Bilateral brachial artery fibromuscular dysplasia

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that usually involves medium- and small-sized arteries. It is most commonly observed in the renal, carotid, and intracerebral arteries, although it has been reported in other arterial beds. The most common form is characterized by medial fibrosis, with or without smooth muscle cell hyperplasia, which can result in luminal narrowing and turbulent flow. There is often a secondary aneurysmal degeneration of the artery, which may or may not be associated with thrombosis or obstruction of flow. This accounts for the typical string-of-beads appearance seen on arteriography. We describe a patient who presented with ischemia of the right hand secondary to fibromuscular dysplasia of the brachial artery. Subsequent studies also demonstrated fibromuscular dysplasia in the other brachial artery as well as mild involvement of the right renal artery. The patient was treated on the symptomatic side with dilatation of proximal lesions, resection of the thrombosed segment, and reconstruction with a reversed saphenous vein graft. Distal pulses were fully restored postoperatively. Pathologic examination confirmed the arteriographic and clinical diagnosis of fibromuscular dysplasia. The salient features of this case are reviewed in addition to the other cases reported in the literature.     

Isolated thigh claudication as a result of fibromuscular dysplasia of the deep femoral artery.

Isolated thigh claudication as a result of fibromuscular dysplasia of the deep femoral artery has not previously been reported. This case report describes a patient with fibromuscular dysplasia of the carotid arteries in whom progressive unilateral thigh claudication developed despite normal femoral pulses. Deep femoral artery occlusion caused by fibromuscular dysplasia was successfully treated by common femoral to distal deep femoral artery bypass. Fibromuscular dysplasia of the infrainguinal arteries is rare but should be included as a possible cause of lower extremity ischemic symptoms.     

Rapid Progression of Native Renal Artery Fibromuscular Dysplasia Following Kidney Donation

Fibromuscular dysplasia is the second commonest anatomical abnormality apart from multiple renal arteries in the potential live donors. Pretransplant evaluation of the donors may include an angiography to evaluate the renal arteries, and failure to recognize renal arterial stenosis, particularly fibromuscular dysplasia, by noninvasive methods may eventually lead to hypertension and ischemic renal failure. We report a case of fibromuscular dysplasia that was undetected by computed tomographic angiography prior to donation. One year after kidney donation, it rapidly progressed to severe symptomatic stenosis with hypertension and acute renal failure. Following renal artery angioplasty, her blood pressure normalized over a period of 2 weeks without any need for antihypertensive medications and the serum creatinine returned to her baseline. The acceptability of renal donors with fibromuscular dysplasia depends on the age, race and the availability of the other suitable donors. Mild fibromuscular dysplasia in a normotensive potential renal donor cannot be considered a benign condition. Such donors need regular follow-up postdonation for timely detection and treatment.     

Fibromuscular dysplasia: noninvasive evaluation of unusual case of renal and mesenteric involvement

Fibromuscular dysplasia is the most common cause of renovascular hypertension in young patients. It primarily involves the renal and carotid arteries, and it is less common in the coronary, iliac, and visceral arteries. Digital subtraction angiography is still the best investigation to determine the location, extent, and complications of renal artery involvement. However, currently, other imaging modalities such as ultrasonography, computed tomography, and magnetic resonance imaging can reveal these findings noninvasively. We present the case of a 43-year-old woman who presented with high blood pressure and headache. Imaging revealed fibromuscular dysplasia of the renal arteries and the superior mesentery artery.     

Digital artery embolization as a result of fibromuscular dysplasia of the brachial artery

A 74-year-old woman was admitted to the hospital with sudden onset of a blue, painful right index finger, and the condition progressed to digital ulceration. The ulcer gradually healed over a period of 2 months. Peripheral pulses, Doppler-derived brachial and radial systolic pressures, and results of an echocardiogram were normal. Digital pulse volume recordings showed obstructive waveforms in all digits of the right hand. Arteriography showed a normal arch and normal innominate, subclavian, and axillary arteries. The midbrachial artery was markedly abnormal and had alternating areas of stenosis and aneurysm formation. Multiple occlusions involved the palmar arch and proper digital arteries. The abnormal brachial artery segment was excised and replaced with an autogenous reversed saphenous-vein conduit. Histologic examination confirmed the lesion to be medial fibromuscular dysplasia. Fibromuscular dysplasia that involves upper extremity arteries is extremely uncommon and rarely presents with digital artery embolization. This case emphasizes the importance of exclusion of proximal reconstructible arterial occlusive disease by means of complete arteriographic examination of patients who were admitted with unilateral finger or hand ischemia.     

