Visual Field Patterns in Neuro-

Ophthalmologic Cases
dr. Hafizhan
dr. Wina Siska Purnama
dr. Riski Prihatningtyas Sp.M(K)
 Visual Field Patterns in Optic
Neuropathy
• Clinically, patients with optic neuropathies present with visual acuity loss, visual
field loss, dyschromatopsia, and an RAPD (in patients with unilateral or asymmetric
damage).
• The optic disc may appear normal, atrophic, or swollen.
• Retinal ganglion cell nerve fibers enter the optic nerve head in 3 major groups

Lesions of the optic nerve result in 3 categories of visual field loss :

1. Papillomacular fibers: cecocentral scotoma paracentral scotoma and central scotoma

2. Arcuate fibers: arcuate scotoma (nerve fiber bundle defect), altitudinal defect
(broader region of arcuate fibers), and nasal (step) defect (temporal portion of arcuate
fibers). These fibers align along the temporal horizontal retinal raphe, so that damage
to them produces defects that do not cross and respect the nasal horizontal meridian.
3. Nasal radiating fibers: temporal wedge defect
Schematic representation of retinal nerve fiber layer entering the optic disc.
The fibers are classified as :
• Arcuate (inferior bundle highlighted)
• Papillomacular (long arrow)
• Nasal radiating (short arrow)
Humphrey perimetry grayscale diagram,
showing superior arcuate visual field
defect corresponding to the inferior
arcuate nerve fiber bundle damage
highlighted
Patterns of visual field loss in optic
neuropathies :

A. Cecocentral scotoma (left; arrow);

paracentral scotoma (right; arrow).

B. Central scotoma

C. Arcuate scotoma

D. Broad arcuate (altitudinal) defect

E. Nasal arcuate (step) defects

F. Enlarged blind spot

A, B, Optic discs in chronic
papilledema, with the
development of refractile
bodies (arrows) representing
lipid exudates from chronic
microvascular leakage.

C, D, Visual field patterns

confirm the presence of mild
diffuse depression in sensitivity
and superior and inferior
arcuate defects.
 Anterior Optic Neuropathies With
Optic Disc Edema
• Papilledema refers to edema of the optic nerve head that results from increased
intracranial pressure (ICP).
• Early papilledema begins at the superior and inferior poles -> encompasses the
nasal optic disc -> creating a c-shaped area of disc edema
• In acute papilledema, optic nerve function, including visual acuity, pupillary
response and color vision, is usually normal.
• Visual fields demonstrate only enlargement of the blind spot.
• With chronic papilledema, visual field defects may include nasal field loss, arcuate
scotomata, and generalized peripheral depression.
 Anterior Optic Neuropathies Without
Optic Disc Edema
Optic Disc Drusen

• Optic disc drusen (ODD)/ hyaline or colloid bodies -> represent refractile, often
calcified nodules located within the optic nerve head
• Often bilateral (75%–86%) but can be asymmetric.
• The pathophysiology of ODD is unclear.
• The drusen represent the product of deteriorating axons, which extrude their
contents into the interstitial space. Over time, the extruded material congeals and
calcifies.

Optic Disc Drusen

A. Fundus photograph -> blurred disc
margin with scalloped edge, refractile
bodies on the disc surface and at the
superior pole, mild pallor, and no
obscuration of retinal blood vessels.
B. Visual field patterns confirmed the
presence of a nasal step produced by
drusen involving the right disc.
C. B-scan ultrasonogram, demonstrating
focal, highly reflective (due to
calcification) elevation within the optic
disc (arrow),
D. Preinjection fundus photograph
demonstrating autofluorescence
(arrow).
E. CT scan of the orbits. Calcified optic
disc drusen are visible bilaterally at the
posterior globe– optic nerve junction
(arrows).

