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The role of renal autotransplantation in pediatric and young adult patients with renal artery disease
The role of renal autotransplantation in pediatric and young adult patients with renal artery disease M a r k L . Jordan, M.D., Andrew C. Novick, M.D., and R o b e r t L. C t m n i n g h a m , M.D.,
Cleveland, Ohio 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 reqtfiring extracorporeal revascularization (n = 14), abdominal aortic hypoplasia (n = 1), and renal artery disease in a small infant (n = 1). Renal revascxflarization 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 fimction. Extracorporeal surgery and autotransplantation have been important additions to the surgical armamentarium for renal artery disease. (J VAse SURG 1985; 2:385-92.) The exact incidence of hypertension in children is not known but is estimated at between 1.5% to 2.5%. Children and adults differ significantly in the spectrum of causative factors that may lead to an elevated blood pressure with a secondary etiology being much more common in children. Among such pediatric patients renal artery disease is secondary in frequency only to coarctation of the thoracic aorta as a cause of surgically correctable hypertension. Although renal revascularization has been estab!'~hed for over 20 years as an effective form of treatment for adult renovascular hypertension, the initial results in children were less satisfactory. 1,2 However, recent reports during the past decade have indicated that successful renal vascular reconstruction is now possible in most pediatric patients. 3-1°This evolution has been due both to the incorporation of microvascular techniques into the armamentarium of the renovascular surgeon as well as an enhanced appreciation of those reconstructive operations that are more satisfactory in children. We report herein our experience with extracorporeal renal revascularizaFrom the Departments of Urology (Drs. Jordan and Novick) and Nephrology (Dr. Cunningham),The Cleveland Clinic Foundation. Reprint requests: Andrew C. Novick, M.D., Head, Section of RenalTransplantation,Deparmlentof Urology,The Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland,OH 44106.
tion and autotransplantation in pediatric and young adult patients. This is a relatively new approach that has helped to improve the outlook for patients in this age group with renal artery disease. MATERIAL AND METHODS From January 1977 to March 1984, renal autotransplantation with or without extracorporeal revascularization was attempted in 16 pediatric and young adult patients with renal artery disease. This series includes seven male and nine female patients, ranging in age from 10 months to 21 years. Renovascular pathologic conditions comprised intimal fibroplasia in seven patients, perimedial fibroplasia in five patients, Takayasu's arteritis in two patients, and an arterial aneurysm in two patients. Renal artery disease was present unilaterally in eight patients and bilaterally in eight patients. In six patients extrarenal vascular disease was also present as manifested by stenosis of both the carotid and mesenteric vessels in two patients, stenosis of the mesenteric vessels alone in one patient, a hypoplastic abdominal aorta in one patient, an aneurysm of the common iliac artery in one patient, and multiple peripheral aneurysms in one patient. Fifteen patients were admitted for evaluation of severe hypertension. All patients were studied preoperatively with the usual diagnostic procedures in385
386
Journal of VASCULAR SURGERY
Jordan, Novick, and Cunningham
T a b l e I. Clinical characteristics a n d surgical t r e a t m e n t in sixteen patients w i t h renal vascular disease
Patient 1 2 3
Bilateral Pathologic renovascular Extrarenalvascular diagnosis disease disease
Age (yr)
Sex
10
M
TA
Yes
F M
IF PF
No No
---
10 mo 18
Aortic hypoplasia
4
16
F
PF
No
--
5
16
F
RAA
No
--
6
18
F
PF
Yes
--
7
20
F
IF
Yes
Carotid and mesenteric
8
9
F
IF
No
--
9
12
F
IF
No
--
10
15
M
TA
Yes
Common iliac artery
11
20
M
IF
No
--
12
9
M
RAA
Yes
13
12
F
PF
Yes
Multiple peripheral aneurysms Mesenteric
14
6
M
IF
No
--
15
21
M
IF
Yes
Carotid and mesenteric
16
17
F
PF
Yes
Operations
Current blood pressure
Left aortorenal bypass Normal Right renal autotransplant Left nephrectomy Normal Right ex vivo repair with hypogastric Normal artery graft, autotransplant Left ex vivo repair with hypogastric Normal artery graft, autotransplant Left ex vivo aneurysmectomy and Normal with primare closure, autotransplant medication Right aortorenal bypass Normal Left ex vivo repair with hypogastric artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo ligation of renal artery • Normal branch, autotransplant Right ex vivo repair with branched Normal saphenous vein graft, autotransplant Right in situ renal aneurysmectomy Normal with Left ex vivo repair with branched medication inferior epigastric artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo aneurysmectomy and Normal primary closure~ autotransplant Right aortorenal bypass Normal with Left ex vivo repair with hypogastric medication artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right aortorenal bypass Normal Left ex vivo repair with hypogastric artery graft, autotransplant Left ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo repair with hypogastric artery graft, autotransplant
IF = intimal fibroplasia; PF = perimedial fibroplasia; TA = Takayasu's arteritis; RAA = renal artery aneurysm.
