text
stringlengths 746
31k
|
|---|
A 23-year-old Chinese female patient presented to our clinic complaining about a 1-year history of a growing lump at the medial third of her right clavicle, associated with recurrent and aggravated pain. Limited range of motion of the right shoulder was reported as well. The patient experienced no compression and difficulty in breathing. Physical examination indicated the right shoulder had limitations in adduction, abduction, back extension, and elevation. A hard lump with a diameter of 7cm × 5 cm lump at the medial third of right clavicle was palpated with pain and slightly swelling. No redness and skin lesion around the lump; no muscular atrophy and weakness; no dislocation or deformity of the shoulder joint and no thoracic varicosity were observed.\nThe X-ray film and computed tomography (CT) scan showed the sclerotic bone remodeling of the medial third of the right clavicle (Fig. ). After admitted to the ward, a percutaneous bone biopsy was performed, revealing a suppurative inflammation with bone destruction, without the signs of malignancy (Fig. ). A diagnosis of clavicle osteomyelitis was suggested. Thus, conservative treatments with antibiotic (based on drug sensitivity, clindamycin was given 1.2 g/d for 2 weeks) and non-steroidal anti-inflammatory drugs were given. The patient was discharged after the pain was relieved.\nIn the next 7 months, the symptom of pain gradually worsened and radiated to her right arm. Despite the intake of non-steroidal anti-inflammatory drugs and treatment with antibiotic, the pain was still obvious. Meanwhile, she started complaining of muscle weakness and limited mobility of the right shoulder. The lump of right clavicle was bigger than before and developing to her right neck. After admitted to the ward, X-ray film and 3D reconstruction of CT scan were carried out. Despite the conservative treatments were given, the pain and swelling of the right clavicle were still not relieved, seriously affecting the patient‘s life quality. Meanwhile, as a young woman, the growing lump of the right clavicle obvious affected the patient‘s appearance. Therefore, after the patient and her family members gave informed consent (Informed consent was obtained from the patient for publication of this case report details), we decided to perform a surgical procedure to remove the lesion and reconstruct the clavicle. Because the inflammatory lesion was limited in the medial third, a subtotal removal and two-stage reconstruction of the right clavicle was approved. In order to reconstruct the large structural defect after removal of the lesion without compromising the beauty of the neck, the application of a personalized 3D printed implant could be an ideal method.\nAfter complete removal of the lesion, the bone defect was replaced with a custom-made antibiotics-loaded (vancomycin) cement spacer (Fig. ), which would allow the formation of an induced membrane according to the Masquelet technique.[ Meanwhile, a pathological biopsy was performed again, confirming the diagnosis of chronic osteomyelitis (Fig. ). Six months later, a 3D-printed prosthesis could be implanted for the clavicle reconstruction.\nThe CT scan of chest and scapula were obtained by a scanner (Siemens) with 1.0 mm each layer. The data was stored and analyzed by Mimics 17.0 software in digital imaging and communications in medicine format. After the 3D model of the right clavicle was constructed by the Mimics 17.0 software (Mimics, Materialise, Leuven, Belgium), it was imported into SIEMENS NX software (Siemens PLM Software Inc, Germany) to design the guiding plate. The design of guiding plate was restored in stereolithography format and then printed by UP BOX+ 3D printer (Beijing Tiertime Technology Co, Ltd, China) using polylactic acid. 3D printing remodel based on the healthy contralateral left clavicle was conducted to simulate the prosthesis of right clavicle. In preoperative plan, the right clavicle was extended 15 mm laterally to ensure the same height with left shoulder (Fig. ). The customized guide plates was installed to insert two screws into the acromial end of clavicle and two screws into the sternal stalk to ensure a strong fixation.\nThe surgical procedure was carried out under general anesthesia, and the patient was positioned in a supine position. After the removal of spacer, the PEEK prosthesis was positioned in the Masquelet cavity. Two ends of the 3D-printed PEEK prosthesis was fixed into the acromial end and the medullary cavity of the sternal stalk respectively after cleaning the residual cavity (Fig. ). The fracture of acromion end of clavicle was found during the operation, so it was fixed with steel wire. The blood vessels and nerves were carefully protected during the operation.\nThe patient achieved fast recovery after the surgery. Dressing replacement and removal of drainage occurs on post-operative day 1. The staples are removed on post-operative day 14. The functional exercise started ever since the drainage was removed. After 2-year follow-up, the patient indicated satisfactory cosmetic and functional outcomes. There was no failure or loosening of implant during 2-year follow-up (Fig. ). The range of motion of right shoulder returned to the level of preoperative assessment 3 months after the surgical reconstruction (Fig. ). While pain visual analogue score was 1/10 at 1 month and 0/10 at last 3 months. The evolution of the Constant-Murley shoulder score was satisfactory with an improvement to 88 points within 6 months. At the follow-up 2 years after operation, the patient reported a great satisfaction with the appearance and shoulder function. |
A 30-year-old woman with good general health was referred to the Oral and Maxillofacial Surgery Department of the University General Hospital of the University of Cuiaba for surgical correction of transverse maxillary atresia. On examination, it was observed that in addition to the transverse maxillary atresia, an osseointegrated implant was placed to replace superior left canine (-). Prior to the SARPE, pre-operative panoramic radiography was evaluated () and the patient was explained over the possibility of having the implant malpositioned.\nThe SARPE was performed under local anesthesia and oral sedation in an outpatient setting. The Hyrax distractor activation was started on the 3rd post-operative day, planned to go as two activations daily (¼ turn each time) until 20% of overcorrection to the inferior dental arch was achieved. During activations, it was observed that the left hemi-maxilla had greater expansion, causing an asymmetry in the frontal view of the maxilla (-), but the decision was to continue the activations as planned. After approximately 18 months of post-surgical orthodontic treatment, an acceptable dental occlusion was noticed except for the malpositioned implant (-).\nAfter clinical and dental casts evaluation, surgical relocation of the malpositioned implant was simulated on a semi-adjustable articulator through a segmental osteotomy. As the prosthesis over the implant was satisfactory, it was planned to use the bracket as a reference to the new position. After a trapezoidal vestibular mucoperisoteal flap (), a 699 drill and a chisel were used to perform two vertical alveolar osteotomies adjacent to the implant, including buccal and palatal bony walls, connected to a buccal horizontal apical osteotomy (). Subsequently, a “greenstick” fracture was done, followed by repositioning of the implant. The bracket on the implant was then connected to the orthodontic arch and stabilized with chemically cured resin surrounding the implant and two adjacent teeth on each side (). The bone gap formed by the movement of the block was filled with halogen bone graft (Geistlich Bio-Oss®, Wolhusen, Switzerland) and the crown of the implant was adjusted to be out of occlusion (). The acrylic resin was removed with 45 days. Radiographic control included the immediate post-operative period, 30, 90 days and 16 months. The patient is currently on a 16 months post-operative follow-up, the bone-implant block shows no clinical or radiographic changes, obtaining the clinical success and patient satisfaction for the treatment performed (-). |
A Caucasian male Greek patient, 51 years old, with a history of kidney transplant in a foreign country 19 months before, presented at the emergency department, after being referred by a primary care center, with obstipation during the previous week and acute abdominal pain. The patient was receiving immunosuppressive medication (cyclosporine, MMF, prednisolone). The patient reported low fever (<37.5°C), which was attributed to cystitis, sweating, pain in the lower abdomen, anorexia, weakness and loss of weight during the previous 2 months. The patient also reported alternating constipation and non-haemorrhagic diarrhea in the previous 15 days. 5 days before, the patient underwent colonoscopy, which revealed an intraluminal mass, partially occluding the lumen of the ascending colon (figure ).\nClinical examination revealed: fever (38.9°C), diffuse abdominal tenderness, especially at the right iliac fossa with intense signs of peritoneal irritation, no bowel sounds and marked tenesmus. Blood tests revealed a mildly elevated WBC (13.700/μl with 82.3% poly) and hypochromic microcytic anaemia. Imaging study revealed air in the peritoneal cavity and free fluid in the pelvis. The patient was diagnosed with hollow viscus perforation and was admitted for surgery.\nDuring laparotomy the findings were a perforation of the cecum, a fragile mass (pseudopolyp), which occluded the lumen approximately in the middle of the ascending colon, diffuse erosions of the mucosa and pseudo membranes. A bezoar was found impacted at the level of the occlusion. There was also marked lymph node enlargement in the mesentery and ischaemia of the cecum. A typical right hemicolectomy was performed and special care was taken so, as not to damage the renal transplant. The pathological and microbiological (from free peritoneal fluid) investigation suggested the diagnosis of tuberculous colitis. Specifically, macroscopic study of the resected tissues revealed multiple ulcerations of the mucosa of the distal ileus, the cecum and the ascending colon. Microscopic examination revealed multiple submucosal granulomas, with aggregations of gigantic cells and localized necrosis of the granulomas, with rupture of the overlaying mucosa. Cryptic abscesses and diffuse inflammatory infiltration of the bowel mucosa, with concurrent thinning, were also observed. The image was compatible with chronic colitis. At the same time, multiple lymph node infiltrations of the mesentery and thrombosis of several distal vessels with local bowel ischaemia were found. M. tuberculosis was isolated from the material sent for culture.\nFrom the blood and respiratory tests that followed, active tuberculosis was diagnosed, with the colon as the known primary site. During his stay at the hospital, the patient received broad spectrum antibiotics for the treatment of the fecal peritonitis and corticosteroids were discontinued. After the patient's exit, he received antituberculous treatment with 300 mg/24 h of isoniazide, 450 mg/24 h of rifampicine and 600 mg/24 h of ethambutole. The patient underwent two colonoscopies, 45 days and 3 months after his exit respectively, which were negative for M. tuberculosis. |
A 21-year-old unbooked multigravida at 30 weeks of gestation was referred with preeclampsia and anhydramnios. Her first pregnancy had resulted in a spontaneous miscarriage at 14 weeks. Her second pregnancy was complicated with preeclampsia and intrauterine fetal death at 28 weeks of gestation. During the current pregnancy, she was on tablet labetalol 100 mg thrice daily and low-dose aspirin for chronic hypertension. Earlier, she had been treated with antihypertensive agents telmisartan and amlodipine during the inter-pregnancy period.\nAt presentation, her pulse rate was 96 beats per minute and blood pressure was 146/92 mmHg in the right arm in the supine position. There was no pallor or edema. Cardiovascular and respiratory system examinations were unremarkable. Uterus corresponded to 28 weeks of gestation, and the fetal heart rate was 142 beats per minute. Obstetric ultrasound revealed a single live intrauterine fetus in cephalic presentation with estimated fetal weight less than the third centile, nil liquor, and absent end-diastolic flow on umbilical artery Doppler, suggestive of fetal growth restriction stage II (Figure ).\nHer hemoglobin was 12.3 g/dL and serum creatinine was 0.7 mg/dL. Erythrocyte sedimentation rate and other acute-phase reactants were not determined.\nShe was admitted and treated with four doses of dexamethasone 6 mg intramuscular 12 hours apart for fetal lung maturity and magnesium sulfate 4 g intravenous loading dose followed by 1 g each hour for 24 hours for fetal neuroprotection. The dose of tablet labetalol was increased to 200 mg thrice daily. However, due to uncontrolled hypertension, she underwent an emergency caesarean section after 48 hours of admission. A preterm live male baby weighing 1,300 g and good Apgar score was delivered. The baby had a neonatal intensive care stay of 10 days. Intraoperatively, her blood pressure was 167/110 mmHg in the left arm and 154/95 mmHg in the right arm. In view of chronic hypertension and intraoperative differential blood pressure in both the arms, Takayasu’s arteritis was considered, and the patient underwent further workup. No bruit was audible. Proteinuria was 1+, and the urine albumin:creatinine ratio was 2.0. Fundoscopy revealed grade 1 hypertensive retinopathy.\nCT angiography of bilateral renal vessels revealed concentric circumferential wall thickening of the distal descending thoracic aorta (Figure ) and bilateral proximal renal artery stenosis (Figure ). The descending thoracic aorta was stenosed over a length of 13 cm with significant luminal narrowing (Figure ), as well as the presence of poststenotic dilatation in the aorta and the right renal artery (Figure ).\nHer final diagnosis was angiographic type III Takayasu’s arteritis, with inactive disease determined using Kerr’s score. She was planned for a stenting procedure in the postpartum period. However, due to the lockdown and closure of routine services in view of the COVID-19 pandemic, the proposed procedure has been deferred. She is currently on antihypertensives, is lactating, and is under follow-up by teleconsultation. Both the mother and the baby are doing well. |
A 48 years-old woman was admitted in January 2015 to the Rheumatology Department of the National Institute of Orthopedics of Tunisia with complaints of inflammatory low back pain and a morning stiffness of one hour for 5 years. The history of the patient has revealed a recent swelling and heel pain and red eyes two times last year. She declared no shortness of breath or other breathing troubles. Furthermore, the patient’s personal and family past medical history was unremarkable. On physical examination, she had typical SA appearance with exaggerated dorsal kyphosis and disappearance of lumbar lordosis. A significant limitation in motion of the cervical and lumbar spine was noted and the Faber’s test and Patrick’s test were positive. Peripheral joint examination revealed swelling of ankles. Tenderness was noted in the insertion of Achilles tendon and plantar fascia. Systemic evaluation was normal except the crackle sounds in the lower zones of lungs. A routine laboratory assessment was unremarkable with a normal rate of serum calcium, sedimentation rate and C-reactive protein. Tuberculin skin test was negative. X-ray examination revealed typical finding of SA with a bilateral sacroiliitis grade 4 (), a triple ray in the lumbar and cervical spine. A pelvic CT confirmed the sacroiliitis fusion ().\nChest X-ray showed a mediastinal enlargement and a reticulonodular infiltrate in the middle and lower lungs ().\nThe chest CT scan confirmed the presence of several and bilateral hilar lymph nodes and parenchymal nodes. The pulmonary functional tests showed a restrictive syndrome. Since sarcoidosis was suspected, bronchoscopy and biopsies were performed showing non-calcified granulomatous reaction without cell necrosis. Ophthalmologist examination showed bilateral sequela of anterior uveitis.\nThe patient cumulated 9 points of Amor criteria confirming the diagnosis of SA. A co-occurrence of axial and peripheral SA and sarcoidosis was established. The patient was treated with 15 mg per week of methotrexate and 1mg/kg/day of prednisone for pulmonary disease with good outcomes. |
A primigravida patient aged 42 years was referred to Department of Obstetrics at 22 weeks and 3 days of gestation with obstetric ultrasonography findings suggestive of ectopia cordis. The patient presented hypothyroidism and chronic hypertension, with no family history of birth defects or CHD. Fetal echocardiography confirmed the presence of ectopia cordis associated with a small ventricular septal defect (VSD) measuring 2.7 mm (Fig.). In addition to ectopia cordis, a fetal morphology ultrasound demonstrated the presence of omphalocele with liver herniation, without other malformations. In light of the echocardiographic and ultrasound findings, the diagnosis of PC was suggested. The patient was followed up, with genetic counseling, psychological support, and serial obstetric ultrasonography for evaluation of fetal growth. At week 34, the patient presented premature rupture of membranes, with spontaneous onset of labor. Considering the presence of ectopia cordis and the breech position of the fetus, a cesarean delivery was selected. The delivery was uneventful, and a female child weighing 2,430 g, with Apgar scores of 8 and 9 at 1 and 5 min, respectively, was born (Fig. .\nWithin 11hr of life, the authors opted for surgical correction, which comprised opening of the remnant of the manubrium, total thymectomy, wide opening of both the pleura, resection of the remaining pericardium, bilateral deinsertion of the last three costal arches, and bilateral wide release of a skin flap from the midline to the anterior axillary line. Using these maneuvers, the heart could be accommodated into the chest cavity; the epigastric omphalocele was corrected with abdominal flap rotation and coverage of the diaphragm with a silicon mesh. Chest tubes were bilaterally placed; an orogastric and urinary catheter was placed, and phlebotomy was performed. Antibiotic therapy was initiated with gentamicin and ampicillin; adrenaline was administered for improving the cardiac output. On the 3rd postoperative day, the patient developed anuria unresponsive to diuretics; peritoneal dialysis was initiated, and vasoactive drugs such as milrinone and noradrenaline were administered. On the 7th postoperative day, the patient progressed with anasarca, and a total abdominal ultrasound showed signs of nephrocalcinosis and ascites; an ascitic fluid culture showed bacterial peritonitis. On the 8th postoperative day, a left pneumothorax with hemodynamic repercussion was diagnosed and promptly drained; parenteral nutrition was then introduced. The patient developed metabolic acidosis and worsening of hemodynamic parameters and died on the 17th postoperative day because of septic shock. |
A 13-year-old boy was presented with a Class I skeletal base and a Class I incisor relationship with increased mandibular plane angle and increased lower facial heights. Extraoral examination demonstrated facial symmetry and a straight profile. The upper left central and lateral incisors were in crossbite. The upper dental centreline was deviated 1.0 mm to the left of the mid-facial axis. The lips were competent at rest. There was 3.0 mm of incisor show on smiling.\nIntra-oral examination revealed fair oral hygiene. There were no active carious lesions. In the maxillary arch, there was a bony defect mesial to the upper left central incisor. A space of 5.5 mm existed in the alveolar ridge where the upper left canine was extracted. The upper left central incisor was buccally displaced and the lateral incisor was palatally displaced. There was anterior mandibular displacement of 2 mm upon closure. The left central and lateral incisors were in crossbite and were retroclined relative to the maxillary plane (Figure ).\nIn the mandibular arch the labial segment was mildly crowded and retroclined relative to the mandibular plane. There was a curve of Spee measuring 2 mm at its deepest part (Figure ).\nIn centric occlusion, the incisor relationship was Class I with overjet of 4 mm at the right central incisors, and with a reverse overjet of 0.5 mm and a reduced and incomplete overbite. The molar relationship was Class I. There was a decreased buccal overjet at the posterior segments.\nRadiographic examination confirmed the absence of the upper right canine and the presence of unerupted third molars and no carious lesions were detected. The upper left central incisor showed short root, and the lower incisors showed pipette shaped roots (Figure ).\nThe cephalometric analysis showed a skeletal Class I base (ANB and Wit's appraisal) with slightly retrognathic maxilla. The upper and lower facial heights were increased. There was an increased mandibular plane angle which was in agreement with increased lower facial height, but in good proportion with the upper facial height. The lower incisors were retroclined, and were on the Point A- Po line (Figure )\nThe aims of the treatment were: 1. To close upper anterior space by mesialization of 21, 2. To eliminate anterior crossbite by proclination of displaced upper anterior teeth, 3. To relieve lower crowding and flatten curve of Spee.\nThe treatment plan was orthodontic treatment with fixed pre-adjusted appliance without extraction in the upper arch; and extraction of 34 in the lower arch. The abnormal positioning of 21 and 22 posed the main problem, and was exaggerated by the bony defect. Proper biomechanics were needed to mesialize the displaced tooth 21 into the anterior space to repair the bony defect. |
A 53-year-old postmenopausal Iranian woman presented to the hospital for a progressively growing painful cesarean scar mass. Her personal history included three previous Pfannenstiel cesarean sections without any medical diseases. The last cesarean section was performed 23 years earlier. Pfannenstiel scars were adjacent to her pubic symphysis. She had no family history of gynecological malignancy. She previously had bloody discharge during her menstrual cycle from a very small orifice inside the abdominal cesarean scar for 16 years. The monthly bloody discharge was stopped after her menopause. She detected a mass that was approximately 3 cm in diameter during her self-examination of her cesarean scar 3 years after menopause. The mass was converted to a huge one over 3 months (Fig. ). On physical examination, a significant large, firm, and fixed mass was observed in her suprapubic Pfannenstiel cesarean scar and extended upward to the anterior abdominal wall. An abdominopelvic computed tomography (CT) scan revealed a 9.1 × 5.6 × 4.7 cm heterogeneous solid mass with a cystic component in the lower part of the rectus muscle. The observed mass expanded to the subcutaneous tissue, with close contact with the pubic symphysis, and extended to the retropubic space. Other pelvic and abdominal organs were observed to be normal in the first imaging (Fig. ). Needle biopsy of the mass was performed under ultrasound guidance. Histology revealed an undifferentiated carcinoma. Thorax CT scan, colonoscopy, upper gastrointestinal endoscopy, mammography, and transvaginal ultrasound were all normal. The patient was scheduled for complete excision of the abdominal wall mass as determined by the cancer multidisciplinary team (MDT). A complete excision of the mass and its surrounding tissues was performed. The permanent histology revealed clear-cell carcinoma arising from the Pfannenstiel cesarean scar (Fig. ). Permanent pathological reports indicated negative excision margins; however, the tumor was very close to the surgical margin in the lower part of the mass adjacent to the pubic symphysis (1 mm).\nThirty days after the surgery, the patient initiated chemotherapy with six cycles of paclitaxel and carboplatin every 3 weeks. To follow up, an abdominopelvic ultrasound was performed 3 months after the last chemotherapy treatment. Ultrasound was normal. The patient was asymptomatic nearly 5 months after the termination of chemotherapy. However, she had abdominal discomfort later on. Positron emission tomography–computed tomography (PET-CT) was performed 5 months after the last chemotherapy treatment (Fig. ) and demonstrated ascites and recurrences in the abdominal wall, retropubic space, and pelvic peritoneum space. Therefore, she received second-line chemotherapy with four cycles of bevacizumab, cisplatin, and doxorubicin. She was resistant to the second-line chemotherapy, and the tumor progressed diffusely in the abdominopelvic space during the second-line chemotherapy course. She had severe abdominal pain and mainly presented gastrointestinal symptoms such as nausea. It was determined by the MDT that the patient was a candidate for palliative therapy. However, she refused the MDT decision and insisted on a second surgery. She had the surgery in another hospital. Her second surgery consisted of total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO), pelvic washing, excision of the abdominal wall mass, segmental resection of a narrow lumen of the large bowel, and end-to-end anastomosis and selective large pelvic lymph node dissection. All specimens showed clear-cell carcinoma involvement. The patient received third-line chemotherapy with three cycles of gemcitabine and vinorelbine. She did not return to our center after the second surgery; however, she sent us her treatment records. Unfortunately, she died 20 months after diagnosis. |
A 50-year-old female patient presented to the institute’s dental hospital complaining of pain and mobility in the lower anterior teeth. The patient’s medical history included a resected malignant tumor in the intestine followed by chemotherapy, which had ended one year ago. Intraoral examination revealed mobility and deep caries on the lower incisors, defective restorations on the lower canines, and bilateral missing lower posterior teeth. In the maxilla, there was a defective upper fixed partial denture exten-ding from the right canine to the left second premolar in addition to a missing right first molar, left first molar, and left second molar. A dental panoramic radiograph and peri-apical films showed multiple recurrent caries on the abutments of the restorations, bone resorption, and peri-apical radiolucencies on the lower anterior teeth. The most interesting aspect was the presence of bilateral impacted maxillary third molars, which were inverted and distally directed (Fig. ). Both were asymptomatic and pathology free clinically and radiographically. The crown of the right inverted third molar was very close to the maxillary sinus (Fig. ). On the left side, the upper second molar existed between the inverted third molar and the sinus (Fig. ).\nThe patient was informed about the presence of the impacted and inverted maxillary third molars. Then the case was discussed with the prostodontist and the final treatment plan was to create a removable complete denture after clearance of the available teeth. Regarding the inverted maxillary third molars, treatment options discussed were to extract or to leave in place. Thereafter, the treatment options were fully explained to the patient, including the risks versus the benefits of surgical removal of such impacted teeth. Finally, conservative management was selected, with the patient’s agreement, and the inverted third molars were left in situ. This case report was registered in the institute research center with a registration number of (FUGRP/2014/175). |
A 62-year-old white male with a past medical history of hypertension, diabetes and a history of myasthenia gravis status post-median sternotomy with thymectomy 6 years ago, presented with 1 month history of shortness of breath, bilateral lower extremity edema and abdominal distention. Surgical history was otherwise unremarkable. The patient was a lifetime non-smoker, denied any use of drugs and endorsed occasional consumption of alcohol. He had initially been seen as an outpatient and underwent a trial of diuresis with loop diuretics with no resolution of symptoms and had worsening of renal function with serum creatinine (Cr) elevation up to 3.6 mg/dl from a baseline of 0.9 mg/dl. Patient had missed his outpatient echocardiography that had been requested during that visit.\nThe patient was referred to admission from the clinic for further workup of worsening shortness of breath. Upon presentation to the hospital, his vitals were temperature of 37°C, pulse rate 93 beats per minute, blood pressure 91/58 mmHg, respiratory rate 15 breaths per minute with oxygen saturation of 97% on room air. His prior systolic blood pressure readings were anywhere between 130 and 150 mmHg. Physical examination was notable for elevated jugular venous pressure estimated to be 20 cm H2O, pitting edema of bilateral lower extremities up to the level of sacrum, ascites and clear lung fields. Pulses paradoxus was 18 mmHg. Heart sounds were not muffled at the time of our examination.\nLaboratory values including complete blood count and a comprehensive metabolic panel were within normal limits except for an elevated Cr of 3.6 mg/dl. Meanwhile, a 12-lead electrocardiogram showed normal sinus rhythm with low voltage waves ().\nThe patient underwent a trans-thoracic echocardiogram (TTE), which showed a large fluid extrinsic collection present anteriorly, resulting in significant compression of the right atrium (RA) and ventricle, as well as the proximal right ventricle (RV) outflow tract (). The fluid appeared anteriorly, but extended posteriorly around the inferior aspect of the RV free wall. There was significant hemodynamic compromise of the right heart with elevated tricuspid mean inflow gradient of 4–5 mm Hg, dyssynergic ventricular wall motion and a restrictive mitral Doppler inflow pattern (peak E wave 92.3 cm/s, peak A wave 52.8 cm/s, E/A ratio 1.75 and peak gradient 3.41 mmHg) ().\nMeanwhile, a computed tomography (CT) scan of chest without contrast was done for further evaluation of the mass and to delineate its margins. It showed an abnormal low- to mid-density fluid collection, which extended anteriorly from the level of the pulmonary artery to the base of the heart, in the expected location of the thymic bed with smooth margins. There appeared to be compression of the RA and ventricle ().\nThe patient underwent CT-guided catheter drainage with pigtail catheter placement with successful drainage of 500 cc of sero-sanguineous fluid with an additional 700 cc output over the course of the following 3 days ().\nThe fluid was sent for pathology and fluid/cell block was negative for neoplastic cells or pathogens. The pigtail catheter was removed after 3 days. The patient had complete resolution of his symptoms and returning of his Cr to 1 mg/dl. Follow-up TTE showed resolution of the fluid collection and the RV normalized in size with normal systolic function with no RV collapse (), which was much improved as compared to the initial echocardiography at the time of presentation (). The patient was discharged on the sixth day of hospitalization in a stable clinical condition with a recommendation for close follow-up. A follow-up echocardiograpy 2 months following discharge was normal with no evidence of pericardial effusion, extrinsic compression of the heart or diminished right ventricular function. |
A 74-year-old gentleman who was referred to vascular surgery clinic with a 9-cm infra-renal abdominal aortic aneurysm and mild back pain. He is known to have hypertension, coronary artery disease, with history of CABG. He was declared as a high risk for open repair by cardiologist, so percutaneous elective EVAR under local anesthesia was planned. His family and psychosocial history were unremarkable.\nA low-profile tri-modular device was chosen because of his small iliac arteries and tortuosity. The procedure was performed by a senior vascular surgery consultant with immense experience in endovascular interventions.\nThe EVAR was started with bilateral femoral percutaneous access. The main body of the endograft was deployed successfully (right femoral access) with its both short limbs facing anterioposterior position due to the severe iliac tortuosity. The CLG was cannulated without difficulty and the position inside the main body of the graft was confirmed by twisting the pig tail catheter inside.\nAccording to the fluoroscopy images, the location of the wire seemed to be in the correct place so deployment of both iliac limbs was performed. Completion angiogram revealed maldeployment of both limbs in to the ipsilateral gate ().\nAfter recognition of the problem we proceeded endovascular salvage. Cannulation of the contra lateral gate was achieved via a retrograde access from the left femoral artery. Hydrophilic wire was manipulated between the maldeployed left iliac limb and the native common iliac artery with the help of Vertebral catheter until we accessed the aneurysmal sac. Another wire was passed and crossed over the flow divider from the ipsilateral limb (right femoral artery). This wire was snared from the left femoral access. Afterwards, three balloon expandable covered stents were deployed starting from the flow divider of the main stent graft all the way down the left CIA bifurcation. The maldeployed limb was squeezed between both iliac stent grafts using kissing balloon technique ().\nPatient recovered from the procedure without complications and discharged home after two days. He was placed on dual antiplatelets therapy for 6 months. At 3-, 6-, and 12-months follow-up, the patient was symptom free and had good distal pulses on physical examination. Computed tomography angiogram (CTA) follow-up revealed complete exclusion of the aneurysm sac, no endoleak, decreased aneurysm size, and patent bilateral EVAR limbs (, ). The patient was instructed to continue aspirin for life and educated about the importance of an annual CTA follow-up. |
A 53-year-old male, wheelchair user, presented to our department with 3 years history of clunking noise in his back while transferring, shortened torso and deteriorated sitting balance. The patient suffered complete paraplegia at T10 from road a traffic accident 39 years back. Primarily the patient was treated by performing T8–T12 fusion with Harrington rod fixation. Spinal instrumentation was removed 10 years later due to implant prominence and pain in upper back, which was completely relieved after implant removal. Despite the patient was wheelchair-user, he managed independently to do his routine self-care activities, completed education and work as a leader of his own international company.\nThe patient was referred to our clinic due to new difficulties to reach shelf's in his kitchen while sitting in the wheelchair, tumour prominences and clunking noise in his back. Clinical examination revealed a kypho-scoliosis with instability distal to the thoracic-lumbar junction. There was a 15 cm increase in body length from sitting to supine posture. He had complete flaccid paralysis of both lower limbs, with sensory loss below T9, and no contractures. He had no history of fever or weight loss. No recent changes had occurred in relation to his bowel and bladder habits.\nRadiographs showed gross destruction of L2 and L3 vertebral bodies leading to kypho-scoliosis at the upper lumbar region with a Cobb angle of 70 degrees in the sagittal plane, paravertebral hypertrophic ossification involving an area from T12 to L3, with destruction of posterior elements, and lateral translation of L2 over L3 (). A CT scan confirmed partial resorption of upper lumbar vertebral bodies and gross para-spinal ossification and deformity (). MRI demonstrated the complex vertebral body destruction, peripheral bony debris, and paraspinal mass with a huge fluid filled cavity extending into posterior elements. A minor syrinx was also seen at T10/T11 following previous spinal cord injury (). Laboratory analysis reveals no signs of infectious pathology. Based on clinical history, imaging, and laboratory findings, the diagnosis of Charcot's lesion was made. Due to the massive cavitary lesion and hyper-intense signal in T2 weighted images, angiography was performed to rule out hyper-vascular nature of the lesion and in order to perform pre-operative embolization.\nGoals of surgical treatment comprised complete excision of the lesion, correction of the deformity and stabilization of the spine. Exposure of the affected level through the posterior approach showed complete destruction of posterior elements and a pseudo joint cavity filled with yellowish coloured fluid without relation to the spinal canal. Tissue samples were taken from neuropathic segment for culture and histological assessment. Pedicle screws were inserted in T12–L1 and L4–L5, unilateral rod was used for temporary stabilization and distraction to work upon the lesion. Complete 3 column resection of the pseudoarthrotic cavity and curettage of sclerotic avascular endplate bone was performed. Resection was kept confined to the anterior pseudocapsule of the lesion. The spine was realigned and Ends of the resected cavity were approximated and temporarily stabilized. A structural allograft (femoral head from the bone bank) was placed in the created void. The rest of the space was filled with morselized local graft. There was good bone quality in the uninvolved vertebrae felt during screw insertion. Intra-operative fluoroscopy revealed kyphosis in thoraco-lumbar region even after closure of the resection site. The construct was extended to T9 proximally, and bilateral Smith Peterson osteotomy was performed between T10/T11 and T11/T12. Correction of the deformity was achieved and the rods were in situ bended and placed. A four rod construct was used for the fixation to prevent future risk of rod breakage (). The lateral posterior elements were decorticated and packed with morselized allograft. Blood loss was 1.5 L in the 6 h procedure.\nPost-operatively the patient recovered uneventfully except for a single episode of reduced blood pressure, for which he stayed an additional day in intensive care. There was 2.5 cm shortening in the posterior column compared to the pre-operative length. He was allowed to mobilize in wheelchair without brace immediately after surgery. Clinically the patient had a straight spine and reported to feel more secure and balanced while sitting in the wheelchair. Biopsy taken the wall of the cavity revealed non-specific fibrosis and the cultures were negative for any infectious pathology. The patient rehabilitated well to the pre-operative status and was allowed to transfer in and out of the wheelchair himself by 6 weeks. At the latest follow up 1 year post-operatively, the patient remained symptom-free and independent doing his routine daily activities (). |
