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21 Surgical Options forRefractory Urinary Incontinence inClassic Bladder Exstrophy
343
the patients, 15 already had undergone AC, 12 had undergone a Mitrofanoff proce­dure and 12 had been treated with BNI in an attempt to achieve continence. Four patients had undergone more than 1 bladder neck procedure. Postoperatively 28 patients were using CIC to empty the bladder (5 per urethra, 23 via Mitrofanoff). Although redo BNR can render a signicant number of patients dry, it was only effective if performed in conjunction with AC.Ashraf Hafez of Mansoura, Egypt, described his experience with the Mitchell BNR and detrusor wraparound aps in 28 children with CBE [68]. His manuscript was described as mini-trigonal tubulariza­tion and bladder neck wraparound with a demucosalized detrusor ap. With a mean follow-up of 2.5years, all 13 patients with augment on CIC via reconstructed outlets were continent which was signicantly higher than the non-augmented cohort. Complete dryness was achieved in 20 (71%) of 28 patients.
The use of non-detrusor muscular aps as an additional substrate for coaptation/ compression of the bladder outlet with or without BNR has been described. The pyramidalis, rectus abdominis, and gracilis muscles have been harvested as wrap­around aps of reconstructed bladder outlet to augment resistance and improve con­tinence [6971].

Bladder Neck Closure

Persistent incontinence after BNR as described can be challenging to achieve uri­nary continence. Repeat BNR may denervate the posterior urethra and distal tri­gone, and ultimately impair any potential for spontaneous voiding. In patients who have undergone multiple surgical procedures involving the bladder neck with intractable urinary incontinence, the muscle quality may be questionable or exten­sive scarring may exist. This clinical scenario does not lend itself to reconstructive efforts. BNC or transection may be the singular way to achieve urethral continence albeit at the expense of alternative bladder drainage in the form of CIC or urostomy. Patient selection is paramount as with any lower urinary tract reconstruction but may be magnied as BNC is most frequently paired with the creation of a CCC using the Mitrofanoff principle. Compliance with CIC is critical to avoidance of bladder perforation and upper urinary tract preservation.
Standard BNC is accomplished by approximating the anteroposterior bladder neck edges in two layers. The distal urethra may or may not be closed, and omentum or a rectus muscle ap is usually interposed between the closed bladder neck and urethra [7274]. Despite these measures, BNC is often associated with such compli­cations as leakage or stula. Khoury etal. described a modication of the standard technique of BNC that involves extensive mobilization of the posterior edge of the bladder neck, elevating it at least 2cm. off of the vagina or rectum in female and male patients, respectively [72]. This free edge of the posterior bladder neck is rolled anteriorly and, hence, the anastomosis is placed anterior and not directly inferior. When concomitant enterocystoplasty is performed, the anterior suture line is begun in the midline by suturing the edge of the detubularized and recongured bowel plate to the anteriorly rolled edge of the bladder base.
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Many series of BNC have been reported with success rates of 40–100% [72,
7581]. Bladder perforation is the most feared complication because of inadequate
drainage and excessive pressures but does not seem to occur at higher rates than BNR.Nguyen and Baskin reported high calculus formation and stula rates (40%) in 20 patients (n=12 CBE patients) who underwent AC and BNC, largely attribut­able to poor compliance with CIC [78]. They also found that incomplete division of the bladder neck portended stula formation. At 3months after surgery, 40% of the patients were completely dry, 20% had leakage via the stoma and 40% had a ure­thral stula. After additional surgeries, 85% of the patients were completely conti­nent 2years following BNC (15% declined further surgeries or had an ileal conduit). Of the 15 patients with follow-up longer than 3years, only 40% remained com­pletely dry, and leakage via the stoma developed in 47% after being dry for 1year. Repeat urodynamic studies did not show any adverse changes in bladder dynamics in the incontinent patients. Stomal stenosis of the CCC occurred in 30% of the patients and bladder stones developed in 40%. Novak etal. described 76 patients (n=42 CBE patients) who underwent AC with BNC [79]. After a mean follow-up of 44months, continence was achieved in 98% of patients and there were no reports of bladder perforation. Complications included two bladder neck stulas and ve patients with stomal leakage. Landau et al. described 12 patients (n = 5 CBE patients), who underwent BNC with a 10-year follow-up [80]. Continence was achieved in all patients, and upper tract dilation was noted in the only patient who did not concurrently receive AC.There were no reports of bladder perforation.

