Chapter 178 Vaginal Agenesis
INTRODUCTION
Description: Vaginal agenesis is congenital absence of the vagina, most often associated with an absence of the uterus (Mayer-Rokitansky-Küster-Hauser syndrome).
Predominant Age: Generally not diagnosed until puberty, often following a delay of 2 to 3 years or more.
ETIOLOGY AND PATHOGENESIS
Causes: Failure of the endoderm of the urogenital sinus and the epithelium of the vaginal vestibule to fuse and perforate during embryonic development. This process is normally completed by the 21st week of gestation. Patients with a congenital absence of the vagina but with a uterus present represent an extreme form of transverse vaginal septum.
