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A 36-year-old male with a medical history of HIV presented to our institution with behavioral changes characterized by decreased responsiveness. He had been diagnosed with HIV after routine screening 14 years prior. His HAART regimen consisted of abacavir-lamivudine, atazanavir, and ritonavir. At baseline, he was able to ambulate and converse without difficulty. Four months before presentation, the patient was noted to have stiffening of his neck and back. During this time, the patient also developed decreased verbal output. When the patient's family first noticed these symptoms, the patient was transferred from his previous residence in a group home into his parents’ house. While residing with his parents, the patient stopped taking his HAART. His mother reported that his symptoms initially improved, but he later developed further worsening of his speech and gait. His mother restarted his HAART, however, the patient continued to worsen to the point of multiple falls and virtually no verbal output. This prompted his family to bring the patient to the emergency room. When admitted to an internal medicine service, he was found to have minimal verbal output, minimal responsiveness to verbal stimuli, and decreased movements. The patient was continued on his HAART. Empiric acyclovir was started to cover possible viral encephalitis. Psychiatry was consulted for possible catatonia. He was given lorazepam without improvement. After failure of this benzodiazepine challenge, neurology was consulted.\nOn our neurology team's initial assessment, the patient was awake and alert but was minimally verbally or physically responsive. He would mouth words, without audible response. He had rigidity in all extremities, more severe in his lower extremities. There was no significant tremor. He also had diffuse hyperreflexia and sustained bilateral ankle clonus. He required assistance to stand, had difficulty initiating movements, had stooped posture, and had a magnetic gait.\nMagnetic resonance imaging (MRI) brain showed extensive leukoencephalopathy with periventricular involvement and subcortical extension in the bilateral frontal and parietal lobes []. Given the symmetry of leukoencephalopathy, degree of global cerebral atrophy, and lack of gadolinium enhancement, this likely represented sequela of HIV encephalopathy. The patient also had subtle mineralization of bilateral basal ganglia. MRI spine was unremarkable. His absolute CD4 count was 208 cells/mcL, and HIV viral load was 36378 copies/mL. Serum analysis revealed a borderline thiamine level at 64 nmol/L. Otherwise serum analysis was unremarkable, including normal ammonia, Vitamin-B12, and folate level. Serum toxoplasma antibodies and rapid plasma reagin were negative. Blood bacterial cultures and mycobacterial cultures were negative.\nCerebral spinal fluid (CSF) analysis revealed 94 mg/dL protein, 58 mg/dL glucose, 2 white blood cells/mcL, and <1 red blood cell/mcL. Serum and CSF cryptococcal antigen were negative. CSF herpes simplex virus, varicella virus, and John Cunningham virus polymerase chain reactions were negative. CSF West Nile virus IgM and IgG were negative. CSF cultures were negative, including bacterial, mycobacterial, and fungal.\nGiven the patient's clinical presentation and radiographic findings, the patient was started on carbidopa-levodopa. This was initiated at 12.5–50 mg TID with gradual titration up to 87.5–350 mg TID (total levodopa 1050 mg daily). With titration of levodopa to 1050 mg daily, the patient had improved extrapyramidal signs and symptoms. He had increased facial expression and improved fluidity and spontaneity of speech. His bradykinesia and gait apraxia also improved significantly. He was also started on amantadine 100 mg twice per day as recommended by the physical medicine and rehabilitation physicians to optimize participation with rehabilitation. Before initiation of amantadine, the patient had already reached the optimal carbidopa-levodopa dose (total levodopa 1050 mg daily) and had shown significant symptomatic benefit. The patient was discharged on HAART, amantadine, trimethoprim-sulfamethoxazole prophylaxis, thiamine, and carbidopa-levodopa 87.5–350 mg TID.\nThe patient was evaluated in clinic 3 months after discharge. He was compliant with the carbidopa-levodopa regimen. He retained the level of functionality he had regained at discharge. Furthermore, he did not display any adverse effects, specifically no dyskinesias or any adverse psychological reactions.
A 42-year-old male was assaulted by four persons, and remembers that some of them jumped on his left knee. He was not sure about the exact mechanism of injury, as he stated that he was under the influence of alcohol. The patient presented with swelling of his thigh and inability to weight bear on the left leg. There was bruising and a palpable depression (Fig ) of the left quadriceps tendon. The patient was able to extend the knee, but not against gravity, with an extensor lag of 30°.\nThe patient reported that, 27 years before the present injury, he had been involved in a road traffic accident sustaining ipsilateral femur and comminuted patellar fractures. The femur underwent intramedullary nailing. The patella was removed, and primary repair of the extensor mechanism was performed. Six years later, the intramedullary femoral nail was removed. In the patient's past medical history, there were no other significant problems to note, and there was no history of quinolones intake. The patient had been generally fit and well.\nPlain radiographs of the knee showed a depression in the soft tissue shadow anterior to the distal femur, extraosseus calcification along the tendon, and absence of the patella (Fig ). Other imaging investigations were not performed, as the clinical signs were classical.\nWe elected to perform a primary quadriceps repair within 24 hours of the injury. A midline incision was made over the previous scar. A complete quadriceps rupture was noted approximately 3 cm proximal to the patellectomy site with a 2.5 cm gap (Fig ). The previous extensor mechanism repair site was well healed. The medial and lateral retinaculums were intact. An end to end repair was performed using continuous, locked heavy absorbable sutures (size 1 Maxon-Monofilament polygyconate synthetic suture, United States Surgical, USA) in multiple layers (Fig ). The ragged margins of the tendon were excised and sent for histopathological examination. This showed disorganisation of collagen fibrils and increased ground substance (mucin like material), lymphocyte infiltration, neovascularisation and fibrins at the rupture site. These findings were consistent with chronic hypoxic tendon degeneration following patellectomy (Fig ), on which superadded changes from the rupture were found (Fig ).\nThe repair was protected with a knee brace for 6 weeks. During that period, the patient was allowed partial weight bearing mobilisation with one crutch during the initial 6 weeks, and advised to wean it off gradually over a period of 2–3 weeks time []. He was encouraged to put full weight bearing at the time of weaning the crutch. The knee brace was removed after 12 weeks. We advised him to refrain from work for 12 weeks. Post-operative recovery was complicated by a superficial infection which settled with 2 weeks of oral antibiotics. We encouraged the patient to start straight leg raise exercises with the knee brace, and gravity assisted passive knee bending from the 4th week. Physiotherapist started closed chain exercises at 8 weeks; and open chain exercises at 12 weeks, and were increased in intensity and duration at 24 weeks of surgery.\nAt 7 months from the injury, there was no extension lag, with a range of active knee flexion from 0° to 100°. Further flexion was restricted by tightness, mild discomfort and pain. The isometric extensor strength of the knee extensors was measured []. The mean of three measurements was taken, and compared to the normal side. The knee extensor strength was (771 N) 44% less when compared to contralateral normal quadriceps strength (1386 N).\nThe overall functional outcome was measured using the International Knee Documentation Committee (IKDC) knee score. This was 121.6 before injury and 51.8 after surgery. A score of 100 is interpreted to mean no limitation with activities of daily living or sports activities and the absence of symptoms. The patient is unable to play football, run and jog, but he is able to speed walk, and has returned to his pre-operative work (which was reliant on his ability to drive) 6 months after this injury.
An 18-year-old female patient presented with fever and weakness in the upper limbs with right hand affection more than the left. Two years before presentation, she used to have fever in the evening and, gradually, she developed a persistent fever in the last 6 weeks without cough or weight loss.\nShe gave a past history of hypertension, which had been diagnosed when she was eight years old. She was regular antihypertensive medication (amlodepine and aldomet). The dosage of her medications had been decreased gradually by her physician over the last few months and were finally stopped completely based on a belief that her hypertension was spontaneously cured! She also gave a history of abdominal operation at the age of 8 years for a right suprarenal mass after discovering her hypertension, and the histolpathological report revealed fatty tissue changes and not pheochromocytoma, as was believed to be, before the operation. The hypertension was not cured after the operation and the antihypertensive medications were resumed.\nThere was no family history of the same illness or TB in her family members.\nThe patient looks ill, pale, underweight (body weight was 45 kg, height 162 cm and BMI = 17). The right upper limb pulsation was absent while it was normal in the left arm, with high force and volume in both lower limbs. Carotid pulsations were present in both sides. Blood pressure (BP) measurement was taken in the four limbs and was recorded as the following: no BP in the right upper limb, 110/70 mmHg in the left upper limb and 200/130 mmHg in both lower limbs.\nThe neck examination showed a significantly enlarged right cervical lymph node measuring 3.5 cm × 2.8 cm, which was firm, not tender and not fixed to the underlying structures.\nCardiac examination showed normal heart sounds at the apex of the heart with a loud S2 at the base and systolic bruit at the left supraclavicular fossa. Other systemic examinations were within normal limits. Chest and abdomen examinations revealed no abnormal findings.\nHemoglobin was 9 gm/dl, ESR = 120 mm in the first hour, C-reactive protein was 20 mg/dl, total white blood cells was normal with predominant lymphocytosis and normal platelet count. The serological study for ANA, cANCA and pANCA were all negative, her liver function test, renal function test, thyroid function test and urine analysis were all within normal limits. The Mantoux test for TB was performed and it was strongly positive (25 mm).\nECG showed sinus tachycardia with left axis deviation and signs of left ventricle enlargement.\nThe chest x-ray and abdominal ultrasound were normal. Echocardiography showed left ventricular hypertrophy with normal other internal dimensions and normal valves. Duplex vascular ultrasound revealed total total occlusion of the right subclavian artery with poststenotic flow pattern while the left subclavian artery was significantly narrowed.\nAn aortogram revealed normal aortic arch with total occlusion of the right subclavian artery [], with significant narrowing of the left subclavian artery []. Abdominal aorta angiography showed right renal artery stenosis with normal left renal artery []. It also showed good collaterals for right upper limb and a nonsignificant lesion at the origin of the left common carotid.\nHistolpathological report of the right cervical lymph node biopsy showed caseating tubercles suggestive of granulomatous tuberculous lymph adenitis.\nStandard anti-TB treatments with steroid were given in addition to antihypertensive medications and aspirin. The patient was improved significantly and the symptoms and cervical lymph nodes disappeared. She was discharged, to be followed-up in the outpatient clinic.
We present the case of a 51-year-old woman with a history of Guillain-Barré syndrome as a teenager, probable neuroborreliosis in the 1980s, and a malignant melanoma in situ in the 1990s. She has given informed consent to this report. The patient had repeat rectal surgeries for sphincter damage after childbirth, and in 2011, she was diagnosed with a T4 rectal cancer causing haemorrhage. No distant metastases were found and treatment with curative intent was decided at a multidisciplinary team conference. She had neoadjuvant chemotherapy with fluorouracil and leucovorin followed by radiation with 50.4 Gy to the rectum. The tumour was resected half a year after the diagnosis. The postoperative course was uneventful except for a superficial wound infection and she was discharged after 3 weeks of hospitalization. After 2 weeks, she was readmitted because of a Takotsubo cardiomyopathy and intestinal obstruction requiring more surgery with resection of a short segment of small bowel. After 1 year, the first lung metastases were diagnosed with a 2-cm metastasis in the right lower lobe and a 5-mm. tumour in the lower left lobe. After 3 pulmonary resective procedures, further lung metastases were seen during follow-up. A second line of chemotherapy was started with oxaliplatin, resulting in shrinkage of the noted lesions. Then, local treatment was done with SBRT, 17 Gy ×3 for 2 right-sided metastases and a resection of the left lung, 3 years after the resection of the primary tumour. Three months later a new lung metastasis was diagnosed and treated with SBRT, 7 Gy ×8. Six months later, 2 more lung lesions were found and a third line of chemotherapy based on irinotecan was started, but as tumour progression was observed, treatment was discontinued after 3 months.\nWithout having the final test result on microsatellite instability in the tumour, it was decided to start PD-1 treatment (this was not within a clinical trial, since the only available trial in Sweden had not opened yet). Further resections were not possible and the given dose of radiation precludes further SBRT. There were a total of 6 confirmed metastases on both sides after the first 3 doses of pembrolizumab (standard dose with 2 mg/kg every third week). The CT evaluation showed size increase of all lung metastases, but no new lesions. The only side effect was fatigue. This early evaluation did not contradict further therapy, but before continuing with the immunological treatment, local ablative treatment with microwaves was proposed, as this has become a standard treatment for liver tumours, and also evidenced with a growing pile of international publications on treatment of lung lesions [, , , ]. The tumours were deemed treatable and were, after 2 more doses of pembrolizumab, done so in 2 sessions 3 weeks apart using a computer-assisted targeting system (CAS-one; Cascination AG, Bern, Switzerland) and microwaves (Angiodynamics) under immobilization of the lungs with high-flow jet ventilation as described in a previous publication []. The patient stayed in hospital overnight after the ablations. During the first ablative session, a pneumothorax was caused and a 16F chest tube with suction was placed and removed after clamping the next day. Images of the procedures are shown in Figure .\nPD-1 treatment was re-started after 2 weeks. After the sixth course of pembrolizumab, the patient felt pain over the liver area, and experienced low appetite and some weight loss. Further investigations showed no signs of liver metastases or damage, but a drop in serum albumin and elevated ESR and CRP. This rise in inflammatory parameters was judged as potential reaction on PD-1 inhibition and treated with betamethasone. For better pain control, amitriptyline was added to the standard analgesic regimen as there could be a neurogenic pain component. After 3 weeks, the seventh dose of pembrolizumab could be given. Two weeks thereafter, the patient experienced weakness of her left lower leg, finger tremors, and numbness on the ulnar side of the right hand. A neurologist ruled out any suspicion of new metastases or any systemic neurological disease. The symptoms were regarded as side effects of pembrolizumab, although mechanistically not fully understood. The dose of corticosteroids was increased and the eighth course of pembrolizumab was given with no worsening of symptoms.\nImages from a follow-up computed tomography are shown in Figure where an inflammatory reaction is initially seen with focal lesions, pseudo-tumours, that later disappeared. After 8 months of follow-up, there have been no signs of new or recurrent lung metastases.\nPresently, the patient is still suffering from neurological symptoms that in spite of efforts giving higher doses of steroids are not declining. The other complication is upcoming diffuse non-malignant lesions in both lungs, interpreted as either inflammatory or infectious. These findings were accompanied by shortness of breath and difficulties to walk longer distances. Steroid treatment has only partially alleviated the symptoms, as has antibiotic treatment, covering both gram-positive bacteria and Pneumocystis carinii. Further investigations are ongoing. As mentioned, the lung metastasis has not recurred, giving her time off anti-tumoural treatment.
A 158 cm, 93 kg (BMI 37.4), 52-year-old woman presented to a tertiary care center for evaluation of a Merkel cell tumor in her right forearm. Wide resection of this forearm tumor and an axillary lymph node dissection were successfully performed. During the course of her evaluation, however, a suspicious mass in her right upper lobe was identified on chest X-ray, and a 2.7 × 2.2 × 2.9 cm hypermetabolic solid nodule in the right apex was confirmed with CT imaging. The patient was asymptomatic. Preoperative pulmonary function tests were normal. Patient was deemed to be optimized for a thoracic surgical procedure and was brought to the operating room.\nAfter routine intravenous sedation with Midazolam and Fentanyl, an epidural was placed at the T6-7 vertebral interspace. Patient was returned to the supine position where general anesthesia was induced intravenously with fentanyl, lidocaine, propofol, and succinylcholine. Her airway was secured via direct laryngoscopy (grade one view) and placement of a styleted 35-French left double lumen endotracheal tube without difficulty. Once the tracheal cuff had passed through the cords, the stylette was removed and bilateral breath sounds were confirmed. Proper placement of the bronchial lumen of the tube in the left main bronchus was confirmed with fiberoptic bronchoscopy. The patient was placed in lateral position for her surgical procedure, and proper placement of the DLT was once again confirmed with the fiberoptic bronchoscope. No blood or signs of bronchial or tracheal trauma were observed.\nFollowing initiation of single lung ventilation, a surgical decision was made to proceed with a right upper lobectomy which was performed in the usual fashion. Individual branches of the pulmonary artery to the right upper lobe were identified and doubly ligated with 2-0 silk suture. Right superior pulmonary vein was mobilized. Branches going to the right upper lobe were identified and divided with silk stick ties and free ties. The fissures between the middle lobe and upper lobe were completed with multiple firings of a GIA stapler. All interlobar lymph nodes were resected. The right upper lobe bronchus was sharply dissected free. The right lung was reinflated and the bronchial stump was competent to 40 cm pressure; however, with positive pressure ventilation and the right hemithorax filled with saline, a small air leak was identified. Further evaluation revealed the air leak to be arising from a long linear tear located along the left mainstem bronchus and extending from the carina as far down the left mainstem bronchus as could be visualized. The double-lumen endotracheal tube was identified in the region of injury. This left main bronchus injury was not evident during the course of the case because the bronchial balloon had successfully isolated the left lung. The injury and resultant air leak only became apparent with deflation of the bronchial balloon and the resumption of right lung ventilation.\nDue to the location of the injury and concern that the current exposure would not provide adequate visualization for surgical repair, a right thoracotomy was performed. The bronchial lumen of the DLT was inserted distal to the defect with the use of a flexible bronchoscope and surgeon observation. With the ability to ventilate the lung distal to the airway rupture, the defect was repaired with a combination of a pericardial patch and pleural flap. No air leak was identified upon completion of the repair, and the chest was closed in the usual fashion.\nThe patient was returned to the supine position, and the anesthesiologist removed the double lumen endotracheal tube under direct vision with a video laryngoscope and placed a single lumen endotracheal tube (Figures and ). At this time, a bronchoscopist conducted a thorough examination of the airway, revealing findings consistent with a right upper lobectomy with intact surgical stump. The remainder of the visible tracheobronchial tree on the right side was normal and fully patent. Examination of the left tracheobronchial tree, findings consistent with a left mainstem bronchial injury beginning approximately 0.5 cm below the main carina and along its medial wall and continuing down to approximately 1 cm above the intermediate carina separating the take-offs to the left upper and lower lobes. The injury itself included a portion of bronchial mucosa emanating into the airway although the airway was fully patent. There was no evidence of residual injury, or leak and distal airways were fully patent (). A single blood clot was evacuated from the airway.\nThe patient was allowed to emerge from anesthesia in the postanesthesia care unit and was making excellent respiratory effort with adequate tidal volumes. She was successfully extubated and observed for any signs of respiratory difficulty in the PACU for approximately 2 hours. She was discharged to the ICU for observation and recovered without any further complication.
We report a 35-year-old Saudi woman with the diagnosis of transposition of the great arteries, ventricular septal defect, severe pulmonary stenosis, a sub-aortic chamber, and a rudimentary right ventricle. She had been diagnosed with these conditions at three years of age and was under regular follow-up at our center. Total cavo-pulmonary anastomosis with the lateral tunnel procedure was performed at the age of 13.5 years. She was kept on Aspirin as antithrombotic treatment for one year after Fontan.\nOur patient experienced menarche at 13 years, but had irregular cycles with menorrhagia. Over the subsequent few years she did not report to the hospital. She conceived repeatedly, had five miscarriages, and delivered three low birth weight neonates. All of her previous pregnancies were spontaneous vaginal delivery (SVD) at a peripheral hospital (Table ). At the time when she presented to us she was pregnant and had three live babies. The first two neonates were full term births the third baby was pre-term baby at 30 weeks of gestation, who was admitted to Neonatal Unit for three weeks for feeding purposes.\nShe was on regular antenatal follow-ups at our center in her last pregnancy. Holter was repeated which showed normal sinus. Transthoracic echocardiography was done showing no Fontan pathway obstruction and no fenestration was seen. There was a mild right and left atrio-ventricular valve insufficiency with good SV function. On routine laboratory investigation, she had iron deficiency anemia. Her Hemoglobin was 10.6 g/dl. She was prescribed iron and multivitamin tablets. She received Aspirin throughout her pregnancy.\nA fetal echo was performed at 16 weeks’ gestation and showed normal cardiac anatomy for the fetus. Her Electro-cardiogram (ECG) and clinical examination during these antenatal visits were normal. Because of less chance of complications, instrumental vaginal delivery is the preferred mode in these patients according to the Association of European Pediatric Cardiology. However, at 36 weeks of pregnancy, our patient had elective cesarean section (CS) because of obstetric preference. Her aspirin was stopped 48 h prior to CS. She delivered a full-term, low birth weight male neonate who weighed 2.1 kg. She was reviewed at a later date by the joint cardiology–obstetric team. She was prescribed sub-cutaneous Enoxaparin and Aspirin for six weeks after CS then she was advice to continue on Aspirin 81 mg once a day, iron and multi vitamin tablets. She was counseled in detail regarding contraception and avoiding further pregnancies, and to regularly have follow-up with the adult congenital heart disease team.\nAt a follow-up visit, 6 weeks after delivery, she was asymptomatic with New York Heart Association functional class II. Her physical examination was unremarkable and oxygen saturation was 93% in room air. N-terminal prohormone of brain natriuretic peptide (248 pg/mL), total protein, and albumin levels were in the normal range.\nA chest X-ray showed a normal heart size with normal pulmonary vascular markings. ECG showed normal sinus rhythm with a heart rate of 70 beat per minute and normal QRS duration. A transthoracic echocardiograph showed a patent Fontan pathway with mild left-sided atrio-ventricular valve insufficiency and good SV function. A treadmill exercise test was performed and she tolerated 8 min of exercise, with 85% achievement of her predicted values. Her 24-h Holter recording was negative for arrhythmia and the average heart rate was 80 bpm.
A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.\nFour days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.\nUpon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.\nThe abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.\nNo bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8.
The patient was 50 years old (1987) when he suffered from a 4 cm left renal mass with no evidence of metastasis for which left radical nephrectomy was performed. Histopathology revealed clear-cell renal cell carcinoma (cRCC), grade II, stage pT1a, with negative surgical margins. The patient was then followed up regularly.\nIn 1995, the patient had a malignant melanoma excised from the left arm. He also underwent Whipple operation in 1996 for a neuroendocrine tumor of the pancreatic head that recurred in February 2000 and partial resection of the pancreas with intraoperative irradiation of surgical bed was done.\nIn October 2000, lower cuts of the contrast-enhanced chest CT revealed a small 8 mm basolateral left pulmonary nodule and metastasectomy was performed. Histopathology revealed the first reported recurrence of the cRCC 13 years after the initial diagnosis with negative surgical margins.\nIn November 2001, the patient underwent right lobar thyroidectomy for asymmetrical thyroid enlargement revealing metastasis of the cRCC with negative surgical margins. Afterwards the patient started interferon-alpha (INF-α), interleukin-2 (IL-2), and 5-fluoruracil for 8 weeks as first- line adjuvant systemic therapy for mRCC.\nIn December 2003, brain CT for severe headache was performed, revealing a 3 × 2 cm focal metastasis in the brain, likely of cRCC type, with temporal lobe hemorrhage. The patient survived the incident and received palliative radiotherapy on this lesion in 2003, 2004, and 2005 (see ).\nIn November 2007, 20 years after the first renal tumor therapy, a 5 cm local tumor recurrence appeared at the left retroperitoneum in the contrast-enhanced CT scan. The cancer recurrence was surgically excised. Recurrence was also observed in the remaining part of the thyroid gland (4 cm), which was again excised and revealed cRCC. Therefore, a second line of systemic therapy with sunitinib (Sutent 50 mg once daily, 4 weeks on and 2 weeks off) was started. The patient received 30 cycles of sunitinib until September 2011.\nIn September 2011, a suspicious 3 cm hepatic focal lesion appeared in the regular follow-up which was operated on through laparotomy and excision of segment IVa, revealing cRCC, GII. Therefore, the regimen was shifted in November 2011 to a third line of systemic therapy, 10 cycles of temsirolimus (Torisel 25 mg, Pfizer; i.v. injection over 30–60 minutes) till follow-up CT in January 2012 revealed small subpleural focal lesion (2 mm) and a posterobasal lesion in the lower left lung lobe (1 cm). The regimen was shifted to a fourth line of systemic therapy with oral sorafenib twice daily (Nexavar 400 mg, Bayer).\nIn October 2012, the follow-up CT showed progressive enlargement of the pulmonary metastasis up to 4 cm and appearance of a new focus at the apical part of the lower right lung lobe and five metastatic foci in the liver. The institutional tumor board decided to start with the fifth line of therapy, bevacizumab (Avastin, Genentech Roche) 10 mg/kg i.v. injection every 2 weeks and INF-α 3 μIU subcutaneous injection three times/week since January 2013. The patient received 18 consecutive cycles of this therapy.\nIn November 2013, two osteolytic bone lesions appeared in two right ribs and the lung metastases progressed. The local tumor board decided radiotherapy of the osteolytic lesions and to start the sixth line therapy with axitinib (Inlyta, P fizer; 5 mg twice a day). Due to intolerable side effects (fatigue and diarrhea), the patient soon stopped the medication on his own.\nIn December 2013, a seventh line therapy was initialized with everolimus (Afinitor, Novartis; 10 mg once daily). Because of progressive enlargement of the lung, liver, and right hilar LN metastases, treatment shifted in June 2014 to eighth line of systemic therapy for mRCC, pazopanib (Vortrient, GSK, 400 mg oral tablets twice daily).\nCurrently, the patient is still coming for regular follow-up checks to our clinic, getting the pazopanib (his 8th line of systemic treatment) as scheduled up to the day. However, the patient is suffering from multiple metastatic symptoms in liver, ribs, and lung as previously described (see ).
A 43 year old male, Muslim Yoruba farmer residing in Osun State of Nigeria presented at the outpatient unit of Osun State Hospital, Asubiaro Osogbo, with complaints of toothache in the lower left jaw of 2 weeks duration as well as cough, dysphagia, left earache and swelling of the neck and face of 5 day duration. The Patient was in his normal state of health until about 2 weeks before presentation when he developed toothache in the lower left quadrant of the jaw. He was advised by his friend to apply acidic chemical used to recharge car batteries to rinse the mouth. On first application of the acidic chemical, he experienced burning sensation and pain making him to immediately rinse with water, which gave him some relief. Some minutes later he reapplied the acidic chemical and held it in the mouth for some minutes “so as to derive maximum benefit” as advised by his friend which he later rinsed off with water but without much relief from the burning sensation and pain. Twelve days later, his medical condition worsened as the pain persisted and increased. He also noticed a swelling around the jaw which progressively increased in size to involve the face and neck. He used different analgesics such as paracetamol and ibuprofen during this period without any improvement but rather worsening of his condition leading to progressive difficulty in swallowing, breathing and talking which made him to present at the out patient department of the State hospital.\nAt presentation, he was a toxic looking young man, conscious, alert well oriented in time place and person. He was having difficulty in talking and moderately dyspneic as evidenced by alar flaring. There was marked diffuse facial swelling extending from the temporal region downwards involving the face to the cervical region bilateally. Swelling was warm to touch, tender and firm in consistency. There was pus discharge from the mouth with marked trismus which prevented thorough intraoral examination. Medical history was significant for regular alcohol intake and smoking. An assessment of Ludwig’s angina with impending respiratory obstruction secondary to topical use of chemical substance to treat an odontogenic infection was made.\nThe Patient was hospitalized and commenced on intravenous fluid therapy and intravenous antibiotics (cefuroxime – 1gram daily and metronidazole – 1.5grams daily). Laboratory investigations (packed cell volume, electrolyte and urea, random blood glucose, fasting blood glucose, microbiology culture and sensitivity testing) were ordered for. He was immeditely taken to the theatre and under local anaesthesia and parenteral analgesia, incision and drainage (I&D) of the submandibular, submental, sublingual, infratemporal and cervical spaces were carried out. About 300mls of pus was drained from the extra oral incision and intraoral discharge. Rubber tubing drains were inserted and thick gauze dressing placed. He was continued on intravenous antibiotics and haematinics.\nThe result of the laboratory investigations were as follows – packed cell volume-33%, microbiology culture and sensitivity testing yielded growth of Streptococcus pyogenes, sensitive to Cefuroxime, Refloxacin, Cimofloxacin and Amoxycillin, results of other investigations were within normal limits. He was also commenced on active jaw exercise and hourly warm saline mouth bath. There was regular change of dressing as copious amount of pus continued to be discharged from the inserted drains with a sustained improvement of his general condition and by the third post I&D day, the drains were removed. By the fourth post I&D day, necrosis of the skin in parts of the cervical region were noted which later broke down to form 3 oval shaped defects with necrotic tissue at the base (two on the left and one on the right side of the neck). The two defects on the left were about 4cm and 5cm in their widest diameters respectively while the one on the right was about 5cm in widest diameter. Debridement and daily dressing with dilute eusol was commenced. By the eighth day post I&D, the patient could tolerate oral intake well, intravenous antibiotics were discontinued and replaced with oral antibiotics. By the seventeenth day post I&D, the patient was discharged to the clinic to continue active jaw exercise and daily dressing of the neck wound which was begining to look healthy.\nWhen the wound was covered with healthy granulation tissue and no sign of infection noticed, the defects were closed under local anaesthesia using a local flap for the defect on the right, a local flap for the 5cm wide defect on the left and a full thickness skin graft harvested from the left supraclavicular region to close the second defect on the left.\nFollowing active jaw exercise (with the use of wooden spatula) and resolution of the infection, proper intraoral examination and radiological investigation was done which revealed the source of infection as a non vital lower right canine tooth which was subsequently extracted. The wound eventually healed totally after a couple of dressings.
A 59-year-old male retired carpenter received a kidney transplant in August 2014 for end stage renal disease resulting from autosomal dominant polycystic kidney disease and was on maintenance immunosuppression including prednisolone, tacrolimus and mycophenolate. His post-transplant course was complicated by several episodes of urosepsis and graft dysfunction with a baseline serum creatinine of 200 μmol/L and estimated glomerular filtration rate (eGFR) of 29 ml/min. He had recently been treated for CMV disease and Clostridium difficile colitis in June 2015. He had no occupational exposures of significance, however kept a pet caged cockatiel for many years which involved regular cleaning of bird droppings from its cage.\nHe was admitted to hospital firstly on 3rd August 2015. He reported a one-month history of pruritic and painless rash affecting the lower legs bilaterally which had been refractory to self-administered moisturising lotion. On examination he was found to have symmetrical erythematous patches on both shins. He was afebrile throughout the hospital admission and there was no history of fevers or rigors. Nor was there a history of headache, nausea or change in mental state, or any respiratory symptoms. Inflammatory markers (CRP, ESR) were not elevated. During this admission to hospital he received less than 24 h of intravenous (IV) flucloxacillin and was diagnosed with probable lipodermatosclerosis. He was discharged home on 4th August 2015. A superficial skin swab was taken during this admission from a site of mild skin erosion and culture did not isolate a specific micro-organism. He was discharged home on his usual medications including prednisolone, tacrolimus and mycophenolate.\nHe represented to hospital on 13th August 2015 with worsening skin rash affecting his lower legs bilaterally (Fig. ). He had received oral flucloxacillin through his primary care physician for 3 days leading up to this second hospital admission. He was diagnosed initially with venous insufficiency complicated by overlying cellulitis and was initially recommenced on IV flucloxacillin. An infectious diseases physician did not think bacterial cellulitis was likely, given the atypical skin appearance and the presence of bilateral involvement. A dermatology opinion was sought on 14th of August with the preliminary diagnosis of lichen simplex chronicus and suggestion to exclude malignancy and infection. Given the cellulitis was bilateral, persistent, and an atypical presentation, suspicion for an atypical infection and the possibility of systemic aetiology was raised. Skin punch biopsies were performed on the 14th August. Yeast-like organisms highly suggestive of cryptococci were identified on histology (Fig. ), which on culture were confirmed to be Cryptococcus neoformans. Unfortunately sub specification of the organism was not carried out.\nOnce a diagnosis of cryptococcal cellulitis was made, further investigations were undertaken to determine the extent of cryptococcal disease and determine if systemic infection was present. A computed tomography (CT) scan of the brain was normal. CT of the chest, abdomen and pelvis revealed several non-pathologically enlarged lymph nodes in the mediastinum, retroperitoneum, and bilateral inguinal regions only. Notably there were no discrete pulmonary lesions seen. Lumbar puncture performed on 19th August demonstrated an elevated opening pressure of 28.5 cm of water (reference range up to 20) with white cell count (WCC) 30, Red cell count 1, protein 370 mg/L and glucose 1.5 mmol/L. Unfortunately a serum glucose was not obtained on the same day. Gram staining showed yeast-like organisms and India ink staining of CSF showed organisms resembling Cryptococci. As soon as disseminated cryptococcosis was suspected following initial lumbar puncture, treatment was commenced on the 19th August. The patient was started on renally dose-adjusted IV flucytosine 1500 mg twice daily and IV liposomal amphotericin 200 mg (3 mg/kg) daily after liaison with the nephrology team. His immunosuppression was reduced by withholding mycophenolate on the advice of the transplant physician. Blood and CSF cultures were both found to grow C.neoformans. Both CSF and serum cryptococcal antigen titre was > 1:1024. These findings confirmed the diagnosis of disseminated cryptococcal infection with high burden of disease.\nTwenty-four hours following commencement on antifungal therapy the patient developed a mild headache without any focal neurological signs. He was transferred to the tertiary transplant centre in Queensland for ongoing management by renal transplant and infectious diseases teams.\nA repeat lumbar puncture was performed revealing an opening pressure of 37 cm of water. Ophthalmology review at this time revealed no papilloedema. Further lumbar punctures were continued with repeat lumbar punctures performed on the 24th August, 25th August, 26th August and 2nd September which continued to culture positive for Cryptococci. A final lumbar puncture performed on 29th September showed 8 white cells (100% mononuclear), protein 600 mg, glucose 3.5 mmol/L with a india ink that was positive for Cryptococci however culture was negative.\nDuring hospital treatment for disseminated cryptococcosis our patient developed a concomitant episode of C.difficile colitis prompting treatment with oral metronidazole. As a result his kidney function temporarily reduced from a baseline of 27 ml/min to 15 ml/min, which was thought to be related to reduced hydration. This subsequently resolved with rehydration and resolution of C.difficile infection. His kidney function remained stable thereafter (baseline eGFR 27 ml/min) on the same regimen of amphotericin and flucytosine. His immunosuppression regimen at the time of C.difficile infection included prednisolone and tacrolimus (with mycophenolate being ceased).\nHe remained in hospital, receiving a total of 6 weeks IV liposomal amphotericin and flucytosine. This induction phase with amphotericin and flucytosine was prolonged (longer than the usual 2 weeks) due to persistence of positive CSF cultures and clinician precaution. He was then changed to oral fluconazole as consolidation and maintenance therapy.\nSince this time our patient has seen significant improvement in his lower leg cellulitis (Fig. ). He no longer has the pet cockatiel bird as this was thought to be a potential source of the initial cryptococcal skin infection. He has not had another hospital admission in relation to his skin disease. His current immunosuppression regimen includes prednisolone and tacrolimus and renal allograft function is stable. He remains on oral fluconazole, and is expected to remain on fluconazole lifelong due to an ongoing need for substantial immunosuppression in the setting of his renal transplant.
A 33-year-old African American woman presented to the emergency medical services of our hospital complaining of a chest pain that began suddenly that morning while she was resting in bed. She described the chest pain as sharp and non-radiating in the peristernal area. Deep breaths and movement exacerbated her chest pain. Nitroglycerin given sublingually partially relieved the pain. On initial presentation she also complained of right foot pain progressively worsening over the past five days. She also admitted to bilateral hand pain worsening over the past three days. The right foot pain prompted an emergency room visit one week prior to this admission for which she was treated with naproxen [Naprosyn] and propoxyphene and acetaminophen [Darvocet], which gave her minimal relief of the pain in her right foot.\nHer medical history is significant only for normal childhood illnesses. Her surgical history only includes a cesarean section. Socially she admitted to drinking two bottles of beer on a daily basis but denied tobacco or drug use. Her father died at the age of 46 secondary to a myocardial infarction, and her mother is alive with hypercholesterolemia, diabetes, hypertension and arthritis. She also has a brother with diabetes. She denies food, drug or environmental allergies. Her home medication includes naproxen and propoxyphene and acetaminophen, which were prescribed to her one week prior to this admission.\nOn initial physical examination she was noted as having violaceous purpuric patches and macules that were non-palpable, non-blanchable lesions distributed bilaterally over her hands and right foot. These lesions were consistent with vasculitis or arterial insufficiency. Her toes and midfoot were completely involved in a stocking pattern with sparing at the heel. Her ankle to middle leg exhibited coalescent ecchymoses and purpura in an incompletely circumscribed distribution around the limb. The upper margins demonstrated scattered petechiae extending to just below her knee. Her popliteal, posterior tibial and dorsalis pedis pulses were palpable. Her left second finger also had violaceous, coalescing, purpuric macules and petechiae extending into the dorsal space between her thumb and second finger. All of her involved extremities were extremely painful to touch or motion with non-pitting edema of moderate degree. Photographs of her involved digits were obtained with consent and can be viewed in Figure and Figure .\nAt the time of presentation the working diagnoses included vasculitis secondary to recent NSAID usage or other collagen vascular disorders versus possible cardiac origin causing thromboemboli. She was initially started on a heparin drip to combat the possibility for further thromboemboli, and solumedrol at 1 mg/kg intravenously daily for possible vasculitic etiology. After the initiation of corticosteroids her ESR decreased from > 140 to 56 to 47 by day three. Her admission laboratory values can be viewed in Additional File .\nInitial imaging studies included arterial ultrasound of the right lower extremity due to the color changes in her skin and pain in the extremity even at rest. Arterial doppler ultrasound demonstrated posterior tibial artery and dorsalis pedis artery with biphasic wave forms with systolic flow in normal ranges without ischemia. Radiographs of her bilateral hands and right ankle due to her complaint of joint pain showed soft tissue swelling with no air in the soft tissues or destructive lesions.\nRheumatology ordered the following laboratory studies including antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies (ANCA) with reflex MPO and PR-3, ESR, cryoglobulins, hepatitis panel, rheumatoid factor, anti-cyclic citrullinated peptide antibody (anti-CCP), serum protein electrophoresis (SPEP), dsDNA, HIV, C3, C4, uric acid and thyroid-stimulating hormone (TSH). Results of these studies were within normal limits except for SPEP which revealed a monoglonal gammopathy with elevated quantitative IgE.\nDespite heparin therapy with PTT in therapeutic ranges, on the second hospital day our patient's clinical condition worsened with an increased pain and loss of palpable or doppler right pedal pulse. Her physical examination demonstrated increased non-pitting edema, loss of right light sensation, and worsening of pain. Computed tomography (CT) angiogram of her abdominal aorta with runoff to the lower extremities revealed abrupt occlusion of her right anterior tibial, posterior tibial and peroneal artery within 5 cm of their origin. There was no indication of filling defect suggestive of emboli proximal to the plane of occlusion. Given the progression of her disease over the initial days, other consideration for diagnosis included neutrophilic dermatosis, purpura fulminans with associated disseminated intravascular coagulation (DIC), and antiphospholipid syndrome or other coagulopathies. Additional labarotoy studies ordered were antithrombin III, protein C and protein S, which all showed normal results. Our patient's platelets remained stable at 200 to 380 thousand range for the entirety of her hospitalization.\nVascular surgery had an initial impression that our patient had a cardiac etiology resulting in the vascular changes in her extremities. Trans-thoracic echocardiogram (TTE), and then transesophageal echocardiogram (TEE), were negative for cardiac vegetations or other source for cardiac thromboemboli. After a cardiac source of thromboemboli was ruled out, her solumedrol medication was increased to 1 gm/kg intravenously daily for three days, and then resumed at 120 mg intravenous daily.\nCulture of her urine sample grew out gram-negative rods (Escherichia coli). Blood cultures also grew out gram-negative rods with E. coli as the infectious organism. Due to bacteremia, our patient was started on intravenous antibiotics including rocephin 1 gm daily and ciprofloxacin 750 mg intravenous twice daily. Culture sensitivities returned two days later showed sensitivity to rocephin, thus prompting us to discontinue her ciprofloxacin medication.\nDermatology performed a four mm-punch biopsy from her left thenar area. The results are shown in Figure and Figure . Sections examined in multiple layers show vessels in the upper dermis in which there is destruction of the vessel wall with what appears to be fibrin and large collections of neutrophils. The picture is typical of leukocytoclastic vasculitis.\nOnce the diagnosis of LCV was established, we started our patient on colchicine 0.6 mg by mouth twice daily. A trial of dapsone 50 mg by mouth daily, then 100 mg by mouth twice daily was initiated on day seven, but there was a subsequent decrease in hemoglobin level by > 2 gm/dL from 11.6 to 9.3 gm/dL. As this can be an adverse effect from the dapsone, we decided to discontinue the medication. After her bacteremia was treated with intravenous antibiotics and resolved, Cytoxan (cyclophosphamide) 750 mg/m2 were initiated. Our patient was also started on prophylactic bactrim to prevent Pneumocystis jiroveci. During the course of her treatment and trial of the above pharmaceuticals, she still did not have optimal response as her gangrene continued to progress. As a final therapeutic attempt, she was started on Revatio (sildenafil) 20 mg by mouth three times daily. During this time she was suffering peroneal nerve paralysis with loss of dorsiflexion of foot and sensory losses along with absent pulses and blackening of her toes. She did receive some benefit from multiple therapies with mild regression of involved areas to both hands and leg, but ultimately amputation was required to avert life-threatening septicemia and worsening rhabdomyolysis with peak myoglobin 2294 ng/mL (normal: 25 to 58 ng/mL), CPK 9975 U/L (normal: 26 to192 U/L), BUN 16 mg/mL (normal: 6 to 20 mg/mL), creatinine < 0.5 mg/dL (normal: 0.5 to 0.9 mg/dL) and leukocytes 25.37 K/uL (normal: 4.5 to 11.0 K/uL).\nOur patient underwent amputation of her right lower extremity (below the knee amputation) on hospital day 13. Within two days her leukocytosis improved to normal range. On day 30 she underwent left second and right third finger amputation at the distal interphalangeal joints (DIPs). A final pathology of the amputated leg showed ischemic necrosis and dry gangrene. The middle and smaller blood vessels demonstrated a vasculitis similar in appearance to previous punch biopsy with neutrophilic infiltrate and fibrin deposit. There were no thrombotic or atherosclerotic occlusions, and myositis with no pyomyositis was noted. The fingers demonstrated a late stage vasculitis with thrombotic occlusion and remnants of acute inflammation with necrotic small and middle vessels.\nOur patient was sent for physical rehabilitation for a short time, and then discharged home on a two-week prednisone taper.
We evaluated a 41-year-old male presented in September 2004 with an episode of acute gastrointestinal bleeding, which was associated with non-Hodgkin's lymphoma with a nonspecific subtype. The patient lived in a remote rural area in Northern Brazil and was promptly transferred to a larger center to receive chemotherapy to avoid a surgical procedure.\nHe was treated with three cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), without clinical response. He was then submitted to a surgical procedure and the anatomopathological study of retroperitoneal lymph nodes was consistent with an infiltrative carcinoma. The immunohistochemical analysis revealed chromogranin and synaptophysin expression and also a Ki 67 < 3%. At this moment carcinoid related markers (glucagon, gastrine, prolactin, PTH, 5HIAA, and chromogranin A) were normal. Because of the discordance between the two biopsies regarding the histological diagnoses, a review was performed in both specimens and there were no histological findings suggesting lymphoma and the hypothesis of a low grade neuroendocrine tumor was confirmed. The patient was then treated with octreotide acetate until clinical recovery and then, without any clinical or radiological evidence of disease, treatment was withheld.\nOn December 2010, he presented with an abdominal obstructive syndrome and an exploratory laparotomy was necessary. The anatomopathological diagnosis of mesenteric lymph nodes resected was consistent with areas containing marginal zone B-cell lymphoma/chronic lymphocytic leukemia and neuroendocrine tumor. Bone marrow was not infiltrated and FDG-PET showed exclusively abdominal nodal disease which was compatible with lymphoma involvement. At this time he was treated with 6 cycles of CHOP and octreotide was started again. CT scan obtained at the end of the treatment showed complete response.\nThe patient's family history was revealed to be very unusual. He referred to the fact that his father had a prostate cancer and the majority (if not all) of the 9 father brothers presented lymphoma or leukemia. These data could not be double checked since these individuals have died many years before in very distant places, but the fact that the patient was able to describe a very unique type of cancer in his relatives, many of them with low level of literacy, suggested that the presence of lymphoproliferative disorders is quite common in the paternal side of the family.\nAt clinical examination the patient lacked any cutaneous stigma of neurofibromatosis, but one of the CT scans revealed the presence of various neurofibromas in the thorax and abdomen (). He was tested for NF1 gene mutations and chromosomal abnormalities encompassing part of the long arm of chromosome 17 containing the NF1 gene.\nThe coding region of the NF-1 gene was analyzed by real-time polymerase chain reaction (RT-PCR) and direct sequencing. Multiplex ligation-dependent probe amplification (MLPA) was also performed to detect the number of mutant copies (deletions and duplications).\nThe following alterations were met: duplication of NF1, TRAF4, and MYO1D. Additionally, fluorescence in situ hybridization (FISH) using probes (RP5-1002G3 and RP5-92689) flanking the NF1 gene in 17q11.2 and CEP17 for 17q11.11.1 was performed. There were three signals (RP5-1002G3conRP5-92689) in the interphases analyzed and two signals (RP5-1002G3conRP5-92689) in 93% of cells. Those findings suggest that the patient had a tandem duplication of 17q11.2.\nOn June 2013, the patient was submitted to routine laboratorial tests and a blood workout displayed a lymphocytosis (7500 lymphocytes/dL). The immunophenotype of the lymphocyte population was mostly composed of a clonal mature B lymphocytes positive for CD19, CD5, CD20, and CD23. These findings were consistent with CLL. FISH analysis displayed a del 17p13.1 ().\nThe patient has been in treatment with octreotide acetate, without signals of disease progression. CLL is under clinical surveillance and the patient is out of treatment in the moment.
An eighty-two-year old female presented to the urology outpatient clinic with a short history of fecaluria, pneumaturia, and passage of urine per rectum. She had an indwelling urethral catheter inserted, because of the total urinary incontinence, for a period of over eight years. This catheter was last changed twelve weeks prior to her visit to the urology clinic by her district nurse. The patient had positive MSUs in the year prior to her admission demonstrating mixed growth of enteric organisms, but no prior febrile urinary tract infections were reported.\nShe received radical radiotherapy alone for a muscle-invasive bladder tumor (stage T2b) in 1991 and underwent abdominal exploration for drainage of an appendicular abscess when she was in her early twenties. The patient had a background history of cerebrovascular disease, hypertension and ischaemic heart disease. She was felt to be an unsuitable candidate for radical surgery for her invasive bladder cancer due to these comorbidities. The decision to manage her incontinence with an indwelling urethral catheter was made having discussed available treatment options with the patient. A long-term indwelling catheter was felt to be the most appropriate option for her given her other extensive comorbidities. She did not have urodynamic testing prior to urethral catheterization. The exchanges of the catheter were being carried out every three months in the community and there had been no reported difficulty with her previous catheter changes. The patient was not undergoing routine cystoscopic surveillance of her bladder.\nOn physical examination she was generally unwell with low-grade fever and pallor. Her abdomen was soft, mildly tender with no palpable masses and no signs of peritonitis. Her urethral catheter drained feculent material mixed with urine.\nLaboratory investigations showed a low hemoglobin of 7.8 gm/dL, white cell count of 17 109/L with a C reactive protein of 233. Magnetic resonance images (MRIs) of the abdomen and pelvis were performed urgently and were reviewed by a senior consultant radiologist and his impression was that those images confirmed the presence of the inflated balloon of the indwelling urethral catheter in the lumen of a bowel segment (Figures and ).\nIn enterovesical fistulas treatment is undertaken depending on the aetiology, clinical status and general condition of the patient. Enterovesical fistulae seldom close spontaneously []. Given the nature of the pathology in this case and the presence of a foreign body in the fistulous tract (the urethral catheter), it was felt that the only way forward was to perform an urgent laparotomy and removal of the foreign body with excision of the fistulous tract. The situation was explained to the patient and she was consented for laparotomy, with all the risks and potential complications of surgery fully explained including both urinary and faecal diversion. On laparotomy there were severe adhesions, and the catheter balloon was found to have penetrated through the bladder wall and was lying in a terminal ileal segment adherent to bladder wall. Excision of this segment and urinary diversion by fashioning an ileal loop conduit was performed. A Hartmann's procedure was carried out due to the high clinical suspicion of presence of another separate colovesical fistula. The patient was transferred postoperatively to the intensive care unit and was moved afterward to the urology ward for 10 days. She had a smooth postoperative course with no recorded complications and is currently on regular urology outpatient clinic followup. Histology from the resected segment of terminal ileum demonstrated chronic inflammation only with no evidence of tumor recurrence.