Continent Catheterizable Channel: Mitrofanoff Principle

CIC through the reconstructed urethra in the CBE patients either with or without BNR may not allow for easy, reliable performance of it. Therefore, an alternative conduit for CIC can be quite advantageous, and in 1980, Paul Mitrofanoff published his clinical series utilizing the appendix as the conduit for a CCC for CIC [81]. The Mitrofanoff principle is based on the implantation of a supple tube within a submu­cosal tunnel with seromuscular support. The urinary reservoir pressure will rise during lling and coapts the lumen of the CCC to prevent retrograde leakage through the CCC and provide continence. The seromuscular layers of detrusor in a bladder or gastrointestinal reservoir, when the bladder is absent or insufcient, can be uti­lized for the site of implantation of the conduit.
Although this Mitrofanoff principle has been applied frequently with the appen­dix, other substitutes have included transversely tubularized bowel segments (Yang­Monti) or ureteral remnants. Careful patient selection and ensuring a low-pressure urinary reservoir are critical elements to achieving the successful outcome of the CCC with a high rate of channel continence. Failure to regularly catheterize and completely empty the bladder or reservoir can lead to urinary tract infection, hydro­nephrosis, or reservoir perforation.
When the appendix is utilized for the CCC, assessment of the appendiceal length and its mesenteric vascular anatomy is important. The appendix can be removed
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with a cuff of cecum to provide length and a wider stoma to help minimize stenosis at the skin level. The mesoappendix should be mobilized until the desired stomal location can be reached without tension. As with ureteral reimplantation, creation of the antireux mechanism can be accomplished in an intravesical or extravesical fashion. For intravesical technique, the bladder can be opened in the sagittal plane to allow for concomitant reconstructive procedures such as bladder outlet procedure and/or AC as indicated. The distal end of the appendix can be tunneled into the blad­der through neohiatus with a minimal submucosal tunnel length of 3cm [81, 82]. Securement of the appendix to the outer bladder wall at the entry into the neohiatus into the submucosal tunnel with 3-0 absorbable suture assists to minimize channel retraction. Bladder immobilization using abdominal wall hitch stitch minimizes catheterization angulation and false passages. For the extravesical technique, the bladder is distended via the indwelling catheter and a 3cm incision is made in the detrusor and seromuscular aps are created on the anterior-lateral bladder wall [83]. The bladder mucosa is not entered during this extravesical dissection. At the distal aspect of the trough, a mucosotomy is made and the bladder mucosa to appendiceal mucosa anastomosis is performed circumferentially using absorbable 4–0 suture. The seromuscular aps of the trough are brought anterior to the channel and sutured to each other. Incorporating the serosa of the appendix with the suture used for sero­muscular ap closure sutures helps maintain the length of the antireux tunnel as well.
Implantation of the CCC into the detrusor muscle is the desired method however in the CBE patients this may present a unique challenge if the native bladder is too small or otherwise unsuitable for CCC tunneling. In these cases, a “serosal trough” strategy can be used to implant the CCC into a bowel segment used for augmenta­tion [84, 85]. Two parallel incisions through the augmented bowel serosa are created to expose the muscularis mucosa and submucosa. A full-thickness insertion orice for the channel is made at the distal end of these incisions. The channel is laid into the tunneled serosal “trough” and implanted into the augmented bladder orice. The serosal aps of the parallel incisions are used to overlay the tunneled channel segment.
It is important regardless of the implantation technique used, the Mitrofanoff channel is intra-operatively tested on multiple occasions, and each time any opera­tive step is completed, for ease of catheterizability and stomal continence while the bladder is lled. This will permit the surgeon to identify which step introduced any change or difculty and adjust accordingly. Depending on stomal location, guide the appendiceal base to the umbilicus or through the rectus muscle after creating a cruciate incision in the rectus fascia. Mature the stoma at the skin level by spatulat­ing the proximal end of the appendix and securing a wide-based V- or U-shaped skin ap at the apex of the spatulation. Key technical points are to maintain the shortest channel possible to facilitate ease of catheterization; avoid kinking; and if possible, xation of the bladder to the undersurface of the abdominal wall.
