OPHTHALMOLOGY PRESENTATION

1. DISC EDEMA
Dr .Sidesh Hendavitharana
{Registrar-ophthalmology}

2. What do you mean by disc
edema?
 Means swelling of the optic disc.
 It includes all causes of active or passive edematous swelling
of the optic disc other than the causes of papilledema

3. You can notice,
Disc hyperemia
Blurred disc margins
Nerve fiber layer swelling
Disc elevation
Tortuous vessels

4. Is it a true edema or not,
 Causes for pseudodisc edema,
1) Optic nerve head drusen
2) Myelinated nerve fibres
3) Morning glory syndrome
4) Tilted disc
5) Small hypermetropic disc
6) Optic disc hypoplasia
7) Bergmeister’s papilla

5. Optic disc drusen
 Composed of hyaline like calcific material within the substance of the optic
nerve head.
 Present in 0.3% of population
 Are often bilateral
 Associations include,
 Retinitis pigmentosa
 Angioid streak
 Alagille syndrome
 Complications-usually rare,
 include CNV,CRVO,CRAO
 Imaging,
 FA-shows progressive hyperfluorescence due to staining but absence of
leakage
 US-most reliable method because of its ability to detect calcific deposits that
show high acoustic reflectivity

6. .

7. Myelinated nerve fibres
 In normal eyes ,optic nerve myelination stops at the
cribriform plate.
 In eyes with myelinated nerve fibres the ganglion cells retain
a myelin sheath.
 Most often unilateral.
 Ocular associations include high myopia,anisometropia
and amblyopia.
 Systemic associations include NF1 and Gorlin syndrome

8. .

9. Morning glory syndrome
 Very rare usually unilateral sporadic condition.
 Signs,
 VA-may be normal or impaired to a variable extent.
 Large disc with funnel shaped excavation surrounded by an
annulus of chorioretinal disturbance.
 Blood vessels emerge from the rim of the excavation in a radial
pattern like the spokes of a wheel.
 Complications,
 Serous retinal detachment develops in about 30% of cases.
 CNV is less common and develop adjecent to the lesion

11. Systemic associations,
 Frontonasal dysplasia-mid facial anomalies consisting of
hypertelorism, flat nasal bridge and occasionally a mid line
notch in the upper lip and a midline cleft in the soft palate.
 Midline brain malformations- absent corpus callosum,
hypoplastic cerebellar vermis, small optic chiasm, malformed
occipital lobe, pituitary deficiency.
 NF2 -less common.
 PHASE syndrome-posterior fossa brain malformations,
large facial hemangiomas and cardiovascular anomalies.

12. .

13. Tilted disc
 Is common and usually bilateral anomaly caused by an oblique
entry of the optic nerve into the globe.
 Signs,
 Small oval or D shaped disc
 Disc margin is indistinct where the retinal nerve fibres are elevated.
 Associated finding includes inferonasal chorioretinal thining and
myopic astigmatic refractive error.
 Perimetry-
 superotemporal defects that do not respect vertical midline.
 Complications-
 uncommon, include CNV and sensory macular detachment.

15. Optic nerve hypoplasia
 Unilateral or bilateral
 Characterized by a diminished number of nerve fibres.
 Commonly involving the midline structures of the brain.
 Predisposition,
 Agents taken by mother during gestation.
 LSD, quinine,anticonvulsants,excess alcohol
 Superior segmental hypoplasia may be associated with
maternal diabetes.

16. presentation
 Severe bilateral cases present with blindness in early infancy.
 Less severe bilateral involvement may cause minor minor visual defects
or squint at any time in childhood.
 Unilateral cases usually present with squint.
Signs,
 VA may be normal or impaired to a variable degree, even to NPL.
 Double ring sign-small gray disc surrounded by a yellow halo of
hypopigmentation caused by concentric chorioretinal atrophy.
 Distance from the fovea to the temporal border of the optic disc often equals
or exceeds 3 times the disc diameter
 Retinal vessels are normal in caliber but tortuous

18. Other features
 Depends on the severity
 Includes astigmatism,field defect , dyschromatopsia , APD,
foveal hypoplasia, aniridia, microphthalmos, strabismus,
nystagmus
 Systemic associations,
 Wide variety of midline developmental brain defects-most
common De Morsier syndrome(septo-optic dysplasia)
 Characterized by absence of septum pellucidum agenesis of
corpus callosum, hypopituitarism
 Bilateral optic nerve hypoplasia may be a marker for
potential endocrine dysfunction.

