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A 60-year-old G3P3003 female not taking hormone replacement therapy was referred to the gynecology clinic for iron deficiency anemia suspected to be secondary to postmenopausal uterine bleeding. She entered menopause at age 50 and had not had any uterine bleeding since that time. The patient reported severe bleeding with associated fatigue, presyncope, and anemia requiring blood transfusion. The patient reported this condition had been present for at least six months, over which time her systemic symptoms of fatigue and presyncope had become increasingly severe. At the time of presentation, she had not previously sought treatment. The patient had a past medical history of chronic lymphadenopathy, hypertension, and thyroid disease status post thyroidectomy and an additional past surgical history of tubal ligation. The patient reported that she was previously informed of enlarged abdominal lymph nodes found incidentally on pelvic imaging. To her recollection, there was no intervention or follow-up of that finding. Medications include levothyroxine and losartan-hydrochlorothiazide tablets.\nPelvic examination revealed an enlarged uterus consistent with a 20-weeks size. Fibroids and irregular contours were also noted. There were no adnexal masses palpated. No axillary, subclavian or inguinal lymph nodes were palpable on examination. Transvaginal ultrasound relieved numerous masses consistent with fibroids; the endometrial stripe was not well visualized for measurement. Measurements of four fibroids were recorded with the largest being 7.26 cm by 5.80 cm. An endometrial biopsy was attempted; however, pathology showed no endometrial tissue was obtained. The patient was agreeable to definitive surgical management and provided consent for abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO).\nPreoperative abdominal and pelvic imaging revealed new retroperitoneal lymphadenopathy in addition to the existing abdominal lymphadenopathy. These lymph nodes were not palpable on examination. The patient underwent a CT-guided core needle biopsy of a solitary retroperitoneal lymph node (Figure ). Initial histopathology was consistent with a benign lymph node containing chronic inflammatory cells with micro-calcifications. A background amorphous eosinophilic material suggested amyloid deposition. Specimens were sent to an outside lab for specialized testing. There was no evidence of poorly differentiated cells or ectopic cell lineages.\nThe patient was taken to the operating room for TAH and BSO by a general obstetrician-gynecologist (OB-GYN). Given the benign appearance of the lymph node specimen, the patient did not provide consent for lymph node dissection. The pelvic cavity was accessed with a midline incision from the pubic symphysis to the umbilicus. Initial inspection of the pelvis showed an enlarged, hyper-vascular uterus with numerous hard submucosal masses (Figure ). The ovaries were atrophic as expected, and fallopian tubes were surgically absent. A sizable cervical fibroid was encountered. During the vaginal incision, this mass was found to be protruding into the vaginal canal, without mucosal invasion at the time of surgery. The vaginal portion of the mass was sharply dissected from the uterine portion of the mass, and the vaginal mass was clamped and displaced from the vaginal lumen. Following removal of the specimen, no lymph nodes were easily palpable. Following surgery, the patient was taken to the recovery room in stable condition following transfusion of one unit of red blood cells.\nHistologic examination of the cervix showed a benign tumor (Figure ). Plasma cells were infiltrating the leiomyoma, indicating an inflammatory reaction. These findings were like the lymph node findings, indicating a relationship between the two disease processes.\nTwo weeks postoperatively, the results of the amyloid confirmatory tests returned. The lymph node specimens were evaluated with Congo red Staining to confirm the diagnosis of amyloid deposits. However, the results were inconclusive due to the limited tissue quantity. Light chain testing revealed a polyclonal B cell population with no atypia or abnormal mitoses.\nUpon follow-up several days later, the patient returned to the clinic for removal of skin staples and review of pathological findings. One month post-operation the patient’s symptoms had resolved. She was hemodynamically stable and had not required additional blood transfusions. |
The 3-day-old term born hypotrophic neonate was admitted to the Department of Pediatric Cardiac Surgery with the initial diagnosis of tetralogy of Fallot.\nThe girl was born by normal spontaneous vaginal delivery at the 39th week of gestation with a birth weight of 2370 g. Her prenatal medical history was complicated by maternal heavy nicotinism in pregnancy. Congenital heart disease was diagnosed at the 3rd day of life, and prostaglandin E1 (PGE1) intravenous infusion was administered.\nThe child was transferred to our department in an emergency setting because of rapid general deterioration with severe cyanosis. Because of hypoxemia and hypercapnia the baby was intubated on admission, and remained on mechanical ventilation during the diagnostics. Also continuous infusion of PGE1 was carried on.\nTransthoracic echocardiogram (TTE) performed on admission revealed DORV with a large subaortic ventricular septal defect. There was 70–80% dextroposition of the wide aorta with mild aortic valve insufficiency, and a severely hypertrophied right ventricle with right ventricle outlet tract obstruction (RVOTO). Pulmonary valve stenosis (pulmonary valve annulus diameter of 3 mm) was present with evidence of a right ventricle – main pulmonary artery pressure gradient of 65 mm Hg. The main pulmonary artery (MPA) was severely hypoplastic through its entire length. The MPA inner diameter was 3.5 mm. Continuous 2 mm diameter left and right pulmonary arteries (LPA and RPA) were seen. There was no echocardiographic pattern of PDA flow; thus the infusion of PGE1 was stopped.\nThe newborn was referred for an emergency percutaneous balloon angioplasty of the right ventricle outflow tract obstruction (RVOTO). The procedure was performed under general anesthesia. Hypoplasia of the MPA and pulmonary bed with a small diameter of the right and left pulmonary arteries was noted. The initial McGoon index was 0.8.\nNevertheless an increase of peripheral saturation was observed after percutaneous angioplasty of the RVOT. The child was extubated and subsequently discharged from the reference department of pediatric cardiology.\nAfter 3 weeks the patient was admitted again because of general deterioration, decrease of saturation (65–70%) and fatigue during oral feeding, which required nasogastric tube feeding. The infant was referred for repeated percutaneous balloon RVOT angioplasty. During the procedure the symptoms of early restenosis were observed; thus 2 vascular stents were directly implanted following telescope stent-in-stent technique in the area of the RVOT and MPA (Abbott Vascular MULTI-LINK, 4 × 8 mm and 3.5 × 15 mm, ). After the procedure the patient’s general condition improved. The increase of blood oxygen saturation and concomitant decrease of RV-MPA pressure gradient in control echocardiography were noted. The anticoagulation protocol with initial heparin infusion (15 mg/kg bw/h) followed by chronic oral aspirin administration (3 mg/kg bw) was continued.\nIn the 10th week of life percutaneous balloon RVOT stent redilatation was attempted. The findings of right ventricle hypertrophy, persistent blood flow from RV to MPA, and an evident increase of both pulmonary arteries’ (LPA and RPA) diameters were obtained (). The efforts to re-dilate RVOT stents were unsuccessful; therefore the 4.32 kg bw infant with observed 70–75% peripheral saturation was referred for a right modified BT shunt, which was made with implantation of a 4.5 mm Gore-Tex tube.\nRegular follow-ups were performed until the age of 7 months. Antegrade flow to pulmonary artery blood flow velocity up to 4 mm/s with mild recurrent diastolic wave from MPA to RV was confirmed. Her peripheral saturations were oscillating over 80% with short-term drops at the time of oral feeding, and viral infections. The child was chronically treated for recurrent urinary tract infections with prolonged antibiotic therapy; thus her weight gain was still less than expected.\nPercutaneous balloon RVOT stent redilatation was performed at the age of 7 months. Control angiography performed to prior scheduled surgery proved correct localization of implanted stents with normally developed pulmonary arteries, with a current McGoon index value of 2.7. Finally at the age of 10 months and 5.7 kg bw, the girl was referred for surgical DORV correction with removal of implanted stents.\nThe procedure was performed via median sternotomy. The agenesia of the thymus gland and the hypoplastic MPA with the presence of palpable stents within the pulmonary trunk were exposed. There was clear evidence of macroscopically visible specific pericardial inflammatory reaction around the area of the stented MPA.\nClassic cardiopulmonary bypass was commenced routinely. At 28°C central temperature the heart was stopped, and after opening the right atrium a large “malalignment” ventricular septal defect (VSD) was identified. The aortic valve was completely overriding the 15 mm-wide VSD, while there was both subpulmonary and subaortic muscular conus in the right ventricle. The outflow from the severely hypertrophied RV to the MPA was secured with the stents protruding the right ventricle cavity. The free wall of the right ventricle was opened. The stents were dissected from fibrous inflammatory-like tissue and removed, and the obstruction of the RVOT was extensively resected (). The “malalignment” VSD was closed using a large Gore-Tex (ML Gore and Associates, EU) patch with running monofilament suture. The RVOT and MPA were supplemented with a Contegra “mono-cusp” xenograft (Contegra, Medtronic, EU). The patient was weaned from CPB with sinus rhythm. In the early post-operative course, periodic arrhythmias of unexplained etiology were observed. Further recovery was uneventful.\nIn consecutively performed follow-up TTE, wide right ventricle outflow tract, unrestricted blood flow from RV to MPA and an extensive patch covering the VSD with the unobstructed left ventricle outflow tract were regularly observed. The present follow-up time is 14 months. The girl undergoes regular outpatient follow-ups in the local pediatric cardiology center. |
A 65-year-old Japanese man, with no symptoms and receiving only oral medication for hypertension, underwent an esophagogastroduodenoscopy for a medical checkup in November 2009. A polypoid lesion, about 10mm in diameter with a deep depression on top, was found on the opposite side of the Vater papilla in the second portion of his duodenum (Figure A). The lesion was covered by normal mucosa except in the depressed portion, which showed tense surface mucosa. The lesion was similar in appearance to a SMT with a central depression. Multiple biopsies were taken from the lesion, but all of the specimens showed normal duodenal mucosa. The lesion was strongly suspected to be a malignant tumor and six more biopsies were taken from the lesion during a second esophagogastroduodenoscopy three months later. Only one of the six biopsy specimens revealed adenocarcinoma. On endoscopy, the tumor was suspected of invading the submucosa or deeper because of its endoscopic appearance; therefore, SR was indicated and he was referred for surgery at our university hospital in May 2010.\nConventional endoscopy prior to surgery showed a polypoid lesion resembling a SMT with a central depression (Figure B). Chromoendoscopy after spraying indigo carmine solution revealed an irregular depression at the top of the tumor with its surrounding area covered by normal duodenal mucosa (Figure C). Narrow-band imaging (NBI) magnified endoscopy showed an obscure mucosal pattern with irregular microvessels [], only in the depressed area (Figure D). A biopsy specimen from the depressed area revealed adenocarcinoma. High frequency endoscopic ultrasonography (EUS) using a 20mHz miniprobe displayed the duodenal wall as a five-layered structure. The tumor was demonstrated as a low echoic mass mainly involving the second or third layers but not the fourth or deeper layers (Figure ). Abdominal computed tomography showed no abdominal lymph node swelling. We predicted that it was possible to remove the tumor by ER and the tumor was removed using a conventional endoscopic mucosal resection technique with submucosal injection of glycerin solution and the snare method, instead of by pancreaticoduodenectomy as planned. The endoscopic mucosal resection was performed with no complications and our patient had a straightforward post-ER course. Our patient was discharged from the hospital a week after the ER.\nHistopathology of the resected lesion showed a tumor with a central depression and bilateral elevations (Figure A). The bilateral elevations were covered by nontumorous glands and pushed up by the tumorous glands showing inverted growth downward (Figure B). The tumorous glands invaded expansively to the muscularis mucosae with no submucosal invasion (Figure C). Tumor cells showed considerable cytological atypia having abnormal mitosis, heteromorphous nuclei, and eosinophilic cytoplasm, and formed irregular papillary structures (Figure D). Histology results from the ER confirmed the tumor was a well-differentiated adenocarcinoma confined to the muscularis mucosae with no lymphovascular invasion and negative margins (Tis N0 M0, Stage 0); complete resection was achieved. Our patient is still alive with no recurrence three years since the tumor was resected by endoscopy. |
We experienced a case of a 26-year-old man with a 40- × 50-mm sacrococcygeal lump that had been present since birth. He had no symptoms and had not received any treatment. One week before the current presentation, he sought treatment for lower abdominal distension and discomfort at our hospital. Pelvic magnetic resonance imaging showed a solid-cystic mass in the pelvic cavity. Anterior sacrococcygeal cysts that measured approximately 96 × 114 × 89 mm were present and reached as high as the first sacrum. Posterior sacrococcygeal cysts were also present and they were irregular and measured approximately 34 × 72 × 60 mm. The preliminary diagnosis was SCT (–).\nThe patient was admitted to our hospital and underwent surgery. The surgery started from the sacrococcygeal region, with a curved cut of 10 cm in length and 7 cm above the anus. The skin and subcutaneous tissue were cut open to show the cystic wall of the retrosacral mass. The mass was completely dissected along the cystic wall, and a large amount of light yellow, porridge-like secretion was present in the sacrococcygeal mass. To separate the larger cyst, which was in front of the sacral tail, the fifth sacral vertebra was removed to further expose the surgical field and complete resection of the cyst. Decompression of the cyst showed a large amount of mucinous jelly-like liquid and the anterior sacral tumor was completely stripped. Because the cyst was closely adhered to the posterior rectal wall, a 1-cm2 portion of the posterior rectal wall was removed. Sutures were applied to repair the rectal wall, and sigmoid colostomy was performed.\nA postsurgical pathological examination showed a large amount of sticky liquid within the cyst and signet ring cells floating within the liquid. A diagnosis of teratoma combined with a mucinous tumor was considered. The immunohistological results were as follows: AE1/AE3 +, epithelial membrane antigen +, CDX-2 +, villin +, cytokeratin 7 −, cytokeratin 20 +, synaptophysin −, CD56 −, chromogranin A −, S-100 −, and Ki-67 50%+. These findings were consistent with a malignant teratoma. The malignant cells belonged to SRCC (–). Tumor marker levels were also measured. The CA724 level was 124.784 U/mL and the carcinoembryonic antigen level was 21.53 ng/mL. All of these results suggested high-grade cancer requiring chemotherapy. The patient then underwent chemotherapy.\nWritten consent was obtained by the patient and his parents for publication of this report and any accompanying images. This case was based on actual routine clinical treatment, and did not involve any ethical human studies. Therefore, we did not need to apply for review by the Ethics Committee or Institutional Review Board. |
A 32-year-old male, previously fit and healthy, presented with epigastric pain and was found to have an abdominal mass on abdominal ultrasound. He was referred to the First Affiliated Hospital, School of Medicine, Zhejiang University for further investigation and treatment in October 2010. There were no signs of neurofibromatosis type 1(NF-1). Physical examination revealed a firm, ill-defined, fixed mass in the upper abdomen. Laboratory findings, including leukocyte and platelet counts, hemoglobin, serum creatinine, liver function, alpha-fetoprotein, carcinoembryonic antigen and cancer antigen 19–9, were all within normal limits.\nContrast-enhanced computed tomography (CT) of the chest and abdomen showed a heterogeneous tissue retroperitoneal mass, approximately 16 cm in diameter (Figure ). Following contrast, heterogeneous enhancement of the mass was noted. Adjacent vessels, such as the common hepatic artery, the portal vein and the inferior vena cava, were compressed. There was no evidence of associated lymphadenopathy or distant metastases. Biopsy of the tumor suggested a soft tissue sarcoma composed of pleomorphic spindle cells.\nAll imaging studies and serology examinations indicated that surgery was feasible. At surgery, a huge, soft, whitish, solid and cystic tumor was found, which occupied the entire abdomen. The tumor appeared to involve the distal stomach, the diaphragm, the hepatoduodenal ligament, the gastrohepatic ligament, the left lobe of the liver and the celiac trunk. It was also compressing the walls of the abdominal aorta and the inferior vena cava. Massive varicose veins were noted in the abdominal cavity. Part of the left lobe of the liver was resected due to tumor infiltration. A distal gastrectomy with a Billroth II anastomosis was simultaneously performed due to tumor involvement of the stomach. In addition, we suspected that the common bile duct had also been invaded by tumor, which was not evident on preoperative imaging, and we therefore performed a resection of the common bile duct, a cholecystectomy and T-tube drainage. Intraoperative histological examination of a frozen section suggested the presence of a soft tissue sarcoma.\nOn gross examination, the tumor measured 16 × 15.5 × 8.2 cm. The cut surface appeared firm and yellowish in the peripheral portion with foci of hemorrhage and excessive necrosis in the center. The margins appeared macroscopically clear.\nOn further histopathological examination, the neoplastic tissue displayed interlacing fascicles of spindle cells with wavy, elongated hyperchromatic nuclei. There was pronounced pleomorphism, an increased mitotic index (>50 mitoses per 10 high-power fields) and hypercellularity. Rhabdomyosarcomatous differentiation was evidenced by foci of scattered, round cells with a prominent eosinophilic cytoplasm and atypical nuclei, which were identified as rhabdomyoblasts (Figure ). Microscopically, the margins were confirmed to be clear of residual tumor.\nImmunohistochemistry demonstrated positive staining of the rhabdomyoblastic cells for desmin (Figure ) and vimentin. Nerve sheath differentiation of the spindle cells was confirmed by S-100 protein (Figure ) positivity. Tumor tissue was negative for smooth muscle actin, HMB-45, CD34 and CD117. Based on these findings, the diagnosis of a MTT was confirmed.\nAccording to the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grading system [], the tumor was classified as grade 3 (a total score of 7: 3 for malignant triton tumor, 3 for >50 mitoses per 10 high-power fields and 1 for <50% tumor necrosis). The surgical margins were estimated as wide []. As no macroscopic or microscopic tumor remained, surgical resection was judged complete.\nFollowing surgery, the patient made a good recovery and was discharged two weeks later. However, during his first follow-up assessment one month after surgery, CT imaging revealed multiple lesions in the liver, which were considered a recurrence of the tumor. Clinical deterioration occurred rapidly, and no subsequent treatment was undertaken. The patient died two and a half months after the operation. |
History—This 26-year-old woman sustained a single GSW to the right-side of her head. EMT found her with a GCS score of 5. Her pupils were equal, round, and reactive at 3.5 mm bilaterally. She was intubated, brought to the hospital, and rushed to the CT scanner in less than 30 minutes.\nImaging highlights—CT showed a right frontoparietal fracture with several displaced bone fragments and a large parietal intraparenchymal contusion [Figure –]. The bullet passed through the falx, causing a subdural hematoma and crossed into the left parietal lobe before exiting the left side of the skull [Figure –]. There was no intraventricular blood and minimal midline shift.\nManagement—Given her poor GCS score at the scene and bilateral injury through eloquent cortex, it was felt that she would likely have a poor outcome and thus although the OR was ready on standby for emergent neurosurgical intervention, we initially opted for conservative management, with insertion of an ICP bolt, measuring an initial value of 37 mmHg. She was maintained on round the clock doses of mannitol and hypertonic saline and the ICPs normalized over the next 72 h, at which time she was observed to have some motor recovery in her arms. Because the patient demonstrated some meaningful neurologic improvement with normalized ICPs, and further study of the CT angiogram did not show any damage to the superior sagittal sinus, we attributed the persistent edema to injury caused by the bone fragments as opposed to venous congestion, and thus opted to take the patient to the OR in a delayed fashion on admission day 7 for decompression. A left hemicraniectomy was performed with removal of the necrotic parietal lobe and boney debris.\nClinical outcome—The patient had an extended stay in a rehabilitation facility where she regained full mental alertness and cognitive skills, however, she remains wheelchair-bound with persistent spasticity in all 4 limbs.\nFor ease of reference, we have summarized the above cases in table format []. |
A 52-year-old man complained of nasal stuffiness along with bleeding, headache, and vomiting. He was referred to our department after magnetic resonance imaging (MRI) showed an enormous mass occupying the nasal and paranasal cavities and extending into the bilateral frontal base [Figure and ]. No neurological deficit other than anosmia was identified. The tumor spread beyond the nasal cavity and paranasal sinuses, and was therefore classified as stage C based on the modified Kadish clinical staging system [].[] Total removal of the tumor was achieved surgically, using a transnasal approach in combination with a bilateral front basal craniotomy [Figure and ]. The dura along the anterior skull base was opened partially and the cranial base was reconstructed using the pericranial flap. Histological examination of the tumor demonstrated proliferating tumor cells with large, oval nuclei containing prominent nucleoli. Scattered necrotic changes, nuclear fission, and Homer–Wright rosettes were identified. These findings were consistent with Hyams grade III ONB [].[] No neurological deficit other than olfactory analgesia was observed after surgery, however, the patient suffered from meningitis caused by cerebrospinal fluid rhinorrhea that developed 1 week postoperatively. Because complete eradication of the rhinorrhea and meningitis required 9 weeks of treatment, radiation therapy was started 10 weeks after the surgery. Large-field irradiation of the resected area, from the nasal cavity to the frontal lobe, was administered with a total of 60 Gy in 30 fractions. The patient was discharged 17 weeks after the surgery and was followed-up on an outpatient basis. At the time of discharge, whole-body enhanced computed tomography (CT) revealed no apparent distal metastasis or tumor recurrence.\nAt 24 weeks postoperatively, the patient noted numbness in both upper limbs, which gradually worsened. He was readmitted 25 weeks after the surgery because of rapidly deteriorating symptoms. Neurological findings on readmission revealed tetraparesis, hypesthesia, and hypoalgesia below C6, hyperreflexia of both legs, and urinary incontinence. CT and MRI revealed an intradural lesion compressing the spinal cord along its right ventral aspect at C5 through T2 [Figure –]; MRI of the head showed no evidence of tumor recurrence. In view of the diffuse nature of the disease, it was decided to surgically relieve the compression by the tumor to avoid complete tetraplegia. A laminectomy from C4 to T3 was performed. Intraoperative findings confirmed the absence of tumor in the epidural space. However, under the arachnoid membrane, the tumor compressed the spinal cord along its right ventral aspect. Because the tumor adhered to the spinal surface and was entangled in several nerve roots, it was removed but not completely. A dural plasty was then performed with a Gore-Tex® membrane to avoid restriction of the subdural space. The tumor was histologically diagnosed as ONB, consistent with the first operation [Figure and ]. After surgery, his urinary incontinence promptly improved. Paralysis of the right upper and both lower limbs remained but seemed to be improving. Residual tumor was seen on MRI performed 1 week after the surgery but the spinal cord was well decompressed [Figure –]. Whole-spine irradiation of 58 Gy in 29-Gy fractions was started 1 week postoperatively. MRI after irradiation no longer revealed residual spinal tumor, but shortly after spinal irradiation, the patient became progressively lethargic. Brain MRI showed multiple leptomeningeal enhanced lesions. General malaise and anorexia followed and the patient developed bilateral pneumonia, which caused his death 16 weeks after the surgery for ISM and 41 weeks after the first operation. During the time before his death, there had been no further deterioration in the motor weakness of his four extremities. |
A 26 year old male was admitted with impaired hearing and tinnitus on the right side and swelling of the right temporal region which was gradually progressive for the last two years. His general physical examination was normal. Neurological examination revealed a severe right conductive hearing loss with a Grade II House-Brackman facial nerve paresis. A diffuse swelling was noted in the right temporal and preauricular region. CT scan of the brain showed a large well defined hyperdense contrast enhancing lesion originating from the right temporal bone – squamous and petrous portions with a large intracranial extension causing uncal herniation [Fig. &].\nThe patient was taken up for surgery with an intention of radical removal. Control of the right external carotid artery (ECA) was obtained in the neck [Fig. inset]. Right frontotemporal scalp flap was raised. The temporalis muscle was seen to be infiltrated by the tumor and was excised seperately. The tumor was firm, reddish brown and vascular. It had destroyed the squamous temporal bone, lateral petrous portion, zygomatic arch and was seen invading the cranium pushing the temporal bone superiorly and medially along with the dura [Fig. ]. Dura was not transgressed. Piecemeal total removal of the tumor was achieved with temporary clamping of the right ECA. The tumor was adherent to the dura but could be peeled off the dura [Fig. ]. Biopsy was taken from surrounding bone, muscle and dura from 4 different sites. A drain was left in the large dead space created by the removal of the tumor. Cranioplasty was planned for a later date.\nPostoperatively the patient developed a right total LMN VII nerve palsy (Grade VI House-Brackman). Hence a right tarsorrhaphy was done to prevent exposure keratitis one week after the first operation. At the same sitting the external auditory canal was also closed to prevent communication of the dead space in the cranium with the external auditory canal after confirming the absence of any collection in the intracranial dead space. The postoperative period was otherwise uneventful. Histopathological examination revealed a neoplasm composed of numerous osteoclast like giant cells amidst a background of mononuclear plump spindle cells suggestive of a GCT [Fig. ]. The histopathological examination of the other 4 areas of bone, dura and muscle did not reveal any tumor infiltration. Postoperative CT scan confirmed a total excision of the tumor [Fig. ]. Since a radical excision of the tumor had been achieved it was decided to defer radiotherapy. Three months after surgery patient was normal but for the deafness and facial palsy. Follow up CT at 6 months and 12 months did not reveal any recurrence.\nNeoplasia of the skull bones are uncommon accounting for only 2.4% – 2.6% of all primary bone tumors []. The majority of giant cell tumors occur in the long bones usually the distal femur, proximal tibia and fibula, distal radius and ulna []. The skull is a rare location for GCT. In the cranium the sphenoid bone is the commonest site followed by the temporal bone [,-]. This can be explained by the fact that the tumor genesis occurs in the endochondral bone instead of intramembranous bone [,-]. The temporal bone has two main components – squamous and petromastoid. The squamous portion develops by intramembranous ossification, while the petreomastoid portion develops from cartilage (endochondral bone). GCTs are commonly seen to arise from the petromastoid portion as was noted in the present case. GCT is commonly seen in the 30–50 years age group with only 16% of patients below 20 years of age [,]. A mild female preponderance is seen but this is more pronounced in the younger age group []. Typically, the tumor presents as an enlarging mass associated with local pain over a period of few weeks to years []. GCT of the sphenoid may present with headache, visual field defects, blindness, diplopia, second through eighth cranial nerve dysfunction, endocrinopathy and change of mental status []. Temporal bone tumors present with pain usually behind the affected ear, deafness and facial weakness as in the present case []. Temporal bone GCT may invade the infratemporal fossa, paranasal sinuses and nasopharynx []. Intracranial extension as in our case may also be present. Dural penetration with invasion into the brain has also been seen [,]. Plain radiography shows radiolucent lesion of the skull and cannot be generally differentiated from other radiolucent lesions. On CT it is seen as a lytic lesion expanding the bony cortex []. Generally these tumors are contrast enhancing due to their vascular nature as seen in our patient.\nThese tumors generally tend to expand and attenuate the bony cortex rather than erode it []. GCT may be vascular and an external carotid angiogram may be required to demonstrate the arterial supply. In our patient the tumor was vascular but no angiogram was performed. However, temporary clamping of the external carotid artery during excision reduced the intraoperative bleeding. Grossly, these tumors are grey to yellow-brown, soft or firm and friable. Small cystic areas and grey-white necrotic foci may be seen. Microscopically, GCT consists of plump spindle shaped or ovoid cells with admixed multinucleated, cytologically benign giant cells. Variable numbers of benign multinucleated cells are seen amidst sheets of benign mononuclear spindle shaped cells with similar nuclear features. The nuclei are generally hypochromatic with inconspicuous nucleoli and mitotic figures are uncommon []. Histological differentiation of GCT may be challenging. The differential diagnoses consist of central giant cell granuloma (CGCG), aneurysmal bone cyst, chondroblastoma, hyperparathyroidism and fibrous dysplasia []. CGCG is a reactive bone lesion that occurs mainly in the jaws []. CGCG and GCT are histologically very similar and the main significant difference is the greater number of nuclei in the giant cells of the GCT [,]. CGCGs are distinguished from true GCTs by their fibrogenic, relatively acellular stroma, extensive osseous metaplasia and the clustering of giant cells around areas of hemorrhage or necrosis []. A key point in the differential diagnosis is that in GCT the stromal cells and giant cells resemble each other particularly with regard to their nuclei, whereas in giant cell reparative granuloma, the osteoclasts and the stromal cells of the fibroblastic type are distinctly different []. Jaffe has subclassified GCT into three grades but such a grading has not been found to correlate with subsequent tumor behaviour or sarcomatous transformation [,].\nThe precise ontogeny of GCT is unresolved. GCT and CGCG are histologically and pathogenetically similar []. Cell cycle associated proteins like MDM2, Ki-67 and PCNA have been seen to be widely expressed in CGCG and GCT. The percentage of Ki-67 and PCNA positive cells are higher in CGCG[]. This means that CGCGs show a higher proliferative activity than GCTs. GCT cells are also seen to produce both MMP-9 and tumor necrosis factor-alpha (TNF-alpha) []. Studies suggest that TNF-alpha secreted by the multinucleated giant cells up-regulates MMP-9 expression in GCT stromal cells by the induction of certain transcription factors, which in turn enhanced the rate of transcription of MMP-9 gene. An essential cell-cell interaction in the regulation of MMP-9 expression exists in GCT []. Although it is the giant cell which is the most prominent feature of these lesions it is the mononuclear spindle cell which is the proliferating cell. Several pathways to induce osteoclast like giant cell formation from monocytes have been reported. The spindle cell recruits monocytes and induces them to differentiate into osteoclastic giant cells through release of cytokines [,]. Receptor activator of nuclear factor kappa B (RANK) ligand is also reported to play a crucial role in osteoclastic cell genesis [-]. It is possible that the soluble RANKL is released from the tumor derived cells and the soluble factor interacts with RANK expressed in monocytes resulting in osteoclast-like cell formation in cooperation with macrophage colony stimulating factor secreted from the cells []. SDF-1 has also been incriminated as one of the significant chemo-attractant factors involved in the recruitment of hematopoietic osteoclast precursor cells during tumor-induced osteoclastogenesis []. The histopathogenesis of GCT and CGCG thus appears to be identical but the biological and clinical behavior of GCT is more aggressive than the latter.\nThe treatment of choice is complete surgical excision which if achieved can be curative [,,]. However the skull base location of these tumors can make total surgical excision hazardous and not possible [,]. The role of adjuvant radiotherapy in eliminating residual tumor tissue is controversial. Some authors claim that GCT is not radiosensitive and radiation may provoke a sarcomatous transformation in the residual tumor tissue [,]. However, other authors recommend a single course of moderate dose super voltage radiation in achieving a high success rate and at the same time lowering the likelihood of malignant transformation [-,]. Radiotherapy remains the only option for unresectable tumors. In our patient radiotherapy was deferred for the present as radical surgical excision was achieved with no residual tumor.\nTreatment of GCT and CGCG with calcitonin and osteoprotegrin has received attention. Osteoclasts express calcitonin receptors and can be inhibited by calcitonin. In GCT and CGCG, tumor giant cells and their precursors also express calcitonin receptors. Clinical studies on treatment of CGCG with calcitonin have shown positive results probably due to control of osteoclastogenesis []. Osteoprotegrin ligand was also selectively overexpressed in GCTs and may indicate another possible target to which antitumor therapy could be directed []. Osteoprotegrin influences osteoclastogenesis and may be used for the treatment of GCT and CGCG. Osteoprotegrin is a "decoy" receptor for RANKL and therefore inhibits the RANK-RANKL interaction, which is a necessary step in the osteoclastogenesis. This is the rationale for a probable therapeutic use of Osteoprotegrin. Its utility is however unproven [].\nGCT can recur especially where only a curettage is employed [,]. Prosser et al recommend primary curettage for intraosseous giant-cell tumors without adjuvant treatment or filling agents, but tumors with soft tissue extension or with local recurrence require more aggressive treatment []. Metastases occur in only 2% of cases and are usually to the lungs but spread to rare areas like lymph nodes, mediastinum, skin, scalp and pelvis has been reported [,]. Our patient is on regular clinical follow up every 3 months and 6 monthly CT scan. He is recurrence free at the end of 12 months. |
A 21-year-old male presented to the emergency department (ED) with a 3-week history of progressively worsening right-sided ear pain refractory to outpatient oral antibiotics (amoxicillin-sulbactam 875 mg twice daily) prescribed by his primary care physician 4 days earlier. Clinical findings were unremarkable except for exquisite right-sided mastoid tenderness and decreased right-sided hearing. Computed tomography (CT) scan without contrast of the temporal bones revealed opacification of the right mastoid air cells with erosion into the mastoid and sigmoid sinus plate consistent with acute coalescent mastoiditis. Magnetic resonance imaging (MRI) venography revealed no occlusion of the sigmoid sinus. The patient was admitted and started on intravenous (IV) cefepime 1 mg every 6 hours for empiric Pseudomonas coverage. The patient's ear pain markedly improved within 24 hours of IV antibiotic administration, and surgical intervention was not planned. He was discharged with oral levofloxacin 750 mg daily for 2 weeks.\nThe patient missed his 1-week follow-up appointment but presented to a separate quaternary care ED 9 days postdischarge with recurrent right ear pain, mastoid tenderness, and worsened hearing loss. Repeat CT scan identified a lytic lesion of the right mastoid with multiple air-fluid levels of the surrounding mastoid air cells (), inconsistent with the previous diagnosis of coalescent mastoiditis. However, given the patient's lack of systemic symptoms, he was discharged from the ED with a refill of levofloxacin 750 mg and was instructed to urgently follow up with otolaryngology.\nTwo days later, the patient followed up in otolaryngology clinic. Given his persistent symptoms and the osteolytic lesion seen on the CT scan, the patient consented to mastoidectomy with biopsy. Debridement of the mastoid cavity revealed necrotic bone with extensive granulation tissue throughout the mastoid cortex, as well as a dehiscent but intact sigmoid sinus. Frozen section was consistent with LCH; thus, additional curettage of the remaining granulation tissue was performed. Final diagnosis of LCH was confirmed, with immunohistochemistry showing positivity for CD1a, S100, and CD68. A tympanostomy tube was placed. Intraoperative cultures were negative for growth.\nAfter an uncomplicated 2-day hospital stay, the patient was discharged and instructed to administer ofloxacin 0.3% otic drops (5 drops twice daily) for 1 month. The patient did well until 2 weeks after surgery when he returned to clinic with recurrent pain in the right mastoid. His hearing had returned to normal, but MRI of the brain without contrast and whole-body positron emission tomography (PET)/CT scan suggested residual disease over the sigmoid sinus. The patient was referred to medical and radiation oncology and received a 2-week course of adjunct external beam radiotherapy starting 9 weeks postsurgery at a total dose of 20 Gy, delivered at 2 Gy per fraction.\nThree months following radiotherapy and 6 months postoperatively, surveillance MRI and PET scans revealed no evidence of recurrent disease. The patient was instructed to repeat imaging in 6 to 12 months but was lost to follow-up. |
A 74-year-old female presented to the emergency department (ED) with upper abdominal pain and melanotic stools. She had an elective open juxtarenal abdominal aortic aneurysm repair a month before her index presentation. She was hemodynamically stable. Her pertinent initial labs showed a hemoglobin of 6.7 g/dl (baseline 9.6 g/dl) with a hematocrit of 23%. Patient did not have any fever or leukocytosis. A CT abdomen with contrast done in the ED for abdominal pain showed nonspecific findings, i.e, irregularity of the “aneurysmal sac” with a small amount of fluid around the sac (see ) which was read by the radiologist as early postsurgical changes. She was admitted and was started on proton pump inhibitors. An esophagogastroduodenoscopy (EGD) was performed that revealed mild duodenitis. Her hemoglobin remained stable the next couple of days, and she was discharged home with a 6-8-week course of proton pump inhibitors. Two months later, she presented again with similar complaints with a drop of hemoglobin. A repeat EGD was performed that did not reveal any obvious source of bleeding, and she was discharged home after stabilization.\nA month later, she came for the third time into the ED with abdominal pain, hematochezia, and profound hypotension. Her pertinent laboratory findings include leukocytosis, low hemoglobin and hematocrit, thrombocytopenia, and transaminitis. She was resuscitated with IV fluids and blood transfusions. She was started on broad spectrum antibiotics after blood cultures were drawn. A CT abdomen and pelvis was performed which showed tiny foci of air at the anterior aspect of the native aneurysm wrap just inferior to the location where duodenum crosses (see ). At that time, a decision was made to perform push enteroscopy instead of simple EGD to evaluate second and third portion of duodenum which showed an aortoduodenal fistula with infected graft adherent to the bowel wall and extruding purulent exudate (see ). She underwent emergent surgical excision of the infected graft and bypass grafting to restore vasculature. Her blood cultures and cultures from the graft revealed methicillin-resistant Staphylococcus aureus (MRSA) and Streptococcus agalactiae. Aggressive management was continued with proper antibiotics in the intensive care unit, but her condition deteriorated, and she expired within several days. |
