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Disc Edema

Fig. 9.1 a, b (a) Optic nerve swelling in the right eye. The disc margins are blurry, and there is no central cup. (b) Sagittal section of a swollen optic nerve.

9.1 Mechanisms of Optic Nerve Edema

Mechanisms of optic nerve edema include the following:

1. Local optic nerve injury, such as from inflammation (anterior optic neuritis or papillitis), ischemia (anterior ischemic optic neuropathy), fluctuations in intraocular pressure
(high, as in acute glaucoma, or low, as in ocular hypotony), and toxicity

2. Blockage of retrograde axonal transport from optic nerve compression (optic nerve tumor or orbital mass) and raised intracranial pressure (papilledema)

9.2 Differentiating True Disc Edema from Pseudoedema

Differentiating true optic nerve head edema from pseudoedema is essential (▶ Table 9.1, ▶ Fig. 9.2 and ▶ Fig. 9.3). In most cases, pseudoedema appearance results from
a congenital anomaly of the optic nerve and does not require any workup, whereas true disc edema is associated with numerous concerning disorders.

Table 9.1 Characteristics of true disc edema versus pseudoedema

True disc edema (▶ Fig. 9.2) Pseudoedema (▶ Fig. 9.3)

Elevated optic nerve Elevated optic nerve

Margins blurry Sharp margins

Vessels obscured Vessels not obscured

Venous dilation and tortuosity Absence of central cup

Peripapillary hemorrhages and exudates Anomalous retinal vasculature (arterial branching)

Leakage on fluorescein angiogram No leakage on fluorescein angiogram

Fig. 9.2 a, b (a) True disc edema with (b) leakage on fluorescein angiography (late phase).

Fig. 9.3 a, b (a) Pseudoedema with (b) no leakage on fluorescein angiography (there is late staining only).

9.3 Differential Diagnosis of Disc Edema

Disc elevation without true swelling:

Optic disc anomalies

Myelinated nerve fibers (▶ Fig. 8.50 and ▶ Fig. 9.4)

Drusen (▶ Fig. 8.44 and ▶ Fig. 9.5)

Tilted disc (▶ Fig. 8.49)

Crowded disc

Optic disc infiltration

Leber hereditary optic neuropathy

True disc swelling:

Elevated intracranial pressure (papilledema) (▶ Fig. 9.6)

Inflammatory optic neuropathy (▶ Fig. 9.7)

Demyelinating

Sarcoidosis or other inflammatory diseases

Infectious

Neuroretinitis

Vascular optic neuropathy

Anterior ischemic optic neuropathy (▶ Fig. 9.8)

Nonarteritic

Arteritic

Diabetic papillopathy

Central retinal vein occlusion (▶ Fig. 9.9)

Carotid-cavernous fistula

Malignant systemic hypertension (▶ Fig. 9.10)

Compressive optic neuropathy

Neoplastic

Meningioma (▶ Fig. 9.11)

Hemangioma

Lymphangioma

Non-neoplastic

Thyroid ophthalmopathy

Orbital inflammatory pseudotumor

Infiltrative optic neuropathy

Neoplastic

Leukemia

Lymphoma

Glioma

Non-neoplastic

Sarcoidosis

Toxic

Metabolic/nutritional deficiencies

Traumatic optic neuropathy

Intraocular hypotony (low intraocular pressure)

Fig. 9.4 Myelinated nerve fibers.

Fig. 9.5 Optic nerve head drusen.

Fig. 9.6 Bilateral papilledema.

Fig. 9.7 a,b (a) Right anterior optic neuritis with moderate disc edema. (b) Axial T1-weighted magnetic resonance imaging of the orbits with contrast and fat suppression, showing enhancement of the right

optic nerve (arrow).

Fig. 9.8 a,b (a) Right anterior ischemic optic neuropathy with mild disc edema and a few peripapillary hemorrhages. (b) Corresponding inferior altitudinal visual field defect on a 30–2 Humphrey visual field

test.

Fig. 9.9 Central retinal vein occlusion with disc edema and numerous retinal hemorrhages distant from the swollen optic nerve.

Fig. 9.10 Malignant systemic hypertension with severe disc edema, retinal hemorrhages, and retinal exudates.

Fig. 9.11 a, b (a) Left optic nerve sheath meningioma with disc edema and shunt vessels. (b) Axial computed tomography of the orbits with contrast showing enhancement along the left optic nerve

(arrows).

