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ICD-10 Coding for Loeys-Dietz Syndrome(Q87.4)

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

Also known as:

LDSLoeys-Dietz

Related ICD-10 Code Ranges

Complete code families applicable to Loeys-Dietz Syndrome

Q87.4Primary Range

Marfan syndrome, Loeys-Dietz syndrome, and other specified congenital malformation syndromes with skeletal involvement

This range includes Loeys-Dietz syndrome as a primary condition, capturing its genetic and clinical manifestations.

Aortic aneurysm and dissection

These codes are used to document specific vascular complications associated with Loeys-Dietz syndrome.

Cleft palate and cleft lip

These codes may be used to document craniofacial anomalies often seen in Loeys-Dietz syndrome.

Key Information: ICD-10 code for Loeys-Dietz syndrome

Essential facts and insights about Loeys-Dietz Syndrome

The ICD-10 code for Loeys-Dietz syndrome is Q87.4, covering Marfan syndrome and other specified congenital malformation syndromes with skeletal involvement.

Primary ICD-10-CM Code for loeys dietz syndrome

Marfan syndrome, Loeys-Dietz syndrome, and other specified congenital malformation syndromes with skeletal involvement
Non-billable Code

Decision Criteria

clinical Criteria

  • Presence of bifid uvula and aortic root dilation

documentation Criteria

  • Genetic test results confirming TGFBR1 or TGFBR2 mutation

Applicable To

  • Loeys-Dietz syndrome

Excludes

Clinical Validation Requirements

  • Genetic testing confirming TGFBR1 or TGFBR2 mutation
  • Clinical diagnosis with major criteria such as aortic aneurysm and craniofacial features

Code-Specific Risks

  • Misclassification with Marfan syndrome
  • Omission of genetic confirmation

Coding Notes

  • Ensure genetic testing results are documented to support the use of Q87.4.

Ancillary Codes

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

Dissection of ascending aorta

I71.01
Use for documenting aortic dissection as a complication of Loeys-Dietz syndrome.

Differential Codes

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

Marfan syndrome

Q87.40
Presence of ectopia lentis and FBN1 mutation differentiates Marfan from Loeys-Dietz.

Ehlers-Danlos syndrome

Q79.6
Absence of COL3A1 mutation and presence of arterial tortuosity differentiates Loeys-Dietz from vascular EDS.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Loeys-Dietz Syndrome to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code Q87.4.

Impact

Clinical: May lead to misdiagnosis or inappropriate treatment., Regulatory: Non-compliance with documentation standards., Financial: Potential for denied claims due to insufficient documentation.

Mitigation Strategy

Use specific terms like 'Loeys-Dietz syndrome', Document genetic test results

Impact

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

Mitigation Strategy

Confirm genetic testing distinguishes TGFBR1/2 from FBN1.

Impact

Lack of genetic test results can lead to audit findings.

Mitigation Strategy

Ensure all genetic testing is documented and linked to the diagnosis.

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 Loeys-Dietz Syndrome, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Loeys-Dietz Syndrome

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

Initial diagnosis of Loeys-Dietz syndrome

Specialty: Genetics

Required Elements

  • Genetic test results
  • Craniofacial features
  • Vascular findings

Example Documentation

Patient presents with hypertelorism, pectus excavatum, and aortic root aneurysm (4.5 cm). Genetic testing confirms TGFBR1 variant.

Examples: Poor vs. Good Documentation

Poor Documentation Example
Aortic dilation noted.
Good Documentation Example
Aortic root aneurysm (4.8 cm, z-score +4.1) with bilateral carotid tortuosity on CTA. TGFB2 variant confirmed via panel testing (CPT 81479).
Explanation
The good example provides specific measurements and genetic confirmation, supporting accurate coding.

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

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