Anterior eye 4 - Uvea Flashcards

1
Q

What does the uvea consist of?

A

• Pigmented middle layer of eye
• Iris: determines diameter of pupil and separates anterior and posterior chambers
• Ciliary body: produces aqueous humour, and changes shape of lens power
• Choroid: provides oxygen and nutrients to outer retina

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2
Q

What are congenital abnormalities of the uvea?

A

• Aniridia
• Axenfield-Reiger syndrome
• Coloboma

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3
Q

What is Aniridia?

A

Rare bilateral disorder of iris hypoplasia
Mutation of PAX6 gene
• Range from mild defect of iris stroma to complete absence of iris
• Can be associated with foveal hypoplasia and nystagmus
• Can be associated with WARG syndrome
- Wilms tumour W
- Aniridia A
- Mental Retardation R
- Genitourinary malformations G
• Glaucoma in 75%

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4
Q

What is Aniridia?

A

Rare bilateral disorder of iris hypoplasia
Mutation of PAX6 gene
• Range from mild defect of iris stroma to complete absence of iris
• Can be associated with foveal hypoplasia and nystagmus
• Can be associated with WARG syndrome
- W: Wilms tumour
- A: Aniridia
- R: Mental Retardation
- G: Genitourinary malformations
• Glaucoma in 75%

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5
Q

What is treatment of Aniridia?

A

• Treat the Glaucoma
• Painted contact lens
• Prosthetic iris implant
• Cataract surgery

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6
Q

What is Axenfield-Riegers syndrome?

A

Term used for a group of disorders involving iris and angle malformations;
• Axenfield Anomaly
• Reigers Anomaly
• Reigers Syndrome

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7
Q

What is Axenfield Anomaly?

A

• Posterior embryotoxon (prominent and anteriorly placed shwalbes line)
• Peripheral iris strands attached to cornea

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8
Q

What is Reigers Anomaly?

A

• Posterior embryotoxon
• Iris stromal hypoplasia
• Ectropion uvea liris pigment on anterior iris surface)
• Corectopia (pupil displacement)
• Full thickness iris defects

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9
Q

What is Reigers Syndrome?

A

• Reigers anomaly + systemic features
• Dental abnormalities
• Facial abnormalities

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10
Q

How is Axenfield-Reiger Managed?

A

• Glaucoma develops in 50%
• Surgical interventions for iris abnormalities
• Supportive

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11
Q

What is Coloboma?

A

• Defect resulting from failure of closure of embryologic fissure
• Can affect any layer from disc to eyelids
• Typically inferonasal portion of iris
• Can be associated with CHARGE syndrome

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12
Q

What does CHARGE syndrome stand for?

A

• Coloboma
• Heart defects
• Choanal Atresia
• Retardation of growth
• Genital abnormalities
• Ear abnormalities

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13
Q

What is Uveitis?

A

• Broad range of disorders
• Any inflammation of any part of the uveal tract
• Wide range of causes and associations

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14
Q

What is the classification of Uveitis?

A

• Location: Anterior (iris), Intermediate (ciliary body), Posterior (choroid), Panuveitis (all layers)
• Cause: Infectious (bacterial, viral, parasitic, fungal), Non-infectious (inflammatory), neoplastic or non-neoplastic
• Onset: Acute, Recurrent (periods in between episodes), Chronic (period lasts <3 months), Persistent (episode lasts >3months)

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15
Q

How is the Anterior chamber graded for cells?

A

• Standardisation of Uveitis Nomenclature group (SUN)
• 1mmx1mm slit beam
• X16 magnification
• Bright light and dim room light

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16
Q

What is the Standardisation of Uveitis Nomenclature group (SUN) grading for cells?

A

• Grade 0 : <1 cells in field
• Grade 0.5 : 1-5 cells in field
• Grade 1+ : 6-18 cells in field
• Grade 2+ : 16-25 cells in field
• Grade 3+ : 26-50 cells in field
• Grade 4+ : >50 cells in field

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17
Q

What is the Standardisation of Uveitis Nomenclature group (SUN) grading for flare?

