Final Exam Flashcards
Cystoid Macular Edema
Accumulation of fluid in Henle’s fiber layer with cyst like spaces.
What is CME called if there are no cyst
clinically significant macular edema. Often seen in diabetic maculopathy.
CME subjective
blurred vision, metamorphosis, washed out vision
What conditions cause CME
DR, Vein occlusion, post surgery (cataracts, glaucoma, retinal detachment), uveitis, RP. Post DR. OU.
Signs of CME
Loss of foveal depression, thickening of fovea, foveal folds.
FA of CME
Increase in vascular permeability, accumulation of dye in OPL of retina, Radial arrangement of fingers in henley (causes a petaloid pattern)
Tx of CME
Topical NSAIDs (voltaren qid) or topical steroid (prednisolone acetate qid) for 1 month and taper. If doesn’t work Consider oral NSAIDS (indomethacin) or oral steroid (prednisone) or oral acetazolamide (diamox). Can also do steroid injection. Can also do vitrectomy.
Epiretinal Membrane
Cellular proliferation along the internal limiting membrane and retinal surface. Epirretinal membrane looks like wrinkled cellophane.
What causes epiretinal membrane
Most are idiopathic. Can be due to prior retinal surgery, intraocular inflammation, vitreous hemorrhage, trauma, cryotherapy.
Epidemiology of epiretinal membrane
increases in incidence with age
Symptoms of Epiretinal membrane
asymptomatic, decreased vision, metamorphosis, micropsia, monocular diplopia.
Epiretinal membrane signs
Normal or decreased acuity. Abnormal ambler grid. BV are tortuous by stretched from the disc. Retinal folds or striae.
Epiretinal membrane Tx
Tx rarely needed. Vitrectomy and membrane peel in patients with reduced acuity (
What does chloroquine and hydroxychloroquine treat.
(used to treat malaria, arthritis, and SLE)
Signs of toxic retinopathy from chloroquine and hydroxychlorquine
small scotoma, supernormal EOG, abnormal photostress test, decreased color vision, VA typically affected later. Abnormal macular pigmentation (bulls eye)
What dosage of chlorine and hydroxyqhloroquine will cause toxic maculopthy?
chloroquine > 250-300 mg/d. Hydroxychloroquine >700-750 mg/d
How to scan patient taking hydroxychlorquine or chloroquine?
DFE, OCT, 10-2 threshold VF, and autofluroscene as baseline. Should be seen every 6 m.
Toxic maculopathy caused by Thioridazine
Used to treat psychotic patients. Can produce decreased vision, night vision problems, ring scotomas, brown discoloration of vision. Will have granular pigment-normally mid peripheral and then coalesces into large areas of pigmentation.
Toxic Retinopathy caused by Tamoxifen.
Tamoxifen used to treat breast cancer. Results in decreased vision, refractive yellow-white spots thought the posterior pole. Can also be seen with drug user as they dilute drugs with it.
Choroidal Folds
Folds of the choroid and the overlying structures. Due to flattening of the posterior pole–>acquired hyperopia as the retina is pushed anterior to the plan of focus.
Choroidal Folds causes
idiopathic, hyperopia, choroidal tumor, CNM, optic disc swelling, orbital tumors, hypotony, orbital and scleral inflammation.
Choroidal folds symptoms
asymptomatic, blurry vision, metamorphsia.
Choroidal folds FA
Will see alternating hypo and hyper fluorescent lines. Hypo=RPE troughs. Hyper=RPE peaks.
Mgmt of choroidal folds
Usually idiopathic. Diagnosis of exclusion.
How much ocular volume does vitreous compromise?
75%
What percent H20 is vitreous?
99
Vitreous attachments
Lens attachments (Weiger’s adhesion), vitreous base (pars plans), Optic disc margin, Macular area
Which vitreous attachment is weakest
Macular
What vitreous attachment is strongest
vitreous base (pars plans and peripheral retina)
Vitreous degenerations
Liquefaction occurs over time. Syneresis=shrinking with inward collapse of vitreous also occurs. Increases in myopes. Creates Lacunae (liquid spots)
Examination of the viterous
Must dilate.
Unusually findings in vitreous
cells, blood, pigment, PVD.
Asteroid Hyalosis (benson’s disease)
90% unilateral. Calcium. Reflective yellow shite spheres-gold. Commonly asymptomatic. Associated with Diabetes, HTN, vascular disease.
Synchysis Scintillans (cholosterolosis bulbi)
Bilateral and rare. Free floating flat crystals of cholesterol. Associated with severe eye disease.
Non-pigmented cells (white) in viterous
Inflammatory cells. May be fine, course, or snowball.
Staffer’s Sign
Pigmented cells (red brown) in vitreous. Occurs with retinal tear or detachment.
