Placental chorioangiomatosis--a high risk pregnancy.
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A case of diffuse chorioangiomatosis leading to fetal hydrops, disseminated intravascular coagulopathy with massive umbilical vein thrombosis and fetal death is described. Although rare, this benign mesenchymatous malformation of the placenta should be kept in mind as a possible cause of neonatal morbidity. Prenatal diagnosis could prevent fetal death. (+info)
An unusual variant of Klippel-Trenaunay-Weber syndrome.
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The association of soft tissue and bony overgrowth, varicose veins and port-wine nevus is known as Klippel-Trenaunay-Weber syndrome. An unusual case of a young woman with this syndrome is presented. In addition to the classic features she had face swelling, bilateral limb involvement and an infected cutaneous lymphangioma. No evidence of arteriovenous fistula was demonstrated. In contrast to an abnormal lymphatic system the deep veins of the limbs were normal. Unilateral lung lesions were demonstrated which have not been described previously in such cases. (+info)
Late complications of hemispherectomy: report of a case relieved by surgery.
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A case of Sturge-Weber disease treated with left hemispherectomy presented, 11 years later, with complications related to delayed intracranial haemorrhage. A loculation syndrome of the right lateral ventricle was detected and it was corrected by a ventriculoatrial shunt operation. The side of the hemispherectomy was evacuated of all the chronic products of haemorrhage, including the subdural membrane. The patient was relieved of her symptoms. It is considered that complications related to delayed haemorrhage after hemispherectomy are remediable. (+info)
Surgical implications of Klippel-Trenaunay syndrome.
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Between January 1956 and July 1981, 40 patients with Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Twenty male and 20 female patients presented with the classic triad of soft tissue and bony hypertrophy of the extremity, hemangioma, and varicosity without evidence of functional arteriovenous fistulae. The lower extremity was involved in 38 patients (95%), the upper extremity in six patients (15%). In four of these cases (10%), both the upper and lower extremities were affected. The disease was unilateral in 34 patients (85%), bilateral in five cases (12.5%), and crossed-bilateral in one case (2.5%). Surgery was done in 13 patients (32.5%), nine of whom were operated on at the Mayo Clinic and four of whom had had previous surgery elsewhere. Excision and stripping of varices were performed in three patients; of these three, a deterioration of symptoms was observed in one, but the procedure was beneficial in the other two. Partial varicectomy was performed in four cases, while resection of the angioma was attempted in eight cases, with good results in three cases of small angiomas. Femoral and tibial epiphysiodesis stopped the overgrowth and produced an excellent result in one case. In four cases of epiphysiodesis at the foot level and two cases of derotational tibial osteotomy, moderate improvement was achieved. The indication for vascular or orthopedic surgery should be carefully considered in each patient who has this syndrome. One patient (2.5%) died of a severe form of the disease, but the relatively benign course is documented by the 21 patients (52.5%) who are free of complaints without any treatment or with elastic support only. (+info)
Klippel-Trenaunay-Weber syndrome: appearance in utero.
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We were able to detect hemihypertrophy of a fetus in a routine prenatal ultrasound examination. The presence of hemangiomas and varicose veins in the hypertrophied left lower extremity at birth enabled us to diagnose the Klippel-Trenaunay-Weber syndrome. The possibility of a congenital arteriovenous fistula causing the constellation of findings was ruled out by a nuclear flow study and a thorough physical examination. A computerized tomographic scan revealed that the hemangiomas were superficial in the hypertrophied left lower extremity and did not interrupt the deep muscle bundles. (+info)
Renal carcinoma in Lindau's disease.
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A case of Lindau's disease is presented where renal carcinoma was detected 2.5 years after surgical removal of a cerebellar haemangioblastoma. This patient had no retinal lesions. The importance of screening for renal carcinoma in patients showing one or more manifestations of the disease and screening relatives is emphasized. (+info)
Segregation and linkage analyses of von Hippel Lindau disease among 220 descendants from one kindred.
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Von Hippel Lindau disease (vHL), an autosomal dominant precancerous condition, had segregated in a large kindred. Fourteen relatives were known to have been affected; record reviews disclosed features of vHL in 15 previously undiagnosed relatives; presymptomatic evaluations detected vHL in 13 additional members of this kindred. Altogether, among 220 descendants of an ancestral couple, 41 had vHL. We screened for HLA haplotypes and for polymorphic gene markers at 31 loci in 102 direct descendants and 16 spouses from this kindred, including 23 with vHL. Linkage analyses failed to reveal a significant lod score with any locus tested, or any HLA linkage disequilibrium. Expression of vHL among the affected relatives was compared with 384 other reported cases of vHL. The age of onset, tissue involvement, and life expectancy in this family were similar to the other reported cases. The sigmoid age-of-onset distribution for vHL most closely matched a square-foot transformation (mean = 26.2(-2) years; variance = 1.224). (+info)
Cerebral venous sinus thrombosis associated with systemic multiple hemangiomas manifesting as chronic subdural hematoma--case report.
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A 35-year-old male was admitted with headache, nausea, and vomiting persisting for 2 days. Computed tomography (CT) revealed a left chronic subdural hematoma. Cerebral angiography demonstrated cerebral venous sinus thrombosis (CVST). He had presented with a subcutaneous mass involving the neck at age 2 years, which was shown to be a cavernous angioma, and thereafter shown signs of consumptive coagulopathy with systemic multiple hemangiomas. Burr hole aspiration of the hematoma was performed. Seventy-two-hours later, he developed clouding of consciousness and right hemiparesis. CT revealed a fresh hematoma in the operated subdural cavity and hemorrhagic diathesis manifested. A frontotemporoparietal large craniotomy was performed to remove the hematoma. Extensive electrocauterization was required. He had a satisfactory postoperative course. Collateral venous pathways, resulting from the CVST due to systemic multiple hemangiomas, may have caused hemodynamic stress in the bridging veins which subsequently induced chronic subdural hematoma. (+info)