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"Fibromuscular dysplasia: When is intervention warrante

"Fibromuscular dysplasia: When is intervention warranted?" 1 Thomas
K. Currya, Louis M. Messina* Seminars in Vascular Surgery. September
2003 • Volume 16 • Number 3

www2.us.elsevierhealth.co...dll/serve?
action=searchDB&searchDBfor=art&artType=full&id=as0895796703000243

Abstract TOP

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. [currently
tendency avoid surgery push window angioplasty, stents – result
patients losing kidneys]

FIBROMUSCULAR DYSPLASIA (FMD) is a 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 artery. [FMD arteries only]

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. Intra- and 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
arteries.

Etiologic factors TOP

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 [wrong –
proof new studies?]

Ischemia is thought to be 1 factor in the development of FMD.19 The
arteries most likely to 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 media-adventitial 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 [indicates racial disparity healthcare]

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

Pathology TOP

The pathological classification of renal FMD was proposed by Harrison
and McCormick in 1971 34 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.
----------------------------------------------------------------------
----------
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 arteries
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

----------------------------------------------------------------------
----------
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 arranged 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.
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.

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

Renal artery disease TOP

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 angiotensin-converting 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 renal function from medication or
progression of disease.

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. [see above – use ACE inhibitors can worsen
renal function]

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. [intervntn only cert cases
older ptnts]

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, fibrosis, 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 dissections.21

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 operation.

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 cylindrical-tipped 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.61

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 beneficial response rates remain similar to earlier series,
the cure rate is generally lower.

Carotid FMD TOP

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
FMD.74

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. [compare to insurance actuarial
reports and analysis] 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

Treatment

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 ischemic 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 disease.78

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

Other vessels TOP

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.

Conclusion TOP

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.

References TOP
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aDivision of Vascular Surgery, Department of Surgery, University of
California, San Francisco, CA, USA
1Competition 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, USA
Copyright © 2003 by Elsevier Science Inc.
doi:10.1016/S0895-7967(03)00024-3


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