19. Causes of true disc edema
Inflammatory-
papillitis, neuroretinitis, papillophlebitis and uveitis
Ocular hypotony
due to any cause
Vascular
CRVO, diabetic papillopathy , uremia, AION
Orbital causes
tumors,Graves orbitopathy, orbital cellulitis
Infiltrative conditions
leukaemias,lymphomas

20. papilloedema
 Is hydrostatic non inflammatory edema of optic disc due to
raised intracranial pressure.
 Is almost always bilateral although it may be asymetrical.
 Etiology-
 Intracranial causes,
 congenital-
 Aqueductal stenosis
 craniosynostosis

21.  Intracranial space occupying lesions
 Brain tumors-commonly tumors arising in posterior fossa-
cerebellum,midbrain,parieto-ocipital region which obstruct
aqueduct of sylvius.
 Abscess,tuberculoma,gumma,subdural
hematoma,aneurysms,hydrocephalus
 Intracranial infections
 meningitis,encephalitis
 IC h’age
 SAH,SDH

22.  Obstruction to CSF absorption by arachnoid villi
Direct blockage of villi by blood and protein
Cavernous sinus thrombosis
Damaged arachnoid villi
 Hypersecretion of CSF
 Tumors of spinal cord
 Idiopathic intracranial hypertension
pseudotumor cerebri,due to drugs like
tetracycline,vit.A,N.acid,contraceptives, corticosteroids

23. .
 Systemic conditions
Malignant hypertension
Pregnancy induced hypertension
Cardiopulmonary insufficiency
Blood dyscrasias
Nephritis
 Diffuse cerebral edema-due to blunt trauma to head.
 Metabolic disorders
Addison’s diseases,diabetic ketoaccidosis, hypoparathyroidism

24. Pathogenesis(hayreh’s theory)
Increased IC pressure,malignant hypertension and orbital
lesions
Increased in tissue pressure in retrolaminar region
Alteration in pressure gradient across lamina cribrosa
Stasis of axoplasm in prelaminar region of optic disc
Axonal swelling in prelaminar region
Extracellular edema
papilledema

25. Clinical features
 Usually bilateral(in case of raised ICP) but may be
unilateral(in case oforbital or ocular causes)
 General features,
 Features of raised ICP
 Headache,worsen on coughing,sneezing or straining
 Projectile vomiting(without nausea)
 Diplopia
 Focal neurological deficit with changes in level of
consciousness.

26. Optic neuritis
 Is an inflamatory,infective and demyelinating process
affecting the optic nerve.
 Types of ON,
1) Ophthalmoscopical classification
2) Aetiological classification

27. Triad of symptoms
 Visual loss
 sudden,progressive and profound visual loss varying in severity
 Eye pain
 dull eye pain aggravated by ocular movement,esp.upward and
downward movement
 Dyschromatopsia

28. Other symptoms
Impaired colour vision
Visual obscuration in bright light(typical feature)
Movement phosphenes and sound induced phosphenes
Uhthoff’s symptoms-episodic transient obscuration of vision
on exertion and increase in body temperature which recovers
at rest.
Pulfrich phenomenon-impaired depth perception for moving
objects

29. Signs of optic nerve
dysfunctions
 Decreased visual acuity and colour vision
 Diminished light brightness and contrast sensitivity
 Marcus Gunn pupil
 Visual field defects

30. pathogenesis
Inflammatory changes in the optic nerve
or/and in the sheath(perineuritis)
Infiltration by T lymphocytes and plasma
cells
Loss of myelin sheath
Degenerative changes and reactionary
gliosis

31. Ophthalmoscopical classification
1) Retrobulbar neuritis
 Normal optic nerve head
 Involve retrobulbar part of optic nerve
 Common in adults
2) Papillitis
 Hyperemia and edema of optic disc
 Associated with peripapillary flame-shaped haemorrhages usually unilateral.
 Common in children and young adults
3) Neuroretinitis
 Characterized by papillitis in association with inflammation of RNFL and
macular star figure
 Occur in children and more often bilateral
 Mostly infectious in etiology.

