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E78.71

E78.71: Barth 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.

Check E78.71 with the rest of the claim: free scrubber

Excludes1 inherited from the categories above E78.71

A category’s note binds every code beneath it.

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

No CMS-HCC V28 category: this code does not change a RAF score.

Medicare coverage

E78.71 is named in 3 Medicare coverage policies: 3 listing it as supporting medical necessity. Which one applies depends on your Medicare contractor.

  • 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)

Alphabetic index entries that lead here

  • Barth syndrome
  • Syndrome›Barth
  • Disorder›bile acid and cholesterol metabolism›Barth syndrome
  • Disorder›cholesterol and bile acid metabolism›Barth syndrome

Code history

Unchanged in every release from FY2024 to FY2027.

Other codes under E78.7

ICD-10-CMFY2027· effective October 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.