0022-534 7/88/1393-0524$2.00/0 Vol. 139, March Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1988 by The Williams & Wilkins Co.
SURGICAL TREATMENT OF THE INCONTINENT FEMALE PATIENT WITH MYELOMENINGOCELE SHLOMO RAZ, RICHARD M. EHRLICH, ERIC J. ZEIDMAN, ANTONIO ALARCON AND SHEILA McLAUGHLIN From the Division of Urology, University of California, Los Angeles, California
Surgical therapy was required for 42 incontinent female patients with myelomeningocele who had urodynamically documented high pressure bladders. Conservative treatment consisting of cholinolytic and alpha-adrenergic agents, and intermittent self-catheterization had failed. The surgical approach consisted of perivesical denervation (for hyperreflexia), Burch bladder neck suspension, enlargement cystoplasty and ureteral reimplantation when required. Among 33 patients (79 per cent) there was no incontinence on intermittent self-catheterization and 6 (14 per. cent) had improvement with rare urgency or stress incontinence. In 3 patients (7 per cent) sphincteric incompetence required a transvaginal sling procedure. (J. Ural., 139: 524-527, 1988) The treatment of incontinence in a patient with myelomeningocele has changed dramatically in recent years with the advent of intermittent self-catheterization 1- 4 and artificial sphincter implantation. 5 - 7 Despite advances in medical management renal function continues to deteriorate or urinary incontinence remains refractory in some patients and, therefore, an operation is indicated. Our approach to this difficult problem is presented. METHODS AND MATERIALS
We reviewed the records of 42 consecutive female patients with myelomeningocele who had incontinence and a urodynamically proved hyperactive bladder (hyperreflexia or poor compliance). Each case had proved refractory to prolonged, aggressive pharmacological therapy in addition to intermittent selfcatheterization. Patient age ranged from 4 to 25 years, and an operation was performed between 1979 and 1985. Followup ranged from 6 months to 7 years. Preoperative urodynamic evaluation, cystourethroscopy, excretory urography (IVP) and voiding cystourethrography were performed for all patients. The urodynamic evaluation involved water-filling cystometry, simultaneous pressure flow study and pelvic floor electromyography with urethral pressure profilometry in selected cases. These studies placed the 42 patients into 2 groups: 33 (78 per cent) had poorly compliant bladders without uninhibited detrusor contractions and 9 (22 per cent) had hyperreflexic bladders with uninhibited detrusor contractions. Leak pressures above 40 cm. water were found in 23 of 42 patients (55 per cent). The upper tracts were normal in 27 patients (64 per cent), while 15 (36 per cent) demonstrated ureteral dilatation secondary to obstruction or reflux. All patients with dilated upper tracts were in the group with leak pressures above 40 cm. water. The surgical approach consisted of perivesical denervation (in the hyperreflexic group), Burch bladder neck suspension, enlargement cystoplasty and ureteral reimplantation if required. Of the 42 patients 37 (88 per cent) underwent cecocystoplasty and 5 (12 per cent) underwent ileocystoplasty. Initially, the bladder was opened widely in a sagittal fashion to facilitate the anastomosis. In recent years we have created an anterior bladder flap by a U-shaped incision in the posterior wall (fig. 1, A and B). After the distal ileum and ascending colon were freed to the hepatic flexure, bowel isolation and side-to-side staple reanastomosis were performed without tension. Attention then was directed to the isolated segment where the line
of staples on the ascending colon was excised and a running 3zero polyglycolic acid suture was applied adjacent to the line of staples in the ileum. The cecum was detubularized by opening its antimesenteric border, forming a patch whose vascular pedicle was based on the ileocecal artery (fig. 1, C and D). The posterior cecal patch was anastomosed to the bladder flap located anteriorly. When large bowel was unavailable an ileal segment near the ileocecal valve was used. The ileal loop was shaped into an S, detubularized by opening it along the antimesenteric border and transformed into a wide patch by suturing the edges. Either patch then was fixed to the posterior wall of the bladder with 2-zero polyglycolic acid running sutures. A urethral catheter and suprapubic tube were left in place until an antegrade cystogram demonstrated no leakage 10 days postoperatively. Ureteral reimplantation was performed whenever the upper urinary tract was dilated from obstruction or reflux. In cases of mild to moderate dilatation the Goodwin technique was performed to reimplant the ureters directly into the cecum. 