Retina cases (oral boards) Flashcards
Macular hole DDx
macular pucker, CME intraretinal cyst
> Pseudohole
- Appearance of hole w/ steep walls
- Caused by vascular tortuosity around macula
> Lamellar Hole
- Thin layer of retina lifted w/ walls “bulging out”
- Maybe precursor or resolved macular hole
Macular hole history questions
previous trauma?
previous eye surgery?
Macular hole exam
complete exam, especially of macula
Perform Watzke-Allen test (slit beam and see if line broken/distorted):
>distortion = pucker
>broken = hole
Macular hole evaluation
OCT = preferred modality
IVFA with early hyperfluorescence w/o leakage for hole
Macular hole stage 1 definition and Rx
Stage 1 = impending hole
Rx: Observe, as 50% will resolve spontaneously
Macular hole stage 2 vs 3 definition and Rx
Stage 2: full thickness hole 400 um, + elevated rim
Rx: vitrectomy +/- ILM peel
if operated w/in 1st 6 mo can regain 1/2 visual loss. others operate up to a year. small portion of patients may benefit even if long-standing history of hole
Education: low risk for RD, but must explain Si/Sx to patient
Macular hole stage 4 definition and Rx
Stage 4: hole with cuff and complete PVD
Rx: vitrectomy +/- ILM peel
if operated w/in 1st 6 mo can regain 1/2 visual loss. others operate up to a year. small portion of patients may benefit even if long-standing history of hole
Education: low risk for RD, but must explain Si/Sx to patient
Bulls eye maculopathy DDx
chloroquine/hydroxychlorquine toxicity cone dystrophy macular degeneration (AMD) Stargardt or fundus flavimaculatus battens = (AR) lysozyme storage dz. H/o seizures and progressive dementia with retinal findings of pigmentary retinopathy
Bulls eye maculopathy history questions
Medications: chloroquine/hydroxychlorquine (dosages)?
History: FH of retinal dz, Sx of photophobia, seizures, ataxia, dementia
New macular hole grading system?
Small 400um
Bulls eye maculopathy exam
BCVA, IOP, DFE with special attention to macular pigment changes, drusen, macular, or peripheral pisciform (fish-like) flecks, CNV
Perform a neurologic exam
Bulls eye maculopathy evaluation
If: patient younger, no RA, not taking chloroquine/hydroxychlorquine then order ERG and IVFA
If ERG shows a non-recordable photopic response I would suspect a Dx of cone dystrophy
If IVFA shows silent choroid I would suspect stargardt
Bulls eye maculopathy A/P
need to evaluation medication dosages.
Maximum daily dose of chloroquine
chloroquine/hydroxychlorquine toxicity education
once toxicity starts, it may continue despite stopping the medication:
Baseline exam
vision, fundus photos, HVF (red test)
OCT MAC
NV fronds with exudates and hemorrhage in child ddx
sickle cell retinopathy (sea-fan shaped)
sarcoid sea fan neovascularization
diabetic retinopathy
eales peripheral retinopathy of unknown etiology
NV fronds with exudates and hemorrhage in child: history and eye exam
medical history of: sickle cell, DM, sarcoid, IVDA
look for in eye exam: uveitis, iris nodules, NVI, DFE
NV fronds with exudates and hemorrhage in child:
tests: sickledex, ACE, lysozyme, fasting blood glucose, CT of chest if sarcoid suspected.
