Current trends in pediatric moyamoya: a survey of international practitioners

Harishchandra Lalgudi Srinivasan, Moran Hausman-Kedem, Edward R. Smith, Shlomi Constantini, Jonathan Roth*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Objective: Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide. Methods: A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments. Results: One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers. Conclusion: This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.

Original languageEnglish
Pages (from-to)2011-2023
Number of pages13
JournalChild's Nervous System
Issue number6
StatePublished - Jun 2021


FundersFunder number
Aldo Jose F. da Silva
Alvaro Cordoba
Antonio Bellas
Arnold Palmer Hospital for Children
Artur Da Cunha
Bristol Institute of Clinical NeurosciencePsychiatry?Kodaira
Bristol Royal Hospital for Children, Bristol, UK)
Catherine Amlie-Lefond
Chiba Children's Hospital
Chief of Pediatric Neurosurgery, Director, Leon Pediatric Neuroscience Center of Excellence
Children?s Memorial Hermann Hospital Co-director of Pediatric Brain Tumor Program, University of Texas MD Anderson Cancer Center
Christian Medical College
Department of Neurological Sciences
Department of Neurology and Center for Genomic Medicine
Department of Neurosurgery
Department of Neurosurgery - Meyer Pediatric Hospital
Department of Neurosurgery Hospital Sant Joan de D?u
Department of Neurosurgery Tianjin Medical University
Department of Neurosurgery, Geneva University Hospitals
Department of Neurosurgery, Shahid Sheikh Abu Naser Hospital
Division of Neurosurgery
Division of Ophthalmology, Tel Aviv Sourasky medical center
Division of Pediatric Neurosurgery, Kitasato University School of Medicine, Tokyo
Division of Pediatric Neurosurgery, Kitasato University School of Medicine, Tokyo, Japan
Division of Pediatric Neurosurgery, Santa M?nica Maternity School
Dr Danil Kozyrev
Dr Marnie Rose Professorship in Pediatric Neurosurgery Departments of Pediatric Surgery and Neurosurgery
Enrico Ghizoni MD
Frederick Boop MD
George Washington University School of Medicine
Hamilton Matushita
Hiroaki Sakamoto (Department of Pediatric Neurosurgery
Instituto Fernandes Figueira
Instituto Nacional de C?ncer
Instituto de Neurocirug?a Asenjo
Instituto de Neurocirugia
Johns Hopkins School of Medicine, Department of Neurology
Leopoldo Mandic Ferreira Furtado
Lu Ma (Dept. of Neurosurgery
Matthieu Vinchon
Medipol University Dept. of Neurosurgery
Megan Barry
Miyagi Children's Hospital
National Institute of Women, Children and Adolescents Health Fernandes Figueira
Nejat Akalan
Osaka City General Hospital
Paola Pergami
Paolo Frassanito
Princess Maxima Center for Pediatric Oncology, department of pediatric oncology neurosurgery
Rebecca Ichord MD
Republican scientific and practical center of neurosurgery Belarus Minsk
Ronan Vieira
Sang-Dae KIM
Satoshi Ihara
Satoshi Kuroda
Selcuk University
Sudipta Kumer Mukherjee
Suman Ghosh MD
Talabaev Mikle
Tokyo Metropolitan Children's Medical Center
Tomomi Kimiwada
Toyama University Hospital
Valley Hospital
Vijeya Ganesan
Massachusetts General Hospital
Harvard Medical School
Children's Hospital Colorado
Health Science Center, University of Tennessee
Śląski Uniwersytet Medyczny
University of Washington
Seattle Children’s Hospital
European Paediatric Neurology Society
University of Alabama
College of Medicine, University of Cincinnati
McGovern Medical School
College of Medicine, Seoul National University
University of Alberta
University College London
University of Pretoria
Ain Shams University
University of Toronto
Mahidol University
Seoul National University Hospital
Kyungpook National University Hospital
Sichuan University
Universidade de São Paulo
Hospital for Sick Children
Radboud Universitair Medisch Centrum
Fundação Oswaldo Cruz
Taipei Veterans General Hospital
West China Hospital, Sichuan University
Centre hospitalier régional universitaire de Lille
Korea University Anam Hospital


    • Pediatric moyamoya
    • moyamoya lifestyle modifications
    • moyamoya survey
    • moyamoya treatment
    • pediatric stroke


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