Ocular Disease:posterior Flashcards
Hruby Lens
◦ Indications: nonctonact examination of the optic disc, macula, posterior pole, and central vitreous
◦ Interpretation: provides a stereoscopic, erect, and magnified image
Three mirror lens indication
examination of the retina extending from the optic disc to the ora seratta. Performed in patients with peripheral retinal concerns such as peripheral vascular disease, history of blunt trauma, and those at risk or with symptoms of a retinal detachment
3 mirror lens interpretation
provides a stereoscopic, reversed, and magnified image of the retina 180 degrees away from he position of the mirror.
Trapezoid mirror on 3 mirror
73 degrees
Evaluate equator
Square mirror on three mirror
67 degrees
Used to eval the area between the anterior equator and the ora
Bullet mirror on 3 mirror
59 degrees
Anteiror chamber angle and the ora
Are 3 mirror views displaced laterally?
No
Indications of 78/90D lens
routine posterior segment evaluations. Easier to use than the Hruby lens and the three mirror. Image magnification and FOV are directly proportional to the pupil diameter and the dioptric power of the lens
Image in 78/90D
real, inverted, and reversed magnified image
Indications of BIO
routine comprehensive evaluation and similar indications as three mirror evaluation
Image in BIO
provides a real image that is magnified, reversed L-R, inverted top to bottom, and located between the examiner and the condensing lens
Red free filter
‣ The green filter (red free) allows easier differentiated of the nerve fiber layer, choroidal lesions, and retinal vasculature. A red free filter will cause a choroidal nevus to become more difficult to visualize or disappear
Scleral depression
◦ Indications: similar indications as a three mirror evaluation. Scleral depression allows oblique viewing of retinal tissue, which increases contrast and allows easier identification of abnormalities
◦ Scleral depression should NOT be performed on patients with recent intraocular surgery or patients with penetrating ocular injury, hyphema, or ruptured globe
Asteroid hyalosis
- epidemiology/Hx: associated with aging; occurs in 0.5% of the population over 60 years of age
- Symptoms: asymptomatic-does not interfere with vision or cause floaters
- Signs: numerous small, yellow-white, refractile particles (calcium phosphate soaps) attached to collagen fibrils in an essentially normal vitreous; unilateral in 75% of the cases
Synchysis scintillans
- pathophysiology/Dx: rare conditio nthat occurs after chronic uveitits, vitreous hemorrhage, and/or trauma
- Signs: unilateral, golden-brown, refractile cholesterol crystals that are freely mobile in the vitreous cavity (often settle inferiorly)
Who gets PVDs
more common in females. Prevalence appx age after 50 years old (50% in 50 year old, 60% in 60 year old, etc)
◦ PVDs occur an average of 20 years earlier in myopes than in emmetropes
◦ Other risk factors include diabetes, intraocular surgery, intraocular inflammation, vitreous hemorrhage, and trauma
Pathophysiology of PVD
the HA-collagen complex in the vitreous is disrupted with age, causing the collagen to clump up in bundles. Liberated collagen can contract within the complex, causing the posterior hyaloid to detach from the retina. Pockets of liquefaction (syneresis) can travel through the hole in the posterior hyaloid and cause separation between the vitreous and the retina. The PVD can be localized, partial, or total
Symptoms of PVD
acute onset floaters, flashes of light, and decreased vision
Photopsia in eyes with acute PVDs
Thought to result from traction at the site of the vitreoretinal adhesions
Signs of PVD
Weiss ring (black or grey ring shaped vitreous opacity over the optic nerve) and anteiror displacement of the posterior hyaloid; may also see vitreous pigment cells (tobacco dust/Shaffer sign) and a vitreous hemorrhage
Vitreous traction during a PVD
can result in ERM, macular holes, vVMT, vitreous and retinal hemorrhages, and retinal breaks. 10-15% of patients with an acute symptomatic PVD will have a retinal break; this risk increases 70% if a vitreous hemorrhage is present. Retinal pigment epithelium can be released into the vitreous (Shaffer’s sign) after a retinal tear, which can aid in the diagnosis
Epidemiology of preretinal/vitreous hemorrhage
ask about trauma and pertinent ocular and systemic diseases, especially DM and HTN
Pathophysiology of preretinal/vitreous hemorrhage
preretinal and victory’s hemorrhages result from trauma or from conditions that cause retinal neo. These include the following
◦ diabetic retinopathy, retinal vein occlusion, sickle cell retinopathy, ROP, and ocular ischemic syndrome
◦ In each of these cases, the neo is preretinal in location and the newly formed vessels lack endothelial tight junctions. The location (preretinal) and strength (leaky) of these vessels created a situation where vitreous traction can cause shearing of the vessels, resulting in hemorrhage formation
Symptoms of preretinal hemorrhage
usually does not cause symptoms unless it involves part of the macula (results in sudden loss of vision or part of the visual field)
Symptoms of vitreous hemorrhage
usually causes painless vision loss and/or black spots that can have corresponding flashing lights
Signs of preretinal hemorrhage
located between the retina and an intact posterior vitreous face; appears very red and often has a keel shape
Signs of a vitreous hemorrhage
located within the vitreous (anterior to the posterior vitreous face). A mild vitreous hemorrhage will be characterized by blood that obscures only part of the fundus. Severe hemorrhages will completely obscure the view of the fundus. Chronic cases will appear yellow
‣ A B scan is indicated if the fundus cannot be viewed throught the vitreous hemorrhage
Types of neo
Preretinal and chorodial
Preretinal neo
leads to preretinal or vitreous hemorrhage and/or tractional retinal detachement. Remember DR VOS for preretinal neo
Chorodial neo
within the subretinal space (CNVM) that results in a subretinal and/or subREP heme, pigment epithelial detachment, and serous retinal detachment. Remember CHBALA for choroidal neo
Epidemiology/Hx of CRVO
prevalence 0.1-0.4%. CRVOs are the 3rd most common vascular cause of vision loss (DR is the second most common). An estimated 7% of patients will have a CRVO in the fellow eye
Risk factors for CRVO
HTN, DM, cardiovascular disease, and open angle glaucoma
Glaucoma and CRVO
◦ Glaucoma is the ocular disease that is most commonly assocaited with CRVOs; up to 40-60% of patients with CRVO have POAG
CRVO in young patients
oral contraceptive pills, protein S/C/antithrombin III deficiency, factor XII deficiency, antiphospholipid Ab syndrome, collagen vascular disease, and AIDs
Pathophysiology/Dx of CRVO
result from compression of an artery on a vein; this leads to turbulent blood flow, venous vessel wall damage, and thrombus formation. CRVOs are usually caused by a thrombus at or near the lamina cribrosa
Symptoms of CRVO
characterized by a sudden, unilateral, painless visions loss in an elderly patient. (90% of patients with a CRVO are > 50 years old)
Signs of CRVO
thrombus formation leads to ischemia and release of VEGF which characteristic retinal findings including retinal hemorrhages in all 4 quadrants, collaterals, dilated torturous retinal veins, CWS, and optic disc edema
Collateral veins in CRVO
‣ Collateral veins become visible over several weeks to months; they are often on the disc and permit blood flow between the retina and chorodial circulations, helping to accelerate drainage of excessive fluid (Retinal edema) into the choroidal circulation after a CRVO
Vision threatening complications of CRVO
◦ Vision threatening complications include macular disease and complication from neo. VEGF stimulates neo of the posterior and anterior segment and has been prove to cause capillary leakage leading to macular edema
‣ 1. Macular disease-macular ischemia, macular edema, and intramacular hemes
‣ 2. Neo-neovascular glaucoma, preretinal/vitreous hemorrhage, and tractional RD
Neovascular glaucoma and CRVO
◦ Neovascular glaucoma is a major concern in patients with a CRVO and is most likely to develop within the 1st 3 months of Dx (90 day glaucoma). 60% of ischemic cases develop iris neo and up to 33% develop neo glaucoma. 6% of non ischemic cases develop rubeosis or angle neo
Leading cause of vision loss in both ischemic and non ischemic CRVO
Macular edema
Ischemic vs nonischemic CRVOs
‣ Ischemic CRVO is defined as 10 disc diameters or more of non-perfusion on FA. 90% of cases present with 20/200 vision or worse, and the prognosis is poor. 16% of nonischemic cases progress to ischemic CRVOs
Epidemiology of BRVO
BRVOs are by far the most common retinal vascular occlusive disease; patients have a similar health history as patients with CRVOs
Most common vascular occlusive disease
BRVO
Risk factors for BRVO
HTN, cardiovascular disease, increased body mass index at 20 years old, and open angle glaucoma
Pathophysiolgoy/Dx
