3. 3rd week of gestation: the first evidence of primitive
eye formation occurs
Neural plate destined to form
prosencephalon
Optic sulcus formation
depression
Formation of optic vesicle and optic stalk
EMBRYOLOGY
4. Optic stalk is the original connection between the
optic vesicle & the forebrain
Optic sulcus deepens & the walls of
prosencephalon bulge out
Optic vesicle formation
Proximal part of optic vesicle become
constricted & elongated
Optic stalk formation
4th week
5. Develops in the frame work of optic stalk
Optic Nerve Head
Formed as the optic stalk encloses the hyaloid
artery (the 8th week)
From the hyaloid artery, the vascular bud
develops within Bergmeister’s Papilla
Hyaloid artery disappears before birth
Bergmeister’s papilla becomes atrophic & the
physiologic cup develops (at 15th week)
EMBRYOLOGY OF OPTIC NERVE
7. Optic Nerve
Axons
Develops from the embryonic optic stalk
Stalk lumen is progressively occupied by the
axons growing from the ganglion cells (the 7th
week)
Axons fully occupy the stalk, reach the brain and
a rudimentary optic chaism is formed (the 8th
week)
Myelination starts near chaism and stops at
lamina cribrosa
8. Optic nerve sheaths:
Glial element:
Ѻ Develops from the neuroectodermal cells
forming the outer wall of the optic stalk
Ѻ Differentiates into astrocytes and oligodendrocyte
Ѻ Form from the mesenchymal cells
Ѻ Begin to appear at the end of the 7th week
9. Vasculature
Ѻ Development of capillaries in the optic nerve
and the CNS is similar
Ѻ Vessels and connective tissue from the pia mater
begin to enter the proximal optic nerve (at the
11th week)
Ѻ Capillaries are separated by astrocyte sheet and
perivascular space
Ѻ Vascularization is completed in the 18th week
10. Weeks of Gestation Length (mm) Developing Events
4 2.5-6 mm Short optic stalk
5 5-9 mm Development of hyaloid
vasculature
6 8- 14 mm Embryonic cleft closes
7 13-18 mm Growth of axons
Formation of optic nerve
8 18-31 mm Stalk fully occupied by axons
Axons of optic nerves reach
the brain
Rudimentary optic chiasm
established
Optic nerve vascularization
starts to form
Glimpse of embryology of optic nerve
11. Week of Gestation Length (mm) Developing Events
11 65-73 mm Vascular-connective septa invade
the nerve
12 80 mm Pia mater, arachnoid & dura
mater distinguishable
Glial filaments appear
14 105 mm Subarachnoid space appears
15 117-123 mm Physiologic cup starts to form
18 160 mm Vascularization of the optic nerve
completed
23 220 mm Myelinization starts
Contd……
12.
13. 2nd cranial nerve
Starts from optic disc, extends upto optic
chiasma
Backward continuation of nerve fiber layer of
retina (axons of ganglion cells)
Also contains afferent fibers of light reflex and
some centrifugal fibers
Optic Nerve
14. • An outgrowth of brain
• Not covered by neurilemma: Does not regenerate
when cut
• Fibers of optic nerve are very fine (2-10 µm in
diameter ) & are millions in number
• Surrounded by meninges unlike other peripheral
nerves
• Both primary & secondary neurons are in retina
Morphologically & embryologically , the optic
nerve is comparable to a sensory tract of
brain (white matter) because:
15. About 47-50 mm in length
Divided into 4 parts:
Intraocular (1 mm)
Intraorbital (30 mm)
Intracanalicular (6-9 mm)
Intracranial (10 mm)
Optic
Nerve
Parts Of Optic Nerve
17. Intraocular Part
Intraocular Part
SNFL Prelaminar Lamina Cribrosa Retrolaminar
Passes through sclera, choroid & appears in eye as
optic disc
1.5 mm in diameter
Expands to 3 mm behind sclera, where the neurons
acquire myelin sheath
Divided into 4 portions (from anterior to posterior):
18.