A male, 52-year-old patient visited the Instituto Nacional de Cancerología (INC) in Bogotá, Colombia, because of the development of a mass in the back region. There had been progressive growth and multiple drainage attempts due to a suspected abscess at various referral sites without relief. He was given non-oncological surgical resection in another institution, and his pathology report was compatible with high-grade sarcoma with positive margins. Additionally, he referred to bilateral vision loss that had been evolving for one month. The initial clinical exam revealed a patient with normal vital signs who was disoriented with regard to space and time though awake and had poor communication with the examiner. He presented a hyperpigmented tumor measuring 10 x 8 cm in the back region, along with a scarred postsurgical lesion without inflammatory signs. The biopsy was reviewed by the INC Pathology Department, which indicated a high-grade sarcoma compatible with myxofibrosarcoma.\nHe was examined by the Ophthalmology Department, which considered bilateral retinal detachment probable. An abdominal computed tomography (CT) scan was performed, which showed a diffuse density alteration of the subcutaneous cellular tissue, without evidence of lesions, probably due to edema. A chest computed tomography (CT) scan with contrast showed an undefined heterogeneous mass dependent on the soft tissues in the posterior thorax wall, with infiltration signs in the muscular plane and the density alteration of the subcutaneous cellular tissue and skin (See Figure ). He was examined by clinical oncology, surgical oncology, and radiation oncology services, which indicated he was not a candidate for chemotherapy, surgical treatment, or radiotherapy because of the significant extension of the tumor into the upper and lower back, the inadequate response of these tumors to chemotherapy, and the patient’s poor Zubrod performance score.\nDuring inpatient care, the patient showed substantial clinical worsening characterized by the progressive deterioration of his consciousness state and persistent oral cavity bleeding. Laboratory tests were requested, which showed the prolongation of blood clotting time due to extended partial thromboplastin time (PTT), with the test value for prothrombin time (PT) being 11.9 seconds and that for PTT being 50.1 seconds (normal value until 28 seconds). The mixing study showed PTT fixing with a 50:50 normal plasma sample that lasted for the first and second hour, suggesting intrinsic pathway coagulation factor deficiency. Even though the mixing study was not suggestive of the presence of inhibitors, the medical history of the patient, the presence of an active neoplasm, and the recent beginning of bleeding created a clinical suspicion of acquired hemophilia. For these reasons, a new mixing study, VIII factor assay, and Bethesda assay to measure factor VIII inhibitors were requested. In addition, treatment with prednisone at 1 mg/kg/day and factor VIII at 1700 international unit (IU) every 12 hours was initiated. The results of these assays showed a basal PTT of 67 seconds, which with the 50:50 sample with normal plasma, was reduced to 38,2 seconds and maintained at around normal values two hours afterwards, with a value of 36,3 seconds. Factor VIII was measured at 4% and remained at this same value (4%) 10 minutes after the injection of intravenous factor VIII. The specific factor VIII inhibitor assay reported 1.6 Bethesda units. A diagnosis of acquired hemophilia was made based on the presence of hidden low-titer inhibitors in the mixing study.\nBased on the findings above, the persistence of oral cavity bleeding, and daily transfusion support in the form of two red blood cell units, IV treatment with an activated prothrombin complex like the factor eight (VIII) inhibitor bypassing activity (FEIBA) at a dose of 3500 units/12 h (50 U/kg every 12 hours) was initiated. During the patient’s evolution, hypofibrinogenemia development, the continuous elevation of D-dimer levels, and progressive thrombocytopenia suggested a diagnosis of disseminated intravascular coagulation (DIC). Because of the unresolved bleeding, it was decided to initiate a transfusion of cryoprecipitate and tranexamic acid oral rinse while augmenting the FEIBA dose. The patient developed a low response to the established treatment, along with more profound neurological impairment (central nervous system bleeding was ruled out) and the persistence of red blood cell, platelet, and cryoprecipitate transfusion requirements. Additionally, the patient had severe hyponatremia in the form of an electrolyte imbalance. Electrolyte correction was initiated, and because of the persistence of bleeding, oral cyclophosphamide was added to the immunosuppressive treatment, with the objective of eradicating the inhibitors. Seven days afterward, because of the lack of clinical response to management and the impossibility of providing specific antineoplastic treatment, with the agreement of the patient´s family, transfusion support was suspended, and palliative care was initiated. The patient died one week after.
A 65 year old female patient with a history of rare migraine attacks developed hyperacute severe global headache with accompanying symptoms of confusion and reduced short term memory. The headache was different from her previously known migraine, which had been asymptomatic for the last six years. The symptoms dissolved completely within 90 minutes. 2.5 weeks later she consulted a doctor, and was admitted to the hospital.\nOn neurological examination there were no focal neurological deficits. There were no paresis or numbness in the limbs, and cranial nerve examination and reflexes were normal. Physical examination was also completely normal.\nOn admission an unenhanced Computer tomography (CT) of the head was performed. There were no signs of haemorrhage or other pathology. CT angiography of the intracranial arteries revealed a 2-3 mm aneurysm in the right posterior communicating artery. There was an anatomical variant with fetal origin of the right posterior communicating artery and a hypoplastic P1-segment of the right posterior cerebral artery. This aneurysm was assumed to have caused “warning leak,” indicating an unstable aneurysm in need of treatment.\nThe aneurysm was successfully treated with carotid artery stenting and aneurysm coiling. Postinterventionally, the patient was clinically asymptomatic without any neurological deficits. The patient was treated with Acetylsalicylic acid and Ticagrelor, as Clopidogrel did not show adequate effect by platelet function tests. The patient was discharged to her home with a planned follow-up for Magnetic resonance imaging (MRI) of the brain three and twelve months postinterventionally.\nOne month after EVT, the patient was rehospitalized due to two weeks of right sided headache and a transient episode with flickering in the left visual field. A CT of the head and a CT angiography were performed and revealed small areas of subacute infarction in the right insula, interpreted to be due to a procedure-related thromboembolic event. There was no bleeding, and cerebral arteries were open. Neurological examination was normal. Except for soreness in the neck muscles, the medical examination was normal. The patient's symptoms were thought to be caused by tension headache, and the patient was discharged the day after.\nOne week after hospital dismissal, the patient was again rehospitalized due to persisting headache and a transient episode of anomic aphasia. Additional to a new CT of the head and CT angiography, a CT perfusion of the head and a brain MRI were performed. The examinations revealed no new findings. The patient had a slight persistent aphasia at admission which completely resolved within an hour. Blood samples were normal. The headache was still thought to be caused by tension headache, and the patient was dismissed after four days.\nFive months after EVT, a follow-up brain MRI was performed. On T2-weighted images, new hyperintense white matter signal changes consistent with vasogenic edema in the vascular territory of the right internal carotid artery were seen (). There were approximately 10 lesions located mainly subcortical in the vascular territory of the right internal carotid artery. The lesions had slight mass effect. There was no restricted diffusion. Contrast enhanced MRI was not performed.\nOne year after EVT, a new follow-up MRI was performed, showing only small remaining areas of signal changes (). The patient's neurological examination was still normal, and the headaches decayed about six months after EVT without specific treatment. The clinical and radiological presentation were interpreted to be suggestive of a foreign body reaction after neurointerventional treatment.
A 4 year-old previously healthy girl was transferred to our PICU from an outside medical center ED for workup and management of generalized anasarca, manifesting as diffuse edema, pleural and pericardial effusions, and abdominal ascites.\nIn the four months prior to admission, her mother noted that she had increasing abdominal distension and facial edema, as well as cough, shortness of breath, and orthopnea. Other symptoms included progressive weakness, fatigue, abdominal pain, and a 5–10 lb. weight gain. She had been evaluated by multiple providers in the ED as well as her PCP due to these symptoms; however, no etiology was identified, and she was ultimately diagnosed with a viral syndrome. Due to worsening abdominal pain and increasing work of breathing, her mother brought her back to the outside hospital ED.\nIn the outside hospital ED, she had a Chest X-ray which showed bilateral pleural effusions and a pericardial effusion. Echocardiography and Neck/Chest/Abdomen CT confirmed the presence of large pericardial and pleural effusions and abdominal ascites (Table ). She required transfer to the outside hospital PICU shortly after admission, where she was intubated secondary to increasing shortness of breath. Pericardiocentesis was performed, and 150 mL of fluid was removed. A pericardial drain was placed as well as bilateral chest tubes. Extensive workup at this time was largely unrevealing from an infectious disease, rheumatologic, and oncologic perspective (including lymph node biopsy and evaluation of the pericardial and pleural fluid). MRI, however, showed diffuse edema of the musculature.\nHer respiratory status improved, so she was extubated and transferred to our institution for ongoing evaluation. On examination, she had diffuse non-pitting edema of all four extremities, facial edema and significant abdominal distension as well as a small aphthous ulcer on her tongue and lymphadenopathy in her cervical chain and bilateral axillae. She was noted to have a wide based gait, some difficulty rolling over, and inability to stand without using her hands to help pull herself up. Her significantly increased abdominal girth likely contributed to her inability to perform activities that required core strength. Full muscle testing was unable to be obtained due to cooperation. No muscle or bony tenderness, joint pain or swelling, or rash were appreciated. Vital signs, including heart rate, blood pressure, temperature, and SpO2 were in normal range for age. Laboratory workup is summarized in Table and is notable for normal muscle enzymes. Repeat MRI revealed diffuse edema of subcutaneous tissues and musculature. Muscle biopsy showed perimysial and perivascular lymphocytic inflammation, consistent with inflammatory myositis, and HLA staining showed significant HLA Class I upregulation consistent with JDM. A diagnosis of juvenile dermatomyositis was made based on her imaging and pathology findings. Myositis specific antibody panel was sent, which was notable only for weakly positive Ro60. Notably, at this time, she did not have the classic clinical manifestations of rash nor the typical laboratory values consistent with myositis, such as elevated CK, ALT, LDH, or aldolase. Furthermore, it was difficult at the time of presentation to differentiate weakness from deconditioning and limitation secondary to enlarged body habitus secondary to edema.\nDuring her hospital course, she experienced reaccumulation of her pleural and pericardial effusions after her drains were removed, requiring repeat drain placement. She was initially treated with furosemide, indomethacin, and colchicine for her refractory edema. Once the pathology results returned consistent with JDM, treatment was initiated with pulse dose (30 mg/kg/day) of IVMP. She had no significant improvement after 5 days of pulse dose IVMP, so 2 g/kg IVIG was administered over 2 days (1 g/kg/day × 2) Two days later, she was noted to have marked improvement clinically in terms of improvement in her edema as well as a decrease in her inflammatory markers. She was discharged shortly thereafter with a plan for 30 mg/kg IVMP every week, 2 g/kg IVIG every 3 weeks, 30 mg (1 m/kg) prednisone daily, and 15 mg methotrexate weekly (15 mg/m2).\nApproximately 2 weeks after discharge, this patient was readmitted to our hospital with significant weakness and re-accumulation of pleural and pericardial effusions and abdominal ascites. At this point, she did display the classic Gottron’s papules consistent with JDM. She was treated with 2 g/kg IVIG and 600 mg of rituximab (750 mg/m2), with improvement in her symptoms. She received another dose of rituximab 3 weeks later as an outpatient. It was delayed by one week due to the initial denial from the insurance company, which was approved on appeal. Over the next several months, she continued to have frequent readmissions for weakness, muscle pain, and recurrent effusions, requiring an increase in frequency of IVIG to 2 g/kg every 2 weeks, and escalating steroids to 30 mg (1 mg/kg) prednisone daily and 30 mg/kg IVMP weekly. Her weekly IVMP was continued for four months after discharge. Oral prednisone was tapered over 7 months after discharge. She continued biweekly IVIG for three more months until the fall of 2020 (Mom was anxious to bring the patient to an infusion center due to a local rise in COVID cases, so treatment was interrupted). IVIG was re-initiated in the spring of 2021 secondary to disease flare as evidenced by weakness and elevated inflammatory markers. Steroids were not restarted. Her current medication regimen includes 2 g/kg IVIG every 3 weeks and 15 mg subcutaneous methotrexate weekly. She has received no additional doses rituximab.\nThis patient continues to exhibit core and upper extremity weakness, but she no longer has edema or reaccumulating effusions. Family is non-compliant with physical therapy, which likely contributes to her continued weakness and deconditioning. She has no evidence of calcinosis or lipodystrophy.
A 71-year-old man was referred to our department complaining of dyspnea, hypoventilation and dysphonia, determined by swelling in the neck region and related to a considerable increase in size of the thyroid gland.\nOn clinical examination, the thyroid gland appeared firm during the acts of deglutition. The patient reported that he had a goiter for more than 20 years and he had never undergone any drug therapy.\nAn ultrasound examination dating back to 1997 documented a complete subversion of the echotexture of the whole gland and the presence of a large nodule in the right lobe displaying a complex echotexture. A further ecography in 2000 documented an increase in volume of the thyroid, which was also the cause of the right carotid bulb dislocation.\nLaboratory investigations revealed significantly elevated thyroglobulin values. A preoperative fine needle aspiration was not significant; it consisted of an acellular smear within a background of inflammatory and necrotic cells without any cytologic specification.\nConsequently, we decided not to repeat this procedure because of the worsening of the patient’s dyspnea, for which a surgical management for liberation of the airways was planned.\nA total thyroidectomy was performed. Although the surgical operation was expected to be very complex, it was performed in a completely linear way. The left lobe was easily separable from the surrounding tissue; it appeared to be in the throes of a nodular transformation. The right lobe appeared uniformly in a nodular transformation and it penetrated the upper part of the neck, adhering to the vessels, from which, however, it was easily dissociated. Lymph nodes were not visible. However, surgical times were also lower than expected, particularly for the removal of the right half, which was relatively easy, and the mass appeared well encapsulated and demarcated (Figure ).\nThe specimen was sent to surgical pathology for definitive diagnosis. The tissue samples were fixed in formalin, then routinely processed and embedded in paraffin. The sections were stained with hematoxylin and eosin. Additional 5-μm sections were cut and subjected to immunohistochemical studies using antibodies to the following antigens: CD34, CD31, factor VIII-related antigen, pan-cytokeratin (Pan-CK) and thyroglobulin. Grossly, the right thyroid lobe measured 10 × 8.5 × 6 cm, the left lobe was 5 × 4 × 1.5 cm and the pyramidal lobe was 4 × 2 × 1 cm in size. The right thyroid lobe was totally occupied by a well circumscribed nodule macroscopically confined within the capsule. The nodule measured 9 × 5 cm and, on the cut sections, appeared cystic and hemorrhagic, with large necrotic tissue areas (Figure ). The tumor was extensively sampled. On histological examination, the periphery of the lesion showed epithelioid areas that were made up of large rounded cells of relatively high nuclear grade, with eosinophilic cytoplasm and prominent nucleoli arranged in rudimentary vascular channels. These neoplastic channels were irregular in shape, and they were lined by a single layer of malignant endothelium forming intraluminal papillary projections (Figure ). An extensive central area of necrosis and hemorrhages was a characteristic feature of the lesion. The capsular surface was not involved with the tumor and a rim of residual thyroid tissue was observed in some peripheral areas. Immunohistochemically, neoplastic cells were strongly positive for CD31, CD34, and factor VIII-related antigen, showing evidence of their endothelial differentiation (Figure ). Diagnosis of this condition can be difficult as the histological features may mimic other malignant vascular lesions. In view of the clinical history and morphological and immunohistochemical findings, a diagnosis of primary angiosarcoma of the thyroid gland was made.\nAs regards adjuvant therapy, our patient has not been subjected to any kind of special treatment because, even today, there are no guidelines and there are various schools of thought as to the best treatment options.\nThe prognosis was not favorable. The postoperative course was not simple: it was decided to admit the patient to our intensive coronary unit for precautionary reasons. After 3 days, due to an atelectasis of the left lung, intubation and subsequently tracheostomy were needed. Breathing difficulties continued for a long time (about 60 days after surgery). Our patient was monitored and follow-up was started but it was too short to be meaningful because unfortunately, our patient died about 3 months later as a result of of cardiac and respiratory complications related to his oncologic condition.
A 44-year-old Asian male patient gradually developed a ventral mass on the penis 7 years before presentation. Initially, the lesion was ~1 cm in diameter and occasionally accompanied by paresthesias and bleeding after friction. He had normal urination and did not visit the hospital at first. When an ulcer developed, he applied erythromycin ointment. Sometimes, the focal ulceration\nhealed, but the condition often recurred. The scope of the lesion gradually increased over the period of 7 years. Finally, he came to the dermatology department of our hospital and underwent penile lesion tissue biopsy. The pathological report showed that he had penile intraepithelial neoplasia. Considering the large range of lesions, he came to the urological ward for hospitalization and surgical treatment. The patient had no clear history of exposure to chemical properties such as arsenic. His HIV antibody test was negative. He did not have a history of smoking or alcoholism. The lesion was located on the ventral side of the penis and the total area was ~2.2 cm ×2.0 cm (). The surface of the penile lesion was pale red with a gray edge. It had an irregular shape and clear boundaries, and the lesion slightly protruded out of the surface of the skin. There was no obvious exudate on the ulceration, and there was no obvious redness or swelling of the surrounding skin.\nAfter continuous epidural anesthesia, a skin incision line was marked at a 1.0 cm margin from the edge of the lesion of the penis of the patient. The scrotal flap range that needed to be isolated was also set before resectioning of the lesion. We cut the skin of the lesion vertically along the designed incision margin line, deep to the deep fascia layer, and completely resected the lesion tissue on the surface of the deep fascia (). After careful hemostasis by bipolar coagulation, we designed a 5.0 cm ×3.0 cm scrotal flap based on the resected wound area and dissociated it on the surface of the deep fascia. With a formed pedicled scrotal flap, we stretched it to the ventral side of the penis and covered the surgical area of the penile skin (). Intermittent suturing of the subcutaneous tissue reduced cavity formation and local exudate accumulation (). The wound area was dressed in gauze under pressure, and the penis was wrapped with a self-adhesive bandage under appropriate tension to promote wound healing. No drainage tube was placed in the operational area. The bandage and gauze were removed to expose the wound and keep it dry 3 days after surgery.\nAfter penile lesion excision, the wound was covered by scrotal flaps with blood supply. There was postoperative edema in the penile wound without infection. At follow-up observation half a month after the operation, the wound edge did not heal completely because of tension (). Through care with wound dressing changes, the wound area finally achieved healing (). Pathological images () showed that the lesion tissue was covered with squamous epithelium and showed finger-like protrusion. The basal part of the lesion showed bulbous advancing growth. The epithelium in the lesion area had severe dysplasia, disordered arrangement of polarity, large nucleus, deep staining, and complete basement membrane. No definite infiltration of basal layer tissue was found. The lesion area showed infiltration of polymorphonuclear giant cells and lymphocytes. The pathological diagnosis was penile intraepithelial neoplasia with granulomatous inflammation. No diseased tissue was found at the incision margin. After 6 months of follow-up, the patient had no recurrence of penile intraepithelial neoplasia and had no obvious discomfort with penile erection.
A 55-year-old, Caucasian woman was referred to our tertiary women’s heart center for persistent chest pain, palpitations, and dyspnea. Her medical history included hypertension, dyslipidemia, chronic anxiety, and bilateral non-obstructive carotid atherosclerosis. She had no prior history of diabetes mellitus, tobacco smoking, alcohol or substance abuse, or adverse pregnancy outcomes. Her family history was significant for premature coronary artery disease. Her father had a history of hypertension and had a myocardial infarction (MI) and coronary artery bypass grafting at the age of 39. Her brother had a history of coronary artery disease and also had a MI at the age of 40. Her occupational history indicated that she had been working in the field of psychology and was still an employee in the same job at the time of the hospital visit and follow-up care.\nTable summarizes the general symptoms and characteristic signs of our patient for the onset of CMD and her progression to HFpEF. She had undergone an exercise treadmill test which revealed ischemic ECG changes and dyspnea. Her initial echocardiogram demonstrated a LVEF of 67%, mild diastolic dysfunction, mild left ventricular (LV) hypertrophy, no significant valvular heart disease, and no pulmonary hypertension. Subsequent invasive left heart catheterization was performed and it showed normal epicardial coronary arteries without angiographic evidence of atherosclerotic plaque. She continued to have exertional symptoms and angina-like chest pain and was subsequently referred to our center for further evaluation of suspected INOCA. During her evaluation and treatment she continued to experience stable angina and exertional dyspnea despite initial management with atorvastatin 20 mg daily, lisinopril 20 mg daily, aspirin 81 mg daily, and sublingual nitroglycerin as needed. She had a poor clinical response to sublingual nitroglycerin. Due to her persistent symptoms and abnormal stress testing, she was referred for coronary reactivity testing (CRT) to establish the diagnosis of CMD.\nOur patient underwent invasive CRT, as previously published []. Testing demonstrated normal coronary flow reserve (CFR) in response to intra-coronary adenosine (CFR 3.1; normal ≥ 2.5), abnormal macrovascular endothelial function to intra-coronary acetylcholine (− 6% change in coronary diameter, constriction; normal, dilation), abnormal microvascular endothelial function (coronary blood flow change 48%; normal ≥ 50%), and abnormal non-endothelial function to intra-coronary nitroglycerin (coronary diameter change + 0%; normal dilation) (Table ). She also underwent cardiac magnetic resonance imaging (CMRI) with perfusion imaging at rest and with adenosine stress (140 μg/kg per minute) which showed circumferential subendocardial perfusion defect at stress, normal LV end-diastolic volume indexed to body surface area (EDVi) of 56.4 mL/m2, LV mass index 42.3 grams/m2, and no LV hypertrophy (septum 7.2 mm and lateral wall 6.0 mm). The myocardial perfusion reserve index (MPRI) was 1.8 which was considered borderline abnormal [] (Table ). There was no evidence of myocardial scar.\nThe diagnosis of CMD was established by the coronary endothelial dysfunction observed with invasive CRT, and carvedilol and eplerenone 25 mg daily were added to her regimen. She was followed regularly in clinic with good control of her blood pressure and serum lipid levels. She reported improvement of her angina and dyspnea along with reduction in the duration and frequency of these episodes.\nTen years after her initial diagnosis of CMD, our patient was hospitalized due to symptoms of dyspnea. She was found to have elevated brain natriuretic peptide (BNP) levels of 406 pg/mL and normal LVEF. She had a computed tomography (CT) angiogram of her chest to evaluate for pulmonary embolism, which was negative but revealed bilateral pulmonary edema. She was treated with intravenously administered furosemide for pulmonary edema and diagnosed as having HFpEF. Subsequently, she was discharged with instructions to increase her eplerenone.\nShe continued to experience worsening dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. A repeat echocardiogram demonstrated normal LV systolic function with an LVEF of 64%, and diastolic dysfunction as evidenced by decreased lateral E′ velocity (4.2 cm/s, indicating impaired myocardial relaxation) and elevated E/E′ ratio 12.9 (suggestive of increased LV filling pressure). She underwent coronary CT angiography which showed absence of coronary atherosclerotic plaque and a coronary calcium score of 0. She was diagnosed as having HFpEF based on clinical symptoms, preserved ejection fraction of 64%, elevated BNP, and evidence of diastolic dysfunction.\nAs part of the WISE – Coronary Vascular Dysfunction (WISE-CVD) Continuation Study (NCT00832702), she underwent a repeat rest-stress CMRI to assess myocardial structure, function, perfusion, and scar, and 13C magnetic resonance (CMR) spectroscopy. Compared to her prior CMRI 7 years ago, she had an increase in LV wall thickness in both the septum and lateral wall (Table ). On CMR spectroscopy, the myocardial triglyceride content was elevated (0.83%) compared to normal control women (mean 0.43%), suggesting myocardial steatosis which is consistent with an ischemia-induced metabolic shift and HFpEF phenotype []. Adenosine stress first pass-perfusion CMRI again showed circumferential subendocardial hypoperfusion (Fig. ) and her MPRI worsened from 1.8 to 1.1, consistent with severe CMD []. There was no evidence of scar on late gadolinium enhancement imaging.
This is a case of a 25 years old Malay girl with learning disability and no significant past medical history, who started noticing a sacral mass since August 2015. The mass was painless and gradually increasing in size. The family members of this patient brought her to a traditional healer. They did not seek any medical treatment until late 2017. By this time, the mass over the sacrum was extremely large. Family members claimed the mass was preventing the patient from lying down flat supine. The patient was also unable to ambulate for the past 2 years. Hence, she was bedbound most of the time. It was difficult for her to sit on the wheelchair. She also felt tired to move because the mass was quite heavy. The family members claimed when the patient was lying down flat, she had to flex her hips and knees to achieve a more comfortable position. In addition, she often slept either in prone position or in supine with multiple pillows below her body. The mother also claimed over the last 2 months, the patient’s body had been getting thinner despite her physical weight was increasing due to the increase in size of the sacral mass. The patient had been passing stool and urine in pampers. There was no past medical history and no family history of cancer. Socially, the patient lived with her mother and siblings. The mother was the main care taker. Her father passed away 10 years ago because of heart attack. The patient previously attended a special needs school, but she stopped going to school since 2015 after developing the sacral mass.\nThis patient was managed in the Southern Region referral centre for Orthopaedic Oncology in Malaysia. On clinical examination in the Orthopaedic Oncology ward, the patient appeared cachexic, she had slightly pale conjunctiva, but she was not dysmorphic. Vital signs were Blood Pressure 142/90, Pulse Rate 98 beats per minute and Temperature 37 degrees Celsius. There was a large mass 40 cm × 30 cm × 20 cm over the sacrum. The mass was firm to hard in consistency and involved both buttocks and the gluteal fold (Fig. ). Dilated veins were noted under the skin overlying the sacral mass. Neurological exam of bilateral lower limb was normal. However, there was generalized wasting of all muscles over the bilateral lower limb. Anal tone was intact.\nLaboratory investigations taken were unremarkable. Computed Tomography of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles (Figs. and ). Superior margin of the sacral bone involvement was up to S2. The mass was predominantly of fluid density with internal enhancing septation and calcifications which suggested primary chordoma more likely (Figs. and ). Magnetic Resonance Imaging done showed similar findings. Skeletal Survey Radiograph did not show any distant metastasis. A Trucut biopsy of the mass was done. Histopathological analysis showed tumour cells with “physaliphorous cells” positive for pancytokeratin, EMA, Vimentin and S-100 immunohistochemistry stainings with minimal mitotic figures and mild nuclear pleomorphism (Fig. ). Brachyury immunohistochemistry staining was not available in our centre. However, the clinical history, morphology of tumour on microscopy and immunohistochemistry staining available were consistent sacral chordoma.\nThe diagnosis of Sacral Chordoma was confirmed. Multidisciplinary team discussion done among Orthopaedic Oncology, General Surgery, Obstetrics and Gynaecology, Blood Bank, Anaesthetic and Plastic Surgery teams. A family conference was done. The family’s aim was for removal of the sacral mass to allow the patient lie supine on bed and sit on the wheelchair.\nSubsequently, the patient undergone Wide Resection and En Bloc Sacrectomy. The Posterior-Only Approach was used with a “Mercedes Star” 3 limbed incision. Duration of surgery was 8 h. The patient was supported with blood products transfusion during surgery. Intraoperatively, the sacral tumour had eroded the sacral bone from S2 to S5 (Figs. , and ). Sacrectomy was done at the level of S2. Sacral nerve roots S2-S5 were all infiltrated by the mass and therefore were unable to be preserved. The mass and surrounding gluteal muscles invaded by the tumour were also all resected. All resection margins were less than 1 mm from the tumour. Primary closure was done without any distant or local flap as per consultation with Plastic Surgery team. The tumour weight was 25 kg (Figs. , and ). Post operatively, the patient was monitored in Intensive Care Unit for 3 days. The patient developed neurogenic bowel and bladder post sacrectomy requiring enema and long-term urinary catheter. In addition, the post-operative course was complicated by wound breakdown and surgical site infection requiring wound debridement. Dressing was done as per local protocol until wound bed granulating well. Split Skin Graft was done about 3 months post wide resection once the tissue culture results were free of significant infection.\nThe patient also required extensive rehabilitation for transfer, ambulation and bowel and bladder care. Rehabilitation was difficult because the patient had learning disability and she had been habitually keeping her hips and knees flexed because of the sacral tumour for the past 2 years. During the last review 5 months post operatively, patient was able to sit on the wheelchair comfortably. The surgical wound was healing well with good uptake of the Split Skin Graft (Fig. ).
A 51-year-old woman was seen in consultation for right arm discomfort and edema (Fig. ). She had a past medical history of multiple bronchiectasis infections in context of congenital ectodermal dysplasia. Recurrent sepsis led to acute kidney injuries and eventually to anuric ESRD. She required hemodialysis 3 times a week, currently through using a right radiocephalic arteriovenous (AV) fistula, for almost 25 years. A renal transplant had failed immediately, 15 years prior. She had a right brachiocephalic vein stenosis, previously stented, and a suspicion of breast calciphylaxis diagnosed 3 years earlier. Her weight was 36 kg (BMI of 12) and she had negligible subcutaneous fat.\nA right upper extremity ultrasound revealed a segmental nonocclusive thrombus in the lower part of the right internal jugular vein (IJV). She then had an angiogram and was diagnosed with a severe brachiocephalic vein in-stent stenosis and a moderate stenosis along the juxta-anastomotic cephalic vein fistula. Balloon dilatations of both stenosis were done but the intervention on the stent was complicated by a small focal contained rupture, which was again balloon dilated to ensure hemostasis. Soon after the intervention, the patient was seen at the thrombosis clinic. She reported improvement of her right arm symptoms and the initially described right nonocclusive IJV thrombosis was thought to be chronic. Surveillance by repeat ultrasound 1 week later without anticoagulation was chosen as initial management.\nThe next ultrasound reported interval progression of the known thrombus in the IJV (now occlusive) and nonocclusive extension into the subclavian vein. The stent was still patent. The patient was reporting worsening pain and swelling over the right side of her neck and her right arm for the last week. As such we diagnosed an acute right upper extremity deep vein thrombosis, most likely provoked by the initial flow disturbance caused by the in-stent stenosis and thereafter by the angiographic intervention.\nShe was treated as an outpatient with apixaban 2.5 mg twice daily for 6 weeks. Apixaban was taken at 10 am and 10 pm, while dialysis treatments were scheduled at 6 pm. At her 1 week follow up visit, the pain and swelling were improved. Over 4 weeks, apixaban anti-Xa levels were measured on dialysis days 8 h (i.e. 6 pm, before dialysis) and 12 h after the morning dose (i.e. trough levels, obtained at 10 pm, after dialysis and before the next dose). Anti-Xa were obtained using a CS-2000i (Siemens) analyzer with Innovance heparin reagent (Siemens) and calibrated by a STA®-Apixaban Calibrator kit (Diagnostica Stago, working range: 20–500 ng/mL). Surveillance ultrasound done 1 and 5 weeks after treatment initiation revealed stability of the upper extremity deep vein thrombosis and patency of the stent.\nOver 4 weeks, the anti-Xa trough levels ranged from 58 to 84 ng/mL, while levels obtained before dialysis, i.e. 8 h after the morning dose, were 92–135 ng/mL. Monitoring was not continued up to the end of the treatment considering there was no trend of anti-Xa levels to increase overtime. There was no concomitant medication with significant apixaban interactions and liver function was normal. These range of anti-Xa levels are similar to what would be expected as a trough level in patients with preserved renal function. There were no adverse events in the 3 months after anticoagulation initiation.
The present case report is about a 61-year-old woman who reported to us with a growth in the floor of mouth that gradually increased in size since last 3 years, without pain. On examination, a 3 cm × 2.5 cm ovoid swelling was palpated lingual to the edentulous alveolar ridge, extending from midline (lingual frenum) to the right premolars []. It was a firm, non-tender, non-compressible, non-reducible, did not move with deglutition and showed no change in size with meals. No pulsations were felt over the mass and no vascular channels seen. The swelling was not fixed to underlying tissues and salivary secretions were normal. Some cervical lymph nodes were palpable but not tender. There was no neural involvement or sudden increase in size. Complete hemogram, serum electrolyte levels, kidney function test and liver function test had all values within the normal range, but for a raised erythrocyte sedimentation rate (50 mm/h). Fine needle aspiration cytology (FNAC) of the lesion suggested PA. Node Ib was palpable, but non-reactive on FNAC. Magnetic resonance imaging revealed a 20 mm × 18 mm × 15 mm well defined well-marginated lesion in right sublingual space in the region of anterior aspect of sublingual gland []. It indented the inferior surface of tongue and right genioglossus muscle and did not cross midline. Multiple mildly enlarged bilateral level Ib, II and III lymph nodes were noted. A provisional diagnosis of PA of sublingual gland was made and local excision of the lesion along with the gland was planned under L.A. Incision was placed from midline extending posteriorly near the alveolar ridge in order to protect the Wharton's duct. With careful dissection, lingual nerve was identified and protected. On surgical exploration, it was found that the mass involved the deep part of submandibular gland and duct with the sublingual gland being compressed by the mass. It was difficult to delineate the exact origin of the pathology (sublingual or submandibular gland); hence it was decided to sacrifice the sublingual gland, deep part of submandibular gland and its duct on the affected side []. Excision was done with a cuff of healthy overlying mucosa. Neck dissection was not indicated, hence not done. Initial histopathological examination, revealed a solid tumor tissue composed of myoepithelial and ductal cells presenting as double layered duct like structures with hyalinization of stroma. The epithelial component showed diversity in the form of sheets, nests and cords with different patterns of ductal arrangements and large areas of secretory material in the intervening areas, suggestive of cellular PA []. However since most of the tumors of submandibular and sublingual gland are malignant, a second examination of the specimen was undertaken. It showed that the entire tumor mass was covered by dense band of fibrous connective tissue. Tumor cells were arranged in a background of predominantly mucous acini, as small tubules lined by cuboidal to oval cells containing prominent nuclei and eosinophilic cytoplasm and surrounded by clear cells and increased hyalinization []. On the basis of this dual cell population, a provisional diagnosis of EMC was suggested. Some focal areas were also observed with cells arranged in a cribriform pattern with pseudocystic spaces that pointed toward Ad CC. Immunohistochemistry showed p-63 reactivity in outer clear cells, S-100 and smooth muscle actin were focally positive and C-kit for Ad CC was negative. Hence, a diagnosis of hybrid carcinoma was ruled out and a final diagnosis of epi-myoepithelial carcinoma was made. There is a chance that being a long-standing tumor; initial PA may have turned into EMC. Patient denied adjuvant Radiotherapy; is being followed-up since last 1 year and has no fresh complains or signs of recurrence until now [].
The patient was a 61-year-old woman, gravida 0, para 0. She was referred to the Hiroshima University Hospital on October 19, 2011, with atypical genital bleeding in the last 4 months. She had been diagnosed in our hospital to have uterus didelphys with double vagina and uterus at the time of examination for infertility at the age of 29. Her past history revealed previous surgery for hip osteoarthritis at age of 20. Her family history was unremarkable. The patient was found to have normal vulva. No renal or any other abnormalities were present. Several pelvic examinations in our hospital showed cervical tumor in the uterus didelphys, with a complete vaginal septum extending to the introitus. However, the uterus didelphys had not been observed by the referring hospital, but initially at our hospital. Because the patient complained of pain during pelvic examination, biopsy was performed under intravenous anesthesia, and the initial pathological result of a cervical tumor in one cervix was CIN 3. Furthermore, pelvic examination, hysteroscopy, and biopsy were performed under intravenous anesthesia in the operating room. At that time, the gynecologic oncologist observed the uterus didelphys with vaginal septum for the first time. There were easy bleeding tumors on each cervix at Cusco’s examination. Macroscopic findings, hysteroscopic findings, and the vaginal septum at BT are shown in . The tumor at the left cervix was circumferential, whereas the tumor at the right cervix had a left side deviation (septum side) at the hysteroscopy. Histopathological findings of the bilateral cervical biopsy specimens indicated non-keratinizing squamous cell carcinoma. Human papillomavirus (HPV) infection was not investigated.\nInitially, in our department, sounding of the right uterus was impossible, while the sounding length of the left uterus was only 2 cm. The tumor on the left side was larger than that on the right side, and pelvic examination revealed that the left side tumor had invaded the left parametrium. No invasion was observed in either of the vaginal walls.\nPositron emission tomography-computed tomography (PET-CT) of the head to the pelvis, CT of the chest to the pelvis, and magnetic resonance imaging (MRI) of the pelvis were performed. These showed a tumor with a maximum diameter of 43 mm (more to the left side), spread across the bilateral uterine cervices, and without pelvic and paraaortic lymph node enlargement. According to the Fédération Internationale de Gynécologie et d’ Obstétrique (FIGO) classification [], the tumor was stage IIB. The serum level of the tumor marker, squamous cell carcinoma (SCC) antigen was elevated to 7.7 ng/ml (normal range, ≤ 1.5 ng/ml). The serum level of the tumor marker, carcinoembryonic antigen (CEA), and blood counts were within the normal ranges. The pre-treatment MRI images of the tumor in bilateral uteri are shown in . The tumor on the right side of the uterus was localized in the cervix. On the other hand, the tumor on the left side showed extrauterine invasion (). shows the MRI before and during treatment, from bilateral uterine bodies to fundus and vagina.\nIn addition to the carcinoma of the uterus and vaginal didelphys, based on the pelvic examination and imaging diagnosis, the tumor invasion range in the patient was considered to be wide (stage IIB, with left parametrial involvement, tumor size of 43 mm). This finding was judged to be an indication for radiotherapy rather than surgery. The type of uterine didelphys in the patient according to Jarcho classification [] was categorized as class 1. Uterus didelphys bicollis (septate vagina) according to the American Society of Reproductive Medicine classification [] was class III. Radiotherapy (RT) was administered via a combination of external beam radiotherapy (EBRT) to the pelvic cavity and intracavitary irradiation. EBRT was given to the whole pelvis using four-field box technique with a 10-MV linear accelerator unit. The daily fraction size was 2.0 Gy, with 5 fractions weekly. Details of the radiation field were reported previously []. A central shield was used after 40 Gy, with external whole pelvic irradiation. From November 22 to December 29, 2011, the total dose administered was 50 Gy (25 fractions/38 days). High-dose-rate intracavitary BT (HDR-ICBT) was given with 192Ir micro-Selectron (Nucletron, The Netherlands). The dose at point A was 6 Gy per fraction, 1 fraction per week, and the number of fractions was 4, for a total dose of 24 Gy on December 20 and 27, 2011 and January 4 and 10, 2012. Total treatment time of RT was 50 days. With the cooperation of the gynecological oncologists, ultrasound guidance was used, and it was confirmed that the tip of the tandem reached the fundus of the uterus. Because each vagina was very narrow and the patient complained of pain at the time of application, all 4 applications were performed under intravenous anesthesia. Oxygen was administered at 3 liters per minute with an oxygen mask. At the time of application, vaginal packing with gauzes containing radio-opaque fiber were used to show the state and thickness of the rectal and bladder sides packing. Tandem and ovoid applicators were used for each application. The ovoid size was 30 × 20 × 14 mm in diameter. At the first treatment, only the left side of the uterus was treated with tandem and ovoid applicators. Because the vagina was very narrow, we could not interpose the flange with the two ovoid applicators (these two applicators deviated to the foot side from the flange). The sounding length to the fundus of the left uterus was only 3.5 cm. At the second to third treatments, a tandem was inserted to the left uterus, whereas two ovoid applicators were placed at the right and left vaginal fornices. By the second time, tandem insertions into the uterine cavities on both sides were attempted. The sounding length of the right uterus was 4 cm. However, when a tandem was first inserted on the right side, the insertion on the left side became insufficient because of the narrowing between the bilateral cervical canals. Therefore, a tandem was inserted only on the left side containing the large tumor. We were worried about the insufficient dose to the whole uterus on the right. Therefore, at the last treatment, a tandem and a right-side ovoid were inserted to each of the right uterus and the right vagina, respectively, and a left ovoid was inserted to the left vagina. Dose distributions of each ICBT are shown []. Although it is an approximation, most of the tumor volumes before treatment with each ICBT (red dashed lines drawn by referring to the pre-treatment MRI image) are surrounded by 6 Gy isodose curves. In addition, the tumor volume just after the second ICBT (yellow dashed line drawn by referring to MRI just after the second ICBT) was completely enclosed within the 6 Gy isodose curve.\nThe distances between the bilateral ovoid sources at each application were 1.2, 3.5, 3.6, and 3.2 (mean, 2.9) cm, respectively. We regarded the thicknesses of X-ray gauze packing as the distance between ovoid and vaginal posterior wall for each ICBT. Those measurements were 0, 4.5, 2.6, and 0 (mean, 1.8) mm. Orthogonal X-rays were used for the calculation of these distances.\nThe rectal dose was evaluated using a rectal dosimeter, and the reference dose point as well as the bladder dose were defined according to the recommendations of the ICRU 38 report []. There were five measurement points based on the rectal dosimeter, and the maximum one was evaluated. The mean doses at point A, at the rectum by the ICRU 38, at the rectum by calculation, at the rectum by dosimeter measurement, and at the bladder by the ICRU 38 per fraction were 6.1 Gy, 7.7 Gy, 5.2 Gy, 4.2 Gy, and 7.8 Gy, respectively. The total doses of these five points were 72.7, 107.9, 74.1, 64.0, and 107.6 GyEQD2, respectively. The patient received six courses of concurrent chemotherapy, with 30 mg/m2 of weekly cisplatin (CDDP). No acute adverse events such as diarrhea occurred in this patient.\nThe primary tumor response was the complete response (CR) by pelvic examination just after CCRT; and by pelvic images on MRI and CT at 4 months after CCRT (). The serum SCC level returned to within normal limits, with a value of 0.8 ng/ml.\nFour years and three months after CCRT, the patient developed a late rectal complication, grade 1 radiation proctitis, which was graded according to the Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer late radiation morbidity scoring criteria []. The patient has been followed-up without treatment because she has no anemia. The patient was alive and well with no evidence of recurrence or any other late complications 6 years and 8 months after the start of CCRT.
A female patient aged 45 years with no known comorbidities came to our department at the university hospital Sahloul Sousse with the chief complaint of a right-sided swelling in the mandible. This swelling has caused slight asymmetry of the face since 6 months, which gradually enlarged up to the present size ().\nThe patient gave a history of generalized weakness, lethargy, and weight loss noticed since past few months. Her family history and past medical history were nonsignificant.\nIntraoral examination revealed a 4.0 × 4.0 cm bulbous mass hard to palpation arising from the mandible and extending from the distal aspect of the lower right first premolar (44) to the second right molar (47) (). She had no associated bleeding or superficial ulceration of the mass. The patient denied pain but mentioned difficulty in eating. Positive response to sensitivity testing was found in the lower right premolars and molars. Neither mobility nor teeth dislocation were noticed.\nRadiographic examination with orthopantamogram showed unilocular radiolucency close to the mandibular first right molar's roots, without involving the mandibular canal. Roots erosion of the relative tooth was noted ().\nA computed tomography (CT) scan showed multiloculated ground-glass ossification of the lesion ().\nAfter the first molar extraction (46) and the surgical excision of the lesion, the histological report was akin to the giant cell tumor ().\nSo, serum parathormone level was advised to rule out metabolic bone disease. Laboratory investigations were done: serum PTH level was slightly high, and serum calcium and phosphorus levels were normal ().\nThe lesion was identified as a brown tumor of hyperparathyroidism, and the patient was referred to the endocrinology department.\nKidney function blood tests were done. Serum creatinine and blood urea nitrogen were normal. Chronic renal failure could not be considered the cause of hyperparathyroidism. However, we noted severe vitamin D deficiency (). The initial impression was of secondary hyperparathyroidism due to this vitamin D deficiency.\nIt was decided that vitamin D supplementation was the best therapeutic option. The patient was started on vitamin D 100,000 IU per fortnight for 6 weeks which is the equivalent of half an ampoule of 200,000 IU/1 ml. Thereafter, the patient was kept on 200,000 IU every 6 months. Her PTH levels decreased after 6 months on vitamin D therapy, and her calcium and phosphorus levels remained in the standards.\nThe patient underwent ultrasound scan of the neck, which showed a left lower lobe parathyroid solid nodule. The lesion had irregular hypoechoic component and was suggestive of parathyroid adenoma. Parathyroid technetium scintiscan (99mTc Sestamibi; Technetium-99 MIBI; methoxy-isobutyl-isonitrile) was requisite and revealed left lower parathyroid adenoma (). Skeleton exploration was done by technetium 99 scintigraphy and did not reveal any abnormally high uptake.\nAccordingly, we retained the diagnosis of primary hyperparathyroidism masked by vitamin D deficiency and caused by parathyroid adenoma.\nThere was a normalization of calcium, PTH, and vitamin D serum levels. The bone mineral density test was not lower than normal which excludes osteopenia and osteoporosis. So, surgical treatment of the adenoma was not indicated. The patient was compliant and presented a favorable evolution ().
