eMedicine Specialties > Radiology > Vascular/Interventional
Fibromuscular Dysplasia (Visceral Arteries)
Updated: May 28, 2008
Introduction
Background
Fibromuscular dysplasia (FMD) is an uncommon angiopathy of uncertain etiology associated with heterogeneous histologic changes that may affect the carotid and vertebral circulation, visceral arteries, and peripheral arteries.1,2,3,4,5,6,7
The frequency with which FMD affects the renal artery varies. Hartnell reports an incidence 2.2% (personal communication). Andreoni et al reported a 4.4% incidence of FMD in renal donors.8 The disease predominantly occurs in young to middle-aged women. The disease ultimately results in arterial stenosis, causing organ ischemia or infarction (see Image below and Image 1 in Multimedia).
Distribution of various types of fibromuscular dysplasia.
The clinical manifestations reflect the arteries involved and most commonly include hypertension caused by renal-artery stenosis (RAS) or strokes from carotid artery disease.9,10 FMD is one of the most important mimics of vasculitis. Although FMD is a pathologic diagnosis, a characteristic angiographic change is the string-of-beads appearance caused by areas of relative stenoses or webs alternating with small fusiform or saccular aneurysms of the artery. The string-of-beads sign is typical of medial fibroplasia, which is only 1 of the types of FMD.11
Conventional flush aortogram in a 47-year-old woman with difficult-to-control hypertension shows the characteristic string-of-beads sign of the right renal artery due to medial fibroplasia.
This 52-year-old man presented with pain in the left upper quadrant and was found to have a 3.2-cm aneurysm of the distal splenic artery. During surgery, the aneurysm ruptured, and splenectomy was performed. Histology of the resected splenic artery revealed intimal fibroplasia. Routine 2-year follow-up showed an enlarging aneurysm of the hepatic artery. Contrast-enhanced axial CT images show several narrowings of the common and proper hepatic arteries with intervening aneurysmal dilatation. Note the circumferential, lobulated tissue that is thickening outside the intima. This was assumed to be a manifestation of intimal fibroplasia.
A 28-year-old man presented with episodic, postprandial abdominal pain, hypertension, ischemic changes in the right toes, and a pulsatile swelling behind the knee. In this ultrasonogram, the superior mesenteric artery has a beaded appearance.
Three-dimensional gadolinium-enhanced magnetic resonance angiograms (MRAs) show medial fibroplasia, which appears as classic string-of-beads sign. This sign is due to multiple stenoses with intervening outpouchings that form a chain. In this case, the lesions involve the main right renal artery and the right accessory renal artery in a 37-year-old man with difficult-to-control hypertension.
Pathophysiology
Leadbetter and Burkland first reported FMD of a renal artery in 1938 when they removed an ectopic kidney from a 5-year-old boy who presented with sustained hypertension.9
FMD may involve any layer of a visceral artery, and it may be classified on the basis of the primary involvement of the arterial wall. The classification system includes intimal, medial, or adventitial fibrosis. The medial variety can be subdivided. In 1967, McCormack et al histologically classified FMD on the basis of the primary site of involvement of the arterial wall.12 Their classification of fibrosing lesions of renal arteries included the categories of intimal fibroplasia, medial fibrosis with microaneurysms, subadventitial fibroplasia, and fibromuscular hyperplasia. They coined the term chain of beads to describe radiographic changes in medial fibroplasia of the renal artery. The term has subsequently been modified to string of beads.11 Medial fibroplasia is the most common type of FMD and represents 80-95% of cases. The string-of-beads sign is classically seen in medial fibroplasia.
Subadventitial fibroplasia can have a similar radiographic appearance. However, in this variant, the size of the aneurysms does not exceed the diameter of the renal artery. Medial fibroplasias may appear as a single stenosis of a visceral artery, but it is most often seen as multiple stenoses with intervening outpouchings that form a chain. This is radiographically depicted as the string-of-beads sign.
