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R. S. Joshi and J. Ramji
upto the levator hiatus with the urogenital bres lateral to the vesicourethral unit. This is important to prevent the anterior displacement when the pubes are brought together [22]. Gearhart etal. emphasise that during the primary closure, the bladder neck and posterior urethra must be long enough and tight enough to prevent bladder prolapse/ eversion of bladder mucosa, at the same time allowing unimpeded egress of urine [6].
Vesicocutaneous stulae are technically considered failures, as the management would necessarily imply a redo surgery [23].
Bladder Neck andOutlet
Bladder outlet obstruction is another troublesome form of failed closure. The severe consequences of outlet obstruction on outcomes are main reasons why no formal bladder neck reconstruction is attempted at the primary repair in multi stage repair of exstrophy (MSRE); rather, a bladder outlet is created, which is incontinent, yet pro­vides enough resistance to stimulate bladder growth [14]. In CPRE also, although the epispadias repair is done simultaneously, there is no bladder neck reconstruction. The symphyseal suture may contribute to stricture formation due to erosion into the posterior urethra. Gearhart and associates have reported a high incidence of strictures with the use of long paraexstrophy aps during bladder closure in MSRE, which are prone to ischaemic necrosis. Ureteric stents exiting from the neourethra may be another culprit, hence the importance of proper placement of tubes and their post­operative management. The neourethra must be calibrated before removing the suprapubic tube [2, 24].
Outlet obstruction has also been observed in CPRE and Kelly’s repair, in form of complete obliteration of the urethra. It is unclear whether this is due to technical problems during extensive mobilisation causing ischaemia to the urethra [2].
The possibility of narrowing of the outlet can be suspected if there are episodes of febrile urinary tract infections or development of hydronephrosis on follow-up [6]. Bladder stones, difcult catheterisation, prolonged dry intervals, straining to void with residual urine or recurrent episodes of epididymitis may be some other presentations indicating outlet obstruction [24].
Complications like loss of urethral plate, glans, corporal body and clitoris may happen in isolation or along with major modes of failure, constituting a ‘complex failure’. Such soft tissue loss may be because of the radical dissection, more so in CPRE [25]. The penile disassembly technique done incorrectly, accentuated by vagaries in the blood supply of the penis, may contribute to an ischaemic injury to the phallus [22]. Other reasons for the soft tissue injury could be arterial spasm, venous congestion subsequent to compartment syndrome after a tight pubic closure or an abnormal blood supply [25, 26]. Atrophy of one or both clitoral bodies has also been observed, a probable consequence of ischaemic insult [27]. Partial or complete loss of the urethral plate could result from damage to the terminal arteries during dissection. Inadvertent injury to the bulbar artery as it exits from the com­mon penile artery into the corpus spongiosum can lead to complete loss of the ure­thral plate [25]. In girls, loss of the urethrovaginal septum has been reported secondary to the use of para-exstrophy aps [26] (Fig.17.2).
17 The Failed Exstrophy Repair: Technique andImplications
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a
c
b
d
ef
Fig. 17.2
Complete dehiscence in a female. (c) Failed CPRE—bladder dehiscence with intact epispadias repair. (d) Bladder prolapse in a male. (e) Prolapsing bladder in a female. (f) Vesicocutaneous s­tula. (g) Failed epispadias repair. (h) Stenosed hypospadiac meatus after CPRE causing outlet obstruction. (i) Complex failure—loss of corpora. (j) Complex failure—loss of hemiglans
Various modes of failed exstrophy repair. (a) Complete dehiscence in a male. (b)
284
ij
R. S. Joshi and J. Ramji
g
h
Fig. 17.2 (continued)
Consequences ofFailed Repairs
A failed repair has negative long-term impacts on the patient and the healthcare system [11]. The consequences of one or more failed repairs are increased scar for­mation and collagen deposition, decreased bladder growth, decreased capacity and decreased rates of voided urethral continence [28]. A successful primary bladder closure is the cornerstone to achieve sufcient bladder capacity and eventually con­tinence. Each failed repair decreases the potential to gain adequate bladder capacity [29]. Failure of the primary closure translates as a failure to get adequate bladder capacity in over 50% patients. The MSRE uses the bladder capacity as the main determinant for BNR.Kasprenski M etal. have reported that BNR alone as a conti­nence procedure was successful in only 53.2% of the patients who were eligible for this procedure [11]. Thus, only half of those who acquire reasonable capacity are continent subsequently, suggesting that other factors like bladder compliance also
17 The Failed Exstrophy Repair: Technique andImplications
Fig. 17.3 Small capacity bladder with scarred abdominal wall after failed repairs
285
play an important role [26]. The chances of continence with volitional voiding go down to 30% with two closures and further to 20% with three or more closures [2].
