Lecture 8 - Vascular Disorders and CME (part 1) Flashcards
where is the fluid located in cystoid macular edema (CME)?
intraretinal in and between muller glia cells, occasionally beneath neurosensory retina (SRF) in outer plexiform layer/Henles layer = forms a petalloid or flower-petal pattern
what does CME look like on FA?
multiple small perifoveal leaks from altered capillary permeability and late pooling in petaloid pattern, disc staining
what does CME look like on OCT?
diffuse retinal thickening with cystic areas of low reflectivity more prominently in the inner nuclear layer and outer plexiform layers (honeycomb-like cystoid spaces)
what is the CME etiology?
diabetic retinopathy, CRVO, BRVO, uveitis (pars planitis), RP, ocular surgery/laser, prostaglandin analogues, CNVM, RD, choroidal hemangioma, Niacin supplement
when does CME happen with cataract surgery?
peak 6-10 weeks post-op with spontaneous resolution 95% during uncomplicated surgeries within 6 months
what causes CME to happen post cataract surgery?
post-op inflammation, surgical complications (vitreous loss or iris prolapse), possible photic effects from microscope
what is the treatment for CME?
high rate of spontaneous resolution, combination steroid + NSAID, triamcinolone acetonide injection, systemic acetazolamide (chronic/RP), anti-VEGF (DM), YAG for vitreous adhesions
what are the retinal findings in Coats disease?
vascular dilations (retinal telangiectasia), ectatic arterioles, microaneurysms, venous dilations (phlebectasias), exudates and fusiform capillary dilations (exudative RD)
what 3 things can patients present with Coats disease?
decreased VA (43%), strabismus (23%) and leukocoria (20%) rarely a painful red eye with NVG
who typically presents with coats disease?
males, unilateral, 80% before age 20 and 66% before age 10 (more severe/acute progressive if under age 4), linked to chromosome 4
what is the treatment for coats disease?
photocoagulation, cryotherapy and in severe cases RD surgery
what is parafoveal (juxtafoveal) retinal telangiectasia?
focal retinal gliosis and telangiectasia of the capillary bed, VA loss occurs from capillary incompetence and exudation, there are refractile changes in advanced cases
what are the 3 types of parafoveal (juxtafoveal) retinal telangiectasia?
type 1 = unilateral, congenital, or acquired, type 2 = bilateral, type 3 = bilateral with retinal capillary obstruction
how does parafoveal (juxtafoveal) retinal telangiectasia look on FA?
the telangiectasic vessels leak, microaneurysms close to the macula and they are connected with small lines
what is seen in type 1 parafoveal (juxtafoveal) retinal telangiectasia?
aneurysmal telangiectasia, usually males/variant of coats disease, circinate exudation, and leber military aneurysms
what is seen in type 2 parafoveal (juxtafoveal) retinal telangiectasia? (most common)
perifoveal telangiectasia, bilateral, M = F, macular thickening, gray appearance, cystic fovea, temporal fovea, later intralesional RPE migration, CNVM, 1/3 abnormal GTT
what is seen in type 3 parafoveal (juxtafoveal) retinal telangiectasia?
progressive vision loss from the obliteration of the perifoveal capillaries
what is the treatment for parafoveal (juxtafoveal) retinal telangiectasia?
type 1 = photocoagulation and types 2/3 = anti-VEGF
what are some differential diagnoses for parafoveal (juxtafoveal) retinal telangiectasia?
BRVO, DR, CME (chronic), radiation retinopathy, carotid artery disease
where do arterial macroaneurysms occur?
2nd order arterioles, usually at an A-V junction, temporal arcades
who typically gets arterial macroaneurysms?
over age 70, HTN and can follow a CRVO
what causes the vision loss in arterial macroaneurysms?
embolic/thrombotic occlusion of the endarteriole (white infarct), sub-ILM hemorrhage or intraretinal, subretinal or vitreous hemorrhage (red infarct), capillary telangiectasia, macular edema and circinate exudation
what does the FA look like with arterial macroaneurysms?
hypofluorescence = blockage + hyperfluorescence = pooling in aneurysm
what is the treatment for arterial macroaneurysms?
treat if vision threatening = mild laser adjacent to aneurysms
what are the 3 phakomatoses?
retinal angiomatosis (arterial), congenital retinal arteriovenous malformations (arterial + venous) and retinal cavernous hemangioma (venous)
what is a retinal angiomatosis?
spherical orange-red capillary hemangioblastoma - fed by dilated tortuous artery and drained by engorged vein (subretinal or epiretinal lesions)
what are the complications of a retinal angiomatosis?
serous RD, macular exudation, vitreous hemorrhage, TRD, possible NVD/NVI, NVE
what causes a retinal angiomatosis?
acquired or hereditary (AD)
von hippel lesions = retinal and optic disc lesions
von hippel-lindau lesions = retinal angiomatosis + CNS and visceral lesions
what can cause death with retinal angiomatosis?
