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World J Hepatol. Nov 27, 2023; 15(11): 1174-1187
Published online Nov 27, 2023. doi: 10.4254/wjh.v15.i11.1174
Budd-Chiari syndrome in children: Challenges and outcome
Arghya Samanta, Moinak Sen Sarma, Rajanikant Yadav
Arghya Samanta, Moinak Sen Sarma, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Rajanikant Yadav, Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
Author contributions: Samanta A contributed to literature review, analysis, data collection and interpretation, and drafting of the initial manuscript; Sen Sarma M contributed to the conception and design of the manuscript, interpretation of the data, and critical revision of the initial manuscript; Yadav R contributed to interpretation of the data and the critical revision of the initial manuscript; all the authors approved the final version of the manuscript.
Conflict-of-interest statement: All the authors declare no conflict of interest for this article.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Moinak Sen Sarma, Doctor, MBBS, MD, Adjunct Associate Professor, Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow-226014, Lucknow 226014, Uttar Pradesh, India. moinaksen@yahoo.com
Received: July 29, 2023
Peer-review started: July 29, 2023
First decision: September 27, 2023
Revised: October 8, 2023
Accepted: October 30, 2023
Article in press: October 30, 2023
Published online: November 27, 2023
Abstract

Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.

Keywords: Budd-Chiari syndrome, Radiological endovascular intervention, Transjugular intrahepatic porto-systemic shunt, Direct intrahepatic porto-systemic shunt, Liver stiffness, Splenic stiffness, Shear-wave elastography

Core Tip: Budd-Chiari syndrome (BCS) is a rare disease of the liver, characterised by obstruction of hepatic venous outflow tract. The effectiveness of radiological endovascular interventions in alleviating clinical symptoms as well as hepatic congestion has been shown both in adults and children. However, unlike in adults, established treatment guidelines have not been developed in children with BCS. Long-term follow-up studies including the prevalence of hepatopulmonary syndrome and hepatocellular carcinoma in this patient population are lacking in children. The role of liver and splenic stiffness measurement by elastography techniques is poorly studied in patients with BCS.