PAPILLEDEMA

Definition
Papilledema is by definition bilateral optic disk swelling due to raised intracranial pressure,
of which the most common causes are cerebral tumors, abscesses, subdural hematoma,
arteriovenous malformations, subarachnoid hemorrhage, acquired hydrocephalus, meningitis,
and encephalitis.
Epidemiology
Epidemiologic data from the 1950s describe papilledema inas many as 60% of patients with
brain tumors. Since then, advances in neuroradiologyhave significantly reduced the incidence
of papilledema. Thediagnostic importance of the disorder has decreased accordingly.
Etiology
In ophthalmology practice, a frequent cause is idiopathic intracranial hypertension. This is
characterized by papilledema, no neurologic abnormality except for perhaps sixth or more
rarely seventh cranial nerve palsy, normal neuroimaging studies, including brain MRI, and
normal cerebrospinal fluid studies apart from increased intracranial pressure.An adequate
theory to fully explain the pathogenesis of papilledema is lacking. Current thinking centers
around a mechanical model in which increased intracranial pressure and impeded axonal
plasma flow through the narrowed lamina cribrosa cause nerve fiber edema. However, there
is no definite correlation between intracranial pressure and prominence of the papilledema.
Nor is there a definite correlation between the times at which the two processes occur.
However, severe papilledema can occur within a few hours of increased intracranial pressure,
such as in acute intracranial hemorrhage. Therefore, papilledema is a conditional, unspecific
sign of increased intracranial pressure that does not provide conclusive evidence of the cause
or location of a process. In approximately 60% of all cases, the increased intracranial pressure
with papilledema is caused by an intracranial tumor; 40% of all cases are due to other causes,
such as hydrocephalus, meningitis, brain abscess, encephalitis, malignant hypertension, or
intracranial hemorrhages. The patient should be referred to a neurologist, neurosurgeon, or
internist for diagnosis of the underlying causes.Less common causes of papilledema are
spinal

tumors,

acute

idiopathic

polyneuropathy

(Guillain-Barr

syndrome),

mucopolysaccharidoses, and craniosynostoses, in which various factors, including decreased

cerebrospinal fluid absorption, abnormalities of spinal fluid flow, and reduced cranial
volume, contribute to the raised intracranial pressure.
Special forms
Foster Kennedy syndrome: This refers to isolated atrophy of the optic nerve due to direct
tumor pressure on one side and papilledema due to increased intracranial pressure on the
other side. Possible causes may include a meningioma of the wing of the sphenoid or frontal
lobe tumor.
Hypotension papilledema: This refers to a nerve fiber edema due to ocularhypotension.
Possible causes may include penetrating trauma or fistula secondary to intraocular surgery.
Pathophysiology
The disc swelling in papilledema is the result of axoplasmic flow stasis with intra-axonal
edema in the area of the optic disc. The subarachnoid space of the brain is continuous with
the optic nerve sheath. Hence, as the cerebrospinal fluid (CSF) pressure increases, the
pressure is transmitted to the optic nerve, and the optic nerve sheath acts as a tourniquet to
impede axoplasmic transport. This leads to a buildup of material at the level of the lamina
cribrosa, resulting in the characteristic swelling of the nerve head. Papilledema may be absent
in cases of prior optic atrophy. In these cases, the absence of papilledema is most likely
secondary to a decrease in the number of physiologically active nerve fibers.
Symptoms and diagnostic considerations
The symptoms of papiledema are headache, nausea, vomiting,short term vision loss, reduced
scope of vision and changes in vision. Visual function remains unimpaired for long time. This
significant discrepancy between morphologic and functional findings is an important
characteristic in differential diagnosis. Early functional impairments can include reversible
obscurations. Perimetry testing may reveal an increase in the size of the blind spot . Central
visual field defects and concentric narrowing of the visual field are late functional
impairments that occur with existing complex atrophy of the optic nerve. Papilledema is
characterized by significant morphologic findings and only slight visual impairment.The
following phases may be distinguished by ophthalmoscopy:

Early phase: First the nasal margin and then the superior and inferior margins of the optic
disk are obscured because of the difference in the relative densities of the nerve fibers (see
optic disk). The optic cup is initially preserved. This is important in a differential diagnosis to
exclude pseudopapilledema and optic disk drusen. The optic disk is hyperemic due to
dilatation of the capillaries, and there is no pulsation in the central retinal vein. Edema can
produce concentric peripapillary retinal folds known as Patons folds.
Acute phase: This is characterized by increasing elevation of the optic disk, radial
hemorrhages around the margin of the optic disk and grayish white exudates. The optic cup is
often no longer discernible. The color of the optic disk will be red to grayish red.
Chronic phase. Significant optic disk edema is present. The optic cup is obliterated, and the
hyperemia will be seen to subside.
Atrophic phase. Proliferation of astrocytes results in complex or secondary atrophy of the
optic nerve.
It takes 2448 hours for early papilledema to occur and 1 week to develop fully. It takes 68
weeks for fully developed papilledema to resolve following adequate treatment. Acute
papilledema may reduce visual acuity by causing hyperopia and occasionally is associated
with optic nerve infarction, but in most cases vision is normal apart from blind spot
enlargement. Chronic atrophic and vintage papilledema are associated with gradual
constriction of the peripheral visual field, particularly inferonasal loss, and transient visual
obscurations. Sudden reduction of intracranial pressure or systolic perfusion pressure may
precipitate severe visual loss in any stage of papilledema.
Differential diagnosis
This includes pseudopapilledema, optic disk drusen abnormalities of the optic disk without
functional impairment, optic disk edema with hypertension, and optic neuritis.
Complications
Medullated nerve fibers with papilledema, chronic papilledema in resolution,chronic
papilledema with pseudo drusen and chornic papilledema with hemorrhagic and exuadative
complications.

