OPTIC NEURITIS RETINAL DETACHMENT OCCIPITAL STROKE VITREOUS HAEMORRHAGE CENTRAL RETINAL ARTERY OCCLUSION CAUSE SUDDEN BLINDNESS

1. SUDDEN LOSS OF VISION
DR. AJAY DUDANI
MUMBAI RETINA CENTER
S V ROAD, SANTACRUZ (W)
DR. YASHESH MANIAR
MANIAR EYE AND PHYSIOTHERAPY
CLINIC, MAHAVIRNAGAR,KANDIVLI(W)
SHRIKRISHNA EYE CLINIC
SHRIKRISHNANAGAR, BORIVLI (E)

2. Visual loss is a common complaint among patients of
different ages with variable presentations. Some
patients describe it as a gray-black curtain that
gradually descends or as blurring, fogging, or dimming
of vision. It usually lasts a few minutes but can persist
for hours. Frequency varies from a single episode to
many episodes during a day; it may continue for years
but more often lasts from seconds to hours. Ischemia is
the most common mechanism of acute visual
dysfunction, and it can affect any aspect of the visual
system.
VISION LOSS

3. Multiple conditions are associated with transient visual
loss. They can be classified according to origin or
pathogenesis, but for the purpose of this article, they
are outlined by source.
CLASSIFICATION

4. Wray has classified TMVL into 3 groups based mostly
on pathogenesis; they include the following:
•Type 1 is characterized by loss of all or a portion of
vision in one eye, lasting seconds to minutes, with full
recovery. It is usually secondary to embolic
phenomenon. Attacks have been correlated with
vessels without critical narrowing but with ulceration.
•Type 2 includes visual loss due to hemodynamically
significant, occlusive, low-flow lesions in the ICAs or the
ophthalmic arteries. Symptoms are brief but frequent,
less rapid in onset, and longer in duration with gradual
recovery.
•Type 3 is believed to be due to vasoconstriction or
vasospasm.

5. The central retinal artery, a branch of the ophthalmic artery,
enters the eye through the optic disc and divides into multiple
branches to perfuse the inner layers of the retina. A branch
retinal artery occlusion (BRAO) occurs when one of these
branches of the arterial supply to the retina becomes occluded.
Branch Retinal Artery Occlusion

6. •In elderly patients, embolic disease is the most common
etiology of a BRAO. In a study of 70 patients with retinal
emboli, 40 were found to have cholesterol emboli, 8 platelet-
fibrin emboli, 6 calcific emboli, and 1 possible myxomatous
embolus. These types of emboli can also be iatrogenically
displaced during cardiac angiography, catheterization
procedures, or any interventional embolization of any branch
of the carotid artery.
Causes

7. •Types of emboli (endogenous and exogenous) include the
following:
•Cholesterol – Atheromatous plaques from the aorto-carotid
system
Platelet-fibrin – Carotid or cardiac thrombosis
Calcific - Calcified cardiac valves and atheromatous plaques of
the carotid artery
Leukoemboli - Vasculitis, Purtscher retinopathy, septic
endocarditis
Fat emboli - Following long bone fractures
Amniotic fluid emboli - Complication of pregnancy
Tumors - Atrial myxoma, mitral valve papillary fibroelastoma
Talc emboli - Long-term intravenous drug abusers

8. Corticosteroid emboli - Complication of intralesional or
retrobulbar steroid injection
Air emboli – Following trauma or surgery
Synthetic particles – From synthetic materials used in
artificial cardiac valves and other vascular procedures;
facial dermal filler (Restylane)

9. •Nonembolic causes of BRAO
•Thrombosis - Atherosclerosis, chemotherapeutic agents, bone
marrow transplants
Inflammatory conditions – Syphilis, toxoplasma, retinochoroiditis,
Behçet disease, Lyme disease, pseudotumor cerebri, Bartonella
infection, HIV infection, posterior scleritis, varicella-zoster
infection, multifocal retinitis with optic nerve edema, West Nile
virus infection
Vasospasm – Migraines, cocaine abuse, sildenafil citrate use
Coagulopathies - Sickle cell disease, Hodgkin disease,
pregnancy, anemia, platelet and clotting factor abnormalities,
protein C, protein S, antithrombin III, factor V Leiden deficiencies,
oral contraceptives, homocystinuria, antiphospholipid syndrome,
chelation therapy

10. Autothrombosis- From a ruptured arteriolar macroaneurysm
Compression - Preretinal arterial loops, vitrectomy surgery,
trauma
Idiopathic - A syndrome of recurrent episodes of multiple
BRAOs in otherwise healthy individuals has been described.
An association with Susac syndrome (microangiopathy of
brain, retina, and cochlea) has been seen in some of these
patients.

