Surgical revascularisation for childhood moyamoya

Children with moyamoya disease/syndrome (MM) have a high rate of recurrent arterial ischaemic stroke (AIS) and associated neurological morbidity; surgical revascularisation (SR) has the potential to prevent this. Here we describe our experience of SR for childhood mm.

This study is a retrospective review of case notes and radiology of children with moyamoya who underwent SR at our centre.

Seventy-three children with MM and who had SR were identified, with median age at presentation of 4.75 years (range 0.6-14.5). Children with >1 AIS or transient ischaemic attack (TIA) and who had not infarcted the whole territory of the diseased carotid artery were offered SR, with the primary aim of preventing further AIS. One hundred and thirty-four SR procedures were undertaken: 82 indirect (62.4 %) and 49 (37.6 %) direct procedures with 3 second SR procedures following initial SR. Median age at surgery was 7.3 (1-18.2) years. There was no perioperative mortality. One anticoagulated child developed an intracranial haemorrhage on the fifth post-operative day. Median duration of follow-up was 34 (5-166) months. Three children (4.1 %) had recurrent AIS (one silent) after SR; two with sickle cell disease had intracranial haemorrhage 1.5 and 11 months post-SR. TIAs improved in 42 out of 53 (79.2 %).

Our experience of SR for paediatric MM is comparable to other large series. SR is safe, and the rate of recurrent AIS after SR is lower than would be anticipated, on the basis of known natural history of MM. There was no observed difference in outcome of recurrent AIS for direct versus indirect procedures in this series.