On histologic evaluation, medial fibroplasias can be subdivided into 2 types: a peripheral form and a diffuse form. The peripheral form generally affects the outer media, replacing the smooth muscle with fibrous-appearing tissue. The diffuse form affects the media more extensively than the other form, with replacement of the media with fibrous tissue and medial thinning. The media can be completely absent in some areas, giving rise to aneurysmal dilatation. Although FMD was initially described in the renal arteries, many other visceral arteries are now known to be involved, and multiple visceral artery aneurysms have been reported.7,13
Although the pathogenesis is not completely understood, humoral, mechanical, and genetic factors, as well as mural ischemia, may play a role. Hormonal factors have been implicated because medial fibroplasias and subadventitial fibroplasias are found predominantly in women. The common association with ptotic kidneys has supported the mechanical theory in which stretching of the renal artery may be responsible for the development of FMD. Ischemia from inadequate nutrition of the renal artery by the vasa vasorum has also been proposed. A deficiency of alpha-1-antitrypsin (AAT) has also been implicated in the development of various disorders affecting medium-sized arteries; these include intracranial aneurysms, cervicocephalic arterial dissections, and FMD. Some have suggested that heterozygous AAT deficiency may be a genetic risk factor for FMD.6,14,15,16,17
Schievink et al retrospectively studied the frequency of FMD in patients with AAT deficiency on postmortem examination in 1983-1992 at the Mayo Clinic.15 Arterial FMD was found in 2 of 6 patients with AAT deficiency (33.3%; 95% confidence interval [CI] = 4.3%, 77.7%), compared with 23 of 6690 patients without the deficiency (0.3%; 95% CI = 0.2%, 0.5%). In patients with both AAT deficiency and FMD, the arterial media was thickened and was composed of irregular arrays of muscular and connective tissue fibers in a background of mucoid ground substance. The authors concluded that these findings provided further evidence of an underlying arteriopathy in patients with AAT deficiency and suggested that FMD may be a nonspecific disorder.
Sang examined the role of several suggested etiologic factors in renovascular FMD in a case-control study of 33 patients with angiographically demonstrated FMD and in 61 control subjects (renal transplant donors) with normal renal arteries.14 Factors included use of oral contraceptives or markers of sex hormone dysfunction, mechanical stress to the renal artery wall, human lymphocyte antigen (HLA) type, cigarette smoking, hypertension for more than 5 years, and a family history of cardiovascular disease.
The risk of FMD was significantly (P = 0.003) increased (odds ratio [OR] = 4.1, 95% CI = 1.5, 10.9) among cigarette smokers. A significant (P <0.001) dose-response relation was noted between cigarette use and FMD (OR = 8.6 for those who had smoked >10 pack-years). Personal history of hypertension for more than 5 years was also an associated factor (OR = 5.0, 95% CI = 1.1%, 22.8%) with a significantly (P = 0.036) increased risk of FMD. HLA-DRw6 antigen was more common in the patients with FMD than in the donor control subjects (OR = 3.00, P = 0.067) or a second group of 934 ambulatory control subjects (OR = 2.51, P = 0.031). Adjustment for cigarette smoking increased the OR to 5.0 (95% CI = 1.3%, 19.6%). A family history of cardiovascular disease was positively but not significantly (OR = 1.7, P = 0.175) associated with FMD.
Boutouyrie et al defined a new carotid phenotype in FMD by using a noninvasive echo-tracking system and found increased wall thickness and distensibility of the radial artery.18 Their data indicated the presence of subclinical lesions at arterial sites distant from the renal arteries, suggesting that renal FD is not a focal but a systemic arterial disease.
