1
Q
What are some ways to classify uveitis
A
Anterior/intermediate (pars planitis)/ posterior (retina/choroid)/ panuveitis.
Infectious/ non infectious +/- systemic association/ masquerade (neoplstic/ non neoplastic), onset (sudden/ insidious)/ duration (3 months or less, persistent)
2
Q
Which interleukin is proposed to drive uveitis
A
IL-6 released by monocytes and macrophages in response to inflammatory cytokines
3
Q
What are some systemic associations of anterior uveitis (infectious/ non infectious/ masquerade)
A
Infectious- VZV/ TB/ Syphilis/ Lyme/ Misc viral infections
Non infectious- HLAB27 positive 20% of those with AAU/ JIA/ Sarcoidosis/ Behcet/TINU/ SLE/ MS/ Drug induced
Masquerade- Neoplastic (lymphoma/ anterior segment melanoma), Non neoplastic (juvenile xanthogranuloma)
4
Q
What is the difference between normal hypopyon and Behcet disease hypopyon
A
normal- high fibrin content make it immobile and slow to absorb
Behect- minimal fibrin so shifts according to head position
5
Q
What is the difference between Koeppe and Busaca nodules. Also what are roseolae, iris pearls, iris crystals (russell bodies) and which conditions are they associated with
A
Busacca- involve iris stroma feature of granulomatous uveitis
Koeppe- pupil margin located, site of PS formation.
Yellowish nodules develop from dilated iris vessels (roseolae) in syphilitc uveitis.
Iris pearls in Lepromatous CAU,
Iris crystals (Russell bodies)- immunoglobulin deposits in CAU/ Fuchs uveitis syndrome
6
Q
What is the difference in the way normal iris vessels present vs rubeosis
A
Normal vessels course radially in contrast to irregular distribution of neovascularization
7
Q
What is the SUN grading for AC cells
A
0 <1 cell
0.5+ 1-5 cells
1+ 6-15 cells
2+ 16-25 cells
3+ 26-50 cells
4+ >50 cells
8
Q
What is the SUN grading for AC flare
A
0 none
1+ faint
2+ moderate (iris and lens details clear)
3+ marked (iris lens details hazy)
4+ intense (fibrin or plastic aqueous)
9
Q
What does posterior uveitis encompass
A
clinical entities of retinitis, choroiditis, retinal vasculitis
10
Q
In what circumstances is systemic investigation of anterior uveitis appropriate
A
recurrent AAU
Severe AAU
Bilateral AAU
AAU persistent/chronic/ resistant to treatment
Granulomatous inflammatory signs
Associated intermediate/posterior uveitis
Ocular/systemic features suggesting underlying disease
11
Q
Which chromosome is the MHC found on
A
6- HLA system
12
Q
What are the systemic associations of HLAB27
A
Ankylosing spondylitis/ reactive arthritis/ psoriatic arthritis/ arthritis in IBD
13
Q
What are some investigations for uveitis
A
HLAB27/ Syphilis serology/ ACE/Lysozyme/ ESR/CRP/FBC/Lyme disease/ANA/ANCA/Quantiferon TB/ HIV/Sacroiliac joint xray/ CXR/ FAF/ OCT/FA/ICG/ UMB/Aqueous tap/Iris biopsy/ Vitreous biopsy/Chorioretinal biopsy/Conjunctival biopsy
14
Q
What are the 2 serology tests for syphilis
A
RPR and VDRL- commonly positive in early infection, used to monitor disease activity, tests become negative over time.
15
Q
What is ACE elevated in
A
granulomatous disease (sarcoid/TB/leprosy)
16
Q
Why is FAF useful as a uveitis investigation and what conditions is it effective in
A
detect lipofuscin and melanin in RPE cells. Provides information on RPE function and integrity of chorioretinal interface. Helpful in assessing inflammatory conditions of the choriocapillaris.
Pronounced AF in MEWDS, serpiginous choroidopathy, AZOOR
17
Q
What conditions show inner retinal infiltrates with hyperreflective foci on OCT
A
Behcet
viral infections
18
Q
What conditions show outer retinal infiltrates with hyperreflective foci on OCT
A
PIC/ MEWDS, APMPPE/ Serpignious choroidopathy/ AZOOR/ AMN
19
Q
What conditions is FFA useful for
A
retinal vasculitis/ occlusive vasculopathy/ optic disc swelling and SRF accummulation/ inflammatory retinal foci showing early hypofluorescence and late hyperfluorescence.
