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A 40-year-old man with a painless swollen right cheek and right eyelid was referred to our department. The swelling had progressed gradually over the course of at least1 month and the patient had shown no improvement despite antibiotic treatment. The patient had clear rhinorrhea and a history of close contact with sheep and dogs. Physical examination revealed a well-shaped, firm, and fluctuant swelling with subcutaneous penetration without any evidence of inflammation. There was no tenderness in the affected area. Anterior rhinoscopy revealed lateral bulging of the right nasal wall. The general condition of the patient was otherwise normal. Computed tomography imaging (CT) of the face showed a loculated soft tissue density in the right maxillary sinus with erosion of the lateral and medial walls, as well as the floor of the orbit. The soft tissue density involved the orbit and compressed the globe (). The appearance of the suspected mass by CT was suggestive of a cystic mass. Fine-needle aspiration (FNA) was performed, but this failed to provide sufficient material for fluid analysis. Given the compression of the orbit, we decided to resect the mass.\nA Caldwell-Luc approach was adopted for excision of the cystic mass ().\nThe mass in the maxillary sinus was yellow-white in color and elastic in nature, suggesting parasitic disease. We carefully removed the cystic mass and irrigated the sinus with hypertonic saline a total of three times. The macroscopic appearance of the extracted mass suggested a hydatid cyst. The cyst was enucleated easily, and multiple daughter cysts were found inside the capsule ().\nHistopathologic examination revealed a hydatid cyst consistent with E. granulosus (). A serologic test of the hydatid cyst revealed a positive 1/264 result, which further confirmed the presence of hydatid disease.\nAn abdominal ultrasound and CT scan of the abdomen and thorax ruled out visceral or pulmonary involvement in the hydatid disease, which confirmed the diagnosis of a primary hydatid cyst of the right maxillary sinus. The patient was in a healthy condition after the operation and the swelling of the right cheek and surrounding areas were resolved rapidly. A 2-month course of albendazole (800 mg/d) with a 2-week interval after the first month was prescribed to the patient for prophylaxis against recurrence. The patient did not show any evidence of recurrence at follow-up 12 months after the operation.
A 52-year-old man with no particular medical history developed sudden-onset left occipital headache, dizziness, dysphagia, and right-sided hemiparesthesia and was transported to another hospital. Head magnetic resonance imaging (MRI) revealed a left lateral medullary infarction due to the left VAD, and he was admitted to that hospital. Conservative treatment was performed, and the patient had an uneventful course, but at his and his family’s request, he was transferred to our hospital 4 days after onset.\nOn admission to our hospital, head MRI was performed again. The MRI findings were the same as those observed at the other hospital; that is, a left lateral medullary infarction due to the left VAD was observed, and there were no abnormal findings in the right vertebral artery [ and ]. Antiplatelet or anticoagulant agents were not administered, and conservative treatment with administration of a free radical scavenger and mild volume expansion was performed. Because his blood pressure remained at approximately 130/80 mmHg, antihypertensive agents were not administered. He had an uneventful course with no exacerbation of symptoms.\nOn day 9 after onset, the patient developed sudden disturbance of consciousness and was found lying on the side of his bed by a nurse. Head computed tomography (CT) showed SAH, mainly in the posterior cranial fossa []. Three-dimensional CT angiography revealed a dissecting aneurysm with active extravasation of contrast agent in the right vertebral artery distal to the posterior inferior cerebellar artery []. Under general anesthesia, emergency internal coil trapping was performed for the dissecting aneurysm of the right vertebral artery [ and ]. The left vertebral artery angiography after internal trapping showed moderate stenosis of the left vertebral artery corresponding to the dissection site, but the basilar artery was visualized in antegrade with no delay of blood flow, endovascular treatment for this stenotic portion was not performed []. After the procedure, there were no ischemic symptoms due to vasospasm after SAH. The patient’s condition improved gradually, and he was discharged with a modified Rankin Scale score of 1. The stenosis of the left vertebral artery due to dissection had resolved on head magnetic resonance angiography (MRA) performed approximately 1 month after onset []. Up to the present, approximately 2 years after onset, no new dissections have occurred in the vertebral artery on either side.
The patient, a 32-year-old Caucasian woman, presented to the West Virginia University Hospital Emergency Department via Emergency Medical Services. The patient had been at her usual baseline state of health with no significant past medical history prior to visiting the chiropractor for neck adjustment earlier that day for tension like soreness. The patient underwent neck manipulation after which she immediately complained of neck pain, diaphoresis, and proceeded to experience cardiac and respiratory arrest. Emergency Medical Services was called, and cardiopulmonary resuscitation was performed with one round of epinephrine administered. It was reported that the patient was pulseless and apneic for 3 minutes prior to EMS arrival. The patient was intubated on transport and her Glasgow Coma Scale score was 3T prior to arrival. Mean arterial blood pressure was 80 with palpable femoral pulses at arrival to the emergency department. Upon arrival in the emergency department, a CT stroke protocol was performed which demonstrated bilateral severe distal cervical vertebral artery dissections with acute thrombotic emboli seen in the left cervical vertebral artery ( and ). This was accompanied by complete occlusion of the basilar tip including the proximal posterior cervical arteries.\nThe patient received an initial bolus of intravenous tissue plasminogen activator (IV rtPA) at this time and the decision was made to proceed with endovascular intervention given the recent onset of occlusion. The patient was brought to the neurovascular angiography suite and femoral access obtained. Angiography of the left vertebral artery demonstrated severe dissection involving the distal cervical vertebral artery segments at the C1-C2 level with presence of sub occlusive thrombi. There was an occlusive clot in the left Posterior Inferior Cerebellar Artery (PICA). Intracranial imaging demonstrated occlusion at the basilar apex with absent filling into the right Posterior Cerebral Artery (PCA). There was occlusion of the distal left PCA. Angiography of the right vertebral artery demonstrated severe dissection of the distal cervical vertebral artery at C1-C2 with the presence of trickle-like flow into the vertebrobasilar junction. No filling was observed in the right PICA territory (). At this point, it was decided that the left vertebral artery offered the best access to the basilar trunk.\nSubsequently, distal aspiration was begun with a Penumbra 5 Max ACE distal aspiration catheter which initially demonstrated slow flow through the suction tubing. The 5 Max ACE was withdrawn into the proximal basilar artery until flow was seen within the suction tubing. Repeat angiography at this time demonstrated recanalization of the basilar apex and proximal PCAs. TICI3 perfusion was seen in the right PCA. Occlusive clot remained in the left distal P2 segment. Given the large size of the PCA, timing of events, and patient’s age, the decision was made to attempt clot retrieval of this. At this time, the Trevo ProVue microcatheter was navigated into the left distal PCA distal to the clot. The Trevo 4 mm × 30 mm stent retriever was deployed for approximately 3 minutes. The suction canister was attached, and the stent retriever was pulled with distal aspiration. No significant recanalization was achieved with what amounted to TICI0 perfusion to the left PCA territory. No further attempts were made as it was believed that the left PCA territory had completed its infarction.\nFollowing completion of endovascular therapy, the patient was taken for immediate MRI Brain with and without contrast for assessment of brainstem integrity and cerebrovascular status prior to transport to the intensive care unit. MRI demonstrated extensive areas of restricted diffusion accompanied by perfusion abnormalities consistent with acute infarction of the posterior circulation, specifically within the bilateral cerebellar hemispheres, right medulla, pons bilaterally, midbrain, thalami, and left occipital lobe (). The following day, additional CT Brain imaging was acquired and demonstrated signs of elevation of intracranial pressure with hydrocephalus, worsening of cerebral edema diffusely, hemorrhagic transformation of the left occipital lobe, continued infarct evolution within the posterior circulation, and cerebellar tonsillar herniation.
A 45-year-old man was ejected in a motor vehicle collision and taken to Community Regional Medical Center, the region's Level I trauma center. He was hemodynamically normal on arrival. His history included a previous motor vehicle collision approximately 20 years prior, at which time he underwent a left thoracotomy and splenectomy. The patient was unable to recall any other details of injuries or surgical interventions from this prior accident. Examination was significant for a left thoracotomy scar. Computed tomography (CT) scan revealed a large left-sided diaphragmatic hernia. His other injuries were relatively minor and did not require operative intervention. The diaphragmatic hernia was suspected to be chronic given the lack of any significant trauma, symptoms, and the history of a suspected previous repair. Given the likely chronic nature of the hernia, the decision was made to perform the repair semielectively.\nOn hospital day 2, the patient was taken for a robotic-assisted, laparoscopic repair of his diaphragmatic hernia. An abdominal approach was chosen due to surgeon discretion, as the patient had both previous abdominal and thoracic surgeries and thus adhesions were likely to be encountered with either approach. At exploration, the hernia was found to be lateral and posterior, and a significant amount of scar tissue was dissected further confirming its chronic nature. The diaphragm was unable to be completely primarily repaired, and a Phasix™ ST Mesh was used as an interposition graft with Prolene sutures to secure it to the diaphragm.\nInitially, the patient did well; however, on postoperative day 5, he had an episode of desaturation and a large amount of hemoptysis. A CT was done and showed significant intra-abdominal and mediastinal air with the suspicion of tracheobronchial injury. The patient was intubated and a bronchoscopy was performed, which revealed blood in the left bronchus but no discrete lesion. A repeat bronchoscopy the following day showed large amounts of clot and mucous casts from the left lung, but again, no discrete defect.\nWhile in the ICU, he had multiple episodes of bilious emesis that filled his ventilator circuit. The first episode occurred approximately two weeks following his index operation. He had a worsening chest X-ray and P/F ratio, suggestive of ARDS. Multiple modes of ventilation were tried to improve his PaCO2. In an effort to decrease the bilious emesis, his endotracheal tube was changed and nasogastric tube replaced.\nAs he continued to have repeated episodes of bilious emesis filling his ventilator circuit, a diagnosis of a gastrobronchial fistula was entertained. For further workup, an abdominal CT scan with oral contrast was ordered and demonstrated contrast in the left lower lobe (). This was attributed to aspiration by the radiologist. The pulmonology service was consulted for repeat bronchoscopy to evaluate for possible gastrobronchial fistula. They declined as they had never heard of the diagnosis and felt that the trauma service should be investigating more likely causes. An upper gastrointestinal (GI) series was negative. An esophagogastroduodenoscopy (EGD) was performed by the primary team and clearly showed two areas with air blowing into the stomach, in synchrony with the ventilator, along the greater curvature and visible PDS suture in the gastric lumen.\nThe patient was taken to the operating room for a left thoracotomy. The gastrobronchial fistula was identified. It appeared that the previous diaphragm repair sutures had incorporated the diaphragm and the stomach but did not involve the lung tissue. As the left lower lobe was found to be unsalvageable, a left lower lobectomy, wedge resection of stomach, and diaphragm repair were performed. Eventually, the patient's condition improved, and he was able to have a tracheostomy performed and weaned from the ventilator.
A two-week-old female was brought in with an initial complaint of multiple masses that failed to regress since birth. She was born from a nonconsanguineous union and the mother’s pregnancy was uneventful. The baby was delivered vaginally at 37 weeks of gestation and multiples masses were found over the body. The treating physician advised follow-ups on discharge. However, the parents decided to seek more medical help for the child and she was admitted for more investigations.\nOn physical exam, the child was alert and active. She was not jaundiced and no pallor was noted on the extremities. Her vitals were all within the normal range and the birth weight and changes in weight corresponded properly. Two masses measuring 26 mm by 19 mm and 19 mm by 17 mm were observed on the forehead and the scalp (Figures -). One smaller mass was seen on the abdomen, measuring 11 mm by 10 mm (Figure ). All three masses were red and non-hemorrhagic. The parents reported that the size of the masses did not change since birth. No similar family history was found and both parents were healthy. Any hepatosplenomegaly was not observed on palpation nor were any other cutaneous lesions detected. She did not present with any other systemic abnormalities. An ultrasound was performed, and it revealed a normal liver, spleen, and kidneys with no masses or lesions. A Doppler examination also showed a fast-flow vascular lesion, as reported by many other articles []. The full blood count showed normal levels of white blood cells, platelets, hematocrit, and red blood cells.\nThe diagnosis of congenital hemangioma was confirmed and the family was properly educated about the condition as well as possible complications. A wait and watch approach was adopted and the child was scheduled for regular visits at three months. The family was asked to properly monitor the size of the masses and informed that appropriate surgical treatment would be provided if the masses fail to regress or if they continue to grow.
We report the case of an 88-year old man with coexistence of descending TAA and AAA. The patient had a long medical history of arterial hypertension, coronary disease and chronic obstructive pulmonary disease (COPD). He smoked about 20 cigarettes a day until 65 years old. At the age of 72 (in 2001) the patient had coronary artery bypass surgery. During coronary angiography in 2009 two drug-eluting stents were implanted in the anterior descending branch and right coronary artery. At the same time two aortic aneurysms were recognized (descending TAA with diameter 50 mm and AAA with diameter 42 mm) and kept under closed observation. In November 2012 when our patient was 84 years old the endovascular repair of abdominal aortic (54 mm) and left iliac common artery (25 mm) aneurysms with the implantation of a bifurcated Gore Excluder stent graft was performed. Preoperative echocardiography revealed akinesia of the inferior wall basal segment and lateral wall middle segment, with left ventricular ejection fraction (EF) = 47%. The essential aberrance in the preoperative laboratory test was thrombocytopenia with platelet level 96 000/ml. However, the endovascular aortic repair was successful and the patient was discharged on the fifth postoperative day. During subsequent hospitalization in January 2013 the TEVAR procedure of a 58 mm × 70 mm descending, angulated TAA aneurysm was performed. Two segments of Zenith TX2 TAA endovascular graft 40 mm in diameter were introduced through the right femoral approach. The proximal segment, 162 mm long, started just below the left subclavian artery. The distal segment, 198 mm long, overlapped partially the proximal one and finished over the diaphragm where the aorta ran at a right angle to the spine. There were no perioperative complications and the patient was discharged on the sixth day after TEVAR.\nIn 2013 and 2014 control spiral CT scan confirmed complete exclusion of the aneurysm sac. The next examination performed in December 2015 revealed excessive type III endoleak due to disconnection of proximal and distal stent graft segments in the descending TAA. The distal part moved in a lateral and dorsal direction (). Maximal aneurysmal sac diameter was 88 mm (baseline 58 mm × 70 mm). There were no endoleaks or sac enlargement after endovascular aneurysm repair (EVAR) of the abdominal aorta. The patient was qualified for endovascular treatment of type III endoleak due to high risk of aneurysmal sac rupture. After initial angiography through the left femoral access disconnection of Zenith TX2 segments was confirmed. The attempt to introduce a stent graft over the stiff guidewire through the left groin was unsuccessful due to extreme angulation of the descending TAA. We decided to use brachial access, and a hydrophilic guidewire was inserted through the left brachial artery to the femoral artery. The tight guidewire between the left brachial and left femoral artery made it possible – despite the difficulties due to severe descending thoracic aorta angulation – to deliver the low profile Cook Zenith Alpha graft (diameter 42 mm, length 225 mm). The inserted segment successfully sealed the separated thoracic graft ().\nInstead of accurate hemostasis increased bleeding to the left groin and arm drains was observed during the first postoperative hours. Finally a pressure dressings to the both places of access stopped bleeding. The patient was discharged in good general condition on sixth postoperative day. There were no endoleaks detected during monitoring CT scan of the chest and abdomen at 3, 6 and 12 months follow up.
Our patient was a 32-year-old previously healthy female at the 39th week of gestation who accessed the first aim department of a primary healthcare centre of a peripheral hospital for severe dyspnoea and chest pain. Her past medical history did not present other hospitalizations for the same symptoms. Due to the clinical manifestations, the patient was initially treated as a case of pulmonary embolic disease and subjected to a massive anticoagulant therapy. Considering the clinical diagnosis and the child to term, an emergent caesarean delivery was performed in order to avoid foetal complications. The caesarean section was successfully performed under general anaesthesia using Stark's method due to the urgency related to the patient's clinical condition of increasing dyspnoea. Moreover, although the pAVM was still unknown at time of the caesarean section, the execution of spinal anaesthesia seems to be not indicated because of the risk of pAVM association with other AVMs, such as those located in the spinal cord, especially in case of HHT.\nThe foetal outcome showed an Apgar index of 3, 6, and 9, respectively, at minutes 1, 3, and 5; these data are in line with the administration of general anaesthesia and the acute maternal condition of severe dyspnoea.\nTaking into account the foetal weight at birth, it showed a restriction of the expected value. The child weight was in fact 2590 gr at 39 weeks of gestation. However, ultrasounds performed during the pregnancy reported a reduction of the potential foetal growth from the 33 weeks of gestation without any Doppler alteration. This phenomenon should be the result of the chronical adaptation of the pregnancy to the unknown pAVM.\nAs far as the macroscopic exam of the placenta is concerned, a percentage of cotyledons infarcts inferior than 10% was reported.\nConsidering the patient's postoperative course, it showed a subsequently worsening of the clinical conditions, resulting in an acute distress syndrome that required an immediate transfer to the Gynaecology and Obstetrics unit of our structure. Due to the critical care panel and the low clinical conditions, the patient was intubated and housed in the ICU department. Considering the acute distress syndrome, a chest CAT scan was performed highlighting the presence of a left pAVM expanded, associated with a massive hemothorax that compressed the correspondent lung. The vital signs panel showed systolic blood pressure of 70 mmHg, diastolic blood pressure of 35 mmHg, pulse rate of 150/min, pulse oximetry saturation 88% on 100% inspired oxygen, afebrile temperature, and respiratory rate of 40/min. Initial labs revealed normal platelets, normal coagulation panel, and haemoglobin of 7 gm/dL. Critical care panel showed pH of 7.4, pCO2 of 43 mm hg, pAO2 60 mmHg, and saturation of 88%. After the placement of a chest tube, 3 litres of frank blood were removed; this action resulted in a normalization of the blood pressure and improved oxygenation on the monitor. The successive management was the clinical observation of the patient's conditions as well as the vital signs and labs test in order to perform the pAVM embolization when the patient clinical conditions will be stable. After three hours from the drainage, worsening of the patient conditions was observed reporting a severe collapse of the vital signs as well as a decrease of antithrombin III, fibrinogen, and haemoglobin values, with parameters of 33%, 122 mg/dl, and 5.8 gm/dl, respectively. Moreover, considering the postoperative caesarean course, the gynaecologic clinical evaluation showed a low uterine fundus contraction and the presence of conspicuous abnormal lochia. Uterine fundal massage was performed as first approach to solve the uterine low contraction followed by Credè's manoeuvre. Due to the failure of both, a pharmacological treatment was attempted starting with a simultaneous administration of intravenous Oxytocin (10-40 UI per 1 litre saline solution) and intramuscular Methylergometrine (0.2 mg one dose). The latter pharmacological approach involves the use of intravenous Sulprostone (0.5 mg per 1 litre saline solution) that was administered within half an hour from the signs of low uterine contraction and abnormal lochia. None of the previous pharmacological treatments succeeded.\nConsidering the reproductive age of the patients, procedures as appositions of tamponade-balloon and embolization of the uterine arteries were taken into account but were not applicable in order of the unstable and precipitant parameters of the woman. Due to the patient's life-threating condition, a simultaneous surgical intervention of thoracic surgeons and gynaecologists had been necessary to solve the urgency, with the performance of a contemporaneous surgical reparation of the pAVM and resection of damaged left lower lobe (LLL) as well as a preventive hysterectomy to avoid the risk of disseminated intravascular coagulation (DIC). The surgical interventions were performed successfully but intraoperative blood transfusions and administration of antithrombin III and fibrinogen were necessary. The postoperative treatment showed a normalization of vital signs and labs panel as well as patient's clinical conditions. Due to the stable condition of the woman, the anaesthetist established the patient's autonomous breathing. After one week from the intervention, chest CT with intravenous contrast was performed showing a 4 cm area of active contrast. Pulmonary angiography confirmed the presence of a pAVM with feeding branch of a basilar left pulmonary artery supplying aneurysmal AVM and dilated draining vein. Transcatheter embolotherapy (TCE) of the culprit vessel was performed by placement of a nonadhesive liquid embolic agent (Onyx 34®). Repeated chest X-ray and chest CT after one week from TCE showed expansion of remaining left lung and signs of pAVM embolization and pulmonary resection of LLL, respectively (). The patient course was subsequently uncomplicated and the discharging home happened after 14 days. MRI evaluation was performed in order to detect any head AVM but the result was negative. The genetic testing for HHT was not performed during this hospitalization period but the genetic examination performed a few months afterwards showed no association.
A 25-year-old Mexican female with family history of ovarian cancer at her maternal side and personal history of a mass on her right breast clinically and radiologically diagnosed as fibroadenoma 2 years ago. The same mass had a growth of 2 cm so an incisional biopsy was performed at another institution. This was positive for mucinous carcinoma, and the patient was referred to our institution for treatment and follow up.\nAt our institution in the biopsy slides revision, we found a tumor composed of mucinous lakes with numerous tumor cells of medium to large size with a moderate amount of eosinophilic cytoplasm confirming the presence of pure mucinous carcinoma. We could not identify the tumor size and surgical margins by no prior radiological and pathological report. []. The tumor was classified as a Low grade tumor (G1) based on the Scarff Bloom Richardson scale. We did not observe perineural or vascular invasion. On immunohistochemistry the results were 100% and 90% positive for estrogen and progesterone receptors respectively with high intensity, and HER2 receptors negative.\nOn physical examination a surgical injury was seen. Upon palpation, this zone felt indurated, edematous and presented as a rough irregular texture. No other lesions were found. An ultrasound was performed, showing avascular distortion and a heterogeneous mass with microcysts (white arrows) []. MRI showed a hypointense irregular mass that indicates a mild enhancement in contrast sequences and a hyperintense sequence in SPAIR. The evaluation of the lymph nodes was not suspicious for metastasis.\nWith the radiological suspicious of residual tumor and pathological findings accomplished at our institution a partial mastectomy and a sentinel lymph node biopsy was performed. Pathological findings showed a residual mass of 25 mm of pure mucinous carcinoma with negative surgical margins. Sentinel lymph node was negative for metastasis.\nMolecular screening with EndoPredict clinic showed a score of 2.7 classifying it as a low risk, reason why this patient was treated with tamoxifen because her positivity to receptors and low risk showed in molecular assay. The clinical and pathological stage IB was confirmed.
A 49 year old woman complained of abdominal pain in November of 2015 with diagnosis of large uterine fibroid, a myomectomy from within the uterus was performed. No malignancy was associated with the specimens recovered. In December of 2016, the abdominal pain persisted and a mass was palpable on physical examination in the mid-abdomen on the right. Colonoscopy was performed and biopsy showed a moderately differentiated adenocarcinoma of the caecum.\nCT was performed in December of 2016 and the primary right colon malignancy along with bilateral ovarian metastases were imaged. Percutaneous biopsy of the right ovarian mass showed well differentiated adenocarcinoma consistent with a colonic primary. From December of 2016 through December of 2017, the patient was maintained on chemotherapy. Initially, she was treated with FOLFOX. After four cycles, because of neuropathy, the oxaliplatin was stopped. The patient was maintained on 5-fluorouracil and bevacizumab.\nIn December of 2017, a CT documented marked regression of the primary tumor. It was not visible by CT. However, multiple lymph nodes within the mesentery of the distal small bowel were enlarged. Also by CT a pelvic mass showed that the right ovary had increased in size to 15 cm in greatest diameter despite the fact that the primary caecal malignancy was no longer visible by CT.\nOver approximately one month the patient became rapidly more symptomatic with abdominal distention from ascites and an expanding right ovarian mass. On February 1, 2018 the patient underwent cytoreductive surgery and HIPEC in a specialized center for management of peritoneal metastases. At the time of surgery she underwent a greater omentectomy, hysterectomy, bilateral salpingo-oophorectomy, complete pelvic peritonectomy, right colon resection, and small bowel resection. She received HIPEC with mitomycin C and doxorubicin with systemic fluorouracil. In order to determine if the multiple enlarged lymph nodes within the small bowel mesentery were involved by cancer, individual lymph nodes were removed and subjected to histopathologic study by permanent sections at a later time. A portion of each of these lymph nodes was sent for pharmacologic analysis for doxorubicin content. shows the pharmacokinetics of intraperitoneal doxorubicin in peritoneal fluid, mesenteric lymph nodes, and plasma. Clearly, the lymph nodes have taken up large amounts of doxorubicin nearly equal to that which was seen within the peritoneal fluid. The increased amounts of doxorubicin within lymph nodes as compared to the plasma can be measured by the area under the curve ratio. The ratio of lymph nodal tissue concentration times time to plasma concentration times time was 40. The area under the curve ratio (AUC ratio) of peritoneal fluid to plasma was 80.\nAfter the HIPEC chemotherapy and biopsies, an end-to-side coloenteric anastomosis was performed. The patient required a 3-week hospitalization and was discharged eating well and having normal bladder and bowel function. Pathology showed cancer in the right ovary and small amounts of cancer remaining at the primary site. No cancer was present in the mesenteric lymph nodes.
A 12 years old female child reported to the Department of Periodontics, Government Dental College and Hospital, Nagpur complaining of gingival swelling, which caused severe compromise in her speech, mastication and aesthetics and led to considerable disfigurement of her facial profile, owing to the severity of gingival enlargement. The patient reported that the gingival enlargement started with the eruption of permanent dentition but progressed slowly to cover the entire permanent dentition. No single tooth was visible due to enlargement in anterior region whereas only occlusal portions of teeth were visible in posterior region. It was difficult to predict whether the positions of teeth were hampered due to massive gingival mass. However, the panoramic radiograph revealed normal bone height and tooth positioning.\nThe extra oral examination revealed an exaggerated convex profile with highly incompetent lips as the tissue bulge was protruding out of the mouth. The intra oral examination revealed generalised gingival overgrowth including both maxillary and mandibular arches extending into the vestibular areas []. The pink, leathery consistency tissue was hard on palpation and covered complete crowns of the entire dentition. The intra-oral examination confirmed that the patient used to masticate with assistance from the gums, however, no ulcerations were found on the occlusal surfaces of the enlarged tissue. A scanty amount of plaque and food debris was entrapped into the posterior teeth which were partially visible amongst the entire dentition with no significant inflammation present around them.\nRoutine blood investigations were done and values were found within normal range although haemoglobin values was slightly below normal range (8.5 gms%). The blood group examination was also done owing to the need for blood transfusion which was deemed necessary for maxillary arch gingivectomy after complete clinical evaluation. Based on the provisional diagnosis, an incisional biopsy was done and sent for histopathological examination to confirm the diagnosis.\nThe patient's medical history did not reveal any drug induced gingival enlargement or hormonal changes and exhibited no signs of hypertrichosis or mental retardation that could be associated with gingival hyperplasia.\nHer family history was significant because her elder sister (15 years old) also revealed the presence of gingival enlargement but, was not willing for treatment as it was not causing any aesthetic and functional compromise. Her parents did not reveal any evidence of gingival overgrowth.\nThe enlarged tissue was removed by the external bevel gingivectomy under local anaesthesia quadrant wise only in the mandibular arch. Periodontal dressing was applied and chlorhexidine, haematinics and fortified B-Complex vitamins were prescribed for two weeks. For maxillary arch gingivectomy was performed under general anaesthesia. The patient was admitted to the hospital a day before the surgery where she was pre-medicated with requisite antibiotics and anti-inflammatory agents (Amoxicillin and Cloxacillin 500 mg, in divided doses 12 hourly and Piroxicam 20 mg, 8 hourly). There was significant blood loss during the surgery which was compensated by putting the patient on blood transfusion (2 pints of 350 ml each over 2 days). The post-operative instructions were reinforced and a custom made acrylic stent was placed against the surgical area containing periodontal dressing. The patient was discharged on the third day and was asked to continue with the medications till 7 days.\nThe histological evaluation of the section revealed moderately dense to highly dense collagenous connective tissue with collagen bundles arranged in haphazard manner. The connective tissue was relatively avascular with scanty inflammatory cells infiltrate. The overlying epithelium was hyper-plastic with enlarged rete ridges. The histopathological picture was suggestive of gingival fibromatosis [].\nPost-surgical healing was uneventful. The stent contained periodontal dressing was removed after one week. The surgical area was profusely irrigated with Betadine and normal saline. Post-operative instructions were reinforced and the patient was recalled after two weeks, one month and three month intervals for post-surgical evaluation. Post surgically her facial profile improved dramatically and the patient was satisfied with the result [].
A 26-year-old female sustained an injury to her right knee at a dance tryout. Radiographs and MRI were obtained for the evaluation of the acute injury (Figures and ). Imaging demonstrated a complete tear of her anterior cruciate ligament, terminal sulcus impaction injury, and a corner fracture of the posterior aspect of the lateral tibial plateau.\nThe patient subsequently underwent an ACL reconstruction utilizing bone-patellar tendon-bone autograft at an outside institution with an uneventful recovery. The initial postoperative radiographs from the outside institution were unremarkable ().\nApproximately 6 months after the ACL reconstruction surgery, the patient had reinjured her right knee from a slip and fall on the ice. Radiographs were obtained at this time (), with the initial interpretation by an outside institution, read as being unremarkable. The patient's symptoms persisted; therefore, A MRI was obtained which demonstrated a new lesion in the anteromedial aspect of the tibia, adjacent to the tibial tunnel (). No specific differential diagnosis was given by the outside radiologist. Additional radiographs were obtained approximately 10 months after the ACL reconstruction due to the patient's persistent symptoms. Radiographs from this time () demonstrated slight interval increase in the size of the medial tibial lesion. At this time, the patient was treated with steroid injection for her symptoms by the outside institution.\nThe patient was referred to our tertiary referral center for further evaluation of the bone lesion and associated tenderness over the anteromedial aspect of the patient's knee in the area of tibial tunnel. Review of outside films by a musculoskeletal radiologist and an orthopedic surgeon in our center included a differential diagnosis of infection, benign fibrous lesion such as fibrous dysplasia, and nonossifying fibroma. At this point, the patient was lost for further follow-up at our institution due to combination of patient's status of serving in the military and living out of state. Approximately 1 year and 7 months after the ACL reconstruction, the patient was referred back to our tertiary referral center for further assessment and treatment of the medial tibial bone lesion. Radiographs and MRI from the outside institution (Figures and ) showed significant progression of the lesion. A biopsy was recommended at this time. We discussed with the patient that the most likely diagnosis was a giant cell tumor, but it was also possible that this was a malignancy due to the aggressive nature of the tumor over the course of one year. We counseled the patient on our plan to proceed with treatment of the tumor if histology confirmed a giant cell tumor, or we would stage and plan surgical intervention at a later date if histology was consistent with a malignancy. Intraoperatively, frozen section of the lesion was consistent with giant cell tumor. At that time, we proceeded with curettage and cementation of the tumor as well as removal of prior ACL screw. Due to extension of the tumor into the medial articular surface, posterior cortex, and insertion site of the medial collateral ligament (MCL), prophylactic fixation of the right tibia and repair of the MCL with soft tissue mobilization and transfer were also performed (). Hematoxylin and eosin stain histological slide demonstrates characteristic giant cells ().\nApproximately 3 months after the giant tumor curettage and cementation, MRI and CT were obtained as a baseline. These studies demonstrated a new area of uncemented intramedullary cavity anterior to the cement (). Given the short interval since prior MRI (), findings are clinically thought to represent postoperative inflammatory change. Subsequent follow-up radiograph, which is approximately 4 months after the curettage and packing surgery, shows stable surgical change (). The patient continues to show clinical improvement in her symptoms and continues with clinical and imaging surveillance.
A 30-year-old woman sought medical attention in our department for a recurrent iliac tumor. Ten years previously, she had felt a growing lump and pain in her right iliac bone that intensified with walking. She was admitted to a local hospital, where the surgeons removed an osseous tumor from her iliac bone, and the subsequent pathological studies showed that it was a low-grade chondrosarcoma. Three years before presentation, the patient felt pain while walking and noticed a similar lump in the previously operated area. Fifteen months before presentation, she visited her local hospital because of increasing pain and growth of the lump in her right iliac bone. Pathological studies following fine needle aspiration showed the same diagnosis of low-grade chondrosarcoma. Three months later, the patient visited our department for more specialized surgical treatment. Physical examination after admission showed a 4- × 4- × 3-cm lump with a clear boundary and no mobility. The patient reported mild tenderness when placing pressure on the lump. The patient had no active infection or skin impairment that could prevent us from scheduling a surgery, no sensory deficiency, and no muscle atrophy. No abnormalities were found in the muscle strength, sensory function, or circulation of the lower extremities. Computed tomography (CT) scans of the pelvic bone showed osteolytic damage in the right iliac body and iliac wing with a large soft tissue mass (). A CT-guided biopsy suggested grade I chondrosarcoma.\nBecause chondrosarcoma is not sensitive to chemotherapy, and considering the availability of a safe and mature technique with which to remove the lesion in a one-step surgical procedure, we considered one-step surgery to be the optimal treatment regimen. Any contraindications for surgical intervention were ruled out with a comprehensive blood test and examinations of the patient’s circulatory, respiratory, urinary, and gastrointestinal systems. Magnetic resonance imaging was performed to exclude early tumor penetration of the pelvic bone outside the large bone mass. After completion of all preoperative evaluations, surgery was scheduled with the consent of the patient and her family. The 3D printed tantalum implant was prepared by reconstruction of the patient’s CT scan with a spatial resolution of 20 lp/cm and density resolution of 0.67 mm ().\nImages of the patient’s pelvic bone and chondrosarcoma were obtained by a CT scanner (Siemens Healthineers, Erlangen, Germany), and the scan data from the Picture Archiving and Communication System (PACS) were reconstructed by Mimics 17.0 software (Materialise NV, Leuven, Belgium). The extent of the lesion was outlined, and the preoperative surgical plan was established using the 3D model. The 3D data including the surgical plan was stored in STL format and sent to an engineer for implant printing. The implant was designed by an engineer according to the surgical plan, bone quality, and patient’s overall condition (e.g., weight and height) under the parameters of “Personalized Additive Manufactured Medical Device Technical Censoring Guidelines (February, 2018)” by the Center for Medical Device Evaluation of China (). Finite element analysis was carried out to evaluate the probable biomechanical stress distribution of the implant. A porous structure was used in the 3D painted implant to avoid bone resorption and implant loosening from stress shielding. The implants were 3D printed using tantalum powder by Xi’an Sailong Metal Materials Co., Ltd. (Xi’an, China). The density of the implant was 2.7 g/m3, the diameter of each aperture was 200 µm, and the extent of porosity was 40%. The muscle and ligament suturing points were designed according to the pelvic CT and magnetic resonance imaging scans. Tumor recession guidance plates were designed to cut the tumor 2 cm beyond tumor border, and a 3-mm-diameter hole was made in the middle of the plate to fix it to the bone with a 2.5-mm-diameter Kirschner wire ().
