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Renal Transplantation in Prune Belly Syndrome
Vol. 115, January Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
RENAL TRANSPLANTATION IN PRUNE BELLY SYNDROME JOHN H. SHENASKY, II*
AND
JOHN D. WHELCHEL
From the Department of Surgery, Urology Service and Transplantation Service, Wilford Hall United States Air Force Medical Center, Lackland Air Force Base, Texas
ABSTRACT
The first reported case of renal transplantation in a patient with prune belly syndrome is presented. Transplantation should be considered for patients with this syndrome in whom renal failure develops despite appropriate urologic reconstruction. A thorough preoperative evaluation ascertaining the status of the lower urinary tract is especially important in these patients. Prune belly syndrome is an unusual condition manifested by hypoplasia of the abdominal musculature, bilateral cryptorchidism ai;id varying degrees of abnormality of the urinary tract. The urologic management of and reconstructive surgery for this condition have been well reviewed previously. 1· ' Although death in these patients usually results from renal insufficiency we were unable to find a report of renal transplantation in such a patient. A recent successful transplant from a living related donor to a young adult with prune belly syndrome is reported herein. CASE REPORT
L. D., WHMC 641021, a 21-year-old wb.i:;e man, was born with prune belly syndrome. During the first year of life he
he was 19 years old and had azotemia. He denied voiding difficulty, was potent and had orgasms but did not ejaculate. Physical examination revealed a flaccid abdominal wall (part A of figure) and bilateral cryptorchidism. Blood pressure was 200/120. Urinalysis and culture yielded pyuria without infection. Hematocrit was 20 per cent, BUN 100 mg. per cent, creatinine 11. 2 mg. per cent, carbon dioxide 16 and peripheral renin values 1.42 ng. per ml. per hour (normal 0.53 plus or minus 0.3). Cystography showed bilateral total vesicorenal reflux but excellent bladder emptying (part B of figure). Cystometrography was interpreted as normal. Since we thought that the renal function would not be significantly improved by ureteroneocystostomy hemodialysis was instituted. After a year of dialysis the patient was re-evaluated
A, appearance of abdomen. Midline incision was used for nephroureterectomies and orchiopexy. Renal transplantation was accomplished through incision just visible above belt in left lower quadrant. B, voiding cystourethrogram demonstrates total bilateral reflux but non-obstructed lower urinary tract.
underwent circumcision, suprapubic cystostomy, excision of a vesicoumbilical fistula (patent urachus) and appendectomy at another institution. When he was 2 years old excretory urography revealed a grossly normal right kidney but marked left pyelocaliectasis. Blood urea nitrogen (BUN) was 22 mg. per cent. The patient did not return for further evaluation until Accepted for publication July 3, 1975. The views expressed herein are those of the authors and do not necessarily reflect the views of the United States Air Force. * Current address: 16 Medical Center, Salisbury, Maryland 21801. 112
and accepted for kidney transplantation. Following a preparatory operation, including bilateral nephroureterectomy, right orchiectomy and left orchiopexy, the patient became normotensive. In September 1974 a 2 antigen match kidney from his sister was transplanted into the left iliac fossa. Ureteroneocystostomy of the donor ureter into the recipient bladder was accomplished without difficulty. The kidney has maintained excellent function (creatinine 0.8 mg. per cent), the urine is sterile and the patient has had no urologic complaints. It has
113
RENAL TRANSPLANTATION IN PRUNE BELLY SYNDROME
been 9 months since the transplantation and he is now gainfully employed on a full-time basis. DISCUSSION
Renal <:!xsplasia ~ith malformation of the urinary tr:1_ct isthe most signifi!:'.l!Dt feature of prune belly SY.!1QIOffie. Wl1ile a significant percentage .of children with this condition iliel;e arIY~Fmiaanaseps 1s~"unnary--dJversfo"n with a subsequent reconst~tlveQ12er.ationwiIIeiiable_s_omepat1ents ri~pver_~tisfactory renJ:!LfvD-ctfon. . . . . . .. -It is unfortunate Th-a~c-our patient did not return for evaluation until he was 19 years old because he may well have benefited from ureteroneocystostomy since he had had sufficient renal parenchyma to sustain life for 19 years bilateral vesicorenal reflux. some with prune have inf'ravesical Williams and Burkholder demonstrated that in some of these patients the bladder and urethra function when to eliminate the a source of infection in the patient. The subsequent course of our patient has been unremarkable and the urodynamic status is normal. Our experience suggests that renal transplantation can be used in prune in whom renal failure despite adequate reconstruction when other therapeutic mo-