BRVOs are usually caused by a thrombus after compression of an artery on a vein at an AV crossing. 60% occur at an AV crossing within the superior/temporal quadrant
BRVOs that do not occur at AV crossings
• BRVOs that do not occur at an AV crossing should be evaluated with vasculitis. Remember that retinal arteries and veins share a common adventitia at AV crossings; this allows a thickened artery to compress the vein
Symptoms of BRVO
BRVOs are characterized by sudden, unilateral, painless visual field loss, blurred vision or no symptoms (if the macula is spared)
Signs of BRVO
retinal signs occur in the area of the distribution of the occluded vessel and include dilated torturous retinal veins, CWS, collateral vessels, and intraretinal hemorrhages
Vision threatening complications of BRVO
◦ Vision threatening complications include macular disease and complications from neo
‣ 1. Macular disease-macular ischemia, macular edema, and intramacular hemorrhage
‣ 2. Neo complications-preretinal/vitreous hemorrhage
Epidemiology/Hx of CRAO
ask about transient loss of vision (amaurosis Fugax). Commonly occurs in elderly patients. Patietns have a 10% risk of CRAO occurring in the fellow eye
Risk factors for CRAO
HTN, DM, carotid occlusive disease, cardiac valve disease
CRAO in young patients
IV drug usage and BC
Pathophysiology/Dx of CRAO
CRAOs most commonly arise from heart and/or carotid artery emboli
‣ Calcific emboli-large dangerous emboli from calcified heart valves often located in the CRA near the optic nerve
‣ Carotid Emboli-smaller cholesterol plaques (hollenhorst plaques)
Worst kind of emboli
Calcific
Usually from heart valves
Things that can cause CRAO
◦ Although retinal emboli are by far the most common etiolgoy for arterial occlusions, there are several other culprits to consider, including GCA, acute elevation in IOP, collagen vascular diseases, IV drug use, oral BC, sickle cell disease, and syphilis
Symptoms of CRAO
acute, profound vision loss (often 20/400 or worse). Unless a cilioretinal artery is present to spare the macula. If VA is LP or worse, strongly consider an ophthalmic artery occlusion
Cilioretinal artery in CRAO
‣ A cilioretinal artery branches form the SPCAs of the choroid and allows the macula to remain functional in a CRAO. It is present in 15-30% of patients
Signs of CRAO
superficial whitening of the inner retinal layers (returns to the normal color after perfusion is restored but does not regain function!), narrowed arterial vasculature, a cherry red spot in the foveola, and an afferent pupillary defect r(secondary to optic disc pallor from orthograde degeneration of RGC axons). Hollenhurst plaques or other emboli are also noted in 20-40% of cases
Neo glaucoma and CRAO
Rare
Epidemiology/Hx of BRAO
similar risk factors to CRAOs
Pathophysiology/Dx of BRAO
90% of cases are caused bu retinal emboli (hollenhurst plaques (most common), calcium, fibrin, and platelet emboli)
Symptoms of BRAO
often asymptomatic; may complain of a VF defect or sudden unilateral painless vision loss if the area of occlusion is close to or involving the macula
Signs of BRAO
superficial whitening of the retina in the distribution of the affected vessels due to retinal infarction and edema; hollenhurst plaques or other emboli are found within the area of occlusion in 62% of cases. The edem and retinal whitening eventually resolves within weeks, but the retinal tissue remains non functional and the patient with have permanent VF defect
Myelinated nerve fibers
are congential, benign, white patches in the superficial retina with feathery edges that represent abnormally myelination of ganglion cell axons anterior the lamina cribrosa. Myelination follows the distribution of the axons, can obscure the retinal vessels, and is typically located near the optic nerve. Differential diagnosis include BRAO and CWS
Indications for a diabetic retinal exam for type I DM
Within 3-5 years of diagnosis
Indications for a diabetic retinal exam for type II DM
At the time of diagnosis
For patietns with no diabetic retinopathy, repeat exams every year
For patients with mild to moderate retinopathy, repeat exams every 6-12 months
For patietns with severe NODR or PDR, repeat examinations every 2-4 months
Leading cause of new cases of blindness in the US for adults 20-74
diabetic retinopathy
Most important risk factors for development of DR
Duration of disease
‣ Patients who are dx with DM before age 30 have a 2% risk per year for developing DR; after 7 years-50%, after 25 years-90% with have DR
Pathophysiology/Dx of DR
DR occurs because of a loss of pericytes and damage to the retinal capillary BM, resulting in a breakdown of the blood retinal barrier. DR can be divided into non proliferative DR (NPDR or background DR) and proliferative DR (PDR)
NPDR
Can be divided into mild, moderate, severe, and very severe
Mild NPDR risk of progression
5% in 1 year
Moderate NPDR risk of progression to PDR
15% in 1 year
Severe NPRD risk of progression to PDR
52% risk in 1 year
Risk of progression for very severe NPRD
75% in 1 year
When is the diagnosis of very severe NPDR made
When two or more criteria are met within the 4-2-1 rule
- Severe retinal hemorrhages in 4 quadrants
- Venous beading in 2 quadrants
- IRMA in 1 quadrant
The diagnosis of NPDR is made when
The patient meets one criteria from the 4-2-1 rule
- Severe retinal hemorrhages in 4 quadrants
- Venous beading in 2 quadrants
- IRMA in 1 quadrant
4-2-1 rule
- Severe retinal hemorrhages in 4 quadrants
- Venous beading in 2 quadrants
- IRMA in 1 quadrant
PDR
occurs in 5% of patients with DR and is diagnosed based on the presence of neovascularization. If left untreated, PDR can progress to devastating outcomes. Patients with PDR who are most at risk for visions loss have high risk characteristics (HRCs)
High risk characteristics of PDR
- Neovascularization of the disc (NVD) greater than 1/4 DD within 1DD of the optic nerve
- Any NVD or NVE with an associated vitreous or preretinal hemorrhage
Symptoms of PDR
patietns are often asymptomatic or May experience vision and metamorphopsia
Signs of PDR
although there are numerous signs of DR, the most important threats to vision include macular disease and neovascularization
Macular disease in PDR
Macular ischemia
Macular edema
Macualr ischemia in PDR
may look normal or thickened; a FA can be used to differentiate macular ischemia from macular edema (macular ischemia will appear as an enlarged fovea avascular zone (hypofluoresce))
Macular edema in PDR
it can occur at ant stage of DR. CSME is based not he following 3 criteria (based on the presence of retinal thickening within the fovea)
• A. Retinal thickening within 500um (1/3DD) of the fovea center
• B. Hard exudates within 500um of the fovea center with adjacent retinal thickening
• C. Retinal thickening of at least 1DD within 1DD of the fovea center
The patient only needs to have one of the three to be diagnosed with CSME
CSME in PDR
- A. Retinal thickening within 500um (1/3DD) of the fovea center
- B. Hard exudates within 500um of the fovea center with adjacent retinal thickening
- C. Retinal thickening of at least 1DD within 1DD of the fovea center
The patient only needs to have one of the three to be diagnosed with CSME
Neovascularization in PDR
Threats to vision from neo include the following
‣ 1. Preretinal/vitreous hemorrhages
‣ 2. Neovascular glaucoma
‣ 3. Tractional RD
Epidemiology/Hx of HTN retinopathy
60 million Americans over the age of 18 years of age have HTN; more prevalent in AA. Essential HTN accounts for 95% of all cases of HTN and is defined as elevated blood pressure with no known cause
Pathophysiology/dx of HTN retinopathy
recall that retinal arteries are able to auto regulate their vessel diameter based on changes in blood pressure; autoregulation is altered at extremely high or chonrically elevated systolic pressures and retinopathy results. Systemic blood pressure must typically be at least 140/110 for the latter stages of HR to occur
Symptoms of HTN retinopathy
commonly asymptomatic. Vision is typically unaffected unless vascular changes cause macular edema (macular star), papilledema, a serous retinal detachement, or a vein occlusion
Stages of HTN retinopathy
1-4, 4 being the worst
Stage 1 HTN retinopathy
mild to moderate diffuse narrowing of the retinal arteries (but no focal constriction)
Stage 2 HTN retinopathy
stage 1 plus focal constriction of the retinal vasculature (AV nicking) and exaggerating of the arterial light reflex
Stage 3 HTN retinopathy
stage 2 plus retinal hemorrhages, CWS, hard exudates (likely in a star configuration within the OPL radiating away from the fovea), and retinal edema
Stage 4 HTN retinopathy
stage 3 plus papilledema (malignant HTN). Patients with malignant HTN must be hospitalized immediately due to high risk of stroke. BP at this stages is usually 220/120.
HTN retinopathy is associated with numerous secondary conditions that can lead to vision loss, including
vascular occlusion, retinal macroaneurysms, NAION, ocular motor palsies, and worsening of DM.