11. Lab Studies
•ESR
•Antitreponemal antibody, antiphospholipid
antibody, antinuclear antibody, rheumatoid factor, serum
protein electrophoresis, hemoglobin electrophoresis,
prothrombin time/activated partial thromboplastin time
(PT/aPTT), fibrinogen, protein C and S, antithrombin III,
and factor V Leiden.
•CBC to evaluate anemia, polycythemia, and platelet
disorders
•Fasting blood sugar, glycosylated hemoglobin,
cholesterol, triglycerides, and lipid panel to evaluate for
atherosclerotic disease
•Blood cultures to evaluate for bacterial endocarditis and
septic emboli

12. Imaging Studies
•Fluorescein angiography
•Optical coherence tomography (OCT)
•Electroretinogram (ERG)
•Serial Humphrey visual field testing
inferior branch retinal artery
occlusion from a platelet-fibrin
embolus. There is retinal
whitening surrounding the
occluded artery.

13. Optical coherence tomography
(OCT)

14. Medical Care
Considering the increased rate of mortality, patients with
BRAO should receive a full medical workup with special
attention to the cerebrovascular and cardiovascular
system. Depending on the findings, carotid endarterectomy
or anticoagulation may be indicated. Lab workup for
coagulopathies should also be performed if no embolic
source is found.

15. Further Outpatient Care
•Patients should initially be evaluated every 3-6 months to
monitor progression. Ocular neovascularization after BRAO
is rare. If neovascularization occurs, panretinal
photocoagulation should be performed.

16. Prognosis
•Recovery from BRAO is usually very good without
treatment; 80-90% of patients improve to a visual acuity
of 20/40 or better. However, some degree of visual field
deficit usually persists.

17. •The Eye Disease Case-Control Study reported the following
findings:
•Systemic hypertension is a risk factor for BRVO.
•Diabetes mellitus and open-angle glaucoma are not risk
factors for BRVO.
•Moderate alcohol consumption reduces the risk of BRVO.
•Patients often complain of a sudden painless decrease of
vision in the affected eye.
•Some may complain of a scotoma.
Branch retinal vein occlusion

18. Causes
•Most cases of BRVO are due to idiopathic factors. Usually,
patients have an anatomical predisposing factor, such as an
arteriovenous crossing where the artery compresses the vein.
This compression leads to clot formation and subsequent
BRVO.
•Inflammatory conditions that affect the retinal veins may
cause local damage that predisposes the individual to
intravascular clot formation with subsequent BRVO. Some of
the inflammatory conditions reported in the literature are the
following:
•Sarcoidosis
•Lyme disease
•Serpiginous choroiditis

19. •Thrombophilic conditions, such as the following, may
also be involved:
•Protein S deficiency
•Protein C deficiency
•Resistance to activated protein C (factor V Leiden)
•Antithrombin III deficiency
•Antiphospholipid antibody syndrome
•Lupus erythematosus
•Gammopathies

20. Lab Studies
•The authors of the Branch Vein Occlusion Study (BVOS) have
recommended against extensive testing in patients with typical
BRVO.
•Certain laboratory studies may be useful in atypical cases (ie,
bilateral cases, those in young patients, those in patients with
a personal or family history for thromboembolism).
Determinations of the following may be helpful:
•Prothrombin time (PT) and activated partial thromboplastin
time (aPTT)
•Protein C, protein S, factor V Leiden, and antithrombin III
•Homocysteine
•Antinuclear antibody (ANA), lupus anticoagulant, and
anticardiolipin

21. Imaging Studies
•Fluorescein angiography
A fluorescein angiogram is obtained as soon as the
hemorrhages have cleared if the patient's vision is still
depressed. The test is usually done 3 months after the event.

22. The purpose is to determine the cause of the visual loss
(eg, macular edema, macular ischemia). If the visual
loss is secondary to macular edema, laser
photocoagulation in a grid pattern may be of benefit.
Conversely, if macular ischemia is responsible for the
visual loss, laser photocoagulation should not be
offered.

23. Optical coherence tomography (OCT): Given its ability to
measure retinal thickness in a quantitative fashion, OCT
is a useful adjunct in the follow-up of patients with
macular edema secondary to BRVO.

24. Complications
•Macular edema
•Retinal neovascularization
•Vitreous hemorrhage
•Tractional retinal detachment
•Rubeosis iridis
•Epiretinal membrane

25. Prognosis
•An analysis of several series indicates that 53% of eyes
obtain 20/40 or better visual acuity, 25% have a visual
acuity between 20/50 and 20/100, and 22% have a visual
acuity of 20/200 or worse.
•The more distal the occlusion is from the optic disc, the
better the visual prognosis.

