mg and Dexamethasone 6 mg for seizure management.\nAt the patient’s local ED, initial contrast CT of the head showed a 1.5 cm rim enhancing lesion with no reported hydrocephalus. He was transferred to our center for further care. On the morning following transfer, the patient suffered a second left-sided seizure at which point an MRI with and without contrast was performed. The MRI revealed a 1.4 × 1.4 × 1.6 cm rim-enhancing lesion in the right middle frontal gyrus with surrounding vasogenic edema suggestive of a pyogenic abscess []. Further, both cerebral hemispheres showed focal areas of abnormal leptomeningeal and patchy cortical enhancement suggestive of early cerebritis.\nAfter visualization of the suspected abscess on MRI [], a repeat CTA was performed revealing a 1 mm aneurysmal dilation in the M4 frontal branch of the right distal middle cerebral artery (MCA) [ and ]. Imaging was highly suggestive of a mycotic aneurysmal rupture with bleed and active inflammation. A cerebral angiogram with embolization was scheduled and performed shortly thereafter. Such cases are typically treated with surgical ligation and removal but due to this patient’s poor condition, a minimally invasive endovascular approach was favored.\nVascular access was first gained through the right femoral artery. An Envoy guide catheter was advanced to the right internal carotid artery through roadmap assistance at which point a 3D angiogram was taken to better visualize the right MCA. Due to the tortuosity of the patient’s right iliac system, the guide catheter was unable to be advanced any further. Instead, left femoral artery access was gained and the vascular catheter was advanced in a similar fashion. The guide catheter was advanced to the M2 branch of the right MCA where it was exchanged for an intermediate catheter over a microcatheter over a Synchro microwire. A superselective M3 and M4 angiogram were then performed, allowing for visualization of the 1 mm infectious aneurysm in the cortical M4 frontal MCA branch [-]. On visualizing the feeding vessels, Onyx 18 material was advanced through the Synchro microwire into the M4 pedicle, filling the aneurysm []. The Envoy catheter was removed and 3-mm J-wires were advanced into the vascular sheaths. The patient remained stable for the duration of the 1-h procedure and was then transported to the neurointensive care unit (NICU) as planned.\nOne day following surgery, the patient developed seizures with partial status epilepticus. CT of the brain showed a hyperdensity in the subarachnoid space with subtle ventriculomegaly due to rupture of the IIA with subarachnoid hemorrhage (Modified Fisher Scale grade 2/Hunt and Hess grade 3) status post embolization. The patient was closely monitored for deterioration of symptoms. Two days later, follow-up CT of the brain revealed stable subarachnoid and intraventricular hemorrhage with no hydrocephalus. It was ultimately decided no external ventricular drain would be placed given the lack of hydrocephalus and stability of bleed. The patient remained in the NICU for 22 days where he was placed on Levetiracetam, Clobazam, and Lacosamide for seizure control. During this time, the patient was followed closely by neurology with daily electroencephalograms and by pulmonary/critical care as he remained intubated on mechanical ventilation post procedure. Infectious disease also followed the patient for infective endocarditis and later began triple antibiotic treatment with Vancomycin, Cefepime, and Metronidazole for ventilator associated pneumonia and right middle frontal gyrus abscess.\nOn leaving the NICU, the patient was evaluated by physical medicine and rehabilitation who initiated aggressive physical therapy, occupational therapy, and speech therapy. The patient was discharged to a hospital-adjacent rehabilitation center 1 week later where he continued to undergo physical, occupational, and speech therapy for 1 month.\nFour months after the patient’s initial hospital discharge, he was deemed a suitable candidate to undergo cardiothoracic surgery. The patient underwent an aortic and mitral valve replacement without complication and was discharged home after 4 days of hospital care.\nFor 6 months, the patient remained out of the hospital with mild lifestyle modifications and changes to mood (including some confusion) which improved on cessation of steroid therapy.