A 44 -year-old man presented to our emergency department (ED) on 10 March 2020 with constant upper abdominal pain for 8 days. The pain radiated to the back for 3 days and was accompanied by anorexia and nausea. He was afebrile and denied any other related symptoms. He has a history of arterial hypertension that was well controlled with losartan 50 mg daily and regular follow-up by a general practitioner. He smoked cigarettes for 20 years, about one pack daily. He denied alcohol or illicit drug use. He was married and worked as a chef. His family history was not relevant.\nOn arrival at the ED, he was alert, oriented and cooperative. His vital signs were: temperature of 37.1°C, blood pressure of 135/74 mm Hg, respiratory rate of 21/min and pulse rate of 98/min. His weight was 79 kg, height 165 cm (body mass index 29 kg/m2). The cardiopulmonary auscultation was normal, abdomen examination revealed tenderness in the epigastric region, no hepatosplenomegaly or masses. Bowel sounds were present and normal. Neurological examination was unremarkable.\nThe relevant laboratory results at the time of admission are provided in . The serum was lipaemic on gross examination as shown in . Laboratory parameters were remarkable for high anion gap metabolic acidosis with uncompensated respiratory alkalosis, HTG, hyperglycaemia, markedly elevated serum lipase levels and urine ketones positive. Serum electrolytes, liver and renal function tests, coagulation profile and lactate dehydrogenase were within normal limits. Chest X-ray and abdominal X-ray were normal. An abdominal CT was performed and demonstrated an enlargement of the the distal pancreatic body and tail with blurred contour and peripancreatic fluid collection; focal hypodense area was found in the pancreatic tail. These findings were suggestive of AP, with necrosis in the pancreatic tail (). There was no evidence of gallstones.\nBased on the evidence of the clinical findings, laboratory and radiological investigations, the patient was diagnosed with HTGP and diabetic ketoacidosis (DKA). Thus, he was admitted to the medical intensive care unit for fluid resuscitation, pain control, bowel rest and intravenous insulin infusion therapy for both HTGP and DKA. The intravenous insulin infusion regimen we used was the DKA insulin therapy protocol. The insulin infusion was initiated at a rate of 0.1 units/kg/hour and was titrated for target a reduction of capillary blood glucose by 3.0 mmol/L/hour, a rise of the venous bicarbonate by 3.0 mmol/L/hour and a maintenance of serum potassium between 4.0 and 5.5 mEq/L. After 1 day of intravenous insulin infusion therapy, his metabolic acidosis resolved and triglyceride level downtrended from 136.4 mmol/L to 11.58 mmol/L (12081 mg/dL to 1025.7 mg/dL). The intravenous insulin infusion therapy with 5% dextrose was continued with monitoring serum glucose every 1–2 hours, triglyceride every 24 hours and electrolytes monitoring every 12 hours. The insulin infusion therapy was stopped on day 4 when there was clinical improvement and the triglyceride level had decreased to 5.3 mmol/L (469.4 mg/dL) (). |
A 73-year-old man with a history of hypertension and dyslipidemia was admitted to our Cardiology Department due to anterior S-T segment elevation acute MI. He was hemodynamically stable and was initially treated with thrombolytic therapy. Urgent coronary angiography was planned because of the absence of reperfusion and hemodynamic deterioration. Transthoracic echocardiography revealed LV enlargement, akinesia of anterior, septal and all apical segments with left ventricular ejection fraction of about 25%. Mild to moderate MR was also noted. There was no post MI mechanical complication. An intra-aortic balloon pump was inserted and coronary angiography was performed which showed severe three-vessel disease that was not amenable to percutaneous intervention. Surgical revascularization was conducted on the second day of admission. After the operation, he was stable for three days, however, he subsequently developed progressive dyspnea, tachycardia and hypotension. Cardiovascular examination was remarkable for a new harsh holosystolic murmur heard maximally at the left lower sternal border. His blood pressure was 95/50 mm Hg and rales were present in the bases of the lungs. Transthoracic echocardiogram showed an apical moderate size simple VSD measuring by color Doppler echocardiography (LV side: 12 mm, RV side: 9 mm, apical rim: 8 mm) with bidirectional shunting (). RV systolic pressure was estimated as 55 mm Hg from a tricuspid regurgitant jet velocity of 3.4 m/s. Due to the patient’s clinical condition, and his refusal to redo cardiac surgery, interventional VSD closure was planned under fluoroscopy and real time transesophageal echocardiography (TEE) (Vivid 3, GE, USA) guidance.\nIn this patient, because of difficulties in the routine arterial approach, we decided to advance the A1 catheter from the femoral vein access to RV, then to LV via VSD. A 0.035 inches ×3 m Amplatz guidewire was advanced to RV crossing VSD into LV, ascending and then descending aorta for better support. The delivery sheath was then advanced from the venous side to the LV cavity over the wire and the dilator and the wire was carefully removed. A 20-mm Amplatzer PIMVSD device (AGA Medical) was used. The occluder device was then delivered to the LV. The device was extruded from the sheath until the LV disc was opened under echocardiographic guidance to ensure that the device did not open in the mitral chordal apparatus (). It was then withdrawn toward the interventricular septum. After further satisfactory echocardiographic evaluation of septal alignment, the RV disc was also deployed and the device was released from the delivery cable ().\nBoth TEE () and left ventriculography () confirmed good placement of the device with no detectable residual shunt, demonstrating good device size selection. The patient showed immediate significant symptomatic improvement. He was transferred to the coronary care unit, and discharged seven days later. As of the date of this case report, the patient is alive and feeling well with no residual shunt detected by transthoracic echocardiography. No procedure-related complications have been recorded during these six years. |
A 26-year-old right-handed white woman with no significant medical history was diagnosed as having MS in 2013 at age 22 and experienced ongoing radiologic activity on both glatiramer acetate and dimethyl fumarate. She transitioned to natalizumab in July 2014 to stabilize disease activity, and her JCV antibody index was positive at 3.58 prior to starting natalizumab. She became clinically and radiologically stable with the initiation of natalizumab until November 2016 when a surveillance MRI of her brain showed asymmetric confluent non-enhancing hyperintensities in the bilateral subcortical precentral gyri consistent with PML (Fig. , ). Cerebral spinal fluid (CSF) showed quantitative polymerase chain reaction (PCR) for JCV of 15 copies/ml, and other CSF studies were within normal limits. A diagnosis of PML was made based on the compatible neuroimaging findings along with the presence of JCV DNA in the CSF. Natalizumab was discontinued after 27 total treatments. Our patient was asymptomatic at the time of PML diagnosis, and she was highly functioning with an Expanded Disability Status Scale (EDSS) of 0. A decision was made to defer plasmapheresis at the time of diagnosis given her high functional status, subtle radiological change, and low viral titer. She was treated with orally administered mefloquine loading dose followed by 250 mg weekly and mirtazapine 15 mg daily.\nRepeat MRI at 2 months following diagnosis showed no changes in her brain lesions. She remained asymptomatic until 3 months after diagnosis when she noticed mild dysmetria of her left hand that progressed to a tremor. The following month a repeat brain MRI revealed a few small enhancing lesions in her left frontal lobe suggestive of IRIS (Fig. , ). The hyperintensities in the bilateral precentral gyri remained stable. Imaging of her cervical spine revealed a new non-enhancing cord lesion. She was then treated for 5 days with intravenously administered immunoglobulin and restarted on glatiramer acetate for MS treatment. A repeat CSF examination in February 2017 showed JCV PCR of 31 copies/ml.\nFive months following her diagnosis, a repeat brain MRI showed interval development of T2 signal abnormality with mild enhancement in multiple areas including the brainstem, cerebellum, and bilateral cerebral hemispheres (Fig. , ). A repeat lumbar puncture was performed. JCV PCR in the CSF was undetectable. Mefloquine and mirtazapine were discontinued. Given the MRI findings, she was treated for ongoing inflammation associated with IRIS versus a possible exacerbation of her underlying MS with high-dose intravenously administered methylprednisolone (IVMP) 1500 mg daily for 3 days. She was then transitioned from glatiramer acetate to ocrelizumab for treatment of MS. Six months following her diagnosis she reported changes in left hand dexterity and right upper extremity phasic spasms. A repeat lumbar puncture was performed and JCV PCR remained undetectable. She continued MRI surveillance followed by treatment with high-dose IVMP for a total of six courses until there was significant resolution of enhancement on her brain MRI (Fig. , ). Following treatment, she has residual left hand dysmetria and tremor as well as right upper extremity phasic spasms. At 1-year follow-up, her EDSS is 2.0. |
A 58-year-old African American male presented to the Emergency Department by ambulance complaining of a constant, sharp, and worsening midepigastric pain that radiated posterior for approximately 30 minutes. The pain was perceived to be 10 out of 10 and was described as an inflated balloon that was squeezing from the inside out. The paramedics found the patient in his house hypotensive and lying in a right lateral decubitus position. The patient was given a bolus of normal saline and his blood pressure responded appropriately. Upon examination, the patient claimed to of had a two-year history of worsening mild midepigastric and left upper quadrant abdominal pain and was seen by his primary care provider whom prescribed him a proton pump inhibitor (PPI) which partially relieved some symptoms. In addition, the patient complained of being nauseated, feeling bloated after meals, having a history of bloody stools one week prior to admission, and increased pain after eating. He denied vomiting or early satiety. Vital signs on admission were stable.\nPast medical history included gastroesophageal reflux disease (GERD), hyperlipidemia and impaired fasting glucose. His medications consisted of a PPI, H2-blocker and simvastatin. The only past surgical history was an appendectomy. The patient was a truck driver for several years. He denied alcohol, drug use, and smoked approximately ½ packs of cigarettes a day for the past 40 years. He reported his two nieces having an unspecified type of cancer but no other known family history of cancer or heart diseases. Review of systems was negative except for gastrointestinal complaints as described above. The patient denied weight loss, fatigue, fever, shortness of breath, and/or chest pain. On physical examination, the abdomen was hard, guarding was noted, and there was direct and rebound tenderness noted in all four quadrants but worse in the epigastric area.\nOn admission, the patient had a complete laboratory workup involving all systems. The abnormalities found mildly decreased were hemoglobin of 12.9 g/dl, hematocrit of 39.6%, albumin of 3.1 g/dl, and sodium of 132 mEq/l. The only abnormalities found mildly increased was the red blood distribution width of 16%, white blood cell count of 15,100 ul, corrected calcium of 11.32 mg/dl, glucose of 129 mg/dl, prothrombin time of 12.2 seconds, and the international ratio of 1.2 seconds. Stool guaiac was negative.\nComputed tomography (CT) of the abdomen and pelvis on admission revealed a large 4.7 centimeter (cm) lobulated hypodense mass in the region of the porta hepatis probably arising from the liver with the possibility of adjacent morphologic tubular lymph nodes or satellite lesions (). Extending from the lobulated mass of the liver was a broad lobulated 10 cm band of density just deep to the rectus muscle extending from the upper abdomen to the level below the iliac crest, which possibly represented extraperitoneal infiltration. Additionally, an enhancing lesion was seen in the caudate lobe of the liver that was most likely metastasis. Blood was noted in both paracolic gutters, and there was a pancake-type density along the anterior abdominal wall, which was most likely blood that extended into the right pelvis representing omental caking. After review of the CT, serum measurements of Cancer antigen (CA) 19-9, Carcino embryonic antigen (CEA), and Alpha-fetoprotein (AFP) were found to be within normal limits. Serum Helicobacter pylori was positive. Colonoscopy and esophagastroduodenoscopy was recommended after review of the CT and findings were mild antral gastritis and a nearly obstructing mass in the descending colon. Recommendations were to rule out other malignancies and surgical intervention. An acute abdomen series with chest radiograph was consistent with the CT with additional findings of patchy and streaky infiltrate of the right and left lung bases and question of the mass in the left upper quadrant displacing the stomach medially. There was no evidence of acute obstruction or air under the diaphragm. Positron emission tomography scan was performed to evaluate for metastases and results showed increase uptake in the left upper quadrant, and about 4 cm of caudate lobe of the liver.\nUtilizing CT guidance, biopsy of the large mass in the left upper quadrant was performed by Interventional Radiology on the same day of admission. Pathology results two days later showed a well-differentiated neuroendocrine carcinoma. The architecture revealed insular and trabecular morphologies, and the Ki-67 index was approximately 30% positive (). Microscopic sections showed nested epithelial cells with moderately increased mitotic activity, nuclear atypia, irregular nuclear contours and hyperchromasia. Immunohistological stains were performed with the following results: the epithelial component was positive for cytokeratin AE1/AE3, neuroendocrine marker synaptophysin was positive, neuroendocrine marker chromogranin-A was positive, neuroendocrine marker neurone specific enolase was positive, and CA 19-9 was negative.\nOn hospital day number six the surgery service performed a diagnostic laparoscopy, exploratory laparotomy with distal pancreatectomy, splenectomy, partial gastrectomy, left colectomy, and resection of the caudate lobe of the liver (). Pre and postoperative diagnoses were well-differentiated neuroendocrine tumor of the pancreatic tail with metastasis to the caudate lobe of the liver. After diagnostic laparoscopy revealed a moderate amount of blood around the peritoneal cavity and metastasis to the liver it was necessary to convert to exploratory laparotomy. Blood was aspirated from the abdominal cavity and sent to cytology, which later revealed numerous neutrophils admixed with red blood cells yet no malignant cells. General inspection of the peritoneal cavity revealed a tumor in the caudate lobe as well as a large 10 cm mass that was fixed in the left upper quadrant. The tumor itself was quite vascular and was surrounded by varices, which was most likely the cause for the blood throughout the abdomen as well as within the lesser sac. Additionally, a periaortic lymph node appeared grossly positive and was then dissected and sent for analysis. There was no evidence of metastases in the pelvis, small bowel, or omentum. The diaphragm was not involved or infiltrated by the tumor and neither was the left kidney or adrenal glands.\nOverall, the surgery was successful. At the end of the procedure two 10 ml Jackson-Pratt drains were inserted into the lesser sac and left upper quadrant.\nGross specimens were sent to pathology in three parts: frozen section of the periaortic lymph node measured 1.0 by 0.8 by 0.4 cm, the caudate lobe of the liver measured 4.0 by 3.5 by 2.5 cm, and the massive left upper quadrant tumor that consisted of an en bloc resection of the spleen, partial transverse colon, partial small bowel, pancreatic tail, partial distal stomach, adipose tissue, and lymph nodes. The spleen measured 12 by 7.5 by 2.5 cm, the colon measured 33 cm in length and 4 cm in diameter, the pancreatic tail measured 12 by 8 by 6 cm, a portion of the small bowel measured 15 cm in length and about 3 cm in diameter, and a small portion of the distal stomach measured 9 by 6.5 cm (). The massive tumor invaded into the serosa of the transverse colon, distal stomach, and small bowel. The tumor appeared to compress the capsule of the spleen, however, no direct invasion was identified (). After sectioning of all the involved organs it was noted that the tumor originated from the pancreas. Within the adjacent adipose tissue, multiple lymph nodes ranging in size from 0.3 to 1.0 cm were analyzed.\nMicroscopic sections were analyzed and found the perioarotic lymph node to be negative for metastatic carcinoma. Microscopic sections of the caudate lobe of the liver showed metastatic well-differentiated neuroendocrine carcinoma (). Sections of the massive left upper quadrant mass showed invasive well-differentiated neuroendocrine carcinoma characterized by cells with high nuclear to cytoplasmic ratio, irregular nuclear contours and hyperchromasia arranged in trabecular and insular patterns. There were 21 of 21 lymph nodes positive for metastatic carcinoma. Margins of resection were free of tumor. Tumor node metastasis (TNM) staging was T4, N1, M1.\nOverall, the patient tolerated the procedure and hospital stay well. During the course of hospitalization his pain was well controlled with morphine, and his glucose was well controlled after being weaned off of an insulin drip and placed on a sliding scale. Vaccinations for encapsulated organisms including pneumococcal, Streptococcus pneumoniae, Haemophilus influenza and Neisseria meningitides were administered. Twenty days post-admission the patient was discharged from the hospital with instructions not to lift more than 15 pounds, and to follow-up with surgery in one week. |
A 60-year-old man with no significant past medical history came to emergency department (ED) complaining of dry cough and fever (between 39 and 40°C) for 1 year and 6 months of evolution. During that period the patient had a significant history of night sweats and weight loss (25 pounds). He denied travels, contact with sick patients, tobacco smoking, chest or abdominal pain. Physical findings included a chronically ill, cachectic patient with bilateral basilar rhonchi on auscultation and no lymphadenopathy. Prior to ED arrival, he had an extensive outpatient work up due to persistent fever which included; chest x-ray, chest and abdomen–pelvic CT scan, CBC, blood culture, urine culture, tuberculin skin test, RPR, hepatitis B and C serology, serum complements, ANA, RF and serum immunoglobulin levels (IgA, IgG, IgM). All of these laboratories yielded normal results and imaging studies did not show lung infiltrates, mass or nodules. Additionally HIV was negative and CD4 count (980 cells/mm3) was normal. Patient continued with fever and developed dry cough 3 months after initial laboratories, reason for which chest x-ray was repeated. At that moment he was diagnosed with pneumonia and received antibiotic therapy with levofloxacin for 7 days. There was mild interval improvement, but after antibiotic therapy was completed his symptoms came back. Three weeks later after antibiotic therapy bronchoscopy for acid-fast bacilli, bacteria, fungi culture and staining was performed due to unresolved pneumonia. No organism growth or positive staining was reported neither by pathology nor by microbiology laboratory. Negative results lead to the suspicion of cryptogenic organizing pneumonia treated with prednisone 60 mg daily for ~5 weeks without success and corticosteroids were tapered down. In fact, symptoms worsened with high grade fever (40°C) and it is at this point when he arrives to ED. Patient was admitted to the hospital and chest CT scan was performed revealing a new anterior paramediastinal abscess measuring 7 cm LG × 5.8 cm AP × 6.7 cm TV with bilateral lung infiltrates (Fig. ). The abscess was drained by interventional radiology and sample cultured on blood agar under aerobic conditions yielded white, rough and dry colonies, which presented gram-positive filamentous branched bacilli and were modified acid-fast bacilli stain-positive suggestive of Nocardia species, this was reported seven days after samples were taken. Molecular analysis by 16 S rRNA gene sequences indicated that the isolate was N. cyriacigeorgica after 2 weeks from initial samples. Trimetropin–sulfametoxazole 1020 mg daily in divided doses was initiated due to susceptibility and continued for 6 months. After 1 week there was significant improvement of symptoms and follow up imaging showed interval resolution of pulmonary infiltrates and abscess reduction (Fig. ). The patient had continuity at outpatient clinics and have fully recovered. |
Case 1. A 58-year-old female university teacher who was living with her husband was referred to our clinic after complaining of a heavy, splitting pain in the four maxillary front post-crown teeth, as if they were being pressed from the side. Her medical history was unremarkable except for hypertension and hyperlipidemia. She was taking candesartan cilexetil and alprazolam. She had no psychiatric history and no significant family history.\nNo particular psychological factors could be identified; however, she was anxious about the unexplained pain for a long time.\nFive months before the first visit, she had undergone root canal treatment of the left mandibular first molar at a primary dental clinic, and the pain in the maxillary right and left central incisors and lateral incisor appeared 2 months after dental treatment. Afterwards, she underwent examination with radiography at a dental college hospital, but no abnormalities were found, and a CT scan at another dental clinic also revealed no abnormalities. She found our department on the Internet and was referred to our clinic by her primary care physician.\nAlthough the patient had anxiety, obvious signs of depression were absent. Her Zung Self-Rating Depression Scale (SDS) score was 53; however, she did not have depressive mood, lack of emotion, lack of energy, nor suicidal idea. Therefore, we started treatment with 10 mg of amitriptyline and increased the dose to 20 mg 1 week later. Her symptoms started to improve 3 weeks after her first visit. She said, “The character of my pain changed from a feeling of the tooth being broken to a pressure feeling.” Her symptoms were cured one and a half months after her first visit. She continued taking 20 mg of amitriptyline for 4 months, and then the dose was gradually tapered and finally ceased 8 months after the first visit. She experienced a remission in her symptoms. |
A 34-year-old Caucasian male had undergone intestinal transplantation originally more than 3 years ago because of chronic intestinal pseudo-obstruction due to familial visceral myopathy . The first intestinal transplant was lost due to volvulus 2 months post-transplant. Retransplantation was performed 18 months ago. Viremic cytomegalovirus (CMV) gastroenteritis was diagnosed 1 year post-transplant. Treatment with intravenous ganciclovir and later oral valganciclovir was successful, and CMV PCR remained negative in blood and intestinal biopsies during follow-up. The patient also suffered another volvulus of the colon, which was successfully corrected operatively. Mild grade 1 acute rejection in the colon occurred 2 months later; the small intestine was normal. The rejection resolved quickly with intravenous steroids; follow-up biopsies at 1 week were normal. After the rejection episode, there were nine follow-up endoscopies showing only mild segmental erythema and distorted mucosal vascular pattern on a confined area in the colon; we speculated the previous volvulus as a cause for these mild findings. In biopsy specimens, there were no signs of rejection or inflammation, and viral samples were negative.\nNorovirus gastroenteritis was diagnosed 2 months after the rejection. The patient was admitted to the hospital due to dehydration, abdominal pain, and fever. Prior to this, the patient's family members had suffered symptoms of gastroenteritis, presumably of viral origin. In our patient, gastroenteritis symptoms with watery diarrhea persisted, and the patient needed repeated episodes of hospitalization due to dehydration. Norovirus PCR from the stools remained repeatedly positive, and the norovirus infection was considered a cause for the chronic diarrhea. Endoscopy findings were unchanged. Nearly 3 months after the initial norovirus infection diagnosis, treatment with oral immunoglobulin was started. An intravenous immunoglobulin solution (Privigen®) was given orally at a dose of 1250 mg (25 mg/kg body weight) four times daily for 2 days (altogether eight doses). The immunoglobulin solution did not bypass the gastric barrier. Tacrolimus trough levels during the preceding 4 months had been stable above 10 ng/L (range 11.5–18.3 ng/L), and the patient had received triple immunosuppression therapy with MPA 360 mg twice daily and methylprednisolone 6 mg daily.\nFour days after the end of the immunoglobulin treatment, the patient was again admitted to hospital due to fever, increased diarrhea, and abdominal pain. Endoscopy via colostomy was performed, and severe biopsy-proven rejection grade 2–3 was diagnosed in the small-bowel and colon (Fig. and Fig. ). CMV and Epstein–Barr virus samples were negative. Tacrolimus trough level was 12.4 ng/L. Initial therapy was by high-dose intravenous methylprednisolone. However, signs of rejection persisted on repeat endoscopy, and finally, a 10-day course of intravenous antithymocyte globulin was started, whereby the acute rejection resolved both symptomatically and in graft biopsies (Fig. ). Donor-specific antibodies were negative. Follow-up endoscopies showed persisting signs of colitis and distal ileitis, but these finally resolved at 3 months after starting immunoglobulin therapy, and the patient has remained rejection-free now 10 months later.\nThe norovirus infection persisted, and the patient was started on nitazoxanide 500 mg twice daily, but without any clear clinical or virologic response. Bacterial, other viral, and parasitic samples were negative (Fig. ). |
A 29-year-old gravida 4, para 3 female with a past medical history notable for type 2 diabetes and elevated HgbA1c at 7.5% was first noted to have a fetal abnormality by nuchal translucency ultrasound (US) at an outside institution, with nuchal translucency measuring 3.8 mm. She underwent genetic counseling but declined further invasive testing. Cell-free DNA screening was performed and results were low risk for Down syndrome, trisomy 13 or 18. Given these abnormalities, however, she underwent further fetal ultrasound at 16 weeks and was noted to have ventriculomegaly with a suspected posterior fossa mass. She presented to our institution at 20 weeks 6 days gestation, and fetal US and brain MRI were performed. Obstetric ultrasound demonstrated symmetric bilateral enlargement of echogenic choroid plexus with associated ventriculomegaly and enlarged extra-axial fluid-filled spaces (Figure ), with differential diagnosis including intraventricular hemorrhage, choroid plexus hyperplasia or choroid plexus papilloma. Head size was larger than expected for gestational age (GA). Biparietal diameter measured 6.7 cm (corresponding with 27 weeks 2 days GA) and head circumference measured 24.2 cm (corresponding with 26 weeks 1 day GA).\nAn MRI scan of the fetal brain performed on the same day demonstrated bilateral ventriculomegaly (Figure ). The lateral ventricles measured up to 14 mm in atrial diameter, with normal morphology of the third and fourth ventricles. There was also marked enlargement of the supratentorial and infratentorial extra-axial subarachnoid spaces. The choroid plexus appeared enlarged bilaterally without definite hemorrhage on echo-planar imaging. Additionally, there was no susceptibility or T2 hypointensity outlining the ventricles or brainstem, suggesting that there was no prior intracranial hemorrhage.\nBased on these MRI findings, targeted Doppler US was performed and demonstrated increased flow within the choroid plexus bilaterally. Thus, the US findings suggested enlarged vascularized choroid, rather than intraventricular hemorrhage. No other fetal morphologic abnormality was noted. Mild parenchymal thinning was present, likely secondary to ventriculomegaly. The findings on prenatal US and fetal MRI suggested bilateral choroid plexus papillomas or hyperplasia with increased cerebrospinal fluid (CSF) production leading to hydrocephalus and enlarged subarachnoid spaces. The differential diagnosis included possible sequelae of dural venous sinus atresia, leading to decreased CSF absorption and engorgement of the choroid plexuses, and a follow-up study was recommended.\nFollow-up sonogram at 22 weeks 6 days gestation demonstrated marked macrocephaly (head circumference 30.4 cm corresponding with 33 weeks 1 day GA) with further increase in the size of bilateral choroid plexuses and of extra-axial CSF volume. Prominent flow was again shown by Doppler US within the enlarged choroid plexus (Figure ), making bilateral choroid plexus papillomas or hyperplasia the most likely diagnoses. Choroid plexus papilloma was favored, due to the relative rarity of choroid plexus hyperplasia [].\nThe fetal head size continued to enlarge. On obstetric sonogram at 31 weeks 3 days, the biparietal diameter measured 22.6 cm and head circumference measured 75.3 cm, both significantly beyond standard measurements of fetal head size.\nThe patient presented in preterm labor at 31 weeks 6 days. Due to marked macrocephaly, fetal cephalocentesis was performed at the time of delivery. Under ultrasound guidance, a 22-gauge spinal needle was introduced through the uterine wall, through the fetal scalp and into the enlarged extra-axial CSF space of the fetal head. The spinal needle was connected to a vacuum bottle, with controlled drainage of 3.5 L of xanthochromic CSF. Post-procedural ultrasound demonstrated no evidence of intracranial hemorrhage. The fetus was subsequently delivered by cesarean section, but demised shortly after delivery, despite efforts at resuscitation. Postnatal pathology confirmed atypical choroid plexus papillomas with ependymal differentiation (WHO grade II; Figure ). |
A 7-year-old girl presented to the accident and emergency department in the evening with a 2-day history of worsening right-sided abdominal pain. The pain was non-migratory in nature and was associated with anorexia, a single episode of diarrhoea and vomiting. This was the first time she had experienced this pain. The patient was fit and well with no other medical conditions. She was premenarcheal. Her mother gave an uneventful perinatal history and family history.\nOn assessment by the surgical team, the patient was found to be feverish with a temperature of 38.1°C. She displayed generalised tenderness over her lower abdomen, but particularly in the right iliac fossa. Her bloods revealed an inflammatory response with a white blood cell (WBC) count of 19 x 109/L and C reactive protein of 62 mg/L. The patient was started on intravenous antibiotics. As the patient remained clinically stable overnight, a plan was made to perform an open appendicectomy the following morning.\nIntraoperatively, she was found to have an appendix adhering to the posterior aspect of the caecum, which looked macroscopically normal. While no free fluid was identified, mesenteric lymph nodes of the small bowel were identified with no Meckel’s diverticulum. A decision was made to remove the appendix.\nBefore closure, a limited inspection of the pelvis was performed through the right iliac fossa wound. This revealed a suspicious black lesion and so the wound was extended to allow further exploration. The patient was found to have a large haemorrhagic torted ovarian cyst measuring 7×3 cm, which ruptured on mobilisation. The attached ovary appeared ischaemic (). The cyst had torted through a full 360º. The ovary’s appearance did not change following detorsion. After detorting the cyst, senior gynaecology input was sought. The advice was that, in spite of the appearance of the ovary, there was potential for it to recover and retain function, and so both the ovary and the cyst were carefully placed back into the pelvis. The wound was closed as per normal.\nOver the following 24 hours, the WBC normalised and the patient improved clinically. She was discharged home and has recovered well. |
A 24 year old male presented with blunt chest pain and respiratory distress. A CT scan was performed which showed a huge heterogeneously enhancing soft tissue mass having solid component, fluid loculi and amorphous calcifications occupying the left hemithorax. It measures 18.5 X 15.8 X 7.6. The main bulk of the tumor was in the lower half and it reached inferiorly upto the left costophrenic sulcus. It was extending along the pleural surface reaching upto the lung apex. The left lung was almost encased by this mass. Similar huge soft tissue density masses were seen on the mediastinal surface of left sided pleura where it took the form of conglomerate mass in the superior mediastinum and on the lateral aspect of pulmonary trunk and heart. Mediastinum was pushed to right side. The main pulmonary trunk showed extrinsic compression and left main pulmonary trunk was severely compressed. Extrinsic compression of left atrium was also noted. There was no pleural effusion or bony erosion by the mass. Right lung was normal (Fig. ). In two initial ultrasound guided preoperative needle biopsies, diagnosis of chondroid hamartoma and sarcomatoid mesothelioma were favored from outside institutions. Bronchoscopy didn’t reveal any intraluminal growth. Metastatic workup including CT scan abdomen and bone scan were unremarkable. Serum tumor markers including lactate dehydrogenase, alpha fetoprotein, beta human chorionic gonadotropin were within normal limits. Thoracotomy with excision of the tumor was planned and intraoperative consultation was requested in which an initial diagnosis of chondroid neoplasm was given with final diagnosis deferred till permanent sections.\nOperative findings include multilobulated and nodular large glistening white tumor located in anterior mediastinum adherent to parietal pleura and effacing the pulmonary parenchyma. However, tumor didn’t appear to invade the lung parenchyma and there was no trachea-bronchial connection. Borders of the tumor were well defined. Tumor was not firmly adherent to the mediastinal structures, pericardium and pleura and was easily scooped out during surgery. There was no apparent invasion into any of the adjacent structures. During surgery left thoracotomy incision was given and pleural cavity is entered through 7th intercostal space. Excision of the tumor was done and cavity was repaired.\nThe specimen received in histopathology department is composed of multiple white glistening nodules of tumor with myxoidy cut surface measuring 20 X 10 cm in aggregate. Twenty two sections from the tumor were submitted. Microscopic examination show neoplastic lesion composed of nests, cords and trabeculae of small to medium sized cells with round nuclei and clear cytoplasm. Background showed myxoid appearance with areas of cartilaginous differentiation. Foci of metaplastic bone formation were also noted. Borders of the tumor were well defined and no invasive into adjacent soft tissue noted. There was not ductal differentiation in tumor cells. No germ cell component (including teratomatous component) noted. No significant atypia, necrosis or mitotic activity was seen in tumor cells (Fig. ). Immunohistochemical stains were also performed. Tumors cells showed positive expression with CKAE1/AE3, CK7, Vimentin, MIC2, S100, ASMA, p63 and GFAP immunostains while CK20, LCA, Chromogranin A, calretinin, TTF1 and CD5 were negative (Fig. ). Focal expression of CD117 and WT1 (cytoplasmic) was also noted. On the basis of these morphologic features and immunohistochemical profile a diagnosis of benign myoepithelioma was favored. Cytogenetic studies were not performed. Postoperative course was unremarkable. No recurrence or metastasis was observed at 6 months follow up. |