Meacham and Brantley reported a family with FMD of the superior mesenteric arteries (SMAs).19 They described a critically ill 17-year-old girl who had an evolving GI infarction; the patient died 11 months after she presented. After surgical revascularization, biopsy of the SMA showed FMD. The patient's close consanguineous relatives were interviewed and examined; the patient's mother and younger sister were found to have abdominal bruits. Arteriograms showed total occlusion of the celiac artery and SMA in the sister and subtotal celiac occlusion in the mother. Postprandial abdominal pain and constipation in the sister prompted elective mesenteric revascularization, and biopsy confirmed FMD identical to that of her older sister. The mother, who was asymptomatic, had single-vessel disease and did not require surgery. This report supports a genetic basis for FMD.
Frequency
United States
Renovascular hypertension, observed mainly in women aged 30-50 years, is the most common manifestation of FMD. Its prevalence in hypertensive patients is estimated to be less than 1%. The true prevalence of the disease is probably higher because in normotensive or asymptomatic hypertensive patients, many cases go undetected.10,20,21
Renovascular disease is an important cause of hypertension in children and is associated with considerable increase in the risk of morbidity and mortality. Secondary hypertension is more common in children than in adults, with 75-80% of cases affecting children. In 70% of all cases of secondary hypertension in children, the cause is fibromuscular hyperplasia. Other associated conditions are aorto-aortitis, midaortic syndrome, Williams-Beuren syndrome, neurofibromatosis, Takayasu arteritis, William syndrome, pheochromocytoma, Kawasaki disease, Fabry disease, Marfan syndrome, and Degos-Köhlmeier disease.22
Tyagi et al described their initial and long-term results of percutaneous transluminal angioplasty (PTA) in the management of renovascular hypertension in children; 11.5% of the patients were found to have FMD.23 A Russian group from the Bakulev Institute of Cardiovascular Surgery described the results of surgery to manage renovascular hypertension; 71 of their 185 pediatric patients had FMD.24
International
No data suggest that the frequency of FMD in other countries is different from that in the United States.
Mortality/Morbidity
The natural history of FMD is variable and not necessarily benign. In 1 study of 42 patients (50% male) with FMD, angiography showed a progression of disease in all patients during follow-up (1 mo to 11 y 4 mo). All forms of FMD are progressive at variable rates.25
By contrast, Pohl and Novick reported disease progression in 33% of 66 patients with FMD.26 No stenosis progressed to complete occlusion, and no clear association with renal atrophy was observed. In general, FMD affects the mid or distal renal artery, and it may be associated with branch RAS. However, the renal microcirculation is normal, in sharp contrast to atheromatous renal vascular disease. Therefore, progression to renal atrophy is the result of hemodynamically significant proximal arterial stenosis that exceeds 75-80%.27,28
Schreiber et al showed that loss of renal function, as demonstrated by an increased serum creatinine level or reduced kidney size, seldom occurs despite progressive disease on angiography.24 Anatomic progression of medial fibroplasias in the renal artery has been reported in 12-66% of patients with disease of the main renal artery. However, as mentioned, deterioration of renal function, as determined on the basis of the creatinine level or a reduction in renal size, seldom occurs despite progression of RAS, as demonstrated angiographically.29
Abbas et al reviewed the experience at the Mayo Clinic with outcomes of hepatic-artery aneurysms (HAAs).30 They retrospectively reviewed 306 patients with true visceral aneurysms diagnosed from 1980-1998, identifying 36 patients (12%) with HAA (23 men, 13 women; mean age, 62.2 y; range, 20-85 y). Most aneurysms were extrahepatic (78%) and single (92%). Mean aneurysmal diameter at presentation was 3.6 cm (range, 1.5-14 cm). Five aneurysms had ruptured (14%), and 4 were symptomatic (11%). Mortality from rupture was 40%. Of 9 patients with ruptured or symptomatic aneurysms, 2 had multiple HAAs, 3 had FMD, and 2 had polyarteritis nodosa. All 5 HAAs that ruptured were of nonatherosclerotic origin (P = 0.001). The authors concluded that HAA is associated with a definite risk of rupture (14%). Risk factors include multiple HAAs and a nonatherosclerotic origin.
