Abstract

The histopathology of the posterior half of one eye and the optic nerves of a man with Hunter's syndrome, who was known to have disc oedema without raised intracranial pressure for 11 years of his life, is reported. The possible pathogenesis of the disc oedema is discussed; an important contributory factor was the deposition of abnormal mucopolysaccharides within the sclera. This caused gross thickening of the sclera with compression of the optic nerve at the intrascleral level.