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

Recent Evidence Check

Exam Tip

ARCUS SENILIS & CORNEAL DEGENERATION - Case Presentation for MS Ophthalmology

1. Opening the Case (How to Start)

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2. History Taking - What to Ask

A. Chief Complaint & Duration

• "How long have you noticed the white ring around your eyes?"
• Typically asymptomatic but may present with:
  • Visual symptoms (rarely - only if central cornea involved or associated pathology)
  • Cosmetic concern
  • Incidental finding on routine eye exam

B. Associated Ocular Symptoms

• Foreign body sensation, dryness (evaluate for dry eye - common in elderly)
• Reduced vision (only if central cornea is hazy or other degenerations present)
• Watering (rule out lid laxity, punctal stenosis, or reflex tearing from dryness)
• Floaters/flashes (rule out retinal pathology)

C. Age of Onset (Critical for Classification)

• Age >40 years → typically arcus senilis (age-related, usually innocent)
• Age <40 years → arcus juvenilis/lipoides (RED FLAG for dyslipidaemia, metabolic syndrome, early CAD)

D. Systemic Risk Factors & Associated Diseases

• Hypercholesterolaemia, hypertriglyceridaemia, dyslipidaemia (family history + personal)
• Cardiovascular disease (MI, stroke, angina, peripheral vascular disease)
• Diabetes mellitus (Type 1 or Type 2)
• Hypertension
• Smoking history (pack-years)
• Family history of premature CAD, dyslipidaemia, xanthelasma
• Chronic kidney disease (dyslipidaemia secondary to uremia)
• Hypothyroidism (causes lipid abnormalities)
• Recent lipid panel results if available

E. Medications

• Statins, antihypertensives, antidiabetic drugs
• Topical medications (chronic use of certain drops may alter lipid metabolism)

F. Lifestyle

• Dietary habits (high cholesterol/saturated fat diet)
• Exercise pattern
• Alcohol consumption

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3. Clinical Examination - Present Systematically

A. Ocular Examination — Anterior Segment

1. Visual Acuity

• Corrected VA in both eyes (arcus itself typically does NOT affect VA unless central cornea involved)
• Compare and record as decimal or Snellen
• Note: poor VA with arcus suggests additional pathology (dystrophy, cataract, etc.)

2. Slit Lamp Examination (THIS IS KEY)

3. Age-Related Corneal Degenerations (Often coexist with arcus senilis)

4. Central Corneal Clarity

• Record if clear or if there is any central haze
• (True arcus senilis should NOT affect central cornea; if present → different diagnosis or comorbid pathology)

5. Anterior Chamber

• Depth (goniometry if glaucoma suspected)
• Look for inflammation (KP, AC reaction) — absent in simple arcus senilis

6. Iris & Pupil

• Iris color, texture, neovascularisation
• Pupil size, reactivity, RAPD

7. Lens

• Grade cataract if present (arcus senilis age-group often has concurrent cataracts)
• Nuclear sclerosis, cortical opacities, posterior subcapsular

B. Posterior Segment Examination

• Vitreous clarity
• Optic disc (cup-to-disc ratio, pallor, excavation)
• Retinal vessels (tortuosity, narrowing, sheathing — signs of atherosclerotic/hypertensive disease)
• Macula (check for drusen, pigmentary changes — macular degeneration is age-associated)
• Peripheral retina

C. Additional Assessments

• Intraocular pressure (Applanation or Rebound tonometry)
• Pupil dilation (if not contraindicated) for comprehensive retinal assessment
• Visual field (if any concern about glaucoma or retinal disease)

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4. Mandatory Tests/Investigations

A. Ocular Imaging

1. Anterior segment photographs (if available)
   • Document arcus morphology
   • Baseline for comparison
2. Corneal topography/Pentacam (optional but useful)
   • Assess corneal regularity
   • Differentiate from other stromal opacities

B. Systemic Investigations (CRUCIAL — especially if arcus juvenilis or if risk factors present)

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5. Differential Diagnosis

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6. Systemic Association - What to Mention

• Age-related, usually innocent finding
• BUT still screen for dyslipidaemia, especially if family history of early CVD

• RED FLAG → mandatory lipid workup
• Associated with:
  • Familial hypercholesterolaemia (FH)
  • Familial dysbetalipoproteinemia (Type III hyperlipoproteinemia)
  • Other severe dyslipidaemias
  • Metabolic syndrome
  • Premature coronary artery disease

