Anterior eye 4 - Uvea Flashcards
What does the uvea consist of?
• 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
What are congenital abnormalities of the uvea?
• Aniridia
• Axenfield-Reiger syndrome
• Coloboma
What is Aniridia?
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%
What is Aniridia?
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%
What is treatment of Aniridia?
• Treat the Glaucoma
• Painted contact lens
• Prosthetic iris implant
• Cataract surgery
What is Axenfield-Riegers syndrome?
Term used for a group of disorders involving iris and angle malformations;
• Axenfield Anomaly
• Reigers Anomaly
• Reigers Syndrome
What is Axenfield Anomaly?
• Posterior embryotoxon (prominent and anteriorly placed shwalbes line)
• Peripheral iris strands attached to cornea
What is Reigers Anomaly?
• Posterior embryotoxon
• Iris stromal hypoplasia
• Ectropion uvea liris pigment on anterior iris surface)
• Corectopia (pupil displacement)
• Full thickness iris defects
What is Reigers Syndrome?
• Reigers anomaly + systemic features
• Dental abnormalities
• Facial abnormalities
How is Axenfield-Reiger Managed?
• Glaucoma develops in 50%
• Surgical interventions for iris abnormalities
• Supportive
What is Coloboma?
• 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
What does CHARGE syndrome stand for?
• Coloboma
• Heart defects
• Choanal Atresia
• Retardation of growth
• Genital abnormalities
• Ear abnormalities
What is Uveitis?
• Broad range of disorders
• Any inflammation of any part of the uveal tract
• Wide range of causes and associations
What is the classification of Uveitis?
• 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)
How is the Anterior chamber graded for cells?
• Standardisation of Uveitis Nomenclature group (SUN)
• 1mmx1mm slit beam
• X16 magnification
• Bright light and dim room light
What is the Standardisation of Uveitis Nomenclature group (SUN) grading for cells?
• 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
What is the Standardisation of Uveitis Nomenclature group (SUN) grading for flare?
• 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)
Vitreous grading of haze?
• 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
What are Uveitis symptoms?
• Red eye
• Photophobia
• Watering
• Blurred vision
• Onset is usually rapid - within days but can be insidious
• None!
• Systemic symptoms
What are Uveitis Signs?
• Ciliary/limbal injection
• Keratic precipitates
• AC Cells
• AC Flare
• Posterior synechiae
• Iris atrophy
• Cataract
• High or Low IOP
What is anterior uveitis?
• 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
How is acute anterior uveitis investigated?
• 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)
What are acute anterior uveitis systemic associations?
• Spondyloarthropathies
• Sero-negative arthropathies
• Inflammatory bowel disease
• Sarcoidosis
• Behcets disease
• Tubulointerstitial nephritis and uveitis (TINU)
• Multiple sclerosis
• Systemic lupus erythematosis (SLE)
• Juvenile idiopathic arthritis
What is Acute Anterior Uveitis treatment?
Treatment in step up technique;
• Topical: atropine, cyclopentolate
• Periocular: Subconj, dexamethasone
• Intraocular: Ozurdex implant
• Systemic: Oral steroids -> Oral second line immunosuppressant
What is Fuchs hetereocromic Cyclitis (FHC)
• Form of uveitis
• Chronic non-granulomatous uveitis
• Often asymptomatic - low grade grumbling uveitis
• 90% unilateral
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
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
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
What are Juvenile idiopathic arthritis? (JIA) signs?
• Band keratopathy
• Uveitis
• Posterior synichae
• Cataract
• Glaucoma
What is Juvenile idiopathic arthritis? (JIA) treatment?
• Topical treatments often insufficient
• Joint treatment with paediatricians and rheumatology
• Require immunosuppression
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
What are Symptoms of Intermediate Uveitis?
• Blurred vision
• Floaters
• Pain/redness
• Photophobia
What are signs of Intermediate Uveitis?
• May have AC cells
• High or low IOP
• Cataract
• Vitreous cells
• Snowballs/snowbanking
• Cystoid macular oedema
What investigations are done for intermediate uveitis?
• Uveitis screen (as for AAU)
• Fluorescein angiography
• AC or vitreous tap
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
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
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
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
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
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
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
Intermediate uveitis - Bacterial causes?
• Tuberculosis
• Syphillis
• Lyme disease
• Cat-scratch disease
• Brucellosis
• Endogenous endophthalmitis; systemic sepsis may spread to eye
What types of chorioretinitis?
• Birdshot chorioretinopathy
• Punctate inner chorioretinopathy (PIC)
• Multifocal choroiditis
What is birdshot chorioretinopathy?
• Uncommon idiopathic bilateral disease
• Commonly affects middle aged women
• HLA-A29 association
• Gradual loss of vision
• Photopsia
• VF loss
• Vitritis
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
What is Punctate Inner Chorioretinopathy? (PIC)
• Typically young myopic females
• Often preceded by a viral illness
• Flickering lights
• Blurred vision
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
What is Multifocal Choroiditis?
• Uncommon bilateral posterior/panuveitis
• AAU in 50%
• Vitritis common
• Chorioretinal lesions
What is a Iris Naevus?
• Iris lesion/tumour
• Pigmented, usually flat iris lesion
• 15% risk of malignant transformation
• Pupil architecture may be affected
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
What is Iris melanoma?
• Similar appearance to naevus
• Can distort iris architecture and pupil margin
• May invade into angle
• Will grow gradually
What are risk factors for Iris Melanoma?
• Fair skin/iris colour
• Iris naevi
• Numerous cutaneous naevi
• Sunlight exposure
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)
What are the different types of choroidal tumours?
• Choroidal Naevus
• Choroidal Melanoma
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
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
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
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
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
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
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
What is choroidermia?
• Progressive, diffuse degeneration of choroid, RPE, and photoreceptors
• X-linked recessive - so generally affect males
• Very poor prognosis
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
What is treatment for choroidermia?
• Supportive
• Low vision assessment
• Blind registration
• Gene therapy research ongoing
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
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
What is treatment for Gyrate atrophy?
• Arginine restricted diet - reduced ornithine levels and can slow disease
• Pyridoxine supplements (Vitamin B6)
• Gene therapy research ongoing
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
