FMD Vascular
    > Vascular Disorders
        > Fibromuscular Dysplasia: When is Intervention Warranted?
New Topic    Add Reply

<< Prev Topic | Next Topic >>
Author Comment
Unregistered User
(11/29/03 11:56 pm)
Fibromuscular Dysplasia: When is Intervention Warranted?
Fibromuscular Dysplasia: When is Intervention Warranted?

By Thomas K. Curry and Louis M. Messina

Fibromuscular dysplasia is a multifactorial arteriopathy that primarily affects small and medium-sized
arteries. It is most common in the renal and internal carotid arteries. Pathological classification is
based on the arterial wall layer most significantly involved. The natural history and incidence of
asymptomatic disease is unknown. The most common lesions become symptomatic as a high-grade
stenosis producing renovascular hypertension or as an embolic source for the cerebral circulation.
Treatment is reserved for symptomatic lesions. Most simple lesions are effectively treated by
catheter-based intervention. Surgical therapy is warranted for more complex lesions. Both produce
durable, long-term results.
© 2003 Elsevier. All rights reserved.
heterogeneous group of noninflammatory,
nonatherosclerotic lesions that can affect virtually
any small or medium-sized artery in the body.
These lesions may become symptomatic as a result
of flow reduction, embolic phenomena, dissection,
or aneurysmal degeneration. Fibromuscular dysplasia
is most prevalent in the renal, carotid, and
iliac arteries and exists in multiple vascular beds in
28% of affected patients.1 Treatment of FMD is
usually confined to symptomatic lesions and generally
consists of open or percutaneous angioplasty
(dilation), arterial bypass, or resection of the diseased
The first description of FMD has been attributed
to Leadbetter and Burkland in 1938.2 They described
a 5-year-old boy with hypertension and an
atrophic right kidney. The hypertension resolved
after nephrectomy. Pathologically, the main renal
artery was stenosed by “an intraluminal mass of
smooth muscle” with thinning of the artery wall
distal to this lesion. The term fibromuscular hyperplasia
was introduced in 1958 in McCormack and
associates’ description of 3 patients with hypertension
and renal artery stenosis.3 The incidence and
anatomical distribution of asymptomatic FMD
throughout the arterial circulation is unknown. The
incidence of asymptomatic renal artery FMD has
been identified through angiographic evaluation of
asymptomatic, nonhypertensive potential renal donors.
The University of California, San Francisco
group has recently reported a review of 716 angiograms
obtained for renal donor evaluation over a
10-year period. Fibromuscular dysplasia was identified
in 47 patients, for a prevalence of 6.6%.4
Andreoni and associates at the University of North
Carolina reviewed 159 consecutive angiograms
obtained during renal donor evaluation. Seven
cases (4.4%) of FMD were identified. Within 5
years, 2 of the 7 patients developed hypertension
and 1 required renal artery angioplasty.5
For symptomatic patients, renal artery involvement
accounts for 60% to 75% of patients. Intraand
extracranial cerebrovascular disease involves
approximately 25% of patients, visceral arteries in
approximately 10%, and extremity arteries in approximately
5%.1 FMD has also been reported in
the spinal,6 subclavian,7 superficial femoral,8 profunda
femoris,9 popliteal,10 brachial,11 and ulnar12
The cause of FMD remains unknown and is
probably multifactorial. The most commonly held
hypothesis includes humoral, ischemic, mechanical,
13 and genetic factors.14
In virtually all series of patients, the preponderance
of women over men patients has been
noted.1,4,5,12,13,15 This finding seems to be contradictory
to the expected antiproliferative effect of
estrogen on vascular smooth muscle at both the
cellular16 and arterial levels.17 In fact, neither oral
contraceptives nor pregnancy represent significant
risk factors for arterial fibrodysplasia.18
Ischemia is thought to be 1 factor in the development
of FMD.19 The arteries most likely to
From The Division of Vascular Surgery, Department of
Surgery, University of California, San Francisco, CA.
Competition interests: Nil.
Address reprint requests to Louis M. Messina, MD, Department
of Surgery, University of California, 505 Parnassus Ave,
M-488, San Francisco, CA 94143.
© 2003 Elsevier. All rights reserved.
190 Seminars in Vascular Surgery, Vol 16, No 3 (September), 2003: pp 190-199
become symptomatic from FMD share certain
characteristics that may impact the development of
lesions. The internal carotid artery, renal artery,
and external iliac artery are all long, straight arteries
that are free of branches. This limits the penetration
of vasa vasora, which usually originate at
branch points. Injury to the vasa vasora has produced
dysplastic lesions in animals.20 The mediaadventitial
junction where perimedial and medial
dysplasias occur is also the region where nutrient
flow from the vaso vasora is most important.21
Mechanically, the internal carotid and renal arteries
are also subject to repeated stretching during
head motion and respiration, which may induce
injury. This hypothesis is supported by the observation
that renal FMD is more prevalent on the
right, which has the longer right renal artery, and is
more subject to renal ptosis than the left kidney.22
Some in vitro studies demonstrate increased production
of collagen, hyaluronate, and chondroitin
sulfate in arteries exposed to cyclical stretching.23
The observations that FMD occurs overwhelmingly
in Caucasians,1,13 has been reported in identical
twins,12,24 and affects family members14,25
indicate a genetic basis for the disease. Rushton
studied 20 families with FMD and noted evidence
of autosomal dominance with variable penetrance
in 60% of the families he studied.14
A number of other etiologic factors have been
proposed. In early series, a high percentage of
patients with FMD were cigarette smokers.26,27
There have been case reports of patients with FMD
and pheochromocytoma,28,29 ergotism,30 methylsergide
maleate use,31 1 antitrypsin deficiency,32
Ehlers-Danlos syndrome type IV, Alport’s syndrome,
cystic medial necrosis, and coarctation of
the aorta.33
The pathological classification of renal FMD
was proposed by Harrison and McCormick in
197134 and revised to include other vessels by
Stanley and colleagues in 1975.13 Fibrodysplastic
lesions are classified pathologically into 4 types,
based on the layer of the arterial wall most involved.