\nThe patient was readmitted to the hospital 6 months after cardiac surgery when he suffered a left sided seizure with associated Todd’s paralysis attributed to possible nonadherence. During the admission, he suffered a pulmonary embolism for which an IVC filter was placed. He was discharged home again with daily speech, occupational, and physical therapy. Since the episode, the patient had no major hospital admission and has been adherent to his antiseizure regimen of Levetiracetam, Lacosamide, Topiramate, and Clobazam.\nAt present, the patient is 1½ years status post initial rupture of the IIA. The patient is currently alive, and his mood has significantly improved per both his family and healthcare team. He has suffered several minor seizures in the interim due to medication titrations. However, he has reached the point where he is again confident enough and possibly eligible to begin driving school. |
The patient was an 18-year-old female who referred to Rheumatology Clinic in Alzahra Hospital. She was complaining from low back pain and paravertebral muscle spasm since two weeks prior to admission. The pain was mainly localized in the right side of her waist and this sudden onset of the pain made her troubles walking. Three days later, she found the pain radiating to her left ear and her lower left molar teeth. At this time, she got aware of a painless huge mass in the left side of her neck. She complained from weight loss about 3 kg over 6 months but she denied any fever or other constitutional symptoms. Increasing pain and the mass in the left side of her neck caused her seek for medication. On admission, she was stable and afebrile. Her left radial pulse was undetectable, but other pulses were completely normal. No sign of ischemia was present on her limbs. A soft and pulsatile mass was observed in the left side of her neck. On auscultation, vascular bruit was audible on the carotid arteries. No positive finding was detected in neurological examination, and fundoscopic evaluation of the eyes was also normal. Left and right arm blood pressures were 80/60 and 120/80 mmHg, respectively. Right and left foot had equal pulse pressure (120/80 mmHg). The laboratory data is summarized in .\nColor Doppler of carotid arteries showed a left common carotid artery aneurysm with dimensions of about 15×17×21 mm with increased intima and media wall thickness in site of dilation. Computed tomography (CT) angiography revealed aneurysmal dilation at mid common carotid in a length of about 4 cm. Diameter of aneurysm at the most dilated portion was about 15 mm. In addition, diameters proximal and distal to aneurysm were 5 mm and 4 mm, respectively. Left carotid distal to aneurysm was well visualized. Rest of carotid and vertebral arterial system showed normal caliber with no significant stenosis. No definite plaque formation was identified. In the region of aortic area, left vertebral artery was arising from the aortic arch. Left subclavian artery was not visualized. There was also narrowing at the origin of right subclavian artery ().\nThe chest radiography, abdominal sonography, and thyroid gland sonography were normal. Echocardiographic findings were compatible with trivial mitral and tricuspid regurgitation. Based on these vascular data, unequal arm blood pressure, leukocytosis and elevated ESR, the diagnosis of the Takayasu's arteritis was made.\nHeparin infusion was started for the patient (2500 units every 3 hours) followed by oral warfarin therapy. She received prednisolone tab (50 mg) daily (in divided doses). She was also treated with infusions of monthly 700 mg cyclophosphamide pulse. After 6 cyclophosphamide pulses, a significant improvement was seen, constitutional symptoms vanished, neck mass was reduced 50% in size, and ESR got the normal range.\nThe left radial artery found to have a weak pulse. Totally, she received 6 pulses of cyclophosphamide within six months. Then, she was treated with 15 mg intramuscular methotrexate per week, 1 mg oral folic acid daily, and 80 mg aspirin daily. In addition, prednisolone was tapered. Like any other patient on chronic corticosteroids, to prevent osteoporosis, she was prescribed with calcium, vitamin D, and a bisphosphonate and perform weight-bearing exercises. Modifiable risk factors for atherosclerosis especially hypertension, smoking, inactivity, diabetes, and hyperlipidemia should be treated maximally. |
