Ocular Manifestations Ocular Manifestations of Systemic Disease of Systemic Disease
Dr. Riyad G. BanayotDr. Riyad G. Banayot
OCULAR OCULAR MANIFESTATIONSMANIFESTATIONS
SYSTEMIC DISEASESYSTEMIC DISEASE
Categories of systemic diseaseCategories of systemic disease
CongenitalCongenital VascularVascular Endocrine/MetabolicEndocrine/Metabolic AutoimmuneAutoimmune IdiopathicIdiopathic
InfectiousInfectious Drugs/ToxinsDrugs/Toxins NeoplasticNeoplastic TraumaTrauma
Eye examinationEye examination
VisionVision External – Look at eyelids, make sure everything looks normal.External – Look at eyelids, make sure everything looks normal. PupilsPupils
Relative afferent papillary defectRelative afferent papillary defect See if pupils are reacting normallySee if pupils are reacting normally
Motility – Have the patient look up, down, right and left.Motility – Have the patient look up, down, right and left. Exam of anterior segment Exam of anterior segment Dilated ophthalmoscopy – Examine retina, optic nerve, and Dilated ophthalmoscopy – Examine retina, optic nerve, and
vessels.vessels. Visual fields – Place you finger in different places in patientVisual fields – Place you finger in different places in patient’’s s
visual field and ask him if he sees it. Test one eye at a time.visual field and ask him if he sees it. Test one eye at a time.
CongenitalCongenital disorders with ocular disorders with ocular manifestationsmanifestations
Down SyndromeDown Syndrome Marfan's SyndromeMarfan's Syndrome Myotonic DystrophyMyotonic Dystrophy Tuberous SclerosisTuberous Sclerosis Congenital Metabolic DisordersCongenital Metabolic Disorders
Lysosomal storageLysosomal storage Carbohydrate metabolismCarbohydrate metabolism
NeurofibromatosisNeurofibromatosis
MarfanMarfan’’s Syndromes Syndrome
Myotonic DystrophyMyotonic Dystrophy
NeurofibromatosisNeurofibromatosis
VascularVascular disorders with ocular disorders with ocular manifestationsmanifestations
HypertensionHypertension MigrainesMigraines EmboliEmboli Amaurosis fugaxAmaurosis fugax
Vascular HypertensionVascular Hypertension
Affects eyes the same way it affects the rest of Affects eyes the same way it affects the rest of the bodythe body
– – Arteriolar sclerosisArteriolar sclerosis
– – Direct pressure damageDirect pressure damage Number one predisposing factor for otherNumber one predisposing factor for other
eye disorders like CRVO, BRVOeye disorders like CRVO, BRVO
Vascular HypertensionVascular Hypertension
In a normal eye, the vessel walls areIn a normal eye, the vessel walls are
transparent - you see the blood (red)transparent - you see the blood (red) Arteriolar sclerosis causing thickening of the Arteriolar sclerosis causing thickening of the
vessel wallsvessel walls
– – Early sclerosis - Early sclerosis - ““copper wirecopper wire”” vessel vessel appearanceappearance
– – More sclerosis - More sclerosis - ““silver wiresilver wire”” appearance appearance
Vascular HypertensionVascular Hypertension
Arteries and veins share the same sheathArteries and veins share the same sheath As arteriolar walls sclerose and thicken, the vein As arteriolar walls sclerose and thicken, the vein
is compressed - AV Nicking is compressed - AV Nicking
Vascular HypertensionVascular Hypertension
Acutely elevated blood pressure can lead to Acutely elevated blood pressure can lead to fibrinoid necrosis of blood vessel wallsfibrinoid necrosis of blood vessel walls
– – Get exudates, cotton wool spots, Get exudates, cotton wool spots, hemorrhageshemorrhages
– – Can also see macular edema, nerve Can also see macular edema, nerve edemaedema
Hypertensive RetinopathyHypertensive Retinopathy
Hypertensive RetinopathyHypertensive Retinopathy
Hypertensive RetinopathyHypertensive Retinopathy
Vascular HypertensionVascular Hypertension
Classically, it is taught that hypertensivesClassically, it is taught that hypertensives
should be referred for ophthalmologicshould be referred for ophthalmologic
evaluationevaluation
– – No point in this - we canNo point in this - we can’’t do anythingt do anything
– – Send them to their primary care physician for Send them to their primary care physician for BP controlBP control
– – To the emergency room if systolic BP over To the emergency room if systolic BP over 200.200.