Elschnig spots
choroidal infarcts that occur in severe HR
Epidemiology/Hx of retinal macroaneurysms
more common in elderly women (7th decade) with HTN or atherosclerosis
Symptoms of retinal artery macroaneurysms
usually asymptomatic, but can have gradual vision loss from macular edema or sudden vision loss from a vitreous hemorrhage
Signs of retinal artery macroaneurysms
unilateral focal area of dilation in a retinal artery (100-250um in diameter) with multi-level hemorrhages (subretinal, intraretinal, preretinal, and/or vitreous hemorrhage) from a ruptured aneurysm with surrounding circulate exudates; often located at an AV crossing
Description of the hemorrhages in retinal artery macroaneurysms
multi-level hemorrhages (subretinal, intraretinal, preretinal, and/or vitreous hemorrhage)
Epidemiology/Hx of venous stasis retinopathy?OIS
Men
Ages 50-80
Pathophysiology/Dx of venous stasis retinopathy/OIS
caused by occlusion of the ICA and/or ophthalmic artery (less common), usually secondary to atherosclerosis; may also occur as a result of GCA
Symptoms of venous stasis retinopathy/OIS
common symptoms include gradual vision loss, dull periorbital pain or HA, and amaurosis fugax
Signs of venous stasis retinopathy/OIS
unilateral (80%) dot/blot hemorrhages of the midperipheral fundus, dilated non-tortuous retinal veins, narrowed retinal arteries, and possible neo of the disc and/or anterior segment (67% of patients with OIS have NVI/NVA at the time of diagnosis)
Difference between OIS and venous stasis retinopathy
‣ If a patient has these retinal findings and carotid artery obstruction, but no anterior segment signs, the condition is called venous stasis retinopathy
‣ The presence of both posterior and anterior segment signs and symptoms is referred to as OIS. OIS is most common in male patients 50-70 years old
OIS is commonly associated with
systemic HTN, DM, and cardiac disease; the latter is most common cause of a 40% 5 year mortality rate in these patients
Amaurosis fugax
type of TIA characterized by transient monocular vision loss that typically lasts seconds to minutes; vision returns to normal after the ischemic event. A carotid artery embolus is the most common cause of amaurosis fugax
TIA vs stroke
characterized by temporal neurological defects due to transient loss of blood flow to the brain. Perfusion is always resorted in less than 24 hours (usually less than 15 minutes), resulting in complete resolution of the patient’s symptoms without any permanent damage. In contrast, a stroke is characterized by permanent neurological deficits due to prolonged loss of blood flows that results in irreversible damage to the brain
Things that cause pre retinal neo
Proliferative Neo DRVOS -DM -ROP -Vein occlusions -OIS -Sickle cell
What are the main problems with preretinal neo
Proliferative, DRVOS
- Preretinal/vit heme
- Tractional RD (vit pulls on neo)
- VEGF=NVG
Number one cause of all occlusions
- HTN
- Plaques from the heart
A clot that stays where it formed
Thrombus
Which causes vein occlusions, thrombus or embolus
Thrombus
Which causes artery occlusions, thrombus or embolus
Embolus
Why do we get thrombus
- HTN/DM (artery prob)
- Artery compressing vein
- Bad/turbulent blood flow
- Thrombus formed
- VEGF released
Which is worse, ischemic or non ischemic CRVOs
Ischemic
What layers of the retina are damage in CRAO
OPL-> ILM
VF loss in BRAO
Respects horizontal midline, Mimics glaucoma
5 most important things in DM
- Preretinal/vit heme
- Tract RD
- VEGF-NVG
- Ischemic macula
- Macular edema
Damage to pericytes in DM
- autoregulation problem
- ONH
- retinal vessels
Break down of BRB
- between RPE cells
- retinal BV
Swelling in ischemia
Ischemia is decreased O2, it initally swells and then thins
Primary HTN ret findings
Mac star (3 or 4) Papilledems (4)
Findings secondary to HTN (other than HTN retinopathy)
CRAO, BRAO, CRVO, BRVO NAION EOM palsy Worsening DM Macroaneurysms
Things that cause NAION
Viagra
Imitrix
Amiodarone
SPCA not doing its job
What should be ordered for someone with OIS
Carotid Doppler
Pathophysiology/Dx of hyperviscosity retinopathy
an increase in resistance to blood flow secondary to elevated levels of proteins, RBCs, and/or WBCs, resulting in impaired circulation of blood flow and oxygen though the microvasculature. As blood flow decreases, blood vessel walls become damaged, causing leakage of fluid and retinal ischemia
The most common cause hyperviscocity retinopathy
hyperglobulinemia, a condition found in Waldenstrom’s macroglobulinemia, multiple myeloma, serum positive RA, SLE, and HIV infection
Signs of hyperviscocity retinopathy
retinal venous dilation, retinal hemorrhages, CWS, and exudates. Central retinal vein occlusion may also occur and is bilateral in 10% of patients
HIV retinopathy
◦ The most common ocular manifestation of HIV/AIDS. Signs include CWS and retinal hemorrhages, similar appearance to DR and early CMV retinitis
◦ Patients are typically asymptomatic. The condition is non infectious in origin
Interferon retinopathy
◦ Patients on interferon therapy can develop retinal findings similar to DR, including CWS and retinal hemorrhages within the posterior pole.
◦ Retinopathy typically occurs within 3-5 months after interferon is started, and tends to resolve without treatment after interferon has been discontinued
How often should patients on interferon retinopathy be followed
◦ Patients on interferon therapy without retinopathy should be followed every 4-6 months; if retinopathy is present, follow ups should occur more frequently
Talc retinopathy
◦ Presents bilaterally in IV drug users who use talc as a filler
◦ The talc gets caught in the retinal capillaries and will appear as multiple, yellow, refractile, deposits that tends to be clustered near the macula
◦ The talc may cause capillary occlusion and retinal ischemia
Periphlebitis
Sarcoidosis
-lac gland, CN VII, OHN, vessels, vitreous
AA female
Hard exudates that leak
Vasculitis=inflammation
An inflammatory condition characterized by exudates around the vessels (seen as white cuffing of the vessels). Retinal edema, ischemia, and hemorrhaging may also occur. Vessel walls will stain on FA
Vascualr sheathing/periphlebitis
Most common causes of vascular sheathing/periphlebitis
syphilis, sarcoidosis (known as candle wax drippings), pars planitis, and sickle cell disease
Testing for vascular sheathing/periphlebitis
◦ Diagnostic systemic lab testing should be done based on the revive of systems and case history to determine the systemic cause of the vascular sheathing
Are bilateral retinoblastoma hereditary or non hereditary
All bilateral=hereditary
Are unilateral RBs hereditary or non
Most of them are nonhereditary
Pathophysiology/Dx of idiopathic juxtafoveolar telangiectasia
abnormal perifovea capillaries present within the juxtafoveal region. IJXT is divided into three categories
Unilateral congenital idiopathic juxtafoveolar retinal telangiectasia
occurs in men in the 4th decade; results in 20/25-20/40 vision
Unilateral idiopathic form of idiopathic juxtafoveolar retinal telangiectasia
occurs in middle aged men; reuslts in 20/25 or better acuity
Bilateral acquired form of idiopathic juxtafoveolar retinal telangiectasia
equal sex distribution in the 5th or 6th decade. Poor visual prognosis
Symptoms of idiopathic juxtafoveolar retinal telangiectasia
Decreased vision
Signs of idiopathic juxtafoveolar retinal telangiectasia
right angle venules and dilated tortuous vessels, hemes, varying degrees of exudates (moderate to none at all) within or nearby the fovea, macular edema, and/or CNVM
Epidemiology/Hx of Coat’s disease
peak incidence in males less than 20 years old; 2/3 of cases present prior to age 10. Progression is more rapid in children under 4 years old, simulating retinoblastoma
Pathophysiology of Coat’s disease
idiopathic peripheral vascular disease. If left untreated, coats disease will gradually progress to a total exudative RD
Symptoms of Coats
decreased vision, strab, leukocoria
Signs of Coats
unilateral, telangiectatic dilated vessels that display a characteristic “lightbulb” appearance. Progression of the disease can lead to marked hard exudates (classic for coats), intraretinal hemorrhagehs, exudative retinal detachement, and NVG (results in red painful eye and potentially blind eye)
How does Coats lead to NVG
A chronic extensive serous detachment in coats disease results in retinal ischemia, which leads to the development of anterior segment neovascularization similar in pathology to the DR VOS conditions. NVA and NVI result in NVG
Epidemiology/Hx of ROP
occurs in premature infants (less than 32 weeks) or low birth weight infants (<1500g) who have received oxygen therapy
Pathophysiology of ROP
immature blood vessels vasoconstriction and stop developing in response to high oxygen concentration, leading to proliferative retinopathy. Threatens to vision include preretinal/vitreous hemorrhages, and tractional RDs
Signs of ROP
leukocoria, strab, vitreoretinal traction, preretinal/vitreous hemorrhages, and tractional RDs
Leukocoria and ROP
white pupillary reflex that resutls from fibrovascular scarring secondary to a tractional RD in cases of ROP
Which part of the retina matures last?
The anterior temporal retina is the last area of the retina to achieve mature vascular development during the 9th month of gestation. This area is most susceptible to neovascularization and subsequent tractional RDs in pre term infants with ROP
Epidemiology/Hx of RB
the most common intraocular malignancy in kids and the second most common of all age groups (choroidal melanoma is the most common). 95% of cases are diagnosed by 5 years of age. No gender or race predilection
The smog common intraocular malignancy in kids
RB
The second most common intraocular malignancy in all age groups
choroidal melanoma
Pathophysiology/ Dx of RB
tumor derived from cells in the developing retina (retinoblasts) as a result of mutations in the Rb tumor suppressor gene
Heritable RB
40%
all bilateral cases and about 10-20% of unilateral cases. However, only 6% of these have a positive family Hx of the condition because the tumor has such a high spontaneous mutation rate. Bilateral affected patients have a 50% chance of passing the disease on to each offspring
Non-heritable RB
60%
85% of all unilateral cases of RB are non heritable. Affected pateitns will not pass the disease on to their offspring
Signs and symptoms of RB
leukocoria, strab, intraocular inflammation, and decreased vision
Differentials for leukocoria
Coat’s disease, toxocariasis, RB, and ROP
Epidemiology/Hx for CHRPE
the lesions are thought to be congenital with no race or sex predilection
Signs of CHRPE
benign, pigmented (brown to black), non-progressive lesions with sharp borders and central hypopigmetned lacunae.