Case 1 This is a 42 years old male with a history of a ruptured appendiceal mucinous neoplasm that was removed 23 years ago. He was asymptomatic until the fall of 1999 when he started complaining of recurrent small bowel obstruction and weight loss. He had undergone multiple investigations and was thought to have underlying Crohn's disease. A computed tomographic (CT) scan of his abdomen in January 2000 demonstrated a large abdominal mass. A laparoscopy was performed for tissue diagnosis of a possible lymphoma. However, at the time of laparoscopy, a large gelatinous mass was found and the diagnosis of PMP was confirmed by histopathology. Debulking surgery with small bowel resections was performed following this diagnosis at the Royal University Hospital of Saskatchewan. Since then he remained well and was reviewed regularly in the outpatient clinic. Four years later (2003), the patient began experiencing obstructive symptoms again due to recurrent disease and he had a further extensive debulking surgery in January 2004. This was followed a year later by another cytoreductive surgery with small bowel resection in 2005. He is currently doing well at a 3 months post-operative follow-up visit.\nThe specimens from 2000 consisted of a portion of small bowel with several gelatinous, "mucinous" masses attached to the peritoneal surface of the serosa of the bowel wall accompanied by the presence of lobulated polypoid gelatinous tissue (Figure ). The specimens from 2004 and 2005 had a similar gross appearance. Histologically, however, the tumor showed significant progression over the years from a predominantly hypocellular, benign looking single layer of bland epithelium in extracellular pools of mucin in year 2000 (Figure ) to a mild increase in epithelial proliferation in 2004 to a hypercellular lesion associated with cytological atypia and numerous free "cell clusters" floating in the extracellular mucin pools and infiltrating the submucosa of the small bowel (Figure ) representing the PMCA variant with a documented past history of ruptured appendiceal mucinous neoplasm 23 years ago with no material available for current pathological review.
A 19-year-old male baseball pitcher repeatedly sought medical attention due to left calf muscle tension and foot numbness for nearly a year. His symptoms were especially aggravated after exercising and with left knee joint extension. Although the patient repeatedly visited the clinic, none of the symptoms were improved. Therefore, the patient was referred from the clinic to our hospital. The physical examination revealed no limitation on left knee joint mobility, no specific discomfort, no weakness in the left lower extremity, and no atrophy or hypertrophy of the muscles. However, paresthesia was reported in the left calf and foot. There was tenderness in the left knee popliteal fossa and medial gastrocnemius muscle. For the appearance, there was no swelling or redness or local heat, but the left toes were colder than the right ones. The imaging studies showed no abnormality in the knee X-ray. However, further musculoskeletal ultrasonography’s transverse view of popliteal space of the posterior knee discovered the left popliteal artery was compressed by popliteus muscle and medial gastrocnemius muscle and an occlusion at the popliteal artery (as shown in ). Therefore, the patient was scheduled for knee MRI. The result showed that the origin of the medial head of gastrocnemius muscle was abnormally inclined to the lateral side, and the popliteal artery was located in the medial side of the medial head of the gastrocnemius muscle (which is rarely observed) and formed an aneurysm (as shown in –). As a symptom and result of imaging studies, the patient was diagnosed with popliteal artery entrapment syndrome, type 2.\nDue to the worsening symptoms, the patient was referred to the division of Vascular Surgery for surgical treatment. The pre-operative ultrasonography revealed a persistent occlusion at the P2 segment of the popliteal artery. During the surgery, it was found that the medial head of the gastrocnemius muscle was not located at the normal anatomical location, and the origin was inclined to the lateral side to compress the popliteal artery, which formed an aneurysm. Therefore, a decompression surgery was performed to surgically remove the partial tendon of the medial head of the gastrocnemius muscle and abnormal left popliteal artery. The left small saphenous vein transplantation surgery was further performed to replace the removed abnormal left popliteal artery. After the operation, the symptoms were completely relieved. Although the muscle strength of the left lower leg was slightly weakened, the patient quickly returned to the stadium to pitch baseball. The patient had signed a written informed consent form to have the case details and any accompanying images published and the manuscript was approved by the Ethics committee of Taichung Hospital, Ministry of Health and Welfare (Taichung, Taiwan) to have the case details published.
A 15-year-old male re-presented to the emergency department (ED) four days after involvement as a restrained rear seat, driver-side passenger in a front-on collision at 60 km per hour. In the absence of demonstrable external injury or clinical signs of trauma, the patient was discharged home from the ED following six hours of stable observation. Over the subsequent four days, he developed increasing abdominal distension and pain, with hematemesis developing in the hours preceding his second presentation to hospital.\nOn examination the patient was hypovolaemic with persistent tachycardia. He had a tense, distended abdomen with a paucity of bowel sounds. An urgent oral and intravenous (IV) contrast computed tomography (CT) of the abdomen demonstrated significant duodenal hematoma with no evidence of acute extravasation of oral contrast. The hematoma was associated with a degree of gastric outlet obstruction with minimal contrast observed in the small bowel. To further investigate the level and degree of gastric outlet obstruction, an oral Gastrogaffin meal study was performed six hour later, which showed complete gastric outlet obstruction ().\nBased on the combination of clinical and radiological findings, the patient was managed conservatively with a nasogastric tube (free drainage and four hourly aspirations) and total parenteral nutrition. The patient initially displayed high daily nasogastric outputs (∼1500 ml), but this started decreasing gradually over the subsequent two-week period. A few days after admission, the patient developed sudden elevation of serum lipase levels raising the suspicion of delayed manifestation of pancreatic injury. A repeat contrast enhanced CT of the abdomen excluded any such injury. The lipase levels normalized over the subsequent 72 hours and the patient continued to improve. A repeat Gastrogaffin meal on day 18 post injury revealed residual narrowing of the lumen of the second and third part of the duodenum but complete resolution of the gastric outlet obstruction (). Based on these findings, the patient was given clear fluids, then advanced to a full diet soon thereafter. The patient was discharged from the hospital without sequelae 22 days later.
The patient was a 69-year-old man who was examined for the main complaint of right submandibular cancer. The medical and family histories of the patient were unremarkable. Initial examination revealed a 15 mm × 10 mm hard, elastic tumor showing no mobility and an irregular margin. On computed tomography (CT) of the neck, a 20 mm × 10 mm nodular shadow with slight enhancement was observed in the right submandibular area (). No clear indication of metastasis to cervical lymph nodes was apparent. No obvious distant metastases were observed on chest CT. Neck magnetic resonance imaging (MRI) revealed a 20 mm × 10 mm tumor with indistinct boundary and slight enhancement in the right submandibular gland, showing signal hypointensity on both T1- and T2-weighted imaging (). Fine-needle aspiration (FNA) biopsy at this location indicated a class III lesion. The eosinophilic cytoplasm of duct epithelial cells and the presence of naked nuclei with prominent nucleoli made it difficult to rule out malignancy ().\nIn view of these findings, we explained the possibility of malignancy to the patient and after gaining informed consent resected the right submandibular gland tumor and conducted right neck dissection. Although no obvious metastasis to lymph nodes was identified, we removed the lymph nodes and submandibular gland together. No adhesion to the surrounding area was apparent. We preserved cranial nerves VII, IX, X, XI, and XII and the cervical nerves. The smooth surface of the removed tumor was light brown in color and solid in appearance (). No particular complications were encountered postoperatively, and the patient was discharged 7 days later. On histopathological examination, hematoxylin and eosin staining revealed a tumor with extensive cytoplasm containing eosinophilic granules in an irregular papillary ring structure. Infiltration of the surrounding salivary gland tissue was also observed. No infiltration of lymph ducts, blood vessels, or nerve peripheries was seen and the stump of the extracted tumor was negative (). On antimitochondrial antibody staining, more tissue with fine blue-stained granules than normal tissue was seen (). Based on these results, oncocytic carcinoma was diagnosed.\nAs histopathology had revealed no infiltration of lymph ducts, blood vessels, or nerve peripheries and the surgical margins were negative, no postoperative treatment was conducted. The patient has been followed up on an outpatient basis, with no recurrence or metastases being identified as of the time of writing, 3 years postoperatively.
The patient was a 33-year-old, 156 cm, and 62.5 kg primipara on the sixth day of the 40th week of pregnancy, and she was hospitalized on the day before the operation for vaginal delivery. The patient had not undergone any specific disease or operation according to her history, and the vital signs were normal at the time of hospitalization. The results of the blood test and urinalysis performed one month before the hospitalization were all normal. There was no cardiomegaly and abnormal finding in the pulmonary parenchyma by the chest radiography, and the electrocardiography results were all normal. The fetal heart rate measured at the time of hospitalization was 154 bpm, and the fetus showed normal reactivity. Although induced labor was carried out by injecting oxytocin during the afternoon on the day of hospitalization, the fetal heart rate was reduced, and thus, the oxytocin injection was stopped. Then, the fetal heart rate returned to normality and natural labor began. When oxytocin was injected again on the day of the operation, the fetal heart rate was reduced. Since meconium staining was found following the amniotic membrane rupture, an emergent Cesarean section was decided. The vital signs that were measured by the electrocardiography, pulse oximetry, and the automated blood pressure device at the operation room were 150 mmHg of systolic blood pressure, 100 mmHg of diastolic blood pressure, 90 of heart rate, and 94% of oxygen saturation. Because there was no specific finding by the inquiry and auscultation, the low oxygen saturation was considered as an error by the low body temperature since the hands and feet of the parturient woman were cold. Spinal anesthesia was decided because a little dry cough was found. After having the parturient woman take the right lateral decubitus position, 0.5% hyperbaric bupivacaine 10 mg mixed with fentanyl 20 ug was injected with a 26 G spinal needle between the L3 and L4 spines. The cold sense check was performed with an alcohol swab to verify the sensory block level to the fourth thoracic spinal segment. Five minutes after the spinal anesthesia, blood pressure was decreased to 90/50 mmHg, and the pulse oxygen saturation to 88%. The parturient woman complained of severe dyspnea and a continued cough and a large amount of sputum mixed with pink bubbles were observed. After ephedrine was immediately injected 3 times, 8 mg each, the blood pressure was recovered to 110/80 mmHg, the heart rate to 90/min, and the oxygen saturation to 92%, and then the operation was initiated. The results of the arterial blood gas study at that moment were pH 7.39, oxygen partial pressure (PaO2) 77.7 mmHg, carbon dioxide partial pressure (PaCO2) 34.5 mmHg, and oxygen saturation 95%. Just after the start of the operation, the spontaneous breathing seemed no longer impossible due to the severe dyspnea and excessive airway secretion. Thus, after injecting propofol 60 mg and succunycholine 70 mg, endotracheal intubation was carried out with the endotracheal tube of the inner diameter 7.0 mm. After the endotracheal intubation, a large amount of pink discharge was continuously aspirated through the tube to the extent that it blocked the tube, and the oxygen saturation of the patient was decreased down to 70%. Following the laparotomy, a great quantity of ascites was observed. After the fetus was delivered, the blood pressure was decreased to 60/40 mmHg, the oxygen saturation to 65%, and the heart rate to 60/min. Although dobutamine and norepinephrine were injected since heart failure was suspected, pulseless electrical activity (PEA) was found and the operation was suspended temporarily to carry out cardiopulmonary resuscitation. After the injection of epinephrine 1 mg and atropine 0.5 mg with continued thoracic compression, the sinus rhythm was recovered and the vital signs were increased to the blood pressure of 150/80 mmHg, oxygen saturation of 95%, and heart rate of 120/min. With the continuous intravenous injection of norepinephrine, as well as dopamine and dobutamine, a central vein catheter was inserted through the right internal jugular vein and an arterial catheter through the left femoral artery. Although the operation was then resumed, a severe hemorrhage was found and the blood pressure was reduced to 60/40 mmHg and the oxygen saturation to 80% at the end of the operation. The results of the arterial blood gas study at that moment were pH 7.154, PaO2 63.5 mmHg, PaCO2 57.5 mmHg, bicarbonate 19.8 mmol/L, and oxygen saturation 85.1%, which indicated a serious respiratory and metabolic acidosis and hypoxia. An electrolyte disorder was also observed as the ionized calcium concentration was 0.6 mmol/L and that of the potassium was 5.78 mmol/L. Bicarbonate was injected to correct the acidosis and the 50% dextrose in water (D/W) solution to which CaCl2 600 mg and insulin were added was also injected. In addition, pentaspan and normal saline were continuously injected through the central vein. Since the possibility of hemorrhage by coagulation disorder, not the surgical hemorrhage, could not be excluded, the packed red blood cells and the fresh frozen plasma were transfused, too. Although the blood test related with disseminated intravascular coagulation (DIC) was carried out during the operation (bleeding time, prothrombin time, activated prothromin time, antithrombin III, fibrinogen, D-dimer, and fibrinogen degradation product), the results could not be obtained because of coagulation of the blood sample. Even after the finish of the operation, the low blood pressure was maintained and the patient returned to the PEA state. Cardiopulmonary resuscitation was performed and epinephrine 1 mg and atropine 0.5 mg were injected 2 times for each. After the patient was recovered to the sinus rhythm, she was moved to the intensive care unit. The total operation duration was 1 hour; the urination was 150 ml; the blood loss was 1,500 ml; the injected fluid was 1,850 ml; and the transfused blood was 1,250 ml. More than 3,000 ml of secretion was discharged through the endotracheal tube and it spouted out continuously even in the intensive care unit. Heart failure took place once again and cardiopulmonary resuscitation was performed continuously. However, the blood pressure was dropped to 53/30-40/20 mmHg, the heart rate to 20-30/min, and the oxygen saturation to 50-60%. The complete heart failure was continued as the patient's status did not become any better, and the patient showed no response to the medications. Though the cardiac compression was continually carried out, the patient died after 3 hours. The autopsy was performed by the National Institute of Scientific Investigation and the cause of death was proved to be amniotic fluid embolism.
A 54-year-old man presented to the emergency department with agitation and seizures following a generalized febrile illness and a progressively worsening headache over 24 hours. He was treated with ceftriaxone IV, metronidazole, and acyclovir. A contrast CT scan of the brain showed no focal central lesion but features in keeping with bilateral chronic middle ear and mastoid disease. A lumbar puncture showed no evidence of raised intracranial pressure (ICP). CSF microscopy supported the diagnosis of meningitis, and on culture streptococcus sensitive to ceftriaxone and amoxicillin was grown. He responded to a three-week course of appropriate systemic antibiotics and made a complete recovery. On reviewing his history, he had been able to produce clear rhinorrhoea on leaning forward for, years and this tested positive for beta-2 transferrin. He had a left conductive hearing loss due to a middle ear effusion for several years. He had a grommet inserted in 2008, which was later removed due to persistent clear otorrhoea. CSF analysis at the time was inconclusive, and a CT scan of the temporal bones reportedly showed no abnormality. In 2000, he had a generalized seizure for which no cause could be found, and he has been on phenytoin ever since. He has type 2 diabetes mellitus for which he is on insulin. There was no past history of head trauma, CNS infections, neurologic, and otologic surgery. A High resolution CT and MRI scans of the petrous temporal bones were performed confirming bilateral tegmen tympani dehiscence, bilateral meningoencephalocoeles and bilateral middle ear and mastoid effusions.\nA middle fossa approach was used to repair both defects starting with the left. Six months later, the contralateral side was repaired. A 20 × 10 mm defect in the tegmen with substantial herniation of brain tissue was noted on the right, and an 8 × 2 mm defect with encephalocoele was present on the left. A strip of cortical bone harvested from the bone flap was placed over the defects which were then covered with a fascia lata graft. Tisseel was used to secure the graft in place. The recovery period was uneventful. CSF leakage ceased after surgery, and his hearing improved bilaterally.
A 56-year-old female was referred to our department from another facility in the patient’s area for a rapidly progressing tumor in the gallbladder and liver area. The patient reported several-month right upper quadrant pain and 4-kg weight loss over the past year. There was no laboratory sign of obstructive jaundice at the day of admission. Preoperative CT and MR scan (Figs. and ) of the liver was performed, and the patient was diagnosed with a tumor in the gallbladder area with a relatively massive infiltration of the S5 and S6 liver segments and extensive regions of necrosis. Given the potentially resectable lesion according to preoperative imaging, exploratory laparotomy was indicated to attempt radical resection. During the exploration, a voluminous tumor was found attached to the peritoneum. Intraoperative ultrasound was performed and revealed a tumor originating from the gallbladder bed area and reaching up to the area of the hepatic hilum and extensive involvement of the hepatoduodenal ligament by the tumor through the lymph nodes. The tumor was classified as inoperable due to this finding. But during the exploration, however, a rupture of the fragile tumor occurred with massive eruption of the necrotic mass and the gallbladder content into the abdominal cavity, accompanied by bleeding of the liver parenchyma. We decided that the condition could only be managed by attempting modified resection. We performed cholecystectomy and non-anatomical resection of hepatic segments S5 and S6 and partial resection of S4 without lymphadenectomy as a debulking operation (Fig. ). The course of hospitalization was uncomplicated, and the patient was discharged to home care on postoperative day 9. Histologically, the tumor was confirmed as MINEN of gallbladder (Figs. , , and ), and its non-neuroendocrine component had the character of moderately differentiated tubular gall bladder adenocarcinoma, while the neuroendocrine component had the appearance of small cell carcinoma and was dominant, accounting for more than 65% of the viable tumor. The neuroendocrine component contained extensive necrosis, with mitotic index 64/10 HPF and a proliferation index of 70% (Fig. ). It was therefore obvious that the prognosis and the subsequent biological behavior would be influenced in particular by the neuroendocrine carcinoma component. Six weeks after the discharge, the patient underwent a follow-up CT scan prior to the initiation of systemic therapy, which revealed a large recurrence of the disease at the resection surface of the liver accompanied by hilar lymphadenopathy. The patient was started on systemic therapy with etoposide and carboplatin in combination with somatostatin analogues with very good radiological effect. We use this regimen as a standard in patients with MINEN of gastrointestinal tract with dominant neuroendocrine component, even with no somatostatin receptors staining available. Now the patient is almost a year after being diagnosed with a tumor, after completion of 6 cycles of adjuvant chemotherapy (carboplatin + etoposide) in combination with biological therapy, the long-acting somatostatin analogues. The patient is in good clinical condition, and while a recently performed PET/MRI scan revealed a hepatic lesion and hilar lymphadenopathy in full regression, there was a spread of small peritoneal and pleural metastases, with a solitary metastasis in Th9. The condition was evaluated as disease progression stage according to RECIST criteria, the patient remains in the follow-up care, and it is now 13 months after surgery (Figs. , , and ).
A 56-year-old man of Asian origin was in his usual state of health until 4 days prior to presentation in our emergency room (ER); he had complaints of a rapidly progressing weakness of bilateral upper and lower limbs immediately prior to which he had a gastrointestinal upset. The weakness started from his lower limbs and gradually involved bilateral upper limbs in a similar glove and stocking fashion. He, however, did not have any sensory deficits and at the initial presentation in our hospital he had no respiratory and ocular muscle involvement. Consciousness and orientation were intact and he did not have significant hemodynamic instability. There was no significant medical or surgical condition requiring long-term hospitalization or medication use in the past. A government officer by profession, our patient had an active lifestyle and had no history suggestive of substance abuse or accidental or intentional poisoning. He lived with his wife and had two sons; one of his sons was living with him to support him and the elder son lived abroad but had been on good terms with the family. Our patient did not take any regular medications apart from the hypoglycemic agent metformin 500 mg administered orally twice daily. He did not smoke tobacco or consume alcohol regularly. During his initial presentation in our intensive care unit (ICU), he was conscious yet unable to speak properly. His vital signs were blood pressure (BP) 120/65 mmHg with no inotropic support, heart rate (HR) 102/minute regular, respiratory rate (RR) 26/minute regular, and he had no fever on admission.\nOur initial assessment led to a provisional diagnosis of GBS and immediate supportive tests were performed. A nerve conduction velocity test showed findings of motor axonal and demyelination neuropathy. A lumbar puncture done on the sixth day of the development of symptoms showed evidence of albuminocytologic dissociation with total counts (TC) of five cells/cc, which were all lymphocytes and CSF protein of 81 mg/dl (Table ). On the sixth day of the development of symptoms and third day of admission in ICU, he had complaints of difficulty in breathing with gradual decline in saturation with pulse oximeter reading of oxygen saturation (SpO2) to < 85% at fraction of inspired oxygen concentration (FiO2) of > 80%, and hypercapnia with partial pressure of carbon dioxide in arterial blood (PaCO2) of 86 mmHg. He was immediately intubated and kept on mechanical ventilator support.\nDiscussion was held with his relatives regarding available treatment options. A plan to initiate IVIG was made and started at 0.4 mg/kg per day for 5 days. The administration of IVIG was not associated with any significant complications. He did not, however, show major signs of recovery from respiratory weakness and was continuously kept on assist-control mode of ventilation with intermittent spontaneous breathing trials. At this time, he had occasional blood-tinged secretion in the subglottic suction and during intermittent endotracheal suctioning. A detailed coagulation profile did not show significant abnormalities. On the ninth day of admission in ICU, a plan for tracheostomy was made anticipating prolonged need for mechanical ventilation and as a part of routine pre-anesthetic preparation, a unit of group-specific (A +ve) blood was asked to be arranged. However, we were then notified by the blood bank that they had problems with cross-matching of the blood. A repeated blood sample of our patient was sent which also had a similar problem of inability to cross-match the blood. A literature search for the possible causes of such an occurrence was made but we only had a few reports of such problems. With a provisional diagnosis of significant hemolysis leading to cross-matching difficulties, further tests were sent (Table ). An arrangement of O negative blood was made as a reserve and a tracheostomy was performed with no major problems.\nHis stay in ICU was then complicated with hospital-acquired chest infections for which he received antibiotics based on organisms’ susceptibility. Liver function tests (LFTs) which were initially deranged subsequently normalized after gradual stabilization of his condition and de-escalation of drugs. Serological tests which included quantitative HIV, hepatitis B surface antigen (HBsAg), and anti- hepatitis C virus (HCV) antibody were negative. He was subsequently moved out of ICU on the 19th day with tracheostomy in situ and on portable bilevel positive airway pressure (BIPAP) support. Three days after being moved to a ward, he was brought back to ICU for sudden-onset dyspnea and tachypnea. He had coarse crepitations more on the anterior aspect of bilateral chest and slight decrease in breath sounds on bilateral basal regions. He did not, however, have fever or changes in vital signs and his consciousness was intact. He was managed conservatively with chest physiotherapy, deep breathing exercises, regular tracheostomy care, and suctioning of secretions from lungs. He recuperated in 2 days and was moved back to a ward where he slowly recovered from his weakness. No other untoward events occurred during this period of approximately 4 months. He is being planned for discharge to the care of a nursing home and the prognosis of the disease has been well explained to his relatives.
The patient, a 45-year-old female, had a several year history of a lump in the right groin. It had never become infected nor had it ever drained. She saw a dermatologist who felt this was a cyst and scheduled excision. While dissecting the lump, the dermatologist appreciated that it was deeper than the skin, closed the skin and sent the patient to a plastic surgeon.\nThe plastic surgeon appreciated this lump with a scar overlying it limiting examination. The plastic surgeon brought the patient to an outpatient surgery center due to her desire to avoid the hospital since this operation took place during the COVID-19 pandemic. With the patient under sedation, the plastic surgeon performed a right groin exploration. Upon dissection, some normal sized lymph nodes but no cyst was found. Deeper dissection revealed a firm mass. Upon opening the enveloping tissue, the plastic surgeon encountered what looked like another lymph node. Emergent intraoperative consultation from a general surgeon was placed.\nUpon further dissection by the general surgeon, it became clear that what the plastic surgeon found was not another lymph node, but rather, the distal tip of the patient’s appendix. Dissection to the level of the fascia revealed that the enveloping tissue was a hernia sac and the entire appendix was incarcerated within a femoral hernia ().\nGiven the lack of any evidence of appendicitis and the lack of any past infection or drainage, the appendix was freed and reduced back into the abdominal cavity without injury. The hernia sac was closed with an absorbable suture and the hernia was repaired using a small polypropylene mesh. The wound was closed in layers and the patient was awoken.\nUpon discharge, the patient was informed of her diagnosis and was advised to call immediately if she developed any abdominal pain as this could indicate appendicitis.\nThe patient never developed appendicitis and had almost no pain postoperatively. She had no postoperative complications. She returned to full physical activities with respect to both exercise and her duties as a nurse.
A seventy-six years old lady presented to our Emergency Department with complaints of progressive worsening of headache eleven days in duration and acute onset of several episodes of vomiting since the past two days. She was referred from another center where she was treated symptomatically with no relief of either pain or vomiting. She reported no weakness or numbness of upper and lower limbs. There was no history of fever, abnormal body movements and loss of consciousness. She reported decreased appetite and alteration of sleep habit. She did not give any history of recent trauma or falls. She however gave a history of head surgery 20 years back for a subdural hematoma that she had due to a fall and a leg fracture 7 years ago which was fixed with plates. An interesting revelation was that the patient had remained unconscious for three months following her previous surgery for SDH evacuation.\nOn examination, she was ill-looking. Her vitals were normal. Cardiovascular, respiratory and abdominal examination were normal with a blood pressure of 140/80 mm Hg , pulse rate of 80 beats per minute, a respiratory rate of 20 breaths per minute and oxygen saturation (Sp02) of 98%. She was afebrile, conscious with a Glasgow Coma Scale of 15 and normal neurologic exam, including that of cranial nerves. Tone, power and reflexes were normal in both upper as well as lower limbs. Sensations to all four limbs were also preserved. Palpation of spine revealed no tenderness or steps.\nInitial laboratory investigations including random blood glucose, hematological profile including that of clotting, liver and renal function tests and other basic tests were normal. A non-contrast CT scan of the head was ordered in the ER which revealed a small hyperdense area surrounded by hypodense areas indicating acute on chronic, right subdural hematoma with a small acute bleed around the temporal lobe and a chronic bleed around the temporoparietal region. The CT also showed a massive hypodense area indicating a large subdural hematoma on the left temporoparietal region, with a very slight midline shift to the right, to go along with a previous burr hole mark on the left temporal bone ().\nA neurosurgical referral was made. She was monitored intensively and treated conservatively with no improvement of her symptoms. Surgery was then planned. A left parietal burr-hole craniostomy was done under local anaesthesia, the following day with aspiration of “machinery-oil” blood under pressure. The headache resolved. The next day after the surgery, the patient complained of headache on the side opposite to which the procedure was performed. The patient was closely monitored in the neurosurgical Intensive Care Unit. After the pain persisted for a day more, a repeat head CT was ordered. It revealed isodense to slightly hyperdense areas in the right temporo-parietal region with a slight midline shift to the left ().\nA right parietal burr-hole craniostomy was performed under local anaesthesia which led to aspiration of dark brown blood under pressure. A repeat CT of the head showed resolution of the hematoma ().\nThree days after the second procedure, the patient complained of frontal headache and nausea. The patient was febrile as well. Repeat CT showed a large bi-frontal pneumocephalus ().\nA chest radiograph showed bilateral infiltrates. These problems were managed medically with high flow oxygen therapy and intravenous antibiotics and the patient recovered eventually (). She was discharged after 13 days of in-hospital stay.
A 23-year-old female presented with ongoing management of a keloid scar on her right auricle with no relevant medical history. At 21 years of age, she had her ear pierced and developed a local infection that was treated initially with topical antibiotics. Subsequently, a keloid scar began to form (). At first presentation, the lesion was observed to be a 1 cm red keloid scar on the mid-helix of the right auricle. Initially, 40 mg of methylprednisolone acetate was injected locally and she was given a prescription for a pressure earing. Mechanical pressure is often used as a means to prevent or treat keloid scars.\nAt 1 month follow-up, during which time she had been wearing the pressure earing consistently, a slight improvement was observed with a mild reduction in the overall size of the keloid scar. Another injection of 40 mg methylprednisolone acetate was given locally ().\nFour months after commencing the initial treatment, the patient presented again, and a 15% improvement was observed with a reduction in the size of the keloid scar tissue to a diameter of 11 mm. Again, the patient received another local injection of methylprednisolone acetate, but at a reduced dose of 20 mg. At 5 months follow-up, the base of the keloid was unchanged and remained at 11 mm. However, the overall volume of the keloid had a flattened appearance as a result of the pressure earing. Again, the patient was administered another injection (locally) at the original dose of 40 mg methylprednisolone acetate. A further 10 weeks on, the base of the keloid scar measured 10 mm and its height was reduced, with the total thickness of the right helix of the involved area measuring 8 mm as opposed to 7 mm in the opposite ear. Of significance, it was noted that part of the normal tissue in the involved area was replaced by the keloid over 1 mm thickness. The dose of 40 mg methylprednisolone acetate was once again injected locally, in conjunction with the pressure earring ().\nA further 2 months on, the base of the keloid remained unchanged at 10 mm; however, the total thickness of the helix reduced to 7 mm. The posterior region of the helix remained a small flat patch, while the anterior of the helix presented with only minimal keloid content. At this time, another injection of 40 mg methylprednisolone acetate was administered. After completing a year’s treatment with methylprednisolone acetate and 3 years after the original piercing, the keloid scar became active again, appearing red and telangiectasic. A final local injection of 60 mg methylprednisolone acetate was administered, resulting in no improvement with a further rapid growth in size ().\nIt was decided to surgically excise the keloid lesion using wide margins, and subsequently, the growth was sent for histological examination. Histological examination determined that the lesion was consistent with keloid and had tiny foci of BCC arising from within. The patient provided informed written consent for the described procedures and the use of digital photography for the purposes of treatment, teaching and use in academic publications.
A 56-year-old man of Asian origin was in his usual state of health until 4 days prior to presentation in our emergency room (ER); he had complaints of a rapidly progressing weakness of bilateral upper and lower limbs immediately prior to which he had a gastrointestinal upset. The weakness started from his lower limbs and gradually involved bilateral upper limbs in a similar glove and stocking fashion. He, however, did not have any sensory deficits and at the initial presentation in our hospital he had no respiratory and ocular muscle involvement. Consciousness and orientation were intact and he did not have significant hemodynamic instability. There was no significant medical or surgical condition requiring long-term hospitalization or medication use in the past. A government officer by profession, our patient had an active lifestyle and had no history suggestive of substance abuse or accidental or intentional poisoning. He lived with his wife and had two sons; one of his sons was living with him to support him and the elder son lived abroad but had been on good terms with the family. Our patient did not take any regular medications apart from the hypoglycemic agent metformin 500 mg administered orally twice daily. He did not smoke tobacco or consume alcohol regularly. During his initial presentation in our intensive care unit (ICU), he was conscious yet unable to speak properly. His vital signs were blood pressure (BP) 120/65 mmHg with no inotropic support, heart rate (HR) 102/minute regular, respiratory rate (RR) 26/minute regular, and he had no fever on admission.\nOur initial assessment led to a provisional diagnosis of GBS and immediate supportive tests were performed. A nerve conduction velocity test showed findings of motor axonal and demyelination neuropathy. A lumbar puncture done on the sixth day of the development of symptoms showed evidence of albuminocytologic dissociation with total counts (TC) of five cells/cc, which were all lymphocytes and CSF protein of 81 mg/dl (Table ). On the sixth day of the development of symptoms and third day of admission in ICU, he had complaints of difficulty in breathing with gradual decline in saturation with pulse oximeter reading of oxygen saturation (SpO2) to < 85% at fraction of inspired oxygen concentration (FiO2) of > 80%, and hypercapnia with partial pressure of carbon dioxide in arterial blood (PaCO2) of 86 mmHg. He was immediately intubated and kept on mechanical ventilator support.\nDiscussion was held with his relatives regarding available treatment options. A plan to initiate IVIG was made and started at 0.4 mg/kg per day for 5 days. The administration of IVIG was not associated with any significant complications. He did not, however, show major signs of recovery from respiratory weakness and was continuously kept on assist-control mode of ventilation with intermittent spontaneous breathing trials. At this time, he had occasional blood-tinged secretion in the subglottic suction and during intermittent endotracheal suctioning. A detailed coagulation profile did not show significant abnormalities. On the ninth day of admission in ICU, a plan for tracheostomy was made anticipating prolonged need for mechanical ventilation and as a part of routine pre-anesthetic preparation, a unit of group-specific (A +ve) blood was asked to be arranged. However, we were then notified by the blood bank that they had problems with cross-matching of the blood. A repeated blood sample of our patient was sent which also had a similar problem of inability to cross-match the blood. A literature search for the possible causes of such an occurrence was made but we only had a few reports of such problems. With a provisional diagnosis of significant hemolysis leading to cross-matching difficulties, further tests were sent (Table ). An arrangement of O negative blood was made as a reserve and a tracheostomy was performed with no major problems.\nHis stay in ICU was then complicated with hospital-acquired chest infections for which he received antibiotics based on organisms’ susceptibility. Liver function tests (LFTs) which were initially deranged subsequently normalized after gradual stabilization of his condition and de-escalation of drugs. Serological tests which included quantitative HIV, hepatitis B surface antigen (HBsAg), and anti- hepatitis C virus (HCV) antibody were negative. He was subsequently moved out of ICU on the 19th day with tracheostomy in situ and on portable bilevel positive airway pressure (BIPAP) support. Three days after being moved to a ward, he was brought back to ICU for sudden-onset dyspnea and tachypnea. He had coarse crepitations more on the anterior aspect of bilateral chest and slight decrease in breath sounds on bilateral basal regions. He did not, however, have fever or changes in vital signs and his consciousness was intact. He was managed conservatively with chest physiotherapy, deep breathing exercises, regular tracheostomy care, and suctioning of secretions from lungs. He recuperated in 2 days and was moved back to a ward where he slowly recovered from his weakness. No other untoward events occurred during this period of approximately 4 months. He is being planned for discharge to the care of a nursing home and the prognosis of the disease has been well explained to his relatives.
A 39-year-old male was transferred to our hospital from another hospital. He had mild tenderness, an obvious bowel pattern and hyperactive bowel sounds; he was able to pass gas occasionally. Before admission, he suffered progressive abdominal distention and gradual deterioration, and he developed malnutrition for two months. A total alimentary tract angiography showed partial enlargement of the ascending colon and transverse colon and partial dilation of the distal small intestine (Fig. ). He was diagnosed with “adult megacolon” and recommended for surgical treatment. However, the operation was not performed because of a significant decrease in platelets (with a minimum of 19 × 109/L) and severe malnutrition. He had been in a car accident 2 years previously. He was the driver and was wearing a seat belt at the time of the accident. During that admission, he was always conscious and was found to have left clavicle fractures and multiple rib fractures. Abdominal examination showed seat belt marks and mild localized tenderness at the site of the abrasions. An abdominal CT scan showed a small amount of fluid (approximately 150 ml) in the abdominal cavity with no solid organ abnormalities. He was hemodynamically stable and was able to pass gas and defecate. He improved rapidly with conservative treatment, was discharged after several days and was asymptomatic. Two months after discharge, he started to have episodes of abdominal distension and intermittent mild tenderness, and he passed gas less frequently than before. However, he improved rapidly again after receiving treatment with traditional Chinese medicine.\nAfter admission, we first tried to improve the general condition of the patient by strengthening parenteral nutrition and correcting electrolyte imbalances. Then, a series of additional examinations were performed to explore the possible reasons for these problems. An abdominal CT scan showed an abrupt narrowing zone at the jejunum (Fig. ). Small balloon colonoscopy found a narrow zone approximately 40–50 cm from the ileocecal valve; the surface mucosa was swollen and erosive, and the upper segment of the intestine was obviously expanded (Fig. ). Laparoscopy was performed on the patient after multidisciplinary discussion and detailed preoperative evaluation. We found severe adhesion between the abdominal wall and intestine as well as a narrow small bowel with a length of 12 cm at approximately 40–50 cm from the ileocecal valve. The mesentery corresponding to the narrow part of the small intestine was also absent, and the proximal intestine was markedly dilatated. Additionally, a thick adhesive band was also found between the dilated proximal intestine and the sigmoid colon, and we thought it might be the main cause of colonic dilation (Fig. ). Therefore, we performed laparoscopic adhesiolysis and partial small bowel resection, and the thick adhesive bands were destroyed. The narrow small bowel with length of 20 cm was removed. Histologically, the area was fibrotic (Fig. ). The patient recovered rapidly and gained 5 kg in the 3 months after surgery. He was very satisfied with the treatment.
