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.     

Ex Vivo Repair and Renal Autotransplantation for Complex Renal Artery Aneurysms in a Solitary Kidney

Renal artery aneurysm (RAA) is a rare clinical entity with an incidence of 0.015-1%. Indications for interventional or surgical repair of RAAs are expanding aneurysms, diameter >2.5 cm, intractable renovascular hypertension, dissecting RAA, hematuria, and renal infarction after distal embolization. Interventional insertion of a stent graft as well as aortorenal bypass implantation are both low-risk procedures in simple aneurysms of the proximal renal artery. However, complex distal renal aneurysms involving several renal artery branches require not only an excellent result of vascular reconstruction, but also a surgical technique offering maximal protection for the kidney during the ischemic period. Here, we present a case of a solitary kidney with two consecutive RAAs of segmental renal artery branches (type 2 RAA). A surgical strategy including renal explantation, ex vivo renal preservation, ex vivo reconstruction of the renal artery, and renal heterotopic autotransplantation was successfully applied. The technique of ex vivo repair is a safe and effective surgical procedure in this clinical setting.     

离体肾动脉瘤切除和自体肾移植术治疗复杂肾动脉瘤

目的 探讨手助腹腔镜法活体取肾、离体肾动脉瘤切除、肾动脉重建和自体肾移植技术治疗复杂性肾动脉瘤的安全性和可行性.方法 2006年10月收治1例42岁复杂性肾动脉瘤男性患者.术前彩超、CT及DSA检查显示左肾动脉瘤3.4 cm×4.3 cm×4.5cm大小,瘤内有部分血栓形成,位于左肾动脉主干分叉部,累及5支分支动脉,邻近肾门.患者有高血压病史,药物控制不佳.术中采用手助腹腔镜法活体取肾成功后,立即对离体肾脏采用4℃肾脏保存液灌注,低温保护肾脏.体外进行肾动脉瘤切除:切取自体右髂内动脉体外行肾动脉重建,最后将肾脏异位移植至右侧髂窝.结果 患者手术成功,围手术期未出现并发症.术后.肾功能正常:彩超复查显示右侧髂窝移植肾动脉及其分支血流通畅无狭窄,肾静脉血流通畅,输尿管无狭窄.术后13个月随访,血压恢复正常,肾功能正常.结论 离体肾动脉瘤切除和自体.肾移植术治疗复杂性肾动脉瘤微创、安全、可行.     

Extracorporeal repair of renal artery branch lesions

Complex renal artery lesions which formerly would have been treated by nephrectomy may now be reconstructed by extracorporeal surgery and autotransplantation. Our experience in 15 patients (17 operations) with renal artery lesions extending into its branches or confined to them is described. Two patients underwent separate operations on both sides. The indications for surgery were severe renovascular hypertension (10 cases), renal artery aneurysm, (5 cases) and deteriorating renal function (2 cases). Overall clinical results after a mean follow up of 3 years were considered excellent in 13 procedures (11 patients). Improvement in 2 patients and failure in the other 2, who ultimately underwent nephrectomy. Discussion is focussed on results, vascular indications, (namely aneurysm, stenosis, dissecting aneurysm and trauma) and surgical techniques. Preservation of functioning renal tissue should be the ultimate goal of renovascular surgery where the ex vivo technique, when indicated, will achieve favorable results in most patients.     

Renal autotransplantation for the treatment of complex renovascular hypertension

OBJECTIVE: In individuals with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be unsuccessful or hazardous in these situations. We evaluated the outcomes of renal autotransplantation. PATIENTS AND METHODS: Between February 1989 and December 2005, we performed 5 renal autotransplantation procedures. The surgical strategy included renal explantation, ex vivo renal preservation, ex vivo reconstruction of the renal artery if necessary, and renal heterotopic autotransplantation. RESULTS: The study subjects (3 men and 2 women) exhibited one of the following indications for surgery: fibromuscular dysplasia (2 patients), Takayasu's arteritis (1), or atherosclerosis (2). All patients exhibited uncontrolled hypertension before renal autotransplantation. Renal arteries of patients were anastomosed either to the external or internal iliac arteries or to both when there were multiple renal arteries. The renal vein was anastomosed end-to-side to the external iliac vein, and ureteral reimplantation was not performed. Mean posttransplantation follow-up was 9.8 +/- 5.7 years (range, 1-16 years). Mortality and morbidity were not observed during the follow-up, and hypertension and renal function normalized or improved in all 5 patients. CONCLUSIONS: Renal autotransplantation is a highly effective procedure to treat complex renovascular lesions; ex vivo renal repair is a safe and effective surgical procedure in the clinical setting.     

