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Central retinal artery occlusion occurring 30 years after successful revascularization surgery for moyamoya disease: case report

  • Case Report - Pediatric Neurosurgery
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Abstract

We report a case of central retinal artery occlusion (CRAO) leading to unilateral blindness occurring in a moyamoya patient 30 years after successful pial synangiosis when she was 6 years old. Imaging studies at the time of the CRAO revealed total occlusion of the ipsilateral cervical and intracranial internal carotid artery, a vessel shown to be patent on MRI/MRA studies for decades previously. This case demonstrates that long-term follow-up of operated moyamoya patients may reveal late secondary complications, of which physicians, patients, and families need to be aware.

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Abbreviations

CRAO:

Central retinal artery occlusion

MMD:

Moyamoya disease

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Authors and Affiliations

  1. Department of Neurosurgery, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA, 02115, USA

    Madeline B. Karsten & R. Michael Scott

  2. Department of Ophthalmology, Weill Cornell Medicine, New York, NY, USA

    Cristiano Oliveira

  3. Department of Neurology, Weill Cornell Medicine, New York, NY, USA

    Alan Z. Segal

Authors
  1. Madeline B. Karsten
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  2. Cristiano Oliveira
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  3. Alan Z. Segal
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  4. R. Michael Scott
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Corresponding author

Correspondence to Madeline B. Karsten.

Ethics declarations

This case report falls under IRB protocol number IRB-P00027869 of Boston Children’s Hospital therefore patient consent was waived.

Conflict of interest

The authors declare that they have no conflict of interest.

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For this type of study, formal consent is not required. This article does not contain any studies with human participants or animals performed by any of the authors.

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Comments

The authors reported interesting case of central retinal artery occlusion (CRAO) occurring thirty years after successful revascularization surgery for Moyamoya disease (MMD). MMD typically reveals hemispheric ischemic symptoms in both children and adults. After revascularization surgery for middle cerebral artery territory, anterior cerebral artery (ACA) or posterior cerebral artery (PCA) involvement is checked in a clinical setting. If the symptoms of ACA or PCA involvement occur, additional revascularization surgery for ACA or PCA territory should be considered.

Ophthalmic artery occlusion at its origin has not always caused CRAO, because there is a lot of collateral flow though external carotid arterial system. In addition, slowly progressive hypoperfusion of ophthalmic artery due to internal carotid artery stenosis may produce collateral flow to central retinal artery from external carotid arterial system. As they mentioned, this type of CRAO was very rare. Cervical and intracranial internal carotid occlusion ipsilateral to the revascularization is one of the possible mechanisms of retinal ischemia. This paper indicates the multiplicity of progression of MMD and the difficulty of treatment of MMD.

Tomohito Hishikawa

Kenji Sugiu

Okayama city, Japan

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Karsten, M.B., Oliveira, C., Segal, A.Z. et al. Central retinal artery occlusion occurring 30 years after successful revascularization surgery for moyamoya disease: case report. Acta Neurochir 162, 2589–2592 (2020). https://doi.org/10.1007/s00701-020-04501-8

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  • DOI: https://doi.org/10.1007/s00701-020-04501-8

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