• Arcus senilis correlates with subclinical atherosclerosis
• Xanthelasma + arcus can indicate supravalvular aortic stenosis or severe systemic dyslipidaemia
• Increased risk of MI, stroke in patients with arcus + dyslipidaemia

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7. Management & Counseling

A. For Arcus Senilis (Age >40, Asymptomatic, Normal Lipids)

1. Reassurance — explain it is benign and does not affect vision
2. Cosmetic concern? → topical lubricants may provide temporary cosmetic improvement but permanent treatment not necessary
3. Lifestyle modification:
   • Heart-healthy diet (low saturated fat, Mediterranean diet)
   • Regular aerobic exercise
   • Smoking cessation
4. Monitoring: Annual eye exams to watch for progression and exclude other pathology

B. For Arcus Juvenilis or Abnormal Lipid Profile

1. Referral to cardiologist/internist for comprehensive cardiovascular risk assessment
2. Lipid-lowering therapy:
   • Statins (first-line, e.g., atorvastatin, rosuvastatin)
   • Ezetimibe if statin monotherapy insufficient
   • PCSK9 inhibitors if severe familial hypercholesterolaemia
   • Bempedoic acid or other agents based on lipid profile
3. Repeat lipid panel after 6-12 weeks on therapy to assess response
4. Long-term cardiovascular risk factor management:
   • Antihypertensive therapy if BP elevated
   • Glycaemic control if diabetic
   • Antiplatelet therapy if prior CVD or high 10-year risk

C. Corneal Degeneration Management

• If asymptomatic (most cases): No ocular treatment needed
• If associated dry eye: Artificial tears, lubricating ointment, punctal plugs, cyclosporine A
• If visual axis involved or symptomatic: Rarely needed; consider excimer laser PTK (phototherapeutic keratectomy) for Schnyder dystrophy if significant central opacification

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8. How to Close the Case

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9. Typical Exam Questions & Answers

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10. Visual Aid for Examiners

• (A) Arcus senilis — peripheral white band with clear zone
• (B) Vogt limbal girdle Type I — Swiss cheese pattern at limbus
• (C) Vogt limbal girdle Type II — smooth limbal band
• (D) Cornea farinata — fine central flour-like deposits
• (E) Crocodile shagreen — anterior polygonal opacities
• (F) West Indian punctate keratopathy — discrete round lesions with halo

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Key Takeaways for Your Viva

1. Arcus senilis = white peripheral stromal band + 1 mm clear limbal zone (pathognomonic)
2. Innocent in elderly, RED FLAG in young (screen for dyslipidaemia & CVD)
3. Does NOT affect vision (if vision loss present → think of other diagnosis)
4. Always assess for cardiovascular risk and refer appropriately
5. Know the other age-related degenerations to differentiate on slit lamp
6. Mention systemic investigations (lipid panel, glucose, TSH, renal function)
7. Management is primarily reassurance + cardiovascular risk reduction

BAND KERATOPATHY (K Opacity) - Case Presentation for MS Ophthalmology Practical/Viva

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1. Opening the Case - How to Start

• "This patient presents with band keratopathy secondary to chronic anterior uveitis from juvenile idiopathic arthritis (JIA)."
• "This is a case of band keratopathy in the setting of end-stage renal disease/hyperparathyroidism."
• "Incidental finding of band keratopathy on routine slit lamp examination in an otherwise healthy individual."

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2. History Taking - Key Questions

A. Chief Complaint & Duration

• Symptomatic or incidental finding?
  • "Do you have pain, foreign body sensation, or redness?"
  • "How long have you noticed the white band across your eyes?"
  • "Has your vision changed recently?"
• Onset: Gradual (weeks to months) vs sudden (typically gradual)
• Progression: Static vs slowly worsening

B. Ocular History (Most Important for Determining Cause)

C. Systemic History (Critical - especially if no obvious ocular cause)

D. Medications

• Topical drops used chronically (preservatives can cause toxicity)
• Systemic steroids (long-term use)
• Vitamin D supplements
• Calcium supplements
• Medications for hyperparathyroidism or hypercalcaemia

E. Risk Factors for Hypercalcaemia

• Sarcoidosis symptoms (cough, dyspnoea, constitutional symptoms)
• Recent immobilization (prolonged bed rest)
• Thyroid disease

