N M Laver1, M H Friedlander, I W McLean. 1. Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000, USA.
Abstract
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.
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.
Authors: Saeed Akhtar; Andrew Tullo; Bruce Caterson; Janet R Davies; Kelly Bennett; Keith M Meek Journal: Br J Ophthalmol Date: 2002-02 Impact factor: 4.638