THE USE OF VESICOSTOMY AS PERMANENT URINARY DIVERSION IN THE CHILD WITH MYELOMENINGOCELE

0022-5347/01/1666-2351/0 THE JOURNAL OF UROLOGY® Copyright © 2001 by AMERICAN UROLOGICAL ASSOCIATION, INC.®

Vol. 166, 2351–2353, December 2001 Printed in U.S.A.

THE USE OF VESICOSTOMY AS PERMANENT URINARY DIVERSION IN THE CHILD WITH MYELOMENINGOCELE JOEL C. HUTCHESON, CHRISTOPHER S. COOPER, DOUGLAS A. CANNING, STEPHEN A. ZDERIC AND HOWARD M. SNYDER, III From the Division of Pediatric Urology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania

ABSTRACT

Purpose: Treatment of the child with myelomeningocele presents the urologist with many challenges. While vesicostomies are usually used for temporary bladder drainage until continent reconstruction is performed, we have a population of postpubertal patients with myelomeningocele in whom vesicostomies have been preserved. We review our experience with these older children. Materials and Methods: A search of our database of 350 patients with myelodysplasia identified 23 with urinary diversion accomplished by permanent vesicostomy. Those who had undergone vesicostomy after age 11 years were designated as group 1. Those who received a vesicostomy early in life that was maintained post puberty were designated as group 2. Indications for vesicostomy, upper tract status before and after vesicostomy, recurrent infections and complications were noted. Upper tract status was determined by serial ultrasounds during routine followup. Results: Group 1 was comprised of 5 patients with mean age at vesicostomy of 17 years and a mean followup interval of 5.2 years. Group 2 was comprised of 18 patients with mean age of vesicostomy at 2.6 years and mean followup interval of 13 years. Hydronephrosis resolved in all children from both groups. Vesicostomy revision was required in 1 patient secondary to prolapse. Other complications included mild prolapse that did not require revision, stenosis requiring revision 5 months after the initial procedure and an early bilateral ureterovesical obstruction that resolved. Of the patients 33% had recurrent upper tract calculi. Conclusions: While the goal of achieving urinary continence is well worth striving for in the child with myelomeningocele, it is not realistic for all patients. We believe that permanent cutaneous vesicostomy is an acceptable alternative that the reconstructive surgeon should consider. This reliable and simple technique ensures safe decompression of the upper urinary tracts, while avoiding the complications of urinary diversion using segments of bowel. KEY WORDS: bladder, meningomyelocele, cystostomy, urinary diversion

Treatment of the child with meningomyelocele presents the urologist with many challenges. Those children with recurrent urinary tract infections, detrusor instability or noncompliant bladders require urological care. The long-term treatment of these patients must ensure low pressure storage and drainage of urine to protect the kidneys.1 Anticholinergic medications and intermittent catheterization have proved an effective treatment method for many children with neurogenic bladder dysfunction secondary to meningomyelocele. Other children have been effectively treated with augmentation cystoplasty and clean intermittent catheterization.2 However, some patients with meningomyelocele are unable to perform or undergo catheterization and require a method of low pressure emptying. Treatment options in this group include lowering outlet resistance by urethral dilation,3 eliminating sphincter activity, that is botulin toxin or sphincterotomy4 or suprasphincteral urinary diversion via vesicostomy.5 While vesicostomy is usually used for temporary bladder drainage until continent reconstruction is performed, we have a population of postpubertal patients with meningomyelocele in whom vesicostomy has been preserved. We review our experience with these older children. MATERIALS AND METHODS

A search of our database of 350 patients with myelodysplasia identified 23 patients with urinary diversion accomAccepted for publication July 13, 2001.

plished by permanent vesicostomy. Those who had undergone vesicostomy performed after age 11 years were designated as group 1. Those who received a vesicostomy early in life that was maintained post puberty were designated as group 2. Indications for vesicostomy, upper tract status before and after vesicostomy, recurrent infections and complications were noted. Upper tract status was determined by serial ultrasounds during routine followup of these patients.

