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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e31829fad46
Original Articles: Hepatology and Nutrition

Results of a Stepwise Approach to Extrahepatic Portal Vein Obstruction in Children

Alberti, Daniele*; Colusso, Mara*; Cheli, Maurizio*; Ravelli, Paolo; Indriolo, Amedeo; Signorelli, Sergio; Fagiuoli, Stefano; D’Antiga, Lorenzo§

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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.

© 2013 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,


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