‣ CHRPEs are typically unilateral and solitary, measuring 1-6mm in diameter. Bilateral multifocal (4 or more) CHRPEs are assocaited with Gardner’s Syndrome-colonoscopy needed
Epidemiology/Hx for choroidal nevus
common condition that occurs in 1-6% of the population and is most commonly in whites
Pathophysiology/Dx of choroidal nevus
common benign focal accumulation of melanocytes within the choroid. Choroidal nevi are thought to be present at birth and are typically non progressive
Growth of a nevus
‣ Growth during puberty is not unusual, but any growth in an adult should raise concern for conversion to malignant melanoma
‣ 10% of suspicious nevi progress to malignant melanoma
The most common risk factors for transformation of a choroidal nevus into a chorodial melanoma are
To Find Small Ocular Melanomas, Use Helpful Hints”
• T=thickness (elevation >2mm)
• F=fluid (subretinal)
• S=symptoms (blurred vision and floaters)
• O=orange pigment (lipofuscin on surface of lesion)
• M=margins (irregular borders)
• UH=ultrasonographic hollowness (acoustically hollow with low internal reflectivity)
• H=halo absence
• The nevus should also around suspicion if it has a large diameter (>4DD or 6mm) or if its close proximity to the ONH
Signs of choroidal nevus
the lesion is typically flat or slightly elevated, less than 5mm in size, and often contain overlying drusen
Most common primary intraocular malignancy
Choroidal melanoma
Things that cause subretinal neo
CHBALA
- Choroidal rupture
- Histo
- Bests
- Angioid streaks
- Lacquer cracks
- ARMD
Problems with Burch’s
Two types of CNVM
Plasma
- clear
- dont treat
- small break
- detachment (PED)
RBCs
- red
- treat
- large break
- hemorrhage
- looks grey/green
Wet vs dry ARMD: PED
Wet PED
=break in bruchs
Dry PED
-drusenoid PED, no break in bruchs
Where is drusen located
Inner collagenous zone of bruchs
Main concerns in dry ARMD
- Drusenoid PED
- GA
- Progression to wet
Macular degeneration is a progressive disease of the
RPE
Bruchs membrane
Choriocapillaris
Who gets AMD
most common in patients over the age of 50. It is the second leading cause of blindness for patients 45-64 (DM number 1); exudative ARMD is the chief cause of vision loss in patients over the age of 50
Risk factors fro ARMD
increasing age (esp >75), ethnicity (whites), positive family history, light iris color, cigarette smoking, hyperopia, HTN, HLD, female gender, and cardiovascular disease
Nutritional factors and light toxicity play roles
Hyperopia and ARMD
Hyperopia greater than 0.75D is assocaited with a 2.5X increased risk of exudative ARMD. 10-20% of patients with ARMD have at least one first degree family member with vision loss from ARMD.
Smoking and ARMD
Current smokers are 2.5x more likely to develop ARMD and 1.7-2.2x more likely to have recurrence of CNV compared to those who have never smoked
What is the most common form of ARMD
No exudative (Dry)
Characteristics of dry ARMD
presence of drusen (hallmark); assocaited RPE abnormalities (mottling, granularity, focal hyperpigmentation) may also be present
Symptoms of dry ARMD
Most patients with dry ARMD do not have severe vision loss; metamorphopsia, gradual vision loss (over months to years), and blurred visions are common complaints
4 main risk factors that increase the progression from dry to wet ARMD
- Multiple soft drusen (especially if confluent)
- Focal hyperpigmentation
- HTN
- Smoking
Most of legal blindness is attributed to
Wet ARMD
Symptoms of exudative ARMD
metamorphopsia, central scotoma, and rapid vision loss
Signs of exudative ARMD
drusen that are associated with signs of a CNVM. CNVMs can leak blood or plasma into two potential places: 1. Sub-RPE space or 2. Subretinal space.
What are the potential presentations of wet ARMD
CNVMs can either leak blood or plasma into two potential spaces; 1) subRPE, 2) subretinal space. This creates 4 potential presentations of wet ARMD
- Subretinal hemorrhage (blood under retina)
- SubRPE hemorrhage (blood under RPE
- Subretinal detachment (plasma under the retina, AKA serous retinal detachment)
- SubRPE detachment (plasma under the RPE, AKA pigmented epithelial detachement (PED))
Another name for a subretinal detachment
Serous retinal detachement
Another name for a subRPE detachment
Pigmented epithelial detachment (PED)
PEDs in dry ARMD
can also occur with dry AMD due to a build up a confluent soft drusen on Bruch’s membrane, creating a space between the RPE and the choroid. This type of sub-RPE detachment is called a drusenoid pigment epithelial detachement. CNVMs can develop over time within drusenoid PEDs
Classic vs occult CNVM
FA can be used to detail exact ares of leakage and classify them as occult or classic
• Classic CNVMs are characterized by a well defined membrane that fills will dye during the early stages of FA
• Occult CNVMs are characterized by a poorly-defined membrane with late appearing and less intense leakage
• Most patients with wet ARMD have a CNVM with a combination of classic and occult features. The term “predominantly classic” means that over 50% of the entire lesion is composed of a classic CNVM
Incidence of involvement of the fellow eye in wet ARMD
incidence of involvement of the fellow eye with wet ARMD is estimated to be 28-36% during the first 2 years; the annual rate of bilaterality is about 6-12% per year for the next 5 years. The overall 5 year risk ranges from 40-85%
Epidemiology/Hx of CSCR
commonly occurs in young to middle aged men (20-50) with a type A personality; associated with stress, pregnancy, steroid use, hypochondriasis, and HTN. A history of similar episodes is common-recurrences occurs in as high as 50% of cases
Pathophysiology of CSCR
unknown etiology. CSR results in RPE and/or choroidal dysfunctions, with resulting accumulation of submacular serous fluid
Symptoms of CSCR
unilateral sudden onset of blurred visions (20/20-2/200); metamorphopsia and a relative scotoma may also occur. Patients may have symptoms even if acuity is 20/20
Signs of CSCR
localized macular serous detachment; 3% of cases will have an RPE detachment as well. FA will reveal a gradual pooling of Fl into a pigmented epithelial detachment or a “smokestack appearance”. OCT can also be used to dx and monitor the condition (now used more frequently than FA). Patietns may also have a hyperopic shift and loss of the foveal light reflex
Permanent changes in CSCR
Patients often have permanent residual RPE changes within the macula after resolution of the condition.
In CSCR, most people improve without treatment in
1-3months
Prognosis of CSCR
Most patients improve without treatemtn by 1-3 months; 94% of patients will regain >20/30 acuity. 66% of patients achieve 20/20 acuity
Vitritis in histoplasmosis
There is NONE!
This is a choroiditis, not a retinitis
Worst case scenario for histoplasmosis
Break in bruchs membrane
-can get the 4 different types of nets
Triad of histoplasmosis
Bilateral choroiditis with
- Peripapillary atrophy
- Peripheral histo spots
- Maculopathy
Difference between histo and toxo
Toxo
- parasite
- retinitis=breakdown in BRB=vitritis
- one eye
- one lesion
Histo
- fungus
- choroiditis
- BRB fine, no vitritis
- both eyes
- multiple lesions
Difference between lacquer cracks and angioid streaks
Lacquer cracks
- young males
- yellow
- coming from macula
Angioid streaks
- always from the disk
- PXE, ED
- PEPSI
- dont want streak across macula
- red in appearance
Glial cells and ERM
Glial cells on the ILM
- From vitreous
- From within the retina
Epidemiology/Hx of histoplasmosis
rare in AA, most comm in the Ohio-Mississippi-river valley.
Pathophysiology/Dx of histoplasmosis
infection caused by histoplasmosis capsulatum, a fungus that grows in soil and material contaiminted with bird or bat droppings.
‣ Recurrences of histo (30% of cases) occur through preexistent histo spots
‣ If spots are present in the disc or macular area, the chance of a symptomatic recurrence is appx 20% over 3 years
Symptoms of histoplasmosis
asymptomatic unless maculopathy develops; the earliest symptom is metamorphopsia
Signs of histoplasmosis
characterized by bilateral choroiditis and the clinical triad of bilateral peripapillary atrophy of the optic nerve, multifocal leasions in the periphery, and maculopathy (including CNVM). The vitreous is always clear in histoplasmosis!
