Experiences with Urinary Undiversion in Children with Neurogenic Bladder

0022-534 7/80/1233-0402$02.00/0

Vol. 123, March Printed in U.S.A.

THE JOURNAL OF UROLOGY

Copyright© 1980 by The Williams & Wilkins Co.

Pediatric Articles EXPERIENCES WITH URINARY UNDIVERSION IN CHILDREN WITH NEUROGENIC BLADDER ALAN D. PERLMUTTER From the Department of Pediatric Urology, Children's Hospital of Michigan and Department of Urology, Wayne State University School of Medicine, Detroit, Michigan

ABSTRACT

Six children have undergone reconstruction of the urinary tract 14 months to 14 years after supravesical diversion for neurogenic bladder dysfunction. Five are continent: 4 by intermittent catheterization and 1 by voiding to completion. One child is just beyond infancy and wets but is not yet on a systematic program. One boy was considered a technical failure despite incontinence because of progressive hydronephrosis from a non-compliant bladder but he subsequently had an augmentation cystoplasty. Urinary undiversion into a neurogenic bladder is an acceptable option as an alternative to ileal conduit revision or for reasons of patient preference, provided bladder storage capacity is adequate at acceptably low resting pressures, without incontinence. Restoration of urinary tract continuity after ileal conduit diversion was first described in 1971 by Hodges and Dretler and their associates. 1• 2 Subsequent reports have emphasized the feasibility of reversing diversions previously considered permanent, with correction of underlying structural defects. 3• 4 There are many patients with supravesical diversion, generally ileal conduit for neurogenic vesical dysfunction, who also might be candidates for urinary reconstruction. Reconstruction in this group requires assurances that the storage function of the bladder is adequate at reasonable pressures, that continence is demonstrable and that a mechanism for bladder emptying is provided. Perlmutter and associates,5 and Kogan and Levitt6 have described methods to evaluate the defunctioned bladder and to improve bladder capacity before a reconstructive procedure. Intermittent catheterization or self-catheterization has been so successful in the management of many children and adults with neurogenic bladder dysfunction that this approach has been adopted widely. 7- 9 Its usefulness makes undiversion in neurogenic bladder disease an acceptable concept. 10 Reports of long-term deterioration after ileal conduit diversion in children11• 12 lend additional support to considerations for elective urinary tract reconstruction. The 6 children who had supravesical diversion for management of neurogenic bladder dysfunction and have undergone urinary tract reconstruction are described herein, including details of preoperative bladder assessment and management in preparation for the definitive procedure. MATERIALS AND METHODS

The 6 children who underwent urinary tract reconstruction were between 14 months and 16 years old (table 1). All had had a previous supravesical diversion for management of neurogenic bladder or its complications: 5 by ileal conduit and 1 (case 2) by bilateral loop cutaneous ureterostomy. All had normal or near normal renal function. Undiversion was offered in case 1 as an alternative to a necessary stomal revision for conduit dysfunction and in case 4 because of progressive, non-obstructive, Accepted for publication June 18, 1979.

hydronephrosis. In the remaining cases social considerations prevailed. PREOPERATIVE EVALUATION AND MANAGEMENT

After renal function studies and excretory urography all patients underwent dynamic, upright, cystography to evaluate bladder size and shape, the status of the bladder base plate and the degree of reflux, if any, into ureteral stumps. In case 1 the child held the fluid load several hours without incontinence until she was relieved by catheterization and this was deemed adequate for undiversion, without additional studies. All subsequent cases underwent a period of cyclic bladder filling through a suprapubic catheter so that continence could be tested during the cycling process. In case 2 it was possible to insert a straight catheter into the bladder through the distal limb of 1 ureter from the loop cutaneous ureterostomy site. In cases 3 through 6 percutaneous cystostomy was done under cystoscopic control (fig. 1, A) because of the small size of the defunctionalized bladder. Once the parents learned the technique of bladder cycling (fig. 1, B) the children were cycled in the evenings at home so that they could attend school. In case 5 cycling failed after several days, as the catheter gradually extruded into the perivesical space during repeated bladder filling and emptying. However, since continence had been demonstrated she did the further cycling by intermittent urethral catheterization, which was done sporadically over a 9-month period until she underwent undiversion. Duration of the cycling program ranged from 1 to 9 months, with the average bladder capacity increasing approximately 3fold from 65 to 220 ml. Oxybutynin, with or without ephedrine or pseudoephedrine, generally was used during the cycling process to minimize detrusor hyperreflexia, to maximally increase detrusor capacity and to provide for bladder neck continence when indicated. When the patients had an adequate bladder capacity a cystometrogram before undiversion assured satisfactorily low filling pressures. Here, too, neuropharmacologic agents were added if needed to suppress detrusor hyperreflexia with the intent of long-term administration. In case 6 the preoperative cystomet-

