Bladder Exstrophy: Evaluation of Factors Leading to Continence With Spontaneous Voiding After Staged Reconstruction

0022-5347/97/1583-1041$03.00/0 WE JOURNAL OF UROLOGY Copyright 0 1997 by A ~ R I C A U N R O ~ I C AS~OCMTION, M. INC.

Vol. lM1,1041-1044, September 1997 M& in U S A .

BLADDER EXSTROPHY: EVALUATION OF FACTORS LEADING TO CONTINENCE WITH SPONTANEOUS VOIDING AFTER STAGED RECONSTRUCTION H . B . LO'M!MA",

Y. MELIN, M. CENDRON, P. LOMBRAIL, P. BEZE-BEYRIE AND J. CENDRON*

From the Department of Pediatric Urology, HSpital Saint Joseph and Department of Social Medicine, H6pitul Robert Lkbd, Park, France, and Section of Urology, Department of Surgery, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampahire

ABSTRACT

Purpose: We performed a long-term retrospective review of patients with bladder emtrophy to evaluate the results of staged surgical reconstruction in regard to urinary continence, spontaneous voiding and preservation of the upper urinary tract. Materials and Methods: We reviewed the charts of 42 boys and 15 girls treated at HGpital St. Joseph for bladder exstrophy between 1965 and 1995. All patients underwent staged repair, including bladder augmentation in 7 (12%) and secondary urinary diversion in 13 (23%). Criteria for good outcome in terms of continence are defined and factors influencing outcomes are reviewed. Results: A total of 38 patients (67%)achieved good or acceptableurinary continence (22 or 39% and 16 or 28%, respectively). Outcome was poor in 19 patients, including 13 (23%) who underwent secondary urinary diversion. Bladder stones, which developed in 13 patients (23%),were the most common long-term complication of bladder exstrophy reconstruction. Ten patients who underwent pelvic osteotomiesultimately had better continence and 9 are dry. Bladder neck reconstruction was performed at a later age overall (mean 10 years). Repeat bladder neck reconstruction was generally associated with poor results.The technique of bladder neck reconstruction did not appear to influence outcome. Conclusions: A carefully planned surgical reconstruction for bladder exstrophy can lead to satisfactory long-term urinary continence in most patients. Factors contributing to successful results include early bladder closure, pelvic osteotomy, adequate bladder neck reconstruction with bladder neck suspension in girls, and a motivated child and family. Alternatives to surgical reconstruction should be discussed. Ultimate predictors of outcome in bladder exstrophy repair are difficult to ascertain. KEYWORDS:bladder, urinary incontinence, urinary diversion, abnormalitiea

During the last 30 years more than 100 patients with bladder exstrophy were treated at Hapital St. Joseph. Management reflected evaluation in the treatment of this important urological malformation. Patients with bladder exstrophy underwent primary urinary diversion via ureterosigmoidosigmoidostomyor ureterosigmoidostomy.1.2 In patients who were not candidates for internal diversion a cutaneous sigmoid or ileal conduit was created. Patients who had a small fibrotic bladder plate were treated in a similar fashion. However, with the high rate of long-term complications reported for internal and external urinary diversion, and in light of the pioneering work of several surgeons in the early 1970s,staged reconstruction of bladder exstrophy was performed.3-7 This approach included primary bladder closure and repair of the anterior wall defect, subsequent bladder neck reconstruction with ureteral reimplantation and genital reconstruction in boys.6.7 The goal of the staged approach for bladder exstrophy repair is to achieve urinary continence with spontaneous voiding and preservation of the upper urinary tract. We retrospectively evaluated our results with staged surgical reconstruction of the lower urinary tract in patients with classic bladder exstrophy. We identified important factors that help achieve urinary continence but do not jeopardize the upper urinary tract.

