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16 Male Epispadias
Recently, Weiss et al. reported results from the Multi-Institutional Bladder Exstrophy Consortium (MIBEC). Of 8 patients with glandular epispadias, 5 were continent (dry for 3h or more), 1 had intermediate continence (dry for 2h or less), and 1 was incontinent. Of 8 patients with penile epispadias, 6 were continent and 2 had intermediate continence. Of 20 patients with penopubic epispadias, 10 were continent, 2 of whom required clean intermittent catheterization, 5 had intermediate continence with one of them on clean intermittent catheterization, and 5 were wet. This series also showed that the proximal repairs will require a bladder neck recon­struction to achieve continence but that even the more distal defects may have blad­der neck incompetence and persistent incontinence [34].
271

Sexual Function

The cosmetic appearance and sexual function of males undergoing isolated epispa­dias repair are not robust. Penile length, quality of erections, and ejaculatory func­tion all play a role in sexual function. Reddy etal. reported that 73% of the males with epispadias reported being engaged in sexual activity, yet 67% (10/15)reported having one or more issues related to sexual function [32]. These included abnormal ejaculation, difcult orgasm, decreased sensation, difculty maintaining erections, penile appearance, and curved erections.
Of Sunil etal. series of patient with male epispadias, only 15 (20%) completed the questionnaire. Although 12 patients (80%) reported overall satisfactory sexual intercourse, 11 (73%) admitted to 1 or more problems with sexual function, includ­ing abnormal ejaculation (53%), diminished sensation (20%), and difculty main­taining an erection (20%) [35].
Of 31 epispadias patients in Bencic et al. series, 7 (22.6%) completed the International Index of Erectile Function (IIEF) questionnaire. The average score was 21.3 (19–26) for the erectile function domain and 8.7 (7–10) for the overall satisfaction domain. Only 2 patients (28.6%) had lower scores in the erectile func­tion domain. Three of the 7 patients had lower scores in the orgasmic domain [29].

Renal Function

Bladder neck reconstruction may place patients with epispadias at risk for renal compromise over time. This risk is lower than in bladder exstrophy as bladder capacity is near-normal in most children with isolated epispadias.
In a series by Joshi etal. from the International Bladder Exstrophy Consortium of 12 patients with isolated epispadias, eGFR was normal in all. Vesicoureteral reux was present in one patient who also developed renal scarring. This suggests that patients with vesicoureteral reux are at risk for pyelonephritis and possible renal scarring, and they should be followed closely after repair [36].
272
P. A. Merguerian

Female Epispadias

Primary female epispadias is rare and occurs in an estimated prevalence of 1:160,000 to 1:480,000 live births [37]. Some of these patients present later on in life when they are unable to be toilet-trained.
In this condition, the dorsal aspect of the urethra is absent and the urinary sphinc­ter including the bladder neck is decient. The clitoris is bid, the perineal body is broadened, and the vagina is anterior. The mons pubis is absent, and the anterior labia are laterally displaced but are fused in the midline posteriorly. The vagina may be stenotic [1]. The internal genital organs (uterus, fallopian tubes, and ovaries) are usually unaffected.
The goals in managing these patients include the following:
1. Creation of a straight urethra
2. Good cosmetic appearance with reapproximation of the genitalia in the midline
3. Reconstructing the bladder neck for continence
4. Maintaining clitoral function
The traditional surgical approach was a staged reconstruction with the second stage being the bladder neck reconstruction [38]. Manzoni and Ransley presented an alternative single-stage technique in 2009, where the urethra, bladder neck, and clitoris were repaired by a perineal approach, but without tailoring the bladder neck to enhance outlet resistance [39]. This approach was then modied to perform a bladder neck tailoring through a perineal approach with favorable outcomes [40,
41]. The advantages of using this approach include improved continence and
reduced morbidity. The radical soft-tissue mobilization described by Kelly has been applied for female primary epispadias [42].
With the complete primary epispadias repair, the bladder neck, urethra, and vagina are mobilized as a unit. Osteotomies may be necessary when the pubic dias­tasis is wide. The intersymphyseal bands are deeply incised to allow the bladder neck and urethra to move posteriorly. The bladder neck is then reconstructed and the urethra tubularized. The symphysis pubis is reapproximated.
The Richard Grady Monsplasty is then performed. This is a vertical Z-plasty closure to provide a 2-layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy. This has been described in the article by Weiss DA etal. that also includes a video of the tech­nique [43].
The perineum is described elsewhere in this book by Pippi Salle etal.
The Kelly repair of female epispadias is described by Leclari MD etal. [42]. Supplementary video related to this article can be found at https://doi.org/10.1016/j.
jpurol.2017.08.017.
Through a short transpubic midline incision, the lateral aspect of the bladder is exposed, giving access to the superior aspect of the pelvic oor. The most anterior
16 Male Epispadias
273
bers of the levator ani muscle are incised at the level of their insertion on the inter­nal obturator muscle fascia, providing exposure to the ischiorectal fat and the uro­genital diaphragm. Perineal dissection allows adequate exposure to the corpora cavernosa. A muscle stimulator is used to identify the bulbospongiosus, ischiocav­ernosus, and transverse perineal muscles. The periosteum of the ischiopubic rami is peeled away to allow mobilization of both corpora cavernosa, until the neurovascu­lar pedicle exiting from the Alcock’s canal is identied. All striated muscular struc­tures, part of the urogenital diaphragm, linking the urogenital complex to the anterior pelvic ring are detached from the bone. Further dissection, on the medial aspect of the corpora, allows separation of the corpora from the urethral plate, which eventually allows repositioning of the bladder neck deep into the pelvis. Reconstruction included ureteral reimplantation if necessary, bladder neck funnel­ing reconstruction, and urethral reconstruction with tubularization over an 8 or 10F catheter. Muscular bers detached from the pelvis and bers of the bulbospongiosus muscle are wrapped around the bladder neck and the upper urethra whenever pos­sible. A corporoplasty is performed by approximating both corpora cavernosa ven­trally to the neo-urethra on the midline, and both glans and clitoris are covered by a common clitoral hood.

