Disorders of the Retina

Macular Disease

  • Age-Related Macular Degeneration

  • Idiopathic Central Serous Chorioretinopathy

  • Cystoid Macular Edema

  • Macular Hole
    • Full-thickness defect in macula (ILM to PRC outer segs)
    • Thought to occur from tangential traction of the cortical vitreous overlying the fovea
    • Staging
      1. Foveal detachment -- decreased or absent foveal depression, presence of yellow spot or ring
      2. Early hole formation -- enlarged yellow ring with eccentric or central dehiscence
      3. Fully developed macular hole -- hole with surrounding cuff of subretinal fluid, operculum may be seen in hole
      4. Full-thickness macular hole with posterior vitreous separation
    • Special Tests
      • Watzke Slit-Beam Test -- patient notices a break in a thin beam centered over a macular hole
      • FA -- early hyperfluorescence without late leakage
    • Disease course
      • Stage 1 holes resolve spontaneously 50% of the time
      • Progress over weeks to years (ave 6 mos)
      • Risk of hole in fellow eye 10-15

  • Epiretinal Membranes
    • Fibrotic membranes which form by cellular proliferation on the inner surface of the retina
    • Classified according to severity
      • Cellophane maculopathy -- translucent membrane with minimal distortion
      • Macular pucker -- distinct tissue easily visible on retinal surface with distortion and wrinkling of macular surface
    • FA -- vascular tortuosity and straightening; may have leakage if contraction is significant

  • Pathologic Myopia
    • Posterior Staphyloma -- area of ectatic sclera with absent or severely atrophic overlying choroidal or retinal tissue

  • Angioid Streaks
    • Breaks in Bruch's membrane radiating from the optic disc (basophilia, calcification, elastic degeneration)
    • 50% associated with systemic disorders (PEPSI)
      • Pseudoxanthoma elasticum -- "plucked-chicken" skin, GI bleed, cardiac disease
      • Ehler-Danlos Syndrome -- hyperextensible skin
      • Paget's Disease -- increased bony mass, high alk phos
      • Sickle Cell and other Hemoglobinopathies (CNVM rare)
      • Idiopathic

  • Photic Retinopathy
    • Solar Retinopathy
    • Light toxicity from Ophthalmic Instruments
  • Drug Toxicity
    • Phenothiazines
      • Pigmentary retinopathy (phenothiazines are absorbed by melanin)
      • Thioridazine (Melaril) retinopathy can be seen after >800mg/day for 3-8 weeks
      • Chlorpromazine (Thorazine) retinopathy can be seen after >2400mg/day for 1 year
    • Tamoxifen
      • Crystalline retinopathy (may represent products of axonal degeneration)
      • Whorl-like corneal opacities
      • Rarely seen in men undergoing hormonal therapy
      • Other crystalline maculopathies:
        • Canthaxanthine Maculopathy -- skin-tanning agent
        • Oxalosis
        • Bietti's Crystalline Dystrophy -- 3rd decade, autosomal recessive
        • Autosomal Dominant Crystalline Dystrophy -- young female patients
        • Calcified Macular Drusen
        • Talc Retinopathy -- IVDA who inject crushed oral medications containing talc
    • Chloroquine and Hydroxychloroquine
      • Chronic use of >250mg/day chloroquine, >6.5mg/day hydroxychloroquine
      • Corneal deposits
      • Bull's-Eye Maculopathy
      • Differential Diagnosis
        • Cone Dystrophy
        • Stargardt's Disease
        • ARMD
        • Benign Concentric Annular Dystrophy
    • Methoxyflurane (secondary oxalosis)
    • Canthaxanthine


