CASE PRESENTATION

PATIENT IDENTIFICATION

CHIEF COMPLAINT

HISTORY OF PRESENTING ILLNESS

PAST OCULAR HISTORY

• History of diabetes mellitus / hypertension / central retinal vein occlusion (CRVO) / ocular ischaemic syndrome - to be elicited
• Previous ocular surgeries or treatments - to be elicited

PAST MEDICAL HISTORY

• Diabetes mellitus, hypertension, cardiovascular disease - to be elicited

DRUG HISTORY / ALLERGIES

• Current medications - to be elicited

FAMILY HISTORY / SOCIAL HISTORY

• Significant family history of glaucoma or diabetes - to be elicited

GENERAL EXAMINATION

• Conscious, alert, oriented
• Built and nourishment: to be recorded
• No pallor, icterus, cyanosis, clubbing, lymphadenopathy, oedema

OCULAR EXAMINATION

Visual Acuity (Unaided)

External Examination

Key Findings

Note: NVI is present in the LE without NVA on slit-lamp assessment; formal gonioscopy is required to fully evaluate the angle for fibrovascular membrane formation and degree of angle closure.

Posterior Segment

• Optic disc: Cup-disc ratio to be recorded; glaucomatous cupping likely in LE
• Macula / vessels / periphery: dilated fundus examination required to identify the underlying aetiology (diabetic retinopathy, ischaemic CRVO, CRAO, ocular ischaemic syndrome)

DIAGNOSIS

STAGING (Wills Eye / Kanski Classification)

PATHOGENESIS (Brief)

1. Ischaemic CRVO (35-50% of NVG cases) - NVG classically occurs ~3 months post-CRVO ("100-day glaucoma")
2. Diabetic retinopathy with retinal ischaemia (10-15%)
3. Ocular ischaemic syndrome / carotid occlusive disease
4. Central retinal artery occlusion

INVESTIGATIONS PLANNED

1. IOP measurement (Goldmann applanation tonometry)
2. Gonioscopy - to assess degree of angle closure, NVA, PAS formation
3. Dilated fundus examination - to identify underlying retinal aetiology; optic disc assessment
4. Fluorescein angiography (FFA) - to confirm retinal ischaemia, delineate extent, plan PRP
5. B-scan ultrasonography (if posterior segment not visualisable) - to rule out retinal detachment or intraocular tumour
6. Anterior segment OCT - angle assessment
7. Systemic workup: FBS/PPBS/HbA1c, lipid profile, BP monitoring, carotid Doppler (if ocular ischaemic syndrome suspected), ECG

MANAGEMENT

Immediate / Medical

1. Reduce inflammation and pain:
   • Topical steroid: prednisolone acetate 1% q1-6h
   • Cycloplegic: atropine 1% t.i.d. (also resists synechiae formation and increases uveoscleral outflow)
2. Lower IOP (if elevated):
   • Topical beta-blocker: timolol 0.5% b.i.d.
   • Topical alpha-2 agonist: brimonidine 0.1-0.2% b.i.d.-t.i.d.
   • Topical/systemic CAI: dorzolamide 2% b.i.d.-t.i.d. and/or acetazolamide 500 mg b.i.d. orally (use with caution if diabetic - risk of renal dysfunction)
   • If urgent IOP reduction needed: mannitol 1-2 g/kg IV over 45 minutes
   • Avoid: miotics (pilocarpine) - contraindicated; prostaglandins - avoid in acute phase
3. Anti-VEGF therapy:
   • Intravitreal bevacizumab (Avastin) 1.25 mg/0.05 ml - causes rapid regression of iris NV, particularly useful if angle not yet fully closed; buys time for PRP to take effect; may delay NVG onset by up to 18 months in CRVO patients
   • Note: effect is temporary; repeat injections likely required - Kanski's, p. 403

Definitive (Treat the Underlying Cause)

1. Panretinal photocoagulation (PRP): If retinal ischaemia is the driver and angle is still open, PRP reduces the angiogenic drive. If cornea is oedematous/hazy, lower IOP first and perform PRP once the cornea clears.

Surgical (If IOP Uncontrolled / Advanced Disease)

1. Glaucoma drainage device (tube-shunt surgery): Consider when NV is inactive and medical control fails; only if useful vision exists to preserve. Risk of postoperative bleeding.
2. Trabeculectomy with MMC (+ adjunctive 5-FU postoperatively)
3. Cyclodiode / cyclophotocoagulation (transscleral): If medical IOP control not possible or in eyes with poor visual potential; lowers IOP and improves comfort.

Given Poor Visual Prognosis (PL only in LE)

1. Given VA of PL in the LE, the primary goal shifts toward pain relief and comfort. Topical steroids and cycloplegics may be sufficient. IOP reduction may not fully alleviate pain in chronic NVG. If all else fails: retrobulbar alcohol injection (pain relief; risk of permanent ptosis), or enucleation/evisceration as a last resort. - Wills Eye Manual, p. 614-615

Fellow Eye (RE) Management

1. Regular monitoring of the RE - VA 6/18 with no NVI currently. Identify and treat systemic cause aggressively to reduce risk to the fellow eye.
2. Manage systemic disease: glycaemic control (HbA1c target), BP control, lipid management, carotid evaluation.

PROGNOSIS

• Poor for the LE - VA of PL at presentation is a poor prognostic indicator (factors predicting poor visual outcome: VA <6/60, IOP >35 mmHg at presentation, young age)
• Best achievable outcome is often a blind but comfortable eye
• Prevalence of blindness in affected eye: 25-50% depending on follow-up duration
• Life expectancy is significantly reduced (~50% of expected) in NVG patients due to underlying systemic disease - aggressive systemic management is essential
• Risk to the fellow eye remains; early identification and treatment of the underlying aetiology is key

SUMMARY

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 401-404)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 610-615)
• He CZ et al. "Anti-VEGF combined with Ahmed glaucoma valve implantation in neovascular glaucoma: a systematic review and meta-analysis." Front Med (Lausanne), 2024. [PMID: 39144652]

HISTORY OF PRESENTING ILLNESS

Onset and Duration

Character and Progression

• Redness of the left eye - gradual onset, persistent, not associated with discharge
• Pain in the left eye - aching in character, moderate to severe intensity, aggravated towards the end of the day; she also describes occasional frontal headache on the same side
• Coloured halos around lights at night (suggestive of corneal oedema secondary to raised IOP)
• Photophobia (sensitivity to light) in the left eye
• No improvement with glasses or blinking

Associated Systemic History

• Diabetes mellitus for approximately [X years] - on irregular oral hypoglycaemic agents / insulin (to be elicited), with poor glycaemic control suspected
• Hypertension - [present/absent] (to be elicited), on antihypertensives
• She denies any history of sudden loss of vision in the past (which would suggest a prior acute vascular occlusive event), though she describes a period several months ago when the left eye vision seemed to "go suddenly darker" before progressive decline - this may represent an unnoticed ischaemic CRVO or CRAO event
• No history of prior ocular trauma, ocular surgery, or prolonged use of steroid eye drops
• No known history of thyroid disease, autoimmune conditions, or malignancy

Ocular History

• No prior spectacle use / uses glasses for distance or near (to be elicited)
• No history of prior glaucoma diagnosis, laser treatment, or intravitreal injections in either eye
• No history of previous acute red eye episodes with pain and nausea (which would suggest prior acute angle closure attacks)
• No history of flashes, floaters, or curtain-like loss of vision (which would suggest retinal detachment)

Review of Systems (Relevant)

Typical Clinical Presentation Profile (NVI/NVG)

1. Prior ischaemic CRVO: History of a sudden painless loss of vision (like "a shadow falling over the eye") weeks to months ago, often preceded by vascular risk factors (HTN, DM, hyperlipidaemia, hyperviscosity). NVG typically appears ~3 months later - the classic "100-day glaucoma."
2. Proliferative diabetic retinopathy: Long-standing poorly controlled DM with prior history of floaters, vitreous haemorrhage (sudden dark spots or complete blackout), or laser treatment. NVG represents end-stage ischaemic retinopathy.
3. Ocular ischaemic syndrome (carotid stenosis): History of ipsilateral dull anterior segment pain that paradoxically worsens on bright light exposure (venous stasis), amaurosis fugax, and associated atherosclerotic vascular disease (angina, claudication).
4. Advanced NVG presentation (as in this patient): The patient presents late when vision is already reduced to PL. Pain, redness, and photophobia dominate the history by this stage. The iris vessels are no longer subtle - NVI is grossly visible. This often reflects delayed presentation or a rapidly progressing underlying cause.

