Safety of collagen cross linking in advanced keratoconus in a patient with scleroderma

Abstract

A 26-year-old female presented to us with complaints of progressive loss of vision in right eye (RE) since past 6 months. Patient was a known case of generalized systemic sclerosis with hypothyroidism for the past 2 years and was under treatment with a rheumatologist Physical features of scleroderma seen in her included diffuse skin tightness, hyper pigmentation of skin of face, abdomen, thighs, legs and arms, with ulcers on fingers. Her best corrected visual acuity was 20/320 in the RE and 20/20 in the left eye (LE). Slit lamp examination showed Fleischer's ring in the RE cornea, and prominent corneal nerves in the LE cornea. Pentacam evaluation revealed advanced keratoconus in RE and Forme Fruste keratoconus in LE. Corneal ectasia in systemic sclerosis is a rare event. Collagen cross linking procedure when undertaken in auto immune disorders may cause adverse reactions. We present a case of systemic sclerosis with advanced keratoconus, who underwent uneventful collagen crosslinking procedure, with stabilization of the cornea post operatively, seen at 6 months follow up. In this case report we emphasize on the factors which need to be taken into consideration when planning for surgical management like collagen cross linking in a collagen vascular disease such as scleroderma.