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Reduction Cystoplasty in Prune Belly Syndrome
THE
Vol. 116, September
JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1976 by The Williams & Wilkins Co.
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME ALAND. PERLMUTTER From the Department of Pediatric Urology, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan
ABSTRACT
Reduction cystoplasty is a useful procedure to treat a large, poorly functioning bladder in boys with prune belly syndrome who are candidates for urinary tract reconstruction. The technique includes elliptical resection of all anomalous urachal tissue and sufficient detrusor dome to reduce bladder capacity to average size and to create a spherical detrusor. Improved detrusor function and a satisfactory or excellent result can be expected. The prune belly syndrome is a triad of abdominal muscular deficiency, undescended testes and urinary dilatation, the manifestations of which vary in severity. 1• 2 In some cases associated renal dysplasia and nephron deficiency are incompatible with survival and, in others renal function is normal despite the urinary tract dilatation. Although opinions differ regarding the need for urologic intervention when urinary tract stasis is mild 2 -• various surgical procedures have been described to manage patients with severe dilatation and dysfunc-
tion. 12 Subsequently, several authors recommended partial cystectomy for treatment of the dilated hypotonic bladder from a variety of causes, with most series emphasizing the decompensated bladder of neglected obstruction. 1 s-2° Such cases have had histologic features of variable muscular atrophy and fibrous tissue replacement, especially marked in the most mobile portion of the bladder-the dome. The results of partial cystectomy vary with the primary disorder, with best results noted in the absence of a severe neurologic deficit.
FIG. 1. Case 1. A, cystogram in newborn. B to D, voiding cystogram when child was 4 ½ years old with catheter in vesicostomy. (B, bladder filling, including urachal portion. C, emptying of bladder base during voiding. D, false residual drained from urachal segment.)
tion who have deteriorating renal function, especially if persistent urinary infection complicates the condition.•-• One such procedure, reduction cystoplasty for rehabilitation of a dilated poorly functioning bladder, has been used sporadically in the prune belly syndrome•- 11 but a group of these patients thus treated has not been reported. Reduction cystoplasty is not a new procedure. In 1937 Orr described resection of the bladder vertex for a large bladder with hypotonia complicating long-standing outlet obstruc-
In 1956 Creevy presented 11 cases of partial cystectomy for hypotonic bladder, including a 4-year-old boy with prune belly syndrome who had an excellent result 7 ½ years postoperatively. 10 Herein is described 4 patients with prune belly syndrome treated by reduction cystoplasty, with a discussion of diagnostic features and criteria for the operation. CASE REPORTS
Case 1. M. P. was referred as a neonate. On excretory urography (IVP) the collecting systems appeared only mildly
Accepted for publication February 6, 1976.
356
357
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
malformed. On a cystogram the bladder appeared to be gourd-shaped and attached to the umbilicus, and mild reflux was noted (fig. 1, A). Voiding was poor and incomplete but there was no urethral obstruction. Initially, satisfactory voiding followed periodic bladder massage but by 2 weeks this had failed and a vesicostomy was done because of chronic retention. The child thrived and when he was 4 ½ years old I restudied him to assess his reconstructibility. An IVP showed stable changes. With a balloon catheter in the vesicostomy a voiding cystogram demonstrated excellent voiding through a patent urethra but also paradoxical voiding into the elongated, diverticulum-like, extension of the bladder dome (fig. 1, B to D). On the basis of apparently satisfactory neuromuscular function of the bladder base I performed a simple dome cystectomy (fig. 2). At followup 6 years postoperatively an IVP was stable, and the patient was well and continuing to grow at the predicted rate (fig. 3). He was continent although he did not void normally and required urecholine. Residual urine was variable, usually around 45 to 50 ml. The patient also had asymptomatic bacilluria, which had not cleared with chemotherapy. Creatinine clearance was normal. Comment: Encouraged by this satisfactory, although not ,,,,,
optimal, reconstruction. I have elected to apply a modified technique-an overlapping 3-layer vest over pants closure with double thickness of muscle-to 3 other boys with prune beliy syndrome (fig. 4). Case 2. R. G. was referred for evaluation when he was 7 years old. He had had a suprapubic tube indwelling since he was 2 years old because of chronic urinary retention. An IVP outlined a small dysplastic right kidney and a good functioning left kidney (fig. 5, A). The mid and lower portions of the left ureter were dilated. A cystogram showed a 600 cc, apparently non-contractile, bladder with a large gourd-shaped extension of the dome to the level of the umbilicus and bilateral reflux (fig. 5, B). The urethra could not be visualized but suprapubic pain and widening of the vesical neck occurred with extreme filling. At cystoscopy the prostate was hypoplastic and the posterior urethra was widely patent. A vest over pants reduction cystoplasty was performed to reduce the bladder capacity from 600 to 150 ml., along with a bilateral ureteroneocystostomy and distal left ureteral tapering. Histologic evaluation of the diverticulum-like extension of
\
t
~' MUCOSAL STRIP REMOVED
FIG. 2. Case 1. Reduction cystoplasty with simple closure
~
FIG. 4. Reduction cystoplasty. After excising fusiform strip of mucosa from 1 side, bladder dome is closed in 3 layers, overlapping tissue to provide band of muscle of double thickness.
