arterial fibrodysplasia n.
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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 fibrodysplasia1
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 fibrodysplasia3
Arterial fibrodysplasia
  • First described 1938 by Leadbetter
  • Second leading cause of surgically correctable of hypertension
  • Incidence < 0.5%
arterial fibrodysplasia pathogenesis
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
arterial fibrodysplasia ddx
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
renal artery dysplasia
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%
renal artery dysplasia1
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
renal artery dysplasia treatment
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)
renal artery dysplasia treatment1
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
renal artery dysplasia treatment2
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
cerebrovascular artery dysplasia
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
other vascular beds
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)
final points
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