British Journal of Urology (1991), 68,543-548 01991 British Journal of Urology
Surgical Management of Incontinence in Bladder Exstrophy* JEAN G. HOLLOWELL and P. G. RANSLEY Department of Urology, Hospital for Sick Children, and the St Peter‘s Hospitals, London
Summary-Between 1978 and 1990,86 patients with previously closed classical bladder exstrophy and 10 patients seeking undiversion have presented for continence management and have undergone selective reconstruction designed for voiding and/or intermittent urethral catheterisation. The reconstruction in these 96 patients has been reviewed. Eight of the 10 patients undergoing undiversion achieved a satisfactory state of continence but 4 required Mitrofanoff procedures to enable catheterisation. Of the other 86 patients, 2 reached a satisfactory state of continence without further surgery; 79 underwent bladder neck surgery for continence either without augmentation (n =32) or with augmentation (n =47). Twenty of the 32 patients who were treated by bladder neck reconstruction alone were later found to require augmentation. Five patients had very early augmentation either to facilitate neonatal closure or on account of severe upper tract dilatation. Of these, 1 became continent without further surgery and 4 demonstrated the need for bladder neck reconstruction. Thus 12 children achieved successful continence (n =6) or are evolving satisfactorily with potential success (n = 6) as a result of bladder neck reconstruction. Of the 71 patients requiring bladder neck reconstruction and augmentation, 68 have completed their surgery. The current status of these patients is: satisfactory in 57 (80%) (42 void/urethral clean intermittent catheterisation (CIC), 7 waiting to learn CIC, 5 Mitrofanoff, 3 artificial urinary sphincter (AUS)). Of the remaining 11 patients (20%), 8 are unsatisfactory to varying degrees and the status of the other 3 is unknown.
Bladder exstrophy remains one of the most difficult surgical challenges in paediatric urology. Historically, most patients underwent urinary diversion after a series of unsuccessful reconstructive surgical procedures. In 1974,D. I. Williams wrote “although functional reconstruction seems the logical treatment and is certainly the one desired by most parents, many urologists are opposed to it on the grounds that the success rate is small, that the children are subjected to multiple procedures and that the complications are by no means negligible with serious urinary infections, pyelonephritis and *Based on a paper presented to the Urological Section of the American Academy of Pediatrics, Annual Meeting, Boston, October 1990 Accepted for publication 19 March 1991
stones” (Williams, 1974). Developments, including neonatal closure and reintroduction of osteotomies (Ansell, 1979; Jeffs et al., 1982; Osterling and Jeffs, 1986), a new single-stageepispadias repair (Ransley et al., 1988), bladder augmentation and clean intermittent catheterisation, have all contributed to the improved results of reconstruction reported over the last decade In this department there has been a firm commitment to reconstruction and no patient with exstrophy has been diverted since 1978. Between 1978 and 1990,86 patients with classical bladder exstrophy (excluding cloaca1 exstrophy, exstrophy variants and epispadias) who have undergone primary bladder closure have presented for continence management. All of these children, plus an additional 10 patients with exstrophy 543
544 presenting for undiversion, have undergone reconstruction designed for voiding and/or intermittent urethral catheterisation. (Undiversions directly to the Mitrofanoff procedure have not been included.) The reconstruction for continence in these 96 patients has been reviewed retrospectively in order to determine the final outcome and the price of this “success”. Patients and Methods Management of exstrophy This report is not a comparison of techniques but rather a discussion of how the evolution of new techniques and the timing of surgical steps have been incorporated into continence management of exstrophy by a single surgeon over the last 12 years. The general philosophy of exstrophy reconstruction over this period has been early bladder closure and annual follow-up until commencement of continence surgery after 4 years of age. In the late 1970s bladder closure was performed in the first few weeks of life without osteotomies. Subsequently, this evolved to closure without osteotomies limited to the first 24 hours of life and any delayed closures performed with posterior vertical osteotomies. Following successful closure, annual follow-up consists of cystoscopy and cystography under anaesthesia and ultrasound to assess the development of bladder capacity and status of the upper tracts. Surgery for continence is undertaken after 4 years of age and once further spontaneous expansion of the bladder appears to have ceased. Techniques The principal technique of bladder neck reconstruction has been a 2-layer Young-Dees-Leadbetter procedure (Young, 1919; Dees, 1949; Leadbetter, 1964). Initially performed without modification, it was subsequently altered by the placement of a single sheet of silicone around the reconstructed bladder neck (Diamond and Ransley, 1986). Early patients suffered from erosion of the silicone as previously documented. This led to the current practice of a circumferential omental wrap of the Young-Dees tube and a single layer of silicone Silastic sheet-0.01 in. non-reinforced (Dow Corning. Midlands, USA) loosely arranged around the omentum. Bladder augmentation has largely been by detubularised cup colocystoplasty; 15 to 30 cm of
BRITISH JOURNAL OF UROLOGY
descending/sigmoid colon were isolated, detubularised and reconstructed as a “cup” before anastomosis to the small bladder element bearing the reimplanted ureters. In those patients in whom a continent catheterisable stoma had been performed (Mitrofanoff procedure) (Mitrofanoff, 1980),it was created either by using the appendix or distal ureter in conjunction with a transuretero-ureterostomy. Bladder neck closure was performed only if necessary to prevent persistent incontinence. The single-stage epispadias repair with ventral urethral placement and corporal rotation (Ransley et al., 1988) has been used since 1985 and most of the earlier patients have undergone revision by this method. The artificial urinary sphincter models used were the AMS-792 and the AMS-800. Exchange of the sphincter cuff for the silicone wrap facilitates its placement.
