Incontinent Epispadias: Surgical Treatment of 38 Case

Incontinent Epispadias: Surgical Treatment of 38 Case

0022-534 7/88/1403-0577$2.00/0 Vol. 140, September Printed in U.S.A. THE JOURNAL OF UROLOGY Copyright © 1988 by The Williams & Wilkins Co. INCONTIN...

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0022-534 7/88/1403-0577$2.00/0 Vol. 140, September Printed in U.S.A.

THE JOURNAL OF UROLOGY

Copyright © 1988 by The Williams & Wilkins Co.

INCONTINENT EPISPADIAS: SURGICAL TREATMENT OF 38 CASES SAMI ARAP, WILLIAM C. NAHAS, AMILCAR M. GIRON, ROMERO BRUSCHINI ANUAR I. MITRE

AND

From the Uropediatric Unit, Clinica Urologica do Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil

ABSTRACT

We review 38 cases of surgically corrected incontinent epispadias with a followup of 5 months to 18 years. In 20 cases the Leadbetter, in 8 the Tanagho and in 8 the Young-Dees techniques of bladder neck reconstruction were used. Of 3 patients with minimal (15 to 25 ml.) bladder capacity the Arap procedure was performed in 1, while small constriction of the bladder neck to improve the bladder capacity and compliance was done in 2. In 1 of the latter patients a 60 ml. capacity was achieved and a secondary Leadbetter operation provided an excellent result. Continence was attained after the initial operation in 18 patients, followup is too short to determine the result in 3 and 15 did not acquire urinary control. Revision of the bladder neck plasty was performed in 11 patients, which resulted in continence in 4 and partial continence in 2. Among 34 patients with an adequate followup 22 (73.3 per cent) are continent and 8 (26.4 per cent) are incontinent. The results were similar with the 3 techniques. (J. Urol., 140: 577-581, 1988) Epispadias, a minor form of the exstrophy complex, represents a rare anomaly, with an incidence of 1 of 117,604 male and 1 of 481,110 female patients.' Preponderance of the anomaly in male subjects has been verified in several studies. 2 The rarity of cases has allowed for publication of only a few large series with long-term results and there have been few therapeutic innovations in the last 50 years. Surgical treatment is standardized and the techniques of trigonal tubularization1- 7 have been used universally. Some innovations have been proposed8 •9 but they have not gained general acceptance. Historically, the results of reconstructive procedures have been variable and unpredictable. However, a well developed bladder with adequate capacity and musculature is considered a prerequisite for attaining continence. 2 We present our personal experience with 38 cases of incontinent epispadias in which we used 3 basic techniques of bladder neck reconstruction with the anterior bladder wall10 or trigonal flap tubularization.3-6 MATERIALS AND METHODS

A total of 38 children with incontinent epispadias was treated between 1967 and 1984. All patients presented with either penopubic or complete epispadias, except for 3 who presented with transitional forms between bladder exstrophy and epispadias. There were 28 boys and 10 girls 7 months to 15 years old at the beginning of treatment (table 1). Radiological studies were performed routinely in all patients, including excretory urography (IVP) and voiding cystourethrography with the bladder distended through a Foley catheter and a balloon occluding the bladder neck. In 18 patients the bladder capacity was evaluated during that procedure and in 20 others it was measured indirectly through radiological study. The upper urinary tract was normal in 36 patients, 1 presented with unilateral renal agenesis and 1 presented with a unilateral ectopic pelvic kidney. Reflux was detected in 12 (31.5 per cent) of the 38 patients, and it was bilateral in 8 and unilateral in 4. The separation of the pubic bones (pubic diastasis) was measured and compared to the bladder capacity (table 2). Of the 20 patients with pubic diastasis less than 25 mm. only 2 had a relatively small bladder capacity compared to 3 of the 14 patients with diastasis larger than 25 mm. (1 patient is not included in table 2). Accepted for publication November 30, 1987. 577