IV FA to assist evaluation and development of Rx plan
Sickle cell A/P:
NV –> needs PRP to ischemic area located anterior to sea fan
RD: PPV precautions (avoid encircling SB or taking down EOMs, avoid epi in local anesthetic, ensure O2 & hydration)
For pts with sickle cell history and no evidence of retinopathy - plan f/u exams q6-12 mo
for patients who develop retinopathy, plan closer f/u q3-4 mo, performing PRP as needed
sickle cell education
SC worst ocular Sx (SC>Sthal>SS>SA)
SS worst systemic Sx
Avoid CAI
variable Px. with good patient education and comprehensive interval f/u, patients can expect to maintain good visual potential. Also need to have PCP involved to discuss si/sx of systemic sickle crisis
Sickle cell signs and stages
–Stage 1: peripheral arteriolar occlusion
–Stage 2: peripheral AV anastomoses
–Stage 3: preretinal sea fan NV (posterior border of nonperfusion; may autoinfarct and turn white)
–Stage 4: Vitreous hemorrhage
–Stage 5: Tractional RD
Black sunburst (mid-peripheral pigmented lesion with spiculated borders) Salmon patch (IRH) Iridescent spots (intraretinal spots s/p resorption of salmon patch) angoid streaks (conjunctival comma-shaped capillaries in inferior fornix)
4 quadrants flame shaped hemorrhages following NFL, swollen ON DDx
CRVO DM Radiation retinopathy hypertensive retinopathy papilledema
4 quadrants flame shaped hemorrhages following NFL, swollen ON History
History Questions: HAs, transient visual obscurations glaucoma, thyroid eye disease, DM, HTN, radiation, hypercoagulable dz, vasculitis (lupus, syphilis, sarcoid) medications (OCP, diuretics, tetracycline, trenitoin), orbital tumors
4 quadrants flame shaped hemorrhages following NFL, swollen ON eye exam
VA pupils Hertel ocular motility gonioscopy (looking for NVI/NVA) IOP DFE looking for NVD and macular edema
4 quadrants flame shaped hemorrhages following NFL, swollen ON tests
IVFA to determine and define macular edema - whether condition is ischemic or non-ischemic
IVFA will show non-perfused areas and the OCT macular swelling
Check: BP, evaluate for DM (FBS, HgA1c)
order screening labs (CBC, PT/PTT, ESR, lipid profile, homcysteine), sarcoid suspects (ACE, lysozyme, chest CT), atypical cases (FT-ABS, Lyme titer, ANA)
Refer for cardiovascular and hypercoagulable work-up as needed
CRVO A/P
suspect ischemic CRVO if: 20/400 VA, APD, no pain
Non-ischemic CRVO: usually has better VA and no APD
Ischemic cases can develop NVI/NVA. Therefore I would follow monthly for 6 months performing eye exams and gonioscopy looking for NV. Should this develop I would initiate PRP to regress NVA/NVI. FOr macular edema I would discuss using IV triamcinolone or anti-VEGF
CRVO education
would discuss with patient that there is a 10% chance of contralateral eye developing a CRVO or branched retinal vein occlusion (BRVO)
retinal whitening with cherry red spot DDx
CRAO, Tay-Sachs, Niemann-Pick (presents in younger children)
retinal whitening with cherry red spot history questions
History of HTN, carotid dz, CV dz, sickle cell, hypercoagulable states
syphilis, migraines, collagen vascular dz
retinal whitening with cherry red spot exam and evaluation
full eye exam: VA, pupils for APD, DFE to search for emboli
Seek etiology of emboli as this can be helpful:
(1) cholesterol emboli (hollenhorst plaque) = refractile bodies seen @ bifurcations arising from carotid artery atheromatous plaques
(2) calcium emboli = white seen distally in retina and arise from cardiac valves
(3) platelet -fibrin emboli = dull, white long arising from carotid atheroma
CRAO can occur with GCA, evaluate for HA, scalp tenderness, jaw claudication, obtain immediate ESR/CRP.
Retinal vasculitis (CG pt as example)
Ddx for etiologies include embolus, in situ thrombosis, GCA, connective tissue disease, hypercoagulable state and less likely syphilis, Behcet’s disease, Susac’s, migraine, trauma.
Plan:
Control comorbidities, including BP
Secondary risk stratification by PCP to include fasting BS, lipid profile, hypercoagulability workup
Will obtain ESR/CRP, CBC with diff, Coag panel, FTA-ABS, ANCA, C3/C4, CXR, UA
Provided h/o stroke and diffuse vascular disease, will obtain Carotid Doppler, cardiac echo
RTC 5-6 weeks to review results, DFE and OCT Mac
CRAO plan
If
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance DDx
Stargardt’s dz
Cone’s dystrophy
hydroxychloroquine or chloroquine toxicity
Batten’s dz
Fundus albipunctatus = white dot pattern in mid-periphery
Si/Sx: night blindness, prolonged dark adaptation, normal fields, vision.
Retinitis punctate albescens: declining vision, fields, night blindness, abnormal ERG
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance history
Timing of vision loss, medicine, medical and family history
For Batten’s: ask about h/o dementia, seizures
aka:
Spielmeyer-Vogt-Batten-Mayou
RP + neuronal ceroid lipofuscinosis
Lipofuscin accumulates in lysosomes
Also: seizure, dementia, ataxia, vacuolization of peripheral lymphocytes
Inheritance: AR, usu. Jewish girls
?fingerprint inclusions on EM of peripheral blood smears?
