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.     

Asymptomatic renal infarction, due to fibromuscular dysplasia, in a young woman with 11 years of follow-up

We report a 27-year-old woman with renovascular hypertension, renal infarction, and hepatic artery aneurysm due to fibromuscular dysplasia. The patient was first noted to have renal artery aneurysm and hepatic artery aneurysm at the age of 17. The renal infarction was asymptomatic and was incidentally detected by magnetic resonance imaging (MRI) examination. Because of the rather peripheral location of the aneurysms, percutaneous transluminal renal artery angioplasty was considered inappropriate. This case suggests the need for long-term and periodical follow-up of patients with fibromuscular dysplasia.     

Natural history of renal artery aneurysm elucidated by repeated angiography and pathoanatomical studies

The findings from repeated angiographies in 16 female and 5 male patients with altogether 34 renal artery aneurysms were studied. The mean interval between the first and last angiography was 35 months. Seven patients had multiple aneurysms. Two to four angiographies were performed in each patient. They showed no change in 28 aneurysms and slight or minimal enlargement, thrombosis or calcification in the other 6. The clinical course was uneventful except for severe hypertension in 3 patients. No rupture occurred. Eight patients, of whom 5 had solitary, saccular aneurysms, were operated upon. Pathoanatomically, fibromuscular dysplasia or secondarily changed fibromuscular dysplasia was found in 7 of them. Four died of unrelated disease having been followed up for 55-204 months (mean 102 months). Nine were alive and symptomless at the end of follow-up 11-195 months (mean 97 months) after the first angiography. The study supports the view that the risk of rupture of a renal artery aneurysm is very small, and indicates that fibromuscular dysplasia is common even when the angiography shows solitary, saccular aneurysm only.     

Multiple basilar artery trunk aneurysms associated with fibromuscular dysplasia

Summary An association between fibromuscular dysplasia (FMD) and intracranial aneurysms has been proposed but the occurrence of multiple aneurysms on the trunk of basilar artery (BA) associated with FMD has not been previously reported.     

Surgical approach to the high subcranial internal carotid artery

Two cases are presented to describe surgical approaches to the high cervical internal carotid artery permitting surgical treatment of conditions such as aneurysms of the high carotid artery, fibromuscular dysplasia, and arteriosclerosis by endarterectomy or resection of a segment with the interposition of a graft.     

Wilson Disease With Giant Splenic Artery Aneurysms Caused by Fibromuscular Dysplasia During Living Donor Liver Transplantation: A Case Report

Liver cirrhosis can cause splenic artery aneurysms (SAA) that pose a threat to patients undergoing liver transplantation. However, liver transplantation with multiple visceral artery aneurysms including giant SAA caused by arterial fragility has never been reported. We describe a 36-year-old man with decompensated liver cirrhosis due to Wilson disease that was complicated by giant SAA and multiple aneurysms in the bilateral renal arteries caused by fibromuscular dysplasia (FMD). The maximal diameter of the triple snowball-shaped SAA was 11 cm. We planned a 2-stage strategy consisting of a splenectomy with distal pancreatectomy to treat the SAA and subsequent living donor liver transplantation (LDLT) to address the liver cirrhosis. This strategy was selected to prevent fatal postoperative infectious complications caused by the potential development of pancreatic fistula during simultaneous procedures and to histopathologically diagnose the arterial lesion before LDLT to promote safe hepatic artery reconstruction. However, a postoperative pancreatic fistula did not develop after a splenectomy with distal pancreatectomy, and the pathologic findings of the artery indicated FMD. The patient underwent ABO-identical LDLT with a right lobe graft donated by his brother. Other than postoperative rupture of the aneurysm in the left renal artery requiring emergency interventional radiology, the patient has remained free of any other arterial complications and continues to do well at 2 years after LDLT.     

