Long-term urological and gynecological outcomes following complete primary repair in females with bladder exstrophy.

INTRODUCTION

Long-term continence outcomes in patients undergoing complete primary repair of exstrophy (CPRE) have shown that a subset of patients do not achieve continence until after puberty. We aim to update the continence outcomes as well as describe gynecological outcomes for females having undergone CPRE.

MATERIALS AND

METHODS

This was a retrospective review between 1989 and 2019 at a single institution. Inclusion criteria were females with classic bladder exstrophy who underwent CPRE. Exclusion criteria were variant diagnoses and age <4 years. Continence was defined as dry for ≥3 h (volitional voiding or clean intermittent catheterization (CIC)). Partial continence was defined as minor stress incontinence or enuresis. Vaginal stenosis was evaluated in post-pubertal patients.

RESULTS

Eighteen patients met inclusion criteria. Median age at last follow-up was 15.9 years (IQR 13.1, 18.4). All patients underwent primary closure <30 days of life (n = 18). Subsequent procedures included bladder augmentation (n = 4), continent catheterizable channel (n = 7), bladder neck injections (n = 12) and bladder neck reconstruction (n = 12). Continence and partial continence were achieved in 6/18 (33.3%) and 9/18 (50.0%), respectively, with mean 3.2 ± 2.5 continence procedures at 9.6 years (IQR 7.3, 15.2). Volitional voiding was seen in 11/18 (61.1%) and 7/18 (38.9%) performed CIC, with no significant difference in continence. Mean bladder capacity was 199 ml ± 96 versus 90 ml ± 29 in the volitional voiding versus CIC group (P = 0.0047). Eleven women with median age of 18.0 years (IQR 15.2, 21.4) had recorded menarche: 6/11 (54.5%) patients reported painful/irregular menses, controlled with hormonal therapy. Six of 11 (54.5%) women had vaginal stenosis managed with vaginal dilation (n = 2) or vaginoplasty (n = 4). Three (27.3%) reported tampon use and penetrative intercourse.

CONCLUSION

Overall, the majority of women who have undergone CPRE achieved complete or partial continence, though most required additional procedures and time to attain it. Additionally, volitional voiding was achievable. Bladder capacity was significantly lower in patients dependent on CIC. Most required medical or surgical interventions for gynecologic concerns post menarche. This study underscores the unique needs of girls and young women with bladder exstrophy and further supports the importance of close long-term urologic and gynecologic management throughout development.