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance exam/evaluation
Complete eye exam
Additional testing: IVFA, ERG, EOG
On IVFA = “silent choroid” pattern with abnormal ERG and EOG based on fundus appearance if Stargardt’s
Cone dystrophy: if decreased color vision, attenuated ERG photopic responses, bull’s eye on fundus photo
Retinal hemorrhages and CWS at arcade distal to an AV crossing history A/P
A: evaluating for cause (HTN, atherosclerosis, DM) and managing sequelae is key to this condition
P: Check BP and consider bloodwork: fasting blood glucose, HGA1c, ANA, RPR, PT/PTT, ESR, homocysteine
Refer patient to internist for CV dz, HTN, CVD, hypercoagulable work-up as dictaed by my exam and history
Rx: chronic macular edema that persisted for 3-6 mo with vision
Retinal hemorrhages and CWS at arcade distal to an AV crossing education
Follow qmonthly for 6 months. Council that this condition can progress and can develop NVG. Ensure they are following with PCP for HTN and DM.
10% risk for contra/L involvement.
Macular pigmented scar, retinitis and overlying vitritis DDx
Toxoplasmosis, ARN, Toxocariasis, TB or syphilis
Macular pigmented scar (slightly smaller than ON), retinitis and overlying vitritis history
History of: eating raw meat, exposure to cats, immunocompromised risk
Macular pigmented scar, retinitis and overlying vitritis exam and evaluation
Full eye exam looking for additional ipsi/L and contra/L CR scars
>Look for AC rxn, spillover from vitritis
>high IOP in active toxoplasmosis helps support Dx
Blood work: toxoplasmosis IgG/IgM Ab titers, VDRL/RPR PPD, CXR, Toxocara ELISA, HIV test in high-risk individuals.
Toxoplasmosis A/P
Rx: typically 6 weeks, Rx immunocompromised cases longer
A: elevated IOP, Rx with IOP lowering gtts other than prostaglandins.
If immunocompromised and lesions are near the macula or disc, recommend starting primethamine, and sulfadiazine with folinic acid to prevent pyrimethamine apalastic anemia
pyrimethamine: need weekly CBC
Patient allergic to sulfa: consider clindamycin or azithromycin
Use: topical/oral steroids, except in immunocompetent patients.
Other Rx protocols: Bactrim DS bid or atovaquone (expensive but acts against both tachyzoites and dormant bradyzoites)
can observe pts who are immunocompetent but have peripheral retinal lesions and non-threatened vision.
Consider working up: TB, syphilis, sarcoid
Toxoplasmosis Education
Immunocompetent patients: good Px with observation for peripheral lesions and medical Rx for lesions threatening vision. Immunocompromised pts have poor Px and would highly recommend HAART
Multiple large CWS and areas of retinal hemorrhage surrounding a normal disc DDx
purtscher retinopathy HTN retinopathy purtscher-like retinopathy CRVO CRAO (much less likely)
Multiple large CWS and areas of retinal hemorrhage surrounding a normal disc history
Info: trauma?
h/o: pancreatitis, EtOHism, renal failure, HTN, recent retrobulbar anesthesia, intraocular injections (i.e. steroid), collagen vascular dz (lupus, scleroderma, dermatomyositis)
Multiple large CWS and areas of retinal hemorrhage surrounding a normal disc exam and evaluation
VA, APD, SLE, DFE, BP
Imaging (chest, head, orbit). Consider long bone fractures and amnio embolus (femals)
IVFA
Work-up for pancreatitis, renal failure, HTN, CVD = BP, amylase, lipase, PT/PTT, internist consult
Purscher’s retinopathy A/P:
no ocular Rx, but must Rx underlying causes
Education: f/u exams with DFE q1-2weeks in 1st month. Vision may not improve but 50% can return to baseline.