MATERIALS AND METHODS

More than 100 patients underwent surgery for classicbladder exstrophy at HSpital St. Joseph between 1965 and 1995, including 42 boys and 15 girls who underwent staged urinary reconstruction. We retrospectively reviewed all patient charts. Evaluation and followup of these patients included yearly clinic visits and physical examination with urinalysis. Radiological evaluation included renal and bladder ultrasound, voiding cystourethmgraphyand excretory urography. Urodynamic studies were performed in only 4 cases. Criteria for urinary continence were d e h e d as good- complete continence with dry intervals of at least 3 hours during the day, no more than 1 voiding event at night, spontaneous voiding with no post-void residual and preservation of the upper urinary tract, acceptable- dry intervals of at least 2 hours during the day, variable degrees of stress or nocturnal incontinence, spontaneousvoiding with little or no post-void residual and preservation of the upper urinary tract, and poorpersistent urinary incontinence andor upper urinary tract deterioration. Factors thought to influence the final clinical outcome were evaluated, including gender, and type and timing of bladder closure, such as early or delayed, single or repeated, with or without pelvic osteotomy, bladder neck reconstruction, age, technique and single or repeat procedure. Statistical analysis was done using the chi-square test * Requests for reprints: Service de Cbirurgie Infantile,Hopital St. with p C0.05 considered statistically significant. Fisher's exact test was used for statistical validation. Joseph, Frue Pierre Larouse 75674, Paris,Cedex 14, France. 1041

BLADDER EXSTROPHY AND CONTINENCE AFTER RECONSTRUCTION

1042

All 57 patients underwent staged reconstruction following the classic sequence of bladder closure, bladder neck reconstruction and genital reconstruction. Bladder closure was performed on day 1 of life in 7 patients and during week 1of life in 2, and delayed closure was done at ages 1 month to 13 years (mean 1.7 years) in 48. Ten patients underwent pelvic osteotomy a t bladder closure. Repeat closure was done in 13 patients (23%)and in 5 the bladder was closed 3 times. Bladder neck reconstruction was performed using the classic Young-Dees procedures and 18 underwent the Mollard modification of bladder neck reconstruction.g Nine patients underwent several bladder neck reconstructions, which explains the greater number of these procedures. Mean age a t bladder neck reconstruction was 10 years (range 6.5 to 20). Additional procedures included urethral dilation in 4 cases, bladder neck incision in 3 and enterocystoplasty with detubularized sigmoid or ileum in 7 (12’70).Indications for enterocystoplasty included low bladder capacity, which caused persistent incontinence. Most patients underwent genital and urethral reconstruction after bladder neck repair. Due to the recent popularity of the Cantwell-Ransley epispadias repair, 5 patients underwent genital reconstruction before bladder neck repair with the goal of increasing bladder capacity.1° All patients with a reconstructed lower urinary tract are on a voiding regimen, which was initiated when the patient and family expressed sufficient motivation for achieving continence. RESULTS

At a mean followup of 12 years (range 1.5 to 28) after bladder neck reconstruction using the aforementioned criteria 38 patients (67%)achieved a good or acceptable result (12 or 39% and 16 or 28%, respectively). In the group with acceptable results followup has been relatively brief and continence has been improving with time. One patient is completely dry but performs intermittent catheterization. The most notable complication was bladder stone formation in 13 patients (23%). Results were poor in 19 patients (33%), including 1 with a brief followup and 13 (23%) who underwent secondary urinary diversion. The criteria used for urinary diversion in these cases were failure of bladder reconstruction and upper tract deterioration. Urinary diversion was also staged with initial conversion to a sigmoid conduit and subsequent creation of a sigmoidosigmoidostomy, relying on the anal sphincter for continence after training with retention enemas. Of the 7 patients who underwent bladder augmentation results were good in 2 with spontaneous voiding, acceptable in 3, including 1on intermittent catheterization, and poor in 2. Of these latter 2 patients 1who is completely incontinent refused further treatment. The other patient underwent enterocystoplasty using nondetubularized sigmoid and subsequent diversion with sigmoidosigmoidostomy 2 years later for intractable incontinence. Gender seemed to be a factor in the success of bladder neck reconstruction, because 71% of the boys but only 53% of the girls had a good or acceptable result. However, these differences were not believed to be statistically significant (table 1). In regard to type of bladder closure, in our experience delayed closure was not a negative factor. Repeat closure decreased the success rate slightly but not significantly. Interestingly, of the 10 patients who underwent pelvic osteotomy at initial bladder closure 9 (90%) achieved a good or TABLE1. Continence according to gender No Good (4F) No. Acceptable (R ) All pta. Boys Girl8

22(391 19 I451 3 (20)

16 (28) 11 (26) 5 133)

No. Poor (R I

Total No. Pts.

