1
RETINAL GRAND ROUNDS-PART II
COURSE # 1216
TESTS OF RETINAL STRUCTURE










Ophthalmoscopy
Fundus photography
Panoramic Imaging (Optos)
Fluorescein Angiography
B- scan ultrasound
OCT III
HRT II, HRT III
GDx VCC
RTA
CT & MRI
TESTS OF RETINAL FUNCTION










Visual Acuity
Pupils
Visual Fields
PHP
Color vision
Contrast Sensitivity
ERGs
E OG
Pattern and mf ERGs
VEPs (pattern & flash)
PANORAMIC OPHTHALMOSCOPY
Optomap Panoramic Ophthalmoscopy
 Images retina through an undilated pupil
 Goes out to 200 degrees
 Employs a red and green lasers-can separate retinal from choroidal lesions
New: OPTOMAP Fluorescein Angiography
 FA of the entire fundus at once may reveal abnormalities missed on standard
FA.
 Peripheral fundus is rarely imaged with standard FA.
 In addition to obvious applications (diabetes, vein occlusion) the new info may
yield insight into the pathophysiology of myriad retinal and choroidal disorders.
2
Clinical Electrodiagnosis
 Visual Evoked Potential (VEP)
 Electroretinography (ERG)
 Electrooculogram (EOG)
Electroretinography
 Rod function- dark adapted eye stimulus is very dim white or blue light
 Cone function- light adapted eye stimulus is bright white or red light
 Cone function- stimulus is 30Hz flicker
 Rod and Cone function- dark adapted eye with bright white stimulus
Tapetoretinal Degenerations
 Retinitis Pigmentosa
 RP related disorders
 Not all patients with night blindness have RP
 Congenital Stationary Night Blindness (CSNB)
CLINICAL PEARL: The appearance of the retina does not always
correlate with the function of the retina.
-Normal appearing retina-flat ERG
-RP appearing retina-normal ERG
Primary Retinitis Pigmentosa-Heritable
-AD RP, AR RP, X-Linked RP
-Diffuse RP
-Sector RP
-RP Sine Pigmento
-Retinitis Punctata Albescens
Variants of RP
Case
 58 y-o male presents for a routine exam
 Needs new reading glasses
 Gross confrontations
– Reports he sees examiner’s fingers but not the fingertips in the superior
field OD and OS
3
Patient 2: Patient’s Brother






62 y-o symptomatic white male
Long-standing history of visual field loss
Stable for years
Condition kept quiet from other family members
No night vision problems
BCVA OD 20/25; OS 20/25
Your Call
 Peripapillary pigmentary retinal degeneration
 Pigmentary paravenous retinal choroidopathy
 Autosomal Dominant Retinitis pigmentosa
 Autosomal dominant pericentral retinochoroidal atrophy
Autosomal Dominant Pericentral Retinochoroidal Atrophy
Something New??…….
 Reported in Retina, January, 2006
 Bass, SJ and Noble KG:
– Autosomal Dominant Pericentral Retinochoroidal Atrophy
 Proband tested for variants on the Rhodopsin (RHO) gene
– A Thr17Met variant was found
– High association with adRP
Pseudo-RP:Usually not heritable
 Inflammatory
 Toxic
 Other masqueraders
-Peripapillary Pigmentary Degeneration
-Paravenous Pigmentary retinopathy
Case
 A 42-year-old female
 Presents with complaints of decreased central vision for many years as a
teenager
 Rx: -7.00 D OD and OS
 BCVA OD=20/40; OS=20/40
4
Your Call
 Gyrate Atrophy
 Myopic Degeneration
 Mellaril Toxicity
 Retinitis Pigmentosa
Additional history…….
 Patients are not always forthcoming in original history
 Additional questioning-after seeing the fundus-reveals pt. was using Mellaril, a
psychotropic drug, for many years before stopping it 15 years ago.
Differential diagnosis of bilateral, uniform whitish spots in the fundus
 Dominant drusen
 Retinitis punctata albescens
 Fundus flavimaculatus (Stargardt Disease)
 Fundus albipunctatus (Congenital Stationary Night Blindness)
 MEWDS
 Talc retinopathy
Syndromic RP
 Although most cases of RP occur in patients without associated systemic
conditions, a minority present with a retinal degeneration as part of a
syndrome
 These include:
Lawrence-Moon Refsum
Bardet-Biedl
Kearns-Sayre
Usher
Alstrom
Alport
Zellweger
Inflammatory/Infectious Diseases of the Retina
Ocular Toxoplasmosis
-Caused by a protozoan
-Most common cause of posterior uveitis in the US
-Most forms are congenital (acquired in utero)
-Central and/or peripheral circular pigmented lesions, often with an
atrophic or fibrotic center
-Most cases re-activate during patients’ lifetime
-Vitritis-hazy fundus
-White, hazy lesion near old satellite lesion
5
-Treatment: Reactivations that threaten vision
-Bactrim
-Prednisone p.o
-Treat anterior uveitis topically if present
Presumed Ocular Histoplasmosis Syndrome (POHS)
-Caused by a fungus (Histoplasmosis capsulatum)
-Enters through the respiratory tract
-Seen in geographic areas near river valleys and/or in areas where there
is with contact with birds (e.g. chicken farms)
- Clinical triad
a. Peripappilary atrophy
b. Discrete small areas of chorioretinal atrophy (histo spots)
c. Choroidal neovascularization at the macula
Toxocara/Ocular Parasititis
- Caused by a nematode
- Ingested through contact with dog or cat feces
- Seen in individuals with a history of pica (eating sand or dirt)
- Nematode larva travels to one eye, causes granulomatous mass
-
usually near the optic nerve or in central retina in one eye only
Can cause diffuse retinal reaction in one eye only in some varaints in
other countries
Case
 18 y-o female from South America (Panama) presents with h/o decreased VA
OD for a long time
 Mother recalls that her daughter had a very “red eye” right eye as a young
child
Your Call
 A. Retinitis Pigmentosa
 B. Trauma with Old Retinal Detachment
 C. Ophthalmic artery occlusion
 D. Diffuse Unilateral Subacute Neuroretinitis (DUSN)
Diffuse Unilateral Subacute Neuroretinitis (DUSN)
 Term coined by Don Gass, MD
 Unilateral loss of vision in youngsters
 Fundus looks like unilateral retinitis pigmentosa, but etiology is inflammatory
not hereditary
6
 ERG is flat in affected eye
 Etiology believed to be similar to a nematode parasite related to but different
than toxocara
Ocular Manifestations of Sarcoidosis
-Localized inflammation/vasculitis along the vessels (“candle-wax”
drippings)
AIDS
UN AIDS/WHO AIDS Epidemic Update: December 2006
Number of people living with HIV in 2006:
 As of the end of 2006, 39.5 million people were estimated to be living with HIV/AIDS
worldwide.
 Adults - 37.2 million (32.1–44.5 million)
 W omen - 17.7 million (15.1–20.9 million)
 Children under 15 years - 2.3 million (1.7–3.5 million)
People newly infected with HIV in 2006:
 Total - 4.3 million (3.6–6.6 million)
 Adults - 3.8 million (3.2–5.7 million)
 Children under 15 years - 530 000 (410 000–660 000)
AIDS deaths in 2006 :



