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1/40. endosonography in the diagnosis of "blue rubber bleb nevus syndrome": an uncommon cause of gastrointestinal tract bleeding.

    Blue rubber bleb nevus syndrome is a rare condition characterized by the presence of multiple angiomatic lesions of the skin. These are associated with similar lesions in other organs, namely in the gastrointestinal tract, causing anemia through chronic bleeding. We describe the case of a 72-year-old woman with microcytic anemia. A barium study revealed irregular lacunae in the distal esophagus. A subsequent endoscopy showed blue nodular lesions similar to angiomas of the esophagus and stomach fundus. endosonography confirmed its angiomatic nature. Exploration of other organs, using magnetic resonance and cranial computed tomography, did not reveal the presence of this type of lesion. In physical examination, two angiomatic lesions were observed on the face and lips, respectively. These were blue in color and compressible, leaving an empty wrinkled sac that rapidly refilled, typical of angiomas.
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2/40. Ruptured primary splenic angiosarcoma into the colon. Presentation as anal bleeding.

    A 71-year-old woman presented with a six month history of constipation and abdominal discomfort, with anal bleeding during the last days. ultrasonography and CT-scan of the abdomen showed a large heterogeneous mass that was located in the splenic region, but the nature and origin of the tumour could not be clearly established preoperatively. The clinical diagnosis was of abdominal tumour with colonic and splenic involvement, and a left hemicolectomy and splenectomy were performed. Pathologic examination revealed a primary angiosarcoma of the spleen with penetration and fistulization of the tumour into the large bowel. The patient received adjuvant radiation therapy, but she died of extensive metastastic disease from her primary angiosarcoma of the spleen nine months after surgery. In summary, splenic angiosarcoma is very difficult to diagnose preoperatively. This highly aggressive neoplasm has an overall poor prognosis, specially if it is associated with rupture and haemoperitoneum. As this case highlights, unusual forms of rupture may lead to atypical clinical presentations, increasing even more the difficulty in the diagnosis.
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3/40. angiography in the diagnosis and therapy of hemorrhage from the large bowel.

    angiography has added a new dimension to the management of hemorrhage from the large bowel. In patients with diverticular hemorrhage, mesenteric angiography not only localizes the bleeding site but, in addition, the bleeding can be acutely controlled with intraarterial infusion of vasopressin, making an emergency colectomy unnecessary. Similarly in patients bleeding from inflammatory bowel disease or in patients with post-operative hemorrhage, angiography provides information about the nature of the lesion and selective arterial infusions of vasopressin can control the bleeding. At times intestinal varices have angiographically been demonstrated as a potential source of rectal hemorrhage while in patients with unexplained lower gastrointestinal bleeding and repeatedly negative barium and endoscopic examinations, angiography has been valuable for the diagnosis of angiodysplasia of the colon.
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4/40. The watermelon rectum.

    This is the first reported case of rectal vascular ectasia in association with gastric antral vascular ectasia (watermelon stomach). The patient, a 39-year-old woman with systemic sclerosis, reported chronic gastrointestinal blood loss and iron deficiency anemia. She had aperistalsis of the distal esophagus, decreased lower esophageal sphincter pressure leading to severe gastroesophageal reflux, and dysmotility of the small intestine. diagnosis was made by endoscopic appearance of visible linear watermelon-like vascular stripes in the gastric antrum and rectum. histology confirmed the vascular nature of this disorder, showing dilated and thrombosed capillaries in the lamina propria. Gastric biopsy showed associated fibromuscular hyperplasia in the lamina propria. However, the colonic biopsy showed only focal hyalinization in the lamina propria. The patient was successfully treated using endoscopic bipolar electrocautery. Watermelon stomach has previously been described in association with systemic sclerosis. Based on the histopathologic similarity between rectal vascular ectasia, gastric antral vascular ectasia, and systemic sclerosis, we suggest that the rectal vascular lesion may represent a component of the gastrointestinal manifestations of systemic sclerosis.
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5/40. Penile metastasis from rectal carcinoma.

    We are presenting a 65-year old patient with metastatic carcinoma of the penis which was discovered 19 months after abdomino-perineal resection for rectal cancer (Duke A). There was also metastasis in the perineum and one rib. Penile biopsy and cavernosography were carried out and established the metastatic nature. The patient declined further therapy and died 5 months after diagnosis.
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6/40. Ectopic prostatic tissue of the anal canal presenting with rectal bleeding: report of a case.

