The ocular histopathology of type I mucopolysaccharidosis was studied with histochemical, ultrahistochemical, and ultrastructural techniques. The latter method showed extensive intracellular accumulation of fibrillogranular vacuoles in virtually every ocular tissue. Ultra-histochemistry proved that the fibrillogranular material was acid mucopolysaccharide. The pathologic mechanism of scleral thickening, retinal pigment epithelial degeneration, and glaucoma were discussed.
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