Childhood moyamoya disease treated by one-staged extensive indirect vascular reconstructive surgery: A case report

Abstract

We report a child with moyamoya disease who was successfully treated by one-staged extensive indirect vascular reconstructive surgery by a combination of encephalo-duro arterio-synangiosis (EDAS), encephalo myo arterio synangiosis (EMAS) and encephalo-galeo-synangiosis (EGS). An 8 year old girl had frequent transient ischemic attacks involving the bilateral extremities starting at 2 years of age. Magnetic resonance imaging showed border zone infarcts on bilateral hemispheres. Cerebral angiogram revealed bilaterally typical moyamoya vessels in the basal ganglia, middle cerebral artery occlusion, and anterior cerebral artery stenosis. Cerebral blood flow (CBF) measurement by SPECT using 133Xe inhalation method demonstrated low CBF at rest and no response to Diamox loading in either cerebral hemisphere except for that in part of the occipital lobe. We performed one staged indirect vascular reconstruction; EDAS on the bilateral posterior branches of superficial temporal artery (STA) and on the left occipital artery, EMAS on the anterior branch of the left STA, and EGS on the left parietal and temporo occipital regions and on the right frontal and parieto occipital regions, resulting in the disappearance of transient ischemic attacks by the second postoperative week. Postoperative examination 3 months after surgery showed rich collaterals from the bilateral external carotid arteries and a 38% increase in resting CBF throughout the cerebrum. Thus, one staged extensive indirect vascular reconstructive surgery may be prudent in treating pediatric moyamoya disease, covering whole areas where preoperative CBF measurement revealed low CBF at rest and impaired vascular reactivity on Diamox loading.