Eur J Vasc Surg 6, 471476 (1992)
Fibro-muscular Renal Artery Disease Treated by Extracorporeal Vascular Reconstruction and Renal Autotransplantation: Short- and Long-term Results I. B. Brekke, G. Sodal, A. Jakobsen, ~. Bentdal, P. Pfeffer, D. Albrechtsen and A. Flatmark Organ Transplantation Section, Department of Surgery B, The National Hospital, Oslo, Norway Over a 16-year period (1973-1989), 63 renal autotransplants were performed in 59 patients for fibro-muscular dysplasia (FMD) with renal artery stenoses (42 kidneys) or aneurysms (21 kidneys). About two-thirds of the autotransplants were performed before percutaneous transluminal angioplasty (PTA) was establishedfor clinical use. However, vascular disease at a site or type not suitablefor PTA was present in 57 (90%) of the kidneys. Hypertension was the leading symptom in 56 patients, including four in whom renal autotransplantation was performed as an emergency for acute renal artery occlusion or malignant hypertension. Blood pressure returned to normal or improved in 51 (91%) and remained unchanged in five patients (9%) following autotransplantation. Three patients with renal artery aneurysm in whom haematuria and loin pain were the indications for treatment, became asymptomatic following surgical intervention. Bilateral renal autotransplantation was performed synchronously in one and sequentially in three patients. There were no operative deaths, but two kidneys were lost postoperatively in two 2-year-old children owing to renal vascular thrombosis. In the follow-up period (mean 4.3 years), one additional kidney was lost at 3 months owing to progressive FMD. Blood pressure and renal function remained stable in all other patients. Based on the excellent results achieved in this series, it is concluded that extracorporeaI vascular repair and renal autotransplantation is a safe procedurefor the patient as well as the kidney affected by FMD. The procedure is advocated as an alternative to in situ reconstruction in patients with renal artery disease not accessible to PTA, such as aneurysms and complex branch renal artery stenoses. Key Words: Renal autotransplantation; Bench surgery; Fibro-muscular dysplasia; Renovascular hypertension; Renal artery stenosis; Renal artery aneurysm.
Introduction Fibro-muscular dysplasias (FMD) are a heterogeneous g r o u p of vascular diseases of u n k n o w n aetiology occurring p r e d o m i n a n t l y in females. 1 While atherosclerosis characteristically involves the proximal portion of the renal artery, FMD is usually located in the distal part and/or in branches of the renal artery, causing stenosis and/or a n e u r y s m formarion. 2 The characteristic angiographic "'string-ofb e a d s " appearance of the artery (Fig. 1) is caused b y the presence of multiple dilatations alternating with deposition of collagen in the media projecting into and thus n a r r o w i n g the lumen. Both stenotic a n d aneurysmal renal artery disease are often associated with h y p e r t e n s i o n which leads to diagnostic interPlease address all correspondence to: I. B. Brekke, Rikshospitalet, The National Hospital, Pilestredet 32, 0027 Oslo 1, Norway. 0950-821X/92/050471+06$08.00/0© 1992Grune & Stratton Ltd.
v e n t i o n a n d is the most c o m m o n indication for treatm e n t . The role of surgery in the m a n a g e m e n t of renal artery stenosis has changed in recent years owing to
Fig. 1. Bilateralfibro-muscular dysplasia of main renal arteries and branches with a typical "beaded" appearance caused by stenoses and aneurysms.
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the development of percutaneous transluminal angioplasty (PTA). The results of PTA in the treatment of FMD with stenosis of the main renal artery are admirable. 3-5 PTA is therefore now the treatment of choice in these cases. However, when aneurysms and branch renal artery stenoses preclude the use of PTA, 6'7 excellent results can be achieved by surgical vascular reconstruction. Our experience in treating renal artery aneurysms and stenosing FMD with extracorporeal vascular reconstruction and autotransplantation of 63 kidneys is presented.
