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ab
ab
Fig. 17.8 Use of rectus aps for reinforcement of the bladder augmentation. (a) Raising the ap.
(b) Covering the repair
R. S. Joshi and J. Ramji
Fig. 17.9 Use of rectus aps in redo repair. (a) Raising the ap. (b) Covering the bladder repair
across the inter-pubic gap to cover the bladder repair. The opened rectus sheath is
repaired, and both sheaths are anchored to the upper end of the muscle ap, to get a
secure abdominal wall closure (Fig.17.9).
A different technique of using the rectus muscle ap in lieu of osteotomy to close
the pelvic brim has also been described. The muscle is detached from the superior
pubic ramus on either side, sutured in midline and then pulled caudad and reanchored at the symphysis to complete the pelvic brim [12].
The possibility of weakness or herniation in the decit created by transposing the
muscle needs to be considered, although there has been no incidence in the shortterm follow-up of our cases. Others who have used these aps have also not reported
this consequence [13, 39].
The redo repair in a female patient essentially follows the same principles, with
a few noteworthy points to emphasise. A similar radical dissection of the bladder
and urethra is done but maintaining the connection to the vagina. The urethrovaginal
septum is kept intact to preserve the blood supply. The lateral aspects of the vesicourethral unit and the lateral vaginal attachments are freed from the pelvic oor. It is
17 The Failed Exstrophy Repair: Technique andImplications
293
a
b
c
Fig. 17.10 Redo CPRE in a female. (a) Failed repair—complete dehiscence. (b) Redo CPRE. (c)
Outcome at 6-month follow-up
important at this point to place a small Hegar’s dilator in the vagina to prevent injury
to the lateral vaginal wall. The dissection of the vagina goes lateral, posterior and
inferior upto the levator hiatus. The genital reconstruction includes a monsplasty
and Y-V labioplasty to bring the vaginal introitus out adequately. Rather than suturing the split clitoris (which may disrupt the delicate nerve supply and cause scaring), the clitoral bodies are simply brought together in midline, and anterolateral
skin aps are sutured over them to create the clitoral hood and anterior fourchette
[27, 41]. If there is signicant scar tissue from the previous surgery, a puboplasty as
described by Vanderbrink etal. may be necessary, where a wedge of scar between
the clitoral bodies is excised and tissues are approximated to create the anterior
fourchette [27] (Fig.17.10).
A vesicocutaneous stula, which has not closed spontaneously, often needs to be
managed like a complete dehiscence. However, in some cases, the epispadias repair
and penile reconstruction may be intact. In such cases, the stula can be closed by
doing a wide local dissection without disturbing the epispadias repair. A rectus
abdominus muscle ap can be used to reinforce the stula repair.
294
Bladder outlet obstruction due to neourethral stricture is a form of failure that
may be quite difcult and prolonged to manage. Initial management begins with
identifying the extent of the problem with a cystoscopy and voiding cystourethrogram. Although a few strictures can be managed with dilatations, many require
multiple procedures. Serial dilatations, intermittent self-catheterisation, urethrotomy and revision of the urethroplasty with buccal grafts are the options with escalating intensity. Suprapubic diversion or even complete defunctionalisation with a
vesicostomy may be necessary with increasing upper tract deterioration. Revision
of the outlet wherever possible is preferred over vesicostomy as the latter will
result in shrunken bladder unt for further reconstruction. The need for long-term
suprapubic diversion ultimately translates into bladder augmentation with
Mitrofannoff [6, 14, 22].
R. S. Joshi and J. Ramji
Failed Genital Reconstruction
While a simple urethrocutaneous stula after the epispadias repair is easily repaired,
the management of some of the more complex genital reconstructive failures may
prove challenging.
Loss of the entire urethral plate may require staged reconstruction using full
thickness skin grafts or buccal mucosal grafts. Tissue expanders with skin grafts
may be necessary where there is loss of penile shaft or skin. Neophalloplasty, using
complex plastic surgical reconstructive techniques, is an option for patients who
have signicant loss of glans, corporal bodies and other soft tissues [6, 14, 22].
These are the most challenging situations, where ideal tissue substitutes are not
available.
