Cases reported "Sclerosis"

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1/36. Possible development of idiopathic sclerosing encapsulating peritonitis.

    We report a rare case of idiopathic sclerosing encapsulating peritonitis (SEP). During a laparotomy before undergoing a distal gastrectomy with Billroth II reconstruction for early gastric cancer, the patient was found to have a membranous encapsulation wrapping each small bowel loop, unlike peritoneal encapsulation or typical SEP. He had complained of persistent heartburn, distension and diarrhea for 2 months in the post-operative course. The second laparotomy, which was performed to improve prolonged transit, revealed typical SEP with a thick and fibrotic membrane that encased the small bowel entirely. Stripping of the sclerosing encasing membrane, separation of the adherent loops of the proximal small bowel, and Braun's anastomosis were performed. The patient complained of epigastric fullness and diarrhea after he was relieved from the complete bowel obstruction for 45 days post-operatively. trimebutine maleate was administrated 5 months after the second operation and this markedly improved his symptoms. This case might reflect the developmental process of idiopathic SEP. In addition, the use of a motility regulator may improve symptoms related to the abnormal intestinal motility by this disease.
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2/36. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.

    BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.
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3/36. Sclerosing encapsulating peritonitis: regional changes of peritoneum.

    Sclerosing encapsulating peritonitis (SEP) is characterized by the diffuse appearance of marked sclerotic thickening of the peritoneal membrane. We experienced a case with SEP accompanied by regional changes of peritoneum. A 37-year-old woman with end-stage renal failure was started on continuous ambulatory peritoneal dialysis in 1985 and was transferred to hemodialysis in 1997. She was admitted because of ileus in 1998 with SEP and died of septicemia. The diagnosis of SEP was confirmed via the autopsy. The root of the mesentery was retracted and shortened. Since the peritoneal change was marked in the regions with free margin of mesentery and was less apparent in the regions not adhered to mesentery, it is indicated that mechanical stress also contributes to the occurrence of SEP. Since calcification and ossification were only seen in a free margin of small bowel from mesentery, it is suggested that there is a close relationship between calcification and ossification. Since fibrosis invaded into the muscle layer, dysfunction of bowel movement as well as bowel obstruction contributed to the appearance of ileus. It is suggested that mechanical stress by the root of mesentery which is retracted and shortened also contributes to the appearance of SEP.
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4/36. Sclerosing hemangioma with lymph node metastasis.

    Sclerosing hemangioma (SH) of the lung is an uncommon type of tumor, which is composed of polygonal and cuboidal cells. This disease is generally regarded as benign but extremely rare cases with lymph node metastasis have been reported. We report a case of SH with a metastasis to the regional lymph nodes. A 19-year-old girl presented with a 2-year history of coughing. A chest X-ray and a CT scan indicated a large mass in the lower lobe. As a result, a left lower lobectomy with a dissection of the hilar and interlobar lymph nodes was performed. The tumor was a well-defined huge mass with partial adhesion to the mediastinal and parietal pleura. The dissected hilar, interlobar, and intrapulmonary lymph nodes demonstrated metastasis. Histologically, the primary and metastatic tumor consisted of polygonal and cuboidal cells. Both types of tumor cells were uniformly immunoreactive to the epithelial membrane antigen (EMA) and the thyroid transcription factor-1 (TTF-1). However, the cuboidal cells tested positive for pancytokeratin, whereas the polygonal cells tested consistently negative. Postoperatively, the patient received chemotherapy and no recurrence or metastasis 2 years after surgery was noted. Although a pulmonary SH is considered to be benign, this case highlights the need for the evaluation of lymph node metastasis.
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5/36. Sclerosing encapsulating peritonitis (SEP) as a delayed complication of continuous hyperthermic peritoneal perfusion (CHPP): report of a case.

    A 47-year-old woman underwent curative resection of advanced gastric cancer, followed by continuous hyperthermic peritoneal perfusion (CHPP). She was readmitted to our hospital 6 months after the operation with a diagnosis of postoperative adhesional ileus. An exploratory laparotomy revealed that the small intestine, which had normal serosa, was folded and enveloped in thickened peritoneum like a "cocoon," suggesting sclerosing encapsulating peritonitis (SEP). Because of tight adhesion in the ileocecal region, resection of the membrane was performed only in the feasible areas, followed by side-to-side anastomosis between the ileum and ascending colon. The patient has remained well for 15 months since this operation with no radiological signs or laboratory findings of recurrence. When small bowel obstruction does not show improvement with conservative treatment, and if the possibility of peritoneal cancer recurrence is excluded by thorough examinations, it is important to perform laparotomy early to resolve the symptoms of bowel obstruction and restore the patient's quality of life.
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6/36. Capillary changes in skeletal muscle of patients with Ullrich's disease with collagen VI deficiency.

