Acute (sudden) vision loss — neuro-ophthalmology / EM triage
NEURO-OPHTHALMOLOGY / EM TRIAGE engine — recognition + time-critical triage + emergency steroid / acute-CRAO-measures / IOP-lowering, then definitive pathway ownership routed OUT by engine_id (neuro.acute-stroke.core.v1, neuro.tia.core.v1, rheum.giant-cell-arteritis.core.v1, neuro.optic-neuritis.core.v1, ophtho.acute-angle-closure-glaucoma.core.v1, ophtho.retinal-detachment.core.v1, ophtho.endophthalmitis.core.v1). Not a duplicate of those engines — its job is the first-minutes triage and the two can-not-miss clocks (CRAO=stroke, GCA=steroid). Two foregrounded can-not-miss windows: CRAO is acute ischemic stroke (Mac Grory Stroke 2021 PMID 33677974) routed exactly like a hemispheric stroke/TIA; GCA/arteritic AION needs empiric high-dose corticosteroid BEFORE temporal-artery biopsy to save the fellow eye (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356). RxCUIs: prednisone 8640, methylprednisolone 6902, prednisolone 8638, acetazolamide 197 — used per the brief; all are the well-established RxNorm ingredient CUIs and are flagged for live RxNav re-confirmation next session (no fabricated codes; ocular massage / anterior-chamber paracentesis / hyperbaric / IV-thrombolysis-pathway / laser PI encoded as non_pharm). Bayesian linkage (pre-test priors by painful/painless × transient/persistent × monocular/binocular × age/vascular-risk; LR+/LR− for RAPD, cherry-red spot, pallid vs hyperemic disc edema, Hollenhorst plaque, jaw claudication/scalp tenderness, ESR/CRP/platelets for GCA; T_treat for empiric GCA steroid before biopsy; the arteritic-vs-non-arteritic AION pivot; cross-engine routing edges by engine_id) is documented in the co-located _design-brief.md + _research-bundle.md; first-class TS LR fields remain schema-blocked (same constraint as the gold-template dossiers). Effect sizes (≥5): CRAO retinal tolerance ~90-100 min before irreversible damage (Hayreh Ophthalmology 1980 PMID 6769079; ~97 min safe / 105 min irreparable Exp Eye Res 2004 PMID 15106952); CRAO concurrent acute brain infarction on DWI ~25-30% (Mac Grory Stroke 2021 PMID 33677974); ONTT 2-year definite-MS rate 7.5% IVMP vs 16.7% placebo, adjusted rate ratio 0.34 (95% CI 0.16-0.74), and oral-prednisone-alone recurrence RR 1.79 (95% CI 1.08-2.95) (Beck NEJM 1992/1993 PMIDs 1734247/8232485); GCA visual improvement ~34% after steroid with fellow-eye involvement seen only with oral (not IV) therapy in the series (Liu Ophthalmology 1994 PMID 7800356); EAGLE intra-arterial fibrinolysis adverse-event rate 37.1% vs 4.3% conservative with no efficacy gain (Schumacher Ophthalmology 2010 PMID 20609991); ONTT 1-year acuity ≥20/40 in 91-95% across all arms — steroid benefit is short-term only (Beck Arch Ophthalmol 1993 PMID 8512477).
Entry points (5)
- symptomSudden 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_painless_monocular_vision_loss
- symptomSudden 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)sudden_painful_vision_loss_with_red_eye_or_pain
- symptomTRANSIENT vision loss (amaurosis fugax / blackout, recovered) — retinal or cortical TIA equivalent; route to TIA/stroke workup (Mac Grory Stroke 2021, PMID 33677974)transient_monocular_or_binocular_vision_loss
- symptomBinocular field loss / homonymous hemianopia / "cortical blindness" with or without other neuro deficits — occipital/post-chiasmal stroke (Mac Grory Stroke 2021, PMID 33677974)binocular_field_loss_homonymous
- historyAge ≥50 with vision loss + new headache / jaw claudication / scalp tenderness / PMR — GCA emergency entry (empiric steroid before biopsy) (Maz ACR/VF 2021, PMID 34235884; Liu Ophthalmology 1994, PMID 7800356)age_over_50_with_headache_jaw_scalp_symptoms
Required inputs (19)
- pain_presentrequiredsymptom • used at ENTRYPainful 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_vs_persistentrequiredsymptom • used at ENTRYTransient (resolved) vision loss = amaurosis fugax / cortical TIA → TIA-equivalent workup; persistent = established CRAO/AION/RD/ON (Mac Grory Stroke 2021 PMID 33677974)
- monocular_vs_binocularrequiredsymptom • used at ENTRYMonocular → eye/optic-nerve to chiasm; binocular homonymous → post-chiasmal/occipital cortical lesion (stroke pathway) (Mac Grory Stroke 2021 PMID 33677974)
- agerequireddemographic • used at CONTEXTAge ≥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)
- gca_symptom_clusterrequiredsymptom • used at CONTEXTNew 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)
