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

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

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

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 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.

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.

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

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

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

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

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.

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.

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.

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

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.

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.

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