The Surgical
Management of Urinary Incontinence Myelodysplastic Children
in
By John G. Pike, Gina Berardinucci, Brigette Hamburger, and George Kiruluta
Montreal, Quebec l Over a 4-year period, 69 patients with intractable urinary incontinence secondary to myelodysplasia have undergone surgical therapy to try to achieve continence. Preoperative evaluation used uroradiological and urodynamic studies, including measurement of leak point pressure and leak point volume. Twenty-one patients had a procedure to increase outlet resistance, 30 patients had bladder augmentation, and 18 patients had both procedures performed. Fifty-seven of the 69 patients have achieved total continence for a success rate of 83%. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: Myelodysplasia; ileocystoplasty; urinary sphincter; urinary incontinence.
artificial
S
INCE THE INTRODUCTION of clean intermittent catheterization by Lapides et al in 1972,’ the urological management of children with neurogenic bladder dysfunction secondary to myelodysplasia has dramatically improved.* This modality, along with pharmacological manipulation of the lower urinary tract, has enabled many of these children to maintain urinary continence, and subsequently achieve social acceptability. Despite these improvements, there remains a significant proportion of myelodysplastic children who fail to respond to maximal medical management and continue to be incontinent. In an effort to help these children with intractable incontinence, surgical intervention is frequently considered. The etiology of incontinence in these children is usually not clear-cut. It may be a result of detrusor hyperreflexia, decreased detrusor compliance, or urethral sphincter insufficiency, either alone or in combination.3 Subsequently, the decision as to which patient will benefit from bladder augmentation alone, a procedure to increase outlet resistance alone, or a combination of the two is often not an easy one. To complicate matters further, recent reports have suggested that the neurourological lesion in myelodysplastic children and resultant urodynamic parameters
From the Division of Pediatric Urology, Department of Surgery, Montreal Children’s Hospital, Shriners ’ Hospital for Crippled Children, and Hopital Sainte-Justine, Montreal, Quebec. Presented at the 21st Annual Meeting of the American Pediatric Surgical Association, Vancouver, British Columbia, May 19-22, 1990. Address reprint requests to George Kiruluta, MD, Division of Pediatric Urology, Department of Surgery, Montreal Children’s Hospital, 2300 Tupper St, Montreal, Quebec H3H IP3, Canada. Copyright o 1991 by W;B. Saunders Company 0022-3468/91/2604-0019$03.00/O 466
change with time.“6 In treating these patients, we have used preoperative urodynamics and uroradiological studies to guide surgical management. MATERIALS AND METHODS Between November 1985 and November 1989,69 myelodysplastic children with intractable urinary incontinence were evaluated and managed surgically. Basic evaluation included serum creatinine determination, abdominal ultrasound, excretory urogram (IVP), voiding cystourethrogram, and baseline urodynamic studies. Leak point pressure and leak point volume were performed as part of the urodynamic assessment.’ These measurements are simply the intravesical pressure and bladder volume, respectively, at which urine was observed to leak out the external meatus. A leak point pressure of ~55 cm H,O was considered evidence of sphincteric insufficiency and indicated the need of a procedure to increase outlet resistance, either a bladder neck sling”,Yin females or implantation of an artificial urinary sphincter in males.“’ Bladder neck sling was carried out by a transabdominal approach. After harvesting a strip of rectus fascia 1.5 cm wide x 14 cm long, periurethral dissection was performed and the fascial strip was passed suburethrally. The two ends of the strip were then attached to the posterior aspect of the pubis with nonabsorbable suture material. We have used the AMS-800 artificial urinary sphincter (American Medical Systems, Minneapolis, MN) and the technique of implantation described previously by Scott et al.‘” All of the patients except for 3 had the cuff placed at the bladder neck. Specifications of the sphincters used are listed in Table 1. A leak point volume of < 100 mL was an indication for bladder augmentation, and this was performed by ileocystoplasty. We have used the “cup patch” technique of ileocystoplasty described by Goodwin et al in 1959.” A combination of low leak point pressure ( < 55 cm H,O) and low leak point volume ( < 100 mL) was an indication for combined ileocystoplasty and either bladder neck sling or artificial urinary sphincter. Postoperative evaluation has included monitoring of renal function, continence status, and periodic radiological surveillance. RESULTS Of the 69 patients, 5 girls have undergone a bladder neck sling, 16 boys have had implantation of an artificial urinary sphincter, 30 patients (including 14 girls and 16 boys) have had an ileocystoplasty, 6 girls had a bladder neck sling and ileocystoplasty, and 12 boys had an artificial urinary sphincter and ileocystoplasty.
Bladder Neck Sling
Of the 5 patients who had a bladder neck sling, one had a bilateral ureteric reimplantation at the time of her sling because of severe, persistent reflux and another had had a bilateral reimplantation 8 years prior to her sling at the age of 8. Postoperatively, all 5 Journalof Pediatric Surgery, Vol26, No 4 (April), 1991: pp 466-471
TREATMENT
OF INCONTINENCE
467
IN MYELODYSPLASIA
Table 1. AMS-860 Artificial Sphincter Specifications
in 28 Patients
PWSSW3
Occlusive
Regulating
Cuff Length
Balloon
No. of
Icm)
(cm H,O)
Patients
6.5 to 10.0
51 to 60
8
4.5 to 9.0
61 to 70
14
4.5 to 8.0
71 to 80
6
Augmentation Ileocystoplasty
patients intermittently catheterize and 3 have achieved total urinary continence (Table 2). Two patients presently have stress urinary incontinence, one of whom has had urodynamics repeated postoperatively. Preoperative urodynamics had shown an areflexive type bladder with a leak point pressure of 20 cm H,O and leak point volume of 220 mL. Postoperatively, the bladder is hyperreflexic with a leak point pressure of 60 cm H,O and leak point volume of 160 mL. To date, the only complication in this group has been a pyelonephritis 2 weeks postoperation in the child who underwent sling and bilateral reimplantation. Upper tracts and renal function have remained stable and the reflux in the child who had ureteral reimplantation has resolved. Artificial Urinary Sphincter
Four of the 16 patients undergoing implantation of an artificial urinary sphincter have had their sphincter removed, 2 because of a urethral erosion, 1 developed a urethral false passage and subsequent urinoma that became infected, and 1 had a postoperative wound infection that would not respond to treatment. Three of the 4 lost their sphincters shortly after implantation, but one child was totally continent and voiding spontaneously on activation of his sphincter for 3 years. Eight boys are managing well with their sphincters and void spontaneously on activation every 3 to 4 hours (Table 2). There have been no sphincter revisions in this group. Two other patients are also dry, but do not void spontaneously and intermittently catheterize every 3 to 4 hours. Two patients, both of whom have required a sphincter revision, continue to be incontinent. Preoperative urodynamics on these patients had shown areflexic bladders but repeat Table 2. Results of Procedures to Increase Outlet Resistance Bladder
studies postoperation show hyperreflexia with low leak point volumes with a deactivated sphincter (103 and 110 mL). To date, upper tracts and renal function have remained stable in this group.