19. Surface Nerve Fiber Layer
Composed of axonal bundles (94% nerve fibers of
retina + 5% astrocytes)
Optic disc is covered by thin layer of astrocytes, ILM
of Elschnig: separates it from vitreous
When central portion of this membrane gets
thickened: Central meniscus of Kuhnt
Near the optic nerve, all layers of retina (except
NFL) are separated from it by: Intermediate tissue
of Kuhnt
20. Prelaminar Region
Predominant structures: neurons and increased
quantity of astroglial tissue
Border tissue of Jacoby (a cuff of astrocytes)
separates the nerve from the choroid
The loose glial tissue does not bind the axon
bundles together as do the Muller cells of the
retina
the disc swells so easily in papilloedema
while the adjacent retina does not
SO
21. Lamina Cribrosa
Fibrillar sieve-like structure
Composed of fenestrated sheets of scleral
connective tissue lined by glial tissue
Bundles of ON fibers leave the eye through LC
Border tissue of Elschnig :
- rim of collagenous tissue with few glial cells
- intervenes b/w the choroid and sclera & ON
fibers
22. Retrolaminar Region
Characterized by decrease in astrocytes & acquisition
of myelin supplied by oligodendrocytes
Addition of myelin sheath doubles the diameter of
ON (from 1.5 to 3.0 mm) as it passes through the
sclera
Axonal bundles are surrounded by connective tissue
septa
The posterior extent of the retrolaminar region is not
clearly defined
23.
24. Ophthalmoscopic Features of
Optic Nerve Head
Optic Disc: part of nerve head visible with
ophthalmoscope
Intra papillary parts:
-optic cup & neuroretinal rim
-separated by scleral ring of Elschnig
Scleral
ring of
elschnig
25. Why the normal disc is Pink
Light entering the disc diffuses
among adjacent columns of
glial cells and capillaries
Acquires the pink color of the
capillaries
Light rays that exit through
the tissue via the nerve fiber
bundles are pink
give the disc its characteristic color
&
26. Nerve fiber loss in chronic glaucoma:
- leads to increased exposure of the lamina
as axons are lost
- its pores become more visible as the cup
enlarges,
- there is increased white reflex at disc
27. Disc size
Disc shape
Usually oval
Vertical diameter being on average 9% longer
than horizontal diameter
The cup is 8% wider in the horizontal
Normal disc area ranges widely from 0.86 mm2
to 5.54 mm2
Macrodiscs: area > 4.09 mm2
Microdiscs: area < 1.29 mm2
28. Applied
Primary macrodiscs : associated with condition
such as pits of the optic nerve ‘Morning glory
syndrome’
Secondary macrodisc : associated with High
Myopia and Buphthalmos
NAION is common in smaller ON heads
due to problems of vascular perfusion and of
limited space
Same is true for optic nerve head drusen
due to blockage of orthograde axoplasmic flow
29. Pseudo papilloedema is encountered with smaller
optic nerve head
-particularly in highly hypermetropic eye
Susceptibility of the superior & inferior disc
regions to damage: due to higher pore-to-disc
area
30. Optic Cup
Funnel shaped depression
- varies in form & size
- usually off-centre towards
the temporal side
Cup correlates with disc:
-large in large discs
-small in small discs (may be absent)
3D measurement of cup shap: using confocal
miscroscopy or stereoscopic techniques
31. Neuroretinal Rim
Tissue outside the cup
Contains the retinal nerve axons as they enter
the nerve head
ISNT rule (inferior- thickest)
Greater axonal mass and vascularity in the
inferotemporal region
32. Applied
In primary open angle glaucoma:
- progressive loss of retinal ganglion cells
- leading to enlargement of cup, particularly at
upper & lower poles of disc
- leading to vertically oval cup
But: Horizontally oval cup-normal
Occurrence of flame shaped haemorrhages on
rim, usually at inferior or superior temporal
margin: early sign of glaucoma
33. Applied
Cup/Disc Ratio
Ratio of cup & disc width
Measured in same meridian, usually vertical or
horizontal
Doesn’t differ by more than 0.2 in 99% subjects
Asymmetry of greater than 0.2 is of diagnostic
importance in glaucoma
34. Parapapillary Chorioretinal Atrophy
Crescentric region of chorioretinal atrophy, found
temporally in normal disc
May be exaggerated in chronic glaucoma or high
myopia
Two zones of PPCRA:
more peripheral zone & is an irregular hypo- or hyper
pigmented region
Corresponds to RPE that failed to extend to the disc
margin
Zone alpha/choroidal crescent
35. Zone beta or Scleral Crescent
Related to disc centrally or zone alpha peripherally
Consists of marked atrophy of pigment epithelium
& choriocapillaries, with good visibility of larger
choroidal vessels
Applied
The zones are larger in total area & individually in
the presence of chronic glaucoma
36.
