Hepatoblastoma and Abernethy Malformation Type I: Case Report

Abstract

A 2-year-old boy presented with pneumonia and an abdominal mass was noted incidentally. A right lobe hepatic mass classified as PRETEXT III and congenital absence of the portal vein with drainage of the superior mesenteric vein to the inferior vena cava (Abernethy malformation type I) were confirmed by computed tomography and angiography. After a clinical diagnosis of hepatoblastoma had been made, he was treated with 4 cycles of doxorubicin and cisplatin and hepatic arterial chemoembolization with doxorubicin, after which the tumor was classified as POSTEXT III. He underwent a right extended hepatic lobectomy with resection of the caudate lobe but died on postoperative day 4 due to hepatic failure. The Abernethy malformation type I is associated with the development of hepatic tumors, and the abnormal blood flow might predispose to hepatic failure after liver resection. Extensive study of the hepatic vasculature is warranted in patients with suspected malformations. Liver transplant could be considered in patients with congenital portosystemic shunt and malignant liver tumors.

Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II

“…Second, hepatic dysfunction, nodular hyperplasia, or tumor are caused by inadequate PV perfusion of the liver. Nodular liver lesions were observed in approximately one-half of reported cases (11,12). Most of the lesions, such as FNH (36.73%), were benign.…”

“…Type I is prone to lead to cirrhosis and liver cancer caused by the absence of portal vein as well as continuous insufficient blood supply to the liver. Consequently, liver transplantation is considered to be the main radical treatment method (12,25,26). In terms of type II, the opening of collateral circulation can relieve the pressure of PV, which may be conducive to the patient.…”

Abernethy Malformation Type II and Concurrent Nodular Hyperplasia in a Rare Female Case

“…Abernethy malformation was first described in 1793 in an autopsy of a 10-month-old female who died of unknown causes and of whom the portal vein bypassed the liver and directly drained into the inferior vena cava [ 2 ]. There have been up to 100 cases currently reported [ 3 ].…”

Congenital Portosystemic Shunts

“…Examples include patients in whom the shunt is associated with a liver malignancy (62,63), or patients with shunts too numerous to close simultaneously or sequentially, although less frequent, patients with life-threatening extrahepatic complications may not withstand multiple IR procedures. These may benefit from primary liver transplantation to restore normal portal flow (60).…”