19 (331 12 (29) 7 (47)

57 42 15

acceptable continence level (5 and 4 patients, respectively) compared to those who underwent closure without osteotomy. The difference was statistically significant ( p <0.08, table 2). Age at bladder neck reconstruction seemed to have an impact on whether continence was achieved (table 3). Patients who underwent bladder neck reconstruction before age 8 years had more difficulty achieving continence, although no significant statistical difference was noted. Overall results were slightly better with the Mollard modification of bladder neck reconstruction than the Young-Dees procedure but this difference was not statistically significant. Five of the 9 patients who achieved acceptable results with the Mollard bladder neck reconstruction had a relatively brief followup and continence appeared to improve with time. Repeat bladder neck reconstruction led to disappointing results in 9 patients of whom 4 had acceptable and 5 had poor results. In the 5 patients who underwent epispadias repair before bladder neck reconstruction bladder capacity increased but there was no difference in time needed to achieve continence. Patients with a motivated family and good support system had better success in achieving continence. DISCUSSION

Bladder exstrophy represents a major malformation of the lower urinary tract, involving the anterior abdominal wall, bony pelvis, pelvic floor musculature, bladder, urethra and genitalia. The incidence is low (1/30,000 live births) and management has evolved significantly in the last 100 years. It is difficult to obtain information on the long-term evaluation of patients with bladder exstrophy in a large series that examines the clinical outcome of urinary continence and preservation of the upper urinary tract. Our study addressed this issue by reviewing the records of a large group of patients treated and followed at a single institution for more than 30 years. The surgical approach to these cases was staged reconstruction, as initially described by Cendron and Mallard: and Jeffs.11 Surgical management of bladder exstrophy has historically varied greatly from urinary diversion to 1-stage reconstruction of the lower urinary tract. Stein e t a1 reviewed their experience with primary urinary diversion for bladder exstrophy,12 but a high rate of long-term complications has led most others to recommend staged surgical reconstruction a s the initial option.4.13 More recently, Fuchs e t a1 reported 1-stage closure with bladder neck reconstruction and encouraging results in a small cohort.14 In staged repair successful bladder closure is the first essential step. Gearhart and Jeffs,15 and Allen e t a116 stressed its importance for a successful outcome. Closure in the neonatal period is advocated but in our experience delayed closure did not affect the ultimate continence rate. Repeat closure after initial prolapse did not have a negative influence on continence in the long term, which confirms other reports.15.17 Although performed in a small number of cases, early pelvic osteotomy appears to contribute significantly to a successful outcome, because it restores pelvic floor anatomy, helps secure bladder closure

TABLE2. Continence outcome according to bladder closure criteria No. Good No. Acceptable No. Poor All pts Age ( mos. I: Older than 1 1 or Younger No. closures:

1 More than 1 No osteotomy Osteotomy

Total No. Pts. 57

20 (431 2 (20)

12 (251 6 (60)

15 (321 6 (60)

47 10

14(36) 8 (44) 17 (36) 5 (50)

13 133) 3 (17) 12 ( 2 6 ) 4 (401

12 131) 7 (39) 18 ( 3 8 ) l(10)