402

403

UNDIVERSION IN NEUROGENIC BLADDER

rogram was performed early when bladder capacity was still limited but was interpreted as showing adequate compliance. OPERATIVE CONSIDERATIONS

nence (fig. 4, A). After the diversion she did well, with improved upper tract morphology and with no positive urine culture (fig. 4, B). However, 5 years later an IVP demonstrated increasing

In 5 cases it was possible to use a variety of ureteral procedures to avoid interposition of intestine. These included ureteroneocystostomy, ureteroureterostomy and transureteroureterostomy. In 2 cases mild preoperative reflux into undilated ureters was disregarded, since Teele and associates showed that such reflux usually disappears once the ureter and bladder are refunctionalized. 13 CASE REPORTS

Case 3. S. W., an 11-year-old black girl, was born with a sacral myelomeningocele. She underwent ileal conduit diversion when she was 3 years old for lack of urinary control. She tolerated the diversion well and had no symptomatic urinary infections. When she was 10 years old she requested urinary undiversion. A preoperative cystogram showed a small bladder capacity with mild reflux into both ureteral stumps (fig. 2, A). After 3 months of bladder cycling a cystogram showed a bladder capacity of 220 ml. and no reflux (fig. 2, B). Bilateral ureteroureterostomy was uneventful. Preoperative and postoperative excretory urograms (IVPs) were essentially normal (fig. 3). She is totally dry for ~5 hours and empties by intermi:.tent selfcatheterization. She requires no adjunctive neuropharmacological medications. Case 4. E. H., a 9-year-old white girl, was born with a sacral myelomeningocele. She underwent ileal conduit diversion for recurrent pyelonephritis, reflux and overflow urinary incontiTABLE

1 Initial Bladder Capacity

Final Bladder Capacity

(ml.)

(ml.)

Age at Diversion

Age at Reconstruction

20mos.

4 yrs.

75

2-MMcE-F-2½

1 wk.

14 mos.

15

40

1

3-SW-F-11

4 yrs.

10 yrs.

100

300

3

4-EH-F-9

3 yrs.

8 yrs.

60

300

3

5-JS-F-16

2½ yrs.

16 yrs.

90

220

9 (sporadic)

6-WH-M-15

3 yrs.

14 yrs.

60

250

6

Case Age No. -Pt.-Sex- (yrs.) l-ME-F-7

A

FIG. 2. Case 3. A, preoperative cystogram shows small bladder and mild right reflux into ureteral stump. B, cystogram 3 months after bladder cycling shows increased bladder capacity and no reflux.

Duration of Bladder Cycling (mos.) None

FIG. 3. Case 3. A, preoperative IVP. B, postoperative IVP shows normal upper tracts.

B

Manometer IV

r

FIG. 1. A technique of percutaneous suprapubic cystostomy under cystoscopic control into small defunctionalized bladder. Trocar is placed low to avoid anterior peritoneal fold. B, technique of bladder cycling through suprapubic cystostomy. Cycling is under manometric control in hospital but need not be so at home.

404

PERLMUTTER

FIG. 4. Case 4. Serial IVP. A, before diversion shows dilated upper tracts with parenchymal loss. B, improvement in upper tract morphology 2 years after ileal conduit diversion. C, progressive, non-obstructive dilatation with possible increased parenchymal loss 5 years after ileal conduit.

B

FIG. 5. Case 4. A, right ureteroureterostomy and left-to-right transureteroureterostomy. B, improved upper tracts 13 months after undiversion

upper tract dilatation (fig. 4, C). After 3 months of bladder cycling the bladder size increased 5-fold from 60 to 300 ml. The child underwent reconstruction by right-sided ureteroureterostomy and a left-to-right transureteroureterostomy above the level of the ipsilateral anastomosis (fig. 5, A). She stays dry generally for 4 to 5 hours but dampens occasionally at times of emotional stress. She takes oxybutynin and once daily nitrofurantoin. An IVP 6 weeks postoperatively showed prompt visualization with stable upper tract morphology (fig. 5, B). Her mother reports excellent school performance and social relationships.