Rupture of an SMA aneurysm is a rare complication of FMD.13
Complete obstruction of the renal artery (20%) leading to total renal infarction has been reported.25 Studying potential arterial donors with angiographic evidence of FMD, Cragg et al found that 26% developed hypertension, compared with 6% of age- and sex-matched control subjects.31
Hepatic and superior mesenteric involvement occurs infrequently, and sporadic cases of severe intestinal ischemia and HAA rupture have been reported. FMD is a rare cause of abdominal aortic aneurysm.32
Race
No racial predilection is reported.
Sex
- With one exception, all types of FMD affect women more often than men.
- The exception is intimal fibroplasias, which has an equal male-female distribution.
- The male-to-female ratio of FMD is 1:3-5.
Age
- FMD generally affects middle-aged adults, mostly women; however, it can affect children as well. The average age range is 30-40 years. The form of FMD affecting middle-aged women is usually medial fibroplasia and differs from that in children. Aneurysmal formation in FMD is well documented in women. However, it is rarely described in children, partly because medial fibroplasia with aneurysm formation is relatively rare in children.
- FMD is an important cause of renovascular hypertension in children.
- The youngest reported patient with FMD of the renal artery was 6 months old.
Anatomy
FMD can affect the renal, hepatic, left gastric, and splenic arteries, the SMA, and the inferior mesenteric artery (IMA). Although classified as dorsal branches, the renal arteries usually arise as lateral aortic branches slightly below the disk between L1 and L2. In rare cases, the renal arteries may arise below the inferior aspect of T12 or below the lower border of L2. The position of the kidney is variable, and though most renal arteries arise between L1 and L2, the length of the renal arteries and the angle between the aorta and the renal arteries varies. The lower the kidneys, the longer and more acutely angulated the renal arteries. The right renal artery may originate slightly anterior to the coronal plane.
Supplemental renal arteries may be problematic for the angiographer because they may be difficult to find, and the catheter tip may obstruct the orifice. The origin of the renal arteries may again be variable; it may arise from D11 down to the iliac vessels. To make things worse for the interventionist, supplemental branches may arise from visceral arteries. Cadaveric studies have shown that single renal arteries are bilaterally present in 72% of cases.
The kidney may be divided into dorsal and ventral segments, and the arteries to these segments can be identified with angiography. The intrarenal branches of renal arteries taper uniformly. The intralobar branches branch repeatedly to give rise to arcuate arteries. The interlobular arteries arise from the arcuate arteries, where they extend into the renal cortex in a parallel fashion. FMD may affect the main renal and intralobar arteries.
RAS caused by FMD affects the middle and distal renal artery in 79% of the patients; a branch renal artery in 4%; or a combination of the 2 in 17%. FMD is bilateral in approximately 65%; the left-to-right ratio is 4:1.
Presentation
Clinical manifestations
The clinical manifestations reflect the arterial bed involved. Patients most commonly present with renovascular hypertension (renal) and stroke (carotid), but rare presentations include subarachnoid hemorrhage, abdominal angina, or claudication of the legs or arms. An acute presentation has been described with renal infarction, rupture of a visceral abdominal aneurysm, and mesenteric and/or intestinal infarction.
FMD often causes hypertension as a result of RAS with no other signs of its presence. It is usually discovered in the workup of difficult-to-control hypertension. The disorder may also be discovered when a bruit is noted over the kidney on a routine examination or on abdominal examination for other disorders.
Patients with FMD of the mesenteric arteries may present with manifestations of bowel ischemia, such as abdominal pain and melena.33 FMD is a pathologic diagnosis, but the characteristic changes may be confirmed by means of angiography, and the diagnosis may be made in the appropriate clinical setting.