dalities have been exhausted. A thorough urologic evaluation will disclose the presence of any urinary tract abnormalities that need correction and will also determine the feasibility of using the patient's own bladder for transplantation. REFERENCES
1. Spence. H. M. and Allen. T.: Congenital absence of the abdominal
musculature. Urological aspects. J.A.M.A., 187: 814, 1964. 2. Williams, D. I. and Burkholder. G. V.: The prune belly syndrome. J. Urol., 98: 244, 1967. 3. Bourne, C. W. and Cerny. J. C.: Congenital absence of abdominal muscles: report of 6 cases. ,J. Urol., 98: 252, 1967. 4. Burke, E. C., Shin, M. H. and Kelalis, P. P.: Prune-belly syndrome. Clinical findings and survival. Amer. J. Dis. Child., 117: 668, 1969. 5. Waldbaum, R. S. and l\/iarshall, . F.: The prune belly syndrome: a diagnostic therapeutic plan. J. Urol., HJ3: 668. 1970.
CONlMENT This paper, reporting th2 first successful allotransplantation into a patient with prune belly and a normal lower urinary tr&ct, demonstrates the need to nreserve a functional bladder in th2,;e patients. Reconstrnctive surge,ry to ensure adequate emptying should be performed as well as an operation to preserve renal tissue. In the event that the operation on the latter fails the reconstructed lower tract could be used for renal allotransplantation as demonstrated these authors. Martin G, McLaughlin The Johns Hopkins Hospital Baltimore, Ivlaryland
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
RENAL TRANSPLANTATION IN PRUNE BELLY SYNDROME JOHN H. SHENASKY, II*
AND
JOHN D. WHELCHEL
From the Department of Surgery, Urology Service and Transplantation Service, Wilford Hall United States Air Force Medical Center, Lackland Air Force Base, Texas
ABSTRACT
The first reported case of renal transplantation in a patient with prune belly syndrome is presented. Transplantation should be considered for patients with this syndrome in whom renal failure develops despite appropriate urologic reconstruction. A thorough preoperative evaluation ascertaining the status of the lower urinary tract is especially important in these patients. Prune belly syndrome is an unusual condition manifested by hypoplasia of the abdominal musculature, bilateral cryptorchidism ai;id varying degrees of abnormality of the urinary tract. The urologic management of and reconstructive surgery for this condition have been well reviewed previously. 1· ' Although death in these patients usually results from renal insufficiency we were unable to find a report of renal transplantation in such a patient. A recent successful transplant from a living related donor to a young adult with prune belly syndrome is reported herein. CASE REPORT
L. D., WHMC 641021, a 21-year-old wb.i:;e man, was born with prune belly syndrome. During the first year of life he
he was 19 years old and had azotemia. He denied voiding difficulty, was potent and had orgasms but did not ejaculate. Physical examination revealed a flaccid abdominal wall (part A of figure) and bilateral cryptorchidism. Blood pressure was 200/120. Urinalysis and culture yielded pyuria without infection. Hematocrit was 20 per cent, BUN 100 mg. per cent, creatinine 11. 2 mg. per cent, carbon dioxide 16 and peripheral renin values 1.42 ng. per ml. per hour (normal 0.53 plus or minus 0.3). Cystography showed bilateral total vesicorenal reflux but excellent bladder emptying (part B of figure). Cystometrography was interpreted as normal. Since we thought that the renal function would not be significantly improved by ureteroneocystostomy hemodialysis was instituted. After a year of dialysis the patient was re-evaluated
A, appearance of abdomen. Midline incision was used for nephroureterectomies and orchiopexy. Renal transplantation was accomplished through incision just visible above belt in left lower quadrant. B, voiding cystourethrogram demonstrates total bilateral reflux but non-obstructed lower urinary tract.
