[Ocular histopathology and ultrastructure in mucopolysaccharidosis-I]

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.