Optic nerve

Applied anatomy:
The optic nerve carries about 1.2 million afferent nerve fibers, which represent
the axons of retinal ganglion cells. Most of these (90%) synapse in the lateral
geniculate body "LGB" (carrying visual stimulation), and the rest (10%) reach
other centers, notably the pre-tectal nuclei in the mid brain carrying light pupillary
reflex. The afferent pathway for light pupillary reflex is the optic nerve while the
efferent pathway is the oculomotor nerve.
The optic nerve is approximately 5 cm long from globe to optic chiasm, where
decussation occurs, the temporal fibers for each nerve pass to ipsilateral optic
tract, while nasal fiber cross to contralateral optic tract (crossing fibers).
Any injury to the axons of ganglion cells before LGB causes optic disc atrophy
seen by fundoscopy (ipsilateral if pre-chiasmal and bilateral if post-chiasmal),
while the injury that occurs after LGB (optic radiation and occipital cortex), will
not cause optic disc atrophy.
Visual center lies mainly on the medial surface of the occipital cortex (Broadman's
area no. 17).

Lesion from above --> craniopharyngioma.

Lesion from below --> Pituitary tumor.

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Optic nerve can be subdivided into four segments:
1- Intra-ocular segment (optic disc, nerve head): about 1mm depth & 1.5mm
in diameter.
2- Intra-orbital segment: 3.0 cm, this part has S shape allowing the eye for
movement without nerve stretching (i.e. the distance from the apex of orbit to
the posterior part of eyeball is less than 3.0 cm).
3- Intra-canalicular segment: 1cm.
4- Intra-cranial segment: 6-8 mm, joins the chiasm.
* The optic nerve is surrounded by pia, arachnoid and dura mater, so the CSF
reaches up to the posterior sclera around the optic nerve.

Axoplasmic transport:
It is the movement of cytoplasmic organelles within a neuron between the cell
body and the terminal synapse of Ganglion cells.
Retinal cotton-wool spots are the result of accumulation of cytoplasmic
organelles due to interruption of axoplasmic flow between the retinal ganglion
cells and their terminal synapses.
Papilloedema is similarly caused by hold-up of axoplasmic flow at the lamina
cribrosa (small pores present at posterior sclera for exit of optic nerve fibers).

Signs of optic nerve dysfunction:

1- Decreased visual acuity.
2- Diminish light pupillary reflex (afferent pupillary defect).
3- Dyschromatopsia (impairment of color vision): affected eye sees the colors
less bright.
4- Diminished light brightness sensitivity.
5- Visual field defect: depends on the type of the pathology, e.g. central
scotomas, centrocaecal scotomas and altitudinal.

Special investigations:
1- Manual kinetic Perimetry (Goldmann) for assessment of peripheral VF.
2- Automated Perimetry for assessment of peripheral and central VF.
3- MRI: detect tumors, degenerative or inflammatory diseases like multiple
sclerosis.
4- Visual Evoked Potential (VEP): is a recording of the electrical activity of the
visual cortex by stimulation of the retina (diagnose any damage from ganglion
cell to occipital cortex), while for diseases from receptors to ganglion cell we
use ERG (Electro-Retinography).
5- Fluorescein angiography: to differentiate between optic nerve diseases and
papilloedema, e.g. optic disc drusen and papilloedema, as drusen do not leak
fluorescein dye while papilloedema leak the dye.

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 Papilledema
It is swelling of the optic nerve head secondary to raised intracranial pressure.
It is nearly always bilateral, although it may be asymmetrical.
All other causes of disc edema in the absence of raised ICP are referred to as
"disc swelling" and usually produce visual impairment.
All patients with bilateral discs swelling should be suspected of having an
intracranial mass until proved otherwise. However, not all patients with raised ICP
(intra cranial pressure) have necessarily developed papilledema.

Pathogenesis
It is caused by impairment of axoplastic transport as a result of increased
intracranial pressure and vascular congestion of central retinal vein.

Symptoms
The patient usually complains from headache especially in early morning. It
may be associated with vomiting which may be projectile. Other symptoms may
be diplopia or focal neurological signs depending on the cause.
Vision is usually not affected in early stages but the patient may complain from
transient obscuration of vision lasting for seconds. Optic nerve functions are
normal in early stages. As the patient develops optic atrophy visual acuity drops
and signs of optic nerve dysfunction appear.