In November 2018 at the University Hospital Cologne, a 33-year-old man from Mozambique presented with a massive, predominant, localized fibrous GE with signs of inflammation that had existed for around five years. The patient had insufficient oral hygiene (Figure ), but no other obvious relevant risk factors (ie, no known pre-existing conditions, no medical history, no prescribed medication intake, no family or orthodontic treatment history). An oral examination was performed, including periodontal status, preparation of X-ray images (Figure ) and creation of a jaw model to produce wound dressing and prosthetic planning (Figure ). In addition to necessary treatment of carious lesions, extraction of root remnants (16 and 25 in radicular cysts), and planned periodontal therapy with gingivectomy followed by wound dressing, the patient presented to the internist and hematologist to rule out underlying hematologic disease and HIV infection as a suspected cause of hyperplasia.\nBetween November 2018 and February 2019, periodontal therapy was initiated including extensive oral hygiene instructions and professional tooth cleaning every four weeks. Treatment of carious lesions and root extractions were carried out at the request of the patient at the family dentist. Once a clean oral hygiene situation had been achieved (February 2019), gingivectomy of the upper jaw was performed followed by gingivectomy of the lower jaw (March 2019), with no complications (Figure ). The excised gingiva was coarse, felt like heavily scarred tissue when cut, and was hard to remove. The excised gingiva was sent for histopathological examination. The histopathological examination of the excised gingiva lesion (hematoxylin and eosin stain; Figure ) revealed a stratified acanthotic squamous epithelium with slight parakeratosis and focal erosion. The underlying stromal connective tissue showed collagen-rich fibrosis and a superficial accentuated infiltrate of inflammatory cells, predominately plasma cells accompanied by a few lymphocytes. Overall, neutrophils were absent except for the small area of surface erosion, which contained some intraepithelial neutrophils. Eosinophils were not detected. Immunostaining for kappa and lambda light chains revealed a polyclonal plasma cell population. Immunoglobulin (Ig) G and IgG4 immunostaining supplemented the analysis. Immunoglobulin IgG is elevated in patients with sickle cell disease and may help discern a sickle cell crisis from stable disease. For exclusion of IgG4 associated autoimmune disease, IgG subtype has also been analyzed. The IgG4/IgG ratio value was a maximum of 0.279 in one HPF, considering 10 different HPFs. The GE was diagnosed as plasma cell gingivitis due to the histologic findings and the exclusion of other differential diagnoses.\nDuring anti-infective therapy, the patient visited the internist and hematologist in January 2019. The family dentist was also contacted regarding regions 16 and 26 for planning of root extraction in this area. Laboratory results (Table ) revealed a microcytic (low MCV), hypochromic (low MCH) erythrocytosis without anemia (normal hemoglobin (Hb) values), as well as deficiencies of vitamin D (calcifediol) and folic acid. The patient received folate and vitamin D supplementation. An HIV infection was excluded. Physical examination was unremarkable, and no clinical symptoms were reported. The red blood test results encouraged further blood analysis by the hematologist, including Hb electrophoreses and parameters of iron status. Iron deficiency was excluded, but Hb electrophoresis revealed that the patient was a heterozygous sickle cell hemoglobin (HbS) carrier, and a heterozygous alpha thalassemia minima were detected.\nThe patient did not present to the family dentist for further care of the carious lesions, as originally requested, but only for the extraction of the root remnants 16 and 26 (February 2019). Therefore, the restorative therapy was performed at the dental clinic (May 2019; Figure ). At the request of the patient, a visit to the orthodontics department of the University Hospital took place before the remaining prosthetic planning for the treatment of regions 16 and 26. Here, Invisalign® therapy for optimizing the upper front jaw combined with close recall intervals for maintaining optimal oral hygiene and care was planned and initiated.\nThe long-term prognosis of this case remains unclear. At six months after gingivectomy, the treatment outcome had been maintained with no signs of recurrence. We assume that inadequate biofilm control by the patient—a main etiologic factor for GE—will contribute to an increased risk of recurrence. To rule out this risk and improve the prognosis, the patient attends a maintenance therapy session every three months, including dental prophylaxis. He is also seeking orthodontic treatment because of his increased esthetic awareness. This therapy will reduce the local factor of tooth crowding and improve the possibility of proper biofilm control. In addition, as folic acid and vitamin D deficiency may be one of the underlying factors for the development of GE, supplementation may help to stabilize long-term treatment success. However, time will reveal the long-term outcomes and will provide information regarding the impact of the underlying hematological conditions on his gingiva. |
An 81-year-old male body was received through the Saint Louis University Gift of Body Program of the Center for Anatomical Science and Education (CASE) with signed informed consent from the donor. The CASE gift body program abides by all rules set forth by the Uniform Anatomical Gift Act (UAGA).\nMultiple arterial variations in the posterior thoracic region were noted during routine dissection (). The TA gave rise to 5 asymmetrical CTs of PIAs. A descending branch of the vertebral artery, as well as variation in the neurovascular organization within several ICS, was also noted.\nOn the right side of the thoracic wall, 2 CTs were observed arising from the TA, along with 2 typical PIAs. The right superior CT arose from the TA between the 7th and 8th thoracic vertebrae (Figures and ). Near this location, the CT gave off a branch to supply the 7th ICS before continuing superiorly and laterally along the vertebral column. Along its path, the CT gave off 3 more PIAs which supplied the 6th, 5th, and 4th ICS before the CT terminated between the 4th and 5th thoracic vertebrae.\nThe right inferior CT originated from the TA at the level between the 10th and 11th thoracic vertebrae (Figures and ). This CT traveled a short distance laterally before it split into 9th, 10th, and 11th PIAs supplying their respective ICS. These 3 PIAs also appeared to take on a more tortuous appearance than is typically seen. The 8th and 9th PIAs originated from the TA in the typical fashion and traveled to the respective ICS (Figures and ). However, the 9th PIA was greatly reduced in size compared to the rest of the PIAs in the area, likely resulting from the dual supply of the 9th ICS.\nOn the left side, 3 CTs were observed arising from the TA, along with 3 typical PIAs. The left superior CT originated from the TA between the 6th and 7th thoracic vertebrae where it then coursed superiorly to supply the 5th, 4th, and 3rd ICS (Figures and ). The middle CT originated from the TA between the 7th and 8th thoracic vertebrae before supplying the 7th and 6th ICS superiorly (Figures and ). The left inferior CT arose from the TA between the 12th thoracic vertebra and the 1st lumbar vertebra before splitting into the 11th PIA and the subcostal artery (Figures and ).\nThe 8th, 9th, and 10th ICS were supplied by typical PIAs arising directly from the TA (Figures and ). Although these PIAs originated and traveled to the corresponding ICS, the 9th and 10th took on a more tortuous course than is typically seen. The 1st and 2nd ICS were supplied by the highest intercostal artery, as is normally described above (Figures and ).\nAdditional variations were noted on the right side of the thoracic cavity. A descending branch of the vertebral artery (DBVA) was found traveling inferiorly and dorsally near the first three ribs, through the costotransverse foramina (Figures , , and ). This DBVA gave off branches to supply the 1st, 2nd, and 3rd ICS on the right side. The highest intercostal artery was still present on the right side; however, it only supplied the 1st ICS, providing dual supply to this space (Figures and ). The left side of 1st and 2nd ICS was supplied by the left highest intercostal artery as normally seen (Figures and ).\nThere was an irregular relationship in the neurovascular bundle of the 6th ICS on the right side (). The order observed was intercostal nerve, vein, and artery from superior to inferior. Additional variations were seen in the neurovascular bundle relationships of the left side in the 2nd (nerve, artery, vein), 3rd (artery, vein, nerve), and 6th (nerve, vein, artery) ICS (Figures and ). |
A 57-year-old man presented for evaluation of an incisional hernia and panniculus in April 2016 (). He had a history of laparoscopic gastric bypass 6 years prior and a 54 kg weight loss. His height and weight at presentation were 175 cm and 90 kg, respectively. He was stable at this weight for 3 years and was a non-smoker. In addition to the gastric bypass, surgical history was significant for lap-assisted ileocoloectomy and primary repair of a recurrent umbilical hernia in 2015 for perforated bowel which occurred during colonoscopic polypectomy, primary repair of an umbilical hernia in 2007, and a laparoscopic cholecystectomy in 1997.\nOn physical exam, he had a well healed midline incision with reducible ventral hernia and umbilical hernia with a hanging panniculus. Abdominal computerized tomography scan showed a midline “Swiss cheese” fascial defect and a left sided periumbilical hernia. The surgical plan included open ventral hernia repair with general surgery in combination with abdominoplasty by plastic surgery. After thorough discussion of the risks, benefits, and alternatives to surgery, the patient was consented for the procedure.\nIntraoperatively, the hernia extended the length of the previous widened midline incision with the sac densely adhered to the umbilical stalk. The initial surgical plan was to sacrifice the umbilicus, but the decision was made intraoperatively to use the original tissue as a skin graft. Therefore, the umbilicus was excised and placed on the back table (). The hernia sac was excised and the edges of the anterior rectus sheath were separated from the posterior rectus sheath. The fascia was then closed primarily without any undo tension. A fleur-de-lis abdominoplasty was performed with removal of the panniculus and excision of the previous midline scar. In order to create a neoumbilicus, a flap of skin and subcutaneous tissue from each abdominal flap was created and de-epithelialized to provide a vascular bed for the graft to adhere (). This was made using a 3:1 flap design and the flaps were approximately 2×6 cm each. These flaps were sutured together and then secured to the anterior abdominal fascia using 3-0 polyglactin suture. The excised umbilicus was thinned to the dermis on the back table and reapplied to the de-epithelialized flaps as a full thickness graft. A 3-0 polyglactin suture was used to attach the undersurface of the umbilicus to the deep crevasse of the flaps and anterior abdominal wall (). The graft was sutured around its perimeter with 5-0 polypropylene followed by application of a Xeroform bolster.\nThe patient tolerated the procedure well. At his initial post-op visit, he demonstrated 100% take of his free umbilical graft. At 9 months postoperatively, his abdomen was free of recurrent hernia and his umbilicus was well healed with an acceptable cosmetic outcome as judged by shape, size, and position of the umbilicus (). |
A 79-year-old Caucasian male, with a past medical history of atrial fibrillation on warfarin and metoprolol, and coronary artery disease on atorvastatin with previous coronary artery bypass grafting and placement of a dual-function pacemaker/ implantable cardioverter defibrillator (ICD), was on a motor boat in a remote location. The patient’s boat went over a wake of a larger boat passing by. He bounced off his seat in a vertical direction and subsequently landed on his tailbone. After the high impact fall, he complained of both immediate lower back and diffuse abdominal pain but did not seek out urgent medical help.\nTwo days after the initial incident, he started to become pale and diaphoretic; additionally, his ICD delivered three shocks over a 30-min period. He presented via ambulance service to a local community hospital in hemorrhagic shock with a blood pressure of 63/22 and heart rate of 118 beats/min. A primary survey was pertinently positive for hemodynamic instability and diffuse abdominal and lower thoracic spine tenderness.\nHe was resuscitated with 1 L of normal saline leading to an improvement of his pressure to 106/88. Initial laboratory investigations included a hemoglobin of 95 g/L, lactate of 6.1 mmol/L, creatinine of 129, and a supratherapeutic INR of 8.8. An initial non-contrast CT abdomen and pelvis showed moderate hemoperitoneum with sentinel clot in the left upper quadrant and pericolic gutter, as well as the area adjacent to the posterior wall of the stomach. An additional finding of a severely comminuted, minimally displaced burst fracture of the T10 vertebral body was noted (). Further interventions included INR reversal with 3 mg of Vitamin K and 3000 units of prothrombin complex concentrate, and administration of 2 units of packed red blood cells and 2 L of normal saline. Based on clinical severity, the patient was transferred to the trauma service at a tertiary-care Level 1 trauma center.\nPrimary survey revealed a protected airway, spontaneous and bilateral air entry, and hemodynamic stability with a blood pressure of 100/60 and a heart rate of 88 beats/min. His abdomen continued to be mildly distended and tender without peritoneal signs, however the patient reported it had improved since his original presentation to the local hospital. Repeat laboratory investigations revealed a stable hemoglobin of 94 g/L, and correction of his INR to 1.2. Given his stable condition, he underwent a CT RIPIT (Rapid Imaging Protocol in Trauma) [] and CT angiogram (CTA) of the abdomen and pelvis. His imaging revealed pseudoaneurysms of the left gastric artery measuring up to 6 mm with another 9 mm rounded area of increased attenuation along the lesser curve of the stomach (, ). No extravasation was seen. Decision was made to monitor the patient closely with serial abdominal exams and repeat imaging in 72 h, or sooner if the patient exhibited any signs of deterioration.\nOver the next 72 h, the patient’s vital signs and abdominal exams, improved and his hematological profile remained stable. Given the patient’s CHADS score of 2, it was decided to hold therapeutic anticoagulation until the patient’s bleeding risk decreased, however deep venous thrombosis prophylaxis was initiated. A repeat CTA was performed to follow the evolution of the two pseudoaneurysms. The imaging study revealed unchanged pseudoaneurysms but noted the distal aspect of the left gastric artery was attenuated in keeping with a focal dissection and intramural thrombus. Secondary to the dissection, the patient was started on 81 mg of aspirin daily. Over the next few days the patient continued to improve clinically, and was discharged home. The patient lived outside of the local area, and arrangements for close follow up were made. |
A 55-year-old male patient with a history of neck pain and numbness of extremities for 6 years presented with hypoesthesia below the inguinal plane. The right upper extremity numbness was heavier than the left. The abovementioned symptoms were aggravated for 1 year resulting in a walking disorder. The patient required crutches when walking, and the maximum distance that he could walk was 50 m. Physical examination showed grade III strength of each muscle in both upper extremities, grade II grip strength, and grade IV strength of each muscle in both lower extremities. Bilateral Hoffman tests were positive, whereas the tendon reflex of both lower extremities was hyperactive. Magnetic resonance imaging (MRI) of the cervical vertebra showed that abnormal odontoid in the occipitocervical junction area and severe surrounding hydrops. The atlantoaxial instability resulted in mild AAD, as detected via computed tomography (CT), and the spinal cord was compressed by the posterior atlas arch (Fig. ).\nAfter failure of conservative treatment, we decided to perform surgical treatment on the patient to address the walking disorder. Preoperatively, we performed cervical CT angiography (CTA) to evaluate the relationship between the important blood vessels and bone in the cervical area. At the same time, 3D CT of the cervical region was performed to make a 3D print model of the patient's occipitocervical area (Fig. ). We simulated the depth and angle of cervical screw insertion according to the 3D print model, and finally determined the most suitable cervical screw for the patient. After adequate preoperative preparation, we performed posterior-only occipitocervical fusion combined with intraoperative 3D navigation system.\nAfter induction of general anesthesia, the patient was placed in a prone position. The vertebrae were exposed through an approximately 25-cm longitudinal midline skin incision from the occipital tuberosity to the spinous process of C6. The skin, subcutaneous tissue, ligaments, and deep fascia were exposed layer by layer. The occipital to the C5 spinous processes were fully exposed. The 3D navigation patient tracker was placed at the C5 level, and C-arm scan of the cervical vertebra with preoperative CT imaging after matching was performed. After matching to obtain high quality images, the navigation device was along the left side of the C2 articular process to determine the nail's insertion point, angle, and depth. Pedicle screws were placed at the C2 level on the left, and pedicle screws were placed at the right side of C2 vertebra in the same manner; then, the lateral mass screws were placed at the sides of C3 and C4. The pre-bent connecting rod was inserted into the screw tail groove and the top wire was locked. Three cranial screw casings were inserted into the left connecting rod. The position and angle of the casings were adjusted. Three cranial screws were inserted into the screw holes, and the right cranial base was manipulated in the same manner. The 3 cranial screw casings and screws were then inserted. The positional depth of the pedicle screw was determined to good by c-arm fluoroscopy. The posterior arch edge was removed by grinding and drilling on the right side of the posterior arch of the atlas, and part of the bone was removed by laminar forceps. With the hook nerve dissection, the dural sac was separated from the posterior arch of the atlas, and the posterior arch of the atlas was removed, thereby fully exposing the spinal cord pulses. The spinal cord surface is covered with thickened fibrous scar bands, which are carefully removed (Fig. ). The spinal cord was covered with a gelatin sponge. The trimmed autogenous cancellous bone and allogeneic artificial bone were implanted on the polished bone surface. After the implantation was confirmed, the neck muscles were restored, and a negative pressure drainage box was retained and fixed. The pedicle screw system was in the right position without displacement, which was confirmed on the intraoperative and postoperative X-ray films (Fig. ).\nOwing to the operation in the upper cervical vertebra and the medulla oblongata region, the patient's respiratory and circulatory systems were very likely to be damaged. After the operation we managed to keep the patient in the state of endotracheal intubation for 24 hours and then slowly come out of the anesthesia, and the patient's vital signs were closely monitored. The patient's vital signs were stable when he woke up, and he was conscious and had good limb movements. His grip strength increased from grade II before surgery to grade IV after surgery. Dexamethasone 5 mg/bid was administered twice a day for 5 days. Neurotrophic and anti-infective therapies were given at the same time. The wound healed at the first stage, and the stitches were removed at 14 days postoperatively. After removal of the stitches, 3D-CT was performed, which showed that the position of the pedicle screw was not significantly changed from that of the X-ray obtained 2 weeks prior (Fig. ). Postoperative MRI was not included in this paper due to the severity of the artifacts. At 3 weeks postoperatively, the patient's walking was ability improved and he was able to walk approximately 100 m by himself when he was allowed to get up and move around with the use of a neck brace. At 6 months postoperatively, the patient reported that the numbness of the limbs was reduced, and he was able to walk >500 m by himself. Physical examination showed grip strength of grade IV, and the rest of the limb muscle strength was grade IV. The tendon reflexes of both lower limbs were normally elicited without hyperactivity. In the past 6 months, there were no other discomforts and related complications, indicating that the operation was successful. |
The physician of a 70-year-old Japanese woman noted the carbohydrate antigen 19-9 (CA-19-9) level, which was elevated to 112 U/mL, and CT revealed a tumor in the pancreatic body. She was referred to our hospital for further examination and treatment. She had diabetes mellitus and no family history of cancer. She had no smoking history or alcohol consumption.\nOn examination, her abdomen was soft and flat without any apparent mass or tenderness. Blood tests demonstrated elevated tumor markers (CA-19-9, 112 U/mL; Span-1, 41 U/mL). Abdominal enhanced CT revealed an 18-mm hypovascular tumor in the pancreatic body and a dilated main pancreatic duct in the tail side of this tumor (Figure ). The tumor was located next to the splenic vein. There was a 7-mm cystic lesion without a nodule in the pancreatic head. Endoscopic ultrasound showed an 18.5-mm low echoic heterogeneous tumor in the pancreatic body and a 10-mm branch duct intraductal papillary mucinous neoplasm (IPMN) in the uncinate process of the pancreas. The main pancreatic duct was narrowed at the pancreatic body and dilated in the tail side in endoscopic retrograde pancreatography (Figure ). We diagnosed PDAC in the pancreatic duct with a branch duct type IPMN in the pancreatic uncus and performed distal pancreatectomy, splenectomy, and lymph node dissection.\nMacroscopic findings of the resected specimen showed a 32 mm × 20 mm tumor in the pancreatic body and a small lesion in the pancreatic tail, 15 mm away from the main tumor (Figure ). Pathological findings revealed a poorly differentiated tubular adenocarcinoma with invasion of the splenic vein at the main tumor (Figure ). Carcinoma in situ continued in the main pancreatic duct, in the range of 15 mm from the invasive cancer. A 1-mm poorly differentiated tubular adenocarcinoma was present in the pancreatic tail parenchyma, 20 mm away from the main invasive cancer (Figure ). There was no continuity between this small lesion and the main tumor or carcinoma in situ, and we diagnosed the small lesion as an intrapancreatic micrometastasis of PDAC. We administered PI4W as perioperative chemotherapy[] and discharged the patient without any complications. She was administered gemcitabine for 6 mo as adjuvant chemotherapy. However, she developed a recurrence in the liver at 16 mo after surgery, and underwent gemcitabine and TS-1 therapy. Nonetheless, she died 35 mo after surgery. |
A 67-year-old man with tarry stools was admitted for painless EGD. He had a history of surgery of cardiac carcinoma and pancreatic neuroendocrine tumor 3 years previously. Before EGD, he did not receive any therapies. After he was sedated intravenously with 1 mg midazolam and 100 mg propofol, the endoscopic examination proceeded smoothly. The gas filled in the whole examination was air, not carbon dioxide (CO2). During the procedure, an anastomotic stoma was found 45 cm from the incisor teeth. Congestion, edema, and erosion in the mucous membrane of the anastomotic stoma were observed. A diverticulum formed by the closed orifice of cardiac carcinoma surgery, with a size of about 1×1 cm, was seen under the anastomotic stoma (). Moreover, some brown blood clots were found inside the diverticulum (). A protuberance with a size of about 2×2 cm was also seen 40 cm from the incisor teeth, the surface of which was smooth (). Further EUS showed that the protuberance could be cystic (). No special abnormalities were seen in the rest of EGD.\nTo identify whether the bleeding originated from the diverticulum, we flushed out the brown blood clots. Then, active bleeding occurred inside the diverticulum (). As we were attempting to find the bleeding spot, a progressive decrease in pulse oxygen saturation occurred, with a minimum level of 82%. The circulation status was stable. Therefore, we had to stop the endoscopic procedure (12 min in total), and the patient was given manual ventilation with 100% oxygen. After 10 min, the pulse oxygen saturation gradually increased to >95%. However, 30 min later, the patient was still unconscious despite being administered with flumazenil. Physical examination revealed a positive right Babinski sign, right gazing in both eyes, and a Glasgow Coma Scale score of 6/15 with E2V1M3. An urgent computed tomography (CT) scan of the brain, chest, and abdomen was immediately performed, which showed evidence of multiple air emboli in the left and right frontal and parietal lobes of the brain (). Furthermore, there were diffused gas density shadows mainly at the edge of the liver ().\nThereafter, the patient was transferred to the intensive care unit (ICU) for further treatment. However, owing to the restrictions of his medical conditions, he was not able to receive hyperbaric oxygen therapy (HBOT). Instead, edaravone ganglioside and citicoline were administered intravenously and an ice cap was placed on his head to help the cerebral cells recover. Iced normal saline containing norepinephrine and Yunnan white medicine was administered nasally to stop upper gastrointestinal bleeding, and omeprazole and octreotide were also administered intravenously to inhibit digestive fluid secretion. Four days later in the ICU, the patient recovered consciousness but with paralysis of the left upper limb and weakness of the right upper limb. Rehabilitation exercise was then implemented to help regain muscle strength. During the hospitalization, further cerebral magnetic resonance scanning revealed sporadic multiple hyperintense signals in the frontal and parietal lobes in T2-weighted images (), whereas transthoracic echocardiography examination showed no evidence of patent foramen ovale (PFO). After nearly 1 month of therapy, the patient was discharged with grade 4 muscle strength of the left upper limb. |
A 72-year-old female with a medical history of AS, diabetes mellitus type 2, chronic kidney disease stage 3, gout, colon cancer treated with resection and colostomy bag placement 18 years prior, hyperlipidemia, hypertension, and morbid obesity presented with GI bleeding evidenced by black tarry stool in her colostomy bag for 7 days. Associated symptoms included fatigue, nausea, and decreased appetite. The patient reported no vomiting, abdominal distension, or abdominal pain. She had not recently taken any nonsteroidal antiinflammatory drugs. Echocardiography 1 month prior to presentation showed aortic valve area of 1.24 cm2, aortic valve mean gradient of 39 mmHg, and aortic orifice peak velocity of 4.11 m/s, indicative of moderate to severe AS.\nThe patient was initially seen at a regional hospital where her hemoglobin (Hgb) level was as low as 6.0 g/dL. During her stay at the regional hospital, she underwent push enteroscopy, colonoscopy, tagged red blood cell (RBC) scan, and angiography. The RBC scan showed delayed images for tracer accumulation in the right colon/terminal ileum. She was transfused 2 units of packed RBCs on day 4 of hospitalization and 3 units of packed RBCs on day 6. She was transferred to our tertiary care center on day 7 of hospitalization.\nOn arrival at our hospital, the patient's vital signs were within normal limits. She was alert and oriented. Her colostomy bag was located on the right lower quadrant of the abdomen and contained black tarry stool. Erythema was visible around the colostomy site. She had an existing colectomy midline wound with a linear bandage beginning below the xiphoid process and crossing the umbilicus. Other significant abdominal findings included abdominal distension and tenderness at the periumbilical and hypogastric region on deep palpation. A large hernia protruded from the pelvic region. Cardiovascular examination was significant for systolic ejection murmur, III/VI in intensity on the Levine scale, in the aortopulmonary area. Eye examination revealed conjunctival pallor.\nOn admission, the patient's laboratory results were significant for Hgb of 8.3 g/dL and a platelet count of 96 platelets/μL. Video capsule endoscopy showed blood in her proximal small bowel, and double-balloon enteroscopy (DBE) showed jejunal angiodysplasia that was treated with argon plasma coagulation. Repeat DBE on day 3 of hospitalization at our facility showed another small jejunal angiodysplasia that was also treated with argon plasma coagulation. Her Hgb remained stable during admission. She was discharged after 3 days of hospitalization with home health to resume wound care for her abdominal wound.\nThe patient continued to have dark stools after discharge. She was readmitted to the regional hospital 10 days after discharge. Repeat complete blood count showed Hgb of 6.2 g/dL. She was transferred back to our hospital the next day after being transfused another 2 units of packed RBCs. Repeat DBE showed a 1-mm focus of active bleeding in the proximal jejunum consistent with a Dieulafoy lesion ().\nThe lesion was treated with argon plasma at 1 L/min and 25 watts. Two homeostatic clips were placed to prevent further bleeding. India ink 0.3 mL was injected to tattoo the area. The patient's Hgb remained stable at 7.9 g/dL during the postoperative observation period. After 2 days, she was discharged home. At follow-up 1 year later, she reported no GI bleeding symptoms since discharge. |
An 80-year-old woman living independently with her daughter presented at the department of otolaryngology of our hospital because of throat pain and common cold symptoms for 2 months. Examination revealed lymphadenopathy in the cervical region, and she was diagnosed with malignant lymphoma (stage III). Treatment was started with 50 mg/day of prednisolone, but improvement was not observed. She was admitted to our hospital because of difficulties with eating. Chemotherapy was started the next day. On day 5 of hospitalisation, she suddenly showed conjugated deviation of the eyes to the right together with left hemiparesis. Her symptoms included left hemispatial inattention, forced grasping with the left hand and left hemiparesis. The Mini-Mental State Examination could not be completed because of her hearing impairment, but she was able to follow our instructions except to release the grasping hand. Her Barthel Index score was 5/100. MRI revealed a high signal intensity area in the right middle frontal gyrus on fluid-attenuated inversion recovery images ().\nPhysiotherapy and occupational therapy were started on the same day. Early in rehabilitation intervention, inattention to the left side, forced grasping with the left hand, muscle weakness (grade 3/5 in the right upper and lower limbs and 5/5 in the finger by the manual muscle testing) and moderate left hemiplegia (Brunnstrom stage 4 in the arm, fingers and leg) were identified. Particularly, her left paretic hand was eager to grasp anything that her left hand touched. Verbal instruction to release the grasp and to refrain from holding any object did not resolve the forced grasping (). In addition, during training of transfer between the bed and the wheel chair, her left hand held the guard rail of the bed and the armrest of the chair, preventing her from standing up. In the walking training, she assumed a forward bending posture, with the left hand tending to hold on to anything. She needed assistance to perform all items of ADLs. She showed increased muscle tone in her left arm during passive movements and a flexor synergistic pattern during active left arm movement. All these findings made training very difficult. Therefore, the goals of rehabilitation in the early period were to resolve the forced grasping and to reduce the amount of assistance in ADL training.\nOne day during medical examination, while exhibiting compulsive grasping, the patient unexpectedly imitated a gesture when prompted by the action of the neurologist (MN). In doing so, she released her hand from the nearby guard rail of the bed that she had grasped. Furthermore, when the examiner made a gesture of putting his right hand (left as seen from the patient) on his knee or his back, the patient imitated the gesture. After that, even when given verbal instruction not to imitate, the patient continued to perform the act of placing her left hand on the left knee or on her back. The act recognised repeatability. Therefore, observed IB was obstinate (). These observations suggest that IB may appear at the same time as GR because of the pathological lesion. We suspected that during the training at rehabilitation, IB could be used to control the GR that hindered the patient from performing ADLs independently. |
In January 2020, a healthy 29-year-old man presented to the emergency department with severe pain of the left lower extremity after falling approximately 20 feet from a ladder. The patient had no significant medical, family, social, or surgical history. He was on no medications and had no known drug allergies. Radiographs revealed anteromedial dislocation of the talus with minor fragmentation of the lateral malleolus and fracture of the posterolateral talar process (Figures and ). Closed reduction under conscious sedation was attempted without success by the emergency physician, and the foot and ankle team was consulted. On evaluation in the emergency department, the left foot and ankle appeared grossly swollen and deformed. No wounds, lacerations, or evidence of skin necrosis were seen. However, the skin was tented on the dorsal medial foot overlying the talar head. Pedal pulses were not palpable, but capillary refill was brisk in the digits and the foot was warm to touch. Sensation in the foot was intact to light touch. All five digits were plantarflexed at the interphalangeal joints, representing a checkrein deformity of the toes (Figure ). The patient was able to actively flex and extend the digits. Compartments were soft and compressible. No other musculoskeletal injuries were identified. The patient was subsequently taken to the operating room for closed, possible open, reduction under anesthesia.\nAfter general anesthesia and relaxation was confirmed, the patient was positioned on the table in a supine position with the end of the table at the level of the knee joint so that the left leg was vertical and at 90° of knee flexion. The foot was grasped with one hand placed dorsally over the midfoot, talar head, and neck and the other hand encircling the medial, posterior, and lateral sides of the calcaneus. With this bimanual grasp, a pushup type maneuver was done pushing the foot toward the floor (Figure ). With a modest amount of distraction placed on the foot, the subtalar and ankle joints reduced. Fluoroscopy confirmed reduction of those joints, but the navicular bone remained locked beneath the plantar lateral edge of the talar head. With this in mind, the forefoot was first slightly plantarflexed and then distracted and pulled dorsally and turned into supination. With this technique, the talonavicular joint was reduced. Then, while holding the hallux in dorsiflexion and foot in supination, fluoroscopy confirmed reduction of the talonavicular joint and the subtalar, and tibiotalar joints. After complete reduction, all toes were relaxed in a neutral position, the checkrein mechanism having been eliminated. Xeroform gauze was placed over the area of skin tenting, followed by a stockinette, bulky soft-tissue dressing, and posterior and stirrup plaster secured with an ACE wrap (Figure ). The patient was admitted postoperatively for 2 days for pain management, neurovascular checks, and physical therapy consultation. Postreduction CT imaging obtained during the hospitalization revealed maintenance of joint reduction with comminuted fracture to the posterolateral fibula and posterolateral process of the talus (Figures –).\nThe patient remained non–weight bearing in the splint for 2 weeks. At 2 weeks, the splint was removed, and non–weight bearing was continued in a tall pneumatic medical boot. The patient was allowed active range of motion exercises out of the boot at 3 weeks. At 6 weeks postreduction, he was allowed minimum weight bearing in the boot around the house only. The patient was walking without the boot at 10 weeks with some pain. A follow-up CT scan, 12 weeks after reduction, again revealed mild comminution to the posterolateral distal fibula with surrounding small bone fragments but no intra-articular loose bodies.\nHe was last evaluated 6 months after his injury. At that time, he related having mild pain and swelling with limitation in recreational activities. He was ambulating without assistive devices. No tenderness was provoked on palpation of the posterior talus or the distal fibula. Radiographic union of the posterolateral distal fibula fracture was seen along with no evidence of AVN of the talus (Figures –). At this last follow-up, he had approximately 20° of motion of both the ankle and subtalar joints. His AOFAS hindfoot score was 81. |
A-29-year-old right-handed Caucasian woman, who works as a model, presented to the hospital with facial weakness. She reported that a week previously she had a sore throat and was seen by her doctor who prescribed antibiotics. Four days later she developed paraesthesias in her hands and feet along with severe myalgia (day 1 of neurological manifestation). On day 3, she noted weakness in eye closure when applying eyeliner. On Day 4, she was at an audition, and was unable to smile for the camera. Later that night, she participated in a runway show. She was able to walk in high heels without difficulty. However, concerns about her face brought her to the emergency department after the show.\nAt presentation, neurological examination revealed facial diplegia. She was unable to close both eyes, purse her lips or smile. Deep tendon reflexes were 3(+) throughout with flexor plantar responses. She had no weakness or sensory loss in her limbs, and there were no respiratory or autonomic features on examination. Cerebrospinal fluid (CSF) showed two lymphocytes with a protein level of 162 mg/dL and normal glucose. Nerve conduction study done on Day 6 showed partial denervation of facial nerves with compound muscle actions potentials markedly decreased bilaterally. No response could be obtained on blink reflex studies bilaterally. There was no evidence of demyelination in the limbs; F waves were present with no delay in latency [Table , ]. Lyme serology was negative, serum and CSF angiotensin converting enzyme levels were 10 U/L (normal, 8–52 U/L). Tests for CSF VDRL and HIV were non-reactive. Antiganglioside antibodies were not sent and no imaging studies of the brain were carried out as her presentation was consistent with a demyelinating peripheral neuropathy. The physician on hospital service elected to treat her with IV immunoglobulin (IVIG 400 mg/kg/day) for five days. By the time above treatment was initiated (Day 7) her paraesthesias had already resolved. During her entire hospitalization, she developed no motor weakness and remained ambulatory. At the time of her discharge on Day 12 she showed some improvement in her facial weakness and was able to approximate her lips as well as furrow her eyebrows. Follow up nerve conduction studies were not carried out. When last seen 6 weeks after her first presentation, she was able to smile normally and no facial weakness was evident on examination. Her deep tendon reflexes were 1(+) bilaterally. |
