Moyamoya disease with a developmental anomaly of the mesenchyme

We treated a Japanese boy with moyamoya disease accompanied by synbrachydactylia, funnel chest, pes equinus and short stature. Angiograms showed the anomalous origin of the occipital artery in addition to the moyamoya vascular network at the base of the brain. A generalised mesenchymal anomaly was suggested in this case.