Exstrophy Epispadias Complex: Outcome Analysis of Repair Performed at A Single Centre

Objective: The exstrophy-epispadias complex (EEC) is a rare developmental defect which affects multiple systems including the genitourinary system. We share our outcome analysis of 34 surgeries performed on 27 EEC cases at our institution.

Material and Methods: This is a retrospective analysis of the outcome and follow up of patients operated for EEC. A total of 27 cases including 15 bladder exstrophy (11 Males, 4 Females) and 19 epispadias (14 Males, 5 Females) were treated from September 2014 to November 2019. Total 34 surgeries were performed on 27 patients which included 7 male cases of closed exstrophy who underwent subsequent epidpadias repair. These included two interesting exstrophy cases also. One of them was a female bladder exstrophy reared as male up to 19 years and another a 31 years male with skin covered exstrophy, diphallia and total urinary incontinence since birth. Epispadias group also included two adult males.

Result: Thirteen pediatric patients underwent primary bladder and abdominal closure. Bladder reclosure rate was 14.2%. One adult male underwent Mitrofanoff diversion. The other adult- 19 year female had bladder closure, followed by bladder augmentation and rectus flap interposition. Male epispadias repair was performed in 14 patients (7 post closed exstrophy and 7 epispadias). Fistula rate after male epispadias repair was 15.3%. Five females underwent repair for epispadias. Bladder neck reconstruction (BNR) was carried out in 6 patients. Continence rates post BNR was 83% (including social continence).

Conclusion: This congenital anomaly requires accomplishment of meticulous repair in staged manner. These cases need follow up from birth to adulthood. Presentation at adult age is extremely rare and related to social stigma, illiteracy and poor access to health care system.

Keywords:Bladder exstrophy; Epispadias; Bladder neck reconstruction; Urinary incontinence; Genitoplasty

Abbreviations: EEC: Exstrophy- epispadias complex; BNR: Bladder neck reconstruction; IPGAM: Incorporated Glanuloplasty and advancement meatal; CBE: Classical bladder exstrophy; CPRE: Complete primary repair of exstrophy; MSRE: Modern staged repair of exstrophy; VUR: Vesicoureteric reflux; SPCL: Suprapubic Cystoloithotomy; CIC: Clean intermittent catheterisation; IVU: Intravenous Urography; BSRI: Bem sex role inventory; SP: Suprapubic; GIDYQ-AA: Gender Identity/ Gender dysphoria questionnaire for adolescent and adults