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

Document Type

Article

Department

General Surgery (East Africa); Internal Medicine (East Africa); Paediatrics and Child Health (East Africa)

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.

Comments

In this work, the author associates with the Department of Neurosurgery, King’s College Hospital, London, UK

Publication ( Name of Journal)

British Journal of Neurosurgery

Creative Commons License

Creative Commons Attribution 4.0 International License
This work is licensed under a Creative Commons Attribution 4.0 International License.

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