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12 Osteotomies forBladder Exstrophy
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rate with a comprehensive approach involving osteotomies, external xation, and Buck’s traction [21]. However, attributing the success solely to osteotomies proves challenging due to inherent patient differences between those who receive osteoto­mies and those who do not. Nonetheless, cases of primary closure failure docu­mented in the literature often lacked osteotomies. Long-term orthopedic outcomes generally lean toward favorability, with most patients achieving normal activity lev­els and mobility comparable to the general population. However, potential long­term adverse outcomes may include hip dysplasia, osteoarthritis, and excessive external rotation foot progression angle [7].
Complications associated with orthopedic reconstruction are generally rare. According to Okubadejo etal., there is a reported 4% rate of orthopedic complica­tions, excluding pin site problems [15]. Although nerve palsies may occasionally occur following osteotomies, they tend to be transient in nature. Non-union at the osteotomy site is infrequent but may arise with larger corrections. Wound complica­tions, especially those linked with posterior incisions, are not uncommon. Additionally, pressure sores resulting from casts or traction devices require vigilant monitoring and appropriate management, as they represent the most prevalent orthopedic issue [15].

References

1. Baird AD, Sponseller PD, Gearhart JP.The place of pelvic osteotomy in the modern era of bladder exstrophy reconstruction. J Pediatr Urol. 2005;1(1):31–6. https://doi.org/10.1016/j.
jpurol.2004.09.001. PMID: 18947531.
2. Cardin S, Herrera-Soto J, Marrero P, Rich M, Swana H, Langford J.Pelvic ring stabilization using anterior subcutaneous internal xation in bladder exstrophy repair. J Pediatr Orthopaedic Soc North Am. 2020;2(3):132.
3. de Mattos CB, Mendes PH, Boechat PR, Júnior JL, da Silva Guimarães L. Bilateral anterior pelvic osteotomy for olosure of bladder exstrophy: description of technique. Rev Bras Ortop. 2015;46(1):107–13. PMCID: PMC4799192.
4. Frey P, Cohen SJ. Anterior pelvic osteotomy. A new operative technique facilitating pri­mary bladder exstrophy closure. Br J Urol. 1989;64(6):641–3. https://doi.org/10.1111/
j.1464- 410x.1989.tb05327.x. PMID: 2627639.
5. Giordano M, Di Lazzaro A, Guzzanti V, Careri S, Bagolan P, Zaccara A, Toniolo R.Oblique pelvic osteotomy in the treatment of bladder exstrophy in neonates. J Pediatr Orthop B. 2019;28(3):207–13. https://doi.org/10.1097/BPB.0000000000000614.
6. Grady RW, Mitchell ME.Complete primary repair of exstrophy. J Urol. 1999;162(4):1415–20. PMID: 10492227.
7. Jani MM, Sponseller PD, Gearhart JP, Barrance PJ, Genda E, Chao EY.The hip in adults with classic bladder exstrophy: a biomechanical analysis. J Pediatr Orthop. 2000;20(3):296–301. PMID: 10823593.
8. Jones D, Parkinson S, Hosalkar HS.Oblique pelvic osteotomy in the exstrophy/epispadias complex. J Bone Joint Surg Br. 2006;88(6):799–806.
X.88B6.17712. PMID: 16720777.
9. Kenawey M, Morakis E, Cervellione R, Keene D, Kelley SP.The true pelvic volume change with various corrective osteotomy techniques for exstrophy-epispadias complex spectrum: the
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value of computer-assisted virtual surgery. J Pediatr Orthop B. 2023. https://doi.org/10.1097/
BPB.0000000000001145. Epub ahead of print. PMID: 38189741.
10. Lloyd-Roberts GC, Williams DI, Braddock GT.Pelvic osteotomy in the treatment of ecto­pia vesicae. J Bone Joint Surg Br. 1959;41-B:754–7.
X.41B4.754. PMID: 13853691.
