Manish Ruhela^1,, Dinanath kumar¹, Chandra Bhan Meena², Rajeev Bagarhatta¹

Moyamoya (meaning a hazy puff of smoke) disease is a rare, idiopathic, persistent, occlusive cerebrovascular disease involving bilateral progressive stenosis or occlusion of a terminal portion of the internal carotid artery, or a proximal portion of the anterior cerebral arteries and the middle cerebral arteries. There are irregular perforating vascular networks (moyamoya vessels), seen in the base of the brain, which produce magnetic resonance images of this ‘puff of smoke’ condition. The association of moyamoya disease and congenital heart disease such as Coarctation of aorta, ventricular septal defect and tetralogy of fallot has been previously reported. Here we report a case of 15 yr old female child presented with seizure episode and was diagnosed as having moyamoya disease with ebstein anomaly. To the best of our knowledge this is the first case report of this co-occurrence of moyamoya disease with ebstein anomaly.

moyamoya disease, ebstein anomaly