Single pelvic kidney & Mullerian duct anomaly

Discussion:

Referred for evaluation of fever. Incidentally she was found to have a single pelvic kidney, absent uterus and ovaries.

Class I — agenesis/hypoplasia
Early developmental failure of the Müllerian ducts, for unknown reasons at around 5 weeks' gestation, results in various degrees of agenesis or hypoplasia of the uterus, cervix and upper two-thirds of the vagina. In agenesis a uterus is not identified or small amounts of rudimentary tissue without differentiation may be present. The most common form is the Mayer–Rokitansky–Kuster–Hauser syndrome. This consists of a combined agenesis of the uterus, cervix and upper portion of the vagina. Symptoms may manifest at puberty as primary amenorrhea with normal secondary sexual characteristics, as ovarian function is preserved. The ovaries originate within the primitive ectoderm and not from the paramesonephros, and thus these patients have normal female sexual development. In uterine hypoplasia, a small but fully differentiated organ is present.

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