Outline
I. Treatable Macular Disorders
A. Age-Related Macular Degeneration
1. Most common cause of blindness in patients over 65 years of age
2. Two major classifications – Dry or atrophic and wet or exudative
a) The clinical presence of CNV determines the classification
3. Characterized by soft drusen 63 microns or larger, hyperpigmentation of
hypopigmentation of RPE, serous RPE or sensory retinal detachment,
hemorrhage (subretinal, retinal), geographic atrophy of RPE, or fibrous
scarring in a patient over 50 years of age.
4. Visual Loss due to degeneration of the photoreceptors secondary to
alterations in the RPE and Bruch’s membrane
B. Atrophic Age-related Macular Degeneration
1. Thickening of Bruch’s membrane, drusen, and mottling of RPE
2. Symptoms: Gradual decreased vision
3. Fundus Findings: Drusen (hard, soft, or calcified), RPE hyperpigmentation
and hypopigmentation, occasionally atrophy RPE and choriocapillaris.
4. FA: Drusen, light up early, may stain late. RPE mottling window defects.
5. Treatment: None proven.
6. Investigational Therapies: Nutritional supplements and laser to drusen.
a) Vitamin Supplements and Eye Disease
b) Age-Related Eye Disease Study
c) CNV PT and PTAMD Trials
C. Exudative Age-Related Macular Degeneration
1. Drusen with clinical findings of CNV
2. Symptoms: Decreased vision with/without metamorphopsia
3. Fundus findings: subretinal hemorrhage, serous RPE or sensory retinal
detachment, hard exudates, cystoid edema of retina
4. FA: Classic- Early discrete, lacy hyperfluorescence, late fuzzy border.
5. Occult CVN: gradual ooze of fluorescence through multiple areas
6. Treatment: Laser ablation (MPS) and photodynamic therapy (PDT)
a) Aragon Study: Effective for well-defined CNV outside FAZ
b) Krypton Study: Effective for Juxtafoveal CNV (non-HTN
c) Subfoveal Study: Effective, but immediate vision loss
d) PDT: Effective when lesion 50% or more “classic”; retreatments
7. Investigational Therapies:
a) Radiation Therapy: RAD study showed no efficacy
b) Transpupillary Thermotherapy: Pilot study-  exudation, stable VA?
c) Submacular Surgery: Pilot Study - removal better than observation?
d) Limited Macular Translocation: complex surgery, ? binocularity
e) Feeder Vessel Ablation: vessels number and size can limit success
f) Medical Therapies: Antiangiogenesis Drugs – VEGF Ab, PKC 412
D. Presumed Ocular Histoplasmosis Syndrome
1. Focal granulomatous choroiditis associated with H. capsulatum fungus
endemic to Ohio and Mississippi river valleys
2. Symptoms: Asymptomatic, decreased vision, or metamorphopsia
3. Fundus findings: peripheral atrophic punched-out choroidal lesions,
peripapillary choroidal scars, and macular lesions +/- CNV.
4. CNV may occur, usually associated with area of previous scarring.
5. Treatment: Laser photocoagulation proven effective CNV outside FAZ
6. Surgical removal of subfoveal membranes has had some success.
E. Myopic Maculopathy
1. Degenerative changes associated with high myopia
2. Symptoms: Decreased vision
3. Fundus findings: Crescents on optic nerve, posterior staphyloma, tessellation,
lacquer cracks, CNV, and choroidal atrophy.
4. Treatment: benefit of laser of other treatments has not been established.
F. Angioid Streaks
1. Dehiscence in Bruch’s membrane associated with degenerative mesodermal
and systemic vascular disorders (pseudoxanthoma elasticum, osteitis deformans,
fibrodysplasia, sickle cell anemia, senile elastosis.
2. Symptoms: Asymptomatic, unless macular CNV present.
3. Fundus Findings: Red-brown or red-gray linear, streaks radiating from
peripapillary area, with a tendency to involve the fovea.
4. Treatment: Laser treatment to CNV may be of benefit.
G. Epiretinal Membrane
1. Most associated with PVD idiopathic, some retinal surgery or other disorder
2. Fibrous metaplasia of astrocytes most common, fibroblasts, RPE cells,
hyalocytes, and vascular endothelium have been reported
3. Symptoms: Most asymptomatic, more severe membranes cause vision loss
and metamorphopsia. Majority are stable, 20% show progressive visual loss
4. Fundus Findings: glistening sheen in fovea or perifoveal area, retinal striae,
vessel traction, punctate hemorrhage, CWS, CME, or pseudohole.
