An Unusual Case of Budd-Chiari Syndrome—A Case Report

Abstract

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.

Unusual hepatic‐portal‐systemic shunting demonstrated by Doppler sonography in children with congenital hepatic vein ostial occlusion

“…As a result, the patient died postoperatively due to hemorrhage of the liver, and the correct diagnosis was determined at autopsy. 27 The range of clinical presentation in patients with BCS varies widely. The classic triad of symptoms consists of abdominal pain, hepatomegaly, and ascites; BCS can appear in a severe form, with fulminant hepatic failure, or it can be indolent and asymptomatic.…”

Familial Budd-Chiari Syndrome in China: A Systematic Review of the Literature

“…Although the congenital theory for the pathogenesis of BCS is hypothesized by early histopathological studies [ 13 – 15 ], it has never been advocated by the current practice guideline and consensus due to the rarity of BCS in childhood and the anatomic and topographic inconsistency of BCS [ 16 – 18 ]. Several scattered case reports have demonstrated familial clustering of BCS, suggesting the possibility of congenital etiology [ 19 – 22 ].…”