A 71-year-old female was referred to our quaternary referral center with a hilar biliary stenosis suspicious for a hilar cholangiocarcinoma (Bismuth classification 3A). She suffered from progressive jaundice, itching, and weight loss (6 kg) for the last 4 weeks. Blood test revealed an elevated bilirubin of 256 µmol/L. She had a history of clinically stable Crohn's disease treated with infliximab. In 2013, she underwent curative treatment for a pT1cG1No infiltrating ductal adenocarcinoma of the left breast. Computed tomography (CT) scan revealed a tumor mass with encasement of the right hepatic artery and right portal vein, which was considered surgically resectable by an extended right hemihepatectomy after internal biliary drainage of the future liver remnant. Due to an insufficient volume of the left lateral liver remnant (22% of the total liver volume), we performed a percutaneous ipsilateral embolization of the segmental right portal vein branches, using polyvinyl alcohol embolization particles (ContourTM PVA Embolization Particles 45–150 μm, Boston Scientific) and interlock coils (Interlock-18 Fibered IDC occlusion system, Boston Scientific). A postprocedural control angiography showed coiled right portal veins with a patent common and left portal vein (Fig. ). Two days after this uneventful procedure, she presented at the emergency room with fever (39.5°C) combined with nausea and headache. A CT scan was performed and revealed a successful embolization of the right portal vein; however, also a detrimental total occlusion of the common and left portal vein due to a massive thrombosis (Fig. ). She was admitted to the hospital and treated with antibiotics intravenously (Clindamycin 600 mg 3 times daily and ciprofloxacin 400 mg twice daily). Immediate portal vein thrombolysis was attempted by continuous intra-arterial infusion of the thrombolytic agent urokinase into the selectively catheterized superior mesenteric artery. Unfortunately, after 3 days of intra-arterial infusion of urokinase, no signs of improvement were seen on the control CT scan. A transhepatic or transjugular retrograde cannulation of the portal vein was considered to be a nonrealistic treatment option due to the extent of thrombosis. No other minimally invasive treatment options were left to solve this major complication. Therefore, we attempted revascularization of the future liver remnant portal vessels by combining a mechanical thrombectomy of the left and common portal vein together with intraluminal catheter-based thrombolysis via a surgically reopened umbilical vein.\nAfter induction of general anesthesia, we performed a (limited) bilateral subcostal laparotomy. No intra-abdominal signs of metastatic disease were found. After ligation of the umbilical ligament, we re-opened the obliterated umbilical vein with surgical forceps in a retrograde fashion until some retrograde backflow from the left portal vein was obtained. Next, the reopened umbilical vein was used to introduce a Fogarty balloon catheter into the left portal vein, and a significant amount of thrombus was removed from the common and left portal vein. Finally, we introduced a central venous catheter (CVC) through the umbilical vein and positioned the tip of the CVC at the base of the portal vein. We fixated the CVC to the umbilical ligament, peritoneal side of the abdominal wall and skin. After the operation, thrombolysis was initiated via the CVC with strict observation of the hemodynamics, plasma fibrinogen concentration and daily angiography via the CVC. Pathologic report of the removed thrombus showed a fresh thrombus with no signs of malignancy (Fig. ). Control angiography after 1 day of thrombolytic therapy revealed recanalization of the left portal vein and portal vein main stem, but also remnant mural clots (Fig. ). Intraportal thrombolytic therapy was continued for another 2 days, after which control angiography demonstrated complete resolution of the PVT and the branches of the left portal vein to segment 2 and 3 of the liver (Fig. ). Thrombolytic therapy was discontinued, and a therapeutic dose of low-molecular-weight heparin was started. The same day, the patient developed abdominal pain, and a CT scan revealed a subscapular hematoma at the right side of the liver as complication of the thrombolysis and full-dose heparin treatment (Fig. ). The therapeutic dose of low-molecular-weight heparin was discontinued, and fortunately repeated imaging