Superior Keratoconus

What is Superior Keratoconus?

Superior keratoconus is a rare eye condition that usually affects both eyes and often develops unevenly. In some rare cases, it might occur in only one eye. Some uncertainty surrounds the existence of superior keratoconus affecting only the upper part of the cornea (the clear, front surface of the eye), but there have been confirmed cases.

Superior keratoconus is characterized by a bulging of the upper part of the cornea, which becomes thin and steepens. As a result, it may affect your vision.

The condition can behave differently in different people and may not show symptoms for many years, while gradually getting worse. It’s often diagnosed in people between ages 10 and 40. In some situations, superior keratoconus may be caused by mechanical disruptions to the cornea, such as drooping eyelids. Common symptoms include worsening or unstable vision quality, as well as discomfort with bright lights, halos, and glare.

Recent advancements in eye scanning technology, namely corneal topography and tomography, have improved our ability to identify and monitor superior keratoconus. These methods map the shape, elevation, and thickness of the cornea, providing essential information for its diagnosis and treatment. Superior keratoconus can often be recognized based on particular patterns in these images, such as steepening at the top and flattening at the bottom, differentiating it from other similar conditions.

Treating superior keratoconus can involve a range of approaches. Nonsurgical options include special contact lenses that help improve your vision by offering a more regular focusing surface. Eye glasses might not always be sufficient due to the irregular shape the cornea can take on. For cases growing worse, a treatment method called corneal collagen cross-linking (CXL) is used. CXL strengthens the cornea by applying a vitamin B2 solution and ultraviolet-A light, creating additional links within the cornea’s collagen fibers. This can stabilize the cornea and prevent it from deteriorating further.

In advanced stages of superior keratoconus, surgery might become necessary. This could involve inserting tiny plastic rings into the cornea to flatten it and improve vision, a procedure particularly beneficial for those not able to wear contact lenses. It might also require a corneal transplant if other treatments don’t work. Despite their potential to significantly improve vision, these procedures come with risks, such as the body rejecting the donor tissue and needing long-term immune system suppressing therapy.

Research indicates genetic factors might play a role in the development of superior keratoconus, suggesting it might be passed down in families. Finding these genetic markers could lead to therapies targeting the root cause of the condition.

Moreover, advancements in technology like Brillouin microscopy and optical coherence elastography enable further studies into the physical properties of the cornea, providing valuable information about superior keratoconus. These could be helpful in early diagnosis and personalized treatment strategies.

The outlook for those with superior keratoconus is usually similar to other cases of keratoconus. The cornea tends to become progressively thinner, and vision can get worse over time. In severe instances, this can cause the cornea to swell suddenly and scar. Aiming to stabilize the cornea while improving vision quality, treatment options may include fitting for contact lenses or corneal cross-linking or transplant or stromal ring insertions.

Superior keratoconus is a complex eye condition which requires a comprehensive approach for effective treatment. Early detection and intervention are essential to avoid significant vision impairment. Ongoing research and advancements in technology continue to advance our understanding and treatment of the condition, with future improvements offering hope for better outcomes for patients.

What Causes Superior Keratoconus?

Superior keratoconus is a condition that causes a bulge in the cornea, the clear front surface of the eye. We don’t exactly know what causes this condition yet. However, several risk factors have been identified. These include eye rubbing, drooping eyelids (blepharoptosis), and connective tissue disorders such as Ehlers-Danlos syndrome, Down syndrome, and Marfan syndrome. There’s a link between superior keratoconus and a condition called atopy, which can cause conditions such as eczema, asthma, and hay fever. However, there’s some debate about this, because people with atopy may be more likely to rub their eyes, due to allergies.

Understanding what causes superior keratoconus is important for early detection and treatment, and for finding ways to prevent it. It’s a complex condition that likely involves a mix of genetic factors, environmental elements, and biochemical imbalances.

Genetics seem to play a big role in superior keratoconus. It’s more common in people who have a family history of the condition. Research has also found that certain genes may be involved. However, there’s currently no single gene that’s been identified as the main cause.

Environment also impacts the development of superior keratoconus. External factors, such as eye rubbing and allergic conditions, can lead to weaknesses in the cornea and contribute to the disease. Chronic eye rubbing, in particular, is strongly linked to this condition. The strain from constantly rubbing your eyes can cause small injuries and weaken the cornea. People with allergic conditions, such as asthma, eczema, and allergic conjunctivitis, are more likely to develop superior keratoconus. This is because the inflammation and itching from these conditions can lead to more eye rubbing.

