Key points

• Most often affects women age 60 to 70
• Usually idiopathic and related to vitreomacular traction (VMT) during posterior vitreous detachment
• Can also occur with high myopia or blunt trauma
• Bilateral involvement risk is clinically relevant (about 10% to 30% reported depending on cohort/setting)

Typical symptoms

• Blurred central vision in one eye
• Metamorphopsia (straight lines look wavy)
• Central scotoma
• Early/impending stages may be minimally symptomatic

Diagnosis

• OCT is the key test for confirmation and staging
• Fundus exam may show a round red foveal lesion with a cuff/halo
• Watzke-Allen slit-beam test may show a perceived break in the beam over a true full-thickness hole
• Differentiate from:
  • Lamellar macular hole
  • Macular pseudohole from epiretinal membrane
  • Chronic CME-related cystic changes
  • Solar retinopathy

Staging

• Stage 1: impending hole
• Stage 2: small full-thickness hole
• Stage 3: full-thickness hole, no complete PVD
• Stage 4: full-thickness hole with complete PVD

• Hole size (small, medium, large)
• Presence/absence of VMT
• Etiology (primary vs secondary)

Management

• Stage 1: often observation (many resolve spontaneously)
• Symptomatic full-thickness holes (especially stage 2+): pars plana vitrectomy with ILM peel and gas tamponade is standard of care
• Better outcomes when treated earlier, especially within about 6 months
• Post-op face-down positioning may be used (shorter protocols common with modern techniques)
• Ocriplasmin may be considered in selected early small VMT-related cases, but use is limited by variable efficacy and side effects
• Cataract progression after vitrectomy in phakic patients is very common

Prognosis

• Anatomical closure rates are high after surgery
• Vision often improves over months
• Smaller holes and shorter duration generally have better visual outcomes
• Some full-thickness holes can close spontaneously

Example images

Fundus stages of macular hole:

OCT appearance of macular hole:

• A quick PubMed search limited to the last 2 years for systematic reviews/meta-analyses on “macular hole treatment” returned no hits, so no immediate high-level contradiction signal was identified from that narrow filter.

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th ed, p. 626-629
• The Wills Eye Manual, p. 866-870

Macular Hole (15-mark answer)

Definition

Epidemiology

• Common in older adults, especially 60 to 70 years
• Female preponderance
• Usually unilateral at presentation, but fellow-eye risk exists (about 10% or more over time)

Etiology and Pathogenesis

1. Idiopathic (most common)
   Related to age-related vitreous changes and anomalous posterior vitreous detachment.
2. Vitreomacular traction (VMT)
   Persistent vitreofoveal adhesion causes anteroposterior and tangential traction, leading to foveal dehiscence.
3. Secondary causes
   • High myopia
   • Blunt ocular trauma
   • Less commonly cystoid macular edema-associated tractional changes

Classification

A. Gass Clinical Staging

• Stage 1: Impending hole (yellow spot/ring at fovea)
• Stage 2: Small full-thickness macular hole
• Stage 3: Full-thickness hole with cuff of subretinal fluid, without complete PVD
• Stage 4: Full-thickness hole with complete PVD

B. OCT-Based Classification (current practical approach)

• Based on:
  • Hole size (small, medium, large)
  • Presence/absence of vitreomacular traction
  • Etiology (primary/secondary)

Clinical Features

Symptoms

• Painless diminution of central vision
• Metamorphopsia
• Central scotoma
• Reading difficulty
• Early stages may be minimally symptomatic

Signs

• Round red foveal lesion
• Gray cuff/halo (subretinal fluid) around full-thickness hole
• Possible operculum
• Loss of normal foveal contour in VMT stages

Investigations

1. Optical Coherence Tomography (OCT)
   Gold standard for diagnosis, staging, and follow-up.
2. Amsler grid
   Detects central distortion.
3. Watzke-Allen test
   Thinning/break in slit beam perception suggests true full-thickness hole.
4. Fundus biomicroscopy
   For morphology and associated vitreoretinal interface abnormalities.
5. FA/FAF (selected cases)
   Usually not required when OCT is diagnostic.

Differential Diagnosis

• Lamellar macular hole
• Macular pseudohole (epiretinal membrane)
• Chronic CME with central cystic change
• Solar retinopathy
• Other foveal lesions mimicking a hole

Management

1. Observation

• Stage 1 holes are often observed because spontaneous resolution can occur.
• Small spontaneous closure can also occur in some full-thickness holes.

