In cases of partially or completely developed secondary sexual characteristics, patients will refer to the physician for primary amenorrhea at an age of 14 or 15 years. Gonadotropin levels are normal and can be stimulated by a LHRH test.
The clinical exam will check that genitalia are normal using complementary ultrasonography and also look for associated clinical and biological hyperandrogenia.
- Cyclic pain and hymeneal convexity suggest the hematocolpos of the unperforated hymen. It will be assessed by ultrasound and treated by radial incisions.
- Complete vaginal partition or vaginal aplasia is visualized using a speculum for virgins. Ultrasound will confirm the presence of a uterus and a hematometria. The goal of surgical treatment will be to obtain a vaginal cavity connecting with the uterus and allowing sexual intercourse.
- In Rokitansky - Kuster-Hauser syndrome, vaginal aplasia is associated with uterine hypoplasia or aplasia. Ovaries and fallopian tubes are usually preserved. Ultrasonographic evaluation is again crucial and will also allow verification of the renal apparatus because renal abnormalities are frequently associated. Laparoscopy will confirm the diagnosis and suggest the most appropriate surgical treatment. Surgery consists in creating a neovaginal cavity by several means to allow sexual intercourse. Pregnancy will nevertheless not be possible because of the lack of a uterus.
- Prepubertal genital tuberculosis with uterine synechia and primary amenorrhea is nowadays exceptional in developed countries.
Revision date: June 20, 2011
Last revised: by Jorge P. Ribeiro, MD