Acute angle-closure glaucoma (AACG)
OPHTHALMOLOGY-EMERGENCY engine — the acute primary angle-closure crisis. Chronic open-angle/PACG IOP surveillance, the migraine pathway, and the SAH/headache-emergency pathway are recognised then routed OUT by engine_id (ophtho.acute-red-eye.core.v1, ophtho.acute-vision-loss.core.v1, neuro.migraine.core.v1, neuro.sah.core.v1, gim.deprescribing.core.v1) — not re-authored here. RxCUIs are the well-established RxNorm ingredient codes for the IOP-lowering bundle: timolol ophthalmic 8814, brimonidine 65373, pilocarpine 8404, acetazolamide 197, dorzolamide 224090, mannitol 6593, prednisolone ophthalmic 8638. Live RxNav re-confirmation is flagged for the next session (PubMed MCP was reached live this session; RxNav re-validation pending). Laser peripheral iridotomy / prophylactic fellow-eye LPI / EAGLE lens extraction / supine positioning / corneal indentation / drug-cessation are non_pharm by design (procedures/positioning/deprescribing). CRITICAL clinical-uncertainty notes: (1) pilocarpine is ineffective and may be harmful at IOP >40-50 mmHg (ischaemic iris sphincter) — sequence it AFTER aqueous suppressants; (2) topiramate/sulfonamide/daratumumab cause a NON-pupillary-block ciliochoroidal-effusion secondary closure where pilocarpine and iridotomy are contraindicated — treatment is drug cessation + cycloplegia + steroid; (3) ZAP (He Lancet 2019, HR 0.53) shows only MODEST prophylactic-LPI benefit in SCREEN-DETECTED suspects and does NOT govern the symptomatic post-crisis fellow eye, which carries a substantially higher attack risk and warrants prophylactic LPI. Bayesian linkage (pre-test AACG-vs-uveitis-vs-migraine-vs-conjunctivitis-vs-scleritis-vs-SAH priors by feature cluster, LR+/LR− for each cardinal sign — fixed mid-dilated pupil, corneal haze, raised IOP, shallow AC, halos — IOP-threshold→urgency mapping, conditional dependencies, decision thresholds, and cross-dossier 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 engine). Effect sizes (≥5): EAGLE clear-lens-extraction vs LPI+medical EQ-5D +0.052 (95% CI 0.015-0.088, p=0.005), IOP −1.18 mmHg (95% CI −1.99 to −0.38, p=0.004), ICER £14,284/QALY (Azuara-Blanco Lancet 2016 PMID 27707497); ZAP prophylactic-LPI incident angle-closure HR 0.53 (95% CI 0.30-0.92, p=0.024), incidence 4.19 vs 7.97 per 1000 eye-years (He Lancet 2019 PMID 30878226); ZAP post-mydriasis spontaneous AAC 0.44 per 1000 eye-years (95% CI 0.11-1.77) and 1 attack per 1587 dilations (Friedman Ophthalmol Glaucoma 2022 PMID 35568336); ZAP post-LPI cataract-progression HR 1.10 (95% CI 0.88-1.36, NS) (Chang Br J Ophthalmol 2022 PMID 35501120); IOP often 40-80 mmHg in crisis and reduction is the only proven means of preventing irreversible optic-nerve damage (Weinreb JAMA 2014 PMID 24825645).
