Cicatricial Conjunctival Disorders

Introduction & Overview - Scarred Sights Story

• Cicatricial conjunctival disorders: Chronic inflammation leading to irreversible conjunctival scarring and subepithelial fibrosis.
• Key features: Fornix shortening, symblepharon (adhesions between palpebral and bulbar conjunctiva), ankyloblepharon (eyelid fusion).
• Impact: Progressive corneal damage, severe dry eye, restricted ocular motility, and potential blindness.

⭐ Stevens-Johnson Syndrome (SJS) and Ocular Cicatricial Pemphigoid (OCP) are major immune-mediated causes with severe ocular surface consequences, often leading to significant visual morbidity if not managed promptly and aggressively.

Key Culprits & Causes - The Scarring Lineup

Diverse factors lead to conjunctival scarring:

Spotlight: OCP & SJS/TEN - Immune System Mayhem

• OCP (Ocular Cicatricial Pemphigoid):
  • Chronic autoimmune (Type II hypersensitivity) vs. conjunctival Basement Membrane Zone (BMZ).
  • Features: Symblepharon, fornix shortening, ankyloblepharon, progressive corneal changes, severe dry eye.
  • Dx: Conjunctival biopsy + Direct Immunofluorescence (DIF) showing linear IgG, IgA, C3 at BMZ.
• SJS/TEN (Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis):
  • Acute Type IV hypersensitivity; commonly drug-induced (e.g., sulfonamides, anticonvulsants) or infection-triggered.
  • Features: Mucocutaneous blistering, epidermal detachment (SJS <10% Body Surface Area (BSA), TEN >30% BSA).
  • Ocular: Severe bilateral conjunctivitis, pseudomembranes, symblepharon, keratitis, corneal ulcers, cicatricial ectropion/entropion.

⭐ Early amniotic membrane transplantation (AMT) in the acute phase of SJS/TEN is crucial for reducing ocular surface scarring and improving long-term visual prognosis. 📌 SJS = Sudden Jeopardy to Sight!

Spotlight: Trachoma & Chemical Burns - Infection & Injury Scars

• Trachoma: Chronic keratoconjunctivitis by Chlamydia trachomatis (serovars A-C).
  • WHO FISTO stages: TF (follicles), TI (intense inflammation), TS (scarring), TT (trichiasis), CO (corneal opacity).
  • Key signs: Arlt's line (scar on tarsal conjunctiva), Herbert's pits (cicatrized limbal follicles).
• Chemical Burns: Ocular emergency! Immediate, copious irrigation vital.
  • Alkali (e.g., $NaOH$, $Ca(OH)_2$): More severe; liquefactive necrosis, ↑penetration.
  • Acid (e.g., $H_2SO_4$): Coagulative necrosis, forms protective barrier.
  • Grading (e.g., Roper-Hall, Dua) guides prognosis.
  • Late sequelae: Symblepharon, ankyloblepharon, corneal opacity, glaucoma.

⭐ Alkali burns are generally more severe than acid burns due to rapid penetration and saponification of fats, leading to deeper tissue damage.

Clinical Picture & Management - Decoding & Defeating Scars

• Symptoms: Chronic irritation, dryness, foreign body sensation, photophobia, progressive ↓vision.

• Signs: Conjunctival hyperemia, subepithelial fibrosis, forniceal shortening, symblepharon, ankyloblepharon, trichiasis, entropion. Corneal: keratinization, vascularization, opacification.

• Management:

  • Medical: Aggressive lubrication; control inflammation (topical/systemic steroids, immunosuppressants like cyclosporine, methotrexate, cyclophosphamide for OCP/SJS).
  • Surgical: Symblepharon release, fornix reconstruction (amniotic membrane transplant (AMT), mucous membrane graft (MMG)), lid correction, keratoprosthesis (KPro) for end-stage.

⭐ Early and aggressive systemic immunosuppression is crucial in Ocular Cicatricial Pemphigoid (OCP) to prevent irreversible blindness.

High‑Yield Points - ⚡ Biggest Takeaways

• Ocular Cicatricial Pemphigoid (OCP): Autoimmune; progressive subepithelial fibrosis, symblepharon, critical forniceal shortening.
• Stevens-Johnson Syndrome (SJS)/TEN: Severe drug-induced reactions; cause cicatricial conjunctivitis, dry eye, keratinization.
• Trachoma: Chlamydia trachomatis; presents with Arlt's line, Herbert's pits, leading to trichiasis, corneal opacity.
• Common cicatricial sequelae: Symblepharon (palpebral-bulbar conjunctival adhesions), ankyloblepharon (eyelid margin adhesions).
• Management: Crucial lubrication, immunosuppression (OCP, SJS), surgery for complications like entropion.
• Alkali burns: More damaging; immediate, copious irrigation is vital.