Artificial
Neck
Urinary
Sling
Sphincter
Of the 30 patients undergoing ileocystoplasty, 16 had this procedure performed solely, although one patient previously had a left nephrectomy and two previously had bilateral ureteric reimplantations. Three patients underwent ileocystoplasty and closure of a vesicostomy previously created for bilateral hydronephrosis and urinary tract infection. Three patients had ileocystoplasty and transureteroureterostomy, one of whom had had a vesicostomy and later closure of vesicostomy and bilateral ureteral reimplantation. Three patients had ileocystoplasty and ureteric reimplantation, two unilateral and one bilateral. The child who had undergone bilateral reimplantation had had a previous reimplantation for high-grade reflux. This recurred approximately 1 year postoperatively. Of the 2 children with single reimplantations, one had a bilateral reimplantation 4 years earlier with reflux recurring on one side only, the other had had previous transureteroureterostomy and cutaneous ureterostomy a year earlier. Two patients had ileocystoplasty, reimplantation, and closure of previously formed vesicostomy. The remaining three patients had undiversions, with two having anastomosis of detubularized ileal conduits to the urinary bladder. One patient had undergone ileoconduit diversion at 5 months of age, which later became obstructed and required three stoma1 revisions. This child had the ileoconduit taken down, primary bilateral ureteral anastomosis, and ileocystoplasty at the age of 7. Postoperatively, 25 patients are continent and manage their augmented bladder by intermittent catheterization every 4 to 6 hours (Table 3). Two patients are continent and void spontaneously by abdominal straining. Three patients have continued to have significant incontinence and are being considered for procedures to improve outlet resistance. One of the children who had ileocystoplasty, closure of vesicostomy, and bilateral reimplantation has been noted to have a mild degree of unilateral ureterovesiTable 3. Results of Bladder Augmentation lleocystoplasty
o/5
1610
Median age (yr) (range)
15 (6-20)
16 (10-20)
Median age (yr) (range)
13 (6-26)
Median follow-up (mo) (range)
24 (8-38)
21 (9-39)
Median follow-up (yr) (range)
29 (g-60)
Male/female
No. totally continent (%)
60
Male/female
16/14
62
No. totally continent (X)
90
67
Spontaneous voiding (%)
92
Intermittent catheterization (%)
Spontaneous voiding (%)
-
Intermittent catheterization (%)
100
33
Stable renal function (%)
100
100
Stable renal function (%)
8 97
PIKE ET AL
cal obstruction and deterioration of renal function on that side. Otherwise, there have been no major complications in this group of patients and renal function and upper tracts have improved or remained stable. All patients irrigate their bladders either once or twice weekly to avoid mucus build up. Ileocystoplasty and Bladder Neck Sling
Of patients undergoing ileocystoplasty and bladder neck sling, 4 underwent both procedures at the same time and another had undergone bilateral reimplantation and bladder neck sling 4 years prior to ileocystoplasty. The remaining patient had a MarshallMarchetti procedure 2 years prior to her definitive procedure. Both of these patients underwent secondary procedures because of persistent incontinence. Postoperatively, all 6 patients are continent on intermittent catheterization every 4 to 6 hours, and all irrigate their bladders once or twice weekly to eliminate mucus (Table 4). None of these patients have had complications and all have stable renal function and upper tracts. One patient has been lost to follow-up for approximately 1 year. Ileocystoplasty and Artificial Urinary Sphincter
Two of the 12 patients who had ileocystoplasty and artificial urinary sphincter implantation had both procedures done simultaneously. Seven patients initially had artificial sphincter implantation and then later required ileocystoplasty because of persistent incontinence. The interval between operations averaged 17 months. Of these patients, one developed a urethral stricture after insertion of his sphincter and subsequently required a urethroplasty (Table 5). Another patient suffered a bladder rupture after sphincter implantation and, after bladder repair, developed incontinence due to detrusor hyperreflexia. This patient was also found to have an adequate leak point pressure but low leak point volume on urodynamic studies prior to his augmentation. Three patients initially had an ileocystoplasty, then Table 4. Results of lleocystoplasty
and Procedure to Increase Outlet
Resistance lleOcyStoplaSty
lleocystoplasty
and
and Bladder
Artificial
Neck Sling
Urinary Sphincter
Median age (yr) (range)
O/6 16 (5-24)
15 (11-21)
Median follow-up (mo) (range)
Male/female
12/O
22 (8-32)
15 (7-30)
No. totally continent (%)
100
91
Spontaneous voiding (%)
-
36
Intermittent catheterization (%)
100
64
Stable renal function (%)
100
100
Table 5. Complications of Surgery in 69 Patients No. of Patients
Bladder neck sling Pyelonephritis Artificial urinary sphincter
1 1 6
Urethral erosion
2
Urethral false passage and urinoma
1
Wound infection
1
Sphincter malfunction
2
lleocystoplasty Ureterovesical obstruction
1 1
lleocystoplasty and bladder neck sling
0
lleocystoplasty and artificial urinary sphincter
7
Urethral structure
1
Bladder rupture
1
Sphincter malfunction
4
Small bowl obstruction
1
Bladder calculus
1
