Causes of Sudden Painless Loss of vision

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There are various causes of Loss of vision; It can be painful or painless. This article summarizes the cause of painless Sudden loss of Vision

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Causes and management of sudden Painless loss of vision

Sudden loss of vision (SLOV) can be devastating to a patient as it happens when they least expect it. SLOV can be painless or painful.

Painless loss of vision, if not diagnosed and treated promptly and appropriately, can result in painful blind eyes. Usually, unilateral loss of vision indicates a localized pathology while bilateral can be intracranial or vascular.

The general consensus is, if the eye is white and looks normal, there is a high chance of blindness


History

Irrespective of painless of painful loss of vision, History must be thorough, detailed and must be carefully documented.

  1. Initial History is crucial for diagnosis, further management and also for medico legal purposes.
  2. Time of onset and duration- sudden or gradual; seconds, minutes, hours, days. Weeks or months
  3. Laterality: Unilateral or Bilateral
  4. Description of vision loss- Scotoma, metamorphopsia, curtain/veil like vision loss, accompanied with aura, total loss with no recovery
  5. Fluctuation of vision: pattern change and pain variation with change in posture
  6. Type of pain: Use subjective pain scale measure from 1-10 to understand the severity. Nature of pain:(throbbing, pulsating, shearing, retro orbital and with eye movements- Direction) If they are accompanied by photophobia, headache, jaw claudication, myalgia, mastication, fever.
  7. Previous similar history and mechanism of loss of vision and treatment; stroke, migraine, amaurosis, ocular inflammation
  8. History of trauma- Blunt, penetrating, perforating, mechanism of trauma.
  9. Previous ocular surgeries or interventions like intravitreal injections or use of steroid implants or laser (YAG, Argon, SLT) in the eyes; Use of eye drops- indications, frequency and duration.
  10. General medical history: Diabetes, Hypertension, cholesterol, thyroid status, cardiovascular, neurological, neurosurgical, vascular, endocrine, connective tissue, cancer, chemotherapy and radiotherapy
  11. Social history: Smoking, Alcohol, Substance abuse, Sexual history
  12. Drug history: Many drugs are known to directly impair vision. All the current medications and dosage must be documented: (Example: if there is a history of use of Warfarin, check for INR status, duration of warfarin and the dosage.)
  13. Allergy history- Severity of reaction to the allergic drug

Examination

Systematic and meticulous examination will help in clinching the diagnosis

Observe: Behavior, Habitus, facial symmetry, Globe position

Record: Best corrected visual acuity for each eye- with glasses, with pinhole, distance and near

Note and document: Obvious facial or ocular anomalies, Ptosis, proptosis, lid position, swelling or hyperemia, symmetry, lacerations or lagophthalmos,

Color vision – with pseudo-Ishihara chart for quick screening, especially if neurological cause is suspected. If poor vision of involved eye, record color vision for the ‘good’ eye.

Confrontational visual field check: Useful in office set up to understand the pattern, field involved and the extent of peripheral or central visual loss.

Pupil; check for Size, Shape and Symmetry. Look for sluggish reaction to light, Anisocoria or RAPD

Conjunctival and corneal sensation: Useful to also check for supra and infra orbital sensations

Extra ocular eye movements- Painful or Painless; if pain in which direction and if consistent. Question on Increase in pain after exercise or hot shower. Use Subjective pain scale.

Slit lamp examination: Examination of anterior and posterior segment slit lamp examination arrows down the diagnosis and This must include a quick adnexal examination, Hyperemia and swelling and ecchymosis of eyelids, hyperemia and swelling of the conjunctiva, episcleral or scleral vessels- (If scleral- document as sectoral or diffuse, necrotizing or non-necrotizing. Check for features of Thinning, melting, perforation and uveal tissue prolapse status; if any of the above seen, record the number of clock hours involved; presence of cork screw vessels) Note of symblepharon, discharge, membranes/pseudo membrane in relevant cases

Limbus: Assessment of limbal inflammation, scarcity of limbal cells, ischemia- number of clock hours involved, signs of trauma, chemical injury

Cornea: Examination without and later with fluorescein stain for foreign body, corneal abrasion, depth of corneal involvement, presence of ulcer, corneal haze, corneal thinning, extent and measurement of infiltrate or ulcer, opacity, scarring, pigmentation and vascularization. Check for Siedel’s. Photo document is preferred practice for objective measurement of lesion if cornea is involved.

