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A 52 years old male patient presented with syncope and self-limited ventricular tachycardia in a referring hospital. Inferior wall ST- Elevation myocardial infarction (STEMI) was diagnosed. He developed ventricular fibrillation requiring endotracheal intubation, cardioversion and cardiopulmonary resuscitation for 2 min.\nCoronary angiography showed severely diseased left anterior descending, and right coronary arteries (Ejection Fraction < 25% (EF). A right coronary artery angioplasty was unsuccessfully attempted and the patient referred for emergency CABG. An intraaortic balloon pump (IABP) was inserted before surgery and myocardial revascularization was accomplished by bypassing the left internal mammary artery to the left anterior descending coronary artery after thrombendarterectomy and saphenous vein grafting to the right coronary artery. Both grafts had good flow to the target vessels as determined by Doppler flow measurements.\nDespite prolonged reperfusion and biventricular pacing weaning from extracorporeal circulation was not possible. Intraoperative transoeosphageal echocardiography (TEE) demonstrated a failing right ventricle, and severely impaired left ventricular function. We decided to support the failing right ventricle by a centrally implanted veno-pulmonary ECMO (V-P ECMO), and left the sternum open due to myocardial edema. Pulmonary artery cannulation was accomplished with two purse string sutures with prolene 5/0 placed in the proximal pulmonary trunk. The PA cannula was tunneled to the left side through a 2 cm stab incision in the 4th intercostal space (midclavicular line) and secured with the purse string sutures. Venous drainage was established through a two stage venous cannula inserted in the right atrium. The cardiopulmonary bypass was transferred to Cardiohelp ECMO circuit (Maquet Cardiovascular, LLC, Wayne, NJ) and the IABP was left in place.\nThe sternum was kept opened by placing a plastic syringe between the two borders of the sternum. Skin and subcutaneous tissues were covered with sterile dressing which was changed daily in the intensive care unit using strictly sterile technique.\nThe patient was covered with broad spectrum antibiotic (Cefotriaxone 1 g /8 h), and anticoagulated with heparin keeping the partial thromboplastin time (PTT) at 50–60/s.\nIn the ICU, the ECMO flow was maintained form 3 to 3.5 L/min and pharmacologic myocardial support was continued to maintain mean arterial blood pressure around 50–60 mmHg and urine output at 0.5 ml/kg/h.\nOn the postoperative day 2, he developed intractable pulmonary edema, elevated left atrial pressure and hemodynamic instability. TEE showed a dilated left ventricle and persistent poor left and right ventricular contractility (EF =15–20%). We decided to insert Impella Recover LP 5.0 (Abimoed Denver, MA USA) to achieve complete left ventricular unloading and continue the use of V-P ECMO to support the right ventricle. Since the sternum was still open, the Impella was implanted thorough am 8 mm Dacron graft sutured to the ascending aorta The Dacron graft was then subcutaneously tunneled and exteriorized through a separate 2 cm transverse incision in the second right intercostal space just lateral to the sternum. Under TEE control, the Impella 5.0 was then advanced through this Dacron graft into the left ventricle and secured with three ligatures and IABP was removed.\nOn POD 3, the Impella 5.0 showed low flow alarm; with high driving pressures TEE revealed kinking of the Impella. After repositioning the right side pressure, left side volume overload and pulmonary edema rapidly decreased (Fig. ). The patient remained hemodynamically stable on biventricular support with V-P ECMO and Impella 5.0 till the 5th postoperative day. When closing the sternum on POD 5, the right atrial two stage cannula was replaced with a long peripheral cannula inserted from the left femoral vein (Fig. ).\nSerial TEE follow up showed no improvement of both right and left ventricular contractility (EF 10–15%), necessitating implantation of a permanent LVAD System (Heart mate II- Thoratec Corporation, USA) on the POD 14. Intra operative TEE control showed no right ventricular improvement so we replaced the directly inserted pulmonary cannula of the V-P ECMO with another cannula inserted through a 8 mm Dacron graft sewn to the main pulmonary artery and tunneled subcutaneously and exteriorized through the old incision for future easy removal under local anesthesia by suture closing the graft. (Fig. ). The patient was consecutively listed for heart transplantation.\nAfter gradual improvement of the right ventricle as demonstrated by serial TEE echocardiography, V-P ECMO could be explanted on POD 66. During this long ICU stay, the patient underwent hemodialysis for renal insufficiency and tracheostomy for prolonged mechanical ventilation with complete recovery. He was transferred to the normal ward on POD 77.\nSix months later the patient underwent orthotropic heart transplantation with uneventful postoperative course; he was discharged from the hospital after 7 months of admission in a good condition, and has completed three and half years of uneventful post-transplant follow up to date. |
A 17-year-old woman with primary amenorrhea was sent to us with a diagnosis of Rokitansky syndrome. The patient was born via normal delivery at a weight of 2400 g when her mother was 17. She was admitted to the hospital at an age of 8 days due to vomiting and moderate dystrophy; she was then diagnosed with primary right PH. She was later readmitted several times for pulmonary insufficiency. Two months later, a diagnosis of congenital right pulmonary hypoplasia with hypoplasia of the right lung artery was confirmed. In an ultrasound examination performed 7 months later, the right kidney was not observed. From more recent (at an age of 15 years) X-ray images taken of the pelvis in the Emergency Unit, the patient was also diagnosed with right hip dysplasia; this condition was initially defined as old secondary osteonecrosis of the right femoral head.\nThe patient reported three years of genital and mammary development as well as cyclic pelvic pain for 4-5 days every month despite the primary amenorrhea. She weighed 42 kg and was 153 cm tall. The physical examination revealed normal external genital development and normal breasts. There was complete vaginal atresia. In the combined rectal examination, the pelvis was noted to be free. A transrectal ultrasound did not confirm the presence of a uterus. In this ultrasound examination, there was difficulty visualizing the ovaries; on the right side, however, vascular dilatations or multicystic embryonary remnants could be observed. The abdominal ultrasound did not show the presence of a right kidney. General and hormonal analyses were normal (FSH 3.9 mUI/mL, LH 6.2 mUI/mL, PRL 7.9 ng/mL, E2 35 pg/mL, P 0.3 ng/mL), and chest X-rays and i.v. pyelography showed right pulmonary hypoplasia, dysplasia of the right hip, and right renal agenesis with a normal left kidney and ureter (see Figure ). Later, a conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH (Agilent Technologies, Santa Clara, CA, USA), detecting 11 copy number variants (CNV) described as normal variants in the databases. Some CNVs (17q12 and 22q11.21 microdeletions) described in other MRKH patients were not present in this case. The geneticists have been studying her family history and Figure shows the pedigree of the family, compatible with an autosomal dominant pattern of inheritance.\nEight months later, a new transrectal ultrasound was performed. Again, there was no evidence to suggest any functioning rudimentary uterine horn. The right ovary appeared to be normal, and there were the same vascular dilatations or multicystic remnants previously seen on the right side. The left ovary was also difficult to identify properly. The patient, who visited the clinic with her parents, wished operation and to have a neovagina created. She was therefore scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft.\nLaparoscopy confirmed the absence of uterus. There was a varicocele and/or retroperitoneal multicystic formations on the right side of the small pelvis. On both sides, an elongated, solid, rudimentary uterine horn could be observed. At its superior end, there were ovaries that were elongated, somewhat dysgenetic (more on the right side), and located very high in both abdominal flanks (Figure ). Rudimentary tubes could also be observed. Although the uterine rudimentary tract was slightly thicker on the left side, there did not appear to be even minimal endometrial cavitation of the solid uterine rudimentary horn. Sectioning and dissection from the introitum to the inside below the urethra was performed via a transperineal procedure, thus forming a wide vaginal neocavity reaching the Douglas pouch. Interestingly, the dissection of the neovaginal cavity tended more toward the left fundus than the right, as if there were more atresia on the right side of the supposed location of the vagina. A prosthesis with a skin graft taken from the right buttock was applied, with Interceed® placed between the skin and the prosthesis (Figure ). The drawing of the genital tract of the patient generated in the surgery theater is shown in Figure . After 10 days, the prosthesis was removed and the state of the neovagina was assessed. After replacement of the prosthesis, the patient was discharged. The patient returned for a follow-up visit 6 months after the operation, and her evolution was normal. She then underwent transvaginal ultrasound that did not show the ovaries properly but showed the multicystic embryonary remnants in the right hemipelvis. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. Likewise, the Magnetic Resonance Imaging (MR) showed right renal agenesis with probable embryonary remnants of a disgenetic kidney in the right hemipelvis with a multicystic morphology (see Figure ). Those cysts seem to have slightly increased in the transvaginal ultrasound, but four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. |
A 71-year-old female was admitted under the surgical service for elective chemoport placement. She was known diabetic and hypertensive and well controlled on medications. She was also diagnosed with stage II gastric cancer. She underwent distal gastrectomy and Bilroth II reconstruction several months ago and was scheduled for chemotherapy as part of her management.\nJust prior to the procedure the patient developed symptomatic bradycardia manifested as near syncope with her heart rate dropping to 40 beats per minute. Consequently, the procedure was cancelled and the patient was admitted to the medical service for further workup. Cardiology was consulted and she was started on Plavix in addition to the Lovenox she was having for DVT prophylaxis. Workup including transthoracic echocardiography (TTE) was within normal and she was cleared for the procedure. Later Plavix was stopped at the time of the procedure.\nThe patient was taken to operating room the following day for the placement of a chemoport. Her chest X-ray () was normal and she was asymptomatic. General anesthesia was induced with no complications. The patient was draped and positioned in Trendelenberg. Ultrasound guided insertion of a right internal jugular venous (IJV) access was attempted with a total of 4 trials using Seldinger's technique. Resistance was met while threading the guide wire. Ultrasound examination showed a chronic clot burden in the Right IJV. The vascular surgery team was consulted intra operatively. A right IJV access using a microset access under fluoroscopic guidance was obtained. The procedure was then completed without any further issues. Following extubation, and upon transfer of the patient to the stretcher, she started complaining of right-sided chest pain radiating to the back. She became tachycardic with a heart rate ranging between 105 and 110 beats per minute. CXR was immediately obtained and it revealed a contained white out in the right upper lung field ().\nSoon after, the patient decompensated with the systolic blood pressure dropping to 60 mm Hg. Volume resuscitation with crystalloids was started and the patient was cross-matched for transfusion of blood products. A third CXR was obtained during this time revealing the progression of a hemothorax in the right side of the chest ().\nA median sternotomy was performed. Upon exploration of the thorax, a posterior mediastinal hematoma tracking into right pleural cavity was detected. There was active bleeding from the puncture site at IJV-subclavian (SCV) junction. By the end of the surgery, the patient received a total of four units of (PRBC), two units of fresh frozen plasma (FFP), and one unit of platelets. The patient was transferred to the surgical intensive care unit (SICU) postoperatively intubated and hemodynamically stable. A right pleural chest tube was kept in place for drainage. Postoperative CXR was done showing marked improvement of the right-sided hemothorax (). The next morning, the patient was extubated and the mediastinal chest tube was removed. The patient had an unremarkable recovery course and was transferred out of the SICU after removal of the right pleural chest tube. |
A 60-year-old man presented to our clinic for follow-up and reversal of his stoma. He underwent a right extended hemicolectomy with diversion loop ileostomy 1 year back for adenocarcinoma of hepatic flexure and received 4 cycles of FOLFOX (5-Fluorouracil, Leucovorin and Oxaliplatin) subsequently. He stayed fine for a year and then started having rising levels of CEA (carcinoembryonic antigen). His CT and PET scan showed high metabolic uptake in segment VIII of liver. No evidence of local recurrence was found. The case was discussed in tumor board at our institution and it was decided to perform a metastatectomy of his liver lesion along with the reversal of his stoma (, ).\nPer operatively many adhesions were found surrounding the liver and it was a technically difficult resection. Intra operative ultrasound was carried out to localize the lesion and its proximity to major vessels. No other lesions were found. A 7 cm by 5 cm wedge of liver segment VIII was resected with Aquamentis/CUSA. Stoma was reversed and the abdomen was closed. No intraabdominal drains were placed. Post operatively patient stayed fine and was discharged home on 12th Post-operative day after he was orally tolerating and fully mobilized. On discharge no intra-abdominal collection was found on ultrasound.\n7 days later the patient presented in emergency department with severe dyspnea and fever for 1 day. His saturation on room air was 84% and respiratory rate was 34.\nOn examination there were decreased breath sounds on right side of chest with dull percussion note. A Chest Xray revealed massive pleural effusion and tracheal deviation to the left side. Immediate tube thoracostomy was done and 2600 purulent fluid was drained. The patient got better. The next day chest tube drained golden colored fluid. The fluid was sent for bilirubin levels and high levels were found confirming our suspicion of biliothorax. Total leucocyte count was 17,000 and total bilirubin was 4.2. He was started on broad spectrum penicillin – Tazobactam with Piperacillin.\nThe patient kept draining 300 mL, 250 mL of bile per day for 7 days. No abdominal collection was found. An ERCP and sphincterotomy was planned but the sphincter was in spasm and a sphincterotomy could not be performed fully. He was planned for another attempt the next day but post ERCP, the drainage started reducing to 150 mL, and then 100 mL and the patient was discharged home with the tube.\nThe drainage eventually stopped and the chest tube was removed on 23rd day. The reason was probably the fact that the bile leak collected and because of no presence of drain in the abdomen, it made its way in the Pleural cavity through the pleuro – peritoneal canal. Another cause could be a diaphragmatic injury which was not identified per operatively, but there was no pneumothorax to suggest that (). |
A 30-year-old male presented with a painless swelling on the left base of the tongue surface, first noted 3 months back. The mass was slowly progressing in size. There were no other associated symptoms. Clinical examination revealed a nontender swelling on the left posterior part of tongue. The mass was firm in consistency, oval in shape, and had an intact mucosal covering, not associated with ulceration, and was not attached to any underlying tissue. Neck examination revealed palpable ipsilateral submandibular and jugulodigastric lymph nodes. A clinical diagnosis of hemangioma was suspected. CT angiogram revealed a left sided highly vascular lesion. Three months later, the patient came back with symptomatic dysphagia, chewing difficulty, and difficulty in breathing especially while sleeping, and CT scan showed a slight increase in the size of the mass.\nSince the possibility of tongue cancer could not be ruled out in the presence of alarming compressive symptoms, incisional biopsy was planned. During the procedure, a significant bleeding was encountered which was difficult to be controlled with diathermy, local anesthesia, pressure packing and surgicel. Thus, it was decided to abort the procedure after hemostasis was completely secured, and the patient was extubated without any complications. Following incisional biopsy, selective embolization of the left lingual artery achieved a near complete devascularization of the mass. Then the patient was sent back for lip-split, mandibulotomy for access and excision of the left tongue tumor. Suturing on the muscle and in the mucosa was performed with 2-0 Vicryl and 3-0 Vicryl and the defect was closed primarily. Postoperative period was unremarkable.\nThree weeks after excision of the tumor, radiological evaluation was performed using PET/Scan and neck MRI. PET/Scan showed a moderate hypermetabolism at the site of surgical site and at the level of 2A lymph nodes with no evidence of distant metastasis. Neck MRI showed postsurgical changes of the tongue with enhancing left upper cervical lymphadenopathy.\nFollowing the excision of the tumor, the specimen was sent to the Anatomic Pathology Department. Grossly, it was a cauliflower-like ulcerating mass lesion which was 4.8 cm in greatest dimension. Histopathological examination revealed a malignant neoplasm formed of large, polygonal cells with clear to lightly esinophilic cytoplasm and hyperchromatic nuclei arranged in a pseudoalveolar pattern with prominent capillary vasculature. Perineural invasion and extensive lymphovascular invasion were present. PAS-positive, diastase resistant intracytoplasmic granules were present (Figure ).\nImmunohistochemical studies ruled out mimickers (Figure ). Tumor cells were negative for Desmin, SMA, S100, CD34, Myo-D1, Myogenin, PAN CK, Synaptophysin, Chromogranin, CD56, Melan A, and HMB45. A diagnosis of primary lingual Alveolar Soft Part Sarcoma was confirmed. Molecular studies were performed. Interestingly, FISH analysis molecular analysis was negative for TFE3 rearrangement. Therefore, a variant TFE3 fusion type or a fusion involving a related gene such as TFEB were suspected.\nAs the histopathology report showed involved surgical margin, patient agreed to have further resection of the lesion through partial glossectomy. Further excision revealed multiple residual foci of ASPS and a close deep resection margin. Left hemiglossectomy plus reconstruction of the left side of the tongue and the floor of the mouth with radial forearm free flap were performed. Adjuvant radiotherapy was completed 4 months postoperatively. A follow up period of 1 year was unremarkable except for multiple bilateral lung micronodules that remained unchanged on successive evaluation with chest imaging. Patient was stable and fully able to perform daily activities without restriction. |
A 49-year-old male presented to our hospital complaining of chest pain. For the past few years the patient reported experiencing intermittent chest pains at night. In addition to the chest pain, the patient developed pyrexia and perspiration beginning a week before admission. He initially visited another hospital for worsening symptoms where examination found abnormal electrocardiography results (the ST elevation in leads aVL and V1 to V6), leukocytosis, and abnormal liver function tests. Echocardiography revealed a decreased cardiac output with regional wall motion abnormality. Abdominal computed tomography (CT) results showed hepatic parenchymal abnormality and a tumor on the left adrenal gland. The patient was transferred to the reporting hospital with these results.\nSeven years prior to presentation the patient had been diagnosed with hypertension and was put on antihypertensive medication, but remained poorly controlled over the 3 years prior to presentation. He was also diagnosed with diabetes mellitus 8 years ago and has been treated with hypoglycemic agents, but his blood sugar was poorly controlled. There was no other medical history.\nOn admission, his blood pressure was 140/90 mm Hg with a pulse rate of 66 beats per minute, was breathing at a rate of 20 times per minute, and had a body temperature of 36.8℃. He had clear consciousness without signs of conjunctival pallor, scleral jaundice, dehydration, and absent dilatation of the jugular vein. On chest auscultation breath sounds were clear without wheezing or crackles detected. Cardiac auscultation revealed a heartbeat that was regular without murmur. On examining the abdomen, bowel sounds seemed reduced and the right upper abdomen had slight but not significant tenderness. No masses were detected on abdomen. No limb edema was seen.\nTwo days prior to admission, the patient had been tested in another hospital. Blood tests resulted as follows: aspartate aminotransferase (AST) 444 IU/L, alanine aminotransferase (ALT) 469 IU/L, alkaline phosphatase (ALP) 1,153 IU/L, white blood cell count 50,000/µL, hepatitis B surface antigen (HBsAg) negative, anti-HBsAb positive, antihepatitis C virus (anti-HCV) antibody (Ab) negative, antihepatitis A virus (anti-HAV) immunoglobulin M (IgM) Ab negative, and anti-HAV IgG Ab positive. On the day of admission to our hospital, the blood test results were as follows: hemoglobin 15.8 g/dL, white blood cell count 59,360/µL, platelet count 244,000/µL, erythrocyte sedimentation rate 4 mm/hr, and C-reactive protein 3.446 mg/L, blood urea nitrogen 22.7 mg/dL, serum creatinine 0.76 mg/dL, serum calcium 7.2 mg/dL, inorganic phosphorous 2.0 mg/dL, sodium 134 mmol/L, and potassium 4.4 mmol/L. The results also showed that total protein was 5.5 g/dL, albumin was 2.7 g/dL, AST 156 IU/L and ALT 317 IU/L, ALP 289 IU/L, and total bilirubin 1.05 mg/dL. Hemoglobin A1c was 10.7%. At fasting, C-peptide was 0.40 ng/mL (range, 0.48 to 3.3), and 2 hours after a meal it was 0.66 ng/mL. Lactate dehydrogenase was 1,240 IU/L. Creatine kinase MB and troponin-T were found to be 5.99 ng/mL (range, 0.6 to 6.3) and 0.073 ng/mL (range, 0.0 to 0.1), respectively.\nChest X-ray found no abnormal finding. Abdominal CT at another hospital detected several small-sized and low attenuation lesions on both lobes of the liver, and a 5×5-cm-sized tumor enhanced unevenly on the left adrenal gland ().\nElectrocardiography revealed sinus rhythm with T wave inversion and QT interval prolongation observed in leads II and V1 to V6 (). Although the cardiac ejection fraction was 69%, echocardiography detected severely hypokinetic wall motion of mid-left ventricle sparing basal and apical wall motions.\nThe tumor on the left adrenal gland was tested for hormones. The results of 24 hours urinalysis were as follows: vanillylmandelic acid (VMA) 33.93 mg/day (range, 0 to 8.0), metanephrines 20.45 mg/day (range, 0 to 1.3), epinephrine 2,034 µg/day (range, 0 to 40), norepinephrine 978 µg/day (range, 0 to 80), serum epinephrine 1,221 pg/mL (range, 0 to 120), and serum norepinephrine 778 pg/mL (range, 100 to 410). Other tests including aldosterone, plasma renin activity, and cortisol were all normal. On 131I-metaiodobenzylguanidine scan examination, uptake of the radioactive isotope was increased in the left adrenal gland, but no other lesions were found ().\nBased on the examination results above, the patient was diagnosed with a pheochromocytoma on the left adrenal gland. On the 12th day of admission oral phenoxybenzamine administration was started, and blood pressure was followed after having ceased all previous antihypertensives. The dosage of phenoxybenzamine was increased to 35 mg/day. After 19 days of administration, resection of the left adrenal gland was performed under laparoscope. The size of the resected tumor was 5×5 cm, and the surface was a mixture of a hemorrhagic and a cystic lesion. The tissue pathological examination showed the tumor cells with eosinophillic cytoplasm and polygonal shape surrounded by mesenchyma of vasculature on H&E staining, while immunohistochemical stains of chromogranin, synaptophysin, and S-100 immunoperoxidase staining revealed the proper findings to diagnose pheochromocytoma as positive (). Thirteen days after tumor resection a panel of endocrine tests was performed to follow-up on the pheochromocytoma. A 24 hours urinalysis showed a VMA of 6.51 mg/day with metanephrines at 0.61 mg/day, epinephrine at <5 µg/day, norepinephrine at 101.48 µg/day, serum epinephrine at <15 pg/mL, and serum norepinephrine at 536.60 pg/mL (). When first admitted, the patient had uncontrolled blood pressure even with amlodipine 10 mg and irbesartan 150 mg plus 12.5 mg of hydrochlorothiazide. However, when discharged, the patient had controlled blood pressure with a reduced dosage of antihypertensives consisting of amlodipine 10 mg and irbesartan 150 mg.\nPrior to his admission to the outside hospital, the patient had no history of new drug administration that might cause liver function failure. He also had no history of drinking and had a normal body weight (body mass index 23 kg/m2). A panel for hepatic viral disease (hepatitis B virus, HCV, HAV) was negative, and no abnormality was found through immunological testing (antismooth muscle, antinuclear, antimitochondria antibody), or screening for genetic diseases (plasma iron parameters, ceruloplasmin, Cu). This led to the challenge of finding a clear cause for the liver function failure. The liver function improved with conservative treatment, and had resolved by the time phenoxybenzamine was administered (). On the 22nd day after left adrenal gland resection, AST and ALT had normalized at 23 and 25 IU/L, respectively (), and normal liver function test remained within normal limits at 80 days after surgery.\nAfter admission to our hospital, the patient's leukocytosis continued, and follow-up tests were performed to examine for secondary causes such as blood diseases, infection, and pheochromocytoma. On the fourth day of phenoxybenzamine administration the white blood cell count had recovered to 5,900/µL and continued to be within the normal range even after surgery ().\nEchocardiography indicated cardiac ejection fraction of 78% on the 15th day after admission (fourth day of phenoxybenzamine administration), and no left ventricle wall motion abnormalities were observed.\nOn admission the patient was administered insulin and metformin, but the range of blood glucose fluctuation was wide. However, after surgery blood glucose levels returned to the normal range without the previously administered medications. Previously suppressed C-peptide secretion was recovered 3 days after surgery. On the 80th day after surgery, the hemoglobin A1c was measured found to be 5.7% (). |
The patient is a 28-year-old male with a history of intravenous heroin abuse and untreated hepatitis C. He presented with worsening shortness of breath–requiring mechanical ventilation–a hemorrhagic stroke, methicillin-sensitive Staphylococcus aureus bacteremia, and large bilateral pleural effusions. Computed tomography (CT) scan of the chest revealed extensive pulmonary infiltration of the lungs with large loculated complex pleural effusions bilaterally. There was no evidence of pulmonary embolism. Transesophageal echocardiography revealed mitral and tricuspid valve endocarditis. Mainly due to his hemorrhagic stroke, it was decided that his endocarditis would be treated medically. However, due to his complex loculated pleural effusions and significant concern for empyema, it was felt that he would benefit from pulmonary decortication and drainage of his pleural abscesses.\nHe was taken to the operating room for right thoracotomy with decortication, lysis of adhesions, and drainage of intrapleural empyema. He tolerated this procedure well, and his initial postoperative chest X-ray showed a significant improvement in his right-sided effusions []. However, on postoperative day 2, despite two appropriately placed chest tubes, he demonstrated what appeared to be a new hemothorax []. Its etiology was unclear. Due to reluctance to return him to the operating room to evacuate his hemothorax, it was decided to attempt instillation of tPA and pulmozyme, through the chest tube, into his pulmonary space on postoperative day 3. At the bedside, 10 mg of tPA in 50 cc of 0.9% normal saline was combined with 5 mg of pulmozyme inhalation solution for a total of 60 cc, and this was administered into the chest tube through an 18-gauge needle into the self-sealing portion of the chest tube connection system []. The chest tube was then clamped for 1 h to allow the drug to accumulate in the pleural space. Upon unclamping of the chest tube, 730 cc of dark sanguinous fluid immediately drained from the chest tube. Follow-up chest X-ray showed a right pleural effusion that was diminished in size compared to the morning's chest X-ray []. Follow-up hemoglobin remained stable at 7.1 g/dL down from 7.5 g/dL. However, the following morning, his hemoglobin dropped to 6.4 g/dL and he was transfused two units of packed red blood cells.\nA surveillance CT scan of his head was performed to monitor the progression of his stroke which did not show any evidence of progression or new pathology after administration of the tPA.\nHe subsequently required left-sided decortication, tracheostomy, and placement of a percutaneous feeding tube–all of which were performed in an uncomplicated manner. Over the next couple of days, his chest tubes and drains were removed without difficulty, his neurologic status improved to the point in which he was awake, following commands, and moving all extremities, and his ventilator requirements improved to the point where he was transferred to a long-term care facility for further rehabilitation and recovery. Two months postoperatively, he was discharged home with his tracheostomy and feeding tube having both been removed. |
A 65-year-old woman presented with a three-month history of aggravating low back and leg pain. At the first examination, the patient had tenderness over the lower lumbar and tension signs on the lower lumbar nerve. There was hypesthesia on the lateral aspect of both lower legs with no definite motor deficits. Laboratory studies showed a high erythrocyte sedimentation rate of 110 mm/hr (normal 0-15 mm/hr), C-reactive protein level of 9.3 mg/dL (normal 0-0.5 mg/dL) and a mild increase in the levels of liver enzymes and level of normal alkaline phosphatase. Tumor markers checked initially were normal. A plain film of the chest demonstrated evidence of prior-healed tuberculosis. A plain radiograph of the lower lumbar spine showed spondylotic changes without a destructive lesion of the vertebral column (). Advanced radiographic studies were performed with a first impression of spinal stenosis or other infectious condition of the spinal column. Magnetic resonance imaging (MRI) revealed several infiltrative lesions with low signal intensity on the T1 weighted images, and isointensity on a T2 weighted image, mainly at L5 as well as at S1 and L1. The lesions were homogenously enhanced. In addition, at the L4-5 level, severe thecal sac and nerve root compression was observed resulting from the epidural mass anteriorly, hypertrophied ligament and facet posteriorly. However, the discs were relatively preserved (). Computerized tomography (CT) was performed. CT scans of these affected sites revealed a severe spinal stenotic condition without any definite destruction of the vertebral body (). From these clinical and radiological findings, a bone marrow infiltrative neoplasm was suspected; in particular, multiple myeloma or a metastasis. Decompression and a biopsy were performed with the aim of making a final diagnosis as well as palliation of the clinical symptoms. The surgical findings revealed the stenotic conditions to coincide with the preoperative radiographic findings. There was a friable soft epidural mass beneath the posterior longitudinal ligament. However, there was no evidence of infection or an abscess. A culture and biopsy for bacterial and tuberculosis were performed. A histological examination of the epidural mass and vertebral body from L5 showed an epitheloid granuloma with areas of central necrosis and the presence of Langhans giant cells (). A culture of the specimen revealed the presence of Mycobacterium tuberculosis. The patient was treated successfully with postoperative chemotherapy consisting of isoniazid, rifampicin, ethambutol and pyrazinamide. |
A 79-year-old woman presented with a right cervical mass. She had undergone a subtotal thyroidectomy in 1986 for multinodular goiter, and the results of the histopathological studies revealed no malignancy. Twenty-four years after surgery, the patient complained about mass, pain, and swallowing difficulty. The only biochemical remaining sequela was subclinical hypothyroidism. Imaging studies that were performed (ultrasound——and CT scan—) revealed an enlarged right thyroid lobe and a right paratracheal mass displacing (but not invading) the carotid artery, the esophagus, and the trachea. A fine needle aspiration cytology (FNAC) was performed but no conclusion could be given according to the cytology found.\nSurgery was performed, finding a thyroid gland strongly adhered to the carotid sheath and invading the posterolateral side of the esophagus. The recurrent laryngeal nerve was identified and respected. During the surgery, the patient suffered from bradycardia secondary to carotid manipulation, being reverted with atropine.\nThe first postoperative day, the physical exploration revealed right ptosis and miosis, being diagnosed with Horner's syndrome. Hematoma and seroma were ruled out as a cause after a careful exploration, and HSd was attributed to damage to the communication between the cervical sympathetic chain and the recurrent laryngeal nerve, associated to palsy of the right vocal cord (). Final histopathological analysis revealed poorly differentiated insular carcinoma of the thyroid, an infrequent finding usually related to a low survival rate (overall five-year survival rate near 20%). The patient was discharged from the hospital and followed a radioactive iodine protocol, without further complications during the followup. To this date (twenty-four months after surgery), she has not complained from additional cervical masses suggestive of recurrence, although the formerly described ptosis and miosis still remain. A stroboscopy has been performed and shows a palsy of the right vocal chord in median position with mild edema and no signs of atrophy. During closure of the glottis, dynamic exploration revealed hyperkinesia of the left hemilarynx. |
A 70-year-old man was referred for the evaluation of blurred vision and a red and inflamed left eye that began seven days after uncomplicated pterygium excision. Her medical history was significant for type 2 diabetes and arterial hypertension and was negative for eye surgery and systemic autoimmune disease. The patient denied recent ocular trauma, night sweats, fevers, skin rashes, or joint pains. Examination revealed visual acuity of 7/10 in the right eye and hand motion in the left eye. Slit-lamp examination showed large necrosis at the surgical site of pterygium excision with many small and large purulent appearing subconjunctival abscesses with a conjunctival and scleral injection of the left eye (Figure ). There was a slight Tyndall phenomenon in the anterior chamber and moderate corneal edema with a number of blood vessels in the cornea and iridocapsular synechiae. Extraocular motility examination revealed mild limitation of abduction in the left eye with normal adduction. Intraocular pressure was normal. Fundus examination was not possible because of corneal edema. The right eye was in perfect condition. A general physical examination was performed, and there was no evidence of systemic vasculitis or arthritis. Surgical debridement of all the subconjunctival abscesses was realized. A culture specimen of this tissue was obtained and was negative. Associated systemic conditions were investigated with chest X-ray and blood tests, including erythrocyte sedimentation rate (ESR), complete blood count, serum uric acid, syphilis serology, serum antibody screen (rheumatoid factor, antinuclear antibodies, anti-DNA antibodies), circulating immune complexes, all of which were normal, apart from a mildly elevated ESR at 18mm/h. At this stage, the patient was suspected as having scleritis following pterygium surgery without any sound argument for infectious or autoimmune origin. In view of the foregoing and because of the poor prognoses of both presentations, the patient was started on high-dose intravenous steroid therapy: methylprednisolone 1000 mg/d for three days in combination with Ceftriaxone 2 g/day and hourly topical administration of vancomycin-ceftazidime. There was an initial symptomatic improvement but the size of lesions did not increase. Four days later, the patient was commenced on systemic and topical antifungals (voriconazole) with a dramatic resolution of the scleral inflammation and pain in three days. Complete remission of scleral inflammation was observed over six weeks with residual areas of scleral thinning. |
A 49-year-old woman was admitted for a large ventral incisional hernia repair. Medical records showed obesity (BMI: 32 kg/m2), orthotopic liver transplantation for alcoholic cirrhosis (2013) and hysterectomy with bilateral salpingo-oophorectomy for endometrial cancer (2014), and recent consultation for pain secondary to noncomplicated incisional hernia but altering her quality of life. Preoperative assessment for cardiac and pulmonary function was suitable. An open ventral incisional hernia repair was performed through a xyphopubic incision and a polypropylene mesh was used in a sublay fashion. The polypropylene mesh was placed in the plane between posterior rectus muscle sheath and rectus abdominis muscle, as in Rives-Stoppa procedure. She was discharged at the first postoperative day. On the fifth postoperative day she was readmitted for increasing abdominal pain with nausea and vomiting. Computed tomography (CT) scan revealed a defect in the posterior layer of the rectus abdominis muscle sheath, with jejunal loops passing through a gap between the posterior sheath and the mesh, which was fixed to the rectus abdominis muscle (). Surgical treatment was decided and laparoscopic approach was performed by the same surgical team. The small bowel loops were visualized between abdominal wall layers (). Careful soft traction maneuvers were initiated and executed and after the complete reduction of unharmed intestine we observed two defects in the posterior layer of the rectus sheath, with a diameter of 3 and 4 cm. Interstitial space (between polypropylene mesh and posterior sheath of rectus abdominis muscle) was explored verifying correct mesh fixation. The detached posterior sheath was fixated to the abdominal wall with absorbable tackers, reinforcing the rim of the defects with double crown technique (). Finally, the greater omentum was relocated to cover the exposed mesh in order to reduce visceral adhesions. The postoperative course was uneventful and patient was discharged at the second postoperative day. At the six-month follow-up there were no signs of recurrence. |
A 47-year-old male was brought to the Emergency department by ambulance after a high speed motor vehicle accident. On arrival, he had a Glasgow Coma Scale of 13, a patent airway with cervical spine collar in situ, spontaneous tachypnoeic breathing with an oxygen saturation of 97% on 15 l of oxygen. He had unequal breath sounds with decreased air entry on the left. He was tachycardic with a blood pressure of 110/70 mmHg. There were multiple bruises and abrasions to his torso and bilateral lower limbs. He had no previous medical history and was otherwise healthy. Initial chest x-ray was suspicious for a left-sided haemopneumothorax (Fig. ) and an intercostal chest tube (ICCT) was inserted to the left side with a repeat x-ray confirming its position. However, there was minimal drain output and little swing. Subsequent computed tomography (CT) scan of the chest detected a left diaphragm rupture with a large diaphragmatic hernia where most of the left thorax was replaced by intra-abdominal contents (Fig. ). A decision was made for emergency laparotomy to repair the diaphragmatic hernia. The patient was intubated in the emergency department prior to transfer to the operating theatre. While no ventilation problems were noted during transfer, the patient’s abdomen was seen to become progressively more distended to a point where it became tight and tympanic on percussion (Fig. ). An underlying lung injury was suspected and the cardiothoracic team was called. At laparotomy, a significant pneumoperitoneum was confirmed with sudden escape of air upon entering the peritoneal cavity. The large hernia containing spleen, stomach and small bowel was reduced and the posterolateral diaphragmatic defect was repaired with a non-absorbable suture. This was followed by a left thoracotomy that identified a superficial laceration in the left upper lobe of the lung which was repaired with Prolene sutures and re-enforced with tissue glue. The patient was then transferred to the intensive care unit in a stable condition and made a good recovery. It is likely that the lung injury was caused secondary to intercostal catheter insertion during resuscitation in the Emergency Department. However, it could also have been secondary to associated left rib fractures or blunt trauma during the accident. |
A 25-year-old woman with one previous normal spontaneous vaginal delivery was referred to our team for an immediate left breast reconstruction for a phylloides tumour – BIRADS III.\nShe first presented to the hospital with a 9-month history of a left breast lump measuring 6 cm × 7 cm. The patient was 2 months pregnant. The initial biopsy showed a benign proliferative lesion with nuclear atypia. The patient received regular follow-up. It was noted that the swelling increased in size during each follow-up until it involved all four quadrants. An ultrasound examination suggested that the mass was a phylloides tumour – BIRADS III. Despite that fact, the patient refused surgery at that time.\nAfter 2 years of follow-up and counselling, the patient finally agreed to the operation. Clinically, her left breast mass measured 20 cm × 15 cm. She underwent a simple mastectomy and immediate breast reconstruction with a unilateral pedicled TRAM flap [].\nThe TRAM flap was raised with the preservation of the rectus sheath as much as possible by identifying the perforators. The flap was transferred and inset, and the rectus sheath was closed primarily using a loop 0 ethilon. Post-operatively, the patient had an uneventful recovery.\nAt the 2-month follow-up, she was confirmed be 11 weeks pregnant. Both the plastic surgeon and the obstetrician closely monitored her pregnancy. The abdominal wall gradually expanded, according to the stages of pregnancy, without any signs of herniation. At 8 months gestation, a review of her abdominal wall indicated that the integrity was preserved and remarkably, there was little or no change in the width of the donor site scar [Figure and ]. A healthy 2.8 kg baby girl was born at term following an uncomplicated spontaneous vaginal delivery.\nThe postpartum follow-up was uneventful. The patient presented to our clinic 2 months post-delivery with no complaints. She had a well-preserved abdominal wall integrity and function with no signs of herniation []. |