Vascular MigrainesVascular Migraines
Migraines are a vasospastic phenomenonMigraines are a vasospastic phenomenon Usually the effects are temporary, but theyUsually the effects are temporary, but they
can be permanent - like a stroke can be permanent - like a stroke
Vascular MigrainesVascular Migraines
Ocular manifestationsOcular manifestations
– – ScintillationsScintillations
– – Transient homonymous hemifield lossTransient homonymous hemifield loss
– – Transient cortical visual lossTransient cortical visual loss
– – Amaurosis fugaxAmaurosis fugax
– – Rarely, CRAO or BRAORarely, CRAO or BRAO
Vascular MigrainesVascular Migraines
TreatmentTreatment
– – Prevent frequent attacksPrevent frequent attacks
– – Examine for visual lossExamine for visual loss
– – Consider discontinuation of oral Consider discontinuation of oral contraceptives as they can lead to thrombotic contraceptives as they can lead to thrombotic eventsevents
Vascular EmboliVascular Emboli
Usually are atherosclerotic/cholesterolUsually are atherosclerotic/cholesterol
plaques from the carotidsplaques from the carotids Can be from other sourcesCan be from other sources
– – heart and heart valvesheart and heart valves
– – IV drug abuse - talcIV drug abuse - talc Manifests as acute visual loss such asManifests as acute visual loss such as
CRAO, BRAOCRAO, BRAO
Emboli affecting the eyesEmboli affecting the eyes
Cholesterol EmbolusCholesterol Embolus
Talc EmboliTalc Emboli
Vascular Amaurosis fugaxVascular Amaurosis fugax
Sudden, painless monocular loss of visionSudden, painless monocular loss of vision
– – Usually manifests like Usually manifests like ““a window shadea window shade
coming downcoming down”” or dimming or dimming
– – By definition, vision returns to normal within By definition, vision returns to normal within a few minutesa few minutes
Caused by temporary arterial occlusionCaused by temporary arterial occlusion
Vascular Blood DyscrasiasVascular Blood Dyscrasias
Hyperviscosity syndromes - thrombosisHyperviscosity syndromes - thrombosis
– – Including polycythemia, myeloma, Including polycythemia, myeloma, leukemialeukemia Thrombocytopenia - bleedingThrombocytopenia - bleeding Anemia - ischemiaAnemia - ischemia
– – Including sickle cell anemiaIncluding sickle cell anemia Lymphoma Lymphoma
Hyperviscosity effects on retinaHyperviscosity effects on retina
LeukemiaLeukemia
LeukemiaLeukemia
Sickle Cell RetinopathySickle Cell Retinopathy
Sickle Cell RetinopathySickle Cell Retinopathy
Endocrine/MetabolicEndocrine/Metabolic
Primary disease in this category is:Primary disease in this category is:
Diabetes MellitusDiabetes Mellitus
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
Most common cause of blindness in theMost common cause of blindness in the
U.S. between the ages of 18 and 74U.S. between the ages of 18 and 74 8000 people become blind annually in U.S.8000 people become blind annually in U.S. Many effects on the eyeMany effects on the eye
- Changes refraction- Changes refraction
- Causes cataracts- Causes cataracts
- Most important is retinopathy- Most important is retinopathy
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
Ocular problems are a function of durationOcular problems are a function of duration
of diseaseof disease
- 5 years - 23%- 5 years - 23%
- 20 years - 90%- 20 years - 90%
- 30 years - 99%- 30 years - 99%
- Rare in children under 10 regardless of - Rare in children under 10 regardless of duration of diseaseduration of disease
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
Nonproliferative diabetic retinopathy (NPDR)Nonproliferative diabetic retinopathy (NPDR)
– – Capillaries develop leaks and occludeCapillaries develop leaks and occlude