Optic Disc Drusen

 Anterior Optic Neuropathies Without
Optic Disc Edema
Leber Hereditary Optic Neuropathy

• The syndrome presents with acute, severe, painless, sequential vision loss (visual
acuity, <20/200) and central or cecocentral visual field impairment

• The classic fundus appearance triad includes :

1. Hyperemia and elevation of the optic disc, with thickening of the peripapillary retina;
although the disc appears swollen, it does not leak on fluorescein angiography
(“pseudoedema”)
2. Peripapillary telangiectasia
3. Tortuosity of the medium-sized retinal arterioles

Leber Hereditary Optic Neuropathy

A. Visual field testing demonstrated a central scotoma in the left eye. Two
months later, the patient lost vision in the right eye.

Leber Hereditary Optic Neuropathy

B. Fundus photographs demonstrate
hyperemic optic discs with blurred margins
and moderately tortuous vasculature. The
left optic disc shows mild temporal pallor.
C, There was no leakage on fluorescein
angiographic studies.

Leber Hereditary Optic Neuropathy

 Anterior Optic Neuropathies Without
Optic Disc Edema
Autosomal Dominant Optic Atrophy
• The most common hereditary optic neuropathy (estimated prevalence is 1:50,000)
• Genetic linkage studies have localized an ADOA gene (OPA1) to a region on chromosome 3 ->
mutations result in loss of mitochondrial membrane integrity and function, with subsequent
retinal ganglion cell degeneration and optic atrophy.
• Visual acuity loss is usually mild to moderate, in the range of 20/30–20/60, although it may
decline progressively.
• Visual field testing demonstrates central or cecocentral loss in most cases.

Autosomal Dominant Optic Atrophy

Autosomal dominant optic atrophy
findings in a 34-year-old man who
had been aware of difficulty reading
the blackboard in fourth grade, with a
mild, gradual decrease in visual acuity
bilaterally since then.

A, B. Optic discs show temporal

atrophy and papillomacular nerve fiber
layer dropout.
C, D, Visual field patterns demonstrate
bilateral cecocentral scotomata, which
extend superotemporally.

Autosomal Dominant Optic Atrophy

 Anterior Optic Neuropathies Without
Optic Disc Edema
Congenital Tilted Disc Syndrome
• The congenital syndrome produces an inferonasal colobomatous excavation of the nerve
tissue
• Congenital tilted disc syndrome is usually (80%) bilateral with thinning of adjacent RPE and
choroid.
• The visual field defects may mimic those of chiasmal compression but are differentiated by
their failure to respect the vertical midline and their partial improvement with myopic
refractive correction.

Congenital Tilted Disc Syndrome

Congenital tilted disc syndrome. Visual field testing results (A, B) show bilateral relative
superotemporal defects not respecting the vertical midline. Fundus photographs show bilateral
tilted discs OD (C) and OS (D).
Congenital Tilted Disc Syndrome
 Posterior Optic Neuropathies
Retrobulbar Optic Neuritis
• Presents as subacute monocular visual loss that develops over several days
• Periorbital pain, particularly with eye movement, occurs in 92% of cases and often precedes
vision loss.
• Perimetry testing most often shows generalized reduction of sensitivity (48%), and any
pattern of visual field loss may appear

Retrobulbar Optic Neuritis

A. Disc photograph -> normal
appearance.
B. Auttomated perimetry ->
central scotoma
C. T1-weighted axial MRI
scan -> enhancement of the
right intraorbital optic nerve
(arrow).
D. T2-weighted axial MRI
scan -> multiple white matter
hyperintensities (arrows)
consistent with
demyelination.

Retrobulbar Optic Neuritis

 Posterior Optic Neuropathies
Thyroid Eye Disease
• Present with progressive enlargement of extraocular muscles or orbital fat hypertrophy
• Progressive proptosis can stretch the optic nerve and cause dysfunction.
• The extraocular muscles can also enlarge to an extent that they compress the optic nerve at
the orbital apex

Thyroid Eye Disease

Thyroid eye disease in a 48-year-old man with a 6-month history of weakness, gradual swelling
around the eyes, and progressively decreased visual acuity over the prior month. Visual acuity
was 20/200 OD and 20/80 OS, with a mild right afferent pupillary defect.
A, B. Automated perimetry testing shows bilateral central and inferior visual field loss.
Thyroid Eye Disease
Axial (C) and coronal (D) CT scans show the optic nerve becoming encroached upon (arrow in C)
by enlarged extraocular muscles.

