Bilateral Paracentral Climatic Droplet Keratopathy in Congenital Methemoglobinemia

A 59-year-old woman, who is a known case of congenital methemoglobinemia, came with complaints of diminision of vision in both eyes for 2 years. The patient noticed whitish discolouration of cornea since teenage and did not undergo any ophthalmic evaluation, as her vision was normal. Systemic examination showed bluish discolouration of nails and mouth. Serum haemoglobin was 17.4 gm% and methemoglobin was 55.8%. Both eyes’ distant vision was counting fingers at 3 m with prominent blood vessels over the upper lid. The right eye showed dark coloured conjunctival blood vessels, climatic droplet keratopathy in the paracentral cornea with stromal thinning and posterior corneal pigments. 3 mm, irregular pupil and reacting to light. Anterior capsular opacity with pigments on the capsule, and nuclear sclerosis grade 2 with posterior subcapsular opacity. Normal disc and macula with two chorio-retinal atrophic patches along the infero-temporal arcade. Left eye climatic droplet keratopathy on the paracentral cornea. 3 mm, round pupil and reacting to light. Lens nuclear sclerosis grade 1 with posterior subcapsular opacity. Fundus was normal. The presence of climatic droplet keratopathy in both eyes can be a sequelae of persistent corneal epitheliopathy associated with congenital methemoglobinemia. The presence of stromal thinning, pigments on the posterior cornea and anterior lens capsule with choroidal patches in the right eye can be due to superadded kerato-uvietis.