Biochemical factors, like imbalances in enzymes and increased oxidative stress, also significantly contribute to the development of superior keratoconus. An imbalance in enzymes in the cornea can lead to thinning and weakening of the cornea. Increased oxidative stress and decreased cornea’s ability to fight off harmful compounds can weaken the cornea as well.

Physical factors, such as the cornea’s physical properties, also play a role in the development of this condition. When the cornea isn’t as stiff and the links between collagen aren’t normal, it can contribute to superior keratoconus. These changes can make the cornea more likely to deform under the eye’s normal pressure.

Lastly, Hormonal changes, such as during puberty and pregnancy, can also impact the progression of superior keratoconus, as these can affect the physical properties of the cornea.

In summary, superior keratoconus is a complex condition with many contributing factors, including genetic predisposition, environmental aspects, biochemical imbalances, physical factors, and possibly hormonal changes. Understanding these factors can help identify people at risk and provide early treatment.

Risk Factors and Frequency for Superior Keratoconus

Superior keratoconus is a very rare type of keratoconus, which is a condition where the cornea, the clear front part of your eye, thins and gets a dome shape. It is considered extremely rare and is thought to account for less than 1% of all confirmed keratoconus cases. General keratoconus occurs from 1.5 to 25 times per 100,000 people each year and affects all ethnicities and genders. It was estimated that this condition could range from 0.2 to 4,790 times per 100,000 people each year. Superior keratoconus mainly affects the upper part of the cornea. Most cases of superior keratoconus tend to go unnoticed until the later stages because they don’t show clear symptoms in the beginning.

• Keratoconus can occur anywhere between 50 to 230 times per 100,000 people in the general population.
• The rate of keratoconus is around 54.5 per 100,000 people in the United States.
• People of Middle Eastern, South Asian, and African descent have reported higher rates of keratoconus.
• Keratoconus tends to appear during adolescence and early adulthood, usually between 10 to 25 years old. The disease progresses over 10 to 20 years before it stabilizes.
• Most keratoconus diagnoses occur during the period when the cornea changes rapidly, which typically happens in a person’s 2nd and 3rd decades of life.
• Keratoconus affects slightly more males than females, with the ratio generally around 1.5 males to 1 female.
• Superior keratoconus is a less common form of keratoconus, and it’s hard to say how many people have it because of its rarity and the difficulty in distinguishing it from similar conditions. However, reports suggest that superior keratoconus may account for less than 5% of all keratoconus cases.
• Just like keratoconus, superior keratoconus can affect people everywhere and of all ethnicities.
• The age at which superior keratoconus starts and how it progresses are similar to that of keratoconus, usually beginning in a person’s 20s or 30s. However, precise data on gender distribution is not available.

Studies from around the world, including the Middle East, Asia, and Africa, have reported higher rates of keratoconus. These rates also reflect the genetic and environmental factors that might affect the occurrence of superior keratoconus in these regions. Knowing more about superior keratoconus can help healthcare providers identify who is at risk and apply early detection and treatment strategies to manage this rare eye disorder effectively.

Signs and Symptoms of Superior Keratoconus

Patients with a condition called superior keratoconus usually experience worsening and fluctuating vision, along with discomfort from bright lights and the appearance of halos around lights. They might also share that they have allergies, a habit of rubbing their eyes, or a family member with the same condition. On examining the patient’s eyes, doctors may notice features like abnormal protrusion, and an ‘inverse Munson sign’, which involves a V-shaped indentation of the upper eyelid when the patient is looking upwards.

Most people with superior keratoconus start noticing changes in their vision in their 20s or 30s, although it can start earlier in some people. The condition tends to progress gradually, with periods of no change followed by periods of rapid decline.

A family history of superior keratoconus or similar conditions affecting the shape of the eye can indicate a genetic connection. If a patient has that type of family history, it’s important to note during the examination because it could point to a higher risk and guide further investigations.

Conditions that cause frequent eye rubbing, such as allergies, may contribute to the development of this condition. It can also be associated with other disorders, such as Down syndrome or Marfan and Ehlers-Danlos syndromes.

