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Abstract #3516 Published in IGR 4-2
Subepithelial corneal amyloid deposits in a case of congenital glaucoma: a case report
Vemuganti GK; Mandal AKCornea 2002; 21: 315-317
PURPOSE: To report a rare association of subepithelial amyloid deposits in a long-standing case of congenital glaucoma. METHODS: A five-year-old girl was brought to the emergency service with complaints of bleeding from the left eye after injury to the eye by a door handle. Parents gave history of enlarged black portion of the eye since birth with increasing white opacity from the age of six months. On examination under general anesthesia, the left eye showed a limbal tear extending from the 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 the three-year follow-up, there was an increase in corneal scarring, with no significant improvement in vision. The child refused keratoplasty and was referred to a low vision center for visual rehabilitation. RESULTS: 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. CONCLUSIONS: 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.
Dr. G.K. Vemuganti, Ophthalmic Pathology Service, L.V. Prasad Eye Institute, Hyderabad, India. geeta@lvpeye.stph.net
Classification:
9.1.1 Congenital glaucoma, Buphthalmos (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
