Symblepharon

Specialty: Ophthalmology.

• conjunctive-palpebral adhesion
• conjunctival fusion
• conjunctival flanges

Why it occurs

• Ocular cicatricial pemphigoid (progressive autoimmune blistering disease presenting with bilateral destructive conjunctival fibrosis)
• Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis in late scarring phase
• Severe acute ocular chemical burn (especially those induced by alkalis that rapidly penetrate the tissues)
• Trauma or laceration of the bulbar and palpebral conjunctiva poorly treated surgically
• Sequela of severe membranous conjunctivitis (caused by Corynebacterium diphtheriae or Streptococcus pyogenes)

Initial workup

Slit lamp biomicroscopy to quantify the location and extent of the conjunctival flanges and measure the depth of the conjunctival fornices; Staining of the ocular surface with sodium fluorescein and rose bengal; Biopsy of peripheral healthy conjunctiva with direct immunoflorescence staining (to identify linear deposits of IgG, IgA, IgM or C3 in the epithelial basement membrane zone, characteristic of cicatricial pemphigoid); Forced duction test.

Standard management

• Prednisolone — 1% eye drops, apply 1 drop every 2-4 hours in acute inflammatory phases of healing conjunctivitis to control fibrin exudate and prevent early adhesions
• Cyclosporine — 1% or 2% eye drops master formula, 1 drop every 12 hours as a chronic conjunctival immunomodulator
• Methotrexate — orally or subcutaneously, 7.5 to 25 mg once a week as a first-choice systemic immunosuppressive regimen in progressive ocular scarring pemphigoid, associated with folic acid
• Cyclophosphamide (alternative systemic immunosuppressive therapy in cases of rapid scar progression). *Note: In acute phases of chemical burns, PMMA conjunctival formers are installed to mechanically prevent symblepharon formation.*