20
Q
What are the duration of action of various dilating agents
A
cyclopentolate 12-24 hours
homatropine 3 days
atropine 7-10 days
21
Q
What are the various treatments for anterior uveitis
A
topical steroids/ cycloplegic agents/ mydricaine no.2 (1 for children)/ TPA/Subconjunctival steroid/ regional steroid injection/intraocular steroids/ systemic steroids/NSAID’s/Antimetabolites
22
Q
What should be done before starting treatment with immunomodulatory medications for non infectious uveitis
A
careful informed consent
discuss potential side effects
active infections especially TB ruled out. Check haem/renal/hepatic function
23
Q
What are the indications of IMMEDIATE treatment with immunomodulatory medications for non infectious uveitis
A
SLE + Retinal vasculitis
Behcet disease + retinal vasculitis
24
Q
What are the indications of EARLY treatment with immunomodulatory medications for non infectious uveitis
A
SO
VKH
Birdshot retinochoroidopathy
Serpiginuous choroidopathy
Multifocal choroiditis and panuveitis
JIA
25
What are the indications of 'ALTERNATIVE TO STEROID' treatment with immunomodulatory medications for non infectious uveitis
Therapeutic- inadequate response to topical /periocular steroids or to an oral dose >0.5mg/kg/day prednisolone/ steroid sparing measure
Ocular acute disease which is sight threatening or chronic and persistent/ exudative RD/ macula involving disease
26
What is a significant risk of using azathioprine/ Anti TNF inhibitor as monotherapy
small but statistically significant risk of lymphoma
27
What are some examples of antimetabolites
MTX
MMF
Azathioprine
All inhibit purine metabolism
28
What are some examples of calcineurin inhibitors (T cells targeted inhibition)
Cyclosporin A
Tacrolimus
Voclosporin
Sirolimus
29
What are some examples of Alkylating agents
Cyclophosphamide
Chlorambucil
30
What are some examples of TNF alpha inhibitors
Infliximab
Adalimumab
Etanercept
Golimumab
Certolizumab
31
What are some examples of IL6 inhibitors
Tocilizumab inhibits IL6 receptors inhibiting pro inflammatory cytokines
32
How long do biopharmacological (mabs) take to work
2 weeks to 3 months
33
What combination of immunosuppression is good for all forms of severe uveitis
steroids and antimetabolites
34
What type of treatment is good for T cell critical uveits and what conditions are these
Calcineurin inhibitors
VKH/Birdshot
35
What type of treatment is good for neutrophil and macrophage critical uveitis and what conditions are these
anti TNF agents
Behcet/Sarcoidosis
36
What type of treatment is good for B cell critical inflammation and what conditions are these
B cell inhibition eg Rituximab
Scleritis particularly ANCA+ related
37
What is the triad of Reactive arthritis and what organisms cause it
arthritis
urethritis
conjunctivitis
shigella
campylobacter
salmonella
chlamydia (commonest cause)
38
What are some systemic features of psoriatic arthritis
multiple types. Plaque type well demarcated raised silvery inflamed plaques on scalp, trunk, arms, legs. Nail changes are pitting/ oncholysis/transverse depression/ arthritis asymmetrical involves DIPs (sausage digits)/ enthesitis
39
What is Fuchs uveitis/ Fuchs Heterochromic iridocyclitis
chronic non granulomatous condition diagnosed at 40. no gender/ age predilection. Evidence implicates rubella virus. Signs in toxoplasmosis similar. Thought most of AC activity due to blood aqueous barrier breakdown rather than inflammation
40
What are the clinical features of Fuchs uveitis syndrome
unilateral (90-95%) cases
gradual blurring due to cataract as a common presentation .
Heterochromia iridis- daylight. Affected eye hypochromic. In blue eyes the posterior dark pigment layer shows through causing the hyperchromic eye to be affected.
Posterior synechiae absent/ AC faint flare/ KP's stellate diffuse over entire cornea/ iris nodules and russell bodies/iris atrophy/ iris vessels/ vitritis/PSC cataract/ glaucoma 60% eyes
41
What are the causes of hypochromic heterochromia iridis
idiopathic congenital
Horner syndrome particularly if congenital
42
What are the causes of hyperchromic heterochromia iridis
unilateral use of topical prostaglandin analogue for glaucoma/ oculodermal melanocytosis (Naevus of Ota)/ Ocular siderosis/diffuse iris narvus/ melanoma/ Sturge Weber syndrome
43
What are the causes of hypo or hyperchromic heterochromia iridis
Fuchs uveitis syndrome
Other chronic anterior uveitides
44
How is Fuchs uveitis treated
Long term monitoring for glaucoma
Topical steroids/ cataract surgery/glaucoma usually needing GDD/ PPV for visually problematic vitreous opacification
45
What are the clinical features of JIA
classified according to extent of joint involvement during first 6 months
oligoarticular- commonest 4 or fewer joints, knees commonly, then ankles and wrists. 75% ANA positive.
Polyarticular (RF -ve) - 5 or more joints affected. Female male 3:1 rato.
Polyarticular (RF +ve) 5 or more joints affected- low risk of uveitis
Systemic- stills disease
Anterior uveitis- even with +4 cells eye can be white and asymptomatic. Sometimes late signs are band keratopathy/ strabismus
46
How is JIA investigated
Paediatric rheumatologist referral
ANA positivity/ HLAB27/ RF
47
What is the screening timeframe for kids with JIA
Initial exam within 6 weeks of first diagnosis. Visual signs like band keratopathy/ synechiae/ cataract lead to urgent ophthalmology referral. Initial 2 monthly exams for 6 months for all newly diagnosed oligoarticular/ psoriatic/polyarticular/ enthesitis patients regardless of ANA status, followed by 3-4 monthly intervals
48
How is JIA treated
Refer to uveitis specialist.
Topical steroids/mydriatics/periocular steroids/oral steorids/ NSAID's/ systemic immunosuppressive agents eg MTX/ infliximab/ adalimumab improves overall visual prognosis
Tocilizumab- recently shown to be effective in refractory uveitis
Secondary glaucma- IOP surgery. Trab + MMC if using anti TNF medication. Baerveldt tube good long term iop control 5% risk of hypotony. Cataract extraction often needed
49
Why is etanercept avoided in JIA
result in severe worsening of inflammation.
50
What are the systemic and ocular features of ulcerative colitis
bloody diarrhoea/ lower abdominal cramps/ urgency/ tenesmus/ tiredness/ weight loss/malaise. Cutaneous lesions oral apthous ulcers/ erythema nodosum/ pyoderma gangrenosum/ arthritis asymmetrical large joints/ sacroilitis/AS in HLAB27 +/ hepatic disease in autoimmune disease/sclerosing cholangitis/ cholangiocarcinoma.
Ocular: AAU/Conjunctivitis/ episcleritis/scleritis
51
What gene is associated with Crohn's disease
CARD 15
52
What are some systemic and ocular features of Crohns disease
abdominal pain/ diarrhoea/weight loss/ fever/ vomiting/ oral apthous ulcers/ cutaneous lesions erythema nodosum/ pyoderma gangrenosum/ anaemia/hypeatic diseas/ skeletal features clubbing/ arthritis/ sacroilitis.
Ocular: AAU/ episcleritis/ scleritis/follicular conjunctivitis/ myositis/ optic neuritis
53
What is Whipples disease
intestinal lipodystrophy- chronic GI inflammatory condition caused by infection with Tropheryma whipplei. (Duodenal biopsy) needed for diagnosis can be cured with antibiotics
54
What are the systemic and ocular features of Whipple disease
IBD with malabsorption. Joint, cardiac, CNS involvement.