Fm. 3. Case 1. IVP when child was 10 years old, 6 years after operation
358
PERLMUTTER
Fm. 5. Case 2. A, IVP in 7-year-old patient with indwelling suprapubic tube. B, preoperative cystogram
Fm. 6. Case 2. IVP 2 years postoperatively
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
FIG. 7. Case 3. IVP and percutaneous nephrostogram, outlining iatrogenic meteropelvicjunction obstruction
the apex was similar to the remainder of the resected bladder dome, with hypertrophied muscle fascicles and only a minimal increase of collagen fibers surrounding the muscle bundles in some areas. Variable thickening, edema, chronic inflammation and patchy fibrosis of the lamina propria also were present. The patient voided with a full and steady stream and residual urine was between O and 5 ml. 2 years postoperatively (fig. 6). The urine has remained sterile except for 2 episodes of pyelonephritis. A wisp of reflux on the right side was noted on 1 postoperative cystogram. Case 3. D. M. was seen when he was 4 years old with an indwelling suprapubic tube, a strictured perinea! urethrostomy and progressive left hydronephrosis (fig. 7), which had followed closure of a cutaneous pyelostomy. Transurethral resection of the valves had been attempted through a perinea! urethrostomy and definite valves were not found. A cystogram outlined bilateral reflux and a large, gourd-shaped bladder. I treated the stricture and performed a dismembered left ureteropyeloplasty with upper ureteral tapering. After the latter procedure the patient voided with an intermittent and dribbling stream whenever the suprapubic tube was clamped and residual urine volumes were 40 to 50 ml. (fig. 8, A). Nine months later he underwent vest over pants reduction cystoplasty, with bilateral ureteroneocystostomy and left distal ureteral tapering. He had a bladder capacity of 225 ml. and voided with variable but usually negligible residual volume 14 months postoperatively, although he required a timed voiding program and double voiding to maintain satisfactory emptying (fig. 8, B). He has maintained a sterile urine on chemotherapy. Case 4. D. B. was seen when he was 15 months old with bilateral nephrostomy, which had been inserted in the neonatal period (fig. 9, A). The patient had a sickly appearance from active urinary sepsis and decreased renal function. Initially, I performed loop cutaneous ureterostomies and removed the nephrostomy tubes. He did remarkably well after these procedures and the blood urea nitrogen returned to normal, and serum creatinine dropped from 1.4 to 1.0 mg. per cent. A cystogram when the boy was 21 months old outlined a gourd-shaped bladder, with a diverticular extension of the apex and massive bilateral reflux (fig. 9, B). Although the catheterized residual volume was most of the fluid freely refluxed out the ureterostomies and no effective urethral voiding occurred. The patient underv;rent \lest over pants reduction \¾'ith
ureteral taperbilateral ureteral reimplantation and lower mg. When he was 25 months old studies confirmed good vesical function so he underwent closure of the loop ureterostomies bilateral ureteropyelostomy and upper ureteral tapering. He voided with a good stream, without residual, and had developed normal urinary continence 11 months postoperatively (fig. 10). The urine was sterile. DISCUSSION
The etiology of tract dilation in with the prune belly syndrome is not certain but is probably developmental. A developmental insult in the 6 to 10-week period of gestation has been proposed to explain the multisystem involvement 1 and electron microscopic studies of the abdominal wall musculature are consistent with this thesis. 21 • 22 The urinary abnormalities vary in severity. The bladder is large and irregular and the ureters are dilated. Bizarre, segmental variations in ureteral dilation are not uncommon. The ureteral changes are not always proportional to the renal changes. Prognosis is more dependent upon the degrees of renal dysplasia and insufficiency and the effects of secondary infection. 