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Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia

Beverley Newman1, Jeffrey A Feinstein, Ronald A Cohen

  • 1Department of Radiology, Stanford University School of Medicine, Lucile Packard Children's Hospital, 725 Welch Road, Stanford, CA 94305, USA. bev.newman@stanford.edu

Pediatric Radiology
|January 14, 2010
PubMed

Insights

Congenital extrahepatic portosystemic shunts (CEPS) in heterotaxy with polysplenia can cause pulmonary hypertension. Successful percutaneous closure of CEPS resolved symptoms in children with patent portal veins.

Area of Science:

  • Vascular Surgery
  • Pediatric Cardiology
  • Medical Imaging

Background:

  • Heterotaxy with polysplenia frequently co-occurs with cardiovascular anomalies.
  • Congenital extrahepatic portosystemic shunts (CEPS) are an underdiagnosed anomaly in this population.
  • Failure to identify CEPS can result in suboptimal patient management.

Observation:

  • Three pediatric patients presented with cyanosis and pulmonary hypertension.
  • All patients had heterotaxy with polysplenia and absence of the intrahepatic inferior vena cava (IVC).
  • Large, splenorenal portosystemic shunts and pulmonary arterial dilatation were noted, indicative of CEPS.

Findings:

  • The study identified CEPS, specifically Abernethy malformation, in conjunction with heterotaxy and polysplenia.
  • Patients exhibited portopulmonary syndrome due to shunting.
  • Portal and hepatic vein patency were crucial for successful percutaneous shunt closure.

Implications:

  • Early diagnosis of CEPS in heterotaxy with polysplenia is vital for appropriate treatment.
  • Percutaneous closure of CEPS is a feasible and effective treatment option when portal veins are patent.
  • Identifying CEPS prevents misdiagnosis and guides effective therapeutic strategies, improving patient outcomes.
Abstract

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