Exam 2

Case 6 demographic

40 y/o caucasian male works as pilot; decreased vision OU

progressive for last 3m; constant and occasional itching

Hx: LEE 1 year ago, nasal allergies, uses lubricants and Patanol when needed, allergies to pollen

FHx: HTN with mother and father

BCVA: 20/70 OU with +0.25D

confrontation fields: FTFC; central scotoma on facial Amsler

CV: mild B/Y defect

SLE: trace hyperemia, papillae +1; every thing else normal

fundus: yellow/white foceal lesions with dark around it

DDx case 6

Bulls eye maculopathy; Chloroquine/Hydroxy/Plaqenile (not taking meds), Stargerdts (presents at younger age), Cone-rod (presents at younger age

Macular cysts: sacular yellow lesion; need OCT, FA, FAF

FA: inject Fl and see pathology of vessels (hyper → leakage and window defect)

FAF → autofluorescence of melanin and lipofuscin (hyper → alot of lipofusin and little melanin; hypo → little lipofuscin/alot of melanin)

Our pts OCT

outer retinal cystic formation → Macular cavitation; empty space/cavity with NO fluid

IZ gone; lost RPE and outer photor

EZ gone; loss of outer and inner photo r

damaged photor/RPE → decrease liposuscin

Our pt FAF

hypo macula ; loss of lipofuscin

damaged production of lipofuscin = damaged RPE

Out pts FA

early hyper

small window defect; loss of RPE/photor, can see through it

Disease causing macular cavitation/cysts in outer retina

cone-rod dystrophy; congenital

achromatopsia; congenital, complete color blind and damage retina

mac tel 2 → neurodegeneration, cavitation of inner/outer layers, fundus different

solar maculopathy; acute phototoxicity, hypo FAF, hyper FA (our dx; pilot)

Mac tel 2 findings (Slide 14-17)

parafoveal grey/green; retinal thinning and decrease melanin

telangiectasia in fundus; vessels go into macula and bend at 90 degrees

crystalline deposits (damaged Muller cells); effects outer and inner layers

hyperplasia of pigment

OCT: inner cavitation most common; ILM drap sign; can have outer cavitation

FAF: hyper parafovea, thin retina and decrease melanin

FA: telangiectasia leakage

Solar maculopathy (case 6 Dx)

acute: damage to IZ/EZ/RPE

chronic: months passes, RPE regenerates, EZ does not (damage to photor still)

VA 20/25-20/100; depends on damage to photor and which ones effected

increase damage of inner photor → worse VA (if most of the damage is in outer photor, the VA is better)

Prognosis: good/guarded; most recovers vision in 3-6m (depends on damage and which photor effected)

recovery also depends on initial VA loss; worse VA loss needs more time to recover

Tx: no specific Tx may give antioxidants to decrease oxidative stress

steroids could decrease inflammation of the macula; but could cause CSCR bc photor already damage (being studied but issue is CSCR)

Case 7 demo

47 y/o caucasian female; secretary; wrinkled distorted vision OU

OS>OD, mild to mod, progressive for one year, constant and worse with time

Hx: LEE 2 years ago, reading glasses, HTN, myotonic dystrophy type 1

previously told had “aging changes” of retina

meds: Lisinopril

ROS: fatigue, progressive lower extremity weakness

BCVA: OD 20/25-1, OS 20/40-2

controntation: FTFC, some distortion in facial amsler

SLE: mild hyperemia, trace papillae,

Case 7 posterior exam

macula: absence of foveal reflex with pigment clumping OU

strange changes in macula

was told pt had aging changes; but our pt is 47 (too young for AMD)

Case 7 auxillary tests

OCY

FAF
FA

Case 7 OCT

OD: ERM present, depositons on RPE, disruption of EZ/IZ/RPE

OS: macular pitt (missing layers)/partial lamellar hole (inferferes with inner layers only, outer layers normal), photor diruption

Case 7 FAF

hyper OU; increased lipofuscin

due to RPE issue; doesn’t get rid of waste and it accumulates

Pattern macular dystrophies

AD inheritance; kids may inherit a different form of pattern dystrophy

bilateral RPE proliferation with increase lipofuscin, okay VA

may have a typical presentation to differing types in each eye

4 types

Looks like AMD at a younger age; mimics other hereditary conditions

Diagnosis

OU Adult vitelliform pattern macular dystrophy; butterfly type

OD: ERM
OS: Lamellar hole

Adult vitelliform pattern macular dystrophy; butterfly type etiology

Retina degeneration slow (RDS) gene

produces peripherin 2 protein; responsible for maintaining photor disc

with pattern dystrophy: gene mutated → less protein → less photor disc maintenance → RPE gets sick → accum lipofuscin

system disease associated to Adult vitelliform pattern macular dystrophy

butterfly pattern type → myotonic dystrophy

all types (mostly fundus pulverulentus/FP) → pseudoxanthoma elasticum

Reticular pattern dystrophy (RPD)

net like pigmentation

most symmetric of all types

ERG: normal/mild decrease

EOG: normal/mild decrease

Macular pattern dystrophy simulating fundus flavimaculatous (MPDSFF)

actual fundus flavimactous → dark choroid, poor VA

pattern dystropht → bright white areas of choroid, VA still good, brighter surrounding area with no dark choroid

