Thromboembolic Pulmonary Hypertension Due to Disseminated Fibromuscular Dysplasia

Abstract

We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.

Moyamoya and Extracranial Vascular Involvement: Fibromuscular Dysplasia? A Report of Two Children

“…The histopathological pulmonary vessel abnormalities were compatible with intimal FMD. We found only two cases with moyamoya and primary pulmonary hypertension in the English literature [4,7]. Fukuhara et al have already suggested that a systemic angiopathy, such as intimal FMD, could be the cause of the moyamoya syndrome as well as the primary pulmonary hypertension in their patient [4].…”

“…Ikeda has studied the pathological features of extracranial arteries in eleven patients with moyamoya disease that had no symptoms of extracranial vascular disease [6]. The intima-tomedia ratio of pulmonary, renal, and pancreatic arteries was significantly increased as compared to control subjects, suggesting that the presence of both systemic and local factors can cause the Fibromuscular dysplasia is a systemic vascular disorder that has been suggested to be the cause of moyamoya syndrome and associated extracranial arterial disease [4,8,9]. The progressive stenosis of the carotid arteries in FMD may lead to the angiographical appearance of moyamoya [2].…”

Development of renal and iliac aneurysms in a child with generalized infantile myofibromatosis

“…Microscopic findings include vascular wall fibroplasia, disruption of the elastic membrane and formation of small saccular aneurysms [10]. Medial fibroplasia is the most common dysplastic lesion, but intimal dysplasia occurs in approximately 10% of FMD and is more often seen in children [11]. The development of arterial aneurysms and vascular lesions mimicking FMD in a child with IM is uncommon and intriguing.…”

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

“…1 A few cases of the disease have recently been reported with extracranial arterial lesions, suggesting a diffuse arteriopathy as the underlying cause. [2][3][4][5][6] We report here the details of a child presenting with the disease in the setting of a multisystemic arteriopathy, including renal and pulmonary arterial stenoses. To our knowledge, an association of these three conditions has not thus far been reported.…”