Epispadias and Incontinence: Surgical Treatment of 27 Children

Vol. 94, Dec.

THE JOURNAL OF UROLOGY

Copyright

© 1966 by The Williams & Wilkins Co.

Printed in U.S.A.

EPISPADIAS AND INCONTINENCE: SURGICAL TREATMENT OF 27 CHILDREN GEORGE V. BURKHOLDER

AND

D. INNES WILLIAMS

From the Hospital for Sick Children, Great Ormond Street, London, England

The external repair of epispadias is generally successful; however, correction of incontinence continues to be a technical problem. Young reported the first 2 male cases to be radically cured of incontinence of urine 42 years ago. 1 These patients had epispadias which he corrected by restoration of the internal and external sphincters and vesical neck. Although this procedure is far from perfect, little progress has been made. 2 A step toward finding better surgical techniques would be to analyze achievements in reconstructing the lower urinary tract. Children with severe epispadias are ideal patients for this sort of analysis. They have wide open bladder necks and rudimentary external sphincter structures. Usually they have not undergone previous surgery and are free from neurological disorders. Unlike exstrophy of the bladder, the ureters and bladder muscle and mucosa are normal and the bladder neck is distinct even though widened. This paper is a review of the results of treatment of incontinence secondary to epispadias in 27 children admitted to the Hospital for Sick Children from 1950 to 1963. It gives an indication of the degree of success that can be attained with reconstruction of the lower urinary tract in patients with mechanical incontinence. EMBRYOGENESIS

Epispadias occurs approximately once in 30,000 births or one-fifth as often as exstrophy of the bladder. Boys are afflicted 3 times as often as girls. 3 Sphincter involvement is noted in 90 Accepted for publication March 1, 1965. Read at annual meeting of The Clinical Society of Genito-Urinary Surgeons, Los Angeles, California, February 25-26, 1965. Read at annual meeting of Western Section, American Urological Association, Inc., San Francisco, California, April 26-29, 1965. 1 Young, H. H.: An operation for cure of incontinence associated with epispadias. J. Urol.,

per cent of the girls afflicted, whereas in boys incontinence is always associated with the complete form and varies inconsistently with the less severe forms. In 1952, Patten and Barry proposed a hypothesis on the genesis of epispadias and vesical exstrophy. 4 To summarize, approximately 14 days after fertilization, the cloacal plate is formed, placing the ectodermal and endodermal layers in direct contact with each other. It is a well-established embryological fact that where these 2 layers are in contact with no intervening mesoderm, a rupture is imminent. Between the fifth and eighth week, the urorectal fold descends into the cloaca to separate the future bladder and urethra from the rectum and should emerge below the paired primordia of the genital tubercle. In exstrophy and epispadias, these primordia appear too far caudally; the urorectal fold divides the cloacal orifice at the level of the corpora and the urethral groove appears on the dorsum rather than the ventral position. Normally, the mesoderm begins to grow ventrally to meet in the midline above the urogenital orifice, but this process is defective in these anomalies. The degree of failure of the mesodermal reinforcement determines the extent of the exstrophic defect when the cloacal membrane ruptures. Associated with complete epispadias are a thin-walled bladder with small capacity, a wide bladder neck rapidly narrowing at the level of the verumontanum, prolapse of the bladder mucosa, incomplete formation of the pubic symphysis with an interpubic band anterior to the bladder neck and underpassing urethra. The external sphincter structures form an open arc with ends attached to the separated pubic bones. A boy will have separated corpora while a girl will have a split clitoris and a flattened mons pubis.

7: 1-32, 1922.

2 Martin, D. C., Turner, R. D. and Goodwin, W. E.: The surgical treatment of urinary incontinence; a review of some of the pertinent literature. Urol. Surv., 13: 111-127, 1963. 'Welch, K. J.: Epispadias. In: Pediatric Surgery. Edited by C. D. Benson, W. T. Mustard, M. M. Ravitch, W. H. Snyder, Jr. and K. J. Welch. Chicago: The Year Book Medical Publishers, Inc., 1962, pp. 1082-1089.

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HISTORY OF CORRECTION

Thiersch was the first to improve urinary control in a patient with epispadias. Exerting 4 Patten, B. M. and Barry, A.: The genesis of exstrophy of the bladder and epispadias. Amer. J. Anat., 90: 35, 1952.