required an artificial urinary sphincter because of persistent incontinence. The time period between operations in these patients averaged 16 months. One of these patients previously had a cutaneous vesicostomy because of massive reflux, and later had closure of vesicostomy and bilateral reimplantation. At the time of ileocystoplasty, the patient had a left-to-right transureteroureterostomy and then right reimplantation because of persistent reflux. Three patients have required revision of their artificial sphincters, one patient requiring a single revision and two others requiring two. One of the two patients who underwent sphincter implantation and ileocystoplasty as a single procedure had been initially managed with an ileal conduit and lost his right kidney because of calculi and recurrent infections. This patient actually underwent removal of his ileal conduit, reimplantation of the left ureter, ileocystoplasty, and insertion of an artificial urinary sphincter as a single procedure. Eleven of the 12 patients have attained urinary continence (Table 4). Four patients void spontaneously with no residuals and 3 void spontaneously but do catheterizations once daily. Four do not void and subsequently do catheterizations either 3 or 4 times daily. All irrigate their bladders either once or twice weekly to control mucus production. One patient has remained incontinent and has been found to have an inadequately functioning sphincter requiring insertion of a higher-pressure balloon. The only other complication in this group was the boy who underwent the extensive procedure described previously. He developed a bowel obstruction about 3 weeks postoperation and this was managed conservatively with subsequent resolution. This patient has also developed a bladder calculus postoperatively. Overall, 57 of the 69 patients treated for intractable
TREATMENT
OF INCONTINENCE
469
IN MYELODYSPLWA
urinary incontinence have achieved total continence, for a success rate of 83%. DISCUSSION The surgical management of myelodysplastic children with intractable urinary incontinence is often frustrating but always challenging. In the past, most of these patients were inadequately managed and, thus, socially ostracized. With the advent of the ileal conduit, there was hope that finally there was surgical treatment that would provide long-term relief for these unfortunate patients. It was not long until clinicians realized that this type of diversion simply opened a Pandora’s box of new clinical problems.” New approaches regarding management of these patients have included continent urinary diversions, the use of detubularized bowel segments to augment the noncompliant or hyperreflexic bladder, or an artificial urinary sphincter and bladder neck sling to bolster outlet resistance. A proportion of patients initially require both procedures in order to achieve a balanced system capable of both storage and control. With experience, it has been found that some patients after undergoing either bladder augmentation or a procedure to increase outlet resistance, require the alternate procedure also, and this is likely due to either a changing neurological lesion (eg, tethered cord) or a change in lower urinary tract dynamics secondary to the initial procedure.4-7 The ideal management of these patients would require minimal intervention and methods to achieve urinary continence without risk of infection or upper tract damage. It is evident that this goal has not been reached and that these patients often undergo multiple surgical procedures with uncertain knowledge of what the future holds. The fact that the surgeon is often unsure of which procedure is most appropriate for a particular patient is of no help to these children. In order to best manage them, various radiological
and urodynamic studies are often performed. The description of leak point pressure and leak point volume by McGuire et al in 1981 allowed some insight to those patients who would be at increased risk of upper urinary tract deterioration and recurrent incontinence.7 We have found that these measurements enhance accurate diagnosis and thus allow better surgical management. Upper tract deterioration is a very serious risk in many of these patients. The importance of life-long radiological surveillance cannot be overemphasized. It has been documented previously, and also observed in a few of the present patients, that in certain cases when the urethral resistance is increased, intravesical pressure may increase or the detrusor may become hyperreflexic.3’9,‘3 This leads to either upper tract deterioration or incontinence, depending on the relative pressures in the upper and lower urinary tract and sphincter mechanism. As more bladder augmentations have been performed and followed over time, it has become evident that there is a risk of malignancy, especially of the bowel segment and at the bowel-bladder anastomotic site-l4 Myelodysplastic patients are generally augmented in childhood and are subsequently at risk for their entire lives, necessitating meticulous long-term surveillance. If more suitable natural or synthetic material becomes available for augmentation, there may come a time when surgeons will be criticized for having put these patients at long-term risk. A recent report has described use of a neuroprosthesis in order to achieve continence in two myelodysplastic patients.15 However, until further experience is gained with this procedure, bladder augmentation and procedures to improve outlet resistance will remain the standard of the surgeon’s armamentarium. The present results indicate that a satisfactory result can be obtained provided patients are carefully selected and have attentive follow-up.