Fibromuscular dysplasia of the carotid arteries

Fibromuscular disease of the carotid artery was identified in 30 patients, which represented 3.2 percent of all patients who had cerebral angiography at Brooke Army Medical Center in the 6 year period from 1978 to 1984. Focal neurologic events were the presenting symptoms in 63 percent of the patients. The majority of the patients were treated with antiplatelet therapy, and eight patients had a total of 10 carotid artery dilatations. The only patients with recurrent symptoms were those who received either no treatment or antiplatelet therapy. There were no recurrent symptoms in the operated patients. This study suggests that surgical treatment for the symptomatic patient may prevent recurrent symptoms with an acceptably low morbidity and mortality. There was, however, no indication that prophylactic dilation of the fibromuscular disease in the asymptomatic patient was beneficial. Fibromuscular dysplasia of the carotid arteries is often associated with intracranial aneurysms, and surgical therapy rather than antiplatelet therapy may be advisable in patients who have intracranial aneurysms. Patients with concomitant atherosclerosis of the carotid artery bifurcation should be treated like any patient with atherosclerotic disease and an endarterectomy should be performed with carotid dilatation when indicated. Fibromuscular disease of the carotid artery is an infrequent angiographic finding that is associated with focal and global neurologic symptoms. Most patients can be effectively treated with antiplatelet drugs with no recurrent symptoms, however, for persistent or progressive symptoms, some patients will require surgical dilatation of the carotid artery. Fibromuscular disease of the carotid artery may lead to catastrophic symptoms of stroke or intracranial hemorrhage if left undiagnosed or untreated.     

Fibromuscular dysplasia of the renal artery: Management and outcome

SUMMARY: Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. the outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). the mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months-10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. the other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

Fibromuscular dysplasia of the renal artery: Management and outcome

Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. The outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). The mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months–10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. The other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

Isolated Iliac Artery Aneurysm Caused by Fibromuscular Dysplasia: Report of a Case

Fibromuscular dysplasia (FMD) can develop in many different arteries, but iliac artery aneurysms are rare. A 69-year-old Japanese woman was admitted to our hospital for treatment of a right common iliac artery aneurysm. Aortography revealed aneurysms in both the right common iliac artery and the left internal iliac artery. Notably, the right common iliac artery aneurysm had a string-of-beads appearance. At surgery, the aneurysms were resected, and replaced with Y-shaped vascular prostheses. The histopathological diagnosis was fibromuscular dysplasia (FMD). We report this case of common iliac artery aneurysm caused by FMD due to its rarity.     

Fibromuscular dysplasia of the renal arteries: a radiological review

Fibromuscular dysplasia is the most common cause of renovascular hypertension in young patients. Digital subtraction angiography is still the best investigation used to determine the location, extent and complication of renal artery involvement. String of beads appearance (reflecting multiple stenoses), aneurysms, focal or tubular stenosis are classic angiographic appearances. The aim of this pictorial essay is to illustrate the various imaging findings of renal artery fibromuscular dysplasia.     

Endovascular therapy for limb salvage in a case of critical lower limb ischemia resulting from fibromuscular dysplasia

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that mostly affects the renal and internal carotid arteries, rarely complicating lower limbs. We report a case of FMD complicating critical limb ischemia due to the obstruction of below the knee arteries, diagnosed and treated with the support of skin perfusion pressure (SPP) measurement. Initial angiogram revealed the anterior tibial artery (ATA) was subtotally occluded showing a "strings of beads" pattern, which is typical of the arteries affected by FMD. SPP guided endovascular therapy was successful for ATA lesions. Intractable rest pain subsequently disappeared and the ulcer was completely healed without amputation.     

A problem in hemodialysis management: fibromuscular dysplasia of radial and ulnar arteries--report of a case.

Fibromuscular dysplasia (medial type) of the radial and ulna arteries, hitherto unreported, has been demonstrated angiographically following the malfunction of an arteriovenous shunt created for dialysis. Retrograde dilatation of the segmental stenoses caused some improvement in flow. No causal relationship is claimed between fibromuscular dysplasia and the arteriovenous shunt.     

Fibromuscular dysplasia of bilateral brachial arteries treated with surgery and consecutive thrombolytic therapy

A 61-year-old female was admitted to our hospital complaining of paresthesia, pain, and intermittent weakness in the right hand. A pulsating mass with bruits had developed on the patient's upper arm. We also noted an absence of radial artery pulsation. The angiographic findings revealed a classic "string of beads" appearance, which involved both brachial and renal arteries. The right brachial artery exhibited an aneurysm, which was filled with thrombus, and the distal radial artery was occluded with thromboemboli. We excised the abnormal brachial artery segment, replacing it with an autogenous reversed saphenous vein conduit. Consecutive thrombolytic therapy was then performed for the treatment of the radial artery embolism. Histological examination revealed that the patient was suffering from medial fibromuscular dysplasia. This uncommon form of fibromuscular dysplasia, which involves both brachial arteries with embolization, can be efficiently treated via surgery and consecutive thrombolytic therapy.     

Fibromuscular Dysplasia of the Superior Mesenteric Artery-Case Report and Review of the Literature

Fibromuscular dysplasia is a multifactorial arteriopathy most commonly affecting the renal and carotid arteries. In this report we present a case of visceral artery involvement, causing occlusion of the superior mesenteric artery and celiac trunk and resulting in visceral ischemia. Treatment consisted of superior mesenteric artery reimplantation. Visceral artery FMD can present as occlusive or aneurysmal disease and treatment depends on patient characteristics and symptoms.     

