Congenital Anomalies of the Retina – Flashcards
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Only when opaque (mechanical problem)
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Do myelinated retinal nerve fibers cause vision loss?
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Myelinated nerve fibers
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In which condition may vessels be partially or completely obscured: myelinated nerve fibers or ischemic events?
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*Plasia - increased NUMBER of cells; pigment in RPE has been dispersed (see clumping of cells)* *Trophy - increased SIZE of cells; cells don't nourish receptor layer well enough to give you good vision*
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*Hyperplasia vs hypertrophy*
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Normally infectious Possibly trauma
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What causes hyperplasia?
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Mechanical (stretch) or trophic (growth factors) signals (think of it like your muscles getting bigger when you work out - the cells get bigger; they don't increase in number)
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What causes hypertrophy?
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-plasia
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Which causes reduced acuity: -plasia or -trophy?
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-trophy (relative scotoma)
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Which causes scotoma: -plasia or -trophy?
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Maybe, but no treatment
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Does CHRPE have associated field loss?
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Bilateral CHRPE, typically "bear tracks" Autosomal dominant Intestinal polyps typically progress to adenocarcinoma (bowel cancer; if seen in kid, basically 100% chance)
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Gardner's syndrome
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Hyperpigmented - dark brown to black
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CHRPE: color
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Geographic/sharp
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CHRPE: borders
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Lacunae
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CHRPE: associated findings
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Flat
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CHRPE: elevation
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Recurrent toxoplasmosis
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Hx of toxo
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Collection of melanocytes (benign "melanoma")
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Nevus definition
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Typically under 2 DD Can be up to 5 DD
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Choroidal nevi: size
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Fuzzy, indistinct
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Choroidal nevi: borders
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Gray to green May be amelanotic (yellow to brown)
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Choroidal nevi: color
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Drusen
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Choroidal nevi: associated findings
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Moderate hypo-reflectivity
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Choroidal nevi: B scan
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Flat to slightly elevated
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Choroidal nevi: elevation
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Well-demarcated
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Choroidal osteoma: borders
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Pale yellow
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Choroidal osteoma: color
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Hyper-reflective
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Choroidal osteoma: B scan
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Melanoma
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Most common primary tumor of the eye
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Uveal melanoma
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Most common primary malignant intraocular tumor in caucasians
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Caucasians Men 55-60
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Uveal melanoma: demographics
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Liver 5-7 months
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What is the most common site for extraocular metastasis from ciliary body and choroidal melanoma, and what is the prognosis?
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Failure of RPE function; *degeneration of RPE* (Pigment in choroid is displacing the fluid that is leaking from vessels used to nourish RPE)
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What does it mean when you see drusen?
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Good with nevi because it means the lesion has been around a long time and hasn't grown (benign)
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When is it relatively good to see drusen?
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Increasing Most > 10 DD at diagnosis
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Choroidal malignant melanoma: size
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Fuzzy, indistinct
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Choroidal malignant melanoma: borders
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Significantly elevated
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Choroidal malignant melanoma: elevation
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*Lipofuscin* (look more orange than drusen) Sentinel vessel
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Choroidal malignant melanoma: associated findings
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*Acoustically hollow* (hypo-reflective) Dome-shaped
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Choroidal malignant melanoma: B scan
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Full body MRI Blood work Check for metastasis
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Choroidal malignant melanoma: treatment
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*Choroidal melanoma*
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*What would you suspect if you observe a large conjunctival blood vessel?*
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-Cancer center for evaluation of mass -Full body MRI or CT (focus on liver) -Complete physical -Blood workup including CBC and liver function studies
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If it is melanoma, how do you refer?
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Removal of contents of the eye (leave sclera intact)
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Evisceration
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Removal of the eye, including the globe (leave the rest of the orbital contents, including bones, muscles, fat, and conjunctiva)
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Enucleation
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Removal of all orbital contents, including muscles, lacrimal gland system, optic nerve, and varying parts of the bone (may or may not remove eyelids)
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Exenteration
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Benign vascular lesion ("vascular birthmarks")
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What is a haemangioma?
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Diameter, height, ultrasonographic hollowness, extrascleral extension
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What can you learn from B-scan ultrasonography?
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Diameter, monitoring for growth
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What can you learn from digital photography?
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Useful for documentation, not so much diagnosis Drusen stain, melanomas have pooling
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What can you learn from FA?
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Least aggressive, best outcome
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Uveal melanoma prognosis: spindle cell A
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More aggressive than spindle cell A
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Uveal melanoma prognosis: spindle cell B
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Most aggressive, high malignancy
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Uveal melanoma prognosis: epithelioid
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Intermediate prognosis
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Uveal melanoma prognosis: mixed cell
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*Ciliary body melanoma* *(sentinel vessel)*
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*What would you suspect if you observe a single large vessel that seems to disappear into the conjunctiva?*
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*Iris melanoma*
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*Of the three types of uveal melanomas, which type is the rarest?*
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*Ciliary body melanoma* More likely to be composed of epithelioid melanoma cells, larger at time of detection, highly malignant
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*Of the three types of uveal melanomas, which type has the poorest prognosis?*
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Solid, acoustically dark mass
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How does ciliary body melanoma appear on B-scan?
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Low risk of dying in 5 years (just photograph and follow - no treatment)
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COMS findings: small tumors
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Equal mortality rates between enucleation and iodine-125 brachytherapy (neither treatment is likely to increase or decrease mortality rates)
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COMS findings: medium tumors
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Equal mortality rates between radiation (PERT) + enucleation and enucleation alone
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COMS findings: large tumors
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YERVOY (ipilimumab)
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What is the only treatment that may be effective against metastatic choroidal melanomas?
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*DIFFUSE choroidal hemangioma*
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*What is congenital anomaly associated with Sturge-Weber syndrome?*
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Nevus = benign, not optic nerve Melanoma = malignant Melanocytoma = benign, optic nerve
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What's the difference between a nevus, a melanoma, and a melanocytoma?
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ON coloboma (otherwise looks like hypertrophic RPE with lacunae)
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This patient has a keyhole pupil
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Tilted disc
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Tilted disc or oblique insertion?
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Tilted disc
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Tilted disc or oblique insertion?
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Oblique insertion
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Tilted disc or oblique insertion?
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(most to least important) 1. Gestational age 2. Weighted oxygen 3. Intraventricular hemorrhage 4. Sepsis 5. Birth weight (particularly ; 3.5 lbs)
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Risk factors for retinopathy of prematurity
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ROP stage 1 (line of demarcation separates the more nasal vascular zone from the more temporal avascular zone)
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What is this stage of ROP?
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ROP stage 2 (line of demarcation becomes a ridge that protrudes into the vitreous - histological evidence of an A/V shunt)
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What is this stage of ROP?
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ROP stage 3 (neovascularization, as a result of anoxia, occurs along the ridge)
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What is this stage of ROP?
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Scarring and fibrosis can occur when the neovascularization extends into the vitreous
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Describe ROP stage 4 (he didn't have an actual picture)
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ROP stage 5 (total retinal detachment)
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What is this stage of ROP?