A 47-year-old man was hospitalized for recurrent dizziness for > 6 months in January 2018. In addition to dizziness, he was prone to fatigue and dozing off. The symptoms of dizziness were similar each time. After rest, his dizziness would improve, without hemiplegia, vagueness of speech, double vision, fever, or headache. The patient had a normal diet, slept for more than 10 hours a day, and had normal urine and stool. He had a history of cerebral infarction 8 years previously and facial neuritis 9 years previously, without obvious residual symptoms. The patient denied a history of exposure to toxic radioactive substances and a history of alcohol or drug addiction. He had smoked 20 cigarettes a day for more than 20 years. A physical examination showed that his blood pressure was 132/99 mmHg. He had a normal mental status, clear speech, no obvious abnormalities in cranial nerve function, and a soft neck. His muscle strength and tension and tendon reflexes were normal, and pyramidal signs were negative. No obvious abnormality was detected in a cardiopulmonary examination. The results of routine stool, blood, and urine tests after admission were normal. The results of liver function and renal function tests, and levels of iron, C-reactive protein, thyrotropin, blood glucose, D-dimer, glycosylated hemoglobin, and blood lipids were also normal. Results of tests for autoimmune vasculitis antibodies, antinuclear antibodies, and blood gas analysis were normal. The homocysteine level was 31 µmol/L and the prothrombin activity was 77%. Thromboelastography, electrocardiography, chest radiography, color Doppler ultrasonography of the abdomen, and 24-hour ambulatory electrocardiography also showed no obvious abnormalities.\nColor Doppler ultrasonography of the carotid artery showed bilateral carotid atherosclerosis and the right vertebral artery was thin. Brain magnetic resonance imaging and brain magnetic resonance angiography showed a small left thalamic infarction (obsolete lesion). Transesophageal echocardiography and contrast-enhanced ultrasonography showed a moderate PFO with a severe right-to-left shunt ().\nAfter admission, the patient was treated with anti-platelet therapy and lipid-regulating, collateral circulation-improving, and blood pressure-lowering medications (as in previous hospitalizations). Although his dizziness was temporarily improved, it was expected to recur soon. Occlusion of the patient’s PFO was performed at the end of February 2018. The patient’s dizziness was relieved without recurrence during the 6-month postoperative follow-up period. |
A 17-year-old boy was referred to the pediatric lipid clinic for evaluation of low HDL cholesterol and low LDL cholesterol. His past medical history was significant for autoimmune lymphoproliferative syndrome (ALPS) type 1A that had been diagnosed in October 2007 at age of 10 years following one year history of bilateral cervical lymphadenopathy. CT scan of the abdomen had revealed generalized adenopathy with numerous prominent lymph nodes in the axilla bilaterally, supraclavicular, retroperitoneal, and inguinal areas and throughout the mesentery. In addition, splenomegaly (18.3 cm pole to pole) was also noted. Biopsy of the cervical lymph node revealed reactive hyperplasia and flow cytometry of peripheral blood revealed 23% double negative T cells and 11% alpha beta double negative T cells. Genetic testing for ALPS was performed and he was found to have a missense mutation, 621 (T>C) (C127R), in one allele of the FAS protein extracellular domain consistent with ALPS type 1A.\nA year after his diagnosis, he was started on mycophenolate mofetil and also received a two-month course of prednisone for progressive lymphadenopathy. Mycophenolate was discontinued and sirolimus was started three years after diagnosis due to continued lymphadenopathy. Lipid profile was checked for the first time nearly three years after diagnosis of ALPS and three months after starting sirolimus due to known effect of sirolimus on increasing triglycerides. HDL cholesterol was noted to be low at 31 mg/dL, with repeat levels of 46 and 60 mg/dL (low < 40 mg/dL, borderline low 40–60 mg/dL, ). LDL cholesterol and triglycerides were normal. Two years after the initial lipid profile, LDL cholesterol and HDL cholesterol became undetectable on multiple occasions over a period of six months. Triglyceride levels were only slightly elevated on two occasions (116 mg/dL and 148 mg/dL; normal < 90 mg/dL; borderline high 90–129 mg/dL and high ≥ 130 mg/dL) but were normal when HDL and LDL cholesterol levels were undetectable. Patient was not obese or diabetic at any of the times when the HDL cholesterol was undetectable with body mass index being between the 50th and 75th age and gender specific percentile. Patient was never on any medications such as peroxisome proliferator-activated receptor agonists, isotretinoin, protease inhibitors, nonselective beta blockers, and androgenic steroids which are known to reduce HDL cholesterol. Patient did not have any malabsorption, liver disease, or other chronic or acute inflammatory disorder.\nHDL cholesterol and LDL cholesterol levels improved with decrease in disease activity as measured by % alpha/beta TCR/DNT levels ( and ). While HDL cholesterol levels have improved but still continue to be low, LDL cholesterol levels have normalized. There was a significant negative correlation between disease activities as assessed by % alpha/beta TCR/DNT levels and total cholesterol (r = −0.9734, p = 0.038) and LDL cholesterol levels (r = −0.9962, p = 0.049). Patient continues to do well with decrease in lymphadenopathy on sirolimus. He continued to have splenomegaly despite the immunosuppression and underwent splenectomy at age 15 years of age in order to make contact sports safer without risk for splenic rupture. |
A 44-year-old female was admitted to our department because of the epigastric pain for the preceding two weeks. Her medical history was significant for arterial hypertension, Hashimoto thyroiditis and hypercholesterolemia. Her surgical history revealed a cesarean section. There were no desmoid tumours in her family.\nThe physical examination revealed a mass on palpation in the mid-abdomen that was easily movable. The physical examination was otherwise normal. Laboratory findings were unremarkable. The level of CEA was within normal limits. A transabdominal ultrasound (US) showed an ovoid well-delineated homogenously hypoechoic mass that was 10.1 × 6.0 × 7.2 cm in size. There was a hyperechoic area in the central part of the tumour with posterior acoustic shadowing that corresponded to intraluminal air. The tumour was circumferentially attached to the wall of the small bowel (). An abdominal computed tomography (CT) revealed a 8.2 × 7.2 × 7.4 cm mass infiltrating the small bowel. The tumour attenuation was of 33 Hounsfield units and it enhanced poorly and homogenously with an intravenous contrast (). The above preoperative imaging studies suggested a GIST involving the small bowel.\nThe patient underwent an elective laparotomy. Intraoperatively, there was an approximately 10 cm well-circumscribed mass in the mesentery that infiltrated the wall of the small bowel and narrowed its lumen (). On inspection, there were also 2–3 small tubercules attached to the serosa of the adjacent bowel that were included within the resection margins. Several similar lesions were found along the distal part of the small bowel and one of them was excised for the pathological evaluation. This gross appearance suggested a gastrointestinal stromal tumour with the peritoneal dissemination. The resection of a 25 cm segment of the small bowel was performed. The postoperative course was uneventful and the patient was discharged in a good health condition. A follow-up US revealed no desmoid recurrence a year after the operation.\nThe primary pathological diagnosis in this particular case was a CD117-negative gastrointestinal stromal tumour. The small serosal tubercules were found to be mesothelial cysts. The principal diagnosis was changed, however, after a consultation at a referral oncological centre. The microscopic examination of the resected specimen identified a fibromatosis in the mesentery. Histologically, the desmoid tumour was composed of spindle cells with elongated coma-shaped nuclei and the immunohistochemistry was negative for both CD117 and for CD34. Beta-catenin overexpression was present on immunohistochemistry (). No mitoses were found in 50 high power fields. |
A 7-year-old female child born of a nonconsanguineous marriage presented to us with headache for the last 20 days, which was insidious in onset, dull aching type, and frontal in location to begin with but progressively worsened in severity over the next few days. It used to be worse in the early morning and was associated with vomiting. The vomiting used to relieve headache. She gave no history of loss of consciousness, seizures, diminution of vision, diplopia, facial asymmetry, difficulty in hearing, change in voice, nasal regurgitation of fluids, or difficulty in swallowing. The patient's antenatal and perinatal period was uneventful, and she had achieved normal developmental milestones. She also had an elder sibling who was developmentally normal to age according to parents. The child was conscious, alert, and playful. She had an enlarged head. Papilledema was present on fundus examination. She had no other neurological deficits and there were no neurocutaneous markers. A contrast-enhanced magnetic resonance imaging of the brain [] revealed large lobulated lesion of size 6.4 cm × 6.3 cm × 8.3 cm which was hypointense on T1, hyperintense on T2, intensely enhancing within the body of the left lateral ventricle causing dilatation of lateral ventricle, causing significant mass effect. Based on radiology, a differential diagnosis of choroid plexus papilloma and intraventricular meningioma was considered. The patient was taken for surgery after informed consent and neuronavigation guided posterior temporal craniotomy flap was raised. Durotomy was done in C-shaped fashion and reflected anteroinferiorly. Navigation guided corticectomy was done in middle temporal gyrus to approach the tumour in left trigonal area. Tumor was completely intraventricular and extended from the left temporal horn to the atrium and body of the left lateral ventricle. It was highly vascular but variable in consistency, i.e. soft at certain areas and firm to hard in others. Gross total excision was done and a ventricular drain was placed. The duration of surgery was 9 h. Intraoperative blood loss was 3000 ml, but there were no episodes of bradycardia, hypotension, desaturation, or hypothermia. The patient was extubated and had no neurological deficits in the postoperative period. Postoperative noncontrast computed tomography of the head [] done revealed no residual tumor. The ventricular drain was removed on the second postoperative day. Histopathology report [] revealed a moderately cellular tumor composed of sheets and whorls of round to oval cells with moderate amount of eosinophilic cytoplasm with mild nuclear atypia suggestive of Grade 1 meningioma. The MIB index was 2%. |
A 66-year-old man who was a farmer of Caucasian origin presented to our specialist clinic after being referred by his general practitioner. He had a 6-month history of back pain in the thoracolumbar region, which was more pronounced when he stood up for a long time and for which he required regular analgesia. He did not state any bladder or bowel disturbance and had no other neurological disturbances. He stated that 6 months previously he had fallen approximately 10 feet from a combine harvester and immediately developed back pain. The patient was a non-smoker and a social drinker of alcohol, and his past medical history included myocardial infarction, deep vein thrombosis/pulmonary embolism, hay fever, asthma, emphysema, diabetes and under-active thyroid. He was taking thyroxine, paracetamol and morphine. A dual-energy X-ray absorptiometry scan demonstrated no evidence of osteoporosis or osteopenia. His clinical examination demonstrated tenderness over T12 but normal distal neurology with normal reflexes and no clonus. Radiographs showed a significant T12 wedge compression fracture (Figure \n). He was referred for magnetic resonance imaging (MRI) (Figure \n) on the basis that he might be a good candidate for kyphoplasty. The MRI scan showed edema within the body of T12 on the short tau inversion recovery sequence. Blood samples taken upon admission did not reveal any abnormality.Two months after the initial consultation, we performed a T12 kyphoplasty with no complications (Figure \n).Approximately 2 weeks after being discharged, the patient presented to the emergency department of our hospital with increasing back pain that improved at rest and with significant amounts of pain medication. He presented for a second time to the emergency department with excruciating pain on the left side of his thoracolumbar region, so he was admitted to our ward. X-rays did not show any further fractures or compromise, but MRI (Figure \n) showed extensive edema in the T11 and L1 vertebral bodies with fluid tracking from the T11-T12 disc into the vertebral body, which was a strong indication of possible pre-disposition to further osteoporosis involvement. All blood tests performed at this time, including full blood count (FBC), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), were normal. The patient was discharged 9 days later after receiving facet joint block injections (1ml of 40mg kenalog + 1ml of Marcaine® 0.25%).\nThe patient presented to the outpatient clinic of our hospital 9 days later with severe back pain. He stated that he had experienced no relief from the facet joint block injections. A MRI study showed edema on the level above and below the facet joint block injection site at the T11/12 Facet joints. He was put into a brace and blood samples were collected for FBC, urea and electrolytes, CRP and ESR. The only abnormal value was CRP (10mm/h), so the patient was put on 300mg of morphine, which did not lead to pain resolution. At the multi-disciplinary team meeting on the same day, the general consensus was that the patient should undergo a posterior fixation of two levels above and below the fracture site (T12), with a biopsy taken at the same time.Posterior instrumentation from T9 to L2 (pedicle fixation of T9-T10 as well as L1-L2, rods in between and a crosslink above T11-T12) was performed 1 month after the patient’s last admission (Figure \n), and he was discharged uneventfully 5 days after that. A biopsy was taken during the instrumentation procedure.\nTwenty days after the procedure the patient was re-reviewed and found to be pain-free while his pain medication had been reduced. The results of the biopsy showed a possible diagnosis of osteoporosis, but nothing else of note. The patient was reviewed 3 months after surgery, at which time his condition had improved significantly. |
The second patient was a 59-year-old male. He was a heavy smoker (10–15 cigarettes daily since 18 years of age) and had a history of previous alcohol abuse of >168 g pure alcohol per week during a period of 10 years. In addition, he had a hiatal hernia and Barrett's esophagus for which he was treated with a proton pump inhibitor (pantoprazole 40 mg daily). The tumor was found in an upper endoscopy preformed routinely. The initial symptom was dysphagia which he had suffered from intermittently during the last one and a half years. Blood tests and a physical examination showed no abnormalities. An area of a 10-cm, white, irregular mucosa with a 1-cm large polyp-like process was observed at gastroscopy (fig. ). Biopsies revealed acute and chronic inflammation as well as infection with nonhemolytic streptococcus, Escherichia coli, and a few fungal hyphae. The tissue was extended-spectrum β-lactamase positive. The patient underwent a series of gastroscopies without malignant findings, just as the initial CT scan was without suspicion of malignant disease.\nThree months later, a biopsy from the esophagus showed a squamous cell papilloma. The following PET-CT scan performed 6 months after the initial visit found tumor growth and a suspicion of malignancy, stage T2N0M0. An endoscopic ultrasonography-guided fine-needle aspiration found no malignant cells. During this period, the patient suffered from dysphagia but had no weight loss, nor was he hospitalized. Despite the lack of malignant findings, the patient was offered an esophagectomy and underwent surgery.\nNine months after the first visit, the pathology report of the resected specimen showed a hyperplastic mucosa with a low-grade VC, a mostly exophytic papillary tumor. The tumor was highly parakeratinized with no dissemination into the lymph nodes or other organs. After a minor pneumonia, for which he received antibiotic treatment, the patient was discharged after 2 weeks. The patient is presently without symptoms. |
A 52-year-old woman visited our hospital with a palpable left neck mass in April 2010. Her medical history showed that she had been diagnosed with left breast cancer (cT2N0M0) in December 2004, and she underwent modified radical mastectomy of the left breast in our hospital. An histopathological examination showed invasive ductal carcinoma with nuclear grade 2, and the absence of axillary lymph node metastases. Immunohistochemistry studies showed positive staining for ER and PR and negative staining for HER2 (score 0). She received five cycles of adjuvant CMF chemotherapy followed by tamoxifen (20 mg daily) for 2 years. She then on her own authority stopped taking tamoxifen and ceased regular surveillance.\nWe performed CT scans of the neck and breast, bone scan, and PET/CT. CT scans showed multiple enlarged lymph nodes in the left axillary region and the left lower cervical chain, and PET/CT showed multiple conglomerated enlarged lymph nodes with high FDG uptake (SUVmax, 7.02) in the left axillary, retropectoral, subclavian, internal mammary, and cervical lymph nodes. There were no metastatic sites except the lymph nodes. A core needle biopsy of the left axillary lymph node was performed, and pathological examination revealed metastatic adenocarcinoma, which was positive for ER, negative for PR, and equivocal for HER2 (score 2). She was postmenopausal based on the serum follicle-stimulating hormone level, and she started receiving the nonsteroidal aromatase inhibitor letrozole in May 2010. She also received radiotherapy to her left chest wall and regional lymphatics including the cervical chain (total dose, 5,040 cGy) between August 18 and September 30, 2010. Follow-up CT scans showed a complete regression of the previously invaded lymph nodes in November 2010.\nAbout 10 months later, she complained of a mild throbbing pain in the right buttock area that was aggravated by walking. On physical examination, there was no palpable mass and tenderness in the buttock area. CT scans of the abdomen-pelvis and PET/CT were performed in August 2011. Abdomen and pelvis CT scans showed no notable findings, but PET/CT showed multiple new enlarged lymph nodes with increased FDG uptake (SUVmax, 15.4) in the right iliac chain and a nodular lesion of about 2.5 cm in diameter (SUVmax, 20.1) in the right gluteus maximus muscle. We decided to switch from letrozole to the steroidal aromatase inhibitor exemestane on disease progression. Three months later, a follow-up abdominal CT scan showed no discernible change, so the patient continued to take exemestane. After 6 months of taking exemestane, PET/CT showed an increase in size of the right gluteal muscle mass and right iliac lymph nodes (). There was no evidence of local recurrence or other distant metastases. Based on PET/CT images, a poorly defined, mildly enhanced mass of 3 cm in diameter was identified in the right gluteus maximus muscle upon abdominal CT scan (). Therefore, we performed ultrasound-guided core needle biopsy of this gluteal muscle mass in February 2012. Pathological examination revealed invasive ductal carcinoma (), which was negative for ER and PR, and equivocal for HER2 (score 2). HER2 gene amplification was not observed by fluorescence in situ hybridization (HER2/CEP17 ratio, 1.26). Thus, expression of ER showed discordance between the primary breast cancer and the metastatic carcinoma. The patient received palliative chemotherapy with docetaxel and epirubicin. After two cycles of chemotherapy, a follow-up abdominal CT scan showed partial regression of the gluteal muscle mass and iliac lymph nodes, with a decrease in diameter of the gluteal muscle mass from 3.0 to 2.1 cm. She continued to receive chemotherapy and follow-up. |
A 30-year old primigravida presented to our center on 17 October 2003 with a one year history of abdominal swelling and amenorrhea of 19 weeks duration. The abdominal swelling started as a small lump but markedly increased in size in the preceding 3 months. It was associated with pain, severe epigastric discomfort, constipation, weakness and swelling of the legs.\nThe patient was ill-looking, clinically pale and had bilateral pitting pedal edema. The pulse rate was 80 beats per minute and the blood pressure was 120/80 mmHg. The respiratory rate was 24 cycles per minute. The abdomen was grossly distended and tense. There was a massive central abdomino-pelvic mass which was firm and irregular, measuring 40 cm from the symphysis pubis.\nAbdominal sonography showed an intra-uterine viable singleton fetus of 20 weeks gestation. It also showed a 30 cm multi-loculated cystic tumor with a thick capsule located at the right posterior-superior aspect of the uterus and free fluid in the peritoneal cavity. A sonographic diagnosis of ovarian tumor in pregnancy was made.\nBlood tests showed a hematocrit of 22%, and normal electrolytes, urea and creatinin levels. The woman's blood group was 0 Rhesus positive and the hemoglobin genotype was AA. Malaria treatment was started following a positive smear test and two units of sedimented cells were administered to correct the anaemia. Because of the severity of the symptoms and the sonographic findings being suspicious of malignancy, surgery was proposed and discussed with the patient. Laparotomy was performed under general anaesthesia with endotracheal intubation. Operative findings included ascites, normal liver, spleen, kidneys, diaphragm, ovaries and fallopian tubes. The uterus was soft and the size was adequate for 20 weeks of gestation. Fetal movements were visible. A cystic subserosal fibroid measuring 32 cm in diameter was situated at the right posterior superior aspect of the uterus. [Fig ]\nThe subserosal fibroid was adherent to the omentum and the anterior abdominal wall. It was removed and the myoma bed was quickly closed with 2-0 polyglactin suture and hemostasis was easily achieved. The estimated blood loss was 600 mls and 2 units of whole blood were transfused intra-operatively. The tumor weighing 7.7 kg was sent for histology.\nIntravenous magnesium sulphate was administered to prevent uterine contractions and the woman had an uneventful post-operative follow up. The post-operative hematocrit was 30% and the woman was discharged from the hospital 10 days after the operation. The histology report showed sections of interlacing bundles of smooth muscles with areas of hyaline degeneration with no evidence of malignancy. Repeat sonography during antenatal care visits showed a normally growing fetus and the remainder of the antenatal period was uneventful. The woman went into spontaneous labor at 38 weeks gestation and delivered vaginally a female baby weighing 3.5 kg with Apgar scores of 8 and 10 at one and five minutes, respectively. Two days post partum the maternal hematocrit was 30% and mother and baby were discharged from the hospital. The 6 weeks post-natal visit was unremarkable. |
A 46-year-old female was referred to the Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Mahidol University, for an evaluation of a swelling left cheek with intermittent pain and numbness of the upper lip. Patients observed a swelling extending from the left lower canthus (the corner of the eye) to the left cheek approximately 6 months ago. Furthermore, blurred vision was experienced.\nExtraoral examination revealed a swelling at the left cheek, left inferior concha and alar of nose. This swelling was rubbery in consistency. Left upper lip had tenderness. The lymph nodes were within normal limit.\nIntraoral examination revealed buccal and palatal swelling of the maxilla. Buccal swelling extended from tooth number 11 to the left tuberosity, whereas palatal swelling involved approximately three-fourth of the palate. The covering mucosa was normal. The area of teeth number 21–24 was an edentulous area. Teeth number 11, 12, 25, 26 and 27 had first to second degree mobility and were positive to electric pulp test.\nRadiographic examination with periapical and panoramic films showed a destruction of alveolar bone of teeth number 11, 12 and 25. Water's radiograph revealed that a left maxillary sinus was unclear, but the wall of the sinus could be traced. Under anesthesia, an incisional biopsy was performed.\nHistopathological examination demonstrated a submucosal mass in a mature collagenous fibrous connective tissue covered by parakeratinized stratified squamous epithelium. The odontogenic epithelial tumor cells were arranged in follicle and cord patterns. Individual tumor island or cord consisted of two cellular features. The peripheral cells were cuboidal or columnar cells with reversed nuclear polarization and nuclear palisading. The central cells were basaloid cells with hyperchromatic nuclei and scant cytoplasm. Mitotic activities of these basaloid cells can be observed. Squamous metaplasia was detected in a few islands. Based on these microscopic features, the diagnosis of ameloblastoma with basal cell pattern was made.\nThe treatment plan of this lesion was partial maxillectomy under sedation. The essential histopathological features of the excisional biopsy were similar to the incisional biopsy. Generally, ameloblastomatous tumor cells were arranged in a follicular pattern. Ameloblast-like cells at the periphery of the islands were obviously observed. Most of the central cells were basaloid cells, whereas central stellate reticulum-like cells were occasionally noted [Figure and ]. Therefore, the histopathological diagnosis of ameloblastoma with basal cell pattern was rendered.\nAfter follow-up for 17 months, a prosthodontist referred this patient back to the Department of Oral and Maxillofacial Surgery to evaluate the two nodules at the left nasal cavity and left maxillary sinus. These masses were red in color and soft in consistency. Under local anesthesia, an incisional biopsy was generated.\nHistopathological examination of the first recurrence revealed that the majority of the tumor islands resemble basaloid ameloblastoma. However, in some areas, atypical tumor cells showing cellular pleomorphism and nuclear hyperchromatism were detected [Figure and ]. Occasionally, low columnar or cuboidal cells arranging themselves in duct-like patterns were observed. In some tumor islands, eosinophilic hyalinized materials and squamous metaplasia were found. Therefore, the diagnosis of this incisional biopsy was AC. Then, wide excision was performed. Histopathological features of an excisional biopsy were similar to incisional biopsy. However, many clear cells were observed. Mitotic activities were more frequently noted. In addition, invasion of cartilage by tumor cells was detected []. Therefore, a diagnosis of AC was confirmed.\nAfter 2 years, patient returned to the Department of Oral and Maxillofacial Surgery because of swelling above the left lower eyelid and cheek. The swelling was soft in consistency without tenderness. The lymph nodes were within normal limit. Endoscopy revealed a slough tissue at the nasal cavity and in the left maxillary sinus.\nRadiographic examination with Water's view revealed homogeneous radiopaque mass at the nasal cavity and in the left maxillary sinus. Computer tomography revealed that the tumor mass destroyed both lateral and inferior walls of the left orbit as well as the lateral oblique muscle. Tumor mass also compressed a left orbit and pushed it anteriorly [Figure and ]. Treatment plan was wide excision with suprahyoid neck dissection and reconstruction with radial forearm free flap. Histopathological examination of excisional tissue confirmed a diagnosis of AC. The patient is still alive 7 years postoperatively. |
An 80-year-old Caucasian female with history of hypertension and chronic back pain presented for emergent repair of a 7.2 cm aneurysm of the ascending aorta with Stanford classification type A dissection. Because the ascending aorta was unsuitable for arterial cannulation, the surgeon elected to perform axillary cannulation via the right subclavian artery with side graft anastomosis. The patient arrived to the operating room (OR) with nicardipine and esmolol infusions running through an 18-gauge peripheral intravenous (IV) line in the right antecubital (AC) fossa. Prior to induction of anesthesia, we disconnected the infusions from the right AC and administered medications though an 18-gauge IV in the left forearm. The patient also had a left radial arterial line (AL), and we placed the pulse oximeter and noninvasive blood pressure (NIBP) cuff on the RUE. After intubation, we placed a right radial AL, and the surgeons placed a left femoral AL. All arterial pressures correlated closely. Additionally, a 9-French central line was placed in the right internal jugular vein. The patient was cleansed and draped for surgery with her arms tucked to her sides.\nShortly after the procedure began, the right radial AL tracing went flat, and the pulse oximeter waveform was lost. We attributed this to the surgeon partially clamping the right subclavian artery in preparation for arterial cannulation. We switched the pulse oximeter to the left hand and relied on the left radial and femoral AL for pressure readings. Just prior to arterial cannulation, we noted that the right radial pressure returned, although about 20 points lower than the left radial/femoral. Immediately after initiating CPB, the right radial mean arterial pressure (MAP) increased to 200 mm Hg, and left radial/femoral MAP decreased from 60 to 30 mmHg. The perfusionist alerted the surgeon about the high line pressures and decreased CPB flows. After a brief attempt to troubleshoot and adjust the cannula with little improvement in pressure or flow, the surgeon proceeded with the operation. Over the next few minutes, the left radial/femoral MAP increased to 60 mmHg.\nAs deep hypothermic circulatory arrest (DHCA) was initiated, the right radial MAP decreased to 30 mmHg and left radial/femoral MAP decreased to 10. When CPB was reinstated, right radial MAP again increased to 200 mmHg. After 29 minutes of DHCA and 265 minutes of CPB, the patient was successfully weaned from CPB, and right radial MAP decreased to about 10 points lower than left radial MAP.\nDespite these issues, the surgery was otherwise uneventful. However, upon the surgical drapes being taken down, we noticed that the patient's RUE was swollen with blisters and bullae from the shoulder to the hand; yet, the skin of the upper arm where the NIBP cuff had been placed was normal as seen in figures. The IV in the right AC appeared to be infiltrated and weeping fluid even though we had not used it during the case and did not have any IV fluids attached to it (Figures and ). The IV and right radial AL were removed in the OR, and a Xeroform gauze dressing was applied to the RUE with the surgeons present. The intensive care unit nurses were instructed to elevate the arm and perform hourly neurovascular checks.\nOn postoperative day 1, the patient complained of tenderness and burning in the RUE, but she maintained adequate capillary refill, motor function, and sensation. Plastic Surgery was consulted to rule out compartment syndrome. They were unsure of the diagnosis but recommended nonoperative management and continued neurovascular checks. Eventually Dermatology was also involved, and they performed a punch biopsy of the patient's right dorsal hand. Their initial diagnosis was allergic contact dermatitis (ACD) due to the fact that the area of skin covered by the NIBP cuff was spared. However, the biopsy showed pauci-inflammatory dermal-epidermal blistering, which did not favor ACD. Direct immunofluorescence was also negative, ruling out localized pemphigus. Given the histologic findings, the final diagnosis was hydrostatic edema/bullae correlating with rapid edema during surgery. The patient continued to be managed nonoperatively with 1% triamcinolone ointment and gauze dressings, and within one month the blisters had completely resolved. |
Patient is a 70-year-old female with past medical history significant for prior breast malignancy status post breast conserving surgery, adjuvant chemotherapy and radiation, and five years of anastrozole treatment, who presented to the Surgical Clinic for evaluation of assumed tender hematoma on the medial aspect of her left thigh. Patient reports that painful swelling appeared on medial portion of proximal left thigh after low grade trauma one month prior when the site in question collided with exercise equipment. She stated that she was experiencing mild discomfort, especially with ambulation, and that the tender area was enlarging. She was planned for follow up in six months for her presumed traumatic hematoma. However, patient returned to clinic three months later as pain was persistent, the “hematoma” continued to enlarge, and was beginning to affect her mobility. At that time, patient underwent ultrasound (US) and computed tomography (CT) to rule out any underlying pathology and evaluate the extent of the hematoma in order to proceed with incision and drainage. The US was pertinent for report of “massive hematoma on medial left thigh, 17.8 cm × 12.4 cm × 12.1 cm, comprised of multiple septations.” Her extremity (CT) scan, revealed a significant mixed solid and cystic mass within the Sartorius muscle of the medial left thigh with multiple nodules and septations (Fig. ). The mass measured approximately 12.2 cm × 12.5 cm × 17.5 cm, and was noted to abut the Superficial Femoral Artery and be in contact with the Saphenous vein. No skeletal involvement was visualized. Given these findings, the diagnosis of hematoma was discarded in favor of neoplasm. She underwent Magnetic Resonance Imaging (MRI), where reports measured a well-defined, T2 intense mass, 11 cm × 13 cm × 19 cm, in the left Sartorius muscle with mixed components of nodules and multiple septa, as well as subacute and chronic hemorrhages, and a diffuse, thick capsule (Fig. ). The mass compressed the Vastus Medialis muscle and displaced the adductor compartment, as well as abutted the Femoral Artery and Femoral Vein, and displaced and compressed the Greater Saphenous Vein, with no documented bone involvement. Due to these findings, the patient underwent biopsy of the mass which revealed a myxoid sarcoma. The decision was made with the patient to proceed with surgical excision of the mass, and pathology results of the excision were significant for a 26 cm Extraskeletal Osteogenic Sarcoma. She underwent Positron Emission Tomography (PET), which was negative. Her post-operative course was complicated by a 10 cm seroma formation at the surgical site. A Jackson-Pratt drain was placed, which had continued daily outputs of greater than 600 mL, so she went to the operating room for evacuation and closure. She developed a site infection and seroma recurrence, then returned to the operating room for debridement and full thickness skin graft with negative pressure wound vac therapy. Prior to beginning chemotherapy, patient had worsening pain and respiratory complaints, thus she elected to pursue palliative therapy and shortly thereafter expired. |
The second case shows a 53-year-old female. Clinical and radiographic examination confirmed a unilateral posterior crossbite due a transverse maxillary deficiency with a significant mandibular skeletal deviation towards the side of the crossbite (Fig. ). Treatment objectives.\nSARME was planned to correct the transverse discrepancy followed by arch leveling with lingual appliances and then a second surgery to correct the mandibular asymmetry.\nSimilar to case 1 impressions were obtained and this time the lingual appliances were manufactured by DW Lingual Systems (Bad Essen, Germany).\nDuring the planning for the production of the lingual brackets, it was noted -similar to case 1- that a surgically assisted rapid maxillary expansion takes place. The transverse width of the upper jaw should be adapted to the lower jaw.\nTwo trans sagittal Benefit mini-implants were inserted in the T-Zone. A silicon impression with the transfer caps was taken. The impression was given to the laboratory together with the lingual molar bands. A Hybrid Hyrax [] was then made and laser welded to the molar bands (Fig. ). Similar to case 1, the lingual appliance was indirectly bonded with a dual cured resin and the maxillary expansion appliance was inserted. In this case the molar bands were cemented with a dual cured resin and the hybrid hyrax was fixed to the mini-implants using the Benefit fixation screws. The first lower arch wire 12 NiTi was inserted while in the upper the brackets were secured with a continuous steel ligature in each quadrant (Fig. ). SARME was performed with an activation rate of two quarter turns twice a day until crossbite correction was achieved at two weeks post-surgery (Fig. ). The Hybrid Hyrax was then blocked. The first upper archwire (12 NiTi) was placed four weeks after surgery (Fig. ). After complete leveling and radiographic re-examination the surgery to correct the asymmetry was performed.\nThe patient has a positive overbite and overjet now. The patient shows a good transversal and sagittal occlusion. |
A 67-year-old man complained of left shoulder pain for 2 weeks without any history of trauma. Bloody effusions were aspirated 5 times at a local orthopedic clinic during a 2-week period before the patient was referred to us. He had no previous history related to hemorrhagic factors. On examination, he had slightly limited range of motion, with active forward flexion to 160°, abduction to 120°, external rotation to 30°, and internal rotation to the fifth lumbar spine with the arm at the side. Passive range of motion was the same as active range of motion. Neer's impingement test and Hawkins test were positive. The greater tuberosity was seen irregular on radiography. MRI demonstrated a hematoma in the anterior portion of the subdeltoid bursa and bony erosion of the lateral acromion, similar to case 1 ().\nArthroscopy was performed to determine the cause of the hemorrhage. A hematoma was not observed in the glenohumeral joint. The intra-articular portion of the long head of the biceps tendon had completely disappeared. A bursal-side partial tear of the rotator cuff was noted. The size of the tear was 25 mm in the anteroposterior direction. Crater formation on the undersurface of the acromion and irregularity of the greater tuberosity were observed, which seemed to be kissing lesions (Figures and ). Active bleeding was seen at the exposed bone marrow of the acromion when the pressure of irrigation was lowered. The undersurface of the acromion was coagulated and the greater tuberosity was abraded to avoid impingement (). Acromioplasty was not performed in this case. The bursal-side partial tear was not repaired at that time ().\nOne month later, the patient underwent arthroscopic rotator cuff repair. The patient regained full range of motion 3 months after rotator cuff repair and returned to work as a taxi driver. At the final follow-up, 14 months after the first arthroscopy, he had no functional deficit. The UCLA score was improved from 14 points preoperatively to 35 points at the final follow-up. Hemarthrosis did not recur in the 14 months postoperatively. |