A 57-year-old male patient with no specific medical history was admitted to University Hospital for paraparesis and sensory disturbances in both lower extremities, caused by a fall 3 months prior to admission. He was diagnosed with CES, due to a L3 burst facture and spinal canal compression. Thus, posterior lumbar interbody fusion was performed (). After surgery, paraparesis improved progressively and no specific symptoms were reported, except neuropathic pain. The patient was transferred to a rehabilitation hospital for conservative treatment of the neurogenic pain and gait training; no specific complication was found during treatment.\nThe patient was transferred to our emergency department with fever and general weakness for 7 days. A complete blood count indicated leukocytosis, and Klebsiella pneumoniae was isolated from blood and urine culture tests. By abdominal ultrasonography, edema was observed in the bilateral renal parenchyma with hyperechogenicity, indicating renal failure, caused by acute pyelonephritis. No local lesion in the renal parenchyma, decreased blood perfusion, or extension of the renal pelvis or renal calyx was observed. After diagnosing sepsis, caused by acute pyelonephritis, meropenem was administered intravenously. After antibiotic treatment for 13 days, vital signs were stable and laboratory findings returned to normal. Antibiotics were stopped on the 15th day.\nThe patient was transferred to the rehabilitation medicine department for comprehensive management of his CES on the 16th day of hospitalization. In the Medical Research Council Manual Muscle Test, muscular weakness was observed in both lower limbs, with hip flexor 4/4 (right/left), knee extensor 3/3, ankle dorsiflexor 3/3, hallucis extensor 3/3, and ankle plantar flexor 2/2 grades. The patient complained of tingling sensations, allodynia, hyperalgesia, and decreased sensations below the third lumbar segment.\nSitting and standing up could be performed independently according to the functional evaluation, but moderate assistance was necessary for balanced-level walking and the Modified Barthel Index was 76 points. The deep tendon reflex was decreased in both lower limbs, though no pathological reflex was observed. Anal sphincter tone and the bulbocavernosus reflex were decreased. An electrodiagnostic study revealed normal sensory nerve conduction in both lower limbs, but the conduction velocity for bilateral peroneal and tibial nerves was slow and the amplitude of the compound muscle action potential was decreased. The H-reflex showed delayed latency in the bilateral tibial nerves. Somatosensory evoked potential stimulating of the bilateral tibial nerves and the bulbocavernosus reflex, stimulating the pudendal nerves, revealed delayed latencies. In needle electromyography, increased insertional activity and abnormal spontaneous activity were observed in muscles innervated from L2 to S2. From these findings, bilateral lumbosacral polyradiculopathy, accompanied by a sacral reflex arc lesion, was diagnosed, and was clinically compatible with CES.\nAt the time of transfer to our department, the patient was voided with an indwelling catheter. The amount of self-voiding after removing the indwelling catheter was 100-200 mL and the residual urine volume was measured to be about 200-300 mL. Voiding sense and desire were normal, and no incontinence, feeling of residual urine or hesitancy was reported. However, timed intermittent catheterization was used for removal of residual urine after self-voiding.\nThe patient had sufficient improvements with walking independently on an even surface level through comprehensive rehabilitative managements, including drug therapy (tramadol 150 mg, gabapentin 900 mg) for neuropathic pains, muscle strengthening exercises, functional electrical stimulation for bilateral lower limbs, and gait training. On the 13th day after transfer, the patient was presented with chills and fever (38.5℃). In a complete blood count, leukocytes revealed 26,110/µL, hemoglobin was 9.3 g/dL, and platelets were 168,000/µL. Creactive protein and the erythrocyte sedimentation rate increased to 34.32 mg/L and 120 mm/h, respectively. In serum biochemical tests, the blood urea nitrogen (BUN)/creatinine ratio increased slightly, to 30.9/1.6 mg/dL, and no other specific finding was observed. A routine urinalysis showed a specific gravity of 1.016, pH 6.0, albumin (2+), glucose (3+), ketones (-), hemoglobin (1+), leukocyte (3+), nitrate (-), and red blood cell 1-4/high power field (HPF), and a white blood cell in excess of 5/HPF by high-resolution microscopy. After diagnosis of recurrent acute pyelonephritis, tazobactam, an empirical antibiotic drug, was administered intravenously. However, Klebsiella pneumoniae was isolated from blood and urine cultures, so the antibiotic was changed to meropenem.\nAfter 3 days, the patient suddenly complained of pain and tenderness on the left flank during voiding and a 10×20 cm-sized mass was palpated in the left abdomen. Vital signs at that time showed a blood pressure of 100/60 mmHg, a heart rate of 115 beats/min, a respiratory rate of 20 breaths/min, and an axillary temperature of 36.5℃. In a complete blood count, leukocytes were 28,900/µL, hemoglobin was 7.1 g/dL, and platelets were 158,000/µL, indicating a sharp decrease in hemoglobin; thus, an acute hemorrhage was suspected. In a coagulation profile test, the prothrombin time was 13.7 seconds, the international normalized ratio was 1.21, and the activated partial thromboplastin time was 35.7 seconds, indicating normal findings. In a serum biochemical test, the BUN/creatinine ratio had increased, to 43.1/3.5 mg/dL, but no other specific finding was observed. In contrast-enhanced abdominal computed tomography (CT), pyelonephritis with multifocal low-density lesions was observed in the left kidney and a perirenal hemorrhage of low density was found in the perinephric space. From the arterial to the delayed phase, active extravasation of contrast media was observed near the perinephric space and was thought to be a hemorrhage in a capsular artery outside the renal capsule.\nThe mass palpated in the left abdomen was found to be a retroperitoneal hematoma with low density in the left iliac fossa (). Systolic blood pressure had decreased by 90 mmHg with declined hemoglobin, thus, emergent transfusion and angiography were performed. No tumor or vascular malformation was found, but coil embolization was carried out due to suspicion of progressive hemorrhage in the left capsular artery (). After transferring the patient to the intensive care unit, conservative treatment for acute renal failure with acute pyelonephritis was performed. No more active bleeding was observed in a follow-up abdominal CT, and the embolized capsular artery was found around the anterior cortex of the left kidney, at the site of the acute pyelonephritis ().\nAfter coil embolization, the hemorrhage stopped, so, nephrectomy was not being considered. However, surgical hematoma removal was conducted, because of persistent abdominal pains. In voiding cystourethrography, the vesicoureteral reflux was not observed, and no voiding images were obtained because the patient failed to void after filling 450 mL of contrast medium even though he had voiding desire. In an urodynamic study, the patient had strong voiding desire when infused with 500 mL of saline, but failed to void due to detrusor areflexia.\nThe patient was administered oral medications, including an alpha blocker and a choline agonist, but intermittent self-catheterization was carried out five times per day due to urinary retention in excess of 500 mL. No additional UTIs or other complications occurred, so he was transferred to a rehabilitation hospital.
An 18-year-old male presented to our institution in 2014 with headaches and vomiting for two weeks. A magnetic resonance image (MRI) of his brain showed a complex extra-axial dumbbell-shaped lesion with the epicenter in the left Meckel’s cave, extending anteriorly to the cavernous sinus and the cerebellopontine (CP) angle posteriorly with mass effect over the brainstem. He underwent a left retromastoid craniectomy and decompression of left CP angle lesion elsewhere. The histopathology was suggestive of malignant melanoma, and he was referred to our institution for further management.\nHis general physical examination revealed a deep gray-blue nevus over the left upper eyelid, extending to the frontal and temporal region. He had dysarthria, left upper motor neuron facial nerve palsy, and right hemiparesis with Grade 4 power in his right upper and lower limbs. The biopsy of the nevus over his left eyelid was reported as superficial, and the deep dermal dendritic melanocytosis with histological features was suggestive of a blue nevus.\nThe MRI of his brain showed a large contrast-enhanced extra-axial mass with solid and cystic components measuring 4 cm x 2 cm x 3 cm in the left cavernous sinus extending through the Meckel’s cave into the posterior fossa (Figure ). A whole-body positron emission tomography-computed tomography (PET-CT) scan confirmed no extracranial disease. He underwent a left temporal craniotomy and zygomatic osteotomy, and we took an interdural middle cranial fossa approach for the radical excision of the tumor.\nPerioperatively, we noted the blue nevus on the left side of the forehead in the ophthalmic distribution of the trigeminal nerve. The pigmentation extended into the subcutaneous tissue and galea. The diploe of the temporal bone was also pigmented. The entire temporal dural convexity was pigmented completely black as were the dural root sleeves of the trigeminal nerve and the lateral and medial walls of the cavernous sinus. The tumor was localized in the cavernous sinus and had a well-defined capsule surrounding the divisions of the fifth cranial nerve. It extended into the posterior fossa through Meckel’s cave. The tumor was completely removed via the cavernous sinus through an interdural approach.\nThe surgical specimen revealed a tumor composed of sheets of moderately large polygonal cells with markedly pleomorphic nuclei with evidence of mitotic activity with foci of necrosis, and occasional cells with intracytoplasmic melanin (Figure ). We also saw small segments of nerve containing ganglion cells with perineural deposits of melanin. The tumor cells showed diffuse positivity for S100 protein and Melan A. Occasional cells were positive for human melanin black (HMB)-45. The ki67/mib-1 was 20% to 25%.\nGiven the coexistent skin lesion reported as a blue nevus, neurocutaneous melanoma was considered as the provisional diagnosis. The postoperative hyperacute MRI showed no residual tumor. His symptoms gradually resolved after surgery. He received postoperative intensity modulated radiotherapy to the tumor bed (4950 cGy in 22 fractions to the planning target volume with a biologically effective dose [BED] of 60.39 Gy and an equivalent dose in 2 Gy fractions [EQD2] of 50.32). He was closely monitored during the follow-up period.\nHe presented again about 20 months later with recurrent symptoms of headaches for two months and diplopia and vomiting for two days. On examination, sensations over the V1, V2, and V3 dermatomes were reduced on the left side. There was masseter and temporalis muscle wasting on the left side with impaired blinking in both eyes. His spino-motor system and higher mental functions were found to be normal.\nA lobulated heterogenous signal intensity mass lesion was seen in the left CP angle and Meckel’s cave. The mass is predominantly isointense and weighted T1, and T2 images were hypointense (Figure ). Various other similar intensity lesions were seen along the left tentorium suggestive of recurrent meningeal carcinomatosis.\nThe brain MRI with contrast showed a recurrent mass in the left Meckel’s cave extending into the CP angle compressing the brainstem. Another lesion of a similar nature was seen in the lateral aspect of the cerebellum. There was evidence of thickening and abnormal enhancement of the adjacent pachymeninges and leptomeninges suggesting disease recurrence with leptomeningeal spread. He was further evaluated with a CT of his thorax and abdomen which ruled out extracranial disease.\nHe underwent a left retromastoid suboccipital re-exploration and subtotal excision of the tumor. A postoperative CT scan of his brain showed no residual disease. However, the patient died five months after the second surgery.
A 29-year old female diagnosed with SLE for 4 years complicated with grade II lupus nephritis presented with status epilepticus. She denied a history of fever on admission, but was treated with cyclophosphamide 1 month prior for an episode of cerebral lupus. She had noticed a papule over the left deltoid region which progressed to an ulcer over 1 week. Fever was noted following several days of hospital admission and the ulcer site became painful. She had worked in paddy fields several months prior to the admission when she was in good health. However, she could not recall any precipitating injury at the affected site during working. She is a mother of two and both pregnancies were uncomplicated. She denied history of alcohol abuse or smoking.\nOn examination she was emaciated and had a GCS score of 15/15 following recovery of status epilepticus. There was no obvious lymphadenopathy. At presentation, the size of the ulcer was about a 3 cm lesion and it gradually developed in to an ulcer with a necrotic center with surrounding erythema. A tentative diagnosis of pyoderma gangrenosum was made with the appearance of the ulcer (Figure ). It gradually advanced into the underlying muscle over 3 weeks of onset despite the antibiotic treatment. Examination of the cardiovascular, respiratory systems, and the abdomen was normal.\nHer full blood count, blood picture, and other supportive investigations showed evidence of microangiopathic hemolytic anaemia, which was suggestive of thrombotic thrombocytopenic purpura which resolved following plasmapheresis. Her ESR was persistently normal. Renal functions were stable during hospital stay, so were the liver profile. Chest radiography revealed evidence of bilateral mild pleural effusions and echocardiography revealed a thin rim of pericardial effusion and good cardiac function. MRI, MRA brain showed evidence of Posterior Reversible Encephalopathy Syndrome. Repeat imaging showed resolved changes.\nA punch biopsy of the skin was done from the lesion and sent for fungal studies and histopathological studies. The direct microscopy examination revealed wide and irregular ribbon-like nonseptate hyphae with right-angle branching suggestive for Mucormycete fungi. Culture was done on Sabouraud dextrose agar with chloramphenicol (at 26°C and 37°C) yielded a white aerial mold, which covered the entire surface of the agar and came up to the lid of the culture bottles after 4 days of incubation (Figure ).\nThe lactophenol cotton blue mount of the growth revealed broad, nonseptate hyaline sterile hyphae. The slide culture test has been attempted with the hope of sporulation, however it was not successful. They only resulted in broad, nonseptate hyaline sterile hyphae without spores. Then the isolate was subcultured on to potato dextrose agar (PDA) and Rose Bengal (RB) agar for induction of sporulation. However, they yielded only sterile mycelia.\nThe isolate was inoculated on nutritionally deficient medium, tap water agar and incubated for 14 days at 37°C. It provided a hazy view of flask shaped sporangium with rhizoids in lactophenol cotton blue mount. Then floating agar method was used and it yielded characteristic flask-shaped sporangium in short sporangeophore with rhizoids after 10 days of incubation (Figure ).The sporangia had a long neck and the apex of the neck closed with a mucilaginous plug. The sporangiospores were cylindrical, with rounded ends. Those morphological features were suggestive for S. vasiformis and the isolate was identified as S. vasiformis.\nThe histopathology of the punch biopsy of the skin also reveled broad aseptate hyphae suggestive of Mucormycetes group of fungi.\nBased on the histopathological evidence of broad aseptate hyphae, suggestive of Mucormycete fungi, the patient was started on IV amphotericin B deoxycholate. Repeated surgical debridement was done and samples were sent for fungal studies. However, local application of antifungals was not included in the management. Her second tissue biopsy, which was taken during debridement after 5 days of IV amphotericin B also had similar direct microscopy findings and yielded S. vasiformis. However third tissue sample which was obtained after 10 days after IV amphotericine B deoxycholate became negative for fungal studies. Following the confirmation of sterile cultures from the subcutaneous biopsies, superficial skin grafting was done which was completely accepted from the wound site. She was treated with intravenous conventional amphotericin B for 28 days and she was asymptomatic when she was discharged from the ward.
A 63-year-old man came to the clinical observation because of a rapid onset of dyspnea and dysphonia along with the development of a bulky node in the left side of the neck. He had been working as an interventional cardiologist in an angiographic room for 15 years at the local Hospital. Family history was negative with regard to malignancies and thyroid disease.\nThe relevant medical history included hypertension treated with valsartan and hydrochlorothiazide and non-insulin-dependent diabetes mellitus treated with metformin. There was no previous history of thyroid disease. Two years before the admission, he was treated with warfarin because of a deep venous thrombosis of the left leg occurred after a short bed rest for prostatitis. He was a heavy smoker.\nThe iodine status of the patient was not known; however, he was from a non-Alpine region and he was still living in the same area which is considered as a mildly iodine insufficient [].\nOn physical examination, the patient had a 8 × 10-cm firm left-sided neck mass with a right-sided shift of the larynx. On ultrasound examination, a nodule of the left thyroid lobe was found measuring 5 and 6-cm in its antero-posterior (AP) and transverse (T) diameters, respectively. The nodule was hypoechoic but inhomogeneous, with no vascularization; at the strain elastography, the nodule ranged from a medium elasticity to a hard pattern. The volume of the right thyroid lobe was reduced with a small hypoechoic nodule. No enlarged lymphnodes were found at the neck ultrasound. Computed tomography (CT) of the neck confirmed a 7 × 5 × 13-cm (T × AP × Long diameters) large, inhomogeneous neck mass originating from the left lobe that caused displacement of the trachea, the left common carotid artery and the left internal jugular vein. No evidence of primary malignancies or suspicious for secondary lesions was found at the CT of the head, abdomen, and pelvis. The chest CT showed a 6-mm round-shaped nodule not suspicious for malignancy close to the parietal pleura at the lower lobe of the right lung.\nA fine needle aspiration cytology (FNAC) of the mass was performed which yielded hemorrhagic smears with few groups of large, epithelioid cells, with vesicular, severely atypical nuclei and eosinophilic dense cytoplasms. A diagnosis of malignancy was given (Category 6 according to Bethesda 2010) with a suggestion for an anaplastic carcinoma (Figure ).\nThe patient underwent a total thyroidectomy and lymphadenectomy of central and left lateral cervical nodes. At the gross pathology examination, the tumor measured 6 × 6 × 12 cm (T × AP × Long diameters) and was partially circumscribed by a fibrous pseudocapsule. The mass had a gray, tan and red cut surface, with areas of hemorrhagic necrosis. Histology showed a vasoformative high grade neoplasia characterized by large epithelioid cells growing in sheets and lining abnormal vascular spaces; some cells showed intracytoplasmic lumina. There were areas of spontaneous necrosis and hemorrhage and a brisk mitotic activity; angioinvasion was noted. The tumor immunostained for vascular markers (CD31, ERG, CD34, factor VIII and vimentin), whereas epithelial differentiation markers were negative (cytokeratins, thyroid transcription factor 1, thyroglobulin, and EMA). The final histologic diagnosis was primary epithelioid angiosarcoma of the thyroid, grade 3 according to FFCCS (Figures ). This diagnosis was confirmed at a second opinion from a different institution. The tumor was restricted to the thyroid with free surgical margins. The mass had substernal extension and displaced the surrounding structures but it did not infiltrate the thyroid capsule, the strap muscles, or other neck tissues. The remaining thyroid tissue had nodular colloid goiter. No lymphnode metastases were detected.\nFifteen days after the thyroidectomy, the patient was operated to prevent rupture of an aneurysm of the abdominal aorta. One month after thyroidectomy, the chest CT showed multiple pulmonary nodular lesions some of them with a solid pattern surrounded by a ground-glass halo, 12 mm in maximum diameter. There was no consensus as to the oncologic relevance of these lesions, therefore, no biopsy was performed. A bone scintigraphy yielded negative results.\nChemotherapy with Epirubicin, Ifosfamide, and Mesna was administered but it was discontinued after 4 cycles because of pancytopenia and infection by Klebsiella Pneumoniae, treated with piperacillin/tazobactam, and by Clostridium difficile, treated with vancomycin. The patient recovered from the infections and, at a 6-month follow-up, the chest CT showed a reduction of the number and volume of the lung lesions with only three of them remaining in the medial lobe of the right lung.\nAt a further 18-month control, the chest CT was unchanged. The 6 mm round-shaped nodule close to the parietal pleura at the lower lobe of the right lung was also found to be stable. One year later, the patient developed pneumonitis and recovered after antibiotic therapy. At that time, he was investigated by neck, chest, abdomen and pelvis CT as well as with FDG-PET without any evidence of disease recurrence.\nAfterward, a 6-month CT follow-up program was started which is still ongoing. At present, the patient is alive with no evidence of disease after 62 months from initial diagnosis.
A healthy, 29-year-old male commercial airline pilot without individual or family history of skin cancer or other malignancies noticed a symptom-free movable nodular mass over the dorsal proximal phalanx of his left middle finger, near the metacarpophalangeal joint. The tumor gradually enlarged to 2 cm in diameter in two years and was associated with local erythematous change. An excision biopsy was performed by a general surgeon in a medical center. Histopathological examination revealed a nodular neoplastic lesion composed of spindle-shaped cells with frequent mitotic figures. Immunohistochemical stain was negative for actin but positive for CD34. Thin collagen fibers crisscrossing between the spindle cells stained positive with Masson trichrome. The pathological diagnosis was consistent with DFSP. Due to the close proximity of surgical margins to the tumor mass and the characterized highly recurrent nature locally of the tumor, a wider excision was strongly suggested by the general surgeon. The pilot visited the plastic surgery outpatient clinics in another hospital 3 weeks later for a second opinion. A wide excision and split-thickness skin graft with donor from left palm hypothenar region were done. Width of the surgical margin was not mentioned in the medical record. A pathology report revealed residual DFSP in the lower dermis and subcutis region of the skin surrounding the previous wound. Two weeks after his second surgery, he was admitted to the previously-mentioned medical center where a plastic surgeon carried out a wide excision of the skin and soft tissue around the previous wound. About 1 cm margin around the lesion was excised. The depth of the defect was 0.5 cm, subcutaneous tissue was involved, but tendons were spared. In order to make sure the surgical margins are free from tumor invasion, even with the frozen section, the reconstruction was delayed until definitive pathology report. The defect over the left middle finger was transiently covered with a cadaveric skin graft after confirming free of tumor invasion over surgical margins and base with intra-operative frozen section analysis (). Functional reconstruction was achieved 1 week later by tissue coverage, using a superficial palmar branch of the radial artery (SPBRA) free flap []. The SPBRA bifurcates from the main trunk of radial artery about 2 cm proximal to the distal wrist crease. Usually, one or two concomitant veins accompanied the SPBRA. After marking the SPBRA, an elliptical shaped 35 × 25 mm flap over the volar surface of distal forearm was designed to facilitate donor site closure (). A proximal incision was made to explore the SPBRA after confirmation by Doppler flowmetry. The flap was elevated, the SPBRA and its two concomitant veins were anastomosed to the digital artery and digital dorsal veins, respectively, under microscope. The flap was harvested as a sensate flap by incorporating palmar cutaneous branch of the median nerve into the flap. The donor site was closed without complication or obvious scarring. The recipient site achieved a full range of motion and showed a good contour. Both dynamic and static two-point spatial discriminations were tested and adequate protective sensation was attained.\nThe pilot did not disclose the whole process of tumor evaluation and management until his next annual aircrew physical examination. Upon doing so, he was grounded immediately. After reviewing pertinent medical records and being closely followed-up for 9 months without recurrence since the last operation, a waiver was granted and the pilot returned to flight status. To date, there has been no evidence of tumor recurrence 4 years after a complete surgical resection.
A 17-year-old girl presented with complaints of a progressively increasing swelling over the front of her left chest over the previous 5 months. The swelling was not painful, situated just adjacent to her left breast, with no associated shortness of breath. There was no history of trauma to the chest wall. She did not complain of an engorgement of the left breast, nipple discharge, or retraction. There was no family history of similar complaints. On examination, her vital signs were stable. There was a large, hard, non-tender mass, 25×30 cm in size, extending from just below the left clavicle to the 6th intercostal space and from the lateral border of the sternum to the mid-axillary line (). The mass itself was adherent to the chest wall although the overlying skin was not. The left breast had been lifted up by the mass and was not adherent to it. There was no palpable breast nodule, ulceration, nipple discharge, or retraction. There were no palpable lymph nodes. Breath sounds were equal and normal bilaterally. The systemic examination was unremarkable. Chest X-ray showed a diffuse opacification over the mid-lung field extending over to the lateral chest wall. Bronchovascular markings in the periphery were normal. A blood investigation revealed a raised lactate dehydrogenase and alkaline phosphatase, positive C-reactive protein, and normal total counts. Contrast-enhanced computed tomography (CT) of the thorax showed evidence of irregular destruction and periosteal reaction involving the left 3rd rib (). There was a large soft tissue component extending through the skin and the subcutaneous region, anterior mediastinum, and in the left upper lobe. This was causing compression of the left upper and lower lobe bronchus with a partial collapse of the underlying lung field. The lesion was also abutting the arch of the aorta and its branches and was found to be displacing the left brachiocephalic vein laterally. Magnetic resonance imaging (MRI) findings were similar with the lesion appearing hyper-intense in the T2-weighted images and hypointense in the T1-weighted images. Multiple necrotic areas were seen in the lesion. Fine-needle aspiration cytology from the mass was inconclusive, and a core-needle biopsy was taken, which was suggestive of chondrosarcoma. The patient was subsequently taken up for the exploration and excision of the mass. The chest was opened via a median sternotomy and the tumor freed from its anterior mediastinal attachment (). The superior, inferior, and lateral extents of the tumor were accessed via a left thoracotomy, dissecting under the sub-mammary plane, and the required resection of the 2nd to the 5th ribs. Sternal involvement required the resection of the left half of the sternum. Extensive involvement of the left lung parenchyma warranted a left pneumonectomy and a completion thoracoplasty to obliterate the potential space (). Major vascular structures and the left main bronchus were uninvolved. The biopsy report revealed features of a dedifferentiated chondrosarcoma with a highly pleomorphic sarcomatous component. Large foci of necrosis were present, and the tumor was found to infiltrate the lung parenchyma. All margins were found free of tumor involvement, thus signifying a complete resection. The patient had an uneventful postoperative course and was discharged on the 6th postoperative day.
A 51-year-old female referred to our department with the complaint of pain and swelling in the left mandibular region since two years. During systemic history, patient revealed low back pain, hypertension and diabetic mellitus Type 2 since 10-11 years. She underwent surgery of the maxillary sinus region which as per her previous records was treated with FESS (functional endoscopic sinus surgery), left conchaplasty with widening of maxillary sinus. The biopsied tissue of the ophthalmic and maxillary region showed inflammatory cells and was not suggestive of any malignancy. During course of time patient underwent root canal treatments of mandibular molars, premolars and incisors for her dental pain. However, the pain did not subside and patient referred to our department. On examination pain was mild, dull, and localized in nature. The family history of the patient was not contributory. Physical examination showed facial asymmetry attributable to diffuse swelling of the left mandibular region. Mouth opening and sensation over the distribution of the left inferior alveolar nerve was normal. Intraorally, the alveolar ridge posterior to the right first molar was covered with intact, normal-appearing mucosa. No teeth showed mobility or pain on percussion. There was no cervical or axillary lymphadenopathy. A two-year-old panoramic radiograph [] showed an ill-defined osteolytic lesion in the left posterior mandibular body, with involvement of the apices of both the first molar and the second molar and in the anterior part of the mandible involving the incisors and premolars of the left side of the mandible. However, a recent panoramic radiograph showed massive increase of erosion in the posterior and anterior part of the mandible [].\nBecause the clinical and radiographic findings led to suspicion of a malignancy, surgical intervention with biopsy of the lesion was planned and performed. The specimen, which was friable and resembled granulation tissue, showed a proliferation of histiocytic cells and an infiltration of eosinophils and neutrophils on microscopic examination. Soft-tissue fragments permeated by hematopoietic cells and predominantly mononuclear cells with clefted nuclei and granular cytoplasm were seen admixed with eosinophils, neutrophils and lymphocytes []. Immunohistochemical staining showed that the histiocytic cells were positive for the CD 68, CD1a, S-100 protein [Figures –], and Myeloperoxidaes were positive in the surrounding granulocytes. The CD 45 was also positive in lymphocytes [].\nThese features were consistent with a diagnosis of eosinophilic granuloma. To evaluate the possibility of systemic histiocytosis X, a radiographic skeletal survey and a visceral ultrasound was performed. These examinations detected no other lesions except a few changes in sacroiliac joint. The results of laboratory tests, consisting of blood and serum biochemical studies, were within normal ranges except there was a significant increase in ESR observed. A chest radiograph did not show any significant abnormality. Consequently, the mandibular lesion was diagnosed as eosinophilic granuloma. After three weeks of surgery the mandibular swelling decreased rapidly, and there was marked improvement in the facial asymmetry. Patient reported again after one year to us with a pain in the right mandibular posterior region. Computed tomography (CT) scans showed a destructive bony lesion extending from the mesial side of the right first premolar to the distal side of the second premolar []. A separate well-defined new radiolucent lesion was observed distal to the left mandibular first molar [].\nSecond surgery with curettage of the lesion was done along with biweekly intralesional 40 mg injections of methylprednisolone in the posterior lesion for the next eight weeks. The patient was also given systemic steroid Defcort 6 mg (Deflazacortil, Mehius India) per day for 30 days in a tapering manner along with surgical curettage. The patient was evaluated after every six months for the next two years. Patient was free of symptoms and signs and her radiographs revealed healed lesions in the anterior and posterior part of the mandible [].
A 28-year-old female patient with a chief complaint of swollen gums was referred to the Department of Periodontology, Navodaya Dental College and Hospital, Raichur (India) completing her orthodontic treatment at a private setup. On clinical examination, patient presented with abundance of calculus, with oral hygiene index of 3.3 referring to poor oral hygiene status. Patient presented with generalized erythematous gingiva with diffuse gingival enlargement. There were generalized deep true periodontal pockets with average probing pocket depth of 7-8mm with Grade II to Grade III mobile teeth in relation to11, 21 []. Patient was systemically healthy.\nA provisional diagnosis of generalized chronic periodontitis with inflammatory gingival enlargement was made. Radiographic investigations such as orthopantomograph (OPG) [] and full mouth intraoral periapical radiographs (IOPA) were advised []. OPG revealed moderate-to-severe bone loss. Angular defect was best appreciated in IOPA of tooth region 11-21. The inference from the clinical and radiographic investigations leads to the definitive diagnosis of generalized chronic periodontitis. No radiographs were available for comparing the condition before the orthodontic treatment.\nA comprehensive treatment plan was proposed which included an initial nonsurgical therapy, full mouth flap surgery with platelet rich fibrin (PRF), hydroxyapatite (HA) bone graft and subsequent esthetic rehabilitation along with reevaluation in between the treatment phase. The patient was explained about the treatment plan and informed consent was obtained for the same. Routine blood examinations were carried out and nothing of relevance was noted.\nTreatment started with Phase-I therapy that included patient motivation, thorough scaling and root planning with proper oral hygiene instructions Patient was on maintenance phase and recall visits were scheduled once in a month to assess plaque control measures and evaluate her response to Phase-I therapy. Re-evaluation for clinical parameters such as gingival inflammation, probing pocket depth, gingival enlargement and mobility was done after 3-month period Extracoronal wire and composite splint was fabricated for tooth region 13-23 before regenerative surgical procedure to manage the mobility associated with the teeth 11, 21 On achieving an improvement in patient's gingival health, decrease in clinical signs, and symptoms of gingival enlargement and inflammation. Surgical intervention was performed, that included internal bevel gingivectomy and modified Widman flap surgery to manage the gingival enlargement as well as the persistent pockets Regenerative procedures were performed wherever angular defects were present. Tooth region 11-21 showed a two-walled defect []. Whereas 16 and 26 both showed three walled defect with Grade II furcation involvement seen in 26 []. After complete debridement, root bio-modification was done with tetracycline (prepared by dissolving 500 mg capsule in 10 ml of saline). PRF was prepared by drawing 10 ml of patient's whole venous blood from antecubital fossa into sterile vacutainer tubes without anticoagulant. These tubes were then placed in the centrifugal machine at 3000 rpm for 10 min. A fibrin clot is then obtained in the middle of the tube just between the red corpuscles at the bottom and the acellular plasma at the top. The PRF thus obtained is mixed with the hydroxyapitite bone graft (sybograf ™ - Eucare pharmaceuticals) with particle size ranging between 600 and 700 μand placed in the 11–21 and 16 regions. The PRF was compressed on a glass slab to form a membrane and placed in the furcation defect of 26. Flap sutured and periodontal dressing placed Postoperative instructions were given to the patient. The patient was asked to refrain from mechanical plaque control measures in the surgically treated quadrant for 2 weeks and no intrasulcular brushing for 8 weeks. Advised soft diet >2 weeks. The patient was advised to use chlorhexidine 0.2% rinse until the completion of the treatment. Patient was prescribed with the following antibiotic and analgesic regimen, amoxicillin 500 mg thrice daily for 5 days, ibuprofen twice a day for 3 days. Sutures were removed 7 days postsurgery Patient was scheduled for maintenance therapy, recalled every 3 months in the first year and then recalled every 6 months. Patient was followed up for 2 years. Re-evaluation at the 2-year period showed an improvement in the clinical parameters, with complete resolution of gingival enlargement and establishment of knife edge margin with pink gingiva. There was a decrease in the mobility of the teeth 11, 21 from Grade III to Grade I []. Splint in the 13-23 region was subsequently removed. Postoperative radiographs at 9 months showed significant bone fill in the areas where regenerative techniques were performed [Figures and ].\nDespite extreme care and caution, patient had loss of interdental papilla which presented as black triangles in the anterior esthetic region as a result of the extensive periodontal surgery. Patient was explained about the periodontal plastic surgical procedures. However, due to her unwillingness for another traumatic and extensive surgery and further orthodontic treatment, an alternate treatment modality of fabricating a gingival prosthesis was preferred. Prosthesis was fabricated from flexible valplast material. Custom tray was fabricated from the diagnostic impression of the maxillary arch. After border molding, a secondary impression was made with addition silicone material. Using injection molding technique valplast prosthesis was fabricated. The patient was instructed with the use of prosthesis. The patient was very much satisfied with the esthetics achieved with the prosthesis [].
The patient is a 29-year-old previously healthy female, gravida 1 para 0, who presented for a scheduled induced abortion via dilation and evacuation at 22-weeks gestation, in the setting of a pregnancy complicated by a fetus with trisomy 22 confirmed on amniocentesis and multiple fetal anomalies. Workup prior to the procedure included a complete blood count which revealed a hemoglobin of 12.1 g/deciliter and platelets of 134 × 103/μl. No history of pre-pregnancy thrombocytopenia was noted. The patient’s pre-procedure systolic blood pressures ranged from 80 to 100 s mmHg.\nThe day prior to the procedure the patient had osmotic cervical dilators placed in the office. On the day of the procedure a singleton fetus with fetal heart tones was confirmed by ultrasound, monitored anesthesia care was administered with fentanyl, midazolam and propofol in an operating room, osmotic cervical dilators were removed, and a paracervical block with 1% lidocaine was performed. The patient then underwent ultrasound-guided dilation and evacuation using electric suction curettage and Bierer forceps. All fetal parts and the placenta were accounted for at the end of the case, with an estimated blood loss of 100 ml and no apparent complications. No hypoxia, tachycardia, or hypotension were noted in the operating room at the time of the procedure.\nIn the post-anesthesia care unit 15 min post-operatively, the patient experienced moderate vaginal bleeding (visually estimated to be 200 ml) with normal vital signs. The leading diagnosis for additional blood loss at this time was uterine atony; methylergonovine 200 micrograms intra-muscular was administered and a 500 ml crystalloid bolus was given. Forty-five minutes after the procedure moderate vaginal bleeding (visually estimated to be additional 200 ml) was noted again. On assessment vital signs were normal, transabdominal ultrasound revealed a thin endometrial stripe and no fluid posterior to the uterus to suggest intra-abdominal hemorrhage. Firm uterine tone was noted on bimanual exam. Rectal misoprostol (800 micrograms) was administered.\nFinally, 75 min post-operatively, the patient became acutely hypotensive (54/42 mmHg). Anesthesia and gynecology teams were immediately called to the bedside. An additional crystalloid fluid bolus was initiated and the decision was made to transfer the patient back to the operating room for evaluation, considering a differential diagnosis for bleeding including retained products of conception, uterine atony, cervical laceration, or coagulopathy.\nIn the operating room, a complete blood count, activated partial thromboplastin time (aPTT), and International Normalized Ratio (INR) were obtained. Monitored anesthesia care was again administered. A 1 × 2 cm piece of clot was removed from the endometrial canal on bimanual exam. Firm uterine tone was noted. Gritty texture and no ongoing bleeding was noted with repeated curettage with a suction cannula. No cervical lacerations were noted, however, hemostatic agents were applied to two site of oozing on the external os. Blood loss was estimated to be 750 ml total at this time.\nThe patient was transferred back to the post-anesthesia care unit, where an additional dose of intramusclular methylergonovine 200 micrograms was administered given continued moderate bleeding while awaiting lab results. Intra-operative lab results returned showing mild anemia, worsened thrombocytopenia, and abnormal elevation of INR and aPTT (Table , Lab 1). Clinical suspicion at this time was for possible AFE, given an episode of significant hypotension and coagulopathy out of proportion to blood loss. STAT repeat labs including a fibrinogen level were ordered, in addition to a ROTEM®.\nROTEM® results (Fig. ) showed very low amplitude fibrinogen, reflected in the low amplitude at 10 min (A10) in the FIBTEM channel, and prolonged clotting time (CT) (359 s) and reduced clot amplitude A10 in the EXTEM (16 mm) channel. Concentrated fibrinogen and transfusion of fresh frozen plasma and platelets were initiated immediately. Additional blood products were ordered from the blood bank. Fifty minutes after the initial ROTEM®, a repeat ROTEM® and repeat STAT labs were sent; at this time the full results were not yet available from the previous labs sent for analysis. More than 2 h after the first infusion of concentrated fibrinogen was initiated based on the first ROTEM® results, the first STAT fibrinogen level resulted as less than 25 mg/deciliter (Table , Lab 2). By the time these labs resulted, a second ROTEM® had been run and continued to show severe hypofibrinogemia, as shown in the FIBTEM A10 value (2 mm), and improvement in EXTEM CT (187 s) (Fig. ).\nConcurrently, the patient developed hypoxia, was placed on a non-rebreather mask and the surgical intensivist team was consulted. Labs sent at the time of the second ROTEM® confirmed ongoing coagulopathy (Table , Lab 3). After receiving 4 g concentrated fibrinogen, 2 units of cryoprecipitate, 4 units of fresh frozen plasma, 2 × 5-pack platelets, and 2 g of tranexamic acid, repeat labs were obtained; one unit cryoprecipitate and 2 units of packed red blood cells were given between obtaining the labs and receiving the results. Upon arrival to the surgical intensive care unit, labs showed worsened anemia (not yet reflecting the 2 units of packed red blood cells given after the labs were obtained), improving thrombocytopenia, normalization of aPTT and INR, and normalization of fibrinogen (Table , Lab 4). Repeat ROTEM® showed normalization of fibrinogen levels (FIBTEM A10 = 13 mm), CT (60 s), and EXTEM A10 (45 mm) (Fig. ).\nAfter transfer to the surgical intensive care unit, a chest radiograph showed diffuse hazy opacification predominant in the peri-hilar regions and lower lungs. The patient received heated high flow nasal cannula secondary to desaturations and 2 × 5-pack platelets for thrombocytopenia. She remained hemodynamically stable, her hypoxia resolved, and she was discharged on room air on post-operative day two. She was doing well and asymptomatic at her two-week post-operative appointment.
We present a 6-month-old African American male child with poor interval growth. His mother has noticed that though he is thought to be breastfeeding appropriately, as defined by feeding 4 ounces of pumped breast milk every 2-3 hours, and has been meeting developmental milestones, his weight and height are not as expected. He has been exclusively breastfed and his mother has not introduced solid foods as of yet to his diet.\nHe was a full term, spontaneous vaginal delivery without complications during the pregnancy or labor. He was in the 50th percentile for both height and weight at his 2 month visit, but has fallen to below the 3rd percentile for weight and is at the 3rd percentile for height. He is on no medications, there are no other siblings with failure to thrive and his mother has no post-partum depression or substance abuse issues. There is no family history of malabsorptive conditions. His mother and father are of normal stature.\nHis review of systems is negative for emesis, diarrhea, fever, appetite changes, swallowing abnormalities, respiratory symptoms, apnea, repeated acute illnesses, or frequent injuries.\nHis weight at the four month well child visit was 6477 grams with a length of 63.5 cm increasing to only 6761 grams and a length of 66 cm by his six month well child visit. His vitals signs are otherwise stable. His physical exam is significant for an alert, playful, developmentally appropriate child, small for his age. His head/neck, cardiac, respiratory, gastrointestinal, genitourinary, musculoskeletal and neurological exams were within normal limits.\nHe is appropriately diagnosed with failure to thrive at this visit based on deviation across two major percentiles on standardized growth curves. His estimated weight needs were calculated to 0.33 kg/month and a follow up visit was established in one month with addition of solid foods and continued breastfeeding with the addition of formula to pumped breast milk for increased caloric intake. His mother was also instructed to keep a strict food diary.\nInterval weight gain was not maintained with a weight of 7045 grams at follow up despite adequate caloric intake estimated based on his food diary and formula supplementation. Laboratory studies ordered were complete metabolic profile, thyroid stimulating hormone (TSH), lead level, and complete blood count (CBC). Electrolytes, kidney function, bilirubin, AST, ALT, protein, albumin, TSH, CBC, and lead were all normal. Alkaline phosphatase was elevated at 4280 (on repeat 6310). Normal should be less than 500 IU/L in neonates and 1000 IU/L in children up to age 9. Follow-up labs including gamma glutamyl transferase (which was normal, suggestive of boney resorption) [], C reactive protein, T3, free T4, phosphate, parathyroid hormone, and 25-OH D were ordered. Phosphate was low at 2.9 (normal 3.0-4.5) and the vitamin D level was 11 (45-50 ng/mL).\nA skeletal survey was ordered showing metaphyseal fraying and cupping of bilateral distal femurs, bilateral proximal and distal tibiae and fibulae, bilateral proximal and distal humeri, bilateral distal radii and ulni and the distal aspects of 2nd through the 5th metacarpals most consistent with rickets of the extremities, see radiograph 1 and 2. These classic findings may be paired with a separation of the periosteum from the diaphysis secondary to unmineralized osteoid when evaluating radiographic evidence of rickets [,]. Radiographic improvement should manifest within 3 months of appropriate treatment. Underlying malabsorptive conditions or noncompliance should be considered if this is not observed [].\nThe diagnosis of rickets was made and the patient was started on 2000 IU of vitamin D and calcium carbonate 1000 mg daily. He was also started on iron sulfate 22 mg daily and Zinc 20 mg daily as recommended by pediatric endocrinology and nutrition staff. His catch-up growth has lead to a current weight (at 2 years of age) in the 45th percentile and height in the 30th percentile (). Follow up labs (calcium, phosphorous, alkaline phosphatase) should be performed one month after therapy is initiated and again at 3 months along with magnesium, PTH, and 25-OH D [,]. Follow up labs in this patient indicated an improvement in vitamin D up to 29 ng/mL with ongoing supplementation continuing.
In November 2018, a 30-year-old woman presented to the Department of Oral and Maxillofacial Surgery of Chonbuk National University Hospital with a complaint of pain and swelling in the left mandible that had appeared 10 days earlier. Eleven years ago, she was diagnosed with CFD (Fig. a, b) and had received bone contouring in the left zygomaticomaxillary complex and left mandibular region under general anesthesia in the same department (Fig. a, b). Postoperative healing was uneventful and 18 months postoperatively, there was no specific problem with the lesion (Fig. c). The patient had subsequently been lost to follow-up until November 2018.\nA review of medical history prior to November 2018 confirmed that she had not received any dental treatment or suffered trauma to the painful left mandible area in recent months. Her pain intensity rating was 4 points on the numeric pain rating scale. Clinical examination revealed slight swelling in the left midface and left submandibular areas, along with bony expansion from the posterior of the left mandibular angle to the inferior aspect of the #34 tooth. The patient did not complain of hypoesthesia or pain when pressure was applied to the area. During an intraoral observation, the swelling was found from the distal aspect of #33 to the mesial vestibule area in relation to #36. During the endodontic examination, tooth mobility and percussion reactions were not observed in #34, 35, and 36. Moreover, the electric pulp test (EPT) showed normal response from #34, 35, and 36. No evidence of gum inflammation, such as periodontal pockets or gingival sulcus swelling and bleeding, was found during the periodontal examination. Furthermore, in the panoramic view, the dental origin with the possible infection source was not observed (Fig. d).\nA well-defined multilocular radiolucent lesion in the left posterior mandibular region was identified on the panoramic radiograph, and the location of the lesion overlapped with the existing FD. In addition, amorphous calcified foci were observed inside the lesion (Fig. d).\nCone-beam computed tomography (CBCT) showed an expansive bone lesion with a ground-glass appearance spanning the left frontotemporal bone, crista galli, orbital wall, ethmoid bone, sphenoid bone, zygoma, pterygoid plate, and maxilla regions (Fig. a). An ill-defined (partially well-defined) irregular osteolytic lesion was observed inside the left mandibular lesion, and cortical thinning, buccolingual expansion, and cortical destruction were also identified (Fig. a).\nA decision to perform a marsupialization procedure was made to first control edema and pain, and second to take a biopsy. The marsupialization procedure was performed after an intraoral incisional biopsy of the area surrounding the #34 and #35 teeth, followed by root canal treatments on these teeth. The biopsy results revealed some evidence of chronic inflammation and that the lesion may be a bony lesion with inflammatory reaction rather than FD. During a 3-week observational period, the size of the lesion was unchanged according to clinical and radiological findings (Fig. b). Eventually, we decided that cyst enucleation under general anesthesia should be performed in the mandibular region. However, in the maxilla region, since there was no pain or discomfort, we decided to follow-up without any surgical treatment.\nAt the time of surgery, the lesion had expanded from the inferior aspect of the #34 tooth to the mesial root of the #36 tooth, with fibrotic tissue scattered within the lesion. Subsequently, the soft tissue lesion was removed by cyst enucleation. The perilesional bone and the roots of the #34, 35 teeth, and the mesial root of the #36 tooth were ground. Electrocautery was applied to the interior of the lesion and a thorough curettage was performed (Fig. ).\nThe lesions removed by cyst enucleation were sent for tissue biopsy. The largest lesion was approximately 3 × 2 × 1.5 cm and was lined by a thick, fibrotic tissue layer (Fig. ).\nHematoxylin and eosin (H and E) stained sections showed dense collagenous tissue surrounding the osseous trabeculae, and peritrabecular clefting was present (Fig. a). Mitosis or atypia was not seen (Fig. b). The biopsy result revealed active nonspecific chronic inflammation with fibrosis.\nIn view of the radiographical and intraoperative findings, the absence of any history of trauma, a low probability of dental infection being the cause (as per endodontic and periodontal examinations), absence of evidence of malignant transformation (confirmed by histological findings) [–], the researchers confirmed that the case involved non-specific cystic degeneration in the CFD site. Postoperative healing was uneventful (Figs. and c), as was the postsurgical follow-up over a 6-month period (Figs. and d). Thereafter, the patient was lost to follow-up.
A 13-year-old male patient reported to the orthodontic clinic with the chief complaint of irregularly placed teeth in upper and lower jaw. His medical and dental history was noncontributory. On examination, he had an asymmetrical face with straight profile and competent lips. Chin was deviated toward the right side in occlusion due to the functional shift of mandible. There were no other associated signs or symptoms of temporomandibular disorder. Molar relation was Class I on both sides on Class III skeletal bases with average mandibular growth pattern. Posterior crossbite extended from canine to the first molar on the right side and on first molars on the left side. Maxillary and mandibular anterior crowding with deep bite was present with highly placed maxillary right canine and lingually placed lower left lateral incisor. There was upper midline shifted toward left side by 3 mm and lower midline shifted toward right side by 3 mm [].\nCephalometric analysis revealed that he had cervical vertebrae maturation index stage 3, Class skeletal bases due to retrognathic maxilla with retruded maxillary and mandibular anteriors. Panoramic radiograph showed full complement of teeth in maxillary and mandibular arch except third molar in all quadrant, which was in almost crown completion stage [].\nModel analysis showed that patient had overjet of 1 mm and overbite of 6 mm with arch length deficiency of 7 mm in maxillary arch and 5 mm in mandibular arch. Asymmetric maxillary arch with a constriction of 4 mm in the molar and 2.5 mm in the premolar region on the right side was found on occlusogram. The initial contact point of upper and lower tooth of patient during closing of jaw [].\nTreatment objective was to correct the posterior crossbite to prevent the functional shift of the mandible toward the right side, to coincide upper and lower midlines and to relieve the maxillary and mandibular anterior crowding and achieve normal overjet and overbite with good intercuspation. The patient was planned to be treated with RME device to expand the maxillary arch and simultaneously correct the upper midline shift with fixed mechanotherapy.\nRME was done using a banded type Hyrax appliance which was palatally soldered bilaterally to the bands of first molar and first premolar on either side with a 19-Gauge stainless steel wire. The molar bands were welded with molar tubes on either side. The removable lower posterior bite plane was given from mesial of the first premolar to the second molar bilaterally on lower arch to jump the bite and Hyrax appliance framework was cemented to the maxillary arch. The screw was turned twice each day, once in the morning and once in the evening, for a total of 0.5 mm activation per day. The activation was carried out until desired 5 mm expansion was achieved in 10 days.\nImmediately after the expansion, 0.022 slot McLaughlin, Bennett, Trevisi preadjusted brackets were bonded on the maxillary arch to correct the maxillary anterior crowding []. The central incisor started moving towards the midline diastema reciprocally by the residual forces of the elastic transseptal fibers and also using power chain. Tight figure of eight was given from maxillary right central incisor to right upper canine to prevent reciprocal movement of the right central incisor. The expansion appliance was left for 4 months aid to in retention. After the removal of the Hyrax appliance, the removable maxillary anterior bite plane was used for bite opening as well as retention. The mandibular arch was bonded, and 0.014″ NiTi is placed for alignment. Once the alignment was completed, coordinated 0.017″ × 0.025″ SS wire was placed in upper and lower arch. Pre- and post-model symmetry were compared with ortho grid. Posttreatment records show proclination of upper and lower anteriors for compensation of arch length-tooth size discrepancy []. Lateral cephalometric superimposition shows a significant amount of growth changes in maxilla, mandible, and soft tissue of face [].