Alternatives toRedo Repair
A failed exstrophy repair may not always be amenable to redo repair. Various factors need to be taken into consideration before deciding the best option for the child,
the foremost being the status of the upper tracts. The morbidities of reclosure are
also high with redo osteotomies, prolonged immobilisation, epidural analgesia and
extended hospital stay. In a child with a poor bladder template, older age or multiple
attempts at closure, it might be prudent to provide a urinary diversion.
Ureterosigmoidostomy
The procedure was popular and accepted in the past, being tolerated well and providing continence. However, the long-term morbidities of recurrent pyelonephritis,
stone formation, electrolyte imbalance, acidosis and risk of malignancy led to it
falling out of favour. Fisch etal. reported the Mainz II, a modication of the classical ureterosigmoidostomy, as an alternative method of internal diversion. By
17 The Failed Exstrophy Repair: Technique andImplications
295
detubularisation and creating submucosal tunnels, the morbidity was reduced,
although the risk of malignancy remains the same. Recently, this procedure has
again been proposed as a valuable alternative in terms of quality of life for selected
patients [22]. A variation of ureterosigmoidostomy using a recto-sigmoid bladder
reservoir has been described as an alternative to multiple attempts at redo repair
[42]. Sarin etal. have reported encouraging results from Yogesh’s cystorectostomy,
a modication of Heitz-Boyer-Hovelacque procedure, directly anastomosing the
bladder plate to the recto-sigmoid pouch, without mobilising the ureters. Such procedures may have a role in low- and middle-income settings and signicantly
improve the quality of life, though our preference remains to embark on a redo
closure whenever feasible [43].
Augmentation Cystoplasty
The most serious consequence of failed exstrophy repairs—especially after multiple
closures—is the effect on the bladder growth. Hollowell and Ransley stated that it
was unrealistic to expect the exstrophied bladder to provide compliant continence
without augmentation cystoplasty in majority of cases [35]. Thus, in an already
abnormal bladder, the chances of attaining good capacity and compliance are
reduced drastically after failed repairs. Hence the option of bladder augmentation
must be considered depending on the various adverse factors. The need for bladder
augmentation in failed exstrophy has been reported to be as high as 73–92% [13].
That said, our initial experience with closure of redo bladders does show similar
bladder capacity increase and low risk of upper tract deterioration as compared to
primary bladder closures [32, 44]. Therefore, our preference remains to embark on
a redo bladder exstrophy closure and observe expectantly for an extended period of
time prior to considering bladder augmentation.
The segment used for augmentation can be ileal, ileocaecal, colonic or gastric as
reported by various authors. It is often accompanied by bladder neck tightening/
closure with provision of a continent catheterisable stoma. A complete discussion of
the techniques and outcomes of different modes of augmentation cystoplasty is
beyond the scope of this chapter and can be found elsewhere.
Outcomes ofRedo Repair
There are some common consensuses about outcomes of reclosure after failed
repairs: (1) reclosure with osteotomy has high success rates in terms of the closure;
(2) the outcomes in terms of long-term continence are at best around 50%; and (3)
multiple re-operative surgeries reduce the chances of attaining a good bladder
capacity and consequently continence rates are very low.
Successful repeat closure rates of as high as 95–98% have been reported with
redo MSRE as well as redo CPRE, osteotomy being a critical component of this
success [6, 11, 14]. Hafez etal. have reported 100% success rates with CPRE for
296
R. S. Joshi and J. Ramji
a
b
c
Fig. 17.11 Long-term outcomes of redo CPRE. (a) Follow-up at nine years—continent with voli-
tional voiding and with good bladder capacity. (b) Follow-up at 4 years—redo CPRE for vesicocutaneous stula and outlet obstruction done at age 13 years—continent with volitional voiding
and with good bladder capacity. (c) Follow-up at 5 years—redo CPRE with hypospadiac meatus
done at 8 years of age—continent with good bladder capacity
failed repairs, when performed in adjunct with osteotomy and with adequate postoperative immobilisation [14]. In our experience also, we have had encouraging
results with redo CPRE and modied Salter’s osteotomy with 99.7% successful
closures in 32 cases (unpublished data). Thus, the experienced surgeon faced with
the challenging task of reclosure can nd some optimism in these numbers. For the
frustrated and anxious parents, counselling regarding this attainable result is imperative [11] (Fig.17.11).
