Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome
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Congenital extrahepatic portosystemic shunt (CEPS) is a rare malformation of the mesenteric vasculature, which may lead to severe complications. In this report, we describe a case series of three children with type II CEPS (presenting as hypoxemia) and hepatopulmonary syndrome (HPS). The first patient was a 4-year-old male who did not receive any specific treatment and subsequently died of brain abscess 5 years after the diagnosis. The second patient was a 10-year-old female with a 5-year history of cyanosis and dyspnea on exertion. She had partial regression of hypoxemia and improved exercise tolerance at 8 months after a surgical shunt closure. The third patient was a 4-year-old male with a 3-year history of cyanosis and decreased exercise tolerance. He had full regression of hypoxemia at 3 months after a transcatheter shunt closure.
What is Known:
• Congenital extrahepatic portosystemic shunt (CEPS) is a rare cause of hepatopulmonary syndrome (HPS).
• There have been few reports in the literature about the management and outcome of HPS in children with CEPS.
What is New:
• CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences.
• Closure of portosystemic shunts may result in resolution of HPS in type II CEPS.
KeywordsAbernethy malformation Congenital extrahepatic portosystemic shunt (CEPS) hepatopulmonary syndrome (HPS) Portosystemic shunt
Activated partial thromboplastin time
Congenital extrahepatic portosystemic shunt
International normalized ratio
Inferior vena cava
Main portal vein
The research was supported by the Medical Guidance Project of Shanghai Science and Technology Commission (No. 14411965300).
Compliance with ethical standards
The research was supported by Medical Guidance Project of Shanghai Science and Technology Commission (No. 14411965300).
This article does not contain any studies with human participants or animals performed by any of the authors.
Contribution of each co-authors
Conceived and designed the experiments: Q.C., F.L. Performed the experiments: L.F., Q.W., J.W. Analyzed the data: Y.G., M.H. Contributed reagents/materials/analysis tools: T.L. Wrote the paper: L.F.
Conflict of interest
The authors declare that they have no conflict of interest.
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