Congenital Portosystemic Hepatic Fistula: A case report

Abstract

Congenital portosystemic fistula (CPF) is a congenital malformation defined by one or more communications between a hepatic portal venous system and a systemic venous cava. Other terminologies exist in the literature, in particular for extrahepatic communications, such as "Abernethy malformation". It may be associated with other malformations or syndromes. Because of this non-physiological vascular communication, PFC can lead to the development of extrahepatic complications: hepatic encephalopathy and pulmonary vascular diseases (hepato-pulmonary syndrome or pulmonary hypertension). It can also lead to hepatic complications with the appearance of benign or malignant tumors and biological and metabolic disorders. We report the case of a newborn with a portosystemic hepatic fistula discovered during neonatal jaundice with cutaneous angiomatosis.