REFERENCES 1. Lapides J, Diokno AC, Silber SJ, et al: Clean intermittent self-catheterization in the treatment of urinary tract disease. J Urol 107:458-462, 1972 2. Action Committee on Myelodysplasia, Section on Urology: Current approaches to evaluation and management of children with myelomeningocele. Pediatrics 63:663-667, 1979 3. Woodside JR, Borden TA: Suprapubic endoscopic vesical neck suspension for the management of urinary incontinence in myelodysplastic girls. J Urol15:97-99,1986 4. McGuire EJ, Woodside JR, Borden TA: Upper urinary tract deterioration in patients with myelodysplasia and detrusor hypertonia: A follow-up study. J Urol 129:823-826, 1983 5. Spindel MR, Bauer SB, Dyro FM, et al: The changing neurourologic lesion in myelodysplasia. JAMA 258:1630-1633, 1987
6. Wang SC, McGuire EJ, Bloom DA: A bladder pressure management system for myelodysplasia--CIinical outcome. J Urol 140:1499-1502,1988 7. McGuire EJ, Woodside JR, Borden TA, et al: Prognostic value of urodynamic testing in myelodysplastic patients. .I Ural 126:205-209, 1981 8. Morgan JE: A sling operation using Marlex polypropylene mesh for treatment of recurrent stress incontinence. Am J Obstet Gynecol106:369-377,197O 9. McGuire EJ, Wang C, Usitalo H, et al: Modified pubovaginal sling in girls with myelodysplasia. J Urol 13594-96, 1986 10. Scott FB, Bradley WE, Timm GW: Treatment of urinary incontinence by an implantable prosthetic urinary sphincter. J Urol 112:75-80, 1974 11. Goodwin WE. Winter CC, Barker WF: “Cup-patch” tech-
470
PIKE ET AL
nique of ileocystoplasty for bladder enlargement or partial substitution. Surg Gynecol Obstet 108:240-244, 1959 12. Shapiro SR, Lebowitz R, Golodny AH: Fate of 90 children with heal conduit urinary diversion a decade later: Analysis and complications, pyelography, renal function and bacteriology. J Uroll14:289-295,1975 13. Roth DR, Vyas PR, Kroovand RL, et al: Urinary tract
deterioration associated with the artificial urinary sphincter. J Urol 135528-530, 1986 14. Filmer RB, Spencer JR: Malignancies in bladder augmentations and intestinal conduits. J Urol 143:671-678, 1990 15. Schmidt RA, Kogan BA, Tanagho EA: Neuroprostheses in the management of incontinence in myelomeningocele patients. J Ural 143:779-782,199O
Discussion P. Pun’ (Dublin, Ireland): These results are very impressive in the surgical management of myelodysplastic children. In Dublin we see a lot of these patients. Seventeen of the 69 children are able to void spontaneously. Forty-two are able to stay dry on intermittent catheterization and only 10 children continue to be incontinent. It is interesting to note that even though 13 of the 28 children who have an artificial sphincter implanted develop major complications, still over half of the patients in this group are able to void spontaneously on activation. In Ireland and Britain we have been less aggressive in managing these children, and I have relied mainly on clean intermittent catheterization preventing upper tract damage by endoscopic correction of a side with reflux and bladder augmentation in selected cases. I have a few questions for Dr Pike. As you mentioned, we see a lot of spina bifida in Ireland, it being the highest incidence anywhere in the world. I would like to ask, during this 4-year review, how many myelodysplastic children with urinary incontinence did you have who could be managed solely by clean intermittent catheterization or pharmacological treatment? Second, a number of investigators have used periurethral injection in some selected cases. Would you like to comment on the role of this? Lastly, in Europe a lot of us use colocystoplasty instead of ileum. 1 wonder if you would comment on your reason for preference of ileum over colon. A. PeEa (New York, NY): As you well know, a patient with spina bifida will represent a urinary problem as well as bowel control problem. I recently saw two patients who underwent augmentation cystoplasty. The surgeons took part of the bowel to augment the bladder and the patients who were perfectly manageable before, from the bowel point of view (I must say that the bowel management in these patients is relatively easy), become unmanageable after these operations. The stools become rather loose and the patients have to be subjected to special diets, immodium, etc. If the patient is sensitive to certain types of food, perhaps one should be more conservative about the use of these procedures and certainly one could think about the gastrocystoplasty
that Dr Mitchell has been using. Another thing that I read in the literature and heard here is that the use of the word continent is interchangeable for the words dry and clean. In bowel management, it is the same and many surgeons talk about continent patients, and what they mean is a clean child. A clean child and dry doesn’t mean continent, because then what term are we going to use for the normal child? A normal child has absolutely continent voluntary voiding, no need for catheterization, no irrigations, and that’s normal and that’s what I call continent, whereas a clean child, we can make children clean with good bowel management or urinary management, but that doesn’t mean he/she is 100% normal. Otherwise, there would be some confusion in the use of terms. H. Hendren (Boston, MA): I think this is a wonderful paper and it illustrates what great steps forward have been made in managing the poor kids that used to fill our clinics, and they all had tubes in their bladders, their urethras, suprapubic tubes, urethral catheters, nephrostomies and so forth. This is a great step forward. We certainly agree with the principals that have been outlined that you have to make the bladder big enough and you have to provide enough resistance. I would just say that, with respect to the latter providing enough resistance, if you do put in a sphincter I think ultimately many of those are going to need to be revised as you have just illustrated and an alternative is to do the classic Young-DeesLedbetter type of narrowing of the bladder outlet to provide that resistance. Of course, the trade for that is that they are then obliged to self catheterize rather than to push a button and open the sphincter, which some of your children can do. I would just emphasize that there are literally hundreds of these children out there that have been diverted in the past and I would urge everybody in this room to keep their eyes open for children who had myelo problems who were treated with diversions, which was the standard way of treating these starting in the mid-1950s and all through the 1960s and even into the mid-1970s. So there are hundreds of young patients and young adults who have urinary diversions for the myelo problem and many of them can be brought back. I
TREATMENT
OF INCONTINENCE
IN MYELODYSPLASIA
have now done 178 undiversions, and there is a large group in there that were these children that had been given up and diverted. You can go back and apply the principals that you have just so nicely outlined for us and resurrect many of those urinary tracts and totally change their social status. J. Pike (response): First of all, approximately 80% to 90% of the patients that we see in our clinic are adequately managed with clean intermittent catheterization and anticholinergies, so that leaves approximately 10% to 20% of the patients who require some sort of surgical intervention. I don’t have any experience with periurethral injection, so I can’t really
471
comment on that. With regard to the use of ileum, we feel that if the child in fact has stable renal function, ileum is as good an alternative as either colon or stomach. Obviously, I think it has proven that stomach is probably a better alternative in children who have poor renal function. As regards diarrhea, we have noticed that this is a problem initially postoperatively, but this usually resolves after 3 to 4 weeks. I would also like to emphasize that close follow-up of these children is extremely important because the lesion can change and the urodynamic findings can change with time after an intervention has taken place.