9.4 Evaluation of the Patient with Disc Edema

Once optic disc edema is confirmed, it should be determined whether it is related to an optic nerve disorder (optic neuropathy) or to raised intracranial pressure. Papilledema is
the term used to describe optic disc edema resulting from raised intracranial pressure (▶ Fig. 9.12). All other optic disc edema is termed disc edema or swollen optic nerve.
▶ Table 9.2 compares the characteristics of disc edema from anterior optic neuropathy with those from raised intracranial pressure.

Fig. 9.12 Bilateral asymmetric (right eye worse than left) mild papilledema from raised intracranial pressure.

Table 9.2 Characteristics of disc edema from anterior optic neuropathy versus those from raised intracranial pressure

Optic neuropathy with disc edema Papilledema (raised ICP)

Decreased visual acuity Normal visual acuity (until late)

Decreased color vision Normal color vision

Central, arcuate, or altitudinal visual field defect Enlarged blind spot, nasal defects, constriction of visual fields

Disc edema more often unilateral Disc edema almost always bilateral

Often isolated (or associated with symptoms or signs related to underlying disease) Other symptoms and signs of raised ICP (headache, nausea, diplopia from sixth nerve palsies,

pulsatile tinnitus, transient visual obscurations)

Focal neurologic symptoms if focal intracranial process

Abbreviation: ICP, intracranial pressure.

The mechanisms responsible for raised intracranial pressure and papilledema are as follows:

Hydrocephalus (▶ Fig. 9.13)

Intracranial mass

Tumor, abscess (▶ Fig. 9.14)

Intracerebral hemorrhage

Subdural/epidural hemorrhage

Large vascular malformation

Meningeal process

Infectious

Inflammatory

Neoplastic

Increased venous pressure

Cerebral venous thrombosis

Idiopathic intracranial hypertension

Fig. 9.13 Axial head computed tomography without contrast showing obstructive hydrocephalus (dilated ventricles).

Fig. 9.14 Axial fluid-attenuated inversion recovery magnetic resonance imaging of the brain showing a large brain tumor with mass effect responsible for raised intracranial pressure.

Most disorders producing raised intracranial pressure are life-threatening emergencies. The finding of papilledema should prompt an immediate workup, ideally in a specialized
center with up-to-date neuroimaging, as well as neurologic and ophthalmological consultations.

The workup should include the following:

Looking for an underlying neurologic process

Careful evaluation of the visual function (visual acuity and formal visual field testing), as papilledema can result in permanent visual loss from secondary optic atrophy

Checking blood pressure (severe systemic hypertension or malignant hypertension may produce bilateral disc edema that mimics papilledema)

Pearls

Although papilledema is a reliable sign of raised intracranial pressure, the absence of disc edema does not rule out raised intracranial pressure in a
patient presenting with headache.

Increased venous pressure produces symptoms and signs of raised intracranial pressure, including papilledema.

The causes of increased venous pressure include all of the causes of decreased venous return (▶ Fig. 9.15):

Fig. 9.15 a, b (a) Drainage of the cerebrospinal fluid (CSF) into the intracranial venous sinuses. The CSF is passively resorbed across the Paccioni granulations (most are within the superior sagittal sinus). In

cases of venous hypertension or venous thrombosis, the CSF resorption decreases, and the CSF pressure increases. (b) Anatomy of the intracranial venous system. ([a] From Schuenke M, Schulte E,

Schumacher U, Ross LM, Lamperti ED, Voll M. THIEME Atlas of Anatomy, Head and Neuroanatomy. Stuttgart, Germany: Thieme; 2007. Illustration by Karl Wesker.) ([b] From Schuenke M, Schulte E,

Schumacher U, Ross LM, Lamperti ED, Voll M. THIEME Atlas of Anatomy, Head and Neuroanatomy. Stuttgart, Germany: Thieme; 2007. Illustration by Markus Voll.)

Right cardiac insufficiency

Pulmonary hypertension

Sleep apnea syndrome

Superior vena cava syndrome

Jugular vein occlusion

Dural fistula

Cerebral venous stenosis

Cerebral venous thrombosis

Cerebral venous thrombosis is a classic cause of raised intracranial pressure (▶ Fig. 9.16; see also Chapter ▶ 20). Patients may present with isolated raised intracranial
pressure, thereby mimicking idiopathic intracranial hypertension. Early recognition may prevent a devastating stroke and visual loss from chronic papilledema.

