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Case Report

Management of Corneal Clouding from Lecithin

Cholesterol Acyltransferase Deficiency

Zemsky, Christine J., BS1; Sherman, Suzanne W., OD, FAAO1; Schubert, Hermann D., MD1; Suh, Leejee H., MD1*

doi: 10.1097/OPX.0000000000001329

SIGNIFICANCE Given that there are few reported cases of lecithin:cholesterol acyltransferase (LCAT) deficiency, recognition of the condition with proper management is notable. Long-term follow-up and contact lens fitting after penetrating keratoplasty provide best possible outcomes.

PURPOSE The purpose of this study was to report a case of LCAT deficiency successfully treated with penetrating keratoplasty and longer-term follow-up with contact lens fitting.

CASE REPORT A 43-year-old white woman of Italian descent presented with corneal clouding and trouble with night vision. The patient had a history of LCAT deficiency, irritable bowel syndrome, gastroesophageal reflux disease, osteoporosis, and hemolytic anemia. Slit-lamp examination demonstrated corneal haze throughout the corneal layers. The corneas had normal pachymetry. Given the opacity of each cornea (right greater than left) and decreased night vision, penetrating keratoplasty was performed on the right eye. At post-operative month 16, the corneal graft remained clear. The patient was able to achieve a best-corrected visual acuity of 20/30+ with a scleral lens.

CONCLUSIONS Penetrating keratoplasty may be necessary to provide better quality of vision in LCAT deficiency patients, specifically to enhance one's contrast sensitivity, despite relatively good Snellen visual acuity.

1Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, New York *

Submitted: January 31, 2018

Accepted: October 7, 2018

Funding/Support: None of the authors have reported funding/support.

Conflict of Interest Disclosure: There are no conflicts of interest to disclose.

Author Contributions: Supervision: LHS; Writing – Original Draft: CJZ; Writing – Review & Editing: CJZ, SWS, HDS, LHS.

© 2019 American Academy of Optometry