Radiation-Induced Moyamoya Syndrome After Proton Therapy in Child with Clival Chordoma: Natural History and Surgical Treatment

Marcello Scala, Sabina Vennarini, Maria Luisa Garrè, Domenico Tortora, Marco Cianchetti, Francesco Fellin, Stefano Lorentini, Marco Pavanello

Research output: Contribution to journalArticlepeer-review


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, 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 for brain tumors.

CASE DESCRIPTION: We report on a child who developed rapidly progressive MMS after proton beam therapy for a clivus chordoma. A combined indirect revascularization procedure by encephalo-duro-arterio-synangiosis and encephalo-myo-synangiosis was performed with good neuroradiologic 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 proton beam therapy. We suggest that an early revascularization procedure should be considered in patients with worsening symptoms and/or sign of neuroradiologic progression of cerebral vasculopathy. This management of MMS could lower the risk of permanent neurologic deficits and improve patients' quality of life.

Original languageEnglish
Pages (from-to)306-309
Number of pages4
JournalWorld Neurosurg.
Publication statusPublished - Mar 2019


  • Cerebral Revascularization/adverse effects
  • Child, Preschool
  • Chordoma/diagnostic imaging
  • Cranial Fossa, Posterior/pathology
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Angiography
  • Magnetic Resonance Imaging
  • Male
  • Moyamoya Disease/diagnostic imaging
  • Platelet Aggregation Inhibitors/therapeutic use
  • Proton Therapy/adverse effects
  • Skull Neoplasms/diagnostic imaging


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