32. Aetiological classification
Demyelinating-most common
 Isolated
 Multiple sclerosis
 Neuromyelitis optica of Devic’s
 Diffuse paraxial encephalitis of schilder
Parainfectious
 may follow a viral infection or immunization
Infectious
o Local-endophthalmitis,orbital cellulitis, sinusitis, meningitis, retinochoroiditis
o systemic,
 Viral-influenza,measles,mumps,chickenpox,herpes zoster,EBV,CMV
 Bacterial-TB,syphilis,cat scratch disease,lyme disease
 Fungal-cryptococosis,histoplasmosis
 Protozoal-toxocara,toxoplasma,malaria,pneumocystic carinii
 Parasitic-cysticercosis

33. .
Non-infectious
 Sarcoidosis
 Immune mediated diseases
 Local
 Uveitis
 Sympathetic ophthalmitis
 Systemic
 SLE
 Polyarewtitis nodosa
 Wegener’s granulomatosis
 Metabolic
 Anemia
 Diabetes mellitis
 Pregnancy
 hyperthyroidism

34. Fundus picture
 Retrobulbar neuritis
Normal fundus
-site of involvement is behind the eyeball.
Temporal disc pallor
-due to involvement of papillomacular bundle
Slight distention of veins and attenuation of arteries(if lesion is
near lamina cribrosa)

35. papillitis
 Hyperemic disc
 Blurring of disk margins
 Disc oedema
 Congested and tortuous retinal veins
 Flame shaped haemorrhages and soft exudates on disc and
retina
 Cloudy vitrious with fine opacities
 Inflammatory cells in vitreous,obliteration of physiological cup
with organised fibrous tissue
 Perivascular sheathing

36. neuroretinitis
 Signs of papillitis
 Retinal oedema/haemorrhages
 Macular star formation

37. Multiple Sclerosis
 Disorder of Central Myelin (Oligodendroglia)
 Brain and Spinal Cord
 Immune Based
 Inflammatory demyelinating disorder
 Axonal injury (Disability)

38. Demyelination Axonal Loss
Inflammation
Multiple Sclerosis:
3 Components
Courtesy Dr. G. Rice

39. Presenting Symptoms of MS
Symptom Approximate Prevalence
Weakness in one or more
limbs
40-50%
Sensory loss/paresthesias 40-45%
Visual loss 16-36%
Gait disturbance/ataxia 5-15%
Diplopia 7-15%
Dizziness/vertigo 5%
Pain 3%
Sensory in face 3%

40. Neuro-ophthalmological Issues
Loss of Vision
(Monocular and Binocular)
Diplopia
Oscillopsia

41. MS and the Visual System
Afferent Visual System
 Vision loss and distortion
Efferent Visual System
 Diplopia and Oscillopsia

42. MS and The Afferent
Visual System
 Pre-chiasmal
Optic Nerve
 Chiasmal
Bitemporal VF defect rare
Junctional Scotoma defect not uncommon
 Post-Chiasmal
Optic tract
Geniculocalcarine pathway

43. Ocular Motility Disorders
 Saccadic abnormalities
 Hypometric
 Hypermetric
 Dysmetria
 Saccadic Intrusions
 Square wave jerks
 Saccadic pulses
 Ocular flutter

44. Ocular Motility Disorders
 Pursuit Dysfunction
 Saccadic Intrusions
 Internuclear Ophthalmoplegia
 MLF Lesion
 Skew Deviation
 Vertical diplopia
 Gaze Palsies
 Dorsal Midbrain Syndrome