DIFFERENTIAL DIAGNOSIS

For Left Eye NVI (Rubeosis Iridis) with Raised IOP / Glaucoma

1. Inflammatory (Uveitic) Glaucoma

• Similarities: Red eye, pain, photophobia, raised IOP, anterior chamber cells and flare, posterior synechiae
• Differences: In uveitic glaucoma, the angle is open with no true neovascularization; iris vessels, when dilated, run in a normal radial pattern (not the irregular, non-radial tortuous pattern of rubeosis). The cause is intraocular inflammation, not retinal ischaemia. Keratic precipitates may be present.
• Key distinguishing feature: NVI is absent in pure uveitic glaucoma; dilated normal-pattern iris vessels may be seen but these are physiological dilatation, not neovascularization.
• Wills Eye Manual, p. 612

2. Primary Acute Angle Closure Glaucoma (AACG)

• Similarities: Acute pain, redness, corneal oedema, markedly raised IOP, decreased vision
• Differences: AACG occurs in hypermetropic eyes with shallow anterior chambers; precipitated by mydriasis. No NVI or NVA. Gonioscopy shows appositional angle closure without fibrovascular membrane. Corneal epithelial oedema with "steamy" cornea. Fixed, mid-dilated, oval pupil. No underlying retinal ischaemia.
• Key distinguishing feature: No rubeosis iridis; structural angle closure without neovascularization.

3. Pseudoexfoliation Glaucoma (PXF Glaucoma)

• Similarities: Elderly patient, raised IOP, progressive vision loss, advanced glaucomatous optic neuropathy
• Differences: White flaky material on anterior lens capsule, pupillary margin, and corneal endothelium ("dandruff-like" deposits). Open-angle on gonioscopy with pigment deposition on TM (Sampaolesi line). No NVI. Typically unilateral or asymmetric.
• Key distinguishing feature: Pseudoexfoliative material on slit-lamp; no rubeosis.

4. Fuchs Heterochromic Iridocyclitis (Fuchs Uveitis Syndrome)

• Similarities: Unilateral, iris vessels may be abnormal, raised IOP possible, cataract
• Differences: Typically young to middle-aged adults. Characteristic fine stellate KPs scattered over entire endothelium. Heterochromia (lighter affected eye). Fine iris vessels may be present but differ from true rubeosis - they are delicate, non-tortuous, and bleed spontaneously on paracentesis (Amsler sign). Low-grade anterior uveitis without posterior synechiae (despite AC reaction).
• Key distinguishing feature: Fine randomly distributed KPs, heterochromia, no posterior synechiae, Amsler sign.

5. Iridocorneal Endothelial (ICE) Syndrome

• Similarities: Secondary angle closure, raised IOP, unilateral, iris changes
• Differences: Typically affects women aged 20-50 years. Abnormal corneal endothelium (beaten-silver appearance). Progressive iris atrophy, corectopia, polycoria. No NVI. Angle closed by abnormal endothelial membrane proliferating across the angle. No underlying retinal ischaemia.
• Key distinguishing feature: Specular microscopy shows abnormal endothelial cells; younger age group; no NVI.

6. Ocular Ischaemic Syndrome (OIS) without NVG (early)

• Similarities: Rubeosis iridis in up to 90% of cases, low or paradoxically normal IOP despite rubeosis (due to poor ciliary body perfusion), dilated tortuous retinal veins, dot-blot haemorrhages in mid-periphery
• Differences: IOP may actually be low despite NVI because ciliary body ischaemia reduces aqueous production. Spontaneous arterial pulsation at disc on ophthalmoscopy. History of carotid artery disease, amaurosis fugax.
• Key distinguishing feature: Low or normal IOP with NVI; carotid stenosis on Doppler.
• Kanski's, p. 403 (Anterior segment section)

7. Chronic Angle Closure Glaucoma (CACG)

• Similarities: Progressive, often silent vision loss, raised IOP, advanced optic neuropathy, PAS on gonioscopy
• Differences: No NVI or NVA. Peripheral anterior synechiae from pupil block mechanism, not fibrovascular contraction. The angle closes in a patchy, non-circumferential pattern early on. No underlying retinal ischaemia.
• Key distinguishing feature: Absence of rubeosis; angle closure by PAS without fibrovascular membrane.

8. Diabetic Iritis (without NVG)

• Similarities: Diabetic patient, anterior chamber flare and cells, iris changes
• Differences: Mild anterior uveitis secondary to metabolic disturbance. Normal iris vasculature. IOP may be normal or mildly elevated. No NVI, no angle neovascularization. Responds to topical steroids and glycaemic control.
• Key distinguishing feature: No rubeosis; normal gonioscopy; mild uveitic picture only.

Summary Table: Differential Diagnosis at a Glance

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 401-404)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 610-615)

CASE PRESENTATION

PATIENT IDENTIFICATION

CHIEF COMPLAINT

HISTORY OF PRESENTING ILLNESS

Background of Diagnosis

On This Visit (Regular Follow-Up)

• No acute pain or redness in either eye since last visit
• No sudden or progressive blurring of vision; vision has remained stable and functional bilaterally
• No episodes of coloured halos (rainbow rings) around lights - which would indicate intermittent corneal oedema from IOP spikes
• No frontal headaches specifically on waking or in the evening
• No nausea or vomiting
• No episodes of dim or misty vision ("like looking through a smoke-filled room") that spontaneously resolved - which would suggest subacute intermittent angle closure episodes

Symptoms to Actively Elicit in PACS Follow-Up

Past Ocular History

• Diagnosed as BE PACS on gonioscopy - date of initial diagnosis: (to be noted)
• Laser Peripheral Iridotomy (LPI) status: (to be recorded - whether performed, patent, bilateral) - per ZAP trial guidelines, LPI may have been offered depending on individual risk assessment
• No prior episodes of acute angle closure
• No prior intraocular surgery
• No history of trauma to either eye
• Spectacle history: likely hypermetropic correction (to be confirmed) - hypermetropia is a strong anatomical risk factor for PACS

Past Medical History

• Systemic conditions such as diabetes mellitus, hypertension - to be elicited; relevant as they may require frequent dilated fundus examinations, which can precipitate acute closure in a PACS eye without a patent iridotomy
• No known sulfonamide allergy (relevant if acetazolamide is ever required)

Drug History

• Current ocular medications: (e.g., pilocarpine 1% b.d. prophylaxis if prescribed)
• Systemic medications: assess for any mydriatic-risk drugs (anticholinergics, antidepressants, antihistamines, sympathomimetics)
• No known drug allergies

Family History

• Family history of angle closure glaucoma or acute glaucoma - first-degree relatives have an increased risk; this should be screened for

Social History

• Retired; independent daily activities
• Literacy/awareness about symptoms of acute angle closure - patient should be educated to present immediately if acute symptoms develop

GENERAL EXAMINATION

• Conscious, alert, cooperative, well-oriented
• No acute distress
• Built and nourishment: adequate
• No pallor, icterus, cyanosis, clubbing, lymphadenopathy, or pedal oedema

OCULAR EXAMINATION

Visual Acuity (Unaided / Best Corrected)

External and Anterior Segment Examination

Intraocular Pressure (IOP)

Gonioscopy (Key Diagnostic Finding)

Gonioscopy is the gold standard for PACS diagnosis. The hallmark is posterior trabecular meshwork ITC in ≥3 quadrants WITHOUT PAS, raised IOP, or optic nerve damage. - Kanski's Clinical Ophthalmology, 10th ed., p. 389

Posterior Segment (Dilated Fundus Examination)

DIAGNOSIS

1. Gonioscopy showing ITC in ≥3 quadrants
2. No PAS
3. Normal IOP
4. Normal optic disc and visual fields

RISK FACTORS (Present in This Patient)

DIFFERENTIAL DIAGNOSIS

For Bilateral Narrow / Occludable Angle with Normal IOP and Vision

1. Primary Angle Closure (PAC)

• Similarities: Narrow angles on gonioscopy, ITC in ≥3 quadrants
• Differences: PAC is distinguished from PACS by the presence of raised IOP and/or PAS in addition to ITC. In this patient, IOP is normal and no PAS are seen on gonioscopy, placing her in PACS not PAC.
• Key distinction: Raised IOP or PAS = PAC; absence of both = PACS.

2. Primary Angle Closure Glaucoma (PACG)

• Similarities: Narrow angles, ITC on gonioscopy
• Differences: PACG requires glaucomatous optic neuropathy (increased CDR, rim thinning, RNFL defect, visual field loss). This patient has normal optic discs and 6/6 P vision bilaterally with no field defect.
• Key distinction: Glaucomatous disc and field changes are absent in PACS.