A 66-year-old woman was diagnosed with a recurrent giant anterior chest wall keloid, which she had had for more than 10 years. Past history consisted of repeated failed excisions and intralesional steroid injections. The patient suffered from local itching and pain, as well as progressive enlargement of the keloid. What was worse was that due to the giant keloid pulling the breasts on both sides of the chest skin, the patient could not walk upright. She felt pain and itching from the keloid and her breathing was also affected, resulting in poor sleep. On physical examination, a large 17 cm (L) × 8 cm (W) × 2 cm (T) epidermal mass was seen on the midline of the chest in the middle of both breasts, with an ulceration and small amount of exudation (Fig. ). The patient had a history of hypertension.\nWe considered that the patient had not been treated with adjuvant therapy (e.g., radiation or injection therapy) after the initial surgery, and that the irregular follow-ups after surgery was one of the reasons for the increase in her keloid size. In addition, the hyperplasia of keloids destroys the hair follicles and sebaceous gland structure of the local skin, which results in the sebum being blocked. As well, the itching caused by keloid hyperplasia prompts the patient to scratch her skin, resulting in repeated infection and ulceration of the local keloid tissue. The new wound of skin stimulates the growth of the keloid, which aggravates the disease. Taking the patient's age and the risk of hypertension into account, we did not choose a one-time resection of the keloid, but instead a treatment of multistage surgery combined with radiotherapy. Histological analysis revealed typical keloid features (Fig. ).\nBefore each operation, the resection area was evaluated. After the single resection, the resection area was directly sutured and the keloid was removed as much as possible. Subcutaneous and intradermal reduction sutures were used for absorbable sutures, and the leather surface was sutured with non-absorbable lines. The 5 mg of the betamethasone injection was injected locally around the wound immediately after the operation. The wound was covered with sterile gauze and pressurized with an elastic bandage.\nOn December 2nd of 2014, we designed a lateral fusiform incision to remove the keloid, of which about 15.0 cm (L) × 4.0 cm (W) × 2.0 cm (T) in size was removed (Fig. A, B). Due to the large radiation area after surgery, radiologists did not recommend radiation therapy because of the risk of radiation carcinogenesis.\nOn May 12th of 2015, before the second operation, the color of the keloid became lighter. We designed a lateral fusiform and a longitudinal incision, removing the keloid, of which about 15.0 cm (L) × 3.0 cm (W) × 1.0 cm (T) in size was removed (Fig. C, D). This relieved the tension between the two breasts, and improved the patient's thoracic activity.\nOn December 15th of 2015, before the third operation, the color of the keloid was obviously rosy and the patient reported relief from pain and itching. We designed a lateral fusiform incision to remove the keloid, of which about 15.0 cm (L) × 2.0 cm (W) × 1.0 cm (T) in size was removed (Fig. E, F).\nOn May 31st of 2016, at the time of the fourth operation, the distance between the breasts on both sides was almost normal, and the thoracic activity of the patient returned to normal. We extended the length of the incision and remove the previously uncut keloid located under the breasts, of which about 20.0 cm (L) × 1.0 cm (W) × 1.0 cm (T) in size was cutted off (Fig. G, H). Radiotherapy was applied within 24 hours after surgery at a dose of 15 Gy (3 Gy/day × 5 days).[\nOn March 9th of 2017, we designed the longitudinal fusiform incision for the fifth operation, and removed the keloid in the longitudinal direction, of which about 5.0 cm (L) × 2.0 cm (W) × 0.5 cm (T) in size was removed (Fig. I, J). Radiotherapy was performed after the operation, and the dose was the same as before.\nDuring the first to third interoperative period, patient was treated once every 4 to 8 weeks with intralesion injections of 5 mg of betamethasone and 2 mg of pingyangmycin. After the third operation, the scar volume was significantly reduced and showed no ulceration and exudation. The intraoperative injection treatment was changed to topical halomethasone cream, imiquimod cream, and asiaticoside cream.\nAt 1 year after surgery, mild hyperplasia was found in the wound, and local injections of betamethasone and 5-fluorouracil were given (Fig. K). At 2 years after surgery, no significant recurrence was observed (Fig. L). The patient was highly satisfied with her result. |
A 41-year-old lady with acute necrotizing encephalitis involving thalamus and secondary cortical venous thrombosis underwent surgical tracheostomy for long-term ventilatory support. The patient had various medical problems including urinary tract infection and a past history of uncontrolled diabetes mellitus. The surgical tracheostomy was uneventful. Eight days later, she had sudden onset of slow bleed from the tracheostomy site. Local exploration with tube in situ was unremarkable. Heparin thromboprophylaxis was withheld temporarily and tracheal suctioning frequency was reduced. The following day, she developed an acute massive hemorrhage with spurting flow from the tracheostoma, with an estimated blood loss of >500 mL in few minutes. Tachycardia was noted, though the blood pressure remained stable.\nAcute management was initiated by the neurointensivist with suction for airway clearance and inflation of the tracheostomy cuff to a pressure of 80 cm H2O. Fluid resuscitation was initiated along with transfusion of blood and blood products. Direct inspection of stoma by endorhinolaryngologists revealed frank arterial spurt. It was noted that the bleeding point was above the cuff; and for the next 5 minutes, the surgeons tried in vain to contain the bleeder. Immediately endovascular management was resorted to and the patient was transferred to cath lab to visualize and embolize the culprit vessel. Direct pressure was applied on the tracheal and paratracheal soft tissues, with which the bleeding was temporarily controlled.\nThe digital subtraction angiography undertaken within 15 minutes of bleeding revealed a pseudoaneurysm arising from the right innominate artery, in contact with the anterior wall of the tracheostomy tube, which was later confirmed by three-dimensional reconstruction of computed tomography (CT) angiogram (). An endovascular stent implantation was performed, traversing and occluding the mouth of the pseudoaneurysm (). In the process, the right subclavian artery was sacrificed. The patient was sedated, paralyzed, ventilated, and resuscitated with colloids and three packed red cell units. She was transferred back to intensive care unit and her further recovery was unremarkable. |
A 64-year-old man, 165 cm in height and 70 kg in weight, visited the department of neurosurgery due to severe pain on the left lateral leg. On physical examination, left ankle dorsiflextion was impaired. Magnetic resonance imaging (MRI) of lumbosacral vertebral column showed a left paramedian disc extrusion, between lumbar 4 and 5 vertebras. The patient was scheduled for discectomy. In premedical history, the patient complained of dysarthria, dysphagia and weakness in both legs, while walking 3 years ago. He also complained of dyspnea, although, there were no abnormalities on the blood gas analysis. On the physical findings by a neurologist, deep tendon reflex was exacerbated and babinski test was positive. The electromyogram (EMG) suggested that the patient had a motor neuron disease and he was diagnosed with ALS. Pulmoary function test was not performed before the operation, due to the undergoing emergency operation for resolution of severe pain on the left leg.\nSpinal anesthesia for discectomy was chosen to avoid respiratory complication, which is associated with general anesthesia. No preanesthetic medication was given. Spinal anesthesia was done between lumbar 3 and 4, via a midline approach in the left lateral position and was injected with 10 mg of 0.5% hyperbaric bupivacaine, plus fentanyl 20 µg using a 25 guage Quinke spinal needle. Immediately after the injection of local anesthetic agent and opioid, the patient was placed back to the supine position. The level of the spinal anesthesia was T10, after 15 minutes and then we changed the patient's position from supine to prone position. Propofol at a rate of 0.5-1.0 mg/kg/hr was infused for sedation with O2 5 L/min via nasal cannula. Intraoperatively, vital signs were as follows: blood pressure of 122 to 139/81 to 95 mmHg; heart rate between 75 and 97 beats/min; and oxygen saturation of 100%. There were no complications for one and a half hour of the surgery. The patient was monitored for one hour, in postoperative anesthetic care units (PACU), and then was discharged from PACU. The patient was fully recovered at 5 hours, after the spinal anesthesia and no exacerbation of neurologic signs or symptoms were revealed. The patient was discharged two days after surgery and at the 3 months follow-up, there were no neurologic complications due to spinal anesthesia. |
A 46-year-old female underwent a pulmonary vein isolation (PVI) for persistent atrial fibrillation (AF) and catheter ablation of bigeminal premature ventricular contractions (PVCs) originating from the right ventricular outflow tract (RVOT). Radiofrequency (RF) applications on the septal aspect of the RVOT eliminated the PVC. However, a sustained VT with a cycle length of 320 ms causing syncope occurred 2 weeks after the procedure (Figure ). The morphology of the VT was identical to that of the prior PVC from the RVOT. Despite RF applications at the RVOT during the second procedure on the day of readmission, the VT recurred immediately after the second procedure. The recurrent VT was refractory to medical therapies including beta blockers, sotalol, and intravenous amiodarone under continuous intravenous sedation. During the third procedure at 15 days after the second procedure, RF applications at the earliest activation site on the left coronary cusp (LCC) failed to suppress the VT, and a bipolar ablation attempted between the RVOT and LCC also failed to eliminate the VT. Since increased sympathetic nerve activity represented by an increased low-frequency/high-frequency (LF/HF) ratio of 4.3, a normal range of >2 on the 24-hour Holter recording, may have been associated with the VT, and a bilateral cardiac sympathetic denervation (CSD), which removed the lower half of the stellate ganglion and T2-T4 sympathetic ganglia, was performed 17 days after the third procedure. Although non-sustained VTs recurred with a cycle length of 360 ms within a day after the surgery, the number of nonsustained VT episodes decreased with a suppressed activity of the LF/HF ratio of 1.0, and the resting heart rate during sinus rhythm of 80 bpm decreased to 50 bpm after the surgery. The VT completely disappeared after initiating oral amiodarone of 200 mg 10 days after the CSD (Figure ), and the patient has been free from any VT for 7 months after the CSD without any complications. |
A 43-year-old male patient is followed in our MS clinic since 1994 with the diagnosis of clinical definite relapsing-remitting MS. The clinical presentation was a grade 4 paraparesis with no other symptoms; the initial MRI showed typical lesions for MS and cerebrospinal fluid analysis revealed positive oligoclonal bands. Interferon beta 1-b was started as treatment in 2003 with clinical efficacy and the patient had a full recovery of the paraparesis. In 2003 a routine brain MRI revealed a new extensive subcortical and deeper white matter lesion localized in the left frontal lobe, which is noncontrast-enhancing, suggesting a tumefactive demyelinating lesion (). There were no clinical changes and imagiological characteristics of this lesion remained unchanged in consecutive MRIs. In 2006, despite the absence of new symptoms or relapses, a poor therapeutic adherence was documented and switch to glatiramer acetate (GA) was performed. In 2008, the patient presented with partial complex and generalized seizures in association with progressive right hemiparesis. A new cerebral MRI () revealed again the large left frontal lesion, but now with space-occupying characteristics, subtle contrast-enhancement, and mass effect, suggesting an infiltrative lesion of glial series. On this basis, a presumptive diagnosis of low grade glioma was made and a cerebral biopsy was performed. Histological examination () revealed cells with clear cytoplasm, round nuclei, and granular chromatin; glial fibrillary acidic protein (GFAP) immunopositivity evidenced neoplastic cells expression; proliferation index was less than 5%. These features were found to be diagnostic of a World Health Organization grade 2 oligodendroglioma. GA was interrupted and patient underwent treatment with conventional fractioned radiotherapy with 30 fractions of 2 Gy to total dose of 60 Gy. Seizures were controlled with valproic acid 1000 mg per day and levetiracetam 1500 mg per day. At 3-year follow-up, sequential MRI revealed both demyelinating and neoplastic stable lesions. After 3 years of follow-up, the patient presents minor right hemiparesis and dysarthria. |
A 76-year-old Japanese woman presented to our facility with epigastric discomfort; she had previously undergone an esophagogastroduodenoscopy (EGD) procedure at another hospital. The EGD had revealed gastric wall thickening, suggesting scirrhous gastric carcinoma, so our patient was referred to our hospital for further examination and treatment.\nA barium upper gastrointestinal X-ray series indicated rigidity and poor extensibility of the gastric wall from the fornix to the lower gastric body, and irregular, enlarged folds were also noticeable (Figure ). EGD revealed reddish and markedly swollen folds at the greater curvature from the upper to the lower part of the gastric corpus (Figure ). The mucosa surface was hemorrhagic and erosive. A biopsy specimen revealed amyloid deposits in the submucosal layer with no malignant findings. Endoscopic ultrasound showed obvious thickening of the third layer corresponding to the submucosal layer with no disruption of the gastric wall structure (Figure ). Computed tomography (CT) revealed thickening of the gastric wall and enlarged circumambient lymph nodes. Biopsy specimens from the rectum and ileum indicated no amyloid deposition. Laboratory test results were negative for Bence-Jones protein in the urine, and serum immunoglobulin levels were normal. No abnormal signs were detected on echocardiography.\nWe resected a representative portion of the lesion by endoscopic mucosal resection (EMR) using the strip biopsy method to obtain sufficient tissue specimens, and we then conducted a detailed histological evaluation (Figure ). There were no complications associated with the EMR. The resected specimens revealed deposition of amyloidal materials in the gastric mucosa and submucosa with no malignant findings. Congo red staining results were positive for amyloidal protein and exhibited green birefringence under polarized light microscopy. Congo red staining with prior potassium permanganate incubation confirmed the light chain (AL) amyloid protein type. An immunohistochemical examination revealed that κ-chain-positive plasma cells were present only in the inflammatory infiltrate in the superficial lamina propria, while λ-chain-producing plasma cells were detected both in the mucosa and in the underlying lymphomatous proliferation (Figures , , ). Based on these results, gastric malignancy, systemic amyloidosis and amyloid deposits induced by inflammatory disease were excluded and the lesion was consequently diagnosed as localized gastric amyloidosis. Our patient was an older woman and there were no findings relative to an increase in gastrointestinal symptoms or anemia, so no further treatment was performed and she continued to be in good condition with no findings of disease progression six years after verification of our diagnosis. |
This 67-year-old woman presented on 3 March 2008 at 16:00 to the neurological emergency room. Earlier that day (at 14:50), her relatives had witnessed the acute onset of a fluctuating paresis of her left arm and leg. Diffusion weighted MR images, carried out at 16:30, revealed an area of restricted diffusion in the right basal ganglia and the deep periventricular white matter and an MR angiogram demonstrated complete occlusion of the right middle cerebral artery (MCA) in the M1 segment. The emergency stroke team decided to initiate intravenous thrombolysis, which started within a 2 h window (16:45). As the patient did not show any clinical improvement within the next 30 min, the attending stroke physician decided to proceed with a bridging concept and endovascular treatment was initiated, after the patient was informed of the proposed procedure. This information included a description of the procedure, the potential risks and benefits and an explanation that extensions of the procedure (eg, surgical craniectomy) or the off-label use of medical devices (eg, coronary stents, etc) might become necessary. The patient gave informed consent to the operator. The neurological examination before digital subtraction angiography (DSA) showed a severe dysarthria and a left-sided hemiplegia with facial nerve palsy (National Institutes of Health Stroke Scale (NIHSS) score of 10). The emergent angiography began 3 h and 20 min after the onset of symptoms and the first DSA run was recorded at 18:12.\nUsing a 4 F diagnostic catheter, selective injection of both vertebral arteries and internal carotid arteries (ICAs) was carried out to show the extent of leptomeningeal collaterals and to rule out distal emboli, eventually not visible on MR angiography. Complete embolic occlusion of the right MCA at the M1 segment with moderate leptomeningeal collaterals between the right anterior cerebral artery and MCA was confirmed ().\nSystemic anticoagulation with 5000 U of heparin was initiated. Via a 6 F Envoy guiding catheter (Cordis, Miami, USA) in the right ICA, a straight Rebar27 was gently advanced to the thrombus under the guidance of a SilverSpeed16 microguidewire (both ev3). Under road map, a thrombectomy brush (pCR, phenox; Bochum, Germany) was deployed in the right angular artery and subsequently slowly withdrawn under continuous aspiration. The brush was loaded with clot material but no recanalization of the target vessel was observed (TICI 1). During the catheterization, the thrombus appeared organized and firm. The chances of success of either LIF or repeated pCR passages were considered low as previous experience has shown very low recanalization rates with pCR and LIF in firm organized emboli. It appeared evident to the operator (HH) that in this situation, more interaction between clot and retrieval device was required. While contemplating the use of either a Catch or a Merci retriever (at this time the only other approved devices for mechanical thrombectomy), we recognized that neither of these tools was in stock. After balancing the potential risks and benefits to this patient and given the uncertain outcome if there was no recanalization, we decided to use a Solitaire stent in the way we would have otherwise used a Catch or a Merci device. The right angular artery was again gently catheterized with a Rebar18 catheter. After removal of the microguidewire, a self-expanding Solitaire stent (4 mm diameter, 20 mm length) was inserted under continuous slow flush with saline solution to the distal end of the microcatheter and was deployed by slowly pulling back the microcatheter. Correct deployment of the Solitaire stent over the whole length of the thrombus was observed under continuous fluoroscopy. In order to avoid mechanically induced vasospasm, 2 mg of glycerin trinitrate were injected intra-arterially. After several minutes of incubation and with the rotating hemostatic valve tightly closed, a 50 ml Luer lock syringe was used for high volume aspiration through the 6 F guiding catheter during withdrawal of the open Solitaire stent and the microcatheter into the guide catheter. Withdrawal was possible with minor effort and was observed under continuous fluoroscopy. As soon as the device became visible, the rotating hemostatic valve was quickly removed from the guiding catheter and another 50 ml of blood were aspirated. The Solitaire stent was loaded with a thrombus (). Contrast injection of the right ICA after removal of the Solitaire confirmed total recanalization of the right MCA (; TICI 3). Neither peripheral emboli nor vasospasm at the previously occluded vessel site was encountered.\nThe entire procedure (from the first to the final DSA run) lasted 59 min. The patient awoke from general anesthesia with only mild neurologic deficit (NIHSS 2). The patient was discharged to a rehabilitation center. At the 30 day follow-up, NIHSS was 0.\nThe patient was heparinized and received 100 mg of acetylsalicylic acid daily as secondary prophylaxis. ECG monitoring revealed atrial fibrillation as a potential source of cardiac embolism and hence the patient was anticoagulated with phenprocoumon. MRI examinations were refused by the severely claustrophobic patient. DSA 24 days after treatment showed a completely normal M1 segment and patent cortical branches of the entire right MCA. Follow-up CT 22 months later showed a small post-ischemic lesion of the right basal ganglia and normal cortex (). |
A 40-year-old-woman with a history of Gardner's Syndrome status post prophylactic colectomy presented with an enterocutaneous fistula and for reevaluation of desmoid tumors. She had three previous abdominal operations including a prophylactic colectomy in 1992. In 1995, she had a resection of small intestine and marginal resection of associated desmoid tumors causing small bowel obstruction. In 2002, an emergent resection of small intestine and desmoid tumors for intestinal perforation removed only a portion of the diffuse mesenteric disease. This operation was complicated by an enterocutaneous fistula from the jejunum that subsequently healed with conservative care. She had no adjuvant radiation therapy at any point. Estrogen and progesterone receptor expression of the tumors was never assessed.\nDespite treatment with anti-inflammatory agents, the patient's abdominal desmoid tumors continued to grow and she was re-admitted 10 months later for recurrence of her enterocutaneous fistula. Radiographic studies showed near-complete small bowel obstruction by desmoid tumor (Figure , ) and complete diversion of the fecal stream through the fistula. She began total parenteral nutrition (TPN). The fistula was not amenable to operative repair without resolution of the distal small bowel obstruction. Options for resolving the distal obstruction included operative resection of the desmoids, a procedure associated with much potential morbidity, or systemic therapy to reduce the size of the desmoids. The patient was offered therapy with anti-estrogen agents or investigational therapy with imatinib. Imatinib was initiated and was ineffective, possibly related to the patient's difficulty with gut absorption. Imatinib was discontinued, and she began a regimen of cytotoxic therapy that is active for sarcoma, including doxorubicin 20 mg/m2 IV on days 1, 2, and 3 and ifosfamide 2 g/m2 daily for 3 days of a 21 day cycle. She received five cycles of this regimen, with a clear radiographic response on CT scan (Figures , ). However, her high-output fistula continued.\nA barium enema (Figure ) demonstrated resolution of the distal obstruction with reflux of contrast through the fistula. Enterocutaneous fistula resection with primary enteroenterostomy was performed without complication. The patient was discharged on TPN for continued nutritional support, although she was able to tolerate oral feedings with normal bowel function. Her midline surgical wound subsequently healed by secondary intent. With the return of gastrointestinal function, antiestrogen therapy with tamoxifen 20 mg po daily was initiated. |
In April 2004, a 79-year-old man presented with a 4 month history of subcutaneous abdominal nodules and bilateral inguinal lymphadenopathy; no other signs or symptoms were present.\nThe patient was retired after various occupations; he had also worked for 7 years at the local port and for 3 years in a motor factory, but he had no memory of direct exposure to asbestos. He had no smoking history. Medical history included partial gastric resection due to gastric and duodenal ulcers with concomitant cholecystectomy for cholelithiasis at 44, surgical removal of a renal stone at 74, diagnosis of benign prostatic hypertrophy at 76.\nA CT of the chest, abdomen and pelvis showed the presence of a large mass occupying the epigastrium and the left upper region of the abdomen (longest diameter: 9 cm), and multiple diffuse abdominal nodules along the peritoneal surface and below the anterior abdominal wall (longest diameter: 5 cm); imaging confirmed the presence of bilateral inguinal lymphadenopathy. A small amount of ascites was present; liver, spleen and kidneys were spared. Chest CT was negative, with no evidence of pleural or pulmonary parenchymal abnormalities. An excisional biopsy of the left inguinal lymphadenopathy was performed. Histological analysis showed malignant neoplasm with a micropapillary, microcystic, and solid growth pattern infiltrating the adjacent soft tissues. Immunohistochemical analysis revealed positive staining of tumour cells for cytokeratins 5 and 7, vimentin, epithelial membrane antigen (EMA), epithelial specific antigen (ESA), calretinin; no reactivity was observed for carcinoembryonic antigen or Tag/B72.3. Histological and immunohistochemical findings were consistent with a well differentiated biphasic malignancy of mesothelial origin.\nIn July 2004 the patient was referred to our Department for management. At admission, he reported progressive clinical deterioration (Karnofsky Performance Status 80), fatigue, unintentional weight loss of 6 kg and persistent fever over the last few weeks. His medications were terazosine and finasteride. Physical examination showed a subcutaneous epi-mesogastric mass (9 × 6 cm) and bilateral inguinal lymphadenopathy. Complete blood count showed haemoglobin 11.6 g/dL, platelet count 559,000 per cubic millimeter, and white cell count 8,400 per cubic millimeter, with a normal differential count; renal function and liver function tests were normal.\nIn consideration of patient's age, and type and stage of disease, palliative chemotherapy with single-agent pemetrexed at 500 mg/m2 i.v. every 3 weeks was proposed []. Pemetrexed was provided by Eli Lilly (Indianapolis, IN) within an Expanded Access Programme. Before chemotherapy start, a written informed consent was obtained and a repeat CT was performed, showing a size increase of all lesions (longest diameter of the mass in the abdominal left upper region: 12 cm) (Fig. ). Treatment started in early August 2004. During the treatment, the patient received vitamin B12 and folic acid supplementation, and steroid prophylaxis []. After the first 2 cycles, a clinical improvement was observed with reduction of frequency and intensity of febrile episodes. Partial response was documented by physical examination (decrease in size of the subcutaneous abdominal mass and bilateral inguinal nodes) and by CT (decrease in size of all known lesions and no new lesions). Treatment was well tolerated with only grade 2 neutropenia, and grade 2 nausea and anorexia. Therefore, pemetrexed was continued for 3 cycles and further tumour shrinkage was documented at physical examination and imaging. Toxicity persisted acceptable with only grade 2 nausea and fatigue; fever disappeared after the third cycle. Three further cycles of pemetrexed were administered without increase in toxicity except for mild fever lasting few days after drug administration. After a total of 8 cycles, the epi-mesogastric mass and the inguinal bilateral lymphadenopathy were no more detectable at physical examination, whereas abdominal CT in April 2005 showed a minimal residue of disease (Fig. ). His Karnofsky Performance Status was 80 and his weight was increased of approximately 10% since treatment start. At that point, after multidisciplinary evaluation and discussion with the patient about therapeutic options, we decided to stop the treatment.\nIn August 2005, a follow-up CT revealed the reappearance of nodular peritoneal lesions. Due to the significant benefit observed and the duration of response (11 months) the patient was retreated with pemetrexed for 4 cycles from August to November 2005, resulting in disease stabilization. At the time of his last appointment at our Department in February 2006, a CT scan confirmed stable disease. |
A 22-year-old female patient with gravida 2, para 0, living 0 and abortion 1 with a history of 24 weeks of amenorrhea, presented to casualty with bleeding per vagina and loss of fetal movements since 2 days. She was from a low socioeconomic background. She had not undergone any antenatal checkups. She had a previous history of abortion. The patient had a history of second-degree consanguineous marriage. Past and personal histories were not significant.\nOn general physical examination, it was observed that the patient was a middle-aged female, moderately built, and nourished. Pallor was present. Per abdomen examination revealed that the uterus was relaxed and uterine height was of 24 weeks. Fetal heart rate was absent. Per vaginal examination revealed partially closed os, admitting only tip of finger, and effacement was absent. Routine biochemical investigations were within normal limits. Hemoglobin level was 7.2g/dL, hematocrit was 22%, and peripheral smear showed microcytic hypochromic and normocytic hypochromic red blood cells with normal white blood cells and platelets, giving a picture of dimorphic anemia. Coagulation profile was within normal limits.\nEmergency antenatal ultrasound examination was carried out and it showed conjoint twins with loss of fetal cardiac activity. After obtaining informed consent from the patient and attenders, induction of labor was initiated with prostaglandins and the pregnancy was terminated by vaginal delivery. Dead male baby weighing 1800g was delivered. After obtaining consent, the fetus was sent to pathology department for histopathological examination.\nExternal examination revealed male fetus with head circumference of 30cm (both the heads), chest circumference of 15cm, abdominal circumference of 20cm, and foot length of 7cm. The fetus had complete duplication of cranial structure with three eyes. One eye of both the duplicated head was fused in the center. Two nose, two ears, two mouths, of which one lip showed cleft lip and palate and another showed only cleft lip, were observed. Fetus showed single short neck, thorax, and abdominal cavity. Both upper and lower limbs were normal.\nBrain was not developed and was replaced by grey brown tissue, showing features of anencephaly. Cervicothoracic and lumbar rachischisis were noted Figure –].\nComputerized tomography (CT) scan and magnetic resonance image (MRI) virtopsy were performed, which revealed duplication of face and confirmed the duplicated facial structures. Both the faces were fused in midline. Fusion of eye was observed in midline. The brain was hypoplastic. There were separate two sets of frontal, parietal, temporal, mandible for each face, which were fused in the center. Occipital bone was absent. One of the fetus had cleft lip and palate. A complete spina bifida with a cleft through the entire spine was observed, suggestive of rachischisis [Figure –]. The fetus had only one short neck, one thoracic, and abdominopelvic cavity showing organs, which were in situs solitus [Figure , –]. No anomaly was detected except for hypoplastic lungs. Autopsy was carried out, which confirmed findings of CT and MRI features. With all these above features, diagnosis of “diprosopus bicephalous triophthalmus” associated with anencephaly and craniospinal rachischisis was made. |
A 50-year-old systemically healthy male presented to the emergency department with a history of traffic accident and possible maxillary fracture. He complaint of mild degree of tenderness and numbness on his left malar area. He also had redness and swelling on his left cheek. Thus, we were suspicious of maxillary fracture. However, facial X-ray showed no visible displacement of zygomaticomaxillary complex. On the other hand, there was increase of haziness in maxillary sinus. Thus, CT was performed for more accurate evaluation. CT scan documented a large amount of fluid collection in left maxillary sinus without fracture line or broken bony structure (). There was a 4-cm long foreign body in maxillary sinus with the shape of a stick. After thorough history taking, we found a history of penetrating injury by chopstick about 30 years ago. He also felt bad smell in his nose and mouth sometimes.\nUnder general anesthesia, gingivobuccal incision was made. Following mucoperiosteal flap elevation, the anterior bone wall of the maxillary sinus was revealed and a 20×20 mm entry cavity for the endoscope was created by osteotomy. Once bleeding was under control, a sterilized endoscope was inserted into the sinus through this cavity. It was used to carefully remove the foreign body. The mucosa of the maxillary sinus was thickened and the sinus was filled with pus. The foreign body was coated with granulation tissues and debris (). It had the shape of a stick made of wood (). Surgical fields were irrigated with sterile saline and a pus was completely removed. After fixing the osteotomy site with miniplate, elevated flap was closed with Monocryl (Ethicon Inc., Somerville, NJ, USA) and Vicryl suture (Ethicon Inc.). After several days of admission with intravenous antibiotics, the patient was discharged without any problem. There were no complications following the operation up to 16 months of follow-up except persist numbness (). |
A 53-year-old man presented with a gradually increasing mass located in the soft tissue of the oral cavity. The lesion was biopsied, and the pathology report showed increased infiltration of the buccal mucosa with monoclonal plasma cells. Immunochemistry demonstrated positivity for CD138, CIgA, and negativity for CD56, Cyclin D1 and CD20. Therefore, the diagnosis of soft tissue plasmacytoma was confirmed, for which he received localized radiotherapy with a total dose of 40 Gy, due to the absence of systemic disease as the bone marrow biopsy revealed the absence of neoplastic infiltration and serum and urine immunofixation were all negative.\nAfter five years, the patient noticed a painless swelling in his right testis. An ultrasound of the scrotum was performed that showed a hypoechoic mass in the right testicle with increased vascularization, and a normal-appearing left testis (Figure ). The patient subsequently underwent a right radical orchiectomy and the histopathology report showed testicular infiltration by a plasma cell neoplasm with identical immunophenotype (CD138+, CIgA+, CD56-, CD20-, Cyclin D1-) to the primary site in the oral cavity. Subsequently, the patient underwent a bone marrow biopsy that showed the absence of monoclonal plasma cell infiltration, and a PET/CT scan that was negative for reactive lesions suspicious of malignancy. In addition, serum and urine protein electrophoresis, as well as serum-free light chain assay were all within normal range, thus, excluding the presence of systemic disease, while complete blood count and full biochemical profile were normal.\nHowever, after one year, the patient presented again with painless swelling of his left testis that was attributed to disease relapse based on the imaging findings (Figure ). He refused to undergo left orchiectomy, thus he proceeded with systemic treatment based on lenalidomide, bortezomib and dexamethasone. Following 4 cycles of treatment, the patient underwent high dose melphalan with autologous stem cell transplantation. The blood tests of the patient revealed an increase in serum creatinine (1.3 mg/dl with 1mg/dl baseline level), normal calcium levels and normal complete blood count. Complete response was achieved with negative PET/CT scan and negative marrow minimal residual disease assessment. The patient continued on lenalidomide maintenance; however, six months later he was diagnosed with disease relapse in his left testicle (Figure ). A PET/CT scan was then performed, and showed a reactive lesion with an increased SUV max of 7 in the left testis, with no other loci suspicious of disease relapse. The blood analysis of the patient revealed no abnormal values. Since the patient refused surgery, he was initiated with next-line therapy, with bortezomib, cyclophosphamide and dexamethasone, he responded partially and still remains in remission (Figure ), while serum protein electrophoresis, immunofixation and serum-free light chain assay, all remain within normal levels. |
A 22-year-old male with a past medical history of pericarditis and pericardial effusion presented to the ED with the chief complaint of facial swelling, which had been present for the prior three weeks. The swelling was predominantly on the right side of his face and upper lip. He had no history of angioedema, had not started any new medications, and was not aware of an environmental exposure that immediately preceded the onset of swelling. In addition to the facial and lip swelling, the patient reported a rash of the same duration on his chest and shoulders. Additional associated symptoms included decreased exercise tolerance, exertional dyspnea, and a single episode of dark, maroon-colored stool. He denied fever, chills, myalgia, arthralgia, chest pain, abdominal pain, nausea, vomiting, odynophagia, dysphagia, and confusion. He was not aware of any sick contacts and he had not traveled recently. He reported that his family did not have a history of chronic illnesses.\nPhysical examination was significant for a blood pressure of 104/58 millimeters of mercury, a pulse of 96 beats per minute, respiratory rate of 16 breaths per minute, a temperature of 36.8° Celsius, and a pulse oximetry reading of 100% on room air. He was a thin young man who did not appear to be in distress or acutely ill. Bilateral facial edema along with edema of the upper lip was noted (). In addition, his conjunctiva, palms, and soles were notable for pallor. A petechial rash was observed on his upper chest, bilateral shoulders, tongue, and soft palate (). A malar rash was also noted (). The remainder of his examination was normal.\nHis initial ED evaluation included a chest radiograph, electrocardiogram, and laboratory studies. The results of pertinent laboratory studies are listed in the . Given his severe thrombocytopenia and anemia, thrombotic thrombocytopenic purpura (TTP) was considered and an emergent hematology consultation was obtained. A peripheral blood smear demonstrated 1–2 schistocytes per high-power field, which initially raised concern for a microangiopathic hemolytic anemia. As a result, a hemodialysis catheter was inserted and plasmapheresis was initiated while the patient was in the ED. He received a unit of packed red blood cells along with corticosteroids and was admitted to the medical intermediate care unit.\nWorkup revealed a positive immunoglobulin G (IgG) Coombs test. He also had a high titer of antinuclear acid antibody and low C3/C4 complements, indicative of an acute exacerbation of an autoimmune disease. The combination of his symptoms, ED workup, and history of pericarditis and pericardial effusion favored the diagnosis of systemic lupus erythematosus (SLE). Within 48 hours after admission, an A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) level returned with 78% activity and less than 5% inhibitor. This result was not consistent with the diagnosis of TTP, and plasma exchange was stopped. Ultimately, the hematologist diagnosed Evans syndrome as a presenting feature of SLE. |
A 52-year-old man presented to our hospital with decreased vision in both eyes due to ocular chemical burns (lime) in 2018. He had undergone lamellar keratoplasty in the left eye 2 years prior. On examination, his visual acuity (VA) in both eyes was hand motions with accurate light perception. Slit-lamp examination revealed total corneal opacification with pannus in the right eye. The left eye showed a failed large lamellar corneal button with 360° corneal vascularization. No underlying anterior chamber details were visualized. Anterior segment ultrasound (ultrasound biomicroscopy, UBM) revealed an eye with a shallow anterior chamber depth and 180° peripheral anterior synechiae. IOP estimated by digital palpation was in the normal range. Ultrasound imaging showed no obvious optic nerve cupping. After discussion with the patient, it was decided to proceed with KPro placement in the left eye in March 2018. His cataract was removed at the time of surgery by anterior vitrectomy, and an aphakic KPro was placed. At postoperative week 4, he had 10/100 vision with a cup disc ratio of 0.5 (Fig. a). At month 2, he started to develop glaucoma de novo, with a decrease in vision to counting fingers (FC) at 2 m. His IOP was uncontrolled with topical and systemic anti-glaucoma medication, and he therefore had an AGV placement approximately 3 months after the KPro implantation. The shunt tube was introduced into the anterior chamber through a tract created by a 23-gauge syringe needle. The tip of the tube is long enough to be seen through the KPro optic. At postoperative day 1, the patient’s IOP was 35 mmHg, estimated by digital palpation, and the tip of the tube was found to be embedded in the residual lens capsule (Fig. b). This situation was managed with one Nd:YAG laser pulse of 1.5 mJ to the membrane at the tube tip, which ruptured the membrane (Fig. c). The membrane retracted posteriorly, and the IOP dropped to 15 mmHg, estimated by palpation. Topical steroids were used to control the mild postoperative inflammation. To date, the IOP has remained low for 7 months. At the last follow-up, his visual acuity was FC at 1 inch with a cup disc ratio of 0.9 (Fig. d). |
A 28-week-old premature boy, with a birthweight of 1280 grams, was intubated with a 2.5 mm endotracheal tube via the nose and ventilated for severe hyaline membrane disease (HMD). After receiving two doses of surfactant, the premature neonate was successfully weaned off ventilation and extubated to nasal continuous positive airway pressure (CPAP). On day 9, his clinical course was complicated by a pulmonary hemorrhage, requiring re-intubation. He was given another dose of surfactant and stabilized on high-frequency oscillation ventilation (HFOV). The chest radiograph showed extensive bilateral pulmonary interstitial emphysema, with the left side more extensively involved in comparison to the right. A hemodynamically significant patent ductus arteriosus was treated by intravenous paracetamol. The baby's condition did not improve, and he was selectively intubated into his right main bronchus. The position of the endotracheal tube was radiologically confirmed, allowing the right lung to be oscillated while the left lung was rested. The baby was nursed on his left side for a period of 36 hours, after which the endotracheal tube was retracted into the trachea and secured in that position. The baby's ventilatory status subsequently improved, allowing for extubation on day 7 after the relapse. On day 28 of life, he presented with severe stridor, requiring re-intubation. Difficulty during intubation suggested that subglottic stenosis might be present. One week after this re-intubation a flexible bronchoscopy was performed, which revealed two major findings. The first was a Cotton grade 2 subglottic stenosis, and the other abnormality was near-complete obstruction of the bronchus intermedius. The subglottic stenosis was dilated to 5 mm with the aid of a balloon dilator (Boston scientific Mustang™ balloon dilatation catheter). The bronchus intermedius was extremely narrow, and a 2.2 mm flexible bronchoscope was not able to pass through the area of stenosis.\nAfter 2 weeks, the bronchoscopy was repeated and the subglottic region had improved to near normal in diameter. The bronchus intermedius stenosis, however, remained unchanged. A chest Computed Tomography (CT) scan was performed to determine the length of the bronchial stenosis. The CT scan confirmed that the stenosis involved a short segment and had a web like in configuration (Figure ).\nIt was decided to balloon dilate the stenosis under fluoroscopy. As the cardiac catheterization suite offered the best quality fluoroscopy, it was decided to perform the dilatation in the suite. The baby was intubated, and a guidewire was inserted into the right main bronchus under fluoroscopy. Water-soluble contrast was injected, and the position of the airway identified. A 3.5 mm coronary artery balloon catheter was inserted into the area of stenosis via the guide wire, and the position was confirmed by fluoroscopy. The balloon was inflated at 16 atmospheric pressure for 20 seconds. This was repeated for another 20 seconds before water-soluble contrast was reinjected, demonstrating significant decrease in the bronchial stenosis (Figure ). Following the procedure, the baby was ventilated for less than 24 hours. At follow-up bronchoscopy 2 weeks later, the stenosis had significantly improved, allowing a 2.8 mm flexible bronchoscope to pass comfortably through the stenotic region. The posterior part of the stenosis had completely resolved, with a small anterior shelf remaining. The baby was discharged with no known respiratory complications, and at follow-up bronchoscopy 6 weeks, after the latter dilatation procedure, the airway remained patent and the baby remained asymptomatic, with a normal chest radiograph. Follow -up bronchoscopy was done due to the risk of restenosis and the fact that the baby was from a rural area, with limited medical services. |