A 51-year-old man was admitted to our surgical unit with a diagnosis of chronic pancreatitis. He was suffering from episodes of continuous abdominal pain for the last one year. The pain was radiated from the right upper quadrant of the abdomen to the back and was associated with nausea, partially relieved by injectable analgesics and aggravated by food ingestion. This challenge led to the loss of appetite then to weight loss which also worsened by the presence of occasional episodes of malabsorption and the development of insulin dependent diabetes mellitus. There was no family history of the similar condition in parents, siblings or first-degree relatives.\nMoreover, there was no history of abdominal trauma in the past. The patient had a history of admissions to different hospitals for the recurrent pain attacks. However, apart from this illness, he had never been to hospitals for any other medical or surgical condition. He was on analgesics, insulin therapy, and pancreatic enzyme supplementation and had never been allergic to the exposed medications.\nThe physical examination showed an emaciated man looking older than his chronological age, appeared pallor but no clinical evidence of jaundice. Abdominal examination was normal. Chest examination found no remarkable findings, and the rest of the physical examination was normal. The laboratory examination showed an increased level of blood sugar, normal levels of serum amylase, renal function tests and liver function test (LFT). No attempt was made to establish the insufficiency of the exocrine pancreatic function. Chest X-Ray and ECG were normal.\nPlain radiology showed opacities in the middle and right upper quadrant abdomen (a). CT abdomen was performed to rule out other associated pathologies like pancreatic pseudocyst, pancreatopleural, pancreatogastric or pancreatocolonic fistulae as the surgical procedure would have been different in the presence of any of the complications. Contrast-enhanced abdominal CT scan revealed impacted stones at the ampulla within the distal bile duct and pancreatic duct. The remaining parts of the pancreas showed normal result with no associated features of chronic pancreatitis (b). Since the contrast-enhanced abdominal CT scan indicated impacted stones in the distal bile duct at the ampulla, MRCP was performed. Initial MRCP suggested stones within the main pancreatic duct (MPD) in the head of the pancreas and dilated common bile duct suggested stone in the distal part of it (c).
A 51-year-old man was admitted to our surgical unit with a diagnosis of chronic pancreatitis. He was suffering from episodes of continuous abdominal pain for the last one year. The pain was radiated from the right upper quadrant of the abdomen to the back and was associated with nausea, partially relieved by injectable analgesics and aggravated by food ingestion. This challenge led to the loss of appetite then to weight loss which also worsened by the presence of occasional episodes of malabsorption and the development of insulin dependent diabetes mellitus. There was no family history of the similar condition in parents, siblings or first-degree relatives.\nMoreover, there was no history of abdominal trauma in the past. The patient had a history of admissions to different hospitals for the recurrent pain attacks. However, apart from this illness, he had never been to hospitals for any other medical or surgical condition. He was on analgesics, insulin therapy, and pancreatic enzyme supplementation and had never been allergic to the exposed medications.\nThe physical examination showed an emaciated man looking older than his chronological age, appeared pallor but no clinical evidence of jaundice. Abdominal examination was normal. Chest examination found no remarkable findings, and the rest of the physical examination was normal. The laboratory examination showed an increased level of blood sugar, normal levels of serum amylase, renal function tests and liver function test (LFT). No attempt was made to establish the insufficiency of the exocrine pancreatic function. Chest X-Ray and ECG were normal.\nPlain radiology showed opacities in the middle and right upper quadrant abdomen (a). CT abdomen was performed to rule out other associated pathologies like pancreatic pseudocyst, pancreatopleural, pancreatogastric or pancreatocolonic fistulae as the surgical procedure would have been different in the presence of any of the complications. Contrast-enhanced abdominal CT scan revealed impacted stones at the ampulla within the distal bile duct and pancreatic duct. The remaining parts of the pancreas showed normal result with no associated features of chronic pancreatitis (b). Since the contrast-enhanced abdominal CT scan indicated impacted stones in the distal bile duct at the ampulla, MRCP was performed. Initial MRCP suggested stones within the main pancreatic duct (MPD) in the head of the pancreas and dilated common bile duct suggested stone in the distal part of it (c).
A 43-year-old female presented with recurrent subcutaneous mass in the left superolateral orbital region []. The mass has been present since she was 4 years old, and there have been no episodes of local infections. She had a history of partial resection of this mass at another department 2 years ago, where only subcutaneous component of the cyst was ligated and resected. Pathological diagnosis was dermoid cyst. In 2 years’ follow-up, she developed a recurrent mass in the same region. Due to her active asthma and the history of adverse events that occurred after contrast MR imaging, contrast imaging studies were not performed. CT and MR imaging showed a subcutaneous mass with possible continuity to the Sylvian cistern by a tract in the sphenoid ridge and lesser wing of the sphenoid bone [ and ]. As 2 years have passed since the initial surgery, we speculated the lesion had already penetrated the subarachnoid space through the dura based on the preoperative imaging. While she had no headache or showed any signs of neurological deficits, we decided to reset the mass and the tract to prevent further recurrence.\nShe underwent frontotemporal craniotomy. When musculocutaneous flap was retracted, the cyst duct was inadvertently damaged, resulting in spill out of greasy and whitish yellow cyst content []. The tumor was relatively large and fanning toward the skin, suggesting that dissection of the tumor through the temporal muscle and fascia could lead to damage to the frontal branch of the facial nerve.[] We thus cut the cyst duct and followed the duct toward the bone [] and to the dura. The duct looked ending in a blind pouch at the surface of the dura [] near the lesser wing of the sphenoid bone. As the lumen was exposed, yellowish debris and hair were appreciated. Intradural space was then carefully inspected and showed no evidence of lesion continuity. Finally, the remaining extracranial mass was totally resected with careful inspection of the facial nerve using a nerve stimulator.\nThe resected specimens showed a cystic lesion filled with keratinized debris and a ductal structure which was connected to the cyst. The cyst wall was lined by a stratified squamous epithelium similar to epidermis. It contained adnexa of skin, such as sebaceous glands and sweat glands []. As for the transitional segment from the cystic lesion and the dura, histiocytes infiltration and hyalinized dura were appreciated, with no dermoid components. Consequently, dermoid cyst and sinus tract were diagnosed.\nThe patient developed mild facial palsy of the corrugator supercilii. House and Brackmann grade was 2. She was discharged home 8 days after operation with modified Rankin scale of 1. At 6 months after the second surgery, she was free of recurrence clinically and radiologically.
A 40-year-old male presented to our office in 2017 complaining of chronic left knee pain. This was his first visit to this office and was establishing care after moving to the area. The patient's electronic medical record was obtained from an outside institution which indicated a past medical history of diabetes, hyperlipidemia, and hereditary multiple exostosis. The patient disclosed that he had multiple osteochondroma removal surgeries which included his left knee, lumbar spine, and left foot at an outside institution. In addition, he had a left hip arthroplasty with refractory numbness/tingling of the leg. The patient stated he had left knee pain for years until he had an osteochondroma removed in his left distal femur in 2016 which seemed to help for 6 months. He stated the pain returned at 7/10 and is worse with movements. He had limited range of motion with 70° of flexion, negative pain with varus and valgus movement, and negative secondary tests. Baseline X-rays were ordered for this patient's knee ().\nThere were osteoarthritic changes with significant osteochondroma formation; however, arthroplasty was not recommended at that time because of the patient's young age and his left lower extremity being distally neurovascular intact. The patient was adamant about having his knee replaced and was referred to a specialist at an outside institution where an arthroplasty of the left knee was performed. The patient returned 3 months later with continued limited active and passive range of motion (<90° flexion). Follow-up radiographs were ordered showing good alignment of a Smith & Nephew posterior stabilized prosthesis without subsidence (). It was decided to perform manipulation under anesthesia followed by 6 weeks of physical therapy which improved his range of motion to 110° flexion.\nThe patient continues to have limited range of motion of his left knee with flexion to 90°. The large posteriorly projecting osteochondroma of his left tibia continues to be asymptomatic and painless, and therefore, it was decided not to perform surgical interventions at this time. Performing an arthroplasty helped relieve the patient's pain, however, did not result in significant improvement of active and passive range of motion of the joint. Overall, the patient is satisfied with his knee replacement but still has refractory numbness/tingling of his distal left lower extremity from his prior hip replacement. The patient has chronic pain elsewhere, which is being closely monitored (Figures and ).
In December 2011, a 69-year-old male patient was admitted to our institution for the evaluation of further therapeutic options of metastatic malignant melanoma. Previously, in March 2009, the diagnosis of an ulcerated nodular solid malignant melanoma of the left cheek (thickness 30 mm, Clark level 5) with infiltration of the left parotid gland had been made. The neoplastic cells carried wild-type B-RAF alleles, but an oncogenic mutation in exon 2 of the N-RAS gene was found. The past medical history was unremarkable except for hypertension, for which antihypertensive combination therapy was given. In March 2009, the patient underwent surgical excision and neck dissection, during which a total of 16 lymph nodes were removed, which histologically did not show any signs of tumor invasion at that time. Subsequently, however, in October 2009, a soft tissue metastasis developed at the base of the resection area, which was treated with local irradiation over a course of 2 months. In December 2010, another subcutaneous metastasis was found above the left base of the mandible, which was surgically resected. Histopathological analysis showed clear resection margins.\nAdjuvant immunotherapy with 3 million units of interferon-α thrice weekly was initiated in January 2011 but terminated in May 2011, when increasing serum concentrations of the tumor marker S100 indicated disease progression under therapy. Moreover, by this time, the patient had developed numbness and weakness of the right arm and left leg. A CT scan revealed newly developed lung metastases and lymph node metastases in the right hilar region as well as below the carina, and an MRI scan showed 2 new brain metastases – one in the left frontal region and another one in the area of the head of the right caudate nucleus.\nAfter a course of stereotactic irradiation (7 × 5 Gy, i.e. a cumulative dose of 35 Gy), another MRI scan performed in December 2011 showed regression of the left frontal brain metastasis and constant size of the right caudate nucleus lesion.\nSystemic therapy with temozolomide was started in August 2011. A restaging CT scan was performed in November 2011 and showed progressive disease under therapy, with an increase in the size of 2 lung metastases and a newly diagnosed lymph node metastasis in the left axilla. At this point, temozolomide therapy was abandoned and the patient was referred to our institution for evaluation of further therapeutic options.\nUpon presentation at our clinic, the neurological symptoms had resolved completely, and the patient reported no relevant symptoms except for an overall feeling of fatigue and lack of energy. At this point in time, the patient was on continuous prophylactic steroid therapy with low-dose decortin. In December 2011, second-line therapy with ipilimumab was initiated at a dose of 3 mg/kg intravenously every 3 weeks for a total of 4 courses. The therapy was well tolerated without any signs of toxicity or tangible adverse effects. In particular, the patient did not develop diarrhea or other signs of autoimmune disease and showed no neurological deficits. Routine follow-up laboratory parameters remained mostly normal except for an isolated increase in serum lactate dehydrogenase (LDH) concentrations of up to 348 U/l during the initial 2 cycles of ipilimumab, which spontaneously resolved upon cessation of ipilimumab administration (fig. ).\nA follow-up CT scan performed in March 2012 demonstrated stable disease with respect to extracranial manifestations, with marked size regression of the mediastinal metastases as well as the left axillar lymph node metastasis and constant size of a right-cervical lymph node (1.3 cm in diameter). Of interest, at this point, the tumor marker S100 had dropped from 0.24 µg/l before initiation of ipilimumab administration in December 2011 to 0.16 µg/l in April 2012 after 4 cycles of ipilimumab (fig. ). However, an MRI scan performed in March 2012 revealed size progression of the brain metastasis in the area of the head of the right caudate nucleus, a newly developed metastasis in the left frontal region as well as several new small metastatic lesions in the left parietal region.\nTherefore, administration of ipilimumab had to be stopped, and another round of percutaneous irradiation of the neurocranium was initiated (5 × 2 Gy per week up to a total dose of 40 Gy, i.e. a cumulative dose of 75 Gy).\nA repeat MRI scan done in October 2012 revealed a decrease of the cerebral metastases by 25–50% on average, without occurrence of any new cerebral metastases. A CT scan of the abdomen, thorax and neck performed in November 2012 showed a mixed response, with regression of some distant organ metastases, while others had progressed or newly developed. By November 2012, the tumor marker S100 had again risen to 0.43 µg/l.\nThe patient is currently in a rather good clinical condition and able to perform the tasks of routine everyday life.
A 55-year-old Japanese woman who was employed as a part-time childcare assistant initially experienced a slight sore throat (day 0). She reported being under stress caused by the daily problems of looking after her elderly parents. On day 8, she became emotionally unstable and required supervision by her daughter all day. She lost all awareness of time and frequently checked the television display and clock. She mistook night for day and was unable to tell the date. In the evening, she was unable to carry out simple cooking tasks, and asked her daughter for help. On day 9, she was conscious but unable to walk as a result of involuntary limb movement; she also repeatedly recalled old memories and expressed concern about her parents. On day 11, she was admitted to a psychiatric hospital, but was transferred to our facility due to suspected encephalitis. At the time of admission, she showed signs of instability in cognition and attention to her surroundings. Although she retained verbal ability, she was disoriented with respect to time and place. Retrograde and anterograde amnesia were also present; she could not recall the work she was engaged in or when and how she was admitted to our hospital. Spontaneous tonic spasms appeared in her limbs bilaterally. A standard bedside neurological examination revealed otherwise normal findings for her cranial nerves, motor and sensory functions, and coordination. She had no special medical and family history or relevant past interventions.\nDuring her hospitalization, the patient informed us that she believed she was 27 years old. She informed us that she was currently living in Aomori Prefecture and working as a receptionist, which accurately reflected events she experienced at age 27. She could recall memories of her marriage at age 25, but not her daughter, whom she gave birth to at 27. On a separate occasion, the patient adopted a birthing posture, as if she was re-experiencing the delivery that she had gone through when she was 27. However after informing her of her true age (55 years old), she recognized this fact, and her memory was transiently corrected accordingly.\nHer MMSE score was 26. She showed decreased measures of attention and concentration, along with delayed recognition, as assessed by the WMS-R; however, her WAIS-III evaluation was normal. A CSF analysis revealed normal cell density, protein, and glucose levels. Antibody titres to HSV, VZV, and EBV were also normal, and HSV was undetected by PCR. Subsequently, elevated serum anti-NMDA receptor antibody titres were detected in both serum and CSF.\nThere were no abnormal findings revealed by brain MRI (Fig. ). However, 123I-IMP brain perfusion SPECT showed increased blood flow bilaterally in the medial temporal lobes and orbitofrontal cortex (Fig. and ). Epileptic discharges and abnormal slow waves were not detected by EEG. Following evaluation, she received a diagnosis of anti-NMDA receptor encephalitis. Oral anti-epileptic drugs (carbamazepine, 200 mg/day; levetiracetam, 2 g/day) and intravenous high-dose corticosteroid therapy (methylprednisolone, 1 g/day for 3 days) improved her symptoms, confirming the diagnosis of AAAD. The patient was discharged on day 51. At the time of discharge, there was no disorientation or amnesia, her MMSE score improved to 28/30. Subsequently, she stopped coming to our hospital and we were unable to perform follow-ups.\nClinical findings for both cases are summarized in Table .
Three weeks after delivery, a 32-year-old woman was admitted to our hospital because of tachypnoea and tachycardia. She was first presented to the gynaecological unit with constant abdominal pain since delivery. The birth had been experienced as traumatic by the patient, but reports documented no complications during the vaginal delivery. The newborn daughter was the first child of the patient and was healthy. There were no abortions in the past. The patient reported not to have eaten solid food for days because of nausea and vomiting, while fluid intake caused no problems. Micturation and defecation where normal, she had no fever. Her medical history included multiple deep vein thromboses in the past (seven, five, and two years ago). Therefore, she received prophylactic treatment with low molecular weight heparin during pregnancy and after delivery. A thrombophilia screening was not yet performed because the patient rejected it for personal reasons. There where no other relevant diseases in the past history and no relevant family history.\nOn admission, clinical examination revealed a muscular defense over the whole abdomen and tenderness in the lower left quadrant. Gynaecological physical examination was without pathological findings. Transvaginal ultrasound revealed an ovarian venous thrombosis on the left side which was confirmed by CT-scan. No additional locations of thrombosis were detected. The dose of low molecular weight heparin therapy was increased from a prophylactic to a therapeutic regiment.\nOver the next hours, tachypnoea worsened while the oxygen saturation remained normal. Increasing tachypnoea, tachycardia and hyperventilation led to the referral of the patient to the internal medicine unit.\nAfter arrival in Internal Medicine, an arterial blood gas analysis was performed and disclosed a severe metabolic acidosis with partial respiratory compensation and a high anion gap (see Table Table ). In addition, marked hypophosphatemia was detected. Further workup revealed the presence of ketone bodies in the urine despite normal blood glucose. She did not have a history of diabetes, and did not drink alcohol. She did not take medication regularly except prophylactic heparin during pregnancy. The presence of keton bodies might – in addition to the ovarian venous thrombosis – have also contributed to the patient’s complaints since abdominal pain is sometimes present in ketoacidosis.\nOur patient was immediately transferred to ICU and an infusion of sodium bicarbonate (8.4 %), potassium-phosphate (50 mmol/50 ml), glucose (20 %), and saline was initiated via a central line. After these measures as well as after immediate re-feeding and discontinuation of breastfeeding, acid–base homeostasis started to normalize and tachypnoea subsided. After ten days, the patient had fully recovered and was discharged home.
A 28-year-old Japanese woman, para 2 (two previous cesarean sections), presented to our hospital at 11 weeks of gestation because of potential CSP. At 9 weeks, she had undergone transvaginal ultrasound (TVUS) at another hospital, which showed the gestational sac to be positioned low in the uterus in the area of her previous cesarean scar. However, when the patient first presented to our hospital, transabdominal ultrasound could not clearly confirm CSP because there was no evidence of placenta preva and the fetus and placenta had grown toward and presented in the uterine cavity. As CSP could be neither confirmed nor ruled out, we opted to carefully monitor the perinatal conditions. At 28 weeks, TVUS showed placental attachment on the anterior wall with a distance between the placental margin and intra OS of at least 77 mm. Seeing no evidence of placenta previa, and we assumed PAS to be less likely ().\nAt 29 weeks of gestation, the woman was admitted because of preeclampsia. Magnesium sulfate was used to prevent seizures, and intramuscular steroid treatment was administered to stabilize fetal respiration after delivery. Despite strict perinatal management, the patient’s preeclampsia became more severe, with increased proteinuria. This prompted an emergent cesarean section at 30 weeks of gestation. At laparotomy, we observed medusa head infiltration of the uterine surface by the placenta along with bulging of the uterine wall, indicating the presence of PAS (). A baby weighing 1,534 g (Apgar scores 5 at 1 min and 7 at 5 min) was delivered through a transverse incision in the uterine fundus, and emergent cesarean hysterectomy was immediately conducted, with no attempt at placenta removal. The total blood loss was 1,370 mL, but maternal condition remained stable and no blood transfusion was required. The patient was discharged in good condition 8 days after the operation. Pathological examination showed villous tissue directly attached to the thin uterine wall, indicating abnormal placental invasion. There was no evidence of villous tissue invasion in the myometrium or uterine serosa (). Later, we obtained the patient’s 9-week ultrasound findings from the hospital she previously visited. These showed an empty uterine cavity and a gestational sac with fetal heartbeat in her cesarean scar (). Our final diagnosis was unexpected PAS caused by a late diagnosed CSP.
51 year old male presented with low back ache with radiation of pain to the right lower limb for 1 ½ months duration. He had pain at rest which aggravated on walking. He walked with a limp. Patient was given epidural injection 3 weeks before he presented to us at another center. There was no relief of pain post injection. Currently he did not have relief with analgesics and physiotherapy and had no relief from epidural injection. There was no history of any previous interventions to the spine or bowel or bladder symptoms. Ankle jerk was absent on the right side. Plantar reflex was equivocal on the right side and down going on the left side. There was no motor power loss or sensory deficit. MRI done showed hypointense lesion that appeared intraradicular in S1 root (arrow mark) with a clear layer of CSF all around the lesion (). Gadolinium MRI did not show any enhancement. There was a clear gap between the dura and the fusiform swelling in the nerve root with no evidence of disc prolapse. A diagnosis of nerve root tumor was made and surgery was advised. Surgery was done with a midline incision with patient in the prone position. Ligamentum flavum on the right side was removed.\nThe upper edge of the S1 lamina was nibbled and the swelling exposed. Due to the long intra spinal course of S1 root, the swelling could be very well exposed.\nThe swelling was seen at a distance from the dura with a definite segment of normal looking nerve root in between (). The swelling was opened longitudinally. Fluffy disc like materials came out from n between the nerve fibres (). No CSF came out from the incision. Material was sent for histopathological examination. The nerve sheath was repaired with 8-0 prolene under microscopic vision. Histopathology report was consistent with degenerated disc material with collagenous tissue showing characteristic cell clusters [] ().\nPost operatively the patient was relieved of his symptoms except for dysesthesia at the sole of foot which remained for one year post surgery. At 5 years follow up the patient has no complains and is doing all his functional activities normally. There is no sensory motor deficient at present.
The case of a nine-year-old female child, who was born with congenital lymphedema of the left leg, is reported. She was referred for treatment when she was one year old. Diagnosis of lymphedema was clinical with perimetric measurements (every 3 cm) along the foot and leg (). Cervical stimulation using the Godoy & Godoy technique was performed until the age of six. It was initially carried out by the medical team but, after teaching the mother, she performed the cervical stimulation with clinical control being carried out by the medical team initially every week, then every fortnight, and eventually once per month. Cervical stimulation is the only lymph drainage technique that has been evaluated in isolation and proven to be efficacious in the reduction of edema. The hypothesis of the mechanism of action is that cervical stimulation causes the contraction of the lymphangions thereby increasing drainage []. Cervical stimulation as monotherapy reduced the perimetry of the limb to within the clinically normal size range without signs of edema in the early morning when the child got up within around three months. At the age of six, a diagnosis of growth hormone deficiency was reached and hormone therapy was initiated. The child began to grow quickly with aggravation of the lymphedema with an increase of 3 cm in the perimetry of the dorsum of the foot. A hand-made low-stretch compression stocking of a cotton-polyester fabric (grosgrain) was added to the treatment regimen. This fabric has low stretch across the material and elasticity in the other direction thus allowing good flexibility of the limb but providing compression. The association of compression to the treatment reduced and controlled the edema. The patient, now nine years old, has been receiving hormones for three years with her leg remaining within the normal size range compared to the contralateral limb. Normalization of the edema using an association of a grosgrain compression stocking and cervical stimulation occurred in about three months ().\nWhen the child wanted to do ballet classes, the mother was advised not to allow her due to repetitive movements involved and the exertion of the foot. As the child insisted, she was permitted and encouraged to use a cotton-polyester compression stocking; changes in the size of the leg were monitored every week, clinically and by perimetry. There were no variations in the size of the leg and the child took ballet classes for more than one year.\nThis study was approved by the Research Ethics Committee of FAMERP (number 3822010).
This 35 year old woman began noticing increasing fatigue in 2014. The fatigue and left lower quadrant pain became increasingly severe so that she went an emergency room. On 19 February 2018, a CT scan was performed that showed an infiltrate into the greater omentum and multiple nodules associated with small bowel mesentery. There was no ascites. Masses were present on both the right and left ovaries. On 14 March 2018, she was taken to the operating room where multiple biopsies were taken. Her left ovary and appendix were removed. Pathology showed a mature cystic teratoma and extensive malignant peritoneal mesothelioma. Multiple biopsies from omentum, surface of uterus, peritoneum from the abdominal wall, and peritoneum from the small bowel showed malignant peritoneal mesothelioma.\nRepeat CT on 2 May 2018 showed malignant peritoneal mesothelioma infiltrating the omentum and accumulating as a diffuse mass in the pelvis. The small bowel mesentery showed multiple prominent lymph nodes. For definitive treatment of her malignant peritoneal mesothelioma, the patient was taken back to the operating room on 3 May 2018. At that time she had a greater and lesser omentectomy, cholecystectomy, pelvic peritonectomy with hysterectomy and right oophorectomy along with cytoreductive surgery of the small and large bowel surfaces. HIPEC was performed with cisplatin, doxorubicin, and systemic ifosfamide with Mesna (2-Mercaptoethanesulfonic acid sodium). During the HIPEC procedure Mayo scissor dissection removed a layer tumor from large and small bowel surfaces along with large and small bowel mesenteric surfaces []. In order to rule out disease within the mesenteric lymph nodes, six of these lymph nodes were harvested during the HIPEC chemotherapy []. Portions of the node were sent for histopathologic analysis and other portions were sent for pharmacologic analysis of the intraperitoneal drug, doxorubicin. The results of the doxorubicin within blood, peritoneal fluid, lymph nodes, and tumor are shown in . The area under the curve ratio of lymph node doxorubicin to plasma doxorubicin was 30. The area under the curve ratio of tumor nodules to plasma was 20 and from peritoneal fluid to plasma was 50.\nAgain in this patient the markedly increased levels of doxorubicin in lymph nodes as compared to plasma were documented.\nThe patient was hospitalized for 15 days and was discharged eating well. An intraperitoneal port was placed at the time of the cytoreductive surgery and will be used to deliver long-term intraperitoneal pemetrexed with systemic cisplatin. Six cycles of treatment are planned. Pathology of resected specimens showed malignant peritoneal mesothelioma; the lymph nodes were negative for cancer.
A 26-year-old female patient was referred to in our clinic from the Internal Medicine department due to intermittent left lower back pain for 2 months and left renal mass. Her medical and family history were unremarkable. Physical examination by palpation was totally normal. Her routine blood investigations were in normal ranges. Routine urine analysis revealed no hematuria. Urine cytology was negative for malignancy. Abdominal computed tomography (CT) revealed a cystic enhanced heterogenic left renal mass about 5 cm in the largest diameter was extending from the renal pelvis to the ureter and causing gross hydronephrosis of the left kidney []. There was no evidence of lymph node or distant metastases. The images favored a renal cell or urothelial-cell carcinoma of the kidney were also considered. After all these findings, we planned to perform transperitoneal left laparoscopic nephroureterectomy and bladder cuff resection. After written informed consent was obtained from the patient, she underwent the surgery with the lateral decubitis position. On the surgery, there was a hydronephrotic left kidney with a mass arising from the pelvis renalis extending into the ureter []. The surgical specimen disclosed the left kidney with a size of 10 cm []. The patient was discharged on the postoperative 2nd day without any complication. The urethral catheter was removed at postoperative 1st week. No recurrence or disease-related mortality was observed in the patient at postoperative 1st year.\nThe tumor was covering entire in the renal pelvis and measured 6.5 cm × 4.5 cm × 4 cm in diameter. The tumor typically contains small and large cysts mixed with solid areas. The lesion appeared to be well-demarcated with no invasion of adjacent kidney parenchyma.\nThe tumor was biphasic with both epithelial and stromal elements. The stromal component was composed of the bland spindle or smooth muscle cells. The stromal portion usually resembles so variant stroma arranged within creased density in pericystic areas. The stromal component was composed of uniform spindle cells without cellular atypia, necrosis, or mitoses. Epithelial components exhibited various-sized tubuloglandular and cystic formations lined by bland flattened cells, hobnail cells, and columnar cells without atypia. Mitotic figures were detected. Immunohistochemically, the stroma cells were positive for estrogen and progesterone receptors, α–smooth muscle actin, desmin, common acute lymphoblastic leukemia antigen 10, and vimentin. Cystic components expressed cytokeratin 7, paired box 2 and 8, and high molecular weight cytokeratin antibodies []. The pathological and immunohistochemical findings were compatible with the diagnosis of MESTK.
A 41-year-old non-smoker obese female patient was examined at the Department of Neurology and the Center for rehabilitation. Relevant history of the patient is summarized on a timeline in Fig. . She showed an onset of neuromuscular disorders during early childhood with a delay in motor and written language development. She had a hard time to complete physical exercise at school and always finished last. There was nothing really alarming in terms of muscle disorder until her first pregnancy at the age of 33 during which she experienced shortness of breath (dyspnea) at the 7 months of gestation and showed signs of weakness of lower limbs afterwards. At the age of 34 she showed worsening of breath symptoms, suffered from sleep apnea and started using a mechanical ventilation machine. Alarming symptoms of skeletal muscle disorders occurred immediately after delivery with progressive but rapidly incapacitating weakness of lower limbs. This worsened during the 3 following years, a period during which she first started being unable to get up by herself and then was unable to climb stairs. At the age of 37, she started to use a cane, then a walker for her daily walking needs. At the age of 39, she started using a wheelchair to move outdoors. Despite these signs of progressive muscle weakness, she never had a thorough neuromuscular investigation. It was wrongly thought that her health problems, in general, was mainly related to being overweight. At the age of 41, she had four episodes of lower limb paralysis during which she was completely unable to move her legs and support her weight. She did not seek medical consultation for the first three episodes. For the fourth, she was admitted to the emergency room (ER) and first referred to the Department of Neurology and then to the Center for Rehabilitation.\nThe family history showed that her mother died at the age of 66 from a heart attack associated with non-compaction cardiomyopathy. There is nothing remarkable in terms of muscle disorders in her father, brother, and sister. However, her only daughter, now at the age of 11, shows signs of muscle disorders with congenital muscular torticollis, excessive growing pains as well as underdeveloped muscles in half of the body.\nPhysical examinations conducted following her admission to the ER at the age of 41 showed normal tone/bulk of the arm muscles. However, muscles in shoulders and upper and lower limbs showed bilateral weakness. Deltoids, biceps and triceps showed moderate weakness with MRC scale of 3/5. Fine finger movements were intact. There was no pronator drift. In the lower limbs, all muscles examined showed the same severity of weakness (2/5) except quadriceps which showed mild weakness (4/5). Hip flexors were extremely weak (1/5); hip abductors and adductors were mildly weak (4/5). Knee extensors and flexors were moderately weak (3/5). Dorsiflexion of feet was severely weak (2/5). Plantar flexors were severely weak (2/5). Deep tendon reflexes were 2+ in the arms, absent in the patella and Achilles. The toes were down-going. No sensory deficit was observed. No sign of dysphagia or involvement of ocular muscles was observed.\nAdditional physical examination conducted during the 2-year follow-up (at the age of 43) showed no worsening of muscle strength. However, this brought new information about the weakness of other muscles, notably in the shoulders, with extremely weak abductors and flexors (1/5). The patient showed decreased perception of vibration in the lower limbs. She reported that she experienced occasional dysphagia.\nElectrophysiological exams showed normal nerve conduction velocities for upper and lower limbs, with all SNAP and CMAP amplitudes in the range of normal values. However, needle EMG revealed a tendency of myotonic potentials, generated by needle insertion. Right deltoids, biceps and ulnar-innerved first dorsal interosseus muscles showed myotonic potentials as well. The right tibialis anterior showed 2+ polyphasic motor unit potentials with myotonic potentials. The right medial gastrocnemius, vastus medialis and vastus lateralis showed myotonic tendencies as well.\nExamination of muscle biopsy of left quadriceps showed features of an end-stage process, consistent with a severe, chronic myopathy. There are scattered clusters of viable muscle fibers which showed myopathic features in the form of a marked variation in fiber size and numerous internal nuclei. In addition, there are scattered fibers with abundant intrasarcoplasmic vacuoles (Fig. a, b). Given we could not clearly observe the presence of hyaline bodies on H&E staining we decided to proceed with p62 immunostaining, a well-known technique for revealing the presence of inclusion bodies []. Immuno-histochemical detection of p62 was performed on a Leica Bond III automated stainer. Following digestion in a low pH citrate solution, sections were incubated in a primary mouse monoclonal anti-p62 antibody, diluted 1:50 (BD Transduction Laboratories Catalog Number 610833). Detection of bound antibody was achieved using the Leica Bond Polymer Refine Detection kit, comprising the secondary antibody, the substrate chromogen DAB (3,3′-Diaminobenzidine tetrahydrochloride hydrate) and the Hematoxylin counterstaining solutions. Immunostaining for p62 revealed, in a proportion of the surviving muscle fibers, diffusely distributed, small intermyofibrillar dots or, more commonly, larger central or eccentric sarcoplasmic inclusion bodies (Fig. c, d). Examination of heart function using Holter ECG monitor carried out following her admission to the ER showed no clinically significant implication of cardiac involvement. However, the patient complained about repeated episodes of oppressive chest pain during the following years. Additional examination conducted during the 2-year follow-up showed signs of cardiac involvement with bradycardia of 58 beats per minute.\nRegarding respiratory involvement, the patient suffered from dyspnea since the age of 34. Examination of lung function conducted following her admission to ER and during the follow-ups showed worsening of her respiratory condition. Spirometry test conducted at the age of 44 showed a very weak pulmonary function, with forced vital capacity (FVC) of 27% and forced expiratory volume in a second (FEV1) of 29% of normal values.\nThe blood samples were collected for whole-exome sequencing (WES) to detect mutations potentially involved in the phenotype of neuromuscular disorders observed for this patient. The genomic DNA was extracted from whole blood and subsequently subjected to whole-exome DNA library construction using the Ion AmpliSeqTM Exome RDY panel (Thermo Fisher Scientific) essentially as described in the manufacturer’s protocol, with barcode incorporation. For the sequencing, samples were loaded on an Ion HI-Q PI Chip v3 and placed onto the Ion Proton instrument (Thermo Fisher Scientific) together with an Ion PI HI-Q sequencing 200 Kit (Thermo Fisher Scientific) and sequenced for 520 cycles according to the manual (See Additional file : Table S1 for parameters). All candidate mutations found by WES were validated by direct Sanger sequencing (See Additional file : Figure S1 for the filtering process). DNA sequences were obtained from the University of California Santa Cruz (UCSC) Genome Browser. Predesigned primers were directly purchased from Thermo Fisher Scientific (See Additional file : Tables S2 for details). Amplicons were sent to Genewiz () for Sanger sequencing.\nWhole exome sequencing showed that a novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg) in the MYH7 gene is a missense single nucleotide variant possibly linked to the clinical findings, found in the DNA of the patient as heterozygous (Fig. ). The novel variant has been submitted to ClinVar database; with the assigned accession number SCV000804311. Two other candidate mutations were identified in the DNA of this patient, namely NM_003085.4:c.368C > A (p.Pro123His) in the SNCB gene and NM_001001557.3:c.746C > A (p.Ala249Glu) in the GDF6 gene (Additional file : Table S3). However, both mutations were discarded after filtering only genes involved in neuromuscular functions that potentially cause clinical features of muscle myopathy observed in this patient (Additional file : Figure S1). Regarding the novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg), bioinformatics analyses showed that nucleotide T coding at the position 1370 of the MYH7 gene is highly conserved across 44 vertebrate species (PhyloP at 1.76). Amino acid substitution from Isoleucine (I) to Arginine (R) at the position 457 of MyHCI suggested a high impact on protein structure (Grantham at 97). Results of analyses obtained from VarSome () suggested a classification of Likely pathogenic for this variant, with evidence of Pathogenic computational results coming from 8 various prediction software including DANN, GERP, dbNSFP.FATHMM, MetaLR, MetaSVM, MutationAssessor, MutationTaster and PROVEAN (vs no benign predictions). Also, the results of analyses using the recommendation of the ACMG and the AMP suggested the same classification of Likely pathogenic for this variant, with combined criteria of 2 moderate (PM1 and PM2) and 2 supporting (PP2 and PP3) [].
