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

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.