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April 1967

Ocular Histopathology in Hunter's Syndrome: Systemic Mucopolysaccharidosis Type II

Author Affiliations

From the Wilmer Ophthalmological Institute of the John Hopkins University and Hospital, Baltimore.

Arch Ophthalmol. 1967;77(4):503-512. doi:10.1001/archopht.1967.00980020505013

The histopathology of an eye from a patient with Hunter's syndrome (systemic mucopolysaccharidosis type II) has been described. The cornea had been optically transparent during the patient's life. The deposition of abnormal mucopolysaccharides in the corneal stroma was minimal, whereas large concentrations were present in the endothelium of the cornea, the epithelial structures of the iris and ciliary body, and in the sclera.

Pigmentary degeneration of the retina was histopathologically demonstrated, confirming, for the first time, prior clinical observations in Hunter's syndrome. It is probable that this retinal degeneration is responsible for the decreased vision of those patients having mucopolysaccharide disorders in whom the ocular media are transparent.

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