Management of Urinary Incontinence Myelodysplastic Children
in
By John G. Pike, Gina Berardinucci, Brigette Hamburger, and George Kiruluta
Montreal, Quebec l Over a 4-year period, 69 patients with intractable urinary incontinence secondary to myelodysplasia have undergone surgical therapy to try to achieve continence. Preoperative evaluation used uroradiological and urodynamic studies, including measurement of leak point pressure and leak point volume. Twenty-one patients had a procedure to increase outlet resistance, 30 patients had bladder augmentation, and 18 patients had both procedures performed. Fifty-seven of the 69 patients have achieved total continence for a success rate of 83%. Copyright o 1991 by W.B. Saunders Company INDEX WORDS: Myelodysplasia; ileocystoplasty; urinary sphincter; urinary incontinence.
artificial
S
INCE THE INTRODUCTION of clean intermittent catheterization by Lapides et al in 1972,’ the urological management of children with neurogenic bladder dysfunction secondary to myelodysplasia has dramatically improved.* This modality, along with pharmacological manipulation of the lower urinary tract, has enabled many of these children to maintain urinary continence, and subsequently achieve social acceptability. Despite these improvements, there remains a significant proportion of myelodysplastic children who fail to respond to maximal medical management and continue to be incontinent. In an effort to help these children with intractable incontinence, surgical intervention is frequently considered. The etiology of incontinence in these children is usually not clear-cut. It may be a result of detrusor hyperreflexia, decreased detrusor compliance, or urethral sphincter insufficiency, either alone or in combination.3 Subsequently, the decision as to which patient will benefit from bladder augmentation alone, a procedure to increase outlet resistance alone, or a combination of the two is often not an easy one. To complicate matters further, recent reports have suggested that the neurourological lesion in myelodysplastic children and resultant urodynamic parameters
From the Division of Pediatric Urology, Department of Surgery, Montreal Children’s Hospital, Shriners ’ Hospital for Crippled Children, and Hopital Sainte-Justine, Montreal, Quebec. Presented at the 21st Annual Meeting of the American Pediatric Surgical Association, Vancouver, British Columbia, May 19-22, 1990. Address reprint requests to George Kiruluta, MD, Division of Pediatric Urology, Department of Surgery, Montreal Children’s Hospital, 2300 Tupper St, Montreal, Quebec H3H IP3, Canada. Copyright o 1991 by W;B. Saunders Company 0022-3468/91/2604-0019$03.00/O 466
change with time.“6 In treating these patients, we have used preoperative urodynamics and uroradiological studies to guide surgical management. MATERIALS AND METHODS Between November 1985 and November 1989,69 myelodysplastic children with intractable urinary incontinence were evaluated and managed surgically. Basic evaluation included serum creatinine determination, abdominal ultrasound, excretory urogram (IVP), voiding cystourethrogram, and baseline urodynamic studies. Leak point pressure and leak point volume were performed as part of the urodynamic assessment.’ These measurements are simply the intravesical pressure and bladder volume, respectively, at which urine was observed to leak out the external meatus. A leak point pressure of ~55 cm H,O was considered evidence of sphincteric insufficiency and indicated the need of a procedure to increase outlet resistance, either a bladder neck sling”,Yin females or implantation of an artificial urinary sphincter in males.“’ Bladder neck sling was carried out by a transabdominal approach. After harvesting a strip of rectus fascia 1.5 cm wide x 14 cm long, periurethral dissection was performed and the fascial strip was passed suburethrally. The two ends of the strip were then attached to the posterior aspect of the pubis with nonabsorbable suture material. We have used the AMS-800 artificial urinary sphincter (American Medical Systems, Minneapolis, MN) and the technique of implantation described previously by Scott et al.‘” All of the patients except for 3 had the cuff placed at the bladder neck. Specifications of the sphincters used are listed in Table 1. A leak point volume of < 100 mL was an indication for bladder augmentation, and this was performed by ileocystoplasty. We have used the “cup patch” technique of ileocystoplasty described by Goodwin et al in 1959.” A combination of low leak point pressure ( < 55 cm H,O) and low leak point volume ( < 100 mL) was an indication for combined ileocystoplasty and either bladder neck sling or artificial urinary sphincter. Postoperative evaluation has included monitoring of renal function, continence status, and periodic radiological surveillance. RESULTS Of the 69 patients, 5 girls have undergone a bladder neck sling, 16 boys have had implantation of an artificial urinary sphincter, 30 patients (including 14 girls and 16 boys) have had an ileocystoplasty, 6 girls had a bladder neck sling and ileocystoplasty, and 12 boys had an artificial urinary sphincter and ileocystoplasty.
Bladder Neck Sling
Of the 5 patients who had a bladder neck sling, one had a bilateral ureteric reimplantation at the time of her sling because of severe, persistent reflux and another had had a bilateral reimplantation 8 years prior to her sling at the age of 8. Postoperatively, all 5 Journalof Pediatric Surgery, Vol26, No 4 (April), 1991: pp 466-471
TREATMENT
OF INCONTINENCE
467
IN MYELODYSPLASIA
Table 1. AMS-860 Artificial Sphincter Specifications
in 28 Patients
PWSSW3
Occlusive
Regulating
Cuff Length
Balloon
No. of
Icm)
(cm H,O)
Patients
6.5 to 10.0
51 to 60
8
4.5 to 9.0
61 to 70
14
4.5 to 8.0
71 to 80
6
Augmentation Ileocystoplasty
patients intermittently catheterize and 3 have achieved total urinary continence (Table 2). Two patients presently have stress urinary incontinence, one of whom has had urodynamics repeated postoperatively. Preoperative urodynamics had shown an areflexive type bladder with a leak point pressure of 20 cm H,O and leak point volume of 220 mL. Postoperatively, the bladder is hyperreflexic with a leak point pressure of 60 cm H,O and leak point volume of 160 mL. To date, the only complication in this group has been a pyelonephritis 2 weeks postoperation in the child who underwent sling and bilateral reimplantation. Upper tracts and renal function have remained stable and the reflux in the child who had ureteral reimplantation has resolved. Artificial Urinary Sphincter
Four of the 16 patients undergoing implantation of an artificial urinary sphincter have had their sphincter removed, 2 because of a urethral erosion, 1 developed a urethral false passage and subsequent urinoma that became infected, and 1 had a postoperative wound infection that would not respond to treatment. Three of the 4 lost their sphincters shortly after implantation, but one child was totally continent and voiding spontaneously on activation of his sphincter for 3 years. Eight boys are managing well with their sphincters and void spontaneously on activation every 3 to 4 hours (Table 2). There have been no sphincter revisions in this group. Two other patients are also dry, but do not void spontaneously and intermittently catheterize every 3 to 4 hours. Two patients, both of whom have required a sphincter revision, continue to be incontinent. Preoperative urodynamics on these patients had shown areflexic bladders but repeat Table 2. Results of Procedures to Increase Outlet Resistance Bladder
studies postoperation show hyperreflexia with low leak point volumes with a deactivated sphincter (103 and 110 mL). To date, upper tracts and renal function have remained stable in this group.