EPISPADIAS AND INCONTINENCE: SURGICAL TREATMENT

external pressure on the bladder neck by means of a pad held by a pelvic belt, his patient was able to remain dry. 5 Others attempted lengthening and torsion of the urethra and claimed shortterm improvement. In 1906, Trendelenburg was the first surgeon to narrow the ring around the bladder neck and stabilize the pubes to reduce tension on the repaired urethra. 6 These early attempts failed to produce acceptable results and ureterosigmoidostomy was considered the only effective way of keeping these patients dry. Early peritonitis and late ascending pyelonephritis resulted in great morbidity and mortality even after Stiles' modification of the anastomosis in 1911.7 In 1922, Young reported his 2 cases of radical cure in male patients with epispadias and incontinence.' He increased the occlusive tension by resecting and narrowing the redundant urethra in the bladder neck and prostate and by isolating the external sphincter muscle, sewing it over the junction of the membranous and prostatic urethra. He commented that sphincters which had never come together could ahnost immediately, after plastic approximation, begin to function norn1ally after years of inaction. 8 Millin devised a procedure to treat congenital urinary incontinence in several young girls in whom vaginal procedures were impossible. 9 He achieved urinary control using a transvesical suprapubic reefing of the bladder neck; however, protracted cystitis resulted which forced him to modify his approach. Desiring an entirely extravesical procedure, Millin conceived of a urethral Rling constructed from the abdominal aponeurosis. He and other surgeons have used the sling procedure for both congenital and stress incontinence in fe1nale patients with considerable success. AIM OF SURGlCAL PROCEDURE

The aim of our surgical procedure is to reconstruct the posterior urethra, especially the supra, Thiersch, C.: Ueber die Entstehungsweise und operative Behandlung der Epispadie. Arch. der Heilkunde, 10: 20--35, 1869. 6 Trendelenburg, F.: The treatment of ectopia vesicae. Ann. Surg., 44: 281-289, 1906. 7 Stiles, H. J.: Epispadias in the female and its surgical treatment. Surg., Gynec., & Obst., 13: 127-140, 1911. 8 Young, H. H.: Genital Abnormalities, Hermaphroditism and Related Adrenal Diseases. Baltimore: The Williams & Wilkins Co., 1937, pp. 44o--472. 9 Millin, T.: Retropubic Urinary Surgery. Baltimore: The Williams & Wilkins Co., 1947.

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membranous portion. The logic of this approach is based upon clinical and physiological findings: The external sphincter is not essential for urinary continence but is responsible for the abrupt cut-off of the stream_IO Patients have been able to start and stop their streams on command even though the external sphincters have been paralyzed experimentally." Therefore, reconstruction of this muscle only increases the efficiency of the urethra. Cine studies have shown that patients who have had the proximal half of the posterior urethra removed by prostatectomy remain continent if the supra-mern.branous portion is intact. When this area is damaged, dribbling results and persists if healing fails to restore soft pliable tissue. An operation to cure incontinence which employs the construction of a neo-urethra from simple bladder wall12 is less successful than one which includes the posterior urethra.1 3 • 14 It is not clear whether this is because it is the most dependent portion of the bladder and allows for ideal drainage, because the vascular and nerve supply from the trigone is preserved, as Leadbetter suggests, or because of the inherent histological arrangement of the urethra. It may be all 3 factors, but the histological arrangement is especially important. No circular muscles forming an internal sphincter are found in the posterior urethra. Instead, there are increased elastic fibers and a thick wall of smooth muscle between striated muscle and mucosa extending longitudinally from the bladder to the distal aspect of the triangular ligament. 15 By exerting occlusive tension on the rugosed urethral mucosa, these tissues obliterate the lumen when the bladder is at rest but allow distensibility during micturition. Young's proce10 Caine, M. and Edwards, D.: The peripheral control of micturition; a cineradiographic study. Brit. J. Urol., 30: 34-42, 1958. 11 Lapides, J., Sweet, R. B. and Lewis, L. W.: Role of striated muscle in urination. J. Urol., 77:

247-250, 1957.

12 Lapides, J.: Structure and function of the internal vesical sphincter. J. Urol., 80: 341-353,

1958.

1 3 Michener, F. R., Thompson, I. 1VI. and Ross, G,, Jr.: Urethrovesical tubularization for urinary incontinence. J. Urol., 92: 203-205, 1964. 14 Leadbetter, G. W ., Jr.: Surgical correction of total urinary incontinence. J. Urol., 91: 261-266,

1964.

15 Tanagho, K A.: Accepted for publication in Brit. J. Urol.

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BURKHOLDER AND WILLIAMS

mucosa elevated

FIG. 1

./1(

1l

/J. \': Sides of urethral ribbon ap)'roximated over a #-8 Pr0nc:h rubber tube

FIG. 2

dure is designed to use all the advantages of the posterior urethra but primarily to bring these properties of the elastic tissues into proper relationship with the urethral lumen. DESCRIPTION OF SURGICAL PROCEDURE

Prior to performing the definitive anti-incontinence procedure, penile lengthening was necessary in four of our 17 male patients. Lengthening was required in order to slacken the bow stringing of the urethra between the bladder neck and the upward-pointing, short, stubby penis.

