Retinal Diseases and Vitreoretinal Surgery Lecture Notes

Comprehensive practice flashcards covering the pathophysiology, diagnosis, clinical trials, and management of retinal and vitreoretinal diseases as discussed in the lecture notes.

Early AMD

Characterized by multiple small drusen with a diameter $< 63\,\mu m$, a few intermediate drusen ($63-124\,\mu m$), or minor RPE abnormalities, with typically unaffected visual acuity.

Intermediate AMD

Defined by numerous intermediate drusen ($63-124\,\mu m$), at least one large drusen ($\geq 125\,\mu m$), or non-central geographic atrophy.

Late AMD

The vision-threatening stage of the disease defined by the presence of either geographic atrophy (GA) or neovascular AMD (nAMD).

Geographic Atrophy (GA)

Also known as "dry" late AMD; characterized by well-demarcated progressive atrophy of the RPE, overlying photoreceptors, and underlying choriocapillaris.

Neovascular AMD (nAMD)

Also known as "wet" AMD; defined by the growth of abnormal blood vessels from the choroid into the sub-RPE or subretinal space.

Window Defect

A clinical examination and fluorescein angiography finding in Geographic Atrophy caused by RPE atrophy and loss of pigment.

AMD Worldwide Prevalence

Affects an estimated $8.7\%$ of the global population and is the leading cause of irreversible blindness in individuals over $50$ in developed countries.

Smoking (AMD)

The most significant modifiable risk factor, associated with at least a two-to-threefold increased risk of developing late AMD.

CFH and ARMS2/HTRA1

The key susceptibility loci for age-related macular degeneration located on chromosome 1 and chromosome 10, respectively.

Hard Drusen

Small drusen ($< 63\,\mu m$) that appear yellow with distinct borders.

Soft Drusen

Larger drusen ($\geq 125\,\mu m$) that appear pale yellow-white with indistinct borders.

Reticular Pseudodrusen (SDD)

Ill-defined yellowish-white lesions arranged in a reticular pattern, histologically located above the RPE in the subretinal space.

Cuticular Drusen

Small, uniform, round, pale-yellow lesions that create a "stars-in-the-sky" or "sawtooth" appearance on fluorescein angiography.

iRORA

Incomplete RPE and Outer Retinal Atrophy; an OCT-based precursor to GA characterized by disruption of the RPE and photoreceptor degeneration without full-thickness loss.

cRORA

Complete RPE and Outer Retinal Atrophy; the OCT correlate of GA requiring choroidal hypertransmission ($\geq 250\,\mu m$), RPE loss, and photoreceptor degeneration.

Pegcetacoplan (SYFOVRE)

A $C3$ inhibitor demonstrated in the DERBY/OAKS trials to reduce the rate of Geographic Atrophy lesion growth.

Avacincaptad Pegol (IZERVAY)

A $C5$ inhibitor demonstrated in the GATHER1/GATHER2 trials to reduce the growth rate of GA lesions.

MNV Type 1

Macular neovascularization where the neovascular complex is located beneath the RPE.

MNV Type 2

Macular neovascularization where the complex has breached the RPE and proliferates in the subretinal space.

MNV Type 3 (RAP)

Also known as Retinal Angiomatous Proliferation; originates from the deep retinal capillary plexus and grows toward the outer retina and choroid.

RPE Tear

A rip in the RPE layer often occurring with large, vascularized pigment epithelial detachments (PEDs), showing a hyper-autofluorescent edge.

ForeseeHome

An advanced home monitoring system using preferential hyperacuity perimetry to detect conversion to nAMD.

Ranibizumab (Lucentis)

An antibody fragment VEGFA-blocker established by the MARINA and ANCHOR trials for nAMD treatment.

Aflibercept (Eylea)

A fusion protein decoy receptor established by the VIEW 1 and 2 trials as non-inferior to ranibizumab.

Brolucizumab (Beovu)

A single-chain variable fragment associated with a rare but significant risk of occlusive retinal vasculitis.

Faricimab (Vabysmo)

A bispecific antibody targeting both VEGF-A and Angiopoietin-2 (Ang-2), allows dosing intervals up to $16$ weeks.

Treat & Extend (T&E)

A regimen where the treatment interval is gradually extended after achieving a dry macula, personalizing injection frequency to disease activity.

Central Serous Chorioretinopathy (CSC)

A condition characterized by serous retinal detachment due to primary choroidal vasculopathy and secondary RPE decompensation.

Pachyvessels

Dilated Haller's layer vessels in the choroid, causing attenuation of the overlying choriocapillaris and Sattler's layer.

Pachychoroid Pigment Epitheliopathy (PPE)

The mildest form of the pachychoroid spectrum, featuring RPE changes over pachyvessels without subretinal fluid.

Polypoidal Choroidal Vasculopathy (PCV)

A variant of Type 1 MNV featuring a branching vascular network and terminal aneurysmal polyps, seen in the pachychoroid phenotype.