45. Ocular Motility Disorders
 Internuclear Ophthalmoplegia:MLF Lesion
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc

46. Internuclear Ophthalmoplegia
 MRI Detection of MLF Lesions
 Proton density>T2>Flair

Proton
Density
T2 Flair

47. Internuclear Ophthalmoplegia
Versional Disconjugacy Index: Assess
adduction vs abduction saccade peak velocity
Most accurate method for identification of INO
is quantitative EOM recording
Clinical detection accuracy vs Recording
93% severe INO
75% moderate INO
29% mild INO
 Frohman et al. Neurology 2003;61:848-850

48. Ocular Motility Disorders
Vestibulo-ocular Dysfunction
 VOR Mismatch
 Failure of VOR Suppression

49. Vestibulo-Ocular reflex
From: Kline & Bajandas. Neuro-ophthalmology Board Review Manual; Slack Inc

50. Dynamic Visual Acuity Test*
 Read eye chart with eyes open and with slow head shake
 Abnormal:
>3 line drop in acuity
* VOR test

51. Nystagmus
Gaze evoked
Direction changing
cerebellar
Direction selective
 vestibular
Ataxic of INO
Vertical (Upbeat or
downbeat)
Rebound
Torsional
Acquired pendular
Periodic alternating
Lid nystagmus
Superior oblique
myokymia*
Interesting but rarely localizing
”

52. Ocular Motility Disorders
 Congenital strabismus
 Latent nystagmus
 DVD (Dissociated Vertical Divergence)
 Convergence spasm
 Voluntary nystagmus
 Congenital or chronic IVth (FAT scan)
 Duane’s Retraction Syndrome

53. Neuromyelitis optica (NMO)
Acute/subacute demyelination, necrosis of optic
nerves, spinal cord
Often preceded by viral illness, associated with
systemic autoimmune disease
Partial responses to steroids, other
immunosuppressants

54. Key clinical features
Optic neuritis
Acute/subacute
neuropathic visual loss
Typically painful
Mild, if any, disc edema
Myelitis
Acute/subacute weakness,
numbness
Bowel/bladder problems
L’hermitte’s sign

55. The broadening spectrum of NMO
‘Textbook’ form’
 Monophasic
 Simultaneous ON and SC
disease
 Bilateral ON involvement
 No disease outside SC, ONs
 No brain MRI lesions
Current description
 >70% recurrent
 ON and SC attacks may be years
apart
 ON disease may be unilateral
 Brain disease occurs (ca. 10%)
 Brain MRI changes may
occasionally resemble multiple
sclerosis
ON – optic nerve
SC – spinal cord

56. NMO – disease or syndrome?
Differential diagnosis
a) Multiple sclerosis
b) Acute disseminated encephalomyelitis (ADEM)
c) Lupus
d) Sjogren’s syndrome
e) Parainfectious

57. How does NMO compare with MS?
Similarities
- Female predilection
- Age of onset
- Relapse rate
Differences
- Geography
- Brain symptoms
- Prognosis
- MRI appearance
- Cerebrospinal fluid
findings
- Response to treatment

58. Ancillary tests in NMO
MRI
Elongated, expansile, enhancing spinal cord lesions
Brain MRI usually normal; occasional multiple-sclerosis-like
plaques or confluent/symmetrical lesions
CSF
>50 white blood cells/mm3 or >5 polymorphonuclear
leucocytes/mm3 common
Oligoclonal bands, ↑IgG synthesis less common

59. ‘The NMO antibody’
IgG autoantibody localizes to glia at blood-brain-barrier
Binds to aquaporin-4, the main water channel in the
central nervous system
About 90% specific, 75% sensitive for NMO
Often + in brain MRI- negative relapsing myelitis/optic
neuritis
Available commercially

60. The epidemiology of MS and NMO differs
in Japan and the West
 Lower prevalence of MS in Japan
 Higher ratio of classic, monophasic
NMO to MS, likely true throughout Asia
 More Japanese ‘MS’ patients present with
bilateral optic neuropathy and severe ON
or SC disease (ca. 25%)
 Up to 60% of ‘Asian optospinal’ MS may
be + for NMO-IgG, implying that this
condition represents recurrent NMO

