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Jeanne L. Rosenthal MD MPOD FACS
Surgeon Director in Ophthalmology
Assoc. Director, Retina Service
Attending Surgeon, Trauma Service
New York Eye and Ear Infirmary
Clinical Professor of Ophthalmology
New York Medical College
Based on AAO Basic and Clinical Science Course, Section 12 Retina and Vitreous, 2006-2007
Part II Chapter 10 Retinal Degenerations Associated with Systemic Disease: I. Disorders involving other organ systems:
A. Infantile-Onset to Early Childhood-Onset Syndromes
2. Differentiate from Leber congenital amaurosis
3. Neuronal ceroid lipofuscinoses (Batten disease)
5. Leber's does not have seizures or deterioration in mental status
Bardet-Biedl Complex of diseases
1. Group of diseases with similar findings:
Hearing Loss and Pigmentary Retinopathy
a. Association of retinitis pigmentosa and congenital sensorineural
c. 10% of RP patients are profoundly deaf
d. Differentiate from Alport syndrome, Alström and Cockayne
1. Spinocerebellar degenerations: Friedreich's ataxia
5. Neuronal ceroid lipofuscinosis (Batten disease)
6. Progressive external ophthalmoplegia syndromes
b. Characteristic ERG abnormality: normal A wave, reduced B wave
2. Bardet-Biedel: pyelonephritis and kidney damage
5. Type II membranoproliferative glomerulonephritis: drusenlike deposits
1. Familial adenomatous polyposis (Gardner syndrome)
similar to congenital hypertrophy of the RPE
b. smaller, more ovoid, more variegated in color, multiple and bilateral
a. Associated with Refsum disease and Sjögren-Larsson syndrome
c. One third have pigmentary abnormalities in the retina
2. Melanoma-associated retinopathy (MAR)
II. Metabolic Diseases:
a group of genetic disorders involving melanin pigmentation,
characterized by congenital hypopigmentation of the hair, skin, and eyes.
d. Strabismus: abnormal retinogeniculostriate projections (temporal
2. True Albinism:
congenitally subnormal vision and nystagmus. True
albinism has foveal hypoplasia.
: Eyes and skin effected.
melanosome. Autosomal recessive. Tyrosinase
positives have some pigment, whereas tyrosinase negatives have
b. Ocular findings include strabismus, subnormal acuity, abnormal VF
membranes, partial aniridia, capsular cataract.
c. Skin erythematous with increased frequency of cutaneous basal and
d. Tyrosinase negatives:
milk-white hair; skin pink to red; eyes light
grey to light blue, with no pigment flecks; prominent red reflex;
e. Tyrosinase positives:
hair becomes yellow to brown with age; eyes
blue to brown with flecks at iris border; fundi “blond”;
pigmented nevi common. Vision 20/60- to 20/100. Hair bulb test
f. Potentially Lethal Forms:
i. Chediak-Higashi Syndrome:
g. Other types include Platinum, Yellow Mutant, Brown, Rufous.
: effecting only eyes.
a. Decreased number of melanosomes, but each melanosome may be
b. Carrier females - spotty iris transillumination, peripheral RPE
mosaicism, macromelanosomes on skin biopsy.
normal or minimally reduced vision, no nystagmus. Autosomal
Normal Pigmentation: pigmented cells, either melanocytes (neural crest @
20 weeks gestation), or pigment epithelial cells (optic vesicle @ 5 weeks)
a. Melanosomes: organelles in which melanin is synthesized. Synthesis
l-tyrosine → l-DOPA → dopaquinone → pheomelanin (red/yellow)
b. Early completion of melanogenesis in RPE is probably essential for
a. Refraction to minimize useful vision.
c. Skin protection and regular examinations for malignant and
e. Strabismus: decrease in nystagmus may follow repair.
f. Prenatal diagnosis: normal fetus has pigmented scalp hair follicles @4
B. Central Nervous System Metabolic Abnormalities
Several metabolic diseases are known to affect the CNS and retina.
