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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

EOG

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.

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 AlbescensVariants 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

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 AtrophySomething 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

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-MoonRefsum

Bardet-BiedlKearns-Sayre

UsherAlstrom

AlportZellweger

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

-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

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)

Women - 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)

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

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:

  1. Symptomatic lattice degeneration with atrophic holes and risk factors (family h/o RD, high myopia, liquefied vitreous, h/o RD)
  2. Symptomatic lattice degeneration with breaks at the border, and/or h/o RD in fellow eye, strong traction

Retinal Consults for Retinal Holes:

  1. Isolated, asymptomatic holes with greater than 1DD radius of cuff of edema
  2. Symptomatic and/or associated with a break or strong vitreous adhesions

Retinal Consults for Operculated Holes:

  1. Isolated, asymptomatic with cuff of edema greater than 1 DD radius or 2DD diameter
  2. Symptomatic, and/or aphakia, and/or h/o RD in fellow eye, and/or strong vitroretinal traction

Retinal Consults for Retinoschisis

  1. Outer layer breaks are present
  2. Progression towards posterior pole
  3. Bullous and advanced