Artificial
Neck
Urinary
Sling
Sphincter
Of the 30 patients undergoing ileocystoplasty, 16 had this procedure performed solely, although one patient previously had a left nephrectomy and two previously had bilateral ureteric reimplantations. Three patients underwent ileocystoplasty and closure of a vesicostomy previously created for bilateral hydronephrosis and urinary tract infection. Three patients had ileocystoplasty and transureteroureterostomy, one of whom had had a vesicostomy and later closure of vesicostomy and bilateral ureteral reimplantation. Three patients had ileocystoplasty and ureteric reimplantation, two unilateral and one bilateral. The child who had undergone bilateral reimplantation had had a previous reimplantation for high-grade reflux. This recurred approximately 1 year postoperatively. Of the 2 children with single reimplantations, one had a bilateral reimplantation 4 years earlier with reflux recurring on one side only, the other had had previous transureteroureterostomy and cutaneous ureterostomy a year earlier. Two patients had ileocystoplasty, reimplantation, and closure of previously formed vesicostomy. The remaining three patients had undiversions, with two having anastomosis of detubularized ileal conduits to the urinary bladder. One patient had undergone ileoconduit diversion at 5 months of age, which later became obstructed and required three stoma1 revisions. This child had the ileoconduit taken down, primary bilateral ureteral anastomosis, and ileocystoplasty at the age of 7. Postoperatively, 25 patients are continent and manage their augmented bladder by intermittent catheterization every 4 to 6 hours (Table 3). Two patients are continent and void spontaneously by abdominal straining. Three patients have continued to have significant incontinence and are being considered for procedures to improve outlet resistance. One of the children who had ileocystoplasty, closure of vesicostomy, and bilateral reimplantation has been noted to have a mild degree of unilateral ureterovesiTable 3. Results of Bladder Augmentation lleocystoplasty
o/5
1610
Median age (yr) (range)
15 (6-20)
16 (10-20)
Median age (yr) (range)
13 (6-26)
Median follow-up (mo) (range)
24 (8-38)
21 (9-39)
Median follow-up (yr) (range)
29 (g-60)
Male/female
No. totally continent (%)
60
Male/female
16/14
62
No. totally continent (X)
90
67
Spontaneous voiding (%)
92
Intermittent catheterization (%)
Spontaneous voiding (%)
-
Intermittent catheterization (%)
100
33
Stable renal function (%)
100
100
Stable renal function (%)
8 97
PIKE ET AL
cal obstruction and deterioration of renal function on that side. Otherwise, there have been no major complications in this group of patients and renal function and upper tracts have improved or remained stable. All patients irrigate their bladders either once or twice weekly to avoid mucus build up. Ileocystoplasty and Bladder Neck Sling
Of patients undergoing ileocystoplasty and bladder neck sling, 4 underwent both procedures at the same time and another had undergone bilateral reimplantation and bladder neck sling 4 years prior to ileocystoplasty. The remaining patient had a MarshallMarchetti procedure 2 years prior to her definitive procedure. Both of these patients underwent secondary procedures because of persistent incontinence. Postoperatively, all 6 patients are continent on intermittent catheterization every 4 to 6 hours, and all irrigate their bladders once or twice weekly to eliminate mucus (Table 4). None of these patients have had complications and all have stable renal function and upper tracts. One patient has been lost to follow-up for approximately 1 year. Ileocystoplasty and Artificial Urinary Sphincter
Two of the 12 patients who had ileocystoplasty and artificial urinary sphincter implantation had both procedures done simultaneously. Seven patients initially had artificial sphincter implantation and then later required ileocystoplasty because of persistent incontinence. The interval between operations averaged 17 months. Of these patients, one developed a urethral stricture after insertion of his sphincter and subsequently required a urethroplasty (Table 5). Another patient suffered a bladder rupture after sphincter implantation and, after bladder repair, developed incontinence due to detrusor hyperreflexia. This patient was also found to have an adequate leak point pressure but low leak point volume on urodynamic studies prior to his augmentation. Three patients initially had an ileocystoplasty, then Table 4. Results of lleocystoplasty
and Procedure to Increase Outlet
Resistance lleOcyStoplaSty
lleocystoplasty
and
and Bladder
Artificial
Neck Sling
Urinary Sphincter
Median age (yr) (range)
O/6 16 (5-24)
15 (11-21)
Median follow-up (mo) (range)
Male/female
12/O
22 (8-32)
15 (7-30)
No. totally continent (%)
100
91
Spontaneous voiding (%)
-
36
Intermittent catheterization (%)
100
64
Stable renal function (%)
100
100
Table 5. Complications of Surgery in 69 Patients No. of Patients
Bladder neck sling Pyelonephritis Artificial urinary sphincter
1 1 6
Urethral erosion
2
Urethral false passage and urinoma
1
Wound infection
1
Sphincter malfunction
2
lleocystoplasty Ureterovesical obstruction
1 1
lleocystoplasty and bladder neck sling
0
lleocystoplasty and artificial urinary sphincter
7
Urethral structure
1
Bladder rupture
1
Sphincter malfunction
4
Small bowl obstruction
1
Bladder calculus
1
