Mild form of Maroteaux-Lamy syndrome: corneal histopathology and ultrastructure.

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.

• Authors: N M Laver, M H Friedlander, I W McLean
• Journal: Cornea (Cornea) Vol. 17 Issue 6 Pg. 664-8 (Nov 1998) ISSN: 0277-3740 [Print] United States
• PMID: 9820948 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Cervical Vertebrae (surgery)
• Cornea (surgery, ultrastructure)
• Corneal Opacity (etiology, pathology, surgery)
• Female
• Follow-Up Studies
• Humans
• Intraocular Pressure
• Keratoplasty, Penetrating
• Middle Aged
• Mucopolysaccharidosis VI (complications, pathology, surgery)
• Ocular Hypertension (etiology, pathology)
• Spinal Fusion (adverse effects)