37.
38. Retinal Vessels
Emerge on medial side of cup, slightly
decentered superonasally
Temporal arteries take an arcuate course as
they leave the disc
Nasal arteries take more direct, though curved
course
Course of arteries and veins is similar but not
identical
this avoids excessive shadowing of rods &
cones
39. Venous pulsation:
Arterial pulsation:
- observed at disc in 15-90% of normal subjects
- due to pulsatile collapse of the veins as ocular
pressure rises with arterial inflow into uvea
- rare, usually pathological
Eg. High ocular pressure or aortic incompetence
40. Intraorbital Part
Extends from back of the eyeball to the optic
foramina
Sinuous course to give play for the eye movements
Covered by dura, arachnoid and pia
The pial sheath contains capillaries and sends septa
to divide nerve into fasciculi
The SAS containing CSF ends blindly at the sclera but
continues intracranially
Central retinal artery, accompaning vein crosses SAS
inferomedially about 10 mm from the eyeball
41. Applied
Posteriorly, near optic foramina, the ON is closely
surrounded by annulus of Zinn & origin of four
rectus muscles
Some fibers of SR & MR are adherent to its sheath
Account for the painful ocular movements seen in
retrobulbar neuritis
42. Relations of intraorbital part of ON
The long & short ciliary nerves & arteries
surround the ON before these enter the eyeball
B/w ON & LR muscle are situated the ciliary
ganglion, divisions of the oculomotor nerve, the
nasociliary nerve, the sympathetic & the
abducent nerve
The ophthalmic artery, superior ophthalmic vein
& the nasociliary nerve cross the ON superiorly
from the lateral to medial side
43.
44. Intracanalicular Part
Applied
Closely related to ophthalmic artery
OA crosses the nerve inferiorly from medial to
lateral side in the dural sheath
Leaves the sheath at the orbital end of the canal
Sphenoid and post ethmoidal sinuses lie medial
to it and are separated by a thin bony lamina
This relation accounts for retrobulbar neuritis
following infection of the sinuses
45. IntracranialPart
Lies above the cavernous sinus & converges with its
fellow to form the chiasm
Ensheaths in pia mater
Receives arachnoid & dural sheaths at the point of its
entry into the optic canal
Internal carotid artery runs, at first below & then
lateral to it
Medial root of the olfactory tract & the anterior
cerebral artery lie above it
Lies above the cavernous sinus & converges with
its fellow to form the chiasm
Ensheaths in pia mater
Receives arachnoid & dural sheaths at the point of
its entry into the optic canal
Internal carotid artery runs, at first below & then
lateral to it
Medial root of the olfactory tract & the anterior
cerebral artery lie above it
46. Meningeal Sheaths Of Optic Nerve
Intracranial part : pia only
Intracanalicular and Intraorbital part :
pia, arachnoid and dura
Anteriorly, all 3 meningeal sheaths
terminate by becoming continuous ith
sclera
47. In the optic nerve head
Exactly same as in retina
Fibers from the peripheral part of the retina:
- lie deep in the retina
- occupy the most peripheral part of the optic disc
Fibers originating closer to the optic nerve head:
- lie superficially in the retina
- occupy a more central portion of the disc
Arrangements of nerve fibres
in optic nerve
48.
