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v1.0.0
ICD-10 Guide
ICD-10 CodesQ56.4

Q56.4

Billable

Indeterminate sex, unspecified

BILLABLE STATUSYes
IMPLEMENTATION DATEOctober 1, 2015
LAST UPDATED09/11/2025

Code Description

ICD-10 Q56.4 is a billable code used to indicate a diagnosis of indeterminate sex, unspecified.

Key Diagnostic Point:

Indeterminate sex, unspecified (ICD-10 code Q56.4) refers to a condition where the sex of an individual cannot be clearly determined due to congenital malformations of the genital organs. This may arise from various conditions such as ambiguous genitalia, where external genitalia do not clearly indicate male or female characteristics, or from disorders of sexual development (DSDs) that can result from chromosomal abnormalities, hormonal imbalances, or anatomical anomalies. Common congenital malformations associated with this code include hypospadias, where the urethral opening is located on the underside of the penis, and cryptorchidism, where one or both testes fail to descend into the scrotum. Uterine malformations, such as agenesis or duplication, can also contribute to indeterminate sex presentations. Accurate coding requires a thorough understanding of the underlying conditions and their implications for the patient's development and treatment options.

Code Complexity Analysis

Complexity Rating: Medium

Medium Complexity

Complexity Factors

  • Variability in clinical presentation of genital malformations
  • Need for detailed documentation of physical examination findings
  • Potential for multiple underlying genetic or hormonal causes
  • Differentiation from other congenital conditions with similar presentations

Audit Risk Factors

  • Inadequate documentation of clinical findings
  • Failure to specify associated conditions or anomalies
  • Misclassification of ambiguous genitalia as indeterminate sex
  • Lack of genetic testing results in the medical record

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Pediatric documentation must include detailed physical examination findings, growth and development assessments, and any interventions or referrals made.

Common Clinical Scenarios

Common scenarios include newborns presenting with ambiguous genitalia, children with hypospadias requiring surgical intervention, and cases of cryptorchidism needing monitoring or surgical correction.

Billing Considerations

Pediatric coders should be aware of the developmental implications of these conditions and ensure that all relevant clinical details are captured to support the diagnosis.

Genetics

Documentation Requirements

Genetic documentation should include family history, results of genetic testing, and any counseling provided regarding the implications of chromosomal abnormalities.

Common Clinical Scenarios

Genetic counseling scenarios often involve discussing the risks of DSDs, interpreting genetic test results, and planning for future pregnancies.

Billing Considerations

Genetic coders must ensure that all relevant genetic syndromes and chromosomal abnormalities are documented to support accurate coding.

Coding Guidelines

Inclusion Criteria

Use Q56.4 When
  • Official coding guidelines for congenital and chromosomal conditions emphasize the importance of specificity in documentation, the need for comprehensive clinical evaluations, and the requirement to capture all relevant associated conditions to ensure accurate coding

Exclusion Criteria

Do NOT use Q56.4 When
No specific exclusions found.

Related ICD-10 Codes

Related CPT Codes

54660CPT Code

Hypospadias repair

Clinical Scenario

Used when a child with hypospadias is undergoing surgical correction.

Documentation Requirements

Document the specific type of hypospadias and any pre-operative assessments.

Specialty Considerations

Pediatric urologists should ensure that all relevant findings are documented to support the procedure.

54640CPT Code

Orchidopexy

Clinical Scenario

Used for surgical correction of cryptorchidism.

Documentation Requirements

Document the location of the undescended testis and any imaging studies performed.

Specialty Considerations

Urologists should provide detailed operative reports to support the procedure.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of congenital conditions, improving the ability to capture the complexity of cases involving indeterminate sex. This specificity aids in better understanding the prevalence and management of these conditions.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of congenital conditions, improving the ability to capture the complexity of cases involving indeterminate sex. This specificity aids in better understanding the prevalence and management of these conditions.

Reimbursement & Billing Impact

The transition to ICD-10 has allowed for more specific coding of congenital conditions, improving the ability to capture the complexity of cases involving indeterminate sex. This specificity aids in better understanding the prevalence and management of these conditions.

Resources

Clinical References

  • •
    American Academy of Pediatrics - Guidelines for the Evaluation and Management of Ambiguous Genitalia
  • •
    Genetics Home Reference - Disorders of Sex Development

Coding & Billing References

  • •
    American Academy of Pediatrics - Guidelines for the Evaluation and Management of Ambiguous Genitalia
  • •
    Genetics Home Reference - Disorders of Sex Development

Frequently Asked Questions

What should I document to support the use of Q56.4?

To support the use of Q56.4, document the clinical findings that led to the diagnosis of indeterminate sex, including physical examination results, any imaging studies, and genetic testing outcomes. Ensure that all associated conditions are also documented to provide a comprehensive clinical picture.

How do I differentiate between Q56.4 and Q56.0?

Q56.4 is used when the sex is indeterminate and cannot be classified as male or female, while Q56.0 is specifically for cases of ambiguous genitalia where the external genitalia are not clearly male or female. Accurate documentation of clinical findings is essential for differentiation.