Total - 2.9 million (2.5–3.5 million)
Adults - 2.6 million (2.2–3.0 million)
Children under 15 years - 380 000 (290 000–500 000)
http://data.unaids.org/pub/EpiReport/2006/2006_EpiUpdate_en.pdf
Coat’s Disease
-Unilateral disease in 90% of cases
-Irregular dilatation of retinal vessels
-Progressive
-Development of intraretinal and subretinal exudation, CME, neovascularization, vitreal hemorrhage
-Most often in males under 20 years of age
-Not hereditary
-No other associated systemic disease
-Need fluorescein angiography
-Will demonstrate central or peripheral
-capillary aneurysms
-collaterals
-capillary drop-out
-Refer for treatment of leaking vessels
7
The Retina and Retinal Periphery -Identification and
Management







Most complete evaluation:
Both DFE with BIO, 90D , 3 mirror and OPTOS
OCT for cross- sectional analysis for additional info
New Topcon, Cannon good
New Zeiss Visucam NM-excellent resolution through small pupil
OPTOMAP best for mid and far periphery
ARIS (advanced retinal imaging system) best for stereo
VISUCAM PRO NM
CASES
Retinal Detachment
Retinal Tears
Lattice Degeneration
Retinal holes
Retinoschisis
Use of high tech imaging in detection of peripheral retinal anomalies
Optos
OCT
VISUCAM
B-scan ultrasound
Clinical Pearls For Peripheral Retinal Degenerative Lesions:
Retinal Consults for Lattice Degeneration:
a. Symptomatic lattice degeneration with atrophic holes and risk factors
(family h/o RD, high myopia, liquefied vitreous, h/o RD)
b. Symptomatic lattice degeneration with breaks at the border, and/or h/o
RD in fellow eye, strong traction
Retinal Consults for Retinal Holes:
a. Isolated, asymptomatic holes with greater than 1DD radius of cuff of
edema
b. Symptomatic and/or associated with a break or strong vitreous
adhesions
8
Retinal Consults for Operculated Holes:
a. Isolated, asymptomatic with cuff of edema greater than 1 DD radius or
2DD diameter
b. Symptomatic, and/or aphakia, and/or h/o RD in fellow eye, and/or
strong vitroretinal traction
Retinal Consults for Retinoschisis
a. Outer layer breaks are present
b. Progression towards posterior pole
c. Bullous and advanced