49. In the proximal region
In the distal region
Exactly as in retina
- i.e. upper temporal & lower temporal fibers are
situated on the temporal half of the optic nerve
- separated from each other by a wedge shaped
area occupied by the Pmb
The upper nasal and lower nasal fibers are situated
on the nasal side
The macular fibers are centrally placed
52. • Supplied by CENTRAL
RETINAL ARTERY
• Occasionally from the
CILIORETINAL ARTERY,
ciliary vessel derived from
prelaminar region
SURFACE
NERVE
FIBRE
LAYER
54. • Supplied by SHORT
POSTERIOR CILIARY
ARTERIES and
ARTERIAL CIRCLE OF
ZINN/HALLER
LAMINA
CRIBROSA
REGION
55. • Supplied chiefly by PIAL
VESSELS & SHORT
POSTERIOR CILIARY
VESSELS , with some help
from CRA & RECURRENT
CHOROIDAL VESSELS
RETRO
LAMINAR
REGION
56. Optic disc edema occurs as prelaminar axons
swell from blocked orthogonal axoplasmic flow at
level of lamina cribrosa
Insufficient blood flow through posterior ciliary
arteries due to thrombosis, hypotension, vascular
occlusion cause ONH infarction
Applied
57. SUPPLIED BY TWO SYSTEMS OF VESSELS
PERIAXIAL SYSTEM OF
VESSLES
AXIAL SYSTEM OF VESSELS
60. The axial system consists of:
- Intraneural branches of central retinal artery
- Central collateral arteries which come off from
CRA before it pierces the nerve
- Central artery of ON
68. In each zone:
- venules drain into central retinal vein or when
present into a duplicated vein (an embryonic
persistence of hyaloid veins)
Occasionally septal veins in retrolaminar region
drain into pial veins
Some small venules from prelaminar region or from
SNFL (optiociliary veins) drain into choroid
70. • CENTRAL RETINAL VEIN
OPTIC NERVE HEAD
• PERIPHERAL PIAL PLEXUS
• CENTRAL RETINAL VEIN
ORBITAL PART
• PIAL PLEXUS WHICH ENDS IN ANTERIOR CEREBRAL &
BASAL VEIN
INTRACRANIAL PART
VENOUS DRAINAGE OF THE
OPTIC NERVE
71.
72. Blood Brain Barrier At The Optic
Nerve
The capillaries of ON head , retina & CNS, have non-
fenestrated endothelial linings with tight junctions
b/w adjacent endothelial cells
Which is responsible for Blood Tissue Barrier to the
diffusion of small molecules across capillaries
However it is incomplete as a result of continuity
b/w the extracellular spaces of choroid and ON head
at level of choroid (in prelaminar region)
73. Signs of Optic Nerve Dysfunction
Reduced VA
Afferent Pupillary Defects
Dyschromatopsia
Visual Field Defects
75. Lesions Of The Optic Nerve
Lesions Of The Visual
Pathway
Complete blindness on the affected side
Abolition of direct light reflex on ipsilateral side &
consensual on contralateral side
Near (accommodation) reflex is present
Causes - optic atrophy
-Traumatic avulsion of optic nerve
-Indirect optic neuropathy
-Acute optic neuritis
76. Lesion Through Proximal Part of Optic Nerve
Ipsilateral blindness
Contralateral hemianopia
Abolition of direct light reflex on affected
side & consensual on contralateral side
Near reflex is intact
77.
