Calcified renal artery aneurism and high blood pressure. A case report and review of the literature

Renal artery aneurysm is a rare disease in children and usually is due to fibromuscular dysplasia. Clinical symptoms are frequently high blood pressure, abdominal pain, and hematuria. Diagnosis is carried out by means of angiography. We report the case of a 13-year-old male patient who had renovascular hypertension due to calcification and aneurysm of fibromuscular dysplasia-associated renal artery. We carried out total nephrectomy to resolve high blood pressure. We suggested that presence of discreet calcification in region of renal artery in a boy with renovascular high blood pressure should guide us toward diagnosis of fibromuscular dysplasia-related renal artery aneurysm.     

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.     

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.     

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.     

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.     

Technique for extracorporeal resection of dissecting renal arterial aneurysm with renal autotransplantation.

A progressively enlarging dissecting aneurysm of the right renal artery secondary to fibromuscular dysplasia presents a difficult surgical problem. We present a technique of extracorporeal approach for correction of such aneurysm in a patient with dissecting aneurysm and hypertension. This technique utilizes a Teflon patch graft to facilitate the arterial anastomosis of the renal autotransplantation.     

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.     

Renal infarction secondary to fibrous dysplasia and aneurysm formation of renal artery

We report a case of a previously healthy forty-year-old man with bilateral renal artery stenosis secondary to bilateral medial fibrous dysplasia. He was additionally found to have a dissecting aneurysm of the left upper renal artery branch with resultant infarction of the upper and middle pole renal segments. We believe renal infarction secondary to medial fibrous dysplasia with occlusion of the renal artery associated with a dissecting aneurysm has not been reported, and we report the first such case. A multimodality treatment approach was utilized.     

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.     

Ex vivo repair of a large renal artery aneurysm and associated arteriovenous fistula

The development of a renal artery to vein arteriovenous fistula due to a large extraparenchymal renal artery aneurysm is uncommon. Previous surgical experience with this entity is limited. Based on the existing surgical literature, nephrectomy has been the treatment of choice. We report preservation of the kidney by surgical correction of this entity using ex vivo "bench" repair in a middle-aged female with fibromuscular dysplasia of the renal artery. The technique, results, and recommendations for surgical management are discussed.     

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.     

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.     

Fibrodysplasia with dissecting aneurysm of the hepatic artery.

The presentation and management of a patient with a symptomatic hepatic artery aneurysm is detailed. The aneurysm occurred secondary to fibromuscular hyperplasia of the hepatic artery and was associated with a limited area of dissection. This patient represents the second reported case of this type of aneurysm of the hepatic artery,     

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 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.     

External carotid artery aneurysm developing after embolization of a ruptured posterior inferior cerebellar artery aneurysm in a patient with cervicocephalic fibromuscular dysplasia--case report

A 30-year-old man presented with an aneurysm of the left posterior inferior cerebellar artery manifesting as subarachnoid hemorrhage and cerebellar infarction. Angiography demonstrated string-of-beads sign typical of fibromuscular dysplasia (FMD) in the extracranial carotid and vertebral arteries. The aneurysm and the parent artery were successfully embolized with Guglielmi detachable coils. Severe vasospasm developed 1 week after admission, and was treated several times by selective injection of vasodilator. A new aneurysm of the left external carotid artery became evident 1 month later, whereas only slight dilation had previously been apparent. This angiographic sequence demonstrated a new arterial dissection. Despite the possibility of damage to the artery during multiple catheterizations, arterial wall changes caused by FMD appear to have been primarily responsible. This case emphasizes the need for particular care in performing vascular interventional procedures in the presence of FMD.     

Fibromuscular dysplasia of the posterior auricular artery: an unusual aneurysmal lesion

A 67-year-old patient was admitted to hospital with a pulsatile mass behind his left ear. The clinical diagnosis was a posterior auricular artery aneurysm, and this was confirmed on Digital Subtraction Angiography. The aneurysm was excised and sent for pathology. The findings were felt to be consistent with fibromuscular dysplasia. This appears to be only the second reported case in the literature of a posterior auricular artery aneurysm.     

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.     

Radial artery as conduit for distal renal artery reconstruction

Reconstruction of the renal artery with both saphenous vein and prosthetic material as bypass graft is durable in atherosclerotic disease. Extensive experience with saphenous vein grafts in pediatric patients and patients without atherosclerosis reveals a disturbing incidence of vein graft aneurysm degeneration. Distal renal artery reconstruction involving small branch vessels is generally not amenable to prosthetic reconstruction. We report a new approach to distal renal artery bypass grafting to avert these limitations. CASE: A 43-year-old man with previously normal blood pressure had malignant hypertension, which proved difficult to control despite use of a beta-blocker and an angiotensin II inhibitor. At renal angiography a fusiform aneurysm was revealed in a posterior branch of the right renal artery. The renal artery aneurysm was resected, and the left radial artery was harvested and used as a sequential aortorenal bypass graft to the two branch renal arteries. The postoperative course was uneventful, and the patient now has normal blood pressure with a calcium channel blocker for maintenance of the radial artery graft. Pathologic analysis revealed a pseudoaneurysm with dissection between the media and external lamella, consistent with fibromuscular dysplasia. CONCLUSION: Autologous artery is the preferred conduit for renal reconstruction in the pediatric population. On the basis of cardiac surgery experience, we used the radial artery and found it to be a technically satisfactory conduit for distal renal reconstruction in a patient without atherosclerosis.     

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.