Disorders of the Posterior Segment OS 212

Bryan Vincent Q. Mesina, MD, DPBO Exam 01

22 October 2019 Trans 06

OUTLINE II. DISORDERS OF THE VITREOUS

I. Introduction V. Disorders of the Optic Nerve A. ANATOMY OF THE VITREOUS
II. Disorders of the Vitreous A. Anatomy of the Optic • Vitreous body
A. Anatomy of the Vitreous Nerve → Clear, avascular body that occupies 80% of the volume and
B. Posterior Vitreous B. Papilledema weight of the eye
Detachment C. Optic Neuritis → Clear matrix comprised by
C. Vitreous Hemorrhage D. Anterior Ischemic Optic
▪ 99% liquid – water
III. Disorders of the Choroid Neuropathy (AION)
E. Toxic / Nutritional Optic
▪ 1% solid – collagen and hyaluronic acid
A. Anatomy of the Choroid → Firmly attached to the:
B. Central Serous Neuropathy (TON/NON)
F. Post-traumatic Optic ▪ Vitreous base – 2 mm anterior and 3 mm posterior to the
Chorioretinopathy (CSCR)
Neuropathy (PTON) ora serrata
IV. Disorders of the Retina
A. Anatomy of the Retina G. Compressive Optic ▪ Posterior lens capsule
B. Diabetic Retinopathy Neuropathy ▪ Large retinal blood vessels
C. Hypertensive Retinopathy H. Optic Nerve Hypoplasia ▪ Macula
D. Central Retinal Artery I. Optic Pit ▪ Optic nerve
Occlusion (CRAO) J. Optic Nerve Coloboma → Any tractions placed on these firm attachments can cause
E. Central Retinal Vein K. Morning Glory Disc detachment
Occlusion (CRVO) Anomaly • Liquefaction of the vitreous gel (syneresis) begins as early as
F. Age-Related Macular References 45-50 years of age (normal physiologic event)
Degeneration (ARMD) Summary → Destabilization of the vitreous gel from enzymatic and non-
G. Retinitis Pigmentosa enzymatic cross-linking of collagen fibers, free radical
H. Retinal Detachment change or decrease in the network density of collagen fibers
I. Cytomegalovirus (CMV) → Can lead to various vitreo-retinal interface problems (i.e.
Retinitis retinal detachment, macular hole, epiretinal membrane,
J. Ocular Toxoplasmosis posterior vitreous detachment, vitreous hemorrhage)
K. Retinoblastoma
B. POSTERIOR VITREOUS DETACHMENT (PVD)
Note: Doc’s tips for studying: Focus on pathology. Note overlaps or • Secondary to vitreous syneresis
repeated diseases present in this lecture as well as in other lectures. • Frequently seen in myopics, elderly, aphakic patients (without
Don’t focus too much on the treatment. His lecture was based on the
lenses), post-trauma or inflammation, post-operative
SIM, but he didn’t include some topics.
• Present with floaters (from vitreous opacities as the vitreous
cortex separates from the glial disc margin) and/or
I. INTRODUCTION flashes/photopsias (from physical vitreo-retinal traction on the
retina)
→ Flashes are not caused by light, but by the physical traction
that stimulates the photoreceptors which make you
seemingly see flashes of light

Figure 2. Presence of floaters (left) and Weiss ring (right & left) in
Figure 1. Anatomy of the eye. PVD.

• Weiss ring
• The posterior segment of the eye involves structures posterior to
→ Round or oval-shaped tissue arising from the detachment of
the iris:
→ Vitreous body the vitreous body from the glial optic disc margin
→ Diagnosed clinically via funduscopy
→ Choroid
→ Retina • Treatment is mainly assurance, observation and regular follow-
→ Optic nerve up to look for retinal tears
→ Can be associated with 15% of acute symptomatic PVD and
• Signs and symptoms of pathologies of the posterior segment
may include: 50-70% of PVD with concomitant vitreous hemorrhage
→ If the floaters increase in size and number or if you have
→ Blurring of vision
▪ Sudden, insidious or gradual sudden-onset blurring of vision, you have to consult an
ophthalmologist immediately because it might mean there is
→ Visual field defect
hemorrhage or retinal detachment involved
→ Metamorphopsia, micropsia, macropsia
→ Flashes/photopsia, floaters
→ Eye pain

TG B19: Serrano, Sia, Sianoya [Salvan] 1 of 22

OS 212: Disorders of the Posterior Segment
C. VITREOUS HEMORRHAGE
• Presence of blood within the vitreous body
• Patients typically complain of floaters associated with sudden-
onset blurring of vision (i.e. dimming, multiple dark
particles/streaks, reddening)
• Bleeding is typically from a retinal vessel
Figure 3. Dense vitreous hemorrhage (left) and sub-hyaloid hemorrhage, a
boat-shaped hemorrhage in between the posterior hyaloid phase and
internal limiting membrane of the retina, creating a potential space for the
blood to collect and forming the boat-shaped configuration (right).
• Arises from a myriad of causes:
→ Avulsed retinal vessel from PVD
▪ The vitreous is firmly attached to the retinal vessel, so
any traction can pull or tear the retinal vessel apart
→ Retinal tear/break cutting through a retinal vessel
→ Neovascularizations (i.e. diabetic retinopathy, pars planitis,
Eals disease)
→ Trauma
→ Tumors
Figure 4. Ocular B-Scan Ultrasound for normal retinal attachment (top) and
for retinal detachment (bottom)
• Diagnosed clinically via funduscopy
→ Ocular B-Scan Ultrasound may be performed to evaluate the
status of the rest of the posterior segment (i.e. check for
retinal detachment, tumors, etc.)
• Management is directed at determining the etiology of the
hemorrhage and clearing the blood
→ Generally, if there are no other pathologies seen within the
posterior segment, the hemorrhage may be observed for 4-6
months and allowed to resorb on its own
▪ Or you can advise patient to do high back rest for 1-2
weeks or give tranexamic acid for 2-5 days
→ In the presence of retinal detachment, or a tumor, or if the
hemorrhage is non-clearing and dense, vitreo-retinal surgery
via Pars Plana Vitrectomy with treatment of the primary
etiology is advised
Exam 01 - Trans 06
III. DISORDERS OF THE CHOROID
A. ANATOMY OF THE CHOROID
Figure 5. Histologic diagram of choroid.
• In Figure 1, the choroid is located between the retina and sclera
(the red layer of tissue)
• Etymology: similar to fetal chorion derived from its vascularity
• Spongy mass of vessels that extends from the optic nerve to the
ora serrata
• In Figure 5, the choroid is thinnest anteriorly (0.1 mm) and
thickest posteriorly (0.25 mm)
• Main function is to provide nutrients to the outer parts of the
retina
• Blood enters the choroid through the short and long posterior
ciliary arteries
• Highest blood flow of any tissue in the body (Doc emphasized
this)
→ Rapid flow acts as a heat-sink to remove thermal energy
from light absorption
→ Oxygen content of choroidal venous blood is only 2-3%
lower than that of its arterial counterpart
B. CENTRAL SEROUS CHORIORETINOPATHY (CSCR)
• Idiopathic serous neurosensory retinal detachment
secondary to an altered barrier and deficient pump function at
the retinal pigment epithelium (RPE)
→ Subretinal fluid accumulation
▪ RPE functions to eliminate fluid from the retina by its
pumping action, and if it’s deficient, there is fluid
accumulation
• Visual disturbances include metamorphopsia, micropsia,
depressed color vision, paracentral scotoma
→ Asymptomatic if the macula is unaffected
• Patient profile:
→ Middle-aged male
→ Type A personality, stressed individuals, hypochondriacs
→ Hysterical/neurotic, on psychiatric medications or steroids
→ Cushing’s syndrome or SLE
• Diagnostics:
Figure 6. Diagnostic findings in CSCR, via fundoscopy (leftmost),
fluorescence angiography (middle), optical coherence tomography (OCT,
rightmost).
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• Treatment:
→ Observation for unilateral cases and first episodes • Three (3) main cell-types for neuronal transmission:
▪ Spontaneous resorption is noted mainly within 3-4 → Photoreceptors
months, but can last up to a year → Bipolar cells
→ Macular laser photocoagulation only performed in: → Ganglion cells
▪ Persistent disease beyond 3-4 months ▪ Axons of the ganglion cells form the nerve fiber layer
▪ Recurrent cases with visual deficit from a previous (Doc asked this in class)
episode • Structure cells:
▪ Chronic signs (i.e. RPE abnormalities) → Muller cells
▪ Patients that need prompt restoration of vision or → Horizontal cells
stereopsis → Amacrine cells
• Blood supply:
IV. DISORDERS OF THE RETINA → Inner two-thirds (inner nuclear layer inwards): central retinal
A. ANATOMY OF THE RETINA artery
→ Outer one-third (outer plexiform layer outwards):
choriocapillaris
• Blood-Retinal Barriers (BRB)
→ Inner BRB
▪ Formed by the tight endothelial cell junctions of the retinal
capillaries
→ Outer BRB
▪ Formed by the tight intercellular junctions between RPE
cells (zonula occludens)
B. DIABETIC RETINOPATHY
• Complication of Diabetes Mellitus
• Manifests mainly as vascular changes in the retina
→ Structural damage to capillary endothelium
▪ Loss of mural pericytes and basement membrane
thickening
→ Retinal hemorrhages
→ Cotton-wool spots and hard exudates
→ Neovascularization