78. Disc usually lacking physiological cup
Have crowded appearance mimicking
papilledema
Hyperopic Optic Disc
Myopic Optic Disc
Disc is larger
Surrounded by white crescent of bare sclera,
on the temporal side
CDR is bigger mimicking glaucomatous cupping
80. Tilted Optic Disc
Occurs when nerve exits the eye at an oblique angle
Superotemporal disc: raised, simulating disc swelling
Inferotemporal disc: flat or depressed
Resulting in an oval-shaped disc with long axis at an
oblique angle
81. Optic Disc Pit
Round or oval, gray or white depression in the optic
disc
Commonly found temporally
82. Optic Disc Drusen
Globules of mucoproteins & mucopolysaccharides
that progressively calsify in the optic disc
Thought to be the remnants of the axonal transport
system of degenerated retinal ganglion cells
83. Myelinated Nerve Fibres
White , feathery patches that follow NFL Bundles
Peripheral edges fanned out
Simulated disc edema
84. Optic Disc Coloboma
Results from an incomplete closure of the embryonic
fissure (inferonasal)
Defect of the inferior aspect of ON
White mass: glial tissue fills the defect
Inferior NRR: thin or absent, superior NRR: relatively
normal
85. Morning Glory Disc
Congenital funnel shaped excavation of the posterior pole
White tuff of glial tissue covers central portion of cup
Blood vessels appear to be increased in no. & emanate
from the edge of disc
86. Optic Nerve Hypoplasia
Optic nerve head appears abnormally small due to a low
no. of axons
Gray or pale disc surrounded by light-colored peripapillary
halo of hypopigmentation d/t concentric chorioretinal
atrophy (Double Ring Sign)
87. Aicardi Syndrome
Rare genetic disorder a/w multiple bilateral
depigmented chorioretinal lacunae clustered around a
hypoplastic, colobomatous or pigmented optic disc
95. Optic Atrophy
It refers to degeneration of the optic nerve, which
occurs as an end result of any pathologic process that
damages axons in the anterior visual system, i.e. from
retinal ganglion cells to the lateral geniculate body.
Remnants of fetal hyaloid artery consists of a small tuft of fibrous tissues
Finger like projection extending anteriorly from the surface of ONH..IN anomaly
Cases
Affaret:retina to pretectal nucleus in mid brain
Centrifugal:
Ocular, orbital, canalicular, cranial
Pass from high iop to low pressure which is equivalevt to icp
Leave the bood supply of central retinal artey and is supplied by ophthalmic and posterior ciliary artery
Make a 90 degree turn and enter into confined space
Become mylinated just behind lamina cribrosa
Makes 200-300 holes through which the optic nerve passes
Fenistrations also shieldes high iop in the retro laminar region
High iop- posterior displacement of lc, increase in size of hole, higher translaminar ressure gradiant, astrocyte secrete nitric acid killing neuron cells
Ring is highly visible in temporal sector
Decrease in superior n inferior region hardly visible
Nasal sector
Non arteretic anterior ischaemic optic neuropathy
@choroidl crescent ,relative scotoma irregularities of RPE
B;SCLERAL crescent ,absolute scotoma atrophy of RPE.LARGE IN GLAUCOMA
VP..PRESSURE gradient varies due to different in pulse pressure betn
1ntraocular space N CSF
ITS sign is inc IOP
Incr .iop equals inc icp
Eye move freely without creating tension in the optic nerve
Also provide an awollowance of 9 mm of proptsis till optic nerve is fully stretched
SAS..SUBARACHNOID SPACE
anteriorerly
Papillomacular bundle
supplied by capillaries derived frm The Retinal Arterioles.
1)Peripapillary & Epipapillary arterioles of CRA origin
2)Precapillary branches from cilioretinal arteries when present
By Centripetal branches from Peripapillary Choroid with some contribution frm vessels of lamina cribrosa.
More often directly from branches of Short Posterior Ciliary Artery & centripetal fine branches from arterial circle of Zinn/ Haller
(This circle arises from the paraoptic branches of the SPCA & is usually embeded in the sclera around the ONH)
No supply from CRA in this region.
It is supplied by centifugal branches from central retinal artery and centrifugal branches from pial plexus formed by branches frm choroidal artery ,circle of zinn,central retinal artery & ophthalmic artery.
The Introrbital Region of optic nerve is supplied proximally by Pial Vascular Network & by neighboring branches of the ophthalmic artery.
Periaxial consists of 6 branches of internal carotid artery: Ophthalmic artery, Long posterior ciliary arteries,Short posterior ciliary arteries,Lacrimal artery , Central artery of retina before it enters ON , Circle of Zinn
The Introrbital Region of optic nerve is supplied proximally by Pial Vascular Network & by neighboring branches of the ophthalmic artery
Central collateral arteries which come off from CRA before it pierces the nerve
The nerve within the optic canal is supplied only by the periaxial system of vessels.The pial plexus in this part is fed mainly by branches from ophthalmic artery.