– – See microaneurysms, dot/blot hemorrhages, See microaneurysms, dot/blot hemorrhages, exudates, and macular edemaexudates, and macular edema
– – Vision loss at this stage is from macular edemaVision loss at this stage is from macular edema
Diabetes MellitusDiabetes Mellitus
NPDRNPDR
Macular EdemaMacular Edema
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
NPDRNPDR
– – As it becomes more severeAs it becomes more severe
• • Marked vascular tortuosityMarked vascular tortuosity
• • More hemorrhagesMore hemorrhages
• • Cotton wool spots Cotton wool spots
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
Proliferative diabetic retinopathy (PDR)Proliferative diabetic retinopathy (PDR)
– – Due to ischemiaDue to ischemia
– – Neovascularization - usually on nerve headNeovascularization - usually on nerve head
– – Vessels bleed into vitreousVessels bleed into vitreous
– – Fibrous tissue develops and causes tractionalFibrous tissue develops and causes tractional
retinal detachmentsretinal detachments
– – PROFOUND visual loss PROFOUND visual loss
PDRPDR
Vitreous HemorrhageVitreous Hemorrhage
Iris NeovascularizationIris Neovascularization
Tractional Retinal DetachmentTractional Retinal Detachment
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
PDRPDR
– – Needs prompt treatment to prevent Needs prompt treatment to prevent vitreous vitreous hemorrhages and retinal hemorrhages and retinal detachmentsdetachments
– – Treatment causes regression of the Treatment causes regression of the vesselsvessels
– – Treatment reduces visual loss by 50%Treatment reduces visual loss by 50%
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
PDRPDR– – TreatmentTreatment
• • Scatter 1000-3000 laser burns Scatter 1000-3000 laser burns throughout retinathroughout retina• • Avoid maculaAvoid macula• • Decreases retinal oxygen need which Decreases retinal oxygen need which decreases the impetus for decreases the impetus for
neovascularizationneovascularization• • Treatment needs to be ongoing Treatment needs to be ongoing
Laser effectivenessLaser effectiveness
EndocrineEndocrineDiabetes MellitusDiabetes Mellitus
The only thing that has been shown to delay the The only thing that has been shown to delay the onset of and/or decrease the severity of diabetic onset of and/or decrease the severity of diabetic eye disease is controlling the blood sugar - up to eye disease is controlling the blood sugar - up to 76% reduction!76% reduction!
All diabetics need an eye exam at least once per All diabetics need an eye exam at least once per yearyear
Autoimmune DisordersAutoimmune Disorders
Connective tissue diseases - Connective tissue diseases - SLE, SjogrenSLE, Sjogren’’s Syndrome, s Syndrome,
Ankylosing SpondylitisAnkylosing Spondylitis GravesGraves’’ disease disease Myasthenia gravisMyasthenia gravis Rheumatoid arthritisRheumatoid arthritis Giant cell arteritisGiant cell arteritis
AutoimmuneAutoimmuneConnective Tissue DiseasesConnective Tissue Diseases
Dry eyes are the most common manifestationDry eyes are the most common manifestation Symptoms:Symptoms:
– – BurningBurning
– – PhotophobiaPhotophobia
– – Foreign body sensationForeign body sensation
Autoimmune SLEAutoimmune SLE
Dry eyesDry eyes ScleritisScleritis Peripheral corneal ulcersPeripheral corneal ulcers RetinopathyRetinopathy Optic neuropathyOptic neuropathy
SLE RetinopathySLE Retinopathy
SLE Optic NeuropathySLE Optic Neuropathy
AutoimmuneAutoimmuneGravesGraves’’ disease disease
Improperly called Improperly called ““thyroid eye diseasethyroid eye disease”” Immune system attacks both thyroid glandImmune system attacks both thyroid gland
and extraocular muscles (EOM)and extraocular muscles (EOM) Not related to thyroid hormone levelsNot related to thyroid hormone levels Can progress even when euthyroid Can progress even when euthyroid