Assessment of continence Obtaining an accurate history of continence status is challenging. Descriptive terms have different meanings for different patients and the history may vary from child to parent. Extensive and diligent questioning was carried out in personal interviews in order to make an accurate assessment of continence during the day and at night, at rest and during activity, and to explore the methods employed to cope with each child. It is important to note that the term “cath/void interval” is used to imply the minimum interval for emptying which will insure that the child will always be dry, not the auerage dry interval. The state of continence of this group of patients covers a wide spectrum and categorisation is somewhat arbitrary. After reviewing the assessments certain patterns appeared and from these the following classification was derived.
1. Excellent : -dry day/night/activity -cath/void intervals > 4 h -no restrictions : activity or fluid intake 2. Good: -dry day/activity -some night-time wetting -cath/void intervals > 3 h -minor coping methods employed
’
Satisfactory
545
SURGICAL MANAGEMENT OF INCONTINENCE IN BLADDER EXSTROPHY
3. Poor: -dry intervals not > 2 h without major effort -protection required 4. Wet: -continuous leaking
I
ence without additional surgery. Three of the 5 subsequently underwent bladder neck reconstruction and I is awaiting further surgery for continence. The remaining 79 children underwent bladder neck reconstruction, with or without augmentation, as their primary continence surgery.
Unsatisfactory
Bladder neck reconstruction without augmentation Bladder neck reconstruction alone, without augmentation, was selected for 32 of the 79 patients. This failed to promote development of capacity and continence in 20 patients and augmentation was subsequently indicated. Upper tract dilatation was a significant factor in the decision for augmentation in 9 of these children. Six of the 32 patients are potentially successful; capacity has progressed but dry intervals currently remain less than 2 h. Five of the remaining 6 patients achieved satisfactory continence, 3 without further surgery and 2 with the AUS. Two of the 5 need CIC, including one with the artificial sphincter (female). The AUS was used in the one additional patient but subsequent removal was required and a Mitrofanoff procedure was performed.
For simplicity in reporting results in this review, the terms “satisfactory” and “unsatisfactory” are used. These 2 general categories have guided management over the years, with the implication that for groups 3 and 4 further surgery may be required.
Results Initial Treatment Ten of the 96 patients in this series were undiverted from ileal or colon conduits and had undergone partial cystectomy. Four of these children achieved a satisfactory state of continence with urethral CIC (3) or voiding with the AUS (1). Four other children were dry but failed urethral CIC and subsequently required the Mitrofanoff procedure. One child is wet and the current status of the other is unknown. The remaining 86 patients (Fig.) had undergone primary bladder closure between the neonatal period and 3 years of age. The later closures were due to a delay in transfer to this centre. Two of these 86 children reached a satisfactory state of continence without further surgery. Five required early augmentation to enable primary bladder closure or for severe upper tract dilatation. As this was not for continence, bladder neck reconstruction was not performed simultaneously. One of these 5 children achieved “serendipitous” voiding contin-
Bladder neck reconstruction with augmentation Bladder neck reconstruction with simultaneous augmentation was the treatment of choice in the other 47 patients, 5 of whom had previously undergone bladder neck reconstruction elsewhere. Upper tract dilatation was a significant factor in the selection of 20 of these patients. These 47 patients, plus the 20 who failed bladder neck reconstruction alone in our centre, plus 4 of the 5 who underwent primary augmentation, comprise the group of 71 patients for whom the combination
’2 Satisfactory no f u r t h e r s u r g e r y
i3*
Potential success
BNRn--( 20
86
71
-
j58J
3
WIL
EElR
+
Augmentation
15
Enhanced
Y 1 Satisfactory
B N R z Bladder n e c k r e c o n s t r u c t i o n .
Fig. Flow diagram of surgical treatment.
546
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of bladder neck reconstruction(s) and augmentation was needed. Three are currently on the waiting list and the other 68 patients have already undergone surgery. Results in 68 patients with bladder neck reconstruction and augmentation are as follows.
tion. Removal of silastic was required for infection or erosion in 9 of 47 patients (20%) in the early years but there have been no problems requiring its removal since March 1986, when the placement technique was modified.
Intermittent catheterisation dificulties. So far, 55 children have attempted urethral CIC. Ten of the 11 females achieved it, although minor meatal revisions were required in 3 of them. The other girl was unable to catheterise owing to angulation of the bladder neck which failed to correct after 3 revisions. Of the 44 males, 18 have achieved CIC without technical problems and 11 with additional endoscopic procedure(s) only; 13 required 1 or more urethral revisions and 2 are on the waiting list for a urethral revision. One failed to achieve urethral CIC and required a Mitrofanoff procedure. Initially unsatisfactory. Good capacity was achieved The majority of the urethral revisions were in boys in 13 patients (20%) but additional surgery was who had their epispadias repair prior to using the needed on account of dry intervals being less than modified technique of ventral urethral placement. 