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ophtho.acute-vision-loss.core.v1PRODUCTION
ophtho.acute-vision-loss.core.v1

Acute (sudden) vision loss — neuro-ophthalmology / EM triage

general_internal_medicineacutesubacuteadultgeriatric
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

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Frame

Detailed

Frame acute vision loss as an EMERGENCY TRIAGE problem, not a leisurely differential. Two can-not-miss clocks dominate: CRAO = acute ischemic stroke (retina dies in ~90-100 min — Hayreh Ophthalmology 1980 PMID 6769079; Mac Grory Stroke 2021 PMID 33677974) and GCA = empiric high-dose steroid before biopsy to save the fellow eye (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356). Definitive pathway ownership is routed OUT by engine_id.

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emergency-triage framing set; CRAO and GCA clocks foregrounded

Patient inputs (19)

Age ≥50 dramatically raises GCA and arteritic-AION pretest probability and lowers the empiric-steroid threshold (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356)

New headache, jaw claudication, scalp tenderness, polymyalgia, constitutional symptoms — the can-not-miss GCA cluster mandating empiric high-dose steroid before biopsy (Maz ACR/VF 2021 PMID 34235884)

Retinal ischemic clock: retina tolerates ~90-100 min of complete CRAO before irreversible loss — drives hyperacute stroke-pathway activation and thrombolysis-window assessment (Hayreh Ophthalmology 1980 PMID 6769079; Mac Grory Stroke 2021 PMID 33677974)

Atherosclerosis/AF/HTN/DM/carotid disease — CRAO and amaurosis carry the same secondary-prevention urgency as cerebral stroke/TIA (Mac Grory Stroke 2021 PMID 33677974)

Painful vs painless is the first triage binary: painless → vascular/retinal/optic-ischemic; painful → AACG, optic neuritis, endophthalmitis, scleritis (Biousse/Newman neuro-ophthalmology framework; Beck ONTT NEJM 1992 PMID 1734247)

Transient (resolved) vision loss = amaurosis fugax / cortical TIA → TIA-equivalent workup; persistent = established CRAO/AION/RD/ON (Mac Grory Stroke 2021 PMID 33677974)

Monocular → eye/optic-nerve to chiasm; binocular homonymous → post-chiasmal/occipital cortical lesion (stroke pathway) (Mac Grory Stroke 2021 PMID 33677974)

A RAPD localises the lesion to the optic nerve/retina and is the single most useful afferent-pathway bedside sign (present in CRAO, AION, optic neuritis; absent in media opacity, functional loss, symmetric bilateral)

Cherry-red spot/box-carring → CRAO; pallid disc edema → arteritic AION; flame hemorrhages/blood-and-thunder → CRVO; detached retina/Shafer sign → RD; vitreous blood → VH; normal fundus + RAPD → retrobulbar ON or posterior pathology (Hayreh & Zimmerman Retina 2007 PMID 17460582)

Erythrocyte sedimentation rate — markedly elevated supports GCA; a normal ESR does NOT exclude GCA (~4-22% biopsy-proven with normal ESR) so it never overrides empiric steroid (Maz ACR/VF 2021 PMID 34235884)

CRP complements ESR for GCA (higher sensitivity; ESR+CRP together miss few cases); both feed the GCA Bayesian chain but neither delays steroid (Maz ACR/VF 2021 PMID 34235884)

Hemiparesis/aphasia/dysarthria/ataxia with vision loss reframes to acute stroke and activates the stroke pathway regardless of the ocular finding (Mac Grory Stroke 2021 PMID 33677974)

Pain on eye movement + central scotoma + dyschromatopsia in a younger patient is the optic-neuritis triad (Beck ONTT NEJM 1992 PMID 1734247)

Prior MS/NMOSD/MOGAD or risk reframes optic neuritis and changes acute therapy (IVMP ± PLEX for severe/NMOSD) (Beck ONTT NEJM 1992 PMID 1734247)