Vascular Disorders

  • Diabetic Retinopathy
    • Leading cause of blindness age 20-64
    • 25% of diabetics have diabetic retinopathy
    • Non-Proliferative Diabetic Retinopathy
      • Eye findings
        • Microaneurysms
        • Dilated capillaries
        • Dot-blot nerve-fiber layer hemorrhages
        • Hard exudates
        • Retinal edema
        • Cotton-wool spots
      • Special tests
        • IVFA - nonperfusion, edema, small NV, capillary dropout
        • US - tractional retinal detachment
        • Color fundus photos
      • Clinically-Significant Macular Edema
        • Defined as:
          1. Thickening within 500 microns of the foveal avascular zone (FAZ)
          2. Hard exudate within 500 microns of FAZ with associated thickening
          3. Zone of thickening one disc area or larger within one disc diameter of the center of the macula
        • ETDRS study showed benefit of focal or grid laser
      • Staging & Follow-Up
        • Mild -- occasional microaneurysms (q12 months)
        • Moderate -- more MAs and scattered hard exudates/cotton-wool spots (q6-12 months)
        • Severe -- 4 quadrants severe retinal hemorrhages OR 2 quadrants of venous beading OR 1 quadrant of severe IRMA (intraretinal microvascular abnormalities) (q3-4 months)
        • Very Severe -- 2 or more quadrants of IRMA (q3-4 months)
    • Proliferative Diabetic Retinopathy
      • Presence of newly formed blood vessels/fibrous tissue arising from the optic disc or retina
      • Closure of arterioles -- ischemia -- vasoproliferative factors -- NV of retina, iris, optic nerve
      • 26% of pts with a 25-50year history of diabetes have PDR
      • Type I DM higher risk than Type II
      • Eye findings
        • NVD, NVE
        • Vitreous or preretinal heme
        • Preretinal fibrosis
        • Tractional RD
      • IVFA -- leakage from NV vessels
      • Treatment
        • May observe closely pts with severe NPDR and low-risk PDR
        • Full scatter PRP, 1200-1600 500micron spots 0.1 sec argon in 2 sessions
      • "High-Risk" PDR
        1. NVD > 1/3 disc area
        2. NVD on or within 1 disc diameter with vitreious or preretinal hemorrhage
        3. NVE at least half of the disc area and vitreous or preretinal hemorrhage
  • Radiation Retinopathy
  • Sickle Cell Retinopathy
    • Types
      • HgAS most common
      • HgSS produces the most severe systemic effects
      • HgSC is least common form, but most common to have retinopathy
      • HgAC and HbS-Thal are other forms
    • Eye findings
      • Salmon patch hemorrhages
      • Refractile deposits (resorbed hemorrhages)
      • Black sunbursts (RPE hypertrophy)
      • Sea-fan neovascularization
    • Staging
      • Stage I: Peripheral arterial occlusion
      • Stage II: Peripheral AV anastomoses
      • Stage III: Sea-fan neovascularization
      • Stage IV: Vitreous hemorrhage
      • Stage V: Tractional retinal detachment
    • Avoid carbonic anhydrase inhibitors, which can worsen sickling.
  • Hypertensive Retinopathy
    • Grading
      • Grade 1 -- Arterial narrowing
      • Grade 2 -- Grade 1 plus focal irregularities
      • Grade 3 -- Grade 2 plus hemorrhage/exudates
      • Grade 4 -- Grade 3 plus disc swelling
  • Embolic Diseases
  • Material Appearance Source
    Calcium White Cardiac Valve
    Cholesterol Yellow Carotid Disease
    Lipid Yellow Chest trauma, bone fracture, pancreatitis
    Steroid White Steroid injection
    Fibrin Grey Carotid disease
    Talc Refractile IV injection (IVDA)

  • Hollenhorst Plaques
  • Central Retinal Vein Occlusion
    • Associated with COAG, HTN, DM, Oral contraceptives, and diuretics
    • Nonischemic
      • 48% resolve completely
      • 30% partially resolve
      • 22% progress to complete occlusion
    • Ischemic
      • May have RAPD
      • 90% never improve past 20/400
      • 30% develop NVI by 3 months
  • Branch Retinal Vein Occlusion
    • Occlusion of the vein at the site of the AV crossing due to atherosclerotic arteriole compressing adjacent vein confined by a common adventitial sheath.
    • Associated with HTN
    • 63% of cases involve the superotemporal quadrant
    • Most spontaneously resolve with vision 20/40 or better
    • More than 5 disc areas of nonperfusion = increased risk for NV
    • Focal grid recommended for persistent CME with intact capillary perfusion after 3 months
    • PRP recommended with retinal or iris neovascularization
  • Central and Branch Retinal Artery Occlusion
    • Profound, sudden, severe, painless loss of vision
    • Macular cherry-red spot
    • Associated with HTN (67%), Carotid atherosclerosis (50%), DM (25%), Valvular disease (25%)
    • Associated with GCA in 1-2% of cases
    • Treatment
      • Most effective in the first 90 minutes
      • Reduce IOP by ocular massage or AC tap
      • Inhalation therapy (95% O2, 5% CO2) for 10 minutes qhour (q4h at night)
      • Oral acetozolamide, ASA
      • IV steroids for patients with GCA
  • Ocular Ischemic Syndrome
    • Condition attributed to chronic severe carotid artery (90% stenosis) or ophthalmic artery obstruction and subsequent ocular hypoperfusion
    • Gradual vision loss (weeks to months) with dull, aching pain
    • Prolonged recovery to bright light
    • 20% bilateral
    • Follow every 1-2 months to watch for rubeosis & glaucoma
  • Macroaneurysms
    • Round or fusiform dilations of the retinal arterioles within the first three orders of bifurcation
    • Associated with systemic HTN
  • Perifoveal Telangiectasis
    • Developmental retinal vascular disorder characterized by ectasia of capillaries (irregular dilatation and incompetance)
    • Group Visual Acuity Demographics IVFA Treatment
      1A 20/25 to 20/40 Unilateral congenital PFT
      40yo men
      Temporal half of macula has telangiectatic vessels with macular exudate and edema Photocoagulation may be helpful; spontaneous resolution possible
      1B 20/25 range Acquired unilateral idiopathic PFT
      40yo men
      Telangiectatic vessels at one o'clock at the edge of the FAZ with or without exudate No treatment
      2 Better than 20/30 Acquired bilateral PFT
      50-60yo men & women
      Most common type
      Temporal foveal vessels, less than 1 DD, with mild dilation and graying of the retina
      Right angle venules present with some RPE hyperplasia
      No treatment
      3 Minimal loss to legal blindness Acquired bilateral PFT with capillary nonperfusion
      Telangiectatic vessels with enlarged FAZ with disc pallor; small gold flecks on retinal surface No treatment