Materials and Methods Patients
nosis was common to 40 patients with renal artery stenosis due to FMD (Table 2). Grades of hypertension are presented in Table 3. Five patients with grade 1 hypertension were children aged 2-9 years. Table 2. Manifestation and treatment of fibro-muscular dysplasias in 63 kidneys
Patients (n)
Kidneys (n)
40
42
38
38
Bilateral simultaneoustx
1
2
Bilateral Sequential tx
1
2
19 17
21 17
2
4
Stenosis Unilateral tx
Aneurysm Unilateral tx Bilateral sequentialtx
Fibro-muscular dysplasia was diagnosed in 59 (25%) of 237 patients who, between April 1973 and April 1989, underwent renal autotransplantation for renal artery disease. The patients were referred to our renal transplant unit from a population of 4 million. The diagnosis was based on characteristic angiography and was histologically confirmed postoperatively. Mean patient age was 35.7 years (range: 2-66 years) and 11 were younger than 16 years. Forty-one (69%) were female. Four patients had solitary kidneys, in three as a result of previous unilateral nephrectomy for renal carcinoma, hydronephrosis and renal artery occlusion respectively, while one patient had unilateral renal agenesis. Total kidney function, based on serum creatinine values, was moderately reduced in three patients with serum creatinine levels of 136204 p,mol1-1 (Table 1). Effective renal plasma flow
tx = renal autotransplantation. Table 3. Classification of blood pressure in 59 patients preoperatively and 1-10 (mean 4.3) years after renal autotransplantation for fibromuscular renal artery disease
Hypertensiongrades 0
1
2
3
4
Preoperative
3
5
16
15
20
Postoperative
40
13
5
1
0
The time-span between diagnosis of renovascular hypertension (RVH) and autotransplantation ranged from I month to 35 years. The stenosis was restricted to the main renal artery in six kidneys treated in the p r e - P T A era, while stenoses involving arterial Table 1. Kidney functin 1-10 (mean 4.3) years after renal auto- branches were present in a total of 36 kidneys. transplantation. Attempts at PTA had been made in four kidneys before autotransplantation. Autotransplantation was ERPF(ml min I) performed electively in 36 patients and as an emergSerum creatinine Autografted Contralateral ency procedure in four. In three of these, surgery was (~mol 1-1) kidney kidney performed as a means of kidney salvage after total renal artery occlusion, two occurring during an Preoperatively 94.7 (60-204) 160 (0-280) 234 (90-380) attempt at PTA (in a solitary kidney in one of these At follow-up 86.6 (55-133) 189 (30-340) 222 (80-350) patients). In the third patient a renal artery stenosis Values represent means (ranges in parentheses). ERPF= effective was primarily treated by PTA and later with an in situ renal plasma flow. bypass. The artery subsequently thrombosed, but the kidney was successfully revascularised by ex vivo (ERPF), measured by isotope renograms, ranged repair and autotransplantation. A 16-year-old boy from 0 to 280mlmin -1 in the kidneys subjected to underwent emergency bilateral simultaneous autoautotransplantation and from 90 to 380mlmin -1 in transplantation because of malignant hypertension with retinal haemorrhage and abductor nerve the contralateral kidneys (Table 1). A history of hypertension leading to the diag- paralysis. Sequential bilateral autotransplantation Eur J VascSurgVol 6, September1992
Renal Autotransplantation
was performed in one patient (Fig. 1) at an interval of 3 months. In 19 patients (21 kidneys), the main radiological and surgical findings were renal artery aneurysms. A single aneurysm located in the main renal artery was present in five kidneys, but aneurysms including the main artery and its branches were found in most cases. Hypertension led to the diagnosis in 16 of the patients and was the indication for surgical intervention. One of these patients had FMD in a solitary kidney. In three normotensive patients, symptoms of the loin-pain/haematuria syndrome, 8'9 led to angiography and the diagnosis. Sequential bilateral renal autotransplantation was performed in two of these patients who developed FMD with aneurysm formation in the contralateral kidney 3 and 7 years after the first autotransplant, respectively. In a total of 21 patients (36%) FMD was bilateral at the time of surgery, or developed in the contralateral kidney during follow-up. In most of these cases the contralateral FMD was without clinical significance.