Plasma renin activity
Antihypertensive therapy may increase or decrease plasma renin levels. Nonsteroidal anti-inflammatory drugs can also decrease plasma renin levels. The baseline plasma renin activity is elevated in 50-80% of patients with renovascular hypertension. Measuring the increase in the baseline plasma renin activity 1 hour after the administration of 25-50 mg of the ACE inhibitor captopril can increase the predictive value of baseline plasma renin activity.34,35 In patients with RAS, the increase in baseline plasma renin activity is exaggerated; this is perhaps the result of the elimination of the normal suppressive effect of high angiotensin II levels on renin secretion in the ischemic kidney.
Other problems to consider
Atherosclerosis
Atheroma is the most common cause of RAS. The stenosis from atheroma is usually orificial or located in the proximal third of the renal artery. Atheromatous RAS is frequently associated with aortic disease.
Systemic necrotizing vasculitis
The vasculitides are a heterogeneous group of diseases characterized by vascular inflammation and necrosis. They have a wide spectrum of manifestations resulting from the involvement of arteries and other vessels of various sizes and locations.
Data from patients with large and small vasculitides suggest a genetic influence in disease susceptibility.
A variety of vasculitides have been described. Common forms include polyarteritis nodosa, giant-cell arteritis, systemic lupus, systemic sclerosis, Wegener granulomatosis, and Henoch-Schönlein purpura.
Angiographic findings in systemic necrotizing vasculitis include 4 basic arterial anomalies: saccular microaneurysms (62%), arterial thrombosis (81%), arterial stenosis (81%), and luminal irregularities (90%). Alterations in the renal vascular flow are also observed in accordance with changes in the cortical medullary differentiation, heterogeneous nephrogram, and prolonged washout. Microaneurysms may regress after immunosuppressive therapy.
Neurofibromatosis
Neurofibromatosis is a rare cause of RAS; it usually occurs as a direct effect of fibrous proliferation of the intima or media. In some cases, neurofibromatous tissue may affect the adventitia, producing periarterial fibrosis indistinguishable from RAS of other causes. These lesions are usually at the origin of the artery, and they may be bilateral.
Congenital stenosis
Congenital stenosis (coarctation of the renal artery) is extremely rare and is assumed to be congenital because of its discovery in early life. This type of stenosis is generally confined to the main renal artery and may be associated with aortic coarctation. Some patients may eventually have changes of arteritis, FMD, or neurofibromatosis.
Standing waves
Standing waves in the renal arteries appear as multiple serrated indentations symmetrically distributed at evenly spaced intervals. These are of pathologic importance and may represent arterial spasm. They may also affect intrarenal branches.
Fibrous musculotendinous band
A fibrous musculotendinous band may cause extrinsic compression of the renal artery.
Atheroma, FMD, thrombus, embolus, or arteritis
Atheroma, FMD, thrombus, embolus, or arteritis may cause branch RAS.
Klippel-Trenaunay syndrome
Klippel-Trenaunay syndrome is a congenital angiodysplasia consisting of a triad of angiomas, osteohypertrophy, and venous varicosities. Visceral involvement is not uncommon and may cause life-threatening complications.
Binswanger disease
Binswanger disease is a complex disorder characterized by a progressive multi-infarct dementia. Patients often present with recurrent transient ischemic attacks (TIAs), ischemic and hemorrhagic strokes, and other blood-supply disturbances that lead to numerous types of vasogenic brain tissue damage of various intensities. Diffuse arteriosclerosis associated with hypertension is often present.36,37
Grange syndrome
Grange and associates reported 4 of 9 siblings with a syndrome of stenosis of the renal arteries and chronic hypertension; variable stenosis or occlusion of cerebral, abdominal, and probably coronary arteries suspected to be caused by FMD; congenital cardiac abnormalities; brachydactyly and syndactyly of the hands and feet; and increased bone fragility consistent with a mild form of osteogenesis imperfecta.16
Three of the affected siblings had mild to moderate learning disabilities. The parents and remaining 5 siblings had normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including FMD, brachydactyly, syndactyly, and osteogenesis imperfecta, and they are autosomal dominant traits in many cases. Explanations for this familial occurrence included autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.