A 58-year-old male presented to our clinic with a one-month history of ulcer on the left lower leg (). One month ago, an off-white patch with an erythematous halo appeared on his left lower leg during the treatment of autosensitization dermatitis. Approximately eight weeks ago, the patient topically administered traditional Chinese medicine on the lower legs to prevent eczema. In the next few days, red patches appeared on the lower legs, accompanied by pruritus. The patient was diagnosed with drug eruption and was treated with antibiotics and antihistamine for approximately three weeks at a local hospital. During the subsequent three weeks, erosion appeared on the left lower leg, and the skin lesions extended to the trunk and upper extremity, accompanied by intense itching. The patient was admitted to the Inpatient Department and was diagnosed with autosensitization dermatitis. This patient was treated with betamethasone by intramuscular injection, venous antibiotics, oral antihistamine, and topical glucocorticoid. One month ago, the previous lesions in the trunk and upper extremity improved, but a new off-white patch with an erythematous halo appeared on the left lower leg, with a size of 5×6 cm. This patient did not have diabetes mellitus or other immunosuppressive conditions.\nBased on the skin manifestation, pyoderma gangrenosum (PG) was considered. Meanwhile, oral glucocorticoids were started, betamethasone was injected into the lesion, and a pathological examination was performed. However, the lesion aggravated to a painful ulcer with rapid growth in the next few days. After broad non-septate hyphae with right-angled branches were found in the dermis in the skin pathology examination, the patient was diagnosed with suspected skin mucormycosis. Interestingly, the KOH examination of the crust on the ulcer revealed a thin septate hypha (). The crust culture under 25°C revealed T. rubrum growth.\nThe disease was confirmed as mixed fungal infection caused by T. rubrum and fungi of subphylum Mucormycotina. Based on this, the investigators decided to start treatment with oral itraconazole of 200 mg bid and topical amphotericin B solution. In addition, surgical debridement was performed, and all devitalized tissues were removed. After two months, the improvement on the lesion was significant. After five months, the lesion totally healed. |
45 year old female patient was brought to our hospital with 4 months history of loss of interest in routine daily activities and inactiveness. The symptoms were insidious in onset with rapid progression over 4 months and sudden worsening in 1 week prior to presentation. There was no history of emotional lability, hallucinations or memory disturbances. There was no history of falls, and no sensory or motor deficits.\nHer general examination and vital signs were unremarkable. Neurological examination revealed severe apathy and impaired attention span and needed repeated coaxing to cooperate. Speech was hypophonic. She could follow single commands. Naming was normal but reading and writing were impaired. Mini Mental State Examination (MMSE) score was 18/30. Bilateral Papilledema and reduced abduction of both eyes was noted. The other cranial nerves were normal. There was evidence of rigidity in all four limbs and bradykinesia was present. Power was normal and all reflexes appeared exaggerated. Plantars were extensor. Gait was slow with narrow base and slow shuffling associated with body bradykinesia. There were no signs of meningeal irritation.\nHer baseline hematological and biochemical investigations were normal apart from peripheral blood smear which revealed microcytic hypochromic picture. Magnetic Resonance Imaging (MRI) brain was done () revealing diffuse deep white matter and periventricular T2 and FLAIR hyperintensities bilaterally which was followed by Digital Subtraction Angiography (DSA) (), which revealed torcular and Superior sagittal sinus DAVFs with arterial feeders from Internal Carotid Artery (ICA) via marginal tentorial artery and from External Carotid artery (ECA) through occipital and middle meningeal artery branches.\nIn view of the presentation with rapidly progressive dementia with parkinsonism and clinical findings of apathy, cerebral dysfunction, raised ICP and parkinsonism with features of pyramidal dysfunction all likely due to venous congestion secondary to DAVF demonstrated in DSA, she underwent endovascular intervention in the form of Squid embolization of the fistula. The arterial feeders to the right Transverse and sigmoid sinuses and torcula as well as the superior sagittal sinus were embolized.\nPost procedure her sensorium improved slowly. She was discharged with advise to follow up. Features of parkinsonism persisted for a few weeks for which Levodopa + Carbidopa was initiated. Over the next 6 months, patient made good progress, became asymptomatic without any residual features of parkinsonism and medication were stopped and she was back to her routine daily activities. A check DSA done after 6 months revealed a residual/recurrent DAVF at the torcula () for which she underwent a second embolization procedure a few weeks later. She continued to remain on follow up. Presently she is asymptomatic without any residual neurodeficit and continuing with all her routine daily activities without hindrance. She underwent an MRI scan during follow up which revealed significant resolution of the white matter hyperintensities which were previously seen (). She is planned to undergo another check DSA a year later to reassess the status. |
The 38-year-old male patient was in a car accident in January 2007 and fractured his right radius. The fracture was exposed (Gustilo Type 2). Before presenting at the Codivilla-Putti Hospital in September 2007, his medical records show that, after initial trauma care, he was subjected to immobilization using a cast and that his fracture was reduced and fixated with a plate and screw system (brand unknown) and bone grafts (unknown) in March 2007. Bone grafting was performed because bone loss occurred following the traumatic fracture. His medical records also indicated that in May 2007 he had suffered from a wound dehiscence that was negative for infection (it is not known whether the natient underwent antibiotic therapy). The next month, the plate and screws were removed, and an external fixation system (brand unknown) was placed.\nHe presented at the Codivilla Hospital complaining of pain and presenting a non-suppurating fistula at the scar site of the previous operations. A radiograph was taken and showed a non-union and bone loss (). In November 2007, the patient underwent surgery aimed at completely debriding the site. A cement spacer containing gentamicin and clindamycin (Copal G+C, Heraeus, Hanau, Germany) , alo ng with vancomycin, was placed into the gap (). At this point, the surgical site was positive for Staphylococcus aureus. 3 months later, in February 2008, as a fistula developed again, the spacer was replaced with a new one (same source as the previous surgery). The antibiotic therapy was interrupted 2 weeks before spacer replacement for allowing bacteria strain identification. Before spacer replacement, a white blood cell scintigraphy, laboratory tests for inflammation, including erythrocyte sedimentation rate, C- reactive protein, and complete blood count were performed and radiography was taken. Based on the results of antibiograms, imipenem and vancomycin also were added, and oral antibiotics were also prescribed (ciprofloxacin, 750 mg twice a day). 3 months later, in May 2008, the fistula had disappeared, and the patient’s leukocyte scintigraphy was negative. After 3 more months, in August 2008, the patient underwent reconstruction using a vascularized fibula graft (). At the subsequent control appointments, radiographs showed nonunion of the graft (). 8 months later, in April 2009, the graft and the osteosynthesis devices that had been concomitantly placed were removed, and a third spacer was positioned.\nIn July 2009, the patient underwent a final surgery to place autogenous bone from the iliac crest; equine bone paste containing equine DBM (Osteoplant Activagen Mouldable Paste, Bioteck, Vicenza, Italy); equine DBM granules (Osteoplant Activagen, Bioteck); autologous BMC obtained during the surgery using a dedicated system (Marrowstim, Biomet, Warsaw, USA); and autologous PRP previously prepared according to standard protocols by the hospital blood transfusion service. Very briefly, the PRP was prepared from a sample of the patient’s blood, using differential centrifugation to separate PRP from platelet-poor plasma and red blood cells. This process increased the platelet concentration approximately four- to five-fold compared to the initial blood sample. Before the application, platelets were activated with thrombin and calcium gluconate. The equine bone paste is a mixture of equine cancellous bone granules (1-2 mm), equine DBM granules (1-2 mm), equine collagen, equine bone powder (<0.2 mm), and water-based gel. The equine bone components are made non-antigenic through an enzyme-based processing method that leaves bone collagen unaltered []. The concentration of the different components is adjusted to make the paste easily moldable. Equine DBM granules are manufactured by applying the same process as that used for equine bone and then subjecting the granules to complete acid demineralization.\nOn the day of the surgery, the patient underwent general anesthesia, and two operatory fields were prepared, one at the right iliac crest and one at the right radius. After gaining proper access to the iliac crest through a small incision and removing part of the cortical bone, autogenous cancellous chips were collected using a surgical spoon. Approximately 60 cc of bone-marrow aspirate was collected from the same iliac access, inserting a 5-hole biopsy needle in different directions to optimize the collection of mesenchymal cells. The chips (10 cc), equine DBM (2 cc), equine bone paste (5 cc), BMC (6 cc), and PRP (4 cc) were carefully mixed in a bowel (). A small amount of calcium gluconate was added to activate the PRP and induce clotting. The mixture was then placed in a pre-cut sterile syringe to shape it, after clotting, as a compact cylinder.\nThe surgical site at the radius was opened, and the spacer was removed (). The two bone extremities were stabilized with a metal fixation plate, and the approximately 7.5-cm-long gap was filled with the mixture, extruded from the pre-cut syringe ( and 8).\nAt 60 days, radiographs () showed that the graft was still undergoing remodeling. However, at 6 months, radiographs () showed that the graft had undergone significant remodeling; its radiolucency had increased significantly. |
A 78-year-old male presented to the emergency department with a history of acute left shoulder pain for 6 hours. The pain started suddenly and woke the patient up from his sleep. It was diffuse throughout the left shoulder, stabbing in character and radiating down to the mid-arm. No prior heavy weight lifting or trauma. He had mild epigastric discomfort that was relieved by passing gas. The patient reported no fever, chills, or sweating. His medical history was notable for diabetes, hypertension, chronic kidney disease not requiring dialysis, alcoholic cirrhosis, and sarcoidosis. Medications included glimepiride, olmesartan, furosemide, and sodium bicarbonate. The patient was a retired worker whose earlier job included heavy weight lifting during his youth. The patient reported a history of smoking and alcohol intake in the past, but had quit about three years earlier to his presentation. Two weeks before this presentation, he had been admitted for an episode of infectious colitis, for which he was prescribed levofloxacin 750 mg daily for five days.\nThe patient was afebrile and vitally stable but appeared in acute pain and holding his left arm with his other arm, avoiding any motion. Examination of the shoulder joint showed marked tenderness on palpation, moderate swelling compared to the contralateral side, and limitation of range of motion due to pain. The pain was elicited on both passive and active movement of the left upper limb in any direction—no deformity, erythema, or warmth. The examination of the rest of the left upper extremity was unremarkable. Scleral icterus was noticed on eye examination. Examination of the abdomen and chest were normal as well.\nBlood levels of inflammatory markers were significantly elevated with an erythrocyte sedimentation rate (ESR) level of 33 mm/hr (reference range < 10 mm/hr), C-reactive protein (CRP) level of 103 mg/l (reference range < 8 mg/l) with normal white cell count. The rest of the labs were unchanged from baseline, with an elevated total bilirubin of 5.1 mg/dl (reference range 0.3-1 mg/dl), creatinine of 1.5 mg/dl (reference range 0.7-1.3 mg/dl), chronically low magnesium of 1.4 mg/dl (reference range 1.9-2.7 mg/dl), platelets count of 64,000/ul, and macrocytic anemia with hemoglobin of 13.8 g/dl and mean corpuscular volume of 105 fl (mg/dl 80-95 fl). Elevated ACE levels of 106 U/l were also noticed (reference range 9-67 U/l). Shoulder X-rays showed bilateral severe acromioclavicular osteoarthritis. Left shoulder MRI showed a complete tear of the supraspinatus tendon, subscapularis tendon, biceps-labral complex, glenohumeral effusion extending to the subdeltoid, and subacromial bursa due to the full thickness tear of the supraspinatus tendon ().\nMultiple attempts to aspirate the glenohumeral fluid for analysis were unsuccessful. The patient was treated with analgesia and rest. The pain improved gradually over two weeks. |
The present case is a 40 year old male patient presented to us with a swelling of the right knee joint of one duration, associated with pain and inability to bear weight on the limb for the past 2 months. He was investigated with radiographs []. The radiographs reveal a geographic pattern of Ludloff’s type 1, with a breach in the posterior cortex. The patient was evaluated for the fitness for anesthesia and for the surgical procedure. We had taken an anteromedial approach to the knee joint. A radical (wide) excision of the tumor was done taking care of avoiding macroscopic spillage of the tumor mass in the operating field. Then the patella was enucleated without disturbing the extensor mechanism of the Quadriceps. Then the ipsilateral Fibula was excised and cut into two struts. The Patella was cut into two fragments. The articular surfaces of the patellar fragments were facing the both condyles of the femur and supported by two Fibular struts vide in []. Thus the joint was reconstructed and the whole construct was stabilized with multiple K wires, a cortical screw and the Quadriceps mechanism was retained by fixing the Patellar tendon to the nearby bone and the limb was immobilized in a plaster of Paris splint. The post op period was uneventful. The plaster was removed after the radiological union was evident and allowed range of movements as much as possible. After a period of one year there was a fracture of lateral Fibular strut which was again re-enforced by one more Fibular strut and followed the same post-op protocol as before. During the follow up period of about 15 years there was good consolidation of the Fibular struts and a reasonably good joint preservation as evident in the post op clinical photographs, video Fig no. & , and the clinical video, vide video no.1r |
A 68-year-old Bosnian female with prior aortic valve replacement with a bioprosthetic valve for aortic regurgitation was admitted to our facility for worsening shortness of breath, fever, and lethargy. She was on a vacation in Bosnia, where she fell ill, was hospitalized for 1 month, and was treated for suspected sepsis and renal failure. Medical records from her prior hospitalization in Bosnia were not available. She had an extensive past medical history, pertinent for coronary artery disease, diastolic congestive heart failure, atrial fibrillation, chronic kidney disease, systemic hypertension, cerebrovascular accidents, and chronic urinary tract infection (UTI). Physical examination revealed neck vein distention, bibasal crackles, and bilateral pitting edema. Auscultation revealed an irregularly irregular heart rhythm, a grade 3/6 systolic ejection murmur in the left lower sternal border, and a grade 2/6 early diastolic murmur. EKG revealed atrial fibrillation with low voltage QRS, without evidence of bundle branch blocks or conduction delays. Within an hour, she became markedly hypotensive and hypoxic. She was subsequently intubated and started on a dopamine and norepinephrine infusion. The patient was treated for septic shock with intravenous vancomycin and meropenem. Two sets of blood cultures showed growth of Enterococcus faecalis that was sensitive to penicillin and vancomycin. A bedside transthoracic echocardiogram (TTE) revealed new paravalvular leakage around the bioprosthetic aortic valve, raising concern for an aortic root abscess. She was transferred to a tertiary care facility for possible surgical intervention. A transesophageal echocardiogram (TEE) revealed an extensive aortic root abscess involving the entire root and base of the anterior mitral leaflet. The abscess had ruptured into the left atrium, which contained a 5–7 cm cystic mass, with a fistula connecting from the aortic root to the left atrial cavity, running through the mass. The abscess was located around the bioprosthetic aortic valve which had a large vegetation and paravalvular leak with severe paravalvular aortic regurgitation (Figures \n–). She became hemodynamically unstable during the TEE and was brought to the operating room for emergent surgery. She underwent homograft aortic valve replacement, aortic root replacement, VSD repair, and ligation of the aortico-left atrial fistula. Tissue samples from the excised aortic root and bioprosthetic valve also revealed growth of Enterococcus faecalis again found to be sensitive to penicillin and vancomycin. Postoperatively, she developed worsening septic shock. She was subsequently treated with ampicillin and gentamicin. She remained intubated and required vasopressor support with milrinone, norepinephrine, phenylephrine, and vasopressin. She became anuric and was placed on CVVHD support. Her leucocyte count as well as INR continued to increase and she progressively became more thrombocytopenic and eventually succumbed to disseminated intravascular coagulation from septic shock. |
A 25-month-old female patient presented with recurrent mass lesion of the sinonasal tract. According to her history, she had feeding difficulties and nasal obstruction since birth. Microphthalmia on the right side was also noticed. Examination revealed mass lesion in the right nasal cavity and maxilla, however, biopsy was noninformative. Then, in October 2015 at the age of eight months, the patient was admitted to the department of maxillofacial surgery of local pediatric hospital. Computed tomography (CT) scans were obtained demonstrating a widespread tumor in the right nasal cavity with severe dislocation of the nasal septum, involving the right maxilla, ethmoid labyrinth, orbit, and cranial base (Figure , , ). In November 2015, the lesion was resected via a lateral rhinotomy in a piecemeal fashion until the bony boundaries of the maxillary antrum were reached around the tumor mass. Postoperative CT scans showed tumor remnants along the lateral nasal wall in proximity to the orbit (Figure ). No complications occurred after surgery. Histologic examination diagnosed chondromesenchymal hamartoma.\nThe patient presented at N.N. Burdenko National Research Center for Neurosurgery (Moscow, Russia) to obtain consultations concerning the management of the remaining lesion. New MRI obtained in August 2016 (Figure , left) showed remnants of the tumor without any deficit, and further follow-up was recommended.\nThe histological specimens were examined in the pathology department, and the diagnosis of NCMH was confirmed.\nOn low magnification, the resected material showed different histological patterns. It consisted of cellular cartilaginous islands and areas that contained fibro-osseous and mesenchymal components (Figure ). The cartilaginous component was composed of cellular cartilage foci with a hyaline cartilaginous matrix. The cells of that foci had a very low level of mitotic activity, and no signs of atypia were found (Figure ).\nA mesenchymal component was represented by quite cellular zones consisting of plump fibroblast-like cells without any mitotic figures and atypia (Figure ). Multiple bone trabeculae (Figure ) and irregular osteoid matrix (blue on Masson – Figure ) were found upon the connective tissue background. Areas of preexisting bone tissue with lamellar structure were present (Figure ).\nImmunohistochemical study with antibodies to SMA, S-100 protein, vimentin, MDM2, CDK4, desmin, CD34, and Ki-67 was performed. The cells of the cartilaginous component were strongly positive for vimentin (Figure ) and S-100 protein (Figure ). The cells of the mesenchymal component are positive for vimentin and focally positive for SMA (Figures and 4D). Immunohistochemical reactions with MDM2 (Figure ) and PanCK (AE1/AE3) were negative, positive control for PanCK was present in the mucosal epithelium (Figure ). Proliferative activity was very low according to Ki67 expression (Figure ).\nIn January 2017 (Figure , right) the next follow-up MRI study showed a progression of the lesion, and surgical treatment was indicated. Since the tumor was limited to the nasal cavity, ethmoid labyrinth, maxillary sinus, and was situated extradurally, the second surgery was performed using endoscopic endonasal technique (March 2017). No distinct margins of the lesion were detectable intraoperatively, therefore the superior portion of the tumor was left in order to avoid skull base penetration. Post-operative course was uneventful, no cerebrospinal fluid leak was detected.\nThe material from the second surgery was represented by hypocellular connective tissue with reactive inflammatory infiltration (Figure ), and the same irregular osteoid matrix (Figure ). In this case, the cartilaginous component was not found.\nAt six months of follow-up, the magnetic resonance imaging (MRI) demonstrated clear nasal cavity and remaining tumor mass in the ethmoid roof and sphenoid sinus (Figure , top). At one year, after the second surgery inflammatory changes had regressed, the tumor is stable and exhibits no increase in the volume (Figure , bottom). |
Our patient was a 39-year-old female with a history of hypertension and preeclampsia who presented to an outpatient visit with sudden onset, severe, substernal chest pain. Other associated symptoms at the time of presentation were diaphoresis, dizziness, and nausea. An electrocardiogram was performed and demonstrated normal sinus rhythm with ST depression V3-V4 and ST elevation in V5-V6. The patient was subsequently transferred to the emergency department for further evaluation. Minutes later, she became unresponsive and was found to be in pulseless ventricular fibrillation. Advanced cardiovascular life support was initiated providing two doses of epinephrine along with one dose of intravenous amiodarone. After three attempts of defibrillation, we achieved successful return of circulation. Bedside echocardiogram demonstrated lateral akinesis with an estimated left ventricular ejection fraction of 10–15%. She was urgently transferred to the cardiac catheterization laboratory, where she had a recurrence of ventricular fibrillation requiring one successful defibrillation attempt. Her cardiac catheterization [] demonstrated 100% stenosis of a large first obtuse marginal artery consistent with spontaneous dissection. Initially, an attempt at percutaneous coronary intervention was made. However, given the nature of the dissection, the patient was managed medically. Following catheterization, she continued to decompensate into cardiogenic shock with elevated lactate and hypotension requiring epinephrine continuous infusion and venoarterial extracorporeal membrane oxygenation (VA-ECMO) with 2.5 L/min of support. An echocardiogram performed while on VA-ECMO revealed a left ventricular ejection fraction of 45–50% with severe hypokinesis of the mid to distal inferolateral, mid anterolateral, and the mid anterior wall. She improved clinically throughout the rest of her stay. She was successfully weaned off VA-ECMO on the third hospitalization day.\nAfter stabilization, she underwent further evaluation for underlying vascular pathology. Computed tomography (CT) angiograms of the chest, abdomen, and pelvis were unremarkable, notably with normal renal arteries. CT angiograms of the head and neck revealed bilateral abnormalities of the cervical internal carotid arteries consistent with FMD. A small dissection of the right internal carotid artery and dissection/aneurysm formation of the left cervical internal carotid artery were also noted []. Additionally, high-grade stenosis of the right A3 segment, a subacute posterior cerebellar infarct, and chronic right lacunar infarct were identified. Evaluation by the neurology team revealed no corresponding neurological deficits. On day five, she underwent a cardiac magnetic resonance imaging (MRI), which showed a transmural ‘5-segment’ infarction involving the basal anterolateral, mid-lateral, apical inferior, and apical lateral walls with evidence of tissue edema and microvascular obstruction indicating an acute cardiac injury in the context of edema and inflammation []. This was felt to be consistent with findings of the previous coronary catheterization. The cardiac MRI also showed recovery of the left ventricular systolic function with an ejection fraction of 54%.\nGenetic testing via microarray to screen for an underlying connective tissue disease was negative for any pathogenic deletions or duplications. Additional workup included antineutrophil cytoplasmic antibodies (c-ANCA, p-ANCA), antineutrophil antibodies, erythrocyte sedimentation rate, and complement C3/C4, which were all within normal limits or negative. The patient improved clinically, and on day 10, she was discharged on aspirin, metoprolol tartrate, and losartan. |
We present a case of a 70-year-old male with no pertinent past medical history who presented to the ED after a syncopal episode at the workplace, preceded by headaches and vision changes. The patient was brought to the emergency room where he was found to be in hypertensive emergency, complaining of lightheadedness and episodes of vomiting. The physical exam was unremarkable. Initial CT head showed intracranial subdural hematoma, subarachnoidal and interventricular hemorrhage. Further trauma imaging was negative. Further imaging computed tomography angiography (CTA) head and neck showed stable intracranial hemorrhage, without evidence of aneurysm, high-grade stenosis, or occlusion. Magnetic resonance (MR) brain on initial admission showed multiple subacute resolving hemorrhages. A focal 1.5 cm rim-enhancing lesion about the right posterolateral temporal lobe with associated blooming on gradient recall echo (GRE) and T2 prolongation was presumed to be a subacute hemorrhagic contusion in the setting of trauma (Figure -C).\nA month later, while undergoing follow-up CT head, a new temporal acute hemorrhage was noted and the patient was hospitalized to the medical intensive care unit. His main complaint was intermittent weakness of the left upper extremity since discharge, but the physical and neurological examinations were unremarkable.\nDue to the unclear etiology of ICH, the patient had a repeat MRI. Much to our surprise, this follow-up MRI showed a significant increase in the size of the previously seen right temporal hematoma with heterogeneous thick peripheral mass-like enhancement with areas of hemorrhage which extended to the adjacent dura. This much uglier appearance and rapidly increasing size were now concerning for either recurrent bleed perhaps into an underlying primary or metastatic hemorrhagic neoplasm.\nThe patient underwent right temporal craniotomy with tumor resection. Pathology showed glioblastoma, WHO grade IV, negative for IDH1 R132H mutation by immunohistochemistry, no epidermal growth factor receptor (EGFR) amplification. Additional molecular workup: positive for Telomerase reverse transcriptase promoter mutation, negative for expression of EGFR deletion mutant. No MGMT methylation detected.\nThe postoperative course was uncomplicated, and the patient was discharged home to follow up with Medical and Radiation Oncology. He completed adjuvant chemoradiation therapy with oral temodar and was on monthly pulsed adjuvant temozolomide (TMZ).\nNine months after tumor resection and whilst still on therapy, the patient was admitted for a new-onset seizure. Imaging revealed tumor recurrence at the surgical bed in the temporal lobe with an additional lesion in the right occipital lobe (Figures , -K). The patient underwent surgical resection for the second time. Thereafter, he was shortly readmitted with lethargy, fatigue, and confusion. At this point, the family opted for palliative care, and a home hospice program was set up and no further treatment was pursued. |
A 53-year-old female with no previous history visited a local hospital due to right wrist pain and swelling caused by falling. She was diagnosed with a distal fracture of the right radius, underwent splinting, and returned home. When she visited the local hospital again 2 days after the injury, blister formation on the right forearm was observed, and she was referred to our hospital. The blister was observed along the splint application area () and was considered to be due to the heat and stuffiness of the splint. Plain X-ray examination revealed a distal radius fracture accompanied by dorsal displacement of the distal bone fragment (AO classification: type A2) (Figures and ). Based on the skin condition, we considered conservative treatment by external fixation using a splint or cast to be difficult, and surgery after improvement of the skin state would be more invasive due to bone union and, therefore, planned minimally invasive locking plate osteosynthesis.\nAs we previously reported, surgery was performed using the Henry approach through a 10 mm incision starting from 15 mm proximal to the radial styloid process at 9 days after injury []. In this patient, there was no skin lesion at this incision site, which allowed this surgical technique (). After reduction of the distal bone fragment using a Kirschner wire, osteosynthesis was performed using a volar locking plate (Acu-Loc 2 proximal plate standard, Nihon Medical Next, Osaka Japan) (Figures and ). After the operation, a favorable alignment was obtained (Figures and ). The wrist was immobilized postoperatively in a bulky dressing without an arm splint until the tissue swelling had decreased.\nAll muscles, vessels, and nerves of the anterior compartment—except the radial artery—were retracted ulnarly. The pronator quadratus was incised transversely at its distal portion and dissected off the periosteum using a periosteal elevator preserving its ulnar and radial insertions. Therefore, active finger motion was encouraged immediately after the operation and wrist mobilization was started as soon, and as much, as pain allowed. Moreover, this approach can avoid the median nerve damages during the surgery.\nSix months after the operation, favorable union of the radius was obtained. The wrist range of motion was as follows: flexion, 70°; extension, 65°; pronation, 85°; and supination, 85°. The visual analogue scale (VAS) was 1/10, and the quick disabilities of the arm, shoulder, and hand (Q-DASH) score was 20.45/100. The Mayo wrist score was 85/100 (excellent). The state of the forearm skin and surgical wound favorably improved (), and she returned to her preinjury job. |
We present a case of a 40-year-old Hispanic male with no prior psychiatric history and a medical history of hypertension and end-stage renal disease (ESRD). He was recommended to visit the emergency department (ED) after he was dizzy with a systolic blood pressure in the 80s following an outpatient dialysis session. By the time he presented in the ED, his blood pressure was 209/125 mmHg with the resolution of his dizziness and he was admitted for hypertensive crisis. The patient reported intermittent non-compliance with his home clonidine. In addition to starting his home hypertensive medications in the ED, ciprofloxacin, and metronidazole were initiated because of his complaint of diffuse abdominal pain for two days and concern for enteritis on computed tomography (CT) of the abdomen.\nOn hospital admission day two, the patient began displaying episodes of confusion and agitation (Table ). He pulled out his intravenous lines, climbed onto the windowsill, and wrote “help” on the window. On day four, he reported hearing voices of his boss’s wife and seeing a person from his dialysis center walking around. The next day, he was calm and pleasant during morning rounds but then bit his right thumb a few minutes later. Despite several staff members holding him down, he bit off his distal thumb and did not appear to be in any pain. He was placed in hard restraints and haloperidol 5mg was administered intramuscularly (IM). The patient became calmer and asked the staff to forgive him. However, within a few seconds, he bit off his distal left index finger. Then, he tried pulling out his permacath with his teeth and spat at staff. He required three administrations of lorazepam 2mg IM before his agitation resolved. He appeared confused throughout this incident and could not explain why he was agitated. Later that night, he verbalized that the “devil” bit his fingers. Plastic surgeons were unable to reattach the avulsed sections of his fingers. He continued to be confused and agitated on day six, but to a lesser degree compared to the previous day. On day seven, the patient stated that he was “dreaming” when he was biting his fingers and was unable to stop himself. For the remainder of the hospitalization, his mental status was improved and stable except for occasional mild agitation.\nThe patient denied any headaches, dizziness, nausea, dyspnea, chest pain, weakness, or vision changes. His systolic blood pressure was most consistently in or near the critical range (i.e. above 180 mmHg) between days one-six (Table ) and required intravenous hydralazine and labetalol daily during this time period. His other vital signs were within normal range except for occasional tachycardia and his neurological examination was unremarkable. There were no significant changes in his electrolyte or glucose levels (Table ). However, his laboratory results showed evidence of his ESRD including elevated blood urea nitrogen and creatinine levels which fluctuated with his thrice-weekly dialysis (Table ), chronic anemia, and secondary hyperparathyroidism. The patient had no known family psychiatric history. He denied any illicit drug use. Urine toxicology was not performed due to oliguria. The patient scored a 16/30 on Montreal Cognitive Assessment on day four. Brain non-contrast computed tomography (CT) on day seven showed possible cerebellar fullness with narrowing of the sulci and subtle asymmetry posteriorly within the parietal and occipital lobes. Electroencephalogram (EEG) on day 10 was consistent with mild or resolving encephalopathy and possible drug effects.\nHis blood pressure medication regimen was optimized by starting labetalol, increasing losartan, continuing nifedipine, and discontinuing metoprolol and clonidine. Ciprofloxacin and metronidazole were stopped after six to seven days as the most likely etiology of his abdominal pain was viral gastroenteritis considering the absence of any other gastrointestinal symptoms and resolution by day three. He received risperidone for four days as well as haloperidol and lorazepam as needed for control of his agitation. On the day of discharge (day 14), the patient’s blood pressure was better controlled (systolic: 134–144 mmHg, diastolic: 80–93 mmHg) and he was fully oriented, calm, and cooperative. He did not remember any of his episodes of agitation but was remorseful for his actions and planned to be fully compliant with his blood pressure medications. At his one-week follow-up visit and multiple visits for urological issues over the next four months, his blood pressure was well-controlled and he did not display any symptoms present during his altered state. |
A 75-year-old right-handed woman presented to the emergency department with headache and mental confusion 6 hours before admission. She had 5 years of formal education and was able to read and write perfectly. There was no prior medical history other than tobacco use for 50 years. Her family reported that she initially complained of difficulties in handling objects with her right hand and they noticed she was confused.\nInitial evaluation revealed a blood pressure of 220 × 120 mmHg and a mild pronator drift of the right upper limb. She was alert and oriented, with no global attention or language abnormalities. Testing was positive for mild right spatial neglect with sensory extinction alone. Her score on the National Institute of Health Stroke Scale (NIHSS) was 1. The patient underwent initial stabilization and a Computed Tomography (CT) scan, which revealed only a residual calcified lesion in the left post-central gyrus. Interestingly, a more detailed cognitive evaluation revealed simultanagnosia (she could not integrate parts of the Boston Cookie Theft Picture or small letters into a global form) and unilateral right optic ataxia (). Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome ().\nCT angiography showed diffuse atheromatosis, with moderate stenosis of 50% in the left carotid bulb due to an ulcerated plaque (). Diffusion-Weighted Imaging MRI revealed small hyperintense lesions in the territory of the left middle cerebral artery, predominantly posterior and cortical (). Serial electroencephalogram testing was negative for epileptiform activity, but there was a focal electrical slowing in the left hemisphere. After a complete neurovascular work-up, we concluded the patient had a stroke due to an evident large artery atherosclerosis mechanism.\nThe patient was treated with dual antiplatelet therapy and a high-potency statin at standard doses for secondary prevention. All cognitive deficits resolved spontaneously within 3 days of admission and she was discharged at day 4 with a modified Rankin-scale of 1 for outpatient follow-up, rehabilitation and smoking cessation. |
