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Celeste
Unregistered User
(1/10/03 8:44 am)
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Fibromuscular Dysplasia
--www.uhrad.com/ctarc/ct015.htm


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Clinical History: Middle aged female with hypertension.

Findings: Digital subtraction angiography of the renal arteries was performed. Selection of the right renal artery demonstrated a diffusely beaded appearance of the right main renal artery to the level of the branch vessels. Findings were unilateral; the left renal artery was unremarkable.

Diagnosis: Fibromuscular Dysplasia

Discussion: There are two main causes for renovascular hypertension, atherosclerosis and fibromuscular dysplasia. Together these account for 2-5% of all cases of hypertension. Atherosclerosis is the more common of the two. Symptoms are variable. Patients may present with vascular insufficiency in other organs, epigastric bruit, metabolic alkalosis, or hypokalemia secondary to hyperaldosteronism.

Screening of patients suspected of having renovascular hypertension can be performed by the captopril scan in which an exaggerated plasma renin activity is seen following administration of captopril. Those with essential hypertension do not demonstrate this response. This test is reported to have a sensitivity and specificity of 95%.

Definintive diagnosis can be made by selective renal arteriographs and bilateral renal vein and systemic venous renin measurements. A ratio of 1.5 to 1 or greater between the renal veins is indicative of renovascular hypertension. However a ratio less than this does not exclude the diagnosis as both kidneys may be involved. Additional testing can be done with ultrasound which is reported to have a sensitivity and specificity of 90 and 95% respectively. Magnetic Resonance Angiography, MRA, can also be performed which has a sensitivity of 100% if the stenosis is greater than 50%. The specificity is approximately 90% with these types of lesions. Spiral CT angiography can also be used for diagnosis.

Fibromuscular dysplasia (FMD), is a developmental lesion of unknown etiology which can affect multiple vessels. It consists of areas of heaped up intima, media, and adventitia alternating with areas of medial destruction, the latter resulting in small focal aneurysms. The disorder can be progressive and affects females greater than males in a ratio of 3:1. It more commonly affects middle aged patients. Involvement of the renal arteries is bilateral in 40%. The middle and distal portions of the main renal artery including the branches are commonly involved. Additional vessel involvement includes the splanchnic and hepatic vessels, iliacs, brachiocephalic. carotids, vertebral and vessels in the upper extremities.

FMD is classified into five categories based on location of involvement within the vessel wall. These are: intimal fibroplasia, medial fibroplasia with aneurysm, medial fibromuscular hyperplasia, subadventitial fibroplasia and adventitial fibroplasia. While these can not be differentiated radiographically medial involvement is most common.

Treatment of FMD can be surgical or via angioplasty. While surgery is more invasive, it is reported to have a lower incidence of restenosis and greater improvement in glomerular filtration rate. Angioplasty is less invasive and results in cure in approximately 50% of patients and improvement in 30%. It is most suitable for noncalcified short segment lesions which are not orificial. Branch vessel lesions are also difficult to angioplasty.

References:
1. Davidson RA, Wilcox CS: Newer tests for the diagnosis of renovascular disease.
JAMA 1992; 268:3353-3358.

2. Galanski M et al: Renal arterial stenoses: Spiral CT angiographs Radiology 1993,
189: 185-199.

3. Harrison EG,Jr, McCormack LJ: Pathologic classification of renal arterial disease in
renovascular hypertension. Mayo Clin Proc 1971;46: 161-167.

4. Johnsrude IS, Jackson DC, Dunnick NR, Practical Approach to Angiography. 1987;
278-301.

5. Luscher TF, Lie JT, Stanson AW, et al: Arterial fibromuscular dysplasia.
Mayo Clin Proc 1987;62:931-952.

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Submitted by:
Scott Owen, M.D.

berthaconley
Unregistered User
(6/25/03 7:51 pm)
Reply
fibromuscular dysplasia
Hi,
I am newly diagnosed with FMD of the carotids.
Information seems to be very scarce. I live in Indiana. My e-mail is BERTHACONLEY@aol.com
I would appreciate ANY information I can get.

THank you,
Bertha

Celeste
Unregistered User
(7/21/03 1:01 am)
Reply
Your FMD Diagnosis
Sent you an email, but we should continue a conversation here so others can benefit and join in. What do you think?

How are you feeling? I was diagnosed April 2001. It seems an life time away. Lately there have been touchy days. It is hard to pin them to FMD, but I am more tired every day and its harder to focus and remember things. How about you? Who is your doctor? Did you enter your "story" in the section here?

Donna
Unregistered User
(9/26/03 9:24 am)
Reply
FMD & a friend
Celeste, I have a very dear friend who has FMD. She is quite ill. Additional things she is dealing w/are panic attacks and horrible pain in her head ("like an ice pick in my head"). Where is there a direct line for the best info on this disease? She is going for her 2nd MRI & MRA tomorrow and has already had a stent put in place. Can you please offer any words of encouragement that I can give to her? I feel so helpless.....Donna

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