Weymann and associates reported a 15-year-old boy with stenosis and occlusion of multiple cranial, renal, and celiac arteries; aneurysm of the basilar artery; bilateral cutaneous syndactyly between fingers 4 and 5; partial cutaneous syndactyly between fingers 3 and 4 on the right hand; brachydactyly; and borderline mental retardation.38 The patient's clinical course was characterized by recurrent abdominal pain, gastritis, and high blood pressure.
Preferred Examination
CT angiography (CTA) with maximum intensity projection (MIP) and quantitative measurement of stenosis is an accurate noninvasive technique for diagnosing stenosis of the visceral arteries, whatever the etiology.39
Magnetic resonance angiography (MRA) produces excellent contrast-enhanced angiograms without the risk of iodinated compounds and radiation exposure. Unlike contrast-enhanced angiography, MRA has no attendant risk of nephropathy caused by contrast agent or cholesterol-emboli syndrome. MRA provides accurate information about the number of renal arteries, the size of the kidneys, and the presence of anatomic variants. Future developments may shorten MRA imaging times to reduce the problem of claustrophobia while still allowing the test to provide both anatomic and functional information.40,41,42,43
Local and regional preferences differ regarding the use of CTA vs MRA for cross-sectional imaging. However, as a group, radiologists are using MRA in place of contrast-enhanced angiography as the diagnostic modality of choice. With both CTA and MRA, mild FMD or FMD in the accessory arteries may be missed; therefore, it is still likely that contrast-enhanced angiography is the criterion standard.
Data support the superiority of MRA over duplex Doppler ultrasonography (US) in patients with uremia associated with RAS, though the data do not specifically apply to FMD.40 As availability increases, MRA will likely become the screening test of choice in the diagnosis of RAS, including FMD.
Beregi et al examined 20 patients with hypertension (mean age, 56 y) and CTA-proven FMD of the renal artery.44 The acquisition protocol was as follows: collimation, 3 mm; table speed, 3 mm/s; and incremental algorithm, 1. A consensus panel reviewed MIP and shaded-surface–display (SSD) reconstructions and transverse sections to determine the sensitivity and specificity of each technique in revealing renal-artery FMD.
Helical CTA enabled successful diagnosis of FMD in all 20 patients. Helical CTA showed 31 of 34 pathologic arteries and 33 of 38 lesions. Aneurysms (>6 mm) on arteriography (n = 12) were revealed in 83% of transverse sections, 75% of MIP reconstructions, and 58% of SSD reconstructions. Lesions that had a string-of-pearls appearance on arteriography (n = 19) were shown on 53% of transverse sections, 84% of MIP reconstructions (P <0.05 vs transverse sections), and 74% of SSD reconstructions. Stenoses (n = 7 on arteriography) were revealed on 57% of transverse sections, 71% of MIP reconstructions, and 57% of SSD reconstructions. MIP alone revealed 30 (79%) of the 38 angiographic lesions; however, use of both MIP and transverse sections increased the sensitivity to 87%.
The authors concluded that helical CTA, especially the combination of transverse sections and MIP reconstructions, can reliably depict renal-artery FMD. They further reiterated that, because some lesions may not be shown, arteriography with pressure measurements is the only technique that can be used to assess the physiologic importance of the dysplasia.
Doppler US can be used to measure the velocity of blood flow. It is a noninvasive technique and has a high sensitivity in expert hands. Color flow Doppler US may demonstrate disorganized flow patterns and a high-velocity flow stream associated with hemodynamically significant stenosis.45,46,47,48
Leung et al compared contrast-enhanced MRA with duplex US for the detection of RAS, with catheter angiography as the standard. Eighty-nine patients with clinically suspected renovascular disease underwent duplex renal scanning and MRA. Sixty also underwent catheter angiography.40 Readers blinded to other imaging results interpreted all studies for RAS. In detecting hemodynamically significant (diameter reduction of at least 60%) main RAS, sensitivity and specificity were, respectively, 90% and 86% for MRA and 81% and 87% for duplex US. Most false readings involved differential grading of stenoses detected with all 3 techniques. When patients with FMD were excluded, the sensitivity of MRA increased to 97%, with a negative predictive value of 98%. MRA depicted 96% of the accessory renal arteries seen with catheter angiography; duplex US showed 5%.