Repeated surgical insults can also lead to brosis and contracture of the bladder plate. This leads to a low-volume stiff bladder after reclosure. Peppas etal. have shown the ratio of collagen to smooth muscle to be much higher in bladders that have been subjected to multiple closures. The increased collagen formation decreases the elasticity and compliance of the bladder, preventing the bladder from reaching its full capacity with growth [16]. Hanna MK etal. report changes of cys­titis cystica, cystitis glandularis and squamous metaplasia on bladder biopsy in cases of failed repairs [30].
The scarring from previous surgery also increases the difculty of subsequent repair, particularly the bladder neck reconstruction. It has been suggested by many that 4–6months should be allowed for the wound to mature. However, waiting for too long a period may aggravate the chronic inammation of the exposed bladder plate and make it stiff and brotic [16] (Fig.17.3).
Consequences of Stricture and Obstruction Posterior urethral stricture and subse­quent outlet obstruction impair the success of any repair signicantly. This is a potentially dangerous complication as it poses a serious threat to the upper tracts and slowly leads to deterioration of renal function [2, 22, 24] (Fig.17.4).
Redo surgeries and other salvage procedure after multiple failures are compli­cated, requiring longer surgical and post-operative recovery time [31]. The cost to the patient, resource utilisation and burden on healthcare system is compounded signicantly when the primary repair fails.
The afore-mentioned clinical and economic consequences of failure have raised the question of centralisation of bladder exstrophy care. Patient outcomes may be better optimised if the surgeries are done exclusively by teams with expertise earned with large volume of cases. The improved outcomes at such high-volume centres of excellence would probably offset the travel obstacles and costs [11, 31]. Long-term
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a
c
b
Fig. 17.4 Effects of outlet obstruction. (a) High pressure bladder with reux. (b) Stone forma- tion. (c) Compromised renal function
17 The Failed Exstrophy Repair: Technique andImplications
287
multi-institutional collaboratives involving experts from high-income countries combined with local experience at well-equipped dedicated centres in middle- and low-income countries are proving to a feasible model for alleviating the disease burden in these areas [32].

Management

The initial management of a failed repair is conservative with debriding the devital­ised tissue, removal of foreign bodies and treating wound infection with culture­appropriate antibiotics. There should be no attempts at immediate repeat closure. A minimum interval of 4–6months is considered optimal before attempting any surgi­cal procedure [3, 11]. This allows time for the wound to mature and the inamma­tion to subside.
Before planning any surgical procedure for failure, a complete evaluation is man­datory. This should include examination under anaesthesia, pelvic x-rays, ultra­sound and renal scans. Cystograms and cystoscopy are indicated in cases of prolapse, vesicocutaneous stula or bladder outlet obstruction, while CT scans or MRI is recommended when previous osteotomies have been done.
The options for a surgeon managing a failed exstrophy repair are delayed redo bladder closure, redo bladder closure with epispadias repair, redo bladder closure with concomitant bladder augmentation, excision of the bladder and continent uri­nary diversion, creating a urinary conduit [6, 33]. The precise management needs to be individualised to correct the factors culpable for the initial failure. It is also nec­essary to consider the socio-economic circumstances, quality of life, and perception of body image. In adolescents presenting with failure, continent urinary diversion may be planned early [29].
Although a failed repair decreases the chances of a compliant, good capacity and continent bladder, all failed primary repairs should be given a chance for redo repair, if the bladder plate is healthy and genital reconstruction is possible.
