Vol. 116. December Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1976 by The Williams & Wilkins Co.
RENAL TRANSPLANTATION IN PATIENTS WITH POSTERIOR URETHRAL VALVES KHALID M. H. BUTT, ANDREW MEYER, SAMUELL. KOUNTZ
AND
KEITH WATERHOUSE
From the Departments of Surgery and Urology, State University of New York, Downstate Medical Center, Brooklyn, New York
ABSTRACT
Abnormalities of the lower urinary tract are relative contraindications for kidney transplantation that have been overcome by corrective operations or construction of conduits. In our 2 patients with posterior urethral valves and defunctionalized lower urinary tracts kidney transplantation with ureteroneocystostomy and later resection of the valves resulted in satisfactory bladder function and normal voiding patterns. Therefore, urinary diversion was completely avoided. Generally, a normal lower urinary outflow tract has been considered a prerequisite for kidney transplantation. 1 Approximately 2.5 per cent of the total Human Renal Transplant Registry comprises recipients with congenital disease and obstructive uropathy. 2 The proportion of these entities in recipients who are less than 20 years old is considerably higher. It is obvious that the relative contraindication of an abnormal lower genitourinary tract for kidney transplantation can be overcome by proper surgical measures. We present 2 cases of end stage renal failure caused by obstruction from congenital posterior urethral valves in which proper planning helped avoid urinary diversion procedures. CASE REPORTS AND RESULTS
Case 1. H. H., a 27-year-old man, was admitted to the hospital for renal transplantation. When this patient was 7 years old bladder outlet obstruction had been noted and an operative procedure was done. The details of this procedure are not available. Four years later rickets, a rising blood urea nitrogen (BUN), marked dilatation of the ureters and free reflux were noted. When the patient was 14 years old a suprapubic cystostomy was done. When the patient was 20 years old (1966) marked azotemia (BUN ranging from 150 to 180 mg. per cent), systemic hypertension and convulsions were noted. After a few initial peritoneal dialyses the patient was placed on maintenance hemodialysis in July 1966. In December bilateral nephrectomy and ureterectomy were done and the patient was maintained on hemodialysis until the current hospitalization for kidney transplantation. On May 8, 1973 the patient received a kidney from an unrelated donor. The kidney was transplanted after preservation on the Belzer pulsatile perfusion apparatus for 4½ hours. There was immediate good urine production. Six days later, when the Foley catheter was removed, cystourethroscopy was done and the posterior urethral valves were resected. Subsequently, this patient had an irreversible rejection of the kidney and a graft nephrectomy and simultaneous retransplantation were done on May 31. The second kidney was from the patient's mother. As in the previous transplant the ureter was implanted into the bladder by a modified Politano-Leadbetter 3 technique. The patient had a small bladder capacity, not holding more than 50 cc at a time. Approximately 6 weeks after the transplant leakage of urine from the suprapubic area was controlled by placing a urethral catheter, with subsequent complete healing of the bladder. Followup 3 years postoperatively revealed exAccepted for publication May 28, 1976. Read at annual meeting of American Urological Association, Las Vegas, Nevada, May 16-20, 1976.