required an artificial urinary sphincter because of persistent incontinence. The time period between operations in these patients averaged 16 months. One of these patients previously had a cutaneous vesicostomy because of massive reflux, and later had closure of vesicostomy and bilateral reimplantation. At the time of ileocystoplasty, the patient had a left-to-right transureteroureterostomy and then right reimplantation because of persistent reflux. Three patients have required revision of their artificial sphincters, one patient requiring a single revision and two others requiring two. One of the two patients who underwent sphincter implantation and ileocystoplasty as a single procedure had been initially managed with an ileal conduit and lost his right kidney because of calculi and recurrent infections. This patient actually underwent removal of his ileal conduit, reimplantation of the left ureter, ileocystoplasty, and insertion of an artificial urinary sphincter as a single procedure. Eleven of the 12 patients have attained urinary continence (Table 4). Four patients void spontaneously with no residuals and 3 void spontaneously but do catheterizations once daily. Four do not void and subsequently do catheterizations either 3 or 4 times daily. All irrigate their bladders either once or twice weekly to control mucus production. One patient has remained incontinent and has been found to have an inadequately functioning sphincter requiring insertion of a higher-pressure balloon. The only other complication in this group was the boy who underwent the extensive procedure described previously. He developed a bowel obstruction about 3 weeks postoperation and this was managed conservatively with subsequent resolution. This patient has also developed a bladder calculus postoperatively. Overall, 57 of the 69 patients treated for intractable
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IN MYELODYSPLWA
urinary incontinence have achieved total continence, for a success rate of 83%. DISCUSSION The surgical management of myelodysplastic children with intractable urinary incontinence is often frustrating but always challenging. In the past, most of these patients were inadequately managed and, thus, socially ostracized. With the advent of the ileal conduit, there was hope that finally there was surgical treatment that would provide long-term relief for these unfortunate patients. It was not long until clinicians realized that this type of diversion simply opened a Pandora’s box of new clinical problems.” New approaches regarding management of these patients have included continent urinary diversions, the use of detubularized bowel segments to augment the noncompliant or hyperreflexic bladder, or an artificial urinary sphincter and bladder neck sling to bolster outlet resistance. A proportion of patients initially require both procedures in order to achieve a balanced system capable of both storage and control. With experience, it has been found that some patients after undergoing either bladder augmentation or a procedure to increase outlet resistance, require the alternate procedure also, and this is likely due to either a changing neurological lesion (eg, tethered cord) or a change in lower urinary tract dynamics secondary to the initial procedure.4-7 The ideal management of these patients would require minimal intervention and methods to achieve urinary continence without risk of infection or upper tract damage. It is evident that this goal has not been reached and that these patients often undergo multiple surgical procedures with uncertain knowledge of what the future holds. The fact that the surgeon is often unsure of which procedure is most appropriate for a particular patient is of no help to these children. In order to best manage them, various radiological
and urodynamic studies are often performed. The description of leak point pressure and leak point volume by McGuire et al in 1981 allowed some insight to those patients who would be at increased risk of upper urinary tract deterioration and recurrent incontinence.7 We have found that these measurements enhance accurate diagnosis and thus allow better surgical management. Upper tract deterioration is a very serious risk in many of these patients. The importance of life-long radiological surveillance cannot be overemphasized. It has been documented previously, and also observed in a few of the present patients, that in certain cases when the urethral resistance is increased, intravesical pressure may increase or the detrusor may become hyperreflexic.3’9,‘3 This leads to either upper tract deterioration or incontinence, depending on the relative pressures in the upper and lower urinary tract and sphincter mechanism. As more bladder augmentations have been performed and followed over time, it has become evident that there is a risk of malignancy, especially of the bowel segment and at the bowel-bladder anastomotic site-l4 Myelodysplastic patients are generally augmented in childhood and are subsequently at risk for their entire lives, necessitating meticulous long-term surveillance. If more suitable natural or synthetic material becomes available for augmentation, there may come a time when surgeons will be criticized for having put these patients at long-term risk. A recent report has described use of a neuroprosthesis in order to achieve continence in two myelodysplastic patients.15 However, until further experience is gained with this procedure, bladder augmentation and procedures to improve outlet resistance will remain the standard of the surgeon’s armamentarium. The present results indicate that a satisfactory result can be obtained provided patients are carefully selected and have attentive follow-up.
