David B Meir1, John M Hutson. 1. F Douglas Stephens Surgical Research Laboratory, Murdoch Children's Research Institute, Australia.
Abstract
OBJECTIVE: To present a hypothesis to explain the various anatomical variants of children with both a vas deferens and retained vagina. MATERIALS AND METHODS: A child with both vasa entering into a blind-ending vaginal sinus opening into the prostatic urethra is presented, followed by a review of studies published in English describing children with a vas deferens and a retained vagina, and normal and abnormal vaginal development. RESULTS: The caudal end of the vas deferens and its relationship to a retained vagina are not well described in the literature. Adequate exocrine levels of androgen inside the Wolffian duct may allow its preservation in conjunction with a related vagina (secondary to inadequate endocrine levels of androgen), with incorporation of the vas into the vaginal wall. CONCLUSIONS: We propose that testosterone and Müllerian-inhibiting substance/anti-Müllerian hormone reach the Wolffian and Müllerian ducts by exocrine passage through the Wolffian duct. Thus, where the gonad is a dysgenetic testis the ipsilateral Wolffian duct will be preserved in proportion to the amount of testosterone available. Where a fetus has adequate exocrine secretion of androgen down the Wolffian duct, but inadequate endocrine blood levels of androgen, the vas deferens and vagina can coexist, with incorporation of the vas into the wall of the vagina.
OBJECTIVE: To present a hypothesis to explain the various anatomical variants of children with both a vas deferens and retained vagina. MATERIALS AND METHODS: A child with both vasa entering into a blind-ending vaginal sinus opening into the prostatic urethra is presented, followed by a review of studies published in English describing children with a vas deferens and a retained vagina, and normal and abnormal vaginal development. RESULTS: The caudal end of the vas deferens and its relationship to a retained vagina are not well described in the literature. Adequate exocrine levels of androgen inside the Wolffian duct may allow its preservation in conjunction with a related vagina (secondary to inadequate endocrine levels of androgen), with incorporation of the vas into the vaginal wall. CONCLUSIONS: We propose that testosterone and Müllerian-inhibiting substance/anti-Müllerian hormone reach the Wolffian and Müllerian ducts by exocrine passage through the Wolffian duct. Thus, where the gonad is a dysgenetic testis the ipsilateral Wolffian duct will be preserved in proportion to the amount of testosterone available. Where a fetus has adequate exocrine secretion of androgen down the Wolffian duct, but inadequate endocrine blood levels of androgen, the vas deferens and vagina can coexist, with incorporation of the vas into the wall of the vagina.