11. McKenna PH, Khoury AE, McLorie GA, Churchill BM, Babyn PB, Wedge JH.Iliac oste­otomy: a model to compare the options in bladder and cloacal exstrophy reconstruction. J Urol. 1994;151(1):182–6; discussion 186–7. PMID: 8254810.
12. 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.
https://doi.org/10.1016/s0090- 4295(03)00791- x. PMID: 14665365.
13. Mundy A, Kushare I, Jayanthi VR, Samora WP, Klingele KE.Incidence of hip dysplasia asso­ciated with bladder exstrophy. J Pediatr Orthop. 2016;36(8):860–4. https://doi.org/10.1097/
BPO.0000000000000571. PMID: 26090968.
14. Nhan DT, Sponseller PD. Bilateral Anterior Innominate Osteotomy for Bladder Exstrophy. JBJS Essent Surg Tech. 2019;9(1):e1. https://doi.org/10.2106/JBJS.ST.18.00018. PMID: 31086719; PMCID: PMC6485763.
15. Okubadejo GO, Sponseller PD, Gearhart JP. Complications in orthopedic management of exstrophy. J Pediatr Orthop. 2003;23(4):522–8. PMID: 12826954.
16. O’Phelan EH. Iliac osteotomy in exstrophy of the bladder. J Bone Joint Surg Am. 1963;45:1409–22. PMID: 14069780.
17. Schmidt AH, Keenen TL, Tank ES, Bird CB, Beals RK.Pelvic osteotomy for bladder exstro­phy. J Pediatr Orthop. 1993;13(2):214–9. PMID: 8459014.
18. Shnorhavorian M, Song K, Zamilpa I, Wiater B, Mitchell MM, Grady RW. Spica casting compared to Bryant’s traction after complete primary repair of exstrophy: safe and effec­tive in a longitudinal cohort study. J Urol. 2010;184(2):669–73. https://doi.org/10.1016/j.
juro.2010.03.057. Epub 2010 Jun 19. PMID: 20639033.
19. Shultz WG.Plastic repair of exstrophy of bladder combined with bilateral osteotomy of ilia. J Urol. 1958;79(3):453–8.
20. Sponseller PD, Bisson LJ, Gearhart JP, Jeffs RD, Magid D, Fishman E.The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am. 1995;77(2):177–89. https://doi.
org/10.2106/00004623- 199502000- 00003. PMID: 7844123.
21. Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am. 2001;83(2):184–93. https://doi.
org/10.2106/00004623- 200102000- 00005. PMID: 11216679.
22. Stec AA, Pannu HK, Tadros YE, Sponseller PD, Fishman EK, Gearhart JP. Pelvic oor anatomy in classic bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol. 2001;166(4):1444–9. PMID: 11547108.
23. Suson KD, Sponseller PD, Gearhart JP. Bony abnormalities in classic bladder exstrophy: the urologist’s perspective. J Pediatr Urol. 2013;9(2):112–22. https://doi.org/10.1016/j.jpu-
rol.2011.08.007. Epub 2011 Nov 21. PMID: 22105005.
24. Vining NC, Song KM, Grady RW. Classic bladder exstrophy: orthopaedic sur­gical considerations. J Am Acad Orthop Surg. 2011;19(9):518–26. https://doi.
org/10.5435/00124635- 201109000- 00002. PMID: 21885697.
25. Wakim A, Barbet JP.Connections of the bladder plate and bladder neck with the bony pelvis in a fetus with classic bladder exstrophy. Urology. 2002;60(1):142–6; discussion 146. https://
doi.org/10.1016/s0090- 4295(02)01715- 6. PMID: 12100941.
26. Wild AT, Sponseller PD, Stec AA, Gearhart JP.The role of osteotomy in surgical repair of bladder exstrophy. Semin Pediatr Surg. 2011;20(2):71–8. https://doi.org/10.1053/j.semped-
surg.2010.12.002. PMID: 21453849.