A 62-year-old, otherwise healthy male was referred to our hospital in November 2007 with a two-year history of a painless mass in his right external auditory canal. The patient had surgical history positive for removal of a small keratoacanthoma in his right external auditory canal in 2001. Since then he had not experienced any discomfort or symptoms until a mass was found in his right external auditory canal during a routine physical exam in 2005. As the lesion was asymptomatic at that time, the patient refused immediate resection proposed by his doctor and decided to postpone treatment until after he retired. Myringoscope revealed a mass in the deep right auditory canal near the tympanic membrane (). The mass was approximately 1 centimeter in diameter, smooth in appearance, and with no sign of bleeding. CT scan revealed a well-circumscribed, slightly enhanced solid mass in the inferior wall of the right external auditory canal without bony erosion (). Facial electromyography, vestibular function tests, and pure tone audiogram all came back normal.\nThe mass was excised through a microscope-guided surgery, during which we removed the flesh-colored mass that was adherent to the surface of tympanic membrane and preserved the canal wall and the tympanic membrane. The excised mass was then sent to pathology.\nTissues were fixed in 10% formalin, imbedded by paraffin, routinely sliced, and HE stained. To our surprise, through light microscopy, we found that the tissues demonstrated cord, trabeculae, or cluster proliferations of moderately differentiated cells in dermal and proper layers, with pleomorphic nuclei and inconspicuous nucleoli, in addition to frequent mitoses. There were also scattered cell fragments with pyknotic nuclei, indicating apoptosis. The cuticular layer was not involved (Figures and ).\nIn order to confirm the diagnosis, immunohistochemistry was carried out on paraffin sections using standard techniques with antibodies against cytokeratin (CK), epithelial membrane antigen (EMA), vimentin, neurone specific enolase (NSE), S-100 protein, smooth muscle actin (SMA), synaptophysin, glial fibrillary acidic protein (GFAP), and chromogranin (ChG). The result revealed strongly positive for CK and NSE (Figures and ) and moderate positive for vimentin, showing a pattern consistent with a neuroendocrine tumor.\nAs we did not see normal tissues on certain regions of the tumor margin, a secondary surgery was proposed for complete clearance of the tumor.\nThe patient underwent a PET scan two days after initial surgery, which did not show evidence of local lymph node involvement or metastasis. Nine days after the first surgery, the patient underwent a sleeve mastoidectomy and tympanotomy, during which we removed the tympanic membrane and performed a sleeve excision of skin and bone in the right external auditory canal. The tympanic cavity was clean. A temporoparietal fascial flap was used to reconstruct tympanic membrane and a postauricular free flap was created to cover the external auditory canal. One week after the second surgery, the patient received a chemotherapy combined of cisplatin, calcium folinate, and tegafur for a three-day course. In addition, a radiotherapy protocol was added, that a level of 200 cGy radiation was delivered each time for a total of 30 times. The patient did not experience any signs of adverse reaction throughout the entire postoperative treatment. He completed the treatment three months after his admission, and was asked for routine followup every two months in the first six months and every half a year thereafter. The total follow-up period was 2 years, no evidence of tumor recurrence was found in the patient. |
A 4-month-old entire female Persian cat was referred to our institution for chronic constipation and acute vomiting. The cat had been treated with mineral oil (PO q12h) and rectal enemas with partial response since the beginning of the clinical signs. Physical examination revealed low BCS, crackles in all lung lobes in the lung auscultation and abdominal discomfort on palpation. Thoracic radiographs showed diffuse increased soft tissue opacity within the lungs, more marked in the cranioventral aspect of the thorax, with an alveolar pattern in the right and left cranial and the right middle lung lobes. There was also a focal alveolar pattern with a perihilar distribution in the caudal and accessory lung lobes (,). Severe diffuse colon distension was noted in the abdominal radiographs. Marked scoliosis of the lumbar spine, partial fusion of the L5–S1 vertebral bodies and decreased pelvic diameter were also noted, so secondary obstructive megacolon was suspected. Rectal enema was performed and supportive treatment based on antibiotics, systemic corticosteroids, bronchodilators and IV fluids was established for the suspected aspiration pneumonia. Mineral oil was immediately discontinued. The patient improved after 2 days and was discharged with antibiotics, systemic corticosteroids, lactulose and a low-residue diet.\nTwo months later, follow-up thoracic radiographs showed an alveolar pattern (similar to that previously described) with granular mineral opacities and ‘sponge-like’ appearance of the previously affected lung lobes (,). Multiple recurrent episodes of respiratory signs were recorded during a year, which partially responded to systemic corticosteroid and bronchodilator therapy. Owing to the refractory medical management, subtotal colectomy was elected as the treatment for the obstipation 1 year later. CT of the thorax was performed prior to surgery, showing an alveolar pattern with air bronchograms in all lung lobes, with a ventral distribution in the cranial and middle lobes, and a perihilar distribution in the caudal and accessory lung lobes. Within the consolidated lobes, patchy ill-defined fat-attenuating areas and punctiform mineral-attenuating structures were present (). Three years later, neither the respiratory signs nor the constipation reappeared. |
A 61-year-old male, ex-smoker, with a history of type 1 diabetes, was referred by his general practitioner (GP) to the ear, nose and throat (ENT) department with odynophagia, dysphonia, dysphagia and anterior neck swelling. The symptoms began while he was eating a cracker and, upon swallowing, he felt a painful crack in the right side of his neck. By the following day, the patient had developed hoarseness and pain on swallowing. He attended his GP five days later after developing neck swelling. At this point, it was thought the symptoms may represent an infection or a possible underlying malignancy; the patient was prescribed a course of oral antibiotics and referred urgently to the head & neck clinic. At subsequent review in clinic, the patient reported ongoing odynophagia, dysphonia and dysphagia. Examination noted some fullness of the anterior neck. Flexible nasendoscopy (FNE) showed pooling of saliva and swelling of the right supraglottis. The patient was otherwise systemically well and managing to maintain adequate oral intake despite his symptoms. An expedited outpatient CT scan of the neck was requested to investigate for possible laryngeal malignancy. While awaiting this investigation, the patient re-presented to his GP with rapidly progressing symptoms and was referred to the ENT on-call team for same-day review. The patient had worsening dysphagia, now only managing sips of water. The anterior neck swelling had increased significantly in size and was now indurated with a central area of fluctuance. He underwent urgent inpatient contrast-enhanced CT scan of the neck (Figures , ).\nThis demonstrated a vertical fracture of the thyroid cartilage to the right of the midline, with depression of the right thyroid lamina posteriorly causing mass effect on the trachea. There was also a large multiloculated collection in the midline just anterior to the thyroid cartilage. This extended superiorly up to the hyoid bone and inferiorly over the thyroid gland. The patient was commenced on intravenous antibiotics and underwent incision and drainage of the anterior neck collection. Through a lower transverse crease incision, 50 ml of frank pus was drained from the cavity and a corrugated drain left in situ. At intubation, the anaesthetist noted the patient had started to develop supraglottic oedema, and he was therefore kept intubated and ventilated overnight on the intensive care unit. Regular intravenous dexamethasone (AAH Pharmaceuticals, London, United Kingdom) was initiated postoperatively. The patient was extubated on postoperative day 1 and started on a soft diet. However, on postoperative day 2 he reported some problems with swallowing. He was kept nil by mouth for a water-soluble contrast swallow, which showed no evidence of pharyngeal or oesophageal leak. Swallowing function subsequently improved with time. The drain was removed on day 3, and the patient was discharged on postoperative day 7 to complete a short course of oral antibiotics. At one-month follow-up, there was no recurrence of symptoms. FNE examination was normal. Ultrasound of the neck confirmed complete resolution of the collection. |
A 47-year-old female presented to the gynaecology outpatient department with a one-year history of abdominal distension. She also had a history of menorrhagia, dyspepsia, and weight loss. On clinical examination, she was noted to have massive ascites with a palpable lower central abdominal mass. On vaginal examination, the uterus could not be felt separately from the mass. Her CA-125 level was 477.1 IU/mL (normal < 30 IU/mL). The possibility of an ovarian tumor or uterine sarcoma was raised clinically.\nUltrasound scan revealed a large mass posterior to the uterus along with massive ascites. CT scan performed showed a large predominantly solid pelvic mass demonstrating loss of fat plane with the uterine myometrium. The ovaries were not localized. There was massive ascites along with minimal left-sided pleural effusion. The diagnostic possibilities considered were those of a solid ovarian neoplasm infiltrating the uterus and a pedunculated uterine fibroid with sarcomatous change. The cytological examination of her ascitic fluid revealed no malignant cells.\nThe patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. About ten liters of serous ascitic fluid was drained. Preoperatively, a large irregular tumor with hemorrhage and degeneration was seen arising from the uterine isthmus.\nGrossly, the specimen with the attached tumor weighed 1.5 kgms. The uterus and cervix together measured 9 × 6 × 4 cm. A large hemorrhagic mass measuring 18 × 15 × 8 cm was seen to arise from the lower posterior part of the uterus (). On sectioning, the mass was noted to be in continuity with the myometrium. The cut surface of the mass was whitish and whorled () with hemorrhagic areas in the periphery. Some dilated blood vessels with clots in their lumina were also present. The endometrium and the rest of the myometrium appeared unremarkable. Both ovaries and Fallopian tubes were normal. Postoperatively, the ascites resolved dramatically and her CA-125 levels normalized.\nHistological examination of the tumor showed a moderately cellular neoplasm composed of interlacing fascicles of spindle cells with interspersed abundant thick muscular walled vessels (). Bland spindle cells were seen swirling around some of the vessels. In areas, the spindle cells showed marked atypia with bizarre hyperchromatic nuclei () and some multinucleate giant cells. However, extensive sampling revealed no excess or atypical mitotic activity, coagulative tumor cell necrosis, or hypercellularity. There was subcapsular and intratumoral hemorrhage along with foci of hyalinisation and edema. The endometrium, bilateral tubes, and ovaries revealed no significant pathology.\nOn immunohistochemistry, the tumor cells showed positivity for smooth muscle actin (SMA) () and desmin (). HMB-45 negativity () ruled out the possibility of a perivascular epithelioid cell tumor (PEComa). The Ki-67 proliferation index () was less than 1%.\nThe patient has had no complications or further symptoms on followup for 6 months post-op and to date. |
A 52-year-old female, who is a known case of diabetes mellitus on oral hypoglycemics, presented to the emergency department complaining of diffuse on and off abdominal pain, nausea, vomiting, and obstipation for 1 week.\nThe patient reported constipation for 1 month before it progressed to obstipation and unintentional 8 kg weigh loss over 3 months. She gave a history of total abdominal hysterectomy in 2015 due to uterine fibroids. The patient had no personal risk factors nor family history of malignancies.\nAbdominal examination was unremarkable while the anorectal examination showed a circumferential rectal mass that is 8-9 cm from the anal verge. Initial laboratory investigations and abdominal X-ray were unremarkable.\nThe patient underwent an urgent colonoscopy which showed a malignant-looking lesion with severe stenosis at the proximal rectum 10 cm from the anal verge (). A biopsy was taken and revealed few signet ring cells with CK7 histochemical stains being positive while CK20 and CDX2 were negative (Figures and ). A performed gastroscopy was unremarkable.\nFurther investigation with CT of the chest, abdomen, and pelvis; MRI of the pelvis; and whole body PET/CT was carried out.\nThe chest CT showed multiple scattered focal lytic and sclerotic lower cervical, dorsal vertebral, rib cage, and sternal metastatic deposits, while the abdomen and pelvis CT with MRI of the pelvis revealed a circumferential heterogeneously enhancing rectal mass lesion (about 7 cm in length) located 7 cm above the anal verge, with a maximum wall thickening of about 1.75 cm, resulting in rectal luminal narrowing with multiple small noncalcific mesorectal, right internal, and right external iliac lymph nodes ().\nThe whole body PET/CT showed the same findings of the CT and MRI previously done (rectal mass and multiple bony metastatic deposits) in addition to suspicious right breast upper inner quadrant lesion ().\nIn view of the almost complete bowel obstruction, the patient underwent laparoscopic diverting loop ileostomy to relieve the symptoms, which also showed disseminated peritoneal metastatic deposits () that were biopsied and turned out to be metastatic carcinoma, showing sheets of pleomorphic malignant signet ring-like cells on histopathology. CK7 and estrogen receptors (ER) were positive (Figures and ) and CK20, GCDFP-15, E-cadherin, WT-1, and Her-2 Neu were negative on immunohistochemistry staining.\nThe patient was further investigated for the right breast mass with ultrasonography and mammogram of the breast that showed 2 ill-defined, irregular lesions in the right breast ().\nAn ultrasound-guided core biopsy of the right breast 10 o'clock lesion was taken (), and the histopathology report came as invasive lobular carcinoma, grade III.\nThe immunohistochemistry and receptor status were positive for CK7—similar to the rectal mass results—ER receptor, and PR receptor, and it was negative for CK20, E-cadherin, and Her-2 Neu receptor, with a tumor proliferative index (by Ki-67) of 1%.\nThe final diagnosis was right breast invasive lobular carcinoma, grade III, T1NxM1, ER +ve, PR +ve, Her-2 Neu -ve, Ki-67 1%, luminal A subtype.\nThe patient's case was discussed in the national tumor board meeting and was planned for palliative chemotherapy and hormonal therapy. |
The patient was a 61-year-old man with multiple left rib fractures (1–6 ribs), left pneumothorax, left lung contusion, and left thoracic subcutaneous emphysema due to a fall injury. The examination showed a partial depression in the left front rib and abnormal breathing (see Fig. ).\nAdmission chest CT examination: 1–6 rib fractures on the left side (of which 3, 4 ribs are long comminuted fractures (see Fig. )); left pneumothorax, left traumatic wet lung; a small amount of liquid pneumothorax on the left side.\nPatient was given early chest straps, multiparametric monitoring, analgesia, and oxygen therapy. The chest pain was still severe. The visual analogue scale scored 7–8 points for the pain at rest and 9 points for the cough.\nPhysical examination revealed that the left chest wall was recessed and abnormally breathed. The CT scan of the rib showed a long comminuted fracture of 3 and 4 ribs. The key to successful operation was the reduction and fixation of these two rib fractures. A preoperative CT scan was performed to reconstruct the 3D model based on the scan results (see Fig. ), and 3D printing technology was used to prepare 3 and 4 rib models (see Fig. ). The three D print models of each fracture segment of the two ribs were adherently reconstructed.\nThe two rib metal plates were separately shaped according to the reconstruction model (see Figs. and ).\nThe patient is scheduled to have a open reduction and internal fixation of 3–6 rib fracture. After general anesthesia, right side lying position, small incision about 8 cm was performed under the edge of 4th rib underarm. The skin was sequentially incised and the subcutaneous tissue was freed layer by layer. The front of the latissimus dorsi muscle and the anterior serratus were exposed. The tunnel was established on the 3rd and 4th rib surfaces from the back of the chest and small muscles to the back of the scapula. The special long hooks lifted the scapula and exposed the scapular operation space. With assistance of endoscope, the electrocautery is useful to expose 3 cm outside the outermost fracture lines of the 3 and 4 ribs. The locking plate was molded on the surface of the third rib before operation, and the broken end of the non-fracture at the anterior and posterior portions of the third rib was well fitted. The distance between the two ends of the metal bone plate exceeded the fracture line to 3 nail holes distance. Under the thoracoscope, the metal plate and the ribs were temporarily fixed with long-angled forceps. The MIPO system was used to drill the holes. Two screws were implanted and locked at both ends to firmly fix the metal plate. In turn, each fracture segment was reset and drilled and secured to a metal plate. The fourth rib is fixed in the same way. Intraoperative image (Figs. , and ). 5, 6 rib fractures given to fix the ribs, not the content of this article, not elaborated. Sufficient to stop the bleeding, the wound was given to leave a negative pressure drainage tube. After a routine thoracoscopic probe of the chest cavity, a closed thoracic drainage tube was placed posterior to the 7th intercostal space and the incision was closed layer by layer. After the chest wall is well-shaped. Three days after surgery review the map (Fig. ). |
A 59-year-old male presented to the general practitioner because of painful ribs. Imaging, performed to rule out pulmonary embolism at the Emergency Department, showed no cause for his symptoms, but accidentally revealed enlarged lymph nodes in the neck. Histopathology obtained from a lymph node biopsy indicated a metastasis of an poorly differentiated adenocarcinoma. The node was almost entirely made up of an epithelial tumor which formed tubular structures. The tumor cells had ample eosinophilic cytoplasm and enlarged polymorphic anisochromatic and elucidated nuclei often with a prominent eosinophilic nucleolus. Both mitoses and apoptosis were observed. At the tumor border vascular invasion was present. Additional imaging examinations revealed a large sinonasal process (). Subsequently, the patient was diagnosed with a primary SDC originating from the sinonasal cavity based on additional clinical information, histomorphology, and immunohistochemistry. Due to cervical and mediastinal lymph node metastases (T3N2bM0 disease), the patient was referred to our hospital, a tertiary referral center for salivary gland cancer, to discuss systemic treatment options. The tumor cells were positive for androgen receptor (AR) (see ). Human epidermal growth factor receptor 2 (HER2neu) status was assessed in accordance with the American Society of Clinical Oncology/College of American Pathologists (ASCO/CAP) guidelines for the evaluation of breast cancer (). Immunohistochemistry (IHC) was inconclusive; IHC score: 2+, therefore fluorescence in situ hybridization (FISH) was performed which indicated no amplification, resulting in a negative HER2neu status. Additional molecular analysis was performed on a lymph node metastasis to detect other potential actionable targets for future systemic treatment options. The assay used for molecular analysis was the TruSight Oncology 500 (Illumina), which is a next-generation sequencing assay that enables comprehensive genomic profiling of 532 genes, furthermore it measures microsatellite instability and tumor mutational burden. The molecular analysis showed no clinically relevant mutations, there was no microsatellite instability, and the total tumor mutational burden was 1.6 mutations per megabase.\nAt the first outpatient visit, the patient asked if the occurrence of his SDC could be somehow related to direct contact with Cr(VI) in work-related exposures in the past. A detailed occupational history revealed that the patient had been working as inspector of construction cranes and harbor cranes since 1984 up until the occurrence of his SDC disease. Initially this inspection work did not involve any use of chemicals until, in 2012, he was asked to perform inspections at three military air bases in the Netherlands over a period of 2–3 months a year. This liquid penetrant inspection was performed to detect hairline cracks in welds of military equipment. For this task, the coating was completely removed by mechanical grinding. The topcoat known as chemical agent resistant coating (CARC) contains the toxic hexamethylene diisocyanate (HDI) and is typically adhered to the metal surface with a chromic acid-containing conversion coat and primer coat containing a chromate pigment. Both are Cr(VI)-based chemical components that generate airborne particles when mechanically abrased (). According to the patient, this procedure resulted in visible dust clouds that caused work clothes to become contaminated. No respiratory protective equipment was used and protective gloves were not always used. During breaks the worker cleaned hands but did not change clothes. When the patient blew his nose after these activities this resulted in a dusty nose secretion matching the color of the paint, indicating a clear direct exposure of the nasal mucosa to the paint grinded to dust. As of 2015, the use of the destructive method was discontinued and replaced by an alternative non-destructive inspection method.\nApart from Cr(VI) exposure, the patient may have come into contact with other chemical substances during a previous occupation (). From 1978 till 1984 he worked as a plumber. Furthermore, the patient had a history of smoking and alcohol consumption. The patient started smoking shag from the age of 16 until 58, with an average of 50 gram of tobacco per week which results in a total of 31 pack years. He started to use alcoholic beverages at the age of 16 and consumes ~24 units of alcohol per week.\nThe patient has been treated with combined androgen deprivation therapy, because of androgen receptor expression. Previous research indicated that this therapy might alter the prognosis of patients. Without therapy patients with advanced disease had a poor overall survival of 5 months, while with androgen deprivation therapy the overall survival was 17 months (). Therapy consists of the combination of a luteinizing hormone-releasing hormone (LHRH) agonist, goserelin subcutaneous 10.8 mg every 12 weeks, and an androgen receptor antagonist, bicalutamide tablet 50 mg once daily. Evaluation CT scans performed 3 months after start of therapy showed a partial response according to the RECIST 1.1 criteria for tumor response evaluation (). The most recent evaluation, 9 months after initiation of treatment, showed an ongoing partial response. |
A 63-year-old female patient with diabetes mellitus presented to the emergency department with the complaints of a painful swelling over the right lower abdominal pain for 4 days. It was insidious in onset and was associated with fever, nausea and reduced appetite. She did not have jaundice and hematemesis. However, she gave history of intermittent constipation with occasional bleeding per rectum (PR), abdominal discomfort and sense of incomplete evacuation after defecation for past 6 months. She had undergone an open laparoscopic cholecystectomy 1 year ago for symptomatic gall stone disease and her postoperative period was uneventful. She also gave history of significant weight loss for past 4 months.\nOn her physical examination, she was pale and malnourished. She was afebrile, tachycardia and tachypnea were present, and she was in acute distress due to pain. On examination of the abdomen, there was a tender fluctuant area over the right lower abdomen, predominantly in the subcutaneous and muscular plane. It measured around 6 × 8 cm with the erythema of the skin and surrounding induration (). There was no sinus or discharge from the skin. The rest of the abdomen was soft and nontender. On rectal examination, there was normal tone and finger stained with stool with no palpable mass noted. Laboratory investigation revealed an elevated white blood cell count of 16.5 (normal range 4.0–10.8 × 103 per liter) with neutrophils, hemoglobin was below normal limit of 7 mg/dl (normal range 12–16 g/dl) and albumin 2.6 (normal range 3.4–4.8 g/dl). The level of carcinoembryonic antigen was highly elevated.\nAfter resuscitation with intravenous fluids, administration of broad-spectrum intravenous antibiotics and intravenous analgesics, a contrast-enhanced computed tomography (CECT) scan was done to further characterize the lesion. CECT of abdomen showed large abscess extending from the subcutaneous and intermuscular planes over the right iliac fossa and breaching the peritoneum. It showed heterogeneous wall thickening of the proximal part of the ascending colon with loss of mural stratification and causing luminal narrowing, measuring 4.8 × 5.1 × 4.5 cm with surrounding inflammatory changes with adjacent well-defined collection 11.3 × 4.5 × 10.9 cm and few air pockets and infiltrating the right lower abdominal wall (). Pus aspirated from the right abdominal swelling and was sent for culture and sensitivity. Patient was taken up for diagnostic laparoscopy and proceeded.\nDuring laparoscopy, a right-sided colonic mass adherent to the lateral abdominal wall with infiltration of the subcutaneous tissue by the tumor was noted ().\nAscending colonic cancer complicated by an anterior abdominal wall abscess was diagnosed. Exploratory laparotomy was performed and a right colonic mass ~9 × 8 cm in diameter was found, which was widely adherent to the lateral abdominal wall, with extension through a fistulous track into the subcutaneous tissue (). There was no distant metastasis. Extended right hemicolectomy and lymph node dissection was performed with limited resection (en bloc) of the involved part of the lateral abdominal wall using a closure technique.\nThe patient had an uneventful postoperative recovery. The patient was discharged on the 14th postoperative day with a healthy wound. The patient was referred to Department of Medical Oncology for further adjuvant therapy. Consent was obtained from the patient for her case to be reported. |
This otherwise healthy 57 year old woman was referred to an otolaryngologist for evaluation of chronic ear infections and recurrent sinusitis. The otolaryngologist diagnosed chronic sinusitis based on the clinical exam and CT imaging. The planned surgery included bilateral endoscopic ethmoidectomy, bilateral middle meatus antrostomy and left inferior turbinate cautery.\nDuring the right posterior ethmoid exploration, a 2 mm tissue fragment was removed for histopathological analysis. Cerebrospinal fluid was observed leaking from the surgical biopsy site. The apparent opening in the cribriform plate was sealed with gelfoam and the remainder of the procedure completed.\nWith concern for a CSF leak an unenhanced CT was obtained post-operatively revealing intracranial penetration and hemorrhage (Figure ). The 2 mm tissue sample obtained during the procedure was identified as normal cerebral white matter. In the recovery room, the patient awakened without difficulty in "satisfactory condition with intact vision."\nNeurosurgical consultation one day after surgery described slight drowsiness and minimal left upper extremity drift. She was empirically started on fosphenytoin and antibiotics. The following day her drift had resolved and she appeared less lethargic.\nThe remainder of the hospitalization was uncomplicated and she was discharged seven days later after completing her course of antibiotics. She took valproic acid for seizure prophylaxis for several days at home, but this was discontinued due to adverse effects.\nAn MRI scan two weeks later (Figure ) documented evolution of the hemorrhage. Restricted diffusion along the course of the injury (image not shown) indicated tissue infarction. A brain MRI three months after surgery (Figure ) revealed resolution of the hemorrhage with residual encephalomalacia along the path of injury involving the right caudate, medial internal capsule and anterior right thalamus.\nOver the ensuing months the family reported that the patient's personality had changed. They described her as apathetic, mildly depressed, emotionally labile and irritable. She herself complained of having no energy and seemed to have lost interest in life and those activities that had previously stimulated her. Prior to the surgery she had worked full time as an aide in a center for learning disabled children plus an additional 20 hours weekly as an aide in a home health agency. She also was involved extensively with her church and community activities. After recovering from the surgery she was unable to return to work. She would sit at home and do nothing. She did not even wish to drive her car any longer. She also felt that her cognitive abilities had declined significantly. She complained of difficulty with attention and concentration. Although she reported no decline in memory, her family did notice that she was more forgetful.\nThe patient was evaluated 6 months after the injury by a rehabilitation neuropsychiatrist who found that she was apathetic, had "blunted and constricted affect" and specifically showed "no evidence of neuro-vegetative symptoms of depression." Based on this assessment, the neuropsychiatrist actually discontinued Prozac treatment started by other physicians and initiated therapy instead with amantadine. The same clinician continued to interact with her until 28 months after surgery and never observed any clinically significant depression. More extensive neuropsychological testing was performed at a different facility 32 months after surgery and concluded again, that the patient's primary cause of cognitive impairment and resultant loss of functionality resulted from an abulic syndrome and not depression. While the patient scored for mild to moderate depression on the Beck Depression Inventory-11, most of the score was based on symptoms that coexist with abulia such as decreased interest in people or activities, loss of pleasure, decreased energy, concentration, motivation and initiative. However, the patient actually scored low on symptoms that are exclusive to depression such as feelings of sadness, worthlessness, guilt and hopelessness. Her apathy, lack of motivation and initiative were the changes that dramatically stood out. Thus it was possible to distinguish clearly between abulia and depression. The ultimate conclusion was that our patient had primary abulia with some depression secondary to her self-perceived change in personality. This fit well with her own assessment. She described her mood as a response to the different person she had become and her inability to live life as she was once able. These neuropsychological tests also revealed other complex functions affected by the injury, namely "executive dysfunction and mild disinhibition." The abulia responded partially to amantadine, 100 mg daily, however, the patient reported that if she missed a few doses her difficulties with initiation and motivation would soon return. |
A 67-year-old, right leg dominant male (1.63 m; 61.4 kg) underwent physical assessment pertaining to reports of unilateral left knee pain and muscle weakness. He first experienced moderate left knee pain five months previously when attempting to carry a heavy object up a flight of stairs. There were no reports of previous injuries to the feet, knees, hips, or back that required medical attention. The pain was self-managed using anti-inflammatory medication and glucosamine with chondroitin, however he still reported a moderate (4/10) amount of knee pain on most days. He was able to ambulate unaided, but occasionally used a cane when walking longer distances. Radiographs confirmed the presence of mild OA in the left knee with definite medial joint space narrowing and one small medial tibial osteophyte (Kellgren and Lawrence grade 2 []). Radiographs of the right knee were unremarkable. Clinical examination did not reveal any ligamentous laxity or patellofemoral involvement, or any observable difference in knee circumference or thigh muscle girth. In accordance with established and institutional safety guidelines for the use of TMS, the participant was screened for history of seizure, medication use, metal implants in the brain or head, and history of neurological diagnosis prior to inclusion in this study [, ].\nAssessment of neuromuscular function included measurement of CS excitability (as quantified using MEPs) and isometric muscle strength. CS excitability was assessed using TMS with a 70 mm double cone coil (Magstim Super Rapid, Magstim Company, Ltd.). Surface bipolar Ag-AgCl electrodes (10 mm diameter) were placed 25 mm apart on the belly of the left and right rectus femoris (RF), and a ground electrode was placed on the patella. The skin was shaved, cleaned, and abraded prior to application of the electrodes to reduce electrical impedance. During stimulation, the coil trajectory that elicited the best MEP for the RF for each leg was marked using BrainSight software (Rogue, Montreal) and stored for future reference, and the marker for the RF trajectory was placed on a template MRI brain image. To reduce both intra- and intersession variability in the application of TMS, the same reference brain and RF trajectory marker were used in each TMS mapping time point. Active motor threshold (AMT) was determined while the participant maintained a low level contraction and defined as the lowest stimulator intensity that generated 5 MEPs across 10 trials, each with a peak-to-peak amplitude () of at least 200 μV. MEPs were then elicited from the motor cortex at stimulus intensities of 105%, 115%, 120%, 125%, 130%, 135%, and 145% AMT. A stimulus response (motor recruitment) curve [] was generated for each limb by calculating the average peak MEP magnitude from ten consecutive stimuli at each intensity, with approximately 1 sec to 4 sec between each stimulus. To account for the possibility of baseline variability, data from each stimulus intensity were normalized to 105% AMT for each stimulation session. Linear regression was then used to compute the slope parameter for the curve, as suggested previously [, ]. This procedure was repeated immediately following a 30-minute exercise session, using the same electrode placement and coil trajectory. This exercise session was intended to familiarize the participant with a home-based exercise program and included unilateral (affected limb only) open-kinetic chain knee extension in sitting, knee flexion in standing, and hip abduction in standing and side lying. Closed kinetic chain exercises include half-squats against a wall, forward lunges, and seated leg presses (not part of the home program due to equipment requirements), all involving both limbs. Resistance, when applicable, was provided from cuff weights attached around the ankle and chosen based on the ability to perform 10 repetitions with moderate difficulty.\nOn a separate visit to the laboratory within the same week as the assessment of CS excitability, maximal isometric muscle strength was measured while the participant was seated in an isokinetic dynamometer (Biodex System 4; Biodex Medical Systems Inc., Shirley, NY). Maximal isometric knee extension torque from each limb was measured with the hip in 90° of flexion and the knee in 30° of flexion. After a warm-up trial for the purposes of equipment familiarization, three isometric trials of five-second duration were completed for each limb. The maximum torque exerted onto the dynamometer from the three trials was identified and normalized to body mass (Nm/kg).\nFollowing baseline muscle strength testing, the participant was prescribed the series of six muscle-strengthening exercises described above and instructed to complete each exercise for 3 sets of 10 repetitions at home on at least four days per week over 8 weeks. Ankle cuff weights were provided for resistance to the seated knee extension, side lying hip abduction, as well as the standing knee flexion, and hip abduction. The participant met with a physiotherapist four times over this period to ensure proper exercise performance and safe progression of resistance, and to perform additional exercises (e.g., seated leg press and standing hip adduction with cable resistance) not possible as part of the home program. CS excitability (using the same electrode placement and coil trajectory from baseline, AMT was determined again) and muscle strength were assessed again at the end of the 8-week muscle strengthening intervention, using the same techniques with the isokinetic dynamometer and outcomes previously described. Finally, overall average knee pain was assessed before and after the strengthening intervention, using an 11-point numerical rating scale with 0 representing “no pain” and 10 representing “worst pain imaginable”.\nBetween-limb differences in MEP amplitude were evident across all stimulus intensities at baseline (). In general, RF MEPs were higher in the unaffected limb compared to the affected limb across all stimulus intensities prior to 30 minutes of exercise. Following the 30-minute exercise familiarization session, MEP amplitudes in both limbs increased at all stimulus intensities, with the exception of 120% and 145% AMT in the unaffected limb. Differences between limbs in the postexercise session were small at all stimulus intensities. Following 8 weeks of lower limb muscle strengthening, MEP amplitudes in the affected limb increased, compared to baseline at all stimulus intensities except 115% of AMT (). Finally, overall knee pain was reduced from 4/10 to 0/10, and maximal isometric quadriceps torque increased from 1.25 Nm/kg to 1.60 Nm/kg following the 8-week strengthening intervention. |