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance DDx
Stargardt’s dz
Cone’s dystrophy
hydroxychloroquine or chloroquine toxicity
Batten’s dz
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance history
Timing of vision loss, medicine, medical and family history
For Batten’s: ask about h/o dementia, seizures
aka: Spielmeyer-Vogt-Batten-Mayou RP + neuronal ceroid lipofuscinosis Lipofuscin accumulates in lysosomes Also: seizure, dementia, ataxia, vacuolization of peripheral lymphocytes Inheritance: AR, usu. Jewish girls
RPE pigmentary changes, macular yellow pisciform lesion, macular “beaten-metal” appearance exam/evaluation
Complete eye exam
Additional testing: IVFA, ERG, EOG
On IVFA = “silent choroid” pattern with abnormal ERG and EOG based on fundus appearance if Stargardt’s
subretinal hemorhage, greenish lesion deep to the retina DDx
NVAMD
POHS
trauma (old choroidal rupture)
polypoidal choroidal vasculopathy (PCV)
Well defined area of retinal necrosis = sectoral retinal hemorrhage, retinal whitening in brush-fire configuration, CWS DDx
V: (less likely BRAO) I: CMV, ARN (immunocompetent)/PORN (immunocompromised), syphilis, toxoplasmosis, Pneumocystis carinii N: lymphoma D: amnioglycoside toxicity I: sarcoidosis C:n/a A: n/a T: scolpeteria E: n/a
well-definited retinitis, hemorrhage, retinal whitening in brush-fire configuration History and Exam
Ask: immune status, esp HIV
Full eye exam: document DFE with fundus photos. Look for retinal holes and RD
Evidence of granulomatous anterior uveitis, vitritis, retinal vascuitis
*PORN pts = immunocompromised, minimal inflammation and vasculitis
Inferior arcade retinitis, hemorrhage, retinal whitening in brush-fire configuration tests
AC/vitreous tap PCR: HZV, HSV, or rarely CMV
alternatively…
blood testing: HZV, HSV (type 1 and 2) IgG and IgM
Perform HIV test, CD4 count (CD4
CMV A/P
Admit and initiate treatment with IV ganciclovir, foscarnet, cidofovir
For patients that progress despite therapy or those that have macular encroachment can use IV injections of antiviral medications
RD patients: consider PPV with SO and laser barricade to mitigate progression.
Retinal holes: consider performing laser barricade for peripheral and perimacular holes. Rx: controversial with regard to benefit.
Internist: help with HAART to improve CD4 count which will augment the patient’s ocular stability
Small white flecks in FAZ, appear to be crystalline DDx
exogenous (tamoxifen, canthaxanthine, halothane anesthetic, oxalate crystals from methoxyflurane, and talc)
Endogenous (Bietti crystalline dystrophy, hyperoxaluria, cystinosis)
Calcific drusen
hard exudates
On DFE: large elevated pigment lesion DDx
choroidal melanoma
nevus
mtz
melanocytoma (less likely 2/2 location and appearance)
On DFE: large elevated pigment lesion history
history: prior knowledge of intraocular lesion to help with onset and timing
ask about visual Sx and history of other cancers
Evaluation: U/S (lesion height and extrascleral extension)
A-scan: see low internal reflectivity
Lesion in this case: nevus unlikely and melanocytomas typically are more darkly pigmented and near the ON
Mtz: usually orange and multifocal
Choroidal melanoma A/P
Ultrasound IVFA - look for double circulation pattern (not required for dx) chest/abdomen CT - look for mtz LFTs and CBC refer to an oncologist
COMS (Collaborative Ocular Melanoma Study):
(1) observation for small choroidal melanomas
(2) plaque brachytherapy (rather than enucleation for medium-sized melanomas
(3) enucleation without preceding radiation for large melanomas
Choroidal melanoma education
mtz work-up with oncologist
Education, in a compassionate way, need for continued monitoring afterwards over years as 10 year risk of mtz related death is as high as ~50%.
Retinal horseshoe tear (with associated blood) DDx
vitreoretinal traction from syneresis
trauma
Retinal horseshoe tear history
timing of Sx, flashes, floaters
h/o trauma
h/o high myopia, family history of RD
Retinal horseshoe tear exam and evaluation
exam: careful scleral depression of the periphery of both eyes to assess for other related retinal pathology (other tears or lattice degeneration)
measure VA
if h/o trauma –> assess for commotio, evidence of open globe injury or IOFB
Retinal horseshoe tear plan and education
laser retinopexy
cryotherapy
RD precautions
Education: causes of retinal tears (vitreous syneresis and VR traction assuming no h/o trauma). Education patient regarding Sx of further retinal tears or detachments such as flashes (new) or increased floaters or curtains.
Educate regarding increased life-long risk of retinal problems in either this or the other eye.
subretinal hemorhage, greenish lesion deep to the retina DDx
NVAMD POHS trauma (old choroidal rupture) polypoidal choroidal vasculopathy (PCV) myopic degeneration central serous chorioretinopathy
subretinal hemorhage, greenish lesion deep to the retina history
History:
FH of AMD
smoking
trauma, laser therapy, living in Ohio River Valley, and HTN.
subretinal hemorhage, greenish lesion deep to the retina exam and evaluation
DFE: macula and periphery. Look for punched out CR lesions (histo spots). Look for drusen or CNV.