When the appendix is absent, insufcient in its length, or other factors preclude its use for Mitrofanoff, an alternative substrate is needed for Mitrofanoff. Colon or ileum is most frequently used and the latter is favored due to the more robust blood
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supply of the small bowel relative to the large bowel. After assessment of mesen­teric vascular anatomy, a 2–3cm segment of ileum is isolated from gastrointestinal continuity on a vascularized pedicle. This segment is opened transversely along the antimesenteric border according to the technique described by Monti [86]. Tubularization of the recongured intestinal segment in a longitudinal fashion over a 12- to 14-Fr catheter is performed in two layers using a 5-0 absorbable monola­ment suture. Implantation of the recongured channel into the bladder and stomal maturation occurs using the aforementioned surgical techniques as with the appen­dix. Another alternative technique for creation of a CCC is the Macedo technique which mandates concomitant AC due to the technique [87]. The CCC is constructed from the same bowel segment that AC occurs. From 35cm of ileum, a 3cm width ap from the anterior and posterior wall of ileum in the mid part of the isolated seg­ment. The continence mechanism of the CCC is based on angulation and a serous­lined tunnel created with three to four nonabsorbable seromuscular sutures.
CCC has been utilized frequently in the CBE population due to the aforemen­tioned challenges of CIC through the reconstructed bladder outlet or following BNC.Kasprenski etal. from the Hopkins team described the simultaneous creation of a CCC at the time of BNR in a series of 24 EEC (n=18 CBE) patients who were felt not to be candidates of BNR by the authors due to their institution’s eligibility criteria [53]. The median bladder capacity of these 18 CBE patients was 220mL and 2 had prior failed bladder closure. For patients with greater than 6months of follow-up time (n=17), 12 (71%) had dry intervals greater than 3h. Of the 10 patients who were completely dry, 7 (58%) achieved volitional voiding per urethra with residual urine emptied through their CS.The remaining 5 (42%) were conti­nent by stomal catheterization alone and did not achieve voiding per urethra. Of the 5 patients, 29% did not achieve continence following a BNR-CCC procedure. Three of the 5 patients (60%) established eventual continence following BNC.In the Maruf series of 350 patients with EEC where continence outcomes were evaluated, a CCC was used in 157 patients as part of BNC and continent diversion with 60 additional patients as part of BNR with (n=30) or without (n=30) AC [3]. This series reported that in those patients who underwent BNR with AC with CCC,
61.3% were continent, with all of these patients performing CIC alone without void­ing. Of those who underwent BNC and continent diversion, all 133 patients were on CIC via CCC.Ulman etal. reported their experience with six CBE patients who had undergone YDL-BNR with an adjuvant CCC [88]. Four patients had a breakdown of their reconstructed bladder necks due to CIC via urethra. AC was performed in all six patients at the same setting as the creation of the CCC.Szymanski etal. as part of the multicenter study of ve North American pediatric centers from 1980 to 2016 examined the use of CIC in 216 CBE patients [8]. At the last follow-up, 67.4% of all patients performed CIC.Among 95 patients with an intact native bladder,
30.5% performed CIC (72.4% via CCC). Among those without BNR or with an AUS, 14.8% performed CIC, compared to 54.8% after BNR and 100% after BNC.Among 107 patients who underwent AC, all performed CIC, typically via CCC (86.9%). Older patients on CIC were more likely to use a CCC, increasing from 61.5% for those younger than 10years to 88.3% for patients >18years. Of 76
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adults without a diversion, 85.5% performed CIC.The CIC rate was 100% in adults who underwent AC and 31.3% in adults with a native bladder. These clinical series emphasize the use of CIC via CCC as a frequently utilized intervention in the CBE population.