Ocular: keratitis, AAU, Vitritis, retinitis + retinal haemorrhages/ cotton wool spots/ vascular occlusion/ multifocal choroiditis.
Neuroophthalmic manifestations eg gaze palsy/ nystagmus, ophthalmoplegia, papilloedema, optic atrophy
55
What is TINU and what are its systemic and ocular features
uncommon disorder of immune origin with acute Tubuolointerstital nephritis and uveitis. Occurs in adolescent girls. Renal disease precedes uveitis.
Systemically: proteinuria/ anaemia/ HTN/ renal failure. Good response to systemic steroids/ renal function returns to normal thereafter.
Bilateral non granulomatous anterior uveitis responding well to steroids. Disc and macula oedema may occur. intermediate/ posterior/panuveitis may occur
56
What are the differentials in a patient with ocular and renal inflammation
sarcoidosis
TB
SLE
TINU
IgA nephropathy
57
What is intermediate uveitis and what entities does it encompass
chronic relapsing disease of insidious onset in which vitreous is primary site of inflammation.
Incorporates
pars planitis/ posterior cyclitis/ hyalitis.
58
What is pars planitis
term used for a subset of IU in which there is snowbanking and or snowball formation but only if the inflammation is idiopathic that is no underlying systemic disorder/ infection.
59
What are the symptoms and signs of intermediate uveitis
insidious onset blurry vision/ vitreous floaters/no pain or redness/ va varies depending on inflammatory activity eg CMO/Anterior uveitis/ vitreous cells with anterior predominance/ vitreous condensation and haze in severe cases/ vitreous snowballs (whitish focal collection of inflammatory cells/ exudates)/ peripheral periphlebitis in MS. Examine fellow eye may show mild vascular sheathing/ snowbanking (grey white fibrovascular and or exudative plaque in all quadrants mainly inferiorly)/ optic disc swelling/ CMO/ macula ERM/Cataract/ glaucoma/ retinal detachment
60
How to investigate intermediate ueitis
inflammatory markers- ESR/CRP/FBC/OCT/FA
61
What conditions are associated with IU
MS- IU may precede other symptoms of demyelination
Sarcoidosis
Lyme associated IU
Syphilis - treponemal/ cardiolipin testing
TB
FUD/ Intraocular lymphoma/ whipple disease/endogenous candida endophthalmitis /toxoplasmosis
62
What are the treatments for intermediate uveitis
Topical steroids do not reach posterior segment so limited role. In mild IU- can help, Regional steroid injection (orbital floor/posterior subtenon/ NSAID PO/ Cryotherapy to pars plana and retinal periphery if inflammation steroid resistant/ peripheral retinal laser adjacent to snowbanking and ischaemic areas on FFA/ intraocular steroid/ systemic steroid/ immunosuppressive agents (MMF/ MTX/ tacrolimus/ cyclosporin)/ interferon beta (MS related IU) and infliximab/ PPV reduce inflammatory intensity / Cataract and glaucoma managed accordingly
63
What is VKH and what racial background people does it affect
idiopathic myultisystem autoimmune disease featuring inflammation of melanocyte containing tissues eg uvea, ear and meninges
Hispanics/ Japanese and heavily pigmented individuals
64
What are the HLA associations of VKH
HLADR1, HLA DR4
65
What are the 2 divisions of VKH
Vogt Koyanagi disease: skin changes/ AAU
Harada: neurological features/ exudative RD predominates
66
What are the clinical features of VKH
Prodromal phase- neurological (meningitis/ rarely encephalopathy with CN palsies/ other focal lesions/ auditory manifestations), Acute uveitis phase (bilateral granulmatous anterior/multifocal posterior uveitis with diffiuse choroidal infiltrates, Dalen Fuchs nodules, vitritis, papillitis, exudative RD's, ciliary effusion with iris-lens diaphgram rotation
Convalescent phase- several weeks later with alopecia/ poliosis/ vtiligo/ depigmented fundus, depigmented limbal lesions (sugiura sign)
Chronic recurrent phase- smouldering anterior uveitis with exacerbations.
67
What are the ocular complications of VKH
MNV
Subretinal fibrosis
preretinal / NVD/ VH/ cataract/ glaucoma
68
What are the investigations for VKH
LP- CSF shows transient lymphocytic pleocytosis and melanin containing macrophages
FAF- HyperAF in areas of serous RD's
Ultrasonography- diffuse choroidal thickening and excludes posterior scleritis
FA in acute phase shows multifocal hyperfluorescent dots at level of RPE followed by subretinal pooling. Chronic phase shows RPE window defects. OCT allows quantification of SRF. ICGA in acute phase shows regularly distributed hypofluorescent spots most of which remain hypofluorescent in later phase. ICG good for monitoring
69
What is the diagnostic criteria for VKH
1- absence of history of penetrating ocular trauma
2- absence of other ocular disease entities
3- Bilateral uveitis
4- neurological + auditory manifestations
5- integumentary findings not preceding onset of CNS/ ocular disease eg alopecia/ poliosis/ vtiligo
Complete VKH- 1-5 present, incomplete 1-3 and either 4 or 5, probably VKH criteria 1-3 must be present
70
What is the treatment for VKH
High dose oral prednisolone 1-2 mg/kg/day- may be preceded by IVMP. Topical steroids/ cycloplegics. Biological blockers eg infliximab used early in disease course if no steroid response
71
What is sympathetic ophthalmia and when can it present post trauma
bilateral granulomatous panuveitis occurring after penetrating trauma where uveal prolapse is a feature. Less commonly can occur after multiple VR procedures.
May present 2 weeks to 3 months after initial injury in 65%.
72
What are some clinical features of sympathetic ophthalmia
history of causative trauma. excited eye red and irritable. Sympathizing eye irritated/ blurry vision/ photophobia/ loss of accommodation. Anterior uveitis in both eyes severe and granulomatous. Fundus shows multifocal choroidal infiltrates in mid periphery with sub RPE infiltrates corresponding to Dalen Fuch nodules. Exudative RD/ Vasculitis/ Optic disc swelling may all manifest. Once chorioretinal scarring occurs leads to depigmented fundus similar to VKH.
