Keratoconus: An updated review

Jacinto Santodomingo-Rubido, Gonzalo Carracedo, Asaki Suzaki, Cesar Villa-Collar, Stephen J Vincent, James S Wolffsohn

Research output: Contribution to journalReview articlepeer-review


Keratoconus is a bilateral and asymmetric disease which results in progressive thinning and steeping of the cornea leading to irregular astigmatism and decreased visual acuity. Traditionally, the condition has been described as a noninflammatory disease; however, more recently it has been associated with ocular inflammation. Keratoconus normally develops in the second and third decades of life and progresses until the fourth decade. The condition affects all ethnicities and both sexes. The prevalence and incidence rates of keratoconus have been estimated to be between 0.2 and 4,790 per 100,000 persons and 1.5 and 25 cases per 100,000 persons/year, respectively, with highest rates typically occurring in 20- to 30-year-olds and Middle Eastern and Asian ethnicities. Progressive stromal thinning, rupture of the anterior limiting membrane, and subsequent ectasia of the central/paracentral cornea are the most commonly observed histopathological findings. A family history of keratoconus, eye rubbing, eczema, asthma, and allergy are risk factors for developing keratoconus. Detecting keratoconus in its earliest stages remains a challenge. Corneal topography is the primary diagnostic tool for keratoconus detection. In incipient cases, however, the use of a single parameter to diagnose keratoconus is insufficient, and in addition to corneal topography, corneal pachymetry and higher order aberration data are now commonly used. Keratoconus severity and progression may be classified based on morphological features and disease evolution, ocular signs, and index-based systems. Keratoconus treatment varies depending on disease severity and progression. Mild cases are typically treated with spectacles, moderate cases with contact lenses, while severe cases that cannot be managed with scleral contact lenses may require corneal surgery. Mild to moderate cases of progressive keratoconus may also be treated surgically, most commonly with corneal cross-linking. This article provides an updated review on the definition, epidemiology, histopathology, aetiology and pathogenesis, clinical features, detection, classification, and management and treatment strategies for keratoconus.

Original languageEnglish
Article number101559
JournalContact Lens and Anterior Eye
Issue number3
Early online date4 Jan 2022
Publication statusPublished - Jun 2022

Bibliographical note

© 2021 The Authors. Published by Elsevier Ltd on behalf of British Contact Lens Association. This is an open access article under the CC BY license


  • Aetiology
  • Classification
  • Detection
  • Epidemiology
  • Histopathology
  • Management
  • Cornea/pathology
  • Keratoconus/diagnosis
  • Humans
  • Male
  • Corneal Topography
  • Contact Lenses
  • Female
  • Corneal Pachymetry


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