26. Central Retinal Artery
Occlusion
Background: In 1859, Van Graefe first described central
retinal artery occlusion (CRAO) as an embolic event to
the central retinal artery in a patient with endocarditis. In
1868, Mauthner suggested that spasmodic contractions
could lead to retinal artery occlusion. There is a multitude
of causes of CRAO, but patients typically present with
sudden, severe, and painless loss of vision.

27. History
•The most common presenting complaint is an acute,
persistent, painless loss of vision in the range of counting
fingers to light perception in 90% of patients. Consider
ophthalmic artery occlusion if visual acuity is worse.
•Some patients may reveal a history of amaurosis fugax
involving transient loss of vision lasting seconds to minutes but
which may last up to 2 hours. The vision usually returns to
baseline after an episode of amaurosis fugax.
•Ask about symptoms of temporal arteritis in the older
population. Patients complain of sudden, painless,
nonprogressive vision loss in one eye. History of headaches,
jaw claudication, scalp tenderness, proximal muscle and joint
aches, anorexia, weight loss, or fever may be elicited.

28. •Ask about any medical problems that could predispose
to embolus formation (eg, atrial fibrillation, endocarditis,
coagulopathies, atherosclerotic disease,
hypercoagulable state).
•Prolonged direct pressure to the globe during drug-
induced stupor or improper positioning during surgery
may lead to CRAO.
•Ask about drug history.

29. Physical
•Determine the degree of vision loss (eg, no light perception, hand
movement, counting fingers).
•Ocular examination includes the following:
•Check for afferent pupillary defect.
•Perform an optic nerve examination to look for signs of temporal
arteritis. Critical signs include afferent pupillary defect,
pale/swollen optic nerve with splinter hemorrhages.
•Cherry-red spot and a ground-glass retina may take hours to
develop.
•The funduscopic findings typically resolve within days to weeks of
the acute event, sometimes leaving a pale optic disc as the only
physical finding.
•Emboli can be seen in about 20% of patients with CRAO.

30. •Boxcar segmentation can be seen in both arteries and
veins. This is a sign of severe obstruction.
•Perform a cardiovascular examination for murmurs or
carotid bruits.
•Perform a systemic examination for temporal tenderness,
jaw claudication, muscle weakness, and fever to evaluate
for temporal arteritis.

31. Causes of CRAO vary depending on the age of the patient.
A detailed analysis of comorbid disease is necessary to
elucidate the cause of the acute visual loss.
•Systemic hypertension seen in two thirds of patients
•Diabetes mellitus
•Cardiac valvular disease seen in one fourth of patients
•Cardiac anomalies such as patent foramen ovale
Causes

32. •Embolism
•Most commonly cholesterol but can be calcific, bacterial,
or talc from intravenous drug abuse
•Associated with poorer visual acuity and higher morbidity
and mortality
•Emboli from the heart are the most common cause of
CRAO in patients younger than 40 years.
•Amaurosis fugax preceding persistent loss of vision
suggests branch retinal artery occlusion (BRAO) or
temporal arteritis and may represent emboli causing
temporary occlusion of the retinal artery.
•Coagulopathies from sickle cell anemia or
antiphospholipid antibodies are more common etiologies
for CRAO in patients younger than 30 years.

33. •Atherosclerotic changes
•Carotid atherosclerosis is seen in 45% of cases of
CRAO, with 60% or greater stenosis in 20% of cases.
•Atherosclerotic disease is the leading cause of CRAO in
patients aged 40-60 years.
•Giant cell arteritis
•Consider in patients older than 65 years, but do not
ignore in younger patients
•May produce CRAO or ischemic optic neuropathy
•Treat to preserve fellow eye

34. •Hypercoagulable state
•Collagen vascular disease
•Oral contraceptives
•Polycythemia
•Polyarteritis nodosa
•Rare causes
•Consider in younger patients
•Behçet disease
•Syphilis
•Sickle cell disease
•Migraine
•Hydrostatic arterial
occlusion
•Increased intraocular
pressure from glaucoma or
prolonged direct pressure to
the globe in unconscious
patients can precipitate
CRAO.

