摘要
Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in children.Variceal bleed and splenomegaly are their predominant presentation.Laboratory features show cytopenias(hypersplenism)and preserved hepatic synthetic functions.Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases.After variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO.Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate.Shunt surgeries prevent rebleed,improve growth and QoL.Non-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade,massive splenomegaly and patent portal vein are discriminating features of NCPF.Shunt surgery is required in severe cases when endotherapy is insufficient for the varices.Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly.Ductal plate malformation forms the histological hallmark of CHF.CHF is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy.Liver transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s syndrome.Combined liver-kidney transplantation is indicated when both liver and renal issues are present.