Endogenous endophthalmitis, CMV/atypical retinitis, and atypical optic neuropathies broaden the differential and lower the imaging/tap threshold

Markedly elevated IOP with a red painful eye, mid-dilated pupil, haloes, nausea = acute angle-closure glaucoma — emergent IOP-lowering then route to glaucoma engine

Thrombocytosis is an independent GCA pointer and part of the inflammatory triad (ESR/CRP/platelets) used to raise pretest probability before biopsy (Maz ACR/VF 2021 PMID 34235884)

Post-operative / post-intravitreal-injection painful red eye with vision loss = endophthalmitis until proven otherwise — emergent tap-and-inject route

Steroid + neuro-imaging safety gating; pregnancy/postpartum also raises CVST/PRES/preeclampsia-related cortical visual loss

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (8)

8 need judgement
  • informationallife_threateningcrao_hyperacute_stroke_equivalent
    Sudden painless monocular vision loss + cherry-red spot / box-carring / Hollenhorst plaque — CRAO is acute ischemic stroke; retinal ischemic clock ~90-100 min (Hayreh Ophthalmology 1980 PMID 6769079; Mac Grory Stroke 2021 PMID 33677974)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninggca_emergency_empiric_steroid
    Age ≥50 + vision loss (arteritic AION/CRAO) + new headache / jaw claudication / scalp tenderness / PMR ± ↑ESR/CRP/platelets — empiric high-dose steroid BEFORE biopsy (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningendophthalmitis_post_procedure
    Painful red eye with vision loss + hypopyon after recent intraocular surgery or intravitreal injection (or endogenous in immunocompromised/bacteremic) — endophthalmitis
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningoccipital_stroke_binocular_visual_loss
    Binocular congruous homonymous field loss / "cortical blindness" with intact pupillary responses ± other neuro deficits — occipital/post-chiasmal stroke (Mac Grory Stroke 2021 PMID 33677974)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereoptic_neuritis_ivmp_not_oral
    Younger patient, painful vision loss with eye-movement pain + central scotoma + dyschromatopsia + RAPD ± disc edema — optic neuritis; oral prednisone alone is contraindicated (Beck ONTT NEJM 1992 PMID 1734247)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereretinal_detachment_macula_on
    Acute curtain/shadow + flashes/floaters + Shafer sign with the MACULA still ON (central vision intact) — same-day surgical emergency before the macula detaches (AAO PPP retinal detachment)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereacute_angle_closure_glaucoma
    Sudden painful vision loss + red eye + mid-dilated non-reactive pupil + markedly elevated IOP + haloes/nausea/vomiting — acute angle-closure glaucoma
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveretransient_visual_loss_tia_equivalent
    Transient monocular (amaurosis fugax) OR transient binocular/cortical visual loss that has resolved — a TIA equivalent demanding urgent stroke-risk workup (Mac Grory Stroke 2021 PMID 33677974)
    Trigger could not be auto-evaluated — needs clinician judgement.

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RISK_STRATIFICATIONrequiredDrives risk stratification
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Recommended regimen