A

• Grade 0 : None
• Grade 1+ : Faint
• Grade 2+ : Moderate (iris lens clear)
• Grade 3+ : Marked (iris lens hazy)
• Grade 4+ : Intense (fibrin or plastic aqueous)

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18
Q

Vitreous grading of haze?

A

• Grade 0: Good view of nerve fibre layer (NFL)
• Grade 1: Clear disc and vessels but hazy NFL
• Grade 2: Disc and vessels hazy
• Grade 3: Only disc visible
• Grade 4: Disc not visible

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19
Q

What are Uveitis symptoms?

A

• Red eye
• Photophobia
• Watering
• Blurred vision
• Onset is usually rapid - within days but can be insidious
• None!

• Systemic symptoms

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20
Q

What are Uveitis Signs?

A

• Ciliary/limbal injection
• Keratic precipitates
• AC Cells
• AC Flare
• Posterior synechiae
• Iris atrophy
• Cataract
• High or Low IOP

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21
Q

What is anterior uveitis?

A

• Commonest form of uveitis is acute anterior uveitis (AAU)
• 50% idiopathic
• Infectious - viral (commonest), TB, syphilis, Lyme disease
• Non-infectious - associated with systemic conditions
• Masquerade - neoplastic or non neoplastic

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22
Q

How is acute anterior uveitis investigated?

A

• Single episode AAU does not require investigation
• Recurrent AAU, bilateral severe AAU or chronic anterior uveitis need investigation
• Uveitis screen
- blood tests
- Urine sample
- chest x-ray
• May consider “AC tap” (aqueous tap)

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23
Q

What are acute anterior uveitis systemic associations?

A

• Spondyloarthropathies
• Sero-negative arthropathies
• Inflammatory bowel disease
• Sarcoidosis
• Behcets disease
• Tubulointerstitial nephritis and uveitis (TINU)
• Multiple sclerosis
• Systemic lupus erythematosis (SLE)
• Juvenile idiopathic arthritis

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24
Q

What is Acute Anterior Uveitis treatment?

A

Treatment in step up technique;
• Topical: atropine, cyclopentolate
• Periocular: Subconj, dexamethasone
• Intraocular: Ozurdex implant
• Systemic: Oral steroids -> Oral second line immunosuppressant

