Introduction: Episodic headache is common in childhood moyamoya disease (MMD). The onset, mechanism, cause of headache and the effect of revascularization surgery on headache are not yet clear.
Methods: We studied 10 cases of children (7 boys and 3 girls) younger than 18 years who underwent revascularization for MMD between 2009 and 2013. We evaluated frequency of headache and cerebral blood flow changes by magnetic resonance angiography (MRA) and single photon emission computed tomography brain imaging with [I123]-labeled iofetamine (IMP-SPECT) before and after surgery.
Results: Patients’ ages ranged from 0 to 15 years at onset and 2 to 17 years at the time of surgery; mean age being 6.7 and 8.0 years respectively. 9 of 10 patients presented with ischemic symptoms and 8 had headache. 5 patients underwent indirect bypass and 5 underwent combined direct and indirect bypass. Cerebral blood flow improvement was obtained in 14 of the 15 cerebral hemispheres revascularized. The mean follow-up duration was 32.9 months. All the patients had good outcomes with improvement of ischemic neurological deficits. Headache improved in 7 (87.5%) of 8 patients.
Conclusions: Headache in pediatric moyamoya disease is associated with change in cerebral hemodynamics. Revascularization including combined direct bypass and indirect techniques may be required to reduce headache in patients with MMD.
Patient Care: This research demonstrates that revascularization surgery in pediatric patients with moyamoya disease carries a low risk, and is effective at preventing future ischemic events. It also alleviates the symptoms related to cerebrovascular insufficiency in most of the cases.
Learning Objectives: By the conclusion of this session, the participants should be able to: 1) Understand the clinical features associated with hemodynamic changes in Moyamoya disease; 2) Discuss various revascularization procedures and their outcomes; 3) Identify combined direct and indirect revascularization procedures as effective treatment for headache associated with pediatric Moyamoya disease.