After falling off a mountain bike down an incline into some brush, a 49-year-old male mountain biker presented to an outside ED with normal vitals, severe vertigo, nausea, intractable vomiting, profound hearing loss, and tinnitus. A CT was performed, which showed opacification of the ear canal, but did not comment on any abnormalities of the inner ear. The patient was transferred to our facility for further management. On examination, there was a spontaneous right-beating nystagmus and the facial nerve was intact. A tree twig was embedded in the left external auditory canal, obscuring visualization of the tympanic membrane.\nTemporal bone CT demonstrated a linear foreign body projecting from the external auditory canal to the oval window, and an additional, separate small foreign body projecting into the vestibule. Presence of extensive intralabyrinthine air was detected radiographically (). On axial view, air bubbles were seen in the vestibule, posterior semicircular canal, and the scala vestibuli compartment of the cochlear basal turn, as well as in the lateral and superior semicircular canals (). Preoperative audiogram conducted at bedside revealed normal hearing on the right side and moderate-to-severe mixed hearing loss on the left.\nThe patient was diagnosed with a traumatic PLF with extensive pneumolabyrinth due to penetrating temporal bone injury and was taken urgently to the operating room less than one day after his inciting injury. A three- centimeter tree twig was lodged in the ear canal and found to be penetrating the tympanic membrane. Postauricular approach included mastoidectomy and intraoperative assessment of the middle ear ossicles and extent of injury. The long process of the incus was dislocated but still attached to the malleus, the stapes was deeply embedded into the vestibule, and the oval window was completely open but covered by blood clot. All penetrating foreign bodies were extracted. Temporalis fascia was used to seal the oval window and a stapes prosthesis was placed. The tympanic membrane perforation was repaired.\nPostoperatively, the patient had rapid and significant improvement of his vertigo. On physical examination, there was minimal spontaneous nystagmus. A four-week postoperative audiogram revealed a mild-to-moderate mixed hearing loss in the left ear with continued improvement at six months. |
This report involves a 59-year-old female who presented with postmenopausal uterine bleeding, increased abdominal girth, and discomfort. The patient was a para 1, postmenopausal Afro-Curaçaoan woman who was referred to our department after presenting with postmenopausal bleeding (PMB), abdominal distention for one month, and worsened abdominal pain for two months. The patient’s symptoms were associated with lower back pain, swelling of her feet, constipation with excessive straining, dysuria, urinary frequency, and urgency. The patient also complained of painful intercourse and postcoital bleeding. She denied nausea, vomiting, dizziness, or diarrhea. She denied a history of liver disease or similar episodes in the past. Her past medical history is significant for a single cerebrovascular accident nine years ago and epilepsy. The patient had one cesarean section at the age of 33 years, cataract surgery 11 years prior, and cholecystectomy 6 years before the current presentation. She reported that her menarche was at the age of 11 years, with regular menstrual cycles lasting for five days. The patient had a pap smear one year before the current visit, and the results were normal. The patient did not take any regular medications, including hormonal therapy. She had no family history of the bowel, breast, ovarian, and uterine cancer. Physical examination revealed abdominal distension with a large mass of 35 cm above the pubic symphysis. There was no fluid wave thrill, no focal tenderness, rebound, or guarding of the abdomen upon examination. Normal cervix with vaginal atrophy was noted. Her blood work was unremarkable. A transabdominal ultrasound scan (USS) showed a cyst with no solid components with minimal free intraperitoneal fluid (Figure ).\nAbdominal CT scan confirmed a large cystic mass, measuring 25 × 25 cm, causing compression around the surrounding organs (Figure ).\nEndometrial dilation and curettage revealed a moderate amount of tissue that was sent for pathological evaluation, and the result showed polypoid endometrium with no hyperplasia and no malignancy.\nOne month later, the exploration of the abdomen through a small vertical incision revealed a massive right ovarian cyst, smooth and gray in color, occupying the entire pelvic and abdominal cavities up to the xiphoid process. No pelvic or para-aortic nodularity was palpated, and no ascites were noted. Approximately 2,600 ml of clear fluid was aspirated from the ovarian cyst followed by a total abdominal hysterectomy with bilateral salpingo-oophorectomy and right ovarian cystectomy (Figure ). The tissue was sent for pathological evaluation and was subsequently found to have mucinous cystadenoma and uterine leiomyoma with adenomyosis. Surgical drainage with the removal of the cyst from the abdominal cavity and total hysterectomy with bilateral salpingo-oophorectomy resulted in complete resolution of the patient's symptoms. |
A 22-year-old woman was admitted to our hospital with a history of intermittent colicky right hypochondrial pain not relieved by any medications for the past 3 months. Initially, she was given proton pump inhibitors, but her pain was not relieved. She had no other symptoms. Her past medical and family histories were not significant. The result of her clinical examination was normal. Upper gastrointestinal (GI) endoscopy showed globular swelling at the medial wall of D2 (Fig. ). The ampulla was situated at the summit of swelling.\nComputed tomography (CT) with oral contrast agent showed dilation of the intramural part of the common bile duct (CBD). A cyst of size 2.4 × 2.3 cm was noted in the second part of the duodenum (Fig. ). On the basis of the above findings, it was reported as type 3 choledochal cyst. The patient was further investigated with magnetic resonance imaging, which showed dilation of the intramural part of the distal CBD. A 2.4 × 2.3 cm cyst was noted in the ampullary region, again consistent with a type 3 choledochal cyst (Fig. ). Then the patient was planned for endotherapy, but owing to difficulty, it could not be negotiated into the ampulla. Finally, the patient was referred to the surgical gastroenterology department for operative management. After multidisciplinary team discussion, an abdominal ultrasonogram (USG) was done, which showed a clear thick wall cyst measuring 4.6 × 2.6 cm between the second part of the duodenum and the head of the pancreas with gut signature (Fig. ). Opening of the CBD into the duodenum was seen just distal to the cystic lesion. There was active peristalsis noted all around the cyst, suggestive of duodenal duplication cyst.\nAfter the diagnosis was confirmed, the patient was taken for elective laparotomy. Intraoperative findings were a 5 × 3-cm cyst over the medial wall of the duodenum extending proximal and distal to the ampulla and displacing it posteriorly (Fig. ). The cyst was communicating with the ampulla by a small opening in its medial wall (Fig. ). So, cyst secretions were drained via the ampulla, which avoided retention of cystic fluid. Part of the cyst wall was shared with duodenal musculature. Upon needle aspiration, the cyst wall contained bile due to communication with the ampulla. The duodenum was Kocherized, and a longitudinal duodenotomy was made for about 5 cm. Partial excision of the cyst was done (Fig. ) because it was closely associated with the ampulla. Marsupialization of the remaining cyst wall was done. The duodenotomy was closed horizontally. Feeding jejunostomy (FJ) was done using a modified Witzel method. A flank drain was kept in place. The postoperative period was uneventful. The patient was started on oral medications on the third day after surgery. The FJ tube was removed after 6 weeks. Histopathology showed the cyst wall was lined by duodenal mucosal epithelium with focal areas of ulceration and composed of tall columnar cells with goblet cells on either side of a common (shared) muscular layer. The submucosa showed lymphoid aggregates with Brunner glands. The common muscular layer showed congested vessels. Histopathological features were suggestive of duodenal duplication cyst (Fig. ). At her 9-month follow-up visit, the patient had no complaints. |
A sixty-one-year-old man with a significant two-year past medical history of bilateral lower extremity weakness and a left parasellar meningioma presented with anorexia, intermittent dysphagia, and early satiety with intermittent nausea, vomiting, and diarrhea from which he had been suffering for the past three months. During this time, he experienced a 20-pound unintentional weight loss. He was not taking any medications known to cause peripheral neuropathy; additionally, he reported a 20 pack/year smoking history and denied alcohol or illicit substance use.\nPrior to admission, in January 2016, he had undergone extensive workup for similar symptoms of abdominal pain, nausea, vomiting, and dysphagia. The resulting workup included a CT of the chest, abdomen, and pelvis that demonstrated a fatty infiltrative change in the liver and a kidney, ureter, and bladder X-ray that showed a nonobstructive abdominal gas pattern with no dilated bowel loops or masses identified. An esophagogastroduodenoscopy done in March 2016 demonstrated dysphagia likely secondary to underlying neurologic issues, along with a small hiatal hernia, and an MRI that June revealed an unchanged left middle cranial fossa mass consistent with meningioma.\nUpon presentation in July 2016, our patient reported bilateral lower extremity pain present over the past six months, which had been attributed to distal axonal motor neuropathy by electromyogram and nerve conduction studies by his outpatient neurologist. He characterized his pain as alternating between throbbing or shooting up his legs, and that he had been started on pregabalin six months prior with some amelioration of his symptoms. Oxcarbazepine had been recently added to his medication, with some results. He also reported weakness in both his feet since receiving radiation therapy for meningioma two years prior, requiring the use of a walker to ambulate.\nIn the emergency department, his creatinine was elevated to 1.31 mg/dL from a baseline of 0.9 mg/dL, and troponins were elevated threefold with a normal EKG. His physical exam was significant for splenomegaly and small-volume perihepatic ascites contributing to abdominal distension. He was admitted for acute kidney injury.\nA CT of his abdomen and pelvis was obtained, revealing small-volume peri-hepatic ascites, splenomegaly (Figure ), and incidentally-noted expansile lesions of the left ischium, acetabulum, and ilium (Figure ).\nDue to these findings and noted bilateral lower extremity neuropathic weakness, we obtained further testing to corroborate our suspicions of a plasma cell disorder. Laboratory data were significant for monoclonal gammopathy (bi-clonal IgG lambda on serum protein electrophoresis), as well as marked endocrinopathy: adrenal insufficiency with a low AM cortisol of 3.6 ug/dL; hypothyroidism with a TSH of 3.76 uIU/mL and low free thyroxine (T4) of 0.45 ng/dL; and hypogonadism with low free testosterone of 0.3 pg/mL. His VEGF levels were elevated to 377 pg/mL. Bone marrow core biopsy confirmed our suspicions of a plasma cell dyscrasia; the core biopsy was 50% cellular, with plasma cells comprising 7% of the smear cellularity, and of which approximately half were noted to be immunophenotypically abnormal.\nGiven his polyneuropathy, organomegaly, endocrinopathy, IgG lambda, skin changes (leukonychia and rubor in his extremities), and extravascular fluid overload, he was diagnosed with POEMS syndrome. He was initiated on lenalidomide and dexamethasone following diagnosis, and eventually received an autologous stem cell bone marrow transplant. |
A 1-year-old male toddler arrived in emergency services with burns on the right hand. A detailed history by his mother revealed the cause of burn to be scalds due to accidental hand immersion in a bucket of hot water at home. Immediately after the burn, some antibiotic ointment was applied over the affected hand and he was rushed to a nearby primary health care facility. In the dispensary after a formal survey, some antibiotic ointment was applied over the burnt area and dressed with sterile cotton gauze.\nOn presentation at our emergency, the child was restless and febrile with a temperature of 100.8 Fahrenheit (F). A quick general survey was done, and tetanus immunization was administered. A through local examination revealed second degree deep dermal burn on the right hand involving both the dorsal [] and palmar aspects []. Under Ketamine anesthesia, the affected hand was washed thoroughly with normal saline and all blisters debrided. No fasciotomy was deemed necessary. Silver sulfadiazine cream (SSD) 1% was applied evenly over the burnt hand and dressing done with sterile cotton gauze pads and bandaged. Oral antibiotic and paracetamol were given after the child was fully conscious.\nThe next day the child was again restless. He had two spikes of fever, highest being 102.4 F. Intravenous fluids and precautionary antibiotics were started. The dressing was soaked, and we observed dirt over the soaked area probably due to surface contact with surroundings by the anxious child. The child was again taken to the operating room for dressing change. After removing the soaked dressing, the SSD cream was washed off with normal saline. The entire area was again covered with SSD cream []. This time the wound with the antibiotic on it was covered a No. 6 latex sterile surgical glove. [] The subsequent pictures of wound healing are shown from Figures and .\nSubsequent dressing changes were done on alternate days. We recorded normal temperatures of the child after second dressing change. The child allowed dressing change without any anesthesia after fourth dressing. The child was discharged from the hospital on the 12th day. Regular hand physiotherapy was advised to the parents during the day and malleable splint [] was given for the night to avoid any post burn contracture formation. The child has been on regular follow up for two months now. The progress of wound healing has been shown at day 11 on both palmar [] and dorsal aspect []. All his wounds have healed satisfactorily on palmar aspect [] and dorsal aspect [] at day 18. Parents are continuing massage therapy and splintage as suggested to them. |
A 41-year-old Australian man was presented to Department of Plastic and Reconstructive Surgery, Dong-Kang General Hospital, with an open wound on the right lateral foot between lateral malleolus and plantar area of the heel, and the following injury was caused by slipping down on the wet floor about a month previously.\nThe patient has been diagnosed with a mild form of EDS and experienced wide scarring on the bony protrusion areas, especially on the anterior portion of the knees, a trauma left from his childhood (). The physical examination revealed the skin hyperextensibility on the neck (), but the hand joint was not overextensible. His toes were laterally deviated (equinovalgus) with medial protrusion of the metatarsophalangeal joint, and there were hypertrophic scars with callus formation on the top of protrusions (). His open wound was initially a large hematoma formed after slipping down, and a part of the epidermal membrane covering the hematoma was removed from the private clinician for drainage of bloody fluid. Later, the dermatologist from the private clinic removed the dead skin on the wound. When he visited our hospital a month after the initial injury, his open wound was elliptically shaped with a 5-cm horizontal length and a 3 to 4-cm height. The wound had infected granulation tissue (). After a 5-day of wound treatment, the wound improved. Elliptical excision of the wound and primary approximation were planned because of the skin hyperextensibility.\nThe open wound was elliptically excised on the suprafascial level () and the wound margins were subcutaneously underminded. Approximation of the wound margins was done with an interrupted 4-0 Vicryl subcutaneous sutures and 6-0 nylon skin sutures. Seven days after the operation, the wound had healed with a mild discharge from the posterior half of the wound. Three weeks after the operation, the wound was a little disrupted with a skin breakage from the nylon sutures. Resuturing was done after the debridement of wound margins, and two bolster stitches with 2-0 nylon were added on the skin about 1 cm apart from the suture line to keep wound margins tension free. However, two days after the bolster stitches, partial skin necrosis was developed on the one anterior suture area. Two bolster stitches were removed and wound dressing changes were repeated every day. However, partially necrotized wound had not healed well (). The entire nylon sutures were taken out about 3 weeks after the reoperation because of the loosening of the nylon sutures.\nDuring the wound treatment, the patient bought collagen wound dressing, FIBRACOL Plus (Acelity/Systagenix, San Antonio, TX, USA), from the UK and the wound began to heal at an accelerated rate after the application of FIBRACOL Plus (). After about 4 months after the first operation, the wound was well healed without aberrant scarring (), and he experiences no pain or discomfort while walking. |
A 32-year-old female patient came to the hospital complaining of left leg numbness. She had experienced a traumatic event, a fall into a waterway, one month ago. After which, she had severe low back pain and was evacuated to the nearby general hospital. Computed tomography (CT) showed an L5 left transverse process fracture and an S2 body fracture, and absolute bed rest (ABR) for one month was recommended (). During this period, her left leg numbness was progressively aggravated. To evaluate whether symptom aggravation during the ABR period was related to the patient's history of a left-side L5–S1 discectomy 2 years previously, magnetic resonance imaging (MRI) was performed. The MRI did not show recurrent disc material, but it did show an annular tear at L4–5 and postoperative scar tissue at L5–S1, and the previous doctor recommended continued conservative management. After the period of ABR was complete, the patient began ambulation and was transferred to our hospital in her hometown. She complained of left leg numbness initially upon arrival, and the more she walked, the more the pain was aggravated, showing hyperalgesia and allodynia.\nGabapentin and Selective Serotonin Reuptake Inhibitors are prescribed to treat these neuropathic symptoms. And we ordered physiotherapy and wheelchair ambulation for the first two weeks. Various nerve blocks were performed, including sympathetic nerve block. Even as the symptoms seemed to subside somewhat, skin flushing still appeared on the left lower leg when the patient was in a standing position (). The persistent skin discoloration seemed to worsen as time went on, despite frequent sympathetic nerve blocks using steroid. The allodynia became so severe that the patient was hesitant to wear a sock. To rule out the possibility of arterial injury, we performed lower extremity CT angiography, and there was no sign of arterial injury around the fracture site. We concluded that her symptoms resulted from lumbosacral plexus injury due to the transverse process fracture. At that time, it was 2 months after the initial trauma. Because this was the acute phase, we wanted to prevent the disease from progressing to the chronic phase. Our clinics have been performing prolotherapy using PDRN solution, and we are familiar with the effects of PDRN. We injected PDRN solution at the ventral surface of the left L5 transverse process, superior and inferior, one ampoule (3 cc) each by using needles used for medial branch block (). Needles was inserted perpendicularly through C-arm-guided approach.\nAllodynia and hyperalgesia were improved one day after the procedure and the skin flushing was diminished (). The patient was discharged 3 days after the PDRN injection and at the 1-month follow-up, her symptoms were very much improved. |
A 59-year-old male with a past medical history of paroxysmal atrial fibrillation and class III obesity presented to the emergency department with complaints of intermittent palpitations for the past 2 weeks, increased watery output from his ileostomy and worsening bilateral lower extremity edema. He denied chest pain, diaphoresis, shortness of breath, dizziness, nausea, vomiting and abdominal pain. On physical examination, he was not in acute distress. He was tachycardic with sinus rhythm and his remaining vitals were stable. Abdominal exam demonstrated a large midline abdominal scar and a right ileostomy. The ileostomy was positive for watery stool. The bilateral lower extremities demonstrated 4+ edema. The rest of the physical exam was unremarkable.\nThe patient’s past surgical history is very complicated. In February 2015, the patient presented for ischemic colitis with bowel perforation, requiring an exploratory laparotomy that resulted in a subtotal colectomy with end ileostomy. His hospital course was protracted due to wound infections and intra-abdominal abscesses. After 7 months, he presented for an ileostomy reversal and underwent a laparotomy with small bowel resection and ileocolic anastomosis. His hospital course was protracted once again due to an intra-abdominal anastomotic leak and multiple abdominopelvic abscesses. This resulted in a resection of his ileoproctostomy, multiple abdominal washouts, a small bowel resection and creation of an end-ileostomy. In 2016, the patient presented multiple times with a recurrent left retroperitoneal abscess along the posterior abdominal wall and underwent three open abdominal surgeries for incision and drainage. A computed tomography (CT) scan from November 2016 did not show any evidence of kidney herniation ().\nLaboratory values showed evidence of an acute kidney injury with suggestive urinary tract infection. Renal ultrasound demonstrated bilateral nephrolithiasis with moderate left hydronephrosis. Abdominal/pelvic CT scan confirmed left proximal ureteral stone with left hydronephrosis and a prominent parastomal hernia at the ileostomy site, which contained portions of the right kidney, right proximal ureter, mesenteric/retroperitoneal fat and some bowel ().\nThe patient was taken to the operating room for a left cystourethroscopy with stent placement. Due to the patient’s extensive surgical history—high risk of surgical complications—and lack of evidence indicating right renal impairment, ileostomy revision and parastomal hernia repair with relocation of the right kidney was not recommended at this time. |
The third case is of a 68-year-old Irish woman who presented to the MMUH in April 2016 with acute laryngitis. She had a background of bipolar affective disorder which had been stable for the past 30 years on monotherapy with lithium. There had been a recent history of lithium toxicity secondary to a deterioration of her renal function, which had been managed at her local psychiatric hospital. After the episode, she had been restarted on a low dose of lithium as well as a low dose of valproate.\nOn presentation to the MMUH she was initially treated jointly by the ear, nose, and throat (ENT) team and medical team and was managed in an ICU environment due to respiratory compromise. She had no oral intake for multiple days. Once stabilized she was transferred to an acute medical ward but an acute onset confusional state with bizarre behavior was noted over a period of 2 days. Due to her psychiatric history the Liaison Psychiatry service was consulted. On review she was severely thought disordered and confused. She was only able to produce a word salad and showed echolalia. She had motor retardation, increased tone, negativism, and posturing on examination. The impression was that she was suffering from acute catatonia. Brain imaging did not reveal acute abnormalities. She was diagnosed as having bipolar I disorder with catatonia as per DSM-5 (Table ).\nAdvice was given to treat her with paliperidone. Her mental state improved slightly as a result, but she remained severely thought disordered and confused for 2 weeks. Eventually, lithium was cautiously reintroduced under close monitoring of her renal function. The reintroduction of lithium was well tolerated and she improved significantly over a 2-week period. At discharge she was no longer thought disordered, she was well orientated, and back to her fully independent baseline. She continues to live independently to date. |
A 64-year-old, 87 kg female presented for clipping of a 4 mm wide neck unruptured saccular anterior communicating artery aneurysm which was discovered incidentally during the evaluation of headaches and memory difficulties. Her past medical history was significant for remote breast carcinoma, hypertension, obstructive sleep apnea, and gastroesophageal reflux disease. She was a lifelong nonsmoker and notable preoperative medications included aspirin, furosemide, and propanolol. Preoperative imaging had no evidence of prior infarcts. After induction of anesthesia, a right internal jugular central venous catheter and radial arterial catheter were placed and maintenance of anesthesia was performed with a combination of 0.5 MAC sevoflurane, propofol 50 mcg/kg/min, and remifentanil 0.125 mcg/kg/min infusions. Mannitol 1 gram per kilogram for a total of 80 grams was administered. The neurosurgeon placed an 80 cm closed-tip, barium impregnated lumbar drainage catheter with a 0.7 mm inner diameter (Medtronic USA, REF 46419) at the L3-4 level prior to the start of surgery.\nThe surgery commenced and was progressing unremarkably. Prior to opening of the dura, 20 mL of CSF was drained over 15 minutes per neurosurgeon request. After dural opening and during dissection of the aneurysm, the surgeon requested the lumbar drain to be opened allowing further drainage of CSF. After approximately 15 minutes, the blood pressure sharply increased over the course of 1 to 2 minutes from a baseline systolic blood pressure of 130 mmHg to over 205 mmHg with an associated decrease in heart rate from 60 bpm to 50 which resolved over the course of minutes. This acute hypertension was treated with a number of interventions including 250 mg propofol and 1 mcg/kg remifentanil boluses aimed at treating light anesthesia as well as a bolus of 7.5 mg labetalol. At the time of the hypertensive event, the propofol, remifentanil, and Sevoflurane dosing had been stable and unchanged for over an hour. There was also no change in the level of surgical stimulation at this time as the dura had been incised and the neurosurgeons were using the operative microscope to expose the aneurysm. After the aneurysm was successfully clipped, the lumbar drain was closed and the surgery was completed without further episodes of hypertension. A total of 60 mL of CSF was drained via the lumbar drain during the case in addition to the losses from the surgical field.\nAt conclusion of the operative procedure, the patient remained comatose and unresponsive despite an hour in the operating room awaiting emergence from anesthesia. A postoperative head CT was obtained prior to transport to the intensive care unit which demonstrated mild cerebral edema and borderline inferior transtentorial herniation, but no significant hemorrhage or focal abnormalities. She was then transported to the Neurocritical Care Unit intubated and ventilated. Initial arterial blood gas analysis on arrival to the ICU did not reveal a cause to her delayed emergence (pH 7.35, pCO2 47 mmHg, pO2 323 mmHg, glucose 204 mg/dL, and sodium 137 mEq/L). She did not receive any benzodiazepines during the case, but did receive 50 mcg of fentanyl on induction and 1 gram of levetiracetam in addition to the propofol, remifentanil, and Sevoflurane maintenance. Initial neurological exam demonstrated midline and equal 4 mm pupils which were reactive to light bilaterally. She withdrew to painful stimuli in all four extremities. On postoperative day (POD) 1, her mental status continued to be depressed with a Glasgow Coma Scale of 7T (E2:V1T:M4). That day, an MRI was obtained which revealed bilateral thalamic infarctions on the diffusion weighted imaging which can be seen in . Supportive care was continued and over the course of several days, her mental status slowly improved to GCS of 10 (E4:V1:M5) on POD 5. This allowed for safe extubation that day. She continued to improve and was alert and oriented to person, place, time, and situation with some memory and attention difficulties by POD 16. A timeline of her in-hospital recovery can be found in . She was discharged home with referrals for home physical, occupational, and speech therapy on POD 19. She continued her gradual neurological improvement and by over a year postoperatively, Neurology records indicated she was living independently and her Montreal Cognitive Assessment score had recovered to a normal 27/30. |
A woman aged 52 years, presented in May 2015 with pain and swelling of the left elbow of 5 months duration. Movements at the elbow were restricted.\nAt the age of 50 years, 4 years after menopause, she had an episode of heavy vaginal bleeding. Endometrial curettings showed well-differentiated endometrioid adenocarcinoma. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy in July 2013. Histopathological examination confirmed a well differentiated endometrioid adenocarcinoma, with myometrial invasion (Stage Ib, Grade 1). She received adjuvant external beam radiotherapy to pelvis (5040 cGy) and brachytherapy to vault thrice. She completed her treatment in December 2013 and since then has been on regular follow-up.\nAt presentation, her performance status was 1. She weighed 59 kg, and her height was 1.4 m. Her body surface area was 1.48 m2 and body mass index is 29.3 kg/m2. Breast and abdomen examinations were unremarkable. Pelvic examination did not reveal any local recurrence. A soft, tender swelling was noted over the lateral aspect of the left elbow toward the lower end of the humerus. Rest of the skeletal examination was normal.\nPlain X-ray showed an osteolytic lesion in the distal humerus with soft tissue swelling. The joint space was well preserved. There was no fracture [].\nMagnetic resonance imaging (MRI) of left elbow showed a lytic expansile lesion in distal metaphyseal region of humerus with cortical erosion. The zone of transition of the lesion with normal bone marrow appears to be wide with irregular margins. There was edema in the muscles around the elbow both anterior and posterior compartments [].\nA technetium bone scan showed focal increased tracer uptake in the distal end of the left humerus. Rest of the skeletal survey was normal.\nA sonography-guided fine needle aspiration cytology showed metastatic deposits of poorly differentiated adenocarcinoma.\nSonography and contrast enhanced computed tomography scan of abdomen and pelvis did not reveal any local or distant metastasis.\nBased on the clinical, radiological, and cytological examination, a diagnosis of isolated humerus metastasis following primary endometrial carcinoma was confirmed.\nShe received a single fraction of radiation (800 cGy) locally to distal humerus to reduce fracture risk. Later, she was given six cycles of paclitaxel (175 mg/m2) and carboplatin (area under the curve 5) at 3-weekly intervals. She was also given intravenous zoledronic acid 4 mg once in 4 weeks. She tolerated chemotherapy well with good response to her symptoms.\nA repeat bone scan showed the persistence of isotope uptake in the left humerus. She is symptom-free and on regular, 3 monthly follow-ups. |
Mr. P D, a 63-year-old male, presented to the emergency room in mid-December with chief complaints of new onset of severe pain in his neck, right chest wall (corresponding to the fifth and sixth intercostal spaces) and right arm, and weakness in both lower limbs. The intensity of pain was a constant 10/10 on the numeric rating scale, not responding to intramuscular injections of diclofenac or tramadol at home. He was a known case of locally advanced nonsquamous cell lung cancer (adenocarcinoma), despite having undergone surgical resection of the tumor, chemotherapy, and radiation earlier. Comorbid illnesses included interstitial lung disease, for which the patient was on oral prednisolone 15 mg/day. Neurological examination revealed spastic paraparesis (power grade 2/5 in both lower limbs) with a flexor response on bilateral plantar reflexes. Bilateral upper limb motor power was grade 3/5. He was admitted under the care of his primary critical care physician. Magnetic resonance imaging (MRI), done in view of the symptoms, revealed the right Pancoast's tumor infiltrating the chest wall, right brachial plexus, and T1, T2, and T3 vertebrae. There was contiguous infiltration of the vertebral body and posterior elements with enhancing circumferential soft tissue extending the foramina and epidural space resulting in cord compression with intramedullary edema. The emergency neurosurgery team was called in for an opinion, and they ruled out surgery because of the advanced disease status of the patient. He was started on an infusion of injection fentanyl at 25 mcg/h. Dexamethasone 8 mg was started twice a day after admission. Fentanyl was escalated to 50 mcg/h and to 80 mcg/h in a day's time by the primary team for his uncontrolled pain. He continued to have breakthrough pain despite this. Owing to his uncontrolled pains, the pain management team was called in, which increased his doses for breakthrough pain by starting intravenous (i.v) morphine 10 mg every fourth hourly, which was increased to 10 mg (i.v) hourly for pain relief. The i.v fentanyl infusion was gradually titrated downward, and fentanyl transdermal patches were applied. In addition, the patient was on adjuvants for neuropathic pain, including baclofen, nortriptyline, tapentadol, and pregabalin. The pain continued to be consistently severe, and progressive (>7/10) on a numeric rating scale over the next 2 days. The Ramsay sedation score was −1, with pain reported on arousal. There was intermittent, new onset, involuntary jerky movement in the right upper limb. The caregivers, the patient, and the team noticed a vicious cycle of intense pain → opioid administration → transient worrisome drowsiness and an increase in myoclonic jerky movements → return of intense pain at the end-of-dose effect. An MRI screening was done to rule out brain metastases. It revealed an increase in the malignant spinal cord compression at D1-D2. The neurology team diagnosed opioid-induced myoclonus after ruling out possible organic causes. The patient was frustrated and reluctant to take any medication since he would attribute the bothersome drowsiness and jerky movements to medications. The pain was attributed at the time to spinal cord injury at D2 with both neuropathic and nociceptive contributors. At this point, on day 5 of admission, the pain management team recommended a continuous cervical epidural catheter after a detailed discussion within the team and with the family. The rationale was to deafferent the constant nociceptive barrage of signals traveling up the cord from D1 and below. The family physician intervened on our behalf as a patient advocate and helped translate our goals of care to both the doctors and the family.\nOn Christmas eve, after an informed consent, with anesthesiology stand by, the pain team inserted a cervical epidural catheter under strict aseptic precautions in the operation theater, under antibiotic cover. The patient was placed in the semi-prone position, an 18-gauge Tuohy needle was inserted between the cervical spinous processes in the midline (translaminar approach), under c arm guidance at C5-C6 level. Needle position was confirmed using a loss of resistance technique with saline and after visualizing tip in anteroposterior and lateral views under single shot and continuous fluoroscopy using water-soluble radiocontrast (iohexol 300). The 19-gauge catheter was threaded through this needle to a depth of 4 cm inside the epidural space, tunneled subcutaneously, and a bacterial filter was attached. We administered an initial bolus of a mixture of injection dexamethasone 4 mg and injection xylocard 2% 4 mL. The rationale for using the steroid was to deliver the steroid as close to the area of the cord compression as possible. The patient tolerated this procedure well. We shifted the patient to the intensive care unit for observation in the night, and he was started on an epidural infusion of the local anesthetic ropivacaine 0.1% concentration at a rate of 3 ml/h. The patient reported an immediate reduction of pain to 3/10 on the numeric rating scale. Mr. P D slept that night, and injection morphine was prescribed for rescue analgesia. He required one dose that night but refused to take any further doses after it made him drowsy. He did not report any episode of intense pain thereafter. The patient was shifted to the ward on Christmas day with a continuous cervical epidural infusion via a nonelectronic, ambulatory, disposable, elastomeric, silastic infusion device (Baxter™) filled with ropivacaine at 5 ml/h at 0.1% concentration. The patient was cheerful, pain-free, and alert with adequate pain relief and improved quality of life. The involuntary movements had stopped, and he was not on any opioids after the 1st day. The only adjunct was pregabalin (450 mg/day). As per protocol for continuous local anesthetic infusions in our institution, on day 5, we stopped the infusion for up to 12 h (pain score was 0) and removed the catheter subsequently. We discussed the possibility of an intrathecal continuous drug delivery implant in the future. However, the pain did not recur after this 5-day period of desensitization, and the patient was subsequently discharged the next day. The patient continued to be absolutely pain-free until day 64 when he passed away due to pneumonia, which was a complication of his spinal cord injury. His words to our team at the time of his terminal discharge from the ward, were, “this has been the best Christmas gift of my life.” |
A 13-year-old right-handdominant boy presented with pain and locking while moving the right shoulder during basketball activity and recurrent swelling around his right shoulder. There was no history of trauma, major illness, or surgery. His pain did not improve with conservative treatment, which included nonsteroidal anti-inflammatory drugs and physical therapy. On physical examination, he had a swollen right shoulder, but there were no signs of erythema, warmth, or muscle atrophy in his right shoulder. Active forward elevation of the right shoulder was 160°, external rotation was 70°, and internal rotation was at level T8. Plain radiographs of the right shoulder showed no abnormal findings (), but computed tomography (CT) and magnetic resonance imaging (MRI) showed several loose bodies in the axillary pouch and a large calcified mass lesion in the subscapular bursa ( and ). Arthroscopy under general anesthesia in the beach chair position was performed through standard posterior and anterior portals. Arthroscopic examination showed several loose bodies in the glenohumeral joint (). However, intrasynovial chondroid nodules were not seen in the glenohumeral joint. A sublabral foramen was found at the anterosuperior portion of the glenoid that communicated with the subscapular bursa (). Multiple large calcified mass lesions and intrasynovial chondroid nodules were seen in the subscapular bursa, suggesting Milgram Stage 2 (transitional phase) (). Loose bodies both in the glenohumeral joint and in the subscapular bursa were removed through a standard anterior portal, and proliferative thickened synovium of the subscapular bursa was also resected using a motorized shaver through the sublabral foramen. The histological examination of the loose bodies confirmed cartilaginous synovial metaplasia consistent with primary synovial chondromatosis (). The range of motion exercise was initiated from the day after surgery. Three weeks after the operation, he was symptom free with full range of motion and was able to return to sports. At follow-up after 5 years, he remained asymptomatic, and there was no clinical and radiographic evidence of recurrence. |
A 54-year-old previously healthy Caucasian female with otherwise unremarkable past medical history presented to emergency department with one-day history of hematochezia and abdominal pain. The patient described crampy left lower quadrant pain with no aggravating or relieving factors. She had a total of five bowel movements since symptom onset with the first bowel movement containing stool mixed with bright red blood followed by predominantly bloody stools. She took no medications on a regular basis and denied having a screening colonoscopy for colorectal cancer at age 50. She reported symptoms of upper respiratory tract infection (cold, sneeze, and cough) for which she took three doses of 120 mg pseudoephedrine purchased from a local grocery store for 1 day prior to symptom onset. Her maternal grandfather had prostate cancer but there was no significant gastrointestinal tumor history in the family. She was a nonsmoker and reported drinking socially (roughly one standard drink) once a week.\nHer admission vitals were within normal limits. Physical examination was consistent with mild tenderness on the left side of abdomen and hypoactive bowel sounds. Rectal examination showed bright red blood without any stool in the rectal canal. Her laboratory values were significant for mild anemia with hemoglobin of 11.5 mg/dl, hematocrit of 34.5%, erythrocyte sedimentation rate 31 mm/hr, and C-reactive protein 2.15 mg/dl. A computed tomography scan revealed mild to moderate mural thickening of the descending/sigmoid colon consistent with colitis without pericolonic abscess, ascites, or free air (). An infectious workup was obtained including blood cultures, stool cultures, gastrointestinal panel for Clostridium difficile, and gastrointestinal viruses but was negative. She was resuscitated with intravenous fluids.\nThe patient underwent colonoscopy which demonstrated segmental moderate inflammation in the sigmoid colon, descending colon and splenic flexure along with internal and external hemorrhoids. There was evidence of submucosal hemorrhages with mild edema in the aforementioned segments of the colon (). Endoscopic findings were highly suspicious of ischemic colitis. Several biopsies were obtained from the inflamed areas which exhibited focal lamina propria eosinophilic change with mild crypt attenuation and loss of goblet cells consistent with mild ischemic changes. There was no evidence of chronic inflammation.\nShe was observed in the hospital for 3 days and her diet was progressed slowly. Her bloody bowel movements ceased after 1 day in the hospital and patient was counseled and educated regarding avoidance of pseudoephedrine and over the counter medications for symptomatic management. |
