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ICD-10 Coding for Congenital Adrenal Hyperplasia(E25.0)

Complete ICD-10-CM coding and documentation guide for Congenital Adrenal Hyperplasia. Includes clinical validation requirements, documentation requirements, and coding pitfalls.

Also known as:

CAHAdrenogenital Syndrome

Related ICD-10 Code Ranges

Complete code families applicable to Congenital Adrenal Hyperplasia

E25.0-E25.9Primary Range

Congenital adrenogenital disorders

This range includes disorders related to enzyme deficiencies in adrenal steroidogenesis, primarily including CAH.

Congenital malformations of adrenal gland

Used for structural adrenal malformations, not enzyme deficiencies.

Key Information: ICD-10 code for congenital adrenal hyperplasia

Essential facts and insights about Congenital Adrenal Hyperplasia

The ICD-10 code for congenital adrenal hyperplasia due to 21-hydroxylase deficiency is E25.0.

Primary ICD-10-CM Code for congenital adrenal hyperplasia

Congenital adrenogenital disorders associated with enzyme deficiency
Billable Code

Decision Criteria

clinical Criteria

  • Presence of 21-hydroxylase deficiency confirmed by lab tests

documentation Criteria

  • Detailed documentation of enzyme deficiency and genetic testing

Applicable To

  • 21-hydroxylase deficiency

Excludes

Clinical Validation Requirements

  • Elevated 17-hydroxyprogesterone (17-OHP) levels via LC-MS/MS
  • CYP21A2 mutation analysis

Code-Specific Risks

  • Incorrectly coding structural malformations as enzyme deficiencies

Coding Notes

  • Ensure enzyme deficiency is documented before using E25.0.

Ancillary Codes

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

Addisonian crisis

E27.2
Use for acute adrenal insufficiency related to CAH.

Hypokalemia

E87.6
Use for electrolyte imbalances in salt-wasting CAH.

Differential Codes

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

Other adrenogenital disorders

E25.8
Use when enzyme deficiency is unspecified.

Primary adrenocortical insufficiency

E27.1
Use for non-CAH adrenal failure.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Congenital Adrenal Hyperplasia to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code E25.0.

Impact

Clinical: Misdiagnosis risk, Regulatory: Non-compliance with coding standards, Financial: Potential claim denials

Mitigation Strategy

Use templates with required fields, Regular training on documentation standards

Impact

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

Mitigation Strategy

Confirm enzyme deficiency and use E25.0.

Impact

Lack of specific enzyme deficiency documentation.

Mitigation Strategy

Implement mandatory documentation templates.

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 Congenital Adrenal Hyperplasia, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Congenital Adrenal Hyperplasia

Use these documentation templates to ensure complete and accurate documentation for Congenital Adrenal Hyperplasia. These templates include all required elements for proper coding and billing.

Newborn screening follow-up

Specialty: Pediatrics

Required Elements

  • 17-OHP levels
  • CYP21A2 mutation analysis
  • Electrolyte levels

Example Documentation

Newborn with elevated 17-OHP (12,000 ng/dL via LC-MS/MS) and CYP21A2 mutation. Diagnosis: Classic salt-wasting CAH.

Examples: Poor vs. Good Documentation

Poor Documentation Example
Infant with positive CAH screen.
Good Documentation Example
Newborn female with elevated 17-OHP (12,000 ng/dL via LC-MS/MS) and CYP21A2 homozygous pathogenic variant. Diagnosis: Classic salt-wasting CAH.
Explanation
The good example provides specific lab results and genetic confirmation.

Need help with ICD-10 coding for Congenital Adrenal Hyperplasia? Ask your questions below.

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