73
What does histopathology show in SO and what is the prognosis of Sympathetic ophthalmia
with aggressive therapy 75% of sympathizing eyes retain VA better than 6/60.
Histopathology- diffuse lymphocytic infiltration of choroid. Scattered aggregates of epitheloid cells seen may containing fine granules of melanin
74
What are the investigations for Sympathetic ophthalmia
OCT- quantifies and monitoring change
B scan- choroidal thickening
FA- multiple foci of leakage at RPE level with subretinal pooling in presence of exudative RD
ICGA- hypofluorescence spots in active disease resolve with treatment
FAF- monitor disease progression
Ultrasound- choroidal thickening and exudative RD in acute stage
75
What is the treatment for sympathetic ophthalmia
enucleation of severely injured eye within 10-14 days of the injury historically effective in preventing/ reducing severity of SO. Evisceration can be done provided all uveal tissue removed.
Steroids- high dose oral prednisolone for several months tapered according to response. Supplementary periocular/ topical/intravitreal /cycloplegis.
immunomodulatory treatment considered early in disease course
76
What are the features of lens induced uveitis
variable pain/photophobia/ redness/ blurry vision/ history of recent complicated/ uncomplicated cataract surgery/ uncommonly injury. Anterior uveitis mild/moderate/severe. Corneal oedema adjacent to AC lens fragment, IOP elevated, lens fragments in anterior/ posterior segment. Vitritis if lens fragments in vitreous cavity. Lens injury in cases of trauma. Complications are CMO/ Glaucoma/ ERM/ RD/ cyclitic membrane
77
What is the treatment for lens induced uveitis
steroids
surgical removal of all lens material from AC/ PPV. Small lens fragments in Posterior segment managed conservatively- will absorb over months. Cycloplegia/ IOP lowering treatment good.
78
What is sarcoidosis
chronic disorder of unknown cause manifesting with non caeseating granulomas inflammatory foci. Affect any organ system but lungs/ lymph nodes commonly involved. Affects Black people more than white 10:1. More common in colder climates. Most common systemic associations of uveitis
79
What are the systemic features of sarcoidosis
respiratory symptoms- cough/ SOB/ Malaise/ arthralgia.
lung disease ranges from mild parenchymal infiltration to severe pulmonary fibrosis.
skin lesions erythema nodosum, lupus pernio, granulomatous papules/ macules, neurological disease, cardiac involvement- arythmias and death, lymphadenopathy.
80
What is the triad of Lofgren syndrome
erythema nodosum
bilateral hilar lymphadenopathy
polyarthralgia
81
What are the ocular features of sarcoidosis
mutton fat KP's and or iris nodules Koeppe and Busacca
TM nodules and or tent shaped PAS
Vitreous snowballs/ string of pearls
Multiple chorioretinal peripheral lesions. Nodular and or segmental periphlebitis (+/- candle wax drippings) and or retinal macroaneurysms in inflamed eye. Optic disc nodule/ granuloma and or solitary choroidal nodule. Perivenous exudates referred to as taches de bougie (severe periphlebitis), peripheral retinal nve.
82
How to investigate suspected Sarcoidosis
IWOS criteria are to do with these 5 tests.
Negative TB test tuberculin skin test. Elevated ACE/ serum lysozyme. CXR showing BHL. Abnormal liver enzyme tests. Chest CT in negative CXR. HRCT better value.
Other tests: Fibreoptic bronchoscopy, thoracic endosonography, lymph node biopsy, calcium/ vitamin D levels, pulmonary function testing. BALF shows CD4/ CD8 T cell ratios.
83
What are the 4 diagnostic levels according to IWOS for diagnosis of sarcoidosis
Definite ocular sarcoid: biopsy supported diagnosis + compatible uveitis
Presumed ocular sarcoid: no biopsy but CXR showing BHL + compatible uveitis
Probably ocular sarcoid: no biopsy, no BHL but >3/7 intraocular signs and >2/5 positive lab tests
Possible ocular sarcoid: lung biopsy negative but >4/7 signs and >2/5 positive lab tests
84
What is the treatment for sarcoidosis
Treat anterior/intermediate uveitis stepwise fashion as idiopathic inflammation
Posterior uveitis (systemic steroids/ immunosuppressive agents TNF inhibitors eg adalimumab), peripheral retinal NVE -sectoral PRP FFA guided. CMO with topical NSAID
Treat cataract and glaucoma as necessary
85
What is Behcet disease
Idiopathic multisystem syndrome with recurrent aphthous oral ulcers/ genital ulceration, uveitis. Vasculitis key pathognomonic component. May involve small/ medium and large veins/ arteries. Mortality occurs due to cardiovascular and CNS complications.
Typically affects people from Turkey/ Middle and Far East (ancient silk road route)
86
What is the HLA association of Behcet disease
HLA-B51
87
What is the ISGBD criteria for diagnosis of Behcet disease
recurrent oral ulceration characterized by oral ulcers at least 3x in 12 month period + any two of
1) Genital ulceration
2) ocular inflammation
3) characteristic skin lesions (erythema nodosum, pseudofolliclulitis, acneiform nodules)
4) pathergy reaction (pustule 24-48 hours after needle prick)
88
What are the ocular features of Behcet disease
AAU (bilateral non granulomatous)- transient mobile hypopyon in relatively white eye
Vitritis- severe/ universal in eyes with posterior segment disease
Retinitis- transient superficial white infiltrates healing without scarring. Exudative RD can occur/ Inflammatory deposits like KP's may be seen on inferior peripheral retina
Retinal vasculitis- arteritis/ phlebitis in contrast to pure venous involvement in sarcoidosis. Also retinal oedema/ CMO. Optic disc hyperaemia/ oedema. Disc and retinal NV
89
What are the features of end stage Behcet disease
optic atrophy
retinal atrophy
gliosis and sheathing
attenuation + ghosting of affected vessels
PS/cataract/ glaucoma/ RD/ phthisis
90
What are the investigations for Behcet disease
HLA B51
Pathergy test
Inflammatory markers ESR CRP
Thtombophilia screening
FA- ischaemic areas aids detection of posterior segment inflammation + monitoring disease activity
Laser flare photometry of AC
Superficial lesion biopsy
Brain MRI/ CTA systemic ischaemia
91
What is the treatment for Behcet disease
Topical steroid if no posterior involvement (Rare)/ systemic steroids + azathioprine 2.5mg/kg daily for initial management of posterior uveitis/ topical/regional steroid used. Cyclosporine or inflixiamb combined with azathioprine/ systemic steroids. Recommended by EULAR in severe eye disease (>2 line reduction and /or retinal vasculitis/ macula involvement). Intravitreal infliximab novel/ Infliximab/ Adalimumab considered for vision threatening BD. Inferferon alfa with our without steroids as per EULA alternative to ciclosporin/infliximab/ azathioprine/ steroid regime.