2 h. This included bladder neck revision (2), AUS Eleven of these 44 boys underwent the new (3) and Mitrofanoff (1). Two are waiting for the epispadias repair prior to or at the same time as the AUS, 2 are hoping for further spontaneous improve- continence reconstruction and only 1 has required ment and the present status is unknown for the a urethral revision to enable CIC. other 3. Bladder perforation. Delayed spontaneous perforaEuolution. The Table summarises the current status tion occurred in 7 patients from 0.5 to 5.3 years of continence and emptying for this group of 68 (mean 3.3) after augmentation. The history sugpatients; 43 (60%) are dry and empty by CIC and/ gested that in 6 patients it was related to indirect or voiding. The AUS and Mitrofanoff procedure trauma with a full bladder and one was due to a served as good alternatives in 8 (10%). In 10 other catheterisation injury. children (15%) success is anticipated with CIC (7), AUS (2) or the Mitrofanoff procedure (1). Four Discussion remain wet to varying degrees and are waiting for further spontaneous improvement or evaluation A discussion of undiversion is beyond the scope of this report. The fact that 4 of the 10 undiverted and the current status is unknown for the other 3. patients eventually required the Mitrofanoff proAnatomical complications. Occlusion of the bladder cedure should not discourage reconstruction deneck occurred 6 times in 5 patients, 3 prior to the signed for urethral CIC or the AUS. However, it use of combined omental and silastic wrap and in 3 should be undertaken only with the clear underafter revision of a previous bladder neck reconstruc- standing by the patient and the family that the outcome is less predictable than in patients whose urinary tract remains intact. Our approach to continence reconstruction for Table Bladder Neck Reconstruction and Augmentaexstrophy has changed over the 12 years included tion : Outcome in 68 Patients in this review. In the early years, all patients were Satisfactory CIC/void managed by bladder neck reconstruction alone as Mitrofanoff the initial continence procedure. The high inciAUS dence of upper tract dilatation, the low overall Awaiting CIC ” success rate of achieving at least ‘ ‘ g ~ ~ dquality Unsatisfactory Awaiting AUS continence, as defined in this department, and the Awaiting Mitrofanoff 20% years required to evolve to this state were unsatisWetlunknown I factory. From this experience evolved more rigid Initially satisfactory. Fifty-five patients (80%) were dry after this surgery. Of the 51 who have remained dry, 41 are voiding and/or on CIC, 6 are waiting to learn CIC (with suprapubic catheters on clamp and release regime), and for 4 who failed CIC the Mitrofanoff procedure was selected. The other 4 later regressed and became wet to varying degrees. One has since become dry with a bladder neck revision. One is waiting for a Mitrofanoff procedure and the other 2 are waiting for further evaluation.
;}
SURGICAL MANAGEMENT OF INCONTINENCE IN BLADDER EXSTROPHY
criteria of selection of bladder neck reconstruction without simultaneous augmentation : bladder capacity > 100 ml and no upper tract dilatation. Of the 65 patients presenting for continence surgery since July 1984, only 13 (15%) have met these criteria. Thus the majority of patients have undergone early augmentation. It is important to note that augmentation was not performed to “treat” hydronephrosis. Dilatation of the upper tracts was interpreted as an indication that the potential for bladder expansion in response to increased outflow resistance without further risk of renal damage had been reached. The definition of “satisfactory” continence used in the management of patients in this series is much more rigid than that reported in other reviews of exstrophy reconstruction (Lepor and Jeffs, 1983; Mesrobian et al., 1988; Connor et al., 1989). The child must have 3-h minimum dry intervals with no episodes of wetting in order to be classified as “good”. In fact, continence any less secure than this, with even the occasional accident, has been considered a failure of the reconstructive procedure. It is important to note that minimum dry interval has been used rather than average (Lepor and Jeffs, 1983). Although either may be an appropriate measure by which the clinican can judge progress, it is only the minimum interval that is relevant to the child because it is this by which he/she judges whether or not protection is required. A dry interval of at least 3 h is deemed necessary to satisfy the child, for this allows him/her to cath/void only at breaktime at school when the other children are also being excused. Continence less secure than this demands that the child regularly leave classes in session, wear nappies/pads or have “accidents”. It has been noted by parents and children that it is precisely these problems that invoke verbal abuse from the child’s classmates. Although a thorough psychological assessment has not been undertaken, it has been observed that all of the significant behaviour problems reported to us in this group of children (varying degrees of social withdrawal and aggressive behaviour) have been related to abuse from their peers. The combination of bladder augmentation with bladder neck reconstruction has several advantages over bladder neck reconstruction alone. Adequate capacity is achieved immediately and without risk of renal damage from high bladder pressures. In the majority of patients continence develops immediately and the quality of continence achieved is superior to that eventually achieved by bladder neck reconstruction alone. Of the 68 patients who
547
underwent bladder neck reconstruction and augmentation, 50 (75%) were initially satisfactory and have not regressed; 25 of these even fit the “excellent” category within this already rigid definition of “satisfactory” continence. An excellent state of continence (dry interval > 4 h) affords the child the luxury of catheterising only once during the school day. The primary problems realised with augmentation have been delayed perforations and the inability to void effectively, which demands intermittent catheterisation. Both apprehension and technical difficulties, especially in the reconstructed male urethra, have caused significant delays in achieving CIC, during which time the children have been managed with suprapubic catheters clamped and then released at intervals of 3 to 4 h. Even though the catheters are changed every 4 to 6 weeks the problem with bladder stones has been significant. However, the children seem to cope better socially being dry with a suprapubic catheter on the clamp and release regime than with wearing nappies during the school years, for the reasons discussed above. Several improvements over the last 2 years have helped to alleviate these long delays in achieving urethral CIC. The addition to the management team of a clinical nurse specialist who is committed to CIC instruction and becomes involved with the children and parents prior to the reconstructive surgery has virtually eliminated the delays due to fear. The new epispadias repair has decreased the technical problems in males. In the past there has been a strong prejudice towards urethral catheterisation. However, the patients who eventually had the Mitrofanoff procedure have seemed very happy with this. More recently, several males have been encouraged to have a continent catheterisable stoma (Mitrofanoff procedure) in conjunction with conventional bladder neck reconstruction. This avoids prolonged use of the suprapubic catheter if there is a delay in establishing urethral CIC and provides the child with a choice of catheterisation routes. We may learn that the suprapubic route will be preferred in many males. However, the preservation of the urethral route for endoscopic access may be important in the long term. The AUS has been a useful adjunct to achieving continence in a number of patients but careful selection is critical. Bladder augmentation does not eliminate this as an option but significant instability must be ruled out and, in our opinion, voiding to completion is a prerequisite for males. It appears safe to use urethral CIC in conjunction with the
