Choroidal Folds

What is Choroidal Folds?

Choroidal folds, a condition first noted by Nettleship in 1884, occur in patients with papilloedema caused by a lesion that takes up space in the eye. These folds look like a series of alternating dark and bright lines beneath the retina, similar to grooves or stripes. They typically line up horizontally but can also be vertical, slanted, circular, or irregular. These folds generally do not go beyond the central area of the eye, known as the equator.

The disease can affect one or both eyes. Over time, these folds usually become wider, smoother, and whiter. Initially, they can be as thin as small blood vessels but gradually become wider and smoother at the edges. When the innermost layer of the eye also develops folds, it’s referred to as a chorioretinal fold.

What Causes Choroidal Folds?

Choroidal folds, which are wrinkles in the layer of blood vessels in the eye, can be caused by various conditions.

Originally, it was thought that the majority of choroidal fold cases happened for unknown reasons. However, recent studies found that a cause can be identified in about 85% of cases with a thorough examination. Although the exact process that causes it is unknown, it may be traced back to inflammation in the back part of the eye’s white area during the fetus’ development or childhood, which later leads to shrinkage of that area.

Next, reduced eye pressure can also cause these folds. And this can happen due to loss of volume in the eye because of loss of lens, clear liquid inside the eye, or jelly-like substance that fills the eye. It can be related to an opening in the eye, after eye surgery, or due to a type of eye injury.

Then, mechanical force on the eye or its layers can lead to choroidal folds. It can happen due to displacement of nearby area in the eye as a result of tumors, or due to pressure on the eye caused by too much tissue pulling on the optic nerve.

Lastly, swelling and buildup of fluid in the eye socket, along with the enlargement of muscles outside of the eye, can cause these folds. This is especially true in Graves’ disease which affects the eyes. Other causes include inflammation of the eye socket, chronic sinusitis and bacterial infections that can cause the underlying white area of the eye to shrink.

In a procedure that involves pulling the white area of the eye, the process not only reduces the eye’s circumference under the buckle but also stretches the blood vessel layer of the eye which causes folds perpendicular to the direction of the buckle. Various mass growths in the eye socket can lead to increased pressure on the eye leading to choroidal folds. Other causes include tumors such as melanoma, nevus and metastasis, certain types of injections, and forced detachment of the blood vessel layer of the eye.

Choroidal folds can also occur due to thickness of the blood vessel layer in eyes that might be short in length, swelling of the optic nerve head, diseases that increase brain fluid, and inflammation of the white area of the eye. Other conditions that could cause it include new blood vessel growth under the retina (choroidal neovascularization), contraction in the area beneath the retina (scar), eye disorder related to space flights, and changes in the long-sightedness with choroidal folds.

Depending on which condition is associated, different factors might be the cause of choroidal folds, including congestion in the blood vessel layer due to low eye pressure or tumors, deformation of the white area of the eye due to optic nerve pulling, prolonged inflammation or traction, or decreased rigidity of the white area of the eye particularly in young myopic people due to the stretched eyeball and thin white area.

Finally, the characteristic lines seen in the case of choroidal folds are thought to be due to congestion along the blood vessel layer of the eye and its connection to an overlying layer. In the absence of these factors, the pigment layer of the retina can slip over the blood vessel layer, potentially preventing fold formation.

Risk Factors and Frequency for Choroidal Folds

We don’t have information on how often choroidal folds occur in the general population.

Signs and Symptoms of Choroidal Folds

Choroidal folds are a condition that can affect patients’ eyes and might show no symptoms or lead to worsened vision. These vision problems are more common in acute cases. It’s usually possible to see choroidal folds in an eye exam with certain types of lenses and filters. These folds can be associated with several other eye conditions and general health issues.

Common examples include:

Idiopathic choroidal folds: This is the most common type, often seen in men who have no symptoms apart from needing glasses for reading. The folds usually appear in both eyes and may indicate several different potential causes.
Acquired hyperopia with choroidal folds: Usually in men, these cases are detected during routine check-ups or when the patient complains of blurry vision. They might also have slight swelling and other eye findings.
Choroidal tumors: In this case, the folds surround the base of the tumor.
Hypotony-related choroidal folds: Usually associated with low eye pressure, these folds are typically broader. Signs also include engorged retinal vessels and, rarely, macular edema.
Nanophthalmos: In this condition, the patient has small, hyperopic eyes deep in the orbit and other features like a retinal fold and macular holes.
Papilledema is usually coupled with symptoms like headaches, vomiting, and sometimes blurred vision. The eyes show signs of papilledema and folds around the optic disc.
Rhegmatogenous retinal detachment: Here, a scleral buckle is usually placed, often leading to folds near the posterior slope of the buckle.
Uveal effusion: This condition is episodic and is usually associated with choroidal detachment and retinal detachment with shifting fluid.
Posterior scleritis: Patients usually experience vision loss in one eye with severe pain and reduced eye movement. The exam often shows a localized elevation in the choroid.
VKH disease: Usually progresses through several stages, including initial discomfort like fever, light sensitivity, the acute stage with vision loss, a recovery stage with hair loss, and finally a chronic phase with cataracts or other serious issues.
SANS: Mostly seen in astronauts with vision problems, disc edema, choroidal folds, and retinal spots after lengthy space travel.
Intra-conal lesions: These patients usually have hyperopia, whereas extra-conal lesions lead to astigmatism. Depending on where the lesion is located, it will impact the fold pattern in the choroid.