This reverse chordee fixes the urethra, preventing it from freely falling posteriorly to a more favorable position for wrapping with the sphincteric muscles. Of several accepted techniques for penile lengthening, the following was the one we found most suitable. First, a suprapubic catheter was introduced to divert urine from the operative site. At the mid-portion of the urethra, 1.5 cm. distal to the rudimentary verumontanum, a V-shaped incision with its apex toward the glans was made through the mucosa and submucosa (fig. 1). Dissecting off these layers in both directions achieved some length, but the greatest increase was derived from dividing the fibrous attachments suspending the shaft from the interpubic band and the distal pubic rami. Lateral skin flaps along the penile shaft were developed and approximated over the denuded dorsum. Usually 2 dermalon sutures with rubber tubing or beads and stops were sufficient to take tension from the skin margins subsequently approximated with chromic catgut. Often a ventral skin-relaxing incision was necessary. The apex of the proximal urethra was sutured to the overlapping skin. The stays were removed in 10 days and 3 to 6 months were allowed for healing. Our incontinence-correcting procedure was a form of the Young-Dees operation. Through a Pfannenstiel incision, the bladder neck and proximal urethra were dissected as far distally as possible before opening the urethra in order to clarify the surrounding structures. Substantial

.I EPISPADIAS AND INCONTINENCE: SURGICAL TREATMENT

Rudimeritary' sph,ncter muscle wrapped in kitt fashion around urethra

Reapprox,mation of interpubic

band

Fm. 3

musculofibrous bands were defined passing on either side of the urethra to attachments to the posterior ends of the pubic bones. These form the rudimentary external sphincter (fig. 2). Length was preserved by separating the bands next to the bones. Usually an artery was divided as this was done. The bladder was opened and the ureteral orifices were probed to be certain they allowed for the submucous dissection necessary to reconstruct a bladder neck and neourethra. The interpubic band was severed and the urethra was laid open, the skin and subcutaneous tissues being preserved by retracting them inferiorly to avoid a cicatricial contraction pulling the penis to its former position. Mucosal strips were elevated and resected from the sides of the urethra leaving a ribbon about 8 mm. wide attached posteriorly (fig. 2). Thus, normal mucosa with surrounding elastic tissue and muscle was left intact. Development of this ribbon was carried superiorly through the widened bladder neck to a level just below the ureteral orifices. At this point, the mucosa was cut at a right angle and dissected laterally, removing it from the underlying muscle from each side. The sides of the urethral ribbon were approximated over a No. 8 French rubber tube with a running 4-0 atraumatic chromic suture. The bladder and urethral muscle were closed in a second layer. A new vesical outlet and longer, narrower urethra resulted (fig. 2, insert). Fibrosis destroys the properties of distensibility and occlusive tension and results in failure to achieve

677

continence. For this reason, manipulation of these tissues was done with special care. At this juncture, a dorsal skin tube was constructed from the glans to the proximal urethra and buried under the lateral skin flaps (figs. 2 and 3). The rudimentary sphincter muscles were wrapped in kilt fashion around the urethra beneath the bladder and held with catgut simulating a Mayo hernia repair (fig. 3). As the splinting tube was removed from the glans, a silk thread, tied to its end, replaced it and was left indwelling as a guide for dilatation if it became necessary. A small drain was placed in the retropubic space. A suprapubic self-retaining catheter was left in the bladder for 10 days. The hiatus between the pubic bones varied, but ordinarily we did not perform osteotomies or attempt to stabilize the pubis except to reapproximate the interpubic ligament with 3 horizontal mattress sutures of nylon (fig. 3, insert). The sling operation devised by Millin was used either to supplement the Young procedure or by itself when indicated. Sling construction began by cutting 2 parallel 1 cm. wide flaps from the aponeurosis of the external oblique extending across both recti from either side. The flaps were passed lateral to the rectus muscle on the same side, wrapped beneath the urethra and sutured together anteriorly. When abdominal pressure increases, these flaps arising from the muscular pedicles tighten the sling and pull the urethra superiorly. To give counter traction anteriorly, a suture was placed between their point of joining and the interpubic band. The prostate made dissection behind the male urethra difficult and discouraged the use of a sling in most boys. RESULTS

In our series of 10 girls and 17 boys with epispadias and complete incontinence, the average age was 4 years at the tin'le we performed surgery. Patients with obvious neurological deficits as an explanation for incontinence were excluded. Our followup period ranged from 1 to 14 years with an average of 5 years. The results were clear-- patients were or were not continent after surgery. When a patient was able to be dry both day and night, the procedure was considered a success. Anything less than this was deemed a failure--even "improvement."

