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Mild form of Maroteaux-Lamy syndrome: corneal histopathology and ultrastructure
N M Laver1, M H Friedlander, I W McLean
1Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000, USA.
Purpose:
Maroteaux-Lamy syndrome is one of the mucopolysaccharidoses (MPSs) that is caused by the incomplete degradation and storage of dermatan sulfate.
Methods:
We describe a 49-year-old female patient with the mild form of the disease (MPS VI-B) who developed bilateral increasing corneal opacification and increased intraocular pressure after cervical-fusion surgery. After treatment of the increased intraocular pressure, she underwent a penetrating keratoplasty of her right eye.
Results:
The histopathologic and ultrastructural features of the corneal button were the accumulation of membrane-bound vacuoles containing fibrillogranular and lamellated material in keratocytes and endothelial cells and thinning of Descemet's membrane with excrescences.
Conclusion:
Our review of the literature reveals only two prior histologic studies of corneas affected by MPS VI B.