  • Familial Exudative Vitreoretinopathy
    • Bilateral autosomal dominant disorder of peripheral vascular development often associated with retinal traction
    • Genetic defect which induces abnormal development of the hyaloid vascular system
  • Retinopathy of Prematurity
    • Risk Factors
      • Birthweight < 1500g
      • Gestational Age < 34 weeks
      • Oxygen therapy
      • Bronchopulmonary dysplasia
      • Sepsis
      • Exchange transfusions
      • Intraventricular hemorrhage
      • Necrotizing enterocolitis
    • Screening
      • All infants < 1500 g or < 34 weeks GA
      • Examine at 32 weeks and then every 2 weeks
      • Disease in Zone 1 or 2 -- examine weekly
    • Classification
      • Zone
        1. Centered on disc with diameter of 2x the macula-disk distance
        2. From zone 1 to nasal ora serrate and temporal equator
        3. All other retina outside zone 2
      • Extent -- number of clock-hours involved
      • Stage
        1. Demarcation line
        2. Ridge
        3. Ridge with extraretinal fibrovascular proliferation
        4. Subtotal retinal detachment
          • 4A -- Extrafoveal
          • 4B -- Includes fovea
        5. Total Retinal detachment
    • Treatment (Threshold)
      • Stage 3 in 5 contiguous OR 8 total clock hours
      • Stage 3 ROP in Zone 1 or 2
      • Plus disease
    • Follow-Up
      • Higher risk for strabismus, myopia, and astigmatism
      • Follow for first 5 years of life
  • Coat's Disease
    • Telangiectatic and aneurysmal retinal vessels with intraretinal and subretinal exudates
    • Primary vascular etiology with breakdown of the blood-retinal barrier vs. endocrinologic basis
    • Juvenile type presents with decreased vision, strabismus, leukocoria
    • Adult type can be asymptomatic or present with decreased vision
    • Eye Findings
      • Localized yellow/green subretinal exudates
      • Serous RD
      • Frequently involves macula
      • Telangiectatic vessels and microaneurysms
    • Poor prognosis
  • Von Hippel's Disease
    • An isolated retinal vascular abnormality characterized by a red/orange intraretinal mass with a dilated feeder artery and draining vein
    • Autosomal dominant with incomplete penetrance OR sporadic
    • 50% are bilateral
    • Angiomatous variant is peripheral and without feeder artery/draining vein
    • Enlarges over time leading to increased exudation, serous detachment, vitreous hemorrhage, or macular pucker
    • May involute spontaneously
    • Von Hippel-Lindau Disease
      • CNS hemangioblastomas
      • Cysts in kidneys, pancreas, liver, epididymis, ovaries
      • Renal Cell Carcinoma
      • Pheochromocytoma
  • Wyburn-Mason Disease
    • A rare ocular-cephalic syndrome in which abnormal retinal AV anastomoses are associated with AVMs in the ipsilateral midbrain region
    • Sporadic
    • Racemose angioma
    • Usually no treatment required
  • Retinal Cavernous Hemangioma
    • Rare vascular hamartoma composed of clumps of dark intraretinal aneurysms
    • Eye findings
      • "Cluster of grapes" -- clumps of intraretinal anuerysms
      • "Pseudohypopyon" -- layering of RBCs in aneurysms
      • Fibroglial membrane on surface
      • Vitreous hemorrhage
      • Subretinal, intraretinal, preretinal hemorrhages
  • Anemia
  • Hyperviscocity