Macula: look for elevation, thickening, hemorrhages near suspicious area, examine OU
IVFA: look for focal leakage to ID classic vs occult
OCT: baseline retinal thickness and define retinal pathology
If substantial hemorrhage, obtain ICG to look for “hairpin” turns of retinal angiomatous proliferation (RAP lesion) or the polyp patterns seen in PCV
NVAMD A/PT study.
Use IVFA and OCT to determine extent of CNV (occult vs classic)
Consider: qmonthly anti-VEGF injections (follow macular thickness/fluid)
anti-VEGF (such as ranibizumab): visual improvement in all types of anti-VEGF agents (ANCHOR, MARINA)
bevacizumab equally effective (CATT study) but not FDA approved
AREDS2 vitamin
If lesion is temporal to fovea: (1) PDT as in TAP trial or laser photocoagulation (MPS study). neither study improved acuity or had rates of stability like MARINA and ANCHOR
NVAMD education
natural history of AMD and options for treatment
Counsel smoking cessation, prescribe AREDS vitamins to reduce rate of progression to end stage AMD (wet or GA) in other eye
Follow q4weeks
Small white
a
Endophthalmitis education
Educate patient regarding natural history of disease = guarded, with ~50% chance of a return to 20/40 or better vision over the course of the next year
Small white flecks in FAZ, appear to be crystalline exam exam/evaluation
Examine contra/L eye for drusen
examine OU for K crystals (Bietti or cystinosis)
Evidence of SRF, MA, NV that can cause hard exudates (usually circinate)
If evidence of DM or NV –> IVFA, OCT, FBS
Crystalline retinopathy (tamoxifen) A/P and education
Plan: d/c tamoxifen if pt sx
d/w patient’s oncology Rx
If aSx pt, then stopping the medication is not necessary, but would monitor closely q2-3 mo or sooner should patient have visual concerns.
Education:
natural history of condition = progressive visual decline if medication is continued. Vision can improve once discontinued. Crystals typically remain
IVFA = petalloid hyperfluorescence pattern DDx
CME (post-cataract = Irvine-Gass syndrome) Other causes of CME: "DEPRIVEN" DM Epinephrine Pars planitis RP Irvine-Gass Venous occlusion E2 (prostagalndin Niacin/Nicotinic Acid
IVFA = petalloid hyperfluorescence pattern history
MCC: complicated CE/IOL w/vitreous loss (examine for vitreous strands to the wound).
Look for inflammation and signs of complicated cataract surgery such as broken capsule, chronic inflammation. Will keep in mind chronic endophthalmitis and will look for evidence of P. Acnes.
prostaglandin gtts
peripheral bone spicule pigmentation with RP
other stigmata of DR
OCT: establish baseline macular thickness
IVFA to look for leakage
Pemberton claims that there are false negative results with OCT detecting CME 10% of the time. This seems hard to believe.
Irvine-Gass A/P
P: NSAID and pred qid and follow for resolution on OCT
lysis of vitreous strands if vitreous to wound causing vitreous traction
Systemic diamox SR 500 mg PO qd may have good results if patient can tolerate it.
Periocular or intraocular steroids can also be used but have high risk for glaucoma. Anti-VEGF has limited success with CME
Education: natural history = resolution of edema and improvement in VA with use of topical meds
F/u: q4-6weeks until CME resolution
MAs and DBHs DDx
DR Retinal vein occlusion HTN retinopathy radiation retinopathy HIV retinopathy CAR(?) hypercoagulable states
MAs and DBHs history and exam
History of: DM, HTN, radition?
Check: BP and gluocose
Complete eye exam: checking vision and IOP
Fundus exam for diabetic hemorrhages. look for vessel engorgement, macular edema, NV
MAs and DBHs evaluation
For macular edema: baseline OCT
IVFA looking for:
(1) areas of leakage or nonperfusion
(2) NV of disc or elsewhere
(3) areas of capillary non-perfusion
DR A/P
Per ETDRS, Rx with focal laser if macular edema:
thickening w/in 500 um of fovea center
hard exudates w/in 500 um of fovea w/assoc thickening
retinal thickening > 1 disc area in size w/in 1 disc diameter of fovea center
DRS: Rx w/PRP if PDR, defined as: 1/3 of the DA any NVD with VH NVE > 1/2 DA with VH NVI?NVA
DR education
natural history of DR: glucose, HTN, lipid control slows progression and decreases mortality (per Diabetes Control and Complications Trial [DCCT])
>pts w/ HgA1c > 7 were 50% more likely to experience severe vision loss in 2 yrs than those w/HgA1c
pseudophakic eye with conjunctival injection, hazy view through anterior segment, layered hypopyon, clear cornea DDx
endophthalmitis
non-infectious/autoimmune uveitis
pseudophakic eye with conjunctival injection, hazy view through anterior segment, layered hypopyon, clear cornea history
DFE looking for vitritis or subretinal abscess
Exam contra/L eye for signs of inflammation
Endophthathalmitis A/P
EVS - pts HM or better = do vitreous tap and inject
PPV for LP or worse
Inject: vancomycin 1 mg/0.1 mL and ceftazidine 2 mg/0.1 mL and culture vitreous and/or AC fluid to direct future therapy
topical Abx, steroids, cycloplegics. Follow pt daily.