A 35 year old female was referred to the oral and maxillofacial pathology department with a chief complaint of painless, non healing oral ulcers on the left buccal mucosa for the last five months duration, which had increased in size. Her detailed medical history revealed that she had experienced regular weight loss (around 3 kg) over the past three to four months. She also complained of cough and feeling of malaise during the past 15 to 20 days. However, her family history was not contributory and she was not on any kind of systemic medication.\nOn extra oral examination, a single cervical lymph node of left side was palpable and enlarged; however, there was no sign of tenderness or fixation to the surrounding tissues (). Intra orally, there was an ulcer on the right buccal mucosa measuring about 1.5 × 1.5 cm in dimension with a shallow ulcerated base and well defined margins. The ulcer was covered by a yellow pseudomembrane and surrounded by an erythematous halo (). There was no other abnormality elsewhere in the oral cavity. Based upon the clinical examination, a differential diagnosis included aphthous ulcer, traumatic ulcer, infections (bacterial, fungal and viral), drug reaction and malignancy, including primary squamous cell carcinoma and lymphoma. Since there was no history of any kind of trauma and the ulcers were chronic, painless and non recurrent, the possibility of traumatic or aphthous ulcers were ruled out. Moreover, the patient was not on any systemic medication; thus the possibility of ulcer due to drug reaction was also ruled out.\nAn incisional biopsy of the ulcer under local anesthesia was performed. Histopathologic examination of the excised specimen showed an ulcerated stratified squamous surface epithelium in association with fibro vascular connective tissue. The connective tissue exhibited granulomatous inflammation containing epithelioid cells, Langhans giant cells and lymphocytic infiltrate with areas of necrosis (Figures and ). This raised the possibility of granulomatous infection, including tuberculosis, sarcoidosis or fungal infection. Subsequent stains for fungi (PAS and Grocott's Silver) and bacteria (Gram stain) were negative. However several acid-fast bacilli were identified with a Ziehl-Neelsen stain, in the sputum ().\nBlood tests were within normal limits except for a raised white cell count (11.1× 109) and raised erythrocyte sedimentation rate (95 mm/hour). The hepatitis C virus test, VDRL (Venereal Disease Research Laboratory) and HIV test were negative. An ELISA (Enzyme-Linked Immunosorbent Assay) test confirmed the presence of antibodies against mycobacterium tuberculosis. However chest X ray did not reveal any characteristic finding. These features were consistent with those of tuberculous granulomatous lesion.\nBased on all the above observations, patient was referred to a physician who initiated a WHO recommended category 1 anti-tubercular therapy DOTS (Directly Observed Treatment, Short Course) with rifampicin (450 mg), isoniazid (600 mg), ethambutol (1200 mg) and pyrazinamide (1500 mg) for two months with three times doses per week, followed with continuation phase with isoniazid (300 mg) and thioacetazone (150 mg) for six months. The patient was reported to our department after 6 months with relatively normal buccal mucosa.
A 26 year old female has been referred to our clinic with right sided hip pain of sub acute onset. Her pain worsened with movement and weight bearing. On clinical examination, she had restricted range of movement with pain and mild joint tenderness. On plain x-ray she found to have AVN of right femoral head suggested by classical crescent sign with preserved articular margins on plain film and underwent extensive evaluation for a cause. Apart from malaise, fatiguability and moderately elevated erythrocyte sedimentation rate (ESR) of 65 mm in 1st h no other symptom or laboratory feature could be found. Her hemoglobin level was 10.6 g/dL and white cell count, platelet count, C-reactive protein was normal. After 6 months she presented with a similar pain in left hip and subsequent work up revealed AVN of left femoral head as well on plain x-ray and findings were similar to the previous right sided hip joint. Other than above presentation she had no other complains at that point. Her past medical history was not significant, she gave birth to a healthy term baby 7 months prior to above presentations and her obstetric history was uneventful. She did not consume alcohol and was a non-smoker. She was not on any form of corticosteroid or other medications prior to the event except nutritional supplements during antenatal period and she has not taken any indigenous medicines. There was no family history of connective tissue or other autoimmune condition of note. At the time she of the second event of hip pain she underwent a comprehensive work up with involvement of a multidisciplinary specialists. In view of persistently high ESR and multiple AVN of bones, she underwent a work up for chronic infections such as tuberculosis, and for autoimmune connective tissue diseases with serological studies including antinuclear antibody (ANA), Anti Smith antibody (Sm), serum complements C3 and C4, Anti Ro and Anti La antibodies. She was also screen for Human Immunodeficiency Virus (HIV) infection, Hepatitis B and C infections. Hematological evaluation for sickle cell disease, thrombophilic conditions including anti-cardiolipin antibodies IgG and lupus anticoagulant test, beta-2 glycoprotein 1 both IgM and IgG. All of the above tests were negative. A probable cause could not be ascertained despite above extensive workup. She has been continuously followed up with a future plan of hip joint replacement. During the subsequent period patient revealed that she felt mild fatiguability and hair loss continuously, which she considered not significant and did not report to the doctor hence further workup was not done for next 4 years. She did not take any medications except simple paracetamol for hip pains. After about 4 years of above events she reported significant hair loss, which was unusually high according to her. On further assessment she found to have isolated thrombocytopenia of less than 100,000/mm3 on repeated blood counts and also continuously high ESR, at times exceeding 100 mm/1st h. Patient was re-evaluated considering above developments. She had positive ANA with titre above 1:160 and a positive anti double stranded DNA (dsDNA) antibody with titre of 233 IU/mL. Both these tests were negative 3 years back. She did not report abnormality in urinalysis or neuropsychiatric manifestations of SLE. In light of above clinical manifestations and serological studies, definite diagnosis of SLE was made according to SLICC 2012 classification criteria. Her coagulation screening was repeated and was negative although beta-2 glycoprotein 1 was not repeated. Her initial presentation of bilateral AVN of femoral head 4 years back was ascertained as related to evolving autoimmune disease, since no other etiology was evidenced. Patient was prescribed hydroxy-chloroquine and simple analgesics in her subsequent management and total hip joints replacement surgery was planned.
The patient is a 57-year-old female who underwent cardiac catheterization via the right common femoral artery two weeks prior to developing a large, symptomatic right common femoral artery pseudoaneurysm ().\nThe patient began complaining of groin pain two weeks after cardiac catheterization. She has a past medical history of aortic valve replacement secondary to aortic valve infective endocarditis, hyperlipidemia, and hypertension.\nShe underwent two attempts of ultrasound-guided thrombin injection of the pseudoaneurysm. On ultrasound, the size of the pseudoaneurysm was found to be 5 cm × 3 cm × 4.6 cm. The neck of the pseudoaneurysm was measured to be 0.8 cm long. The two attempts involved using a 21 gauge needle to administer 1000 units and 2000 units of thrombin, respectively, into the pseudoaneurysm under ultrasound guidance and with the assistance of compression. Due to the size of the aneurysmal cavity and a relatively large pseudoaneurysm neck, injections were found to be unsuccessful on follow-up ultrasound (Figures and ). It was then decided to attempt endovascular closure of the neck of the pseudoaneurysm. All risks were discussed with the patient.\nAfter identification by the attending surgeon, the patient was transferred to the procedure room table in the catheterization lab. The patient received IV sedation, and local anesthesia was used prior to ultrasound-guided percutaneous access to the left common femoral artery. During the procedure, vital signs, including blood pressure, heart rate, respiratory rate, and oxygen saturation, were monitored by an ACLS certified nurse.\nAfter a 21 gauge needle was placed into the projection of the vessel lumen, a guidewire was placed into the left iliac artery. An angiographic catheter and guidewire were used to perform selective cannulation of the contralateral right common iliac artery. Then, a 6 French long access sheath was placed to perform an angiogram. The neck of the pseudoaneurysm was visualized (), and a 0.014 guidewire was placed into the proximal portion of the neck.\nA 21 gauge needle was used to cannulate the proximal portion of the neck percutaneously from the right groin. The previously placed guidewire was used as a landmark to place the tip of the 21 gauge needle into the pseudoaneurysm. After blood return was noticed from the needle, a 0.018 guidewire was placed into the lumen of the right common femoral artery. A 6 French access sheath was placed over the guidewire. Fluoroscopy was then used to visualize the deployment of a vessel closure device (VASCADE 6 French). This was done without difficulty, and the collagen patch was positioned outside the vessel wall in the area of the pseudoaneurysm neck. Interval angiogram revealed partial occlusion of the pseudoaneurysm neck ().\nIt was then decided to place an occlusive 8 mm balloon into the lumen of the right common femoral artery to facilitate pseudoaneurysm thrombosis. The balloon was insufflated up to 8 ATM for 600 seconds. This was done twice in total. Interval angiogram then revealed complete occlusion of the pseudoaneurysm blood flow (Figures and ).\nAll wires and catheters were removed at this point, and a left common femoral artery access sheath was kept in overnight. Postoperatively, the patient had no complications, and formal ultrasound confirmed complete thrombosis of the pseudoaneurysm. The access sheath was then removed without issue. There were no ischemic complications due to balloon occlusion in the immediate postoperative period.
A 4-month-old boy was admitted to our university hospital due to an enlarged cervical mass following carotid attempt at interventional repair of aortic coarctation diagnosed via transthoracic echocardiography. The patient had previously been referred to the pediatric catheterization lab for the definite repair of the coarctation as well as pressure gradient correction; however, initial attempts at introducing the guidewire into the arterial system (first femoral, then brachial, and finally right carotid) failed. The patient was thereafter transferred to the ward for further treatment, but his parents chose to take him home. Three weeks later, the patient was admitted to the operating room for an emergency operation to evaluate and possibly evacuate the mass and also to assess and repair the carotid artery rupture and coarctation ( and ). Before the decision for surgery had been made, a Doppler examination of the carotid artery ruled out a pseudoaneurysm of the carotid artery under the hematoma. Volatile anesthetics, supplemented with ketamine, were planned for the patient; and under standard non-invasive monitoring, 3mg/Kg intramuscular ketamine plus 1.5% halothane was administered. With the patient breathing spontaneously under deeper levels of anesthesia, laryngoscopy showed that the glottis was not difficult for intubation. A peripheral intravenous line was established for muscle relaxants, and the trachea was intubated without any need for extra devices. Additionally, an arterial line in the right brachial artery was established to commence invasive arterial monitoring. The left subclavian approach was chosen for the insertion of the central venous line at the first attempt, which demonstrated the central venous pressure waveform.\nThe patient was operated on in the supine position with a roll below the neck to extend the neck gently and produce a “thyroid position”. The surgical approach was initially based on making the proximal and distal control of the right external carotid, but the hugeness of the cervical mass rendered it impossible. The skin over the mass was, therefore, gradually dissected and the mass, which was a hematoma around and inside the sternocleidomastoid muscle, appeared ( and ). The hematoma was evacuated and the bleeding site, the punctured right external carotid, was repaired by direct suturing. Subsequently, the sternocleidomastoid muscle was repaired, a hemovac drain was inserted, and the overlying skin was sutured.\nThe patient was then placed in the right lateral decubitus position for a thoracotomy approach both to repair the coarctation of the aorta with a subclavian flap angioplasty and to close the patent ductus arteriosus (PDA). After this surgical procedure, the pressure gradient at the level of the coarctation was corrected, the left lung was re-expanded, the thorax was closed, and the skin was sutured.\nThe patient was transferred to the pediatric cardiac intensive care unit and was extubated the following day. Transthoracic echocardiography was indicative of no significant gradient across the repaired site of the coarctation, and the patient was discharged to the ward after 3 days. At three weeks’ follow-up, there was no significant lesion in the neck and transthoracic echocardiography demonstrated no residual coarctation.
A 34-year-old man was referred to our surgical unit from a cottage hospital on the day he was involved in a road traffic accident as a pedestrian. He was going to work when he was hit by an overlapping van along the road. He fell down and the van ran over his left thigh and left inguinal area. He lost consciousness and found himself at a private hospital where initial resuscitation was performed and he was transferred to Kenyatta National Hospital. On examination he was found to be dehydrated, had peritonitis, degloving injury over the left inguinal area, and bruised perineum. Radiological investigations revealed pelvic fracture (). He was optimized and laparotomy was performed a day after admission. Intraoperatively, the sigmoid colon was found to be bruised and perforated, 10 cm of which were resected and Hartman's colostomy performed. Debridement of the injured left inguinal abdominal wall left a 5 cm by 10 cm defect (). An attempt was made to stitch it to the skin over the thigh. On the third postoperative day the attempted stitching gave way due to infection. The patient also started to bleed on an off from the pelvic fracture through the defect. The bleeding was managed by tranexamic and packing and later stopped by the tenth postoperative day. The wound was managed by daily dressing until it was well granulated by the fifth month after the injury. Decision was made to reverse the colostomy and close the abdominal wall defect at the same time to save time and money. Colostomy reversal and pedicled tensor fascia lata flap were used to close the defect in the anterior abdominal wall defect. Split thickness skin graft was used from the same thigh to close the other defect. On the fifth postoperative day he developed burst abdomen. This was repaired a day later after optimization and healed well. The flap and the graft took well (). He has been discharged and is on follow up in the surgical outpatient clinic with no complications.
A 45-year-old man presented to our attention for a large pulsatile formation on the volatile face of left forearm, which he reported to have enlarged in the last year (Fig. ). At examination, we observed absence of radial pulse and direct flow on the ulnar artery at continuous-wave Doppler, and a large pulsatile formation was evident along the course of the left brachial artery, associated with forearm venous dilatation. Further Doppler ultrasound (DUS) examination showed a fusiform aneurysm of the brachial artery with a maximum diameter of 3.5 cm and longitudinal extension of 5 cm up to the brachial bifurcation, with thrombus formation, which partially occluded the brachial artery lumen and the origin of the radial artery, which was completely occluded. The cephalic vein after AVF was patent and dilated. The patient had a history of chronic renal impairment “failure” caused by glomerulonephritis in 2000, then AVF for dialysis was realized in other institution, and finally he was addressed to kidney transplantation in left iliac fossa in 2004. In 2011 the AVF was ligated with suture on the radial artery and the dilated cephalic vein was left in place. A DUS examination performed in 2018 showed patency of the brachial artery with a diameter of 3.5 cm and patency of the ulnar and radial artery up to the wrist. He also had a computed tomography scan without media contrast, which confirmed the DUS report.\nConsidering the presence of the diffuse thrombus in the brachial artery and the occlusion of the radial artery occurring in the last year, we decided to remove the brachial aneurysm with a venous bypass on the ulnar artery. The left great saphenous vein (GSV) was studied at DUS and resulted suitable as a graft. The patient was treated under general anesthesia. We first exposed the brachial artery that appeared tenaciously adherent to the surrounding tissues from the midarm to its bifurcation with a bilobed appearance, and satellite brachial veins were collapsed and flattened by the aneurysm. The radial artery was occluded at its origin, and the ulnar artery was soft and pulsating, suitable for bypass anastomoses. Then a proximal portion of about 20 cm of GSV was removed in the left groin. The radial artery was ligated at the origin. An aneurysmal section of the brachial artery of about 15 cm was removed. End-to-end anastomosis between the venous graft and the brachial artery and the ulnar artery was realized with running polypropylene 6/0 sutures (Fig. ). The entire aneurysmal cephalic vein was then removed. The patient was discharged in good general condition on the second postoperative day.\nOne-month control ultrasound showed complete resolution of the aneurysm and graft patency. Clinical examination showed normal upper limb motility, direct flow on the distal ulnar artery, without any neurological impairment, with a good esthetic result. At 6-month follow-up the patient had complete recovery, without any further complications (Fig. ).
This patient is a 40-year-old male who works in an office and spends most of his time seated behind a desk. Approximately 2 years prior to the start of this study, he caught a bad cold, after which he began to experience severe dizziness. He then began experiencing a variety of symptoms, including vertigo, ringing in the ears, headaches, severe nausea and vomiting, especially while performing rolling or rotation movements. His symptoms were particularly serious in the evening. He felt a loss of ability to focus on his surroundings and experienced difficulty concentrating on targets. In addition, he reported feeling particularly dizzy when performing head rotations while engaged in close dialogue with others, and he felt especially uncomfortable in crowded places. His family members also noticed that he could not walk or swim in a straight line. He gradually lost the ability to perform the head movements necessary for breathing while free-style swimming. The patient emphasized that his balance was good prior to the onset of the disease and that he had not previously experienced symptoms of dizziness. His dizziness had become a major obstacle in his work and daily life. The caloric test results 1 week prior to the study showed a left-sided canal paresis of 34%.\nDuring the training process, this patient was able to adapt quickly. Although he did experience slightly increased discomfort after training in the 1st week, he quickly adapted and completed his 2nd and 3rd weeks of training. He was able to perform balance board and tandem stance training. By the end of the 5th week, he was able to complete the exercise at head rotation speeds of 130-140o/s. Significant improvements were observed with respect to decreased discomfort and dizziness at work and during daily life activities.\nOne month after the completion of training, the patient was able to complete the DGI and POMA assessments quickly. Although he still exhibited a slight shift in the horizontal head movement assessment, his confidence and activity level had both significantly improved. He still reported feeling slightly dazed at times, but he was generally able to conduct daily activities and perform his regular work duties smoothly.
A 5-year-old female fell down on a pen while playing. The tip of the pen became lodged inside the right orbitocranial region. Her parents did not witness this injury initially, and there was no history of any neurological symptoms. At first, she was treated in a local clinic with simple suturing of the laceration at the upper eyelid (). Four days later, she presented to a local hospital with orbital swelling without fever where she was found to have a retained orbitocranial foreign metallic body. She was started on antibiotics and subsequently referred to our hospital for further management. Upon arrival, she was complaining of pain from the right eye with no associated neurological symptomatology. On clinical examination, she was afebrile and all other vital signs were stable. Sutured laceration was noted at the superior medial upper eyelid. Ophthalmological examination revealed restriction of the upward gaze due to muscle entrapment; the globe was slightly pushed downward due to the swelling and muscle entrapment (). No cerebrospinal fluid (CSF) leakage was noted. She had a Glasgow coma score (GCS) of 15 with no neurological dysfunction. Computed tomography (CT) scans of the brain and orbits showed a 20 mm × 15 mm metallic foreign body attached in the medial portion of the right orbital roof, representing the pen tip (). The foreign body's distal end was penetrating the right frontal lobe inferior surface, whereas the proximal end was situated at the upper orbital cavity. A rim-like enhancement was seen at the frontal lobe around the foreign body tip suggestive of an abscess formation surrounded by cerebral edema (). Her laboratory investigations did not reveal any leukocytosis in the blood, and the result of the blood culture was negative. The patient was kept on an antibiotic regiment of vancomycin, ceftazidime, and metronidazole.\nAfter one day of being admitted to our hospital, she underwent foreign body removal and debridement of the brain abscess. Transcutaneous upper eyelid surgery was carried out by a team that included both oculoplastic and neurological surgeons. Upper eyelid skin crease was opened exploring the superior subperiosteal space, exposing the proximal end of the foreign body that comprised the pen's metallic tip and pieces of glass from the pen's shaft (). The pen tip was attached to the superior orbital roof, and after removal, CSF leakage and minimal purulent discharge were noted. The dura was opened, and the pen tip penetrated the frontal lobe inferior surface. Ink from the pen was noted on the brain and orbital muscles. Debridement and irrigation were performed at the tract of the penetrating wound. A small piece of surgical patch was placed between the bone and the dural opening to prevent CSF leakage into the orbit. Finally, the wound was closed and dressing was applied ().\nPostoperatively, she had transient periorbital swelling. The culture from the brain abscess was negative. This was likely due to the antibiotic treatment initiated prior to this patient's transfer to our hospital. Subsequently, she had full extraocular movement with improvement of the upper-gaze limitation, and the wound healed well. She was last seen 2 months after the surgery, and she was having mild right-eye ptosis. Her CT scan followup showed resolution of the brain abscess and edema after a course of 3 weeks of antibiotics ().
A 7-month-old boy presented with an abdominal mass since birth which gradually increased in size to occupy nearly whole of abdomen. There was no history of hematuria. Antenatal history revealed that a left renal mass had been detected on ultrasound at 7 months of gestation [] and the child was discharged on day 3 of life without further investigations.\nOn examination, the child had a poor general condition and was malnourished. There was distension of abdomen due to the large intraabdominal mass. Ultrasound of the abdomen revealed a predominantly cystic 16 cm × 13 cm × 10 cm left renal mass and the findings were confirmed by contrast-enhanced computed tomography []. The right kidney and the inferior vena cava were normal. The chest radiograph showed clear lung fields with the pushed up diaphragm. Fine needle aspiration cytology (FNAC) was not performed as the mass was predominantly cystic. A provisional diagnosis of the cystic renal tumor was made and the child was taken up for nephroureterectomy. Exploratory laparotomy revealed a large primarily cystic left renal mass with no evident extracapsular extension or lymphadenopathy []. Left nephroureterectomy with lymph node sampling was performed but despite careful tissue handling, inadvertent tumor spill occurred due to rupture of cystic lesion with extremely thin walls. Right kidney did not have any gross lesion. Histopathological examination demonstrated a favorable histology triphasic Wilms’ tumor with rhabdomyoblastic differentiation []. A final diagnosis of stage III left Wilms’ tumor was made and the child was started on DD4A (vincristine, actinomycin D, and adriamycin) chemotherapy regimen of NWTS adjusted for age and abdominal irradiation was planned. Before the child could receive radiotherapy, a nodule at the incision site and abdomen distension was noticed when the child was admitted for the third cycle of chemotherapy []. The FNAC from the incision site nodule revealed a small round cell suggestive of blastemal cells []. The contrast-enhanced CT scan of abdomen showed a large heterogenous mass filling almost whole of abdominal cavity with involvement of the retroperitoneal and the pelvic regions. Contrast-enhanced CT scan of chest was normal. The child was subsequently started on salvage chemotherapy which consisted of ifosfamide, carboplatin, and etoposide (ICE) at age-adjusted dose. The child tolerated the chemotherapy poorly and needed prolonged GM-CSF administration because of pancytopenia. There was a little decrease in the size of the mass after completion of two cycles of chemotherapy and the child presented with a sudden increase in abdominal distension, severe pallor and shock. Abdominal ultrasonography revealed gross ascitis probably due to tumor rupture. The child expired despite active resuscitation.
Our patient is a 36-year-old female with a history of adult onset seizures. She was born and raised in Mexico and emigrated to the United States at the age of 8. Since arriving to the United States, she had never returned to Mexico. She had her first seizure about 10 years before presentation, at which point she was placed on anti-epileptic medications that were subsequently weaned off without any further seizure activity in the past several years. No prior imaging was available for our review, but the patient denied report of any intracranial lesions that could be identified as the culprit of the adult onset seizures.\nShe initially presented to an outside institution with progressively worsening bilateral headaches over the past year that were associated with vertigo and intermittent nausea. The headaches were worse at night and on waking up in the morning, and they improved throughout the day. A computerized tomography (CT) scan demonstrated ventriculomegaly and transependymal flow, with evidence of obstruction at the level of the fourth ventricle. She was referred to our clinic by our neurology colleagues, where she was found to have no neurologic deficits on examination. Outpatient magnetic resonance imaging (MRI) demonstrated obstructed hydrocephalus secondary to a lobulated cystic mass within the fourth ventricle, with a streak of internal linear enhancement most consistent with racemose NCC. A worm was easily identified within the cyst on the MRI [].\nGiven the chronic nature of the lesion and her benign clinical examination, she was scheduled for elective surgery a few weeks later. She underwent right frontal burr hole for endoscopic third ventriculostomy and placement of external ventricular drain (EVD), then she was positioned prone and underwent suboccipital craniotomy for resection of the fourth ventricular cystic mass followed by duraplasty. The cyst was removed as a single en bloc specimen, although during the process of removal, a small portion of the cyst ruptured into the ventricle – this was quickly suctioned and extensively irrigated. Gross examination [] and pathology [] demonstrated a mature T. solium plerocercoid larvae measuring 1.2 × 0.5 × 0.2 cm encased in a tan, gelatinous, cystic membrane.\nPostoperatively, she had no neurologic deficits. Her EVD was quickly weaned with minimal drainage and low intracranial pressure, so it was removed on postoperative day 2. She underwent ophthalmologic examination, demonstrating no evidence of an active ocular infection or involvement of NCC, but concern for a chronic, inactive choroidal inflammatory process. Our infectious disease colleagues were consulted and found no other risk factors for NCC aside from living in Mexico until the age of 8, and she was negative for tuberculosis infection. Given her longstanding history of seizures, but recent presentation with an active lesion, it was felt that she may have been auto-infecting. She underwent a 21-day course of albendazole with a 14-day dexamethasone taper for protection against aseptic meningitis. She was also prescribed a single dose of praziquantel at the end of her therapy given concerns for possible auto-infection from her gastrointestinal system. On discharge, her headaches, vertigo, and nausea had completely resolved.
A 40-year-old man with progressive dyspnea was referred to our center for evaluation of a giant mediastinal mass. The only other symptom was cough occurring ten months prior to admission. The patient had no underlying disease, no positive family history, and no drug consumption. Clinical evaluation revealed decreased left-sided lung sounds with no adenopathy and organomegaly and no signs indicative of heart compression. Results of laboratory tests were normal as well as cardiac echocardiography and abdominal sonography.\nChest X-ray () showed a large left-sided mass density with obvious mediastinal shift including trachea and heart displacement with barely visible lung in the apex and no other lesions in contralateral lung or ribs including calcification, cavity, and nodule.\nChest computed tomography (CT) () showed a large heterogeneous fatty mass in the left hemothorax with mediastinal shift, complete collapse of the lung, left hemidiaphragmatic inferior displacement, and no tissue invasion. The huge size of this tumor made it very difficult to have a correct preoperative diagnosis regarding the exact location or origin of the mass. The case was discussed with pulmonologist and oncologist colleagues and the surgical resection of the mass was recommended with no needle biopsy attempt. We decided not to perform magnetic resonance imaging (MRI) or positron emission tomography (PET) as well as fine needle biopsy aspirate (FNAB) in our patient, as these would not have changed the management plan or the need for surgery in an uncomplicated patient.\nTotal excision of the mass was performed via a left posterolateral thoracotomy, although the tumor was too large to be resected as an encapsulated lesion (). No pleural fluid and no suspicious mediastinal lymph nodes were observed and no metastatic lesion was detected. Pathologist examination showed a four kg fatty tumor measuring 40 × 33 × 8 cm covered by a thin layer of capsule composed of large lobules of mature fat cells with no atypia and interspersed thymic tissue. Adipose and thymic tissues had no evidence of malignancy ().\nThere was no postoperative complication and the patient remains alive and disease-free, twelve months after the intervention.
A 61-year-old woman had the third operation of revision hip arthroplasty for displacement of the cup from a previous total hip arthroplasty that had been performed when she was 43 years old. The previous implant was removed and a new implant was inserted through a posterolateral approach. In her previous two surgeries, there were no problems with unexpected bleeding. In the blood tests before this current operation, there were no abnormal values including coagulation tests: APTT was 27.1 sec, and PTINR was 0.87. The operation lasted 500 minutes and the total bleeding during the surgery was 3060 grams. Before leaving the operating room, no problems related to the surgery were noted. In her hospital room, the outflow from the drain was 1110 grams at the postoperative time of 8 hours. Her blood pressure did not decrease, and she did not have DIC. At postoperative day 3, an outflow from the drain was 250 grams, and thus we decided to remove the drain. After removing the drain tube, the surgical wound was not inflamed, the patient was not in pain, and her blood tests showed normal values. But, at postoperative day 7, suddenly, she complained of severe thigh pain and her thigh was swollen. We suspected that postoperative bleeding had continued, but her blood hemoglobin was 6.7 g/dL which was almost the same value as her previous test. Based on these findings, we did not provide any treatment. At postoperative day 18, her thigh pain naturally disappeared. At postoperative days 23 and 27, she complained of severe thigh pain and her thigh was swollen again. We thought that the cause of the thigh pain and swelling was arterial bleeding and tested for this complication with computed tomography using a contrast medium. Active bleeding was not observed; however, a hematoma around the hip joint was visualized by this method (). Thus, we punctured the hematoma and 150 grams of uncoagulated bloody liquid was aspirated (). Because the thigh pain was gradually relieved, we did not do any further treatment. Her thigh remained slightly swollen. She was transferred to a rehabilitation hospital. At the rehabilitation hospital, the hematoma enlarged gradually and she again complained of thigh pain. Therefore, a doctor at the rehabilitation hospital punctured the hematoma and about 50 mL of uncoagulated bloody liquid was aspirated. Because the bloody liquid continued to flow from the puncture needle hole, she was admitted back to our hospital (). We still thought that the cause of the thigh pain and swelling was an arterial bleed, but this was not observed by computed tomography using a contrast medium. We planned to perform angiography to see if she had a transcatheter arterial embolization. Before the angiography at postoperative day 115, we completely removed the hematoma with a 2 cm surgical skin incision. We removed about 1,000 grams of uncoagulated lightly colored bloody liquid (). During the surgery, it is easy to confirm internal bleeding, but we did not find any active bleeding. Three days later, the hematoma had enlarged gradually and the thigh pain returned (). In light of the previous case, we suspected acquired coagulation factor XIII(13) deficiency and tested factor XIII(13) activity. Coagulation factor XIII(13) activity was 49% (normal value 70%~140%). As a countermeasure to the bleeding due to coagulation factor XIII(13) deficiency, we decided to give her coagulation factor XIII(13) for five days after surgical removal of the hematoma. We completely removed the hematoma via the same 2 cm surgical skin incision. Five days later, the hematoma enlarged slightly, but she did not have thigh pain. Thus, after surgery she had a blood transfusion of 12 units fresh frozen plasma in all for three days after she had the blood product factor XIII(13) for five days. At the time when administration of blood product was completed, her factor XIII(13) activity was 200% (normal value 70%~140%). Because the hematoma did not enlarge and she did not have thigh pain, she left the hospital and went home. Until now, we have not seen a recurrence of the hematoma.
A 36-year-old female jumped from a four-foot high deck landing flatly on both feet. She felt a pop in the right knee and experienced extreme pain and inability to bear weight on the limb. She sought emergency medical attention where radiographs revealed a joint effusion, and a wavy appearance to the tendon []. There was no patellar elevation, fracture or malalignment. Clinically the patient was believed to have an ACL tear. The patient was treated with a knee brace and instructed to follow-up with an orthopedic surgeon and obtain an MR examination of the knee.\nThe MR examination of the right knee was obtained 10 days later and demonstrated abnormal increased T2 signal in the midsubstance of the ACL and abnormal orientation of the ligament consistent with a complete ACL disruption []. Bone contusions in the posterior medial and lateral tibial plateaus as well as the lateral femoral condyle were present. The patellar tendon was abnormal in morphology and signal. The tendon had an abnormal wavy contour at the junction of the middle and distal thirds of the tendon and had abnormally increased signal on T2-weighted images. The axial images demonstrated near complete disruption of the patellar tendon with only a few intact fibers.\nOrthopedic follow-up visit was delayed for nearly 2 months. At the time of presentation she had persistent pain, instability, and very limited range of motion. She had a positive Lachman test of the right knee. Although the patient had tenderness over the patellar tendon, there was no palpable tendon defect and she was able to hold a straight leg raise with minimal extensor lag. The patient was diagnosed with a complete ACL tear and high-grade partial patellar tendon tear. Physical therapy was instituted to help prevent postoperative fibrosis. A delayed ACL reconstruction was schedule 8 weeks later.\nA diagnostic arthrogram at the time of surgery confirmed a complete midsubstance ACL tear. The ACL was reconstructed with an ipsilateral hamstring autograft. The patellar tendon was not directly inspected at the time of surgery and was treated clinically as a partial patellar tendon tear. The patient's postoperative course was complicated by complex regional pain syndrome requiring multiple nerve block injections. A year following the initial injury, the patient had continued stiffness, difficulty with walking and had developed quadriceps muscle atrophy.
A 68-year-old male with past medical history of coronary artery disease status post coronary artery bypass graft (CABG), aortic valve replacement along with replacement of the root of the ascending aorta 10 months prior to presentation and recent hemorrhagic cerebrovascular accident (CVA), came to the hospital with complains of acute onset of severe abdominal pain and melena for 1 day. He also attested to chronic abdominal pain and a 30-pound weight loss over the last 8 months prior to these acute symptoms. His physical exam on presentation was positive for severe bilateral lower abdominal tenderness. Apart from a hemoglobin of 10 mg/dl and a positive stool occult blood test, the rest of his basic lab work up was unremarkable (white blood cell/platelet count, comprehensive metabolic panel, and PT/INR included). Hepatitis B, hepatitis C, and human immunodeficiency virus (HIV) testing were negative. The electrocardiogram (EKG) showed sinus rhythm and left ventricular hypertrophy (). An emergent computerized tomography (CT) scan of the abdomen revealed features suggestive of an embolic infarct in the left kidney (Figures and ) and within the mid-one-third of the superior mesenteric artery causing luminal narrowing and also suspected to be extending to the takeoff of small bowel branches. Segmental mural thickening of at least one small bowel loop was noted which strongly favored acute bowel ischemia as a cause of his abdominal pain (). Incidental findings on CT of the abdomen were also strongly suspicious for large eccentric thrombus in the ascending aortic graft and the aortic root which were further investigated and confirmed with a CT scan of the chest (). Cardiology and cardiothoracic surgery were consulted. A CT scan of the head was performed to assess the recent CVA and showed a subacute hemorrhage along the left-sided temporal parenchyma (). CT head imaging was obtained from the facility where the patient presented 3 months prior for cerebral hemorrhage and in comparison, to the most recent CT scan of the head, the hemorrhage appeared stable. The hemorrhage was suspected to be secondary to thromboembolism. After a review of the risks and benefits of anticoagulation to prevent extension of this suspected thrombus, heparin was initiated. An echocardiogram revealed dilatation of the ascending aorta and mild paravalvular leak around the bioprosthetic aortic valve. Gastroenterology was consulted and an emergent esophagogastroduodenoscopy was performed which was negative for any causes of upper gastrointestinal bleed. A hypercoagulable workup was performed which did not reveal any apparent cause of a prothrombotic state. Anticoagulation was held and subsequently, the patient underwent a redo sternotomy under cardiopulmonary bypass with extensive lysis of adhesions, removal of the thrombosed aortic valve and graft, ascending and proximal aortic arch replacement utilizing a 30 mm Dacron graft, and aortic valve replacement with a 25 mm Edwards Magna Ease bovine pericardial valve. The patient also underwent an explorative laparotomy as a part of a staged procedure to address the ischemic bowel caused by the presumed septic emboli. Intraoperatively, the patient was found to have a portion of small bowel that had become necrotic. The necrotic bowel was excised and an end to end anastomosis was performed.\nThe aortic graft and thrombus were sent for culture and pathology. Histopathological examination of the aortic graft and cusps of the aortic valve revealed chronic inflammation and was also notable for abundant acute angle branching septate fungal hyphae (). The patient was immediately started on amphotericin B and voriconazole pending finalization of cultures and sensitivities. On postoperative day four, three culture reports from the graft came back positive for dematiaceous mold, suggestive of Bipolaris species. The minimum inhibitory concentration (MIC) was 0.25 ug/dl for voriconazole and 0.03 ug/ml for amphotericin B. A decision was made to continue the same antifungal regimen on the basis of sensitivities and further speciation was not performed. Patient had a good postoperative course and was later discharged on amphotericin B and voriconazole for at least 1 month with continued follow-up with an infectious disease specialist.