Figure 9. Cystoid macular edema (leftmost) and neovascularizations

Figure 7. Ten-layered structure of the retina.
Note: Doc said he assumes the class already memorized the ten
layers “for your exam. Kaya niyo ‘yan.”
• Ten-layered structure that contains a 3-neuron relay system
that transmits photochemical information through the retina
(from the outside, in), to the optic nerve, then to the occipital
cortex
(middle and rightmost).
• Vision loss mainly comes secondary to:
→ Macular edema
▪ Can occur at ANY STAGE (mild, moderate or severe
stage) of diabetic retinopathy
→ Macular ischemia
→ Neovascularization sequelae – hemorrhage, tractional retinal
detachment, neovascular glaucoma
• Intensive blood sugar control has been proven to be a key
modifiable factor to reduce risk, delay the onset of or slow down
the progression of diabetic retinopathy
• Blood pressure control has been shown in some studies to
slow the progression of diabetic retinopathy
• Screening:
→ Type 2 DM
▪ Examine upon diagnosis, then annually
→ Type 1 DM
▪ Examine 3-5 years after diagnosis, then annually
→ Pregnant patients
▪ Regardless of type, examine pre-conception or first
trimester, then every 1-3 months if the stage is severe
• Treatment
→ At the initial stages, observation and regular monitoring of
the retinopathy are sufficient

Figure 8. Three main cell-types for neuronal transmission from the retina
(photoreceptors, bipolar cells, ganglion cells).

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NON-PROLIFERATIVE DIABETIC RETINOPATHY • In cases of PDR, the following treatment maybe done:
(NPDR) → Laser treatment
▪ Panretinal photocoagulation (to destroy ischemic retina,
Table 1. Clinical findings for NDPR stages. lowering oxygen demand/consumption and increasing
Stage Clinical Findings oxygen tension via the scars formed by the laser marks
None No retinopathy on the retina and inducing neovascular regression)
Mild Microaneurysms only → Intravitreal anti-VEGF injections (to decrease the load of
Moderate More than just microaneurysms (i.e. PLUS VEGF inside the eye)
hemorrhage, cotton-wool spot, hard exudate, → Vitreo-retinal surgery
venous beading, intraretinal microvascular ▪ Vitreo-retinal surgery – to evacuate hemorrhage, sever
abnormalities (IRMA) BUT less than severe NDPR traction membranes, re-attach the retina
Severe Any of the following:
1. >20 intraretinal hemorrhages in each of the 4
quadrants
2. Definite venous beading in at least 2 quadrants
3. Prominent IRMA in at least 1 quadrant

AND no signs of proliferative retinopathy

a.k.a “4-2-1 rule of Diabetic retinopathy”

Figure 12. Laser-treated retina in PDR (about 1,200 laser marks per eye).

C. HYPERTENSIVE RETINOPATHY
• Retinal manifestation of systemic hypertension from disordered
autoregulation and breakdown of the blood-retinal barrier (i.e.
fibrinoid necrosis)
• 3 Main Pathologies:
→ Vascular constriction - generalized or focal arteriolar
narrowing
→ Leakage - abnormal vessel permeability
▪ manifests as flame- shaped hemorrhages, retinal edema,
hard exudates, optic disc edema
→ Arteriosclerosis - intimal layer hyalinization, medial layer
hypertrophy & endothelial hyperplasia
▪ presents as focal narrowing and straightening of the retinal
arterial walls
Figure 10. Clinical findings in NDPR stages. • May lead to structural vascular changes, ischemia and its
various sequelae
PROLIFERATIVE DIABETIC RETINOPATHY (NPDR) → Neovascualrization
• Neovascularization eventually occurs due to → Retinal Vaso-occlusive diseases
→ Angiogenic stimulus (deprived of oxygen supply) + • Mainstay of treatment is strict and adequate control of blood
vasoproliferative factors (VEGF) + viable source of pressure
endothelial cells
• The poorly structured vessels cross the internal limiting Table 2. Hypersensitive Retinopathy Classification. [SILM]
membrane and use the posterior hyaloid face as a scaffold Keith-Wagner-Barker Scheie
→ Any physical traction within the vitreo-retinal interface may Stage Clinical Findings Stage Clinical Findings
cause rupture of the fragile vessels leading to hemorrhage Slight or modest
I I Broadening of the
• Stage: Proliferative narrowing of retinal arteriolar light
→ Clinical Findings: One or more of the following: arterioles reflex; Minimal AV
▪ Neovascularization (AV ratio > 1:2) crossing changes
▪ Vitreous/Pre-retinal hemorrhage
II Modest to severe II Obvious broadening of
narrowing of retinal the arteriolar light
arterioles reflex; Moderate AV
(AV ratio < 1:2 or crossing changes
AV nicking)
III Stage II + Cotton- III Copper-wire arterioles;
wool spots or Marked AV crossing
flame-shaped changes
hemorrhages
IV Stage III + Optic IV Silver-wire arterioles;
Disc Edema Severe AV crossing
Figure 11. Clinical findings in PDR. changes
*Keith-Wagner-Barker is more used in the clinics

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Figure 13. (a) Arteriolar Narrowing – compare the width of the arteries to
the vein (b) Copper-wiring manifested by the yellowish tinge in the vessel
lumen.
Figure 14. Silver-wiring manifested by the sclerosed vessels.
Figure 15. (a) GRADE III - note the cotton-wool spots and flame-shaped
hemorrhages (b) GRADE IV – with optic disc edema and hard exudates.
• Vascular Occlusive Diseases caused by Hypertensive
Retinopathy
→ Central Retinal Artery Occlusion (CRAO)
→ Central Retinal Vein Occlusion (CRVO)
D. CENTRAL RETINAL ARTERY OCCLUSION (CRAO)
• One of only two “true” Ophthalmic emergencies (very well-
emphasized)
• Sudden, painless, unilateral, severe loss of vision (i.e.
counting fingers to light perception BUT NEVER NO LIGHT
PERCEPTION (NLP) – this is usually presented by ophthalmic
artery occlusion rather than CRAO because you still have supply
for the outer 1/3 of your retina) [Mesina, 2019]
→ Patients sometimes report a prodrome of transient vision
loss several times over a span of days to weeks prior
• Usually related to systemic vascular diseases
→ Hypertension, Arteriosclerosis, Collagen Vascular Disease,
Hematologic disorders, Cardiac Valvular Disease
• Can sometimes be caused by prolonged pressure on the globe
or massive retrobulbar hemorrhage after trauma
→ Massage beds have “donuts” pillow for the face that causes
prolonged pressure on the eyes after massage of surgery
causing CRAO
• Thrombosis at the level of the lamina cribrosa
→ Lamina cribrosa – scaffold of the axons of your optic nerve
• Retina is very pale due to lack of blood flow and edema
→ The darker macula becomes more prominent as the
choroidal vasculature under the foveola is intact = Cherry
Red Spot (or Chico Brown Spot)
Exam 01 - Trans 06
Figure 16. (a) Cherry Red Spot - Retina is edematous, macula with an
intact choroidal vasculature underneath (b) CRAO - No filling of your retinal
vasculature, only filling of the choroidal vasculature – meaning the central
retinal artery is really blocked (non-perfusion of retinal arteries).
Figure 17. Dyed here is the set of your cilioretinal arteries. It’s a normal
variant in 30% of the normal population. So when their retinal artery gets
occluded, they still have better vision than those without ciliary arteries due
to the collateral vessels they have.
• Infarction of the inner two-thirds of the retina, thus prognosis for
visual recovery is very poor
→ 66% of patients have a VA of 20/400 or worse
→ Some reports have pegged the “golden period” for
treatment for salvage of vision at 90 minutes from onset
(before irreversible damages occur)
• Some patients may retain better VA due to patency of the
Cilioretinal Artery
• A branch of the Posterior Ciliary Artery that directly supplies an
inner portion of the retina - seen in only 30% of the population
• Treatment:
→ Immediate lowering of intraocular pressure to improve
perfusion by reducing intraocular resistance to blood flow
▪ Ocular Massage - sustained pressure for 10-15 seconds,
then release (rationale: dislodge and release the thrombus
stuck in the retinal artery)
▪ Brown bag breathing to induce hypercarbia (hypercarbia
lowers your IOP)
▪ Anterior Chamber paracentesis (using a needle to drain
the fluid in your eye to lower your IOP)
▪ Acetazolamide & other IOP lowering agents
▪ Clot-lysing agents
E. CENTRAL RETINAL VEIN OCCLUSION (CRVO)
• Sudden, painless, blurring of vision
• Thrombosis at the level of or posterior to the lamina
cribrosa
• Usually related to systemic vascular diseases in the elderly
→ Hypertension, Diabetes Mellitus, Arteriosclerosis
• Patients with Open Angle Glaucoma may also be predisposed
to the development of this disease
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Table 3. Two Types of Central Retinal Vein Occlusion.