OPERATIVE TECHNIQUE

The technique used for vesicostomy has been previously described.5–7 Briefly, a 2 cm. midline transverse incision is made midway between the pubic bone and lower edge of the umbilicus. A transverse rectus fascia incision exposes the rectus muscle. The bladder is filled to aid the dissection as the perivesical fascia is incised to expose the detrusor. The dissection is performed extraperitoneally with the anterior surface of the bladder exposed using traction sutures. As blunt dissection is used to push the peritoneum cephalad, the urachus and dome of the bladder are identified. As the bladder dome is pulled through the fascial incision the umbilical vessels and proboscis of the urachus are ligated. The urachus and small portion of the bladder dome are excised, leaving a 24F defect in the thickened bladder wall. It is important to place the vesicostomy in a position such

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that the posterior bladder wall is taut so that the risk of prolapse of the back wall of the bladder through the vesicostomy is reduced. This placement ensures that the vesicostomy will achieve effective bladder decompression. The detrusor is sutured to the fascia 1 cm. below the edge of the cystostomy. Additional sutures may be placed in the edges of the fascial incision to ensure it is no larger than 24F to decrease further the risk of prolapse. An everting stoma is sutured to the skin by a series of interrupted sutures. A catheter is placed through the stoma overnight and the parents are sometimes instructed to dilate it with a soft eyedropper at home for several months. RESULTS

Group 1 was comprised of 5 patients, with a mean age at vesicostomy of 17 years (range 13 to 20) and a mean followup interval of 5.2 years. Group 2 was comprised of 18 patients with mean age at vesicostomy of 2.6 years and mean followup interval of 13.2 years. Indications for vesicostomy included hydronephrosis, recurrent urinary tract infection, vesicoureteral reflux, renal scarring and bladder calculi (table 1). Hydronephrosis resolved in all children from both groups. Vesicostomy was considered to be the method of diversion of choice due to the inability and/or unwillingness to perform clean intermittent catheterization in the majority of these patients. In Group 1, 4 of the 5 patients were unwilling to perform clean intermittent catheterization regularly while the fifth had developmental delay. Of 5 patients 1 used a stoma appliance, while the remainder were maintained in diapers. In Group 2 developmental delay was a major factor in deciding to maintain vesicostomy in 13 patients (72%) and 5 were satisfied with the vesicostomy and did not desire clean intermittent catheterization. Of the 18 patients 2 successfully used stoma appliances while the remainder were dry in diapers. Since vesicostomy, no episodes of febrile urinary tract infection or urolithiasis have occurred in group 1. Vesicostomy revision was required in 1 patient 6 months post-vesicostomy for prolapse (table 2). Only 2 febrile urinary tract infections occurred in group 2. Complications of the vesicostomy in this group included mild prolapse that did not require revision, stenosis requiring revision 5 months after the initial procedure and an early bilateral ureterovesical obstruction that resolved 4 weeks postoperatively (table 2). Of 18 patients 6 (33%) had recurrent upper tract calculi with bladder calculi also developing in 2 of the 6 (table 3). In 2 patients with large stone burdens simple nephrectomy of kidneys with preexistent poor function was performed. Of the 6 patients 5 had thoracic level myelomeningocele and were relatively immobile. Upper tract stones were recurrent and associated with febrile urinary tract infection in 2 patients who ultimately underwent simple nephrectomy. The kidneys of these 2 patients had long-standing poor function. The etiology of the bladder stones was thought to be secondary to upper tract stones that passed into the bladders. Although no stasis was observed on serial ultrasounds of these patients, it is likely that stasis of urine and/or subclinical urinary tract infection propagated the growth of the bladder calculi.

TABLE 1. Indications for vesicostomy No. pts. Mean age at vesicostomy (yrs.) Yrs. followup No. hydronephrosis (%) No. recurrent urinary tract infection (%) No. vesicoureteral reflux (%) No. failed clear intermittent catheterization No. developmental delay

Group 1

Group 2

5 17 5.2 3 (60) 4 (80) 1 (20) 4 1

18 2.6 13.2 15 (83) 10 (56) 7 (39) 2 13

TABLE 2. Complications of vesicostomy No. Group 1

No. Group 2

0 0 1 0

1 1 0 1

Stenosis requiring revision Bilat. ureteral obstruction* Prolapse requiring revision Severe excoriation * Resolved 4 weeks postoperatively.