Acute vision loss — etiology-stratified emergency therapy (recognise → stabilise → route OUT)
axis: acute_vision_loss_etiology_emergencystep 1 - Step 1 — GCA suspected: empiric high-dose corticosteroid BEFORE biopsy
Selected step "Step 1 — GCA suspected: empiric high-dose corticosteroid BEFORE biopsy" — Age ≥50 with vision loss (arteritic AION/CRAO) AND any of: new headache, jaw claudication, scalp tenderness, PMR, ↑ESR/CRP/platelets — do NOT wait for ESR or temporal-artery biopsy
  • methylprednisolone
    first line
    systemic_corticosteroid
    500-1000 mg • IV • once daily × 3 days (max: 1000 mg/day)
    triggers: gca_with_threatened_or_established_vision_loss, arteritic_aion, arteritic_crao
    Maz ACR/VF 2021 (PMID 34235884) — IV pulse glucocorticoid for GCA with threatened/established vision loss; Liu Ophthalmology 1994 (PMID 7800356) — IV therapy associated with less fellow-eye involvement and slightly better visual prognosis vs oral. Goal: save the FELLOW eye.
    rxcui 6902
  • prednisone
    first line
    systemic_corticosteroid
    1 mg/kg (typically 60 mg) • PO • once daily (max: 80 mg/day)
    triggers: gca_suspected_no_iv_access_or_no_acute_vision_loss
    Maz ACR/VF 2021 (PMID 34235884) — high-dose oral glucocorticoid started immediately on strong GCA suspicion when IV pulse not indicated/available; never delayed for biopsy (biopsy yield preserved for ~2 weeks)
    rxcui 8640
  • low_dose_aspirin_adjunct_and_urgent_temporal_artery_biopsy
    add on
    gca_adjunct
    triggers: gca_suspected
    Maz ACR/VF 2021 (PMID 34235884) — antiplatelet adjunct consideration; arrange temporal-artery biopsy ≤2 weeks but treat first. Definitive taper + tocilizumab owned by rheum.giant-cell-arteritis.core.v1.

ed playbook — drug actions (4)

  1. 1. IV methylprednisolone (GCA with vision loss)
    rxcui 6902
    500-1000 mg • IV • once daily × 3 d
    trigger: Age ≥50 + arteritic AION/CRAO + GCA cluster — empiric, before biopsy (Maz ACR/VF 2021 PMID 34235884)
    Save the fellow eye; IV pulse for threatened/established loss (Liu Ophthalmology 1994 PMID 7800356)
  2. 2. Oral prednisone (GCA suspected, no acute vision loss / no IV access)
    rxcui 8640
    1 mg/kg (≈60 mg) • PO • once daily
    trigger: Strong GCA suspicion without threatened vision loss (Maz ACR/VF 2021 PMID 34235884)
    Immediate high-dose oral steroid; biopsy yield preserved ~2 weeks
  3. 3. IV methylprednisolone (optic neuritis)
    rxcui 6902
    1000 mg • IV • once daily × 3-5 d
    trigger: Optic neuritis triad in younger patient (Beck ONTT NEJM 1992 PMID 1734247)
    Speeds recovery; oral prednisone alone contraindicated
  4. 4. Acetazolamide (acute angle-closure)
    rxcui 167
    500 mg • IV/PO • once then per IOP
    trigger: Painful red eye + ↑IOP + mid-dilated pupil
    Emergent aqueous suppression bridge to laser PI

Auto-drafted A&P note

ed

Subjective

- Possible entry pathways: Sudden PAINLESS monocular vision loss — CRAO / AION / CRVO / RD / vitreous hemorrhage until excluded; CRAO = acute ischemic stroke (Mac Grory Stroke 2021 AHA Scientific Statement, PMID 33677974); Sudden vision loss WITH eye/peri-ocular pain — acute angle-closure glaucoma, optic neuritis, endophthalmitis, scleritis, arteritic AION with headache (Maz ACR/VF 2021, PMID 34235884; Beck ONTT NEJM 1992, PMID 1734247); TRANSIENT vision loss (amaurosis fugax / blackout, recovered) — retinal or cortical TIA equivalent; route to TIA/stroke workup (Mac Grory Stroke 2021, PMID 33677974).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Acute (sudden) vision loss — neuro-ophthalmology / EM triage** (ophtho.acute-vision-loss.core.v1).
Phenotype framing: Terminal differential with pivot findings: CRAO (cherry-red spot + sudden painless monocular loss + retinal clock pivot — STROKE) vs arteritic AION/GCA (≥50 + pallid disc edema + jaw claudication + ↑ESR/CRP/platelets pivot — STEROID) vs non-arteritic AION (small crowded "disc at risk", afebrile, normal ESR, vasculopath pivot) vs CRVO (blood-and-thunder fundus pivot) vs rhegmatogenous RD (curtain + flashes/floaters + Shafer sign pivot) vs vitreous hemorrhage (loss of red reflex + diabetic/PVD pivot) vs optic neuritis (young + pain on movement + dyschromatopsia + RAPD + normal/edematous disc pivot) vs acute angle-closure glaucoma (painful red eye + mid-dilated pupil + ↑IOP + haloes pivot) vs occipital/homonymous stroke (binocular congruous field cut + intact pupils + other neuro signs pivot) vs endophthalmitis (recent surgery/injection + hypopyon + pain pivot) vs functional vision loss (normal RAPD/fundus/OCT + non-physiologic fields + tunnel/spiral pivot — diagnosis of exclusion).
Scope: Frame acute vision loss as an EMERGENCY TRIAGE problem, not a leisurely differential. Two can-not-miss clocks dominate: CRAO = acute ischemic stroke (retina dies in ~90-100 min — Hayreh Ophthalmology 1980 PMID 6769079; Mac Grory Stroke 2021 PMID 33677974) and GCA = empiric high-dose steroid before biopsy to save the fellow eye (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356). Definitive pathway ownership is routed OUT by engine_id.

