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1/12. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.

    INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.
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2/12. Retinal arteriovenous communication.

    BACKGROUND: Disturbances in development of the embryonic vascular system, though uncommon, may cause formation of retinal arteriovenous communications (RAC). Such anomalies may also involve the intracranial, orbital, and maxillofacial blood vessels. Field loss, neovascular glaucoma, optic atrophy, Parinaud syndrome, hemiparesis, hemiplegia, and proptosis may be associated with RAC. CASE REPORT: A 27-year-old woman was referred for photography of a vascular anomaly in her left eye. I noted a large arteriovenous malformation joining major temporal branches of the left central retinal artery and vein. External and internal examination of both eyes was otherwise unremarkable. Visual-field testing revealed nasal field disturbances for the left eye, but none for the right eye. CT scan showed no obvious intracranial abnormalities. CONCLUSIONS: Visual-field testing may show scotomas associated with retinal arteriovenous communications. It is possible for patients with RAC to have no associated vascular abnormalities elsewhere in the circulation of the head.
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3/12. Pancreaticobiliary arteriovenous malformation with common bile duct dilation in a patient with hemobilia.

    Arteriovenous malformation of the pancreas is a very rare entity. We describe a case involving the head of the pancreas associated with progressive hemobilia bleeding from the lower part of the common bile duct. The patient was a 50-year-old man with acute epigastralgia. Endoscopic retrograde cholangiopancreatography revealed hemobilia and cystic dilation of the common bile duct. angiography demonstrated increased blood volume in the head of the pancreas and early filling of the superior mesenteric and portal veins. abdominal pain and progressive anemia caused by hemobilia required surgical treatment. Histologic examination of the resected specimen revealed marked proliferation of the blood vessels in the pericholedochal area and the exact point of bleeding from the pancreaticobiliary arteriovenous malformation.
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4/12. angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.

    A case of angiolymphoid hyperplasia with eosinophilia (ALH) is reported in a 33-year-old woman who developed an auricular nodule during the second trimester of her pregnancy. angiolymphoid hyperplasia with eosinophilia usually occurs on the head and neck of young adults and is more common in women than in men. Characteristic histologic features of ALH present in this case included proliferation of thick-walled blood vessels lined by prominent endothelial cells, infiltration of the interstitium by chronic inflammatory cells (mainly eosinophils), and presence of lymphoid follicles with germinal centers. The auricular tumor was completely excised. Thirteen months after excision, the patient remains tumor free. Although there are not many case reports on ALH during pregnancy or involving use of oral contraceptive pills, sex hormones may play a role in the pathogenesis of ALH. This hypothesis, in the context of cases previously described in the literature, and the differential diagnosis of ALH are discussed.
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5/12. Arteriovenous malformation of the uterus--a cause of massive operative bleeding.

    arteriovenous malformations of the uterus are extremely rare and they occur either in congenital or acquired forms. The most common clinical presentation is abnormal uterine bleeding, which may be aggravated by therapeutic curettage. Because of their rare incidence and clinical importance in management of patients, we report a case of arteriovenous malformation causing serious bleeding during a hysterectomy for uterine leiomyoma. The patient was a 47-year-old multiparous woman who had a history of chronic vaginal bleeding for one year. Numerous anomalous blood vessels draining into the right and left uterine arteries were found on the anterior wall of the uterus and parametrium.
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6/12. angiomatosis: a vascular malformation of infancy and childhood. Report of 17 cases.

    angiomatosis is a complex vascular malformation of infancy and childhood consisting of proliferating blood vessels with accompanying mature fat and fibrous tissue, lymphatics and sometimes nerves, that may involve skin, subcutaneous tissue, skeletal muscle and occasionally bone; lesions are non-encapsulated with poorly defined infiltrative borders. Treatment is surgical, with local recurrence being common. We report 17 cases of angiomatosis presenting in children. Recurrences occurred in 10 patients, with multiple recurrences occurring in four. One child was treated with foot amputation followed two years later by mid-thigh amputation in an attempt to control local disease. histology in all cases showed a mixture of small and medium-sized blood vessels, fat, connective tissue and lymphatics; nerves were increased in several cases. All lesions showed nests of proliferating capillaries, arranged in a lobular pattern, pushing into adjacent muscle and fat. This appearance was not seen in a large comparison group of vascular soft tissue lesions, and may serve as an indicator of angiomatosis with its associated risk of recurrence.
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7/12. Arteriovenous malformation of the bladder presenting as gross hematuria.

    Arteriovenous malformation, in which direct communication is present between arterioles and venules, are reflected histologically by abrupt changes in the thickness of the medial and elastin layers of the vessels. Another result of the lack of the interposed capillary bed is abnormal dilation and, often, advanced small vessel disease, which is due to the increased intravascular pressures as well as to the basic defects in the blood vessel walls. The diversion of arterial flow and small vessel disease may lead to ischemia, which is postulated to stimulate proliferation of the vascular channels in these lesions. Hence, they tend to grow slowly with time. The ischemia, increased pressure, and small vessel disease predispose to ulceration and hemorrhage, which is a common mode of presentation for these lesions. Common sites for arteriovenous malformations are the intestine, central nervous system, lungs, and extremities. The lesion has not been reported in the urinary bladder. The present case of massive hematuria was found at autopsy to be due to an arteriovenous malformation of the bladder neck.
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8/12. Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia.

    Clinical and radiological findings of hepatogenic pulmonary angiodysplasia are reported in two cases. Myriad spidery pulmonary blood vessels are seen on plain radiographs and verified with right to left intrapulmonary shunting on pulmonary angiogram and pulmonary isotopic perfusion scan. Pathophysiology and differential diagnosis are discussed. We propose that the term "pulmonary angio-dysplasia" should include: 1) Pulmonary telangiectasia 2) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) 3) Classical discrete pulmonary arteriovenous fistula 4) Hepatogenic pulmonary angiodysplasia: pulmonary arteriovenous communications with right to left shunting secondary to hepatic cirrhosis.
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9/12. Computed tomography of benign angiomatous lesions of the extremities.

    Thirteen patients with benign angiomatous lesions of the extremities were evaluated by CT. The relationship of the lesion to specific muscle groups and fascial planes was defined better by CT than on plain radiography or angiography. Computed tomography without contrast enhancement demonstrated well circumscribed masses of lower attenuation value than the surrounding muscle. Eight of the 11 patients who received intravenous injection of contrast material showed characteristic punctate or curvilinear structures within the mass, which had attenuation values similar to normal blood vessels. The characteristic CT features of benign angiomatous lesions of the extremities are presented.
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10/12. Intracranial angiolipoma: report of two cases.

    Intracranial tumours composed of adipose tissue and abnormal blood vessels are rarely recognized. We report two such cases; one showed the radiological and histological appearances of a combined lipoma and an arteriovenous malformation, the other the appearances of a lipoma with a prominent capillary component. Computed tomography did not give any indication of their vascular nature, but surgery was complicated by intra- and postoperative haemorrhage.
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