Intraviterous hemorrhages
Ruptured vessel bleeds through break in hyaloid membrane. Associated with DR, Retinal breaks without RD, rhegmatogenous RD, RVO. Slow reabosrption
Retroviterous hemorrhage
Blood trapped between retina and hyaloid membrane. Associated with trauma, DR, retinal breaks without RD, RD, RVO. Shifts with eye movement. Boats and blobs.
PVD
Hyaloid membrane is pushed forward due to liquification.
PVD symptoms
Floaters, photopsia, blurry vision, possible metamorphosis, possible red hue of vision.
Signs with PVD
Weiss Ring, Possible hemorrhage, may be able to see collapsed posterior limiting layer of the vitreous,
Epiretinal membrane and PVD
Associated with PVD. 75% of eyes with PVD. May result in macular edema or full thickness macular hole.
How to dx Macular hole
Stratus OCT.
Categories of vitreous detachments
complete PVD with collapse, complete PVD without collapse, incomplete PVD with collapse, anterior vitreous detachment.
Retinal involvement with PVD
Breaks usually at posterior base. PVD found in 80% of patients with retinal tears. Retinal tears with PVD occurs in 10%.
Mgmt of PVD
DFE with scleral depression (normally retinal detachment will occur 6 weeks after PVD), Advise, RTC 1-2 weeks then 1 month later, retinal referral if break noted.
Persistent Hyperplatic Primary Viterous (PHPV)
Failure of primary vitreous to regress, unilateral, full term gestation with no oxygen supplementation.
Anterior PHPV
Mild to severe lens involvement with minimal posterior pole changes. Mgmt with surgery
Posterior PHPV
Vitreous membrane and vitreoretinal adhesion, peripapilallary RPE changes, pale hypo plastic disc. Mgmt-monior.
Congenital hereditary retinoschisis
Retinal splits at NFL. Bilateral. X-linked. Inferotemporal 50% with a veil membrane that does NOT run to ora. Macular involvement 98%. RD 25%, vitreous hemorrhage 40%.
How to diff. congenital hereditary retinoschisis from RD
Will not move or bounce around at all. Retinal with move with RD. Will have an absolute field defect (with RD can have normal field as cells still firing)
Iatrogenic Viterous changes
PVD (from surgeries), vitrectomy (removal of vitreous for RD surgery), vitreal prolapse (herniation of vitreous through breaks/holes), literal hemorrhage (break during surgery)
Peripheral retina
zone from the equator to the ora errata. 3DD in width.
Landmarks in peripheral retina
vortex vein, long posterior ciliary nerves, short posterior ciliary nerves, ora serrated, pars plans, vitreous base
Cystoid degeneration
Present in almost everyone. Retinoschisis association. Found in peripheral retina
Chrotioretinal degeneration
Found in peripheral retina. AKA pigmentary degeneration. Very common
Paving stone degeneration
Found in peripheral retina. AKA cobblestone degeneration. 27% population and in myopes more. Inferior temporal retina more common.
Reticular pigmentary degeneration
Peripheral retina findings. Bone spicule like RPE mottling. Elder patients.
Equatorial drusen
AKA peripheral druse. Not involving macula=benign
Snowflake degneration
inherited condiiton. Don’t worry about. Peripheral retina.
Lattice degneration
Thin areas of the retina. IN mid periphery. Holes often form. Education on RD
Pigmented lattice degeneration
Thin areas of the retina but with pigmentation.
Snail track degeneration
lattice variation in a snail track line. Risk of RD.
White without Pressure
Usually benign.Seen temporal in most eyes and bilateral. Vitreous liquefaction. Retinal holes/breaks can occur.
Vitreoretinal adhesion
No tear-watch. Tear-refer.
How to best view peripheral retina
Scleral indentation
What is the distance from ora to equator?
1/5 the entire retina
Where is paving stone degeneration normally found
Inferior nasal
Where is lattice normally found
superior.
Enclosed oral bay
Entrapment of pars plans around the areas of ora. More common in nasal. Not important. DDX with depression and will not lift with dynamic movement of the tissue.
What color are holes in the retina?
Red
Meridional Fold
Whiter, slightly elevated compared to the retina. Involve all neural layers. 1/2-4 DD. Vessels course over them. Most commonly aligned with processes.
Incidence of Meridional Folds
25% of eyes. Bilateral in 50%. M>F. Usually only one fold but multiple in 27%. Congenital but can increase in size with age.
Location of Meridional Folds
Superior nasal. Perpendicular to ora. Radially oriented folds of redundant retinal tissue
Meridional Complex
Meridional folds that covers both bay and process.
Associated findings with meridional folds
Vitro-retinal tags at end of folds. Can also find holes at posterior end possible and this can lead to retinal detachment (especially temporally). Holes even more common in meridional complex.
Management of meridional folds
Scleral depression to check for holes. Follow every 3 months for RD if hole or tag. If risky use cryopexy.