A 9-year-old boy presented to the emergency department with palpitations in daily activities, and without any other complications and no past medical history detected. He had such episodes before and was treated by beta blockers (propranolol) without any recuperation during the past year. The patient was clinically stable. His blood pressure was 100/60 mm Hg at the initial, and his pulse rate was 130 beats per minute. In auscultation of both lungs found nothing and abdominal, spleen and liver examination, were normal.\nThe electrocardiography (ECG) detected sinus tachycardia with normal axis (). Although plane chest radiography was normal, an apical four-chamber view echocardiography showed an echolucent space next to the posterior wall of the left ventricle with compression effect (). The mass was extrinsic and confined to the pericardium. The right and left ventricular chamber size and function were normal, and there was no valvular heart disease. Then, a chest CT scan with contrast showed a retrosternal mass characterized with a clear serous fluid with compression on the posterior wall of the left ventricle (). To rule out the causes, inflammatory factors, blood culture, viral antibodies, collagen vascular markers, sputum acid-fast bacilli (AFB) and PPD test results all were normal.\nThe patient was referred to a cardiac surgeon. Surgeon accessed to the mediastinum after median sternotomy, then the pericardium opened, and then an 8×7×6 cm anterior mediastinal cyst filled with necrotic debris was found. The mass was removed and was sent to a pathologist for further evaluation, and it was reported as a pericardial cyst with mesothelial lining and a cyst wall composed of loose connective tissue (). The cyst had thick walls and compressed the left ventricle. After 10 days, the patient was discharged and a 12-month follow-up by transthoracic echocardiogram did not demonstrate any mass in the pericardium (). The patient remains symptom-free after 12 months. |
An 81-year-old male with a history of hypertension, atrial fibrillation, congestive heart failure (CHF) with ejection fraction of 45% presented to the emergency room with progressive shortness of breath for 3 months. He also complained of cough, productive of white sputum with generalized weakness and 10-lb weight loss over the past 2 months. On presentation, the patient was afebrile, with blood pressure of 93/55 mm Hg, and oxy-hemoglobin saturation of 92% on 2 liters of oxygen via nasal cannula. Physical examination revealed crackles over bilateral chest, 1+ lower extremity edema bilaterally. Blood tests showed white count of 12 400. Brain natriuretic peptide level was 454 ng/mL. Cardiac troponin T was negative.\nPostero-anterior chest radiograph revealed round opacity in the right lung. Computed tomography scan of the chest was performed, which confirmed multiple round densities in both the lung fields () along with mild mediastinal lymphadenopathy. The patient was being admitted for treatment with acute systolic CHF exacerbation and was also being worked up for lung malignancy. The patient received diuretics that improved the breathing status.\nDifferential diagnoses at this stage were non–small cell carcinoma of the lung, adenocarcinoma of the lung, or lymphoma due to the presence of the lymphadenopathy. The patient underwent interventional radiology guided core needle biopsy for further evaluation of the lung densities. Immunohistochemical stains were positive for CD30 and CD15 in a population of large atypical cells amid a background of CD3-positive nonneoplastic cells. Stains for CD20 and CD1A were negative. These results combined with the histological findings support the diagnosis of classical Hodgkin’s lymphoma of the lung with histological appearance confirming nodular sclerosis type.\nDue to extensive cardiac disease (CHF, atrial fibrillation, and bioprosthetic aortic valve replacement), the patient could not be started on standard chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine. Instead the patient was started on cyclophosphamide, vincristine, and prednisone. The patient was discharged after medical optimization with follow-up at the cancer center, where he received a cycle of chemotherapy.\nThe patient was readmitted within 20 days for acute respiratory distress with hemodynamic instability. He was intubated and placed on vasopressor support. Clinical and radiological findings suggested acute respiratory distress syndrome. The patient subsequently suffered from a cardiac arrest and could not be resuscitated and expired. |
A 34-year-old male with no significant past medical history presented with generalized body pain and swelling of multiple joints. His symptoms slowly progressed and he developed severe backache and body pain which forced him to walk with support. Pain was so severe that someone had to help him get into his car. He had no history of trauma and was not on any medication. He did not have fever or other systemic symptoms. Initial clinical examinations were normal apart from generalized bone tenderness. The systemic examination did not reveal any features of Cushing’s syndrome.\nThe laboratory investigations showed elevated alkaline phosphatase, low serum phosphate, normal levels of parathyroid hormones (PTH), calcium, and vitamin D (Table ). His routine blood count, renal function, and serum protein electrophoresis were normal. The urine test was negative for Bence Jones protein.\nX-ray of the cervical, thoracic and lumbar spine, shoulder, pelvis, and sacroiliac joint were also normal. Due to multiple joint pain and bone tenderness, a whole body MRI scan was done which was suggestive of osteoporosis with multiple insufficiency fractures of the sacrum, distal end of tibia and fibula, ribs, and acetabulum as well as multiple compression fractures of the vertebral body (Figures -). The bone densitometry showed a T score of -3.5 in the lumbar spine (Figure ).\nA subsequent bone scan revealed multiple foci of increased radiotracer uptake likely from Looser's zones/pseudo fractures and costochondral beadings. Also increased uptake at proximal femoral and tibial growth plates likely from pseudo reactivation of growth plates. Multiple levels of increased vertebral uptake were noticed from compression fractures and at the sacroiliac joints from sacral insufficiency fracture. Findings were highly suggestive of osteomalacia. Left calcaneus three-phase bone scan revealed positive uptake suggestive of insufficiency type stress fracture. Delayed bone phase also showed bilateral distal tarsal/proximal metatarsal uptakes indicative of pseudo fractures/stress fractures (Figure ).\nHe was then reviewed at an endocrine clinic and further work up was done for hypophosphatemia and metabolic bone disease. The tubular maximum reabsorption of phosphate to glomerular filtration rate ratio (TmP-GFR) was 1.9 mg/100 ml (2.5-3.5 mg/dl) suggestive of TIO.\nIn order to locate the tumor, a whole body CT scan was done which detected a vascular lesion arising from the lateral wall of the left nasal cavity at the level of the choana (Figure ). A subsequent nasopharyngoscopy revealed a vascular tumor at the posterior end of the inferior turbinate and the positron emission tomography (PET) scan showed uptake in the same region (Figure ).\nHe underwent excision of the tumor and histology confirmed a hemangiopericytoma. Patient had a marked clinical and biochemical improvement on follow up and started walking independently within few weeks. His repeat bone mineral density after two years showed a T score of -1.1 in the lumbar spine. |
The patient was a 15-year-old Caucasian female diagnosed with autism spectrum disorder and intermittent explosive disorder. She was admitted to the the inpatient unit with suicidal ideations. She had a longstanding history of significant problems with social relationships, anxiety, and impulse control. She reported average academic performance but had difficulties with integrating into a social environment. She also reported a multitude of worries, including not being socially accepted, not performing well in school, and being perceived as socially inadequate. There were no developmental delays reported in this patient. However, there were reports of fainting spells and neonatal jaundice during the patient’s early neonatal life. She denied any previous history of inpatient psychiatric hospitalization but reported receiving outpatient psychiatric treatment. She reported a previous suicide attempt but denied any past medical history. Her medications upon admission were Lexapro 10mg daily and Aripiprazole 2 mg once daily. She denied any substance abuse history. Family history was negative for mental health problems and suicide attempts, but positive for alcohol abuse. On examination, this patient was isolative and impulsive. She reported poor sleep, hopelessness, and racing thoughts. She described her mood as depressed. She reported a long history of being bullied. She also admitted to suicidal ideations with a plan and intent. She denied experiencing hallucinations and also denied any history of physical or sexual abuse. Patient and family requested medication change since she experienced akathisia on Aripiprazole. This patient was started on Lurasidone 20mg PO every night for mood and Guanfacine 0.5mg PO twice daily for impulsivity after admission. This dose of Lurasidone was titrated up to 60mg PO every night. At the beginning of her treatment, the patient demonstrated poor social interactions, emotional dysregulation, and interpersonal relationships problems. The patient reported a noticeable improvement in her mood a few days after treatment began. After nearly two weeks of treatment, suicidal ideations were no longer present. The patient participated more in group activities and demonstrated positive interactions with her peers as the treatment progressed. She also reported that the Lurasidone helped to eliminate negative thinking. The patient reported no side effects from the medication and stated that it was helping her with sleep. |
A 66-year-old Korean male patient presented with an ill-defined erythematous indurated patch on the umbilicus (). The patient had not received any treatment for 1 year as the lesion was asymptomatic. Recently, the patient was referred to our center after an incisional biopsy. The tumor was cleared after three stages of MMS. We received the patient's consent form about publishing all photographic materials.\nHistopathologic examination revealed basaloid tumor cells forming nests and cords in the dermis and subcutaneous tissue (). The tumor showed cribriform or tubular structure without connection to the epidermis. Abundant mucin was observed inside the tubular structure (). Tumor cells had hyperchromatic nuclei but did not demonstrate distinct cellular atypia (). Immunohistochemical study against receptor tyrosine kinase (cKIT) and epithelial membrane antigen (EMA) showed strong positive for tumor cells. The tumor was diagnosed as ACC upon histopathologic examination and immunohistochemical study. Physical examination and whole-body positron emission tomography fused with computed tomography (PET/CT) performed to exclude the possibility of metastasis from other original lesion showed no evidence of additional lesion. Finally, the patient was diagnosed as PCACC.\nIn the process of removing the tumor, the umbilicus was completely eliminated with an asymmetrical defect that extended into the left upper quadrant, which required elaborate reconstruction. The size of the postoperative defect was 2.0×1.8 cm (). An elliptical repair was done at the left side of the defect to close the paramedian defect as well as to remove the dogear. To close the remaining central defect and to recreate the concavity of the umbilicus, about 0.5 cm of the soft tissue was removed from the surgical margin, deeper layered purse string sutures were placed to adjust the size of the umbilicus, and the superficial purse string suture involving the dermal layer was tacked at 3 areas at 12, 4, and 8 o'clock directions to the underlying linea alba (). Concave structure of the umbilicus was recreated at the center of the abdomen (). Follow-up at six months showed a well-healed surgical defect with recreated concave and normal looking umbilicus and its shape was well maintained at 20-month follow-up ().\nThe patient showed no local recurrence or distant metastasis at 30-month follow-up. |
A 51-year-old postmenopausal female presented with the chief complaint of an inferior visual field defect in the right eye and decreased central vision lasting for one day without any other concomitant symptoms. The patient had no significant past medical history, and claimed to have no antecedent ocular trauma. Nothing abnormal was found upon general physical examination. Her best corrected visual acuity (BCVA) was 20/200, and the intraocular pressure was 18 mmHg. Nothing abnormal was found in the anterior segment, and the vitreous was clear, without cells or pigments. Biocular indirect ophthalmoscopy revealed focal retinal detachment involving the area from the macula to the supratemporal vascular arcades, with subretinal depigmentation and pigmentation. A group of spherical subretinal tumors was noted beneath the detached retina. The tumors were semi-translucent, pale, smooth-faced, lobulated, and stationary. Neither proliferous tissue nor retinal breaks were found in the area of retinal detachment (Fig. ). The subretinal fluid did not move with postural change. The results of fluorescein fundus angiography (FFA) were as follows: during the arteriovenous phase, large vascular networks inside the tumor could be seen, and due to a large area of choroid capillary atrophy, large choroidal vessels could be seen around the tumor (Fig. ). The fluorescein gradually accumulated in the tumor over time, but did not leak from the tumor into the subretinal fluid (Fig. ).\nThe initial diagnosis was exudative retinal detachment and a subretinal neoplasm in the right eye.\nThe retina reattached spontaneously seven days after hospitalization. To our surprise, the retina detached again and the subretinal fluid recurred in the same area three days after treatment with antibiotic eye drops. Visual acuity and intraocular pressure did not change. Due to the repeated recurrence of retinal detachment and the unknown nature of the subretinal neoplasm, the patient underwent vitrectomy surgery with informed consent. The subretinal neoplasm was removed, and the retina was reattached with a silicone oil intraocular tamponade during the operation. During the surgery, the subretinal neoplasm was found to be spherical, lobulated and smooth-faced, and to be connected to the white fibrotic tissue under the tumor by a slender peduncle without any synechia to the retina (Fig. ). The tumor was soft, deformable and elastic. The dendritic vascular network could be clearly detected inside the tumor when transilluminated with an optical fiber. The tumor was completely removed from the eye after cutting the peduncle, and was then fixed in formaldehyde. The fundus is shown after removal in Fig. .\nThe excised mass was fixed in 10 % formalin and embedded in paraffin. Several 6-μm sections were cut from each paraffin block, two sections were stained with hematoxylin and eosin (H&E) and periodic acid Schiff-Alcian blue, and the others were stained with various antibodies (immunohistochemistry (IHC)). Immunohistochemical staining was performed using the streptavidin-peroxidase system (Ultrasensitive; Mai Xin Inc., Fuzhou, China) according to the manufacturer’s instructions. Commercially available, pre-diluted monoclonal antibodies against the following antigens were used: cytokeratin (CK), nerve tissue protein S100, glial fibrillary acidic protein (GFAP) (all Thermo Fisher Scientific Inc., Fremont, CA, USA). H&E staining revealed that the tumor was solid and covered with pseudostratified ciliated columnar epithelium containing scattered goblet cells, which were prominently shown by Alcian blue stain (representative goblet cell was shown in Fig. by arrow). There was loose connective tissue just beneath the epithelia, which contained blood vessels, fibroblasts, fibrocytes, lymphocytes, plasmocytes, and macrophages. IHC was positive for CK (Fig. ), but negative for S100 (Fig. ) and GFAP (Fig. ).\nHistologically, the tumor was composed of pseudostratified ciliated columnar epithelium and scattered goblet cells. IHC was positive for CK and negative for S100 and GFAP. Therefore, the tumor was diagnosed as a subretinal heterotopic respiratory epithelium.\nTo avoid overtreatment, including chemotherapy and radiotherapy, differentiating this tumor from other malignant tumors, including amelanotic malignant melanomas and metastatic carcinomas, was important.\nAmelanotic malignant melanomas are rare, lack pigment, and have round edges like the tumor found in this patient. In addition, the FFA results found in patients with malignant melanomas are typically similar to those in this case. However, intraocular malignant melanomas may extend into adjacent intraocular tissues or outside the eye through the scleral canals, or by intravascular invasion. Furthermore, malignant melanomas are composed of spindle-shaped cells, with or without prominent nucleoli, and large epithelioid tumor cells. There was no evidence of adjacent tissue invasion nor typical spindle-shaped cells in this case.\nDue to the rich blood supply of the choroid, it is an important site for blood-borne metastases. In females, carcinoma of the breast is the most common source, while in males, lung, liver, genitourinary, and gastrointestinal malignancies are the usual primary sites. Metastatic carcinomas can have a similar appearance as the tumor in this case, including being pale, smooth-faced and lobulated, and are also stationary. Meanwhile, carcinomas often have serous retinal detachment, which is again, similar to the tumor described in this case. In patients suffering from metastatic carcinomas, metastasis to the choroid usually becomes apparent after diagnosis of the primary malignancy. However, the appearance of a choroidal metastasis may occasionally precede the diagnosis of the primary neoplasm, but upon histological examination, malignant cells can be found in the tissue sections. In this patient, nothing abnormal was found through a detailed general physical checkup, and no malignant cells were found in the tumor.\nAlso, it is necessary to differentiate this tumor from some benign tumors, including choroid hemangiomas and choroid osteomas.\nChoroid hemangiomas and choroid osteomas can appear similar to this tumor in fundus examinations. Especially in choroid hemangiomas, which is more dome-shaped and often has a serous retinal detachment, it is difficult to identify only in fundus examination. Fluorescein Angiography is an important examination to identify the choroidal hemangioma. Typically, during the early phase, there are irregular areas of hyper-fluorescence in choroid hemangiomas. This hyper-fluorescence reflects the filling of vascular spaces within the tumor. This separates the choroidal hemangiomas from other solid, vascular tumors. A persistent hyper-fluorescence in the late phase can be caused by an accumulation of fluorescein in the vascular spaces of the hemangioma, or by pooling of the dye in subretinal fluid.\nChoroidal osteomas are benign tumors of the choroid that are composed of mature bone. These tumors are typically found in healthy young females in the second or third decades of life, are relatively flat, and have pseudopod-like processes on the edges of the lesion. The diagnosis of choroidal osteoma was ruled out in this case since no osteocytes were found in the tissue section. |
M. E. S. M., male, 6 years and 5 months old, from Itapecuru, state of Maranhão, Brazil, with a history of seven recurrences of VL.\nThe child was 3 years and 1 month old (March 2016) when he was first diagnosed with VL based on a clinical, epidemiological, and parasitological diagnosis, and he was treated at that time with MA for 30 days. Over the next two years, he presented with recurrences with clinical manifestations and laboratory alterations compatible with active disease. The myelogram was performed after three relapses. Amastigote forms of Leishmania spp. were found. He presented his first relapse in August 2016, and the MA treatment regimen was repeated for another 30 days. In December 2016, after the second relapse, the patient was treated with LAmB, with a total dose of 35 mg/kg for 10 days, without any change in the splenomegaly. The third recurrence in April 2017 was treated with amphotericin B deoxycholate (DAmB), at a total dose of 32 mg/kg. In August 2017, in the fourth relapse, LAmB was administered at a total dose of 50 mg/kg. After the fifth relapse, which occurred in March 2018, MA was used again for 30 days. After the sixth recurrence, which occurred on July 2018, a total dose of 48 mg/kg of LAmB was administered for 16 days. After each therapeutic regimen, he showed a regression of fever and an improvement in laboratory parameters, despite pancytopenia oscillations, while maintaining a hepatomegaly and a splenomegaly with little variations in their measurements (spleen persistently 10-12 cm from the left costal margin).\nAn immunological evaluation was carried out at the Institute of Tropical Medicine in the state of São Paulo, Brazil. Flow cytometry and cytokine dosage revealed a specific immunosuppression for Leishmania spp., but with a satisfactory cellular and humoral immune response. No primary or secondary immunodeficiencies were documented. For six months, prophylaxis with MA was performed fortnightly, but without disease control. The seventh recurrence, which occurred in August 2018, was treated with LAmB, with a total dose of 50 mg/kg.\nWe opted to perform therapeutic splenectomy in August 2018, because of the persistence of splenomegaly, hepatomegaly, and pancytopenia. The spleen measured 20.5 × 12.0 × 8.0 cm and weighed 1.063 kg. Histopathology revealed the presence of amastigote forms of Leishmania spp. within the splenic histiocytes and perisplenic lymph nodes ().\nAfter the surgical procedure, the child showed clinical improvement. However, 10 months after splenectomy, the child had hepatomegaly, without pancytopenia and without fever. We performed a new myelogram that revealed Leishmania spp. amastigote forms with hypocellular marrow and preserved maturation. He was treated for 30 days commencing in March 2019 with Miltefosine, a drug supplied by the Pan American Health Organization and the Brazilian Ministry of Health upon request after being informed about the patient's condition. During this period, we performed an in-depth immunodeficiency assessment, which did not show lymphoproliferation. The prophylaxis with MA remained biweekly for six months (the patient was still receiving this medication at the time this report was being written, without any clinical manifestations or alterations in the laboratory parameters). |
A 16-year-old male was presented with a history of growth in the upper right back teeth region for 8 days. The growth was sudden in onset and was gradually increasing in size. The patient reported minimum discomfort with a bleeding episode from the growth following toothbrush trauma for 1 day. The family, dental, medical, and surgical history was unremarkable. On general physical examination, mild pallor was detected in conjunctivae and nailbeds. Extraoral examination revealed a solitary diffuse swelling on right middle one-third of the face with no secondary changes in the overlying skin []. There was no evidence of regional or distant lymphadenopathy. On intraoral inspection, a solitary well-defined sessile, proliferative, 3.5 cm × 3 cm, roughly oval growth obliterating the buccal vestibule completely was noted arising from the buccal marginal, interdental, and attached gingivae of maxillary right first and second molars. The surface of the growth appeared irregular and nonlobulated. The color of the growth was white with interspersed dark red areas and blood clots over the surface. There was evidence of pus discharge and bleeding from the growth. Palpation revealed a mildly tender, soft to firm, bleeding friable growth []. A provisional diagnosis of pyogenic granuloma was considered. Peripheral giant cell granuloma, solitary fibrous tumor, hemangioendothelioma, angiosarcoma, amelanotic melanoma, and metastatic lesion were kept in the differential diagnosis. Intraoral periapical radiograph of maxillary right first and second molars showed a homogeneous soft tissue density extending from maxillary right first molar to maxillary right second molar with no evidence of calcifications. Orthopantomograph also revealed similar findings with intact maxillary sinus floor [Figure and ]. Routine blood investigations revealed mild anemic status. Incisional biopsy was performed from the lesion for which histopathological diagnosis of pyogenic granuloma was given. After incisional biopsy, the growth rapidly increased in size within 10 days to reach 6 cm × 4 cm in dimension [Figure and ]. Considering pyogenic granuloma as the suspected diagnosis, excisional biopsy of the lesion was performed under general anesthesia. On excisional biopsy microscopy sections, ulcerated epithelium with underlying connective tissue showed complex branching vascular channels lined by plump endothelial cells. Surrounding tissues showed spindle cells with areas of hemorrhages and necrosis [Figure , , and ]. Immunoreactivity was positive for vimentin, CD31, CD34, and KI-67 marker [Figure , , , and ]. CD107 was reportedly negative. Hence, a final diagnosis of epithelioid HE was made. Thorough physical examination was undertaken to evaluate metastatic status. Fortunately, there was no evidence of local or distant metastasis and the patient was recurrence-free 5 years postsurgery. |
A 40-year-old married male presented with persistent painful erection lasting for 8 days. He did not have diabetes or hypertension and was not on any medications. About 9 days earlier, he presented to a surgeon with left ureteric colic. Preliminary investigations revealed a left lower ureteric stone of 5 mm with no upstream dilation. Complete blood counts ruled out blood disorders such as sickle cell anemia, leukemia, and thrombocytosis. Reticulocyte count, prothrombin time were also normal. There was no history of papaverine injections or drug abuse and urine screening for toxic drugs such as morphine was not performed.\nThe only drug he received was tablet drotaverine 80 mg twice daily for 5 days. The first dose was taken at 7 p.m and on waking up next morning the patient noticed tumescence in the penis. He ignored it, the colic had settled and so he attended his office for the next 2 days, and continued to take drotaverine. He denied having sexual contact or genital stimulation during or after the colic. He had no addictions and was not using any recreational drugs.\nBy the 5thday, he had developed painful persistent erection, consistent with priapism, associated with poor urinary flow and incomplete voiding. He was then referred to us for further management. On arrival, 9 days after the onset of tumescence, the patient was stable and the penile color Doppler showed no flow in the penis [] which confirmed ischemic priapism.\nSeveral attempts to aspirate the cavernosum under general anesthesia failed to draw blood, even for the blood gas study. The patient underwent El-Ghorab shunt surgery. The incision was made on the glans, distal to the corona. Both the cavernous bodies were filled with dark blood and clots. Cavernotomies were made on the distal most part and 5–7 mm of tunic was excised. All the clots and dark blood were milked out from the base of the penis up to the tip which resulted in detumescence of the penis. Glans was sutured with 4/0 polydioxanone suture, and a Foley catheter was placed. Compression dressing was applied. Histopathology of the cavernosal tissues on both the sides showed changes consistent with ischemic priapism []. |
A 58-year-old male with a past medical history of atopic dermatitis and severe psoriasis on Risankizumab (Skyrizi) presented with a chief complaint of progressive left leg swelling for a week associated with erythema and pain. The patient had a chronic history of skin breakdown on his bilateral legs due to psoriasis. He was vacationing in Belize and was walking barefoot on the ocean beaches. Initially, he noticed a burning sensation and increased redness of the left lower limb when he was still in Belize. The swelling, pain, and erythema progressed. The blisters developed a week after initial symptoms (see Figure ) and two days before the arrival in the emergency room. He did not report any fever and chills.\nOn physical examination, he was afebrile with a temperature of 36.8 degree celsius. He was hypotensive with a blood pressure of 88/61 mmHg and had tachycardia with a pulse rate of 114 beats per minute. He had bilateral lower limb erythema, with swollen and tender left leg, and blisters on the lateral aspect of the left leg. He had mild leukocytosis with a white blood cell count of 12,300 cells/microliters with an absolute neutrophil of 8.17(*10^3 cells/microliter). His blood culture showed many Gram-negative rods. The point of care lactate was 2.8 and the sepsis protocol was initiated with IV fluid resuscitation and broad-spectrum antibiotics. The ultrasound Doppler of the bilateral lower limbs ruled out deep vein thrombosis (DVT). He was admitted to the medical ICU for further evaluation and management. The Infectious Disease team was consulted for antibiotics management.\nThe General Surgery was consulted for the clinical concern of necrotizing infection. The CT scan showed no obvious abscess or gas in the subcutaneous tissue. The patient complained of the disproportionate pain in comparison to the erythema and then developed hemorrhagic bullae in the interim. His clinical condition did not improve, and his pain was difficult to control. He underwent surgical intervention for the clinical suspicion of necrotizing fasciitis. The intraoperative findings were consistent with non-necrotizing bullous cellulitis (see Figure ).\nThe blood culture differentiated into Shewanella algae. The wound culture and intraoperative tissue culture also grew Shewanella algae. The patient was initially started on ceftazidime and ciprofloxacin. The micro-organism was pan-sensitive (see Table ), including ciprofloxacin, and the subsequent blood culture from day 2 did not demonstrate any bacterial growth. The patient was discharged on oral ciprofloxacin 750 mg twice a day for 14 days. The patient was seen in the outpatient Infectious Disease clinic after 10 days. The wound was minimally draining and the decision was made to extend antibiotics therapy by one week.\nUnfortunately, the patient was re-admitted for reinfection of the wound with methicillin-resistant Staphylococcus aureus (MRSA) infection. He required wound debridement and the Infectious Disease team recommended a prolonged course of antibiotics. He completed six weeks of IV vancomycin and oral ciprofloxacin following his wound debridement. The wound that was left to heal by secondary intention was healing well and >90% closed in his last Infectious Disease clinic follow up. The IV antibiotics were discontinued at that time. |
A gravida 4, parity two woman aged 54 years who was three years menopausal underwent surgery for cholangiocellular cancer, type B, one year ago (T3BN0, Stage 3B). She received gemcitabine and 5-fluorouracil infusion therapy every two weeks for 8 cycles and chemoradiation with 5-fluorouracil infusions after surgery. After one year, she was admitted to the emergency unit with the main symptoms of abdominal swelling and pain. An abdominal ultrasound scan revealed bilateral ovarian masses and she was referred to our gynecologic oncology section of the department of obstetrics and gynecology. The gynecologic examination of the external genitalia was compatibly normal regarding her age. Bimanual examination revealed a mass that filled the pelvis and extended to the umbilicus. Magnetic resonance imaging showed a cystic, hemorrhagic mass with polypoid protrusions from the cyst wall, measuring 105x95x65 mm, originating from the left ovary. There was also a tumor on the right ovary sized 3.5x4x5 cm, which shared the same properties. Hematologic and biochemical tests were normal. Serum tumor markers calcium (CA) 125 and CA 19-9 were 35.8 U/mL and 1782.7 U/mL, respectively. Upper and lower gastrointestinal system endoscopies were also found normal. She underwent a diagnostic laparotomy. A lobulated 18x20 cm mass that originated from the left ovary and filled the pelvis, and a 5x6-cm cystic septal lesion originating from the right ovary were observed in the lower abdomen (). No tumor formation or implant was diagnosed in the pelvic peritoneum or in the upper abdomen. Peritoneal wash fluid and multiple peritoneal biopsies were taken from all quadrants. The left adnexal mass was excised and sent for frozen section examination. The result of frozen section examination was reported as a malignant tumor with mucinous features. Debulking surgery was performed. Microscopic examination of paraffin sections from bilateral ovaries and omentum revealed mucinous adenocarcinoma (). Malignant cells were also observed in the peritoneal wash fluid. A pathologic evaluation was also performed by comparing the findings of the previous material diagnosed as Klatskin tumor with the wash fluid sample and it was observed that both materials had similar characteristics of the same tumor. Immunohistochemical marker tests were performed to determine the tumor’s primary origin. There was positive staining for CDX2, cytokeratin 19, and cytokeratin 20 detected in the tumor cells. Cytokeratin 7, PAX8, estrogen, and progesterone receptors were found negative. The immunohistochemical findings supported pancreaticobiliary and gastrointestinal origin. The final result of the pathologic examination confirmed a metastatic adenocarcinoma of the hepatic ducts. |
A 17-year-old boy reported to the outpatient Department of Oral Medicine and Radiology with ulcers on his lips, tongue, and the floor of his mouth, which left scars after healing. His medical history revealed that he has been suffering from these episodes for the past 3 years, and has already been treated with various topical agents, steroids, and antibiotics, but reported no significant relief over the past year. The episodes of ulcers had increased. These lesions were extremely painful and interfered with eating, drinking, and speaking. There was no relationship with food or trauma, nor any history of allergies, but he reported flares during periods of emotional stress and during school exams. We recorded his pain on a visual analogue scale (VAS), with a score of 8.\nClinical examination showed an asymptomatic ulcer on the left retro commissure area with a perilesional erythematous halo, which was covered with a pseudo-membrane and was over 10 mm in diameter (a). Ulcers with a similar presentation were seen on the tongue’s left lateral border (b) and on the right upper labial mucosa (c). A diagnosis of major RAU was given based on the history, symptoms, and clinical examination. No relevant medical or family history was noted. Detailed medical history was taken in order to rule out systemic disorders associated with lesions clinically similar to RAS, such as nutritional deficiencies leading to anemia, inflammatory bowel diseases, cyclic neutropenia, Behcet’s syndrome, Sweet’s syndrome, and MAGIC syndrome. There was no history of periodic fever, genital or cutaneous ulcers, pharyngitis, or cervical adenitis. Ophthalmological examination revealed no ocular ulcers. The patient came to us with his previous colonoscopy reports, and they were normal with no sign of inflammatory bowel disease. The patient did not have a history of chronic severe malnutrition, anemia, abdominal pain, or diarrhea. All laboratory investigations—including a complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and levels of folic acid, iron, ferritin, and vitamins B2, B6, and B12—were in the normal range.\nThe patient’s previous therapies included high-potency topical steroids, tacrolimus ointment, chlorhexidine rinses, topical tetracycline, topical lidocaine, oral colchicine (0.4 mg twice daily), oral azathioprine (200 mg/d), and oral prednisone ranging in doses of 10 to 40 mg. Only prednisone out of all the above showed positive results, and the dose required to ease symptoms sufficiently was 40 mg/day over the previous year. For the past year the patient was on prednisone (40 mg/day). Despite this prednisone dose, the patient was not comfortable during food intake and speech articulation, and during exams, the lesions exacerbated further. Hence, we added levamisole to his previous therapeutic regime, and the patient responded dramatically to this combination.\nThe patient was given systemic prednisone (15 mg per day) orally and the immune-modulating agent levamisole (50 mg tab), to be taken 3 times daily (150 mg total dose) for 3 consecutive days per week, and the regime was continued for 3 months. There was a partial regression of the lesions after the first application, and the VAS score was reduced to 3. Two weeks after the administration of the drugs, the patient showed 80% clinical improvement of ulcerations. Further applications brought about partial healing of the ulcers on the left retro commissure area (d) and complete healing of the ulcers on the tongue (e) and upper labial mucosa (f). Over the next several weeks, all remaining ulcerations had healed entirely. Prednisone was stopped after tapering its dosage slowly after 1 month, and levamisole was continued for a further 3-month period. The patient was followed up for one year; there was a significant reduction in the severity, frequency, and number of ulcerations, as well as a significant reduction in pain. During this period, no adverse effects of the drug were observed. |
