Journal of Pediatric Surgery 49 (2014) 244–250

Contents lists available at ScienceDirect

Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg

Isabella Forshall Lecture

The Management of Bladder Exstrophy: The Manchester Experience Alan P. Dickson ⁎ Department of Paediatric Urology, Royal Manchester Children’s Hospital, Oxford Road, Manchester M13 9WL, United Kingdom

a r t i c l e

i n f o

Article history: Received 29 October 2013 Accepted 9 November 2013 Key words: Bladder exstrophy Centralisation of surgical services

a b s t r a c t Aim: Bladder exstrophy (BE) is a severe congenital malformation with life-long implications. This article discusses the current surgical management and describes the development of the nationally commissioned bladder exstrophy service in Manchester, UK. Methods: Outcome of BE surgery in Manchester was retrospectively reviewed. A Medline search was also undertaken and the published outcomes reviewed for the Modern Staged Repair of Bladder Exstrophy (MSRE), the Complete Primary Repair of Exstrophy (CPRE), and Radical Soft-Tissue Mobilisation (RSTM). Results: Sixty-seven infants with BE were treated in the period 2000–2012. Twenty-six infants underwent primary closure during the neonatal period, and in twenty-one this was successful. The remaining forty-one infants underwent delayed closure, and all were successful. Twenty-six children underwent MSRE, and continence with urethral micturition was achieved in sixteen (62%) (ten alone and six with urethral clean intermittent catheterisation). A further five (19%) are continent following bladder neck closure, cystoplasty, and continent diversion. Seven (26%) of the twenty-six patients are completely dry overnight. Twenty infants underwent primary ureteric reimplantation, and none have renal scarring. By contrast, renal scarring (unilateral n = fourteen; bilateral n = five) was found in nineteen of thirty-seven infants who did not undergo reimplantation. Conclusions: Specialised experience has allowed demonstrable improvement in bladder exstrophy outcomes throughout the period of the study. © 2014 Elsevier Inc. All rights reserved.

Bladder exstrophy (BE) is a rare, serious congenital abnormality. Many would say that it is the most severe of those congenital anomalies which do not affect the central nervous system. This is because of its implications on long-term bladder function, genital appearance, sexual function and its associated psychological impact (Figs. 1 and 2; Tables 1–2). The British Association of Paediatric Surgeons conducted a collective review of BE practice and outcomes, published in 1991 [1]. The experience of eight of the 12 centres in the UK and Ireland which looked after bladder exstrophy patients at that time contributed. Even with the best interpretation, urinary continence rates were only 5%–12% (Table 1). Great Ormond Street Hospital, London published its own data in 1991 [2] from a mixed group of primary neonatal (n = 86) and secondary (n = 10) referrals. Though the figures are difficult to interpret; three children seemed to have satisfactory voiding continence after successful primary closure (using closure combined with augmentation in one), three children following bladder neck continence surgery, and six had potential satisfactory voiding continence but dry intervals were b 2 h. In total, therefore, at best interpretation, voiding continence outcomes were again 7%–14%. These results were disappointing to the paediatric urologists and paediatric surgeons involved and revealed that ⁎ Tel.: +44 1617011635; fax: +44 1617012630. E-mail address: [email protected] 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.11.031

continence outcomes for British children with BE were not good. This stimulated some centres, such as Manchester [3], to opt for salvage procedures which produced urinary dryness on the basis of Mitrofanoff clean intermittent catheterisation (CIC) regimens applied to augmented large capacity bladders which had had the bladder neck surgically closed, or narrowed to such an extent as to be effectively closed (Table 2). Bladder augmentation, bladder neck closure and Mitrofanoff clean intermittent catheterisation (CIC) regimens were seen to transform the lives of children with bladder exstrophy in the 1990s. This was a very exciting phase in paediatric urology because for the first time we could virtually guarantee dryness to the parents of children with BE. In the late 1990s however, something quite unexpected and dramatic happened to British paediatric surgery. The Department of Health in England and Wales decided to rationalise the surgery of biliary atresia to only three centres — Leeds, Birmingham and Kings College Hospital in London, following high profile television programmes and a media campaign. The aim of rationalisation was to increase the experience of the care and surgery of children with biliary atresia in a small number of centres, in accord with the evidence published by McKiernan PJ et al. (2000) [4] and the general evidence in the surgical literature that high volume centres produced better surgical outcomes in complex conditions [5–7]. The British Association of Paediatric Urologists (BAPU) took note of this development and approached the Department of Health in

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250

Fig. 1. Features of classic bladder exstrophy (top male; bottom female): low umbilicus, exposed small bladder and bladder neck, pubis diastasis, rectus and pelvic floor divarication, anterior ectopia of anus and/or vagina and epispadias or bifid clitoris.