Anterior chamber: Look carefully for presence of cells, flare, fibrin, pigments, hypopyon, Hyphemia, foreign body, tumors Van-Herricks grading of chamber, Anterior chamber depth assessment with Smith technique

Iris: Iris stromal features, anterior segment tumors, neovascularization, iris atrophy, Iris bombe, Transillumination, atrophy and

Pupil: Anisocoria, RAPD, presence of synechiae, pseudo exfoliation material, pupil shape, symmetry and size in dim and ambient light. The source of light must be adequately bright and the check must be done both in ambient and dim light.

Lens: subluxation, dislocation, lens capsule and the zonule status, cataract- lens opacity can be quantified by LOC III grading

Retrolental: cells, flare, bleed. One must take advantage of slit lamp tilt to view the vitreous better

Posterior segment: Check for media clarity, vitreous status, presence of Weiss ring, posterior pole for optic nerve, spontaneous venous pulsation, Papillo macular bundle, macula, retina, arteries, veins, peripheral and equatorial retina with 90D/78D. Assess the choroidal status. Three mirror lens assessment and Indirect ophthalmoscope with indentation will add value.

Optic nerve: size, shape, margins, contour, neuro retinal rim health, peripapillary atrophy, CD ratio, venous pulsation, nerve bleed, hyperemia, swelling and pallor to be checked and documented

Systemic examination based on the cause- to include Central nervous system, cranial nerve examination, respiratory system, cardiovascular system, endocrinological, radiological and vascular system examination as part of multi-disciplinary examination

If functional vision loss suspected – VA, color check, mirror test, prism bar test, Visual field examination, EDT

Causes

Painless cause of vision loss

1 . Vitreous hemorrhage[1]:

Rule out Trauma

Common painless cause of VH is
  • Proliferative Diabetic retinopathy
  • Retinal vein Occlusion (CRVO, BRVO, HRVO)
  • Retinal detachment with traction
  • Retinal tears (especially Horse shoe tear with vessel traction)
  • Choroidal neovascularization
  • Hemorrhagic Posterior vitreous detachment
  • Proliferative vitreo-retinopathy
Other causes, not as common are
  • Hypertensive retinopathy – most commonly retinal artery macro aneurysm, in the first vascular arcade.
  • Eales disease
  • ROP/FEVR
  • Macular or retinal telangiectasia
  • Localized causes- like uveitis (can be painless or painful: Bechet's, PAN, Sarcoidosis, syphilis, POHS, toxoplasmosis, MFC, etc.;), Retinitis (Sheathing, exudates, vascular leakage)
  • Blood dyscrasia and hemoglobinopathies
  • Valsalva retinopathy

Painful causes of vitreous hemorrhage can be secondary to trauma, chemical injury, Ocular ischemic syndrome, Terson’s syndrome, Sickle cell hemoglobinopathy, GCA, later stage of RVO’s, posterior uveitis and angle closure NVG

Investigations

Ocular

Ultra sound B scan with A scan overlay- gives a clue to aid in diagnosis

  • low reflectivity on A scan suggests PVD; attachment of vitreous to the posterior pole, - partial PVD
  • High reflectivity – Retinal detachment – if with tenting into vitreous- tractional detachment, smooth and convex- Rhegmatogenous detachment, Dome shaped- choroidal or exudative retinal detachment;
  • Elevated posterior vitreous phase with delay in movement with ocular motility- sub retinal hemorrhage
  • AS-OCT- if angle or AC involved secondary to VH
  • Gonioscopy
  • Fundus fluorescein angiogram,
  • OCT/OCT-Angio- optic nerve head and macula- if media is clear
  • Fundus photograph
  • Brain and orbit imaging- in trauma, foreign body, tumors
Systemic

Blood sugar, CBC, bleeding time, clotting time, Sicke cell status, hemoglobinopathy assessment, INR check, uveitis work-up (if cause), ECG, carotid doppler scan, Chest X ray and Echocardiogram.

