Multivisceral Fibromuscular Dysplasia in Childhood: Case Report and Review of the Literature

We report here a 9-year-old girl with fibromuscular dysplasia of many muscular arteries including both renal and internal carotid arteries, the celiac artery, superior mesenteric artery, and one external carotid artery. She suffered from severe renovascular hypertension with beginning secondary cardiac decompensation, typical angina abdominalis, and neurological signs, including severe headaches and hemianopsia. Surgery was performed for all major vessels and the outcome is good 2.5 years after the operation. The clinical presentation, differential diagnosis, and treatment options of fibromuscular dysplasia in childhood are discussed and the literature is reviewed.     

Fibromuscular dysplasia associated with simultaneous spontaneous dissection of four peripheral arteries in a 30-year-old man

A 30-year-old man had a sudden bout of severe abdominal pain. An enhanced computed tomographic scan revealed dissections of the celiac artery, superior mesenteric artery, left renal artery, and right external iliac artery; stenosis of the right renal artery; and left kidney infarction. After careful evaluation, the patient was diagnosed with fibromuscular dysplasia (medial dysplasia), based on the findings obtained from the enhanced computed tomographic scan. This case is extremely rare because fibromuscular dysplasia occurred concurrently with simultaneous spontaneous dissections of four peripheral arteries in a young man.     

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.     

Successful surgical treatment of a patient with multiple visceral artery aneurysms due to fibromuscular dysplasia

Multiple visceral artery aneurysms due to fibromuscular dysplasia are rare. A 43-yr-old man with a pulsatile abdominal mass detected by ultrasonography had multiple visceral artery aneurysms diagnosed by angiography. This included a huge superior mesenteric artery aneurysm. Aneurysm resection and arterial reconstruction was performed successfully. Pathologic examination revealed fibromuscular dysplasia of the medial fibroplasia type.     

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.     

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.     

Fibromuscular dysplasia of the brachial artery: a case report and review of the literature.

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily medium-sized and small arteries. Fibromuscular dysplasia is characterized by medical fibrosis with or without smooth muscle cell hyperplasia and may produce luminal impingement with severe turbulence. Secondary aneurysmal deformity with or without thrombosis may also contribute to the obstruction. Fibromuscular dysplasia most commonly involves the renal and carotid arteries, with upper-extremity disease rarely reported. This case report describes a patient with digital embolization from brachial artery fibromuscular dysplasia. Angiography demonstrated significant narrowing and irregularity with a characteristic "string-of-beads" appearance of the right midbrachial artery. The abnormal segment was resected and reconstructed with a reversed saphenous vein graft. Histologic studies revealed disruption of the internal elastic lamina and disorientation of the hyperplastic medial smooth muscle cells characteristic of fibromuscular dysplasia.     

Renal arteriovenous fistula associated with fibromuscular dysplasia

A case is reported of a woman with long-standing progressive hypertension and an abdominal bruit who on angiography demonstrated fibromuscular dysplasia of the right renal artery and an arteriovenous fistula in the upper pole of the right kidney. The various causes of renal arteriovenous fistula are reviewed. The relationship of mural aneurysms seen in fibromuscular dysplasia and the formation of arteriovenous fistulas by rupture of these aneurysms is discussed. Review of the English literature indicates a probable causal relationship between fibromuscular dysplasia of the renal arteries and intrarenal arteriovenous fistulas.     

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.     

Traumatic occlusion in fibromuscular dysplasia of the carotid artery

A patient with fibromuscular dysplasia involving the cervical carotid artery developed subtotal occlusion after a trivial head injury. An association between occlusion in fibromuscular dysplasia-diseased carotid arteries and trivial blunt head injuries is suggested and appropriate treatment options are discussed.     