Fig. 9.16 Sagittal T1-weighted magnetic resonance imaging without contrast showing a hyperintense superior sagittal sinus (arrows) from cerebral venous thrombosis.

When evaluating a patient with presumed papilledema (raised intracranial pressure), neuroimaging needs to be obtained emergently to rule out an intracranial process (▶ Fig.
9.17). Magnetic resonance imaging (MRI) with contrast of the brain is the ideal test and is the most sensitive to detect intracranial masses, infiltrative and meningeal processes,
and cerebral venous thrombosis. Computed tomography (CT) without contrast, which is often the test of choice in the emergency room, is in most cases not helpful in these
patients, unless it is followed by brain MRI. Indeed, the CT is helpful to detect intracranial hemorrhages, hydrocephalus, and large mass lesions, but it does not rule out any of
the other intracranial lesions. Patients with a normal head CT should be investigated further with brain MRI (see Chapter ▶ 4). A normal brain MRI scan in the setting of
papilledema suggests a meningeal process, venous hypertension, or idiopathic intracranial hypertension as the cause of raised intracranial pressure. A lumbar puncture with
measurement of the cerebrospinal fluid (CSF) opening pressure and CSF analysis should always be performed.
Fig. 9.17 Diagram illustrating the diagnosis of disc edema. OP, opening pressure; CSF, cerebrospinal fluid.

9.5 Classification and Progression of Papilledema

Patients with papilledema often have no visual symptoms initially. They may complain of “flashing lights” or transient visual obscurations (brief episodes of visual loss
occurring in one or both eyes), often precipitated by changes in posture, such as standing up after bending over. Untreated chronic papilledema results in visual loss: central
visual acuity is normal until late, and patients develop insidious progressive visual field constriction (▶ Fig. 9.18, ▶ Fig. 9.19, ▶ Fig. 9.20, ▶ Fig. 9.21, ▶ Fig. 9.22).

Fig. 9.18 Early papilledema. The disc borders are blurry and elevated. There is a peripapillary halo.

Fig. 9.19 Moderate papilledema. All borders are obscured, and the disc appears larger. The blood vessels are also obscured. There is a peripapillary halo.

Fig. 9.20 Severe papilledema. The entire optic nerve head are elevated and obscured with numerous hemorrhages and exudates. The margins of the nerves and the vessels cannot be seen. The veins are

dilated and tortuous.

Fig. 9.21 Severe, chronic papilledema. The optic nerves protrude anteriorly with a dome-shaped appearance. There are exudates extending into the macula.

Fig. 9.22 a, b Secondary optic atrophy. (a) With time, untreated papilledema diminishes, even in the setting of persistently elevated intracranial pressure. The discs become atrophic, and the retinal vessels

become narrow and sheathed. (b) The nerves become flat and pale. Peripapillary changes persist from previous disc edema.

Formal visual field testing (Humphrey perimetry shown in ▶ Fig. 9.23) is often abnormal in papilledema. Blind spot enlargement and nasal defects are common initially (top,
▶ Fig. 9.23). They may progress, usually circumferentially, to involve the central 30 degrees of the visual field (middle). Severe devastating visual field loss (bottom) is often
permanent if raised intracranial pressure is not promptly treated (note that even with the severe visual field loss seen in the bottom example, visual acuity was still relatively
preserved at 20/25 OD [right eye] and 20/40 OS [left eye]).

Fig. 9.23 a–c Progression of visual field defects in papilledema (24–2 Humphrey visual fields). (a) Early changes with enlarged blind spots. (b) Constriction of visual fields, worse nasally. (c) Severe

constriction.

Pearls

Visual loss from papilledema happens with any cause of papilledema. Hence it is very important to look for papilledema in all patients with headache
or known hydrocephalus, brain tumor, or meningitis and to prevent visual loss when papilledema is present.

9.6 Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension (IIH), previously called pseudotumor cerebri, is defined as increased intracranial pressure with normal imaging and normal CSF contents.
By definition, papilledema from a meningeal process or cerebral venous thrombosis should not be classified as IIH. Patients with IIH have symptoms and signs of raised
intracranial pressure, such as headaches, nausea, pulsatile tinnitus, papilledema (and visual loss), and diplopia from unilateral or bilateral sixth nerve palsy. The management of
the disease is based on the severity of headaches and the presence of visual loss, specifically visual field deficits.