A 62-year-old woman presented with a one-month history of sudden painless visual loss in the right eye. On examination, best corrected visual acuity (BCVA) was 20/20 in both eyes. Intraocular pressure was 21 mmHg in both eyes. Dilated funduscopic examination in the right eye revealed retinal emboli inferior to the optic disc obstructing a small arteriole associated with retinal ischemia (Figures and ). The left eye was unremarkable. There was no prior past ocular history. The patient had hypertension and hypercholesterolemia and had an extensive smoking history.\nFluorescein angiography revealed delayed retinal perfusion along the inferior arcade in the right eye (Figures and ). Optical coherence tomography showed normal foveal contour with inner retinal ischemia and thickening, consistent with an acute inferotemporal branch retinal artery occlusion (). The patient was started on Latanoprost at nighttime in the right eye to lower the intraocular pressure in hopes to increase reperfusion of the retina. An extensive cardiovascular workup was done, and significant carotid artery stenosis of less than 70% was found. She was started on plavix and aspirin by her cardiologist. The patient continued with BCVA 20/20 OU vision and persistent highly refractile peripapillary emboli; retinal ischemia resolved. Her OCT showed inner retinal atrophy (Figures and ).\nUnexpectedly, the patient presented with a three-day history of sudden painless visual loss OD a year and a half after her initial presentation. BCVA was counting fingers (CF) in the right eye. Fundus exam revealed new superotemporal retinal ischemia associated with two new emboli. OCT demonstrated thickening and hyperreflectivity of the inner retinal layers consistent with an acute BRAO OD (). The patient refused fluorescein angiography at that time. Urgent workup revealed worsening stenosis to 80% in her right common carotid artery. The vascular surgery referral prompted a right carotid endarterectomy with a carotid stent placement approximately two months following her presentation with acute vision loss.\nAfter endarterectomy, vision in the right eye improved from counting fingers to 20/200 and 20/250 at 2 months and 6 months, respectively. Postoperatively, retinal whitening resolved and reduced intraretinal edema was noticed (). One year after endarterectomy, visual acuity was 20/30. The retinal swelling had resolved (). |
A 49-year-old Caucasian lady with history of polysubstance use disorder and related complications including, recurrent cutaneous abscesses, spinal diskitis and septic thrombophlebitis presented to the emergency room with complaints of intermittent fevers and right hip pain. A month prior to the presentation, she had left another hospital against medical advice after being diagnosed with Methicillin-resistant Staphylococcus aureus bacteremia and right hip septic arthritis. Post discharge, she was off antibiotics, but continued heroin and methamphetamine and was taking multiple dose of ibuprofen in addition for pain control. On admission, her physical exam was notable for severe tenderness in her right hip, marked bilateral lower extremity edema and multiple deep, circular, punched-out looking atrophic scars involving all extremities at prior skin popping (subcutaneous injection of illicit drugs) sites. Pertinent laboratory data included chronic anemia with a hemoglobin of 9.8 g/dL, WBC count of 10.23 k/uL and a platelet count of 395 k/uL. She had negative HIV, Hepatitis B antibodies and elevated Hepatitis C antibody with undetectable Hepatitis C viral load. Her basic metabolic profile noted a sodium of 140 mmol/L, potassium of 3.5 mmol/L and a creatinine of 2.9 mg/dL (estimated glomerular filtration rate of 17 ml/min) which was a significant rise from the normal creatinine levels a month earlier. Her urine analysis noted >500mg/dL proteinuria with a protein/creatinine ratio of 28.25. She had no monoclonal proteins on serum or urine electrophoresis. CT scan of her right hip noted marked degenerative changes in the right hip, with right acetabular protrusion and cortical disruption of the medial acetabular wall. She was diagnosed with right hip osteomyelitis and was in acute renal failure with evidence of nephrotic range proteinuria. She was placed on antibiotics (daptomycin) and underwent hip arthroscopy with irrigation and debridement along with lavage shortly after admission. Differential diagnoses considered for her renal disease included acute tubular necrosis due to sepsis, post infectious glomerulonephritis, focal segmental glomerulosclerosis associated with heroin use, acute interstitial nephritis from NSAIDs and membranoproliferative glomerulonephritis associated with Hepatitis C. She underwent a renal biopsy which revealed acute tubular necrosis and secondary (AA) amyloidosis with the classic apple green birefringence when stained with Congo red () and positive immunohistochemical stain for serum amyloid A protein (). Two weeks after admission she underwent Girdlestone arthroplasty. During the hospital stay, she developed intermittent hypotension, had evidence of primary adrenal insufficiency attributed to amyloidosis and was initiated on steroids. She was discharged home after completion of prolonged antibiotic therapy with daptomycin and was maintained on oral doxycycline. She was referred to outpatient opioid treatment program. Eight months after her admission, she remained committed to be off all illicit drugs and underwent right total hip replacement. Her creatine levels normalized (estimated GFR of 82 ml/min) but she continued to have proteinuria from renal amyloidosis (protein/creatinine ratio of 28.25) and required diuretic therapy for symptomatic management of her edema. |
A 56-year-old woman with a history of breast cancer was referred to the Department of Investigational Cancer Therapeutics in January 2015. She had no pertinent medical history and was in good health until early 2005, when she felt a mass in her left breast. She was a non-smoker and had a family history of breast cancer and prostate cancer. Physical examination demonstrated a 3 × 3 cm mass in the 10 to 11-o’clock position in the superior aspect of the left breast and a 2 cm lymph node in the left axilla. Ultrasound-guided core needle biopsy performed in February 2005 demonstrated an estrogen receptor- and progesterone receptor-positive, human epidermal growth factor receptor 2 (HER-2/neu)-negative invasive mammary carcinoma of the left breast [modified Black’s nuclear grade 1 (well differentiated)], carcinoma in situ, low grade, solid type, without necrosis). CT scans indicated no evidence of metastasis; however, fine-needle aspiration of the left axilla lymph nodes demonstrated metastatic carcinoma consistent with the primary breast tumor.\nIn March 2005, the patient was enrolled on a clinical trial that included weekly paclitaxel chemotherapy for 12 cycles followed by six cycles of 5-fluorouracil, epirubicin, and cyclophosphamide. Four months later, imaging studies indicated a reduction in tumor size. In September 2005, she underwent a left skin-sparing total mastectomy with axillary lymph node dissection followed by delayed reconstruction using a submuscular tissue expander. Then, from October to December 2005, she underwent post-operative adjuvant radiation therapy (50 Gy in 25 fractions to the left central chest and left lateral chest wall with an additional 10 Gy boost to the tumor bed). She also intermittently received Tamoxifen between 2005 and 2008. In December 2006, imagining studies demonstrated bone metastasis and the patient was treated with radiation therapy and anastrozole plus fulvestrant from December 2006 to March 2014. The bone disease stabilized, but she had disease progression in her lungs and lymph nodes. Although her tumor did not harbor an alteration in the PI3K/Akt/mTOR pathway, she was treated with the standard-of-care exemestane and everolimus from March 2014 to January 2015.\nIn January 2015, CT scans demonstrated increasing thoracic lymphadenopathy and pulmonary metastatic disease, and a nuclear medicine bone scan demonstrated bone metastasis involving the left ilium, acetabulum, and ischium. The patient was referred to the Department of Investigational Cancer Therapeutics in January 2015 and underwent biopsy of a lung tumor. Molecular profiling of the tumor demonstrated the following genomic alterations: ATM serine/threonine kinase (ATM) loss in exons 57 to 63, notch receptor 2 (NOTCH2) A3F, estrogen receptor 1 (ESR1) Y537S, and amplification of the following genes: cyclin D1 (CCND1), myeloid cell leukemia 1 (MCL1), BRCA2 interacting transcriptional repressor (EMSY), fibroblast growth factor 19 (FGF19), fibroblast growth factor 3 (FGF3), and fibroblast growth factor 4 (FGF4) (). Early assessments suggested that preclinical models with increased cyclin D1 or cyclin-CDK-Rb pathway activation may have increased sensitivity to CDK4/6 inhibitors. However, the patient did not receive a CDK4/6 inhibitor because clinical trials were not available at that time. She was presented at the multidisciplinary conference for optimization of treatment selection. On the basis of tumor molecular profiling demonstrating multiple mutations in FGF (FGF19, FGF3, and FGF4), the patient was offered an investigational therapy comprising FGFR inhibitor, but she pursued treatment with eribulin; after four cycles she developed disease progression. Subsequently, she was treated with 11 cycles of fulvestrant and palbociclib and was taken off study owing to progressive disease in August 2017.\nOn the basis of her tumor’s NOTCH2 A3F alteration, in October 2017, the patient was enrolled on a clinical trial consisting of a NOTCH inhibitor, cisplatin, and gemcitabine. Her best RECIST response was PR per RECIST 1.1 criteria on cycle 12, day 19. She received 22 cycles of the investigational drug combination for 19 months and was taken off study owing to disease progression. Adverse events included impaired hearing and thrombocytopenia.\nThe patient underwent genetic counseling, and testing for germline BRCA1 and BRCA2 mutations was negative. CtDNA analysis in October 2018 demonstrated multiple somatic mutations which included a breast cancer type 1 (BRCA1) nonsense mutation on exon 10 (). On the basis of these findings, the patient was enrolled on a clinical trial with a PARP inhibitor. Her best RECIST response was SD with a 14% decrease in tumor measurement from baseline per RECIST 1.1, and the PFS duration was 9.1 months ( and ). Imaging studies in January 2020 demonstrated evidence of progressive disease and she was taken off study. Subsequently, she received radiotherapy and doxorubicin. At the time of this report, the patient was still alive, and the overall survival duration was 14.8+ months (). |
The patient discussed in this case is a 66-year-old female with an unknown past medical history since the patient had not been seen by a physician for approximately 43 years. The patient was transferred to our university hospital from a community hospital for management of chest pressure, shortness of breath, and vaginal bleeding. The patient presented with a one-week history of chest pressure. This was described as a feeling of tightness which occurred several times over the span of one week and was not associated with exertion. Her shortness of breath had been progressively worsening over one month. Prior, to one month ago, she reportedly was able to ambulate approximately 100 yards without difficulty. By the time of her presentation, the patient stated that she was only able to ambulate approximately 20 yards due to shortness of breath on exertion. The patient stated that her vaginal bleeding was similar in volume as compared to previous menstrual cycles and had been occurring approximately two times per week. Her past surgical history was significant for hiatal hernia repair and tonsillectomy, both during adolescence. Furthermore, she smoked once pack of cigarettes daily for fifteen years and has quit two months prior to admission. She had two aunts who passed away due to myocardial infarctions at unspecified ages. On review of systems, the patient reported decreased appetite, fatigue, diaphoresis, cough, shortness of breath, chest pressure, nausea, vomiting, dysuria, hematuria, vaginal bleeding, pallor, light-headedness, and a 20-pound weight loss over several months. Physical exam at time of admission was unremarkable.\nIn the Emergency Department, the patient underwent an EKG which demonstrated Q waves in inferior leads. Her troponin T was 0.61 ng/mL. Subsequent troponin T levels were measured, and within 24 hours, this increased to 5.88 ng/mL. Therefore, the patient was diagnosed with a non-ST elevation myocardial infarction and cardiology was consulted.\nThe patient was found to have a hemoglobin level of 4.5 g/dL and hematocrit level of 18.3%. It was presumed that this anemia was secondary to the patient's reported vaginal bleeding. Anticoagulation for NSTEMI was therefore not recommended. On further evaluation, the patient was found to have an elevated white blood cell count of 39,900 cells/mm3, elevated uric acid of 8.2 mg/dL, hyperkalemia as high as 5.6 mmol/L, hyperphosphatemia as high as 4.7 mg/dL, and an elevated creatinine of 2 mg/dL. Thus, she was suspected to have tumor lysis syndrome given these multiple electrolyte aberrancies. The patient was transfused with two units of packed red blood cells and rasburicase was administered.\nA transvaginal ultrasound was performed which demonstrated a prominent cervical mass compressing the left ureter and associated hydronephrosis. Given the acute kidney injury as diagnosed due to a rising creatinine to 2 mg/dL, nephrostomy tube insertion was initially recommended but not placed due to the patient's poor prognostic factors. She was neither anuric nor oliguric. The patient underwent cervical biopsy and was found to have invasive squamous cell carcinoma of the cervix. The cervical cancer had eroded a 1.5 cm hole into the bladder extending down the anterior vaginal wall approximately at the introitus, according to the physical examination performed by the Gynecology team.\nDue to NSTEMI, transthoracic echocardiogram was performed demonstrating an ejection fraction of 30-35%, increased PA systolic pressure, grade III diastolic dysfunction, and mitral and tricuspid regurgitation. Cardiac catheterization was performed one day after admission demonstrating a total occlusion of left anterior descending and distal left circumflex arteries. Lack of collateral vessel perfusion suggests that occlusion was not attributed to chronic atherosclerotic changes and more likely due to acute blockage. In addition to initiating a continuous heparin infusion at this time, the patient was offered revascularization but declined. Risk of sudden death was thoroughly explained but the patient was not experiencing chest pain and wanted to focus on managing her vaginal bleeding rather than revascularization.\nThe patient proceeded to have a sustained elevated heart rate but decreased blood pressure likely due to cardiogenic shock and atrial fibrillation with rapid ventricular response. Heparin was discontinued, and digoxin was initiated.\nOn day seven, she demonstrated ventricular tachycardia with ambulation, losing consciousness and subsequently going into asystole. ACLS was initiated, the patient was defibrillated and resuscitated, and the patient was intubated and supported on vasopressors. A family meeting was held, and it was decided that comfort care measures would be pursued. The patient died five minutes after extubation.\nAfter the patient's demise, pathological results were finalized. Based on the surgical pathology report of the cervical mass, moderately differentiated invasive squamous carcinoma with extensive necrosis was seen ().\nHemopathology report described that flow cytometry analysis demonstrated a clonal B cell population most consistent with CLL/SLL. Peripheral smear confirmed this diagnosis (). FISH study demonstrated deletion of 11q and interstitial deletion of 13q (67-73% of cells). Deletions of 11q, as seen in this case, are observed in approximately 8-19% of CLL cases []. Furthermore, deletions of 13q, again seen in this case, are demonstrated in approximately 68% of CLL cases []. Deletions are a poor prognostic marker thus suggesting evolution of the cell line in this patient. Monosomies 11, 13, and 14 were present in 3-7.5% of the cells, but there was no evidence of trisomy 12, deletion of 17p, or t (11; 14). |
The patient is a 60-year-old woman who presented to our hospital with new-onset upper back pain. The patient complained of upper back pain for 6 months accompanied by left intercostal neuralgia, which was refractory to analgesic treatment. The patient underwent gross total resection of a meningeal tumor 10 years ago. The pathology result was a hemangiopericytoma with a high proliferation index that was classified as WHO grade 2. The patient received twenty-seven cycles of adjuvant radiotherapy after surgical resection. Then, the patient had an excellent post-operative course, and yearly follow-up computed tomography (CT) scans confirmed a complete resection and no recurrence of the tumor until March 2017. Ten years after resection of the meningeal tumor, her upper back pain started. First of all, the patient admitted to another hospital, the positron emission tomography/computed-tomography (PET-CT) showed the lesion at the left T3 vertebral body and in the right lobe of the liver without other sites metastasis. The diagnosis of metastatic SFT was suggested by immunohistochemistry of the T3 mass after CT-guided percutaneous thoracic puncture biopsy was performed. The immunohistochemical study demonstrated CD34(+) and STAT6(++). Then, abdominal and thoracic magnetic resonance imaging (MRI) were performed. The abdominal MRI revealed an enhanced lesion in the right lobe of the liver, with a size of ~2.7 × 2.8 × 3 cm (). The patient complained of no discomfort in the abdomen and did not present with jaundice. The thoracic MRI showed an expansive intraosseous mass at the left T3 vertebral body. The bony mass expanded along the left of the T3 vertebral body, with retropulsion toward the spinal canal and compression of the spinal cord at this level (). The thoracic CT scans showed that the size of the lesion to the left of the T3 vertebral body was ~3.7 × 5.3 cm (). The Tomita score was 4, and the Tokuhashi score was 9–11, indicating long-term control requiring wide or marginal excision. On the basis of these findings, the surgical plan included marginal excision of the T3 tumor with T2-4 pedicular screw fixation (). The surgery was performed smoothly without complications or significant blood loss. The preoperative symptom of back pain was relieved, and a radiograph of the thoracic spine showed proper fixation. Then, the diagnosis of SFT was suggested by immunohistochemistry performed on the spinal mass. The immunohistochemical study demonstrated CD34(+) and STAT6(+) (). The patient had an excellent post-operative recovery. After 3 months, a second abdominal MRI showed that the mass in the right lobe of the liver grew larger, and the size was ~3 × 3.5 × 3.5 cm (). A right hemi-hepatectomy was performed to remove the liver lesion by gross total resection. The diagnosis of SFT was confirmed with the expression of CD34 and nuclear expression of STAT6 by immunohistochemistry performed on the liver mass (). As the prognosis of malignant and metastatic SFTs was poor, we suggested the patient underwent the adjuvant radiotherapy after complete resection of thoracic and liver metastasis SFT. But the patient refused adjuvant radiotherapy and carried out post-operative follow-up on time. Two-year follow-up thoracic MRI and abdominal CT revealed no disease recurrence at the T3 () and the liver (). |
A 50-year-old female patient presented with a complaint of painful swelling in the left upper jaw that was present since 8 months. History revealed that she had undergone extraction of her mobile and painful left maxillary third molar 2 months back. Later, she noticed a small painful swelling that gradually increased to reach the present size.\nIntraoral examination revealed a diffuse ulceroproliferative growth on the left alveolar mucosa in the third molar region of size approximately 4 cm × 3 cm extending anteroposteriorly from distal aspect of the 25 to maxillary tuberosity and mediolaterally 4 mm away from the midline of the palate and laterally encroaching the vestibule []. Mucosa over the lesion appeared erythematous with yellowish slough. The swelling was associated with pain and discomfort on mastication and deglutition.\nIntraoral periapical radiograph irt 25, 26, 27 shows well-defined radiolucency with irregular border and on the alveolar ridge irt 26, 27 shows diffuse rarefactions surrounding the bone []. Maxillary occlusal radiograph shows a well-defined radiolucent bony defect in the left posterior region of palate in relation to 26 and 27 []. Spiral computed tomography showed a soft density lesion measuring 5 cm × 3.5 cm involving alveolar process of the left maxilla and extending into the adjacent pharyngeal mucosal space [].\nAn incisional biopsy was done. Microscopic examination showed round cells with large nucleus, a thin rim of eosinophilic cytoplasm with prominent nucleoli and few cells with clear cytoplasm arranged in pseudo alveolar pattern with central discohesive floating cells [Figure and ]. The cytoplasm of tumor cells showed positive staining with phosphotungstic acid hematoxylin (PTAH) and Masson trichrome [Figure and ]. Few strap cells showed PTAH positive staining with no evidence of cross striations []. For confirmatory diagnosis panel of immunohistochemical markers was done and the tumor cells showed strong positivity for vimentin [] and Myo-D [] and were negative for epithelial membrane antigen [] and S-100, []. Correlating the clinical, radiographical, histopathological and immunohistochemical findings the case was diagnosed as alveolar RMS (ARMS). After final diagnosis, the patient was referred to higher cancer center for further appropriate treatment. |
A six-hour-old male newborn was referred to our eye clinic from a private hospital in which he was born for the management of the eversion of both upper eyelids. He was the sixth child to a 29-year-old woman. A history of malaria in the third trimester was reported. This was managed accordingly. Pregnancy was regularly followed up. Delivery was at term. Labour and delivery were both uneventful.\nExamination revealed bilaterally everted upper eyelids with severe chemosis (). Examination after instillation of a topical anesthetic agent and retraction of the upper eyelids with Desmarres retractors revealed grossly normal eyeballs with negative fluorescein staining of the cornea in an otherwise healthy looking baby.\nThe baby was admitted and managed as follows: application of neomycin and polymyxin eye ointment and padding of the prolapsed conjunctiva with gauze dressings soaked in 5% hypertonic saline once daily. Daily eye cleansing was performed to keep the external eye clean of any discharge.\nOn the second day, there was reduction in the size of the chemosis (). Treatment was continued. On the third day, the chemosis fully resolved and the lids reverted back to normal position () but everted mildly at the margin when the baby cried. The baby developed fever with a temperature of 39°C. Neonatal sepsis was suspected and the baby referred to the neonatal unit of another hospital with an available ophthalmic unit.\nPaediatrician examination revealed no morphological abnormality. C reactive protein was positive at 12 mg/L. Full blood count showed white cell count in the normal range but the differential count revealed granulocytosis. Urine and cerebrospinal fluid cultures were negative. The baby was admitted and treated for neonatal sepsis with intravenous Ampicillin, Netilmicin, and Cefotaxime for 8 days. While being admitted, the eyes were no longer padded and 5% hypertonic saline eye drops were administered four times daily; antibiotic ointment was used twice daily. Fever subsided on the 5th day.\nAt 3-week followup, the child could open the eyes spontaneously (). Ocular examination revealed clear corneas; anterior chambers of normal depth; round, central, and reactive pupils; and normal fundi. |
A 77-year-old female patient who had right tympanomastoidectomy operations twice in another hospital with the diagnosis of chronic otitis media with cholesteatoma was admitted to our clinic with a complaint of discharge in the right ear. The right external auditory canal was filled with a cholesteatomatous mass. On diffusion-weighted temporal bone MRI, soft tissue images showing peripheral contrast enhancement were seen in the right mastoid cells, mastoid antrum, and middle ear. There was no restriction in diffusion-weighted imaging of this lesion, and thus, cholesteatoma was not considered as a diagnosis (Figures and ). The patient's temporal bone CT images were reported as soft tissue obliterating the right external ear canal, showing extension to the middle ear cavity, causing destruction in the ossicular chain, mastoid antrum, tegmen tympani, and mastoideum (). No sinonasal pathology was found in the patient's history. Examination and imaging of the sinonasal region showed no additional pathology (). There was advanced sensorineural hearing loss on audiologic evaluation. The patient reported having had hearing loss for many years. In the left ear, both bone conduction and air conduction were 37 dB. Canal wall-down tympanomastoidectomy was performed under general anesthesia. All pathological tissues were cleaned, and because there were no perioperative or postoperative problems, the patient was discharged on postoperative day 2 and asked to return for a routine follow-up visit. The histopathological diagnosis was granulation tissue.\nAbout 6 months after the operation, the patient came back because of recurrence of ear discharge. There was a cholesteatomatous mass observed by otomicroscopic evaluation. Revision surgery was performed, and partial petrosectomy and cavity obliteration of the fatty tissue were done. Histopathologic evaluation of the cleaned tissues was reported as cholesteatoma.\nTwo months after the operation, the patient was admitted to the clinic with a vegetative mass in the postauricular scar area. She also had grade 5 facial paralysis according to the House–Brackmann classification on the right side. The patient was hospitalized again, and revision surgery was performed under general anesthesia. There was an ulcerovegetan mass filling the mastoid area () and eroding bone on the horizontal and vertical facial canal. It was observed that the pathology proceeded to the medial line of the petrous bone. Since the dura was completely exposed, the neurosurgery team was included in the operation. All lesions were cleared, and total petrosectomy was performed. The damaged dura was repaired. The operation was terminated by obliterating the cavity containing the fat tissue. Pathologic evaluation of the specimen was reported as “IP-high grade squamous dysplasia” (). The patient received a dose of 16 gy in 8 fractions. Radiotherapy could not be continued because of deterioration of the patient's general medical condition. General health problems worsened, and 4 months after the last operation, the patient passed away due to multiple organ failure. |
The patient was a man aged 43 years who had undergone a heart bypass surgery in 2009. He was reported to have developed initial symptoms of sore throat and cough on 27 January 2015. He consulted local city hospital 3 days later and Cefotaxime (3 g) was given intravenously at the Outpatient Department (OPD) of the hospital. However, his symptoms were not alleviated, and he was admitted into a provincial hospital on 3 February.\nA chest radiograph showed multi-node and patchy consolidation in the lower and middle lobe of both lungs on 4 February (), with rapid progression of ground-glass opacities and consolidation in both lungs on 6 February ().\nThe blood biochemistry tests are summarized in . The results show that the patient’s white blood cell count decreased during the early stages, which returned to normal levels after treatment, and abnormally increased in the later stages. Neutrophil count exhibited a trend similar to the white blood cell count. Lymphocyte count was consistently lower than normal. Coagulation index detected that D-dimer remained abnormally high, and the platelet count remained normal. Activated partial thromboplastin time and thrombin time were prolonged in the whole process. Arterial blood gas analysis showed that PO2, PCO2, , and SO2 levels remained below normal in all test. After testing for blood electrolytes, K+ levels were found to be normal while Na+ levels had persistently decreased. Liver and kidney were dysfunctional, as indicated by the increased levels of aspartate aminotransferase, creatinine, and blood urea nitrogen.\nThe patient’s condition persistently deteriorated and the patient developed shortness of breath, weakness, poor appetite, coma, and anuria because of progression to severe pneumonia with pleural effusion, and multiple organ dysfunction syndrome (MODS). He died of acute respiratory distress syndrome (ARDS) and MODS on 6 February despite the daily administration of broad-spectrum antibiotics (Meropenem 1 g, Vancomycin 1 g, and Tigecycline 300 mg by intravenously), antiviral drugs (Ganciclovir 450 mg by intravenously and Oseltamivir 150 mg by orally), cocorticoids (Meprednisone 80 mg by intravenously), and with mechanical ventilation at the provincial hospital.\nTranstracheal aspirate sample of the patient was collected on 4 February and H5N6 infection was confirmed by China National Center for Disease Control and Prevention (CDC) using real-time reverse transcription polymerase chain reaction (real-time RT-PCR). Tests for other respiratory agent, such as Middle East respiratory syndrome coronavirus (MERS-Cov), severe acute respiratory syndrome coronavirus (SARS-Cov), and other subtype influenza virus, were totally negative. Later on, the virus was isolated from embryonated chicken eggs. Homologous comparison in GenBank with BLAST showed the viral HA and NA genes are closely related to A/chicken/Shenzhen/552/2013(H5N6), and the HA had a multiple amino acid sequence, “LRERRRKRG,” at cleavage site, belonging to 2.3.4.4 clade (phylogenetic tree was declined to reproduce here by China national CDC). The six internal genes were from the avian influenza virus H9N2 subtypes.\nEpidemiological investigation showed that 2 days before the symptoms presented, the case had contact with wild birds, which were hunted from local city wetland. In addition, he had not contacted with live poultry or individuals with fever or influenza-like illness during 2 weeks before illness onset. The patient’s close contacts, totaling 117 persons, were all healthy following medical observation for 7 days after their last exposure to the case. Environment investigation showed that unusually large amount of death of wild birds or poultry was not found in local place during last 1 month. On 10 February, local CDC collected 13 wild bird feces samples from the wetland and 33 samples, including poultry feces, poultry cage surface swab, chicken lung tissue, and market sewage from local live poultry markets. Results showed no H5-positive sample from wetland, while 63% (21/33) of the samples from local live poultry markets were H5 positive.\nEthical approval for this case report was granted by the Ethics Committee of Yunnan Provincial Center for Disease Control and Prevention, China. Written consent was obtained from the patient’s wife for publication of information and photographs. |
We report a case of a 6-month old girl referred to the Nairobi hospital with a massive, progressively growing congenital mass in the oral cavity. A thorough medical history revealed no co-morbidities with all vital signs within normal limits. Upon physical examination, the patient was found to have difficulty in breathing, difficulty feeding and inability to close the mouth. Intra oral examination revealed a large swelling involving more than two-thirds of the tongue, most of which lay outside the oral cavity (Figure ). The lesion was a deep red-purple color, soft on palpation, non-tender and normal in temperature with no appreciable thrills but blanched on application of pressure. Examination of the surrounding structures was unremarkable.\nMagnetic Resonance Imaging (MRI) with gadolinium revealed a well-circumscribed soft tissue mass measuring 3.9 cm × 4.1 cm × 5.8 cm. It involved most of the tongue except for the base and extended to both lateral borders, while inferiorly, involved the entire thickness of the tongue to its ventral surface. The mass was hypointense on T1-weighting and was found to be hyperintense on T2- weighting. Multiple low signal foci were noted which were compatible with flow voids (Figure ). Digital subtraction angiography revealed the feeder vessels to be anomalous branches of the lingual arteries. These findings, coupled with blood collection in the interior of the lesion, informed the diagnostic hypothesis of a congenital lingual hemangioma.\nThe lesion was monitored closely over a period of 1 year with the expectation of improvement by involution, however, the lesion showed no signs of reducing in size and in fact enlarged over this duration (Figure ). After a multidisciplinary discussion, immediate embolization of the feeder vessels and subsequent surgical resection of the lesion was proposed in order to improve the quality of life for the patient and prevent fatal airway obstruction. Embolization of the lingual arteries (LA’s) was performed through a transfemoral approach. Using the bilateral lingual arteriograms, the feeder vessels from the right and left LA’s were selectively embolized as close to the lesion as possible using absorbable gelatin sponge particles (Gelfoam, 100–200 μm) under fluoroscopic guidance. Post-embolization angiograms of the LA’s were performed to confirm if all feeder vessels had been occluded (Figure ).\nOne month post embolization, a color change from deep red/purple to a lighter shade was noted and the lesion seemed to have reduced slightly in size as shown by its surface having mucosal folds as opposed to appearing stretched and shiny prior to embolization (Figure ). Despite this, feeding and airway management were still a major problem for the patient. A month later, naso-tracheal intubation under endoscopic guidance was performed and a modified key hole surgical technique was used to reduce the bulk of the lesion (Figure ). A full thickness elliptical wedge incision was made on the dorsum of the tongue using electrocautery, accompanied by two diverging incisions on the anterior portion of the tongue. Hemorrhage was controlled by compression of the posterior aspect of the tongue using moist gauze after which excess tissue from the central and anterior portion of the tongue was excised. Suturing was done in various planes using vicryl 3.0 after attaining the best fitting of the tongue into the oral cavity. In the immediate post-operative period, there was slight swelling of the tongue which resolved in a few days.\nPostoperative hospital stay was uneventful with no medical or surgical complications. The patient was fed via a nasogastric tube for a period of 5 days after which it was removed and oral feeding begun. The patient was discharged on the 8th day after surgery. Histology of the tissue specimen revealed the diagnosis to be a capillary hemangioma (Figure ). At the final follow-up visit, the tongue had healed entirely and could fit within the mouth comfortably. The patient showed marked improvement in speech and swallowing functionality with no other complaints (Figures and ). |
A 32-year-old man was admitted to our clinic with ear discharge, pain, and hearing loss. From otoscopy, a mass was identified, covered by skin and protruding from the postero-inferior wall of the entrance to the external auditory canal. The passage of the canal was significantly narrowed by the tumor, and the tympanic membrane was not visible. After 1 week of topical treatment (ciprofloxacin, dexamethasone), the patient’s symptoms and clinical findings were observed to improve. Audiological tests and a computed tomography (CT) scan were performed. The pure tone audiogram indicated conductive hearing loss with a mean air-bone gap of 25 dB. The CT scan showed a soft tissue density mass protruding from the postero-inferior wall of the entrance to the external auditory canal and obstructing the canal with no invasion of other tissues (). Fine-needle aspiration biopsy was performed; however, an insufficient number of specialized cells were present in the pathological samples to permit diagnosis.\nOne month after the appearance of symptoms, surgical excision was scheduled under local anesthesia. Under microscopic view, after transcanal lateral circumferential skin incision, a skin flap was elevated and the encapsulated tumor with a diameter of approximately 20 mm was seen and excised en bloc. The skin flap was replaced to cover the surgical area and two Merocel® ear packs were placed in the external ear canal. After surgery, the patient was prescribed analgesics (paracetamol, 3 × 500 mg/day) for 7 days.\nThe tumor was off-white in color with the dimensions of 20 × 12 × 15 mm3. Microscopic examination showed glandular structures which were composed of two cell layers: epithelial and myoepithelial. The inner layer consisted of middle-sized cylindrical or cuboidal cells. Abundant eosinophilic cytoplasm, arranged in a columnar to cuboidal shape, was seen in the luminal cells. The outer layer consisted of spindle cells with elongated nuclei. Neither atypia nor mitotic activity was observed. All of the microscopic features were indicative of CA () and the surgical margin was clear.\nThe patient has been tumor free for 12 months postoperatively (), with no hearing loss or symptoms of external otitis in this period. Written informed consent was obtained from the patient. |
A 47-year-old African American male presented to the hospital for worsening bilateral lower extremity pain and blisters for 1 week. It started gradually, progressing to 7/10 intensity over the previous three months. It was episodic, triggered by heat, lasted from two to ten hours and relieved by external cooling. It radiated from his ankles to toes bilaterally and was associated with warmth, redness, and swelling. The patient had developed significant functional impairment including inability to run/walk long distances as these activities triggered his symptoms. He had been immersing his feet in cold water and cooling them in front of the air conditioning for 4-6 hours a day to alleviate his symptoms. One week prior to presentation, he had developed painful blisters on both feet expressing serous fluid. He denied fevers, chills, trauma, purulent discharge, bleeding, similar previous episodes or family history of a similar rash. His primary care physician prescribed gabapentin which did not relieve his symptoms. The patient had no complaints of the upper extremities or digits. On presentation, vital signs were stable, patient was afebrile. The physical examination was unremarkable except for edema, erythema, and tenderness to palpation on light touch on both feet and ankle. No motor and sensory changes were noticed. Pulses were palpable bilaterally. .\nDifferential diagnosis included acute infection i.e. cellulitis secondary to repeated cold water immersions and dry air cooling as well as secondary erythromelalgia from autoimmune disorders, sexually transmitted infections and myeloproliferative syndromes. Consequently, Rheumatology service was consulted, and recommended work-up for secondary causes of EM. Vascular surgery evaluation found no need for urgent intervention as acute infection and peripheral vascular disease seemed unlikely.\nBased on the clinical picture, hemodynamics and unremarkable lab results, acute infection was ruled out. His chronic symptoms required outpatient follow up with rheumatology for long term management of erythromelalgia. He was advised to continue pain management as prescribed by his PCP until his follow up rheumatology appointment to discuss his lab results.\nHowever, the patient returned to the ED one week after with worsening symptoms. His vital signs were stable, physical exam showed that moderate erythema progressed from bilateral feet up to the lower third of anterior shins, edema with multiple scattered blisters in different stages of healing along with macerated scales, and pitted keratolysis on bilateral plantar surfaces. He had intact sensation and complained of tenderness to palpation.\nRepeat labs were unremarkable except for an elevated CRP. The rheumatological work up sent out on previous admission showed weakly positive ANA titer at 1:40 and a positive lupus anticoagulant.\nGiven the cellulitis-like picture of the lower extremities, he was admitted and started on IV vancomycin. Dermatology was consulted for further recommendations. Imaging was ordered for confirmation of the diagnosis. , and .\nThe patient was managed by a multidisciplinary team composed by medicine, dermatology, rheumatology, and podiatry. Based on the clinical findings, rheumatological lab results and imaging, the patient was diagnosed with primary erythromelalgia with secondary bacterial (cellulitis) and fungal (tinea pedis) superinfection and trench foot from prolonged cold-water immersions. Protein electrophoresis was also done to rule out myeloproliferative disorders.\nAfter 3 days of IV vancomycin therapy, he was transitioned to trimethoprim-sulfamethoxazole DS for 7 days. He was also given Azole anti-fungal cream for topical application to his lesions. His wounds were regularly cleaned and dressed. For his EM, he was started on gabapentin 200 TID, pregabalin 75 mg BID and lidocaine patch. Tylenol was added PRN and daily Aspirin 325 was started. Intermittent ice packs were applied for symptomatic relief and he was advised to stop cold water immersions.\nDuring his hospitalization, he showed mild improvement in pain but still complained of ambulation triggering his symptoms. His cellulitis resolved and wound cultures prior to discharge showed no bacterial growth. Symptomatically, his pain level dropped to 2/10, lasting 2 hours and improving with ice packs. After 4 days of hospitalization, he was discharged with a course of antibiotics, aspirin, pregabalin, and lidocaine patch. He was given follow up appointment for the rheumatology clinic for long term management of EM. He was also educated about a specialized erythromelalgia center in the city as genetic testing for the disease was not available at our institution. |