3. Plateau Iris Configuration

• Similarities: Narrow angle on gonioscopy; angle closure despite anatomically normal or only mildly shallow central AC depth; female sex
• Differences: Plateau iris is characterised by a flat or minimally convex central iris plane (unlike the convex iris bowing of pupillary block). The angle closure occurs due to anteriorly rotated/positioned ciliary processes, not a small eye. A "double hump" sign on indentation gonioscopy is characteristic. Anterior chamber depth centrally is normal or near-normal.
• Key distinction: AS-OCT or UBM showing anteriorly rotated ciliary processes; LPI does not fully open the angle (persistence of angle closure after iridotomy = plateau iris syndrome).

4. Secondary Angle Closure - Lens-Induced (Phacomorphic)

• Similarities: Narrow angle, older patient, occludable angle
• Differences: In phacomorphic glaucoma, the angle closure is caused by an intumescent or swollen lens (often due to rapidly maturing cataract or hypermature cataract). The IOP is typically significantly elevated, and the AC is acutely very shallow due to the increased lens volume pushing the iris anteriorly. There is a history of rapidly progressing cataract and acute pain.
• Key distinction: IOP is elevated; lens appears swollen/intumescent; acute presentation.

5. Secondary Angle Closure - Pseudoexfoliation Syndrome

• Similarities: Older female patient; gonioscopy may show narrow/occludable angle
• Differences: Pseudoexfoliation (PXF) produces characteristic white flaky deposits on the anterior lens capsule, pupillary margin (classic "double-ring sign"), and corneal endothelium. PXF can cause secondary angle closure by zonular weakness and anterior lens movement, or by TM obstruction with PXF material. No PXF material is seen in pure PACS.
• Key distinction: PXF deposits on slit-lamp, Sampaolesi line on gonioscopy.

6. Secondary Angle Closure - Uveitic (Iris Bombe)

• Similarities: Shallow AC, occludable-appearing angle
• Differences: 360-degree posterior synechiae from chronic uveitis cause iris bombe, where aqueous trapped in the posterior chamber pushes the entire peripheral iris forward against the TM. AC inflammation (KPs, cells, flare, posterior synechiae) is prominent. The angle is closed by bowed-out peripheral iris. History of uveitis is usually present.
• Key distinction: 360-degree posterior synechiae causing iris bombe; active or chronic intraocular inflammation; KPs.

7. Nanophthalmos / Microphthalmos

• Similarities: Bilateral very shallow AC, narrow angles, high hypermetropia, short axial length
• Differences: Nanophthalmos is defined by axial length <20 mm with a small but structurally normal eye. The entire eye is reduced in size. Often associated with high hypermetropia (>8D), thick sclera, uveal effusions, and retinal folds. The angle closure tendency is extreme.
• Key distinction: Very short axial length on biometry; very high hypermetropia; thick sclera on B-scan.

8. Iridocorneal Endothelial (ICE) Syndrome

• Similarities: Angle changes visible on gonioscopy; may cause secondary angle closure
• Differences: ICE syndrome typically affects young to middle-aged women (not elderly). Characterised by abnormal corneal endothelium (beaten-silver or hammered appearance on specular microscopy), iris atrophy, corectopia, and secondary angle closure from an endothelial membrane growing across the angle. Typically unilateral. This patient is bilateral and elderly.
• Key distinction: Unilateral; abnormal endothelium on specular microscopy; iris structural changes; younger age.

9. Malignant Glaucoma (Aqueous Misdirection) - Post-operative

• Similarities: Very shallow AC bilaterally, raised IOP
• Differences: Malignant glaucoma follows intraocular surgery (typically trabeculectomy or cataract surgery) and presents with paradoxical shallowing of the AC despite a patent iridotomy. IOP is typically elevated. Not applicable here unless the patient had prior surgery.
• Key distinction: Post-surgical history; patent PI; paradoxical flat AC.

Summary Table: Differential Diagnosis at a Glance

MANAGEMENT PLAN (At This Follow-Up)

1. Gonioscopy - repeat to check for any new PAS or progression of ITC
2. IOP measurement (Goldmann applanation tonometry) - confirm within normal limits
3. Dilated fundus exam - assess CDR, RNFL, disc appearance; compare with baseline
4. Visual field testing (Humphrey/Goldmann) - to confirm no field defects
5. AS-OCT / van Herick grading - document angle width and anterior chamber depth
6. LPI (Laser Peripheral Iridotomy) status check - confirm patency of prior iridotomy if performed; the ZAP trial indicates LPI has a small but meaningful prophylactic benefit and should be offered to highest risk PACS patients (symptoms of intermittent closure, need for frequent dilation, occupational risk, systemic mydriatic drugs)
7. Patient education - counsel on symptoms of acute angle closure (pain, redness, halos, vomiting) and instruct to present immediately if these occur; avoid triggers (dark environments, prone reading, mydriatic medications)
8. Systemic review - update medication list; screen for drugs that can precipitate closure

PROGNOSIS

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 388-396)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 610-612)
• He CZ et al. "Anti-VEGF combined with Ahmed glaucoma valve implantation in neovascular glaucoma." Front Med (Lausanne) 2024 [PMID: 39144652] (for contextual NVG reference from prior case)

CASE PRESENTATION

PATIENT IDENTIFICATION

CHIEF COMPLAINT

HISTORY OF PRESENTING ILLNESS

Onset and Duration

Character of Pain

• Deep, boring, aching in nature - not superficial or gritty
• Severe in intensity, disproportionate to the degree of external redness
• Radiates to the right temple, forehead, brow, and jaw on the same side
• Wakes her from sleep in the early hours of the morning - a classic and important feature
• Worsens with eye movement and with gentle touch/pressure over the globe
• Responds poorly to common analgesics (paracetamol / over-the-counter pain relief)
• Improves partially later in the day, though persistent through the episode

Recurrent Episodes

• The eye was very tender to touch
• Vision was slightly blurred during attacks but not permanently reduced
• Redness did not blanch with topical vasoconstrictors (she was told at a prior visit that the phenylephrine test was negative - blood vessels did not blanch with 10% phenylephrine drops, confirming deep scleral vessel involvement rather than superficial episcleral congestion)
• Episodes resolved partially with anti-inflammatory treatment but recurred

Associated Ocular Symptoms

• Blurring of vision during acute attacks (VA 6/9 unaided, corrects to 6/6 BCVA - no permanent structural visual loss at present)
• Photophobia during episodes
• Lacrimation (watering of the eye)
• No discharge (no mucopurulent element suggesting infective conjunctivitis)
• No halos around lights (no corneal oedema suggesting raised IOP as primary feature)
• No floaters or flashes (no posterior segment involvement currently)
• Left eye unaffected in all episodes

Systemic History - Directed Review (Critical for Establishing Aetiology)

1. Joint History - Rheumatoid Arthritis

• The patient gives a history of symmetrical joint pain and swelling predominantly involving the small joints of both hands and wrists for [X years]
• Morning stiffness lasting more than 1 hour, gradually improving with activity
• Joints affected include MCPs, PIPs, wrists bilaterally - symmetrical distribution
• She reports gradual deformity of the fingers over time
• History of RA diagnosed [X years] ago, currently on DMARDs/NSAIDs (to be confirmed)
• RA is the most common systemic association of scleritis - all forms of immune-mediated scleritis have been described in RA, and the clinical course tends to be more aggressive - Kanski's, p. 312

2. Respiratory Symptoms - Cough and Shortness of Breath

• The patient reports intermittent dry cough for approximately 3-4 years, not associated with fever or sputum production
• Associated with shortness of breath on exertion (SOB), gradually progressive
• No haemoptysis
• No known TB contact; BCG vaccination status unclear
• Differential significance: These respiratory symptoms in the context of scleritis raise the possibility of:
  • Granulomatosis with polyangiitis (GPA/Wegener's) - pulmonary involvement + scleritis + urinary symptoms (cANCA positive in >90% active disease)
  • Sarcoidosis - pulmonary involvement, ocular disease, urinary manifestations
  • RA-associated interstitial lung disease - a recognized extra-articular manifestation of RA
  • Systemic Lupus Erythematosus (SLE) - pleuritis/pleural effusion causing breathlessness

3. Urinary Symptoms - 3-4 Years Duration

• The patient reports urinary symptoms for 3-4 years: (nature to be clarified - haematuria, frequency, dysuria, frothy urine, reduced output)
• Haematuria would raise concern for granulomatosis with polyangiitis (GPA) with renal involvement, or SLE nephritis
• Proteinuria / frothy urine would suggest lupus nephritis or vasculitis-associated glomerulonephritis
• Dysuria / frequency alone may suggest recurrent UTI, though in the systemic context must prompt renal evaluation
• This triad of scleritis + respiratory + urinary symptoms has a high index of suspicion for GPA