1 • 2 In the 4 cases presented each patient had good renal function and the ureteral involvement, although varied, was not extreme. Thus, a major effort could be directed toward correcting the bladder dysfunction. Each of the 4 patients had an abnormally large, gourdshaped bladder, characteristic of the prune belly syndrome. The cause of this malformation, while not proved, is likely to be developmental. The tubular elongation of the detrusor apex, which attaches to the umbilicus, suggests a failure of regression of the urachal component of the primitive bladder. The cause of the vesical dysfunction likewise is undetermined. There may be a neurogenic component. Cystometrograms were not done in these cases but vesical sensation was uniformly present, although possibly reduced in 2 Pressure flow voiding studies reported Nunn and Stephens, 1 and by Williams and Burkholder, 2 rule out an obstructive component for the majority of these children. Only a minority of the most afflicted have urethral obstruction or atresia. 23 In the absence of a more faH into a
360
PERLMUTTER
Fm. 8. Case 3. A, pre-reconstruction cystogram after treatment of stricture. B, IVP 14 months postoperatively
While segmental muscular deficiency and fibrosis are significant features of the megaureters in the prune belly syndrome, 2 • 24 these changes are not as prominent in the prune belly bladder. 25 The bladders of these 4 boys all had histologic changes of diffuse muscle hypertrophy, no gross areas of segmental fibrous replacement of muscle and with only minimal, patchy, thickening of the fibrous septae surrounding muscle bundles. If the unusual shape of the bladder dome represents a persistent urachus, then perhaps this contributes to inefficient voiding. The innervation of the urachal area, as compared to the bladder base, has not been described but may be reduced as suggested by the first case in which there was paradoxic voiding from the body of the blander into the urachal remnant, which acted as a diverticulum. Since this phenomenon was not reproduced in the next 3 patients other factors may account for poor voiding. Voiding is most efficient when the bladder is round and the unusual shape may result in decreased detrusor efficiency. Reduction cystoplasty may achieve its benefit by an
improvement in detrusor shape or by a reduction in bladder volume. Laplace's law supports a favorable effect of a reduced bladder volume on detrusor function (fig. 11). 26 If the enlarged bladder is represented as a sphere and a portion is excised to reduce its size, and if one also assumes the same degree of muscle stretch or unit equilibrium length at maximum filling, then the unit mural tension will be the same. Under these conditions, reducing the radius by 50 per cent will double the intraluminal pressure. The relationship of pressure change to volume change is an exponential one, the pressure being inversely proportional to the cube root of the volume. Therefore, decreasing the volume by half will increase the pressure by approximately 25 per cent. Pressure changes of these magnitudes may be adequate to increase the effectiveness of voiding or to enhance the opening of the vesical neck at the initiation of voiding. The technique of reduction cystoplasty by elliptical excision of the bladder dome is simple. Binard and Zoedler have described in detail the overlapping vest over pants 3-layer
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
361
FIG. 9. Case 4. A, bilateral nephrostogram in 15-month-old patient. B, lateral cystogram when patient was 21 months old shows apical extension of bladder and bilateral reflux.
FIG. 10. Case 4. A, IVP 11 months after cystoplasty and bilateral ureteroneocystostomy with right ureteral tapering. B, cystogram 11 months postoperatively.
362
PERLMUTTER p
:=
RT
2
R= I
IF T's ARE EQUAL,
P
p = 2
= I
FIG. 11. Laplace's law with diagrammatic representation of inverse relationship between radius (R) and pressure (P) for spheres of 2 sizes, assuming equal unit mural tensions (7) for each sphere.
closure. 20 While there are no data to support that a double thickness of bladder muscle in the mobile portion of the bladder improves detrusor contractility after partial cystectomy as compared to simple closure, the results in 3 cases so treated justify continued application of this easy modification. Electron microscopic examinations and in vitro tension, stretch and stimulus studies of the excised bladder smooth muscle may in the future contribute to a better understanding of the dysfunction of the large, irregular bladder of the prune belly syndrome. ADDENDUM
Since submission of this paper 2 other boys with prune belly syndrome have undergone reduction cystoplasty. Both are improved, although 1 requires urecholine and phenoxybenzamine for effective voiding.
