Alamri Alexander, Hever Pennylouise, Cheserem Jebet, Gradil Catia, Bassi Sanj, Tolias Christos M
a Department of Neurosurgery , King's College Hospital , London , UK.
Br J Neurosurg. 2019 Apr;33(2):161-164. doi: 10.1080/02688697.2017.1339227. Epub 2017 Jun 15.
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.
A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015. Two patients underwent behavioural screening.
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.
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.
文献中已对烟雾病综合征（MMS）治疗中的脑-硬脑膜-动脉-血管吻合术（EDAS）进行了详细描述，然而在镰状细胞贫血（SCA）所致的MMS中，EDAS的应用仍存在争议，且长期随访情况不佳。我们报告一组因SCA相关MMS接受EDAS治疗的SCA患者病例系列，描述其术后病程，并对EDAS在镰状细胞贫血治疗中的作用进行文献综述。
对2007年至2015年在我院为SCA进行的所有儿科EDAS手术进行回顾性研究。两名患者接受了行为筛查。
共有8例继发于SCA的MMS患者接受了EDAS治疗。7例患者进行了单侧EDAS，无并发症发生。1例患者接受了双侧EDAS，但两次手术间隔两年。随访磁共振血管造影显示，烟雾病侧支循环无进展，也未发生进一步缺血事件，其中1例患者侧支循环明显消退。所有患者均已恢复正常学校活动。
EDAS是一种耐受性良好的针对MMS患儿的血管重建手术。在我们的镰状细胞病患者组中，预防进一步梗死使这些儿童能够恢复正常学校活动。
