An important consideration is that in girls with CPC, within the overall pattern of associated urinary tract anomalies, there is a fairly wide spectrum of anatomy of the LUT i.e. the urethra, bladder neck, UB capacity and urinary continence [3, 4]. An earlier study reported that in patients without UI, the bladder neck appeared competent on CUS, although the urethra was usually short and wide, suggesting that bladder neck integrity, even with a short urethra, was the main anatomic factor responsible for urinary continence [3].
Accurate volumetric assessment of UB capacity is difficult in these patients because of the open bladder neck. Radiologic studies and CUS are important to study LUT anatomy and subjectively assess UB capacity. Detailed history-taking is essential as the incidence of UI may be underestimated in infants and young girls. The parents of some infants stated that the girl was passing urine normally in a stream but further questioning revealed that on crying and/ or while standing, the child leaked urine [4]. An earlier study reported that only 1/ 10 girls with CPC < 1 year age were assessed to have UI while 9/ 12 (75%) older girls had UI [3]. In the present study, in 3 of the 8 patients, PUI/ CUI became apparent only at an older age.
Both PUI and CUI are major disabilities as the girls are almost always wet, need diapers and cannot participate in normal activities, resulting in significant stress and psychological trauma. Surgical techniques for managing BNI include BNR procedures [8–10] as well as other surgical interventions [14]. Apart from an earlier study from our center [4], the only other report of management of UI in a girl with CPC is of a child with Type I CPC and CUI thought to be due to bladder hypoplasia [6]. UI was managed by ureteric reimplantation into an ileal conduit [6].
There are several anatomic reasons why BNR procedures may not be uniformly successful in treating UI in these patients. There is a short, wide urethra with frequently a widely open bladder neck which often has a posterior V- shaped defect. This makes the procedure technically difficult and in two patients, the V- shaped defect was long needing midline closure before constructing the neo-urethra. The UB is also thin-walled and the neo-urethral tube may not provide adequate muscular resistance. The trigone is small with low- placed ureteric openings close to the bladder outlet [4] and constructing an adequately long urethral tube can be difficult. It may be necessary to reimplant the ureters at a more cranial level which is hazardous as the ureters are usually of normal caliber. In addition, UB capacity is subnormal for age. In 2 patients with a 'fair' result after BNR [11], AC was considered to increase UB capacity and the dry interval but was refused by the patients. Additionally, a high and inaccessible urethral opening as in several girls may make CIC by this route difficult. However, as there is a spectrum of severity of UI, if BNI is not severe and UB capacity is not markedly reduced, BNR may be beneficial [11], especially if combined with AC and a catheterizable stoma. Overall, however, partial benefit with a diurnal dry interval of up to 3 hours and a high incidence of nocturnal and stress incontinence may not be a satisfactory long-term result.
An earlier report had suggested that if procedures to provide effective bladder outlet resistance with or without AC were unsatisfactory, an effective option may be BNC and AC with a catheterizable stoma [4]. The segment of bowel available for AC in the more common Types I- II CPC is limited by the normal proximal colon being either absent (Type I CPC) or very short (Type II CPC) [2, 15], virtually ruling out colocystoplasty. Thus, it may become necessary to use the stomach or small bowel for augmentation [4] although, especially in Type I/ II CPC, using a substantial length of ileum for AC does aggravate the risk of short- bowel syndrome. One useful feature in Types I- III CPC is that, as described earlier in 4 girls with CPC and UI, during tubularization of the colonic pouch, a segment of the pouch can be preserved with its leash of vessels [4]. This pouch segment was laid open and successfully used for AC in one patient reported here. Duci et al also described the clinical details of five cases treated for CPC in two Italian centers who were retrospectively reviewed assessing the fate of the terminal dilated colon [16]. The authors emphasized on the double vascular arcade which allows pouch splitting and lengthening that enabled increasing UB capacity in one case and reconstruction of the vagina in 2 patients. However, the authors did not specify the reasons and the details of the case in which a colonic pouch segment was used for AC [16]. Wester et al also reported two girls with CPC in whom vaginal reconstruction was performed by longitudinal splitting of the colonic pouch, using the redundant patch of pouch colon for the purpose [17]. Kurian et al reported a case of CPC where the colonic pouch was used to augment a high-pressure neurogenic bladder [18].