8. Waldbaum, R. S. and Marshall, V. F.: The prune belly syndrome: a diagnostic therapeutic plan. J. Urol., 103: 668, 1970. 9. Welch, K. J. and Kearney, G. P.: Abdominal musculature deficiency syndrome: prune belly. J. Urol., 111: 693, 1974. 10. Creevy, C. D.: Partial cystectomy for the hypotonic bladder: report of eleven cases. J. Urol., 75: 456, 1956. 11. Hendren, W. H.: Restoration of function in the severely decompensated ureter. In: Problems in Pediatric Urology. Edited by J. H. Johnston and R. J. Scholtmeijer. Amsterdam: Excerpta Medica, pp. 1-56, 1972. 12. Orr, L. M.: Management of a tonic bladder due to obstruction of vesical neck. South. Med. J., 30: 519, 1937. 13. Crabtree, E. G. and Muellner, S. R.: The bladder in prostatism: an operation for excessive bladder hypertrophy. J. Urol., 60: 593, 1948. 14. Fish, G. W.: Surgery of the dilated bladder. J. Urol., 63: 802, 1950. 15. Houtappel, H. C. E.: Subtotal cystectomy for atonic bladder. Brit. J. Urol., 24: 222, 1952. 16. Fitzpatrick, R. J., Orr, L. M., Hayward, J. C. and Glanton, J. B.: Subtotal cystectomy for atonic bladder. J. Urol., 68: 206, 1952. 17. Gotzen, F. J.: Die subtotale Cystektomie zur Behandlung der Atonie der Harnblase. Z. Urol., 48: 577, 1955. 18. Jonsson, G.: Subtotal cystectomy for atonic bladder. Acta Chir. Scand., 112: 51, 1956. 19. Weinberg, S. R., Tanenbaum, B. and Bertoni, G.: Hypotonic bladder: treatment by partial cystectomy. Urology, 3: 43, 1974. 20. Binard, J. E. and Zoedler, D.: Treatment of the hypotonic, decompensated urinary bladder. Int. Surg., 50: 502, 1968. 21. Afifi, A. K., Rebeiz, J., Mire, J., Andonian, S. J. and Der Kaloustian, V. M.: The myopathology of the prune belly syndrome. J. Neurol. Sci., 15: 153, 1972. 22. Mininberg, D. T., Montoya, F., Okada, K., Galioto, F. and Presutti, R.: Subcellular muscle studies in the prune belly syndrome. J. Urol., 109: 524, 1973. 23. 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. 24. Palmer, J. M. and Tesluk, H.: Ureteral pathology in the prune belly syndrome. J. Urol., 111: 701, 1974. 25. Burkholder, G. V., Harper, R. C. and Beach, P. D.: Congenital absence of the abdominal muscles: a clinicopathologic correlation. Amer. J. Clin. Path., 53: 602, 1970. 26. Ruch, T. C.: The urinary bladder. In: Physiology and Biophysics. Edited by T. C. Ruch and H. D. Patton. Philadelphia: W. B. Saunders Co., pp. 1010-1021, 1966.
REFERENCES
1. Nunn, I. N. and Stephens, F. D.: The triad syndrome: a composite
2.
3.
4. 5.
6.
7.
anomaly of the abdominal wall, urinary system and testes. J. Urol., 86: 782, 1961. Williams, D. I. and Burkholder, G. V.: The prune belly syndrome. J. Urol., 98: 244, 1967. Burke, E. C., Shin, M. H. and Kelalis, P. P.: Prune-belly syndrome. Clinical findings and survival. Amer. J. Dis. Child., 117: 668, 1969. Carter, T. C., Tomskey, G. C. and Ozog, L. S.: Prune-belly syndrome: review of ten cases. Urology, 3: 279, 1974. Lattimer, J. K.: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: a report of 22 cases. J. Urol., 79: 343, 1958. McGovern, J. H. and Marshall, V. F.: Congenital deficiency of abdominal musculature and obstructive uropathy. Surg., Gynec. & Obst., 108: 289, 1959. Spence, H. M. and Allen, T.: Congenital absence of abdominal musculature: urologic aspects. J.A.M.A., 187: 814, 1964.