The pathological findings correlate with
angiographic abnormalities and progression of disease
(Table 1). This was demonstrated by Goncharenko
and colleagues who performed serial angiography
on 42 patients with FMD and
renovascular hypertension, all of whom had progression
of disease angiographically.35
Intimal fibroplasia accounts for approximately
5% of renal FMD lesions. It typically affects young
adults and children, boys and girls equally,13 and is
often bilateral. Lesions tend to be slowly progressive.
Intimal fibroplasia appears on angiogram as a
long, irregular tubular stenosis of the main renal
artery, progressing to a discrete, smooth focal stenosis
in the older patient.35 Some young children
present with short proximal stenosis. Histologically,
there are accumulations of irregularly ar-
Table 1. Pathologic and Angiographic Features of FMD
Lesion Classification Frequency (%) Pathologic Findings Angiographic Findings
Intimal fibroplasia 5 Subendothelial collagen deposition
in the intima with disruption of
internal elastic lamina
Long, smooth, tubular stenosis in
younger patient. Bilateral in
majority. Progress to smooth
discrete stenosis
Medial hyperplasia 1-2 Isolated smooth muscle hyperplasia
without inflammation or fibrosis
with normal intima and adventitia
Concentric stenosis in main renal
Medial fibroplasia 80-90 Alternating areas of thickened and
thinned media. Outer media with
fibrous tissue. Inner media with
ground substance and collagen
separating smooth muscle. Intima
and adventitia usually normal in
all but most advanced cases
“String of beads” appearance with
aneurysmal dilatation greater
than normal vessel diameter
alternating with web-like
stenoses. Bilateral in 55%.
Branch vessel disease in 25%
Perimedial fibroplasia 10-15 Accumulation of elastic tissue
between adventitia and media.
Normal-appearing intima
Multiple high-grade stenoses in
main renal arteries without
aneurysmal dilation. Progresses
to occlusion
ranged subendothelial mesenchymal cells within a
matrix of loose fibrous connective tissue. The internal
elastic lamina is frequently fragmented, but
always present. The medial and adventitial structures
are usually normal.13 Intimal fibroplasia has
also been described in the carotid artery.36
Medial hyperplasia is the most rare lesion, found
in 1% of patients.13,27 Angiographically, medial
hyperplasia appears as isolated lesions in the midportion
of the main renal artery, making it difficult
to differentiate from intimal hyperplasia. There is
no aneurysmal degeneration, and the lesions do not
extend into branch vessels. It is found most often in
women who are in the 4th through 6th decade of
life and have had a relatively short duration of
hypertension. Histologically, there are normal intimal
and adventitial structures, with an increase in
minimally disorganized medial smooth muscle.
Because this lesion may be a precursor to medial
fibroplasias, some experts have suggested that it
does not represent a distinct pathological type.21
Medial fibroplasia accounts for 80% to 90% of
lesions in both the renal and carotid arteries, and it
has also been described in the visceral and iliac
arteries.13,35 Like other FMD lesions, it is also
most common in Caucasian women in the 3rd to
5th decade of life. Medial fibroplasia is bilateral in
55% of cases and extends into branch renal vessels
in 25%.When unilateral, it occurs in the right renal
artery in 80% of cases. Angiographically, medial
fibroplasia appears as sequential, web-like stenoses
and aneurysmal dilatations of variable severity in
the “string of beads” appearance that can extend
into branch vessels (Fig 1A and B). Histologically,
the intimal and the internal elastic lamina are
normal, except in advanced lesions. The adventitia
is also normal. The extent of involvement in the
media varies, ranging from isolated involvement of
the outer media to extension through the entire
media. The outer media often demonstrates compact
fibrous tissue, while the inner media demonstrates
accumulation of collagen and ground substance
separating disorganized bands of smooth
muscle cells. Alternate medial thickening and thinning
accounts for aneurysmal development.
Perimedial dysplasia accounts for 10% of FMD
lesions in the renal arteries. Patients are typically
younger women with hypertension. Angiography
usually demonstrates multiple high-grade stenosis
of the main renal artery without aneurysmal dilatation.
