Abstract

MAYER - ROKITANSKY - KÜSTER - HAUSER SYNDROME: ABOUT A CLINICAL CASE

Jeraf Narjisse*, Driouech Aicha, Ouzaa Aziza, Erhaymini Khalil, Mohammed Hassan Alami, Zakia Tazi, Filali Adib and Rachid Bezad

ABSTRACT

MAYER - ROKITANSKY - KÜSTER - HAUSER syndrome (MRKH) is the most common cause of congenital absence of the vagina associated with malformations of the uterus. The body and cervix are missing. There is only a bilateral uterine outline thus forming two buds connected by a transverse peritoneal fold. The tubes and ovaries are normal. It is a rare birth defect caused by abnormalities in the early stages of embryogenesis. Several causes have been considered but have not yet been proven and validated. This syndrome may be accompanied by other abnormalities including kidney, bone and hearing. The diagnosis is based on the clinic and a few simple additional examinations such as ultrasound and MRI. We report the case of an 18-year-old married girl who consults for desire for fertility, amenorrhea and cyclic pain, in whom clinical examination suspects ROKITANSKY syndrome confirmed by ultrasound and MRI.

Keywords: MAYER - ROKITANSKY - KÜSTER - HAUSER syndrome, amenorrhea.