2026 ICD-10-CM Diagnosis Code H35.54Dystrophies primarily involving the retinal pigment epithelium

ICD-10-CM Codes›H00–H59›H30-H36›H35

ICD-10-CM H35.54
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

H35.54 is a billable ICD-10-CM diagnosis code for dystrophies primarily involving the retinal pigment epithelium. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 124 through 125. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Retinal and vitreous conditions.

Code Identity

ICD-10-CM Code
H35.54
Billable Status
Yes — Valid for Submission
Code Describes
Dystrophies primarily involving the retinal pigment epithelium
Short Description
Dystrophies primarily w the retinal pigment epithelium
Parent Code
H35.5
Hereditary retinal dystrophy

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH30-H36Disorders of choroid and retina
CategoryH35Other retinal disorders
This CodeH35.54Dystrophies primarily involving the retinal pigment epithelium

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Adult vitelliform macular dystrophy
  • Amelogenesis imperfecta co-occurrent with cone rod dystrophy
  • Best vitelliform macular dystrophy
  • Biallelic RPE65 mutation associated retinal dystrophy
  • Fundus pulverulentus
  • Hereditary retinal dystrophy primarily involving retinal pigment epithelium
  • Macular vitelliform deposits
  • Martinique crinkled retinal pigment epitheliopathy
  • Multifocal pattern dystrophy of retinal pigment epithelium simulating fundus flavimaculatus
  • Pattern dystrophy of macula
  • Progressive cone-rod dystrophy
  • Reticular dystrophy of retinal pigment epithelium
  • Retinal pigment epithelial dystrophy
  • Retinal vitelliform deposits
  • Spondylometaphyseal dysplasia with cone-rod dystrophy syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Vitelliform retinal dystrophy

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Dystrophy, dystrophia
      • retinal (hereditary)
        • involving
          • pigment epithelium
    • Dystrophy, dystrophia
      • tapetoretinal

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR EYE005
Retinal and vitreous conditions
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Hereditary Retinal Dystrophy

    an inherited form of retinal dystrophy.

Patient EducationClinical

Retinal Disorders

The retina is a layer of tissue in the back of your eye that senses light and sends images to your brain. In the center of this nerve tissue is the macula. It provides the sharp, central vision needed for reading, driving and seeing fine detail.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert H35.54 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
362.76 Vitelliform dystrophy
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About H35.54Overview

Is H35.54 (Hereditary retinal dystrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report dystrophies primarily involving the retinal pigment epithelium on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does H35.54 group to?

When dystrophies primarily involving the retinal pigment epithelium is the principal diagnosis on an inpatient stay, it groups to MS-DRG 124, 125, with relative weights from 0.7678 to 1.3231 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of H35.54?

Under the General Equivalence Mappings, dystrophies primarily involving the retinal pigment epithelium converts to ICD-9-CM 362.76 (vitelliform dystrophy). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.