8 Severely dilated ureters were reimplanted into the proximal portion of the ileocecal segment and an antireflux mechanism was created by intussusception of the ileocecal valve with 3 rows of staples. The intussusception was reinforced on its exterior with polyglycolic acid mesh and interrupted 3-zero polypropylene sutures. Additional fixation was obtained by placing a row of staples between the wall of the enlarging segment and the wall of the intussuscepted segment. DoubleJ* silicone stents were placed in all patients and they were removed at 3 weeks. At the time of bladder augmentation perivesical denervation was performed in patients with a hyperreflexic bladder. In this procedure the bladder was mobilized completely from all surrounding attachments except for the completely mobilized urethra, ureters and descending branches of the inferior vesical arteries. A delicate and extensive dissection of the bladder base from the vaginal wall was required. A Burch colposuspension was done in all patients. At the level of the bladder neck and mid urethra a pair of No. 1 polyglycolic acid sutures was approximated and sutured to the ipsilateral iliopectineal ligaments. Additional resistance within the urethra was obtained by placing these sutures somewhat closer to the urethra than usual to create urinary retention. Half of the patients underwent the operation between 1982 and 1985. In all patients a voiding cystogram and IVP were performed between 3 and 6 months postoperatively. A complete
Accepted for publication July 10, 1987.
* Medical Engineering Corp., New York, New York.
SURGICAL TREATMENT OF INCONTINENT FEMALE PATIENTS WITH MYELOMENINGOCELE
Fr~. L Operative tech~ique [or cecocystoplas~y. 1, posteriorly placed U incision in bladder. B, v<:ide incision in posterior bladder forming anterior bladder patch. C, 1Solat10n and detubulanzat10n of cecal segment. D, cecum opened along antimesenteric border forming patch.
urodynamic evaluation identical to that described for the preoperative studies was performed 1 year postoperatively (unless symptomatology warranted an earlier evaluation), and either an IVP or ultrasound was done yearly. RESULTS
Results were considered excellent if the patient was on intermittent self-catheterization with no incontinence, greatly improved if the patient was on intermittent self-catheterization with occasional stress or urgency incontinence and a failure if the patient suffered from persistent incontinence. Of 42 patients 33 (78 per cent) had excellent results, 6 (14 per cent) were greatly improved and 3 (7 per cent) were considered failures (see table). All 3 failures were owing to sphincteric incompetence and required a transvaginal sling procedure. 9 All 3 patients are currently continent on self-catheterization. Figure 2 displays a radiograph obtained 6 months postoperatively. No bowel obstruction, peritonitis or bowel fistulas were noted. The bladder-to-bowel anastomosis was widely patent in all patients and required no revisions. Prolonged leakage of urine from the drain (more than 3 weeks) occurred in 2 patients but this resolved spontaneously. There were 3 wound infections that cleared after incision and drainage. Difficulty with selfcatheterization in the first 3 months owing to mucous plugs occurred in 25 per cent of the patients but this resolved completely after 1 year in 40 of 42. One patient had a stone surrounding a staple of the proximal ileum, which later passed spontaneously. Asymptomatic bacteriuria occurred in 60 per cent of the patients and prophylactic antibacterial agents were continued for 3 months postoperatively. Clinical lower urinary tract symptoms developed in 30 per cent of the patients once a year and in 25 per cent symptoms occurred more often than once a year. There were 5 upper tract infections in 3 patients who under-
Results in 42 patients Result Excellent Greatly improved Failures
No incontinence Occasional incontinence Persistent incontinence
No.(%) 33 (79)* 6 (14) 3 (7)
* Of these patients 3 required a transvaginal sling procedure to eliminate incontinence.
