Choroidal dz Flashcards
Choroidal hemangiomas
Choroidal hemangiomas= benign and never metastasize. Therefore, systemic chemotherapy is never indicated. The most viable current treatment for these benign tumors is PDT.
In cases unresponsive to PDT, radiation therapy (e.g. brachytherapy or external beam radiation) may be employed. Small choroidal hemangiomas causing no visual symptoms may be observed with serial fundus photographs.
Gyrate atrophy vs choroidemia
Both gyrate atrophy (AR) and choroideremia (XR) have diffuse loss of the RPE and choroid. Neither disease features retinal vessel attenuation unlike patients with retinitis pigmentosa.
One helpful fundus feature that helps to distinguish gyrate atrophy from choroidermia is HYPERPIGMENTATION of the remaining RPE in gyrate atrophy.
Choroidal mtz
Usually flat and ill-defined, yellow-white to gray-yellow in color. Can be assoc/w/overlying RPE changes in a “leopard spotting” pattern.
(unlike choroidal melanoma which presents with a “mushroom configuration”)
Most commonly from?
Breast (in women)
Lung (in men)
Usually flat growth pattern (not collar-button / mushroom-shaped)
Choroideremia
X-linked recessive
– Males 1st or 2nd decade
– Carrier women – fundus pigment changes only
• Usually presents with nyctalopia
• Near total absence of choroid, choriocapillaris, RPE
• Early abnormal ERG
• Poor prognosis (VA < 20/200 by age 50)
CHRPE & systemic association
Congenital hypertrophy of the RPE
Benign
Gardner Syndrome
Intestinal polyposis & fish-shaped CHRPE
CHRPEs are usually small, ovoid, variegated, multiple, bilateral
Inheritance: AD
Choroidal Osteoma
Benign
May mimic melanoma
Peripapillary or macula
Test?
B-scan shows high-amplitude echo with posterior shadowing
May develop CNV
Very slowly progressive
Sclerochoroidal Calcification
Yellow placoid appearance
Test?
auto-fluorescent
FA: early blockage with late staining
B-scan shows high-reflectivity
Maybe associated with? Hyperparathyroidism Vitamin D intoxication Gitelman/Bartter dx Renal disease
Choroidal Melanoma Risk factors
Risk factors Thickness >2mm Fluid (SRF) Symptom Orange (pigment) Margin (near optic nerve) Hollowness (on B-scan) Halo (absence) Drusen (absence)
To Find Small Ocular Melanomas Using Helpful Hints Daily
Risk of nevus conversion to melanoma
0 factor: 3% at 5 yrs
1 factor: 38% at 5 yrs
2+ factors: >50% at 5 yrs
Factors predicting survival?
Size of scleral contact and cell type
Mean of 10 largest melanoma cell nuclei (MLN)
Monosomy 3 (correlates with mets)
Choroidal Melanoma Dx and Px
Ultrasound? Low reflectivity Callender classification? Spindle cell nevus Spindle A cells Spindle cell melanoma
Best prognosis
Mix of spindle A & B cells
25% 15-year mortality
Mixed
50% 15-year mortality
Epithelioid melanoma
Worst prognosis
Epithelioid cells
75% 15-year mortality
Collaborative Ocular Melanoma Study (COMS) - large
Large (>10mm height, >16mm dia)
Enucleation = pre-XRT + enucleation
Collaborative Ocular Melanoma Study (COMS) - Medium
Medium (2.5-10mm height, <16mm dia)
PBT = enucleation in 5 yr mortality
Collaborative Ocular Melanoma Study (COMS) - small
Small (1.5-2.4mm height, 5-16mm dia)
Detailed risk factors for growth
Choroidal Melanoma Risk factors (outside COMS), mtz, sentinel vessel?
Risk factors?
Melanosis oculi, nevus of Ota
Mushroom-shaped when tumor breaks through Bruch’s membrane
Glaucoma develops from liberation of melanin clogging TM
Sentinel vessel?
Sign of ciliary body melanoma
Metastasis to?
Hematogenous to liver
Uveal Effusion Syndrome Causes & associations?
Causes?
Idiopathic
Scleritis
Nanophthalmos
Associated with? Hyperopia, glaucoma FA findings? Leopard spots Usually no leakage
Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP) and assoc/FA/Rx?
Paraneoplastic diffuse thickening of choroid with discoloration
Look like large nevi
Cancer association?
Ovarian, uterine, & lung CA
FA findings?
Leopard spots
Treatment?
Intravitreal rituxamab
