SEGMENTAL AGENESIS OF THE VAGINA AND FALLOPIAN TUBES

A CASE REPORT

Abstract

Müllerian anomalies are defined as the absence or underdevelopment of structures derived from the Müllerian duct, including the fallopian tubes, uterus, and the upper two-thirds of the vagina. It is considered a variant of Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH) and the most common form of Müllerian dysgenesis. MRKH involves the agenesis or dysgenesis of the Müllerian portion of the vagina and uterus during embryogenesis, clinically presenting as primary amenorrhea, normal secondary sexual characteristics, a 46,XX karyotype, and a short vagina (depth of 1 to 2cm). Diagnosis can be made through physical examination, hormonal profiling, and imaging studies. Studies suggest that the preferred first-line treatment should involve the use of vaginal prostheses for pressure dilation, with surgery reserved for cases where clinical treatment is unsuccessful. The objective of this study is to describe the case of a young patient who experienced severe pain in the hypogastric region, primary amenorrhea, and normal secondary sexual characteristics. During the diagnostic investigation, the occurrence of MRKH was confirmed.