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%
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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
So unusual it’s very existence is debate, so we won’t spend any more time on it.

9. Renal artery dysplasia
So unusual it’s very existence is debate, so we won’t spend any more time on it.

10. Renal artery dysplasia
So unusual it’s very existence is debate, so we won’t spend any more time on it.

11. Renal artery dysplasia
So unusual it’s very existence is debate, so we won’t spend any more time on it.

12. Renal artery dysplasia
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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)
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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
So unusual it’s very existence is debate, so we won’t spend any more time on it.

16. Renal artery dysplasia Treatment
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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)
So unusual it’s very existence is debate, so we won’t spend any more time on it.

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
So unusual it’s very existence is debate, so we won’t spend any more time on it.