cerebellar hemangioblastoma and renal cell carcinoma
what is the management for a retinal angiomatosis?
imaging to rule out CNS/visceral lesions, early diagnosis is key, wide-angle FA, photocoagulation, PDT with verteporfin and cryotherapy
what is a congenital retinal arteriovenous malformation?
a developmental anomaly - no intervening capillary bed exists (racemose angioma), single or complex anastomotic system, unilateral, non-hereditary, retina or optic nerve involved
what does a congenital retinal arteriovenous malformation look like on FA?
no leakage - hyperfluorescence of entire tumor
what is a retinal cavernous hemangioma?
grape-like clusters of thin-walled saccular angiomatous lesions inner retina or optic nerve head, sporadic, AD inheritence
what does a retinal cavernous hemangioma look like on FA?
blood flow is stagnant, slow/delayed filling, plasma-erythrocyte layering, no leakage, no RD/SRF/exudates
what is the treatment for retinal cavernous hemangioma?
usually asymptomatic but can bleed from vitreous traction - treat if there is bleeding only (laser or cryotherapy)
what causes radiation retinopathy?
ionizing radiation (external beam or plaque) - delayed onset (18m), slowly progressive, microangiopathy (similar to DM), exposure to doses 30-35 grays
what are the retinal findings in radiation retinopathy?
CWS, retinal hemorrhages, microaneurysms, perivascular sheathing, capillary telangiectasias, macular edema, and disc edema, capillary non-perfusion on FA (NVE, NVD, NVI)
what are some other ocular complications of radiation retinopathy?
optic atrophy, CRVO/CRAO, CNVM, NVG, TRD
what is the treatment for radiation retinopathy?
macular edema = PRP or intravitreal injection of trimcinolone acetonide or anti-VEGF
what is the primary cause of VA loss in patients with radiation retinopathy?
edema + ischemia (not the CME)
what is the pathogenesis of sickle cell disease?
a base substitution in the B-globin gene results in the amnio acid replacement GAG to GTG (glutamic acid by Valine) - the sickle cell hemoglobin is deoxygenated and it can block blood flow in microcirculation
which type of sickle cell causes the most ocular complications? systemic?
ocular = SC and SThal systemic = SS (most common)
what causes retinopathy from sickle cell disease?
peripheral arteriolar occlusion leading to capillary non-perfusion - neovascularization at the border between perfused and non-perfused retina
what are the non-proliferative signs of sickle cell?
conjunctival coma sign, iris atrophy, salmon patch - halo - iridescent spots, black sunburst, arterial silver wiring, foveal depression sign, optic nerve sign and angioid streaks
what is the conjunctival coma sign in non-proliferative sickle cell?
dark red coma/corkskrew from vascular occlusion (SS»SC), usually in lower bulbar conjunctiva, vasodilation makes them disappear and vasoconstriction (phenylephrine) increases them
what happens with the salmon patch in non-proliferating sickle cell?
pre-retinal or superficial 1DD vessel blow-out from ischemia - halo develops and changes from red to orange to salmon - residual simple with ILM reflecting at margin with refractile granules of hemosiderin-laden macrophages (may have schisis cavity ILM and neurosensory retina)
what is the black sunburst sign in non-proliferative sickle cell?
intraretinal RPE hypertrophy, hyperplasia and pigment migration, may have iridescent spots, speculated appearance and perivascular location, may develop CNVM
what is the arteriole silver wiring sign in non-proliferative sickle cell?
more common in HbSS - also seen in CRVO/BRVO, HTN
what is the foveal depression sign in non-proliferative sickle cell?
increased FAZ, degenerative ischemic thinning of the inner retina (also seen in albinism)
what is the optic nerve sign in non-proliferative sickle cell?
small red dots or linear/Y shaped configuration, pre-capillary arterioles plugged with sickled erythrocytes
when are angioid streaks seen in non-proliferative sickle cell?
6% in SS and even seen in AS
what are the proliferative stages of sickle cell?
1 = peripheral arteriole occlusions + peripheral nonperfusion, 2 = peripheral arteriovenular anastomoses, 3 = preretinal “sea fan” neo, 4 = vitreous hemorrhage, 5 = tractional RD
how is proliferative sickle cell different than PDR?
neo PDR begins post-equatorially and PSR is more peripheral with a sea fan appearance
what is the management for sickle cell?
all african americans should be screened for sickle cell trait (traumatic hyphema), ischemic optic neuropathy may result from high IOP, early AC washout with hyphema with increased IOP, cautious with CAIs (worsen sickling) and treat NVE with light laser to asvascular retina
in sickle cell, which part of the retina do you use scatter photocoagulation?
all avascular areas - all the neo and capillary drop out
when is surgery indicated in sickle cell?
non-clearing vitreous hemorrhage, RD, retinal tears typically at the base of the sea fan precipitated by PRP
