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Arterial Fibrodysplasia

Arterial Fibrodysplasia. 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. Arterial fibrodysplasia.

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Arterial Fibrodysplasia

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  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. Arterial fibrodysplasia • 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 4th 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 1st 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

  10. Renal artery dysplasia

  11. Renal artery dysplasia

  12. Renal artery dysplasia

  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 15-50 years old • Pathogenesis poorly understood • PTA treatment of choice • Stents usually not needed

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