Thirty-six patients underwent suprapubic reconstruction of the bladder neck. Vesicoplasty was performed either by trigonal bladder tubularization (8 Young-Dees and 20 Leadbetter procedures) or by anterior bladder wall tubularization (8 Tanagho procedures) (figs. 1 and 2). Of the 3 patients with minimal bladder capacity 1 was treated by Arap's procedure 11• 12 and 2 underwent small constriction of the bladder neck to improve urinary retention and, consequently, bladder capacity and tonus. A secondary Leadbetter procedure was performed in 1 of the latter patients. Before 1977 the selection of the operative technique was based on the presence of reflux and bladder size. Thereafter all patients underwent trigonal tubularization. Routinely, the operation was postponed until the child was 3 years old to allow for better characterization of the continence status. In 5 patients the operation was performed before age 3 years because of continuous urinary leakage. Previous operations had been performed at other institutions in 5 patients. Followup ranged from 5 months to 18 years, with an average of 62 months. One child was lost to followup. In most male patients penile plastic reconstruction was deferred until after the anti-incontinence surgery. Penile reconstruction consisted of an initial stage of penile elongation13 ·14 followed 6 or more months later by ureteroplasty. The results of these procedures are the subject of another report. RESULTS

In the evaluation of the results it is important to define precisely such terms as complete continence. Complete urinary control means no leakage at rest or during physical activities and no need for daytime protection. 15 Nocturnal enuresis was not considered a factor in this definition. Continence was achieved in 18 patients after the initial antiincontinence operation, including 15 who maintained an excelTABLE 1.

Pt. Age (yrs.) 1-2 3-4 5-10 >10 Totals

Patient age at initial operation Boys

Girls

Totals

3 12

2 3 3

5 15 14 4

11

2

2

2ii

Tii

38

578

ARAP AND ASSOCIATES TABLE

Initial Bladder Capacity (ml.)

2. Pubic diastasis, bladder capacity and continence Pubic Diastasis i;;25 mm. (13 pts.)

Pubic Diastasis <25 mm. (20 pts.) Continence

Minimal (0-59) Adequate (i;;60)

14

FIG. 1. Trigonal tube (Leadbetter's procedure).

Incontinence

Partial Continence

1

1 1

3

with bilateral ureteral reimplantation

(q .

.

FIG. 2. Anterior bladder wall tube (Tanagho's procedure)

lent result during the initial 12 to 24 months of followup. All of these patients were older than 5 years, except for a 3-yearold boy. The remaining 3 patients acquired urinary control after longer periods and, coincidentally, they were the youngest children in the series (1, 2 and 3 years old). The establishment of continence had no relationship to puberty and in 3 cases continence preceded puberty. Generally continence was preceded by a period of incomplete control during which longer dry periods between micturitions were noted progressively. All children who became continent had been partially continent previously. Continence has not been achieved in 3 patients but followup is short (less than 18 months) and progressively longer dry periods between micturitions have been noted. Although it is too early for a definite evaluation, we believe that these children will be continent within a few months. In 15 cases incontinence persists after 12 to 24 months of followup and the preoperative conditions have not improved. Of these patients 11 underwent revision of the bladder neck plasty or "retubularization", after which 4 became continent, 4

Continence

Incontinence

Partial Continence

7

2 2

2

remained incontinent and 2 attained partial continence (1 after 5 months and 1 after 6 years). The last patient has been reoperated upon recently and followup is inadequate to date. No additional operations have been performed on the remaining 4 patients and they remain incontinent. Three patients presented with extremely small bladder capacities, varying from 14 to 25 ml. An Arap procedure was performed in 1 case, which resulted in partial continence. The other 2 children were treated by small constriction of the bladder neck to improve the bladder capacity. The operation was successful in 1 patient whose bladder developed a 60 ml. capacity and who attained perfect continence after a secondary Leadbetter procedure (fig. 3). (This case is included in the 18 continent patients.) The other child has been operated upon recently and followup is inadequate to date. When analyzed according to surgical technique, Tanagho's procedure resulted in 5 cases of continence and 3 cases of incontinence (table 3). Complications included 3 cases of bladder stones that were removed suprapubically, although they recurred twice in 1 (table 3). Postoperative vesicoureteral reflux was a common finding (bilateral in 5 cases) and unilateral reflux persisted in only 2 patients (fig. 4). Reoperation was difficult and uniformly unsuccessful in 2 cases of incontinence. This fact has discouraged us from use of the procedure routinely, even when there is no reflux. Leadbetter's operation was performed in 20 patients and followup is adequate in 18. Of these patients 12 achieved urinary control, 4 remained incontinent and 2 are partially continent (1 with a short followup). Continence was achieved after the initial operation in 8 cases and after "retubularization" in 4. Four patients remained incontinent even after reoperation. Surgical complications were noted with ureterovesical reimplantation, especially in the early cases of this series. Ureterovesical obstruction (4 cases) and vesicoureteral reflux (5 cases) had to be corrected surgically. Renal scars secondary to these complications developed in 4 patients (4 renal units) (table 3). Eight patients underwent a Young-Dees vesicoplasty, of whom 5 are continent, 1 is incontinent and 2 are clearly achieving continence, although followup is short (table 3). Complications included bilateral vesicoureteral reflux in 4 cases, 1 of which was corrected surgically by the extravesical procedure of Gregoir. 16 The remaining 3 children are being followed conservatively (table 3). Comparison of the results according to bladder volume is analyzed in table 2. Of the 29 patients with an adequate bladder capacity 21 are continent, 5 are incontinent and 3 are partially continent with a short followup, although they are getting progressively better. Of the 5 patients with inadequate bladder capacity 3 are incontinent and 1 is only partially continent after 5 years and 1 reoperation. The fifth patient (not included in table 2) had an extremely prolapsed bladder (15 ml.), for which she underwent initial partial constriction of the bladder neck. After 18 months bladder capacity was 60 ml. and Leadbetter's procedure resulted in continence (fig. 3). The degree of malformation was evaluated by the epispadiac deformity, pubic diastasis and bladder size. The pubic diastasis generally was greater when the bladder capacity was small. The attainment of urinary control was related to the characteristics of the bladder. However, we found no direct relationship between continence and pubic diastasis. The continence rate showed no statistical difference (chi-square = 2.355) between pubic diastasis smaller or larger than 25 mm. (table 2).