35 Histologically, there is accumulation of
elastic tissue between the media and the adventitia,
which causes the stenosis. There is some increase
in medial ground substance, with a normal appearing
intima.13 Goncharenko and colleagues’ study
indicates that this lesion progresses to total occlusion
of the renal artery with greater frequency than
does medial fibrodysplasia.35
The primary clinical manifestation of renal
FMD is renovascular hypertension. While renal
FMD accounts for up to 40% of renovascular
hypertension. Since renovascular hypertension is
rare in adults, FMD accounts for only 1% to 2% of
all patients with hypertension.37,38 The diagnosis
should be suspected in women who experience the
sudden onset of diastolic hypertension (diastolic
blood pressure 115 mm Hg) in the first 4 decades
of life.21 Hypertension tends to be refractory to
simple drug therapy. As in atherosclerotic renovascular
occlusive disease, use of an angiotensinconverting
enzyme 1 inhibitor can often worsen
renal function in unilateral disease.
The Michigan experience through the mid-
1980s included 133 women and 11 men, with mean
ages of 39 and 31 years, respectively. The average
duration of hypertension was 42.5 months. Blood
pressures averaged 206/122 mm Hg off medication
and 184/111 mm Hg with drug therapy.15 More
recent series indicate that the typical patient with
renovascular hypertension secondary to FMD is
older, with a longer duration of hypertension and a
higher prevalence of renal branch vessel disease.
39,40 These patients are in age ranges where
essential hypertension and atherosclerotic disease
are more prevalent; since FMD may be a coincidental
finding, this may have significant implications
for the success of therapy.
In the pediatric population, the incidence of both
atherosclerosis and essential hypertension are negligible,
making FMD a much more common cause
of hypertension in this age group. The presence of
hypertension in a pediatric patient without intrinsic
renal disease should always prompt evaluation for
a correctable cause.21
Indications for intervention and treatment of
renal FMD in the adult population are moderate to
severe hypertension and presence of a hemodynamically
significant renal artery stenosis. Screening
tests for renal artery stenosis include duplex
ultrasonography, magnetic resonance angiography,
and radionuclide imaging. When indicated by
screening tests, catheter-based angiography is the
most useful study for determining anatomical and
functional significance of renal artery lesions. Indicators
of significant stenosis during catheter
studies include pressure gradients of 10 mm Hg
across the lesions and demonstration of spontaneous
or pharmacologically induced collateral flow
around areas of stenosis.35,41,42 Renal vein renin
sampling can also be useful in determining functional
significance in select patients by calculating
the renal vein renin ratio and the renal: systemic
renin index.17,38,42-44
Drug Therapy
Hypertension in most patients with renal FMD
can be controlled with medication. Factors that
influence and may influence the risk–benefit ratio
of drug therapy include drug side effects, patient
compliance, and possible deleterious effects on
Fig 1. Renal fibromuscular dysplasia
of the medial fibrodysplasia
type. (A) Selective angiography of
the right renal artery demonstrating
multiple areas of high-grade stenosis
with disease extending into the
branch vessels. (B) Midstream aortogram
demonstrating medial fibrodysplasia
of the right renal artery.
Note the alternating areas of stenosis
and dilation. Note the ptosis of
the right kidney.
renal function from medication or progression of
The principles of drug therapy are based on the
pathophysiology of renal artery stenosis and the
renin–angiotensin–aldosterone system. In patients
with unilateral stenosis, renin-angiotensin–mediated
vasoconstriction is the primary mechanism of
hypertension. The normal contralateral kidney is
able to produce a natriuresis, thus avoiding aldosterone-
mediated volume retention. The normal
kidney is exposed to the full effect of hypertension,
eventually leading to parenchymal disease.45,46 In
patients with bilateral stenoses, the natriuresis cannot
occur, leading to sodium and volume retention,
which is the main mechanism of hypertension in
these patients. These mechanisms can be thought
of as renin-dependent and volume-mediated hypertension,
respectively.38 Volume-mediated hypertension
is also important in patients with renal
artery stenosis in a solitary kidney, or unilateral
stenosis with contralateral parenchymal disease.21
Many drugs are effective in treating renovascular
hypertension by a variety of mechanisms.
-blocking agents are often first line agents in
treating renovascular hypertension by inhibition of
renin secretion.47 Angiotensin-converting enzyme
(ACE) inhibitors or an angiotensin II antagonist
should be considered in patients with renin-dependent
hypertension.38 ACE inhibitors function by
causing efferent arteriolar vasodilatation. In the
presence of bilateral disease, this can cause a
severe reduction of the glomerular filtration rate,
potentially leading to azotemia.48,49 In patients
with volume-mediated hypertension, diuretics
should be added. Additional agents include calcium
channel blockers and vasodilators in patients
with more severe hypertension.
Percutaneous Angioplasty
Because of the potential for cure of hypertension,
invasive intervention is warranted in all
young patients with renovascular hypertension. In
older patients, invasive intervention is warranted in
patients with poor control despite aggressive drug
therapy and in patients unable to tolerate medication.
First reported by Gruntzig and colleagues in
1978,50 percutaneous angioplasty has been demonstrated
to be highly effective in treating renal FMD
and has become the dominant mode of treatment of
renal FMD at most institutions. The mechanism by
which balloon angioplasty enlarges the arterial
lumen in FMD is by stretching the arterial wall,
which results in separation of the intima from the
media, fracture of the media, and stretch of the
adventitia beyond its elastic recoil. Subsequent
changes include smooth muscle cell necrosis, fi-
brosis, and some degree of neointima formation.51
Approximately 85% to 93% of adult patients
with hypertension secondary to renal FMD benefit
from percutaneous angioplasty,21 and 50% to 74%
are cured.21,52 Current studies indicate restenosis
rates of 23%; however, restenosis rates do not
correlate with recurrence of arterial hypertension.
