Indeterminate sex and pseudohermaphroditism

This code describes congenital conditions where an individual's external genitalia are ambiguous, making it difficult to determine their sex at birth, or where there is a discrepancy between the chromosomal, gonadal, and phenotypic sex. This includes conditions like ovotesticular disorder of sex development (true hermaphroditism) and various forms of pseudohermaphroditism, where gonads are consistent with one sex but external genitalia are ambiguous or resemble the opposite sex.

Use this code for newborns or individuals diagnosed with ambiguous genitalia, disorders of sex development (DSD), or conditions where there is a mismatch between genetic sex and phenotypic appearance. Documentation should clearly indicate findings such as ambiguous external genitalia, hypospadias with undescended testes, clitoromegaly, or other signs of atypical sexual differentiation.

• Do not use this code for simple hypospadias or cryptorchidism without other signs of ambiguous genitalia.
• Consider sequencing this code as the primary diagnosis when the indeterminate sex is the reason for admission or evaluation.
• Ensure documentation specifies the type of pseudohermaphroditism or if it's truly indeterminate sex, as more specific ICD-10 codes will be available.