4. Skin Rash

• The patient gives a history of skin rash (nature, distribution, onset to be elicited):
  • Malar/butterfly rash over the cheeks and nose, worse in sunlight → SLE (up to 70% skin involvement, characteristic butterfly rash)
  • Purpuric or petechial rash on lower limbs → vasculitis (GPA, PAN)
  • Erythema nodosum (tender nodules on shins) → sarcoidosis, inflammatory bowel disease, reactive arthritis
  • Discoid plaques → discoid lupus
  • Psoriatic plaques → psoriatic arthritis with scleritis
• The specific character of the rash should be documented with a dermatology review if needed

Summary of Multi-System Involvement (Suspected Diagnosis Framework)

Past Ocular History

• Recurrent similar episodes of right eye pain and redness
• Prior treatment with topical / systemic NSAIDs or steroids (to be confirmed)
• No prior ocular surgery or trauma
• No history of herpes zoster ophthalmicus (nodular scleritis is disproportionately common post-HZO)
• No contact lens use
• No history of pterygium surgery or scleral buckling

Past Medical / Surgical History

• RA - on treatment (verify current DMARDs - methotrexate, hydroxychloroquine, sulfasalazine, biologics)
• Any prior diagnosis of connective tissue disease, vasculitis, or autoimmune condition
• No prior TB, no prior renal disease on record
• No prior surgical history

Drug History

• Current DMARDs / NSAIDs for RA (to be listed)
• Note: NSAIDs are the first-line treatment for non-necrotizing scleritis; check if already on adequate doses
• Hydroxychloroquine (RA drug) - requires annual macular screening
• Allergies: to be elicited

Family History

• Family history of autoimmune disease, RA, SLE

Social History

• Married; housewife
• No smoking (relevant to exclude RA-ILD aggravation and sarcoidosis)
• No significant occupational exposure

GENERAL EXAMINATION

• Conscious, alert, well-oriented
• No acute distress; mildly uncomfortable due to right eye pain
• Built and nourishment: adequate
• Hands: Assess for symmetrical joint swelling, MCP/PIP involvement, ulnar deviation, Boutonniere/Swan neck deformities consistent with RA
• Skin: Assess for rash, erythema nodosum, purpura, photosensitive lesions
• Cervical lymphadenopathy: assess (sarcoidosis, lymphoma)
• Respiratory: Air entry, crepitations, wheeze (ILD/sarcoid/GPA)
• No pedal oedema

OCULAR EXAMINATION

Visual Acuity

External Examination

Phenylephrine Test (Key Diagnostic Test)

Interpretation: Non-blanching of deep scleral vessels with 10% phenylephrine confirms inflammation at the level of the deep episcleral/scleral vascular plexus, distinguishing scleritis from episcleritis. - Kanski's, p. 306

IOP, Posterior Segment

• IOP: to be measured (may be elevated in active scleritis)
• Dilated fundus examination: to assess for posterior scleritis signs (choroidal folds, disc oedema, exudative RD, retinal striae)
• B-scan: if posterior scleritis suspected (T-sign - fluid in sub-Tenon space)

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS

I. Ocular Differential Diagnosis - Conditions Mimicking Scleritis

1. Episcleritis

• Similarities: Acute-onset red eye, mild discomfort, interpalpebral distribution, can be recurrent, may be associated with RA
• Differences: Episcleritis involves only the superficial episcleral vessels - the congestion is bright red, not violaceous. Pain is mild or absent (not the deep, boring, sleep-disturbing pain of scleritis). Vision is normal. Most importantly, vessels blanch with topical 2.5-10% phenylephrine - the key distinguishing test. Episodes are shorter (days to 3 weeks), self-limiting, and usually respond to topical treatment alone.
• Key distinction: Phenylephrine blanching positive; mild pain; no deep scleral involvement; bright red rather than violaceous hue.

2. Acute Anterior Uveitis (Iridocyclitis)

• Similarities: Painful red eye, photophobia, reduced VA, recurrent attacks, association with systemic autoimmune disease (HLA-B27 spondyloarthropathies, RA, sarcoidosis)
• Differences: In uveitis, the redness is circumcorneal/ciliary flush (deep limbal injection). Pain is photophobia-driven and worsens when light enters the eye. KPs, anterior chamber cells and flare, posterior synechiae are the hallmarks. The sclera itself is not inflamed. Phenylephrine does not differentiate as meaningfully. VA reduction is more pronounced.
• Key distinction: KPs and AC cells/flare on slit lamp; ciliary flush pattern; no scleral nodularity or tenderness.

3. Acute Angle Closure Glaucoma (AACG)

• Similarities: Severe unilateral ocular pain, redness, reduced vision, photophobia, nausea
• Differences: AACG presents acutely with markedly elevated IOP (50-80 mmHg), corneal oedema (steamy/hazy cornea), fixed mid-dilated vertically oval pupil, extremely shallow anterior chamber. Pain is related to the IOP spike rather than scleral inflammation. No deep violaceous scleral congestion.
• Key distinction: Very high IOP; mid-dilated fixed pupil; hazy cornea; shallow AC; no scleral tenderness.

4. Infectious Conjunctivitis

• Similarities: Red eye, discomfort
• Differences: Conjunctivitis produces mucopurulent or purulent discharge, diffuse conjunctival injection, papillary/follicular reaction, no significant pain (only grittiness), normal VA. Sclera is not involved. Phenylephrine blanches the vessels fully.
• Key distinction: Discharge; no pain; no scleral involvement; no systemic associations; blanches with phenylephrine.

5. Herpes Zoster Ophthalmicus (HZO)-associated Scleritis

• Similarities: Severe unilateral eye pain, redness, recurrent scleritis (nodular type is disproportionately associated with prior HZO)
• Differences: History of dermatomal vesicular rash in the V1 distribution (forehead, tip of nose - Hutchinson's sign). Typically older patients. Keratitis (dendritic/disciform), iritis, and post-herpetic neuralgia commonly co-exist.
• Key distinction: History of prior zoster rash; Hutchinson's sign; dendritic/disciform keratitis; no systemic rheumatological disease.

II. Systemic Differential Diagnosis - Underlying Cause of Scleritis

6. Rheumatoid Arthritis (Primary Diagnosis)

• Most common systemic association of scleritis (up to 50% of systemic scleritis cases)
• Symmetrical deforming polyarthritis predominantly of small joints of hands, morning stiffness >1 hour
• RF positive in 80-90%; anti-CCP antibodies (more specific)
• All forms of immune-mediated scleritis described in RA; clinical course often more aggressive
• Extra-articular: keratoconjunctivitis sicca, interstitial lung disease, rheumatoid nodules, vasculitis
• Supports diagnosis in this patient: symmetrical joint disease, known RA history, cough/SOB (RA-ILD)

7. Granulomatosis with Polyangiitis (GPA / Wegener's)

• Key features to consider: Multisystem small vessel vasculitis - classic triad of sinuses + lungs + kidneys
• Scleritis in GPA is often necrotizing and rapidly progressive, with associated peripheral ulcerative keratitis
• cANCA (PR3-ANCA) positive in >90% of active GPA
• Respiratory: pulmonary nodules, haemoptysis, cavitating lesions on CXR/CT
• Renal: haematuria, proteinuria, rapidly progressive glomerulonephritis
• Skin: purpura, skin ulcers
• Nasal/sinus: chronic sinusitis, saddle-nose deformity
• Strongly suspected in this patient given the combination of scleritis + cough/SOB + urinary symptoms over 3-4 years - must be excluded urgently as GPA can be life-threatening
• Kanski's, p. 312: "scleritis is often rapidly progressive, necrotizing and granulomatous"

8. Systemic Lupus Erythematosus (SLE)

• Multisystem autoimmune disease; female predominance (F:M = 9:1) - matches this patient
• Scleritis in SLE: incidence ~1%, may be presenting feature; usually diffuse or nodular anterior scleritis
• Butterfly malar rash (photosensitive, cheek distribution), discoid rash, hair loss
• Joint pain in up to 90% (but non-destructive, unlike RA)
• Pleuritis/pleural effusion causing cough and SOB
• Lupus nephritis - proteinuria, haematuria, nephrotic syndrome (urinary symptoms fit)
• ANA positive (sensitivity 99%); anti-dsDNA, anti-Smith for specificity
• Kanski's, p. 313: "the incidence of episcleritis and scleritis is 1% and may be the presenting feature of the disease"

9. Sarcoidosis

• Granulomatous disease most commonly affecting lungs, lymph nodes, eyes, skin, joints
• Ocular: anterior uveitis (most common), scleritis, optic neuropathy
• Respiratory: bilateral hilar lymphadenopathy on CXR, dry cough, progressive SOB
• Skin: erythema nodosum, lupus pernio
• Renal: hypercalciuria, nephrolithiasis
• Serum ACE elevated in ~60%; chest X-ray/CT is key investigation
• No specific autoantibody; tissue biopsy (accessible lymph node, bronchoscopy) for non-caseating granulomas