A 52-year-old man presented to the emergency department with LBP. The patient fell from approximately 2 m-container box to the ground 2 hours previously. He complained of no pain and no numbness in either leg. A neurological examination revealed grade 5/5 muscle strength and no sensory changes in the lower extremity. Normal rectal tone was present and there was no saddle anesthesia. Plain lateral radiography and computed tomography (CT) scan showed a burst fracture with spinous process fracture at L3 with more than 50% of the vertebral body height loss measured on Picture Archiving and Communication System (π-ViewSTAR, INFINITT Healthcare Co., Seoul, Korea) (). Spinal canal diameter was estimated at the level of the fracture on the axial CT image that traversing the level of the pedicle and was defined as the distance between the midsagittal border of the posteriorly dislocated bone fragment and the lamina. The normal size of the canal was estimated by the average midsagittal diameter of the levels adjacent to the fracture. Spinal canal diameter at the fractured level was 7.92 mm using this method and about 55% narrowing of the spinal canal was observed compared to the normal level (). Magnetic resonance imaging (MRI) revealed findings suspicious of injury in the supraspinous ligament (). Initially, we thought the patient had no neurological deficits, so internal fixation and posterior fusion without decompression of the thecal sac were planned under the impression of an unstable burst fracture.\nHe underwent pedicle screw fixation from L1 to L4 and posterior fusion the next day, with internal distraction to reduce the fragment away from the spinal canal. Motor strength and sense in his legs were intact postoperatively. Pain was tolerable 3 days postoperatively, and the patient began to ambulate without difficulty. The Foley catheter was removed. He did not feel the desire to urinate. He was able to expel only a few drops of urine by applying pressure to the anterior abdominal wall when he tried to void at the time of 500 cc of urine in his bladder measured with bladder ultrasonography. This was managed initially by regular intermittent catheterization, but he complained of urethral discomfort. A Foley catheter was placed again the next day. He also complained of constipation, but this improved with kinetic agents (mosapride 10 mg three times a day) and laxatives (magnesium oxide 500 mg three times a day). Plain lateral radiography and CT scan confirmed restoration of sagittal alignment and appropriate positioning of the screws (). Although spinal canal diameter increased slightly to 9.29 mm, significant thecal sac compression was detected (). We recommend MRI and additional anterior decompressive surgery, but he refused because of uncertainity of recovering from voiding difficulty after the surgery and his poor economic status. The Foley catheter was removed again 1 week later, but he did not void as before. Electromyography (EMG), nerve conduction study (NCS), and urodynamic study (UDS) were performed 3 weeks after the surgery. EMG and NCS showed no abnormalities in L2-S1 roots. However, abnormal spontaneous activity and decreased motor action potential amplitude in the abductor hallucis muscle were detected, which did not exclude the possibility of S2 root denervation. Further electrodiagnostic evaluation for pudendal somatosensory evoked potentials and anal sphincter EMG were recommended, but he refused. No bladder detrusor muscle activity and areflexic neurogenic bladder were found in the UDS. He was managed with suprapubic cystostomy for 3 months. Voiding improved gradually during that time. CT scan of the lumbar spine at that time revealed a more resolved fractured bone fragment, and the spinal canal diameter increased to 10.94 mm (). After removing the cystostomy, he was able to void by himself, but had a weak stream and residual urine. He voided with abdominal straining or sitting on the toilet seat to void fully. Voiding has not returned to normal at the 15-month follow-up.
A 55-year-old Vietnamese male with no significant past medical history presents to a local community hospital emergency department because of abdominal pain and distention of two-week duration. The abdominal pain was generalized, described as cramping, present throughout the day, had no association with meals, and was getting progressively worse. The patient also reported nausea and vomiting clear material. He denied any recent fever, chills, night sweats, weight loss, change in bowel habits, sick contacts, and consumption of raw food. In addition, he denied any chest pain, shortness of breath, joint swelling, and skin rash. After immigration from Vietnam 11 years ago, his only travel outside the USA was back to his homeland one year prior to this illness. The patient was not taking any prescribed or over the counter medications or herbal compounds and denied any allergies. His physical examination showed no skin rash or jaundice, cardiopulmonary examination showed no abnormality, and the abdomen was moderately distended, with active bowel sounds, diffuse tenderness without rebound, and moderate ascites. There was no hepatomegaly or abdominal mass.\nIn the emergency department, a complete blood count and comprehensive metabolic panel were significant for an elevated white blood count of 15.400 with 36% eosinophils. Abdominal and pelvis computer tomography (CT) showed moderate ascites with thickening of the gastric antrum and proximal small bowel (). The patient was admitted to the general medical service and placed on bowel rest and intravenous fluid hydration. Further he underwent esophagogastroduodenoscopy (EGD), which demonstrated mild duodenitis and biopsies demonstrated mild nonspecific acute inflammation predominantly lymphocytic. Stool tests were negative for ova and parasites. In addition, a screen for Cryptosporidium, Cyclospora, Isospora, and Sarcocystis did not reveal evidence of recent infection. Over several days, the patient's abdominal pain improved, diet was advanced, and he was discharged home with an empiric trial of albendazole for a presumptive diagnosis of parasitic infection.\nTwo weeks after discharge, the patient was readmitted with worsening abdominal pain. Physical examination showed increased abdomen distention. Repeated blood counts and serum biochemical tests demonstrated an increase in white cell count of 17.100/mL with 71% eosinophils (absolute eosinophil count of 12.141/μl with normal upper limit <450) (). Liver function tests continued to be within normal limits. Serum IgE level was elevated at 548 IU/mL (normal < 180). Repeat stool tests were negative for ova and parasites. Furthermore, immunologic studies for Toxocara, Trichinella, Strongyloides, Filiaria, Schistosoma, Echinococcus, and Cysticerus were negative.\nRepeated EGD was nondiagnostic. Flow cytometry of peripheral blood revealed no myelo- or lymphoproliferative findings. Serum β-2-microglobulin and LDH were 2.2 mg/dL (normal: 0.8–3.0) and 170 U/L (normal: 80–200), respectively. Ultrasound guided abdominal paracentesis showed WBC count of 6600/mL, 95% of which were eosinophils (), LDH 284 mg/dL, albumin 3.2 g/dL (simultaneous serum albumin 4.1 g/dL). In order to exclude small bowel lymphoma, the patient underwent diagnostic laparoscopy with full-thickness biopsy of an inflamed portion of the jejunum. This revealed skipped areas of hyperemia and discoloration involving the small intestine and to a lesser degree the colon in addition to yellow-green ascites (). Histopathological evaluation showed marked eosinophilic infiltration of the muscularis propria and serosa with concomitant mild acute inflammatory reaction (). There was no evidence of malignancy, granuloma, TB, or parasites.\nThe constellation of clinical presentation and histopathological findings were suggestive of eosinophilic gastroenteritis. Subsequently, the patient was started on oral prednisone (20 mg/day). Two weeks later and with noticeable symptomatic improvement, the prednisone was tapered over a two-week period. After completion of steroids, the patient's abdominal pain and physical finding of ascites completely resolved and a peripheral blood count revealed an absolute eosinophil count of 300/μl (nL < 450). Furthermore, IgE level dropped to 105 IU/mL and CT imaging of the abdominal and pelvis showed complete resolution of the ascites and small bowel thickening. Four months have elapsed since treatment and the patient remains asymptomatic on no medications.
A 64-year-old Hispanic male presented to an outpatient surgical setting at an inner-city community hospital complaining of a newly discovered right axillary mass. The patient had no past medical or surgical history other than a left inguinal hernia repair performed 6 years prior for a reducible, asymptomatic, left inguinal hernia. On physical examination, the patient was noted to have a large, firm, irregular, fixed, non tender mass in the right axilla, consistent with a pathologic lymph node. He denied any constitutional or B symptoms. He also denied any weight loss, sick contacts, or recent travel. The patient was offered a short course of oral antibiotic therapy and was asked to return to the office in 2 weeks. When the patient returned, the mass had slightly increased in size. The decision was made to perform a right axillary lymph node excisional biopsy. Microscopic examination revealed scattered Reed-Sternberg (RS) or mummified cells mixed with reactive inflammatory cells within fibrous nodular background (Fig. ). Immunohistochemical stain results revealed that RS cells were positive for CD15 and CD30 (Fig. ). The final pathology was reported as Hodgkin’s lymphoma, nodular sclerosing type.\nHe was immediately referred to a hematologist/oncologist, and the patient was prepared to initiate chemotherapy. Various tumor markers, hepatitis panel, and HIV testing were all negative. During this time, the patient developed right epitrochlear lymphadenopathy discovered by the oncologist. When seen again by the surgeon during a postoperative visit, the patient complained of left scrotal pain with an associated left inguinal mass, which he attributed to a recurrence of his previously repaired left inguinal hernia. Upon close examination, it was determined that the patient had left inguinal lymphadenopathy, with characteristics similar to the right axillary contents that were previously excised. The oncologist, once notified of this finding, requested a PET/CT scan. The scan revealed multiple enlarged right subclavian, right axillary, and left inguinal lymph nodes with moderate to significant FDG uptake. Based on the nature of Hodgkin’s lymphoma, the recommendation was made for an excisional biopsy of the left inguinal lymph node due to the low likelihood of the mass having the same HL origin (as would be more likely with non-Hodgkin’s lymphoma).\nA left inguinal lymph node excisional biopsy was performed concurrently with placement of a left subclavian Mediport. Grossly, the excised mass had a similar appearance to the right axillary lymph nodes removed 2 months earlier. It was an encapsulated, flesh colored structure. Unexpectedly, the microscopic examination revealed thin fibrous septae surrounded by small round blue cells with an alveolar growth pattern. Immunohistochemical and molecular studies confirmed the diagnosis of rhabdomyosarcoma, alveolar type (Fig. ). The pathologic slides were sent to a tertiary center and reviewed for a second opinion, and the diagnosis was concurred. The dilemma now faced is this: with two primary neoplasms, which one should be treated first and what modalities of treatment would be most effective? Given that this presentation is exceedingly rare, the patient was referred to a highly specialized tertiary cancer center in order to provide the best multidisciplinary approach to this oncologic dilemma.\nHodgkin’s lymphoma typically presents as painless lymphadenopathy. The so called B symptoms, which include weight loss, fever, and night sweats, may be present and implicate a poorer prognosis. Due to mediastinal involvement, the nodular sclerosing type may present with chest pain, cough, and shortness of breath. Often, a plethora of diagnostic modalities aid in the diagnosis of Hodgkin’s lymphoma, but ultimately, a tissue diagnosis is needed. This is usually obtained surgically with an excisional lymph node biopsy, as intact morphology is required for an accurate diagnosis. The one exception to this is in head and neck masses, where a fine needle aspiration (FNA) may be pursued in order to rule out squamous cell carcinoma, which is very common in this anatomic location. Once the diagnosis is made, a clinical stage is deciphered, in order to orchestrate the treatment plan.\nHodgkin’s lymphoma can be described in four stages as follows: stage I (a single lymph node area or single extranodal site), stage II (two or more lymph node areas on the same side of the diaphragm), stage III (lymph node areas on both sides of the diaphragm), and stage IV (disseminated or multiple involvement of the extranodal organs). Four treatments options are available depending on the nature of the disease: radiation therapy, induction chemotherapy, salvage chemotherapy, and hematopoietic stem cell transplantation. An example of an induction chemotherapeutic regimen is cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) []. When induction chemotherapy fails, or the patient experiences relapse, a salvage chemotherapeutic regimen such as ifosfamide, carboplatin, and etoposide (ICE) can be initiated. Of note, HIV testing is obtained in all HL patients, as HAART therapy significantly improves outcomes in HIV positive patients with Hodgkin’s lymphoma [].\nRhabdomyosarcoma typically presents as a mass or area of localized swelling, and less than half of these patients present with pain. However, symptoms can be specific to the anatomic location of the lesion (e.g., cranial nerve compression in head/neck tumors invading the skull base). Diagnosis is made with a surgical biopsy (usually an incisional biopsy). CT and MRI are helpful for planning surgical resection, as well as planning routes and doses for potential radiation therapy post operatively. Locoregional and metastatic disease can be investigated with a bone marrow biopsy, chest CT scan, technetium diphosphonate bone scan, and even a lumbar puncture (for parameningeal primaries). Mainstay therapy for rhabdomyosarcoma is surgical resection. High-risk histology and advanced stage may warrant chemotherapy with or without radiation. For example, non-metastatic alveolar rhabdomyosarcoma is usually treated with the vincristine, dactinomycin, and cyclophosphamide (VAC) protocol with the addition of radiation therapy [].\nSpecifically relevant to this case, our patient initially presented with two groups of enlarged lymph nodes on the same side of the diaphragm and did not present with B symptoms. He subsequently developed involvement of a new group of lymph nodes on the other side of the diaphragm (left inguinal) without development of B symptoms. This was a rare presentation which raised the suspicion of the oncologist, prompting a second excisional biopsy. Specific to this case of Hodgkin’s lymphoma with synchronous rhabdomyosarcoma (alveolar type), overlapping treatment is available, thus making treatment options less cumbersome. Margin negative (R0) resection is the goal for the rhabdomyosarcoma. Given its aggressive histology and the possibility of incomplete resection, chemoradiation will likely be pursued. The CHOP induction chemotherapeutic regimen for Hodgkin’s lymphoma has some degree of overlap with the VAC protocol for rhabdomyosarcoma (vincristine being used for both). The challenge for the chemotherapist is the dose frequencies and potential toxicities that these patients may experience.
A 48-year-old, para 5, postmenopausal black African woman who was human immunodeficiency virus (HIV)-negative presented to our casualty department with a 5-year history of progressive abdominal swelling. Five years prior to presenting, which was 1 year before she reached menopause, she had noticed that her abdomen was gradually distending. Her symptoms were associated with constipation, feeling of incomplete rectal emptying, early satiety, vomiting, and urinary frequency and urgency. She did not have any chronic illnesses and had a negative personal and family history of ovarian, uterine, bowel, and breast cancers. She was not receiving any medication prior to this presentation. She had delivered five children by cesarean section, and they were all alive and well. She lived in a rural area and was a subsistence farmer. She did not smoke and did not drink alcohol.\nShe had ascitic taps three times in 1 week at a district hospital before referral to a higher-level hospital because of recurrent reaccumulation of ascites. A transabdominal ultrasound scan (USS) showed generalized ascites with a thick fluid with septa, as well as bilateral mild hydroureter and hydronephrosis. Again, the ascites was drained twice. One month later, she underwent computed tomography (CT), which showed a large predominantly cystic lesion that occupied almost the entire abdominal and pelvic cavities, which were distended, causing a marked mass effect on surrounding organs and bowel. The lesion had areas of internal septation predominantly on the right flank with no features of metastatic disease. Tumor markers measured during this admission are shown in Table .\nThe patient was referred to a tertiary hospital but only went 5 years later. Upon admission, she had marked temporal wasting, with bilateral pitting lower limb edema extending to her sacrum. She had a normal breast examination. Her blood pressure was elevated at 167/93 mmHg, with tachycardia of 150 beats/min. Her body temperature was 36.8 °C. She had equal air entry bilaterally, and her cardiorespiratory and neurological systems were normal. She had a lower midline scar with massive abdominal distention that was nontender and had a positive fluid thrill test result (Fig. ).\nResults of investigations showed microcytic anemia with hemoglobin of 9.9 g/dl, white blood cell count of 7 × 103/μl, and platelet count of 250 × 103/μl. She had normal urea and electrolytes and hypoalbuminemia of 21 g/L, with results of the rest of her liver function tests being normal. Urinalysis did not show abnormalities.\nHer case was discussed by a multidisciplinary team (MDT) that included gynecological oncologists, radiation oncologists, general surgeons, anesthetists, and nursing staff. The MDT considered the risks of death following hemodynamic instability and bleeding as well as the postoperative risks of deep vein thrombosis, pulmonary embolism, difficulty weaning off the ventilator, and death. The conclusion of the meeting was to take the patient for staging laparotomy despite these risks.\nPreoperatively, the patient and her relatives were counseled on the possible complications. The patient received a transfusion with 2 Units (U) of packed cells (PCs). The team also contemplated drainage of the mass preoperatively but was unable to secure appropriate drains to drain the thick green fluid from the mass.\nAt laparotomy, through a right paramedian incision, a huge abdominopelvic mass was found filling the whole abdomen and pelvis. The liver, spleen, and hemidiaphragms looked normal. The mass was shelled out by blunt dissection (Fig. ). The mass burst during mobilization, however, with rapid drainage of dark-colored fluid and decompression 50 min into the surgery. Subsequently, the mass was stripped off the anterior abdominal wall and completely excised. A total abdominal hysterectomy was done. The anterior abdominal wall was noted to have very deficient layers. Estimated blood loss was 450 ml. Anesthetically, the patient was unstable, particularly after the rapid decompression. She received a massive transfusion of 7 U of PCs, 6 U of fresh frozen plasma, and 4 L of gelafundin with 7 L of Ringer’s lactate. The surgery lasted about 3 hr. The patient was admitted to the intensive care unit (ICU) for cardiopulmonary support.\nThe patient was critically ill in the ICU. On the day of admission, she had a cardiac arrest and was successfully resuscitated. She required inotropic support and ventilation. By day 5, she had developed hepatosplenomegaly, uremia, and an increased international normalized ratio. She was leaking serous fluid from the suture line, with darkening of the previously stretched skin of the anterior abdominal wall. She remained anemic with thrombocytopenia. She received a further 6 U of PCs. She was kept on albumin and given high-protein energy feeds by the dietitian in view of her hypoalbuminemia and malnutrition. By day 6, she was noted to have disseminated intravascular coagulopathy. She was given vitamin K. A pneumothorax was noted, and the cardiothoracic surgeon inserted a chest drain. She was also noted to have aspiration pneumonia following a self-extubation on the same day. By day 10, she was noted to have multiple organ failure with oliguria, falling level of consciousness, aspiration pneumonia, a gangrenous abdominal wall (Fig. ), and coagulopathy.\nShe required inotropic support and ventilation. She died on day 10 after surgery. The pathology report showed a partial cystic lesion with benign epithelia of mucin-secreting columnar cells, no evidence of stromal invasion, no stratification, and no atypia and intraluminal mucin (Fig. ). The cyst walls were necrotic with fibrinopurulent exudate. Normal ectocervical and endocervical mucosa was observed. The endometrium was inactive, and there was evidence of an anterior fibroid. This confirmed the diagnosis of benign mucinous cystadenoma. Figure illustrates the sequence of events.
A 23-year-old French man of African origin, an elite football player, sustained a midshaft anterior cortex tibial stress fracture 2.5 years ago. Initially, he was treated with cast immobilization, no weight bearing for 3 months, ultrasound stimulation, and electromagnetic field therapy. The fracture did not heal; he had pain during gait, so he continued no weight bearing for 3 additional months. After that period, the fracture site still was not healed, so he underwent an operation performed by his team doctor. In this operation, the medullary canal of his tibia was reamed and an im nail was inserted.\nUnfortunately, the fracture site did not consolidate again, even 18-months postoperatively, so he presented to our clinic for counseling. It was obvious from the X-ray (Fig. ) that a nonunion of the fracture had occurred.\nHe did not smoke tobacco and he had a free medical history. When he presented to our clinic, the area at the fracture site was swollen and painful when palpated. The pain got worse when he attempted to walk with full weight bearing, so he had to use crutches. An examination of the peripheral nervous system of his lower extremities did not provide us with any pathologic findings. In addition, the laboratory examinations for possible endocrine or metabolic disorders were negative (Table ), so he was advised to have a reoperation to address this nonunion. The treatment options for such cases include nail exchange, drilling of the fracture site, bone grafting, or removal of the nail and internal fixation with a plate. We performed a tension band plate fixation, which is a technique already described for the treatment of anterior tibial stress fractures that failed non-operative treatment [], with bone grafting and without removing the nail.\nA longitudinal incision was made just lateral to the anterior tibial crest centered over the fracture site. The fascia over the tibialis anterior was divided, the muscle lifted off and the fracture site was visualized. The necrotic bone and callus at the fracture site was debrided with the use of an osteotome and a curette. Transverse drilling around the fracture site was done to promote healing and osteoblastic activity. Bone marrow from the ipsilateral iliac crest was inserted into the fracture site and a tension band plate was applied over the im nail.\nWe used a 6-hole, 4.5 mm locking compression plate. The plate was prebended and the screws were placed in a compression manner to achieve a tension band effect to the fracture site. A cortical screw was put first to the distal hole closest to the fracture site and then a cortical screw to the closest hole proximal to the fracture site to ensure compression of the fracture. Consequently, one unicortical locking screw was inserted proximally to the fracture site and the other two distally. With the use of locking and non-locking screws we minimized the pressure at the periosteum, which can damage blood supply to the poorly vascularized bone. The screws were angled in a different axis in order to bypass the nail (Fig. ).\nPostoperatively, our patient was advised to wear an orthotic boot and to not bear weight for 6 weeks. Range of motion exercise involving knee and ankle and isometric exercises were initiated immediately postoperatively. After 6 weeks he progressed to weight bearing as tolerated. At 3 months postoperatively he was pain free and started light jogging, swimming, and plyometric and core stabilization exercises. At 6 months postoperatively the complete radiologic union of the fracture was evident (Figs. and ). He was symptom free; he resumed at that time a full training program and he returned to play football 6 months postoperatively at his preinjury high competition level.
A 19-year-old woman presented to a headache specialty clinic complaining of a daily headache for 3 months. She awoke one morning with the headache and it had been continuous since that time. She denied any triggering event surrounding headache onset including no flu-like illness, stressful-life event or surgical procedure. The only possible trigger was a change in her birth control prescription 1 month prior. Her previous headache history was very minimal with infrequent tension-type headaches (TTHs), although she had several family members with probable migraine. The current headache was bifrontal and temporal in location. Average daily pain intensity was severe 7–8 out of 10 on VAS scale. The pain was sharp and throbbing in quality and was constant with no pain-free moments. There were no aura-type spells. Associated symptoms included nausea, vomiting, photophobia, phonophobia, osmophobia, and lightheadedness. She denied having any cranial autonomic symptoms with her headaches. There was a possible positional component to the headache being somewhat better while lying supine and in the morning hours but never any pain freedom. She also stated that over the past 3 months she had two spells of loss of consciousness in which the patient believed she fainted. There was no tongue biting or incontinence with these spells and she was unaware of any post-ictal confusion. However, there were no witnesses to these events. Her past medical history was significant for bipolar disorder. This was well controlled on carbamazepine. General and neurologic examinations were non-focal except for the presence of occipitonuchal and temporal tenderness to palpation bilaterally. Her mental status evaluation was normal. She had questionable cervical spine hypermobility on examination. She was taking ibuprofen on a daily basis without any benefit. She started the ibuprofen only after she developed the daily headaches. She had tried oral sumatriptan, but it was not effective. Prior to her evaluation at the headache clinic, she had a brain MRI with and without gadolinium which was read as a normal study; however, review of the imaging questioned loss of signal in the superior sagittal sinus. A diagnosis of probable NDPH was made, but further neuroimaging was suggested to rule out secondary mimics including an magnetic resonance venogram (MRV) and a magnetic resonance angiogram (MRA) of the intracranial and extracranial vessels. An EEG was also ordered for the loss of consciousness spells to make sure she was not having seizures. The patient was prescribed gabapentin as a preventive agent and was given salsalate, metoclopramide, baclofen and hydroxyzine as abortive agents. The following day she took gabapentin for the first time and added a metoclopramide tablet for nausea. Within minutes of taking these medications, the patient became giddy and after 10 min had a witnessed generalized tonic-clonic seizure lasting 8 min in duration with post-ictal confusion. She was brought to the hospital and there was seen by an epilepsy specialist. Further questioning of the patient and her mother revealed that for the past 3 months, the same time she was experiencing her daily headache, she had begun to have episodes of staring spells each lasting up to 10 s in duration and in which she would be non-responsive, have lip smacking and be picking at her clothes. These were occurring two times per week on average. In addition, she would experience occasional myoclonic jerks but these were very infrequent. Her EEG on hospital admission demonstrated 3–5 Hz generalized spike and polyspike wave discharges. MRV, MRA brain and neck were normal. Based on her clinical history of probable absence seizures, new generalized tonic-clonic seizures, myoclonic jerks and her EEG pattern, it was felt that this was most consistent with a diagnosis of JME. She was started on lamotrigine for the JME with a plan of tapering her off the carbamazepine which can worsen JME symptoms. On follow-up evaluation 3 weeks after hospital discharge and on a dose of lamotrigine 100 mg/day, she was both seizure free and headache free. She has now been followed for 9 months without any recurrent headaches or seizures remaining on the lamotrigine.
A 59-year-old woman presented for evaluation of scalp alopecia. Her past medical history was significant for PT1cN1mi estrogen receptor (ER)+, progesterone receptor (PR)+, human epidermal growth factor receptor (HER)2+ g3 invasive ductal carcinoma of the right breast diagnosed 15 months earlier. She had been treated with bilateral lumpectomy with right-sided sentinel lymph node biopsy and started chemotherapy nine months earlier; she received pertuzumab, docetaxel, carboplatin, and trastuzumab every three weeks for six cycles and was maintained on trastuzumab 6 mg/kg every three weeks for one year. Three weeks after completing taxane chemotherapy, she began treatment with anastrozole 1 mg daily (which was switched to tamoxifen 20 mg daily due to joint pain). She was also treated with radiation therapy and is currently on neratinib 240 mg daily; neratinib is a tyrosine kinase inhibitor anticancer drug used to prevent recurrence in patients with early-stage HER2+ breast cancer who have finished at least one year of post-surgery trastuzumab therapy.\nShe noted hair loss beginning after her first course of systemic chemotherapy. It became more extensive throughout the remainder of her treatment. She had not experienced any regrowth of scalp hair since the completion of chemotherapy nor during her current hormonal therapy.\nCutaneous examination revealed alopecia of the scalp. The clinical presentation was most consistent with female pattern alopecia with diffuse and nearly complete hair loss on the central and vertex region with retention of hair on the occipital scalp. There was partial, diffuse hair loss – to a lesser degree – on the parietal scalp bilaterally (Figure ). There was also loss of hair on the eyebrows, axillae, pubic region, and upper lip. However, these areas had already slowly started to show regrowth.\nBiopsies from the right and left sides of her parietal scalp, in areas of alopecia with some preservation of follicles, were performed for horizontal and vertical sectioning. Both showed similar pathologic changes of a non-scarring alopecia. The predominant feature noted was extensive miniaturization of the hair follicles; this change was most suggestive of androgenetic alopecia. However, other findings – present to a lesser extent – included pigment casts in hair follicles, increased catagen to telogen ratio, and empty fibrous tracks; these changes may be observed in alopecia areata.\nCorrelation of the patient’s history, clinical presentation, and pathologic findings supported a diagnosis of antineoplastic (chemotherapy and hormonal) treatment-associated alopecia. Specifically, her features were consistent with those previously reported in patients with breast cancer after taxane chemotherapy and adjuvant hormonal therapy who developed permanent alopecia []. Treatment was initiated with minoxidil 5% foam to be topically applied to the scalp twice daily.\nThe patient returned for follow up four months later. She was pleased with the clinical outcome and had noticed increased scalp hair growth; however, she commented that she always used minoxidil once daily and occasionally twice daily. In addition, hair growth on the eyebrows, axillae, and pubic area continued to demonstrate clinical improvement. She decided to continue treating her scalp in a similar manner.\nHer subsequent follow-up visit, six months later (after ten months of topical minoxidil therapy), showed additional hair regrowth. Specifically, the central and vertex area of her scalp had thickening of her hair; in addition, there was new hair growth on the parietal regions bilaterally (Figure ). She continues to use 5% minoxidil foam once daily.
A 53-year-old man noticed some swelling of his face. A chest x-ray showed a large mass projecting over the right lung field (fig ). A CT scan of the chest showed an enormous anterior mediastinal mass contiguous with the posterior aspect of the sternum and the right ribs, occluding the superior vena cava and compressing the heart (fig ). Blood tests were normal, tumor markers and Acetyl Choline Receptor Antibodies were negative. The patient underwent bronchoscopy and the biopsy showed the features of a carcinoid tumor.\nHe underwent excision of the tumor, which required resection of the superior vena cava and insertion of a graft, resection of the right phrenic nerve and plication of the diaphragm.\nThe finding at surgery was an enormous spherical tumor with an extremely vascular capsule due to the venous collaterals. The tumor was 20 cm from pole to pole and 15 cm in diameter. On the left it bulged into the left pleural space, displacing the phrenic nerve laterally. Superiorly it obliterated the left innominate vein and extend into the strap muscles. There was an enlarged lymph node. On the right it had obliterated the confluence of the innominate veins and the first centimeter or so of the superior vena cava. It enclosed the phrenic nerve over its complete length and was adherent to the medial aspects of the upper and middle lobes of the right lung. There was some thickening of the visceral pleura in this area and there appeared to be some superficial infiltration. Posteriorly it was adherent to the pericardium and there was some thickening of the ascending aorta, consistent with longstanding pressure effects. There was a small bloody pericardial effusion and a rather large right pleural effusion. There was no evidence of any pleural deposits or intrapericardial deposits.\nThe histology of the resection specimen showed a tumor weighing 2.1 kg. The tumor appeared encapsulated with an irregular surface. The tumor invaded the surrounding fat and pleura. There was infiltration into the underlying lung tissue lymphatics. There was some residual thymic tissue within the surrounding fat. Metastatic deposits were found in the right hilar lymph node and the node removed from the left cervical horn of the thymus. The tumor was classified as an atypical carcinoid of the thymus (neuroendocrine carcinoma of intermediate grade 2).\nOne month later he underwent a CT scan of the chest. There were findings consistent with a recent sternotomy and a graft of the superior vena cava. There was a fluid filled cyst at the base of the right lung and areas of atelectasis and pleural thickening were noted in both lungs.\nAn I-123 MIBG scan was performed with negative results for detection of any residual or recurrent pathology.\nHe was treated with postoperative radiotherapy and chemotherapy.\nOne year later, a follow-up CT scan of the chest was obtained with no findings of recurrence or abnormal lymph nodes in the mediastinum. Linear atelectasis was detected in the lower lobes of both lungs and elevation of the right hemi diaphragm was noted. In addition a bone scan was performed with a positive finding at the posterior arch of the 8th right rib. The patient was advised to have a SRS, for a more accurate evaluation of his status, as it was considered that "functional" imaging performed by Nuclear Medicine examinations, might detect pathological processes earlier than "structural" images, such as CT.\nSpot images of head-neck, thorax, abdomen and lumbar region were obtained 3 hr after injection of 5 mCi In-111-DTPA-D-Phe 1-pentetreotide. The scan was carried out on a Sopha single headed tomographic gamma camera coupled to a dedicated NXT computer system. Spot images of the areas of interest were obtained with a high-energy all-purpose collimator using 20% windows centered at 171 and 245 keV.\nThe SRS was reported as showing an area of abnormally increased concentration of the radiopharmaceutical at the anatomical area of the mediastinum due to either a mass remnant or a recurrence of the disease or an inflammatory process (fig. ). The bone scan finding did not take up the radiopharmaceutical. A repeat bone scan after a further 9 months was negative.\nThus, the main questions that we had to answer were: is there a mass remnant, or a recurrence of the disease? and, can we exclude a metastatic lesion at the site of the finding on the bone scan?
This paper presents the case of a 50-year-old male German academic who developed neurocognitive deficits, affective symptoms, and predominant delusions for over one and a half years. The patient, as well as his family, have given their signed written informed consent for this case report, including the presented images, to be published. The patient reported a pronounced feeling of thirst, although he was drinking 10–20 liters of water a day, and he was absolutely convinced that he would die of thirst. He did not hear an imperative voice encouraging him to drink more. He said that he needed to drink less water and wondered how his body could retain all the liquid. He was delusionally convinced that he had lost the ability to urinate. In contrast, sonography repeatedly detected no residual urine. Moreover, he suffered from severe insomnia (the patient had the feeling of being completely unable to sleep), reduced cognitive performance—for example, being unable to remember dates—and reduced energy levels, as well as complete loss of interests. In the external assessment, disinhibition, flattened affect, accelerated speech and slow thinking were striking. He had suffered from recurrent depressive episodes since he was 34 years old. For the past eight years, depression had been more severe. In the period between 45 years and 48 years, he had taken high doses of Z-drugs in order to treat insomnia. At the same time, alcohol abuse had developed until two years ago (up to the age of 48 years). At the age of 48, he had suffered a single epileptic status, which was interpreted as the result of alcohol and Z-drug withdrawal in an external hospital. At that time, epileptic activity was described in EEG. Over the last two years he had not consumed any substances (). In addition, hyponatremia (i.e., 130 mmol/L; reference range: 136–145 mmol/L) had been revealed half a year prior to diagnosis. On admission to our hospital, a normal sodium concentration was measured. Additional repeated laboratory testing showed a urine osmolality of 73 and 45 mosm/kg, respectively, and a suppressed urine sodium concentration of less than 20 mmol/L. During a thirst trial, urine osmolality increased to 651 mosm/kg, which indicates psychogenic polydipsia and excludes a syndrome of inappropriate antidiuretic hormone (SIADH).\nDiagnostic findings: The diagnostic examinations were conducted approximately one and a half year after symptom exacerbation after admission to our special ward. In serum, anti-LGI1 antibodies were repeatedly positive in different laboratories (see ), whereas CSF antibody testing was negative. A serum titer of 1:80 (reference <1:20) was measured using cell-based assays. CSF analyses showed normal to slightly elevated white blood cell (WBC) counts (maximum 5/µL; reference <5/µL) and evidence of a blood–brain barrier dysfunction (protein concentration up to 557 mg/L; reference: <450 mg/L; albumin quotients up to 9.5, reference: <8). Oligoclonal bands were always negative. Fluid-attenuated inversion recovery (FLAIR) MRI sequences depicted hyperintensities right-mesio-temporally and on the right side of the amygdala. In the left thalamus, a small, possibly microangiopathic, lesion was detected. In addition, there was a slight grey–white matter blurring (cf. [] ). The routine EEG was normal in the visual assessment. Independent components analyses or rather automatic detection of intermittent EEG slowing remained inconspicuous (cf. []). A cerebral FDG-PET examination showed moderate hypometabolism of the bilateral mesial to medial frontal cortices, which was interpreted as being possibly due to an early manifestation of frontotemporal dementia [], whereas a sequela of substance and/or alcohol abuse was rated to be less likely. It should be noted that there was no mesial temporal or striatal hypermetabolism (), which can be present in active limbic or anti-LGI1 encephalitis. A whole-body FDG-PET/computer tomography scan detected no metabolic or structural pathologies suggestive of malignancy or inflammation. Neuropsychological testing using the Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) test battery showed deficits in Mini Mental State Examination (25 of maximum 30 points), word-list savings, and trail-making tests B and A/B. The test for attentional performance by TAP showed deficits in working memory (missings, false alarms), set shifting (overall index) and alertness (reaction times with and without sound). All diagnostic findings are summarized in .\nIllness, somatic, and family histories: The patient’s past medical history was inconspicuous in terms of in-utero and birth complications. He had not suffered from febrile convulsions, inflammatory brain diseases, relevant systemic infections, or craniocerebral traumata during his childhood or adolescence. There was no evidence of neurodevelopmental or personality disorder. Seven years earlier, at the age of 43, he had developed a deep vein thrombosis by unclear coagulopathy. For several years, he had suffered from leg ulcer on the left inner ankle. He had also suffered from arterial hypertension, treated with ramipril 10 mg/day. His history of autoimmune diseases, infections, or cancer was negative. In the family history of neuropsychiatric diseases, only his father had suffered from depression.\nTreatment and outcome: Classic psychiatric treatment with sertraline, venlafaxine, mirtazapine, reboxetine, several tricyclic drugs, lithium, zopiclone, olanzapine, risperidone, haloperidol, quetiapine, and clozapine previously administered had no relevant positive effect on symptom relief. Under clozapine, he had developed myocarditis. After immunological findings gave evidence for chronified limbic autoimmune encephalitis, glucocorticoid pulse therapy with 500 mg of intravenous methylprednisolone daily for five days and subsequent oral tapering over two months (starting with 50 mg) led to slight improvement of mood and neurocognitive symptoms including temporal orientation, executive functions, word list savings and alertness times (with no difference between the conditions with and without sound). However, new deficits were found in fluency and word list learning with more word intrusions, while deficits in working memory and set shifting were persisting. A second steroid pulse 3 months after the first pulse with oral tapering did not lead to relevant improvement. However, the signal hyperintensities in the MRI showed a tendency to reduction. Further treatment (e.g., with plasmapheresis and/or rituximab) was refused by the patient and his legally authorized parents. Furthermore, l-thyroxine (due to hypothyroidism), vitamin B1 (prophylactic after earlier alcohol abuse), and vitamin D (due to serologically proven deficiency) were supplemented.
A 14-month old boy presented with respiratory distress, wheeze and hypoxia. This was preceded by 24 h of coryza, fever and reduced feeding. He was admitted directly to intensive care, where he was diagnosed with pneumonia. Treatment was started with empirical antibiotics. He was intubated and ventilated for 4 days, after which he was extubated and stepped down to high dependency unit (HDU) on continuous positive airways pressure (CPAP) ventilation. He continued to have persistently increased work of breathing, persistent expiratory wheeze and symptoms and signs of air trapping.\nThough he did not progress to respiratory failure, weight gain and oxygenation was achieved only by initiation of Heated Humidified High Flow Nasal cannula therapy (HHHFNC).\nPolymerase chain reaction (PCR) testing of nasopharyngeal aspirates (NPAs) revealed the persistence of rhinovirus and adenovirus for 10 weeks in both NPAs and bronchoalveolar lavages (BALs), with parainfluenza type 3 found in just one NPA sample. Adenovirus was also found in blood by PCR testing. Rhinovirus is not normally tested for in blood samples and there was no validated assay available for this. One BAL and one urine sample were screened for cytomegalovirus (CMV) by PCR also, and found to be negative. One stool sample was tested and found to be negative for rotavirus and adenovirus (despite the persistence of adenovirus in the respiratory samples at this time).\nThere was no significant family history of any genetic diseases and the patient was born at term. Mum is a smoker but states that she did not smoke during her pregnancy. During pregnancy, intrauterine growth restriction was identified. At the time of admission his weight was < 0.4th centile, but this was increasing. His parents report poor feeding since birth and an increased work of breathing from 5 months old. He is known to the allergy services for severe eczema and faltering weight, and has been diagnosed with cow’s milk protein allergy. His eczema medications have included topical tacrolimus, moderately potent topical steroids and emollient therapy.\nHis other medical history includes two previous episodes of bronchiolitis, three and 1 month prior to this admission (including one overnight stay during a trip to Australia). Prior to admission, he had only received two courses of antibiotics in his life – once for an infected BCG vaccine site and once for an ear infection.\nDuring his inpatient stay, he had 2 further intercurrent bacterial respiratory infections, treated by broad spectrum antibiotics. Immunological investigations revealed an IgG and IgM hypogammaglobunemia but a normal IgA. He was up-to-date with his childhood immunisations prior to admission, but has had a poor response to tetanus and twice to haemophilus B, demonstrated by low IgG levels to both. He was also found to have near absent B-cells and a reduced number of T-cells, and prophylaxis for Pneumocystis jiroveci was commenced. HIV testing was negative on two occasions. We withheld any further live virus vaccinations, in view of his immunodeficiency (Table , Fig. ).\nAt this stage, a CT chest was performed (Fig. ). The findings (mosaic pattern attenuation of both lung fields with a combination of air trapping and oligaemia, more pronounced in expiratory images) were consistent with BO which was believed to be secondary to the persistent adenovirus rather than the rhinovirus infection. Reasons for this were mainly that rhinoviruses are predominantly upper respiratory tract infections, whereas AdVs can cause more systemic infections and AdV DNA was detected in the blood. No biopsy or histopathological investigation was performed in order to confirm BO.\nA flexible bronchoscopy showed structurally normal airways. Screening for tuberculosis was negative.\nAfter a multidisciplinary team discussion, it was decided to commence pulse methyl-prednisolone therapy (3 daily doses of 10 mg/kg given every month) and long-term azithromycin (10 mg/kg 3 doses every week) for management of BO. Prior to commencing the steroids, cidofovir was administered to control the adenovirus replication and reduce the dissemination of the adenovirus.\nCidofovir (5 mg/kg, intravenously, with probenecid) was given on fortnightly basis for 4 months, during which he his renal function was monitored weekly. All the patient’s renal function test results remained within our institutional laboratory’s normal ranges (sodium 133–146 mmol/L; potassium 3.5–5.0 mmol/L; urea 1.5–5.0 mmol/L; creatinine 15–31 μmol/L) throughout the treatment period with cidofovir, indicating good tolerance to the drug. Since commencing steroid therapy, it was possible to gradually wean him off his HHHFNC, and he has since been thriving well. He was discharged on supplemental oxygen, which was weaned off over the next 12 months.\nAn array comparative genomic hybridisation (CGH) revealed mosaic tetrasomy of short arm of chromosome 9. There are no reports yet of immunodeficiency in association with mosaic tetrasomy 9p, nor any specific vulnerability to any particular viral infections [], but has previously been associated with auto-immune conditions associated with IFN-1 dysregulation (predisposing to inflammatory myositis and lupus-like syndrome) [].\nA conclusive diagnosis of a specific immunodeficiency/immunomodulation is yet to be made in this case, as this profile does not fit any primary immune deficiency. Molecular genetic testing for mutations in 82 genes associated with primary immunodeficiency and gastrointestinal disorders (TIGER panel, Great Ormond Street Hospital, London) did not identify any clearly pathogenic variants. The patient is currently being given weekly supplemental immunoglobulins.\nMost recently, the child has now cleared the long-term rhinovirus and adenovirus infections, and has been weaned off steroids and long term oxygen therapy. His weight gain has been adequate on air, and he is still too young for spirometry testing. Ongoing problems include recent Pseudomonas aeruginosa infection, with a wet cough, which is likely due to the bronchiectasis component of the BO disease.
The patient is a 54-year-old white male with a past medical history significant for bipolar I disorder with psychotic features and posttraumatic stress disorder admitted from the emergency department to the acute psychiatric floor with symptoms of mania and psychosis. Prior to admission, the patient had been taking variable doses of lithium for more than 10 years with poor adherence noted by the patient and prescription fill history. Per hospital records, the maximum daily dose of lithium the patient received was 900 mg. However, during this period, the patient received care at hospitals outside of the health care system, so it is unknown if the dose was adjusted.\nIn the emergency department, hospital day (HD) 1, serum lithium concentration was negligible at <0.2 mEq/L (reference range 0.6-1.2 mEq/L). Due to past therapeutic success, lithium was reinitiated with 450 mg lithium carbonate sustained action in the emergency department. Once admitted, an additional 900 mg of lithium carbonate sustained action was administered on HD 1 for a total dose of 1350 mg with plans to reinitiate a maintenance dose of 900 mg daily. Labs were not drawn until approximately 12 hours after receiving the 900-mg dose of lithium, so a baseline serum calcium was not available. Also on this day, the patient was continued on his home regimen of quetiapine 300 mg at bedtime. Serum calcium was measured to be at least 11 mg/dL through HD 4. On HD 5, serum lithium was therapeutic at 0.9 mEq/L with an elevated serum calcium of 11.1 mg/dL and normal PTH of 62 pg/mL. On HD 6 the patient remained asymptomatic of hypercalcemia but requested a different medication be started due to fear of thyroid complications he read about in an informational handout. Lithium was discontinued, valproic acid 750 mg twice daily was initiated, and quetiapine was increased to a total daily dose of 400 mg due to continued psychotic symptoms. Serum calcium taken on HD 10 and HD 13 were within normal limits at 10.1 mg/dL (). The psychiatric symptoms began to subside, and he was discharged on HD 15 on valproic acid 750 mg twice daily and quetiapine 400 mg daily. No other medications were administered during admission other than haloperidol intramuscularly for agitation on HD 2.\nOther potential causes of hypercalcemia were assessed. The patient did not exhibit any other signs of malignancy and had a thyroid-stimulating hormone that was within normal limits at 1.02 μU/mL (reference range 0.36-4.50 μU/mL). Additionally, the patient did not report taking any medications, vitamins, or supplements prior to admission, nor was he taking any medications inpatient that were suspected to increase serum calcium.\nDuring past trials of lithium therapy, including a time frame of 3 to 6 months prior to admission, the patient experienced a similar rise in serum calcium with a normal PTH (). The serum calcium collected in the absence of lithium treatment nearest this admission was 6 months prior. This value (10.1 mg/dL) appears to be the patient's baseline serum calcium in the absence of lithium, chronically near the upper limit of normal but <10.2 mg/dL. In all cases, serum albumin was within normal limits (3.4-5.5 g/dL), suggesting that serum calcium values were accurate. Using this information, lithium was considered the probable cause of hypercalcemia based on the Naranjo Algorithm Adverse Drug Reaction Probability Scale.
A 50-year-old caucasian woman was referred to our clinic with a 12 months history of severe, recurrent pain episodes in her right knee. She was a non-smoker, with no other notable family or medical history for any pathological condition. Systemic diseases such as gout, systemic sclerosis, dermatomyositis, and sarcoidosis or any metabolic or endocrine disorders such as hyperparathyroidism and renal failure were excluded. There were no past trauma incidents. Her orthopedic surgeon had diagnosed a calcifying lesion of the MCL 9 months prior to admission to our hospital and had treated her conservatively under the assumption of a calcifying tendinitis-like pathological condition. After some temporary pain relief she still suffered from pain at the medial side of the knee and limited range of motion. The conservative management included analgesics, non-steroidal anti-inflammatory drugs (NSAIDs), electro-therapy and shock wave therapy.\nPhysical examination of the right knee revealed local pain and swelling over the medial femoral condyle with intact soft tissues and no joint effusion. The patient was able to fully extend her knee but flexion was limited to 120° with increasing pain at the MCL beginning with 90° flexion. Patellar mobility was not limited. There was no ligament laxity and clinical tests for detection of meniscus lesions were all negative. There was no sensorimotor deficit. Blood tests showed no evidence of haematological or metabolic abnormality. Knee x-rays revealed linear soft tissue opacity medial to the femoral condyle, suggesting a lobate calcifying lesion within the proximal and middle section of the MCL and surrounding tissue. Antero-posterior, lateral and merchant’s views verified the presence of the calcified lesion. MRI without contrast agent of the knee confirmed the presence of this lobate structure, which was characterized by an hypointense signal on the T2 weighted images, located in direct vicinity of the intact MCL in the coronal and sagittal plane, with direct contact to the ligament structure, which was seen in the sagittal and axial plane images of the knee. There were no signs for a mature bony lesion (Figs. and ).\nDue to failure of conservative treatment regimes, we recommended surgery and performed an arthroscopy followed by an open procedure with removal of the calcified lesion. Intraoperatively, we found areas of chondromalacia grade 2 of the patellofemoral compartment and the medial femur condyle. The calcification body was directly attached to the MCL, which was structurally intact. Its consistency was toothpaste-like analogous to the macroscopic appearance of a calcifying tendinitis of the shoulder. The directly adhering parts of the soft tissues were also removed.\nThere were no complications during the postoperative course. Weight bearing started with 20 kg and increased to full weight bearing over 2 weeks, which was well tolerated.\nAfter wound healing and recovery of the soft tissues, the patient was pain-free after 4 weeks. Clinical examination and radiographic evaluation in the next 12 months demonstrated no signs of recurrence without any clinical symptoms (Fig. ).\nDuring these follow up examinations, the patient described recurrent symptoms of pain and restricted movement of the right shoulder, which had persisted previously for over a year. She had suffered from calcifying tendinitis in both of her shoulder joints 17 years ago. Back then, the x-ray pictures had shown calcification bodies near to the greater tubercle at the insertion of the supraspinatus tendon. These lesions had been treated successfully using shockwaves at that time, and had not re-appeared since.\nA current MRI survey demonstrated a rupture of the supraspinatus tendon. The patient successfully underwent a shoulder arthroscopy with a rotator cuff repair in our department. Surgery revealed signs of degenerative changes of the tendon, which had led to the rupture, but no evidence for residual calcium deposits within the supraspinatus tendon were found (Table ).
