Non-Cirrhotic Portal Hypertension in Children: Current Management Strategies.

Purpose of Review: Non-cirrhotic portal hypertension (NCPH) is primarily comprised of extrahepatic portal venous obstruction (EHPVO), followed by congenital hepatic fibrosis (CHF) and non-cirrhotic portal fibrosis (NCPF). Various complications besides variceal bleeding pose a management challenge. This review summarizes the best evidence-based management practices for children with NCPH. Recent Findings: Endotherapy in EHPVO eradicates esophageal varices but does not tackle problems like ectopic varices, portal biliopathy, massive splenomegaly, growth failure, etc. Meso-Rex shunt is a physiological shunt that ameliorates nearly all complications in EHPVO. In children with NCPF and isolated CHF, endotherapy remains the mainstay of management, with shunt surgery useful when indicated. Summary: Beyond variceal eradication, endotherapy has a limited role, and evaluation for meso-Rex shunt should be done at the beginning in EHPVO. Endotherapy constitutes the major therapeutic option in NCPF and CHF, except in a small subset who may require liver or a combined liver-kidney transplantation. [ABSTRACT FROM AUTHOR]