Ronan J Doherty1,2, John Caird3, Darach Crimmins3, Peter Kelly4, Sean Murphy4, Christopher McGuigan5, Niall Tubridy5, Mary D King3, Bryan Lynch3, David Webb6, Desmond O'Neill7, Dominick J H McCabe2,7,8,9, Peter Boers10, Mary O'Regan6, Joan Moroney1, David J Williams11, Simon Cronin12, Mohsen Javadpour13,14,15. 1. National Neurosurgical Centre, Beaumont Hospital, Dublin, Ireland. 2. School of Medicine, Trinity College Dublin, Dublin, Ireland. 3. Departments of Neurology and Neurosurgery, Temple Street Children's University Hospital, Dublin, Ireland. 4. Department of Neurology, Mater Misericordiae University Hospital, Dublin, Ireland. 5. Department of Neurology, St Vincent's University Hospital, Dublin, Ireland. 6. Department of Neurology, Our Lady's Children's Hospital Crumlin, Dublin, Ireland. 7. Stroke Service and Departments of Neurology and Geriatric Medicine, Tallaght University Hospital, Dublin, Ireland. 8. Vascular Neurology Research Foundation, Tallaght University Hospital, Dublin, Ireland. 9. Department of Clinical Neurosciences, Royal Free Campus, UCL Queen Square Institute of Neurology, London, UK. 10. Department of Neurology, University Hospital Limerick, Limerick, Ireland. 11. Royal College of Surgeons in Ireland, Dublin, Ireland. 12. Department of Neurology, Cork University Hospital and University College Cork, Cork, Ireland. 13. National Neurosurgical Centre, Beaumont Hospital, Dublin, Ireland. mjavadpour@rcsi.ie. 14. School of Medicine, Trinity College Dublin, Dublin, Ireland. mjavadpour@rcsi.ie. 15. Royal College of Surgeons in Ireland, Dublin, Ireland. mjavadpour@rcsi.ie.
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.
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.