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Female primary epispadias: Radical soft tissue mobilization (Kelly procedure) or perineal urethroplasty ?
Marc-David LECLAIR, MD, PhD1, Thierry VILLEMAGNE, MD2, Guillaume LEVARD, MD3, Emilie EYSSARTIER, MD4, Marie BEY, MD5, Benjamin FREMOND, MD, PhD6, Philippe RAVASSE, MD, PhD7, Yves HELOURY, MD1.
1Children University Hospital, NANTES, France, 2University Hospital, TOURS, France, 3University Hospital, POITIERS, France, 4University Hospital, ANGERS, France, 5Pediatric Surgery, SAINT-BRIEUC, France, 6University Hospital, RENNES, France, 7University Hospital, CAEN, France.

BACKGROUND:
Primary female epispadias is a rare condition, presenting as a spectrum from almost-continent girls with bifid clitoris, to total sphincteric incontinence with small bladder.
We report on the results of a tailored management of female epispadias, based on bladder assessment at diagnosis.
METHODS:
Girls with primary female epispadias were included in this retrospective monocentric study. Bladder assessment at diagnosis included cystoscopy, cystourethrogram, and cystomanometry.
Patients with adequate bladder at diagnosis (normal bladder capacity adjusted to age/normal compliance) underwent cervicourethroplasty through perineal approach as a primary procedure (groupe 1). Girls with small or poorly compliant bladder were treated with radical soft tissue mobilization and cervicoplasty (Kelly repair, group 2).
Follow-up was based on functional results, US and DMSA renal scans, uroflowmetry with residuals measurements , and cystomanometry whenever necessary.
Functional results were classified according to Continence Scores as follows :
- grade 0 : total incontinence
- grade I : able to retain with dry intervals, still wearing protections
- grade II : sufficient dry intervals/day, wet at night.
- grade III : Dry days/nights.
RESULTS:
14 consecutive girls were referred to our unit after being diagnosed with primary female epispadias at birth (n=6) or later for incontinence (n=8, median age : 30 months) from 2006 to 2013.
Preoperative investigations showed near-normal bladder capacity in 8 patients, and small or poorly compliant bladder in 6/14.
Accordingly, 8 girls underwent primary perineal urethroplasty, and 6 girls were treated with primary Kelly radical soft tissue mobilization.
At last follow-up (median 32.5 postoperative months [8-102]), 9/14 girls showed near-normal continence grade II (n=1) or grade III (n=8), and 5/14 present some dry intervals (continence grade I, all of them being either below toilet-training age, or with a postoperative follow-up <12months).
In the perineal urethroplasty group, most girls (6/8) developed normal continence, with normally growing and compliant bladder. Among them, 1/6 eventually revealed obvious obstructive voiding pattern, according to cystomanometry, uroflows, and postvoid residuals without any complications. Of the remaining two, one girl with inadequate bladder growth required bladder augmentation with bladder neck repair, and the latter with adequate bladder underwent subsequent intracervical bulking agents injections and secondary Kelly repair to increase infra-cervical resistances.
In the Kelly repair group, 3/6 patients developed acceptable social continence (grade II or III). One of them showed adequate bladder growth, but obstructive pattern at 36 months after the Kelly operation, and is now dry under CIC.The three remaining girls are currently below toilet training age and are not evaluable, but yet present some dry intervals.
CONCLUSIONS:
Female epispadias remains a challenging malformation. Surgical strategy based on preoperative bladder capacity and compliance assessment provides acceptable social dryness rates in up to 64% of patients.
Perineal urethroplasty, when applied to children with adequate bladder capacity, offers dryness in most children with minimal risks of passive obstruction.
In children with small bladders, the Kelly operation, when performed early, may help to enlarge bladder and provides continence in >50% of the cases. Whether the Kelly repair truly provides active resistances remains to be investigated.


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