245

1998 with the request that a similar rationalisation be applied to BE and related conditions (e.g. epispadias and cloacal exstrophy). This resulted in Great Ormond Street Hospital in London and the Royal Manchester Children’s Hospital becoming responsible for the care of all children in England who suffered problems within the Bladder Exstrophy Epispadias Complex (BEEC) spectrum from 2000 onwards. There was (and still remains) however a significant difference between the biliary atresia and bladder exstrophy situations. When biliary atresia was rationalised, there was uniform agreement worldwide regarding as to how the condition should be managed. A Kasai portoenterostomy performed “early” is optimum, with liver transplant being utilised later should this primary drainage procedure fail [8,9]. Most paediatric surgeons had experienced successful Kasai procedures in their own practice but no such uniformity of opinion or success had been reached in the field of BE reconstruction. Indeed, until relatively recently, BE was often associated with early deaths due to renal damage and malfunction. Modern planned BE management really didn’t start until pioneered by Robert Jeffs in the 1970s at the Johns Hopkins Hospital, Baltimore, USA [10,11]. That centre remains probably the leading BE centre in the world and certainly has produced the majority of published literature on this subject, now in excess of 200 papers. From the outset, they advised a multi-procedure “staged” approach which with a number of variations is now known as the Modern Staged Repair (MSRE) [12,13]. However, other centres have struggled to reproduce their outcomes and even though these children undergo multiple surgical procedures most centres have found that the majority never gain volitional voiding continence. Furthermore, the nature of the tubular bladder neck, reconstructed from the trigone as a continence mechanism in the MSRE does not create an impression of achieving a physiological sphincter and normal bladder function. These issues have therefore stimulated others to consider other surgical approaches in attempts to provide more physiological function (e.g. Mitchell Complete Primary

Fig. 2. Pelvic view of Mitchell Complete Primary Repair of Exstrophy (CPRE). (Published with kind permission of R Grady, Seattle Childrens Hospital).

246

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250

Table 1 BAPS Collective Review of bladder exstrophy: 1991 (n = 80). N = 80 Dry interval N 3 h

6

Dry interval N 2 h Maximum voided volume N 200 ml Maximum voided volume b 100 ml Urinary diversion Urinary diversion b 2 years of age

4 1

Two had undergone bladder augmentation

14 37 20

Repair of Exstrophy (CPRE) [14] and the Kelly Radical Soft-tissue Mobilisation (RSTM) [15]) and others again to advise primary urinary diversion (e.g. Mainz ureterosigmoidostomy [16,17] and the Cologne pouch [18]) in the hope of avoiding the need for multiple surgeries and potential upper tract deterioration. In this paper I will describe the techniques of the MSRE, CPRE and the KRSM and focus on the outcome of each as they have developed in the literature. The development of the Manchester BE service to date will then be discussed. As I remain committed to the concept of achieving normal volitional voiding continence for children with BE, there is no discussion of primary diversion as definitive management.

1. Modern Staged Repair of Bladder Exstrophy (MSRE) The Modern Staged Repair of Bladder Exstrophy [12,13], as its name suggests, consists of different surgical steps, each dealing with different aspects of the problem. All surgery in this condition is difficult and hazardous, and the important thinking behind the staged approach includes reduction of risk to both the kidneys and the phallus by minimising the magnitude of the surgical assault by dividing the reconstruction into two or three operations [19]. The MSRE is seen by its supporters as the “safe” approach. The initial staged approach was bladder closure, bladder neck repair and then finally epispadias repair. The MSRE however has altered that order. The bladder exstrophy is closed in the early days of life, sometimes with or without pelvic osteotomy to facilitate closure of the pubic diastasis. In the girl, this is effectively a complete repair but without formal bladder neck functional reconstruction. In the boy however, the primary operation leaves the epispadias unrepaired, and the epispadias and complete urethral reconstruction is then completed around the age of one year. There is some evidence that epispadias repair enhances bladder development [20]. The third stage, bladder neck reconstruction (BNR) is performed only if the bladder develops a satisfactory capacity, thought to be at least 100 ml. This operation combines two key components; bilateral ureteric reimplantation to correct vesico-ureteric reflux and bladder neck reconstruction (BNR), performed by layered tubularisation of trigonal tissue cephalad to the internal urethral meatus, in an attempt to provide bladder outlet resistance and sphincter function. The timing of this in practice is very variable, being critically dependent on both the child’s and parents’ ability to cooperate with the required training programme and the possibility of prolonged intermittent catheterisation (CIC). Ideally, the aim is to achieve this surgery by school age, but in practice it is sometimes

Table 2 Manchester Bladder Exstrophy 1990–2000 (n = 23). • 1/23 dry voiding + Clean Intermittent Catheterisation (CIC). • 22 dry on CIC after Bladder Augmentation/CIC/Mitrofanoff.

