IOVS The Oncologist
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(Investigative Ophthalmology and Visual Science. 1971;10:568-580.)
© 1971 by The Association for Research in Vision and Ophthalmology, Inc.

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Conjunctival Ultrastructure in Mucolipidosis III (Pseudo-Hurler Polydystrophy)

HARRY A. QUIGLEY 1 and MORTON F. GOLDBERG 1

1 Eye Pathology Laboratory, Wilmer Ophthalmological Institute, Johns Hopkins Hospital, Baltimore, Md., and the Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago, Ill.

Conjunctival biopsies from two sibs with mucolipidosis III and from their clinically normal parents were examined by histochemistry and electron microscopy. Connective tissue cells were filled with single membrane-limited vacuoles which frequently contained membranous lamellar material. These findings indicate abnormal mucopolysaccharide and glycolipid accumulation in this disorder and suggest a mechanism for the corneal clouding in these patients.

Key Words: mucolipidosis • ultrastructural pathology • conjunctiva • corneal opacification • acid mucopolysaccharide • glycolipid • mucopolysaccharidosis • sphingolipidosis

Submitted on June 11, 1971
Accepted on June 29, 1971






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