Treatment

  • Conservative: One or more of the following, depending on the cause of vitreous bleed.
  • Ocular: Observation with follow up if minimal hemorrhage with no surgical cause. Bed rest, head end elevation, avoid NSAIDs, blood thinners, if possible
  • Intravitreal injections with anti VEGF or steroid implants.
  • Argon laser –focal or sectoral or pan retinal photocoagulation
  • Surgical: PPV with vitreous lavage +/- gas or silicone oil tamponade and +/- Endo laser +/- Cryotherapy
  • Anti-Glaucoma medication in relevant cases
  • General management: Glycemic control, Blood pressure control, cholesterol optimization, review of systems.
  • Prognosis: Depends on the cause.

2. Retinal detachment and retinal tear[2]:

Retinal tear and rhegmatogenous retinal detachment are symptomatic.

If macula is involved or if there is combined RRD/TRD or with vitreous hemorrhage, there may be sudden loss of VA, mostly painless.

Rhegmatogenous retinal detachment:

Assess PVD; Be aware that low or moderate myopes with previous recent eye surgery; RRD in another eye are high risk

Other uncommon causes: connective tissue anomalies like Stickler syndrome, Marfan’s, Ehler-Danlo's syndrome vitreo-retinopathies like Wagners and Jansen's syndrome, homocystinuria, acute retinal necrosis syndrome; Pars-planitis and ocular toxoplasmosis,

Fundus: SRF that is dome shaped, freely mobile retina that is convex and corrugated associated with breaks or tears and the fluid following Lincoffs' rule. Most common site is superior temporal quadrant. There may be more than one break if carefully examined. The field defect is relative with no laser uptake (contrary to retinoschisis), if chronic associated with pigment demarcation lines.

Exudative retinal detachment:

The cause of this detachment is subretinal. [3]Central vision is spared until late. Peripheral visual field defect and if associated with inflammation, Pain is the common presenting symptom.

The features are that of spontaneous detachment Clinically on examination, retina is dome shaped, smooth and convex with no associated tears or breaks associated with shifting and position dependent sub retinal fluid.


Examine to check for Nonophthalmic eyes, UES, FEVR, Coat’s, CSR, Retinoblastoma, hypertensive retinopathy, choroidal colobomas, choroidal tumors, lymphoma, secondaries, look for excessive cryotherapy or recent pan retinal photocoagulation scars, Examine sclera for scleral buckle, check for connective tissue disorders, Chronic renal disease, DIC. (ERD may be painful in: IOID, posterior scleritis, active inflammatory uveitis like sarcoidosis, post operative inflammation, Lyme, syphilis, CMV, Toxoplasmosis, ).

Tractional Retinal detachment:[4]

Unlike rhegmatogenous and exudative retinal detachment, traction detachment is concave with shallow SRF distribution. The retina is rigid, stretched ischemic and may be associated with fibrovascular gliosis. There may be significant pigment disturbance

Causes are: proliferative diabetic retinopathy, Burnt out and overly treated PDR, Coats disease, ROP, PVR,long standing or untreated RVO’s, Sickle cell retinopathy, FEVR, VMT, retinal dysplasia and retinal X syndrome

Management:

Conservative for Exudative retinal detachment:

  • infection: broad spectrum antibiotics[5]; Inflammation: ocular or systemic steroids and/ or immunosuppressants. If associated with exudates or neovascularization: anti-VEGF; If Tumor: chemotherapy, radiosurgery or selective surgery if localized.
  • Surgery RRD/ERD: PPV with one of the following options, scleral buckle, pneumo-retinopexy[6], gas or silicone oil or Densiron tamponade, SRF drainage, delamination, membrane dissection, relaxing retinectomy or segmentation with or without anti-VEGF injections.
  • If PVR – Offer poor prognosis [7]

Central retinal artery occlusion/Branch retinal artery occlusion

H/o Sudden, painless loss of vision

Causes:
  • Carotid artery[8] and CVS causes

Carotid emboli; Cholesterol emboli (Hollenhorst retinal arteriolar plaques); Fibrin platelet emboli (can cause amaurosis fugax) ;Calcific emboli; Cardiac emboli (younger patients);Thrombi -from myocardial infarct or mitral or aortic valve stenosis; Vegetative thrombi from bacterial endocarditis; Myxomatous thrombi from atrial myxoma

