Congenital portosystemic shunts (CPSS) are rare malformations, which connect the portal venous system and the systemic circulation. The disorder is discovered at prenatal screening, neonatal cholestasis as an incidental finding or by systemic complications such as pulmonary hypertension, encephalopathies or liver nodules. CPSS are associated to cardiac malformations and several syndromes. Intervention radiology plays a key role in treatment by closure of the shunt, which is indicated, if the patient has complications, the shunt is extrahepatic, or it is still persistent at two years of age, using a two-step approach, as summarised and discussed in this review.

Bidragets oversatte titelDiagnosis and treatment of a multisystemic disorder in children called congenital portosystemic shunts
TidsskriftUgeskrift for Laeger
Udgave nummer31
StatusUdgivet - 2 aug. 2021


  • Child
  • Female
  • Heart Defects, Congenital
  • Humans
  • Hypertension, Pulmonary
  • Infant, Newborn
  • Portal Vein/diagnostic imaging
  • Portasystemic Shunt, Transjugular Intrahepatic
  • Pregnancy
  • Vascular Malformations


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