A 75-year-old woman (46.2 kg, 153.7 cm tall, and American Society for Anesthesiologists physical status II) was scheduled for a partial duodenectomy. She provided written informed consent for participation and publication. Her previous surgical history included ovariectomy under general anesthesia at the age of 38 years, for which the surgical and anesthetic procedures were uneventful. She also had a history of hypertension, which was treated with angiotensin II receptor blockers. She had no other past medical history. At presentation, she was not taking any herbal preparations or anticoagulants. She had no family history of bleeding tendency.\nFour months prior to her surgery, she developed epigastric pain and an endoscopic examination revealed a duodenum tumor; she was scheduled for a partial duodenectomy. Laboratory findings on admission were as follows: normal electrocardiogram and chest X-ray, hemoglobin 13.4 g/dL, platelet count 21.5 × 104 μL, and the coagulation tests showed prolonged activated partial thromboplastin time (APTT 89.7 s); however, the prothrombin time and the international normalized ratio of prothrombin time were normal (PT 10.3 s, PT-INR 1.01). Hemophilia A and B are usually considered first, especially in males, whenever a prolonged APTT is noted; however, our female patient had no family history of bleeding. Further investigation revealed severely deficient levels of factor XI, and factor XI deficiency was diagnosed. The levels of activity of her factors VIII, IX, XI, and XII are shown in Table .\nIt is obvious that patients with severe clotting factor deficiencies are at risk of bleeding from the surgical site, and anesthetic management is one of the most important concerns for the attending anesthesiologist. To avoid massive bleeding during the surgical procedure, 4 units of fresh frozen plasma (FFP) was transfused (in Japan, the estimated volume per unit of FFP is 120 mL/unit) 1 day prior to the surgery. We performed another coagulation test immediately before entering the operating room, and her APTT level improved to 36.2 s.\nIn our facility, epidural anesthesia is the first choice of analgesia for open upper abdominal surgery. However, because the patient had a potential risk of epidural hemorrhage, we avoided epidural anesthesia and selected ultrasound-guided subcostal transversus abdominis plane (TAP) block with rectus sheath block. General anesthesia was induced by target-controlled infusion of propofol at a target plasma concentration of 3 μg/mL. Fifty milligrams of rocuronium was administered after loss of response to verbal commands. Remifentanil was initiated at a rate of 0.3 μg/kg/min, and propofol was adjusted to maintain a bispectral index of 40–60. Subcostal TAP block with rectus sheath block was performed in the supine position. After skin preparation with 0.5 % chlorhexidine, the ultrasound probe was placed parallel to the subcostal margin near the xiphoid process, the needle was advanced to the TAP, and 20 mL of 0.25 % levobupivacaine was administered to the TAP. After subcostal TAP block, the rectus muscle was imaged with the ultrasound probe on the level of the umbilicus and the needle was advanced to the posterior rectus sheath, and 10 mL of levobupivacaine was administered.\nThe surgical procedure itself was uneventful, and recovery was satisfactory. The operation duration was 289 min, and the anesthesia duration was 406 min. The blood loss was 222 mL, and the urinary output was 1250 mL. The patient received 3600 mL of fluid administration. The patient reported no pain at the surgical site after extubation. Postoperative supplemental analgesics were administered. The patients received intravenous flurbiprofen axetil and acetaminophen on the first postoperative day. After started oral intake, loxoprofen sodium was administered and reported no pain.\nAfter the surgery, thin, watery-pink exudate was seen from surgical drain, but on the seventh postoperative day, the color of the fluid changed to have a more slightly reddish consistency. Although amount of exudate from the surgical drain did not increase, the drain was removed on the same day. The levels of exudate from the surgical drain after surgery are shown in Table . Coagulation tests were performed every day after the surgery. The PT and PT-INR levels were normal throughout the perioperative period. The perioperative APTT levels are shown in Fig. .\nFactor XI deficiency, which is relatively common among Ashkenazi Jews, is associated with injury-related bleeding []. This is sometimes called “hemophilia C,” is distinguished from hemophilia A and B by the absence of hemorrhage inside joints and muscles, and has equal incidence in individuals of either gender. The level of the deficiency does not determine the bleeding risk [], and bleeding tendency varies among individuals. Patients who have severe factor XI deficiency are at risk of massive hemorrhage; however, some do not have this tendency [].\nPatients with factor XI deficiency need specific management during surgery. Previously, adverse surgical outcomes of patients with factor XI deficiency have been reported, including cerebral hemorrhage [] and spinal epidural hematoma []. Different surgical management strategies, each with varying degrees of risk, have been reported, including FFP, factor XI concentrates, and desmopressin [].\nTo manage hemorrhage tendency during the perioperative period, anesthesiologists must determine an appropriate method and tailor it to each patient. We used 4 units of FFP prior to the surgical procedure. The estimated half-life of factor XI is 45 h, which is similar to standard FFP []; therefore, we judged FFP transfusion 1 day prior to surgery which would prevent intraoperative and acute phase massive hemorrhage. As shown in Fig. , APTT levels improved from 89.7 to 36.2 s after FFP transfusion. We investigated the transition level of APTT after surgery. The level of APTT was gradually prolonged to 52.6 s by postoperative day 7. After the surgery, the color of the exudate fluid from surgical drain had changed to slightly reddish, which may have been related to the change of APTT. Considering the estimated half-life of factor XI, we anticipated that APTT would rise to the same level as the preoperative value. Fortunately, APTT remained shorter than expected, and we did not need to use additional FFP.\nThe disadvantages of plasma which are the large volumes are required and the potential for allergic reactions and infections. The patient described in our case report had no cardiac disease, and only 4 units of FFP were used, so fluid overload was not an issue; however, FFPs should be transfused carefully, especially in patients who have cardiac failure. Although factor XI concentrates are an effective treatment, they are not currently available in Japan.\nIn addition to medication, safe and reliable anesthesia is required to manage perioperative hemorrhage. Epidural anesthesia is one of the most useful methods to alleviate surgical pain during the perioperative period. Although the incidence of neurologic dysfunction resulting from hemorrhagic complication associated with neuraxial blockade, including epidural anesthesia, is unknown [], the risk of bleeding is increased, especially in the presence of coagulopathy. In recent years, peripheral nerve blocks using ultrasound have gained popularity. It serves as an effective alternative analgesia when an epidural is contraindicated or refused. Although guidelines for regional anesthesia in the patient receiving anticoagulants are available, regional anesthesia for patients with a clotting factor deficiency is dependent on the careful decision-making by the attending anesthesiologist. In this case, we opted to avoid the potential risk of a spinal hematoma associated with epidural anesthesia and decided to control perioperative pain using a peripheral nerve block. Subcostal TAP block provides analgesia in the upper abdominal area [], and combined with a rectus sheath block that provides effective pain relief for the umbilical are, we were able to alleviate pain in our patient. Neurologic dysfunctions after epidural anesthesia are very rare but devastating. Appropriate management for patients with clotting factor deficiency should result in an uneventful recovery. |
A 51-year-old right-handed female presented to cognitive clinic for evaluation of worsening psychiatric and behavioral symptoms. The patient was receiving psychiatric care for generalized anxiety and obsessive compulsive disorders since her early 20s. The main symptoms of her initial conditions included significant anxiety and intermittent preoccupations with repetitive behaviors. None of these symptoms interfered with her daily living and functioning. The patient’s family reports that despite psychiatric symptoms, she had no problems with memory, social judgment or functioning at home or outside the house. Historically, the patient required multiple psychiatric medications to manage her symptoms, but reported poor response to treatment. At some point, the patient became addicted to alprazolam and alcohol, requiring admission to inpatient alcohol rehabilitation facility. The patient did not use any substances for 8 years prior to presenting to our clinic.\nThe patient’s husband reports significant changes in her behavior 1 year prior to our evaluation. The patient started withdrawing from her usual activities, displaying inappropriate behaviors, using foul language and making tactless comments. She started showing psychomotor hyperactivity with frequent pacing. It was becoming more obvious that the patient’s empathy is deteriorating, and the patient was becoming more self-centered. She also became preoccupied with her looks, at the same time developing marked deterioration in her personal hygiene. Her attention span and immediate memory were worsening: she was not able to multitask or pay attention to more than one subject at a time. The patient also intermittently started getting lost in a familiar environment. There were no face or object recognition deficits reported. There were no symptoms of eating disorders or changes in food preference reported. The patient’s husband denied balance impairment.\nExamination demonstrated a poorly kempt woman. She was very frustrated about being at the doctor’s office. Her insight and judgment were very limited. She was frequently loosing the track of thought, and her speech would become tangential. The patient displayed verbal perseverations, confabulations, mild intermittent word-finding deficits and rare semantic paraphasias. The patient made several inappropriate comments and displayed episodic memory impairment, which would improve with cueing. There were no recognition deficits. Neurological examination demonstrated full eye movements, normal muscle tone and strength and preserved normal deep tendon reflexes. There were no asymmetric sensory findings, tremor or ataxia.\nMagnetic resonance imaging (MRI) of the brain demonstrated significant asymmetric right hemisphere atrophy, mostly involving the anterior right temporal lobe ().\nThe diagnosis of behavioral variant FTD was made based on 2011 clinical criteria. There was no neuropathological diagnosis available for this patient at the time of this report. |
This unique case involves a 68-year-old female who was hit by a car as a pedestrian crossing the street and was dragged along for a distance. She was 35-year-old at the time of injury which resulted in an open right tibial fracture with severe soft tissue loss over the proximal one-third of the tibia. She had a total of 5 surgeries over 3 years to treat her fracture and overlying infection. A medial gastrocnemius flap was rotated to cover the medial knee and tibia. Her ultimate reconstruction was a knee fusion at age 38, which was stabilized with a cast rather than internal fixation. Her knee fusion surgery was successful. Since her fusion she has had no problems with infection.\nTwenty-four years later, at the age of 62, she presented to our clinic with severe pain in her back and ipsilateral hip. She wanted her knee fusion taken down and converted to a TKA. A careful pre-operative review indicated intact muscular firing of her quadriceps musculature. Clinically, she had an intact patellar tendon into the tibial tubercle. Studies evaluating for residual infection at the knee and tibia were negative. Her pre-operative radiograph of the knee fusion is shown in Figure .\nA salvage TKA was performed with segmental resection of her distal femur. She was reconstructed with a custom reduced-size endoprosthetic hinge device based upon the Orthopaedic Salvage System (OSS™) knee design (Zimmer-Biomet, Warsaw, IN, United States). Due to her soft tissue loss over the medial knee and tibia, the prosthetic joint line was placed cephalad. Her post-operative recovery was unremarkable. Her ultimate knee range of motion within the first year was 0-80 degrees. In addition, she had no knee extensor lag. Her reconstruction is shown in Figure .\nSix years post-operatively, at her scheduled follow-up, the patient described mild distal thigh and knee pain while walking. Her knee was cool on examination. Knee range of motion was 0-80 degrees of flexion with no extension lag and her knee felt stable. Radiographs showed concerning findings in the distal diaphysis (Figure ). There was a radiolucent line in the distal diaphyseal cement mantle, but more concerning was a periosteal erosion and surrounding soft tissue mass that resembled a possible neoplastic process. A CT scan of the distal femur revealed an eccentric lesion of the distal diaphysis of the femur that appeared to be emanating from the outer cortex of the medial diaphysis. The soft tissue mass was approximately 7 cm in length and 2-3 cm in breadth and was partly surrounded by a calcific border (Figure ).\nHer knee joint aspiration was negative for culture growth, including fungus and acid fast bacillus. The articular white blood cell count was 259 with 71% neutrophils. Alpha-defensin and Synovasure® (Zimmer-Biomet, Warsaw, IN, United States) tests were both negative. Her serum CRP and ESR levels were normal. Evaluation for metastatic disease was negative.\nOur surgical plan was for en bloc removal of the femoral diaphysis, including the femoral stem. We chose not to biopsy the mass in order to avoid neoplastic contamination of the extended endoprosthetic joint space.\nPost-operative radiographs of the revision surgery are shown in Figure . Nine centimeters of the femoral diaphysis was resected en bloc with the femoral stem, femoral diaphysis, and tumor mass. The femur was reconstructed with additional intercalary segments using the OSS Knee system. Fixation to the native femur was achieved with a femoral compress device (Zimmer-Biomet, Warsaw, IN, United States). Upon submission of the en bloc specimen to pathology, the tumor was cut open. We saw a multi-cystic, dark grey colored pseudotumor. The base of the pseudotumor emanated from the femoral diaphysis. We did not see any metallosis within the articular joint space (Figure ).\nThe microscopic examination (Figure ) of the resected en bloc section revealed a partially solid and cystic lesion encased in a fibrous pseudo-capsule. There were numerous foreign body histiocytes containing waxy amorphous material. Perivascular lymphocytes and histiocytes were prominent. The specimen was formally diagnosed as a metallic debris pseudotumor. There was no evidence of any neoplastic process.\nThe patient's post-operative recovery was again uneventful. She had complete wound healing. Her range of motion at one-year follow-up was 0-88 degrees of flexion and there was no extensor lag. |
A 39-year-old right hand dominant male sought our services with a history of eight months of pain in the posterior region of the left shoulder, with no history of acute trauma. The patient stated that the pain began after starting a job where he carried sandbags of approximately 25 kg on the affected shoulder. The pain began after two months on the new job and progressively worsened with time. The pain was worse with exertion. The pain was partially relieved with rest and the use of non-steroidal anti-inflammatory drugs. At the time of his first appointment, the patient was unable to work, and he did not report any comorbidity.\nPhysical examination of the left shoulder revealed no gross deformity. On palpation, the patient reported pain over the scapular spine, approximately 5 cm medial to the lateral border of the acromion. The passive range of motion was normal, and active forward elevation, abduction and external rotation were limited to 130°, 90° and 30°, respectively. The patient exhibited pain and decreased strength on the Jobe and infraspinatus tests as well as negative signs for a superior labrum anterior and posterior (SLAP) lesion and acromioclavicular arthrosis. The functional assessment scored 10 points on the University of California at Los Angeles (UCLA) Shoulder Rating scale and 7 points on the visual analog scale.The initial radiographs revealed a transverse fracture of the base of the acromion on the spinoglenoid notch without deviation (Figure ). A non-surgical treatment with analgesics, rest, absence from work, and physical therapy (analgesia, stretching, and strengthening) was used for four months, with no clinical improvement. New imaging exams (radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) revealed the absence of consolidation (Figures and ). Thus, after 4 months of conservative treatment and 1 year after the onset of symptoms, surgical treatment was recommended.\nThe patient was placed in the prone position, and a 10 cm incision was made along the scapular spine towards the lateral edge of the acromion. The trapezius-deltoid fascia was opened towards the spine. The deltoid and trapezius muscles were detached, exposing the pseudarthrosis focus, with hypertrophic callus. A partial resection of the hypertrophic callus was performed, and the scapular spine was anatomically reduced. The osteosynthesis was performed with a 6-hole 3.5-mm locking compression plate (LCP) with 3 screws locked proximally and 2 distal screws, 1 of them locked and 1 cortical screw placed eccentrically, resulting in an interfragmentary compression of 1 mm (Figure ). The lateral fixation with 3 screws was not possible due to the location of the fracture. No bone graft was used, and the incision was closed in layers, reinserting the muscles using transosseous sutures.The operated limb was immobilized with an arm sling for 6 weeks, and the mobilization of the hand, wrist, and elbow was performed beginning on the 1st postoperative day. Passive shoulder movements were started at 3 weeks, free active movements at 6 weeks and resistance movements at 8 weeks. After 4 postoperative months, the patient had recovered full range of motion. The radiograph (Figure ) and the CT performed at 5 postoperative months (Figure ) revealed the healed fracture. After 6 months, the patient rarely reported mild pain and returned to his work. The UCLA scale was 33 at 6 months of follow-up and 35 at 12 months. The maximum UCLA score is 35 points. The patient recovered full painless range of motion and normal strenght. |
We present the case of a forty-one year old male patient with previous medical history of Diabetes Mellitus, who never had any surgical intervention. He presented to our Emergency Department complaining of peri-umbilical pain for 1 day. The pain was moderate in severity and colicky in nature, associated with nausea and vomiting. There was no history of hematemesis, melena, or hematochezia. His vital signs were unremarkable. Abdominal examination revealed moderate tenderness in the peri-umbilical region with no rebound tenderness or guarding and no organomegaly. Bowel sounds were audible, and rectal examination was normal. His laboratory investigations were within normal range.\nHe underwent Computed Tomography (CT) of the abdomen and pelvis, which revealed a short segment of small bowel intussusception at the left upper quadrant with a target sign appearance. There were no signs of bowel obstruction or ischemia. (Figures and ). Since the patient’s symptoms of pain and vomiting were persistent and the CT was showing small bowel intussusception in a diabetic patient who has no known risk of developing transient intussusception, in addition to the surgeons concern of irreducibility and possible presence of underlying pathology, the decision was made to take him for laparoscopic exploration. In the operating room following general anesthesia, the patient was placed in supine position with split legs. Transabdominal ultrasound was performed prior to skin cut and it showed the small bowel intussusception with target sign (Figure ) confirming the CT finding. The exploration was performed using three ports. On inspecting the peritoneum, there was no free fluid nor were there signs of inflammation. The small bowel was inspected from the ligament of Trietz till the ileo-cecal valve. Multiple segments of the small bowel were observed intussuscepting and reducing spontaneously (Additional file ). During the running of the small bowel no visible masses or other pathology were identified.\nFollowing that, we made a small laparotomy of 3cm in size by enlarging the 12mm peri-umbilical port. This was used to manually examine the bowel for small nonvisualized intra-luminal or intra-mural masses or polyps; the result was also negative. The patient had uneventful recovery and discharged home on day 3 post operation. The patient was informed and educated about his condition, was prescribed anti-spasmodic for symptomatic relief when needed and was instructed to return to emergency care if an attack does not resolve. The patient also had a small bowel follow-through study 4 weeks post surgery, which was normal. There were no adverse or unanticipated events.\nAll data reported in the manuscript have been visualized and then approved by our University Hospital Ethics Committee and all procedures carried out on the patients were in compliance with the Helsinki Declaration. Furthermore, the authors confirm that a written informed consent was obtained from the patient for publication of this case report and accompanying images. |
A 14-year-old boy presented at outpatient department with firm, nontender swelling of left maxillary region, for 6 months. There was no history of trauma. His extra-oral examination showed mild facial asymmetry, with the solitary diffuse swelling sparingly evident in left middle third of face, extending from 1 mm above the ala of the nose till the left commissure (). Bilateral submandibular lymph nodes were tender on palpation and were about 2 × 1 cms each. Intra-oral examination revealed the presence of retained deciduous tooth 63. Diffuse swelling measuring 2 × 3 cms in size with vestibular obliteration in relation to 21–24 was evident (). A panoramic radiograph revealed a well-defined radiolucency with sclerotic rim enclosing whole of an impacted canine (23) in left maxilla (). The sclerotic rim was seen to be attached at the apex and not at cervical region of the tooth (differentiating factor between an AOT and dentigerous cyst) (). An occlusal radiograph confirmed the presence of an impacted 23 (). On the basis of clinical and radiographic findings, the provisional diagnosis of follicular AOT with an impacted 23 was given. An incisional biopsy was performed and to our surprise, cystic lining comprised of 2-3 cell layers thick, nonkeratinized epithelium resembling reduced enamel epithelium was seen (). The mass was enucleated in toto and the cyst was separated out easily from the adjoining bone and was removed with the involved tooth. Histopathological examination of the specimen revealed cystic epithelium, 2-3 layers in thickness, resembling reduced enamel epithelium. Predominantly, the supporting connective tissue capsule was comprised of bundles of collagen fibers, arranged parallel to cystic epithelium. In one bit, overlying cystic epithelium was seen with the underlying capsule comprised of cuboidal to columnar epithelial cells forming a rosette-like structures about a central space containing eosinophilic material (). In the same bit, few tubular or duct-like structures were also seen. These ducts consist of central space surrounded by a layer of cuboidal to columnar cells, nuclei of which were polarized away from the central space (). Correlation of the histological findings with clinical and radiographic findings persuaded us to give a final diagnosis of cystic AOT. The postoperative course was uneventful and there were no signs of recurrence till 2 years later. |
A 62-year-old female patient with a history of mild hypertension for over 10 years presented with paroxysmal exertional chest pain for 1-week, which progressively worsened over the past 12 hours, was admitted to our center. There was no significant positive physical findings. The electrocardiogram (ECG) showed slight ST segment elevation of leads III and avF during chest pains. Her echocardiography revealed a normal functioning heart (left ventricular ejection fraction: 65%) with no abnormal wall motion or cardiac enlargement. Her troponin level was 967.6 ng/L (normal, ≤14 ng/L).\nThe patient underwent coronary angiography (CAG) due to the possibility of an acute non-ST segment elevation myocardial infarction. It revealed an aneurysm at the ostium of the right coronary artery (RCA), which resulted in total occlusion of the proximal segment of the RCA, except for the conus branch (Fig. ). There was no obvious obstruction or pathological lesions in the left coronary artery. Next, computer tomography angiography (CTA) of the head, neck, thoracic, and abdominal aorta was performed to rule out other potential arterial diseases. It revealed an aneurysm in the distal segment of right renal artery as well as an aneurysmal dilatation in the third lumbar plane of abdominal aorta, and no obvious abnormalities in the head and neck arteries (Fig. ). The patient's C reactive protein, serum creatinine, immunoglobulin, antinuclear antibody, anticardiolipin antibody, and antineutrophil cytoplasmic autoantibody levels were all normal.\nDue to the patient's ongoing angina and the presence of an aneurysm at the ostium of RCA, which resulted the total occlusion of the proximal segment of the RCA, the patient eventually underwent aneurysmal ligation with a concomitant distal bypass graft. However, the renal artery and abdominal aortic aneurysms were conservatively treated due to no related symptoms. The patients made an uneventful recovery and were discharged on the tenth day. At 11-month follow-up, the patient was alive and well without limitations in her daily life. |
The case of this report is a 36-year-old married, nonsmoking, non-alcoholic female with primary infertility since 9 years of married life undergoing continuous interventions and repeated ART. Previous specialized evaluations include hormonal assays such as follicle-stimulating hormone, luteinizing hormone, estradiol, thyroid function test and AMH; ultrasonography (USG); and karyotyping. Investigations into the male factor revealed OAT. She underwent three courses of intrauterine insemination (IUI) and three courses of ICSI which were unsuccessful. AMH value before fourth ICSI was very low after which she was advised for an ICSI with a donor ovum.\nThe patient got married in the year 2005 at the age of 27 years. Investigations carried out on both partners after 2 years of married life revealed OAT. After correcting the sperm count with oral medications, she underwent three cycles of IUI in 2010 which was unsuccessful. Due to OAT and failed IUIs, she was subjected for ICSI after necessary hematological evaluation and hormonal assays which came out to be normal. AMH value was 2.5 ng/mL before first ICSI on November 3, 2011. Six eggs were retrieved and one embryo was transferred, but there was no rise in human chorionic gonadotropin (hCG) value. Second ICSI was on May 10, 2012, in which three eggs were retrieved and three matured embryos were transferred. hCG value raised to >1500 mIU/ml on May 31, 2012, but had tubal abortion on June 3, 2012. Third ICSI was on July 21, 2013 and three embryos were transferred from five retrieved eggs. She had threatened abortion on August 19, 2013, and USG on August 20, 2013, showed single intrauterine gestational sac with subchorionic hematoma and a repeated scan on September 3, 2013, revealed an empty endometrial cavity. Routine investigations carried out on January 2, 2014, before fourth ICSI revealed very low AMH value (0.07 ng/mL). She was advised for undergoing next ICSI with a donor ovum due to the low AMH value. As the couple was not willing to accept a donor egg, they visited our outpatient department (OPD) on February 1, 2014 in the hope of undergoing ICSI with her own ova by improving the AMH value with Ayurvedic treatment. Her medication includes Eltroxin 50 mcg for hypothyroidism since 2010. Her history was relevant only for chickenpox in 2005 and jaundice in 2012. Family history was negative for any premature ovarian failure or low AMH. Her personal history revealed a regular bowel habit and sound sleep. Her appetite was apparently normal and the tongue was uncoated. She attained menarche at the age of 12 years with 2–3 days’ duration in 26–28 days’ interval. The amount was scanty with noticeable vaginal dryness since 3 months. Her obstetric history was gravida 2 abortion 2 ectopic pregnancy 1 (G2A2E1). On examination, she was calm and pain free. Her blood pressure was 120/80 mmHg, pulse rate 70/min and body mass index 23 kg/m2. She is of Vata Kapha Prakṛiti with Madhyama Satva (moderate mental strength) and Madhyama Koshtha (moderate bowel). Her physical examination and examination of external genitalia did not revealed any abnormal findings. Per speculum examination showed a healthy nulliparous cervix without any significant abnormality. Bimanual examination revealed an anteverted mobile uterus with a negative cervical motion tenderness. Medical history of the patient is detailed in .\nIn the view of symptoms of Artava Kshaya (hypomenorrhea), the present case was diagnosed as Dhatukshayajanya Vandhyata (infertility due to depletion of body tissues). The assessment was done by comparing baseline AMH value with repeated evaluation after 3 months of Shamana (oral medication) and Shodhana treatment (purification therapy). Baseline AMH value was 0.07 ng/ml (reference range: 2–6.80).\nThe therapeutic plan was to administer Shamana Chikitsa (oral medication) followed by Shodhana Chikitsa (purification therapy). Shamana Chikitsa was done for 3 months with Vaishavanara Churna and Mahanarayana Taila. As her clinical symptoms and AMH value after Samana Chikitsa showed satisfactory improvement, she was subjected to Shodhana Chikitsa. Initially, her Agni was corrected by Dipana-Pachana (improving digestion) with Vaishavanara Churna as a Purvakarma (preparation) of Shodhana. She attained Nirama Lakshana (signs of digestion of Ama) by 2 days after which Accha Snehapana (intake of oil) was started with Mahanarayana Taila. Samyak Snigdha Laksana (signs of proper oleation) was observed after 6 days of Snehapāna. Mridu Virechana (mild purgation) was performed on the administration of Trivritlehya. After Virechana, she was subjected to Yoga Basti and Uttara Basti (medicated enema). After completion of Shodhana therapy, she was discharged from the hospital [].\nAfter 3 months, the patient was advised to have a follow-up in the OPD with a reassessment of AMH value. It was observed that there was a marked improvement in the AMH value as shown in . |
A 27 years-old primigravida Ethiopian mother presented with a history of pushing down pain and passage of liquor of 6 hours duration at gestational age of 30 weeks plus 6 days from a 24 weeks ultrasound. Her last normal menstrual period was unknown. She had no history of vaginal bleeding. She had no history of fever. She had no history of other underlying obstetric or medical problem. She had antenatal care follow-up at our hospital. Her medical, surgical, psychosocial, and family history was unremarkable. Obstetric ultrasound was done twice in the second trimester (at 24 weeks and 26 weeks gestation) which documented a normal dichorionic-diamnotic (DCDA) twin pregnancy with no evidence of any congenital anomaly.\nUp on physical examination, the vital sign of the patient was stable. Her blood pressure was 110/70 mmHg. She had a pulse rate of 88 beats per minute, temperature of 37 °C, and respiratory rate of 16 breaths per minute. The pertinent finding was on pelvic examination—cervix was 3 cm dilated with 80% effacement. First twin was in complete breech presentation at station of − 1 and membrane was ruptured and clear liquor.\nWith the assessment of latent phase of labor plus twin pregnancy (DCDA) first twin in breech presentation plus early preterm pregnancy, obstetric ultrasound was done. The sonographic findings was first twin in breech presentation with estimated fetal weights of Twin A of 1200 g and twin B cephalic presentation 1100 g. The biophysical profile for both twins was reassuring. Baseline fetal heartbeat was 126 and 140 for twin A and twin B, respectively. With an indication of twin pregnancy (first non-vertex) in labor, emergency cesarean section (CS) was decided but patient refused for the CS delivery despite being thoroughly counseled repeatedly.\nThe fetal heart rate for both twins was monitored using CTG at labor ward and it was normal throughout until she entered second stage of labor. She entered second stage of labor after 4 hours of labor. One hour in to the second stage, at station of + 2, locked twin was diagnosed. By that time, first twin was delivered up to the shoulders. Consultant on duty was consulted immediately, and manual maneuvers was attempted to dislodge the interlocking. However, it was not successful multiple times. Still, it was difficult to convince the mother to undergo abdominal delivery after 5 minutes of trial of the maneuvers. By the time our patient agreed for CS delivery, the first baby was already asphyxiated and fetal heart beat was negative when we arrived at the operation theater. The fetal heartbeat of the second twin was also detected to be in bradycardic range (106–112 beats per minute) as it was checked before proceeding with CS delivery.\nWith an indication of locked twins Cesarean delivery was done, a low-vertical CS. First, second twin in cephalic presentation was extracted after the chin-to-chin twin interlocking was relieved manually. The attending surgeon's right hand rested on the lateral side of the second baby's face and it slipped the chin of the second twin with a gently push from the lateral side. Then, the head of the second twin was extracted cephalic by a push technique.\nDelivery of the babies was effected without the need of decapitation of the first twin. The outcome was a 1000 g female second twin born with APGAR score of 4, 2, and 2 in the first, 5th and 10 minutes respectively. The baby passed away after 20 minutes of resuscitation. Chest compressions synchronized with a positive pressure ventilation, and administration of adrenaline three doses was instilled. Subsequently, the first twin, a 1200 g male still-born was delivered vaginally with simple traction from below. The mother was counselled for autopsy of both neonates but she declined it. The decision to delivery time was 20 minutes. The mother was discharged with full recovery after three days of stay in the maternity ward. Her post-operative follow-up visit after a week didn’t document any abnormality. She had a good wound healing. |
A 20-year-old African American man was admitted to a psychiatric facility for psychosis. On initial presentation, the patient had an antalgic gait, which he attributed to his history of dopa-responsive dystonia. His mood was depressed and his affect was restricted. He had disorganized thought process and was slow to recall. He endorsed auditory hallucinations, paranoid delusions, depressive symptoms, frequent night awakenings, and persecutory nightmares. Per the ambulance report, the patient was wandering the streets in a confused state, so bystanders called 911. The patient stated that he had been homeless for the past 3 weeks. During this 3-week period, he admitted to not being complaint with his medications. Urine toxicology screen was negative.\nPer medical records, he was diagnosed with dopa-responsive dystonia at age 11 after a 2.5-year history of progressive abnormal gait. He was initially misdiagnosed with tight heel cords at age 10 and treated with serial casting that resulted in good improvement on the right but marginal improvement on the left. His toe walking became more pronounced overtime accompanied by worsening left calf pain and stiffness, increasingly frequent falls, and new onset of intermittent torticollis. These symptoms worsened over the course of the day. He was eventually taken to an urban teaching hospital, where he was diagnosed with dopa-responsive dystonia based on clinical presentation and marked improvement on a levodopa trial. Magnetic resonance imaging of the brain and spine was unremarkable at the time.\nAt age 15, he was diagnosed with schizoaffective disorder bipolar type. His psychiatric history is also significant for multiple psychiatric hospitalizations, history of previous suicide attempts with medication overdose, and history of trauma. He also endorsed marijuana use since age 15 and daily tobacco use since age 18. He denies using any other illicit drugs. Per collateral information from his mother, his schizoaffective disorder has never been well controlled given the conflicting effects of his medications. She also mentioned that he was placed in individualized education programs as a child due to learning disabilities. His family history is significant for bipolar disorder on his maternal side. His family history on his paternal side is unknown. In addition to carbidopa-levodopa, his outpatient medications included sertraline, divalproex sodium, aripiprazole, and benztropine.\nOn hospital day 1, he was started on carbidopa-levodopa 25/100 mg tablet three times daily for dopa-responsive dystonia. On day 2 of his hospital course, sertraline 50 mg once daily, benztropine 2 mg twice daily, divalproex sodium 500 mg twice daily, and risperidone 0.5 mg twice daily were added to his medication regimen. We started him on a low-dose risperidone to avoid exacerbating his dopa-responsive dystonia symptoms. Physical exams were also performed daily to assess for dystonia and parkinsonian symptoms. His initial physical exam revealed an antalgic gait secondary to left lower extremity dystonia, which improved by hospital day 2 and resolved by hospital day 3. On hospital day 3, he became agitated and aggressive with staff members, which led to intramuscular administrations of haloperidol 10 mg, diphenhydramine 50 mg, and lorazepam 2 mg. He continued to endorse auditory hallucinations, so risperidone was increased to 0.5 mg in the morning and 1 mg at bedtime. His auditory hallucinations resolved and then returned on day 6. He reported hearing “good” voices and “bad” voices. He also continued to endorse depressive symptoms, multiple night awakenings, and persecutory nightmares. As a result, his risperidone dosage was increased to 1 mg twice daily. On hospital day 7, the patient reported hearing “mumbling” voices only and improvement in his sleep and depressive symptoms. On hospital day 8, his auditory hallucinations fully abated. By hospital day 10, he slept throughout the night, no longer had depressive symptoms, and had normal spontaneous speech. His thought process was linear, logical, and goal-oriented. His mood and affect was euthymic and full range. No psychotic symptoms were noted. The patient was compliant with his medications throughout the whole hospital course and his daily physical exams were negative for dystonia or parkinsonian symptoms since day 3 of his hospitalization. He was subsequently discharged on hospital day 14 with appropriate outpatient follow-up. |
A 75-year-old male with hypertension and medically treated aortic dissection that occurred several years ago again presented with severe chest and back pain. A CT scan revealed an aortic dissection (Stanford type B) combined with a massive aneurysmal dilatation, and the intimal tear site was nearly 2 cm from the left suclavian artery. Because the aortic aneurysm involved an aortic arch, the proximal landing zone was less than 1 cm wide (). Because of his age and the characteristics of the lesion, there was the possibility of further operation related complication (perioperative mortality, morbidity, stroke, paraplegia etc.) and the patient did not want open surgery; we therefore decided to perform TEVAR rather than open surgery. The patient was taken to the cardiac catheterization laboratory and an arteriotomy for the left femoral artery was performed under general anesthesia. The 035 inch wire was placed in the ascending aorta and the left subclavian artery through the left radial approach. The aortogram confirmed a large aortic aneurysm adjacent to the origin of the left subclavian artery. Two thirds of the origin of the left common carotid artery was covered by the proximal part of a 40×160 mm SEAL thoracic aortic stent (S&G biotech, Seongnam, Korea) to achieve an adequate landing zone. Another 40×130 mm SEAL thoracic aortic stent (S&G biotech, Seongnam, Korea) was deployed in the first aortic stent graft with a 7 cm overlapped segment to protect against disconnection of the aortic stent grafts. After selection of the left common carotid artery using a 5 Fr Judkin right catheter through the gap between the stent graft and the left carotid artery, we passed a 035 inch Amplatz stiff wire into the left carotid artery through the right femoral artery. An 8×60 mm SMART nitinol stent (Cordis, Hialeah, FL, USA) was then deployed into the left common carotid artery. The final angiogram showed excellent results with good flow to both the thoracic aorta and the left common carotid artery. No endoleak was noted (). On the clinical follow-up of this patient (20 months to date), the patient has had no problems related to the graft and the follow-up CT scan showed no sign of malfunctioning grafts, restenosis of the stent in the left carotid artery or other complications. |
A 56-year-old female was admitted to our hospital with a fever, double lower extremity edema, and recent weight loss. Eleven years ago, she had a mitral valve replacement with a 27 mm St. Jude mechanical valve, and six years ago she had a bioprosthetic tricuspid valve replacement with a 29 mm Edwards bioprosthetic valve due to severe tricuspid valve regurgitation. Since earlier this year, she had recurrent abdominal distension and double lower extremity edema, symptoms that were alleviated by treatment with a diuretic. She first presented to a local hospital in January with complaints of fever and cough for the previous month. A chest X-ray at that time indicated pulmonary infection, and transthoracic echocardiography showed bioprosthetic tricuspid valve calcification and medium regurgitation, but no vegetation. She was prescribed oral antibiotics and diuretics for two months. Despite decreased coughing and evidence of improvement in her chest X-ray films, she continued to suffer from abdominal distension and double lower extremity edema. Three different blood cultures were negative, and she was admitted to our hospital in Iune. The patient had no history of intravenous drug abuse or diabetes mellitus. Her body weight had declined by 10 kg since this year.\nOn admission to our unit, she had a temperature of 37 °C, atrial fibrillation with an average rate of 70 beats/min, and blood pressure of 120/60 mmHg. Auscultation indicated a diastolic sound below the xiphoid process. Laboratory tests showed leukocytosis (15.5 × 109 WBCs/L) and anemia (75 g Hb/L). Her serum level of C-reactive protein was 15 mg/dL, erythrocyte sedimentation rate was 25 mm/h, and a chest X-ray film demonstrated scattered patches in both lower lung fields. The patient’s status began to deteriorate on the second day after admission. She suffered from intermittent whole-body chills and a fever spiking up to 39 °C. Transthoracic echocardiography showed large vegetations attached to the bioprosthetic tricuspid valve, damage to two of the three leaves, and severe tricuspid regurgitation. Blood cultures were prepared from three samples collected at hourly intervals, and led to a diagnosis of A. baumannii of the bioprosthetic tricuspid valve. Antimicrobial susceptibility testing showed that the isolate was sensitive to cefoperazone sodium and sulbactam sodium, but resistant to imipenem, penicillin, ampicillin, clindamycin, and linezolid. Thus, we initiated treatment with intravenous cefoperazone sodium and sulbactam sodium. After 4 weeks of antibiotic therapy, her pyrexia was completely controlled and intermittent blood cultures showed negative results. We therefore performed re-tricuspid valve replacement.\nWe completed valve replacement in the beating heart under cardiopulmonary bypass, during which we noted vegetations on all 3 leaves and damage to 2 of the leaves (Figs. and ). We used a dilute iodine solution in normal saline- cefoperazone sodium to irrigate the surface of the tricuspid annulus after removal of the bioprosthetic valve. A 29 mm St. June mechanical prosthetic valve was inserted according to the patient’s choice. Intraoperative cultures of the vegetations were negative. Postoperatively, she received intravenous cefoperazone sodium and sulbactam sodium for 2 months. Before discharge, all post-operative numerous blood cultures showed negative results and the patient reported that cardiac insufficiency-related symptoms were significantly ameliorated. During the follow-up period, there was no fever, no symptoms of infection, and no cardiac insufficiency.\nA. baumannii was initially thought to be an organism of questionable pathogenicity, but has emerged as a major cause of nosocomial infections. Isolates of this species are frequently resistant to multiple antimicrobial agents, and can therefore cause life-threatening infections in susceptible patients [–]. Invasive medical procedures and use of broad-spectrum antibiotics increase the risk for A. baumannii infection. Consequently, episodes of A. baumannii bacteremia occur most frequently in patients admitted to intensive care units. Other reports indicate that the most common pathogens responsible for prosthetic valve endocarditis are of nosocomial origin, such as coagulase-negative staphylococci, Staphylococcus aureus, or Gram-negative bacilli []. Prosthetic valve endocarditis due to Acinetobacter is rare, and the present case of late bioprosthetic prosthetic valve endocarditis by A. baumannii is very unusual.\nOlut and colleagues presented a case of early prosthetic valve endocarditis due to A. baumannii that was accompanied by a cutaneous eruption. In this case, a 6–7 mm vegetation was present on the aortic valve. Although appropriate antibiotics were started immediately, the patient died of septic shock and disseminated intravascular coagulation []. Menon and colleagues reported a case of infective endocarditis caused by A. baumannii complex in a 27-year-old male who underwent surgical repair of a ruptured aneurysm of the sinus of Valsalva one month previously. This patient died of sepsis before appropriate antibiotic therapy could be started []. Kumar et al. reported a 23-year-old female who underwent surgical replacement of the mitral valve and developed late endocarditis of the mechanical prosthetic valve due to A. baumannii complex. This patient, who received surgical replacement with a Starr-Edwards mechanical prosthetic valve 5 years previously, was treated with ofloxacin and amikacin and was soon afebrile []. Gradon et al. reported community-acquired infective endocarditis of a native valve that was caused by A. calcoaceticus subspecies anitratus. They further reported that 5 of 15 previously described patients with native valve endocarditis and 1 of 6 with prosthetic valve endocarditis died. These authors recommended therapy with antimicrobial agents known to be active against Acinetobacter [].\nIn our patient, bioprosthetic tricuspid valve endocarditis developed six years after surgery, similar to the patient reported by Kumar et al. Although our patient’s symptoms and signs of cardiac insufficiency were initially misinterpreted, diagnosis was firmly established following isolation of A. baumannii from blood cultures. After admission, transthoracic echocardiography confirmed the presence of large vegetations attached to bioprosthetic tricuspid valve, which were not noted in her initial visit to a local hospital. Generally, imipenem is active against A. baumannii, but this species has developed resistance to multiple antimicrobial agents, such as imipenem, penicillin, ampicillin, clindamycin, and linezolid. Fortunately, the usual treatment for this patient is an active β-lactam alone, preferably one with a limited spectrum. Thus, we administered intravenous cefoperazone sodium and sulbactam sodium for about two months, a treatment that was successful.\nThe choice of the type of tricuspid prosthesis remains a matter of debate. In our patient’s second operation, a biological prosthetic valve was used in anticipation that this valve would have a slower degenerative evolution for the lower pressure stress in the right heart and was less likely to cause a thrombotic event [–]. There is no evidence that biological prosthetic valves are more likely to cause endocarditis. However, based on echocardiography, large vegetations were attached to the biological prosthetic tricuspid valve that damaged the biological leaves and led to severe tricuspid regurgitation. This necessitated surgery and valve replacement and the patient chose a mechanical valve for this third operation. Similar to many other reports on the outcomes following surgery for tricuspid valve infective endocarditis, we obtained a favorable result [, ]. Following surgery and intravenous antibiotic therapy, the patient’s symptoms of cardiac insufficiency had resolved. |