Cystoid Degeneration
Tiny bubble appearance next to ora beneath vitreous base. Salt and pepper appearance. Thickened retinal tissue. Slightly opaque. Can be difficult to distinguish from WWP.
Cystoid degeneration location
Superior temporal retina. Bilateral and symmetrical. May go all the way to the equator. .
Incidence of cystoid degeneration
Common in children over age of 8 but progresses with age.
Where does the cystoid degeneration occur
Cystoid spaces occur between retinal layers (outer plexiform layer)
Cystoid degeneration associations
Retinoschisis. With time can extend between inner and outer limiting membrane.
Cystoid Degeneration Managment
No tx. If hole found in tissue, very little chance of RD. Holes usually within vitreous base so no traction-tugging is from a broad base and not pulling on that hole and fluid in the retina goes into cyst spaces.
White without pressure description
Usually parallel to ora. Milky white/opalecent. Scleral depression increases whiteness. Present without pressure. Posterior margin is irregular, yet sharp. Flat.
White with pressure
Only can see it when pressing on retina with pressure
White without pressure incidence
32% of eyes. Increases with age. More commonly seen in more deeply pigmented race.
White without pressure location
Between ora and equator. Can extend to posterior pole. Inferior nasal least common. Can increase with age.
What causes white without pressure?
Unknown cause but two theories. Increased reflectivity of photoreceptor outer segment or manifestation of peripheral vitreous traction.
White without pressure tx
No significant associated findings. No management unless vitreous degeneration, nearby lattice degeneration, or history of large retinal tear in other eye.
Pavingstone degeneration description
appears as multiple round punched out lesions. Yellow white in appearance because seeing sclera. Discrete margins often with pigment. Can see large choroidal vessels at base. Excavated and not elevated.
Paving stone degeneration incidence
22% of eyes. Increases with age
Paving stone degeneration location
80% in inferotemporal quadrant. usually between ora and equator.
Cause for paving stone degeneration
Infarcts in choriocapillaries. Inner retinal layers and vitreous are not affected. There is thinning in outer retina.
Tx for paving stone degeneration
Sometimes coalesce to yield scallops of pigment. No associated findings. Only need to document.
Reticular Senile Pigmentary Degeneration Description
Pigment clumping
Reticular Senile pigmentary degeneration incidence
18% of population. Bilateral in almost all cases
Reticular Senile Pigmentary degeneration location
Can be anywhere but possibly more apparent nasal. Can extend to equator.
What is reticular senile pigment degeneration often seen as?
RP. Will not have no VF defects or night blindness though
What causes reticular senile pigment degeneration
Loss of pigment granules from some RPE cells with increase in others. Pigment may be deposited near retinal venules
Clinical course of reticular senile pigmentary degeneration
Increases with age. No associated findings. No management necessary.
Vitreoretinal Tufts Description
Discrete white to gray irregular clumps on retinal surface. Elevated. Granular appearing. Can be surrounded by cystoid.
Vitreoretinal Tufts incidence
Non cystic tufts seen in 72% of population. Bilateral 50% of the time
Vitreoretinal Tufts location
Most common nasal. usually located just posterior to ora in vitreous base
What are vitreoretinal tufts
Small masses of cells of degenerated retina or proliferated glial cells. Retina is intact with a tuft.
Clinical course of vitreoretinal tufts
Remain stationary in size and number.
Vitreoretinal tufts associated findings
firm attachments between retina and vitreous can cause retinal breaks.
Management of vitreoretinal tufts
Make sure there are no holes at base. If hole present watch q3m then q6m. If risks are present consider treating holes with cryopexy.
Risks with vitreoretinal tufts
If have any of these send out. Complains of flashing lights, pigment in vitreous, RD in fellow eye.
Lattice Degeneration
Well circumscribed oval or elliptical lesion. Criss-cross pattern of white lines in classic (only 6-9%). Well demarcated area of retinal degeneration that has a dull, rough appearance. Blood vessels that cross the lesion give it the name. Fairly normal retina is always present between lattice degeneration and the ora serrata. May have pigmented borders.
Where does lattice tend to occur?
Superior.
What is lattice if it goes right against the ora?
It is cystoid degeneration
What are we worried about with lattice degeneration?
A hole forming in the lattice. We are concerned about the posterior leading border of the lattice. Nothing will prevent it from heading straight down to the macula.
Incidence of lattice degeneration
8-11% population. 42% there are atrophic holes. Bilateral 50% of the time.
Location of lattice
between periphery and equator. 5-7 and 11-1. If near equator tend to be radial and follow blood vessel.
What is lattice degeneration
Pigmented cavity in center due to atrophic retinal thinning. Lines are blood vessels with thickened walls. Continues with normal vessels outside of lesion. Will have virtual tent with form attachment on border. Vitreous liquification above lesion.
