(133) Vaginal Reconstruction Using Urinary Bladder Tissue in a Patient with OEIS Complex

Background: Various methods of vaginal replacement have been described. The surgical approach to creation of a neovagina is dependent on the congenital malformation, available native tissue, patient’s age, and presence of functional uterine structures. Omphalocele, cloacal exstrophy, imperforate anus, and spinal anomaly (OEIS) complex is a severe congenital defect that is associated with Mullerian anomalies and duplicated external genitalia. This is the first case in the literature to describe the use of native bladder tissue for vaginal reconstruction in a child with OEIS complex.

Case: Our patient was prenatally diagnosed with OEIS complex and born via cesarean section at 37 weeks gestation. As a newborn, she underwent exploratory laparotomy, omphalocele closure, hindgut rescue with creation of colostomy, unification of hemi-bladders, and assessment of Mullerian structures. Externally, she was noted to have widely spaced pubic bones with a midline bifid clitoris and a single perineal opening leading into bladder plate tissue. Mullerian structures were visualized bilaterally with palpable cervices and bilateral distended vaginas fused with the bladder trigone. Vaginoscopy at almost 2 years old demonstrated the single perineal opening communicated with the bladder plate and bilateral pouches consistent with hemi-vaginas. The left-sided opening was blind ending, but the right side had a visualized cervix. At 3 years old, she underwent the second stage of OEIS repair with urology, orthopedics, and gynecology. The single perineal opening was examined and again found to be contiguous with the fused bladder. Bilateral hemi-vaginas inserted into this fused bladder. Patency of bilateral Mullerian structures was demonstrated with saline perturbation. A portion of the bladder contiguous with the adjoining bilateral hemi-vaginas was then mobilized and tubularized. This created both a neovaginal space for future intimacy and patency of bilateral Mullerian structures through the previously present native perineal opening. Both ureters were reimplanted into the remaining reconstructed bladder plate and an ileal segment augmented the mobilized native bladder tissue to create a right-sided ileovesicostomy.

Comments: This is the first case report describing the use of native bladder tissue for creation of a neovagina in a patient with OEIS complex. In our patient, the bilateral hemi-vaginas congenitally inserted into the native bladder, allowing bladder to be used opportunistically to create a patent Mullerian system. More long-term research is needed to determine overall success rates, complications, and patient satisfaction with urinary bladder neovaginas.