A 36-year-old male with no relevant past medical history initially presented with chronic diffused bony pain mainly around the knee and elbow joints with on and off generalized body pruritus. These complaints had been present for the last 4 years with worsening reported in the previous 2 months and developed blurred vision and exophthalmos with difficulties in walking.\nThe patient had been investigated in multiple hospitals with different subspecialties with no definite diagnosis. The patient received analgesic and vitamin D for bone pain, topical light steroid creams and intravenous antihistamine injections for generalized body pruritus after each flare-up.\nOn admission to our department (2016), the patient's general appearance was normal and hemodynamically stable. The initial laboratory workup revealed a normal WBC count and differential, normal hemoglobin and mild thrombocytosis (120 × 109/L). Renal and liver functions were normal. Tumor markers and autoimmune and rheumatologic screening were negative for all serologic markers. The inflammatory markers such as C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) were mildly elevated.\nThe patient underwent radiological studies, with bilateral X-ray of the knees demonstrating bilateral sclerotic changes in the femoral and tibial bones. Humeral X-ray showed intramedullary sclerosis at the distal end of humeral bone. The lateral view showed involvement of the proximal ulnar and radial bones without pathological fracture (Fig. ). 99mTc-methylene diphosphonate and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed bilateral symmetric uptake in the long bones, right clavicle, and mandible as well as subcutaneous and muscle soft tissue involvement in the lower limbs. Computed tomography (CT) and MRI for the lower limbs revealed multifocal intramedullary sclerosis affecting both legs and distal portions of both femoral bones. Pituitary involvement and retrobulbar soft tissue infiltration were demonstrated as enhancing lesions on MRI and high FDG uptake on PET/CT brain images. Fused PET/CT images and CT images demonstrated bilateral perinephric soft tissue thickening and uptake (Fig. ).\nAccordingly, CT-guided left proximal tibia bone biopsy was performed. Histological findings of the bone biopsy reported as diffuse infiltration by foamy histiocytes and fibrosis and rare Touton-like giant cells with immunohistochemical (IHC) profile consistent with ECD with no granuloma and negative for malignancy. On IHC staining, the histiocytes were positive for CD68, CD163, and factor XIIIa, and negative for CD1a, S100, and langerin (Fig. ).\nOnce the diagnoses of ECD was confirmed and because of its aggressive nature with involvement of the orbit disturbing the patient's visual function with marked bilateral exophthalmos and gradual loss of vision, the patient underwent decompression surgery to alleviate the tissue pressure over the optic nerve, which resulted in improvement of the visual acuity.\nThe treatment started with pegylated IFN-α 180 μg/week subcutaneously. Treatment was well tolerated and particularly efficacious for bone pain and constitutional symptoms without adverse effect and the compliance of the patient was excellent. The pain started to decline with time till disappearance by the second month of treatment, platelet count returned to normal range, and ESR and CRP deceased but were still elevated; however, they were less than the prior treatment level. The patient did not require analgesia as before the treatment.\nFDG-PET and CT scan were performed after 6 months of the beginning of treatment and showed stabilization or improvement of the intraorbital and suprasellar enhancing lesions and other radiological findings detected at diagnosis which were considered as partial good response as disease stabilization was achieved and the patient's clinical status was improved. |
A 6-year-old male patient complaining about a growing, painful swelling over the right parotid and submandibular area, was referred to our department for proper evaluation and management. This longitudinal mass had been noticed approximately one year ago. It remained rather small and stable until two months prior to evaluation, at which time it entered a rapid growth phase, nearly doubling in size. As a result, the patient reported mild pain radiating into the periauricular region. Medical and familial history did not reveal significant findings and no previous trauma was reported.\nPhysical examination included a full head and neck evaluation. Palpation indicated a right-side parotid mass, with a contiguous nodular lesion, approximately 3 cm in diameter. The lesion was painful, noncircumscribed, nonpulsating, rather soft, and of a rubbery consistency while adherent to deep planes. Some smaller nodules appeared at the right postauricular and submandibular region. Due to its size and location, the mass created serious facial disfigurement and asymmetry, with no clinical evidence, however, of any facial weakness or facial nerve impairment. An overall clinical examination also revealed randomly distributed cutaneous café au lait spots on the patient's trunk and extremities (more than 6 and the largest being over 15 mm in diameter). Although these are typical signs of NF-1 we found no other indication of the syndrome such as Lisch nodules or cutaneous neurofibromas.\nOn these grounds, a gadolinium enhanced magnetic resonance imaging (MRI) was ordered. Imaging confirmed the presence of the mass and localized the lesion to the superficial lobe of the parotid gland, suggesting a neurofibroma adherent to, and possibly originating from, the intraparotid portion of the facial nerve (). Fine needle aspiration for cytology (FNA) was performed but was inconclusive.\nAfter informed consent was obtained, the patient underwent a typical subtotal superficial parotidectomy, under general endotracheal anesthesia. Intraoperatively, the lesion was found to be a well delimited, tan colored mass, of a gelatine-like consistency which was strictly adherent to the main trunk of the facial nerve. However, accurate tissue-conserving dissection indicated that although in close contact/proximity, it strangely did not originate from the facial nerve. Instead, the neurofibroma seemed to stem from what was identified as the intraparotid branches of the auriculotemporal nerve. Intraoperative view also revealed that longstanding compression to the trunk of the facial nerve had moved the division to temporofacial and cervicofacial branches far more anteriorly than expected, thus hampering dissection (). Despite altered anatomy and heavy tumor vascularization, we achieved tumor dissection and excision while ensuring facial nerve integrity.\nFor histologic examination a nonencapsulated, multilobulated mass measuring 6 × 4.2 × 3 cm and weighting 20 grams was sent to the Pathology Department. Overall, the mass was soft with nodular areas, resembling a “bag of worms.” The cut surface of the mass had a gray-white, glistening appearance, without foci of hemorrhage or necrosis (). Hematoxylin and eosin stained sections showed a lesion composed of bundles of nerve fibers infiltrated by Schwann cells, fibroblasts, and perineural-like cells encompassed partly by perineurium. The neoplastic cells were arranged in a fascicular pattern of growth and had a serpentine configuration with elongated nuclei with pointed ends (). Neither increased mitotic activity nor atypia were present. The stroma exhibited extensive myxoid degeneration with the presence of strands of collagen and inflammatory aggregates composed of lymphocytes, plasma cells, eosinophils, and scattered mast cells. The neoplasm penetrated the parotic gland thus destructing the normal lobular architecture of the gland (). Despite the extensive sampling, no areas of malignant transformation were observed. Interestingly, the neoplasm penetrated an intraparotid lymph node through the hilum followed by blood vessels (). The final histopathologic diagnosis was intraneural plexiform neurofibroma.\nOverall postoperative course was uneventful and no significant complications were noted, except for a mild transient facial paresis which was fully resolved during postoperative day 1. Cosmetic disfigurement was restored and periauricular pain disappeared. The patient was discharged after 48 hours. Six months later, he remains free of symptoms, postoperative complications, or signs of tumor recurrence. |
The patient was a 4-week-old male infant born at an estimated gestational age of 30 weeks with a birth weight of 1665 grams and APGAR scores of 8 and 9 at one and five minutes, respectively. Gross congenital anomalies were not identified. The mother was a gravida 3, para 2, abortion 1, 21-year-old African American with an adequate prenatal care. Her pregnancy was complicated by group b streptococcus urinary tract infection and placental abruption requiring pregnancy termination. In addition, there was a history of hand-foot-mouth syndrome in his sibling two weeks prior to delivery. Immediately after birth, the neonate developed respiratory distress and cyanosis requiring intubation and admission to the neonatal intensive care unit. The initial physical exam revealed a grade III-IV holosystolic murmur and generalized edema. The initial X-rays and echocardiographs demonstrated a moderate to large patent ductus arteriosus (PDA), moderate to severe pulmonary hypertension, and biventricular changes suggestive of hypertrophic cardiomyopathy. Bedside PDA ligation was performed. Subsequent studies revealed Coxsackie B viremia (confirmed by polymerase chain reaction (PCR)); however, TORCH serology were all negative. Viral induced hypertrophic cardiomyopathy was the presumptive diagnosis. However, daily chest X-rays demonstrated progressive nonspecific calcification of the chest soft tissue and the vessels of both arms (). Despite multiple intravenous immunoglobulin treatments, loop diuretics, phosphodiesterase 3 inhibitor, and dopamine for the presumed viral induced cardiomyopathy, his symptoms progressed and the patient expired at 4 weeks of age secondary to intraventricular brain hemorrhage and multiorgan failure. At autopsy, there was severe anasarca, but gross congenital anomalies were not identified. Cardiovascular dissection revealed increased biventricular wall thickness () with evidence of gross arterial calcification along the large vessels. Microscopic sections of the heart demonstrated cardiac myocyte nuclei enlargement consistent with hypertrophy and focal calcium deposition and fibrosis within the myocardium. However, inflammation suggestive of myocarditis could not be identified (Figures and ). The coronary arteries demonstrated focal calcification of the internal elastic lamina (), while sections from the ascending and descending aorta exhibited extensive calcification with disruption of the elastic lamina and intimal fibrosis (). Other organs including lungs, brain, pancreas, kidneys, skin, muscles, and testicles also displayed extensive calcium deposition involving the internal elastic lamina with ingrowth of loose fibromyxoid tissue resulting in luminal narrowing and obstruction. The renal hilar vessels were extensively involved (). The gross and microscopic features were consistent with the diagnosis of IIAC. In this case, Coxsackie B viremia and a history of hand-foot-mouth syndrome in the sibling as the etiology of the cardiopulmonary failure were a red herring. However, the single nucleotide polymorphism microarray analysis indicated no clinically significant abnormality and was consistent with a normal male chromosome complement, but the gene sequencing to evaluate the presence or absence of ENPP1 gene mutation was not performed. This case report aims to emphasize the importance of taking into consideration this rare but curable disease in infants presenting with refractory cardiopulmonary failure. |
A 66-year-old Caucasian man with metastatic melanoma and unresectable, locally advanced pancreatic adenocarcinoma was admitted to the intensive care unit (ICU) with an anaphylactoid reaction following the attempted biopsy of a thyroid nodule.\nThe melanoma had initially been diagnosed by shave biopsy of the back in September 2015. The patient underwent wide excision in October 2015. He then developed painful axillary lymphadenopathy, which a fine-needle aspiration biopsy (FNAB) in March 2016 confirmed was recurrent, metastatic melanoma with the BRAF V600E mutation. His pancreatic malignancy was initially discovered during staging for melanoma on axial imaging of the chest, abdomen, and pelvis in October 2015. A large, low-density infiltrative pancreatic mass was demonstrated on computed tomography (CT), along with tumor involvement of the splenic and hepatic arteries and portal, superior mesenteric, and splenic veins. The diagnosis of pancreatic adenocarcinoma was confirmed by sampling via endoscopic ultrasound. He had received two cycles of chemotherapy with gemcitabine and protein-bound paclitaxel, though this had been temporarily stopped due to gastrointestinal side effects. A hereditary disorder such as melanoma-pancreatic cancer syndrome was postulated, although not confirmed by genetic testing.\nDuring a re-staging positron emission tomography (PET) scan in April 2016, increased metabolic activity with maximum standardized uptake value (SUV) of 14 was seen in the right thyroid, corresponding to a moderately hypervascular nodule measuring 3.1 x 2.8 x 3.8 cm by ultrasound (Figure ). This was suspicious for malignancy. At this time, the patient had normal thyroid function tests and was clinically euthyroid.\nThe patient opted to undergo further diagnostic evaluation of the thyroid nodule by fine needle aspiration biopsy (FNAB) in August 2016. Following tissue infiltration with 1% lidocaine to the anterior neck at the beginning of the procedure, the patient became tachycardic and hypotensive. The procedure was aborted and the patient was transferred to the ICU.\nUpon arrival to the ICU, he was afebrile with an initial heart rate of 131 beats per minute, blood pressure 66/42 mmHg, and saturating 98% on room air. He complained only of abdominal discomfort and new diarrhea. He denied knowledge of an allergy to lidocaine. His exam was notable for an older man in no apparent distress, with non-tender and benign abdominal exam. His initial laboratory evaluations were notable for leukocytosis (white blood cell count of 18.6 x 109/L), and serum lactic acid of 2.4 mmol/L. The patient received intravenous fluids with improvement in his tachycardia and hypotension. Further infectious evaluations were negative, including blood cultures, urine culture, stool culture, stool ova and parasite, C. difficile toxin polymerase chain reaction (PCR), and chest x-ray. Laboratory evaluations on the second day of admission demonstrated normalization of his WBC count, but revealed evidence of acute kidney injury (serum creatinine 2.2 mg/dL, with a prior normal baseline), with the latter attributed to prerenal azotemia from his hypotensive episode. The patient did not require antibiotics, nor did he require vasoactive agents. He was discharged home on hospital day two after improvement and stabilization in his clinical status. On repeat laboratory assessment two weeks later, his renal function had normalized.\nUpon follow up with his oncologist, he was started on immunotherapy for melanoma with nivolumab after discussions of the risks and benefits. Further evaluation of the thyroid nodule was not pursued. |
A 33-year-old woman, gravida 3, para 3, non-obese and with no chronic diseases, underwent TLmRH as curative treatment for clinical stage IA1 cervical cancer. Two months later, she presented to our department with abdominal pain and genital bleeding after her first sexual intercourse after surgery from the previous day.\nWe sutured the vaginal cuff with absorbable sutures during initial surgery. We performed colpotomy with ultrasonic device and monopolar device in both cases.\nBoth patients were immediately diagnosed with VCD (Figure ). The prolapsed organ was found to be the intestine and it remained within the vagina without evisceration out of the vagina. The color of the intestine was normal, indicating that there was no ischemia present. After washing of the prolapsed intestine, we pushed back the prolapsed intestine, with sterilized gauze to prevent herniation outside of the vagina until operation. Vaginal approach repair (repair from the vaginal cavity), open approach repair, or laparoscopic approach repair were treatment choices. Suturing from the vagina could shorten vaginal length. To prevent recurrence, we thought it would be better to suture the peritoneum. We already resected the vagina about 2 cm in the initial surgery in both cases; thus, we would like to avoid further shortening. We thought that the open approach should be avoided considering its invasiveness if we could safely avoid this complication laparoscopically. Thus, we initially employed total laparoscopic repair.\nThe ureter was separated from the paravaginal tissue during initial cancer surgery, losing its normal anatomical position. This may cause ureteral damage during repair (Figure ). Thus, in order to avoid ureteral injury and to create a tight suture, the vaginal wall was separated by 1.0 cm, to the extent that concrete vaginal cuff suture could be made (Figure ), and suturing was complete (Figure ). The peritoneum was sutured to prevent recurrence, hematoma, and infection. The postoperative course was good. The cuff remained intact at 1-, 2-, 3- and 6-month examination. We suggested that sexual intercourse is safe 6 months after surgery.\nInterviews conducted on these cases after the 6-month examination revealed that normal sexual intercourse was performed without troubles in both cases. |
A 10-year-old girl visited our affiliated hospital, complaining of pudendal deformity. The patient was born at 39 weeks of gestation by normal delivery as the second child, and the birth weight was 3,144 g. There was no disorder in the course of pregnancy in her mother. Intake of androgenic medicine or the disorders of ovaries and uterus was not observed by a periodic medical examination. No pudendal deformity was clearly observed at birth, but lateral asymmetry of the pudendal region was noticed at about 4 years old. A child care worker pointed out that she pressed her heel to the crotch while sitting on her folded legs. After 5 years old, her mother confirmed that her clitoral hood clearly hypertrophied. After entering primary school, a teacher in charge pointed out that she pressed her crotch to a chair or bar, and the mother told her to stop it, but she repeated this behavior every day. When she strongly wanted to be absent from an overnight school trip at 9 years old, the mother brought her to the Pediatric Department. Various tests were performed suspecting disorders of sex development (DSD). On the first visit, the height was 132 cm and the body weight was 26 kg. The clitoral hood was enlarged. The appearance was similar to the vulva in children with congenital adrenal hyperplasia, and the clitoris size was 8 x 5 mm. Labial fusion or adhesion was not detected, and the urinary tract and vagina were open at the normal positions. No masculinization, such as acne and polytrichosis, was noted (). Intake of androgenic medicine or the disorders of prepuce was not observed. In the blood test, the sex chromosome was 46, XX. The blood count, blood chemistry, and hormonal test were normal (). On abdominal ultrasonography, the uterus and ovaries were present. Abdominal CT and MRI examinations showed no tumorous lesion.\nBased on the above examination and test findings, DSD was considered negative. The patient was diagnosed with clitoral hood enlargement and referred to our department to undergo clitoral hood reduction. For surgery, a longitudinal incision was designed for the dorsal side in order to resect the clitoral hood by cut and try. In the clitoral region, the clitoral hood and corpus cavernosum were dissected through an inverted V-shape incision. The volume of the exposed corpus cavernosum clitoridis was reduced while conserving the neurovascular bundle, following the Marberger method []. The clitoral hood was resected into a triangle shape and used for labial formation. On histopathological examination, lymphedema and venous tasis in a grade consistent with the influence of foreskin excision were observed. No abnormality was noted in the corpus cavernosum. Her postoperative course was uneventful. As of 10 months after surgery, favorable improvement of the appearance was noted (). |
Two months ago, a 72-year-old woman presented to her primary care physician with an episode of melena that was suggestive of hemorrhage of the upper gastrointestinal tract. Upon presentation the patient was hemodynamically stable with normal laboratory tests and no evidence of active bleeding in the last 48 hours. Hospitalization was not required and the evaluation was completed in the outpatient department.\nThe patient was subjected to further investigation. Upper gastrointestinal endoscopy revealed mild, diffuse oesophagitis and a small sliding hiatal hernia. At the antrum of the stomach, a 5 cm, well circumscribed submucosal mass with normal overlying mucosa was observed (Figure ). Multiple regular biopsies were taken and some histological features of leiomyoma were identified. An endoscopic ultrasound confirmed the submucosal lesion which originated from the muscularis propria, measured 1.9 × 2.4 cm and was extending in the second, third and fourth layer of the stomach.\nThe patient was subsequently referred for surgical consultation. Physical examination revealed a 72-year-old female who was awake and alert, appeared healthy and looked younger than her stated age. Her abdomen was soft, non-distended, without palpable masses. The stool was negative for occult blood. The hemoglobin level was 13.1 g/dL with normal biochemical profile. Tumor markers were within reference ranges. Abdominal radiography showed normal amount and distribution of gas within the bowel. An abdominal computer tomography scan demonstrated a 3 cm localized, prepyloric enhancing mass at the lesser curvature of the stomach (Figures ). Lymphadenopathy was not observed. The differential diagnosis involved mesenchymal and other benign gastrointestinal stromal tumors.\nThe patient was taken to the operative room electively. She was subjected to antrectomy and Roux-en-Y anastomosis. The stomach contained a 2 × 2 × 1.7 cm well circumscribed tumor. (Figures ). The histopathologic findings of the lesion were characteristic of glomus tumor of the antrum. In detail, cut surface of the specimen, demonstrated a grayish-white nodular tumor, arising from the submucosa and extending through the muscularis of the stomach, without involving the serosal surface. Histologically, the tumor was composed of sheets of glomus cells, without nuclear pleomorphism and no mitotic figures. The cells had eosinophilic and focally clear cytoplasm. Throughout the tumor telengiectatic vessels were observed and some contained aggregates of glomus cells in their walls (Figure ). Immunohistohemically, the tumor cells were positive for smooth muscle actin (Figure ) and vimentin and negative for desmin, CD34, CD117, S-100 protein and cytokeratins (AE1/3, CAM 5,2). The proliferating marker Ki-67 was < 5%. The residual gastric mucosa showed atrophic gastritis with focal intestinal metaplasia in the pylorus region. Five lymph nodes retrieved from the major omentum were free of metastatic tumor. The patient recovered uneventfully and was discharged 5 days after surgery. |
A 78-year-old male, who had been a patient of the osteopathic family clinic for over 10 years, presented with his wife to discuss a pre-surgical assessment for the placement of an intracranial shunt. He had a past medical history of diabetes mellitus, hypertension, hyperlipidemia, and a stroke in 2013. The stroke resulted in left hemiparesis affecting the lower extremities more than the upper extremities but was followed by a complete recovery. For the past two years, he developed a progressive short-stepped shuffling gait with hesitancy occurring with turning and swaying only to the right side while walking. Additionally, he described nocturia with increased bladder frequency and urgency and subjective memory difficulty without any mental impairment. The patient ambulated with a cane and stated that his biggest concern was swaying to the right with a fear of falling. He described difficulty in turning and freezing due to this feeling of loss of balance. The patient denied dyskinesias, hallucinations, nightmares, tremors, headache, or nausea. His most recent MRI suggested stable mild-to-moderate diffuse ventriculomegaly. He was referred to a neurologist at NYU Langone who diagnosed NPH and subsequently was referred to a neurosurgeon at Columbia Presbyterian who deemed the patient a candidate for a surgical shunt.\nDue to his hesitancy toward the recommended invasive procedure, the patient agreed to try craniosacral therapy to relieve the NPH prior to getting the shunt recommended by the neurosurgeon. The patient tolerated the first osteopathic therapy session well and returned to the office one week later. He stated that he noticed a significant improvement with his gait two to three days after the first session, but symptoms had slightly returned towards the end of the week. A second craniosacral session was performed, and the patient felt an improvement in his NPH symptoms after the therapy.\nCraniosacral therapy sessions were conducted one week apart lasting 15 minutes. Upon the third session, the patient stated that his gait had significantly improved and turned 90 degrees quicker without significant hesitation. The patient continued to ambulate with a cane but was able to walk faster and felt more comfortable in crowded environments which he had not felt before. The patient also stated that his bladder urgency improved slightly over the previous three weeks. The remaining sessions were spaced two weeks apart until only monthly sessions were necessary to help alleviate symptoms concerning gait.\nAn MRI was performed in March 2021, which was three months after the initial relief of symptoms from craniosacral therapy. This was compared to a previous MRI performed in February 2020, which was nine months prior to receiving osteopathic treatment. A neuroradiologist was consulted, and the imaging results showed no significant change in the ventriculomegaly. Yet, the patient agreed that symptoms were diminished after craniosacral therapy. According to a study of patients that received a shunt, imaging plays an important role in the diagnosis and follow-up, but the ventricular size or degree of atrophy on subsequent MRIs does not correlate with patient response and symptoms []. A normal axial T2 MRI, the patient’s MRI performed on February 4, 2020, and the patient’s MRI performed on March 17, 2021, are shown in Figures -). |
A 68-year-old asthmatic male patient presented to our center with 12 days history of melena. He denied any previous episode of melena or hematochezia or bleeding from another site. The patient did not have any other associated symptom, and had no other co-morbidities, or medication use. Upon referral, he was uncomfortable and looked pale. He was vitally unstable, with a blood pressure of 90/60 mmHg and a pulse rate of 120 beats/min. The examination revealed the presence of clotted blood on the anal verge, and some tarry stool on digital rectal examination. The hemoglobin level was 7.7 g/dl, the hematocrit was 22.8, and the blood urea nitrogen was 8 mg/dl. The prothrombin time and the partial thromboplastin time were normal.\nResuscitation was performed with transfusion of 2 units of packed red blood cells and intravenous fluids. He was admitted to the ICU for intensive monitoring. After admission and stabilization, upper and lower endoscopies were performed without demonstrating the bleeding site. They only revealed clotted and red blood throughout the colon.\nTechnetium-labeled red blood cell bleeding scan was done to localize the site of bleeding. This scan showed no evidence of early focal increased uptake in the abdomen to indicate active gastrointestinal bleeding during early images, but in the delayed images, it revealed that there was a focal uptake in the right and transverse colon. After that, capsule endoscopy was also performed without findings. As the angiography became available, the patient underwent selective angiography without findings noted at that time. These tests were inconclusive because they were performed while the episodes of bleeding ceased.\nAfter 8 days of conservative management and negative investigations to define the cause of the bleeding, a sudden drop in hemoglobin level from 10.8 mg/dl to 6.9 mg/dl occurred over 12 h, which mandated operative management. Exploratory laparotomy was performed. Extensive jejunal saccular pouches were found 10 cm distal to duodenojejunal junction extending 1.6 m distally Fig. . The bleeding was difficult to control and the decision to clamp the major branched was performed. Division of the small bowel proximal and distal to the diseased part using gastrointestinal stapler was performed with side to side primary anastomosis Fig. . The specimen was a part of small bowel, 117 cm in length, with congested wall and multiple pouches at the mesenteric site. Opening of the specimen showed normally looking mucosa with active bleeding that stopped after awhile. No polyps or masses were detected. We reviewed the angiography achieve after that and a suspicious shadow reflecting the diverticular outpouching was detected.\nMicroscopic examination of the specimen revealed many diverticula; some of which being true diverticula, while the others are devoid of muscularis propria (false diverticulae). Within the diverticula and in the intervening portions of the bowel wall, there were numerous dilated thick- and thin-walled small blood vessels in the submucosa. Additionally, submucosal intermediate-sized vascular clusters and feeder vessels in the muscularis propria and serosa were present. The overall features were those of small intestinal diverticulosis and arteriovenous malformations. The latter involves the diverticula and intervening portions of the bowel wall Fig. .\nPostoperatively, the patient was doing well, discharged home on day 5 postoperatively, with an uneventful postoperative course. He was followed up 2 years after that without complications. |
A 19-year old man presented to the emergency department, following exposure to a bomb blast injury. He had a patent airway, but he was tachypneic, agitated, confused, and pale. His blood pressure was 70/30 mm Hg and pulse rate 120 per minute. The right radial pulse was impalpable due to a compound fracture of the arm. There was a 4-cm wound in the right sixth intercostal space in the midclavicular line. The abdomen was tense and tender all over. Abdominal ultrasonography was positive for hemoperitoneum. A right-sided intercostal drainage tube was inserted for hemopneumothorax.\nExploratory laparotomy was carried out. Two liters of blood were found in the peritoneal cavity. The liver was lacerated and actively bleeding. The gallbladder was found injured; cholecystectomy was done. Suturing of the liver did not control the bleeding. Thus, a decision was made to insert perihepatic packs. Two gastric perforations were repaired. A 6 cm × 3 cm × 2 cm shrapnel was found anterior to the first part of the duodenum (). A drain was left near the Morrison's pouch.\nThe vascular surgeon explored the lacerated wound of the right arm and found that the ulnar artery was completely transected at the bifurcation of the brachial artery. Reconstruction was accomplished using a brachio-ulnar reversed saphenous vein graft.\nOn the first postoperative day one liter of blood was recovered from the abdominal drain. On the third postoperative day and before shifting the patient to the operating theatre for packs removal, CT of the abdomen was done; showed a 3 cm × 1.8 cm IHPA of the left hepatic artery (). Hepatic angiography confirmed the presence of the IHPA emerging from a side branch of the left hepatic artery (). Microcoil embolization of both afferent and efferent loops of the aneurysm was done (). The perihepatic packs were removed on the same day. There was mild oozing from the hepatic injury site, which was easily controlled with TachoSil® Fibrin Sealant Patches ().\nPostoperatively, the abdominal drain started to bring bile stained fluid in large amounts, so Somatostatin Analogue, (Octreotide, Novartis Pharmaceuticals Corp), injection was started at a dose of 100 mcg, SC TID, in attempt to decrease the high output biliary fistula. On the sixth postoperative day, the patient developed upper gastrointestinal bleeding. Gastroduodenoscopy revealed mild oozing from the sutured edges of the previously repaired gastric perforations, but the procedure was complicated with an iatrogenic gastric perforation. A follow-up CT showed an extra luminal leak of the oral contrast from the stomach with a sizeable subhepatic collection, right-sided hydropneumothorax, and right lung collapse (). We did not notice much of gastric leak in the drain during the subsequent hours, may be because of the Octreotide effect, so we decided to keep the patient NPO, and we started Total Parenteral Nutrition (TPN), aiming to treat both gastric and biliary fistulas conservatively.\nThree days later, the intercostal drainage tube started to bring bile. Hence, biliopleural fistula was diagnosed. As the patient had no significant leukocytosis and no fever at that time, we continued the conservative management with TPN and Octreotide.\nAs the right-sided pleural effusion had transformed into a well-formed empyema, decortication was performed (). Eight weeks after admission, the patient was discharged home. He was followed up for one year; no late complications were encountered. Follow up CT showed normal looking both lung and liver parenchyma with no evidence of recurrence of the IHPA (). |
A 56-year-old male, 168 cm tall and weighing 71 kg, with a traumatic right rotator cuff injury, was scheduled for arthroscopic reconstruction surgery under general anesthesia. This patient had a history of diabetes mellitus and was taking oral hypoglycemic agents and insulin. For several years, he experienced recurrent episodes of severe left ocular pain and increased IOP, which later resolved. On close ophthalmic examination, he was diagnosed with Posner-Schlossman syndrome. There was mild epithelial edema of the cornea with a few keratic precipitates, and his cup/disc ratio was 0.5 with no visual field change. During the acute crisis period, the measured IOP in his left eye was above 30 mmHg and the IOP in his right eye was 13 mmHg. His symptoms were relieved after applying eye drops containing dorzolamide, a carbonic anhydrase inhibitor, and prednisolone acetate, but frequently recurred. Three weeks before his scheduled surgery, the IOP in his left eye rose again and he became extremely anxious about the operation. After consultation with an ophthalmologist, we decided to measure the patient's IOP intraoperatively using portable tonometry, and obtained informed consent from the patient for this perioperative risk.\nPreoperative laboratory findings, including hemoglobin concentration, liver function and coagulation tests, and arterial blood gas analysis, were normal, as were chest X-rays. The patient had a left axis deviation and an incomplete right fascicular block on electrocardiography and a mild restrictive pattern on pulmonary function test without any significant symptoms. In the morning of the day of surgery, the patient received 7.5 mg of midazolam per os. Before entering the operating room, his vital signs were stable, with a blood pressure of 133/75 mmHg, a heart rate of 82 beats per min, and an oxygen saturation of 99%. Two drops of 0.5% proparacaine hydrochloride were applied to each eye, and IOP was measured by portable tonometry (Tono-Pen Avia, Ametek, NY, USA) (). Initially, IOP was 19 mmHg in his left eye and 16 mmHg in his right eye. A 20-gauge catheter was inserted into his left radial artery to monitor continuous arterial pressure. Anesthesia was induced with propofol 100 mg, lidocaine 75 mg, rocuronium 50 mg, and a supplemental dose of fentanyl 100 µg, and ventilation was controlled to maintain end tidal carbon dioxide at 35 ± 5 mmHg. Anesthesia was maintained with 2-2.5% sevoflurane in an oxygen-air mixture (FiO2 0.5). Immediately after tracheal intubation, IOP increased to 23 mmHg in his left eye and 21 mmHg in his right eye, but decreased within 5 min to 14 mmHg and 12 mmHg, respectively. Initial arterial blood gas analysis showed an Hct of 39%, a pH of 7.48, a PaCO2 of 34 mmHg, and a PaO2 of 240 mmHg. After changing the patient's position to left lateral decubitus, the IOP was 15 mmHg in his left eye and 14 mmHg in his right side, but increased to 20 mmHg and 14 mmHg, respectively, after skin incision and arthroscopic insertion. The concentration of inhaled sevoflurane was adjusted in response to instantaneous IOP variation, and the occurrence of IOP surge was closely monitored. During the 210 min operation, the patient's vital signs remained stable and no adverse event occurred. Serial measurements by portable tonometry showed that his IOP remained within an acceptable range throughout the entire perioperative period (). The patient's postoperative course was uneventful. |
A 31-year-old female, gravida 2, para 2 (G2 P2), referred to the gynecologic clinic with a main complaint of secondary infertility during the last twelve months. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. The patient had two previous C-sections. Her menstrual cycles were ovulatory. Spermogram was unremarkable and hormonal assay did not show any abnormality. HSG was performed and revealed a migrated copper IUD with its long tail out of the uterine cavity (Fig. ). Plain abdominopelvic radiography also indicated a rotated T-shaped IUD in the pelvis (Fig. ). A transvaginal ultrasound was also performed by a skilled radiologist who reported a copper IUD in the bladder lumen with a small portion of it within the bladder musculature. The patient had taken multiple courses of antibiotics for urinary tract infection (UTI), but her symptoms never disappeared. The patient also underwent cystoscopy for recurrent infection last year, but no pathological finding was detected. Eventually, the patient said that her symptoms were related to the possible adhesions following two previous operations. The patient had a history of IUD insertion following her last C-section about eight years ago. After three years, however, the patient decided to remove it due to recurrent vaginal secretions. Three years later, her IUD was expelled, and another copper IUD was inserted. The patient removed the other copper IUD for becoming pregnant about two years ago. The patient was very confident of its removal, but her recent HSG, interestingly, demonstrated a rotated copper IUD in the pelvic cavity. Physical examination was essentially normal. A baseline complete blood count, urea, and creatinine levels were normal. The patient was candidate for a hysteroscopic cystoscopy. Hysteroscopy was normal except for a small fibrotic defect at the lower segment of the uterus. During cystoscopy, the long tail of the copper IUD was found on the posterolateral border of the bladder far from ureteral offices (Fig. ). The two short arms adhered to each other were embedded in the mucosal and muscular layer (Fig. ). No calculus was observed within the bladder cavity. Using glycine as the media, a mono-polar loop entered the bladder. A gentle and brief cautery was applied on the mucosa where the shadow of a short arm of the device was observed. The copper IUD was safely removed through the urethra using a special grasper. There was no major defect in the place of the copper IUD in the bladder. The bleeding points were effectively cauterized (Fig. ). The patient was discharged the next day with an indwelling catheter. After catheter removal, the patient did not complain of any urinary symptoms. |