Endophthathalmitis education
Educate patient regarding natural history of disease = guarded, with ~50% chance of a return to 20/40 or better vision over the course of the next year
Dark lesion under vessels w/o elevation in the choroid DDx:
nevus
CHRPE
melanoma (but no signs of lipofuscin, elevation, disruption of bruchs membrane)
Dark lesion under vessels w/o elevation in the choroid history and work-up
DFE with gonioscopy and dilation
binocular biomicroscopy: to determine elevation (gold standard)
On U/S: look for low to medium internal reflectivity or scleral excavation
>2.5 mm elevation (suspicious for melanoma)
If
Choroidal nevus Rx and education
Rx: observation for nevus
Rx if mlenaoma - COMS (small tumors = observation; medium tumors = brachytherapy [as good as enucleation])
Nevus Advice: reassure that this is benign common Nevus condition, Excellent Px, conversion to melanoma is rare.
Also advise that even benign lesions may grow. If visual Sx occur such as wavy lines or flashes to contact office immediately
Nevus F/u: q6mo to q12 mo with fundus photos
Melanoma education: up to 50% 10 year mortality with these tumors. F/u with oncologist for yearly scans and monitoring.
Red extra-macular retinal lesion (solitary) - associated with feeder artery and draining vein. lipoidal exudates suggest retinal edema approaching the fovea
retinal capillary hemangioma
retinal cavernous hemangioma
racemos angiomatosis
choroidal hemangioma
Single afferent and single efferent vessel
small vessel composition of the mass iself
presence of exudate
retinal capillary hemangioma work-up, Rx/f/u
work-up:
A and B scan U/S to confirm the inta-retinal location and also confirm high internal reflectivity consistent with a vascular tumor
Perform FA and SD-OCT fo confirm and delineate the exudate.
Rx: NO established FDA- approved Rx. Consider laser or cryotherapy of this benign tumor since the secondary exudation is nearing the fovea.
Work-up: appropriate systemic dz
Follow pt closely for progression of tumor and exudation
retinal capillary hemangioma history
FH since RCH = AD
also RCH can be phakomatosis assoc/w/RCC
retinal capillary hemangioma exam
pay special attention to proximity of secondary exudation to the fovea
Sectoral retinal hemorrrhages DDx
MCC = BRVO 2/2 DM, HTN (primary or secondary), vasculopathies DDX of causes includes: >glaucoma >tumor (hypercoagulable state) >infection (CMV 2/2 HIV) >vascular (atherosclerosis) >inflammation >iatrogenic (radiation) >trauma >autoimmune (lupus) >medications (OCP)
Sectoral retinal hemorrrhages history and Sx
Ask about HTN, DM, HL, HIV, glaucoma and examine OU
Painless decreased vision
BRVO exam
VA, IOP –> both can e affected by the BRVO from retinal edema and NVI
VA can be Px
DFE: look for venous engorgement, tortuosity, and NVD, NVI, NVE, NVA
Look for signs of DR in contra/L eye
Look for signs of chronic HTN (AV nicking and arteriolar attenuation)
BRVO work-up
OCT (quantify retinal thickness and detailed evaluation of cystic fluid) and IVFA (subtle NVE and retinal vascular nonperfusion)
gonioscopy - evaluate for PAS, NVA, NVI
BRVO Rx
Per BRAVO and CRUISE trials for BRVO and CRVO, will consider anti-VEGF Rx for macular edema.
Laser photocoagulation can be also employed per the BVOS trial.
PRP indicated for any NV dz (anteriorly or posteriorly)
Surgery for cases of anterior segment neovascular glaucoma or rarely TRD involving central macular 2/2 posterior segment neovascularization
BRVO Advice
vascular disease and HTN is the main etiology.
Px is guarded if VA is severely decreased
Goal is to Rx NV to prevent VA loss from NVG
BRVO follow-up
Short-term f/u: Follow pt qmonthly to monitor for NV and macular edema.