There are several reports describing BNC with/ without AC as a successful means of achieving urinary continence in patients with a variety of conditions in whom other bladder outlet surgery have failed [19–26]. Bergman et al evaluated 52 patients who underwent BNC for neurogenic UI [19]. Mean follow-up was 20 months and mean age 13.9 years. Forty-two patients (81%) underwent concomitant AC. Catheterizable stomas included 46 appendicovesicostomies (88%) and six Monti tubes (12%) [19]. Complete continence was achieved in 44 patients (88%) after one procedure. Out of six incontinent patients (12%), one had a vesicourethral fistula and five had incontinence at the stoma. Twelve of 50 patients had stomal stenosis (24%), with six requiring urgent evaluation (12%) and six requiring surgical revision (12%) [19]. The authors concluded that BNC is safe and effective for achieving urinary continence in children with neurogenic voiding dysfunction [19]. Landau et al reported 12 children in whom BNC with construction of a catheterizable stoma was performed with/ without AC for bladder exstrophy, spinal dysraphism and other conditions [20]. Although 6 patients needed reoperation, ultimate success rate was 100%. Similarly, Nguyen and Baskin reported 12 children in whom BNC was performed for a variety of indications and found it to be effective for achieving urinary continence when other bladder outlet surgery had failed [21]. There was however a high incidence of complications and need for reoperation [21]. At 3 months after surgery 40% of the patients were completely dry, 20% had stomal leakage and 40% had a urethral fistula. After additional surgeries, 85% of the patients were completely continent 2 years following the BNC [21]. However, of the 15 patients with > 3 years follow-up, only 40% remained completely dry; stomal leakage developed in 47%, stomal stenosis in 30% of the patients, and bladder stones in 40% [21]. Kavanagh et al reported 28 consecutive patients who underwent BNC with enterocystoplasty and Mitrofanoff diversion for structural and neurogenic conditions [22]. Of these patients, 19 (68%) had undergone 20 unsuccessful bladder neck procedures before BNC. Median time from BNC was 69 months. BNC was initially successful in 27 of the 28 (96.4%) patients [22]. One patient required subsequent closure of a postoperative VVF. In 11 patients, 16 additional procedures included stomal injection of bulking agents (n = 2), stomal revision for stenosis (n = 2) or prolapse (n = 1), percutaneous lithotripsy (n = 1), open cystolithotomy (n = 2), lithotripsy for upper tract stones (n = 4), repair of augment rupture (n = 3) and retrograde ureteral stenting for stone (n = 1) [22]. There were no observed cases of progressive or de novo hydronephrosis. The authors concluded that BNC with enterocystoplasty and Mitrofanoff diversion is effective in achieving continence in complex cases as a primary or secondary therapy [22]. Hoebeke et al reported 17 children who underwent BNC with continent diversion for neurogenic UI (n = 10), exstrophy bladder (n = 5), and other conditions (n = 2) [24]. Previous surgery for UI had been performed in 12 patients with 36 procedures while primary BNC was done in 5 patients. For continent diversion, the appendix was used in 13, ureter in 2, a Monti procedure in 1, and an ileal valve in 1 patient. BNC was combined with ileal augmentation in 9 children while 4 children had been augmented earlier [24]. Mean age at surgery was 13.5 years. After BNC, all patients were completely dry. One girl had some stomal incontinence which disappeared after recent AC. Other stomal complications included difficulty in catheterization (n = 3), appendiceal polyps (n = 3), and stomal stenosis needing revision/ surgical correction (n = 2). All complications were seen in the first 6 months after BNC. The authors stated that considering the high success rate, low complication rate, and the very high patient satisfaction, BNC should be regarded as an important procedure and if reconstruction fails, BNC should be considered as it gives the highest continence rate [24]. Hernandez-Martin et al analyzed the records of 20 patients with the vesical exstrophy complex who underwent BNC after several failed procedures to improve UI [25]. Median age at BNC was 11.5 years. Overall, 17 patients had concomitant AC. The catheterizable stoma was made with appendix (n = 14), bowel (n = 3), and ureter (n = 3) [25]. Median follow-up was 10 years. Urinary continence was achieved initially in 16 patients (80%); 4 had a bladder neck fistula, 3 of them underwent surgical revision and achieved dryness. Long-term complications were: bladder stones (n = 8), stomal problems (stenosis − 4; leaks- 3), bladder perforation (n = 2), and orchitis (n = 1) [25]. The authors stated that BNC is an effective approach to UI when other procedures have failed. In the long-term, the most frequent complications are those related with catheterizable stoma and stones [25].