A boy aged 2 years and 6 months presented with refractory constipation and abdominal distension and was admitted to the Pediatric Surgical Unit. He had passed meconium within 24 h of birth in the postnatal ward with no signs of abdominal distension; progressively he developed chronic constipation.\nAt the clinical evaluation, his abdomen was found to be tender and distended, although quiet. Abdominal radiography showed a distal obstruction (). Suction rectal biopsies demonstrated nerve hypertrophy. Transanal Soave pullthrough was performed and at the intraoperative evaluation the absence of ganglion cells in the submucosal and myenteric plexus of the rectum and sigmoid colon confirmed the length of HD. The aganglionic colon was resected and the normally innervated bowel was brought down and sutured to the area just above the anal sphincter.\nAt the age of 3 years and 2 months, an infectious enterocolitis (C. Difficile) confirmed by the finding of the specific toxin by microbiological laboratory testing occurred and an antibiotic treatment was administered, with resolution of symptoms.\nOne month later, he presented with marked abdominal distension and melena; occasional episodes of vomiting was also reported. Abdominal obstruction was detected at radiography (). Upper endoscopy and colonoscopy were then performed. Stomach and small bowel mucosal biopsies were normal. Full-thickness surgical distal colon biopsies showed the presence of mature ganglion cells and revealed an eosinophilic-predominant inflammatory infiltrate in the myenteric plexus and surrounding tissues of sigmoid colon and rectum (>60 eosinophils/ 10 HPF, X40) (). An EMG was then diagnosed.\nThe patient had peripheral eosinophilia (8%) and an elevated age-related IgE concentration (423kU/l). A panel of specific IgE tests (immuno-CAP system) was then performed, showing sIgE positive for casein, α-lactalbumin, β-lactoglobulin, egg white, wheat, and maize.\nAvoidance diet for cow's milk proteins, egg, wheat and maize was then prescribed. An elemental formula was introduced as a milk substitute; the symptoms resolution was observed.\nAfter 3 months of dietary modification, colonoscopic mucosal biopsies showed a mild chronic inflammatory cell infiltrate of lymphocytes in rectal tissue, with evidence of histologic remission of intestinal eosinophilic infiltrate (15 HPF in the sigmoid colon, 12 and 10 HPF in the rectum at 10 and 2 cm from anal orifice, respectively) ().\nAt 15 months from the start of the diet, colon biopsies showed a complete resolution of esosinophilic infiltrate and the child's well-being persisted. |
A 50-year-old male patient presented with complaints of gradually progressive loss of vision in his right eye over 3 months. There was no history of fever, headache, vomiting, seizure, loss of consciousness, photophobia, and weakness of body. On examination, the patient was alert, orientated, and afebrile. There was no evidence of peripheral lymphadenopathy. There was a loss of perception of light in the right eye. Rest of the neurological examination did not reveal any abnormality. A contrast-enhanced magnetic resonance imaging (MRI) of the brain revealed an intensely enhancing solid mass in an extradural location in the suprasellar cistern, close to the pituitary stalk. Anteriorly, the mass extended to the right orbital apex []. The possible differential diagnoses were meningioma and RDD. Endocrinological evaluation did not reveal any abnormality. He underwent maximal safe resection (optic nerve decompression) via fronto-temporal craniotomy approach. Intraoperative findings included a soft to firm, moderately vascular tumor, lifting and surrounding the optic nerve more on the right side. It was extending into carotico-optic space partially encasing the internal carotid artery (ICA). Postoperative MRI of the brain suggested complete removal of the lesion, with minimal dural enhancement along the right temporal lobe []. Postoperative histopathological report showed a tumor consisting of variable numbers of histiocytes intermixed with plasma cells, eosinophills and lymphocytes. Histiocytes showed features of emperipolesis. On immunohistochemistry, the histiocytes showed immunopositivity for S-100 protein suggesting a diagnosis of intracranial RDD [].\nHe was symptomatically stable for 1 year. Thereafter, he presented with gradually progressive diminution of hearing in his left ear over 3 months and acute worsening of vision in his left eye over 3 weeks. On examination, visual acuity had decreased to finger counting at 2 feet on the left and there was loss of perception of light in the right eye. Pure tone audiometry revealed complete loss of hearing in his left ear although hearing was intact in his right ear. A repeat MRI of the brain showed an intensely contrast enhancing mass lesion in the right suprasellar cistern, extending into the right cavernous sinus, encasing the intracavernous segment of ICA and abutting the right temporal lobe. There was also contrast enhancement and thickening involving the left vestibulo-cochlear and facial nerves, extending into the left internal auditory canal [Figure –]. He was given a trial of oral corticosteroids, but there was symptomatic progression after 2 weeks. Hence, local radiation was delivered to the suprasellar mass and the lesion involving the left vestibulo-cochlear and facial nerves to a total dose of 20 Gy in 10 fractions over 2 weeks by three-dimensional conformal radiotherapy (3D-CRT). He was evaluated 1 month after completion of radiation. Pure tone audiometry showed an improvement in hearing in his left ear though ophthalmological evaluation did not show any improvement in visual acuity. A brain MRI, done 3 months after completion of radiation therapy showed radiologically stable disease. Twenty months after completion of radiotherapy, he complained of decrease in vision in the left eye and brain MRI revealed a 5 cm × 4.9 cm × 4 cm intensely enhancing mass lesion in suprasellar region involving bilateral cavernous sinuses and ICAs (right > left), compressing optic chiasm and reaching up to left superior orbital fissure without obvious intra-orbital extension. Similarly, enhancing lesions were noted in bilateral tentorium, left cerebello-pontine angle, intracranial segment of left VII and VIII cranial nerve. A 2.6 cm × 1.6 cm enhancing lesion was also noted in the right occipito-parietal lobe along tentorium. The entire dura mater was thickened with nodular enhancements suggestive of diffuse leptomeningeal disease [Figure –]. In view of progressive, diffuse leptomeningeal disease, the patient is being considered for systemic chemotherapy. |
A 27-year-old Pakistani woman presented in outpatient department with complaints of spells of crying, laughing without any reason and aggressive behavior for three years with few period of being symptom-free. Her general physician considering her symptoms referred her to a psychiatrist. The psychiatrist keeping histrionic personality disorder as the top most differential diagnosis gave her some medication (which she didn’t have any record of) and offered some cognitive therapy. The patient's family stopped taking her to the session due to the stigma of psychiatric treatment. Her condition did not improve. Four months before presentation, she started to develop gradual, progressive and painful weakness of right lower limb. The next three months she could not do her daily routine activities and eventually became bedridden. On examination, she had bilaterally positive Babinski sign, increased tone in the lower extremity, exaggerated knee reflex, and diminished ankle reflex, power in lower limb was ⅖ and decreased sensation as well. In upper limbs, she had a normal tone with inverted biceps reflex and absent brachioradialis reflex, sensations were intact in the upper limb. There was decreased sensation on left side of the face suggesting left trigeminal nerve involvement. Taste sensation was lost on the right side of anterior two-third of the tongue indication right facial damage. Rest of the cranial nerves were normal in function. The fundoscopic examination was normal. A provisional diagnosis of inflammatory demyelinating diseases was made. Magnetic resonance imaging (MRI) of brain and spinal cord without gadolinium contrast were done which showed abnormal signal intensity areas involving multiple bilateral subcortical superficial white matter of frontal, parietal and temporal regions and deep white matter of right parietal and left temporal regions. Lesions were hypointense on T1 weight image (T1W1) and hyperintense on T2W1 and fluid-attenuated inversion recovery (FLAIR) sequence. There was no perilesional edema, mass effect or midline shift. MRI of spine did not show any abnormality. Cerebrospinal fluid (CSF) examination showed positive oligoclonal bands, and hence the patient was labeled as Primary progressive multiple sclerosis (Figure ). |
A 64-year-old female with a history of ventilator dependent respiratory failure secondary to end-stage chronic obstructive pulmonary disease (COPD) and bioprosthetic mitral valve replacement 8 years ago was admitted to the medical intensive care unit (MICU) for worsening respiratory failure from pulmonary edema and multifocal pneumonia secondary to Escherichia coli. She was diuresed and completed a course of antibiotics with ceftriaxone. A transesophageal echocardiogram (TEE) was performed during this admission revealing severe prosthetic mitral valve stenosis, mildly elevated pulmonary artery pressures, a normal tricuspid valve, and no vegetation on any valve. She was ultimately transferred to a long term postacute care hospital. Over the following month, the patient continued to decline in clinical status, and further investigation was undertaken. Rapid-growing mycobacterium was isolated from three cultures for mycobacteria from a tracheal aspirate. Interferon Gamma Release Assay (IGRA) and M. tuberculosis complex polymerase chain reaction (PCR) of a tracheal aspirate were negative. Rapid-growing mycobacterium was isolated from multiple blood cultures from a peripherally inserted central catheter (PICC) and from peripheral venipuncture. M. fortuitum was the organism identified in all cultures. Empiric antibiotic therapy was initiated with amikacin, imipenem, and clarithromycin; and the PICC line was removed. The resistance-pattern confirmed that the isolate was sensitive to amikacin and imipenem, but resistant to clarithromycin. The patient was ultimately readmitted to the MICU for hypotension and concerns for overdiuresis and volume depletion. A transthoracic echocardiogram (TTE) was performed (40 days after the previous TTE and 12 days after initiation of antibiotics) revealing a new 10 mm × 11 mm vegetation on the ventricular aspect of the pulmonic valve, severe tricuspid regurgitation, and severe prosthetic valve mitral stenosis (). The patient was not a candidate for pulmonic valve replacement and medical management was continued. Given the grim prognosis, the patient ultimately decided to stop antibiotic therapy and entered hospice where she ultimately passed away. The patient completed 16 days of antibiotics at the time she was discharged to hospice. |
A 45-year-old male patient presented to us with a single value of raised S PSA of 7.16 ng/ml. He did not give any history of LUTS, but complained of nonspecific generalized bony ache. The pain did not have a specific pattern or a characteristic feature. He was being evaluated for this complaint, on the lines of arthritis, at some other medical facility. His blood tests were normal (serum calcium, serum phosphate, serum PTH levels and antibodies negative). His roentgenograms and bone scan tests were unremarkable. He was also advised S PSA levels that turned out to be elevated and he was referred to us for further management.\nHis digital rectal examination was within normal limits with no signs of prostatitis. TRUS showed 37 g of homogenous and unremarkable prostate. We repeated the S PSA test after 4 weeks of the previous value and it showed a progressive pattern (S PSA = 10.47 ng/ml). Therefore, we planned a prostatic biopsy of this patient. A 12-core TRUS-guided prostatic biopsy was performed. The histopathologic examination (HPE) of tissue [] revealed stromal aggregate of lymphoplasmacytic cells, periductal condensation of plasma cells with intraepithelial infiltration and destruction, and dense fibro-collagenous tissue with intense plasma cell infiltration. The features were suggestive of autoimmune prostatitis. On further immunohistochemistry evaluation, plasma cells showed IgG4 positivity.\nSubsequently, serum IgG4 levels were obtained that was found raised (3.18g/L). We also advised an abdomino-pelvic contrast CT scan keeping in mind the systemic nature of the disease and association of IgG4 prostatitis with pancreatitis. However, it showed normal findings with no abnormalities in the pancreas with mild enlargement of prostate []. The patient was counseled regarding the absence of any prostatic malignancy but the need for regular follow up. As no specific urological symptoms were present, no active treatment from urological point of view was advised. But, to evaluate effect on S PSA levels, corticosteroids were started. We observed a steady decline in S PSA levels after starting medication 2 and 4 weeks (4.16 and 3.22 ng/ml, respectively) later. The patient is on regular follow-up till date with normal S PSA levels on corticosteroids. |
A 14-year-old male patient (30 kg weight) with the diagnosis of fourth ventricular ependymoma was posted for suboccipital craniotomy and excision of tumor in the prone position. Preoperative assessment did not reveal any other comorbidities, previous seizures episodes, drug allergies, and normal laboratory values (including renal functions). The patient was conscious, alert, and cooperative. However, examination showed that the patient was emaciated (due to poor nutrition) and had been bed ridden due to the primary disease for 1 month.\nIn the operation theater, after connection of monitors, intravenous access with an 18 gauge cannula was established. To compensate for any hemodynamic disturbances caused following anesthesia induction and prone positioning it was decided to preload the patient with 500 ml of 0.9% sodium chloride over 15 min. Preloading started and about 300 ml of 0.9% normal saline was infused over the next 10 min. When about 200 ml of the fluid was remaining in the fluid bag, 1.5 g injection ceftazidime (preinduction antibiotic prophylaxis as per departmental protocol) was diluted and added in the fluid bag for simultaneous administration before induction of anesthesia. 200 ml of this fluid was rushed within 5 min and just as the infusion completed the patient developed generalized convulsions. A Guedel's airway was immediately inserted inside the patient's oral cavity to prevent tongue bite. The seizures did not subside in the succeeding 20 s and injection midazolam (4 mg) was administered. The seizures abated with the administration of midazolam, and the patient could be ventilated with a face mask. Blood samples were sent promptly for estimation of blood gases, electrolytes, sugar, and serum albumin levels. Vital parameters of the patient were stable and remained so, thereafter. The reports of investigations meanwhile showed slight acidosis (pH - 7.24), hypercarbia (49 mmHg), and hypoalbuminemia (albumin-3 mg/dL). Blood sugar and electrolytes were within normal levels. By the time the laboratory reports (which were not grossly abnormal) had arrived, the patient had regained consciousness with a Glasgow coma scale of 14/15 (eye opening to speech). After mutual discussions with the surgeons, we decided to continue with the surgery after making provisions for elective postoperative ventilation. Injection thiopentone sodium (120 mg) and a nondepolarizing muscle relaxant (injection rocuronium, 30 mg) were now administered and the patient was intubated. Invasive lines (arterial and central venous catheters) were placed for stringent monitoring of hemodynamics and regular intraoperative sampling.\nSubsequently, the patient was turned prone for surgery. The surgical course lasted for 4 h and was uneventful. Postoperatively, the patient was transferred to the Intensive Care Unit (ICU) for overnight elective ventilation in view of the perioperative adverse event (convulsions). He could be safely extubated the following morning. Injection ceftazidime was omitted from the postoperative antibiotic schedule and replaced with Ciprofloxacin. He was vigilantly monitored for recurrence of convulsions, which, however, did not occur. His postoperative stay in ICU (for 2 days) and ward (for 6 days) was unremarkable and after making a satisfactory recovery, he was discharged on the 10th day after surgery. |
A 43-year-old right handed woman presented in 2006 with difficulty writing, impaired dexterity of the right hand, and mild unsteadiness with walking. Her sole initial symptom was difficulty writing with the right hand and with time, other dexterous movements with the right hand became affected. Her left hand was asymptomatic. On examination, she scored 30 on the MMSE. There was a subtle dysarthria. She walked well except for a slight tendency to veer to either side. There was mild bilateral upper extremity dysmetria and mild ataxia with heel-to-shin. With the hands outstretched, as well as when performing movements with the right hand (especially writing), the proximal and distal thumb flexed involuntarily into the palm. When attempting to write, there was flexion of the right thumb, extension of the wrist, and excessive flexion at the PIP joint of all fingers. She did not notice any sensory tricks for dystonia and did not have mirror dystonia. Her reflexes were 2+ in the upper extremities and 3+ in the lower extremities. Brain MRI demonstrated cerebellar and brainstem atrophy.\nHer mother was reported to have a progressive gait disorder, presenting with unsteadiness, hand clumsiness and dysarthria. We later learned that her mother had been diagnosed with SCA2 with 37 CAG repeats. Prior to death, her mother had a resting tremor, nystagmus, and cervical dystonia. Our patient’s maternal grandmother, two of three siblings and several maternal uncles and cousins were all similarly affected; a total of ten individuals were considered symptomatic in a three generation pedigree (Fig. ).\nThe patient was not seen again until eight years later, at age 51. During that time, there had been only mild progression of cerebellar symptoms but the dystonia of the right hand continued to worsen to the extent that she was barely able to write and the dystonia also impaired many other tasks with the right hand. On examination, dysarthria remained mild. There was severe writer’s dystonia (Additional file 1: Video 1) with excessive flexion of the thumb and all digits, poor control of the pen, and extension of the wrist. With the hands outstretched there was flexion of the right thumb into the palm and mild extension of the first finger at the MCP joint. There was moderate dysmetria with finger-to-nose on the right. There was slight dysdiadokokinesia of the left hand and mild dysmetria with finger-to-nose on the left. There was mild gait ataxia. Although the saccades were not overtly slow, when making saccades between horizontal targets, they were almost always performed with a simultaneous blink, which can be sign of subtle slowing or difficulty in initiation []. There was no significant improvement with 600 mg per day of levodopa, the maximally tolerated dose of trihexyphenidyl, as well as botulinum toxin. As such, in 2015, at age 52, she underwent deep brain stimulation (DBS) of the left globus pallidus internus (GPi), resulting in significant improvement in the dystonia. The monopolar Medtronic device achieved optimal settings at: Lead C + 0-, frequency of 165 Hz, voltage of 2.4 V and PW of 450 μs.\nWhen seen 2 years after DBS (Additional file 2: Video 2), there was much less spontaneous flexion of the right thumb into the palm with the arms outstretched. Although not shown on the video, there was marked improvement in her ability to write within the first several months following DBS. Compared to pre-operatively, she performed finger-to-nose better on the right suggesting that dystonia rather than ataxia was the main cause of impairment prior to DBS. The dysmetria of the left upper extremity had progressed but there was still only mild dysdiadokokinesia bilaterally. The gait ataxia had progressed but she was still ambulatory without an assist device and reported no falls. She was unable to tandem walk. |
A 45-year-old Caucasian woman was diagnosed with a variant of MS in 2015, specifically, Balo’s Concentric Sclerosis (BCS). Neuroimaging findings at this time are summarized in . Her past medical history included an important episode of optic neuritis in 2015. Following the diagnosis of BCS and negative John Cunningham virus (JCV) screening, the patient was started on natalizumab 300 mg intravenous (IV) every four weeks. Four weeks after the fourth dose of natalizumab, the patient presented dizziness, vertigo accompanied by dysarthria, weakness on the left side and blurred vision to the right eye. Three days later, the patient was admitted for the exacerbation of neurological symptoms. Magnetic resonance imaging (MRI) revealed multiple diffuse subcortical lesions, left more than right, frontal, temporal and in the basal ganglia. At this moment, the suspected diagnosis was more in favor of exacerbation of MS than progressive multifocal leukoencephalopathy (PML) or lymphoma. Microbiology testing was performed and sent out of our health care centre to be analyzed. In the meantime, the patient received methylprednisolone 1 g IV daily for five days and plasma exchange for suspected exacerbation of MS and PML, respectively. On day 3 of hospitalization, the patient showed further neurological deterioration with severe bradycardia and was admitted to the intensive care unit (ICU). The patient’s neurological manifestation was psychomotor retardation, progressive balance disorder with unstable gait, neck stiffness at the end of flexion and paresis of left limbs. A second MRI showed important radiological deterioration (). After multiple examinations that excluded other possible diagnoses, a brain biopsy performed on day 6 revealed diffuse CNS B cell lymphoma ( and ). The same day, microbiology results came in with JCV in the cerebrospinal fluid still negative and EBV in the blood positive. On day 8, a “debulking” chemotherapy (rituximab 500 mg/m2 and methotrexate 3500 mg/m2) was initiated with a significant improvement in her symptoms over the following days. On day 26, she began the MATRix regimen for four cycles every three weeks (see ) and was discharged on day 33 []. For consolidation, radiotherapy was not an option because of possible neurotoxicity due to her MS. She received high-dose chemotherapy followed by an autologous stem cell transplant (ASCT) (see ) with a good tolerance. Thirty-five months later, the patient is still in remission of her lymphoma. Furthermore, since the ASCT she has also experienced complete remission of her MS, for which she is no longer receiving treatment. |
A 66 year old indo-aryan male presented with complaint of blurring of vision in right eye (RE) over 7 months. He had undergone cataract surgery 6 years back following which he had good vision in both the eyes. Seven months back he noticed sudden diminution of vision in RE for which he didn’t seek any medical attention and received no treatment. He had no past history of ocular trauma, any other ocular surgery in both eyes or any relevant systemic illness. There was no history of high myopia, diabetes mellitus, hypertension and connective tissue disorder in the patient. He smoked occasionally but didn’t consume alcohol. He was carpenter by occupation. He had two brothers who didn’t suffer from any similar eye problem. At presentation, his vitals were stable. On systemic examination, he had normal vesicular breath sounds bilaterally, heart beat was normal with no added sound, abdomen was soft to palpation and neurological examination was normal. On local examination, his both eyes were pseudophakic, and vision of the RE was hand movement and that of the left eye (LE) was 6/6. His RE had a subtotal rhegmatogenous retinal detachment (RD) with PVR-B (The updated Retina Society Classification) [] for which vitrectomy was planned. Routine laboratory investigations like CBC, serology, liver and renal functions were performed and were within normal limits.\nWith a diagnosis of subtotal RD, the eye was being operated using 23 gauge vitrectomy systems. After completing near total vitrectomy, perfluorocarbon liquid (PFCL) injection was started using a double bore cannula. During injection of PFCL liquid to flatten the retina, there was sudden change in visibility of the fundus due to increase in haze of the media. There was associated forward protrusion of the eyeball and contact with the binocular indirect ophthalmomicroscope (BIOM) lens. On removing the BIOM, a bullous RD was visible behind the intraocular lens (IOL) and the globe was hypotonus. It was a difficult situation and the surgeon was unable to comprehend what was going on. The cause of hypotony was being explored when the possibility of some mal-functioning of infusion system came into the mind of the surgeon. To overcome the hypotony, air was switched-on in the infusion cannula. This further deteriorated the situation. Air escaped in the anterior chamber, IOL complex dislocated posteriorly, and 180° inferior retinal dialysis was noticed. For some time, the surgeon could not comprehend as to what was going on. Then, ballooning of the conjunctiva was noticed and a provisional diagnosis of scleral rupture was made. Conjunctival peritomy was made superiorly and scleral defect was noticed at 10 o’clock, 10mm behind the limbus extending 9mm in clockwise direction behind the superior rectus muscle. Intraocular tissue incarceration and air leak was visible from the wound (Figures and ).\nRepairing the ruptured globe with retinal tissue incarceration in a vitrectomised eye during vitrectomy was a challenge. If we switched on air in infusion cannula, the turbulence caused at the site of rupture was far more than when balanced salt solution was switched on. Decreasing the infusion pressure would lead to globe collapse. In any situation, repositioning the incarcerated retina was not possible and retinectomy was performed followed by flattening of detached retina with PFCL. This was followed by repair of scleral defect using 9-0 nylon with lots of difficulty. The superior rectus muscle had to be disinserted and reinserted to facilitate scleral repair. Dislocated IOL was removed, vitrectomy was completed and 360° laser retinopexy was performed. RD surgery was then completed and silicone oil was used for tamponade. On first postoperative day visual acuity was hand movement and cornea was edematous. Retina could be poorly visualized and appeared to be attached under oil clinically. He was given topical prednisolone acetate (1%) drop 2 hourly for a week and then tapered gradually over weeks. He was also given topical moxifloxacin (0.5%) eye drop 6 hourly for 4 weeks and topical atropine (1%) eye drop three times a day for 4 weeks. During follow up visits, the retina remained attached under silicone oil and the best corrected visual acuity was 6/36 at last follow-up of 4 months. |
A 30-year-old man was referred with 3-day history of clustered scaly papules on the right chest wall. The patient had episodes of groups of erythematous papules with shooting pain distributed on his right side of chest alone in the dermatome 9 days ago, which quickly turned into vesicles within 2 days. He was diagnosed as HZ and started on treatment of valaciclovir on the 4th day after the appearance of painful papules, resulting in rapid pain relief after being treated for 2 days. On the 6th day of the course of disease, the patient found that the vesicles began subsiding and became scaly papules rapidly in situ. No new areas were involved except the site of prior HZ lesions. He had no trauma or localized application of medications before the painful papules appeared. The patient had 7-month history of scalp psoriasis showing good response to topical application of calcipotriol ointment, but he used the medicine irregularly resulting in recurrent lesions sometimes. He never had KP lesions, and any other associations, since the onset of psoriasis. His family history was also unremarkable.\nCutaneous examination revealed that scaly erythematous papules and plaques located on the scalp and forehead (Figure A), and groups of clustered erythematous papules with silver scales in the dermatome distributed on the right side of chest wall where the prior HZ lesions occurred (Figure B, C). After removal of the scales on papules, underlying bleeding points were present. No other kinds of lesions were observed elsewhere including limbs, nails, and the trunk except the site where the prior HZ eruptions involved. The patient was diagnosed as KP of psoriasis, and was treated with topical application of clobetasol propionate and calcipotriol compounds, which resulted in excellent response after a week of treatment (Figure D). On 1 year of follow-up, the patient had occasional recurrence of psoriasis when the treatment was stopped; no new zosteriform eruptions reoccurred. Interestingly, the recurrent lesions occurred only on the scalp and the areas of prior shingles each time (Figure E,F). Histology from thoracic lesion showed regular epidermal hyperplasia with test-tube-shaped rete ridges, thinning over some derma papilae, acanthosis, parakeratosis, and lack of granular layer (Figure ). |
This is the case of a 13-year-old black African boy of the Bantu ethnic group, a student, who presented at the pediatric emergency room of Yaounde University Hospital Center with intense chest and vertebral pains, evolving for 48 hours before admission. A week before, in the course of a brawl his left forearm was twisted, resulting in a sharp and permanent pain in his left forearm associated with a functional impotence without any cutaneous lesions. At home, his tutor gave him paracetamol and diclofenac that were administered orally followed by a consultation the next day at a traditional healer. The traditional healer carried out scarifications on our patient’s forearm, consisting of multiple superficial incisions of the skin made by a blade, supplemented by the application on the cutaneous lesions of an ointment composed of herbs, leaves, and earth, which would be likely to contain Clostridium tetani spores. Less than 48 hours later, there was an onset of a generalized pain, predominant in our patient’s back and in his sternal region.\nThe adolescent lives with his aunt in town. There were no elements in favor of a non-accidental injury or child abuse. He had no history of chronic disease; he has never had an operation. The immunization status of the child was unknown to the next of kin.\nOn general examination on his presentation to our emergency department, he was conscious and ill-looking. His temperature ranged between 36.8 and 38.3 °C, his pulse was 88 beats per minute, his pupils were equal and reactive to light stimulus, and his blood pressure was 105/70 mmHg. He presented a trismus, spinal stiffness, a generalized contracture with abdominal rigidity, and opisthotonus. In addition, there were also spasms triggered by noise, light, and touch during care.\nThe loco-regional examination of his left upper limb revealed a balm based on herbs and black earth placed under a traditional splint. After removal of the latter, scarifications were visible with areas of cutaneous necrosis (Fig. ). The rest of the examination was otherwise normal.\nAn X-ray of his left forearm showed a slightly displaced shaft fracture of the two bones of his forearm classified Orthopaedic Trauma Association (OTA)/AO 22-A3 (Fig. ). His hematological and blood electrolytes profiles were within normal limits. No biologic test was performed for the detection of tetanus antitoxin antibodies in whole blood due to the non-availability of this test in our setting at the time.\nThe diagnosis of generalized tetanus complicating a closed fracture of the forearm with subsequent opening was made. Generalized tetanus was thus diagnosed a few hours after our patient’s admission on the basis of the clinical examination in particular, and an obvious point of entry in an individual with unknown vaccination status who clinically presented a trismus, a generalized contracture, as well as paroxysms.\nHe was subsequently admitted 6 hours later to our intensive care unit. He was placed in solitary confinement in a dimly lit room; a nasogastric tube was placed, and sedation and diazepam myorelaxation were performed. The etiological treatment consisted of an antibiotherapy based on metronidazole that was intravenously, directly, and slowly administered and an immunotherapy with equine anti-tetanus serum. Subsequent disinfection of the point of entry with resection of necrotic tissue was performed to reduce microbial growth and release of toxins.\nThe evolution was marked by a gradual deterioration in his state of consciousness, dysautonomic manifestations, particularly blood pressure surges, and tachycardia alternating with bradycardia. Moreover, the occurrence of several episodes of tonic–clonic paroxysms required additional doses of muscle relaxant and intubation. He died a few hours later in our intensive care unit, 12 hours after admission to our hospital. |
A 38 year-old Iranian woman with a triplet (three chorionic and three amniotic) pregnancy was hospitalized at 29 weeks and 2 days gestational age due to one-time high blood pressure at 140/90mmHg and elevated liver enzymes. A week before admission, she had been hospitalized in another hospital for about 5 days to evaluate the cause of increased liver enzymes. She had a 2-year history of primary infertility and had become pregnant by ovulation induction, and also had a 5-year history of hypothyroidism, which was euthyroid during pregnancy. She had also been diagnosed with gestational diabetes a month before admission, which was being treated with 16 units of insulin daily (6 units Levemir and 10 units NovoRapid), so her blood glucose levels were maintained in a normal range, and glycated hemoglobin concentration was 5.6 %. She was at risk for preeclampsia due to her advanced age and triplet pregnancy, so aspirin was administered. On the other hand, due to hospitalization for more than 3 days, she was at increased risk of deep vein thrombosis and was treated with enoxaparin. Additionally, given the possibility of iatrogenic preterm delivery, she had received a course of betamethasone for fetal lung maturation.\nTwo weeks before hospitalization, liver enzymes increased to four times the normal levels: alanine amino transferase (ALT) 218 units per liter (U/L) and aspartate amino transferase (AST) 283 U/L. After a thorough evaluation and ruling out preeclampsia, and due to a probable diagnosis of gestational cholestasis, she was treated with ursodeoxycholic acid (300 mg twice a day) from 1 week before hospitalization. Lab tests at admission included the following: ALT = 94 U/L, AST = 57 U/L, total bilirubin = 0.7, direct bilirubin = 0.1, and lactate dehydrogenase (LDH) = 276 U/L. Other tests including white blood cell count, hemoglobin, platelet, serum creatinine, and urinalysis were in the normal range. She underwent 24-hour Holter blood pressure monitoring, and among all measurements, only 18.8% of systolic and 15.6% of diastolic blood pressure exceeded the set limit of 140 and 90 mmHg, respectively. Echocardiographic findings were quite normal, and 24-hour urine protein was also reported in the normal range. On the second day of hospitalization, an ultrasound exam was performed, which showed that biophysical scores and amniotic fluid were normal in all three fetuses. One of the fetuses had increased umbilical artery resistance (pulsatility index [PI] > 95%) and estimated weight below 5%, but umbilical cord and middle cerebral artery findings were normal in the other two fetuses. On the fourth day of hospitalization, the patient developed fever and cough. The following day, due to the persistent fever and cough and also an onset of myalgia, real-time reverse transcriptase polymerase chain reaction (RT-PCR) was conducted on nasopharyngeal swabs for SARS-CoV-2 nucleic acid. All steps including sample collection, processing and laboratory testing were based on World Health Organization (WHO) guidelines. Chest X-ray and computed tomography scan were not performed due to the patient’s lack of consent.\nThe result of RT-PCR was positive for the SARS-CoV-2 virus. However, the patient’s clinical symptoms were mild. She had a mild fever, with maximum temperature of 38.3 °C. She had no complaint of shortness of breath, diarrhea, nausea or vomiting, her respiratory rate was 18–20 per minute, and oxygen saturation was above 95% at all times. On the same day that she developed clinical symptoms, an ultrasound exam was performed: the fetus who already had increased umbilical artery resistance showed an exacerbated condition involving absent umbilical artery end-diastolic flow, and another fetus showed umbilical artery resistance (PI > 95%), but umbilical cord and middle cerebral artery findings were normal in the third fetus, and biophysical scores and amniotic fluid were normal in all three fetuses. Based on these findings, the patient underwent serial ultrasound exams, and unfortunately, exacerbated umbilical flow resistance in the second fetus also progressed to absent umbilical artery end-diastolic flow (Fig. ). However, Doppler and biophysical scores were normal in all fetuses. Finally, 6 days after the beginning of clinical symptoms, the biophysical scores declined in the two fetuses with absent umbilical artery end-diastolic flow, so the patient underwent cesarean section due to rapid deterioration of fetal conditions and exacerbated placental insufficiency. The first baby was born weighing 1320 g with umbilical cord pH 7.25, and her 5-minute APGAR [appearance, pulse, grimace, activity, respiration] score was 4; the second baby was born weighing 1600 g with umbilical cord pH 7.23, and his 5-minute APGAR score was 7; the third baby was born weighing 1250 g with umbilical cord pH 7.21, and her 5-minute APGAR score was 6. In order to protect the babies from infection with the virus, delayed cord clamping was not performed, skin-to-skin contact of mother and babies was not practiced, and the babies were separated from the mother immediately after birth. All three babies were intubated and were admitted to the neonatal intensive care unit (NICU), where they were kept in separate, isolated rooms. RT-PCR of nasopharyngeal swabs for SARS-CoV-2 nucleic acid was carried out for all three newborns immediately after birth. The mother was discharged 3 days after cesarean section, and 2 weeks later she had recovered completely without any complications.\nTwo of the babies, weighing 1250 and 1320 g, each received three doses and the other baby received two doses of surfactant. All three newborns developed clinical symptoms of sepsis and pulmonary hemorrhage. The primary results of the COVID-19 test were negative for all three newborns. Because of the poor general conditions of the newborns, and considering the false-negative probability of the initial test, the COVID-19 test was repeated immediately after receiving the first test results, and the result was positive for the baby who weighed 1600 g and also had better umbilical cord and placental circulation before birth. Unfortunately, we did not examine the umbilical cord blood and amniotic fluid samples for the virus, so we cannot conclusively link the COVID-19 RT-PCR positive test in this fetus to vertical transmission, but because the babies were completely isolated and had no suspected exposure during the period between the two tests, the possibility of vertical transmission cannot be ignored.\nThe baby who weighed 1320 g died 3 days after birth with collapsed white lung and sepsis. The baby who weighed 1250 g also had symptoms of sepsis and died 13 days after birth. The baby who weighed 1600 g and had a positive COVID-19 test eventually recovered and was discharged in good general condition 3 weeks after birth. |