Renal artery aneurysm. Treatment by ex-vivo reconstruction and autotransplantation: three cases and literature review

Renal artery aneurysms (RAAs) is a rare clinical entity: the prevalence is approximately 0.01%-1% in the general population. Complex aneurysms of the first ramification of the main renal artery often require nephrectomy for adequate excision. From December 2002 to July 2007, we treated 3 patients with complex RAA. All the patients were treated with ex vivo reconstruction of the renal artery followed by autotransplantation of the kidney into the ipsilateral iliac fossa. Observation is suggested for asymptomatic complex renal artery aneurysms measuring less than 2 cm in diameter. Surgical treatment by aneurysmectomy and reconstruction in vivo or ex vivo technique is indicated for RAA causing renovascular hypertension, dissection, embolization, local expansion and for those in women of childbearing age with a potential for pregnancy, or asymptomatic more than 2 cm in diameter. Ex vivo repair and renal autotransplantation is a safe and effective treatment for the management of complex renal artery aneurysms.     

Aneurysms of the renal artery: surgical management with special reference to extracorporeal surgery and autotransplantation

27 patients underwent surgical arterial reconstruction for renal artery aneurysms. Hypertension was present in 21 cases. The indication for surgery was the prevention of hemorrhagic rupture in association with hypertension. Extracorporeal surgery was performed 13 times for complex aneurysms involving several branches of the renal artery. Simple autotransplantation was performed 3 times for aneurysms located on the main renal artery. In situ surgery was performed on 11 patients (5 aneurysmectomy-arteriorrhaphies and 7 bypass operations). Results on high blood pressure showed that 10 of the 14 hypertensive patients operated by extracorporeal surgery and/or autotransplantation were cured. 1 delayed nephrectomy was performed in this group and 1 death was observed. 39 of the 46 peripheral anastomoses were patent postoperatively. All patients treated with aneurysmectomy-arteriorrhaphy were cured. In patients treated with bypass operations, 3 thromboses of the bypass and 2 failures on hypertension were observed. Aneurysmectomy and simple arterioplasty are preferred for simple renal artery aneurysms. For complex lesions involving several branches and of an intrarenal location, extracorporeal surgery and autotransplantation represent an effective treatment on hypertension and preservation of kidney function.     

Evolving strategies for the repair of complex renovascular lesions.

Fifty-five patients with 59 complex renovascular lesions required two or more branch artery anastomoses during aortorenal grafting. Forty-five reconstructions involving 112 branches were facilitated using hypothermic ex vivo perfusion preservation, whereas 14 involving 28 branches were repaired in situ. Ex vivo repair was used whenever the kidney was considered unreconstructable by in situ techniques. Fibromuscular dysplasia predominated and the branched internal iliac artery was used for renal artery substitution. There were no deaths and only one kidney (ex vivo) was lost. Branch vessel occlusion occurred in two of 140 anastomoses (1.4%). Ninety-eight per cent (51/52) of the heparinized patients had cure or improvement at mean follow-up of 5 years. No late graft dysfunction occurred in postoperative angiographic follow-up. The branched internal iliac artery is uniquely suited and remains the preference of the authors for the replacement of the diseased renal artery and its branches. The in situ repair is ideally suited for lesions limited to the renal artery bifurcation. Ex vivo repair is reserved for complex or reoperative distal arterial lesions. Unique characteristics in the group include: bilateral lesions (25%), solitary kidney (22%), reoperative lesions (16%), children (9%), and coexisting significant aortic disease (7%). In situ and ex vivo repair meet all the challenges of complex renovascular disease. The strategies outlined will achieve outstanding long-term total and segmental renal salvage in the treatment of hypertension or aneurysmal disease. When ex vivo repair is required, it can be accomplished with only one additional simple maneuver, the reanastomosis of the renal vein.     

Renal cell carcinoma with renal artery aneurysm treated by extracorporeal surgery and autotransplantation--a report of 2 cases]

We experienced two cases of renal cell carcinoma with renal artery aneurysm in the contralateral kidney. These cases have not been reported in the literatures which we have so far explored. In our cases, firstly tumor was resected, and then extracorporeal surgery and autotransplantation were performed on the renal artery aneurysm of the opposite side. Their postoperative courses were uneventful. Only a few instances of extracorporeal surgery and autotransplantation for renal artery aneurysm in a solitary kidney have been reported in the literature. However, no report of a case treated by extracorporeal surgery and autotransplantation for renal artery aneurysm in the contralateral kidney after surgical treatment of renal cell carcinoma has been made. Regarding the treatment of renal artery aneurysm, extracorporeal surgery and autotransplantation appeared satisfactory in our experience. It was a safe and assured surgical procedure. We recommend extracorporeal surgery and autotransplantation for renal artery aneurysm when management in situ is considered difficult.     