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3. Clinical Examination - Present Systematically

A. Visual Acuity & Basic Assessment

• Corrected visual acuity in both eyes
• Ocular motility (usually normal unless associated uveitis)
• Pupils (look for posterior synechiae from chronic uveitis)

B. Slit Lamp Examination - CRITICAL (This is where diagnosis is made)

CLASSIC PRESENTATION OF BAND KERATOPATHY:

C. Detailed Slit Lamp Findings to Document

1. Right eye band keratopathy:
   • Location: Horizontal band in interpalpebral fissure
   • Extent: [specify which meridians involved — e.g., 3-9 o'clock most severe, extending toward 12 and 6]
   • Density: Mild/moderate/severe opacification
   • Swiss cheese pattern: Present/absent
   • Limbal zone: Clear zone present between band edge and limbus = [specify 1-2 mm]
   • Central involvement: None/mild/moderate/severe
   • Surface: Smooth/roughened/protruding
   • Epithelial defects: Present/absent (indicate if there are ulcerations from severe disease)
2. Associated findings:
   • Keratic precipitates (KP) → suggests chronic uveitis
   • Anterior chamber cells/flare → active or chronic uveitis
   • Posterior synechiae → chronic uveitis (especially JIA)
   • Iris abnormalities (nodules, atrophy)
   • Pupil reactivity and size
   • Corneal scarring beyond the band
   • Conjunctival injection (chemosis if severely inflamed)
3. Anterior chamber:
   • Depth
   • Inflammation (0-4+ scale)
   • Aqueous haze
4. Iris & Lens:
   • Synechiae (location and extent)
   • Iris color changes (heterochromia in JIA)
   • Cataract (anterior subcapsular, posterior subcapsular, cortical — common in JIA)

D. Posterior Segment (with dilated pupil)

• Vitreous inflammation (vitritis, snowball opacities if severe uveitis)
• Optic disc pallor or hyperemia
• Retinal vasculitis if present
• Macular edema (common in chronic uveitis)
• Peripheral retinal whitening or sheathing (vasculitis)

E. Intraocular Pressure

• Measure with applanation tonometry
• Important: Many band keratopathy cases have history of glaucoma; IOP may be elevated or patient may be on glaucoma medications

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4. Mandatory Clinical Tests/Investigations

A. Slit Lamp Photography

• Document band keratopathy appearance
• Baseline for monitoring

B. Anterior Segment OCT (if available)

• Delineate the depth of calcium infiltration
• Assess anterior stromal involvement
• Useful for follow-up after treatment

C. Systemic Investigations (CRITICAL — Must Do This)

• Investigations may be limited to confirming that systemic calcium is normal

D. Inflammatory Markers (if uveitis is suspected cause)

• ANA, RF, HLA-B27, ACE, TB-IGRA (depending on clinical suspicion)

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5. Classification & Causes - Present Clearly

Band Keratopathy Etiology Classification:

A. Ocular Causes (Most Common ~80% of cases)

B. Metabolic/Systemic Causes (Metastatic Calcification, ~20% of cases)

C. Hereditary/Genetic Causes

• Myotonic dystrophy (can have band keratopathy)
• Ichthyosis (familial band keratopathy)
• Familial band keratopathy (rare AR condition)

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6. Differential Diagnosis

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7. Management - How to Present to Examiner

A. Mild/Asymptomatic Band Keratopathy

1. Observation:
   • Many patients have no symptoms
   • Monitor every 3-6 months with slit lamp
2. Supportive care:
   • Artificial tears (preservative-free, 4-6 times/day)
   • Lubricating ointment at night (e.g., Lacri-Lube, Refresh PM)
   • Avoid topical irritants
3. Bandage soft contact lens (if mild foreign body sensation)

B. Moderate/Symptomatic Band Keratopathy (Foreign body sensation, mild vision loss)

1. Continue supportive care (as above)
2. Cycloplegic drops (e.g., cyclopentolate 1%) to reduce pain from ciliary spasm
3. Topical antibiotic ointment (e.g., erythromycin) to prevent epithelial defects
4. Topical NSAIDs (e.g., ketorolac q.i.d.) for pain control (use judiciously, max 2 weeks, risk of toxicity)
5. Consider bandage contact lens for comfort and epithelial protection
6. Systemic analgesics (e.g., acetaminophen ± codeine) for severe discomfort

