Pediatric non-cirrhotic portal fibrosis: role of endoscopic management in determining long-term outcome

Non-cirrhotic portal fibrosis (NCPF) is a rare cause of pediatric portal hypertension. There is abundant literature in adults but paucity of data in children. We aimed to evaluate and compare the endoscopic and long-term outcomes of children with NCPF.

Consecutive children (≤ 18 years) diagnosed with NCPF evaluated for clinical and endoscopic profile and outcome. The cohort underwent 3 weekly endoscopic sessions until esophageal variceal eradication followed by 6–12 monthly endoscopic surveillance. Non-bleeders and bleeders were compared for endoscopic outcome.

Forty-five NCPF children with median age of 14.5 (6–18) years and symptom duration 12 (1–120) months presented with spleen-related issues (78%), esophageal varices (96%), primary gastric varices (56%), and portal hypertensive gastropathy (89%). Thirty-three patients undergoing endotherapy (secondary prophylaxis n = 22, primary prophylaxis n = 11) showed primary eradication of varices after 5 (2–12) sessions. 36% showed recurrence of esophageal varices in 11 (6–42) months and secondary gastric varices developed in 12%. Overall 87% patients required endoscopic intervention at onset or follow-up. Poor outcome was observed in ten patients (n = 9 bleeders). Three children died of variceal bleeding before endoscopic eradication of esophageal varices. Three developed decompensation after a median follow-up of 48 (3–120) months and referred for liver transplantation. Four patients required surgery for portal hypertension.

Overall, endoscopic outcome of NCPF is favorable. One-third patients have recurrence of esophageal varices. Small proportion of bleeders have poor outcome.