The index patient was a 34-year old female referred to the bariatric clinic by the general practitioner on her own request to treat her morbid obesity. She was born with a normal birth weight but large head circumference for which she never had a diagnostic analysis. At the age of five, her body weight was already significantly higher compared to her peers. No specific life events could explain her obesity. Cognitive development was normal and she followed normal education. She underwent treatment for recurrent nasal polyps. Her mother also had a large head size and suffered from morbid obesity as well. She was diagnosed with thyroid cancer and died from a pulmonary embolism after placement of an Adjustable Gastric Band. A maternal aunt was diagnosed with breast cancer before the age of 50 and the maternal grandmother died from breast cancer at young age. The younger sister of the index patient was overweight and was reported to also have a large head size (Figure ).\nSince childhood, the index patient followed several different coaching programs to change her eating behavior and exercise pattern to induce weight loss. She lost weight several times but was never able to maintain her weight loss. At the time of the intake procedure at the bariatric clinic, her height was 1.69 m (SD −0.2) and weight 164 kg (SD +6.8), resulting in a Body Mass Index (BMI) of 57.6 kg/m2 and a predominant abdominal obesity. Head size was not measured at that time since this is not part of bariatric screening procedures. Biochemical analysis of the blood revealed no abnormalities, and excluded endocrine hormonal disorders such as hypothyroidism. The fasting glucose level was 5.9 mM.\nThe combination of early onset morbid obesity resulted in suspicion of a genetic cause of her obesity. She was offered diagnostic genetic analysis of 52 obesity–associated genes to identify a possible underlying genetic obesity cause.\nThe patient was eligible for bariatric surgery and underwent a sleeve gastrectomy without complications (performed in 2014 using a standardized fashion). At 1, 2 and 3 years after surgery, she achieved a percentage Total Body Weight Loss of 39.4, 48.8 and 44.9, respectively. This resulted in a current BMI of 30.1 kg/m2. This was within the range of the results which were observed in a control group of 18 female patients, with a negative obesity genetic test result. These female patients were matched for age and BMI and achieved a percentage Total Body Weight Loss (TBWL) of 30.3 after 1 year, 31 after 2 years and 30 after 3 years of follow-up.\nA few months after surgery, the result of the obesity gene panel analysis was returned and showed heterozygosity for a known pathogenic mutation in the PTEN gene (): c.202T>C p.(Tyr68His). This mutation has been described previously in patients with PTEN Hamartoma Tumor Syndrome (PTEN HTS) (Marsh et al., ). No other pathogenic mutations were shown in the remaining 50 obesity–associated genes (Table ). At the genetic clinic, a head circumference of 63 cm (+5SD) and pedigree analysis (family history of multiple tumors) further supported the molecular diagnosis of PTEN HTS.\nAccording to the PTEN HTS guidelines, patients with pathogenic PTEN mutations are advised to visit the outpatient clinic for familial tumors, for lifelong surveillance of tumors that are associated with the PTEN mutations (Dutch Guidelines, ; Eng, ). Our patient underwent additional biochemical laboratory- and ultrasound screening to exclude thyroid gland carcinoma. Besides a few benign nodules on the ultrasound, no abnormalities could be determined. A yearly follow-up ultrasound of her thyroid gland and yearly serum thyroid stimulating hormone analysis was advised. Screening for breast, endometrium and colorectal cancer, also revealed no anomalies.
A 40-year old male laborer applied to our clinic with a complaint of sustained pain on his right shoulder and an inability to move his right shoulder after being injured in an in-vehicle car accident. According to his anamnesis, he was diagnosed with second and third rib fractures and pneumothorax that were initially treated at a thoracic surgery department for 6 weeks. After he was discharged, he applied to our clinic with right shoulder pain. We discovered that an AC dislocation type 6 had been overlooked when he first admitted to emergency department. There wasn’t a relevant family or medical history.\nOn physical examination, we observed that the patient’s right shoulder was swollen and deformed. There was local tenderness on the AC joint but no neurological deficits in the right shoulder or arm. Active range of motion (ROM) of the right shoulder also could not be determined due to pain. The patient was keeping his right shoulder internally rotated; there was a restriction during passive internal rotation, and passive external rotation was very painful.\nRadiographs showed an inferiorly displaced distal clavicle (). Computed tomography demonstrated subcoracoid AC dislocation type 6 (). The distal clavicle was in front of the glenoid and blocking the humeral head. Nondisplaced and healed right scapular body fractures and multiple rib fractures were also spotted.\nSix weeks after the accident, we performed open reduction and internal ligament and capsular repair of the AC joint. The patient was placed in a beach chair position. An inverted “J” incision was made extending from posteromedial corner of AC joint to the lateral border of coracoid process. The deltoid and trapezius muscles were found to have been stripped off the lateral end of the clavicle. In addition, the acromioclavicular ligaments were disrupted. The fibrotic soft tissue in acromioclavicular joint was removed. Conjoint tendon was intact; the distal clavicle was felt under the coracoid process. Coracoacromial and coracohumeral ligaments around coracoid process were cut, and pectoralis minor tendon was released from the medial border of the coracoid. The distal clavicle was removed under coracoid process by releasing the soft tissue. By abducting the shoulder, the lateral traction of the scapula was done. Thus, the distal clavicle was reduced into its anatomical position. After the reduction, because of the osseous disorganization related to the clavicular osteolysis, the distal tip of clavicle was excised about 5 mm. Because of the inferior displacement and a 6-week injury, coracoclavicular ligaments could not be evaluated clearly. There were no coracoclavicular ligament parts that could be repaired. With the help of the K-wire, four bone tunnels were opened in both acromion and distal clavicles. Internal bracing was performed for acromioclavicular joint with No: 2 ethibond© sutures (Ethicon LLC/US) []. Finally, the deltotrapezial fascia was attentively repaired over the top of the clavicle. After the incision was closed, the upper extremity was placed on an arm sling. Postoperatively, shoulder movement was restricted but wrist and elbow movement was possible. Passive ROM exercises were initiated 4 weeks after surgery. Active ROM exercises were initiated at fifth week after surgery, and stretching and strengthening exercises were prescribed as a home program at the second month, postoperatively. At final follow-up first year postoperatively, he had no pain when moving his injured shoulder, and his motion was similar to the other shoulder (forward elevation, 175°/175°; internal rotation to T10, and external rotation, 60°/60°; bilaterally; ). Final radiographs 1 year after the surgery showed good reduction of AC joint with minimal ossification, but no calcification or bony bar imaging (). At that time, the patient returned to his previous daily activities and his job with pain-free right shoulder movement.
A 41-year-old male with a past medical history of intravenous drug abuse (IVDA) presented to our emergency department as a transfer following two days of medical management of rhabdomyolysis at an outside community hospital. He initially presented with weakness and numbness of the lower extremities. He had been using intravenous heroin and was lying supine on a floor for approximately 24 hours.\nUpon presentation to our emergency department, the patient had pain in his bilateral lower extremities and no appreciable motor function below the level of the knee. He was in mild distress with a temperature of 37°C, pulse of 104 beats per minute, blood pressure of 146/56 mmHg, respiratory rate of 18 and oxygen saturation of 97% on room air. Examination of the lower extremities revealed erythema and tension in the bilateral posterior thigh compartments. In contrast, the medial thigh compartments bilaterally along with all four leg compartments were soft. Gluteal and buttock compartments were soft and compressible bilaterally. Motor strength testing revealed 1/5 strength bilaterally with manual testing of ankle dorsiflexion and plantarflexion, and knee flexion and extension. The patient was found to have bilateral sciatic nerve palsies, with no sensation bilaterally in the tibial nerve, deep and superficial peroneal nerve distributions. Both legs were warm and pink with brisk capillary refill along with 2+ dorsalis pedis pulses bilaterally. Provocative testing demonstrated pain with passive range of motion of the knees bilaterally and no pain with passive ankle dorsiflexion and plantarflexion. Lab work was significant for a creatinine phosphokinase (CPK) of 231,360 U/L.\nGiven the high suspicion for compartment syndrome, the patient underwent compartment pressure testing via Stryker pressure monitor system. Diastolic blood pressure recording measured 75 mmHg. The right posterior thigh compartment measured 75 mmHg and left posterior thigh compartment measured 30 mmHg. Based on the history and physical examination findings, the patient was diagnosed with bilateral thigh compartment syndrome likely secondary to his recent IVDA and subsequent immobilization and was taken to the operating room for urgent bilateral posterior thigh fasciotomies.\nIn the operating room, the right posterior compartment was approached first. The intermuscular septum was released with the fascia along the posterior compartment for full release of both the anterior and posterior compartments of the right thigh (Figure ).\nFollowing fasciotomy, cautery was used to confirm the viability of the exposed muscles in the anterior and posterior compartments. The skin surrounding the incision for the compartment release was too tense for safe closure, and so it was decided to place a wound vac. A 22 x 8-cm negative pressure vacuum-assisted closure (VAC) dressing was then placed over the wound (Figure ).\nAttention was then paid to the left thigh. Compartment pressures were rechecked. The left posterior thigh compartment measured 30 mmHg. Given these findings, the decision was made to forego the compartment release due to the morbidity associated with fasciotomies.\nSerial wound vac changes were then carried out on postoperative day (POD) two and four. On POD six, we removed the wound vac and placed a DermaClose device for assistance with skin closure. On POD 10, his skin edges were closable and so he underwent complex wound closure approximately 20 cm in length.\nThroughout operative treatment and recovery, the patient was given antibiotics and developed no further complications. His CPK levels returned to normal. He was discharged on antibiotics and was given counseling on the potential adverse effects of IV-drug abuse. He had a favorable recovery at two-week follow-up.
An eight-year-old female patient who suffered from tooth trauma half a year ago was treated in the local general hospital. Recently, she came to Department of Pediatric, School of Stomatology, the Fourth Military Medical University for treatment, with the chief complaint of recurrent swelling of the gum in the anterior teeth region and occlusal pain. Intraoral clinical and X-ray examination showed that the neck of the crown oftooth 21 was broken at the 1/3 position, white filling was seen on the fracture surface, probing (–), knocking (+), and no obvious loosening of the tooth was found (). Periapical radiograph showed that the apical foramen with tooth 21 was open with periapical shadow and the pulp chamber was wide.\nAccording to the treatment plan, the tooth was sterilized, triple antibiotic paste (main components were metronidazole, ciprofloxacin and minocycline) was sealed, and the status of the periapical region was observed. After the inflammation was eliminated, apexification was performed. After root development was completed at the age of ten, the root canal was treated and the defect of the tooth was repaired. The tooth will be permanently repaired when the child grows up into an adult.\nAt a follow-up visit, we found that the periapical shadow in the tooth gradually became smaller. Apexification was performed using Vitapex (). Six months later, X-ray radiography showed that there was radiolucency in the root canal, the length of root increased and the apical barrier formed. Under the rubber dam isolation, the length of the root canal was measured and the root canal was prepared. The root canal was initially prepared with #10 and #15 K filings, then prepared with nickel-titanium instruments, and washed with 5.25% sodium hypochlorite and normal saline alternately. The root canal was filled with cold gutta-percha (). During the filling of the root canal, multiple radiographs from various angles showed that radiolucency still existed in the root canal ().\nCone-beam computed tomography (CBCT) showed that the roots were bifurcated in the middle third of the root in the mesiodistal direction, and there were two apical foramina in the apices. The root canals were explored and dredged under the microscope. We found that in the middle third of the root in the mesiodistal direction, a thin layer of canal wall divided the root canal into two independent canals. The root canals were measured again for their lengths, prepared with nickel-titanium instruments, and sealed with calcium hydroxide for two weeks before the next visit. Under the rubber dam isolation, the root canals were washed with 5.25% sodium hypochlorite and normal saline and dried, and the master gutta-percha cone was fitted. The root canals were filled with the vertical compression technique. After the operation, the quality of the filling was evaluated on a periapical radiograph (). Finally, Z350 composite resin was used to repair the tooth.\nThe patient was followed-up at three, six, and 12 months after endodontic treatment. Clinical examination showed that the tooth had no percussion pain, the sinus was completely healed, gingival tissue was normal, periodontal probing depths were 2–3 mm, and there was no abnormal loosening. Periapical radiography showed that there was no periapical radiolucency with 21, no external absorption in the root and the periapical periodontal ligament was continuous.
In November 2011, a 70-year-old Caucasian female with a background of essential hypertension, diverticulosis, and mild chronic obstructive pulmonary disease presented with an eight week history of significant weight loss (24 kilos), early satiety, postprandial dyspepsia, and nausea with intractable vomiting right after recovering from a lower respiratory tract infection for which she completed two courses of antibiotics in the community (amoxicillin and clarithromycin) with complete recovery. She was a life long smoker of 10–20 cigarettes per day and was drinking thirty units of alcohol weekly. She had no family history of gastric or bowel malignancy.\nOn clinical examination, her body mass index was reduced with no pallor or jaundice. Respiratory examination revealed slightly decreased air entry bilaterally but otherwise was unremarkable.\nRoutine electrocardiogram showed sinus rhythm with occasional premature ectopics. Her relevant laboratory findings including liver function tests, urea and electrolytes, bone profile, amylase, C-reactive protein, tumor markers, and full blood count were all within the normal range, except for a low number of platelets and prolonged aPTT.\nInitial differential diagnosis included gastric outlet obstruction, malignancy, and peptic ulceration. Chest and abdominal X-ray ruled out mechanical obstruction. Upper and lower gastrointestinal tract endoscopy revealed erythematous mucosa in the body of the stomach and multiple sigmoid diverticulae but ruled out ulceration, obstruction, and malignancy.\nFurther investigation with abdominal-pelvic computed tomography and magnetic resonance imaging of her small bowel showed few clinically insignificant incidental non-gastrointestinal abnormalities but no other evidence of intra-abdominal pathology.\nHaving ruled out mechanical obstruction and in view of persistence of intractable vomiting, gastroparesis was suspected clinically even though there were no findings suggestive of gastric stasis or dilatation in the previous investigations. Thus, patient was further investigated with gastric emptying studies which was found to be grossly abnormal with half emptying time to be approximately three times the upper normal limit for patient's age, confirming a diagnosis of severe gastroparesis.\nInitially, patient was conservatively treated with the combination of metoclopramide-erythromycin and dietary modifications for small frequent low in fat semisolid meals but, due to inadequate control of her symptoms and further deterioration of her nutritional status, it was decided that nasojejunal feeding followed by total parenteral nutrition was required. Her clinical condition improved and patient was then referred for consideration of temporary and if well tolerated permanent gastric pacing.\nFollowing the insertion of the gastroscopic neuromodulator wires and despite the improvement of patient's symptom's score, gastric emptying study further deteriorated and permanent gastric pacing was deferred based on current evidence.\nAs nasojejunal feeding was well tolerated and long term total parenteral nutrition was declined by the patient, distal gastrectomy with a wide Roux en Y gastrojejunostomy was considered in order not only to facilitate gravity led gastric emptying but also to prevent aspiration.\nPatient underwent the procedure uneventfully and the excised specimen of the stomach was found to be largely devoid of ganglion cells which extended up to the resection margins in the duodenum with associated chronically inflamed nerve fibers suggestive of gastroparesis due to nerve injury ().\nPostoperatively, although intractable vomiting and nausea improved, patient could only tolerate small amount of enteral nutrition which was inadequate to meet her nutritional needs.\nOne week later, patient developed severe nosocomial lower respiratory tract infection with Klebsiella pneumoniae and Pseudomonas aeruginosa requiring intensive care unit admission and, unfortunately, died three weeks postoperatively.\nPremortem CT scans and bronchoscopy did not show signs of malignancy but postmortem examination revealed evidence of small cell lung carcinoma (SCLC) with malignant cells to be present along the excised thoracic lymph nodes but without identification of the primary tumor.
A 51-year-old man was admitted due to a wound at the right hand and progressive subcutaneous nodules at the right arm for 1 month. Three months prior to the admission, he was bitten at the right hand by a stray kitten. The cat was sick and died 6 days after the bite due to unknown diseases. The patient felt pain after the bite and immediately flushed the wound with tap water for several minutes but did not take other measures for dealing with the wound. He had no fever, and the wound healed 2 weeks after the bite. However, 1 month prior to the admission, the healed wound cracked with pus, and he found multiple subcutaneous nodules at the right arm. He felt nauseous occasionally but did not have fever, pain, vomiting, cough, dyspnea, pain, and headache. He went to local clinics and received antibiotics, but he did not know which agents were given. His wound appeared to be gradually healed again but became a verruca-like nodule, and new subcutaneous nodules emerged at the right arm. He went to the West China Hospital for further treatment. He had been a cottonsmith for many years and lived in Liangshan Region, Sichuan Province, Southwest China, with a subtropical climate. He had no travel history within the past few years. He was previously healthy with no underlying diseases such as diabetes. He drank alcohol occasionally and did not smoke cigarettes or use illicit substances.\nOn admission, he was afebrile. A 2 cm × 1.5 cm nodule-like wound was present at the back of the right hand (panel A, ) with mild tenderness, and pus emerged when the wound is pressed. About 20 subcutaneous nodules without erythema (about 1 to 1.5 cm in size; panel B, ) were detected at the right arm. There were no other remarkable findings on physical examination. The blood test on admission revealed white blood cells 5.24 × 109/L (reference range 3.5 to 9.5 × 109/L) with 55.5% neutrophils (reference range 40% to 75%) and 35.1% lymphocytes (reference range 20% to 50%), hemoglobin 159 g/L (reference range 115 to 150 g/L), and platelets 205 × 109/L (reference range (100 to 300 × 109/L). Serum biochemistry tests on admission showed that all values were in the normal range including total bilirubin 19.6 μmol/L (reference range 5 to 28 μmol/L), alanine aminotransferase 28 IU/L (reference range <50 IU/L), aspartate aminotransferase 24 IU/L (reference range <40 IU/L), albumin 43.7 g/L (reference range 40 to 55 g/L), globulin 27.2 g/L (reference range 20 to 40 g/L), urea 7.7 mmol/L (reference range 3.38 to 8.57 mmol/L), and creatine 57 μmol/L (reference range 53 to 140 μmol/L). He was married and denied any sexual activities outside the marriage. Serum tests for HIV, hepatitis B virus (HBV), hepatitis C virus (HCV), and syphilis were negative. An interferon gamma release assay for tuberculosis (Wantai BioPharm, Beijing, China) was negative. Pus was collected by pressing the wound after proper disinfection and was sent for culture.\nCulture of the pus on Sabouraud Dextrose Agar (Autobio, Zhengzhou, Henan, China) at 28°C after 3 days grew Sporothrix spp. (; a macroscopic view is shown in panel C, and a microscopic view [lactophenol cotton blue stain] is shown in panel D), which was also able to grow on self-prepared Potato Dextrose Agar at 30°C. The diagnosis of sporotrichosis was therefore established. Genome DNA of the strain was prepared using the sodium dodecyl sulfate (SDS) lysis method for yeast as described previously []. Partial sequences of the chitin synthase 1 gene chs and the DNA topoisomerase II gene top2 were amplified from the strain as described previously [,], and amplicons were sequenced using the Sanger method. The partial sequence of chs (389 bp) and top2 (632 bp) was 100% and 99.5% (629/632 bp), identical to the corresponding sequences of Sporothrix globosa CBS 120340 (GenBank accession no. LVYW01 []), respectively, but was 98.7% (381/386 bp) and 84.2% (532/632 bp) to Sporothrix schenckii 1099–18 (GenBank accession nos. XM_016735087.1 [] and XM_016734889.1 [ XM_016734889.1/]). To further confirm the species identification, we designed 3 pairs of primers to amplify genes encoding DNA polymerase α subunit B (Sk/g-F1, 5′-CCATTGTTCCGTATGCCACGT; Sk/g-R1, 5′-CGGGCCCGAGGCGAACAT), DNA-directed RNA polymerase I subunit (Sk/g-F2, 5′-GTTCATCGTGGTAGTTTCT; Sk/g-R2, 5′-TCGAGCGCCGACTTGTTGATAAT), and DNA-directed RNA polymerase II subunit (Sk/g-F3, 5′-ATCCAGTCGGAGGTGTCGCTGGTGCGC; Sk/g-R3, 5′-CGCACCTTGACGTACCGCAG), and amplicons were sequenced. The obtained 572-bp, 830-bp, and 980-bp sequence was all 100% identical to that of S. globosa CBS 120340 (GenBank accession no. LVYW01) and was 94.4% (540/572 bp), 96.9% (804/830 bp), and 97.2% (953/980-bp) identical to that of S. schenckii 1099–18 (GenBank accession nos. XM_016732750.1 [], XM_016729801.1 [], and XM_016737181.1 []), respectively. The corresponding sequences of the strain in this study have been deposited in GenBank under accession nos. MN956987 and MT126697-MT126700. Therefore, it was confirmed that the strain belonged to S. globosa, which was previously known as clade III of S. schenckii [] and is the prevalent Sporothrix species in Asia including China [].
A 73-year old female patient 150 cm tall and weighing 50 kg visited our hospital presenting with the complaints of lower back pain and radiating pain to right lower extremity. The lower back pain started when the patient fell down nine years ago. At first, mild lower back pain had occurred from time to time, but severe pain with a visual analogue score (VAS) of 8 or higher started to occur three months prior to her visit. The patient did not have any particular medical history except osteoporosis diagnosed in her late fifties. She felt pain as if her body was collapsing or sinking, accompanied by pulling pain at the hip and groin. The pain became more severe when standing up and moving, and relieved when lying down at rest. The physical examination showed tenderness at the first lumbar (L1) spinous process region. There were no abnormal findings in any other neurological examinations. The plain radiograph showed severe osteoporosis over the vertebral bodies, a severe compression fracture of the L1 vertebral body in which the anterior compression rate was 70% or higher, and an intravertebral vacuum cleft in the vertebral body (). Magnetic resonance imaging showed a low signal area of L1 in the T1 weighted image and a high signal in the T2 weighted image ().\nDespite conservative treatment for more than four weeks including continuous epidural pain control, the patient's pain did not decrease and she was rejecting surgical procedures such as a spinal fusion. Although the case was not a general indication of percutaneous vertebroplasty and kyphoplasty due to the severe collapse of the vertebral body, we decided to do transpedicular kyphoplasty using this method to block the cement outflow by slowly injecting highly viscous bone cement.\nBefore the procedure, cefazolin 1 g was intravenously injected to the patient as a preventive antibiotic. We had the patient take the prone position, supporting her abdomen with a pillow, in order to reduce abdominal lordosis. To check the patient's state during the procedure, we monitored the blood pressure, heart rate, and oxygen saturation, with supplement of oxygen through a nasal cannula. The skin on the area to be operated on was aseptically prepared and draped. For the sake of sedation and analgesia during the procedure, when needed, we injected fentanyl intravenously with a total of 100 µg. Using the C-arm image, we verified the pedicle of L1 and determined a working cannula implantation position and angle. Local infiltration anesthesia was performed with 1% lidocaine on the expected cannula implantation pathway. Under fluoroscopic C-arm guidance, we implanted the cannula into the fractured vertebral body through the vertebral pedicle. At the moment when the left cannula was inserted into the vertebral body, air flowed into the vertebral body with a popping sound, and the level of the vertebral body recovered spontaneously (). The cannula was also inserted into the right side, and balloons were inflated with a pressure of 50-100 psi to recover the level of vertebral body. Then, 4.5 ml of bone cement was slowly injected through each side of cannulas through the L1 vertebral body (). While injecting the bone cement, cement leakage out of the vertebral body was meticulously examined in both posteroanterior and lateral fluoroscopic views. The kyphoplasty was successfully implemented without cement leakage or clinical problems. The patient was discharged after the procedure without any particular abnormal findings. After the procedure, the VAS of the patient was 2, indicating that the preoperative pain almost disappeared. Currently, the patient during follow-up visits has not received any particular treatments except for the medication for osteoporosis.
A 35-year-old African American man was initially sent to our emergency room to evaluate a possible head trauma after a witnessed fall. He complained of frequent falls and leg weakness for 3–4 months. The weakness appeared to be progressive and persistent without episodic worsening. He had to use a cane to compensate but was still able to ambulate. His family members also endorsed cognitive defects over the past few months before admission. These impairments consisted mainly of apathy and withdrawal from social interactions; he used to be fully independent but now stayed at home most of the time. His medical history was only significant for alcohol abuse (three to four cans of beer and red wine daily for 2 years), and his family history was unremarkable.\nPhysical examinations showed an age-appropriate, alert, and oriented man. He was able to engage in conversations with a paucity of speech and flat affect; he also made eye contact and followed all commands during our evaluations.\nThere was bilateral weakness with spasticity in all lower extremity muscle groups. The deep tendon reflex was hypoactive with an upward response of the plantar reflex on the left side. He also had a wide-based unsteady gait and poor limb coordination. The remainder of the neurological examination and a general examination were unremarkable.\nInitial brain computed tomography (CT) in our emergency room demonstrated a mass lesion occupying the left caudate and extending into his frontal lobe (Fig. ). A brain MRI showed bilateral and symmetric hyperintense signals in the corpus callosum, periventricular white matter, and internal capsule (Fig. ) with an axial fluid-attenuated inversion recovery (FLAIR) sequence with gadolinium enhancement (Fig. ). A cervical spine MRI was negative for cord compression and abnormal signals.\nRoutine blood tests, as well as human immunodeficiency virus (HIV), syphilis, and thyroid function tests, were unremarkable, and his serum vitamin B12 level was borderline (289 picogram per milliliter). A cerebral spinal fluid sample revealed a mildly elevated protein level (120 mg per deciliter) with a normal cell count (two cells per milliliter) and glucose level (79 mg per deciliter). The sample was negative for cytology, oligoclonal bands, culture, and Epstein–Barr virus DNA polymerase chain reaction (PCR). His urine toxicology panel was negative for common recreational drugs.\nThe initial impression was an acquired demyelinating white matter disease due to nutritional deficiency. A trial vitamin supplement (500 mg administered intravenously three times a day and 1000 μg of cyanocobalamin administered intramuscularly daily) was given based on his alcohol abuse history, but no symptom improvements were seen during the following week. An inherited condition was thus suspected given the negative workup, the non-response to treatment, and the symmetric involvement in the brain MRI. This suspicion was validated by his peroxisomal fatty acid profile (Table ), and a diagnosis of X-ALD was confirmed according to the identification of a pathogenic mutation in the ABCD1 gene: c.1489 2A>G (g.153005544). Adrenocortical insufficiency was not identified during the evaluation. No seizure activity was witnessed or shown on video-electroencephalography.\nHis neurological condition deteriorated rapidly. He progressed into a non-verbal, quadriplegic patient dependent on a ventilator within 5 months of the initial encounter, and after 1 year of follow up he had degenerated into a vegetative state. Genetic counseling was offered to his family, and all of his siblings were negative for the ABCD1 gene mutation.
A 10-year-old girl attended with her father after she was referred to Paediatric Dentistry Clinic for diagnosis and treatment. The main chief complaint was the discoloured front teeth. Furthermore, bullying at school was occurring due to her teeth appearance. In addition, general teeth sensitivity was affecting the upper central incisor, especially during brushing and temperature change. A detailed dental and medical history was obtained from the child as well as the parents. Dental family history revealed that the relatives did not suffer from similar dental conditions. However, the patient was born prematurely at 8 weeks, and she was in an incubator for the first 8 weeks of birth. This was followed by a good recovery leading to not requiring any medication or hospitalisation during her first few years after her birth. Upon intraoral examination, we noted a mixed dentition stage, with partially erupted teeth, and caries-free primary and permanent teeth (except for the mobile upper right first primary molar). Regarding the occlusion, we noted a class II division one incisor relationship, skeletal class II relation with increased vertical proportion, midline shift to the left side, and an 8 mm anterior open bite, and the child was occluding solely on the left first permanent molar. The hypoplastic phenotype was affecting the enamel of both dentitions (more prominent in permanent dentition) with demarcated yellowish-brown discolouration at the incisal third of lower incisors, upper central incisors, first molars, and primary teeth. The features obtained from both clinical and radiographic examination were consistent with a possible diagnosis of hypoplastic AI (Figures –). A dental panoramic tomogram (DPT) taken showed all permanent teeth and hypoplastic defects affecting the teeth, including the unerupted second molars ().\nThe treatment planning was based on preserving the remaining tooth structure until the patient can reach the age when she can receive a definitive treatment, keeping in mind the need to relieve the symptoms including sensitivity, improve the occlusion, and aesthetic demands. Following discussion with the child and parents, it was agreed to use a noninvasive approach with no tooth structure removal. The treatment plan was divided into a prevention, restorative, and maintenance phase. In the prevention phase, enforcement of oral hygiene instructions was done at every visit. Sodium fluoride varnish 22,600 ppm (Duraphat, Colgate, USA) was applied to reduce dental sensitivity for three consecutive weeks to reduce teeth sensitivity, protect and prevent remaining structures from future loss, and, at the same time, to avoid caries development [, ].\nDuring the planning visit, orthodontic consultation was obtained. Due to the skeletal open bite tendency of the patient combined with class II and the increased vertical proportion, the orthodontic treatment may include skeletal anchorage device or orthognathic surgery. It was recommended to delay the orthodontic treatment and reevaluate the case after one year, so a referral to Orthodontic department for periodic follow-up until treatment is initiated was done.\nRestorative treatment was done for the permanent incisors and first molars. A direct composite veneer was performed to the upper and lower incisors: following the manufacturer's instruction, an adhesive system was applied (Optibond Solo plus, single component, Kerr Co., CA, USA). The enamel shade was applied in the cervical region, and shade A2 and shade A1 were used to the middle and cervical thirds (GRADIA® Anterior, GC corporation, USA) Finishing and polishing procedures of the restorations were performed using sequential Sof-Lex discs (3M ESPE, Seefeld, Germany). Regarding the first molars, the upper right and lower left were affected more by posteruptive breakdown when compared to the other two molars. As a result, a preformed metal crown (stainless steel crowns; 3MTM ESPETM, St Paul, MN, USA) was done and cemented using glass ionomer cement (Aquacem®; Dentsply, Milford, DE, USA). The other two molars were restored by composite, as the remaining enamel was sufficient to perform a retentive filling (Figures and ).\nFollow-up was done for 15 months. The treatment showed a maintained tooth structure, good oral hygiene, and eruption of premolars ().
A 60-year-old gentleman who was previously diagnosed with type 2 diabetes mellitus, hypertension, and a cerebrovascular accident, which he suffered two years prior to presentation, which left him with residual right-sided upper and lower limbs weakness, for which he is undergoing physiotherapy with continuous improvement, presented with a history of right eye sudden painless drop in the vision of one-day duration. He denied any history of trauma to the eye or head and gave no history of previous eye symptoms or surgeries. Apart from the right-sided upper and lower limb weakness, his general and systemic examination was unremarkable.\nHis ophthalmic examination showed visual acuity of hand movement in the right eye and 6/6 in the left eye, with normal intraocular pressure in both eyes (18 mmHg in the right and 12 mmHg in the left). Slit-lamp biomicroscopy showed grossly normal anterior segments of both eyes with immature cataracts. Fundus examination of the right eye by binocular indirect ophthalmoscopy showed no view of the retina due to a grade 4 vitreous hemorrhage, while fundus examination of the left eye was unremarkable.\nB-scan ultrasonography of the right eye showed a large mushroom-shaped mass invading the choroid and retina, and protruding into the vitreous cavity in the inferior aspect of the posterior pole (Figure ) with a shallow exudative retinal detachment observed in the peripheral rim of the mass, with dense vitreous hemorrhage (Figure ).\nMagnetic resonance imaging (MRI) of the orbits was also done and showed a small mass suggestive of a choroidal melanoma along the posterior aspect of the right globe extending into the vitreous and measuring 8 x 6 mm. The lesion appears of high signal intensity on T1-weighted images and low intensity on T2-weighted images and shows mild contrast enhancement in the post-contrast images. The vitreous body shows an abnormal V-shaped appearance with abnormal high signal intensity on T2-weighted images mostly suggestive of vitreous hemorrhage with posterior vitreous detachment (Figures -).\nAdditional testing included positron emission tomography (PET) scan, chest X-ray, and laboratory studies, which included complete blood count (CBC), serum electrolytes, and liver function test (LFT), which were all within normal limits, excluding the possibility of metastasis to the lungs and liver, and confirming that the choroidal mass is a localized primary ocular mass.\nAfter discussing the treatment options with the patient, and due to the unavailability of plaque brachytherapy and the concurrent presence of a vitreous hemorrhage, which would already mandate a pars-plana vitrectomy, a decision was made to book the patient for an early surgical transretinal endoresection via the pars plana route.\nUnder local anesthesia (peribulbar block), routine phacoemulsification complicated by a posterior capsular rent was first done with the implantation of a sulcus intraocular lens. Then, a standard 23-gauge three-port pars plana vitrectomy was performed to clear the vitreous hemorrhage, after which a mushroom-shaped mass measuring approximately 6 disc diameter in size (Figure ) was seen protruding into the vitreous cavity inferior to the fovea (the base of which was 1 disc diameter inferior to the inferior vascular arcade). Micro scissors were used to obtain two biopsy specimens from the mass. After that, complete tumor transretinal endoresection using the vitrector was performed, during which endodiathermy was used frequently to stop bleeding from the mass to ensure adequate visibility at all times. Perfluorocarbon liquid was injected for foveal protection, drainage of subretinal fluid, and stabilization of the retina, while the edges of the resultant retinotomy were carefully cauterized using endodiathermy (Figure ). This was followed by argon laser photocoagulation peripheral to the endodiathermy marks, placing three rows of laser photocoagulation around the retinotomy (Figure ). Finally perfluorocarbon liquid/air exchange was performed and 1000cs silicone oil was injected as a tamponade.\nBoth solid biopsy specimens and diluted vitreous samples from the aspirated fluid were sent to the lab for histopathological examination. However, the pathologist report showed that there was not enough sample for adequate histopathological grading, with mainly melanocytes in the slide.\nAt one week post-operation, the best-corrected visual acuity of the right eye had improved to 6/36 with remaining anterior uveitis and fundus examination showed a flat retina with white laser marks around the retinotomy. At one month post-operative, the visual acuity had further improved to 6/9 with a flat retina and silicone oil tamponade in situ. Optical coherence tomography (OCT) of the macula (Figure ) and fundus photography (Figure ) at one month post-operative are shown below.\nPost-operative MRI, which was done at one week post-operative to ensure complete resection, showed no evidence of residual tumor, and complete resolution of the exudative retinal detachment was seen pre-operatively, with a visible silicone oil bubble in the globe (Figure ).\nThe patient was also referred to the oncology department to continue monitoring tumor control in liaison with the ophthalmology department with regular follow-up appointments, and he will be posted for silicone oil removal after six to nine months.
A 38-year-old Brazilian man presented with a 4-day history of chest pain radiating to his abdomen and back. He was diagnosed with an extensive Stanford type B aortic dissection originating at the base of left subclavian artery extending into the iliac arteries. With the exception of the left renal artery, visceral branches were supplied by the true lumen. He was managed conservatively with blood pressure control and discharged home. On computed tomography (CT) aortogram 8 weeks later, the dissection appeared stable, however, the diameter of the distal thoracic descending aorta had increased to 64 from 59 mm. His arch was deemed unsuitable for stent grafting due to an acute angle (60-degrees), resulting in a Gothic arch; and he was planned for open surgical repair.\nThree months later, the patient represented with severe left sided chest pain. A CT aortogram demonstrated fusiform dilatation of the distal arch and aorta measuring 73 × 69 mm () with interval false lumen dilation. The decision was made to proceed with an open thoracoabdominal aortic aneurysm (TAAA) repair utilising cardiopulmonary bypass with deep hypothermic circulatory arrest. A pre-operative cerebrospinal drain was placed to decrease the risk of spinal ischaemia. The patient was positioned in left lateral position with a thoracoabdominal incision made through the fifth intercostal space. Retroperitoneal dissection revealed vessels of good calibre and an extensive TAAA (). Peripheral cardiopulmonary bypass was instituted via the left femoral artery and vein utilising an 8 mm Dacron graft, and a 25Fr venous cannula. The patient was cooled to 18 degrees Celsius and placed in Trendelenburg positioning for clamping of the mid-thoracic aorta.\nThe aneurysm was incised and the dissection flap unroofed with a 2 cm cuff created for the proximal anastomosis using a 26 mm Dacron graft. Arterial inflow was established via an 8 mm sidearm, with clamping of the femoral line, deairing of the cerebral circulation and rewarming of the patient. The Dacron graft was then clamped proximal to the arterial inflow and femoral bypass reinstated with the clamp moved to above the diaphragm. Multiple small intercostal arteries were oversewn, with two large intercostals at T10 controlled with Fogarty catheters and anastomosed utilising separate 8 mm Dacron grafts. The cross-clamp was moved to above the renal arteries, allowing a Carrel patch containing the coeliac trunk and superior mesenteric arteries to be fashioned. Continuous perfusion to the superior mesenteric artery was provided via a 12Fr cannula. The femoral inflow was clamped, and the abdominal aortic clamp removed, with fenestration of the aorta to ensure the left femoral artery was supplied by the true lumen. A size 28 mm Dacron graft was anastomosed to the suprarenal aorta, a clamp placed superior to the suture line, and femoral inflow restored. The Carrel patch containing mesenteric vessels was anastomosed to the anterior aspect of the graft.\nThe graft was positioned through the aortic opening in the diaphragm and the 8 mm Dacron graft supplying the intercostals anastomosed towards the posterolateral. The 26 and 28 mm Dacron grafts were anastomosed in the mid-thoracic aorta with deairing and clamping of femoral inflow. The patient was weaned from bypass without difficulty and the venous line removed with repair of the femoral vein. The femoral and thoracic 8 mm Dacron inflow grafts were ligated and divided, with repair of the diaphragm and costal margins undertaken. The bypass time was 312 minutes and upper body circulatory arrest time 39 minutes. He was transferred to intensive care in a stable condition. His immediate postoperative course was complicated by vasoplegia, requiring noradrenaline, and coagulopathy requiring massive transfusion.\nThe patient became febrile on day 6 postoperatively and was diagnosed with ventilator-acquired pneumonia and commenced on tazocin and vancomycin. He remained febrile, with surgical wound dehiscence and escalation of his antibiotics to meropenem and vancomycin. Despite adequate treatment of his pneumonia and wound dehiscence with antibiotics and surgical debridement, 3 weeks later the patient had persistent tachycardia to 180 bpm without haemodynamic compromise. Transthoracic echocardiography revealed moderate global impairment with trivial pericardial effusion. Postural telemetry () demonstrated a sitting heart rate of 101 bpm that increased to 144 bpm on standing and 164 bpm on mobilisation without incrementation of the blood pressure (110/54 mmHg sitting, 100/67 mmHg standing), consistent with POTS. The patient was commenced on metoprolol, fludrocortisone and ivabradine with improvement in tachycardia. He received multiple blood transfusions with increase in haemoglobin from 90 to 110 g/L. He was discharged home 6 weeks after his operation with no disability.
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 52-year-old man, with a known history of osteopetrosis and previous fractures in his bilateral femurs and right tibia, was referred for treatment of a right subtrochanteric femur fracture in January 2018. His medical history is summarized in Table , which shows that the patient suffered from chronic osteomyelitis of tibia after traction treatment of tibial tubercle. A pedigree chart was drawn according to his family history (Fig. ). In the chart, III-2 is the patient, and his previous generation, sister, cousin and his wife had no similar medical history, whereas two of his brothers suffered from osteopetrosis, and one of them presented with osteomyelitis of the mandible. Additionally, his daughter was healthy, but his niece suffered from osteopetrosis. When admitted to the hospital, he presented with severe functional limitation of the right hip and pain, and his radiograph (Fig. ) showed that the lateral transverse fracture of the right femur was mildly displaced with a dense sclerotic line. In addition, the bone cortical density of the pelvis and the bilateral femur increased, and the proximal regions of bilateral femurs were deformed, with rough and irregular bone cortex, as well as narrowed medullary cavity, and signs of "sandwich vertebrae" were seen on the lower lumbar vertebrae. Additionally, laboratory testing showed mild anemia and slight hypocalcemia. According to family history, we judged the genetic pattern of the patient to reflect autosomal recessive inheritance. Together, his condition was diagnosed as IRO, accompanied by chronic osteomyelitis, mild anemia and hypocalcemia.\nBefore the operation, we evaluated the patient's bone condition and decided to treat the fracture using internal fixation with DHS. Importantly, we made sufficient preparation for the operation to deal with potential adverse events, such as drill bit fracture and bone necrosis. During the operation, we exposed the fracture site and found that the end presented a solid white amorphous appearance. As speculated, no medullary cavity existed in the femur. Two drill bits were used to create holes before inserting the screws with extreme care so as not to shatter the femoral shaft, and we chose a short hip screw with a length of 65 mm. The femur was resistant to drilling and it was done slowly under constant irrigation with ice-cold saline and with repeated cleaning of the drill bit. Eventually, we took triple longer duration than usual to complete this operation. X-ray images of our patient's pelvis, right hip, and right femur were taken 1 week after the operation (Fig. a) and showed satisfactory alignment of the fracture had occurred. Unfortunately, the right femoral internal fixation screws fractured (Fig. b), caused by a careless sprain 3 months after the operation. After re-admission, we performed a repeat of the DHS internal fixation for postoperative screw fracture in a right subtrochanteric fracture, and postoperative X-ray examination showed that the fracture was aligned (Fig. c).\nDrawing a lesson from the previous failure of internal fixation, a double hip herringbone brace was fixed for 3 months to prevent the internal fixation from breaking again. Our patient returned for follow-up 3 months after the revision, at which time the radiograph still failed to demonstrate any healing progression (Fig. a). A blurred fracture line and callus formation were observed at 6 months after the revision (Fig. b). However, good evidence of callus formation and fracture healing was demonstrated by the radiograph at 12 months after the revision surgery (Fig. c). At 15-months follow-up, there were no clinical signs of infection, and laboratory tests remained within the normal range, except for a minor abatement in hemoglobin and serum calcium. At present, the patient displays stable internal fixation, with no limitation of activities, and is pain-free.
A 42-year-old female presenting with headaches was found to have a 5 mm laterally projecting right middle cerebral artery (MCA) bifurcation aneurysm and a 5 mm BX aneurysm []. Given the patient’s young age, surgical treatment was offered as an alternative to endovascular treatment. After discussion of the risks and benefits, the patient opted for a minimally invasive supraorbital keyhole craniotomy for clipping of both aneurysms.\nPreoperative computed tomography angiography (CTA) evaluation of the patient’s aneurysms revealed aneurysms amenable for surgical clipping through a supraorbital keyhole approach (SOKA). The MCA bifurcation aneurysm was noted to be at the level of the lesser wing of the sphenoid bone and evaluation of the anatomical accessibility of the BX aneurysm was determined using the orbital roof-dorsum estimation line as previously described.[] Briefly, the elevation of the orbital roof off the anterior skull base is determined in the coronal plane which is then translated on to the midsagittal plane; this point is then extended to the top of the posterior clinoid and continuing until it reaches the basilar artery (BA) determining the most superior exposure of the BA. Utilizing this line, it was estimated that the superior most exposure through the SOKA would be the neck of the aneurysm with further inferior exposure afforded by the superior extent of the craniotomy off the roof of the orbit [].\nThe patient was positioned supine and the head was placed in three-point fixation. A right-sided approximately 5 cm eyebrow incision was made extending from the supraorbital notch to just lateral to the superior temporal line []. A 2 cm × 1 cm craniotomy was made above the orbital rim. A curvilinear dural incision was made and the frontal lobe gently dissected off the anterior skull base without the use of fixed retractors. The opticocarotid cistern was opened to release cerebrospinal fluid (CSF) and facilitate brain relaxation. The Sylvian fissure was then dissected to expose the MCA bifurcation aneurysm. A right-angled clip was applied and placement was confirmed with mICG-VA using a 0.5 mg/kg bolus of ICG []. Attention was then turned to the BX aneurysm. Arachnoid dissection around the optic nerve was performed to allow unencumbered access into the opticocarotid and carotidoculomotor triangles. Through the carotidoculomotor triangle, Liliequist’s membrane was opened until the aneurysm dome was identified. The aneurysm dome and neck were visible; however, the more proximal BA and perforator origins were obscured by the posterior clinoid. A 0 degree, 4 mm outer diameter, ICG capable endoscope (Storz; Tuttlingen, Germany) was introduced through the opticocarotid window (OCW) allowing complete visualization of the more proximal BA and perforator origins. Another bolus of ICG was administered and the aneurysm was inspected with eICG-VA. A posterior clinoidectomy was considered but ultimately deemed unnecessary given the improved visualization. Adenosine was available, if necessary, for intraoperative rupture. After careful dissection of the perforators off the aneurysm neck, a straight blade aneurysm clip was placed under a microscopic view. Postclipping inspection of the aneurysm utilizing eICG-VA demonstrated good exclusion of the aneurysm with no neck remnant and intact parent and perforator vessels [].\nPostoperative CTA confirmed adequate placement of clips with no residual []. Patient recovered well and was discharged on a postoperative day 2 with no short- or long-term complications or neurologic deficits at 3-month follow-up.
A 55-year-old man of Indian ethnicity, presented to the outpatient department of general surgery with lower abdominal distension for the past one and a half months and a continuous dull aching lower abdominal pain for past 15 days. Patient was a chronic smoker and gave no history of trauma, anorexia, weight loss, altered bowel habits, vomiting, fever, or any urinary symptoms such as hematuria. On examination, patient was moderately built, and abdominal fullness was apparent on inspection, more so in the left lower abdomen. A vague mass of approximate size of 20 × 15 cm was palpable, extending from left iliac and left hypogastrium to umbilical region, crossing the midline and reaching up to right midclavicular line at the level of iliac crest. The lump had a smooth surface, ill-defined margins and lower margin could not be reached on palpation. The lump was nontender with firm, cystic consistency, and restricted mobility in all the directions. There was no inguinal lymphadenopathy and digital rectal examination was unremarkable. Patient was further investigated for differential diagnoses of mesenteric cyst, pancreatic pseudocyst, duplication cyst, or cystic metastasis. Ultrasound abdomen showed a large cystic lesion with multiple septae, left to the urinary bladder in the pelvis. Liver, spleen, pancreas, and bilateral kidneys were within normal limits. A contrast enhanced computed tomography (CECT) scan of abdomen and pelvis showed a large 14.4 × 14.6 × 9.2 cm multilocular cystic mass with enhancing septations and lobulation, occupying the left side of abdominal cavity extending downwards into the pelvis and displacing the gut loops and the urinary bladder to the right side. Mass was encasing the left iliac artery and there were no enlarged lymph nodes in paraaortic and pelvic region. The attenuation coefficient of the mass was in the +10 to 15 HU range. There were no ascites and no evidence of small or large bowel obstruction ().\nA fine needle aspiration cytology of the mass showed RBCs, neutrophils, and lymphocytes in a proteinaceous background, entertaining the diagnosis of lymphangioma. A provisional diagnosis of retroperitoneal lymphangioma/mesenteric cyst was made and the patient underwent an exploratory laparotomy. Upon laparotomy, a large (15 × 15 cm), retroperitoneal multicystic mass was found, which was encasing the left common iliac bifurcation, left iliac vessels, left ureter, left gonadal vessels, and left vas deferens. The mass was pushing the urinary bladder towards right side. There was a plane of dissection among the vessels, ureter, urinary bladder, and the cyst, which was dissected free completely using sharp and blunt dissection. It was filled with approximately 1000 cc of thick, cloudy fluid. Frozen-section study of a portion of wall of the cyst was suggestive of lymphangioma. After removing the cyst completely, the retroperitoneum and the mesentery were inspected closely for any remnants. The bowel was inspected twice and was found to be healthy, viable, and without any evidence of involvement. The patient tolerated the procedure well and his postoperative course was uneventful.\nGross examination of the specimen, showed a mass of glistening white tissue and serial sectioning revealed multiple cysts filled with cloudy fluid.\nHistologically, the mass was composed of variable sized cystic spaces lined by flattened endothelial cells, which were positive for D2-40 immunostains, consistent with features of lymphatic vessels (Figures and ). The larger spaces had fascicles of smooth muscle, and nearly all of them were filled with pale pink proteinaceous material. Small lymphoid aggregates were present. The stroma showed acute on chronic inflammation, edema and fibrosis. Thus, the diagnosis of a retroperitoneal multilocular cystic lymphangioma was histologically confirmed. The patient has been on regular follow-up over the last one year and there has been no evidence of recurrence, clinically as well as on imaging.
