Advanced spheroidal degeneration

Abstract

Spheroidal degeneration, involving the cornea and/or the conjunctiva, is characterized by amber-colored homogeneous, translucent spherules in the corneal stroma, Bowman’s membrane, and subepithelium. The condition has a higher prevalence in areas with extreme temperatures, low humidity, high wind, and presence of sand. We report the case of a 46-year-old man with a 10-year history of gradual progressive diminution of vision, severe blepharospasm, and photophobia. Examination revealed bilateral plaques of amber-colored nodules covering about half of the cornea. Superficial keratectomy was performed for the lesions in both eyes, leaving an epithelial defect overlying a plane of opaque cornea. Histopathology showed amorphous protein in the anterior stroma, confirming the clinical diagnosis of advanced grade 4 spheroidal degeneration. Visual acuity and other symptoms dramatically improved, and the patient was scheduled for keratoplasty.