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BURKHOLDER AND WILLIAMS TABLE

1. Resi,lt of surgical correction:

17 boys and 10 girls Boys

Girls

Operation

Success Failure Success Failure ------ --

Young procedure Sling procedure Combined procedures

6 0 2

2 3 2

8 0 1

1 0 2

------- -

Totals .......

........

8 47%

9 53%

7 70%

3 30%

We found it necessary to allow several years to pass before finally evaluating these procedures. One of our patients achieved ideal urinary control 9 years after corrective surgery. Such a protracted period may be necessary in order for the patient to 1) mature the prostate and verumontanum as Culp believes, 16 2) learn to control the muscles of micturition, 3) enlarge bladder capacity, 4) reduce bladder irritability, or 5) develop atrophic bladder neck and external sphincter musculature to normal strength and bulk. The Young operation was done in all but 3 patients and is our definitive procedure (table 1). Although it has less than a 50 per cent chance of success in boys, it is definitely the best we have to offer them. Used with the sling operation, the results are slightly better. The technical difficulties normally encountered in a sling procedure in male patients prevented us from using it alone but it was very effective in girls. The fact that the sling by itself was successful in 3 girls without a failure and is technically easier to perform in female patients may account for their superior over-all success rate. However, female patients usually have had better results with corrective procedures for incontinence. It was the policy to operate more than once if necessary, but the outcome has not proved very satisfactory, Twelve children required more than 1 procedure, some having had first attempts at other hospitals. We were able to reverse failures in only 3 cases. There is no doubt that untouched cases without previous scarring have a better chance of success. However, some of these recalcitrant cases have more deficiency and anomalous construction initially. These facts, along with the strict criteria for 16

Culp, 0.: Personal communication.

cure, may explain a slightly lower percentage of successes than obtained by combining the results of Dees, Campbell, and Gross and Cresson. A total of 24 patients with varying degrees of incontinence was described; 11 of 15 male patients and 7 of 9 female patients acquired good control.17-19 Continence was achieved in 15 of our 27 patients. Three of these had serious urinary tract complications: chronic infection, calculi, reflux, or upper tract deterioration. Of the 12 children who were classified as failures, two had stress incontinence and two were dry at night and wet during the day. One girl had calculi and a boy had badly infected urine resulting in pyelonephritis. Fistulas developed in the lower abdomen or base of the penis in 3 boys but these were closed easily without sequelae. There were no deaths. Seven cases were considered irreparable and we have resorted to other means of keeping the patients dry: ureterosigmoidostomy, ureterocutaneostomy, vesicostomy, ileal conduit, penile urinal, and two vesico-colic anastomoses. DISCUSSION

Surgical closure of the bladder neck and sphincter structures is a technical dilemma. If the closure is too loose, incontinence persists and, if too tight, severe complications supervene. In 1964 Williams and Savage presented a series of 51 patients with exstrophy in whom closure had been attempted. 20 Only five (1 boy and 4 girls) achieved continence and of these, 2 girls and the boy had upper tract deterioration, retention, stone formation and/or constant urinary infection. Each of these 3 children had reflux. Because only 4 per cent were dry without complication following a technically difficult procedure, primary diversion either by ileal conduit or ureterosigmoidostomy was considered best. Exceptions would include girls with good bladder walls and boys with small defects similar to those seen in complete or grade 3 epispadias. 17 Dees, J. E.: Congenital epispadias with incontinence. J. Urol., 62: 513-522, 1949. 18 Campbell, M.: Epispadias; a report of 15 cases. J. Urol., 67: 988-999, 1952. 19 Gross, R. E. and Cresson, S. L.: Treatment of epispadias; a report of 18 cases. J. Urol., 68: 477--488, 1952. 20 Williams, D. I. and Savage, J. P.: Reconstruction of the exstrophied bladder. Accepted for publication in Brit. J. Surg.

EPISPADIAS AND INCONTINENCE: SURGICAL TREATMENT

In our series, 12 of the 27 children are now dry without complication--an improvement over the outcome of exstrophy of the bladder, although reconstruction of the bladder neck and urethra was the same. It appears that surgical attempts to correct incontinence in these anomalies do result in improvement in inverse proportion to the extent of the anatomical defect. For this reason, we can expect at least a 50 per cent chance of gaining ideal control in a boy with incontinence due to epispadias or mild exstrophy of the bladder. The prognosis is more favorable for girls. The priorities of our surgical goals should be to

679

prevent renal damage and to keep the patient dry. The alternatives to the Young and Millin techniques are not free from renal damage or complication and only with the ureterosigmoid type of diversion will the patient be dry. We believe it is to the patient's advantage to offer him the chance of being normal by means of these 2 techniques, separately or in combination, and hold the alternative procedures in abeyance if they fail. Department of Surgery, Division of Urology, UCLA Center for the Health Sciences, Los Angeles, California (G.V.B.)