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Moyamoya disease and moyamoya syndrome in Ireland: patient demographics, mode of presentation and outcomes of EC-IC bypass surgery

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Abstract

Background

There are no previously published reports regarding the epidemiology and characteristics of moyamoya disease or syndrome in Ireland.

Aims

To examine patient demographics, mode of presentation and the outcomes of extracranial-intracranial bypass surgery in the treatment of moyamoya disease and syndrome in Ireland.

Methods

All patients with moyamoya disease and syndrome referred to the National Neurosurgical Centre during January 2012–January 2019 were identified through a prospective database. Demographics, clinical presentation, radiological findings, surgical procedures, postoperative complications and any strokes during follow-up were recorded.

Results

Twenty-one patients were identified. Sixteen underwent surgery. Median age at diagnosis was 19 years. Fifteen were female. Mode of presentation was ischaemic stroke in nine, haemodynamic TIAs in eight, haemorrhage in three and incidental in one. Sixteen patients had Moyamoya disease, whereas five patients had moyamoya syndrome. Surgery was performed on 19 hemispheres in 16 patients. The surgical procedures consisted of ten direct (STA-MCA) bypasses, five indirect bypasses and four multiple burr holes. Postoperative complications included ischaemic stroke in one patient and subdural haematoma in one patient. The median follow-up period in the surgical group was 52 months; there was one new stroke during this period. Two patients required further revascularisation following recurrent TIAs. One patient died during follow-up secondary to tumour progression associated with neurofibromatosis type 1.

Conclusions

Moyamoya is rare but occurs in Caucasians in Ireland. It most commonly presents with ischaemic symptoms. Surgical intervention in the form of direct and indirect bypass is an effective treatment in the majority of cases.

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Acknowledgments

The authors would like to thank Ms. Deirdre Nolan and Ms. Paula Corr from the audit and research department, Beaumont Hospital, for helping with chart reviews and data collection.

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Authors and Affiliations

  1. National Neurosurgical Centre, Beaumont Hospital, Dublin, Ireland

    Ronan J. Doherty, Joan Moroney & Mohsen Javadpour

  2. School of Medicine, Trinity College Dublin, Dublin, Ireland

    Ronan J. Doherty, Dominick J. H. McCabe & Mohsen Javadpour

  3. Departments of Neurology and Neurosurgery, Temple Street Children’s University Hospital, Dublin, Ireland

    John Caird, Darach Crimmins, Mary D. King & Bryan Lynch

  4. Department of Neurology, Mater Misericordiae University Hospital, Dublin, Ireland

    Peter Kelly & Sean Murphy

  5. Department of Neurology, St Vincent’s University Hospital, Dublin, Ireland

    Christopher McGuigan & Niall Tubridy

  6. Department of Neurology, Our Lady’s Children’s Hospital Crumlin, Dublin, Ireland

    David Webb & Mary O’Regan

  7. Stroke Service and Departments of Neurology and Geriatric Medicine, Tallaght University Hospital, Dublin, Ireland

    Desmond O’Neill & Dominick J. H. McCabe

  8. Vascular Neurology Research Foundation, Tallaght University Hospital, Dublin, Ireland

    Dominick J. H. McCabe

  9. Department of Clinical Neurosciences, Royal Free Campus, UCL Queen Square Institute of Neurology, London, UK

    Dominick J. H. McCabe

  10. Department of Neurology, University Hospital Limerick, Limerick, Ireland

    Peter Boers

  11. Royal College of Surgeons in Ireland, Dublin, Ireland

    David J. Williams & Mohsen Javadpour

  12. Department of Neurology, Cork University Hospital and University College Cork, Cork, Ireland

    Simon Cronin

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  1. Ronan J. Doherty
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  2. John Caird
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  4. Peter Kelly
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Corresponding author

Correspondence to Mohsen Javadpour.

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Conflict of interest

DJH McCabe’s research group has received grant funding from the Trinity College Dublin Innovation Bursary, the Meath Foundation, Ireland, The Vascular Neurology Research Foundation, Ireland and an unrestricted educational grant from SINNOWA Medical Science & Technology Co., China. None of the above charities or funding bodies had any influence on the conduct of this study, or had any influence on the decision to submit the final manuscript for publication.

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This study was approved by the institutional audit board of Beaumont Hospital, Dublin, Ireland.

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Doherty, R.J., Caird, J., Crimmins, D. et al. Moyamoya disease and moyamoya syndrome in Ireland: patient demographics, mode of presentation and outcomes of EC-IC bypass surgery. Ir J Med Sci 190, 335–344 (2021). https://doi.org/10.1007/s11845-020-02280-w

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