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ICD-10 Guide
ICD-10 CodesQ56.1

Q56.1

Billable

Male pseudohermaphroditism, not elsewhere classified

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

Code Description

ICD-10 Q56.1 is a billable code used to indicate a diagnosis of male pseudohermaphroditism, not elsewhere classified.

Key Diagnostic Point:

Male pseudohermaphroditism is a condition characterized by the presence of male chromosomes (typically XY) and male gonads (testes) but with ambiguous or female-typical external genitalia. This condition arises due to various factors, including hormonal imbalances during fetal development, which can lead to incomplete virilization of the external genitalia. Common associated congenital malformations 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. Additionally, ambiguous genitalia may present, complicating the clinical picture. Uterine malformations can also be observed in some cases, particularly when there are underlying chromosomal abnormalities. Accurate diagnosis often requires a multidisciplinary approach, including genetic testing and endocrinological evaluation, to determine the underlying cause and guide management. Treatment may involve surgical correction of genital anomalies and hormone therapy to promote the development of secondary sexual characteristics.

Code Complexity Analysis

Complexity Rating: Medium

Medium Complexity

Complexity Factors

  • Variability in clinical presentation and associated conditions
  • Need for comprehensive documentation of physical examination findings
  • Potential for overlapping diagnoses with other congenital conditions
  • Requirement for genetic testing results to support diagnosis

Audit Risk Factors

  • Inadequate documentation of clinical findings
  • Failure to include genetic testing results
  • Misclassification of ambiguous genitalia
  • Incorrect linkage of procedures to diagnosis

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Detailed physical examination notes, including descriptions of genitalia and any associated anomalies.

Common Clinical Scenarios

Evaluation of newborns with ambiguous genitalia, management of hypospadias repair, and follow-up for cryptorchidism.

Billing Considerations

Consideration of psychosocial aspects and parental counseling regarding the condition and its implications.

Genetics

Documentation Requirements

Genetic testing results, family history, and any syndromic associations.

Common Clinical Scenarios

Genetic counseling for families with a history of intersex conditions and evaluation for chromosomal abnormalities.

Billing Considerations

Understanding the genetic basis of the condition and implications for future pregnancies.

Coding Guidelines

Inclusion Criteria

Use Q56.1 When
  • Follow the official ICD
  • CM coding guidelines for congenital conditions, ensuring accurate documentation of the clinical scenario, associated anomalies, and any relevant genetic testing results

Exclusion Criteria

Do NOT use Q56.1 When
No specific exclusions found.

Related ICD-10 Codes

Related CPT Codes

54660CPT Code

Orchidopexy

Clinical Scenario

Surgical correction of undescended testis in a patient with cryptorchidism.

Documentation Requirements

Operative report detailing the procedure and indication.

Specialty Considerations

Pediatric urology may be involved in the surgical management.

54300CPT Code

Hypospadias repair

Clinical Scenario

Surgical intervention for hypospadias in a male patient.

Documentation Requirements

Preoperative assessment and operative report.

Specialty Considerations

Collaboration with pediatric urology for optimal outcomes.

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 nuances of male pseudohermaphroditism and its associated anomalies.

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 nuances of male pseudohermaphroditism and its associated anomalies.

Reimbursement & Billing Impact

The transition to ICD-10 has allowed for more specific coding of congenital conditions, improving the ability to capture the nuances of male pseudohermaphroditism and its associated anomalies.

Resources

Clinical References

  • •
    American Academy of Pediatrics - Disorders of Sex Development

Coding & Billing References

  • •
    American Academy of Pediatrics - Disorders of Sex Development

Frequently Asked Questions

What is the difference between male pseudohermaphroditism and true hermaphroditism?

Male pseudohermaphroditism involves male chromosomes and gonads but ambiguous external genitalia, while true hermaphroditism includes both ovarian and testicular tissue, leading to a different clinical presentation and management approach.