Keratoconus and Ectatic Disorders

Keratoconus and Ectatic Disorders

Keratoconus and Ectatic Disorders

On this page

Keratoconus 101 - Cone Zone Primer

  • Definition: Progressive, non-inflammatory corneal ectasia; features central/paracentral thinning, steepening, & conical protrusion.
  • Pathophysiology: Involves collagen fibril slippage, altered enzyme activity (e.g., ↑MMPs, ↓TIMPs), & oxidative stress.
  • Epidemiology: Onset typically adolescence/early adulthood; usually bilateral but asymmetric.

    ⭐ Keratoconus is a bilateral, asymmetric, progressive, non-inflammatory corneal thinning disorder, typically starting at puberty. Keratoconus progression and corneal thickness

Etiology & Risks - Why The Cone?

  • Genetic Factors:
    • Strong familial association; ↑ risk with positive family history.
    • Specific gene loci implicated (e.g., VSX1, MIR184, DOCK9).
  • Systemic Associations: 📌 "DOWNright MARvellous Elastic Osteo-Allergies"
    • Atopy, Vernal Keratoconjunctivitis (VKC) - commonest.
    • Ehlers-Danlos syndrome.
    • Marfan syndrome.
    • Osteogenesis Imperfecta.
    • Down syndrome.
  • Environmental Triggers:
    • Chronic, vigorous eye rubbing is a key modifiable risk.

⭐ Chronic eye rubbing is a significant and modifiable risk factor strongly implicated in the development and progression of keratoconus.

Clinical Picture & Diagnosis - Spotting & Mapping

  • Symptoms: Progressive vision blurring & distortion (ghosting, monocular diplopia), glare, photophobia, frequent Rx changes, contact lens intolerance.
  • Key Signs:
    • Direct Ophthalmoscopy: Oil droplet/Charleux sign.
    • Retinoscopy: Scissoring reflex (irregular astigmatism).
    • Slit Lamp: Central/paracentral stromal thinning, conical protrusion, Vogt's striae (vertical stress lines), Fleischer's ring (iron at cone base), Munson's sign (V-shape lower lid on downgaze), Rizzuti's sign (conical reflection), prominent corneal nerves. Apical scarring in advanced cases.
    • 📌 Mnemonic: "Vogt Flew Over Munson's Rizzuts & Scissored Oil"
  • Investigations:
    • Corneal Topography/Tomography: Gold standard. Shows inferior steepening, asymmetric bow-tie. Kmax > 47.2 D.

    ⭐ Corneal topography is the most sensitive method for detecting early keratoconus and monitoring its progression.

    • Pachymetry: Corneal thinning, apex typically < 500 µm.
    • Anterior Segment OCT.
    • Amsler-Krumeich classification (staging).

Corneal topography maps for keratoconus diagnosis Slit-lamp and OCT of Keratoconus

Management Spectrum - Taming The Cone

Goals: Improve visual acuity, halt/slow progression, CL tolerance.

  • Optical Correction:
    • Spectacles: Mild cases.
    • Contact Lenses: RGP lenses (mainstay), hybrid, scleral for irregular astigmatism.
  • Corneal Collagen Cross-linking (CXL):
    • Riboflavin (Vit B2) + UVA light.
    • Standard Dresden protocol: Riboflavin 0.1% in 20% dextran, UVA $3 \text{ mW/cm}^2$ for $30 \text{ min}$ (total $5.4 \text{ J/cm}^2$).
    • Mechanism: Strengthens cornea, halts/slows progression.
    • Indication: Documented progression.

    ⭐ Corneal Collagen Cross-linking (CXL) is the only currently available treatment that can halt or slow the progression of keratoconus.

  • Intracorneal Ring Segments (ICRS):
    • PMMA segments in stroma.
    • Action: Flattens central cornea, regularizes shape.
    • Indications: CL intolerant, clear central cornea.
  • Keratoplasty (Corneal Transplant):
    • Indications: Advanced disease, scarring, CL intolerance, steep cones.
    • Types: Deep Anterior Lamellar Keratoplasty (DALK - preferred if endothelium healthy), PKP.

Keratoconus: Healthy to Severe Corneal Changes

Other Ectasias & Complications - Cone's Cousins & Crises

  • Pellucid Marginal Degeneration (PMD): Inferior crescentic thin (4-8 o'clock), 'crab-claw' topo, high ATR. Protrusion above.
  • Keratoglobus: Rare. Generalized limbus-limbus thin, globular. High myopia. Rupture risk.
  • Post-LASIK Ectasia: Iatrogenic. Progressive steep/thin post-LASIK.
  • Acute Corneal Hydrops (KC/KG): Descemet's break → stromal edema. Sudden ↓vision, pain. Conservative care.

    ⭐ In Pellucid Marginal Degeneration, the area of maximum corneal protrusion is typically superior to the band of peripheral inferior thinning, unlike keratoconus where it's usually at the apex of the thinned area. Pellucid Marginal Degeneration: Stages and Symptoms

High‑Yield Points - ⚡ Biggest Takeaways

  • Keratoconus is a progressive, non-inflammatory corneal thinning causing conical ectasia.
  • Strong association with eye rubbing, atopy, Down syndrome, and connective tissue disorders.
  • Classic signs include Munson's sign, Vogt's striae, and Fleischer's ring (iron line).
  • Corneal topography/tomography is crucial for early diagnosis and monitoring progression.
  • Corneal Cross-Linking (CXL) is the primary treatment to halt progression.
  • Pellucid Marginal Degeneration (PMD) shows inferior thinning with "crab-claw" topography.
  • Keratoglobus involves generalized thinning and globular protrusion, with high perforation risk.

Practice Questions: Keratoconus and Ectatic Disorders

Test your understanding with these related questions

Reis-Buckler dystrophy affects which layer of the cornea?

1 of 5

Flashcards: Keratoconus and Ectatic Disorders

1/10

_____ keratopathy occurs due to exposure to UV rays.

TAP TO REVEAL ANSWER

_____ keratopathy occurs due to exposure to UV rays.

Labrador

browseSpaceflip

Enjoying this lesson?

Get full access to all lessons, practice questions, and more.

Start Your Free Trial