TABLE 3. Outcomes No. Group 1 (%) No. Group 2 (%) Resolution of hydronephrosis Recurrent pyelonephritis Urolithiasis: Upper tract Lower tract

4 (100) 1 (20) 0 0 0

15 (100) 2 (11) 6 (33) 6 (33) 2 (11)

DISCUSSION

As our understanding of neurogenic bladder dysfunction has increased, the urinary complications of spina bifida have been decreased by strategies aimed at the low pressure storage and emptying of urine.1 If untreated, high pressure neurogenic bladder dysfunction may result in hydroureteronephrosis, vesicoureteral reflux, fibrosis of the bladder wall, recurrent urinary tract infection and ultimately renal failure.2 Urinary diversion by ileal or colonic loop not only required a urostomy, but in long-term followup had a high level of complication and, thus, was unsatisfactory therapy for the high pressure bladder.8 Current management strategies are aimed at ensuring intravesical storage pressures below 40 cm. H2O without cutaneous urinary diversion. Bladder augmentation by intestine currently represents the gold standard for bladder enlargement.2 Once safe storage pressures are ensured, the issue of urinary continence can be considered. Patients with competent bladder necks and large capacity, compliant bladders can achieve continence with intermittent catheterization and, if need be, anticholinergics. However, many patients with meningomyelocele have incompetent bladder necks and will require surgical correction to increase outlet resistance, often in conjunction with bladder augmentation. Although permanent supravesical urinary diversion is considered to be undesirable in the treatment of a high pressure bladder, not all patients are candidates for continent reconstruction. Some patients with meningomyelocele do not possess the mental acuity or lack the social support systems to ensure reliable compliance with intermittent catheterization. Possible options in these patients include indwelling catheterization, sphincterotomy or vesicostomy. Long-term indwelling catheterization poses the risk of chronic infection and squamous cell carcinoma. 9 Sphincterotomy often requires multiple procedures and does not guarantee low pressure emptying.10 Cutaneous vesicostomy has been previously reported as a means of temporary urinary diversion in children manifesting signs of upper tract deterioration secondary to high pressure bladder.9 –19 Temporary vesicostomy was used in 23 of 95 patients (24%) with neurogenic bladder and vesicoureteral reflux at the Hospital for Sick Children in Toronto. In that series 22 patients had successful closure and 50% required no further surgery.17 Mandell et al reported on 10 patients at Boston Children’s Hospital treated with temporary vesicostomy performed between the ages of 1 month and 3 years.18 Patients were left with vesicostomies from 4 months to 22 years. Bladder prolapse was reported in 2 patients and bladder stones in 1. No patient had deterioration of the upper tracts while diverted. No decrease in bladder capacity was observed after vesicostomy closure. In another series from the Children’s Hospital of Philadelphia 48 patients with a neurogenic bladder were treated with temporary vesicos-

VESICOSTOMY FOR URINARY DIVERSION

tomy.22 Vesicostomy was performed because of hydroureteronephrosis in the majority of cases (44 of 48). A total of 16 patients underwent subsequent closure with no resulting loss in bladder volume. Resolution of upper tract changes occurred in all but 1 patient who required vesicoureteral reimplantation for independent ureterovesical junction obstruction (primary obstructive megaureter). In our series we describe 2 groups of patients in whom cutaneous vesicostomy has been used as a form of permanent urinary diversion. Group 1 is comprised of 5 patients who underwent vesicostomy after puberty and for whom conservative treatment had failed. Upper tract deterioration had developed, heralded by recurrent infections and hydronephrosis, and all patients were either unable or unwilling to catheterize. Group 2 is comprised of 18 patients who had vesicostomy placed early in life as a means of urinary diversion and were never believed to be suitable candidates for vesicostomy closure and/or continent reconstruction. Complications related to vesicostomy include prolapse, stomal stenosis and cystolithiasis. Prolapse and stenosis are significant complications and may result in ineffective decompression. There has been 1 reported case of herniation of the bowel secondary to vesicostomy prolapse.20 Prolapse and stenosis are best avoided by ensuring that the most cephalad aspect of the bladder is brought out through a 24F fascial defect.5 The 33% rate of stone formation in group 2 is consistent with those observed in children with spina bifida treated with continent reconstruction.24 Six children had upper tract stones and 2 of these had cystolithiasis as well. We believe that the bladder stones were likely secondary to the upper tract stones. The high stone rates warrant routine surveillance with ultrasound to ensure that the upper tracts are being effectively drained. CONCLUSIONS