Identification of choroidal folds can offer clues about other potential ocular or systemic issues, so it’s important to get routine eye exams to catch these issues early.

Testing for Choroidal Folds

Fundus fluorescein angiography (FFA) is a type of test that can reveal severe changes in the back part of the eye (the fundus) not visible with a routine eye exam. After injecting a dye called fluorescein, bright and dark patches can be seen, providing a clear image. This method can detect more issues than a simple eye exam using light.

If you have a condition called VKH disease, FFA can show specks of bright patches that later stages develop a pooling effect, encased by a dark ring. Central serous chorioretinopathy (CSCR), another condition that affects your eye, shows up on FFA as a unique pattern of leakage, often thought to resemble an inkblot, smokestack, or a free-flowing leak. For those with choroidal metastasis, FFA is particularly helpful to show the size and type of the lesion that wouldn’t usually be picked up during a clinical examination.

Indocyanine green angiography (ICGA) is preferred for examining conditions related to choroidal folds. Here, VKH disease and CSCR present specific signs that can be identified via this imaging technique.

An ultrasound B scan (USG) offers a non-destructive way of researching the health of your eyes. Although it won’t identify choroidal folds, it does show if the back of your eye (posterior sclera and choroid) is flat or thick. It’s notably useful for checking a few different conditions, including choroidal masses, retrobulbar mass, and posterior scleritis. The B scan can also measure the thickness of the choroid and assess flatness at the back of your eye in cases of orbital masses.

Another test usually carried out is an optical coherence tomography (OCT). Here, the OCT can identify the presence of bumpy retinas often found in cases of choroidal folds. Additional findings are related to CSCR and VKH diseases.

Measuring blood pressure is also crucial, especially if there’s concern over accelerated hypertension.

Finally, a CT scan or MRI might be essential to examine any potential underlying issues causing problems with vision. Occasionally, a lumbar puncture (where a small amount of fluid is taken from your spine) might be necessary.

If choroidal folds and thyroid orbitopathy are linked, tests on thyroid function may be carried out if the condition is suspected.

Treatment Options for Choroidal Folds

When a patient has choroidal folds, or wrinkles within the eye, it’s often a bit more of a challenge if those folds only appear in one eye. In these cases, they are more likely to have serious eye or orbital (eye socket) diseases. To treat these folds, doctors need to focus on what’s causing them in the first place. This can sometimes be tricky to diagnose. Doctors need to take a personalised approach for each patient to make sure serious conditions are properly diagnosed.

Some people have choroidal folds without any symptoms, meaning there’s usually no need for treatment. Similarly, patients who have farsightedness due to choroidal folds typically don’t need treatment. Treatment for choroidal tumours, a possible cause of the folds, will depend on the type of tumour.

If the patient has central serous retinopathy (CSCR), which is a build-up of fluid under the retina, they’ll usually start with lifestyle changes. If the condition is chronic or keeps coming back, doctors can use different types of lasers to treat the leakage point. Other treatment options include photodynamic therapy, drugs that decrease fluid in the eye, and steroid therapies.

Severe cases of fluid build-up in the uveal tract (the middle layer of the eye) can be treated surgically. Eyes that are smaller than average come with a risk of complications like fluid build-up and angle-closure glaucoma (high eye pressure). In this case, the treatment focuses on these complications. Angle-closure glaucoma can be treated with laser or surgery.

In cases of papilledema, a condition where the optic nerve at the back of the eye becomes swollen due to increased pressure in the skull, doctors need to identify and manage the cause of this increased pressure.

In the case of low eye pressure or posterior scleritis (an inflammation of the outer coat of the eye), treatment aims to find the cause. Painkillers and corticosteroids can be prescribed. If these are ineffective, intravenous (into blood vein) corticosteroids can be used. For a specific inflammatory disease called VKH, high-dose corticosteroids and immune modifying therapy may be required. If abnormal blood vessels are growing in the choroid, anti-VEGF (drugs that stop the formation of new blood vessels) can be used. Choroidal scars are typically just observed without treatment.