In the early stages, superior keratoconus may not show any noticeable changes to the eye. In the later stages, however, an examination might reveal:

• Thinning and steepening of the top central and adjacent parts of the cornea
• Vertical stress lines in the eye caused by pressure on a layer of the cornea called the Descement membrane
• Partial breaks in the topmost or lowermost layers of the cornea
• A brown or yellow ring in the cells at the base of the cornea, also known as Fleischer rings
• A bright reflection on the side of the cornea when light is shone on it from the side (Rizutti’s sign)
• A swelling in the cornea, which can leave a scar after it subsides

An additional test called a scissoring reflex on retinoscopy may also indicate irregular astigmatism, a common feature of this condition. Specialized imaging of the eye, such as corneal topography, can reveal steepening and asymmetry in the eye – key signs of superior keratoconus. The back part of the eye is usually normal unless complications have arisen from advanced disease.

Through careful history taking and thorough physical examination, including advanced tests, healthcare providers can accurately diagnose superior keratoconus and separate it from other conditions affecting the cornea. This comprehensive approach is crucial for managing the condition appropriately and preventing significant loss of vision.

Testing for Superior Keratoconus

When it comes to evaluating superior keratoconus, a condition that affects the shape of the cornea in the eye, your doctor will use a mix of physical exams, high-tech imaging methods, and other tests. The aim is to accurately determine how far the disease has progressed and how best to treat it.

Your doctor will first test your vision in different ways to see if you have superior keratoconus. This involves measuring your refraction, which is how light waves bend when they enter your eye. They’ll also test your standard visual acuity (which is how well you can see) both with and without corrective lenses.

Next, your doctor may use a microscopic tool called a slit lamp. This device lets your doctor get a close look at the structure of your cornea and pick out any signs of superior keratoconus.

Another tool at your doctor’s disposal is corneal topography. This test gives your doctor a detailed image of the curve and structure of your cornea and can reveal telltale signs of superior keratoconus. One common sign is thinning and steepening of the upper portion of the cornea.

Your doctor might also use a method called optical coherence tomography (OCT). This technology uses light waves to take very detailed pictures of your eyes. In particular, OCT can measure the thickness of your cornea, which can uncover changes linked to superior keratoconus that might be hidden due to changes in the outermost layer of the cornea.

Another test is retinoscopy, which can spot even the early stages of keratoconus. If you have this disease, retinoscopy will show a scissoring reflex, where two light bands move toward and away from each other.

Corneal tomography is yet another test your doctor can use. This technique creates a 3D image of your cornea and can measure both the front and back surfaces of the cornea as well as its thickness. This test is particularly good at detecting early keratoconus and cases that aren’t yet showing symptoms.

Your doctor may also measure the thickness of your cornea, a process known as pachymetry. This step is important in assessing how thin your cornea has become, and whether treatments like corneal cross-linking (CXL) are suitable for you. Regular measurement of corneal thickness helps to check how your disease is progressing and how well you are responding to treatment.

To get further information about the strength and flexibility of your cornea, your doctor might use machines like the Ocular Response Analyzer (ORA) and CorVis ST. These tools can help your doctor make an early diagnosis and evaluate your risk of developing the disease.

Detailed refraction tests can be used to measure how nearsighted (myopic) or farsighted (hyperopic) you are and how much your cornea is curved (astigmatism). These measurements are key to tracking any changes over time.

In some cases, your doctor might recommend genetic testing. This test can identify any genetic changes linked to keratoconus. This can be particularly helpful if other family members also have keratoconus or your case doesn’t fit the usual pattern.

Your doctor might also examine the density and shape of the cells on the innermost layer of your cornea, especially if your keratoconus is advanced or you have had surgery on your cornea. This can provide additional information about your disease and help to guide treatment.

By following these evaluation steps, doctors can accurately diagnose superior keratoconus, track how it’s progressing, and customize the best possible treatment plan for each individual patient.

Treatment Options for Superior Keratoconus

Superior keratoconus is a condition that alters the shape of the cornea (the clear, dome-shaped front surface of your eye). The way to treat it depends on how far the disease has progressed and how quickly it is doing so. There are both non-surgical and surgical methods of treatment available.