later. One of the drawbacks of this approach therefore is that even when successful, the child will not achieve “dryness” until after starting formal education. This and the requirement for multiple operations are the two major criticisms of the MSRE. The outcomes of MSRE have been difficult to define and detailed results have been sparse in the literature over the past twenty years. Furthermore, different authors describe their results using different outcome variables. As might be expected, the best results have been reported from Johns Hopkins Hospital most recently in 2007 [12] in boys and 2008 [13] in girls. They report that 47/67 boys (70%) with ≥ 5 years of follow-up are dry day and night following neonatal closure, a Cantwell–Ransley epispadias repair and BNR, without need for augmentation or CIC. A further 7 boys (10%) were dry by day but damp at night. Thirteen boys remained completely incontinent. Similar results were reported in 41 girls [13] with the figures being 74%, 10% and 16% respectively. Surer et al. [21] reported what are probably the best continence results in BE ever, from a cohort of children who had had their BNR at Baltimore following their initial bladder closure at another hospital. Thus, 83% (56/68) achieved continence with urethral micturition without having had augmentation or the need for CIC. Shaw et al. [22] from Indiana, USA have also reported excellent outcomes from the MSRE in 2004, achieving continence in 85% of children. They described continence as dryness for N 3 h, no stress incontinence and dryness at night. These results, on first assessment, seem excellent, but within this continent group 69% have required bladder augmentations and 67% utilise CIC. In other words, their continence was not volitional voiding continence, and further analysis shows that only 23% of the original group (11/48) achieved this successful goal. Mouriquand et al. [23] reported in 2003 45% (36/80) of patients dry after BNR and 89% dry after augmentation, Mitrofanoff and secondary bladder neck procedures. Lottmann [24] reported only 21% of their patients achieving continence with urethral micturition after BNR following a mean of 12 years of follow-up. Other groups report variable outcomes of between 12% and 40%. Outside of Johns Hopkins Hospital, where the technique was developed, it seems therefore that the usual achievement in relation to normal continence with urethral micturition is on average around 22%. Most of the follow-up attention has been directed to continence outcomes because of the nature of the abnormality. BE patients however commonly suffer psychological sequelae secondary no doubt to their incontinence issues, their abnormal genital appearance and their sexual concerns. Authors from various centres have reported these issues, including from Johns Hopkins who have also reported the best continence outcomes. Reiner et al. [25] has found that 20/20 displayed 1 anxiety disorder and 19/20 displayed two. Ebert et al. [26] reported greater than 90% of males have serious penile concerns and actively wish psychological support. There is a relative paucity of information regarding other aspects of outcome in the contemporary literature. It is generally agreed however that damage to the penis in the form of partial or complete loss of the glans or corporal bodies occurs rarely following MSRE and probably should never occur. Information regarding renal follow-up is also disappointing and perhaps surprising in the context of the known reflux problems that these children have. Hussmann et al. showed that 90% of factors predisposing to renal scarring following staged reconstruction of classical BE were related to vesico-ureteric reflux [27]. These children are at significant risk of renal scarring but there are limited data available. Shaw et al. [22] reported that 90% of their patients have normal ultrasound scans and Schaeffer et al. [28] published this year data based on eGFRs suggesting normal renal outcomes, but this does not reveal an accurate assessment of renal damage because change in GFR is a relatively late change as kidneys deteriorate. As eGFR detects only significant changes, surgical reconstruction may still cause more subtle renal damage.