This part of the optic nerve is exclusively supplied frm periaxial system of vessels.
The pial plexus here is contributed by 4 sources;
-branches frm the ICA either directly or through the recurrent branch of anterior superior hypophyseal artery (supply the inf. Aspect of ON containing lower retinal fibres
-branches frm ant.cerebral artery (supply the sup. Aspect of ON containing upper retinal fibers)
-small recurrent branches frm Ophthalmic artery
-& the twigs frm ant. Communicating artery.
recurrent branch of anterior superior hypophyseal artery, small recurrent branches frm Ophthalmic artery
Aneurysm; swollen area
Chiefly by central retinal vein & to lesser extent via pial venous system,Both system drain into the ophthalmic venous system in the orbit & less commonly directly into cavernous sinus
As There is no barrier to diffusion across the highly fenestrated capillaries of the choroid
• Reduced visual acuity for distance and near is common, but is non-specific; acuity may be relatively preserved in some conditions.
• Relative afferent pupillary defect
Visual field defects, which vary with the underlying pathology, include diffuse depression of the central visualfield, central scotomas, centrocaecal scotomas, nerve fibre bundle and altitudinal
• Dyschromatopsia is impairment of colour vision, which in the context of optic nerve disease mainly affects red and green.
• Diminished light brightness sensitivity, often persisting after visual acuity returns to normal, for instance following the acute stage of optic neuritis. • Diminished contrast sensitivity
Optic disc edema refers to the ophthalmoscopic swelling of the optic disc with a concurrent increase in fluid within or surrounding the axons
Hyperimia is an excess of blood in a part due to local or general relaxation of arterioles
Optic disc pallor refers to an abnormal coloration of the optic disc as visualized by a fundoscopic examination. The disc normally has a pink hue and a central yellow depression. With optic disc pallor, an abnormal pale yellow color is evident
Atrophy; reduced in size and therefore strength
These are characterized by marked loss of vision or complete blindness on the affected side a/w abolition of direct light reflex on ipsilateral side & consensual on contralateral side .
Salient features of such lesions are;
The nasal nerve fibers form convex loop in termina part of opposite optic nerve , therefore ipsilateral blindness due to lesion of proximal most part of optic nerve is a/w contralateral field defect. (knee of wernicke)
It is common B/L but often asymmetric condition,in which optic nerve insert obliquely into the gloobe. Often a/w Myopia & Oblique Astigmatism. The superior pole of the optic disc may appear elevated with posterior displacement of the inferior nasal disc, or the disc can be horizontally tilted, resulting in an oval-appearing optic disc with an obliquely oriented long axis
Fig. (A) borderline tilted disc with thin inferotemporal neuroretinal rim (B) markedly tilted – and torsional – disc with situs inversus (temporal vessel deviate nasally before turning temporally) and associated inferonasal chorioretinal thinning (& myopic astigmatic error as findings)
○ A torsional (torsioned) disc is said to be present when its long axis is inclined at more than 15° from the vertical meridian, a line at 90° to a horizontal line connecting the foveola to the centre of the optic disc
Rare, usually unilateral condition. It represents a herniation of neuroectodermal tissue within the ON. Round or oval, gray or white depression in the optic disc ,usually in temporal part of disc; but occasionally central or elsewhere, disc itself is larger than in unaffected eye.
(Fig.B); The disc contains a greyish round or oval pit of variable size, usually temporal ; (Fig.C) Serous macular detachment ;develops in about half of eyes with non-central disc pits
Complication: Macular Retinoschisis & Subsequent Serous Retinal Detachment.
Drusens are intrapapillary refractile bodies,B/L, a/w RP.
A)Buried disc drusen (B) exposed disc drusen (C) drusen with secondary choroidal neovascularization;
Buried drusen. Particularly in childhood, drusen may be obscured beneath the disc surface, is a common cause of pseudopapilloedema. Elevated disc with Scalloped margin & no physiological cup, vascular tortuosity
• Exposed drusen. +nt in adults, Drusens r at or close to the disc surface appear as whitish pearl-like lesions of a range of sizes ;Complications (rare) include juxtapapillary choroidal neovascularization (Fig C), vitreous haemorrhage and vascular occlusions, particular anterior ischaemic optic neuropathy.