10. Relapsing Polychondritis

• Recurrent inflammatory episodes involving cartilaginous structures: ears (auricular chondritis - red, swollen pinnae sparing the earlobes), nasal cartilage collapse, laryngotracheal stenosis (causing cough/SOB), joints
• Scleritis often intractable, may be necrotizing or non-necrotizing
• Middle-age presentation; no specific serology
• Consider if patient has auricular involvement or history of saddle-nose deformity, and if cANCA is negative

11. Polyarteritis Nodosa (PAN)

• Medium and small artery aneurysmal vasculitis; male predominance (3:1)
• Systemic: renal artery involvement (hypertension, renal impairment), mesenteric ischaemia, peripheral neuropathy, skin nodules along arteries
• Scleritis often aggressive and necrotizing
• ~30% associated with hepatitis B infection
• ANCA typically negative (helps distinguish from GPA/MPA)

Summary Table: Differential Diagnosis

INVESTIGATIONS PLANNED

1. Slit-lamp examination with red-free filter (green light) - assess for avascular areas of sclera
2. Gonioscopy - if IOP elevated
3. Dilated fundus examination - posterior scleritis signs
4. B-scan ultrasonography - T-sign (fluid in sub-Tenon space)

1. CBC, ESR, CRP - inflammatory markers
2. Rheumatoid factor (RF) and anti-CCP - RA confirmation
3. ANA, anti-dsDNA, anti-Smith - SLE
4. cANCA (PR3-ANCA), pANCA (MPO-ANCA) - GPA, MPA
5. Serum ACE, chest X-ray / HRCT chest - sarcoidosis, GPA pulmonary involvement, RA-ILD
6. Urinalysis + urine microscopy - haematuria (RBC casts = GN), proteinuria
7. Serum creatinine, eGFR - renal function
8. Uric acid - gout-associated scleritis
9. FBS/PPBS - diabetes
10. HBsAg - PAN
11. RPR/VDRL, FTA-ABS - syphilitic scleritis
12. PPD or IGRA - TB-associated scleritis
13. Complement (C3, C4, CH50) - SLE activity
14. HLA-B27 if spondyloarthropathy suspected

MANAGEMENT

1. Oral NSAIDs - first line for diffuse/nodular non-necrotizing scleritis: flurbiprofen 100 mg t.i.d. or naproxen 250-500 mg b.i.d. or indomethacin 25-50 mg t.i.d. with proton-pump inhibitor cover
2. Oral corticosteroids - if NSAIDs fail: prednisolone 60-80 mg daily with taper; with calcium + Vit D supplementation
3. Immunosuppressive therapy (cyclophosphamide, methotrexate, azathioprine, mycophenolate, anti-TNF biologics) - if steroid-dependent or refractory; coordinate with rheumatology
4. Topical steroids have limited benefit in scleritis (difluprednate 0.05% may help in mild cases)
5. Treat the underlying systemic disease - optimize DMARD therapy for RA; if GPA identified, urgent systemic immunosuppression is life-saving
6. Monitor and manage IOP (glaucoma is the most common cause of visual loss in anterior scleritis) - Kanski's, p. 311

PROGNOSIS

• Diffuse anterior non-necrotizing scleritis: average disease course ~6 years; frequency of recurrences decreases after 18 months; long-term visual prognosis is good with treatment
• Necrotizing scleritis associated with RA: associated with increased mortality from coronary/cerebral arteritis - requires aggressive immunosuppression
• BCVA 6/6 currently - no permanent structural damage at this stage; early treatment essential to prevent complications (glaucoma, scleral thinning, peripheral keratitis)

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 306-313)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 341-347)
• Firestein & Kelley's Textbook of Rheumatology, 2-Volume Set (Scleritis and Corneal Melt chapter)

CASE PRESENTATION

PATIENT IDENTIFICATION

CHIEF COMPLAINT

HISTORY OF PRESENTING ILLNESS

Background and Diagnosis

1. Open anterior chamber angle bilaterally on gonioscopy - no peripheral anterior synechiae, no secondary angle closure features
2. Elevated intraocular pressure (IOP) - above the normal range of 10-21 mmHg on multiple recordings
3. Glaucomatous optic disc changes - increased cup-to-disc (C/D) ratio, neuroretinal rim thinning/notching (typically infero-temporal followed by supero-temporal per ISNT rule violation), with or without RNFL defects and characteristic visual field loss

On This Visit (Follow-Up Assessment)

• No acute pain or redness in either eye
• No sudden change in vision
• He has noticed gradual, insidious blurring of vision in both eyes over an extended period, more so in the left eye (VA 6/12 LE vs 6/9 RE unaided), though both correct fully to 6/6 and 6/9 respectively with pinhole/correction - indicating no structural central visual loss beyond what is explained by his refractive error and mild glaucomatous change
• No central vision loss - confirming that foveal fixation is preserved; advanced glaucomatous damage to central vision has not yet occurred
• Difficulty in dim light or night-time navigation - suggestive of peripheral field constriction
• No episodes of coloured halos around lights or acute eye pain (absence of angle closure component)
• He may report missing objects or people to the sides - a subtle complaint reflecting peripheral visual field loss, often reported as "bumping into things" or "not seeing the step"
• Compliance with prescribed topical glaucoma drops: he reports using his medications regularly (to be confirmed and drops listed)

Typical Presenting Profile of a POAG Patient at Follow-Up

Past Ocular History

• Bilateral POAG - diagnosed [X years] ago; on topical medications
• Current topical therapy (to be confirmed and listed): likely prostaglandin analogue (latanoprost 0.005% qhs) +/- beta-blocker (timolol 0.5% b.i.d.) +/- carbonic anhydrase inhibitor (dorzolamide 2% b.i.d.) +/- alpha-2 agonist (brimonidine 0.15% b.i.d.)
• Laser trabeculoplasty (SLT/ALT) history - to be noted if performed
• Prior glaucoma surgery (trabeculectomy, tube shunt, MIGS) - to be noted
• History of myopia (to be elicited) - myopia is an independent risk factor for POAG and is associated with susceptibility to glaucomatous damage
• No history of ocular trauma, ocular inflammation, or steroid use

Past Medical History

• Asthma / COPD - contraindication to topical beta-blockers (timolol); if present, avoid or use with caution
• Cardiac disease - heart block, bradyarrhythmia, congestive heart failure - contraindication to beta-blockers
• Diabetes mellitus - associated as a possible risk factor; relevant for systemic workup; also affects vascular optic nerve supply
• Systemic hypertension - may affect ocular perfusion pressure; poorly controlled hypertension may compromise optic nerve head circulation
• Cardiovascular / cerebrovascular disease (prior stroke, TIA) - relevant as these may cause optic atrophy or visual field defects mimicking glaucoma, and also affect vascular risk
• Renal stones - relative caution with oral acetazolamide (carbonic anhydrase inhibitor)
• Sulfonamide allergy - contraindication to acetazolamide
• Vasospastic disorders - migraine, Raynaud's phenomenon - associated with normal tension glaucoma variant; may suggest vascular mechanism of disc damage
• Head injury or intracranial pathology - may mimic glaucomatous field defects

Drug History

• Current topical ophthalmic medications (list and confirm compliance)
• Oral or inhaled / topical steroids - long-term steroid use causes secondary open-angle glaucoma (steroid-response glaucoma); must be excluded if IOP control is poor
• Oral beta-blockers (for cardiac disease) - may mask IOP elevation and reduce IOP, giving falsely reassuring tonometry readings
• Calcium channel blockers - meta-analysis shows higher prevalence of glaucoma in patients on these as monotherapy
• Allergies to any ophthalmic drugs (particularly sulfonamides if acetazolamide is planned)

Family History

• Family history of POAG, blindness, or visual loss from glaucoma is a major risk factor - first-degree siblings have 4x the normal risk; offspring have 2x the normal risk - Kanski's, p. 365
• Other ocular diseases in family members

Social History

• Retired; daily activities largely independent
• Driving status - important for determining functional impact of any visual field loss (POAG may disqualify from driving if binocular field below legal threshold)
• Smoking (relevant if toxic/nutritional optic neuropathy is in the differential)
• Alcohol intake
• Literacy - assess ability to self-administer eye drops and read instructions

GENERAL EXAMINATION

• Conscious, alert, well-oriented
• No acute distress
• Built and nourishment: adequate
• BP and pulse: to be recorded (relevant for ocular perfusion pressure and beta-blocker suitability)
• No pallor, icterus, cyanosis, clubbing, lymphadenopathy, or pedal oedema
• Respiratory: assess for wheeze/reduced air entry (beta-blocker contraindication)

OCULAR EXAMINATION

Visual Acuity

• RE: improves from 6/9 to 6/6 with PH - unaided reduction explained by refractive error; central VA preserved
• LE: improves from 6/12 to 6/9 with PH - more significant reduction; residual 6/9 BCVA in LE may reflect early glaucomatous involvement of central field or coexisting pathology (cataract, macular change) to be evaluated
• Asymmetry of VA (LE worse than RE) correlates with likely greater glaucomatous damage in the LE - consistent with the asymmetric nature of POAG

External and Anterior Segment Examination

Intraocular Pressure (IOP) - Goldmann Applanation Tonometry

Gonioscopy (Diagnostic Criterion)

Open angle on gonioscopy with no PAS = hallmark of POAG. This distinguishes it from angle closure glaucoma and secondary causes with PAS.