Another observation with CPRE is the high number of patients (77%) requiring
a hypospadiac meatus and further surgeries for correction [14]. The purpose of
17 The Failed Exstrophy Repair: Technique andImplications
297
CPRE to decrease the number of surgeries is lost with the requirement of staged
hypospadias correction. Out of the 32 redo cases in our cohort, ten patients required
a hypospadiac meatus. In the past few years, we have been giving the patients preoperative testosterone. Our observation is that with the use of testosterone along
with adequate mobilisation, an orthotopic meatus can be achieved in most patients,
save those who have severe chordee and very short urethral length. Methods for
urethral plate augmentation at the time of CPRE, like use of a transverse island ap
of preputial skin as a tube to achieve an orthotopic meatus, have been reported [45].
Vesicoureteric reux is almost universal after bladder closure, whether primary
or redo. The practice of concomitant ureteric reimplant is still in the evolutionary
stage, but preliminary observations are that the incidence of reux is decreased, and
if present, the grades are low [46, 47]. Reimplant at the time of redo closure is also
possible in carefully selected cases where the bladder is healthy and pliable. Whether
the outcomes in form of reux and hydroureteronephrosis are similar remains to
seen. By preventing or decreasing the grade of reux, the benets would probably
be improved bladder cycling, enabling the previously compromised bladder to grow
better [46]. The detrimental effects of reux causing urinary tract infections with a
high-pressure bladder on the upper tracts may also be neutralised to some extent;
however, this can be corroborated only with long-term analysis of larger cohorts
subjected to this procedure [47].
With all the importance being given to the role of osteotomy, it is essential to also
review the outcomes with respect to redo osteotomy. Many of the failed repairs
occur in spite of an osteotomy at the primary surgery, due to the various other factors involved. A redo osteotomy is almost always necessary in these patients. Studies
have shown that redo osteotomy is feasible and safe without many complications
caused by the repeated disruptions of the bony pelvis [48]. The overall complication
rate of osteotomy, including redo osteotomy, is only 4%, which includes neurologic
complications (nerve palsies, femoral nerve injury and sciatic nerve injury), bony
complications (non-union, malunion) and leg length asymmetry [49]. Novak etal.
reported that the gait was normal in 95% cases even with repeat osteotomy [3]. The
authors have also found redo osteotomy to be safe and effective with three instances
of transient peroneal nerve palsies as the only complication.
While the redo surgery is often successful in experienced hands, the same cannot
be said for the bladder growth and continence rates. The repeated surgical stress on
the bladder, the broses and the pathological changes from a long-exposed bladder
mucosa take a severe toll and consequently hinder the growth and chances of being
dry. According to a large series of failed exstrophy closures reported from Johns
Hopkins, where the patients underwent MSRE and subsequent continence procedures, only 18% of these patients ultimately achieved a good continence outcome
per urethra. Of the rest, 90% were dry, but at the expense of ileocystoplasty and
lifelong catheterisation. As a matter of fact, only 38% of the patients with successful
reclosures were considered suitable for BNR [3].
Similar outcomes have been reported with CPRE done with osteotomy. In the
series reported by Hafez etal., only 14% of successful reclosures achieved continence with volitional voiding. The rest required BNR or bladder closure with
298
R. S. Joshi and J. Ramji
bladder augmentation [45]. Again, we have adopted an observational approach with
early physical therapy for our patients following redo repairs—usually until the
children reach puberty, as long as the upper tracts are safe. At that point, if bladder
capacity, urodynamic ndings and continence history are unlikely to support continence, then further denitive reconstruction such as bladder neck closure with augmentation is considered.
With such low rates of long-term good outcomes, the question that arises is
whether to undertake the arduous task of redo surgeries. Although a secure pelvic
bone approximation is a requisite for successful reclosure, the low continence rates
bring forth the relevance of osteotomy and its morbidities in the setting of a bladder
augmentation. Many authors believe that bladder augmentation is the best option
for the dehisced exstrophy [13]. Mainz and Stein have advocated abandoning any
bladder reconstruction in failed repairs, and instead creating a conduit or rectal reservoir [14]. Many experts believe that this is a viable option in a low-resource
setting.
In light of the drastic consequences of a failed repair, it is important that redo
surgeries should be undertaken only at high-volume centres, which have the expertise and experience to provide optimal outcomes. As such centres are far and few,
the economic burden on the families with respect to prolonged hospital stay and
frequent long-distance travel is high. What then is the justication for planning a
redo surgery with all the drawbacks rather than bladder augmentation or continent
diversion?