Long-term: q6mo and I will inform the patient’s PCP that the patient is at higher risk of stroke given recent studies correlating strokes and vessel occlusions
Encourage optical CV risk factor control: BG, BP, cholesterol, diet and exercise manangement.
DR Rx
PDR = PRP CSME = grid or focal Rx
Recently, FDA approved ranibizumab for Rx of macula edema based on RISE and RIDE clinical trial
These trails provided evidence that ranibizumab can Rx diabetic macular edema and improved vision.
SRF (dome-shaped)/serous RD DDx
Vascular/endocrine (CSCR) Neoplasm (choroidal tumor) Inflam/Idiopathic/Autoimmune (VKH; uveal effusion syndrome*; macular detachment 2/2 RRD, pigment epithelial detachment from other causes like CNV; vitelliform macular detachment) Congenital (optic pit w/macular edema) Age: retinoschisis Trauma
left out: Infection, Drugs
*Uveal effusion syndrome = rare syndrome of idiopathic exudative detachments of choroid, ciliary body and retina thought to arise from impaired posterior segment drainage usually associated with sclera thickening.
serous RD history and exam
History: stress, steroids, type A personality
Exam: examine ON for ON pit
serous RD/SRF Work-up
OCT: SRF, look for macular schisis
>ON pit: ON excavations
>thickened choroid: VKH, CSCR, uveal effusion syndrome
>vitelliform lesions
IVFA:
Look for CNV
PED: early hyperF with LATE pooling
CSCR: leakage advancing through the stages of angiography in smoke-stack pattern (10% of time), more likely to see expansile dot
ICG:
CSCR: hyperF with late staining
R/o CNV
late staining with tumor
CSCR Rx
Observation - Most cases resolve spontaneously in 6 weeks and return to baseline VA.
Laser treatment or ocular PDT in pts with:
>requirement for rapid visual recovery (airline pilots)
>poor vision in fellow eye 2/2 CSCR
>no resolution of fluid s/p several months
>recurrent episodes with poor vision
>severe forms of CSCR
Rx reduces the DURATION of Sx but does not affect final VA.
Experimental use: PO rifampin and mifepristone (RU486) in chronic or bilateral cases
CSCR advice and follow-up
advice (discuss w/patient the natural history of CSCR). Commonly found in men with type A personalities in high stress jobs. Metamorphopsia is common Sx of CSR
F/u: q4-6 wks until resolution of edema.
Dome-shaped exudative RD (without corrugation or surface membranes, no obvious traction) or mass causes DDx
VINDICATE (left out infection and drugs)
Vascular (HTN)
Neoplasm (retinal capillary hemangioma/VHL, choroidal hemangioma, choroidal malignant melanoma)
Inflammatory/Idiopathic/Autoimmune (uveitis: VKH, SO, pars planitis, posterior scleritis); CSCR, (glomerulonephritis)
Congenital (Coats’ dz)
Trauma
Endocrine/Metabolic (hypoTH, pre-eclampsia/eclampsia)
Dome-shaped exudative RD (without corrugation or surface membranes, no obvious traction or mass) EXAM
Exudative RD located inferiorly, display shifting fluid, and have a smooth appearance
Appearance of a smooth RD behind lens is almost pathognomonic for exudative RD.
Chronic exudative RD can lead to NVG
DFE: evaluate for peripheral tears/breaks, R/O RRD, and R/o TRD (usually taut, immobile, concave surface that does NOT extend to ora serrata)
Dome-shaped exudative RD (without corrugation or surface membranes, no obvious traction or mass) causes EVALUATION
B-scan - confirm shifting fluid, choroidal thickness, evlaute for masses
A-scan: evaluate internal reflectivity if mass is found
OCT - verify thickened choroid using EDI (enhanced depth imaging) in uveitis, image SRF +/- CME === IVFA - useful to evaluate: tumors (vasculature, feeder vessels) vascular abnormalities
ICG - superior to FA to show intrinsic vaculature, hot spots, washout phenomenon for tumors
High internal reflectivity on U/S
choroidal hemangioma
Choroidal hemangioma Rx
Individualized Rx based on:
>potential for visual recovery
>Sx extent
>loss of vision
Rx goal: induce tumor atrophy with resolution of SRF and tumor-induced foveal distortion w/o destryoing fxn of overlying retina
Goal: not to obliterate tumor
Rx options: laser photocoagulation (moderately intense, white rxn on tumor surface to eliminate serous exudation), cryotherapy, ocular photodynamic therpay (PDT) with verteporfin (visudyne) using standard treatment parameters
I-125 plaque brachytherapy, low dose XRT
Choroidal hemangioma Px and education
VA loss can be progressive and irrversible when the fovea is involved in chronic cases
poor VA results: can be expected despite resolution of fluid exudates from chronic macular edema and photoreceptor loss
ARN Rx
Systemic IV acyclovir until resolution of retinitis then oral for 1-2 mo
Alternatively: lesions more peripheral, then use oral therapy with valacyclovir instead of IV therapy
Alternative: ganciclovir
Follow BUN and Creatinine for nephrotoxicity
Both PO and topical steroids after pt begins to respond to prevent inflammatory complications
ARN education
high risk of developing RRD with numerous hole snad giant tears 2/2 retinal necrosis
Careful observation of fellow eye
Chronic vitritis history
ROS:
>unexplained fevers, chills, night sweats, fatigue, HA, weight loss
PMH: cancer history
> LA (cervical, supraclavicular), CNS Sx?