INCONTINENT EPXSPADIAS

FIG. 3. C. T. M., 18-month-old girl, with prolapsing bladder and 15 ml. capacity. A, initial IVP shows permanently empty bladder with minimal capacity. Bladder neck constriction was performed in September 1982. B, IVP in March 1983 reveals development of bladder capacity. C, when bladder capacity was 60 ml. Leadbetter procedure was performed. Patient is completely continent with near normal bladder capacity. TABLE

3. Procedure, complications and definitive results Tanagho

No. pts. No. continent (%) No. incontinent (%) Complications (No. pts.)

Leadbetter

Young-Dees Total No.(%)

5 (73)

16 12 (75)

6 5 (83)

30 22 (73.3)

3 (27)

4 (25)

1 (17)

8 (26.7)

8

Bladder stones Ureterovesical (3), tranobstruction (4), reflux sient reflux (3), persist(5) ent reflux

Reflux (4)

(2)

Fm. 4. S. A. S., 5-year-old boy, was operated on in 1971. A, initial voiding cystourethrogram shows wide bladder neck and absence of reflux. B, voiding cystourethrogram after Tanagho procedure reveals anterior bladder wall tube and transient bilateral reflux.

In summary, of the 34 patients with adequate followup 22 (73.3 per cent) are continent (15 boys and 7 girls), 8 (26.4 per cent) are incontinent (6 boys and 2 girls) (table 3), and 4 are partially continent and the final result has not been defined yet. Of the other 4 patients 2 were lost to followup and the results in 2 are not considered owing to alternative treatment (Arap's procedure and bladder neck constriction). There was no significant difference between continence in boys (60 per cent) and girls (77 per cent) (chi-square= 0. 785). DISCUSSION

Patient age for correction of incontinence is a controversial issue, although the general tendency is to operate on children