548
AUS in females (Gonzalez et al., 1989) and we believe that there may be a wider role for its use in the future. The omentum and silastic wrap provide vascularised supple support for the Young-Dees tube and an excellent plane for subsequent placement of a sphincter cuff. It is important to avoid wrapping the silastic directly around the bladder neck but instead loosely around the omentum. The ultimate outcome of reconstruction in patients with bladder exstrophy is a good to excellent state of continence with relatively low morbidity to the upper tracts for all patients, and voiding or intermittent urethral catheterisation (if preferred) for the majority. The price paid has been multiple procedures and admissions to hospital, prolonged use of a suprapubic catheter with its attendant problems while establishing CIC and a risk of delayed bladder perforation. However, these problems have decreased over the period of this review as new developments in management have evolved. Adoption of these new treatments by the surgeon and careful selection for each patient should enable children currently undergoing reconstruction to face a more predictable outcome with a reduced number of surgical procedures and better cosmetic results. The early experience in this department indicated that the potential of the exstrophy bladder without augmentation to provide satisfactory compliant continence is an unrealistic ambition in the majority of cases and early augmentation may therefore be appropriate in a high proportion of them. However, the long-term outcome of bladder augmentation in children with respect to acidosis (Nurse and Mundy, 1988), somatic growth (Wagstaff et al., 1990), malignant potential (Nurse and Mundy, 1989) and delayed perforation (Elder et al., 1988; Rink et aI., 1988)during the course of their lifetime is unknown. Thus it is realised that there is a need for better methods of assessing the potential of the closed exstrophy bladder and more objective criteria for selection for augmentation. Acknowledgements The support for J.G.H. from the St Peter’s Research Trust is gratefully acknowledged. This study was greatly facilitated by the information technology support provided by Sister Fay and Mr L. Watkinson. The dedicated care of Sister Valerie Allen and the nursing staff of ward 6CD at the Hospital for Sick Children, Sister Anne Blight and Sister Sue Keeble and the nursing staff of the Children’s Ward at the Shaftesbury Hospital, has contributed enormously to the welfare of these children. Sister Brid Carr is to be congratulated on her expertise in counselling and intermittent catheterisation instruction which has brought so many children to a successful outcome.
BRITISH JOURNAL OF UROLOGY
References Ansell, J. S. (1979). Surgical treatment of exstrophy of the bladder with emphasis on neonatal primary closure : personal experience with 28 consecutive cases treated at the University of Washington Hospitals from 1962 to 1977: techniques and results. J . Urol., 121,650-653. Connor, J. P., Hensle, T. W., Lather, J. K. et al. (1989). longterm follow-up of 207 patients with bladder exstrophy: an evolution in treatment. J . Urol., 142,793-796. Dees, J. E. (1949). Congenital epispadias with incontinence. J . Urol.,62, 513-522. Diamond, D. A. and Ransley, P. G. (1986). Bladder neck reconstruction with omentum, silicone and augmentation cystoplasty-a preliminary report. J . Urol., 136,252-255. Elder, J. S., Snyder, H. M., Hdbert, W. C. et d. (1988). Perforation of the augmented bladder in patients undergoing clean intermittent catheterisation. J . Urol., 140, 1159-1 162. Gonzalez, R., Koleilat, N., Austin, C. et d.(1989). The artificial sphincter AS800 in congenital urinary incontinence. J . Urol., 142,512-515. Jeffs, R. D., Guice, S. L. and Oesch, I. (1982). The factors in successful bladder closure. J . Urol., 127,974-976. Leadbetter, G. W., Jr. (1964). Surgical correction of total urinary incontinence. J . Urol., 91, 261-266. Lepor, H. and Jeffs, R. D. (1983). Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J . Urol., 130, 1142-1 145. Mesrobian, H. J., Kelalis, P. P. and Kramer, S. A. (1988). Longterm follow-up of 103 patients with bladder exstrophy. J . Urol., 139, 719-722. Mitrofanoff,P. (1980). Cystotomie continente trans-appendiculaire dans le traitement des vessies neurologiques. Chir. Pediatr., 21,297-305. Nurse, D. E. and Mundy, A. R. (1988). Metabolic complications ofcystoplasty. Br. J . Urol.,63, 165-170. Nurse, D. E. and Mundy, A. R. (1989). Assessment of the malignant potential of cystoplasty. Br. J . Urol.,64,489492. Osterling, J. E. and Jeffs, R. D. (1987). The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy : analysis of 144patients treated at the Johns Hopkins Hospital between 1975 and 1985. J . Urol., 137, 258-262. Ransley, P. G., Duffy, P. G. and Wollin, M. (1988). Bladder exstrophy closure and epispadias repair. In Operative Surgery (Paediatric Surgery), ed. Spitz, L. and Nixon, H. H. Pp. 620632. London: Butterworths. Rink, R. C., Woodbury, P. W. and Mitchell, M. E. (1988). Bladder perforation following enterocystoplasty. J . Urol., 139 pt.lI,234A, abstract 285. Wagstaff, K. E., Woodhouse, C. R. J., Duffy, P. G. et aL (1991). Delayed linear growth in children with enterocystoplasties. Br. J . Urol. (In press). Williams, D. I. (1974). Epispadias and exstrophy. In Encyclopedia of Urology, Supplement 15: Urology in Childhood. Pp. 270-279. Berlin : Springer-Verlag. Young, H. H. (1919). An operation for the cure of incontinence of urine. Surg. Gynecol. Obstet., 28, 84-90.
The Authors Jean G. Hollowell, MD, Honorary Registrar in Urology. Philip G. Ransley, MA, FRCS, FAAP(Hon), Consultant Paediatric Urologist. Requests for reprints to: P. G. Ransley, 29 Orde Hall Street, London W Cl N 3JL.