Stages of papilloedema

1- Early features of papilledema:

- Visual symptoms are absent and visual acuity is normal.
- Optic disc shows hyperemia and mild elevation.
2- Established papilledema:
- Transient visual obscurations lasting a few seconds.
- Visual acuity is normal or reduced.
- Optic disc shows severe hyperemia, moderate elevation and indistinct margin.
3- Atrophic papilledema:
- Visual acuity is severely impaired.
- Optic discs are dirty grey color, slightly elevated and indistinct margin.

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Other differential diagnosis of bilateral discs swelling:
1- Malignant hypertension – always check the blood pressure in all cases of
bilateral disc swelling
2- Bilateral simultaneous Papillitis.
3- Bilateral compressive thyroid ophthalmopathy.
4- Bilateral simultaneous AION.
5- Bilateral compromised venous drainage in central retinal vein occlusion or
carotid-cavernous fistula.

Optic neuritis
It is an inflammatory or demyelinating process affecting the optic nerve.
1- Ophthalmoscopic classification:
a- Retrobulbar neuritis: in which the optic disc appearance is normal, at least
initially, because the optic nerve head is not involved. It is the most frequent
type in adult and is frequently associated with multiple sclerosis.
b- Papillitis: in which the pathological process affects the optic nerve head. It is
characterized by variable hyperemia and edema of the optic disc, which may
be associated with parapapillary flame-shaped hemorrhages. Papillitis is the
most common type of optic neuritis in children, although can also affect
adults.

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2- Etiological classification:
a- Demyelinating: which is by far most common cause usually young females
with Multiple Sclerosis (MS).
b- Para-infectious: it is follow a viral infection or immunization.
c- Infectious: which may be sinus-related or associated with syphilis, lyme
disease, cat-scratch fever or Herpes zoster.
d- Autoimmune: may be associated with systemic autoimmune disease.

Clinical features
 Reduced visual acuity – whereas in papilloedema VA is not affected until
late in the disease
 Ocular pain increased with eye movement
 Relative afferent pupillary defect RAPD or Marcus Gunn pupil
 Visual field defect – central scotoma, centrocecal scotoma or altitudinal
field defect ..etc.
 Dyschromatopsia - red colour desaturation is noted
 Optic disc appearance by fundoscopy (see above)

Investigation
 VEP shows increased latency of signal in optic neuritis
 MRI may show demyelinating plaques in multiple sclerosis

Differential diagnosis
 Papilloedema
 Anterior ischemic optic neuropathy
 Compressive optic neuropathy
 Toxic optic neuropathy
 Malignant hypertension

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Treatment of optic neuritis:
It is according to the etiology. If the cause is demyelination in MS, the patient may
need IV methylprednisolone, then oral prednisolone.

Optic atrophy
It is an important sign of advanced optic nerve disease. It is of two types:
1- Primary optic atrophy:
It is occurs without antecedent swelling of the optic nerve head. It may be
caused by lesions affecting the visual pathways from the retro laminar (behind
lamina cribrosa) portion of the optic nerve to the lateral geniculate body. Lesions
anterior to the optic chiasm result in unilateral optic disc atrophy, whereas those
involving the chiasm and optic tract will cause bilateral optic disc atrophy.

Causes:
- Retro bulbar neuritis (but not Papillitis, as it is preceded by disc swelling)
- Compressive lesions, such as tumors and aneurysms.
- Hereditary optic neuropathies.
- Toxic and nutritional optic neuropathies.
Signs:
- Pale, flat disc with clearly delineated margin.
- Reduction in number of small blood vessels on the disc surface.

2- Secondary optic atrophy:

It is preceded by swelling of the optic nerve head.
Causes:
- Papillitis.
- Chronic papilledema.
- AION (Anterior Ischemic Optic Neuropathy): usually occurs in old age
patients, it is of two types; non arteritic [in diabetes, hypertension] and arteritic
.e.g. Giant cell arteritis.

Signs:
- White or dirty grey, slightly raised disc with poorly delineated margins due to
gliosis.
- Reduction in number of small blood vessels on the disc surface.

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 Primary optic atrophy Secondary optic atrophy

Abnormal pupillary reaction

Applied anatomy

LIGHT REFLEX

The pupillary light reflex consists of four neurons.