Given that our knowledge about the exstrophied bladder is still vastly inadequate, it is impossible to predict which bladders undergoing reclosures will do well
in the future. The chance of continence with volitional voiding is less than 30%, yet
not negligible. Additional benets of redo repairs also include correction of rotational pelvis deformity and pelvis support in females, which should be considered.
It seems logical to propose that redo repair would be a reasonable option in carefully selected cases with a good bladder template; and when undertaken at a centre
with good volume, resources and expertise. The best approach would be to educate
and counsel the families thoroughly about different available options and their possible outcomes, understand their expectations as well as their limitations and
encourage their participation in charting out a plan which is best suited to the needs
of their child.
References
1. Purves JT, Gearhart JP. The bladder exstrophy-epispadias-cloacal exstrophy complex. In:
Gearhart J, etal., editors. Pediatric urology. 2nd ed. Amsterdam: Elsevier; 2010. p.386–415.
2. Elderwy AA, Grady R. The exstrophy epispadias complex. In: Wilcox D, et al., editors.
Pediatric urology: surgical complications and management. Blackwell; 2008. p.83–91.
3. Novak TE, Costello JP, Orosco R, etal. Failed exstrophy closure: management and outcome.
J Pediatr Urol. 2010;6:381–4.
4. El-Sherbiny MT, Hafez AT, Ghoneim MA.Complete repair of exstrophy: further experience
with neonates and children after failed initial closure. J Urol. 2002;168(4):1692–4.
17 The Failed Exstrophy Repair: Technique andImplications
5. Grady RW, Mitchell ME.Complete primary repair of exstrophy: surgical technique. Urol Clin
N Am. 2000;27(3):569–78.
6. Gearhart JP, Peppas DS, Jeffs RD.PAEDIATRIC UROLOGY: the failed Exstrophy closure:
strategy for management. Br J Urol. 1993;71(2):217–20.
7. Gearheart JP, Peppas DS, Jeffs RD.Complications of paraexstrophy aps in the reconstruction
of classical bladder exstrophy. J Urol. 1993;150:627–30.
8. Wild AT, Sponseller PD, Stec AA, Gearhart JP.The role of osteotomy in surgical repair of bladder exstrophy. In: Seminars in pediatric surgery, vol. 20, No. 2. WB Saunders; 2011. p.71–8.
9. Hofmann A, Haider M, Promm M, Neissner C, Badelt G, Rösch WH.Delayed primary closure
of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to
neonatal surgery. J Pediatr Surg. 2022;57(10):303–8.
10. Morrill CC, Haffar A, Harris TG, Crigger C, Gearhart JP.Current management of classic bladder exstrophy in the modern era. Afr J Urol. 2023;29(1):27.
11. Kasprenski M, etal. Modern management of the failed bladder exstrophy closure: a 50-yr
experience. Eur Urol Focus. 2018;6:383. https://doi.org/10.1016/j.euf.2018.09.008.
12. Bhatnagar V.Bladder exstrophy: an overview of the surgical management. J Indian Assoc
Pediatr Surg. 2011;16(3):81–7.
13. Sam CJ, Kurian JJ, Kishore R, Arunachalam P, Sen S.Management and outcome in dehisced
exstrophy with a simplied bladder re-closure and further reconstruction. J Pediatr Urol.
2020;16(6):836–e1.
14. Novak TE.Failed exstrophy closure. In: Seminars in pediatric surgery, vol. 20, No. 2. WB
Saunders; 2011. p.97–101.
15. Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder
exstrophy closure: complications and impact on success. Urology. 2003;62(6):1109–13.
16. Gearhart JE, Ben-Chaim J, Sciortino C, Jeffs RD.The multiple reoperative bladder exstrophy
closure: what affects the potential of the bladder? Urology. 1996;47(2):240–3.
17. Da H.Closure of the exstrophic bladder: an evaluation of the factors leading to its success and
its importance on urinary continence. J Urol. 1989;142:522–4.
18. Purves JT.Modern approaches in primary exstrophy closure. In: Seminars in pediatric surgery,
vol. 20, No. 2. WB Saunders; 2011. p.79–84.
19. Grady RW, Mitchell ME.Complete primary repair of exstrophy. J Urol. 1999;162(4):1415–20.
20. Gearhart JP, Baird A, Nelson CP.Results of bladder neck reconstruction after newborn complete primary repair of exstrophy. J Urol. 2007;178(4S):1619–22.
