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

Abstract

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.

Factors Associated with the Presentation of Moyamoya in Childhood

“…10 Predisposing conditions associated with moyamoya include sickle cell disease, neurofibromatosis type 1, cranial irradiation, trisomy 21, cardiac anomalies such as pulmonary artery hypertension/stenosis, renal artery stenosis, hypertension, hyperthyroidism, William syndrome, and Alagille syndrome. 6,[11][12][13][14][15][16][17][18][19][20][21][22] Other less commonly 26 primordial dwarfism, glycogen storage disorder type 1A, Russell-Silver syndrome, and Hurler syndrome. 27 In 2 large US series totaling 239 cases of childhood moyamoya, almost half had no predisposing condition identified.…”

Primary Pulmonary Hypertension and Moyamoya Disease: Case Presentation and Review of Literature

“…Ou et al on 2006 reported the first case, to the best of their knowledge, a child with the unusual association of moyamoya disease and both systemic and pulmonary hypertension. Histological examination revealed fibromuscular dysplasia as the common designation for a diffuse arteriopathy (12). Klein et al on 2007 reported 2 patients.…”

Juvenile Pulmonary Hypertension Associated with Fibromuscular Dysplasia

“…FMD also affects craniocervical arteries (25-30%) (1) and sometimes causes Moya-moya disease because of intracerebral arterial occlusion. There are a few reports of FMD involving the pulmonary artery (2,3). This report presents a case of FMD with severe pulmonary hypertension due to multiple stenoses in the peripheral pulmonary arteries and with multiple systemic arterial stenoses lacking in systemic hypertension.…”