RESULTS

Five patients generally were dry for ~4 hours: 4 on an intermittent self-catheterization program and 1 with effective voiding using a neuropharmacological regimen (table 2). Variable dampness did occur in 4 cases but all children seemed happy with the result of the undiversion. While case 2, the youngest patient, could be judged a failure because of small bladder volumes and marked wetting the parents have refused adjunctive neuropharmacological treatment because of her age. Case 6 has been the most difficult management problem.

405

UNDIVERSION IN NEUROGENIC BLADDER TABLE

Case No.

Reconstructive Procedure

Complications

2

Neuropharmacologic Agents

Results

Duration of Followup (yrs.)

Lt. ureteroneocystostomy, rt.-tolt. transureteroureterostomy

None

Generally none (occas. ephedrine)

Occasionally damp, intermittent self-catheterization every 4-5 hrs.

3½

2

Bilat. ureteroureterostomy

Early postop. pyelonephritis, 1 episode

None (parents wish to wait)

Wet, intermittent catheterization for small vols.

11/,

3

Bilat. ureteroureterostomy

Early postop. pyelonephritis, episode

None

Dry 5 hrs., intermittent selfcatheterization

1%

4

Rt. ureteroureterostomy, lt.-tort. transureteroureterostomy

None

Oxybutynin

Generally dry 4-5 hrs., intermittent self-catheterization

l'/12

5

Bilat. ureteroureterostomy

None

Oxybutynin, ephedrine

Voids to completion, occasionally damp

%

6

Antireflux implant of tapered ilea! conduit

Prolonged intestinal obstruction, then progressive hydronephrosis, non-compliant bladder

Propantheline, oxybutynin, imipramine (different times)

Generally dry, intermittent selfcatheterization, recent augmentation ileocystoplasty

%

Although he has been continent the postoperative course was complicated by prolonged intestinal obstruction. Progressive hydronephrosis was apparent 2 months postoperatively and was managed by percutaneous nephrostomy. The bladder proved to be inelastic postoperatively, with a low compliance cystometrogram showing a steep and linear pressure increase with filling. With neuropharmacological medications and continued postoperative bladder cycling, protecting the upper tracts by the nephrostomy, bladder capacity increased gradually to 400 ml. but the compliance of the bladder did not improve. Because of excessive bladder pressures at reasonable volumes the boy recently underwent a technically satisfactory augmentation cystoplasty, which at the time of this report is too recent for evaluation. DISCUSSION

Success of urinary tract reconstruction into a neurogenic bladder in this small series confirms that undiversion is a viable option that should be considered whenever there is a malfunctioning ileal conduit, evidence of upper tract deterioration or a strong desire on the patient's part for restoration of urinary tract continuity. In all except case 2 improvement in body image was quite striking and patients' attitudes toward school and social relationships were much more positive. Even in case 6 in which an inelastic bladder postoperatively led to complications requiring augmentation cystoplasty the patient has had a strong motivation to maintain the undiverted state and the ultimate result is likely to be satisfactory. The risk for complications after this kind of operation is apparent. 14 The simplest form of undiversion feasible is most appropriate, with more elaborate and innovative techniques reserved for those with inadequate ureters. 15 Similarly, an augmentation cystoplasty should not be considered unless a period of bladder cycling does not result in a bladder of adequate capacity with low resting pressures, with or without neuropharmacological agents. Marked reduction in bladder capacity is common in children when the bladder has been defunctionalized but this is reversible generally once function has been reestablished.16 However, permanent contracture can occur 17 and undiversion into a neurogenic bladder should not be attempted without preliminary bladder cycling. Bladder cycling is a convenient and easy way to assess bladder potential completely. Although a cystometrogram is essential preoperatively it need not be obtained until a maximum response after bladder preparation and cycling have been achieved, when it will be a more accurate reflection of the postoperative state. Since most children with neurogenic bladder dysfunction from myelodysplasia will require intermittent self-catheterization there is little

need for sphincter electromyography. Demonstration of continence during cycling is an adequate prerequisite for an operation. Not reported in this series are 2 patients in whom bladder cycling failed to provide for an adequate capacity or in whom there was inadequate continence. Augmentation intestinal cystoplasty in association with an anterior urethrovesical suspension is planned for one of these. Other management options that can be applied to patients being considered for urinary tract reconstruction into a neurogenic bladder include a formal urethral lengthening procedure 18 or an implantable urinary sphincter. 19 ADDENDUM

