primary pulmonary hypertension and moyamoya disease: case presentation and review of literature

case presentation we reported a 2-year-old boy with pph, who presented unusual and intractable seizure which suggested the diagnosis of md. we also reviewed a few previous reports about the association of pph with moyamoya disease. discussion moyamoya disease is a kind of cerebral vasculopathy and should be considered as a differential diagnosis for patients with unusual seizure attacks who are suffering from primary pulmonary hypertension. introduction primary pulmonary hypertension (pph) is an uncommon disease in children. this pulmonary vascular disease may be idiopathic, familial or a kind of hereditary systemic vasculopathy. multisystemic vascular diseases in children are rare and may be under-recognized. a few reports have described the involvement of cerebral vessels such as moyamoya disease (md) in patients with pph. md is a unique cerebrovasculopathy, characterized by occlusion of the internal carotid arteries.