1. The first connects the retina with the pre-tectal nucleus in the mid-brain at the
level of the superior colliculus. The reflex is mediated by the retinal
photoreceptors. Impulses originating from the nasal retina are conducted by
fibers which decussate in the chiasm and pass up the optic tract to terminate in
the contralateral pre tectal nucleus. Impulses originating in the temporal retina
are conducted by uncrossed fibers which terminate in the ipsilateral pre tectal
nucleus.
2. The second connects the pre tectal nucleus to both Edinger-Westphal nuclei by
internuncial fibers. This is why a unilateral light stimulus evokes a bilateral
and symmetrical pupillary constriction. Damage to these internuncial neurons
is responsible for light-near dissociation in neurosyphilis and pinealomas.
3. The third connects the Edinger-Westphal nucleus to the ciliary ganglion inside
the orbit. In the orbit, these parasympathetic fibers pass in the inferior division
of the third cranial nerve and reach the ciliary ganglion via the nerve to the
inferior oblique muscle.
4. The fourth leaves the ciliary ganglion and passes with the short ciliary nerves
to innervate the sphincter pupillae. The ciliary ganglion is located within the
muscle cone, just behind the globe. It should be noted that, although the ciliary
ganglion contains other nerve fibers (sensory and sympathetic), only the
parasympathetic fibers synapse there.

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NEAR REFLEX

The near reflex triad consists of: (1) increased accommodation, (2) convergence of
the visual axes and (3) constriction of the pupils. The term 'light-near dissociation'
refers to a condition in which the light reflex is absent or abnormal, although the near
response is intact. Vision is not a prerequisite for the near reflex, and there is no
clinical condition in which the light reflex is present but the near response absent.
Although the final pathways for the near and light reflexes are the same (i.e. third
nerve, ciliary ganglion, short ciliary nerves), the center for the near reflex is ill-
defined. There are probably two supra nuclear influences: the frontal and occipital
lobes. The mid-brain center for the near reflex is probably located in a more ventral
location than the light reflex (in pre-tectal nucleus) and this may be one of the reasons
why compressive lesions such as pinealomas preferentially involve the dorsal
pupillomotor fibers, sparing the ventral fibers until late.

SYMPATHETIC SUPPLY

The sympathetic supply consists of three neurons:

1. The first starts in the posterior hypothalamus and descends, uncrossed, down
the brain stem to terminate in the ciliospinal center of Budge located between
C8 and T2.
2. The second passes from the ciliospinal center of Budge to the superior cervical
ganglion in the neck. During its long course, it is closely related to the apical
pleura where it may be damaged by bronchial carcinoma (Pancoast's tumor) or
during surgery on the neck.

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 3. The third ascends along the internal carotid artery to enter the skull, where it
joins the ophthalmic division of the trigeminal nerve. The sympathetic fibers
are also passing through ciliary ganglia but without relay and it are reaching
the ciliary body and the dilator pupillae muscle via the nasociliary nerve and
the long ciliary nerves.

Afferent pupillary conduction defects

A total afferent pupillary defect (TAPD, Amaurotic pupil) is caused by a complete

optic nerve lesion and is characterized by the following:

1. The involved eye is completely blind (i.e. no light perception).

2. Both pupils are equal.
3. When the affected eye is stimulated neither pupil reacts but when the normal
eye is stimulated both pupils react normally.
4. The near reflex is normal in both eyes.

A relative afferent pupillary defect (RAPD, Marcus Gunn pupil) is caused by an

incomplete optic nerve lesion or severe retinal disease, but not by a dense cataract or
vitreous hemorrhage. The clinical features are those of an Amaurotic pupil but more
subtle. The difference between the pupillary reactions is enhanced by the 'swinging-
flashlight test' in which each pupil is stimulated in rapid succession. When the
abnormal pupil is stimulated it dilates instead of constricting. This paradoxical
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reaction of the pupil to light occurs because the dilatation of the pupil, by
withdrawing the light from the normal eye, outweighs the constriction produced by
stimulating the abnormal eye.

Examples of diseases that cause relative afferent pupillary defect

1. Optic neuritis
2. Ischemic optic neuropathy
3. Traumatic optic neuropathy
4. Central retinal artery occlusion
5. Central retinal vein occlusion
6. Total retinal detachment

Right relative afferent pupillary defect. OD: right, OS: left.

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