underwent circumcision, suprapubic cystostomy, excision of a vesicoumbilical fistula (patent urachus) and appendectomy at another institution. When he was 2 years old excretory urography revealed a grossly normal right kidney but marked left pyelocaliectasis. Blood urea nitrogen (BUN) was 22 mg. per cent. The patient did not return for further evaluation until Accepted for publication July 3, 1975. The views expressed herein are those of the authors and do not necessarily reflect the views of the United States Air Force. * Current address: 16 Medical Center, Salisbury, Maryland 21801. 112
and accepted for kidney transplantation. Following a preparatory operation, including bilateral nephroureterectomy, right orchiectomy and left orchiopexy, the patient became normotensive. In September 1974 a 2 antigen match kidney from his sister was transplanted into the left iliac fossa. Ureteroneocystostomy of the donor ureter into the recipient bladder was accomplished without difficulty. The kidney has maintained excellent function (creatinine 0.8 mg. per cent), the urine is sterile and the patient has had no urologic complaints. It has
113
RENAL TRANSPLANTATION IN PRUNE BELLY SYNDROME
been 9 months since the transplantation and he is now gainfully employed on a full-time basis. DISCUSSION
Renal <:!xsplasia ~ith malformation of the urinary tr:1_ct isthe most signifi!:'.l!Dt feature of prune belly SY.!1QIOffie. Wl1ile a significant percentage .of children with this condition iliel;e arIY~Fmiaanaseps 1s~"unnary--dJversfo"n with a subsequent reconst~tlveQ12er.ationwiIIeiiable_s_omepat1ents ri~pver_~tisfactory renJ:!LfvD-ctfon. . . . . . .. -It is unfortunate Th-a~c-our patient did not return for evaluation until he was 19 years old because he may well have benefited from ureteroneocystostomy since he had had sufficient renal parenchyma to sustain life for 19 years bilateral vesicorenal reflux. some with prune have inf'ravesical Williams and Burkholder demonstrated that in some of these patients the bladder and urethra function when to eliminate the a source of infection in the patient. The subsequent course of our patient has been unremarkable and the urodynamic status is normal. Our experience suggests that renal transplantation can be used in prune in whom renal failure despite adequate reconstruction when other therapeutic mo-
dalities have been exhausted. A thorough urologic evaluation will disclose the presence of any urinary tract abnormalities that need correction and will also determine the feasibility of using the patient's own bladder for transplantation. REFERENCES
1. Spence. H. M. and Allen. T.: Congenital absence of the abdominal
musculature. Urological aspects. J.A.M.A., 187: 814, 1964. 2. Williams, D. I. and Burkholder. G. V.: The prune belly syndrome. J. Urol., 98: 244, 1967. 3. Bourne, C. W. and Cerny. J. C.: Congenital absence of abdominal muscles: report of 6 cases. ,J. Urol., 98: 252, 1967. 4. Burke, E. C., Shin, M. H. and Kelalis, P. P.: Prune-belly syndrome. Clinical findings and survival. Amer. J. Dis. Child., 117: 668, 1969. 5. Waldbaum, R. S. and l\/iarshall, . F.: The prune belly syndrome: a diagnostic therapeutic plan. J. Urol., HJ3: 668. 1970.
CONlMENT This paper, reporting th2 first successful allotransplantation into a patient with prune belly and a normal lower urinary tr&ct, demonstrates the need to nreserve a functional bladder in th2,;e patients. Reconstrnctive surge,ry to ensure adequate emptying should be performed as well as an operation to preserve renal tissue. In the event that the operation on the latter fails the reconstructed lower tract could be used for renal allotransplantation as demonstrated these authors. Martin G, McLaughlin The Johns Hopkins Hospital Baltimore, Ivlaryland