Non-surgical management options are aimed at improving and maintaining your sight quality in the simplest, least intrusive ways possible. Depending on how serious the condition is, treatments can vary from glasses to special lenses that sit on the white part of the eye (known as scleral lenses).

For mild superior keratoconus, the focus is on keeping your vision sharp, often by simply prescribing glasses. There is also the option to use hard contact lenses (RGP or scleral lenses), depending on your personal comfort and preference.

In cases of moderate superior keratoconus, contact lenses are most commonly used. RGP lenses, designed to correct the irregularities in the shape of the cornea, are one option. However, some people find these lenses uncomfortable, especially if there is or was scar tissue on the cornea. Another option is scleral lenses, which create a pool of tears between the cornea and the lens. This helps to correct irregularities in the cornea form and improves vision. Scleral lenses also have a lower chance of moving out of place on the cornea and are generally more comfortable for users.

In severe superior keratoconus, where there is a high risk of scarring on the eye’s cornea, scleral lenses are the recommended treatment.

Surgical treatment for superior keratoconus involves various procedures designed to stabilize the cornea and improve eyesight. From corneal cross-linking, which strengthens the cornea’s structure, to full or partial corneal transplants, and even specialized methods like corneal ring implants are available options.

Cross-linking is a process that stabilizes the cornea by strengthening its fibers. It involves removing part of the outer layer of your cornea, administering a special compound (riboflavin) and then exposing your eye to UV-A light. This interestions strengthens your cornea. This treatment is often coupled with laser eye surgery (photorefractive keratectomy) to enhance sight correction.

In situations when lenses are no longer effective in enhancing eyesight, corneal transplants might be needed. Penetrating keratoplasty (PKP) is a full-thickness cornea transplant that removes all layers of the affected cornea and replaces them with a healthy one from a donor. After surgery, patients generally see a satisfactory improvement in their vision. However, in some cases, additional vision correction might be required.

Deep anterior lamellar keratoplasty (DALK) is a partial-thickness corneal transplant that replaces the outer layer and front part of the cornea. This method offers faster vision recovery and a lower risk of graft rejection and failure when compared to a full transplant.

Intrastromal corneal ring segments, small plastic rings, can be inserted into the cornea to help flatten it, which tends to improve vision and reduce short-sightedness. This method is often used for correcting vision but it does not treat or slow the disease progression.

Beyond these treatments, medications like topical corticosteroids and nonsteroidal anti-inflammatory drugs might be used to control inflammation after surgery, and prevent complications. For those with severe vision impairment that can’t be rectified via surgery or contacts, low vision aids and other rehabilitative services are crucial to improving one’s quality of life.

Regular checks are crucial for tracking the progression of the condition and efficacy of the treatment. Doctors use advanced imaging techniques to assess changes in corneal shape and thickness over time.

Having a long-term health condition like superior keratoconus can also have psychological impacts. Counseling and support groups may provide emotional support for patients, helping them manage those impacts. Dealing with superior keratoconus requires a multi-faceted strategy, and with doctors adhering to modern guidelines, patients can expect to retain and even improve their vision, and overall quality of life.

What else can Superior Keratoconus be?

When a patient has a thinned cornea, physicians need to consider several possible causes. Some of the potential conditions they need to rule out include:

• Corneal ectasia, a type of corneal thinning related to medical procedures
• Terrien marginal degeneration, a rare and slow-acting thinning condition
• Fuchs endothelial dystrophy, a genetic disorder that affects the innermost layer of cornea
• Corneal warpage, which could be induced over time by wearing contact lenses

Superior keratoconus is a subtype of a condition called keratoconus and is often confused with several other disorders that also affect corneal curvature and thickness. Doctors must ensure an accurate diagnosis by also considering:

• Pellucid Marginal Degeneration: A condition where the cornea thins typically in the lower part, giving rise to a unique pattern seen in corneal topography
• Keratoglobus: A rare disorder that causes the entire cornea to thin, assuming a globular shape
• Terrien Marginal Degeneration: Another rare condition involving slow, progressive thinning of the cornea
• Posterior Keratoconus: This primarily affects the back surface of the cornea
• Contact Lens-Induced Corneal Warpage: Prolonged use of rigid contact lenses can lead to changes in cornea shape that might mimic other disorders
• Post-Laser Assisted In Situ Keratomileusis (LASIK) Ectasia: This is a potential complication of LASIK eye surgery characterized by corneal thinning and bulging
• Corneal Dystrophies: A group of conditions that lead to structural changes in the cornea, visible as opacities or deposits on clinical examination

To diagnose these conditions accurately, doctors use a detailed eye examination, corneal topography, and tomography. Interest in advanced imaging techniques is growing; these can offer comprehensive visuals of the cornea’s surface and thickness, which is essential for accurate diagnosis and successful management of cases.

