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1/13. Bleeding from cavernous angiomatosis of the rectum in Klippel-Trenaunay syndrome: report of three cases and literature review.

    Klippel-Trenaunay syndrome (KTS) is a congenital vascular anomaly characterized by limb hypertrophy, cutaneous hemangiomas, and varicosities. GI hemorrhage is a potentially serious complication secondary to diffuse hemangiomatous involvement of the gut. We report on three patients with KTS who presented with transfusion-dependent anemia and life-threatening bleeding due to extensive cavernous hemangiomas involving the rectum. Two patients were treated by proctocolectomy and coloanal anastomosis, which preserved anal function while controlling bleeding. The third patient required an abdominoperineal resection because of extensive rectal, perianal, and perineal angiomatosis. The literature on the evaluation and management of GI hemorrhage in KTS, particularly of colorectal origin, is reviewed.
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ranking = 1
keywords = angiomatosis
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2/13. intracranial hypertension in Sturge-Weber/Klippel-Trenaunay-Weber overlap syndrome due to impairment of cerebral venous outflow.

    klippel-trenaunay-weber syndrome (KTWS) is a rare mesodermal phakomatosis characterized by (1) cutaneous haemangiomata (usually unilateral and involving an extremity) (2) venous varicosities and (3) osseous and soft tissue hypertrophy, also of the affected limb. sturge-weber syndrome (SWS), also a mesodermal phakamatosis, is characterized by meningofacial angiomatosis with cerebral calcification. Overlap between KTWS and SWS is recognized. We describe the case of a young woman with features of both KTWS and SWS who presented with symptoms of acute intracranial hypertension, including headache, vomiting and marked visual impairment. cerebral angiography revealed paucity of the superficial cortical veins overlying one of the cerebral hemispheres and centripetal venous drainage via small deep venous channels. Based upon this pattern of cerebral venous outflow, we postulate a mechanism to explain the acute episode of intracranial hypertension.
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ranking = 0.2
keywords = angiomatosis
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3/13. An infantile case of sturge-weber syndrome in association with klippel-trenaunay-weber syndrome and phakomatosis pigmentovascularis.

    sturge-weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent klippel-trenaunay-weber syndrome has been reported. klippel-trenaunay-weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case of the sturge-weber syndrome in combination with the Klippel-Trennaunay syndrome and phakomatosis pigmentovascularis in a 4-month-old infant. He showed nevus flameus on the right leg and both part of the face and back, leptomeningeal angiomatosis on right hemisphere, hypertrophy of the right leg, hemiconvulsion on the left and also evidences of congenital glaucoma and nevus of ota. Very rare case combined with these three kinds of phakomatosis has been reported.
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ranking = 0.2
keywords = angiomatosis
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4/13. macrocephaly in association with unusual cutaneous angiomatosis.

    Ten patients are presented who demonstrate a newly recognized association of macrocephaly with unusual angiomatosis and limb asymmetry in three somewhat similar cutaneous vascular disorders: klippel-trenaunay-weber syndrome, the combination of Sturge-Weber anomaly with klippel-trenaunay-weber syndrome, and cutis marmorata telangiectatica congenita. The etiology of the macrocephaly in patients with these conditions is unknown. The majority (seven of ten) of these children have no evidence of central nervous system dysfunction.
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ranking = 1
keywords = angiomatosis
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5/13. Unusual retinal manifestation in a combination of Sturge-Weber and Klipplel-Trenaunay syndrome--a case report.

    An unusual retinal manifestation of arteriovenous communications is reported in a case showing a combination of Sturge-Weber and Kippel-Trenaunay syndromes. The arteriovenous communications are thought to be similar to those seen in the limbs in Parkes-Weber syndrome. This retinal finding indicates that the three syndromes are intricately related to each other and may be an incomplete manifestation of a single pathophysiologic entity which may be named neurocutaneous angiomatosis.
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ranking = 0.2
keywords = angiomatosis
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6/13. spinal cord arteriovenous malformations and the klippel-trenaunay-weber syndrome.

    Five cases in which the klippel-trenaunay-weber syndrome was associated with a spinal cord arteriovenous malformation are reported: they formed part of a series of 150 spinal arteriovenous malformations. Hypertrophic lesions, dilated arteries and varicose veins were present in every case, but cutaneous angiomatosis was found in only two. In each case, there was an intramedullary AVM. The relationship of the klippel-trenaunay-weber syndrome to the regional angiomatous phacomatoses is discussed.
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ranking = 0.2
keywords = angiomatosis
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7/13. Skeletal diseases associated with angiomatosis.

    Basically, there are two conditions in which angiomatosis is associated with underlying skeletal disease. The first is Maffucci's syndrome in which angiomatosis is associated with multiple enchondromatosis. Two patients with this disease are presented and its clinical and radiologic features are reviewed. The second is "congenital angiectatic hypertrophy" in which angiomatosis is associated with localized hypertrophy of underlying bones, soft tissues and occasionally internal vercera. Four patients with this condition are presented, illustrating the subtypes of closely related diseases within a broad spectrum.
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ranking = 1.4
keywords = angiomatosis
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8/13. pregnancy in a patient with the klippel-trenaunay-weber syndrome: a case report.

    A rare case of pregnancy in a patient with the syndrome of Klippel-Trenaunay-Weber is described. The arterio-venous anomalies in this patient originally occurred in her right leg, but in the course of her first pregnancy she also developed circumscript angiomatosis at the left and right side of the uterus. Her pregnancy was uneventful. However, because of prominent vascular changes in the cervix and lower uterine segment, there appeared to be a cephalo-pelvic discongruency. For this reason a cesarean section was performed at term. Postoperatively, during heparinization, she had signs of abdominal bleeding, which could be controlled conservatively. There were no signs of intravascular coagulation or cardiac decompensation. A review of the scarce literature on diffuse uterine angiomatosis and angiomatosis occurring as a result of the syndrome of Klippel-Trenaunay-Weber is given. The clinical course and the risks of a pregnancy with this condition are discussed. It is concluded that the angiomatosis based on the syndrome of Klippel-Trenaunay-Weber is less hazardous than a diffuse angiomatosis of the uterus without this disease, the main risk being diffuse intravascular coagulation at or after delivery.
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ranking = 1
keywords = angiomatosis
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9/13. Severe hemorrhage from intestinal hemangiomatosis in Klippel-Trenaunay syndrome: pitfalls in diagnosis and management.

    A child with Klippel-Trenaunay syndrome (KTS) and severe anemia caused by bleeding from diffuse intestinal hemangiomatosis is presented. Hemangiomas of the bowel should be considered in any child with unexplained anemia and coexisting cutaneous hemangiomas. The diagnostic workup of patients with KTS and symptomatic hemangiomatosis is outlined with respect to indications for surgical management.
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ranking = 1.2
keywords = angiomatosis
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10/13. hematuria, rectal bleeding and pelvic phleboliths in children with the Klippel-Trenaunay syndrome.

    Three young patients with Klippel-Trenaunay Syndrome (KTS), pelvic hemangiomatosis and bleeding are presented. Two children had rectal bleeding and the third presented with hematuria. Radiologic investigation of these patients with complicated angiodysplasia is reviewed. In infants and children calcified phleboliths seen on plain radiographs point toward the correct diagnosis. Angiography shows the vascular malformation which is usually on the venous side. Computed tomography delineates the angiomatous masses and identifies upward extension in the pelvis. urography or renal ultrasound may show an associated kidney anomaly or tumor.
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ranking = 0.2
keywords = angiomatosis
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