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25
What is Fuchs hetereocromic Cyclitis (FHC)
• Form of uveitis • Chronic non-granulomatous uveitis • Often asymptomatic - low grade grumbling uveitis • 90% unilateral
26
What are some Fuchs hetereocromic Cyclitis (FHC) Signs?
• iris heterochromia (lighter eye is affected eye) • Stellate KPs • Mild flare, occasional cells (0.5+) • Iris atrophy • Iris nodules • Cataract • Raised IOP
27
What is treatment for Fuchs hetereocromic Cyclitis (FHC)?
• Observation • Topical steroids for exacerbations • Topical treatment for Glaucoma - may need laser/surgery • Cataract surgery - higher risk of complications
28
What is Juvenile idiopathic arthritis? (JIA)
• Commonest condition associated with childhood uveitis • Asymptomatic therefore children diagnosed with arthritis as screened • Uveitis may be presenting feature of the disease • So ALL children (<12 yrs old) with uveitis should be referred to the eye clinic
29
What are Juvenile idiopathic arthritis? (JIA) signs?
• Band keratopathy • Uveitis • Posterior synichae • Cataract • Glaucoma
30
What is Juvenile idiopathic arthritis? (JIA) treatment?
• Topical treatments often insufficient • Joint treatment with paediatricians and rheumatology • Require immunosuppression
31
What is Intermediate Uveitis?
• Involves ciliary body • Primary site of inflammation is the vitreous • 50% are idiopathic • Systemic associations - Sarcoidosis - Multiple sclerosis - Behcets - Infectious ALL patents with signs of intermediate uveitis must be referred urgently
32
What are Symptoms of Intermediate Uveitis?
• Blurred vision • Floaters • Pain/redness • Photophobia
33
What are signs of Intermediate Uveitis?
• May have AC cells • High or low IOP • Cataract • Vitreous cells • Snowballs/snowbanking • Cystoid macular oedema
34
What investigations are done for intermediate uveitis?
• Uveitis screen (as for AAU) • Fluorescein angiography • AC or vitreous tap
35
Intermediate Uveitis treatment?
• Topical: Predforte, Maxidex, Cyclopentolate, Atropine • Antibiotic/ Antiviral/ Anti fungal • Intra ocular steroid: Ozurdex • Systemic steroids - 2nd line immuno-supression - 3rd line immuno-supression
36
What are Viral causes of intermediate uveitis?
• Herpes Simplex Virus (HSV) • Varicella zoster virus (VZV) • Cytomegalovirus (CMV) • Epstein Barr virus (EBV) - More common in immunosuppressed patients
37
What is Cytomegalovirus (CMV) retinitis?
• Usually immunosuppressed patients • Most commonly seen in HIV patients when CD4 count is less than 50; opportunistic infection • Treatment is for the eye but also to prevent infection elsewhere • HAART therapy; immune boosting
38
What causes Acute Retinal Necrosis (ARN) and Progressive Outer Retinal Necrosis (PORN) and what is it?
• Herpes simplex virus (HSV) • Virus zoster virus (VSV) Causes:- • Necrosis (dead) of retinal tissue • ARN: Very dense vitritis, starts peripherally and progresses centrally • PORN: Starts centrally and works to periphery
39
What is the diagnostic criteria for ARN? (ALPDVIS)
• Appearance : One or more foci of full-thickness retinal necrosis with discrete borders • Location : Peripheral retina (usually adjacent/outside temporal arcades) • Progression : Rapid (responds to treatment) • Direction : Circumferential • Vessels : Occlusive vasculopathy ( arterial) • Suggestive features : Optic neuropathy/atrophy, scleritis, pain
40
What is the diagnostic criteria for PORN? (ALPDVIS)
• Appearance : Multiple foci of deep retinal opacification, which may be confluent • Location : Peripheral retina, macular involvement • Progression : Extremely rapid • Direction : No consistent direction • Vessels : No Vascular inflammation • Suggestive features : Perivenular clearing of retinal opacification
41
What is ARN/PORN treatment?
• Porn - Intravitreal Foscarnet - IV Ganciclovir - HAART therapy if HIV +ve • ARN - Intravitreal Foscarnet - IV Aciclovir - Oral Valaciclovir - Famciclovir - Systemic corticosteroids - Topical corticosteroids - Barrier laser
42
Intermediate uveitis - Bacterial causes?
• Tuberculosis • Syphillis • Lyme disease • Cat-scratch disease • Brucellosis • Endogenous endophthalmitis; systemic sepsis may spread to eye
43
What types of chorioretinitis?
• Birdshot chorioretinopathy • Punctate inner chorioretinopathy (PIC) • Multifocal choroiditis
44
What is birdshot chorioretinopathy?
• Uncommon idiopathic bilateral disease • Commonly affects middle aged women • HLA-A29 association • Gradual loss of vision • Photopsia • VF loss • Vitritis
45
What is treatment for birdshot chorioretinopathy?
• Commenced on Steroids • HLA-A29 +ve • CT and MRI brain to exclude space occupying lesion • Now on second line immunosupression
46
What is Punctate Inner Chorioretinopathy? (PIC)
• Typically young myopic females • Often preceded by a viral illness • Flickering lights • Blurred vision
47