    PURPOSE: Ectopic prostatic tissue at various sites within and outside the genitourinary system has been reported previously. A case of ectopic prostatic tissue located in the anal canal causing rectal bleeding is presented. METHOD: The patient was referred to our clinic with rectal bleeding. At rectal examination a bleeding sessile polypoid mass 2.5 cm in size was found in anal canal and removed surgically. RESULTS: Histopathologic and immunohistochemical staining of the specimen confirmed the prostatic nature of the tissue. CONCLUSION: Prostatic heterotopia is significant in several respects. Either it may be an important cause of hematuria or unusually, as in our case, it may cause rectal bleeding. In addition, ectopic tissue may be endoscopically confused with malignancy in either urinary or lower gastrointestinal system. This and other reports may disclose the genesis and significance of this peculiar tissue remnant.
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7/40. Successful surgery using recombinant factor viia for recurrent, idiopathic nonulcer duodenal bleeding in a patient with Glanzmann's thrombasthenia.

    A 68-year-old man with Glanzmann's thrombasthenia suffered from recurrent cryptogenic bleeding originating in the upper duodenal flexure. Extensive endoscopic procedures and medical treatments were unsuccessful and proximal duodenectomy was proposed. In preceding years platelet transfusions had often had a suboptimal result and were complicated by allergic reactions. Surgery was carried out while he was being treated with recombinant factor viia. Neither major blood loss nor other complications occurred. Histological examination of the bleeding site failed to show abnormalities and the nature of this patient's bleeding problem remained unexplained. Glanzmann's thrombasthenia is a rare autosomal recessive disorder of platelet aggregation characterized by a lifelong bleeding tendency due to abnormalities of the glycoprotein IIb-IIIa membrane complex. Common clinical manifestations include purpuric type bleeding, epistaxis, menorrhagia and gingival bleeding. Spontaneous bleeding is uncommon but posttraumatic and postoperative hemorrhage may be particularly serious. There is no specific treatment. Prophylactic and therapeutic platelet transfusions are the cornerstone of supportive treatment. In many patients the efficacy of this approach is diminished by allo-anti-platelet antibodies. We report on a patient with Glanzmann's disease with recurrent nonulcer duodenal bleeding refractory to conservative medical treatment. Despite documented suboptimal effectiveness of platelet transfusions, he underwent successful surgery with administration of recombinant factor viia (rFVIIa).
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8/40. angiodysplasia as a cause of gastrointestinal bleeding in childhood.

    Whereas in adults angiodysplasia is a frequent cause of gastrointestinal bleeding, in children this disorder is extremely rare. A 7 10/12 year old girl is presented suffering over 3-4 months from mild but recurrent rectal bleeding. blood count and serum ferritin and transferrin levels were normal. The rectosigmoideoscopy revealed a rectal lesion, which was confirmed histologically as angiodysplasia. Pathological investigation of the biopsies included HE staining and immunohistological staining of endothelial cells with anti-CD34 and anti-von willebrand factor. A follow-up period of three years revealed spontaneous regression of the angiodysplastic lesion at the rectosigmoideal localisation, which could be confirmed by endoscopy. CONCLUSION: The outcome of the few pediatric patients described in the literature was reviewed. Due to the lack of conclusive understanding of the nature of this extremely rare vascular disorder and the variable outcome described, a wait and see attitude should be assumed in cases of less clinical affection.
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9/40. Management of an esophagogastric fibromatosis in a child: a case report.

    Intraabdominal fibromatosis is an uncommon neoplasm. The aggressive nature of these tumors and the potential for major morbidity secondary to resection can present a difficult surgical dilemma. A 9-year-old boy presented with anemia and vomiting. He had esophagogastric fibromatosis diagnosed and underwent distal esophagectomy, total gastrectomy, Hunt-Lawrence jejunal pouch, and Roux-en-Y esophagojejunostomy. This report describes the successful surgical management of an esophagogastric fibromatosis for the first time in the English-language literature. The management of this tumor is discussed with particular regard to the very unusual clinical presentation.
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10/40. diagnostic imaging of the small bowel in a case of occult gastrointestinal bleeding.

    A case of a male patient with occult gastrointestinal bleeding and negative gastroscopy and colonoscopy is presented and discussed. Oral small bowel barium follow-through was performed upon the patient request. It documented a jejunal neoformation. The analysis of radiologic findings directed towards a probably extramucosal origin. Subsequent CT evaluation confirmed a neoformation inseparable from a jejunal loop with inhomogeneous contrast enhancement, therefore of suspected malignant nature. The histological examination on the surgical specimen documented the presence of a stromal tumor of indeterminate malignancy.
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