Outcomes

Alyami etal. reported on the long-term outcomes comparing the traditional Young­Dees- Leadbetter repair versus the modied perineal approach. They had a total of 12 patients with isolated female epispadias, three of whom underwent the tradi­tional repair and 7 underwent the perineal repair. None of the 3 patients who under­went the traditional repair were continent, while 4 of 7(57%) who underwent the perineal approach were continent.
Leclair et al. compared the perineal approach to the Kelly repair [42]. They included 16 patients in their study. Seven females underwent the perineal approach, and of these, 5 of 7 were continent during the day and 4 of 5 were also continent at night. They state that 3 of the 5 required bladder neck injection to achieve conti­nence. Of the 9 patients undergoing the Kelly repair, 8 were evaluable and all achieved diurnal continence and 3 of 8 were fully continent though one required clean intermittent catheterization for obstructive micturition.
There are signicant limitations to the above studies including the small number of patients in the surgical group. The comparison groups may also not have been similar as the more severe forms of epispadias most likely required a more radical approach. Because of the rarity of this condition, multi-institutional and longer term studies are required to determine the benet of the complete primary repair versus the perineal repair versus the Kelly repair.
274
P. A. Merguerian

Conclusion

Epispadias represents one end of the spectrum of the bladder exstrophy-epispadias complex. Repair of epispadias is a challenging operation even in expert hands. The incidence is rare, and repair should be performed in centers of excellence in order to provide the best possible outcome.
As this condition is rare, published long-term outcomes related to continence and sexual function are sparse. Studies show that continence can be achieved in the majority of patients with epispadias but over 80% require multiple procedures to achieve social continence. Erectile and ejaculator functions are satisfactory, yet there is continued dissatisfaction with genital appearance.

