What are the typical presentation and treatment of vernal keratoconjunctivitis?

Vernal Keratoconjunctivitis: Presentation and Treatment

Vernal keratoconjunctivitis (VKC) is a severe, sight-threatening allergic eye disease characterized by giant papillae on the upper tarsal conjunctiva with a "cobblestone" appearance, requiring aggressive treatment with topical anti-inflammatory medications to prevent permanent visual loss.

Clinical Presentation

Key Symptoms

• Severe ocular itching and irritation
• Constant tearing
• Photophobia
• Thick mucous ropy discharge
• Foreign body sensation
• Pain (when corneal involvement occurs)

Key Signs

• Giant papillae on the upper tarsal conjunctiva ("cobblestone" appearance) 1, 2
• Gelatinous infiltrations around the limbus (Horner-Trantas dots)
• Severe conjunctival injection
• Shield ulcers (in severe cases)
• Upper eyelid edema
• Punctate epithelial keratopathy

Pathophysiology

VKC is more complex than a simple type I hypersensitivity reaction:

• IgE- and T cell-mediated disease 3
• Th2 lymphocyte predominance 4
• Eosinophil, mast cell, and structural cell activation
• Ocular surface remodeling can lead to severe complications 5

Treatment Algorithm

1. Mild VKC

• First-line therapy:
  • Topical mast cell stabilizers (e.g., cromolyn sodium 4%) 6, 3
  • Topical antihistamines with mast cell-stabilizing activity
  • Cool compresses
  • Preservative-free artificial tears

2. Moderate VKC

• Add to first-line therapy:
  • Dual-acting agents (e.g., olopatadine, lodoxamide) 5
  • Consider short-term topical corticosteroids for acute exacerbations 1

3. Severe VKC

• Acute exacerbations:

  • Topical corticosteroids (short-term use) 1
  • Monitor for IOP elevation and cataract formation

• Long-term management:

  • Topical cyclosporine (0.05%-2%) 1, 3

    • Effective for long-term treatment
    • Allows reduced use of corticosteroids 1
    • Prevents seasonal recurrences

  • Topical tacrolimus (0.03%-0.1%) as alternative immunomodulator 1, 7

    • 0.03% for children 2-15 years
    • 0.03% or 0.1% for patients ≥16 years

4. Refractory/Complicated VKC

• Supratarsal injection of corticosteroid for sight-threatening cases 1
• Systemic immunosuppression (rarely needed):
  • Montelukast
  • Oral cyclosporine or tacrolimus 1
• Surgical intervention for complications (corneal ulcers/scars) 5

Complications to Monitor

• Corneal shield ulcers and scarring
• Keratoconus (associated with eye rubbing) 1
• Steroid-induced complications:
  • Glaucoma
  • Cataracts
  • Increased risk of infections
• Limbal stem cell deficiency

Important Considerations

1. VKC should be controlled prior to corneal cross-linking to decrease risk of sterile keratitis 1

2. Patient education is crucial:

   • Chronic nature of disease
   • Potential complications
   • Importance of avoiding eye rubbing
   • Need for long-term follow-up

3. Follow-up visits should include:

   • Visual acuity measurement
   • Slit-lamp biomicroscopy
   • IOP measurement (if on corticosteroids)
   • Pupillary dilation to evaluate for cataracts

Prognosis

• Typically affects children and young adults
• Often seasonally exacerbated
• Can persist for years with periods of remission and exacerbation
• May resolve spontaneously after puberty in many cases
• Untreated severe VKC can lead to permanent visual loss 5

VKC requires aggressive management due to its potential to cause severe visual complications. Early recognition and appropriate treatment are essential to prevent permanent damage to the ocular surface.