cluding intravenous p y e l o g r a p h y , i s o t o p e renography, a o r t o g r a p h y a n d selective renal arteriography, renal f u n c t i o n parameters, a n d peripheral a n d differential renal vein renin determinations. A n g i o g r a p h i c studies d e m o n s t r a t e d vascular disease confined to the m a i n renal artery in t w o patients, whereas involvem e n t o f o n e o r m o r e renal arterial branches was observed in 14 patients. T h e p r e o p e r a t i v e s e r u m creatinine level was < 2 . 0 m g / d l in all cases. Renal revascularization was i n d i c a t e d as t r e a t m e n t for severe h y p e r t e n s i o n in 15 patients and to prevent r u p t u r e o f a large noncalcified renal artery a n e u r y s m in one patient. All patients u n d e r g o i n g a t t e m p t e d revascularizat i o n w e r e evaluated p o s t o p e r a t i v e l y w i t h a renal flow
scan a n d digital s u b t r a c t i o n o r c o n v e n t i o n a l renal arteriography. Serial s e r u m creatinine d e t e r m i n a t i o n s and b l o o d pressure recordings were o b t a i n e d at 3, 6, a n d 12 m o n t h s postoperatively, a n d at yearly intervals thereafter. F o l l o w - u p i n f o r m a t i o n was obtained t h r o u g h direct contact w i t h the patients o r their referring physicians. T h e f o l l o w - u p interval r a n g e d f r o m 6 m o n t h s to 7 years ( m e a n 2.5 years). RESULTS T h e p r i m a r y indications for u n d e r t a k i n g autot r a n s p l a n t a t i o n in this series were branch renal artery disease r e q u i r i n g extracorporeal mierovascular repair in 14 patients, h y p o p l a s i a o f the a b d o m i n a l aorta in one patient, a n d the presence o f a small-caliber dis-
Volume 2 Number 3 May 1985
Renal autotransplantation in young patients 387
Fig. 1A. Selective right renal arteriography in 12-year-old girl (patient 9) demonstrates intrarenal branch disease involving each of two right renal arteries. Pathologic diagnosis was intimal fibroplasia with dissection.
Fig. l B . Patient 9 (see legend to Fig. 1A). Sketch illustrating preoperative extent of branch renal artery disease (left) and method of extracorporeal revascularization with a branched autogenous saphenous vein graft (right). eased main renal artery in one infant. The method of renal autotransplantation and techniques for performing extracorporeal revascularization are described in detail elsewhere, n'12 When extracorporeal revascularization was done, the ureter was divided and the kidney was repaired on a separate workbench; this was followed by autotransplantation and ureteroneocystostomy. When autotransplantation alone was performed, the ureter was left intact.
Table I describes the clinical characteristics and specific operations performed in these 16 patients with renal artery disease. Autotransplantation alone was attempted in two patients. In one patient with a hypoplastic abdominal aorta (patient 1), this was successfully performed. In a second patient, an infant with a very small diseased main renal artery (patient 2), autotransplantation was not possiblc because of the unsuspected finding at surgery of obliteration of
388
Jordan, Novick, and Cunningham
Fig. lC. Patient 9 (sec legend Figs. 1A and 1B). Operative photograph of completed extracorporeal vascular repair just prior to renal autotransplantation. the inferior vena cava and iliac veins; a nephrcctomy was done in this case. Extracorporeal renal vascular reconstruction and autotransplantation were attempted on 15 occasions in 14 patients with intrarenal branch artcrial diseasc. The number of diseased arterial branchcs requiring repair ranged from onc to four With a mean of 2.8 diseased branches for each operated kidney (Fig. 1). Extracorporcal revascularization and autotransplantation werc successfully performed in 14 cases, including one patient (patient 16) who underwent staged bilateral autotransplants (Figs. 2 and 3). Extracorporeal rcvascularization was performed with a branched autogenous vascular graft in 12 cases (hypogastric artery = 10, saphenous vein = 1, and infcrior cpigastric artery = 1) and by aneurysmectomy with primary closure in two cases. In one patient with focal stenosis and an aneurysm involving a tertiary intrarenal arterial branch (patient 8), the diseased portion of the branch could not be completely exposed even after an extensive extracorporeal dissection into the renal sinus (Fig. 4A and Fig. 4B). At this point the branch was simply ligated proximal to the diseased segment and autotransplantation was performed. A postoperative isotope renal scan dem-
Journal of VASCULAR SURGERY
Fig. 2A. Left renal arteriogram in ]9-year-old woman demonstrates aneurysmal and stenosing fibrous disease involving main renal artery and three branches. onstrated excellent function of the autotransplanted kidney with sacrifice of only a small amount of functioning renal parenchyma. This patient experienced residual hypertension for 1 month postoperatively, following which she has remained normotensive wiV~ no requirement for antihypertensive medication. Staged bilateral revascularizations were done in six of eight patients with bilateral renal artery disease. In two patients only unilateral revascularization was performed because of minimal vascular disease involving the opposite renal artery; both of these patients currently have a normal blood pressure with no requirement for antihypertensive medication. Successful renal revascularization was accomplished in 14 of the 16 patients in the overall series. Postoperative isotope renographic and angiographic studies have shown no cases of stenosis or occlusion involving any of the repaired main or segmental renal arteries. Currently all 16 patients have a normal blood pressure. Thirteen patients do not require drug therapy whereas three patients are receiving low-dose antihypertensive medication. Postoperative renal function is stable or improved in all patients and the
Volume 2 Number 3 May 1985
Renal autotransplantation in young patients 389
Fig. 2B. See legend to Fig. 2A. Sketch illustrating extent of vascular disease in patient (left) and method ofextracorporeal revascularization with branched graft ofhypogastric artery (right).