No severity triggers fired against current inputs.

Plan

Regimen axis: **Acute vision loss — etiology-stratified emergency therapy (recognise → stabilise → route OUT)** — step "Step 1 — GCA suspected: empiric high-dose corticosteroid BEFORE biopsy".
1. methylprednisolone 500-1000 mg IV once daily × 3 days (systemic_corticosteroid, first line) — Maz ACR/VF 2021 (PMID 34235884) — IV pulse glucocorticoid for GCA with threatened/established vision loss; Liu Ophthalmology 1994 (PMID 7800356) — IV therapy associated with less fellow-eye involvement and slightly better visual prognosis vs oral. Goal: save the FELLOW eye.
2. prednisone 1 mg/kg (typically 60 mg) PO once daily (systemic_corticosteroid, first line) — Maz ACR/VF 2021 (PMID 34235884) — high-dose oral glucocorticoid started immediately on strong GCA suspicion when IV pulse not indicated/available; never delayed for biopsy (biopsy yield preserved for ~2 weeks)
3. low_dose_aspirin_adjunct_and_urgent_temporal_artery_biopsy (gca_adjunct, add on) — Maz ACR/VF 2021 (PMID 34235884) — antiplatelet adjunct consideration; arrange temporal-artery biopsy ≤2 weeks but treat first. Definitive taper + tocilizumab owned by rheum.giant-cell-arteritis.core.v1.

Setting playbook (ed) — Triage the four binaries in minutes; activate stroke pathway for CRAO/amaurosis/cortical loss; give empiric high-dose steroid for suspected GCA before biopsy; emergent IOP-lowering for AACG; route definitive ownership OUT by engine_id (Mac Grory Stroke 2021 PMID 33677974; Maz ACR/VF 2021 PMID 34235884)
4. IV methylprednisolone (GCA with vision loss) 500-1000 mg IV once daily × 3 d — Age ≥50 + arteritic AION/CRAO + GCA cluster — empiric, before biopsy (Maz ACR/VF 2021 PMID 34235884) (Save the fellow eye; IV pulse for threatened/established loss (Liu Ophthalmology 1994 PMID 7800356))
5. Oral prednisone (GCA suspected, no acute vision loss / no IV access) 1 mg/kg (≈60 mg) PO once daily — Strong GCA suspicion without threatened vision loss (Maz ACR/VF 2021 PMID 34235884) (Immediate high-dose oral steroid; biopsy yield preserved ~2 weeks)
6. IV methylprednisolone (optic neuritis) 1000 mg IV once daily × 3-5 d — Optic neuritis triad in younger patient (Beck ONTT NEJM 1992 PMID 1734247) (Speeds recovery; oral prednisone alone contraindicated)
7. Acetazolamide (acute angle-closure) 500 mg IV/PO once then per IOP — Painful red eye + ↑IOP + mid-dilated pupil (Emergent aqueous suppression bridge to laser PI)

