Common Posterior Segment Conditions and Anomalies
Joseph Sowka, OD, FAAO, Diplomate
White Without Pressure (W w/o P)
 30% incidence in general population
 10 times more common in dark skinned individuals
 Usually bilateral
 Questionable change over time
 Gray-white translucent area with scalloped borders
 Often mistaken for retinal detachment by beginning optometry students
 Flat
 Represents an abnormal vitreoretinal interface
 Disorganized retina
 Possibly some vitreoretinal traction
 Abnormal vitreoretinal interface
 Pseudoholes due to normal retinal coloration in the whitened areas
 Closer to ora
 Named for the phenomenon of white with pressure on scleral indentation
 Totally benign
Clinical Pearl: White-without-pressure is most common in Black patients and next most
common in Asian patients. It is uncommon in Caucasian patients. If you think that you
see white-without-pressure in a Caucasian patient, you are likely seeing a prominent
vitreous base
Prominent Vitreous Base:
 Runs circumferentially around the eye within 1-2DD of the Ora
 Runs parallel to ora
 2-4 mm posterior to ora on retina side and 2 mm anterior to ora on pars plana side
 Looks like normal retina, followed by a ‘dark’ line, then an area of ‘brighter’ retina
 Common in light colored fundi, rare in darker fundi
 A strong point of attachment for the retina and the last aspect to pull away in vitreous
detachment
RPE Hypertrophy
 Also referred to as congenital hypertrophy of the retinal pigment epithelium (CHRPE)
 Represents enlargement of RPE cells
 Usually very dark
 May be lightly colored
 Bear tracks
 Large, scalloped borders
 Lacunae (extremely diagnostic), which will enlarge and multiply over time
 Relationship to colorectal CA (multiple areas) – Gardner’s syndrome
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Familial adenomatous polyposis (FAP) - a hereditary bowel disorder with propensity to
malignancy
 Patients with 4 or more RPE hyperplasia-like lesions and a family history of FAP (or
colorectal cancer) should undergo routine colonoscopy
 Patients with Gardner’s syndrome have lesions that are slightly different from typical
RPE lesions in that they are multiple and scattered and pisciform
Well demarcated and clear due to superficial level
Very small (finding a snowball in a snowstorm) to several disc diameters (very scary, but just
as benign)
Some flat enlargement occurs in majority over time, but this is extremely difficult to
document without photographs
 Likely due to flattening and shifting of cells to account for RPE normal aging attrition
RPE hypertrophic lesions themselves are totally benign
 No malignant potential
RPE Hyperplasia
 Represents RPE cells invading sensory retina
 Response to injury, retinal tear, retinal detachment (demarcation line), lattice degeneration,
etc.
 Demarcation line - 90 days post retinal detachment
 Black
 Bone spicules - pseudo retinitis pigmentosa
 Chorioretinal scars
 If antecedent condition inactive - RPE hyperplasia is benign in that it does not progress
without reason, but will still cause vision/field loss.
If, after this lecture, you can not tell the difference between RPE hypertrophy and RPE
hyperplasia and confuse the two conditions in clinic, my head will explode!
Chorioretinal Scar
 Post- inflammatory or traumatic
 Retinal atrophy or fibrosis
 RPE hyperplasia
 Scar itself is benign
 Vitreoretinal traction
 May unfortunately involve the macula
 Toxoplasmosis a very common cause
 RPE hyperplasia is a component of chorioretinal scarring- a differential diagnosis shouldn't
be: chorioretinal scar vs. RPE hyperplasia - they are essentially parts of the same entity.
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Clinical Pearls: Lesions in the choroid tend to have their outlines and true coloration
altered due to the fact that they are beneath the RPE, which is a pigmented structure.
Changes in the RPE such as hypertrophy and hyperplasia typically are dark and well
circumscribed because the RPE is under the retina, which is a clear structure.
Clinical Pearl: Any unusual coloration or anatomic curiosity around the area of a vortex
vein complex IS a vortex vein complex until proven otherwise.
Cystoid Degeneration:
 Present in 100% of eyes over 8 years old
 May resemble lattice, but cystoid is elevated whereas lattice degeneration is depressed
 More common temporally
 Stops at the posterior border of vitreous base
 Two types
1. Typical: cysts in the outer plexiform layer. Hazy, gray, stippled appearance of the
retina. Precursor to retinoschisis development. May have lamellar hole formation.
2. Reticular: cysts in the nerve fiber layer. Well demarcated and typically occurs
posterior to typical cystoid. More likely to develop full thickness breaks.
 Cystoid degeneration itself is generally benign. Monitor with annual examinations.
Primary Chorioretinal Atrophy
 Also known as cobblestone degeneration or pavingstone degeneration
 30% of patients
 Rare in African Americans and other dark skinned individuals
 Almost always seen in lighter than normal colored (blond) fundi
 Pale with RPE hyperplasia surrounding
 Choroidal vessels seen beneath
 80% are located in inferiotemporal quadrant
 Congenital
 Loss of choriocapillaris, particularly the precapillary arterioles
 Atrophy of RPE and outer retinal layers
 Inner retinal layers intact
 Benign
Clinical Pearl: In patients with blond fundi, you should expect to see cobblestone lesions.
In fact, you will likely see cobblestones, if you look hard enough.
Peripheral Tapetochoroidal Degeneration:
 Synonymously known as peripheral senile pigmentary degeneration
 Granular pigmentation between ora and equator in approx. 20% over age 40 and increases
with age
 Degeneration and loss of pigment granules in the RPE
 Loss of photoreceptors
 Thickening of Bruch’s membrane
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Loss of capillaries in choriocapillaris
Develops a granularity and irregularity of the RPE
Somewhat difficult to distinguish from retinitis pigmentosa
Benign
Myelinated Nerve Fibers:
 Often emanate from the disc
 May be isolated within the posterior pole
 Generally does not extend beyond posterior pole
 Mostly congenital, but can be acquired later in life and both progress and regress
 Has very little visual or clinical significance
 Some dense lesions have been known to produce visual field defects, but this is not
common
 Also called medullated nerve fibers
 There appears to be a well-documented but as-of-yet unnamed syndrome consisting of
(aniso)myopia and anisometropic amblyopia in association with myelinated nerve fibers. This
syndrome appears distinct from simple unilateral myopia with amblyopia and from
myelinated nerve fibers without myopia or amblyopia. Macular changes including irregular
pigmentation and loss of foveal reflex are often present in association in these cases and may
contribute significantly to vision loss beyond amblyopia.
Asteroid Hyalosis
 "All that glitters is not gold"
 Occurs in vitreous, not your condensing lens
 Multiple, quadrantic, or total
 Calcium salts
 Cholesterol crystals
 Asymptomatic
 Totally benign
 Can be so dense that observation of the fundus is impaired, but is still minimally
symptomatic or disturbing to the patient.
Clinical Pearl: Whenever you are asked in clinic at what age does a vascular disease
become most prevalent and you have absolutely no idea, say, “65 years old” and, with the
exception of diabetes, you will be right most of the time.
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