The authors concluded that contrast-enhanced MRA is a useful technique for diagnosing atherosclerotic renovascular disease. It overcomes the major limitations of duplex renal scanning. However, duplex imaging has the advantage of providing hemodynamic information, and it appears to be best suited for the assessment of suspected FMD.
Radionuclide renography with technetium-mercaptoacetyltriglycine (MAG3)–captopril has a high sensitivity and specificity and adds a physiologic element to the diagnosis of RAS. In FMD, a positive captopril renographic finding supports intervention, though hypertension is unlikely to be cured.34,35
Hypertensive urography is of historical interest and is no longer used as a screening technique for RAS. Likewise, plain-film radiography has a limited role in patients presenting acutely as a result of mesenteric ischemia, which is a rare complication of FMD.
Renal arteriography can be performed by using conventional or digital subtraction technique. Angiography is particularly indicated when vascular intervention is contemplated. Carbon dioxide has recently emerged as an alternative angiographic contrast agent for use in combination with digital subtraction angiography (DSA) to avoid the risk of conventional nephrotoxic contrast agents in patients with severe renal insufficiency.
At present, angiography remains the criterion standard for detecting arterial stenoses and aneurysms, though its role is being redefined. The diagnostic and prognostic information available from captopril renography and the increasing availability of MRA have reduced the use of renal arteriography as a diagnostic tool, except in evaluating kidneys with intrarenal branch-artery stenoses and those with complex vascular anatomy, including multiple accessory arteries.
Limitations of Techniques
With CTA or MRA, the physician may overlook mild cases of FMD. Most false-negative and false-positive results for RAS arise from accessory renal arteries. MRI is expensive, and its availability is limited.
Measurements of the size of RAS on angiography (an important clinical consideration) may be imprecise and do not permit an assessment of the cross-sectional area or, most importantly, the flow through the stenotic segment. Some of these limitations may be overcome by using pressure measurements across the stenosis and by using intravenous US (IVUS). However IVUS has limited availability.
The various histologic types of FMD are difficult to distinguish on angiograms; this difficulty has an important clinical bearing from a prognostic point of view.
Doppler US is operator dependent, time consuming, and cumbersome. Several factors, including anatomic, technical, patient-related, and pathologic factors, can affect Doppler US.
The false-positive and false-negative rates of hypertensive urography are too high for it to be used as a screening test for RAS. Acceptance of radionuclide renography as a primary screening tool for RAS has been hindered by the lack of standardized protocols.
Differential Diagnoses
Renal Artery Stenosis/Renovascular
Hypertension
Other Problems to Be Considered
Atherosclerosis
Systemic necrotizing vasculitis
Neurofibromatosis
Congenital stenosis
Standing waves
Fibrous musculotendinous band
Klippel-Trenaunay syndrome
Binswanger disease
Grange syndrome
More on Fibromuscular Dysplasia (Visceral Arteries) |
 Overview: Fibromuscular Dysplasia (Visceral Arteries) |
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Further Reading
Keywords
FMD, carotid artery stenosis, carotid artery aneurysm, visceral artery stenosis, visceral artery aneurysm, peripheral artery stenosis, peripheral artery aneurysm, renal artery stenosis, renal-artery stenosis, RAS, renal artery fibrosing lesions, intimal fibroplasia, medial fibrosis with microaneurysms, subadventitial fibroplasia, fibromuscular hyperplasia, segmental mediolytic arteriopathy, alpha-1-antitrypsin deficiency, AAT deficiency
