title: Clinical features of congenital portosystemic shunt in children.
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.
  
  
  CONCLUSIONS
  Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.
authors.affiliation: Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Myung Jin
authors.initials: MJ
authors.lastname: Kim
authors.forename: Jae Sung
authors.initials: JS
authors.lastname: Ko
authors.forename: Jeong Kee
authors.initials: JK
authors.lastname: Seo
authors.forename: Hye Ran
authors.initials: HR
authors.lastname: Yang
authors.forename: Ju Young
authors.initials: JY
authors.lastname: Chang
authors.forename: Gi Beom
authors.initials: GB
authors.lastname: Kim
authors.forename: Jung-Eun
authors.initials: JE
authors.lastname: Cheon
authors.forename: Woo Sun
authors.initials: WS
authors.lastname: Kim
mesh_terms: D002648:Child; D002675:Child, Preschool; D004621:Embolization, Therapeutic; D005260:Female; D005500:Follow-Up Studies; D006503:Hepatic Veins / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D014682:Vena Cava, Inferior / Q000002:abnormalities*