Durga Prasad1, Moinak Sen Sarma2, Surender Kumar Yachha1, Anshu Srivastava1, Ujjal Poddar1. 1. Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India. 2. Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India. moinaksen@yahoo.com.
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.
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.
Authors: Don C Rockey; Stephen H Caldwell; Zachary D Goodman; Rendon C Nelson; Alastair D Smith Journal: Hepatology Date: 2009-03 Impact factor: 17.425