Ischemic
• More severe loss of vision
• (+) RAPD
• Fundus is covered with
multiple splinter & flame-
shaped hemorrhages
• (+) Cotton-wool spots
• Retinal veins are tortuous
and dilated
• (+) Optic Disc Edema
• VA usually <20/200
• 60% risk of
neovascularization
Non-Ischemic
• A.k.a. Venous Stasis
Retinopathy
• Less severe loss of vision
• Usually (-) RAPD
• Fewer retinal hemorrhages
• Usually (-) Cotton-wool spots
• Retinal veins are tortuous and
dilated
• Usually (-) Optic Disc Edema
• VA better than 20/200
• Much better prognosis
PATHOLOGICAL CHANGES IN THE MACULA SEEN IN
ARMD
Non-neovascular (Dry-type)
• More common, less severe visual loss
• Gradual, painless blurring of vision (central)
• Classified into: early, intermediate, advanced [not for LU IV Level]
• Hallmark: DRUSEN formation
→ Look like hard exudates
→ Bruch’s membrane thickening from deposition of basal
debris made of granular lipid-rich material or phospholipid
• Atrophy of the Retinal Pigment Epithelium

Figure 19. Hard Drusen – looks like hard exudates, well defined. More
related to dry-type ARMD.

Figure 18. (a) ISCHEMIC – multiple hemorrhages of flame-shaped

Figure 20. Soft Drusen – looks like soft exudates but yellow with fluffy
throughout the entire posterior pole, with macular and disc edema, and
margins, and are larger than hard drusen. Predispose patients to develop
no filling over your veins (b) NON-ISCHEMIC – less hemorrhages, no
the wet-type ARMD.
disc edema, better VA, some filling of your veins.

• Treatment:
→ Non-ischemic: no definitive ocular treatment; but primary
cause must be ascertained and addressed
→ Ischemic:
▪ Panretinal Photocoagulation (laser therapy) if with at least
2 clock hours of Iris neovascularization
▪ Intravitreal steroid or anti-VEGF injections (especially for
macular edema)
F. AGE-RELATED MACULAR DEGENERATION (ARMD) Figure 21. Optical Coherence Tomography (OCT) scans of dry-type ARMD
• One of the major causes of central visual loss in the world in – mount-like elevations
people over 50 years of age
→ Incidence rises sharply after the age of 75 • Endpoint of untreated non-neovascular ARMD: Geographic
• Risk factors: Atrophy of the RPE
→ Definite: age
→ Implicated: smoking (debated to be elevated as a definite
risk factor of ARMD), cardiovascular disease, ethnicity,
undue exposure to UV rays, lack of Vitamin C, other factors
that increase oxidative stress
• 2 types of changes in the macula:
→ Normal Age-Related Changes
→ Pathologic Age-Related Changes
• 2 types of Pathologic Age-Related Macular Degeneration:
→ Non-neovascular (Dry-type)
→ Neovascular (Wet-type)

NORMAL AGE-RELATED CHANGES ON THE MACULA

• Accumulation of lipofuscin granules, residual bodies Figure 22. Dry and atrophic RPE.
• Loss of photoreceptor density and distribution, melanin granules
and involution of the choriocapillaris

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• Treatment:
→ General:
▪ UV Avoidance
▪ Increase in green-leafy vegetable intake
▪ Smoking cessation
▪ Amsler grid monitoring
→ Non-neovascular Specific: Age-related Eye Disease Study
(AREDS) – for intermediate or advanced unilateral cases of
ARMD
▪ Subset 1: 500mg Vitamin C + 400 IU Vitamin E + 80mg
Zinc Oxide + 15mg Beta carotene + 2mg Cupric oxide
NOTE: we do not give Subset 1 for smokers because Beta
carotene increases the risk for lung cancer
▪ Subset 2 (smokers): Lutein + Zeaxanthin + Omega 3
Long-chain PUFA substitute
Neovascular (Wet-type)
• VA is about 20/200 or less
• Sudden-onset blurring of vision, metamorphopsia
• Hallmark: CHOROIDAL NEOVASCULARIZATION
→ Buds of neovascular tissue from the choriocapillaris
perforate the outer Bruch’s membrane
• Patients present with bilateral progressive visual field loss and
abnormal electroretinogram measurements
→ Notable symptoms include nyctalopia (“night blindness”)
and central blurring of vision
• Several patterns of inheritance:
→ Autosomal recessive*
→ X-linked recessive*
→ Autosomal dominant**
* Have the earliest onset and worst prognosis
** Patients with the dominant form may be symptom free until
middle age
• The defect in most patients is in the gene that encodes for
rhodopsin
• RETINITS PIGMENTOSA TRIAD:
→ Waxy Optic Disc pallor
→ Bony spicule-like changes (secondary to macrophage
apoptosis) or RPE atrophy (actual death of photoreceptors
and migration of RPE into your retina [Mesina, 2019])
→ Attenuation (or narrowing) of the retinal vessels

Figure 23. Choroidal neovascularization – a group of vessels that pierce

through your RPE and has grown into your neurosensory retina. These
vessels are very fragile and they bleed. They create hemorrhages in your
neurosensory retina, and create scars and fibrosis.

• Endpoint of untreated neovascular ARMD: Extensive retinal

scarring and damage from recurrent subretinal hemorrhage

Figure 25. These 4 images are in the PPT but were not elaborated by Dr.
Mesina.
• Other features:
→ Cystoid Macular Edema
→ Posterior Subcapsular Cataract formation
→ Vitreous Cells
• Treatment:
→ No known treatment
→ Cataract extraction may help improve vision
Figure 24. subretinal fibrosis with fresh hemorrhages, with relatively older → Daily supplementation with Vitamin A 15,000 IU or 25,000
hemorrhages that present as serous exudates. IU twice a day may help slow down deterioration of ERG
changes, with no known demonstrable effect on vision
• Treatment: → UV protection is debatable
→ General:
▪ UV Avoidance H. RETINAL DETACHMENT
▪ Increase in green-leafy vegetable intake • Separation of the inner layers of the retina from
▪ Smoking cessation theunderlying retinal pigment epithelium (RPE) & choroid,
▪ Amsler grid monitoring often with accumulation of fluid in the subretinal space
→ Neovascular Specific:
▪ Photodynamic Therapy with Verteporfin (light therapy for NOTE: when referring to neurosensory retina, it’s from the internal
the macula) limiting membrane to the photoreceptor layers
▪ Itravitreal Anti-VEDF injections (Ranibizumab,
Bevacizumab, Aflibercept) Non-neovascular (Dry-type) • Symptoms:
→ Flashes/photopsias
G. RETINITIS PIGMENTOSA → Shower of floaters that resemble spots, bugs or cobwebs
• Panretinal dystrophy that belongs to a group of → “Wavy”/“watery” vision
heredodegenerative diseases (aka tapetoretinal diseases) that → Veil or curtain obstruction vision
involve photoreceptor & pigment epithelial dysfunction → Sudden decrease in vision
• Progressive degeneration of the rods & cones with migration of • 2 Types:
the pigment epithelial cells into the retina → Rhegmatogenous - with a retinal tear, break or hole

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→ Non-Rhegmatogenous - no tear, break or hole • Treatment:

▪ Tractional → Retinal Detachment Surgery (principles):
▪ Exudative ▪ Find all breaks
▪ Create a chorioretinal irritation around each break to seal
RHEGMATOGENOUS it
▪ Bring the retina and choroid into contact for a sufficient
time to produce chorioretinal adhesion
→ Treatment:
▪ Pars Plana Vitrectomy (bore three holes into the pars plana
and retaching the retina)
▪ Pneumatic Retinopexy (using of C3F8 gas to reattach
retina in cases where the tear is small and there is only one
hole)
▪ Focal Laser Therapy (if the retinal detachment is still small
and you can barricade the region of retinal detachment)
▪ Scleral Band & Buckling (putting a silicone band around
Figure 26. Rhegmatogenous Retinal Tear
the eye muscles to decrease the vector force of your retinal
detachment and bring your choroid and your neurosensory
• With a retinal tear, break or hole usually at the periphery retina into adhesion)
• Most common
• “rhegma” – Greek word for tear, rupture, or breach
• Liquefied vitreous passes through the retinal break to reach the
potential space between the RPE & Neurosensory Retina
• Liquified retina fills the space and keeps the space between your
retina and your choroid
• IOP is usually lower in the affected eye (because your RPE cells
can absorb liquified vitreous thereby decreasing the volume of
your vitreous humor in your eye, lowering you IOP)
• Detached retina looks corrugated and undulates with movement
• Configuration is more convex toward the front of the eye
• Shifting of fluid from supine to upright is UNCOMMON