92
What immuno combination should NOT be used in Behcet disease and what is the risk
Interferon alfa + azathioprine NOT recommended due to risk of myelosuppression.
Interferon sole treatment as an alternative to the usual ciclosporin/ infliximab/ azathioprine/ steroid regime
93
What is the definitive host for toxoplasma gondii and what is the intermediate host
cat definitive
mice/ livestock/birds/ humans intermediate hosts. Oocytes excreted in cat faeces then ingested by intermediate hosts including via contaminated water supplies.
94
What is the differene between bradyzoite and tachyzoite (toxoplasma gondii)
bradyzoite cysts are inactive stage lying dormant within tisues such as eye / brain/ skeletal muscle. Bradyzoite cysts rupture to release tachyzoites, the profilerating active form stimulating inflammatory reaction.
95
What is the commonest cause of focal retinitis causing inflammation adjacent to a chorioretinal scar
Toxoplasma gondii
96
What are the ocular features of toxoplasmosis
symptoms: unilateral acute/ subacute floaters/ blurry vision/ photophobia. Spill over anterior uveitis common. Single inflammatory foci of fluffy white retinitis or retinochoroiditis associated with pigmented scar typical. Lesions tend to involve posterior pole. De novo foci associated with old scar. Vitritis- severe impair fundal visualization. Vasculitis may be arterial more commonly venous. Optic disc oedema common. Extensive/fulminant involvement immunocompromised. Absent retinochoroiditis. Neuroretinitis. Recurrence common average attacks is 2.7 per patient.
97
What are the investigations for Toxoplasmosis
Serology Toxoplasmosis IgG antibodies within 1-2 weeks of initial infection. Indicate exposure to organism at some point in the past. IgM positivity means infection acquired in last 1 year.
PCR testing of intraocular fluid is sensitive, highly specific. Ocular fluid antibody assessment of specific IgG in aqueous is reasonably sensitive, OCT shows macular oedema, B scan exclude RD in presence of severe vitritis.
98
What is the treatment for toxoplasmosis
antibiotics. Difficult to eradicate parasite. Treat for 4-6 weeks with complete healing over 4-6 months. Treatment of congenital toxo in neonates with antimicrobials for 1 year reduce development of scars. Prednisolone used in conjunction with antibiotics
Topical steroid and mydriatic
PPV in select cases.
Prevention of recurrence with co-trimoxazole
99
What are the antibiotic options in Toxoplasmosis
Triple therapy (pyrimethamine, sulfadizine, steroids) sometimes supplemented with clindamycin. Folic acid given with pyrimethamine as it is a folic acid inhibitor.
Azithromycin used in combination with pyrimethamine, folic acid and steroid. Co-Trimoxazole BD in combination with prednisolone better tolerated
100
Ocular toxo doesnt always need treatment. When is treatment advised
sight threatening lesion involving macula/ papillomacular bundle/ optic nerve head/ major blood vessel
Severe vitritis
Active ocular toxoplasmosis in immunocompromised
101
What is Toxocariasis
infestation with common intestinal ascarid (roundworm) of dogs. Toxocara canis.
Human infestation is via ingestion of soil/ food contaminated with ova shed in canine faeces. These ova travel via intestine into blood and eye/ liver/lungs/brain with inflammation
102
What are some signs of ocular toxocariasis
unilateral- around 2/3 cause permanent visual impairment. Causes:
chronic endophthamitis - present with leucokoria, strab, floaters, unilateral visual loss. Fundus shows grey white exudate similar to snowbanking in pars planitis. Posterior pole or peripheral granuloma without inflammation occurs in older child. 1-2DD yellow round white granuloma present in posterior fundus. VR traction may lead to complications due to macula distortion/ RD. Chorioretinal scar. May also cause DUSN
103
What are some investigations for toxocarasis
FBC- eosinophilia
Hypergammaglobulinaemia IgE
Serology- antibodies to Toxocara canis in 50% cases
Ultrasound if vitreous hazy
Aqueous/ vitreous sampling for eosinophilia/ antibody detection/ PCR
104
What is the ocular treatment for toxocariasis
prevention by good hygiene practice/ deworming pets
Steroids- topical/ regional /systemic
Anthelmintic agents- mebendazole/ thiabendazole- worm death may PROMOTE inflammation.
PPV for sight threatening tractional sequelae
105
What is Onchocerciasis
affects eyelid, skin. Second most common cause of infectious blindness in the world. Endemic in Africa and other regions. Parasite is helminth Onchocerca volvulus. Vector is Simulium blackfly which breeds in fast flowing water hence the term 'river blindness'. Larva are transmitted when flies bite to obtain blood. Migrate to subcutaneous sites to form onchocercomas where microfilariae are produced by adult worms. Degenerating microfilariae excite an intense inflammatory reaction
106
What are the clinical features of onchocerciasis
systemic: mainly dermatologic with pruritus, maculopapular rash involving buttocks, extremities. 'leopard skin'. onchcercomas.