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12 Osteotomies forBladder Exstrophy
27. Yazici M, Sözübir S, Kilicoglu G, Bernay F, Incesu L, Ariturk E.Three-dimensional anatomy of the pelvis in bladder exstrophy: description of bone pathology by using three-dimensional computed tomography and its clinical relevance. J Pediatr Orthop. 1998;18(1):132–5. PMID:
9449115.
28. Yazici M, Kandemir U, Atilla B, Eryilmaz M. Rotational prole of lower extremi­ties in bladder exstrophy patients with unapproximated pelvis: a clinical and radiologic study in children older than 7 years. J Pediatr Orthop. 1999;19(4):531–5. https://doi.
org/10.1097/00004694- 199907000- 00022. PMID: 10413008.
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Common Adjuvant Surgeries inExstrophy/Epispadias Care
ElizabethRoth, TravisGroth, andJohnKryger

Ureteral Reimplantation

Among the many considerations for surgeons at the time of bladder exstrophy repair is whether or not to perform ureteral reimplantation at the time of initial closure. Due to the anterior herniation of the bladder in bladder exstrophy, the ureter inserts poste­riorly into the bladder and closer to the bladder neck. There is a signicantly shorter intramural tunnel. VUR is present in a large proportion of bladder exstrophy patients after initial bladder closure, with estimates of >75% prevalence postoperatively, Dickson [9]. Recurrent pyelonephritis within this population typically affects 20–50% of patients in the 90-day postoperative period, even with the use of continuous antibi­otic prophylaxis, Braga [3], Grady [14]. In certain surgical approaches, such as Young­Dees-Leadbetter bladder neck reconstruction or Kelly radical soft tissue mobilization, ureteral reimplantation may be required to facilitate reconstruction without undue risk of ureteral obstruction, Dickson [9], Tourchi [25]. With complete primary repair of exstrophy (CPRE), there is better bladder urinary cycling due to enhanced sphincter tone at the bladder neck. However, longer periods of urine holding and bladder pres­sure can also result in worsening VUR.This results in a pressure pop-off mechanism for the bladder and can compromise bladder distension during cycling and can result in worsening hydronephrosis. There may be a theoretical improvement in bladder cycling from bilateral ureteral reimplant (BUR) to facilitate better bladder distension during the dry intervals between voiding. Ureteral reimplant has also reduced hydro­nephrosis that is secondary to VUR.Lastly, correction of VUR will reduce the inci­dence of UTI.There is some evidence for larger bladder capacity at age 4years in children closed with a CPRE-BUR combined approach, Ramji [22].
13
E. Roth · T. Groth · J. Kryger (*) Pediatric Urology, Children’s Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA e-mail: Eroth@childrenswi.org; Tgroth@childrenswi.org; Jkryger@childrenswi.org
© 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_13
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E. Roth et al.
Cephalo-trigonal or cross-trigonal approaches may be considered and should be driven by the general shape of the bladder plate and which approach will achieve the most optimal tunnel length. With either technique, the procedure starts by identify­ing each ureteral orice and cannulating with a small stent or feeding tube (3.5–5 Fr). Each ureteral orice is circumscribed and dissected free of the intramural tun­nel. The trajectory of the ureter will be more medial and caudal than in primary VUR, and there is an increased risk for inadvertent ureteral injury if the altered trajectory is not appreciated. Once the ureter is adequately freed from the intramural tunnel, a new trajectory can be plotted.
Cephalo-trigonal Technique The cephalo-trigonal approach to ureteral reimplan-
tation is a bladder exstrophy-specic surgical technique rst described by Canning etal. [4]. It utilizes the known altered trajectory of the ureter in exstrophy patients. There is a posterior insertion of the ureter that enters near the bladder neck. It natu­rally angles in a cephalad direction. The reimplant is further complicated by a very small bladder. This technique advances the orice cranially. This allows advance­ment and elongation of the intramural tunnel while preserving the laterality of the orice and minimizes risk for extravesical kinking of the ureter that would lead to obstruction.