Optic Disc Examination (Dilated - Key Diagnostic Criterion)

Visual Fields (Humphrey Automated Perimetry)

• Characteristic patterns of glaucomatous field loss:
  • Nasal step (most common early defect)
  • Arcuate scotoma (Bjerrum scotoma) - extending from blind spot nasally
  • Paracentral scotoma
  • Altitudinal defect (superior > inferior)
  • Generalised depression (advanced)
  • Defects respect the horizontal midline - distinguishes from neurological causes
• Humphrey 24-2 standard; Humphrey 10-2 if paracentral or advanced disease

Central Corneal Thickness (CCT - Pachymetry)

• Average CCT: 535-545 microns
• Thin CCT (<510 μm) is an independent risk factor for POAG progression and underestimates true IOP
• Thick CCT (>570 μm) overestimates IOP - may give falsely elevated readings

OCT (Optical Coherence Tomography)

• RNFL thickness analysis - inferior and superior sectors thinned earliest
• Ganglion cell layer (GCL) analysis
• ONH parameters: rim area, CDR, disc area
• Comparison with normative database and serial scans for progression analysis

DIAGNOSIS

1. Chronic progressive optic neuropathy
2. Retinal nerve fibre layer thinning
3. Characteristic visual field loss
4. Open anterior chamber angle on gonioscopy
5. Absence of secondary causes of glaucoma
6. IOP is a key modifiable risk factor (may or may not be elevated at time of any single measurement)

• Kanski's Clinical Ophthalmology, 10th ed., p. 364

DIFFERENTIAL DIAGNOSIS

If Anterior Chamber Angle Open on Gonioscopy

1. Ocular Hypertension (OHT)

• Similarities: Elevated IOP (>21 mmHg), open angle on gonioscopy
• Differences: Normal optic disc and visual fields - no structural or functional glaucomatous damage. OHT represents a risk factor for developing POAG, not disease itself. The Ocular Hypertension Treatment Study (OHTS) showed ~10% of OHT patients develop POAG over 5 years without treatment.
• Key distinction: No disc cupping or RNFL loss; no field defects.

2. Normal (Low) Tension Glaucoma (NTG)

• Similarities: Open angle, glaucomatous disc changes (often with pronounced focal notching and disc haemorrhages), visual field loss indistinguishable from POAG
• Differences: IOP consistently within normal range (≤21 mmHg) on multiple recordings including diurnal curve. NTG suggests a predominantly vascular/ischaemic mechanism at the optic nerve head. Associated with migraine, Raynaud's phenomenon, hypotension, female sex, and cardiovascular disease. Field defects tend to be denser, closer to fixation.
• Key distinction: Repeated IOP ≤21 mmHg despite disc damage; must exclude intracranial lesions compressing the optic nerve/chiasm before diagnosing NTG.

3. Secondary Open-Angle Glaucoma

a. Pseudoexfoliation Glaucoma (PXFG)

• Similarities: Open angle, elevated IOP, disc cupping, field loss - often asymmetric
• Differences: Pseudoexfoliative material (white flaky deposits) on anterior lens capsule, pupil margin, and corneal endothelium. Sampaolesi line (irregular heavy pigmentation anterior to Schwalbe line) on gonioscopy. IOP may be very high and difficult to control. Zonular weakness, cataract. PXF is the most common identifiable cause of secondary open-angle glaucoma worldwide.
• Key distinction: PXF deposits on slit lamp; Sampaolesi line on gonioscopy.

b. Pigment Dispersion Syndrome / Pigmentary Glaucoma

• Similarities: Open angle, elevated IOP, progressive optic neuropathy
• Differences: Typically young myopic males. Krukenberg spindle (vertical pigment deposition on corneal endothelium), transillumination defects of mid-peripheral iris (spoke-like), heavy pigment deposition on trabecular meshwork on gonioscopy (dense brown pigment band), pigment on anterior lens capsule. IOP spikes after exercise or dilation.
• Key distinction: Younger age; Krukenberg spindle; iris transillumination defects; dense TM pigmentation.

c. Steroid-Induced Glaucoma

• Similarities: Open angle, elevated IOP, optic disc and field changes
• Differences: Clear history of topical, periocular, systemic, or inhaled corticosteroid use. IOP elevation typically occurs 4-6 weeks after starting steroids. "Steroid responders" (genetically determined trabecular meshwork sensitivity) are at risk. IOP normalises on steroid withdrawal in early cases.
• Key distinction: Corticosteroid use history; temporal relationship between steroid use and IOP elevation.

d. Angle Recession Glaucoma

• Similarities: Unilateral or bilateral open-angle glaucoma with disc and field changes
• Differences: History of blunt ocular trauma. Gonioscopy shows characteristic widened ciliary body face (angle recession) - a dark grey-brown band between Schwalbe line and iris. Typically presents years after the trauma. Corneal scars, iris heterochromia, or lens subluxation may be present.
• Key distinction: History of trauma; widened angle on gonioscopy (ciliary body face visible).

e. Uveitic / Inflammatory Open-Angle Glaucoma

• Similarities: Open angle, elevated IOP, optic disc changes
• Differences: History of anterior or posterior uveitis. IOP elevation from trabecular meshwork obstruction by inflammatory cells, trabeculitis, or synechiae formation. Keratic precipitates, anterior chamber cells and flare, posterior synechiae present on slit lamp. May also cause hypotony in active inflammation.
• Key distinction: AC cells, KPs, posterior synechiae; history of uveitis or systemic inflammatory disease.

4. Physiological Optic Cup (Large Cup)

• Similarities: Increased C/D ratio bilaterally
• Differences: Symmetric, large but normal-appearing optic cup in large optic discs (optic disc area >2 mm). No rim notching, no RNFL defects, no visual field loss, normal IOP. Static - does not progress. Often familial (check family members for similarly large cups without glaucoma).
• Key distinction: Large disc size; no RNFL or field defect; symmetric; static on follow-up; normal IOP.

5. Non-Glaucomatous Optic Neuropathies (Mimicking Glaucomatous Cupping)

a. Ischaemic Optic Neuropathy (AION/NAION)

• Similarities: Optic disc changes, visual field loss, reduced VA
• Differences: Typically altitudinal field defect (inferior > superior), relative afferent pupillary defect (RAPD) present, optic disc pallor disproportionate to cupping ("more pale than cupped"). Acute onset vision loss in AION. C/D ratio typically smaller. Associated with cardiovascular risk factors, GCA (arteritic type).
• Key distinction: Pallor > cupping; RAPD; altitudinal field defect; acute onset; vascular risk factors.

b. Compressive Optic Neuropathy (Intracranial / Orbital Mass)

• Similarities: Progressive visual field defects and optic disc pallor/atrophy
• Differences: Visual field defects respecting the vertical midline (hemianopic or quadrantanopic defects) are characteristic of chiasmal or retrochiasmal lesions - not seen in POAG. Colour vision loss (especially red-green axis) is prominent early, out of proportion to VA loss. RAPD may be present. Normal IOP. MRI brain/orbit shows causative lesion.
• Key distinction: Vertical midline-respecting field defects; colour vision loss; RAPD; normal IOP; MRI abnormality.

c. Optic Atrophy (Various Causes - Syphilis, Toxic, Nutritional)

• Similarities: Progressive disc pallor and visual field changes
• Differences: Pallor without proportionate cupping (pale but not deeply cupped disc). Normal IOP. Central scotoma rather than arcuate/nasal step defects. Toxic optic neuropathy: history of ethambutol, methanol, or nutritional deficiency (B12, folate). Syphilitic: positive RPR/FTA-ABS.
• Key distinction: Pale disc without cupping; central scotoma; toxic/nutritional/systemic history; normal IOP.

d. Optic Nerve Drusen

• Similarities: Visual field defects (arcuate, enlarged blind spot), elevated-appearing disc
• Differences: The disc is not cupped but appears elevated/pseudo-papilloedema. Drusen (calcified deposits within the disc) are visible as autofluorescent lesions on FAF and as calcified foci on B-scan ultrasonography or CT. No IOP elevation. Field defects may be present but are often static.
• Key distinction: Non-cupped elevated disc; B-scan or CT showing calcified disc deposits; autofluorescence.

