Long-Term Outcomes of Extrahepatic Portal Vein Obstruction—A Nationwide Registry-Based Follow-Up Study From 1987 to 2023

Abstract

Background and Aims: Extrahepatic portal vein obstruction (EHPVO) is among the main causes of childhood portal hypertension. We analysed nationwide long-term outcomes of EHPVO in relation to various management options. Methods: In total, 62 consecutive patients with EHPVO diagnosed between 1987 and 2023 were followed up through hospital records and national health care registries. Results: Median follow-up age was 18.8 (13.1–27.1) years. Oesophageal varices (n = 60, 97%) were diagnosed at median age 5.5 (3.4–7.6) years. Overall, patients underwent 9 (4–15) gastroscopies and 6 (4–10) sclerotherapies/band ligations. Thirty-six (58%) patients experienced median 1 (1–3) variceal bleeds. Forty-nine (79%) patients underwent 56 shunt procedures with overall 86% (42/49) patency rate. Patients receiving primary mesoportal bypass (n = 36) since 2002, underwent median 8 (4–12) upper endoscopies compared to 25 (9–27) among those without shunt surgery (n = 13, p = 0.01). During the last 5 years of follow-up 3 (5%) patients experienced variceal bleeding: 1/49 after shunt surgery and 2/13 without shunt surgery (p = 0.09). Thrombocytopenia had resolved in 76% of patients with patent shunts compared to 33% with occluded shunts. Six (10%) patients developed symptomatic portal hypertensive biliopathy at median age 18.8 (12.0–24.2) years, none of whom had a functioning shunt (6/6 vs. 41/56, p < 0.01) and 5 of whom had undergone splenectomy (5/6 vs. 3/56, p < 0.01). Conclusions: Surgical shunts prevented variceal bleeding effectively in the long-term, and mesoportal bypass reduced the number of endoscopic interventions. Previous splenectomy and absence of a functioning shunt were associated with the development of portal hypertensive biliopathy.