Authored by Vivek Vasudeo*
Abstract
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
Introduction
The exstrophy-epispadias complex (EEC) is a rare congenital disorder involving multiple systems. It includes a spectrum from epispadias, classical bladder exstrophy (CBE) to cloacal exstrophy with ascending complexity. CBE is the most common type, with incidence of one per 10,000 to 50,000 births and twice more prevalent in males [1,2]. Risk factors for CBE include Caucasian race, young maternal age, multiparity and children born after in vitro fertilization [3,4]. The purpose of this paper is to analyse our clinical data and results of 27 exstrophy epispadias males and females, with wide variety, presenting at different ages including four adults. CBE can be repaired by either the modern staged repair of exstrophy (MSRE) or complete primary repair of exstrophy (CPRE) in a single stage. The first step of repair (in MSRE) is closure of the bladder and abdominal wall, effectively converting CBE into complete epispadias with incontinence. Malleability of pelvic bones aids in sound abdominal wall repair if carried out within 72 hours of birth. This repair with balanced posterior outlet resistance is very crucial for decreased inflammation, fibrosis, improved bladder growth and decreased need for urinary diversion [5]. Tension free closure and pelvic immobilisation are vital for success [6]. Genitoplasty and urethroplasty are accomplished in females in the first stage. The second stage in males is closure of epispadias with a modified Cantwell-Ransley repair at 6 to 12 months of age. This procedure promotes bladder growth and capacity, critical for continence. Cantwell et al first described the technique of ventral urethral transposition for epispadias in 1895 in two cases [7]. Modified Cantwell technique was originally described by Ransley et al to attain good functional and cosmetic outcome [8]. It consists of partial disassembly of corporal bodies, urethroplasty and Ransley incision and suturing corpora in midline resulting in medial rolling of corporeal bodies [9]. Incorporated Glanuloplasty and advancement meatal (IPGAM) technique helps to attain orthotopic meatal position [10]. Female epispadias repair incorporates urethroplasty, genitoplasty (monsplasty and clitoroplasty) and bladder neck repair performed in one or two stages [11]. In the third stage, Young-Dees-Leadbetter bladder neck reconstruction (BNR) is performed to achieve continence provided patient achieves adequate bladder capacity (usually at the age of 5-9 years). This stage is combined with bilateral ureteral reimplantation to correct vesicoureteric reflux (VUR).
Materials and Methods
Ethical approval for the study was obtained from the Institutional Ethical Committee. Informed consent was obtained from all individual participants or parents for whom identifying information was included in this article. We retrospectively reviewed the data of EEC cases managed at our institution from September 2014 to November 2019. Age, sex, surgical procedures at different stages, complications and follow up were recorded from a prospectively maintained EEC registry. A total of 27 cases were managed including 15 bladder exstrophy and 19 epispadias. None of these had a prenatal diagnosis or born after invitro fertilisation. Associated anomalies in pediatric CBE patients included inguinal hernia in three (bilateral in one), rectal prolapse in two and right undescended testis in one. All surgeries were performed by a single surgeon MC Arya. All patients underwent routine blood biochemistry and ultrasound KUB. All bladder exstrophy cases were neonates or infants except two interesting cases. First was a female who was reared as male up to 19 years and another a 31 years male with skin covered exstrophy, diphallia and total incontinence. Our approach to all exstrophy patients was MSRE except in the two adult cases. Out of 19 epispadias cases 14 were males and 5 females. Female epispadias cases were classified according to Davis classification[12].
Surgical techniques
Exstrophy closure
Bladder, posterior urethra and abdominal wall closure were done in first stage at presentation as the bladder plate was adequate and there was no hydronephrosis. Key steps in this repair were bilateral ureteral cannulation, adequate mobilisation of bladder template by sharp dissection, division of urogenital diaphragm and approximation in midline, creation of neoumbilicus, approximation of symphysis pubis and application of Bryant’s traction postoperatively (Figure 1). Female exstrophy closure also included genitoplasty (Figure 2).
Male epispadias repair
This was done in second stage at 6 months to 1 year. The procedure adopted was slightly different from modified Cantwell Ransley procedure in the way that the urethral plate was mobilised off the corpora except at the glans. This facilitated transposition of urethra ventrally and closure of urethral plate in two layers thereby reducing fistula incidence (Figure 3). Further this allowed better medial rotation of corpora after Ransley incision to correct chordee. Partial disassembly also avoids hypospadiac meatus, a common complication of complete dissesembly (Mitchell). Glansplasty was followed by dorsal transposition of ventral prepuce by Byar’s flap. In all cases, the neurovascular bundles were identified, isolated and preserved to prevent glans necrosis.
Female epispadias repair
Flat mons, bifid clitoris and patulous or dorsally split urethra are the hallmarks of female epispadias. Glabrous skin of mons pubis was excised, subcutaneous fat was mobilised from both sides and brought in midline. After applying stay sutures, dorsally a wedge of redundant tissue was excised to narrow urethra. Urethra was reconstructed over 10 F Foley catheter using 6-0 polydioxanone. Medial side of clitoris and labia minora were denuded and closed over neourethra (Figure 4).
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