A Rare Presentation Of Keratomalacia In The Setting Of Superior Mesentric Artery Syndrome

To describe a rare presentation of Keratomalacia in the setting of malabsorption caused by benign gastric outlet obstruction with SMA syndrome and coagulopathy

We report the case of a 14 year old malnourished girl, who presented in a wheelchair, with a nasogastric tube in situ, with itching and blurring of vision in the left eye since 5 days. She was admitted at a locally for 11 days for superior mesenteric artery syndrome, treated with hourly NG tube feeds, a hematinic syrup & multivitamin supplements. On exam, she was found to be emaciated. Her VA in the RE was 20/20 and LE was 20/50, with and intraocular pressure (IOP) of 7 and 4 mm Hg. The LE had a nasal corneal dellen with edema, and adjacent patch of boggy conj congestion along with scleritis. The rest of the exam & RE was unremarkable.Smear was negative but cultures grew p. aeruginosa and she was started on hourly antibiotcs & atropine. Her CRP and ESR were raised, but CTD and ANA profile was negative. diagnosis of sclerokeratitis made & patient was assesed for systemic immunosuppression. The child returned after 3 days with no relief and was immunocompetent. Considering systemic condition, the possibility of keratomalacia was discussed and the child was given a high dose of vitamin A after consulting a paediatrician,with topical lubricants. On her follow up 2weeks later, the child walked into OPD with a complete resolution of symptoms and improvement in her overall systemic health. The corneal edema, patch of scleritis with boggy congestion had completely resolved with UCVA of 20/50. She has been steadily improving and has no ocular complaints at present.

Vitamin A deficiency a major public health issue in India, causes preventable blindness in children and pregnant women. Despite supplementation programs, it persists due to malnutrition & poor diet. Sudden onset,rapidly progressing corneal ulcers in uninflamed eyes suggests keratomalacia, in debilitated patients with malabsorption &protein loss. If undiagnosed,condition leads to corneal perforation &blindness. in cases of short term Vit A deficiency &severe protein deficiency keratomalacia can preceed keratinization. Recognizing these signs in cachectic patients is important for physicians in developing countries, to prevent ocular morbidity. This is the first documented case of keratomalacia in a child with SMA syndrome and malnutrition.