Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Sherif Maher

Presentation

Primary amenorrhea and obesity.

Patient Data

Age: 25 years
Gender: Female

The uterus and the upper two-thirds of the vagina are absent while the lower third of the vagina appears normal. Bilateral uterine buds on the lateral pelvic walls anterior to and abutting the ovaries. Normal appearing ovaries with functional ovarian cysts. A little amount of free pelvic fluid was also present.

Case Discussion

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Rokitansky syndrome, is a
malformation of the female genital tract that is due agenesis of müllerian duct derivatives. The uterus and upper two-thirds of the vagina are absent. It affects approximately one in 4500 live births. Ovarian function is normal; thus, patients usually present during adolescence with primary amenorrhea in the presence of normal pubertal development and secondary sexual characteristics.

Special thanks to Dr Jakkani Ravikanth

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