Redo Exstrophy repair may be undertaken as a single-stage CPRE or as MSRE.The basic tenets of a redo repair include: (1) radical dissection of the blad­der and posterior urethra, (2) deep placement of the bladder, bladder neck and ure­thra as one unit in the pelvis, (3) osteotomy with external xators or traction to secure post-operative immobilisation, (4) adequate post-operative sedation, pain relief and control any distracting forces and (5) pre-operative intramuscular testos­terone, which by increasing phallic length and girth may reduce likelihood of inad­vertent injury [26].
The redo repair outlined below is what the authors follow and is much the same as the complete primary repair described in another chapter in this textbook. The challenge at the outset is to enter the correct surgical planes between the bladder detrusor and rectus fascia circumferentially around the bladder plate. The scarred and brous tissue from the previous failed repair will often render these planes dif­cult to identify, so care must be taken to ensure that the detrusor muscle of the bladder is preserved. Once the planes are identied, the goal is to radically dissect
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the vesicourethral unit, releasing all the adhesions from the previous surgery. The planes between the detrusor muscle and fascia are often easiest to visualise laterally, where the dissection begins to identify the medial border of the anterior rectus sheath. The idea is to enter the perivesical fat and dissect the bladder superiorly and inferiorly on either side. The supercial ligaments of the bladder are divided, but it is recommended not to continue with the deep pelvic dissection at this point to avoid injury to the corpora cavernosa that may not be fully visualised in this anterior plane.
Rather, the superior dissection at the dome of the bladder is performed, separat­ing the peritoneum and supercial adhesions from the dome. In redo cases, some­times it is impossible to avoid entering the peritoneum. If this occurs, the peritoneotomy may even facilitate lateral dissection when the planes are especially obliterated from previous surgery.
Once this lateral dissection is completed in an antegrade direction up to the lat­eral intersymphyseal bands, we proceed to the penile skin dissection. Regardless of whether a previous epispadias repair has been done, the penile skin is degloved, the neurovascular bundles are identied all the way to their course underneath the bone at the corporal insertion into the pubic bone. Often this dissection is particularly difcult as the brosis from the previous surgery can cause the bundles to be dis­placed from their original position. Once the bundles are safeguarded, the deep intersymphyseal bands are accurately identied and divided under vision (Fig.17.5). The radical division of all the bands in this area between the bladder neck, urethra and the pubis helps to completely free the bladder plate, which can then be easily lifted up or pushed into the pelvis without any restriction. It would be worth bearing in mind that enthusiastic medial dissection at this point could compromise the width of the bladder neck.
Often, a failed repair may also have a failed epispadias repair, or it might be a failure of the rst stage of bladder closure in a MSRE, where the epispadias repair had not been undertaken. It is invariably necessary to dissect the urethral plate or the reconstructed tube off the corpora and redo the epispadias repair to adequately mobilise the vesicourethral unit. The modied Cantwell-Ransley technique is applied, separating the urethral plate rst ventrally and then dorsally starting at
Fig. 17.5 Lateral dissection—identication and division of deep intersymphyseal bands
17 The Failed Exstrophy Repair: Technique andImplications
289
midshaft and moving proximally and distally. The broses from the previous sur­gery make this dissection tedious. The proximal prostatic and bladder neck dissec­tion is tricky, where the urethra may get thin or narrow. Similarly, the distal dissection from the glans may be troublesome. The tip of the urethral plate is kept connected to the glans. Sometimes, however, the proximal radical mobilisation may shorten the urethra, or the distal plate may not be supple enough, or may have been damaged in the previous surgery. In such cases, it is more advisable to give a hypospadiac meatus, as described by Mitchell and Bagli’s modication with a complete disas­sembly [22]. The pre-operative use of testosterone has been advocated as a means to enhance the growth of the urethral plate, increase its pliability and vascularity and minimise the incidence of hypospadias, especially in redo cases and older children [26, 34]. The authors have also been able to obtain an orthotopic meatus in most cases with the use of pre-operative testosterone enanthate intramuscular injections (2mg/kg at 5weeks and 3weeks before surgery) combined with topical application of testosterone cream.
Concomitant bilateral ureteric reimplantation may be possible in some redo repairs based on the size and quality of the bladder plate. A cephalotrigonal or cross­trigonal method may be used. The safety and efcacy of reimplantation during CPRE has been well established [35].