Clinical course of lattice degeneration
Becomes visible in teens and increases with age. Associated with RD, holes, overlying vitreous liquification, fine white specks in lesion.
Lattice degeneration Mgmt
Follow regularly. Consider laser prophylaxis if holes. Consider tx with risk. Tx needed if history of rd in other eye. Holes increase chance of RD if PVD or cataract removal.
Snail Track Degeneration
Glistening white area in the retina. Usually found between equator and ora. 80% within 2 DD anterior to equator. Most frequently temporal.
Associated conditions with snail track degeneration
As with lattice can result in retinal breaks or holes. Normally RD only occur in a very small amount
Tx for snail track degeneration
With large holes may condition retionoplexy
Atrophic retinal hole description
Look pinker or refer then surrounding tissue. Round and discrete cut out
Where are atrophic retinal holes found
all quadrants
What causes atrophic retinal holes
atrophic retinal thinning.
Are atrophic retinal holes associated with RD
very rarely. No virtual attachment to them
Mgmt for atrophic retinal hole
Consider laser tx if in periphery with traction or risk.
Retinal horseshoe tear Description
Elevated flap of light colored retinal tissue in V shape. Tears may be round, linear, or horseshoe shaped depending on characteristics of retina and vitreous.
Incidence of retinal horseshoe tear
6.4% of retinal breaks
Location of retinal horseshoe tear
Superior more often.
Who has a higher chance of horseshoe tears?
Myopes.
What causes Retinal horseshoe tear
degeneration of inner retinal layers, overlying vireos degeneration, and traction. Often trauma induced tear. One end attached to vitreous tuft. Lattice on flap quite common.
Management of Retinal horseshoe tear
Due to vitreous attachment and the flap it leads to RD more often. Always refer for treatment.
Are smaller are larger retinal horseshoes more scary?
Smaller indicates it has been there longer so less of a scary situation.
Operculate tears
Retinal hole with flap of tissue completely torn off.
Who is operculated tears found most commonly in?
Older people because of their association with PVD.
Where are operculated tears found?
between ora and equator. Most often superior but any quadrant is possible.
How are percolated tears caused?
Most often trauma
Are percolated tears correlated with RD?
No. Very rarely cause.
MGMT of percolated hole
Tx if high myopia, aphasia, extensive degeneration, or family history of RD.
What will be seen with RD
Top with decrease as fluid can now drain out, photpsia, shafer’s sign.
RD description
Separation of neurosensory retina from the RPE. White folds of retinal tissue, masks choroid detail, tissue moves with eye movement.
RD VF
Will only have a relative scotoma or normal. Photoreceptors are still functioning.
When will you see a pigmented demarcation line with RD?
If stable for 3 m.
Symptoms of a RD
Initially related to vitreous traction on retina. Light flashes due to pulling and floaters that appear.
RD epidemiology
Can be any age. Myopes >6, Aphakes have high risk 1 year after surgery.
What condition can result in an exudative retinal detachment?
best’s disease
RD cause
Fluid accumulates between RPE and sensory retina
Rhegmatogenous RD
Full thickness break. Atrophic holes and tractional tears allow vitreous into the sub-retinal space and leading to separation of sensory retina from RPE. (the retina is not intact).
What condition cause rhegmatogenous RD
high myopia, lattice, family history, PVD, trauma, RRD in fellow eye, Marfan’s syndrome, stickler’s syndrome.
Tractional RD
Pre-retinal. Chronic traction of the inner surface via neovascularization and scarring (The retina is intact)
What causes traction RD
DR. VOS. Proliferative diabetic retinopathy, retinopathy of prematurity, vein occlusion, ocular ischemic syndrome, proliferative sickle cell retinopathy.
Exudative RD
Sub-retinal. Leakage of fluid into the sub retinal space from damage to RPE or choroidal blood vessels (the retina is intact)
Causes of exudative RD
Vascular conditions, inflammatory conditions, neoplasia conditions, and congenital anomalies. CNVM conditions (CH BALA)-chroidal rupture, histoplasmosis, best’s, angled streaks, lacquer cracks, wet-amd
What condition has an increased prevalence of lattice and RD?
transillumination defects associated with PSD.
RRD symptoms
flashes of light, floaters or recent onset or many, curtain, loss or blurring of vision.
Subclinicical RRD
A retinal break with surrounding sub retinal fluid extending at least 1DD from the break but no more than 2DD posterior to the equator.
Types of RRD
- subclinical 2. retinal break without detachment (both can lead to clinical) 3. Clinical
Types of Clinical RRD
Macula off, macular on and not likely to detach, macula on and likely to detach
Do RRD progress normally?
No most do not. The distance of the detachment from the fovea was the only risk factor for foveal detachment.