‣ CNVM is a late manifestation: if it occurs, it is most likely to happen between the ages of 20 and 45
Differences between histo and toxoplasmosis
Although toxoplasmosis and histoplasmosis are both types of posterior uveitis, they are characterized by very different etiologies and presentations
• Toxoplasmosis is caused by a parasite that results in retinitis, which is characterized by WBCs in the vitreous due to a breakdown in the blood retinal barrier. Remember ONE EYE, ONE LESION
• Histoplasmosis is caused by a fungus that leads to choroiditis; it does NOT cause a breakdown in the blood retinal barrier and does NOT present with a vitritis! Remember BOTH EYES and MULTIPLE LESIONS
Epidemiology/Hx of pathological myopia
pathological myopia has a genetic predisposition, with elongation of the globe beginning in early childhood. Occurs in 2% of the population, most commonly in women during young adulthood. The condition is also referred to as myopic degeneration
Pathophysiology/Dx of pathological myopia
defined as a refractive error >-6.00D spherical equivalent and/or axial length >26.5mm. Axial lengthening in the anterior-posterior direction results in scleral thinning and choroidal atrophy
What refractive error and axial length define pathological myopia
> -6.00D
>26.5mm
Symptoms of pathological myopia
patient may be asymptomatic or complain of decreased vision and metamorphopsia
Macular and posterior pole signs of pathological myopia
- Posterior staphyloma (the hallmark of pathological myopia): posterior bulging of the weakened sclera
- Oblique insertion of the optic nerve
- Fuch’s spots (focal subretinal hyperpigmentation secondary to scarring or CNVM
- Lacquer cracks
- Macular holes
Peripheral signs of pathological myopia
- Lattice degeneration
- Snail track degeneration
- Pavingstone degeneration
- Retinal breaks
- Retinal detachments
Non retinal signs of pathological myopia
- Premature cataracts (NS and esp PSC)
- Extensive vitreous syneresis
- PVD
Lacquer cracks in pathological myopia
occur in about 5% of high myopes. They appear as fine yellow irregular lines that represent large breaks in Bruch’s membrane. Choroidal neo can result and lead to severe vision loss. Lacquer cracks frequently present in young males and may be one of the earliest findings in pathological myopia
Pavingstone degeneration
discrete, circular areas of yellow-white chorioretinal atrophy in the retinal periphery. Also referred to as cobblestone degeneration. This condition is not a predisposing factor for a RD and has no clinical importance
Epidemiology/Hx of ERM
more common in females. Prevalence increases with age; occurs in 2% of patients older than 50, and 20% of patietns older than 75
‣ The condition is often idiopathic but may be caused by PVDs, retinal breaks, cataract or other intraocular surgeries, and trauma
Pathophysiology of ERM
ERMS result from glial cell proliferation on the ILM. Vitreous traction can result in residual glial cells from the posterior hyaloid (back of the viteous) on the ILM, or can cause small pores to develop in the ILM; this allows intraretinal glial cells to gain access to the anterior side of the ILM for proliferation
Symptoms of ERM
may be asymptomatic or complain of decreased vision and/or metamorphopsia
Signs of ERM
mild ERMS are characterized by fine, glistening membrane (cellophane maculopathy). Advanced ERMS appear as thick, gray-white membranes with associated retinal folds (macular pucker)
Most patients with ERM have
A PVD
Epidemiology/Hx of macular hole
83% of cases are associated with aging; women are more commonly affected
Pathophysiology/Dx of macular hole
results from posterior vitreous traction on the macula. Most commonly idiopathic (senile), but may also develop after trauma, surgery, CME, or inflammation. The condition has a bilateral onset in 25-30% of cases. The risk of developing a macular hole in the fellow eye varies from 5-16% (there is no risk of a PVD has occurred)
Symptoms of macula holes
decreased vision and/or metamorphopsia. Full thickness macular holes are assocaited with 20/200 or worse vision
Signs of macula hole
condition is characterized by a round, red, well delineated spot in the macula.
Stage 1 macula hole
impending hole with loss of the foveal depression and a yellow spot or ring at the fovea
Stage 2 macular hole
round, small, full-thickness hole with a pseudo-operculum
Stage 3 macular hole
large, full thickness hole with an operculum. Patients will report a positive Watzke-Allen sign
Stage 4 macula hole
stage 3 plus a PVD
Positive Watzke Allen test
characterized by a complete break in the middle of a thin line of light projected within the macula
Macular photostress test
determine the patients BCVA prior to starting the test. Howl a bright light 2cm from the patient’s eye and instruct the patient to look at the light for 10s. Measure the time it takes for the patient to read one line less than his/her BCVA. Norma recovery time is less than 60s.
Choroidal folds
Waves within the choroid, Bruchs membranes, and RPE that develop secondary to mechanical stress on or within the choroid. Characterized bu alternating light and dark striations within the fundus. Macular involvement may lead to symptoms of metamorphopsia and reduced VA
What causes choroidal folds
◦ Commonly the result of ocular or systemic conditions that cause mechanical stress on the choroid, including choroidal, optic nerve, or orbital tumors, posterior scleritis, choroidal detachment, choroidal inflammation, orbital pseudotumor, TED, orbital myositis, hypotony, and intracranial HTN
Pathophysiology/Dx of albinism
group of disorders characterized by a mutation in the genes responsible for the production of melanin. Albinism may affect the skin and eyes (oculocutaneous) or the eyes only (ocular albinism)
Signs of albinism
characterized by hypopigmetnation of the skin and fundus, it is translucency, foveal hypoplasia, ON hypoplasia, icrocornea, nystagmus, strab, and mis-routing of the temporal optic nerve fibers through the optic chiasm
Symptoms of albinism
photophobia and reduced VA
Systemic conditions associated with albinism
Hermanksy-Pudlak and Chediak-Higashi syndromes.
Number one cause of RRDs
Myopia
Epidemiology/Hx of RP
the most common retinal dystrophy and can be non-heritable or heritable (most commonly AD), or in association with uncommon systemic disorders; Ushers syndrome (5% of RP patients) is the most common associated systemic condition. The average age of diagnosis is 9-19.
Systemic disease assocaited with RP
Ushers
- AR
- hearing loss
Pathophysiology/Dx of RP
generic term given to a group of hereditary conditions (29 loci associated with various phenotypes) characterized by a progressive loss of PR and RPE function. Although rods and cones are involved, rod damage is more dominant. There is tremendous variability in presentations, which correlates with the mode of inheritance
Symptoms of RP
night blindness (most common symptom) and peripheral vision loss (only in dim light in early stages). It often takes years to decades for symptoms to develop; by 30 years of age, over 75% of patients are symptomatic
Signs of RP
classic triad of retinal bone spicule pigmentation (pigment clumping in the mid-periphery), arteriolar attenuation, and waxy optic nerve pallor
‣ Other signs include PSC, optic disc drusen, macular changes (atrophy, CME, ERMs), keratoconnus, myopia, and progressive contraction of the VF
ERG and RP
can be utilized for dx; in the early stages of RP, the scotopic ERG is reduced, and the photopic ERG is relatively normal.
Epidemiology/Hx of Stargardts
Stargardts disease is the most common hereditary macular dystrophy. The onset is typically in the first Ir second decade of life, most commonly between the ages of 6 and 20. The inheritance is typically AR. There is no sex predilection
What inheritance is stargardts
AR
Pathophysiology of Stargardts
most often due to a mutation in the ABCA4 transmembrane protein that is responsible for moving all-trans retinal from the photoreceptor disc lumen to the cytoplasm. This results in a toxic accumulation of all-trans retinal within the PR discs, leading to degeneration of the PRs and the RPE. Fundus flavimaculatus and stargardts disease are considered variants of the same disorder
Fundus flavimaculatus
diagnosis is reserved for patients WITHOUT macular dystrophy signs; it often presents later in life. Patients are commonly asymptomatic, although vision loss can still occur if fleck like lesions involve the macula
Symptoms of stargardts disease
rapid vision loss and color vision abnormalities. The level of decreased vision is often out of proportion with the fundus appearance in the early stages of the disease. Acuity is typically 20/200 bu the third decade and is stable or slowly progressive thereafter
Signs of Stargardts
early stages are characterized by bilateral yellow flecks scattered in a pisciform configuration throughout the posterior pole and mid-periphery. Non-specific RPE mottling of the macula May also be apparent. Later stages are characterized by a classic “beaten bronze” macular appearance (“bulls eye maculopathy”) and “salt and pepper” pigmentary changes in the periphery.
ERG and stargardts
ERG is normal in the early stages of the disease, but becomes abnormal as the condition progresses
Epidemiology/Hx of choroideremia
inheritance is X linked recessive, so only males are affected and all daughters are carriers. Onset is typically within the 1st decade
Pathophysiology/Dx of choroidermia
due to deficiency in rab geranyl-geranyl transferase, an enzyme utilized in membrane metabolism
Symptoms of choroideremia
night-blindness and peripheral vision loss. Night blindness occurs early in life in males and progresses to total night-blindness within a period of 10 years. Most patients have good visions until 50-60 years of age. In females, the condition is benign and non-progressive.
