Papilledema

Gina Lowell
September 27, 2005
Anatomy
Papilledema
Pathophysiology
Arrest of axoplasmic transport
1.2 million axons converge at the optic disc, forming the optic nerve
Constant anterograde and retrograde flow of materials responsible
for maintenance of the axons
Elevated ICP inhibits outflow of these materials, resulting in optic
disc edema, or papilledema
Early Chronic Optic atrophy
Papilledema
Etiology
Increased intracranial pressure
Blood
Hypertension, malignant
Hemorrhage
Obstruction of venous outflow (venous thrombosis)
Brain
Mass lesions (tumor, abscess)
Cerebral edema (trauma, HIE, electrolyte abnormalities, metabolic
aberrations, meningitis)
CSF
Increased production (choroid plexus papilloma - rare)
Decreased drainage (communicating/non-communicating
hydrocephalus, shunt malfunction)
Idiopathic
Pseudotumor cerebri (Idiopathic intracranial hypertension)
Idiopathic intracranial hypertension
Most commonly seen in adult females
Prevalance: 1-19 cases per 100,000 depending on body weight
Obesity is frequently associated with IIH in adults
Pathogenesis: Unknown
Theories include increased brain water content, elevated venous pressure
inhibiting CSF resorption, endocrine dysfunction
Clinical associations such as thyroid disease, Addison disease, SLE,
medications (tetracycline, minocycline, steroids, growth hormone, OCPs), or
vitamin supplements (vit A) should be classified as secondary pseudotumor
syndromes
Presentation
Headache: most common
Transient visual symptoms: double vision, blurring
Tinnitus
Papilledema
Must have this to diagnose IIH
Usually bilateral; can be unilateral
CN VI palsy
Normal CT/MRI
Elevated opening pressure on LP: >250mm H20 (lying down)
Idiopathic intracranial hypertension
Less commonly seen in children
Different clinical picture
Girls and boys affected equally
Obesity is not often seen
Retrospective review of 10 prepubertal children with IIH showed:
Most common presenting symptoms: stiff neck, diplopia (40%, 40%)
Most common presenting sign: strabismus (CN VI palsy) (80%)
Sustained visual field abnormalities (85%)
Severe unilateral visual loss (1 patient)
Index of suspicion for secondary pseudotumor syndromes or
other causes of optic disc edema must remain high
Idiopathic intracranial hypertension
Sequelae
Chronic papilledema
Axonal injury
Optic atrophy
Visual impairment
Potentially irreversible
Treatment
Medical
Steroids
Acetazolamide
(Weight loss)
Surgical
Lumboperitoneal shunt
Optic nerve sheath fenestration
Serial LPs
Optic neuritis
Inflammation of the optic nerve resulting in demyelination
Etiologies
Parainfectious
Viral, measles, mumps, varicella, pertussis
Infectious
Lyme disease, syphilis
Immunocompromised: TB, toxoplasmosis, CMV, cryptococcus
Post-vaccination
Autoimmune
Sarcoidosis
Vasculitides, SLE
Multiple sclerosis
ONTT (optic neuritis treatment trial)
Frequently represents the first manifestation of MS
Optic neuritis
Presentation
Abrupt loss of vision
Usually unilateral, can be bilateral
Pain with extraocular movements
Afferent pupillary defect
Marcus Gunn pupil
Optic disc
Swelling: Papillitis
No swelling: Retrobulbar neuritis
Treatment
IV steroids
Close follow-up
Optic neuritis
Normal Papillitis
Early papilledema
References
Binder DK et al. Idiopathic intracranial hypertension [literature
review]. Neurosurgery. 2004;54(3):538-552
Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial
hypertension in prepubertal pediatric patients: characteristics,
treatment, and outcome. Am J Ophthalmol. 1999;127(2):178-182
Forsyth R, Farrell K. Headache in childhood. Pediatr. Rev.
1999;20:39-45
Kennedy C, Carter S. Relation of optic neuritis to multiple sclerosis
in children. Pediatrics. 1961;28(3):377-387
Larsen GY, Goldstein B. Increased intracranial pressure. Pediatr.
Rev. 1999;20:234-239
Rothermel H. Optic neuropathy in children with Lyme disease.
Pediatrics. 2001;108:477-481
Yanoff: Ophthalmology. 2nd Edition, Copyright 2004. Chapters
189,190.