Mayer-Rokitansky-Küsterhauser syndrome

case report

Abstract

The Mayer-Rokitansky-Küster-Hauser syndrome is characterized by congenital absence of the upper third of the vagina, uterus and fallopian tubes as a result of agenesis or hypoplasia of Müller’s ducts. It is the second most common cause of primary amenorrhea and occurs in 1: 4000 to 1: 5000 women, and it may be associated with urinary tract congenital anomalies in 30-40% of cases. From these, pelvic kidney and unilateral renal agenesis is the most common. This case report deals whit the Mayer-Rokitansky-Küster-Hauser syndrome associated with pelvic kidney, the diagnosis of which was carried out through a clinic radiological assessing, in which ultrasonography was the initial method. In this case, it is also approached socio-cultural and economic aspects involved, mainly in treatment.