Signs of choroidermemia
progressive, bilateral, diffuse atrophy of the ROE and choriocapillaris, causing exposure of the underlying sclera. The macula is often spared until late stages of the disease. The optic disc and retinal blood vessels are often unaffected
DiffDx for night blindness
gyrate atrophy, choroidermia, and RP
Epidemiology/Hx for cone dystrophy
onset within 1-3rd decade. Inheritance is variable, but most commonly AD
Symptoms of cone dystrophy
slowly progressive decrease in central vision, severe photophobia, and severe color vision loss; symptoms are worse during the day. Cone dystrophy has a poor prognosis, with vision deteriorating to 20/400 by the 4th decade
Signs of cone dystrophy
‣ Early-normal fundus appearance by abnormal cone function on the ERG
‣ Late-central GA of the RPE with a bulls eye macular appearance, vessel attenuation, and temporal pallor of the optic nerve (similar to RP) with severe deutan-tritan color defects, an abnormal photopic ERG, and fine nystagmus
DiffDx for bulls eye maculopathy
stargardts disease, progressive cone dystrophy, chloroquine and hydroxychloroquine toxicity, and thioridazine toxicity
Epidemiology/Hx for bests
uncommon condition. Inheritance is AD. The age of onset varies but the condition classically presents in early childhood
Pathophysiology of bests
due to abnormal accumulation of material (posibly lipofuscin) in the RPE
Symptoms of bests
the majority of cases are detected with little or no patient symptoms (75% better than 20/40). Patients eventually complain of decreased vision
Signs of bests
characterized by a bilateral, yellow, round, subfoveal (“egg yolk”) lesion
Egg yolk in bests
thought to be an abnormal accumulation of lipofuscin within the RPE cells. The egg-yolk can remain stable for years with only a mild reduction in VA (20/30-20/50) into midlife
Stage 1 bests
previtelliform): characterized by an abnornal EOG (Arden ratio < 1.8) with a normal fundus in an asymptomatic patient
Stage 2 bests
vitelliform): egg-yolk macular lesion appears; this is most likely to occur between the ages of 3-15
Stage 3 best
pseudohypopyon): the entire lesion can become absorbed with little to no affect on vision.
Stage 4 bests
vitelliruptive): the egg yolk starts to break up (scrambled egg), mild VA loss is expected at this stage
Stage 5 bests
(end stage): characterized by moderate to severe vision loss due to choroidal neovascularization, hemorrhage, atrophy, and/or macular scarring
ERG and bests
normal ERG but abnormal EOG (even prior to vision loss or fundus signs)
Adult foveomacular vitelliform dystrophy vs bests
present in patients aged 30-50. Although similar signs to Bests, overall prognosis is better with minimal metamorphopsia, mild VA loss, normal EOG and ERG and slight tritan color defect.
Gyrate atrophy epidemiology/Hx
inheritance is AR, symptoms are prevalent in most patients by age 10
Pathophysiolgoy of gyrate atrophy
bilateral chororetinal degeneration due to a deficiency in the mitochondrial enzyme ornithine aminotransferase. Ornithine blood plasma levels will be high and can help in the dx if the clincal picture is unclear
Symptoms of gyrate atrophy
nyctalopia (night blindness), decreased vision, constricted VF
Signs of gyrate atrophy
multiple, well-defined, scalloped areas of peripheral chorioretinal atrophy; the lesions being in the midperiphery during childhood and then coalesce to engulf most of the posterior pole, with the macula being spared until the 4th or 7th decade. Associated with PSC, high myopia, and astigmatism
Epidemiology/Hx of RRDs
occur more often in males, typically over the age of 45 years old. Risk factors for RRD include
‣ Previous ocular surgery. Pseudophakic RDs often reuslts from small retinal holes at the vitreous base
‣ PVD: 10-15% of patients with an acute symptomatic PVD will have a retinal break
‣ Trauma
‣ Family history of RRD or previous occurrence of RRD. Both eyes are eventually involved in about 10% of cases.
‣ Myopia: 40% of all RDs occur in myopic eyes
‣ Lattice degeneration
Pathophysiology of RRDs
result from retinal breaks (full thickness retinal defects) that allow vitreous into the subretinal space, leading to separation of the sensory retina from the underlying RPE. Breaks include atrophic holes and vitreo-retinal traction tears
A break in the retina
The term BREAK=atrophic HOLES and traction TEARS. Thus an RRD refers to a retinal detachment that was caused by a hole or a tear
Atrophic holes and RRDs
round, often small, full-thickness defects that are NOT associated with vitreoretinal traction and therefore have a low risk for subsequent detachment. Holes are caused by chronic atrophy of the sensory retina and are most likely to be located in the temporal retina (superior > inferior quadrant)
Retinal tears and RRDs
caused by vitreous traction. Types of retinal tears include flap (horseshoe) takers and operculated tears
Flap tears
more serious)-the flap is present because of uneven vitreous traction; vitreoretinal traction often persists in these cases (vitreous stays attached to the flap), increasing the risk of a retinal detachment compared to operculated tears
Operculated tears
the initial vitreoretinal traction resutls in an even, symmetric tear. After forming the tear, the vitreous pulls away and vitreoretinal traction no longer persists, reducing the risk for a RD
Which is more serious, flap or operculated tears
Flap
Symptoms of RRDs
classic symptoms include vitreous floaters, flashing lights (photopsia), a curtain or veil over the vision, and decreased acuity
In RRDs, is it helpful to know what quadrant the flashes and floaters are?
The quadrant location in which a patient reports flashes of lights is of no value in predicting the location of a the primary retinal break; however, the quadrant location for VF defects is often valid. For example, if the field defect is reported in the inferior nasal quadrant, a superior temporal retinal break is expected
Signs of an acute RRD
convex undulating retina with clear subretinal fluid that does not shift with body position; Shafer’s sign (pigment cells in AC) may or may not be present. Additional signs may include a mild iritis and lower IOP in the affected eye
Signs of a chronic RRD
pigment demarcation line will be present (3 months or longer). Additional signs include intraretinal cysts, fixed folds, and/or subretinal precipitates
Most likely location for a retinal break in patients with RRD
the ST quadrant is the most likely location for a retinal break in patients with and RRD. 50% of eyes with an RRD will have more than one retinal break; in most of these cases the breaks are located within 90 degrees of one another.
Lattice degeneration
◦ Lattice is present in 6-10% of patients; 20-33% of patients with RRDs will have lattice degeneration; however only 1% of patients with lattice degeneration develop RRDS
◦ Lattice is an area of peripheral retinal thinning that is typically circumferential and concentric with the ora serrata.
◦ The inner portion of the lesion is atrophic (thin), while the outer margins of the lesions have a firm adhesion to the vitreous. The majority of lesions DO NOT contain the criss cross pattern of white sclerosed vessels; only 12% of patients have this classic appearance
Location of lattice
often bilateral and mor commonly located temporally and superiority
What systemic diseases can have lattice like lesions in the retina
Patients with Marfans syndrome, Sticklers syndrome, and Ehlers Danlos syndrome can have lattice like lesions (atypical lattice) that increase the risk of RD
Vitreoretinal tufts
small, focal areas of vitreous traction located in the retinal periphery. They occur in 5% of the population and are the second most common peripheral retinal lesion associated with RD (lattice is first); less than 1% of patients with vitreoretinal tufts develop a RD
Types of non RRDs
Serous
Tractional
Serous RD (exudative)
result from subretinal disorders that damage the RPE and a,low fluid accumulation under the retina. Examples include ARMD, inflammatory conditions, vascular conditions, neoplasms, and miscellaneous causes. Patients with exudative RDs are typically asymptomatic, unless the detachment involves the macula
Tractional RD
most commonly caused by PDR, ROP, and proliferative sickle cell retinopathy. Patients with TRDs usually do not have complaints of flashes and floaters and may be completely asymptomatic. Possible symptoms include decreased vision or progressive VF defects
Epidemiology/Hx of age related degenerative retinoschisis
occurs in 4-7% of the general population; more common in patietns 40 years or older
Pathophysiology/Dx of age related degenerative retinoschisis
degenerative process that results from a splitting of the OPL and the INL, resulting in elevation of the inner retina that mimics a RD
Symptoms of age related degenerative retinoschisis
typically asymptomatic; vision loss is rare
Signs of age related degenerative retinoschisis
dome shaped bullous elevation most commonly located inferiorly/temporally. Unlike a RD, the retina i immobile, bilateral findgins are common, and an absolute VF defect will correspond to the area of elevation
‣ 70% of patietns are hyperopic
‣ “Snowflake” or “frosting” and sheathed retinal vessels occur in the elevated retinal layer of the retinoschisis
‣ Inner wall breaks and outer wall breaks can occur in a retinoschisis. Outer wall breaks are more dangerous- they are required to cause a retinoschisis-associated RD. Outer wall breaks will appear “pock marked” on scleral depression. They can be surrounded with laser treatment to prevent an RD
Inner and outer wall breaks in age related degenerative retinoschisis
‣ Inner wall breaks and outer wall breaks can occur in a retinoschisis. Outer wall breaks are more dangerous- they are required to cause a retinoschisis-associated RD. Outer wall breaks will appear “pock marked” on scleral depression. They can be surrounded with laser treatment to prevent an RD
Pathophysiology/Dx of angioid streaks
large breaks in Bruch’s membrane that result from damage to the elastic core of Bruch’s membrane. 50% of cases are idiopathic; the remaining cases are caused by systemic disease
Most common condition associated with angioid streaks
Remember PEPSI for the most common causes of angioid streak: PXE (by far the most common cause), Ehlers Danlos Syndrome, Paget’s disease, Sickle-cell disease, and idiopathic
Symptoms of angioid streaks
may be asymptomatic or have profound vision loss from CNVM. 70% of patients will eventually have some form of vision loss
Signs of angioid streaks
spoke like (around the disc), linear, well-demarcated red/orange or brown lines within the elastic core of Bruch’s membrane
Toxocariasis pathophysiology/Dx
caused by toxocara canis or cati, which is the intestinal nematode in dogs or cats
Symptoms of toxocariais
patients may complain of floaters and blurred vision
Signs of toxocariass
ocular infection causes a significant unilateral inflammation response, resulting in optic nerve edema, RD, vitritis, endophthalmitis, and subretinal granulomas. large, white chorioretinal scars are present after the active infection has resolved
Chororetinitis
form of posterior uveitis that is characterized by inflammation of the choroid and retina. Examples include toxoplasmosis, toxocariasis, sarcoidosis, syphilis, CMV, serpiginous choroidopathy, birdshot, TB, and onchocerciasis
Acute posterior multifocal placoid pigment epitheliopathy
◦ Occurs acutely in young adults, typically after a viral illness. Characterized by bilateral, yellowish, flat, subretinal lesions. Disc edema and RD may also occur
◦ Usually resolves without treatment after a few weeks
Central areolar choroidal dsytriphy
◦ An AD condition characterized by bialteral large areas of GA in the macula. Will cause vision loss in the 4-5th decade. Poor prognosis
Senile choroidal atrophy
results from choroidal thinning and may cause an exaggerated tigroid fundus appearance due to increased visibility of the choroidal vasculature
What kind of RD in DRVOS
Tractional
What kind of RD in CHBALA
Serous
Glaucoma
the most likely source of an optic neuropathy. It is a leading cause of irreversible blindness worldwide. In the US, it is estimated that 2.79mill people have POAG, and this number is expected to increase in the future. The following will focus on subjects related to glaucoma
What is the most likely source of optic neuropathy
Glaucoma
an IOP greater than 21 (IOP>24 per the OHTT) on consecutive visit is in a patient with an open angle and without glaucomatous optic neuropathy
Ocular HTN
Risk factors for conversions of OHTN to POAG
◦ IOP-the only risk factor we can control!