Fig. 2C. Postoperative arteriogram of patient (see Fig. 2A) following autotransplantation demonstrates patent main renal artery and repaired branches. current mean serum creatinine level is 0.9 mg/dl. There were two postoperative complications in this series--a hydrothorax in one patient and a rctroperitoneal fluid collection in another patient, both of which resolved with appropriate treatment. DISCUSSION
Renal artery disease in the pediatric or young adult patient is generally caused by one of the fibrous dysplasias, most commonly intimal or perimedial fi-
Fig. 3A. Patient illustrated in Fig. 2 had tmdergone left aortorenal saphenous vein bypass 6 years previously. Right renal arteriography demonstrates mild expansion of saphenous vein graft and branch stenoses (arrow) beyond site of anastomosis. broplasia. Other causes include an arterial aneurysm, arteriovcnous malformation, Takayasu's arteritis, neurofibromatosis, thromboembolic disease, and trauma. 13 The typical clinical presentation for most of these disorders is an asymptomatic patient with recently discovered hypertension by a routine physical examination. Bilateral and/or branch renal artery involvement are frequently observed, particularly with the fibrous dysplasias, and extrarenal vascular disease may also be present? 4
390 Jordan, Novick, and Cunningham
Journal of VASCULAR SURGERY
Fig. 3B. See legend to Fig. 3A. Following extracorporeal revascularization and autotransplantation, digital subtraction angiography shows patent right main renal artery and branches (arrow). On pathologic study, right renal artery branches were found to be involved with recurrent fibrous dysplasia.
Fig. 4A. Selective right renal arteriogram demonstrates stenosis and aneurysm involving tertiary intrarenal upper pole arterial branch (arrow).
Fig. 3C. See legends to Figs. 3A and 3B. Postoperative intravenous pyelography demonstrates excellent unobstructed fimction of both autotransplanted kidneys. 'Since Leadbetter and Burkland is first reported cure of renovascular hypertension in a child by nephrectomy in 1938, surgical treatment has become established as the optimal approach to management of this disease. Operative therapy ofrenovascular disease in the young patient is generally directed at relief
of hypertension, although preservation of renal function is of equal concern with diseases that are known to cause progressive vascular obstruction, such as intimal or perimedial fibroplasia.16 Occasionally resection of a renal artery aneurysm is indicated to obviG% the risk of rupture associated with certain clinical features.17 Although new medical agents, such as beta blockers and converting enzyme inhibitors, have proved very effective in treating renin-mediated hypertension, they have no place in the definitive treatment of young patients since their use would mean lifelong comrfiitment to drug therapy w i t h the significant risk of losing renal function from progressive disease. It is currently impossible to determine the role of percutaneous transluminal angioplasty in the treatment of these types of renovascular disease because of insufficient long-term data on its use in these settings. Also the presence of branch renal artery disease has thus far been considered a contraindication to undertaking percutaneous transluminal angioplasty in most centers, is In general the cure rates following surgical treat-
Volume 2 Number 3 May 1985
Renal autotransplantation in young patients 391
Fig. 4B. See legend to Fig. 4A. Operative photograph of removed flushed kidney following extensive extracorporeal dissection into renal sinus. Diseased portion of involved branch (arrow) was not accessiblesurgically. This branch was then ligated. (From: Novick AC. Microvascular reconstruction of complex branch renal artery disease. Urol Clin North Am 1984; 11:465.)
ment of renovascular hypertension have been better in children than in adults, probably because of a shorter duration of hypertension in the former. Yet, prior to 1972, surgical cure of this disease in children was most often achieved through nephrectomy rather than by revascularization.l'2 More recent reports during the past decade have demonstrated that renal vascular reconstruction can now bc successfiflly performed in most pediatric patients. 3-~°This evolution has occurred both because of the development of microvascular techniques as well as a better appreciation of specific operative approaches that are most ('~cacious in children. It is important to emphasize that pediatric renal artery disorders should be repaired as soon as the diagnosis is made, rather than waiting until the child is older and the vessels larger, as some have advocated. The latter approach carries the risk of developing secondary nephrosclerosis from suboptimally controlled hypertension or ischemic atrophy of the involved kidney caused by progressive renal artery obstruction. The special problems associated with renal vascular reconstruction in children relate primarily to the small size of the diseased vessels and frequent branch renal artery involvement. In addition, the available surgical options for performing revascularization are more limited since some techniques that have proved effective in adults are contraindicated in children. For example, splenorenal bypass is a satisfactory operation in selected adult patients but has
yielded very poor results in children and is best avoided in this age group. 7'19 Also, the problem of aneurysmal expansion of aortorenal saphenous vein grafts has been observed more frequently in small children. 2°,21 This may reflect an enhanced susceptibility to mural ischcmia of vein segments procured from younger patients, but, regardless, it would seem prudent not to use saphcnous vein grafts in the prepubertal age group. Aortorenal bypass with an autogenous vascular graft is currently the mainstay technique for performing renal rcvascularization in the pediatric patient. A graft of autogenous hypogastric artery is preferred; however, in older children a saphenous vein graft is also acceptable. In some children with proximal renal artery disease and an adequate amount of disease-free distal artery, aortorenal reimplantation may bc performedY Although the indications for the latter technique are limited, it has yielded excellent results and has the advantage of requiring only a single vascular anastomosis. Renal autotransplantation alone, without extracorporeal vascular repair, is indicated in children with aortic hypoplasia that has also been termed "the middie aortic syndrome. "23 In these patients the iliac vessels are generally spared and moving the kidney into the iliac fossa away from the diseased aorta provides satisfactory treatment. Also, in the small child in whom use of a saphenous vein graft is contraindicated, autotransplantation is generally the second-
