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68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
7. Joint Meeting mit der Britischen Gesellschaft für Neurochirurgie (SBNS)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

14. - 17. Mai 2017, Magdeburg

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Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in sickle cell disease

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  • Alexander Alamri - King’s College Hospital, Department of Neurosurgery, London, United Kingdom
  • Pennylouise Hever - King’s College Hospital, Department of Neurosurgery, London, United Kingdom
  • Jebet Cheserem - King’s College Hospital, Department of Neurosurgery, London, United Kingdom
  • Catia Gradil - King’s College Hospital, Department of Neurosurgery, London, United Kingdom
  • Sanj Bassi - King’s College Hospital, Department of Neurosurgery, London, United Kingdom
  • Christos M. Tolias - King’s College Hospital, Department of Neurosurgery, London, United Kingdom

Deutsche Gesellschaft für Neurochirurgie. Society of British Neurological Surgeons. 68. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 7. Joint Meeting mit der Society of British Neurological Surgeons (SBNS). Magdeburg, 14.-17.05.2017. Düsseldorf: German Medical Science GMS Publishing House; 2017. DocMO.05.03

doi: 10.3205/17dgnc026, urn:nbn:de:0183-17dgnc0266

Veröffentlicht: 9. Juni 2017

© 2017 Alamri et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.

Gliederung

Text

Objectives: Encephalo-duro-arterio-synangiosis (EDAS) for treatment of Moyamoya syndrome (MMS) has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of such SCA patients who have undergone EDAS and describe their clinical, radiographic and psycho-social outcomes.

Methods: A retrospective review of all the paediatric EDAS procedures conducted in our Institution for SCA from 2007 to 2015 and post-operative Strengths and Difficulties Questionnaires (SDQ) were undertaken by two of the patients.

Results: A total of eight patients with MMS secondary to SCA underwent EDAS. The mean age at the time of surgery was 13 years (range 8 to 17). Unilateral EDAS was performed without complication in seven patients. One patient underwent bilateral EDAS but with a two-year gap in between procedures. Follow-up magnetic resonance angiography demonstrated no progression of Moyamoya collaterals or further ischaemic events with regression of collaterals clearly visible in one patient. Both patients undertaking SDQs showed maintenance of their pre-operative baseline in terms of psycho-social functioning. All patients have demonstrated a return to normal school activities.

Conclusions: EDAS is a well-tolerated revascularisation procedure for children with MMS. The prevention of further infarcts in our group with sickle cell disease has allowed these children to resume normal school activities.