Complications from CCC arise with short-term and extended follow-up. Stomal stenosis at the skin level is the most commonly reported problem with a reported incidence between 10% and 40% [8992]. False passages can also occur from repeated erroneous catheterizations or excessive angulation within the channel and can usually be managed with a temporary indwelling catheter [93]. Stomal inconti- nence is infrequent and may be due to a short intravesical tunnel, persistently ele­vated reservoir pressures, or a stula between the channel and the bladder. Lowering elevated detrusor pressures either medically or surgically can resolve the stomal incontinence when secondary to abnormal storage pressures. For inadequate conti­nence mechanism due to shortened antireux tunnel, endoscopic management with Deux has been reported [94]. A formal subfascial revision with redo implantation to address the inadequate antireux mechanism of the channel is another surgical intervention performed as well for stomal incontinence.

Augmentation Cystoplasty

The chapter thus far has focused on surgical techniques to address incompetent bladder outlets and alternative bladder emptying conduits in the form of CCC. However, the urinary incontinence in CBE patients may be secondary to impaired bladder capacity with or without abnormal storage characteristics result­ing in an insufcient bladder reservoir. Use of pharmacotherapies (i.e. anticholiner­gics and/or beta-3 agonists) should be used prior to surgical procedures to improve bladder storage characteristics. When medical management is ineffective, AC is a highly effective procedure to simultaneously decrease elevated detrusor end-lling pressures, improve bladder compliance, and increase bladder capacity.
The use of which gastrointestinal segment is utilized for AC varies by patient anatomy and surgeon preference. Gastric, ileal, and colonic segments have all been used in CBE patients [8, 95, 96]. Gastric segments are less utilized for AC compared to other segments due to the typical normal small and large intestine anatomy of CBE patients. Advantages of sigmoid colon for AC are its anatomic proximity to the bladder and the thick muscular taenia of the colon may serve as a more robust sub­strate for ureteral or CCC implantation than the thinner seromuscular layer of ileum. This author preferentially uses ileum for AC because of its mesenteric mobility, relative abundance compared to sigmoid colon, and tendency to generate less mucous than the colon. Detubularization and reconguration of the intestinal seg­ment used for AC into a U- or W-shape provide multiple advantages, including maximization of the volume achieved for any given surface area, blunting of bowel contractions, and improvement of overall capacity and compliance. This augment patch is then sutured to the bladder after making a sagittal incision to provide wide enterovesical anastomosis.
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AC has been performed in varying rates of CBE published series. Szymanski etal. in a multicenter study over three decades retrospectively reviewed 212 CBE patients who underwent primary bladder closure where 108 (50.9%) underwent augmentation, 10 (4.7%) diversion (1 with a previous augmentation), and 95 (44.8%) neither [8]. After controlling for follow-up time in this series using survival analysis, the probability of AC was 14.9% by age 5years, 50.7% by 10years, and
70.1% by 18 years. After controlling for follow-up time, this probability varied signicantly by treatment center; the probability at 10years ranged between 26.7% and 64.5%, depending on the center. Among 87 children who underwent a bladder outlet procedure without simultaneous AC for persistent urinary incontinence after primary closure, the probability of future AC was 46.4% at 5years and 60.7% at 10years after the bladder outlet procedure and did not differ among centers. Maruf etal. published that out of 432 CBE cohorts treated at Johns Hopkins, 76 and 165 patients underwent AC with BNR or BNC, respectively (56%, 241/432) [3]. Cervellione etal. reviewed 32 CBE patients who underwent bladder closure using a staged repair technique that underwent a salvage procedure for continence [97]. AC was performed in 23/32 (72%) and the remaining 9 patients had either neobladder or urinary diversion procedures.
The complete primary repair technique for CBE has been touted to normalize anatomy at the initial repair, thereby allowing early bladder lling and cycling which may inuence bladder function, capacity, and possibly the need for AC.In a multicenter study of CBE patients, Weiss etal. showed 54 CBE patients who under­went bladder closure using a complete primary repair technique reviewed at three centers prior to the creation of a clinical consortium between the centers over a 20-year period (1993–2013) [7]. The median age of patients at the last follow-up was 12years, and they reported AC was performed in 10/54 (19%) with BNR or BNC.Ellison etal. and DiCarlo etal. reported their own single-institution experi­ence with continence outcomes and procedures following complete primary repair technique in 29 and 61 CBE patients, respectively [1, 51]. Ellison etal. reported that 6/29 (20%) underwent AC which was very similar to 12/61 (19%) in the DiCarlo series undergoing AC.No study has been published to date to discern if the CPRE proves to carry a lower risk for AC.