Venous Overload Choroidopathy

A hypothesis that impaired choroidal venous outflow at the vortex veins leads to congestion and hyperpermeability in CSC.

Inkblot and Smokestack

Traditional patterns of active RPE leakage seen during fluorescein angiography in acute cases of CSC.

Leopard Spots

Granular, window-defect-like areas of RPE atrophy seen in chronic central serous chorioretinopathy.

Chronic CSC

Defined by persistent subretinal fluid lasting longer than $6$ months, often associated with RPE decompensation and outer retinal atrophy.

Type 1 Diabetes

An autoimmune disease causing absolute insulin deficiency, typically with juvenile onset.

Type 2 Diabetes

Characterized by insulin resistance and relative insulin deficiency, typically associated with obesity and adult onset.

Severe NPDR (4-2-1 Rule)

Presence of severe retinal hemorrhages in $4$ quadrants, venous beading in $2+$ quadrants, or moderate IRMA in $1+$ quadrant.

Microaneurysms

Focal saccular outpouchings of retinal capillaries and the earliest clinical sign of diabetic retinopathy.

Cotton Wool Spots

Nerve fiber layer infarcts resulting from capillary occlusion, appearing as white patches on the retina.

IRMA

Intraretinal Microvascular Abnormalities; remodeled capillary beds or shunts that bypass areas of non-perfusion, indicating pre-proliferative disease.

Clinically Significant Macular Edema (CSME)

Retinal thickening/hard exudates within $500\,\mu m$ of the center, or a zone of thickening one disc area in size within one disc diameter of the center.

Proliferative Diabetic Retinopathy (PDR)

Characterized by neovascularization on the disc (NVD) or elsewhere (NVE) or the presence of vitreous/preretinal hemorrhage.

Glycemic Memory

The concept from DCCT/UKPDS trials that early intensive glycemic control provides long-term protection against DR progression.

Fenofibrate

A lipid-lowering agent proven in the ACCORD-Eye and FIELD studies to reduce DR progression and the need for laser treatment.

Keith-Wagener-Barker (KWB) Grade 4

The most severe grade of hypertensive retinopathy, characterized by Grade 3 signs plus optic disc edema (papilledema).

Elschnig Spots

Hyperpigmented spots with a halo of hypopigmentation, representing areas of healed RPE infarcts in hypertensive choroidopathy.

Ischemic CRVO

Defined by $> 10$ disc areas of capillary non-perfusion on FA; carries a $30-50\%$ risk of neovascular glaucoma.

Ocular Ischemic Syndrome (OIS)

Results from chronic severe ocular hypoperfusion, usually due to $> 90\%$ stenosis of the ipsilateral internal carotid artery.

Cherry-Red Spot

The classic clinical sign of Central Retinal Artery Occlusion (CRAO) caused by retinal opacification contrasting with visible choroid at the fovea.

Susac Syndrome

A rare autoimmune endotheliopathy defined by the triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss.

Arterial Macroaneurysms

Acquired focal dilations of retinal arterioles that can cause chronic exudation or acute hemorrhage.

Black Sunbursts

Patches of RPE hypertrophy at sites of resolved deep retinal hemorrhages in Sickle Cell Retinopathy.

Sea-fan Neovascularization

The classic configuration of neovascularization in Proliferative Sickle Cell Retinopathy, occurring at the junction of perfused and non-perfused retina.

Irvine-Gass Syndrome

Cystoid macular edema occurring after cataract surgery due to breakdown of the blood-retinal barrier.

Coats Disease

An idiopathic condition characterized by retinal telangiectasia and massive subretinal exudation, typically affecting young males unilaterally.

Von Hippel-Lindau Disease

An autosomal dominant syndrome featuring retinal capillary hemangioblastomas and systemic tumors like renal cell carcinoma.

Purtscher Flecken

Characteristic polygonal white patches seen in the retina after severe trauma or acute pancreatitis.

Terson Syndrome

Intraocular hemorrhage occurring in the setting of acute intracranial hemorrhage, most commonly subarachnoid hemorrhage.

Plus Disease

A severe indicator in ROP characterized by venous dilation and arteriolar tortuosity of the posterior pole vessels.

High Myopia

Defined by the International Myopia Institute as a spherical equivalent refractive error ($SER$) of $\leq -6.00\,D$.

Pathologic Myopia

High myopia accompanied by structural changes such as posterior staphyloma, myopic maculopathy, or optic neuropathy.

Gamma Zone

An area of bare sclera between the optic disc border and Bruch's membrane opening (BMO) seen in myopic eyes.

Lacquer Cracks

Fine linear breaks in the RPE-Bruch's membrane-choriocapillaris complex, representing a risk factor for myopic CNV.

Orthokeratology (Ortho-K)

The overnight wear of rigid lenses to temporarily reshape the cornea and create peripheral myopic defocus to slow axial growth.

Myopic Traction Maculopathy (MTM)

Also known as myopic foveoschisis; characterized on OCT by splitting of retinal layers due to vitreoretinal traction in a staphyloma.

