The Optic Nerve

Optic Nerve Head Edema Often Reflects Increased Intracranial Pressure

Optic nerve head (optic disc) edema refers to a swelling of the optic nerve head where it
enters the globe. Optic nerve head edema can result from various causes, the most important
of which is increased intracranial pressure. The term papilledema, which is still widely used
in that context, is imprecise because no optic papilla exists.

Other important causes of optic nerve head edema are (1) obstruction to the venous drainage
of the eye (as may occur with compressive lesions of the orbit), (2) infarction of the optic
nerve (ischemic optic neuropathy), (3) inflammation of the optic nerve close to the eyeball
(optic neuritis, papillitis), and (4) multiple sclerosis.

Edema of the optic nerve head is characterized clinically by a swollen optic disc that displays
blurred margins and dilated vessels (Fig. 29-15). Frequently, hemorrhages (Fig. 29-16),
exudates, and cotton-wool spots are seen, and concentric folds of the choroid and retina may
surround the nerve head.
Acutely, optic nerve head edema results in few if any visual symptoms. As the condition
becomes established, swelling of the optic nerve head enlarges the normal blind spot. After
many months, atrophic changes lead to a loss of visual acuity.

Rubbin pathology

FIGURE 29-15. Chronic papilledema. The optic nerve head is congested and protrudes
anteriorly toward the interior of the eye. It has blurred margins, and the vessels within it are
poorly seen. In contrast to acute papilledema, the veins are not so congested, and hemorrhage
is not a feature.




FIGURE 29-16. Hemorrhage in papilledema. The optic nerve head is markedly congested,
with dilated veins and a blurred margin. A small hemorrhage is evident within the optic nerve
head at its junction with the retina. Several small cotton-wool spots are present within
the adjacent retina.



Figure 11-26 The left ocular fundus as seen with an ophthalmoscope.
An increase in CSF pressure slows venous return from the retina, causing edema of the retina (fluid
accumulation). The edema is viewed during ophthalmoscopy as swelling of the optic disc, a condition
called papilledema. Normally, the disc is flat and does not form a papilla. Papilledema results from
increased intracranial pressure and increased CSF pressure in the extension of the subarachnoid
space around the optic nerve (Fig. 7.50A).

Moore

Etiologi



The swollen disc (Figure 14.2 ) is an important and often worrying sign.
Papilloedema is the term given to disc swelling associated with raised
intracranial pressure (ICP), accelerated hypertension and optic disc ischaemia.
Papillitis , a condition with a similar fundoscopic appearance, is due to optic
neuritis (infl ammation) affecting the nerve head. Visual loss always occurs with
optic neuritis but is uncommon with the papilloedema of hypertension
and/or raised intracranial pressure; it is, however, a feature of ischaemic
papilloedema.



The differential diagnosis of disc swelling is shown in Table 14.1 . Some
normal optic nerve heads may appear to be swollen, due a crowding of nerve
fi bres entering the disc. This is termed pseudopapilloedema and occurs
particularly in small, hypermetropic eyes where the nerve entry site is reduced
in size. Note also that myelinated nerve fi bres occurring on the nerve head may
be mistaken for optic disc swelling. During development, myelination of the
optic nerve begins proximally and spreads centrifugally to be completed at the
lamina cribrosa at term. Occasionally, as a developmental variant, the process
extends into the peripapillary retinal nerve fi bre layer as a patch of light -
scattering, myelinated fi bres; the normally unmyelinated retinal nerve fi bre
layer is partly myelinated, giving it a feathery, white appearance which refl ects
the organization of the nerve fi bre layer.

In myopia it is common to see a pale
myopic crescent of peripapillary atrophy, at the temporal margin of the optic
disc. In high myopia the optic disc may be surrounded by such an atrophic area
( peripapillary atrophy ), which may be confused with disc swelling.


