Abstract

Congenital variations of the reproductive system arise during embryonal organogenesis, although their clinical manifestations present later in adolescence or during reproductive years due to functional disorders such as abnormal menstruation, sexual dysfunction, infertility, and pregnancy loss. Surgical treatment of congenital variations is performed according to variant anatomy and functional disorders. However, many congenital anatomic variations are difficult to categorize based on current classification systems. The aim of this study is to distinguish female genital anatomic variations for appropriate surgical treatment based on a clinical analysis of uterovaginal malformations and disorders of sex development. The deviant anatomy defined as variations, which has been used in the manuscript instead of anomalies. A retrospective review was conducted of 718 cases of uterovaginal malformation and 144 cases with disorders of sex development. Physical variations were assessed by ultrasound, magnetic resonance imaging, and laparoscopy. Genetic studies were conducted for individuals with disorders of sex development. Classification of variants was determined by physical findings, genetic findings, and clinical presentations. A unified systematization for female genital anatomic variations was proposed, based on the genome (karyotype), gonadal morphology, internal and external genital anatomy. The internal anatomy was classified to uterovaginal variations, which have distinguished to 11 basic types and 24 variants, according to morphologic patterns. Surgical treatments are proposed based on these variants. These new classifications provide a framework for clinical management and appropriate surgical treatment of female genital anatomic variants, with the aim of improving reproductive outcomes.

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