References

1. Grady RW, Mitchell ME.Management of epispadias. Urol Clin North Am. 2002;29:349–60.
2. Dees JE.Congenital epispadias with incontinence. J Urol. 1949;62:513.
3. Cendron PS, Cho M, Pennison I, Rosoklija DA, Diamond JG.Borer: Anatomic ndings asso­ciated with epispadias in boys: Implications for surgical management and urinary continence. J Ped Urol. 2018;14:42–8.
4. Woodhouse CRJ, Kellett MJ.Anatomy of the penis and its deformities in exstrophy and epi­spadias. J Urol. 1984;132:1122.
5. Silver RI, Yang A, Ben-Chaim J, etal. Penile length in adulthood after exstrophy reconstruc­tion. J Urol. 1997b;158:999.
6. Gearhart JP, Yang A, Leonard MP, etal. Prostate size and conguration in adult patients with bladder exstrophy. J Urol. 1993c;149:308.
7. Hurwitz RS, Woodhouse CRJ, Ransley PG.The anatomical course of the neurovascular bun­dles in epispadias. J Urol. 1986;136:68.
8. Arap S, Nahas WC, Giron AM, etal. Continent epispadias: surgical treatment of 38 cases. J Urol. 1988;140:577.
9. Kramer SA, Kelalis P.Assessment of urinary continence in epispadias: review of 94 patients. J Urol. 1982a;128:290.
10. Ben-Chaim J, Peppas DS, Jeffs RD, etal. Complete male epispadias: genital reconstruction achieving continence. J Urol. 1995b;153:1665.
11. Diamond D, Ransley PG.Male epispadias. J Urol. 1995;154:2150–5.
12. Gearhart JP, Leonard MP, Burgers JK, Jeffs RD.The Cantwell-Ransley technique for repair of epispadias. J Urol. 1992;148:851–4.
13. Mitchell ME, Bagli DJ.Complete penile disassembly for epispadias repair: the Mitchell tech­nique. J Urol. 1996;155:300–4.
14. Grady RW, Mitchell ME.Management of epispadias. Urol Clin N Am. 2002;29:349–60.
15. Gearhart JP, Jeffs RD. The use of parenteral testosterone therapy in genital reconstructive surgery. J Urol. 1987;138:1077–8.
16. Makedonsky J.The use of Human Chorionic Gonadotropin (HCG) for penile reconstruction in bladder Exstrophy and Total Epispadias. Eur J Ped Surgery. 2006;16:428–31.
17. Suson KD, Sponseller PD, Gearhart JP.Bony abnormalities in classic bladder exstrophy: the urologist’s perspective. J Ped Urol. 2013;9:112–22.
18. Diamond DA, Ransley PG.Male epispadias. J Urol. 1995;154:2150–5.
19. Gearhart JP, Sciortino C, Ben-Chaim J, Peppas DS, Jeffs RD.The Cantwell-Ransley epispa­dias repair in exstrophy and epispadias: lessons learned. Urology. 1995;46:92–5.
20. Bar-Yosef Y, Sofer M, Ekstein MP, Binyamini Y, Ben-Chaim J.Results of Epispadias repair using the modied cantwell-ransley technique. Urology. 2017;99:221–4.
16 Male Epispadias
21. Kramer SA, Jackson IT.Bilateral rhomboid aps for reconstruction of the external genitalia in epispadias-exstrophy. Plast Reconstr Surg. 1986;77:621–31.
22. Pippi Salle JL, Elifranji M, Abbas T, Alhadi A, Elkadhi A, Leslie B, Vallasciani S.Technical details in the primary repair of male epispadias: step by step video. Urol Video J. 2022;13:10027.
https://doi.org/10.1016/j.urolvj.2022.100127.
23. Baka-Jakybiak M.Bladder neck, urethral and penile reconstruction in boys with the exstrophy­epispadias complex. BJU Int. 2000;86:513–8.
24. Pippi Salle JL, Jednak R, Capolicchio JP, Franca IMP, Labbie A, Gosalbex R.A ventral rota­tional skin ap to improve cosmesis and avoid chordee recurrence in epispadias repair. BJU Int. 2002;90:918–23.
25. Kibar Y, Roth C, Frimberger D, Kropp B.Long-term results of penile disassembly technique for correction of epispadias. Urology. 2009;73:510–4.
26. Surer I, Baker LA, Jeffs RD, Gearhart JP.The modied Cantwell-Ransley repair for Exstrophy and Epispadias: a 10-year experience. J Urol. 2000;164:1040–3.
27. Thomas JS, Shenoy M, Mushtaq I, Wood D.Long-term outcomes in primary male epispadias. J Ped Urol. 2020;16(80):e1–6.
28. Braga LH, Lorenzo AJ, Bagli DJ, Khoury AE.Outcome analysis of isolated male epispadias: single center experience with 33 cases. J Urol. 2008;179:1107–12.
29. Bencic M, Bizic M, Joksic I, Stojanovic B, Djordjevic ML.Isolated male Epispadias repair: long-term outcomes. Life. 2024;14:446.
30. Tourchi A, Hoebeke P. Long-term outcome of male genital reconstruction in childhood. J Pediatr Urol. 2013;9:980–9.
31. Djordjevic ML, Bizic MR, Martins F, Kojovic V, Krstic Z.Treatment for failed epispadias repair presenting in adults. J Urol. 2013;190:165–70.
32. Reddy SS, Inouye BM, Anele UA, Abdelwahab B, Le B, Gearhart JP, Rao PK.Sexual health outcomes in adults with complete male epispadias. J Urol. 2015;194:1091–5.
33. Cendron M, Cho PS, Pennison M, Rosoklija I, Diamond DA, Borer JG.Anatomic ndings associated with epispadias in boys: Implications for surgical management and urinary conti­nence. J Ped Urol. 2018;14:42–6.
34. Weiss DA, Lee T, Roth EB, Cendron M, Goetz J, Kryger JV, Groth TW, Shukla AR, Mitchell ME, Canning DA, Borer JG.Male epispadias repair: outcomes at three sites prior to the estab­lishment of a multi-institutional collaboration. J Ped Urol. 2024;20:e1-408.e6.
35. Reddy SS, Inouye BM, Anele UA, Abdelwahab M, Le B, Gearhart JP, Rao PK.Sexual health outcomes in adults with complete male Epispadias. J Urol. 2015;194:1091–5.
36. Joshi RS, Eftekharzadeh S, Shukla AR, Ramji J, Hingorani SR, Canning DA, Pippi-Salle JL, Merguerian P, Defoor WR Jr, Frazier J, Weiss DA, Reddy PP.Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: results from the international bladder exstrophy consortium. J Ped Urol. 2023;19(34):e1–34, e9.
37. Allen A, Rodjani J, Kelly M, Inoue JM.Hutson: female epispadias: are we missing the diag­nosis? BJU Int. 2004;94:613–5.
38. Peters C, Gearhart JP, Jeffs R.Epispadias and incontinence: the challenge of the small bladder. J Urol. 1988;140:1199–201.
39. Manzoni G, Ransley P.Primary surgical intervention for female epispadias. J Pediatr Urol. 2007;3:S73.
40. Cheikhelard A, Aigran Y, Lottmann H, Lortat-Jacob S. Female epispadias manage­ment: perineal urethrocervicoplasty versus classical Young-Dees procedure. J Urol. 2009;182(4):1807–11.
41. Bhat AL, Bhat M, Sharma R, Saxena G.Single stage perineal urethroplasty for continence in female epispadias: a preliminary report. Urology. 2008;72(2):300–3.
42. Leclair MD, Faraj S, Villemagne T, Carrouget J, Arnaud A, Heloury Y.Primary female epispa­dias: perineal approach or Kelly repair? J Pediatr Urol. 2018;14:33–9.
43. Weiss DA, Steffe E, Borer JG, Groth TW, Roth EB, Kryger JV, Shukla AR, Canning DA, Mitchell ME.The Richard Grady Monsplasty: a vertical Z-plasty technique. J Pediatr Urol. 2021;17(4):575–6.
275
The Failed Exstrophy Repair: Technique andImplications
RakeshS.Joshi andJaishriRamji