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(a)
(c)
Methods
When performing unilateral autotransplantation, the affected kidney was usually removed through a flank incision. The kidney was then perfused with chilled (4°C) low molecular weight dextran or with EuroCollins solution. While submerged in a basin with ice-slush saline solution, the affected portion of the artery was excised and reconstructions were performed using a variety of techniques as previously described. 1°-13 In most cases arterial reconstruction was accomplished by variations of the "double barrel technique" [Fig. 2(a)] alone (n = 29), or in combination with interposition of an autologous vein or
(e)
"
~
Fig. 2. The various methods applied for renal artery reconstruction. artery graft (n = 19) as shown in Figure 2(b) and Figure 3. Excision of a short segment and approximation of the cut ends by direct end-to-end anastomosis [Fig. 2(c)] was performed in five kidneys. Simple excision of the affected portion of the main renal artery [Fig. 2(d)] was performed in eight, while grafts as
(b) (c) Fig. 3. Branchrenal artery stenosis and aneurysms (a) treated by ex vivo excisionof the affectedvessel portion (b) and revascularisation (c) with grafts from the unaffected main renal artery (ra) and saphenous vein (sv). Radiographic appearance after autotransplantation to the right hypogastric artery (d). Eur J Vasc Surg Vol 6, September1992
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(sudden unexplained death). This patient's preoperative grade 3 hypertension had been permanently reduced to grade i after the operation.
Postoperative complications (6)
(c)
Fig. 4. Autologoushypogastricartery used for renal artery reconstruction. (From:Brekke199010.) shown in Figure 2(e) and Figure 4 were used in two cases. After a mean cold ischaemia time of 2.7 h, the kidney was reimplanted to the ipsilateral iliac fossa with the renal vein anastomosed to the external iliac vein and the renal artery to the internal (hypogastric) (Fig. 3) or external iliac artery. Iliac endarterectomy was required in seven patients because of extensive atherosclerosis. Uretero-neocystostomy was accomplished by the method described by Paquin. 14 The mean hospital stay was 9.4 days. The patients were readmitted at 3 and 6 months and later at annual intervals for a maximum of 10 and a minimum of 1 year. The mean observation time was 4.3 years. Kidney function was assessed pre- and post-transplantation by creatinine measurements in 56 patients and isotope renograms in 35 patients. Blood pressures were registered and patients with a diastolic blood pressure of 90 mmHg or below without medication were classified as normotensive. Hypertensive patients were allocated to one of the following four hypertensive grades. Grade 1: moderate hypertension requiring moderate (one drug) anti-hypertensive treatment. Grade 2: diastolic blood pressure above 90mmHg despite two or more anti-hypertensive drugs. No organ manifestation of hypertension. Grade 3: diastolic blood pressure between 110 and 121 (or between 100 and 110 when combined with organ manifestations of hypertension) despite two or more anti-hypertensive drugs. Grade 4: diastolic blood pressure of 121 or more (or 110 or more in patients with organ manifestations) despite two or more anti-hypertensive drugs. The patient allocation to grade of hypertension is presented in Table 3.
Results
There were no perioperative deaths and, at present, all patients are alive except one 40-year-old man who died with a functioning autotransplant and a normal serum creatinine level 5 years after the operation Eur J VascSurg Vol 6, September1992
In two boys, both aged 2 years, the autotransplanted kidney was lost because of vascular thrombosis on postoperative days 9 and 16, respectively. Other complications were pneumonia in two patients, superficial w o u n d infection in three and parotitis in one patient.
Kidney function In addition to the two paediatric kidneys lost postoperatively, one kidney was lost 3 months after autotransplantation as a consequence of progressive FMD and vascular thrombosis. Angiographically diagnosed asymptomatic recurrence or progression of residual FMD was seen in four further autografted kidneys. This was successfully treated by PTA in three, and left without further treatment in one patient. Thus 60 of 63 autotransplanted kidneys (95%) retained long-term function. No deterioration of autograft function was observed during long-term follow up (Table 1). The occurrence of FMD in the contralateral kidney was observed in four kidneys of which two were successfully autotransplanted 3 and 7 years after the first operation.