Entry points (5)
- symptomSevere acute unilateral eye/brow pain with blurred vision and coloured halos around lights (classic AACG triad — AAO PPP Gedde Ophthalmology 2021 PMID 34933744)severe_unilateral_eye_pain_with_halos
- symptomAcute red painful eye with frontal headache and nausea/vomiting — the systemic-mimic presentation that masquerades as acute abdomen / migraine / gastroenteritis (Weinreb JAMA 2014 PMID 24825645)red_painful_eye_with_headache_and_vomiting
- imagingExam finding: red eye + mid-dilated fixed/oval poorly-reactive pupil + steamy/hazy cornea + shallow anterior chamber (cardinal AACG sign cluster — AAO PPP Gedde Ophthalmology 2021)mid_dilated_fixed_pupil_hazy_cornea_exam
- medicationNew topiramate / sulfonamide / sympathomimetic / anticholinergic / daratumumab with acute bilateral visual blurring + eye pain — secondary angle-closure entry (Agarwal Indian J Ophthalmol 2019 PMID 31436195; Edwards J Glaucoma 2020 PMID 32487951)precipitant_drug_with_acute_visual_symptoms
- historyHyperope / shallow anterior chamber / Asian ancestry with symptom onset in dim light, prone position, or stress (anatomic/demographic predisposition — AAO PPP Gedde Ophthalmology 2021)hyperopic_or_asian_ancestry_with_dim_light_onset
Required inputs (15)
- eye_pain_laterality_and_severityrequiredsymptom • used at ENTRYSevere acute unilateral eye/brow pain is the dominant AACG symptom; bilateral simultaneous onset shifts the prior toward drug-induced secondary angle closure (AAO PPP Gedde Ophthalmology 2021 PMID 34933744; Agarwal Indian J Ophthalmol 2019 PMID 31436195)
- coloured_halos_around_lightsrequiredsymptom • used at ENTRYColoured haloes around lights reflect corneal epithelial oedema from acutely raised IOP — a high-LR AACG pointer that is absent in conjunctivitis and uveitis (Weinreb JAMA 2014 PMID 24825645)
- nausea_vomiting_with_eye_painrequiredsymptom • used at CONTEXTVagally-mediated nausea/vomiting accompanies severe IOP elevation and is the feature that misdirects to acute abdomen / gastroenteritis / migraine; it also precludes oral CAI dosing (AAO PPP Gedde Ophthalmology 2021)
- pupil_shape_and_reactivityrequiredimaging • used at CONTEXTA mid-dilated (4-6 mm), vertically-oval, fixed/poorly-reactive pupil is near-pathognomonic for AACG and pivots away from uveitis (small/miotic pupil) and conjunctivitis (normal pupil) (AAO PPP Gedde Ophthalmology 2021)
- corneal_clarity_and_anterior_chamber_depthrequiredimaging • used at CONTEXTSteamy/hazy oedematous cornea + shallow/flat anterior chamber is the structural signature; a deep chamber and clear cornea argue strongly against AACG (AAO PPP Gedde Ophthalmology 2021)
- intraocular_pressurerequiredvital • used at CONTEXTIOP (often 40-80 mmHg in a crisis) defines diagnosis, urgency, and the IOP→time-to-irreversible-optic-nerve-damage curve; the single most actionable measurement (Weinreb JAMA 2014 PMID 24825645; AAO PPP Gedde Ophthalmology 2021)
- precipitant_drug_reviewrequiredmedication • used at CONTEXTTopiramate / sulfonamide / sympathomimetic / anticholinergic / TCA / antihistamine / daratumumab are precipitants; topiramate-class causes idiosyncratic bilateral ciliochoroidal-effusion secondary closure that does NOT respond to iridotomy (Agarwal Indian J Ophthalmol 2019 PMID 31436195; Alzendi Saudi J Ophthalmol 2021 PMID 34085015; Edwards J Glaucoma 2020 PMID 32487951)
- sulfonamide_allergyrequiredhistory • used at RED_FLAGSSulfonamide allergy gates systemic/topical carbonic-anhydrase-inhibitor use (acetazolamide, dorzolamide) — a core bundle drug (AAO PPP Gedde Ophthalmology 2021)
- thunderclap_or_worst_headache_featuresrequiredsymptom • used at RED_FLAGSThunderclap / "worst headache of life" / focal neurology mandates SAH exclusion before anchoring on AACG — recognise then route OUT to neuro.sah.core.v1 (Weinreb JAMA 2014 PMID 24825645)
- hyperopia_or_prior_angle_closurehistory • used at CONTEXTHyperopia / short axial length / prior angle-closure episode / family history elevates the pretest prior and flags the anatomically-crowded fellow eye for prophylactic iridotomy (AAO PPP Gedde Ophthalmology 2021)
- pregnancyhistory • used at TREATMENTPregnancy gates acetazolamide (avoid first trimester / near term) and mannitol (volume/teratogenic caution) in the IOP-lowering bundle (AAO PPP Gedde Ophthalmology 2021)
