Chimera 46, XX/46, XY

This code represents true hermaphroditism or ovotesticular disorder of sex development (DSD), specifically when an individual possesses both XX and XY cell lines, resulting in the presence of both ovarian and testicular tissue. This rare genetic condition leads to ambiguous genitalia and variable internal reproductive anatomy.

Assign this code when documentation confirms a diagnosis of 46, XX/46, XY chimera, indicating the presence of both male and female chromosomal cell lines within the same individual. This diagnosis is typically established through karyotyping and histological examination of gonadal tissue.

• Ensure documentation explicitly states "chimera" or "chimerism" with the specific 46, XX/46, XY karyotype.
• Do not confuse with mosaicism, which involves different cell lines arising from a single zygote, whereas chimerism involves cell lines from two or more distinct zygotes.
• Sequence this code as the primary diagnosis when it is the reason for the encounter, followed by codes for associated clinical manifestations or complications.