Congenital absence of vagina

This code describes the complete congenital absence of the vagina, a rare developmental anomaly where the vaginal canal fails to form during fetal development. This condition is often associated with other Müllerian duct anomalies, such as uterine agenesis or hypoplasia.

This code is used when documentation clearly indicates a diagnosis of congenital absence of the vagina, typically identified during adolescence due to primary amenorrhea and/or inability to engage in sexual intercourse. Supporting documentation includes physical examination findings, imaging studies (e.g., MRI, ultrasound) confirming the absence of the vaginal canal, and genetic testing if performed.

• Do not use for acquired vaginal absence due to trauma or surgery; those conditions would be coded differently.
• Consider sequencing with codes for associated anomalies, such as uterine agenesis (Q50.6) or renal anomalies, if present.
• Ensure the documentation specifies "congenital" absence to differentiate from other causes of vaginal agenesis or atresia.