2. Pharmacologic vitreolysis

• Ocriplasmin in selected VMT/small early holes
• Limited use due to variable efficacy and adverse effects

3. Surgical treatment (standard for symptomatic full-thickness holes)

• Pars plana vitrectomy + ILM peeling + gas tamponade
• Relieve traction, promote hole closure
• Better outcomes when operated early (preferably within ~6 months of onset)
• Postoperative prone positioning may be advised for selected duration

Prognosis

• High anatomical closure rates after surgery
• Visual recovery occurs over months
• Better visual outcomes with:
  • Smaller hole size
  • Shorter symptom duration
  • Earlier surgery
• Cataract progression in phakic eyes after vitrectomy is common

Complications

• Persistent/recurrent hole
• Cataract progression
• Retinal detachment (uncommon but important)
• Other vitrectomy-related complications

Follow-up

• Monitor with serial OCT
• Counsel regarding symptoms of retinal detachment
• Periodic fellow-eye assessment due to bilateral risk

Short conclusion

CMV Retinitis (15-mark answer)

Definition

Etiology and Risk Factors

• Causative agent: Human cytomegalovirus (HHV-5)
• Major risk groups:
  • HIV with very low CD4 count (especially <50 cells/µL)
  • Solid organ/hematopoietic stem cell transplant
  • Prolonged corticosteroids, chemotherapy, biologics, immunosuppressants
  • Hematologic malignancy, congenital immunodeficiency

Pathogenesis

• CMV reactivation occurs in profound immunosuppression
• Hematogenous spread to retina causes full-thickness retinal necrosis
• Associated occlusive vasculitis and hemorrhage
• Lesions spread centrifugally along vessels if untreated
• Healed lesions become atrophic scars, predisposing to retinal breaks and detachment

Clinical Features

Symptoms

• Floaters
• Blurred vision
• Scotoma
• Photopsia
• Painless progressive visual loss
  (Early peripheral disease may be asymptomatic)

Signs (Fundus)

• Yellow-white granular retinal necrosis with retinal hemorrhages
  classic “pizza pie” or “cottage cheese with ketchup” appearance
• Perivascular whitening/sheathing
• Minimal anterior chamber/vitreous inflammation compared with lesion extent
• May be unilateral initially, with risk of fellow-eye involvement if untreated

Clinical Patterns

1. Fulminant/edematous form
   Dense hemorrhagic necrotizing lesions, often posterior pole
2. Indolent/granular form
   Peripheral granular lesions with slower progression
3. Frosted branch angiitis-like vasculitis (less common)

Diagnosis

1. Dilated fundus examination (cornerstone)
2. Wide-field fundus photography for documentation
3. OCT for macular involvement/complications
4. Aqueous/vitreous PCR (CMV DNA) when diagnosis is uncertain or atypical
5. HIV testing/CD4 count and systemic immunologic workup where relevant

Differential Diagnosis

• Acute retinal necrosis (HSV/VZV)
• Progressive outer retinal necrosis
• Toxoplasma retinochoroiditis
• Syphilitic retinitis
• Candida endophthalmitis
• Intraocular lymphoma

Treatment

1) Anti-CMV induction therapy

• Valganciclovir (oral) commonly used if sight-threatening disease is absent
• IV ganciclovir for severe/bilateral/systemic disease
• Foscarnet (alternative or resistant/intolerant cases)
• Cidofovir less favored due to toxicity profile

2) Local therapy for sight-threatening lesions

• Intravitreal ganciclovir or foscarnet, often combined with systemic therapy for rapid local control

3) Maintenance and immune restoration

• Continue maintenance therapy until immune recovery (in HIV, sustained CD4 improvement on ART)
• Start/optimize ART in HIV patients (timing individualized)
• Coordinate with physician/infectious disease specialist

Monitoring and Follow-up

• Frequent retinal exams during induction (often weekly initially)
• Watch for progression at lesion borders, new lesions, fellow-eye disease
• Hematologic and renal monitoring for drug toxicity
• Long-term follow-up for structural sequelae

Complications

• Rhegmatogenous retinal detachment (major cause of severe visual loss)
• Optic atrophy
• Macular scarring/edema
• Immune recovery uveitis after ART initiation
• Drug adverse effects:
  • Ganciclovir/valganciclovir: neutropenia, thrombocytopenia
  • Foscarnet: nephrotoxicity, electrolyte disturbances
  • Cidofovir: nephrotoxicity, uveitis/hypotony

Prognosis

• Visual outcome depends on:
  • Lesion location (macula/optic nerve involvement worse)
  • Speed of diagnosis and treatment
  • Degree of immune reconstitution
• Prognosis has improved markedly in the ART era, but relapse and late complications remain significant.

Prevention

• Early HIV diagnosis and effective ART
• In transplant and high-risk patients: virologic monitoring and pre-emptive CMV strategies
• Patient education to report new floaters, field defects, or blurred vision immediately

Short conclusion