Splanchnic bed vascular malformations and the development of portal hypertension

Abstract

The vascular lesions, angiomas, and arteriovenous malformations are known to be associated with a number of complications. These lesions, when present in the liver, may precipitate heart failure in infants. Recent reports suggest that although the prognosis is good for the majority of infants who survive the neonatal period, the lesions are capable of causing portal hypertension later in life. We have encountered three patients whose vascular lesions were in continuity with the portal circulation who subsequently went on to develop portal hypertension. One patient had angiomas of the liver, one splenic and colonic angiomas, and the third had diffuse arteriovenous malformations of the intestine. Data are presented and literature is reviewed, which suggest that any vascular lesion that is capable of substantially increasing portal blood flow may lead to the development of portal hypertension. The treatment of such lesions should be aggressive. If surgical management is not possible, alternative treatments such as embolization should be attempted early in order to increase the chance for successful therapy.