21. Hammouda HM, Kotb H.Complete primary repair of bladder exstrophy: initial experience
with 33 cases. J Urol. 2004;172(4):1441–4.
22. Wein AJ, Kavoussi LR, Partin AW, Peters CA.Campbell-Walsh urology E-book: 4-volume set.
Elsevier Health Sciences; 2015.
23. Massanyi EZ, Shah B, Schaeffer AJ, DiCarlo HN, Sponseller PD, Gearhart JP.Persistent vesicocutaneous stula after repair of classic bladder exstrophy: a sign of failure? J Pediatr Urol.
2013;9(6):867–71.
24. Baker LA, Jeffs RD, Gearhart JP.Urethral obstruction after primary exstrophy closure: what is
the fate of the genitourinary tract? J Urol. 1999;161(2):618–21.
25. 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(4 Part
2):1696–701.
26. Gearhart JP, Baird AD.The failed complete repair of bladder exstrophy: insights and outcomes. J Urol. 2005;174(4):1669–73.
27. VanderBrink BA, Stock JA, Hanna MK. Aesthetic aspects of reconstructive clitoroplasty
in females with bladder exstrophy–epispadias complex. J Plast Reconstr Aesthet Surg.
2010;63(12):2141–5.
28. Massanyi EZ, Shah BB, Baradaran N, Gearhart JP.Bladder capacity as a predictor of voided
continence after failed exstrophy closure. J Pediatr Urol. 2014;10(1):171–5.
29. Ebert A, Schott G, Hirschfelder H, Rösch WH.Management of failed bladder exstrophy closure in adolescence and adulthood. BJU Int. 2000;86(7):901–4.
299
300
30. Hanna MK, Bassiouny I.Challenges in salvaging urinary continence following failed bladder exstrophy repair in a developing country. J Pediatr Urol. 2017;13:270.e1. https://doi.
org/10.1016/j.jpurol.2016.12.019.
31. Nelson CP, North AC, Ward MK, Gearhart JP.Economic impact of failed or delayed primary
repair of bladder exstrophy: differences in cost of hospitalization. J Urol. 2008;179(2):680–3.
32. Joshi R, Shrivastava D, Ramji J.A model for sustained collaboration to address the unmet global
burden of bladder exstrophy-epispadias complex and penopubic epispadias: the International
Bladder Exstrophy Consortium (vol 153, pg 618, 2018). JAMA Surg. 2018;153(7):692.
33. Woo LL, Thomas JC, Brock JW.Bladder and cloacal exstrophy. In: Coran A, etal., editors.
Pediatric surgery e-book: expert consult-online and print. Elsevier Health Sciences; 2012.
34. Gearhart JP, Mathews R, Taylor S, Jeffs RD.Combined bladder closure and epispadias repair
in the reconstruction of bladder exstrophy. J Urol. 1998;160(3 Part 2):1182–5.
35. Bhatnagar V, Dave S, Agarwala S, Mitra DK.Augmentation colocystoplasty in bladder exstrophy. Pediatr Surg Int. 2002;18:43–9.
36. Salle JP, 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;13:100127.
37. Pippi Salle JL, Jednak R, Capolicchio JP, França IM, Labbie A, Gosalbez R.A ventral rotational skin ap to improve cosmesis and avoid chordee recurrence in epispadias repair. BJU
Int. 2002;90(9):918–23.
38. Barla RK, Sen S.Skin cover in epispadias repair by dorsal transposition of a ventral Island
ap: a modication of the Pippi Salle technique. Pediatr Surg Int. 2015;31:1099–102. https://
doi.org/10.1007/s00383- 015- 3785- x.
39. Friedlander DA, Lue KM, Michaud JE, Gearhart JP, Redett RJ, Di Carlo HN.Repair of vesicocutaneous and urethrocutaneous stulae with rectus muscle ap in a bladder exstrophy patient.
Urol Case Rep. 2017;13:42–4.
40. Michaud JE, Ko JS, Lue K, Di Carlo HN, Redett RJ, Gearhart JP.Use of muscle pedicle aps
for failed bladder neck closure in the exstrophy spectrum. J Pediatr Urol. 2016;12:289.e1.
https://doi.org/10.1016/j.jpurol.2016.03.006.