COMMENT As a general thesis I do not believe that most patients with prune belly syndrome need surgical intervention, despite some rather dilated urinary tracts on x-ray. Proper urinary antisepsis will usually suffice to maintain a stable course. All 4 cases described in this article had prior surgery and certainly reduction cystoplasty had a place in their reconstructions. However, as a primary procedure, I doubt that "domectomies" will be needed very often in the management of prune belly syndrome. J. W.D. REPLY BY AUTHOR I agree with the editorial caution that surgery is not generally needed in the management of prune belly syndrome. However, diversion is still occasionally done for the infant with intractable infection and this article demonstrates the feasibility of later reconstruction in selected patients. In addition, reduction cystoplasty should be considered, as based on the physiologic principles described, for the unoperated case with chronic retention or high residuals.
Vol. 116, September
JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1976 by The Williams & Wilkins Co.
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME ALAND. PERLMUTTER From the Department of Pediatric Urology, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan
ABSTRACT
Reduction cystoplasty is a useful procedure to treat a large, poorly functioning bladder in boys with prune belly syndrome who are candidates for urinary tract reconstruction. The technique includes elliptical resection of all anomalous urachal tissue and sufficient detrusor dome to reduce bladder capacity to average size and to create a spherical detrusor. Improved detrusor function and a satisfactory or excellent result can be expected. The prune belly syndrome is a triad of abdominal muscular deficiency, undescended testes and urinary dilatation, the manifestations of which vary in severity. 1• 2 In some cases associated renal dysplasia and nephron deficiency are incompatible with survival and, in others renal function is normal despite the urinary tract dilatation. Although opinions differ regarding the need for urologic intervention when urinary tract stasis is mild 2 -• various surgical procedures have been described to manage patients with severe dilatation and dysfunc-
tion. 12 Subsequently, several authors recommended partial cystectomy for treatment of the dilated hypotonic bladder from a variety of causes, with most series emphasizing the decompensated bladder of neglected obstruction. 1 s-2° Such cases have had histologic features of variable muscular atrophy and fibrous tissue replacement, especially marked in the most mobile portion of the bladder-the dome. The results of partial cystectomy vary with the primary disorder, with best results noted in the absence of a severe neurologic deficit.
FIG. 1. Case 1. A, cystogram in newborn. B to D, voiding cystogram when child was 4 ½ years old with catheter in vesicostomy. (B, bladder filling, including urachal portion. C, emptying of bladder base during voiding. D, false residual drained from urachal segment.)
tion who have deteriorating renal function, especially if persistent urinary infection complicates the condition.•-• One such procedure, reduction cystoplasty for rehabilitation of a dilated poorly functioning bladder, has been used sporadically in the prune belly syndrome•- 11 but a group of these patients thus treated has not been reported. Reduction cystoplasty is not a new procedure. In 1937 Orr described resection of the bladder vertex for a large bladder with hypotonia complicating long-standing outlet obstruc-
In 1956 Creevy presented 11 cases of partial cystectomy for hypotonic bladder, including a 4-year-old boy with prune belly syndrome who had an excellent result 7 ½ years postoperatively. 10 Herein is described 4 patients with prune belly syndrome treated by reduction cystoplasty, with a discussion of diagnostic features and criteria for the operation. CASE REPORTS
Case 1. M. P. was referred as a neonate. On excretory urography (IVP) the collecting systems appeared only mildly
Accepted for publication February 6, 1976.