Surgical Management of Incontinence in Bladder Exstrophy* JEAN G. HOLLOWELL and P. G. RANSLEY Department of Urology, Hospital for Sick Children, and the St Peter‘s Hospitals, London
Summary-Between 1978 and 1990,86 patients with previously closed classical bladder exstrophy and 10 patients seeking undiversion have presented for continence management and have undergone selective reconstruction designed for voiding and/or intermittent urethral catheterisation. The reconstruction in these 96 patients has been reviewed. Eight of the 10 patients undergoing undiversion achieved a satisfactory state of continence but 4 required Mitrofanoff procedures to enable catheterisation. Of the other 86 patients, 2 reached a satisfactory state of continence without further surgery; 79 underwent bladder neck surgery for continence either without augmentation (n =32) or with augmentation (n =47). Twenty of the 32 patients who were treated by bladder neck reconstruction alone were later found to require augmentation. Five patients had very early augmentation either to facilitate neonatal closure or on account of severe upper tract dilatation. Of these, 1 became continent without further surgery and 4 demonstrated the need for bladder neck reconstruction. Thus 12 children achieved successful continence (n =6) or are evolving satisfactorily with potential success (n = 6) as a result of bladder neck reconstruction. Of the 71 patients requiring bladder neck reconstruction and augmentation, 68 have completed their surgery. The current status of these patients is: satisfactory in 57 (80%) (42 void/urethral clean intermittent catheterisation (CIC), 7 waiting to learn CIC, 5 Mitrofanoff, 3 artificial urinary sphincter (AUS)). Of the remaining 11 patients (20%), 8 are unsatisfactory to varying degrees and the status of the other 3 is unknown.
Bladder exstrophy remains one of the most difficult surgical challenges in paediatric urology. Historically, most patients underwent urinary diversion after a series of unsuccessful reconstructive surgical procedures. In 1974,D. I. Williams wrote “although functional reconstruction seems the logical treatment and is certainly the one desired by most parents, many urologists are opposed to it on the grounds that the success rate is small, that the children are subjected to multiple procedures and that the complications are by no means negligible with serious urinary infections, pyelonephritis and *Based on a paper presented to the Urological Section of the American Academy of Pediatrics, Annual Meeting, Boston, October 1990 Accepted for publication 19 March 1991
stones” (Williams, 1974). Developments, including neonatal closure and reintroduction of osteotomies (Ansell, 1979; Jeffs et al., 1982; Osterling and Jeffs, 1986), a new single-stageepispadias repair (Ransley et al., 1988), bladder augmentation and clean intermittent catheterisation, have all contributed to the improved results of reconstruction reported over the last decade In this department there has been a firm commitment to reconstruction and no patient with exstrophy has been diverted since 1978. Between 1978 and 1990,86 patients with classical bladder exstrophy (excluding cloaca1 exstrophy, exstrophy variants and epispadias) who have undergone primary bladder closure have presented for continence management. All of these children, plus an additional 10 patients with exstrophy 543
544 presenting for undiversion, have undergone reconstruction designed for voiding and/or intermittent urethral catheterisation. (Undiversions directly to the Mitrofanoff procedure have not been included.) The reconstruction for continence in these 96 patients has been reviewed retrospectively in order to determine the final outcome and the price of this “success”. Patients and Methods Management of exstrophy This report is not a comparison of techniques but rather a discussion of how the evolution of new techniques and the timing of surgical steps have been incorporated into continence management of exstrophy by a single surgeon over the last 12 years. The general philosophy of exstrophy reconstruction over this period has been early bladder closure and annual follow-up until commencement of continence surgery after 4 years of age. In the late 1970s bladder closure was performed in the first few weeks of life without osteotomies. Subsequently, this evolved to closure without osteotomies limited to the first 24 hours of life and any delayed closures performed with posterior vertical osteotomies. Following successful closure, annual follow-up consists of cystoscopy and cystography under anaesthesia and ultrasound to assess the development of bladder capacity and status of the upper tracts. Surgery for continence is undertaken after 4 years of age and once further spontaneous expansion of the bladder appears to have ceased. Techniques The principal technique of bladder neck reconstruction has been a 2-layer Young-Dees-Leadbetter procedure (Young, 1919; Dees, 1949; Leadbetter, 1964). Initially performed without modification, it was subsequently altered by the placement of a single sheet of silicone around the reconstructed bladder neck (Diamond and Ransley, 1986). Early patients suffered from erosion of the silicone as previously documented. This led to the current practice of a circumferential omental wrap of the Young-Dees tube and a single layer of silicone Silastic sheet-0.01 in. non-reinforced (Dow Corning. Midlands, USA) loosely arranged around the omentum. Bladder augmentation has largely been by detubularised cup colocystoplasty; 15 to 30 cm of
BRITISH JOURNAL OF UROLOGY
descending/sigmoid colon were isolated, detubularised and reconstructed as a “cup” before anastomosis to the small bladder element bearing the reimplanted ureters. In those patients in whom a continent catheterisable stoma had been performed (Mitrofanoff procedure) (Mitrofanoff, 1980),it was created either by using the appendix or distal ureter in conjunction with a transuretero-ureterostomy. Bladder neck closure was performed only if necessary to prevent persistent incontinence. The single-stage epispadias repair with ventral urethral placement and corporal rotation (Ransley et al., 1988) has been used since 1985 and most of the earlier patients have undergone revision by this method. The artificial urinary sphincter models used were the AMS-792 and the AMS-800. Exchange of the sphincter cuff for the silicone wrap facilitates its placement.
