Ann Hepatobiliary Pancreat Surg.  2020 May;24(2):203-208. 10.14701/ahbps.2020.24.2.203.

Living-donor liver transplantation for Abernethy malformation - case report and review of literature

• Nam HD

• KMID: 2500807
• DOI: http://doi.org/10.14701/ahbps.2020.24.2.203

Abstract

Abernethy malformation was named for the rare congenital absence of the portal vein (CAPV), also known as congenital extrahepatic portal-systemic shunts (CEPS). This could be classified as complete (type 1) or incomplete shunt (type 2) according to Morgan-Superina classification. Its presentation may show under variable signs and symptoms such as hepatopulmonary syndrome, hyper-ammonia, hepatic masses and liver failure…. This usually combined with other congenital anomalies (cardiac anomaly, trisomy…). This report presented a 10 year-old boy with growth retardation and mild mental recognition, intermittent hyperammonia, elevated liver enzymes, huge inoperable mass in the right liver. MS CT and MRI findings: hyperplasia of liver parenchyma with superior mesenteric vein confluenced with splenic vein before draining directly into the inferior vena cava (Abernethy anomaly type 1b). Living donor liver transplantation underwent using right lobe from his mother. Anatomopathology findings of the native liver showed chronic hepatitis with cirrhosis 4/6 Knodel-Ishak. Postoperatively, the patient still attained optimal liver function and has returned to normal life at 12-month follow-up. Liver transplantation was a reasonable indication for CAPV type 1. Living donor liver transplantation was effective and practical in the scarcity of donor organ.

Keyword

Abernethy malformation; Congenital absence of portal vein; Congenital extrahepatic portosystemic shunt; Living donor liver transplantation

Figure

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  Fig. 1 (A) Abdomen 3-phase CT Scan; (B) visceral vascular 3D reconstruction showed portal vein drained into inferior vena cava in end-to-side manner.

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  Fig. 2 Intraoperative view: portal vein drained into inferior vena cava in end-to-side manner.

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  Fig. 3 Native liver macroscopic mass explanted view.

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  Fig. 4 Pathology findings charactered with chronic hepatitis, cirrhosis. Portal triads with arterioles and interlobular bile ducts, absence of portal central venules (H&E stain, ×400).

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  Fig. 5 Abdomen 3-phase CT Scan 3 weeks after right-lobe graft liver transplantation (Patent recipient PV as well as graft right PV).

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