Lecture 3/6 - acquired macular diseases Flashcards
what causes central serous chorioretinopathy (CSR)?
idiopathic = altered barrier/deficient RPE pump or leaking choriocapillaris (retina + choroid problem)
what is the pathophysiology of CSR?
fluid accumulates under RPE - breaks through and accumulates under the retina - causes an RPE/retina detachment (localized and well defined)
who typically gets CSR?
healthy 25-55 year old male, white/hispanic/asian, Type A personality, possible stress etiology
what are the associations with CSR?
exogenous or endogenous steroids, cushings syndrome, organ transplants, SLE, HTN, sleep apnea, GERD, psych meds, pregnancy
what are the signs/symptoms of CSR?
sudden onset blurred/dim vision (hyperopic shift), micropsia, metamorphopsia, paracentral scotoma, decreased color vision, VA 20/20 - 20/200, may have headache, unilateral or asymmetric bilateral
what are the 3 patterns of CSR in FA?
expansile dot pattern (most common), smokestack pattern (most characteristic), and diffuse pattern
what is seen on an OCT of CSR?
increased choroidal thickness and fluid under the RPE/retina (if there are clusters of deposits, it has been there awhile)
what is seen with fundus autofluorescence (FAF) in CSR?
pigment mottling in the area of RPE disturbance and altered autofluorescence corresponding to site of focal RPE leak (precipitates)
what is seen with ICG in CSR?
“washout pattern” in late phase - helps distinguish atypical diffuse CSR in older patients from occult CNVM and idiopathic polypoidal choroidal vasculopathy
what is the prognosis for CSR?
generally good except in chronic, recurrent cases and bullous CSR
most (80-90%) have spontaneous resolution in 3-4 months
what treatment is available for CSR?
observe 3-6 months - then laser photocoagulation at leakage or PDT if it is needed
RTC 3-4 weeks after laser to monitor for CNVM
what are signs of chronicity in CSR?
cystic changes (microcavities) in neurosensory retina or widespread RPE abnormalities
what is an optic nerve pit?
small, hypopigmented, yellow/white, oval/round, excavated colobomatous defect
where is an optic nerve pit usually located?
inferior temporal at disc margin - mostly unilateral and asymptomatic
what can an optic nerve pit cause?
serous macular detachments - can be cerebral spinal fluid or liquid vitreous
what will an OCT show for a serous macular detachment caused by an optic nerve pit?
macular schisis and SRF (subretinal fluid)
what is the treatment for a serous macular detachment caused by an optic nerve pit?
laser along the edge of the optic nerve pit forming a barrier to fluid, use a gas bubble or vitrectomy
what is an epiretinal membrane (ERM)?
semitranslucent, avascular, fibrocellular membrane = covers the internal limiting membrane (ILM)
what cells are involved with an ERM?
proliferating glial (astrocytes or muller) cells, hyalocytes, fibroblasts, myofibroblasts and macrophages
what causes an ERM?
most common is idiopathic = abnormal vitreoretinal interface with a PVD or vitreous separation after age 50 (10-20% are bilateral and asymmetric)
what are secondary causes of an ERM?
retinal vascular occlusions, uveitis, trauma, intraocular surgery, retinal breaks
how do you know if a patient with a PVD will develop an ERM?
it depends on where the plane of the vitreous is located - if the hyalocytes are detached with the vitreous they die, if they stay they proliferate on ILM
what are the grading classifications of an ERM?
mild/cellophane (no metamorphopsia), moderate (retinal striae), severe (macular pucker)
what are the symptoms of an ERM?
micropsia, macropsia, metamorphopsia, decreased VA, diplopia, floaters, or asymptomatic
what are the signs of an ERM?
retinal vessel traction (straightening of the perimacular vessels), optic nerve staining on FA, intra-retinal hemorrhages, superficial retinal whitening CWS, pseudohole
what is the treatment for an ERM?
monitor with amsler if mild, only treat if vision is less than 20/50 or there is intolerable distortion = PPV + ERM peel
what is vitreomacular traction syndrome (VMT)?
an incomplete separation of the posterior vitreous to the posterior pole at the macula
what causes a VMT?
a partial PVD with cellular proliferation
what are the signs of a VMT?
abnormal opacities in central vitreous, traction over the macular and optic nerve, similar signs to ERM without an ERM (retina is distorted, cystic, tented with shallow detachment)
what is seen on FA with VMT?
macular vessels leak, disc distortion, early CME
what is the treatment for VMT?
same as ERM - only if the VA is less than 20/50 or severe distortion, may have spontaneous resolution
what causes an idiopathic macular hole?
tractional forces at early PVD (older patients, F>M)
what are the stages of an idiopathic macular hole?
stage 0/premacular hole, stage 1a foveolar detachment, stage 1b foveal detachment, stage 2 full thickness (less than 400um), stage 3 fully developed (greater than 400um) +/- operculum and stage 4 fully developed hole + PVD
what happens when an idiopathic macular hole is at stage 0?
perifoveal PVD, subtle loss of foveal depression and normal VA (most do not progress)
what happens when an idiopathic macular hole is at stage 1a?
loss of foveal depression + small yellow spot (pseudocyst with perifoveal vitreous detachment)
what happens when an idiopathic macular hole is at stage 1b?
loss of foveal depression + small yellow ring
what happens when an idiopathic macular hole is at stage 2?
dehiscence as a tractional break “roof” of pseudocyst,
what happens when an idiopathic macular hole is at stage 3?
hole is >400um, rim is elevated/thickened, VA 20/40-20/200, posterior hyaloid attached to optic disc/detached from fovea, +/- operculum
what happens when an idiopathic macular hole is at stage 4?
fully developed hole + complete PVD
what is seen on the FA in an idiopathic macular hole?
window defect (loss of normal tissue that was blocking the macula)
what is the management for an idiopathic macular hole?
spontaneous resolution in 50% of cases - no treatment if stage 1
stages 2-4 = surgery only if VA is less than 20/50 (vitrectomy + peel and gas bubble for positioning)
what is ocular ischemic syndrome (OIS)?
rare, but vision-threatening, condition associated with severe carotid artery occlusive disease (stenosis or occlusion) leading to ocular hypoperfusion (slowly progressive VA loss)
what is OIS associated with?
periorbital aching pain, amaurosis fugax, prolonged recovery after exposure to a bright light (post stress test)
what is seen on slit lamp examination with OIS?
