Fibromuscular dysplasia

Belan A, Vesela M, Vanek I, Weiss K, Peregrin JH. Percutaneous transluminal angioplasty of fibromuscular dysplasia of the internal carotid artery. Cardiovasc Intervent Radiol 1982 5 79-81. 

HassoAN, Bird CR, Zinke DE, Thompson JR. Fibromuscular dysplasia of the internal carotid artery percutaneous transluminal angioplasty. AJR Am J Roentgenol 1981 136 955-960. 

Begelman SM, Olin JW (2000) Fibromuscular dysplasia. Curr Opin Rheumatol 12 41-47... 

Osborn AG, Anderson RE (1977) Angiographic spectrum of cervical and intracranial fibromuscular dysplasia. Stroke 8 617-626... 

Schievink WI, Puumala MR, Meyer FB et al. (1996) Giant intracranial aneurysm and fibromuscular dysplasia in an adolescent with alpha 1 -antitrypsin deficiency. J Neurosurg 85 503-506... 

Zimmerman R, Leeds NE, Naidich TP (1977) Carotid-cavernous fistula associated with intracranial fibromuscular dysplasia. Radiology 122 725-726... 

Atherothromboembolism ntracranial small vessel disease Leukoaraiosis Dissection (Table 6.4) Fibromuscular dysplasia Congenital arterial anomalies Moyamoya syndrome Embolism from arterial aneurysms Inflammatory vascular diseases Irradiation... 

Fig. 6.21 Fibromuscular dysplasia. ceMRA (a) of the left internal carotid artery shows alternating stenoses (arrows) in this patient with fibromuscular dysplasia. 2D TOP MIP image (b) also demonstrates the stenoses, but these can be confused with shce misregistration artifact...

Renal arteries are more commonly investigated by MRI rather than by CT. However, CTA in combination with oblique MIP or VRT is helpful in the detection of different pathologies of the renal arteries, such as atherosclerotic stenosis, fibromuscular dysplasia or Takayasu arteritis. Post-processing of the renal arteries also allows measurement of the degree of stenosis prior to intervention, or as followup control (Sabharwal et al. 2006). 

Various hereditary connective tissue disorders have been associated with formation of aneurysms, most likely as a result of the weakening of the vessel wall. Intracranial aneurysms may develop in 10%-15% of patients with polycystic kidney disease, an autosomal dominant disorder. Although Marfan syndrome was previously identified as a risk factor for aneurysms, a recent study did not find any significant relationship (Conway et al. 1999). Coarctation of the aorta, fibromuscular dysplasia and pheochromocytoma are associated with intracranial aneurysms, most likely because of the elevated blood pressure that occurs in these conditions. 

Lee EK, Hecht ST, Lie JT (1998) Multiple intracranial and systemic aneurysms associated with infantile- onset arterial fibromuscular dysplasia. Neurology 50 828-829 Lee KC, Joo JY, Lee KS, Shin YS (1999) Recanalization of completely thrombosed giant aneurysm case report. Surg Neurol 51 94-98... 

The pathogenesis of arterial stenosis or occlusion may be secondary to a wide spectrum of etiologic factors including atherosclerosis, thrombosis, embolic events of various etiology, fibromuscular dysplasia, vasculitidies, dissection, trauma, external compression, and vasospastic syndromes. Atherosclerosis is by far the most common cause of arterial stenosis. 

Fibromuscular dysplasia has been reported in a child with in utero cocaine exposure [38 ]. 

A 21-month-old boy began vomiting daily. He had been exposed to cocaine in utero. His symptoms improved initially and then deteriorated, with loss of consciousness. He developed pneumonia, a dilated cardiomyopathy, and presumptive myocarditis, had a respiratory arrest and renal failure and died. Postmortem findings were consistent with dilated cardiomyopathy and the major coronary arteries had moderate luminal narrowing by inti-mal fibroplasia. Histology showed changes of intimal fibroplasia diffusely present in the intramyocardial coronary artery branches, consistent with intimal fibroplasia, a rare variant of fibromuscular dysplasia. There was no evidence of myocarditis. 

Fibromuscular dysplasia is an idiopathic disease of small and medium sized arteries. The authors postulated that cocaine had altered transforming growth factor beta, which had caused intimal fibroplasia. 

Thomas KR, Thomas SP, Hewan-Lowe KO, Pestaner JP. Fibromuscular dysplasia in association with intrauterine cocaine exposure. Cardiovasc Pathol 2007 16 313-6. 

Bisschops RH, Popma JJ, Meyerovitz MP (2001) Treatment of fibromuscular dysplasia and renal artery aneurysm with use of a stent-graft. J Vase Interv Radiol 12 757-760... 

Fibromuscular dysplasia (FMD) is an inherent arterial wall abnormality that classically affects the media of the renal arteries and can be associated with renal artery aneurysms. Several subtypes of FMD have been described and the disorder can affect other medium-sized vessels including the carotid, vertebral, brachial, and visceral arteries. For the angiographer, FMD has the classic beaded appearance often described as a string of pearls. Both aneurysms and dissections can be seen with this disorder. The treatment for FMD is angioplasty of the intraluminal webs, which results in significant remodeling. 

---