cellent kidney function with a serumcreatinine of approximately 1 mg. per cent and a urine voiding volume of 300 to 400 ml. Case 2. K. S., a 17-year-old male youth, was admitted to the hospital for placement of an arteriovenous fistula in preparation for maintenance hemodialysis. This patient had a 12-year history of urologic problems. When he was 5 years old he had presented with hematuria and posterior urethral valves were found. Bilateral cutaneous ureterostomy was done at that time. However, the patient continued to have progressive deterioration of renal function. In April 1975 he was prepared for hemodialysis and in June the patient underwent bilateral nephroureterectomy and a kidney from his brother was transplanted, with the ureter of the graft being implanted into the bladder. Six days later transurethral resection of the posterior urethral valves was done. Two weeks later a voiding cystourethrogram did not reveal any obstructive problems and the patient was discharged from the hospital (see figure). At 9-month followup the patient continued to have excellent kidney function with a creatinine of less than 1 mg. per cent and, whereas the initial voided volumes of urine were between 50 and 100 cc, the average voided specimen now measured between 400 and 500 cc and the patient had a perfectly normal pattern of urination. DISCUSSION
The bladders in our 2 patients had been defunctionalized for 7 and 12 years because of bilateral nephroureterectomy and supravesical urinary diversion, respectively. At the time of kidney transplantation the bladders in these patients were thick-walled and contracted, with small capacity. However, the capacities rapidly achieved normal dimensions in a few weeks. Schmaelzle and associates demonstrated experimentally that the apparently reduced capacity of the defunctionalized bladder is rapidly regained after function is restored. 4 This experimental work has been amply confirmed in clinical situations. Hendren described his experience with 32 children who had had previous urinary diversion and underwent an operation to refunctionalize the bladder. 5 Seventeen of these patients had urethral valves and 24 of the 32 children had been considered to have permanent urinary diversion. Hendren pointed out that it is not possible to test adequately bladder function prior to refunctionalization when the patient has had diversion for many years. He emphasized that the majority of these bladders, although small from long-standing disuse, would stretch to a reasonable capacity soon after refunctionalization. Tanagho described his experience with 12 young children with congenital bladder outlet obstruction from posterior
708
RENAL TRANSPLANTATION IN PATIENTS WITH POSTERIOR URETHRAL VALVES
709
in their 7 patients with previous total urinary diversion that the use of the bladder after kidney transplantation, by doing a ureteroneocystostomy, led to completely normal bladder function. 10 It also has been emphasized that it is difficult to assess the bladder when there is a suprapubic diversion, massive reflux and infection. Bilateral nephrectomy, ureterectomy and closure of cystostomy must be done prior to bladder evaluation. 11 In view of the difficulty in evaluating bladder function, Hendren has suggested that, in the absence of stigmas of the neurologic dysfunction of the bladder, refunctionalization of the bladder should always be done. He has not found any bladder function that proved entirely unsatisfactory. 5 In patients with posterior urethral valves, end stage renal failure, supravesical diversion or bilateral nephrectomy and ureterectomy, in whom the urinary tract is left dry, the timing of resection of posterior urethral valves is extremely important. With adequate urine production having been established with a successful renal allograft the urinary stream has a salutary effect in maintaining the appropriate caliber of the posterior urethral lumen after fulguration of the valves, which would, in a patient without a urinary stream, be expected to lead to scarring and stricture formation. On the basis of our experience with kidney transplantation in patients with posterior urethral valves, we suggest that it is indeed the optimal therapy. Urinary diversion by means of intestinal conduits and so forth should be avoided. The bladder has been shown to function normally, progressively enlarging its capacity to normal levels. Ablation of the urethral valves is best done after excellent urine production has been established. REFERENCES
Case 2. A, pre-transplant voiding cystourethrogram shows marked distension of prostatic urethra, shrunken bladder and poor flow of contrast medium through urethra. B, voidihg cystourethrogram after transplantation and posterior urethral valve resection shows improved flow of contrast medium through urethra, improved capacity of bladder and decreased distension of prostatic urethra.