Management for RD
Silicone oil, scleral buckle, laster photocoagulation, aspiration of sub retinal fluid, pneumatic retinopathy (gas bubbles), primary vitrectomy.
What else does scleral buckle cause?
Myopic shift.
Retinoschisis Description
Fluid filled and cyst like, relatively immobile, blanches when depressed, see choroidal detail, sheathed vessel possible, honeycomb appearance of the outer wall possible, absolute scotoma.
Where is retinoschsis most commonly found?
bilateral inferior temporal. Next is superior temporal.
Retinoschsis incidence
3.7% patients. Increasing with age and hyperopia. F>M
Pathophysiology of retinoschsis
Retinal splitting between inner plexiform and outer nuclear layers. Inner layer is stretched and thinned. No demarcation line expected.
Clinical course of retinoschsis
Progress very slowly. Associated with holes in inner or outer layers. Very low potential for RD
MGMT of retinoschsis
DDX from RD. If within 25 degrees of temporal arcades FU q6m. Laser only if macular area in danger of inner and outer holes present.
CHRPE vs. Nevus
Deeper and will leave with red free.
How many Nevus convert to melanoma?
1/9,000
Risk factors for cancerous nevus acronym
To find small ocular melanoma using helpful hints daly
Risk factos for cancerous nevus
Thickness: >2 mm Fluid: Subretinal Symptoms: phopsia, Vision loss Orange Pigment overlying lesion Margin touch ONH Ultrasound hollowness Halo absesnse Druse absence.
How to monitor nvus
No features initially monitored twice yearly and followed up annually. 1/2 features should be monitored every 4-6m. Nevi with 3+ should be evaluated at an experienced center for management alternatives and possible treatment .
Where does a primary choroidal melanoma tend to metastasize to?
Liver. Won’t survive this. This is death. Less common sites are skin and lung.
CHRPE
Unifocal or multifocal. Unifocal CHRPE results in pigmented, flat, round lesions with distinct margins. No change in color with depression. Stable in size but their color may change. Full or partial hypo pigmentation ring seen around the lesion.
What finding is diagnostic for CHRPE
Halo
Where are CHRPEs normally found
Most occur in temporal funds.
Multifocal CHRPE
AKA bear tracks. Presence of a number of small CHRPE lesions. Associated with gardner’s syndrome.
Avulsed vitreous base
Vitreous base will appear as floating white strip and can be twisted
What causes avulsed vitreous base
Due to blunt trauma. Vitreous base has been pulled away from ora.
Who is avulsed vitreous base most common in
young
Where is avulsed vitreous base found
usually superior nasal.
Clinical course with avulsed vitreous base
No changes. Associated with vitreous hemorrhages or RD
MGMT of avulsed vitreous base
No treatment unless RD.
Oral Pearls
Usually appear as white glistening round object on dark background. Can be pigmented. Only seen with scleral depressing.
Incidence of oral pearls
20% of eyes
Location of oral pearls
On pars plana
Cause of oral pearls
Druse like structures beneath dentate processes may be large and lose their pigment epithelial covering.
MGMT of oral pearls
Benign incidental finding. No associated findings/risks.
Pars Plana Cysts
Looks like blisters. Increased with scleral depression.
Pars Plana cysts incidence
7% population. Increases with age. Acquired and not congenital. More commonly found with RD and posterior uveitis.
Location of pars plans cysts
Most likely temporal.
Cause of pars plans cysts
separation of non pigmented and pigmented epithelium.
Tx for pars plana cysts
no mgmt
Neoplams
New or abnormal growth. Growth is uncontrolled and progressive
Melanoma
Tumor made up of melanin pigmented cells. Mestatic.
Hamartoma
benign tumor like nodule. Composed of cells and tissue that don’t normally occur in that tissue.
Blastoma
Neoplasm composed of embryonic cells of the tissue/organ
Carcinoma
Cancer that begins in the skin or in tissues that line or cover the body organs.
Enhanced depth imaging OCT
Allows you to image deeper into the retina.
Choroidal Nevus
Benign choroidal neoplasm. Increased pigment in choroid. Typically flat or minimally elevated. May see overlying druse. May have serous retinal detachment over nevus. Probably present at birth and grows maximally during prepubertal years (rare to grow after that)
How many people have a choroidal nevus
30%
Choroidal Nevus and malignancy
Can convert. When under 2DD 95% are benign. Document and follow up.
A choroidal lesion will _______ with a red free filter
disappear
A choroidal lesion will _____ with an infrared filter on
enhance
What does drusen over nevus indicate?
Long standing
What size should a benign nevus be
2-5 DD
How often to follow up with a nevus?
Biannual
When to suspect malignancy
5 DD +, elevation, druse that is more orange, may see feeder vessels.
What additional tests to do with nevus that may be malignant?
ultrasound, FAs, photo documentation, P32.
