2027 ICD-10-CM Diagnosis Code H35.54Dystrophies primarily involving the retinal pigment epithelium
ICD-10-CM Codes›H00–H59›H30-H36›H35
- Billable — Valid for Submission
- Chronic Condition
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 2027 (October 1, 2026 through September 30, 2027) 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
Code Classification
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
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to H35.54: its own notes plus those printed at H35.5, H35, and Chapter 7. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Vitelliform retinal dystrophy
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- certain conditions originating in the perinatal period (P04-P96)
- certain infectious and parasitic diseases (A00-B99)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
- diabetes mellitus related eye conditions (E09.3-, E10.3-, E11.3-, E13.3-)
- endocrine, nutritional and metabolic diseases (E00-E88)
- injury (trauma) of eye and orbit (S05.-)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
- syphilis related eye disorders (A50.01, A50.3-, A51.43, A52.71)
Notes
General instructions on how these codes are used.
- Use an external cause code following the code for the eye condition, if applicable, to identify the cause of the eye condition
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name H35.54, its category, or a range that includes it.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Hereditary Retinal Dystrophy
an inherited form of retinal dystrophy.
Patient EducationClinical
Retinal Disorders
Retinal disorders are conditions that affect the retina, the thin light-sensitive tissue at the back of your eye. The retina senses light and sends signals to your brain so you can see.
The full article covers:
- What are retinal disorders?
- What causes retinal disorders?
- What are common retinal disorders?
- What are the symptoms of retinal disorders?
- How are retinal disorders diagnosed?
- How are retinal disorders treated?
- Can retinal disorders be prevented?
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.
Code HistoryHistory
Questions About H35.54Overview
What is the ICD-10 code for dystrophies primarily involving the retinal pigment epithelium?
The ICD-10-CM code for dystrophies primarily involving the retinal pigment epithelium is H35.54 (sometimes written as H3554). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
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, 2026 through September 30, 2027.
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 125, 124, with relative weights from 0.7699 to 1.2470 depending on complications. Higher weights mean higher Medicare reimbursement.
Can H35.54 be reported with a code from H31.1?
Not as a rule. The Excludes1 note at H35.5, which applies to H35.54, lists "dystrophies primarily involving Bruch's membrane (H31.1-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
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.
