Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.
Frank DiPaolaAndrew T TroutAshley E WaltherAnita GuptaRachel SheridanKathleen M CampbellGreg TiaoJorge A BezerraKevin E BoveManish PatelJaimie D NathanPublished in: Digestive diseases and sciences (2019)
CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.