Chronic vitritis DDx
I: toxoplasmosis, syphilis, TB, ARN
N: lymphoma (MCC = primary intraocular lymphoma)
I: birdshot, pars planitis, sarcoidosis, multifocal choroiditis, APMPPE, Behcet’s
Not used: vascular, drugs, congenital, age, trauma, endocrine
Chronic vitritis work-up
CBC, ESR ACE HLA-B27, HLA-B51 ANA VDRL/RPR/FTA-ABS/MHA-TP
If negative –> MRI of brain and LP for CNS cytology
If negative –> PPV with UNdilute vitreous Bx (~1cc), flow cytometry for B and T cell markers, kappa/lambda light chains
Ancillary tests: IL6, IL10 (high IL10 to IL6 ratio = suggestive of intraocular lymphoma)
Intra-ocular lymphoma (diffuse large B-cell NHL) A/P
Rx: intravitreal MTX and rituximab
Orbit XRT if no CNS involvement
Most pts have CNS involvement, so Rx with chemoRx with blood-brain disruption or high-dose systemic MTX
Perform: complete mtz survey and bone marrow Bx
Intra-ocular lymphoma (diffuse large B-cell NHL) education/Px
60-80% develop CNS lymphoma w/mean 29 mo. Poor Px if CNS involvement.
Granulomatous uveitis mnemonic for DDx
SVS by SW-PTT (mnemonic):
Sarcoidosis,
VKH,
Sympathetic Ophthalmia,
Syphilis,
Wegener’s
Phacoantigenic Uveitis/Glaucoma,
Toxocariasis (posterior pole peripheral granulomas), toxoplasmosis, (anterior & posterior granulomatous),
TB.
Other rare conditions: leprosy, brucellosis, P. acnes, fungal infection (cryptococus, aspergillus), HIV
Granulomatous uveitis exam
ciliary injection AC cell/flare; hypopyon iris nodules, rubeosis, synechiae increased/decreased IOP cataract pars planitis optic nerve hyperemia chorioretinitis periphlebitis CME
Granulomatous uveitis work-up
CBC with differential, ESR
VDRL/RPR, FTA-ABS, MHA-TP
ELISA or indirect immunofluorescence assay (IFA) for toxoplasma IgM and IgG titers
ACE, lysosyme
Other lab tests: ANA, RF, anti-CCP ELISA for Lyme IgM and IgG HIV Ab CXR or CT scan (sarcoidosis, TB) sacroiliac radiograph (anklyosing spondylitis) UA
If unclear Dx:
HLA-A29 (birdshot)
vasculitis (ANCA = Wegener’s, polyarteritis nodosa)
Raji cell and C1q binding assays for circulating immune complexes (SLE, systemic vasculitis)
complement C3, C4, total complement (SLE, cryoglobulinemia, glomerulonephritis), soluble IL-2 receptor
Toxoplasmosis chorioretinitis Rx
Anterior inflammation: topical steroids and cycloplegia
Systemic steroids for posterior pole lesions or those with intense
Immunocompetent individuals: small peripheral lesions may be observed since they often heal spotaneously
Rx with antibiotics 4-6 wks to kill tachyzoites (no effect on cysts) in the following conditions:
>decreased VA
>moderate-severe vitreous inflammation
>lesions that threaten macula, papillomacular bundle, ON
most pts respond well to: TMP-SUF
aggressive lesions or posterior pole lesions: triple therapy (pyrimethamine, folinic acid and one of the following… sulfadiaizine, clindamycin, clarithromycin, azithromycin, atovaquone)
If immunocompromised and high-risk, then may need to do prophylactic Rx.
