MAYER-ROKITANSKY-KÜSTER-HAUSER SYNDROME: A LITERATURE REVIEW ON UTERINE AGENESIS

Abstract

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a rare congenital malformation characterized by the absence of the uterus and the upper two-thirds of the vagina in women with a 46XX karyotype and normal secondary sexual characteristics. Diagnosis typically occurs during adolescence due to primary amenorrhea and can have a significant emotional impact on patients. This review aims to explore the etiology, diagnosis, and therapeutic options for MRKH, focusing on conservative treatment through vaginal dilation and surgical interventions such as vaginoplasty. Vaginal dilation is recommended as the first-line treatment due to its minimally invasive approach and high success rates. However, surgery remains an effective alternative for patients who do not respond to conservative treatment. The importance of multidisciplinary follow-up is emphasized, considering the psychological and physical challenges faced by patients.