Cicatricial Conjunctival Disorders

On this page

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:

CategoryKey Examples & Notes
AutoimmuneOcular Cicatricial Pemphigoid (OCP), Stevens-Johnson Syndrome (SJS)/TEN, Linear IgA Disease
InfectiousTrachoma (late stage, Chlamydia trachomatis), Diphtheria, Adenoviral (severe EKC)
TraumaticChemical burns (alkali > acid), Thermal burns, Surgical (e.g., multiple glaucoma surgeries)
Drug-InducedTopical (long-term antiglaucoma: timolol, pilocarpine; antivirals: idoxuridine), Systemic (practolol)
AllergicSevere Atopic Keratoconjunctivitis (AKC), Vernal Keratoconjunctivitis (VKC) - rare
OthersRadiation therapy, Paraneoplastic syndromes

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. Ocular manifestations of SJS and OCP

⭐ 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.

Arlt's line and follicles in trachoma

⭐ 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.

Practice Questions: Cicatricial Conjunctival Disorders

Test your understanding with these related questions

A patient complains of loss of visual acuity, deafness, and enlargement of the maxilla.

1 of 5

Flashcards: Cicatricial Conjunctival Disorders

1/10

The severe form of mechanically induced papillary conjunctivitis is known as _____

TAP TO REVEAL ANSWER

The severe form of mechanically induced papillary conjunctivitis is known as _____

giant papillary conjunctivitis.

browseSpaceflip

Enjoying this lesson?

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

Start Your Free Trial