Reconstruction of the Urinary Tract in Prune Belly Uropathy

0022-5347/78/1196-0824$02.00/0 THE JOURNAL OF UROLOGY Copyright © 1978 by The Williams & Wilkins Co.

Vol. 119, June

Printed in U.SA.

RECONSTRUCTION OF THE URINARY TRACT IN PRUNE BELLY UROPATHY JOHN R. WOODARD

THOMAS S. PARROTT

AND

From the Department of Surgery (Urology), Emory University School of Medicine and the Henrietta Egleston Hospital for Children, Atlanta, Georgia

ABSTRACT

Ten patients with prune belly uropathy, including 7 neonates, were treated with extensive surgical tailoring of the upper urinary tracts using primarily the upper ureteral segment. Simultaneous transabdominal orchiopexy, particularly in the neonate, is described as a useful adjunct. Our early results in these patients justify this aggressive approach. With rare exceptions the prune belly syndrome occurs in male subjects and is characterized by a deficiency of the abdominal muscles, undescended testes and dilatation of the urinary collecting system. Most have intestinal malrotation, many have a talipes equinovarus deformity and some have a patent urachus. Also reported are congenital hip dislocation and a variety of other associated anomalies. H The syndrome occurs approximately once in 35,000 live births and its etiology, although unknown, is probably related to a morphogenic abnormality in the paraxial, intermediate and lateral plate mesoderm in the segmenting embryo around 23 days of development. 7• 8 Since the most consistent features of the syndrome are cryptorchidism and the characteristic uropathy it has intrigued urologists for many years. The bladder usually is attached to the umbilicus, thick and seemingly hypertrophied but not trabeculated. The bladder neck and trigone are illdefined and the prostatic urethra is peculiarly large and triangular.Although apparently narrow the prostatic urethra is seldom truly obstructed by stricture, atresia or hypoplasia. 2• 6 • 9• 10 Most impressive are the typically huge, tortuous dilated ureters with large, flabby renal pelves. Vesicoureteral reflux is usually present. The renal parenchyma may appear normal, although hydronephrosis is the rule and cysts with other dysplastic changes are common, a factor that may predetermine the chance for survival. The testes are usually intraabdominal and most often overlie the ureter at the pelvic brim.11, 12 To the urologist the full-blown syndrome presents a challenge, since almost half of the 156 patients described in the literature before 1970 were postmortem reports. Of these patients 20 per cent were stillborn or neonatal deaths and another 30 per cent died by age 2 years, chiefly ofrenal failure and urinary sepsis. 1• 11 · 13 Of the 87 cases published between 1950 and 1970 Waldbaum and Marshall were successful in tracing 56 patients. 14 Of these patients 39 (70 per cent) were dead and another 9 (16 per cent) appeared near death. That is, 86 per cent appeared to be treatment (or non-treatment) failures. With this statistical background we have adopted an aggressive approach to the management of prune belly uropathy. MATERIAL AND METROS

Since 1969 we have been directly responsible for the care of 16 private patients with prune belly syndrome at the Henrietta Egleston Hospital for Children. Most of the spectra of this syndrome are filled by these patients. Two had less than Accepted for publication July 15, 1977. Read at annual meeting of American Urological Association, Chicago, Illinois, April 24-28, 1977.