  • Vascular

Systemic lupus, polyarteritis nodosa, Protein C deficiency, protein S deficiencies, antithrombin-III deficiency, anti-phospholipid syndrome, Hyperhomocystenemia,

  • Ocular

Rule out Painful causes of CRAO: ↑ IOP of more than 60mmHg, GCA, retrobulbar hemorrhage, intra Orbital compressive tumor, IOID, RD surgery especially with scleral buckle or large volume of gas in eyes, Sickling haemoglobinopathies, Vasospasm (migraine) and Susac syndrome

Ocular associations with CRAO are; Optic nerve head drusen, retinal migraine.

Management:

Urgent stroke team referral and vascular specialist referral; Start anticoagulants if atrial fibrillation is the cause,

Central retinal vein occlusion/Branch retinal vein occlusion/Hemi-retinal vein occlusion

Presentation can be painless which can become painful blind eye that can lead to painful phthisis if not managed appropriately at early stages

Risk factors:
  • Age>65 years,
  • Hypertension,
  • diabetes,
  • hypercholesterolemia,
  • atherosclerosis,
  • obesity,
  • Smoking
  • OCP[9]
  • Glaucoma
Uncommon causes

Dehydration, Myeloproliferative disorders (Myeloma, polycythemia, sickle cell disease), hyperhomocystenemia, protein S, protein C, anti-thrombin deficiency, factor V Leiden mutation, Walden Strom's macroglobulinemia, anti-phospholipid antibodies, inflammatory causes – secondary RVO due to occlusive Vaso phlebitis- SLE, PAN, Bechet's GPA, Sarcoidosis, Good pastures, Chronic renal failure[10]

Assessment must be multi-disciplinary:

Investigations:
Ocular:

FFA: collateral confirmation, non-perfusion and delayed filling in ischemia, macular edema, leakage at neovascularization sites,

OCT: Macular edema, macular thickening, Intra retinal fluid, cystoid edema, sub retinal fluid, Epiretinal membrane in chronic cases.

Systemic

BP, FBC, ESR, blood sugar, U&E and lipids for all patients.

There is no need for an extensive investigation unless warranted and they include CRP (if GCA, inflammation), ANA, RF, ANCA, serum ACE, thrombophilia screening (protein S, protein C, factor V, anti-thrombin) anti-DNA, CXR, anti-cardiolipin, syphilis /Lyme screening, routine and exhaustive blood check offers limited yield and therefore not required.

Treatment:

Ischemic without NV: close monitoring and 3 monthly follow up for up to years, then consider discharge- beware of ‘100-day glaucoma’[11]

With NV: PRP +/- intravitreal injections; Steroid implants in the eye. Micro pulse laser not much in use but can be tried in non-responsive patients.

Adjunct treatment: Cycloplegic drops; Lubricants for compromised epithelium, steroid eye drops if eye inflamed, ↓ IOP with anti-glaucoma drops.

if no response to anti Glaucoma drops- consider cryoablation/valve surgery if no response;

painful blind eye with nil visual potential- retrobulbar alcohol, evisceration/enucleation.

Amaurosis Fugax

The Amaurosis Fugax Study Group classification for cause of TIA[12]

  • Embolic- most common cause of TIA
  • Hemodynamic
  • Ocular
  • Neurologic
  • Idiopathic
Causes

Carotid artery atherosclerosis,

Carotid artery emboli

Cardiac causes of emboli

Vasculitis

Coagulation disorders

Gaze evoked amaurosis

  • Optic nerve drusen
  • Papilledema

Painful amaurosis (Amaurosis Fugax, by definition is strictly painless, however there are causes that can mimic TIA warranting urgent stroke team referral and emergency management)

  • GCA
  • Migraine
  • Anterior ischemic optic neuropathy
  • Raised IOP

H/O curtain like loss of vision – usually lasting seconds to minutes but rarely hours. The loss of vision can happen multiple times in a day. Recovery is of the same pattern as the vision loss but it is usually slower. Vision loss can be generalized (systemic) or gaze evoked (localized cause- orbit or retro orbital lesion). While commonly uniocular, binocular causes have been documented. Commonly known as ‘mini stroke ‘in the eyes,