AutoimmuneAutoimmuneGravesGraves’’ disease disease
Manifestations:Manifestations:
– – ProptosisProptosis
– – Lid retractionLid retraction
– – Exposure keratopathyExposure keratopathy
– – Strabismus (misaligned eyes)Strabismus (misaligned eyes)
– – Optic neuropathy Optic neuropathy
GravesGraves’’ Ophthalmopathy Ophthalmopathy
GravesGraves’’ Ophthalmopathy Ophthalmopathy
GravesGraves’’ Ophthalmopathy Ophthalmopathy
GravesGraves’’ Ophthalmopathy Ophthalmopathy
Autoimmune Autoimmune GravesGraves’’ disease disease
Treatment - as neededTreatment - as needed
– – Artificial tearsArtificial tears
– – Oral steroidsOral steroids
– – Strabismus surgeryStrabismus surgery
– – Lid surgeryLid surgery
– – Orbital decompression surgeryOrbital decompression surgery
– – Orbital radiation Orbital radiation
Autoimmune Autoimmune Myasthenia gravisMyasthenia gravis
Autoimmune reaction to the acetylcholineAutoimmune reaction to the acetylcholinereceptors in muscle tissuereceptors in muscle tissue
Ocular manifestations:Ocular manifestations:– – PtosisPtosis– – StrabismusStrabismus– – Facial weaknessFacial weakness– – Worse at the end of the day - fatigues Worse at the end of the day - fatigues easily easily
Myasthenia gravisMyasthenia gravis
Autoimmune Autoimmune Myasthenia gravisMyasthenia gravis
Other manifestations:Other manifestations:
– – Proximal limb weaknessProximal limb weakness
– – Difficulty swallowing or breathing (which can Difficulty swallowing or breathing (which can be deadly)be deadly)
– – Sometimes triggered by an underlyingSometimes triggered by an underlying
thymoma - always get chest CT thymoma - always get chest CT
AutoimmuneAutoimmuneMyasthenia gravisMyasthenia gravis
Confirm diagnosis with edrophonium (Tensilon) Confirm diagnosis with edrophonium (Tensilon) test and checking for acetylcholine receptor test and checking for acetylcholine receptor antibodiesantibodies
Treatment:Treatment:– – Oral anticholinesterase (e.g. pyridostigmine)Oral anticholinesterase (e.g. pyridostigmine)– – SteroidsSteroids– – Immunosuppressive therapyImmunosuppressive therapy– – Surgical removal of any thymomaSurgical removal of any thymoma
AutoimmuneAutoimmuneRheumatoid ArthritisRheumatoid Arthritis
Dry eyesDry eyes EpiscleritisEpiscleritis ScleritisScleritis Corneal ulcerationCorneal ulceration UveitisUveitis
Rheumatoid EpiscleritisRheumatoid Episcleritis
Rheumatoid ScleritisRheumatoid Scleritis
Rheumatoid Necrotizing ScleritisRheumatoid Necrotizing Scleritis
Rheumatoid Scleromalacia PerforansRheumatoid Scleromalacia Perforans
Rheumatoid Corneal Ulceration Rheumatoid Corneal Ulceration
Autoimmune Rheumatoid ArthritisAutoimmune Rheumatoid Arthritis
Treatment:Treatment:
– – Artificial tearsArtificial tears
– – Topical steroidsTopical steroids
– – Oral steroidsOral steroids
– – Immunosuppressive therapyImmunosuppressive therapy Treat in conjunction with a rheumatologistTreat in conjunction with a rheumatologist
Autoimmune Juvenile Rheumatoid Autoimmune Juvenile Rheumatoid ArthritisArthritis
Ocular manifestations:Ocular manifestations:
– – Asymptomatic iritisAsymptomatic iritis
– – Secondary cataracts and glaucomaSecondary cataracts and glaucoma PauciarticularPauciarticular Rheumatoid factor -veRheumatoid factor -ve ANA +ANA +
Idiopathic Disorders With Ocular Idiopathic Disorders With Ocular ManifestationsManifestations
SarcoidosisSarcoidosis Multiple SclerosisMultiple Sclerosis HLA B27-associated disorders - ReiterHLA B27-associated disorders - Reiter’’s,s,
ankylosing spondylitis, etcankylosing spondylitis, etc BehcetBehcet’’s diseases disease WegenerWegener’’s granulomatosiss granulomatosis
Idiopathic SarcoidosisIdiopathic Sarcoidosis