A previously healthy 10-year-old Asian girl presented to the emergency department with headache, vomiting, and one week of mild nonproductive cough. Her headache started the evening prior to presentation, was gradual in onset and frontotemporal in location, and improved with acetaminophen but subsequently woke her from sleep. It was accompanied by two episodes of emesis. On presentation to the ED, the patient described her headache pain as 3 out of 10 in severity. She denied photophobia, had no further nausea, and denied abdominal pain. She reported that the headache worsened with standing and improved with lying down. Review of systems was significant only for pallor.\nThe patient was otherwise healthy with no prior medical issues and taking no regular medications. She was fully vaccinated and had no known allergies. Her family history was significant for frequent headaches in her mother and maternal aunt. She was living with her parents and brother and attending 4th grade.\nVital signs demonstrated blood pressure 111/56, pulse 104, temperature 37.1°C, respiratory rate 22, and oxygen saturation 100% on room air. Initial exam revealed a well-appearing female and was unremarkable including a normal fundoscopic exam and a normal complete neurologic exam.\nThe patient received ibuprofen and oral rehydration and her headache further improved. A presumptive diagnosis of migraine headache was made and was discharged with primary care follow-up the following day.\nTwo days after her initial emergency department visit, the patient returned to the ED with worsening headache, myalgia, subjective fever, and diffuse weakness. The patient's mother reported that the patient was unable to stand or walk and as a result her mother had been carrying her, including to and from the bathroom. The patient endorsed nausea but no further vomiting.\nVital signs demonstrated blood pressure 105/49, pulse 123, temperature 36.9°C, respiratory rate 30, and oxygenation saturation of 97% on room air. On exam, the patient was moderately ill appearing, lying in bed responding slowly to questions. Her lips were noted to be cracked and with some oozing blood. No oral lesions were noted in the mouth. Pupils were equal, round, and sluggishly reactive bilaterally. Neck was supple with no adenopathy noted. Cardiovascularly, she was noted to be tachycardic with a regular rhythm and II/VI flow murmur. Her respiratory exam was normal. Her abdominal exam was benign with no organomegaly. Neurologically the patient was noted to be slow to respond to questions and moving slowly but without focal deficits. She was, however, unable to walk without assistance. Skin exam revealed diffuse ecchymoses on the lower extremities bilaterally.\nLaboratory studies were ordered along with a rapid brain MRI, and pediatric neurology was consulted.\nLaboratory results were as follows: hemoglobin 2.6 g/dL, hematocrit 8.3%, platelets 10K/uL, and WBC 60.5K/uL with 83% blasts in the differential. CRP was 15.7 mg/L, and ESR was 125 mm/h. Electrolytes showed sodium 139 mmol/L, potassium 4.0 mmol/L, chloride 100 mmol/L, carbon dioxide 24 mmol/L, BUN 11mg/dL, creatinine 0.56 mg/dL, glucose 129, magnesium 2.5mg/dL, and phosphorus 3.6 mg/dL. LDH was 359 U/L, uric acid was 2.3mg/dL. PTT was 29.4 seconds, PT was 19.5 seconds, and INR was 1.7. Fibrinogen was 117 mg/dL and D-dimer was >10,000 ng/mL. Review of peripheral blood smear () demonstrated many primitive cells with round and lobated nuclei, numerous cytoplasmic granules with Auer rods readily identified, and some cells with multiple Auer rods.\nRapid MRI Brain () was obtained which demonstrated leptomeningeal enhancement in the supratentorial parenchyma suggestive of leptomeningeal carcinomatosis, a hemorrhagic lesion in the corpus callosum, multiple subdural hematomas with mild mass effect, and petechial hemorrhages throughout the brain.\nPediatric Oncology was consulted and treatment initiated emergently with ATRA, dexamethasone, allopurinol, cefepime, and blood products including packed red blood cells, platelets, and cryoprecipitate. The patient was subsequently admitted to the pediatric intensive care unit.\nIn the pediatric ICU the patient received several transfusions with platelets, cryoprecipitate, and fresh frozen plasma to manage DIC. She was continued on an induction course of chemotherapy including ATRA, dexamethasone, idarubicin, and arsenic trioxide. Molecular analysis of the peripheral blood was positive for PML-RARA, confirming the diagnosis of APL. After the coagulopathy improved and the patient stabilized, lumbar puncture was performed with administration of intrathecal chemotherapy. The cerebrospinal fluid was notable for the presence of leukemia cells, confirming the involvement of the central nervous system. The patient's encephalopathy gradually improved and she returned to her baseline mental status by day 7 of treatment. The patient was noted to have elevated opening pressure on lumbar punctures, which worsened over the course of induction, attributed to pseudotumor cerebri secondary to ATRA.\nUpon completion of the first 28 days of induction therapy, repeat lumbar puncture and bone marrow studies demonstrated no morphologic evidence of acute promyelocytic leukemia, consistent with remission. |
A 20-year-old pregnant mother with a period of amenorrhea (POA) of 28 weeks was admitted to our tertiary care centre due to left-sided severe buttock pain, five days prior to admission. The pain was very severe and she could not even walk due to the pain. She has taken paracetamol as over-the counter-medication and had no satisfactory response. She had a recent history of DHF, which we treated with appropriate fluid management and supportive care, and was discharged eight days before after complete recovery. Initially, she was admitted to a local hospital due to buttock pain for which paracetamol was given as a painkiller, with minimal response. Due to the short duration of her condition and unavailability of facilities, no further investigations were conducted during the local hospital admission. Following her discharge, she experienced the same kind of pain again, after which she was admitted to our tertiary care centre. She did not have other small or large joint pain, swelling and there were no features to suggest enthesitis. She did not have fever, dysuria, vaginal discharge, or skin eruptions. There was no recent history of sore throat or diarrheal illnesses. She also had no past history of joint pain, photo-sensitive skin rashes, oral ulcers, or alopecia. There was no past history of altered bowel habits or contact history of tuberculosis.\nOn general examination, she was not pale or icteric. There was no red eye, malar rash, oral ulcers, or any peripheral stigmata to suggest ongoing vasculitides. Respiratory system examination was normal and did not reveal any features to suggest lung fibrosis. Cardiovascular system examination revealed mild tachycardia (110 beats per minute) and normal blood pressure. There was no evidence of aortic regurgitation or mitral valve prolapsed. Abdomen was non-tender and had no hepato-splenomegaly. The nervous system and peripheral joint examination were normal. X-ray of her sacroiliac joint revealed features of left-sided sacroiliitis which was evidenced by blurring of joint margins with minimal sclerosis of the left sacroiliac joint (see X-rays in Figures -). Full blood count (FBC) revealed white blood cells 14000/µL of which 77% were neutrophils. Hemoglobin concentration was 10 g/dl and platelets 553000/µL. Erythrocyte sedimentation rate (ESR) was 103mm/1st hour and CRP was 9 mg/dl. Dengue IgM and IgG both were positive. Urine full report, liver function tests, renal function tests, and uric acid were within normal range. Blood and urine culture both were sterile. Tuberculin skin test was negative. Antinuclear antibody (ANA) and rheumatoid factor were negative. Retroviral, Hepatitis B and C screenings were negative. Human leukocyte antigen (HLA B27) was negative. Thyroid function tests and iron studies all were within normal limits. We managed her with regular paracetamol and oral tramadol appropriately to alleviate her pain and she gradually improved over the next week. We reviewed her after two weeks with FBC and ESR. At her two-week review, the white blood cell count was down to 12200/µL and platelet count was 365000/µL. Her ESR report was 43 mm/1st hour. After two weeks, significant clinical and biochemical improvement was observed following appropriate symptomatic management. |
A-25-year old male patient reported to the Department of Periodontics, Mamata Dental College and Hospital, Khammam, Andhra Pradesh, with the chief complaint of receding gum and hypersensitivity in relation to lower left lateral incisor (tooth number 32). The patient was a nonsmoker with a good general health and had received no antibiotics and/or periodontal therapy during the previous six months. On intraoral examination, Miller's class II recession was seen in relation to tooth number 32. Trauma from occlusion and tooth malposition with respect to the involved tooth was ruled out clinically. Prior to therapy, clinical measurements including probing depth (2 mm), recession depth (5 mm), recession width (3 mm), clinical attachment level (CAL, 7 mm), and width of keratinized tissue (3 mm) were obtained using a Williams periodontal probe. Clinical photographs were taken preoperatively () and postoperatively (). Intraoral periapical radiograph (IOPA) of the area showed no bone loss in relation to number 32 ().\nThe patient was educated and motivated about the procedure and informed consent was obtained. Oral hygiene instructions with emphasis to brushing habits were given. Thorough scaling and root planing were done. The patient was periodically recalled to assess the oral hygiene and gingival status.\nThe proposed flap design for the surgical procedure has been shown in . Following local anesthesia, an intracrevicular incision through the bottom of the crevice followed by mesial and distal vertical releasing incisions were made including both papillae adjacent to 32 (). A partial thickness flap was reflected by sharp dissection as close to the periosteum as possible, beyond the mucogingival junction, and was extended until the partial thickness flap could be passively positioned over the defect without tension (). Following flap elevation, the exposed root surface was gently planed with sharp curettes. The exposed root surface was then conditioned with 50 mg/mL tetracycline solution for 3 minutes with subsequent saline rinsing using a three-way syringe.\nSubepithelial connective tissue graft was obtained () in the region of number 24 and number 25 from the palate with two incisions (L-shape) (), to prevent severe postoperative pain and discomfort and for early wound healing. The harvested connective tissue graft was sutured over the defect using a 5-0 vicryl suture to cover the graft, both papillae were first sutured midbuccally in relation to number 32 () followed by suturing of vertical incisions using 5-0 mersilk suture without tension (). A periodontal dressing (Coe-Pak) was applied to the surgical site to protect the site from irritation.\nPatient was instructed to discontinue tooth brushing and to avoid trauma or pressure at the surgical site. A 0.12% chlorhexidine rinse was prescribed twice daily for 2 weeks and amoxicillin 500 mg thrice daily for 5 days to prevent infection. The patient was recalled after 10 days for suture removal. The patient was enrolled in a maintenance programme (professional plaque control and oral hygiene instructions) and was instructed to resume mechanical tooth cleansing with a soft toothbrush using the roll technique after 2 weeks. |
A 5-month-old female presented to our hospital with a harsh S1 sound and a grade IV systolic murmur on the left sternal boarder. A transthoracic echocardiogram was performed, which revealed a homogeneous mass measuring 26 × 22 mm (Fig. ), with a broad base from ventricular septum and the body protruding into the right ventricular chamber, causing an accelerated right ventricular outflow (Vmax = 4.0 m/s, PG = 64 mmHg) with an obstruction (Fig. ). At the time of admission, the overall pediatric evaluation was normal including the laboratory data, chest radiograph and electrocardiogram. The cardiac-magnetic resonance imaging confirmed the presence of a solid mass appending to the right side of ventricular septum (Fig. -). However, all of the radiological examinations did not reveal the pathological nature of the mass.\nConsidering the size and location of the mass as well as the potential complications, surgery was the best option for this patient. As soon as the pericardium was incised after median sternotomy, a tenacious mass was palpable on the right ventricular free wall. Under cardiopulmonary bypass, an incision was made on the free wall of right ventricle, exposing a white, tenacious, non-capsulated egg-like neoplastic growth protruding into the right ventricular chamber, with its base arising from interventricular septum. The body of the tumor was adjacent to the right ventricular outflow tract, causing an obstruction (Fig. ). The tumor was resected along with its base, measuring 27 × 25 × 22 mm (Fig. ), and the incision on the right ventricle was primarily closed. The intra-operative pathological screening reported that the specimen was consisted with fibroblasts along with collagen deposition, combined with the negative results reported from the microbiology, which confirmed the diagnosis of cardiac fibroma (Fig. ). The patient’s postoperative course was uneventful and got discharged by the expected duration. There was neither any recurrence of the tumor nor any complications during the 3-month follow-up evaluation with a normal cardiac function. |
We assess a 59-year-old woman in a gynecological oncology center. The CA125 level is 153 U/ml. On transvaginal ultrasound, we describe a solid ovarian mass with a maximal lesion diameter of 59 mm, a maximal diameter of the largest solid component of 59 mm as well, and without acoustic shadowing. There is presence of fluid outside the pouch of Douglas (ascites). If we introduce these parameters in the ADNEX model (in this case, application for smartphone), we obtain the results and column charts as illustrated in . First we add the risk predictions for the four malignant subgroups to obtain the total risk of malignancy, which is 95.3% for this patient. Thus the tumor is likely to be malignant. Then, we look at the differentiation between four malignant subgroups and observe a predicted risk for secondary metastatic cancer of 21.2% (compared to a baseline risk of 4.0%) and a risk for stage II-IV ovarian cancer of 67.9% (compared to a baseline risk of 14.1%). The predicted risks for the other subgroups are lower (4.6% for stage I cancer and 1.6% for borderline) and are also smaller than the baseline risks.\nThe corresponding relative risks (i.e. ratio of predicted risk and baseline risk) for secondary metastatic cancer and stage II-IV ovarian cancer are 5.3 and 4.8, respectively. The relative risks for borderline or stage I ovarian cancer are below 1. With the knowledge of the increased risk for advanced stage ovarian cancer and more importantly secondary metastatic cancer, clinicians may adjust their preoperative diagnostics by excluding a tumor of other primary origin, such as gastric cancer or breast cancer. In case of a solid tumor these investigations may include PET-CT, MRI whole body diffusion, gastroscopy, colonoscopy, or mammography. This is important as appropriate therapy depends on the origin of the primary tumor. This patient was eventually diagnosed with metastatic gastric cancer (Krukenberg tumor). |
Woman 52 year old with severe grade III APLD came in consultation. She was also aware of her familiar condition. Liver transplantation was offered to her in another center but she refused. Previous MRI from another center showed multiple giants cysts compromising the whole abdominal cavity. Cysts reached hypogastric area and both inferior quadrants. Higher segments of the liver seemed to be respected. Globulous deformation of the abdomen was clearly observed. Despite aesthetic and mild abdominal pain symptoms were not as significant as they were in her two sister so she did not want to underwent liver resection too. However she kept coming to consultation every six months.\nTwo years later she came back presenting jaundice with serum levels of FAL>1700 mg/dl. Bilirrubin 9,8/7,9 mg/dl. New MRI showed cyst compression of the hepatic pedicle including common bile duct and portal vein ( A and 3B)\nSurgery was soon programed and approach was discussed. We decided laparoscopic approach but being aware room will be very reduced even after pneumoperitoneum. We decided to start by performing aspiration and evacuation of the big lower and anterior cysts to intend reducing the mass and make new space for the laparoscopic procedure. By performing this gesture followed by several fenestration and partial cysts resections space was increased within the abdominal cavity so laparoscopic liver resection could be then performed. Once gallbladder was located, we then identified two cysts that seemed to be responsible for hepatic pedicle compression. Fenestration was done and, then, intra-operative cholangiogram showed no further compression of the common bile duct with adequate passage of contrast to duodenum. Standard cholecystectomy was completed and later laparoscopic left lateral sectionectomy was done in association with several more cyst fenestration. Post-operative course was un-eventful and patient was discharged at 6th day with decreasing levels of FAL and bilirubin. Late follow up imaging control are still to come. |
A 66-year-old man was referred to the University of Utah Infectious Diseases clinic for evaluation of recurrent right thigh soft tissue infection. These symptoms began in 2011 when he underwent left ankle surgery following minor trauma. No indwelling hardware, other than surgical screws, was placed in the ankle. Several weeks after surgery, the patient noted the development of a firm nodule and swelling in the right groin and upper thigh, which he described as ‘a pocketful of rocks’. A magnetic resonance imaging (MRI) was performed, which revealed multifocal intermuscular and intramuscular rim-enhancing fluid collections in the proximal anterior lateral thigh. The lesion ulcerated and developed into a chronic, non-healing wound. The patient underwent surgical debridement, followed by several weeks of oral antibiotics targeting Gram-positive coccobacilli recovered from intra-operative tissue culture that were not further speciated. Several months after surgery, the wound site became inflamed and ulcerated, requiring repeat surgical debridement and antibiotics. At the time of infectious diseases referral in late 2016, the patient had undergone 11 episodes of surgical debridement of the right upper groin and thigh followed by short course antibiotics over a 5-year period.\nThe patient’s medical history is significant for well-controlled diabetes mellitus (HbA1c = 5.7%), hypercholesterolemia, hypertension, prostate cancer and West Nile virus (WNV) encephalitis in 2006 from which he recovered without incident. On physical examination, a 2-cm irregularly shaped, well-healed scar was identified immediately adjacent to the surgical site on the anterior right lower extremity. The patient attributed this scar to a distant childhood traumatic injury, when a large stick penetrated his right thigh after he fell from a tree house onto a marshy forest floor. He recalls removing the penetrating foreign body and never sought medical attention for this wound, which healed spontaneously. The wound was never explored for retained foreign bodies, and he denied any history of surgery or invasive procedures prior to the ankle surgery in 2011.\nUpon examination, the patient was oriented and in no distress. The blood pressure was 116/64 mm Hg, pulse was 76 beats per minute, respirations were 16 per minute, oxygen saturation was 93% on room air and temperature was 36.4°C. Examination of the right anterior thigh revealed a serpiginous, well-healed surgical scar that was non-tender and non-erythematous. Two well-healed irregularly shaped scars were noted adjacent to the surgical site. There was full range of motion and no tenderness over the hip joint or lumbar spine. He exhibited normal gait and had no inguinal lymphadenopathy. Laboratory results showed no abnormalities in blood counts, complete metabolic panel or inflammatory markers. The patient’s blood work was negative for human immunodeficiency virus, Hepatitis B and Hepatitis C.\nMultiple intra-operative tissue cultures from surgical debridement recovered either Paenibacillus species or Gram-positive coccobacilli not further speciated, including the most recent debridement in 2016. He had been prescribed a variety of post-operative antibiotic regimens and reported that trimethoprim-sulfamethoxazole (TMP-SMX) treatment brought symptom relief, with no recurrence during the antibiotic course. At the time of infectious diseases consultation, the patient was taking TMP-SMX after the most recent surgical debridement. A repeat MRI was scheduled. Five days prior to repeat imaging, the patient discontinued TMP-SMX treatment. MRI revealed no fluid accumulation or recurrence of infection, and the patient was advised to remain off TMP-SMX.\nThe patient remained healthy and pain-free for 3 months following TMP-SMX interruption, when fatigue and weakness returned, as well as tenderness and swelling at the surgical site. A superficial abscess at the site of debridement was drained, and tissue cultures grew P. macerans. An MRI was performed, with multifocal intermuscular and intramuscular rim-enhancing fluid collections within and around the vastus lateralis (Fig. ). The patient underwent right thigh surgical debridement and wash out. Antibiotic susceptibility testing of intra-operative tissue culture identified TMP-SMX-susceptible P. macerans (Table ); fungal and mycobacterial cultures were negative. Pathology showed focal necrobiotic granulomatous inflammation in soft tissues. The patient was continued on a regimen of 360 mg oral TMP-SMX BID indefinitely and has remained afebrile and clinically stable since reintroduction of therapy. |
A 77-year-old male with a past medical history significant only for gout, and hypertension presented as a stroke alert. His last known normal time was 12 hours prior, before going to sleep. His wife noted an episode of right facial droop and speech slurring, which prompted her to call emergency medical services. His wife also described an episode of thrashing in the bed and urination. She mentioned that the day before presentation the patient was “not feeling well” and took aspirin. He did not mention experiencing any specific symptoms.\nUpon presentation to the emergency department, the patient could localize to pain but had a decreased level of consciousness. Vital signs were unremarkable, but frequent premature ventricular complexes were seen on the electrocardiogram. The patient’s wife did not know of any prior history of this. Physical examination was remarkable for 5-mm dilated pupils bilaterally, crackles bilaterally at the lung bases, and decreased level of consciousness. The National Institutes of Health Stroke Score (NIHSS) was 18: 2 for level of consciousness, 3 for visual fields, 2 for the left arm, 2 for the right arm, 2 for the left leg, 2 for the right leg, 3 for best language, and 2 for dysarthria. Laboratory evaluation revealed no leukocytosis, a normal glucose of 115 mg/dL, and lactic acid of 1.4 mmol/L. Urine drug screen was negative, as was the urinalysis. The patient had normal vital signs and thus did not meet the criteria for sepsis. Initial computed tomography (CT) of the brain did not reveal an infarct or hemorrhage (Figure ).\nCT angiography (CTA) of the head and neck did not show any sign of aneurysm but revealed scattered carotid calcifications, a left parotid mass of 2 cm, and a right apical cavitary lesion with peripheral calcifications confirmed by chest radiography (CXR). CXR also revealed several small pulmonary nodules scattered in both lungs as well as a widened upper mediastinum. The patient was still not responsive after imaging was performed, and a lumbar puncture was about to be performed since the patient still had altered mental status. As consent was obtained, the patient slowly became responsive, and started answering questions and opening his eyes. Due to acute improvement in mental status, lumbar puncture was not performed.\nMRI was then performed, which revealed a small acute infarct in the bilateral thalami and adjacent central aspect of the midbrain, right larger than the left (Figure ). The patient was admitted for further neurological workup. |
A 70-year-old male with history of amyotrophic lateral sclerosis (ALS), dysphagia status post gastrostomy tube placement, chronic respiratory failure status post tracheostomy, and gastroesophageal reflux disease presents with six-day duration of melanotic stools. The patient was mechanically ventilated with gastrostomy tube and tracheostomy in place. His physical exam otherwise was benign. He was found to have acute onset anemia with hemoglobin of 7.1 g/dl. He was suspected of having peptic ulcer disease secondary to NSAIDs given the history of chronic musculoskeletal pain with NSAID use. Due to continual decrease in hemoglobin requiring daily blood transfusions despite conservative management, upper endoscopy was performed. It demonstrated a bleeding ulcer distal to pylorus in the duodenal bulb. Endoscopic technique was used to remove the clot, but the procedure resulted in bleeding that could not be stopped leading to poor visualization despite epinephrine injections. Due to his multiple comorbidities, he was deemed to be a poor surgical candidate. The patient was referred to interventional radiology for embolization of gastroduodenal artery (GDA).\nDuring the embolization of GDA, the celiac angiogram demonstrated no evidence of active extravasation, but contour irregularity at the level of the mid-GDA was noted. Prophylactic coil embolization of GDA was performed achieving GDA stasis. Post-embolization angiography of the SMA demonstrated no evidence of bleeding from IPDA branches. Incidentally noted was a replaced right hepatic artery arising from the SMA.\nDespite embolization of GDA, patient’s hemoglobin continued to decrease to 6.8 g/dl and the patient continued to have melena and hematemesis resulting in hypotension. The patient required an additional nine units of packed red blood cells (pRBCs), six units of platelets, and five units of fresh frozen plasma. Computed tomographic angiography (CTA) showed a large bleed from IPDA off the SMA immediately posterior to the origin of the replaced RHA (Figure ).\nThe patient was referred for urgent embolization by interventional radiology.\nDuring angiography, no active extravasation was identified and given that the GDA was already embolized, the most reasonable source vessel in the suspected territory would be the IPDA. The replaced RHA was selected and IPDA was identified as its first branch (Figures , ).\nWhile the branch arising directly from the replaced RHA represented the posterior IPDA, the anterior IPDA was seen as collateral vessel going back to the middle colic artery (Figure ).\nHowever, no focus of active bleeding could be identified from digital subtraction angiography from the posterior IPDA or the anterior IPDA. Cone beam computed tomography (CBCT) catheter angiography of the posterior IPDA was performed to identify the region of the duodenal bleed analogous to the region of active extravasation seen on abdominal CTA. The same location on the posterior wall of the duodenum seen on abdominal CTA was matched on CBCT angiography (Figure ).\nSubsequently, the posterior IPDA was embolized using n-butyl cyanoacrylate (NBCA) (Trufill n-BCA Liquid Embolic, Codman Neurovascular, Raynham, Massachusetts, USA) mixed with ethiodol 1:4 dilution ratio administered via the microcatheter (Figure ).\nPost-embolization angiography of the SMA demonstrated successful embolization of the posterior IPDA with sparing of the anterior IPDA. The patient became hemodynamically stable post procedure and no longer required blood products transfusion. His hemoglobin stabilized at 8.6 g/dl. The patient was successfully discharged to a skilled nursing facility to continue his recovery. At eight weeks follow-up the patient remained asymptomatic and he agreed to participate in this case report. |
A 5-year-old girl was admitted due to left hemiparesis and left facial palsy. She had developed symptoms of cough and fever 6 days before. Although she had taken oral antibiotics prescribed at a local clinic, her symptoms did not show improvement. On the fourth day of her illness, she was referred to a secondary hospital and was diagnosed as pneumonia. She was admitted to that hospital and was treated with antibiotics of ceftriaxone and clarithromycin, intravenously. On the sixth day of her illness, her fever subsided. However, she abruptly developed left hemiparesis and left facial palsy, and was referred to Inha University Hospital. Past medical history of the patient and family were unremarkable.\nOn admission, she was fully conscious and oriented. Vital signs showed a blood pressure of 127/60 mmHg, heart rate of 102 beats/min, respiratory rate of 24 breaths/min, and body temperature of 37.6℃. Her heart beat was regular without murmurs, while breath sounds were coarse with inspiratory crackles auscultated on both lung fields. On neurologic examination, pupils were isocoric with prompt light reflexes. Extraocular movements were full in both eyes, while there were defects in the left visual fields. She was unable to form facial expressions on the lower half of her left face, while she was able to wrinkle her forehead normally, indicating left side central type facial palsy. Motor strengths were decreased to grade III in both the left upper and lower extremities. Sensory was intact, while hemineglect was revealed on the left side. Hyperreflexia was noted on the left biceps and ankle jerks. Pathologic reflexes of Babinski sign and ankle clonus were present on the left.\nChest radiography conducted on admission showed pneumonic infiltration and pleural effusion in the left lung field (). Brain magnetic resonance imaging revealed acute ischemic infarction in the right MCA territory (), and brain MRA showed complete occlusion of the right MCA at the M1 portion (). Transthoracic echocardiography and electrocardiography results were normal. TFCA revealed complete occlusion of the right MCA at the M1 portion, with no evidence of vasculitis or Moyamoya disease ().\nLaboratory exams conducted on the day of admission showed a hemoglobin of 10.5 g/dL, hematocrit 30.1%, white blood cell (WBC) count of 5,420/µL, comprising 82.8% neutrophils, 9.2% lymphocytes, and 4.2% monocytes, and platelet count of 250,000/µL. C-reactive protein was 7.15 mg/dL while other electrolyte and biochemistry laboratory exams were normal. Coagulation studies revealed normal prothrombin and activated partial thromboplastin time, while fibrinogen level was elevated to 674 mg/dL (normal range, 200–400 mg/dL) and D-dimer level to 1.7 µg/mL (normal range, 0–0.4 µg/mL). Analysis of the patient's cerebrospinal fluid (CSF) showed a WBC count of 3/µL, glucose concentration of 90 mg/dL, and protein concentration of 15 mg/dL. Initial serum antibody titers to M. pneumoniae detected by enzyme-linked immunosorbent assays (ELISA) were 5,021 U/mL for IgM (normal range, 0–949 U/mL) and 5.5 AU/mL for IgG (normal range, 0–9.99 AU/mL). Polymerase chain reaction (PCR) of M. pneumoniae, influenza viruses, enteroviruses, and herpes simplex viruses were negative in the CSF.\nThe patient was diagnosed as pneumonia, strongly suggestive of M. pneumoniae origin associated with acute cerebral infarction. Antibiotics of ceftriaxone and clarithromycin were administered for 14 days, mannitol for 7 days, and methylprednisolone for 3 days, intravenously. Antithrombotic therapy with aspirin at doses of 2 mg/kg was given daily. There was no fever after admission and respiratory symptoms showed gradual improvement. Rehabilitation therapy was initiated on the fifth hospital day. Pneumonic infiltration on chest radiography started to show improvement on the sixth hospital day.\nThe results of laboratory exams in order to identify possible predisposing causes of the patient's stroke were all negative. Serum analysis of prothrombotic agents and factors including protein C, protein S, antithrombin III, lupus anticoagulant, homocysteine, lipid profiles and plasma lipoprotein levels were normal. Analysis of auto-antibodies associated with autoimmune and rheumatologic diseases, including antiphospholid antibodies, anticardiolipin antibodies, antinuclear antibodies, anticytoplasmic antibodies, anti-DNA antibodies, and rheumatoid factors were all negative. No other bacterial or viral organisms were documented in the serum or CSF on culture studies. Serum antibody titers to M. pneumoniae detected by ELISA 3 weeks after admission were 7,300 U/mL for IgM and 26.65 AU/mL for IgG.\nThe patient was discharged on the 32nd hospital day after receiving the inpatient rehabilitation schedule. At discharge, both left hemiparesis and left facial palsy had improved, although mild neurologic deficits regarding fine motor skills were observed. Motor strengths of grade V were checked on all extremities and unaided walking was capable. |
A 15-year-old Caucasian male with a medical history of attention deficit hyperactivity disorder (ADHD), neurofibromatosis type 1, and epilepsy presented to our institution. Additionally, this patient also had a known optic nerve glioma as well as a biopsy-proven low-grade glioma of the midbrain, likely secondary to neurofibromatosis type 1. The initial presentation was described as clusters of complex partial seizures, with associated automatisms of lip smacking and left arm raising that usually lasted less than a few minutes followed by a postictal period of increased somnolence. The patient also endorsed rare headaches. It was also noted that the patient was having increasing balance and coordination issues, listlessness, ataxia, and worsening behavior. His speech was slow but fluent and sometimes disjointed. On clinical examination, the upward gaze was slightly restricted and convergence was slow but consistent with his brain stem lesion. He did not have any finger-to-nose ataxia or any major dysmetria. However, he did have very subtle left lower extremity weakness.\nMRI with MR angiogram was obtained in light of these symptoms. MRI demonstrated interval stability of optic nerve glioma (not shown) and a midbrain glioma with no other acute abnormalities (Figure -). No sulcal FLAIR hyperintensity or enhancement was noted. Additionally, on MR angiogram, there was stenosis of the right intracranial internal carotid artery with the absence of flow signals of the right middle cerebral artery. Multiple small collaterals were seen in the region of the proximal right middle cerebral artery. The remainder of the anterior and posterior circulation appeared normal. Stenosis of vessels of anterior circulation with collateralization raised suspicion for the possibility of Moyamoya syndrome. After evaluation of these findings, it remained unclear whether these events were related to the patient's psychiatric issues, polypharmacy, high drug levels of phenobarbital, or progression of a possible right-sided Moyamoya syndrome. A catheter angiogram was recommended to further characterize the extent of his Moyamoya vasculopathy. The catheter angiogram with bilateral internal and external carotid artery and selective right vertebral artery injections was performed and this demonstrated severe occlusive changes of the right internal carotid artery with Moyamoya collateralization as well as significant contribution to the anterior circulation via the posterior cerebral artery and contralateral internal carotid artery as well as ipsilateral autosynangiosis (Figure -). There were signs of possible mild steal phenomenon of the left cerebral hemisphere secondary to diverted flow through the anterior communicating artery (Figure -). Further evaluation with MR perfusion imaging was suggested.\nSubsequently, MRI, MR angiogram, and MR perfusion studies were performed in the same setting. MRI showed no new area of cerebral ischemia or infarction. No intracranial hemorrhage was observed. Brain parenchymal changes were stable from the prior studies. MR angiogram demonstrated complete stenosis of the right internal carotid artery at the carotid terminus, distal to the takeoff of the posterior communicating artery. The M1 segment of the right middle cerebral artery was not visualized secondary to stenosis at the region of middle cerebral artery (MCA). Instead, multiple small collateral vessels were seen in that region. M2 and M3 segments of the right middle cerebral artery were formed by collaterals. An A1 segment of the right anterior cerebral artery was not seen. The remainder of the right anterior cerebral artery appeared normal in caliber and was formed by the anterior communicating artery presumably by flow from the contralateral anterior cerebral artery (ACA). There was a prominent right posterior communicating artery, which supplied the collaterals to the right MCA territory. Multiple prominent collaterals arising from the right external carotid artery were seen supplying the peripheral half of the right cerebral hemisphere (Figure ). These findings were also unchanged from the prior MR angiogram study.\nMR perfusion was then performed using a dynamic susceptibility contrast (DSC) T2 star technique after injection of gadolinium-based contrast. Blood flow maps for cerebral blood flow (CBF), cerebral blood volume (CBV) and mean transit time (MTT) were obtained and values of relative cerebral blood volume (rCBV), relative cerebral blood flow (rCBF), and MTT were generated. Similar regions of interest (ROIs) were drawn in both cerebral hemispheres in the MCA territory and corresponding values of rCBV, rCBF, and MTT were obtained quantitatively. Additionally, qualitative color maps were obtained for assessment of these parameters. There were no significant quantitative differences in rCBV, rCBF, and MTT values between the two cerebral hemispheres. Additionally, on the color maps, there were no significant differences between the two hemispheres qualitatively. Furthermore, when perfusion parameters of the cerebellum were considered as the internal reference standard (as the posterior circulation was normal on MR Angiogram), no significant differences were seen in perfusion of either of the cerebral hemispheres with respect to the cerebellum (Figure ). This indicated adequacy of collateral circulation in the right cerebral hemisphere, the hemisphere that was supplied by the stenosed internal carotid artery (ICA). Based on these findings, the neurosurgery service recommended that revascularization surgery is not required and the patient was kept on medical therapy. A careful lab evaluation demonstrated that the patient's blood level of phenobarbital was high and therefore, it was inferred that the change in the patient’s symptoms was related to excess phenobarbital. The patient’s dose was readjusted. Gradually, the patient’s clinical symptoms improved and approximately two years later, the patient continues to do well with medical management. |
An 8-year-old Japanese girl was referred to our clinic for the evaluation of a left facial nerve paralysis, left cervical zoster, and swelling of the right submandibular area. She developed cervical zoster and facial paralysis on the first and second day of illness, respectively, and was given prednisolone (1 mg/kg/day) and valaciclovir (1500 mg/day) orally by a pediatrician from the fourth day of illness. She subsequently developed a right submandibular swelling on the eighth day. She did not notice hearing loss or vertigo. She had been vaccinated against mumps at the age of 2 and had no obvious episode of mumps infection previously. Although she had also been vaccinated for chickenpox at the age of 2, she developed chickenpox at the age of 4. Another past medical history was notable for an operation for Fallot's tetralogy at the age of 2.\nAt the first visit to our clinic on the tenth day of illness, a physical examination revealed a complete left facial paralysis (House-Brackmann grade VI). Electroneurography of the facial nerve revealed nearly complete denervation of the left facial nerve (). Pure tone audiometry showed normal hearing in both ears. Stapedial reflex was absent in the left ear. The patient also had swelling of the parotid glands bilaterally and the right submandibular gland. Computed tomography images of the neck that had been examined in the pediatric department on the eighth day of illness confirmed sialadenitis of the left parotid gland () and right submandibular gland. We also noted herpes zoster in the left parotid area and posterior cervical area corresponding to the C2-3 area (). Serologic tests done on the 17th day of illness revealed the elevation of both antimumps and anti-VZV IgM antibodies by an enzyme-linked immunosorbent assay ().\nHer sialadenitis disappeared within 1 week. Her left facial movement also gradually improved to be House-Brackmann grade 2 with slight synkinesis 7 months later. Follow-up serological tests 6 weeks after the onset of illness revealed a decrease in both IgM antimumps and anti-VZV antibodies (). |
The present case report is about a 29-year-old man who was referred to Department of Periodontics, Saveetha Dental College, India, with a complaint of pain in relation to the left lower tooth. On examination, the patient was systemically healthy and had not taken any long-term anti-inflammatory medications or antibiotics.\nOn periodontal examination and radiographic evaluation, the patient presented with an intrabony defect extending up to apical third of the mesial root [] of left mandibular first molar (#36) with a probing depth of 8 mm using William's periodontal probe []. The patient also presented with pain in relation to #36 tooth and had pain on percussion. There was a lingering type of pain when subjected to heat test using a heated gutta-percha point. The diagnosis was made to be primary chronic periodontitis with secondary endodontic involvement in relation to left mandibular first molar (#36).\nInitial therapy consisted of oral hygiene instructions, which were repeated until the patient achieved an O’Leary plaque score of 20% or below.[] Scaling and root planning of the teeth were performed. Patient was referred to Department of conservative dentistry and endodontics for root canal therapy in relation to #35 and #36 teeth (which were symptomatic to the heat test).\nAt 4 weeks following phase 1 therapy, a periodontal re-evaluation was performed to confirm the suitability of #36 tooth for this periodontal surgical procedure. Clinical measurements were made using William's periodontal probe with graduation to a precision of 1 mm.\nBlood sample was taken on the day of the surgery according to the PRF protocol with a REMI 3000 centrifuge and collection kits. Briefly, 6 ml blood sample was taken from the patient without an anti-coagulant in 10 ml glass test tubes and immediately centrifuged at 3000 rpm for 12 min. A fibrin clot was formed in the middle of the tube, whereas the upper part contained acellular plasma and the bottom part contained red corpuscles. The fibrin clot was easily separated from the lower part of the centrifuged blood. The PRF clot was gently pressed between two sterile dry gauges to obtain a membrane, which was later minced and added to the graft material (OSSIFI™) [].\nAn intrasulcular incision was made on buccal and lingual aspect of the tooth of left mandibular teeth (#35, 36 and 37) along with a vertical incision, extending to the muco gingival junction in relation to the distal aspect of #35. A full thickness triangular flap was raised and inner surface of the flap was curetted to remove the granulation tissue. Root surfaces were thoroughly planned using hand instruments and ultrasonic scalers. The left mandibular first molar demonstrated mesial intrabony defect. After removing granulation tissue thoroughly, mesial intrabony defect was found to extend in buccal and apical aspect [].\nBriefly, alloplast (OSSIFI™) and was applied to the defect walls and root surfaces [Figures , and ]. The alloplast with PRF was then condensed using amalgam condensers. The flap were repositioned to their pre-surgical levels and sutured with silk utilizing an interrupted technique [].\nAfter the operation, the patient was prescribed systemic antibiotics (amoxicillin 500 mg tid, 3 days), non-steroidal anti-inflammatory drug (combiflam tid, 3 days) and 0.12% chlorhexidine rinse (twice a day for 4 weeks). Sutures were removed after 7 days. Clinical healing was normal with neither infectious episodes nor untoward clinical symptoms. The patient was seen at 1st week, 2nd week, 1st month, 3rd and 6th month.\nPeriapical intraoral radiographs were obtained from the periodontal defect site at baseline, 3 months and 6 months after surgery []. The radiographs were obtained with paralleling technique using film holders. To standardize, putty index was made and patient was asked to bite on it along with that of the holder.\nIn this case report, the greater reduction in pocket depth and gain in clinical attachment were found after 6 months of the follow-up []. These are the important clinical outcomes for any periodontal regenerative procedures. Radiographs revealed significant bone fill in the intrabony defect compared with measurements at baseline []. |
A 28-year-old man presented at a traumatic surgery unit in an outlying hospital, where an external rotation of the left leg and a patellar dislocation were detected. Examination also revealed clinical signs of a neurofibromatosis-1 (Figure ). The patient had stumbled doing his work as a cook and had fallen on his flexed left knee. Conventional radiography of the knee demonstrated an osteochondral flake near the medial patellar margin, whereas the femoral fracture remained unnoticed (Figure ). After reposition of the patellar dislocation, the joint was stabilized with an orthosis, and the patient was sent home.\nTwo days later, because of increasing pain and swelling of the left knee, his family doctor arranged further radiographic examinations with MRI and CT scans, revealing the Hoffa fracture. The patient was then referred to a medical center for traumatic surgery. Surgical treatment consisted of diagnostic arthroscopy, open reduction of the fracture, and internal fixation with three 40-mm headless compression screws (Figure ). The screws were placed in posterior to anterior and caudal to cranial directions. The anterior horn of the lateral meniscus was fixed to the joint capsule using FiberWire 2-0, and the ruptured retinaculum was fixed to the medial patellar margin with two Mitek anchors. A 3 × 3 cm tumor close to the articular capsule turned out to be a lipoma and not, as initially suspected, a neurofibroma.\nAfter surgery, the knee was stabilized with a knee orthosis, and partial weight-bearing was prescribed for 10 weeks. Fracture healing was regular. In <4 months after the accident, the patient returned to work without any orthopaedic therapeutic appliances. In this case, the occurrence of a femoral fracture after a low-velocity trauma was probably influenced by reduced bone quality because of an underlying NF1, but NF1 did not influence fracture healing. Two years after the operation, the range of motion of both knees was unlimited, and no osteoarthrotic changes were noticed. |
