Article Text

Download PDFPDF
Measuring corneal clouding in patients suffering from mucopolysaccharidosis with the Pentacam densitometry programme
  1. H M Elflein1,
  2. T Hofherr1,
  3. F Berisha-Ramadani1,
  4. V Weyer2,
  5. C Lampe3,
  6. M Beck3,
  7. S Pitz1
  1. 1Department of Ophthalmology, University Medical Centre, Johannes Gutenberg University Mainz, Germany
  2. 2Institute of Medical Biostatistics, Epidemiology and Informatics (IMBEI), University Medical Centre, Johannes Gutenberg University Mainz, Germany
  3. 3Villa metabolica, Centre for Lysosomal Storage Diseases, Children's Hospital, University Medical Centre, Johannes Gutenberg University Mainz, Germany
  1. Correspondence to Dr Heike M Elflein, Department of Ophthalmology, University Medical Centre, Langenbeckstr. 1, Mainz D-55131, Germany; heike.elflein{at}


Aim 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.

  • Cornea
  • Diagnostic tests/Investigation

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.