61. Laotian woman
Age 47-52: 4 bouts
unilateral optic
neuritis
Age 54: transverse
myelitis
Exam: no light
perception OD,
20/20 OS, spastic
paraparesis
NMO-IgG positive

62. NMO: the latest criteria
• History of optic neuritis
• History of acute myelitis
• Two of three of:
 MRI spinal cord lesion > 3 segments
 + NMO-IgG antibody
 Brain MRI not consistent with multiple sclerosis

63. Who will relapse?
Older patients with more common, sequential optic
neuritis/myelopathic disease
Less severe disease at onset
High-titer + NMO-IgG antibodies
Step-wise progression portends worse prognosis than
monophasic disease

64. Treatment for NMO*
Relapses/acute disease
IV methylprednisolone 1000 mg/day, 3-5 days
Plasmapheresis
Prevention / stabilization
Consensus: ABCR drugs not helpful
Azathioprine 2.5-3 mg/kg/day
Concurrent prednisone 1 mg/kg/day, tapering slowly
after azathioprine takes effect
Mycophenolate mofetil, Mitoxantrone, Rituximab, IVIg,
Plasmapheresis possible second liners
* No class I or II data

65. Optic Neuritis Clinical Treatment
Trial
 Initiated in 1988, enrolled 457
patients, utilizing 15 clinical centers
throughout U.S.
 Patients randomized to one of three
regiments
A) Oral Prednisone (1mg/kg/day for
14 days
B) IV Methylprednisolone (250mg
every 6 hours for 3 days,
followed by Oral Prednisone 1
mg/kg for 11 days
C) Oral Placebo for 14 days
 Eligible Patients
a) 18 to 46 years of age
b) Acute unilateral optic neuritis with
visual symptoms 8 days or less
c) +RAPD and Field Defect in
affected eye
d) No previous episodes of Optic
Neuritis in affected eye
e) No previous corticosteroid
treatment for optic neuritis or M.S.
f) No systemic dx other than M.S.
that could cause Optic Neuritis

66. Key Findings of ONTT
1. Corticosteroid Therapy for Optic Neuritis
has no long term beneficial effect on vision
2. Methylprednisolone regiment sped
recovery by 1-2 weeks
3. Patients receiving Oral Prednisone
regiment didn’t experience any benefit, in
fact there was a recurrence rate double of
the other groups
4. Patients with MRI scans demonstrating 2
or more white matter lesions, treated with
IV steroids experience a 2 year protective
effect (36% untreated vs 16%) that
disappeared after year 3

67. Key Findings of ONTT
 Periventricular white matter lesions demonstrating
demyelination most critical for assessing risk of developing
M.S.
 Zero Lesions: 25% chance of developing M.S. within 5 years
 One Lesion or more: 72% chance of developing M.S. in 15 year
period
 Lower risk of developing M.S. associated:
a) male sex
b) optic disc swelling
c) atypical features of optic neuritis (absence of pain, NLP
vision, peripapillary hemorrhages, retinal exudates)

68. Literature Review
Control All M.S. Eyes M.S. without
Optic Neuritis
M.S. with hx
of Optic
Neuritis
Low Contrast
(2.5%) # of
letters correct
25 +/- 7 (61
eyes)
16 +/- 10 (239
eyes)
18 +/- 10 (150
eyes)
11 +/- 11 (87
eyes)
Spectralis
Domain OCT
(peripapillary
RNFL
thickness μm)
92.9 +/- 10
(61 eyes)
84.3 +/- 12.8
(239 eyes)
87.6 +/-11.1
(150 eyes)
78.4 +/- 13.6
(87 eyes)
• Study found significant relationship between well validated
National Eye Institute: Vision Related Quality of Life Survey
Score and the following:
• Low Contrast Visual Acuity Score
• RNFL
• Clinical Trials utilizing OCT to evaluate the effectiveness of
immunomodulatory therapy

69. THANK YOU