9.6.1 Diagnosis of IIH

The criteria for the diagnosis of idiopathic intracranial hypertension are as follows:

Signs and symptoms of raised intracranial pressure (including papilledema)

No localizing neurologic signs, in an alert patient, other than abducens nerve paresis

Normal neuroimaging studies (neuroimaging should include a good quality MRI scan ± magnetic resonance venography [MRV] or computed tomographic venography
[CTV] to rule out cerebral venous thrombosis). Nonspecific signs of increased intracranial pressure are common and include empty sella, flattening of the globes, dilation of
the optic nerve sheath, meningoceles, and stenosis of the intracranial transverse venous sinuses.

Documented increased opening pressure (≥ 250 mm of water) but normal CSF composition

Primary structural or systemic causes of elevated intracranial pressure excluded (e.g., chronic meningitis or cerebral venous thrombosis)

9.6.2 Cause of IIH

The cause of this disorder is unknown. It involves most often young, obese women and may be associated with other factors. The main factors associated with IIH are obesity or
recent weight gain, sleep apnea syndrome, chronic anemia, and medications (vitamin A, isoretinoid, tetracycline, and cyclosporine).

9.6.3 Treatment of IIH

The goals of the management of IIH are to relieve headaches and diplopia and to preserve visual function (▶ Fig. 9.24). There is a high spontaneous remission rate.

Fig. 9.24 Management of idiopathic intracranial hypertension (IIH).

The lumbar puncture performed as part of the workup is usually the first step of the treatment, as it immediately decreases the intracranial pressure (at least transiently).
Headaches that do not improve (at least transiently) after lumbar puncture are unlikely to be entirely the result of raised intracranial pressure.

In rare cases, patients develop rapidly progressive visual loss that requires emergent surgical treatment. A brief course of intravenous steroids is sometimes helpful in this
setting, but steroids should not be prescribed routinely or chronically in IIH (because of weight gain and rebound effect).

Surgical treatments in idiopathic intracranial hypertension include the following:

CSF shunting procedures (performed by neurosurgeons) (▶ Fig. 9.25)

CSF drainage into the peritoneum most often

Lumboperitoneal shunt or ventriculoperitoneal shunt

Preferred when headaches are severe

Obstruction or disconnection requires a revision in about 50% of lumboperitoneal shunts.

Optic nerve sheath fenestration (performed by ophthalmologists) (▶ Fig. 9.26)

Decompression of the optic nerve by making a window into its dural sheath from a transconjunctival medial or lateral approach

Done on the eye with the worst visual function first (often needs second eye surgery)

Preferred when visual loss is predominant and headaches are mild

The fenestration fails in up to one third of cases within 3 years.

Endovascular venous stenting of a stenosed transverse sinus (performed by interventional neuroradiologists) (▶ Fig. 9.27)

Most IIH patients have bilateral stenoses of the distal portion of the intracranial transverse venous sinus (▶ Fig. 9.27). Although these stenoses contribute to the
intracranial hypertension (▶ Fig. 9.28), they are not necessarily the primary cause of increased intracranial pressure and they do not need to be treated in most patients.

Rarely, endovascular stenting of a stenosed sinus can be proposed to decrease the intracranial venous hypertension and reduce the intracranial pressure.

Fig. 9.25 Idiopathic intracranial hypertension. Improvement of symptoms and signs after a lumboperitoneal shunt procedure (top images: before shunt; bottom images: after shunt). There are bilateral

sixth nerve palsies (bilateral abduction deficits), bilateral papilledema, and visual field defects.

Fig. 9.26 Optic nerve sheath fenestration. The optic nerve sheath is exposed after a transconjunctival medial or lateral approach. A few slits or a window is made with a sharp blade into the sheath,

allowing cerebrospinal fluid to escape (arrows).

Fig. 9.27 Magnetic resonance venography with contrast showing the major intracranial venous sinuses (red arrows) and bilateral distal focal transverse venous stenoses (yellow arrows) in idiopathic

intracranial hypertension. The left image shows the venous sinuses seen from behind, and the right image shows a lateral view of the venous sinuses.

Fig. 9.28 Venous hypertension in idiopathic intracranial hypertension. The stenosed transverse venous sinuses impair venous return from the brain into the internal jugular vein, thereby increasing the

intracranial venous pressure. The increase in venous pressure is responsible for impaired passive cerebrospinal fluid (CSF) resorption into the intracranial venous sinuses, contributing to intracranial

hypertension. Increased intracranial pressure (ICP) results in collapse of the transverse venous sinuses, with resultant worsened transverse venous stenosis, with subsequent worsening of the

intracranial venous hypertension.

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