The second patient died was a 39-year-old female with univentricular heart (tricuspid atresia), who had had previously two cardiac surgeries (the last was atrio-pulmonary Fontan, 33 years earlier). The patient suffered from a serious right atriomegaly with frequent episodes of atrial tachycardia, so she was a candidate for Fontan conversion surgery. Pre-operative ventricular function was mildly reduced (50%). In the post-operative, the patient suffered from severe single ventricle dysfunction resulting in LCOS and the need for ECMO implantation. Both patients could not be weaned from ECMO due to multi-organ failure.\nSeven patients required pace-maker implantation due to post-operative sinus node dysfunction or atrioventricular conduction abnormalities. All patients were discharged on oral antiarrhythmic for 3–6 months and anticoagulants for 6 months. At discharge, 15 patients were in sinus rhythm, 5 had a stable pacemaker rhythm, 2 had atrial fibrillation, and 1 atrial flutter. One patient discharged in sinus rhythm had a pacemaker implant 5 months after the operation due to the presence of sinus node dysfunction.\nDuring a median follow-up of 14 months (IQR 7–27), there was no late mortality and 17/23 patients had an improvement of NYHA functional class. Five patients in NYHA III progressed to class II and 4 to class I; eight patients progressed from class II to class I.\nAt follow up electrocardiogram, 16 patients were in sinus rhythm, 6 with stable pacemaker rhythm, and 1 with permanent atrial fibrillation.\nRecurrence of arrhythmia occurred in 2/23 (8.6%) patients, more than 3 months after surgery. These patients presented at surgical ablation with history of atrial fibrillation lasting 4 and 19 years, respectively, and both had atrial fibrillation, which was treated with right-sided Maze rather than Cox maze III due to technical issues. Sixteen (69%) patients are in stable sinus rhythm, 12 without any anti-arrhythmic therapy. At median follow up of 14 months (IQR 7–27), freedom from recurrence of arrhythmia was 90.9% and cumulative risk of recurrence was 9.6% (). |
A 54-year-old man presented to our emergency room after being involved in a motor vehicle accident in which the van he was travelling in overturned. He was conscious and oriented, and his only complaint was bleeding from the right ear and pain in the area when he tried to open his mouth.\nOn examination, he was found to have swelling and tenderness in front of the right ear. There was a 1 cm long vertical laceration just medial to the right tragus []. There was no active bleeding. His mouth opening was a full range but accompanied by pain in the right pre-auricular area. His dental occlusion was normal.\nThe laceration was sutured under local anaesthesia, and a screening X-ray of the skull was ordered. The X-ray showed a spring in the right infratemporal fossa []. This was initially thought to be an artifact but on questioning the patient, he recalled pulling out a ballpoint pen, which had impaled him in his right ear, immediately after the accident. He had been carrying the pen in the right breast pocket of his shirt when the accident occurred.\nA computed tomography (CT) scan was carried out which showed a 3 cm long spring in his right infratemporal fossa [].\nHe was taken up for exploration of the wound under general anaesthesia with the plan to approach the infratemporal fossa through the intraoral route, using the assistance of an image intensifier, if required.\nThe wound was gently probed using a medium artery clamp with a finger in the mouth acting as a guide.\nThe tract was 8 cm long extending anteromedially into the infratemporal fossa. Multiple fragments of black plastic and a 3 cm long spring were removed, obviating the need for an open procedure. After thoroughly irrigating the wound, it was loosely closed with 5-0 nylon sutures. His post-operative period was uneventful. |
A 4 year old female presented with huge abdominal swelling first noticed by her parents at 2 years of her age (See Figure ). The swelling had increased in size since then to occupy almost whole of the abdomen. Right labia had a swelling which increases in size on standing and decrease on lying down. On examination abdomen was found to be distended, felt tense with flanks full. The swelling over the labia was reducible on manipulation suggesting its continuity with the abdominal swelling.\nUltrasound abdomen showed 30 × 20 × 20 centimeters huge, multiloculated, multiseptate, lesion extending from xiphisternum to pelvis with extension into the right labia majora. Lesion was causing lateral and posterior displacement of bowel loops and compression of liver and both kidneys. A provisional diagnosis of omental, mesenteric or ovarian cyst was made. CT scan was done to throw some more light on the site of origin. Non-contrast film showed the cyst filled with clear fluid possibly arising from the mesentery. Contrast film was suggestive of multiloculated cyst. Pelvic films proved the labial extention of the cyst (See Figure ), but still the exact site of origin remained unclear.\nPatient was taken up for exploratory laparotomy and midline vertical incision was kept. After opening the peritoneum a thin walled cyst was seen popping out of the skin incision (See Figure ). On exploration it was found attached to the greater omentum (See Figure ).\nCyst was deflated partially by aspiration and we found the swelling over labia majora got reduced as we deflated the cyst and no adhesions were found between the cyst wall and labia. Complete excision of the cyst wall along with part of omentum was done. Histopathological report of the cyst wall showed endothelial cell lining, foam cells, lymphatic spaces, lymphoid tissue and smooth muscles which are diagnostic of lymphangiomatous cyst (See Figure ). Patient was under follow up for since six months and had no clinical and radiological signs of recurrence. |
Eleven years ago, when our patient was a 33-year-old unmarried nulligravida, she developed CCC of the ovary for the first time. At that time, her menstrual cycle was regular and 30 days long, with dysmenorrhea, manifesting as lower abdominal and lumbar pain, persisting for 5–7 days. There was no family or medical history of note. She had visited a local clinic with the chief complaint of lumbar pain, where a right ovarian tumor 8 cm in size that included solid internal components, was identified, and she was referred to the University of Tsukuba Hospital. Transvaginal ultrasound had revealed a right adnexal mass with solid components, 68 mm × 53 mm in size, and an intramural uterine fibroid 23 mm × 27 mm, with no hypertrophy of the endometrium, which measured 2.1 mm. Blood counts and blood biochemistry test results revealed no abnormalities. Her serum CA19-9 level was 45.9 U/ml, CA125 was 22 U/ml, and CEA level was 1.3 ng/ml. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an 80-mm unilocular cystic mass in the right ovary with a papillary protrusion of longest diameter 45 mm. The left ovary was not enlarged, and there was no obvious peritoneal dissemination or enlarged lymph nodes. Surgery, comprising right adnexectomy, left ovarian biopsy, partial omentectomy and uterine fibroid removal, was performed. The right ovary was enlarged to 8 cm in size, and it was removed without intraoperative rupture and with its capsule intact. The left ovary was not enlarged, but was seen to have a small endometrial cyst, which was resected. The absence of peritoneal dissemination and enlarged lymph nodes was confirmed during surgery. The pathological diagnosis was CCC localized to the right ovary and endometriosis was observed in the left ovary, with ascites cytology Class III. The cytology was composed of clusters that include atypical cells having somewhat nuclear enlargement, and it makes a diagnosis difficult to distinguish mesothelial cells from malignant cells. The patient expressed a strong desire to preserve her fertility. Hence, pelvic and para-aortic lymph node dissections were performed via staged laparotomy, which revealed no metastases in any of the 91 pelvic or para-aortic lymph nodes that were removed, leading to a diagnosis of Stage IA CCC. Four courses of postoperative combination paclitaxel (175 mg/m2) and carboplatin (AUC6) chemotherapy (TC therapy) were administered to complete the initial treatment.\nNine years after the initial therapy, MRI during regular 6-monthly monitoring revealed the appearance of a 95-mm polycystic mass with a mural nodule of longest diameter 53 mm in the left adnexal region. Those findings were never seen at the previous MRI. At this time, her serum CA19-9 level was 12.9 U/ml, CA125 was 18.3 U/ml, and CEA level was 0.5 ng/ml, all of which were within normal limits. Disease recurrence in the contralateral ovary was diagnosed, and total abdominal hysterectomy, left adnexectomy and omental biopsy were performed. At the time of surgery, the left ovary was enlarged to 95 mm in size, and it was removed intact without intraoperative rupture of its capsule. The internal lumen of the tumor contained several milky-white mural nodules extending around 15 mm into the cavity (Fig. ). The histological diagnosis was CCC, but in addition to the possibility of recurrence, it was also considered that the tumor might have developed de novo, and the fact that clear cell adenofibroma (CCAF) was also present (Fig. ) suggested that this might have provided the genesis for its development. In addition, there were no endometriotic lesions in the non-solid cyst wall, and it only consists of fibrous membrane. The tumor was localized to the left ovary, and since ascites cytology was negative, it was diagnosed as Stage IA disease. The treatment was completed with four courses of postoperative TC therapy.\nTwo years after the completion of treatment, the patient is continuing outpatient monitoring with no sign of recurrence to date. |
A 28-year-old female with insignificant past medico surgical history presented with one day of acute onset pain in the periumbilical region that later migrated and confined to the RIF. She had associated intermittent fever, nausea, and loss of appetite. She did not have any urinary symptoms, bowel irregularities, or gynecological complaints. Abdominal examination was performed by two senior surgeons at two different occasions; the same day had findings of guarding and rebound tenderness at RIF. Hematological tests showed polymorphonuclear leukocytosis with left shift. Biochemical tests and urinalysis were normal. Urinary pregnancy test was negative. Abdominal radiographs were unremarkable. USG could not visualize appendix and was inconclusive except for probe tenderness in RIF. CT scan of the abdomen could not be done due to unavailability. A clinical diagnosis of acute appendicitis was made assigning an Alvarado score of 9/10. Laparotomy was performed using the Lanz incision in RIF. Intraoperatively appendix was found to be normal without evidence of inflammation or infection in RIF. In view of symptoms and signs, a possibility of other pathology was thought. Walking the bowel proximally up to 3 feet (1 m) did not show a Meckel's diverticulum or any other small bowel lesions. There were no obvious mesenteric lymph nodal enlargement and pelvic organs looked pristine. Approaching closure, just when the medial edge of the incision was retracted superomedially, a hemorrhagic lesion seemed to appear little deeper in the mid abdomen. Therefore, the incision was extended transversely from the medial edge to explore further. Entire bowel was explored and this revealed an ulcerated lesion measuring 7 × 5 cm arising from the antimesenteric border of the ileum 8 feet (2.5 m) from ICJ with localized interloop hemoperitoneum and inflammatory exudates as shown in . Resection of ileal segment containing the lesion was performed followed by restoration of bowel continuity and peritoneal toileting. The lesion was subsequently reported to be an ulcerated malignant ileal GIST.\nHistopathologically, gross examination confirmed the operative findings, and the cut section revealed a nodular lesion protruding out of the serosal surface measuring 7 × 5 cm along with 2 lymph nodes each measuring 2 × 1 cm.\nMicroscopically, the growth from the ileum had villous lining epithelium with focal ulceration. The submucosal region had a circumscribed nodule with proliferation of loosely cohesive spindle cells; some of which were arranged in vague storiform pattern and others in long fascicles. There were areas with epitheloid cells forming small anastomosing nests and cords. The areas in between these showed skenoid fibers along with focal areas of hemorrhage, infarction, and congestion as shown in . The mitotic figures were seen (8/50 high-power field). The lymph nodes were microscopically identified to be reactive, and the resected margins of the ileum were free of tumor.\nBased on tumor size and mitotic activity, possibility of a malignant GIST was suggested along with immunohistochemical analysis (CD117 and CD34) for further confirmation. The patient had an uneventful recovery and was discharged on the 8th postoperative day. She was advised to review a week later at the outpatients but failed to report. All possible contacts were used to trace her, but she remained inaccessible and lost to follow-up. |
A 39-year-old woman presented with a 2-month history of recurrent episodes of chest pain and syncope. Cardiovascular examination showed a normal heart rhythm, and a continuous murmur could be heard at the second intercostal space of the left sternal margin. An electrocardiogram showed nonspecific ST-T abnormalities. A computed tomography scan showed a widened pulmonary artery and pericardial effusion. TTE revealed an echo-dense mass (5.86 × 5.68 × 4.53 cm) in the anterior and inferolateral walls of the left ventricle () on the apical four-chamber view. Color Doppler flow imaging did not detect blood perfusion into the tumor (). However, the mass was enhanced on MCE (). The left ventricle was mildly dilated with normal systolic function (ejection fraction of 58%). A patent ductus arteriosus was also found (diameter of 0.6 cm). The estimated systolic pulmonary artery pressure was 77 mmHg ().\nCMRI revealed a mass located in the left ventricular anterior and lateral walls (). The mass appeared hypointense on equally enhanced T1-weighted images () and hyperintense on T2-weighted images, showing unclear boundaries with the normal myocardium ().\nBecause the tumor was located in the left ventricle, the tumor was relatively large, and its benign or malignant nature could not be determined on preoperative examination. No surgical contraindications were identified. Arterial catheter ligation and total tumor resection were performed under extracorporeal circulation and general anesthesia. The patient recovered well after surgery and was discharged from the hospital 1 week later. During the operation, the surgeon found a large soft mass (8.0 × 5.0 × 5.0 cm) in the middle and lower segments of the anterior and lateral walls of the left ventricle. The histopathological diagnosis of the biopsy specimen was a neurogenic tumor with short spindle cells in degenerative nodules (). Spindle cell immunohistochemical analysis revealed the following results: cytokeratin (weak +), epithelial membrane antigen (weak +), CD34 (+), STAT6 (−), desmin (−), smooth muscle actin (+), S-100 (+), Ki-67 (1%–2% +) (), B-catenin (−), vimentin (+), CR (−), and WT-1 (−).\nThis study was approved by the Medical Ethics Committee of the Second Affiliated Hospital, Third Military Medical University. The study did not interfere with routine treatment procedures, and the patient provided verbal informed consent. |
A 38-year-old male recent immigrant from the Dominican Republic presents to the emergency department with chief complaint of hemoptysis of two-week duration. The hemoptysis was associated with an increased shortness of breath and dry cough which began two months prior to admission and had progressively worsened. The patient further described right-sided chest pain, intermittent, 5/10, sharp, gnawing/dragging in nature radiating to the shoulders especially upon recumbency and relieved with sitting upright or leaning forward. He further describes neck pain and fullness, as well as progressive heaviness of the arms bilaterally with decreased strength and intermittent lack of ability to grasp objects. The patient had no significant past medical history and had never smoked in the past and no significant occupational/environmental exposures.\nFurther review of the patients past medical history revealed history of a doctor's visit 18 months prior to admission for similar complaints in the Dominican Republic based on reports provided by the patient; computed tomography of the chest along with “neurologic testing” were completed at that time. Documentation provided at this time by the patient of CT chest showed a right superior lobe pulmonary nodule measuring 1.8 × 1.5 cm. The patient, however, was discharged with a diagnosis of bronchitis and was treated with a course of antibiotics, and patient was lost to follow up.\nPhysical examination revealed tachycardia, ruddy discoloration of the face, and neck and upper chest with marked fullness noted around the neck extending from the superior aspect of the chest to the angle of the jaw. Examination revealed diminished ability and difficulty to sustain upward gaze for greater than 20 seconds was further noted. Snellen's card exam revealed visual acuity of 20/30 on the right and 20/25 on the left with subjective diplopia reported during the examination as well as during testing for finger to nose agnosia. Diminishing speed and increased difficulty to complete testing of rapid alternating movements were observed. Inability to maintain a sustained hold against gravity of the upper extremities level with the shoulders while holding the arm in an outstretched position was noted on the right greater than the left. Repeat examination following a period of rest produced similar results. No air-entry was heard at the right lung base. The physical examination was otherwise unremarkable and the patient was hemodynamically stable. A complete blood count and complete metabolic panel were also ordered and were significant for a large leukocytosis (WBC: 19.1, PMN: 75 & band: 2); rest of the laboratory tests were unremarkable ().\nCT chest was ordered and revealed a large right mediastinal mass measuring 8.8 × 6.2 × 9.6 cm (Figures and ), consistent with malignancy, involving the right cardiac atrium with occlusion of the right main pulmonary artery either invaded or compressed by the mass, and no significant opacification of the right pulmonary arteries. The mass was further found to compress the superior vena cava with mild deviation to the left, also extending to the perihilar right upper lobe. A small right-sided pleural effusion was seen as well as a pericardial effusion measuring 1 cm anteriorly. A 1.2 × 0.5 cm pleural based nodule was found in the posterior left lower lobe. The patient was subsequently admitted to the hospital for further evaluation and a request for documentation of previous testing, including the “neurologic testing,” was placed ().\nOn hospital day two, the patient was taken for CT guided biopsy of the mediastinal mass. Pathologic analysis of the sample was read as adenocarcinoma of the lung, poorly differentiated. Immunohistochemical stains showed positive for CK7 () and TTF1 () and negative for P63 and Napsin A markers.\nSubsequently cardiothoracic surgery was involved to place a pericardial window for drainage and characterization of the pericardial fluid. Perioperative assessment revealed a hemodynamically stable patient. The patient denied any pain only mild fatigue and chest discomfort. Review of systems revealed remained similar to admission with a substernal dragging sensation and generalized feeling of fatigue. The neck range of motion remained slightly limited due to mild pain with flexion. A Mallampati grade 2 score was given to the individual with a thyromental distance of 8 cm, and sternomental distance of 10 cm, with natural dentition and no loose, chipped, or missing teeth. The patient complained of mild shortness of breath. The patient was subsequently intubated and received generalized anesthesia. Cytological examination of fluid obtained from the pleural effusion and pericardial fluid demonstrated pleomorphic malignant epithelial cells arranged in small clusters and glands and singly. Postsurgery recovery was complicated by inability to become extubated. The patient was subsequently placed in the intensive care unit. Due to prolonged intubation and inability to extubate the patient, it was then decided to place a tracheostomy tube for the patient.\nOn day ten of admission records of the nerve conduction studies completed upon initial presentation in the Dominican Republic demonstrated a decrease in the axillary nerve conduction velocity. Repeated nerve stimulation test showed a progressive decline in the compound muscle action potential (CMAP) amplitudes by 18.9%, and a more marked postexercise decrement in CMAP amplitudes was noted. These findings were reported as consistent with myasthenia gravis.\nWith discussion of the patient's prognosis and the patient's wishes with family members, a do not resuscitate order (DNR) was elected. On day 27 of admission, the patient was found to be in asystole and subsequently expired. A postmortem analysis was unfortunately not done. |
A 40-year-old woman with prior mediastinal radiation therapy (RT) due to lung cancer at the age of 3 presented with recurrent syncope during exercise. During most of her adulthood, she had felt a reduced physical capacity compared to her peers. Echocardiography revealed moderate stenosis and insufficiency of a tricuspid aortic valve. She had no classical risk factors for development of coronary artery disease: no history of smoking, no diabetes, normal cholesterol levels, no arterial hypertension, and no family history of premature ischemic heart disease. Initial assessment consisted of exercise testing and coronary CT angiography. Exercise testing resulted in dizziness and significant ST-segment depression of 2-3 mm in inferior and lateral leads, but no chest pain. CT angiography revealed severe stenosis of the left main coronary artery () and ostial stenosis of the right coronary artery (RCA), confirmed by coronary angiography (CAG). Following a joint cardiology-cardiothoracic multidisciplinary team meeting (MDT), percutaneous coronary intervention (PCI) with drug eluting stents was performed in the left main coronary artery and ostially in the RCA.\nA few months later, ischemia was revealed during routine exercise testing as part of the outpatient cardiac rehabilitation program. A new CAG revealed stent occlusion ostially in the RCA. Re-PCI with stenting was performed; however, symptoms persisted, and re-echocardiography now revealed progression to significant aortic valve stenosis and significant insufficiency. Due to prior mediastinal RT and excessive aortic calcification and fibrosis as well as narrow diameter of both the aorta ascendens (annulus 23 × 17 mm and sinutubular junction 20 × 21 mm), descendens, and arcus, transcatheter aortic valve implantation (TAVI) was recommended by consecutive MDT meetings. TAVI was performed by inserting a 20 mm Sapien 3 valve via femoral approach without complications, along with redilatation of the RCA. She was asymptomatic at three years follow-up after TAVI.\nSevere coronary atherosclerosis and valvular heart disease have been reported as rare sequelae following mediastinal RT []. RT-induced coronary artery disease is associated with a characteristic pattern: primarily proximal stenosis, typically located at the ostium of the RCA and the left main coronary artery []. In RT-induced valvular heart disease, left-sided heart valves are more commonly affected [] and patients with prior mediastinal RT and severe aortic stenosis have significantly reduced long-term survival compared to patients with aortic stenosis but without prior RT []. Adults treated with RT in childhood may present with cardiac disorders as late as 30 years after therapy, and cardiac screening is therefore important []. Suggestions for a screening program containing echocardiogram and transesophageal echocardiography 10 years postradiation and every 5 years are available, but no standardized long-term screening program exists []. Thus, we stress caution and awareness of symptomatic patients with prior mediastinal RT due to an increased risk of severe coronary atherosclerosis and valvular heart disease even among younger adults without conventional cardiovascular risk factors. |
A 15-year-old man with spastic tetraplesia diagnosed with CLS at age 3 was referred to our hospital. One year before admission, the patient's parents noticed that he could not walk long distances and had a spastic gait. His gait disturbance became worse over time. One month before admission, the patient could not walk even several meters and had decreased upper extremity locomotor activity, such as an inability to elevate his arms. At the time of admission, he could not get up and could only make slight movements of the distal potions of his extremities.\nThe patient's appearance was consistent with previous descriptions of patients with CLS. He had a prominent forehead, coarse faces, broad nose, thick septum, wide mouth, and tapering fingers (Fig. ). He could emit a groan but could not have a conversation. We were unable to perform assess sensory function on physical examination. He could move his neck but not his trunk or extremities. Spastic weakness and very brisk muscle stretch reflexes were observed in all 4 extremities, and Babinski sign was observed. A balloon-catheter was placed because of dysuria. We suspected his symptoms were caused by cervical spinal cord damage, and performed imaging studies.\nComputed tomography (CT) showed hypoplasia of the posterior arch of the C1 vertebra, and continuous calcification of the YL from C2 to T3 (Fig. ). Magnetic resonance imaging (MRI) revealed that the cervical spinal cord from C1 to T1 was compressed by the calcification (Fig. ). Image quality for MRI was low because the patient reacted poorly to anesthesia and moved during the examination. Although diagnostic information was limited, we diagnosed acute exacerbation of cervical myelopathy, and performed emergency surgery.\nUnder general anesthesia in the prone position, the C1 to C7 laminae were exposed. Twenty millimeters of the width of the C2 to C7 laminae were removed using a high-speed drill. Adhesions between the calcification and dura mater were gently stripped off, and the laminae were resected en block with the calcification (Fig. ). Fifteen millimeters of the width of the C1 posterior arch was removed using a high-speed drill. Pulsating dura mater was observed after laminectomy, but the pulse was weak. The dura mater appeared hypertrophic; however, we did not incise the dura mater.\nCalcification was observed in the YL (Fig. ). Crystal analysis confirmed that the calcification consisted of calcium pyrophosphate and tricalcium phosphate.\nA postoperative CT showed sufficient posterior decompression, although calcified dura mater was observed (Fig. ). Movement of the extremities gradually improved after the operation. Two weeks after the operation, the patient could raise his arms over his head, have a meal with spoon, and walk with the assistance of a walker. After undergoing rehabilitation for 3 months, he could care for himself without any help in his daily activities. One year after the operation, he could run. Approximately 3 years after the operation, he fell down and developed increased spasticity of the lower limbs for 2 weeks, which resolved without any treatment. At that time, there was no worsening of symptoms in the upper limbs. We hypothesized that the temporary paralysis was caused by thoracic spinal cord damage, because there was canal stenosis secondary to ossification of the YL at the upper thoracic level. From this event to 8 years after the operation, his symptoms remained stable. Radiographs taken 1 week after the operation showed cervical hyperlordosis that has remained stable for 8 years. CT examination at 5 years after surgery showed progression of calcification around the dura mater but no spinal cord compression. |
A 29-year-old lady presented to the surgical out-patient department with the complaints of pain at the periumbilical region for one day which later shifted to the right iliac region. Pain abdomen was associated with 2–3 episodes of vomiting and anorexia. There was no history of fever, burning micturition, black-colored stool or passage of reddish urine. She gave no history of such illness among the family members, not any history of surgery in the past. She took pain killers for the associated pain. Clinical examination revealed tenderness on palpation in the right iliac fossa with rebound tenderness. Laboratory examination showed total leucocyte counts of 11,300/mm3 with neutrophilia (80%) and serum amylase of 98 U/l. Ultrasonography abdomen showed features of acute appendicitis with wall-to-wall diameter of 8 mm along with minimal free fluid in the pelvic and right iliac region. In view of the diagnosis of acute appendicitis, the patient was planned for open appendectomy as laparoscopic facilities were not available. The appendix was positioned in pelvic position with around 50 ml of collection in pelvis, inflamed along with a swelling around 4 × 3 cm involving the tip of the appendix. (A and B) In view of probability of carcinoids or malignancy, inflammatory fibroid polyps, gastrointestinal stromal tumors and lymphoma, the spillage of the appendiceal content was controlled and the lesion was removed enbloc with the meso-appendix. No enlarged mesenteric lymph nodes were evident. The cut section of the tip revealed a grey white mucoid area with 3 ml of pus and a faecalith. (C) Pus culture sensitivity showed growth of E coli sensitive to Nitrofurantoin and Cotrimoxazole. The patient was discharged on 2nd post-operative day without complications.\nHistopathological examination of the specimen showed submucosal edematous stroma consisting of inflammatory infiltrates composed of lymphocytes, plasma cells, eosinophils and focal formation of lymphoid follicles. The proliferation of scattered spindle to ovoid cells with plump nucleus and small nucleoli resembling myofibroblasts along with proliferating blood vessels with unremarkable over lying epithelium were evident suggestive of Inflammatory myo-fibroblastic tumor. (A and B) Post-surgical follow up in 6 months showed no any evidences of recurrence. The patient continues to be followed up in the clinic. |
A 74-year-old female presented to the emergency department (ED) with upper abdominal pain and melanotic stools. She had an elective open juxtarenal abdominal aortic aneurysm repair a month before her index presentation. She was hemodynamically stable. Her pertinent initial labs showed a hemoglobin of 6.7 g/dl (baseline 9.6 g/dl) with a hematocrit of 23%. Patient did not have any fever or leukocytosis. A CT abdomen with contrast done in the ED for abdominal pain showed nonspecific findings, i.e, irregularity of the “aneurysmal sac” with a small amount of fluid around the sac (see ) which was read by the radiologist as early postsurgical changes. She was admitted and was started on proton pump inhibitors. An esophagogastroduodenoscopy (EGD) was performed that revealed mild duodenitis. Her hemoglobin remained stable the next couple of days, and she was discharged home with a 6-8-week course of proton pump inhibitors. Two months later, she presented again with similar complaints with a drop of hemoglobin. A repeat EGD was performed that did not reveal any obvious source of bleeding, and she was discharged home after stabilization.\nA month later, she came for the third time into the ED with abdominal pain, hematochezia, and profound hypotension. Her pertinent laboratory findings include leukocytosis, low hemoglobin and hematocrit, thrombocytopenia, and transaminitis. She was resuscitated with IV fluids and blood transfusions. She was started on broad spectrum antibiotics after blood cultures were drawn. A CT abdomen and pelvis was performed which showed tiny foci of air at the anterior aspect of the native aneurysm wrap just inferior to the location where duodenum crosses (see ). At that time, a decision was made to perform push enteroscopy instead of simple EGD to evaluate second and third portion of duodenum which showed an aortoduodenal fistula with infected graft adherent to the bowel wall and extruding purulent exudate (see ). She underwent emergent surgical excision of the infected graft and bypass grafting to restore vasculature. Her blood cultures and cultures from the graft revealed methicillin-resistant Staphylococcus aureus (MRSA) and Streptococcus agalactiae. Aggressive management was continued with proper antibiotics in the intensive care unit, but her condition deteriorated, and she expired within several days. |
The patient was an 11-year-old male with a past medical history positive for psoriasis that presented in December 2016 with a complaint of an enlarged lymph node on the right side of the neck of approximately 3 months’ duration. He was born in Vietnam, but adopted at a young age and raised in Michigan. Therefore, family history could not accurately be obtained. There was no history of any precipitating viral upper respiratory infection or any other inciting events. Over 3 months, there were no changes or fluctuation in size of the enlarged lymph node. There was no history of dysphagia, odynophagia, dysphonia, or otalgia. There was no history of weight loss or night sweats. In addition, there was no history of fever.\nThe patient had previously been evaluated by a primary care physician who trialed a course of antibiotics without any improvement or resolution of the neck mass. In addition, an ultrasound of the neck was obtained which demonstrated multiple large right-sided neck lymph nodes, with the largest measuring 2.7 cm. Multiple lymph nodes in the left neck, which measured approximately 1 cm, were noted as well. Epstein-Barr Virus (EBV) serology was also performed. Epstein-Barr Virus Capsid IgG was positive, EBV Capsid IgM was negative, EBV Early Antigen AB was positive, and EB Early Antigen AB was positive. These results indicated the patient was likely exposed to an EBV infection in the past, with no active or recent infection. A Monospot test was also completed and negative.\nOn clinical examination, the patient’s neck demonstrated palpable right-sided cervical lymphadenopathy, with a prominent 2-cm lymph node in level 5. It did not appear infectious, as there was no evidence of erythema, edema, or warmth. Other than this palpable lesion, the remainder of his otolaryngologic examination was unremarkable and the patient remained completely asymptomatic. No other infectious serology or workup was pursued.\nGiven the chronicity of this enlarged lymph node, a CT soft-tissue neck with and without contrast was obtained. The Computed Tomography (CT) scan demonstrated diffuse lymphadenopathy, particularly within the right posterior triangle of the neck. The largest lymph node measured approximately 1.6 to 2 cm in greatest dimension. At this point, there was high suspicion for lymphoma. Therefore, an excisional lymph node biopsy was performed and the gross specimen was sent fresh for histopathological diagnosis. Given the low suspicion for an infectious etiology, no specimen was sent for culture.\nAccording to the pathology report provided by the local community hospital, the specimen demonstrated “extensive necrotizing granulomatous inflammation with abscess formation, suggestive of cat-scratch disease—pending final consultation.” However, further analysis was requested, and the specimen was also sent to the University of Michigan Laboratories. On further analysis, the final pathology report concluded the specimen as a benign reactive lymph node, consistent with KFD. In detail, their report revealed the specimen to have retained nodal architecture with extensive patchy areas of necrosis. Surrounding the areas of necrosis, the cellular elements were composed by numerous histiocytes, small lymphocytes, and a few plasma cells with no neutrophils or eosinophils present. In addition, the histiocytes present in the specimen featured “crescentic” nuclei.\nAdditional immunohistochemical stains were performed. The majority of T-cells expressed CD-8, rather than CD-4. Numerous histiocytes stained positive for CD-68. Furthermore, the histiocytes expressed CD-163, as well as myeloperoxidase. Finally, CD-123 immunostain highlighted plasmacytoid dendritic cells predominantly located around necrotic areas. Flow-cytometry studies were also performed, showing a decreased CD4/CD8 ratio and no definitive evidence of monoclonal B-cell population or aberrant T-cells. These findings showed a benign process, consistent with KFD lymphadenitis.\nThe patient’s cervical lymphadenopathy resolved in 2 months without complication. On recent follow-up via phone call in July 2018, the patient continues to be asymptomatic with no development of any signs and symptoms consistent with SLE. |
A 42-year-old male, nonsmoker, with medical condition significant for hypertension presented to the emergency department after a fall followed by two episodes of seizures. On presentation physical examination was notable for altered level of consciousness and mild symmetrical decrease in power of 4/5 in all four limbs. Laboratory workup including complete blood count, electrolytes, coagulation panel, lipid profile, urine, and serum drug screen was unremarkable. CT scan head revealed a 1.5 cm left temporoparietal lobe intraparenchymal hemorrhage with surrounding edema as shown in (). As part of the diagnostic workup, an ECG was also performed on admission which was normal. The patient was admitted to the neurointensive care unit (NICU) for further management. A computerized tomography angiogram was performed, which showed early draining veins at the site of the lesion, suspicious for an underlying vascular malformation. Subsequently a cerebral angiogram was performed which confirmed the presence of an AVM underlying the hemorrhage (). A partial embolization of the AVM was performed, and the patient was boarded for surgical resection ().\nOn day 3 of admission, the patient complained of sudden-onset chest pain. He described it as left sided, retrosternal, sharp, nonradiating pain, worsened when lying down on left side, lasted 2-3 minutes and then resolved spontaneously. It did not recur however prompted an ECG which showed sinus rhythm with nonspecific ST segment elevation in leads V3-V6 (). Cardiology was consulted who deemed the ECG changes as J point elevation suggestive of benign early repolarization and not a true acute coronary event. A high sensitivity cardiac troponin assay done immediately and repeated two times at 6 hours and 12 hours from the onset of symptoms remained negative (<0.017 ng/ml; normal value <0.057 ng/ml). A transthoracic echocardiogram (TTE) performed later that day revealed no regional wall motion abnormalities or left ventricular dysfunction. The next day, patient was taken for craniotomy and surgical resection of the AVM (). The surgery was uneventful. A follow-up ECG on the postoperative day 1 revealed pronounced ST elevation with new T wave inversions (in leads V2-V6) highly suggestive of acute STEMI (). The patient was completely asymptomatic with no chest pain or other cardiac symptoms. Serial estimation of high sensitivity cardiac troponin was again negative (<0.017 ng/ml) and a repeat TTE was unremarkable. Given these findings and the absence of the symptoms, no intervention was done and he was monitored in the NICU.\nThe patient did not have any further untoward event(s) and continued to do well postoperatively with normalization of his ECG changes over the next 48 hours (). He improved neurologically and was transferred out of the ICU on day 7. He was subsequently discharged on day 12 with home health physical therapy, neurosurgery, and cardiology follow-up appointments. An exercise stress test was eventually performed 3 months' after discharge which did not reveal any evidence of coronary artery disease. |
A 57-year-old man had noticed a mass in the right parotid area for several weeks. The mass was solid and firm with no pain or tenderness. Liquid-based aspiration cytology was performed (). The cytologic findings were numerous scattered osteoclast-like multinucleated giant cells and isolated mononuclear cells. The osteoclast-like giant cells and most of the mononuclear cells had benign-looking nuclei, but a few mononuclear cells had slightly atypical nuclei with clumped chromatin and prominent nucleoli. There was a small cluster of slightly atypical epithelial-like cells. We considered the diagnosis to be a giant cell tumor possibly with a carcinomatous component.\nThe resected parotid gland contained a 1.8 cm-sized well-circumscribed round tumor (). The cut surface was mottled with light and dark brown colors. There was no necrosis or hemorrhage. Light microscopic examination revealed a well-circumscribed but not encapsulated tumor (). The tumor consisted of evenly distributed multinucleated giant cells, which were morphologically indistinguishable from osteoclasts, and round or short-spindled mononuclear cells. At first sight the tumor appeared to be a giant cell tumor of bone, but a careful examination revealed that many mononuclear cells had hyperchromatic nuclei with clumped chromatin. Many mitotic figures were found, up to 30 per 10 high-power fields. In addition, there were two small foci of carcinomatous component. The carcinomatous component was very small, such that it disappeared in the additional sections. The nuclei of the carcinoma cells did not appear to be very malignant but the cells were arranged in a cribriform pattern so that they were recognized as malignant. It appeared that there was no transitional area between the giant cell tumor and the carcinomatous component. There was no metastatic lesion in the cervical lymph nodes.\nImmunohistochemically, osteoclast-like giant cells were diffusely positive for vimentin and CD68, and negative for cytokeratin and epithelial membrane antigen (). Mononuclear cells were diffusely positive for vimentin, partly positive for CD68, and negative for cytokeratin and epithelial membrane antigen. The carcinoma cells were positive for cytokeratin and epithelial membrane antigen, and negative for vimentin and CD68. |
