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ICD-10 Coding for Noonan Syndrome(Q87.19)

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

Also known as:

Noonan's SyndromeNoonan's Disease

Related ICD-10 Code Ranges

Complete code families applicable to Noonan Syndrome

Q87.1-Q87.9Primary Range

Congenital malformation syndromes predominantly associated with short stature

This range includes Noonan syndrome and related congenital malformation syndromes.

Key Information: ICD-10 code for Noonan syndrome

Essential facts and insights about Noonan Syndrome

The ICD-10 code for Noonan syndrome is Q87.19, used for congenital malformation syndromes with short stature.

Primary ICD-10-CM Code for noonan syndrome

Other congenital malformation syndromes predominantly associated with short stature
Billable Code

Decision Criteria

clinical Criteria

  • Presence of characteristic facial features and congenital heart defects

coding Criteria

  • Genetic confirmation of a mutation in the Ras/MAPK pathway

documentation Criteria

  • Detailed documentation of clinical features and genetic test results

Applicable To

  • Noonan syndrome

Excludes

Clinical Validation Requirements

  • Confirmed genetic mutation (e.g., PTPN11, SOS1, KRAS)
  • Characteristic facial features
  • Congenital heart defects

Code-Specific Risks

  • Misclassification if genetic testing is not documented
  • Omission of associated manifestation codes

Coding Notes

  • Ensure genetic testing results are documented to support the diagnosis of Noonan syndrome.

Ancillary Codes

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

Hypertrophic cardiomyopathy

I42.2
Use when hypertrophic cardiomyopathy is present in a patient with Noonan syndrome.

Pulmonary valve stenosis

Q22.1
Use when pulmonary valve stenosis is diagnosed in a patient with Noonan syndrome.

Short stature

R62.50
Use to document short stature in a patient with Noonan syndrome.

Differential Codes

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

Prader-Willi syndrome

Q87.11
Presence of hyperphagia and obesity, not typical in Noonan syndrome.

Sotos syndrome

Q87.3
Characterized by overgrowth and advanced bone age, unlike Noonan syndrome.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Noonan Syndrome to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code Q87.19.

Impact

Clinical: Misdiagnosis or delayed diagnosis due to lack of specificity., Regulatory: Increased risk of audits and coding queries., Financial: Potential denial of claims due to insufficient documentation.

Mitigation Strategy

Use specific terms for facial features and genetic findings, Ensure all clinical manifestations are documented

Impact

Reimbursement: Incorrect DRG assignment leading to potential underpayment., Compliance: Non-compliance with coding guidelines., Data Quality: Inaccurate clinical data representation.

Mitigation Strategy

Ensure documentation specifies Noonan syndrome to use Q87.19.

Impact

Reimbursement: Potential loss of additional reimbursement for complex cases., Compliance: Failure to capture the full clinical picture., Data Quality: Incomplete data affecting patient care and research.

Mitigation Strategy

Always code associated conditions like cardiomyopathy or short stature.

Impact

Lack of documentation for genetic testing when coding Noonan syndrome.

Mitigation Strategy

Ensure all genetic test results are included in the patient's record.

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 Noonan Syndrome, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Noonan Syndrome

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

Patient with confirmed Noonan syndrome and cardiac manifestations

Specialty: Cardiology

Required Elements

  • Characteristic facial features
  • Genetic test results
  • Echocardiogram findings
  • Growth chart data

Example Documentation

Patient exhibits classic Noonan facies, including ptosis and low-set ears. Echocardiogram reveals hypertrophic cardiomyopathy. Genetic testing confirms SOS1 mutation. Growth chart: height <3rd percentile.

Examples: Poor vs. Good Documentation

Poor Documentation Example
Patient has Noonan syndrome.
Good Documentation Example
Diagnosis: Noonan syndrome (PTPN11-positive). Findings: pulmonic stenosis (peak gradient 40mmHg), delayed puberty (Tanner stage 2 at age 14), and platelet aggregation defect.
Explanation
The good example provides specific genetic and clinical details, supporting accurate coding and billing.

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

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