Conditions related to space travel are managed similarly to papilledema. Specific glasses can be used, and other techniques have been explored to control shifts of fluid in the body. If a patient has orbital lesions (abnormal tissues in the eye socket), the treatment will depend on the nature of these lesions. Infections in the orbit and sinuses are typically treated with broad-spectrum antibiotics for 2 to 3 weeks.

For Graves ophthalmopathy, which is an autoimmune condition impacting the eyes, management requires coordination between endocrinologists and ophthalmologists. Treating an overactive thyroid is important, but not always needed before addressing urgent eye symptoms. The condition is managed with anti-thyroid drugs, radiation therapy, or surgical removal of the thyroid. Supportive steps for the eye, such as lubricants and quitting smoking, are helpful. Steroids and radiation therapy may be viable options, along with surgical procedures if appropriate.

Retinal and choroidal folds are comparable conditions that are key in relevant diagnoses. Generally, retinal folds match the expected pattern for fluorescein (a dye used for some eye tests). They’re usually thinner compared to choroidal folds, and seem to stem from damaged areas in the retina. There’s usually an irregular vitreous (the gel-like substance in the eye) or vitreoretinal interface related to these folds.

Retinal folds may show increased twisting of the retinal blood vessels and can appear due to reasons like a membrane on the retina’s surface, scars beneath the retina, abnormal blood vessels below it, fluid under the retina, thickening of the uveal (layer of the eye) or sclera (white part of the eye), or issues with the optic nerve (which carries visuals from the eyes to the brain).

Lines of fine pigmentation, interspersed with light orange, are typically found in cases with both retinal and choroidal folds. These are common in macular degeneration (an eye condition affecting the macula) and resemble a thick, crumpled fabric when observed with a slit-lamp (a microscope used for eye exams). This condition is particularly linked with detachment of the pigment epithelium (layer beneath the retina) and the growth of abnormal blood vessels beneath the retina.

What to expect with Choroidal Folds

The outcome for patients with choroidal folds often depends on other health conditions they may have:

* Typically, people with idiopathic choroidal folds, or sudden nearsightedness with choroidal folds, maintain good vision health and have a positive outlook.
* When choroidal folds are present alongside diode endolaser photocoagulation – a specific eye treatment – they are usually stable in the long term.
* If you have low eye pressure, also known as hypotony, and this is detected and dealt with early, your health outcome is generally good. In some cases, the choroidal folds may even go away if the pressure in your eye returns to normal.
* If you have a uveal effusion, or fluid build-up in your eye, the prognosis can vary. In severe cases, this condition could potentially lead to permanent vision loss.
* In cases of posterior scleritis, an inflammation at the back of your eye, your vision outlook is usually good if treatment is started quickly.

For Vogt-Koyanagi-Harada disease, a rare disorder affecting the eyes, prompt and strong steroid treatment generally leads to a good vision outcome. Similarly, the outcome is typically good in cases of space-flight associated neuro-ocular syndrome, a condition related to changes in the eye due to space flight.

Possible Complications When Diagnosed with Choroidal Folds

Small flaws can appear in the retina along the deep wrinkles in the choroid, which is the layer of blood vessels in the back of the eye. These flawed areas can later become associated with the growth of new, and often abnormal, blood vessels in the choroid. As a result, regular and careful monitoring is necessary to catch these changes.

Preventing Choroidal Folds

Patients should be given proper guidance regarding the outcome of choroidal folds, a condition affecting the eyes. If the cause of the choroidal folds is unknown, doctors typically observe the condition closely. Meanwhile, for all other cases where the cause is known, patients need to undergo further evaluations and treatment plans based on the identified cause.

Frequently asked questions

Choroidal folds are a condition characterized by alternating dark and bright lines beneath the retina, resembling grooves or stripes. They occur in patients with papilloedema caused by a lesion that occupies space in the eye. These folds typically line up horizontally but can also be vertical, slanted, circular, or irregular.