Non-pharmacologic actions:
- Activate stroke pathway / call stroke team for CRAO, amaurosis, cortical/homonymous loss, or focal deficit (Mac Grory Stroke 2021 PMID 33677974)
- Ocular massage / IOP-lowering / anterior-chamber paracentesis only as low-yield temporising while the stroke team mobilises — do NOT delay routing (Mac Grory Stroke 2021 PMID 33677974)
- STAT DWI-MRI for binocular/cortical loss and for CRAO (concurrent brain infarction common)
- Emergent ophthalmology for endophthalmitis (tap-and-inject), macula-on RD (same-day surgery), uncontrolled AACG

AVOID / contraindication checks:
- Oral prednisone alone contraindicated in optic neuritis (Beck ONTT NEJM 1992 PMID 1734247 — increased recurrence vs placebo; only as post IVMP taper)
- Do not delay empiric steroid for ESR or temporal artery biopsy in suspected GCA (Maz ACR/VF 2021 PMID 34235884 — fellow eye loss is preventable; biopsy yield preserved ~2 weeks)
- Intra arterial fibrinolysis not recommended for CRAO (EAGLE Schumacher Ophthalmology 2010 PMID 20609991 — no benefit, ↑adverse events; do not substitute for stroke pathway routing)
- Do not delay stroke pathway for ocular massage or paracentesis (Mac Grory Stroke 2021 PMID 33677974 — temporising measures are low yield; retinal clock ~90 100 min Hayreh PMID 6769079)
- Steroid and neuroimaging pregnancy gating (pregnancy/postpartum also raises CVST/PRES/preeclampsia cortical visual loss)
- Acetazolamide avoid sulfa allergy and renal dose adjust (calc.ckd_epi_2021; AACG bridge only)

Monitoring

Regimen monitoring:
- GCA steroid response headache visual symptoms 24-72h and fellow eye vigilance (Liu Ophthalmology 1994 PMID 7800356)
- CRAO stroke neuro checks and completion of vascular secondary prevention workup (Mac Grory Stroke 2021 PMID 33677974)
- optic neuritis serial acuity colour fields recovery by 1 year (Beck Arch Ophthalmol 1993 PMID 8512477)
- AACG serial IOP until definitive laser PI
- ESR CRP trend during GCA steroid therapy (Maz ACR/VF 2021 PMID 34235884)

Setting (ed) monitoring:
- Reassess RAPD/acuity and neuro status at frequent intervals while routing
- IOP recheck until controlled in AACG

Follow-up plan: CRAO/amaurosis: stroke/cardiology secondary prevention is the long-tail deliverable — antithrombotic, vascular-risk control, carotid intervention if indicated (Mac Grory Stroke 2021 PMID 33677974) via neuro.acute-stroke.core.v1 / neuro.tia.core.v1. GCA: long glucocorticoid taper + tocilizumab + relapse surveillance via rheum.giant-cell-arteritis.core.v1. Optic neuritis: MS/NMOSD/MOGAD demyelination workup + DMT via neuro.optic-neuritis.core.v1. Low-vision rehabilitation and counselling for irreversible loss; functional vision loss → supportive follow-up.
- Close-out criterion: secondary-prevention / disease-specific follow-up handed to the routed engine_id

Monitoring phase: GCA: steroid response (headache/visual symptoms within 24-72 h), fellow-eye vigilance (untreated fellow-eye involvement risk is high — Liu Ophthalmology 1994 PMID 7800356), ESR/CRP trend. CRAO/stroke: neuro checks, completion of stroke secondary-prevention workup (carotid/cardiac/embolic). Optic neuritis: serial acuity/colour/fields — most recover acuity by 1 year even without steroid (Beck Arch Ophthalmol 1993 PMID 8512477). RD/endophthalmitis/AACG: per receiving engine.