Figure 29. Scleral Band and Buckling

NON-RHEGMATOGENOUS
• 2 Types:
Figure 27. Horse-shoe tear (left), Operculated (with a flap covering) Retinal → Tractional
Tear (right) → Exudative
• Risk factors:
→ Aphakia/Pseudophakia (without lens, or artificial intraocular Tractional
lens implant) • 2nd most common type
→ Myopia (usually of -6 and above) • Fibrous membranes on the vitreous & surface of the retina
→ Family History of Retinal Detachment contract and elevate the neurosensory retinal layers
→ Detachment in the fellow eye → Made up of fibroblasts, glial cells, RPE cells with contractile
→ Ocular Trauma properties
→ Lattice Degeneration • Retina has a smooth surface and is immobile
• Configuration of detachment is more concave toward the front
of the eye
• May eventually cause rhegmatogenous RD
• Common causes:
→ Diabetic Retinopathy
→ Proliferative Vitreoretinopathy
→ Ischemic Retinopathy
→ Penetrating Trauma
→ Retinopathy of Prematurity
• Treatment:
→ Sever the traction membranes and reattach the retina via
surgery (via laser or putting oil or gas inside your eye)
Figure 28. Lattice degeneration, notice the black and white streaks that
resemble atrophic holes at the periphery of the retina

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I. CYTOMEGALOVIRUS (CMV) RETINITIS

• Most common cause of congenital viral infections
• Most common ophthalmic manifestation in HIV/AIDS
→ Especially in patient with CD4+ counts of <50 cells/µL
• Full-thickness hemorrhagic retinal necrosis with yellow-
white cloudy retinal lesions or infiltrates
→ Minimal to no vitritis
FULMINANT
• Fulminant - large areas of hemorrhage, typically in the posterior
pole, following the distribution of the RNFL

Figure 30. These 3 images are in the PPT but were not elaborated by Dr.
Mesina.

Exudative
• Accumulation/leakage of fluid into the subretinal space from Figure 33. Pizza Pie Look-alike. Cheese – infiltrates. Darker, more defined
damage of the retinal blood vessels or RPE (without any hole or ones (Pepperoni). Softer red ones (tomato sauce).
tear)
• Caused primarily by diseases of the RPE & Choroid GRANULAR / INDOLENT
• Convex configuration • Granular/Indolent - typically in the retinal periphery; little or no
• Smooth appearance hemorrhages or necrosis
• (+) Shifting fluid from supine to upright (emphasized)
→ When the patient is supine, you will see an almost complete
detachment of the retina in the fundus
→ When the patient sit up, the retinal detachment is less seen
(almost ½ is only seen)
• May be associated with:
→ Systemic vascular disease (i.e. malignant hypertension,
eclampsia)
→ Neoplasia (i.e. metastasis)
→ Ocular Inflammatory disease (i.e. Uveitis)
• Treatment:
→ Manage the underlying cause
→ Usually not surgical
→ Usually oral or systemic steroid therapy

Figure 34. Granular Type CMV Retinitis.

FROSTED BRANCH ANGITIS

• Frosted Branch Angiitis – perivascular type

Figure 31. Retinal detachment where a thick nerve in the center is visible.

Figure 35. “Snow lining your vessels”.

TREATMENT
• Treatment:
→ Oral Valganciclovir (900mg BID for 3 weeks as induction,
then 450mg BID as maintenance)
→ Intravenous Ganciclovir (500mg IV q12 for 2-3 weeks as
induction, then 5 mkday infusions as maintenance)
Figure 32. Retinal detachment with no visible nerve. It’s just a bullous → Foscarnet, Cidofovir, Intravitreal Ganciclovir
configuration of your retinal detachment.

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

J. OCULAR TOXOPLASMOSIS ▪ Sulfadiazine (2-4g/day LD; then 4g/day maintenance)

• Most common cause of infectious retinochoroiditis in adults & ▪ Prednisone (0.5-1mkday)
children ▪ Clindamycin (300mg 4x/day)
• Definitive hosts: Cats ▪ *Folinic Acid - prevent leukopenia and thrombocytopenia
• Intermediate hosts: Humans, pigs, lamb, fowl → Alternatives:
• Modes of transmission: Oocyst (soil), Bradyzoite (Tissue cyst), ▪ Trimethoprim-Sulfamethoxazole 800mg/160mg tab BID
Tachyzoite for 6 weeks
→ Ingestion of undercooked, infected meat, contaminated ▪ Atovaquone, Azithromycin
water, fruit, vegetables, unpasteurized millk, inadvertent ▪ Spiramycin - in pregnant patients
ingestion of cat feces/ litter K. RETINOBLASTOMA
→ Transplacental - greatest risk at 3rd trimester
• Most common intraocular malignancy of childhood
→ Inoculation through a break in the skin esp. through the soil
• No known sexual or racial predilection
→ Blood Transfusion or Organ transplantation
• Average age of onset: 18 months
• Ocular Manifestations:
→ Rarely seen beyond 6 years of age
→ Variable anterior chamber reaction
→ Retinochoroiditis (predilection for posterior pole & macula)
→ “Headlight in the fog” (Vitritis)
→ Focal white retinitis adjacent to a pigmented chorioretinal
scar
→ Kyrieleis arteritis - vasculitis in the vicinity of active lesions
(also found in Syphilis)

Figure 39. Retinoblastoma.

• Most common presenting sign is leukocoria (“white pupil”) or

cat’s eye reflex (“matang pusa”)
→ Can also present with strabismus, eye redness, hypopyon,
buphthalmos (enlargement of the globe), proptosis (in later
stages)
Figure 36. Focal white retinitis adjacent to a pigmented chorioretinal scar.

Figure 40. Leukocoria.

• Differentials for Leukocoria (emphasis for clinical rotations)

→ Cataract
→ Persistent Fetal Vasculature
▪ previously known as Persistent Hyperplastic Primary
Vitreous
Figure 37. “Headlight in the fog” – Fog is the hazy media.
▪ Persistent fetal vasculature (Hyaloid artery remnant). In the
womb, the vitreous is supplied by the hyaloid artery which
regresses 8-9 mos. after delivery. But in these patients, it
remains patent that creates a whitish reflex/ it’s a vessel
that runs from your optic nerve to your posterior lens
capsule.
→ Coats’ Disease (massive retinal exudation)
→ Retinopathy of Prematurity
→ Retinal Detachment
→ Endophthalmitis
→ Ocular Toxocariasis
→ Ocular Tuberculosis
Figure 38. Kyrieleis retinitis. Vessels are studded with white lesions
(vasculitis). NOT pathognomonic for ocular toxoplasmosis.

• Systemic Manifestations:
→ Hydrocephalus
→ Intracranial Calcifications
→ 3-fold increase in Toxoplasma IgG titers or (+) IgM after 5
days of life
• Triad in Congenital Toxoplasmosis:
→ Hydrocephalus
→ Intracranial Calcification
→ Chorioretinitis
• Treatment:
→ Quadruple Therapy
▪ Pyrimethamine (50-100mg/day LD; then 25-50mg/day
maintenance) Figure 41. Cataract Hyaloid.

