Q99.1 46, XX true hermaphrodite

The ICD-10-CM code for 46, XX true hermaphrodite is Q99.1 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

17: Congenital malformations, deformations and chromosomal abnormalities (Q00-QA0)

Section

Q90-Q99: Chromosomal abnormalities, not elsewhere classified (Q90-Q99)

Category Q99

13 codes (10 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to Q99.1:

• Swyer syndrome
• Hermaphrodite, hermaphroditism (true) › 46,XX with streak gonads
• Syndrome › Swyer
• Hermaphrodite, hermaphroditism (true) › 46,XY with streak gonads
• Karyotype › 46,XY › hermaphrodite (true)
• Karyotype › 46,XX › hermaphrodite (true)
• Dysgenesis › gonadal (due to chromosomal anomaly) › pure
• Anomaly, anomalous (congenital) (unspecified type) › chromosomes, chromosomal › sex › gonadal dysgenesis (pure)

Inclusion Terms

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

U.S. Hospital Utilization

• An estimated 110 U.S. inpatient stays in 2023 included Q99.1 among the documented diagnoses.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

POA indicator for congenital conditions/anomalies and Q00-Q99 exception

Congenital conditions and anomalies Assign “Y” for congenital conditions and anomalies except for categories Q00- Q99, Congenital anomalies, which are on the exempt list. Congenital conditions are always considered present on admission.

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)