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250

2. Mitchell Complete Primary Repair of Exstrophy (CPRE) Functional assessment of bladders following MSRE repair indicated that normal physiological function was not the norm. The BNR seemed to produce a fixed bladder outlet obstruction which sometimes balanced with intravesical pressure, thereby achieving dryness. The penile disassembly technique was first reported by Mitchell et al. in 1996 [29] in relation to epispadias repair, and then expanded fully into the CPRE and reported in 1999 [14]. The procedure is based on the principle that BE represents anterior bladder herniation and therefore, repair should be based on repositioning the bladder posteriorly into the pelvis. The bladder, bladder neck and urethra are considered a single unit and the operation does not include bladder neck reconstruction. Mitchell et al. [28] reported the penile disassembly technique for epispadias, and demonstrated that the penis can be separated into its three components, the two corpus cavernosum bodies and the corpus spongiosum, which can later be reassembled. This technique facilitates complete anatomical reconstruction of the bladder in the newborn period by optimizing surgical mobilization of the proximal urethra and bladder neck by allowing visualisation and incision of the intersymphyseal ligaments, thereby causing the bladder and urethra to shift posteriorly into the pelvis and allowing the reconstruction of the anterior perineal muscular complex around the posterior urethra as an external urethral sphincter. The concept of this clever operation is quite difficult to visualise and understand without actually seeing it — suffice it to say that the disassembly of the penile bodies provides exposure of the anterior pelvic floor, otherwise not seen, and thereby facilitates its reconstruction. One of the major drawbacks of the procedure however is its requirement to sacrifice the urethral plate in up to 50% of boys and accept a proximal hypospadiac external urethral meatus as the primary outcome. The concept of a normally functioning pelvic floor and sphincter in bladder exstrophy however was attractive to many paediatric urologists. Investigators [30] reported exciting evidence of physiological bladder function based on formal urodynamic assessment, something not previously achieved in BE children. Long-term outcomes of the CPRE were eagerly awaited but unfortunately are disappointing. Hammouda and Kotb [31] reported in 2004 a continence rate of 74% in a group of 33 patients with very low incidence of serious complications. Almost 50% of this continent group however had not reached the age of toilet training which cast significant doubts on the reliability of the data. Unfortunately there are no longer term data that have been published from this group of patients. The group referred to above from Boston Children’s Hospital, USA reviewed their experience of 32 CPRE procedures in 2007. Only 19% of children older than 4 years were dry after CPRE alone [32]. Mitchell’s own group reported in 2008 [33] on 39 children, and of those who had reached toilet training age, 74% had achieved continence intervals of 2 h but, 86% had undergone bladder neck surgery after the CPRE. Most disappointing of all, in Shoukry et al.’s group [34] of 51 children, 15.6% had failed closures and not one patient achieved dryness after CPRE, all eventually requiring bladder augmentation and CIC regimens. It also became clear the upper tracts were not immune from complications, many children developing hydronephrosis and renal concerns [34,35]. Our own limited experience of CPRE in Manchester has been mixed. We have one child completely continent and normal but the other six have required further major continence procedures and 50% developed significant renal concerns during follow-up. The evidence from the literature does not suggest any improvement in outcome compared to the staged repair. Although a few children achieve normality after CPRE alone, the majority require multiple operations — in other words, several stages including a bladder neck repair. Furthermore, as time passed it became clear that sometimes there was a serious price to pay for the

247

required radical penile dissection. Sporadic reports of penile atrophy [36,37] were followed by papers first from a multicentre study by Cervellione et al. [38] reporting 23 children suffering significant penile injury following CPRE – and then from Baltimore, also reporting patients who had suffered penile loss [39] – clearly a huge insult to a child already compromised by the original anomaly. It is sad that there are few detailed recent long-term outcomes of CPRE in the literature in the last four years, and perhaps indicative of the realisation that unfortunately this intelligent attempt to achieve a physiological bladder may not after all be a success. 3. Kelly Radical Soft-tissue Mobilisation (RSTM) The Kelly repair is a multi-staged repair, the concept again directed at achieving normal pelvic floor and sphincteric function. The exstrophy is closed in the neonatal period without osteotomy. There then follows a second stage around the age of 9 months when a radical dissection of the pelvic floor muscles along with the pudendal pedicles off the pubic rami takes place in order to facilitate a tension free reconstruction of the pelvic floor around the posterior urethra. The boys are left with a penoscrotal hypospadiac external urethral meatus which is then corrected utilising a two stage Bracka type repair. The technique was a development [15] in Melbourne Children’s Hospital, Australia of that first described by Ansell [40] in 1979. Reports about patient outcomes have been sparse. The Melbourne group, reported excellent initial results in 1995, [15] 73% of patients achieving continence. Long term results were presented in 2007 [41] and reported in 2009 [42]. Only 19% of patients were continent (defined as daytime dry intervals N 3 h with ≤ 2 wet nights per month). Another 13% required bladder augmentation and CIC and were dry but the rest were incontinent. Furthermore, cosmetic outcomes were generally poor, particularly related to the protuberant uncorrected diastased pubic bones producing an unpleasant pubogenital impression. Unfortunately, there is no information regarding penile functional outcomes, a matter of great interest because of the radical pudendal dissection. In contrast, Cuckow et al. from Great Ormond Street Hospital, London has reported in abstract form exceptional outcomes from RSTM [43], the last abstract in 2010 indicating 73% of children completely normally dry by day and 25% completely continent by night. Also, the report implies extremely low complication rates. Particularly interesting is that they note that bladder function seems to improve [44] as time goes by. This development is extremely exciting and a detailed report is eagerly anticipated. It remains to be seen however as to whether other groups can replicate these outcomes. An Italian group, Berrettini at al., recently reported [45] concerns in terms of complications and penis in 2009. Purves and Gearhart [46] reported very serious complications of this approach in 2008, including penile loss and need for cystectomy. 4. Manchester Bladder Exstrophy Prior to 2000, most centres in the UK saw approximately 1 new infant per year with BE. Since 2000 Manchester has seen 75 new infants with classic BE. There are approximately 20 infants born per year in the UK so Great Ormond Street Hospital has seen ~ 150. Theirs and ours are two very significant cohorts of children with this rare anomaly, and the two centres are now amongst the most experienced in the world. Even Johns Hopkins Hospital in Baltimore USA does not see as many new infants with BE as either of the British units. The national bladder exstrophy project in the UK commenced in 2000. The three surgical methods discussed above were well described when we determined in Manchester what direction we should follow. My late colleague David Gough, visited Melbourne Children’s Hospital where he had trained, to review the process and outcomes of the RTSM, but our decision following that review, was to