Assessment of continence Obtaining an accurate history of continence status is challenging. Descriptive terms have different meanings for different patients and the history may vary from child to parent. Extensive and diligent questioning was carried out in personal interviews in order to make an accurate assessment of continence during the day and at night, at rest and during activity, and to explore the methods employed to cope with each child. It is important to note that the term “cath/void interval” is used to imply the minimum interval for emptying which will insure that the child will always be dry, not the auerage dry interval. The state of continence of this group of patients covers a wide spectrum and categorisation is somewhat arbitrary. After reviewing the assessments certain patterns appeared and from these the following classification was derived.
1. Excellent : -dry day/night/activity -cath/void intervals > 4 h -no restrictions : activity or fluid intake 2. Good: -dry day/activity -some night-time wetting -cath/void intervals > 3 h -minor coping methods employed
’
Satisfactory
545
SURGICAL MANAGEMENT OF INCONTINENCE IN BLADDER EXSTROPHY
3. Poor: -dry intervals not > 2 h without major effort -protection required 4. Wet: -continuous leaking
I
ence without additional surgery. Three of the 5 subsequently underwent bladder neck reconstruction and I is awaiting further surgery for continence. The remaining 79 children underwent bladder neck reconstruction, with or without augmentation, as their primary continence surgery.
Unsatisfactory
Bladder neck reconstruction without augmentation Bladder neck reconstruction alone, without augmentation, was selected for 32 of the 79 patients. This failed to promote development of capacity and continence in 20 patients and augmentation was subsequently indicated. Upper tract dilatation was a significant factor in the decision for augmentation in 9 of these children. Six of the 32 patients are potentially successful; capacity has progressed but dry intervals currently remain less than 2 h. Five of the remaining 6 patients achieved satisfactory continence, 3 without further surgery and 2 with the AUS. Two of the 5 need CIC, including one with the artificial sphincter (female). The AUS was used in the one additional patient but subsequent removal was required and a Mitrofanoff procedure was performed.
For simplicity in reporting results in this review, the terms “satisfactory” and “unsatisfactory” are used. These 2 general categories have guided management over the years, with the implication that for groups 3 and 4 further surgery may be required.
Results Initial Treatment Ten of the 96 patients in this series were undiverted from ileal or colon conduits and had undergone partial cystectomy. Four of these children achieved a satisfactory state of continence with urethral CIC (3) or voiding with the AUS (1). Four other children were dry but failed urethral CIC and subsequently required the Mitrofanoff procedure. One child is wet and the current status of the other is unknown. The remaining 86 patients (Fig.) had undergone primary bladder closure between the neonatal period and 3 years of age. The later closures were due to a delay in transfer to this centre. Two of these 86 children reached a satisfactory state of continence without further surgery. Five required early augmentation to enable primary bladder closure or for severe upper tract dilatation. As this was not for continence, bladder neck reconstruction was not performed simultaneously. One of these 5 children achieved “serendipitous” voiding contin-
Bladder neck reconstruction with augmentation Bladder neck reconstruction with simultaneous augmentation was the treatment of choice in the other 47 patients, 5 of whom had previously undergone bladder neck reconstruction elsewhere. Upper tract dilatation was a significant factor in the selection of 20 of these patients. These 47 patients, plus the 20 who failed bladder neck reconstruction alone in our centre, plus 4 of the 5 who underwent primary augmentation, comprise the group of 71 patients for whom the combination
’2 Satisfactory no f u r t h e r s u r g e r y
i3*
Potential success
BNRn--( 20
86
71
-
j58J
3
WIL
EElR
+
Augmentation
15
Enhanced
Y 1 Satisfactory
B N R z Bladder n e c k r e c o n s t r u c t i o n .
Fig. Flow diagram of surgical treatment.
546
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of bladder neck reconstruction(s) and augmentation was needed. Three are currently on the waiting list and the other 68 patients have already undergone surgery. Results in 68 patients with bladder neck reconstruction and augmentation are as follows.
tion. Removal of silastic was required for infection or erosion in 9 of 47 patients (20%) in the early years but there have been no problems requiring its removal since March 1986, when the placement technique was modified.