A 55-year-old male presented with severe pain and numbness in the groin that radiated to the left anterior thigh. After the sudden onset of the pain, weakness on the left lower extremity developed and this had worsened for one week. He had a previous history of a thrombectomy in the left femoral artery two years before and in the left external iliac artery one month before. He had taken warfarin for two years after the first thrombectomy. On admission, his vital signs were stable. His prothrombin time international normalized ratio was 1.6, the hemoglobin was 9.7 g/dL and the platelet count was 223×103/µL. Physical examination revealed paresis of the left quadriceps muscle with strength of 4/5 and a diminished knee jerk reflex. The femoral, popliteal and dorsalis pedis pulses were palpable but slightly weak and no change in the skin color was found. He kept the left knee flexed and complained of aggravated pain when he extended the knee. Initially, vascular insufficiency due to partial arterial occlusion by thrombus was suspected. But computed tomography (CT) angiography and Doppler ultrasonography revealed patent proximal arteries and intact flows on the lower extremities. The source images for CT angiography revealed the iliacus muscle hematoma (). Because the left psoas muscle was compressed and displaced by the hematoma compared with right psoas muscle and the femoral nerve runs between the psoas tendon and the iliacus muscle, compression or displacement of the left femoral nerve was suspected. The warfarin was immediately discontinued and he was managed with pain control and regular neurological assessment. But, his pain and weakness were further increased over the next five days. He became unable to raise the left leg and to stand without assistance. Electromyography and nerve conduction study showed left femoral neuropathy. After confirming the normalization of his coagulation parameters, percutaneous catheter insertion was performed under ultrasonographic guidance for aspiration and drainage of the hematoma. However, only a small amount of the hematoma was drained and then surgical evacuation of the hematoma via the left retroperitoneal approach was performed. The hematoma was under significant pressure and the psoas muscle was compressed. Soon after making the incision on the iliacus fascia, old blood clot gushed out. The hematoma was gently evacuated with the suction and forceps and irrigated, and a Jackson Pratt drain was placed for continued drainage.\nThe postoperative course was uneventful and the paresis of the quadriceps muscle slowly improved. Abdominal CT scan showed a decreased amount of the hematoma one month after the surgery (). He demonstrated recovery of his femoral nerve function, with an improvement of the quadriceps strength to Grade 4/5 at the 1-year-follow-up examination, but limping in his left leg remained.
A two-week-old female was brought in with an initial complaint of multiple masses that failed to regress since birth. She was born from a nonconsanguineous union and the mother’s pregnancy was uneventful. The baby was delivered vaginally at 37 weeks of gestation and multiples masses were found over the body. The treating physician advised follow-ups on discharge. However, the parents decided to seek more medical help for the child and she was admitted for more investigations.\nOn physical exam, the child was alert and active. She was not jaundiced and no pallor was noted on the extremities. Her vitals were all within the normal range and the birth weight and changes in weight corresponded properly. Two masses measuring 26 mm by 19 mm and 19 mm by 17 mm were observed on the forehead and the scalp (Figures -). One smaller mass was seen on the abdomen, measuring 11 mm by 10 mm (Figure ). All three masses were red and non-hemorrhagic. The parents reported that the size of the masses did not change since birth. No similar family history was found and both parents were healthy. Any hepatosplenomegaly was not observed on palpation nor were any other cutaneous lesions detected. She did not present with any other systemic abnormalities. An ultrasound was performed, and it revealed a normal liver, spleen, and kidneys with no masses or lesions. A Doppler examination also showed a fast-flow vascular lesion, as reported by many other articles []. The full blood count showed normal levels of white blood cells, platelets, hematocrit, and red blood cells.\nThe diagnosis of congenital hemangioma was confirmed and the family was properly educated about the condition as well as possible complications. A wait and watch approach was adopted and the child was scheduled for regular visits at three months. The family was asked to properly monitor the size of the masses and informed that appropriate surgical treatment would be provided if the masses fail to regress or if they continue to grow.
A 59-year-old Asian male presented to the gastroenterology office for a follow-up of a previously found duodenal lesion. He had an EGD at an outside facility 2 years earlier for evaluation of abdominal pain which showed a 1.2 cm duodenal polypoid lesion in the second portion of the duodenum. Pathology of the polyp revealed heterotopic gastric mucosa with benign lobules of gastric glands. He also was found to have chronically active H. pylori gastritis with numerous Helicobacter organisms and atrophic gastritis with patchy areas of intestinal metaplasia. At that time, he was instructed to follow up for a repeat endoscopy for surveillance in two years. As recommended, two years later he presented to our office for surveillance of his duodenal lesion. Due to his history of atrophic gastritis and a large duodenal lesion, we performed an upper endoscopy that showed atrophic gastritis, duodenitis, and a single 10 mm polyp in the second portion of the duodenum. The duodenal polyp was biopsied. Pathology revealed polypoid gastric metaplastic mucosa with focal epithelial atypia including nuclear enlargement, stratification, nucleoli, and few mitoses. The patient was referred for endoscopic ultrasound to further characterize the lesion and complete resection. Several weeks later he underwent an endoscopic ultrasound that revealed a 13-mm pedunculated and sessile polyp on the lateral wall opposite to the major papilla in the second portion of the duodenum (). The polyp was removed en bloc using a hot snare following a 4-mL saline lift. Two endoclips were placed for hemostasis. Pathology revealed a pyloric gland adenoma (Figures , , and ) with predominantly low grade dysplasia (Figures and ) and some small areas of focal high grade dysplasia. The slides were read by the in-house pathologist and were also reviewed by a GI expert pathologist at the University of Michigan. The patient was recommended to follow up for surveillance upper endoscopy in 6 months due to the areas of high grade dysplasia found on pathology. He was lost to follow-up for over one year. Fifteen months after the PGA was removed, the patient did follow-up for a surveillance upper endoscopy. A duodenal scar was found at the site of the PGA and a biopsy was taken from the site. There was no evidence of residual pyloric gland adenoma. The patient was then recommended to follow up for surveillance endoscopy in 5 years in accordance with the ASGE guidelines for surveillance of gastric adenomatous polyps.
A 14-year-old girl was referred by the local dentist to the Department of Pedodontic and Preventive Dentistry, Seema Dental College and Hospital after a failed attempt to extract the mandibular right premolar. On intra oral examination nothing was visible but on extra orally swelling was seen on swelling was due to the periapical lesion wrt 46 that was confirmed on radiographic and clinical examination (). On Intraoral periapical (IOPA) radiograph of the respected site, a noninfected broken root was diagnosed in relation to right lower second premolar ().\nTo maintain the functional matrix in the arch during growth period, allotransplant was planned as treatment after the extraction of root. Patients who had reported for the extraction of the mandibular right premolar for orthodontic reasons were recalled to the department. This donor was investigated for hepatitis B, C and HIV which turned out to be negative. Informed consents were obtained from both the donors and the recipient. Under local anesthesia the teeth of donor were carefully extracted with minimal trauma after completing an endodontic treatment RCT ().\nThe tooth was placed immediately in a chilled solution of 2% chlorhexidine (). Holding the tooth by the crown, the root surface was thoroughly cleaned off all the blood with the same solution using syringe. The apical end of tooth was closed by the biodentine. Throughout the procedure, care was taken to ensure that the root surfaces of this teeth were left undisturbed. This was to ensure minimal damage to the periodontal fibers. The teeth were then transplanted to the recipient's after the extraction of root stem extraction of recipient and this procedure is done within 10 minutes of extraction and was splinted with splint (). Before transplantation the socket was irrigated with saline to clean off debris following which fresh bleeding was induced by forceful irrigation. Intra oral periapical radiograph was taken to confirm the position of transplanted teeth in the socket before splinting ().\nThe wound healed uneventfully after 4 weeks () and prosthesis porcelain crown was given on the tooth. Subsequently, periodic follow-up showed that the allograft is clinically firm and radiographically no inflammation or replacement resorption (). This case is being continuously monitored to detect any future resorptive activity.
The patient, a 32-year-old Caucasian woman, presented to the West Virginia University Hospital Emergency Department via Emergency Medical Services. The patient had been at her usual baseline state of health with no significant past medical history prior to visiting the chiropractor for neck adjustment earlier that day for tension like soreness. The patient underwent neck manipulation after which she immediately complained of neck pain, diaphoresis, and proceeded to experience cardiac and respiratory arrest. Emergency Medical Services was called, and cardiopulmonary resuscitation was performed with one round of epinephrine administered. It was reported that the patient was pulseless and apneic for 3 minutes prior to EMS arrival. The patient was intubated on transport and her Glasgow Coma Scale score was 3T prior to arrival. Mean arterial blood pressure was 80 with palpable femoral pulses at arrival to the emergency department. Upon arrival in the emergency department, a CT stroke protocol was performed which demonstrated bilateral severe distal cervical vertebral artery dissections with acute thrombotic emboli seen in the left cervical vertebral artery ( and ). This was accompanied by complete occlusion of the basilar tip including the proximal posterior cervical arteries.\nThe patient received an initial bolus of intravenous tissue plasminogen activator (IV rtPA) at this time and the decision was made to proceed with endovascular intervention given the recent onset of occlusion. The patient was brought to the neurovascular angiography suite and femoral access obtained. Angiography of the left vertebral artery demonstrated severe dissection involving the distal cervical vertebral artery segments at the C1-C2 level with presence of sub occlusive thrombi. There was an occlusive clot in the left Posterior Inferior Cerebellar Artery (PICA). Intracranial imaging demonstrated occlusion at the basilar apex with absent filling into the right Posterior Cerebral Artery (PCA). There was occlusion of the distal left PCA. Angiography of the right vertebral artery demonstrated severe dissection of the distal cervical vertebral artery at C1-C2 with the presence of trickle-like flow into the vertebrobasilar junction. No filling was observed in the right PICA territory (). At this point, it was decided that the left vertebral artery offered the best access to the basilar trunk.\nSubsequently, distal aspiration was begun with a Penumbra 5 Max ACE distal aspiration catheter which initially demonstrated slow flow through the suction tubing. The 5 Max ACE was withdrawn into the proximal basilar artery until flow was seen within the suction tubing. Repeat angiography at this time demonstrated recanalization of the basilar apex and proximal PCAs. TICI3 perfusion was seen in the right PCA. Occlusive clot remained in the left distal P2 segment. Given the large size of the PCA, timing of events, and patient’s age, the decision was made to attempt clot retrieval of this. At this time, the Trevo ProVue microcatheter was navigated into the left distal PCA distal to the clot. The Trevo 4 mm × 30 mm stent retriever was deployed for approximately 3 minutes. The suction canister was attached, and the stent retriever was pulled with distal aspiration. No significant recanalization was achieved with what amounted to TICI0 perfusion to the left PCA territory. No further attempts were made as it was believed that the left PCA territory had completed its infarction.\nFollowing completion of endovascular therapy, the patient was taken for immediate MRI Brain with and without contrast for assessment of brainstem integrity and cerebrovascular status prior to transport to the intensive care unit. MRI demonstrated extensive areas of restricted diffusion accompanied by perfusion abnormalities consistent with acute infarction of the posterior circulation, specifically within the bilateral cerebellar hemispheres, right medulla, pons bilaterally, midbrain, thalami, and left occipital lobe (). The following day, additional CT Brain imaging was acquired and demonstrated signs of elevation of intracranial pressure with hydrocephalus, worsening of cerebral edema diffusely, hemorrhagic transformation of the left occipital lobe, continued infarct evolution within the posterior circulation, and cerebellar tonsillar herniation.
A 20-year-old Japanese woman presented to a local hospital with lower abdominal pain. She had mental retardation and epilepsy, and was unable to indicate her intentions. She was found to have a multicystic tumor in her pelvic cavity and was referred to our gynecologic department. Her lower abdomen was soft and showed slight swelling without tenderness. Magnetic resonance imaging revealed that a multicystic tumor occupied her abdominal cavity. Because the tumor did not have a solid component or enhancement with gadolinium enhancement and tumor markers were normal (carbohydrate antigens [CA] 125, CA19-9 and carcinoembryonic antigen), a benign ovarian tumor was suspected. A laparotomy was performed, unexpectedly revealing swelling of her bilateral ovaries. The right ovarian tumor was the size of an adult head and the left ovarian tumor was the size of a fist. A right oophorectomy and left ovarian cystectomy were performed. Gross findings were that the bilateral ovarian tumors seemed benign without a solid or papillary component. The pathological diagnosis of the right ovarian tumor was mucinous cystadenoma; however, the pathological diagnosis of the left ovarian tumor was an intestinal type mucinous borderline tumor. Our patient did not receive any adjuvant therapy and we planned to follow up every three months.\nTwo years and nine months after the primary surgery, a multilocular tumor, approximately 10 cm in diameter, was detected in her pelvic cavity on follow-up ultrasonography. A computed tomography scan revealed a multicystic tumor in her pelvic cavity, without contrast by a radiopaque agent (Figure ). Magnetic resonance imaging revealed round cystic lesions in her pelvis, with low to intermediate intensity on T1-weighted images and high intensity on T2-weighted images (Figure ). A small part of the cyst wall was slightly enhanced with the administration of gadolinium. The preserved left ovary was not separately identified. Therefore, a recurrent ovarian borderline tumor was suspected.\nOur patient could not give her own opinion regarding her disease so her parents made all decisions regarding her treatment. Our patient had suffered from dysmenorrhea for a long time and her parents therefore requested removal of her uterus and remaining ovary.\nOur patient then underwent a second surgery and a multicystic tumor measuring 10 × 10 cm was found in her pelvic cavity. The tumor consisted of many non-isolated cysts of diverse sizes (Figure ), which adhered to the surface of her ovary, mesentery, mesocolon and pelvic wall. Slight transparent ascites was present. Her uterus was normal and her left ovary was thumb-sized without swelling. A hysterectomy, left oophorectomy, omentectomy, pelvic lymph node biopsy, appendectomy and tumorectomy of her abdomen were performed. Complete resection of the lesion was achieved.\nOn histology, epithelium with a mucinous component was not seen in the cyst or cyst wall (Figure ). In the removed left ovary, a tiny component of mucinous cystadenoma was present though neither a mucinous borderline tumor nor a mucinous adenocarcinoma was present. Immunohistochemical staining of the lining cells of the cyst wall were positive for calretinin and Wilms’ tumor protein and negative for D2-40. The removed uterus, lymph nodes and omentum were normal. Our patient was finally diagnosed with BMPM. She recovered uneventfully and was discharged from the hospital on the seventh day after the operation. Our patient received estrogen replacement therapy and remains free from recurrence nine months after the second operation.
A 43-year old, asymptomatic woman was admitted to our hospital by her family doctor after receiving a chest-x-ray during routine clinical examination. The x-ray showed a mediastinal mass overlapping the aortic arch region (Fig. ). For verification a computed tomography (CT) was performed and revealed incidentally a type B dissection, which was most likely chronic without information of the index date, originating from an aneurysm of a left cervical arch with a maximum diameter of 6 cm (Fig. ). The left renal artery, the coeliac trunc and the main part of the superior mesenteric artery branched from the false lumen without a sign of malperfusion of the organs. Because of the huge diameter and the potential risk of rupture, an urgent surgical repair was planned. Before intervention the patient got a blood pressure adjustment by ACE inhibitor. Betablocker was not necessary because of a resting pulse under 60 beats per minute. For neurological online monitoring, sensitive and motor evoked potentials were monitored. Spinal drainage was installed 1 day before the procedure. Surgical access was carried out through median sternotomy and an additional left lateral thoracic incision through the fourth intercostal space (Hemi-Clemshell). Simultaneously to the preparation of the aneurysm, partial cardiopulmonary bypass was installed in the left groin by cannulation of the femoral artery and vein under echocardiographic guidance. During selective ventilation of the right side, the left lung was mobilized by transsection of the Ligamentum pulmonale and preparation of the perianeurysmatic tissue and adhesions. After identification and preparation of the recurrent and phrenic nerve and the supraaortal branches, the descending aorta was clamped and a distal anastomosis performed with a straight graft (20 mm). The visceral arteries partially branched from the false and true lumen without a sign of malperfusion. Before the final distal anastomosis, we performed a fenestration of the dissection membrane about a length of 5 cm to keep the perfusion of both lumina. The left carotid artery originated from the aortic arch with a distance of only 1 cm from the aneurysm. The left axillary artery branched directly from the aneurysm and was dissected and reimplanted with a separate 8 mm sidegraft to the 20 mm straight graft between the distal arch and proximal descending aorta. (Fig. ). The procedure was performed with partial cardiopulmonary bypass (CPB) of 87 min, aortic clamp time of 62 min under normothermic condition. The patient was extubated on first postoperative day and recovered well.\nBiopsy of aortic tissue showed a picture consistent with arteriosclerosis and loss of smooth muscle cells, rupture of the elastic fibres and fibrosis of the media. The intima could not be visualized in detail.\nThe patient was discharged to cardiac rehabilitation at 13th postoperative day and recovered well. Last follow up with computed tomography was performed 3, 5 years after initial operation with a good and stable result of the dissection membrane and a perfusion of both lumina. The patient is able to resume a normal life without limitations.
Our patient was a 32-year-old previously healthy female at the 39th week of gestation who accessed the first aim department of a primary healthcare centre of a peripheral hospital for severe dyspnoea and chest pain. Her past medical history did not present other hospitalizations for the same symptoms. Due to the clinical manifestations, the patient was initially treated as a case of pulmonary embolic disease and subjected to a massive anticoagulant therapy. Considering the clinical diagnosis and the child to term, an emergent caesarean delivery was performed in order to avoid foetal complications. The caesarean section was successfully performed under general anaesthesia using Stark's method due to the urgency related to the patient's clinical condition of increasing dyspnoea. Moreover, although the pAVM was still unknown at time of the caesarean section, the execution of spinal anaesthesia seems to be not indicated because of the risk of pAVM association with other AVMs, such as those located in the spinal cord, especially in case of HHT.\nThe foetal outcome showed an Apgar index of 3, 6, and 9, respectively, at minutes 1, 3, and 5; these data are in line with the administration of general anaesthesia and the acute maternal condition of severe dyspnoea.\nTaking into account the foetal weight at birth, it showed a restriction of the expected value. The child weight was in fact 2590 gr at 39 weeks of gestation. However, ultrasounds performed during the pregnancy reported a reduction of the potential foetal growth from the 33 weeks of gestation without any Doppler alteration. This phenomenon should be the result of the chronical adaptation of the pregnancy to the unknown pAVM.\nAs far as the macroscopic exam of the placenta is concerned, a percentage of cotyledons infarcts inferior than 10% was reported.\nConsidering the patient's postoperative course, it showed a subsequently worsening of the clinical conditions, resulting in an acute distress syndrome that required an immediate transfer to the Gynaecology and Obstetrics unit of our structure. Due to the critical care panel and the low clinical conditions, the patient was intubated and housed in the ICU department. Considering the acute distress syndrome, a chest CAT scan was performed highlighting the presence of a left pAVM expanded, associated with a massive hemothorax that compressed the correspondent lung. The vital signs panel showed systolic blood pressure of 70 mmHg, diastolic blood pressure of 35 mmHg, pulse rate of 150/min, pulse oximetry saturation 88% on 100% inspired oxygen, afebrile temperature, and respiratory rate of 40/min. Initial labs revealed normal platelets, normal coagulation panel, and haemoglobin of 7 gm/dL. Critical care panel showed pH of 7.4, pCO2 of 43 mm hg, pAO2 60 mmHg, and saturation of 88%. After the placement of a chest tube, 3 litres of frank blood were removed; this action resulted in a normalization of the blood pressure and improved oxygenation on the monitor. The successive management was the clinical observation of the patient's conditions as well as the vital signs and labs test in order to perform the pAVM embolization when the patient clinical conditions will be stable. After three hours from the drainage, worsening of the patient conditions was observed reporting a severe collapse of the vital signs as well as a decrease of antithrombin III, fibrinogen, and haemoglobin values, with parameters of 33%, 122 mg/dl, and 5.8 gm/dl, respectively. Moreover, considering the postoperative caesarean course, the gynaecologic clinical evaluation showed a low uterine fundus contraction and the presence of conspicuous abnormal lochia. Uterine fundal massage was performed as first approach to solve the uterine low contraction followed by Credè's manoeuvre. Due to the failure of both, a pharmacological treatment was attempted starting with a simultaneous administration of intravenous Oxytocin (10-40 UI per 1 litre saline solution) and intramuscular Methylergometrine (0.2 mg one dose). The latter pharmacological approach involves the use of intravenous Sulprostone (0.5 mg per 1 litre saline solution) that was administered within half an hour from the signs of low uterine contraction and abnormal lochia. None of the previous pharmacological treatments succeeded.\nConsidering the reproductive age of the patients, procedures as appositions of tamponade-balloon and embolization of the uterine arteries were taken into account but were not applicable in order of the unstable and precipitant parameters of the woman. Due to the patient's life-threating condition, a simultaneous surgical intervention of thoracic surgeons and gynaecologists had been necessary to solve the urgency, with the performance of a contemporaneous surgical reparation of the pAVM and resection of damaged left lower lobe (LLL) as well as a preventive hysterectomy to avoid the risk of disseminated intravascular coagulation (DIC). The surgical interventions were performed successfully but intraoperative blood transfusions and administration of antithrombin III and fibrinogen were necessary. The postoperative treatment showed a normalization of vital signs and labs panel as well as patient's clinical conditions. Due to the stable condition of the woman, the anaesthetist established the patient's autonomous breathing. After one week from the intervention, chest CT with intravenous contrast was performed showing a 4 cm area of active contrast. Pulmonary angiography confirmed the presence of a pAVM with feeding branch of a basilar left pulmonary artery supplying aneurysmal AVM and dilated draining vein. Transcatheter embolotherapy (TCE) of the culprit vessel was performed by placement of a nonadhesive liquid embolic agent (Onyx 34®). Repeated chest X-ray and chest CT after one week from TCE showed expansion of remaining left lung and signs of pAVM embolization and pulmonary resection of LLL, respectively (). The patient course was subsequently uncomplicated and the discharging home happened after 14 days. MRI evaluation was performed in order to detect any head AVM but the result was negative. The genetic testing for HHT was not performed during this hospitalization period but the genetic examination performed a few months afterwards showed no association.
A 31-year-old gravida 3 para 1 patient presented to antenatal clinic at 19 weeks and 3 days' gestation to discuss the results of her morphology scan which had demonstrated a grade 4 placenta previa covering the cervical os. She had a medical history significant for Arnold Chiari malformation requiring craniotomy in 2006 as well as correction of a Syringomyelia in 2005. She also suffers from irritable bowel syndrome but was taking no regular medications and had a BMI of 23. Her first pregnancy resulted in a spontaneous miscarriage that did not require dilatation and curettage. Her second pregnancy resulted in a planned elective caesarean due to concerns about raised intracranial pressure during labour, as recommended by her neurologist. She had routine antenatal care this pregnancy which had been unremarkable to date.\nThe morphology scan demonstrated a small omphalocele but otherwise no significant structural defects and estimated foetal weight was noted to be within the normal range. During the clinic review, the fetus was found to have a heart rate well below 100 bpm. Repeat ultrasound the following day confirmed IUFD. This ultrasound also demonstrated evidence of an abnormally invasive placenta with the appearance of dysplastic vascular hypertrophy. An obstetric MRI was performed which supported the diagnosis of morbidly adherent placenta. This showed a low lying inhomogeneous placenta, dysplastic vascular hypertrophy, ill-defined placental bands, and an overall impression of some areas of increta with no overt evidence of percreta.\nOptions were discussed with the patient who decided for conservative management in order to optimise her chance of preserving her fertility. This was balanced against potential complications of prolonged conservative management of an IUFD, including sepsis and coagulopathy. A plan was made for serial ultrasounds as an outpatient, to be followed by induction of labour when placental blood flow was no longer detectable. Twenty-seven days following IUFD confirmation, the patient was admitted to hospital with abdominal cramping and associated small antepartum haemorrhage (APH).\nUltrasound scan at 31 days showed a minimal reduction in blood flow through the anterior placenta and to the cervix. At day 33 she suffered a further 300mL APH. Given her increasing blood loss and minimal changes to placental blood flow on ultrasound, she was administered a dose of 80mg methotrexate intramuscularly with the hope of accelerating devitalisation of the placenta. A repeat dose of methotrexate was planned for five days' time. During the subsequent two days after the administration of methotrexate, the patient continued to suffer moderate bleeds and increasingly significant contractions. 35 days following IUFD she spontaneously delivered a male fetus with only minimal bleeding during delivery.\nA brisk 2 L postpartum haemorrhage (PPH) followed delivery and the patient was immediately taken to the operating theatre for examination under anaesthesia and attempted manual removal. A urinary indwelling catheter was inserted and remained in situ for the entirety of the operation. Due to only partial removal (approximately 80%) of the placenta being achieved manually, the case quickly progressed to laparotomy. Intraoperative findings revealed a full thickness increta at the previous caesarean incision just above the level of the bladder. Hysterotomy was performed with a transverse incision made above the prior caesarean incision, and the remaining placenta was removed manually, creating a 3x3cm plug-like defect anteriorly. This defect was closed with a primary closure separate to the hysterotomy incision. In addition, the placental bed was oversewn to establish haemostasis. A Foley's catheter was inserted vaginally and inflated with 60mL normal saline. Total blood loss was 4 litres (L): 2L immediately postpartum, 1L while attempting per vaginal manual removal of the placenta, and 1L intraoperatively. Massive transfusion protocol was activated with the patient receiving 10 units of packed red cells, 6 units of fresh frozen plasma and 5 units of cryoprecipitate. The patient remained stable throughout the process. A further 3 units of packed cells was given over the next two days for persistent anaemia. The fetus was found to weigh 170g. No cause for foetal demise was identified and the family decided against an autopsy. Pathological examination of the placenta was performed. This was noted to be difficult due to extensive haemorrhage and areas of necrosis commensurate with intrauterine foetal death and prolonged intrauterine retention. There was no evidence of funisitis or umbilical cord vasculitis to support a diagnosis of chorioamnionitis nor were any pathogens observed. The degree of decidual haemorrhage and necrosis made a histological diagnosis of placenta accreta impossible.\nThe patient recovered without significant complication over the following days and was discharged 1 week later on oral antibiotics and aperients. Six weeks after discharge the patient was seen in a postnatal follow-up clinic. She experienced minimal lochia in the postpartum period and was feeling generally well.
A 41-year-old non-smoker obese female patient was examined at the Department of Neurology and the Center for rehabilitation. Relevant history of the patient is summarized on a timeline in Fig. . She showed an onset of neuromuscular disorders during early childhood with a delay in motor and written language development. She had a hard time to complete physical exercise at school and always finished last. There was nothing really alarming in terms of muscle disorder until her first pregnancy at the age of 33 during which she experienced shortness of breath (dyspnea) at the 7 months of gestation and showed signs of weakness of lower limbs afterwards. At the age of 34 she showed worsening of breath symptoms, suffered from sleep apnea and started using a mechanical ventilation machine. Alarming symptoms of skeletal muscle disorders occurred immediately after delivery with progressive but rapidly incapacitating weakness of lower limbs. This worsened during the 3 following years, a period during which she first started being unable to get up by herself and then was unable to climb stairs. At the age of 37, she started to use a cane, then a walker for her daily walking needs. At the age of 39, she started using a wheelchair to move outdoors. Despite these signs of progressive muscle weakness, she never had a thorough neuromuscular investigation. It was wrongly thought that her health problems, in general, was mainly related to being overweight. At the age of 41, she had four episodes of lower limb paralysis during which she was completely unable to move her legs and support her weight. She did not seek medical consultation for the first three episodes. For the fourth, she was admitted to the emergency room (ER) and first referred to the Department of Neurology and then to the Center for Rehabilitation.\nThe family history showed that her mother died at the age of 66 from a heart attack associated with non-compaction cardiomyopathy. There is nothing remarkable in terms of muscle disorders in her father, brother, and sister. However, her only daughter, now at the age of 11, shows signs of muscle disorders with congenital muscular torticollis, excessive growing pains as well as underdeveloped muscles in half of the body.\nPhysical examinations conducted following her admission to the ER at the age of 41 showed normal tone/bulk of the arm muscles. However, muscles in shoulders and upper and lower limbs showed bilateral weakness. Deltoids, biceps and triceps showed moderate weakness with MRC scale of 3/5. Fine finger movements were intact. There was no pronator drift. In the lower limbs, all muscles examined showed the same severity of weakness (2/5) except quadriceps which showed mild weakness (4/5). Hip flexors were extremely weak (1/5); hip abductors and adductors were mildly weak (4/5). Knee extensors and flexors were moderately weak (3/5). Dorsiflexion of feet was severely weak (2/5). Plantar flexors were severely weak (2/5). Deep tendon reflexes were 2+ in the arms, absent in the patella and Achilles. The toes were down-going. No sensory deficit was observed. No sign of dysphagia or involvement of ocular muscles was observed.\nAdditional physical examination conducted during the 2-year follow-up (at the age of 43) showed no worsening of muscle strength. However, this brought new information about the weakness of other muscles, notably in the shoulders, with extremely weak abductors and flexors (1/5). The patient showed decreased perception of vibration in the lower limbs. She reported that she experienced occasional dysphagia.\nElectrophysiological exams showed normal nerve conduction velocities for upper and lower limbs, with all SNAP and CMAP amplitudes in the range of normal values. However, needle EMG revealed a tendency of myotonic potentials, generated by needle insertion. Right deltoids, biceps and ulnar-innerved first dorsal interosseus muscles showed myotonic potentials as well. The right tibialis anterior showed 2+ polyphasic motor unit potentials with myotonic potentials. The right medial gastrocnemius, vastus medialis and vastus lateralis showed myotonic tendencies as well.\nExamination of muscle biopsy of left quadriceps showed features of an end-stage process, consistent with a severe, chronic myopathy. There are scattered clusters of viable muscle fibers which showed myopathic features in the form of a marked variation in fiber size and numerous internal nuclei. In addition, there are scattered fibers with abundant intrasarcoplasmic vacuoles (Fig. a, b). Given we could not clearly observe the presence of hyaline bodies on H&E staining we decided to proceed with p62 immunostaining, a well-known technique for revealing the presence of inclusion bodies []. Immuno-histochemical detection of p62 was performed on a Leica Bond III automated stainer. Following digestion in a low pH citrate solution, sections were incubated in a primary mouse monoclonal anti-p62 antibody, diluted 1:50 (BD Transduction Laboratories Catalog Number 610833). Detection of bound antibody was achieved using the Leica Bond Polymer Refine Detection kit, comprising the secondary antibody, the substrate chromogen DAB (3,3′-Diaminobenzidine tetrahydrochloride hydrate) and the Hematoxylin counterstaining solutions. Immunostaining for p62 revealed, in a proportion of the surviving muscle fibers, diffusely distributed, small intermyofibrillar dots or, more commonly, larger central or eccentric sarcoplasmic inclusion bodies (Fig. c, d). Examination of heart function using Holter ECG monitor carried out following her admission to the ER showed no clinically significant implication of cardiac involvement. However, the patient complained about repeated episodes of oppressive chest pain during the following years. Additional examination conducted during the 2-year follow-up showed signs of cardiac involvement with bradycardia of 58 beats per minute.\nRegarding respiratory involvement, the patient suffered from dyspnea since the age of 34. Examination of lung function conducted following her admission to ER and during the follow-ups showed worsening of her respiratory condition. Spirometry test conducted at the age of 44 showed a very weak pulmonary function, with forced vital capacity (FVC) of 27% and forced expiratory volume in a second (FEV1) of 29% of normal values.\nThe blood samples were collected for whole-exome sequencing (WES) to detect mutations potentially involved in the phenotype of neuromuscular disorders observed for this patient. The genomic DNA was extracted from whole blood and subsequently subjected to whole-exome DNA library construction using the Ion AmpliSeqTM Exome RDY panel (Thermo Fisher Scientific) essentially as described in the manufacturer’s protocol, with barcode incorporation. For the sequencing, samples were loaded on an Ion HI-Q PI Chip v3 and placed onto the Ion Proton instrument (Thermo Fisher Scientific) together with an Ion PI HI-Q sequencing 200 Kit (Thermo Fisher Scientific) and sequenced for 520 cycles according to the manual (See Additional file : Table S1 for parameters). All candidate mutations found by WES were validated by direct Sanger sequencing (See Additional file : Figure S1 for the filtering process). DNA sequences were obtained from the University of California Santa Cruz (UCSC) Genome Browser. Predesigned primers were directly purchased from Thermo Fisher Scientific (See Additional file : Tables S2 for details). Amplicons were sent to Genewiz () for Sanger sequencing.\nWhole exome sequencing showed that a novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg) in the MYH7 gene is a missense single nucleotide variant possibly linked to the clinical findings, found in the DNA of the patient as heterozygous (Fig. ). The novel variant has been submitted to ClinVar database; with the assigned accession number SCV000804311. Two other candidate mutations were identified in the DNA of this patient, namely NM_003085.4:c.368C > A (p.Pro123His) in the SNCB gene and NM_001001557.3:c.746C > A (p.Ala249Glu) in the GDF6 gene (Additional file : Table S3). However, both mutations were discarded after filtering only genes involved in neuromuscular functions that potentially cause clinical features of muscle myopathy observed in this patient (Additional file : Figure S1). Regarding the novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg), bioinformatics analyses showed that nucleotide T coding at the position 1370 of the MYH7 gene is highly conserved across 44 vertebrate species (PhyloP at 1.76). Amino acid substitution from Isoleucine (I) to Arginine (R) at the position 457 of MyHCI suggested a high impact on protein structure (Grantham at 97). Results of analyses obtained from VarSome () suggested a classification of Likely pathogenic for this variant, with evidence of Pathogenic computational results coming from 8 various prediction software including DANN, GERP, dbNSFP.FATHMM, MetaLR, MetaSVM, MutationAssessor, MutationTaster and PROVEAN (vs no benign predictions). Also, the results of analyses using the recommendation of the ACMG and the AMP suggested the same classification of Likely pathogenic for this variant, with combined criteria of 2 moderate (PM1 and PM2) and 2 supporting (PP2 and PP3) [].