went ureteral reimplantation into the bowel. All 5 infections responded to intravenous antibiotics. The upper tract changes were improved or stable as measured by renal function studies, periodic ultrasound and an IVP. Three patients had serum creatinine levels of 1.4 mEq./1. preoperatively, which remained at this level. Two patients required alkalization with sodium bicarbonate for hyperchloremic acidosis. In 13 of 15 patients requiring ureteral reimplantation into the bowel the reflux resolved. In 2 patients the antireflux-reinforced ileocecal valve failed but there were no failures in the reflux mechanism in patients with a direct antireflux reimplantation into the cecum by the Goodwin technique. 8 Both patients remained on oral antibacterial suppression and self-catheterization without upper tract symptoms or deterioration, probably owing to the low pressure system created by the cystoplasty. Temporary ureteral obstruction occurred in 3 patients but this resolved completely within 4 to 6 months. DISCUSSION
Inability to store urine in the female patient with myelomeningocele may be owing to bladder dysfunction and/ or urethral incompetence. Therapeutic alternatives include bladder training, anticholinergic medication, intermittent self-catheterization and electrical stimulation. The majority of the patients will respond to conservative measures but if these measures fail an operation is required and the treatment must be tailored to
RAZ AND ASSOCIATES
FIG. 2. Representative cystogram from patient on intermittent selfcatheterization 6 months postoperatively demonstrates normal shape of bladder with no segment of bowel apparent radiographically.
the source of the incontinence. In this small cohort our goal was not to restore normal voiding but rather to create a low pressure reservoir by cystoplasty and to increase urethral resistance by modified bladder neck suspension. Before 1984 a denervation procedure was performed in conjunction with bladder augmentation to eliminate hyperreflexic bladder contractions. We were concerned with a distinct group of patients, that is girls with myelomeningocele who had hyperactive bladders unresponsive to conservative therapy. All patients in this group primarily had bladder dysfunction. Changes in bladder pressure owing to poor compliance or uninhibited contractions affect the sphincteric unit, making the diagnosis of primary sphincter incompetence difficult. 10- 12 Augmentation cystoplasty with denervation has been shown to increase periods of continence to at least 6 hours in neurogenic vesical dysfunction. 13 When this effort failed and primary sphincter incompetence was shown to exist, alternatives included a-agonist medication, 14' 15 polytetrafluoroethylene (Teflon) injection, 16 artificial sphincter5 - 7 or slings. 17 The artificial sphincter allows for voiding and continence between voidings by a semiautomatic device activated by the patient. Because of the tendency toward outlet obstruction a common requirement before insertion of an artificial sphincter is to perform a flap urethroplasty that renders the patient completely incontinent. The artificial sphincter in this way allows the patient to void with no need for intermittent self-catheterization. Despite this operation many of the patients are incapable of emptying the bladder after insertion of the artificial sphincter and self-catheterization remains necessary. Ischemic atrophy of the urethra may require an alteration in cuff size or pressure balloon. Moreover, mechanical failure is an ever present problem. 18 Another disadvantage noted more recently in long-term followup is a change in urodynamic parameters after artificial sphincter implantation, which has led to silent renal deterioration. 18- 20 These foregoing considerations are especially important in the child and young adult.