◦ Race-AA-4-5x more likely to develop glaucoma
◦ Family Hx:overall proportion of POAG related to genetics is around 16%. Having a first degree relative (parent, sibling, child) with glaucoma is associated with a 3 to 4 fold increase risk of POAG
◦ Age-greater than 90% of those with glaucoma are over the age of 55
◦ Thin corneas
What is the only risk factor we can control for converion of OHTN to POAG
IOP
CCT readings and risk of glaucoma
- <555um=higher risk
- 555-588um=no change of risk based on CCT
- > 588um=lower risk
What CCT should we be worried about in glaucoma
<555
the most common type of glaucoma that occurs in 0.5-2.1% of those over age 40. It is defined as glaucomatous optic nerve damage with corresponding VF loss that occurs with IOPs greater than 21mmHg with an open angle on gonio. The exact cause of the increased IOP is not known
POAG
Signs for detection of POAG
damage to the optic nerve. Larger C/Ds, asymmetry between the optic nerves, focal vertical thinning or notching, nerve fiber bundle defects, and vascular signs (baring, hemorrhages) are important clinical signs. Recall that the larger the optic disc, the larger the expected cup size
ISNT rule in glaucoma
suggests that normal optic nerves should have the most rim tissue in the inferior quadrant, then superior, nasal, and the temporal quadrant (thinnest). However, glaucoma may initially damage the inferior OR superior rim
VF loss in POAG
◦ Initial field loss is variable, but nasal steps are the most common. Paracentral and arcuate defects are also relatively common
Symptoms of POAG
usually asymptomatic until later stages when significant peripheral or central vision is lost
Port wine stains and glaucoma
vascular birthmarks that have a high association with ipsilateral glaucoma (45% of cases). It is rarely associated with systemic disorders-most notably Sturge-Weber syndrome (5% of cases)
Pseudoexfoliation syndrome and glaucoma
age related systemic condition that is most commonly in caucasians, especially those of Scandinavian decent. PXE is the most common identifiable cause of elevated IOP and resultant glaucoma
Most common identifiable cause of elevated IOP and resultant glaucoma
Pseudoexfoliation syndrome
Characteristics of Pseudoexfoliation syndrome
characterized by abnormal, white, flaky despots whose exact compilation and cause of formation remain unknown. The flaky deposits are found throughout the body and may have systemic implications
◦ In the eye, these deposits can be found on the pupillary margin, on the lens capsule in a bulls eye pattern, on the lens zonules, and in the TM
◦ Gonio often reveals a Sampoalesi’s line due to the accumulation of pigment that is releases as the deposits rub against the posterior iris epithelium.
Sampaolesi’s line
increased pigmentation anterior to Schwalbes line; it is assocaited with PES and PDS
What is Pseudoexfoliation syndrome associated with
poor pupil dilation and an increased risk of lens subluxation and cataract surgery complications due to weak lens zonules
Patient presentation of pseudoexfoliation syndrome
majority of patients present with unilateral or bilateral asymmetric pseudoexfoliative glaucoma. The risk of developing glaucoma is 15% within 10 years
Pigmentary dispersion syndrome
Condition that is usually bilateral, more common in caucasians, and presents in young patients. The condition is more common in myopes and males.
‣ Pigmentary dispersion Syndrome is thought to resutls from higher anterior chamber pressure that causes excessive bowing of the iris posteriorly, resulting in more contact between the iris and lens zonules; this leads to pigment shedding off the posterior edge of the iris
Symptoms of PDS
often asymptomatic, but may have blurred vision and halos around lights after exercising or pupil dilation
Signs of PDS
TIDs, Krukenburg’s spindle (vertically oriented line of pigment on the corneal endo), pigment on the anterior capsule of the lens and the iris surface, and TM hyperpigmentation
Prognosis of PDS
Pigment dispersion syndrome may burn out over time as the lens thickens, resulting in less contact between the posterior iris epithelium and lens zonules
Risk of glaucoma and PDS
risk of glaucoma at 5 years is 10% and 15 years is 15%
Angle recession glaucoma
characterized by a wide open angle with a recessed iris and widened ciliary body band. It is most often unilateral and results from blunt trauma. Although the angle appears wide open, the TM is damaged, resulting in an increased risk of glaucoma over time. 10% of patietns with angle recession involving at least 2/3 of the angle will develop glaucoma
Considered a type of POAG in which the NFL damage occurs at lower pressures; most define the condition as IOP less than 21mmHg with an open angle on gonio and glaucomatous ON damage with corresponding VF loss.
NTG
Who is at risk of NTG
Females are at a greater risk and the condition has the highest prevalence among Japanese patients. Additional risk factors include vascular disorders such as Raynaud’s phenomenon or migraines, low blood pressure, sleep apnea, hypercoagulation, and taking BP meds before bedtime (may decrease ocular perfusion pressure)
What must you do before Dx NTG
Before dx NTG, it is important to take diurnal IOP readings to ensure the diagnosis is truly NTG rather than POAG. This who develop NTG typically have IOPs in the high teens.
Signs of NTG
similar to those of POAG, but Drance hemorrhages are more common in NTG. Initial VF defects in NTG are usually more focal (but more dense) and closer to fixation because the temporal and interotemporal rim tissue is more commonly affects first (compared to POAG)
Other causes of optic neuropathy and NTG
Since IOP is within the normal range in NTG, it is important to rule out other possible sources of the optic neuropathy, especially cases of suspected unilateral NTG. Consider asking about a history of hemorrhagic shock, myocardial infarction, anemia, syphilis, and vasculitis
Primary angle closure glaucoma
• can be acute or intermittent (sub acute) and occurs as a result of the posterior pressure in the posterior chamber pushing the peripheral iris anteirorly into contact with the TM, blocking part or all of the TM and impeding aqueous outflow. The pupil may or may not be blocked in angle closure glaucoma
What are the two main causes of primary acute angle closure glaucoma
Pupillary block
PI
Pupillary block
occurs in patients with anatomically narrow angles (hyperopes) when the aqueous flow through the pupil is blocked (usually by the lens but can also be the vitreous). Patients are most at risk for angle closure when the pupil reaches the mid position after coming down from dilation, as this is the point of greatest iris-lens contact
Risk factors for pupillary block related angle closure
hyperopes, advancing cataracts, Asians and eskimos, and lens subluxation
Plateau iris
characterized by anteriorly positioned ciliary processes that push the peripheral iris forward into contact with the TM. Slit lamp and gonio reveals a flat iris plane, a normal central anterior chamber depth, but a convex peripheral iris.
What can be done to differentiate plateau iris and pupillary block
peripheral iridotomy or iridectomy can be performed to differentiate pupillary block from plateau iris configuration; in the latter, the peripheral iris proximity to angle structures will be unchanged after the procedure.
Sub acute or chronic angle closure
occurs when part of the angle closes, causing episodes of elevated IOP without patient symptoms. Subacute angle closure is more common than acute angle closure. Chronic angle closure should be expected in patients with occludable angles and any of the following signs
‣ PAS or pigment splotching the TM
‣ Progressive optic nerve damage with corresponding VF loss
Acute angle closure
defined as angle closure (no TM visible with gonio) causing an acute IOP spike (50-100mmHg) and patient symptoms. Signs and symptoms include:
‣ Vomiting, intense ocular pain, HA, halos, nausea, and progressive vision loss.