Treatment
The treatment of papilledema must be directed to the underlying cause. Idiopathic intracranial
hypertension generally affects obese young women, and maintained weight loss is the
primary treatment objective. The major morbidity is visual loss due to papilledema, but
headaches may also be troublesome. Oral acetazolamideusually 250 mg one to four times
daily but up to 500 mg four times dailyor diuretics such as furosemide are usually effective
in reducing optic disk swelling. Lumboperitoneal shunting or optic nerve sheath fenestration
is indicated if there is severe or progressive loss of vision or if medical therapy is not
tolerated. Repeated lumbar punctures are rarely indicated except as a temporary measure
prior to surgical therapy. Systemic steroid therapy may be helpful if there is severe acute
visual loss but is generally contraindicated because of associated weight gain. Headaches
usually respond to control of intracranial pressure, but other treatments, such as anti-migraine
therapy, may be helpful. It is essential that patients with idiopathic intracranial hypertension
undergo regular visual field assessments.
Prognosis
A chronic case of mild Papilledema can make a person lose vision moderately. But once
vision loss begins, it can become permanent in a span of few days or weeks. Hence, urgent
treatment should be carried out to cure the condition at the earliest.

Early phase: The nasal margin of the optic disk is partially obscured.The optic disk is
hyperemic due to dilatation of the capillaries, and the optic cup is still visible.

Acute Phase: The optic disk is increasing elevated and has a gray to grayish red color. Radial
Hemorrhages around the margin of the optic disk and grayish white exudates are observed.
The optic disk can no longer be clearly distinguished.

OPTIC NERVE
BASIC SCIENCE
The optic nerve extends from the posterior pole of the eye to the optic chiasm. After this
characteristic crossing, the fibers of the optic nerve travel as the optic tract to the lateral
geniculate body. Depending on the shape of the skull, the optic nerve has a total length of 35
55mm. The nerve consists of:
An intraocular portion.
An intraorbital portion.
An intracranial portion.

Intraocular Portion of the Optic Nerve

The intraocular portion of the optic nerve is visible on ophthalmoscopy as the optic disk. All
the retinal nerve fibers merge into the optic nerve here, and the central retinal vessels enter
and leave the eye here. The complete absence of photoreceptors at this site creates a gap in
the visual field known as the blind spot.
Shape and size: The optic disk (Fig. 13.2) is normally slightly vertically oval with an average
area of approximately 2.7mm2 and a horizontal diameter of approximately 1.8mm. There is a
wide range of physiologic variability in the size of the optic disk; its area may vary by a
factor of seven, and its horizontaldiameter by a factor of two and one-half.
Color: The normal physiologic color is yellowish orange. The temporal half of the optic disk
is usually slightly paler.
Margin: The margin of the optic disk is sharply defined and readily distinguished from the
surrounding retinal tissue. On the nasal side, the greater density of the nerve fibers makes the
margin slightly less distinct than on the temporal side. A common clinical observation is a
crescent of pigment or irregular pigmentation close to the optic disk on the temporal side;
sometimes the sclera will be visible through this crescent.
Prominence of the optic disk: The normal optic disk is not prominent. The nerve fibers are
practically flush with the retina.

Neuroretinal rim: This consists of the bundles of all the optic nerve fibers as they exit through
the scleral canal. The rim has a characteristic configuration: The narrowest portion is in the

temporal horizontal region followed by the nasal horizontal area; the widest areas are the
vertical inferior and superior areas.
Optic cup: This is the slightly eccentric cavitation of the optic nerve that has a slightly
flattened oval shape corresponding to that of the neuroretinal rim. It is the brightest part of
the optic disk. No nerve fibers exit from it. The size of the optic cup correlates with the size
of the optic disk; the larger the optic disk, the larger the optic cup. Because enlargement of
the optic cup means a loss of nerve fibers in the rim, it is particularly important to
documentthe size of the optic cup. This is specified as the horizontal and vertical ratios of cup
to disk diameter (cup/disk ratio). Due to the wide range of variability inoptic disk size, it is
not possible to specify absolute cup/disk ratios that indicate the presence of abnormal
processes.
Central retinal artery and vein: These structures usually enter the eye slightly nasal to the
center of the optic disk. Visible pulsation in the vein is normal. However, arterial pulsation is
always abnormal and occurs with disorders such as increased intraocular pressure and aortic
stenosis. Cilioretinal vessels are aberrant vessels originating directly fromthe choroid (short
posterior ciliary arteries). Resembling a cane, they usually course along the temporal margin
of the optic disk and supply the inner layers of the retina.
Blood supply to the optic disk: The optic disk receives its bloodsupply from the ring of Zinn,
an anastomotic ring of small branches of theshort posterior ciliary arteries and the central
retinal artery. Both groups of vessels originate fromthe ophthalmic artery, which branches off
of the internal carotid artery and enters the eye through the optic canal. The central retinal
artery and vein branch into the optic nerve approximately 8mm before the point at which the
optic nerve exits the globe. Approximately 10 short posterior ciliary arteries penetrate the
sclera around the optic nerve.
The Intraorbital and Intracranial Portion of the Optic Nerve
The intraorbital portion begins after the nerve passes through a sieve-like plateof scleral
connective tissue, the laminacribrosa. Inside theorbit, theoptic nerve describes an S-shaped
course that allows extreme eye movements. After the optic nerve passes through the optic
canal, the short intracranial portion begins and extends as far as the optic chiasm. Like the
brain, the intraorbital and intracranial portions of the optic nerve are surrounded by sheaths of

dura mater, pia, and arachnoid. The nerve receives its blood supply through the vascular pia
sheath.