Intermittent catheterisation dificulties. So far, 55 children have attempted urethral CIC. Ten of the 11 females achieved it, although minor meatal revisions were required in 3 of them. The other girl was unable to catheterise owing to angulation of the bladder neck which failed to correct after 3 revisions. Of the 44 males, 18 have achieved CIC without technical problems and 11 with additional endoscopic procedure(s) only; 13 required 1 or more urethral revisions and 2 are on the waiting list for a urethral revision. One failed to achieve urethral CIC and required a Mitrofanoff procedure. Initially unsatisfactory. Good capacity was achieved The majority of the urethral revisions were in boys in 13 patients (20%) but additional surgery was who had their epispadias repair prior to using the needed on account of dry intervals being less than modified technique of ventral urethral placement. 2 h. This included bladder neck revision (2), AUS Eleven of these 44 boys underwent the new (3) and Mitrofanoff (1). Two are waiting for the epispadias repair prior to or at the same time as the AUS, 2 are hoping for further spontaneous improve- continence reconstruction and only 1 has required ment and the present status is unknown for the a urethral revision to enable CIC. other 3. Bladder perforation. Delayed spontaneous perforaEuolution. The Table summarises the current status tion occurred in 7 patients from 0.5 to 5.3 years of continence and emptying for this group of 68 (mean 3.3) after augmentation. The history sugpatients; 43 (60%) are dry and empty by CIC and/ gested that in 6 patients it was related to indirect or voiding. The AUS and Mitrofanoff procedure trauma with a full bladder and one was due to a served as good alternatives in 8 (10%). In 10 other catheterisation injury. children (15%) success is anticipated with CIC (7), AUS (2) or the Mitrofanoff procedure (1). Four Discussion remain wet to varying degrees and are waiting for further spontaneous improvement or evaluation A discussion of undiversion is beyond the scope of this report. The fact that 4 of the 10 undiverted and the current status is unknown for the other 3. patients eventually required the Mitrofanoff proAnatomical complications. Occlusion of the bladder cedure should not discourage reconstruction deneck occurred 6 times in 5 patients, 3 prior to the signed for urethral CIC or the AUS. However, it use of combined omental and silastic wrap and in 3 should be undertaken only with the clear underafter revision of a previous bladder neck reconstruc- standing by the patient and the family that the outcome is less predictable than in patients whose urinary tract remains intact. Our approach to continence reconstruction for Table Bladder Neck Reconstruction and Augmentaexstrophy has changed over the 12 years included tion : Outcome in 68 Patients in this review. In the early years, all patients were Satisfactory CIC/void managed by bladder neck reconstruction alone as Mitrofanoff the initial continence procedure. The high inciAUS dence of upper tract dilatation, the low overall Awaiting CIC ” success rate of achieving at least ‘ ‘ g ~ ~ dquality Unsatisfactory Awaiting AUS continence, as defined in this department, and the Awaiting Mitrofanoff 20% years required to evolve to this state were unsatisWetlunknown I factory. From this experience evolved more rigid Initially satisfactory. Fifty-five patients (80%) were dry after this surgery. Of the 51 who have remained dry, 41 are voiding and/or on CIC, 6 are waiting to learn CIC (with suprapubic catheters on clamp and release regime), and for 4 who failed CIC the Mitrofanoff procedure was selected. The other 4 later regressed and became wet to varying degrees. One has since become dry with a bladder neck revision. One is waiting for a Mitrofanoff procedure and the other 2 are waiting for further evaluation.
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criteria of selection of bladder neck reconstruction without simultaneous augmentation : bladder capacity > 100 ml and no upper tract dilatation. Of the 65 patients presenting for continence surgery since July 1984, only 13 (15%) have met these criteria. Thus the majority of patients have undergone early augmentation. It is important to note that augmentation was not performed to “treat” hydronephrosis. Dilatation of the upper tracts was interpreted as an indication that the potential for bladder expansion in response to increased outflow resistance without further risk of renal damage had been reached. The definition of “satisfactory” continence used in the management of patients in this series is much more rigid than that reported in other reviews of exstrophy reconstruction (Lepor and Jeffs, 1983; Mesrobian et al., 1988; Connor et al., 1989). The child must have 3-h minimum dry intervals with no episodes of wetting in order to be classified as “good”. In fact, continence any less secure than this, with even the occasional accident, has been considered a failure of the reconstructive procedure. It is important to note that minimum dry interval has been used rather than average (Lepor and Jeffs, 1983). Although either may be an appropriate measure by which the clinican can judge progress, it is only the minimum interval that is relevant to the child because it is this by which he/she judges whether or not protection is required. A dry interval of at least 3 h is deemed necessary to satisfy the child, for this allows him/her to cath/void only at breaktime at school when the other children are also being excused. Continence less secure than this demands that the child regularly leave classes in session, wear nappies/pads or have “accidents”. It has been noted by parents and children that it is precisely these problems that invoke verbal abuse from the child’s classmates. Although a thorough psychological assessment has not been undertaken, it has been observed that all of the significant behaviour problems reported to us in this group of children (varying degrees of social withdrawal and aggressive behaviour) have been related to abuse from their peers. The combination of bladder augmentation with bladder neck reconstruction has several advantages over bladder neck reconstruction alone. Adequate capacity is achieved immediately and without risk of renal damage from high bladder pressures. In the majority of patients continence develops immediately and the quality of continence achieved is superior to that eventually achieved by bladder neck reconstruction alone. Of the 68 patients who