urethral valves, who had had supravesical diversion for 2 months to 3 years. In 4 of these patients the bladder had contracted to such a degree that it failed to expand despite successful reconstruction of the urinary tract continuity. Tanagho made a plea to maintain the supravesical diversion of the decompensated upper urinary tract resulting from bladder outlet obstruction for the shortest possible time. 6 On the other hand, some authors are now recommending a complete reconstruction primarily for the infants with obstructive uropathy. 7 Successful use of intestinal conduits has been reported for kidney transplantation in patients with obstructive uropathy from posterior urethral valves or bladder neck obstruction. 8 • • In addition to the problems associated with major surgical procedures, such as the preparation of a conduit in a uremic patient, urinary tract conduits leave much to be desired in the quality of life for the patient. Cerilli and associates have shown
1. Merrill, J.P.: Medical management of the transplant patient. In: Human Transplantation. Edited by F. T. Rapaport and J. Dausset. New York: Grune & Stratton, 1968. 2. Advisory Committee to the Renal Transplant Registry: The 12th report of the Human Renal Transplant Registry. J .A.M .A., 233: 787, 1975. 3. Politano, V. A. and Leadbetter, W. F.: An operative technique for the correction of vesicoureteral reflux. J. Urol., 79: 932, 1958. 4. Schmaelzle, J. F., Cass, A. S. and Hinman, F., Jr.: Effect of disuse and restoration of function on vesical capacity. J. Urol., 101: 700, 1969. 5. Hendren, W. H.: Urinacy tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974. 6. Tanagho, E. A.: Congenitally obstructed bladders: fate after prolonged defunctionalization. J. Urol., 111: 102, 1974. 7. Hendren, W. H.: A new approach to infants with severe obstructive uropathy: early complete reconstruction. J. Pediat. Surg., 5: 184, 1970. 8. Stenzel, K. H., Stubenbord, W. T., Whitsell, J. C., Lewy, J. E., Riggio, R. R., Cheigh, J. S., Marshall, V. F. and Rubin, A. L.: Kidney transplantation. Use of intestinal conduits. J.A.M.A., 229: 534, 1974. 9. Markland, C., Kelly, W. D., Buselmeier, T., Kjellstrand, C., Simmons, R. and Najarian, J.: Renal transplantation into ileac urinary conduits. Transplant. Proc., 4: 629, 1972. 10. Cerilli, J., Anderson, G. W., Evans, W. E. and Smith, J.P.: Renal transplantation in patients with urinary tract abnormalities. Surgery, 79: 248, 1976. 11. Salvatierra, 0.: Discussion of article by Cerilli and associates. 10
THE JOURNAL OF UROLOGY
Copyright © 1976 by The Williams & Wilkins Co.
RENAL TRANSPLANTATION IN PATIENTS WITH POSTERIOR URETHRAL VALVES KHALID M. H. BUTT, ANDREW MEYER, SAMUELL. KOUNTZ
AND
KEITH WATERHOUSE
From the Departments of Surgery and Urology, State University of New York, Downstate Medical Center, Brooklyn, New York
ABSTRACT
Abnormalities of the lower urinary tract are relative contraindications for kidney transplantation that have been overcome by corrective operations or construction of conduits. In our 2 patients with posterior urethral valves and defunctionalized lower urinary tracts kidney transplantation with ureteroneocystostomy and later resection of the valves resulted in satisfactory bladder function and normal voiding patterns. Therefore, urinary diversion was completely avoided. Generally, a normal lower urinary outflow tract has been considered a prerequisite for kidney transplantation. 1 Approximately 2.5 per cent of the total Human Renal Transplant Registry comprises recipients with congenital disease and obstructive uropathy. 2 The proportion of these entities in recipients who are less than 20 years old is considerably higher. It is obvious that the relative contraindication of an abnormal lower genitourinary tract for kidney transplantation can be overcome by proper surgical measures. We present 2 cases of end stage renal failure caused by obstruction from congenital posterior urethral valves in which proper planning helped avoid urinary diversion procedures. CASE REPORTS AND RESULTS
Case 1. H. H., a 27-year-old man, was admitted to the hospital for renal transplantation. When this patient was 7 years old bladder outlet obstruction had been noted and an operative procedure was done. The details of this procedure are not available. Four years later rickets, a rising blood urea nitrogen (BUN), marked dilatation of the ureters and free reflux were noted. When the patient was 14 years old a suprapubic cystostomy was done. When the patient was 20 years old (1966) marked azotemia (BUN ranging from 150 to 180 mg. per cent), systemic hypertension and convulsions were noted. After a few initial peritoneal dialyses the patient was placed on maintenance hemodialysis in July 1966. In December bilateral nephrectomy and ureterectomy were done and the patient was maintained on hemodialysis until the current hospitalization for kidney transplantation. On May 8, 1973 the patient received a kidney from an unrelated donor. The kidney was transplanted after preservation on the Belzer pulsatile perfusion apparatus for 4½ hours. There was immediate good urine production. Six days later, when the Foley catheter was removed, cystourethroscopy was done and the posterior urethral valves were resected. Subsequently, this patient had an irreversible rejection of the kidney and a graft nephrectomy and simultaneous retransplantation were done on May 31. The second kidney was from the patient's mother. As in the previous transplant the ureter was implanted into the bladder by a modified Politano-Leadbetter 3 technique. The patient had a small bladder capacity, not holding more than 50 cc at a time. Approximately 6 weeks after the transplant leakage of urine from the suprapubic area was controlled by placing a urethral catheter, with subsequent complete healing of the bladder. Followup 3 years postoperatively revealed exAccepted for publication May 28, 1976. Read at annual meeting of American Urological Association, Las Vegas, Nevada, May 16-20, 1976.