A 39-year-old male who was diagnosed with primary cholesterol hepatolithiasis attended our out-patient department for a routine medical check-up. Further inquiry revealed a history of extracorporeal shock wave lithotripsy (ESWL) due to left ureteral calculus one year ago; no other aberrations were noted and physical examinations were normal. Liver function tests demonstrated a normal level of liver transaminases, including aspartate aminotransferase, alanine aminotransferase and γ-glutamyl transpeptidase. No clear elevation of tumor markers including α-fetoprotein (AFP), carcinoembryonic antigen (CEA), CA125 and CA-19-9 was observed. Hepatitis virus markers were all negative. Ultrasound examination showed multiple cholesterol stones in the intrahepatic bile ducts of the left lobe and hepatic steatosis. Further examinations were performed after admission of the patient to hospital. Abdominal computed tomography (CT) scans revealed several round high-density shadows within the intrahepatic biliary tract of the left lobe, which was noticeably dilated (Fig. and ). A low-density mass with an estimated magnitude of 2.0 × 1.8 cm and a vague margin was also observed in the CT scan. The mass was located in the right hepatic lobe and showed signal enhancement around the wall in arterial phase, gradual enhancement toward the central area of the lesion in the portal venous and delayed phases (Fig. and ). No other abnormalities were noted. Magnetic resonance cholangiopaneretography (MRCP) demonstrated multiple T2 low signal intensity foci in the left intrahepatic bile duct concomitant with cholangiectases. At the request of the patient and to rule out the possibility of cancer, a transabdominal radical excision was performed based on the clinical diagnosis of hepatolithiasis and the left hepatic lobe was successfully removed. Postoperative recovery was uneventful.\nGross examination showed a few yellowish-white biliary calculi within the left hepatic lobe and distinct strictures of several biliary tracts. The dilated bile ducts had a maximum circumference of 1.2 cm. Histological examination of the resected specimen showed pancreatic tissues distributed along the wall of the bile duct and composed of acinar cells and duct elements without islets of Langerhans (Fig. ). The acinar cells contained eosinophilic granules (Fig. ). The pathological diagnosis was intrahepatic cholangiectases with hepatolithiasis concomitant with pancreatic heterotopia. |
A 39-year-old woman was referred to our clinic for persistent elevated β-hCG after surgical evacuation of a blighted ovum. She complained of amenorrhea combined with intermittent vaginal spotting and lower abdominal discomfort. She had an obstetric history of 2 full-term vaginal deliveries and one induced abortion. Fourteen weeks earlier, she had undergone surgical curettage for a blighted ovum at 7 weeks of gestation at a local clinic. Physical examination was normal except mild low abdominal tenderness, and vital signs were normal. Her serum β-hCG was 95 mIU/mL. A review of her previous sonography, performed before curettage, revealed a gestational sac with a yolk sac in the endometrial cavity, a diagnosis compatible with a blighted ovum. We telephoned the obstetrician who had performed the curettage and confirmed that the previous procedure was uneventful with no suspicious uterine perforation. Transvaginal sonography revealed an empty uterus and a 2-cm mixed echoic mass in the uterine fundus (). Color Doppler evaluation demonstrated abundant arterial and venous flow on the mass (). Magnetic resonance imaging (MRI) showed a 2.3×1.3 cm mass lesion of heterogeneous signal intensity with many tortuous tubular signal voids in the uterine fundus. The endometrial-myometrial junction was intact (); the mass was heterogeneously well enhanced after contrast material infusion. Also, multiple small cysts in left ovary were noted with MRI.\nAfter conservative care including analgesics for 2 weeks, her β-hCG level had slightly decreased to 73 mIU/mL but her abdominal discomfort was persistent, and the mass on ultrasonography had increased to 2.7×2.0 cm. At this time, the possible differential diagnosis included placenta site trophoblastic tumor (PSTT), heterotopic pregnancy, and remnant placenta. Diagnostic laparoscopy was planned in order to acquire samples for histology. We suggested resection of the mass, but the patient opted for total hysterectomy due to fear of malignancy and further pregnancy.\nLaparoscopy revealed a 3-cm brown and bluish mass on the fundus and multiple small bluish round cystic implants of 5–10 mm on the left ovary and left broad ligament (). We performed a laparoscopic total hysterectomy and excised all cystic implants. Histologic examination of the bluish masses showed normal trophoblastic tissue with degenerated and necrotic chorionic villi in the hemorrhagic background. This mass was located within left fundus without disruption of endometrial-myometrial junction. The postoperative course was uneventful, and β-hCG was <5 mIU/mL at 13 days after surgery. |
A 37-year-old multiparous female patient presented to our outpatient clinic with complaints of right breast swelling and stiffness. A physical examination of the patient revealed a well-defined firm mass in the upper outer quadrant of the right breast, approximately 10 x 5 cm in size, extending beneath the areola. An axillary examination of the patient revealed no lymphadenopathy. Breast ultrasonography showed a lobulated, contoured, well-defined, large, hypoechoic multiple solid lesions of heterogeneous pattern in contact with each other in the right breast parenchyma; the largest had a diameter of 7-8 cm and a breast Doppler examination showed no marked vascularization on the mass (Figure ).\nA unilateral mammography was requested. Right breast mammography revealed smooth, contoured, multiple nodular opacities close to the skin without any ductal structure extending to the skin, the largest with a diameter of 6-7 cm (Figure ) and a bilateral breast magnetic resonance imaging (MRI) was requested.\nThe breast MRI revealed several, smooth, contoured lesions close to each other, almost completely filling the right breast with equivocal signal feature changes like breast structures, which might partly be consistent with debris and with a complicated appearance; the largest had a size of 7.7 x 4.1 cm. After an intravenous (IV) contrast injection, peripheral contrast enhancement in the cyst wall was observed without marked mural nodular or solid field enhancement at the cyst level. (Figures -).\nA Tru-cut biopsy was performed on the mass. In the pathological examination, benign breast tissue was observed, including tissue fragments lined with keratinized squamous epithelium, keratin materials, and two ductal structures as a separate fragment. Available histomorphological findings were considered consistent with epidermal inclusion cysts. Then, total tumor excision with negative surgical margins and an intraglandular flap reconstruction was performed on May 17, 2017. A post-operative pathological examination yielded epidermal cysts. In the pathological examination, macroscopically, there was a 10-cm-diameter mass with well-defined borders and multiple lobulations (Figure ); microscopic evaluation reported an epidermal cyst (Figures -). |
An 83-year-old woman, with a height of 1 meter and 65 cm and a weight of 85 kg (BMI = 31.22), was transferred to our department because of a reverse pertrochanteric-subtrochanteric fracture AO 31-A3 (). The patient had a cardiovascular disease of moderate severity, though her social life was very active, and the Harris Hip Score (HHS) [] and the Short Form 12 Health Survey (SF-12) [] were both 94 points (). The anesthetic risk was ASA 3 [], and she did not require intensive care after surgery. After reduction, internal fixation was done using a short Affixus® nail (Zimmer Biomet™, Warsaw, Indiana, USA) which is 180 mm long. The shaft was 9 mm wide, the lag screws were 100 mm long, and one distal static locking screw was used (). In the 1st postoperative day, rehabilitation began, and by the 2nd day, she was walking with total progressive weight-bearing. She was discharged on the 7th postoperative day. Six months after surgery, at the last control, the HHS was 66 and the SF-12 was 74, and the radiographs showed subtrochanteric nonunion and medial displacement of the distal fragment (). Thereafter, the patient seek medical assistance in another hospital. Six months later, she underwent radiographic studies which showed incomplete breakage of the nail at the hole for the locking screw (), though no surgical treatment was indicated. There was no pain in the hip, and 2 years following primary surgery, radiographs done in the other hospital showed further incomplete nail breakage at the hole for the lag screw (). No further treatment was planned, and later on, the patient reported mild pain while flexing the hip. One year later, i.e., three years after surgery, the patient seek further assistance because of the sudden severe hip pain, and the radiographs showed complete fracture of the nail at both the proximal and the distal holes (). The HHS was 26 points and the SF-12 was 35 (see ). She underwent revision surgery, with removal of the broken nail () and total hip arthroplasty with a 46 mmØ Plasmacup® acetabular cup with a 28 mmØ bearing liner in polyethylene (Aesculap, B. Braun, Melsugen, Assia, Germany) fixed with two screws of 24 and 32 mm length, a 28 mmØ Prevision® metal femoral head, and a 240 mm long modular revision stem (Aesculap, B. Braun, Melsugen, Assia, Germany), with proximal segment P1/0 mm and distal segment 12 mmØ, with the addition of three free metal cable cerclages (). Twelve months after the revision surgery, the HHS was 80 points and the SF-12 was 90 points (Figures and ). |
A 20-year-old female, with bilateral multiple conjunctival granulomatous lesions and conjunctival injection, presented with tearing, photophobia, severe itching, foreign body sensation, and redness. Her problem was present for the past 9 months, and she underwent conjunctival biopsy sampling twice followed by topical corticosteroids with no response. The patient was given a questionnaire for the symptoms of itching, redness, and foreign body sensation with a grading scale of 0 or none, 1 or mild, 2 or moderate, and 3 or severe to report the severity of each individual symptom. In addition, she underwent a complete ophthalmic examination. Two weeks' before starting the study, all other medications including ophthalmic corticosteroids were discontinued. The informed consent was obtained before the start of the study.\nBest-corrected visual acuity was 20/20 in both eyes. Systemic and laboratory assessments were all normal, except a moderate eosinophilia. Two biopsy samples were seen by two separate ophthalmologists and pathologists and infectious causes were ruled out by PAS and Ziehl–Neelsen staining. Epithelioid histiocytes, Langerhans giant cells, lymphocytes, a diffuse infiltration of eosinophils, moderate plasma cells, and few neutrophils in subepithelial stroma were found in the specimen.\nA diagnosis of an allergic conjunctival granuloma was confirmed based on above and another finding known as Splendore–Hoeppli phenomenon [Fig. and ]. Tacrolimus eye drop was reconstituted by adding balanced salt solution to tacrolimus vial for injection under sterile condition in the local laboratory to achieve a 0.005% concentration.[] To evaluate the effectiveness of the drug, the patient was treated by application of tacrolimus eye drop (0.005%) in her right eye every 6 h while the left eye was put on vehicle control placebo (ophthalmic solution free of tacrolimus). The patient was followed up 3 days after starting the medication and every week in the 1st month and then every 2 weeks in the next 5 months. The signs and symptoms started to reduce dramatically and lesions disappeared in her right eye [Figs. -] in 3 weeks while no improvements were observed in the left eye. After 1 month, the placebo was switched to tacrolimus eye drop, and in further follow-ups, a similar course was obtained in the left eye. The score of symptoms was reduced from 9 before treatment to 3 after the treatment in both eyes. |
A 17-year-old woman with hypertrophic cardiomyopathy (HCM) was transferred to our hospital after successful resuscitation from ventricular fibrillation (VF). After the patient was diagnosed with HCM based on the echocardiogram and cardiac biopsy 2 years prior, she had been free from any symptoms or arrhythmic events while taking a beta-blocking drug and ACE inhibitor. Both her father and grandfather had HCM, and her father died suddenly at 53 years old. Although she did not complain of anything, her BNP level was constantly high (500–970 pg/ml), so cibenzoline of 200 mg was added 3 months prior for the purpose of improvement of diastolic function caused by attenuation of the LV contraction and prevention of arrhythmic events. Then, she collapsed while walking due to VF.\nShe arrived at our hospital while undergoing therapeutic hypothermia. Her height was 159 cm and weight 56 kg. Her blood pressure was 158/89 mmHg. Physical examination revealed coarse crackles in her whole chest. Blood tests revealed normal renal function and electrolytes. The plasma cibenzoline concentration was not checked.\nAn ECG showed ectopic atrial rhythm with heart rate of 63 bpm, complete right bundle branch block, and left ventricular hypertrophy with ST-T changes with QRS width of 148 ms and QTc of 0.514 (Figure ). Those findings were almost like those before the episode. The serial ECGs showed that QRS width gradually prolonged over 2 years from 110 to 147 ms, suggesting that the conduction disturbance from the His–Purkinje system through the ventricular myocardium had been progressing (Figure ). The ECG findings including QRS width did not change after commencement of the administration of cibenzoline.\nAfter she recovered almost fully without severe neurologic sequelae, a subcutaneous implantable cardioverter–defibrillator (S-ICD) was selected for second prevention as she was young and pacing therapy was thought as unnecessary. The surface ECG screening prior to the S-ICD implantation was performed. On the screening, the location of the surface electrodes was the fifth intercostal space along the left mid-axillary line (left leg, LL), 1 cm lateral to the left sternal border and 1 cm above the xiphoid process (left arm, LA), and 14 cm cranial to electrode LA on the left parasternal line (right arm, RA). A ground electrode was placed on the right lower extremity. The alternate vector was from RA to LA electrode, primary vector from LA to LL electrode, and secondary vector from RA to LL electrode. All 3 vectors satisfied the S-ICD screening template in both supine and upright positions; therefore, she was thought to be eligible for S-ICD. Then, she underwent treadmill exercise testing for further screening for the S-ICD with the 3 screening leads of the S-ICD. During exercise, QRS duration increased progressively from 160 ms at rest at a heart rate of 66 bpm to 200 ms at peak exercise at a heart rate of 100 bpm, accompanied by monomorphic ventricular tachycardia (VT) at 150 bpm. QRS width during the VT extended further, resulting in polymorphic VT (Figures and ). After she had collapsed, the 3rd DC shock finally succeeded in terminating the VT. This VT was thought to be a proarrhythmia of cibenzoline, and therefore, cibenzoline was changed to sotalol. Re-treadmill exercise testing while receiving sotalol revealed no change in QRS width during exercise, nor did it induce any VT at a similar exercise burden. Her S-ICD was set with the primary vector as the sensing configuration, and she was well without any ICD shocks while taking sotalol. |
A 68-year-old female patient presented with complaints of low vision and central vision loss in her right eye for the past month. Her best corrected visual acuity was hand motion in both eyes. Intraocular pressure was 19 mmHg in the right eye and 17 mmHg in the left eye. Slit-lamp examination revealed bilateral nuclear sclerosis. On fundus examination, bilateral posterior staphyloma with myopic degenerative changes were observed, as well as a shallow RD associated with the posterior staphyloma in the right eye. Examination by optical coherence tomography (OCT) showed RD associated with the full-thickness macular hole in the center of the posterior staphyloma of the right eye (). Anterior-posterior axis length was 33.65 mm. B-mode ultrasonography showed significant posterior bulging of the sclera ().\nSurgical repair was done by dissecting the conjunctiva and Tenon’s capsule in an approximately 150-160 degree area of the superotemporal region of the right eye, and bridle sutures were passed through the superior and lateral rectus. In the superotemporal region, 5/0 nylon sutures were placed in the sclera approximately 20 mm from the limbal zone where the implant would be fixed between the insertion points of the superior and inferior oblique muscles. Following phacoemulsification and intraocular lens implantation in the posterior chamber, triamcinolone acetonide (TA)-assisted PPV and ILM peeling were performed. Before securing the explant (AJL Ophthalmic) to the superotemporal region, a fiber-optic light attached to the explant was used to check where the explant contacted the posterior pole by transillumination (). Laser photocoagulation was applied to the hole and degenerative areas in the peripheral retina, followed by fluid-gas exchange using C3F8.\nThe patient was recommended to lie in prone position for 3 days postoperatively. Fundus examination and B-mode ultrasonography performed at postoperative 2 months revealed a bulge in the macular area associated with the local explant (). At postoperative 3 months, the patient’s visual acuity was counting fingers from 1 meter. Fundus examination showed that the macular hole had closed and the retina was attached. These findings were confirmed with OCT (). |
A 24-year-old office worker male presented to outpatient orthopedics department of our hospital with nonresolution of his symptoms despite a previous surgical intervention. His complaints dated 6 months back when he sustained trivial trauma following which he was unable to bear weight on his right lower extremity. There was no history of fever or constitutional symptoms. An X-ray right hip and pelvis were performed which revealed a subtrochanteric fracture of the right femur with displaced fracture fragments []. There was no evidence of osteoporosis, lytic/blastic lesion suggestive of bone tumor. Subsequently, he underwent open reduction with intramedullary nail fixation for fracture stabilization.\nAfter 6 months postsurgery, he presented to our outpatient with complaints of persistent local pain and swelling. He was unable to bear weight on the right lower limb. Fresh radiographs revealed lysis around the implant with resorption of the fracture ends []. Notably, there was no evidence of callus formation. Computed tomography scan or magnetic resonance imaging could not be performed due to financial constraints. Suspecting persistent low-grade chronic pyogenic infection, he was planned for surgical debridement with external fixation. Intraoperatively, the muscles and soft tissues were found to be indurated. Pearly white structures and granulation tissue were identified at lesional site. A through betadine wash was performed, excised tissue sent for histopathology, and external fixator applied.\nThe excised specimen consisted of multiple fragments including pearly white-flattened structure measuring 2.8 cm × 2 cm × 1 cm. Microscopy revealed fragments of thick eosinophilic acellular lamellated membrane corresponding to parasite exocyst []. Degenerating bits of thin endocyst/inner nucleated germinal layer were seen occasionally. High magnification showed characteristic refractile dagger-shaped hooklets []. Hence, diagnosis of hydatid disease of the right femur was rendered. Ensuing ultrasound abdomen and chest did not reveal liver or lung involvement. The patient was started on 10 mg/kg albendazole into two divided doses with fat rich meals, to be continued for 2 years. Since the diagnosis was established postoperatively, wait and watch policy was decided; further surgical intervention to be done only if indicated clinically. |
A 65-year-old male with history of non-insulin-dependent diabetes mellitus (HbA1c of 6.5%) and schizoaffective disorder presented with multiple new skin lesions starting in July 2016. The patient had underlying eczema and he complained that his lesions were worsening over the prior three weeks, starting on his right knee, with subsequent spread to his left knee and bilateral upper extremities. The lesions appeared different from his baseline eczema and did not itch. The patient's primary care physician noticed this rash on the entire circumference of his upper extremities and extensor surface of his thighs bilaterally (). The lesions were described as purple or dark red plaques and papules with a discrete circular pattern, without underlying erythema. In addition, some of the papules had minimal pustular drainage. There were fewer lesions in sun exposed areas; the patient was started on triamcinolone cream for presumed guttate psoriasis. However, he showed no improvement at follow-up three weeks later.\nThe patient reported no sick contacts or international travel. The only travel he reported was to Montana in April of 2016 and New Mexico in 2015, but he did not have any acute illnesses while traveling or subsequently. The patient lives with his significant other and does not have any pets at home. He denied any known insect bites.\nThe differential diagnosis included atypical erythema migrans, viral exanthem, tinea, or potentially Majocchi's granuloma (MG). A punch biopsy of the left medial forearm and left thigh lesion demonstrated a deep fungal infection and dense suppurative and granulomatous inflammation in the dermis centered around a follicle (), with budding yeast and hyphal forms within the follicle (), which was consistent with Majocchi's granuloma. PAS stain noted budding yeast forms, and Gram, AFB, and Fite stains were unrevealing. A deep fungal culture grew a very light growth of Malbranchea species, identified by morphology, with no evidence of histoplasmosis or blastomycosis. The patient was evaluated for immunosuppressive conditions and tested negative for HIV, hepatitis B, and hepatitis C. Complete blood count and metabolic panel were normal.\nThe patient was prescribed itraconazole but could not obtain it secondary to financial reasons. Therefore, he was started on oral terbinafine for Majocchi's granuloma, and lesions started to resolve. Subsequent fungal blood cultures were negative. |
A 54-year-old female presented to our colorectal practice after evaluation by her gynecologist with symptoms of abdominal cramps, mucus and bloody vaginal discharge, and recurrent urinary tract infections (UTI). Her only significant surgical history was an IUD removal in 2011. The patient had no significant family history and had not had a colonoscopy. Physical exam revealed the abdomen to be non-tender to palpation with an easily reducible umbilical hernia. She had no rebound tenderness, guarding or palpable masses.\nA transvaginal ultrasound, obtained by the patient’s gynecologist, showed a linear hyperechoic structure within the fundal portion of the endometrial canal. The suspicion was that of a retained intrauterine device. In addition, there was a right-sided avascular complex cyst measuring 2.5 × 2.2 × 1.8 cm3. A CT scan of the pelvis was then obtained. The CT scan, highlighted in Figs and , revealed multiple foci of gas within the left ovary and fistulization of the adjacent sigmoid colon with questionable air in the fallopian tube.\nA colonoscopy was scheduled to exclude a malignancy in the colon and to rule out other colonic pathology. The colonoscopy showed a mild degree of inflammation in the sigmoid colon with diverticular disease and a small amount of purulent exudate. No polyps or tumors were seen.\nTwo weeks later the patient was taken to the operating room for a hand-assisted laparoscopic colon resection and left oophorectomy. Intraoperatively, there were adhesions between the sigmoid colon and left anterolateral pelvic sidewall. Figure shows the inflamed left ovary and fallopian tube adjacent to the sigmoid colon. The left ovary and a small portion of the left fallopian tube were dissected, and then resected en bloc with the sigmoid colon. Figure depicts the uterus status post-resection. An end-to-end anastomosis was performed to the inraperitoneal rectum. The patient had an uncomplicated hospital course and was discharged home on post-operative Day number 2. One month later, the patient was doing well with normal bowel function, resolution of her UTI’s and resolved abdominal pain.\nPathologic exam revealed a resected portion of rectosigmoid that contained a diverticulum that had fistulized into the attached ovarian tissue, seen in Fig. . An adherent portion of fallopian tube was also present. The fistula tract was lined by granulation tissue with acute and chronic inflammatory cells and focal foreign body giant cells as seen in Fig. . The fistula extended into the ovarian stroma, causing a focal tuboovarian abscess. No other diverticula were present. |
A 10-month-old girl presented to us with a tuft of hair over the lower back since birth. She was moving her lower limbs less as compared to her upper limbs. Bladder and bowel functions were not impaired. Examination revealed tuft of hair in midline in the lumbar region. She also had scoliosis with curvature towards the left side. Her head size was normal for her age. Noncontrast Computed tomography spine revealed bony spur at the level of L3 vertebral body [] and it was directed superiorly []. Magnetic resonance imaging scan was done which showed spinal dysraphism in the lumbosacral region with split cord malformation type 1 with two hemicords separated by a spur [Figure and ]. Cord was ending at L3 [].\nD 12 to L 4 laminotomy was done. Intraoperatively split cord was extending downwards from L 1. There was bony septum arising from posterior surface of L 3 vertebral body and it was ascending up to L 2 level. There was a single dural sac and the bony septum was indenting the dural sac from anterior aspect, not dividing the posterior dura into two sheaths (contrary to what is seen usually in Type I split). The conus was split into two halves by the septum and there was a single filum. The septum was seen at the lower part of split only, making it type I C split (as per the classification given by Mahapatra and Gupta).[] The septum was excised and both the dural envelopes were opened and sutured into one sheath. The filum was thickened and detethering was also done. The patient developed weakness in both her lower limbs in the post-operative period. She was given methylprednisolone intravenous for 48 h and at the time of discharge (post-operative day 7), she had recovered power in both lower limbs up to the preoperative level. |
A previously healthy 21-year-old Caucasian female presented to the emergency department in a hypotensive and febrile state with associated weakness and vomiting. She reported having a sore throat and fever three days prior, for which she went to urgent care and was prescribed azithromycin for bilateral tonsillitis. Over the course of three days, the azithromycin did not relieve her symptoms, and instead, they progressed to also include nausea and vomiting. On the third day, she described a sensation of feeling “something burst” in her neck, which improved her sore throat but lead to the presence of blood in her sputum, prompting her visit to the emergency department.\nOn initial assessment, she had a fever of 104oF, a blood pressure of 79/39 mmHg, a heart rate of 134 bpm, and a swollen and tender right neck. Her laboratory evaluation showed leukopenia and thrombocytopenia, and a contrast CT scan of the neck revealed enlarged bilateral palatine tonsils and a filling defect in the right internal jugular vein (Figures -). The combination of her physical exam, laboratory, and image findings pointed to the diagnosis of Lemierre’s syndrome with progression to sepsis. She was promptly admitted to the critical care unit and started on vancomycin and piperacillin/tazobactam.\nOn the second day of admission, her fever persisted and she developed acute hypoxic respiratory failure. A chest CT with contrast was performed and showed bilateral pleural effusion, patchy consolidations, and nodular opacities suggestive of lung abscess (Figure ). She was then started on furosemide and IV meropenem to treat her pleural effusions and abscesses, respectively. Her fever, sore throat, and inflammation resolved in response to the antibiotics, however, her thrombus in the right internal jugular vein persisted. She was in a continual state of hypercoagulability, so the decision was made to have her Nexplanon® etonogestrel implant removed. Over the next two days, her condition started to improve with a decrease in her C-reactive protein (CRP) and an increase in her platelet count and blood pressure. At this point her throat pain, fever, and vomiting had resolved, but her right neck and ear were tender to palpation and she complained of mild chest tightness.\nUnfortunately, her condition declined once again the following day, with recurrent fever and worsening neck pain. Repeat CT showed progression of the right internal jugular vein thrombosis into a fully occluded vessel, with wall thickening and hyperemia indicating thrombophlebitis (Figure ). The thrombophlebitis extended inferiorly to the infrahyoid muscle and supraclavicular fat, and her temperature continued to increase from 100.7oF to 101.1oF. While the thrombophlebitis was extensive, there were no signs of neck abscess or acute odontogenic infection. At this point, heparin was started as her thrombocytopenia had improved since intake.\nIn an effort to prevent septic embolism, vascular surgery was consulted and a surgical ligation was planned. However, during the ligation procedure, the patient was found to have severe inflammation and purulent material within the right internal jugular vein, so drainage and a suppurative thrombectomy were performed instead of the initially planned ligation. The thrombus and purulence from the internal jugular vein were retrieved and sent for culture and pathology, which showed signs of extensive acute inflammation but no organism growth. The thrombectomy was successful, and over the next five days, the patient’s condition greatly improved. She was discharged home with clopidogrel, apixaban, IV meropenem, and vancomycin, which were found to be the optimal antibiotic choices based on sensitivity testing. |
A 31-year-old woman with no previous history presented with persistent severe headache and repeated vomiting at a local clinic. MRI of the head revealed a pineal tumor with obstructive hydrocephalus. She was eventually transferred to our hospital for further treatment. She underwent an urgent ventricular-peritoneal shunt. After the ventricular-peritoneal shunt, she improved dramatically. Subsequently, gadolinium enhanced-MRI of the brain showed heterogeneous enhancement in the pineal region without pituitary tumor and leptomeningeal and ventricular dissemination (). Blood examination showed normal serum levels of α-fetoprotein, β human chronic gonadotrophin and placental alkaline phosphatase. Cerebral angiography revealed a vascular rich tumor fed by the posterior choroidal artery and arterial-venous shunting. In order to confirm the pathological diagnosis and remove the tumor, she underwent subtotal resection of the pineal tumor with craniotomy using the occipital transtentorial approach. In the intraoperative findings, the tumor was soft and bled easily. It was easy to dissect the tumor from the splenium. We concluded that the tumor originated in the pineal region, not in the splenium or midbrain. Finally, we performed subtotal resection because it was very difficult to stop the bleeding in the left superior and lateral parts of the tumor. She exhibited no new neurological deficits after the surgical resection.\nHistopathological findings showed the feature of glioblastoma composed of poorly differentiated pleomorphic tumor cells with marked nuclear atypia and brisk mitotic activity (). Focal necrosis with pseudopalisading was found, and MIB-1 proliferation index was high (43.7%). Immunohistochemistry showed a positive reaction to the glial fibrillary acidic protein, S-100, nestin, and INI-1. On the other hand, it showed a negative reaction to synaptophysin and NFP-MH. After surgical resection, she was treated by radiotherapy with 60 Gy and daily administration temozolomide (75 mg/m2) for forty-two days. Two months later, postoperative MRI showed no change of the residual tumor but leptomeningeal dissemination surrounding the brain stem and upper cervical spinal cord (). She continued with temozolomide chemotherapy. Three months after surgery, she experienced severe lumbago and bilateral leg pain. Thoracico-lumbar MRI showed drop metastasis (). She and her family refused any additional treatment, and finally she died five months after the initial diagnosis. |
A 55-year-old male presented with insidious onset diplopia while looking to the right and to distant objects of 3 months duration. There was no history of visual blurring, jaw deviation, facial asymmetry, hearing loss or tinnitus. There was no associated headache, vomiting, seizures or loss of consciousness, limb weakness, gait, and bowel or bladder disturbances. On examination, patient was conscious with stable vital signs. Higher mental functions were normal. There was right side sixth nerve palsy and nystagmus on looking to the right. Visual acuity and visual field examination were normal. There were no other cranial nerve, motor or sensory deficits. Other system examinations were within normal limits. Magnetic resonance imaging of the brain showed an expansile mass lesion involving entire clivus that was T1- and T2-isointense and showed contrast enhancement. There was erosion of posterior wall of the sphenoid sinus with lesion bulging into the sinus. Pituitary was normal []. With a preoperative diagnosis of clival chordoma infiltrating into the sphenoid sinus, the lesion was decompressed by subfrontal trans basal approach.\nMicroscopy showed respiratory epithelium with an underlying neoplasm composed of sheets of plasma cells with a moderate amount of cytoplasm and eccentric round nuclei []. Cells were positive for CD 138, CD 38 and CD 56, while negative for CD 20, cytokeratin and synaptophysin. Ki 67 (MIB) labeling index was < 5%. Cells were kappa light chain positive and negative for lambda light chains thus establishing monoclonality. A diagnosis of plasmacytoma of clivus was given, and workup for myeloma was suggested.\nPatient underwent whole-body X-ray studies, serum and urine electrophoresis, complete blood counts, serum calcium levels and a bone marrow biopsy, as part of myeloma investigations, which were negative.\nPatient received radiotherapy (50 gray given in 25 fractions) and is now 8 months postoperative and doing well. The right side lateral rectus palsy is still persisting. |
A 45-year-old woman presented to her gynecologist with 6 months of increasing abdominal cramping and marked distention despite a 35-pound intentional weight loss. She denied other systemic or gastrointestinal symptoms. Her medical history was notable for in vitro fertilization resulting in twin pregnancy 7 years prior. She was otherwise healthy with a body mass index of 27 and not taking any medications. Physical examination was remarkable for a non-tender, but firm and distended abdomen with palpable findings suspicious for a large mass. Lower extremity edema was not identified in either leg suggesting that the mass was not compressing the iliac veins and likely superior to the pelvis. All laboratory testing, including serum hCG, was within normal limits.\nComputed tomography (CT) scan of the abdomen and pelvis revealed a large heterogeneous, hypodense mass measuring 27 cm x 20 cm x 13 cm with irregular, central solid-enhancing components (Fig. a, b). Based on cross-sectional imaging, the mass appeared distinct from the uterus, without associated ascites or lymphadenopathy. An ultrasound-guided core biopsy demonstrated an admixture of mature adipocytes and spindle cells. Immunohistochemical staining showed diffusely positive for smooth muscle actin, desmin, and estrogen receptor, but was negative for HMB-45 (Fig. c–g). Taken together with the imaging findings, the histopathological characteristics confirmed a diagnosis of lipoleiomyoma.\nGiven the symptomatic nature of the mass, the patient underwent exploratory laparotomy to resect the tumor. Upon entering the peritoneum, the mass was immediately identified and encompassed most of the abdominal cavity. The tumor appeared to originate below the inferior border of the stomach with attachments to the mesentery and omentum and displaced the transverse colon into the pelvis. The tumor was well vascularized with feeding arteries from the mesentery and a large draining vessel to the superior mesenteric vein. Grossly, the tumor appeared well-circumscribed and was removed intact without disruption of the capsule (Fig. h). Cut section of the tumor showed a fibrofatty appearance (Fig. i). The final pathology confirmed a 30 cm x 20.5 cm x 12.5 cm lipoleiomyoma. Flow cytometric analysis of the tumor revealed a small population of T cell infiltrate (CD45+, CD3+), with a greater proportion of both CD8+ and CD4+ infiltrates compared to normal fat from the same patient (Fig. a). However, these populations are smaller than what has been shown in aggressive soft tissue sarcomas, which is consistent with the benign nature of lipoleiomyoma. Culturing of the dissociated tumor revealed a heterogenous population of cells with characteristics of fibroblast, smooth muscle cell, and endothelial cells (Fig. b). Lipoleiomyoma cells demonstrated increased proliferation compared to the adipose tissue, which is consistent with the rapid proliferation of the tumor compared to normal adipose tissue (Fig. c). Overall, the patient’s post-operative course was uncomplicated, and she was discharged on post-operation day 3. At her 3-week follow-up visit, she was feeling well, and her surgical incision was healing appropriately. |
