Klippel-Trénaunay syndrome associated with chronic disseminated intravascular coagulation and massive osteolysis

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The Klippel-Trénaunay syndrome is characterized by a hemangiomatous lesion of a limb with varices and limb hyperplasia. Recently a boy was evaluated with this syndrome in association with two other rare conditions, chronic disseminated intravascular coagulation, and massive osteolysis. These disorders are discussed and their possible interrelationships explored.

Case report

A 15-year-old boy was first examined at the Cleveland Clinic in March 1972. He was the second of two children; his mother had had a normal pregnancy, labor, and delivery. As a neonate, a large hemangioma involving the left buttock and part of the left thigh was noted. Circumcision at that time was uneventful. No medical problems were described until the patient was 13 months old when he reportedly had a pathologic fracture of the left tibia which healed poorly despite extensive therapy. Consultants concluded that the hemangioma over the left limb involved the tibia causing delayed healing of the fracture. Following that event, little growth of the left leg was attained, although the limbs were described as being equal in length prior to that time.

The patient did well until March 1972, when he was first examined for continued oozing from the site of plantar wart surgery. No history of epistaxes, bleeding gums, or petechiae was elicited, although he did complain of always having bruised easily. Physical examination revealed a healthy looking boy without evidence of bruising or petechiae. A large cavernous hemangioma involving the left buttock and extending onto the thigh of a shortened left limb was the. . .



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