52 After technically successful angioplasty,
failure to improve hypertension correlates with
longer duration of hypertension and older age of
the patient at presentation.
Complications of angioplasty include renal artery
dissection and perforation, which can occasionally
be salvaged with stenting. Other complications
include contrast nephropathy, hematoma
and pseudoaneurysm at the percutaneous arterial
access site.53
Contraindications to angioplasty include macroaneurysms,
branch vessel disease and complex
Surgical Therapy
Surgical therapy is a highly effective and durable
treatment of fibromuscular dysplasia. Compared
to angioplasty, surgical results are associated
with a higher cure rate, approaching 90%.21 However,
such results depend on a properly planned
and executed surgical reconstruction. At 1 institution,
reoperation for complications of renal artery
reconstruction surgery result in secondary nephrectomy
rates of 40% to 60%.54 Adequate preoperative
imaging is imperative in planning a successful
Adequate exposure can be obtained through a
midline or transverse supraumbilical incision. An
advantage of the transverse incision is ease in
handling instruments in the plane of the renal
artery, which is perpendicular to the longitudinal
axis of the body. After exploration, the viscera are
displaced to the opposite side of the abdomen. For
right-sided reconstructions, the right colon is mobilized
and the colon, duodenum, and pancreas are
reflected to the left. After the right renal vein is
identified at its confluence with the vena cava, it is
dissected circumferentially. In order to provide
adequate mobilization, the extrarenal branches of
the renal vein are ligated. The right renal vein is
then retracted superiorly. The right renal artery is
identified lateral to the vena cava and dissected
distally. If required, the right border of the vena
cava can be retracted medially, allowing proximal
access to the right renal as far as its origin. For
left-sided reconstruction, the left colon is mobilized
medially. Again, adequate mobilization of the
renal vein, with ligation and division of the adrenal,
ascending lumbar and gonadal veins is key to
adequate exposure of the left renal artery.
If the aorta is to serve as the inflow vessel, the
infrarenal segment should be dissected circumferentially
for a distance of 5 cm. A vein graft is then
harvested. Following anticoagulion with heparin,
the aorta is clamped, and a longitudinal arteriotomy
is made. The length of the arteriotomy should
be 2 to 3 times the diameter of the graft. The
graft–aorta anastomosis is constructed with 5-0
polypropylene suture, and the graft is then routed
to avoid kinking or twisting. For right-sided reconstruction
procedures, this may be ante or retrocaval.
Antecaval routes simplify reoperation. For
left-sided reconstruction, the graft is usually routed
behind the left renal vein. A brisk diuresis is
established with administration of intravenous
mannitol and/or dopamine-1 agonists. The renal
artery is then clamped and divided. The kidney
should be flushed copiously with cold heparinized
saline solution and controlled distally with microvascular
clamps. For patients with intraparenchymal
or branch vessel stenosis, rigid cylindricaltipped
dilators can be passed through the
arteriotomy in increments of 0.5 mm until satisfactory
dilatation is accomplished. Most often, such
disease is best treated by ex vivo reconstruction.
The bypass is performed as an end-to-end, spatulated
anastomosis with either running or interrupted
suture, depending on the diameter of the
renal artery and the age of the patient. After completion
of the anastomosis, the clamps are removed
and the kidney perfused. An assessment of the
renal anastomosis is then made with duplex ultrasonography.
When satisfactory, the heparin effect
is reversed with intravenous protamine sulfate.
Postoperatively, an imaging study is obtained with
either magnetic resonance or catheter-based digital
subtraction angiography.
Graft material should be individualized for the
patient. Autogenous greater saphenous is the conduit
of choice for most adults if available.55 Synthetic
grafts may be used for large diameter main
renal artery reconstructive procedures, but are less
compliant and not appropriate to use when constructing
anastomosis to small segmental vessels.
56,57 Hypogastric artery grafts are the conduits
of choice in pediatric and young adult patients.58,59
Occasionally, splenorenal or hepaticorenal bypass
is indicated when absence of celiac stenosis has
been demonstrated.60 Ex vivo repair is favored in
all cases of complex branch vessel disease that
extends beyond the first bifurcation.21 Occasionally,
resection of the diseased segment and direct
reimplantation into the aorta may be appropriate
for the pediatric patient with limited orificial disease.
Results of Surgical Therapy
Surgical treatment of renal artery FMD is a
highly effective modality for treatment of renovascular
hypertension in properly selected patients
who undergo an appropriate operation. Large series
over the past 20 years have demonstrated
either cure or improvement in hypertension in over
90% of patients, with operative mortality rates that
approach zero. Over the past decades, the primary
nephrectomy rate has also decreased, probably due
to the evolution in advanced reconstruction techniques.
As with many vascular surgery procedures, recent
reports of renal FMD39,40 have demonstrated
changes in the clinical profile, patient demographics,
prevalence of concomitant atherosclerotic disease,
and complexity of operative repairs. These
changes are probably due to better drug therapy,
increased use of angioplasty for the majority of
lesions, and increased awareness of the need to
identify potential causes of reversible hypertension
in more elderly patients. While the overall benefi-
cial response rates remain similar to earlier series,
the cure rate is generally lower.