Surgical technique is critical when performing AC to minimize complications. Adequate length of the intestine (20cm of either the large or small intestine) uti­lized will minimize the risk of re-augmentation [98]. Benz etal. reviewed a total of 166 patients with CBE who underwent AC and 17 (10%) patients underwent a re­augmentation. The length of the bowel used for AC was predictive of the need for re-augmentation. In the re-augmentation cohort, the mean amount of bowel used during the rst AC procedure was 12cm compared to the mean amount of bowel used for non re-augmentations which was 20.8cm. There is also a need to widely open the bladder to minimize the risk of postoperative hourglass deformity contrib­uting to poor drainage and persistent abnormal bladder storage characteristics. Bladder perforation is once again the most feared complication of AC and in large series has been reported to occur 5–10% in large clinical series of patients with CBE and other diagnoses [99101]. Detubularization and retubularization are useful for
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minimizing the risk of bladder perforation [102]. The most commonly reported need for additional surgery after AC is the presence of bladder calculi and occurs in 15–30% of patients [102105] There is a high recurrence rate observed in patients with AC that initially developed a stone-acquiring second bladder calculi within a short period of time of their rst occurrence. Recurrence of the bladder stone in the AC patient was independent of the method of removal and found to be similar for both open or endoscopic extraction [106, 107].

Continent Urinary Diversion

In the rst half of the twentieth century, standard care of the CBE patients was not predicated on bladder preservation as closure and functional use of the exstrophic bladder was met with poor success rates [108, 109]. Urinary diversion, with cystec­tomy, was near universal and this frequently involved urorectal anastomosis for CBE patients as rst described by Simon in 1852 [110]. Coffey in 1930 rened the ureteral anastomosis technique to reduce the risk of reux and ureterosigmoidos­tomy was pursued as a standard of care for CBE patients [111]. The ureterosigmoid­ostomy was accompanied by a high incidence of metabolic acidosis and intestinal dysfunction. This was noted in patients who underwent ureterosigmoidostomy with extended follow-up and improved survival, an increased risk of colorectal carci­noma which dampened the enthusiasm for this technique [112115]. At the same time as the initial reports describing neoplastic complications following ureterosig­moidostomy became more published, Eugene Bricker popularized the ileal conduit as an incontinent form of urinary diversion—the so-called “Bricker Bladder” [116]
To overcome the disadvantages of the classical ureterosigmoidostomy and reduce the number of postoperative upper urinary tract complications as well as to improve the continence rates, Fisch and Hohenfellner introduced the rectum-sigma pouch (Mainz Pouch II), which transformed the high-pressure segment of the recto­sigmoid into a low-pressure reservoir by detubularization and reconguration [117]. Fixation of the rectosigmoid junction at the promontory as well as parallel ureteral implantations guarantee a straight ureteral path and prevent kinking and subsequent obstruction. The antimesenteric opening of the bowel creates a low-pressure reser­voir, which is expected to be essential for good day and night continence and con­fers advantages for the upper urinary tract. The group from Mainz described their technique for minimizing the upper tract complications with antireux ureteral reimplantation whether the ureter was normal caliber or dilated [118]. D’elia etal. utilized the Mainz II pouch in 123 patients over 10years (n=26 with EEC) with reported continence rates day and night of 97% and 95%, respectively [119]. 70% of the patients were on alkalinizing oral medications to address subclinical acidosis seen on blood gas to prevent the development of clinical hyperchloremic acidosis. Mingin etal. [17] described ve patients with bladder exstrophy who underwent the Mainz pouch II procedure between 1996 and 1998 to create a rectosigmoid pouch, allowing urine to drain into and be eliminated via the rectum [120]. Three patients required oral sodium bicarbonate to correct metabolic acidosis, but upper tracts
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remained nondilated, all patients were continent during daytime and nighttime, and there were no episodes of pyelonephritis during follow-up, which was between about 1 and 3years.