What is the most common adult primary intraocular malignant tumor
malignant choroidal nevus
What population very rarely gets malignant choroidal melanoma
African americans
Circumscribed malignant choroidal melanoma
More common, better prognosis. Highly elevated, grayish-green, mottled with brown, black, orange. Associated with serous RD, VH, and proptosis.
Diffuse malignant choroidal melanoma
Less common, worse prognosis. Horizontal growth pattern, poorly defined edges.
Ocular findings associated with malignant choroidal melanoma
Hard exudates, serous detachment of the retina, choroidal folds, sub retinal and intraretinal hem, literal hem, secondary glaucoma, cataracts, anterior and/or posterior uveitis.
Sx of Malignant choroid melanoma
May be asymp. May have reduced VA, may or may not have VF defect, photopisa/floaters.
Secondary Malignant choroidal melanoma common causes
Males-lung, liver cancer. Females-breast.
Primary lesions are often _____, while secondary are often____
unilateral, bilateral.
Performing transillumination of sclera for lesion
Pigmented lesions will not transmit the light. Non pigmented will transmit the light.
Diagnosing malignant choroid melanoma
Serial retinal photographs (monitor change), FA, B-scan, P32 uptake.
B-Scan and malignant choroidal melanoma
B-scan ultrasonography will show the mass. Retinal detachment can often occur over the melanoma and hide it. B-scan will reveal it.
P32 test
Perform on large tumors. The uptake of P in malignant cells is greater than in normal cells.
Enucleation for choroidal malignant melanoma
Perform on large tumors with functional vision loss or growth around ONH. Fit prosthesis 5-12 weeks later.
Radioactive plaques
Option for malignant choroidal melanoma. Gamma radiation. Plaque stitched to tissue for several days. Can cause retinopathy, cataracts, and vitreous hem.
Transpupillary thermotherapy
Diode laser delivering radiation through the pupil. Most effective at tumor apex
Combination TTT/Plaque
Sandwich therapy for malignant choroidal melanoma
Which malignant choroidal melanoma lesions would you must observe
Small tumors (less than 3 mm thick and 10 mm in diameter) Have low metastatic potential.
Local resection
Lots of complications. Not a good choice
Heavy charged particle irradiation
External beam. Proton bean irradiation. Uses cyclotron generated heavy ions. Can be used on large tumors. Limited availability.
Systemic problems with malignant choroidal melanoma
Can spread to other organs. Males-lung, liver. Females-breast cancer.
A bening nevus will _____ with FA
Hypofluoresce
A malignant nevus will ____ with FA
Hyper
Monosomy 3
Only one chromosome 3. Poor prognosis. Testing for choroidal melanoma
Class 1 melanoma
Two chromosome 3 copies, low metastasis risk
Class 2 melanoma
Only one chromosome 3, high metastasis risk.
BAP1
BRCA-Associated Protein 1 mutation or inactivation. BRCA is breast cancer. Also associated with lung cancer from asbestos. Often found in choroidal tumors with a higher risk of metastasis.
Choroidal Osteomas
Acquired, slow-growing intrachoroidal calcification near ONH. Benign but has potential for growth. Bone-like tumor.
What percent of choroidal stemmas are unilateral
75%
What does a choroidal osteoma look like?
Yellow white to orange red lesion with scalloped edges
Choroidal ostema on A vs B scan
A=reflective B=elevated
Tx for choroidal ostema
Monitor. Prognosis variable and unpredictable. Can cause vision loss.
Ciliary Body Malignant Melanoma
May involve pigment or non pig. epithelium or storm. Hard to see and normally detected once big enough to grow into pupil.
How to detect CB malignant melanoma
Pressure on lens may breast secondary irregular astigmatism, corectopia, anterior dislocation of lens, focal opacity of th lens, focal dilation of episcleral vessels, glaucoma, RD. Erosion of iris root and forward extension of tumor into the anterior chamber. Erosion of the sclera with extension of the tumor outward. Anterior uveitis.
Tx for CB malignant melanoma
Enucleation (if large and spread to anterior choroid). Sector reaction for small to medium.
Melanocytoma
ONH tumors.
Melanocytoma characteristics
Dark black, elevated mass on the ON. Usually found in darker skin. Unilateral. Growth may occur over 5-20 years. May spread off the disc.
How much of ON is normally involved in a melanocytoma?
Usually 50% of less of the ON is involved. Usually the inferior part.
____ have no vision reduction with melanocytoma
75%
TX for melanocytoma
None. Photodocument/VF. Routine follow up.
Mestatic Carcinoma of the choroid
Secondary Tumor. The eyes may be one of the first sites of malignant spread.
Primary tumor that travels to the eyes are common from where
lung breast, kidneys, GI.
Mestatic carcinomas of the choroid description
Can occur anywhere but common in posterior pole. Flat or slight elevation, placoid lesions. Creamy-white to yellow to gray.
