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August 1971

Ultrastructural Ocular Pathology of Hunter's Syndrome: Systemic Mucopolysaccharidosis Type II

Author Affiliations

From the Wilmer Ophthlamological Institute, Johns Hopkins Hospital and University, Baltimore.

Arch Ophthalmol. 1971;86(2):164-177. doi:10.1001/archopht.1971.01000010166009

THE SYSTEMIC mucopolysaccharidoses (MPS) are of ophthalmologic interest because about 75% of patients with these inherited disorders of mucopolysaccharide metabolism develop clinically significant corneal clouding.1 The histologic and ultrastructural pathologic condition of the clouded corneas from these patients has been the subject of many reports.2-7 However, only one report8 has described the ocular histopathology of a systemic MPS case with clear corneas, which occurred in a patient with Hunter's syndrome.

Hunter's syndrome (systemic MPS type II), one of the six or more variants of the systemic MPS,1 resembles the more common Hurler's syndrome (gargoylism, systemic MPS type I) in several respects. Clinically, Hunter's syndrome appears as a less severe form of Hurler's with respect to progressive growth and mental retardation, coarse gargoyle-like facies, multiple skeletal deformities, hepatosplenomegaly, and early death. Both syndromes are also characterized by the increased urinary excretion and excessive tissue accumulations of two

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