Ruptured renal artery aneurysm in a pregnant uninephric patient: successful ex vivo repair and autotransplantation

Renal artery aneurysm rupture during pregnancy is a rare event, with only 22 cases recorded in the literature. Maternal and fetal mortality rates have been high, and renal salvage with in situ repair of the renal artery has been documented in only three cases. We present here a case report of renal artery aneurysm rupture in a pregnant patient with congenital absence of the contralateral kidney, which was treated successfully with ex vivo renal artery reconstruction and autotransplantation. The literature on renal artery aneurysm rupture in pregnancy is reviewed and technical aspects of renal artery reconstruction and autotransplantation are presented.     

Ex vivo renal artery reconstructions: indications and techniques

Ex vivo renal artery surgery has been reported by several investigators and has extended the role of revascularization in the treatment of lesions previously managed by nephrectomy alone. Several techniques are available for use, and selection of the most appropriate method can be tailored to the specific anatomy being managed. Our total experience included 27 kidneys that have been managed by ex vivo renal artery reconstruction. Lesions managed in this manner include two kidneys with renal artery stenosis and renal tumors, one kidney with a congenital branch arteriovenous malformation, and 24 kidneys with branch occlusive or aneurysmal disease from fibromuscular dysplasia. Postoperative angiography was performed in 22 cases and defined successful revascularization without technical error in 20 cases. One operative death occurred as a result of myocardial infarction. One patient required reoperation to control bleeding, and two patients had temporary acute tubular necrosis during the postoperative period. Techniques employed included ex vivo repair with autotransplantation to the iliac system (six kidneys), mobilization and perfusion without transection of the renal vein (10 kidneys), and ex vivo perfusion and repair with replacement into the original renal fossa (11 kidneys). The authors believe this latter technique of reconstruction to be preferable to autotransplantation for the usual patient undergoing ex vivo repair of complex renovascular lesions.     

Autotransplantation of solitary kidney with renal artery aneurysm treated with laparoscopic nephrectomy and ex vivo repair: a case report

Introduction

Renal artery aneurysms (RAA) are extremely rare clinical entities with associated morbidities including hypertension and rupture. Although most RAA can be treated with in vivo repair or endovascular techniques, these may not be possible in patients with complex RAA beyond the renal artery bifurcation. We report a case of RAA in a patient with a solitary kidney that we treated successfully by extracorporeal repair and autotransplantation and the 2-years follow-up.

Case Report

A 64-year-old woman with a history of right nephrectomy for renal cell carcinoma presented with RAA found on routine computed tomography (CT). Preoperative workup demonstrated a 2.2 × 2.1 × 3-cm aneurysm in the distal left renal artery that was not amendable to in vivo or endovascular repair. The patient underwent a laparoscopic-assisted left nephrectomy, ex vivo renal artery aneurysm repair, and autotransplantation. She did well postoperatively and in clinic follow-up was found to have a creatinine of 1.2 mg/dL at the end of 2 years and stable blood pressure control.

Discussion

This patient with RAA in her solitary kidney was successfully treated with laparoscopic-assisted nephrectomy, ex vivo repair, and autotransplantation. Her creatinine was stable postoperatively despite absence of a second kidney.     

Ex vivo renal artery reconstruction.

Ex vivo renal artery reconstruction and autotransplantation is a relatively recent addition to the surgical armamentarium for renal vascular hypertension. Ten consecutive patients were considered for this surgical therapy and form the basis of this communication. The patients were treated by a combination of methods including bilateral ex vivo reconstruction, unilateral in situ and contralateral ex vivo reconstruction, and unilateral ex vivo reconstruction and contralateral nephrectomy. Replacement of the diseased segment of the renal artery in all ex vivo reconstruction consisted of arterial autografts including hypogastric artery, splenic artery, common iliac, and external iliac artery. In the ex vivo reconstruction, the ureter was either left intact or was transected and reconstructed by standard ureterovesicle implantation. After surgery all patients became normotensive without antihypertensive medication. Although this is a relatively small series, the uniform good results in these patients with extensive disease suggest that ex vivo renal artery reconstruction is a safe and effective method of treatment. Thus, it should be more widely applicable, expecially in those patients with renal vascular disease who were previously thought to be inoperable or eligible for nephrectomy only.     

Renal artery aneurysm. Long-term relief of renovascular hypertension by in situ operative correction

Renal artery aneurysm is an uncommon cause of renovascular hypertension. Nephrectomy or, more recently ex vivo arterial reconstruction have been recommended as the treatment of choice. In contrast, we advocate in situ repair of the aneurysm and any associated lesions. Twenty-five hypertensive patients with 30 renal artery aneurysms were treated by tangential aneurysmectomy with primary arteriorrhaphy, saphenous vein patch angioplasty, or bypass graft. Nephrectomy was performed in two patients, one for a ruptured aneurysm. There was no operative mortality. Follow-up was obtained on all patients six months to 19 years after operation. Hypertension was relieved immediately and in the long-term in the majority of survivors. We believe these results indicate that despite the presence of severe renovascular disease, the affected kidney can be preserved and hypertension successfully relieved by a direct surgical approach without recourse to either nephrectomy or ex vivo reconstruction.     