A 46-year-old white female with a 15 pack-year smoking history initially presented 3 years ago with intense pain in the left side of the mouth that radiated to her left ear for 2 months. She was found to have a 2 cm exophytic lesion on the left lateral border of her tongue that was diffusely keratotic and extremely tender on examination. Fiberoptic laryngoscopy revealed normal findings in the nasopharynx, oropharynx, and hypopharynx. Histological biopsy of the tongue lesion confirmed well-differentiated squamous cell carcinoma of the left lateral border of the tongue. Radiographical findings on positron emission tomography (PET) scan showed hyperactivity along the left lateral aspect of the tongue and a mildly hypermetabolic left level IIa cervical lymph node with no evidence of distant metastases ().\nThe patient was treated with a left hemiglossectomy and bilateral neck dissection. Pathologic evaluation revealed a 2.4 cm moderately differentiated, infiltrating squamous cell carcinoma of the left lateral tongue lesion invading into the skeletal muscle with a 0.5 cm maximal thickness. There was perineural invasion, but no lymphovascular invasion, and all margins were free of cancer. A total of 3 out of 22 lymph nodes were positive for carcinoma: 0 out of 10 in right neck level II-III, 2 out of 5 left level I with no extracapsular extension (ECE), 0 out of 1 left level II, and 1 out of 6 left level III with no ECE.\nShe was staged as pT2 pN2b M0 (stage IVA, AJCC 7th edition 2010) squamous cell carcinoma of the left lateral tongue. She received adjuvant treatment with concurrent afatinib and radiotherapy to a total dose of 6000 cGy in 30 fractions over 6 weeks to the oral cavity and bilateral necks, which was completed in 3 months after diagnosis.\nInterval radiographical imaging did not show any evidence of disease recurrence or distant metastases until 2018. At that time, the patient had developed increasing left arm pain, left ear pain, and left throat pain. She also reported intermittent chest pressure, exertional dyspnea, and intermittent dizziness with positional changes.\nIn early 2018, a PET scan () and magnetic resonance imaging (MRI) of the chest () showed a new 3.4 cm left apical pleural mass encasing the left subclavian artery and abutting the left subclavian vein, both of which were patent. A computed tomography-guided fine-needle aspiration (CT-FNA) of the apical lung mass revealed squamous cell carcinoma.\nShortly after, the patient began systemic therapy with cisplatin and etoposide and radiation therapy to the left apical lung lesion. Following the 20th fraction of radiation therapy, a repeat computed tomography (CT) scan of the chest revealed a new 1.2 cm lesion in the inferior interventricular septum of the heart. A cardiac ultrasound was performed and demonstrated a mass in the left ventricle. The patient went on to complete radiation therapy to a total of 6000 cGy in 30 fractions. Further diagnostic imaging with a cardiac MRI was performed, which revealed a mass infiltrating the left ventricle, inferior myocardium, epicardial fat, and pericardium with associated mobile thrombus formation (). A PET/CT scan demonstrated hypermetabolic lesions in the left neck, right thigh muscles, lung parenchyma, heart, anterior mediastinum, left scapula, and posterior right rib (). Transthoracic echocardiogram (TTE) showed a 1.6 × 1.4 cm mobile mass in the left ventricle cavity that appeared to be attached to the base of the papillary muscle and a normal left ventricular ejection fraction of 60%. An electrocardiogram (ECG) revealed normal sinus rhythm with T-wave inversion in the inferior leads and V3–V6.\nThe patient initiated anticoagulation and systemic therapy with nivolumab.
A 45-year-old male motorcyclist with a history of hypertension, hyperlipidemia, and coronary artery disease was brought to the emergency department after being struck by another car on the highway at speeds of at least 40 miles per hour. Upon presentation, the patient was evaluated using Advanced Trauma Life Support (ATLS) principles. He had a patent airway on arrival and was breathing spontaneously on room air. His initial heart rate was 87 beats per minute, and his blood pressure was 124/63 mmHg without signs of significant hemorrhage. He had an initial Glasgow coma score (GCS) of 15 with equal and reactive pupils. The patient admitted to consuming alcohol and had a serum alcohol of 243 mg/dL. A later CT of the head demonstrated a subcutaneous hematoma without any intracranial abnormalities. His remaining physical examination revealed left lower quadrant abdominal pain without signs of peritonitis, ankle deformities bilaterally, pain with hip range of motion, and blood at the urethral meatus. Given his physical examination findings, subsequent imaging confirmed an unstable pelvic fracture with diastasis of the symphysis pubis of 6 cm, widening of the left sacroiliac joint, a left ischial pubic ramus fracture, and a urethral injury (). He also had a left ankle dislocation and a right compound fracture of the distal tibia and fibula. No intraabdominal injuries were identified on CT imaging of the abdomen. The pelvis was stabilized with a binder by the orthopedic surgeons with subsequent emergency irrigation, debridement, and open reduction and internal fixation (ORIF) of the open ankle fracture as well as reduction of the left ankle dislocation. He was extubated after the procedure and monitored in the ICU while the remaining preoperative medical workup was completed including X-rays and CT scans with 3D reconstructions of the pelvis reconstructions. A hydromorphone patient-controlled analgesia (PCA) pump was utilized for pain control.\nOn hospital day 2, the patient was deemed fit for surgery and was taken to the operating theater for a combined operation by the orthopedic surgeons for ORIF of the pubic diastasis, sacral fracture, and sacroiliac joint followed by the trauma surgeons to reconstruct the abdominal wall and inguinal canal. The trauma team performed the exposure of the pubic symphyseal region and the pubic diastasis. A Pfannenstiel incision was made, and the planes were dissected exposing the left spermatic cord. The orthopedic team then performed a gentle open reduction of the pubic diastasis taking care to ensure that the bladder and urethra were not incarcerated. The Asnis III cannulated screw system and a Matta pubic symphyseal plate (Stryker GmbH, Switzerland) were utilized under C-arm fluoroscopic guidance with appropriate alignment of the AP and inlet and outlet pelvis views. Once the Mata plate was in place and the orthopedic reduction was completed, we proceeded to reconstruct the anterior abdominal wall. Since the Cooper ligament was destroyed, it was dissected to allow direct visualization of the pubic rami. The abdominal wall defect was measured to be 10 × 12 cm. We then used a modified Stoppa technique by placing the 6 × 6 in Prolene mesh under the damaged internal inguinal ring, making sure the spermatic cord on the left side was not injured or pinched, securing it in place using sutures, including direct suturing to the periosteum of the repaired pubic symphysis and the plate as needed. The medial borders of the mesh were tucked inside the opened rectus sheath on the right side and secured laterally with fires of a 5 mm Covidien Endotack (Medtronic, MN, USA) to the remnants of the conjoint ligament. The midline was then repaired with sutures, including the mesh as reinforcement. The patient did well postoperatively with postreduction films demonstrating appropriate alignment (). He was discharged to rehab on postoperative day 5. There were no recurrences during the follow-up period of 10 years.
A 52-year-old male, chronic alcoholic and smoker presented to the emergency department with acute abdominal pain. The serum amylase level was elevated and a diagnosis of acute pancreatitis was made. The patient was managed conservatively and was satisfactorily discharged after a two week-stay in hospital. The patient developed abdominal pain again after 2 months and presented to the outpatient department at another institute where a diagnosis of acute on chronic pancreatitis was made. The patient was referred and when he presented to us he was ambulatory and complained primarily of vague upper abdominal pain with no signs of peritonitis. He also complained of occasional episodes of vomiting and weight loss. He was carrying a report of an endoscopic ultrasound (EUS) done elsewhere, films of which were not available. The EUS report showed a 3×3 cm pseudocyst in the region of neck of pancreas with possible communication with dilated main pancreatic duct (MPD). The pseudocyst was compressing the distal common bile duct (CBD) causing moderate biliary dilation. At our institution, he was further evaluated with a transabdominal USG and a contrast enhanced CT (CECT) to look for the severity and extent of disease. The USG () showed the portal vein as anechoic but with no color flow within and also showed communication between the cyst and portal vein. The CECT showed the pseudocyst () in the pancreatic neck and completely thrombosed portal vein and its branches () which was misinterpreted as dilated CBD on EUS. There were multiple periportal collaterals and areas of necrosis in segment V and VII of liver which showed subtle peripheral enhancement and were considered as cholangiolar abscesses. There was suggestion of communication of the pseudocyst with the MPD.\nMRI was performed to investigate a possible communication of the pseudocyst with the MPD and to further characterize the liver lesions. It showed the pseudocyst at the neck of the pancreas with communication with the dilated MPD. The cyst was seen just above the spleno-portal confluence with a defect in the posterior wall and a possible direct communication with the portal vein. The portal vein showed a signal characteristic consistent with fluid i.e. homogenously hyperintense on T2-weighted imaging and hypointense on T1-weighted imaging. There was some plaque-like material along the wall of the main portal vein and its branches which showed hyperintense signal on T1-weighted imaging and hypointense on T2-weighted imaging (, ). On post contrast images there was no opacification of the portal vein (). These features did not correlate with the expected signal character of the thrombus but rather confirmed the presence of fluid within the portal vein similar to that in the pseudocyst with the plaque-like material along the wall showing the character of residual lysed thrombus. There were non-enhancing areas of necrosis in segments V and VII of the liver and thrombus in the distal part of the superior mesenteric vein and splenic vein. The CBD was normal in caliber and there was no intrahepatic biliary dilation. Thus a diagnosis of rupture of pancreatic pseudocyst into the portal vein with lyses of the portal vein thrombus and associated liver necrosis was made. The MRI findings suggested this diagnosis thus obviating the need of any invasive diagnostic modality. Since the main complaint of the patient was generalized abdominal pain and distention which was well controlled with regular medication, a decision to continue with conservative treatment was taken and the patient was kept on close follow up and review. The patient did well thereafter with no significant increase or development of complaints on 6-month follow up. The patient is still on regular follow up and doing well.
A 20-year-old pregnant mother with a period of amenorrhea (POA) of 28 weeks was admitted to our tertiary care centre due to left-sided severe buttock pain, five days prior to admission. The pain was very severe and she could not even walk due to the pain. She has taken paracetamol as over-the counter-medication and had no satisfactory response. She had a recent history of DHF, which we treated with appropriate fluid management and supportive care, and was discharged eight days before after complete recovery. Initially, she was admitted to a local hospital due to buttock pain for which paracetamol was given as a painkiller, with minimal response. Due to the short duration of her condition and unavailability of facilities, no further investigations were conducted during the local hospital admission. Following her discharge, she experienced the same kind of pain again, after which she was admitted to our tertiary care centre. She did not have other small or large joint pain, swelling and there were no features to suggest enthesitis. She did not have fever, dysuria, vaginal discharge, or skin eruptions. There was no recent history of sore throat or diarrheal illnesses. She also had no past history of joint pain, photo-sensitive skin rashes, oral ulcers, or alopecia. There was no past history of altered bowel habits or contact history of tuberculosis.\nOn general examination, she was not pale or icteric. There was no red eye, malar rash, oral ulcers, or any peripheral stigmata to suggest ongoing vasculitides. Respiratory system examination was normal and did not reveal any features to suggest lung fibrosis. Cardiovascular system examination revealed mild tachycardia (110 beats per minute) and normal blood pressure. There was no evidence of aortic regurgitation or mitral valve prolapsed. Abdomen was non-tender and had no hepato-splenomegaly. The nervous system and peripheral joint examination were normal. X-ray of her sacroiliac joint revealed features of left-sided sacroiliitis which was evidenced by blurring of joint margins with minimal sclerosis of the left sacroiliac joint (see X-rays in Figures -). Full blood count (FBC) revealed white blood cells 14000/µL of which 77% were neutrophils. Hemoglobin concentration was 10 g/dl and platelets 553000/µL. Erythrocyte sedimentation rate (ESR) was 103mm/1st hour and CRP was 9 mg/dl. Dengue IgM and IgG both were positive. Urine full report, liver function tests, renal function tests, and uric acid were within normal range. Blood and urine culture both were sterile. Tuberculin skin test was negative. Antinuclear antibody (ANA) and rheumatoid factor were negative. Retroviral, Hepatitis B and C screenings were negative. Human leukocyte antigen (HLA B27) was negative. Thyroid function tests and iron studies all were within normal limits. We managed her with regular paracetamol and oral tramadol appropriately to alleviate her pain and she gradually improved over the next week. We reviewed her after two weeks with FBC and ESR. At her two-week review, the white blood cell count was down to 12200/µL and platelet count was 365000/µL. Her ESR report was 43 mm/1st hour. After two weeks, significant clinical and biochemical improvement was observed following appropriate symptomatic management.
A 71-year old, female patient with a previous history of non-Hodgkin lymphoma and transient ischemic attack came to the emergency room of our University Hospital for sudden onset of right hemifacial paraesthesia, edema of the lower lip (Fig. ) and accentuation of an already present tinnitus. The current presentation had been preceded by a few blisters similar to those usually observed in herpes labialis, and no aphthous ulcer was detected on mouth inspection. Background therapy included aspirin and betahistine, with no personal and family history of adverse drug reactions, atopy, contact dermatitis, urticaria, angioedema, cranial nerve palsy, granulomatous or inflammatory diseases. After symptomatic treatment by intravenous steroids and antihistamines, the patient was discharged with prescription of a short-course therapy with oral prednisone and cetirizine []. This resulted in partial remission of symptoms, but 1 week later the patient was readmitted to ER for symptom recurrence and worsening of lip edema without detectable oral cavity and tongue alterations. Due to the apparent involvement of the 5th cranial nerve, a varicella-zoster virus (VZV) infection was hypothesized and therapy with valaciclovir initiated. On occasion, a blood sample was drawn showing evidence of anti-VZV IgG with undetectable IgM. One month later, on further admission at the ER for the same clinical picture associated with swelling over the left zygomatic region, an angioedema of unknown origin was suspected. Thus, a course of twice daily dose of 10 mg cetirizine was prescribed [–]. However, this approach was ineffective and also the subsequent replacement of aspirin with clopidrogel and temporary withdrawal of betahistine resulted in no improvement. IgM and eosinophil count, as well as plasma levels of angiotensin converting enzyme were in the normal range, thus helping to exclude the hypothesis of Gleich syndrome or sarcoidosis. Patch testing for dental materials was also negative, and complement screening was then performed with evidence of normal levels of circulating C1q (143 mg/L) and both antigen (302 mg/L) and functional (109%) C1-Inhibitor (C1-INH). On the contrary, C4 was low (0.03–0.04 g/L; NR 0.09–0.36 g/L) and C3 fluctuated around the lowest levels of the referral range (0.93–0.82 g/L; NR 0.9–1.8 g/L) on repeated assessments. These findings ruled out the possibility of acquired AE due C1-INH deficiency [–], prompting us to explore the (auto)immune-inflammatory state: antineutrophil cytoplasmic antibody tested negative, whilst low titer (1:160) anti-nuclear antibodies (ANA) were found along with antiphospholipid antibodies (lupus anticoagulants; anti-cardiolipin, anti-β2-glycoprotein IgM), possibly related to complement consumption [].\nAs ultrasound scan detected only a subcutaneous, hypoechogenic thickening of the inferior lip and we did not find any further clinical or laboratory sign of systemic inflammation, recurrent swelling was interpreted as a form of idiopathic angioedema and treated with tranexamic acid after a thrombophilia screen testing negative for further risk factors [, ]. Both this antifibrinolytic drug and a following, therapeutic course with the leukotriene receptor antagonist montelukast [] failed to solve the edema. Finally, clinical picture, blood analyses, and lack of response to any of the previous therapies suggested the possibility of Melkersson–Rosenthal syndrome []. Thus, the patient was referred to the Dental Clinic, where a mucosal biopsy of the affected lower lip was performed (Fig. ). Histopathological examination showed non caseating granulomas (Miescher’s cheilitis), consistent with a diagnosis of MRS. The two aggregates of non-caseating granulomatous inflammation consisted of lymphocytes and epithelioid histiocytes, and few multinucleated giant cells, clustered around scattered vessels (Fig. ). Special staining for identification of fungal microorganisms and acid-fast bacteria were negative. No foreign material could be detected even at polarization. One month of oral steroid (prednisone, 25 mg qd, gradually tapered to 5 mg) resulted in remission of lip swelling but not in definitive recovery. However, since then patient’s perception of both symptoms and aesthetic relevance decreased, and at present she undertakes a few-days regimen of prednisone only when feeling a relapse of edema.
A 44-year-old Caucasian man with a diagnosis of Alport's syndrome, established at another centre, and two previous kidney transplants was referred to our renal department in June 2009. Graft function was failing and he was approaching the need to start renal replacement therapy.\nIn 1973, he was investigated for hereditary nephritis and megathrombocytopaenia (platelet count of 7 × 109/L, size not available). He was also diagnosed with bilateral sensorineural deafness. A kidney biopsy was performed at this stage; however, the patient's early clinical reports were not available; hence, there was no record of the biopsy result. It could not be ascertained whether he was diagnosed with any ocular abnormalities. He eventually started haemodialysis via a tunnelled line in 1987 and later that year he received a kidney transplant from his father. This failed in 1999, when he went on to peritoneal dialysis for 16 months. He then received a second kidney transplant from his brother.\nOn presentation to our low-clearance clinic in 2009, liver impairment was noted ( and ). This was longstanding as records from 1985 revealed an alkaline phosphatase (ALP) of 420 U/L and an alanine transaminase (ALT) of around 68–92 U/L. There was a moderate improvement in the transaminase levels on withdrawing azathioprine and simvastatin. His platelet count was 5 × 109/L, and a blood film showed large platelets. Platelet size was not documented as this is not routinely measured in our hospital. A bone marrow examination showed active haematopoiesis and abundant megakaryocytes. Despite several courses of steroids, the thrombocytopaenia did not improve, and a possible diagnosis of steroid-refractory idiopathic thrombocytopaenic purpura (ITP) was considered.\nA year later, he started on peritoneal dialysis; however, this had to be stopped due to recurrent episodes of staphylococcal peritonitis. Haemodialysis was initiated via a tunnelled line. A left radiocephalic arteriovenous fistula was created a few months later, but this was never used due to persistent thrombocytopaenia.\nDuring this period of time, it was revealed that this man's son was similarly affected with deafness, renal impairment and thrombocytopaenia. It was also confirmed that his brother (not the brother who had donated a kidney) and his two sons had a similar phenotype. The brother was also noted to have deranged liver function tests; however, we had no access to this patient's records; hence, we have no further details. This clear male-to-male hereditary transmission and the close association with megathrombocytopaenia made the initial diagnosis of X-linked Alport's syndrome less likely and genetic studies were performed. Sequential analysis revealed that he was heterozygous for a missense mutation in exon 17 of the myosin heavy chain-9 (MYH9) gene on chromosome 22, in the arginine residue at amino acid 702 of the motor domain. Steroid treatment for the presumed ITP was stopped and his family was offered genetic testing. After interrogating other members of the family, it was revealed that our patient's brother and his family were diagnosed with the same genetic mutation in 2005.\nIn the meantime, a deterioration in the man's liver function tests was observed ( and ), together with the development of ascites and encephalopathy. Both the patient and his family denied alcohol intake. A computed tomography scan of his abdomen revealed mild smooth peritoneal thickening in the lower abdomen and posterior pelvis. He is currently being investigated for a possible diagnosis of encapsulating peritoneal sclerosis (EPS).
This 16-year-old male presented to the emergency department with new onset headache and visual changes of 2 days duration. The headache was described as sudden in onset, constant in nature, and bitemporal in location. This was associated with development of vision loss 1 day characterized by darkening of his vision, progressing from the superior to the inferior visual fields that lasted approximately 30 minutes with subsequent persistence of blurry vision. Without resolution of his symptoms, he presented to the emergency department.\nOn questioning, there was no prior history of seizure activity or of precocious puberty, or of genital abnormalities at birth. He had undergone corrective surgery for polydactyly of the left hand at 9 months of age. Polydactyly was also reported in his mother and elder brother.\nHis neurological examination was benign without abnormalities of extraocular movements, pupillary reflexes, facial motor strength, or sensation; he did not have papilledema. His genitourinary examination was normal and appropriate for age. Inspection of the left hand revealed webbing between the 3rd and 4th digits, with a well-healed scar on the ulnar aspect over the 5th metacarpophalangeal joint.\nCT scan of the head without contrast identified a 30 mm mass in the suprasellar region. Follow-up MRI of the brain with and without contrast confirmed this, identifying a nonenhancing, 30 x 27 x 30 mm mass along the midline of the posterior aspect of the suprasellar cistern contiguous with the posterior floor of the hypothalamus and tuber cinereum (). The mass was isointense to the gray matter on all sequences and appeared to produce significant mass effect on the posterior aspect of the optic chiasm and both optic tracts, with anterior displacement of the pituitary stalk.\nHormonal testing revealed intact anterior pituitary gland function. There were no symptoms suggestive of central diabetes insipidus. Serum germ cell markers were negative. Formal visual field testing did not exhibit deficits. Formal otolaryngology evaluation did not reveal a bifid uvula or epiglottis.\nRadiographs of the left hand revealed several morphologic abnormalities, including partial fusion (syndactyly) of the 3rd and 4th metacarpals, and polydactyly of the middle and distal phalanges of the 5th finger ().\nWhile the headache persisted for 24 hours and then resolved with use of analgesics, the visual changes resolved spontaneously within 4 hours of admission. The hypothalamic hamartoma was suggested to be asymptomatic and not the cause for his temporary visual loss. Other etiologies were not identified.\nBased on the polysyndactyly of his left hand and MRI findings consistent with a hypothalamic hamartoma, PHS was considered.\nWhile optic nerve decompression via right supraorbital craniotomy was considered because of the acute visual changes, the multidisciplinary team and patient elected for close follow-up without intervention in light of the syndromic presentation, negative formal visual field testing, and historically benign nature of hamartomas.\nSubsequent gene sequencing of GLI3 performed at GeneDx (Gaithersburg, MD) revealed a heterozygous c.2388delT variant causing a frameshift, starting with codon Leucine 797, changing the amnio acid Leucine to a Tyrosine, and thus creating a premature stop codon at position 12 of the new reading frame, denoted p.Leu797TyrfsX12. This pathogenic variant is predicted to cause loss of normal protein through protein truncation. This variant had also not been previously reported. We were unable to clinically and genetically evaluate his mother and brother. Genetic counseling was offered but declined by the family.
A 60-year-old Indian male relative of a patient visiting the dental outpatient department was noticed with a massive swelling on the right side of his face. Verbal consent was obtained from him for examination. History revealed a slowly growing painless swelling of the right mandible of 30 years duration that gradually extended to involve the upper jaw, right side of face and temporal region. The swelling was well-circumscribed, roughly ovoid, lobulated, nontender and firm and measured 18 cm × 12 cm × 11 cm, extending 3 cm above supraorbital margin superiorly and behind pinna of the ear laterally on the right side. Intra oral examination revealed a huge nontender fleshy mass of the right mandible of differing consistency displacing the tongue, obliterating buccal sulci and extending from lower right canine to retromolar area. There was partial and spontaneous loss of dentition. Posterior-anterior view of the skull revealed a huge soft tissue mass shadow on the right side of face involving posterior part of the maxilla and with mandibular destruction []. Coronal section of computed tomography (CT) showed lesion extended from tempero-parietal region till the hyoid bone with displacement of greater wing of sphenoid, temporal bone and maxillary antrum. Axial section of CT scan at a higher level showed erosion of the temporal bone with dural extension. Three-dimensional reconstructed views showed the entire tumor extent that had resorbed the mandible, zygoma and temporal bone on the right side []. Baseline laboratory investigations revealed low hematocrit, raised platelet count and erythrocyte sedimentation rate, but normal protein levels. The patient was provisionally diagnosed with multicystic ameloblastoma. Fine-needle aspiration cytology from the swelling yielded a hemorrhagic, cystic fluid consisting of inflammatory cells in a fibrinous background. Histopathological examination of the tissue specimen obtained by incisional biopsy from the swelling revealed typical ameloblastomatous follicles with varying amounts of granular cell changes, cystic degeneration, and squamous metaplasia in the follicles []. It was thus diagnosed as a GCA. After improving patient's hematocrit, hemimandibulectomy with disarticulation was performed. The tumor mass was resected along with the parotid gland, facial nerve, zygoma and zygomatic arch of the ipsilateral side. Intra operatively, a 2 cm squamous part of the temporal bone was resorbed with a dural tear, but there was no cerebrospinal fluid leakage. Reconstruction was done with pectoralis major myocutaneous flap. The excised specimen measured 17 cm × 11 cm × 9 cm and weighed 500 g. The interior showed multiple locules, few with whitish nodular projections. Histopathological findings from the specimen samples were in agreement with the initial diagnosis. However, our attention was drawn to the unique epithelial changes seen in this case. There were bland looking epithelial cells with flattened epithelium and whorling resembling squamous odontogenic tumor and conventional ameloblastomatous islands budding from the oral epithelium with focal to extensive proliferations of GCA islands form oral epithelium. Granular cell changes were noted extending right across the entire oral epithelium, from the basal and spinous layers up to the superficial layers []. To ascertain the nature of granular cells, immunohistochemistry was performed with CD68, bcl-2, and Ki-67. Intense positivity for CD68 was shown by granular cells in the follicles, but not by the peripheral ameloblastic cells. The latter however showed moderate staining to bcl-2 and weak staining to Ki-67. The granular cells showed occasional faint to no reactivity toward Ki-67 []. The patient was disease free in a 4-year follow-up.
A 45-year-old woman was referred to the Vulvovaginal Disease Clinic with debilitating vulvar burning and itching over the right labium majus, also causing severely disturbed sleep. Vulvar pain had been persisting for 2 years and was considered intractable. Careful history taking revealed that she had two vaginal deliveries and was known to suffer from Crohn’s disease involving the right hemicolon, terminal ileum, and rectum, for which she was treated with azathioprine orally and rectally applied mesalazine. The patient also had a laparoscopic fallopian tube ligation. Finally, she had a history of atopy, and reported occasional seasonal allergic rhinitis symptoms.\nThe patient was initially followed at a regional hospital. A blind biopsy was taken from the right labial skin in a dermatology practice; it showed no epidermal or dermal anomalies. Several topical treatments were initiated empirically, including vaginal antibiotic and fungistatic agents and various corticoid ointments, without providing relief. During a surgical consult, a nodule was detected in the right labium majus and was the presumed cause of the chronic pain. It was removed under general anesthesia, and had histological features indicative of a ruptured hair follicle. As no diagnosis was obtained, the patient was also asked to discontinue all medications, including those for treatment of Crohn’s disease. The patient was eventually referred to our hospital, which is a tertiary referral center.\nCareful examination of the vulva and vagina revealed neither visible anomalies nor any other findings indicative of vulvar inflammatory, infectious, or neoplastic mucocutaneous disease. During the clinical exam, the patient was asked to point out the pain area, showing the right labiocrural fold, the anterior surface of the right labium majus, and right medial upper thigh, though burning sensation of the right labium majus was the most prominent complaint and was initially mentioned by the patient as the only complaint. Tactile sense assessment was performed with a piece of cotton wool, a cotton-tip swab, and a sharp wooden stick over these areas and further compared with the contralateral sites. While varied dysesthetic tactile responses were obtained, the overall pattern was hypoesthesia upon soft touch and pronounced hyperesthesia and allodynia upon sharp touch. Though often difficult to differentiate from ilioinguinal neuralgia, this particular pattern was highly suggestive of genitofemoral neuralgia, involving both the genital and femoral branches of the genitofemoral nerve (). Maximal passive hip flexion further provoked burning over the right labium majus, though not the thigh.\nSubsequently, electrophysiological examination through somatosensory evoked potentials (SSEP) assessment of the labia majora was performed. The labia majora were stimulated with bipolar surface electrodes (cathode proximally) at an intensity of two times the sensory threshold, with stimulus duration of 0.2 ms and a frequency of 2 Hz. Averaging 256 sweeps, responses were recorded twice to ensure reproducibility. Recording was done using needle electrodes, with the active electrode placed 2 cm behind the Cz position of the International 1 0–20 system and the reference electrode at the Fpz position. The ground electrode was placed over the right anterior thigh. Sensitivity was set at 10 µV and analysis time at 100 ms. Recordings were performed with the Medelec Sapphire device (Vickers Healthcare Co, Woking, UK). SSEP responses over the right compared to the left labium majus showed a prolonged latency (32.2 versus 29.1 ms corresponding to ~10% prolongation) and considerably decreased amplitude (0.50 versus 1.58 µV corresponding to ~70% lower amplitude).\nAdditionally, imaging of the pelvis was conducted through magnetic resonance imaging (MRI), whereby T1-, T2- and proton density-weighted sequences were obtained, though no anomalies could be documented.\nInitial pain relief was procured through application of a 5% lidocaine patch fit to the right labium majus and left in place during daytime for 12 hours a day. While providing some attenuation of pain during the day, the patient continued to experience severely disturbed sleep. Nortriptyline at a daily dose of 25 mg was ill-tolerated without any effect on vulvar pain. Venlafaxine was slowly titrated, starting with a dose of 37.5 mg and adding up in increments of 37.5 mg per day at intervals of 7 days, until a daily dose of 150 mg was reached. While reporting mild side effects of vivid dreaming and increased transpiration, the patient experienced rapid improvement of vulvar pain, which allowed her to resume normal sleep at night, after 2 years of sleep deprivation. She gradually tapered off use of the lidocaine patch and reported that she no longer experienced vulvar discomfort during activities of daily life. In view of the side effects under venlafaxine, the treatment regimen was changed to duloxetine 60 mg a day, resulting in disappearance of the side effects, while maintaining optimal pain relief of the vulva. Discomfort over the right thigh, primarily described as itching rather than burning, did persist however, for which we initiated a compounded topical gabapentin cream in a 6% concentration formulated in a nonionic cream, resulting in significant relief of discomfort over the right thigh. At some point in time during follow-up, the patient reported a slight but hindering exacerbation of vulvar pain under duloxetine. Careful history taking revealed that the pain reoccurred following a bicycle day trip, and the patient was therefore instructed on vulvar care with emphasis on limiting vulvar mechanical strain as a known aggravating factor. One year after initial assessment, the patient remains pain free under continued treatment with duloxetine orally 60 mg a day and 6% gabapentin cream applied twice daily. For this kind of approach or publication, Ghent University Hospital Institutional Review Board approval was not deemed necessary by our institution. Written informed consent from the patient was obtained.
A 28-year-old man presented to the outpatient department with complaints of chronic pain over the right preauricular region for the last 4 years. The pain was gradual in onset and progressive in nature. Pain was aching in nature with the sensation of heaviness over the right preauricular region. There was no history of diminished or altered sensations over the face. There was also history of intermittent serous discharge from the right ear for the last 4 years. Patient had also noticed diminished hearing in the right ear with the onset of discharge. He denied any history of trauma, seizures, vomiting, fever, or other associated symptoms. In view of these symptoms, patient was diagnosed to have chronic suppurative otitis media and underwent mastoidectomy twice over a period of 4 years at other institute.\nOn examination, higher mental functions were normal. There was conductive hearing loss in the right ear, Rinne's test was negative, and Weber's test was lateralized to the right ear. Facial nerve function was normal. Rest of the cranial nerves and neurological examination was normal. On local examination, a tender bony hard swelling of 3 cm × 2 cm × 2 cm size was felt in the right preauricular region. There were no other signs of inflammation over the swelling. The scar of previous surgery was well healed.\nComputed tomography (CT) scan of the brain with and without contrast revealed an extradural heterogeneously enhancing mass in the right temporomastoid region []. MRI scan of the brain showed an extra-axial solid cystic lesion in the right temporomastoid region. The lesion was hypo to isointense on T-1 weighted, heterogeneously hyperintense on T-2 weighted and showed heterogeneous enhancement on gadolinium contrast images []. Preoperative digital subtraction angiography was showing a tumour blush in the right temporal skull base and adjacent middle fossa. The predominant vascular supply was from the right middle meningeal artery. The right petrous carotid artery was encased by the tumor.\nThe patient underwent a right temporal craniectomy with wide exposure of the petrous bone. The tumor was devascularized by coagulating the middle meningeal artery early in the procedure. The tumor was located in the middle temporal fossa outside the temporal dura mater. The lesion was variegated in appearance and had a well-formed capsule. There were cystic spaces within the tumor which were filled with straw-colored fluid. The lesion was densely adherent to the temporal dura mater with extensive destruction of normal surrounding bone, however, there was no evidence of breach in the surrounding duramater. A small portion of the tumor which was extending toward the base of the skull was left behind. An en bloc gross total excision was achieved.\nHistolopathological examination had shown thin bony trabeculae which were enclosing the dilated blood-filled spaces. These septae consist of fibrocollagenous tissue admixed with loosely woven bone and multinucleate giant cells. There was no evidence of malignancy. These histological features with the clinical presentation were characteristic of ABCs [].\nIn the immediate post-operative period, the patient had grade 2 (House and Brackmann) Facial paresis which had recovered back to normal with a short course of steroids at the first follow-up. Post-operative CT scan had shown the gross total excision of the tumor except the portion which was extending to the skull base [].\nAt the last follow-up of 12 months, the facial nerve function was normal and hearing in the right ear had improved on clinical examination.
Our patient is a 54-year-old female with a past medical history of hypothyroidism and very severe obesity (BMI 48 kg/m2). She underwent laparoscopic gastric sleeve surgery in the year 2012. Results were non-satisfactory in terms of weight loss with a difference of 6 kg/m2 in BMI post-procedure. So after six years, she underwent a laparoscopic biliopancreatic diversion with a duodenal switch. She had an uneventful postoperative recovery period. An upper gastrointestinal (GI) study contrast post-procedure did not reveal any evidence of obstruction or leak. The patient was discharged home two days after the procedure. A few days later, she started experiencing three episodes of nausea with brown-colored vomitus. She was found to be septic, with a heart rate of 110 beats per minute and temperature of 100.2oF. Her white blood cells count was 12/mm3.The source of infection was presumed to be intraabdominal considering her symptoms. Computed tomography (CT) of the abdomen and pelvis showed mildly dilated proximal small bowel loops. The patient was started on empiric antibiotic therapy with ceftriaxone 1 gm intravenous (IV) daily and metronidazole 500 mg IV every eight hours. Symptoms did not improve, so she was taken back to the operating room for diagnostic laparoscopy. Partial small bowel obstruction was noted along with ischemia of a segment of the ileum that was part of the duodenoileostomy due to mesenteric dissection. She underwent an open revision of the small bowel anastomosis with resection and anastomosis for the obstruction revision of the duodenoileostomy. Her hospital stay post-surgery remained uneventful. Diet was advanced gradually throughout the hospital course and a week later, the patient was discharged home with outpatient follow-up. Three weeks after that procedure, she noticed a productive cough with thick, yellow, foul-smelling phlegm and shortness of breath. She saw her primary care physician. A chest X-ray performed showed a right lung infiltrate with a right-sided pleural effusion. She was started on treatment with augmentin 500 mg/125 mg every eight hours. Her symptoms became worse so she came to the emergency room. Her vitals showed blood pressure 129/79 mmHg, heart rate 86 beats per minute, respiratory rate 20 breaths per minute, and temperature 98.6oF. Pulse oxygen saturation was 97% on room air. Mild leukocytosis was evident (white blood cells count 11.4/mm3 with no bands or left shift). A chest CT showed loculated, right-sided hydropneumothorax with almost total collapse of the right lung (Figure ).\nThere was a fistulous connection evident, extending from the surgical anastomosis in the stomach/bowel in the right upper quadrant through the right hemidiaphragm to the right hemithorax. These CT scan findings were new as compared to a CT scan obtained for this patient six months prior to the duodenal switch when she presented to the emergency department for non-specific left-sided chest pain. To analyze the anatomy of the fistula further, an upper gastrointestinal fluoroscopic contrast study was performed that showed a large fistula from the distal stomach prior to the duodenal bulb opening to the right pleural cavity (Figure ).\nConsultations from gastroenterology and cardiothoracic surgery teams were obtained. Chest tube drains were placed with the plan of eventually performing a video-assisted thoracoscopic surgical decortication. Post-procedure CT showed patent chest tubes draining the right pleural cavity. The drained fluid was exudative in nature as per Light’s criteria (fluid lactate dehydrogenase > 12,000 u/L and total protein ratio = 0.7) and culture from the right lung empyema grew Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, and Candida albicans. An infectious disease consultation was placed at this time. The patient was started on levofloxacin 750 mg IV daily for two weeks as per the sensitivity result obtained for Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa. Micafungin 100 mg IV daily was started for the infection with Candida albicans. This was later switched to Diflucan 400 mg IV daily for a total of two weeks. Repeat cultures from the draining fluid were negative toward the end of the second week.\nFor treatment of the fistula, the patient was transferred to another facility for esophagogastroduodenoscopy (EGD) and possible clipping of the fistula due to the unavailability of that particular service in our hospital. As a result, there was a delay of 16 days from admission to the treatment of the fistula. When the EGD was performed, it showed that there was no anastomotic leak from the previous surgery. No evidence of any stricture was identified at the previous anastomosis. A small fistulous tract was noted in the distal part of the antrum likely secondary to ulcer formation that was noted in very close proximation to the fistula tract. An endoscopic clipping was performed. A post-procedure contrast study performed on the same day revealed complete closure of the fistulous opening. The patient was transferred back to our facility after the procedure. An upper GI contrast study performed two days later showed residual leakage from the distal stomach to the right upper quadrant. A repeat EGD was performed along with fulguration of a fistulous opening with argon beam coagulation and repeat orthoscopic clip application with complete obliteration of the fistula tract. This was confirmed by an upper gastrointestinal contrast study showing no persistent fistulous communication between the post-bulbar duodenum and pleural space (Figure ).\nAfter confirmation of fistula obliteration, a video-assisted thoracoscopic surgery was performed followed by the washout of the right pleural space with the placement of a right-sided chest tube. The patient was discharged home after the procedure and has been followed by multidisciplinary teams on an outpatient basis.
In June 2002, a 36-year-old woman presented to her primary health care doctor with a history of flushing, diarrhoea, night sweats, and a clinically detectable mass in her left medial supraclavicular fossa. Her past medical history consisted only of essential hypertension for which she did not require prescribed therapy. Her family history included a brother with a diagnosis of sarcoma and two other non-first-degree relatives with primary brain malignancies.\nFine needle aspiration confirmed the diagnosis of medullary thyroid cancer, and in July 2002, she underwent total thyroidectomy with left-sided modified radical neck dissection and central compartment clearance. At this point, concerns were raised regarding optimal cytoreduction as the appearances of the central compartment, level 4 and level 5 nodes, were that of extensive disease. In order to maximise local disease control, she received adjuvant radical radiotherapy delivering 60 Gy to the thyroid bed.\nTwo years after completion of treatment, in February 2004, follow-up repeat imaging reported a recurrent nodule at level 4 of her neck. Subsequent resection confirmed this to be recurrent medullary thyroid cancer with no evidence of distant spread at the time. She continued to be monitored at the oncology clinic and remained disease-free until four years later, in May 2008, when computer tomography (CT) imaging revealed new pulmonary parenchymal metastases. These were closely monitored for the next 2 years with repeat imaging and measurement of calcitonin levels. In May 2010, it was decided that the patient should embark on systemic anticancer treatment.\nShe was offered participation in a phase 2 clinical trial with the agent lenvatinib (E7080) and she commenced treatment with 24 mg once daily in May 2010. One week into therapy, it was noted that she was marginally hypertensive with a blood pressure of 140/100 mmHg. No proteinuria was identified at this point, but she was commenced on 5 mg of amlodipine to manage hypertension. Monitoring of blood pressure and urinalysis continued as per study protocol.\nAfter two completed cycles of lenvatinib, CT imaging reported a reduction in size of all lesions. Further tumour assessment after 4 months confirmed a partial response to treatment with a 50% reduction of the sum of the long diameters of target lesions. She was experiencing various grade 1 toxicities throughout this time but was keen to maintain treatment given the good response. Due to the multiple low-grade toxicities, the dose of lenvatinib was initially reduced to 20 mg and thereafter to 14 mg.\nIn December 2011, 19 months after starting lenvatinib, she developed mild ankle oedema. Urinalysis carried out at the time identified proteinuria. A subsequent 24-hour urine collection identified 3.1 g/litre of proteinuria, equating to a urinary protein creatinine ratio (UPCR) of 625. The patient had not started any other medications and the incidence of proteinuria was felt to be lenvatinib related. Treatment with lenvatinib was ceased; however, due to concerns regarding possible intrinsic renal disease, she underwent screening for glomerulonephritis which was negative.\nA subsequent renal biopsy showed focal segmental glomerulosclerosis (FSGS) in two of twelve viable glomeruli, with tuft-capsule adhesion, hyalinosis, segmental intracapillary hypercellularity, and segmental splitting of capillary walls, predominantly in regions of segmental sclerosis. There was mild tubular atrophy, interstitial fibrosis, mild/moderate arterial intimal fibroelastic thickening, and mild arteriolosclerosis. Immunofluorescence showed no staining in glomeruli. Electron microscopy showed mild patchy reduplication of the basement membrane and effacement of only 20% of podocyte foot processes. There were no widespread electron dense deposits and no endothelial cell tubule-reticular inclusions. The endothelial cells showed no evidence of activation or damage. The appearances were consistent with a diagnosis of focal segmental glomerulosclerosis (FSGS). The lack of widespread podocyte foot process effacement suggests a secondary form of FSGS, which in the context of anti-VEGF treatment, could be mediated by microangiopathy. Whilst there was no histological evidence of acute thrombotic microangiopathy, it is possible that some of the pathological changes seen (splitting of glomerular capillary walls and mild arteriosclerosis) could be related to chronic low-grade endothelial cell damage. Based on histology, it is not possible to be certain whether the FSGS was caused by direct podocyte injury or whether it was related to endothelial cell injury. Histological slides are illustrated below in Figures , , and .\nThroughout this time, excretory function remained stable. Treatment with an ACE-inhibitor (ACEi) was introduced but due to poor tolerance and the quick improvement of the proteinuria after cessation of lenvatinib, the ACEi was stopped and patient's blood pressure was monitored closely.\nWithdrawal of lenvatinib had a marked effect on the levels of proteinuria, as illustrated in .\nThe patient continued follow-up at the renal clinic on a regular basis until July 2013, when she was discharged with no evidence of proteinuria, normotensive and with normal excretory renal function.\nAfter her discharge from the renal clinic and between 2013 and 2017, the patient was treated with vandetanib, nintedanib, and cabozantinib with no evidence of recurrent renal disease.\nE7080, also known as lenvatinib, is a potent inhibitor of the receptor protein kinases VEGFR-2 and VEGFR-3 but also displays inhibitory binding properties against VGFR-1, FGFR-1, and PDGFRα/β, albeit at significantly higher IC50 (half maximal inhibitory concentration, IC50). Its ability to restrain angiogenesis was shown on human umbilical vein endothelial cells (HUVEC) where E7080 inhibited VEGFR-2 phosphorylation and thereby capillary tube formation []. Apart from angiogenesis, E7080 decreased lymphangiogenesis in both the primary tumour of human breast adenocarcinoma cells in xenografts as well as in metastatic nodules in the lymph nodes of nude mice bearing these tumours []. Glen et al. showed in preclinical experiments that abrogation of FGFR and PDGFR signalling by E7080 inhibited invasion and migration of human melanoma cells lines (DX3) and human osteosarcoma epithelial cells (U2OS) []. Its potency against FGFR-1 differentiates E7080 from other currently approved tyrosine kinase inhibitors with antiangiogenesis properties [, ].\nThe preclinical data above were confirmed in several early phase human trials with E7080 in 2011 and 2012 in US, Europe, and Japan. Whilst establishing pharmacokinetic and pharmacodynamic properties of the drug, safety and preliminary efficacy was also well described. Lenvatinib was well tolerated at doses from 10 mg BID to 25 mg OD [–] and was associated with a reduction in disease activity biomarkers [], partial response, and stable disease according to response evaluation criteria in solid tumours []. These findings were further established in phase 2 trials and notably responses were demonstrated in thyroid cancer [–].\nApproval in thyroid cancer was granted in light of significant improvement in progression-free survival (PFS) compared with placebo in patients with radioiodine-refractory differentiated thyroid cancer in a phase 3 study (SELECT study) []. Lenvatinib improved median PFS over placebo by almost 15 months (HR 0.21; p < 0.01) and induced an objective response rate of 64.8%. The median survival results were diluted due to crossover of the patients from the placebo arm to the treatment arm; nevertheless, a subgroup analysis on patients stratified by age showed that older patients (>71 years old) had a survival advantage when treated with lenvatinib compared to placebo (HR, 0.53; p = 0.02), and the younger subgroup achieved a PFS of 20.2 months versus 3.7 m (p < 0.001) [].\nProteinuria and hypertension are the two most commonly reported side-effects of VEGF inhibitors and frequently the cause for therapy discontinuation. Proteinuria is used as a surrogate marker for glomerular damage and hypertension often accompanies and aggravates this.\nThe pathophysiology of proteinuria and glomerular damage in anti-VEGF therapy remains complex and far from well understood. Biopsy-proven cases of glomerular disease in anti-VEGF therapy are few; however, most have demonstrated changes in keeping with glomerular thrombotic microangiopathy (TMA) histology, with predominant endotheliosis and membranoproliferative changes [, ]. Other histological changes documented include cryoglobulinaemic glomerulonephritis, acute interstitial nephritis, collapsing and crescentic glomerulonephropathies, and FSGS plus TMA [–].\nIt has been theorized that hypertension is caused by decreased vascular production of nitrous oxide induced by inhibiting VEGF. This leads to renal haemodynamic compromise and subsequent proteinuria (much akin to exercise-related proteinuria) []. However, a mouse model study showed that glomerular injury preceded hypertension [] and many cases document glomerular injury in the absence of hypertension [], indicating that it cannot be the only trigger for proteinuria in anti-VEGF treated patients.\nInhibition of VEGF in podocytes (by injection of anti-VEGF antibodies or VEGF gene deletion) results in loss of endothelial fenestrations in glomerular capillaries, proliferation of glomerular endothelial cells, loss of podocytes, and proteinuria in mice [, ]. VEGF appears to be a crucial endothelial survival factor and its inhibition often manifests as TMA, a histology strikingly similar to that of severe preeclampsia—as placenta overproduces a soluble VEGF receptor (fms-like tyrosine kinase 1) that acts as a VEGF antagonist.\nIzzedine et al.'s 8-year follow-up study results from 2014 shed great light in anti-VEGF-related renal injury. It showed that in 100 patients who developed renal disease whilst on anti-VEGF treatment, the main histology associated with TKIs was minimal change disease and/or collapsing-like focal segmental glomerulosclerosis (MCN/cFSGS), a FSGC variant which is considered a separate entity to FSGS. In the same analysis, TMA histology was most frequently associated with VEGF-ligand targeted therapy (such as bevacizumab and aflibercept) suggesting two, possibly distinct pathophysiologies [, ] between renal damage caused by targeting the VEGF ligand as opposed to targeting the VEGFR tyrosine kinase domain. This could potentially be explained by considering the associations and signal transduction pathways between podocytes, endothelial cells, and VEGF. Podocytes produce vascular endothelial growth factor (VEGF), whereas VEGF receptor tyrosine kinases (RTKs) are expressed by both podocytes and glomerular endothelial cells.\nOur case demonstrates a secondary form of FSGS pathology which cannot confidently be attributed to TMA but could potentially represent the end result of chronic low-grade endothelial cell damage. The moderate histological findings were in keeping with a less-severe clinical course of the FSGS, with fast resolution of proteinuria and hypertension. More significantly, rechallenging the patient with additional three agents blocking the VEGF axis did not result in recurrence of the renal damage.