While the goal of achieving urinary continence is well worth striving for in the child with myelomeningocele, it is not realistic for all patients. We believe that permanent cutaneous vesicostomy is an acceptable alternative that the reconstructive surgeon should consider. This reliable and simple technique insures safe decompression of the upper urinary tracts while avoiding the complications of urinary diversion using segments of bowel. REFERENCES

1. McGuire, E. J., Woodside, J. R., Borden, T. A. et al: Prognostic value of urodynamic testing in myelodysplastic patients. J Urol, 126: 205, 1981

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2. Sutherland, R. S., Mevorach, R. A., Baskin, L. S. et al: Spinal dysraphism in children: an overview and an approach to prevent complications. Urology, 46: 294, 1995 3. Bloom, D. A., Knechtel, J. M. and McGuire, E. J.: Urethral dilation improves bladder compliance in children with myelomeningocele and high leak point pressures. J Urol, 144: 430, 1990 4. Dykstra, D. D. and Sidi, A. A.: Treatment of detrusor-striated sphincter dyssynergia with botulinum A toxin. Arch Phys Med Rehabilitation, 71: 24, 1990 5. Duckett, J. W. and Ziylan, O.: Uses and abuses of vesicostomy. AUA Update, Series XIV:16, 1995 6. Duckett, J. W.: Cutaneous vesicostomy in childhood. Urol Clin North Am, 1: 485, 1974 7. Blocksom, B. H., Jr.: Bladder pouch for prolonged tubeless cystostomy. J Urol, 78: 398, 1957 8. Schwarz, G. R. and Jeffs, R. D.: Ileal conduit urinary diversion in children: computer analysis of followup from 2 to 16 years. J Urol, 114: 285, 1975 9. Locke, J. R., Hall, D. E. and Walzer, Y.: Incidence of squamous cell carcinoma in patients with long-term catheter drainage. J Urol, 133: 1034, 1985 10. Lockhart, J. L. and Pow-Sang, J. M.: Indications and problems with external urethral sphincterotomy. Probl Urol, 3: 44, 1989 11. Allen, T. D.: Vesicostomy for the temporary diversion of the urine in small children. J Urol, 123: 929, 1980 12. Sonda, L. P. and Solomon, M. H.: Twenty-year outcome of cutaneous vesicostomy. J Urol, 124: 326, 1980 13. Bruce, R. R. and Gonzales, E. T., Jr.: Cutaneous vesicostomy: a useful form of temporary diversion in children. J Urol, 123: 927, 1980 14. Cohen, J. S., Harbach, L. B. and Kaplan, G. W.: Cutaneous vesicostomy for temporary urinary diversion in infants with neurogenic bladder dysfunction. J Urol, 119: 120, 1978 15. Belman, A. B. and King, L. R.: Vesicostomy. Useful means of reversible urinary diversion in selected infants. Urology, 1: 208, 1973 16. Noe, H. N. and Jerkins, G. R.: Cutaneous vesicostomy experience in infants and children. J Urol, 134: 301, 1985 17. Agarwal, S. K., Khoury, A. E., Abramson, R. P. et al: Outcome analysis of vesicoureteral reflux in children with myelodysplasia. J Urol, 157: 980, 1997 18. Mandell, J., Bauer, S. B., Colodny, A. H. et al: Cutaneous vesicostomy in infancy. J Urol, 126: 92, 1981 19. Snyder, H. M., III, Kalichman, M. A., Charney, E. et al: Vesicostomy for neurogenic bladder with spina bifida. J Urol, 130: 724, 1983 20. Mutchnik, S. E., Hinson, J. L., Nickell, K. G. et al: Ileovesicostomy as an alternative form of bladder management in tetraplegic patients. Urology, 49: 953, 1997 21. Blyth, B., Ewalt, D. H., Duckett, J. W. et al: Lithogenic properties of enterocystoplasty. J Urol, 148: 575, 1992