older than 3 years. Postponing surgery would allow for better evaluation of the preoperative continence status, offer more developed structures to work with and ensure better cooperation to accept essential instructions in toilet training. 15• 1 7.1 8 Reconstruction was performed in 5 of our patients before they were 3 years old. Of these children 3 achieved complete continence, 1 remains incontinent after 1 year and 1 underwent constriction of the bladder neck. As soon as a minimal bladder capacity is attained vesicoplasty will be performed in this last patient. Immediate urinary control should not be expected after early correction of urinary incontinence because of the obvious lack of patient cooperation and maturity, although correction allows for early development of detrusor tonus and enlargement of bladder capacity. It seems to us that patient age at initial surgery depends only on the objective evaluation of preoperative continence. In our 5 patients there were no technical difficulties and the final results were unaffected. Over-all 38 patients were treated. In 2 children followup is short, 2 underwent alternative treatment and 34 underwent tubularization with adequate followup. Definitive evaluation of continence, as discussed previously, in 30 of the 34 patients revealed complete continence in 22 (73.3 per cent) and incontinence in 8 (26.7 per cent) (table 3). These results represent the personal experience of one of us (S. A.), leading us to some subjective considerations. No difference was found between the use of an anterior bladder wall tube or a trigonal tube. Ureteral reimplantation should only be performed when a trigonal tube of adequate length (3 to 4 cm.) is impossible to obtain without it or when vesicoureteral reflux is present preoperatively. However, when Tanagho's procedure failed initially reoperations were difficult and even useless in 2 cases. For these reasons we now prefer trigonal tubularization, leaving the anterior bladder tube for selected cases only. Complete urinary control was obtained within the first 2 years postoperatively but in a few cases it took several years to achieve such control. Factors influencing this evolution seemed to be related to the psychological maturity of the child, and degree of development of the detrusor tonus and bladder compliance. Frequently, urge incontinence was noted, which essentially was related to poor bladder compliance and hyperreflexia in the initial postoperative period (partial continence). Generally complete urinary control was obtained when bladder compliance and control of bladder contraction developed to near normal standards. A urodynamic study to document this evaluation is the subject of another report. Some speculative conclusions have been made in an attempt to explain the late development of urinary continence. The increase in bladder capacity and muscular activity seems to be related to this fact. 2' 15 Achievement of continence at puberty in boys often is credited to maturation of the prostate, 17 al-

580

ARAP AND ASSOCIATES

though similar evolution has been found in female patients. The explanation for female patients that "hormonal factors and increased pelvic muscular development" 15 result in late development of continence is even more speculative. Culp reported pubertal continence in 11 boys and 2 girls.17 When one considers the male preponderance of this pathological condition, this fact cannot strongly suggest that "maturation of the prostate" is the cause of acquiring continence. In other words, more boys could be continent at puberty because there are more boys than girls with epispadias. We believe that the psychological development of the patient and the increase in quality and volume of the bladder are better explanations for this fact. In 3 of our patients continence was achieved late postoperatively but before puberty. In the remaining patients progressive improvement of continence was clearly related to better bladder function in regard to volume, compliance and hyperreflexia. These findings will be discussed in a separate publication. The importance of bladder volume was demonstrated by the achievement of continence in 71 per cent of our patients with favorable bladder volumes compared to only 20 per cent of those with small capacity bladders. The interval between surgery and complete urinary continence could possibly represent the time necessary to obtain adequate bladder function. Previous operations performed elsewhere did not influence our results significantly. Of 6 of our initial failures 4 were corrected by a reoperation, but a second attempt failed in the remaining 2. We believe that any unsuccessful case deserves a repeat attempt at correction. The initial operation allows for improvement of bladder hyperreflexia and compliance. Complications were noted most in the earlier cases of our series. The Leadbetter procedure was associated with ureterovesical obstruction and/or reflux (9 complications in 16 renal units). Scars developed in 4 patients and 4 renal units, illustrating clearly the high morbidity associated with obstruction and/or reflux with this type of surgery. Later cases were less prone to complications (1 reflux in 23 renal units) regardless of whether the Politano-Leadbetter19 or Cohen20 reimplantation procedure was used. Surgeon experience and technical improvement had a role in this incidence. Complications associated with Tanagho's procedure 21 were few, essentially consisting of vesicoureteral reflux (more often transient) and bladder stones. His technique avoids ureteral reimplantation, which is not necessary in cases without reflux. However, protrusion of the newly created bladder neck may be a cause of the bladder stones. Three patients with minimal bladder capacity were treated by alternative methods. In 1 case the Arap procedure was performed without improvement. Creation of constriction at the bladder neck was done in 2 cases, which provided an excellent result. This approach is similar to the technique used for bladder exstrophy in staged reconstruction, 22 and it allows for improvement of detrusor tone, and bladder control and capacity, which are critical factors to obtain a good result after trigonal tubularization. We believe that in every case of minimal bladder capacity an initial attempt should be made to expand bladder volume. Strict adherence to technical details should be observed by an experienced surgeon to achieve better results, and we suggest several aspects that we consider critical for a good result. The pubic symphysis should be split when reconstructing the subsymphyseal urethra. The tube must be of sufficient length (at least 3 to 4 cm.). The mucosal flap should be narrow (10 to 12 mm.) and meticulously sutured. Excess mucosa should be removed and the underlying detrusor should be deep to envelop the newly constructed mucosal tube. The 2 lateral flaps resulting from the 2 parallel trigonal incisions that delineate the trigonal tube should be rotated cranially and incorporated to the bladder to avoid loss of bladder capacity. After the bladder is closed, it must be suspended to the abdominal wall to provide good fixation of the urethra and to avoid its fall upon the

trigonal tube. As observed by others15• 21 • 22 retention often was noted in the immediate postoperative period. Temporary use of intermittent catheterization or dilation resolved the retention. Urinary diversion with its inconveniences should not be performed primarily for the treatment of incontinent epispadias. Comprehension by the patient and family, associated with the surgeon's effort and determination, will propitiate continence in most cases. Urinary diversion should be performed only after failure of reconstructive attempts. REFERENCES