Introduction

Over the past several decades, there has been a dramatic evolution in the manage­ment of bladder exstrophy. Advanced surgical techniques and improved post­operative management have contributed to better results in functional and cosmetic outcomes. Successful primary closure is a key factor for bladder growth and eventu­ally continence. In the modern era, contemporary success rates of exstrophy closure have improved signicantly, and the long-term continence rates range around 45–60% depending on the type of repair done [1].
With increasing experience and knowledge of the surgical anatomy, the inci­dence of complete dehiscence has decreased from 13% to 1% [2]. Yet failures in different forms still occur frequently. A failed exstrophy closure is a devastating setback to subsequent genitourinary reconstruction, urinary continence and long­term impact on the patient and healthcare system.
The occurrence of bladder prolapse, complete dehiscence, vesicocutaneous s­tula, outlet obstruction or any combination of factors along with loss of urethra, corpora or glans/clitoris constitutes a failed exstrophy closure. Although repeat clo­sure is often feasible, there are negative implications on the fate of the urinary tract. The chances of achieving continence after failed and multiple closures decrease dramatically. Children with one or more failed closures are ultimately an extremely challenging subset with poorer outcomes.
17
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_17.
R. S. Joshi (*) · J. Ramji Department of Pediatric Surgery, B. J. Medical College and Civil Hospital, Ahmedabad, Gujarat, India
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_17
277
278
In this chapter, we will try to outline the possible reasons for failed closures, the types of outcomes that constitute failure, the early and late management of failed closure, including principles of redo closures, and the outcomes of redo exstrophy closures.
R. S. Joshi and J. Ramji
Reasons forFailure ofPrimary Closure
Regardless of choice of surgery, the objectives of surgery should aim at tension-free closure of bladder, pelvic ring and abdominal wall; protection of the upper tracts by avoiding outlet obstruction and pyelonephritis; and preservation of the genital and urethral soft tissues. Consequently, a failed closure results when any one of these objectives is not accomplished adequately.
Pre-operative, technical or post-operative causes can be the attributable factors for failure, either in isolation, or more commonly, multifactorial.