A 33-year-old woman was admitted to psychiatry inpatient with a complaint of suicidal ideation. The patient has a past history of multiple psychiatric disorders like BPD, MDD, and anxiety issues for about last eight to 10 years. She had multiple suicide attempts in the past most recent being two weeks back when she tried to suffocate herself with the help of a medical device tubing. On inquiry, she said she just wanted to feel the pain, not kill herself. On further questioning, she was found to have passive suicidal thoughts as well as an active plan to harm herself. Her plan was to kill herself with the carbon monoxide poisoning by turning on the engines of four cars parked in the garage. According to her, she felt better at the time of the last admission and these suicidal thoughts just returned two to three days back. She had multiple admissions and emergency department (ED) visits related to her psychiatric conditions as well as five suicidal attempts. During one of her admission when she took multiple tablets of Advil® (Pfizer, New York, USA) in an attempt to kill herself, she was evaluated for ECT by a psychiatrist but the decision was made in favor of dialectical behavior therapy (DBT) as they felt these symptoms are because of her BPD. According to the patient she has been compliant with the therapy that has helped her in coping day-to-day issues. The patient also confirmed that she has never recovered from these active and passive suicidal thoughts which have progressed to even worse state in the last four months.\nOn further evaluation, the patient reported feelings of hopelessness and worthlessness most of the time along with a guilt of things for what she has done in the past. She also reported a decrease in sleep to about five hours per night along with difficulty in staying asleep and poor appetite and energy. Her concentration was normal. She continues to engage in her interests in reading and photography. The patient states she has been a "warrior" for years. She endorses a few prior panic attacks where she felt shaky, short of breath, and had chest pain. She could not recall how long they lasted or when her last episode was. Screening for mania, psychosis, and obsessive compulsive disorder was unremarkable. There was no reported history of alcohol, tobacco, or illicit drugs.\nMinnesota multiphasic personality inventory-2 (MMPI-2) results suggested the presence of depression, anxiety, overall distress, and a personality disorder. All of these scores are in the moderate to severe ranges and are rather similar to one another. The psychologist during this visit interpreted that these results do not suggest that the personality disorder is the main factor driving her clinical presentation and that her presenting symptoms are due to MDD. She was recommended ECT as it was determined that these symptoms are due to resistant MDD.\nThe patient had a past history of multiple psychiatric drug trials in the last eight years which included medicines like fluoxetine, sertraline, venlafaxine, amitriptyline and even augmented therapy with antipsychotics was tried with aripiprazole and thyroxine which all proved ineffective in this patient. Considering her condition and beneficial outcomes in such a treatment-resistant patient, a trial of ECT was the consensual decision of all the panelist psychiatrists. The patient agreed to this mode of therapy.\nThe first session was done with the parameters mentioned in Table .\nAfter the first session parameters were changed for the rest of the 12 sessions which are mentioned in Table .\nA total of 16 sessions were conducted with a break after 13 sessions. The frequency of sessions was three per week for the first 10 sessions and then two sessions every week and last three sessions were conducted once a week. The patient was evaluated after every session and there was a remarkable improvement from the sixth session onwards. After 13 sessions there was a thorough evaluation and the patient reported improved mood and no active or passive suicidal ideations and she was discharged. She remained symptom-free for four to five months but then reported again with another suicide attempt. She was restarted on ECT, and 16 more sessions were conducted with the same frequency and same parameters.\nOn her recent visit, she endorsed a significant improvement in her depressive symptoms and denied active suicidal ideations. She also reported an improved quality of life.
This patient is a 41 year old female that underwent a composite mandibulectomy with resection of the oral floor for squamous cell carcinoma. She was immediately reconstructed with free fibula osteocutaneous flap for her mandibular and oral floor defect. The free fibula flap was harvested from her right leg preserving 6 cm of fibula proximally and distally for knee and ankle stability. A large, 14 × 18 cm skin paddle was harvested along with the fibula flap to reconstruct the floor of her mouth, the defect of which was extensive. The entire skin paddle survived and she went on to achieve bony union of both sides of the fibula graft and the single osteotomy. Fixation of the fibula to the mandible was performed using a Stryker Locking Reconstruction Plate from the right mandibular ramus to the left angle of the mandible. The vascular microanastomosis between the Peroneal artery and vein of the fibular flap was achieved in an end-to-side manner with the External Carotid Artery and Internal Jugular Vein, respectively. Her initial postoperative course was uneventful. She eventually began eating well, speaking and breathing without difficulty and her feeding tube and tracheostomy were removed. She did however require postoperative radiotherapy to the mandible, oral floor, and neck.\nTwelve months postoperatively, she developed thinning of the chin skin and exposure of the anterior portion of the mandibular plate. CT scan confirmed bony union of the free fibula to the mandible bilaterally. Her skin was very thin throughout the neck and, interestingly, the skin had epithelialized under the exposed reconstruction plate. At this time it was decided to approach her plate directly and in a manner that would safely not disrupt the bone graft and epithelialized skin below the reconstruction plate. A small extension of the skin opening was performed in the lateral direction on both sides. The exposed screws were removed transcutaneously. The titanium reconstruction plate was then removed with a single cut on both sides, immediately proximal to the point of initial exposure using a Large Surgical Pin Cutter. The plate was then removed and the skin was closed in simple fashion with 5-0 nylon sutures that were removed 2 weeks later. The wound went on to heal well.
A 47-year-old woman suffering from recurrent shortness of breath over a period of 2 months was admitted to our hospital. Initially, she was diagnosed with coronary heart disease (CHD) at a local hospital and received standard medical therapy for CHD (including aspirin, atorvastatin, ACE inhibitor and vasodilators). However, subsequent coronary angiography showed no significant stenosis of the coronary arteries. Because coronary spasm and microvascular lesions could not be ruled out, doctors continued to give her routine treatment for CHD. The patient’s symptoms persisted after treatment at the local hospital, and she was subsequently referred to our hospital for treatment. TTE showed a left ventricle diastolic diameter of 64 mm and diffuse hypokinesis, with an LVEF of 34%. It is noteworthy that an apical ventricular aneurysm with a wall thickness of 3 mm had formed. Multiple, developed muscle bundles were found in the left ventricle, which also divided the left ventricle into two distinct chambers. Blood entered the apical aneurysm in diastole and was ejected out of it in systole, as it occurs in the normal LV. The diameter of the communication measured 25 mm in diastole. The flow rate from the main chamber to the apical aneurysm was 150 cm/s. The patient also had no family history of heart disease. Subsequent CMR suggested that the myocardial three-layer structure in the left ventricular apex disappeared with ventricular aneurysm formation (Fig. ), which was strikingly similar to case 1. We conducted investigations to find the common causes of ventricular aneurysm, but there were no special findings other than moderate anemia (Hemoglobin: 8 g/dL, normal range: 11–15 g/dL) due to thalassemia. The reason for the more obvious decrease in cardiac function in this patient may partially be related to anemia, which would increase cardiac output and is one of the causes of cardiomyopathy []. Although there was no previous echocardiogram showing the presence of the intraventricular muscle bundles, we assumed that case 2 shared a similar underlying mechanism with case 1 because her imaging findings were almost identical to his. The only differences from case 1 were the symptoms and treatment strategy for this patient. Since this patient was younger and had more severe symptoms, we suggested an open surgery to dissect the intraventricular bundle and aneurysm but the patient declined. The symptoms of heart failure have improved significantly with treatment during the 3 months of follow up.
A 54 year old female patient reported to our department with the chief complaint of pain and swelling in the right lower back region of the jaw since three months ago. Initially, the patient developed pain in the teeth in the fourth quadrant of the jaw, followed by a localized swelling. Within one month, the teeth became mobile and were extracted, however the swelling persisted and grew progressively. The patient had no contributing medical history however she had a habit of quid chewing two to three times per day since 20 years ago.\nGeneral physical examination of the patient revealed signs of anemia with pale conjunctiva and nail beds. There was a gross facial asymmetry with diffuse swelling involving middle and lower third of the right side of the face extending from ala-tragus line to approximately four centimeters beyond the inferior border of the mandible (Figures 1 and ). The swelling extended anteriorly to the angle of the mouth and posteriorly to the posterior border of the ramus of the mandible. On palpation, two swellings were appreciated. One swelling which was extending from ala-tragus line to the inferior border of the mandible was fixed. The other swelling extended below the inferior cortex, ovoid in shape and measured approximately four centimeters in diameter. Both of the swellings were tender and firm in consistency. The condyle of the mandible on the same side was not palpable, while the jaw was deviated to the right on opening and closing the mouth (). The bilateral submandibular lymph nodes were palpable, tender, firm in consistency and not fixed. Intraorally, vestibular obliteration was seen extending from the canine to the anterior border of the ramus, posteriorly. The extraction socket distal to the second premolar showed erythematous granular mass covered partly with necrotic slough (). There was no obvious relation between the mandibular mass and the overlying mucosa. On palpation, the findings of inspection were confirmed, while the swelling was tender and indurated with no cortical expansion. First and second premolars were vertically compressible into their sockets. Considering the aggressive nature and the extension of the growth, carcinoma of the right mandibular alveolus and intraosseous carcinoma were considered in the differential diagnosis.\nPanoramic radiograph revealed gross destruction of the right body and ramus of the mandible extending from the right lower canine to the mandibular notch with bay and promontory appearance of the margins. Right lower premolars showed floating tooth appearance with few radiopaque flecks of bone scattered within the lesion (). Posterior-anterior radiograph of mandible showed involvement of both medial and lateral cortical plates (). A screening chest radiograph was made to rule out any primary lesion in the lungs however, nothing abnormal was found.\nCT scan was performed to know the extension of the lesion. It revealed a destructive lesion in the right half of the mandible extending from subcondylar to right parasymphyseal region. It showed a unicentric lesion with inside out growth and permeative type of destruction. The margins were indistinct with wide zone of transition suggesting an intraosseous malignant neoplasm. Masseter and part of pterygoid muscle was bulky suggesting infiltration. CT scan also showed a large oval well-defined submandibular mass with central necrosis suggestive of metastatic submandibular lymph node ().\nHigh resolution ultrasonography was performed to know the lymph node involvement. It revealed a well defined nodular mass inseparable from the right submandibular salivary gland with central cavitation and papillary projections into the cavity suggesting a neoplastic process involving the right submandibular gland ().\nAspiration of the submandibular mass was done which revealed a clear straw colored fluid (). Cytological smear showed squamous cells with mild pleomorphism and large number of inflammatory cells spread over a background of eosinophilic acellular material. Histopathological report revealed islands of tumor epithelial cells with abundant keratin formation scattered over a densely fibrous stroma with dense chronic inflammatory infiltrate suggesting a well- differentiated squamous cell carcinoma ().\nPatient was subjected to wide surgical excision of the lesion (hemimandibulectomy). Radical neck dissection was performed using Macaffe incision sacrificing internal jugular vein, sternocleidomastoid muscle, spinal accessory nerve, omohyoid muscle, facial artery and vein, occipital artery and submandibular gland and its duct. Reconstruction was done with pectoralis major myocutaneous flap. Follow up of the patient showed satisfactory healing of the wound with mild disfigurement of the face due to scar contracture ().
A 68-year-old male with past medical history of coronary artery disease status post coronary artery bypass graft (CABG), aortic valve replacement along with replacement of the root of the ascending aorta 10 months prior to presentation and recent hemorrhagic cerebrovascular accident (CVA), came to the hospital with complains of acute onset of severe abdominal pain and melena for 1 day. He also attested to chronic abdominal pain and a 30-pound weight loss over the last 8 months prior to these acute symptoms. His physical exam on presentation was positive for severe bilateral lower abdominal tenderness. Apart from a hemoglobin of 10 mg/dl and a positive stool occult blood test, the rest of his basic lab work up was unremarkable (white blood cell/platelet count, comprehensive metabolic panel, and PT/INR included). Hepatitis B, hepatitis C, and human immunodeficiency virus (HIV) testing were negative. The electrocardiogram (EKG) showed sinus rhythm and left ventricular hypertrophy (). An emergent computerized tomography (CT) scan of the abdomen revealed features suggestive of an embolic infarct in the left kidney (Figures and ) and within the mid-one-third of the superior mesenteric artery causing luminal narrowing and also suspected to be extending to the takeoff of small bowel branches. Segmental mural thickening of at least one small bowel loop was noted which strongly favored acute bowel ischemia as a cause of his abdominal pain (). Incidental findings on CT of the abdomen were also strongly suspicious for large eccentric thrombus in the ascending aortic graft and the aortic root which were further investigated and confirmed with a CT scan of the chest (). Cardiology and cardiothoracic surgery were consulted. A CT scan of the head was performed to assess the recent CVA and showed a subacute hemorrhage along the left-sided temporal parenchyma (). CT head imaging was obtained from the facility where the patient presented 3 months prior for cerebral hemorrhage and in comparison, to the most recent CT scan of the head, the hemorrhage appeared stable. The hemorrhage was suspected to be secondary to thromboembolism. After a review of the risks and benefits of anticoagulation to prevent extension of this suspected thrombus, heparin was initiated. An echocardiogram revealed dilatation of the ascending aorta and mild paravalvular leak around the bioprosthetic aortic valve. Gastroenterology was consulted and an emergent esophagogastroduodenoscopy was performed which was negative for any causes of upper gastrointestinal bleed. A hypercoagulable workup was performed which did not reveal any apparent cause of a prothrombotic state. Anticoagulation was held and subsequently, the patient underwent a redo sternotomy under cardiopulmonary bypass with extensive lysis of adhesions, removal of the thrombosed aortic valve and graft, ascending and proximal aortic arch replacement utilizing a 30 mm Dacron graft, and aortic valve replacement with a 25 mm Edwards Magna Ease bovine pericardial valve. The patient also underwent an explorative laparotomy as a part of a staged procedure to address the ischemic bowel caused by the presumed septic emboli. Intraoperatively, the patient was found to have a portion of small bowel that had become necrotic. The necrotic bowel was excised and an end to end anastomosis was performed.\nThe aortic graft and thrombus were sent for culture and pathology. Histopathological examination of the aortic graft and cusps of the aortic valve revealed chronic inflammation and was also notable for abundant acute angle branching septate fungal hyphae (). The patient was immediately started on amphotericin B and voriconazole pending finalization of cultures and sensitivities. On postoperative day four, three culture reports from the graft came back positive for dematiaceous mold, suggestive of Bipolaris species. The minimum inhibitory concentration (MIC) was 0.25 ug/dl for voriconazole and 0.03 ug/ml for amphotericin B. A decision was made to continue the same antifungal regimen on the basis of sensitivities and further speciation was not performed. Patient had a good postoperative course and was later discharged on amphotericin B and voriconazole for at least 1 month with continued follow-up with an infectious disease specialist.
A 71-year-old man with a known history of end-stage diabetic nephropathy and hypertension presented to the emergency room with a history of acute right abdominal pain for six hours. The pain was localized in the right loin, colicy in nature, and radiated to the right inguinal region. He was on the chronic hemodialysis program for chronic renal failure and had just completed his last dialysis eight hours earlier. Examination demonstrated an elderly man in some distress with right loin tenderness and guarding. Blood pressure and pulse were within normal limits for his age. Laboratory results were normal except for an elevated C reactive protein level and serum creatinine. His hemoglobin and the white cell count were within normal limits. A working diagnosis of right renal colic was made and an urgent supine abdominal radiograph and non enhanced CT scan of the kidneys, ureters, and bladder (KUB) were requested to confirm the clinical diagnosis. The supine abdominal radiograph () demonstrated extensive arterial calcification, what was initially considered to be a “normal bowel gas” pattern but no radio-opaque calculi were detected. A nonenhanced CT KUB scan was performed to detect ureteric obstruction and hydronephrosis from renal or ureteric calculi. On the CT study small quantities of linear gas were detected in the peripheral portal vein branches of the caudate lobe of the liver (), the peripheral mesenteric veins branches to the caecum and ascending colon, and a thickened wall of cecal wall with pericecal fat stranding (). Calcification of the walls of the superior and inferior mesenteric arteries was noted. Both kidneys were small with cortical thinning but there were no renal or ureteric calculi detected. There was no hydronephrosis or ureteric dilatation present. A radiological diagnosis of cecal and ascending colon infarction was made. The patient was taken to theatre for laparoscopy which confirmed patchy cecal wall infarction. A right hemicolectomy was then performed with a primary anastomosis. Transmural necrosis of the cecal wall was confirmed on histology. The patient had a stormy postoperative course but has since recovered from his surgery 4 months ago.
A 25-year-old Mexican female with family history of ovarian cancer at her maternal side and personal history of a mass on her right breast clinically and radiologically diagnosed as fibroadenoma 2 years ago. The same mass had a growth of 2 cm so an incisional biopsy was performed at another institution. This was positive for mucinous carcinoma, and the patient was referred to our institution for treatment and follow up.\nAt our institution in the biopsy slides revision, we found a tumor composed of mucinous lakes with numerous tumor cells of medium to large size with a moderate amount of eosinophilic cytoplasm confirming the presence of pure mucinous carcinoma. We could not identify the tumor size and surgical margins by no prior radiological and pathological report. []. The tumor was classified as a Low grade tumor (G1) based on the Scarff Bloom Richardson scale. We did not observe perineural or vascular invasion. On immunohistochemistry the results were 100% and 90% positive for estrogen and progesterone receptors respectively with high intensity, and HER2 receptors negative.\nOn physical examination a surgical injury was seen. Upon palpation, this zone felt indurated, edematous and presented as a rough irregular texture. No other lesions were found. An ultrasound was performed, showing avascular distortion and a heterogeneous mass with microcysts (white arrows) []. MRI showed a hypointense irregular mass that indicates a mild enhancement in contrast sequences and a hyperintense sequence in SPAIR. The evaluation of the lymph nodes was not suspicious for metastasis.\nWith the radiological suspicious of residual tumor and pathological findings accomplished at our institution a partial mastectomy and a sentinel lymph node biopsy was performed. Pathological findings showed a residual mass of 25 mm of pure mucinous carcinoma with negative surgical margins. Sentinel lymph node was negative for metastasis.\nMolecular screening with EndoPredict clinic showed a score of 2.7 classifying it as a low risk, reason why this patient was treated with tamoxifen because her positivity to receptors and low risk showed in molecular assay. The clinical and pathological stage IB was confirmed.
A 61 year old current male smoker presented with moderate exertional angina and shortness of breath. Significant comorbidities included intermittent claudication, chronic bronchitis and asbestosis related benign pleural disease. Examination revealed a harsh ejection systolic murmur across the whole precordium, radiating to carotid area. Echocardiogram confirmed moderate aortic stenosis with a peak gradient of 62 mm Hg and mean gradient of 34 mm Hg across the aortic valve, mild aortic regurgitation, left ventricular hypertrophy and preserved left ventricular function. Left heart catheterization demonstrated a gradient of 30 mm Hg across the aortic valve. Aortic root angiogram showed mild aortic regurgitation and a small and atretic normally arising proximal right coronary artery and a normally arising left coronary artery (Fig ). The selective right coronary angiogram demonstrated the atretic right coronary artery (RCA) supplying the SA nodal, right atrial and the proximal right ventricular branches and petering out thereafter (Fig ). The selective left coronary angiogram showed a normal left main stem (Fig ), ostial and mid vessel stenotic disease in left anterior descending artery (LAD) and a normal circumflex artery (Fig ). LAD continued across the left ventricular apex as posterior descending artery (PDA), running along the posterior interventricular septum up to the atrioventricular groove, where it gave off the left ventricular branch to the inferior surface of left ventricle and thereafter continued as the distal RCA without establishing any communication with the atretic proximal RCA (Fig ). Left ventriculogram confirmed well preserved left ventricular function. At operation, there was moderately severe left ventricular hypertrophy. The left anterior descending artery, after running its normal course in the anterior interventricular groove, ran across the left ventricular apex (Fig ) to gain the posterior interventricular groove, where it continued as the posterior descending artery up to the crux of the heart, thereafter ascending for a brief distance as the distal right coronary artery after having given off a smaller left ventricular branch to the inferior surface of the left ventricle. There was significant palpable disease in proximal and mid LAD as well as in its large diagonal (Dx) branch. There was no continuity between the atretic proximal RCA and the anomalous distal RCA. Circumflex artery was a normal sized vessel with a normal sized obtuse marginal branch. Aortic valve was tricuspid in configuration, moderately stenotic with fused commissures and thickened and calcified leaflets.\nEmploying cardiopulmonary bypass with moderate hypothermia and right superior pulmonary vein vent and using both antegrade and retrograde cold blood cardioplegic arrest, aortic valve replacement with 21 mm St Jude mechanical prosthesis and triple coronary artery saphenous vein bypass grafting to LAD, Dx and PDA was performed. Patient made uncomplicated postoperative recovery, was transferred to ward on 2nd postoperative day and home on 8th postoperative day.
A 50 year old man presented to our outpatient department with a three month history of an altered consistency in his right testicular prosthesis. There was no history of trauma, pain or systemic upset. Scrotal examination revealed a palpable left testicular prosthesis and an irregular soft mass was noted in right hemiscrotum. The overlying skin was normal and no regional adenopathy was evident.\nHis past history was remarkable for a right testicular teratoma seventeen years earlier treated by radical orchidectomy and adjuvant chemotherapy (Belomycin, Etoposide and Carboplatin). Twelve months following this he underwent retroperitoneal lymph node dissection for residual adenopathy. Four years later he represented with a second testicular tumour in his left testis which was treated with radical orchidectomy. Histology revealed malignant teratoma which was again treated with adjuvant chemotherapy. Following his second radical orchidectomy he opted for bilateral testicular prosthesis insertion in 1996 with concomitant testosterone replacement therapy. Follow up since insertion had been unremarkable.\nPreliminary laboratory investigations revealed normal full blood count, renal profile, erythrocyte sedimentation rate and tumour markers. Scrotal ultrasonography revealed a normal contralateral left testicular prosthesis (figure ) and a ruptured right prosthesis with reverberation artefact described as a "stepladder" pattern [] on sonographic findings typically found in breast prosthesis rupture (figure ). Following discussion with the patient, and in view of his asymptomatic state it was decided to leave the prosthesis in situ and adopt a conservative management strategy with biannual outpatient review.\nRupture remains an infrequent occurrence []. It is accepted that the longer the time interval between initial native testis removal and placement of a prosthesis the greater the incidence of complication []. John et al have previously documented a twelve year interval between placement and rupture in a patient who required exploration and prosthesis removal []. In this case the patient had noticed no difficulties with his bilateral implants up to thirteen years post insertion. Hage et al in 1999 described cases of unilateral testicular implant rupture in a selected series of patients who had undergone transgender surgery with concomitant neoscrotal formation and bilateral implants. All of these patients had a history of trauma or suspected intraoperative puncture and all underwent exploration of the affected area [].
A 47-year-old female with renal failure secondary to adult polycystic kidney disease had repeatedly refused the formation of an AV fistula for haemodialysis due to severe needle phobia. She was commenced on peritoneal dialysis in 2008, which became inadequate as a result of membrane failure in 2010. She was commenced on haemodialysis via twin tunnelled Tesio® (Medcomp, PA, USA) lines inserted via a left internal jugular approach having had temporary right internal jugular access previously.\nShe was referred to the vascular surgery unit with the line in situ for 2 years following a failed attempt to remove the malfunctioning venous limb of the catheter on the renal unit. A chest X-ray showed the tip of the catheter at the level of the right atrium (Fig. ). Following discussion at the multi-disciplinary meeting, the decision was made to attempt removal of the line by a combined endovascular and surgical approach under general anaesthesia.\nUnder general anaesthesia, a cut down was performed onto the left internal jugular vein at the entry point of the catheter. The fibrin sheath was incised and traction on the catheter failed to release it. Simultaneous traction and snaring of the catheter tip via a femoral venous approach was failed. A hydrophilic guide-wire was passed through the catheter and out of a side hole of the line, which was then snared in the right atrium. Synchronous traction was applied to both ends of the line via the snare and the external portion of the line, but the line could not be freed. Balloon angioplasty within the lumen of the line was performed in order to attempt splitting of the surrounding fibrin sheath. Furthermore, upon traction of the external portion of the line, the unusual finding of a palpable transmitted pulse via the catheter was noted. In order to investigate this further, a trans-oesophageal echocardiogram was performed intra-operatively. This showed the proximal portion of the line passing through the tricuspid valve, with its tip adherent to one of the valve leaflets (Fig. ). At this stage, the external part of the catheter was excised and the catheter remnant internalized with a plan for subsequent removal via open-heart surgery.\nAt median sternotomy with cardio-pulmonary bypass, the line was identified with its tip attached to the septal leaflet of the tricuspid valve (Fig. ). The tip of the line was disengaged, the line removed and the leaflet defect repaired. The patient made a complete recovery, was recommenced on dialysis via a right internal jugular line and discharged home 10 days following the surgery. Unfortunately the patient steadfastly refuses the formation of an AV fistula for vascular access.
A 56-year-old man of Asian origin was in his usual state of health until 4 days prior to presentation in our emergency room (ER); he had complaints of a rapidly progressing weakness of bilateral upper and lower limbs immediately prior to which he had a gastrointestinal upset. The weakness started from his lower limbs and gradually involved bilateral upper limbs in a similar glove and stocking fashion. He, however, did not have any sensory deficits and at the initial presentation in our hospital he had no respiratory and ocular muscle involvement. Consciousness and orientation were intact and he did not have significant hemodynamic instability. There was no significant medical or surgical condition requiring long-term hospitalization or medication use in the past. A government officer by profession, our patient had an active lifestyle and had no history suggestive of substance abuse or accidental or intentional poisoning. He lived with his wife and had two sons; one of his sons was living with him to support him and the elder son lived abroad but had been on good terms with the family. Our patient did not take any regular medications apart from the hypoglycemic agent metformin 500 mg administered orally twice daily. He did not smoke tobacco or consume alcohol regularly. During his initial presentation in our intensive care unit (ICU), he was conscious yet unable to speak properly. His vital signs were blood pressure (BP) 120/65 mmHg with no inotropic support, heart rate (HR) 102/minute regular, respiratory rate (RR) 26/minute regular, and he had no fever on admission.\nOur initial assessment led to a provisional diagnosis of GBS and immediate supportive tests were performed. A nerve conduction velocity test showed findings of motor axonal and demyelination neuropathy. A lumbar puncture done on the sixth day of the development of symptoms showed evidence of albuminocytologic dissociation with total counts (TC) of five cells/cc, which were all lymphocytes and CSF protein of 81 mg/dl (Table ). On the sixth day of the development of symptoms and third day of admission in ICU, he had complaints of difficulty in breathing with gradual decline in saturation with pulse oximeter reading of oxygen saturation (SpO2) to < 85% at fraction of inspired oxygen concentration (FiO2) of > 80%, and hypercapnia with partial pressure of carbon dioxide in arterial blood (PaCO2) of 86 mmHg. He was immediately intubated and kept on mechanical ventilator support.\nDiscussion was held with his relatives regarding available treatment options. A plan to initiate IVIG was made and started at 0.4 mg/kg per day for 5 days. The administration of IVIG was not associated with any significant complications. He did not, however, show major signs of recovery from respiratory weakness and was continuously kept on assist-control mode of ventilation with intermittent spontaneous breathing trials. At this time, he had occasional blood-tinged secretion in the subglottic suction and during intermittent endotracheal suctioning. A detailed coagulation profile did not show significant abnormalities. On the ninth day of admission in ICU, a plan for tracheostomy was made anticipating prolonged need for mechanical ventilation and as a part of routine pre-anesthetic preparation, a unit of group-specific (A +ve) blood was asked to be arranged. However, we were then notified by the blood bank that they had problems with cross-matching of the blood. A repeated blood sample of our patient was sent which also had a similar problem of inability to cross-match the blood. A literature search for the possible causes of such an occurrence was made but we only had a few reports of such problems. With a provisional diagnosis of significant hemolysis leading to cross-matching difficulties, further tests were sent (Table ). An arrangement of O negative blood was made as a reserve and a tracheostomy was performed with no major problems.\nHis stay in ICU was then complicated with hospital-acquired chest infections for which he received antibiotics based on organisms’ susceptibility. Liver function tests (LFTs) which were initially deranged subsequently normalized after gradual stabilization of his condition and de-escalation of drugs. Serological tests which included quantitative HIV, hepatitis B surface antigen (HBsAg), and anti- hepatitis C virus (HCV) antibody were negative. He was subsequently moved out of ICU on the 19th day with tracheostomy in situ and on portable bilevel positive airway pressure (BIPAP) support. Three days after being moved to a ward, he was brought back to ICU for sudden-onset dyspnea and tachypnea. He had coarse crepitations more on the anterior aspect of bilateral chest and slight decrease in breath sounds on bilateral basal regions. He did not, however, have fever or changes in vital signs and his consciousness was intact. He was managed conservatively with chest physiotherapy, deep breathing exercises, regular tracheostomy care, and suctioning of secretions from lungs. He recuperated in 2 days and was moved back to a ward where he slowly recovered from his weakness. No other untoward events occurred during this period of approximately 4 months. He is being planned for discharge to the care of a nursing home and the prognosis of the disease has been well explained to his relatives.
A 65-year-old woman with a history of laparoscopic DG with lymph node dissection for early gastric cancer 6 months earlier had a 40 mm splenic tumor that was growing. During laparoscopic DG, lymph node dissection around the common hepatic artery and ligation of the left gastric artery, right gastric artery, and left and right gastroepiploic arteries were performed. Pathological diagnosis of gastric cancer was pT1bN0M0 StageIA (UICC 8th TNM). The splenic tumor with 30 mm large in the lower pole of the spleen was incidentally detected before DG with enhanced computed tomography (CT). It was diagnosed as a benign tumor with well-defined margins and homogeneous enhancement (). The splenic tumor was observed for 6 months without resection after DG. Intraoperative biopsy of the splenic tumor was not performed during DG due to the risk of complications such as bleeding. However, in the subsequent enhanced CT suggested the tumor increased in size to a diameter of 40 mm and positron emission tomography showed abnormal 18F-fluorodeoxyglucose uptake in the splenic tumor () with a possibility of being a malignant splenic tumor such as malignant lymphoma or metastasis. Three-dimensional CT angiography showed that the PGA and LIPA had become more developed to supply the remnant stomach ().\nWe planned to perform laparoscopic splenectomy without resection of the remnant stomach in case of adequate preservation of the blood supply to the remnant stomach after SGA resection. During laparoscopic splenectomy, we resected the SGA, so the arterial blood supply to the remnant stomach depended on the PGA and LIPA. We examined the blood supply to the remnant stomach with intraoperative ICG fluorescence imaging using a near-infrared camera system after clamping the splenic artery and vein at the splenic hilum, the planned transection line. First, 5 mg of ICG in 2 mL of saline was gently injected through a peripheral vein. ICG fluorescence imaging was illuminated with a near-infrared laser beam in the laparoscopic system. Imaging was generated using the 1588 AIM Endoscopic Near Infrared Visualization camera system (Stryker Corporation, Kalamazoo, MI, USA). Within 45 s after ICG injection, blood flow from the greater curvature spreading to the lesser curvature of the remnant stomach was visualized with ICG fluorescence (/Fig. 4). The remnant stomach did not show any perfusion defects, suggesting adequate blood supply from the PGA and LIPA, allowing for splenectomy with remnant stomach preservation. Total operative time was 143 min. Intraoperative blood loss was 5 mL.\nThe final pathological diagnosis of the splenic tumor was sarcoidosis. The patient was discharged on postoperative day 8 without complications. Two years after surgery, gastric cancer and sarcoidosis have progressed without recurrence.\nThis work has been reported in line with the SCARE criteria [].
A 75-year-old Caucasian female was referred to the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University of Vienna with a diagnosis of polyneuropathy. The patient described a bilateral heel pain with a gradual onset, beginning approximately 4 months before the referral. The pain was of an undulating intensity, with maxima reaching 81 mm on a visual analog scale while walking upon getting out of bed. As the painful area was located medially on the heel, the patient was forced to put her weight on the lateral rim of the foot while walking.\nThe walking ability was severely reduced, with a maximum uninterrupted walking distance of approximately 100 m. The limiting factor was a pain in the lateral compartment of the left knee, which had become apparent about the same time as the heel pain. However, recurring knee pain had been known for several years before the referral.\nShe was suffering from breast cancer, which was diagnosed in 2014 and had been treated with surgery, followed by adjuvant chemotherapy. Metastatic bone disease was ascertained at the time of diagnosis and consisted of lesions in the spine, pelvic bones, and several ribs. No recent progression of the underlying disease had been observed.\nIn the clinical examination, the heel pain was localized on both sides along the medial part of the insertion of the plantar aponeurosis on the calcaneus, as well as along the most medial bundle of its central part. Pressure on these areas elicited pain of the same quality and location as experienced during walking.\nA recent bone scintigraphy showed no tracer uptake in the feet. Uptake in the medial condyle of the left femur and of the left tibia was consistent with osteoarthritis.\nPlain radiographs revealed a plantar heel spur on both sides, without indication of bone lesions. The plain radiograph of the left calcaneus is shown in Fig. .\nUltrasound showed a thickening of the plantar aponeurosis near the insertion on the calcaneus, with a thickness of 4.9 mm on the left side and 4.8 mm on the right side.\nPolyneuropathy was excluded through electrodiagnostic testing.\nfESWT was administered on both heels over 5 weekly sessions using the F10G4 therapy source of a PiezoWave 2 device (Richard Wolf GmbH, Knittlingen, Germany). One thousand five hundred impulses were administered on each foot with a frequency of 5 Hz, at a focus depth of 15 mm and an average energy flow density of approximately 0.39 mJ/mm2. The focus depth was chosen according to the thickness of the tissues superficial to the aponeurosis, measured during ultrasound imaging.\nfESWT did not cause any adverse events. The reported pain intensity decreased gradually after each session. At week 6, the patient reported a maximum pain intensity of 14 mm on the visual analog scale. The patient reported no pain in the heels up to 12 weeks after fESWT, during which time she received physical therapy for knee pain due to osteoarthritis.
A 30-year-old woman presented with a 3-month history of a painful mass and a 1-month history of skin ulceration on her left breast. She had been pregnant once and delivered a son 18 months before the symptoms developed. Her left breast had been kicked by her son, after which a breast mass that measured approximately 4 × 4 cm in diameter developed. Initially, the patient developed pain in the left breast with progressive growth of the mass. Before being admitted to our hospital, she was initially treated with penicillin and cephalosporin intravenously, and she took oral and topical Chinese herbal medicines for 2 weeks previously. None of these treatments stopped the growth of the breast mass or alleviated her pain. Meanwhile, she developed other symptoms, including skin ulceration of the left breast, nodular erythema on both her shins, and swelling and pain in both knees and ankle area, which made walking without support difficult.\nAt the time of hospital admission, the patient was afebrile (temperature, 36.3°C) and in apparent good general condition. She complained of pain accompanied with tenderness in the left breast. Upon examination by a specialist, a hard mass measuring 10 × 13 cm with an ill-defined boundary was palpable in the left breast, which also had nipple retraction. Several skin ulcerations on her left breast were noted. The breast mass occupied almost the entire left breast and was connected to the ulcerated skin but not to the chest wall. The right breast appeared normal. The patient showed symptoms of an autoimmune disease: Moderate pitting edema in the lower limbs; swelling and tenderness in both knees and the ankle area; multiple erythematous nodules on both shins and around the ankle area; and tenderness in the erythematous area.\nConsistent with diagnosis by palpation, Doppler ultrasound examination of the left breast revealed a diffuse thickening of the mammary gland [], and mammography revealed a heterogeneously dense region with no sign of calcification in the left breast []. Magnetic resonance imaging (MRI) was also performed on both breasts and demonstrated evident asymmetry in the appearance of the breasts and ill-defined margin of the left mammary gland []. Consistent with the patient's lack of response to antibiotic treatment, there was no evidence of bacterial growth in the ulceration exudate in culture, and we did not detect any microbes in the smear test. Therefore, we excluded microbe infection as the cause of GM in this patient.\nBlood tests suggested that the patient had leukocytosis (white blood cells: 13.25 × 109/L; neutrophils: 10.60 × 109/L; lymphocytes: 1.89 × 109/L; eosinophils: 0.01 × 109/L; and basophils: 0.02 × 109/L) without anemia or thrombocytopenia (hemoglobin: 114 g/L and platelet count 460 × 109/L). Her rheumatoid factor level (20.1 IU/mL) was slightly higher than normal (<20 IU/mL), and her C-reactive protein (5.11 mg/dL) was significantly higher than normal (<0.8 mg/dL). The levels of autoimmune antibodies, Such as antistreptolysin O, anticyclic citrulline peptide, immunoglobulin (Ig) G, IgA, and IgM, were all within the normal range and the levels of both perinuclear antineutrophil cytoplasmic antibody (pANCA) and cytoplasmic antineutrophil antibody (cANCA) were below the detection limit.\nUpon admission, the patient received levofloxacin and metronidazole intravenously for 1 week. The ulcerated area was cleaned and the abscess was drained. However, despite these treatments, her condition did not improve. Her body temperature fluctuated between 36.4°C and 37.8°C. Pathological examination on fine needle biopsy of the breast mass revealed the presence of a large number of neutrophils, lymphocytes, and plasma cells in the stroma of the left mammary gland. In addition, there was infiltration by multinucleated giant cells and the formation of small blood vessels []. This result further confirmed the clinical diagnosis of GM.\nOral steroids were offered as a treatment but rejected by the patient because of her concerns about adverse side effects. To debulk the mass, we performed abscess drainage, after which the patient developed increased joint pain and nodular erythema. Upon the patient's request, conservative surgical resection of the left breast was performed. The patient's condition improved significantly after the surgery. Her breast and joint pain resolved, joint swelling and nodular erythema disappeared, and she was discharged 6 days after the surgery. To date, the patient has been followed-up for 11 months, and no recurrence has occurred.
