Non-Cirrhotic Portal Hypertension in Children: Current Management Strategies

AbstractPurpose of ReviewNon-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 FindingsEndotherapy 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.SummaryBeyond 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.
Source: Current Hepatitis Reports - Category: Infectious Diseases Source Type: research