Hermaphroditism, not elsewhere classified

This code signifies the presence of ambiguous or mixed primary and secondary sexual characteristics in an individual, where the gonadal tissue contains both ovarian and testicular elements. It represents a disorder of sex development (DSD) where the internal and external genitalia do not align with a single, clear sex. This condition is congenital and results from abnormalities in chromosomal, gonadal, or anatomical development.

Use this code for patients diagnosed with true hermaphroditism, characterized by the presence of both ovarian and testicular tissue (ovotestes) or a combination of an ovary on one side and a testis on the other. Documentation should clearly state the presence of both types of gonadal tissue, often confirmed through biopsy or imaging, and describe the ambiguous external genitalia. This code is appropriate when a more specific DSD code, such as those for ovotesticular DSD with a specific karyotype, is not available or documented.

• Do not use this code for pseudohermaphroditism, where gonadal sex is clear but external genitalia are ambiguous; instead, use codes specific to male or female pseudohermaphroditism.
• Always review documentation for specific chromosomal abnormalities (e.g., 46,XX, 46,XY, or mosaicism) and use more specific DSD codes if available and supported.
• Consider sequencing this code as a principal diagnosis when it is the primary reason for the encounter, especially for diagnostic workup or initial management of the condition.