41. Promm M, Roesch WH.Recent trends in the management of bladder exstrophy: the Gordian
knot has not yet been cut. Front Pediatr. 2019;7:110.
42. Kanojia RP, Rao KL, Menon P, Agarwal S, Bawa M, Mahajan JK, Samujh R.Recto sigmoid
bladder reservoir for patients with exstrophy: three-dimensional evaluation for outcome analysis. J Pediatr Urol. 2014;10(6):1176–80.
43. Sarin YK, Sekhon V.Exstrophy bladder– reconstruction or diversion for the underprivileged.
Indian J Pediatr. 2017;84(9):715–20.
Jul 18. PMID: 28721463.
44. Joshi RS, Eftekharzadeh S, Shukla AR, Ramji J, Hingorani SR, Canning DA, Pippi-Salle JL,
Merguerian P, DeFoor WR Jr, Frazier JR, Weiss D.MP67-04 kidney function outcomes after
complete primary repair of exstrophy: results from the international bladder exstrophy consortium. J Urol. 2021;206(Supplement 3):e510.
45. Hafez AT, El-Sherbiny MT, Shorrab AA, El-Mowa H.Complete primary repair of bladder
exstrophy in children presenting late and those with failed initial closure: single centre experience. J Urol. 2005;174(4):1549–52.
46. Ramji J, Weiss DA, Romao RL, Eftekharzadeh S, Shah J, Frazier JR, Reddy PP, Merguerian
PA, Salle JP, Canning DA, Joshi RS. Impact of bilateral ureteral reimplantation at the time
of complete primary repair of bladder exstrophy on reux rates, renogram abnormalities and
bladder capacity. J Pediatr Urol. 2021;17(3):393–e1.
47. Braga LH, Lorenzo AJ, Jrearz R, Bagli DJ, Salle JL.Bilateral ureteral reimplantation at primary bladder exstrophy closure. J Urol. 2010;183(6):2337–41.
48. Nelson CP, King J, Sponseller PD, etal. Repeat pelvic osteotomy in patients with failed closure of bladder exstrophy: applications and outcomes. J Pediatr Surg. 2006;41:1109–12.
49. Okubadejo GO, Sponseller PD, Gearhart JP. Complications in orthopedic management of
exstrophy. J Pediatr Orthop. 2003;23(4):522–8.
https://doi.org/10.1007/s12098- 017- 2419- 9. Epub 2017
R. S. Joshi and J. Ramji
Perioperative Management ofBladder
Exstrophy
AnjanaKundu, ArvidYung, andRitaAgarwal
Background
Bladder exstrophy is a rare congenital malformation of the genitourinary tract
affecting approximately 1in 50,000 live births, more commonly among males [1, 2]
and with a preponderance in low- and middle-income countries (LMICs) [3, 4]. The
bladder exstrophy-epispadias complex (BEEC) refers to a malformation including a
severe midline abdominal wall defect, wide separation of the pubic symphysis, and
an anteriorly positioned open bladder and urethra. Epispadias and classical bladder
exstrophy may occur in isolation, but cloacal exstrophy often carries with it various
other malformations of the gastrointestinal tract, skeletal system, and neurological
system [5]. The bladder remains open on the abdominal wall in both classical bladder exstrophy and cloacal exstrophy.
A surgical repair involves complex and extensive reconstruction of the bladder
and its adjacent structures to restore satisfactory functional and cosmetic anatomical
integrity, with the goal of achieving urinary continence and a high quality of life.
The surgical techniques have been described in detail in the earlier chapters and
consist of several different techniques. The most common surgical repair techniques
include either a single-staged closure of the bladder and genitalia, i.e., complete
primary repair (CPRE) [4, 6] or a planned modern staged repair (MSRE) [7, 8].
Although the majority of surgical repair in high-income countries (HICs) occurs
within days of birth, in LMICs, these defects may either go unrepaired well into
18
A. Kundu (*) · A. Yung
Department of Anesthesiology and Perioperative Medicine, Golisano Children’s Hospital and
University of Rochester, Rochester, NY, USA
e-mail: Anjana_Kundu@URMC.Rochester.edu
R. Agarwal
Department of Anesthesiology, Lucille Packard Children’s Hospital, Stanford University,
Palo Alto, CA, USA
© 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_18
301