Sudden Painful Loss of Vision

Sudden or rapidly progressive visual loss accompanied by ocular, periocular or head pain. Many causes are sight threatening and have specific time critical treatments; the pain pattern, redness of the eye, and pupil findings steer diagnosis.

• Causes:
  • Red eye with pain: acute angle closure, microbial keratitis and corneal ulcer, endophthalmitis, uveitis (acute anterior, with hypopyon), scleritis (including necrotising), hyphaema, chemical and thermal injury, penetrating injury, neovascular glaucoma, and herpes zoster ophthalmicus.
  • White (quiet) eye with pain: optic neuritis (pain on eye movement), giant cell arteritis, posterior scleritis, orbital apex and cavernous sinus lesions, pituitary apoplexy, ischaemic optic neuropathy with pain, carotid dissection, and retrobulbar haemorrhage.
  • Proptosis with pain: orbital cellulitis and abscess, orbital compartment syndrome, thyroid eye disease, idiopathic orbital inflammation, cavernous sinus thrombosis.
• Contexts: recent surgery or injection (endophthalmitis), contact lens wear (keratitis), trauma, immunosuppression, sinusitis, systemic autoimmune disease.

• Acute angle closure: severe pain, headache, nausea and vomiting, haloes around lights, red eye, mid-dilated fixed oval pupil, corneal oedema, shallow anterior chamber, hard globe (intraocular pressure 40 to 80 mmHg).
• Microbial keratitis: pain, photophobia, discharge, white corneal infiltrate with epithelial defect, hypopyon; contact lens wear is the main risk factor.
• Endophthalmitis: marked pain, rapidly falling vision, hypopyon, vitritis, lid swelling, within days of surgery or injection or in an endogenous source.
• Optic neuritis: pain worse on eye movement, young adult, dyschromatopsia, central scotoma, relative afferent pupillary defect, normal or swollen disc.
• Scleritis: severe boring pain radiating to the face, violaceous scleral injection, tenderness, associated autoimmune disease.
• Giant cell arteritis: headache, jaw claudication, scalp tenderness, visual loss, diplopia.
• Orbital cellulitis: fever, proptosis, painful limited motility, chemosis, relative afferent pupillary defect.
• Cavernous sinus thrombosis and apex syndromes: bilateral progression, ophthalmoplegia, sensory loss, high fever.
• Uveitis: photophobia, ciliary flush, keratic precipitates, small irregular pupil, cells and flare.

• Systematic examination: acuity, pupils, intraocular pressure (except in open globe), slit lamp with fluorescein, gonioscopy where safe, dilated fundus examination, motility, proptosis, eyelid, and facial sensation.
• Immediate targeted tests: microbiological scrapes and cultures for keratitis; vitreous and aqueous tap for endophthalmitis; blood cultures for fever; ESR, CRP and platelets for giant cell arteritis; CT orbits and sinuses with contrast and MRI with venography for orbital and cavernous disease; MRI brain and orbits for optic neuritis and apex lesions; B-scan for posterior scleritis and vitreous disease; anterior segment optical coherence tomography for angle closure.
• Blood tests: full count, glucose, renal function, autoimmune screen (ANA, ANCA, rheumatoid factor), tuberculosis and syphilis tests as indicated.

• Acute angle closure (emergency): acetazolamide 500 mg IV (or orally), timolol 0.5 percent one drop, apraclonidine 1 percent or brimonidine 0.2 percent one drop, pilocarpine 2 percent one drop every 15 minutes for 2 doses once pressure has begun to fall, topical steroid (prednisolone acetate 1 percent), analgesia and antiemetics, and if pressure remains above 40 mmHg: mannitol 20 percent 1 to 2 g/kg IV over 30 minutes. Definitive laser peripheral iridotomy within 24 to 48 hours and prophylactic iridotomy of the fellow eye.
• Microbial keratitis: corneal scrape, then intensive broad spectrum drops every hour (fortified cefuroxime 5 percent with gentamicin 1.4 percent, or moxifloxacin 0.5 percent or levofloxacin 1.5 percent hourly), cycloplegia (atropine 1 percent), and tailoring to cultures; admit severe cases.
• Endophthalmitis: urgent vitreous tap and intravitreal vancomycin 1 mg in 0.1 mL with ceftazidime 2.25 mg in 0.1 mL (repeat at 48 to 72 hours as needed), vitrectomy for severe disease, and topical fortified antibiotics.
• Optic neuritis: methylprednisolone 1 g IV daily for 3 days (or oral 500 mg to 1250 mg daily for 3 to 5 days), with demyelinating disease evaluation.
• Giant cell arteritis: methylprednisolone 1 g IV daily for 3 days then prednisolone 1 mg/kg daily, regardless of biopsy.
• Scleritis: oral prednisolone 1 mg/kg daily for necrotising or severe disease with immunosuppression (methotrexate, cyclophosphamide, rituximab), or NSAIDs (flurbiprofen 100 mg three times daily, indomethacin 25 to 50 mg three times daily) for mild diffuse disease.
• Orbital cellulitis and abscess: admit; ceftriaxone 2 g IV daily plus metronidazole 500 mg IV every 8 hours (add vancomycin 15 to 20 mg/kg for suspected MRSA); drainage for abscess or visual compromise.
• Cavernous sinus thrombosis: broad spectrum antibiotics for 3 to 4 weeks IV, with anticoagulation.
• Retrobulbar haemorrhage and compartment syndrome: immediate lateral canthotomy and cantholysis.
• Anterior uveitis: prednisolone acetate 1 percent every 1 to 2 hours with taper, cyclopentolate 1 percent three times daily.
• Neovascular glaucoma: pressure lowering drops and acetazolamide, intravitreal anti-VEGF, panretinal photocoagulation, and drainage surgery.
• Review within 24 hours of any treatment decision, and admit patients with sight threatening infections, high pressure, or orbital disease.