Effect on hypertension The effect of vascular reconstruction and autotransplantation of the affected kidney on the blood pressure is shown in Table 3. In 56 initially hypertensive patients followed for 1-10 years (mean 4.3 years), the blood pressure was returned to normal or improved in 51 (91%). Thirty-seven were cured, i.e. became normotensive without anti-hypertensive drugs, while the blood pressure was considerably reduced in 14 patients. The hypertension was unaffected by the surgical intervention in five patients of whom two were children aged 2 years.
Discussion
In accordance with previous reports, 11"13'15-19 the
Renal Autotransplantation
results presented demonstrate that ex vivo vascular reconstruction with renal autotransplantation is a valid therapeutic approach in patients with renal artery FMD inaccessible to PTA. The procedure is associated with a high success rate and few serious complications. A return to normal or an improvement in blood pressure was achieved in 91% of patients, and stabilisation or improvement of function in 60 of 63 kidneys. Renovascular hypertension and threatened kidney function are the most common indications for intervention, as in 95% of patients in this series. However, resection of a renal artery aneurysm may occasionally be indicated for local symptoms as in three of our patients, or to obviate the risk of aneurysm rupture. 2° During the last decade, PTA has gradually become the treatment of choice for those stenotic portions restricted to the main renal artery, 4'21 and the majority of renal FMD cases are now treated by PTA. However, PTA has its limitations and is not without complications as previously reported. 3"s'22 This was demonstrated in two of the patients in this series, who experienced renal artery occlusion during PTA. To avoid serious complications it has been suggested that PTA is limited to non-orificial stenoses of the main renal artery in patients with two functioning kidneys. 22, 23 About two-thirds of the 59 patients included in the presented series were treated before PTA had been properly established for clinical application. However, the renal artery abnormalities were aneurysms and complex renal artery stenoses involving branch arteries, e.g. vascular abnormalities not easily accessible to PTA in 57 of the 63 autografted kidneys. Surgical revascularisation remains the treatment of choice for these selected categories of renal artery anomalies and for patients in w h o m renal artery PTA has been unsuccessful. Because simple, main artery FMDs are often treated in local hospitals while the more complex cases are referred to our renal transplant unit, the proportion of FMD cases unsuitable for PTA cannot be assessed from our series of patients. However, Novick 6' 7 considered as many as 30% of renal FMD cases unsuitable for PTA. Whether the reconstruction should be performed in situ or using the extracorporeal technique must be decided by the complexity of the anomaly and the preference of the surgeon. However, bench surgery and autotransplantation is an extremely safe approach which should be given priority in centres where renal transplantation is routinely performed.lB, lS, lS The development of microvascular techniques has improved the results of ex vivo surgery
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and extended its applicability to include most paediatric cases and the most complex renal artery abnormalities. 11"24 The advantage of performing extracorporeal repair of the kidney in such cases includes optimal exposure and a bloodless field with the kidney protected from warm ischaemia allowing generous time for accurate repair work.
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percutaneous transluminal dilatation of a renal artery stenosis.
Lancet 1978; 1: 801-802. 22 DEAN RH, CALLIS JT, SMITH BM, MEACHAM PW. Failed percutaneous transluminal renal angioplasty: experience with lesions requiring operative intervention. J Vasc Surg 1987; 6: 301-307. 23 SHr~Rm EG, WITZ M, MORAG B. Revascularisation for a poorly functioning solitary kidney. Eur J Vasc Surg 1991; 5: 421-423.
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24 MARTINEZ A, NOVlCK AC, CUNNINGHAM R, GOORMASTIC M. Improved results of vascular reconstruction in pediatric and young adult patients with renovascular hypertension. J UroI 1990; 144: 717-720.
Accepted 30 April 1992