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Scleromalacia Perforans Management

Introduction

paulScleromalacia perforans, sometimes referred to as anterior necrotising scleritis, is characterised by thinning of the sclera and cornea in patients with rheumatoid arthritis as well as certain systemic diseases. This is a progressive disease that has the potential to result in blindness.

Epidemiology of scleromalacia perforans

The disease is of autoimmune origin causing chronic inflammation. The disease affects the middle-aged to the elderly, particularly people over 40 years of age. The condition occurs unilaterally in over 60% of cases.

Ocular presentation of scleromalacia perforans

The patient often complains of dry eyes with gritty irritation and accompanying photophobia as the condition worsens. Later, the cornea is sloughed off signaling melting. Anterior scleritis is evident when a blackish hue is visible through the thinned sclera.

scleromalacia
Left eye showing scleral changes and corneal compromise as a result of scleromalacia perforans. A bandage contact lens was fitted to drape the cornea. P.Ramkissoon, 2020.

Management of scleromalacia perforans

The patient is questioned about accompanying joint pains and encouraged to see the family physician. OCT pachymetry shows peripheral corneal thinning. Slit-lamp biomicroscopy will reveal inflammation and scleral changes. Ocular lubricants are prescribed to moisten the eyes while silicone hydrogel contact lenses are fitted to serve as bandage contact lenses. A tint is incorporated in the spectacle lenses to alleviate photophobia. Oral NSAIDs and topical steroids are used for bringing the ocular inflammation under control and analgesics are essential to relieve pain. Collaboration between optometrists, ophthalmologists, and rheumatologists is crucial in effectively managing this condition.

Clinical Pearls

  • Scleral translucency follows recurrent episcleritis in scleromalacia perforans.
  • Question the patient about chronic joint pains and stiffness.
  • Painful eyes are accompanied by blurred vision and photophobia.
  • There is associated pain with dryness of eyes.
  • Look for visible uveal tissue when there is scleral thinning. This is evident by scleral discoloration.

Conclusion

Scleromalacia perforans is represented by progressive thinning of the sclera generally presenting with a blackish-blue hue visible through the thinned sclera. The approach to scleromalacia management is to treat the systemic ailment and manage the ocular manifestations and prevent melting of the sclera and cornea.

References

  1. Kanski JJ. Clinical Ophthalmology. A systematic approach. 5th ed,Philadelphia: Butterworth-Heinemann, 2003 158-9.
  2. Rheumatoid Arthritis (RA). http://www.medicinenet.com/rheumatoid_arthritis/article.html. Accessed 15 August 2020.
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