Knudson's "Two-Hit Theory"

Explains the genetic basis of retinoblastoma, requiring two somatic hits (sporadic) or one germline plus one somatic hit (hereditary).

Trilateral Retinoblastoma

Bilateral retinoblastoma combined with a concurrent intracranial neuroblastic tumor, usually in the pineal gland.

Plaque Brachytherapy

A treatment modality for medium-sized intraocular tumors where a radioactive source (e.g., Iodine-125) is sutured to the sclera.

Uveal Melanoma

The most common primary intraocular malignancy in adults, classically appearing as a pigmented dome- or mushroom-shaped choroidal mass.

Monosomy 3

The loss of one copy of chromosome 3; it is the most powerful predictor of metastatic disease in uveal melanoma.

Vitreoretinal Lymphoma (VRL)

A primary CNS lymphoma mimic that often presents as a "masquerade syndrome" resembling chronic uveitis.

CHRPE

Congenital Hypertrophy of the RPE; flat, pigmented lesions that, if multiple or atypical, are linked to Familial Adenomatous Polyposis.

SUN Classification

Standardization of Uveitis Nomenclature used to grade aqueous cells and flare and to categorize uveitis anatomically.

Laser Flare Photometry

A quantitative, objective technique that measures light scattered by proteins and cells in the anterior chamber.

Snowbanking

A plaque of inflammatory exudate over the pars plana, pathognomonic for pars planitis.

Toxoplasmosis

The most common cause of infectious posterior uveitis, presenting as "headlight in the fog" focal necrotizing retinochoroiditis.

Vogt-Koyanagi-Harada (VKH) Disease

A multisystem autoimmune disorder targeting melanocytes, causing bilateral granulomatous panuveitis and serous retinal detachments.

Acute Retinal Necrosis (ARN)

A devastating necrotizing retinitis caused by herpesviruses (VZV, HSV) featuring peripheral retinitis and occlusive vasculitis.

Standard Medical Therapy for Uveitis

A stepladder approach using corticosteroids first, followed by immunosuppressants (methotrexate) and then biologics (adalimumab).

Rhegmatogenous Retinal Detachment (RRD)

Occurs when a retinal tear allows liquefied vitreous to enter the subretinal space, often following a PVD.

Proliferative Vitreoretinopathy (PVR)

The formation of contractile membranes on retinal surfaces; the most common cause of retinal detachment surgery failure.

Pneumatic Retinopexy

An office-based procedure for RRD involving cryopexy or laser followed by an intravitreal gas bubble injection.

ILM Peeling

Surgical removal of the internal limiting membrane to increase retinal compliance and improve closure rates in macular hole surgery.

Epiretinal Membrane (ERM)

A sheet of fibrocellular tissue on the inner retinal surface that causes visually significant metamorphopsia.

Dropped Nucleus

A cataract surgery complication where retained crystalline lens matter falls into the vitreous cavity, requiring PPV.

Post-Operative Endophthalmitis

An ophthalmic emergency managed with an immediate vitreous tap/antibiotic injection or vitrectomy if vision is LP or worse.

Fundus Autofluorescence (FAF)

A non-invasive imaging technique that uses blue light to excite lipofuscin in the RPE to assess metabolic health.

Indocyanine Green Angiography (ICGA)

The "gold standard" for visualizing the choroidal circulation, specifically useful for diagnosing PCV and CSC.

DRIL

Disorganization of Retinal Inner Layers; an OCT sign of ischemia and a poor prognostic indicator in diabetic retinopathy and RVO.

Outer Retinal Tubulations (ORT)

Circular structures in the outer nuclear layer representing degenerating photoreceptors in end-stage retinal disease.

Ultra-Widefield (UWF) Imaging

An imaging system capturing the fundus out to $200$ degrees in a single shot to assess peripheral pathology.

IL-10 to IL-6 Ratio

A diagnostic marker in vitreous fluid where a ratio $> 1$ is highly suggestive of vitreoretinal lymphoma.

Voretigene Neparvovec (Luxturna)

The first FDA-approved gene replacement therapy delivered via subretinal injection to treat RPE65-associated retinal dystrophy.

Electronegative ERG

A pattern showing a normal a-wave with a severely reduced b-wave ($b:a \text{ ratio } < 1$), seen in X-linked retinoschisis.

RPE65

An enzyme critical to the visual cycle; mutations cause a profound lack of lipofuscin and a very low background FAF signal.

Stickler Syndrome

A connective tissue disorder featuring high myopia, "optically empty" vitreous, and high risk of giant retinal tears.

Mutton-fat Keratic Precipitates

Large, greasy-appearing inflammatory cell clusters on the corneal endothelium signaling granulomatous uveitis.

Arden Ratio

A value derived from the electrooculogram (EOG); a ratio $< 1.5$ indicates bestrophinopathy even with a normal fundus.

PAMM

Paracentral Acute Middle Maculopathy; an OCT band in the inner nuclear layer representing deep retinal capillary ischemia.