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underwent bladder neck reconstruction and augmentation, 50 (75%) were initially satisfactory and have not regressed; 25 of these even fit the “excellent” category within this already rigid definition of “satisfactory” continence. An excellent state of continence (dry interval > 4 h) affords the child the luxury of catheterising only once during the school day. The primary problems realised with augmentation have been delayed perforations and the inability to void effectively, which demands intermittent catheterisation. Both apprehension and technical difficulties, especially in the reconstructed male urethra, have caused significant delays in achieving CIC, during which time the children have been managed with suprapubic catheters clamped and then released at intervals of 3 to 4 h. Even though the catheters are changed every 4 to 6 weeks the problem with bladder stones has been significant. However, the children seem to cope better socially being dry with a suprapubic catheter on the clamp and release regime than with wearing nappies during the school years, for the reasons discussed above. Several improvements over the last 2 years have helped to alleviate these long delays in achieving urethral CIC. The addition to the management team of a clinical nurse specialist who is committed to CIC instruction and becomes involved with the children and parents prior to the reconstructive surgery has virtually eliminated the delays due to fear. The new epispadias repair has decreased the technical problems in males. In the past there has been a strong prejudice towards urethral catheterisation. However, the patients who eventually had the Mitrofanoff procedure have seemed very happy with this. More recently, several males have been encouraged to have a continent catheterisable stoma (Mitrofanoff procedure) in conjunction with conventional bladder neck reconstruction. This avoids prolonged use of the suprapubic catheter if there is a delay in establishing urethral CIC and provides the child with a choice of catheterisation routes. We may learn that the suprapubic route will be preferred in many males. However, the preservation of the urethral route for endoscopic access may be important in the long term. The AUS has been a useful adjunct to achieving continence in a number of patients but careful selection is critical. Bladder augmentation does not eliminate this as an option but significant instability must be ruled out and, in our opinion, voiding to completion is a prerequisite for males. It appears safe to use urethral CIC in conjunction with the
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AUS in females (Gonzalez et al., 1989) and we believe that there may be a wider role for its use in the future. The omentum and silastic wrap provide vascularised supple support for the Young-Dees tube and an excellent plane for subsequent placement of a sphincter cuff. It is important to avoid wrapping the silastic directly around the bladder neck but instead loosely around the omentum. The ultimate outcome of reconstruction in patients with bladder exstrophy is a good to excellent state of continence with relatively low morbidity to the upper tracts for all patients, and voiding or intermittent urethral catheterisation (if preferred) for the majority. The price paid has been multiple procedures and admissions to hospital, prolonged use of a suprapubic catheter with its attendant problems while establishing CIC and a risk of delayed bladder perforation. However, these problems have decreased over the period of this review as new developments in management have evolved. Adoption of these new treatments by the surgeon and careful selection for each patient should enable children currently undergoing reconstruction to face a more predictable outcome with a reduced number of surgical procedures and better cosmetic results. The early experience in this department indicated that the potential of the exstrophy bladder without augmentation to provide satisfactory compliant continence is an unrealistic ambition in the majority of cases and early augmentation may therefore be appropriate in a high proportion of them. However, the long-term outcome of bladder augmentation in children with respect to acidosis (Nurse and Mundy, 1988), somatic growth (Wagstaff et al., 1990), malignant potential (Nurse and Mundy, 1989) and delayed perforation (Elder et al., 1988; Rink et aI., 1988)during the course of their lifetime is unknown. Thus it is realised that there is a need for better methods of assessing the potential of the closed exstrophy bladder and more objective criteria for selection for augmentation. Acknowledgements The support for J.G.H. from the St Peter’s Research Trust is gratefully acknowledged. This study was greatly facilitated by the information technology support provided by Sister Fay and Mr L. Watkinson. The dedicated care of Sister Valerie Allen and the nursing staff of ward 6CD at the Hospital for Sick Children, Sister Anne Blight and Sister Sue Keeble and the nursing staff of the Children’s Ward at the Shaftesbury Hospital, has contributed enormously to the welfare of these children. Sister Brid Carr is to be congratulated on her expertise in counselling and intermittent catheterisation instruction which has brought so many children to a successful outcome.
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References Ansell, J. S. (1979). Surgical treatment of exstrophy of the bladder with emphasis on neonatal primary closure : personal experience with 28 consecutive cases treated at the University of Washington Hospitals from 1962 to 1977: techniques and results. J . Urol., 121,650-653. Connor, J. P., Hensle, T. W., Lather, J. K. et al. (1989). longterm follow-up of 207 patients with bladder exstrophy: an evolution in treatment. J . Urol., 142,793-796. Dees, J. E. (1949). Congenital epispadias with incontinence. J . Urol.,62, 513-522. Diamond, D. A. and Ransley, P. G. (1986). Bladder neck reconstruction with omentum, silicone and augmentation cystoplasty-a preliminary report. J . Urol., 136,252-255. Elder, J. S., Snyder, H. M., Hdbert, W. C. et d. (1988). Perforation of the augmented bladder in patients undergoing clean intermittent catheterisation. J . Urol., 140, 1159-1 162. Gonzalez, R., Koleilat, N., Austin, C. et d.(1989). The artificial sphincter AS800 in congenital urinary incontinence. J . Urol., 142,512-515. Jeffs, R. D., Guice, S. L. and Oesch, I. (1982). The factors in successful bladder closure. J . Urol., 127,974-976. Leadbetter, G. W., Jr. (1964). Surgical correction of total urinary incontinence. J . Urol., 91, 261-266. Lepor, H. and Jeffs, R. D. (1983). Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J . Urol., 130, 1142-1 145. Mesrobian, H. J., Kelalis, P. P. and Kramer, S. A. (1988). Longterm follow-up of 103 patients with bladder exstrophy. J . Urol., 139, 719-722. Mitrofanoff,P. (1980). Cystotomie continente trans-appendiculaire dans le traitement des vessies neurologiques. Chir. Pediatr., 21,297-305. Nurse, D. E. and Mundy, A. R. (1988). Metabolic complications ofcystoplasty. Br. J . Urol.,63, 165-170. Nurse, D. E. and Mundy, A. R. (1989). Assessment of the malignant potential of cystoplasty. Br. J . Urol.,64,489492. Osterling, J. E. and Jeffs, R. D. (1987). The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy : analysis of 144patients treated at the Johns Hopkins Hospital between 1975 and 1985. J . Urol., 137, 258-262. Ransley, P. G., Duffy, P. G. and Wollin, M. (1988). Bladder exstrophy closure and epispadias repair. In Operative Surgery (Paediatric Surgery), ed. Spitz, L. and Nixon, H. H. Pp. 620632. London: Butterworths. Rink, R. C., Woodbury, P. W. and Mitchell, M. E. (1988). Bladder perforation following enterocystoplasty. J . Urol., 139 pt.lI,234A, abstract 285. Wagstaff, K. E., Woodhouse, C. R. J., Duffy, P. G. et aL (1991). Delayed linear growth in children with enterocystoplasties. Br. J . Urol. (In press). Williams, D. I. (1974). Epispadias and exstrophy. In Encyclopedia of Urology, Supplement 15: Urology in Childhood. Pp. 270-279. Berlin : Springer-Verlag. Young, H. H. (1919). An operation for the cure of incontinence of urine. Surg. Gynecol. Obstet., 28, 84-90.
The Authors Jean G. Hollowell, MD, Honorary Registrar in Urology. Philip G. Ransley, MA, FRCS, FAAP(Hon), Consultant Paediatric Urologist. Requests for reprints to: P. G. Ransley, 29 Orde Hall Street, London W Cl N 3JL.