‣ Prominent signs include a hazy cornea, mid-dilated pupil that responds poorly to light, ciliary flush, and glaucomflecken
‣ IOP must be quickly lowered so that corneal edema will resolve, allowing for a peripheral iridotomy
Glaucomflecken
anterior subcapsular opacities that resutls from lens epithelial cell ischemia and necrosis secondary to high pressures
What is the greatest threat to vision in acute angle closure
CRAO
occurs when IOP is higher than the prefusuion pressure of the CRA
Topamax and glaucoma
used for treating migraines, weight loss, epilepsy, can cause acute secondary angle closure. It causes supraciliary effusion, which moves the lens and iris forward into contact with the TM, resulting in angle closure. This typically occurs within the first month of use or if the dosage is increased
Patients with a history of angle closure are at higher risk of
higher risk for developing open angle component of glaucoma due to TM and ONH damage. Will manifest as high IOP with ON damage with an open angle on gonio, and may be immediate or years later. Pts with narrow angle AND open angle component of glaucoma have mixed mechanism glaucoma
Mixed mechanism glaucoma
Pts with narrow angle AND open angle component of glaucoma have mixed mechanism glaucoma
serious condition that frequently results in severe vision loss and a painful eye. Neovascular glaucoma most commonly occurs with condition that results in severe retinal ischemia
NVG
Most common cause of NVG
CRVO
-followed by PDR, OIS, and RD. Carotid artery disease and CRAO are less common causes
Most important sign to recognize in NVG
rubeosis of the iris. The pupillary margin should be examined closely for capillary tufts or early signs of neo.
Progression of rubeosis of the iris can cause secondary angle closure via what mechanisms
‣ Neo of the angle is always accompanies by fibrous tissue that forms a membrane over the TM, preventing aqueous outflow. A significant fibrous membrane can develop even with only 1-2 vessels on the angle
‣ The fibrovascular membrane can stick to the iris, pulling it into contact with the TM and causing secondary angle closure. This is known as “zippering of the angle”
Occurs as a result of a PAS and PS formation in uveitis. Remember that uveitis causes the iris to become inflamed and sticky. The iris can stick to the lens or the TM
Uveitic glaucoma
PS and uveitic glaucoma
will only cause an elevation in IOP when there is 360 degrees of attachment between the iris and the lens. This results in iris bombe and pupillary block, which moves the iris anteriorly into contact with the TM
PAS and uveitic glaucoma
PAS will cause varying degrees of IOP elevation depending on the extent of angle involvement
Importance in managing uveitis aggressively
very important to manage uveitis aggressively to prevent the formation of PS and PAS. If PS occur during the current epidose of inflammation, every effort should be made to break them. Once the current uveitis episode resolves, PS can only be removed surgically
Congenital glaucoma
◦ Onset is typically birth to 3 months of age; the condition is most often bilateral and is more common in males. Congenital glaucoma results from a developmental abnormality in the AC that impedes aqueous outflow
an enlarged eye with a corneal diameter >12mm that occurs by one year of age in patients with congenital glaucoma as a result of elevated IOP
Buphthalmos
Group of disorders characterized by abnormal corneal endothelium that grows onto the iris or in the angle, increasing the risk for glaucoma. Signs include corneal edema, iris atrophy, PAS, and angle closure. These syndromes are more common in females and patients 20-50;
ICE syndromes
What are the ICE syndromes
‣ Essential Iris atrophy-iris thinning with resultant heterochromia, polycorea, corectopia, and ectropion uvea
‣ Chandler’s syndrome-the corneal endothelium will have a”Beaten metal” appearance with corneal edema and corectopia
‣ Iris-Nevus-Syndrome (Cogan-Reese Syndrome)-Nodules will be present on the anterior iris surface
What are the inflammatory glaucoma’s
- glaucomatocyclitic crisis (Posner-Schlossman syndrome)
- Fuch’s Heterochromic iridocyclitis
- Phacolytic glaucoma
Characterized by acute trabeculitis that results in an acute elevation of IOP (40-60mmHg). There will be few cells and flare in the AC, and gonio will reveal an open angle. The condition occurs most often to young to middle aged patients and is characterized by recurrent unilateral episodes that often burn out over time
• Glaucomatocyclitic Crisis: AKA Posner-Schlossman syndrome
chronic, non-granulomatous, low grade anterior uveitits with stellate KPs. Additional signs include iris heterochromia and iris/angle neo. Patients have an increased risk of glaucoma (due to chronic TM damage) and cataracts due to chronic inflammation
Fuchs heterochromic iridocyclitis
results from a hyper mature cataract that leaks lens material into the AC, resulting in blockage of the aqueous outflow through TM. Cells, flare, and iridescent lens particles will be present within the AC
Pahcolytic glaucoma
RNFL and glaucoma
glaucoma is characterized by retinal ganglion cell death and corresponding RNFL loss. Upon clinical exam, the RNFL loss can be difficult to see, especially if it is early or mild. In an effort to detect structural optic nerve damage earlier, computerized instruments have been developed to detect mild changes in the RNFL. They may also be helpful to detect progression
Imaging for the RNFL
‣ GDx uses scanning laser polarizer to detect thinning of the RNFL
‣ OCT uses optical coherence tomography
‣ HRT uses confocal scanning laser ophthalmoscopy for a topographical evaluation of the ONH and peripapillary rim
OCT and glaucoma
• The OCT Stratus (zeiss) is a time domain system and the Spectralis (Heidelberg) and Cirrus (zeiss) are spectral domain systems. Spectral domain results in better resolution and is faster and more sensitive. Swept source, the newest technology, is even better than spectral domain because if higher sensitivity and speed
Macular evaluation in glaucoma
the retinal ganglion cells are densest in the macula; studies have shown that patients with glaucoma show thinner areas in the macula that correspond with VF loss. There are 3 instruments that are capable of analyzing macular ganglion cell thickness: RTVue Ganglion Cell Complex Analyzer (optovue), Spectralis Posterior Pole Asymmetry Analyzer (Heidelberg), and CIrrus Ganglion Cell Analysis (Zeiss)
Macular evaluation instruments for glaucoma
‣ RTVue measures the three innermost macular layers and compares the patients data to a normative database
‣ The spectralis does NOT have a normative database but allows for inter and intra-eye asymmetry results
‣ The cirrus measures only the ganglion cell-IPL complex and compares resutls to a normative database
When to do gonio
performed in order to Dx the appropriate type of glaucoma. Additional indications include narrow angles on Van Herick, signs of PDS (TIDs and Kruckenberg spindle), iris neo, suspicious iris lesion, Hx of ocular trauma, or when following a CRVO
Documentation of gonio
performed in order to Dx the appropriate type of glaucoma. Additional indications include narrow angles on Van Herick, signs of PDS (TIDs and Kruckenberg spindle), iris neo, suspicious iris lesion, Hx of ocular trauma, or when following a CRVO
AC angle structures from posterior to anterior
: I Can See The Stupid Line=iris, CB, scleral spur, TM, Schwalbes line
‣ Static automated perimetry (SAP)
the Humphrey VF is the most popular SAP threshold used
‣ Short Wavelength Automated Perimetry (SWAP)
uses a yellow background with blue stimuli to detect early glaucomatous damage that is not detected with SAP
Frequency Doubling Technology (FDT)
fast (90s per eye) screening field that uses sinusoidal gratings as the stimulus
Extent of normal VF
100 degrees temporal, 60 degrees nasal and superior, and 70 degrees inferior. The blind spot is located 15 degrees temporal and 7.5 degrees in diameter
Reliability indices
fixation loses, FP, FN, and short term fluctuations (STF)
Short term fluctuations on VF
determined by randomly retesting certain points. Poor performance on STFs could indicate poor reliability of early glaucoma
Fixation losses > ____ will be flagged on VF
20%
FP/FN errors > ___ will be flagged on VF
33%
Grayscale on VF
this is a gross representation of the VF. The darker areas indicate reduced sensitivity. This can be used for patient education purposes but has minimal value in interpretation
Total deviation on VF
compares how well the patient did compared to the database of healthy, age-matched samples
• A zero indicates the patient scored the same as the normative database. A positive number means the patient performed better than expected. A negative number means the patient performed worse than expected
• The darker the probability box on the total deviation probability plot, the greater the patients response deviated from the expected response
Pattern deviation
filters out diffuse loss (cataracts) to show only focal areas of deviation that are more typical in glaucoma
Glaucoma hemifield test
compares sensitivity levels between the upper and lower hemifields. Remember that glaucoma often causes asymmetric ONH damage, resulting in an asymmetric field loss above and below the horizontal midline. The GHT will state WNL, borderline, ONL, or reduced sensitivity
Visual field indies (VFI)
this gives a measure of the rate of visual field loss over time and is given as a % of normal. 100% means no loss of the VF, 0% means total blindness
Mean deviation on VF
Mean deviation (MD): the average of the differences between the patients overall sensitivity and the overall sensitivity of the normative database. MD is an indication of diffuse loss; the more negative the MD, the more general depression in the VF
Pattern standard deviations on VF
compares the patients shape of the hill of vision to the shape of the hill of vision of the normative database. PSD indicates focal areas of depression that are typical in glaucoma. It is always an absolute value; higher PSD means greater focal VF loss.