Palubinskas and Ripley initially described FMD
in the carotid arteries in 1964.62 Connett and Lansche
described the first operative repair of symptomatic
carotid FMD in the next year.63 The disease
can present with cerebrovascular embolic or
hemorrhagic events, spontaneous or posttraumatic
dissection, aneurysmal degeneration, tinnitus, or as
asymptomatic bruits.12,64-69
Incidence and Distribution of Disease
The true incidence of asymptomatic carotid
FMD is unknown. Fibromuscular dysplasia is
noted on 0.5% to 3% of cerebral angiograms obtained
in patients with neurological symptoms,70
but was identified in only 0.02% of patients in a
large autopsy series.71 Carotid FMD is located in
the middle or upper one third of the internal carotid
artery (ICA) in 95% of cases and is bilateral in
60% to 80% (Fig 2). It is associated with asymptomatic
intracranial aneurysmal disease in 7% of
patients; conversely, up to 51% of patients with
symptomatic intracranial aneurysms have carotid
FMD.72 Vertebral artery FMD is present in 7% to
19% of patients.73 As with renal FMD, most patients
are Caucasian women, and the majority of
the pathology demonstrates medial fibrodysplasia.
73 Up to one third of patients will have associated
atherosclerotic disease of the carotid artery,
which makes assignments of symptoms to discrete
lesions difficult.73 Renal FMD has been reported in
approximately one third of patients with carotid
Natural History
Most series of patients with carotid FMD include
symptomatic and asymptomatic patients
identified during angiography. Many studies also
include patients with intracranial neoplastic disease
who present with global symptoms. No series
has long-term follow-up of large numbers of patients,
which limits our understanding of the natural
history of this disease. The Emory experience
documents 49 patients with carotid FMD. Eleven
asymptomatic patients were not treated, and none
developed symptoms during a mean follow-up of
78 months. None of the other 38 patients who
presented with symptoms developed a new transient
ischemic attack or stroke in an untreated
artery during the follow-up period.12 In the Brooke
Army Medical Center experience, 7 asymptomatic
patients remained asymptomatic, regardless of
medical or surgical treatment. However, 2 of 13
patients with focal symptoms treated with observation
or medication continued to have symptoms.
70 In the Mayo Clinic series, none of the 27
asymptomatic patients in a group of 82 patients
with FMD discovered during angiography developed
cerebral ischemic symptoms over an average
80 months follow-up.71 There are anecdotal reports
of progression of disease in patients who have
undergone serial angiography.73
Because of the low incidence of ischemic symptoms
developing in asymptomatic individuals,
treatment beyond antiplatelet agents is probably
not warranted. For patients with localized embolic
symptoms, treatment with antiplatelet agents may
be effective at reducing symptoms, but patients
should be considered for definitive treatment.
Given the prevalence of aneurysmal disease, all
patients with carotid FMD should have imaging of
the intracranial circulation. Management of both
intracranial aneurysmal disease and extracranial
atherosclerotic disease should be individualized
and treated as appropriate. Invasive treatment
should be reversed for the patient with focal isch-
Fig 2. Angiogram demonstrating carotid artery fibromuscular
dysplasia. Note involvement of the distal internal carotid
artery with sparing of the proximal artery.
emic symptoms. Graduated internal dilation with
either Bake’s dilators or with dilator-shunts75 is
decreasing at most centers but remains appropriate
in patients undergoing open operation for other
pathology. Because of the distal nature of the
disease, the internal carotid artery should be mobilized
to the level of the styloid process. After
heparinization, dilators from 1.5 mm to 4 mm are
passed through a transverse arteriotomy. The artery
is retracted proximally to straighten the vessel and
avoid perforation. Back bleeding and flushing are
performed to remove debris from of the lumen
prior to arteriotomy closure. Another technique
championed by the Loma Linda group involves
open control of the vessel with performance of
balloon angioplasty. The potential advantages of
this technique include radial rather than sheer force
to dilate the vessel, low profile passage through
noninvolved areas of normal vessel and precise
localization of the lesions.76
The University of California, San Francisco experience
with open ICA dilation includes 101 patients
in which 150 operations were done without
mortality. Three patients had strokes in the perioperative
period, all of which resolved in the postoperative
period.74 The European experience with 70
patients undergoing surgery resulted in 95% actuarial
probability of 5-year stroke-free survival. Of
the 5 patients with central nervous system events
during long-term follow-up, 2 were due to cardiac
events and 1 due to untreated contralateral carotid
Transfemoral angioplasty is rapidly becoming
the treatment modality of choice for carotid FMD.
Previous concerns about negotiating tortuous anatomy
are becoming less worrisome as lower profile
systems and wires become available. There are no
series directly comparing surgical against percutaneous
treatment for this disease. Reports on endovascular
and surgical treatment for carotid dissections
have included mixed populations of patients
with iatrogenic dissections, trauma, and FMD.77
Dissections due to carotid FMD should be initially
treated with anticoagulation. Intervention
should be reserved for aneurysmal degeneration
and high-grade stenosis that has failed to resolve
after a period of anticoagulation. Surgical treatment
is associated with a high rate of transient
cranial nerve injury and need for ligation of the
internal carotid artery in up to 10%.79 Small, mixed
series of patients treated endovascularly are reported,
but the long-term results of percutaneous
stenting for carotid dissections are unknown.77
Iliac and visceral FMD is encountered less frequently
than carotid and renal disease. Iliac lesions
can present as embolic sources or as hemodynamicaly
significant stenoses. The choice between standard
bypass or endovascular techniques should be
individualized in symptomatic patients. Patients
with visceral FMD likewise should be treated
based on their symptoms.
