Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in children with sickle cell disease
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Authors
Alamri, Alexander
Hever, Pennylouise
Cheserem, Jebet
Gradil, Catia
Bassi, Sanj
Tolias, Christos M.
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Issue Date
2019
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Article
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Abstract
BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment 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 SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015. Two patients underwent behavioural screening. RESULTS: A total of eight patients with MMS secondary to SCA underwent EDAS. 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. 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.
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British Journal of Neurosurgery
Volume
33
Issue
2