Short answer · Medically reviewed summary · Last updated: 2026-04-07

Stargardt disease is primarily coded under ICD-10-CM code H35.52 (Pigmentary retinal dystrophy), while its historical ICD-9-CM code is 362.75 (Other dystrophies of the retina). These codes are used by healthcare providers and insurance systems to specifically identify this form of inherited macular degeneration. What is the clinical significance of these codes for Stargardt disease? In the medical coding system, Stargardt disease is categorized under retinal dystrophies because it involves the progressive degeneration of the macula, the central part of the retina responsible for sharp vision.

2 people with Stargardt Disease have shared their first-person experience on this question at DiseaseMaps.

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ICD10 code of Stargardt Disease and ICD9 code

ICD-10 and ICD-9 codes for Stargardt Disease, with classification details for clinicians, coders and patients.

ICD9 and ICD10 codes of Stargardt Disease

Stargardt disease is primarily coded under ICD-10-CM code H35.52 (Pigmentary retinal dystrophy), while its historical ICD-9-CM code is 362.75 (Other dystrophies of the retina). These codes are used by healthcare providers and insurance systems to specifically identify this form of inherited macular degeneration.



What is the clinical significance of these codes for Stargardt disease?


In the medical coding system, Stargardt disease is categorized under retinal dystrophies because it involves the progressive degeneration of the macula, the central part of the retina responsible for sharp vision. While the ICD-10 code H35.52 is the most specific identifier used globally, clinicians often document Stargardt disease alongside relevant secondary codes if associated complications, such as significant visual impairment or legal blindness, are present. Proper coding ensures that patients receive appropriate coverage for specialized diagnostic tests, such as electroretinography (ERG) or optical coherence tomography (OCT), which are vital for monitoring the progression of Stargardt disease.



How does the classification of Stargardt disease impact patient care?


Because Stargardt disease is a rare genetic condition, accurate diagnostic coding is essential for tracking patient outcomes and facilitating research. Within the DiseaseMaps community, 284 people with Stargardt disease have connected to share their experiences, highlighting the importance of standardized medical documentation. Precise coding allows your ophthalmologist to communicate effectively with genetic counselors and insurance providers, ensuring that the specific needs of the Stargardt disease community—such as low-vision aids and genetic testing—are recognized by healthcare systems.



What are the diagnostic features typically associated with these codes?


When a physician assigns the ICD-10 code for Stargardt disease, they are typically documenting several hallmark clinical findings discovered during a comprehensive eye exam. These features help differentiate the condition from other forms of macular degeneration:



  • Presence of Flecks: Yellowish-white, lipofuscin-rich deposits (pisciform flecks) appearing in the retinal pigment epithelium.

  • Macular Atrophy: Thinning of the central retina, often described as having a "beaten bronze" appearance.

  • Visual Acuity Decline: Progressive loss of central vision, typically beginning in childhood or adolescence, while peripheral vision usually remains preserved.

  • ABCA4 Mutation: Genetic confirmation via molecular testing, which identifies the underlying cause in the majority of Stargardt disease cases.



Next steps



  • Consult with a retinal specialist or an inherited retinal disease (IRD) expert to ensure your medical records accurately reflect your diagnosis.

  • Request a referral to a certified genetic counselor to discuss the inheritance pattern and implications for family members.

  • Join the DiseaseMaps community to connect with the 284+ members who share experiences regarding care, clinical trials, and adaptive living strategies.

  • Check your insurance portal or speak with your clinic's billing department to confirm that your diagnostic codes are up to date for your current treatment plan.



Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.



References


Author: DiseaseMaps Editorial Team
Reviewed against authoritative medical sources (NIH GARD, Orphanet, OMIM)
Last updated: 2026-04-07
Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
3 answers
ICD-10-CM Code H35.50 - Unspecified hereditary retinal dystrophy

Posted Sep 4, 2017 by Harry Batten 2021
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Posted Apr 4, 2018 by Etem 2500

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