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A-Z Glossary

Table of Contents

Maculopathy

Vasudha Rao
Written byVasudha Rao
Vasudha Rao
Vasudha Rao4th Year Optometry Student, UC BerkeleyBerkeley, CA

Bio

Vasudha Rao is a fourth-year optometry student at the UC Berkeley School of Optometry with aspirations of specializing in ocular disease or community care. She is currently on clinical rotations. She enjoyed serving veterans at the Miami Veterans Affairs Hospital and learning from renowned clinicians at the Bascom Palmer Eye Institute as a part of her clinical training. Vasudha is passionate about helping underserved communities receive optometric care, and actively volunteers to provide vision screenings to those in need. In her spare time she enjoys partaking in a game of Scrabble, enhancing her cooking skills, and sharing her clinical cases on her Instagram page @visions.of.vasudha.

  • Last updated December 2, 2022

What is Maculopathy?

Maculopathy is defined as a disease of the macula. There are many different eye diseases that can cause maculopathy. The macula is responsible for detailed, high-resolution central vision and color vision. The macula is used to see fine details when we do activities like reading books, knitting, or recognizing faces and colors. Maculopathy is a degenerative condition of the macula that can cause central vision loss. Colors might also look different if there is maculopathy. Examples of maculopathies are medication-induced toxicity, bull’s eye maculopathy, and cellophane maculopathy.  

Key Takeaways

  • Maculopathy is any condition that damages the macula. The macula is responsible for central vision, color vision, and seeing fine details.
  • Maculopathy has many different causes. It may be caused by certain medications, genetic mutations, or low eye pressure.
  • Symptoms of maculopathy can include blurry central vision, visual distortion, and impaired blue-yellow color perception.
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Understanding Maculopathy

Maculopathy is any condition that causes disease of the macula. The macula is a vital structure for vision, as it enables us to see fine print and color. Maculopathy may cause gradual blurring of the central vision. Maculopathy is a broad diagnosis that does not specify the cause of the macular disease. There are many different types of maculopathies. 

Types of maculopathy include:

Medication induced maculopathy (Chloroquine/ Hydroxychloroquine maculopathy): 

Hydroxychloroquine (Plaquenil) or Chloroquine medications are often prescribed by medical doctors to treat autoimmune issues such as lupus or rheumatoid arthritis. If these medications are used for many years, or in high dosage amounts, they can affect the macula. The drug can be toxic to the macula because it binds to melanin in the macula. Maculopathy is more likely to occur if the medications are used for more than five years. People who take these medications should get an eye exam at the time of starting the medication and annually thereafter.

Bull’s eye maculopathy 

Bull’s eye maculopathy is a general term for any maculopathy that causes a ‘bulls eye’ appearance of the macula. Bull’s eye maculopathy looks like a dark-colored ring surrounding the macula. The appearance is similar to a bullseye at the center of a dartboard. Causes of bull’s eye maculopathy are Stargardt disease, rod-cone dystrophy, and Batten disease. 

Paracentral acute middle maculopathy

Paracentral acute middle maculopathy is caused by lack of blood supply to the retina. It can cause a ‘black spot’ in the vision. 

Cellophane maculopathy

Cellophane maculopathy happens when cells grow on top of the outer layer of the retina. The cells can contract, which makes the macular tissue develop ripples. Cellophane maculopathy makes the vision seem wavy or distorted. 

Hypotony maculopathy

Hypotony maculopathy occurs when the eye pressure is dangerously low. If the eye pressure is too low, the eye’s structures can collapse inwards. Low eye pressure causes wrinkling of the macula and can affect the central vision.

Torpedo maculopathy

Torpedo maculopathy is a rare congenital condition that causes a white spot in the macula. 

Crystalline maculopathy

Crystalline maculopathy is a medication-induced damage of the macula. It is caused by tamoxifen medication, which is used to treat breast cancer. Crystalline maculopathy causes shiny crystals to be deposited in the macula. 

Pigmentary maculopathy

Pigmentary maculopathy is a medication-induced maculopathy. It is caused by Elmiron medication, used to treat bladder pain. It is also caused by antipsychotic medications such as thioridazine and chlorpromazine. Pigmentary maculopathy causes dark-colored deposits of pigment near the macula.

Symptoms

Maculopathy can damage the function of the macula. The macula provides our eyes with clear detail, color vision, and central vision. Symptoms of maculopathy may include: 

  • A dark spot or blind spot in the center of the vision
  • Blurry vision
  • Distorted vision
  • Trouble seeing at night or in the dark
  • Difficulty telling colors apart, especially blue and yellow
  • Difficulty reading
  • Straight lines seem wavy or crooked

Causes And Risk Factors

Maculopathy may be congenital or acquired. Risk factors for developing a maculopathy include:

  • Age over 50 years
  • Obesity
  • Smoking
  • Cardiovascular disease
  • Caucasian
  • Family history of maculopathy 
  • High blood pressure 
  • Diabetes
  • High cholesterol 

Diagnosis

Maculopathy is diagnosed by eye physicians who do tests to look for abnormalities in the macula or retina.

Maculopathy tests include: 

  • Dilated eye examination: The doctor will use drops to dilate your pupils. They will then use a special lens to see inside the back of the eye. The dilation allows the doctor to see the macula clearly. 
  • Amsler grid: Doctors may ask you to look at an Amsler grid full of squares. It is likely that maculopathy is present if there are distorted, wavy lines or any missing spots in the vision.
  • Fluorescein angiography: A yellow dye will be injected into a vein in your arm during this examination. Doctors can observe the presence of any leaks in the macula as the dye enters the eye’s blood vessels.
  • Optical coherence tomography  (OCT): This machine performs a microscopic scan of the retina to analyze each layer of the retina, including the macula.

Treatment

Treatment depends on the cause of maculopathy. If it’s medication-related, stopping the medication may prevent further damage but will not reverse the damage to the macula. The macula cannot regenerate once damaged. Treatment for maculopathy focuses on stopping the disease from progressing and preventing further vision loss.

Bibliography

  1. “What Is Macular Degeneration? – AMDF.” American Macular Degeneration Foundation, www.macular.org/about-macular-degeneration/what-is-macular-degeneration.
  2. What Is Macular Degeneration? “What Is Macular Degeneration?” American Academy of Ophthalmology, 10 May 2019, www.aao.org/eye-health/diseases/amd-macular-degeneration.
  3. National Eye Institute. “Age-Related Macular Degeneration | National Eye Institute.” Nih.gov, 2019, www.nei.nih.gov/learn-about-eye-health/eye-conditions-and-diseases/age-related-macular-degeneration.
  4. “Macular Degeneration | Frequently Asked Questions.” IrisVision, 31 Jan. 2018, irisvision.com/macular-degeneration-frequently-asked-questions/.

Related Terms

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Schlemm’s Canal

is a cylindrical vessel that collects fluid (aqueous) inside the eye and drains it into blood vessels

Oculus Sinister

is the Latin term that translates to “left eye”, often used to denote the left eye’s glasses prescription

Leber Congenital Amaurosis (LCA)

is a congenital retinal dystrophy resulting in nystagmus and significantly reduced vision

Aberrations

are flaws or imperfections in the way that an optical system constructs images

Limbus

is the border between the clear part of the eye (cornea) and the white part of the eye (sclera)

Puncta

play an important role in tear drainage. Puncta can be occluded to treat dry eye disease and may improve symptoms

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