complete deficiency in the abdominal wall, mild dilatation of the urinary tract, no reflux and have required treatment only for cryptorchidism. The parents of 1 severely affected child refused treatment and he died of urinary sepsis and uremia at the age of 5 years. Another child had severe renal dysplasia confirmed by biopsy, remained severely azotemic after satisfactory proximal loop ureterostomy and is considered no candidate for reconstruction. Two others died of pulmonary causes during the second day of life and never became candidates for urologic treatment. Herein we report on 10 patients with the full-blown syndrome who were treated during the last 7 years. Management consisted of extensive upper urinary tract reconstruction, either as a staged or primary procedure, and with orchiopexy (table 1). Complete followup data are available on each patient. Surgical technique. The abdomen is opened through a midline incision with excision of the umbilicus in the neonate. Currently, no attempt is made to remain extraperitoneally. The bladder is allowed to lie outside of the lower end of the incision, while the bowels are lifted from the upper end to afford superb exposure. The posterior peritoneum is incised over the ureters from the kidney to the bladder and the ureter is mobilized at the pelvic brim, making no attempt to straighten the kinks in the lower two-thirds since this portion is not to be used in the reconstruction (fig. 1, A). Before the ureter is detached from the bladder the ureteropelvic junction should be inspected. As the ureter is being mobilized care is taken to identify the spermatic vessels, which also are mobilized carefully (but not skeletonized) to their organs if necessary. The ureter is transected at its junction with the bladder. The extreme length of the ureters and entirely adequate length of the spermatic cords are obvious (fig. 2). In the neonate 5 to 6 cm. of proximal ureter is usually more than adequate to reach the bladder and, therefore, the remainder of the distal ureter is excised. The proximal ureter is straightened but unlike the typically huge lower ureter it may be only slightly dilated and may not require tapering (fig. 1, B). However, if it is as much as 1 cm. wide the ureter is tapered over a lOF catheter with the tapering often extending into the renal pelvis. A modified Paquin technique is used to reimplant the ureter and splinting ureteral catheters are left indwelling for 10 or 11 days. 15 A nephrostomy tube is usually left in each kidney until postoperative contrast studies indicate satisfactory ureteral drainage. We believe it is advantageous to resect the typically huge bladder dome after which the bladder is closed in layers around a Malecot catheter (fig. 1). 16 The orchiopexy is then completed by tunneling bluntly through the abdominal wall at the external inguinal ring with a hemostat that slips easily into the scrotum, where it can be spread to make a pouch. A silk suture through the tunica albuginea is used to draw the

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RECONSTRUCTION OF URINARY TRACT IN PRUNE BELLY UROPATHY TABLE

Case No.

1. Data on 10 consecutive patients undergoing extensive surgical tailoring of the upper urinary tracts for prune belly uropathy

PL

Hospital No.

Birth Date

Age at Presentation

JD

4-12-99

2-6-65

6 yrs.

TI

3-33-81

9-6-69

4 days

Previous Operation Loop ureterostomy and end ureterostomy None

3

IS

4-9-53

1-20-72

1 day

None

4

DM

4-27-15

8-10··72

1 day

None

5

GW

4-62-53

12-15-70

3 yrs.

Suprapubic tube

6

JW

5-17-46

9-16-75

3 days

None

MB

5,-27-75

2-1-76

2 days

None

8

H)

KE

5-34-32

4-28-76

2 days

None

DB

5-48-99

11-19-76

1 day

None

KH

5-52-93

1-14-77

1 day

None

Operations Performed Bilat. ureteroneocystostomy Lt orchiopexy and rt. orchiectomy Bilat. loop ureterostomy Bilat. dry reimplant Bilat. revision ureterovesical junction Bilat. ureteroneocystostorny Rt. ureteroneocystostomy with orchiopexy Lt. ureteroneocystostomy with orchiopexy Lt. ureteroneocystostomy Rt. ureteroneocystostomy with rt. orchiopexy Bilat. ureteroneocystostorny Bilat. orchiopexy Bilat. ureteroneocystostomy with IL orchiopexy Repeat lt. ureteroneocystostorny Bilat. pyeloplasty and vesicostomy Bilat. ureteroneocystostomy and bilaL orchiopexy Bilat. ureteroneocystostomy and bilat. orchiopexy Repeat bilat. ureternneocystostomy Rt. ureteroneocystostomy, lt. nephroureterectomy and bilat. orchiopexy Bilat. ureteroneocystostomy and bilat. orchiopexy

Age at Operation 7 yrn.

Time of

Followup 5 yrs,

11 9

2 yrs. 3 yrs. 5 yrs. 13 days

6½mos. 2wks. 3mos. 3 yrs. 5 10

2 yrs. 5 years

3½

4mos.

l1/zyrs.

5 days 5mos.

1 yr.

10 days

2¼mos. 13 days

1 YL 6mos.

12 days

4 m.os.

FIG. LA, typical prune belly uropathy with upper ureter being less severely dilated. Portion between arrows is to be discarded. Dotted line shows site of bladder dome resection. appearance after resection of bladder dome and ureteral reimplantation with bladdm· closure. Nephrostomy tubes and ureteral splints shown) are used.

testis into the scrotum and is tied over a button. 'l'he abdominal closure is routine. This total operation has been tolerated in the last 6 including 5 neonates. CASE REPORTS

Case 1 J. D. had undergone ureterostomies elsewhere followed by an unsuccessful attempt at reconstruction, resultm end ureterostomies. When the patient was 6 years the ureters were into the bladder using the ureter proximal to ureterostomy and removing ureteral stumps. The patient has undilated pyelogood bladder function and has remained free

