[Congenital absence of the vagina. Mayer-Rokitansky-Kuster-Hauser syndrome. Presentation of a case and review of the literature]. / Ausencia congénita de vagina. Síndrome Mayer-Rokitansky-Kuster-Hauser. Presentación de un caso y revisión de la literatura.

ABSTRACT

The normal development of the feminine reproductive tract depends on complete interaction between genetic, hormonal and environmental factors to produce the differentiation of the Müellerian, Wolff and urogenital sinus. The alteration in this balance result in a wide spectrum of the reproductive tract anomalies including imperforated hymen, vaginal septum, vaginal atresia, incomplete fusion of the Müellerian conducts and Müellerian aplasia. In order to evaluate the feminine genital tract disorders, we studied a feminine with amenorrhea and primary sterility. Ultrasonography and diagnosis laparoscopy where performed. Incomplete fusion of Müellerian conducts was observed. Blood genetics tests plus biopsy of ovarian tissue were taken which reported 46 XX karyotype with no structural anomalies. Conservative evaluation and management.