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

• Caused by mutations on the RB1 gene on the long arm of • Prognosis & Follow-up:
Chromosome 13 (13q14) → Tumors with extraocular extension (especially with large
→ Tumor-suppressor gene whose presence (even of a single tumors involving the orbit) have higher rates of metastasis
allele) protects against the development of the tumor and lead to worse prognosis
→ Knudson’s Two Hit Hypothesis: → Patients must be closely followed-up for signs of
▪ One single retinal cell suffers a mutation on one allele of recurrence or development of new tumors
the RB gene, then after some time suffers another → Genetic counselling is necessary for the parents
mutation on the remaining allele
▪ The loss of the tumor suppression on both alleles allows V. DISORDERS OF THE OPTIC NERVE
that particular cell to multiply uncontrollably A. ANATOMY OF THE OPTIC NERVE
• Two types of mutation that occur in your retinoblastoma: • Carries 1.0-1.2 million different fibers originating from the retinal
(1) Sporadic mutations ganglion cells
→ Tumor development occurs later (about 24 months of age) • Traverses the sclera through the 200-300 channels of the lamina
→ Unilateral cribrosa (main support)
→ Takes longer because two alleles should be targeted • Axonal transport is high-oxygen requiring and is sensitive to
(2) Inherited mutations ischemic, inflammatory and compressive processes
→ Tumor development occurs earlier (about 12 months of age) • 50 cm long from the globe to the optic chiasm
→ Bilateral with multiple tumors • Anatomical Subdivisions:
→ Behaves as an autosomal dominant trait with marked → Intraocular
penetrance → Intraorbital
• Evaluation → Intracanalicular
→ Thorough and extensive medical, family, birth and maternal → Intracranial (optic chiasm)
history
→ Complete 5-point ophthalmologic examination
→ Dilated funduscopy
→ Systemic & Neurologic physical examination to check for
signs of metastasis (especially in advanced stages)
→ Ocular B-Scan Ultrasound
▪ Especially in cases where there is no view of the posterior
segment
→ Cranial CT-Scan
▪ Most useful ancillary test
▪ Check for calcifications & extension into the brain

Figure 43. Blood supply of the Optic Nerve. Note multiple arteries that
supply the nerve. Even with ischemic optic neuropathy, vision is preserved
via these vessels.

• Nerve Fiber Layer – recurrent retinal arterioles from the central

retinal artery (CRA)
• Pre Laminar Layer – Short posterior ciliary artery (SPCA), circle
of Zinn-Haller,
Figure 42. (a) Ocular ultrasound of a retinoblastoma – hyperechoic due to • Laminar Layer – SPCA, circle of Zinn-Haller, Pial arteries
calcifications within the tumor (b) CT scan of patient with retinoblastoma • Retrolaminar Portion – SPCA, Pial arteries
showing calcifications that have reached the optic nerve (invasion of
optic nerve). B. PAPILLEDEMA
• Treatment:
→ Visual loss is a serious consideration BUT threat to life is of
utmost importance
▪ Primary objective: save the child’s life
▪ Secondary objective: preserve as much vision as
possible without increasing the risk for metastasis
▪ Tertiary objective: make the patient’s appearance as near
normal as possible to minimize psychological impact
→ Multi-disciplinary approach: Ophthalmologist, Pediatrician,
Hematologist-Oncologist, Radiation Oncologist, Geneticist
→ Small-sized tumors may be treated with laser,
thermotherapy, brachytherapy or cryotherapy depending on
the size and location
→ Medium-sized tumors may be treated with chemotherapy
+/- radiotherapy combined with the modalities for small-sized
tumors Figure 44. Grading of Papilledema. From left to right, top to bottom: Grade
0: nasal disc edema; Grade 1: C-shaped disc edema; Grade 2: entire
→ Large tumors occupying more than half of the eye with no
disc margin without obscuration of vessels; Grade 3: entire margin with
hope for vision restoration undergo enucleation (removal of obscuration of some vessel; Grade 4: entire disc margin with obscuration
the entire globe with sparing of muscles and orbital fat) of vessels at margin and surface of the disc; Grade 5: disc is no longer
▪ The enucleated specimen is sent for histopathologic visible.
examination to look for signs of extraocular spread (i.e.
massive choroidal, scleral and post-laminar optic nerve • Edema of the optic nerve head that results from increased
invasion) intracranial pressure
▪ Adjuvant chemotherapy and radiotherapy are usually • Indistinguishable from disc edema resulting from other causes
utilized especially in those with high-risk histopathologic • Symptoms
characteristics - to prevent recurrence → Headache
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OS 212: Disorders of the Posterior Segment
→ Nausea
→ Vomiting
→ Transient visual obscurations
▪ May be unilateral or bilateral vision loss lasting about
seconds
• Immediate neuroimaging is warranted to ascertain the cause
and treat the underlying etiology
ACUTE PAPILLEDEMA
Figure 45. Acute papilledema. Left: Disc hyperemia; Right: feathery striated
margins of retinal nerve fiber layer (white lines)
• Hyperemia of the optic disc
• Dilation and telangiectasia of the surface and radial peripapillary
vessels
• Peripapillary Retinal Nerve Fiber Layer (RNFL) is edematous,
greyish white, opalescent, with feathered, straited margins that
obscure the disc edge and retinal vessels coursing through it
• Absence of spontaneous venous pulsations
→ Intracranial pressure (ICP) is higher than intraocular
pressure
• Visual acuity, color vision, pupillary responses are usually
normal
Visual Field Examination shows enlarged blind spots
CHRONIC PAPILLEDEMA
Figure 46. Features of Papilledema. Early: hyperemic disc (considered
acute) Chronic: pale disc, tortuous vessels and collaterals. Recall: dead
cells do not swell. In patients with chronic papilledema with dead axons,
you will not see disc edema anymore; even with increased ICP but with
dead axons, edema is not observed.
• Chronically elevated ICP (months to years) leading to
deterioration of Optic Nerve Function
• Features:
→ Disc pallor – as a result of chronic axonal loss
→ Gliosis of the Peripapillary RNFL – appears grayish, less
fluffy and more membranous
→ Optociliary shunt vessels / Retinochoroidal collaterals
→ Refractile bodies of the disc
→ Paton’s lines – peripapillary retinochoroidal folds
• Central visual field involvement and decreased visual acuity
occur late in the process
• Process is usually bilateral
Exam 01 - Trans 06
• Relative Afferent Pupillary Defect (RAPD) may occur if optic
nerves are asymmetrically involved
C. OPTIC NEURITIS
• Generally, affects the retrobulbar portion of the optic nerve
• In approxiamtely 33% of cases, the inflammation may involve
the anterior portion of the optic nerve – making it edematous (i.e.
Papillitis)
• Disc edema is usually hyperemic and diffuse
• Papillitis is more common in post-viral and infectious optic
neuritis than in the demyelinating type
→ More common in children presenting as bilateral profound
visual loss
• Presents with sudden-onset, oftentimes painful (especially on
eye movement), and blurring of vision
→ In typical cases, visual acuity continues to deteriorate from
day 1 – 14 post-ictus (sub-acute), then reaches nadir
(lowest point)
• About 90% of patients will have good functional visual acuity
• The probability of recurrence of optic neuritis in either eye within
5 years is 28%
→ Visual recovery after a second episode in the same eye is
generally very good
DIAGNOSTICS
• Cranial MRi with Gadolinium Contrast and Fluid Attenuation
Inversion Recovery (FLAIR) sequence to determine association
with Multiple Sclerosis
TREATMENT
• Similar whether optic disc edema is present or not
• Optic Neuritis Treatment Trial (ONTT) Regimen:
1. IV Methylpredinosolone – 1g/day for 3 days
2. Oral Prednisolone – 1mg/kg/day for 11 days
• No treatment is also an option
D. ANTERIOR ISCHEMIC OPTIC NEUROPATHY (AION)
• Most common acute optic neuropathy in patients > 50 years
old
• Sudden, painless, monocular vision loss that develops over
hours to days
• Visual acuity may be diminished
• Visual filed loss always occurs
→ Altitudinal or arcuate scotomas most commonly
• RAPD is present unless if involvement is bilateral
• Optic disc edema develops at onset and may precede visual
loss
• Classified into Artretic (AAION) vs. Non-artretic (NAION)
NON-ARTERITIC (NAION)
• More common (90-95% of AION cases)
• Occurs in a relatively younger age group (60 years old)
• Relates to compromised optic disc microcirculation in eyes with
structural crowding of the disc
→ Histology shows the area of infarction located within the
scleral canal alone (Compartment Syndrome theory)
• Visual impairment is usually reported upon awakening
• Visual loss is usually less severe than AAION (> 20/200 in >
60% of patients)
• Most common pattern of visual field loss is altitudinal, but any
pattern may be seen
Figure 47. Pattern of visual field loss. Altitudinal loss; either entire northern
or entire southern field is lost.
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• The optic disc in the contralateral eyes is typically small in • No racial, sex, or age predilections
diameter and demonstrates a small or absent physiologic cup • In most cases, the lesion is not limited to the optic nerve
(Disc-at-Risk) → It may extend to the retina, optic chiasm and tracts
PATHOPHYSIOLOGY
• Unknown
• Generally accepted that the common pathway for most toxins
involves:
→ Mitochondrial injury
→ Imbalance of intra- and extracellular free radical
homeostasis

