Fibromuscular dysplasia as the substrate for systemic and pulmonary hypertension in the setting of Moya-Moya disease.

We report the first case, to the best of our knowledge, of a child with the unusual association of Moya-Moya disease and both systemic and pulmonary hypertension. Histological examination revealed fibromuscular dysplasia as the common denominator for a diffuse arteriopathy.