The patient is a 28-year-old male with prior history of a grade 3 astrocytoma, who presented to our emergency department (ED) after having a general tonic-clonic seizure (GTC). Initially, his disease was appreciated four years prior when he presented with a GTC and was found to have a grade 3 astrocytoma of the left occipital lobe (Figure ). He underwent maximal safe resection followed by adjuvant EBRT to 45 Gy in 25 fractions at an outside institution. Due to reasons outside the patient’s control, he received only two cycles of adjuvant temozolomide following EBRT. He developed disease recurrence, presenting as a GTC six months prior to this ED presentation, where imaging demonstrated tumor progression with increased extension into the parietal lobe (Figure ). He subsequently underwent a second maximal safe resection later that month. Final pathology returned as GB. Follow-up imaging four months afterward demonstrated disease progression and he was started on bevacizumab as well as a tumor treatment field (TTF) device. He unfortunately only tolerated TTFs for one month, ending one month prior to the ED presentation.\nUpon admission to our hospital from the ED, MRI demonstrated progression of disease in the left occipital-parietal lobes with extension into the splenium and anterior-inferior extension into the left thalamus and basal ganglia (Figure ). His physical exam was notable for mild right-hand weakness, but he was otherwise neurologically intact. Despite changes to his antiepileptic medication, he had seizure recurrence a few weeks following admission. Presuming that the area of tumor recurrence received a definitive dose in the past, the consensus decision was to proceed with repeat maximal safe resection with GammaTile placement. A dose of 60 Gy was prescribed to a 5 mm depth using a total of eight tiles, each containing four Cesium-131 3.5U seeds, to line the post-operative cavity volume of 17.6 cc. A significant portion of the occipital-parietal disease was debulked, with final pathology again demonstrating a grade 4 astrocytoma with molecular studies indicating an IDH-mutated, ATRX mutated, and MGMT promoter methylated phenotype with hypermutation. Postoperatively he was noted to have right upper and lower extremity weakness/spasticity with right foot drop and mild right face weakness. He required a cane to assist with ambulation and reported word-finding difficulties and decreased short-term memory. He was planning to start adjuvant temozolomide; however, he developed a severe GTC with increased muscle weakness and altered mental status two months later. MRI following this episode demonstrated mildly increased enhancement to the tissue surrounding the surgical bed with the progression of disease in the splenium and left thalamus/basal ganglia (Figure ).\nGiven this symptomatic disease progression, a treatment plan was made to take the progressive regions of disease outside the irradiated GammaTile volume to 35 Gy in 10 fractions. As shown in Figure , an initial planning target volume (PTV) was delineated consisting of the T1 post-contrast-enhancing disease with a 5 mm margin. The volume that received greater than 35 Gy from the GammaTile treatment was excluded from the PTV. The PTV was then separated into two portions by subdividing the remaining volume between tissue that received less than 17.5 Gy (PTV1), and that which received 17.5-35 Gy (PTV2). Using a volumetric modulated arc therapy plan with five arcs, one non-co-planar, a homogeneous dose of 35 Gy in 10 fractions was delivered to PTV1. This dose was calculated to have a biologically equivalent dose in 2 Gy fractions (EQD2) of 45 Gy. Dose painting gradually decreased the dose from 35 Gy to as low a dose as achievable approaching the resection bed border of PTV2 (Figure ). A composite of the GammaTile dose volume (Figure ) with a dose delivered from PTV1 and PTV2 resulted in a homogeneous dose of approximately 50 Gy EQD2 to the residual disease extending into the splenium and the left thalamus/basal ganglia (Figure ). Digital imaging and communications in medicine (DICOM)-RT data from the initial 45 Gy delivered from the outside institution were obtained, and cumulative dose to critical organs at risk (OARs), including the brainstem, ocular structures, and cochlea, were within established constraints.\nDue to the hypermutation phenotype of his tumor, the patient was started on CCNU (100 mg/m2) and completed his EBRT with no issue or progression of his current neurological symptoms. He was last seen in follow-up by our team three months after his most recent EBRT, at which point he reported significant improvement in the right leg tremors/spasms and improved headache. He demonstrated decreased right foot drop but continued to have word-finding difficulties and short-term memory deficits. He has not developed any new areas of muscle weakness or paresthesia, visual loss, or other forms of altered sensoria. The current plan is to complete six cycles of CCNU with alternative systemic therapy thereafter upon disease progression. While temozolomide could be used to this effect, the high mutational burden found in his most recently resected disease would permit entry into currently available trials of immunotherapy. |
A 42-year-old male, nonsmoker, with medical condition significant for hypertension presented to the emergency department after a fall followed by two episodes of seizures. On presentation physical examination was notable for altered level of consciousness and mild symmetrical decrease in power of 4/5 in all four limbs. Laboratory workup including complete blood count, electrolytes, coagulation panel, lipid profile, urine, and serum drug screen was unremarkable. CT scan head revealed a 1.5 cm left temporoparietal lobe intraparenchymal hemorrhage with surrounding edema as shown in (). As part of the diagnostic workup, an ECG was also performed on admission which was normal. The patient was admitted to the neurointensive care unit (NICU) for further management. A computerized tomography angiogram was performed, which showed early draining veins at the site of the lesion, suspicious for an underlying vascular malformation. Subsequently a cerebral angiogram was performed which confirmed the presence of an AVM underlying the hemorrhage (). A partial embolization of the AVM was performed, and the patient was boarded for surgical resection ().\nOn day 3 of admission, the patient complained of sudden-onset chest pain. He described it as left sided, retrosternal, sharp, nonradiating pain, worsened when lying down on left side, lasted 2-3 minutes and then resolved spontaneously. It did not recur however prompted an ECG which showed sinus rhythm with nonspecific ST segment elevation in leads V3-V6 (). Cardiology was consulted who deemed the ECG changes as J point elevation suggestive of benign early repolarization and not a true acute coronary event. A high sensitivity cardiac troponin assay done immediately and repeated two times at 6 hours and 12 hours from the onset of symptoms remained negative (<0.017 ng/ml; normal value <0.057 ng/ml). A transthoracic echocardiogram (TTE) performed later that day revealed no regional wall motion abnormalities or left ventricular dysfunction. The next day, patient was taken for craniotomy and surgical resection of the AVM (). The surgery was uneventful. A follow-up ECG on the postoperative day 1 revealed pronounced ST elevation with new T wave inversions (in leads V2-V6) highly suggestive of acute STEMI (). The patient was completely asymptomatic with no chest pain or other cardiac symptoms. Serial estimation of high sensitivity cardiac troponin was again negative (<0.017 ng/ml) and a repeat TTE was unremarkable. Given these findings and the absence of the symptoms, no intervention was done and he was monitored in the NICU.\nThe patient did not have any further untoward event(s) and continued to do well postoperatively with normalization of his ECG changes over the next 48 hours (). He improved neurologically and was transferred out of the ICU on day 7. He was subsequently discharged on day 12 with home health physical therapy, neurosurgery, and cardiology follow-up appointments. An exercise stress test was eventually performed 3 months' after discharge which did not reveal any evidence of coronary artery disease. |
A 17-year-old left-handed male with severe Tourette's syndrome, attention deficit hyperactivity disorder (ADHD), and obsessive compulsive disorder (OCD) was evaluated by pediatric neuropsychology and child psychiatry prior to the date of electrode implantation for DBS for treatment of refractory tic symptoms. The patient was treated with bilateral centromedian parafascicular complex (CM-Pf) stimulation and was followed for 1.5 years. Preoperative neuropsychometric testing was noteworthy for borderline general intellectual functioning and commensurate academic achievement (). Cognitive weaknesses were noted in executive functioning (e.g., abstract reasoning, planning, and organization) and in learning, but memory retention was largely intact. The patient also exhibited bilateral fine motor dexterity impairment and difficulty with visual-motor integration. The patient's father revealed clinically significant concerns about anxiety, depression, somatic behaviors, and atypical behaviors (e.g., repetitive behaviors). He also indicated concerns about hyperactivity, attentional problems, poor adaptability, and poor independent functioning. As per history provided by the patient and his family, the genesis of the patient's psychiatric symptoms coincided with the evolution of the patient's TS. These progressively worsened over time and had been refractory to pharmacologic and nonpharmacologic intervention. The patient presented to our tertiary care center for consideration to initiate DBS treatment due to the severity of his neurologic and neuropsychiatric symptoms.\nPreoperative psychiatric evaluation demonstrated severe psychosocial impairment related to treatment refractory Tourette's syndrome. Social impairment was associated with significant amounts of missed school and anxiety related to tics. Attempts to garner employment were hampered with being overwhelmed by his neurologic symptoms. Past psychiatric history was significant for three prior psychiatric hospitalizations, all occurring within the context of managing a complicated psychotropic medication regimen, and treatment approach for comorbid psychiatric conditions (). Inpatient hospitalization prior to electrode implantation was recommended to safely taper some of the psychotropic medications that could interfere with DBS treatment.\nPostoperative neuropsychometric testing revealed general cognitive functioning that had remained stable across time (). The patient continued to have difficulty with learning information but retained information once learned. No significant changes were noted in the patient's cognitive ability compared with baseline testing.\nPostoperative psychiatric evaluation was noteworthy for significant functional and psychosocial gains. No concerns for suicidal or self-injurious thoughts or actions were noted. The patient's psychotropic medication load was also significantly reduced (). Improved psychosocial functioning corresponded with improvement in tic symptoms (). |
A 36-year-old male, a seasoned cyclist with no past medical history, presents to the emergency department with complaints of lightheadedness and diaphoresis after a bicycle fall. Patient was participating in a bicycle race when another rider ahead of him fell causing the patient to swerve to avoid him. Patient states that he fell on his left side and hit a tree with his right leg. Patient was wearing a helmet and did not suffer any chest or head trauma. After the fall, he felt lightheaded and diaphoretic and complained of mid back pain. Patient denied any chest pains or shortness of breath. Patient was subsequently brought to the hospital directly following the accident by ambulance.\nIn the emergency department, patient was noted to be in no acute distress; initial blood pressure was 128/69 mmHg with pulse of 65 beats per minute. He was afebrile, not tachypneic, and well appearing with marked right thigh swelling and tenderness to his medial thigh. Given the dizziness and diaphoresis initially, patient had an ECG performed which showed lateral ST segment elevation () and had a subsequent troponin I that was positive, 0.49ng/mL, with a Creatine Phosphokinase (CPK) of 617 U/L.\nThere was initial concern for a possible cardiac contusion, although the patient had no chest wall trauma and thus was admitted for further evaluation. As an inpatient, an echocardiogram was performed demonstrating normal right and left ventricular function and trace pericardial effusion while the patients troponin continued to trend upwards towards a maximum of 21ng/mL. He was loaded with Aspirin and Clopidogrel as well as initiation of a heparin infusion, Lisinopril, and a Beta Blocker. Coronary angiography was subsequently performed demonstrating a spontaneous coronary artery dissection of left anterior descending coronary artery. No further diagnostic study was performed at that time. Further history revealed that he took multiple caffeine Jello shots and drank a large cup of coffee prior to participation in the race. He denied cocaine, amphetamine, or other performance enhancing drug use ().\nThe patient's CPK and troponin trended downwards on conservative medical management and his back pain resolved; therefore a stent was not placed. The patient was visiting from outside the area; discharge planning included repeat coronary angiography in 6 weeks and instructions that he will not be able to perform competitive cycling again. Should his dissection extend at that period of time or patient become symptomatic, stent placement would be considered. Patient was to continue the Aspirin and Clopidogrel until the repeat angiography was performed. Patient was discharged with plans to follow up with a cardiologist in his home state. Multiple follow-up phone calls made us unable to reach the patient and he was subsequently lost to follow-up. |
A 17-year-old boy with history of fall from height of approximately 15 meters presented to our institution after receiving primary care at another hospital. At presentation, he was conscious, hemodynamically stable maintaining oxygen saturation at 98% on room air with no visible signs of respiratory distress. His Glasgow coma scale (GCS) was 15, and was able to move all four limbs. The patient had sustained open fracture both bone left leg along with fracture right ankle.\nChest radiograph showed no intrathoracic injury with normal lung parenchyma. Computed Tomography showed burst fracture of fifth lumbar vertebra with canal compromise () and ruled out any injury to head, cervical spine, thorax and abdomen. After primary care, patient was admitted to the orthopedic ward for spine stabilization surgery and surgery for lower limb fracture.\nOn day four post-admission, an emergency consultation call was sent to our intensive care unit (ICU) in view of patient’s deteriorating status. When seen, he was grossly pale and febrile at 101 F, pulse rate of 140 per minute, systolic blood pressure of 80 mmHg and respiratory rate of 32 per minute maintaining oxygen saturation around 90% on oxygen face-mask. Patient’s GCS was 15. Chest auscultation revealed bilateral diffuse coarse crepitation and he was immediately transferred to the ICU. Initial arterial blood gas (ABG) showed partial pressure oxygen (pO2) of 49mm Hg on oxygen by face mask. Patient was intubated, sedated, paralyzed and put on mechanical ventilation with initial settings of volume assist control and high positive end expiratory pressure (PEEP). Central venous catheter was secured in right internal jugular vein under ultrasound guidance. An arterial line was secured in right radial artery for invasive blood pressure and arterial blood gas analysis. Chest radiograph showed bilateral fluffy opacities (). Preliminary blood investigations were mostly unremarkable except for hemoglobin of 6.8 mg/dl and raised ESR of 44. Fundus exam specific for FES was normal and there was no petechial rash on general examination. Urine for fat globules was positive. Over the next few hours, patient’s hypoxemia worsened requiring higher fraction inhaled oxygen (FiO2) of up to 0.8 and PEEP of 16 cm water. A decision was made to turn the patient into prone position after discussion with the orthopedic surgery team in view of unstable lumbar spine fracture. Patient’s family were informed about the specific risks and benefits of prone positioning, particularly in a patient with pre-existing unstable lumbar spine fracture and a written consent for the same was obtained. Positioning was done with the help of 5 trained ICU staff, using logrolling technique for turning the patient lateral followed by a 6th member placing the spinal board under the patient. After securing the patient on the spinal board, he was then shifted to one edge of the bed while the head gel support, chest and pelvic roll were placed in position. Patient was then shifted to prone position and spinal board was removed. Patient’s hands were abducted and placed next to the head and all the pressure points were padded using pillows and cotton rolls.\nOver the next 16 hours, we kept our patient sedated and paralyzed in prone position during which he received targeted fluid therapy and remained hemodynamically stable. Wake up test and pupil examination were done at regular intervals. Serial ABG’s showed dramatic improvement with Fio2 requirement decreasing to 0.4 and PEEP of 8 following which patient was repositioned supine. Two units packed red cells were transfused while the patient was prone. Chest radiograph showed resolving lung infiltrates and we decided to electively mechanically ventilate our patient for next 24 hours in supine position. Weaning was started next day and patient was extubated a day after and transferred back to ward 24 hours later.\nThe patient later on went on to have multiple surgical procedures for injuries to spine and bilateral lower limbs. He was then followed for 8 weeks in the out-patient department following discharge during which he recovered well without any neurovascular deficit. |
An 86-year-old female with a history of metastatic ovarian cancer presented to the ED with painful bilateral lower extremity edema and a left lateral leg ulceration. Her metastatic ovarian cancer had been diagnosed by malignant pleural effusion five months earlier, and she had completed neoadjuvant chemotherapy with carboplatin and Taxol approximately one week prior to this presentation. She was admitted to the hospital and started on cefazolin for left lower extremity cellulitis on hospital day one.\nOn admission, plain films and ultrasound did not reveal any evidence of osteomyelitis, fracture, DVT, or abscess to the left lower extremity. On exam, she had 3+ pitting edema below the knee bilaterally as well as chronic venous stasis changes. The patient also had a venous ulcer (approximately 2 cm in diameter) on the anterolateral aspect of the distal third of her left lower leg. At the time of admission, this venous ulcer had some serous weeping but no purulent drainage or fluctuance on examination. Her initial Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was 4, suggesting a low risk for necrotizing fasciitis; however, on hospital day 3, her CRP began to uptrend and she became febrile. At this point, her antibiotics were switched from cefazolin to vancomycin to cover MRSA.\nOn hospital day five, the patient was noted to have a new erythematous area over the anterior left knee, inferior to the patella (). Ultrasound revealed a small fluid collection superficial to the patellar tendon in the infrapatellar region measuring 3.3 × 2.5 × 0.4 cm (). The infrapatellar bursa was aspirated and sent for culture. The patient was started on piperacillin-tazobactam, given the patient's immunocompromised status and subsequent risk for atypical and gram-negative organisms.\nAn MRI was performed on hospital day seven (this was delayed due to the patient's pacemaker) but did not reveal any evidence of osteomyelitis. The patient was clinically improved after starting piperacillin-tazobactam, and vancomycin was discontinued on hospital day seven. On hospital day eight, aspirate cultures returned with Pseudomonas aeruginosa; she was stable for discharge at that time and was sent out with a ten-day course of levofloxacin (culture was pan-sensitive) and close follow-up with infectious disease. |
A 36-year-old male patient was admitted to our hospital with the complaint of non-specific chest pain. He had a history of smoking and hypertension, potential risk factors for coronary artery disease. Also his father at the age of 46 years had died suddenly with unclear etiology. At the time of the evaluation, the patient had been on antihypertensive therapy with beta blocker for 2 years. The chest pain was not typical for angina pectoris but the patient was very nervous due to his family history.\nThe physical examination did not reveal any abnormal findings. Blood pressure was 140/90 mmHg and pulse 72 beats/min. Cardiac and lung auscultation were normal. The electrocardiogram (ECG) and chest X-ray were normal. Routine blood and biochemical laboratory tests were within normal limits. Transthoracic echocardiograpic examination revealed normal left ventricular size and contractility. The left ventricular ejection fraction was 65%. For further evaluation an exercise test was required but this was not possible as the patient had recently undergone knee surgery for meniscopathy. Therefore, to evaluate chest pain and to detect or rule out coronary artery disease, the patient underwent computed tomography coronary angiography (CTCA). CTCA was performed using a 64 slice CT scanner (Siemens Sensation 64, Germany). Scan parameters were as follows: Slice collimation, 32 × 2 × 0.625 mm; rotation time, 0.33 mins; tube voltage, 120 kV; tube current, 600 mA; and pitch, 0.2. The average heart rate was 71 bpm during the scan. The scan time was 7 sec. CT angiography was triggered automatically by the arrival of the contrast bolus (automatic bolus tracking). A prescan was taken at the level of the aortic root and a region of interest (ROI) was placed on the ascending aorta. As soon as the signal density level in the ascending aorta reached the predefined threshold of 120 Hounsfield units (HU), the scan started. We injected 80 ml nonionic contrast medium (Iomeron 400/ml; Iomeprol, Bracco, Italy) at a flow rate of 5 ml/s in the left antecubital vein. This was followed by a 40 ml saline chaser bolus at a flow rate of 4 ml/s to wash out contrast from the right ventricle. During the scan, the ECG was recorded simultaneously. The reconstruction interval for the coronary arteries with the fewest motion artifacts was determined (images at 75% of the R-R interval) and used for further analysis. For reconstruction of axial images, we used a slice thickness of 0.75 mm and a slice width of 0.5 mm. A medium soft-tissue reconstruction kernel (B30f) was used for reconstruction. For post-processing, an external workstation (Leonardo, Siemens, Germany) was used. In addition to the transverse source images, multiplanar reformations (MPRs), curved MPR images, maximum intensity projections (MIPs), and volume rendered (VR) images were utilized for the evaluation. VR reconstructions depicted the vascular anatomy well and were used for 3-dimensional (3D) orientation.\nCoronary CTA demonstrated an anomalous left anterior descending artery (LAD) arising from the same ostium with the right coronary artery, which coursed downwardly along the interventricular sulcus, and another LAD arising from the left main coronary artery, which spread to the anterior wall of the left ventricle toward the left ventricular apex [Figures and ]. The right coronary artery (RCA) and the left circumflex artery (LCX) were normal. There were no significant coronary stenoses or occlusions. However, the anomalous LAD was coursing between right ventricular outflow tract and aorta which should be considered to be critical because of the potential to provoke myocardial ischemia or even sudden cardiac death []. These CTCA findings were consistent with double LAD, with one vessel arising from the ostium of right coronary artery and the second one arising from the left main coronary artery. The patient was informed about the anomaly and the potential complications. He did not want to undergo a surgical revascularization. He is now on follow up with no signs of myocardial ischemia. |
A 64 year old Caucasian male presented to the Emergency Department with haematemesis and melaena for 3 days. He was a known smoker with no history of analgesic or alcohol abuse. 28 years previously, he had sustained a stab injury to left hypochondrium for which he was managed conservatively.\nOn examination he was pale, tachycardic and hypotensive (pulse rate of 106/min, blood pressure of 94/55 mm of Hg and saturating 100% on air). Systemic examination revealed no abnormality except black tarry stools in the rectum.\nHaemoglobin concentration on admission was 5.9 gm/dl, with a serum urea concentration of 16.2 mmol/lit and serum creatinine of 116 mmol/lit. Liver function test including clotting profile, auto antibody and hepatitis B & C screen were normal. A chest radiograph done revealed opacity in the left lower zone (Fig ).\nHe responded transiently to fluid resuscitation with the haemoglobin concentration falling to 4.9 gm/dl. An urgent upper gastrointestinal endoscopy was done, which revealed the presence of antral erosions in the stomach and complex gastricfundal varices. No therapeutic intervention was performed.\nAn ultrasound scan of abdomen revealed an enlarged spleen (15.2 cms), portal vein measuring 11 mm in diameter (upper limit of normal) and prominent veins noted at the splenic hilum. The liver and biliary tree appeared normal.\nA computerised tomography scan of abdomen in arterial and venous phase confirmed the presence of a diaphragmatic tear and the spleen to be lying in the left hemi thorax (Fig ). Considerable number of varices was demonstrated in the splenic hilum and perigastric region. The portal vein, its confluence with the splenic vein and the superior mesenteric vein were patent. The appearances of the splenic vein on the scan were consistent with thrombosis.\nA diagnosis of left sided portal hypertension as a result of isolated splenic vein thrombosis secondary to trauma causing a diaphragmatic tear and splenic herniation into the left hemi thorax was made. The patient was offered splenectomy with diaphragmatic repair to treat his splenic vein thrombosis and gastric varices.\nThe patient declined to have surgery and self discharged himself against medical advice. |
The patient is a 34-year-old man with eight class education. He was referred to a psychiatrist on behalf of his company because of decreased job performance due to obsessive behavior.\nThe patient was diagnosed with obsessive-compulsive disorder (OCD) in Axis I and border intelligence in Axis II accordance with Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition diagnostic criteria. Fluoxetine began with a 10 mg daily dose and had reached 60 mg/day 5 months later. Symptoms of OCD showed an appropriate response to treatment, but the patient complained of decreased libido, so bupropion was added to the treatment at a dose of 75 mg daily.\nThe next morning, about 11 h after reception of the first dose of bupropion, the patient referred to the emergency room while complaining of muscle contractions in the right side of the face, neck, and shoulder. Biperiden was intramuscularly injected to the amount of 5 mg, and the patient was referred to a psychiatrist due to lack of response. The patient's head was in a state of extension. During the spasms episode, his eyes turned upward and the patient was unable to make eye contact. There were no other signs such as fever, and neurological tests were normal. There was no family history of head trauma, seizure or substance abuse, or family history of psychiatric disorders and movement disorders. With respect to previous psychiatric history and the diagnostic check, neither conversion nor malingering disorder was raised for the patient. Secondary acute dystonia diagnosis was connected to bupropion, and an abnormal involuntary movement scale (AIMS) was done. The AIMS score was 22. Thus, biperiden was intramuscularly injected to the patient to the amount of 7.5 mg and the patient's symptoms were resolved after about 30 min. Bupropion treatment was halted.\nComplete blood count, Vitamin B12 level, folic acid level, ferritin, biochemistry, and screening for Wilson's disease were performed. Cranial magnetic resonance imaging was performed to rule out secondary causes of dystonia and showed no pathologic point. At follow-ups 24 h, 2 weeks, 4 weeks, 8 weeks, and 12 weeks later, no recurrence of symptoms of dystonia occurred. Treatment and follow-up of the patient in the outpatient clinic still continue, and the patient is under treatment with fluoxetine up to 50 mg/day. |
A 79-year-old female presented to our institution with a lump on her left buttock that had appeared one month previously. Physical examination revealed a dome-shaped tumor, 22 cm × 14 cm in diameter. The tumor was elastic, hard, and asymptomatic. No regional lymph nodes were palpable. Magnetic resonance imaging (MRI) demonstrated a multilobular intermuscular mass on the left buttock (Figures –). The tumor was attached to the proximal femur, and the sciatic nerve was also involved. Abnormal signal extension along the muscle fascia of the gluteal muscles was obvious. An excisional biopsy showed spindle-shaped tumor cells in the loose myxoid stroma. The nuclei of the tumor cells were slightly hyperchromatic and irregularly shaped. Only a few atypical mitosis and necrosis were found. Based on the histopathological features, the tumor was diagnosed as myxofibrosarcoma (Figures and ). To determine the histological grade, the FNCLCC tumor grade was assigned using a modified updated version of the original FNCLCC system based on three criteria: tumor differentiation, the mitotic index, and tumor necrosis []. A score was attributed independently to each parameter, and the grade was obtained by adding the three attributed scores. Lesions with a total score of 2 or 3 were classified as grade 1, whereas total scores of 4 or 5 and 6 were deemed grade 2 and grade 3, respectively []. In this case, the histological grade was determined as grade 1. As the tumor was attached to the proximal femur and the sciatic nerve, hip disarticulation was necessary to achieve a tumor-free margin. However, we speculated that postoperative radiation therapy with limb-sparing surgery could prevent the local recurrence of an extremely low-grade myxofibrosarcoma. In addition, we hesitated as such a radical procedure would lead to serious functional loss. Therefore, we performed limb-sparing surgery. The tumor was resected with the gluteal medius muscle, the gluteal maximum muscle, and the adductor muscle. The tumor was stripped from the proximal femur. The sciatic nerve was preserved by removing it from the tumor. As the tumor was exposed intraoperatively, the surgical margin was determined as grossly positive. The histological grade of the resected tumor was also determined as grade 1 (Figures and ). To prevent local recurrence, 60 Gy of postoperative radiation was delivered. The postoperative period was uneventful; however, 15 months after the operation, an MRI showed local recurrence (). A plain X-ray taken for the evaluation of her left shoulder pain showed a pathological fracture and metastatic bone tumor of the left clavicle (). A total body computed tomography scan documented multiorgan dissemination including the skeletal bones, lung, whole spine, liver, subcutaneous layer of abdomen, and paravertebral muscles (Figures –). To control the pain due to a pathological fracture of the clavicle, a partial claviculectomy was performed. Histological grading was still grade 1 (Figures and ). There was no progression to a high-grade lesion. To prevent spinal cord injury due to a pathological fracture of the spine, radiation therapy was delivered to the whole spine. However, the patient's serious physical status did not permit further chemotherapy, and she died 8 months after diagnosis of remote metastases due to rapid tumor progression. |
Sixty-three-year-old woman presented with multicentric left breast cancer requiring mastectomy. She is 69 inches tall with a weight of 215 pounds and a corresponding BMI of 31.8 kg/m2. She has a history of inferior pedicle breast reduction surgery performed 20 years ago (Fig. 1). Despite her previous reduction, she has a very large breast volume and footprint that will be difficult to replicate on the reconstructed left side. She desires nipple preservation and wants to keep her native right breast. We proceed with a left nipple-sparing mastectomy through her previous vertical limb and immediate prepectoral reconstruction using a full-height variable-projection tissue expander (width = 16 cm, height = 16.5 cm, projection = 6.8 cm, and volume = 850 ml) with anterior coverage using an acellular dermal matrix. Twelve weeks later, we exchange her tissue expander for the largest anatomical implant available, a full-height extra-projection 775 ml implant (width = 15.5 cm, height = 16 cm, and projection = 7.1 cm). She also undergoes a contralateral reduction of 300 g to achieve better symmetry. The final result is shown in Figure . Despite using the largest and tallest implant available and reducing the right breast by an additional 300 g, the entire upper pole of the left breast was depleted with significant size asymmetry between the breasts. We discussed multiple sessions of lipofilling to fill this defect but felt that an autologous flap would be more definitive. The LICAP flap was chosen to reconstruct the upper pole of the left breast. An intraoperative photograph is shown in Figure , where an extended flap is dissected based off the known perforators that arise anterior to the latissimus muscle at the level of the inframammary fold as previously described. This flap is rotated on its pivot point and used to reconstruct the upper pole of the breast by suturing it to the underlying pectoralis muscle. The final result 6 months after surgery is shown in Figure , where we have reconstructed the upper pole of her breast with the LICAP flap and have acceptable symmetry between the 2 sides. The patient is discharged the same day, and drains are removed on postoperative day 4. |
A 67-year-old woman presented to our hospital with a 2-day history of pain and a feeling of coldness in her right hand. In her past medical history she had not received any treatment for dyslipidemia. There is no other medical history or smoking history. Upon physical examination she had no heart murmur or leg edema; her blood pressure was 154/96 mm Hg (left brachial), and oxygen saturation of peripheral artery was 96% at room air. Her right hand was pale and the right radial artery was pulseless. An electrocardiogram (ECG) showed sinus rhythm at 79 bpm. The serum D-dimer level was slightly increased (2.18 µg/mL). Computed tomography (CT) scan confirmed thromboembolism in the distal part of the right brachial artery, left pulmonary artery and right kidney infraction (). Cerebral infraction and stenosis of the main cerebral artery were not detected by the head magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA).\nDoppler sonography of right upper limb revealed the disappearance of blood flow at the distal part of the brachial artery, and collateral circulation to the radial artery from the brachial artery. In this case, multiple thromboemboli occurred in both the systemic and pulmonary circulation. Thus, we suspected paradoxical thromboembolism. Transesophageal echocardiogram and venous ultrasonography findings showed the presence of a PFO and DVT (). Thus, we diagnosed paradoxical embolism due to PFO. Photoplethysmogram (PTG) of her right hand was very slow on admission (). The condition of her right upper limb had been relieved from 2 days previously due to the development of collateral circulation. Therefore, we thought that neither emergency operation nor catheter intervention for thrombectomy was necessary in this patient. We started treatment with urokinase (UK) 240,000 U/day intravenous injection (IV) and unfractionated heparin (UFH) continuous IV (target activated partial thromboplastin time (APTT) 60 - 80 s). As her condition and the serum D-dimer level were improving we started catheterization at day 7 after admission (); however the thrombus still remained in the brachial artery. Although her pain was relieved, her right hand was still cold and PTG of the right hand at day 7 was slow. For her outpatient care, based on the viewpoint of providing rapid anticoagulant therapy within the therapeutic range, having longest periods of initial intensive therapy we chose treatment using ribaroxaban (15 mg, two tablets twice daily) (). Three weeks after discharge from the hospital, her right hand had become warm and the serum D-dimer level and PTG were normalized (). From that time, we changed ribaroxaban to the maintenance dose (15mg, one tablet once daily). Three months after discharge, Doppler sonography and MRA of her right arm confirmed the achievement of reperfusion of her brachial artery (). |
A 32-year-old man complained of left aural fullness in August 2005. He had no past medical or family history. In addition, he experienced left hearing loss and left tinnitus in July 2007. He consulted an ENT practitioner, but his symptoms were not improved. Thereafter, left temporal bone tumor was revealed in a computed tomography (CT) scan. There was no history of headache, nausea, vomiting, or other neurological symptoms. He was referred to our hospital for further evaluation and management in September 2007.\nExamination of the ear revealed a bulging, subcutaneous bulging tumor from the flaccid part to the umbo of the left tympanic membrane (). Audiologic examination demonstrated conductive hearing loss of the right ear with an air-bone gap. The cranial nerve examination showed that the cochlear nerve was intact.\nCT of the temporal bone showed a soft-tissue-density specification indicating bone destruction, 3 cm in size, at the left temporal bone with massive extension to the mastoid antrum and the temporomandibular joint (). Coarse calcification was seen inside the tumor. The tumor extended to the anterior wall of the left external auditory canal, resulting in narrowing of the canal. Furthermore, the tumor reached the facial nerve and the middle ear, and ossicles were embedded in the tumor. In the coronal section, the tumor invaded the skull base, and the outline of the tumor showed osteosclerosis with decalcification ().\nImaging examinations using magnetic resonance imaging (MRI) revealed a low-signal-intensity area on T2-weighted images and a nonhomogenous high-signal-intensity area on T1-weighted images that measured 3 cm in diameter at the left temporal bone. After intravenous gadolinium, the mass showed unequal enhancement (). In the coronal section, the border between the tumor and the left mandible head was indistinct. In addition, the dura mater of the middle cranial fossa appeared to be involved by the tumor based on the dural enhancement ().\nThe patient subsequently underwent surgery to remove the lesion. We confirmed that the tumor did not reach the stapes, and therefore the incudostapedial joint was detached. After mastoidectomy, a part of the tumor around the incus was examined by frozen section. We removed the tumor using transmastoid and middle fossa approach (). The diagnosis of the frozen section during the operation was GCT. We peeled the tumor from the middle fossa dura. We removed the incus and the head of the malleus and confirmed the widely exposed horizontal portion of the facial nerve. The entire tumor was then extirpated. Ossiculoplasty was performed with the columella on the stapes. After the operation, the patient experienced mild facial palsy of the left side, but the facial palsy had almost completely recovered by 12 months after the surgery. Postoperatively, otoscopic examination became normal. The pure tone audiometer showed that his hearing improved to a normal level. No clinical or radiological evidence of tumor recurrence was detected for 4 years.\nPathological examinations from specimens of the tumor revealed round and spindle-shaped mononuclear cells admixed with numerous multinucleated giant cells (), characteristic of giant cell tumors. |
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