Young women presenting with chronic mesenteric ischemia--two case reports

Chronic mesenteric ischemia is primarily a disease of the elderly, who have widespread atherosclerosis of the celiac, superior mesenteric, and inferior mesenteric arteries. Risk factors include smoking, vasculitis, fibromuscular dysplasia, and arterial dissection. Presented here are 2 cases of women under age 50 years burdened with this condition, owing primarily to advanced arteriosclerosis. These cases illustrate a need for increased awareness of mesenteric ischemia as a cause for postprandial abdominal pain in young patients with other stigmata of advanced arterial occlusive disease.     

A preliminary study of the role of duplex scanning in defining the adequacy of treatment of patients with renal artery fibromuscular dysplasia.

Renal artery fibromuscular dysplasia is a well-known cause of hypertension whose presence is confirmed by its typical arteriographic appearance. The functional significance of these lesions is often difficult to determine, particularly when both renal arteries are involved. Duplex scanning has been shown to be accurate for the detection of renal artery stenosis and estimation of the degree of narrowing. To test whether duplex scanning results after intervention correlate with clinical outcome, we reviewed the studies on nine patients with renal artery fibromuscular dysplasia who had been treated by either angioplasty or surgery. A total of 18 arteries were treated. Fourteen of the 18 treatments were successful as measured by a reduction in blood pressure and antihypertensive drugs. In four instances treatment was unsuccessful. For those patients who had clinical improvement, the hemodynamic parameters from the renal artery also improved. In the patients for whom treatment failed, the velocities recorded from the site of narrowing did not improve after intervention. Thus it appears that duplex scanning along with the clinical results may be used to document the basis for failing to improve after treatment be it angioplasty or operation.     

Imaging modalities for renal artery stenosis in suspected renovascular hypertension: prospective intraindividual comparison of color Doppler US, CT angiography, GD-enhanced MR angiography, and digital substraction angiography

The aim of the study was to evaluate the diagnostic accuracy of Color Doppler US, CT Angiography (CTA), and GD-enhanced MR Angiography (MRA) compared with digital subtraction angiography (DSA) for the detection of renal artery stenosis in patients with clinically suspected renovascular hypertension. Fifty-eight patients with suspected renovascular hypertension were enrolled in the study. All patients underwent Color Doppler US, CTA and GD-enhanced MRA. DSA was the gold standard method for the number of renal arteries, existence and degree of stenosis, or evidence of fibromuscular dysplasia. DSA depicted 132 renal arteries, 16 stenoses, and 4 arteries with fibromuscular dysplasia. Color Doppler US failed to detect 1 main and 14 polar arteries. CTA depicted all main renal arteries and 7/16 polar arteries, but failed to detect stenosis in two accessory vessels. Likewise, MRA did not detect stenotic accessory renal arteries, depicted 9/16 polar renal arteries, but missed two main renal arteries. All methods depicted the four main renal arteries with fibromuscular dysplasia. The overall sensitivity, specificity, and positive and negative predictive accuracy were 75%, 89.6%, 60% and 94.6%, respectively, for color Doppler US; 94%, 93%, 71%, and 99%, respectively, for CTA; and 90%, 94.1%, 75%, and 98%, respectively, for GD-enhanced MRA. CTA and GD-enhanced MRA have comparable and satisfactory results with respect to the negative predictive accuracy of the suspected renal artery stenosis. The concept of an imaging algorithm including US as screening test when appropriate and CTA or MRA as the second step-procedure is suggested. Therefore, DSA may be reserved for cases with major discrepancies or therapeutic interventions.     