392
Jordan, Novick, and Cunningham
choice procedure when aortorenal bypass with a hypogastric arterial graft is not possible. Extracorporeal microvascular reconstruction and autotransplantation are indicated for disease involving branches of the renal artery that are either intrarenal or less than 1.5 mm in diameter. 12'24 Prior to the development of this approach, many patients with branch renal artery disease were considered either inoperable or candidates for total or partial nephrectomy. The advantages of extracorporeal revascularization include optimum exposure and illumination, a bloodless surgical field, greater protection of the kidney from ischemia, and more facile employment of microvascular techniques and optical magnification. Removing and flushing the kidney also causes it to contract in size, thereby enabling more peripheral dissection in the renal sinus for mobilization of distal arterial branches. Finally, the completed branch anastomoses can be tested for patency and integrity prior to autotransplantation. In the present series successful vascular reconstruction was accomplished with this approach in 13 of 14 patients with branch renal artery disease, including one patient who underwent staged bilateral extracorporeal repairs. In one patient with a single diseased renal artery branch that proved inaccessible, simple ligation of the isolated branch and autotransplantation were performed; this resulted in normalization of the blood pressure while preserving functioning parenchyma from the uninvolved part of the kidney. CONCLUSIONS Renovascular hypertension in the child or young adult is a potentially curable disease and revascularization with an autogenous vascular graft currently represents the optimum form of surgical therapy. It is now possible to achieve successful vascular reconstruction with preservation of functioning parenchyma ha the majority of these patients. The development of extracorporeal surgery and autotransplantation has been an important advance toward improving the technical efficacy of renal vascular reconstruction, particularly in this age group. REFERENCES
1. Foster JH, Pertinger WA, Oates JA, Rhamy RK, Klatte EC, Burko HC, Bolasny BL, Gordon R, Puyau FA, Younger RK. Malignant hypertension secondary to renal artery stenosis in children. Ann Surg 1966; 164:700-13. 2. Coran AG, Schuster SR. Renovascular hypertension in childhood. Surgery 1968; 64:672-7. 3. Coran AG, Whitehouse Jr WM, Stanley JC. Technical considerations in the surgical management of renovascular hypertension in children. J Pediatr Surg 1981; 16:890-7.
Journal of VASCULAR SURGERY
4. Fry WJ, Ernst CB, Stanley JC, Brink B. Renovascular hypertension in the pediatric patient. Arch Surg 1973; 107: 692-8. 5. Hendren WH, Kim SH, Herrin JT, Crawford JD. Surgically correctable hypertension of renal origin in childhood. Am J Surg 1982; 143:432-42. 6. Kaufman JJ, Goodwin WE, Waisman J, Gyepes MT. Renovascular hypertension in children. Report of seven cases treated surgically including two cases of renal autotransplantation. Am J Surg 1972; 124:149-57. 7. Novick AC, Straffon RA, Stewart BH, Benjamin S. Surgical treatment of renovascular hypertension in the pediatric patient. J Urol 1978; 119:794-9. 8. Stoney RJ, Cook PA, String ST. Surgical treatment of renovascular hypertension in children. J Pediatr Surg 1975; 10:631-9. 9. Stanley JC, Fry WI. Pediatric renal artery occlusive disease and renovascular hypertension. Etiology, diagnosis and operative treatment. Arch Surg 1981; 116:669-76. 10. Kyriakides GK, Najarian JS. Renovascular hypertension, ii children: Successful treatment by renal autotransplantation. Surgery 1979; 86:611-6. 11. Novick AC. Extracorporeal renal surgery and autotransplantation. In: Novick AC, Straffon RA, eds. Vascular problems in urologic surgery. Philadelphia: WB Saunders Co, 1982. 12. Novick AC. Management ofintrarenal branch arterial lesions with extracorporeal microvascular reconstruction and autotransplantation. J Urol 1981; 126:150-4. 13. Novick AC. Renal vascular hypertension in children. In: Kelalis P, King L, Belman B, eds. Clinical pediatric urology. Philadelphia: WB Saunders Co, 1984. 14. Rybka SJ, Novick AC. Concomitant, carotid, mesenteric and renal artery stenosis due to primary intimal fibroplasia. J Urol 1983; 129:798-800. 15. Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol 1938; 39:611-26. 16. Meaney TF, Dustan HP~ McCormack LJ. Natural history of renal artery disease. Radiology 1968; 91:881-7. 17. Poutasse EF. Renal artery aneurysm. J Urol 1975; 113: 443-9. 18. Flechner SM. Percutaneoustransluminaldilatation.Areali~r~c appraisal in patients with stenosing lesions of the renal arte,-j. Urol Clin North Am 1984; 11:515-27. 19. Vermeulen F, Stas F, Delegher C, Buyssens N, Stalpaert G, Proesmans W, Lauwers G, Sloof F~ van Pelt L, van Gorp L. Surgical correction of renovascular hypertension in children. J Cardiovasc Surg 1975; 16:21-34. 20. Dean RH, Wilson JP, Burko H, Foster JH. Saphenous vein aortorenal bypass grafts: Serial arteriographic study. Ann Surg 1974; 180:469-78. 21. Stanley JC, Ernst CB, Fry WJ. Fate of 100 aortorenal vein grafts: Characteristics of late graft expansion, aneurysmal dilation, and stenosis. Surgery 1973; 74:931-44. 22. Noble MJ, Novick AC, Straffon RA, Stewart BH. Aortorenal reimplantation in treatment of renovascular hypertension. Urology 1979; 14:566-8. 23. Kaufman IJ. The middle aortic syndrome: Report of a case treated by renal autotransplantation. J Urol 1973; 109: 711-5. 24. Salvatierra O, Olcott C, Stoney RJ. Ex-vivo renal artery reconstruction using perfusion preservation. J Urol 1978; 119:16-9.