The closure of the vesico-urethral unit begins at the bladder neck. There is no formal bladder neck reconstruction, but an attempt to funnel the bladder neck area is made to increase the outlet resistance without causing obstruction. This is done by marking an area of approximately 18 to 20mm width depending on the age and clinical appearance, denuding lateral triangular strips of mucosa for creating a 10mm length of funnelled wall. Three or four sutures with 4–0 or 5–0 PDS are taken, to be tied later after the bladder closure.
The bladder closure is started after bringing out and securing the ureteric cathe­ters, suprapubic tube and a per-urethral stent. The closure is done in single or double layer with 3-0 PDS, with simple or mattress sutures, as per the quality of the bladder and surgeon’s preference. The urethroplasty is done preferably with interrupted sutures, leaving the glansplasty for after pubic bone approximation.
As discussed earlier, osteotomy is imperative for a successful redo closure. An adequate osteotomy will allow the surgeon to place the bladder and posterior ure­thra in the pelvis while the hip bones are internally rotated to bring the pubes in close approximation to bridge the intersymphyseal gap. Depending on the severity of the diastasis, expertise of the orthopaedic surgeon and whether a previous oste­otomy has been done, the osteotomy best suited for the patient can be performed. Sometimes a combination of transverse innominate and vertical iliac osteotomy may be necessary, if the diastasis cannot be overcome by a single osteotomy. The authors have found the modied Salter’s osteotomy to be adequate in most cases. Usually, a single strong 1-0 PDS suture securely knotted on the outside is sufcient to keep the approximation in place. Sometimes an additional suture may be neces­sary. Such a snug approximation can sometimes cause vascular compression and create a penile compartment syndrome. Active bleeding from the glans and any glans congestion should be checked for before being satised with the symphyseal
290
Fig. 17.6 Leg traction and external xators for secure immobilisation
R. S. Joshi and J. Ramji
closure. It may be necessary to release the suture and re-approximate with a small gap between the pubic bones to ensure that there is no vascular compromise.
The glansplasty is now completed; the urethra is shifted ventrally, and the cor­pora are approximated dorsally in external rotation with 4-5 sutures of 5-0 PDS, as demonstrated by Pippe Salle [36]. Depending on the availability and quality of skin, the penile skin cover can be done by different techniques: a ventral rotational skin ap described by Pippe Salle, dorsal transposition of a ventral ap (reverse Salle technique), a transverse island ap or reverse Byar’s aps. Except for the reverse Byar’s, all the others aim at avoiding a dorsal suture line, which improves the cos­mesis [37, 38].
The abdominal wall closure can now be done in a tension-free manner. In rare instances, lateral relaxing incisions can be given, to decrease undue tension. It is vital to ensure that the tubes are all securely xed before the patient is shifted. Our preferred method of immobilisation for stabilising the osteotomy is the use of exter­nal xators for 4weeks. The addition of leg traction like modied Bryant’s may provide extra security (Fig.17.6).
Post-operative care focuses primarily on immobilisation, adequate pain relief with epidural analgesia, wide spectrum antibiotics and antispasmodics to prevent bladder spasms, along with the prevention of any other causes of raised abdominal pressure such as distension and constipation. Ureteric stents are left in for 2weeks and suprapubic drainage is maintained for 6weeks (Fig.17.7).
bc
17 The Failed Exstrophy Repair: Technique andImplications
a
291
Fig. 17.7 Redo repair in failed exstrophy. (a) Failed repair with complete dehiscence. (b) Redo CPRE with osteotomies. (c) Outcome on 6-month follow-up
Use ofRectus Muscle Flaps
The bladder neck region and intersymphyseal area are the most vulnerable areas for dehiscence or stula formation. The use of rectus muscle pedicled aps has been described to reinforce the closure in the inter-pubic gap in redo bladder closures and repair of vesicocutaneous stulae; as well as in bladder neck closures and bladder augmentation in failed repairs [13, 39, 40] (Fig.17.8).
We have similarly used the rectus muscle ap in some redo repairs for reinforc­ing the closure, and sometimes also as an alternative to osteotomy in older patients. The rectus muscle is separated off the anterior and posterior rectus sheaths, taking care not to damage the anterior sheath. The muscle is divided at the upper end of the incision and mobilised till its attachment to the pubic bone and preserving its vascu­lar supply from the inferior epigastric vessels. The muscle ap can now be turned