In June 2016, a 17-year-old boy came to our attention after being referred to us following a motorcycle accident. Informed consent from the patients was obtained. He immediately appeared non-compliant with healthcare workers. The first clinical evaluation revealed important and generalised pain of the right leg as well as functional impotence, tibial fracture with medial exposure of degree IIIB of about 10 cm of extension at the level of the distal middle third of the right tibia, contamination of the same outbreak with material that probably originated from the road surface, and various lacerated contusion wounds spread around the exposure ().\nThe patient showed no apparent deep vascular-nervous deficits and reported being a habitual smoker. After appropriate evaluation by the general emergency room surgeon in order to rule out abdominal and/or cranial lesions, the patient was sent to radiology to perform anteroposterior and lateral radiographs, which showed a complex multi-segmented displaced tibia fracture (type 42-C3) with an attached disrupted fracture of peroneal malleolus, type 44-B2 ().\nThe patient was surgically treated with a reduction and stabilization with external fixation. The patient was placed in the supine position, and the exposure was debrided to remove the contaminating residues. Various bruised wounds and bruises were thoroughly cleaned. The fracture was reduced by mounting an external fixation implant in a hybrid configuration with a tibial socket of three proximal Schanz screws and three distal 2 mm transverse wires anchored to a semicircular frame ().\nOne month later, the patient underwent a clinical and radiographic follow-up evaluation, which showed an implant with good grip and a reduction of the fracture abutments. The patient underwent new clinical and radiographic checks two months and three months after his surgery (). The multi-fragmentation of the fracture still showed no stable consolidation during these controls, with poor presence of bone callus; conversion to intra-medullary nailing had been planned at soft tissue healing.\nThree months after surgical treatment, the patient suffered a new motorcycle accident in which he reported the refracture and decomposition of the anatomical axis and the abutments, deformity of the anatomical profile, and damage to the soft tissues and external fixation system ().\nA new surgical treatment was performed. The external fixator was removed, the fracture abutments were reduced, and a long cast with a trans-calcaneal traction was performed. The cast was anteriorly open because the tibial fracture focus was prominent at the level of the soft tissue, encompassing the trans-calcaneal traction bracket ().\nIn the following days, vacuum-assisted closure (VAC) therapy was started to allow the progressive healing of the soft tissues overlying the tibial fracture focus ().\nAt the visit carried out in November 2016, the patient’s soft tissues had significantly improved, while the fracture was basically unchanged and not fully healed ().\nAfter 45 days with a plaster cast, a decision was made to perform a definitive surgical treatment. This involved intramedullary nailing blocked with two proximal screws and two distal screws to realign and reduce the tibial and peroneal fractures ().\nSubsequent monthly and two-monthly clinical-radiographic follow-up evaluations showed progressive healing of the tibia and fibular fractures and improvement of the functional clinical prognosis ().\nSuch fractures can lead to a significant loss of functionality and may limit the ability to walk, run, and participate in sports and work. Progressive loading on the lower right limb was possible after about 15 days after the surgical nailing operation. This was performed to stimulate bone healing, while remaining compatible with the expressed tolerance of the patient.\nTo evaluate the results, we considered both clinical and radiological criteria. We performed the last and accurate objective examination and radiographic follow-up evaluation in February 2017, two months after the intramedullary nailing surgery, which showed sufficient knee and ankle joint motion with an initial consolidation of the fracture. Physiotherapy played an important role in recovering the normal joint function. The initiation of physiotherapy often varies, depending on the type of surgery performed and the severity of the fractures and damage to soft tissues.\nA rehabilitation consisting of passive and active muscular mobilisation exercises was carried out to assist functional joint recovery as well as the maintenance of posture in orthostatism, balance, proprioception, adequate tension, and muscular strength. All of these were limited and did not allow for normal daily activities, following the long period of immobilization. The muscles were strengthened and affected the lower limb from the hip to the ankle. In the first period (after about 30 days), isometric toning exercises were performed by contracting the limb at one-minute intervals. Balance exercises were performed on the injured limb, toning exercises were performed using axial compression, and flexion exercises were performed by extending the foot and leg toward the thigh. At the beginning of rehabilitation, the use of crutches was necessary. In general, with progressive walking and balance improvement, patients may only require one crutch before they can move and walk freely, according to the patient’s tolerance. The walking style, amplitude of movement, strength, swelling, pain, and surgical scars were all taken into consideration. Ice-based therapies, electrical stimulation, and heat were applied to reduce pain and swelling and to allow for earlier bone healing. Swimming and cycling helped with muscle recovery. Also, lifting exercises and lowering of the affected leg were performed to assist the recovery of the quadriceps femoris and the gastrocnemius.\nSome rehabilitation treatments were performed at home after being introduced by a physiotherapist. Massages and scar tissue mobilisation were performed. The recovery times vary for each patient and can only be approximately calculated. They mostly depend on the conditions at the start of rehabilitation after surgery. Physiotherapy for fracture cases similar to the present one usually lasts 4 to 10 weeks. In the presented case, physiotherapy lasted about seven weeks until the recovery of mobility and return to normal activities were safely achieved. Patient compliance is essential for good results, and, in this case, the patient was oftentimes non-compliant. |
A 27-year-old Pakistani, Asian man, a medical student, with no previous comorbidities and no past history of tobacco smoking and alcohol intake, presented with 3 months’ history of frequent bouts of lower respiratory tract infections associated with exertional dyspnea, arthralgias, gradual weight loss, low grade fever, easy fatigability, and anorexia. His family history was also insignificant for any respiratory or other systemic pathology. In the fourth month of his illness, his fever became high grade associated with profuse sweating. All the baseline investigations were carried out on an out-patient basis. His white cell count was raised and a chest X-ray showed basal consolidation. A suspicion of pneumonia was made and he was started on broad-spectrum antibiotics. His condition improved temporarily but the symptoms re-emerged after a few days. He also received a course of orally administered fluoroquinolones but there was no improvement. A subsequent high resolution computed tomography (HRCT) scan of his chest revealed bilateral ground-glass haziness with areas of traction bronchiectasis, more so in the posterior basal segments of both lower lobes, suggestive of ILD (Figs. and ). He was given dexamethasone which improved his respiratory symptoms. In the sixth month of illness, he developed progressive proximal muscle weakness. He had difficulty in rising up from a chair and had difficulty in rising up from a squatting position. Throughout this period he had progressive weight loss (19 kg lost in 2 months) and profuse sweating. He was admitted to our hospital and thoroughly investigated. With time his proximal muscle weakness became severe and defined. It now involved his upper limbs as well and he had difficulty in combing his hair. He also gave a history of painful and cyanosed finger tips in winter.\nHe had lost muscle bulk (19 kg lost in 2 months) and was pale. He was febrile with recorded fever of 39.4 to 40.0 °C (103–104 °F). There was symmetric arthralgia but no arthritis. A chest examination still revealed bi-basal inspiratory crackles.\nRoutine and specialized investigations were carried out, which are tabulated in Table .\nA bone marrow biopsy was done to exclude any myeloproliferative disorder that could have been responsible for the continuously raised total leukocyte count (TLC). It turned out to be normocellular with no evidence of leukemia or lymphoma. His 25-hydroxy vitamin-D was deficient with a value of 4.9 ng/ml (normal value, 30–100 ng/ml; severe deficiency, < 20 ng/ml). An ultrasound of his abdomen was suggestive of only cholelithiasis, with no pleural or pericardial effusions. Bronchoalveolar lavage for acid-fast bacilli (AFBs), GeneXpert, and AFB culture/sensitivity and cytology was negative. A muscle biopsy was consistent with inflammatory myopathy.\nSince his musculoskeletal features could not be explained by ILD alone, a full autoimmune profile was sent: extractable nuclear antigen (ENA) profile. Anti-Scl 70 immunoglobulins G (IgG), anti-SRP IgG, anti-Mi2 IgG, anti-PL-7, and anti-PL-12 were negative. Anti-Jo-1 antibodies turned out to be positive in titers of 81 AU/ml (normal range 6–12 AU/ml). The clinical features in conjunction with elevated muscle enzymes like creatinine phosphokinase (CPK), anti-Jo-1 positivity, muscle biopsy, and HRCT findings led to a presumptive diagnosis of ASS. He was commenced on pulsed intravenous methyl prednisolone 1 gm daily for 3 days followed by orally administered prednisolone (1 mg/kg body weight), and vitamin D3 and calcium supplements at our hospital. He responded to the treatment. His CPK reduced and symptoms improved. He was discharged on orally administered glucocorticoids, orally administered azathioprine, proton pump inhibitors, and vitamin D3 analogs (to prevent corticosteroid-induced osteoporosis). Six months later, he is on a maintenance dose of orally administered steroids and azathioprine and is symptom free. The case is being presented after informed consent from the patient. |
A 39-year-old man was involved in a high-velocity motor vehicle accident. He sustained an open fracture of the right elbow, with significant loss of the external humeral condyle and partial loss of the olecranon. This fracture was classified as a Gustillo type IIIA injury. There was no neurovascular compromise. The patient was treated in a community center, close to the accident, where he received surgical care (debridement and partial excision of the olecranon). The wound was fully closed. The upper arm was then immobilized in a splint (). IV antibiotics (cefazolin-gentamicin) were started for 5 days.\nThe day following his elbow surgery, the patient fell in a staircase and sustained a C7-C8 and C8-T1 fracture-dislocation. This injury caused neurologic damage (quadriparesis), and his right arm became his only functional limb. Following this injury, the patient was moved to our tertiary center to get spinal fusion. During the spinal surgery, the elbow was tested under fluoroscopy. The patient's elbow showed varus instability and a positive pivot shift test. A CT scan of the elbow was obtained the following day and showed bony loss from the external humeral condyle and subluxation of the radial head ().\nWe decided to treat the patient's elbow surgically. The surgery underwent nine days after the initial trauma (after transfer from the community center, spine procedure and elbow imaging). A posterior approach to the elbow was used along with an extensive elbow debridement. A tricortical iliac crest graft was then collected from the patient's right side to replace the humeral condyle bone loss. A tendinous graft was collected from his third and fourth extensor digitorum longus tendons to reconstruct the lateral collateral ligament. The tendinous graft was fixed to the bone graft through two tunnels (anterior to posterior and lateral to medial). The iliac crest graft was then fixed to the humerus with a cancellous screw. A five-hole one-third tubular plate was used as buttress. The graft was left prominent on the lateral side to improve lateral stability of the elbow. The lateral collateral ligament was then reconstructed by fixing the tendinous graft to the proximal ulna with two bundles. We took care to preserve our reconstructed lateral collateral ligament's isometry during flexion and extension movements. Reduction and stability of the elbow joint were verified under fluoroscopy. The wound was closed with staples, and a posterior splint was applied for three weeks. 14 days postoperative X-rays are presented on . At three weeks following the surgery, passive and active ranges of motion were initiated.\nWhile wearing the splint, the patient developed a pressure wound on the lateral side of his elbow (at the site of the reconstruction). This wound later complicated into a Nocardia septic arthritis, which required surgical debridement. Four months after the reconstruction, range of motion was 30-120 degrees with full pronation and supination, without any evidence of instability.\nTwo months later, instrumentation was removed and a rotational flap was done to increase soft tissue coverage of the articulation. Five months after that procedure, the patient developed a Nocardia osteomyelitis of the humerus and was treated with 6 weeks of IV antibiotics (penicillin) and a surgical debridement. The patient responded well to our treatment. Eleven months after the initial intervention, range of motion of the joint ranged from 30 to 120 degrees of flexion, without clinical instability. Osteosynthesis was achieved according to X-rays of the elbow (). |
A 71-year-old Caucasian male with MG was known to suffer from nonobstructive hypertrophic cardiomyopathy and paroxysmal atrial fibrillation and a dilated left atrium. Multiple unsuccessful electrical and chemical cardioversions (using sotalol and amiodarone) had been attempted in the past. Therefore, ablation of the AV node was performed and a permanent dual chamber pacemaker was implanted. Nonetheless, at the time of admission to hospital the patient was complaining of fatigue, palpitations and dyspnea that were rapidly linked to uncontrolled atrial fibrillation at a heart rate of 120/minute. Previously, for a long time he was on metoprolol 100 mg BID concomitantly with diltiazem 300 mg OD for heart rhythm control. To further control the heart rate, he received in sequence atenolol 100 mg OD and, more than 24 hours after diltiazem cessation, verapamil SR 240 mg OD to replace his previous regimen. Two hours after receiving his first dose of verapamil, the patient began to complain of weakness and dyspnea. He presented with signs of shock with blood pressure at 70/50 mm Hg and heart rate at 101/minute. Lung and cardiac auscultation appeared normal with no new murmur of acute valvular failure, pulmonary congestion or signs of pulmonary embolism. An electrocardiogram (ECG) revealed paced rhythm (Figure ). A quick bedside echocardiogram showed diffuse hypokinesis of both ventricles with an ejection fraction reduced to 20%. Eighteen months before, an echocardiogram revealed normal left ventricular function and ejection fraction of 66%. At that time, the ventricular rate was 110 beats/min while the patient was on long-acting diltiazem 240 mg OD and cilazapril 5 mg OD. Cardiac catheterization was performed within minutes; coronary arteries appeared without significant stenosis, but there was a diffuse hypokinesis. At this point, cardiogenic shock secondary to calcium channel blocker intoxication was suspected. Blood glucose was 4.3 mmol/L, electrolytes (Na+, K+, Cl-) were 138, 5.2, and 105 mmol/L, respectively, all within normal limits. One hundred percent oxygen administration was performed resulting in a saturation of 98% and a pCO2 to 41.5 mm Hg (normal 35 to 45 mm Hg). Verapamil was stopped and the patient received intravenous glucagon, calcium chloride and dopamine. The measure of AchR-Ab was positive at 0.23 nmol/L (normal ≤0.02 nmol/L) and the measurement of antistriated muscle antibodies was also positive at 1:3840 (normal <1:60). A few hours later, another echocardiogram was performed and there was an improvement in ventricular function, resulting in recovery of systolic blood pressure to between 125 and 130 mm Hg. Five days later, the ejection fraction had returned to normal as had as the patient's electrocardiogram (Figure ). Re-challenge with verapamil was not performed. The patient died two years later. |
A 29-year-old man, a former semiprofessional handball player, had a traumatic rupture of the proximal side of the patellar tendon of the left knee in 2016 during a match. No associated disease was reported. Primary surgery was performed in another health facility through a median approach using 2 anchors for tendon repair protected by an additional ipsilateral semitendinosus graft (patellar and tibial tunnels). The patient came to our health facility following severe functional deficits after an iterative rupture without having experienced any new trauma 13 months after the initial surgery. The iterative rupture of the knee extensor mechanism was also an iatrogenic fracture of the transverse patellar tunnel (). Clinically, walking was not possible, there was a lack of active extension and hemarthrosis with pain. There were no scar problems, no signs of deep or superficial infection, and no cutaneous wound. A huge gap was clinically observed between the patella and the patellar tendon. Considering the patient's age, his preinjury sports level, and lack of active extension, the decision was made to perform a revision procedure. An artificial ligament (LARS®) and two adjustable loops, free ends of the PULLUP® BTB (SBM SAS, France), were used to enhance the patellar tendon repair.\nA preoperative lateral standard X-ray was taken of the contralateral knee at 30° of flexion to measure the Caton-Deschamps index and patellar height (). The patient was placed in a supine position under general anesthesia with a tourniquet at the proximal part of the thigh. The previous median approach was used. The patellar fracture and the site of the previous rupture were cleaned to remove fibrous tissue and hematoma. Previous anchors were left in place.\nThe first step was to place the ligament advanced reinforcement system (LARS® polyethylene terephthalate fibers 6 mm ref. L030307 ACFAR 32 CK). A new transverse tunnel was drilled in the tibia, distally to the tibial tunnel of the initial surgery, with a 5.5 mm drill, and the LARS® was inserted in the tibial tunnel. The artificial ligament was then passed through the lateral retinaculum and above the patella at the junction with the quadriceps tendon in a Pulvertaft manner and through the medial retinaculum to return to its origin (). Two longitudinal tunnels were drilled in the patella using a 2.4 mm drill. The loops of a PULLUP® BTB (the plate was removed from the device) were first passed into the patellar tendon and then into the patella through the two longitudinal tunnels using a shuttle relay. Next, the free ends were pulled down in the opposite patellar tunnels. At the proximal side of the reconstruction, the two free ends of the PULLUP® BTB were inserted into each braid to close the system (Figures and ). The distal and medial ends of the LARS® were tightened with a clamp in order to restore normal patellar height and secured with 2 staples. Then, the 2 PULLUP® BTB loops were tightened (). The previous tendon rupture was closed and reinforced with separate X-knots using absorbable sutures. The skin was closed. The knee was placed in an articulated brace with compressive ice therapy for 24 hours, and a postoperative X-ray was taken ().\nFor postoperative care, weight bearing was not allowed for 6 weeks and mobilization of the knee was immediately started between 0 and 45° for 3 weeks then from 0 to 90° from 3 to 6 weeks. No complication was reported during the postoperative period. At 3 months, the patient was pain free and could walk without the aid of crutches. He was able to resume handball practice at 6 months after a control MRI. At one-year follow-up, he was able to play handball with complete knee extension strength (compared with the contralateral side) and was able to return to a semiprofessional level. The range of motion of the knee was 0-0-130°. The MRI at 1 year showed complete healing of the patellar tendon and the bone (). |
The patient is a 66-year-old Caucasian male (height 186 cm; weight 98 kg) who acutely developed binocular visual disturbance at the age of 24 years. Visual acuity progressively declined within 1 month to seeing only contours. Upon ophthalmological work-up, LHON was suspected. Visual acuity slightly improved over the following years. The diagnosis was genetically confirmed by detection of the primary LHON mutation m.3460G>A at the age of 49 years. At the age of 60 years, he underwent right hip replacement therapy. At the age of 61 years, he experienced a speech disturbance for 4 days, which was attributed to an ischaemic stroke in the right frontoparietal and occipitoparietal regions with complete remission. Carotid ultrasound at that time revealed occlusion of the right carotid artery. A mild stenosis was diagnosed on the left carotid artery. Cardiovascular risk factors included arterial hypertension and hyperlipidaemia. Since the age of 65 years, he has experienced recurrent nonsystematic vertigo. He had a previous history of smoking 5 pack/years until the age of 61 years. His history was further positive for recurrent, sometimes traumatic falls. The family history was positive for LHON in his brother, the oldest sister of his mother, the second sister of his mother, and one female cousin. The mutation of the index case was also found in his brother and one aunt. The brother with LHON had LVHT, a complex-I defect on biochemical investigations of the muscle homogenate, was an alcoholic, had died suddenly, and had been previously described. The index patient’s mother had died from a ‘valve abnormality’. His brother had WPW syndrome as well.\nAt the age of 66 years, he was able to read letters with a height of approximately 4 cm and was able to watch television on an oversized screen. He was in a depressive mood since the death of his partner 5 years earlier and the sudden, unexplained death of his brother 1 year earlier, whom he found dead in his flat. Neurological examinations revealed markedly reduced visual acuity, positional tremor, past-pointing bilaterally, and reduced Achilles tendon reflexes (already present at age 50 years but not at age 24 years). Serum lactate, which was normal at age 24 years, was 2.5 mmol/l (normal range, 0.5–1.6 mmol/l), creatine kinase was 238 U/l (normal value, <190 U/l), and folic acid was 2.49 ng/ml (normal range, 4.6–18.7 ng/ml). Nerve conduction studies revealed a sensory polyneuropathy of the mixed type in the lower legs. Carotid ultrasound confirmed the previously diagnosed occlusion of the right carotid artery but could not confirm stenosis of the left carotid artery, where only plaques were found.\nCardiologically, the patient had a history of poorly controlled arterial hypertension since the age of 25 years. At 50 years of age, he started suffering from palpitations. At that time, electrocardiogram (ECG) showed a delta wave indicating WPW syndrome, signs of left ventricular hypertrophy and a right bundle-branch-block (RBBB). Echocardiography revealed an increased thickness of the left ventricular walls and LVHT affecting the apex and the posterior wall. Systolic function was normal. By Doppler-sonography, diastolic dysfunction (impaired relaxation) was diagnosed. Treatment with 20 mg lercanidipine, orally, twice daily and 32 mg candesartan, orally, daily was initiated. His history was negative for syncopes, atrial fibrillation, or resuscitation. The patient refused further cardiological follow-up.\nIn May 2017, at the age of 66 years, the patient was admitted to the Second Medical Department with Cardiology and Intensive Care Medicine, Krankenanstalt Rudolfstiftung, Vienna, Austria because of increasing vertigo during the previous 1 year and agreed to undergo further cardiological investigations. The clinical cardiological examination was normal. Blood pressure was initially high but normal at discharge. Transthoracic echocardiography showed LVHT and myocardial thickening but normal systolic function. The ECG was unchanged showing RBBB, occasional ventricular ectopic beats, and WPW syndrome with a PQ-time of 104 ms and an intermittent delta wave. During telemetric monitoring, multiple ventricular ectopic beats and nonsustained ventricular tachycardia were observed. Echocardiographically, left ventricular wall thickening had increased and LVHT was only visible in the posterior wall (). During stress-testing, the RBBB morphology of the QRS-complex disappeared at 55 Watts and recurred during rest. Considering these findings and the family history, it was decided to perform an electrophysiological investigation because of suspected left lateral pathway of the WPW syndrome. Electrophysiological investigations revealed a ‘bizarre’ cardiac conduction system, which is why the cardiologists refrained from undertaking radiofrequency ablation (RFA). Instead, implantation of an implantable cardioverter defibrillator (ICD) was proposed but refused by the patient. His last medications included: 50 mg trazodone, orally, daily; 100 mg acetyl-salicylic acid orally, daily; 20 mg lercanidipine, orally, twice daily; 32 mg candesartan, orally, daily; 2 mg lorazepam, orally, daily; 4 mg doxazosin, orally, three-times daily; 10 mg escitalopram, orally, daily; 10 mg ezetimibe, orally, daily; 1 mg rilmenidine, orally, twice daily; and 5 mg folic acid, orally, daily. |
A 55-year-old male was admitted to our hospital complaining of intermittent left-sided abdominal pain for two weeks. Vital signs were within normal and examination was unremarkable except for mild abdominal tenderness in the left upper quadrant. Urine analysis revealed mild hematuria, and abdominal ultrasound was performed which showed two stones in the lower pole of the left kidney measuring about 4 and 12 mm with an absence of hydronephrosis and normal right kidney. As a treatment for his condition, extracorporeal shock wave lithotripsy (ESWL) was performed with a total of 3,000 shocks were delivered at 15 kv. The patient tolerated the procedure well, and there were no complications during the procedure.\nOne day after ESWL, the patient started complaining of mild to moderate epigastric pain radiating to the back, the pain decreased with ibuprofen administration and the patient didn't seek medical advice. About seven weeks after ESWL, the patient presented to the emergency department with severe epigastric pain associated with nausea, vomiting, and early satiety. The patient had free past medical and surgical history and he is off medication and no history of alcohol consumption. On general examination, he was not icteric or feverish and an abdominal exam revealed epigastric fullness with severe tenderness. Laboratory investigations revealed markedly elevated serum amylase (6320 U/L). Complete blood count (CBC), liver enzymes, serum electrolytes, and lipid profile were within normal limits. An abdominal CT scan with contrast showed a giant multiseptated cyst measuring about 10 × 11 cm occupying the body and the tail of the pancreas (). Few small stones in the lower calyx of the left kidney also were found. The gallbladder, intrahepatic and extrahepatic biliary systems were completely normal.\nEndoscopic ultrasound- (EUS-) guided drainage was performed as a therapeutic procedure for giant symptomatic pseudocyst using a Pentax linear echoendoscope (EG-3870UTK 3.8). On the EUS, there was a huge pancreatic pseudocyst with internal septation ( (a)). The Niti-S SPAXUS stent (from Taewoong Medical) with 16 mm diameter, 20 mm length was placed with excellent drainage of >1500 cc of fluid and debris (). The patient tolerated the procedure well, and there were no complications. One day after the procedure, a CT scan showed excellent results with regression of the pseudocyst, and the patient was discharged home on levofloxacin 750 mg for five days.\nOn 4 weeks of follow-up, the patient reported disappearance of symptoms. Repeated EUS showed resolution of the pseudocyst and SPAXUS stent was removed without complications ( (b)). |
A 68-year-old woman was scheduled to undergo surgery for pelvic organ prolapse at the Department of Obstetrics and Gynecology in our institute. No family member of the patient had a history of breast or ovarian carcinoma. Contrast-enhanced CT performed as part of the preoperative examination showed a 1.4-cm-diameter tumor in the upper lateral quadrant of the left breast (Figure ). The tumor shadow was suspected to be a breast carcinoma, and the patient was referred to our department. The patient had no complaints of nipple discharge or pain. On physical examination, there was a mobile, irregular mass of 1 cm in the largest clinical diameter. In the preoperative diagnostic workup, the patient underwent a bilateral mammogram (MMG) which showed a high-density mass with no calcification in the breast parenchyma, highly suspicious for malignancy (Figure ). Breast ultrasonography (US) showed a 1.3-cm, irregularly shaped, hypoechogenic mass (Figure ). Serum tumor markers were within normal limits. A core needle biopsy of the mass demonstrated malignant cells proliferating in a cribriform or papillary pattern.\nWith a diagnosis of invasive ductal carcinoma, tubule forming type, in the upper lateral quadrant of the left breast, the patient underwent total mastectomy and sentinel lymph node biopsy. The patient chose total mastectomy to avoid postoperative radiotherapy. As a result, it was no longer necessary to evaluate the test for intraductal extension, and intensive MRI was not performed. Intraoperative pathological examination confirmed that the two sentinel lymph nodes sampled in the axilla were negative for metastasis, and no axillary node dissection was performed.\nThe surgical specimen contained a hard, grayish, solid, and lobulated tumor with defined margins with a diameter of 1.5 cm (Figure ). Microscopically, the tumor was composed entirely of packed epithelial islands with a cribriform appearance (Figure ). The islands were irregularly shaped with an ovoid outline and separated by relatively thin fibrous connective tissue. Well-defined, rounded spaces (cribriform growth pattern) were formed within the islands, and proliferating tumor cells were columnar and rather uniform, having rounded to ovoid nuclei with nuclear pleomorphism grade of G1 (Figure ). Cribriform ductal carcinoma in situ (DCIS) was seen around the tumor; on pathological examination, total tumor area was 2.7 cm × 1.5 cm and invasive area was 1.5 cm × 1.0 cm. No lymphovascular invasion was identified, and the surgical margins were free of the tumor. On immunohistochemistry, the tumor islands had no positive cells for the myoepithelial marker CD10 (Figure ), whereas myoepithelial cells were preserved around the DCIS (Figure ). The tumor was classified as an invasive cribriform carcinoma of pure type. Nuclear immunoreactivity was strong for both estrogen receptor (ER) and progesterone receptor (PgR) and seen in 90% of the tumor cells (Figure ). Human epidermal growth factor receptor 2 (HER2) expression was absent (Figure ), and the Ki-67 labeling index was low (7%) (Figure ). Two axillary sentinel lymph nodes were examined intraoperatively and showed no metastasis. In accordance with the 8th edition of the Union for International Cancer Control (UICC) TNM classification, the tumor was staged as StageⅠA (pT1cN0M0).\nThe patient is presently free from local recurrence and metastasis 10 months after surgery on adjuvant hormone therapy. We are treating with an aromatase inhibitor, and planning 5 years of treatment. |
The case concerns a 16-year-old female patient with single ventricle, pulmonary atresia and malposed great arteries after the Hemi-Fontan procedure and aortic valve homograft implantation between the right atrium and right pulmonary artery. The homograft was closed interventionally, while the left lung was separated and supplied by a Blalock Taussig shunt at the age of 7 years []. Nine years later, this shunt became occluded and the left lung was supplied by collaterals only. The shunt was replaced by an 8 mm Gore-Tex tube graft. After an initial increase in the oxygen saturation from 60% to mid 70%, she developed massive left sided pleural effusions with haemodynamic compromise. It was clear that the left sided shunt was too large. So far the only therapeutic option for patients with pulmonary overcirculation due to an inadequate shunt size has been surgery. However, a seventh surgical intervention was much too risky and undesirable for the patient. The decision was made to place several bare stents into the shunt in order to reduce the internal graft lumen. The procedure was performed under general anaesthesia. An initial angiography confirmed a large 8 mm left shunt (). The shunt was crossed with the JR catheter and Terumo glide wire from the right femoral artery. Then the Cook extra-stiff wire and over it the Mullins long 7 Fr sheath were advanced into the left pulmonary artery. Four Genesis XD stents (Johnson & Johnson, NJ, USA; two 10 mm × 25 mm and two 10 mm × 29 mm) mounted on Opta Pro balloons (Cordis, NJ, USA) were implanted into the Gore-Tex tube one over another. Control angiography confirmed the proper position of the devices (). The day after the procedure oxygen saturation rose from 45% to 60% (with no additional oxygen supply) and transthoracic echocardiogram (TTE) showed an increase in the peak gradient across the shunt from 10 to 25 mm Hg. In the next few days the patient's condition improved, with better exercise tolerance and diminished oedemas. The chest X-ray showed a well-aired left lung. She was discharged home after 2 weeks and keeps steadily improving. The 4-month follow-up showed oxygen saturation of approximately 70% and left pulmonary artery pressure of 42/36/40 mm Hg in the last cardiac catheterization. |
Woman 52 year old with severe grade III APLD came in consultation. She was also aware of her familiar condition. Liver transplantation was offered to her in another center but she refused. Previous MRI from another center showed multiple giants cysts compromising the whole abdominal cavity. Cysts reached hypogastric area and both inferior quadrants. Higher segments of the liver seemed to be respected. Globulous deformation of the abdomen was clearly observed. Despite aesthetic and mild abdominal pain symptoms were not as significant as they were in her two sister so she did not want to underwent liver resection too. However she kept coming to consultation every six months.\nTwo years later she came back presenting jaundice with serum levels of FAL>1700 mg/dl. Bilirrubin 9,8/7,9 mg/dl. New MRI showed cyst compression of the hepatic pedicle including common bile duct and portal vein ( A and 3B)\nSurgery was soon programed and approach was discussed. We decided laparoscopic approach but being aware room will be very reduced even after pneumoperitoneum. We decided to start by performing aspiration and evacuation of the big lower and anterior cysts to intend reducing the mass and make new space for the laparoscopic procedure. By performing this gesture followed by several fenestration and partial cysts resections space was increased within the abdominal cavity so laparoscopic liver resection could be then performed. Once gallbladder was located, we then identified two cysts that seemed to be responsible for hepatic pedicle compression. Fenestration was done and, then, intra-operative cholangiogram showed no further compression of the common bile duct with adequate passage of contrast to duodenum. Standard cholecystectomy was completed and later laparoscopic left lateral sectionectomy was done in association with several more cyst fenestration. Post-operative course was un-eventful and patient was discharged at 6th day with decreasing levels of FAL and bilirubin. Late follow up imaging control are still to come. |
A 14-year-old girl presented to the Department of Gynecology of the First Affiliated Hospital of Xi'an Jiaotong University with no obvious cause of persistent and severe lower abdominal pain that began 8 h prior. After receiving 450 mL of plasma at another hospital, she was referred to our facility since her test results suggested that her fibrinogen was only 0.10 g/L. She developed spontaneous epistaxis bleeding 7 days after she was born. At 2 years of age, CFD was diagnosed. She had a history of spontaneous auditory canal bleeding at 7 years of age. Her menarche took place at the age of 13, every menstrual period she needed to use more than 40 sanitary pads, accompanied by fatigue, rapid heartbeat, and unable to attend physical education classes or daily physical exercise, showing that she may have hypermenorrhea. She even had two hospitalizations due to hemorrhagic anemia caused by menorrhagia 9 and 3 months prior. But no medical treatment was performed afterwards. Upon presentation at our hospital, she was in the middle of her menstrual cycle. Her abdominal pain was accompanied by nausea, vomiting, dizziness, and palpitation. Besides, she denied the history of smoking.\nAfter admission to the Department of Gynecology, we had a detailed physical examination. She was 155 cm and weighed 50 kg. Her vital signs exhibited blood pressure of 107/74 mmHg, a heart rate of 105 beats/min, a respiration rate of 23 breaths/min, and the body temperature of 36.5°C. General examination revealed marked pallor, but the mind was clear and no bleeding spots on skin. Abdominal examination revealed a soft abdomen without tenderness or rebound pain, but the mobile voiceless was positive. No obvious abnormalities were found in other physical examinations. Since the girl and her parents firmly asserted a history of asexual life, we didn't perform a gynecological examination.\nThen the patient underwent transabdominal ultrasound demonstrating that a visible 3.1 cm × 2.4 cm cystic mass next to her right ovary. The mass boundary was clear and the morphology was regular. No obvious blood flow signal was observed. The fluid dark area in front of her uterus was approximately 8.3 cm × 8.0 cm, and the fluid dark area of her uterus rectal lacuna was 5.5 cm × 2.8 cm, where sparse spot reflection was visible. The right iliac fossa level was 3.9 cm, and that of the left iliac fossa was 2.0 cm. There was no abnormality in the appendix.\nThe patient's coagulation function examination revealed signs of difficult coagulation. Her prothrombin time was 23.30 s (normal: 11–14 s), prothrombin activity was 37% (normal: 84–128%), international standard ratio was 2.07 (normal: 0.94–1.3), activated partial thromboplastin time was 43.8 s (normal: 28–43.5 s), thrombin time was 33.3 s (normal: 14.0–21.0 s), fibrinogen was 0.30 g/L (normal: 2–4 g/L). Her hemoglobin was 71 g/L.\nWhen the girl was admitted to our hospital due to abdominal pain, we considered it may be ruptured corpus luteum, ruptured follicles, ectopic pregnancy, torsion of the ovarian cyst or appendicitis. Since the girl was too young, and deny the history of sex life resolutely, we ruled out the possibility of ectopic pregnancy. Her abdominal pain was not metastatic, and there were no signs of peritoneal irritation. Ultrasound showed the appendix was normal, so appendicitis can also be ruled out. Ultrasound images confirmed the presence of hemoperitoneum, so the possibility of torsion of ovarian cyst pedicles was also low, thus the initial judgment was ruptured corpus luteum or ruptured follicles. The complete diagnosis of the girl included: hemoperitoneum (ruptured corpus luteum? ruptured follicles?), CFD, abnormal uterine bleeding-coagulation (AUB-C), and hemorrhagic anemia (moderate).\nConsidering the patient's vital signs were stable, based to the advice of a Department of Hematology consultation, we utilized conservative treatment including rehydration support, aminocaproic acid hemostatic treatment, 2 units of red blood cell suspension, 200 mL of frozen plasma, and 6 g of fibrinogen. After 3 days of hospitalization, her fibrinogen reached 0.50 g/L. We then placed a LNG-IUS (52 mg/piece) under intravenous anesthesia. Diagnostic curettage was performed simultaneously. The pathological results suggested that it was the proliferative phase of the uterus (), it also clarified that this hemoperitoneum was caused by ruptured follicles rather than the corpus luteum. The patient received COC (30 μg ethinylestradiol with desogestrel, 30 DSG) on the day of surgery, and then one tablet at approximately the same time every day for 63 consecutive days. When her anemia was corrected, COC administration was changed to 21 days of continuous medication as usual. |
A 46 years/old African American female was admitted to an outside hospital where she initially presented with shortness of breath and hypoxia. She had a history of right coronary artery stenting several years ago surrounding an inferior myocardial infarction possibly with right ventricular involvement and relatively preserved left ventricular (LV) ejection fraction. Her follow-up and compliance had been suboptimal in the interim period. Initially, she had a peripheral oxygen saturation of 77% with a pAO2 of 47 mmHg. She showed no evidence of an acute coronary syndrome, and a head CT performed for history of falls demonstrated no significant abnormalities. A shunt study in the form of ventilation perfusion scan revealed early uptake of radioisotope in the brain, suggestive of a right-to-left shunting. Subsequently, a transesophageal echocardiogram revealed a severe right-to-left shunt with immediate appearance of bubbles in the left side although no apparent atrial or ventricular septal defect was seen. LV ejection fraction was moderately depressed, and the right ventricle and atrium were noted to be dilated. Severe tricuspid regurgitation was described. () She was then transferred to The Christ Hospital, Cincinnati, Ohio, for further evaluation in the cardiac catheterization laboratory for a presumed occult atrial septal defect and possible percutaneous closure.\nUpon admission to our institution vital signs were stable, and physical exam was pertinent for elevated jugular venous pressure and severe lower extremity pitting edema. She was 5′2″ and 285 lbs on admission. Heart sounds were normal with only a soft holosystolic murmur. Electrocardiogram showed sinus rhythm with inferior Q waves and no acute changes. Evaluation in the cardiac catheterization laboratory revealed the following hemodynamic profile: right atrium (RA) 25 mmHg, right ventricle (RV) 45/25 mmHg, pulmonary artery (PA) 45/20 mmHg, pulmonary capillary wedge pressure (PCWP) 17 mmHg. There was no oxygen saturation step-up between RA to RV or the PA. Intracardiac echocardiography demonstrated an intact interatrial septum and numerous attempts to find a defect using catheters were unsuccessful. Coronary angiography showed patent right coronary artery (RCA) stent and minimal disease in the left coronary system.\nHer oxygen requirements were only 2-3 L/min by nasal cannula at baseline, to maintain oxygen saturation of 93%. To relieve discomfort due to her severe volume overload, and as the target volume was deemed to be in excess of 20 lbs, she was started on ultrafiltration using the Aquadex system (CHF Solutions, Inc.), with eventual removal of approximately 25 lbs of weight. With this degree of volume removal, it was noted that the patient's oxygen requirement appeared to increase to 8 L/minute oxygen via oximizer. A repeat transesophageal echocardiogram (TEE) was performed confirming the findings of the outlying hospital. Furthermore, on this study, a “ridge” at the inferior aspect of the interatrial septum was noted associated with a color Doppler signal away from the RA. () A 3-dimensional assessment of the LA suggested a small opening at the inferoposterior corner. () A cardiac CT angiogram demonstrated moderate cardiomegaly with a small pericardial effusion; however, a severely dilated coronary sinus was detected, possibly with an “unroofed” appearance, with apparent communication into the LA. ().\nWith this presumptive diagnosis, two attempts to cannulate the coronary sinus for angiography were made. Initial attempt from the right femoral vein was not successful partly due to a thebesian valve at the coronary sinus ostium; hemodynamic profile at this point, after 25 lbs of volume removal, showed RA 25, PA 40/15, PCWP 12 mmHg. A repeat attempt was made 2 days later in the electrophysiology suite, via the right internal jugular approach, with adequate engagement of the coronary sinus. During this attempt, the patient's respiratory status worsened and she was intubated in the suite. Venogram of the coronary sinus revealed a large coronary sinus that appeared to have two outlets, one to the RA and another to the LA. Direction of contrast flow was entirely from the RA to the LA ().\nThe patient was then referred to surgery where a communication between the CS and the LA was repaired and the tricuspid valve repaired with an annuloplasty ring. The right ventricle was noted to be “entirely infarcted.” After surgical correction, her pO2 was 123 on 100% FiO2 with O2 saturations at 99%. Postoperatively, central venous pressures remained elevated at 20 mmHg. Despite initial hemodynamic instability, her major difficulty involved inability to wean from the ventilator complicated by pneumonia and eventual gram negative sepsis. After approximately 20 days, she underwent a tracheostomy and was discharged to a long-term acute care facility, where she was successfully decannulated 1 month later. |
