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(2) Tri A Clinical opthalmology > Anterior eye 4 - Uvea > Flashcards

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
Q

What is Fuchs hetereocromic Cyclitis (FHC)

A

• Form of uveitis
• Chronic non-granulomatous uveitis
• Often asymptomatic - low grade grumbling uveitis
• 90% unilateral

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26
Q

What are some Fuchs hetereocromic Cyclitis (FHC) Signs?

A

• iris heterochromia (lighter eye is affected eye)
• Stellate KPs
• Mild flare, occasional cells (0.5+)
• Iris atrophy
• Iris nodules
• Cataract
• Raised IOP

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27
Q

What is treatment for Fuchs hetereocromic Cyclitis (FHC)?

A

• Observation
• Topical steroids for exacerbations
• Topical treatment for Glaucoma - may need laser/surgery
• Cataract surgery - higher risk of complications

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28
Q

What is Juvenile idiopathic arthritis? (JIA)

A

• 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
Q

What are Juvenile idiopathic arthritis? (JIA) signs?

A

• Band keratopathy
• Uveitis
• Posterior synichae
• Cataract
• Glaucoma

30
Q

What is Juvenile idiopathic arthritis? (JIA) treatment?

A

• Topical treatments often insufficient
• Joint treatment with paediatricians and rheumatology
• Require immunosuppression

31
Q

What is Intermediate Uveitis?

A

• 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
Q

What are Symptoms of Intermediate Uveitis?

A

• Blurred vision
• Floaters
• Pain/redness
• Photophobia

33
Q

What are signs of Intermediate Uveitis?

A

• May have AC cells
• High or low IOP
• Cataract
• Vitreous cells
• Snowballs/snowbanking
• Cystoid macular oedema

34
Q

What investigations are done for intermediate uveitis?

A

• Uveitis screen (as for AAU)
• Fluorescein angiography
• AC or vitreous tap

35
Q

Intermediate Uveitis treatment?

A

• Topical: Predforte, Maxidex, Cyclopentolate, Atropine
• Antibiotic/ Antiviral/ Anti fungal
• Intra ocular steroid: Ozurdex
• Systemic steroids
- 2nd line immuno-supression
- 3rd line immuno-supression

36
Q

What are Viral causes of intermediate uveitis?

A

• Herpes Simplex Virus (HSV)
• Varicella zoster virus (VZV)
• Cytomegalovirus (CMV)
• Epstein Barr virus (EBV)

  • More common in immunosuppressed patients
37
Q

What is Cytomegalovirus (CMV) retinitis?

A

• 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
Q

What causes Acute Retinal Necrosis (ARN) and Progressive Outer Retinal Necrosis (PORN) and what is it?

A

• 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
Q

What is the diagnostic criteria for ARN? (ALPDVIS)

A

• 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
Q

What is the diagnostic criteria for PORN? (ALPDVIS)

A

• 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
Q

What is ARN/PORN treatment?

A

• 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
Q

Intermediate uveitis - Bacterial causes?

A

• Tuberculosis
• Syphillis
• Lyme disease
• Cat-scratch disease
• Brucellosis
• Endogenous endophthalmitis; systemic sepsis may spread to eye

43
Q

What types of chorioretinitis?

A

• Birdshot chorioretinopathy
• Punctate inner chorioretinopathy (PIC)
• Multifocal choroiditis

44
Q

What is birdshot chorioretinopathy?

A

• Uncommon idiopathic bilateral disease
• Commonly affects middle aged women
• HLA-A29 association
• Gradual loss of vision
• Photopsia
• VF loss
• Vitritis

45
Q

What is treatment for birdshot chorioretinopathy?

A

• Commenced on Steroids
• HLA-A29 +ve
• CT and MRI brain to exclude space occupying lesion
• Now on second line immunosupression

46
Q

What is Punctate Inner Chorioretinopathy? (PIC)

A

• Typically young myopic females
• Often preceded by a viral illness
• Flickering lights
• Blurred vision

47
Q

what is treatment for birdshot chorioretinopathy?

A

• 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
Q

What is Multifocal Choroiditis?

A

• Uncommon bilateral posterior/panuveitis
• AAU in 50%
• Vitritis common
• Chorioretinal lesions

49
Q

What is a Iris Naevus?

A

• Iris lesion/tumour
• Pigmented, usually flat iris lesion
• 15% risk of malignant transformation
• Pupil architecture may be affected

50
Q

What are iris nodules and their 3 different types?

A

• Can be present in inflammatory condiditons and systemic disease
• FHC - Busacca and Koeppe nodules
• Lisch nodules - Neurofibromatosis
• Brushfield spots - Down’s syndrome

51
Q

What is Iris melanoma?

A

• Similar appearance to naevus
• Can distort iris architecture and pupil margin
• May invade into angle
• Will grow gradually

52
Q

What are risk factors for Iris Melanoma?

A

• Fair skin/iris colour
• Iris naevi
• Numerous cutaneous naevi
• Sunlight exposure

53
Q

What are ciliary body melanomas and their treatment?

A

• Difficult to diagnose
• Often present late with visual symptoms
• Risk factors - as for iris melanoma
Treatment:
•Radiotherapy
• Enucleation
• Chemotherapy if metastasis (usually liver)

54
Q

What are the different types of choroidal tumours?

A

• Choroidal Naevus
• Choroidal Melanoma

55
Q

What is choroidal Naevus?

A

• 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
Q

What are signs of choroidal Naevus?

A

• 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
Q

How is choroidal Naevus treated?

A

• 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
Q

What is choroidal melanoma?

A

• Commonest intraocular malignancy but still rare
• Usually asymptomatic, incidental finding
• Metastasis can occur to liver
• Mortality is 50% at 10 yrs

59
Q

What are risk factors and signs of Choroidal Melanoma?

A

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
Q

What are some signs of a metastasis?

A

• Solitary elevated (dome shaped) pigmented mass
• “collar stud” appearance
• Lipofuscin
• Localised retinal detachment
• Vitiris (advanced)
• Sentinal vessels, rubeosis, choroidal folds

61
Q

What are choroidal dystrophies and some types?

A

• 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
Q

What is choroidermia?

A

• Progressive, diffuse degeneration of choroid, RPE, and photoreceptors
• X-linked recessive - so generally affect males
• Very poor prognosis

63
Q

What are symptoms and signs of choroidermia?

A

• 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
Q

What is treatment for choroidermia?

A

• Supportive
• Low vision assessment
• Blind registration
• Gene therapy research ongoing

65
Q

What is Gyrate atrophy?

A

• Rare Autosomal recessive condition
• Enzyme deficiency leading to choroidal atrophy
• Unable to break down ornithine
• Poor prognosis
• Legal blindness by age 50

66
Q

what are Gyrate atrophy signs and symptoms?

A

• 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
Q

What is treatment for Gyrate atrophy?

A

• Arginine restricted diet - reduced ornithine levels and can slow disease
• Pyridoxine supplements (Vitamin B6)
• Gene therapy research ongoing

68
Q

What are some clinical take home messages to remember regarding uveitis?

A

• 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

(2) Tri A Clinical opthalmology (19 decks)

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