What to expect with Superior Keratoconus

Superior keratoconus, much like general keratoconus, predicts an outlook where the cornea (the clear, dome-shaped front surface of your eye) keeps getting thinner, leading to a decline in vision. Roughly 10% to 20% of patients might need a corneal transplant at some stage of this disease.

The future prognosis of a person with superior keratoconus depends on how advanced the disease is when diagnosed, how quickly it progresses, and how effective the treatment methods are. If superior keratoconus isn’t managed properly, it can get worse over time and cause serious vision problems.

There are several key factors that influence the prognosis:

• Early Detection and Treatment: Regular screenings and advanced imaging techniques, like corneal topography (a non-invasive test that maps the curve of the cornea), can notably improve the prognosis. Early treatments like CXL can stop the disease from getting worse and help maintain vision.
• Treatment Success: How well the treatment works is majorly responsible for overall prognosis. Treatments like RGP and scleral lenses, CXL, and, in severe cases, OPK or DALK play a key role in managing the condition and improving visual outcomes.
• Stage at Diagnosis: If diagnosed at an earlier stage, the prognosis is generally better as there are more effective treatments available to stop the disease from progressing.
• Treatment Adherence: It’s crucial for patients to follow their prescribed treatments and regular check-ups to achieve the best results. Not doing so can lead to the disease getting worse and poor vision outcomes.

When it comes to long-term prognosis:

• Stable Vision with Treatment: With the right and timely treatment, many patients can achieve stable vision and continue having a good quality of life. Particularly, CXL has been successful in stabilizing the cornea and stopping it from becoming more misshapen.
• Potential for Visual Rehabilitation: Even in advanced stages, surgical procedures like TPK and DALK can restore much of the visual function. But, these procedures might have risks like graft rejection and need long-term follow-up.
• Risk of Complications: Patients are at risk of complications like corneal scarring, sudden swelling of the cornea, and graft failure after keratoplasty (cornea transplant). These complications can negatively affect the prognosis, and may need additional treatments.
• Genetic Factors: If a patient has a family history of keratoconus, they may experience faster progression and might need more aggressive treatment.

Overall, with early detection and proper management, the prognosis for superior keratoconus is generally good. Advances in eye-checkup technologies and treatments have significantly improved our ability to address this condition effectively. It’s crucial to regularly monitor and customize treatment plans for the best visual outcomes and improve the quality of life for patients with superior keratoconus.

Possible Complications When Diagnosed with Superior Keratoconus

Superior keratoconus complications can significantly impact vision clarity and quality of life, and are similar to general keratoconus complications. Here are some common troubles:

• Progressive corneal thinning and bulging: This condition, a trademark of keratoconus, leads to drastic vision distortion and problems.
• Corneal scarring: This happens when the cornea thins and becomes more uneven, especially as it reaches advanced stages. This scarring can seriously affect vision and may require surgical attention.
• Acute corneal hydrops: This sudden and severe problem occurs when the Descemet membrane ruptures, causing corneal swelling, pain, and a sudden drop in vision.
• Contact lens intolerance: As superior keratoconus progresses, corneal shape may become too irregular, making it hard or even impossible to wear contact lenses.
• Graft failure post-keratoplasty: Patients who undergo OPK or DALK surgery have an increased risk of graft rejection or failure due to immune reactions, infection, or troubled healing.
• Visual disturbances: Patients often experience significant visual disturbances, including seeing duplicate images, having an oversensitivity to light, seeing halos and experiencing double vision. These symptoms can affect daily activities and decrease the overall quality of life.
• Psychological impact: This chronic and progressive condition can cause psychological stress and anxiety. The effects on vision and lifestyle limitations can lead to frustration and depression.
• Recurrent corneal infections: Patients are at an increased risk of repeated corneal infections due to the compromised corneal integrity. This can lead to inflammation that complicates the condition and can cause additional scarring and vision loss.