- time_since_onsetrequiredsymptom • used at CONTEXTRetinal 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)
- associated_neuro_deficitsrequiredsymptom • used at RED_FLAGSHemiparesis/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)
- rapd_relative_afferent_pupillary_defectrequiredsymptom • used at INITIAL_WORKUPA 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)
- fundus_findingsrequiredsymptom • used at INITIAL_WORKUPCherry-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)
- eye_pain_with_movementsymptom • used at CONTEXTPain on eye movement + central scotoma + dyschromatopsia in a younger patient is the optic-neuritis triad (Beck ONTT NEJM 1992 PMID 1734247)
- intraocular_pressurevital • used at INITIAL_WORKUPMarkedly elevated IOP with a red painful eye, mid-dilated pupil, haloes, nausea = acute angle-closure glaucoma — emergent IOP-lowering then route to glaucoma engine
- esrrequiredlab • used at INITIAL_WORKUPErythrocyte 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)
- crprequiredlab • used at INITIAL_WORKUPCRP 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)
- plateletslab • used at INITIAL_WORKUPThrombocytosis 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)
- vascular_risk_factorsrequiredhistory • used at CONTEXTAtherosclerosis/AF/HTN/DM/carotid disease — CRAO and amaurosis carry the same secondary-prevention urgency as cerebral stroke/TIA (Mac Grory Stroke 2021 PMID 33677974)
- demyelinating_or_nmosd_historyhistory • used at CONTEXTPrior MS/NMOSD/MOGAD or risk reframes optic neuritis and changes acute therapy (IVMP ± PLEX for severe/NMOSD) (Beck ONTT NEJM 1992 PMID 1734247)
- immunocompromisehistory • used at CONTEXTEndogenous endophthalmitis, CMV/atypical retinitis, and atypical optic neuropathies broaden the differential and lower the imaging/tap threshold
- pregnancyhistory • used at TREATMENTSteroid + neuro-imaging safety gating; pregnancy/postpartum also raises CVST/PRES/preeclampsia-related cortical visual loss
- recent_intraocular_surgery_or_injectionhistory • used at RED_FLAGSPost-operative / post-intravitreal-injection painful red eye with vision loss = endophthalmitis until proven otherwise — emergent tap-and-inject route
12-phase flow (12)
- 1FRAMEFrame 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.advance: emergency-triage framing set; CRAO and GCA clocks foregrounded
- 2ENTRYCapture the four triage binaries up front: painful vs painless, transient vs persistent, monocular vs binocular, ± other neuro deficits. These collapse the differential before any test (Biousse/Newman framework; Mac Grory Stroke 2021 PMID 33677974).inputs: pain_present, transient_vs_persistent, monocular_vs_binocularactions: workup.acute_vision_lossadvance: all four triage binaries recorded
- 3CONTEXTRisk substrate: age (≥50 → GCA prior up), the GCA symptom cluster (headache/jaw claudication/scalp tenderness/PMR), time since onset (retinal clock), vascular risk factors (CRAO/amaurosis = stroke-equivalent secondary-prevention urgency), demyelinating history, immunocompromise. This phase assigns the pretest priors.inputs: age, gca_symptom_cluster, time_since_onset, vascular_risk_factors, eye_pain_with_movement, demyelinating_or_nmosd_history, immunocompromiseactions: workup.gca_temporal_arteritisadvance: priors assigned by triage axes × age/vascular-risk; GCA cluster scored
- 4RED_FLAGSTime-critical screen: (1) CRAO or amaurosis or any focal neuro deficit → activate stroke pathway, route to neuro.acute-stroke.core.v1 / neuro.tia.core.v1 (Mac Grory Stroke 2021 PMID 33677974); (2) age ≥50 + GCA cluster → empiric high-dose steroid NOW, do not wait for ESR/biopsy (Maz ACR/VF 2021 PMID 34235884); (3) post-op/post-injection painful eye → endophthalmitis route; (4) acute angle-closure → emergent IOP-lowering. Recognised and started here; ownership routed OUT.inputs: associated_neuro_deficits, recent_intraocular_surgery_or_injectionactions: protocol.crao, protocol.stroke, workup.acute_strokeadvance: CRAO/stroke, GCA, endophthalmitis, AACG red flags screened and time-critical action initiated