1. Dees, J.E.: Congenital epispadias with incontinence. J. Urol., 62: 513, 1949. 2. Klauber, G. T. and Williams, D. I.: Epispadias with incontinence. J. Urol., 111: 110, 1974. 3. Young, H. H.: A new operation for epispadias. J. Urol., 2: 237, 1918. 4. Young, H. H.: An operation for the cure of incontinence associated with epispadias. J. Urol., 7: 1, 1922. 5. Dees, J.E.: Epispadias with incontinence in the male. Surgery, 12: 621, 1942. 6. Leadbetter, G. W., Jr.: Surgical correction of total urinary incontinence. J. Urol., 91: 261, 1964. 7. Martinez-Pineiro, J. A.: Sistemas funcionales musculo-elasticos del estroma prostatico. Arch. Esp. Urol., 17: 286, 1964. 8. King, L. R. and Wendel, R. M.: A new application for transvaginal plication in the treatment of girls with total urinary incontinence due to epispadias or hypospadias. J. Urol., 102: 778, 1969. 9. Hendren, W. H.: Congenital female epispadias with incontinence. J. Urol., 125: 558, 1981. 10. Tanagho, E. A., Smith, D. R., Meyers, F. H. and Fisher, R.: Mechanism of urinary continence. II. Technique for surgical correction of incontinence. J. Urol., 101: 305, 1969. 11. Arap, S., Giron, A. M. and Menezes de Goes, G.: Initial results of the complete reconstruction of bladder exstrophy. Urol. Clin. N. Amer., 7: 477, 1980. 12. Arap, S. and Giron, A. M.: Surgical reconstruction of bladder exstrophy. World Urology Update Series, vol. 2, lesson 24, 1985. 13. Kelley, J. H. and Eraklis, A. J.: A procedure for lengthening the phallus in boys with exstrophy of the bladder. J. Ped. Surg., 6: 645, 1971. 14. Johnston, J. H. and Kogan, S. J.: The exstrophic anomalies and their surgical reconstruction. Curr. Prob. Surg., p. 1, August 1974. 15. Kramer, S. A. and Kelalis, P. P.: Assessment of urinary continence in epispadias: review of 94 patients. J. Urol., 128: 290, 1982. 16. Arap, S., Abrao, E.G. and Menezes de Goes, G.: Treatment and prevention of complications after extravesical antireflux technique. Eur. Urol., 7: 263, 1981. 17. Culp, 0. S.: Treatment of epispadias with and without urinary incontinence: experience with 46 patients. J. Urol., 109: 120, 1973. 18. Cendron, J. and Melin, Y.: L'epispadias: apropos de cent huit cas. Ann. Ped., 27: 463, 198v. 19. Politano, V. A. and Leadbetter, W. F.: An operative technique for the correction of vesicoureteral reflux. J. Urol., 79: 932, 1958. 20. Cohen, S. J.: Ureterozystoneostomie: eine neue Antireflux technik. Akt. Urol., 6: 1, 1975. 21. Tanagho, E. A.: Bladder neck reconstruction for total urinary incontinence: 10 years of experience. J. Urol., 125: 321, 1981. 22. Jeffs, R. D., Guice, S. L. and Oesch, I.: The factors in successful exstrophy closure. J. Urol., 127: 974, 1982.

EDITORIAL COMMENT This review of a large series of patients with excellent long-term followup makes a significant contribution to the pediatric literature. The authors have stressed several important points that will enhance success in producing urinary continence in patients with epispadias. Timing of surgery. Vesical neck reconstruction should be deferred until children are approximately 3 years old to allow for better evaluation of the preoperative continence status and to allow for acceptance of essential instruction in toilet training, which is critical to obtaining a successful result. Bladder capacity. The authors have demonstrated clearly the relationship between bladder capacity and urinary continence (table 2 in