Pre-operative Factors

Various patient-related factors may contribute to failure of the primary repair; a wide pubic diastasis, an ill or undernourished infant and an unprotected or improp­erly preserved bladder plate, which delays the primary surgery. Technical precision and the surgeon and institutional experience, along with lack of concomitant oste­otomy, are also known to impact the outcomes [3].

Technical Aspects

From a technical point of view, adequate vesicourethral mobilisation is a key factor contributing to successful closure. Experienced surgeons advocate aggressive pos­terior repositioning of the bladder, bladder neck and the urethra. This is based on the principle that exstrophy represents an anterior bladder herniation. The posterior placement is facilitated by the simultaneous dissection of bladder and urethral plate and division of the intersymphyseal band. Failure to release this band increases the chances of dehiscence [4, 5]. Surer etal. and EL Sherbiny etal. have showed that this principle helps prevent signicant complications even in repair of failed clo­sures [4].
The rate of dehiscence may also be correlated with exiting the drainage cath­eters from the neo- urethra, rather than a suprapubic exit site [6]. This manoeuvre may cause ischaemia, especially if paraexstrophy aps are used. The aps can necrose and cause the inter-pubic stitch to erode into the urethra, with resultant infection, dehiscence or urethral stricture. Currently the role of paraexstrophy aps is limited mostly to staged repairs when there is severe shortening of the urethra [7].
17 The Failed Exstrophy Repair: Technique andImplications
279
The Role ofOsteotomy
Studies have proven the importance of osteotomy in the surgical treatment of blad­der exstrophy. The major role of osteotomy is in relaxing the tension on the bladder closure and the abdominal wall closure to allow for tension-free, adequate healing. In the absence of osteotomy, the abnormal separation of the pubic bones and the external rotation of the innominate bones place a signicant tension on the soft tis­sues during reconstruction of the bladder, urethra and the anterior abdominal wall. It has been documented that the use of osteotomy has improved the success rates of soft tissue closure and urinary continence and resolved the gait abnormalities [8].
Some surgeons believe that when the primary repair is done in newborns, liga­mental laxity allows the pelvis to be closed without signicant tension. Osteotomy becomes mandatory once exstrophy repair is delayed beyond 72h, in cases of poorly malleable pelvis and when the pubic diastasis is more than 4cm [9, 10]. A rm anterior pelvic ring resulting from osteotomy supports the urethral suspension and contributes in preventing bladder prolapse or dehiscence [8]. Gearhart has reported in his study of 170 failed repairs that osteotomy had been done in only 29% at the time of the primary repair [11]. The rate of failure of abdominal wall closure and bladder closure has diminished to less than 5% at experienced centres with the incorporation of osteotomy in the repair of bladder exstrophy. Various types of oste­otomies have been described, like posterior iliac, anterior osteotomy of the superior pubic rami, anterior diagonal iliac, modied Salter’s innominate and vertical iliac ramotomy [8]. The combined transverse innominate and vertical iliac osteotomy have been shown to have lower rates of dehiscence and prolapse compared to other osteotomies [10]. The benets of osteotomy far outweigh the complication rate of 4% shared collectively by the various osteotomies [8]. At the authors’ institute, all primary repairs are done after 3months of age with osteotomy in all cases. The dehiscence rate has been less than 2%, and there have been very few complications.
On the other hand, certain authors believe that osteotomy is not an essential ele­ment of the exstrophy repair. The downsides are the added morbidity, complications and requirement of an expert orthopaedic surgeon. Even after the osteotomy, the pubes eventually drift apart over a period of time; and hence the osteotomy itself may not be a long-term contributor to continence [12]. Bhatnagar etal. and Sudipta etal. have reported good results with the use of rectus aps to facilitate the closure instead of osteotomy and believe that it is not necessary either for the closure or for the achievement of continence [10, 13].
When talking about failed repairs and redo closures, the pendulum swings greatly in favour of osteotomy. There is ample evidence of osteotomy increasing the suc­cess rates of reclosures as reported by various authors [8, 1416]. The greatest suc­cess rates in reclosures have been achieved when the osteotomy was combined with secure post-operative immobilisation with external xators and modied Buck’s traction. Conversely, the outcomes were universally poor regardless of the type of immobilisation, if the osteotomy was not done [15] (Fig.17.1).