T. had undergone loop cutaneous ureterostomy A tapered reimplantation 2 years later, even with revision, was unsuccessful (fig. 3). However, when patient was 5 years old a successful bilateral reimplantation was achieved using the upper ureter proximal to the w,t·c,r,,.:t.rrnnm and the distal

ureteral stumps. The dome of the bladder has been resected subsequently. Comment: These 2 cases demonstrate the small fraction of total ureteral length necessary for reconstruction and also tend to confirm the greater functional of the upper ureter. 17 Cases 3 and 4. I. S. and D. M. were reconstructed as neonates without the use of preliminary 4 and 5). However, it was considered prudent at that complete only 1 side at a time, leaving in a contralateral nephrostomy tube until the second side was reconstructed several months later. Comment: It was with these patients that the idee. simultaneous orchiopexy was conceived on the rationale that the spermatic vessels could be mobilized as the ureter was being straightened (fig. 6). 12 Both tolerated the procedures so well that in all (cases 5 to 10) bilateral reconstruction with orchiopexy has been done at a single operation.

826

WOODARD AND PARROTT

Case 7. M. B. had bilateral ureterovesical junction obstructions with tightly distended renal pelves, which were encountered before detaching the lower ureters. Bilateral dismembered pyeloplasty and a vesicostomy were performed, and the ureteral tailoring was postponed until 5 months later. If a kidney appeared non-contributory, as in case 9, it was removed along with its ureter. RESULTS

There have been no mortality and no serious morbidity from the operations performed on these 10 patients (table 2). All

have satisfactory drainage of the ureters into the bladder. There is only 1 patient with unilateral reflux and no patient has a serum creatinine in excess of0.9 mg. per cent. Recurrent bacteriuria has occurred in some children but never with clinical or laboratory evidence of renal involvement. All patients have 1 or both testes well down into the scrotum with an intact spermatic cord. Each child is well with normal growth. Although aggressive in attacking the reflux, ureteral redundancy and cryptorchidism we have, aside from resecting its dome, done nothing to combat deficient bladder detrusor function. This aspect has been evaluated recently by others. 18 DISCUSSION

Fm. 2. Surgical photograph of case 10 shows excessive ureteral length and adequate spermatic cord length after dissection.

The role of extensive surgical reconstruction of the urinary tract in patients with prune belly syndrome is understandably controversial. Some noted authorities recommend a much more conservative surgical approach or surveillance with medical treatment and no surgical intervention. 1• 6• 19 However, the excessively high mortality rate from urological complications is inescapable in this syndrome, with urinary sepsis and uremia the usual culprits. Even some patients who seem to do well enough to justify conservative treatment may die suddenly of urinary infection in early adulthood. 20 Despite the lack of urodynamic evidence of true obstruction it seems difficult to expect that such redundant and static urinary systems as these can be protected indefinitely from infection. The morphology of the ureter and bladder creates a difficult technical problem for the surgeon and many of the cases reported previously have been technical failures resulting in permanent urinary diversion. 10 However, with recent evidence that the upper few centimeters of ureter are structurally and functionally superior to the lower portion, 17 and with improved techniques and increased experience in extensive ureteral tailoring in the young infant, this failure rate should be reduced greatly. Resection of the dome of the bladder as suggested by Perlmutter, and perhaps additional measures to reduce bladder outlet resistance, may facilitate bladder emptying and further improve the prognosis. 16• 18 In these 10 consecutive patients from whom ureteral redundancy has been removed to eliminate stasis and reflux has been corrected we believe that the prognosis for longevity has been improved greatly. Furthermore, it is possible that the

Fm. 3. Case 2. A, neonatal excretory urogram (IVP) shows severe prune belly uropathy. B, IVP after satisfactory reconstitution of urinary tract using ureters proximal to cutaneous ureterostomies for reimplantation.

RECONSTRUCTION OF URINARY TRACT IN PRUNE BELLY UROPATHY

Fm. 4. Case 3. A, original and B, followup IVPs after neonatal ureteral tailoring without preliminary diversion

Fm. 5. Case 4. A, original IVP and B, followup retrograde pyelogram after primary neonatal reconstruction

Fm. 6. Results of neonatal orchiopexy. A, case 3. B, case 4

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WOODARD AND PARROTT TABLE

2. Followup data on 10 consecutive patients undergoing extensive ureteral tailoring for prune belly uropathy

Case No. -Pt.