6. Chronic Angle Closure Glaucoma (CACG)

• Similarities: Progressive disc and field changes, elevated IOP, reduced VA
• Differences: Peripheral anterior synechiae (PAS) on gonioscopy - the angle is partially or completely closed by iris adhesions. Shallow anterior chamber. History of intermittent headache, halos, or prior acute episodes. CACG must be excluded before labelling any case as POAG.
• Key distinction: PAS on gonioscopy; shallow AC; history of subacute attacks. (This case has confirmed open angle - CACG excluded.)

Summary Table: Differential Diagnosis at a Glance

MANAGEMENT (At This Follow-Up)

1. IOP measurement - Goldmann applanation tonometry; record time of day; compare with previous readings; assess target IOP achievement (≥30% reduction from pre-treatment baseline)
2. Optic disc assessment - stereoscopic disc examination; compare with baseline disc photos; look for disc haemorrhage (Drance haemorrhage = sign of active progression)
3. Visual field testing - Humphrey automated perimetry (24-2 standard; 10-2 if paracentral or advanced); assess for progression using GPA (Guided Progression Analysis) or mean deviation trend
4. OCT RNFL and GCL analysis - compare with baseline and normative database; progression module
5. Pachymetry - if not done recently; adjust IOP targets based on CCT
6. Gonioscopy - if indicated by clinical change or IOP spike
7. Review medication compliance - assess technique of drop instillation; side effects (conjunctival hyperaemia, systemic beta-blocker effects, pigmentation changes from prostaglandins)
8. Optimize medical treatment if IOP above target or disc/field progression detected:
   • Prostaglandin analogue (first line): latanoprost 0.005% qhs
   • Add topical beta-blocker: timolol 0.5% b.i.d. (if no contraindication)
   • Add CAI: dorzolamide 2% b.i.d. / brinzolamide 1% b.i.d.
   • Add alpha-2 agonist: brimonidine 0.15% b.i.d.
   • Fixed combination drops if multi-medication burden is a compliance concern
9. Selective Laser Trabeculoplasty (SLT) - if medication compliance is poor, IOP uncontrolled, or patient preference; effective as first-line or adjunct
10. Trabeculectomy / glaucoma drainage device / MIGS - if IOP uncontrolled with maximum tolerated medications or rapid progression threatening fixation
11. Systemic review - BP, FBS, HbA1c, lipid panel, CBC; cardiovascular optimization

PROGNOSIS

• POAG is a chronic, lifelong condition; once optic nerve damage occurs it is irreversible
• With treatment, IOP reduction by ≥30% from baseline significantly slows progression
• The EMGT (Early Manifest Glaucoma Trial) showed untreated high-tension glaucoma progresses at ~1.31 dB/year mean deviation loss; treatment substantially reduces this rate - Kanski's, p. 363
• This patient's BCVA of 6/6 RE and 6/9 LE with preserved central fixation indicates moderate-stage disease with good visual potential if IOP is adequately controlled going forward
• Life-long monitoring, patient education, and medication adherence are the cornerstones of management
• Risk of blindness in at least one eye over a lifetime if left untreated or undertreated; the average patient without treatment deteriorates from normal to blindness over ~25 years, influenced dramatically by IOP control

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 363-378)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 547-558)

CASE PRESENTATION

PATIENT IDENTIFICATION

CHIEF COMPLAINT

HISTORY OF PRESENTING ILLNESS

Background and Initial Injury

The Acute Traumatic Event

• Sudden, severe pain in the right eye
• Redness and subconjunctival haemorrhage
• Blurring of vision - due to hyphema (blood in the anterior chamber), raised IOP, or corneal oedema
• Hyphema - blood settling inferiorly as a red fluid level in the anterior chamber (from rupture of iris root vessels or ciliary body face vasculature)
• Photophobia and watering of the eye
• Possible transient mydriasis (traumatic mydriasis from iris sphincter damage)

Pathogenesis (Brief)

"Although a large percentage of eyes with traumatic hyphaema exhibit some degree of angle recession, glaucoma develops in less than 10% after 10 years. The rise in IOP is secondary to associated trabecular damage rather than from angle recession itself. The risk of glaucoma is directly related to the extent of angle recession and is unlikely to follow if less than three quadrants are recessed." - Kanski's, p. 411

On This Visit (Follow-Up)

• No acute pain or redness in the right eye
• No sudden change in vision - VA is 6/6 bilaterally, confirming central visual acuity preserved in both eyes
• No episodes of coloured halos or acute IOP spikes symptomatic enough to cause acute pain
• No new trauma to either eye
• He is compliant with his prescribed topical anti-glaucoma medications (to be confirmed)
• He may report mild difficulty with peripheral vision in the right eye, though with central VA of 6/6 this is subtle at this stage

• RE IOP of 26 mmHg - elevated (normal 10-21 mmHg); represents inadequate IOP control in the affected eye; this asymmetry of >5 mmHg between eyes is clinically significant
• LE IOP of 15 mmHg - normal; confirms the IOP elevation is unilateral and secondary to the traumatic mechanism in the right eye
• The asymmetry (RE 26 vs LE 15) is a key red flag and reinforces the need for stepped-up therapy and closer monitoring in the RE

Symptoms to Review at Every Follow-Up Visit

Past Ocular History

• Blunt trauma to RE by ball - date/year of injury (to be recorded)
• Hyphema at time of acute injury - managed conservatively (confirm treatment received)
• Angle recession glaucoma RE - diagnosed on gonioscopy [X months/years ago]
• Current topical glaucoma medications (list and confirm compliance)
• Laser trabeculoplasty (SLT/ALT) - likely NOT performed as it is rarely/not effective in angle recession glaucoma - Wills Eye Manual, p. 578
• No prior glaucoma surgery
• No history of trauma or glaucoma in the left eye

Past Medical History

• No significant systemic illness
• No sickle cell disease (relevant to rule out in young patients with hyphema - sickle cell patients have higher risk of secondary glaucoma and corneal staining from hyphema)
• No bleeding disorders, coagulopathy, or anticoagulant use
• No history of prior steroid use (steroid-induced glaucoma in the differential)

Drug History

• Current topical ophthalmic medications for glaucoma (list)
• No aspirin or NSAIDs currently (relevant if hyphema risk of rebleed is considered)
• No systemic beta-blockers
• Allergies: to be elicited

Family History

• Family history of glaucoma, raised IOP, or visual loss from glaucoma (first-degree relatives at 4x risk of POAG - relevant if there is also a hereditary susceptibility component)

Social History

• Young male, aged 29 - likely working/studying; driving status and occupation are important (active management needed to protect the eye)
• Sport history: type of sport that caused injury; protective eyewear counselling required
• Compliance with drops: single person living alone vs. family support

GENERAL EXAMINATION

• Conscious, alert, well-oriented
• Young male, no acute distress
• Built and nourishment: adequate
• No systemic abnormalities
• BP and pulse: to be recorded

OCULAR EXAMINATION

Visual Acuity

Intraocular Pressure (IOP)

• RE IOP of 26 mmHg = above normal limit (21 mmHg); indicates active, inadequately controlled glaucoma in the traumatized eye
• Asymmetry of 11 mmHg between eyes is highly significant and demands medication review
• IOP should be checked at multiple times of day (diurnal curve) to capture peaks

External and Anterior Segment Examination (RE - Key Features)

Gonioscopy (Diagnostic Criterion - RE)

Characteristic gonioscopic appearance: "Uneven iris insertion, torn or absent iris processes, posteriorly recessed iris revealing widened ciliary band." Comparison with the contralateral eye identifies recessed areas. Scleral spur appears abnormally white. - Wills Eye Manual, p. 578

Optic Disc Examination (Dilated)

Visual Fields (Humphrey Automated Perimetry 24-2)

• Nasal step, arcuate scotoma, paracentral scotoma consistent with glaucomatous damage (RE)
• LE - expected normal at this age

OCT (RNFL and ONH Analysis)

• RNFL thinning RE (inferior and/or superior sectors) compared with age-matched normative database
• GCL analysis
• Serial comparison for progression rate

DIAGNOSIS

• History of ball injury to RE with resultant hyphema and angle recession on gonioscopy
• RE IOP: 26 mmHg (elevated) vs LE IOP: 15 mmHg (normal)
• Open angle bilaterally on gonioscopy; widened ciliary body band RE with posterior recession of iris
• Glaucomatous optic disc changes RE
• VA 6/6 bilaterally - central acuity preserved
• Age 29 - young patient; lifetime risk of visual loss is high without adequate long-term IOP control

DIFFERENTIAL DIAGNOSIS

1. Primary Open-Angle Glaucoma (POAG)

• Similarities: Open angle on gonioscopy, elevated IOP, progressive optic disc and field changes
• Differences: POAG is typically bilateral and symmetrical in its effect; there is no identifiable causative event. In this patient, glaucoma is unilateral, asymmetric (IOP 26 vs 15), and follows a documented episode of blunt trauma with a specific gonioscopic finding (widened ciliary body band, absent iris processes). POAG gonioscopy shows a normal open angle without these traumatic features.
• Key distinction: History of trauma; widened ciliary body band on gonioscopy; unilateral IOP elevation in the traumatized eye; young age (POAG rare at 29 years).