showed no progression of the subcapsular hematoma. After 4 weeks, CT volumetric analysis revealed a future liver remnant volume of 31% of the total liver volume. Accordingly, the patient underwent an uncomplicated extended right hemihepatectomy, including resection of the caudate lobe and extrahepatic bile ducts, followed by a hepatico-jejunostomy reconstruction. Because of a small persistent partial common PVT, we resected 1 cm of portal vein and reconstructed the vein by an end-to-end anastomosis between the portal vein main stem and the left portal vein. Pathology report stated a 4-cm hilar cholangio-adenocarcinoma with invasion of the gallbladder with free resection margins and metastases in 3 out of 10 resected hilar lymph nodes (Fig. ). After 19 days of an uneventful postoperative stay, the patient left the hospital in a relatively good clinical condition. She remained well for 2 years after surgery without any signs of recurrent PVT. Unfortunately, a CT scan performed due to abdominal complaints 2 years after the surgery revealed local tumor recurrence for which palliative care was started. |
A 54-year-old female was brought to the hospital by EMT (emergency medical team) with suspicion of opiate overdose. EMT was called by a family member after the patient was found to be less responsive and difficult to arouse. The patient received 4 mg of naloxone subcutaneously in field by the EMT which improved patient's responsiveness but pushed her into severe opiate withdrawal. In the emergency department, the patient was found to be awake, alert, and anxious. She was not oriented but was able to follow simple commands. The patient was also diaphoretic, tachypneic with respiratory rate in 30 and tachycardic with heart rate in 150 s. Lungs were clear on auscultation with good bilateral air entry. Cardiovascualr exam was unremarkable with regular rhythm good pulses present in all four extremities. Abdominal exam was also unremarkable. Neuromuscular exam showed tremulousness and muscle fasciculation in different muscle groups of the face, upper limb, and lower extremities, exacerbated deep tendon reflex and normal motor strength with severe pain on movement of left hip. The patient had a past medical history of AIDS, hepatitis C, and bipolar disorder but as per her home medication list she was not on any treatment for them. At home, the patient was on 150 mg of methadone for maintenance and oral morphine sustained release and immediate release for pain control for her recent hip fracture. The first ABG done at the time of patient's ED presentation was on a nonrebreather mask; it showed pH 7.694, PCO2 19.6 mmHg, PO2 224 mmHg, O2 saturation of 100%, and lactate of 2.97 and her initial EKG showed bigeminy with the rest of the details obscured by artifacts as seen in . In the next 30 minutes patient's respiratory rate had decreased to 18–25 breaths per minute at which time a repeat ABG was done on room air that showed pH of 7.641, PCO2 20.3 mmHg, PO2 of 86 mmHg, and O2 saturation of 98% with lactate of 2.48. While being on telemetry, the patient had sinus tachycardia with PVCs and runs of bigeminy. The patient was subsequently deemed stable and taken for CT scan of heat and chest for further evaluation, while in CT room the patient went into generalized tetany and then into cardiac arrest with initial rhythm of ventricular tachycardia that degenerated to torsade de pointes and ventricular fibrillation. Code was run for 25 minutes, during which the patient was intubated and received 2 doses of epinephrine and 4 gm of magnesium and was shocked 3 times. She had a return of spontaneous circulation after the third shock. The patient was initially started on hypothermia protocol; however, it was abandoned as patient showed movement of extremities. The labs obtained prior to patient's cardiac arrest showed Na+ 138, K+ 3.4, Mg+ 2.0, Ca2+ 8.9, HCO3 18, and anion gap of 16; however, this sample was hemolyzed. Repeat labs obtained 30 minutes after intubation showed K+ 3.5, Ca2+ 7.6, iCa2+ 3.92, Mg+ 2.6, and HCO3 16 with anion gap of 17. EKG obtained after cardiac arrest showed sinus tachycardia. Urine toxicology obtained for the patient was positive only for methadone and opiates. Patient's serum albumin level was 2.9. The patient was extubated on day 2 and was discharged from hospital on day 8 of hospitalization. |
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