Skip to Main Content

++
Table Graphic Jump Location
Favorite Table | Download (.pdf) | Print
The Problem
The optic nerve is abnormal
Common Causes
Optic nerve hypoplasia
Papilledema
Optic nerve coloboma
Glaucoma
Other Causes
Pseudopapilledema
Myelinated nerve fibers
Albinism
KEY FINDINGS
History
Optic nerve hypoplasia
If bilateral, often presents with poor vision and abnormal eye movements in infancy
Unilateral hypoplasia may be associated with strabismus due to decreased vision
If pituitary dysfunction, may have poor growth, developmental delay, and abnormal stress response
Papilledema
Headache
Double vision
Transient visual obscuration (brief episodes of dimmed vision)
Idiopathic intracranial hypertension
Frequently associated with medication in children
Corticosteroids, retinoic acid
Also associated with obesity
Optic nerve coloboma
Abnormal pupil appearance (if iris coloboma present)
Poor vision or strabismus if fovea affected
Associated systemic diseases
CHARGE Association
Glaucoma
May have family history
Infants and young children
Light sensitivity
Eye appears large, cornea cloudy
Older children
Usually asymptomatic
Pseudopapilledema
Optic disc drusen
Trisomy 21
Farsightedness
Myelinated nerve fibers
Decreased vision due to myopia
Examination
Optic nerve hypoplasia
Infant with poor vision, nystagmus
Poor pupil responses
Papilledema
Visual acuity usually normal (unless severe)
Optic nerve elevated, swollen, hemorrhages, cotton wool spots
Decreased outward movement of eye due to sixth nerve palsy
Optic nerve coloboma
May have associated iris coloboma
Variable involvement of optic nerve, retina
Usually inferonasal quadrant
Glaucoma
Infants and young children
Corneal clouding, eye larger than normal
Usually unable to visualize optic nerve
Older children
Enlarged cup:disc ratio
Pseudopapilledema
Trisomy 21—abnormal vascular pattern
Optic nerve drusen
Irregular lumpy appearance
White deposits within nerve
Myelinated nerve fibers
White feathery appearance beginning at optic nerve
Extend along course of retinal nerve fibers
++

Similar to examination for retinal hemorrhages, evaluation of the optic nerve is often difficult in pediatric patients, particularly infants and toddlers. In older children, examination of the nerve may be part of the routine well-child examination, or may be performed due to specific symptoms (such as headache). The presence of papilledema requires prompt evaluation, including neuroimaging and consultation with a neurologist. If the patient has an abnormal-appearing nerve, but no symptoms of increased intracranial pressure, referral to a pediatric ophthalmologist should be considered to evaluate for pseudopapilledema, which could obviate the need for further extensive testing. Most children with other abnormal optic nerve findings should be referred to a pediatric ophthalmologist.

++

What Shouldn’t Be Missed

++

Optic nerve hypoplasia is a frequent cause of very poor vision and nystagmus in infants. Due to the difficulty of direct ophthalmoscopic evaluation of the optic nerves in infants with nystagmus, such patients require referral to a pediatric ophthalmologist. Optic nerve hypoplasia may be associated with pituitary abnormalities, and these patients may be unable to mount a normal stress response, potentially causing severe problems during even mild illnesses. This possibility should be kept in mind until the patient is evaluated by an endocrinologist.

++

Papilledema may occur in patients with idiopathic intracranial hypertension. In children, this is most commonly associated ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessPediatrics Full Site: One-Year Subscription

Connect to the full suite of AccessPediatrics content and resources including 20+ textbooks such as Rudolph’s Pediatrics and The Pediatric Practice series, high-quality procedural videos, images, and animations, interactive board review, an integrated pediatric drug database, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessPediatrics

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.