Congenital absence of ovary, bilateral

This code signifies the complete congenital absence of both ovaries, a rare developmental anomaly. This condition results in primary amenorrhea, infertility, and lack of secondary sexual characteristics due to the absence of estrogen production. It is distinct from acquired ovarian absence or unilateral agenesis.

This code is appropriate for documenting cases of bilateral ovarian agenesis identified at birth or during subsequent evaluation for primary amenorrhea or infertility. Documentation should clearly state the congenital and bilateral nature of the ovarian absence, often supported by imaging studies (e.g., ultrasound, MRI) and genetic testing.

• Ensure documentation explicitly states "congenital" and "bilateral" absence of ovaries.
• Do not confuse with acquired absence of ovaries (e.g., due to oophorectomy) or unilateral agenesis.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially in cases of primary amenorrhea or infertility workup.