A 40 year old lady from Kosovo, was referred to our hospital for severe pulmonary arterial hypertension and a mass in right atrium suspected for vegetation. About one month before, she was admitted in another hospital and received iv medication. The patient was febrile and the C-reactive protein, white cell count and erythrocyte sedimentation rate were elevated. Blood cultures demonstrated a methacilin sensitive Staphylococcus aureus growth.\nTransthoracic echocardiograhy demonstrated a mobile, irregularly shaped, oscillating and highly mobile mass, located above the tricuspid valve septal leaflet (Fig. ). A clear jet across a small defect between left ventricle and right atrium consistent with Gerbode type defect was identified. The direction of the Doppler signal also leads to the true diagnosis (Fig. ). Cardiac magnetic resonance demonstrated a supravalvular flow associated with infravalvular jet according to the type C acquired Gerbode defect (Fig. and ). A normal lung scan excluded pulmonary embolism. The tricuspid regurgitation was considered mild- to- moderate with estimated pulmonary arterial systolic pressure about 60-80 mmHg.\nThe patients underwent surgery after 2 weeks of antiobiotic therapy. Through a right atriotomy, large vegetation was attached to the septal leaflet and anterior leaflet of tricuspid valve was identified. On removal of the vegetation, a defect was found communicating between the left ventricle and right atrium (Fig. and ). This defect represented an acquired Gerbode defect and was closed by two 5/0 pledgeted prolene sutures (Fig. ). Then the septal leaflet of tricuspid valve was resected and was replaced with a trimmed autologous pericardial patch. Anteriorly the newly created septal leaflet was attached to the anterior leaflet. Then, two synthetic chorda were employed (Fig. ). The hydraulic maneuver demonstrated trivial tricuspid valve regurgitation (Fig. ). Then the right atrium was closed. After an uneventfully post-operative period, the patient was discharged home in good clinical condition. Echocardiogram demonstrated trivial tricuspid valve regurgitation and no residual shunt. One year later the patient was doing well. The transthoracic echocardiography at follow-up demonstrated a moderate tricuspid valve regurgitation and no residual shunt. |
A 53-year-old man was admitted to the local hospital with left upper abdominal pain lasting 2 weeks. A gastroscopy showed a bulging gastric lesion in the antrum. However, the pathology was benign. The patient did not receive any treatment, and still had abdominal pain. Then, he was referred to the Second Affiliated Hospital of Xi’an Jiaotong University. Physical examination revealed no mucocutaneous pigmentation, no palpable masses in the abdomen, but left upper abdominal tenderness. The patient had no family history of polyps or tumors in the gastrointestinal tract. Laboratory tests were normal, and the tumor markers were within normal limits. Abdominal computed tomography revealed that the gastric wall in the antrum was thickened (Fig. ). A gastroscopy showed a giant and extensive bulging gastric lesion located on the greater curvature and posterior and anterior walls of the gastric antrum, involving three-quarters of the gastric wall. The mucosal surface of the lesion was smooth without erosion or ulcer. Magnifying endoscopy with narrow-band imaging showed the almost normal microvascular morphology of the lesion (Fig. ). Endoscopic ultrasonography was performed, showing a low-echo area in the muscularis mucosae and no invasion into the submucosal layer, without enlarged celiac lymph nodes (Fig. ). An endoscopic mucosal resection was performed, and a bigger specimen was sent for histopathological examination to confirm the diagnosis. The final pathology report indicated a hamartomatous polyp (Fig. ). After excluding PJS, a solitary Peutz–Jeghers-type hamartomatous polyp was diagnosed. Then, the patient underwent an ESD. The resected specimen was measured in 110 × 80 × 4 mm3 (Fig. ). We treated patients with acid suppression. The patient recovered quickly, without any complications. A gastroscopy on the fourth day after endoscopic surgery showed a large postoperative artificial ulcer (Fig. ). A gastroscopy on the 40th day after endoscopic surgery showed a narrow lumen of the stomach caused by ulcer healing. Also, a polyp of 4 × 4 mm2 was observed in the lesser curvature of the gastric body (Fig. ). The patient was followed up regularly. |
A 50-year-old female was referred by an otorhinologist with a large chin ulcer and toothache. The medical history was unremarkable, having never had a medical consultation due to the poor socioeconomic situation. There was a 20-year history of gradually enlarging black papule on her chin. The patient complained of unpleasant facial appearance and a dull pain in the chin region. On extra oral examination, there was a large ulcer (10×6.5 cm) with a firm and indurated margins on the chin extending to the mandibular vestibule and necrosis was evident in surrounding bone (). On palpation, drainage of pus was evident from the ulcer. The mandibular right lateral incisor and canines were mobile. Central incisors were prior to admission due to extreme mobility. Radiographic examination revealed an ill-defined radiolucency of the mandibular symphyses (). Cytological examination did not show any evidence of spirochete infection. Although the lesion had an atypical appearance, the slow course, in addition to clinical characteristics such an enrolled boarders, destructive ulcer, and concurrent involvement of skin and bone led the diagnosis of BCC as the first possibility. Incisional biopsy was performed at the margin of the ulcer. Other differential diagnosis included necrotizing ulcerative gingivostomatitis with facial involvement (noma), osteomyelitis, leishmaniasis, Wegener granulomatosis, deep fungal infection, and amelanotic melanoma. The histopathologic sample consisted of uniform ovoid, and dark staining basaloid cells with moderate sized nuclei and relatively little cytoplasm. The cells were arranged into well-demarcated islands and strands, which appeared to be raised from the basal cell layer of the overlying epidermis and invaded into the underlying connective tissue (). The histopathologic evaluation of the resected bone showed secondary osteomyelitis due to the extensive ulcer. The clinical diagnosis of BCC was confirmed. Surgical resection was planned for the lesion. During the surgery, the involved muscle and mandibular bone were resected and reconstructed with a total mandibular reconstruction plate (). The soft tissue defect was reconstructed with deltopectoral flap. The patient refused second stage plastic surgery, however, the final outcome was an acceptable reconstruction, but the lips remained incompetent (). The patient was followed-up for 3 years and no recurrence was observed. A written consent was obtained from the patient for medical case report. |
A previously healthy 9-year-old girl presented with a 1-year history of slowly progressive hoarseness and dysphagia. She had no significant history of trauma. The patient denied any neck pain or headache upon presentation to our hospital. Neurological examination revealed left glossopharyngeal, vagus, and hypopharyngeal nerve palsy. Her laboratory data were normal, showing no abnormalities of calcium metabolism or hormones.\nImaging findings revealed a large mass that occupied the clivus. Computed tomography (CT) revealed a calcification of mass lesion with osteolytic changes in the clivus [-]. The lesion extended from the condyle to the sella turcica. Bilateral condyles were invaded by the lesion and had slightly collapsed. The margin of the lateral side was the medial side of the bilateral internal carotid arteries. The mass also involved the left jugular foramen and hypoglossal canal. Magnetic resonance imaging showed that the lesion had relatively low intensity in both T1- and T2-weighted images and was heterogeneously enhanced by gadolinium [-]. A heavy T2-weighted image revealed that the left glossopharyngeal nerve, vagus nerve, and hypoglossal nerve were encased by the mass. A whole-body CT scan and positron emission tomography revealed no other lesions.\nAn endoscopic endonasal transclival approach was performed to confirm the pathological finding and to decompress the left lower cranial nerves. Intraoperatively, the lesion was hemorrhagic and white-yellow in color, and consisted of relatively soft tissue []. Some hard calcifications were present in the lesion as well. The lateral side of the lesion was resected until the C5 portion of the medial side of the internal carotid artery was exposed []. The upper side of the lesion was resected until normal dura and bone were exposed []. On the left inferior lateral side, the left hypoglossal canal was opened []. The lesion around the condyle was not resected to preserve occipito-cervical stability. Transient bilateral abducens nerve palsy newly appeared and disappeared within 3 months. No other complications such as cerebrospinal fluid leak or meningitis occurred. Postoperative imaging findings showed that the lesion was resected between the dorsum sellae and lower clivus without the condyle joint []. Over the last 6 months without other treatment, the residual lesion has not regrown. The deformation of the craniovertebral junction has not changed compared to preoperative findings.\nThe pathological findings demonstrated an area of calcification with abundant spindle-shaped fibroblasts and multinucleated giant cells. Some focal hemorrhage was present that was surrounded by osteoclast giant cells and inflammatory cell infiltration including neutrophils. No atypical mitotic figures and no mononucleated tumor cells were observed. In addition, immunohistochemical staining revealed that both H3.3G34W and K36M were negative. The final diagnosis was a GCRG []. |
A 9-year-old apparently healthy boy presented with a gradually increasing painful swelling of 4 months' duration over his lower back. The pain was localized to the lower back, severe in intensity, and piercing in nature; it did not radiate and increased with movements. It was associated with urinary incontinence for the same duration. There was no history of trauma. There was no history of any significant medical or surgical illness in the child or his family.\nLocal examination of the site revealed a small tender swelling in the lumbosacral region, with no signs of inflammation, no visible scar / sinus, and no abnormal pulsations. The swelling was fixed to the underlying structures and the overlying skin was freely mobile. The child was anemic, with hemoglobin of 10.2 gm%; the ESR was 62 mm in the first hour (Westergren's method). The other routine investigations were within normal limits.\nRadiographs of the pelvis and lumbosacral spine revealed posterior scalloping of the sacral vertebrae and widened ill-defined neural foramina; there was an overlying right-sided soft tissue haze. MRI of the lumbosacral spine revealed a lobulated soft tissue mass with both intraspinal and extraspinal components in the sacral region (S2–S4). It was isointense to pelvic muscles on T1W and hyperintense, with areas of necrosis, on T2W images [ and ]; there was intense enhancement on postcontrast scans []. It had caused widening of the spinal canal and the sacral foramina and showed clubbed finger-like projections along the path of the sacral spinal nerves. There was presacral extension and infiltration of the pyriformis and right erector spinae muscles bilaterally. Based on the MRI findings, we made the diagnosis of a complex nerve sheath tumor / soft tissue sarcoma of the sacral nerve roots.\nAn open biopsy of the lesion revealed a proliferative growth. Microscopically, there were monomorphic-appearing small round cells arranged in sheets, with dispersed congested vessels. The cytoplasmic borders were indistinct, producing a syncytial appearance. Mitoses were seen. The nuclei appeared to be round to cleaved. These features are suggestive of Ewing's sarcoma / PNET []. Based on the clinicopathological and radiological findings, we diagnosed Ewing's sarcoma / PNET of the sacral nerve plexus.\nThe patient was started on chemotherapy (VAIA regimen: vincristine, adriamycin, ifosfamide, and actinomycin D), which was followed by radiotherapy to the tumor bed. Radionuclide scan of the whole body (Tc-99m-MDP) after six cycles of chemotherapy revealed no evidence of metastases. The patient responded well to the chemotherapy and radiotherapy and became asymptomatic. Repeat scans showed almost complete resolution of the mass. |
A 21-year-old male presented with complaints of swelling in the neck and fever of 7 months duration. On evaluation, he was diagnosed with Hodgkin's Disease III B. He was treated with seven cycles of combination chemotherapy (total dose for all 7 cycles = Adriamycin 350 mg/m2, bleomycin 140 mg/m2, vinblastine 84 mg/m2, and dacarbazine 5250 mg/m2 ABVD) from February 2007 to August 2007. After the second cycle of chemotherapy, he developed dysuria. A burning sensation in the urethra was persistent for some time after micturition. There was no hematuria or turbid urine. His urine was sterile and the symptoms subsided after symptomatic treatment. He did not have any evidence of urological disease before the start of treatment. He had similar episodes though milder after subsequent cycles. In August 2007, after seven cycles, he presented with dyspnea and cough. A computed tomography (CT) scan of the chest showed features suggestive of bleomycin-induced pulmonary toxicity. Pulmonary function tests revealed obstructive airway disease. He was treated with antibiotics and corticosteroids. A positron emission computerised tomography (PET - CT) after seven cycles of chemotherapy was negative for any residual disease. In March 2008, he presented with burning micturition and a poor stream of urine. The stream was narrow and he had to strain. A urine culture was sterile. An ascending urethrogram showed a stricture of 1–1.5 cm in length at the level of membranous urethra extending to prostatic urethra []. A cystoscopy performed with a No. 17 Fr rigid cystoscope (Karl Storz, Germany) revealed a normal anterior urethra and a stricture starting in the membranous urethra. The cystoscope could not be passed beyond. An ultrasonography revealed post void residue of 80 ml. The patient did not have a history of previous trauma, catheterization, prior instrumentation, or any infective urethritis. A urine cytology was negative. A biopsy from stricture was suggestive of focal edema, myxoid changes, focal mild infiltrates of plasma cells, lymphocytes and eosinophils, and fibrosis without any atypical cells. An optical internal urethrotomy was done in April 2008. Foley's catheter was removed after 10 days. Presently, the patient is able to void freely with an adequate stream of urine. |
A 38-year-old man presented to his primary care physician with complaints of dry cough, left-sided pleuritic chest pain and left-sided back pain of 2 weeks duration. He was found by chest radiography to have an infiltrate in the left lower lobe and treated for community-acquired pneumonia with moxifloxacin for 10 days. After completing the course of antibiotics, he presented again with worsening symptoms of left-sided chest pain and attesting to a weight loss of approximately 5 lbs over the past month. A repeat chest radiograph showed worsening of the left lower lobe infiltrate and an enlarging effusion. A CT scan of the chest showed a large effusion on the left with some loculation inferomedially (fig. ). His laboratory analysis revealed a decrease in hemoglobin from 14.0 to 10.4 g/dl within the past 10 days as well as a leukocyte count of 10.6 × 109/l. Laboratory work-up also demonstrated normal platelets and a normal metabolic profile. With a high suspicion for hemorrhagic effusion, he underwent video-assisted thoracoscopic surgery which revealed a large 12.5 × 8.0 cm gelatinous mediastinal mass with bleeding into the pleural cavity (fig. ). The mass was noted to be compressing the posterior aspect of the left atrium. The origin of the mass was unclear. The patient had a negative work-up for tumor markers including alpha-fetoprotein, CA-125, CA 15-3, CA 19-9 and carcinoembryonic antigen. A biopsy of the mass revealed myxoid stroma with pleomorphic cells and atypical nuclei with high mitotic activity most consistent with myxofibrosarcoma (fig. ). Pleural fluid cytology was negative for malignancy and the patient was staged as T2bN0M0. He was evaluated for surgical resection of the mass and was advised preoperative radiation therapy. Unfortunately, the mass continued to progress even while he was receiving radiation therapy. He was then started on chemotherapy with a regimen of doxorubicin, ifosfamide and mesna. His clinical course was complicated by multiple episodes of neutropenic fevers, pancytopenia requiring transfusions as well as pericardial and pleural effusions. In addition to this, in subsequent imaging it was found that the mass was infiltrating the pericardium. Follow-up scans showed progression of his cancer and the development of liver metastases. His condition deteriorated over a period of a few months with worsening respiratory symptoms due to progression of the mass in size. |
A 40-year-old Caucasian male presented with a 6-week history of chronic unremitting frontal headaches, followed by left eyelid swelling, blurry vision, and diplopia. He subsequently developed nausea and vomiting. He denied any fever, nasal congestion, night sweats, or weight loss. His past medical history was significant for bipolar disorder with no other relevant findings. The patient was not using any current medications and he denied a history of immunosuppresive drugs. Moreover, his family history was only positive for hypertension, without any mention of autoimmune disease. On examination, the patient was afebrile, pulse was 69 per minute, respirations were 23 per minute, and blood pressure was 150/89 mmHg.\nThe physical exam was notable for exophthalmos of the left eye as well as a palpable mass on the left eyelid. Neurological exam revealed third, fourth, and sixth cranial nerve palsy in the left eye. The patient also displayed decreased visual acuity in the affected eye with associated early disc edema. The remainder of the physical exam was unremarkable. The patient’s complete and differential blood counts, serum chemistry and erythrocyte sedimentation rate were normal. Given the findings, the patient was referred for computed tomography (CT) to rule out a mass lesion.\nCT of the orbit with contrast revealed two frontal lobe lesions, the largest of which appeared solid measuring 3.6 × 3.8 cm on coronal image. The lesion was seen eroding through the orbital roof, exerting significant mass effect on the superior rectus muscle, and causing deformity of the left globe. An additional ring-enhancing left frontal lesion was seen adjacent to the solid tumor measuring 2.7 × 1.9 × 2.7 cm.\nSubsequent magnetic resonance imaging (MRI) of the brain with and without contrast revealed a mass arising in the left orbital roof with destruction of the sphenoid bone. Posterior to the mass was a round hyperintense cystic area within the left inferior frontal cortex with surrounding severe vasogenic edema. Mass effect was visualized with midline shift from left to right of the septum pellucidum and subfalcine herniation (Fig. ). The contrast enhanced study showed an extensive lytic mass measuring at least 3.4 × 2.2 cm in size. At the time, the etiology of the lesions was felt to be more likely neoplastic rather than infectious in the absence of any known immunocompromised state. Therefore, blood cultures were not obtained.\nThe patient consequently underwent a left frontal craniotomy for partial resection of the masses. Upon incision the large left frontal lobe lesion was full of purulent material from which multiple cultures were acquired. At this point, the differential favored intracranial abscess rather than neoplasm. Post-operative MRI noted that the previous ring-enhancing lesions were decreased in size. Additionally, the left retro-orbital hyperdense mass had resolved. The prior midline displacement was improved. Persistent vasogenic edema throughout the left frontal lobe was noted; however, the mass effect had resolved. Preliminary pathology from abscess resection revealed granulomatous inflammation and necrosis with positive acid-fast bacilli (AFB) cultures. Intraoperative bacterial, anaerobic and fungal cultures however were all negative. Routine blood cultures, AFB blood cultures and QuantiFERON® TB Gold test were also negative. The patient’s sputum culture however was positive for AFB. Additionally, the patient was tested for HIV following abscess resection with negative results. Moreover, the patient’s CD4 count was 515 cells/mm3 (32 %). The patient was started on an anti-TB regimen consisting of Rifampin, Isoniazid, Pyrazinamide, and Ethambutol.\nCT of the thorax was performed to rule out a concomitant pulmonary infection. The study demonstrated calcified bilateral hilar lymph nodes, as well as multiple calcified mediastinal lymph nodes involving the prevascular, pretracheal, precarinal, right paratracheal, and subcarinal space, reflecting prior granulomatous disease or sarcoidosis. Results from endobronchial biopsy revealed noncaseating granulomas. Final tissue cultures from the brain lesions and sputum revealed MAC. The patient was started on a 12-month MAC treatment regimen consisting of Clarithromycin, Ethambutol, and Rifampin. The patient’s neurological symptoms, exopthalmous, diplopia and mass effect fully resolved over the following 6-months. The blurry vision persisted, but was corrected with glasses. Sputum AFB was negative at 7-months. Furthermore, follow-up MRI at 8-months was negative for intracranial abscess. |
A 37-year-old man with a history of mitral valve prolapse presented to the emergency department (ED) with a two-day history of fevers (up to 105° Fahrenheit (F) at home) and twelve episodes of grossly bloody diarrhea, which began approximately two hours after eating take-out Chinese food. The patient also reported dizziness and lightheadedness upon standing, likely secondary to dehydration from significant quantities of diarrhea.\nIn the ED, the patient was febrile with a temperature of 103.5°F, tachycardic with a heart rate of 114 beats per minute, and orthostatic, but was otherwise hemodynamically stable. Laboratory studies were unremarkable. In addition to aggressive intravenous (IV) hydration, the patient was started on IV ciprofloxacin and metronidazole for the empiric treatment of bacterial gastroenteritis. Although blood and urine cultures were negative, the patient's stool culture found moderate Shigella sonnei. The patient's diarrhea gradually resolved following initiation of IV antibiotics and he was subsequently discharged on oral ciprofloxacin to complete a seven-day course of therapy.\nOn admission, a routine electrocardiogram () incidentally demonstrated an incomplete right bundle branch block along with 1.5 mm ST segment elevations with descent into inverted T waves in leads V1 and V2. On hospital day 1, a repeat ECG demonstrated similar findings. A subsequent ECG on hospital day 2 (), when the patient was afebrile, demonstrated resolution of the previously seen electrocardiographic findings and restoration of a regular sinus bradycardia. Detailed questioning of the patient (and his mother) revealed that there was no reported family history of sudden cardiac death or known arrhythmias. The patient's father did have coronary artery disease and had undergone percutaneous coronary intervention at the age of 67. Otherwise, neither the patient nor his family members had ever heard of the term “Brugada” and were not aware of any other significant family history of cardiac disease. The patient himself had reportedly had an episode where he “passed out” approximately eight years prior to presentation and had a reportedly normal electrocardiogram and an echocardiogram which demonstrated mitral valve prolapse but was otherwise unremarkable. He stated that his doctor at the time told him that he was likely dehydrated. The patient otherwise denied any chronic chest pain, shortness-of-breath, palpitations, or any limitation of his exercise tolerance. Given the lack of cardiovascular symptoms during the current episode and resolution of previously seen ECG findings after defervescence, the patient was discharged. He followed up with an electrophysiologist following discharge and had a repeat ECG which was unremarkable. He opted not to pursue any further testing but will follow regularly with his outpatient physician for followup of his abnormal ECG. |
A 64-year-old male, with a history of poorly controlled Type II diabetes mellitus (HbA1c = 16), presented with right arm weakness, visual disturbance, expressive aphasia, and confusion. Magnetic resonance imaging (MRI) of the brain revealed multifocal brain abscesses (right frontal lobe, right occipital lobe, and left parietal lobe) []. The patient was started on IV vancomycin, ceftriaxone, and metronidazole on the day of admission. The right occipital abscess was needle-tapped the next day, which grew Streptococcus intermedius; unfortunately, antibiotic susceptibilities could not be extracted due to inadequate growth. Despite treatment with systemic, broad-spectrum antibiotics for more than a month, the patient deteriorated into a comatose state, requiring intubation for airway protection. A repeat MRI of the brain showed IVROBA and associated ventriculitis []. An external ventricular drain was placed []. The patient underwent further needle drainage of the right frontal and left parietal abscesses. Daily CSF Gram-stain remained positive for Gram-positive cocci despite prolonged treatment with IV antibiotics. Due to continual clinical decline, intraventricular vancomycin therapy was initiated. The patient was given 10–20 mg of intraventricular vancomycin daily, through the external ventricular drain; dosages were adjusted to achieve the CSF vancomycin therapeutic trough level of 20–25 mg/L.[] A week later, IV rifampin was added to the antibiotic regimen and ceftriaxone was switched to cefotaxime 2 g every 4 h. The CSF Gram-stain turned negative after 11 days of intraventricular therapy, and 5 days after the CSF, vancomycin level became therapeutic. On the same day, the patient gradually became more alert. The patient received intraventricular vancomycin for a total of 30 days. He was discharged on 2 months after his initial presentation. At discharge, the patient could follow simple commands, exhibit a steady gait, and demonstrate full strength in all extremities; although he still had expressive aphasia, his speech was improving. More recently, the patient was employed at a rehabilitation facility. |
A 58-year-old male was diagnosed with left maxillary sinus squamous cell carcinoma and underwent a surgical operation in September 2013. He complained of nasal obstruction, facial swelling and intermittent epistaxis three months later. Dark red neoplasm located in the patient left nasal cavity was observed. CT scan showed the invasion of multiple structures including anterior left frontal sinus, ethmoid sinus, maxillary sinus, nasal septum, pterygopalatine fossa and hard palate. The patient received tumor radical excision and confirmed to be squamous cell carcinoma by pathological examination.\nAfter the surgery, the patient received radiotherapy of 70 Gy in fractions of 2 Gy. However, one year and five months after post-operative treatment, the patient presented with complaints of intermittent epistaxis and decreased vision of the left eye for two months. Examination showed that he had loss of vision and a tumor was located on the top wall of the left residual hard palate. The patient agreed to receive extended resection of the left maxilla. After that, he received radiotherapy of 50 Gy in fractions of 1.3Gy and 5 courses of chemotherapy, including paclitaxel (IV, once every three weeks, 240mg at a time) (135 mg/m2) and nedaplatin (IV, once every three weeks, 140mg at a time) (80 mg/m2). The complication of leukopenia was observed and it was recovered after using recombinant human granulocyte colony-stimulating factor. Oral ulcer healed, but hair losing because of early chemotherapy. Nausea and vomiting were disappeared after injection of antipathectic.\nThree years after the first surgery, the patient came back and complained of hearing loss and otorrhea for 10 months. White neoplasm located at the patient left EAC was observed. CT scans showed the invasion of multiple structures including EAC, tympanic cavity, mastoid and occipital. MRI distinctly showed that there was a lesion occupying the left temporal bone nearby the left middle fossa of skull (size:27.5mm x 38.0mm x 40.5mm, T4) (Figure ). The tumor tissues were confirmed as TBSCC by using H&E staining and Immunohistochemistry (Figure ).\nThen, after radical resection of the tumor for 3 months, it recurred and invaded the left cerebella hemisphere and temporal lobe (size:40.5mm x 52.3mm x 63.6mm, T4) by MRI scan (Figure ). That made removing the recurrent tumor more difficult and risky.\nGiven the failure of tumor resection, we decided to explore the options for targeted therapy for this patient. WES was employed to detect the target gene mutations from patient tumor/normal tissue pairs on the Illumina NextSeq500 sequencing platform and using a TruSeq Rapid Capture Exome Kit for library construction. The WES data was then analyzed using OncoDecoderTM (Genomic Future, Inc.). The significantly mutated cancer-related genes that were identified in the tumor tissue compared to the adjacent normal control included CTNNB1 and VEGFR-2 (Table ).\nSpecifically, we found a missense mutation p.Met12Leu in exon3 of CTNNB1 and missense mutation p.Gln472His(exon11) in VEGFR-2. To detect the gene expression level of VEGFR-2 and its ligand vascular endothelial growth factor(VEGF), quantitative real time PCR on mRNA extracted from both tumor and normal tissues was carried out, and results showed significant overexpression of both VEGF and VEGFR-2 genes in the tumor tissue compared to the normal tissue (Figure ).\nBased on these findings, we carefully screened currently available targeted drugs that may potentially be used for treatment of head and neck cancers. We determined a treatment regimen of bevacizumab and pemetrexed. The ethics committee of the Affiliated Zhongshan Hospital of Dalian University approved the study (The Institutional Review Board approval number is 2016102). The patient received concurrent Bevacizumab (IV, one time per two weeks, 300mg each time for 2 weeks) treatment and pemetrexed (IV, one time a day, 890mg per 21 days for 4 weeks) treatment. The patient was found in slightly red erythra with itch, and these symptoms resolved spontaneously within 48 hours. Fifteen days later, MRI revealed that the lesion occupying the left temporal lobe space had significantly shrunk by 95% to a small hypointense area (size13.2mm x 20.5mm x 27.0mm, T4) (Figure ). Visual Analogue Scale scores of headaches were from 10/10 to 2/10. On targeted therapy Day 35, the patient received extended resection of the temporal bone and reconstruction. Two months later, the patient remains free of new symptoms. Long-term follow-up care has been established for the patient. |
A 31-year-old, gravida 1, para 1 Caucasian woman with a positive pregnancy urine test underwent a routine transvaginal ultrasound (TVUS) at 4 weeks plus 5 days of amenorrhea in which a gestational sac was not definitely identified. Her general physical examination and past medical history were unremarkable. The obstetric history revealed that her previous birth was performed via the cesarean route with 2 layers closure 1 year ago with no further complications noticed after the operation. The patient was clinically stable and signs and symptoms positively correlating to an ectopic pregnancy were absent (Table ). She received oral instructions to have a new ultrasound taken after 7 weeks of amenorrhea. The β-human chorionic gonadotropin (β-HCG) levels were not counted at this initial visit.\nAt 6 weeks plus 1 days of amenorrhea, she reported vaginal bleeding and underwent a new TVUS and 3-dimensional ultrasound that showed a suspected SCP with a viable fetus (positive fetal heart rate). Ovaries appeared normal and the pouch of Douglas had no fluid (Fig. ). The CSP diagnosis was then made using previously established diagnostic criteria: previous history of cesarean section, secondary amenorrhea with irregular vaginal bleeding and increased β-HCG levels, empty uterine cavity and regular endometrial lining, empty cervical canal, and diminished myometrial layer between the bladder and gestation sac.\nAfter being informed about the different treatment options as well as the possible benefits and risks of each method, the patient decided to stop the pregnancy via the medical route using the proposed novel combination of multidose MTX and mifepristone. A written informed consent was obtained from the patient. Baseline serum β-HCG was checked at 12,072.13 mUI/mL (Table ) and tests for liver, renal function, and a complete blood count were performed.\nMedical treatment consisted of 1 mg/kg MTX, 75 mg total, intramuscular, on days 0, 7, 14 and mifepristone, pos, on day 7. Serum β-HCG was measured every other day, until the titers declined to 50%, and then weekly, until normal levels were reached (Table ). Also, tests for liver, renal function, and a complete blood count were checked every 3 days. At 8 weeks plus 1 day of gestation, β-HCG decreased to 8923.37 mUI/mL and a TVUS confirmed absence of a fetal heartbeat (Fig. ). Moreover, resolution of the gestational sac was monitored with a weekly TVUS. At 20 weeks, no visible gestational signs were noticed (Fig. ). |
A 27-year-old white South African male presented at a periodontal practice complaining of painful "gums" particularly in the anterior part of the mouth, and halitosis. His general health was satisfactory; he was a non-smoker and other than grinding his teeth was not aware of any etiological factors, such as mouth breathing, that could have contributed to his problems.\nAfter examination a diagnosis of necrotizing ulcerative gingivitis (NUG) was made, based on the classical punched out papillae, combined with the patient's symptoms of pain and halitosis.\nThe clinical picture was, however, more complex. In addition to the classical punched out papillae seen with NUG, there was inflammatory gingival hyperplasia, and marked occlusal wear (probably due to his grinding habit), and short clinical crowns, all of which, in the presence of plaque could contribute to the gingival inflammation (Figure ).\nAn intra-oral radiograph (Figure ) showed that the incisor teeth were not fully erupted. While the short clinical crowns could be due to occlusal wear or delayed tooth eruption, another possibility was that altered passive eruption (APE) was present, which is a predisposing factor to NUG []. Metronidazole 200 mg tablets three times a day were prescribed at the first visit and combined with palliative treatment, consisting of scaling, home care instruction and a mouthwash.\nOne week later, there was some resolution of the gingival inflammation (Figure ). Possible gingival and periodontal surgical correction of the apparent gingival overgrowth was discussed with the patient who chose not to have this treatment. Thereafter he was seen intermittently; three years after the initial consultation he presented with synthetic crowns on the maxillary incisors, placed there by another practitioner. The associated gingivae were red, shiny with rolled margins and marked inflammation (Figure ). Clinical examination also showed the presence of APE which we have defined as "when a tooth has reached the occlusal plane and the gingival margin in the mid-line of the tooth is at the junction between the cervical and middle third of the clinical crown; or in the middle third or coronal third of the clinical crown in the absence of inflammation, hypertrophy or hyperplasia of the gingiva" []. A periodontal probe showed clinical crown length (Figure ) and an intra-oral radiograph (Figure ) confirmed, that the crowns were not placed at or adjacent to the cemento-enamel junction (CEJ), but markedly coronal to it, as one would expect if APE is not recognized.\nThe synthetic crown margins were just apical to the gingival margin, a long way from the CEJ, on the convex facial surface of the clinical crowns, in a position conducive to trauma from "food impaction" and the accumulation of plaque, contributing to chronic inflammation []. |
