Agenesis and aplasia of uterus

This code describes the congenital absence or incomplete development of the uterus. It represents a rare Müllerian duct anomaly where the uterus fails to form or is rudimentary. This condition often presents with primary amenorrhea and may be associated with other reproductive tract abnormalities.

Use this code for patients diagnosed with complete absence of the uterus (agenesis) or its partial, underdeveloped formation (aplasia). Documentation should clearly state the congenital nature of the uterine anomaly, typically identified through imaging studies such as MRI or ultrasound, and clinical examination.

• Differentiate from hypoplasia (underdevelopment) of the uterus, which is coded elsewhere.
• Always review documentation for associated anomalies of the cervix, vagina, or fallopian tubes, as these may require additional codes.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially for diagnostic workups or counseling.