2027 ICD-10-CM Diagnosis Code H35.53Other dystrophies primarily involving the sensory retina

ICD-10-CM Codes›H00–H59›H30-H36›H35

ICD-10-CM H35.53
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

H35.53 is a billable ICD-10-CM diagnosis code for other dystrophies primarily involving the sensory retina. 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

ICD-10-CM Code
H35.53
Billable Status
Yes — Valid for Submission
Code Describes
Other dystrophies primarily involving the sensory retina
Short Description
Other dystrophies primarily involving the sensory retina
Same as the full description in the CMS dataset.
Parent Code
Hereditary retinal dystrophy

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH30-H36Disorders of choroid and retina
CategoryH35Other retinal disorders
This CodeH35.53Other dystrophies primarily involving the sensory retina

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Bilateral degeneration of macula
  • Bilateral fundus flavimaculatus of eyes
  • Cone dystrophy
  • Fundus flavimaculatus
  • Fundus flavimaculatus of left eye
  • Fundus flavimaculatus of right eye
  • Hereditary retinal dystrophy primarily involving sensory retina
  • Hyaline retinal dystrophy
  • Progressive cone dystrophy
  • Progressive rod dystrophy
  • Retinitis pigmentosa-deafness syndrome
  • Retinitis pigmentosa-deafness-ataxia syndrome
  • Rod dystrophy
  • Sensory retinal dystrophy
  • Sorsby pseudoinflammatory fundus dystrophy
  • Usher syndrome type 1
  • Usher syndrome type 1F
  • Usher syndrome type 2

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to H35.53: 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.

  • Stargardt's disease

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From H35.5 Hereditary retinal dystrophy applies to 5 codes
  • dystrophies primarily involving Bruch's membrane (H31.1-)

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.

From H35 Other retinal disorders applies to 175 codes
From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • 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.

From Chapter 7 (H00-H59) Diseases of the eye and adnexa applies to 2,677 codes
  • 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 H35.53, its category, or a range that includes it.

Excludes1 1

These codes carry an Excludes1 note naming H35.53: they are not reported together with it, unless the two conditions are unrelated.

  • H35.4 Peripheral retinal degeneration
    hereditary retinal degeneration (dystrophy) (H35.5-) names H35.5-, which includes this code

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.

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

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 H35.53 to ICD-9-CMHistory

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

ICD-9-CM
362.75 Sensory retina 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 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About H35.53Overview

What is the ICD-10 code for other dystrophies primarily involving the sensory retina?

The ICD-10-CM code for other dystrophies primarily involving the sensory retina is H35.53 (sometimes written as H3553). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is H35.53 (Hereditary retinal dystrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other dystrophies primarily involving the sensory retina on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does H35.53 group to?

When other dystrophies primarily involving the sensory retina 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 H35.53 be reported with a code from H31.1?

Not as a rule. The Excludes1 note at H35.5, which applies to H35.53, 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 H35.53?

Under the General Equivalence Mappings, other dystrophies primarily involving the sensory retina converts to ICD-9-CM 362.75 (sensory retina dystrophy). The mapping is a direct match.