BASICS

DESCRIPTION

Papilledema is defined as optic disc swelling secondary to elevated intracranial pressure. It is typically bilateral but can be asymmetric.

Pediatric Considerations

Unlike in adults, idiopathic intracranial hypertension (IIH) occurs with equal frequency in prepubescent boys and girls.

Pregnancy Considerations

Treatment with acetazolamide during pregnancy is controversial and generally avoided during the first trimester of pregnancy. Intra-abdominal catheters and CSF diversion procedures may be limited by the growing fetus.

EPIDEMIOLOGY

Incidence

• The incidence and prevalence of all causes of papilledema are difficult to establish.

• IIH, also known as pseudotumor cerebri, is most common in women with obesity between 18 and 45 years of age.

• IIH 1–3 per 100,000 in the general population

• 20 per 100,000 in women with obesity ages 20–40 years

• The incidence of IIH is equal in prepubescent boys and girls.

RISK FACTORS

• Weight gain and obesity predispose to IIH.

• Sleep apnea may increase the risk of papilledema (IIH).

• Some medications (tetracyline, vitamin A, growth hormone) may cause elevated intracranial pressure.

• Hypercoagulability predisposes to CVST.

• Surgical procedures such as internal jugular vein ligation (CVST)

Genetics

• No clear genetic predisposition to papilledema

• Some conditions may have a genetic component (e.g., hyercoagulable disorders predisposing to CVST).

• Some familial reports of IIH

GENERAL PREVENTION

• Weight loss (IIH)

• Serial eye examinations for patients taking medications thought to predispose to elevated intracranial pressure.

PATHOPHYSIOLOGY

• Mostly a mechanical process whereby nerve fibers within the optic nerve are compressed by elevated CSF pressure in the subarachnoid space. This results in blocked axoplasmic flow. As swelling progresses vascular obstruction may occur, with dilation of the retinal venous system.

ETIOLOGY

The compartment surrounding the brain and spinal cord act as a closed system containing neural tissue, CSF, and the vascular system. Alterations in any of these three components may cause elevated intracranial pressure.

COMMONLY ASSOCIATED CONDITIONS

• IIH (most common in ophthalmic practice): The diagnostic criteria for IIH include the absence of a mass lesion on neuroimaging, elevated opening pressure on lumbar puncture, and normal CSF consistency (1)[A].

• Venous obstruction (including CVST), which may cause stroke and death

• Intracranial mass lesion

• Cerebral edema

• Meningitis

• Thickening of the skull and craniosynostosis syndromes

• Hydrocephalus

• Endocrinologic dysfunction

• Spinal cord or choroid plexus tumor (rare)

DIAGNOSIS

HISTORY

• Symptoms related to the underlying cause of papilledema: for example, headache and stiff neck in meningitis, seizures and focal deficits from mass lesion, and stroke related to venous sinus thrombosis

• Symptoms that can be related to elevated intracranial pressure include the following:

– Headache, often positional and improves in the recumbent position and worsens with the Valsalva maneuver

– Nausea and vomiting

– Pulsatile or pulse-synchronous tinnitus or other auditory phenomena likely related to turbulent flow within the cerebral venous sinuses

– Transient visual obscurations or temporary blackouts of vision lasting seconds, often related to a change in position. They are thought to occur from a transient loss of perfusion due to optic disc swelling.

– Diplopia occurs most commonly from sixth nerve palsy, which may be unilateral or bilateral. It may also be from comitant esotropia and rarely from third or fourth nerve palsy.

PHYSICAL EXAM

• Visual acuity is generally normal in early papilledema unless there is another abnormality (e.g., choroidal folds).

• Pupils are brisk early on, as the optic neuropathy progresses they may become sluggish and, with asymmetric involvement a relative afferent pupillary defect may be present.

• Color vision is relatively preserved unless central visual function is compromised.

• Visual field defects are nerve fiber bundle type: arcuate defects and enlargement of the blind spot. As optic disc edema progresses central visual function may be lost.

• Optic disc edema is most commonly bilateral but may be asymmetric and rarely unilateral. Grading schemes (e.g., the Frisén staging system) have been proposed to provide a semiquantitative measure of the optic disc edema (2)[C]. Early changes include opacification of the retinal nerve fiber layer, loss of spontaneous venous pulsations, and loss of the normal physiologic cup. Later vascular changes occur with optic disc hemorrhages, cotton wool spots, and retinal and choroidal folds. More severe disc edema may cause retinal vascular occlusions and retinal, subhyaloid, and vitreous hemorrhage.

• Hard exudate may form within the retina in a pattern of a macular star.

• Choroidal folds, typically horizontal and within the macula may cause a reduction in visual acuity.

• Concentric folds around the optic disc (Paton’s lines) may occur with optic disc swelling.