Our study also shows that surgical BNC with AC and continent diversion is a satisfactory option to treat refractory UI in girls with CPC. However, preoperative counseling is essential to ensure that the patient or care-giver will reliably catheterize the stoma as the surgery leaves the urinary reservoir with no pop-off mechanism [14]. Early and late complications after BNC procedures appear to be related in part to compliance with CIC [21] and a regular postoperative CIC and reservoir irrigation regimen is necessary. The advantages include a dry status and preservation of the native UB without any surgery on the ureters. This procedure is however difficult as the multiple earlier surgeries make abdominal access and dissection lengthy and tedious. It is difficult to close the widely open UB neck, especially with its often deficient posterior rim. In 3 of the 4 cases reported here, the incision in the anterior bladder wall was carried down just lateral to the midline on each side, laying open the bladder neck and short urethra completely. This extremely helpful maneuver is possible without pubic symphysiotomy as the more cranial urethral opening, the short urethra, and the widely open bladder neck with its posterior defect greatly aid exposure and dissection. Another limitation in Types I- III CPC is that it may not be possible to use the appendix as a catheterizable channel as it is often absent/short and stubby or else duplicated [1, 2, 15]. The anatomy of the pouch colon and its earlier surgical management may also preclude the use of the appendix for this purpose. A Monti procedure [13] is then necessary, further increasing the technical difficulties, and risks of complications.
During BNC, it is essential that the posterior lip of the bladder neck and trigone is mobilized sufficiently to be rolled anteriorly and the bladder closed in at least two layers. This is especially important as the distal remnant of the colonic pouch is closely apposed to the trigone, UB neck and urethra and the two hemivaginas making dissection of the UB neck and urethra from these structures difficult. Iatrogenic tears in the vagina(s) are common and their repair leaves a vaginal suture line. As an interposition layer after BNC, omentum and a RA muscle flap have been described [14, 27]. Omentum may not be easily accessible or available in girls with CPC because of the shortened colon, the anatomy of the colonic pouch, and the previous surgeries. After the experience of the first case where a 6 mm VVF developed postoperatively, we have subsequently used a RA muscle and fascia flap based on the inferior epigastric artery [27] as an interposition between the UB and vaginal suture lines. An important technical point is that the native bladder should be opened widely posteriorly to prevent a narrow-mouthed anastomosis which can result in the augmentation segment behaving as a diverticulum predisposing to stone formation [14], a complication that occurred twice in one of our patients. Our follow up period is relatively short but 3 of the 4 patients required reoperation. Difficulty in stomal catheterization resolved over time and Hoebeke et al [24] also reported that only 2/ 8 stomal complications needed revision under anesthesia. However, long-term urologic follow-up is essential as late occurrence of stomal complications and stone formation is not infrequent [21, 22].
In conclusion, although BNC and AC with a continent stoma is technically demanding with several early and late complications, it is overall a satisfactory option to achieve continence in girls with CPC and UI, vastly improving their quality of life. Considering the high incidence of severe UI in girls with CPC, during primary surgery for the bowel anomaly, a segment of the colonic pouch should be preserved for possible use for AC during a subsequent BNR/ BNC procedure. In girls with favorable LUT anatomy, BNR with/ without AC may be considered as the initial procedure, especially if the urethral opening provides easy access for CIC. BNC should be the primary procedure for managing UI when in girls with unfavorable LUT anatomy and the procedure of choice after failed BNR.
Table 1
Details of patients undergoing surgery for UI (n = 8).
