Scleromalacia Perforans: Types and Complications

Scleromalacia perforans is a rare yet serious eye condition that can result in loss of vision. Scleromalacia perforans, also known as necrotizing scleritis, is the most severe form of scleritis, sometimes leading to loss of the eye. It is often associated with systemic disease, such as severe rheumatoid arthritis.

Scleromalacia perforans is characterized by severe thinning of the sclera of the eye and can be categorized into two types: with inflammation, and without inflammation.

Overview

Scleritis is an inflammation of the sclera, the white outer covering of the eye. Blood vessels travel through and on top of the sclera and can be involved in scleritis. Scleritis can be destructive to the eye, causing intense pain and loss of vision for some people. Although scleritis may be associated with trauma or infection, many times scleritis cases are associated with an underlying systemic disease such as rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, polyarteritis nodosa, granulomatosis with polyangiitis, herpes zoster virus, gout, and syphilis. These conditions cause general inflammation in the rest of the body.

Types of Scleritis

Anterior scleritis, the most common type, affects the front portion of the sclera. There are three types of anterior scleritis:

  • Diffuse scleritis is the most common type and is easily treatable. This type displays general redness and inflammation in the front portion of the sclera.
  • Nodular scleritis has nodules or bumps which are often tender to the touch, on the surface of the eye.
  • Necrotizing scleritis (also called scleromalcia perforans) is the most severe form of anterior scleritis.

Posterior scleritis is the rarest form. It affects the back portion of the eye. This form can cause complications such as retinal detachment and angle-closure glaucoma.

Scleromalacia perforans accounts for only 4% of scleritis. People that suffer from scleromalacia perforans have severe pain. The pain can be much greater than how the eye actually appears.

Symptoms

  • Redness
  • Chemosis (conjunctival swelling)
  • Eyelid swelling
  • Proptosis (eye bulging outward)
  • Decreased vision

Additionally, doctors may see:

  • Choroidal folds
  • Retinal elevation
  • Optic disc swelling
  • Macular swelling
  • Retinal detachment

Complications

  • Vision loss
  • Large amounts of astigmatism
  • Anterior uveitis
  • Cataract
  • Glaucoma
  • Globe perforation

The most serious complication of scleromalacia perforans is the perforation of the globe of the eye. When this happens, you can actually see through the sclera, the white part of the eye, and view the tissue underneath. In this case, a scleral graft will be surgically fastened to the sclera. The type of tissue used for the graft can be donor tissue.

Medical treatment of the scleritis once it gets to this point is challenging. Many doctors will try steroids and non-steroidal anti-inflammatory drugs, but they do not seem to work very well. In many cases, immuno-modulatory drugs may be used to prevent progression to scleral perforation.

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