Results of a stepwise approach to extrahepatic portal vein obstruction in children

BACKGROUND:: The management of extrahepatic portal vein obstruction (EHPVO) in children is controversial. We report our experience with a prospective evaluation of a stepwise protocol based on severity of portal hypertension and feasibility of mesoportal bypass (MPB). METHODS:: After diagnosis, children with EHPVO underwent surveillance endoscopies and received nonselective β-blockers (NSBBs) or endoscopic variceal obliteration (EVO) when large varices were detected. In patients who failed NSBBs and EVO, we considered MPB as first-line and shunts or transjugular intrahepatic portosystemic shunt (TIPS) as second-line options. RESULTS:: Sixty-five children, median age 12.5 (range 1.6-25.8), whose age at diagnosis was 3.5 (0.2-17.5) years, were referred to our unit. Forty-three (66%) had a neonatal illness, 36 (55%) an umbilical vein catheterisation. Thirty-two (49%) presented with bleeding at a median age of 3.8 years (0.5-15.5); during an 8.4-year follow-up period (1-16), 43 (66%) had a bleeding episode, 52 (80%) were started on NSBBs, 55 (85%) required EVO, and 33 (51%) required surgery or TIPS. The Rex recessus was patent in 24 of 54 (44%), negatively affected by a history of umbilical catheterisation (P=0.01). Thirty-four (53%) patients underwent a major procedure: MPB (13), proximal splenorenal (13), distal splenorenal (2), mesocaval shunt (3), TIPS (2), and OLT (1). At the last follow-up, 2 patients died, 53 of 57 (93%) are alive with bleeding control, 27 of 33 (82%) have a patent conduit. CONCLUSIONS:: Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.