1. Neuronal ceroid lipofuscinosis (Batten disease)
b. accumulation of lipopigments within lysosomes of neurons,
c. progressive dementia, seizures, visual loss, pigmentary
d. Findings: optic atrophy, macular pigmentary changes with
mottling of the fundus periphery, low or absent ERG
e. Later onset cases may have bull's-eye-maculopathy
f. adult forms of NCL do not have ocular manifestations
2. Abetalipoproteinemia and vitamin A deficiency
b. apolipoprotein B is not synthesized. Leads to fat malabsorption
and deficiencies of fat-soluble vitamins
d. most common cause of vitamin A deficiency retinopathy is in
3. Peroxisomal disorders and Refsum disease
b. Dysfunction or absence of peroxisomes or peroxisomal enzymes
a. excessive quantities of incompletely metabolized acid
b. autosomal recessive except for type II (Hunter) – x-linked
c. Retinal dystrophy caused by storage of heparan sulfate only:
i. MPS IH (Hurler syndrome) and MPS IS (Scheie syndrome):
syndrome): pigmentary retinopathy, no corneal
a. Tay-Sachs disease (GM2 gangliosidosis type I)
i. deficiency of subunit A of hexosaminidase A
ii. glycolipid accumulation in brain and retina
b. cherry red spot can also be seen in Sandhoff disease (GM2
i. glucosylceramide in liver, spleen, lymph nodes, skin and bone
d. Niemann-Pick disease: absence of sphingomyelinase isoenzymes
i. Type B: mildest. Macular halo is diagnostic.
ii. cherry-red spot – myoclonus syndrome
iii. Goldberg-Cotlier syndrome (GM1 gangliosidosis type IV)
gastrointestinal tract, CNS, heart, and RE system
tortuous conjunctival and retinal vessels,
a. Cystinosis: defect in transport out of lysosomes
i. accumulation of intralysosomal cystine
depigmentation of RPE with pigment clumps; good vision
i. mitochondrial myopathy with "ragged-red" fibers on muscle
iii. With cardiomyopathy, known as Kearns-Sayre syndrome
b. NARP syndrome (neurogenic muscle weakness, ataxia, RP)
encephalomyopathy, lactic acidosis, stroke)
III. Systemic Drug Toxocity:
A. Chloroquine and hydroxychloroquine:
used in collagen-vascular diseases and
1. Toxicity related to duration of treatment, total dose, and patient age.
2. Blurred vision is major symptom. May have decreased color vision or
3. “Bull’s Eye maculopathy most common. Loss of foveal reflex with increased
: Used in malarial prophylaxis. Ocular toxicity rare if
total dose is less than 300 g. Daily dose of less than 250 mg.
more commonly used. Used to treat
rheumatoid arthritis. Lower incidence of toxicity. 5-7 mg/kg/day is safe.
No single test reliable in indicating early, reversible toxicity.
a. Damage may progress despite stopping drug.
photography, visual acuity, color vision, and Humphrey-type VF.
d. Ophthalmoscopic changes may precede symptoms.
e. FA shows typically more than seen ophthalmoscopically.
7. “Bull’s Eye” Maculopathy: Diff. Dx.:
1. Bind to melanin granules. Concentrated in uvea and RPE.
: abnormal pigmentation of lids, conjunctiva,
cornea, lens capsule and lens. Retinopathy rare, but reported.
: severe retinopathy.
d. Pigment stippling in post. pole early, becomes widespread but patchy.
e. Atrophy of RPE and choriocapillaris. Areas of hypo and
C. Other Agents:
treatment of breast cancer
b. Crystalline retinopathy in very high doses.
3. Methoxyfluorane (Penthrane):
a. Broken down to oxalic acid + calcium, then to calcium oxalate salt.
b. Oxalate crystals accumulate in inner retina and RPE.
c. Also can be seen with ingestion of ethylene glycol
a. used IV to treat transfusional hemosiderosis
d. ring scotoma and widespread mottled pigmentary changes
5. Isotretinoin (Accutane):
a. Assoc. with poor night vision and decreased dark-adaptation
a. used in HIV patients as prophylaxis against Mycobacterium avium
7. Cardiac glycosides (Digitalis):
c. May occur in 50% of patients taking more than 100 mg.
d. No permanent effects have been reported.
Chapter 11. Peripheral Retinal Abnormalities:
I. Retinal Breaks: full-thickness defect in neurosensory retina.
A. Horseshoe tear: flap of retina pulled anteriorly secondary to PVD.
B. Operculated hole: piece of retina pulled completely free.
E. Dialysis: break along the ora serrata.
F. Giant break: extending more than 90 degrees circumferentially
1. Associated with closed or penetrating injuries
a. Direct contusion (coup) causes contusion necrosis
b. Contrecoup injuries caused by compression of globe and
c. Stretch injuries to ocular coats: choroidal ruptures most
common, with tearing of RPE and choriocapillaris
d. Stretch injuries to vitreous cause tears along anterior or
circumferential linear break at the vitreous base
f. Avulsion of vitreous base is pathognomonic of trauma
Traumatic Retinal Detachment:
ii. If chronic, may show demarcation lines, thin retina, intraretinal
precipitates, normal or elevated intraocular
II. Posterior Vitreous Detachment:
B. Separation of posterior hyaloid from the retina
C. Normally, firm attachment of vitreous at vitreous base, optic disc, macula,
major vessels, lattice degeneration, scars.
