What Is Crystalline Retinopathy?

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Crystalline retinopathy encompasses a wide spectrum of conditions characterized by refractile deposits in the retina, which have varied origin and composition. Most patients with crystalline retinopathy do not have pain, but they often report a decrease in visual acuity, night blindness, sensitivity to light, and eyelid twitching. In this article, we'll discuss the symptoms, causes, and treatments for crystalline retinopathy.

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Crystalline Retinopathy Symptoms

Most people affected by crystalline retinopathy do not have pain. Many people may not exhibit any symptoms, and only an eye examination by an eye specialist, such as an optometrist or ophthalmologist, would detect crystals in the retina. 

Symptoms people affected by crystalline retinopathy might notice include:

  • General decrease in vision
  • Night blindness
  • Sensitivity to light (photophobia)
  • Uncontrollable twitching of the eyelid

Causes

Causes of crystalline retinopathy can include:

  • Diseases: People who have chronic retinal detachments or wet age-related macular degeneration are at risk for crystalline retinopathy. Primary hyperoxaluria, a condition in which the body produces too much oxalate which causes crystals to form in the body and tissues, can lead to crystalline retinopathy. Cystinosis is a genetic disorder where the body has an abnormal accumulation of cystine, which forms crystals in the body’s blood vessels and also leads to crystalline retinopathy. Sjögren-Larsson syndrome, causes abnormal metabolism of essential fatty acids, which can lead to accumulation of lipids in the retina.
  • Medications: There are specific medications that can lead to the creation of crystals in the vessels of the retina. Tamoxifen, a medication often used in the treatment of a cancer known as breast adenocarcinoma, can cause crystalline retinopathy. However, this toxic side effect is relatively rare. An older inhaled anesthetic known as methoxyflurane, which is no longer used due to toxic effects on the kidneys, has also been linked to crystalline retinopathy.  
  • Toxic ingestions: Ethylene glycol, an active ingredient in antifreeze solution, ingestion has been linked to crystalline retinopathy. People who crush oral medications, such as methadone, which is used to decrease a person’s dependence on opioids, can develop crystalline retinopathy. The talc and the ethylene glycol create crystals in the blood vessels that block proper blood flow in the smaller blood vessels of the retina.
  • Embolism: Cardiovascular disease is a risk factor for crystalline retinopathy People who have cardiovascular disease often have cholesterol or lipid deposits built up within the body’s blood vessels. A lipid deposit can break off and travel through the blood vessels. If the lipid deposit lodges in the blood vessels of the retina, it can cause crystalline retinopathy.
  • Iatrogenic: For a person with a retinal detachment, repair of the retina is important to preserving vision. One of the potential complications of retinal detachment repair is development of crystalline retinopathy from the products used to reattach and secure the retina.

Recap

Many conditions have been linked to the development of crystalline retinopathy. These conditions can cause the formation of crystals or lipid molecules within the blood vessels of the retina. Certain medications with the side effect of forming crystals in the blood vessels, toxic ingestions that create crystals in the blood vessels, and side effects of certain medical procedures can all lead to crystalline retinopathy.

Diagnosis

An eye specialist, such as an optometrist or ophthalmologist, can perform several procedures to identify and diagnose crystalline retinopathy, including:

  • Fundus autofluorescence: Fundus autofluorescence imaging is used to record fluorescence (emission of light by a substance that has absorbed light or other electromagnetic radiation) that may occur naturally in the structures of your eyes or as a result of a disease process. Fundus autofluorescence imaging can provide information about the health and function of the retina.
  • Spectral domain optical coherence tomography: This test can determine if there is loss of the outer retinal layer.
  • Fluorescein angiography: This is used to rule out other active leakage or abnormal blood vessel growth that can lead to blood leakage.
  • Visual field testing: Assessment of visual fields may identify central or ringlike blind spots.

Additionally, lab tests may be ordered to determine if there is an underlying medical condition, such as a genetic disorder or cardiovascular disease, that is causing the crystalline retinopathy.

Getting annual eye exams is the best way to identify crystalline retinopathy early and potentially halt and reverse damage to the retina.

Treatment

Treatment for crystalline retinopathy is to remove the causative drug or agent, if possible, such as the toxic ingestion or medication inducing the condition. For underlying genetic disorders or conditions that cannot be reversed, symptomatic treatment may be indicated. 

Ensuring a person affected by hyperoxaluria has plenty of fluids and adding oral medication like potassium citrate to prevent crystal buildup may be appropriate. Other conditions, such as advanced cystinosis, may require dialysis and renal transplantation due to kidney damage caused by the inherited disease. 

For cardiovascular disease, lowering your intake of dietary fats and eating more fruits and vegetables can be effective. For Sjögren-Larsson syndrome, restricting fats and adding in supplements can help metabolize the fatty acids.

Whenever possible, eliminating the cause of crystalline retinopathy is the best treatment. For other conditions, neutralizing the development of the crystals or lipid molecules with medications or other interventions, like dialysis, may be indicated.

Prognosis

Depending on the causative agent, prognosis is favorable. If a medication is causing crystalline retinopathy, the condition of the retina can improve once the medication is stopped. In other cases, such as toxic ingestions, changes to the retina may be irreversible and permanent.

Summary

Crystalline retinopathy is a retinal complication as a result of an underlying condition, certain medication use, toxic ingestions, blockage of retinal vessels by lipids, or a side effect of a treatment or procedure. A person who has any of the conditions that could lead to crystalline retinopathy should see an eye specialist, like an optometrist or ophthalmologist, routinely. This can help them identify crystalline retinopathy before irreversible damage occurs and preserve vision.

A Word From Verywell

Crystalline retinopathy is a result of many different medical conditions, and people affected may not have any symptoms while retina damage is occurring. Being aware of the various underlying medical conditions that can cause crystalline retinopathy, as well as getting regular exams from an eye specialist, can identify crystalline retinopathy early. The earlier crystalline retinopathy is identified, the sooner treatment can start and the better the prognosis.

Frequently Asked Questions

  • How can so many different things cause crystalline retinopathy?

    Crystalline retinopathy can be the result of different medical conditions, medications, ingestions of toxins, and embolic and iatrogenic causes. Some conditions lead to a formation of crystals in the body’s blood vessels, while others lead to the accumulation of excessive lipid molecules in the body’s blood vessels. 
    In both cases, the crystals or lipid molecules block the blood vessels and prevent the delivery of nutrient-rich blood and oxygen to the cells of the retina. Ultimately, whether the blockage is due to crystals or lipids, the retina cannot receive the nutrients and oxygen it needs, and crystalline retinopathy can occur. 

  • How common is crystalline retinopathy?

    The majority of literature available on crystalline retinopathy indicates that it's relatively rare. Tamoxifen has several side effects, of which crystalline retinopathy is one of the rarer ones, at 1.5%–11.5%. Sjögren-Larsson syndrome is rare and estimated to affect fewer than 0.4 people per 100,000. Crystalline retinopathy is a late-stage finding in people with Sjögren-Larsson syndrome.

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3 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. American Academy of Ophthalmology. Crystalline retinopathy. Updated August 21, 2021.

  2. Kim HA, Lee S, Eah KS, Youn YH. Prevalence and risk factors of tamoxifen retinopathy. J Ophtha. 2019;127(4):555-557. doi:10.1016/j.ophtha.2019.10.038

  3. Nanda T, Kovach JL. Ophthalmic findings in late stage sjogren-larrson syndromeRetin Cases Brief Rep. 2019;13(3):251-254. doi:10.1097/ICB.0000000000000583