A case of primary congenital glaucoma with unilateral corneal amyloidosis

BACKGROUND: Histopathological evaluation of primary congenital glaucoma with extensive corneal amyloidosis. CLINICAL FINDINGS: A three-month-old infant. At the first examination, corneal diameter was 12 mm in both eyes, intraocular pressure (IOP) 43 mmHg in the right eye and 47 mmHg in the left eye. A diagnosis was made of primary congenital glaucoma. At 4 months of age bilateral goniotomy, at 1 year and 2 months of age bilateral goniotomy, and at 2 years and 2 months of age bilateral trabeculectomy and the superficial keratectomy of the right eye were done. At 14 years and 11 months of age the enucleation of the right eye was done because of corneal opacity, visual loss, and figure. HISTOPATHOLOGICAL FINDINGS: In the cornea disappearance of Bowman's membrane, accumulation of amyloid in the rough endoplasmic reticulum of the basal cells and subepithelial fibroblasts, and thickeniny of Descemet's membrane could be seen. At the peripheral anterior synechia new Descemet's membrane was spread over the anterior surface of the peripheral iris. CONCLUSION: Unilateral corneal amyloidosis might have been present in infancy, amyloid produced in the rough endoplasmic reticulum of the basal epithelium and subepithelial fibroblasts and deposited in the subepithelial region. At puberty amyloid might have been produced in the subepithelial fibroblasts. The poor IOP control at puberty might have been due to the complication of neovascular glaucoma.