Cleveland, Ohio 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 reqtfiring extracorporeal revascularization (n = 14), abdominal aortic hypoplasia (n = 1), and renal artery disease in a small infant (n = 1). Renal revascxflarization 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 fimction. Extracorporeal surgery and autotransplantation have been important additions to the surgical armamentarium for renal artery disease. (J VAse SURG 1985; 2:385-92.) The exact incidence of hypertension in children is not known but is estimated at between 1.5% to 2.5%. Children and adults differ significantly in the spectrum of causative factors that may lead to an elevated blood pressure with a secondary etiology being much more common in children. Among such pediatric patients renal artery disease is secondary in frequency only to coarctation of the thoracic aorta as a cause of surgically correctable hypertension. Although renal revascularization has been estab!'~hed for over 20 years as an effective form of treatment for adult renovascular hypertension, the initial results in children were less satisfactory. 1,2 However, recent reports during the past decade have indicated that successful renal vascular reconstruction is now possible in most pediatric patients. 3-1°This evolution has been due both to the incorporation of microvascular techniques into the armamentarium of the renovascular surgeon as well as an enhanced appreciation of those reconstructive operations that are more satisfactory in children. We report herein our experience with extracorporeal renal revascularizaFrom the Departments of Urology (Drs. Jordan and Novick) and Nephrology (Dr. Cunningham),The Cleveland Clinic Foundation. Reprint requests: Andrew C. Novick, M.D., Head, Section of RenalTransplantation,Deparmlentof Urology,The Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland,OH 44106.
tion and autotransplantation in pediatric and young adult patients. This is a relatively new approach that has helped to improve the outlook for patients in this age group with renal artery disease. MATERIAL AND METHODS From January 1977 to March 1984, renal autotransplantation with or without extracorporeal revascularization was attempted in 16 pediatric and young adult patients with renal artery disease. This series includes seven male and nine female patients, ranging in age from 10 months to 21 years. Renovascular pathologic conditions comprised intimal fibroplasia in seven patients, perimedial fibroplasia in five patients, Takayasu's arteritis in two patients, and an arterial aneurysm in two patients. Renal artery disease was present unilaterally in eight patients and bilaterally in eight patients. In six patients extrarenal vascular disease was also present as manifested by stenosis of both the carotid and mesenteric vessels in two patients, stenosis of the mesenteric vessels alone in one patient, a hypoplastic abdominal aorta in one patient, an aneurysm of the common iliac artery in one patient, and multiple peripheral aneurysms in one patient. Fifteen patients were admitted for evaluation of severe hypertension. All patients were studied preoperatively with the usual diagnostic procedures in385
386
Journal of VASCULAR SURGERY
Jordan, Novick, and Cunningham
T a b l e I. Clinical characteristics a n d surgical t r e a t m e n t in sixteen patients w i t h renal vascular disease
Patient 1 2 3
Bilateral Pathologic renovascular Extrarenalvascular diagnosis disease disease
Age (yr)
Sex
10
M
TA
Yes
F M
IF PF
No No
---
10 mo 18
Aortic hypoplasia
4
16
F
PF
No
--
5
16
F
RAA
No
--
6
18
F
PF
Yes
--
7
20
F
IF
Yes
Carotid and mesenteric
8
9
F
IF
No
--
9
12
F
IF
No
--
10
15
M
TA
Yes
Common iliac artery
11
20
M
IF
No
--
12
9
M
RAA
Yes
13
12
F
PF
Yes
Multiple peripheral aneurysms Mesenteric
14
6
M
IF
No
--
15
21
M
IF
Yes
Carotid and mesenteric
16
17
F
PF
Yes
Operations
Current blood pressure
Left aortorenal bypass Normal Right renal autotransplant Left nephrectomy Normal Right ex vivo repair with hypogastric Normal artery graft, autotransplant Left ex vivo repair with hypogastric Normal artery graft, autotransplant Left ex vivo aneurysmectomy and Normal with primare closure, autotransplant medication Right aortorenal bypass Normal Left ex vivo repair with hypogastric artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo ligation of renal artery • Normal branch, autotransplant Right ex vivo repair with branched Normal saphenous vein graft, autotransplant Right in situ renal aneurysmectomy Normal with Left ex vivo repair with branched medication inferior epigastric artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo aneurysmectomy and Normal primary closure~ autotransplant Right aortorenal bypass Normal with Left ex vivo repair with hypogastric medication artery graft, autotransplant Right ex vivo repair with hypogastric Normal artery graft, autotransplant Right aortorenal bypass Normal Left ex vivo repair with hypogastric artery graft, autotransplant Left ex vivo repair with hypogastric Normal artery graft, autotransplant Right ex vivo repair with hypogastric artery graft, autotransplant
IF = intimal fibroplasia; PF = perimedial fibroplasia; TA = Takayasu's arteritis; RAA = renal artery aneurysm.