- creatininelab • used at TREATMENTRenal function gates acetazolamide and mannitol dosing/contraindication (anuria, severe renal impairment) (AAO PPP Gedde Ophthalmology 2021)
- cardiac_pulmonary_comorbidityhistory • used at TREATMENTHeart failure / asthma-COPD / heart block gates systemic carryover of topical timolol (β-blockade) and mannitol volume load (Weinreb JAMA 2014 PMID 24825645)
- electrolytes_and_glucoselab • used at INITIAL_WORKUPAcetazolamide causes hypokalaemia/metabolic acidosis; mannitol shifts volume/osmolality — baseline electrolytes/glucose support safe bundle delivery and monitoring (AAO PPP Gedde Ophthalmology 2021)
- gonioscopy_or_anterior_segment_imagingimaging • used at BRANCHING_WORKUPGonioscopy (when cornea clears) ± anterior-segment OCT / UBM confirms appositional angle closure and distinguishes pupillary-block from plateau-iris / ciliochoroidal-effusion (lens-vault) mechanisms — drives definitive procedure choice (AAO PPP Gedde Ophthalmology 2021; Emanuel Curr Opin Ophthalmol 2014 PMID 24463418)
12-phase flow (12)
- 1FRAMEFrame as a SIGHT-THREATENING ophthalmic emergency masquerading as a systemic illness — the trap is mislabelling severe eye pain + headache + vomiting as acute abdomen, migraine, or gastroenteritis (Weinreb JAMA 2014 PMID 24825645). IOP reduction is the only proven means of preventing irreversible optic-nerve damage; chronic open-angle management, the migraine pathway, and the SAH pathway are routed OUT, not authored here.advance: AACG-emergency scope confirmed; not-this-engine pathways routed by engine_id
- 2ENTRYRecognise the classic triad (severe unilateral eye/brow pain + blurred vision + coloured halos) OR the systemic-mimic presentation (red eye + frontal headache + nausea/vomiting) OR a precipitant-drug entry; capture laterality and halos up frontinputs: eye_pain_laterality_and_severity, coloured_halos_around_lightsactions: workup.acute_red_eyeadvance: entry trigger present; laterality + halos recorded
- 3CONTEXTBuild the AACG prior: the cardinal sign cluster (mid-dilated fixed/oval pupil, steamy cornea, shallow AC, IOP often 40-80 mmHg), the nausea/vomiting that misdirects and precludes oral CAI, anatomic/demographic predisposition (hyperopia, Asian ancestry, prior episode), and the precipitant-drug review (bilateral simultaneous → secondary/ciliochoroidal-effusion mechanism)inputs: nausea_vomiting_with_eye_pain, pupil_shape_and_reactivity, corneal_clarity_and_anterior_chamber_depth, intraocular_pressure, precipitant_drug_review, hyperopia_or_prior_angle_closureactions: workup.acute_red_eyeadvance: cardinal cluster + IOP + pretest prior + mechanism (pupillary-block vs secondary) assigned
- 4RED_FLAGSTime-critical screen: IOP at the level/duration that risks irreversible optic-nerve damage → STAT ophthalmology + immediate medical IOP-lowering bundle; thunderclap/worst-headache/focal-neurology → recognise then route OUT to neuro.sah.core.v1; sulfonamide allergy → gate carbonic-anhydrase inhibitors before they are orderedinputs: thunderclap_or_worst_headache_features, sulfonamide_allergyactions: protocol.angle_closure, calc.news2advance: optic-nerve-damage time window flagged + STAT ophtho activated; SAH mimic screened/routed; CAI allergy gate set
- 5INITIAL_WORKUPConfirm by IOP (tonometry) + slit-lamp/penlight (cornea, pupil, AC depth); baseline electrolytes, glucose, renal function to deliver acetazolamide/mannitol safely; CBC/CMP if systemic toxicity or admission anticipated; ECG/vitals if mannitol volume load planned in cardiac patientsinputs: electrolytes_and_glucose, creatinineactions: panel.cmp, panel.renaladvance: IOP + anterior-segment exam documented; baseline labs sent; medical bundle initiated
- 6BRANCHING_WORKUPMechanism decision: pupillary-block primary closure (LPI definitive) vs plateau iris (iridoplasty) vs lens-vault/phacomorphic (lens extraction — EAGLE) vs ciliochoroidal-effusion drug-induced secondary closure (STOP drug + cycloplegia + topical steroid — iridotomy does NOT help). Gonioscopy ± AS-OCT/UBM once cornea clears; route precipitant-drug review out to gim.deprescribing.core.v1inputs: gonioscopy_or_anterior_segment_imagingactions: workup.acute_vision_lossadvance: mechanism classified; secondary/drug-induced separated from primary pupillary-block; precipitant review routed
