title: Corneal hysteresis in mucopolysaccharidosis I and VI.
abstract: OBJECTIVE
  High intraocular pressure (IOP) and glaucoma are often suspected in patients with mucopolysaccharidosis (MPS). To determine corneal hysteresis (CH) and IOP in children with mucopolysaccharidosis I-Hurler (MPS I-H) and MPS VI.
  
  
  METHODS
  Clinical measurements with ocular response analyzer (ORA).
  
  
  RESULTS
  In seven patients, five with MPS I-H treated with stem cell transplantation (SCT), and two with MPS VI, one treated with SCT and the other with enzyme therapy, the IOP was examined with ORA. Ocular response analyzer measurements were made at a median age of 8.7 years in the patients with MPS I-H and at a median age of 9.3 years in the patients with MPS VI. Earlier measurements had raised suspicion of high IOP in one patient. The ORA showed an increased CH and a falsely high IOP values in all 14 eyes. The recalculated IOPs were normal in all 14 eyes. Mild to severe corneal opacities were present in all 14 eyes. Optic disc areas, borders and cupping were clinically normal in the 12 of 14 eyes that were possible to examine. Severe corneal opacities hampered optic disc evaluation in the older patient with MPS VI. Three eyes in two patients had normal thickness of the retinal nerve fibre layer measured with scanning laser polarimetry with corneal compensation (GDx VCC). No patient was diagnosed or treated for glaucoma.
  
  
  CONCLUSIONS
  The IOPs are often falsely high because of an increased resistance of the cornea and correlate to the extent of corneal clouding. In this small, cross-sectional study, it appears that corneal resistance is directly correlated with corneal clouding, although a longitudinal study that evaluates resistance as the cornea clears with treatment would provide more direct evidence that corneal deposits are directly related to resistance. A correct measured IOP can avoid unnecessary medical or surgical hypotensive treatment.
authors.affiliation: Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden. kristina.tear-fahnehjelm@sankterik.se
authors.forename: Kristina Teär
authors.initials: KT
authors.lastname: Fahnehjelm
authors.forename: Enping
authors.initials: E
authors.lastname: Chen
authors.forename: Jacek
authors.initials: J
authors.lastname: Winiarski
mesh_terms: D001696:Biomechanical Phenomena / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000503:physiopathology*; D003318:Corneal Opacity / Q000503:physiopathology* / Q000601:surgery; D003430:Cross-Sectional Studies; D004548:Elasticity / Q000502:physiology*; D056947:Enzyme Replacement Therapy; D005260:Female; D006801:Humans; D007429:Intraocular Pressure / Q000502:physiology*; D008297:Male; D008059:Mucopolysaccharidosis I / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D009087:Mucopolysaccharidosis VI / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D020051:N-Acetylgalactosamine-4-Sulfatase / Q000627:therapeutic use; D033581:Stem Cell Transplantation; D014065:Tonometry, Ocular; D014792:Visual Acuity / Q000502:physiology
chemical_list: D020051:N-Acetylgalactosamine-4-Sulfatase