Sigmoid neovaginoplasty in the androgen insensivity syndrome (AIS): the ideal technique for the pediatric urologist?

Abstract

Purpose AIS (Morris syndrome) is the most frequent cause of male pseudohermaphroditism and consists of a sex-linked recessive hereditary sexual disorder resulting from a loss-of-function mutation of the androgen receptor gene located on the long arm of the X chromosome. The complete or partial lack of response to androgen stimuli, results in undervirilization of external genitalia, absent or poor müllerian development, undescended testes, and XY karyotype with female phenotype. We present a video of a sigmoid neovaginoplasty in a 17 years old patient who previously underwent in childhood a bilateral inguinal mass exploration and therefore orchiectomy allowing histopathological diagnosis Material and methods Under general anaesthesia physical examination showed a 2 cm deep vagina and a narrow vaginal introitus. A Pfannenstiel incision was performed and a 14 cm segment of sigmoid colon was mobilised.The cranial border of the sigmoid was closed by two uninterrupted 3.0 vicryl suture layers. The vaginal dome was identified in the pelvis with the help of a Hegar dilator taking care not to involve the bladder wall. The dome was incised and the sigmoid anastomosed to the vagina by interrupted 3.0 vicryl sutures. A Silicone conical prosthesis was kept in the neovagina for 21 days to avoid retraction of the anastomosis. Results The patient had a nice clinical result and after removal of the silicone prosthesis she has been dilating the anastomotic area at home at least once a day without any evidence for stricture. Mean follow-up is now 6 months. Conclusions The use of the sigmoid colon is an excellent source of tissue to perform a neovaginoplasty in children and adolescents and has the advantage of producing an immediate nice end result. Furthermore it is a straightforward procedure for pediatric urologists who are trained to work with intestinal segments for other pathologies. AIS (Morris syndrome) is the most frequent cause of male pseudohermaphroditism and consists of a sex-linked recessive hereditary sexual disorder resulting from a loss-of-function mutation of the androgen receptor gene located on the long arm of the X chromosome. The complete or partial lack of response to androgen stimuli, results in undervirilization of external genitalia, absent or poor müllerian development, undescended testes, and XY karyotype with female phenotype. We present a video of a sigmoid neovaginoplasty in a 17 years old patient who previously underwent in childhood a bilateral inguinal mass exploration and therefore orchiectomy allowing histopathological diagnosis Under general anaesthesia physical examination showed a 2 cm deep vagina and a narrow vaginal introitus. A Pfannenstiel incision was performed and a 14 cm segment of sigmoid colon was mobilised.The cranial border of the sigmoid was closed by two uninterrupted 3.0 vicryl suture layers. The vaginal dome was identified in the pelvis with the help of a Hegar dilator taking care not to involve the bladder wall. The dome was incised and the sigmoid anastomosed to the vagina by interrupted 3.0 vicryl sutures. A Silicone conical prosthesis was kept in the neovagina for 21 days to avoid retraction of the anastomosis. The patient had a nice clinical result and after removal of the silicone prosthesis she has been dilating the anastomotic area at home at least once a day without any evidence for stricture. Mean follow-up is now 6 months. The use of the sigmoid colon is an excellent source of tissue to perform a neovaginoplasty in children and adolescents and has the advantage of producing an immediate nice end result. Furthermore it is a straightforward procedure for pediatric urologists who are trained to work with intestinal segments for other pathologies.