Gastrointestinal manifestations of vascular anomalies in childhood: varied etiologies require multiple therapeutic modalities

Abstract

Background/purpose: Vascular anomalies, including hemangiomas and vascular malformations afford complex diagnostic and therapeutic challenges when gastrointestinal (GI) manifestations are present.

Methods: Twenty-one patients evaluated or treated in our Vascular Anomalies Program from 1993 through 1997 were reviewed retrospectively with regard to presentation, treatment modalities, and outcome.

Results: Four patients had hemangiomas, and 17 had various vascular malformations. GI symptoms 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 intussusception (n = 1), protein-losing enteropathy (n = 1), and hypersplenism (n = 1). Four patients had proven portal hypertension. Fourteen had associated musculoskeletal or cutaneous lesions. Congestive heart failure, partial anomalous pulmonary venous return, pulmonary edema, and pleural or pericardial effusion occurred in one patient each. Bleeding was the most common symptom of both hemangiomas and malformations. Of four patients with hemangiomas, three were treated with corticosteroids or interferon. Endoscopic banding and embolization of an associated arterioportal hepatic shunt were each used in one patient. One patient died. The malformations were treated with resection (n = 8), endoscopic banding or sclerosis (n = 7), percutaneous or intraoperative sclerosis (n = 5), embolization or device interruption (n = 3), and portosystemic shunt (n = 2). GI symptoms were ameliorated in 12 patients with malformation, improved in two, unchanged in two, and one died after prolonged palliation.

Conclusions: Vascular anomalies with gastrointestinal manifestations are heterogeneous in their presentation and type. Although bleeding is the most common symptom of both hemangiomas and vascular malformations, treatment differs. Pharmacological angiogenesis inhibition is the mainstay of hemangioma therapy. Resection, endoscopic or radiologic vascular obliteration, and portal decompression are important in treating vascular malformations. An individualized and interdisciplinary approach is often required to successfully diagnose and treat these complex lesions.