Generalized, multisystem inflammatoryGeneralized, multisystem inflammatory
disorderdisorder 25% exhibit ocular manifestations25% exhibit ocular manifestations Much more common in blacks andMuch more common in blacks and
hispanics than whites hispanics than whites
Idiopathic SarcoidosisIdiopathic Sarcoidosis
Ocular manifestations:Ocular manifestations:– – Orbital and eyelid granulomasOrbital and eyelid granulomas– – Lacrimal gland infiltration - causing dry eyeLacrimal gland infiltration - causing dry eye– – Conjunctival nodulesConjunctival nodules– – Uveitis (intraocular inflammation) - mostUveitis (intraocular inflammation) - most
common manifestationcommon manifestation– – Iris nodulesIris nodules– – RetinopathyRetinopathy
Sarcoid UveitisSarcoid Uveitis
Sarcoid RetinopathySarcoid Retinopathy
Idiopathic SarcoidosisIdiopathic Sarcoidosis
Treatment:Treatment:
– – Topical steroidsTopical steroids
– – Systemic steroidsSystemic steroids
– – Sometimes immunosuppressive therapySometimes immunosuppressive therapy
Infectious Diseases With Ocular Infectious Diseases With Ocular ManifestationsManifestations
HIV/AIDSHIV/AIDS Herpes ZosterHerpes Zoster CandidiasisCandidiasis SyphilisSyphilis TBTB Lyme diseaseLyme disease Many others Many others
Infectious HIV/AIDSInfectious HIV/AIDS
Ocular manifestations:Ocular manifestations:
– – Dry eyeDry eye
– – KaposiKaposi’’s sarcoma of eyelidss sarcoma of eyelids
– – HIV retinopathyHIV retinopathy
– – Cytomegalovirus infection (CMV)Cytomegalovirus infection (CMV)
HIV RetinopathyHIV Retinopathy
Infectious HIV/AIDSInfectious HIV/AIDS
CMV retinitisCMV retinitis– – Leading cause of visual loss in AIDSLeading cause of visual loss in AIDS– – 25% of AIDS patients get it25% of AIDS patients get it– – CD4 <50CD4 <50– – Hemorrhagic necrosis of retinaHemorrhagic necrosis of retina– – Indicates poor prognosis for survivalIndicates poor prognosis for survival– – Fortunately, infrequently seen since advent ofFortunately, infrequently seen since advent of
protease inhibitorsprotease inhibitors
HIV/AIDS: CMV RetinitisHIV/AIDS: CMV Retinitis
Infectious HIV/AIDSInfectious HIV/AIDS
Treatment of CMV retinitis:Treatment of CMV retinitis:
– – IV ganciclovirIV ganciclovir
– – IV foscarnetIV foscarnet
– – Intravitreal ganciclovirIntravitreal ganciclovir
– – Ganciclovir sustained-release intravitrealGanciclovir sustained-release intravitreal
implants implants
Infectious Herpes Zoster Infectious Herpes Zoster OphthalmicusOphthalmicus
10-15% of zoster is ocular10-15% of zoster is ocular Follows distribution of V1Follows distribution of V1 Eye is often involvedEye is often involved
- Roughly 5-10%- Roughly 5-10%
- Roughly 40% if tip of nose involved- Roughly 40% if tip of nose involved
Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
Prevalence increases with agePrevalence increases with age In patients under 40 - suspect HIVIn patients under 40 - suspect HIV
Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
Painful, crusty, vesicular lesions alongPainful, crusty, vesicular lesions along
distribution of nervedistribution of nerve Exam (of eyes):Exam (of eyes):
– – Corneal stromal keratitis (melting)Corneal stromal keratitis (melting)
– – UveitisUveitis
– – RetinitisRetinitis
– – Neuritis Neuritis
Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
Herpes Zoster Ophthalmicus Herpes Zoster Ophthalmicus
Herpes Zoster OphthalmicusHerpes Zoster Ophthalmicus
Treatment:Treatment:
– – Oral acyclovir 800mg 5x/day (or IV)Oral acyclovir 800mg 5x/day (or IV)
– – Should be started within 7 days of onsetShould be started within 7 days of onset
– – With eye involvement:With eye involvement:
• • Topical steroids (not trifluridine)Topical steroids (not trifluridine)
• • LubricationLubrication