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Mary Ann Lavery, MD; W. Richard Green, MD; Ethylin Wang Jabs, MD; Martha W. Luckenbach, MD; James L. Cox

Arch Ophthalmol. 1983;101(8):1263-1274.

Abstract
• The ocular histopathology of systemic mucopolysaccharidosis, type III-B (Sanfilippo's syndrome) was studied using histochemical and ultrastructural techniques. Cytoplasmic, single-membranebound vacuoles containing the major storage product, acid mucopolysaccharide, were found in virtually every ocular tissue. Lamellar cytoplasmic membranous bodies of complex lipid were found mainly in the retinal ganglion cells and the lens epithelium. Many tissues had inclusions that were of an intermediate type and were composed of combined fibrillogranular and lamellar membranous material. Hypopigmentation of the neuroepithelial pigment layers (ie, iris, ciliary, and retinal) seems to be the result of autophagocytosis with melanolysis. Photoreceptor cell degeneration was similar to that seen in some forms of retinitis pigmentosa. The mechanism of photoreceptor cell degeneration is unknown. It may be the result of metabolic dysfunction due to accumulation of mucopolysaccharide in the retinal pigment epithelium.

Author Affiliations
From the Eye Pathology Laboratory, Wilmer Ophthalmological Institute (Drs Lavery, Green, and Luckenbach and Mr Cox) and the Departments of Pathology (Dr Green) and Pediatrics (Dr Jabs), The Johns Hopkins Medical Institutions, Baltimore.

Footnotes
Accepted for publication June 30, 1982.

Reprint requests to the Eye Pathology Laboratory, The Johns Hopkins Hospital, 600 N Wolfe St, Baltimore, MD 21205 (Dr Green).

This investigation was supported in part by the Pearl M. Stetler Research Fund (Drs Lavery and Luckenbach) and research grant 01684-04 from the National Eye Institute, Bethesda, Md (Dr Green).

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