CONGENITAL ABSENCE OF THE PORTAL-VEIN - 2 CASES AND A PROPOSED CLASSIFICATION-SYSTEM FOR PORTASYSTEMIC VASCULAR ANOMALIES

Congenital absence of the portal vein (CAPV) is an unusual condition t hat often is associated with other anoma lies. This is the first repor t of reduced size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no po rtal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complica ted by severe mesenteric edema caused by an increase in portal pressur e when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indica te that the CAPV can be classified into one of two groups of portasyst emic anomalies. Copyright (C) 1994 by W.B. Saunders Company