Clinical features of congenital portosystemic shunt in children

Research output: Contribution to journalReview article

29 Scopus citations

Abstract

Clinical features, images, complications, treatments, and prognosis of 10 children with congenital portosystemic shunt (CPSS) were reviewed. Nine children were diagnosed with intrahepatic shunts while one presented with extrahepatic shunt. CPSS was detected by prenatal ultrasonography in four infants. Three infants presented with galactosemia without an enzyme deficiency. Two children presented with mental retardation and attention deficit hyperactivity disorder. Pulmonary hypertension developed in two patients. Spontaneous closure occurred in four infants with intrahepatic shunts including patent ductus venosus. The shunts were closed using transcatheter embolizations in four patients with intrahepatic shunts. Conclusion: Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.

Original languageEnglish
Pages (from-to)395-400
Number of pages6
JournalEuropean Journal of Pediatrics
Volume171
Issue number2
DOIs
StatePublished - Feb 2012

Keywords

  • Congenital portosystemic shunt
  • Embolization
  • Galactosemia
  • Hyperammonemia
  • Pulmonary hypertension

Cite this