Abstract
Objective Pediatric moyamoya is a rapidly progressive intracranial angiopathy affecting the anterior [internal carotid artery (ICA) bifurcation, middle (MCA), anterior (ACA)] and posterior (PCA) cerebral circulation. Headaches, transient Ischemic attacks (TIAs) and strokes are common initial presentations. We present the preoperative diagnostic workup, neurosurgical treatment and postoperative outcome in newly diagnosed patients referred to our Moyamoya Center, University Children's Hospital Zurich. Methods Thirty four children (female:male 21:13) diagnosed with Moyamoya angiopathy at a mean age of 7.7 years at time of surgery underwent preoperative workup and cerebral revascularisation between 2011 and 2014. Depending on clinical symptomatology, number of arterial territories involved on cerebral angiogram and extent of perfusion reserve deficits on H215-O PET with acetazolamide challenge, surgery was tailormade to each individual child. Results Bilateral angiopathy was observed in 29 patients with involvement of MCA in all, ACA in 24 and PCA in 10. All but 2 patients were clinically symptomatic at time of surgery with all showing perfusion reserve deficits after acetazolamide-H215O-PET in one or more affected arterial territories. Hundred and five revascularisations were performed with 41 in ACA (bilateral in19 patients), 54 in MCA (bilateral in 23 patients) and 10 in PCA territories (bilateral in 1 patient). Multiple combined direct (EC-IC bypass: extracranial to intracranial bypass) and indirect cerebral revascularisation procedures (EDAS: encephalo-duro-arterio-synangiosis, EGPMS: encephalo-galeaperiost-myo-synangiosis and EDS: encephalo- duro-synangiosis) were procedures of choice. There were no perioperative complications. All patients remain stroke free at 1.6 years (1 month to 3 years) follow-up. TIAs and headaches have continued in 2/33 (0.06%) and 3/33 (0.09%) patients but with reduced intensity and frequency. Long term follow-up (up to age of 18 years) continues. Conclusion Early systematic diagnostic workup is essential in pediatric moyamoya. Individualised tailor made multiple cerebral revascularisations are effective in prevention of future strokes.
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