Case Report: Management of Corneal Clouding from Lecithin: Cholesterol Acyltransferase Deficiency.

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.

Correlation of Scheimpflug densitometry changes with clinical outcomes after corneal crosslinking.

PURPOSE: To characterize changes in densitometry after corneal crosslinking (CXL) and correlate it with visual outcomes. SETTING: Tertiary referral academic medical center, New York, New York, USA. DESIGN: Retrospective case series. METHODS: Patients with progressive keratoconus or post-laser in situ keratomileusis ectasia had CXL following the Dresden protocol. The corrected distance visual acuity (CDVA) and Pentacam imaging were obtained at baseline and follow-up visits. RESULTS: Fifty-seven patients were followed for a mean of 15 months (range 1 to 24 months) after CXL. The CDVA improved significantly from baseline to 6, 12, 18, and 24 months postoperatively. The change in densitometry of the mid-stromal layer, 2.0 to 6.0 mm annulus, at 6 months was correlated with the improvement in CDVA at 6, 12, and 24 months (all P < .10). The increase in densitometry of the mid-stromal layer, centermost 0.0 to 2.0 mm annulus, at 6 months was significantly associated with the decrease in maximum keratometry (K) at 6 and 12 months (both P < .05). Last, the change in densitometry at 6 months was significantly correlated with the decrease in specific higher-order aberrations (HOAs) (P < .05). CONCLUSIONS: Although the greatest and most durable post-CXL densitometry change was in the anterior layer, the degree of increased densitometry haze in the mid-stromal layer was most associated with and possibly predictive of improvement in CDVA, maximum K, and HOAs. The persistence of corneal haze at 6 months, measured by increased densitometry, might be a prognostic marker for CXL effectiveness.