Disposition

Current setting: ed — Triage the four binaries in minutes; activate stroke pathway for CRAO/amaurosis/cortical loss; give empiric high-dose steroid for suspected GCA before biopsy; emergent IOP-lowering for AACG; route definitive ownership OUT by engine_id (Mac Grory Stroke 2021 PMID 33677974; Maz ACR/VF 2021 PMID 34235884)

Disposition criteria:
- Stroke pathway / stroke unit for CRAO/amaurosis/cortical loss
- Admit for IV steroid pulse + biopsy ≤2 wk for GCA with vision loss
- Emergent vitreoretinal / ophthalmology for RD/endophthalmitis/AACG; outpatient neuro-ophthalmology if functional loss after organic exclusion

Escalation triggers (move to higher acuity):
- CRAO/amaurosis/cortical loss/focal deficit → route to neuro.acute-stroke.core.v1 / neuro.tia.core.v1 (Mac Grory Stroke 2021 PMID 33677974)
- GCA with threatened/established vision loss → admit for IV pulse, route to rheum.giant-cell-arteritis.core.v1 (Maz ACR/VF 2021 PMID 34235884)
- Endophthalmitis / macula-on RD / uncontrolled AACG → emergent ophthalmology

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Sudden painless monocular vision loss + cherry-red spot / box-carring / Hollenhorst plaque — CRAO is acute ischemic stroke; retinal ischemic clock ~90-100 min (Hayreh Ophthalmology 1980 PMID 6769079; Mac Grory Stroke 2021 PMID 33677974)
- [LIFE_THREATENING] Age ≥50 + vision loss (arteritic AION/CRAO) + new headache / jaw claudication / scalp tenderness / PMR ± ↑ESR/CRP/platelets — empiric high-dose steroid BEFORE biopsy (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356)
- [LIFE_THREATENING] Painful red eye with vision loss + hypopyon after recent intraocular surgery or intravitreal injection (or endogenous in immunocompromised/bacteremic) — endophthalmitis

Citations

- Mac Grory et al, Stroke 2021 — AHA Scientific Statement on Management of Central Retinal Artery Occlusion (CRAO = acute ischemic stroke) + Maz et al, Arthritis Rheumatol 2021 — ACR/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis + Optic Neuritis Treatment Trial (Beck NEJM 1992/1993; Arch Ophthalmol 1993) + EAGLE Trial (Schumacher Ophthalmology 2010, intra-arterial fibrinolysis NOT recommended) + Hayreh CRAO retinal-survival studies + AAO Preferred Practice Pattern (Retinal Vein Occlusions / Retinal Detachment, current cycle) [PMID:33677974](https://pubmed.ncbi.nlm.nih.gov/33677974/)
- Cited evidence (PMID 34235884) [PMID:34235884](https://pubmed.ncbi.nlm.nih.gov/34235884/)
- Cited evidence (PMID 1734247) [PMID:1734247](https://pubmed.ncbi.nlm.nih.gov/1734247/)
- Cited evidence (PMID 8232485) [PMID:8232485](https://pubmed.ncbi.nlm.nih.gov/8232485/)
- Cited evidence (PMID 8512477) [PMID:8512477](https://pubmed.ncbi.nlm.nih.gov/8512477/)

Last reconciled with current guidelines: 2026-05-17.
References
  • Mac Grory et al, Stroke 2021 — AHA Scientific Statement on Management of Central Retinal Artery Occlusion (CRAO = acute ischemic stroke) + Maz et al, Arthritis Rheumatol 2021 — ACR/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis + Optic Neuritis Treatment Trial (Beck NEJM 1992/1993; Arch Ophthalmol 1993) + EAGLE Trial (Schumacher Ophthalmology 2010, intra-arterial fibrinolysis NOT recommended) + Hayreh CRAO retinal-survival studies + AAO Preferred Practice Pattern (Retinal Vein Occlusions / Retinal Detachment, current cycle) — PMID:33677974
  • Cited evidence (PMID 34235884) — PMID:34235884
  • Cited evidence (PMID 1734247) — PMID:1734247
  • Cited evidence (PMID 8232485) — PMID:8232485
  • Cited evidence (PMID 8512477) — PMID:8512477