356
357
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
malformed. On a cystogram the bladder appeared to be gourd-shaped and attached to the umbilicus, and mild reflux was noted (fig. 1, A). Voiding was poor and incomplete but there was no urethral obstruction. Initially, satisfactory voiding followed periodic bladder massage but by 2 weeks this had failed and a vesicostomy was done because of chronic retention. The child thrived and when he was 4 ½ years old I restudied him to assess his reconstructibility. An IVP showed stable changes. With a balloon catheter in the vesicostomy a voiding cystogram demonstrated excellent voiding through a patent urethra but also paradoxical voiding into the elongated, diverticulum-like, extension of the bladder dome (fig. 1, B to D). On the basis of apparently satisfactory neuromuscular function of the bladder base I performed a simple dome cystectomy (fig. 2). At followup 6 years postoperatively an IVP was stable, and the patient was well and continuing to grow at the predicted rate (fig. 3). He was continent although he did not void normally and required urecholine. Residual urine was variable, usually around 45 to 50 ml. The patient also had asymptomatic bacilluria, which had not cleared with chemotherapy. Creatinine clearance was normal. Comment: Encouraged by this satisfactory, although not ,,,,,
optimal, reconstruction. I have elected to apply a modified technique-an overlapping 3-layer vest over pants closure with double thickness of muscle-to 3 other boys with prune beliy syndrome (fig. 4). Case 2. R. G. was referred for evaluation when he was 7 years old. He had had a suprapubic tube indwelling since he was 2 years old because of chronic urinary retention. An IVP outlined a small dysplastic right kidney and a good functioning left kidney (fig. 5, A). The mid and lower portions of the left ureter were dilated. A cystogram showed a 600 cc, apparently non-contractile, bladder with a large gourd-shaped extension of the dome to the level of the umbilicus and bilateral reflux (fig. 5, B). The urethra could not be visualized but suprapubic pain and widening of the vesical neck occurred with extreme filling. At cystoscopy the prostate was hypoplastic and the posterior urethra was widely patent. A vest over pants reduction cystoplasty was performed to reduce the bladder capacity from 600 to 150 ml., along with a bilateral ureteroneocystostomy and distal left ureteral tapering. Histologic evaluation of the diverticulum-like extension of
\
t
~' MUCOSAL STRIP REMOVED
FIG. 2. Case 1. Reduction cystoplasty with simple closure
~
FIG. 4. Reduction cystoplasty. After excising fusiform strip of mucosa from 1 side, bladder dome is closed in 3 layers, overlapping tissue to provide band of muscle of double thickness.
Fm. 3. Case 1. IVP when child was 10 years old, 6 years after operation
358
PERLMUTTER
Fm. 5. Case 2. A, IVP in 7-year-old patient with indwelling suprapubic tube. B, preoperative cystogram
Fm. 6. Case 2. IVP 2 years postoperatively
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
FIG. 7. Case 3. IVP and percutaneous nephrostogram, outlining iatrogenic meteropelvicjunction obstruction
the apex was similar to the remainder of the resected bladder dome, with hypertrophied muscle fascicles and only a minimal increase of collagen fibers surrounding the muscle bundles in some areas. Variable thickening, edema, chronic inflammation and patchy fibrosis of the lamina propria also were present. The patient voided with a full and steady stream and residual urine was between O and 5 ml. 2 years postoperatively (fig. 6). The urine has remained sterile except for 2 episodes of pyelonephritis. A wisp of reflux on the right side was noted on 1 postoperative cystogram. Case 3. D. M. was seen when he was 4 years old with an indwelling suprapubic tube, a strictured perinea! urethrostomy and progressive left hydronephrosis (fig. 7), which had followed closure of a cutaneous pyelostomy. Transurethral resection of the valves had been attempted through a perinea! urethrostomy and definite valves were not found. A cystogram outlined bilateral reflux and a large, gourd-shaped bladder. I treated the stricture and performed a dismembered left ureteropyeloplasty with upper ureteral tapering. After the latter procedure the patient voided with an intermittent and dribbling stream whenever the suprapubic tube was clamped and residual urine volumes were 40 to 50 ml. (fig. 8, A). Nine months later he underwent vest over pants reduction cystoplasty, with bilateral ureteroneocystostomy and left distal ureteral tapering. He had a bladder capacity of 225 ml. and voided with variable but usually negligible residual volume 14 months postoperatively, although he required a timed voiding program and double voiding to maintain satisfactory emptying (fig. 8, B). He has maintained a sterile urine on chemotherapy. Case 4. D. B. was seen when he was 15 months old with bilateral nephrostomy, which had been inserted in the neonatal period (fig. 9, A). The patient had a sickly appearance from active urinary sepsis and decreased renal function. Initially, I performed loop cutaneous ureterostomies and removed the nephrostomy tubes. He did remarkably well after these procedures and the blood urea nitrogen returned to normal, and serum creatinine dropped from 1.4 to 1.0 mg. per cent. A cystogram when the boy was 21 months old outlined a gourd-shaped bladder, with a diverticular extension of the apex and massive bilateral reflux (fig. 9, B). Although the catheterized residual volume was most of the fluid freely refluxed out the ureterostomies and no effective urethral voiding occurred. The patient underv;rent \lest over pants reduction \¾'ith
