Retinal Pigmented Fundus

1. Pigmented Fundus
Lesion
dr. Frenky R. de Jesus
National Eye Centre
Postgraduate diploma in Ophthalmology

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
• Floaters
• 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
partially pigmented.
• Overlying the choroidal melanoma, there are usually retinal pigment
epithelial changes (eg, drusen), patches of atrophy, and orange
discoloration.
• 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.
Ophthalmologic examination

5. Diagnosis
• 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
Ultrasonography
• A-scan ultrasonography.
• B-scan ultrasonography.

6. 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.
Diagnosis

7. Ultrasonography
• B-scan ultrasonography.
• To evaluate possible extraocular extension, to estimate tumor size for
periodic observation, and to plan therapeutic intervention.
Diagnosis

8. Angiography
• Small choroidal melanomas may show such changes ranging from normal
angiography to hypofluorescence secondary to blockage of background
fluorescence.
• Larger melanomas may show a patchy pattern of early hypofluorescence
and hyperfluorescence followed by late intense staining.
Diagnosis

9. Imaging studies
• Chest x-ray.
• CT scanning of the globe and orbit.
• MRI of the globe and orbit.
Diagnosis

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
diameter)
• 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
circumference.
• 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.
Management

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
characteristic;
• Bilateral yellow-white lesions
at the level of the RPE; of
various size and shape, such
as round, oval or pisciform
(fish-shaped).
• 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 -
periphery.
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

15. Management
• No treatment.
• No smoking or be around cigarette smoke.
• Avoid taking large doses of it as a supplement.

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.
Signs and
Symptoms

17. Congenital Hypertrophy of the
Retinal Pigment Epithelium

18. Diagnosis
• 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
hyperfluorescent.
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.
Diagnosis

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.
Diagnosis

21. Treatment
• 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
time.

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
choroid.
• 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.
Symptoms
Choroidal nevus

24. • Ultrasound:
Diagnosis

25. • Fluorescent angiography:
Diagnosis

26. • OCT:
Diagnosis

27. • OCT:

28. Management
• Do not require treatment.
• Follow-up.
• In rare cases, a nevus on the outer wall of the eye that is affecting the eye’s
appearance may be removed with surgery.

29. Others…
Retinitis Pigmentosa.
Pigmented paravenus
chorioretinal atrophy.
Leber congenital amaurosis.

30. THANK YOU