Review
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Oct 27, 2021; 13(10): 1269-1288
Published online Oct 27, 2021. doi: 10.4254/wjh.v13.i10.1269
Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations
Moinak Sen Sarma, Jayendra Seetharaman
Moinak Sen Sarma, Jayendra Seetharaman, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Author contributions: Sarma MS performed the conception, final drafting of the manuscript; Seetharaman J performed the primary drafting of the manuscript.
Conflict-of-interest statement: Authors have no conflict of interest to disclose.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, MD, Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India. moinaksen@gmail.com
Received: April 30, 2021
Peer-review started: April 30, 2021
First decision: June 15, 2021
Revised: June 27, 2021
Accepted: September 19, 2021
Article in press: September 19, 2021
Published online: October 27, 2021
Processing time: 174 Days and 18.5 Hours
Core Tip

Core Tip: The review discusses the natural history, endoscopic outcome, and management of non-cirrhotic causes of portal hypertension in children, especially in resource constraint developing nations. Extrahepatic portal vein obstruction is the most common cause of portal hypertension in developing countries. Endoscopic variceal ligation and sclerotherapy effectively eradicate the esophageal varices. Other complications require shunt surgery that ultimately reverses portal hypertension. Non-cirrhotic portal fibrosis has favourable outcomes in terms of variceal bleeding and mortality. Isolated congenital hepatic fibrosis (CHF) has a relatively good outcome. Liver transplantation is required when CHF is associated with Caroli’s disease, recurrent cholangitis, and decompensation. The presence of significant renal disease requires combined liver and kidney transplantation.