A 47-year-old Sri Lankan male presented in October 2006 with a 3-month history of bilateral red watery eyes with a foreign body sensation. He had RA, which was quiescent at presentation for which he was taking oral diclofenac, prednisolone (PDN), and methotrexate (MTX) with calcium and folic acid supplements.
On examination, his best corrected visual acuity (BCVA) was 6/19 right eye (OD) and 6/9 left eye (OS). There was an inferonasal peripheral crescent-shaped area of corneal thinning (80%) forming a gutter from 3 to 6 o'clock, 1.4 mm wide in the right eye, while in the left eye there was a smaller inferonasal gutter with 20% thinning. His PDN and MTX were increased and hourly topical carmellose sodium 0.5% added. Initial reduction of ocular discomfort prompted reduction of his systemic medication, but on review within 9 days BCVA OD was reduced to 6/38 and the right anterior chamber was shallow with a small corneal perforation noted in the corneal gutter. This was plugged with iris and not actively leaking. The perforation was repaired with corneal cyanoacrylate glue and a bandage contact lens (BCL) was placed for comfort and exchanged at 6 weekly intervals thereafter. Slow tapering of his systemic medication was attempted again. On review 4 months later in February 2007, a small leak from the previous perforation was noted. After further cyanoacetate glue repair, he underwent multilayered amniotic membrane graft (AMG) to the right eye in March 2007.
Initially his postoperative course was satisfactory but attendance at follow-up appointments was poor. In June 2009, he attended with marked discomfort and redness of both eyes and BCVA of 6/15 OD and 6/7 OS. He was systemically well with no signs of active RA. He was found to have marked bilateral inferior corneal stromal thinning with perforations in the inferotemporal mid-periphery and a small iris prolapse plugging the hole in both eyes (Figure 1). Bilateral emergency tectonic penetrating keratoplasties (PKP) were performed, decentered infero-nasally in order to replace the thinned cornea (Figure 2). Lamellar grafts were initially attempted but was converted to a PKP as a satisfactory lamellar plane was impossible to create due to a soft eye from the perforations. His systemic medications were again increased.
One week postoperatively, BCVA was 6/38 OD and 6/19 OS but one month later he presented with recurrent, bilateral paracentral corneal melts. Glue repair was again performed and topical cyclosporine 0.5% twice daily was added to both eyes. He subsequently underwent a further triple-layered AMG and second PKP to both eyes in September 2009.
Infliximab (5 mg/kg intravenously) was commenced on the first postoperative day and he continued to receive cycles at the same dose at weeks 2 and 6 and then every 8 weeks, while PDN and MTX were gradually reduced. Since he was from an area of high prevalence of tuberculosis, prophylactic isoniazid was commenced. He underwent uncomplicated phacoemulsification with posterior chamber intraocular implantation in both eyes in June 2010. Eighteen months following infliximab therapy both eyes remained quiet with no signs of further corneal melting. The BCVA was 6/48 in both eyes, limited by subepithelial haze and thinning of his corneal grafts (Figure 3).