Hypothesis / aims of study
To describe the presentation and outcome of surgical management strategies applied to the adolescent and adult patient population (age >14 years) with exstrophy/epispadias who presented with persistent incontinence even after previous numerous surgeries at our institution. These patients present a difficult management problem [1-3]. When they are dealing with difficult issues like body image and sexual awareness, the added burden of ongoing incontinence causes major anxieties and lifestyle restrictions [1-3].
Study design, materials and methods
A retrospective review of the electronic medical records of patients with exstrophy-epispadias complex managed from January 1998 to December 2022 was undertaken. Patients aged >14 years at presentation, with detailed medical records were selected. Data on presenting symptoms, clinical findings, previous and present surgical intervention, post-operative complications, secondary procedures and follow-up periods were recorded.
Results
Thirty-six patients of bladder exstrophy-epispadias complex aged > 14 years presented to our institution over this 24-year period, out of which 32 had undergone previous intervention. A total of 30 (19 male and 11 female) patients who presented primarily with persistent incontinence into adolescence or adulthood following previous repairs were identified and their records reviewed. Median age of presentation was 21 years ( IQR,14-25.8 ) and they had undergone a median of 4 surgeries prior to presentation (range, 2-10). Three patients had already been augmented with bowel along with a Mitrofanoff channel. One patient was lost to follow up and two refused surgical intervention and 27 underwent a surgical continence procedure .
Of the 27 patients, 24 had already undergone a bladder neck reconstruction (BNR) previously for continence ( group A)and 3 were yet to undergo a BNR (group B)
In group A, 18 patients underwent a redo BNR of which augmentation cystoplasty was required in 16 with a Mitrofanoff channel added in 13 of them. In two patients an additional colposuspension and a tensor fascia lata sling and. rectus sheath sling were also performed respectively for better continence. An additional 2 female patients in group A who had already been augmented before underwent colposuspension only. Four patients in group A opted for urinary diversion, with bladder neck closure and continent cutaneous diversion being done in 2 and an incontinent diversion in the form a ileal conduit and a ureterostomy ( in view of elevated creatinine ) being done in the other 2 patients.
In group B, two patients underwent a BNR with Mitrofanoff with bowel augmentation required in one of them. The remaining one patient in group B opted for an incontinent diversion in the form of an ileal conduit.
The median follow-up was 42 ( (IQR,24-117) months. In the redo BNR group, two patients ( one male and female ) continued to have significant incontinence post redo BNR, the cause being a urethrovaginal fistula in the female which was successfully managed with fistula closure, however the other male patient needed a bladder neck closure (BNC) with a Mitrofanoff channel ultimately for continence. In addition, three patients had difficulty in doing CIC and needed additional procedures in the form of a Mitrofanoff channel, Mitrofanoff stomal revision and a meatoplasty respectively. Apart from the secondary continence procedures, additional secondary procedures in the form of caesarean section for pregnancy , stone procedure ( PCNL, URSL) and vaginoplasty, prolapse repair were needed in 5 patients .
One of the girls who underwent colposuspension only continued to leak postoperatively and since she was reluctant for a bladder neck closure, an additional autologous rectus sheath sling was placed which made her continent.
In the diversion group, one patient developed bilateral renal stone disease requiring bilateral percutaneous stone removal .
At last follow up, only one patient was volitionally voiding to completion, with the rest doing CIC through Mitrofanoff channel or by perurethral route. With respect to continence, after excluding 5 patients who underwent urinary diversion, 16/22 (72.7%) patients achieved full urinary continence with no need for pads. Two patients needed <3 pads per day and four patients needed >3 pads per day. All patients returned to normal activity with an improvement in the quality of life postoperatively.
Interpretation of results
The vast majority of Adolescent or adults with BEEC who present with persisting incontinence have undergone some form of bladder neck reconstruction as was evident in our study ( 24/27, 88.8%). Traditionally, failure of bladder neck reconstruction in patients with exstrophy epispadias complex is often considered as an indication for bladder neck closure. While some patients are open to this option, in our experience the majority of the adolescents and adult patients though weren’t averse to using a continent stoma, almost all of them were reluctant to consent to an irreversible option of a bladder neck closure and prefer to keep the per urethral passage open. Hence, it became imperative that we explore surgical options to achieve continence without a formal bladder neck closure. In this study we observed that a redo BNR along with adjunct procedures like colposuspension and autologous slings can have good continence outcomes with nearly 70 % of patients becoming free of pads.
We also observed that augmentation rates are quite high in our cohort ( 23/27,85%) as probably because of the long duration of incontinence these bladders haven’t developed and have a small capacity necessitating augmentation. Also, in most cases the available bladder wall is used up in bladder neck reconstruction and there is little bladder wall left for effective contraction, hence volitional voiding is not possible in most of these patients as was evident in our stud . Addition of a Mitrofanoff channel allows effective emptying of the augmented bladder and also gives an alternate channel bypassing the reconstructed bladder neck which sometimes can be difficult to negotiate. In our study, nearly 79 % (19/24) had a Mitrofanoff channel constructed for facilitating CIC.
Concluding message
Exstrophy-epispadias complex is difficult to manage especially in resource-poor settings and some children with exstrophy/epispadias reach adolescence and remain incontinent. However, successful rehabilitation and an improved quality of life are possible even in cases presenting in adults. For these patients, modern reconstructive techniques provide hope of continence. With careful preoperative assessment, exact surgical precision, and regular follow-up, a successful outcome can be expected in virtually all cases without the need for external urine collection devices.