To manage the complications, the following practices can be beneficial:

• Regular monitoring and early intervention: Early detection and managing complications are critical. Regular follow-up visits and advanced imaging practices can help monitor the development and detect complications in a timely manner.
• Customized treatment plans: Treatments should be personalized to the patient’s needs and take into account the severity of the illness and any associated complications. Practices can include customized contact lenses, CXL, or surgical procedures.
• Psychological support: Providing psychological support and advice can help patients to cope emotionally with their condition and enhance their overall well-being.

Awareness and management of the complications connected with superior keratoconus is a necessary step for healthcare providers in order to better patient outcomes and their quality of life.

Recovery from Superior Keratoconus

After you’ve had eye surgery, like OPK or ICRS insertion, it’s very important for your doctor to keep a close eye on your recovery. This means regular check-ups to make sure your wound is healing properly, the stitches are holding, and to look for early signs of your body rejecting the graft or an infection developing.

It’s also really important for you to take all the medication your doctor prescribes. This includes eye drops with antibiotics to prevent infection and eye drops with anti-inflammatory medication to control any swelling. You might also be given creams that help your wound to heal and reduce the chances of your body rejecting the graft.

One of the things your doctor will watch for is signs that your body is rejecting the graft, especially in the first year after surgery. If you notice symptoms like redness, pain, sensitivity to light or a sudden drop in your vision, you need to see your eye doctor right away.

After surgery, there are a few different ways to help improve your sight. One of these is fitting you with special contact lenses, like RGP or scleral lenses. These help correct any remaining vision problems and make the surface of your eye smoother, which helps you see better.

If your vision is significantly affected, you might find physical and occupational therapy helpful. Therapists can teach you how to use aids for low-vision and strategies to go about your daily life safely and effectively.

Part of your treatment will also be learning more about your condition and how to manage it. You might find support groups and counselling services helpful for both practical advice and emotional support. It’s good to make lifestyle changes that can help your recovery and protect your eye health, like stopping activities that might injure your eyes, protecting them from UV rays and keeping healthy with good eating and regular exercise.

Regular check-ups over the long term are important to keep track of the graft’s stability and the overall health of your cornea. These check-ups also give your healthcare team the chance to update your treatment if needed. A well-planned aftercare and rehabilitation schedule can greatly improve your sight and quality of life if you have superior keratoconus.

Preventing Superior Keratoconus

It’s important for individuals to have their eyes checked regularly to catch superior keratoconus at an early stage, especially if superior keratoconus runs in the family. Superior keratoconus is a condition that affects the clear front surface of the eye (cornea), making it bulge out like a cone. Once diagnosed with this condition, individuals should try to avoid rubbing their eyes as this could make things worse. They also need to be aware that their condition can get worse over time, but there are a variety of treatment options to help manage it. Some individuals may need a medical procedure, such as corneal transplantation, to improve their vision, with a 10% to 20% chance for needing this kind of intervention.

The doctor may advise them on actions they can undertake to prevent their condition from getting worse or manage it better. Regular screenings are very important, especially for people at risk of developing this condition. This screening may involve special types of eye imaging, such as corneal topography and tomography. Do whatever can be done to minimize eye rubbing is also useful. This may include wearing glasses to protect the eyes or using allergy medications if needed. It is also crucial to wear protective eyewear when partaking in activities that can cause eye injuries.

Having a good understanding of superior keratoconus is key. A doctor will educate patients about the nature of this condition, which can get worse over time and can affect vision, and why regular checks of the condition are needed. Patients can also learn more about multiple treatment options that might be beneficial for them, including non-surgical treatment (like contact lenses) and surgical procedures (such as CXL and OPK). They should understand that getting treatment at an early stage can improve their outcomes and keep the condition from getting worse.

Patients with superior keratoconus are also advised to make changes in their lifestyle to better manage their condition. This includes using recommended glasses or contact lenses, avoiding playing contact sports without proper eye protection, and treating conditions (like allergies) that may cause them to rub their eyes more. It’s also crucial for these individuals to have different types of support. Information about support groups, counseling services, and patient advocacy organizations can help them manage the emotional aspects of living with this chronic eye condition.

By taking steps to prevent the condition from getting worse and better educating patients about superior keratoconus, this condition can be managed more effectively. This can lead to improved vision and a better quality of life for those affected.