Fibromuscular dysplasia of the superior mesenteric artery--case report and review of the literature

Fibromuscular dysplasia is a multifactorial arteriopathy most commonly affecting the renal and carotid arteries. In this report we present a case of visceral artery involvement, causing occlusion of the superior mesenteric artery and celiac trunk and resulting in visceral ischemia. Treatment consisted of superior mesenteric artery reimplantation. Visceral artery FMD can present as occlusive or aneurysmal disease and treatment depends on patient characteristics and symptoms.     

Renal fibromuscular dysplasia

Renal artery fibromuscular dysplasia is a noninflammatory, nonatherosclerotic vasculopathy that can affect renal arteries at various degrees with different severity. The etiology is still unknown, but there is a strong belief that a genetic disorder is the main cause for the pathogenesis of this disease. The main presentation is a sudden onset of recalcitrant hypertension at a young age, which is usually resistant to medical treatment. Once renal artery fibromuscular dysplasia is suspected, several diagnostic tools are available to make an accurate diagnosis. The advent of minimally invasive interventions has revolutionized the options for treatment. This update should provide the clinician with a base understanding of available evidence for diagnosing and treating renal artery fibromuscular dysplasia.     

An unusual case of fibromuscular dysplasia

The case of a patient with abdominal angina due to fibromuscular dysplasia is described. Arteriography revealed dysplasia of both renal arteries and occlusion at their origins of both coeliac and mesenteric arteries. Digital subtraction angiography later showed fibromuscular dysplasia of both carotid artery systems. An unusually long right internal iliac artery allowed it to be anastomosed to the superior mesenteric artery. Follow-up studies have confirmed patency of this reconstruction.     

Fibromuscular Hyperplasia of the Iliac Arteries

Fibromuscular hyperplasia is an uncommon disease of arteries with an unknown (etiology and an obscure natural history. It occurs rarely in the iliac vessels and may cause intermittent claudication. Two cases of fibromuscular hyperplasia of these arteries are reported. Both patients are middle-aged women. One presented with intermittent claudication and has been treated by aortofemoral bypass. The other is at present symptomless; the diagnosis was made in the course of the investigation of an abdominal bruit. Some features of the condition arc discussed.     

Diagnosis, management, and future developments of fibromuscular dysplasia

Fibromuscular dysplasia (FMD) is a nonatherosclerotic noninflammatory vascular disease that primarily affects women from age 20 to 60, but may also occur in infants and children, men, and the elderly. It most commonly affects the renal and carotid arteries but has been observed in almost every artery in the body. FMD has been considered rare and thus is often underdiagnosed and poorly understood by many health care providers. There are, however, data to suggest that FMD is much more common than previously thought, perhaps affecting as many as 4% of adult women. When it affects the renal arteries, the most common presentation is hypertension. When it affects the carotid or vertebral arteries, the patient may present with transient ischemic attack or stroke, or dissection. An increasing number of patients are asymptomatic and are only discovered incidentally when imaging is performed for some other reason or by the detection of an asymptomatic bruit. FMD should be considered in the differential diagnosis of a young person with a cervical bruit; a "swishing" sound in the ear(s); transient ischemic attack, stroke, or dissection of an artery; or in individuals aged ≤ 35 years with onset hypertension. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with aneurysms should be treated with a covered stent or open surgical repair. Little new information has been published about FMD in the last 40 years. The recently instituted International Registry for Fibromuscular Dysplasia will remedy that situation and provide observational data on a large numbers of patients with FMD.     

Fibromuscular disease of the renal artery: A new histopathologic classification

To evaluate the relationship between clinical, radiologic, and histopathologic features in various types of renal artery fibromuscular disease, the records of 37 patients operated upon for a total of 44 lesions of the renal artery (30 unilateral and seven bilateral) were retrospectively reviewed. Of these, 36 had hypertension associated with stenosis or aneurysm of the trunk or branches of the renal artery; one had aneurysm of the renal artery without hypertension. The histopathologic study included 38 arterial segments retrieved from 33 patients and underscored the difficulties in using topographic criteria to classify lesions. While all arterial aneurysms were associated with involvement of the media, more than one layer of the artery was involved in 65.7% of cases. Topographic forms, such as the perimedial type, were difficult to classify. Based on the study results, it appears essential to distinguish patients with fibrosis and smooth muscular hyperplasia (type I: 19 patients, 23 arteries) from patients with isolated fibrosis (type II: 14 patients, 15 arteries). In group II, preoperative hypertension was more long-standing (p<0.04), more lesions were extended distally (p<0.05), and the probability of recovery from hypertension at 52 months follow-up was lower. We have distinguished two different evolutive stages of fibromuscular disease of the renal artery, one characterized by fibrosis with smooth muscle hyperplasia and the second, associated with more severe prognosis, characterized by isolated fibrosis.Presented at the Annual Meeting of the Société de Chirurgie Vasculaire de Langue Française, June 20–21, 1991, Marseille, France.