  • Toxoplasmosis
    • Toxoplasma gondii
      • Obliqate intracellular parasite
      • Spread through cat feces
      • Tachyzoite invasion leads to retinitis until encysted by host immune response
    • Demographics
      • 20-70% of persons in US have postitive titers
      • Only 3% of acquired toxoplasmosis involves the eye
      • Most cases of retino-choroiditis are congenital
    • Eye Findings
      • Fine stellate KP
      • Mild AC inflammatory reaction
      • Moderate to severe vitritis -- "Headlight in Fog"
      • Patches of necrotizing retinitis, often adjacent to pre-existing scar
      • CME, Optic Neuritis, Vasculitis, scleritis, papillitis
      • BRVO in areas of retinitis
    • Special Tests
      • Toxoplasma IgG and IgM titers
      • Sabin-Feldman Dye Test -- tests the ability of serum antibody to fix complement and lyse cells
      • ELISA or indirect fluorescent antibody test
      • FTA-Abs, PPD, ELISA for Toxocara
    • Disease Course
      • Reactivated retinitis resolves over weeks to months
      • Average number of episodes: 2.7
      • 40% have single episode
      • Multidrug treatment limits spread
        • Sulfadiazine
        • Pyrimethamine
        • Folinic acid (Leucovorin)
        • Clindamycin, azithromycin, clarithromycin, spiramycin, minocycline, atovaquone, TMP/SMX are alternatives
        • Prednisone (20-80mg daily)
        • Infected Pregnant Women: Sulfadiazine, Clindamycin, or Spiramycin
      • Follow weekly and observe for medical complications of treatment
      • CBC bi-weekly while being treated
  • Sarcoidosis
    • Multisystem granulomatous disorder of unknown etiology
    • Increased incidence in African-Americans and Native Scandinavians
    • 90% have intrathoracic involvement
    • Special Tests
      • CXR
      • Biopsy of skin, lymph nodes, lung, liver, or conjunctiva
      • ACE detects total body granuloma content
      • Gallium scan of head, neck, mediastinum
      • Serum lysozyme, serum protein electrophoresis (SPEP), and serum calcium
      • Kveim Skin Test (No Longer Used) -- nodule induction using a suspension of human sarcoid tissue
  • Birdshot Retinochoroidopathy
    • Multiple, bilateral, often symmetric foci of hypopigmentation at the level of the choroid with visual loss secondary to CME and overlying nerve fiber layer atrophy
    • HLA-A29 association
    • Blurred vision, floaters, nyctalopia, altered color vision
    • Females > Males
    • Eye Findings
      • Discrete, creamy hypopigmented spots less than 0.5mm in diamter at the level of the choroid that are located predominantly posteriorly
      • Mild vitreous inflammation
      • May have attenuation of retinal vasculature with inflammatory sheathing, NFL hemorrhages, subretinal NV, CME, surface wrinkling
      • Disc edema/atrophy may be present
      • Minimal anterior segment involvement
    • May be associated with vitiligo or systemic vasculopathy
    • Foci of choroidal depigmentation may represent sites of previous accumulation of inflammatory cells below the RPE or focal depigmentation of choroidal melanocytes
    • Characterized by numerous exacerbations and remissions
    • 20% develop simultaneous, idiopathic glaucoma
    • Follow every 1-4 weeks during acute phase, 3-6 months during quieter phases
  • Punctate Inner Choroidopathy
    • Multifocal choroiditis with small yellow dots at the level of the RPE and inner choroid
    • Myopic women 16-40 years old
    • 2-6 slightly fuzzy yellow lesions 100-300 microns diameter located at the level of the RPE and inner choroid clustered in the macula and mid-periphery; may have overlying subretinal fluid collections
    • 25% develop choroidal neovascularization
    • Recurrent symptomatic episodes; no new lesions after 3 months
  • Acute Posterior Multifocal Placoid Pigment Epitheliopathy
    • Uncommon illness of young adults
    • Prodromal flu-like illness followed by the acute onset of multiple bilateral creamy plaques at the level of the RPE and inner choroid
    • New lesions develop up to 3 weeks after initial onset
    • Most patients recover good vision over 2-6 weeks
    • Poorer visual prognosis with foveal involvement (May use short-course steroids, but no proven benefit)
  • Serpiginous Choroidopathy (Geographic Helicoid Peripapillary Choroidopathy)
    • Rare, recurrent chorioretinitis affecting 30-60 yo's
    • Bilateral, fuzzy, pale, yellow subretinal patches extending from the optic nerve with adjacent atrophic scarring
    • Unknown etiology, possible association with von Willebrand factor
    • Careful follow-up with Amsler grids to rule out development of choroidal neovascular membranes
  • Multifocal Choroiditis and Panuveitis Multifocal Choroiditis, from RedAtlas.com
    • Unilateral disorder of young women
    • Multiple, recurrent, circular, pale, subretinal inflammatory lesions causing visual loss
    • Unknown etiology -- thought to be a direct infectious or secondary immune response
    • Chronic, recurrent disorder lasting months to yeasr with new lesions developing in either eye without regard to location of previous lesions
    • 50% of pts have vision worse than 20/200 in the affected eye
    • Steroids indicated for patients with CME, vitritis, extensive choroiditis, subretinal neovascularization
  • Multiple Evanescent White Dot Syndrome
    • Rare, self-limited disease of acute, unilateral visual loss following viral illness
    • Young adults, 75% women
    • Multiple creamy lesions at the level of the RPE
    • Etiology unknown, may be associated with viral illness precipitating a metobolic disturbance in the RPE-PRC complex
    • Acuity returns to normal within a few weeks
    • A chronic, bilateral form with multiple recurrences has been described
    • Choroidal neovascular membranes are rarely seen
  • Sympathetic Ophthalmia
  • Vogt-Koyanagi-Harada Syndrome
  • Whipple's Disease
  • Acquired Immunodeficiency Syndrome
  • Ocular Histoplasmosis
  • Diffuse Unilateral Subacute Neuroretinopathy
  • Pneumocystitis
  • Lyme Disease
  • Leber's Stellate Neuroretinitis/Cat-Scratch Disease
  • Cytomegalovirus
  • Acute Retinal Necrosis Syndrome
  • Progressive Outer Retinal Necrosis