Since this manuscript was submitted for publication evaluation of the augmentation cystoplasty in case 6 has demonstrated satisfactory increase in detrusor capacity and improvement in vesical wall compliance but ileovesical stenosis requiring revision has developed. REFERENCES

1. Hodges, C. V., Lawson, R. K. and Seabaugh, D. R.: Temporary urinary diversion by ilea! conduit. J. Urol., 105: 196, 1971. 2. Dretler, S. P., Skinner, D. G. and Leadbetter, W. F.: Bilateral ureteroureterostomy after ilea! conduit diversion. J. Urol., 105: 365, 1971. 3. Dretler, S. P., Hendren, W. H. and Leadbetter, W. F.: Urinary tract reconstruction following ileal conduit diversion. J. Urol., 109: 217, 1973. 4. Hendren, W. H.: Urinary tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974. 5. Perlmutter, A. D., Gonzales, E. T., Jr. and Christensen, L. C.: The contracted bladder and ureteral diversion-one approach to reconstruction: a case report. J. Urol., 113: 716, 1975. 6. Kogan, S. J. and Levitt, S. B.: Bladder evaluation in pediatric patients before undiversion in previously diverted urinary tracts. J. Urol., 118: 443, 1977. 7. 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. 8. Hilwa, N. and Perlmutter, A. D.: The role of adjunctive drug therapy for intermittent catheterization and self-catheterization in children with vesical dysfunction. J. Urol., 119: 551, 1978. 9. Mulcahy, J. J., James, H. E. and McRoberts, J. W.: Oxybutynin chloride combined with intermittent clean catheterization in the treatment of myelomeningocele patients. J. Urol., 118: 95, 1977. 10. Colodny, A.H.: Evaluation and management of infants and children with neurogenic bladders. Rad. Clin. N. Amer., 15: 71, 1977. 11. Schwartz, G. R. and Jeffs, R. D.: Heal conduit urinary diversion in children: computer analysis of followup from 2 to 16 years. J.

406

PERLMUTTER

Urol., 114: 285, 1975. 12. Shapiro, S. R., Lebowitz, R. and Colodny, A.H.: Fate of90 children with ileal conduit urinary diversion a decade later: analysis of complications, pyelography, renal function and bacteriology. J. Urol., 114: 289, 1975. 13. Teele, R. L., Lebowitz, R. L. and Colodny, A. H.: Reflux into the unused ureter. J. Urol., 115: 310, 1976. 14. Richie, J.P. and Sacks, S. A.: Complications of urinary undiversion. J. Urol., 117: 362, 1977. 15. King, L. R.: Undiversion: when and how? J. Urol., 115: 296, 1976. 16. Firlit, C. F.: Use of defunctionalized bladders in pediatric renal transplantation. J. Urol., 116: 634, 1976. 17. Lome, L. G. and Williams, D. I.: Urinary reconstruction following temporary cutaneous ureterostomy diversion in children. J. U rol., 108: 162, 1972. 18. Leadbetter, G. W., Jr.: Surgical correction of total urinary incontinence. J. Urol., 91: 261, 1964. 19. Sc_ott, F._B., Bradley,"'!· E. and Timm, G. W.: Treatment of urinary mcontmence by an implantable prosthetic urinary sphincter. J. Urol., 112: 75, 1974.

EDITORIAL COMMENT The author has emphasized the feasibility of reconstructing urinary tracts previously diverted for a neurogenic bladder. The procedure provides for improved self-image and a better quality of life for the patient and, often, results in sterilizing a urinary tract that had bacilluria while diverted into a bag. The author has stressed the need to select with great care those patients with diversion for neurogenic bladder who may be suited for undiversion. Some cases can be relatively straightforward, if ureters can be rejoined to re-establish continuity of !he up~er and lower tracts. Others can be complex and time-consuming if the ileal loop must be tunneled and implanted or if augmentation cy~toplasty is required. Since diversion was used so frequently for these children_ for about 20 years, dating from about 1955, there are many older children and young adults who presently are living with bags who deserve re-evaluation for the possibility of undiversion.

W Hardy Hendren Department of Urology Massachusetts General Hospital Boston, Massachusetts