title: Central corneal thickness and its relationship to intraocular pressure in mucopolysaccararidoses-1 following bone marrow transplantation.
abstract: OBJECTIVE
  To examine the ocular findings in mucopolysaccaridoses-1 (MPS-1) and the relationship between intraocular pressure (IOP) and central corneal thickness following bone marrow transplantation.
  
  
  METHODS
  
  
  
  METHODS
  clinical practice.
  
  
  METHODS
  23 subjects with MPS-1 following bone marrow transplantation were examined.
  
  
  METHODS
  age, sex, visual acuity, presence of strabismus, refractive error, fundus examination, intraocular pressure, and central corneal thickness were assessed for each individual.
  
  
  METHODS
  correlation of central corneal thickness with intraocular pressure. Only the right eye was used in correlations.
  
  
  RESULTS
  Forty-six eyes of 23 subjects were examined. All subjects had been treated with successful bone marrow transplantation with mean follow-up of 8.5 years. Ages ranged from 17 months to 19 years (SD 5.03). Of the subjects, 60.8% were female; 85.5% were hyperopic; 8.6% had best-corrected visual acuity of >/=6/12; 34.5% had best-corrected visual acuity of <6/12 and >6/36 with 56.9% <6/36; 30% had strabismus. IOP ranged from 13 to 41 mm Hg with four children on antiglaucoma therapy. Central corneal thickness ranged from 484 microm to 705 microm and was moderately correlated with measured IOP in the right eye (r = 0.56). Corneal opacification moderately correlated with central corneal thickness (r = 0.57). Seventy percent had a normal disk evaluation; 30% had abnormal disk cupping.
  
  
  CONCLUSIONS
  This is the first large case series of MPS-1 subjects demonstrating a correlation between measured IOP and central corneal thickness. All subjects had corneal opacification moderately correlating with corneal pachymetry.
authors.affiliation: Our Lady's Hospital for Sick Children, Ophthalmic Department, Crumlin, Dublin, Republic of Ireland.
authors.forename: Paul
authors.initials: P
authors.lastname: Connell
authors.forename: Kathryn
authors.initials: K
authors.lastname: McCreery
authors.forename: Aoife
authors.initials: A
authors.lastname: Doyle
authors.forename: Fiona
authors.initials: F
authors.lastname: Darcy
authors.forename: Anne
authors.initials: A
authors.lastname: O'Meara
authors.forename: Donal
authors.initials: D
authors.lastname: Brosnahan
mesh_terms: D000293:Adolescent; D000328:Adult; D016026:Bone Marrow Transplantation / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000000981:diagnostic imaging / Q000473:pathology*; D004596:Electroretinography; D005260:Female; D005500:Follow-Up Studies; D005901:Glaucoma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007429:Intraocular Pressure / Q000502:physiology*; D008297:Male; D033401:Microscopy, Acoustic; D008059:Mucopolysaccharidosis I / Q000150:complications* / Q000601:surgery; D010786:Photoreceptor Cells / Q000503:physiopathology; D011184:Postoperative Period; D011379:Prognosis; D012307:Risk Factors; D013997:Time Factors; D014065:Tonometry, Ocular