Pediatric Non-cirrhotic Portal Fibrosis: Role of Endoscopic Management in Determining Long-term Outcome

Overview

Journal Hepatol Int

Publisher Springer

Specialty Gastroenterology

Date 2019 Aug 31

PMID 31468295

Citations 4

Authors

Durga Prasad

Moinak Sen Sarma

Surender Kumar Yachha

Anshu Srivastava

Ujjal Poddar

Affiliations

Soon will be listed here.

Abstract

Aims: 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.

Methods: 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.

Results: 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.

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

Citing Articles

Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.

Shukla A, Rockey D, Kamath P, Kleiner D, Singh A, Vaidya A Hepatol Int. 2024; 18(6):1684-1711.

PMID: 39546143 DOI: 10.1007/s12072-024-10739-6.

Endoscopic Primary Prophylaxis to Prevent Bleeding in Children with Esophageal Varices: A Systematic Review and Meta-Analysis.

Alatas F, Monica E, Ongko L, Kadim M Pediatr Gastroenterol Hepatol Nutr. 2023; 26(5):231-238.

PMID: 37736217 PMC: 10509019. DOI: 10.5223/pghn.2023.26.5.231.

Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations.

Sen Sarma M, Seetharaman J World J Hepatol. 2021; 13(10):1269-1288.

PMID: 34786165 PMC: 8568571. DOI: 10.4254/wjh.v13.i10.1269.

Long-term outcome of pediatric non-cirrhotic portal fibrosis from the viewpoint of endoscopic profile.

Kotani K, Kawada N Hepatol Int. 2020; 14(2):164-166.

PMID: 32170597 DOI: 10.1007/s12072-020-10028-y.

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