35. Lab Studies
•Laboratory studies are helpful in determining the etiology of
CRAO.
•CBC to evaluate anemia, polycythemia, and platelet
disorders
•Erythrocyte sedimentation rate (ESR) evaluation for giant
cell arteritis
•Fibrinogen, antiphospholipid antibodies, prothrombin
time/activated partial thromboplastin time (PT/aPTT), and
serum protein electrophoresis to evaluate for coagulopathies
•Fasting blood sugar, cholesterol, triglycerides, and lipid
panel to evaluate for atherosclerotic disease
•Blood cultures to evaluate for bacterial endocarditis and
septic emboli

36. Imaging Studies
Imaging studies are helpful in determining the etiology of
CRAO.
•Carotid ultrasound imaging to evaluate atherosclerotic
disease. This appears to be more sensitive than carotid
Doppler, which only determines the flow.
•Magnetic resonance angiogram may be more accurate in
detecting obstruction.
•Fluorescein angiogram
•Delay in arteriovenous transit time (<11 seconds is normal).
•Delay in retinal arterial filling
•Arterial narrowing with normal fluorescein transit after
recanalization

37. Early filling of cilioretinal
artery
Non-filling of other vessels Late staining of vessel
walls

38. Imaging techniques of carotid artery
Duplex scanning
• High resolution real-time B-scan ultrasonography
with Doppler flow analysis
• Injection of contrast medium
into superior vena cava
• Also images ischaemic
cerebral lesions
Magnetic resonance
angiography
• Images produced by computer-
assisted subtraction techniques
• Excellent visualization
• Potential morbidity
Digital intravenous
subtraction angiography
Intra-arterial angiography
• Seldom performed

39. Treatment options for carotid disease
Antiplatelet therapy
• Aspirin 75 mg daily
• Aspirin + dipyridamole (Persantin)
Anticoagulants - if antiplatelet therapy ineffective
• Clopidorel (Plavix) 75 mg daily
• Patients with other risk factors for stroke
• Symptomatic carotid stenosis > 70%
Carotid endarterectomy
Medical Surgical

40. Central Retinal Vein
Occlusion
Central retinal vein occlusion (CRVO) is a common retinal
vascular disorder. Clinically, CRVO presents with variable
visual loss; the fundus may show retinal hemorrhages,
dilated tortuous retinal veins, cotton-wool spots, macular
edema, and optic disc edema. In view of the devastating
complications associated with the severe form of CRVO, a
number of classifications were described in the literature. All
these classifications take into account the area of retinal
capillary nonperfusion and development of neovascular
complications.

41. 2 clinical types
ischemic and nonischemic
Nonischemic CRVO is the
milder form of the disease. It
may present with good vision,
few retinal hemorrhages and
cotton-wool spots, no relative
afferent pupillary defect, and
good perfusion to the retina.
Nonischemic CRVO may
resolve fully with good visual
outcome or may progress to the
ischemic type.

42. Ischemic CRVO is the severe form
of the disease. CRVO may present
initially as the ischemic type, or it
may progress from nonischemic.
Usually, ischemic CRVO presents
with severe visual loss, extensive
retinal hemorrhages and cotton-
wool spots, presence of relative
afferent pupillary defect, poor
perfusion to retina, and presence of
severe electroretinographic
changes. In addition, patients may
end up with neovascular glaucoma
and a painful blind eye.

43. hemorrhages extending all over
the fundus -same patient

44. Clinical features
Patients should undergo a complete eye examination,
including visual acuity, pupillary reactions, slit lamp
examination of the anterior and posterior segments,
undilated examination of the iris, gonioscopy, fundus
examination with indirect ophthalmoscope, and fundus
contact lens.

45. •Visual acuity: Best-corrected vision always should be
obtained. It is one of the important indicators of final visual
prognosis.
•Pupillary reactions may be normal and may present with
relative afferent pupillary reflex. If iris has abnormal blood
vessels, pupil may not react.
•Conjunctiva: Advanced stages may show congestion on
conjunctival and ciliary vessels.
•Cornea: Advanced stages may show diffuse corneal edema
obscuring the visibility of internal structures.
•Iris may be normal. Advanced stages may show
neovascularization. These vessels are detected best on an
undilated iris. Initially, vessels may be seen around pupillary
margins and peripheral iridectomy openings if present.

46. Fundus examination
•Retinal hemorrhages may present in all 4
quadrants.
•Hemorrhages can be superficial, dot and
blot, and/or deep.
•In some patients, hemorrhages may be
seen in peripheral fundus only.
•Hemorrhages can be mild to severe,
covering the whole fundus giving a "blood
and thunder appearance."
•Dilated tortuous veins: Veins may be
dilated and tortuous.

47. •Optic disc edema: Optic disc may be
swollen during early stage disease.
•Cotton-wool spots are more common
with nonischemic CRVO. Usually, they
are concentrated around the posterior
pole. Cotton-wool spots may resolve
in 2-4 months.

48. •Neovascularization of the disc
•Fine abnormal neovascularization on
the disc (NVD) or within 1 disc
diameter from the disc may be present.
•NVD indicates severe ischemia of the
retina.
•Sometimes NVD is difficult to
differentiate from optociliary shunt
vessels.
•NVD can lead to preretinal or vitreous
hemorrhage.