39 18 47 10

BLADDER EXSTROPHY AND CONTINENCE AFTER RECONSTRUCTION

-

TWLE 3 Continence outcome according to bladder neck reconstruction criteria

~~~-

No Good No Acceptable

No Poor

Total No

l%l

tP I

PtS

22 (39)

16 (28)

19133)

57

19 (441 3 (21)

12 (281 4 (291

12(28) 7(501

43 14

18 1451 4 (261

71181 9 (53)

15(381 41241

40 17

22(46)

12(25) 4 (44)

14(38l 5 (56)

48 9

(%I

All pts Age lyrz I Older than 8 8 or Younger Technique Young-Dees Mollard Bladder neck reconstruction Single Repeat~-

0

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dure on patients who are no younger than 7 years old. In addition, the child must be mature enough to perform clean intermittent catheterization, if required. Therefore, bladder neck reconstruction may be delayed until the patient is sufficiently motivated. Repeat bladder neck reconstruction produced disappointing results, since only 4 patients had acceptable and 5 had poor outcomes. This result may be due to increased scarring a t the level of the reconstructed urethra, which may prevent proper continence. Patients who do not achieve good results after bladder neck reconstruction may be candidates for a continent catheterizable stoma.24 In terms of voiding, all of our patients report good bladder fullness sensation and a normal urinary stream but most must apply suprapubic pressure to empty the bladder completely. Our study is limited in that urodynamic evaluation is not available to confirm this information.

I

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and allows excellent reconstruction of the lower anterior abdominal wall, creating a homogeneous pelvic pressure cavity.'",'* However, for a long-term result we agree with Sponseller et a1 on the necessity of fixing the osteotomies for a t least 6 weeks after surgery.'" In our 10 patients who underwent pelvic osteotomies 9 achieved a good result. In the patient with a poor outcome several bladder closure procedures, failed previous attempts at bladder neck reconstruction, and serious social and psychological issues were involved. Our goal regarding bladder neck reconstruction is to have the patient achieve continence with spontaneous voiding. In addition, preservation of the upper urinary tract with low intravesical pressures is paramount. Our results clearly confirm that this outcome is feasible in a large number of patients and others have obtained similar or better res u l t ~ . '19-21 ~, Whatever the technique of bladder neck reconstruction, the most important factor is to create a narrow and straight posterior urethra 2.5 cm. or more long with no stricture or fistula. Therefore, we prefer to wrap the urethra with 1or 2 detrusor muscle flaps. Interestingly, our poor results occurred in girls rather than in boys, which may be explained by the fact that bladder neck suspension was not done at reconstruction. In girls bladder neck suspension has been advocated by Husmann21 and Gearhart"" et a1 to prevent bladder and urethral descensus with an increase in intra-abdominal pressure. We have now modified our approach in girls to incorporate bladder neck suspension. Hollowell et a1 stated that bladder neck reconstruction may not be a satisfactory procedure to achieve continence, since it decreases bladder capacity and may severely damage detrusor function.23 They proposed alternative solutions, such as bladder neck injections with bulking agents or implantation of an artificial sphincter. Given our results, we believe that these solutions need further evaluation and are useful only when bladder capacity is significantly decreased. In such cases bladder augmentation may be needed, which requires clean intermittent catheterization through the urethra or a continent abdominal stoma.I5 Urethral reconstruction and enterocystoplasty can be performed a t the same time to allow catheterization via a continent mechanism, as described by Gearhart and Jeffs.'.' Furthermore, a small capacity bladder may stretch after bladder neck reconstruction, although this may be unpredictable.17 In our retrospective analysis we found that strong family and patient cooperation is essential to achieve continence with spontaneous voiding. Intensive exercises for holding urine and voiding in the postoperative period allow a better outcome, and the patient has a greater chance of success with these exercises if the family is supportive. In our opinion, bladder neck reconstruction can be performed a t any age provided the patient is motivated. Preoperatively we consulted with an experienced behavioral psychologist before performing bladder neck reconstruction to assess child anc1 family motivation better. We presently perform the proce-

CONCLUSIONS

Urinary continence can be achieved with spontaneous voiding, no post-void residual and preservation of the upper urinary tract in patients with bladder exstrophy. Initial adequate bladder closure, even when delayed or repeated, pelvic osteotomy and bladder neck reconstruction performed by an experienced surgeon are essential. In addition, a compliant, large capacity bladder helps achieve urinary continence. When bladder capacity and compliance are poor, bladder augmentation with clean intermittent catheterization is an acceptable alternative but it should be considered only when reconstruction fails and it should not be performed in a patient who is too young. REFERENCES

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