280
ab
Fig. 17.1 Complete dehiscence within days of closure—no osteotomies done. (a) Male. (b) Female
R. S. Joshi and J. Ramji
Post-Operative Sources ofWound Tension
Successful closure depends as much on reducing the tension on the closure as on maintaining the tension-free closure in the post-operative period. Inadequate anal­gesia, poor immobilisation, abdominal distension and poor urinary drainage are some of the factors that can increase the wound tension in the post-operative period and lead to a failed repair [14].
In the initial post-operative period, the focus should be on controlling these adverse forces aggressively. Epidural analgesia with hourly pain assessments and adjunctive sedation whenever needed is crucial to keep the child still and comfort­able. The nasogastric tube should be removed only when the child is stooling ade­quately, and abdomen is soft.
Post-operative immobilisation plays a crucial role in successful closure. It allows the tension-free closure enabled by osteotomy to heal adequately. The use of mummy wraps and spica casts has been shown to be rife with complications, with little benet [15]. Modied Buck’s traction or modied Bryant’s traction has been advocated with satisfactory outcomes [10, 11]. The best results have been reported with the external xators along with modied Buck’s traction for 6–8weeks. The leg traction may be the most effective way to control the movement of the legs as well as keep the buttocks and pelvis at on the bed [15].
Proper management of the outlet of the reconstructed bladder is of prime impor­tance. Early dislodgement of the urinary drainage tubes poses a signicant risk for failure [17]. Special attention should be given to securing the tubes and leaving them in for an adequate period. Generally, we leave the ureteric catheters for 14days, the per-urethral stent for 21days and the suprapubic tube for 6 weeks. However, there may be institutional variations in the protocol [6, 12, 13]. The clo­sure can also be compromised by wound infection, ultimately leading to dehiscence or a stula. Hence the importance of broad-spectrum intravenous antibiotics and nutritious diet cannot be overemphasised.
17 The Failed Exstrophy Repair: Technique andImplications
281
Modes ofFailure
Failure of primary exstrophy repair can manifest as dehiscence, prolapse of bladder, vesicocutaneous stula, urethral stricture or bladder neck obstruction. A complex failure constitutes a combination of one or more of these along with corporal, ure­thral or glans loss.
Kasprenski etal. have reviewed the outcomes of 170 patients with a failed clo­sure. They documented bladder prolapse and dehiscence as the commonest forms (around 35%) and complex failures comprised another 11% [11].
Modes of primary closure failure Prolapse 61 35.9 Dehiscence 60 35.3 Vesicocutaneous stula 05 2.9 Obstruction 06 1.8 Complex 19 11.2 Unknown 15 8.8 Others 07 4.1
n
%
Complete dehiscence of the bladder and abdominal wall often occurs early in the post-operative period. The causes for this disastrous occurrence have been described in the previous section. One of the most important causes is the technical failure. Irrespective of the type of repair done, radical mobilisation of the vesicourethral unit for it to be placed deep in the pelvis is the critical step [4, 18]. Gearhart and Jeffs emphasise the need for this radical dissection along with a secure pelvic ring closure in MRSE.A study by Gearhart compared the success rates with and without osteotomy and with different methods of immobilisation. Success rate of 75% was documented with osteotomy and went up to 96% with immobilisation by external xators or modied Buck’s traction. Spica cast and mummy wrapping were found to be far less effective [15].
Complete primary repair of exstrophy (CPRE) as described by Grady and Mitchell also targets at returning the anatomy to more normal location, allowing pelvic dia­phragm to assist in continence. The dissection of the intersymphyseal band, the ante­rior part of the perineal membrane from the pubis, is essential for deep placement of the vesicourethral unit. The bladder, bladder neck and urethra are mobilised and closed as one unit, using penile disassembly and partial bladder neck tailoring [4, 5,
19]. Proponents of CPRE report good early outcomes with low rates of failures in
form of dehiscence, stula and soft tissue loss. The failure rates go up to 58% in cases done without an osteotomy [20, 21].
Another common form of failure is bladder prolapse often not recognised in the early post-operative period as it is less obvious. The abdominal wall is intact as is the bladder closure, but the bladder prolapses inferiorly through a separated pubic diasta­sis or out the bladder neck. (3) Insufcient dissection of the bladder and urethra from the inferior ramus of the pubis moves the outlet cephalad and increases the chances of bladder prolapse. Similarly, the dissection must be adequate posteriorly and inferiorly