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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§

Journal of Pediatric Gastroenterology and Nutrition: November 2013 - Volume 57 - Issue 5 - p 619–626
doi: 10.1097/MPG.0b013e31829fad46
Original Articles: Hepatology and Nutrition

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.

*Paediatric Surgery

Gastroenterology Unit

Liver Unit

§Paediatric Hepatology, Gastroenterology, and Transplantation. Hospital Papa Giovanni XXIII, Bergamo, Italy.

Address correspondence and reprint requests to Lorenzo D’Antiga, Paediatric Hepatology, Gastroenterology and Transplantation, Azienda Ospedaliera Papa Giovanni XXIII, Piazza Oms 1, 24127 Bergamo, Italy (e-mail:

Received 29 January, 2013

Accepted 6 June, 2013

The authors report no conflicts of interest.

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