49. •Neovascularization elsewhere
•Neovascularization elsewhere
(NVE) is not as common as NVD.
•NVE indicates severe ischemia of
the retina.
•NVE can lead to preretinal or
vitreous hemorrhage.

50. •Optociliary shunt vessels are abnormal
blood vessels on the disc, directing blood
from retinal circulation to choroidal
circulation, which indicate good
compensatory circulation.
•Preretinal or vitreous hemorrhage
•Macular edema with or without exudates
•Cystoid macular edema
•Lamellar or full-thickness macular hole
•Optic atrophy
•Pigmentary changes in the macula

51. Causes
Central retinal vein obstruction has been associated with
various systemic pathological conditions, although the exact
cause and effect relationship has not been proven. Some of
the conditions in which CRVO has been associated include
the following:
•Systemic vascular disease
•Hypertension
•Diabetes mellitus
•Cardiovascular disease
•Blood dyscrasias
•Polycythemia vera

52. •Lymphoma
•Leukemia
•Clotting disorders
•Activated protein C resistance
•Lupus anticoagulant
•Anticardiolipin antibodies
•Protein C
•Protein S
•Antithrombin III
•Paraproteinemia and dysproteinemias
•Multiple myeloma

53. •Vasculitis
•Cryoglobulinemia
•Syphilis
•Sarcoidosis
•Autoimmune disease - Systemic lupus erythematosus
•Oral contraceptive use in women
•Other rare associations
•Closed-head trauma
•Optic disc drusen
•Arteriovenous malformations of retina

54. Optical coherence tomography
•Optical coherence tomography (OCT) scanning is a
noninvasive, noncontact, transpupillary imaging
technology that can image retinal structures in vivo with
a resolution of 10-17 µm. OCT quantitatively measures
the retina in micrometers in situ and in real time.
•OCT can detect even subtle macular edema in the
presence of significant hemorrhages, which is not
evident by fluorescein angiography because of
blockage from hemorrhage.
•OCT is useful in quantitatively monitoring the
development of macular edema and resolution with
treatment.

55. Fluorescein angiography
•most useful test for the evaluation of
retinal capillary nonperfusion,
posterior segment
neovascularization, and macular
edema.

57. Medical Care
No known effective medical treatment is available for
either prevention or the treatment of CRVO.
Identifying and treating any systemic medical
problems to reduce further complications is
important. Because the exact pathogenesis of the
CRVO is not known, various medical modalities of
treatment have been advocated by multiple authors
with varying success in preventing complications and
preserving vision.

58. TREATMENT
•Aspirin
•Anti-inflammatory agents
•Isovolumic hemodilution
•Plasmapheresis
•Systemic anticoagulation with warfarin, heparin, and
alteplase
•Fibrinolytic agents
•Systemic corticosteroids
•Local anticoagulation with intravitreal injection of
alteplase

59. Intravitreal injection of triamcinolone
•In patients with macular edema, injection of
triamcinolone (0.1 mL/ 4 mg) into the vitreous cavity
through pars plana has been shown to be effective not
only in resolving the edema but also in corresponding
improvement in vision.
•Even though the exact mechanism of action of
intravitreal injections of corticosteroids is not known, the
triamcinolone crystals in the vitreous cavity probably
reduce VEGF concentrations in the vitreous cavity. This
leads to a reduction in capillary permeability and macular
edema. The main drawback of an injection of
triamcinolone was posttreatment recurrences of macular
edema, requiring repeat triamcinolone injections,
typically every 3-6 months.

60. Laser photocoagulation is the
known treatment of choice in treatment of
various complications associated with
retinal vascular diseases (eg, diabetic
retinopathy, branch retinal vein
occlusion). Panretinal photocoagulation
(PRP) has been used in the treatment of
neovascular complications of CRVO for a
long time. However, no definite guidelines
exist regarding exact indication and timing
of PRP. A National Eye Institute
sponsored multicenter prospective study,
the Central Vein Occlusion Study
(CVOS), gave guidelines for treatment
and follow-up care of patients with CRVO.

61. •Chorioretinal venous anastomosis
•Radial optic neurotomy
•Vitrectomy

62. Retinal detachment (RD)
Separation of sensory retina from RPE by subretinal fluid (SRF)
Rhegmatogenous - caused by a
retinal break
Non-rhegmatogenous -
tractional or
exudative

63. •Papilledema/neoplasm causes visual loss by mechanically
compressing or physiologically destroying the optic nerve.
If confined to the orbits, vision is affected unilaterally with a
decline in central acuity, dyschromatopsia, and an afferent
pupillary defect in most instances. Visual field defects
involve central/paracentral and arcuate scotomas, nasal
steps, or temporal wedges. Funduscopy shows a swollen,
pale, or normal retina.
Papilloedema/neoplasm

64. Trauma

65. Hyphema

66. Ruptured globe
•A ruptured globe results from
a full-thickness traumatic
disruption of the sclera or the
cornea as a result of blunt or
penetrating trauma to the eye.
Open globe should be
suspected in any patient who
has a history of trauma to the
eye, especially with a
laceration or puncture wound
that extends through the
eyelid, followed by pain and
decreased visual acuity.