cluding intravenous p y e l o g r a p h y , i s o t o p e renography, a o r t o g r a p h y a n d selective renal arteriography, renal f u n c t i o n parameters, a n d peripheral a n d differential renal vein renin determinations. A n g i o g r a p h i c studies d e m o n s t r a t e d vascular disease confined to the m a i n renal artery in t w o patients, whereas involvem e n t o f o n e o r m o r e renal arterial branches was observed in 14 patients. T h e p r e o p e r a t i v e s e r u m creatinine level was < 2 . 0 m g / d l in all cases. Renal revascularization was i n d i c a t e d as t r e a t m e n t for severe h y p e r t e n s i o n in 15 patients and to prevent r u p t u r e o f a large noncalcified renal artery a n e u r y s m in one patient. All patients u n d e r g o i n g a t t e m p t e d revascularizat i o n w e r e evaluated p o s t o p e r a t i v e l y w i t h a renal flow
scan a n d digital s u b t r a c t i o n o r c o n v e n t i o n a l renal arteriography. Serial s e r u m creatinine d e t e r m i n a t i o n s and b l o o d pressure recordings were o b t a i n e d at 3, 6, a n d 12 m o n t h s postoperatively, a n d at yearly intervals thereafter. F o l l o w - u p i n f o r m a t i o n was obtained t h r o u g h direct contact w i t h the patients o r their referring physicians. T h e f o l l o w - u p interval r a n g e d f r o m 6 m o n t h s to 7 years ( m e a n 2.5 years). RESULTS T h e p r i m a r y indications for u n d e r t a k i n g autot r a n s p l a n t a t i o n in this series were branch renal artery disease r e q u i r i n g extracorporeal mierovascular repair in 14 patients, h y p o p l a s i a o f the a b d o m i n a l aorta in one patient, a n d the presence o f a small-caliber dis-
Volume 2 Number 3 May 1985
Renal autotransplantation in young patients 387
Fig. 1A. Selective right renal arteriography in 12-year-old girl (patient 9) demonstrates intrarenal branch disease involving each of two right renal arteries. Pathologic diagnosis was intimal fibroplasia with dissection.
Fig. l B . Patient 9 (see legend to Fig. 1A). Sketch illustrating preoperative extent of branch renal artery disease (left) and method of extracorporeal revascularization with a branched autogenous saphenous vein graft (right). eased main renal artery in one infant. The method of renal autotransplantation and techniques for performing extracorporeal revascularization are described in detail elsewhere, n'12 When extracorporeal revascularization was done, the ureter was divided and the kidney was repaired on a separate workbench; this was followed by autotransplantation and ureteroneocystostomy. When autotransplantation alone was performed, the ureter was left intact.
Table I describes the clinical characteristics and specific operations performed in these 16 patients with renal artery disease. Autotransplantation alone was attempted in two patients. In one patient with a hypoplastic abdominal aorta (patient 1), this was successfully performed. In a second patient, an infant with a very small diseased main renal artery (patient 2), autotransplantation was not possiblc because of the unsuspected finding at surgery of obliteration of
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Fig. lC. Patient 9 (sec legend Figs. 1A and 1B). Operative photograph of completed extracorporeal vascular repair just prior to renal autotransplantation. the inferior vena cava and iliac veins; a nephrcctomy was done in this case. Extracorporeal renal vascular reconstruction and autotransplantation were attempted on 15 occasions in 14 patients with intrarenal branch artcrial diseasc. The number of diseased arterial branchcs requiring repair ranged from onc to four With a mean of 2.8 diseased branches for each operated kidney (Fig. 1). Extracorporcal revascularization and autotransplantation werc successfully performed in 14 cases, including one patient (patient 16) who underwent staged bilateral autotransplants (Figs. 2 and 3). Extracorporeal rcvascularization was performed with a branched autogenous vascular graft in 12 cases (hypogastric artery = 10, saphenous vein = 1, and infcrior cpigastric artery = 1) and by aneurysmectomy with primary closure in two cases. In one patient with focal stenosis and an aneurysm involving a tertiary intrarenal arterial branch (patient 8), the diseased portion of the branch could not be completely exposed even after an extensive extracorporeal dissection into the renal sinus (Fig. 4A and Fig. 4B). At this point the branch was simply ligated proximal to the diseased segment and autotransplantation was performed. A postoperative isotope renal scan dem-
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Fig. 2A. Left renal arteriogram in ]9-year-old woman demonstrates aneurysmal and stenosing fibrous disease involving main renal artery and three branches. onstrated excellent function of the autotransplanted kidney with sacrifice of only a small amount of functioning renal parenchyma. This patient experienced residual hypertension for 1 month postoperatively, following which she has remained normotensive wiV~ no requirement for antihypertensive medication. Staged bilateral revascularizations were done in six of eight patients with bilateral renal artery disease. In two patients only unilateral revascularization was performed because of minimal vascular disease involving the opposite renal artery; both of these patients currently have a normal blood pressure with no requirement for antihypertensive medication. Successful renal revascularization was accomplished in 14 of the 16 patients in the overall series. Postoperative isotope renographic and angiographic studies have shown no cases of stenosis or occlusion involving any of the repaired main or segmental renal arteries. Currently all 16 patients have a normal blood pressure. Thirteen patients do not require drug therapy whereas three patients are receiving low-dose antihypertensive medication. Postoperative renal function is stable or improved in all patients and the
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Renal autotransplantation in young patients 389
Fig. 2B. See legend to Fig. 2A. Sketch illustrating extent of vascular disease in patient (left) and method ofextracorporeal revascularization with branched graft ofhypogastric artery (right).
