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The Fate of the Complete Female Epispadias and Female Exstrophy Bladder: Is There A Difference?

Monday, October 22, 2012: 1:18 PM
Grand Ballroom B (Hilton Riverside)
Kristina D. Suson1, Janae Preece, MD2, Nima Baradaran3, Heather N. DiCarlo, MD4 and John P. Gearhart3, (1)Pediatric Urology, Johns Hopkins University School of Medicine, Baltimore, MD, (2)Division of Urology, University of Maryland Medical Center, Baltimore, MD, (3)Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, MD, (4)Urology, Stony Brook University Medical Center, Stony Brook, NY

Purpose Complete female epispadias (CFE), occurring much more rarely than classic bladder exstrophy (CBE) in females, is thought to offer a more benign clinical course. Voided continence rates up to 80% are quoted. Given that one of the predictors of continence in exstrophy patients is bladder capacity, the authors compared the bladder capacities of girls with CFE to those of girls with CBE and evaluated continence.

Methods After obtaining IRB approval, females with CFE or CBE were identified from an institutionally approved prospective database. Inclusion criteria included female gender, minimum of two bladder capacity (BC) measurements, and initial reconstructive surgery after 1991. For CBE, only those undergoing primary closure were included. Charts of 23 CFE and 24 female CBE (3 delayed primary closure) patients were retrospectively reviewed.

Results CFE girls presented later than CBE girls (33.3 months vs 0.5 months, p < 0.001) and underwent their first reconstructive procedure at an older age (34.7 months vs 0.7 months, p < 0.001). There was no difference in total urologic procedures (7.8 vs 8.9, p = 0.369). CFE patients had a lower initial age-adjusted BC when compared to girls with CBE (23.8 vs 67.3 mL/year of life, p < 0.001). CFE patients also had a lower final age-adjusted BC (21.0 vs 35.0 mL/year of life, p = 0.004). Rate of bladder growth did not differ between these groups (23.5 vs 27.4 mL/y, p = 0.5). When CFE patients who underwent initial genital reconstruction and urethroplasty at one year or less are compared to those undergoing reconstruction when older than one year, there are no statistically significant differences in final age-adjusted BC or rate of bladder growth. 9% of CFE patients and 25% of CBE patients achieved at least a two hour dry interval from initial reconstruction (p = 0.245). The choice and success of continence procedure, when performed, is presented in Table 1.

Conclusion This study suggests that CBE females have higher initial and final age-adjusted bladder capacities, with a similar rate of growth, as CFE females. This may reflect creation of outlet resistance at a younger age than those with CFE, although no difference was identified between CFE patients initially repaired ≤ or > 1 year. Females with CFE undergo similar procedures to obtain continence as those with CBE and a successful primary closure.

 

 

Complete Female Epispadias

Female Bladder Exstrophy

Bladder Neck Reconstruction Continent Stoma

64.3% (100% continent*)

80% (87.5% continent, 12.5% 2 hour dry interval)

Bladder Neck Reconstruction + Augment + Stoma

14.3% (100% continent)

0%

Bladder Neck Transection Augment + Stoma

21.4% (100% continent)

20% (100% continent)

*Of patients who have followed up at least 1 year after reconstruction.