Unilateral renal agenesis and female genital tract pathologies

OBJECTIVES: To analyze the gynecological pathologies and extragenital anomalies associated with unilateral renal agenesis (URA) and the possible origin of these congenital anomalies. DESIGN: Retrospective case-control study. SETTING: University Hospital. POPULATION: This study included 276 women with genitourinary malformations who had undergone hysterosalpingography (and/or laparoscopy) and pyelography with images available for review. METHODS: There were 60 cases of women diagnosed with genital malformations and congenital URA and 216 control cases of women with genital tract malformations and both kidneys present. All cases were categorized according to an embryological-clinical classification and the type of Müllerian malformation (American Society for Reproductive Medicine (ASRM) classification) and then compared by type for the presence of gynecological and extragenital pathologies. MAIN OUTCOME MEASURES: Genital malformations, endometriosis, leiomyomas and skeletal anomalies. RESULTS: URA was generally associated with either agenesis of all of the derivatives of the urogenital ridge on the same side of the body, which were usually found on the left, or distal mesonephric anomalies such as a double uterus with a blind hemivagina or unilateral cervico-vaginal atresia, which were most frequently on the right. The uterine malformations that were most commonly seen in women with renal agenesis were bicornis-bicollis, didelphys and unicornuate uteri. Women with bicornuate or didelphys uteri and renal agenesis had more gynecological pathologies, such as endometriosis, than those with both kidneys present. CONCLUSIONS: URA and major uterine malformations are frequently related, and individuals with bicornuate or didelphys uteri have endometriosis more often than those without renal agenesis. Those malformations that seem to be caused by the absence or anomaly of a mesonephric duct lead to renal agenesis, ipsilateral vaginal anomalies (blind or atretic hemivagina) and failure of the induction function of the Wolffian ducts on the Müllerian ducts, causing uterine malformations.