Pediatr Gastroenterol Hepatol Nutr.  2019 Jan;22(1):80-85. 10.5223/pghn.2019.22.1.80.

The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

Affiliations
  • 1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. i101016@skku.edu
  • 2Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University School of Medicine, Seongnam, Korea.

Abstract

PURPOSE
Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China.
METHODS
We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017.
RESULTS
A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy.
CONCLUSION
There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

Keyword

Congenital extrahepatic portosystemic shunt; Korean children

MeSH Terms

Child*
China
Consensus
France
Hemorrhage
Hepatic Encephalopathy
Humans
Korea
Ligation
Liver Transplantation
Vena Cava, Inferior
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