Arterial fibrodysplasia
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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


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