67. Anterior segment complications of blunt
trauma
Sphincter tear
Cataract Angle recession
Hyphaema
Lens subluxation
Iridodialysis Vossius ring
Rupture of globe

68. Macular hole Optic neuropathy
Equatorial tears
Posterior segment complications of blunt trauma
Choroidal rupture and
haemorrhage
Commotio retinae
Avulsion of vitreous base
and retinal dialysis

69. Complications of penetrating trauma
Flat anterior chamber
Vitreous haemorrhage
Damage to lens and iris
Endophthalmitis
Tractional retinal detachment
Uveal prolapse

70. Vitreous hammorhage
Eale’s disease

71. Optic Neuritis, Childhood
Optic neuritis implies an inflammatory process involving
the optic nerve. In children, most cases are due to an
immune-mediated process. These cases may be
associated with a viral or other infection or with
immunization

72. Optic neuritis may be the first manifestation of multiple
sclerosis (MS) or part of a more diffuse demyelinating
disorder, including acute disseminated encephalomyelitis or
Devic disease.
Optic neuritis may be related to specific infections, diseases
of the adjacent sinuses or orbital structures, and infectious
and infiltrative diseases of the brain or meninges that involve
the optic nerves.
Optic Neuritis

73. The following definitions aid in further understanding optic
neuritis:
Papillitis - Optic neuritis involving the optic disc with disc
edema
Retrobulbar optic neuritis - Optic neuritis involving the optic
nerve behind the globe. The optic disc appearance should be
normal in first-time episodes of retrobulbar optic neuritis.
Bilateral simultaneous optic neuritis - Optic neuritis in both
eyes occurring within 3 weeks of each other
Bilateral sequential optic neuritis - Optic neuritis occurring in
both optic nerves but separated by a period of more than 3
weeks

74. OPTIC NEUROPATHIES
1. Clinical features
2. Special investigations
5. Leber hereditary optic neuropathy
3. Optic neuritis
4. Anterior ischaemic optic neuropathy (AION)
• Retrobulbar neuritis
• Papillitis
• Neuroretinitis

75. Signs of optic nerve dysfunction
• Reduced visual acuity
• Diminished light
brightness sensitivity
• Dyschromatopsia
• Afferent pupillary
conduction defect

76. Applied anatomy of afferent conduction
defect
Anatomical pathway Signs
• Equal pupil size
• Light reaction
- ipsilateral direct is absent or
diminished
- consensual is normal
• Near reflex is normal in both eyes
• Total defect (no PL) = amaurotic
pupil
• Relative defect = Marcus Gunnpupil
3rd

77. Visual field defects
Central scotoma
Altitudinal Nerve fibre bundle
Centrocaecal scotoma

78. Optic disc changes
• Retrobulbar neuritis
• Early compression
Normal
• Papilloedema
• Papillitis and neuroretinitis
Swelling
• Optic nerve sheath meningioma
• Occasionally optic nerve glioma
Optico-ciliary shunts
• Postneuritic
• Compression
Atrophy
• AION
• Hereditary optic atrophies

79. Special investigations
Orbital fat-suppression techniques in
T1-weighted images
Assessment of electrical activity of
visual cortex created by retinal
stimulation
MRI Visually evoked potential

80. Classification of optic neuritis
Retrobulbar neuritis
(normal disc)
• Demyelination - most common
• Sinus-related (ethmoiditis)
• Lyme disease
Papillitis (hyperaemia and
oedema)
• Viral infections and immunization
in children (bilateral)
• Demyelination (uncommon)
• Syphilis
Neuroretinitis (papillitis
and macular star)
• Cat-scratch fever
• Lyme disease
• Syphilis

81. Non-arteritic AION
• Pale disc with diffuse or sectorial oedema
• Eventually bilateral in 30% (give aspirin)
• Age - 45-65 years
• Altitudinal field defect
Presentation
Acute signs
• Few, small splinter-shaped haemorrhages
• Resolution of oedema and haemorrhages
• Optic atrophy and variable visual loss
Late signs

82. FA in acute non-arteritic AION
Generalized hyperfluorescence
Increasing localized
hyperfluorescence
Localized hyperfluorescence

