An international multicenter analysis of the outcome of pediatric first variceal hemorrhage

Background & Aims: Management of portal hypertension in children including primary prophylaxis of variceal bleeding is controversial due to limited understanding of mortality risk after first variceal hemorrhage (1VH). This international multicenter study was designed to advance understanding of outcomes after pediatric 1VH. Approach & Results: Center-specific characteristics and approaches to portal hypertension were determined at 44 international sites. K-means clustered the centers based upon volume and complexity of approaches [cluster 1 high (n=5), cluster 2 low (n=21), cluster 3 intermediate (n=18]). Clinical features and outcomes after 1VH were available from 345 subjects. Factors associated with overall survival (OS) and survival with native liver (SNL) were estimated by Kaplan-Meier survival curves, log rank tests, univariable and multivariable Cox regression models. OS and SNL were high in portal vein thrombosis and noncirrhotic diseases and significantly higher than biliary atresia and cirrhotic diseases ( p <.001). Diminished SNL was observed in decompensated disease [diuretics (i.e. ascites) or hospitalization at 1VH], biliary atresia with poor bile flow, cirrhotic disease, after sclerotherapy or no endoscopic intervention. Diminished OS was noted in those with cirrhosis or requiring sclerotherapy. 6-week mortality was determined and compared among clusters using average treatment effect under the Rubin causal model. Overall 6-week mortality was 4.1%, 1.4% in nonhospitalized subjects and was significantly higher in cluster 2 versus 1 centers ( P =.010). Conclusions: Immediate mortality after 1VH in children is low compared to adults. Outcomes after 1VH are diagnosis-specific and worse in decompensated disease and after sclerotherapy. Center-specific factors may influence immediate outcomes after 1VH.

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Journal
Hepatology
Published
2026-10-07
DOI
https://doi.org/10.1097/hep.0000000000001881
Primary Topic
Liver Disease and Transplantation
Type
article
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article

An international multicenter analysis of the outcome of pediatric first variceal hemorrhage

Giuseppe Indolfi, Suporn Treepongkaruna, Amy G. Feldman, Mercedes Martínez et al.
Hepatology
Liver Disease and Transplantation
article

An international multicenter analysis of the outcome of pediatric first variceal hemorrhage

Giuseppe Indolfi, Suporn Treepongkaruna, Amy G. Feldman, Mercedes Martínez, Jane Hartley, Tassos Grammatikopoulos, Benjamin L. Shneider, Angelo Di Giorgio, Mohit Kehar, Ryuji Komine, Pamela L. Valentino, Songpon Getsuwan, Denise Aldrian, Winita Hardikar, Jean Pappas Molleston, Mureo Kasahara, Hubert P. J. van der Doef, Oanez Ackermann, Voytek Slowik, Orith Waisbourd‐Zinman, Marianna Durilová, James E. Squires, Antoine Gardin, Scott A. Elisofon, Carolina Jiménez‐Rivera, Alexis J. Gumm, Matjaž Homan, Samar H. Ibrahim, Kulkarni Sakil, Eyal Shteyer, Lee M. Bass, Keith Z. Hazleton, AGAF Roberto de Franchis, Jesús Quintero-Bernabeu, Kexin Guo, Simon Lam, Catalina Jaramillo, Thomai Kotsou, Yael Mozer‐Glassberg, Wikrom Karnsakul, Anita K. Pai, Pooja Reddy Spector, Chaitri Desai, Elizabeth Bannister, Maria Mercadal-Hally, Anja Praprotnik Novak, Rima Fawaz, Georg Friedrich 1798-1836 Vogel, Rustam Yuldashev, Oleg Godik, Kee-Seang Chew, Deborah Goldman, Sara Kathryn Smith, Alexandre Ferreira, Jordache Taylor, Julio Pimenta, Amal Aqul, Rodrigo Vázquez-Frias, Simona Carrera, Katherine Sweeny, Sarah Berkenfeld, Way-Seah Lee, Sarah Bedoyan, Riccardo Superina, Dariia Voroniak, Shinya Yokoyama, Xiaotian Gao, César Ulises Amaro-Reynoso, JC Gana
article en