ureteral taperbilateral ureteral reimplantation and lower mg. When he was 25 months old studies confirmed good vesical function so he underwent closure of the loop ureterostomies bilateral ureteropyelostomy and upper ureteral tapering. He voided with a good stream, without residual, and had developed normal urinary continence 11 months postoperatively (fig. 10). The urine was sterile. DISCUSSION
The etiology of tract dilation in with the prune belly syndrome is not certain but is probably developmental. A developmental insult in the 6 to 10-week period of gestation has been proposed to explain the multisystem involvement 1 and electron microscopic studies of the abdominal wall musculature are consistent with this thesis. 21 • 22 The urinary abnormalities vary in severity. The bladder is large and irregular and the ureters are dilated. Bizarre, segmental variations in ureteral dilation are not uncommon. The ureteral changes are not always proportional to the renal changes. Prognosis is more dependent upon the degrees of renal dysplasia and insufficiency and the effects of secondary infection. 1 • 2 In the 4 cases presented each patient had good renal function and the ureteral involvement, although varied, was not extreme. Thus, a major effort could be directed toward correcting the bladder dysfunction. Each of the 4 patients had an abnormally large, gourdshaped bladder, characteristic of the prune belly syndrome. The cause of this malformation, while not proved, is likely to be developmental. The tubular elongation of the detrusor apex, which attaches to the umbilicus, suggests a failure of regression of the urachal component of the primitive bladder. The cause of the vesical dysfunction likewise is undetermined. There may be a neurogenic component. Cystometrograms were not done in these cases but vesical sensation was uniformly present, although possibly reduced in 2 Pressure flow voiding studies reported Nunn and Stephens, 1 and by Williams and Burkholder, 2 rule out an obstructive component for the majority of these children. Only a minority of the most afflicted have urethral obstruction or atresia. 23 In the absence of a more faH into a
360
PERLMUTTER
Fm. 8. Case 3. A, pre-reconstruction cystogram after treatment of stricture. B, IVP 14 months postoperatively
While segmental muscular deficiency and fibrosis are significant features of the megaureters in the prune belly syndrome, 2 • 24 these changes are not as prominent in the prune belly bladder. 25 The bladders of these 4 boys all had histologic changes of diffuse muscle hypertrophy, no gross areas of segmental fibrous replacement of muscle and with only minimal, patchy, thickening of the fibrous septae surrounding muscle bundles. If the unusual shape of the bladder dome represents a persistent urachus, then perhaps this contributes to inefficient voiding. The innervation of the urachal area, as compared to the bladder base, has not been described but may be reduced as suggested by the first case in which there was paradoxic voiding from the body of the blander into the urachal remnant, which acted as a diverticulum. Since this phenomenon was not reproduced in the next 3 patients other factors may account for poor voiding. Voiding is most efficient when the bladder is round and the unusual shape may result in decreased detrusor efficiency. Reduction cystoplasty may achieve its benefit by an
improvement in detrusor shape or by a reduction in bladder volume. Laplace's law supports a favorable effect of a reduced bladder volume on detrusor function (fig. 11). 26 If the enlarged bladder is represented as a sphere and a portion is excised to reduce its size, and if one also assumes the same degree of muscle stretch or unit equilibrium length at maximum filling, then the unit mural tension will be the same. Under these conditions, reducing the radius by 50 per cent will double the intraluminal pressure. The relationship of pressure change to volume change is an exponential one, the pressure being inversely proportional to the cube root of the volume. Therefore, decreasing the volume by half will increase the pressure by approximately 25 per cent. Pressure changes of these magnitudes may be adequate to increase the effectiveness of voiding or to enhance the opening of the vesical neck at the initiation of voiding. The technique of reduction cystoplasty by elliptical excision of the bladder dome is simple. Binard and Zoedler have described in detail the overlapping vest over pants 3-layer
REDUCTION CYSTOPLASTY IN PRUNE BELLY SYNDROME
361
FIG. 9. Case 4. A, bilateral nephrostogram in 15-month-old patient. B, lateral cystogram when patient was 21 months old shows apical extension of bladder and bilateral reflux.
FIG. 10. Case 4. A, IVP 11 months after cystoplasty and bilateral ureteroneocystostomy with right ureteral tapering. B, cystogram 11 months postoperatively.