Thyroid Eye Disease

 Posterior Optic Neuropathies
Toxic or Nutritional Optic Neuropathy
• Optic neuropathy resulting from toxic exposure or nutritional deficiency
• Characterized by gradual, progressive, and painless vision loss that is bilateral and symmetric.
• Initial findings -> Subtle depression of central visual sensitivity
• Progressively more severe -> Central vision loss worsens, decrease in visual acuity and color
vision and a central scotoma
• Methanol and ethylene glycol toxicity result in a rapid onset of severe bilateral vision loss
with prominent disc edema.

Toxic or Nutritional Optic Neuropathy

Optic neuropathy caused by nutritional deficiency in a 42-year-old woman with a history of 4
bowel resections who presented with bilateral blurred vision and trouble recognizing colors.
Visual acuity was 20/70 OD and 20/200 OS, without an afferent pupillary defect.
A, B, Visual field results demonstrate a cecocentral scotoma on the left and a relative central
scotoma on the right.
Toxic or Nutritional Optic Neuropathy
C, D, Fundus appearance shows mild temporal optic atrophy OU, with papillomacular nerve
fiber layer dropout. After treatment with multivitamins and hydroxycobalamin injections, field
defects resolved completely and visual acuity returned to 20/20.

Toxic or Nutritional Optic Neuropathy

Toxic or Nutritional Optic Neuropathy
 Chiasmal Lesions
Extrinsic lesions affecting the chiasm

The most common lesions producing the chiasmal syndrome :

• Pituitary adenoma
• Parasellar meningioma
• Craniopharyngioma
• Parasellar internal carotid artery aneurysm
• Other CNS mass lesions

Extrinsic lesions affecting the chiasm

A, B, Visual field patterns from a patient with a pituitary tumor, showing bitemporal
depression worse superiorly, with margination along the vertical midline.
C, A T1-weighted coronal MRI scan shows an intrasellar enhancing mass, with extension
into the suprasellar cistern and upward displacement and compression of the chiasm
(arrow).

Extrinsic lesions affecting the chiasm

 Chiasmal Lesions
Intrinsic lesions affecting the chiasm

• These lesions include infectious (eg, tuberculosis, Lyme disease) and inflammatory
(eg, sarcoidosis, multiple sclerosis) causes of chiasmal neuritis
• Neoplasms can be either primary (eg, glioma) or secondary (eg, metastasis).
• Significant closed-head trauma can injure the chiasm, resulting in a bitemporal
hemianopia.
• Chiasmal injury may result from parasellar radiation therapy as well.

Intrinsic lesions affecting the chiasm

Chiasmal neuritis in a 36-year-old man with sudden-onset visual changes in both eyes.
A, Visual field testing showed a “junctional scotoma” with a central visual field defect in the left eye (left panel) and
temporal hemianopic defect in the right eye (right panel).
B, Coronal T1-weighted, postgadolinium MRI scan shows enhancement (arrow) of
predominantly the left side of the chiasm. C, Follow-up MRI scan shows resolution of the
enhancement shown in B.
Intrinsic lesions affecting the chiasm
D, Repeat visual field testing shows substantial improvement in both eyes.

Intrinsic lesions affecting the chiasm

 Retrochiasmal Lesions
Lateral Geniculate Body
• LGB is a highly organized and layered retinotopic structure
• Lesions in this region therefore can give highly localizing visual field defects

Lateral Geniculate Body

Visual field defects of the lateral geniculate body. Automated visual field testing shows (A) a
central wedge-shaped homonymous sectoranopia caused by lateral posterior choroidal
artery occlusion,

Lateral Geniculate Body

(B) a loss of the upper and lower homonymous quadrants, with preservation of the horizontal
wedge resulting from occlusion of the anterior choroidal artery.

Lateral Geniculate Body

Terima Kasih