A 31-year-old female, gravida 2, para 2 (G2 P2), referred to the gynecologic clinic with a main complaint of secondary infertility during the last twelve months. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. The patient had two previous C-sections. Her menstrual cycles were ovulatory. Spermogram was unremarkable and hormonal assay did not show any abnormality. HSG was performed and revealed a migrated copper IUD with its long tail out of the uterine cavity (Fig. ). Plain abdominopelvic radiography also indicated a rotated T-shaped IUD in the pelvis (Fig. ). A transvaginal ultrasound was also performed by a skilled radiologist who reported a copper IUD in the bladder lumen with a small portion of it within the bladder musculature. The patient had taken multiple courses of antibiotics for urinary tract infection (UTI), but her symptoms never disappeared. The patient also underwent cystoscopy for recurrent infection last year, but no pathological finding was detected. Eventually, the patient said that her symptoms were related to the possible adhesions following two previous operations. The patient had a history of IUD insertion following her last C-section about eight years ago. After three years, however, the patient decided to remove it due to recurrent vaginal secretions. Three years later, her IUD was expelled, and another copper IUD was inserted. The patient removed the other copper IUD for becoming pregnant about two years ago. The patient was very confident of its removal, but her recent HSG, interestingly, demonstrated a rotated copper IUD in the pelvic cavity. Physical examination was essentially normal. A baseline complete blood count, urea, and creatinine levels were normal. The patient was candidate for a hysteroscopic cystoscopy. Hysteroscopy was normal except for a small fibrotic defect at the lower segment of the uterus. During cystoscopy, the long tail of the copper IUD was found on the posterolateral border of the bladder far from ureteral offices (Fig. ). The two short arms adhered to each other were embedded in the mucosal and muscular layer (Fig. ). No calculus was observed within the bladder cavity. Using glycine as the media, a mono-polar loop entered the bladder. A gentle and brief cautery was applied on the mucosa where the shadow of a short arm of the device was observed. The copper IUD was safely removed through the urethra using a special grasper. There was no major defect in the place of the copper IUD in the bladder. The bleeding points were effectively cauterized (Fig. ). The patient was discharged the next day with an indwelling catheter. After catheter removal, the patient did not complain of any urinary symptoms. |
A 69-year-old Korean female patient visited the Department of Allergy and Clinical Immunology, Cheju Halla General Hospital, due to urticaria and itching for 5 months. She had no specific family history or past medical history. She had been taking fexofenadine daily for 2 months before the visit to our clinic (Table ). There were no inducible factors, including stress, exercise, cold or sunlight exposure. Based on the diagnostic criteria for CSU, a diagnosis of chronic spontaneous urticaria (CSU) was made []. The clinical severity of chronic spontaneous urticaria was evaluated using the Urticaria Severity Score by Jariwala et al. []. Of the 12 questions, 10 were scored on a scale of 0–7, with higher scores reflecting greater severity of symptoms, increased disruption of quality-of-life components including sleeping, work/school attendance, and social life, and increased amounts of medication of antihistamine or oral corticosteroid use. Of the 12 questions, 2 reflected body areas experiencing symptoms and had 8 answer choices per question; increased scores suggested more extensive urticaria involvement. One of the USS questions is about the amount of oral corticosteroid used, which was double weighted, and the item score was multiplied by 2 considering that the need for oral corticosteroids reflected an increased severity of disease. However, steroid was not used in this report. The USS could be calculated by adding the score of each question, thereby resulting in a maximum of 93 and minimum of 0.\nIn the clinical severity scoring, the patient scored a 5 and showed initial improvement, the reduction of medication (medication reduction), the state of medication free (medication free), the state of symptom-free (symptom free) and remission (continuous symptom-free more than 4 weeks).\nTo evaluate allergic status and allergen sensitization for exogenous allergens, basic allergic tests (blood tests and the skin prick test) were conducted before and after treatment. Before the tests, all patients ceased antihistamines for at least 5 days. The patient had blood tests for complete blood counts with differentials, serum eosinophil cationic proteins, serum total IgE and IgE levels for specific allergens using a multiple allergosorbent test (MAST, Green Cross PD, Korea). In the MAST test, the specific IgEs for 41 allergens were evaluated as described in a previous report []. The test results showed the levels of specific IgE for each allergen, and a normal negative range was 0.000–0.349 IU/mL. Additionally, a skin prick test was also performed for 53 allergens as described previously []. Histamine hydrochloride 10 mg/ml was used as a positive control, and physiological saline was used as a negative control. The allergy results were interpreted by measuring the wheal size. Reactions were read after 15 min and described as negative (0, no reaction), 1 + (reaction greater than the control reaction but smaller than half the size of the histamine reaction), 2 + (equal to or more than half the size of the histamine reaction), 3 + (equal to or more than the size of the histamine reaction) and 4 + (equal to or more than twice the size of the histamine reaction). The minimum size of a positive reaction was 3 mm.\nAll four patients underwent laboratory tests and skin prick tests before and after treatment. Characteristically, no patients showed abnormally elevated results in the classical allergy tests, including eosinophil fraction in white blood cell counts, basophil fraction in white blood cell counts, eosinophil cationic protein (ECP) and serum total IgE levels (Table ). However, the sensitization patterns in blood and skin were variable and different among the four cases (Table ).\nHistobulin™ (Green Cross, Korea) is composed of 12 mg human immunoglobulin/0.15 μg histamine complex (2 ml in an ample). Patients who wanted to cease medication due to CSU participated, and Histobulin™ therapy was conducted to determine whether it induced remission or not, resultantly ceasing medication without symptoms and signs. Histobulin was administered by subcutaneous injection in the deltoid areas of the upper arm every week. In all cases, patients were instructed to take levocetirizine when they were uncomfortable or if the condition interfered with normal living, working or sleeping.\nThe patient in case 1 received 12 injections of Histobulin. Her initial clinical severity was 38 points. The clinical progress is shown in Fig. a. She showed initial clinical improvement after the second injection (Initial Improvement) (Fig. b; Tables , , ). The weekly medication frequency was reduced after the third injection (Medication Reduction). After the eighth injection, she no longer took medication (Medication Free). She was symptom free (Symptom Free) continuously (Remission) after the tenth injection. Remission was defined when symptoms and signs were not present for 4 weeks without medication. Her remitted state was maintained for more than 18 months, i.e., until the present. |
A 16-year-old female patient was admitted to the emergency unit complaining of a pulsatile, frontal headache over the last 12 hours accompanied by nausea with photo- and phonophobia. The symptoms evolved with diplopia, difficulty in performing adduction of the right eye, and dysarthria; imbalance started subtly about 1 hour after the onset of pain. The patient reported that at the beginning of the symptoms she presented partial loss of vision on the lateral field of the right eye, scotomas, and blurry vision, which lasted about 30 minutes, with spontaneous resolution. She denied fever or any other neurological complaint, or the use of oral contraceptives. She took dipyrone before going to the emergency department. Her medical history included the diagnosis of migraine that started with the menarche at the age of 10 years, when she began presenting premenstrual migraine of variable intensity with visual aura, with predominant laterality also variable. The previous aura was characterized from scotomas, blurred vision, tunnel vision, and partial loss of vision, which was always reversible, with a maximum duration of 60 minutes. The patient did not remember having presented symptoms like diplopia or ataxia during previous events.\nThe physical examination revealed tachycardia, blood pressure of 150/80 mmHg, and a normal temperature. The neurologic examination revealed a wakeful, vigilant, and cooperative patient. Visual acuity was normal and without alterations on the field of vision; her pupils were isochoric and the direct response, the consensual response, and the accommodation of the pupils were normal. Paresis of the right eye when looking to the left was present (), as well as diplopia, and a slow saccadic movement of the left eye in abduction, with horizontal nystagmus with the preservation of ocular convergence. Other cranial nerves presented normal function. Strength, sensitivity, and deep reflexes were preserved and symmetric. At the examination of station when the patient’s feet were close together, she swayed backward, which was slightly worsened when she closed her eyes. Her gait was ataxic and she had a fall tendency to the left. Diadochokinesis, appendicular movement speed, and the finger-to-nose test were normal, but the heel-shin test was dysmetric at the left side. Other systems showed no abnormalities.\nNo improvement in the neurologic examination was noted after the patient was medicated with metoclopramide, ketoprofen, and dexamethasone; however, the pain and nausea did cease. The patient was hospitalized and submitted to a thorough etiological investigation.\nThe cerebral computed tomography (CT) and the angiotomography were normal. On the second day of hospitalization, a partial improvement of the symptoms was noted, but the diplopia remained steady. The cerebral magnetic resonance imaging (MRI) showed a focus of diffusional restriction in the paramedian right dorsal portion of the midbrain, which was consistent with the topography of the ipsilateral medial longitudinal fasciculus, without significant expansive effect, exhibiting hyperintense focus in T2 and fluid-attenuated inversion recovery (FLAIR), which did not show impregnation by the contrast medium (). The cerebral () and cervical MRI angiography and the cerebrospinal fluid analysis with protein electrophoresis were normal. The electrocardiogram (ECG), echocardiogram, and Holter ECG were normal. Biochemical analysis and thyroid function tests were normal. Antinuclear antibodies (ANA) was positive 1/640 exhibiting a fine nuclear dot pattern. However, antibodies anti-SS-B/LA, anti-SS-A/Ro, anti-SM, anti-double strain DNA, anti-neutrophil cytoplasmic antibody, and lupus anticoagulant tests were negative. Complement fractions C3 and C4 were normal. Anti-HIV, HBsAg, and anti-hepatitis C virus were negative. Acute phase proteins, serum lactate, and muscle enzymes were normal. The serum dosage of protein C, homocysteine, and antithrombin III tests were normal. The search for factor V Leiden was negative.\nThus, the clinical features, the imaging examinations, and the thorough investigation were consistent with the diagnosis of cerebral infarction without any risk factor other than migraine. The patient was prescribed salicylates and propranolol with gradual improvement. She was referred to an outpatient clinic for follow-up. |
A 38 year old Cameroonian married male, presented to our health service with facial rash, oral mucosa sores and discharging eyes of 2 days duration.\nHe reported being apparently well till 3 days prior to presentation when he ingested ivermectin 12 mg per os single dose served during a nationwide anti-filarial campaign. A day later (2 days prior to presentation), he noticed blisters on his lips which later extended to involve his oral mucosa. This was associated with pains on chewing and soreness of his entire oral mucosa which impaired feeding. Rashes developed on his face simultaneously. The rashes were itchy, scaly and extending towards the hairline. A day prior to consultation, his eyes became reddish and discharged clear fluid. The eyes were itchy and painful. The patient did not have fever and there were no other associated symptoms. Inability to feed well and his disfigured face prompted consultation in our health facility.\nOn reviewing his past medical history, he denied taking other medications including paracetamol prior to the onset of the symptoms. He however admitted taking a similar dose of ivermectin 1 year earlier during a similar campaign with no reactions. His wife was HIV positive and on highly active antiretroviral therapy. He reported that he was last tested for HIV 6 months prior to presentation and the test was negative. He did not provide any record of the test results. He had no known chronic illness and denied having any known drug or food allergies.\nOn examination he was ill looking and in painful distress. There was a scarf around his face covering the oral lesions. The conjunctivae and sclerae were reddish and there was no cervical lymphadenopathy. His vital signs were within normal limits; blood pressure was 118/70 mmHg, heart rate; 84 beats/min, respiratory rate; 20 breaths/min, temperature; 37.2 °C, O2 saturation; 98% and weight 64 kg. There were desquamating hyper-pigmented rashes on his face with whitish plaques. The rashes were on the nasal bridge and extended to the malar area, sparing the nostrils. There were black eschars and erythematous erosions on the lips with sores and blisters in the oral mucosa (Figs. , ). His eyes were erythematous, tearing and had sticky secretions that made his eyelids difficult to separate. There were no other skin lesions and the rest of his examination was normal.\nBased on the clinical picture, a diagnosis of SJS was made with a differential of Lyell’s syndrome. Patient was counselled and tested for HIV. His test result was positive. A repeat 1st line test and second line test confirmed HIV seropositivity. Following HIV seropositivity, a CD4 count done to evaluate his immunological status was 568 cells/mm3. Other baseline tests revealed normal full blood count, liver enzymes and kidney function tests.\nHis management was mainly supportive: steroids (Dexamethasone 4 mg intramuscular route single dose, then prednisone 5 mg tablet: 4 tablets twice daily for 7 days then 2 tablets twice daily for 5 days and 1 tablet twice daily for 3 days), oral antihistamines (chlorpheniramine 4 mg tablets: 1 tablet twice daily for 10 days), antibiotic eye drops (ciprofloxacine eye drop: 1 drop on both eyes twice daily for 10 days), oral hygiene and nutritional support. He was reviewed a week later with much improved lesions and on week 3, most of the lesions had resolved. |
Our patient is a 67-year-old male with a history of hypertension, type 2 diabetes, and hyperlipidemia, who went through the Inova Movement Disorders Program due to a history of fluctuating, progressing uncontrollable movements of the left side of his body. He reports these movements had been present for around 10 years, worsening over time.\nAt baseline, he always felt that his left arm and leg were moving, at times exhibiting large amplitude movements which could knock things down or affect his walking, making him fall. He described it as abnormal, uncontrollable movements that made his arm ‘reach around or fling out’ His leg was also described to ‘dance on its own.’ While at baseline, these were present but minimal stress or anxiety would exacerbate the movements. There were no movement issues on his right side, nor any facial movements noted.\nHis exam showed episodic, mild with some interspersed moderate-amplitude quick movements of the left hand, forearm, proximal arm muscles, as well as movement of the left leg. These were not suppressible, brought out more by distraction or with anxiety, such as discussing an MRI scan as he was claustrophobic. He also demonstrated some lateralizing movements of the head to the left, but no facial or tongue movements. His right side showed no such signs, and his neurological exam was otherwise unremarkable.\nHe was sent for a CT scan of the head initially due to a fear of MRI scanners, and was found to have asymmetric calcification of the right caudate nucleus and anterior putamen (Figure ). The calcification spared the anterior limb of the internal capsule. Laboratory work for parenchymal calcification was then sent and was normal. The MRI of the brain with contrast demonstrated both hypointense gradient echo (GRE) signal in the right caudate nucleus and putamen, as well as an underlying DVA (Figures -).\nGiven the lateralizing of his deficits and correlation with opposite hemispheric parenchymal calcification, he was diagnosed with unilateral hyperkinetic movements as a product of putamen and caudate calcification from an underlying DVA. Consideration was made for focal hemorrhage, but presentation and imaging were not thought to reflect this pathology. He was then treated with 0.25 mg clonazepam 2x daily with significant improvement in his hyperkinetic movements. |
A 22-year-old multiparous woman with a history of primary sclerosing cholangitis complicated by portal hypertension, esophageal varices, and thrombocytopenia was admitted at 29 0/7 weeks for evaluation and management of shortness of breath and lower extremity edema. Her obstetric history was significant for prior spontaneous preterm birth with placement of an ultrasound-indicated McDonald cerclage at 20 1/7 weeks of gestation in the index pregnancy. At the time of cerclage placement, the 5 mm Mersilene (polyester) tape was noted to be suboptimally placed along the posterior rim, distal to the internal os, so a second McDonald cerclage of 0-polyester suture was placed cephalad to the tape. She was admitted at 28 0/7 weeks for preterm labor and the cerclages were removed with no remarkable anatomic irregularities. She received antenatal corticosteroids and magnesium sulfate during her admission for preterm labor and was discharged on hospital day #3 with a cervical exam of 1cm dilation and 70% effacement. During her readmission at 29 0/7 weeks for shortness of breath, she began to have contractions and moderate vaginal bleeding on hospital day #3. Her cervical exam was 4 cm and 90% effaced. She was transferred to labor and delivery, where she continued to have a rapid cervical change to 6 cm. A brief fetal heart rate bradycardia occurred with spontaneous recovery to a normal baseline. At this time, findings were concerning for placental abruption or possible coagulopathy from decompensated liver failure with a total estimated blood loss of 500 mL. Amniotomy was performed to expedite vaginal delivery. However, cervical dilation did not continue and complete cessation of vaginal bleeding was noted. Careful visualization of the cervix revealed that the posterior rim of cervix was detached and traversing the presenting fetal head creating a tension band that prevented further dilation or descent. This band of cervical tissue was clamped and suture-ligated followed by rapid delivery. She delivered a male infant with a weight of 1325 g and Apgar scores of 5 and 7 at 1 and 5 minutes, respectively; arterial cord pH was 7.33. Following delivery of the placenta, vaginal exam revealed a partial posterior cervical detachment from 2 to 9 o'clock. The avulsed portion of the cervix did not appear necrotic and was bleeding briskly. Hemostasis was achieved with a single running locked suture. Her postpartum course was otherwise unremarkable, and she was discharged on postpartum day #2. At her six-week postpartum visit her cervical exam was remarkable for a normal appearing anterior lip; however the posterior lip was not visible and not palpable on digital exam (). Pathology was not available for the fragments of cervix that were excised and the placenta was remarkable for acute chorioamnionitis. |
A 61-year-old female was admitted to our hospital suffering from 48 h of acute abdominal pain, characterized by epigastralgia without radiation and no response to spasmolytics or analgesics.\nThe patient had been suffering intermittent episodes of mild discomfort of the gastrointestinal tract such as bearable diffuse abdominal pain and feeling of flatulence that persisted for one year. The pain pattern was not related to defecation or eating, there was no nausea, vomiting, weight loss, melaena, change in bowel habit, urinary symptoms, or fever. She was managed conservatively as thought to be a functional gastrointestinal disorder.\nShe describes the pain as aggravating suddenly and sharp in nature. She presented to the emergency department after 48 h of the pain acutely worsened. The pain was in the epigastrium and across the anterior abdomen, was sharp and constant without radiation.\nShe had no antecedents of alcohol, tobacco, or drug abuse.\nShe had a medical history of arterial hypertension and trigeminal neuralgia and had no surgical history. In her family history, there were two cases of colorectal cancer (mother and sister) without other illnesses.\nThe patient experienced epigastric tenderness upon palpation, although she had no rebound tenderness, muscle tension, or a palpable mass. She had no other relevant findings.\nLaboratory test results including complete blood count, liver function tests, serum amylase and lipase, biochemistry, were within normal ranges.\nAn abdominal computed tomography (CT) scan showed a solid mass of the tail of the pancreas that contacted the lesser curvature of the gastric body. Magnetic resonance imaging (MRI) showed a pancreatic head, uncinate process, neck, and body of normal morphology. A solid nodular mass 29 mm in diameter was confirmed in the pancreatic tail, hypointense in T1, heterogeneous with hyperintense areas in T2, with enhancement after the administration of i.v. contrast predominantly towards the latter phase. Severe atrophy of the tail of the pancreas and upstream dilation of the main pancreatic duct was observed. The intra- and extrahepatic bile ducts were of normal caliber. This hypovascular nodule was highly suspicious of malignancy, probably PDAC. No regional or distant metastases were visualized in the abdomen (Figure ).\nComplementary imaging studies for staging were performed. Thorax CT revealed 10 solid nodules between 3-6 mm distributed in both lungs, which, due to their distribution, were suspicious of secondary implants. |
A 44-year-old lady suffered from end-stage renal failure secondary to IgA nephropathy and was started on continuous ambulatory peritoneal dialysis in January 2002. She developed an episode of peritonitis due to Mycobacterium chelonei in October 2007. It necessitated peritoneal catheter removal and switching to haemodialysis. Since the switch to chronic haemodialysis, she was noted to have worsening control of serum phosphate level and secondary hyperparathyroidism. Her intact parathyroid hormone level had progressively increased to 318 pmol/L in January 2010. The pre-dialysis phosphate and corrected serum calcium levels ranged from 3.1 to 3.9 mmol/L and 2.5 to 2.65 mmol/L, respectively. She received no calcium supplement. A total parathyroidectomy was originally planned in late February.\nShe received a cadaveric renal transplant in early February 2010. A session of haemodialysis was delivered immediately before the surgery. The pre-dialysis serum phosphate and corrected calcium were 3.4 and 2.6 mmol/L, respectively. The deceased donor was a 48-year-old lady who died from intracerebral haemorrhage and was on inotropic support before organ retrieval. Both the donor and the recipient were O positive in blood group with four human leukocyte antigen mismatches. The cold ischaemia time was 17 h. The immunosuppressive regimen consisted of prednisolone, mycophenolate sodium and cyclosporin.\nThe initial graft function was inadequate. An allograft renal biopsy was performed on Day 3 and it revealed Type IA acute T-cell-mediated rejection and acute tubular necrosis (ATN) (). She was treated with 3 days of pulse intravenous methylprednisolone, and cyclosporin was switched to tacrolimus for maintenance immunosuppression. However, the response was suboptimal and she was restarted on regular haemodialysis on Day 7. The allograft did not function well throughout the first month post-transplantation, and she remained dialysis dependent during this period. Allograft biopsy on Day 16 and 27 both demonstrated no evidence of acute rejection but features of ATN persisted, albeit less severe as compared with the first biopsy. She finally became dialysis independent 2 months after transplantation, but the graft function remained unsatisfactory with her serum creatinine staying between 310 and 397 μmol/L. Serum samples of the recipient taken at Week 2 and 8 post-transplantation identified no donor-specific antibody. Subsequent biopsy on Day 58 and 106 also failed to reveal any evidence of rejection but persistent acute tubular injury with prominent regenerative changes. C4d and SV40 stainings had been negative throughout the five consecutive allograft biopsies, and no viral inclusion body had ever been detected in these specimens. However, there were intratubular calcification, which was consistent with calcium–phosphate crystal deposition and chronic changes, first evident in the second biopsy done on Day 16, which progressively increased in amount in the subsequent biopsies. The allograft biopsy on Day 58, indeed, displayed numerous intraluminal calcifications in the tubules with obstruction and distension of tubules. In the biopsy on Day 106, prominent intratubular calcifications persisted but, in addition, there were also moderate-to-severe tubular atrophy and interstitial fibrosis, while ATN became less conspicuous. The overall picture was compatible with nephrocalcinosis due to acute phosphate nephropathy resulting in chronic allograft damage. The trends of her serum calcium and phosphate levels after transplantation are shown in .\nAt 5 months post-transplantation, the allograft function further deteriorated and the patient returned to regular haemodialysis. |
A 19-year-old male presented with a swollen left mandible and a palpable mass at the level of element 37. He had mild pain since a few weeks. The symptoms had been attributed to a dental problem by a local dentist, and root canal treatment had been performed, without any effect on the pain or swelling.\nAt the initial consultation, a mass was clearly palpable. A panoramic image showed a sclerotic, sharply delineated lesion with a low-attenuation halo, fused with the root of the adjacent molar (). A panoramic radiograph obtained 3 years earlier, for post- orthodontic treatment evaluation, did not shown any tumour sign ().\nCBCT showed a heterogeneous mandibular mass associated with the element 37 causing compression and infero-lingual displacement of the inferior alveolar and mental nerve. The mandibular cortex was thinned at the vestibular side and absent at the lingual side (). Despite the relatively rapid growth, the diagnosis of benign cementoblastoma was proposed based on the classic appearance as a periapical, sclerotic, sharply delineated lesion with a low-attenuation halo, directly fused to the root of the tooth.\n3D renderings showing the affected anatomic area were obtained (). Considering lesion size and location, a possible pathological fracture of the mandible pre- or post-operatively was considered.\nIn order to prepare for such risk or complication, 3D planning and printing were made available. The virtual 3D planning was performed in PROPLAN software (Materialise, Leuven, Belgium). The pre-operative CT patient Digital Imaging and Communications in Medicine (DICOM) images were imported into the software where threshold was applied to segment the mandible and the lesion. Both objects were further manually refined and 3D models were created. The inferior alveolar mandibular nerves were traced and 3D models were created. All 3D models were revised by the surgeon prior to export. These models were then exported as Standard Template Library (STL) files for 3D printing. The STL files were imported into the software of the professional Objet Connex 350 printer (Stratasys, Eden Prairie, MN) which is a polyjet printer with layer thickness of 30 µm. The mandible was printed in transparent hard material while the lesion and nerves in hard white material.\nThe 3D printed model allowed for possible fast and accurate (pre)bending of an osteosynthesis plate. Furthermore, considering the tumour size as well as the size of the expected post-surgical bone defect, the possibility for needing a bone graft was also taken into account.\nA standard surgical approach was used (). The tumour could be completely dissected, and the mental nerve could be separated from the tumour without injuring the nerve. Element 37 was removed. The cementoblastoma was split in pieces using a surgical drill, and the pieces were carefully removed, taking care to avoid mandibular fracture or nerve injury. The inferior alveolar nerve could be identified at the bottom of the resection cavity and appeared intact. The mandibular wisdom tooth was not removed in order to minimise the risk of pre- and post-operative fracture. While the mandibular cortex was deficient at the lingual side (as shown pre-operatively by the CBCT images and 3D model), the lower part of the mandibular bone could be kept intact during the procedure and fracture did not occur. The resection cavity was filled with 8 L-PRF membranes. Tight intermaxillary fixation was applied for 6 weeks. No bone grafts were placed in order to facilitate interpretation of the post-operative evolution, taking into account the possibility of tumour recurrence. The post-operative course was uneventful. Follow-up imaging obtained 4 years and 6 months after surgery showed normal healing with progressive new bone formation and closure of the bone defect (). |
A 38-year-old male with a history of epilepsy presented to us with increasing seizure frequency. His semiology consisted of generalized tonic clonic convulsions. A physical examination revealed that he was neurologically intact with new postictal right lower extremity weakness, which resolved during the hospitalization. T2 weighted MR imaging revealed a 24.2 mm x 16 mm x 13.3 mm roughly ovoid AVM located in the right sylvian fissure. Cerebral angiography demonstrated primary arterial feeders arising from the anterior division of the right middle cerebral artery (MCA) with venous drainage into the right middle cerebral vein and the vein of Labbe (Figure ). Figure shows a three dimensional (3D) dynamic computed tomography angiogram (CTA) reconstruction of the AVM (Figure ).\nThe patient then underwent endovascular embolization using Onyx 18 (Covidien, Irvine, CA) where near-complete embolization of the AVM was achieved with minimal contrast filling. Towards the end of the procedure and during the attempt to remove the microcatheter, its tip was noted to be adhered to the Onyx cast at the perinidal location. Unsuccessful attempts were made to withdraw the microcatheter by reducing the slack in the system, and traction was then applied over a period of 25 minutes. Given the tortuosity of the vessels and tension noted on the MCA branch, the decision to transect the catheter at the groin site was made to prevent acute intracranial vascular injury. After transection, the distal catheter tip was noted to be positioned along the descending thoracic aorta (Figure ). Since the patient was scheduled for neurosurgical resection of the AVM the next day, the catheter was removed at that time. The patient was started on the weight-based heparin drip to prevent clot formation along the retained catheter and admitted to the intensive care unit (ICU).\nThe following morning, the patient was taken to the operating room where a pterional craniotomy was performed. The sylvian fissure was then split medial to lateral exposing the proximal internal carotid. This was followed distally until visualizing the bifurcation and M1 was readily identified. We could visualize the retained intraluminal catheter at this point. We then turned our attention to the cortical surface where a venous varix was seen. We circumferentially dissected the AVM maintaining venous outflow throughout. Hemosiderin staining around the resection bed suggested prior hemorrhage. We then encountered the two major feeding vessels, which were then bipolared and divided, one of which had the adherent catheter. The entire catheter was then removed (Video ). Intraoperative angiography was not available. The entirety of the 50 cm catheter was removed during open craniotomy (Figure ). A follow-up at 12 months revealed that the patient was well with no neurologic deficits. |
This study was approved by Soonchunhyang University Bucheon Hospital Institutional Review Board (IRB) and informed consent was waived by the IRB (2020-02-017).\nA 58-year-old man came to the emergency room with multiple lacerations and swelling on his face after having been struck on the face by falling heavy machinery while welding in a factory. He was alert, and his Glasgow Coma Scale score was 15 on presentation. Blood pressure and respiration were stable. At the time of the trauma, he reported CSF leakage from the nose. No discharge from the nose was seen in the emergency room. Numerous areas of air density were visualized on the basal cisterns, lateral ventricles, and third ventricle. On initial computed tomography (CT), a linear skull base fracture was found in the central skull base and extensive pneumocephalus was found. Furthermore, a tiny air density was detected in the jugular fossa (). At this time, he had limited lateral gaze of his right eye, but no symptoms was observed associated with lower cranial nerve damage such as hoarseness, difficulty swallowing nor dysarthria. Also, he did not feel any nasal discharge and no further intracranial lesions were noted on brain pre-contrast CT on the day after admission. In addition, CT angiography was performed to verify injury to the petrous carotid artery because of the petrous skull fracture. No arterial injury was found, but a contrast-filling defect was noted in the right jugular fossa and the sigmoid sinus (). At that time, venous sinus changes were not carefully observed. The patient was transferred to the general ward on the third day after the trauma.\nBy the morning of the fourth day after the trauma, he developed mild fever of 37.8°C and dyspnea due to aspiration pneumonia, but no new neurological signs were observed. In the afternoon, the patient suddenly lost consciousness and was comatose, and there was no spontaneous respiration. We performed intubation and cardio-pulmonary resuscitation for 15 minutes, then his blood pressure and pulse were normalized, but breathing did not return, so a ventilator was applied. The patient was semi-comatose with abnormal extension to pain, and the pupils were reactive to light with size 2/2 mm. No newly developed abnormal lesions were found in a brain pre-contrast CT taken 3 hours after respiratory arrest. The air density in the basal cisterns had mostly disappeared, but small air bubbles were still noted in the right jugular fossa ().\nSpontaneous breathing recovered and the patient was weaned off the mechanical ventilator on the second day of intubation. He showed no improvement in consciousness. Brain magnetic resonance imaging (MRI) was performed to evaluate his poor consciousness on the eighth day of respiratory arrest. Ill-defined hyperintense lesions were noted on the right medulla, pons, and some parts of the right cerebellum on T2-weighted images. The lesions mainly showed high signal intensity on diffusion-weighted images with a high apparent diffusion coefficient (ADC) value on ADC map, indicating vasogenic edema. There was an intralesional hemorrhage of dark SI on T2* gradient echo images and small cytotoxic edema in an area of restricted diffusion in the right pons and medulla (). These findings were compatible with neuroimaging findings suggesting venous infarction induced by venous congestion. We started low-molecular-weight heparin (enoxaparin 6,000 IU every 12 hours, subcutaneously) and continued for approximately 72 hours. Unfortunately, the cerebral hemispheres showed diffuse changes compatible with hypoxic brain injury on MRI taken after 8 days from respiratory arrest (). The right sigmoid sinus and jugular vein were occluded on magnetic resonance (MR) venography underwent 9 days after respiratory arrest. On 23th days from respiratory arrest, no injury was observed in the vertebrobasilar artery on catheter cerebral angiography (). The right petrosal vein and sinus were not traced, and contrast-filling defects on the right sigmoid sinus and jugular vein were noted on cerebral angiography (). Conservative treatment was provided for pneumonia, and the patient was tracheostomized. Unfortunately, he did not regain consciousness and survived for only 2 years. |
Our patient is a 40-year-old Asian man with complaints of bleeding and discomfort in his anus of 2 months’ duration. He was an employee with average income who did not smoke tobacco or drink alcohol. He had no weight loss or urinary symptoms, and no substantial family history. He denied any significant medical or surgical history. His abdomen was soft, non-tender, and non-distended, with normoactive bowel sounds. In examination, a mass could be touched by finger tips. The mass was large and bleeding. In subsequent examinations, blood was detected in a stool sample. His vital signs were: blood pressure, 130.77 mm Hg; respiratory rate, 18 breaths/minute; heart rate, 83 beats/minute; and temperature within normal limits. Oxygen saturation was 98% on room air on admission. In colonoscopy, a large lobular tumor was diagnosed at 4 cm above the dentate line, which was suspicious for malignancy. Various samples were taken from the tumor. The rest of his large intestine did not show a clear pathologic lesion in the colonoscopy.\nHigh-grade adenocarcinoma was reported in pathological examinations. In subsequent diagnostic procedures, his carcinoembryonic antigen (CEA) level was normal. Computed tomography (CT) scans revealed that metastatic lesions were not detected in his liver, abdominal viscera, and chest. In CT scans with or without contrast, and magnetic resonance imaging (MRI) scanning, an ectopic kidney was detected incidentally on his right pelvis without any prior urinary symptoms. The left kidney was in its original location, and both kidneys were functional. Renal function tests provided normal results.\nIn subsequent investigations done by MRI scanning for staging the tumor, a pelvic rectum tumor was reported to be interfering with the T3 N1 mesorectal lymph nodes (Figs. , ). The case was discussed in a multidisciplinary cancer team; afterward, our patient was regarded as a candidate for neoadjuvant radiotherapy. He underwent 45 GY radiation in 25 fractions to the pelvis along with capecitabine. He underwent total mesorectal excision (TME) surgery to maintain the ectopic kidney 6 weeks later. After abdominal exploration, his abdominal viscera were examined. There was no metastatic lesion in his liver and abdomen (Fig. ).\nAfter mobilization of the left colon and the splenic flexure, and the closure of the inferior mesenteric artery (IMA), in the avascular plane, the mesorectum was separated from the fascia propria, and the mesorectal lymph nodes and hemorrhoidal vessels in the anterior and pelvic nerves were fully mobilized, and the distal rectum was removed by an appropriate margin (Fig. ).\nHis right kidney was completely inside the pelvis, and while the kidney was carefully protected by the retractor, an attempt was made to minimize the damage to the ectopic kidney because there was a possibility of damage to the pelvic nerve and nephrectomy.\nThe blood of the right kidney appeared to be supplied by the right superior iliac artery. During the surgery, hematuria occurred to our patient, which was resolved by hydrating him. Then, coloanal anastomosis and temporary ileostomy were performed on our patient. He was transferred to our intensive care unit (ICU). He underwent laparotomy again due to anastomosis leakage a week following the surgery. As a result, a colostomy was performed. Postoperatively, after the reappearance of symptoms, stabilization, and healing of the wounds, he was referred to medical oncology and started adjuvant chemotherapy with 5-fluorouracil, folinic acid, and oxaliplatin (FOLFOX). Follow-up testing (for a year) included routine medical history and physical examination (every 3–6 months), blood tests such as serum CEA, colonoscopy, and radiologic imaging. He was dissatisfied with the permanent colostomy after the end of the treatment. However, the satisfying result was that his kidney was preserved (Additional file ). Before the surgery, the potential risks and damage to his ectopic kidney and the possibility of its removal were explained to our patient and his consent was obtained. His general condition is appropriate after 1 year and his quality of life has been reported to be satisfying despite the permanent colostomy. |