Also known as opaque nerve fibres Normally, the medullation of optic nerve proceeds from brain downwards to the eyeball and stops at the level of lamina cribrosa. Occasionally the process of myelination continues after birth for an invariable distance in the nerve fibre layer of retina beyond the optic disc.(whitish patch with feathery margins,usually +nt adjoining the disc margin). unilateral, VA is likely to be reduced if the central macula is involved; perimetry may show an absolute scotoma corresponding to the involved area of retina (a/w High myopia, Anisometropia, Amblyopia)
Fig Mild juxtapapillary myelinated nerve fibres (One or more whitish striated patches with feathery borders)
The embryonic fissure of the developing eye is located inferiorly and slightly nasally, and extends from the optic nerve to the margin of the pupil; a coloboma is a defect in one or more ocular structures due to the fissure’s incomplete closure. fully-developed coloboma typically presents inferonasally as a very large whitish bowl shaped excavation, which apparently looks as the optic disc.VA is often decreased; amblyopia and refractive error may be present ,a superior visual field defect may +nt
Fig A) Small disc coloboma; A focal, glistening white, bowl-shaped excavation, decentred inferiorly so that the inferior neuroretinal rim is thin or absent and normal disc tissue is confined to a superior wedge
Complications (rare) include serous retinal detachment, progressive neuroretinal rim thinning and choroidal neovascularization.
Unilateral , ○ A white tuft of glial tissue overlies the central portion and represents persistent hyaloid vascular remnants. ○ The blood vessels emerge from the rim of the excavation in a radial pattern like the spokes of a wheel. They are increased in number and it may be difficult to distinguish arteries from veins. VA may be normal or impaired to a variable extent.
FIG large disc with a funnel-shaped excavation surrounded by a ring-shaped chorioretinal disturbance;
Complication;serous retinal detachment
The hypoplastic optic nerve, unilateral or bilateral, carries a diminished number of nerve fibres. ); a foveola–disc centre distance of three or more times the disc diameter strongly suggests hypoplasia
Fig A. Mild hypoplasia consists simply of a smaller than normal disc B. The double-ring sign
Other Features: aniridia,microphthalmus,strbismus,nystagmus
Severe bilateral cases present with blindness in early infancy with roving eye movements or nystagmus. A relative afferent pupillary defect may be present; both pupils may have sluggish light responses in bilateral cases.Optic disc hypoplasia is associated with a wide variety of developmental midline brain defects; pituitary and hypothalamic deficits are common. Historically, the most frequent association has been considered to be ‘septo-optic dysplasia’ (de Morsier syndrome) – bilateral optic nerve hypoplasia, absent septum pellucidum, corpus callosum dysgenesis
Fig. The ocular fundus in Aicardi syndrome. (A) multiple bilateral depigmented chorioretinal lacunae clustered around a hypoplastic disc & (B) colobomatous or pigmented optic, other ocular features can include cataract and coloboma.
Megalopapilla is a typically bilateral condition in which both the horizontal and vertical disc diameters are 2.1 mm or more, or the disc area is greater than 2.5 mm2. Although the cup-to-disc ratio is greater than normal, the cup should retain its normal configuration with no evidence of focal neuroretinal rim loss. Although OCT may show peripapillary retinal nerve fibre layer thinning, macular ganglion cell complex imaging is typically normal.
Peripapillary staphyloma is a non-hereditary, usually unilateral condition in which a relatively normal disc sits at the base of a deep excavation whose walls, as well as the surrounding choroid and retinal pigment epithelium (RPE), show atrophic changes (Fig A). VA is markedly reduced and local retinal detachment may be present.
Optic nerve aplasia is an extremely rare condition in which the optic disc is absent or rudimentary and retinal vessels are absent or few in number and abnormal.
B/L, may be asymmetrical. ‘Disc swelling’ and ‘disc oedema’ are non-specific terms that include papilloedema but also a disc swollen from other causes. Idiopathic Intracranial HTN(Pseudotumour Cerebri) imp cause of raised intracranial press.