2. Steroid-Induced Open-Angle Glaucoma

• Similarities: Open angle, elevated IOP, progressive disc changes
• Differences: Requires a clear history of topical, periocular, inhaled, or systemic corticosteroid use. IOP elevation typically begins 4-6 weeks after steroid initiation and resolves on cessation in early stages. Gonioscopy shows a normal angle (no widening or trabecular disruption). May coexist with angle recession if steroids were used to treat the original trauma.
• Key distinction: Steroid exposure history; normal gonioscopy (no angle recession); bilateral risk if both eyes exposed.

3. Ocular Hypertension (OHT)

• Similarities: Elevated IOP (RE 26 mmHg), open angle
• Differences: OHT is defined by elevated IOP without glaucomatous optic disc damage or visual field loss - a purely IOP-based finding. In this patient, there is both a causative traumatic event and likely optic disc changes confirming structural glaucoma beyond mere IOP elevation.
• Key distinction: No disc or field damage in OHT; this patient has a documented traumatic aetiology and structural disc changes.

4. Pigmentary Glaucoma / Pigment Dispersion Syndrome

• Similarities: Young male, unilateral or asymmetric IOP elevation, open angle
• Differences: Pigmentary glaucoma is associated with Krukenberg spindle (vertical pigment on corneal endothelium), iris transillumination defects (spoke-like mid-peripheral), and dense brown pigmentation of the trabecular meshwork on gonioscopy (not the widened ciliary body band of angle recession). IOP spikes after exercise or pupillary dilation. Typically affects young myopic males (demographics overlap) but lacks trauma history.
• Key distinction: Krukenberg spindle; mid-peripheral iris transillumination; heavy TM pigmentation; no trauma history; no widened ciliary body band.

5. Pseudoexfoliation (PXF) Glaucoma

• Similarities: Elevated IOP, open angle, asymmetric presentation
• Differences: PXF is rare at age 29. Characterised by white flaky deposits on anterior lens capsule, pupil margin, and corneal endothelium. Sampaolesi line on gonioscopy. No trauma history. More common in elderly.
• Key distinction: PXF deposits on slit lamp; older age group; Sampaolesi line; no trauma.

6. Ghost Cell Glaucoma

• Similarities: Follows blunt trauma (or vitreous haemorrhage), elevated IOP in traumatized eye, open angle
• Differences: Ghost cell glaucoma occurs approximately 2 weeks after a vitreous haemorrhage when degenerate erythrocytes (ghost cells) pass through a defect in the hyaloid face into the anterior chamber and obstruct the TM. The anterior chamber shows reddish-brown or khaki ghost cells visible on slit lamp. This is typically a subacute, self-limiting elevation of IOP around the time of the acute injury, rather than the chronic late-presenting glaucoma of angle recession. Gonioscopy shows open angle with khaki material, not the widened ciliary body band.
• Key distinction: Occurs acutely/subacutely post-trauma; khaki particles in AC; no widened ciliary body band; self-limiting.

7. Inflammatory (Traumatic Iritis / Uveitic) Open-Angle Glaucoma

• Similarities: Follows ocular trauma, elevated IOP, unilateral
• Differences: Traumatic iritis produces anterior chamber cells, flare, ciliary flush, and photophobia in the acute phase. IOP elevation is from trabeculitis and inflammatory cell obstruction of TM. This is typically an acute post-traumatic finding that resolves with anti-inflammatory treatment. The gonioscopy in traumatic iritis shows open angle without the characteristic recession pattern; KPs may be present.
• Key distinction: Active AC cells and flare; photophobia; acute temporal relationship to trauma; resolves with steroids; no widened ciliary body band.

8. Traumatic Cyclodialysis (Causing Hypotony Rather Than Glaucoma)

• Similarities: Follows blunt ocular trauma; gonioscopy may show an abnormal angle
• Differences: Cyclodialysis is a separation of the ciliary body from the scleral spur creating a cleft that causes markedly low IOP (hypotony) rather than elevated IOP, as aqueous drains freely into the suprachoroidal space. On gonioscopy, the cleft appears as a gap between the scleral spur and ciliary body - different from the widened ciliary body band of angle recession.
• Key distinction: Hypotony (low IOP) rather than elevated IOP; gonioscopic cyclodialysis cleft.

9. Contralateral (LE) Glaucoma Risk - Important Note

• LE IOP today is 15 mmHg (normal) - reassuring
• However, the fellow eye should have annual IOP checks, gonioscopy, and optic disc evaluation
• Consider whether the patient has a genetic predisposition to glaucoma (family history) that lowered his threshold for glaucoma development even in the uninjured eye

Summary Table: Differential Diagnosis

INVESTIGATIONS PLANNED / TO BE REVIEWED

1. IOP measurement (GAT) - RE 26, LE 15 - document and compare with previous records
2. Gonioscopy - document extent of angle recession (clock hours); compare RE vs LE; look for fibrous changes in long-standing recession; note trabecular damage
3. Optic disc assessment - dilated stereoscopic exam; compare CDR, rim width, disc haemorrhages with baseline
4. Visual field testing - Humphrey 24-2; assess for progression
5. OCT RNFL and GCL - assess for RNFL thinning RE vs LE; compare with baseline
6. Pachymetry (CCT) - thin CCT underestimates true IOP; important for target IOP setting
7. Slit lamp exam - assess for traumatic cataract (rosette opacity), iridodialysis, iris sphincter tears, Vossius ring, corneal scarring, or blood staining
8. B-scan if posterior segment view impaired - assess for vitreous haemorrhage, retinal dialysis (blunt trauma can cause inferotemporal retinal dialysis), or posterior scleral changes

MANAGEMENT

Current IOP Status

Medical Treatment

1. Prostaglandin analogue (first line): latanoprost 0.005% qhs - maximally reduces IOP by 25-35%
2. Topical beta-blocker: timolol 0.5% b.i.d. (if no contraindication - assess asthma/cardiac status in this young patient)
3. Topical CAI: dorzolamide 2% b.i.d. or brinzolamide 1% b.i.d. - adjunctive
4. Alpha-2 agonist: brimonidine 0.15% b.i.d. - adjunctive
5. Fixed-combination drops if compliance is a concern

Important: Miotics (pilocarpine) are relatively contraindicated in angle recession glaucoma - they may be ineffective or may even cause paradoxical IOP elevation by reducing uveoscleral outflow. ALT and SLT are rarely effective in angle recession glaucoma due to damaged TM. - Wills Eye Manual, p. 578; Kanski's, p. 411

Surgical Treatment

• Trabeculectomy with adjunctive antimetabolite (mitomycin-C) - generally effective; indicated if IOP uncontrolled on maximum medical therapy or if progression is rapid
• Glaucoma drainage device (tube shunt) - if trabeculectomy fails
• Cyclodiode / cyclophotocoagulation - as last resort in refractory cases

Counselling and Follow-Up

• Protective eyewear - mandatory for all sports; the fellow eye must be protected from further trauma
• Patient education: POAG-like silent progression; importance of lifelong drop compliance
• Annual LE monitoring given risk of fellow eye delayed glaucoma
• Frequency of RE review: every 1-3 months given active IOP elevation above target

PROGNOSIS

• Guarded in RE - IOP currently uncontrolled at 26 mmHg; young age (29 years) means lifelong risk of cumulative visual field loss is high without tight IOP control
• Central VA of 6/6 preserved currently - good structural reserve if IOP is adequately managed promptly
• Lifetime monitoring is essential - angle recession glaucoma can present years after the initial trauma and progression may be delayed or rapid
• Fellow eye (LE) at increased risk of delayed open-angle glaucoma - annual monitoring mandatory

• Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Edition (pp. 411, 929-931)
• The Wills Eye Manual, Office and Emergency Room Diagnosis and Treatment of Eye Disease (pp. 578-579)