Cicatricial Ptosis - Case Presentation for MS Ophthalmology Practical/Viva

1. How to Open the Case

2. History

• Onset: sudden (post-trauma, post-surgery, burns) vs gradual (chronic blepharitis, trachoma, ocular cicatricial pemphigoid, Stevens-Johnson syndrome)
• Precipitating cause: lid laceration, chemical/thermal burn, herpes zoster ophthalmicus, trachoma, chronic conjunctivitis, previous lid surgery, radiotherapy to the orbit, long-standing contact lens wear (giant papillary conjunctivitis), symblepharon-forming disease
• Progression: static after the inciting event, or progressive (suggests active cicatrising disease like ocular cicatricial pemphigoid - important because active disease must be controlled before any surgery)
• Associated symptoms: foreign body sensation, watering, trichiasis-related irritation, dryness, diplopia (rule out mechanical restriction), photophobia
• Past history: trauma, lid/orbital surgery, radiotherapy, chronic eye drops (mention specifically since glaucoma drops and long-term steroid drops can contribute to aponeurotic changes, though the cicatricial component is separate)
• Old photographs to establish baseline lid position when the timeline is unclear - Kanski's Clinical Ophthalmology, p.90

3. Examination - Present in This Order

A. General inspection

• Compare both sides for symmetry, look for visible scar bands, skin tethering, lid notching, madarosis, symblepharon, ankyloblepharon

B. Standard ptosis measurements (do these on every ptosis case, examiners expect them cold)

C. Features that specifically point to CICATRICIAL ptosis (mechanical ptosis subtype)

• Visible or palpable scar/fibrous band in the lid, often vertically oriented, tethering the lid to underlying tissue
• Lid does not move freely with the globe on up/downgaze - restricted excursion due to tethering rather than true levator weakness
• Skin shortage/tightness - look for lagophthalmos on gentle lid closure
• Associated entropion, ectropion, trichiasis, lid notching, symblepharon (fornix examination with lid eversion)
• Absent or distorted lid crease from scar disruption
• Lash line irregularities - loss of lashes (madarosis) in the scarred segment
• No worsening on sustained upgaze/fatigue (helps rule out myasthenic ptosis) and no diurnal variation

D. Mandatory associated/differentiating tests (say these out loud in the exam even if normal)

1. Pupil examination - to exclude Horner syndrome and third nerve palsy as a coexisting/alternative cause
2. Bell's phenomenon - assess corneal protection before planning any surgery
3. Fatiguability test / prolonged upgaze - to rule out myasthenia gravis
4. Ice pack test and edrophonium (Tensilon) test if myasthenia is suspected clinically as a comorbid cause
5. Phenylephrine 2.5% test - to assess Muller muscle contribution (differentiates aponeurotic component from pure cicatricial component; response indicates some resection of Muller's muscle or conjunctiva could help)
6. Jaw-winking test (Marcus Gunn) - excludes synkinetic ptosis
7. Orbicularis strength - to exclude seventh nerve involvement/pseudoptosis
8. Corneal sensation and tear film assessment (Schirmer's test, TBUT) - crucial before surgery since cicatricial disease often coexists with dry eye/exposure risk
9. Visual field testing (Humphrey/Goldmann with lid taped up vs down) - to document functional field loss for surgical justification, especially in medico-legal or insurance cases
10. Slit lamp examination of fornices and conjunctiva - look for symblepharon, foreshortening, keratinisation (especially if ocular cicatricial pemphigoid is suspected - biopsy with immunofluorescence may be needed)
11. Old photographs - baseline comparison

4. Differential Diagnosis to Mention

• Aponeurotic (involutional) ptosis
• Neurogenic ptosis (Horner syndrome, third nerve palsy)
• Myogenic ptosis (myasthenia gravis, myotonic dystrophy, chronic progressive external ophthalmoplegia)
• Pseudoptosis (contralateral lid retraction, hypotropia, enophthalmos, brow ptosis, dermatochalasis)

5. Management - Present as a Stepwise Plan

Step 1: Treat the underlying/active disease first

• If due to an active cicatrising process (ocular cicatricial pemphigoid, Stevens-Johnson syndrome, chemical burn sequelae), control inflammation first with systemic immunosuppression/anti-inflammatory therapy (dapsone, systemic steroids, cyclophosphamide as indicated) before any surgical correction - operating on active disease leads to recurrence.
• Treat associated trichiasis, exposure keratopathy, dry eye with lubrication, bandage contact lens, or punctal occlusion as a bridge to surgery.

Step 2: Surgical correction (once disease is quiescent, usually 6-12 months of stability)

• Release of the cicatricial band/scar with excision of fibrous tissue is the primary step, since simple levator surgery will fail if the tether is not released.
• Reconstruction of the posterior lamella if there is conjunctival/tarsal shortage: mucous membrane graft (buccal or hard palate), amniotic membrane graft, or tarsoconjunctival graft from the fellow eye.
• Skin grafting/local flaps (full-thickness skin graft, Z-plasty) if anterior lamellar skin shortage is contributing to the tethering.
• Once the lid is adequately released and lamellae reconstructed, address residual ptosis based on levator function:
  • Good/normal levator function → levator resection/advancement
  • Poor levator function (≤4 mm) → frontalis (brow) suspension using autologous fascia lata, silicone rod, or banked fascia
  • Mild ptosis with good Muller response on phenylephrine test → Fasanella-Servat procedure or Muller muscle-conjunctival resection

Step 3: Postoperative care

• Lubrication to prevent exposure keratopathy (patients with cicatricial disease often have poor Bell's phenomenon and reduced corneal sensation)
• Monitor for recurrence of scarring, undercorrection, or lagophthalmos
• Address residual entropion/trichiasis if present with lid margin rotation procedures

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