Table 4. Toxins and Vitamins involved in TON/NON

Most Common Afflicted Most common Afflicted
Toxins Vitamins*
Figure 48. Disc-at-Risk. Note small optic disc with • Methanol • Vitamin B12 Deficiency
cup to disc ratio of 0.2 or 0.1.
• Ethylene Glycol • Folic Acid (B9) Deficiency
• Amiodarone • Thiamin (B1) Deficiency
• 5-year risk of contralateral involvement is 15%
• Linezolid • Deficiency in proteins with
• Associations:
→ Structural crowding of the disc (Disc-at-Risk) • Ethambutol sulfur-containing amino acids
• Isoniazid → Methionine
→ Systemic Hypertension
• Rifampicin → Cysteine
→ Diabetes Mellitus (particularly in young patients)
• Chloramphenicol → Homocysteine
→ Hyperlipidemia
• Hydroxychloroquine Taurine
→ Hypercoagulable disorders
→ Sleep apnea • Penicillamine
→ Nocturnal hypotension • Cisplatin
→ Phosphodiesterase inhibitors (i.e. Sildenafil) • Vincristine
• Lead
Diagnostics • Carbon dioxide
• Ischemic work-up *Deficiencies are commonly observed in patients who have
→ CBC chronic disease of the gastrointestinal tract, who are alcoholics,
→ Urinalysis and those who are incarcerated
→ Fasting blood sugar
→ HbA1c TREATMENT
→ Lipid profile • Discontinue medication, substance abuse, avoid exposure
→ Creatinine or replacement of dietary deficiency
• Optic atrophy eventually develops if the cause is not corrected
Treatment If corrected, prognosis for visual recovery is good (over several
• Untreated NAION generally remains stable after reaching the months)
low point of visual function
• No proven therapy for NAION F. POST-TRAUMATIC OPTIC NEUROPATHY (PTON)
• There is also no proven prophylaxis for the fellow • Damage to the optic nerve caused by trauma to the head, orbit,
uninvolved eye or globe
• Vitamin B supplements may be given for the optic nerve • Classified as Direct or indirect
Continue to observe patients and wait for improvement → Direct – avulsion, laceration (by bone fragment), or
compression (by hemorrhage) of the nerve
ARTERITIC (AAION) → Indirect – transmitted shearing forces damage the optic
• Not common in the PH; not discussed by the lecturer nerve and its blood supply at its intracanalicular tether point;
• Occurs in older age group (>70 years) more common than direct type
• Severe, immediate visual loss
E. TOXIC / NUTRITIONAL OPTIC NEUROPATHY • + RAPD – earliest sign
(TON/NON) • Color vision defects may be present
• Optic disc may appear normal at onset, but becomes atrophic
within 4-8 weeks
• Poor prognosis for visual recovery, but spontaneous recovery
of some visual function has been documented
DIAGNOSTICS
• Neuroimaging
→ Assess the extent of injuring and detect any associated
intracranial, facial, or orbital injury
TREATMENT
• Orbital or cranial surgery may be performed but may not affect
Figure 49. TON/NON. Note pale discs. the prognosis for the optic nerve
• No clear benefit for the use of corticosteroids or optic canal
• Damage to the optic never caused by exposure to toxins, drugs, decompression
metals, organic solvents, alcohols, carbon dioxide or nutritional → In the setting of severe head trauma, corticosteroid therapy
deficiency is not recommended
• Gradual, progressive, painless, bilateral, symmetric visual loss
→ Papillomuscular bundle defects – central/cecocentral, with or
without generalized field depression
• May present with color vision reduction

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G. COMPRESSIVE OPTIC NEUROPATHY • Retinal vessel diameter seems large relative to the disc and
tortuous
• Visual acuity ranges from 20/15 with minimal visual field defects
to no light perception (NLP)
→ Nearly all eyes have visual field loss and bilateral
involvement (56-92%)
• May be associated with midline or hemispheric brain defect,
endocrinologic abnormalities and congenital suprasellar tumors
• Triad of Optic Nerve Hypoplasia: De Morsier Syndrome
(Septo-Optic Dysplasia)
→ Optic never hypoplasia
→ Absent septum pellucidum; thin or absent corpus callosum
→ Pituitary dwarfism (growth hormone is most commonly
affected)
Figure 50. Compressive optic neuropathy. Note temporal disc pallor with • Recognized teratogens:
increase in cup disc ratio that is not glaucomatous in nature. → Quinine
→ Ethanol
• Slowly progressive, painless, monocular or binocular vision loss → Anti-convulsants
and visual field defects
→ i.e. Bitemporal hemianopsia in chiasmal syndromes I. OPTIC PIT
• + RAPD if asymmetric involvement
• Intraorbital or intracanalicular subtypes may have associated
signs of orbital disease such as
→ Eyelid edema
→ Retraction
→ Lag
→ Ptosis
→ Proptosis
→ Extraocular muscle abnormality
• Optic disc may be normal or mildly atrophic at presentation
• Can progress to diffuse atrophy and loss of vision if the etiology
is uncorrected
• Commonly associated with Figure 53. Optic pit. Like a “hole” through the optic nerve that may cause
→ Optic nerve sheath meningioma detachment of macula and fluid enters the pit and enters the subretinal
→ Optic never glioma space.
→ Cavernous hemangioma
→ Pituitary adenoma • Depression of the optic disc surface that is often gray or white
→ Craniopharyngioma • Located inferotemporally
• Associated with a mild visual filed defect (paracentral or arcuate
scotoma)
• Serous detachment of the macula develops in 25-75% of
cases
→ Probably due to liquefied vitreous entering the subretinal
space through the optic pic and into the macula

J. OPTIC NERVE COLOBOMA

Figure 51. MRI of pituitary macroadenoma. (Lecturer did not describe
further).

H. OPTIC NERVE HYPOPLASIA

Figure 54. Opting nerve coloboma. Left: incomplete disc, Right: disc, retina
and choroid are absent, only sclera is present

Figure 52. Double-ring sign. Note very small disc and the tortuous vessels
that pass through the small tunnel.
• A congenital optic disc anomaly
• Small optic disc (1/2 – 1/3 of normal diameter)
→ May seem pale, gray, or hyperemic and surrounded by a
yellow peripapillary halo, which is in turn bordered by a ring
of increased or decreased pigmentation (Double-ring sign)
• Fewer fibers in the optic never than normal
• A congenital optic disc anomaly
• Result from incomplete closure of the embryonic fissure
• Usually occurs inferiorly, with deep excavation of the optic
nerve substance
• May extend to the adjacent choroid and retina
• Visual field defects and RAPD may be present depending on the
severity of the abnormality
• May occur with colobomas of the iris choroid, or retina, or be
part of a syndrome

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K. MORNING GLORY DISC ANOMALY

Figure 55. Morning glory anomaly. Note large optic nerve and radial
emanation of vessels from disc. Note white tuft of glial tissue at the
center of the disc surface.

• A congenital anomaly
• Funnel-shaped staphylomatous excavation of the optic nerve
and peripapillary retina
• More common in females
• Unilateral
• Disc is enlarged, pink or orange, and either excavated or
recessed within the staphyloma
• Chorioretinal pigmentation surrounds the excavation
• A tuft of white glial tissue is present on the central disc surface
• Characteristic feature: radial emanation of vessels from the
disk
• Visual acuity is often 20/200 or worse when accompanied by
RAPD
• Non-rhegmatogenous serous retinal detachments occur in
26-38% of cases
• Neuroimaging is warranted to evaluate for a basal
encephalocoele and CNS vascular anomalies.