• Several changes in the appearance of the optic disc may develop over time, which are not specific for papilledema. Disc pallor occurs and a reduction in the number of optic disc hemorrhages and cotton wool spots can occur. Pseudodrusen (areas of blocked axoplasmic flow that look like optic disc drusen but are not calcium deposits) may develop. Alterations of the retinal pigment epithelium may occur around the optic disc in areas where there was prior swelling or subretinal fluid. Gliosis may develop on the surface of the optic disc. Once it is damaged the optic disc may not swell from papilledema.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

• Possible higher prevalence of anemia in IIH

• Coagulopathy may exist in CVST.

Follow-up & special considerations

Can check electrolytes, particularly potassium levels, in patients treated with diuretics

Imaging

Initial approach

• Neuroimaging of the brain is vital. MRI is preferred over CT in most patients. Some recommend magnetic resonance venography (MRV) or computed tomographic venography (CTV) to exclude CVST in all patients with papilledema, particularly in those without a structural lesion who do not fit the typical demographic profile of IIH (3).

• MRV and CTV may show narrowing of the cerebral venous sinuses (which may be a reflection of elevated intracranial pressure).

• Nonspecific imaging findings from elevated intracranial pressure may include an empty sella, increased T2-weighted signal consistent with increased CSF around the optic nerves, posterior bowing of the globes, and enhancement in the area of the optic nerve heads.

Follow-up & special considerations

In atypical patients with papilledema, spinal cord imaging may reveal a tumor or structural abnormality in the region of craniocervical junction.

Diagnostic Procedures/Other

• Ultrasonography (with the 30° test) may be helpful (e.g., rule out optic disc drusen).

• Lumbar puncture may be used to measure the opening pressure and assess CSF consistency.

• Intracranial pressure monitoring may be used to follow and document spikes in CSF pressure.

Pathological Findings

• Optic disc edema with axonal swelling

• Retinal nerve fiber layer thinning

DIFFERENTIAL DIAGNOSIS

• Hypertensive retinopathy

• Optic disc drusen

• Congenitally anomalous optic discs

• Optic disc edema from other causes including ischemic optic neuropathy, inflammatory optic neuropathy (particularly neuroretinitis), and compressive optic neuropathy

TREATMENT

MEDICATION

First Line

• Acetazolamide

• Corticosteroids to decrease brain edema in patients with mass lesions or cerebral edema

• Corticosteroids have also been used in IIH.

• Anticoagulants for CVST

Second Line

• Furosemide and other diuretics

• Topiramate, both for the headache and to help with appetite suppression in patients with IIH

ADDITIONAL TREATMENT

General Measures

Weight loss for patients with IIH

Issues for Referral

• Neurosurgery for mass lesions and CSF diversion procedures (4)[B]

• Consider hematologic consultation for coagulation disorders in patients with CVST

• Endocrinologic evaluation in some patients (e.g., polycystic ovarian syndrome)

Additional Therapies

• Gastric bypass surgery and ensuing weight loss can lead to an improvement of papilledema in patients with IIH (5)[C].

• Repeated lumbar punctures can be performed as a temporizing measure to lower CSF pressure (e.g., during pregnancy) in patients with IIH.

SURGERY/OTHER PROCEDURES

• Neurosurgery for mass lesions and CSF diversion procedures such as ventriculostomy

• Ventriculoperitoneal and lumboperitoneal shunting

• Surgical intervention is often undertaken after a trial of medical therapy in IIH.

• Optic nerve sheath fenestration, particularly in patients with progressive visual loss despite medical therapy, more marked visual loss, and those without headache as a predominant symptom

IN-PATIENT CONSIDERATIONS

Initial Stabilization

Depending on the cause of papilledema some patients may require hospitalization (e.g., for intravenous antibiotics in meningitis or hydrocephalus with impending brain herniation)

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Depending on degree of optic disc edema, visual deficit, and choice of treatment

Patient Monitoring

• Serial eye examinations with visual field assessment should be considered.

• Depending on the cause of papilledema, patients may need to follow with other specialists including neurosurgeons to monitor tumors or shunts, and neurologists to treat headache.

DIET

• Weight loss (often 10–20% of body weight) can cause resolution of optic disc edema in IIH.

• Current clinical trials to determine the efficacy of acetazolamide versus weight loss in IIH.

PATIENT EDUCATION

• Patients should be instructed to return for examination if they develop progressive symptoms including visual loss, headaches, and diplopia.

• Recurrent or progressive papilledema (depending on the etiology) may represent:

– Tumor growth

– Recurrent or progressive CVST

– Progressive optic disc edema from IIH

– Worsening hydrocephalus

PROGNOSIS

Systemically depends on the cause of the papilledema. Visual outcome depends on the degree of optic disc edema and visual loss at the onset of treatment. Excellent visual prognosis for patients with mild optic disc edema.

COMPLICATIONS

• Fixed visual loss

• Chronic headaches

• Allergic reactions or side effects to medication (particular acetazolamide)

• Complications related to lumbar puncture (bleeding, infection, postspinal headache)

• Complications related to shunts (infection, catheter migration, failure necessitating revision)

• Complications from optic nerve sheath fenestration, including visual loss and diplopia

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