83. Superficial temporal arteritis
• Headache
• Age - 65-80 years
• Scalp tenderness
Presentation
• Superficial temporal arteritis
• Jaw claudication
• Polymyalgia rheumatica
• Temporal artery biopsy
• ESR - often > 60, but normal in 20%
• C-reactive protein - always raised
Special investigations
• Acute visual loss

84. Histology of giant cell arteritis
• High-magnification shows giant cells
• Granulomatous cell infiltration
• Disruption of internal elastic lamina
• Proliferation of intima
• Occlusion of lumen

85. Arteritic AION
• Affects about 25% of untreated patients with giant cell arteritis
• Severe acute visual loss
• Treatment - steroids to protect fellow eye
• Bilateral in 65% if untreated
• Pale disc with diffuse oedema
• Few, small splinter-shaped haemorrhages
• Subsequent optic atrophy

86. Leber hereditary optic neuropathy
Maternal mitochondrial DNA mutations
Signs
• Disc hyperaemia and dilated capillaries
(telangiectatic microangiopathy)
• Vascular tortuosity
• Swelling of peripapillary nerve fibre
layer
Presents
• Typically in males - third decade
• Occasionally in females - any age
• Initially unilateral visual loss
• Fellow eye involved within 2 months
• Bilateral optic atrophy
• Subsequent bilateral optic atrophy

87. Intraocular foreign bodies
•Intraocular foreign bodies are small particles that have
penetrated the cornea or the sclera. This condition
commonly occurs in the workplace; signs can be subtle,
causing only light erythema and local discomfort. Visual
acuity is often decreased markedly, but normal visual
acuity is possible and does not rule out an intraocular
foreign body. Smaller objects may produce few, if any,
signs or symptoms and may be difficult to discover
without a high index of suspicion. With large objects,
obvious disruption of the anterior segment, a visible
penetration site, hyphema, or cataract may be seen.

88. Imaging Studies:
•CT scans are the test of choice for IOFB localization. A
consultation with the CT technician is helpful in selecting the
optimal section so as to reduce the risk of a false-negative
result. Helical CT scans have a very high identification rate.
•Plain x-ray is useful if a metallic IOFB is present and a CT
scan is unavailable.
•MRI generally is not recommended for metallic IOFBs.
•Ultrasound is a useful tool in localizing IOFBs, and its
careful use is possible even if the globe is still open;
alternatively, intraoperative use after wound closure can be
attempted. The ultrasound biomicroscope may help with
IOFBs in the anterior segment.

89. IOFB CT SCAN

90. Endophthalmitis, Bacterial
Background: Bacterial endophthalmitis is an inflammatory
reaction of the intraocular fluids or tissues caused by
microbial organisms.

91. History
The clinical presentation is dependent on the route of entry,
the infecting organism, and the duration of the disease. In
general, patients complain of a decrease in vision, often with
a red eye. Most patients also may complain of a deep ocular
pain. Classification is based on routes of entry.
•Exogenous
•Endogenous

92. Exogenous
•Acute postoperative (<6 wk postoperative)
•Usually, infection occurs 2-10 days after surgery.
•Patients present with visual loss greater than expected in the
usual postoperative course.
•Ocular pain is seen in 75% of the patients.
•Use of postoperative antibiotic and anti-inflammatory drugs may
blunt the severity of the disease and possibly delay medical
attention.
•Delayed onset or chronic pseudophakic postoperative (>6 wk
postoperative)
•Typically, patients present with mild-to-moderate inflammatory
red eye, reduced vision, and photophobia.

93. •Chronic indolent course is present.
•Patients may be diagnosed with idiopathic uveitis and
treated with topical steroids with temporary
improvement.
•Rule out fungal species.
•Filtering bleb associated: Clinical features are similar
to acute postoperative infection with purulent bleb
involvement.
•Posttraumatic: History of trauma is present, and
infection usually progresses rapidly.

94. Endogenous
•No recent history of ocular surgery is present.
•Confusion with delayed onset or chronic postoperative is
possible if suspicion for endogenous route is not ruled out.
•Rarely bilateral.

95. Physical
General findings
•Visual acuity decreased below the level expected
•Lid edema
•Conjunctival hyperemia
•Corneal edema
•Anterior chamber cells and flare
•Keratic precipitates
•Hypopyon
•Fibrin membrane formation
•Vitritis
•Loss of red reflex
•Retinal periphlebitis if view of fundus possible

96. Specific findings
•Delayed onset or chronic: Occasionally, findings display a
white plaque within the equator of the remaining lens capsule.
•Filtering bleb associated: A purulent bleb is seen occasionally
with areas of necrosis in the sclera from the use of
antimetabolites.
•Posttraumatic: Evidence of penetrating trauma is seen with
the possibility of an intraocular foreign body.
•Endogenous: Patient may appear systemically ill.

