What condition is known to cause whorl keratopathy?

Whorl keratopathy, characterized by vortex-like opacities in the cornea, is primarily associated with Fabry's disease. This condition is a genetic lysosomal storage disorder caused by a deficiency of the enzyme alpha-galactosidase A, leading to an accumulation of specific glycosphingolipids in various tissues, including the cornea. The accumulation affects the corneal epithelium, resulting in the classic appearance of whorl keratopathy, often noted during an eye examination.

In contrast, the other conditions listed do not manifest with whorl keratopathy. Osteogenesis Imperfecta is primarily known for its effects on bone fragility and does not typically involve corneal changes of this nature. Sickle Cell Anemia is associated with various ocular complications, such as retinal ischemia or hemorrhage, but not with whorl keratopathy. Phenylketonuria primarily affects metabolic processing and can lead to developmental issues if not managed with a low-phenylalanine diet; however, it does not produce corneal changes characteristic of whorl keratopathy. Therefore, Fabry's disease is the distinct condition associated with this specific corneal finding.