cellent kidney function with a serumcreatinine of approximately 1 mg. per cent and a urine voiding volume of 300 to 400 ml. Case 2. K. S., a 17-year-old male youth, was admitted to the hospital for placement of an arteriovenous fistula in preparation for maintenance hemodialysis. This patient had a 12-year history of urologic problems. When he was 5 years old he had presented with hematuria and posterior urethral valves were found. Bilateral cutaneous ureterostomy was done at that time. However, the patient continued to have progressive deterioration of renal function. In April 1975 he was prepared for hemodialysis and in June the patient underwent bilateral nephroureterectomy and a kidney from his brother was transplanted, with the ureter of the graft being implanted into the bladder. Six days later transurethral resection of the posterior urethral valves was done. Two weeks later a voiding cystourethrogram did not reveal any obstructive problems and the patient was discharged from the hospital (see figure). At 9-month followup the patient continued to have excellent kidney function with a creatinine of less than 1 mg. per cent and, whereas the initial voided volumes of urine were between 50 and 100 cc, the average voided specimen now measured between 400 and 500 cc and the patient had a perfectly normal pattern of urination. DISCUSSION
The bladders in our 2 patients had been defunctionalized for 7 and 12 years because of bilateral nephroureterectomy and supravesical urinary diversion, respectively. At the time of kidney transplantation the bladders in these patients were thick-walled and contracted, with small capacity. However, the capacities rapidly achieved normal dimensions in a few weeks. Schmaelzle and associates demonstrated experimentally that the apparently reduced capacity of the defunctionalized bladder is rapidly regained after function is restored. 4 This experimental work has been amply confirmed in clinical situations. Hendren described his experience with 32 children who had had previous urinary diversion and underwent an operation to refunctionalize the bladder. 5 Seventeen of these patients had urethral valves and 24 of the 32 children had been considered to have permanent urinary diversion. Hendren pointed out that it is not possible to test adequately bladder function prior to refunctionalization when the patient has had diversion for many years. He emphasized that the majority of these bladders, although small from long-standing disuse, would stretch to a reasonable capacity soon after refunctionalization. Tanagho described his experience with 12 young children with congenital bladder outlet obstruction from posterior
708
RENAL TRANSPLANTATION IN PATIENTS WITH POSTERIOR URETHRAL VALVES
709
in their 7 patients with previous total urinary diversion that the use of the bladder after kidney transplantation, by doing a ureteroneocystostomy, led to completely normal bladder function. 10 It also has been emphasized that it is difficult to assess the bladder when there is a suprapubic diversion, massive reflux and infection. Bilateral nephrectomy, ureterectomy and closure of cystostomy must be done prior to bladder evaluation. 11 In view of the difficulty in evaluating bladder function, Hendren has suggested that, in the absence of stigmas of the neurologic dysfunction of the bladder, refunctionalization of the bladder should always be done. He has not found any bladder function that proved entirely unsatisfactory. 