ERG: normal/slightly decreased (true fundus flavimaticus/stargardts decrease ERG)

Adult onset foveal vitelliform pattern dystrophy (AOFVPD)

mimics best in adults

yellow egg yolk of macula

older patients, better VA

ERG: normal or slightly decreased

EOG: barely effected; normal/slight decrease (best has decreased EOG)

Fundus pulverulentus (butterfly)

looks like AMD in younger pts; symmetric btwn eyes

no drusens present

diffuse RPE clumping

ERG: normal or mild decrease

EOG: normal or mild decrease

Pattern dystrophy management

some may progress; butterfly type worsens with age (decrease vision and CNVM)

if 70 y/o with pattern dystrophy; must also check for AMD (hard to decipher), monitory for atrophy of AMD (OCT, FAF)

monitor and f/u with all types (butterfly type; monitor for CNVM)

can give antioxidants bt cannot prevent progression

Case 8 demo

76 y/o caucasian male, retired, bothersome pain in OS started last night and gotten worse (unbearable); very severe and sudden (14 hours ago)

vision very blurry; HA, nausea, vomiting

ocular Hx: phaco Sx with IOL OU 5 years ago, YAG capsulotomy OS 8 months ago, another laser in OS 10 years ago doesnt remember; LEE 8m ago

medical Hx: hypothyroid, depression, chronic pain; Synthroid, Trazadone, Tramadol

ROS: chronic pain and uses narcotics (Tramadol)

BCVA: OD 20/25-1, OS 20/70-2

pupils: OD 3.5mm in dark 2mm in light; OS 5mm in light and dark; +2 APD OS

Confrontation: FTFC OS, generalized constriction OS

SLE exam case 8 (slide 7 and 8)

lids/lashes/adnexa → MGC 2+

conjunctiva: hyper +1 OD, hyper 2+ OS

A/C: deep/quiet OD, OS look at image

patent LPI in OS (laser Sx pt couldn’t remember)

IOPs: OD 11, OS 52

gonio: OD SS seen in all quadrants, OS ant TM and SL seen 360 (closed)

case 8 fundus

OD: 0.55/0.55, negative FR, RPE changes, post pole healthy, no breaks/detachments

OS: 0.85/0.85, notching, negative FR, RPE changes, post pole healthy, no breaks/detachments

Additinal tests for case 8

anterior chamber OCT

anterior chamber B scan

Case 8 anterior chamber OCT

normal curvature of the iris

angle closed the whole way (periphery and central)

no iris bombe

Case 8 anterior B-scan

normal curvature of the iris

angle closed the whole way (periphery and central)

no iris bombe

DDx for case 8

primary/pupillary block angle closure glaucoma : LPI will always work; pupillary block with iris bombe (central A/C is open and periphery is closed)

secondary: LPI will NOT work, central and peripheral A/C is closed

Aqueous misdirection syndrome (our Dx)

must r/o tumors (push everything forward), choroidal detachment

testing for primary/pupillary block angle closure

iris bombe (convex iris)

open centrally and closed peripherally

NOT OUR DX

Aqueous misdirection syndrome (malignant glaucoma); secondary closure

closed central and peripheral; everything pushed forward

after Sx, AH escapes anterior chamber and goes back behind vitreous and pushes vitreous forward

too much volume overload in posterior chamber; creates pressure in front of eye (complete closure of center and periphery)

Treatment for aqueous misdirection syndrome

decrease pressure by medications that decrease AH production; B blockers, CAIs, Adrenergic agonist

decrease vitreous volume; dehydrate the vitreous with hypertonic solutions (Mannitol and Glycerol)

Push iris back with cycloplegics (cyclo and atropine); dilating pupil releases the iris and allows relaxing/moving back (stops accommodation)

CANNOT USE PILOCARPINE (will worsen)

if medications dont work (50% pts) → laser with Sx

Surgeries/laser for misdirection

try meds first: cycloplegia, AH suppressants, osmotic agents, steroids

phakic pt: cataract extraction with IOL impantation → posterior capsulotomy and disruption of anterior hyaloid → vitrectomy

pseudo/phakic: ND: YAG capsulotomy and disruption of anterior hyaloid → trans-scleral cyclodiode laser → vitrectomy

Complications of AH misdirection

peripheral anterior synechiae

corneal edema and decompensation; due to damage

accelerate cataract formation/progression in phakic pts

myopic shift; everything shifted forward but NOT the retina

retinal or choroidal detachments

macula detachment

Prognosis of AH misdirection

good-guarded; many treatment options

recurrent; second resentation is 2x harder to treat

other eye has 75-80% likely to get malignant glaucoma as well

prophylaxis for other eye is cycloplegics, cataract Sx, wait to see if anything happens (laser if anything happens)

three steps → Tx one eye, follow one eye, follow fellow eye

Case 9