248

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250

stick with the Hopkins approach — the Modern Staged Repair (MSRE). Although our previous experience of BE was limited numerically to that expected from our Northwest of England referral base, 1–2 infants per year, we did at least have some experience of the MSRE in that small group. Our continuation of that approach, but in greater numbers, provided us an opportunity to learn about BE. There was an acceptance however, that the MSRE may not be physiologically ideal, and because of the growing enthusiasm for CPRE, we were interested to venture for a short time towards that procedure but poor outcomes returned us to the staged approach. Prior to 1991, the majority of children were incontinent or had a cutaneous diversion. Our challenge therefore was to improve measureable outcomes and to move BE on in terms of UK performance. The points of reference for us were initially the 1991 BAPS paper and our own 2006 paper which reflected 1990s UK practice. There have also however been standards published and set by the Hopkins group over 20 years against which we could compare our staged approach and to which we could aspire. As the project has developed, we have recognised important issues which have not been assessed or well analysed in the literature and to which we have given our attention. The following were some of the issues for us to address. Hopkins had demonstrated that the most important single factor in terms of gaining eventual continence is achieving successful primary closure [47]. It is also imperative for the bladder to achieve sufficient capacity if development of a satisfactory voiding habit is to be achieved; otherwise eventual continence cannot even be contemplated. These issues have been important for us to measure, but the more important question was whether they were translatable into voiding and continence success. If we could achieve any of these goals, could we achieve them without renal complication? How would the children eventually grow up in terms of citizenship, lifestyle and sexual function? Could we contribute anything new to the world’s view on bladder exstrophy? 5. Antenatal Service Our earliest involvement with some of our BE patients begins with antenatal care. For two decades now the signs for BE have been well defined. Diagnosis of BE is suggested on antenatal scan by absence of bladder, low insertion of umbilical cord and bony pelvis anomaly [48]. As the recognised centre in the north of England we receive many antenatal referrals of fetuses with suspected BE. What we don’t know is how many fetuses with BE are terminated without due consideration from an informed source. We regard antenatal referral as an urgent issue because the pressure is often on to terminate the pregnancy when a fetus has a serious anomaly. We see the parents as soon as possible after contact and this gives us opportunity to provide information about the condition and outcomes. A group of professionals meets the parents, always a paediatric urologist and senior nurse specialist and sometimes our exstrophy psychologist as well. Our role is to impart information, sometimes to help the parents towards a decision about termination, but not to make the decision for them. Although the antenatal features of BE have been defined for many years, we noted that the literature was sparse on any aspect of antenatal diagnosis. We reviewed our experience of antenatal diagnosis [49] in 2011 and were surprised at the outcomes. One finding of which we were completely unaware was that only 25% of BE infants are diagnosed antenatally. Only a service created by a rationalisation of BE care could have could have performed this research. This single finding has many implications — two at least extremely important. Firstly BE cannot be eradicated by termination and secondly as about 75% of BE is not diagnosed antenatally expensive litigation against the NHS on the basis of wrongful birth because of missed antenatal diagnosis may not be possible.

Table 3 Primary healing outcomes comparing the two groups, neonatal closure and delayed closure.

Sex Primary Healing without dehiscence Death

Neonatal

Delayed

26

41

15 male 21 1

26 male 41 0

6. Primary Closure BE is a herniation of the open bladder and posterior urethra through a lower abdominal wall defect and is densely adherent to the diastased pubic symphysis. The primary closure in MRSE involves mobilising the bladder off both the abdominal wall and the pubic bones, closing the bladder, performing pelvic bone osteotomy, reapposing the pubic diastasis and closing the abdominal wall defect. For the first period of the project, we performed bladder closure in the neonatal period facilitated by posterior iliac bone osteotomy. Since 2007, following successful experience with delayed closure in small bladder templates to allow them some growth, we have consistently performed delayed closure (Table 3) around the age of 3 months facilitated by an anterior iliac bone osteotomy. Between 2000 and 2007, 21 of 26 infants who underwent primary repair in the neonatal period, had successful closure. Between 2007 and 2012, 41 infants underwent delayed closure and all were successful. Because of concerns regarding loss of bladder compliance occurring after prolonged exposure of the bladder we have studied bladder capacity development. Table 4 shows equal bladder capacity development when neonatal and delayed closures are compared. Delayed primary closure permits the child to spend time at home with the family before a prolonged hospital admission and is associated with better primary healing without loss of potential capacity. Because the babies achieve successful primary closure, they are then best placed for potential continence development later [47].