5 In patients with posterior urethral valves, end stage renal failure, supravesical diversion or bilateral nephrectomy and ureterectomy, in whom the urinary tract is left dry, the timing of resection of posterior urethral valves is extremely important. With adequate urine production having been established with a successful renal allograft the urinary stream has a salutary effect in maintaining the appropriate caliber of the posterior urethral lumen after fulguration of the valves, which would, in a patient without a urinary stream, be expected to lead to scarring and stricture formation. On the basis of our experience with kidney transplantation in patients with posterior urethral valves, we suggest that it is indeed the optimal therapy. Urinary diversion by means of intestinal conduits and so forth should be avoided. The bladder has been shown to function normally, progressively enlarging its capacity to normal levels. Ablation of the urethral valves is best done after excellent urine production has been established. REFERENCES
Case 2. A, pre-transplant voiding cystourethrogram shows marked distension of prostatic urethra, shrunken bladder and poor flow of contrast medium through urethra. B, voidihg cystourethrogram after transplantation and posterior urethral valve resection shows improved flow of contrast medium through urethra, improved capacity of bladder and decreased distension of prostatic urethra.
urethral valves, who had had supravesical diversion for 2 months to 3 years. In 4 of these patients the bladder had contracted to such a degree that it failed to expand despite successful reconstruction of the urinary tract continuity. Tanagho made a plea to maintain the supravesical diversion of the decompensated upper urinary tract resulting from bladder outlet obstruction for the shortest possible time. 6 On the other hand, some authors are now recommending a complete reconstruction primarily for the infants with obstructive uropathy. 7 Successful use of intestinal conduits has been reported for kidney transplantation in patients with obstructive uropathy from posterior urethral valves or bladder neck obstruction. 8 • • In addition to the problems associated with major surgical procedures, such as the preparation of a conduit in a uremic patient, urinary tract conduits leave much to be desired in the quality of life for the patient. Cerilli and associates have shown
1. Merrill, J.P.: Medical management of the transplant patient. In: Human Transplantation. Edited by F. T. Rapaport and J. Dausset. New York: Grune & Stratton, 1968. 2. Advisory Committee to the Renal Transplant Registry: The 12th report of the Human Renal Transplant Registry. J .A.M .A., 233: 787, 1975. 3. Politano, V. A. and Leadbetter, W. F.: An operative technique for the correction of vesicoureteral reflux. J. Urol., 79: 932, 1958. 4. Schmaelzle, J. F., Cass, A. S. and Hinman, F., Jr.: Effect of disuse and restoration of function on vesical capacity. J. Urol., 101: 700, 1969. 5. Hendren, W. H.: Urinacy tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974. 6. Tanagho, E. A.: Congenitally obstructed bladders: fate after prolonged defunctionalization. J. Urol., 111: 102, 1974. 7. Hendren, W. H.: A new approach to infants with severe obstructive uropathy: early complete reconstruction. J. Pediat. Surg., 5: 184, 1970. 8. Stenzel, K. H., Stubenbord, W. T., Whitsell, J. C., Lewy, J. E., Riggio, R. R., Cheigh, J. S., Marshall, V. F. and Rubin, A. L.: Kidney transplantation. Use of intestinal conduits. J.A.M.A., 229: 534, 1974. 9. Markland, C., Kelly, W. D., Buselmeier, T., Kjellstrand, C., Simmons, R. and Najarian, J.: Renal transplantation into ileac urinary conduits. Transplant. Proc., 4: 629, 1972. 10. Cerilli, J., Anderson, G. W., Evans, W. E. and Smith, J.P.: Renal transplantation in patients with urinary tract abnormalities. Surgery, 79: 248, 1976. 11. Salvatierra, 0.: Discussion of article by Cerilli and associates. 10
