Mayer-rokitansky-kuster-hauser syndrome : a study of fifteen cases

Abstract EN

Although Mayer Rokitansky Kuster Hauser (MRKH) is a rare entity, it is a fairly common cause of primary amenorrhoea.

The purpose of the study is to establish guideline for the investigation of a patient suspected to have a MRKH and to look for some congenital defects which may be associated with this syndrome.

We reviewed fifteen cases of MRKH seen at the King Fahd Hospital of the University, Al-Khobar, and Al-Madina Maternity and Children's Hospital, Al-Madina, Saudi Arabia.

The age of the patients ranged between 14 to 24 years old (mean 17.3 years old).

Eight of the patients complained of delayed menstruation, five of whom were unable to conceive, and two complained of dyspareunia.

All patients were investigated by cytogenetic studies, hormonal profile, intravenous pyelography, pelvic ultrasound and a diagnostic laparoscopy.

Cytogenetic studies revealed that all fifteen patients were normal females of 46XX karyotype.

Hormonal studies in nine patients were within normal range, but in the other six the hormonal values correlated well with polycystic ovary syndrome and this were confirmed by pelvic ultrasound.