Subepithelial corneal amyloid deposits in a case of congenital glaucoma: a case report

To report a rare association of subepithelial amyloid deposits in a long-standing case of congenital glaucoma.

A 5-year-old girl was brought to the emergency service with complaints of bleeding from the left eye after injury to the eye with the door handle. Parents gave history of enlarged black portion of the eye since birth with increasing white opacity from the age of 6 months. On examination under general anesthesia, the left eye showed a limbal tear extending from 7 to 3.30 o'clock position and uveal prolapse. The right eye showed corneal edema, megalocornea, central white raised plaque, and high intraocular pressure. A diagnosis of bilateral congenital glaucoma with open globe injury, left eye, corneal scarring with degeneration, right eye was made and was advised enucleation. The enucleated eye was submitted for routine histologic examination. At a 3-year follow-up, there was an increase in the corneal scarring, with no significant improvement in vision. The child refused keratoplasty and was referred to low vision centre for visual rehabilitation.

The enucleated eye was distorted and filled with blood. The cornea showed confluent pink homogenous wavy deposits in the subepithelial region. These deposits appeared brick red with Congo Red stain and showed apple green birefringence when viewed under polarized filters, confirming the amyloid nature of the deposits. There was loss of ganglion cell layer in the detached retina.

The subepithelial amyloid deposits, presumably bilateral, in advanced case of congenital glaucoma were possibly secondary to the long-standing edema, scarring and vascularization. These secondary changes should be kept in mind as they could contribute to further deterioration in vision in advanced cases.