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.