7. Vesico-ureteric Reflux and Upper Tracts It has been generally accepted that most children with BE have vesico-ureteric reflux. This indeed was our impression as well. Exstrophy closure produces a small bladder cavity with laterally placed ureteric orifices — a recipe for reflux. We found that this reflux led to urinary tract infection and renal scarring. In 2008 we took a difficult decision to try to reimplant the ureters at the time of the primary bladder closure, in order to prevent reflux. Technically, this is now the most difficult and worrying part of the operation, but it has transformed upper tract outcomes for us. The operation is different from a normal re-implant. The ureters are much more adherent to the bladder and are directed inferolaterally rather than superolaterally. The antireflux tunnel has to be formed superiorly in order to gain sufficient tunnel length and to avoid the area to be used for eventual BNR. We have now performed this procedure on 25 consecutive infants, no matter the condition of the bladder, without causing ureteric damage or significant obstruction. One child developed transient moderate (15 mm) bilateral

Table 4 Comparison of bladder capacity development following neonatal and delayed closure (n = 45).

Sex Mean capacity age 12–24 months

Neonatal (n = 21)

Delayed (n = 24)

11 male 73 ml

14 male 73 ml

P value

1.00

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250 Table 5 Renal outcomes: Group1 — without primary reimplantation of ureters (n = 37) (mean follow-up 7.1 years) and Group 2 — after primary ureteric reimplantation (n = 20) (mean follow-up 2.7 years). None Vesico-ureteric reflux (VUR) Group 1 8 Group 2 11 Hydronephrosis Group 1 24 Group 2 19 Renal Scarring Group 1 18 Group 2 20 a

Unilateral

Bilateral

P-valuea

8 3

21 1

0.005

8 0

5 1

0.02

14 0

5 0

b 0.0001

Fisher exact test.

hydronephrosis after removal of stents, but this resolved spontaneously. Table 5 summarises the outcome. Although we do not have detailed data regarding urinary infection in the two groups, we are aware that our children now hardly ever have any issue with UTIs. The data are biased towards the delayed group because of the much shorter follow-up, but we know that most of the renal scarring and hydronephrosis that we observed in the first group occurred in the early years of their lives. The evidence seems very clearly to be in favour of ureteric re-implantation at the time of primary bladder closure. 8. Urinary Continence Although many issues measure the outcome of BE reconstruction, the major goal is to achieve controlled dryness. We have always taken the view that the aim for all of the children should be volitional voiding per urethra. We reviewed our continence outcomes in 2012 for the first cohort of children. Fifty-eight classic BE children had been treated by MSRE between 2000 and 2011. Thirty-one were ≥ 5 years at the time and were suitable for review. Data about continence were not available for five children. Table 6 summarises the continence outcomes of the 26 (13 females) children as assessed by a senior nursing specialist. All underwent the MSRE and 16/26 (62%) are continent and able to micturate urethrally, of which 6 also perform urethral CIC. Four (15%) are intermittently incontinent and able to micturate urethrally and are being treated with biofeedback techniques. Five (19%) are continent following bladder neck closure, bladder augmentation and continent urinary diversion. One child (4%) received a urostomy and is continent. Centralisation has achieved 62% voiding continence in our practice and only 23% have required urinary diversion (urostomy of Mitrofanoff) so far. We have not yet had sufficient experience with biofeedback bladder training to provide analysed outcomes but preliminary observations are positive. These early outcomes are encouraging but long term outcomes are what will count in the end. Long-term followup and reporting are essential if true understanding of surgical outcomes is to be achieved. We hope however that the improvements