Length of Followup

Serum Creatinine (mg.%)

Excretory Urogram

1-JD 2-'IT 3-IS 4-DM 5-GW 6-JW 7-MB 8-KE 9-DB 10-KH

5 yrs. 2 yrs. 5 yrs. 4½ yrs. 3½yrs. l½yrs. 1 yr. 1 yr. 6mos. 4mos.

0.9 0.8 0.5 0.6 0.8 0.6 0.3 0.4 0.3 0.6

Stable Stable Stable Stable Stable Stable Stable Stable Stable Stable

Voiding Cystourethrogram No reflux No reflux No reflux No reflux No reflux No reflux No reflux Rt. reflux No reflux No reflux

adjunct of neonatal orchiopexy may offer potential for fertility not previously afforded these patients.

10.

REFERENCES

1. Burke, E. C., Shin, M. H. and Kelalis, P. P.: Prune-belly syndrome. Clinical findings and survival. Amer. J. Dis. Child., 117: 668, 1969. 2. Nunn, I. N. and Stephens, F. D.: The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J. Urol., 86: 782, 1961. 3. Osler, W.: Congenital absence of the abdominal musculature, with distended and hypertrophied urinary bladder. Bull. Johns Hopkins Hosp., 12: 331, 1901. 4. Silverman, F. M. and Huang, N.: Congenital absence of the abdominal muscle associated with malformation of the genitourinary and alimentary tracts: report of cases and review of literature. Amer. J. Dis. Child., 80: 91, 1950. 5. Welch, K. J. and Kearney, G. P.: Abdominal musculature deficiency syndrome: prune belly. J. Urol., 111: 693, 1974. 6. Williams, D. I. and Burkholder, G. V.: The prune belly syndrome. J. Urol., 98: 244, 1967. 7. Ives, E. J.: The abdominal muscle deficiency triad syndromeexperience with ten cases. Birth Defects, 10: 127, 1974. 8. Smith, D. W.: Recognizable patterns of human malformation: genetic, embryologic, and clinical aspects. In: Major Problems in Clinical Pediatrics, 2nd ed. Philadelphia: W. B. Saunders Co., vol. 7, p. 5, 1970. 9. Grossman, H., Winchester, P. H. and Waldbaum, R. S.: Syndrome of congenital deficiency of abdominal wall musculature

11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

Testis Rt. Absent Inguinal Scrotal Scrotal Inguinal Abdominal Inguinal Inguinal Scrotal Scrotal

Lt.

Scrotal Scrotal Scrotal Abdominal Scrotal Scrotal Scrotal Scrotal Scrotal Scrotal

Recurrent Bacteriuria No Rare No Occasional Yes Occasional No Rare Yes Yes

and associated genitourinary anomalies (prune belly syndrome). Prog. Pediat. Radio!., 3: 327, 1970. Rogers, L. W. and Ostrow, P. T.: The prune belly syndrome. Report of 20 cases and description of a lethal variant. J. Pediat., 83: 786, 1973. Lattimer, J. K.: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: a report of 22 cases. J. Urol., 79: 343, 1958. Woodard, J. R. and Parrott, T. S.: Orchiopexy in the prune belly syndrome. Submitted for publication, 1977. McGovern, J. H. and Marshall, V. F.: Congenital deficiency of the abdominal musculature and obstructive uropathy. Surg., Gynec. & Obst., 108: 289, 1959. Waldbaum, R. S. and Marshall, V. F.: The prune belly syndrome: a diagnostic therapeutic plan. J. Urol., 103: 668, 1970. Woodard, J. R. and Keats, G.: Ureteral reimplantation: Paquin's procedure after 12 years. J. Urol., 109: 891, 1973. Perlmutter, A. D.: Reduction cystoplasty in prune belly syndrome. J. Urol., 116: 356, 1976. Palmer, J.M. and Tesluk, H.: Ureteral pathology in the prune belly syndrome. J. Urol., 111: 701, 1974. Snyder, H. M., Harrison, N. W., Whitfield, H. N. and Williams, D. I.: Urodynamics in the prune belly syndrome. Brit. J. Urol., 48: 663, 1976. Duckett, J. W., Jr.: The prune-belly syndrome. In: Clinical Pediatric Urology. Edited by P. P. Kelalis and L. R. King. Philadelphia: W. B. Saunders Co., pp. 615-635, 1976. Culp, D. A. and Flocks, R. H.: Congenital absence of abdominal musculature: report of 2 cases. J. Iowa Med. Soc., 44: 155, 1954.