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ICD-10 Coding for Rett Syndrome(F84.2)

Complete ICD-10-CM coding and documentation guide for Rett Syndrome. Includes clinical validation requirements, documentation requirements, and coding pitfalls.

Also known as:

Rett's DisorderCerebroatrophic Hyperammonemia

Related ICD-10 Code Ranges

Complete code families applicable to Rett Syndrome

F80-F89Primary Range

Pervasive and specific developmental disorders

This range includes Rett syndrome as a specific developmental disorder.

Other degenerative diseases of the nervous system

Includes codes for cerebral degenerations that may be used as ancillary codes with Rett syndrome.

Key Information: ICD-10 code for Rett syndrome

Essential facts and insights about Rett Syndrome

The ICD-10 code for Rett syndrome is F84.2, applicable when clinical criteria and genetic confirmation are met.

Primary ICD-10-CM Code for rett syndrome

Rett's syndrome
Billable Code

Decision Criteria

clinical Criteria

  • Presence of MECP2 mutation and clinical symptoms of Rett syndrome

documentation Criteria

  • Detailed regression timeline and stereotypic movements

Applicable To

  • Rett's disorder

Excludes

  • Autistic disorder (F84.0)

Clinical Validation Requirements

  • Regression after 6-18 months of normal development
  • Stereotypic hand movements
  • Loss of acquired purposeful hand skills
  • MECP2 mutation confirmation

Code-Specific Risks

  • Incorrect use without genetic confirmation
  • Failure to document specific regression timeline

Coding Notes

  • Ensure all diagnostic criteria are documented and genetic testing results are included.

Ancillary Codes

Additional codes that should be used in conjunction with the main diagnosis codes when applicable.

Other specified degenerative diseases of nervous system

G31.8
Use when cerebral degeneration is documented alongside Rett syndrome.

Differential Codes

Alternative codes to consider when ruling out similar conditions to the primary diagnosis.

Autistic disorder

F84.0
Autistic disorder is characterized by lack of skill acquisition rather than regression.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Rett Syndrome to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code F84.2.

Impact

Clinical: Misdiagnosis risk due to lack of detailed history., Regulatory: Non-compliance with coding standards., Financial: Potential claim denials due to insufficient documentation.

Mitigation Strategy

Ensure detailed patient history is recorded., Verify genetic test results are included.

Impact

Reimbursement: Incorrect coding may lead to denied claims., Compliance: Non-compliance with coding guidelines., Data Quality: Inaccurate clinical data representation.

Mitigation Strategy

Code R62.50 until molecular testing confirms Rett syndrome.

Impact

Lack of genetic test results in patient records.

Mitigation Strategy

Implement checklist for genetic testing documentation.

Documentation errors, coding pitfalls, and audit risks are interconnected aspects of medical coding and billing. Addressing all three areas helps ensure accurate coding, optimal reimbursement, and regulatory compliance.

Frequently Asked Questions

Common questions about ICD-10 coding for Rett Syndrome, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Rett Syndrome

Use these documentation templates to ensure complete and accurate documentation for Rett Syndrome. These templates include all required elements for proper coding and billing.

Neurology Progress Note

Specialty: Neurology

Required Elements

  • History of regression
  • Current stereotypies
  • Genetic confirmation
  • Supportive findings

Example Documentation

**History of Regression:** Normal development until 14 months. Loss of purposeful hand use and speech. **Current Stereotypies:** Hand-wringing, 45 episodes/hour. **Genetic Confirmation:** MECP2 mutation confirmed.

Examples: Poor vs. Good Documentation

Poor Documentation Example
Child has Rett syndrome with some hand movements.
Good Documentation Example
23mo female with MECP2 exon 4 deletion confirmed by MLPA, regression of 5-word vocabulary at 18mo, persistent hand-wringing stereotypies (45 episodes/hour), and new-onset awake bruxism.
Explanation
The good example provides specific genetic confirmation, detailed regression timeline, and precise description of stereotypic movements.

Need help with ICD-10 coding for Rett Syndrome? Ask your questions below.

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