Operative Strategy and Optimal Timing in Feminizing Genitoplasty

Abstract

Life prognosis and quality of life have improved in patients with persistent cloaca, cloacal exstrophy, and disorders of sex development; however, the long-term reproductive function of these patients is not satisfactory. In this study, we aim to determine the optimal surgical timing and operative strategy in feminizing genitoplasty (vaginoplasty). In Keio University Hospital, a multidisciplinary team consisting of experts in pediatric surgery, pediatrics, urology, and gynecology is formed, and tailored treatment is provided for each patient. In this study, the cases of 5 patients (two had persistent cloaca and the other three had either cloacal exstrophy, congenital adrenal hyperplasia, or primary hypogonadism) who underwent feminizing genitoplasty were retrospectively reviewed. The length of the common channel, each patient’s wish, and past surgical history were all taken into account to determine the operative method that each patient underwent. A patient with persistent cloaca and a long common channel underwent the vaginal substitute method (intestinal canal). The other patient with persistent cloaca had strong adhesion and required regenitoplasty by the skin-flap method. The cloacal exstrophy patient underwent vaginal re-pull-through. The congenital adrenal hyperplasia patient had vaginal stenosis after the previous partial urogenital mobilization and required total urogenital mobilization. In the patient with primary hypogonadism, the pelvic peritoneum was utilized to create a neovagina. All but one patient experienced fair postoperative conditions with adequate vaginal patency. Given the variety of diseases requiring feminizing genitoplasty, drawing a tailored roadmap of the treatment strategy and optimal timing by a multidisciplinary team from the infancy period is essential to fulfill each patient’s need.