Parasitic Disorders

  • Onchocerciasis ("River Blindness")
  • Toxocara Retinochoroiditis


Hereditary Disorders

  • Vitelliform Dystrophy (Best's Disease)
  • Stargardt's Disease/Fundus Flavimaculatus
  • Leber's Congenital Amaurosis
  • X-Linked Juvenile Retinoschisis
  • Retinitis Pigmentosa
  • Cone Dystrophy
  • Oguchi's Disease
  • Congenital Stationary Night Blindness
  • Fundus Albipunctatus
  • Choroideremia
  • Gyrate Atrophy
  • Central Areolar Choroidal Dystrophy
  • Pattern Dystrophies of the RPE
  • Metabolic Diseases
    • Cherry-Red Spot
    • Mucopolysaccharidosis
    • Sphingolipidoses
    • Cystinosis


Peripheral Retinal Abnormalities

  • Posterior Vitreous Detachment
  • Lattice Degeneration
  • Cobblestone Degeneration (Pavingstone Degeneration)
  • Retinal Tears and Holes
  • Senile Retinoschisis
  • Retinal Detachment
    • Rhegmatogenous
    • Tractional
    • Exudative
  • Proliferative Vitreoretinopathy



  • Developmental Abnormalities
  • Posterior Vitreous Detachment
  • Asteroid Hyalosis
  • Amyloidosis


Posterior Segment Trauma

  • Commotio Retinae
  • Traumatic Macular Hole
  • Retinal Tears and Dialyses
  • Giant Retinal Tears
  • Choroidal Effusion
  • Suprachoroidal Expulsive Hemorrhage
  • Choroidal Rupture
  • Choroiretinitis Sclopetaria
  • Purtscher's Retinopathy
  • Terson Syndrome
  • Intraocular Foreign Body
  • Endophthalmitis
  • Sympathetic Ophthalmia



  • Malignant Melanoma of the Choroid and Ciliary Body
  • Choroidal Osteoma
  • Choroidal Hemangioma
    • Retinal Capillary Hemangioma
    • Cavernous Hemangioma of the Choroid
  • Retinal Hamartoma
  • Intraocular Lymphoma


Major Studies

  • Diabetic Retinopathy Study
  • Diabetic Retinopathy Vitrectomy Study
  • Early Treatment Diabetic Retinopathy Study
  • Endophthalmitis Vitrectomy Study
  • Macular Photocoagulation Study
  • Central Vein Occlusion Study
  • Branch Vein Occlusion Study




All pages are Copyright ©2006 by Dennis H. Goldsberry, M.D., P.E.
Reproduction or archival of any protion of these pages is strictly prohibited except by express written permission