Setting expectations: The influence of pubic diastasis and surgical burden in isolated female epispadias.

INTRODUCTION

Female epispadias represents an abnormal or incomplete fusion of the dorsal urethra. There can be varying degrees of urethral malformation with or without coexisting abnormalities of the bladder neck. Patients often have a bifid clitoris, depressed mons pubis, and ill-defined labia minora. Occurring in 1:400,000 live female births and is rarer than other Bladder Exstrophy-Epispadias Complex (BEEC) diagnoses, including isolated male epispadias. Like all pathologies within the BEEC, this condition is associated with varying degrees of widened pubic diastasis. This study sought to evaluate continence outcomes in women with epispadias as well as investigate a correlation between width of pubic diastasis and surgical burden.

METHODS

An IRB-approved, prospectively maintained, single-institutional BEEC database was utilized to identify female patients with isolated epispadias. Electronic medical records were reviewed for data pertaining to patient demographics, epispadias repair, genital reconstruction, and continence procedures and outcomes. Width of pubic diastasis was recorded through measurements obtained from radiographic imaging. Continence was defined as being dry between voids or catheterization, with no daytime or nighttime leakage. The aforementioned factors were assessed for their impact on continence outcomes.

RESULTS

72 isolated female epispadias cases were found in the database. 34 patients with complete data were included in the study. Of the 34 patients, 18 (53%) achieved continence while the remainder (16, 47%) were incontinent. Median width of pubic diastasis in the continent group (2.90, IQR 2.23-3.9, cm) when compared to the incontinent cohort (3.00, IRQ 2.73-3.50, cm) was not statistically different (p = 0.5669). Methods of bladder emptying in the continent group include: 4 (22.2%) void per urethra, 13 (72.2%) catheterize per stoma, and 1 (5.6%) per rectum. This is significant when compared to the incontinent group, where 16 (94.1%) void per urethra and 1 (5.9%) catheterizes via stoma (p < 0.0001). All 18 continent patients required at least one additional surgery after index epispadias repair to achieve continence. Those that received an increasing number of continence surgeries had a significantly wider pubic diastasis (2.30 cm vs 2.55 cm vs 4.00 cm) (p = 0.0025).

CONCLUSIONS

This study evaluates the potential correlation of continence status with surgical burden and pubic diastasis in female epispadias patients. This cohort required at least one continence surgery after the initial epispadias repair to become dry and most continent patients utilize CIC. These findings can aid with clinical decision making, regarding proceeding with continence surgery and management of patient and parent expectations.