Although we have not found this problem in our patients routine followup of the upper tracts is mandatory in any patient undergoing artificial sphincter implantation or a urethral sling procedure. Therefore, we have been reluctant to insert an artificial sphincter in the female myelomeningocele patient. Rather than attempting to establish normal voiding we provide a low pressure reservoir (by pharmacology or cystoplasty), increase urethral resistance (by bladder neck suspension or vaginal sling) 17• 21 and begin intermittent self-catheterization. This leaves the urinary sphincter intact and converts failure to store to failure to empty. This approach must be reserved for patients who have manual dexterity and the ability to reach the urethral area, since intermittent self-catheterization every 4 to 6 hours replaces the normal voiding mechanism. The advantages are apparent immediately, since most patients will be spared a urethral operation (only 3 of 42 patients required sling procedures). Although improving bladder compliance alone often will eliminate sphincter incontinence we chose, nevertheless, to increase urethral resistance by performing bladder neck suspension. Sutures were applied close to the urethra (as in the Marshall-Marchetti operation 22 ) to increase intentionally resistance and urinary retention. Unfortunately, we have been unable to predict preoperatively which patients will have improved urinary continence from cystoplasty and modified suspension of the bladder neck and which will require a subsequent sling procedure. Thus, we perform the sling procedure at a separate setting only after this approach fails. The second operation (when required) is done transvaginally with minimal morbidity. The efficacy of bladder denervation can be debated. From prior clinical and experimental studies we found that perivesical denervation could eliminate hyperreflexic bladder contractions but it often created a poorly compliant bladder. 23 ' 24 In the last 3 years we have performed cystoplasty alone in cases of high pressure, hyperreflexic bladders. This effectively increased the capacity of the bladder and improved bladder compliance so that the success of the operation was not altered. We do not believe that the form of cystoplasty is important to the over-all results. Cecum was used whenever available, although ileum was equally successful when cecum could not reach the bladder secondary to a previous operation, infection or radiation. The only important considerations in choosing the bowel for the construction were that the bowel must first be detubularized to eliminate effective peristaltic contractions, and that the bowel-to-bowel anastomosis must be performed as wide as possible and without tension. Dividing the bladder in its sagittal plane (like a clam) or as a patch proved to be equally effective in preventing the hourglass deformity with its associated poor results. Ureteral reimplantation was performed whenever the upper urinary tract was dilated from obstruction or reflux. Direct bladder ureteral reimplantation in the patient with a neurogenic and hyperactive bladder is associated with• a high failure rate. 25 By performing this reimplantation into the bowel one can bypass the diseased system, thus, increasing the chances for success. It is important to reimplant the ureters in a nonrefluxing fashion to protect the upper tracts and to preserve adequate long-term renal function. Ordinarily, this goal can be obtained by use of the Goodwin technique and reimplantation directly into the cecum. 8 In severely dilated ureters we did not use an antireflux tunnel because of the increased chance for ureteral obstruction. In such cases we elected to reimplant the ureters into the proximal portion of the ileum attached to the cecum and we created an antireflux mechanism by intussuscepting the ileocecal valve. In 2 of 12 patients with severely dilated upper tracts creation of the antireflux ileocecal valve failed to prevent reflux. Improvement in the technique of fixation of the intussuscepted segment with a ring of polyglycolic acid mesh and fixation of the nipple into the wall of the
SURGICAL TREATMENT OF INCONTINENT FEMALE PATIENTS WITH MYELOMENINGOCELE
enlarging segment with staples has obviated this problem during the last 3 years. We have noted no deterioration of the upper tracts. Of 15 patients requiring ureteral reimplantation into bowel 3 suffered pyelonephritis postoperatively, which was not unexpected considering the degree of upper tract involvement and dilatation in these patients. By following the principles outlined one can expect a high rate of success in this group of carefully selected patients. We were able to improve urinary continence substantially in all patients treated, with a subsequent sling procedure required in only 3. There were no major complications and no cutaneous diversions were required. Further followup will be necessary to determine the effect of lifelong catheterization associated with chronic bacteriuria, and the fate of the kidneys and bowel segments involved in this procedure.
11. 12. 13. 14. 15. 16.
