"Radiation-induced moyamoya syndrome after proton therapy in a child with clival craniopharyngioma: natural history and surgical treatment".

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"Radiation-induced moyamoya syndrome after proton therapy in a child with clival craniopharyngioma: natural history and surgical treatment".

World Neurosurg. 2018 Dec 21;:

Authors: Scala M, Vennarini S, Garrè ML, Tortora D, Cianchetti M, Fellin F, Lorentini S, Pavanello M

Abstract
BACKGROUND: Proton therapy has proven to be effective and safe in the treatment of radioresistant skull base tumors such as chordomas. Thanks to the peculiar physical properties of the proton beam, the radiation energy is delivered in a narrow space called the Bragg peak and the surrounding normal tissues receive a minimal amount of the radiation dose. This is important to lower the risk of radiation-induced damage, especially in children. However, local adverse effects in proximity to the target volume may occur. In particular, the development of moyamoya syndrome (MMS) has been rarely reported in children receiving proton beam therapy (PBT) for brain tumors.
CASE DESCRIPTION: We report on a child who developed rapidly progressive MMS after PBT for a clivus chordoma. A combined indirect revascularization procedure by encephalo-duro-arterio-synangiosis (EDAS) and encephalo-myo-synangiosis (EMS) was performed with good neuroradiological and clinical outcome.
CONCLUSIONS: Regardless of the presence of known risk factors for MMS, strict neuroimaging surveillance is indicated in all patients treated with radiotherapy, including those receiving PBT. We suggest that early revascularization procedure should be considered in patients with worsening symptoms and/or sign of neuroradiological progression of cerebral vasculopathy. This management of MMS could lower the risk of permanent neurological deficits and improve patients' quality of life.

PMID: 30583132 [PubMed - as supplied by publisher]

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