Abstract

Background & Aims: Management of portal hypertension in children including primary prophylaxis of variceal bleeding is controversial due to limited understanding of mortality risk after first variceal hemorrhage (1VH). This international multicenter study was designed to advance understanding of outcomes after pediatric 1VH. Approach & Results: Center-specific characteristics and approaches to portal hypertension were determined at 44 international sites. K-means clustered the centers based upon volume and complexity of approaches [cluster 1 high (n=5), cluster 2 low (n=21), cluster 3 intermediate (n=18]). Clinical features and outcomes after 1VH were available from 345 subjects. Factors associated with overall survival (OS) and survival with native liver (SNL) were estimated by Kaplan-Meier survival curves, log rank tests, univariable and multivariable Cox regression models. OS and SNL were high in portal vein thrombosis and noncirrhotic diseases and significantly higher than biliary atresia and cirrhotic diseases ( p <.001). Diminished SNL was observed in decompensated disease [diuretics (i.e. ascites) or hospitalization at 1VH], biliary atresia with poor bile flow, cirrhotic disease, after sclerotherapy or no endoscopic intervention. Diminished OS was noted in those with cirrhosis or requiring sclerotherapy. 6-week mortality was determined and compared among clusters using average treatment effect under the Rubin causal model. Overall 6-week mortality was 4.1%, 1.4% in nonhospitalized subjects and was significantly higher in cluster 2 versus 1 centers ( P =.010). Conclusions: Immediate mortality after 1VH in children is low compared to adults. Outcomes after 1VH are diagnosis-specific and worse in decompensated disease and after sclerotherapy. Center-specific factors may influence immediate outcomes after 1VH.

Hepatology
Northwestern University (US), University of Southern California (US), Children's Hospital of Wisconsin (US), Boston Children's Hospital (US), Seattle Children's Hospital (US), Cleveland Clinic (US), Royal Children's Hospital (AU), Primary Children's Hospital (US), Children's Mercy Hospital (US), Mayo Clinic (US), University Medical Center Groningen (NL), Children's Hospital of Los Angeles (US), Innsbruck Medical University (AT), Johns Hopkins University (US), Inserm (FR), Pontificia Universidad Católica de Chile (CL), King's College - North Carolina (US), University of Calgary (CA), Baylor College of Medicine (US), University of Milan (IT), Universität Innsbruck (AT), University of Washington (US), Washington University in St. Louis (US), Université Paris-Saclay (FR), Children's Hospital of Eastern Ontario (CA), Johns Hopkins Medicine (US), Ljubljana University Medical Centre (SI), Shaare Zedek Medical Center (IL), Riley Hospital for Children (US), Bikur Cholim Hospital (IL), Yale University (US), University of Malaya (MY), Children's Hospital Colorado (US), Hospital Infantil de México Federico Gómez (MX), University Hospital in Motol (CZ), Birmingham Women's Hospital (GB), Ospedale Papa Giovanni XXIII (IT), Schneider Children's Medical Center (IL), Meyer Children's Hospital (IT), Wits University Donald Gordon Medical Centre (ZA), Vall d'Hebron Hospital Universitari (ES), Children's Hospital of Pittsburgh (US), National Center For Child Health and Development (JP), Hospital das Clínicas da Universidade Federal de Minas Gerais (BR), Ramathibodi Hospital (TH), Texas Children's Hospital (US), King's College Hospital (GB), Tashkent Pediatric Medical Institute (UZ), Bicêtre Hospital (FR), Children's Specialized Hospital (US), Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Indiana University – Purdue University Indianapolis (US), Nagoya University (JP), Columbia University (US), The University of Texas Southwestern Medical Center (US), Vanderbilt University Medical Center (US), University of Colorado Denver (US)
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Liver Disease and Transplantation
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