1. Lapides, J., Diokno, A. C., Silber, S. J. and Lowe, B. S.: Clean intermittent self-catheterization in the treatment of urinary tract disease. J. Urol., 107: 458, 1972. 2. Lapides, J., Diokno, A. C., Gould, F. R. and Lowe, B. S.: Further observations of self-catheterization. J. Urol., 116: 169, 1976. 3. Guttmann, L. and Frankel, H.: The value of intermittent catheterization in the early management of traumatic paraplegia and tetraplegia. Paraplegia, 4: 63, 1966. 4. Kass, E. J., Koff, S. A. and Diokno, A. C.: Fate of vesicoureteral reflux in children with neuropathic bladders managed by intermittent catheterization. J. Urol., 125: 63, 1981. 5. Gonzales, R. and Sheldon, C. A.: Artificial sphincters in children with neurogenic bladders: long-term results. J. Urol., 128: 1270, 1982. 6. Light, J. K., Flores, F. N. and Scott, F. B.: Use of the AS792 artificial sphincter following urinary undiversion. J. Urol., 129: 548, 1983. 7. Light, J. K. and Scott, F. B.: Total reconstruction of the lower urinary tract using bowel and the artificial urinary sphincter. J. Urol., 131: 953, 1984. 8. Goodwin, W. E.: Ureterosigmoidostomy (open transcolonic uretero-intestinal anastomosis). In: The Craft of Surgery, 2nd ed. Edited by P. Cooper. Boston: Little, Brown & Co., vol. III, part XVIII, chapt. 116, p. 1518, 1971. 9. Raz, S.: Personal communication. 10. Bauer, S. B., Colodny, A. H., Hallet, M., Khoshbin, S. and Retik, A. B.: Urinary undiversion in myelodysplasia: criteria for selec-
17. 18. 19. 20. 21. 22. 23. 24. 25.
tion and predictive value of urodynamic evaluation. J. Urol., 124: 89, 1980. Gonzales, R. and Sidi, A. A.: Preoperative prediction of continence after enterocystoplasty or undiversion in children with neurogenic bladder. J. Urol., 134: 705, 1986. Mitchell, M. E.: The role of bladder augmentation in undiversion. J. Ped. Surg., 16: 790, 1981. Linder, A., Leach, G. E. and Raz, S.: Augmentation cystoplasty in the treatment of neurogenic bladder dysfunction. J. Urol., 129: 491, 1983. Caine, M., Raz, S. and Ziegler, M.: Adrenergic and cholinergic receptors in the human prostate, prostatic capsule, and bladder neck. Brit. J. Urol., 47: 193, 1975. Obrink, A. and Bunne, G.: The effect of alpha-adrenergic stimulation in stress incontinence. Scand. J. Urol. Nephrol., 12: 205, 1978. Carrion, H. M. and Politano, V. A.: Periurethrai polytef (Teflon) injection for urinary incontinence. In: Female Urology. Edited by S. Raz. Philadelphia: W. B. Saunders Co., chapt. 20, pp. 293298, 1983. McGuire, E. J. and Lytton, B.: Pubovaginal sling procedure for stress urinary incontinence. J. Urol., 119: 82, 1978. Scott, F. B., Fishman, I. J. and Shabsigh, R.: The impact of the artificial urinary sphincter in the neurogenic bladder on the upper urinary tracts. J. Urol., 136: 636, 1986. Light, J. K. and Pietro, J.: Alteration in detrusor behavior and the effect on renal function following insertion of the artificial urinary sphincter. J. Urol., 136: 632, 1986. Bauer, S. B., Reda, E. F., Colodny, A.H. and Retik, A. B.: Detrusor instability: a delayed complication in association with the artificial sphincter. J. Urol., 135: 1212, 1986. Hadley, H. R., Zimmern, P. E., Staskin, D.R. and Raz, S.: Transvaginal needle bladder neck suspension. Urol. Clin. N. Amer., 12: 291, 1985. Marchetti, A. A., Marshall, V. R. and Shultis, L. D.: Simple vesicourethral suspension: a survey. Amer. J. Obst. Gynec., 74: 57, 1957. Freiha, F. S. and Stamey, T. A.: Cystolysis: a procedure for the selective denervation of the bladder. J. Urol., 123: 360, 1980. Leach, G. E., Goldman, D. and Raz, S.: Surgical treatment of detrusor hyperreflexia. In: Female Urology. Edited by S. Raz. Philadelphia: W. B. Saunders Co., chapt. 23, pp. 326-334, 1983. Bauer, S. B.: Vesicoureteral reflux in children with neurogenic bladder dysfunction. In: Management of Vesicoureteric Reflux. Edited by J. H. Johnston. Baltimore: The Williams & Wilkins Co., vol. 10, chapt. 15, pp. 159-177, 1984.