2. Choroidal Melanoma
• Is the most common primary malignant instraocular tumor and the second
most common type of primary malignant tumor in the body.
• It most often aﬀects whites of northern European descent.
Choroidal melanoma may present with the following symptoms:
• Blurred visual acuity
• Paracentral scotoma
• Painless and progressive visual ﬁeld loss
• Severe ocular pain
• Weight loss, marked fatigue, cough, or change in bowel or bladder habits.
Signs and Symptoms
3. Ophthalmologic examination
• Small choroidal melanomas (nodular, dome-shaped, and well-circumscribed
mass under the retinal pigment epithelium)
• As choroidal melanomas grow, may adopt more irregular conﬁgurations
(eg, bilobular, multilobular, or mushroom shapes).
• Diffuse choroidal melanoma, characterized by lateral growth throughout the
choroid with minimal elevation.
• May have coloration, from amelanotic to darkly pigmented; some are
• Overlying the choroidal melanoma, there are usually retinal pigment
epithelial changes (eg, drusen), patches of atrophy, and orange
• Choroidal melanoma may remain undetected underneath a large exudative
retinal detachment, a subretinal hemorrhage, or a vitreous hemorrhage.
4. • Infrequent presentations are a painful blind eye with cataract and proptosis
from tumor transscleral orbital extension.
• Anterior choroidal melanomas might show sentinel vessels that feed the
metabolically active tumor.
• Transscleral growth of an anterior choroidal melanoma may appear on
examination as a small subconjunctival area of abnormal hyperpigmentation.
• Liver enzyme levels are indicated in any patient with uveal melanoma. The
most sensitive tests of hepatic function are serum levels of the following:
• Alkaline phosphatase
• Glutamic-oxaloacetic transaminase
• Lactate dehydrogenase
• Gamma-glutamyl transpeptidase
• A-scan ultrasonography.
• B-scan ultrasonography.
• A-scan ultrasonography of the eye is useful for tumors thicker than 2-3 mm.
• Choroidal melanoma characteristically shows an initial prominent spike,
followed by low-to-medium internal reﬂectivity with diminishing amplitude
and a signiﬁcant echo.
• Small choroidal melanomas may show such changes ranging from normal
angiography to hypoﬂuorescence secondary to blockage of background
• Larger melanomas may show a patchy pattern of early hypoﬂuorescence
and hyperﬂuorescence followed by late intense staining.
10. • Observation; in particular, tumors of less than 2-2.5 mm in elevation and 10
mm in diameter.
• Enucleation is the classic approach to choroidal melanomas; for large (basal
diameter >15 mm and height >10 mm) and complicated tumors.
• Plaque brachytherapy is a widely accepted alternative to enucleation for
medium-sized posterior uveal melanomas (< 10 mm in height and < 15 mm in
• External beam irradiation with protons or helium ions is a frequently used
alternative for the treatment of medium-sized choroidal melanomas.
• Pars plana vitrectomy endoresection.
• Block excision (sclerouvectomy) is an alternative treatment method
reserved for small tumors covering less than one third of the globe’s
• Laser photocoagulation and transpupillary thermotherapy are used to
treat selected small choroidal melanomas that are located away from the
fovea and are less than 3 mm in thickness.
• Orbital exenteration.
11. Stargardt disease and Fundus ﬂavimaculatus
• Stargardt disease (juvenile macular dystrophy) and fundus ﬂavimaculatus
(FFM) are regarded as variants of the same disease despite presenting at
diﬀerent times and carrying diﬀerent prognosis.
• Characterized by diﬀuse accumulation of lipofuscin within the RPE that
results in vermilion fundus and a ‘dark choroid’.
• Presentation is in the 1st-2nd decades with bilateral gradual impairment of
central vision which may be out of proportion to the macular changes.
• Some present in adult life.
13. Flecks have the following
• Bilateral yellow-white lesions
at the level of the RPE; of
various size and shape, such
as round, oval or pisciform
• Distribution may be at the
posterior pole exclusively or
e x t e n d i n g t o t h e m i d -
Stargardt disease and Fundus ﬂavimaculatus
• Macula may be initially normal or show non-speciﬁc mottling.
• An oval ‘snail-slime’ or ‘beaten-brozen’ appearance.
• Geographic atrophy that may have a bull’s eye conﬁguration.
14. a. FA shows macular hyperﬂuorescence and a ‘dark’ choroid.
b. FA shows hyoperﬂuorescent spots.
c. Indocyanine green angiography (ICGA) shows hypofuorescznt spots.
Stargardt disease and Fundus ﬂavimaculatus
16. Congenital Hypertrophy of the
Retinal Pigment Epithelium
• Congenital retinal pigment epithelial hypertrophy is usually found before
patients reach 30 years ago.
• They may enlarge with time, but are not malignant.
• CHRPE Associated with Gardner’s Syndrome (familial colonic polyposis).
• In contrast, acquired retinal pigment epithelial hypertrophy is typically found
later in life.
• They are typically jet-black to gray, ﬂat, with a halo around its edges. Variable
in size, RPEH lesions may develop lacunae of lightly colored areas of atrophy.
• Almost all patients with retinal pigment epithelial
hypertrophy do not have symptoms.
• These pigmented intraocular lesions are found
during fundus examination.
• RPEH lesions tend to be black or atrophic. They may be surrounded by a halo
of less pigmented tissue or exhibit a sharp demarcation line.
• Ultrasonography typically shows that RPE hypertrophy is ﬂat to minimally
elevated and slightly hyper-reﬂective.
19. • Fluorescin angiography of RPE hypertrophy typically demonstrates
blockage of ﬂuorescence (except in areas of atrophy which are
A, (Lightly pigmented congenital hypertrophy of the retinal pigment epithelium (RPE) with several lacunae).
B, Fundus autoﬂuorescence imaging shows hypoautoﬂuorescent pigmented areas and isoautoﬂuorescent lacunae.
C, Infrared reﬂectance imaging shows hyporeﬂectivity of the lesion and hyperreﬂective lacunae. An arrow points to
a lacuna that the raster line passes through.
D, Enhanced-depth imaging optical coherence tomography of ﬂat congenital hypertrophy of the RPE (CHRPE) with
thickened, irregular RPE.
20. • Optical coherence tomography (OCT) of RPE hypertrophy will demonstrate
both thickening and thinning. The overlying retina is thinned, the retinal
pigment epithelium is both thickened or can be thinned. The underlying
choroid is typically thinned.
• Ultrasonography and ﬂuorescein angiography is typically used to diﬀerentiate
RPE hypertrophy from uveal melanoma and certain rare intraocular tumors.
Serial observation is warranted in that RPE hypertrophy can enlarge over
22. Choroidal Nevus
• A choroidal nevus (plural nevi) is a ﬂat, benign, colored spot that appears
on the inside of the eye, similar to freckles or moles on the skin.
• A choroidal nevus refers speciﬁcally to a pigmented mark that forms in the
• It is caused by melanocytes.
• Nevi usually never causes symptoms.
• Common, Caucasian population (5-10%) of the population.
• Rare in African Americans.
23. Some nevi have features include:
• Tumor thickness greater than 2.0 mm
• Presence of subretinal ﬂuid
• Visual symptoms
• Presence of orange pigment
• Absence of drusen
• Absence of retinal pigment epithelial change adjacent to nevus
• Nevus margin touching the optic disc.
• Rarely causes symptoms.
• However, if a choroidal nevus leaks ﬂuid or is associated with the growth of
abnormal blood vessels (neovascularization) patients can become
symptomatic. Such changes can cause a localized retinal detachment/
degeneration, ﬂashing light and loss of vision.