The diagnosis is MDT. Common cause is optic nerve transient hypoperfusion[13]-

Arrange for urgent stroke team referral to start on high dose anti platelet, anti-coagulants. If plaques suspected from carotid, urgent Vascular surgeon opinion

CNV with large macular bleed:

There are multiple causes for macular bleed but age-related macular degeneration bleed is the most common

The history is that of sudden painless loss of vision with gray scotoma or ‘blind spot’ in the central field of vision, metamorphopsia.

Ask for age (wet AMD), Refractive status (myopic CNVM, Polypoidal choroidal vasculopathy), connective tissue disorder ( Angioid streaks); ocular tumors (hemangioma, melanoma), post pan retinal photocoagulation.

Treatment:
  • Anti-VEGF's, PDT- if sub foveal/PCV with variable outcomes)
  • Surgical: pneumatic displacement of sub macular bleed[14] with perfluorocarbon +/- recombinant tissue plasminogen activator with good success.
Prognosis:

Depends on the cause and response to treatment.

Papilledema

The term papilledema is specific to optic nerve swelling secondary to space occupying lesion in the brain.[15]

Check for: color vision, red desaturation, saccades, pursuits, accommodation, corneal sensation, bells phenomenon, nystagmus, RAPD, gaze evoked amaurosis, IOP check, look for proptosis (axial, Non-axial) ) and ptosis

Stereo disc evaluation shows bilateral disc swelling (use Friesen scale grading for staging), loss of spontaneous venous pulsation, peripapillary hemorrhages, retino-choroidal folds, collaterals, shunt vessels and optic atrophy.

Investigate:

Cardiac status, previous history of Intra cranial bleed or tumors, hyper coagulation or bleeding/coagulation abnormalities,

Check for BMI.

MRI with contrast,MRV (for sinus thrombosis, venous pathology), LP after MRI

Ocular:

FFA,,OCT of the optic nerve, stereo photo for optic nerve, Humpfreys visual fields

Treatment:

Shared care- Neurologist, neurosurgeons, endocrinologist, radiologists, ENT and ophthalmologists play a role as part of multi-disciplinary team work up

Spontaneous ( painless) Hyphema

  • Rule out sickle cell disease, OIS and trauma
  • Chronic glaucoma-NVG (painful in later stages)
  • tumors of the eye- Retinoblastoma, Iris melanoma
  • Long standing anterior uveitis ( patient will have Photophobia)
  • vascular anomalies –juvenile Xanthogranuloma
  • Neovascularization of iris- due to any cause
  • Myotonic dystrophy
  • Lympho-proliferative or myelo-proliferative disorders: lymphoma, Leukemia, Hemophilia, Von Willibrand disease
  • Drugs; Warfarin, Aspirin
  • UGH syndrome
Examination:

Assess the size of hyphema ( one quarter, quarter, half or complete),Look for the cause of bleed in spontaneous hyphema. Be aware that bleed can happen again after five days or during clot lysis. Check for

Corneal edema or haze

Corneal blood staining – May cause hemosiderosis

Risk of amblyopia in children if more than 50% anterior chamber involved for more than 5 days[16]

↑ IOP- Secondary Glaucoma

Assess fundus if view is clear or perform ultrasound B-scan to check for posterior segment bleed or tumor.

Management
Conservative:
  • Admit
  • Bed rest,
  • Head elevation
  • Topical steroids and cycloplegic agents
  • Topical glaucoma medication
  • Consider using Aminocaproic acid • Tranexamic acid
  • Avoid aspirin,NSAIDS and warfarin after cardiology opinion
Surgery:
  • AC paracentesis and washout
  • Clot expression and AC wash-out

If IOP still high,

  • trabeculectomy

Functional vision loss:

History of sudden severe loss of bilateral vision – but no correlation to history of examination. Patient navigates easily around the room when no one around to observe. Recent stressful event-psychosomatic conversion.

Assessment:

Normal anterior and posterior segments, normal pupil, normal neurological examination. normal EDT and neuroimaging. Diagnosis must be made only after excluding organic causes.

