53 LONG-TERM OUTCOMES OF EXTRAHEPATIC PORTAL VEIN OBSTRUCTION – A REGISTRY BASED FOLLOW-UP STUDY
Topi Luoto, Antti Koivusalo, Risto Rintala, Mikko Pakarinen
Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Central Hospital, University of Helsinki, Helsinki, Finland

Abstract

Aim of the study: Extrahepatic portal vein obstruction (EHPVO) is one of the main causes of childhood portal hypertension. We analyzed long-term outcomes of an institutional EHPVO cohort with or without shunt surgery.

Methods: After ethical approval 54 consecutive patients with EHPVO diagnosed between 1987 and 2017 were followed up through national health care registries and hospital records.

Main results: Median follow-up age was 17.6 (range 3.4-45) years. None of the patients were lost to follow-up or died. Esophageal varices (n=53) were diagnosed at the age of 5.5 (range 0.3-13) years. Patients underwent 9.5 (range 1-55) gastroscopies and 6 (range 0-29) sclerotherapies. Thirty-one (57%) patients had variceal bleeding (median 1, range 1-17) necessitating Sengstaken-Blakemore tube in three (6%). Overall, 39 (72%) patients underwent 46 shunt procedures with 82% (32/39) patency rate at the end of follow-up: Rex 72% (21/29), Warren 100% (4/4), side-to-side splenorenal 80% (4/5), proximal splenorenal 50% (1/2), mesocaval 25% (1/4), other 100% (2/2). Nine (17%) patients had undergone splenectomy (n=8) or splenic embolization (n=1). During the last five follow-up years 4 (7%) patients had experienced variceal bleeding episodes: 0/32 with patent shunt, 1/7 with occluded shunt and 3/15 without shunt (p=0.03). Resolution of hypersplenism was comparable after different shunt procedures (Rex 90% vs non-Rex 78%, p=0.6), although Rex shunt increased platelet levels more effectively [101 (6-233) vs 67 (-29-110) E9/L, p=0.04]. Five (9%) patients developed symptomatic portal biliopathy at the age of 22 (14-25) years. All of them had undergone splenectomy (5/8 vs 0/46, p<0.01), and had no shunt or occluded shunt (5/22 vs 0/32, p<0.01).

Conclusion: Different surgical shunts prevented variceal bleeding slightly better than endoscopic management in the long term, while Rex shunt alleviated hypersplenism most effectively. Splenectomy and the absence of a functioning shunt associated with development of portal biliopathy.


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