ICD-10-CM diagnosis code
E78.72 Smith-Lemli-Opitz syndrome
- Billable
- Yes, a complete code (FY2026)
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Other codes in E78
18 complete codes in this category.
- E78.3Hyperchylomicronemia
- E78.41Elevated Lipoprotein(a)
- E78.49Other hyperlipidemia
- E78.5Hyperlipidemia, unspecified
- E78.6Lipoprotein deficiency
- E78.70Disorder of bile acid and cholesterol metabolism, unspecified
- E78.71Barth syndrome
- E78.72Smith-Lemli-Opitz syndrome
- E78.79Other disorders of bile acid and cholesterol metabolism
- E78.81Lipoid dermatoarthritis
- E78.89Other lipoprotein metabolism disorders
- E78.9Disorder of lipoprotein metabolism, unspecified
Documentation tips
- Make sure the note supports every element of the title, “Smith-Lemli-Opitz syndrome”: each detail in it should be traceable to the history, examination or assessment.
- Code to the highest specificity the documentation supports. If a detail is missing from the note, add it to the note rather than choosing a less specific code by default.
- Check the Tabular List and official guidelines for instructional notes (“code first”, “use additional code”, Excludes1/Excludes2) that apply to E78.
The international WHO ICD-10 also has E78, with its inclusion and exclusion notes; titles and detail can differ from ICD-10-CM.
ICD-10-CM is developed by the National Center for Health Statistics (NCHS), Centers for Disease Control and Prevention, U.S. Department of Health and Human Services, and is based on the WHO ICD-10. Codes and titles are reproduced unchanged from the FY2026 code list (effective 1 October 2025). Their use here does not imply endorsement by CDC, HHS or the U.S. Government. Changes effective on or after 1 October 2026 may not be reflected yet; check the current release at cdc.gov or cms.gov before submitting claims.
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