2026 ICD-10-CM Diagnosis Code H35.50Unspecified hereditary retinal dystrophy

ICD-10-CM Codes›H00–H59›H30-H36›H35

ICD-10-CM H35.50
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

H35.50 is a billable ICD-10-CM diagnosis code for unspecified hereditary retinal dystrophy. 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
H35.50
Billable Status
Yes — Valid for Submission
Code Describes
Unspecified hereditary retinal dystrophy
Short Description
Unspecified hereditary retinal dystrophy
Same as the full description in the CMS dataset.
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 CodeH35.50Unspecified hereditary retinal dystrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Aland Islands eye disease
  • Aphonia, deafness, retinal dystrophy, bifid halluces, intellectual disability syndrome
  • Ataxia with tapetoretinal degeneration syndrome
  • Autosomal dominant late-onset retinal degeneration
  • Autosomal recessive bestrophinopathy
  • Benign concentric annular macular dystrophy
  • Bothnia retinal dystrophy
  • Central obesity
  • Choreoathetosis
  • Cleft lip retinopathy syndrome
  • Cone dystrophy
  • Cone dystrophy with supernormal rod response
  • Congenital anomaly of macula
  • Congenital cerebellar hypoplasia co-occurrent with tapetoretinal degeneration
  • Congenital coloboma of bilateral irides
  • Congenital coloboma of iris
  • Congenital coloboma of iris of left eye
  • Congenital coloboma of iris of right eye
  • Congenital hypoplasia of external genitalia
  • Congenital hypoplasia of male external genitalia
  • Congenital hypoplasia of penis
  • Congenital hypotrichia
  • Diplegia
  • Ectodermal dysplasia with ectrodactyly and macular dystrophy syndrome
  • Encephalopathy, intracerebral calcification, retinal degeneration syndrome
  • Familial benign flecked retina
  • Familial progressive retinal dystrophy, iris coloboma, congenital cataract syndrome
  • Fundus albipunctatus
  • Hereditary retinal dystrophies in lipidoses
  • Hereditary retinal dystrophy
  • Hereditary vasopressin-related polyuria
  • Hypotrichosis with juvenile macular degeneration syndrome
  • Immuno-osseous dysplasia
  • Infantile cerebellar and retinal degeneration
  • Kandori fleck retina syndrome
  • Leber's amaurosis
  • Microcornea, rod-cone dystrophy, cataract, posterior staphyloma syndrome
  • MORM syndrome
  • Nanophthalmia
  • North Carolina macular dystrophy
  • Occult macular dystrophy
  • Oligocone trichromacy
  • Optic atrophy associated with retinal dystrophy
  • Paresis of left lower limb
  • Paresis of right lower limb
  • Progressive chorea
  • Progressive retinal dystrophy due to retinol transport defect
  • Retinal degeneration, nanophthalmos, glaucoma syndrome
  • Retinal dystrophy
  • Retinal dystrophy in cerebroretinal lipidosis
  • Retinal dystrophy with inner retinal dysfunction and ganglion cell anomalies
  • Retinal flecking
  • Retinitis punctata albescens
  • Retinohepatoendocrinologic syndrome
  • Rod dystrophy
  • Roifman syndrome
  • Severe early childhood onset retinal dystrophy
  • Severe oculo-renal-cerebellar syndrome
  • Short stature, skeletal dysplasia, retinal degeneration, intellectual disability, sensorineural hearing loss syndrome
  • Spastic diplegia
  • Spastic paralysis
  • Spastic tetraparesis
  • Spondyloepiphyseal dysplasia, sensorineural hearing loss, intellectual disability, Leber congenital amaurosis syndrome
  • Stargardt's disease
  • Tetraparesis
  • Vasopressin deficiency
  • Weakness of bilateral lower limb
  • Weakness of left lower limb
  • Weakness of left upper limb
  • Weakness of right lower limb
  • Weakness of right upper limb
  • X-linked intellectual disability, limb spasticity, retinal dystrophy, arginine vasopressin deficiency

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.

    • Amaurosis(acquired) (congenital)
      • Leber's congenital
    • Best's disease
    • Dystrophy, dystrophia
      • retinal (hereditary)
    • Leber's
      • congenital amaurosis

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

  • Cone Dystrophy

    a general term which describes a group of rare eye disorders that affect the cone cells of the retina. cone dystrophy can cause a variety of symptoms including decreased visual clarity or acuity when looking straight ahead (central vision), a reduced ability to see colors, and an increased sensitivity to light (photophobia).

  • Hereditary Retinal Dystrophy

    an inherited form of retinal dystrophy.

  • Bietti Crystalline Corneoretinal Dystrophy|BCD

    an autosomal recessive condition caused by mutation (s) in the cyp4v2 gene, encoding cytochrome p450 4v2. it is characterized by multiple glistening intraretinal crystalline deposits scattered throughout the posterior pole associated with progressive atrophy of the retinal pigment epithelium and choroidal sclerosis. the crystalline deposits are also present in the corneal limbus in some individuals.

  • Retinal Dystrophy

    chronic and progressive degeneration of the retina of the eye.

  • Retinal Dystrophy with Inner Retinal Dysfunction and Ganglion Cell Abnormalities|RDGCA

    an autosomal dominant condition caused by mutation(s) in the itm2b gene, encoding integral membrane protein 2b. it is characterized by progressive loss of central vision, and inner retinal dystrophy with ganglion cell abnormalities.

  • Retinal Dystrophy with or without Extraocular Anomalies|RDEOA

    an autosomal recessive condition caused by mutation(s) in the rcbtb1 gene, encoding rcc1 and btb domain-containing protein 1. it is characterized by severe retinal dystrophy. associated extraocular abnormalities may or may not be present.

  • Brachial Amyotrophic Diplegia|BAD|FAS|Flail Arm Syndrome|MIBS|Man-in-barrel Syndrome

    a neurodegenerative condition characterized by asymmetric weakness in the upper extremities resulting from segmental lower motor neuron dysfunction.

  • Diplegia

    paralysis affecting corresponding parts on both sides of the body.

  • Diplegia of Upper Limbs|Diplegia of upper limbs

    evidence of diplegia of the upper limbs.

  • Neurodevelopmental Disorder with Spastic Diplegia and Visual Defects|MRD19|Mental Retardation, Autosomal Dominant 19|NEDSDV

    an autosomal dominant condition caused by mutation(s) in the ctnnb1 gene, encoding catenin beta-1. it is characterized by severe intellectual disability, progressive spastic diplegia, visual impairment, and dysmorphic craniofacial features.

  • Quadriplegia|Bilateral Diplegia|Bilateral Diplegia|Quadriplegia, unspecified|Tetraplegia

    paralysis of all four limbs.

  • Spastic Diplegia|Little's Disease|Spastic diplegic cerebral palsy

    a type of cerebral palsy characterized by spasticity and hypertonia of the lower extremities bilaterally, particularly the legs, hips, and pelvis; this is the most common (70%) form of cerebral palsy.

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 H35.50 to ICD-9-CMHistory

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

ICD-9-CM
362.70 Hered retin dystrphy NOS
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 H35.50Overview

Is H35.50 (Hereditary retinal dystrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report unspecified hereditary retinal dystrophy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does H35.50 group to?

When unspecified hereditary retinal dystrophy 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 H35.50?

Under the General Equivalence Mappings, unspecified hereditary retinal dystrophy converts to ICD-9-CM 362.70 (hered retin dystrphy NOS). 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.