Fibromuscular dysplasia continues to have an
unclear natural history in the asymptomatic patient.
Symptomatic lesions usually present as either renovascular
hypertension or carotid lesions that embolize
and cause transient ischemia attacks or
stroke. Treatment for either renal or carotid FMD
is usually catheter based for simple lesions, with
surgery reserved for more complex lesions.
1. Luscher TF, Keller HM, Imhof HG, et al: Fibromuscular
hyperplasia: Extension of the disease and therapeutic outcome;
results of the University Hospital Zurich Cooperative Study on
Fibromuscular Hyperplasia. Nephron 44(Suppl 1):109-114, 1986
2. Leadbetter WF, Burkland CE: Hypertension in unilateral
renal disease. J Urol 39:611-626, 1938
3. McCormack LJ, Hazard JB, Poutasse EF: Obstructive
lesions of the renal artery associated with remediable hypertension.
Am J Pathol 34:582(abstract), 1958
4. Neymark E, LaBerge JM, Hirose R, et al: Arteriographic
detection of renovascular disease in potential renal donors:
Incidence and effect on donor surgery. Radiology 214:755-760,
5. Andreoni KA, Weeks SM, Gerber DA, et al: Incidence of
donor renal fibromuscular dysplasia: does it justify routine
angiography? Transplantation 73:1112-1116, 2002
6. Saunders FW, Birchard D, Willmer J: Spinal artery aneurysm.
Surg Neurol 27:269-272, 1987
7. Hirooka S, Oshikiri N, Kimura M, et al: A left subclavian
arterial aneurysm caused by fibromuscular dysplasia: A case
report. Kyobu Geka 48:221-223, 1995
8. Ritota P, Quirke TE, Keys RC, et al: A rare association of
fibromuscular dysplasia of the femoral artery with aneurysm
and occlusion treated alternatively. J Cardiovasc Surg (Torino)
35:239-241, 1994
9. Schneider PA, LaBerge JM, Cunningham CG, et al: Iso-
lated thigh claudication as a result of fibromuscular dysplasia of
the deep femoral artery. J Vasc Surg 15:657-660, 1992
10. Neukirch C, Bahnini A, Delcourt A, et al: Popliteal
aneurysm due to fibromuscular dysplasia. Ann Vasc Surg 10:
578-581, 1996
11. Shipolini AR, Wolfe JH: Fibromuscular dysplasia and
aneurysm formation in the brachial artery. Eur J Vasc Surg
7:740-743, 1993
12. Stewart MT, Moritz MW, Smith RB, et al: The natural
history of carotid fibromuscular dysplasia. J Vasc Surg 3:305-
310, 1986
13. Stanley JC, Gewertz BL, Bove EL, et al: Arterial fibroplasia,
histopathologic character and current etiologic concepts.
Arch Surg 110:561-566, 1975
14. Rushton AR: The genetics of fibromuscular dysplasia.
Arch Int Med 140:233-236, 1980
15. Stanley JC, Whitehouse WM Jr, Graham LM: Operative
therapy for renovascular hypertension. Br J Surg 69:S63, 1982
16. Karas RH, Caur W, Tassi L, et al: Inhibition of vascular
smooth muscle cell growth by estrogen. Circulation 88:I 325,
17. Vargas R, Wroblewska B, Rego A, et al: Oestradiol
inhibits smooth muscle cell proliferation of pig coronary artery.
Br J Pharmacol 9:612, 1993
18. Stanley JC, Gewertz BL, Fry WJ: Renal: systemic renin
indicies and renal vein renin ratios as prognostic indicatiors in
remedial renovascular hypertension. J Surg Res 20:149, 1976
19. Sarkar R, Messina LM: Renovascular disease. Pathology
of renal artery occlusive disease, in Ernst CB, Stanley JC (eds):
Current Therapies in Vascular Surgery (ed 3). St. Louis, MO,
Mosby, 1985, pp 764-774
20. Sottiurai V, Fry WJ, Stanley JC: Ultrastructural characteristic
of experimental arterial medial fibroplasia induced by
vasa vasorum occlusion. J Surg Res 24:169, 1978
21. Messina LM, Stanley JC: Renal artery fibrodysplasia and
renovascular hypertension, in Rutherford RB (ed): Vascular
Surgery (ed 5). Philadelphia, WB Saunders, 2000, pp 1650-1655
22. Kaufman JJ, Maxwell MH: Upright aortography in the
study of nephroptosis, stenotic lesions and hypertension. Surgery
53:736-742, 1963
23. Leung DYM, Glagov S, Matthews MB: Cyclical stretching
stimulates synthesis of matrix components by arterial
smooth muscle cells in vitro. Science 191:475-477, 1976
24. Bigazzi R, Bianchi S, Quilici N, et al: Bilateral fibromuscular
dysplasia in identical twins. Am J Kidney Dis 32:E4,
25. Halpern MM, Sanford HS, Viamonte M Jr: Renal artery
abnormalitied in three hypertensive sisters: probable familial
fibromuscular hyperplasia. JAMA 194:512-513, 1965
26. Nicholson JP, Teichman SL, Alderman MH, et al: Cigarette
smoking and renovascular hypertension. Lancet 2:765-
766, 1983
27. Sang CN, Whelton PK, Hamper UM, et al: Etiologic
factors in renovascular fibromuscular dysplasia, a case control
study. Hypertension 14:472-479, 1989
28. DeMendonca WC, Espat PA: Pheochromoctoma associated
with arterial fibromuscular dysplasia. Am J Clin Pathol
75:749-754, 1981
29. Brewster DC, Jensen SR, Novelline RA: Reversible
renal artery stenosis associated with pheochromocytoma.