249

we have seen in bladder closure and upper tract outcomes in our second cohort will translate into better continence outcomes. The literature suggests that they should. 9. Other Benefits of Rationalisation Our data, as summarised above, have been either presented and/or published and/or being considered for publication. Along the way we have published/presented studies on other various subjects including pelvic bone growth in exstrophy, an improved method for umbilicoplasty in exstrophy patients which is being used in other umbilical deficient situations as well, self-esteem in adolescent exstrophy patients, bladder capacity in bladder exstrophy and run a European study on the Incidence of BE (awaiting publication). We have published sex guide booklets for both sexes for our transitional adolescent BE patients. The service has been developed into a multidisciplinary lifespan service now with a database of 186 patients. We provide an antenatal service when required and in excess of 40 MDT BE clinics per year for the paediatric, adolescent and adult age groups. The multidisciplinary team is led by the paediatric urologists but includes consultant orthopaedic, urology, and gynaecology colleagues, a team of specialist nurses devoted to the BE patients and a full-time BE consultant psychologist. 10. Conclusions This article deals with a huge clinical problem to which there is no agreed solution. The discussion regarding the different operative approaches demonstrates the reasons for disagreement. The lack of reconstructive success has even driven some countries to immediate diversion as their primary choice for management. The concentration of experience in Manchester has allowed us to think about BE to a different depth. The volume of activity has helped us recognise errors, learn from them and change practice. It is now possible for us to look back over 12 years and see what we did not know when we commenced and to drive improvement. We still do not know how BE should be managed and we are certainly not claiming that the MSRE is the correct approach. However, one thing is sure; we know volumes more about treating BE than we did when we were treating 1 case per year. It is my view that the UK experience of biliary atresia and BE are examples of practice which could be applied to other rare conditions which we as paediatric surgeons manage. Can we defend countrywide occasional practice in serious conditions which have lifelong functional and quality of life implications for our patients? It is for senior professionals in the specialty to lead in this area of development, not wait for politicians or media to direct. Acknowledgments The author wishes to acknowledge the important members of the children's bladder exstrophy team in Manchester and their essential contributions: Mr R. Cervellione, Sr J Fishwick, Dr R Hurrell, Mr T Khan, Dr R Vashisht and Sr B Whitnall. References

Table 6 Continence outcome for MSRE in Manchester in children N 5 years of age (n = 26). n

%

Continenta and micturate per urethra without any CIC 10 38 Continent and micturate per urethra and perform CIC 6 23 Intermittently incontinent but micturate per urethra (biofeedback) 4 16 Continent after bladder neck closure, bladder augmentation and 5 19 Mitrofanoff (CIC) Continent after urostomy 1 4 a

ICCS definitions of children’s continence.

[1] de la Hunt MN, O'Donnell B. Current management of bladder exstrophy: a BAPS collective review from eight centres of 81 patients born between 1975 and 1985. J Pediatr Surg 1989;24:584–5. [2] Hollowell JG, Ransley PG. Surgical management of incontinence in bladder exstrophy. Br J Urol 1991;68:543–8. [3] Cervellione RM, Bianchi A, Fishwick J, et al. Salvage procedures to achieve continence after failed bladder exstrophy repair. J Urol 2008;179:304–6. [4] McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000;355(9197):25–9. [5] Birkmeyer JD, Siewers AE, Finlayson EV, et al. Hospital volume and surgical mortality in the United States. N Engl J Med 2002;346:1128–37. [6] Begg CB, Cramer LD, Hoskins WJ, et al. Impact of hospital volume on operative mortality for major cancer surgery. JAMA 1998;280:1747–51.

250

A.P. Dickson / Journal of Pediatric Surgery 49 (2014) 244–250

[7] Edwards EB, Roberts JP, McBride MA, et al. The effect of the volume of procedures at transplantation centers on mortality after liver transplantation. N Engl J Med 1999;341:2049–53. [8] Davenport M, De Ville de Goyet J, Stringer MD, et al. Seamless management of biliary atresia in England and Wales (1999–2002). Lancet 2004;363:1354–7. [9] Chardot C, Carton M, Spire-Bendelac N, et al. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology 1999;30:606–11. [10] Jeffs RD. Functional closure of bladder exstrophy. Birth Defects Orig Artic Ser 1977;13:171–3. [11] Lepor H, Jeffs RD. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol 1983;130:1142–5. [12] Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 2007;3:311–5. [13] Purves JT, Baird AD, Gearhart JP. The modern staged repair of bladder exstrophy in the female: a contemporary series. J Pediatr Urol 2008;4:150–3. [14] Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol 1999;162: 1415–20. [15] Kelly J. Vesical exstrophy: repair using radical mobilisation of soft tissues. Pediatr Surg Int 1995;10:298–304. [16] Zabbo A, Kay R. Ureterosigmoidostomy and bladder exstrophy: a long-term follow-up. J Urol 1986;136:396–8. [17] Stein R, Fisch M, Stockle M, et al. Urinary diversion in bladder exstrophy and incontinent epispadias: 25 years of experience. J Urol 1995;154:1177–81. [18] Boemers, T: 2010 (Personal communication). [19] Gearhart JP: 2005 (Personal communication). [20] Gearhart JP, Jeffs RD. Bladder exstrophy: increase in capacity following epispadias repair. J Urol 1989;142:525–6. [21] Surer I, Baker LA, Jeffs RD, et al. Modified Young–Dees–Leadbetter bladder neck reconstruction in patients with successful primary bladder closure elsewhere: a single institution experience. J Urol 2001;165:2438–40. [22] Shaw MB, Rink RC, Kaefer M, et al. Continence and classic bladder exstrophy treated with staged repair. J Urol 2004;172:1450–3. [23] Mouriquand PD, Bubanj T, Feyaerts A, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003;92:997–1001. [24] Lottmann HB, Melin Y, Cendron M, et al. Bladder exstrophy: evaluation of factors leading to continence with spontaneous voiding after staged reconstruction. J Urol 1997;158:1041–4. [25] Reiner WG, Gearhart JP. Anxiety disorders in children with epispadias-exstrophy. Urology 2006;68:172–4. [26] Ebert A, Scheuering S, Schott G, et al. Psychosocial and psychosexual development in childhood and adolescence within the exstrophy-epispadias complex. J Urol 2005;174:1094–8. [27] Husmann DA, McLorie GA, Churchill BM. Factors predisposing to renal scarring: following staged reconstruction of classical bladder exstrophy. J Pediatr Surg 1990;25:500–4. [28] Schaeffer AJ, Stec AA, Baradaran N, et al. Preservation of renal function in the modern staged repair of classic bladder exstrophy. J Pediatr Urol 2013;9:169–73.

