Effect of Revascularization on Headache Associated with Moyamoya Disease in Pediatric Patients

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. 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 single photon emission computed tomography brain imaging with [I123]-labeled iofetamine (IMP­SPECT) before and after surgery. 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 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. 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.