Unilateral with high ICP occurs in; Foster-Kennedy Syn
It develops as a result of stasis of axoplasm in prelaminar region,due to alteration in press.gradient across Lamina Cribrosa
Fig Blurring of disc margin,peripapillary haemorrhage,Hyperaemia
The ‘crescent sign’ (Fig. 19.19B) refers to an echolucent area in the anterior intraorbital nerve thought to represent increased separation of the nerve and its sheath. Fig Ultrasonography in papilloedema.(B) transverse B-scan showing crescent sign (arrowheads); Fig.Papilloedema. (A) Early; (B) acute established; (C) chronic; (D) atrophic – same eye as (C)
Early (Fig.A)
○ Mild disc hyperaemia with preservation of the optic cup.
○ Indistinct peripapillary retinal nerve striations and disc margins.
Established (acute – Fig. B)
○ Severe disc hyperaemia, moderate elevation with indistinct margins and absence of the physiological cup.
○ Venous engorgement, peripapillary flame haemorrhages and frequently cotton wool spots.
Circumferential retinal folds (Paton lines) may develop, especially temporally (see Fig. 19.7C). Chronic (Fig. 19.20C)
○ Disc elevation; cotton wool spots and haemorrhages are characteristically no longer present.
○ Optociliary shunts (see Ch. 13) and drusen-like crystalline deposits (corpora amylacea) may be present on the disc surface.
Atrophic (secondary optic atrophy – Fig. 19.20D)
○ VA is severely impaired.
○ The optic discs are grey–white, slightly elevated, with few crossing blood vessels and indistinct margins.
It includes inflammatory & demyelinating disorder of ON.Most common cause: Multiple sclerosis,Neuromyelitiss Optica(Devics Ds)
Diagnostic sign; Marcus Gunn Pupil(RAPD) VF: Central or centrocaecal scotoma
Fig Papillitis is character ized by hyperaemia and oedema of the optic disc, which may be associated with peripapillary flame-shaped haemorrhages
Pt may c/o mild dull eyeache,pain is usually aggravated by ocular movements esp.in upward & downward direction due to attachment of some fibres of sup. Rectus to dura mater.
Neuroretinitis is characterized by papillitis in a/w inflam. of the retinal nerve fibre layer and a macular star
Fig A) Papillitis a/w peripapillary and macular oedema
B) A macular star typically appears as disc swelling settles; the macular star resolves with a return to normal or near-normal visual acuity over 6–12 months.
Optical coherence tomography (OCT) demonstrates sub- and intraretinal fluid to a variable extent.
• Fluorescein angiography (FA) shows diffuse leakage from superficial disc vessels.
Pri.O.A=without any complicating process eg.Syphilitic O.A., Lebers ds,Retrobulbar Nuritis in MS produses Pri.O.A
Sec.O.A=occurs following any pathologic process eg papillitis in MS produces sec.O.A
Fig Optic atrophy. (A) Primary due to compression; Flat white disc with clearly delineated margins (B) secondary due to chronic papilloedema , prominent Paton lines Slightly or moderately raised white or greyish disc with poorly delineated margins due to gliosis Peripapillary circumferential retinochoroidal folds, especially temporal to the disc (Paton lines), sheathing of arterioles and venous tortuosity may be present.
(AAION) is caused by giant cell arteritis (GCA) Cilioretinal artery occlusion may be combined with AAION
(NAION) is caused by occlusion of the short posterior ciliary arteries resulting in partial or total infarction of the ONH. Predispositions include structural crowding of the optic nerve head so that the physiological cup is either very small or absent, hypertension (very common), diabetes mellitus, hyperlipidaemia, collagen vascular disease,;
FIG A) A ‘chalky white’ oedematous disc in arteritic ischaemic optic neuropathy B) Diffuse or sectoral hyperaemic disc swelling, often a/w a few peripapillary splinter haemorrhages,
(PION) is much less common than anterior variety. It is caused by ischaemia of retrolaminar portion of ON.