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The mucopolysaccharidoses (MPSs) are rare hereditary diseases. They are classified into six types by the distinct lysosomal accumulations of glycosaminoglycans, which give rise to the progressive clinical features with involvement of multisystems. Ophthalmic complications, such as corneal stromal opacity, pigmentary retinal degeneration, optic nerve atrophy, and glaucoma, are common in patients with MPSs.
Cysts in various organs have been reported in patients with MPSs—for example, multiple dentigerous cysts, multifocal large cysts in the white matter and arachnoid of the brain, and bone cysts.1,2 In the eye, membrane bound vacuoles in the non-pigmented epithelium of the ciliary processes have been observed by electron microscopy.3 However, iridociliary cysts have never been reported in patients with MPSs.
We present two cases of multiple iridociliary cysts in two patients with MPSs, one with Scheie syndrome and the other with Maroteaux-Lamy syndrome.
A 18 year old woman, who was diagnosed with Scheie syndrome (MPS type IS) by enzyme assay. The activity of α-l-iduronidase in peripheral blood lymphocytes was …
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