An international multicenter analysis of the outcome of pediatric first variceal hemorrhage
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.
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Authors: Benjamin L. Shneider, Xiaotian Gao, Kexin Guo, Catalina Jaramillo, Jean Pappas Molleston, Julio Pimenta, Riccardo Superina, Alexandre Ferreira, Alexis J. Gumm, Amal Aqul, Amy G. Feldman, Angelo Di Giorgio, Anita K. Pai, Anja Praprotnik Novak, Antoine Gardin, Carolina Jiménez‐Rivera, César Ulises Amaro-Reynoso, Chaitri Desai, Dariia Voroniak, Denise Aldrian, Elizabeth Bannister, Eyal Shteyer, Georg Friedrich 1798-1836 Vogel, Giuseppe Indolfi, Hubert P. J. van der Doef, James E. Squires, Jane Hartley, JC Gana, Jesús Quintero-Bernabeu, Jordache Taylor, Katherine Sweeny, Kee-Seang Chew, Keith Z. Hazleton, Lee M. Bass, Maria Mercadal-Hally, Marianna Durilová, Matjaž Homan, Mercedes Martínez, Mohit Kehar, Mureo Kasahara, Oleg Godik, Orith Waisbourd‐Zinman, Pamela L. Valentino, Pooja Reddy Spector, Rima Fawaz, Rodrigo Vázquez-Frias, Rustam Yuldashev, Ryuji Komine, Kulkarni Sakil, Samar H. Ibrahim, Sarah Bedoyan, Sara Kathryn Smith, Scott A. Elisofon, Simon Lam, Shinya Yokoyama, Simona Carrera, Songpon Getsuwan, Suporn Treepongkaruna, Sarah Berkenfeld, Thomai Kotsou, Deborah Goldman, Voytek Slowik, Way-Seah Lee, Wikrom Karnsakul, Winita Hardikar, Yael Mozer‐Glassberg, AGAF Roberto de Franchis, Oanez Ackermann, Tassos Grammatikopoulos
Institutions: University of Washington, Northwestern University, Robert H. Lurie Comprehensive Cancer Center of Northwestern University, University of Colorado Denver, Columbia University, Johns Hopkins University, King's College - North Carolina, University Medical Center Groningen, Children's Hospital of Eastern Ontario, University of Calgary, University of Southern California, Ramathibodi Hospital, Pontificia Universidad Católica de Chile, Inserm, Washington University in St. Louis, Yale University, Indiana University – Purdue University Indianapolis, Riley Hospital for Children, Johns Hopkins Medicine, Innsbruck Medical University, Universität Innsbruck, Royal Children's Hospital, Nagoya University, Vall d'Hebron Hospital Universitari, Children's Mercy Hospital, The University of Texas Southwestern Medical Center, Université Paris-Saclay, University of Milan, University of Malaya, Children's Hospital of Los Angeles, Mayo Clinic, Ljubljana University Medical Centre, King's College Hospital, Meyer Children's Hospital, University Hospital in Motol, Schneider Children's Medical Center, Boston Children's Hospital, Baylor College of Medicine, Vanderbilt University Medical Center, Cleveland Clinic, Tashkent Pediatric Medical Institute, Bicêtre Hospital, Children's Hospital Colorado, Hospital das Clínicas da Universidade Federal de Minas Gerais, Texas Children's Hospital, Children's Specialized Hospital, Ospedale Papa Giovanni XXIII, Primary Children's Hospital, Shaare Zedek Medical Center, National Center For Child Health and Development, Children's Hospital of Pittsburgh, Hospital Infantil de México Federico Gómez, Seattle Children's Hospital, Birmingham Women's Hospital, Children's Hospital of Wisconsin, Bikur Cholim Hospital, Wits University Donald Gordon Medical Centre