A 58-year-old African-American female presented to a hospital emergency room with a superotemporal marginal corneal ulcer of the left eye two clock hours in length, with mild corneal thinning. Uncorrected visual acuity on the initial exam was 20/30 in the right eye and 20/50 in the left. Both eyes showed diffuse punctate corneal epitheliopathy in the palpebral fissure. Her past medical history included alcohol use since the age of 14, serious alcoholism since the age of 34, and chronic pancreatitis and diarrhea since the age of 52. She reported that she drank 6-12 beers per day. She had a smoking history of >50 pack-years as well as a history of gastrointestinal bleed, liver cirrhosis, cholecystectomy, chronic obstructive pulmonary disease, and anaphylactic reaction to peanuts. She had lived in urban USA since birth.
After corneal culture, the patient was started on hourly topical moxifloxacin 0.5% eyedrops in the left eye as well as artificial tears in both eyes. Culture was positive for alpha-hemolytic streptococcus and catalase-positive Gram-positive cocci in groups with preliminary identification as coagulase-negative Staphylococcus. She returned 3 days later, with an unchanged exam except for mild injection of the right eye, and was maintained on the same antibiotic drops but with an increase in artificial tears in the right eye. Three days after that (6 days after the initial visit), she presented to an outpatient office with bilateral marginal corneal ulcers (fig. 1, fig. 2, fig. 3), both with a thinning >50% and parallel to the limbus. The left eye also had a small hypopyon. Uncorrected visual acuity was 20/40 right, 20/80 left. She did not complete workup or treatment and was lost to follow up for over 1 week. She returned to the emergency room 17 days after initial presentation, with a complaint of bilateral severe vision decrease and painful swelling of the left lower lid of 2 days duration. She was hospitalized at that point.
On admission, vision was hand motions in both eyes. The right eye had deep marginal thinning for 6 clock hours inferiorly. The left cornea had a marginal area of deep stromal thinning temporally for 5 clock hours, and the inferior lid showed tender erythema and edema. Diffuse severe bilateral corneal edema and hypopyon (1.5 mm in the right eye and 1.0 mm in the left eye) were present. B-scan ultrasound of the fundus of both eyes was unremarkable.
After corneal culture, the patient was placed on bilateral topical ocular application of fortified vancomycin eyedrops (25 mg/ml) every hour (for Gram-positive coverage) and moxifloxacin 0.5% eyedrops every hour (for Gram-negative coverage). Cultures from admission grew Pseudomonas aeruginosa and Staphylococcus hominis from the right eye and Staphylococcus warneri and Staphylococcus lugdunensis from the left eye. On the third day of hospitalization, when culture results were available, the eyedrops were changed to fortified vancomycin every 2 h and fortified tobramycin (14 mg/ml) every 2 h. Tobramycin was started because of its potency against Pseudomonas. The presence of three Staphylococcus species in the cultures warranted continuation of the vancomycin eyedrops, but we decreased the frequency to every 2 h around the clock to reduce epithelial toxicity and patient stress. The left lower lid cellulitis did not yield any material for culture, but the patient received IV cefazolin (1 g every 8 h) for 2 weeks with resolution of the cellulitis. Due to the severe corneal ulceration, bilateral amniotic membrane grafts (AmbioDisk; IOP Ophthalmics, Costa Mesa, Calif., USA) with an overlying 16.0-mm Kontur bandage contact lens (Kontur Kontacts, Hercules, Calif., USA) were placed on the fourth day of hospitalization. A urinary tract infection found during hospitalization was culture-positive for vancomycin-resistant Enterococcus and was treated with linezolid.
Testing revealed a marked vitamin A deficiency, with a level of 0 mug/dl (reference range 18-77). Vitamin A analysis was performed by LabCorp using high performance liquid chromatography. The patient had mild protein malnutrition with total protein 6.5 g/dl (reference range 6.6-8.7). Her BMI was 21.4 (normal range 18.5-25). Laboratory analysis showed hypokalemia, hyponatremia, hypomagnesemia, and hypocalcemia. Testing ruled out HIV, tuberculosis, syphilis, sarcoidosis, rheumatoid arthritis, hepatitis A, B, C, and Wegener granulomatosis. The ANA titer was elevated (40) with a fine speckled pattern, but not to the level of significance (80). Sjogren syndrome testing for SS-A/Ro, SS-B/La, Smith antibody, and ribonucleoprotein antibodies were all negative by microparticle multiple immunoassay. Erythrocyte sedimentation rate and C-reactive protein were normal.
Intramuscular vitamin A was not available, so it was repleted with 100,000 IU orally for 3 days, followed by 50,000 IU orally every day for 2 weeks. After initial treatment of the infections and after ruling out systemic tuberculosis, systemic steroids were given (IV methylprednisone starting at 50 mg daily and tapered over 2 weeks). The patient also received thiamine, folate, ascorbate, calcium, vitamin D, multivitamins, topical cyclosporine 0.05% eyedrops, and intensive eye lubrication. The patient's diarrhea, pancreatic enzyme deficiencies, electrolyte abnormalities, and protein malnutrition were treated, and she received extensive nutritional and alcoholism counseling.