After dissection of the ureter, a submucosal tunnel is sharply created from the native entry site in a cranial direction on the ipsilateral side of the bladder (Fig.13.1). As in other ureteral reimplantation techniques, the tunnel length should ideally be 3–5 times the width of the ureter to ensure optimal resolution of VUR postopera­tively. Ideal tunnel length may not be possible with a small bladder plate or with a bladder plate with modest craniocaudal dimensions. Care must also be exercised to consider directional shifts of the ureteral trajectory that may occur with correspond­ing bladder closure. After passing the ureter through the tunnel to the neo-orice, the ureter is secured to the adjacent mucosa with an absorbable suture in an inter­rupted fashion. The neo-orice is then assessed by passing the stent through the reimplanted ureter to ensure a gentle trajectory that passes easily in a retrograde direction. The process is then repeated with the contralateral ureter.
Advantages of a cephalo-trigonal technique include minimization of extravesical ureteral obstruction by taking advantage of the unique trajectory of the ureter in bladder exstrophy and maintaining the laterality of each ureter relative to the mid­line. Disadvantages include limited tunnel length with a small bladder plate as well as difculty accessing the ureters cystoscopically through the urethra due to the cranial location and caudal trajectory of the intramural tunnel.
Cross-trigonal Technique (Cohen) First described as a technique for primary
VUR, the Cohen cross-trigonal technique is also well suited for use in bladder exstrophy patients, especially in those where the transverse dimensions of the blad­der plate exceed the craniocaudal dimensions, Cohen [6]. With this approach, the ureter is mobilized as above, but the submucosal tunnels are created sharply to advance the right ureter across the midline to exit on the left and the left ureter
13 Common Adjuvant Surgeries inExstrophy/Epispadias Care
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Fig. 13.1 Cephalo-trigonal ureteral reimplant
across the midline to exit on the right (Fig.13.2). The surgeon will have to choose which ureter to place cranially based on the trajectory and size of the ureters overall. A 1:3–5 ratio of ureteral width to tunnel length is again recommended. The ureteral orice is secured to the adjacent mucosa at the new site with an absorbable suture. A small-caliber catheter or stent is again placed retrograde to ensure a smooth tra­jectory of the ureter. Care must be taken to adequately mobilize the ureter to ensure that there is a smooth trajectory for the proposed new ureteral hiatus to avoid issues with extravesical kinking leading to ureteral obstruction. A small segment of the excess ureter may need to be excised to facilitate a smooth trajectory of the reim­planted ureter. Surgeons must also consider how bladder closure may alter ureteral trajectory and avoid advancing the tunnel signicantly up the bladder sidewall to avoid obstruction.
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Fig. 13.2 Cross-trigonal ureteral reimplant
E. Roth et al.
Advantages to the Cohen cross-trigonal approach include maximizing tunnel length in patients with limited craniocaudal bladder plate dimensions, better cysto­scopic access to the ureters for future urologic surgical needs (but it can still be difcult), and familiarity of the technique to most pediatric urologists and surgeons. Limitations include increased risk for extravesical ureteral obstruction unless famil­iar with technical modications for exstrophy and frequent need to resect a portion of distal ureter to ensure an appropriate ureteral trajectory.
Outcomes Multiple surgical groups have published clinical outcomes from blad-
der exstrophy patients who underwent ureteral reimplantation at the time of initial bladder closure, indicating that it is safe and technically feasible in experienced hands, Dickson [9], Braga [3], Ramji [22], Garat [13], Jarosz [17]. Documented clinical benets include decreased incidence of febrile UTI, Braga [3], decreased renal scar on nuclear scintigraphy, Dickson [9], and decreased need for subsequent ureteral surgery, Dickson [9], Braga [3], Jarosz [17]. While the rate of VUR is decreased in reported cohorts, the overall rate of postoperative VUR in exstrophy patients is signicantly higher than in primary VUR literature (0–40% versus <5%), Dickson [9], Braga [3], Jarosz [17].