what is treatment for birdshot chorioretinopathy?
• Systemic steroids • 2nd line immunosuppression • Anti-VEGF for secondary CNV • Aim of treatment is to reduce scarring near disc/fovea and reduce risk of CNV
48
What is Multifocal Choroiditis?
• Uncommon bilateral posterior/panuveitis • AAU in 50% • Vitritis common • Chorioretinal lesions
49
What is a Iris Naevus?
• Iris lesion/tumour • Pigmented, usually flat iris lesion • 15% risk of malignant transformation • Pupil architecture may be affected
50
What are iris nodules and their 3 different types?
• Can be present in inflammatory condiditons and systemic disease • FHC - Busacca and Koeppe nodules • Lisch nodules - Neurofibromatosis • Brushfield spots - Down's syndrome
51
What is Iris melanoma?
• Similar appearance to naevus • Can distort iris architecture and pupil margin • May invade into angle • Will grow gradually
52
What are risk factors for Iris Melanoma?
• Fair skin/iris colour • Iris naevi • Numerous cutaneous naevi • Sunlight exposure
53
What are ciliary body melanomas and their treatment?
• Difficult to diagnose • Often present late with visual symptoms • Risk factors - as for iris melanoma Treatment: •Radiotherapy • Enucleation • Chemotherapy if metastasis (usually liver)
54
What are the different types of choroidal tumours?
• Choroidal Naevus • Choroidal Melanoma
55
What is choroidal Naevus?
• Present in 5-10% of population • Incidental finding • Grow during childhood but not adulthood • Any change is suspicious • Usually flat - up to 2mm is benign • Must monitor life long for conversion
56
What are signs of choroidal Naevus?
• Pigmented retinal lesion with indistinct margins • Over lying drusen • Flat • Depigmented halo • Suspicious signs - Lipofuscin (orange pigment on surface) - Any Subretinal fluid - Symptoms - Within 3 mm of optic disc - >2mm height and 5mm diameter
57
How is choroidal Naevus treated?
• Monitoring with serial photography • 6 monthly initially then annually • If any concerns then refer to HES • Patients referred back to community need lifelong monitoring
58
What is choroidal melanoma?
• Commonest intraocular malignancy but still rare • Usually asymptomatic, incidental finding • Metastasis can occur to liver • Mortality is 50% at 10 yrs
59
What are risk factors and signs of Choroidal Melanoma?
Risk factors as for iris melanoma Suspicious signs • Lipofuscin (orange pigment on surface) • Any SRE • Symptoms • Within 3 mm of optic disc • >2mm height and 5mm diameter
60
What are some signs of a metastasis?
• Solitary elevated (dome shaped) pigmented mass • "collar stud" appearance • Lipofuscin • Localised retinal detachment • Vitiris (advanced) • Sentinal vessels, rubeosis, choroidal folds
61
What are choroidal dystrophies and some types?
• Group of inherited disorders affecting choroidal function • Uncommon • RE, choriocapilaris and photorectors with progressive involvement of large choroidal vessels Some types include: • Choroideremia • Gyrate atrophy • Progressive bifocal chorioretinal atrophy
62
What is choroidermia?
• Progressive, diffuse degeneration of choroid, RPE, and photoreceptors • X-linked recessive - so generally affect males • Very poor prognosis
63
What are symptoms and signs of choroidermia?
• Symptoms - Usually diagnosed in adolescence - Nyctalopia - Reduced peripheral vision - Eventual loss of central vision • Signs - RPE mottling (in female carriers) - Mid peripheral RPE changes, which gradually spread both centrally and peripherally • End-stage - Choroidal vessels run over bare sclera - Retinal arteriolar attenuation - Optic atrophy
64
What is treatment for choroidermia?
• Supportive • Low vision assessment • Blind registration • Gene therapy research ongoing
65
What is Gyrate atrophy?
• Rare Autosomal recessive condition • Enzyme deficiency leading to choroidal atrophy • Unable to break down ornithine • Poor prognosis • Legal blindness by age 50
66
what are Gyrate atrophy signs and symptoms?
• Symptoms - Nyctalopia - Peripheral field loss - Central field loss • Signs - Mid-peripheral de-pigmented spots - Well-defined peripheral atrophic patches with scalloped edge - Atrophic lesions eventually coalesce - Fovea lost in later stages - May develop ERM or CMO
67
What is treatment for Gyrate atrophy?
• Arginine restricted diet - reduced ornithine levels and can slow disease • Pyridoxine supplements (Vitamin B6) • Gene therapy research ongoing
68
What are some clinical take home messages to remember regarding uveitis?
• Congenital conditions usually associated with syndromes • Almost ALL uveitis warrants urgent referral to HES • Any intermediate uveitis should be referred to casualty/acute referral clinics • ALWAYS DILATE PATIENTS AND CHECK RETINAL PERIPHERY • Suspicious naevi/choroidal mass lesions should be referred urgently • Dystrophies should be referred routinely for counselling, genetic testing and partial sight/severe sight impairment