Case 1 was a 63-year-old man who presented with a 4-month history of productive cough and started to deteriorate after having a fever for a week. The patient had an 8-year history of cigarette smoking, and his father had been diagnosed with lung cancer. Repeated antibiotic treatment had been used since he started coughing, which produced a small amount of yellow sputum, and on January 6, 2018, he was diagnosed with community-acquired pneumonia. Unfortunately, his condition did not improve, and he gradually developed persistent right chest pain and shortness of breath after activity. He was admitted to our hospital on March 19th, and chest computed tomography (CT) showed an irregular solid nodule in the anterior segment of the upper lobe of the right lung and lamellar density shadows in the lower lobe of the right lung ( and ). A bronchoscopic examination showed that the bronchi were unobstructed, slightly congested and had a mild amount of purulent exudate. A transbronchial lung biopsy (TBLB) was performed in the posterior basal segment and dorsal segment. Galactomannan (GM) of bronchoalveolar lavage fluid (BALF) was 1.068. No fungus, interstitial fibroblastic proliferation, incrassation or fibrosis was seen in the lung tissue biopsy, but acid-fast staining and PAS negativity suggested organizing pneumonia; the patient was then diagnosed with organizing pneumonia. The patient was started on prednisone 30 mg orally once a day on March 27, and he was discharged after his symptoms of cough, chest pain and shortness of breath had gradually improved. He took prednisone as prescribed after discharge. However, his condition worsened with a fever that developed on May 8, and he was admitted to the hospital on May 15. On examination, his body temperature was 39.6°C, and his superficial lymph nodes were not swollen. A small moist rale was detected in the lower right lung. Routine blood tests revealed a leukocyte count of 15.85×109/L, a red blood cell count of 4.18×1012/L, a hemoglobin level of 110.70 g/L, and a neutrophil percentage of 83.0%. His hypersensitive C-reactive protein was 171.23 mg/L, the erythrocyte sedimentation rate was 70 mm/h, and serum procalcitonin was 0.707 ng/mL. The serum was positive for AIGAs. His plasma HIV antibody, blood culture, beta-D-glucan, GM and Cryptococcus latex agglutination tests were all negative. The lesion in the right upper lobe was still present, and the high-density lesions in the posterior and outer basal segments of the lower lobe of the right lung were slightly smaller on May 16. A percutaneous lung biopsy was performed on May 21, 2018. The right lower lung biopsy showed a chronic inflammatory lesion but no granuloma or neoplastic lesion. Immunohistochemistry with PAS and acid-fast staining showed that the chronic inflammatory lesions were negative. After the admission of the patient and after considering an infection in the lung, the dosage of prednisone was gradually reduced and ultimately discontinued on May 24. The patient was successively treated with imipenem-cilastatin, voriconazole, compounded sulfamethoxazole and linezolid but still had a fever, with the highest temperature being 39.1°C. On May 30, his symptoms, including difficulty breathing, gradually worsened, and he developed type I respiratory failure. Therefore, he was transferred to the intensive care unit (ICU) for endotracheal intubation for salvage treatment. After admission to the ICU, the patient had scattered herpes in his right anterior chest wall and posterior chest wall. Herpes zoster was clinically diagnosed, and acyclovir sustained release tablets were given as oral antiviral treatment. T. marneffei was cultured from lung biopsy tissue performed on June 4, and chest CT showed growth of the two lung lesions ( and ). Liposomal amphotericin B was started on June 6 as an antifungal agent; the patient’s temperature returned to normal, and the patient’s cough and shortness of breath improved after 3 days. He was transferred back to the general ward. Unfortunately, liposomal amphotericin B was discontinued due to progressive renal impairment and was replaced by voriconazole 0.2 g twice daily starting on June 19. He was discharged after a chest CT showed that the lung lesions were significantly smaller ( and ) on June 25. He was started on a course of itraconazole after discharge. He developed another episode of cough and fever with a maximum temperature of 40°C on December 25, 2020. A chest CT showed that the nodules in the right upper lobe of the lung were significantly larger and that the lesions in the lower lobes of both lungs were stable on January 15, 2019 ( and ). Bronchoscopy revealed that the mucosa in the anterior segment of the right upper lobe had lost its normal structure, and the lumen was narrow. The pathology from the TBLB performed in the anterior segment of the right upper lobe showed primary adenocarcinoma (–). The patient was confirmed to have right lung adenocarcinoma and TSM. However, he was discharged after refusing treatment and died in February 2019.
Patient 3 was a 16 year old girl that first presented in our outpatient clinic at 12 years of age. She had been born preterm by emergency caesarean section due to pre-eclampsia. Respiratory support through CPAP (“continuous positive airway pressure”) was needed for 4 days as well as a nasogastric tube. After discharge, new-born development was normal until the fourth month of life, when axial hypotonia was first noticed. At 12 months, discrete dyskinetic movements began. Developmental milestones were only met partially and with delay: at the age of 2 years, she was able to crawl and at 2.5 years, she was able to walk unassisted yet in a clumsy way and with need for a wheel-chair for longer distances. With 2 years, first hyperkinetic exacerbations appeared, lasting up to 1 min and occurring several times per hour. These hyperkinetic attacks started suddenly, were triggered by strong emotions, movement, temperature changes (for instance during bathing) and occurred rarely at night. During childhood, these sudden movement spells increased in frequency. Other clinical features such as hypersalivation, dysarthria and oral/tongue dyskinesia were reported. There were no signs of cognitive impairment. Family history was negative for movement disorders. Extensive clinical work-ups prior to first visit at our outpatient clinic including cerebral MRI at 1 and 5 years of age, analysis of cerebral spinal fluid including neurotransmitter metabolism and screening for metabolic disorders had remained elusive. Genetic analyses for dystonia, paroxysmal dyskinesia or episodic ataxia were negative but revealed a compound heterozygote ATM mutation of unknown significance that was first considered responsible for the disorder. Re-evaluation at the age of 12 revealed the pathogenic ADCY5 mutation. Treatment with levodopa was initiated at age of two (13 mg/kg/day) and led to an increase of dyskinesia. Carnitine, carbamazepine and clonazepam had no effect on the movement disorder.\nClinical examination at first visit showed a slim, pre-pubertal 12-year-old girl with permanent generalized choreoathetosis that was superimposed by intermittent myoclonus and dystonia. Axial hypotonia with dropped head was evident mainly when trying to stand up and during ambulation. She could stand unsupported for a few seconds. She would walk > 10 m without aid in a clumsy and dystonic manner. Perioral and tongue dyskinesia were nearly permanent and comprised slow choreatic movements as well as short jerks. Speech was effortful and dysarthric. Even though there was no apparent facial palsy, voluntary control of facial mimics and tongue movement were impaired. Deep tendon reflexes were brisk, pyramidal signs were negative. Limb muscle tone was normal, muscle strength fulfilled 5/5 MRC (“Medical Research Council” Scale). During the exam, the movement disorder exacerbated for episodes of < 1 min duration without an obvious trigger. The patient was not taking any medication at that time.\nAt the age of 13, DBS electrodes were implanted bilaterally in the GPi and a rechargeable Boston Scientific Vercise RC impulse generator subcutaneously in the left upper thorax. Immediately after surgery a reduction of dyskinesia was observed and sustained for about 6–8 weeks. 3 months after surgery, monopolar review was performed and new parameters were set, leading to a decrease in the hyperkinetic baseline movement disorder as well as the episodic storms. More than that, the ability to sit freely and to walk as well as the involuntary tongue movements and voluntary tongue control were improved. There was no effect on hypersalivation or dysarthria. 2 years after implantation the patient reported improved gait as biggest achievement of the stimulation. Furthermore, sitting and standing as well as tongue and facial dyskinesia were reported with clinically meaningful improvement. The sudden attacks during the day had become rarer. At the last follow-up visit, 3 years after implantation, the baseline movement disorder had deteriorated slightly, which resolved after subtle changes in current settings.\nRetrospective video ratings of the AIMS revealed a mild improvement of involuntary movements, no evident improvement was assessed by the BFMDRS (see Table for details). The girl and her father reported a subjective general improvement of 40–50% after DBS. 1 year after surgery, the battery of the IPG emptied completely due to technical problems. With delay of about 1 week involuntary movements increased leading to a visit of the emergency department. The transient worsening of the movement disorder resolved after proper charging of the IPG.
A 30-year-old woman presented with gradually increasing weakness of both upper and lower limbs. She reported having had a similar episode twice previously, most recently 2 months ago; and at all of these occasions, she had documented hypokalemia and she was managed by a local physician with parenteral and oral administration of potassium chloride that improves her condition on these occasions. Resting after exercise or eating did not precipitate the weakness and there was no family history of similar illness. Patient also had complaints of recurrent oral ulceration, photosensitivity, hair loss, loss of appetite, easy fatigability, pain in multiple joints and she experienced this pain almost throughout the day, but it was worse in the morning. The patient also had significant morning stiffness and daytime fatigue. All these symptoms were present since 6 months. She had also off and on low grade fever over 3 months before admission but she had no burning in the urine, cough and abdominal pain or vomiting, pruritus, dryness of mouth, difficulty in swallowing, gritty or sandy sensation under eyelids and Reynaud's phenomenon. On the day of admission, the patient also complained of difficulty in swallowing solid food and inability to speak loudly but there was no history of visual blurring, diplopia, facial asymmetry, sensory, bladder, or bowel complaints. She had a clear sensorium with no history of seizures, involuntary movements, root pains, or band-like sensation over the trunk. She did not give a history of prior drug intake, colicky abdominal pain and recent vaccination.\nSix months before her current presentation, the patient had progressive weakness in all four limbs; it started in one limb, but progressed to all four limbs within a few hours and her past medical record and history given by her husband revealed that she was conscious and oriented at the onset of weakness. Her eye movements were normal and there was no facial asymmetry and seizure at the onset of weakness. At current admission to our hospital in physical examination, blood pressure was 130/80 mm Hg; Pallor was present. There was no edema, icterus and lymphadenopathy. Musculoskeletal and neurological examination revealed muscle strength was poor (flicker 1/5) in all the muscle groups. Deep tendon reflexes were intact and planter reflex was flexor bilateral. Laboratory investigations done revealed blood urea 28 mg/dl, serum creatinine 1.3 mg/dl, hypokalemia 2.2 mmol/L, hyperchloremic metabolic acidosis (chloride 112 mEq/L, arterial blood pH 7.279) and alkaline urine (urinary pH 7.5). Radiograph of the abdomen did not show any nephrocalcinosis. Ultrasonography of the abdomen revealed right nephrolithiasis with bilateral normal size kidney and normal liver size and echotexture. In view of the normal anion gap acidosis (12.4 mmol/l), alkaline urine (urinary pH 7.5), low serum potassium (2.2 mmol/l), absence of Fanconi's syndrome, positive urinary anion gap (46 mmol/l), the diagnosis of dRTA was made. Type 2 RTA was excluded, as her 24 h urinary excretion of phosphate and uric acid was normal with no glycosuria. Renal biopsy was deferred in view of normal renal function and no active sediment in urine routine microscopy. The patient was started on oral potassium and bicarbonate supplementation. Oral hydroxychlroquine and a short course of steroid were also given. Her biochemical parameters corrected with treatment and she attained blood urea of 24 mg/dl, serum creatinine 1.0 mg/dl, potassium 3.9 mEq/L, chloride 95 mEq/L and arterial blood pH of 7.332. She had continued on the oral supplements with sodium bicarbonate (500 mg twice daily) and oral hydroxychloroquine 200 mg twice a day and had been asymptomatic on regular follow-up [].
M.J., a 38-year-old man developed sudden severe abdominal pain, nausea, and vomiting after shoveling snow. Prior to this event, he denies having had any episodes of hypertension, tachycardia or diaphoresis, although several months prior he was diagnosed with essential hypertension and was started on lisinopril. In addition, he denied any recent abdominal or flank trauma. Of note, his past medical history is significant for a diagnosis of MEN2A which was made at the age of 18 months, and a prophylactic total thyroidectomy at age 10 secondary to elevated serum calcitonin levels. Since that time he has had no further follow-up, although of his two children, his daughter has been diagnosed with MEN2A and undergone a prophylactic total thyroidectomy 2 years prior to this event. On arrival, paramedics found him near syncopal and diaphoretic with a heart rate of 180 bpm and systolic blood pressure of 64 mmHg. Fluid resuscitation was initiated and the patient was taken to an outside hospital. Initial evaluation at the local level II trauma center was notable for a heart rate of 150 bpm, systolic blood pressure of 70 mmHg, diffuse peritoneal signs, a hematocrit of 34%, INR of 1.0 and PTT of 30.4. Following resuscitation with additional crystalloid and 2 units of packed red blood cells (pRBC), his hematocrit was 34%, INR 2.4 and PTT 66.2. A non-contrast abdominal computed tomogram revealed bilateral adrenal masses and a large amount of intra and retroperitoneal hemorrhage (Figure ). In light of his hemodynamic instability and CT findings, emergent laparotomy was undertaken. Upon entering the abdomen, a large amount of blood was encountered and immediate control of the abdominal aorta was obtained to manage the ongoing hemorrhage and facilitate resuscitation which ultimately required 12 units of pRBCs, 4 units of fresh frozen plasma (FFP) and 6 units of platelets. A bleeding source was identified in the left upper quadrant (LUQ) in the retroperitoneal fat which was oversewn. The abdomen was packed with laparotomy pads and closed; the blood loss was estimated to be 8000 cc.\nThe patient was subsequently transferred to our facility for further care. On arrival he was intubated and sedated with a blood pressure of 90/35 mmHg, heart rate 129 bpm, Hct 36.3%, INR 2.7 and fibrinogen 117 mg/dL. On initial examination his abdomen was tense and distended, and his extremities were cold. Ongoing hemorrhage was suspected given the coagulopathy and persistent hypotension, therefore aggressive resuscitation with blood products was resumed. An initial bladder pressure of 33 mmHg along with poor urine output, hypotension and a tense abdominal examination raised suspicion for an evolving abdominal compartment syndrome; therefore a second emergent exploration was undertaken. On entry into the abdominal cavity, the right colon was found to be frankly ischemic and persistent hemorrhage from the LUQ was again noted. As the source of bleeding could not be readily identified, an emergent splenectomy was performed, and laparotomy pads were again packed into the LUQ. Once adequate control of the bleeding was obtained with packing, attention was turned to performing a right hemicolectomy. A Bogota bag with a wound V.A.C (KCI, TX) was then fashioned for temporary abdominal closure. Following closure of the abdomen, the patient suffered cardiac arrest with pulseless electrical activity. Advanced cardiac life support measures were initiated and a perfusing rhythm was obtained shortly thereafter. Given the history of MEN2A and bilateral adrenal masses, the diagnosis of occult pheochromocytoma was entertained. The blood pressure swings were controlled with phentolamine and a sodium nitroprusside infusion with good effect. The patient was returned to the surgical intensive care unit for further management.\nIn the intensive care unit, the patient continued to have a labile blood pressure, a persistent base deficit, decreasing hematocrit and drainage of large amount of blood from the VAC, therefore he was emergently taken to interventional radiology. Diagnostic angiography revealed contrast extravasation from the left adrenal artery which was embolized with 250 micron Embozene™ (CeloNova BioSciences, GA) microspheres and Gelfoam™ (Pfizer, NY) slurry to good effect (Figure ). In addition, extravasation from a left intercostal artery was also controlled with embolization. Although no active extravasation was noted from the transected end of the splenic artery, embolization was performed for additional security. Following this procedure, the patient's Hct stabilized and no further significant hemorrhage was encountered throughout the rest of his admission. Subsequently, a continuous infusion of sodium nitroprusside was required to mange the malignant hypertension. On post-operative day three, treatment with phenoxybenzamine was started for α-adrenergic blockade.\nSerum metanephrines and normetanephrines levels were found to be markedly elevated at 14.0 nmol/L (reference range 0.00-0.49) and 24.3 nmol/L (reference range 0.0-0.89) respectively. Thereafter, his recovery was relatively unremarkable; he underwent two additional procedures to restore bowel continuity and for abdominal wall closure. He was discharged in good condition to a rehabilitation facility on hospital day 25 with instructions to continue taking phenoxybenzamine and labetolol. He returned after approximately 4.5 months for a bilateral retroperitoneoscopic adrenalectomy. Of note, intra-operatively, scarring and adhesions were noted between the left adrenal gland and surrounding periadrenal and perirenal fat. Final pathologic examination revealed a 5 cm right and 4 cm bi-lobed left adrenal (Figure ) pheochromocytomas without evidence of definite vascular invasion or extension beyond either gland. He has since been seen in clinic for routine follow-up, and found to be recovering well, requiring labtelol 100 mg PO bid for adequate blood pressure control. He is currently taking hydrocortisone, 10 mg bid for steroid replacement.
This is a case of a 30 year-old gentleman with a history of Crohn's disease. He was on a regimen of infliximab, infused every 8 weeks and oral methotrexate daily. He had no other significant medical history. He was in his usual state of health until he developed a sore throat and fevers on day 0. His symptoms began while he was traveling in Europe. During his trip, he took a 10-day course of amoxicillin and felt some improvement.\nAfter returning to the US he felt well but on day 14 he developed fevers and sore throat again. He was evaluated at an urgent care clinic and sent home with a diagnosis of a viral syndrome and instructed to treat this with non-steroidal anti-inflammatory agents. He felt some improvement initially but presented again on the day of admission with concern that he may not be well enough for his infusion of infliximab. He was found to have a temperature of 38.3 C and on day 30 was sent to the emergency department for further evaluation.\nIn the emergency department, he reported a non-productive cough, fevers, and sore throat. He had elevated liver enzymes: aspartate aminotransferase (AST) 340, alanine aminotransferase (ALT) 540, alkaline phosphatase 145. Additional testing was sent including a Monospot test, cytomegalovirus (CMV) and Epstein's Barr virus (EBV) serum viral levels, respiratory viral panel by PCR, adenovirus serum viral level, HIV antibody/antigen as well as HIV viral level, viral hepatitis serologic panel, human herpesvirus type 6 (HHV6) serum viral level, Varicella zoster virus (VZV) serum viral level, and syphilis IgG. He was admitted to the inpatient medicine service for fevers and hepatitis of unknown origin. His social history was remarkable for frequent trips to the Midwest for work. He did not pursue outdoors activity while traveling. He did not smoke, drink alcohol or use illicit drugs.\nOn day 31 he developed daily fevers to 40 C and subsequently progressed to hypoxemic respiratory failure that required high-flow supplemental oxygen and transfer to the intensive care unit. He had a chest CT showing ground-glass opacities at the lung bases and a left upper lobe nodular opacity (, ). His CT also demonstrated tonsillar enlargement and splenomegaly. He had a laryngoscopy performed that revealed an exudative pharyngitis. He was noted to have atypical lymphocytosis that peaked to 12,000 cells/uL on day 33. He was started on broad-spectrum antibiotics for presumed hospital-acquired pneumonia; including coverage of atypical organisms. He underwent a bronchoscopy on day 33. Bronchoalveolar lavage (BAL) fluid was negative for Legionella culture, AFB stain and culture, pneumocystis stain, and bacterial culture. Mycoplasma, zygomycetes and Mycobacterium tuberculosis were not detected by PCR. A respiratory viral panel by PCR from the lavage fluid was positive for Bocavirus and one of 2 samples for aspergillus galactomannan was moderately elevated but later determined to be negative on repeat testing.\nOn day 33 his AST peaked at 351 and his ALT at 662. His viral studies sent earlier returned negative. He had further serologic testing for atypical organisms including Blastomyces, Coccidioides, Q fever, Bordetella pertussis, as well as urine Legionella antigen that all returned negative. He was started on methylprednisolone and his fevers improved. His antibiotics were discontinued when his bacterial cultures were negative at 48 h. On day 37 the fungal PCR from BAL returned positive for Histoplasma capsulatum and cultures from that fluid later grew the organism. Urine Histoplasma antigen testing, sent earlier during his hospitalization, ultimately returned with a titer of 2.16 ng/mL (normal high 0.1 ng/mL). His Coccidioides antibody was also positive, but this was felt to be due to cross-reactivity from his Histoplasma infection.\nOn day 37 he was started on liposomal amphotericin B and 6 h later he had his highest fever to 42 C along with hypotension, tachycardia and worsening hypoxemia despite having received a dose of methylprednisolone earlier that day. He was felt to have a paradoxical worsening with treatment and exacerbation of immune reconstitution inflammatory state rather than an infusion reaction due to amphotericin given this time course . His infliximab was restarted the following day with subsequent resolution of his fever, hemodynamic instability and improvement in his respiratory status within hours of this infusion. He continued amphotericin B for one week before transitioning to oral itraconazole. His ALT/AST improved significantly. He was discharged on oral itraconazole with continued clinical improvement and recovery as an outpatient.
A 35-year-old gravida 6, para 5 mother who is 38-week pregnant from last normal menstrual period has presented to Tercha General Hospital (a rural hospital in Southern Ethiopia). The patient is referred from a health center 60 kms far from this hospital for suspected “big baby” in labor. The patient was an illiterate housewife. In terms of past obstetrics history, all previous deliveries occurred at home vaginally with live birth with no major complication. During the index pregnancy, she had antenatal care visits at a nearby health center without ultrasound examination. She reports that the current pregnancy is heavier than previous ones and associated with significant discomfort than her previous pregnancy experiences. Otherwise, she has no self or family history of twinning in the past.\nExamination shows a stable gravida with normal vital signs. Abdominal examination shows big for date uterus with two cephalic poles in the lower abdomen and positive fetal heartbeat. Standard ultrasound examination confirmed twin pregnancy with both in cephalic presentation and adequate amniotic fluid; single placenta with no visible dividing membrane; fetal heartbeat is visible at two sites and is in a normal range. Upon pelvic examination, the cervix is 8cm dilated with left occiput-anterior position at a station 0. Fetal membrane is ruptured with clear liquor passing. With diagnosis of twin pregnancy (both cephalic presenting), in active phase of first stage of labor patient is admitted to labor ward and management of labor started in the standard way.\nIn the next few hours labor progressed well and the first baby is crowning. Duty midwives are attending the delivery. Subsequently, with maternal effort the head and upper extremities of the first baby are delivered and the remaining part of the fetus is delivered by ‘gentle' traction by the midwives. But after delivery of the whole body, baby 1 remained ‘attached' to the mothers' perineum, though the baby is crying vigorously (). The midwives started to shout for help and senior obstetrician arrived.\nOn reevaluation, we noticed the same and we found that the anterior abdomen of baby 1 from xiphisternum to the site of umbilical cord insertion is continuous into the uterine cavity. This led to sudden and unexpected consideration of the possibility of conjoined twins. Bedside ultrasound showed alive remaining fetus with fetal heart rate of 76 and in a transverse lie with the head in the right iliac fossa and fetal dorsum anterior. Initial attempt to access the extremities and aid delivery of the remaining fetus vaginally is not possible due to failure to reach the extremities for intrauterine manipulation. Emergency laparotomy is decided.\nEmergency laparotomy under general anesthesia with midline subumbilical abdominal incision and lower uterine segment vertical hysterotomy is performed. We corrected the lie of the born baby such that it is parallel to that of the unborn baby; with deep vaginal examination along with the caudal end of the attachment and managed to manipulate the lower extremities of the intrauterine fetus to vagina and after grasping those with the right hand vaginally, we brought it to the perineum. Then there is careful manipulation to bring those extremities posterior to the born baby with a second assistant holding and manipulating the born baby away from the area of manipulation. Progressive delivery of the second baby of the conjoined pairs is affected by total breech extraction with minimal difficulty.\nBoth newborns were depressed at completion of the procedure and recovered after aggressive resuscitation for 10 minutes (). Both are male and their combined weight is 5800 gm. Ultrasound examination of the twins shows shared liver with no other organs shared. Latter the second baby passed away after 1 hour of stay at the NICU. The second baby died after 20 hours of stay, during transportation to higher center for possible emergency separation. The mother was discharged to home on her sixth post-op day after counseling.
A 63-year-old woman was admitted to our hospital with serious headache and vomiting. Five months before admission, she had undergone surgery for a primary advanced gastric cancer at a local hospital. However, as the hospital went bankrupt, it was impossible for us to obtain details of her histopathological information. On admission, her consciousness level was Glasgow Coma Scale score 14 (E4, V4, M5). Her arms and legs showed no paralysis, and there was no history of head trauma. Blood examinations revealed mild anemia and a decrease in platelet count. However, this did not represent a state of disseminated intravascular coagulation (DIC). Head computed tomography (CT) revealed a crescent-shaped fluid collection in the right frontoparietal subdural space with a solid mass attached to the internal surface of the cranial bone. Similarly, magnetic resonance imaging (MRI) showed a tumor that projected into the subdural space and invaded the skull bone and dura mater (Fig. a, b). However, no metastatic lesions were observed in the brain and meninges. Local recurrence of gastric cancer and metastases were not confirmed. In addition, the presence of breast cancer, lung cancer, and gastrointestinal carcinoma was excluded based on a systemic search. We diagnosed metastasis from gastric cancer to the dura mater complicated by subdural fluid collection. However, the precise relationship between tumor and subdural fluid collection remained unclear.\nAt the first operation, burr-hole evacuation of the subdural fluid collection was performed under local anesthesia. The membrane was not present under the dura mater. The subdural fluid collection displayed a xanthochromic yellow transparence with viscosity. Furthermore, it solidified immediately at room temperature, compatible with Froin syndrome, and there was no evidence of hemorrhage. Cytological examinations revealed no blood cells or malignant neoplastic cells within the fluid collection. Although symptoms improved temporarily, serious headache reappeared, and disturbance of consciousness developed 2 days later. Head CT again demonstrated an increase in subdural fluid collection. Although the patient’s illness was already in its terminal phase, her family expected symptoms to improve. We therefore performed craniotomy under general anesthesia 3 weeks after the first operation in order to prevent recurrence of the subdural fluid collection and tumor removal.\nAt the second operation, the tumor was found to be growing in the periosteum, muscle, and subcutaneous tissue through the cranial bone, wide invasion of the tumor into the dura mater under the craniotomy was evident. The membrane was not observed in the subdural space. The subdural fluid collection displayed a yellowish transparence similar to that at the first operation. The tumor had not invaded the brain and meninges.\nHistological examinations of the dura mater revealed diffuse infiltration by malignant neoplastic cells. These cells exhibited a cribriform pattern and tubular formation, compatible with a diagnosis of metastatic, poorly differentiated adenocarcinoma of the dura mater (Fig. a). We were unable to compare this specimen with the gastric adenocarcinoma found at the previous hospital. However, we reached the definitive diagnosis of metastasis from the primary gastric cancer to the dura mater, because no malignant tumors in other organs were confirmed. Almost all vessels of the dura mater were infiltrated by numerous tumor cells (Fig. b). However, ruptured vessels and leakage of blood corpuscle components around the vessels were not found (Fig. a). On special staining, many tumor cells demonstrated positivity for Alcian blue, which is compatible with the presence of adenocarcinoma cells that produce mucus. Furthermore, mucinous material was observed in the stroma (Fig. c). These findings strongly suggested that the subdural fluid collection was a consequence of mucus secretion by the adenocarcinoma. After the second operation, the patient’s headache improved, but aggravation of her state of consciousness progressed again and she fell into a coma 2 weeks later. She died of DIC on day 78 after hospitalization. Permission for autopsy was not obtained.
A 50-year-old lady was admitted to hospital with multiple non-specific symptoms which included generalised weakness, numbness on both upper limbs associated with spasms and loss of appetite. She had a past medical history of cervical and lumbar spondylosis. She was not taking any prescribed or over the counter medications. Vitals were stable except for elevated blood pressure of 160/100 mmHg. Physical examination was grossly normal. She did not have any signs of hypercortisolism or virilisation.\nHer potassium was 1.7 mmol/L and sodium 144 mmol/L (Table ). ECG showed ST segment depression in leads V5 and V6. Urine potassium to urine creatinine ratio was elevated (>1.5) indicating urinary losses of potassium. She was started on potassium supplementation.\nThe full adrenal screen performed on this patient showed an elevated aldosterone to renin ratio.\nThe patient was sent for computed tomography (CT) adrenal scan which revealed a left adrenal mass of 6 cm × 5 cm with irregular edges and central calcification. However, the tumour density and contrast washout were non-specific and was not able to further aid in characterising the adrenal mass. A CT scan of the brain, thorax, abdomen and pelvis performed to detect any evidence of metastases was negative.\nDue to the size of large adrenal mass which raises the suspicion of malignancy, the patient subsequently underwent laparoscopic left adrenalectomy. The 6 cm × 5 cm adrenal mass was removed with adequate resection margin. Her blood pressure and potassium levels normalised after surgery and she remained clinically well. Histology of the adrenal mass showed tumour cells positive for synaptophysin and inhibin. The tumour cells were arranged predominantly in a trabecular pattern with some areas showing sheet formation. A few clear tumour cells were seen. The tumour cells had large nucleoli and were pleomorphic. Mitotic activity was 33/50 hpf. There were areas of focal necrosis with vascular, sinusoidal and capsular invasion. The histology fulfilled more than three aspects of the Modified Weiss criteria to support the diagnosis of adrenocortical carcinoma (Table ).\nThe patient was commenced on 3 g of mitotane daily which was gradually titrated in 1 g increments weekly to 8 g of mitotane daily in divided doses. At 8 weeks she achieved a plasma mitotane concentration of 16 mg/L. Hydrocortisone replacement was started at 10 mg twice a day. The adequacy of replacement was monitored using adrenocorticotrophic (ACTH) levels. She had CT scans 2 monthly to look for metastasis. A positive emission tomography (PET) scan done 2 months after surgery showed no suspicious focal fludeoxyglucose (FDG)-avid lesion. Unfortunately the patient presented 2 months later (4 months after surgery) with acute abdominal pain.\nCT scan showed ruptured hypervascular nodule along the omentum raising the possibility of a metastatic nodule. No further investigations could be performed as the nodule was not amenable to biopsy. Her abdominal pain resolved and she was closely monitored. CT scan done 10 months after the adrenalectomy raised possibility of disease progression with new pulmonary and liver nodules suspicious of metastasis. She then was referred to medical oncology and underwent radiotherapy for 2 months. Four cycles of chemotherapy (Cisplatin, Etoposide and Doxorubicin) was given. Further chemotherapy could not be given due to episodes of neutropenic sepsis.\nThe patient continued to deteriorate despite chemotherapy and was subsequently referred to palliative care. She died 28 months after her initial presentation to hospital.
A 57-year-old Asian man without relevant previous medical history was hospitalized following a fall from the 15th floor of a condominium. A witness said he had fallen from the condominium and had contacted electrical wires before hitting the ground. Prior to being transferred to the hospital, when the emergency medical service personnel arrived on scene, the patient had no pulse, but spontaneous circulation returned after 4 min of chest compression. On hospital arrival, the patient’s airway was maintained spontaneously, respiratory rate was 30 breaths/min, blood pressure was unmeasurable but the left femoral artery pulse was weakly palpable, heart rate was regular at 136 beats/min, body temperature was 36.1 °C, and Glasgow Coma Scale was E3V1M5. His right inner thigh was lacerated halfway around, and he was actively bleeding from this site. Blood analysis data on admission are shown in Table . We immediately initiated a massive transfusion protocol, secured the airway of the patient with endotracheal intubation and temporarily ligated the proximal exposed superficial femoral artery (SFA), deep femoral artery (DFA) just distal part after branching lateral femoral circumflex artery (LFCA), and superficial femoral vein separately. As we could not detect backflow from the distal artery, possibly because of intimal injury, spasm, or thrombus, and also as there was no time to expose this artery, we could not temporarily shunt it. As well, a tourniquet could not be applied because the injury site was too proximal to the torso. In parallel with preparations to initiate resuscitative endovascular balloon occlusion of the aorta via the left femoral artery, we packed the thigh and sequentially searched for other sites of bleeding because of the patient’s continued hemodynamic instability. Although we found no other sites of bleeding according to an extended focused assessment with sonography for trauma examination, subsequent plain pelvic radiography suggested the potential for retroperitoneal bleeding with iliac fracture. Considering the patient’s hemodynamics, we performed retroperitoneal packing by making suprapubic incision and entering preperitoneal space comprising the para-vesical and presacral space and then packing pads were placed.\nAfter this was completed, the patient was stable enough to undergo contrast-enhanced computed tomography (CECT). CECT showed fractures of the 4th lumbar vertebra and right iliac body concomitant with arterial extravasation at this site (Fig. a), so we performed transcatheter arterial embolization (Fig. b). Although CECT showed that the right SFA and DFA just distal part after branching LFCA were interrupted (Fig. ), narrow blood flow in the distal popliteal artery seemed to be maintained by collateral perfusion from LFCA as pulse doppler detected a weak pulse in the dorsal pedis artery. The ankle brachial pressure index at this time was approximately 0.54. After confirming good consciousness of the patient and resolving resuscitation-related conditions such as hypothermia and acidosis, we decided to perform revascularization of the femoral artery and vein by bypassing them with great saphenous vein grafts at approximately 24 h after the initial injury. The operation was performed with the patient in the supine position. Minor fresh thrombus was obtained from distal side of SFA and SFV by Fogarty catheter insertion and we could obtain sufficient back flow from both vessels. We decided to sacrifice the DFA and vein and bypass the SFA. The great saphenous vein was harvested from the left limb, and a graft of about 7 cm in length was anastomosed to the intact intima of the SFA (Fig. a). As the superficial femoral vein also appeared to be bypassable, we bypassed it with a great saphenous vein (Fig. b). All anastomoses were performed by use of 6-0 monofilament suture with the parachute technique.\nThe soft tissue was also severely injured with some loss of tissue apparent. We initially considered covering the anastomosed vessels with viable muscle, but as the tissue deficit was not completely uncoverable, negative pressure wound therapy was applied over the muscle tissue.\nThe patient was extubated on the 4th hospital day, and musculocutaneous grafting was performed 22 days after the initial operation following completion of negative pressure wound therapy. Postoperative CECT showed the patency of flow through the femoral vessels (Fig. ) and the score of ABI improved 1.15. As adjuvant therapy, although we could not administrate anticoagulant drugs prior to surgery on the basis of concomitant injury, systemic unfractionated heparin infusion was used during and post 10 days after bypass surgery. And then we converted to oral administration of warfarin and controlled the international normalized ratio of prothrombin time at the range of 2.0–2.5.\nThe patient was transferred to a rehabilitation hospital 52 days after admission without any symptom such as swelling or intermittent claudication.
A 29-year-old man with a history of exercise-induced asthma (not requiring treatment for 10 years) and obesity (body mass index 39) presented to the emergency room in respiratory distress with evidence of systemic inflammatory response syndrome. His history was pertinent for heavy e-cigarette use for the previous 2 years. Of note, he admitted that he previously smoked ~1–2 packs of cigarettes and 0.5 g of cannabis per day from ages 19 to 27 but quit both of those when he started vaping. He admitted to owning 12 different vaping pens, 8 of which were used for nicotine and 4 for THC. He used a variety of flavors and estimated that he went through a 30-ml bottle every 7–10 days. He also reported that 5 days prior to the onset of symptoms, he tried a new branded vaping product that contained THC. Two days prior to presentation, he experienced rigors with associated nausea and several episodes of emesis. Despite cessation from all vaping activities, he became increasingly dyspneic with shortness of breath with minor activity prompting him to seek emergent evaluation. Upon presentation, he demonstrated evidence of hypoxemic respiratory distress with severe tachypnea with increased work of breathing and mild accessory muscle use. He reported coughing bouts with deep inhalation and associated production of white frothy sputum. He denied hemoptysis, chest pain, recent illness, travel or sick contacts. His examination was significant for tachycardia, tachypnea and distant breathing sounds with fine crackles in bilateral lung fields. An electrocardiogram showed sinus tachycardia (heart rate 115) with no ST-segment changes. Chest radiograph showed diffuse pulmonary infiltrates bilaterally (). Laboratory evaluation demonstrated an elevated white blood cell count of 19.5 cells/μl (the cell differentials by percentage were all within normal range and there was no eosinophilia present). Troponin was negative, and both brain natriuretic peptide and D-dimer were not significantly elevated (82 pg/ml and 195 ng/ml, respectively).\nTherapy consisted of scheduled nebulized albuterol, methylprednisolone (30 mg intravenous [i.v.] given twice over 8 hours, and then changed to prednisone 40 mg b.i.d.) and empirical antibiotic treatment with azithromycin and ceftriaxone. The patient continued to have fevers (Tmax 101.6), worsening hypoxemia (requiring up to 12 l via high-flow nasal cannula) and leukocytosis.\nOn Day 2, the patient’s respiratory status worsened and an arterial blood gas (ABG) analysis revealed a pH of 7.48, pCO2 of 33 and a pO2 of only 48 while on 8 l O2 high-flow nasal cannula. He did meet the Berlin criteria for severe ARDS and therefore was transferred to the intensive care unit (ICU) for closer observation but never required invasive mechanical ventilation. A computerized tomography (CT) chest angiogram with i.v. contrast revealed findings significant for pneumonitis ().\nAt this point, a workup for an infectious source including blood cultures, urine legionella, antigen, urine antigen for Streptococcus pneumoniae, antibody screen for coccidiomycosis, human immunodeficiency virus and respiratory polymerase chain reaction panel was negative. Antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies, cytoplasmic & perinuclear and anti–double-stranded DNA antibody were also negative. An ABG showed respiratory alkalosis with hypoxemia. Acute phase reactants were elevated including: C-reactive protein of 243.93 mg/l, procalcitonin of 0.17 ng/ml and sedimentation rate of 35 mm/h. Antimicrobials were discontinued after 3 days of duration and supportive care continued including supplemental oxygen, steroids and albuterol nebulizer treatments. By the sixth day of his hospital stay, the patient’s symptoms had resolved and was able to ambulate without supplemental oxygen. He was discharged home with an inhaler and to complete a course of prednisone 40 mg daily for five more days. His vaping paraphernalia was submitted to the county health department and was subsequently found to indeed contain THC. His case was also reported to the Centers for Disease Control and Prevention. The patient followed up with his primary care provider within 2 weeks of discharge and no further treatment was indicated. Additionally, upon contacting the patient 8 months after his hospitalization, we were pleased to discover that he has quit using e-cigarettes and has not had any further episodes of respiratory distress or need for an inhaler.
We present the case of 32-year-old female patient who presented to the urologist with fever lasting for the previous two months, usually 37°C to 38°C, with occasional temperature leaps to 40°. Laboratory blood tests showed leukocytosis with cell counts up to 19,000. The patient did not complain of lumbar pain. Urinalysis showed leukocyturia, erythrocyturia, and slight positivity for protein. The patient was treated with a combination of amoxicillin, clavulonic acid, and cefuroxime. Abdominal ultrasound and multiphase CT depicted a large, expansive, heterogenous mass in the lower pole of the left kidney measuring 92 × 59 × 76 mm. CT scans showed a renal neoplastic mass with increased peripheral inhibition and poor central inhibition, possibly due to central necrosis. The tumor was unsharply demarcated from the surrounding parenchyma and pelvic system (). Regional lymphadenopathy was present, but there were no signs of propagation in the main renal vein or perinephric fat tissue, and there were no signs of liver or bone metastasis. Chest CT showed no signs of lungs metastasis or mediastinal lymphadenopathy. In order to determine the final histopathological diagnosis, histopathological and immunohistochemical analyses were performed, complemented by reverse transcryptase-polymerase change reaction (RT-PCR). Gross examination of the slitted specimen showed a sharply circumscribed tumor measuring 9 × 6 × 6 cm and replacing a large part of the kidney. The renal tumor displayed infiltrated parenchyma but no expansion into the pelvic system and ureter or signs of spreading into the main renal vein. Gross appearance of the tumor was white-yellow with large necrotic areas. The microscopic analysis of perinephric fat tissue, regional lymph nodes, and the left adrenal gland revealed no signs of metastasis. Microscopic analysis showed that the tumor was composed of solid nests and stripes of tumor cells with small oval and round nuclei, and some of the nuclei contained small nucleoli (). The tumor cell cytoplasm was scanty and pink with coarser appearance of chromatin. Mitotic figures were also found. Immunohistochemical analysis included MIC2 gene product/CD 99 (), neuron-specific enolase (NSE) (), CD3, CD 20, chromogranin, synaptophysin, vimentin, and neurofilament. Immunohistochemical staining revealed positive expression of CD99 and NSE in all tumor cells. The tumor cells did not stain with CD3, CD 20, chromogranin, synaptophysin, vimentin, or neurofilament. RT-PCR analysis for EWS-FLI1 fusion proteins was positive for type II 191 pb and negative for type I 125 pb. Diagnosis of rPNET set on the basis of characteristic morphological characteristics and results of immunohistochemical analysis was confirmed by RT-PCR. After nephrectomy, the patient received 14 cycles of a VAC/IE regiment adjuvant chemotherapy comprised of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Our patient was disease-free for nine years and eight months. Blood and urine laboratory test values were in the normal range.\nThe study was approved by the institutional ethical committee and conducted according to the principles of the declaration of Helsinki of the world medical association. The authors declare that they have no conflicts of interest. The authors did not receive any grants for writing this article.
We present the rare case of an otherwise healthy 56-year-old woman who was initially referred to our department due to incidentally found high levels of serum thyroglobulin (Tg) (2500 ng/ml, normal values: 1.4–78). The levels of the thyroid hormones (T3, T4), the thyroid-stimulating hormone (TSH), and the antithyroid antibodies were within normal range values. A neck ultrasonography (US) had already been performed describing a heterogeneous thyroid gland and bilateral small nodules with benign radiological patterns.\nA magnetic resonance imaging (MRI) of the neck was not diagnostic demonstrating no remarkable pathological findings of the thyroid gland.\nFurther investigation was followed to exclude an ectopic origin of Tg. A US of the abdomen followed by a computed tomography scan (CT) of the upper and lower abdomen was performed. A heterogeneous mass sized 11 × 9 × 9.9 cm was detected in the pelvis. The lesion had irregular cystic and solid components, findings highly suggestive of an ovarian teratoma. The tumor was contiguous with the left ovary with no signs of invasion to the neighbor organs. The right ovary showed no pathology, and the uterus had features of fibromatosis. No other intraabdominal pathology was described ().\nTaking into consideration these findings, surgical excision of the adnexal mass was the treatment of choice. Provided that Tg levels remained high postoperatively, the option of a complementary total thyroidectomy was also discussed with the patient. Given the patient's postmenopausal age, a laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was initially proposed. However, taking into account the patient's preference as well as the fact that no pathology of the right ovary was detected, a more conservative approach was finally decided. A laparoscopic left salpingo-oophorectomy en bloc with the irregular mass was performed. The tumor was removed intact, preserving its capsule and preventing any content spillage (). The patient's postoperative course was uneventful.\nMacroscopic examination of the tumor showed an irregular grey-yellow cystic neoplasm, filled with hair and fat-like tissue. Histopathological report set the diagnosis of a left ovarian mature cystic teratoma composed predominantly of thyroid tissue. Thyroid follicles were revealed in fibrous septa, and the immunohistochemistry result was positive for thyroglobulin. No remarkable findings were observed in the left fallopian tube.\nDuring a three-month follow-up period, the Tg blood levels were within the normal range. An additional US of the thyroid gland confirmed no remarkable findings. In accordance with the patient's desire, we recommended an annual follow-up including laboratory and imaging examinations avoiding any further surgical procedure.\nSix months later, the patient readmitted in our department due to a new remarkable increase in the Tg blood levels far above the normal (>1500 ng/ml). A CT scan of the lower abdomen demonstrated a 11 × 10.8 × 11.5 cm pelvic mass. The lesion was located centrally in the pelvis and consisted of solid and cystic compartments. Additionally, a limited fluid collection was also revealed in the right pelvis. No other pathology was detected ().\nAn early recurrence of the struma ovarii was suspected. Following written informed consent from the patient, an open total hysterectomy with right salpingo-oophorectomy was successfully performed. The pelvic mass was removed en bloc with the remaining genital organs. Neither rupture of the tumor nor intraperitoneal seeding was encountered. The patient had an uncomplicated postoperative period.\nThe pathologic report described a grey-yellow mass sized 12 × 8.5 × 7.4 cm totally occupying the right ovary. A nodular lesion with a maximal diameter of 0.4 cm was additionally detected at the uterine wall. The microscopic examination reported typical findings of an ovarian teratoma. Immunohistochemical markers of the thyroid tissue were found positive, and the definitive diagnosis of a benign struma ovarii was set. The right fallopian tube had a normal configuration and histology. Lastly, the nodular lesion of the uterus was found to be a small leiomyoma.\nDuring a one-year follow-up period, the patient is in a good clinical condition. Tg levels remain within the normal range, and consecutive US examination of the thyroid gland as well as of the lower abdomen has not revealed any pathology.