[29] Mitchell ME, Bagli DJ. Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol 1996;155:300–4. [30] Borer JG, Gargollo PC, Kinnamon DD, et al. Bladder growth and development after complete primary repair of bladder exstrophy in the newborn with comparison to staged approach. J Urol 2005;174:1553–7. [31] Hammouda HM, Kotb H. Complete primary repair of bladder exstrophy: initial experience with 33 cases. J Urol 2004;172:1441–4. [32] Gargollo PC, Borer JG. Contemporary outcomes in bladder exstrophy. Curr Opin Urol 2007;17:272–80. [33] Shnorhavorian M, Grady RW, Andersen A, et al. Long-term follow-up of complete primary repair of exstrophy: the Seattle experience. J Urol 2008;180:1615–9. [34] Shoukry AI, Ziada AM, Morsi HA, et al. Outcome of complete primary bladder exstrophy repair: single-center experience. J Pediatr Urol 2009;5:496–9. [35] Husmann DA. Surgery insight: advantages and pitfalls of surgical techniques for the correction of bladder exstrophy. Nat Clin Pract Urol 2006;3:95–100. [36] Husmann DA, Gearhart JP. Loss of the penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol 2004;172:1696–701. [37] Lazarus J. Penile loss following complete primary repair of bladder exstrophy. J Pediatr Urol 2009;5:519–20. [38] Cervellione RM, Husmann DA, Bivalacqua TJ, et al. Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol 2010;6:450–6. [39] Schaeffer AJ, Stec AA, Purves JT, et al. Complete primary repair of bladder exstrophy: a single institution referral experience. J Urol 2011;186:1041–6. [40] Ansell J. 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 1979;121:650–3. [41] Carroll D, McMullin N, O’Brien M, et al. Outcomes in bladder exstrophy: 30 years’ experience in a single-centre. J Pediatr Urol 2007;3(Supp 1):S81–2. [42] Jarzebowski AC, McMullin ND, Grover SR, et al. The Kelly technique of bladder exstrophy repair: continence, cosmesis and pelvic organ prolapse outcomes. J Urol 2009;182:1802–6. [43] Cuckow P, Desai D, Ryan K. Physiological reconstruction of the lower urinary tract in bladder exstrophy — 10 years experience with the Kelly operation. J Urol 2010;183:e210. [44] Cuckow P. 2010 (personal communication). [45] Berrettini A, Castagnatti M, Rigamonti W. Radical soft tissue mobilization and reconstruction (Kelly procedure) for bladder exstrophy repair in males: initial experience with nine cases. Pediatr Surg Int 2009;25:427–31. [46] Purves JT, Gearhart JP. Complications of radical soft-tissue mobilization procedure as a primary closure of exstrophy. J Pediatr Urol 2008;4:65–9. [47] Baradaran N, Cervellione RM, Orosco R, et al. Effect of failed initial closure on bladder growth in children with bladder exstrophy. J Urol 2011;186:1450–4. [48] Gearhart JP, Ben-Chaim J, Jeffs RD, et al. Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 1995;85:961–4. [49] Goyal A, Fishwick J, Hurrell R, et al. Antenatal diagnosis of bladder/cloacal exstrophy: challenges and possible solutions. J Pediatr Urol 2012;8:140–4.

The management of bladder exstrophy: the Manchester experience.

Bladder exstrophy (BE) is a severe congenital malformation with life-long implications. This article discusses the current surgical management and des...
755KB Sizes 4 Downloads 2 Views