GASTROINTESTINAL MANIFESTATIONS OF VASCULAR ANOMALIES IN CHILDHOOD - VARIED ETIOLOGIES REQUIRE MULTIPLE THERAPEUTIC MODALITIES

Background/Purpose: Vascular anomalies, including hemangiomas and vasc ular malformations afford complex diagnostic and therapeutic challenge s when gastrointestinal (GI) manifestations are present. Methods: Twen ty-one patients evaluated or treated in our Vascular Anomalies Program from 1993 through 1997 were reviewed retrospectively with regard to p resentation, treatment modalities, and outcome. Results: Four patients had hemangiomas, and 17 had Various vascular malformations. GI sympto ms began in infancy or early childhood in all patients. Manifestations included GI bleeding (n = 15), obstruction (n = 2), diarrhea (n = 2), ascites(n = 2), pain (n = 1), emesis(n = 1), ileo-ileal intussuscepti on (n = 1), protein-losing enteropathy (n = 1), and hypersplenism (n = 1). Four patients had proven portal hypertension. Fourteen had associ ated musculoskeletal or cutaneous lesions. Congestive heart failure, p artial anomalous pulmonary venous return, pulmonary edema, and pleural or pericardial effusion occurred in one patient each. Bleeding was th e most common symptom of both hemangiomas and malformations. Of four p atients with hemangiomas, three were treated with corticosteroids or i nterferon. Endoscopic banding and embolization of an associated arteri oportal hepatic shunt were each used in one patient. One patient died. The malformations were treated with resection (n = 8), endoscopic ban ding or sclerosis (n = 7), percutaneous or intraoperative sclerosis (n = 5), embolization or device interruption (n = 3), and portosystemic shunt (n = 2). GI symp toms were ameliorated in 12 patients with malfo rmation, improved in two, unchanged in two, and one died after prolong ed palliation. Conclusions: Vascular anomalies with gastrointestinal m anifestations are heterogeneous in their presentation and type. Althou gh bleeding is the most common symptom of both hemangiomas and vascula r malformations, treatment differs. Pharmacological angiogenesis inhib ition is the mainstay of hemangioma therapy. Resection, endoscopic or radiologic vascular obliteration, and portal decompression are importa nt in treating Vascular malformations. An individualized and interdisc iplinary approach is often required to successfully diagnose and treat these complex lesions. J Pediatr Surg 33:1163-1167 Copyright (C) 1998 by W.B. Saunders Company.