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11.09.2024 | Research

Experience of an interdisciplinary management for pediatric Moyamoya disease: application of a novel Hemispheric Surgical Score

verfasst von: Santiago Ezequiel Cicutti, Guido Patricio Gromadzyn, María Celeste Buompadre, Carlos Rugilo, Flavio Requejo, María Laura Gonzalez Dutra, Javier Danilo Gonzalez Ramos, Sebastián Gastón Jaimovich

Erschienen in: Child's Nervous System | Ausgabe 12/2024

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Abstract

Purpose

The primary purpose of this study was to develop and implement a novel Hemispheric Surgical Score to guide the treatment of pediatric patients with Moyamoya disease (MMD). Additionally, we aimed to describe a comprehensive flowchart for the evaluation, treatment, and follow-up of these patients and to share our experience with the interdisciplinary management of a large pediatric cohort at a referral pediatric hospital.

Methods

We conducted a retrospective observational study using medical records of patients diagnosed with MMD at the Pediatric Hospital “Prof. Dr. Juan P. Garrahan” in Buenos Aires, Argentina, from July 2013 to July 2023. From July 2016 onward, data were analyzed prospectively following the implementation of the Hemispheric Surgical Score and the flowchart. Evaluations included clinical, MRI, and angiographic criteria, and patients were managed by an interdisciplinary team. Demographic, clinical, and neuroimaging data were collected and analyzed.

Results

Eighty hemispheres from 40 patients were analyzed, with cerebral revascularization performed on 72 hemispheres from 37 patients. The Hemispheric Surgical Score and flowchart standardized treatment decisions, and reduced the need for invasive studies like angiographies for follow-up. The majority of patients (79.1%) had favorable outcomes, with complete disease progression arrest and no worsening of imaging nor clinical scores during a median follow-up of 35.8 months.

Conclusion

The Hemispheric Surgical Score and the comprehensive flowchart have improved the management of MMD in pediatric patients by standardizing treatment and reducing unnecessary invasive procedures. This interdisciplinary approach has led to better patient outcomes, highlighting the need for further validation in larger studies and comparisons of different revascularization techniques through randomized clinical trials.

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Titel: Experience of an interdisciplinary management for pediatric Moyamoya disease: application of a novel Hemispheric Surgical Score
verfasst von: Santiago Ezequiel Cicutti
Guido Patricio Gromadzyn
María Celeste Buompadre
Carlos Rugilo
Flavio Requejo
María Laura Gonzalez Dutra
Javier Danilo Gonzalez Ramos
Sebastián Gastón Jaimovich
Publikationsdatum: 11.09.2024
Verlag: Springer Berlin Heidelberg
Erschienen in: Child's Nervous System / Ausgabe 12/2024
Print ISSN: 0256-7040
Elektronische ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-024-06602-6

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Experience of an interdisciplinary management for pediatric Moyamoya disease: application of a novel Hemispheric Surgical Score