History
The crucial feature of disc swelling due to raised intracranial pressure is that
there is rarely an associated visual loss, although some patients, with advanced
papilloedema, may develop fl eeting visual loss lasting seconds when they alter
posture from lying to standing ( obscurations of vision).
Other important clues to the presence of raised intracranial pressure include:
headache, worse on waking and made worse by coughing;
nausea, retching;
diplopia (double vision), usually due to a sixth nerve palsy;
other neurological signs, if the raised pressure is due to a cranial space -
occupying lesion visual fi eld loss; cranial nerve palsy;
a history of head trauma suggesting a subdural haemorrhage;
a history of medications sometimes associated with raised intracranial pressure
(e.g. oral contraceptives, tetracyclines).



Signs
The optic disc is swollen, its edges are blurred and the superfi cial capillaries
are dilated and thus abnormally prominent. There is no spontaneous venous
pulsation of the central retinal vein. This has a physiological basis. The central
retinal vein is exposed to cerebrospinal fl uid (CSF) as it leaves the optic nerve
to join the veins of the orbit. Normally, venous pressure in the retinal veins
at the nerve head is just above ocular pressure. Venous pulsation occurs
because the vein collapses briefl y with each rise in ocular pressure with arte-
rial infl ow. When the CSF pressure is higher than the ocular pressure, as
occurs in papilloedema due to raised intracranial pressure, the pressure in
the veins at the disc rises above the ocular pressure and so spontaneous
venous pulsation is lost. Absence of spontaneous venous pulsation is seen in
5 20% of those with normal nerve heads, but in this case venous collapse at
the nerve head can be induced by light pressure on the globe.
A large blind spot will be found on visual fi eld testing, corresponding to the
swollen nerve head. In chronic papilloedema the fi eld may become constricted.
A fi eld defect may, however, be caused by the space - occupying
lesion causing the papilloedema.
Abnormal neurological signs may indicate the site of a space - occupying
lesion.
Investigation
CT and MRI scanning will identify any space - occupying lesion or enlargement
of the ventricles. Following neurological consultation (and normally after a
scan) a lumbar puncture will enable intracranial pressure to be measured.
Treatment is dependent on fi ndings.
Lecture note ophtalmo

The retina is the innermost layer, or "camera film," of the eye. The retina is attached firmly to
the underlying choroid at the optic nerve posteriorly and at the ora serrata anteriorly. Between
these two points, the retina is in contact with the choroid but it is not attached. The ora serrata
is the junction of the retina and ciliary body. The retina is only 0.4 mm in thickness and is
thinnest in the region of the macula. Histologically, the retina is made up of 10 distinct layers.
Basically, the retina senses light through the rods and cones in its outer layer (closest to the
RPE), performs initial signal processing in its middle layer, and encodes and transmits the
data in its inner layer, the nerve fiber layer. The nerve fiber layer is directly under the inner
limiting membrane of the retina, the layer closest to the vitreous. These nerve fibers course
along the inner portion of the retina and aggregate to form the optic nerve. On leaving the
eye, the nerve fibers become myelinated.
Within the retina are several important structures: the optic disc, the retinal vessels, and the
macula. Figure 10-8 illustrates the retina of the left eye.
The optic disc is located at the nasal aspect of the posterior pole of the retina. This is the head
of the optic nerve, from where the nerve fibers of the retina exit the eye. The optic disc is 1.5
mm in diameter and is ovoid. It is lighter than the surrounding retina and appears yellowish-
pink. The disc margins are sharp with some normal blurring of the nasal portion. African-
American patients may have pigmentation at the margins. The physiologic cup is the center
of the disc, where the retinal vessels penetrate. This small depression normally occupies
about 30% of the disc diameter.
The retinal vessels emerge from the disc and arborize on the retinal surface. The arteries are
brighter red and thinner than the veins. An artery-to-vein ratio of 2:3 is normal.

