MCMedicalCodingSoftware
E78.71

E78.71Barth syndrome

Billable / specific codeICD-10-CM

E78.71 is a billable, specific ICD-10-CM code for barth syndrome. It is valid for submission on a claim.

Excludes1 inherited from the categories above E78.71

These are not written on E78.71, but they bind it: a note on a category applies to every code beneath it. Billing E78.71 with anything listed here is a denial.

Written on E78.7 Disorders of bile acid and cholesterol metabolism

  • Niemann-Pick disease type C (E75.242)

Written on E78 Disorders of lipoprotein metabolism and other lipidemias

Risk adjustment

E78.71 does not map to an HCC and does not risk-adjust under CMS-HCC V28. That is normal — most ICD-10 codes do not. It still needs to be coded correctly; it just will not move a RAF score.

Medicare coverage

E78.71 is named in 3 Medicare coverage policies 3 listing it as supporting medical necessity. Which of them applies to you depends on your Medicare contractor, and coverage genuinely differs by state.

  • A53060Billing and Coding: Ophthalmology: Extended Ophthalmoscopy and Fundus Photography
  • A56726Billing and Coding: Ophthalmology: Posterior Segment Imaging (Extended Ophthalmoscopy and Fundus Photography)
  • A57071Billing and Coding: Ophthalmology: Posterior Segment Imaging (Extended Ophthalmoscopy and Fundus Photography)

How E78.71 is indexed

Coders do not find codes by browsing the tabular list — they look them up in the alphabetic index, under the word the physician wrote. These are the index entries that lead here, so you can see which chart wordings map to E78.71.

  • Barth syndrome
  • SyndromeBarth
  • Disorderbile acid and cholesterol metabolismBarth syndrome
  • Disordercholesterol and bile acid metabolismBarth syndrome

Code history

E78.71 has not changed since FY2024 — no addition, revision or deletion across the three most recent ICD-10-CM releases.

Related codes at this level

Codes that share E78.7. If E78.71 is not quite right, the correct code is usually one of these.

Questions about E78.71

Is E78.71 a billable ICD-10-CM code?
Yes. E78.71 is a billable, specific ICD-10-CM code and is valid for submission on a claim.
Where does E78.71 sit in the tabular list?
Endocrine, nutritional and metabolic diseases (E00-E89), in the block Metabolic disorders (E70-E88).
What cannot be coded together with E78.71?
E78.71 carries Excludes1 notes, which means the conditions they list can never be coded together with it on the same encounter. Some are written on the categories above E78.71 (E78.7, E78) and apply to it just the same.
ICD-10-CMFY2026-Apr· effective April 1, 2026

Loaded directly from the CMS/NCHS ICD-10-CM public-domain release — see data sources and our editorial policy. If this page disagrees with the CMS tabular list, this page is wrong. Reference information for professional coders; not medical or billing advice.