A 12-year-old, previously healthy Hispanic female, presented for excess weight gain over the past 12 months. The patient and father reported that she was a sedentary teen at baseline and had a history of eating frequently throughout the day and into the night due to emotional distress or boredom. The family had not attempted any lifestyle interventions to date as the parent-teen relationship was quite strained and it was difficult for either parent to encourage or promote appropriate, healthy eating habits. After evaluation from the team, it was identified that one of the biggest sources of discord between the patient and parents was that the teen did not want to eat breakfast. It was determined that a TLE approach with the eating window starting at 11 AM would support the patient’s desire to not consume breakfast and create a structure to her eating pattern 3 days per week.\nThe entire family (mother, father, 15 and 7 year old sister) decided to implement a TLE approach consisting of eating between 11 AM-7PM, 3 days per week and adhering to an age-appropriate healthy diet the remaining 4 days of the week. The family returned for the 1 month visit and the patient’s zBMI had trended down by −0.2 SD. She reported great satisfaction with the intervention and shared that by removing the battle over her breakfast consumption with her parents the parent-teen relationship was improving. Her zBMI decreased by 0.32 SD over the 4 months follow up period compared to baseline (). She reported that the TLE approach was feasible and easy to adhere to and she enjoyed how her focus was not on the amount of food she was eating but the time of day she was eating. Per report, by removing the need for calorie counting or restriction of macronutrient content, she did not feel deprived and explained that it felt more like a lifestyle change than a traditional diet. The entire family, at the 4 month visit, reinforced this idea to the provider team.
A 14-year-old girl presented with a gradual onset of fatigue and apathy. Laboratory analysis revealed a pancytopenia as summarized in Table . Liver function tests were within normal limits. Her medical history was non-specific except for a preterm birth at 7 months and observation at the neonatal intensive care. At that time a venous umbilical catheter was placed for intravenous fluid administration. However, catheter position was not documented by abdominal plain film. There was no history of hepatitis or other diseases in this otherwise healthy girl. Screening abdominal ultrasound was within normal limits, except for a splenomegaly with a maximal splenic diameter of 17 cm. In order to exclude portal venous and hepatic parenchymal disorders a magnetic resonance angiography (MRA) as well as a transjugular liver biopsy and pressure measurements were performed. MRA revealed a discrete, focal irregularity of the extrahepatic portal vein main branch. The liver biopsy was within normal limits without signs of fibrosis or cirrhosis. Pressure measurements showed a wedged hepatic venous pressure of 11 mmHg and inferior vena cava pressure of 9 mmHg. Further, a gastroscopy was performed, revealing major varices in the lower esophagus and signs of hypertensive gastropathy. The varices were endoscopically ligated, as it was suggested that the anemia could be associated with occult or intermittent bleeding from these varices. Finally, additional laboratory analysis could not identify any thrombophilic parameter disorder. Based on these findings, it was decided to perform direct transhepatic portography and percutaneous treatment.\nUnder general anesthesia, access to the right portal vein was made using ultrasound guidance. Venography clearly revealed a moderate focal stenosis of the main portal vein, potentially associated with a focal intimal dissection. Additionally very large gastric and esophageal variceal collaterals were also clearly visible (Fig. a). Pressure measurements over the stenosis were: 16 mmHg proximal and 6 mmHg distal to the stenosis resulting in a pressure gradient of 10 mmHg. Balloon angioplasty with a 10-mm diameter balloon did not result in sufficient re-expansion of the stenosed segment. It was decided to insert a self-expanding nitinol stent with a length of 4 cm and a nominal diameter of 12 mm (Zilverstent, Cook Medical, Bloomington, IN, USA) which was postdilated up to 10 mm diameter. Pressure measurements after stenting were: 12 mmHg proximal and 10 mmHg distal to the stented segment resulting in a residual pressure gradient of 2 mmHg. Completion venography confirmed the correct appositioning and expansion of the stent and absence of any collateral flow (Fig. b). Patient recovered well after the procedure and could leave the hospital 2 days later. Follow-up was performed by clinical, ultrasound, and laboratory evaluation at regular intervals: every 3 months in the first year and yearly thereafter. Clinically, all signs of chronic anemia disappeared and gastroscopy was normal without visible esophageal varices or hypertensive gastropathy. Ultrasound evaluation could demonstrate a normal volume of the spleen with maximal diameters after 1, 2, 3, 4, and 5 years of 13.2 cm, 13.2 cm, 13.6 cm, 13.8 cm, and 14 cm, respectively, which is at the upper limit of normal values (12–14 cm). Duplex-ultrasound flow measurements were on all evaluations between 40 and 60 cm/s over a 5-year follow-up period. Finally, values of red blood cell, white blood cell, and platelet count normalized during follow-up (Table).
A 42-year-old male patient presented to out patient department (OPD) with a complaint of severe pain in right hip joint. After routine clinical and radiological examination, he was diagnosed to be suffering from advanced osteonecrosis of the right femoral head. Total hip arthroplasty (THA) was planned for the right hip. At the same time he had a mild pain in left hip joint. Radiographs of the left hip joint were normal. Taking into consideration the patient complaint and osteonecrosis of the right femur head, magnetic resonance imaging (MRI) scan was performed for the left hip joint to rule out early osteonecrotic changes as a cause of pain. MRI revealed large osteonecrotic lesion involving more than 80% geographical area of articular surface with MR crescent sign []. There was no apparent collapse of the subchondral bone. The Hospital for Special Surgery (HSS) score of left hip was 68 points at the time of surgery. Core decompression and porous tantalum rod insertion was done for the left hip joint. Postoperative period was uneventful. Patient was advised non-weight bearing mobilization with use of crutches for six weeks, followed by gradual increase to full weight bearing as tolerated. He had complete relief from pain and full range of motion for seven months after surgery. After that he complained of mild pain of gradual onset in the left hip and groin region. Radiographic examination showed a well placed implant with no signs of subchondral collapse or depression in the articular surface []. Patient was prescribed analgesic medication and advised to follow-up after one month. At the next follow-up, patient complained of worsening of pain which hampered his daily living activities. MRI scan of the left hip joint was performed to evaluate the status of osteonecrosis. The scan showed a well located metal implant (porous tantalum rod) in the necrotic area, with reactive marrow signal changes around the tip of the implant, without any evidence of femoral head collapse []. The reactive marrow signal was reported to be, probably revascularization signal change or reactive edema due to implant insertion. Patient was reassured about the clinical and radiological status of the disease process and prescribed analgesic medication. However, patient attended OPD after three weeks with excruciating pain and insisted on total hip arthroplasty (THA) for the left hip joint. The HSS score worsened to 78.6 points on last follow-up. Considering this case as a clinical failure of tantalum rod, THA was performed on the left hip joint. The femur head with tantalum rod in situ was sent for histopathological examination (HPE). Patient had complete relief from pain after THA on the left hip joint. The HSS score the case improved from 79 preoperatively to 96 points postoperatively.\nOn gross examination, the implant was well placed in the center of necrotic area []. There was a small gap between the implant and the adjacent bone at the distal interface with apparent new bone formation around the tip and the margins of the implant.\nThe HPE [] at 12× magnification revealed well formed bony trabeculae in contact with the implant surface without gap, but the cancellous bone around the tip was not new bone formation []. The implant revealed active proliferation of young fibroblasts in vascular rich stroma and dense cellular rim lining the surface of the implant material []. The cellular rim was supposed to be a possible osteoblastic proliferation that could not be technically evaluated in the specimen. On 100× magnification, several foci of new bone sprouting from the interface zone into the porous implant were evident []. Dead loose connective tissue existed between the implant and irregularly disarrayed cancellous marrow bone at the tip of the implant.\nThese findings could be compared with failure cases in our study, which had associated radiological progression. The pores of the failed implants had dead marrow tissue with fat necrosis and infiltration of chronic inflammatory cells.
A 57-year-old male with advanced upper and middle thoracic esophageal cancer underwent a subtotal esophagectomy via the transthoracic approach and a reconstruction via a gastric tube through the posterior mediastinal route at our hospital. The pathological examination revealed poorly differentiated squamous cell carcinoma that had invaded the adventitia and involved the lower thoracic paraesophageal lymph node. Therefore, the pathological diagnosis of the tumor was T3N1M0 Stage III according to the TNM classification of the International Union for Cancer classification []. Because the cancer involved tissue near the surgical margin of the cervical esophagus, the patient underwent postoperative CRT.\nFive years later, gastrointestinal fiberscopy and endoscopic ultrasound revealed intra-submucosal gastric tube cancer, which was located on the anterior wall of the antrum (Fig. ). The pathological diagnosis of biopsy specimens was signet ring cell carcinoma. Computed tomography (CT) did not show either enlarged lymph nodes or distant metastasis.\nThe gastric tube cancer was considered a contraindication for endoscopic resection because of the histological type and the fact that it had invaded the deep submucosal layer. Moreover, we considered that surgically removing the gastric tube via the posterior mediastinal route would be extremely difficult, and the patient also preferred to avoid this procedure. Thus, we treated the gastric tube cancer by CRT using docetaxel and S-1 with a concurrent total dose of 60 Gy of radiation. A clinical study performed at our institute indicated that the docetaxel/S-1 combination had the potential to improve the survival in patients with advanced or recurrent gastric cancer [], so we select this regimen for our patients with gastric tube cancer.\nThis strategy considerably reduced the size of the cancer, and resulted in a complete response. The patient then underwent maintenance chemotherapy with S-1 for 1 year. The patient was monitored biannually by gastrointestinal fiberscopy and CT, and recurrent foci remained undetectable over the follow-up period. However, several chronic ulcers and ulcer scars were sometimes evident in the gastric tube, and the patient continued to take a proton pump inhibitor.\nThree years after the CRT for gastric tube cancer, he visited our hospital on an emergency basis with severe dyspnea and fatigue. A blood gas analysis on admission revealed severe respiratory failure with a PaO2 of 65.9 mmHg and a PaCO2 of 34.5 mmHg (O2, 5 L/min). The findings of chest X-rays and CT images revealed severe pneumonia, atelectasis and pneumothorax (Fig. ). He was transported to the intensive care unit, where he was intratracheally intubated and placed on a ventilator with 60 % oxygen inhalation.\nThe patient was administered an antibiotic agent, and the right thoracic cavity was drained. Air did not leak, and turbid fluid did not flow from the thoracic tube. Bronchoscopy confirmed the presence of a fistula delivering digestive juice into the lung between the base of the right inferior lobe bronchus and the gastric tube (Fig. ). Therefore, the severe pneumonia was considered to have occurred due to the reflux of digestive juice into the lung through the fistula. Furthermore, because air did not leak and turbid fluid did not flow from the thoracic tube, we considered that a rupture from the GBF into the pleural cavity had not occurred, and the pneumothorax on a CT was not secondary to the GBF, but was actually a mild spontaneous pneumothorax, because the patient developed a spontaneous pneumothorax several times previously due to severe emphysema.\nThe contents of the gastric tube were drained by inserting a nasogastric tube with suction. However, digestive juice frequently refluxed into the lung, and the respiratory failure persisted. We considered that conservative therapy alone would be insufficient to allow the patient to recover from such a terminal state, and thus, surgical intervention remained the most feasible option at 3 days after admission.\nWe considered that separating the gastric tube from the bronchus by repairing the defects would be very difficult in this patient, because the severe respiratory failure rendered an invasive procedure via a thoracotomy and one-lung ventilation impossible. Therefore, we decided to leave the GBF and separate the respiratory and digestive tracts without a thoracotomy. We transected the duodenum, inserted a decompression catheter into the gastric tube as a gastrostomy, brought the cervical esophagus to the left cervical region as an esophagostomy and closed the upper end of the gastric tube. A feeding jejunostomy was also constructed for postoperative nutritional management (Fig. a). At the end of the procedure, a tracheotomy was prepared, and artificial ventilation was managed with as low an airway pressure as possible.\nThis procedure stopped the reflux of digestive juice into the lung through the fistula, and the patient recovered from the severe pneumonia. The patient then underwent 2 months of management with antiulcer agents, physical rehabilitation and nutritional support to prepare him for reversing the esophageal discontinuity.\nThe esophageal defect was repaired via a secondary reconstruction 70 days after the first surgery. The esophagus was reconstructed using a pedicled jejunum with microvascular anastomosis in a Roux-en-Y fashion through a subcutaneous route in the anterior chest wall. The second and third branches of the jejunal vessels were divided and ligated near their roots, and the long segmented jejunum was subcutaneously pulled up together with a pedicle of the fourth branch. The internal thoracic artery and vein were then anastomosed to the third jejunal artery and vein, respectively, in the jejunal graft. The cervical esophagostomy was taken down, and the lifted ileum and cervical esophagus were manually anastomosed by hand sewing in the neck. The anal end of the pedicled jejunum was anastomosed to the jejunum in the Roux-en-Y configuration (Fig. b). The decompression tube was not removed at the secondary surgery, because it was considered necessary to remove gastric contents.\nThe patient started taking oral nutrition 20 days after the secondary reconstruction, and was discharged 38 days after the bypass.
A 56-year-old female was referred to our department from another facility in the patient’s area for a rapidly progressing tumor in the gallbladder and liver area. The patient reported several-month right upper quadrant pain and 4-kg weight loss over the past year. There was no laboratory sign of obstructive jaundice at the day of admission. Preoperative CT and MR scan (Figs. and ) of the liver was performed, and the patient was diagnosed with a tumor in the gallbladder area with a relatively massive infiltration of the S5 and S6 liver segments and extensive regions of necrosis. Given the potentially resectable lesion according to preoperative imaging, exploratory laparotomy was indicated to attempt radical resection. During the exploration, a voluminous tumor was found attached to the peritoneum. Intraoperative ultrasound was performed and revealed a tumor originating from the gallbladder bed area and reaching up to the area of the hepatic hilum and extensive involvement of the hepatoduodenal ligament by the tumor through the lymph nodes. The tumor was classified as inoperable due to this finding. But during the exploration, however, a rupture of the fragile tumor occurred with massive eruption of the necrotic mass and the gallbladder content into the abdominal cavity, accompanied by bleeding of the liver parenchyma. We decided that the condition could only be managed by attempting modified resection. We performed cholecystectomy and non-anatomical resection of hepatic segments S5 and S6 and partial resection of S4 without lymphadenectomy as a debulking operation (Fig. ). The course of hospitalization was uncomplicated, and the patient was discharged to home care on postoperative day 9. Histologically, the tumor was confirmed as MINEN of gallbladder (Figs. , , and ), and its non-neuroendocrine component had the character of moderately differentiated tubular gall bladder adenocarcinoma, while the neuroendocrine component had the appearance of small cell carcinoma and was dominant, accounting for more than 65% of the viable tumor. The neuroendocrine component contained extensive necrosis, with mitotic index 64/10 HPF and a proliferation index of 70% (Fig. ). It was therefore obvious that the prognosis and the subsequent biological behavior would be influenced in particular by the neuroendocrine carcinoma component. Six weeks after the discharge, the patient underwent a follow-up CT scan prior to the initiation of systemic therapy, which revealed a large recurrence of the disease at the resection surface of the liver accompanied by hilar lymphadenopathy. The patient was started on systemic therapy with etoposide and carboplatin in combination with somatostatin analogues with very good radiological effect. We use this regimen as a standard in patients with MINEN of gastrointestinal tract with dominant neuroendocrine component, even with no somatostatin receptors staining available. Now the patient is almost a year after being diagnosed with a tumor, after completion of 6 cycles of adjuvant chemotherapy (carboplatin + etoposide) in combination with biological therapy, the long-acting somatostatin analogues. The patient is in good clinical condition, and while a recently performed PET/MRI scan revealed a hepatic lesion and hilar lymphadenopathy in full regression, there was a spread of small peritoneal and pleural metastases, with a solitary metastasis in Th9. The condition was evaluated as disease progression stage according to RECIST criteria, the patient remains in the follow-up care, and it is now 13 months after surgery (Figs. , , and ).
A 40-year-old male presented to our office in 2017 complaining of chronic left knee pain. This was his first visit to this office and was establishing care after moving to the area. The patient's electronic medical record was obtained from an outside institution which indicated a past medical history of diabetes, hyperlipidemia, and hereditary multiple exostosis. The patient disclosed that he had multiple osteochondroma removal surgeries which included his left knee, lumbar spine, and left foot at an outside institution. In addition, he had a left hip arthroplasty with refractory numbness/tingling of the leg. The patient stated he had left knee pain for years until he had an osteochondroma removed in his left distal femur in 2016 which seemed to help for 6 months. He stated the pain returned at 7/10 and is worse with movements. He had limited range of motion with 70° of flexion, negative pain with varus and valgus movement, and negative secondary tests. Baseline X-rays were ordered for this patient's knee ().\nThere were osteoarthritic changes with significant osteochondroma formation; however, arthroplasty was not recommended at that time because of the patient's young age and his left lower extremity being distally neurovascular intact. The patient was adamant about having his knee replaced and was referred to a specialist at an outside institution where an arthroplasty of the left knee was performed. The patient returned 3 months later with continued limited active and passive range of motion (<90° flexion). Follow-up radiographs were ordered showing good alignment of a Smith & Nephew posterior stabilized prosthesis without subsidence (). It was decided to perform manipulation under anesthesia followed by 6 weeks of physical therapy which improved his range of motion to 110° flexion.\nThe patient continues to have limited range of motion of his left knee with flexion to 90°. The large posteriorly projecting osteochondroma of his left tibia continues to be asymptomatic and painless, and therefore, it was decided not to perform surgical interventions at this time. Performing an arthroplasty helped relieve the patient's pain, however, did not result in significant improvement of active and passive range of motion of the joint. Overall, the patient is satisfied with his knee replacement but still has refractory numbness/tingling of his distal left lower extremity from his prior hip replacement. The patient has chronic pain elsewhere, which is being closely monitored (Figures and ).
An 8-year-old girl presented with recurrent attacks of left flank pain of 6 months duration. She had no episodes of urinary tract infection. Ultrasound revealed left hydronephrosis with dilatation of the upper ureter. Intravenous pyelography (IVP) showed hydronephrosis with dilated left upper ureter with sudden tapering and normal lower ureter below the area of tapering []. She was diagnosed to have left upper ureteric obstruction and was taken up for laparoscopic exploration.\nDuring surgery, we found that the upper ureter near the site of obstruction was surrounded by a sheet of vessels []. The ureter was sandwiched between a dilated periureteric vein and the gonadal vein. The gonadal vein instead of crossing from the lateral to medial side of the ureter, made a U shaped loop laterally, attached to the ureter at the apex of U and was seen draining into an abnormally placed lumbar vein with tributaries coming from above and below the confluence. The part of gonadal vein attached to the ureter was thrombosed with turbulent flow seen near the junction of gonadal vein with the lumbar vein. A small artery was seen entering the vein above the site of clot and causing turbulent flow in the vein. All the vessels were ligated proximally and distally laparoscopically and the parts of vessels attached to ureter were removed. The dilated gonadal vein attached to the left ureter was found to contain clotted blood. Complete ureterolysis was done at the site of obstruction. As ureter seemed normal, ureteroureterostomy was not done A line diagram is produced here for understanding the anomaly [].\nPostoperatively, Doppler study was done to uncover major vascular anomalies and was found to be normal. Double J (DJ) stent was removed after 15 days. Subsequently the patient continued to have abdominal pain again and a repeat IVP at 2 months showed similar obstruction as in the past. The patient was operated again and the obstructing segment was excised and ureteroureterostomy was performed. Ultrasound done after 3 months showed complete resolution of hydronephrosis and the patient remained asymptomatic.
A 68-year-old male with multivessel CAD was referred from an outside hospital for surgical evaluation. He had been deemed not a PCI candidate based on his SYNTAX score and anatomy as well as being of too high risk for CABG. A thorough history revealed classic demand ischemia on exertion, and a physical examination was significant for a large discrepancy in the radial pulses with the left radial artery pulsation being significantly lower than that in the right arm. Blood pressure readings confirmed a difference of nearly 60 mmHg in systolic blood pressure between the two extremities. On cardiac catheterization, left subclavian stenosis was observed just distal to the origin of the artery as well as significant multivessel coronary artery disease and a porcelain aorta (). Further work-up included carotid duplex testing which revealed reversal of flow in the left vertebral artery confirming left subclavian artery stenosis, and noncontrast computed tomography verified the presence of heavily calcified ascending aorta.\nAfter numerous discussions with the patient, his family, and the interventional cardiology team, it was decided that total coronary revascularization via surgery was the optimal treatment. We believed an off-pump CABG procedure—with the inflow to the diagonal, obtuse marginal, and left anterior descending arteries all coming off of the left internal mammary artery—could be problematic in the patient's long term given his degree of left subclavian artery stenosis. As a result, we decided to stage a left subclavian artery stent placement with a next-day plan for a four-vessel, on-pump CABG (left internal mammary artery to left anterior descending artery, reversed saphenous vein graft to right coronary artery, reversed saphenous vein graft to diagonal artery, and reversed saphenous vein graft to obtuse marginal artery) and ascending aortic replacement. This was accomplished by first carrying out a right axillary artery dissection and sewing an 8 mm graft to the axillary artery after administration of 5000 units of heparin.\nSubsequently, the patient underwent deep hypothermic circulatory arrest with systemic cooling to 18°C body temperature for a total cooling time of 45 minutes. Then, under circulatory arrest, his ascending aorta was replaced with a 32 mm graft. Of note, we did visualize the left subclavian stent which was in appropriate position. During the rewarming phase, the planned CABG × 4 was carried out, and the patient underwent an unremarkable postoperative recovery and was discharged home after 4 days.
During routine upper limb dissection for first year medical students, the BP of 43-year-old female cadaver showed the complete absence of MCN and variant branches of MN in the arm of both the upper limbs.\nIn the right axilla, the lateral cord of BP had three branches i.e. lateral pectoral nerve, nerve to CB and lateral root of MN. The lateral pectoral nerve pierced the clavipectoral fascia and supplied the pectoralis major as usual but during its course it communicated with the medial pectoral nerve (MPN) of medial cord of BP (). The MCN was completely absent. The nerve to the CB was a small motor twig arising directly from the lateral cord which innervated the CB muscle. The lateral and medial root of MN formed the MN proper and descended between the biceps brachii and brachialis muscle. It then crossed the brachial artery from lateral to medial and finally entered the cubital fossa. In the arm, deep in the biceps brachii, the MN gave two branches: a direct branch to the biceps brachii and another long branch which, after supplying the brachialis muscle, continued as the LCNF and supplied the lateral side of the skin of forearm. The medial cord also varied in the origin of medial cutaneous nerve of arm and forearm arising from a single common trunk, which became separated in the middle of the arm ().\nThe lateral cord of BP in the left axilla branched four times: the lateral pectoral nerve; nerve to CB and lateral root of MN and an additional lateral root of MN. The MCN was absent and the nerve to CB was a small twig which supplied and terminated in the muscle itself (). No communication between lateral and MPNs was found.\nThe MN was formed by two lateral roots from the lateral cord and one from medial root from the medial cord. After the formation, the left MN had the same course as the right side. During its course between the biceps brachii and brachialis, the MN split off a single long branch from which branches to the biceps brachii and brachialis were derived. After supplying these two muscles it continued as LCNF, which supplied the lateral side of the skin of forearm. The medial cutaneous nerve of arm and forearm arose from a common trunk, and separated in the middle of the arm, similarly to the right side ().
An 89-year-old man presented with a history of episodes of a paroxysmal headache on the right occipital region, superior and posterior to the ear, over the past 40 years. He complained of abrupt onset, sharp, electric-like pain in the right occiput radiating toward the vertex accompanied by an intense and stabbing pain localized in the depth of the auditory canal. Palpation of the right greater occipital nerve elicited the pain. A single pain episode usually lasted for a few weeks. The pain in the depth of the right auditory canal did not appear during every episode but only when the occipital pain was severe. The pain attacks usually occurred at the time the patient felt fatigued or when he was afflicted by a cold with or without fever. During the latest episode, the occipital pain was accompanied by symptoms of a herpes zoster and/or herpes simplex reactivation in the territories of the right maxillary and auriculotemporal nerves. Specifically, the skin was erythematous and small vesicles appeared on the right side of the face, around the right mouth angle and the right external auditory canal. The viral smear was not obtained and the vesicles resolved spontaneously in weeks. The frequency of the pain attacks was increasing from once in every 1 or 2 years in the antecedent decade to two or three times per year in recent years. Neurological examination revealed normal findings with no hypesthesia in the affected area, no cranial autonomic features, and no occipital skin lesions. Pain was eased temporarily by local anesthetic nerve block with lidocaine. Magnetic resonance image (MRI) examination of the cervical spine and brain was normal. A diagnosis of ON in combination with NIN was established based on symptoms which fulfilled the International Headache Society (ICHD-II) criteria []. In the antecedent decade, the patient did not take any pain killer. In recent years, carbamazepine was used at a dosage of 0.3 g daily (divided into three times) during the pain attacks and the intensity and frequency of the pain attacks could be eased, but the episodic duration of pain attacks was not reduced.
A 56-year-old female was referred to our department from another facility in the patient’s area for a rapidly progressing tumor in the gallbladder and liver area. The patient reported several-month right upper quadrant pain and 4-kg weight loss over the past year. There was no laboratory sign of obstructive jaundice at the day of admission. Preoperative CT and MR scan (Figs. and ) of the liver was performed, and the patient was diagnosed with a tumor in the gallbladder area with a relatively massive infiltration of the S5 and S6 liver segments and extensive regions of necrosis. Given the potentially resectable lesion according to preoperative imaging, exploratory laparotomy was indicated to attempt radical resection. During the exploration, a voluminous tumor was found attached to the peritoneum. Intraoperative ultrasound was performed and revealed a tumor originating from the gallbladder bed area and reaching up to the area of the hepatic hilum and extensive involvement of the hepatoduodenal ligament by the tumor through the lymph nodes. The tumor was classified as inoperable due to this finding. But during the exploration, however, a rupture of the fragile tumor occurred with massive eruption of the necrotic mass and the gallbladder content into the abdominal cavity, accompanied by bleeding of the liver parenchyma. We decided that the condition could only be managed by attempting modified resection. We performed cholecystectomy and non-anatomical resection of hepatic segments S5 and S6 and partial resection of S4 without lymphadenectomy as a debulking operation (Fig. ). The course of hospitalization was uncomplicated, and the patient was discharged to home care on postoperative day 9. Histologically, the tumor was confirmed as MINEN of gallbladder (Figs. , , and ), and its non-neuroendocrine component had the character of moderately differentiated tubular gall bladder adenocarcinoma, while the neuroendocrine component had the appearance of small cell carcinoma and was dominant, accounting for more than 65% of the viable tumor. The neuroendocrine component contained extensive necrosis, with mitotic index 64/10 HPF and a proliferation index of 70% (Fig. ). It was therefore obvious that the prognosis and the subsequent biological behavior would be influenced in particular by the neuroendocrine carcinoma component. Six weeks after the discharge, the patient underwent a follow-up CT scan prior to the initiation of systemic therapy, which revealed a large recurrence of the disease at the resection surface of the liver accompanied by hilar lymphadenopathy. The patient was started on systemic therapy with etoposide and carboplatin in combination with somatostatin analogues with very good radiological effect. We use this regimen as a standard in patients with MINEN of gastrointestinal tract with dominant neuroendocrine component, even with no somatostatin receptors staining available. Now the patient is almost a year after being diagnosed with a tumor, after completion of 6 cycles of adjuvant chemotherapy (carboplatin + etoposide) in combination with biological therapy, the long-acting somatostatin analogues. The patient is in good clinical condition, and while a recently performed PET/MRI scan revealed a hepatic lesion and hilar lymphadenopathy in full regression, there was a spread of small peritoneal and pleural metastases, with a solitary metastasis in Th9. The condition was evaluated as disease progression stage according to RECIST criteria, the patient remains in the follow-up care, and it is now 13 months after surgery (Figs. , , and ).
A 17-year-old female patient reported to the Department of Periodontology and Oral Implantology with a chief complaint of severe mobility of her upper and lower front teeth. She had started noticing the mobility since the past two years and it was noticed that the mobility had increased progressively over the years. This was the first dental visit for the patient and the past medical and social histories were noncontributory. There was no relevant familial history of the disease and hence the patient was considered to be a first generation sufferer. The clinical intraoral examination revealed the presence of normal color and morphology of the crowns of all teeth. Stable gingival and periodontal health was confirmed by a thorough intraoral assessment, which demonstrated a clinical absence of gingival inflammatory changes, absence of bleeding on probing, as well as, loss of clinical attachment. However, assessment of mobility revealed grade III mobility of the upper and lower anterior teeth and all the four first premolars. The second premolar and molar teeth remained immobile. The patient demonstrated good oral hygiene and there were no other relevant intraoral findings noted. An orthopantamogram of the patient revealed rudimentary and nearly absent root structures in relation to the upper and lower anterior teeth, the premolars, and molars []. The intraoral periapical radiographs revealed a defective morphology with a strikingly flared, inverted, crescent-shaped appearance of the roots of the mandibular first molar teeth []. There was a generalized total obliteration of the pulp tissues within the pulp chambers and root canals [Figures –]. An interesting feature was the absence of periapical radiolucencies and periradicular lesions. Also, there were no associated osseous changes or bone pathologies detected. Based on the clinical and radiographic findings of the disease, the diagnosis was established as dentin dysplasia type I. However, the findings of this case did not fit into any subtypes of the existing classification systems of the disease. The observations of an absence of familial hereditary pattern, the absence of periradicular radiolucent lesions and osseous pathologies, and the variant morphological defects of the molar roots were diverse from the classical findings of the various subtypes of dentin dysplasia type I reported to date.\nThe treatment plan was formulated based on the age of the patient, the expected esthetic outcome, and the long-term prognosis of the therapy. The patient had completed the craniofacial growth phase and was highly concerned about the esthetic outcome of the treatment. As the patient was 17 years old at the time of the visit to the dental office, a major period of growth and growth spurts was completed. Hence, no further changes in the craniofacial structure that might cause gross future occlusal derangement were deemed to occur and no further growth investigations were carried out. Taking all these multiple factors into consideration, an implant-based oral rehabilitation was finalized as the treatment for this patient. The treatment plan was explained in detail to the patient and her family and a written informed consent was obtained from the patient as well as the guardian of the patient.\nThe surgical sites extending bilaterally between the first premolar regions in the maxilla and mandible were anesthetized using local administration of 2% lignocaine hydrochloride (LOX, Neon Laboratories Ltd., Mumbai, India) with 1: 200,000 adrenaline. All the Grade III mobile teeth were extracted and the osteotomy site preparations were carried out under copious irrigation with sterile isotonic saline solution. After establishing proper depth and direction, a total of 10 self-threaded titanium implant fixtures, including six in the maxillary and four in the mandibular regions were placed []. Temporary maxillary and mandibular removable partial denture prostheses were delivered []. Postoperative care included a prescription of 8 mg of betamethasone and 2 g/ day dosage of amoxicillin, for 10 days. The patient was advised to rinse with warm saline solution for the first two weeks to promote flap healing without disturbing the surgical sites. The patient was also instructed to use 0.12% chlorhexidine gluconate mouthwash (Peridex, Zila Pharmaceuticals, Phoenix, AZ, USA), twice daily, to facilitate plaque control. The surgical sites were checked every two weeks for a period of two months. Following six months of healing, the second surgical procedure to uncover the implants was performed, the abutments were placed, and the final prostheses were delivered [Figures and ]. The patient and family members expressed a positive response toward the superior esthetic and functional outcome of the treatment. The patient has been put on a strict maintenance program with a first year recall interval of three months, followed by a review once in six months to one year, based on the home care performance and assessment of the intraoral health status.
A 13-year-old boy was referred to the department of oral and maxillofacial surgery in a tertiary referral centre and presented with a right facial cellulitis, and a hard mass was palpable in his right maxilla The patient reported that the symptoms had begun 1 week previously but that he had noticed a mass in the right maxillary molar area 6 months previously. The infection was treated with empirical intravenous antibiotic therapy. Extraoral examination revealed a hard swelling over the right maxilla, with an associated intraoral calcified mass and missing molar teeth. There was no associated sensory nerve deficit. Plain radiographs and computed tomography demonstrated an extensive calcified lesion in the right maxilla, extending to the infraorbital rim (Figures and ). An incisional biopsy was taken and histopathological examination suggested the presence of odontoma or fibrous dysplasia.\nSurgical removal of the lesion was planned using a transoral approach under general anaesthesia. A mucoperiosteal flap was raised and the tumour was identified (). The lesion was found to be eroding into the infraorbital rim. An upper lip split incision was made to facilitate complete removal of the tumour and maintain the integrity of the orbital floor and allow safe dissection and release of the infraorbital nerve. The tumour was enucleated intact using an osteotome and a periosteal elevator, while maintaining continuity of the infraorbital rim and orbital floor. The ipsilateral buccal fat pad was mobilised and advanced to repair the maxillary defect, following tumour removal.\nHistopathological examination confirmed the diagnosis of complex odontoma. The patient made an uneventful recovery and had no sensory nerve deficit. The extraoral incision has healed well with minimal scarring; the patient is pleased with the appearance and healing of his extraoral incision (). Prosthetic impressions were taken to facilitate construction of a maxillary obturator. To date, the patient has been followed up for a period of 36 months. There has been no clinical or radiographic evidence of recurrence. He will be considered for autogenous bone grafting and placement of osseointegrated dental implants and an overlying fixed prosthesis in the future.