Live microfilariae may be seen in the cornea/ vitreous/ suspended in AC after patient postures face down. AAU, posterior synechiae, keratitis punctate, chorioretinitis bilateral involving temporal fundus sparing macula until late, optic neuritis
107
What is the treatment for Onchocerciasis
ivermectin kills microfilariae but not adult worms. given annual for many years. Reduce transmission rates. Moxidectin may be superior to Ivermectin. Doxycycline for 6 weeks. Suramin, steroids- AAU responsive
108
What is DUSN
Diffuse Unilateral Subacute Neuroretinitis
Clinical syndrome due to presence of single motile subretinal nematode eg Toxocara canis/ Baylisacaris Procyonis/ Anycylostoma caninum.
Treat with Photocoagulation to seclude the worm
109
What are the stages of HIV infection
a) flu like illness occuring 2-4 weeks after infection, sometimes with rash.
b) clinical latency asymptomatic period of several years (8)
c) AIDS in half of HIV individuals in 10 years- defined as HIV + with CD4+ T count <200 cells/microlitre or development of 1 or more AIDS defining conditions eg oesophageal candidiasis/ pneumocystis jirovecii/cryptosporidiosis/ CMV/ Kaposi sarcoma/ Lymphoma
110
What are the ocular features related to HIV
Eyelid: blepharitis/ kaposi sarcoma/ HZO. Orbital cellulitis aspergillosis, B cell lymphoma. squamous cell carcinoma- conj.
KCS increased keratitis incidence HSK/ HZO/Fungal. AAU with ocular infections. HIV related micro-angiopathy. Due to declining CD4 count and high plasma HIV- RNA levels. Marker for CMV retinitis risk. Protozoal toxoplasma retinochoroiditis, fungal pneumocystitis choroiditis, cryptococcal, syphilis, TB, B cell intraocular lymphoma. Meningitis/ encephalopathy.
111
What are some ocular features of CMV retinitis
reduced vision from macula involvement or with floaters from vitritis. AAU occur mild with little/ no injection. Cataract late stage finding. Vitritis mild- except in immune recovery. Retinitis is 1/2 areas of dense white retinal infiltration associated with flame shaped retinal haems (pizza pie) beginning peripherally and extending along vascular arcades. Peirpheral areas granular with rounded haems.
Optic neuritis/ retinal necrosis/ frosted branch angiitis shows marked vascular sheathing in 6%
112
What is the treatment for CMV retintiis
Liaise with ID physician. ART mainstay of treatment. Discontinue treatment when CD4+ >100-150 cells/ microlitre
Valganciclovir
IVT ganciclovir/Foscarnet/cidofovir
PPV with endolaser demarcation and silicone oil tamponade
Steroids for immune recovery uveitis
Prognosis poor if macula involved
113
What is immune recovery uveitis in CMV retinitis
cause of limited visual outcome in CMV retinitis, due to rejuvenated immune response against residual viral antigen following immune reconstitution with ART. Can progress to phthisis in some cases
114
What is Progressive retinal necrosis (PRN)
Rare but devastating form of necrotizing retinitis caused by VZV and other herpesviruses. Occurs in patients with AIDS. Poor prognosis
115
What are the ocular features of PRN/PORN
Rapid progressive unilateral/ bilateral visual loss. AAU/ vitritis minimal in contrast to CMV retinitis and ARN.
116
What are the 3 stages of retinitis in PORN/PRN
EARLY- multifocal homogenous yellow white deep retinal infiltrates. macula may be involved at any stage giving cherry red spot
ESTABLISHED/ MIDDLE- signs spread rapidly around the retina with extensive full thickness necrosis. Vasculitis absent or mild and significant haemorrhage uncommon. Inflammation clears perivenular translucency seen
LATE- retinal atrophy follow as arteriolar blood supply decreases. RRD common and optic atrophy
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What are the investigations for PORN/ PRN/ ARN
Vitreous and or aqueous PCR for viral DNA/ antibody essay less effective
118
What is the treatment for PRN/PORN
Immune rescue with ART + aggressive antiviral therapy eg IVT/ IV ganciclovir/ foscarnet. VR surgery for RD yields poor results
119
What is acute retinal necrosis (ARN)
Rare but devastating necrotizing retintis. Affects otherwise healthy individuals and tends to be caused by HSV/ VZV. Poor prognosis more than half achieving only 6/60 due to retinal/ optic nerve ischaemia or RRD
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What are the systemic and ocular features of ARN
Systemic- ARN occurs with HSV encephalitis/ herpetic skin infections
Ocular features- unilateral blurry vision/ floaters/ pain usually a feature. Prominent anterior uveitis and vitritis (panuveitis), episcleritis/ scleritis may occur. One or more discrete foci of peripheral retinal necrosis. Deep yellow white infiltrates with well defined borders. Retinal haemorrhages but less prominent than CMV retinitis. Circumferential spread of retinal involvement. Posterior pole involvement late. Optic neuritis sometimes features. Occlusive retinal vasculitis including arteritis.
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What is the treatment for ARN
Aciclovir/ oral valaciclovir/ IVT ganciclovir/ foscarnet. Systemic steroids 24 hours after starting antiviral therapy. Laser retinopexy no effect on necrotic areas no change to RD rates. PPV for RRD with silicone oil tamponade
122
What is the treatment for HSV anterior uveitis
Topical steroids and topical cycloplegia + oral antiviral. Steroids used with caution if epithelial disease present.
123
What is the treatment for VZV anterior uveitis
Topical steroids and topical cycloplegia + oral antiviral. For repeated recurrences may need systemic antiviral prophylaxis.
124
What are some ocular features or rubella uveitis
salt pepper retinitis, chronic anterior uveitis, implicated as a cause of fuchs uveitis, glaucoma, microphthalmos
125
What is presumed ocular histoplasmosis syndrome (POHS)
Histoplasma capsulatum infection following inhalaton of yeast form of this dimorphic fungus leading to systemic mycosis histoplasmosis. Lung involvement most common feature. Common in AIDS. Endemic to Missisipi river valley in USA
126
What are the ocular features of POHS
Asymptomatic until MNB present.
