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Arterial Fibrodysplasia. Arterial fibrodysplasia  Heterogeneous group of nonatherosclerotic, noninflammatory occlusive and aneurysmal diseases  Classified.

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Presentation on theme: "Arterial Fibrodysplasia. Arterial fibrodysplasia  Heterogeneous group of nonatherosclerotic, noninflammatory occlusive and aneurysmal diseases  Classified."— Presentation transcript:

1 Arterial Fibrodysplasia

2 Arterial fibrodysplasia  Heterogeneous group of nonatherosclerotic, noninflammatory occlusive and aneurysmal diseases  Classified by layer affected – intima, media, adventitia  Most often renals and carotids, but described everywhere in the body

3 Arterial fibrodysplasia

4  First described 1938 by Leadbetter  Second leading cause of surgically correctable of hypertension  Incidence < 0.5%

5 Arterial fibrodysplasia Pathogenesis  Unknown  Genetic – more common among first degree relatives with FMD and certain alleles of ACE  Hormonal influences on smooth muscle  Mechanical stress

6 Arterial fibrodysplasia DDx  Atherosclerosis – usually occurs at origin or proximal part of vessels in older patients with usual risk factors  Vasculitis – may look like FMD on imaging, but will have biochemical (or pathologic) evidence of inflammation

7 Renal artery dysplasia  Medial fibrodysplasia -- the big one (85%)  90% female, usually 4 th decade  Rare among African Americans  Morphology ranges from focal stenosis to series of stenoses with intervening aneurysmal outpouchings (“string of beads”)  Affects distal main renal artery, extending into 1 st order segmanetal branches 25%

8 Renal artery dysplasia  Progression (new lesion, worse stenosis, larger aneurysm, HTN, loss of renal parenchyma) of disease occurs in 12-66% of patients, usually premenopausal women  In one series, 18% developed complete occlusion

9 Renal artery dysplasia

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13 Renal artery dysplasia Treatment  Medical treatment of HTN  Revascularization for patients who failed medical therapy, are noncompliant, or with loss of renal volume due to ischemic nephropathy  Surgery – 70-90% success rate (worse with longstanding HTN, concomitant atherosclerosis, complex branch vessel repair)

14 Renal artery dysplasia Treatment  PTA – mainstay of treatment  Lower morbidity, still allows for surgery later  Equally effective in main renal artery and branch stenoses  Stents usually reserved if results suboptimal after balloon or if dissection  Complications in 14% (access related problems, dissection, perforation, renal segment infarction)  Restenosis up to 27% after 2 years

15 Renal artery dysplasia Treatment  Follow-up after revascularization  Duplex imaging after procedure, 6 mo, 12 mo, then yearly to detect disease progression, restenosis, or loss of renal volume

16 Renal artery dysplasia Treatment

17 Cerebrovascular artery dysplasia  0.4% of patients undergoing cerebral arteriogram  May cause HA, tinnutus, syncope, TIA, stroke  Symptoms may be due to stenosis, embolism or aneurysm rupture  In last 10 years, PTA has supplanted surgery as preferred treatment

18 Other vascular beds  External iliac arteries next most commonly affected  May present with claudication, critical limb ischemia, or peripheral embolism  In mesenteric arteries, may lead to intestinal angina or acute mesenteric ischemia (rarely)

19 Final points  Nonatherosclerotic, noninflammatory disease affecting medium sized arteries (most often renals)  Most commonly women years old  Pathogenesis poorly understood  PTA treatment of choice  Stents usually not needed


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