Abstract

*Hanane Ouhame, Fatima Zahra Belkouchi, Samir Bargach and Mounia Yousfi

ABSTRACT

Mayer Rokitansky-Kuster-Hauser syndrome or utero-vaginal aplasia syndrome is a very rare birth defect. It is characterized by an absence of the uterus or a rudimentary uterus when the ovaries are normal. This anomaly is mainly represented by primary amenorrhea as well as unsatisfactory or even impossible sexual intercourse. The external genitalia and secondary sexual characteristics are normally developed, in particular breast growth and pubic hair growth. The karyotype is 46 XX, with no clearly visible chromosomal anomaly, and the hormonal balance is considered normal. The diagnosis is confirmed by imaging. This syndrome can be isolated (Type I) or associated with other renal, skeletal, auditory or cardiac malformations (Type II). The therapeutic attitude is very complicated, and should always start with non-surgical methods; it is only after failure that the surgical methods are indicated and requires an experienced medical team without forgetting the psychological support of the patient. Through our 3 cases, we will study Mayer Rokitansky-Kuster-Hauser syndrome with a review of the literature.