Chapter Notes: Sclera and Episclera

Introduction to Episcleritis

Episcleritis is a prevalent and benign condition that typically resolves on its own. It affects approximately 20-30% of young adults. Importantly, episcleritis does not progress to true scleritis.

There are two main types of episcleritis:

• Simple Episcleritis
• Nodular Episcleritis

Clinical Features of Episcleritis

• Mild Discomfort: Individuals may experience mild discomfort in one eye.
• Watering: Increased tearing or watering of the affected eye.
• Tenderness: The eye may be tender to the touch.

Examination Findings

• Simple Episcleritis: Characterized by redness that can be either sectoral (localized to a specific area) or diffuse (spread across a larger area).
• Nodular Episcleritis: Involves the presence of a nodule with surrounding redness.

Treatment for Scleritis

Mild Cases: No treatment is necessary.

Treatment Options:

• Topical steroids and/or topical non-steroidal anti-inflammatory drugs (NSAIDs).

Recurrent Cases:

• Oral Flurbiprofen (100 mg three times a day) to alleviate pain and inflammation.
• Oral Indomethacin (50 mg twice a day) to reduce pain and inflammation.

Scleritis

Scleritis involves granulomatous inflammation of the scleral coat of the eye. The scleral coat is thinnest where the muscles attach, which is located more posteriorly than anteriorly.

Classification of Scleritis

Anterior Scleritis:

• Non-Necrotizing:
  • Diffuse.
• Necrotizing:
  • With inflammation.
  • Without inflammation.

Posterior Scleritis:

• Necrotizing with inflammation.
• Surgically induced scleritis.

Systemic Conditions Associated with Scleritis

Patients experiencing necrotizing scleritis may be suffering from one of the following systemic conditions:

• Rheumatoid Arthritis:. chronic inflammatory disorder affecting joints, which can also impact other systems in the body.
• Connective Tissue Vascular Disorders:Conditions that involve inflammation of blood vessels and connective tissues, such as:
  • Polyarteritis Nodosa: A serious blood vessel disorder causing inflammation and damage.
  • Systemic Lupus Erythematosus: An autoimmune disease where the body's immune system attacks its own tissues.
  • Wegener's Granulomatosis: A condition causing inflammation of blood vessels, particularly in the respiratory tract and kidneys.
• Miscellaneous Conditions:
  • Relapsing Polychondritis: A rare disorder causing recurrent inflammation of cartilage.
  • Herpes Zoster: Also known as shingles, a viral infection causing a painful rash.

Treatment Options for Scleritis

• Non-steroidal Anti-inflammatory Drugs (NSAIDs): These medications are commonly used to reduce inflammation and pain in scleritis.
• Oral Steroids: Corticosteroids taken by mouth can help control severe inflammation in scleritis cases.
• Immunosuppressive Drugs: These are indicated specifically for necrotizing inflammatory scleritis, where there is a need to suppress the immune response to prevent further tissue damage.

Scleromalacia Perforans

• Scleromalacia perforans is a form of anterior necrotizing scleritis that occurs without inflammation.
• It is commonly observed in women with long-standing seropositive rheumatoid arthritis.
• This condition typically does not present symptoms initially and starts with the appearance of a yellow necrotic patch on the sclera.
• Over time, it leads to significant thinning of the sclera, resulting in large areas where the uvea is exposed.
• Spontaneous perforation of the sclera is rare and usually requires factors such as increased intraocular pressure to occur.
• According to the Review of Ophthalmology, there is currently no effective treatment for necrotizing non-inflammatory scleritis.

Staphyloma

• Staphyloma is a condition characterized by the swelling of the eyeball and the herniation of uveal tissue.

Types of Staphyloma:

• Anterior Staphyloma: This type occurs when there is a defect in the cornea, often referred to as pseudo-corneal conditions.
• Intercalary Staphyloma: This type happens through the limbus and is associated with peripheral corneal ulcers.
• Ciliary Staphyloma: This condition arises through the area of the ciliary body, typically 2-3 mm from the limbus. It is commonly linked with scleritis, perforating injury, and absolute glaucoma.
• Equatorial Staphyloma: This type occurs through the choroidal region and is associated with conditions like scleritis and pathological myopia.
• Posterior Staphyloma: This condition is found behind the equator and is characteristic of pathological myopia. It can also result from other issues such as posterior scleritis and perforating injuries.
• Blue Sclera: This condition is marked by a widespread blue discoloration of the sclera due to thinning.

Causes of Buphthalmos:

• Osteogenesis Imperfecta: This is a genetic disorder characterized by fragile bones that are prone to breaking easily, often without any obvious cause.
• Ehlers-Danlos Syndrome: This group of disorders affects connective tissues, primarily in the skin, joints, and blood vessels. It leads to hypermobility of the joints and skin that bruises easily.
• Pseudoxanthoma Elasticum: This genetic condition affects the elastic fibers in the skin, blood vessels, and other tissues, resulting in various health complications.
• Buphthalmos: Buphthalmos itself is a condition where the eye enlarges abnormally, typically due to increased pressure within the eye.
• Healed Scleritis: This condition involves inflammation of the sclera, which is the white outer layer of the eye. If not properly treated, it can lead to complications.