97. Lab Studies
•Perform culture and sensitivity studies on aqueous and
vitreous samples to determine type of organism and
antibiotic sensitivity.
•If endogenous bacterial endophthalmitis is suspected, a
systemic workup for the source is required. This workup
includes the following:
•Blood culture
•Sputum culture
•Urine culture

98. Imaging Studies
•Perform ultrasound of the posterior pole if view of fundus is
poor.
•B-scan ultrasound
•Typically, choroidal thickening and ultrasound echoes in the
anterior and posterior vitreous support diagnosis.
•Occasionally, another source of inflammation other than or in
addition to bacterial such as retained lens material may be
seen.
•The ultrasound is also important to provide a baseline prior to
intraocular intervention and to assess the posterior vitreous
face and areas of possible traction.

99. •Rarely, a retinal detachment is seen concurrently with
endophthalmitis.
•A CT scan rarely is performed unless trauma is involved.
Thickening of the sclera and uveal tissues associated with
various degree of increased density in the vitreous and
periocular soft tissue structures may be seen.
•If an endogenous route is considered, perform other
imaging modalities to rule out potential sources.
•Two-dimensional echocardiogram
•Chest x-ray

100. Medical Care
Bacterial endophthalmitis is an ocular emergency and
urgent treatment is required to reduce the potential of
significant visual loss.
•All patients should have therapy consisting of intravitreal
and topical antibiotics, topical steroids, and cycloplegics.
•The Endophthalmitis Vitrectomy Study (EVS) identified
that the use of periocular and intravenous antibiotics are
not required in endophthalmitis following cataract surgery.
Medical therapy was found to be statistically as effective
as surgical intervention when the presenting vision was
hand motion or better. Use caution in interpreting the data
from the EVS; apply it cautiously to non–cataract-related
endophthalmitis.

101. •When the inflammation is severe, systemic and
periocular therapy may be used in non–cataract-
induced, delayed onset, filtering bleb–associated, and
posttraumatic endophthalmitis.
•In endogenous endophthalmitis, systemic, topical,
and possibly periocular therapy usually is required.

102. Surgical Care
Surgical intervention usually is performed urgently except in
the delayed onset category where elective surgery may
suffice.
•Indications for surgical therapy
•Acute pseudophakic postoperative - When the presenting
vision is light perception or worse
•Delayed onset or chronic postoperative - If marked
inflammation or a subcapsular plaque is identified, surgical
removal is required.

103. •Filtering bleb associated - If marked inflammation is
present. Take care not to disturb the bleb if some
function still exists. To allow the possibility of a shunt
valve to be placed at a later time, make an attempt to
minimize the disturbance to the superior conjunctiva.
If the patient is aphakic, performing the pars plana
vitrectomy from the temporal side using a limbal
approach may be required.
•Posttraumatic - If marked inflammation or rapid
onset occurs

104. Complications
•Retinal necrosis
•Retinal detachment
•Retinal necrosis
•Vitreous tap
•Vitrectomy
•Increased intraocular pressure
•Retinal vascular occlusion
•Optic neuropathy
•Panophthalmitis
•Hypotony
•Ciliary body shut down
•Leaking wound
•Retinal detachment
•Cyclodialysis cleft
•Medication

105. Miscellaneous
Hysteria/malingering
•Patients with hysterical blindness or loss of vision, despite
alleged loss of vision, are still capable of maneuvering in a
room. Pupillary reactions are normal. Loss of vision is a
subconscious conversion symptom. A purely functional loss of
vision can be assumed when the visual field is constricted
markedly, orientation when walking is intact, and pupillary
reactions to light are normal.
•A fluid transition exists between a hysterical and malingering
patient and aggravated loss of vision. If the patient indicates a
unilateral loss of vision, examination should be conducted in
such a way that the patient does not know which eye is being
tested or the actual size of the optotypes

106. Drugs
•Methyl alcohol poisoning
•Quinidine, sildenafil (Viagra), vardenafil (Levitra), and
tadalafil (Cialis)
•Sudden monocular visual loss due to nonarteric anterior
ischemic optic neuropathy (NAION) has been reported in
a small number of patients taking sildenafil (Viagra),
vardenafil (Levitra), and tadalafil (Cialis) for erectile
dysfunction. The US Food and Drug Administration (FDA)
advised health care professionals of the potential risk of
sudden visual loss that may be attributed to the use of
phosphodiesterase-5 (PDE-5) inhibitors.

107. •Idiopathic
•Migraine or scintillating scotoma may occur on a
persistent basis or may recur after an absence of
decades. Physiologic and anatomical bases have not
been fully explained but are believed to involve
vasospasm. Shimmering scotomas, with or without
color or perception of movement, are commonly
reported, usually lasting less than 30 minutes.