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Congenital portosystemic shunts in children: recognition, evaluation, and management

O Bernard1, S Franchi-Abella, S Branchereau

  • 1Hépatologie pédiatrique, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.

Seminars in Liver Disease
|February 12, 2013
PubMed

Insights

Congenital portosystemic shunts, a rare condition in children, can lead to serious complications. Surgical or radiological closure is often effective, preserving liver function and minimizing the need for transplantation.

Area of Science:

  • Pediatric Surgery
  • Hepatology
  • Vascular Anomalies

Background:

  • Congenital portosystemic shunts (CPSS) affect 1 in 30,000 children.
  • CPSS are associated with severe complications including neonatal cholestasis, liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy.

Purpose of the Study:

  • To review the diagnosis, management, and outcomes of congenital portosystemic shunts in children.
  • To emphasize the effectiveness of shunt closure and the limited role of liver transplantation.

Main Methods:

  • Review of diagnostic modalities including prenatal ultrasonography, galactosemia screening, and abdominal ultrasound.
  • Discussion of interventional radiology and surgical techniques for shunt closure.
  • Assessment of liver revascularization potential post-closure.

Main Results:

  • Small intrahepatic shunts may spontaneously resolve within the first year of life.
  • Extrahepatic and persistent intrahepatic shunts require closure via interventional radiology or surgery.
  • The liver's portal system demonstrates plasticity, allowing revascularization after shunt closure.

Conclusions:

  • Congenital portosystemic shunts necessitate timely diagnosis and management.
  • Interventional and surgical closure techniques are highly effective, promoting liver revascularization.
  • Liver transplantation is rarely indicated for CPSS due to successful shunt management.

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