Metastatic carcinoma of the choroid objective
multifocal or solitary. Grows faster than malignant melanoma. Surface has characteristic mottled pigment clumping. Extensive exudative RD. Pain may be associated.
Prognosis with me static carcinoma of the choroid
Prognosis is poor. enucleation is more conservative. chemotherapy, irradiation, photocoagulation, cryotherapy, irradiation, sector excision may be used.
Metastatic carcinoma of optic nerve
Very rare to have direct metastasis to ON.
Metastatic carcinoma of ON symptoms
loss of VAs
Mestatic carcinoma signs
swollen nerve her, yellowish tumor above the normal ON. Venous tortuosity or CVO
Prognosis with metastatic carcinoma
Mean survival 10 month
TX for metastatic carcinoma
Irradiation/chemo
When to enucleate with metastatic carcinoma?
If pain from secondary uveitis or intractable glaucoma is severe
Leukemia
Infiltration of retina, choroid, and ON. Infiltration into ON displaces normal neurons and causes Vision loss. Occurs in terminal stage of disease. Survival rate less than 12 m.
TX for leukemia
Irradiation may delay or abort the n. destruction
what is the most common primary ocular tumor in childhood
retinoblastoma
Retinoblastoma
Malignant, congenital intraocular tumor. Mostly dx by age 4. (1/4) bilateral and (3/4) unilateral.
Origin of retinoblastoma
Undifferentiated retinal neural tissue. Present at birth and develops during first 2 years.
Average number of tumors with retinoblastoma
Average of 5. Metastasis by blood stream. Mortality in 18%
Genetics with retinoblastoma
AD, high penetrance. 6% of all cases. Most are sporadic in origin. 25% can be passed on. Should do genetic counseling.
Subjective retinoblastoma
white pupil, leukocoria (2/3 present in this way) Poor VA, infant may rub eyes
Retinoblastoma Signs
No pigment, dull chalky white lesion. High calcium content so reflective on ultrasound. Hyper fluoresce during FA.
TX for Retinoblastoma
Enucleation (large tumors), photocoagulation (small) Brachytherapy, irradiation (medium to large), cryotherapy (small peripheral tumors)
Embryonic Medulooepithelioma
CB equivalent to retinoblastoma. Involves non-pigmented layer. Unilateral most common.
Presenting signs with embryonic medulloepithelioma
Usually leukocoria. Usually in first decade of life.
Signs with medulloepithelioma
Poor vision. May have pain
Objective with medulloepithlioma
white mass in pupil, may find corresponding mass in the iris and/or anterior chamber.
TX with medulloepithelioma
Sector extraction, many times too late to enculeate.
Adult medulloepithelioma
A carcinoma. Multiple white to gray fluffy mass of inner surface of ciliary body. usually associated with trauma to the eye or post inflammation.
Prognosis with adult medulloepithelioma
Growth rate is slow. Locally invasive. Sector if small. Enculcleate if large.
CHRPE
Are of enlarged RPE cells.
Prevalence of CHRPE
1.2-4.4%
CHRPE objective
single or multiple presentations. Dark gray in color. Well demarcated. Flat but may have an area of hypo pigmentation surrounding (halo Nevi). Area may show a scotoma.
What is multiple CHRPE called
bear track or animal track
Will CHRPE disappear with red free?
NO
Tx with CHRPE
Document, monitor changes. Follow up with routine eye exam.
CHRPE and FAP
CHRPE connected to familial adenomatous polyposis. FAP is AD disorder. 4 or more lesions is specific.
What causes Retinal pigment epithelial hyperplasia
Occurs due to insults or trauma to retina, chorioretinal inflammations, scars, or choroidal neovascarulization. Occurs by invasion of RPE into sensory retina.
Tx of Retinal Pigment epithelial Hyperplasia
Non-progressive if underlying cause not active. Treat the cause and monitor for change.
Retinal Pigment epithelial Hyperplasia appearance
Appears as a jet black irregular pigment. Size and shape varies.
Cerebroretnnal Angiomatosis (von Hipple-Lindau dz)
Predisposes pt to benign and malignant tumors. Angiomatosis of retina, CNS, and visceral organs. Vascular tumors.
Inheritance with Cerebroretnnal angiomatosis
AD with variable expression.
Onset of cerebroretinal angiomatosis
30s, No racial or sexual predilection.
Ocular manifestation with cerebroretnnal angiomatosis
Tumors start slow and enlarge. Tumors in vessels and arteries. More common in mid-peripheral retina but may be found anywhere in the ONH.
How many of patients with cerebroretnnal angiomatosis have ocular manifestations?
25%
How often is cerebroretnnal angiomatosis bilateral
50%
Ocular complications with cerebroretnnal angiomatosis
As the tumor enlarges it tend to leak hemorrhages and hard exudates. Can cause secondary glaucoma and RD.