C. Severe/Vision-Threatening Band Keratopathy (Central opacity, epithelial breakdown, significant pain)

PRIMARY TREATMENT: CHELATION WITH EDTA

1. Anesthesia: Topical anesthetic (proparacaine or tetracaine, 0.5%)
2. Epithelial debridement:
   • Use sterile #15 blade or spatula
   • Gently remove corneal epithelium overlying the calcium deposit
   • Avoid excessive scraping of stroma
3. EDTA Application:
   • Solution: Disodium EDTA 1.5-3.0% (or 3-4% per Wills Eye Manual)
   • Source: Compounding pharmacy (since commercially prepared ophthalmic EDTA is not routinely available)
   • Application method: Soak cellulose sponge or cotton-tipped applicator in EDTA solution
   • Duration: Apply and wipe over band for 15-20 minutes (some references say up to 60 minutes)
   • Endpoint: Continue until all visible calcium has been removed (turn to light, look for loss of white opacification)
4. Irrigation:
   • Thoroughly rinse with normal saline to remove EDTA solution
5. Post-treatment:
   • Topical antibiotic (e.g., erythromycin ointment or moxifloxacin drops)
   • Cycloplegic (cyclopentolate 1-2%)
   • Bandage soft contact lens (strongly recommended for comfort and epithelial protection)
   • Topical antibiotic drops (e.g., moxifloxacin q.i.d.) or ointment
   • Systemic analgesic (acetaminophen ± codeine)
6. Follow-up:
   • See patient in 3-5 days to assess epithelial healing
   • Examine every few days until epithelial defect heals (may take 1-2 weeks)
   • Remove bandage contact lens once epithelialized

• Multiple sessions may be necessary if calcium recurs
• MUST be at least 15-20 minutes of application for adequate chelation

ALTERNATIVE/ADJUNCTIVE TREATMENTS:

D. Treat Underlying Systemic Disease

• Hyperparathyroidism: Parathyroid surgery referral if confirmed
• ESRD: Optimize phosphate binders, vitamin D metabolism, PTH control with nephrologist
• Vitamin D toxicity: Discontinue supplements, increase fluids, reduce calcium intake
• Sarcoidosis: Systemic steroids or immunosuppression by pulmonologist
• Paget disease: Bisphosphonates for bone turnover control
• Multiple myeloma: Oncology referral

E. Monitor for Recurrence

• Follow-up timeline: Every 3-12 months depending on severity
• Re-chelation: Can be repeated if band keratopathy recurs
• Excimer laser PTK: Consider if recurrence is rapid or residual central scarring limits vision

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8. Typical Viva Questions & Model Answers

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9. How to Close the Case - Summary for Examiner

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10. Key Teaching Points for Your Exam

What Examiners Expect You to Know:

1. Band keratopathy = calcium in Bowman layer + anterior stroma
   • NOT in stroma alone (unlike other degenerations)
   • Level is DIAGNOSTIC
2. Horizontal band in INTERPALPEBRAL fissure
   • UV or air exposure pattern (sun-exposed area)
   • Bilateral and symmetric
3. Swiss cheese appearance (lucid spaces)
   • Diagnostic clue for band keratopathy
   • Differentiates from arcus senilis
4. Clear limbal zone (1-2 mm)
   • Important diagnostic feature
   • Separates band edge from limbus
5. Most common cause = Chronic anterior uveitis (JIA)
   • Testable: JIA → chronic uveitis → band keratopathy
   • Also: IK (syphilis historically), glaucoma, dry eye, phthisis
6. Systemic screening is MANDATORY
   • If no obvious ocular cause
   • Check: calcium, phosphate, PTH, renal function, vitamin D
   • Screen for hyperparathyroidism, ESRD, sarcoidosis
7. EDTA chelation is gold standard
   • 1.5-3% disodium EDTA
   • 15-20 minutes application
   • Post-chelation: bandage CL + antibiotic + cycloplegic
8. Management: Mild = observe; Moderate = supportive; Severe = EDTA ± excimer laser PTK
9. Differential diagnosis: Arcus senilis (lipid, diffuse border), Salzmann nodules (nodular, not band), lipid keratopathy (yellow hue), Vogt girdles (limbal, 3 & 9 o'clock)
10. Always mention underlying disease management to prevent recurrence

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Final Tip for Your Viva: