Q99.1

46, XX true hermaphrodite

Billable / specificPOA exempt

Q99.1 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.

Inclusion terms

Applicable to

  • 46, XX with streak gonads
  • 46, XY with streak gonads
  • Pure gonadal dysgenesis

Includes (from Q99)

  • Note: Codes from this chapter are not for use on maternal records

Excludes2

Not included here — may be reported together when both conditions exist.

  • inborn errors of metabolism (E70-E88) (from Q99)
  • mitochondrial metabolic disorders (E88.4-) (from Q99)

Also known as

  • 46, XX with streak gonads
  • 46, XY with streak gonads
  • Pure gonadal dysgenesis
  • Anomaly, anomalous, chromosomes, chromosomal, sex, gonadal dysgenesis
  • Dysgenesis, gonadal, pure
  • Hermaphrodite, hermaphroditism, 46,XX with streak gonads
  • Hermaphrodite, hermaphroditism, 46,XY with streak gonads
  • Karyotype, 46,XX, hermaphrodite
  • Karyotype, 46,XY, hermaphrodite
  • Swyer syndrome
  • Syndrome, Swyer

Parent code

  • Q99Other chromosome abnormalities, not elsewhere classifiedHeader

Related codes

  • Q99.0Chimera 46, XX/46, XYBillable
  • Q99.2Fragile X chromosomeBillable
  • Q99.8Other specified chromosome abnormalitiesHeader
  • Q99.9Chromosomal abnormality, unspecifiedBillable

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