    We examined the capillaries in muscle biopsy specimens from two patients with Ullrich's disease with collagen VI deficiency by light and electron microscopy. collagen VI plays an important role in platelet aggregation for binding von willebrand factor. Using immunohistochemistry, collagen VI was shown to be absent on capillaries from patients with Ullrich's disease, while von willebrand factor, collagen IV, and vascular endothelial growth factor were normally expressed. Electron microscopy revealed narrow lumens, large nuclei in endothelial cells, and fenestration of a capillary. The number of pinocytotic vesicles per unit endothelial cytoplasm was increased. The cytoplasm of endothelial cells was strongly stained with uranyl acetate and lead citrate. Replication of the capillary basement membrane was observed. On the other hand, easy bleeding and coagulation were not observed in the two patients. These findings suggested that the collagen VI deficiency might have caused the electron microscopic changes of capillaries, while the function of the capillaries is apparently retained.
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7/36. Sclerosing encapsulating peritonitis: differential diagnosis to peritoneal encapsulation and abdominal cocoon--a case report.

    A 59 year old man presented with symptoms of partial bowel obstruction. Small bowel x-ray studies did not allow to identify the nature of the intestinal process in the upper ileum. At laparotomy small bowel encapsulation with a whitish membrane was encountered. Despite partial removal of this membrane small bowel obstruction persisted and two weeks postoperatively the patient died of peritonitis and cardiac insufficiency. autopsy findings revealed massive fibrous adhesions in the abdomen with granulomatous inflammation. The presence of foreign body giant cells and bifringent crystals were characteristic for talcum powder. The latter suggested a causal role of an appendectomy 45 years earlier. The diagnosis of sclerosing encapsulating peritonitis as established in our patient needs to be separated from peritoneal encapsulation, a congenital malformation, and abdominal cocoon, which contains histological elements of inflammation. This case report should draw attention to these entities in the differential diagnosis and surgical management of small bowel obstruction.
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8/36. Membranocystic lesion in sclerosing lipogranuloma of the scrotum: an ultrastructural study.

    Two cases of intrascrotal sclerosing lipogranuloma are reported in which affected adipose tissues showed peculiar membranocystic lesions (MCL) similar to the features described in Nasu-Hakola disease. On light microscopy, the MCL were seen in the degenerative fatty tissues and within multinucleated giant cells, which were positive for periodic acid-Schiff stain and resistant to diastase digestion. Ultrastructurally, two types of MCL were observed; one type was characterized by thick membranes composed of vesicular to minute tubular or fibrillar substructures, and the other type was characterized by thin membranes composed of only small numbers of tiny vesicles. The substructures in both types of MCL contained lipid droplets similar to those in the inner space. These results support the concept that the membranes in the MCL are derived from degenerated fat droplets.
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9/36. Cutaneous sclerosing perineurioma of the digits: an uncommon soft-tissue neoplasm. Report of two cases with immunohistochemical analysis.

    BACKGROUND: Cutaneous sclerosing perineurioma is a recently characterized, uncommon tumor composed of perineurial cells, which exhibits immunoreactivity for epithelial membrane antigen (EMA). These lesions occur preferentially in children and young adults and usually develop as dermal or subcutaneous nodules in the hands or palms. methods: We report two cases of cutaneous sclerosing perineurioma in young patients without stigmata of neurofibromatosis. Histologically, these lesions were well-circumscribed masses and were characterized by a variable number of epithelioid and spindle cells with wavy nuclei end elongated cytoplasmic processes embedded in a dense collagen stroma. RESULTS: These cells showed focal whorling formation, demonstrated robust immunoreactivity for EMA and CD99, and were uniformly negative for S-100 protein, actin (HHF-35), CD34, cytokeratin AE1-3, and CD57. CONCLUSION: We comment on the differential diagnosis of fibrous cutaneous lesions based on immunohistochemistry.
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10/36. Pathological features of surgically excised polypoidal choroidal vasculopathy membranes.

    The histopathological features are reported of surgically excised specimens from five patients with polypoidal choroidal vasculopathy, which had been diagnosed by indocyanine green angiography. On stereomicroscopy, four of the five cases demonstrated large choroidal arterioles with an inner elastic layer. Disruption of the inner elastic layer and arteriosclerotic changes of the vessels were identified by light microscopy. Transmission electron microscopy demonstrated increased deposition of basement membrane-like material, together with collagen fibres, in the arteriolar walls. This study indicates that large choroidal arterioles and venules can be found in excised specimens from patients with polypoidal choroidal vasculopathy and arteriosclerosis is an important pathological feature.
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