Despite the surgical and technical advances for the modern staged reconstruction of the bladder involving the preservation of the bladder early in life, there is a subset of CBE patients where the bladder is not deemed a suitable substrate for reconstruc­tion utilizing many of the lower urinary tract reconstruction previously discussed in this chapter such as BNR, CCC, and AC.Cystectomy of the exstrophic bladder is rarely performed in contemporary practice where the modern staged repair is the preferred surgical philosophy. Ko etal. described the Johns Hopkins experience in 18 patients of whom (n=15 with CBE), six were performed primarily with diver­sion and twelve were performed secondarily. Of the patients with primary cystec­tomy this occurred at age ranges of 1–9years due to what authors state were “small, brotic, noncompliant, polyp-covered bladders” of poor quality unsuitable for immediate closure [121]. Bladder template size and tissue quality were followed in these patients before determining that they were “unsuitable for closure.” [121] The authors concluded from this series and their extensive experience that cystectomy should be considered in select CBE patients where the bladder template is of inad­equate size or of too poor quality to permit primary closure, patients with low blad­der capacity after prior closure either due to inadequate growth or loss of capacity, particularly at the time when a continence procedure is being considered.
When rectal diversion is not pursued an incontinent conduit with ileum or colon into abdominal wall stomal appliance has been employed. The relative simplicity of the procedure compared to continent reconstructive techniques has been offset by the long-term complications from a urostomy stoma (retraction/stenosis) as well as ureterointestinal anastomotic complications. Deterioration of the upper urinary tract and renal function, calculi formation, and stomal stenosis are observed as late com­plications at rates of 50–86% with ileal conduits in pediatric patients [122, 123]. With ileum, there is typically a reuxing ureteral anastomosis while colon affords the opportunity of non-reuxing ureterocolic anastomosis. The group from Mainz reported on 105 pediatric patients (n= 16 CBE) with colonic conduit with non­reuxing ureteral anastomosis with a mean follow-up of 16years in 84 patients [124]. Early and late stenosis at the ureterocolic anastomosis and stomal stenosis occurred in 7.6 and 15.5% of the cases, respectively. Eight kidneys without function after recurrent pyelonephritis, calculi, or stenosis at the ureterocolic anastomosis were removed during follow-up. A total of 31 patients had complications during follow-up and 18 underwent conversion to continent urinary diversion.
When the use of closed continent urinary diversion is preferred or selected over a conduit, and the bladder deemed unusable, there are various continent urinary reservoirs (CURs) that have been described in the literature [82, 87, 125128]. As with AC, the creation of CUR is predicated on detubularized and recongured bowel segment with typical nonreuxing ureteroenteric anastomosis. The CUR relies upon a continent catheterizable efferent limb created from a variety of sub­strates such as in situ appendix, tapered ileum, or recongured bowel. The appen­diceal lumen can inuence the maximal diameter of the catheter used for drainage.
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Wiesner etal. retrospectively compared the intussuscepted ileal nipple and in situ, submucosally embedded appendix as continence mechanisms in 400 patients who underwent cutaneous ileocecal pouch (Mainz pouch I) [129]. Continence mecha­nisms were nearly equal between the two techniques. 36% of the entire cohort required intervention for a stomal complication with a higher rate of stomal steno­sis seen (32% v 17%) in an in situ, submucosally embedded appendix compared to intussuscepted ileal nipple, most likely due to the smaller diameter of the appen­dix. However, stone complication was seen in 20% of the ileal nipple group com­pared to 10% in the appendiceal group likely related to the use of metal staples to create the intussuscepted ileal nipple. Continence rates of the two outlets were very good (82% in ileal and 93% for appendix). The complications associated with CUR increase with increased follow-up and are not dissimilar to that of AC; such as reservoir calculi, reservoir perforation, metabolic disturbances, and gastrointestinal [130].
As long-term follow-up of reconstructive procedures in CBE patients such as conduits, AC, or CUR have revealed a considerable complication rate; interest in ureterosigmoidostomy as a simple and appliance-free technique of continent uri­nary diversion has rekindled. Whether bladder reconstruction techniques per­formed for continence that preserves the exstrophy bladder, which frequently are dependent on CIC, are superior to the rectal urinary diversion which can be emp­tied voluntarily via the natural rectal sphincter and is performed at a single oper­ation, will continue to be the subject of much controversy and discussion over time.

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