Triad: multiple white atrophic chorioretinal histo spots 200 microns diameter, peripapillary atrophy, absent vitritis. linear mid peripheral scars can occur. MNV late manifestation associated with pre existing histo spot.
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What are the investigations for POHS
HLA testing- POHS associated with HLAB7, DRw2
serological test helpful IF positive but usually negative in absence of systemic mycosis
FA/OCT when MNV suspected
128
What is the treatment for POHS
Spontaneous MNV regression may occur. without treatment most eyes have VA 6/60 or less. IVT anti VEGF for MNV, Amsler grid testing for fellow eye weekly
129
What are risk factors for endogenous candida endophthalmitis
IVDU
septic focus due to indwelling catheter, chronic lung disease eg CF, general debilitation and diabetes. Uncommon in AIDS
130
What are the clinical features of candida endophthalmitis
peripheral fundus lesions may cause little/ no visual disturbance while central lesions or severe vitritis manifest earlier. Slower progression compared to bacterial endophthalmitis. Bilateral involvement common. AAU uncommon/ mild in early disease. Vitritis marked with fluffy cotton ball or string of pearl colonies. Chorioretinitis 1 or more small creamy white lesions with vitritis. Retinal necrosis lead to RD with PVR
131
What are the investigations for candida endophthalmitis
Vitreous biopsy- vitreous cutter than needle-> PCR and culture + sensitivities
Systemic investigations like blood and urine culture
132
What is the treatment for candida endophthalmitis
Antifungal treatment- choose according to local micro support. IV amphotericin B in combination with oral flucytosine, voriconazole orally or IV has broad spectrum of action with low resistance high ocular penetrance.
PPV at earlystage especially for severe/ unresponsive disease. Reduces fungal load, facilitate therapeutic agent penetration, clear ocular media
133
What is TB uveitis
chronic granulomatous inflammation caused by Mycobacterium TB. Primary lung disease but spread via blood to eyes. Immune deficiency risk factors when atypical mycobacterial like m. avium may cause disease
134
What are the ocular features of TB uveitis
AAU- granulomatous- iris nodules present and broad PS
Vitritis common secondary to anterior/ intermediate/ posterior primary foci. Macula complications are CMO/ ERM formation. Choroidal granuloma (tubercle) choroiditis multifocal and centrifugally spreading serpiginuous pattern. Choroiditis tracks retinal vessels specific for TB. Retinal vasculitis venous. Retinal haems and vascular occlusion with ischaemia + preretinal/ disc NV
135
What is Eales disease
represent a hypersensitivity reaction to TB
136
What are the investigations for TB uveitis
Tuberculin skin test + within 48 hours. Tspot TB test detects latent TB. Sputum test with PCR/ IGRA blood test (80% sensitive in active disease) and independent of previous BCG vaccine. HIV status. CXR/ PET CT. Ocular aqueous and vitreous sampling rarely yields demonstrable bacteria. FAF allows activity staging. lesions become hypoFAF with healing
137
What is the treatment for TB uveitis
prolonged multi drug therapy (often 4 initially). Ethambutol cause optic neuropathy, rifabutin cause AAU. Topical and systemic steroids used together to reduce inflammation induced damage especially in early weeks of treatment. Laser to ischaemic retina with preretinal NV
138
What is the difference between primary/ seocondary/ tertiary and latent syphilis
Primary- painless ulcer (chancre) on genital/ anus
Secondary- maculopapular rash and other systemic features
Tertiary- 40% of untreated cases cardiovascular manifestations aortitis, neurosyphilis, gummatous infiltration of bone and viscera
139
What are the ocular features of Acquired syphilis
AAU- granulomatous/ non granulomatous. Bilateral in 50% ,roseolae are dilated iris capillaries developing into yellow nodules, IOP elevated. Chorioretinitis localised but multifocal, bilateral associated with vitritis, exudative RD. Acute syphilitc posterior placoid chorioretinopathy (ASPPC) large pale yellow subretinal lesion in posterior pole- secondary infection of choriocapillaris, ground glass retinitis, optic neuritis and neuroretinitis
140
What are the investigations for acquired syphilis
serology
systemic assessment by appropriate specialist. LP to rule out neurosyphilis, HIV status established. Aqueous and vitreous sampling for PCR indicated.
141
What is the treatment for acquired syphilis
supervision by ID team. Extended course of parenteral penicillin. Alternatives inpenicillin allergy Topical / systemic steroid given with antibiotics to ameliorate inflammatory damage
142
What is the Jarish Herxheimer reaction
systemic response to treponemal antigens released on commencement of therapy and may include progression of ocular signs
143
How do you differentiate a head louse from an adult tick
tick has eight legs
louse has six legs
144
What are the systemic signs of Lyme disease
several days after a bite annular skin rash erythema chronicum migrans forms with constitutional symptoms.
Neurological (Cranial nerve palsies, meningitis, cardiac) within a few weeks
Stage 3 chronic arthritis large joints, polyneuropathy, encephalopathy
145
What are the ocular features of Lyme disease
Uveitis uncommon but can be anterior intermediate (common) or posterior. Transient conjunctivitis/ bilateral stromal keratitis/ episcleritis/ myositis.
146
What is the treatment of Lyme disease
Acute disease treatment highly effective with oral doxycycline (not in children and pregnancy). amoxicillin , erythromycin
147
What is endogenous bacterial endophthalmitis
intraocular infection from source within the body- Gram positives predominate in North America/ Europe. Gram negatives in East Asia. Risk factors of debilitating disease are IVDU, Indwelling catheter, tooth abscess, endocarditis, UTI. Visual prognosis poor than post op endophthalmitis
148
What are the ocular features of bacterial endogenous endophthalmitis
diagnosis delayed. differential is endogenous fungal endophthalmitis. Blurry vision/ pain and redness. Signs similar to post op endophthalmitis though retinal infiltrates an early feature reflecting route of infection
149
Investigations for bacterial endogenous endophthalmitis
search for septic focus (blood/ urine cultures/ endocarditis/ LP in collab with physician). Aqueous and vitreous samples for MC&S
150
What is the treatment for bacterial endogenous endophthalmitis
Systemic infection with IV abx according to local guidelines. Broad spectrum abx may be necessary. Endophthalmitis treated with IVT and PO fluoroquinolone. Systemic steroids is undefined. PPV may improve prognosis in some patients
151
What are the ocular features of cat scratch disease including treatment
neuroretinitis commonest manifestation, focal retinitis, choroiditis, vasculitis rare. Can be associated with preauricular lymphadenopaty (parinaud oculoglandular syndrome).
