Pathophysiology:

Mortality/Morbidity:

History:

Symptoms of papilledema include transient visual obscurations (e.g.: dimming or blackout of vision in one or both eyes lasting for a few seconds) which may be predominantly or uniformly orthostatic (i.e.: after bending over), progressive loss of peripheral vision in one or both of the eyes, most often starting in the nasal inferior quadrant, followed by loss of central visual field (possibly affecting visual acuity) and, lastly, loss of color vision, and blurring and distortion (i.e.: metamorphopsia) of central vision caused by macular wrinkling and subretinal fluid spreading from the swollen optic disc. Sudden visual loss is due to intraocular hemorrhage secondary to peripapillary subretinal neovascularization related to chronic papilledema.

Physical:

Visual acuity is usually normal until significant peripheral visual field loss with progressive postpapilledema optic atrophy has occurred. Occasionally limited abduction of one or both of the eyes results from increased ICP. This loss of ocular mobility is termed false-localizing sixth cranial nerve palsy.

Lab Studies:

An increased incidence of anti-cardiolipin antibodies has been reported in patients with IIH. This test should always be obtained in working up patients suspected of having IIH.

Other blood tests recommended include a CBC, Sed rate, Iron testing, ANA, anti-dsDNA, coagulation panel, and Lyme disease screening tests.

Imaging Studies:

MR venography can be reserved for patients who are at greater risk for dural venous sinus thrombosis, such as those with suggestion of thrombosis on MRI, nonobese or male individuals, or those with a documented procoagulant state. Sagittal T1- weighted images often provide excellent views of the superior sagittal sinus, and these typically are included in routine MRI.

Brain CT scan is less expensive than MRI and is adequate to rule out larger tumors or lesions, but it is not as sensitive as MRI for meningeal infiltration and/or dural venous sinus thrombosis.

Procedures:

Cerebrospinal fluid studies should include opening pressure, WBC counts with differential, RBC counts, protein and protein electrophoresis, glucose, cultures, serologic markers, tumor markers, and cytology. Most patients with typical history, gender, and body habitus need only routine CSF tests. However, extra fluid should be frozen in case the preliminary workup reveals unexpected abnormalities, such as pleocytosis or elevated gamma globulin, indicating that more complete investigation for autoimmune, infectious, or neoplastic conditions is warranted.

Medical Care:

When new visual field loss is documented, medical management should be coupled with plans for emergency surgical intervention if the visual function continues to deteriorate or does not improve immediately with corticosteroid treatment.

Diet is an important adjunct to treatment. A weight-reduction diet coupled with an exercise program is strongly advised to all patients with IIH as evidence demonstrates that weight loss is associated with improvement of papilledema in these patients.

Surgical Care:

Prognosis:

Medical/Legal Concerns:

The exact time window within which vision loss can be reversed after symptomatic decline is not known. Opinions among experts in the field vary as to how rapidly and aggressively any given patient should have been treated. Usually erring on the side of rapid intervention (hours to days) in such patients is better. This is a dramatic opportunity to save vision that can be easily lost. A major medicolegal pitfall is created when poor outcome is coupled with the perception of delayed treatment.

One of the standard teachings has been that pregnancy exacerbates or triggers the onset of symptomatic IIH. However, at present little statistical evidence exists of a causal association between the two conditions, beyond the fact that both events are common in the age group and gender that is predominantly affected by the disease.