NVI, increased IOP, AC reaction
what is it called if there is only posterior segment findings vs. anterior + posterior?
posterior = venous stasis syndrome
anterior + posterior = OIS
who typically gets OIS?
older patients (50-70) M>F, associated with HTN, DM, CAD
what are the OIS signs seen during DFE?
narrowed arteries, dilated but not very tortuous veins, unilateral (80%) microaneurysms and hemorrhages (deep, round, mid-periphery), NVD/NVE
what is a differential diagnosis of OIS?
CRVO - this has normal artery pressure vs. OIS has low artery pressure and central retinal artery will collapse with gentle pressure
what is seen on FA with OIS?
delayed choroidal filling, delayed arteriovenous transit time and prominent vascular staining (arteries)
what is seen on ERG in OIS?
diminished amplitude of A and B waves = inner and outer retinal ischemia
what is the etiology of OIS?
atherosclerosis, inflammatory conditions (Eisenmenger syndrome, giant cell arteritis, etc.)
what is the treatment if the patient develops NVI in OIS (90% are blind within 1 year)?
full scatter PRP
what is the treatment for OIS?
carotid artery stenting and endarterectomy
what is purtscher retinopathy?
loss of vision after acute compression injuries to the thorax or head, unilateral or bilateral loss
what are the signs of purtscher retinopathy?
large CWS, hemorrhages, retina edema surrounding the optic disc, APD
what is seen on FA with purtscher retinopathy?
arteriolar obstruction and leakage
what are some conditions associated with purtscher or purtscher-like retinopathy?
trauma, acute pancreatitis, amniotic fluid embolism, autoimmune diseases
what is the pathophysiology of purtscher retinopathy?
injurry-induced complement activation - granulocyte aggregation and leukoembolization - arterial occlusion (especially peripapillary)
other causes = fat emboli from bone, air emboi
what is valsalva or venous retinopathy?
increase in venous pressure - ruptured small superficial capillaries in the macula = hemorrhagic detachment of ILM
what are the signs/symptoms of valsalva or venous retinopathy?
VA mildly reduced,vitreous hemorrhage or subretinal hemorrhage are possible
what are the differential diagnoses for valsalva or venous retinopathy?
PVD and macroaneurysms
what is seen on the OCT of valsalva or venous retinopathy?
it looks like a macular hole but the top layers are still intact
what is hyperviscosity syndrome?
increased viscosity secondary to elevated protein levels = causes decreased flow and damage to vessels (leakage/ischemia)
who typically gets hyperviscosity syndrome?
younger patients, athletes (symptoms do not match history - blood work is usually needed)
what are the signs/symptoms of hyperviscosity syndrome?
CWS, exudates, CRVO is possible
what conditions have high protein levels (hyperglobulinemia) seen in hyperviscosity syndrome?
waldenstrom’s, multiple myeloma, SLE, HIV, Rh+ve, rheumatoid arthritis
when is HIV retinopathy seen?
70% in advanced disease, 40% in symptomatic intermediate stage HIV and 1% in asymptomatic HIV infection
what distinguishes HIV retinopathy from CMV?
the CWS are smaller, superficial location, no progression and tendency to resolve over weeks-months
what retinal findings are seen in HIV retinopathy?
CWS, hemorrhages (flame and blot), telangiectatic vascular changes and microaneurysms, areas of capillary non-perfusion (NVD/NVE), retinal vein and artery occlusions
what does interferon retinopathy look like?
retinal hemorrhages (superficial, linear, patchy), CWS around optic disc, ischemia on FA, VA is usually unaffected, unilateral or bilateral
what is atypical in interferon retinopathy?
BRVO, pre-retinal hemorrhage and VA affected
when does interferon retinopathy show up?
2 weeks to 3 months after starting interferon therapy (incidence depends on initial dose = DM patients have a higher incidence/progression)
what is the treatment for interferon retinopathy?
disappears spontaneously during therapy or rapidly after stopping therapy
when is talc retinopathy seen?
usually with long term drug user - they develop collaterals in the lungs (venous and artery anastomose) and gets distributed in the retina
what is Terson syndrome?
a vitreous, sub-ILM, or sub-hyaloid hemorrhage caused by an abrupt intracranial hemorrhage (blood in brain = subarachnoid or subdural hemorrhage)
between ages 30-50
what causes terson syndrome?
possible an acute intracranial hemorrhage - acute rise in the intraocular venous pressure, peripapillary and retinal vessels rupture
what is the treatment for terson syndrome?
vitrectomy if non-resolving (VA unaffected once the blood clears - spontaneously)
what are the signs of child abuse syndrome?
periorbital ecchymosis, traumatic retinoschisis and retinal folds
what causes the retinal damage in child abuse syndrome?
contrecoup forces from trauma, oscillating vitreous traction of formed vitreous