E. Symptoms may include flashes and/or floaters. Commonly asymptomatic.
F. If symptomatic, 15% have retinal tear.
G. If associated with vitreous hemorrhage, 70% have retinal tear.
III. Lesions Predisposing to Retinal Detachment:
A. Lattice Degeneration
: full-thickness vitreoretinal abnormality
c. Histology: discontinuity of the internal limiting membrane of the
margin of lesion, atrophy of inner retina.
d. Underlying cause of 20-30% of all retinal detachments
e. Will cause detachment in only about 1% of patients with lattice
f. Typical ophthalmoscopic findings: white lines of Vogt, vascular
B. Vitreoretinal Tufts:
small retinal elevations caused by focal vitreoretinal
4. cystic and zonular-traction tufts may predispose to retinal tear/hole
C. Meridional Folds
: folds of redundant retina at ora. Occasional tear at
Enclosed ora bays:
islands of pars plana epithelium posterior to ora
E. Peripheral Retinal Excavations
: may be atypical lattice
IV. Lesions Not Predisposing to Retinal Detachment:
: discrete areas of ischemic atrophy of outer
retina, RPE, and choriocapillaris. Usually inferior.
: occurs after trauma, inflammation, tumor, or degeneration
: degenerative or congenital. RPE looks dark or black.
Congenital hypertrophy of RPE (“bear tracks”),
usually in groups
b. With aging as degenerative change in periphery.
c. Histology: larger than normal cells with large spherical melanin
V. Prophylactic Treatment of Retinal Breaks:
A. 6% of all eyes have breaks, but incidence of RD in population is only 0.07%
B. Symptomatic flap tears have high risk of RD, therefore usually treated.
C. Acute operculated holes less dangerous because vitreous traction relieved.
E. Asymptomatic flap tears: usually low risk.
May treat with lattice, myopia,
F. Lattice Degeneration: may treat in presence of high myopia, retinal
detachment in fellow eye, flap tears, aphakia.
H. Subclinical detachment: subretinal fluid extends more than 1 disc diameter
from the break but not more than 2 dd posterior to the equator.
VI. Retinal Detachment
A. Rhegmatogenous Retinal Detachment:
1. Liquid vitreous passes through break in the retina into subretinal space.
3. Shafer’s sign
: pigmented cells in anterior vitreous.
6. Anatomic reattachment rate overall is 90%.
7. Post-operative vision: dependent on whether macula detached and how long
a. Sparing macula: 87% have 20/50 or better
b. Macula detached: 37% have 20/50 or better
i. If less than 1 week, 75% have 20/70 or better
c. Complications effecting final vision:
B. Tractional Retinal Detachment:
1. Caused by vitreous membranes pulling on retina.
2. Usually membranes can be seen on examination.
3. Retina has smooth surface, concave, and is immobile.
6. If rhegmatogenous component present, may require vitrectomy + buckle.
C. Exudative Retinal Detachment:
1. Treatment is usually directed at underlying disorder.
2. Usual causes are inflammatory or neoplastic.
4. Smooth appearance of detached retina.
VII. Differential Diagnosis of Retinal Detachment:
B. Typical peripheral cystoid degeneration
2. Cystoid cavities in outer plexiform layer
3. Benign; may progress to typical degenerative retinoschisis
C. Reticular peripheral cystoid degeneration
1. Typical and reticular forms, difficult to differentiate clinically.
2. Reticular form: split occurs in nerve fiber layer
c. 23% have outer layer breaks (rolled edges)
3. Typical form: split occurs in outer plexiform layer.
a. White dots: remnants of Muller cells bridging cavity
E. Differentiation from Retinal Detachment:
2. Not associated with "tobacco dust" or vitreous hemorrhage
4. No demarcation lines, cysts, or RPE atrophy
1. Schisis associated with 3% of retinal detachments
a. associated with slowly developing detachments
a. Collapse of cavity may occur, causing RD
Chapter 12. Diseases of the Vitreous
I. Normal Anatomy
A. Vitreous consists of collagen and hyaluronic acid
B. Vitreous Base: from 1.5-2.0 anteriorly and 1.0-3.0 posteriorly to the ora serrata
1. Anterior Vitreous Cortex: anterior to vitreous base
2. Posterior Vitreous Cortex: posterior to vitreous base
a. Adherent to basal lamina of the internal limiting membrane of the
II. Posterior Vitreous Detachment
See Notes from Chapter 11
1. Persistent attachment of vitreous to macula causes distortion and elevation of
2. Attachment to fovea can cause macular hole
3. Remnants of vitreous on inner retina can cause ERM
III. Developmental Abnormalities
Tunica Vasculosa Lentis
B. Prepapillary Vascular Loops
2. Normal retinal vessels which grew into Bergmeister’s papilla
5. Associated with BRAO, amaurosis fugax, vitreous heme
C. Persistent Hyperplastic Primary Vitreous (PHPV)
1. Results from failure of primary vitreous to regress.
a. New terminology: Persistent fetal vasculature (PFV)
a. Occurs with anterior PHPV or isolated
d. Retinal (falciform fold) extending from the disc
e. Differentiate from ROP, toxocariasis, and FEVR
IV. Hereditary Hyaloideoretinopathies with Optically Empty Vitreous
1. Wagner’s (not associated with RD): autosomal dominant
2. Jansen’s (high incidence of RD): autosomal dominant
B. Associated with Systemic Abnormalities
1. Stickler’s (Marfanoid): most common
3. Spondyloepiphyseal dysplasia, variant and congenital forms
5. Vitreoretinal Degeneration in Facial Clefting Syndrome
C. Wagner’s and Jansen’s Syndromes
2. Myopia, strabismus, cataract, vitreous degeneration, chorioretinal
. Abnormal ERG late
D. Hereditary Progressive Arthroophthalmopathy of Stickler
2. Myopia, open angle glaucoma, cataract, perivascular pigmentation,
, posterior breaks, poor prognosis
5. Flattening of mid-face and nasal bridge, cleft palate, Pierre-Robin
anomaly (micrognathia, cleft palate, glossoptosis)