5. FA: useful in evaluating for CME
6. Treatment: Epiretinal membrane peeling considered with significant vision
loss (>20/70 or significant distortion).
H. Macular Hole
1. Idiopathic most common, also with trauma, myopia, CME, and inflammation
2. Most common in 7th decade and in women more than men (2:1)
3. Tangential traction of prefoveal cortical vitreous elevates foveola (Stage 1);
prefoveal vitreous cortex separates with a small eccentric hole (Stage 2); hole
enlarges (>400 microns) (Stage 3); PVD present (Stage 4).
4. Fundus findings: Stage 1: yellow spot or ring, loss of foveal depression;
Stage 2: small eccentric hole, opercula may be present; loss of yellow ring Stage
3: hole enlarges, nodular yellow deposits on RPE (50%) and surrounding cuff of
sensory retinal detachment; Stage 4: Weiss ring present.
5. Treatment: Stage 1: observation. Stage 2-4: PPV with removal of posterior
hyaloid and fluid-gas exchange with postoperative facedown positioning.
I. Diabetic Maculopathy
1. Clinically significant macular edema (CSME), retinal thickening that
involves or threatens the fovea.
2. Symptoms: decreased VA or normal vision
3. Fundus findings: microaneurysms, hard exudates, retinal thickening
4. CSME: retinal thickening within 500 microns of fovea; hard exudates within
500 microns of fovea with adjacent retinal thickening; retinal thickening of 1DD
or larger within 1DD of the fovea.
5. FA: obtained to direct treatment, diagnosis is made clinically.
6. Treatment: Focal laser (60% reduction in moderate vision loss ETDRS)
J. Branch Vein Obstruction
1. Obstruction of retinal vein at A/V crossing, involving quadrant of retina or
small, localized area as in macular branch vein obstruction
2. Symptoms: Normal or decreased vision
3. Fundus findings: acute - wedge-shaped area of retinal hemorrhages, may
have CWS or retinal edema, with the tip at an A/V crossing.
4. Associated with hypertension, CV disease, and glaucoma.
5. FA: to assess decreased VA or extent of capillary non-perfusion
6. Decreased vision: Hemorrhage over fovea, macular edema, macular capillary
non-perfusion
7. Treatment: Underlying contributing systemic disorder, focal laser for
macular edema persisting for 3 months, scatter laser for neovascularization.
K. Central Serous Chorioretinopathy
1. A disorder of unknown etiology, associated with stress, in 20 to 50 yr old
2. Symptoms: blurred vision, micropsia, metamorphopsia, and positive scotoma
3. Fundus finding: well-defined, shallow, sensory retinal detachment often with
an underlying small RPE detachment, which is sometimes outside the SRD. RPE
detachment without SRD or multiple RPE’d are seen on occasion.
4. FA: Focal leak that spreads in diffuse manner
5. Examination of fellow eye often shows evidence of pervious (small area of
RPE disturbance) or current disease (RPD’d)
6. Treatment: observation v. focal laser (shorten SRD duration, no effect VA)
7. Severe, recurrent or chronic cases lead to RPE mottling and vision loss or
gutters of RPE depigmentation.
L. Cystoid Macular Edema
1. Intraretinal accumulation of extracellular fluid in outer plexiform layer
2. Causes: retinal inflammatory diseases, tumors, retinal vascular diseases
(macroaneurysm, telangiectasia, radiation), traction disorders, hereditary
disorders, toxic (latanoprost, nicotinic acid), and post-operative.
3. Irvine-Gass syndrome post cataract extraction
4. Symptoms: Decreased vision
5. Incidence decreased with intact posterior capsule.
6. Fundus findings: Cystic vacuoles seen with fundus biomicroscopy
7. FA: leakage from deep perifoveal capillaries in wreath like pattern.
a) Classic stellate figure late shots
8. Treatment:
a) Macular telangiectasia: laser photocoagulation
b) Retinal Macroaneurysm: laser photocoagulation
c) Toxic: removal of offending agent
d) Inflammation: Treat underlying disorder
e) Post-operative: topical NSAID or sub-Tenon’s steroid injection