H35.53 Other dystrophies primarily involving the sensory retina

ICD-10-CM H35.53: Other dystrophies primarily involving the sensory retina

ICD-10-CM code H35.53 designates other dystrophies primarily involving the sensory retina, a category that includes Stargardt disease and cone-rod dystrophies. This code is a single billable entity without a laterality character. The underlying conditions are inherited retinal dystrophies, which are heterogeneous genetic disorders causing the degeneration of retinal photoreceptors. 5 2

Plain-language overview

Inherited retinal dystrophies are a heterogeneous group of genetic disorders caused by gene mutations that primarily affect retinal photoreceptors. These mutations alter the biological mechanism that converts light into bioelectrical signals, disrupting transmission along the visual pathways to the brain. 2

What this code represents

ICD-10-CM code H35.53 represents other dystrophies primarily involving the sensory retina. This diagnostic category includes specific entities such as Stargardt disease and cone-rod dystrophies. 5

Coding details

Code H35.53 is a single billable code without a laterality axis. This means the code lacks a character to specify right eye, left eye, or bilateral involvement. 5

The code H35.53 falls under the H35.5 category for hereditary retinal dystrophy. It is distinct from H35.54, which designates dystrophies primarily involving the retinal pigment epithelium. 6 4

Clinical context

Retinal dystrophies are degenerative disorders of the retina with clinical and genetic heterogeneity. Presentations include dimness of vision, color blindness, night blindness, peripheral vision abnormalities, and progression to complete blindness. 1

Retinal dystrophies can be subdivided into rod-dominated diseases, cone-dominated diseases, and generalized forms involving both rods and cones. Macular diseases within this group include autosomal recessive Stargardt disease, pattern dystrophy, and Best disease. 1

Documentation considerations

Clinical evaluation of retinal dystrophies includes obtaining a primary clinical history and utilizing fundus autofluorescence imaging to assess the health of the retinal pigment epithelium. Degenerative changes noted in these dystrophies include outer retinal tubulations and inner retinal pseudocysts. 1

What the sources add

Clinical reference materials from the American Academy of Ophthalmology and the Centers for Medicare and Medicaid Services both list H35.53 alongside H35.54. The CMS source references H35.53 within a billing and coding article for scanning computerized ophthalmic diagnostic imaging. 4 3

Coverage and utilization context

The Centers for Medicare and Medicaid Services article references H35.53 within the context of billing and coding for scanning computerized ophthalmic diagnostic imaging. This reference establishes the code's appearance in payer operational documents. 3

Sources

1. Bookshelf — ncbi.nlm.nih.gov; accessed 2026-07-25.
2. State of the Art on Inherited Retinal Dystrophies: Management and Molecular Genetics — pmc.ncbi.nlm.nih.gov; accessed 2026-07-25.
3. Article - Billing and Coding: Scanning Computerized Ophthalmic Diagnostic Imaging (SCODI) (A57804) — cms.gov; accessed 2026-07-25.
4. ICD-10-CM Retina — aao.org; accessed 2026-07-25.
5. Stargardt Disease and Sensory Retinal Dystrophy — moyae.com; accessed 2026-07-25.
6. ICD-10-CM 2024 | H35 - Other retinal disorders — unboundmedicine.com; accessed 2026-07-25.