A 35-year-old man suffered from immunoglobulin-A nephropathy 2 years before admission and was treated with steroid therapy. He complained of epigastric pain about 9 months before. A mass on the greater curvature of his stomach was found by gastroscopy and gastric cancer (signet cells) was diagnosed by biopsy. The abdominal CT scan showed that there were tumours in the abdominal cavity with enlarged para-aortic lymph nodes. His body surface area was approximately 1.57 m2 as determined from the DuBois formula. He started taking S-1 50 mg, twice daily for 3 consecutive weeks, followed by a 2-week rest period 8 months ago. He also started intravenous cisplatin every 5 weeks and took dipyridamole, lansoprazole, granisetron hydrochloride and naproxen orally every day. He received six cycles of chemotherapy and showed a partial clinical remission.\nHe received 60 mg cisplatin for the last time about 1 month before his admission, and his serum creatinine increased slightly (from 95.47 to 114.92 μmol/l) 1 week after receiving cisplatin. He continued to report appetite loss and went to hospital 1 week before his admission. He also took a blood examination after the consultation and his serum creatinine had increased to 683.33 μmol/l. However, his doctor did not know the result until after his admission. He continued to take S-1 and the other drugs for about 1 week from the start of the renal insufficiency. His appetite loss and general fatigue were aggravated and he consulted the emergency room in our hospital.\nHe was drowsy and on the Glasgow Coma Scale his score was 13 of 15. His blood pressure was 133/82 mmHg and his heart rate 87 beats/min. He complained of a slight stomachache, and slight tenderness was found on physical examination. There was no free air or niveau on the abdominal X-ray. The serum creatinine and blood urea nitrogen were elevated to 1919.16 μmol/l and 32.49 mmol/l, respectively. The patient was anuric. A nephrological consultation was requested; the patient appeared to be dehydrated and there were no signs of hydronephrosis on abdominal ultrasonography. His acute kidney injury was ascribed to a combination of drug-induced kidney injury and dehydration. All drugs were withdrawn and HD was performed for 3–4 h with an APS-11MD dialyzer (Asahi Kasei Medical Co. Ltd) and with a blood flow between 120 and 150 ml/min on the second, third, fifth and sixth day of hospitalization; the plasma concentrations of 5-FU and CDHP were measured.\nOn the fifth day his urine output gradually increased and the serum creatinine decreased to the previous value (serum creatinine decreased to 85.75 μmol/l on the eighth day). The plasma concentration of 5-FU decreased from 840 ng/ ml before the first HD to 93.4 ng/ml after the second HD (Figure ). His HD had continued till the sixth day.\nHowever, on the fourth day, diarrhoea and stomatitis appeared till the sixth day. He was diagnosed with pancytopenia (Figure ). Granulocyte colony stimulating factor (G-CSF) and antibiotics were administrated, and on Day 7 the neutrophil count decreased to approximately 50/mm3. Finally, the neutrophils increased on Day 18 and the fever decreased, together with improvement of the fever, the stomatitis and diarrhoea. The patient could be discharged on Day 26 after admission. |
We describe the successful use of a continuous ESP block for a 57-year-old woman who underwent left pneumonectomy and single lung transplantation via posterolateral thoracotomy incision complicated by atrial fibrillation requiring cardioversion and subsequent therapeutic anticoagulation with heparin. The patient did not receive any preoperative regional techniques as per our typical institutional practices for lung transplantation at the time. The patient developed atrial fibrillation during surgery that was not responsive to repeated attempts at electrical cardioversion and was started on an amiodarone infusion at 1 mg/min immediately postoperatively. Atrial fibrillation persisted, and 8 hours postoperatively, a heparin infusion was initiated at 15 units/kg/hr in anticipation of repeated cardioversion with a goal of activated partial thromboplastin time of 2 times greater than baseline. Three hours after surgery, the patient was extubated and transitioned to heated Hi-Flow Nasal Cannula (HFNC) at fraction of inspired oxygen (FiO2) of 65% at 50 liters (L) to maintain her oxygen saturation >88% on continuous pulse oximetry. For the first 24 hours postextubation, the patient maintained adequate oxygen saturation on HFNC with a range of 40–70 L and FiO2 of 45–65% without attempting to ambulate. Shortly following extubation, the patient reported uncontrolled pain, despite repeated administration of intravenous opioids (pain scores 5/10 at rest and 10/10 with exertion), with resultant difficulty participating in adequate pulmonary hygiene (). Pain scores were assessed as per standard nursing protocol every 4 hours on a 0–10 numerical rating scale, and oxygenation was assessed with continuous pulse oximetry.\nFollowing extubation, the patient was continued on her baseline dose of 10 mg oxycodone every 8 hours for chronic back pain and was additionally administered intravenous Toradol 15 mg every 6 hours and oral acetaminophen 650 mg every 6 hours for the first 7 postoperative days. Thirty-six hours following extubation, the patient continued to exhibit difficulty to control pain (10/10 with ambulation) and continued to have respiratory compromise despite HFNC of 60–70% at high flows. During a 130-foot walk, the patient was unable to maintain oxygenation saturation levels above 87% despite continued use of HFNC with FiO2 of 70% and 70 L. In the first 36 hours postoperatively, the patient consistently reported pain scores ranging from 8 to 10. After obtaining informed consent, an ESP catheter was performed as described by Forero et al. []. At the time of ESP catheter placement, systemic anticoagulation with heparin was maintained with a therapeutic partial thromboplastin time (PTT) of 79.7 seconds. A high frequency linear ultrasound transducer (BK Medical Flex Focus 400, Peabody, MA) was positioned in a longitudinal parasagittal orientation 3 cm lateral to the T5 spinous process, and the erector spinae muscle was identified. A 17 gauge, 5 cm Tuohy needle (Arrow® StimuCath® Teleflex Medical, Morrisville, NC) was advanced from cephalad to caudad, in-plane to the ultrasound probe, in order to achieve a final position deep to the erector spinae muscle. This location was confirmed with visualization of 0.2% ropivacaine 15 ml spreading cranially and caudally beneath the erector spinae muscle. Subsequently, a 19 gauge, single orifice, wirewound catheter (Arrow) was advanced, such that 3 cm remained beneath the erector spinae muscle. Following a negative test dose, an additional 0.2% ropivacaine 10 ml was administered through the catheter. A continuous ESP infusion utilizing an elastomeric pump was then initiated: 0.2% ropivacaine at 8 ml/hour continuous infusion; the patient controlled a bolus dose of 0.2% ropivacaine at 5 ml every 30 minutes and a 16 ml/hour lockout dose. The ESP catheter and infusion were maintained for 92 hours, during which time, the patient endorsed lower pain scores, no additional intravenous opioid medications for breakthrough pain, improved oxygenation, and stronger cough (). The patient continued to demonstrate paroxysmal atrial fibrillation and was thus continued on heparin infusion with a therapeutic range of partial thromboplastin time for the first 6 postoperative days and was then transitioned to warfarin therapy. Heparin infusion was not interrupted for catheter removal. No apparent complications related to the ESP catheter were noted following catheter removal on physical examination during the remainder of the patients' lengthy hospital stay. |
A 2.8-kg-one-day old female baby was brought to the hospital with respiratory distress and multiple growth protruding from oral cavity []. The child had macrostomia, cleft palate, bifid tongue, and two oral growths one measuring approximately 25 × 20 × 15 mm attached to hard palate and other in the sublingual region measuring approximately 12 × 10 × 15 mm. Computed tomography (CT) finding showed large well defined cystic lesion of size 23 × 17 × 12 mm in left parasagittal palatal region with internal fat fluid density and calcified densities (Hypertrophied palatal bones). Similar small cystic lesion of size approximate 11 × 8 × 13 mm is seen within oral cavity at sublingual region. On examination, the child was vigorous, tachypneic, and there was flaring of ala nasae. Mallampati grading could not be assessed.\nExcision of the tumor was scheduled on the day 3rd of life of the baby.\nOn arrival in operating room, routine monitoring (electrocardiogram, pulse oximeter, noninvasive blood pressure, and temperature) were attached. Difficult intubation cart and pediatric bronchoscope were kept ready to encounter any difficulty in intubation, and even surgical team were also standby to perform emergency tracheostomy if required. Oxymetazoline nasal drop was put in both nostril and patency of nare was checked by nasogastric feeding tube to rule out any extension of mass. Injection atropine 0.01 mg/kg was given through already secured intravenous access to limit secretions. For preoxygenation, a bigger size transparent circular silicon face mask was selected along with surgical pads to form a seal around the nose, lower jaw, and the mass to ensure proper bag and mask ventilation without causing any trauma to mass. Preoxygenation was done for 5 min. Once it was established that mask ventilation was possible, anesthesia was induced by sevoflurane and oxygen in gradually increasing concentration. When the patient was anesthetized, the mass was covered with surgical pads to avoid any injury to it and a gentle check laryngoscopy was done to visualize the glottis with Miller's blade 1.5; as glottis was visible, injection succinylcholine 1.5 mg/kg was given. After adequately lubricating the endotracheal tube (ET) with 2% lignocaine jelly, right nasal intubation was done with 3 mm internal diameter uncuffed ET by using Magill's forceps. Tube was fixed on the anterior surface of the nose. Injection fentanyl 2 μg/kg and injection atracurium 0.5 mg/kg were given. Anesthesia was maintained with oxygen, nitrous oxide, and sevoflurane. Pharyngeal packing was done by the surgeon before performing procedure. Sublingual mass was excised completely while mass at hard palate could be excised in piecemeal []. There was not significant blood loss and hemostasis was done. The duration of surgery was 40 min. At the end of surgery, neuromuscular blockade was reversed by injection neostigmine 0.07 mg/kg and injection glycopyrrolate 0.02 mg/kg and extubation could be done smoothly []. Patient was shifted to postoperative care unit. Nasogastric feeding was started on 2nd day and on fourth postoperative day, oral feeding was started and patient was discharged on fifth postoperative day. |
A 73-year-old woman with a history of breast cancer with liver metastasis presented with increasing pain in the left eye more than the right, associated with increasing blurring of vision in both eyes 3 months after beginning PTX. She was diagnosed with chronic posterior blepharitis associated with mild symblepharon formation 7 years ago. Her ocular history was otherwise unremarkable. Mild inferior pannus was noted and there was no evidence of LSCD. No lagophthalmos or lid abnormalities was noted in either eye. Breast cancer was diagnosed 3 years ago and liver metastases less than 1 year ago. She was initially started on doxorubicin and cyclophosphamide for 12 weeks, then docetaxel and trastuzumab for the next 12 weeks with dosing every 3 weeks. She responded well to treatment and was carried on with trastuzumab for another 52 weeks. For the past year she has been taking exemestane 25 mg daily, however given a recurrence of her breast cancer as liver metastases, she was started on weekly PTX 80 mg/m2 and trastuzumab 8 mg/kg. While on her third month of PTX with good response, she presented to the Cornea Service with decreasing vision associated with redness and pain. Her past medical history included non-insulin dependent diabetes mellitus well-controlled on metformin. She had never been at soft contact lens wearer. Upon examination, best-corrected distance visual acuity was 20/100 in the right and counting fingers in the left eye. Bilateral stable temporal and nasal small symblephara were noted since her initial visit in 2006 (Fig. a, b). Blunting of the corneal sensation was noted on the left. Peripheral neovascularization, peripheral stromal scarring, and a whorled epitheliopathy staining late with fluorescein consistent with LSCD from 10 to 5 o’clock were present on the right cornea. A central neurotrophic ulcer measuring 1.4mmx2.8 mm, with central stromal haze and thinning to 50%, and complete LSCD with 360 degrees of conjunctivalization staining late with fluorescein were noted on the left cornea (Fig. ). Intraocular pressures were 14 in both eyes. There were mild nuclear sclerotic lens changes noted bilaterally.\nDifferential diagnoses for LSCD in this patient included chronic posterior blepharitis, paraneoplastic ocular cicatricial pemphigoid (OCP) and LSCD secondary to PTX toxicity. LSCD secondary to other ocular surface disorders such as neurotrophic keratopathy has been reported. Given that there was blunting of the corneal sensation in the left eye, this factor must also be considered []. A large conjunctival biopsy showed chronic inflammation and fibrosis, with no pathological or immunohistochemical features of OCP. Her chronic blepharitis had been documented to be under control and stable without significant flares over the years.\nA provisional diagnosis of LSCD secondary to PTX was made. She was started on a regimen of preservative-free artificial tears, preservative-free prednisolone (0.5%) eyedrops 4 times daily, and autologous serum tears 4 times daily. A temporary lateral tarsorrhaphy performed 2 weeks later did not reduce the non-healing epithelial defect over another 2 weeks. Thus, PTX was discontinued by her oncologist and she was switched to doxorubicin. A month later, the epithelial defect was completely healed although her vision remained at count fingers on the left due to central corneal scarring. The tarsorrhaphy was subsequently released, and her ocular surface remained stable over 19 months. The vision and extent of LSCD remained unchanged on the left. The right eye showed recovery of best corrected vision to 20/30 with only mild peripheral pannus noted. |
A 2.8 kg, 4-day-old boy, the second child of non-consanguineous parents, was referred to our institute for the management of respiratory distress and septicemia. He was born by normal vaginal delivery after an uneventful antenatal period, and was mildly depressed at birth with Apgar score of 1 and 6 at 1 and 5 minutes respectively. He was resuscitated with bag and mask to which he responded and then was shifted to the intensive care unit on oxygen hood. His condition improved slowly and he was weaned off oxygen over 72 hours. He was administered expressed breast milk feeds on day four but developed choking, aspiration of feeds and respiratory distress.\nOn arrival, the baby was floppy, had a weak cry and shallow breathing. The neonate's trachea was intubated with a 3.5 mm endotracheal tube (ETT) and fixed at a length of 9 cm. Lungs were ventilated with help of a pressure controlled ventilator with FiO2 0.7 and moderate pressures. Chest X-ray showed aspiration pneumonitis in the right upper lobe. Patient was started on second line antibiotics, Cefotaxim and Amikacin. Blood culture grew Klebsiella pneumonei and antibiotics were changed to meropenem according to the sensitivity. Patient developed multiple episodes of desaturations associated with dislodgement of ETT to the esophagus. It was observed that when ETT was pushed further in the trachea, the baby ventilated better and there was no ETT dislodgement []. This raised the possibility of a congenital anomaly of the airway.\nFurther evaluation confirmed a small patent ductus arteriosus and bilateral retinal coloboma. Computerized tomography (CT) scan chest showed evidence of a defect in posterior larynx at the level of vocal cords and inflammatory changes in the left upper lingual and right lower lobe. A diagnostic bronchoscopy was done under general anesthesia to confirm the diagnosis. Patient was given fentanyl 4mcg and atropine 0.4mg intravenous (IV). Anesthesia was induced by inhalation of air, oxygen and sevoflurane. Muscle relaxation was achieved with atracurium 1mg IV. Rigid bronchoscopy with a 2.5 mm sized scope showed a large LTEC extending up to the middle of the trachea (Type III LTEC) []. The bronchi were normal.\nRepair of cleft was performed after two days under general anesthesia. Before the surgical repair, a large bore (10F) nasogastric tube was placed and the ETT was re-enforced at the angle of the mouth with a stay suture. Monitoring included pulse oximetry, capnography, electrocardiogram, temperature, noninvasive blood pressure and urine output. There was a 2.5 cm long posterior laryngeal cleft extending from just below the vocal cords till the suprasternal notch with a common tracheo-esophageal passage. The cleft was repaired by placing long strips of sternomastoid muscle between trachea and esophagus. The trachea was repaired over the 3.5 mm ETT. Esophagus was repaired over the nasogastric tube []. We did not encounter any tube displacement during surgery. A tracheostomy tube was placed through the third tracheal ring. The ETT was pulled up above the tracheostomy and was fixed. It was kept as a stent for one week. Analgesia was provided by intermittent IV doses of fentanyl. The surgery was completed uneventfully over 2 hours. The oxygen saturation and hemodynamic parameters were stable during this period. Blood loss was minimal and 25ml of 5% dextrose with half normal saline was given as infusion during surgery.\nPostoperatively, the baby was stable and total parenteral nutrition followed by expressed breast milk feeds were started via the nasogastric tube. The baby was weaned off ventilator after 4 days and given oxygen supplementation via T-piece over the tracheostomy. Two weeks after the surgery, patient developed copious drooling of secretions. Bronchoscopy under general anesthesia ruled out the possibility of a fistula but endoscopy revealed esophageal stricture which was dilated using Savary-Gilliard dilators and feeds were resumed. Patient had swallowing difficulties due to pharyngo-esophageal dysfunction postoperatively. Later patient developed significant aspiration of feed and respiratory distress which required re-intubation; however, he deteriorated further and developed disseminated intravascular coagulation with sepsis and died at two and a half months of age, despite supportive measures. |
A 64-year-old woman was admitted to our hospital with a pulsatile mass swelling over the left cervical region and right hemiparesis after cough for 1 day. She had a history of fibromatosis, but no previous history of trauma, operation or inflammation in this region. On physical examination, a 5-cm large pulsatile swelling with blowing bruit was found over the left cervical region and multiple fibromas were found in regions of face, neck, and trunk. Neurological examination revealed muscular strength of grade IV according to the ‘manual muscle test (MMT)' grading system and hypoesthesia of right limbs, including touch and pain sensation. Computed tomography angiography (CTA) and DSA revealed a left high-flow internal carotid-jugular fistula at the first cervical level and twisted left internal carotid artery (ICA) (Figures ). Blood flow from the vertebrobasilar artery and right ICA via the circle of Willis supplied the left anterior cerebral artery and middle cerebral artery and drained backwards into the petrosal segment of left ICA (Figures ).\nWe chose embolization of both the fistula and parental artery, because no appropriate covered stent could be used to pack the fistula in the condition of reservation of left ICA. The purpose of first endovascular treatment was to isolate the fistula, by blocking both the backward blood flow from right ICA and the forward blood flow from left ICA. A Headway-21 stent catheter was selectively inserted into the distal part of fistula in the segment of carotid cavernous sinus, meanwhile an Echelon-10 microcatheter was placed in the distal part of stent catheter. One LVIS 5.5*30 mm stent, with its characteristic relatively compact mesh, was used to cover the distal part of fistula to avoid coils being pushed into internal jugular vein and heart (Figure ). However, the 3.5 cm fistula could not be covered by the LVIS stent. Therefore, 10 coils were additionally used one by one, to block the fistula from the distal to proximal part through an Echelon-10 microcatheter (Figure ). When considering the fast-backward blood flow from the right ICA after embolization, we could not guarantee the safety of blocking by Onyx. We chose one detachable balloon to block the ICA near the proximal part of fistula to stop the forward blood flow, and the backward blood flow would be stopped in the second treatment (Figures ).\nThe blowing bruit was reduced significantly after the first treatment, but became worse after 2 months later. The patient was admitted to our hospital again, and DSA revealed residual blood flow in the fistula from the left ICA and premature balloon deflation (Figures ). It was fortunate that LVIS stent blocked the balloon into the internal jugular vein. An additional 13 coils were used to pack the fistula with double-microcatheter techniques, under the multi-angle DSA projection, to avoid the coil protruding into the internal jugular vein (Figures ). Afterwards, two detachable balloons were used to block the proximal part of left ICA again (Figure ). DSA revealed the forward blood flow disappeared, the low-flow backward blood from right ICA still supplied the fistula, and a normal ipsilateral jugular vein (Figure ). Because the fistula was mostly blocked, and the low-flow backward blood may promote the formation of thrombosis in the distal part of fistula, we stopped the second endovascular treatment and planed a DSA examination to evaluate the effect of embolization and to determine further treatment. After this treatment, the patient did not feel the blowing bruit or any other discomfort.\nHowever, the patient complained of swelling and pain in the left occipitocervical region 2 days later. Physical examination revealed a large subcutaneous mass, with volatility and severe tenderness. Emergent CT revealed a subcutaneous hematoma in left occipitocervical region, without abnormalities in brain (Figures ). DSA in the hybrid operation room showed the coils in the fistula were stable without no forward blood flow in the proximal part of fistula. However, there was still a little backward blood flow through the right ICA and vertebrobasilar artery into right internal jugular vein, via the distal part of fistula. The lower part of fistula was not shown, because the blood drained into the right internal jugular vein through the sigmoid and transverse sinus reversely (Figures ). It was suggested that the occipitocervical subcutaneous hemorrhage was caused by poor ipsilateral jugular drainage, which was the result of the formation of thrombosis in fistula extending to the left internal jugular vein, thus blocking forward and backward reflow. We inserted an Echelon microcatheter through the left vertebrobasilar artery-posterior communicating artery, then selectively to petrosal segment of the left ICA, where 3 coils were put in, and then a 1.2 ml Onyx-18 was injected slowly. DSA examination after this procedure revealed that the backward blood flow distal to the fistula disappeared (Figures ). An open operation to remove the hematoma was preformed after the interventional operation. Multiple capillary hemorrhages were found from behind the sternocleidomastoid muscle after removing clots. The operation to stop bleeding was successful, and the postoperative course was uneventful. A 6-month follow-up DSA demonstrated a totally occluded fistula allowing the patient to engage in light manual labor with normal neurological functioning after the operation (Figures ). |
A 65-year-old white male presented to our clinic for a second opinion. Seven years earlier, he noticed a skin rash over the anterior part of his neck. He was initially evaluated by a dermatologist, who prescribed topical steroids leading to temporary improvement of the lesion. Unfortunately, the rash returned and was associated with the tightening of the skin over his neck.\nThree years later, despite the occasional use of topical steroids, the skin tightness started to spread down his body, affecting his chest, upper and lower extremities. The patient underwent multiple skin biopsies, and the histopathological report was consistent with lymphocytic infiltration, possibly EF. Peripheral eosinophilia was also present.\nBased on the biopsy report, the patient was referred to a rheumatologist who diagnosed his disease as EF and first prescribed Plaquenil, and then, due to a lack of improvement, switched to 15 mg of methotrexate (MTX) orally once a week, 1 mg of folic acid orally once a day, and prednisone orally as needed.\nDespite being compliant with his regimen, the skin over his body was getting progressively tighter, and was associated with a lack of energy and joint pain that affected his hands, elbows, knees, and feet. The skin around his neck became so indurated that swallowing solid food was difficult. He developed contraction of the upper and lower extremities associated with severe muscle weakness, requiring a wheelchair for daily activities.\nThree years prior to presentation in our clinic, he had developed a diffuse erythematous rash all over his body requiring hospitalization and was subsequently diagnosed with Stevens-Johnson syndrome and was treated with high doses of steroids.\nAt the time of our evaluation, the patient denied chest pain, skin rash, shortness of breath, Raynaud’s phenomenon, uveitis, episcleritis, mouth ulcers, or blood in his urine or stools. Physical examination showed a debilitated, frail male who appeared older than his age. Eyes, heart, lung, and abdominal examinations were unremarkable.\nDuring the oropharyngeal evaluation, he could not open his mouth fully nor stick his tongue out of the oral cavity. The musculoskeletal evaluation showed diffuse muscle atrophy and proximal and distal muscle weakness (grade 4-/5+) of the upper and lower extremities (). Reduced range of motion of the knees, elbows, neck, ankles, and feet associated with severe contraction of these joints was noted. Osteoarthritis of both knees was also noticed. There was no evidence of synovitis.\nSkin evaluation demonstrated indurated and thickened skin (peau d’orange) over the face, neck, anterior chest, with the upper and lower extremities extending to the fingers and toes bilaterally. There was a groove sign in both forearms (). Neurologically, reduced sensation at the level of both feet was noted Cranial nerves II-XII were intact. Complete blood count, renal function tests, liver enzymes, aldolase, and creatine kinase levels were normal.\nAntinuclear antibodies (ANA), anti-Ro (SSA), anti-La (SSB) antibodies, anti-Smith, anti-Scl-70, anti-centromere, and anti-Jo antibodies were all negative. Erythrocyte sedimentation rate and C-reactive protein were within normal limits. Magnetic resonance imaging (MRI) of the right lower extremity, with and without a contrast agent, showed the presence of diffuse edema and enhancement throughout the thigh musculature. There was no fascial enhancement, abscesses, or evidence of osteomyelitis. Electromyography examination showed the presence of severe sensorimotor polyneuropathic and myopathic processes. Biopsies of the fascia and vastus lateralis muscle are described below ( and ).\nDue to the diagnosis of EF and DM, we decided to continue treatment with 15 mg of MTX orally once a week and daily folic acid to control the EF and initiate therapy with two doses of rituximab 1000 mg given 15 days apart in order to treat DM. We also initiated prednisone therapy – 40 mg orally over a month with slow taper due to the severity of muscle weakness. |
An 18-year-old female was admitted to the emergency room with an alleged history of attempted suicide in which an unknown quantity of paraquat (liquid form) was consumed at her residence. The patient was managed at local hospital with (intravenous (IV) fluids, antiemetic, and H2 blockers) and brought to our hospital for further treatment after 24 h. She had difficulty in opening her mouth and a decreased urine output. There was no history of vomiting, loose stools, abdominal pain, seizures, or fever. At the time of examination, the patient was conscious and oriented. There was neck edema. Examination of the oral cavity showed mucosal erosion of tongue, palate, and lips with oral bleeding []. On clinical examination, the pulse rate was 98 beats per min, regular, blood pressure (BP) was 130/80 mmHg, with respiratory rate of 22 per min. Cardiovascular system was normal. There was difficulty in breathing, but there were no added sounds on examination of the respiratory system. Pupils were bilaterally equal and reactive to light. The patient was intubated as there was associated laryngeal edema and difficulty in breathing. Gastric lavage was performed and charcoal was given in the emergency department. She was kept on elective ventilation. In the intensive care unit (ICU), she received IV fluids and an antiemetic as a supportive measure. Her initial chest X-ray showed a left lower zone infiltrate []. She had high serum urea (221 mg/dL) and creatinine (8.78 mg/dL) on the day of admission that gradually reduced to normal after two sessions of dialysis. Blood and urine cultures were sterile. Other blood investigations including thyroid and liver functions were normal. Urine examination was normal. Ultrasound of the abdomen showed bilateral grade I changes in the kidneys. Two-dimensional (2D) echo and electrocardiogram (ECG) were normal. upper gastrointestinal (GI) endoscopy showed corrosive injury to the esophagus and proximal stomach. Endotracheal culture showed Klebsiella pneumoniae. She received intravenous methylprednisolone 1 g in 200 ml normal saline every 2 h daily for 3 days, IV cyclophosphomide 750 mg (15 mg/kg/day) in three divided doses (in 200 ml of N-saline over 2 h) for 2 days followed by IV dexamethasone 5 mg 6 hourly, injection N-acetylcysteine 2 g stat followed 1 gm TID for 5 days, vitamin C (500 mg/amp) 6 g/day IV, vitamin E (400 i.u./tab) 2 tabs QID. The patient did not respond to treatment and expired on the 12th day post injury as a result of septicemia and respiratory failure. |
A healthy 35-year-old male with no medical complication resorted, complaining of a dull pain in the mandibular first molar area on the left side. He stated that RCT of the aforementioned tooth was done by a general practitioner one year earlier. Upon clinical examination the tooth contour showed that it had been prepared for prosthetic crown which according to the patient was later removed to help in eliminating the tooth abscess. The tooth had a defective discolored composite build-up and was not mobile or tender to percussion. Visual scanning revealed a local tender inflammation overlying the buccal mucosa in the furcal region. A draining sinus tract was evident ~2 mm from the gingival margin within the keratinized mucosa. Careful periodontal probing of the tooth showed that pocket depth was within the normal range (<3mm). On a parallel radiography, previous RCT had a moderate quality. A large inter-radicular lesion was evident (). The sinus tract was traced with a #30 gutta-percha point (Ariadent, Tehran, Iran) and according to the second radiograph, its path did not lead to the root apices indicating that the lesion was not related to the apical and middle zones of the root canals (). On both cliché, an opaque bulk of material was evident on the coronal section of the mesial root filling that suggested the existence of an unusual event (i.e. strip perforation) and dentist’s effort to seal off that area which could potentially be the source of lesion ( and ).\nThe possible treatment options including tooth extraction with/without replacement and perforation repair with orthograde re-accession and coronal restoration were explained for the patient. In accordance with the patient preferences, the option of saving the tooth via strip perforation repair with CEM cement was chosen. The patient signed an informed consent.\nAfter administering 0.2% chlorhexidine rinse (Behsa Co., Tehran, Iran), the tooth was isolated. The restorative material was removed and all canal orifices were located. The coronal ~4-5 mm of the root filling material was extirpated and 5.25% NaOCl was left in the canals for ~5 minutes. Meanwhile CEM cement (BioniqueDent, Tehran, Iran) was prepared according to manufacturer's instruction. After drying the canals with paper points (Ariadent, Tehran, Iran), CEM cement was placed into the orifices. The biomaterial was gently packed with a dry cotton pellet and appropriate paper points to obtain a good adaptation. Then it was covered with a moistened cotton pellet and the tooth was temporarily restored (Coltosol; AsiaChemiTeb Co., Tehran, Iran). A control post-operative radiography showed the flow of CEM filling through the perforation site into the lesion that confirmed the pre-operative diagnosis ().\nOn a subsequent visit one week later, all sings/symptoms had subsided and the buccal swelling in the gingiva had faded away. The patient was referred for the prosthetic treatment of the tooth. One-year follow-up radiography revealed complete healing of the lesion and its replacement with bone (). The tooth was totally functional and symptomless. |
A 66-year-old woman with a significant past medical history of well-controlled hypertension was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was within normal range at that time and therefore the anti-GBM antibody was not tested. The first renal biopsy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus (Fig. ), and deposition of IgA and C3 in mesangial areas by immunofluorescence microscopy (Fig. ). Weak but significant IgG deposition was also observed in glomeruli in the distribution somewhat different from IgA or C3 (Fig. ). The electron-dense deposits were observed in mesangial areas by electron microscopy. Therefore, the diagnosis was IgA nephropathy. Antihypertensive therapy was initiated, mainly with an RAS inhibitor. Eight months later, the patient’s serum creatinine suddenly rose to 4.53 mg/dL (it was 1.04 mg/dL from the routine blood test 1 month before). Urinalysis showed 100 red blood cells per high power field and urinary protein excretion of 12.3 g/gCr (Fig. ). The serological tests that were performed to differentiate the cause of rapidly progressive glomerulonephritis revealed the presence of anti-GBM antibody at the titer of 116 IU/mL and the absence of anti-nuclear antibody and anti-neutrophil cytoplasmic antibody. Laboratory findings on admission are summarized in the Table .\nAfter admission, treatments with hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy followed by oral prednisolone at the dose of 50 mg/day were initiated. The second renal biopsy was performed at 4 weeks after admission in order to assess the probability of renal recovery and to make the final diagnosis. It revealed cellular to fibrocellular crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli by light microscopy. By immunofluorescence study, linear IgG deposition along the glomerular capillary walls and mesangial staining for IgA were observed. On the other hand, C3 deposition was observed in the mesangium as well as in the glomerular capillary walls (Fig. ). Electron-dense deposits were observed in mesangial areas, similarly as in the first biopsy, by electron microscopy (Fig. ). Based on the aforementioned findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Plasmapheresis was performed eight times, anti-GBM antibody gradually decreased, and alveolar hemorrhage was prevented. However, her renal function could not be restored and she underwent maintenance hemodialysis (Fig. ).\nAdditional immunosuppressant was not given because the patient did not show any sign of pulmonary involvement and because the renal recovery was quite unlikely from clinical (continuous oliguria and hemodialysis dependence) as well as histological (crescent formation in most of non-sclerotic glomeruli) point of view.\nClinical and histological presentations from IgA nephropathy (at the time of first renal biopsy) and from anti-GBM disease (at the time of second renal biopsy) were summarized in the Table . |
A 19-year-old girl affected by XP had come to our attention for a suspected advanced cSCC.
She was born in Sicily and, initially, she was followed by the dermatological centers in that region. No other family member was affected by the same pathological condition and her parents were not consanguineous. In her medical history, ophthalmic and neurological affections were not reported. Regarding skin involvement, from 5 to 13 years of age, she underwent repeated surgical excisions for a total of 24 neoformations which, on histological examination, were found to be basal cell carcinomas (BCCs) and cSCCs. The last resection was at the age of 14 due to a cSCC of the right lower eyelid. At 18 years, she received vismodegib treatment for an advanced BCC developing alopecia as an adverse event.
We firstly visited the patient in December 2018. On skin examination, a large hard swelling of the right superior eyelid extending to the nasal pyramid has been observed. An incisional biopsy was performed, resulting in a moderately-differentiated SCC. A CT scan was carried out to exclude secondary localizations. The MRI of the facial massif showed an infiltrating lesion of 62 × 47 mm. It extended from the right upper eyelid region affecting the orbital cavity’s medial wall and dislocating the eyeball from which it appeared inseparable. It jutted medially into the nasal cavities infiltrating the nasal septum and, below that, it crossed the floor of the orbit projecting into the ipsilateral maxillary sinus.
The interdisciplinary consultation did not indicate surgical treatment, therefore the anti PD-1 cemiplimab was requested in the nominate use programme and it was decided to add radiotherapy. Pending authorization of the drug, the patient was qualified for proton beam radiotherapy (PBR), which was performed with a total dose of 59.4 Gy in 33 fractions from January to March 2019. The treatment was well tolerated and, in consideration |
A female patient aged 22 years presented with a swelling on the left side of the face since past 4 months, which gradually enlarged up to the present size. Patient gives history of generalized weakness, lethargy and weight loss noticed since past few months. Her family history and past medical history was non-significant.\nOn extra-oral examination a diffuse swelling was noticed on left side of the face, measuring approximately 6 cm × 7 cm, extending superiorly from the infraorbital margin to inferiorly 3 cm below the lower border of mandible, medially 2 cm away from the ala of nose to tragus of the ear laterally (). The skin over the swelling was shiny and stretched. There was no ulceration or erythema noticed over the swelling. On palpation, swelling was firm to hard and tender.\nOn intraoral examination, an ulceroproliferative growth in the vestibule on the left side, extending from the mesial aspect of lower left canine to the retromolar region posteriorly. Obliteration of buccal and lingual vestibule was observed. The growth was covered with pseudomembranous slough, and the indentations of upper molars were seen over the swelling. The swelling has displaced lower first premolar buccally and lower second premolar lingually, lower first molar anteriorly and lower second and third molars posteriorly ().\nOn palpation swelling was soft to firm in consistency and non-tender. No associated discharge or bleeding noticed. Left submandibular lymph nodes were palpable which was tender and firm in consistency. Provisional diagnosis of ameloblastoma and a differential diagnosis of aneurysmal bone cyst, odontogenic cyst were given. Radiographic examination with orthopantamogram showed large unilocular radiolucency on the left side, extending from mandibular first premolar to mandibular third molar without involving the lower border of mandible. There was generalized decrease in density of mandible, and there was resorption of roots of teeth from second premolar to the second molar ().\nPatient was advised blood investigations that revealed alkaline phosphatase and PTH levels of 762 IU/L (Normal: 36-14 IU/L) and 452.5 pg/ml (Normal: 12-65 pg/ml) respectively. Serum calcium level was 12.5 mg/dl (Normal: 8.5-10.5 ml/dl).\nPatient underwent high-resolution sonography of neck, which revealed a well-defined lesion below the lower pole of the thyroid on the right side between the carotid and trachea measuring about 2.25 cm × 0.46 cm. The lesion had hyperechoic solid component and cystic component with septations. A parathyroid technetium scintiscan (99Tcm SESTAMIBI; Technetium-99 MIBI; methoxy-isobutyl-isonitrile) findings were showing abnormally high uptake is observed at the lower pole of the thyroid lobe interpreted as hyperplasia of right inferior parathyroid with possible brown tumor of mandible ().\nAn incisional biopsy of the mandibular lesion was done which revealed hemorrhagic fibrovascular connective tissue with multinucleated giant cells consistent with the diagnosis of a giant cell lesion. Parathyroid adenoma excision was done under general anesthesia. Recovery was uneventful and patient discharged after 1 week. The resected gland was histologically suggestive of hyperplasia. The surgical removal of the bony lesion was done by curettage. Patient was given oral calcium supplementation in addition to vitamin D3 for possible post-operative hypocalcemia. Considerable reduction in the size of the oral lesion was noted after 6 months. |
A 52-year-old Caucasian male with history of bipolar disorder was brought in by his son to the emergency room (ER) following severe bilateral injuries to his eyes as an attempt to self-enucleate. The patient called his son to his bedroom after attempting to manually pull his eyes out with his fingers because he was instructed to do so by God and felt the need to cleanse myself from sin. The patient was transferred to the ER by ambulance and appeared calm. The son reports that the patient had been acting strange and displayed decreased appetite and increased wakefulness. He was noted to draw complex numerical pyramid schematics in a notebook, and endorsed that God was speaking to him more over the past week. He was diagnosed with bipolar disorder 18 years ago with most recent manic episode occurring 16 years ago, resulting in a failed suicide attempt by hanging and subsequent hospitalization. Since then, the patient followed up with an outpatient psychiatrist on Risperdal 1 mg every night (QHS) and lithium 900 mg QHS and was otherwise noted to be highly functional and performed well at a high functioning professional. The patient had no other history of self-harming behavior.\nOn mental status exam he appeared well groomed with good hygiene and was dressed appropriately for the weather. Speech was within normal limits for rate, rhythm, and volume. He exhibited normal psychomotor behavior; mood was good, and affect was inappropriately flat given the severity of his injuries. He related to his examiners in a nonchalant demeanor. His thought process was linear and goal-directed as evidenced by his stating I do not feel any pain or remorse, I feel I have done the right thing. What I was supposed to do and I am cleansed now. He endorsed auditory hallucinations of God’s voice, and denied visual hallucinations, paranoid ideation, and suicidal or homicidal ideations. He had no overt impairment of impulse control and was able to answer questions appropriately. His insight and judgment were impaired.\nOn ophthalmology exam the patient’s visual acuity (VA) was no light perception (NLP) for both eyes (OU). The patient’s eyes were found to have severe proptosis, extensive conjunctival lacerations and hemorrhage, and visibly avulsed extraocular muscles (). A CT scan of the orbits demonstrated small and irregular globes, air within the orbits, and intraocular hemorrhage (). Multiple foci of air and soft tissue stranding is also seen (). The optic nerves were on stretch with posterior tenting of the globe suggesting severe optic nerve injury. Due to the optic nerve injury, the patient was started on intravenous (IV) methylprednisolone to reduce orbital edema and optic nerve inflammation and placed on IV and topical antibiotics. He was taken to the operating room by ophthalmology to repair the conjunctival lacerations, reattach the extraocular muscles and to limit the exposure of his eyes by detaching his lateral canthal tendons and suturing his eyelids closed with tarsorraphies ().\nPostoperatively, the patient was admitted inpatient for medical and psychiatric stabilization. The psychiatry team evaluated the patient and gave the diagnosis of bipolar mania with psychotic features, with the most recent episode constituted by hyperreligious delusions and command auditory hallucinations. When he arrived on the inpatient psychiatric unit he was disorganized, responded to internal stimuli, and expressed thoughts of self-harm such as cutting off his arm to further cleanse himself. As his lithium level was therapeutic on admission (1.22), his mood stabilizer was switched to Depakote 1500 mg QHS and Risperdal was increased to 1 mg twice a day (BID). An ophthalmic examination one week after surgery demonstrated NLP vision OU with nonreactive pupils OU and complete ophthalmoplegia. Over the course of 3 weeks he slowly gained insight to the extent of the damage he caused and expressed some regret. He was discharged in stable psychiatric condition with close outpatient psychiatric follow-up. |
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