Fibrose hepática congênita associada à trombose portal: coincidência? / Congenital hepatic fibrosis associated with portal vein thrombosis: Is it coincidental?

Fibrose hepática congênita (FHC), doença fibropolicística, ocorre em várias formas, tendo como manifestação clínica inicial, em crianças, sangramento digestivo alto em pré-escolares entre um e três anos. História clínica similar à trombose portal congenita. Ainda está sob investigação os fatores etiopatogênicos da FHC, e o presente relato de um raro caso de associação entre estas duas afecções, aparentemente congênitas, adiciona argumento para a recente sugestão de que essa associação não seja apenas coincidência

An unusual case of Budd-Chiari syndrome--a case report.

The authors report a rare case of congenital Budd-Chiari syndrome in a twenty-eight-year-old male mongoloid. The patient was submitted to azygous-portal disconnection, because of the syndrome of portal hypertension supposedly due to cirrhosis of the liver. He died of hemorrhage of the liver on the third postoperative day. Autopsy revealed a congenital fibrotic obstruction of all suprahepatic veins, with a wide, round ligament containing a functional umbilical vein, which had been routinely ligated during surgery. An extensive review of the literature showed no similar report. The authors speculate that the inadvertent interruption of the round ligament, which until then had served as a pathway for venous draining of the liver, followed by ligation of the anastomoses between the portal and azygous systems, was the factor that triggered the lethal outcome. Thus, this appears to be the first case of congenital Budd-Chiari syndrome predominantly maintained at the expense of the round ligament of the liver, with a patent vascular branch.