Fig. 2C. Postoperative arteriogram of patient (see Fig. 2A) following autotransplantation demonstrates patent main renal artery and repaired branches. current mean serum creatinine level is 0.9 mg/dl. There were two postoperative complications in this series--a hydrothorax in one patient and a rctroperitoneal fluid collection in another patient, both of which resolved with appropriate treatment. DISCUSSION
Renal artery disease in the pediatric or young adult patient is generally caused by one of the fibrous dysplasias, most commonly intimal or perimedial fi-
Fig. 3A. Patient illustrated in Fig. 2 had tmdergone left aortorenal saphenous vein bypass 6 years previously. Right renal arteriography demonstrates mild expansion of saphenous vein graft and branch stenoses (arrow) beyond site of anastomosis. broplasia. Other causes include an arterial aneurysm, arteriovcnous malformation, Takayasu's arteritis, neurofibromatosis, thromboembolic disease, and trauma. 13 The typical clinical presentation for most of these disorders is an asymptomatic patient with recently discovered hypertension by a routine physical examination. Bilateral and/or branch renal artery involvement are frequently observed, particularly with the fibrous dysplasias, and extrarenal vascular disease may also be present? 4
390 Jordan, Novick, and Cunningham
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Fig. 3B. See legend to Fig. 3A. Following extracorporeal revascularization and autotransplantation, digital subtraction angiography shows patent right main renal artery and branches (arrow). On pathologic study, right renal artery branches were found to be involved with recurrent fibrous dysplasia.
Fig. 4A. Selective right renal arteriogram demonstrates stenosis and aneurysm involving tertiary intrarenal upper pole arterial branch (arrow).
Fig. 3C. See legends to Figs. 3A and 3B. Postoperative intravenous pyelography demonstrates excellent unobstructed fimction of both autotransplanted kidneys. 'Since Leadbetter and Burkland is first reported cure of renovascular hypertension in a child by nephrectomy in 1938, surgical treatment has become established as the optimal approach to management of this disease. Operative therapy ofrenovascular disease in the young patient is generally directed at relief
of hypertension, although preservation of renal function is of equal concern with diseases that are known to cause progressive vascular obstruction, such as intimal or perimedial fibroplasia.16 Occasionally resection of a renal artery aneurysm is indicated to obviG% the risk of rupture associated with certain clinical features.17 Although new medical agents, such as beta blockers and converting enzyme inhibitors, have proved very effective in treating renin-mediated hypertension, they have no place in the definitive treatment of young patients since their use would mean lifelong comrfiitment to drug therapy w i t h the significant risk of losing renal function from progressive disease. It is currently impossible to determine the role of percutaneous transluminal angioplasty in the treatment of these types of renovascular disease because of insufficient long-term data on its use in these settings. Also the presence of branch renal artery disease has thus far been considered a contraindication to undertaking percutaneous transluminal angioplasty in most centers, is In general the cure rates following surgical treat-
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Renal autotransplantation in young patients 391
Fig. 4B. See legend to Fig. 4A. Operative photograph of removed flushed kidney following extensive extracorporeal dissection into renal sinus. Diseased portion of involved branch (arrow) was not accessiblesurgically. This branch was then ligated. (From: Novick AC. Microvascular reconstruction of complex branch renal artery disease. Urol Clin North Am 1984; 11:465.)
ment of renovascular hypertension have been better in children than in adults, probably because of a shorter duration of hypertension in the former. Yet, prior to 1972, surgical cure of this disease in children was most often achieved through nephrectomy rather than by revascularization.l'2 More recent reports during the past decade have demonstrated that renal vascular reconstruction can now bc successfiflly performed in most pediatric patients. 3-~°This evolution has occurred both because of the development of microvascular techniques as well as a better appreciation of specific operative approaches that are most ('~cacious in children. It is important to emphasize that pediatric renal artery disorders should be repaired as soon as the diagnosis is made, rather than waiting until the child is older and the vessels larger, as some have advocated. The latter approach carries the risk of developing secondary nephrosclerosis from suboptimally controlled hypertension or ischemic atrophy of the involved kidney caused by progressive renal artery obstruction. The special problems associated with renal vascular reconstruction in children relate primarily to the small size of the diseased vessels and frequent branch renal artery involvement. In addition, the available surgical options for performing revascularization are more limited since some techniques that have proved effective in adults are contraindicated in children. For example, splenorenal bypass is a satisfactory operation in selected adult patients but has
yielded very poor results in children and is best avoided in this age group. 7'19 Also, the problem of aneurysmal expansion of aortorenal saphenous vein grafts has been observed more frequently in small children. 2°,21 This may reflect an enhanced susceptibility to mural ischcmia of vein segments procured from younger patients, but, regardless, it would seem prudent not to use saphcnous vein grafts in the prepubertal age group. Aortorenal bypass with an autogenous vascular graft is currently the mainstay technique for performing renal rcvascularization in the pediatric patient. A graft of autogenous hypogastric artery is preferred; however, in older children a saphenous vein graft is also acceptable. In some children with proximal renal artery disease and an adequate amount of disease-free distal artery, aortorenal reimplantation may bc performedY Although the indications for the latter technique are limited, it has yielded excellent results and has the advantage of requiring only a single vascular anastomosis. Renal autotransplantation alone, without extracorporeal vascular repair, is indicated in children with aortic hypoplasia that has also been termed "the middie aortic syndrome. "23 In these patients the iliac vessels are generally spared and moving the kidney into the iliac fossa away from the diseased aorta provides satisfactory treatment. Also, in the small child in whom use of a saphenous vein graft is contraindicated, autotransplantation is generally the second-