END OF TRANS

REFERENCES
Dr. Mesina’s Lecture

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

SUMMARY PAGES
Summary Table 1. Disorders of the Posterior Segment
Disease Description / Pathology Diagnosis Treatment / Management Notes
DISORDERS OF THE VITREOUS
Posterior Vitreous • Secondary to vitreous syneresis Diagnosed clinically via Mainly assurance, observation and Can be associated with
Detachment • Present with floaters and/or flashes/photopsias funduscopy regular follow-up to look for retinal 15% of acute symptomatic
• Presence of Weiss ring (round/oval shaped tissue tears PVD and 50-70% of PVD
from the detachment of the vitreous body from the with concomitant vitreous
glial optic disc margin) hemorrhage
Vitreous Hemorrhage • Presence of blood within the vitreous body • Diagnosed clinically via • Management is directed at
• Patients typically complain of floaters funduscopy determining the etiology of the
• Bleeding is typically from a retinal vessel • Ocular B-Scan Ultrasound hemorrhage and clearing the blood
• Arises from a myriad of causes: may be performed to evaluate • If no other pathologies, hemorrhage
→ Avulsed retinal vessel from PVD the status of the rest of the may be observed 4-6 months and
→ Retinal tear/break cutting thru a retinal vessel posterior segment allowed to resorb on its own
→ Neovascularizations • Vitreo-retinal surgery via Pars Plana
→ Trauma Vitrectomy w/ treatment of the
→ Tumors primary etiology if in the presence of
retinal detachment or tumor
DISORDERS OF THE CHOROID
Central Serous • Idiopathic serous neurosensory retinal detachment • Fundoscopy (blister-like • Observation for unilateral cases and
Chorioretinopathy secondary to an altered barrier and deficient pump elevation of the macula first episodes
function at the retinal pigment epithelium (RPE) • FA (expansile dot or • Macular laser photocoagulation
• Asymptomatic if the macula is unaffected Smokestack or Diffuse)
• Patient profile: • OCT (Neurosensory
→ Middle-aged male detachment)
→ Type A personality, stressed individuals,
hypochondriacs
→ Hysterical/neurotic, on psychiatric medications
or steroids
→ Cushing’s syndrome or SLE
DISORDERS OF THE RETINA
Diabetic Retinopathy • Complication of diabetes mellitus • Vision loss mainly comes • Intensive blood sugar control • Screening:
(DR) • Manifests mainly as vascular changes in the retina secondary to (proven to be a key modifiable factor → Type 2 DM –
• Structural damage to capillary endothelium (loss of → Macular edema (occurs at to reduce risk and delay onset of examine upon
mural pericytes and basement membrane ANY STAGE of diabetic diabetic retinopathy) diagnosis, then
thickening) retinopathy) • Blood pressure control slows the annually
→ Macular ischemia progression of diabetic retinopathy → Type 1 DM –
→ Neovascularization • At initial stages, observation and examine 3-5 yrs
sequelae regular monitoring of the retinopathy after diagnosis, then
are sufficient annually
→ Pregnant patients –
regardless of type,
examine pre-
conception or 1st
trimester, then every
1-3 months if stage
is severe
• Non-Proliferative See Table 1 in the body
DR

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

• Proliferative DR Neovascularization eventually occurs due to Clinical findings: 1. Laser treatment – panretinal
angiogenic stimulus (deprived of oxygen supply) photocoagulation
+ vasoproliferative factors (VEGF) + viable source One or more of the following 2. Intravitreal Anti-VEGF injections
of endothelial cells 1. Neovascularization 3. Vitreo-retinal surgery to evacuate
2. Vitreous/Pre-retinal hemorrhage, sever traction
hemorrhage membranes, re-attach the retina
Hypertensive • 3 Main Pathologies: See Table 2 in body for clinical • Mainstay of treatment is strict and
Retinopathy → Vascular constriction - generalized or focal findings adequate control of blood
arteriolar narrowing pressure
→ Leakage - abnormal vessel permeability
▪ Manifests as flame-shaped hemorrhages,
retinal edema, hard exudates, optic disc
edema
→ Arteriosclerosis - intimal layer hyalinization,
medial layer hypertrophy & endothelial
hyperplasia
▪ Presents as focal narrowing and straightening
of the retinal arterial walls
Central Retinal Artery • One of only two “true” Ophthalmic emergencies Fundoscopy, Fluorescence • Immediate lowering of intraocular
Occlusion (CRAO) (very well-emphasized) Angiography pressure to improve perfusion by
• Sudden, painless, unilateral, severe loss of reducing intraocular resistance to
vision (i.e. counting fingers to light perception BUT blood flow
NEVER NO LIGHT PERCEPTION (NLP) – this is → Ocular
usually presented by ophthalmic artery occlusion → Brown bag breathing to induce
rather than CRAO because you still have supply for hypercarbia (hypercarbia lowers
the outer 1/3 of your retina) your IOP)
→ Anterior Chamber paracentesis
→ Acetazolamide & other IOP
lowering agents
→ Clot-lysing agents
Central Retinal Vein • Sudden, painless, blurring of vision Fundoscopy, Fluorescence • Non-ischemic: no definitive ocular
Occlusion (CRVO) • Thrombosis at the level of or posterior to the Angiography treatment; but primary cause must
lamina cribrosa be ascertained and addressed
• Ischemic:
→ Panretinal Photocoagulation
(laser therapy) if with at least 2
clock hours of Iris
neovascularization
→ Intravitreal steroid or anti-VEGF
injections (especially for macular
edema)
• Ischemic CRVO • More severe loss of vision Fundoscopy, Fluorescence
• (+) RAPD Angiography
• Fundus is covered with multiple splinter & flame-
shaped hemorrhages
• (+) Cotton-wool spots
• Retinal veins are tortuous and dilated
• (+) Optic Disc Edema
• VA usually <20/200
• 60% risk of neovascularization

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

• Non-iscehmic • aka Venous Stasis Retinopathy Fundoscopy, Fluorescence
CRVO • Less severe loss of vision Angiography
• Usually (-) RAPD
• Fewer retinal hemorrhages
• Usually (-) Cotton-wool spots
• Retinal veins are tortuous and dilated
• Usually (-) Optic Disc Edema
• VA better than 20/200
Much better prognosis
Age-related Macular • 2 types of changes in the macula: Fundoscopy, Fluorescence
Degeneration → Normal Age-Related Changes Angiography, Ocular Coherence
(ARMD) → Pathologic Age-Related Changes Tomography
• 2 types of Pathologic Age-Related Macular
Degeneration:
→ Non-neovascular (Dry-type)
→ Neovascular (Wet-type)
• Non-Neovascular • More common, less severe visual loss Fundoscopy, Fluorescence • General:
ARMD • Gradual, painless blurring of vision (central) Angiography, Ocular Coherence → UV Avoidance
• Classified into: early, intermediate, advanced [not for Tomography → Increase in green-leafy
LU IV Level] vegetable intake
• Hallmark: DRUSEN formation → Smoking cessation
→ Look like hard exudates → Amsler grid monitoring
→ Bruch’s membrane thickening from deposition • Non-neovascular Specific: Age-
of basal debris made of granular lipid-rich related Eye Disease Study
material or phospholipid (AREDS) – for intermediate or
• Atrophy of the Retinal Pigment Epithelium advanced unilateral cases of ARMD
→ Subset 1: 500mg Vitamin C +
400 IU Vitamin E + 80mg Zinc
Oxide + 15mg Beta carotene + NOTE: we do not give
2mg Cupric oxide Subset 1 for smokers
→ Subset 2 (smokers): Lutein + because Beta carotene
Zeaxanthin + Omega 3 Long- increases the risk for lung
chain PUFA substitute cancer
• Neovascular ARMD • VA is about 20/200 or less Fundoscopy, Fluorescence • General:
• Sudden-onset blurring of vision, metamorphopsia Angiography, Ocular Coherence → UV Avoidance
• Hallmark: CHOROIDAL NEOVASCULARIZATION Tomography → Increase in green-leafy
→ Buds of neovascular tissue from the vegetable intake
choriocapillaris perforate the outer Bruch’s → Smoking cessation
membrane → Amsler grid monitoring
• Neovascular Specific:
→ Photodynamic Therapy with
Verteporfin (light therapy for the
macula)
→ Itravitreal Anti-VEDF injections
(Ranibizumab, Bevacizumab,
Aflibercept) Non-neovascular
(Dry-type)

Retinitis Pigmentosa • Panretinal dystrophy that belongs to a group of Fundoscopy, Fluorescence • No known treatment
heredodegenerative diseases (aka tapetoretinal Angiography • Cataract extraction may help
diseases) that involve photoreceptor & pigment improve vision
epithelial dysfunction

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

• Progressive degeneration of the rods & cones with • Daily supplementation with Vitamin
migration of the pigment epithelial cells into the A 15,000 IU or 25,000 IU twice a day
retina may help slow down deterioration of
• RETINITS PIGMENTOSA TRIAD: ERG changes, with no known
→ Waxy Optic Disc pallor demonstrable effect on vision
→ Bony spicule-like changes (secondary to • UV protection is debatable
macrophage apoptosis) or RPE atrophy (actual
death of photoreceptors and migration of RPE
into your retina
→ Attenuation (or narrowing) of the retinal vessels

Retinitis Detachment • Separation of the inner layers of the retina from Fundoscopy
theunderlying retinal pigment epithelium (RPE)
& choroid, often with accumulation of fluid in the
subretinal space

NOTE: when referring to neurosensory retina, it’s

from the internal limiting membrane to the
photoreceptor layers
• Rhegmatogenous • With a retinal tear, break or hole usually at the Fundoscopy • Retinal Detachment Surgery
Detachment periphery (principles):
• Most common • Pars Plana Vitrectomy
• Shifting of fluid from supine to upright is • Pneumatic Retinopexy
UNCOMMON • Focal Laser Therapy
• Scleral Band & Buckling
• Non- • 2 Types: Fundoscopy • Manage the underlying cause
regmatogenous → Tractional • Usually not surgical
Detachment ▪ 2nd most common type • Usually oral or systemic steroid
▪ Fibrous membranes on the vitreous & therapy
surface of the retina contract and elevate the
neurosensory retinal layers
→ Exudative
▪ Accumulation/leakage of fluid into the
subretinal space from damage of the retinal
blood vessels or RPE (without any hole or
tear)
▪ Caused primarily by diseases of the RPE &
Choroid
▪ Convex configuration
▪ Smooth appearance
▪ (+) Shifting fluid from supine to upright
(emphasized)

