title: Measuring corneal clouding in patients suffering from mucopolysaccharidosis with the Pentacam densitometry programme.
abstract: OBJECTIVE
  To identify a means to objectively measure corneal clouding in patients with mucopolysaccharidosis in a prospective controlled clinical trial.
  
  
  METHODS
  Corneal haze was assessed by slit lamp examination and measured using the densitometry programme of the Pentacam, a rotating Scheimpflug camera in 33 mucopolysaccharidoses (MPS) patients and 32 controls.
  
  
  RESULTS
  Pentacam measurements were available in 31 right and 31 left eyes of 32 patients and in 32 left and right eyes of 32 subjects in the control group. Slit lamp findings correlated very well with corneal density measurements (Spearman correlation right eye (OD)/left eye (OS)=0.782/0.791). MPS patients had higher density units (median OD/OS=14.1/14.7) than control subjects (median OD/OS=6.7/6.9, p<0.001). In patients, the corneal centre density values (median OD/OS=13.8/14.0) did not differ from corneal periphery values (median OD/OS=14.3/14.7).
  
  
  CONCLUSIONS
  The densitometry programme of the Pentacam provides objective measurement of corneal haze in mucopolysaccharidosis patients.
authors.affiliation: Department of Ophthalmology, University Medical Centre, Johannes Gutenberg University Mainz, Germany. heike.elflein@unimedizinmainz.de
authors.forename: H M
authors.initials: HM
authors.lastname: Elflein
authors.forename: T
authors.initials: T
authors.lastname: Hofherr
authors.forename: F
authors.initials: F
authors.lastname: Berisha-Ramadani
authors.forename: V
authors.initials: V
authors.lastname: Weyer
authors.forename: C
authors.initials: C
authors.lastname: Lampe
authors.forename: M
authors.initials: M
authors.lastname: Beck
authors.forename: S
authors.initials: S
authors.lastname: Pitz
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003318:Corneal Opacity / Q000145:classification / Q000175:diagnosis*; D003720:Densitometry / Q000295:instrumentation*; D003941:Diagnostic Techniques, Ophthalmological; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009083:Mucopolysaccharidoses / Q000145:classification / Q000175:diagnosis*; D010781:Photography / Q000295:instrumentation; D011446:Prospective Studies; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
keywords: Cornea; Diagnostic tests/Investigation