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choice procedure when aortorenal bypass with a hypogastric arterial graft is not possible. Extracorporeal microvascular reconstruction and autotransplantation are indicated for disease involving branches of the renal artery that are either intrarenal or less than 1.5 mm in diameter. 12'24 Prior to the development of this approach, many patients with branch renal artery disease were considered either inoperable or candidates for total or partial nephrectomy. The advantages of extracorporeal revascularization include optimum exposure and illumination, a bloodless surgical field, greater protection of the kidney from ischemia, and more facile employment of microvascular techniques and optical magnification. Removing and flushing the kidney also causes it to contract in size, thereby enabling more peripheral dissection in the renal sinus for mobilization of distal arterial branches. Finally, the completed branch anastomoses can be tested for patency and integrity prior to autotransplantation. In the present series successful vascular reconstruction was accomplished with this approach in 13 of 14 patients with branch renal artery disease, including one patient who underwent staged bilateral extracorporeal repairs. In one patient with a single diseased renal artery branch that proved inaccessible, simple ligation of the isolated branch and autotransplantation were performed; this resulted in normalization of the blood pressure while preserving functioning parenchyma from the uninvolved part of the kidney. CONCLUSIONS Renovascular hypertension in the child or young adult is a potentially curable disease and revascularization with an autogenous vascular graft currently represents the optimum form of surgical therapy. It is now possible to achieve successful vascular reconstruction with preservation of functioning parenchyma ha the majority of these patients. The development of extracorporeal surgery and autotransplantation has been an important advance toward improving the technical efficacy of renal vascular reconstruction, particularly in this age group. REFERENCES
1. Foster JH, Pertinger WA, Oates JA, Rhamy RK, Klatte EC, Burko HC, Bolasny BL, Gordon R, Puyau FA, Younger RK. Malignant hypertension secondary to renal artery stenosis in children. Ann Surg 1966; 164:700-13. 2. Coran AG, Schuster SR. Renovascular hypertension in childhood. Surgery 1968; 64:672-7. 3. Coran AG, Whitehouse Jr WM, Stanley JC. Technical considerations in the surgical management of renovascular hypertension in children. J Pediatr Surg 1981; 16:890-7.
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4. Fry WJ, Ernst CB, Stanley JC, Brink B. Renovascular hypertension in the pediatric patient. Arch Surg 1973; 107: 692-8. 5. Hendren WH, Kim SH, Herrin JT, Crawford JD. Surgically correctable hypertension of renal origin in childhood. Am J Surg 1982; 143:432-42. 6. Kaufman JJ, Goodwin WE, Waisman J, Gyepes MT. Renovascular hypertension in children. Report of seven cases treated surgically including two cases of renal autotransplantation. Am J Surg 1972; 124:149-57. 7. Novick AC, Straffon RA, Stewart BH, Benjamin S. Surgical treatment of renovascular hypertension in the pediatric patient. J Urol 1978; 119:794-9. 8. Stoney RJ, Cook PA, String ST. Surgical treatment of renovascular hypertension in children. J Pediatr Surg 1975; 10:631-9. 9. Stanley JC, Fry WI. Pediatric renal artery occlusive disease and renovascular hypertension. Etiology, diagnosis and operative treatment. Arch Surg 1981; 116:669-76. 10. Kyriakides GK, Najarian JS. Renovascular hypertension, ii children: Successful treatment by renal autotransplantation. Surgery 1979; 86:611-6. 11. Novick AC. Extracorporeal renal surgery and autotransplantation. In: Novick AC, Straffon RA, eds. Vascular problems in urologic surgery. Philadelphia: WB Saunders Co, 1982. 12. Novick AC. Management ofintrarenal branch arterial lesions with extracorporeal microvascular reconstruction and autotransplantation. J Urol 1981; 126:150-4. 13. Novick AC. Renal vascular hypertension in children. In: Kelalis P, King L, Belman B, eds. Clinical pediatric urology. Philadelphia: WB Saunders Co, 1984. 14. Rybka SJ, Novick AC. Concomitant, carotid, mesenteric and renal artery stenosis due to primary intimal fibroplasia. J Urol 1983; 129:798-800. 15. Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol 1938; 39:611-26. 16. Meaney TF, Dustan HP~ McCormack LJ. Natural history of renal artery disease. Radiology 1968; 91:881-7. 17. Poutasse EF. Renal artery aneurysm. J Urol 1975; 113: 443-9. 18. Flechner SM. Percutaneoustransluminaldilatation.Areali~r~c appraisal in patients with stenosing lesions of the renal arte,-j. Urol Clin North Am 1984; 11:515-27. 19. Vermeulen F, Stas F, Delegher C, Buyssens N, Stalpaert G, Proesmans W, Lauwers G, Sloof F~ van Pelt L, van Gorp L. Surgical correction of renovascular hypertension in children. J Cardiovasc Surg 1975; 16:21-34. 20. Dean RH, Wilson JP, Burko H, Foster JH. Saphenous vein aortorenal bypass grafts: Serial arteriographic study. Ann Surg 1974; 180:469-78. 21. Stanley JC, Ernst CB, Fry WJ. Fate of 100 aortorenal vein grafts: Characteristics of late graft expansion, aneurysmal dilation, and stenosis. Surgery 1973; 74:931-44. 22. Noble MJ, Novick AC, Straffon RA, Stewart BH. Aortorenal reimplantation in treatment of renovascular hypertension. Urology 1979; 14:566-8. 23. Kaufman IJ. The middle aortic syndrome: Report of a case treated by renal autotransplantation. J Urol 1973; 109: 711-5. 24. Salvatierra O, Olcott C, Stoney RJ. Ex-vivo renal artery reconstruction using perfusion preservation. J Urol 1978; 119:16-9.