Fibromuscular dysplasia of renal arteries

This case reports a young child having uncontrolled hypertension, resulting from bilateral renal artery stenosis due to fibromuscular dysplasia presenting with abdominal pain, headache and visual disturbance. Diagnostic features and management is discussed.     

Fibromuscular dysplasia of the cervico-cephalic arteries

Six cases of fibromuscular dysplasia of the cervical and cephalic portions of the internal carotid arteries, including their intracranial branches are reported. It should perhaps be pointed out that one of the cases was from the Sudan. As far as we know, the condition has never before been reported in a male African. The condition was associated with an intracranial aneurysm in four of our cases. To our knowledge only three autopsied cases of fibromuscular dysplasia involving intracranial arteries are on record. In our six cases the diagnosis was based on angiographic evidence, and three of the cases, two with intracranial involvement, were verified post mortem.     

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 presenting with asymptomatic bilateral renal infarctions

Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic vascular disease. It is the second cause of renovascular hypertension after atherosclerosis. Although FMD usually has a good prognosis, renal infarctions and artery dissections have been described. We present the case of a 38-year-old woman with hypertension and asymptomatic bilateral renal infarctions. Bilateral FMD of segmental branches of the renal arteries was diagnosed by digital subtraction angiography after an exhaustive study. Previous intake of nonsteroidal anti-inflammatory drugs may also have played a significant role in the development of renal infarctions. To our knowledge, bilateral renal infarctions complicating FMD have been reported in only four previous cases; only in one of those cases, renal infarctions were asymptomatic.     

Fibromuscular dysplasia of the internal carotid arteries

Seven patients with fibromuscular dysplasia of the internal carotid arteries have been operated upon at Walter Reed Army Medical Center. One lesion was treated by graduated dilatation with Bake's dilators combined with resection, end-to-end anastomosis, and vein patching of a tortuous segment. All other lesions were treated by graduated dilatation with an arterial dilator-shunt. All of these patients are asymptomatic presently. One patient has been operated upon recently because of symptoms related to the previously unoperated side as well as mild symptoms related to the previous operation. Two other patients with arteriographic evidence of fibromuscular dysplasia are being followed clinically. One is asymptomatic and one has minimal symptoms. Both are being treated with acetylsalicylic acid in hopes of preventing microembolization from these lesions. Important technical considerations in treating this condition are meticulous dissection of the internal carotid artery as near to the base of the skull as possible, confining the arteriotomy to the region of the carotid bulb, and straightening the carotid artery while passing the dilator under direct vision. A technique for routine shunting in these patients now is available.     

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.     

Fibromuscular dysplasia of the extracranial carotid arteries. Case reports

Seven cases of fibromuscular dysplasia of the carotid artery are presented. Six of these patients presented with transient or fixed neurological deficit and 1 with intracranial haemorrhage. The uncertainty about the natural history of this condition is highlighted, and guidelines to the management of symptomatic patients as well as the more controversial incidentally diagnosed asymptomatic patient are outlined. A novel form of intra-operative use of a Grunzig balloon dilatation catheter in the management of fibromuscular dysplasia is presented.     

Fibromuscular dysplasia of the internal carotid arteries. Clinical experience and follow-up.

Fibromuscular dysplasia of the internal carotid arteries is a rare condition that may cause transient ischemic attacks, stroke and death. The preferred method of treatment of symptomatic lesions is graduated dilatation. Over a six year period, we have dilated 18 lesions. During a follow-up period ranging from 13 to 70 months (mean: 48.3 months), none of the patients operated on has developed a stroke or "hard" ocular/neurologic symptoms in the territory of the artery/arteries operated upon. Additionally, no patient has developed a stroke related to the contralateral asymptomatic, and consequently unoperated, carotid artery. Five patients with global symptoms have not had either carotid artery dilated and none has developed transient ischemic attacks or stroke during a follow-up period ranging from 22 to 100 months (mean: 42 months). These data demonstrate that a rational plan of management is dilatation of lesions associated with hard ocular/neurologic symptoms and nonoperative management of asymptomatic patients and patients with global symptoms.     

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.     

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.     

Fibromuscular dysplasia of the carotid artery

A series of 101 patients with carotid fibromuscular disease have been treated and followed for at least one year. Transient ischaemic attacks and amaurosis fugax were the most common presentation, while 22% of patients had had a completed stroke prior to surgery. Graduated dilatation was the most commonly used surgical method. In 150 operations, there were no deaths and three operative strokes. The operative repair has proven to be very durable and associated with a low incidence of neurologic events.     

A case of rete mirabile with congenital dysplasia of bilateral internal carotid arteries

We report a case of dysplasia of the congenital bilateral internal carotid arteries with the rete mirabile. The rete mirabile is not usually seen in the course of human growth, but it is a common finding in other mammals. Accordingly, some investigators have thought that the rete mirabile is "developmental shift". Our case has dysplasia of the bilateral internal carotid arteries (one is aplasia and the other is hypoplasia), but the patient had suffered from no ischemic symptom because her brain had been sufficiently fed by each of the rete mirabile. Angiographically, the frequency of the rete mirabile formation is about 1/10,000. There were 20 cases reported until 1997 (including our case). There were 5 cases (27.8%) with ischemic symptoms in spite of internal carotid artery dysplasia, 2 cases (11.1%) with intracerebral hemorrhage, 6 cases (33.3%) with subarachnoid hemorrhage (there were only two cases with aneurysm) and 5 cases without symptoms. We have tried to class the rete mirabile by angiographical findings. One is the M type finding resembling moyamoya vessels in stages 3 & 4 of moyamoya disease, and the other is the N type finding resembling a nidus of arteriovenous malformation. M type occurred in younger patients more often than N type, so M type may be the previous stage of N type.     

Fibromuscular dysplasia of the external iliac arteries: surgical treatment by graduated internal dilatation technique.

A case of fibromuscular dysplasia of the external iliac arteries treated by graduated internal dilatation is presented. At follow-up extending to 7 years, the external iliac arteries are patent with no evidence of recurrent disease by arteriography, and the patient remains asymptomatic. The recently reported successes with percutaneous balloon catheter angioplasty offers a nonsurgical alternative in the treatment of external iliac fibromuscular disease. A consideration of balloon catheter angioplasty in future similar cases is suggested. If balloon catheter angioplasty is unsuccessful, the option of operation remains available.     

Persistant hypokalemia due to bilateral fibromuscular dysplasia of the renal arteries

Bilateral fibromuscular dysplasia of the renal arteries was the underlying pathology in a 51-year-old woman with severe hypokalemia and borderline hypertension. Very high renin levels were found in both renal veins. The secondary hyperaldosteronism was most probably responsible for the persistant hypokalemia in this patient.