Textbook physic diagnosis
PAPILLOEDEMA PAPILLITIS
Visual Acuity Normal (usually) Reduced
Pupil Normal Poor response to
direct light
Visual Field Normal (increased Central scotoma
blind spot) or field defect
Colour Vision Normal Defective
Usually Usually
bilateral unilateral

The ophthalmoscopic appearance of disc oedema in papilloedema
and papillitis is the same. Papilloedema is differentiated from papillitis
by the presence of other clinical features.
Papilloedema is a passive swelling of the optic disc commonly
caused by raised intracranial pressure. The condition is usually
bilateral. Vision is normal unless the macula is affected by oedema
or exudates. Rarely is the vision diminished because of optic atrophy
in severe unrelieved papilloedema. The visual fields and colour vision
are also normal although the blind spot is sometimes enlarged. The
pupillary reflex to light is normal.
Papillitis is an inflammation of the optic nerve, frequently of
uncertain aetiology. Disseminated sclerosis is an important cause.
The condition is usually unilateral. Because the optic nerve is
inflamed, there is usually marked visual loss. A central scotoma is
present and the eye may have defective colour vision, especially to
red. The pupil is dilated, with sluggish or no reaction to direct light.

Ischaemic optic neuropathy
Ischaemia to the optic nerve head from arteriolar sclerosis and
temporal arteritis also causes sudden visual loss with a swollen optic
disc in the elderly population. It is important to exclude temporal
arteritis by blood erythrocyte sedimentation rate (ESR). Systemic
steroids will protect the unaffected eye. The prognosis for the affected
eye is usually poor and optic atrophy usually follows.
papilloedema
Pseudo-papilloedema is a variation in the appearance of the optic
disc which is sometimes mistaken for true disc oedema. It should be
clearly differentiated from the latter in order to avoid unnecessary
investigations and anxiety to the patient.
One common cause of pseudo-papilloedema is hypermetropia
where the disc margin is blurred. Other common causes include
drusen (yellowish-white deposits at the optic disc) and opaque
myelinated nerve fibres. Fundal fluorescein angiography can help to
distinguish pseudo-papilloedema from true disc oedema.

Color atlas ophthalmology


OCULAR MANIFESTATIONS
Papilledema is observed on fundus examination, by direct or indirect
ophthalmoscopy. Indirect ophthalmoscopy with a 20 D lens provides a
better stereoscopic view. A 90 D lens used in conjunction with biomicroscopy
provides excellent magnification and stereopsis. It is useful to
characterize the changes in the optic nerve head that occur in papilledema
as being mechanical or vascular in nature.


The five mechanical clinical signs of optic disc edema are:
Blurring of the optic disc margins.
Filling in of the optic disc cup.
Anterior extension of the nerve head (3 D = 1 mm of elevation).
Edema of the nerve fiber layer.
Retinal or choroidal folds, or both.
The five vascular clinical signs of optic disc edema are:
Venous congestion of arcuate and peripapillary vessels.
Papillary and retinal peripapillary hemorrhages.
Nerve fiber layer infarcts (cotton-wool spots).
Hyperemia of the optic nerve head.
Hard exudates of the optic disc.

In addition, elements of optic disc swelling can be used to help characterize
the papilledema as early, fully developed, chronic, or late.

In early papilledema, disc hyperemia, disc swelling, blurring of the disc margins, and blurring of the nerve fiber layer are
found (Fig. 9-6-1).
In fully developed papilledema, gross elevation of the optic nerve head and engorged and dusky veins appear, peripapillary
splinter hemorrhages and sometimes choroidal folds arise, and retina striae are seen (Fig.
9-6-2).

In chronic papilledema, fewer hemorrhages occur, the optic disc cup is obliterated completely, less disc hyperemia is seen,
and hard exudates occur within the nerve head (Fig. 9-6-3).

In late disc edema, secondary optic atrophy occurs, disc swelling subsides, retinal arterioles are narrowed or sheathed, and
the optic disc appears dirty gray and blurred, secondary to gliosis (Fig. 9-6-4).

1. Rubin R, Strayer DS, Rubin E. Rubin's Pathology: Clinicopathologic Foundations of Medicine:
Wolters Kluwer Health/Lippincott Williams & Wilkins; 2011.