Tests to confirm: OKN: shows normal corrective saccades, prism tests: normal breaking of fusion; Goldman perimetry: spiraling or significant peripheral constriction of visual fields, Ishihara: Inconsistent response. Crossed cylinder technique, and fogging technique to reinforce functional loss objectively

Management:

Counselling, reassurance, encouragement. Referral to a specialist with experience in handling patients with functional visual loss

Purtscher's like retinopathy:[17]

While putschers retinopathy is common in head injury, putschers like retinopathy is a term used for non-traumatic conditions like acute pancreatitis, fat embolism, post-partum, long bone fractures, severe generalized vasospasm, Hemolytic uremia syndrome, Renal failure or thoracic and abdominal crush injury resulting in sudden severe loss of painless vision, retinal whitening (usually at the junctions between artery and vein), cotton wool spots, retinal hemorrhage, retinal vascular sheathing, vessel attenuation, RPE changes and eventually optic nerve pallor.

Investigation:

MDT approach based on cause Ocular examinations include FFA, Fundus photo, OCT and ERG ( reduced A and B waves).

No treatment options available. IV steroids have little or limited evidence in treatment

References:

  1. Regillo, C. D., et al. "Basic and Clinical Science Course, Section 12: Retina and Vitreous." San Francisco, American Academy of Ophthalmology (2011).
  2. Colucciello, Michael. "Rhegmatogenous retinal detachment." The Physician and sportsmedicine 37.2 (2009): 59-65.
  3. Gariano, Ray F., and Chang-Hee Kim. "Evaluation and management of suspected retinal detachment." American family physician 69.7 (2004): 1691-1698
  4. Solinski, Mark A., et al. "13. Tractional retinal detachments." Disease-a-Month 67.5 (2021): 101142.
  5. Wolfensberger, Thomas J., and Adnan Tufail. "Systemic disorders associated with detachment of the neurosensory retina and retinal pigment epithelium." Current opinion in ophthalmology 11.6 (2000): 455-461.
  6. Yannuzzi, Nicolas A., et al. "Clinical outcomes of rhegmatogenous retinal detachment treated with pneumatic retinopexy." JAMA ophthalmology 139.8 (2021): 848-853.
  7. Pastor, J. Carlos. "Proliferative vitreoretinopathy: an overview." Survey of ophthalmology 43.1 (1998): 3-18.
  8. Douglas, DANIEL J., et al. "The association of central retinal artery occlusion and extracranial carotid artery disease." Annals of surgery 208.1 (1988): 85.
  9. Stowe 3rd, G. C., Z. Nicholas Zakov, and Daniel M. Albert. "Central retinal vascular occlusion associated with oral contraceptives." American journal of ophthalmology 86.6 (1978): 798-801.
  10. Hayreh, Sohan Singh, et al. "Systemic diseases associated with various types of retinal vein occlusion." American journal of ophthalmology 131.1 (2001): 61-77.
  11. Rong, Andrew J., et al. "Predictors of neovascular glaucoma in central retinal vein occlusion." American journal of ophthalmology 204 (2019): 62-69.
  12. Varner, Paul. "Redefining amaurosis fugax." African Vision and Eye Health 74.1 (2015): 5.
  13. Bacigalupi, Michael. "Amaurosis fugax-A clinical review." Internet Journal of Allied Health Sciences and Practice 4.2 (2006): 6.
  14. Sharma, Sumit, et al. "Pneumatic displacement of submacular hemorrhage with subretinal air and tissue plasminogen activator: initial United States experience." Ophthalmology Retina 2.3 (2018): 180-186.
  15. Schirmer, Clemens M., and Thomas R. Hedges. "Mechanisms of visual loss in papilledema." Neurosurgical focus 23.5 (2007): E5.
  16. Bansal, Svati, et al. "Controversies in the pathophysiology and management of hyphema." survey of ophthalmology 61.3 (2016): 297-308.
  17. Agrawal, Ashish, and Martin Andrew McKibbin. "Purtscher's and Purtscher-like retinopathies: a review." Survey of ophthalmology 51.2 (2006): 129-136.
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