Our patient was a 68-year-old Australian Caucasian man who had a right above-the-knee amputation following a motorcycle accident in 1959, when he was 19 years of age. He initially perceived a painless phantom that dissipated soon after amputation. He did not have painful neuromata, but experienced paroxysmal shock-like stump pain two to three times yearly that would settle within 24 hours. We first assessed our patient's phantom pain in a questionnaire study in 2005 [], three years after the onset of his phantom pain, and more recently via an interview and clinical examination in 2009 conducted to investigate his late-onset phantom pain. The patient provided written, informed consent for the publication of this case report, and both studies were approved by local and hospital ethics committees.\nIn 2002, our patient was diagnosed with moderate to poorly differentiated adenocarcinoma which had infiltrated through the full thickness of the bowel wall and into one regional lymph node. He promptly underwent right hemicolectomy. Fifteen days later he was diagnosed with pulmonary emboli and secondary pneumonia. Thrombotic occlusion had developed in the right superficial femoral vein approximately 5 cm distal to the long saphenous junction and extending proximally to the level of the distal common femoral artery. The patient was advised against prosthesis use until the blood clot cleared approximately four months after the initial surgery.\nOur patient completed a six-month course of chemotherapy with leucovorin 38 mg and 5-fluorouracil (5-FU) 800 mg, which were administered with domperidone 10 mg and dexamethasone 4 mg to 8 mg. There was no prophylactic administration of vitamin E before chemotherapy. Little note was made of the effect that these agents had on our patient's stump and phantom pain, except that he was advised to bandage his swollen stump during the third cycle and he reported nerve pain in the stump by the sixth cycle. The possible cause of stump swelling was not recorded.\nOur patient noted the presence of a painful phantom foot, telescoped near the stump, and a definite increase in stump pain and hyperalgesia, which was particularly pronounced after prosthesis use, which began during the course of chemotherapy treatment. He presently takes carbamazepine (200 mg daily) and tramadol (200 mg daily) to manage his pain. Our patient is unable to differentiate between his stump and phantom pain, as they both occur within the same region, often simultaneously, and are characterized by the same sensations. Deep manipulation of the stump (with fingers) now triggers shock-type pains; however, providing even pressure with the prosthesis helps to alleviate pain, indicating the absence of any continuing irritation of the stump. The phantom sometimes feels cold, but never hot or burning.\nOur patient's pain is exacerbated by sitting, increased levels of activity, heavy lifting, hot weather, sweating, and stress. He has never noticed any increase or change in pain in relation to toileting, having a full bladder or bowel, or genital stimulation. He finds that walking and keeping occupied reduces his pain. On the basis of the McGill Pain Questionnaire [], he described his pain as jumping, tingling, aching, intense, numb, cold, and nagging (see Table for pain intensity and unpleasantness ratings).\nOn the Leeds assessment of neuropathic symptoms and signs pain scale [], our patient scored 7 out of 16, responding positively to "having pain that feels like strange sensations in the skin characterised as pricking, tingling, or pins and needles" and "having pain that comes on suddenly in bursts for no apparent reason when he is still."\nThe patient did not exhibit allodynia on the stump when lightly stroked with cotton wool, but exhibited hyperalgesia and a reduced pinprick threshold in the stump region (pinprick was rated at 45 out of 100 on the Visual Analogue Scale (VAS), where a score of 0 is not painful and a score of 100 is the worst possible pain), compared to the arm (8 out of 100) and the lower shin of the intact leg (10 out of 100). The patient's perception threshold to Von Frey filaments was the same between his arm, stump, equivalent region on the intact leg, and lower shin on the intact leg at a pressure of 2.05 g, indicating diminished protective sensation in all regions. In the stump, 15.00 g was perceived as just painful (VAS score 15 out of 100). When tested for temporal summation (10 applications of the 15 g filament at a frequency of 1 second), the patient experienced marked wind-up, with an increase in pain intensity to 56 out of 100. Given the reduced protective sensations noted above, such a pattern may be considered suggestive of hyperpathia.
A 40-year-old male presented to our office in 2017 complaining of chronic left knee pain. This was his first visit to this office and was establishing care after moving to the area. The patient's electronic medical record was obtained from an outside institution which indicated a past medical history of diabetes, hyperlipidemia, and hereditary multiple exostosis. The patient disclosed that he had multiple osteochondroma removal surgeries which included his left knee, lumbar spine, and left foot at an outside institution. In addition, he had a left hip arthroplasty with refractory numbness/tingling of the leg. The patient stated he had left knee pain for years until he had an osteochondroma removed in his left distal femur in 2016 which seemed to help for 6 months. He stated the pain returned at 7/10 and is worse with movements. He had limited range of motion with 70° of flexion, negative pain with varus and valgus movement, and negative secondary tests. Baseline X-rays were ordered for this patient's knee ().\nThere were osteoarthritic changes with significant osteochondroma formation; however, arthroplasty was not recommended at that time because of the patient's young age and his left lower extremity being distally neurovascular intact. The patient was adamant about having his knee replaced and was referred to a specialist at an outside institution where an arthroplasty of the left knee was performed. The patient returned 3 months later with continued limited active and passive range of motion (<90° flexion). Follow-up radiographs were ordered showing good alignment of a Smith & Nephew posterior stabilized prosthesis without subsidence (). It was decided to perform manipulation under anesthesia followed by 6 weeks of physical therapy which improved his range of motion to 110° flexion.\nThe patient continues to have limited range of motion of his left knee with flexion to 90°. The large posteriorly projecting osteochondroma of his left tibia continues to be asymptomatic and painless, and therefore, it was decided not to perform surgical interventions at this time. Performing an arthroplasty helped relieve the patient's pain, however, did not result in significant improvement of active and passive range of motion of the joint. Overall, the patient is satisfied with his knee replacement but still has refractory numbness/tingling of his distal left lower extremity from his prior hip replacement. The patient has chronic pain elsewhere, which is being closely monitored (Figures and ).
A 34-year-old man was transferred to our institution four days after a quad bike accident in rural Greece. Among other minor injuries, the patient had suffered a small wound posterior to the tragus of his left ear (). Primary closure of the wound was performed at a local infirmary; no radiological investigations had been performed at the time. Ninety-six hours later the patient presented at our hospital with obvious signs of left sided peripheral facial paralysis (). The aforementioned wound was slightly painful on palpation but appeared inconspicuous otherwise. When questioned about the circumstances of the accident, the patient explained that one wheel of the quad bike had become detached at full speed, catapulting him into low growing brushwood. According to the patient, he had noted (but quickly dismissed) a drooping of his left corner of the mouth shortly after the accident; the patient stated to be free of other symptoms and to be otherwise healthy. (For a details systematic facial palsy assessment, see []) Upon admission at our hospital, a CT scan of the head was performed to rule out intracranial hemorrhage and fractures of the skull. These images raised suspicion of a foreign body deep to the left auricular concha (). No other pathologies were found; in particular, there was no indication of a fracture of the temporal bone. The further non-surgical management included concussion monitoring and wound care. An MRI scan was subsequently ordered to assess the integrity of the left facial nerve and its relation to the aforementioned foreign body. The MRI scan finally confirmed the presence of a cylindrical foreign body with surrounding edema in the immediate vicinity of the temporal styloid process and the course of the left facial nerve ().\nOver the period of four days, some recovery of facial nerve function was noted clinically; however, facial expression remained strikingly asymmetrical. Regular moisturization of the left eye was necessary due to persistent lagophthalmos (). There was no evidence of facial nerve discontinuity. Written informed consent was obtained for exploration of the auricular lesion and foreign body removal. Superficial wound dissection revealed a piece of wood measuring 30 × 3 mm; it was readily extracted using a pair of forceps (). A second CT scan of the head was performed thereafter to verify complete foreign body removal. Facial nerve function presently improved and the patient was discharged home two days postoperatively. When the patient was seen again six weeks later, he was back at work and free of complaints with full recovery of facial nerve function.
A 35-year-old female presented with an asymmetric, voluminous tumor on the right side of her abdomen, which had gradually grown during the past one year. The patient had three children and had no family history of neoplasia. She was not addicted to tobacco and alcohol. She had a history of surgical excision of a solid tumor of size about 3 cm × 5 cm, located in the right hypochondrium. The surgery was done two years back. She had biopsy report of spindle cell neoplasm, but no slides or blocks were available for review at our institute. The surgery was not followed by radiotherapy or chemotherapy.\nPhysical examination on admission revealed a tumor of size around 20 cm × 20 cm in the right side of abdomen, extending to the opposite side beyond the umbilicus (). The tumor was fixed to the overlying skin and the underlying muscle. The skin appeared reddish with vascular engorgement, but there was no ulceration. It was not accompanied with any regional lymphadenopathy.\nComputed tomography (CT scan) depicted a large lobulated heterogeneously contrast enhancing soft tissue mass on the right side of anterior abdominal wall, extending from the lower chest wall downwards up to right side of pelvis, inseparable from right rectus, external oblique, and internal oblique muscles (). There was no evidence of metastases to distant organs on imaging. Fine needle aspiration cytology of the lesion showed plump spindle cells in clusters and small groups, suggesting recurrent spindle cell neoplasm.\nShe was subjected to surgery which consisted of a wide resection of the tumor along with the surrounding muscular layer and peritoneum. The resection exceeded the macroscopic limits of the tumor by 4 to 5 cm in order to accomplish a tumor-free margin. The defect was reconstructed with omentum and prolene mesh. The skin defect was covered with rotational skin flaps and split skin grafting (Figures and ).\nThe resected tumor, measuring 19 × 19 × 5 cm, was yellowish white in colour without central necrosis and hemorrhage on its cut surface. Routine histological examination with hematoxylin and eosin revealed that the tumor is comprised of spindle shaped cells arranged in sheets and fasciculated bundles with cigar shaped nuclei (). Occasionally tumor giant cells were found. Mitotic figures were quite frequent, around 8–10 per 10 high-power fields. All the margins were free. In immunohistochemistry (IHC), the tumor cells were positive for smooth muscle actin (SMA) and CD34 but negative for desmin and S-100 (Figures and ). The Ki-67 index was 10–15%. The histologic grade of the tumor (grading of the French Federation of Cancer Centers—FFCC) was Grade II (2 + 2 + 0). With these findings, the diagnosis of subcutaneous leiomyosarcoma was confirmed.\nPostoperative period was uneventful (). Taking into consideration the large tumor size, high mitotic rate, and recurrent setting, postoperative radiotherapy was given. The oncological treatment has now been in course for 4 months, with no clinical sign of local recurrence.
A 43 year old male, Muslim Yoruba farmer residing in Osun State of Nigeria presented at the outpatient unit of Osun State Hospital, Asubiaro Osogbo, with complaints of toothache in the lower left jaw of 2 weeks duration as well as cough, dysphagia, left earache and swelling of the neck and face of 5 day duration. The Patient was in his normal state of health until about 2 weeks before presentation when he developed toothache in the lower left quadrant of the jaw. He was advised by his friend to apply acidic chemical used to recharge car batteries to rinse the mouth. On first application of the acidic chemical, he experienced burning sensation and pain making him to immediately rinse with water, which gave him some relief. Some minutes later he reapplied the acidic chemical and held it in the mouth for some minutes “so as to derive maximum benefit” as advised by his friend which he later rinsed off with water but without much relief from the burning sensation and pain. Twelve days later, his medical condition worsened as the pain persisted and increased. He also noticed a swelling around the jaw which progressively increased in size to involve the face and neck. He used different analgesics such as paracetamol and ibuprofen during this period without any improvement but rather worsening of his condition leading to progressive difficulty in swallowing, breathing and talking which made him to present at the out patient department of the State hospital.\nAt presentation, he was a toxic looking young man, conscious, alert well oriented in time place and person. He was having difficulty in talking and moderately dyspneic as evidenced by alar flaring. There was marked diffuse facial swelling extending from the temporal region downwards involving the face to the cervical region bilateally. Swelling was warm to touch, tender and firm in consistency. There was pus discharge from the mouth with marked trismus which prevented thorough intraoral examination. Medical history was significant for regular alcohol intake and smoking. An assessment of Ludwig’s angina with impending respiratory obstruction secondary to topical use of chemical substance to treat an odontogenic infection was made.\nThe Patient was hospitalized and commenced on intravenous fluid therapy and intravenous antibiotics (cefuroxime – 1gram daily and metronidazole – 1.5grams daily). Laboratory investigations (packed cell volume, electrolyte and urea, random blood glucose, fasting blood glucose, microbiology culture and sensitivity testing) were ordered for. He was immeditely taken to the theatre and under local anaesthesia and parenteral analgesia, incision and drainage (I&D) of the submandibular, submental, sublingual, infratemporal and cervical spaces were carried out. About 300mls of pus was drained from the extra oral incision and intraoral discharge. Rubber tubing drains were inserted and thick gauze dressing placed. He was continued on intravenous antibiotics and haematinics.\nThe result of the laboratory investigations were as follows – packed cell volume-33%, microbiology culture and sensitivity testing yielded growth of Streptococcus pyogenes, sensitive to Cefuroxime, Refloxacin, Cimofloxacin and Amoxycillin, results of other investigations were within normal limits. He was also commenced on active jaw exercise and hourly warm saline mouth bath. There was regular change of dressing as copious amount of pus continued to be discharged from the inserted drains with a sustained improvement of his general condition and by the third post I&D day, the drains were removed. By the fourth post I&D day, necrosis of the skin in parts of the cervical region were noted which later broke down to form 3 oval shaped defects with necrotic tissue at the base (two on the left and one on the right side of the neck). The two defects on the left were about 4cm and 5cm in their widest diameters respectively while the one on the right was about 5cm in widest diameter. Debridement and daily dressing with dilute eusol was commenced. By the eighth day post I&D, the patient could tolerate oral intake well, intravenous antibiotics were discontinued and replaced with oral antibiotics. By the seventeenth day post I&D, the patient was discharged to the clinic to continue active jaw exercise and daily dressing of the neck wound which was begining to look healthy.\nWhen the wound was covered with healthy granulation tissue and no sign of infection noticed, the defects were closed under local anaesthesia using a local flap for the defect on the right, a local flap for the 5cm wide defect on the left and a full thickness skin graft harvested from the left supraclavicular region to close the second defect on the left.\nFollowing active jaw exercise (with the use of wooden spatula) and resolution of the infection, proper intraoral examination and radiological investigation was done which revealed the source of infection as a non vital lower right canine tooth which was subsequently extracted. The wound eventually healed totally after a couple of dressings.
A 33-year-old woman was sent to the general surgery department due to upper-right abdominal pain for 2 weeks. A physical examination showed that the skin color and sclera of the patient were normal; however, a mass could be felt in the upper-right abdomen. The patient had received no relevant interventions prior. Laboratory findings revealed that the liver transaminase of the patient was slightly increased: alanine aminotransferase was 120 U/L, aspartate aminotransferase was 109 U/L, and r-glutamyl transpeptidase was 180 U/L, with the levels of tumor markers and serum total bilirubin (7 μmol/L) within the normal limits. CT found a liquid dark area of about 4 × 3 × 3 cm in the theoretical location of the pancreatic segment of the common bile duct with no enhancement found in the cystic wall by the enhanced CT scan. An aspherical expansion of the cystic dilatation was detected by both MRI and magnetic resonance cholangiopancreatography (MRCP). Therefore, the patient was diagnosed with a choledochal cyst based on the results of the radiological images preoperatively ().\nSubsequently, the patient signed the informed consent and took a laparoscopic choledochal cyst resection. Under the laparoscopy, the gallbladder was found extremely dropsical with an unclear structure in the Calot's triangle. The gallbladder was resected as a routine operation, and the specimen was examined for pathology analysis. The stump of the cystic duct was lifted and separated medially along the cystic duct. The isolated cystic dilatation was found in the middle part of the cystic duct, and the caudal portion was found behind the head of the pancreas and converged into the common bile duct at an acute angle and low insertion. The surgery was converted to a laparotomy for the unclear structure and the possibility of anatomic variation of the bile duct. Because the drainage to the bile duct was through a narrow neck and the extrahepatic bile duct was not cystic, the cystic duct was eventually removed (). Different from the preoperative diagnosis, the patient was diagnosed with a CDC based on the postoperative pathological findings (). The abdominal pain of the patient disappeared with normal serum aminotransferase and transpeptidase. The patient was discharged a week after surgery and was asymptomatic a month later.
A 41-year-old non-smoker obese female patient was examined at the Department of Neurology and the Center for rehabilitation. Relevant history of the patient is summarized on a timeline in Fig. . She showed an onset of neuromuscular disorders during early childhood with a delay in motor and written language development. She had a hard time to complete physical exercise at school and always finished last. There was nothing really alarming in terms of muscle disorder until her first pregnancy at the age of 33 during which she experienced shortness of breath (dyspnea) at the 7 months of gestation and showed signs of weakness of lower limbs afterwards. At the age of 34 she showed worsening of breath symptoms, suffered from sleep apnea and started using a mechanical ventilation machine. Alarming symptoms of skeletal muscle disorders occurred immediately after delivery with progressive but rapidly incapacitating weakness of lower limbs. This worsened during the 3 following years, a period during which she first started being unable to get up by herself and then was unable to climb stairs. At the age of 37, she started to use a cane, then a walker for her daily walking needs. At the age of 39, she started using a wheelchair to move outdoors. Despite these signs of progressive muscle weakness, she never had a thorough neuromuscular investigation. It was wrongly thought that her health problems, in general, was mainly related to being overweight. At the age of 41, she had four episodes of lower limb paralysis during which she was completely unable to move her legs and support her weight. She did not seek medical consultation for the first three episodes. For the fourth, she was admitted to the emergency room (ER) and first referred to the Department of Neurology and then to the Center for Rehabilitation.\nThe family history showed that her mother died at the age of 66 from a heart attack associated with non-compaction cardiomyopathy. There is nothing remarkable in terms of muscle disorders in her father, brother, and sister. However, her only daughter, now at the age of 11, shows signs of muscle disorders with congenital muscular torticollis, excessive growing pains as well as underdeveloped muscles in half of the body.\nPhysical examinations conducted following her admission to the ER at the age of 41 showed normal tone/bulk of the arm muscles. However, muscles in shoulders and upper and lower limbs showed bilateral weakness. Deltoids, biceps and triceps showed moderate weakness with MRC scale of 3/5. Fine finger movements were intact. There was no pronator drift. In the lower limbs, all muscles examined showed the same severity of weakness (2/5) except quadriceps which showed mild weakness (4/5). Hip flexors were extremely weak (1/5); hip abductors and adductors were mildly weak (4/5). Knee extensors and flexors were moderately weak (3/5). Dorsiflexion of feet was severely weak (2/5). Plantar flexors were severely weak (2/5). Deep tendon reflexes were 2+ in the arms, absent in the patella and Achilles. The toes were down-going. No sensory deficit was observed. No sign of dysphagia or involvement of ocular muscles was observed.\nAdditional physical examination conducted during the 2-year follow-up (at the age of 43) showed no worsening of muscle strength. However, this brought new information about the weakness of other muscles, notably in the shoulders, with extremely weak abductors and flexors (1/5). The patient showed decreased perception of vibration in the lower limbs. She reported that she experienced occasional dysphagia.\nElectrophysiological exams showed normal nerve conduction velocities for upper and lower limbs, with all SNAP and CMAP amplitudes in the range of normal values. However, needle EMG revealed a tendency of myotonic potentials, generated by needle insertion. Right deltoids, biceps and ulnar-innerved first dorsal interosseus muscles showed myotonic potentials as well. The right tibialis anterior showed 2+ polyphasic motor unit potentials with myotonic potentials. The right medial gastrocnemius, vastus medialis and vastus lateralis showed myotonic tendencies as well.\nExamination of muscle biopsy of left quadriceps showed features of an end-stage process, consistent with a severe, chronic myopathy. There are scattered clusters of viable muscle fibers which showed myopathic features in the form of a marked variation in fiber size and numerous internal nuclei. In addition, there are scattered fibers with abundant intrasarcoplasmic vacuoles (Fig. a, b). Given we could not clearly observe the presence of hyaline bodies on H&E staining we decided to proceed with p62 immunostaining, a well-known technique for revealing the presence of inclusion bodies []. Immuno-histochemical detection of p62 was performed on a Leica Bond III automated stainer. Following digestion in a low pH citrate solution, sections were incubated in a primary mouse monoclonal anti-p62 antibody, diluted 1:50 (BD Transduction Laboratories Catalog Number 610833). Detection of bound antibody was achieved using the Leica Bond Polymer Refine Detection kit, comprising the secondary antibody, the substrate chromogen DAB (3,3′-Diaminobenzidine tetrahydrochloride hydrate) and the Hematoxylin counterstaining solutions. Immunostaining for p62 revealed, in a proportion of the surviving muscle fibers, diffusely distributed, small intermyofibrillar dots or, more commonly, larger central or eccentric sarcoplasmic inclusion bodies (Fig. c, d). Examination of heart function using Holter ECG monitor carried out following her admission to the ER showed no clinically significant implication of cardiac involvement. However, the patient complained about repeated episodes of oppressive chest pain during the following years. Additional examination conducted during the 2-year follow-up showed signs of cardiac involvement with bradycardia of 58 beats per minute.\nRegarding respiratory involvement, the patient suffered from dyspnea since the age of 34. Examination of lung function conducted following her admission to ER and during the follow-ups showed worsening of her respiratory condition. Spirometry test conducted at the age of 44 showed a very weak pulmonary function, with forced vital capacity (FVC) of 27% and forced expiratory volume in a second (FEV1) of 29% of normal values.\nThe blood samples were collected for whole-exome sequencing (WES) to detect mutations potentially involved in the phenotype of neuromuscular disorders observed for this patient. The genomic DNA was extracted from whole blood and subsequently subjected to whole-exome DNA library construction using the Ion AmpliSeqTM Exome RDY panel (Thermo Fisher Scientific) essentially as described in the manufacturer’s protocol, with barcode incorporation. For the sequencing, samples were loaded on an Ion HI-Q PI Chip v3 and placed onto the Ion Proton instrument (Thermo Fisher Scientific) together with an Ion PI HI-Q sequencing 200 Kit (Thermo Fisher Scientific) and sequenced for 520 cycles according to the manual (See Additional file : Table S1 for parameters). All candidate mutations found by WES were validated by direct Sanger sequencing (See Additional file : Figure S1 for the filtering process). DNA sequences were obtained from the University of California Santa Cruz (UCSC) Genome Browser. Predesigned primers were directly purchased from Thermo Fisher Scientific (See Additional file : Tables S2 for details). Amplicons were sent to Genewiz () for Sanger sequencing.\nWhole exome sequencing showed that a novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg) in the MYH7 gene is a missense single nucleotide variant possibly linked to the clinical findings, found in the DNA of the patient as heterozygous (Fig. ). The novel variant has been submitted to ClinVar database; with the assigned accession number SCV000804311. Two other candidate mutations were identified in the DNA of this patient, namely NM_003085.4:c.368C > A (p.Pro123His) in the SNCB gene and NM_001001557.3:c.746C > A (p.Ala249Glu) in the GDF6 gene (Additional file : Table S3). However, both mutations were discarded after filtering only genes involved in neuromuscular functions that potentially cause clinical features of muscle myopathy observed in this patient (Additional file : Figure S1). Regarding the novel variant NM_000257.3: c.1370 T > G (p.Ile457Arg), bioinformatics analyses showed that nucleotide T coding at the position 1370 of the MYH7 gene is highly conserved across 44 vertebrate species (PhyloP at 1.76). Amino acid substitution from Isoleucine (I) to Arginine (R) at the position 457 of MyHCI suggested a high impact on protein structure (Grantham at 97). Results of analyses obtained from VarSome () suggested a classification of Likely pathogenic for this variant, with evidence of Pathogenic computational results coming from 8 various prediction software including DANN, GERP, dbNSFP.FATHMM, MetaLR, MetaSVM, MutationAssessor, MutationTaster and PROVEAN (vs no benign predictions). Also, the results of analyses using the recommendation of the ACMG and the AMP suggested the same classification of Likely pathogenic for this variant, with combined criteria of 2 moderate (PM1 and PM2) and 2 supporting (PP2 and PP3) [].
An eight-year-old boy was evaluated for severe headache and vomiting in the emergency room. These symptoms began 20 days before hospital admission and were not accompanied by fever or other complaints. During a previous outpatient visit for a progressive headache, a blood pressure of 160/100 in one arm was detected on physical examination and the patient was referred to a local hospital for control and investigation. In the emergency room, the patient evolved with abnormal mental status and agitation. A grade II of VI systolic ejection murmur on the left-upper sternal border was heard during the cardiovascular exam. The patient was transferred to the intensive care unit (ICU) at the same hospital.\nIn the ICU, the patient had one seizure episode, and was prescribed carbamazepine, and remained without more episodes. Intravenous calcium channel blocker and furosemide were prescribed for blood pressure control, but the patient started to present amaurosis, confusion, and left hemiparesis.\nThe main diagnosis was a hypertensive emergency with encephalopathy. Further investigation with brain computed tomography (CT) showed no signs of elevated intracranial pressure. Other imaging and laboratory exams including renal arteries Doppler ultrasound and cerebrospinal fluid analysis, and dosage of metanephrine and cortisol showed no abnormalities. A transthoracic echocardiogram could not visualize the aortic arch. A brain magnetic resonance imaging (MRI) revealed bilateral hyperintense signals in the subcortical and cortical areas of the occipital and parietal lobes. This finding was consistent with a posterior encephalopathy (Figure ).\nDespite the improvement of the hemiparesis and clinical stability, reduced visual acuity remained and the patient was discharged to the pediatrics wards for further investigation of the case, after one week in the ICU.\nThe next day, a significant difference in blood pressure was observed between the upper (systolic pressure of 172 and diastolic pressure of 106 mmHg) and lower limbs (systolic pressure of 99 and diastolic pressure of 60 mmHg). An MRI angiography was requested, which diagnosed coarctation of the aorta as the causative factor for the hypertensive emergency and secondary encephalopathy.\nThe coarctation of the aorta was corrected with transcatheter stent placement, and the patient was discharged afterward.\nDuring follow-up, a sequel loss of peripheral visual field was described by ophthalmology as tubular vision (Figure ) without fundoscopy alterations. The anticonvulsant carbamazepine was maintained for seizure control, and the patient achieved normal blood pressure with beta-blockers. An MRI one year later shows reduced focal areas of bilateral hyperintense signal in the parieto-occipital white matter (Figure ). The improvement of the clinical and imaging findings supported the diagnosis of a posterior reversible encephalopathy (PRES).
A 40-year-old woman with 2 previous normal deliveries presented to surgeons at King's Mill Hospital in April 2013 with a 3-year history of intermittently discharging persistent right groin sinus. The discharge was purulent in nature; she often felt generally unwell. The patient had a past medical history of laparotomy for ruptured pyosalpinx in 2000. Subsequently the patient was seen in gynecology clinic with a history of recurrent lower abdominal pain in 2008 and 2011. Initially the patient was treated with antibiotics and she was given the option of pelvic clearance. The patient elected to have conservative management.\nOn examination by the surgeons the patient was noted to have a discharging sinus in the right groin with the working diagnosis of hidradenitis. A CT sinogram was organized. The sinogram demonstrated a sinus extending from the right groin crease to the right adnexa with the contrast tracking behind the uterus and the upper vagina. In view of these findings the patient was referred to the gynecology team for further assessment.\nAt gynecological assessment the patient reported a history of right iliac fossa pain and constant discharge from the right groin sinus. On abdominal examination the patient was noted to have a midline laparotomy scar and discharging right groin sinus. On pelvic examination there was reduced mobility of the pelvic organs and thickening noted in the right adnexal region. After liaising with the surgeons a plan was made for laparotomy, right salpingo oophorectomy, and excision of fistulous tract. The patient was informed regarding the risks to adjacent vascular structures due to the proximity of the tract to the major blood vessels.\nThe patient had a laparotomy under the joint care of the gynecologists and surgeons on May 27, 2014. At laparotomy the patient was noted to have a normal left tube and ovary which was attached to left pelvic side wall. The uterus was normal and she had a normal right ovary and right pyosalpinx. The fistulous tract was communicating to the cornual end of right fallopian tube posterior to the round ligament; it then followed the path of the round ligament, opening at the level of inguinal ring in the right groin.\nRight salpingectomy was performed and the right round ligament was divided near the right cornual end of the uterus; a right groin fistulous tract of 3 cm was excised. Histopathological examination demonstrated pyosalpinx with features of chronic and acute inflammation with microabscesses and fistulous tract showing inflammatory granulation tissue without evidence of malignancy. The patient was discharged home on the 3rd postoperative day.\nThe patient was readmitted on the 6th postoperative day with headache. She was seen by the anesthetists who treated her with a blood patch for postdural headache. The patient made a good postoperative recovery followed by the blood patch and she was discharged home on the 8th postoperative day. The patient was reviewed in the clinic at 8 weeks postoperatively. She had made a good recovery with completely healed right groin wound and laparotomy scar.
A 63-year-old Chinese woman presented to our department after the discovery of a mass in the hepatic portal entrance on a CT scan at a local hospital.\nThe patient initially visited the local hospital with complaints of chronic intermittent abdominal discomfort. A CT scan of the gallbladder, liver, pancreas, and spleen was consequently performed. A mass in the hepatic portal entrance was noted and the case was referred to our hospital for further investigation. Physical examination was unremarkable, preliminary blood tests and tumor markers were within normal limits. The patient had no known liver diseases. For additional work-up, the patient underwent CT with contrast, MRI, and CTA at our department.\nHistopathological report concluded that the mass resected from the liver lobe consisted of thyroid and liver tissue as portrayed in Figure -. Immunohistochemical studies for thyroid transcription factor 1 and thyroglobulin were both positive as shown in Figure and .\nA non-enhanced CT scan revealed a well-circumscribed, heterogeneous and high attenuation mass in comparison to the liver parenchyma with calcification. A contrast- enhanced CT scan of the abdomen and pelvic area revealed a group of high density (compared to the surrounding liver parenchyma) nodules with exhibition of well-defined borders in the hepatic entrance with calcification and an expansive size of 43 mm × 38 mm. The lesion was greatly enhanced in the arterial phase and there was prolonged enhancement in the venous phase with decreasing attenuation, as shown in Figure .\nOn CTA, in the hepatic entrance, a mass with low attenuation alongside calcification in the middle was observed. In the arterial phase, the mass was supplied by a blood vessel branching from the right hepatic artery as shown in Figure .\nMRI of the upper abdomen demonstrated subsequent enhancement in the hepatic entrance with mixed signal intensity in the observed mass on both T1 and T2 weighted images. The mass was demarcated from the surface of the liver and there was diminished signal intensity in the arterial phase and in the venous phase, as shown in Figure .
In January 2010, a 51-year-old female presented to a rural hospital with a one month history of left lower and upper abdominal quadrant pain. The patient was admitted to the hospital with the preliminary diagnosis of pancreatitis. After admission, the patient developed gross hematuria. It was discovered that the patient had a past medical history significant for a stab injury in 1998 to her left kidney with involvement of the left renal artery. At that time, the patient underwent a transabdominal/transperitoneal partial nephrectomy. Due to the patient's complicated past medical history and her present gross hematuria, the decision was made to transfer the patient to our service for a higher level of care.\nAfter admission to our hospital, a transurethral catheter was placed, which showed gross hematuria. Upon further evaluation, the decision was made to perform a cystoscopy and evacuation of blood clots. The urethrocystoscopy showed no gross pathology in the urethra and bladder. The left and right orifice were normal in size, place, and configuration. In order to locate the source of bleeding, bilateral ureteroscopy was performed. The right ureter and renal pelvis were found to be unremarkable. The left ureter also appeared to be free of pathology. However, at approximately the level of the ureteropelvic junction, a papillary mass was noted that appeared to extend into the left renal pelvis. A biopsy was unable to be obtained due to significant bleeding. After the blood clots were evacuated, and taking into account the past history of hematuria with a small, atretic kidney, it was deemed necessary to pass a stent into the left ureteral pelvis. The patient tolerated the procedure well. The results of the cystoscopy were discussed with the patient, and a nephrectomy was being considered to remove the tumor. However, after cystoscopy the patient developed an ileus. She was also very malnourished. Once her bleeding and ileus resolved, the decision was made to allow her to be discharged home to recuperate in order to better tolerate the nephrectomy. The patient was for a followup appointment two weeks later to evaluate for a nephrectomy. The patient did not show up for her appointment and was lost to followup.\nSeven months later, the patient was admitted to the hospital complaining of progressive left flank and left-sided abdominal pain that gradually increased in intensity for four days. It was accompanied by nausea and vomiting, dysuria, and frequency. The patient stated that she had no bowel movement for 3 weeks. She also complained of poor appetite and weight loss, but did not know the amount of the weight loss over the past few months.\nOn physical exam, the patient was found to be in moderate distress. She was alert and oriented with mild cachexia. The abdomen was soft, with moderate tenderness to palpation of the left side with the pain radiating to the left groin. The abdominal exam was negative for rebound tenderness and positive for bowel sounds. A surgical scar 3 inches in diameter was visible in midline. Two ventral hernias were also visible on the abdomen. The physical exam also showed costovertebral angle tenderness bilaterally, with marked tenderness on the left side. The patient also was found to be septic at that time.\nFor further evaluation of her newly diagnosed ileus, the patient underwent a gastrointestinal series with small bowel imaging. The proximal small bowel demonstrated intermittent mild dilatation. The obstructive point was not clearly identified. Contrast media extended through the jejunum and ileum without evidence of obstruction. The terminal ileum was normal in appearance (). Additionally, a colonoscopy was performed which revealed a stricture at the site of the splenic flexure. No intraluminal tumor was encountered. A standard computed tomography (CT) scan of the abdomen and pelvis with and without IV contrast was obtained. The CT scans showed some stranding and thickening of the soft tissues immediately below the lower pole of the left kidney. There was also some stranding immediately around the splenic flexure that began immediately below the tail of the pancreas. No soft tissue density was present to suggest a mass. There was no evidence of bowel obstruction, but the small bowel in the abdomen was mildly distended and filled with fluid possibly representing mild ileus. The nephroureteral stent was noted on the left side in stable position ().\nAt this point, the working diagnosis of a malignant tumor of the left-sided renal pelvis invading into the perirenal space with involvement of the descending colon and tail of the pancreas was made. Once the patient was stabilized, the left-sided ureteral stent was removed and the patient was optimized for an exploratory laparotomy. The patient underwent radical (en bloc) resection of the left kidney including partial colectomy, splenectomy, partial pancreatectomy, and partial small bowel resection. Intra-operatively, a large mass in her left upper quadrant was encountered that appeared to originate from the left kidney. The tumor was locally invasive, involving the very proximal jejunum, the splenic flexure of the colon, the left adrenal gland, and the distal aspect of the pancreas. This situation necessitated an en block resection. On gross pathology, the measurement of tumor size was not definite, but appeared to be 8 cm in maximal dimension. The specimen was partially necrotic and presumably obliterating the colon. The tumor did not involve the lumen of the colon or small intestine. Microscopic examination showed a poorly differentiated carcinoma of the colon invading into the kidney. Noteworthy, the tumor was only identifiable in the submucosal layer of the colon. Immunohistochemical stains for CK20 and CK7 were performed and found to be uniformly CK20 positive and CK7 negative (). These findings strongly evince a colonic origin for the entire tumor. At this point, the diagnosis of an extraluminal colonic carcinoma invading into the kidney was made. Tumor stage according to the TNM-system was pT4b, pN1b, pM1a. After adequate recovery from surgery, the patient will undergo adjuvant chemotherapy.

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