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.     

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 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 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.     

Elastic tissue dysplasia of the internal carotid artery.

Elongation with kinking of the internal carotid artery is not an uncommon finding. Cerebral vascular insufficiency has been reported secondary to this abnormality. Although arteriosclerosis is a frequent cause in adults, the etiology in children has never been explained. Three children with significant kinking of the internal carotid artery are reported here. Each underwent surgical correction by excision and reconstruction. Histological review showed the elastic tissue to be disrupted, diminished in amount, and unevenly distributed. Retrospective examination of this elastic tissue dysplasia showed it to be localized, there being normal morphology both proximally and distal to the involved area. There was recurrence of elongation and kinking in one patient due to incomplete excision. Correction was accomplished after re-excision of the involved tissue with a vein graft interposition. From this study, it can be concluded that elongation and kinking of the internal carotid artery in children is secondary to elastic tissue dysplasia. It is imperative that the entire involved area be removed in order to prevent recurrence. The excised specimen should be studied histologically with elastic tissue stains. Such stains cannot be performed as frozen section. Should permanent studies show the margins of resection not to be beyond the dysplastic tissue, careful followup of these children is essential to recognize recurrence.     

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.     

Aneurysm of the extracranial internal carotid artery caused by fibromuscular dysplasia

A case of aneurysm of the extracranial internal carotid artery (ICA) caused by fibromuscular dysplasia (FMD) is reported. The patient also had an aneurysm of the contralateral intracranial ICA, but the renal arteries were normal. A review of the literature shows extracranial ICA aneurysms to be uncommon, with only 3% caused by FMD. The surgical management of such difficult lesions at the base of the skull is discussed.     

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.     

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.