V3-06: FEMALE EPISPADIA: A RARE ABNORMALITY TREATED BY PERINEAL AND INFRAPUBIC APPROACH
VideoIntroductions and Objectives
Female epispadia is a rare congenital anomaly occurring in 1 of 500,000 live births. The male/female ratio is 3 to 4:1. Most of the patients present with urinary incontinence and a genital deformity characterized by a bifid clitoris, depressed mons pubis, and ill-defined labia minora, is covered by smooth glabrous skin, and terminates anteriorly with the corresponding half of the bifid clitoris. The vagina and internal genital organs are usually normal. We aimed to demonstrate the perineal infrapubic approach to treat this rare abnormality.
Patient presented at an age of . We defined and created a flap of the mucosal urethral plate located from the epispadic meatus up to anterior supra-pubic area between both bifid clitoris. The flap was moved inferiorly and gave access to pubic bone. A careful infra-pubic dissection of the flap was performed to allow further bladder neck and proximal urethra tightening. We tubularized the mucosal flap excising redundant mucosal tissue adapting the urethra for a 6F catheter. Subsequent overlapping and folding of adjacent muscular and subcutaneous tissue in two layers was done. The labia minora plasty and monsplasty were performed and bifid clitoris were approximated in the midline. Vulvoplasty was completed and distal urethral meatus was anchored cranially inferiorly to clitoral hood.
Patient had an uneventful postoperative course, the Foley tube was removed after 7 days. Present follow-up is 5 months, parents reported urinary continence of a 3 hours period.
The one-stage perineal urethroplasty increases the urethral and bladder neck resistance, mainly by elongating urethral segment and folding adjacent muscular and subcutaneous tissue. It is a simple and effective method for the development of both continence and cosmesis in female epispadias, avoiding initial abdominal surgery that could be reserved for the failure cases.