Renal artery aneurysm: ex vivo repair and autotransplantation: case report and review of the literature

The incidence of renal artery aneurysm is unknown, its natural history is unclear and unpredictable, and the clinical symptoms are of little or no value in diagnosis. The risk of rupture is high in pregnant women, as in splenic artery aneurysms and in aneurysms greater than 2 cm in size. Digital subtraction angiography is the best diagnostic test. When an aneurysm is identified, surgery is the best treatment option to avoid hypertension or rupture of the aneurysm. Because of advances in organ preservation, nephrectomy, ex vivo repair, and autotransplantation is a safe and successful procedure. We report the case of a 2-cm-wide neck aneurysm that was treated by nephrectomy, ex vivo repair, and auto-transplantation.     

Bilateral ex vivo repair and kidney autotransplantation for complex renal artery aneurysms: A case report and literature review

We report the surgical management of a bilateral renal artery aneurysm diagnosed in a 41‐year‐old patient with a history of recurrent abdominal pain. The preoperative contrast‐enhanced computed tomography showed a complex saccular aneurysm on both renal arteries within the renal hilum. The characteristics of aneurysms precluded endovascular procedures, and a double‐step bilateral ex vivo reconstruction with kidney autotransplantation was planned. The intra‐ and postoperative period was uneventful. Imaging and laboratory examinations show preservation of renal function, and patient is symptom‐free at 10‐month follow up.     

Intraoperative evaluation of blood perfusion by laser-assisted indocyanine green angiography after ex vivo vascular reconstruction of intrahilar renal artery aneurysm

The surgical reconstruction of intrahilar renal artery aneurysms (RAAs) is a difficult surgery because of complex anatomy. We present a case of right intrahilar RAA diagnosed in a 67-year-old man. We performed ex vivo reconstruction using an organ preservation solution to prevent postoperative renal failure. We assessed graft patency and blood perfusion was assessed by laser-assisted indocyanine green angiography using the SPY system after autotransplantation. Postoperative renal insufficiency was not observed. The results demonstrate that ex vivo reconstruction of intrahilar RAAs using an organ preservation solution, and graft patency and blood perfusion evaluation using the SPY system are effective methods for preserving renal function.     

Ruptured renal artery aneurysm during pregnancy: successful ex situ repair and autotransplantation

Rupture of a renal artery aneurysm during pregnancy is a rare event, with a high mortality rate for both mother and fetus. Until now, 25 cases have been reported in the English medical literature. Renal salvage with in situ repair of the renal artery has been documented in only four cases, and successful ex situ repair and autotransplantation in only one case. We report the case of a mother and fetus who both survived acute rupture of a renal artery aneurysm after treatment with ex situ repair and autotransplantation.     

Renal artery stenosis and aneurysms associated with neurofibromatosis

Renal artery lesions associated with neurofibromatosis may involve stenosis and aneurysm formation at all levels of the renal artery to the intraparenchymal branches, and usually are associated with hypertension. A 13-year-old boy with type I neurofibromatosis and severe hypertension presented with multiple aneurysms and multiple stenotic lesions in the renal artery and segmental arteries. The patient underwent ex-vivo renal artery repair with autologous hypogastric artery and autotransplantation to the iliac fossa and was clinically improved. The characteristic histologic findings are presented. A review of the recent literature comparing different treatment modalities for renovascular hypertension in children with neurofibromatosis suggests that surgery remains the best treatment alternative.     

The role of renal autotransplantation in pediatric and young adult patients with renal artery disease

From 1977 to 1984, renal autotransplantation was attempted in 16 pediatric and young adult patients with renal artery disease, ranging in age from 10 months to 21 years. Renal revascularization was indicated as treatment for severe hypertension in 15 patients and to prevent rupture of an arterial aneurysm in one patient. The reasons for undertaking renal autotransplantation were branch renal artery disease requiring extracorporeal revascularization (n = 14), abdominal aortic hypoplasia (n = 1), and renal artery disease in a small infant (n = 1). Renal revascularization was successfully accomplished in 14 of 16 patients, including one patient who underwent staged bilateral extracorporeal repairs. Obliteration of the inferior vena cava and iliac veins precluded autotransplantation in one patient and a nephrectomy was done. In one patient extracorporeal ligation of an inaccessible renal arterial branch was accomplished with autotransplantation. Currently all 16 patients are normotensive with excellent renal function. Extracorporeal surgery and autotransplantation have been important additions to the surgical armamentarium for renal artery disease.