Aneurysms of the visceral arteries

Visceral artery aneurysms are uncommon. During the period 1975-88, 32 patients were treated at the Royal Melbourne Hospital for true and false visceral artery aneurysms. There were 18 males and 14 females, with an age range of 12-86 years. Of the 32 patients, 26 were symptomatic and six were asymptomatic. True aneurysms were found in 20 patients and false aneurysms in 10. A further two were dissecting aneurysms. Of the arteries involved, 17 were renal, six were hepatic, five were splenic, one was superior mesenteric, one was left colic and there were two patients with aneurysms at multiple sites. Aetiological factors included atherosclerosis, fibromuscular dysplasia, pancreatitis, and trauma. Only one patient presented in pregnancy. Rupture occurred in 12 patients and two died as a result of this complication. All the true hepatic artery aneurysms presented in this way. Pre-operative investigations included plain radiology, computerized tomography with contrast, nuclear scanning and selective angiography. Operative treatment was required in 22 cases, 12 as an emergency and 10 as an elective procedure. Surgical options included aneurysm excision with or without arterial reconstruction, aneurysmorrhaphy with flap arterioplasty, or ligation of the aneurysm. Embolization was successfully employed in two patients and eight were merely observed without complications. Surgical therapy is recommended for any patient with symptoms, for any woman of child-bearing age and for all hepatic artery aneurysms.     

Hepatic and splenic artery aneurysms

Accounting for 80% of all visceral artery aneurysms, splenic and hepatic artery lesions are rare but potentially life threatening. Although their natural history has not been well-defined, the high mortality associated with emergent repair suggests an aggressive approach is indicated. While repair is clearly mandated in patients with a symptomatic aneurysm or contained rupture, the following asymptomatic lesions also warrant intervention: (1) splenic artery aneurysms in patients with the potential to become pregnant or requiring liver transplantation, (2) hepatic aneurysms in patients with polyarteritis nodosa or fibromuscular dysplasia, (3) splenic or hepatic artery aneurysms greater than 2.0 cm in diameter, and (4) splenic or hepatic pseudoaneurysms. Although open surgical interventions have traditionally been the mainstay of therapy, endovascular techniques have increasingly been applied to this problem. Dictated predominately by the need to maintain distal end-organ perfusion, potential therapies include open surgical ligation, autogenous reconstruction, endovascular coil embolization, or percutaneous endograft placement. While offering alternative approaches, endovascular techniques have not dramatically altered the risk-to-benefit ratio in asymptomatic lesions, and both open and percutaneous approaches present viable options for elective repair. Although explored in only a limited number of patients, the application of endovascular techniques to the symptomatic patient offers the greatest potential for significant improvements in morbidity and mortality.     

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.     

Vascular abnormalities in the neck associated with intracranial aneurysms

In 102 cases of ruptured intracranial aneurysms, the cervical as well as the cranial areas were explored by angiography. Loops in the course of the cervical vessels were present in 28 patients; features of fibromuscular dysplasia were present in 31 patients; and in 50 patients, no abnormalities were observed. In 7 patients, both cervical anomalies were present. Loops were associated with single aneurysms (95%), located primarily on the anterior communicating artery (76%), predominantly in men (M/F = 1.6). Aneurysms with fibromuscular dysplasia affected women more than men (F/M = 7), were frequently multiple (58%), and were located mainly on the intracranial part of the internal carotid and vertebral arteries (51%). These data suggest new concepts of aneurysm formation from inherited diseases and should permit the detection of some aneurysms before rupture.     

Aneurysms of the visceral arteries: report of 5 cases

Visceral artery aneurysms are uncommon and usually result from atherosclerosis, periarteritis nodosa and fibromuscular dysplasia. Hepatic artery aneurysms were detected in two patient, splenic artery aneurysms in three. In four patients rupture occurred. In the two patients with hepatic artery aneurysm hemobilia from arterial rupture into the common bile duct and intraperitoneal bleeding in lesser sac was assessed. Ruptured aneurysms of the splenic artery with free intraperitoneal bleeding occurred in two patients, one patient had an asymptomatic splenic artery aneurysm. In four patients the diagnosis was made by contrast-TC and/or celiac and mesenteric angiography. In four patients excision of the aneurysm was successfully performed. One patient with ruptured hepatic artery aneurysm and in which resection and revascularization was made died.     