Other proposed benets of early ureteral reimplantation are improved bladder cycling efciency, potentially leading to the development of greater bladder capac­ity. The international group based in India noted increased bladder capacity at age 4years in patients who underwent concurrent BUR at initial closure, though this nding is likely multifactorial, Ramji [22]. In our experience, we have found BUR at the time of initial closure to be helpful in patients with large bladder plates, both male and female.
In carefully selected patients, reimplantation can facilitate improved bladder cycling, protect against recurrent pyelonephritis in the postoperative period, and possibly improve capacity and potential for volitional voiding. However, this must be weighed against the additional operative time, increased tissue handling and
13 Common Adjuvant Surgeries inExstrophy/Epispadias Care
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mobilization required, and additional surgical complexity added to an already com­plex surgical problem. Thus treating surgeons must individualize the decision on whether bilateral ureteral reimplantation is warranted on a case-by-case basis.

Inguinal Hernia

It has been well established that classic bladder exstrophy patients have a signi­cantly higher risk of developing inguinal hernias when compared to the general population. Connolly [7] The historically reported incidence of inguinal hernias in classic bladder exstrophy ranges from 56% to 86% in males and 8% to 15% in females. There have been several theories for this, which include innate weakness of the abdominal wall, lack of obliquity of the inguinal canal, along with the increase in intra-abdominal pressure after bladder exstrophy closure, Husmann [16], Muecke [21].
Several studies have assessed risk factors for inguinal hernias in classic bladder exstrophy patients. Male gender is a signicant risk factor for developing an inguinal hernia. Most recently in 2021, Lee etal. reported that 64.2% (34 of 53) of males devel­oped inguinal hernias and only 5.3% of (2 of 38) females developed inguinal hernias after CPRE in patients with bladder exstrophy, Lee [19]. It has been previously reported by Lavien etal. that pelvic osteotomy is associated with a decreased inci­dence of inguinal hernia. In this series, 25% of patients developed an inguinal hernia if an osteotomy was performed versus 46% if an osteotomy was not performed. In their study, both female sex and osteotomies were associated with decreased risk for development of an inguinal hernia along with decreased risk for recurrence. Age at bladder closure was not associated with hernia formation, Lavien [18].
Inguinal hernias in classic bladder exstrophy patients are at risk for incarcera­tion. Lee etal. assessed the timing of inguinal hernias following CPRE. In their study, 17% of patients presented with incarcerated hernias. The greatest risk for inguinal hernia formation was within the rst 6 months following CPRE.They also noted decreased rates of development of inguinal hernias after 12months of age, Lee [19]. Ellison et al. reported that simultaneous hernia closure at the time of CPRE was safe and decreased the risk of future development of inguinal hernias. In their study, 36% (9 of 25) of patients developed inguinal hernias if not repaired at the time of closure versus 11% (2 of 18) who developed inguinal hernias if previ­ously repaired at the time of CPRE, Borenstein [2].
The recurrence rate for inguinal hernias is signicantly higher in the bladder exstrophy population than in the general pediatric population. Previous studies have demonstrated a recurrence rate of 17–34% following inguinal hernia repair follow­ing bladder exstrophy closure versus a 0.3–1.1% recurrence rate in the general pedi­atric population, Stringer [23], Lavien [18], Borenstein [2], Ein [10].
Several different approaches for inguinal hernia repairs have been described in bladder exstrophy patients. This includes a pre-peritoneal approach by Connely etal., which is typically performed at the time of bladder closure and standard open inguinal approach, Connolly [7], Ellison [11].
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Monsplasty

Another important adjuvant surgical step is the monsplasty. Patients with bladder exstrophy experience signicant diastasis of the pubic rami. This results in the separation of the soft tissues above the pubic rami, which is the mons pubis. Even after surgical approximation of the pubic rami, there is separation of the mons pubis in the midline. Often there can be a deep cleft in the pubic midline. This gap can slowly worsen over time as the bony pelvis grows and the pubic diasta­sis widens.