JAMA 248:1094-1096, 1982
30. Fievez M, Philippart F, Hustin J: Ergotism: Anatomoclinical
study of a case. Angiology 26:491-498, 1975
31. Regan JF, Poletti BJ: Vascular adventitial fibrosis in a
patient taking methysergide maleate. JAMA 203:1069-1071,
32. Schievink WI, Meyer FB, Parisi JE, et al: Fibromuscular
dysplasia of the internal carotid artery associated with alpha-1-
antitrypsin deficiency. Neurosurgery 43:229-234, 1998
33. Begelman SM, Olin JW: Fibromuscular dysplasia. Curr
Opin Rheumatol 12:41-47, 2000
34. Harrison EG, McCormack LJ: Pathological classification
of renal arterial disease in renovascular hypertension. Mayo
Clin Proc 46:161-167, 1971
35. Goncharenko V, Gerlock AJ Jr, Shaff MI, et al: Progression
of renal artery fibromuscular dysplasia in 42 patients as
seen on angiography. Radiology 139:45-51, 1981
36. Schievink WI, Bjornsson J: Fibromuscular dysplasia of
the internal carotid artery: A clinicopathological study. Clin
Neuropathol 15:2-6, 1996
37. Alimi Y, Mercier C, Pellissier JF, et al: Fibromuscular
disease of the renal artery, a new histopathological classification.
Ann Vasc Surg 6:220-224, 1992
38. Olin JW, Novick AC: Renovascular disease, in Young
JR, Olin JW, Bartholomew JR (eds): Peripheral Vascular Diseases
(ed 2). St. Louis, MO, Mosby, 1996, pp 321-342
39. Anderson CA, Hansen JK, Benjamin ME, et al: Renal
artery fibromuscular dysplasia: Results of current surgical therapy.
J Vasc Surg 22:207, 1995
40. Novick AC, Ziegelbaum M, Vidt DG, et al: Trends in
surgical revascularization for renal artery disease: Ten years
experience. JAMA 257:498, 1987
41. Brookstein JJ, Walter JF, Stanley JC, et al: Pharmacoangiographic
manipulation of renal collateral blood flow. Circulation
54:328, 1976
42. Ernst CB, Brookstein JJ, Montie J, et al: Renal vein renin
ratios and collateral vessels in renovascular hypertension. Arch
Surg 104:496, 1972
43. Luscher TF, Greminger P, Kuhlmann TJ, et al: Renal
venous renin determinations in renovascular hypertension. Diagnostic
and prognostic value in unilateral renal artery stenosis
treated by surgery or percutaneous tranluminal angioplasty.
Nephron 44:17, 1986 (suppl 1)
44. Stanley JC, Fry WJ: Renovascular hypertension secondary
to arterial fibrodysplasia in adults: Criteria for operation and
results of surgical therapy. Arch Surg 110:922, 1975
45. Mounier-Vehier C, Haulon S, Devos P, et al: Renal
atrophy outcome after revascularization in fibromuscular dysplasia
disease. J Endovasc Ther 9:605-613, 2002
46. Monunier-Vehier C, Lions C, Jaboureck O, et al: Parenchymal
consequences of fibromuscular dysplasia renal artery
stenosis. Am J Kidney Dis 40:1138-1145, 2002
47. Buhler FR, Laragh JH, Baer L, et al: Propranolol inhibition
of renin secretion. N Engl J Med 287:1209, 1972
48. Chrysant SG, Dunn M, Marples M, et al: Severe reversible
azotemia from captopril therapy: Report of three cases and
review of the literature. Arch Intern Med 143:347, 1983
49. Hricik DE, Browning PK, Kopelman R, et al: Captoprilinduced
renal insufficiency in patients with bilateral renal artery
stenosis or renal artery stenosis in a solitary kidney. N Engl
J Med 308:373, 1983
50. Gruntzig A, Vetter W, Meier B, et al: Treatment of
renovascular hypertension with percutaneous transluminal dilation
of a renal artery stenosis. Lancet 1:801, 1978
51. Kerlan RK: Angioplasty, In: LaBerge JL (eds): Interventional
Radiology Essentials, Philadelphia, PA, Lippincott William
& Wilkins, 2000, pp 147-164
52. Birrer M, Do DD, Mahler F, et al: Treatment of renal
artery fibromuscular dysplasia with balloon angioplasty: a prospective
follow-up study. Eur J Vasc Endovasc Surg 23:146-
152, 2002
53. Mahler F, Triller J, Weidmarim P, et al: Complications
in percutaneous transluminal dilation of renal arteries. Nephron
44:60, 1986 (suppl 1)
54. Stanley JC, Whitehouse WM Jr, Zelenock GB, et al:
Reoperation for complications of renal artery reconstructive
surgery undertaken for treatment of renovascular hypertension.