A 72-year-old left-handed man with past medical history of atrial fibrillation, congestive heart failure and mitral valve repair, but no history of malignancy, presented to the Emergency Department in 2016 for evaluation of right arm pain. The patient heard a crack in his arm while dressing and subsequently his arm pain worsened. His pain, when he was evaluated at the Emergency Department, was subjectively rated as 5/10, worse with activity and palpation and relieved with rest. No edema or erythema was noted. There was no axillary or cervical adenopathy. His pulses were normal and his sensation to light touch was intact. Radiographs obtained in the Emergency Department revealed a minimally angulated proximal right humeral fracture at the superior aspect of a linear sclerotic lesion in the proximal humeral diaphysis. The linear sclerotic lesion was thought to be likely a bone infarct (Figure and Figure ). No definite soft tissue lesion was noted. His fracture was treated conservatively with splinting.\nHowever, his pain progressively worsened, so repeat radiographs were obtained a couple of weeks later to assess healing of the fracture at his fracture follow-up clinic visit (Figure ). These subsequent radiographs demonstrated the development of a lytic lesion with surrounding periosteal reaction at the fracture site. No osteoid production/mineralization was appreciated. Magnetic resonance imaging (MRI) showed a T1-isointense (Figure ), T2 heterogeneously hyperintense (Figure ), heterogeneously enhancing lesion (Figure ) originating from the intramedullary cavity with osseous destruction of the humerus and a soft tissue component that measured up to 15 cm in superior-inferior dimension. A serpiginous, linear area of low T1 and low T2 signal consistent with a bone infarct was noted at the lesion, and this area of infarct extended more distally in the humeral diaphysis. This bone infarct corresponded to the linear area of sclerosis seen in the humeral diaphysis on prior radiographs.\nUltrasound-guided core needle biopsy of the soft tissue component of the lesion was performed. Histological analysis of the biopsy showed neoplastic spindle and epithelioid cells with focal osteoid formation (Figure ). A few giant cells were also noted. There were frequent mitotic figures (4 per high powered field). Areas of hemorrhagic necrosis were present. No chondroid material was noted. Immunohistochemical stains with adequate controls were performed. The epithelioid cells were negative for TTF-1, S100, AE1/3, PSA, HMB45, ERG, CD31, CAIX, HepPar, PAX8, and pancytokeratin. INI1 was retained. This immunoprofile combined with the histological findings supported a diagnosis of a tumor of bone origin and were most consistent with an osteogenic sarcoma (Figure ). Staging 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed localized disease (Figure ).\nHe was treated with reduced dose cisplatin/adriamycin given his age. He had a poor response to chemotherapy with enlargement of the mass through chemotherapy with the development of circumferential disease around the humerus. He underwent a right forequarter amputation. He developed lung metastases and an L4 spinal metastasis with pathological fracture two months following surgery. He received palliative radiation therapy to the L4 vertebra and died seven months after initial presentation. |
A 40-year-old woman with SLE and the antiphospholipid syndrome presented acutely with sudden onset left sided loin pain. She was on life-long warfarin therapy for multiple DVTs and pulmonary emboli and hydroxychloroquine. Ultrasound scans showed a 10 mm focal area in the left kidney due to scarring or a small angiomyolipoma. A CT scan several months later showed a 2.7 × 3.9 cm left adrenal incidentaloma (), and she was referred to the endocrine clinic.\nOn examination, she was normotensive and had no clinical signs of endocrinopathy. Investigations including cortisol after 1 mg dexamethasone suppression overnight, urinary free cortisol (twice), urinary catecholamines (twice), aldosterone renin ratio, 17-hydroxyprogesterone, and chromogranins were all within the reference range. MRI scans showed a solid left adrenal lesion measuring 2.4 × 4 cm. without significant fat and no interval growth. However, in view of the phenotype and size of the adrenal mass she was referred to the endocrine surgeons. Following a review of her history of loin pain, and imaging, it was decided that she had a likely benign NSAI (uniform soft tissue signal intensity, well-defined margins, no local invasion, appropriate size, and no interval growth over 3 months), who may have developed acute symptoms as a result of haemorrhage into the lesion. Therefore a conservative approach was adopted with scans to be repeated in 3–6 months. Although the size of the lesion initially remained stable, imaging one year later revealed that it had increased in size to 3 × 5.4 cm. The patient therefore had left laparoscopic adrenalectomy. At surgery, there was no macroscopic invasion of adjacent structures and the lesion was excised with periadrenal fat.\nHistology revealed a nonencapsulated high-grade leiomyosarcoma thought to have arisen from connective tissue but separate from the adrenal gland, that is, a retroperitoneal leiomyosarcoma (RL). There were atypical pleomorphic spindle cells forming a herringbone pattern in areas (). These cells had cigar shaped nuclei with some multinucleated forms. Immunostaining revealed strong expression of smooth muscle actin and desmin. There was no AE1/AE3, S100, CD117, CD34, or CD99 expression. Proliferation fraction on Ki67 immunostaining was approximately 30%. Staging CT confirmed only localised disease. She had radiotherapy as recommended by the Sarcoma MDT. |
A 58-year-old designated female at birth with a past medical history of insomnia and depression who presented to clinic requesting HIV pre-exposure prophylaxis (PrEP), because he anticipate having new sexual partners soon. He is not currently sexually active. He is open to multiple types of sexual encounters, but has no planned partner and is not sure yet what sexual practices he will participate in. He achieved appropriate developmental milestones as a child. The patient wanted to transition 16 years back but a lack of awareness of medical and surgical interventions available and resources held him back. He came through with his decision to transition 15 months ago with a good support system around him. The patient has no prior history of cerebrovascular accidents, thromboembolism, myocardial infarction, seizures, or fractures. He is currently using three pumps of topical 1.62% 20.25 mg testosterone per day and has been on testosterone for 15 months. He is tolerating it well without any side effects. He denies any alcohol use and smokes half a pack per day of cigarettes. Current medications apart from testosterone include vitamin D supplements, melatonin, folic acid, and magnesium oxide.\nHis hemoglobin was 14.7 g/dL and hematocrit was 44%. The patient’s creatinine increased since the initiation of testosterone therapy. Creatinine before starting hormonal therapy was 0.9 mg/dL, and the recent laboratory findings showed creatinine of 1.2 mg/dL. Liver function tests were all within a normal range. The HIV antibody test, gonorrhea, chlamydia, and syphilis screen were negative. The urinalysis showed that the urine culture was negative and renal ultrasound showed normal size kidneys with no hydronephrosis.\nGiven the patient’s age of 58, the weight of 147 pounds, and using the birth gender, the estimated creatinine clearance of 48 mL/min was calculated per Modification of Diet in Renal Disease (MDRD) and 52 mL/min per Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) creatinine equation. He was denied emtricitabine/tenofovir disoproxil fumarate (Truvada) PrEP for HIV prevention given that his estimated glomerular filtration rate (eGFR) was less than 60 mL/min. However, if his eGFR were calculated using his current gender, which turned out to be greater than 60 mL/min, he would have qualified for the PrEP (). |
Case 1 is a 48-year-old male of Chinese ancestry diagnosed with metastatic left-sided adenocarcinoma of the colon at age 45. Given his young age at onset he was referred for GCRA. His family history was devoid of other cancers (Figure ). In the absence of polyposis, his early age at diagnosis prompted the pathology laboratory to complete microsatellite instability testing and immunohistochemistry for the mismatch repair (MMR) proteins (MLH1, MSH2, MSH6, PMS2) associated with Lynch syndrome (LS), both of which returned showing no evidence of defective MMR. Given his young age, residual small risk for LS, and remaining concern for potential MUTYH-associated polyposis or attenuated familial adenomatous polyposis (AFAP), a multigene cancer panel was chosen to try and better understand potential hereditable cancer risk. He was found to carry a pathogenic mutation in CDH1, designated c.283C > T.\nCDH1 is a tumor suppressor gene that encodes epithelial cadherin. Germline mutations have been associated with hereditary diffuse gastric cancer (HDGC), a rare autosomal dominant condition historically thought to be highly penetrant, with evidence to suggest a cumulative diffuse gastric cancer risk of 80% by 80 years. Women with HGDC also have a 39–52% risk for lobular breast cancer (, ). Given the lack of efficacy in available screening for diffuse gastric cancer, current consensus guidelines indicate that prophylactic gastrectomy should be considered for mutation carriers (). There is currently insufficient evidence to suggest that colorectal cancer (CRC) is part of the spectrum of HDGC-related cancers (, ), so the genetic finding does not appear to explain the patient’s phenotype. This specific mutation has been reported once previously and was associated with multiple individuals with invasive lobular breast cancer in a family without DGC or CRC ().\nThe identification of a CDH1 mutation in this case was considered an incidental, yet potentially meaningful test result for the family. Given the absence of stomach cancer in the family, his mutation was likely either de novo (a new germline mutation), or the familial penetrance of gastric cancer is low with this mutation; similar to the above family previously reported in the literature (). Given his current poor prognosis no further management recommendations were made for him at this time. Unfortunately, both of his parents were deceased and therefore it may not be possible to further clarify whether this was an inherited mutation with low penetrance, or a de novo mutation. If a sibling pursues testing and tests positive, parental gonadal mosaicism (a proportion of a parent’s sperm or eggs had the mutation) could be another possibility. Either way, in this situation testing for all first-degree relatives was recommended to clarify if there are other individuals at elevated cancer risk. His children were recommended to have testing between the ages of 18 and 21. For individuals identified with the mutation, gastrectomy would need to be considered since DGC surveillance has unproven value (). Enhanced breast screening, including annual breast magnetic resonance imaging (MRI) for female carriers, would also be recommended (). |
A 24-year-old, 165-lb male Caucasian patient with diabetes mellitus type 1 presented to the emergency department with a complaint of abdominal pain accompanied by nausea and vomiting. The patient stated that he was unable to self-administer basal insulin for two days after he ran out of insulin pen needles. His vital signs on presentation revealed blood pressure of 84/53 mmHg, heart rate of 103 beats per minute, respiratory rate of 18 breaths per minute, and temperature of 98.2 °F (36.7 °C). Physical examination at time of presentation showed that he was ill-looking, with dry oral mucosa and poor skin turgor. Initial diagnostic tests showed high anion gap, metabolic acidosis, ketonemia, and hyperglycemia, prompting the start of diabetic ketoacidosis management and subsequent intensive care unit (ICU) transfer (Table ). At this time, the patient only had a gauge-22 peripheral line placed at the dorsum of his left hand after multiple unsuccessful attempts at placing a larger peripheral line gauge.\nIn the ICU, efforts at internal jugular vein central line placement were unsuccessful as well due to the patient’s low volume status, making the target vein extremely difficult to cannulate even by increasing Trendelenburg tilt and doing venipuncture at end inspiration. Right subclavian vein catheterization using the infraclavicular approach was instead performed. However, the postprocedure chest X-ray showed that the catheter was somehow in the aorta (Fig. ). Blood gas obtained from the subclavian central line revealed arterial placement, prompting removal of the catheter with no serious consequences to the patient (Table ; Fig. ). A right femoral line was successfully placed, and continuous management of diabetic ketoacidosis was achieved with eventual normalization of the anion gap and clinical improvement of the patient. The patient stayed in the intensive care unit for 2 days and was then transferred to the medicine floors for continuation of glucose management with eventual discharge on the 4th day after case management; social work assisted in securing his insulin supplies, and diabetic education was performed. |
The patient was a 79-year-old man with a history of diabetes mellitus type 2, hypertension, paroxysmal atrial fibrillation and chronic heart failure, who presented at the emergency department of a community hospital in Amstelveen, the Netherlands. He had been tired and short of breath for the last two weeks, with increasing dyspnea and confusion for 1 day. At presentation he was disoriented and hypoxic, had a heart rate of 100/min and mean arterial pressure of 60 mmHg. Chest X-ray showed infiltrates bilaterally. Laboratory results showed a C-reactive protein of 100 mg/L, leucocytes of 10 × 109/L and a lactate acidosis with acute kidney failure. The SARS-CoV-2 PCR on a nasopharyngeal swab tested positive. Due to respiratory insufficiency, he was immediately admitted to the intensive care unit for intubation and invasive ventilation and hemodynamic support with norepinephrine. Intravenous ceftriaxone and ciprofloxacin were started empirically, the latter switched to flucloxacillin when sputum cultures showed growth of 104–105 CFU of S. aureus.\nFour days after admission, the condition of the patient deteriorated, requiring increasing dosage of vassopressors and respiratory support, and showed decreased kidney function. Hydrocortisone treatment of 200 mg/day and meropenem were started, and ventilation in prone position and continuous renal function replacement therapy were initiated. In the following days, the clinical condition of the patient improved; prone position ventilation was discontinued after two days, norepinephrine and renal replacement therapy could be discontinued. The hydrocortisone dose was lowered after four days.\nHowever, respiratory support could not be weaned below an inspired oxygen fraction of 45%, and the patient was transferred to a tertiary care hospital 14 days after admission. Here, the patient again deteriorated, requiring increasing respiratory support and continuous renal function replacement therapy had to be restarted. Chest X-ray showed bilateral consolidations, more outspoken in the right lung. Sputum cultures showed growth of A. fumigatus, the serum galactomannan index was 1.7, serum 1,3-ß-D-glucan 28 picogram/mL, and anidulafungin and voriconazole were started next to vancomycin and ceftazidime. Subsequent non-bronchoscopic broncho-alveolar lavage (BAL) had a galactomannan index of >7.9, and showed growth of A. fumigatus. Molecular resistance typing showed no resistance associated mutations for azoles. Agar-based screening using VIPcheck (Radboud University Medical Center, Nijmegen, the Netherlands []) showed no phenotypic resistance to azoles. Despite therapy, the patient showed increasing hypoxia, hypercapnia and hypotension. Further medical treatment was deemed futile by the attending care team, and it was decided to abstain for further treatment. The patient died 17 days after admission. Consent for autopsy was provided; macroscopically, bilateral lung edema was found, with multiple white-yellow consolidations, and multiple lung emboli in the right lung artery. Both lungs showed abscess filled with pus up to a diameter of 6.5 cm (A,B). Cultures of two separate biopsies of these lesions showed growth of A. fumigatus, but not of S. aureus. Histologically, there was a profound congestion, edema and intra-alveolar hemorrhage with formation of hyaline membranes, interstitial inflammation and fibroblastic proliferation throughout the lungs. These findings are consistent with severe diffuse alveolar damage (A).\nIn addition, sampling from abscesses showed collections of neutrophils and necrotic tissue inside the cavities, and hemorrhagic infarction of the surrounding lung parenchyma (B). No Aspergillus hyphae could be detected in PAS-diastase and Grocott stains. |
A 67-year-old male initially underwent contrast-enhanced multidetector CT of the abdomen in Sept 2014 at another hospital, which showed an incidental 4.2 × 3.9 cm mass in the upper abdomen. This was interpreted as a head of pancreas mass causing portal vein compression. The patient was asymptomatic; physical and laboratory examinations were all unremarkable.\nThe patient was lost to follow-up and did not receive any further investigation or treatment. Although still asymptomatic, he re-presented 12 months later for a repeat MDCT, which showed that the mass had increased in size to 4.8 × 5.0 cm. Cavernous transformation of the portal vein was also observed. The patient underwent exploratory laparotomy, which showed a tumour at the upper border of the pancreas and duodenum compressing the portal vein. A 1-cm lesion in the subcapsular aspect of segment II of the liver was also seen. The presumed pancreatic tumour was deemed unresectable and multiple biopsies were taken. The liver lesion was resected and the histology of both samples showed spindle cell tumour. The patient also underwent a positron emission tomography-CT, which showed the lesion to be hypermetabolic with SUVmax of 7.3. No metastatic disease was identified.\nThe patient was then referred to the hepatobiliary team in Queen Mary Hospital in Hong Kong for further assessment. The patient remained asymptomatic clinically with normal laboratory results including liver biochemistry (bilirubin 4 μmol l–1, alkaline phosphatase 54 U l–1, alanine aminotransferase 29 U l–1 and aspartate aminotransferase 23 U l–1). A repeat MDCT 2 months later showed that the lesion had a soft tissue density on non-contrast scans. The mass now measured 5.3 × 5.9 × 6.4 cm, extending from the superior mesenteric vein/splenic vein confluence to the porta hepatis. It exhibited heterogeneous enhancement with feeding vessels seen on the arterial phase, both within and around the mass. On the portal venous phase, numerous collaterals were seen surrounding the mass with a sharp interface between the mass and opacified portion of the main portal vein at the porta hepatis giving the appearance of a “beak” (). The pancreatic duct was mildly dilated and measures 3 mm. The biliary tree was not dilated. There was splenomegaly measuring 13.5 cm craniocaudally. No gastroesophageal varices or ascites was appreciated. Based on these MDCT and histological findings, a preoperative diagnosis of primary leiomyosarcoma of the main portal vein was made.\nThe patient underwent Whipple’s procedure and portal vein reconstruction using cadaveric graft. Intraoperative findings confirmed the tumour arising from inside the portal vein and confined to the lumen with no macroscopic evidence of disease spread. Histological examination of the tumour showed spindle cells with enlarged, pleomorphic, hyperchromatic nuclei and abundant eosinophilic cytoplasm. Patchy coagulative necrosis was also seen. Mitotic figures were at 8 per 50 high power fields, and atypical mitotic figures were found. There was focal infiltration into adjacent pancreatic parenchyma. Immunohistochemical stains showed the tumour was positive for actin, h-caldesmon and desmin but negative for myogenin, c-kit and S100 protein. The final histological diagnosis was of a primary LMS of the portal vein (). The patient made an uneventful recovery and no adjuvant radiotherapy or chemotherapy was given. He was followed up for 4 months in our institute with no complications. |
A 67-year-old Japanese woman visited our hospital with swelling of the right temporal region that had been present for the prior three weeks and left-sided hemiparesis for the prior one week. The patient had no history of medication or surgical intervention. The results of routine laboratory tests were normal. On computed tomography (CT), the right temporal mass consisted of two components; an extracranial component in the right masticator space and an intracranial component in the right middle cranial fossa (). The skull base sandwiched by the two components of the mass was not destroyed, but rather showed sclerosis and thickening ().\nOn magnetic resonance imaging (MRI), the extracranial component showed homogeneous high intensity on T2W () and iso-intensity on T1-weighted (T1W) images compared to that in the muscle. The intracranial component showed heterogeneous high intensity on T2W () and T1W images (). Many flow voids were seen within the mass. On diffusion-weighted imaging (DWI), the solid area showed mild high intensity (). The minimum apparent diffusion coefficient (ADC) of the solid area was 0.88 × 10−3 mm2/s.\nOn coronal contrast-enhanced fat-suppressed T1W images, the mass showed homogenous contrast enhancement in the extracranial component, whereas it showed heterogeneous enhancement in the intracranial component. The extracranial and intracranial components communicated via the foramen ovale (). Magnetic resonance angiography (MRA) showed that many branches of the right external carotid arteries (branches from deep temporal artery and middle meningeal artery) were distributed in the tumor (). Perfusion-weighted images showed elevated blood flow and blood volume in the tumor.\nAs the intracranial component of the mass caused compression and distortion of the brainstem, which threatened the life of the patient, emergency surgery was performed and the intracranial component of the mass was resected. The tumor was fibrous and severely adhered to the dura of the temporal base, although the margin between the tumor and temporal lobe was clear. Intraoperative findings proved the intracranial tumor component was connected to the extracranial component through the foramen ovale.\nThe histopathologic analysis showed the tumor consisted of interlacing, fascicular proliferated spindle-shaped tumor cells and fibrocollagenous stroma (). Rhabdomyoblast-like cells with cellular atypia were occasionally observed (). Bone invasion of the tumor cells was identified. Dilated blood vessels, necrotic changes, and hemorrhages were frequently seen. On immunohistochemistry, the tumor cells were positive for desmin (), myogenin (), and vimentin, but negative for caldesmon and CD34. The Ki-67 labeling index was 36.6%. In conjunction with the histological and immunohistochemical findings, the tumor was diagnosed as ssRMS.\nThe patient’s symptoms were relieved after the surgery and no additional treatments were performed. The patient died of an intracranial recurrence of the tumor at approximately three months after the surgery. |
A 14-year-old male patient came to our department with a huge cauliflower-like progressive ulcerated neck mass and bilateral axillary lymphadenopathy for 8 months with grade intermittent fever, weight loss, and fatigue for the past 4 months.\nEight months before, he was diagnosed tubercular lymphadenitis by fine-needle aspiration cytology (FNAC) and given ATT (anti-TB treatment) for the past 6 months. After complete the anti-tubercular drug (ATD), the neck mass was again progressively increased with bilateral axillary lymphadenopathy. Patient also complained low fever, loss of appetite, weight loss, and night sweat. After 1 month, he came to our outpatient department.\nIn our department, the physical examination revealed a bulky ulcerative cauliflower-like diffuse anterior neck mass measuring 8 cm × 8 cm involving the submental and anterior cervical region and bilateral axillary lymphadenopathy measuring 3 cm × 2 cm each [ and ]. FNAC from the solid area of ulcerated neck mass and axillary lymph node were done, and the cytosmear revealed sparse large atypical lymphoid cells over a background of necrosis and acute inflammatory cells. Thus, the diagnosis was suspicion of NHL []. His routine blood examination was normal except for increased erythrocyte sedimentation rate and neutrophilic leukocytosis with left shift. Serological test such are HIV, hepatitis B surface antigen, and HIV were negative. X-ray and ultrasonography abdomen and pelvis findings were unremarkable. Computed tomography scan of the neck showed cervical lymphadenopathy with central necrosis. Punch biopsy from the ulcerated mass was done and histopathology section showed the presence of atypical lymphoid cells characterized by large cells with round morphology. Some amount of eosinophilic cytoplasm, fine chromatin, and prominent central nucleoli scattered over a loose fibro-collagenous stroma. Some cells had plasmacytoid morphology. There were areas of dense neutrophillic aggregation and necrosis. The diagnosis was given as DLBCL []. Immunehistochemistry reports revealed the tumor cells are CD20 and negative for Alk-1 and CD30. The final diagnosis was concluded as DLBCL. Peripheral smear and bone marrow aspiration cytology did not show the presence of any atypical lymphoid cells. |
A 56-year-old male, a chronic smoker (11 pack/year), presented with 5-month history of right lumbar region pain, intermittent hematuria, and weight loss. His past medical history was unremarkable. Clinical examination revealed a bimanually palpable right flank mass.\nAn abdominal computerized tomography (CT) scan showed a 25 × 11 × 8 cm heterogeneous mass occupying the right kidney and extending into the renal vein lumen while compressing the IVC (). The rest of the abdominal cavity was unremarkable. A CT scan of the thorax showed nodular lesions that may correspond to a secondary location of his tumor.\nThe other laboratory findings were normal.\nThus, a right radical nephrectomy with the resection of the thrombus of the right renal vein was performed.\nIn the laboratory, the gross examination revealed a huge encapsulated mass weighing 2300 g and measuring 27 × 13 × 9 cm. The cut surface was gray-white showing a whorled appearance with foci of hemorrhagic and necrotic changes. The tumor was attached to the renal vein wall. A portion of the tumor occupied the renal vein lumen, but most of this huge tumor extended beyond the hilum area compressing the normal renal parenchyma ().\nMicroscopic examination showed an encapsulated tumor composed of interlacing fascicles of spindle-shaped cells with elongated blunt-ended nuclei, coarse chromatin, and moderately abundant eosinophilic cytoplasm, intermingled with pleomorphic cells presenting marked nuclear pleomorphism, hyperchromasia, and prominent nucleoli. The mitotic count was 8 per 10 high-power fields with 3 atypical mitoses. Areas of tumor necrosis were present. A portion of the wall of the renal vein was sarcomatous in continuity with the tumor. There was a capsule separating the tumor from the normal renal parenchyma. The renal vein's surgical resection margin was free of tumor ().\nIn the immunohistochemical study (), the tumor cells showed diffuse positivity for smooth muscle actin (SMA), heavy chain caldesmon (H-caldesmon), and desmin. They were negative for cytokeratin AE1/AE3, epithelial membrane antigen (EMA), CD34, melan A, and HMB45.\nThe pathological diagnosis of leiomyosarcoma of the renal vein, grade II of the FNCLCC (the National Federation of Centers for the Fight Against Cancer) and pT4NxMx (UICC, 8th edition 2017), was therefore retained.\nAfter surgery, the patient's general condition was very impaired and no neoadjuvant treatment was administered. The patient passed away one month later. |
A 45-year-old male presented with complaints of episodic dizziness occurring two – three times per day in the past eight months. He had attended Neurology out patient department (OPD) in October 2006 for episodes of giddiness. The Computerized Tomography (CT) Scan taken at the time was normal and no neurological disorder was detected at that time. He came for psychiatric consultation eight months later on his own. Meanwhile, he had been treated symptomatically at general medical and surgical departments. Random blood sugar showed 58 mg% at the time of admission.\nDuring the onset of the illness, he was having only episodes of giddiness lasting 1-2 min. This progressed to episodes of confusion with other behavioral changes lasting 5-30 min. During the episodes of confusion, patient had slurred speech, altered behavior like banging his head against the wall, walking aimlessly and talking irrelevantly. Patient was having intense sweating and autonomic symptoms during the episodes. Clonic movements and tremors of both arms were also observed during some episodes.\nDuring the episodes he was not oriented to time, place and person. He was also completely amnesic for the episodes after he came out of the episodes. Episodes initially occurred in the afternoon and then more in the nights during sleep and in the evenings. He had no incontinence of urine during the episodes. He never had any generalized seizures or aura or premonitory symptoms preceding the episodes.\nGeneral and systemic examination was normal. Central nervous system examination showed mild dysarthria. Psychomotor activity was normal and mood was euthymic. Thought and perception were normal. Cognitive functions showed mild impairment. Insight was present. At psychiatric OPD he was diagnosed as episodic confusional state and admitted for observation and to exclude insulinoma.\nAfter admission in the ward, he developed an episode which was reversed by administration of glucose. Patient was in confusional state with autonomic symptoms like tachycardia and profuse sweating. Clonic movements of limbs were also observed. The patient was not oriented to time, place and person. Blood glucose level showed 50 mg% during this episode []. |
A 3-year-old girl presented with cyanosis. On examination heart rate of 130/min, blood pressure of 100/64 mmHg, and arterial oxygen saturation of 81% were noted. Echocardiography revealed an unbalanced atrioventricular septal defect with double outlet right ventricle and left atrioventricular valve stenosis. The ostium primum atrial septal defect was tiny and restrictive. The ventricular septal defect was very large and biventricular repair was not feasible. A patent ductus arteriosus was also present. Cardiac catheterization showed mean pulmonary artery pressure of 77 mmHg with pulmonary artery wedge pressure of 37 mmHg. Atrial septectomy and ligation of the patent ductus were done along with pulmonary artery banding with 34 mm Mersilene. The postoperative course was uneventful.\nTwo years later, the child presented with massive ascites with pedal edema and facial puffiness. There was no history of fever, recurrent chest pain, or any other systemic illness during this time. The ascites was gradually progressive. On physical examination, she had elevated jugular venous pressure with hepatomegaly and ascites. There was no pulsus paradoxus. Routine blood investigations were normal. Abdominal ultrasound showed free fluid, hepatomegaly, single left sided spleen, and no other abnormality. Her chest X-ray [] showed cardiomegaly and pulmonary venous hypertension. Electrocardiography showed ectopic atrial rhythm, first-degree atrioventricular block, and poor R wave progression but was unchanged from the previous recording.\nEchocardiography showed features of congestion such as dilated inferior vena cava and lack of inspiratory collapse. There was no pericardial effusion. Biatrial enlargement was also present. There was no significant atrioventricular valve regurgitation, and systemic ventricular function was normal. Respiratory variation in inflow velocities across the atrioventricular valves or septal bounce were not appreciated due to the presence of a large ventricular septal defect and atrial septectomy. Diagnosis of constrictive pericarditis (CP) was suspected despite cardiomegaly and atrial dilation.\nCT angiogram, [] however, did not reveal pericardial thickening or calcification. Previous records did not suggest any evidence of the development of postpericardiotomy syndrome (PPS) in the first year after surgery. There was no history of tuberculosis.\nA repeat cardiac catheterization showed elevation of right atrial, ventricular diastolic, and pulmonary artery diastolic pressures all being nearly equal around 20 mmHg. Mean pulmonary artery pressure was 33 mmHg with pulmonary vascular resistance index (PVRI) of 2.9 Wood units. The right atrial pressure of 22 mmHg in the absence of a significant left to right shunt, ventricular dysfunction, or atrioventricular regurgitation was suggestive of a constrictive physiology.\nThe diagnosis was confirmed at operation. The pericardium was found to be adherent to the underlying epicardium over the right atrial and ventricular walls. The pericardium, however, was not significantly thickened. Histopathological examination of the excised pericardial tissue showed fibrosis, hyalinization, and focal chronic inflammation consistent with pericarditis [].\nSince the calculated PVRI at preoperative catheterization was 2.9 Wood units, and the pulmonary arteries were thin walled at pulmonary arteriotomy, bilateral bidirectional cavopulmonary shunt with antegrade flow interruption was carried out 4 days after pericardiectomy as an intermediate palliation for the future. The staged surgery was done in view of the unusual combination of POCP and the presence of an underlying condition requiring univentricular palliation and can be argued. This is, however, not the point of this communication. There was a remarkable regression in the ascites and marked symptomatic improvement. On follow-up 1-year after pericardiectomy the child is active and growing adequately []. |
An otherwise healthy 23-year-old Caucasian man presented to our Emergency Department reporting a sensation of “bubbles” under the skin of his neck and mild chest pain. He denied any dyspnea, neck pain, dysphagia, odynophagia, or dysphonia. He reported that he had “snorted” a small amount of cocaine two nights prior to his Emergency Department presentation. He did not have a history of chronic or regular drug use. There was no history of any trauma or vigorous physical exercise. He had mild rhinorrhea but no coughing or vomiting. He did not take any regular medication, but reported the occasional use of illicit drugs such as cocaine and ecstasy.\nOn examination, he was alert and responsive without any airway compromise or respiratory distress. His pulse rate was 96 beats per minute with a blood pressure of 120/73mmHg. He had a respiratory rate of 18 breaths per minute with oxygen saturation of 97% on room air and he was apyrexial. Subcutaneous crepitations were palpable across his neck and the superior part of his chest. The rest of his physical examination was unremarkable.\nResults from baseline blood tests, including arterial blood gases, were unremarkable and an electrocardiogram showed a normal sinus rhythm with no arrhythmias or segmental changes. A plain posterioranterior chest radiograph revealed subtle pneumomediastinum and soft tissue emphysema at the base of his neck with no associated pneumothorax (Fig. ). A plain radiograph of his neck demonstrated extensive subcutaneous emphysema (Fig. ). Computed tomography of his neck and chest was performed, and confirmed the above radiographic findings (Figs. and ). No additional sequelae of cocaine use, such as granulomas, emphysema, or bullae, were seen on pulmonary imaging. In order to rule out any esophageal involvement, our patient was transferred to a tertiary center for a barium swallow fluoroscopy study, which was normal (Fig. ).\nHe was admitted under the care of the cardiothoracic surgery team at the tertiary center. He was managed conservatively and observed for a further 24 hours, during which the subcutaneous emphysema gradually improved although did not resolve completely. He was discharged home after a total of 48 hours in hospital. He was followed-up in the community by his general practitioner and was found to be well 3 months later. |
A 40-year-old male presented with chief complaints of lump in the central part of his left breast for 3 months. It was gradually increasing in size but there was no history of pain. He did not have any significant past history of illness and his family history was unremarkable. On examination, it was a firm, mobile, nodular, non-tender swelling and showed an area of ulceration on the overlying skin. The lump grossly measured 6.5 cm × 4 cm and the area of ulceration measured 3.2 cm × 2 cm. His hematological and biochemical parameters were within normal limit. Fine needle aspiration cytology (FNAC) was done from the swelling and smears showed large number of ovoid- to spindle-shaped cells with mild to moderate nuclear atypia and scanty cytoplasm. The cells were arranged discretely and also in some clusters in a myxoid background []. Impression of FNAC smears was 'mesenchymal lesion, probably intermediate grade neoplasm'. The patient then underwent left-sided modified radical mastectomy []. Gross appearance of the tumor was partly solid and partly cystic. The solid area was grayish fleshy in appearance. Histopathological examination of sections from tumor revealed a tumor composed of spindled-shaped cells having plump nuclei, arranged in intersecting fascicles and storiform pattern []. The tumor cells showed nuclear pleomorphism and increased mitotic figures (8-10/10 high power field) were seen in some areas []. Plexiform capillary network was seen in the background with areas of hemorrhage and myxoid change []. The tumor involved overlying skin, nipple and areola []. Surgical lines of resections were tumor-free and all the 19 lymph nodes dissected from axillary pad of fat showed reactive changes. Impression of histopathological examination was spindle cell sarcoma of left breast, and paraffin blocks of the tumor were subjected to immunohistochemical (IHC) test. On IHC test, tumor cells were reactive to CD34 [] and non-reactive to S-100, smooth muscle actin, desmin, cytokeratin and epithelial membrane antigen. So, the final diagnosis of DFSP with areas of fibrosarcomatous change was given. |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.