The patient is a 47-year-old Caucasian female who presented to the Emergency Department of an academic tertiary-care hospital in the Midwestern United States with complaint of left-sided weakness of the upper and lower extremities and right gaze preference three weeks after a right pontomedullary infarct complicated by Posterior Reversible Encephalopathy Syndrome (PRES) [that initial infarct had been treated in a different state]. Imaging revealed an acute infarct in the posterior limb of the right internal capsule without hemorrhagic transformation and an acute punctate infarct in the right parietal subcortical white matter with corresponding diffusion restrictions, as well as remote evidence of subcortical chronic diffuse microhemorrhages (). The Psychiatry Consultation & Liaison service was consulted on hospital day 2 after the patient reported, “I want to strangle myself with my oxygen cord.”\nOn initial evaluation, the patient reported history of anxiety treated previously by her primary care physician (PCP). She reported she had been frustrated with her medical condition but really did not intend to harm herself. She reported fluctuating mood since her initial stroke and had “good days and bad days.” She denied prior history of inpatient or outpatient psychiatric care or prior suicide attempts. She was oriented to person and place, but not time, was able to state the days of the week forwards, but not backwards, and endorsed visual hallucinations during her hospitalization. This presentation was felt to be consistent with delirium, and she was started on quetiapine 25 mg.\nFollowing a six-day medical admission, the patient was discharged to the acute inpatient rehabilitation unit housed within the hospital. Extensive diagnostic studies did not reveal an underlying etiology for the strokes, which were thought to be due to uncontrolled hypertension.\nPsychiatry was reconsulted by the rehab physicians for management of problematic behaviors. The patient exhibited ego-dystonic behaviors for which she would later apologize including repeatedly climbing out of bed, shouting for nursing assistance without clear need for help, shoving her fist into her mouth to induce vomiting, and periodic, purposeless screaming. These behaviors were disruptive to staff and other patients on the unit. While initially conceptualized as residual hyperactive delirium, her behaviors persisted and continued testing for underlying causes of delirium including electrolyte derangement, occult infection, new or evolving cerebrovascular event, or excess medication burden which were unrevealing\nAfter 60 days of acute rehab, she had reached maximal benefit of that intervention and continued exhibiting behaviors incompatible with nursing home disposition. The patient was then transferred to the university's geriatric psychiatry inpatient unit on an involuntary mental health commitment for behavioral management.\nIneffective medication trials prior to transfer included quetiapine (25 mg at bedtime and 25 mg several times daily as needed), mirtazapine (7.5 mg at bedtime), olanzapine (initial trial of 2.5 mg at bedtime and 2.5 mg several times daily as needed and a second trial of 15 mg and 2.5 mg several times daily as needed), buspirone (15 mg TID), divalproex (initial trial of 750 mg at bedtime and a second trial of 500 mg TID with lactulose and levocarnitine for hyperammonemia), melatonin (9 mg at bedtime), propranolol (40 mg QID), trazodone (150 mg at bedtime), gabapentin (200 mg several times daily as needed), dextromethorphan (20 mg BID, given as Robitussin), and clonazepam (0.5 mg AM and 1 mg PM).\nThroughout this period, the patient remained intermittently apologetic for her behaviors. Orientation was typically attuned to person, sometimes place, and generally not to month or year. She consistently denied depressed mood, anxiety, visual hallucinations, auditory hallucinations, paranoia, suicidal ideation, or homicidal ideation. Thought process remained concrete and perseverative with limited spontaneous speech output and paucity of thought content. Language remained intact without evidence of aphasia. Recent and remote memory were difficult to assess formally due to behavioral disturbance, but she had difficulty remembering recent details of her hospital course and remote details of her life prior to moving to her current city. She required staff assistance for completion of toileting, dressing, and feeding. She had deficiencies in executing complex motor tasks, such as getting out of bed, and was frequently found diagonal in bed with a limb tossed over the side-rail. These deficiencies were in excess of the residual motor effects of her strokes and suggestive of alterations in visuospatial skills, executive function, and planning. Her aberrant vocalizations did not appear goal-oriented and were not ameliorated by staff presence. This presentation persisted and was thought to represent a new cognitive baseline meeting diagnostic criteria for major vascular neurocognitive disorder with behavioral disturbance.\nNonpharmacological strategies including music, sensory stimulation, one to one time with staff, and frequent repositioning were tried without improvement in her symptoms. Additional ineffective medication trials following transfer to inpatient psych included fluoxetine (60 mg per day), retrial of dextromethorphan with fluoxetine as an enzymatic inhibitor (again to 20 mg BID), retrial of quetiapine (up to 600 mg total per day), haloperidol (5 mg several times daily as needed IM), oxycodone (5 QID), lorazepam (up to 6 mg daily), carbamazepine (200 TID), and chlorpromazine (50 QID). Throughout these trials, the patient continued to exhibit frequent periods of severe psychomotor agitation requiring vest restraint and purposeless screaming alternating with periods of oversedation following medications. Other than providing intermittent sedation, no particular combination of medications proved effective in treating the target symptoms.\nAt this point, having exhausted all reasonable behavioral and pharmacologic options, the inpatient psychiatric team recommended ECT as a last intervention prior to pursuing a palliative approach. Medical Ethics was consulted and felt ECT to be consistent with her previously articulated beliefs and wishes.\nThe patient was formally evaluated by the ECT service and, given her incapacity to consent, a court order was obtained for the procedure. She underwent an acute course of bitemporal ECT using a MECTA Spectrum 5000Q machine. She received methohexital and succinylcholine as anesthetic and relaxant agent, respectively. A dose-titration method was used to determine stimulus intensity. She received treatments at 50% over seizure threshold with the following parameters: pulse width: 1 millisecond, frequency: 20 Hz, duration: 2 sec. Treatments were given three times per week. She was maintained on chlorpromazine (50 mg QID) and lorazepam (1 mg QID) during the treatments. Following the sixth ECT treatment, the patient rarely engaged in purposeless yelling, and remained quiet most of the day, experienced normalization of her sleep wake cycle, but still exhibited purposeless movements and psychomotor agitation requiring a vest restraint at night.\nFollowing the third week of ECT treatments, she was consistently having low scores on the Pittsburgh Agitation Scale (PAS) and had minimal requirements for as needed medications for agitation []. While she still required a vest restraint overnight, her psychomotor agitation had improved dramatically. She resumed feeding herself with her right arm and tolerated pureed foods for the first time in six months. Following an acute course of 16 treatments, ECT was tapered to twice weekly and she started sertraline 25 mg in preparation for further decrease in ECT frequency. She remained stable and was successfully discharged to a nursing home with continuation of ECT as an outpatient. Following the expiration of the original court order for ECT, outpatient ECT was discontinued and the patient's family chose to not pursue a renewal of the order for continued treatment. She received 29 treatments in total. Nursing home staff reported that her behaviors remained in control after stopping ECT and she was thereafter able to return home with her parents.
A 24-year-old male presented with persistent, severe abdominal pain and gangrene of his toes for 6 months. His history dated back to 1999 when he was 8-year old and he had tender macular lesions over legs, abdominal pain, weight loss, testicular pain, persistent fever and right ulnar palsy. There was no past history of any comorbid illness. Serum inflammatory markers were raised and HIV and hepatitis B surface antigen (HbsAg) were negative. Orchidopexy was performed for suspected torsion of right testis which failed to relieve the pain. PAN was suspected on clinical grounds by a pediatrician and conventional angiography of abdominal vessels and CT scan of abdomen was normal. After treatment with prednisolone and oral cyclophosphamide, his symptoms subsided and after four months he stopped the treatment. He had a long remission until February 2003, when abdominal pain recurred. This was managed conservatively and disease went into remission after a few days.\nIn February 2007, disease relapsed with left testicular pain. Orchidopexy was done on the left side and testicular biopsy showed left testicular atrophy and acute orchitis without arteritis. It was managed conservatively and symptoms remitted after a few weeks. Diagnosis of PAN was not suspected due to lack of objective findings and spontaneous resolution of symptoms.\nIn November 2014, 6 months before presenting to us, he had fourth relapse with abdominal pain and pain in toes of both feet. This time the abdominal pain was very severe with marked anorexia and progressive loss of weight. Video colonoscopy, jejunal biopsy and CT angiography of abdomen was normal and capsule endoscopy revealed jejunal and ileal ulcers. PAN was reconsidered and treatment with prednisolone and azathioprine was started. Symptoms showed no response and abdominal pain continued unabated. Thereafter dry gangrene of toes of both feet developed. Anti-nuclear antibody (ANA), Anti-neutrophil Cytoplasmic antibody (ANCA), anti-ENA and HbsAg were repeatedly negative. Serum lead and cryoglobulin levels were normal. Intravenous cyclophosphamide pulse was given but he did not tolerate it so it was discontinued. He was treated with various pain management measures including NSAIDs, tramadol, pregabalin, amitryptiline and buprenorphine patches but to no avail.\nHe presented to our center with a body weight of 24 kg. Toes of his both legs and fingers were gangrenous (Fig. ). Because of his earlier history of intolerance to intravenous cyclophosphamide (CYC) infusions, oral CYC 50 mg/d was started along with prednisolone. Since intensity of abdominal pain was not consistent with repeatedly normal colonoscopy and angiographic findings, a major functional component was considered as an explanation. Psychotherapy and antidepressants proved futile. However, after a month he developed acute peritonitis because of gangrene and perforation in multiple segments of ileum and colon. Right hemicolectomy, small bowel resection and ileocolostomy were performed. CT angiography of the abdomen showed multiple splenic infarcts, bilateral renal cortical infarcts and microaneurysms of terminal branches of abdominal aorta (Fig. ). For the first time angiographic evidence of the disease was seen. Ironically, the histopathology did not reveal evidence of vasculitis in the resected specimen. Postoperatively, intravenous immunoglobulin (IVIG), 0.4 mg/kg/d was given for 5 days. In spite of surgery and IVIG, pain in his abdomen, fingers and toes continued. After 15 days, he developed cerebellar abscess and large hydrocephalus (Fig. ). Urgent suboccipital craniotomy with abscess drainage and extraventricular drainage was done. However, he did not recovery and succumbed to his illness.
A 65-year-old male was admitted to the emergency department with acute shortness of breath and chest discomfort. The symptoms were gradually progressive over the past few days. He had an episode of acute upper respiratory infection in the past week and was on decongestants for symptoms of cold and congestion. The patient tested negative for influenza A and B. He had a significant medical history of mitral valve prolapse, benign prostatic hyperplasia, and hernia repair. He had no significant history of smoking, alcohol or drug abuse. Upon examination, the patient’s vitals were within normal limits. The patient was not overweight and had a bodyweight of 72 kg. The jugular venous pressure was normal. The heart rate was regular and in normal sinus rhythm. A soft ejection systolic heart murmur was present. The breath sounds were present and were equal on both sides of the chest.\nThe lab results were significant for brain natriuretic peptide (BNP) levels elevated up to 407 pg/ml (normal up to 100 pg/ml), normocytic normochromic anemia, leukocytosis with increased neutrophils, and mild thrombocytopenia consistent with bacterial pneumonia. However, blood cultures were negative, likely due to empiric antibiotic treatment. The BNP levels returned to normal after the valve replacement.\nThe electrocardiogram (ECG) showed normal sinus rhythm with premature atrial contractions, prolonged QT interval, and ST depression followed by subsequent elevation in the lateral leads. No arrhythmias were present. The chest X-ray showed bilateral interstitial infiltrates. The computed tomographic angiogram (CTA) of the chest showed diffuse bilateral airspace opacities, consistent with multifocal pneumonia (Figure ). Small bilateral pleural effusions were present.\nNo aortic aneurysm was seen. Pulmonary embolism was ruled out. There was borderline pulmonary artery enlargement reflecting a degree of pulmonary artery hypertension. A potential right renal parapelvic cyst was identified. The patient was admitted for treatment and further testing. The treatment for bilateral pneumonia was started with oxygen through high flow nasal cannula and antibiotics including ceftriaxone and azithromycin. The patient was started on heparin to provide prophylaxis for deep vein thrombosis.\nIn light of his previous significant history of mitral valve prolapse, a transthoracic echocardiogram (TTE) was performed that showed a normal-sized left ventricle with concentric mild left ventricular hypertrophy as well as mild right ventricular and moderate right atrial dilatation. The left ventricular ejection fraction was found to be normal (0.6-0.65). He had grade III diastolic dysfunction with a restrictive pattern, consistent with markedly increased left atrial pressure. No left atrial mass or thrombus was visualized. There was mild mitral valve thickening with a flail posterior mitral leaflet. There were no vegetations on the mitral valve. No rupture of chordae tendineae was noted. The mitral regurgitant jet was anteriorly directed consistent with posterior leaflet pathology. There was severe mitral valve regurgitation as well as mild tricuspid and aortic valve regurgitation. There was no pericardial effusion. However, severe pulmonary hypertension was noted; the coronary angiography revealed normal coronary arteries with right coronary artery dominant circulation in the heart. The aortic root was normal in size.\nThe patient’s medications during hospitalization included aspirin, famotidine, furosemide, and acetaminophen. The mitral valve replacement was done. The post-procedure echocardiography confirmed the bioprosthetic valve in place. No valvular or para-valvular regurgitation was seen. The left ventricular function was normal. The patient had normal sinus rhythm post-procedure upon ECG. The patient recovered and was discharged in good condition on postoperative day seven without any complications.\nThe native mitral valve was received in the pathology department for histopathological examination. The macroscopic examination of the valve showed two valvular cusps measuring 3.0 x 2.0 x 0.1 cm each along with multiple separate pieces measuring 0.3 x 0.2 x 0.2 cm, which included chordae tendineae measuring 2.5 x 2.0 cm. The surface of the cusps showed yellow nodules with focal calcifications. No other gross abnormalities were noted. The microscopic examination revealed aggregates of mature adipocytes in the spongiosa layer of the valve, replacing the collagen and elastic fibers (Figures -).\nThere was no extension of adipocytes to the valve annulus. Calcific atherosclerosis was seen, but there were no foamy macrophages or vegetations. The immunohistochemical stain for S-100 was positive and showed adipocytes (Figure ).
A 26-year-old right-dominant man sustained a complex injury to the palmar surface of his right hand in a rollover motor vehicle accident in April 13, 2015, and he was treated at an outlying hospital. The thenar musculature and its motor branch were crushed. The flexor digitorum superficialis to the index was avulsed. Segmental losses of the digital nerves to the ulnar index and radial middle fingers were noted. He sustained zone 2 lacerations of the flexor digitorum profundus to the index finger and of both flexor tendons to the middle finger. The first reconstructive procedure addressed the tendons. The lacerations were repaired, and the thumb required a transfer of the extensor indicis proprius to the abductor pollicis brevis. At a subsequent procedure, nerve repairs were performed with 4 cm interposition sural nerve grafts. Coverage consisted of a volar forearm fasciocutaneous flap, the pedicle for which was a single perforator 2 cm proximal to the radial styloid, dissected in the suprafascial plane. The flap was rotated in-plane 180 degrees and inset. The donor site was covered with a split thickness graft from the thigh. By postoperative day (POD) 5, swelling of the flap was noted. By POD 14, the skin paddle was black and the patient was transferred to our institution ().\nBased on previous reports of autologous fat grafts for burn scars, we had previously utilized subcutaneous and periarticular infiltration of SVF for the treatment of burn scar contractures of the hand and were able to document neovascularization beneath the skin grafts and around the metacarpophalangeal (MP) joints. These findings were consistent with previous reports of angiogenic properties of SVF, the rationale being the known production by such cells of VEGF. With the possibility of using SVF transfer to assist in wound healing, we obtained informed consent and the patient was brought to surgery. The flap was debrided down to the adipofascial layer, which was noted to be pale with little signs of vascularity ().\nSVF cells were prepared from 370 mL of adipose tissue harvested directly into a sterile tissue processing canister (The GID Group, Inc., SVF-1, Louisville, CO, USA). The lipoaspirate was washed, and then underwent collagenase digestion. After centrifugation, the SVF cells concentrated at the bottom of the device, and were removed using a 6-inch needle. The total viable SVF cell count, 6.8 × 107, was obtained using image cytometry. The cells were suspended in 30 cc of Ringer’s lactate and injected into the substance of the adipofascial flap, into the subjacent palmar tissues, and beneath the previously healed split thickness skin graft covering the forearm donor site.\nWith the hope that the SVF-induced neoangiogenesis could help the remaining tissue of the flap to survive, we injected SVF into the subcutaneous border surrounding the fascia, the palmar tissues subjacent to the fascia, and the substance of the fascia itself. We also injected SVF into a zone of dense scar crossing the volar wrist crease and beneath the skin graft covering the volar forearm donor site.\nOn POD 3 (from the SVF procedure), the surface of the flap demonstrated diffuse neovascularization. The wound measured 10 cm × 3.5 cm (). With subsequent local debridement and saline dressing changes, the width had diminished to 2.5 cm by POD 10 () and it was completely closed by 21 days (), when the scar crossing from the volar forearm donor site to the palm remained dense at the wrist crease, making it impossible to extend the hand into neutral. The patient then proceeded into rehabilitation.\nThe initial rehabilitation evaluation on June 30, 2015 showed the following ranges of motion (in degrees, right hand vs. left hand): wrist extension 20/48, wrist flexion 70/123, index finger flexion 70/90 at the MP and 45/110 at the proximal interphalangeal joint (PIP), and middle finger flexion 90/90 at the MP and 70/110 at the PIP degrees. The patient could not perform composite flexion. The volar forearm skin graft was dry and stiff.\nThe initial rehabilitation evaluation on June 30, 2015 showed:\nWrist extension: right hand 20 degrees; left hand 48 degrees\nWrist flexion: right hand 70 degrees; left hand 123 degrees\nFinger range of motion: (1) right index finger, MP 70 degrees and PIP 45 degrees; (2) right middle finger, MCP 90 degrees and PIP 70 degrees\nVolar forearm skin graft: able to pinch the skin\nThe procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national, National Autonomous University of Nicaragua-Léon) and the Helsinki Declaration of 1975, as revised in 2000. Informed consent was obtained from the participant in this case study. The patient provided consent to publish this case study in all formats.\nUnder general anesthesia, a wetting solution (1 L Ringer’s lactate, 50 cc lidocaine 1%, and 1 cc epinephrine 1:1000) was infused into both the superficial and deep compartments in the zones designated for liposuction through incisions made with a #11 blade using a standard multiport infusion cannula. Liposuction was performed using a 4 mm Mercedes cannula with 370 mL of adipose tissue harvested directly into a sterile tissue processing canister (The GID Group, Inc., SVF-1, Louisville, CO, USA). The lipoaspirate was washed three times inside the canister to remove red cells and oils. Approximately 125 ml of Ringer’s lactate solution was added to the adipose tissue with collagenase enzyme (Worthington Biochemical Corporation, CLS-1, Lakewood, NJ, USA) at a concentration of 200 CDU/ml of total volume. The collagenase was injected into the canister through a sterile 0.22-micron filter (Millex-MP, Millipore, Cork, Ireland). The mixture was dissociated for 40 minutes in an incubated shaker table at 38°C and 150 RPM. After dissociation, human albumin was added to achieve a concentration of 2.5 and thereby stopping the reaction through the binding by albumin of calcium and magnesium ions essential for enzyme function. The mixture was centrifuged for 10 minutes at 800x gravity. The SVF cells concentrated at the bottom of the device, and were removed using a 6-inch needle (Abbocath-T, Hospira Ireland, Sligo, Ireland). The cells were resuspended in Ringer’s lactate solution to a total of 20 cc. Ten microliters of SVF were taken from the final suspension and submitted for differential staining. Two samples were then passed through an image cytometer (ADAM-MC, Portsmouth, NH, USA) for the counting of mononucleated cells and to assess cell viability. The viable index was 184,000 cells per gram of dry fat. The total viable cell count was 6.8 × 107.\nThe hand therapy regimen consisted of passive motion, active motion, and desensitization twice a week.\nContrast agent was injected into the left arm and allowed to circulate. Images were taken in the sagittal plane of the right index finger to correlate with the plane of the flap.\nThe rehabilitation evaluation was conducted on September 8, 2015, which was 4 months after the application of SVF cell therapy, showed that the cutaneous tissues of the palm were pliable. This was remarkable, because, given the length of time involved in the reconstruction, we were expecting a great deal more fibrosis and possible involvement of the flexor tendons. The scar crossing the volar wrist crease had softened by a considerable degree, allowing for increased wrist extension. Sensation was restored to the radial middle finger and proximal ulnar index finger but not yet to the pulp of the index. Full composite flexion has been achieved. There is a 10-degree loss of extension at the PIP level of the middle finger. The patient gained control and strength of the thumb through the tendon transfer with restoration of abduction in all planes, and opposition pulp-pulp to all fingers. The strength of the key pinch and chuck pinch continued to improve. The donor site skin graft demonstrated suppleness to pinch, with wrinkles forming with normal motion ().\nThe final rehabilitation evaluation at 6 months showed the following changes in range of motion (in degrees, right hand only):\nWrist extension: right hand 48 degrees (previously 20 degrees)\nWrist flexion: right hand 110 degrees (previously 70 degrees)\nIndex finger flexion: unchanged, will need z-plasties\nMiddle finger flexion: MCP 90 degrees (unchanged) and PIP 90 degrees (previously 70 degrees)\nFull composite flexion achieved\nThumb: restoration of full chuck and key pinch, with a full range of motion\nVolar forearm skin graft showed significant improvement in overall softness and elasticity ().\nIn the 3D angiography CT scan, the contrast agent disclosed a separate layer of enhancement in the immediate subcutaneous tissues of the right palm and wrist. Vessels were observed to be in continuity with the palmar arch and with the digital arteries of the index finger (video, available as Supplementary Material online at ).
A 63-year-old African American male initially presented to the hospital with an elevated prostate-specific antigen (PSA) level of 9.41 ng/mL. The PSA from a year prior was normal at 0.80 ng/mL. The patient’s symptoms included occasional dysuria and infrequent nocturia. The patient denied any other lower urinary tract symptoms, hematuria, abdominal or pelvic pain, bowel abnormalities, hematochezia, weight loss, night sweats, or loss of energy. The digital rectal exam at the time revealed a large volume prostate with induration along the left side of the gland.\nThe patient was scheduled for a prostate biopsy. However, about two weeks after his initial visit, he presented to an outside hospital with complaints of urinary retention. He was found to have acute kidney injury from progressing bladder obstruction due to a combination of benign prostatic hyperplasia and his malignancy. A urinary Foley catheter was placed, but the patient quickly developed hematuria due to traumatic catheter insertion. When this did not resolve, he presented to this institution for further evaluation, during which time the patient denied any further subjective complaints. His kidney function eventually improved, and the patient subsequently underwent a transrectal ultrasound with a 12-core needle biopsy of the prostate. During the exam, the prostate appeared to have irregular prostatic tissue extension from the left base of the prostate. Pathology revealed small cell neuroendocrine carcinoma that was present bilaterally in the base, mid-prostate, and apex of the prostate gland (12 out of 12 cores positive) (Figures -). There was evidence of perineural invasion. Immunostains showed cytokeratin and synaptophysin positivity, with a weakly positive PSA.\nShortly after his biopsy, the patient again presented to the hospital with worsening renal function. Ultrasound at this time revealed bilateral hydronephrosis. A CT of the abdomen and pelvis showed a prominent nodule in the inferior aspect of the prostate measuring 4.2 cm by 5.0 cm by 4.2 cm, which extended posterolaterally to the right (Figures -). There was evidence of invasion of the left anterolateral aspect of the rectum and the bladder anteriorly. There were also two markedly enlarged right pelvic sidewall lymph nodes lateral to the external iliac vessels, measuring 2.2 cm by 3.3 cm and 1.8 cm by 2.7 cm, respectively. Besides, there was evidence of disseminated sclerotic osseous metastasis across the axial and appendicular skeleton representing prostate cancer metastasis. Lastly, there was confirmed moderate bilateral hydronephrosis with ureteral prominence. He next underwent bilateral percutaneous nephrostomy (PCN) pigtail catheter placement, and then received his first cycle of carboplatin [area under the curve (AUC) 5] with etoposide (100 mg/m2). Soon after this, the patient developed gross hematuria from his right PCN and associated anemia, all of which ended up resolving in a week. A follow-up CT of the abdomen and pelvis two weeks after his first cycle of chemotherapy showed worsening of the numerous osteoblastic lesions scattered throughout the osseous structures, which was consistent with osteoblastic metastatic disease. The right pelvic lymphadenopathy also progressed in the given interval. The patient was planned for the second cycle of chemotherapy; however, after extensive discussion with him and his family, the patient decided to pursue hospice care. The decision to opt for hospice care was made in just over three months from the time of diagnosis.
A 78-year-old man presented to an ophthalmology clinic complaining of a gritty sensation in both eyes for several months, with exacerbation in the left eye over the past 2 weeks. He had a history of diabetes mellitus treated with oral hypoglycemic drugs and chronic obstructive pulmonary disease. He was diagnosed with background diabetic retinopathy. Examination using a Snellen chart showed a visual acuity of 6/12 in both eyes, meaning that he would need to approach to a distance of 6 meters to read letters that a person with normal acuity would be able to read at 12 meters. The patient noticed pain and swelling of his left eyelid at that time, and developed headache and vomiting 1 week later. Five days before admission, he developed patchy erythema with clustered vesicles with varying sizes on his left forehead and upper eyelid. On the day of admission, he developed drooping of the left upper eyelid, and was admitted to the infectious diseases ward with a diagnosis of HZO in the distribution of V1 on the left side, complicated by superficial punctate keratitis.\nThe patient was initially treated with intravenous acyclovir (500 mg every 8 hours), topical antiviral ointment (acyclovir 3%), and non-steroidal anti-inflammatory drugs to relieve the pain caused by severe swelling and erythema of his left upper eyelid. On day 4 of admission, the inflammation of the left upper eyelid had resolved, but he was still unable to elevate the eyelid. Physical examination showed markedly crusted skin lesions in the distribution of V1 on the left side, with a positive Hutchinson’s sign and complete levator paralysis. Examination of the extraocular movements with the eyelids held open showed near-complete ophthalmoplegia on the left side (Figure a). His visual acuity was 6/12 on the right side and 3/60 on the left side. He had anisocoria with the left pupil, which was poorly reactive to light and accommodation, measuring up to 6 mm in diameter. He was diagnosed with OAS, and brain magnetic resonance imaging (MRI) on day 5 of admission showed swelling of the left periorbital tissues and left-sided exophthalmos, with no evidence of a retro-orbital space-occupying lesion. Orbital fat-suppressed MRI on day 6 of admission showed orbital myositis and enhancement of the optic nerve sheath (Figure a).\nThe patient was diagnosed with HZO complicated by OAS, and systemic steroid therapy was initiated on day 6 of admission. Adduction and supraduction of the left eye started to improve on day 3 after the initiation of steroid therapy. He received intravenous acyclovir (500 mg every 8 hours) for 15 days, and oral prednisolone (25 mg twice daily; >1 mg/kg per day) for 4 days followed by 12 weeks of prednisolone therapy that was tapered at monthly visits. The patient was discharged after completion of the acyclovir therapy. His visual acuity recovered to 6/12 on the right side and 6/30 on the left side, and his extraocular movements gradually improved, with residual limitation of abduction and paralysis of the left upper eyelid at the completion of the steroid therapy (Figure b). Follow-up at 180 days after the onset of symptoms showed persistent limitation of abduction and paralysis of the left upper eyelid. Repeat orbital MRI at 180 days showed no changes to the orbital lesions compared with the MRI findings on day 6 of admission (Figure b).
A 38-year-old African American female presented to the emergency department for chest pain. The patient's past medical history was significant for hypertension, for which she had not taken her lisinopril for about 2 years, and diabetes, controlled with diet. Additionally, the patient had a history of smoking for 20 years and had quit about 3 months prior to admission; however, 1 week prior to the onset of chest pain, the patient was started on oral bupropion 150 mg every morning for smoking cessation for tobacco cravings. In the emergency department, the patient received aspirin chewable 162 mg by mouth and 3 sublingual nitroglycerin tablets, which relieved the chest pain, and was admitted to the general medicine floor in order to rule out an acute coronary syndrome. The next day, prior to her stress test, the patient went to the cardiology clinic, where she became confused and began talking about demons and auras. The patient's husband noted that this behavior was strange, and the patient was escorted back to the medical unit. On the medical floor, the patient was requesting to be examined by a female because having a male examine her would be “inappropriate” because “together (they) are the body of Christ.”\nThe patient was evaluated by psychiatry, and the patient's husband provided further insight into the patient's bizarre behavior. According to the patient's husband, the patient started acting strange when she woke up the morning before coming to the emergency department. She was hyperverbal, religiously preoccupied, and distracted with flight of ideas. The husband also reported that the patient had similar manic symptoms about 5 years prior after using cannabis and was hospitalized for a brief period at a private hospital. Per the husband, the symptoms resolved, and the patient was discharged with a diagnosis of schizophrenia. The patient was treated with oxcarbazepine 300 mg per day, risperidone 1 mg twice daily, and fluoxetine 20 mg daily, and it appears that the patient took these medications for approximately 6 months following this previous hospitalization. Because of the current sudden changes in mood, the patient was admitted to the inpatient mental health floor for mood stabilization. While in the inpatient psychiatry unit, the patient continued to exhibit behaviors consistent with manic delirium, such as agitation, hypersexuality, and combativeness, requiring multiple doses of intramuscular haloperidol 5 mg and lorazepam 4 mg, given as needed. The patient was started on lithium 600 mg twice daily, and appropriately drawn lithium trough levels increased up to 1.74 mEq/L due to acute worsening of renal function. However, the patient was without side effects or any resolution of symptoms. The patient's mania and psychosis continued to worsen despite trials of haloperidol 10 mg twice daily and risperidone 1 mg at bedtime, and ultimately the patient required restraints as she was no longer responding to intramuscular haloperidol 10 mg, lorazepam 2 mg, or olanzapine 10 mg. After 10 days of admission on the mental health unit, lack of response, and dangerous behaviors, the psychiatry team resolved to perform emergency ECT. After the first ECT treatment, the patient remained agitated and delusional and continued to require restraints and as needed haloperidol and lorazepam. She received her second treatment of ECT the following day, which she tolerated.\nThe psychiatry team resolved to admit the patient to the intensive care unit (ICU) for sedation to avoid injury with a goal of 24 hours without restraints and intravenous medications before being transferred back to inpatient psychiatry. The patient was admitted and placed on a dexmedetomidine infusion with haloperidol and lorazepam as needed for agitation, which the patient continued to need during the initial ICU course. The patient's third ECT resulted in some improvement in her behaviors. The patient remained in the ICU for a total of 6 days, and during the last 2 days of the ICU stay, the patient was not sedated and did not require any medication or restraints. The patient was stable and not psychotic or manic, so she was discharged home after her fourth ECT with instructions to follow up with outpatient psychiatry. Postdischarge, the patient received 4 more ECT treatments. Three months postdischarge and 2 months post-ECT, the patient remained stable without any pharmacologic treatment and only psychotherapy.
A 65-year-old man was diagnosed with pulmonary adenocarcinoma, then treated with chemotherapy. The cancer metastasized, and the patient was diagnosed with end-stage lung cancer. Cancer pain was treated with oral opioid therapy. Metastasis of the cancer caused obstructive jaundice and cholangitis, and the patient experienced septic shock. The attending physician performed emergent endoscopic biliary stent placement, in order to treat obstructive jaundice. The patient was medicated with continuous infusion of noradrenaline (0.2 μg/kg/min) through a peripheral vein, in order to maintain blood pressure. He underwent repeated placement of peripheral venous catheters, such that it became difficult to identify adequate peripheral veins for placement and maintenance of indwelling catheters. In the context of this treatment, the patient's congestive heart failure continued, which caused ascites and edema of the lower extremities.\nThe patient experienced orthopnea and could not sleep at night. The attending physician requested that the anesthesiologists secure a reliable venous line. The anesthesiologists checked the patient's groin and thighs; they excluded the femoral vein as a candidate for central venous access due to massive edema of the thighs. The anesthesiologists attempted to allow the patient to assume the supine or reverse Trendelenburg position; however, these positions caused worsening of the patient's dyspnea. The patient could solely tolerate the sitting position. The anesthesiologists then discontinued attempts to catheterize via the internal jugular and subclavian veins, because of the risk of air embolism during central venous catheterization of the patient in the sitting position.\nThe right subclavian vein was suspected to exhibit narrowing, based on computed tomography scan images (Fig. ). Thus, the anesthesiologists chose the left arm for insertion of a PICC, and found that the medial brachial vein exhibited sufficient size (diameter 4 mm) to place a PICC with ultrasound examination. The anesthesiologists allowed the patient to assume the sitting position, and placed the patient's arm on an over-bed table. PICC placement (4.5 Fr, double lumen, Argyle PICC Kit, Nippon Covidien, Inc., Tokyo, Japan) was performed using ultrasound guidance (6-15 MHz, SonoSite Edge, SonoSite Japan Co., Tokyo, Japan) with maximal sterile barrier precaution (Fig. ). Cannulation was performed without complications. The technique is summarized as follows (Fig. ).\nThe patient was allowed to sit on chair with a backrest.\nThe patient's stability was ensured (e.g., with aid from an assistant or nurse).\nThe patient's arm was raised and cleaned with disinfectants (1% chlorhexidine alcohol).\nThe adjustable over-bed table was covered with a disinfected drape.\nThe patient's arm was placed on the table.\nThe arm was allowed to abduct approximately 90° via adjustment of the height of the table.\nThe operator wore a cap, mask, and surgical gown (maximal sterile barrier precaution).\nThe assistant placed a towel between the drape and table to ensure that the medial side of the patient's arm faced upwards.\nThe ultrasound probe was covered with a sterile plastic cover.\nLocal anesthetic (1% lidocaine 3 mL) was injected with a 25-G needle.\nA 20-G over-the-needle catheter (48 mm in length) was inserted into the skin.\nThe vein was accessed using a short-axis out-of-plane approach; the anterior wall of the vein was penetrated using a long-axis in-plane approach.\nThe PICC was placed using the modified Seldinger technique.\nThe anesthesiologists attempted to locate the catheter tip in the inferior vena cava or upper right atrium; however, the catheter tip could not proceed to the vena cava. Therefore, the catheter tip was placed in the left brachiocephalic vein (Fig. ). The left brachial vein and left subclavian vein appeared to maintain blood flow after PICC placement (Fig. ).\nContinuous infusion of noradrenaline and opioid therapy was initiated through the PICC. The patient's orthopnea was slightly ameliorated, and he could sleep at night; however, he remained in the sitting position. The patient died 11 days after insertion of the PICC due to respiratory failure. The PICC was used without difficulty until his death.
A 40-year-old female presented to the emergency department (ED) with influenza. She was found to have hypokalemia with potassium of 3.3 mEq/L and moderate persistent asthma. Hypokalemia was managed with IV fluid hydration and 20-mL KCl. She received Tamiflu (Genentech, Inc., San Francisco, CA) 75 mg for five days, Toradol (Roche Holding AG, Basel, Switzerland) 15 mg IV, and ibuprofen 600 mg as supportive management for influenza. Moderate persistent asthma was treated with budesonide and formoterol fumarate via nebulizer. A consult was made with psychiatry because she admitted to an eating disorder and polysubstance abuse. She had been suffering from a longstanding lack of appetite since the age of 16 and perceived herself as too thin. She expressed a desire to gain weight. She was found to have a BMI of 15.1 in the ED and had been incredibly frustrated by the lack of appetite stimulation despite her prescribed use of olanzapine. During the interview, she mentioned with distress that her weight was down to 90 pounds. She complained of nausea when attempting to eat without the aid of cannabinoids. She informed us that she had had a poor appetite since the age of nine and admitted to experiencing abuse in her childhood. She had originally used cannabis for appetite stimulation daily. Five years ago, she had lost custody of her children to their father. She had had to undergo drug screens by the authorities after this and, as a result, she had switched to K2 since it was not detectable on drug screens. When she used K2, she would binge to the extent that she would often end up vomiting. The patient’s history of drug use included one pack of cigarettes daily, narcotics, and benzodiazepines, with multiple rehabilitations. Her most recent rehab had been three months ago. She was temporarily living with her mother but said she would have to move out as it was subsidized housing. During the interview, she described herself as an addict and homeless. There was no mention of addiction to cocaine during the interview, but she was found to be cocaine- positive on a urine drug screen. She was involved with Alcoholics Anonymous and Narcotics Anonymous.\nThe patient was not interested in going to rehab at the time we spoke to her. Our evaluation of the patient found that she did not meet the criteria for anorexia nervosa. The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) requires that three criteria be met for a diagnosis of anorexia nervosa : (1) restriction of energy intake that leads to low body weight; (2) intense fear of gaining weight or becoming fat or persistent behavior that prevents weight gain; and (3) distorted perception of body weight and shape []. The patient met the first criteria with her BMI of 15.1 but failed to meet the other two. The patient was very motivated to gain weight and was exasperated that medicine was failing to improve her appetite. She was on olanzapine at the time of admission, which she claimed did not improve her appetite. There was no fear of gaining weight on her part and, in fact, she had a desperate desire to gain weight. The patient also did not meet criteria three as she perceived herself as underweight and found it grotesque. She similarly did not meet the criteria for bulimia nervosa because one of DSM-5's key criteria for the condition is inappropriate compensatory behavior to prevent weight gain []. Contrary to this, she was, in fact, doing everything she could to gain weight. Two days after our interview, the patient was discharged from the ED with the resolution of flu-like symptoms and hypokalemia as well as improvement of shortness of breath and cough from asthma. Her potassium at discharge was 3.7 mEq/L.
A 32-year-old man presented himself at the hospital with a complaint of numbness, causalgia, and paresthesia in the lateral region of his left ankle. Three years ago, his ankle was hit by a steel pipe, and the symptoms developed three months after open reduction and internal fixation of an ankle fracture. Based on the patient's history, traumatic partial peroneal nerve injury was suspected, and conservative treatment was started. Despite the treatment, the pain worsened and the patient received the adhesiolysis of the peroneal nerve a year before his visit to the hospital. However, the pain kept worsening, and spread below his ankle. Radiofrequency thermocoagulation and epidural patient control analgesia were performed without any improvement, so the patient presented himself again at the hospital. Based on the patient's history, he was receiving neuropsychiatric treatment for panic disorder, but he had no particular disease. When he first visited this hospital, he complained of the following subjective symptoms: pain similar to a knife cutting into his left ankle; a hot, burning feeling; an electric shock-like pain that radiated to his calf or the dorsum of his foot; a cold, tingling sensation; and decreased sensation in the regions below his knees. Based on the visual analog scale (VAS), his pain score was 9. Allodynia, hyperalgesia, body temperature asymmetry, change in sweating, and a reduced range of motion were observed in his left ankle. A three-phase bone scan revealed no particular findings, but an electromyography showed neuropathy of his superficial and deep peroneal nerve, and a digital infrared thermographic image showed that the painful site was up to 2.67℃ lower in temperature than the healthy areas of the patient's body. Pregabalin (150 mg), nortriptyline (15 mg), and Ultracet® were administered daily, and a lumbar epidural block, epidurography, and continuous ketamine infusion were performed. As the patient did not appear to respond to these treatments, we decided to try the spinal cord stimulation. A guide needle for an electrode was tunneled through the epidural space between the 1st and 2nd lumbar vertebrae, and the first electrode was placed in the middle of the 9th vertebral body. After the induced paresthesia was confirmed at the painful site, the lead and the extension cables were connected, fixed at the supraspinatus ligament while leaving a margin to avoid tension on the fixing area, and then guided out through the subcutaneous tunnel. After a test period of seven days, the pain decreased by more than 50%, and the dosage of Ultracet® was also decreased from three times to once daily, which allowed for the transplant of a permanent battery. The outpatient follow-up showed the VAS of 2-4, and a decreased dose of pregalin to 150 mg and of nortriptyline to 10 mg. One month after the operation, however, the patient reported a sudden cessation of stimulation, and accordingly, he was advised to visit the hospital. A simple lumbar X-ray was taken, and disconnection of the electrode was suspected (). The spinal cord stimulator was removed and inspected, which confirmed the disconnection (). As the patient wanted re-transplantation of the stimulator after a discussion, the lead was replaced. The patient reported a VAS score of 3 with sufficient electric stimulation and the stimulator has worked normally without particular problems as of now, four months after the operation.
Our patient is 67-year-old Turkish man, a primary school graduate, and a forest ranger who lives in a rustic area. He was fully informed about the study and written consent was obtained from him prior to examination. In his medical examination he explained that his gums started bleeding at a very early age. At age 24, he started to smoke since he thought smoking would help to stop bleeding. Currently, he smokes 1-1.5 packs a day. At age 30, he started to experience difficulty in eating and complained of tooth mobility and gum bleeding. He claimed that his teeth ached a lot and as a result the teeth were extracted by a non-dentist or himself. Consequently, at the age of 50 to 60, he lost most of his teeth. At age 25, he was diagnosed with periodontitis. Nonetheless, he had not seen a dentist for this problem or performed any personal oral hygiene.\nOur patient complained of pain in the oral cavity especially on the tongue, buccal mucosa and had difficulty in chewing and swallowing solid food for six months.\nHis past medical history was significant for a tonsillectomy as a child. All the symptoms in his medical reviews were negative. Spirometric pulmonary tests were normal. High resolution computed tomography (HRCT) showed minimal emphysematous areas over both apices and non-specific sequelae (Figure ). Head and neck examinations were normal. Magnetic resonance imaging of the tongue revealed no sign of abnormality. He underwent extensive tests to identify the etiologies associated with systemic amyloidosis. Tests such as rectal biopsy, bone marrow biopsy, echocardiogram, abdomen tomography, serum and urine protein electrophoresis as well as liver function test were all normal. However, our patient was diagnosed as chronic renal failure with proteinuria and hypoalbuminemia (Table ). Renal biopsy showed AA type amyloidosis.\nClinical examination revealed poor oral hygiene and heavy plaque accumulation. Our patient was almost completely edentulous. The mean periodontal probing depth was 9.10 ± 0.84 mm (range 8-10 mm) in his remaining teeth. The tongue was diffusely enlarged (macroglossia) and clear red appearance and bilateral white plaques bleeding easily by gentle removing, and irregular translucent papules were present (Figure ). Based upon our patient's history, these papules developed spontaneously or after minor trauma. The enlarged tongue has interfered with speech and swallowing, and caused sleep apnea. In addition, two painful, small flat-based ulcers with erythematous halos and a white ulcer bed were detected, localized on the right and left buccal mucosa.\nCultures from the plaques on the tongue and oral cavity were all negative for bacteria and fungi. The biopsies were obtained from the tongue, buccal mucosa and retromolar trigon. Secondary amyloidosis (AA type), was diagnosed by histological and immunohistochemical findings (Figure ).\nOur patient received comprehensive periodontal therapy, which included careful oral hygiene instructions, curettage combined with non-surgical and surgical therapy. Serum inflammatory markers and some laboratory data improved dramatically at the second week of the periodontal therapy more than at any other time interval (Table ). Restorative treatment started after his periodontal condition was stabilized (around three months after periodontal therapy). Intra-oral radiographs showed poor bone density (Figure ).\nThe Congo red method was used to detect amyloid in tissue sections []. Amyloid was identified as the AA type on immunohistochemical tests with the use of monoclonal antibodies specific to amyloid A [].
A 36-year-old female presented to the maxillofacial surgery clinic complaining of gradually developing asymmetry of the right side of the face for the past 15 years. The progressive asymmetry of the entire right side of the face was noticed by her family members. Mandibular deflection toward the healthy side and overgrowth were noticed 15 years before, progressing gradually until they reached the present proportion. Furthermore, there was progressive development of pain in the TMJ region while opening the mouth. There was no history of trauma, vascular collagen diseases, infections, or surgery on the face or jaws. Her medical history and family history were noncontributory.\nThe extraoral examination confirmed facial asymmetry due to the downward displacement of the entire right mandible and an increase in the vertical height of the middle and lower facial thirds on the right side. The chin had deviated to the right side, and the lip line had slightly shifted downward, tilting toward the right side. Furthermore, mild tenderness was noted in her TMJs bilaterally, a clicking sound was heard during movement of the left TMJ, and tenderness was noted in the right side of the neck. The intraoral examination revealed a slight shift of the mandibular midline to the right side along with a mandibular cant. In addition, she had a posterior open bite, that is, posterior teeth of both the jaws were slightly tilted lingually to maintain the occlusion.\nThe panoramic radiographic image revealed significant uniform asymmetrical enlargement of the right mandibular condyle and elongation of the neck of the right mandibular bone, along with improper dental occlusion of the right side. The right gonial angle was characteristically rounded off and the mandibular canal was displaced to the lower border of the right mandible (Figure ). Computed tomography (CT) scan was performed to further characterize the abnormality. Three-dimensional (3D) volume-rendering CT showed asymmetry and differences in the size of both condylar heads as well as elongation of the neck of the mandibular condyle on the right side (Figure ). Accordingly, a three-phase bone scan along with single-photon emission computed tomography (SPECT) was performed to determine the activity of the condylar heads. The three-phase bone scan was unremarkable in blood flow (not shown) and blood pool (Figure ) images over the head region. However, three-hour delayed planar images demonstrated markedly increased focal tracer uptake at the right temporomandibular region (Figure ). Furthermore, there was bilaterally asymmetrical increased focal tracer uptake localized to the maxilla and mandible, which was related to the dental pathological process.\nThereafter, SPECT demonstrated intense focal activity corresponding to the right mandibular condyle, which appeared enlarged and measured 1.9 × 1.5 cm compared to the left mandibular condyle which measured 1.6 × 0.6 cm in transverse and anteroposterior dimensions. It was associated with elongation of the right mandibular neck and tilt of the mandible to the left side (Figure ). The left mandibular condyle demonstrated normal physiological tracer uptake. The relative uptake of the right mandibular condyle was markedly increased at 73.4%, while the relative uptake of the left mandibular condyle was only 26.6%. There was no evidence of erosive or sclerotic changes at the TMJ bilaterally (Figure ). Moreover, there were no erosive or sclerotic changes at the TMJs bilaterally.\nThe clinical and radiologic findings were consistent with a diagnosis of unilateral CH of the right side of the face. The patient was referred to a tertiary center to be managed accordingly.
A 22-year old woman presented to our clinic with a palpable mass for 6 months. The mass was painless. Her medical history was not remarkable for any disorder. On physical examination she had a palpable mass filling the left upper quadrant and epigastrium. On laboratory examination she had normal levels of total protein, albumin, globulin, alanine aminotransferase, aspartate aminotransferase, blood urea nitrogen, serum creatinine, carbohydrate antigen 19–9 (Ca19-9), carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP). She also had negative serology for hepatitis B and C viruses. On ultrasonography there was a hypoechoic, solid mass with sharp contours and heterogenous pattern which had a size of 16 x 10 cm and diffuse cystic-degenerative areas and which appears hypervascular on Doppler USG (A). The described mass was considered to reside exophytically in the left lobe of the liver. An urgent abdominal tomography showed a giant solid mass that originated from the inferior part of the medial segment of the left lobe of liver and that extended inferiorly. Its size was approximately 17 × 15 x 11 cm. It had smooth contours and marked hypervascularity. It contained diffuse cystic-degenerative areas. A giant hepatic adenoma was primarily considered in the differential diagnosis, which also included liver tumors of mesenchymal origin or hepatocellular carcinoma on a non-cirrhotic basis (B).\nThe patient’s abdominal cavity was explored with a subcostal incision. There was a mass with smooth contours, measuring 15 x 12 cm in the left lobe of the liver, which grew exophytically. Other parts of the liver were normal. The mass’s portion out of the liver was of hypervascular appearance that compressed adjacent tissues but was easily separable from them. The mass was excised with liver tissue and gall bladder, with a negative surgical margin, with the help of an ultrasonic dissector and cautery. There was no additional lesion in the abdominal cavity ().\nThe macroscopic examination of the hepatic resection material revealed a tumoral lesion with a size of 14 × 12 x 13 cm and a cross-sectional color of yellow, which contained diffuse hemorrhagic and necrotic areas, 2 cm apart from the surgical margin. Sections prepared from the tumor showed that it was separated from the adjacent hepatic parenchyma with a clear border but showed infiltration of the parenchyma in a few foci (A). The tumor was highly cellular, the components of which were spindle in shape from place to place and epithelioid in most areas, and they had round-ovoid nuclei and abundant eosinophilic cytoplasm (B). There were interspersed cells that showed nuclear coarsening. Tumor’s background was highly rich in vascularity and there were interspersed free hemorrhagic foci.\nImmunohistochemical study showed negative staining with Pan-CK, Hep-Par, CD117. There was diffuse cytoplasmic positivity with HMB-45 (C) and SMA (D). The background rich vascular network was positively stained with CD34, CD31 and Factor 8 while tumor cells were not. Two mitotic figures were noted under 50 gross magnification. Morphological appearance and immunohistochemical study results suggested a PEComa. Although the criteria for malignancy have not been clearly defined for hepatic PEComas, considering a tumor size greater than 5 cm, presence of more than 1 mitosis under 50 GMA, and infiltrative growth pattern, which have been associated with tumor recurrence or metastatic process for soft tissue or gynecological tumors, the case was considered a malignant PEComa.\nThe patient was discussed in general surgery and oncology councils, which recommend no therapy. The patient recovered uneventfully, and no additional therapy was recommended. She was discharged 3 days after the surgery. She was put under close follow-up; her tri-monthly control tomographic examinations revealed no pathology. She is under follow-up without recurrence 10 months after the surgery ().

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