- 7DIFFERENTIALTerminal differential of the red painful eye with headache/vomiting, with named pivots: AACG (mid-dilated fixed pupil + steamy cornea + raised IOP + shallow AC + halos pivot) vs acute anterior uveitis (small/miotic pupil + hypopyon/cells + normal-or-low IOP pivot) vs migraine/cluster headache (normal eye exam + normal IOP + photophobia-without-corneal-oedema pivot) vs conjunctivitis (discharge + normal pupil + normal IOP + no pain pivot) vs scleritis (boring pain + scleral violaceous hue + normal IOP pivot) vs subarachnoid haemorrhage (thunderclap + neurology + normal IOP → route to neuro.sah.core.v1 pivot)actions: workup.acute_headacheadvance: single best diagnosis selected; SAH/secondary-cause mimics excluded or routed
- 8RISK_STRATIFICATIONSeverity by IOP magnitude × duration of attack × optic-nerve/visual-field status × bilateral or fellow-eye anatomic risk × refractoriness to the medical bundle; very high IOP for prolonged duration or non-response within ~1 h of the bundle → surgical/laser urgency upgrade and inpatient/transfer dispositioninputs: intraocular_pressureactions: calc.news2advance: IOP-duration severity + fellow-eye risk + refractoriness assigned
- 9TREATMENTSTEPWISE medical IOP-lowering bundle as a temporising bridge to definitive laser: (1) supine positioning + corneal indentation; (2) topical timolol + brimonidine + pilocarpine (pilocarpine ineffective if IOP >40-50 mmHg due to iris-sphincter ischaemia — give after IOP partly lowered) + topical prednisolone; (3) systemic/topical acetazolamide/dorzolamide (sulfonamide-allergy and renal gating); (4) hyperosmotic IV mannitol if refractory (cardiac/renal/pregnancy gating); then DEFINITIVE laser peripheral iridotomy of the affected eye once cornea clears, PROPHYLACTIC LPI of the anatomically-narrow fellow eye, and consider EAGLE early-lens-extraction. Drug-induced secondary closure → STOP precipitant + cycloplegia + topical steroid (NOT pilocarpine, NOT iridotomy).inputs: pregnancy, creatinine, cardiac_pulmonary_comorbidity, sulfonamide_allergyactions: protocol.angle_closureadvance: medical bundle delivered + IOP trending down; definitive laser PI arranged; fellow-eye prophylaxis planned; precipitant stopped if secondary
- 10DISPOSITIONSTAT same-day ophthalmology is mandatory in all cases. Refractory IOP, prolonged attack, bilateral/secondary closure needing systemic therapy, or vomiting precluding oral therapy → admit/transfer for IV mannitol + urgent laser/surgery; responsive primary pupillary-block with arranged same-day LPI and reliable follow-up → may complete laser as urgent outpatient (ophthalmology-directed). Pure migraine/conjunctivitis/SAH-mimic confirmed → exit AACG pathway, route by engine_id.inputs: intraocular_pressureadvance: disposition documented; definitive laser timing and fellow-eye plan confirmed
- 11MONITORINGRecheck IOP at 30-60 min intervals during the bundle; expect a meaningful fall within ~1 h on an effective bundle — failure to break the attack mandates escalation to mannitol then urgent laser/surgery, not silent continuation; monitor for acetazolamide hypokalaemia/acidosis and mannitol volume overload/electrolyte shift; post-LPI IOP check and patency confirmationinputs: intraocular_pressure, electrolytes_and_glucoseactions: panel.cmpadvance: IOP broken and trending to safe range OR escalation triggered
- 12FOLLOWUPDefinitive LPI of the affected eye and PROPHYLACTIC LPI of the anatomically-narrow fellow eye (untreated fellow-eye attack risk is substantial in symptomatic angle closure — distinct from screen-detected ZAP suspects where prophylaxis benefit is modest, He Lancet 2019 PMID 30878226); EAGLE early-lens-extraction discussion in lens-driven disease (Azuara-Blanco Lancet 2016 PMID 27707497); lifelong glaucoma surveillance for residual optic-nerve damage / chronic angle closure; permanent documentation + deprescribing of the precipitant drug and patient warning card; route chronic IOP care and precipitant medication review out by engine_idinputs: precipitant_drug_review, hyperopia_or_prior_angle_closureadvance: definitive + fellow-eye laser plan documented; precipitant deprescribed/flagged; chronic glaucoma surveillance arranged