- 5INITIAL_WORKUPBedside afferent exam: visual acuity each eye, RAPD (the key localising sign), confrontation fields, dilated fundus (cherry-red spot vs pallid disc edema vs blood-and-thunder vs detachment vs vitreous blood — Hayreh & Zimmerman Retina 2007 PMID 17460582), IOP, colour vision. STAT ESR + CRP + platelets + CBC if any GCA suspicion (results never delay steroid). Urgent neuro-imaging (DWI-MRI) for binocular/cortical or CRAO (concurrent brain infarction common).inputs: rapd_relative_afferent_pupillary_defect, fundus_findings, intraocular_pressure, esr, crp, plateletsactions: workup.acute_red_eye, panel.cbc, panel.inflammation, calc.nihssadvance: afferent exam + fundus + IOP done; GCA inflammatory labs sent; imaging ordered if indicated
- 6BRANCHING_WORKUPBranch by fundus + triage axes: cherry-red spot → CRAO (stroke pathway, GCA screen if ≥50); pallid disc edema + ≥50 + ↑ESR/CRP → arteritic AION (steroid emergency); hyperemic disc edema, <50, eye-movement pain, RAPD → optic neuritis (MRI brain/orbits, AQP4/MOG); blood-and-thunder → CRVO; macula-threatening detachment → RD (urgent vitreoretinal); dense vitreous blood → VH (B-scan); high IOP + closed angle → AACG; normal eye + homonymous field → occipital stroke (DWI-MRI). Temporal-artery biopsy / vascular imaging arranged but never gates steroid.inputs: fundus_findings, eye_pain_with_movementactions: workup.acute_stroke, workup.acute_headache, panel.cmpadvance: syndrome localised; confirmatory test arranged; emergency therapy not delayed by it
- 7DIFFERENTIALTerminal 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).advance: single best diagnosis selected; arteritic-vs-non-arteritic AION pivot explicitly resolved; co-existence (CRAO + GCA) flagged
- 8RISK_STRATIFICATIONStratify by sight- AND life-threat. CRAO/amaurosis: stroke risk — NIHSS for any neuro deficit, ABCD2 for transient retinal/cortical ischemia, urgent vascular workup (Mac Grory Stroke 2021 PMID 33677974). GCA: threatened/established vision loss → IV pulse vs oral high-dose decision (Maz ACR/VF 2021 PMID 34235884). RD: macula-on (same-day surgical emergency) vs macula-off (more time-tolerant). Systemic-instability overlay (NEWS2/qSOFA) for endogenous endophthalmitis/sepsis or stroke with deterioration.inputs: associated_neuro_deficits, time_since_onsetactions: calc.abcd2, calc.nihss, calc.news2, calc.qsofaadvance: sight-threat tier + stroke/life-threat tier assigned
- 9TREATMENTEmergency, etiology-stratified, ROUTE-OUT-after-stabilise: (1) GCA suspected → empiric high-dose corticosteroid IMMEDIATELY (oral prednisone ≥1 mg/kg, or IV methylprednisolone pulse if threatened/established vision loss) before biopsy, + aspirin, route to rheum.giant-cell-arteritis.core.v1 for taper/tocilizumab (Maz ACR/VF 2021 PMID 34235884; Liu Ophthalmology 1994 PMID 7800356). (2) CRAO → activate stroke pathway: ocular massage / IOP-lowering / anterior-chamber paracentesis are low-yield non-pharm temporising measures; IV thrombolysis within a tight window is the active stroke-pathway question (intra-arterial fibrinolysis NOT recommended — EAGLE Schumacher Ophthalmology 2010 PMID 20609991); route to neuro.acute-stroke.core.v1. (3) Optic neuritis → IV methylprednisolone speeds recovery; oral prednisone alone is contraindicated (↑recurrence) (Beck ONTT NEJM 1992/1993 PMIDs 1734247/8232485); route to neuro.optic-neuritis.core.v1. (4) AACG → emergent IOP-lowering (topical aqueous suppressants + acetazolamide ± osmotic), route to ophtho.acute-angle-closure-glaucoma.core.v1. (5) Endophthalmitis → emergent tap-and-inject route. Steroid/imaging pregnancy gating applied.inputs: pregnancy, gca_symptom_cluster, time_since_onsetadvance: etiology-specific emergency therapy started AND definitive ownership routed by engine_id
- 10DISPOSITIONCRAO / acute stroke / amaurosis → emergent ED + stroke unit (route to neuro.acute-stroke.core.v1 / neuro.tia.core.v1). GCA with vision loss → admit for IV pulse + urgent rheum/ophtho + biopsy ≤2 wk. Optic neuritis → admit/observation for IVMP + neurology. RD macula-on → same-day vitreoretinal surgery. Endophthalmitis → emergent ophthalmology. AACG → emergent ophthalmology after IOP control. Functional vision loss → reassurance + outpatient neuro-ophthalmology after organic causes excluded.inputs: associated_neuro_deficitsadvance: disposition + routed engine_id documented
- 11MONITORINGGCA: 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.inputs: esr, crpactions: panel.inflammationadvance: etiology-appropriate monitoring plan active or handed off to routed engine
- 12FOLLOWUPCRAO/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.inputs: vascular_risk_factors, demyelinating_or_nmosd_historyadvance: secondary-prevention / disease-specific follow-up handed to the routed engine_id