Ocular tx for cerebroretnnal angiomatosis
Depends on location and size. Irradiation, photocoagulation, cryotherapy.
Systemic tx for cerebroretnnal angiomatosis
annual physical, renal analysis, MRI/CT q3 years.
Encephalotrigeminal Angiomatosis (Sturge weber)
Intracranial angioma, facial angioma, and choroidal angioma. Usually ipsilateral and present at birth. Inheritance pattern unclear and no sexual or racial predilection.
Cutaneous angioma with enchephalotrigeminal angiomatosis
Nevus flammeus or port wine stain. Present at birth.
Intracranial lesion with encephalotrigeminal angiomatosis
Can have epileptic seizes or VF defects, etc.
Choroidal Angioma
With encephalotrigeminal angiomatosis. A choroidal hemangioma. Can be localized or diffuse.
Localized choroidal angioma
Occurs with storage-weber. Yellow to red orange lesion. Elevated circular area. May cause serous retinal detachment
Diffuse choroidal angioma
More common in storage-weber. Entire funds is deep red. Might miss unless compare to other eye.
Other ocular manifestations with encephalotrigeminal angiomatosis
Primary concern is congenital glaucoma, 30% develop glaucoma. Heterochromia iridis. Conj. vessels show dilation and are tortuous.
Dx of encephalotrigeminal angiomatosis
Ultrasound shows choroidal thickening but no excavation or orbital involvement. FA shows the large choroidal vessels.
TX for encephalotrigmeinal angiomatosis
control seizures, tx for glaucoma, PDT for choroidal hemangioma, photocoagulation for RD.
Arteriovenous malformations
Not a true phacomatoses. Involves the retina and CNS with A/V malformations (dilated and tortuous retinal arteries and veins). Embryonic origin.
Ocular manifestations with arteriovenous malformations
Usually unilateral. Enlarged tortuous and frequently more vessels. May involve single vessel, one area, a single quadrant, or the entire fundus.. Arteries and v are similar in appearance. May project forward from the retina. Large lesions can cause hemes. Normally asymp.
Systemic manifestations of Arteriovenous malformation
lesions in brain. May cause epilepsy.
Tx for arteriovenous malformation
Congenital has no progression. Retinal AVMs (especially if continuous with ON) referred for neurological consult.
Tuberous Sclerosis (bourneville’s dz) epidemiology
No race or gender preference. Auto dominant. 50% new mutations.
Sebaceous Adenoma (pringle’s Dz)
cutaneous manifestation of tub. sclerosis. Angiofibroma. Begins about age 5. Pale pink, usually around the nose and lip area. Lesions vary in size. Highly vascularized red papular.
Achromatic nevi
Cutaneous manifestation of tub. sclerosis. Congenital white patches. Hypo pigment. Usually on trunk or limbs.
Shagreen patch
tub. sclerosis cuatnous manifestation. Diffuse fibrous thickening of the skin. This lesion has an elevated orange peel appearance. May find small fibroma at the fingernail side.
Ocular manifestation of tub. sclerosis
Small tumors on conj, hypo pigmentation iris spots, may see retinal lesion (Astrocytoma)
Astrocytoma with tub. sclerosis
50% of those with t.s. usually unilateral but bilateral (15%). Astrocytoma arising from inner layers of retina.May be white or multiple. Gray-white. usually located on the post. pole near the ON. Can be found anywhere. Raised and nodular (mulberry). Druse of ONH associated. Slow growth. May break off and mestasisize.
SE with astrocytoma
VF defect.
Tx for astrocytoma
tx symptoms. Refer if papilledema.
Neurofibromatosis (von Recklinghausens disease)
Congenital but may not be seen until late childhood. Autosomal dominant with varied expression.
Neurofibromatosis cutaneous manifestations
Appearance of Cafe spots. Hyper pigmentation areas. 6 or more think this disease. Plexiform neurofibromas-subcutanous tumors that are diffuse. Fibroma molluscum-skin tumor appear as small subcutaneous nodules over the entire body.
Systemic manifestations with Neurofibromatosis
CNS: multiple tumors in brain. Bone: abnormal spinal and skull Visceral: can include neurofibroma of all organs
Ocular: neurofibromas can be found in the ocular tissue.
Ocular manifestation in Neurofibromatosis
Partial to complete ptosis of the lid. Produces S shaped lids. Proptosis due to tumor (may be pulsating due to absence of sphenoid). Creators heterochromia. Can be tumors in conj, cornea, and iris. Can also have retinal and choroidal tumors but are more rare.
Associated conditions with Neurofibromatosis
Congenital, unilateral glaucoma is associated.
Tx for Neurofibromatosis
Surgical removal of the tumors. Prognosis is variable. Optic n. gliomas may respond to irradiation.