J Vasc Surg 2:133, 1985
55. Stanley JC, Ernst CB, Fry WJ: Fate of 100 aortorenal
vein grafts: Characteristics of late graft expansion, aneurysmal
dilation and stenosis. Surgery 74:931, 1973
56. Lagneau P, Michel JB, Charrat JM: Use of polytetrafluoroethylene
grafts for renal bypss. J Vasc Surg 5:738, 1987
57. Kaufmann JJ: Long-term results of aortorenal Dacron
grafts in the treatment of renal artery stenosis. J Urol 111:298,
58. Novick AD, Steward BH, Straffon RA, et al: Autogenous
arterial grafts in the treatment of renal artery stenosis.
J Urol 118:919, 1977
59. Stoney RJ, Cooke PA, String ST: Surgical treatment of
renovascular hypertension in children. J Ped Surg 10:631, 1975
60. Khauli RB, Novick AC, Ziegelbaum M: Splenorenal
bypass in the treatment of renal artery stenosis: Experience with
sixty-nine cases. J Vasc Surg 2:547, 1985
61. Stanley JC, Zelenock GB, Messina LM, et al: Pediatric
renovascular hypertension: A thirty year experience of operative
treatment. J Vasc Surg 21:212, 1995
62. Palubinskas AJ, Ripley HR: Fibromuscular hyperplasia
in extrarenal arteries. Radiology 82:451-455, 1964
63. Connett M, Lansche JM: Fibromuscular hyperplasia of
the internal carotid artery: Report of a case. Ann Surg 162:59,
64. Effeney DJ, Krupski WC, Stoney RJ, et al: Fibromuscular
dysplasia of the carotid artery. Aust N Z Surg 53:527-531,
65. Collins GJ, Rich NM, Clagett GP, et al: Fibromuscular
dysplasia of the internal carotid arteries. Ann Surg 194:89-96,
66. Andersen CA, Collins GJ, Rich NM, et al: Spontaneous
dissection of the internal carotid artery associated with fibromuscular
dysplasia. Am Surg 78:263-266, 1980
67. Rhee RY, Gloviczki P, Cherry KJ Jr, et al: Two unusual
variants of internal carotid artery aneurysms due to fibromuscular
dysplasia. Ann Vasc Surg 10:481-485, 1996
68. Duncan MA, Dowd N, Rawluk D, et al: Traumatic
bilateral internal carotid artery dissection following airbag deployment
in a patient with fibromuscular dysplasia. B J Anaesth
85:476-78, 2000
69. Eachempati SR, Sebastian MW, Reed RL II: Posttraumatic
bilateral carotid artery and right vertebral artery dissections
in a patient with fibromuscular dysplasia: Case report and
review of the literature. J Trauma 44:406-409, 1998
70. Wesen CA, Elliott BM: Fibromuscular dysplasia of the
carotid arteries. Am J Surg 151:448-451, 1986
71. Corrin LS, Sandok BA, Houser OW: Cerebral ischemic
events in patients with carotid artery fibromuscular dysplasia.
Arch Neurol 38:616-618, 1981
72. Cloft HJ, Kallmes DF, Kallmes MH, et al: Prevalance of
cerebral aneurysms in patients with FMD: A reassessment.
J Neurosurg 88:436-440, 1998
73. So EL, Toole JF, Dalal P, et al: Cephalic fibromuscular
dysplasia in 32 patients: Clinical findings and radiological
features. Arch Neurol 38:619, 1981
74. Effeney DJ, Ehrenfeld WK, Stoney RJ, Wylie EJ: Why
operate on carotid fibromuscular dysplasia? Arch Surg 115:
1261-1265, 1980
75. Collins GJ Jr, Hobson RW II, Rich NM, et al: Arterial
dilator-shunt for use in carotid artery fibromuscular hyperplasia.
Am J Surg 130:381-385, 1975
76. Smith LL, Smith DC, Killeen JD, et al: Operative ballon
angioplasty in the treatment of internal carotid artery fibromuscular
dysplasia. J Vasc Surg 6:482-491, 1987
77. Liu AY, Paulsen RD, Marcellus ML, et al: Long term
outcomes after carotid stent placement for treatment of carotid
artery dissection. Neurosurgery 45:1368-1374, 1999
78. Chiche L, Bahnini A, Koskas F, et al: Occlusive fibromuscular
disease of arteries supplying the brain: Results of
surgical treatment. Ann Vasc Surg 11:496-504, 1997
79. Muller BT, Luther B, Hort W, et al: Surgical treatment of
50 carotid dissections: Indications and results. J Vasc Surg
31:980-988, 2000

<< Prev Topic | Next Topic >>

Add Reply

Email This To a Friend Email This To a Friend
Topic Control Image Topic Commands
Click to receive email notification of replies Click to receive email notification of replies
Click to stop receiving email notification of replies Click to stop receiving email notification of replies
jump to:

- FMD Vascular - Vascular Disorders - Home -

Disclaimer of Information

Double left click on any word in this forum for a definition of that word (not a link)

Powered By ezboard® Ver. 7.32
Copyright ©1999-2005 ezboard, Inc.