362
PERLMUTTER p
:=
RT
2
R= I
IF T's ARE EQUAL,
P
p = 2
= I
FIG. 11. Laplace's law with diagrammatic representation of inverse relationship between radius (R) and pressure (P) for spheres of 2 sizes, assuming equal unit mural tensions (7) for each sphere.
closure. 20 While there are no data to support that a double thickness of bladder muscle in the mobile portion of the bladder improves detrusor contractility after partial cystectomy as compared to simple closure, the results in 3 cases so treated justify continued application of this easy modification. Electron microscopic examinations and in vitro tension, stretch and stimulus studies of the excised bladder smooth muscle may in the future contribute to a better understanding of the dysfunction of the large, irregular bladder of the prune belly syndrome. ADDENDUM
Since submission of this paper 2 other boys with prune belly syndrome have undergone reduction cystoplasty. Both are improved, although 1 requires urecholine and phenoxybenzamine for effective voiding.
8. Waldbaum, R. S. and Marshall, V. F.: The prune belly syndrome: a diagnostic therapeutic plan. J. Urol., 103: 668, 1970. 9. Welch, K. J. and Kearney, G. P.: Abdominal musculature deficiency syndrome: prune belly. J. Urol., 111: 693, 1974. 10. Creevy, C. D.: Partial cystectomy for the hypotonic bladder: report of eleven cases. J. Urol., 75: 456, 1956. 11. Hendren, W. H.: Restoration of function in the severely decompensated ureter. In: Problems in Pediatric Urology. Edited by J. H. Johnston and R. J. Scholtmeijer. Amsterdam: Excerpta Medica, pp. 1-56, 1972. 12. Orr, L. M.: Management of a tonic bladder due to obstruction of vesical neck. South. Med. J., 30: 519, 1937. 13. Crabtree, E. G. and Muellner, S. R.: The bladder in prostatism: an operation for excessive bladder hypertrophy. J. Urol., 60: 593, 1948. 14. Fish, G. W.: Surgery of the dilated bladder. J. Urol., 63: 802, 1950. 15. Houtappel, H. C. E.: Subtotal cystectomy for atonic bladder. Brit. J. Urol., 24: 222, 1952. 16. Fitzpatrick, R. J., Orr, L. M., Hayward, J. C. and Glanton, J. B.: Subtotal cystectomy for atonic bladder. J. Urol., 68: 206, 1952. 17. Gotzen, F. J.: Die subtotale Cystektomie zur Behandlung der Atonie der Harnblase. Z. Urol., 48: 577, 1955. 18. Jonsson, G.: Subtotal cystectomy for atonic bladder. Acta Chir. Scand., 112: 51, 1956. 19. Weinberg, S. R., Tanenbaum, B. and Bertoni, G.: Hypotonic bladder: treatment by partial cystectomy. Urology, 3: 43, 1974. 20. Binard, J. E. and Zoedler, D.: Treatment of the hypotonic, decompensated urinary bladder. Int. Surg., 50: 502, 1968. 21. Afifi, A. K., Rebeiz, J., Mire, J., Andonian, S. J. and Der Kaloustian, V. M.: The myopathology of the prune belly syndrome. J. Neurol. Sci., 15: 153, 1972. 22. Mininberg, D. T., Montoya, F., Okada, K., Galioto, F. and Presutti, R.: Subcellular muscle studies in the prune belly syndrome. J. Urol., 109: 524, 1973. 23. 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. 24. Palmer, J. M. and Tesluk, H.: Ureteral pathology in the prune belly syndrome. J. Urol., 111: 701, 1974. 25. Burkholder, G. V., Harper, R. C. and Beach, P. D.: Congenital absence of the abdominal muscles: a clinicopathologic correlation. Amer. J. Clin. Path., 53: 602, 1970. 26. Ruch, T. C.: The urinary bladder. In: Physiology and Biophysics. Edited by T. C. Ruch and H. D. Patton. Philadelphia: W. B. Saunders Co., pp. 1010-1021, 1966.
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COMMENT As a general thesis I do not believe that most patients with prune belly syndrome need surgical intervention, despite some rather dilated urinary tracts on x-ray. Proper urinary antisepsis will usually suffice to maintain a stable course. All 4 cases described in this article had prior surgery and certainly reduction cystoplasty had a place in their reconstructions. However, as a primary procedure, I doubt that "domectomies" will be needed very often in the management of prune belly syndrome. J. W.D. REPLY BY AUTHOR I agree with the editorial caution that surgery is not generally needed in the management of prune belly syndrome. However, diversion is still occasionally done for the infant with intractable infection and this article demonstrates the feasibility of later reconstruction in selected patients. In addition, reduction cystoplasty should be considered, as based on the physiologic principles described, for the unoperated case with chronic retention or high residuals.