is this disc normal ?

Is This Disc Normal ?

Raed Behbehani , MD FRCSC

Introduction

• Ophthalmologist are called to evaluate optic discs frequently .

• R/O Papilledema , infiltrative processes, compression or atrophy.

• Anomalous disc is not a diseases disc .

History

• Asymptomatic

• Loss of vision , eye pain .

• Cardinal symptoms of high intracranial pressure.

Cardinal Symptoms of High ICP

• Headache : holocranial , tension-like band , worse upon awakening and in supine position and is reduced when they are active.

• Migraine : aura, unilateral , throbbing , light sensitivity , phonophobia, nausea and vomiting , family history of migraine and car sickness.

Cardinal Symptoms of High ICP

• Tinnitus : “swishing” sound , synchronous with pulse and worse when supine. (transmission of high ICP causes turbulent flow in venous sinuses).

• Tinnitus is very rare in other types of headaches .

Cardinal Symptoms of High ICP

• Transient visual obscuration : Brief , bilateral (seconds) dimming in vision related to change in posture.

• If unilateral , optic disc drusen (not related to position).

• Differentiate from Uhthoff's phenomenon.

Cardinal Symptoms of High ICP

• Diplopia : Binocular horizontal (sixth nerve paresis).

Examination• Optic nerve cup : usually small or absent in

anomalous optic disc. In papilledema it is present until late stage.

• Vessel branching : trifurcations , loops , increased branching

• Blood vessels dilated , congested• opacification of the RNFL —> blurring of vessels—

>hemorrhages and exudates• Spontaneous venous pulsations : can be absent in

normal.

Normal vs Anomalous

Early Papilledema

“as the disc swells lateralwards, it displaces the retina.- throwing it into a series of folds which run concentric with the edge of the disc. This lateral bulging is

due to the distension of the most peripheral nerve fibers” Paton and Holmes 1911

Papilledema ?

http://library.med.utah.edu/NOVEL/Hoyt

Papilledema ?

http://library.med.utah.edu/NOVEL/Hoyt

Features of Anomalous discs

Psudopapilledema True PapilledemaDisc margin vessels clear Disc vesses obscured

Elevation confined to disc Elevation of the peripapillary RNFL

Small cupless disc Loss of cup late

Anamolous disc vessels (tri-, quadrifurcation)

Normal vessels

No hemorrhage or exudates

NFL hemorrhage, cotton wool spots, exudate

Ancillary Testing• Ultrasound : Hyper-reflective bodies• OCT : Can be useful in differentiating true vs false

papilledema , buried drusen sometimes do not to exhibit a significant difference in reflectivity from surrounding disc tissue.

• Fluorecin Angiogroaphy : Red-free (drusen) , staining and leakage with fluorescin (inflammation).

• Neuro-imaging : MRI, CT• LP

Patton’s lines

Outer retinal folds (ORF)

Patton’s lines

Peripapillary Wrinkles (PPW)

Copyright © 2016 Journal of Neuro-Ophthalmology. Published by Lippincott Williams & Wilkins.

Deformation in Bruch’s Membrane

FIG. 6. A. Optic disc tissue in a patient with papilledema, showing the upward angling and displacement of Bruch's membrane (red arrows) in the right and left eyes. B. There is a change in displacement of Bruch's membrane in the same eye before and after treatment of raised intercranial pressure (23).

Optical Coherence Tomography in Papilledema: What Am I Missing? Kardon, RandyJournal of Neuro-Ophthalmology. 34():S10-S17, September 2014.doi: 10.1097/WNO.0000000000000162

Copyright © 2016 Journal of Neuro-Ophthalmology. Published by Lippincott Williams & Wilkins.

OCT in Distinguishing Papilledema vs Pseudopapilledema

FIG. 7. A very large, coalescing druse imaged in several SD-OCT modalities. A. Fundus photo with 2 vertical markers placed on either side of the druse (obtained with 3D disc scan on Topcon 3D OCT 2000). B. Low-resolution SD-OCT image, obtained on same 3D disc scan. C. High-resolution image, obtained with 7-line Raster on Topcon 3D OCT 2000. D. High-resolution (5-line Raster) image, obtained with Zeiss Cirrus HD-OCT (30). SD-OCT, spectral domain optical coherence tomography.

Optical Coherence Tomography in Papilledema: What Am I Missing? Kardon, RandyJournal of Neuro-Ophthalmology. 34():S10-S17, September 2014.doi: 10.1097/WNO.0000000000000162

Optic Nerve Drusen

• Most common cause for anomalous discs

• 2% population

• Can be superficial or buried.

• “Scalloped” disc margin.

Optic Nerve Drusen

Buried Drusen

SDOCT in Buried Drusen

Focal, round, hyper-reflective mass causing nasal optic disc elevation with an irregular contour (arrows).

Disc Drusen

Tilted Disc

• Myopia

• Oblique insertion (tilted) , margin indistinct

Myelinated Nerve Fibers

• Bight-white , feathery appearance over the retina.

Little red disc

Peri-papillary Atrophy

Case Failed a driving test. Started on Xalatan, two months later IOP was

19 OU. Has been driving for the last 10 years “with no

accidants”.Strabismic amblyopia OD , Strabismus surgery.

High Myopia ( -7 D OU ). Mother : Diabetes.

Segmental Optic Nerve Hypoplasia

Segmental Optic Nerve Hypoplasia

• Maternal diabetes in 1s trimester.

• No other systemic abnormalities.

• Can be found in families with no history of maternal diabetes.

• Interference in gestational development of superior retinal ganglion cells or their axons.

• Occasionally associated congenital lesions of the retina , chiasm, and posterior visual pathway.

Segmental ONH

Homonymous Hemioptic Hypoplasia

140 By the kind permission of Professor WF Hoyt.

Morning Glory Disc Anomaly

• Funnel-shaped excavation with peripapillary pigment changes.

• Usually unilateral .• Central glial tuft.• Vessels emanate

from periphery.• MRI/MRA is

warranted

Morning Glory Disc Anomaly

Morning Glory Disc Anomaly

• Wide head , flat nose , hypertelorism , midline notch in upper lip or palate.

• Agenesis of cropus callosum, achiasmia , posterior dilatation of lateral ventricles.

• Panhypopitutarism.• Ipsilateral intracranial vascular dysgensis

(hypoplasia of carotid and major vessels. • PHACE syndrome (posterior fossa , facial

hemangioma , arterial anamolies , cardiac and aorta and eye) anomalies.

PVL

Optic Nerve Coloboma

• Failure of closure of embryonic fissure.

• Deep excavation more prominent inferiorly.

• Unilateral or bilateral.

• Serous macular detachment.

• CHARGE , Aicardi syndrome, Goldenhar.

Morning Glory Disc Coloboma

Symmetric defect Asymmetric (inferior)

central glial tuft No glial tuftPeripapilalry pigmentary

changesMinimal Peripapillary pigmentary changes

Anomalous retinal vessles Normal vasculature

Aicardi Syndrome• Infantile spasms , agenesis

of corpus callous, abnormal EEG.

• Anolmalous disc and peripapillry chorioretinal lacunae.

• Coloboma , ONH , and pigmentation.

• Facial skeletal and vertebral abnormalities.

Papillorenal syndrome

• No central vessels in excavation (vacant disc)

• Progressive renal disease and later renal failure.