REFERENCES 1. Lapides J, Diokno AC, Silber SJ, et al: Clean intermittent self-catheterization in the treatment of urinary tract disease. J Urol 107:458-462, 1972 2. Action Committee on Myelodysplasia, Section on Urology: Current approaches to evaluation and management of children with myelomeningocele. Pediatrics 63:663-667, 1979 3. Woodside JR, Borden TA: Suprapubic endoscopic vesical neck suspension for the management of urinary incontinence in myelodysplastic girls. J Urol15:97-99,1986 4. McGuire EJ, Woodside JR, Borden TA: Upper urinary tract deterioration in patients with myelodysplasia and detrusor hypertonia: A follow-up study. J Urol 129:823-826, 1983 5. Spindel MR, Bauer SB, Dyro FM, et al: The changing neurourologic lesion in myelodysplasia. JAMA 258:1630-1633, 1987
6. Wang SC, McGuire EJ, Bloom DA: A bladder pressure management system for myelodysplasia--CIinical outcome. J Urol 140:1499-1502,1988 7. McGuire EJ, Woodside JR, Borden TA, et al: Prognostic value of urodynamic testing in myelodysplastic patients. .I Ural 126:205-209, 1981 8. Morgan JE: A sling operation using Marlex polypropylene mesh for treatment of recurrent stress incontinence. Am J Obstet Gynecol106:369-377,197O 9. McGuire EJ, Wang C, Usitalo H, et al: Modified pubovaginal sling in girls with myelodysplasia. J Urol 13594-96, 1986 10. Scott FB, Bradley WE, Timm GW: Treatment of urinary incontinence by an implantable prosthetic urinary sphincter. J Urol 112:75-80, 1974 11. Goodwin WE. Winter CC, Barker WF: “Cup-patch” tech-
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PIKE ET AL
nique of ileocystoplasty for bladder enlargement or partial substitution. Surg Gynecol Obstet 108:240-244, 1959 12. Shapiro SR, Lebowitz R, Golodny AH: Fate of 90 children with heal conduit urinary diversion a decade later: Analysis and complications, pyelography, renal function and bacteriology. J Uroll14:289-295,1975 13. Roth DR, Vyas PR, Kroovand RL, et al: Urinary tract
deterioration associated with the artificial urinary sphincter. J Urol 135528-530, 1986 14. Filmer RB, Spencer JR: Malignancies in bladder augmentations and intestinal conduits. J Urol 143:671-678, 1990 15. Schmidt RA, Kogan BA, Tanagho EA: Neuroprostheses in the management of incontinence in myelomeningocele patients. J Ural 143:779-782,199O
Discussion P. Pun’ (Dublin, Ireland): These results are very impressive in the surgical management of myelodysplastic children. In Dublin we see a lot of these patients. Seventeen of the 69 children are able to void spontaneously. Forty-two are able to stay dry on intermittent catheterization and only 10 children continue to be incontinent. It is interesting to note that even though 13 of the 28 children who have an artificial sphincter implanted develop major complications, still over half of the patients in this group are able to void spontaneously on activation. In Ireland and Britain we have been less aggressive in managing these children, and I have relied mainly on clean intermittent catheterization preventing upper tract damage by endoscopic correction of a side with reflux and bladder augmentation in selected cases. I have a few questions for Dr Pike. As you mentioned, we see a lot of spina bifida in Ireland, it being the highest incidence anywhere in the world. I would like to ask, during this 4-year review, how many myelodysplastic children with urinary incontinence did you have who could be managed solely by clean intermittent catheterization or pharmacological treatment? Second, a number of investigators have used periurethral injection in some selected cases. Would you like to comment on the role of this? Lastly, in Europe a lot of us use colocystoplasty instead of ileum. 1 wonder if you would comment on your reason for preference of ileum over colon. A. PeEa (New York, NY): As you well know, a patient with spina bifida will represent a urinary problem as well as bowel control problem. I recently saw two patients who underwent augmentation cystoplasty. The surgeons took part of the bowel to augment the bladder and the patients who were perfectly manageable before, from the bowel point of view (I must say that the bowel management in these patients is relatively easy), become unmanageable after these operations. The stools become rather loose and the patients have to be subjected to special diets, immodium, etc. If the patient is sensitive to certain types of food, perhaps one should be more conservative about the use of these procedures and certainly one could think about the gastrocystoplasty
that Dr Mitchell has been using. Another thing that I read in the literature and heard here is that the use of the word continent is interchangeable for the words dry and clean. In bowel management, it is the same and many surgeons talk about continent patients, and what they mean is a clean child. A clean child and dry doesn’t mean continent, because then what term are we going to use for the normal child? A normal child has absolutely continent voluntary voiding, no need for catheterization, no irrigations, and that’s normal and that’s what I call continent, whereas a clean child, we can make children clean with good bowel management or urinary management, but that doesn’t mean he/she is 100% normal. Otherwise, there would be some confusion in the use of terms. H. Hendren (Boston, MA): I think this is a wonderful paper and it illustrates what great steps forward have been made in managing the poor kids that used to fill our clinics, and they all had tubes in their bladders, their urethras, suprapubic tubes, urethral catheters, nephrostomies and so forth. This is a great step forward. We certainly agree with the principals that have been outlined that you have to make the bladder big enough and you have to provide enough resistance. I would just say that, with respect to the latter providing enough resistance, if you do put in a sphincter I think ultimately many of those are going to need to be revised as you have just illustrated and an alternative is to do the classic Young-DeesLedbetter type of narrowing of the bladder outlet to provide that resistance. Of course, the trade for that is that they are then obliged to self catheterize rather than to push a button and open the sphincter, which some of your children can do. I would just emphasize that there are literally hundreds of these children out there that have been diverted in the past and I would urge everybody in this room to keep their eyes open for children who had myelo problems who were treated with diversions, which was the standard way of treating these starting in the mid-1950s and all through the 1960s and even into the mid-1970s. So there are hundreds of young patients and young adults who have urinary diversions for the myelo problem and many of them can be brought back. I
TREATMENT
OF INCONTINENCE
IN MYELODYSPLASIA
have now done 178 undiversions, and there is a large group in there that were these children that had been given up and diverted. You can go back and apply the principals that you have just so nicely outlined for us and resurrect many of those urinary tracts and totally change their social status. J. Pike (response): First of all, approximately 80% to 90% of the patients that we see in our clinic are adequately managed with clean intermittent catheterization and anticholinergies, so that leaves approximately 10% to 20% of the patients who require some sort of surgical intervention. I don’t have any experience with periurethral injection, so I can’t really
471
comment on that. With regard to the use of ileum, we feel that if the child in fact has stable renal function, ileum is as good an alternative as either colon or stomach. Obviously, I think it has proven that stomach is probably a better alternative in children who have poor renal function. As regards diarrhea, we have noticed that this is a problem initially postoperatively, but this usually resolves after 3 to 4 weeks. I would also like to emphasize that close follow-up of these children is extremely important because the lesion can change and the urodynamic findings can change with time after an intervention has taken place.