Imaging Modalities for Renal Artery Stenosis in Suspected Renovascular Hypertension: Prospective Intraindividual Comparison of Color Doppler US,CT Angiography,GD-Enhanced MR Angiography,and Digital Substraction Angiography

The aim of the study was to evaluate the diagnostic accuracy of Color Doppler US, CT Angiography (CTA), and GD-enhanced MR Angiography (MRA) compared with digital subtraction angiography (DSA) for the detection of renal artery stenosis in patients with clinically suspected renovascular hypertension. Fifty-eight patients with suspected renovascular hypertension were enrolled in the study. All patients underwent Color Doppler US, CTA and GD-enhanced MRA. DSA was the gold standard method for the number of renal arteries, existence and degree of stenosis, or evidence of fibromuscular dysplasia. DSA depicted 132 renal arteries, 16 stenoses, and 4 arteries with fibromuscular dysplasia. Color Doppler US failed to detect 1 main and 14 polar arteries. CTA depicted all main renal arteries and 7/16 polar arteries, but failed to detect stenosis in two accessory vessels. Likewise, MRA did not detect stenotic accessory renal arteries, depicted 9/16 polar renal arteries, but missed two main renal arteries. All methods depicted the four main renal arteries with fibromuscular dysplasia. The overall sensitivity, specificity, and positive and negative predictive accuracy were 75%, 89.6%, 60% and 94.6%, respectively, for color Doppler US; 94%, 93%, 71%, and 99%, respectively, for CTA; and 90%, 94.1%, 75%, and 98%, respectively, for GD-enhanced MRA. CTA and GD-enhanced MRA have comparable and satisfactory results with respect to the negative predictive accuracy of the suspected renal artery stenosis. The concept of an imaging algorithm including US as screening test when appropriate and CTA or MRA as the second step-procedure is suggested. Therefore, DSA may be reserved for cases with major discrepancies or therapeutic interventions.     

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.     

Symptomatic hydronephrosis from renal artery aneurysm associated with fibromuscular dysplasia: management with transarterial embolization

A 56-year-old woman presented with right-sided flank pain, dizziness, and nausea. Ultrasonography revealed hydronephrosis of the right kidney. CT showed a nodular mass at the ureteropelvic junction. Subsequent CT angiography revealed the nodular mass as a distal aneurysm of the right renal artery compromising the ureteropelvic junction. Selective diagnostic angiography confirmed the diagnosis of a distal renal artery aneurysm. Moreover, angiography incidentally detected fibromuscular dysplasia of both renal arteries. The renal artery aneurysm was then successfully managed by transarterial embolization using detachable bioactive coils.     

Fibromuscular dysplasia in the extremities

Four patients had fibromuscular dysplasia of the extremities involving the brachial, deep femoral, popliteal, anterior and posterior tibial and peroneal arteries. One of them demonstrated a string-of-beads appearance in the superficial veins of the arm and this was pathologically confirmed as fibromuscular dysplasia. We believe this is the first report of this disease affecting the peripheral arteries and the superficial veins of the extremities. The sympathetic system seems to be related to the development of fibromuscular dysplasia.     

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.     

Operative balloon angioplasty in the treatment of internal carotid artery fibromuscular dysplasia

Seven patients with symptomatic fibromuscular dysplasia have had eight internal carotid arteries treated by operative balloon dilatation. This technique is described in detail and provides three distinct advantages over conventional graduated intraluminal dilatation--atraumatic passage of the catheter through the affected vessel with fluoroscopic guidance; precise dilatation of the involved segment of the internal carotid artery; and the application of a radial force against the arterial wall rather than a longitudinal shear force, thereby making intimal damage less likely. There were no treatment complications in this group of patients. This technique of balloon angioplasty is compared with the results of treatment in eight patients with fibromuscular dysplasia of the internal carotid artery, who had percutaneous transfemoral angioplasty. Long-term follow-up revealed the resolution of symptoms in all patients.     

Dissektion der Nieren- und Beckenarterien beidseits bei fibromuskulärer Dysplasie vom intimalen Typ

A 36-year-old patient experienced spontaneous pain in the left side together with the symptoms of intermittent claudication. Investigation revealed left renal infarction as well as dissection of the pelvic arteries on both sides due to fibromuscular dysplasia of the intimal type. One year after implantation of an aorto-bifemoral bypass, the patient is symptom free. The patient's remaining renal artery continues to show no signs of pathological changes.