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 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 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 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.     

Vascular complications associated with spontaneous aortic dissection

Three hundred twenty-five cases of spontaneous aortic dissection seen at two institutions between 1965 and 1986 were reviewed to assess the incidence, morbid sequelae, and specific management of aortic branch compromise. Noncardiac vascular complications occurred in 33% of the study group, and in these patients the overall mortality rate (51%) was significantly (p less than 0.001) higher than in patients without (29%) such complications. Although aortic rupture was the strongest correlate of mortality (90%), death specifically related to vascular occlusion was common when such occlusion occurred in the carotid, mesenteric, and renal circulation. There was a strong correlation between stroke and carotid occlusion (22/26 cases), yet specific carotid revascularization was only used during the chronic phase of the disease. Similarly, peripheral operation was ineffective in reducing the mortality rate in the setting of mesenteric (87%) and renal (50%) ischemia. Fifteen patients required either fenestration or graft replacement of the abdominal aorta for acute obstruction, rupture, or chronic aneurysm development. Thirty-eight patients (12%) demonstrated some degree of lower extremity ischemia, and one third of these required a direct approach on the abdominal aorta or iliofemoral segments to restore circulation. Selected patients with acute aortic dissection may require peripheral vascular operation in accordance with a treatment strategy that directs initial attention to the immediate life-threatening complications.     

Migrating transient osteoporosis of the hip in a 30-year-old man

Transient osteoporosis of hip is a condition of unknown etiology, presenting as painful limping, and characterized by osteopenia of the involved joint without preexisting disease or immobilization. Most of the cases were reported in middle-aged men, and one-third of the cases develop in women in the third trimester of pregnancy. The hypothesis that this condition leads to avascular necrosis of the hip has been disproved by various reports and hence does not warrant any surgical interference. This is a self limiting condition, which needs regular follow-up. The authors report a case of migrating transient osteoporosis of the hip in a 30-year-old man successfully treated with antiresorptive treatment.     

Left atrial isolation for chronic atrial fibrillation associated with aortic arch dissection and aortic valve disease in a 70-year-old man

A 70-year-old male with aortic regurgitation, chronic atrial fibrillation (Af) and chronic aortic dissection of the aortic arch was treated simultaneously. We found a limited dissection in front of aortic arch during operation. We performed total arch replacement using a branched prosthesis and aortic valve replacement, in addition to a modified left atrial isolation for chronic Af under the aid of extracorporeal circulation, and selective cerebral perfusion. Left atrial isolation is a simpler procedure than maze procedure and left side maze procedure for eliminating Af. This simple procedure makes possible a simultaneous extended operation for complicated aortic and cardiac disease even in an elderly patient.     

Fibromuscular dysplasia in donor kidneys - experience with three cases

Abstract. In 1987, three patients received kidney grafts bearing medial fibroplasia at our hospital. Two of the grafts were from a cadaveric donor and one was from a living related donor kidney. The vascular affection was known before transplantation. Only one of the recipients developed stenosis and hypertension. With balloon catheter dilatation, the progressive stenosis of the renal graft artery could, however, be successfully corrected.     

Embryonal carcinoma of the testis associated with prostate cancer in a 72-year-old man

A 72-year-old Japanese man presented with a painless swollen left scrotal mass with elevated levels of serum alpha-fetoprotein and prostate specific antigen. The patient underwent high orchiectomy under diagnosis and a final pathological examination revealed embryonal carcinoma of the left testis. A systematic needle prostate biopsy under guidance of transrectal ultrasound revealed prostate cancer (Gleason score, 8) on the left lobe (T2aN0M0). Systemic chemotherapy was given for retroperitoneal lymph node metastasis of testicular cancer and hormonal therapy (LH-RH analog) was given for prostate cancer. The patient was well with no evidence of metastasis from the testicular cancer or prostate cancer and with no elevation of serum alpha-fetoprotein or prostate specific antigen 26 months after the orchiectomy.     

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.     

Fibromuscular dysplasia in donor kidneys — experience with three cases

In 1987, three patients received kidney grafts bearing medial fibroplasia at our hospital. Two of the grafts were from a cadaveric donor and one was from a living related donor kidney. The vascular affection was known before transplantation. Only one of the recipients developed stenosis and hypertension. With balloon catheter dilatation, the progressive stenosis of the renal graft artery could, however, be successfully corrected.