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KMID : 0816120080110010056
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Korean Journal of Pediatric Gastroenterolology and Nutrition
2008 Volume.11 No. 1 p.56 ~ p.59
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A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect
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Lee Hae-Jeong
Lee Jee-Hyun
Huh June
Kang I-Seok
Lee Heung-Jae
Suh Yeon-Lim
Yoo So-Young
Choe Yon-Ho
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Abstract
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Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 56∼59)
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KEYWORD
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Abernethy malformation, Focal nodular hyperplasia, Hepatic encephalopathy
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