It has been especially signicant in the appearance of the female genitalia that appears more open at the introitus, with a at or concave mons pubis and bid cli­toris and clitoral hood. Long-term data have shown that women with exstrophy­epispadias complex report dissatisfaction with their genital appearance, Mathews [20], Woodhouse [27]. The appropriate reconstruction of the mons pubis and clito­ral structures can greatly enhance the cosmetic appearance of the genital area for girls and boys.
After the approximation of the pubic bones at initial closure, then the mons­plasty is undertaken, Cook [8]. This can also be performed in subsequent surger­ies at any age. If it is being performed at a later stage of surgery, then the rst step is to excise the scar in the midline along its length and excise the non-hair-bearing skin. This will create the natural appearance of the escutcheon at nal closure. The lipoma of the mons pubis is then mobilized from each side of the pubis by dissecting the cephalad and lateral and anterior edges until each lipoma can be freely mobilized to the midline. The lipomas are approximated in the midline with layers of 4–0 or 5–0 absorbable interrupted sutures. This creates one central mons above the pubic bones. Sometimes they are sufciently generous in size to allow them to overlap in the midline and further reduce future separation. The skin edges are excised as much as tension allows to remove non-hair-bearing skin to create the natural appearance of the escutcheon. The skin is approximated cos­metically with absorbable sutures and often Steri-Strips to reduce tension on the skin edges during healing.
Prior to completing the skin closure of the mons, the clitoral hood is recon­structed. The clitoral bodies can be approximated in the midline, and the medial edges of the glans clitoris can be de-epithelialized and approximated with ne absorbable monolament sutures. But we often do not perform direct approxima­tion of the clitoral bodies on newborn closure to avoid neurovascular injury. Then each clitoral hood can be easily approximated in the midline to enhance the cos­metic natural appearance of the genitalia. The technique described by Grady is a vertical z-plasty, Weiss [26]. Each hemi-clitoral hood is incised along it’s medial edge to create two layers. The inferior layers are approximated in the midline with ne absorbable monolament suture to form the posterior layer of the clitoral hood. Then the superior layers are approximated along their medial edges.
These steps to enhance the mons pubis and clitoral bodies are rather easy steps that add considerable improvement to the pubo-genital appearance.
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Umbilicoplasty

Due to the anterior abdominal wall defect in bladder exstrophy, affected children are born with an abnormally low-set umbilical cord at the insertion of the urachus on the exposed bladder dome, rather than the typical location approximately 2/3 of the way between the xiphoid and the pubic symphysis. The umbilicus plays an impor­tant aesthetic landmark on the abdomen. The surgical reconstruction of a neoumbi­licus should be performed in conjunction with other exstrophy surgeries to create an orthotopic neoumbilicus.
A variety of techniques have been described, Sumfest [24], Cervellione [5], Feyaerts [12], Barroso [1], and Hanna [15]. The site of the neoumbilicus is selected by tracing a line across the anterior abdominal wall between the anterior superior iliac crests. The neoumbilicus is centered on this axis in the midline. Next, a ap or free graft of adjacent skin is selected. The area under the graft or ap is defatted down to the anterior abdominal fascia, and the skin is secured in this location to form an indented neoumbilicus. Three examples of neo-umbilicoplasty (NU) tech­niques are diagrammed below: Free graft, spiral-ap (S-ap), and rhomboid ap. We conducted an unpublished patient satisfaction survey of our MIBEC patients who were at least 3years postoperative from umbilicoplasty. The preference was for free graft NU (Figs.13.3, 13.4, 13.5, 13.6, 13.7, 13.8, and 13.9).
Fig. 13.3 Free Graft NU.A circular graft is harvested from the intended neo-umbilical site which is located at the intersection of the midsagittal line and the level of the iliac crest. After the free graft and underlying fascia have been defatted, the free graft is centrally secured to the exposed fascia with permanent or absorbable monolament