The signs and symptoms of Choroidal Folds can vary depending on the specific condition or underlying cause. In some cases, patients may not experience any symptoms at all, while in others, Choroidal Folds can lead to worsened vision. Common signs and symptoms associated with Choroidal Folds include: - Blurry vision: Patients may complain of blurry or distorted vision, which can affect their ability to see clearly. - Swelling: Some cases of Choroidal Folds may be accompanied by slight swelling in the affected eye. - Engorged retinal vessels: Hypotony-related Choroidal Folds, which are associated with low eye pressure, may exhibit engorged retinal vessels. - Macular edema: Although rare, Choroidal Folds can sometimes lead to macular edema, which is characterized by fluid accumulation in the macula. - Headaches: In cases of Papilledema, which is often coupled with Choroidal Folds, patients may experience headaches. - Vomiting: Papilledema can also cause vomiting in some individuals. - Blurred vision: Along with headaches and vomiting, Papilledema may also result in blurred vision. - Folds around the optic disc: During an eye exam, folds around the optic disc may be observed in patients with Papilledema. - Vision loss: Posterior scleritis, another condition associated with Choroidal Folds, can cause vision loss in one eye. - Severe pain: Patients with posterior scleritis may experience severe pain in the affected eye. - Reduced eye movement: Along with vision loss and pain, reduced eye movement is a common symptom of posterior scleritis. - Hair loss: VKH disease, which can present with Choroidal Folds, may progress to a recovery stage characterized by hair loss. - Cataracts: In the chronic phase of VKH disease, patients may develop cataracts or other serious issues. - Disc edema: SANS, a condition primarily seen in astronauts, can cause disc edema, which is swelling of the optic disc. - Retinal spots: SANS may also result in the appearance of retinal spots in the affected eye. - Hyperopia or astigmatism: Depending on the location of intra-conal or extra-conal lesions, patients may experience hyperopia or astigmatism, respectively. - Impact on fold pattern: The presence of intra-conal or extra-conal lesions can impact the fold pattern in the choroid. It's important to note that routine eye exams are crucial for the early detection of Choroidal Folds and any associated ocular or systemic issues.

Choroidal folds can be caused by various conditions, including inflammation in the back part of the eye's white area during development or childhood, reduced eye pressure, mechanical force on the eye or its layers, swelling and buildup of fluid in the eye socket, thickness of the blood vessel layer in eyes, diseases that increase brain fluid, inflammation of the white area of the eye, new blood vessel growth under the retina, contraction in the area beneath the retina, eye disorder related to space flights, changes in long-sightedness, congestion in the blood vessel layer, deformation of the white area of the eye, prolonged inflammation or traction, and decreased rigidity of the white area of the eye.

The doctor needs to rule out the following conditions when diagnosing Choroidal Folds: 1. VKH disease 2. Central serous chorioretinopathy (CSCR) 3. Choroidal metastasis 4. Conditions related to choroidal folds that can be identified via indocyanine green angiography (ICGA) 5. Choroidal masses, retrobulbar mass, and posterior scleritis that can be checked using an ultrasound B scan (USG) 6. Bumpy retinas often found in cases of choroidal folds that can be identified using optical coherence tomography (OCT) 7. Potential underlying issues causing problems with vision that can be examined using a CT scan or MRI 8. Thyroid orbitopathy if choroidal folds and thyroid orbitopathy are linked 9. Macular degeneration, which is commonly associated with retinal and choroidal folds

The types of tests needed for Choroidal Folds include: - Fundus fluorescein angiography (FFA) to detect the presence of choroidal folds and assess their severity. - Ultrasound B scan (USG) to determine if the back of the eye is flat or thick and to check for other conditions such as choroidal masses and posterior scleritis. - Optical coherence tomography (OCT) to identify bumpy retinas associated with choroidal folds. - Measurement of blood pressure to assess for accelerated hypertension. - CT scan or MRI to examine any potential underlying issues causing problems with vision. - Thyroid function tests if choroidal folds and thyroid orbitopathy are suspected to be linked. - Lumbar puncture (in some cases) to collect fluid from the spine. - Personalized approach and additional tests as needed to properly diagnose any serious conditions associated with choroidal folds.

The treatment for Choroidal Folds depends on the underlying cause. If the folds are not causing any symptoms or if they are due to farsightedness, treatment may not be necessary. However, if the folds are caused by conditions such as choroidal tumors, central serous retinopathy, or fluid build-up in the uveal tract, treatment options include lifestyle changes, laser treatment, photodynamic therapy, drugs to decrease fluid, steroid therapies, and surgical intervention. The specific treatment approach will vary based on the individual patient and the severity of the condition.

The prognosis for Choroidal Folds depends on other health conditions and factors, but generally, the outlook is positive. People with idiopathic choroidal folds or sudden nearsightedness with choroidal folds usually maintain good vision health. If certain conditions like low eye pressure or uveal effusion are detected and treated early, the health outcome is generally good. However, in severe cases of uveal effusion or other conditions like posterior scleritis, there may be a risk of permanent vision loss.

An ophthalmologist.

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