Cytomegalovirus • Most common cause of congenital viral infections
(CMV) Retinities • Most common ophthalmic manifestation in
HIV/AIDS
→ Especially in patient with CD4+ counts of <50
cells/µL
• Full-thickness hemorrhagic retinal necrosis
with yellow-white cloudy retinal lesions or
infiltrates
Fundoscopy • Oral Valganciclovir (900mg BID for
3 weeks as induction, then 450mg
BID as maintenance)
• Intravenous Ganciclovir (500mg IV
q12 for 2-3 weeks as induction, then
5 mkday infusions as maintenance)
• Foscarnet, Cidofovir, Intravitreal
Ganciclovir

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

• Fulminant CMV • Fulminant - large areas of hemorrhage, typically in Fundoscopy
the posterior pole, following the distribution of the
RNFL

• Granular / Indolent • Granular/Indolent - typically in the retinal periphery; Fundoscopy

CMV little or no hemorrhages or necrosis

• Forsted Branch • Frosted Branch Angiitis – perivascular type Fundoscopy

Angitis CMV
Ocular • Most common cause of infectious retinochoroiditis Fundoscopy • Quadruple Therapy
Toxoplasmosis in adults & children → Pyrimethamine (50-100mg/day
• Ocular Manifestations: LD; then 25-50mg/day
→ Variable anterior chamber reaction maintenance)
→ Retinochoroiditis (predilection for posterior pole → Sulfadiazine (2-4g/day LD; then
& macula) 4g/day maintenance)
→ “Headlight in the fog” (Vitritis) → Prednisone (0.5-1mkday)
→ Focal white retinitis adjacent to a pigmented → Clindamycin (300mg 4x/day)
chorioretinal scar → *Folinic Acid - prevent
→ Kyrieleis arteritis - vasculitis in the vicinity of leukopenia and
active lesions (also found in Syphilis) thrombocytopenia
• Triad in Congenital Toxoplasmosis:
→ Hydrocephalus
→ Intracranial Calcification
→ Chorioretinitis
Retinoblastoma • Most common intraocular malignancy of • Thorough and extensive • Visual loss is a serious consideration
childhood medical, family, birth and BUT threat to life is of utmost
• No known sexual or racial predilection maternal history importance
• Average age of onset: 18 months • Complete 5-point → Primary objective: save the
→ Rarely seen beyond 6 years of age ophthalmologic examination child’s life
• Caused by mutations on the RB1 gene on the long • Dilated funduscopy → Secondary objective: preserve
arm of Chromosome 13 (13q14) • Systemic & Neurologic as much vision as possible
→ Tumor-suppressor gene whose presence physical examination to check without increasing the risk for
(even of a single allele) protects against the for signs of metastasis metastasis
development of the tumor (especially in advanced → Tertiary objective: make the
→ Knudson’s Two Hit Hypothesis: stages) patient’s appearance as near
▪ One single retinal cell suffers a mutation on • Ocular B-Scan Ultrasound normal as possible to minimize
one allele of the RB gene, then after some → Especially in cases where psychological impact
time suffers another mutation on the there is no view of the • Multi-disciplinary approach:
remaining allele posterior segment • Small-sized tumors may be treated
▪ The loss of the tumor suppression on both • Cranial CT-Scan with laser, thermotherapy,
alleles allows that particular cell to multiply → Most useful ancillary test brachytherapy or cryotherapy
uncontrollably → Check for calcifications & depending on the size and location
extension into the brain • Medium-sized tumors may be
treated with chemotherapy +/-
radiotherapy combined with the
modalities for small-sized tumors
• Large tumors occupying more than
half of the eye with no hope for
vision restoration undergo
enucleation (removal of the entire

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

globe with sparing of muscles and
orbital fat)
DISORDERS OF THE OPTIC NERVE
Papilledema • Edema of the optic nerve head secondary to Immediate neuroimaging to Graded from 0 to 5
increased intracranial pressure ascertain cause and treat
• Symptoms: underlying etiology
Headache, nausea, vomiting, transient visual
obscurations
• Acute Papilledema • Hyperemia of optic disc
• Edematous Peripapillary retinal nerve fiber layer
• Absence of spontaneous venous pulsations
Enlarged blind spots on visual field examination
• Chronic • Chronic Elevated ICP
Papilledema • Deterioration of the optic nerve function
• Disc pallor, gliosis of Peripapillary RNFL
• Optociliary shunt vessels / Retinochoroidal
collaterals
• Refractile Bodies of disc
• Paton’s lines
• Bilateral
RAPD
Optic Neuritis • Affects the retrobulbar portion of the optic nerve Cranial MRI and FLAIR • ONTT Regimen (IV • 90% of patients will
• Disc edema: hyperemic and diffuse sequence to determine Methylpredinosolone followed by have good functional VA
• Sudden-onset and painful with blurring of vision association with Multiple Oral Prednisolone) • 25% chance of
Deterioration of VA starts from Day 1-14 Sclerosis No treatment is also an option recurrence of optic
neuritis in either eye
within 5 years
Anterior Ischemic • Most common acute optic neuropathy (for patients RAPD is present unless if
Optic Neuropathy < 50 y.o.) involvement is bilateral
(AION) • Sudden, painless, monocular vision loss
Visual field loss always occurs
• Non-Arteritic AION • More common (90-95% of AION cases) than • Ischemic work-up • No proven therapy or prophylaxis 5-year risk of contralateral
AAION • May have Vitamin B involvement is 15%
• Prevalent in younger (60 y.o.) age group supplementation
• Relates to compromised optic disc microcirculation Observe for improvement
in eyes with structural crowding in disk
• Altitudinal field loss is common but any pattern may
be seen
Disc-at-risk with cup to disc ratio of 0.2 or 0.1 (very
small)
• Arteritic AION Not discussed by lecturer because it is not common in Occurs in the older age group (>
the PH 70 y.o.)
Toxic / Nutritional • Damage due to exposure to toxins, drugs, metals, Discontinue medication, substance Deficiencies commonly
Optic Neuropathy organic solvents, alcohols, carbon dioxide, or abuse, avoid exposure or replacement observed in patients with
nutritional deficiency of dietary deficiency chronic GI problems, who
• Gradual, painless, and bilateral symmetric visual are alcoholics, who are
loss incarcerated
• Unknown pathophysiology but attributed to
common pathway for toxins:
→ Mitochondrial injury

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OS 212: Disorders of the Posterior Segment Exam 01 - Trans 06

Disease Description / Pathology Diagnosis Treatment / Management Notes

→ Imbalance of intra- and extracellular free radical
homeostasis
• Highlighted toxins: TB medication: Ethambutol,
Isoniazid, Rifampicin
Highlighted Deficiencies: Vit B12, B9, B1, proteins
with sulfur-containing amino acids
Post-traumatic Optic • Damage to the optic nerve due to trauma Neuroimaging to assess extent • Orbital or cranial surgery (may not
Neuropathy • Direct (avulsion, laceration, compression) of injury affect prognosis for optic nerve)
• Indirect (transmitted shearing forces) No clear benefit from corticosteroids or
• Severe, immediate visual loss optic canal decompression
• RAPD is the earliest sign
• Optic disc normal at onset but atrophic within 4-8
weeks
Poor prognosis for visual recovery
Compressive Optic • Slowly progressive, painless, monocular or Fundoscopy and MRI of pituitary
Neuropathy binocular vision loss and visual field defects.
+ RAPD if asymmetric involvement
Optic Nerve • Small optic disk (1/2 – 1/3 of normal diameter)
Hypoplasia • Double-ring sign
• VA ranges from 20/15 with minimal visual field
defects to NLP
• Triad of Optic Nerve Hypoplasia (De Morsier
Syndrome)
→ Optic nerve hypoplasia
→ Absent septum pellucidum; thin or absent
corpus callosum
Pituitary dwarfism
Optic Pit • Depression of optic disc surface (gray or white
appearance)
• Inferotemporally located
Serous detachment of the macula (in 25-75% of
cases)
Optic Nerve • Incomplete closure of the embryonic fissure
Coloboma • Occurs inferiorly with deep excavation
May extend to choroid, retina, and iris.
Morning Glory Disc • Funnel-shaped staphylomatous excavation of the • Neuroimaging to evaluate for
Anomaly optic nerve basal encephalocoele and
• More common in females CNS vascular anomalies
• Unilateral with chorioretinal pigmentation
• Characteristic feature: radial emanation of vessels
from disc
Non-rhegmatogenous serous retinal detachments in
26-38% of cases

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