The White of the Eye

Sclera Definition: White of The Eyeball

The sclera is commonly known as the “white” of the eye. It surrounds your entire eyeball, except for the clear covering in the front of your eye, the cornea. The area where the sclera meets the cornea is called the limbus.

Several layers comprise the sclera, including (in order from outermost to innermost layer) the episclera, stroma, lamina fusca, and endothelium. The front part of your sclera is covered with a thin layer of tissue with blood vessels, called the bulbar conjunctiva.

Purpose & Function of The Sclera

The sclera is a tough, fibrous, opaque tissue that protects the inner structures of your eye from trauma and helps maintain the shape of your eyeball. Most of your eyeball is filled with a gel-like fluid called the vitreous humor. Your eye muscles, which control your eye movements, also attach firmly to the sclera. 

Sclera Problems

Several conditions can affect the sclera. It is important to note that while some problems may appear to affect the sclera, they actually affect the overlying bulbar conjunctiva. The appearance can be misleading because the bulbar conjunctiva is mostly transparent.

Sclera conditions include:

• Episcleritis is an inflammation of the outermost layer of the sclera. Symptoms include redness, mild pain, swelling of the conjunctiva, eyelid swelling, and raised nodules on the eye.  In most cases, only a section of the sclera is affected (sectoral), although the entire white part of the eye can be affected (diffuse). About 26 to 36 percent of those with episcleritis have an associated systemic condition such as rheumatoid arthritis, lupus, Crohn’s disease, Behcet’s disease, or gout.
• Scleritis is inflammation of the sclera. Its presentation can be similar to episcleritis, but typically causes significant pain, light sensitivity, and possibly decreased vision. Scleritis appears as a deeper red (almost violet) than episcleritis. Approximately 30% of people with scleritis have collagen vascular diseases, including rheumatoid arthritis, lupus, and ankylosing spondylitis. This condition is more severe than episcleritis and may cause permanent damage to the eye if left untreated. 
• Scleromalacia perforans is a rare and severe condition in which your immune system attacks the tissue of the sclera, causing significant thinning and bulging. Because of thinning, the underlying uveal tissue is visible, which gives the sclera a bluish appearance. In rare cases, the eye can rupture. Scleromalacia perforans is often associated with older women with long-standing rheumatoid arthritis.
• Scleral icterus is a term to describe jaundice that affects the eyes, giving the whites of the eyes a yellow appearance. The name is not entirely accurate because it affects the conjunctiva, not sclera. However, many doctors still refer to this condition as scleral icterus. This condition occurs when your body has high bilirubin levels, which is a yellowish substance that is produced when your red blood cells normally break down. Liver disease or other serious health conditions can cause high bilirubin levels.
• Scleral plaques are blue-gray calcified plaques that appear on the sclera. They are related to older age, benign, and do not cause any symptoms. 
• Blue sclera occurs when the sclera is thinned such that you can see the dark, uveal tissue underneath, which is where the sclera gets its blue appearance. The cause may be congenital (present at birth), such as in osteogenesis imperfecta, which causes the baby to have bones that break easily. Blue sclera is also associated with Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Acquired blue sclera (developed later in life) may be a sign of severe iron deficiency.

Types of Congenital Scleral Abnormalities

There are other conditions present at birth that may affect the sclera. Some of these are harmless, but others are more severe and may affect vision or eye health.

• Ocular melanosis is the pigmentation of the conjunctiva overlying the sclera. They typically look like flat, brown spots on the white part of the eye, similar to a freckle on the skin. Melanosis can be congenital or acquired. 

There are two acquired forms, primary and secondary acquired melanosis. The primary form is more common in light-skinned individuals and may grow in size. In rare cases, primary acquired melanosis may develop into malignant (cancerous) melanoma. The secondary form is associated with hormonal changes, radiation exposure, chemical exposure, or metabolic disorders. People with darker skin and hair are more likely to have secondary acquired melanosis, which rarely develops into melanomas.

• Nevus of Ota, also called oculodermal melanocytosis, is a congenital condition that causes hyperpigmentation of the eye, eyelids, and other surrounding areas. The pigment often appears blue or brown. Typically, the nevus does not require treatment, but some people desire laser therapy to lighten the spots for cosmetic reasons. Nevus of Ota is associated with a higher risk of glaucoma and, in rare cases, melanoma of the eye.
• Ectasia (staphyloma) is a congenital thinning and bulging of the sclera. The thinning usually occurs near the limbus and also affects the cornea. The staphyloma often presents with a blue appearance. Staphylomas can also develop on the back portion of the eye, particularly in people with high myopia (nearsightedness). 
• Colobomas occur when there is some tissue missing in the eye at birth, which results in the incomplete formation of a particular structure of the eye. Colobomas can affect the eyelid, iris (giving the pupil a cat-eye appearance), lens, optic nerve, choroid, and retina. Optic nerve, choroidal, or retinal colobomas can cause scleral thinning and staphylomas.

Types of Sclera Procedures & Surgeries

Scleral expansion is a somewhat controversial procedure that is designed to treat presbyopia. Presbyopia is the loss of the ability to focus up close with age. The surgery involves inserting four plastic pieces into the sclera, between the eye muscles. This technology is still improving, and some researchers feel that this procedure can become the gold standard in presbyopia treatment.

A scleral buckle procedure is performed to repair a retinal detachment. During the surgery, the doctor uses cryopexy (freezing therapy) to seal your retina to the eye. Then, the surgeon places a silicone or plastic band around your eyeball (this is the scleral buckle), which helps the retina stay in place. The buckle remains in your eye permanently.