Case No. | CPC subtype; Age (y) at BNR and BNC | UI (CUI/ PUI) | Outpatient and EUA findings | CUS findings | Operative findings and Procedure | Outcome of BNR and/ or BNC |
1. | Type III CPC BNR- 21 y | CUI | 'Low' confluence of 4 perineal openings; wide urethral meatus; intervaginal VF. | Wide urethral opening; urethra < 1 cm long; BNI, small capacity UB; lateral, low-placed ureteric orifices. | Small capacity UB, ureteric orifices normal caliber, very laterally placed. BNR with 1.5 cm long urethral tube (over 10Fr catheter). Vagino-fistula septum divided each side. | Poor result |
2. | Type II CPC BNR- 19 y | CUI | 'High' confluence of 3 perineal openings; excoriations + ve. | Virtual absence UB neck; moderate capacity UB. | Severe BNI; moderate capacity UB; long, posterior V-shaped defect UB neck; laterally placed ureteric openings. BNR with 2 cm urethral tube (8 Fr) after closure V-shaped defect. Vaginal septum divided. | Poor result |
3. | Type II CPC, appendix absent. BNR- 12 y | PUI | 'Low' confluence of 3 openings: wide urethral meatus | Wide urethra, 2 cm long; partial BNI; UB capacity moderate; lateral, low-placed ureteric orifices. | Moderate capacity UB; ureters low down, laterally placed. BNR with 2.5 cm urethral tube (10 Fr). | Fair result: Diurnal dry period, 2–3 hours. Nocturnal UI + ve. |
4. | Type I CPC BNR- 11 y | PUI | 'Low' confluence of 4 openings; wide, patulous urethral meatus; Intervaginal VF; excoriations + ve. | Virtually no urethra; BNI; UB capacity moderate; ureteric orifices not clearly made out; trigone small. | Moderate capacity UB; ureteric openings very low and lateral, almost on anterolateral wall UB. BNR with 2.5 cm urethral tube (8 Fr) after bilateral trans-trigonal ureteric reimplantation more cranially. | Fair result: Diurnal dry period, 2 hours. Nocturnal continence 3–4 hours. |
5. | Type II CPC; short, stubby appendix. BNR- 8 years BNC- 14 years. | Earlier no UI detected; Later CUI | 'High' confluence of 4 openings; urethral opening wide; Intervaginal VF. | Urethral meatus wide; BNI; UB capacity small; trigone poorly developed; ureteric orifices normal. | Urethra short, wide; UB neck patulous, V-shaped defect posteriorly; UB capacity small, trigone poorly developed, ureteric orifices laterally placed. BNR with 2.8 cm urethral tube (10 Fr). BNC at 14 years: Ileocystoplasty, BNC, Monti procedure | BNR: Poor result BNC: Final result Satisfactory. |
6. | Type II CPC. BNR- 13 years BNC- 22 years | CUI | ‘High' confluence of 4 openings; Intervaginal VF; excoriations + ve. | UB neck completely open, very small UB; ureters opening very low-down, antero-laterally. Long posterior V- shaped cleft extending to 1.5 cm proximal to ureteric openings. | BNR with 3 cm urethral tube (10 Fr). Posterior V- shaped cleft closed before BNR. BNC: Ileocystoplasty, BNC, Mitrofanoff procedure. RA flap over suture-line. | BNR: Poor result BNC: Final result Satisfactory. |
7. | Type I CPC. Primary BNC- 13 years | Earlier no UI detected; later CUI | 'Low' confluence of 4 openings. Distal urethral fistula opening within wide urethral meatus; excoriations + ve. | Widely open UB neck, especially posteriorly; ureters opening low-down, laterally. | -Primary surgery: TC and pull-through, segment of pouch preserved with leash of vessels and stoma; vaginal septum divided. -BNC: Posterior V- shaped defect UB neck; moderate-sized UB; Pouch segment-cystoplasty, BNC, Monti's procedure. RA flap over suture-line. | BNC: Final result satisfactory. |
8. | Type II CPC, short, stubby appendix. Primary BNC- 11 years | Earlier no UI detected; later CUI | 'High' confluence of 3 openings. | Urethral opening ‘Very High’; urethra very short. UB-neck widely open; proximal urethral fistula just distal to UB neck. | -Primary surgery: TC and pull-through. -BNC: Posterior V- shaped defect UB neck; moderate-sized UB; ureters opening low down, laterally. Ileocystoplasty, BNC, Monti procedure. RA flap over suture-line. | BNC: Final result satisfactory. |
CPC = Congenital pouch colon; UI = urinary incontinence; CUI = complete UI; PUI = partial UI; EUA = examination under anesthesia; CUS = cystourethroscopy; BNR = bladder neck reconstruction; BNC = bladder neck closure; VF = vestibular fistula; BNI = bladder neck incompetence; UB = urinary bladder; TC = tubularizing colorraphy; RA = rectus abdominis
Table 2. Postoperative complications in girls undergoing BNC procedure (n= 4).
Complication
|
Number
|
Early complications
- Partial anastomotic dehiscence of the ileocystoplasty [Case 6]
-Prolonged drainage from pelvic drain [Case 7]
-Transient adhesive bowel obstruction [Case 8]
-Difficulty in CIC [Case 7]
|
1
1
1
1
|
Late complications
-Vesico-vaginal fistula (VVF) [Cases 5, 8]
-Calculus/ calculi in reservoir [Case 5 (with stomal leak), Case 6]
-Unilateral grade II VUR [Cases 5, 7]
|
2
2 (Twice in Case 5)
2
|