Thrombosed giant coronary artery aneurysm presenting as an intracardiac mass

Giant coronary artery aneurysms are rare in adults and are usually found in association with Kawasaki's disease arising in childhood. We report a case of a thrombosed giant right coronary artery aneurysm presenting as an intracardiac mass detected after inferior wall myocardial infarction. Histologic analysis indicated that fibromuscular dysplasia was the underlying cause of the aneurysm.     

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.     

Some popliteal aneurysms are congenital

Although the majority of popliteal aneurysms are of atherosclerotic origin and are discovered in patients aged more than 50, the aetiology of a small minority may be attributed to arterial trauma, septic disruption, Behçet's disease, a medial fibromuscular dysplasia, or popliteal entrapment. The aim of this study was to propose a possible congenital origin for popliteal aneurysms without evidence of any other aetiology. A retrospective review of five younger patients (aged 20-45 years) with seven non-atherosclerotic popliteal aneurysms was performed. In patient nos 1 and 2, histological examination showed that the arterial wall had been replaced by an abundant collagenic tissue. After replacement by a saphenous bypass graft, these patients did well with a follow-up ranging from 1 to 15 years. Patient no. 3 was not operated on because of a symptomless occlusion of the tibial and peroneal arteries, and remains well 10 years later. Patients nos 4 and 5 were treated with a saphenous bypass graft. The last patient also had associated bilateral congenital anomalies of the division of the popliteal arteries. In the absence of any evidence of any classical aetiology, particularly fibromuscular dysplasia and popliteal entrapment, a congenital aetiology is proposed in aneurysms diagnosed in younger patients. The complex composition of the popliteal artery, being composed by an association of three original segments, may induce a fragility of the arterial wall that may be responsible for aneurysmal deterioration as well as abnormal branching or popliteal artery entrapment. These aneurysms are associated with the same risk of thrombosis as atherosclerotic aneurysms and therefore, they should be subjected to the same therapeutic considerations.     

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.     

Aneurysm of internal carotid artery. Clinical symptoms, surgical treatment, results

Aneurysms of the extracranial portion of the internal carotid artery is rare pathology - only 2.2% of all surgical procedures on this portion of the internal carotid artery are caused by this disease. Ten-year experience in surgical treatment of these aneurysms is presented in article. Symptoms and etiology of this disease are considered. It is demonstrated that fibromuscular dysplasia is one of the main causes of these aneurysms. Strategy and policy of surgical treatment, clinical cases are analyzed. Resection of the aneurysm with angioplasty or grafting is optimal. End-to-end anastomosis is possible in patients with kinking. Results of surgical treatment are positive, there is no hospital lethality.     

Multiple aneurysms of the splenic artery caused by fibromuscular dysplasia

Splenic artery aneurysms (SAAs) are relatively rare. Moreover, there has been only one previous report of fibromuscular dysplasia (FMD) affecting the splenic artery alone. We describe a 64-year-old man with long, segmental, large, and multiple SAAs in whom the splenic artery branched from the aorta. The patient underwent endoaneurysmorrhaphy and splenectomy, with ligation in the proximal segment of the splenic artery. Histopathological analyses of resected specimens showed characteristics compatible with FMD. To our knowledge, long, segmental, large, and multiple SAAs caused by FMD have not previously been reported.     

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.     

Renal Artery Aneurysm Secondary to Fibromuscular Dysplasia in a Young Patient

An 8-year-old male was found on routine physical examination to have a blood pressure of 220/110. Renal angiography demonstrated bilateral renal artery stenosis and an aneurysm of the distal left renal artery with branch involvement. At operation, the left renal artery stenosis and aneurysm was repaired by ex vivo arterial reconstruction and autotransplantation of the kidney. Pathologic evaluation of the resected aneurysm confirmed the diagnosis of fibromuscular dysplasia. Fibromuscular dysplasia is the most common cause of renal artery stenosis in children over 1 year of age and can in rare cases be associated with the development of renal artery aneurysms. In complex cases of renal artery stenosis with involvement of renal artery branches, ex vivo repair and orthotopic autotransplantation is an excellent approach for surgical management.Presented at the Twenty-second Annual Meeting of the Southern California Vascular Surgery Society, La Jolla, CA, April 30-May 2, 2004.     

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.