7. Marfanoid habitus with joint hyperextensibility (stiffness and soreness
a. Congenita form: manifest in 1st year of life; short limbs, cleft palate,
b. Childhood form: dwarfism with short trunk
c. Tarda form: dwarfism with short trunk, hip and back abnormalities
b. abnormalities of vertebral bodies, can cause paralysis
c. Facial abnormalities with cleft palate
3. Vitreoretinal Degeneration in Facial Clefting Syndrome
b. Hallmarks are family history of RD, facial clefting, vitreoretinal
e. Facial abnormalities and cleft palate
f. Optically empty vitreous with condensations and epiretinal membranes,
pigmentation, lattice, retinal breaks and RD
V. Familial Exudative Vitreoretinopathy (FEVR)
A. Autosomal dominant; full-term infants
C. Tractional RD’s with temporal dragging of disc vessels
D. Retinal exudation, especially in babies and adolescents
VI. Asteroid Hyalosis
A. Oval, white bodies adherent to framework of vitreous gel.
B. Most frequently seen in patients over 50; overall incidence 1 in 200.
D. Associated with diabetes in 30%; also associated with HTN.
E. Rarely causes significant visual symptoms.
A. Also known as Hemophthalmos, Synchysis Scintillans.
B. Highly refractile golden crystals freely floating in vitreous gel and anterior
A. Associated with dominantly inherited form
B. Bilateral vitreous opacities, “glass wool”
C. Amyloid also can be deposited in retinal vessels, choroid, TM
D. Associated with hemorrhages, exudates, CW spots, peripheral retinal neov.
E. Systemic findings: polyneuropathy, CNS abnormalities. Deposits in heart, skin, GI
IX. Spontaneous Vitreous Hemorrhage
A. About half caused by diabetic retinopathy
E. Neovascularization with BRVO or CRVO (3-10%)
G. Congenital retinoschisis and pars planitis
X. Pigment Granules
A. Shafer’s Sign:
pigmented cells in anterior vitreous in absence of trauma,
XI. Vitreous Abnormalities Secondary to Cataract Surgery
A. Vitreous loss associated with incarceration in wound.
7. Irvine-Gass Syndrome (with disc edema)
Chapter 13: Posterior Segment Trauma
I. Evaluation of the Patient Following Ocular Trauma
II. Blunt Trauma (no break in tissue)
A. Object causing injury does not penetrate eye
B. Blunt trauma may cause rupture of the eyewall
C. Anterior and posterior segment injuries may coexist
D. Look for angle recession, hyphema, vitreous hemorrhage, retinal tears, RD,
choroidal rupture, macular hole, avulsed
. Vitreous hemorrhage
1. From injury to ciliary body, retina, or choroid
3. Ultrasound if posterior segment cannot be visualized.
D. Commotio Retinae
1. Contrecoup injury to outer retinal layers by shock waves
3. In posterior pole, called Berlin’s edema
4. Cherry red spot in macula because of lack of photoreceptors in fovea
6. Visual recovery [variable; poor if increased foveal thickness found on OCT]
E. Choroidal Rupture
1. Stretch injury secondary to compression of globe in A-P axis
2. Tear in Bruch’s membrane, RPE, and choriocapillaris
3. Usually temporal to and concentric to disc
5. Associated with subretinal hemorrhage
6. Can develop subretinal neovascularization
F. Traumatic macular hole
1. Caused by contusion necrosis, vitreous traction, Berlin’s edema, or
2. May also occur after subretinal hemorrhage
3. Lightning and electrical injury can also cause macular hole
1. Produced by high-speed missile injuries to the orbit
3. Subretinal and retinal hemorrhages early
4. Extensive pigment alteration and scar formation late
5. If macula is involved, visual prognosis is poor
. Scleral Rupture
1. Most common locations are at limbus or under muscle insertions
2. Conjunctival chemosis and hemorrhage, decreased ductions, deep anterior
III. Lacerating and Penetrating Injuries (entrance wound only)
A. Cutting or tearing of eyewall by sharp object
B. Penetrating injury is caused by a laceration at a single site on the globe.
C. Careful wound closure of total extent of wound
D. Very posterior wounds may be left open
E. Complications include traction RD, cyclitic membrane, phthisis bulbi.
F. Optimal timing of vitrectomy is unknown.
1. Defer until 10-14 days to reduce risk of bleeding, allow cornea to clear, and
IV. Perforating Injuries (entrance and exit wounds)
A. Close anterior wounds. Small posterior wounds may be left unrepaired.
B. Vitrectomy useful in vitreous hemorrhage. Usually delay 7 days to allow
C. Remove posterior hyaloid during vitrectomy.
V. Intraocular Foreign Bodies
B. X-rays and CT scanning may be helpful.
2. Pars plana magnet extraction if small, non-encapsulated, easily
3. Intraocular magnet extraction following PPV
4. Intraocular forceps if non-magnetic or large
3. Pure copper highly toxic (chalcosis)
b. affinity for limiting membranes (Descemet’s).
iii. opacities in aqueous, vitreous, and in retina.
a. deposited in epithelial tissues of iris, ciliary body,
b. Ferric ions cause production of oxidants causing Haber-Weiss reaction
catalyze generation of hydroxyl radicals,
c. damage primarily to photoreceptors and RPE.
VI. Posttraumatic Endophthalmitis
A. Occurs in 2-7% of penetrating injuries
B. Higher incidence with FB’s and in rural settings (remember gardening!)
3. Inflammation with fibrin, hypopyon, vitreous inflammation
E. Culture and sensitivity of anterior chamber and vitreous
5. prophylactic use if no sign of endophthalmitis is controversial
VII. Sympathetic Ophthalmia
A. Avoid primary enucleation if globe can be repaired
B. Enucleation within 2 weeks if no potential for recovery
C. Incidence 1 in 500 cases of penetrating injury
D. Onset usually within 3 months to 1 year, but can be many years later
2. multifocal infiltrates in RPE (Dalen-Fuchs nodules) or choroid
VIII. Shaken Baby Syndrome/Child Abuse
5. resemble Terson syndrome, Purtscher’s, or CRVO
IX. Avulsion of the Optic Disc
A. Can occur from extreme dislocation of globe
Be sure to review Chapter 14 "Adverse Effects of Electomagnetic Energy on the Retina",
Chapter 15 "Laser Therapy for Posterior Segment Diseases", and Chapter 16
If you can get a copy of it, I also highly recommend the AAO Monography "Retinal
Detachment Principles and Practice" by Hilton, McLean, and Brinton, 1995 which is
unfortunately out of print.
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