Obstetrics & Gynecology
The fact that this patient has normally developed secondary sex characteristics proves that the hypothalamic-pituitary-ovarian function is normal. Patients with testicular feminization syndrome do not have pubic or axillary hair. In this syndrome, testicles produce testosterone but the target cells in the hair follicles are believed to lack androgen receptors. Patients with an imperforate hymen usually complain of cyclic lower abdominal pain that worsens progressively because of uterine and vaginal distention with accumulated obstructed menstrual flow. The remaining diagnosis is genital tract anomaly, the most common of which is vaginal agenesis. Patients with congenital absence of the vagina usually lack a uterus as well and are likely to seek medical advice for primary amenorrhea at puberty or later, as this patient did. The development of secondary sex characteristics, ovaries, and sex chromosome complement is normal in patients with Rokitansky-Küster-Hauser syndrome.
- Rokitansky-Küster-Hauser syndrome (vaginal and uterine agenesis).
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