Paulin S, Rajakumar A, Menon J, Shanmugam N, Rela M. Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation. World J Transplant 2026; 16(1): 111122 [DOI: 10.5500/wjt.v16.i1.111122]
Corresponding Author of This Article
Akila Rajakumar, FRCA, Head, Department of Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Rela Institute, 7 CLC Works Road, Chromepet, Chennai 600044, Tamil Nadu, India. drakila.rajakumar@gmail.com
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Anesthesiology
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Minireviews
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This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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/
Mar 18, 2026 (publication date) through Jan 14, 2026
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World Journal of Transplantation
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2220-3230
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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA
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Paulin S, Rajakumar A, Menon J, Shanmugam N, Rela M. Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation. World J Transplant 2026; 16(1): 111122 [DOI: 10.5500/wjt.v16.i1.111122]
World J Transplant. Mar 18, 2026; 16(1): 111122 Published online Mar 18, 2026. doi: 10.5500/wjt.v16.i1.111122
Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation
Susan Paulin, Akila Rajakumar, Jagadeesh Menon, Naresh Shanmugam, Mohamed Rela
Susan Paulin, Liver Transplant Anaethesia and Critical Care, Dr. Rela Institute and Medical Centre, Chennai 600044, Tamil Nādu, India
Akila Rajakumar, Department of Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Rela Institute, Chennai 600044, Tamil Nadu, India
Jagadeesh Menon, Institute of Liver Disease and Transplantation, Dr. Rela Institute and Medical Centre, Bharath Institute of Higher Education and Research, Chennai 600044, India
Naresh Shanmugam, Department of Paediatric Hepatology, Rela Institute, Chennai 600044, Tamil Nādu, India
Mohamed Rela, Institute of Liver Disease and Transplantation, Dr. Rela Institute and Medical Centre, Chennai 600044, India
Co-first authors: Susan Paulin and Akila Rajakumar.
Author contributions: All authors contributed to the conception and design of the review; Paulin S and Rajakumar A performed the literature search and synthesized the findings and drafted the initial manuscript; Menon J, Rela M, and Shanmugam N provided critical feedback on the manuscript's structure and content; all authors reviewed and approved the final manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts 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: Akila Rajakumar, FRCA, Head, Department of Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Rela Institute, 7 CLC Works Road, Chromepet, Chennai 600044, Tamil Nadu, India. drakila.rajakumar@gmail.com
Received: June 27, 2025 Revised: July 25, 2025 Accepted: November 5, 2025 Published online: March 18, 2026 Processing time: 201 Days and 15.1 Hours
Core Tip
Core Tip: Most metabolic disorders are liver-based, and liver transplantation (LT) has emerged as an effective cure for a variety of inherited metabolic disorders in the pediatric population. LT has been shown to offer a cure, deter disease progression and provide symptomatic improvement in patients with inherited metabolic disorders. Perioperative management of these children presenting for LT with inborn errors of metabolism requires disease specific and patient specific strategies in all aspects right from preoperative assessment, intraoperative and postoperative management compared to those presenting with other indications. Knowledge about the distribution of deficient enzymes, the percentage of enzymes replaced by LT, and the extent of extrahepatic involvement helps anticipate and manage complications in the perioperative period.
