Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay syndrome.

The Klippel-Trenaunay syndrome is a congenital vascular anomaly consisting of the triad of soft-tissue and bony hypertrophy of the extremities, hemangiomas and/or lymphangiomas, and varicosities. From 1956 to 1983, 42 patients with the Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Of the 42 patients, 16 had involvement of the trunk (thorax, abdomen and/or pelvis). Twelve of the 16 had evidence of the malformation at birth and 4 had evidence shortly thereafter. Lesions involving the trunk included hemangiomas (75%) and lymphangiomas (50%). Complications included rectal bleeding, hematuria, colonic obstruction, hemothorax, paraparesis secondary to clotting disorder, and compression of the external urethral meatus. One child died of enlargement of diffuse lymphangiomas and hemangiomas of the abdomen and chest. Eight of the 16 patients underwent 13 operative procedures. These included excision of the superficial lymphangiomas, resection of the rectosigmoid colon, and resection of an extremely large retroperitoneal mass of hemangiomatous-lymphangiomatous tissue. While progression requiring major surgery was seen in several patients, most patients had supportive therapy, with minimal surgery being necessary.