Ultrastructural Ocular Pathology of Hunter's Syndrome

Abstract

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.¹The histologic and ultrastructural pathologic condition of the clouded corneas from these patients has been the subject of many reports.^2-7However, only one report⁸has 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,¹resembles 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