Treat with PO Doxycycline
152
What is Leprosy
Hansen disease. Chronic granulomatous infection caused by Mycobacterium leprae and M. lepromatosis. Mode of spread uncertain but nasal secretions thought to cause chronic immune response with peripheral neuropathy
153
What are the systemic features of leprosy
Tuberculoid (paucibacillary)- 1- more hypopigmented maculaes/ anaesthetic skin patches
Borderline (multibacillary) most common form similar to tuberculoid but numerous and extensive lesions
Lepromatous (multibacillary) widespread cutaneous thickening with leonine facial appearance, peripheral plaques and nodules, URT involvement and peripheral nerve lesions
154
What are some ocular features of leprosy
keratitis- thickened beaded corneal nerves, PEE, pannus and CoNV, AAU- chronic and low grade described as plasmoid (fibrin), miosis and iris atrophy from impaired dilator pupillae innervation, iris pearls (pathognomonic under 0.5mm in diameter)
155
What are the investigations for leprosy
skin/ ocular specimen show AFB. Lepromin test distinguishes between tuberculoid and lepromatous leprosy
156
What is the treatment for leprosy
systemic: combination of extended duation with antibiotics: dapsone, rifampicin, clofazimime. BCG vaccine offers protection.
ocular: AAU treated with topical steroids
157
How are the white dot syndrome divided
choriocapillaritis
stromal choroiditis
158
What is MEWDS
uncommon idiopathic disease acute onset in young adult females. 25-50% describe preceeding viral illnes. Influenza vaccine known to trigger thsis. Immune induced inflammation of choriocapillaris suspected. Multimodal imaging is key to make diagnosis
159
What are the clinical features of MEWDS
Painless monocular blurring (6/9 to 6/60), subjective scotomas and photopsias. Subtle posterior vitritis in 50%, posterior pole lesions numerous small (100-300 microns), ill defined deep grey white patches sparing the fovea with orange granular appearance and dulled reflex. Optic disc oedema present sometimes. Recovery occurs after weeks leaving residual signs. If recurs, suspect idiopathic multifocal choroiditis
160
What are the investigations for MEWDS
VF- Blind spot enlargement with temporal VF defect
FA shows early hyperfluorescence in areas of white dots with late staining. Vessel wall leakage and disc staining may be seen. FAF shows hyperFAF spots during active inflammation corresponding to macula lesions. ICGA shows hypoF spots in late angiographic phase. OCT shows IS/OS/ disruption
161
What is the treatment for MEWDS
Not required as spontaneous improvement in 8-10 weeks
162
What is the ICG Angiographic classification of choroiditis
1A- Primary choriocapillaritis (unknown trigger)- MEWDS, APMPPE, Serpiginous choroidopathy, MFC, PIC
1B- Secondary choriocapillaritis (pathogen triggers immune response)- ASPPC, TB related serpiginous choroiditis
2A- Primary stromal choroiditis (stroma target of inflammation)- HLA-A29 Birdshot, VKH, SO
2B- Secondary stromal choroiditis (secondary to systemic inflammation)- sarcoid, tubercular chorioretinitis, syphilitic chorioretinitis)
163
What is APMPPE (Acute posterior multifocal placoid pigment epitheliopathy)
uncommon bilateral idiopathic inflammatory disorder. Affects young to middle aged adults of both genders. Viral prodrome in 1/3rd. Speculated to occur due to cell mediated immunity to viral antigen. Inflammation at the level of choriocapillaris leads to hypoperfusion and ischaemia of RPE + photoreceptors.
164
What are the clinical features of APMPPE
Subacute moderate visual impairment with central/ paracentral scotomas. If macula involved VA can range 6/12 to 6/60. AAU/ vitritis very mild. Multiple large deep yellow-white placoid lesions initially at posterior pole. Within weeks, the majority fadewith residual RPE disturbance of varying severity. SRF macula fluid seen.
Neuro symptoms can happen with cerebral vasculitis
165
What is the prognosis of APMPPE
Visual symptoms and retinal appearance typically improve within weeks and long term prognosis good. But subgroup of patients with recurrence and longer period of disease activity. Factors predicting poor prognosis are
1) age >60
2) unilaterality
3) interval before second eye involvement of 6 months
4) disease recurrence
5) leaking from choroidal veins
166
What investigations are useful in APMPPE
Alternative diagnoses excluded
HLA-B7, HLA- DR2 in many patients
FA of active lesions show early dense hypoF and late hyperF. Late hyperF may result from vascular leakage
FAF- scattered hypoAF surrounded by hyperAF corresponding to placoid lesions
ICGA- active placoid lesions manifest as early and late hypoF secondary to poor choriocapillaris perfusion
OCT macula- disruption of outer retinal layers
OCTA- abnormal flow in choriocapillaris
CNS imaging in patients with neuro symptoms r/o retinal vasculitis
167
What is the treatment for APMPPE
Usually not required, but consider steroids for patients with macular involvement. Steroids and ciclosporin for cerebral vasculitis.
168
What is Serpiginous choroidopathy
usually bilateral but asymmetrical. Inflammation in outer retina and choriocapillaris. Occurs in middle age, affects men more than woman. Associated with HLA-B7. Recurrent disease with poor prognosis. 25% have final VA <6/60. TB/ Syphilis can result in a similar patter (serpignioid)
169
What are the clinical features of serpiginous choroidopathy
initial unilateral blurring of central vision/ scotoma/ metmorphopsia. AAU/Vitritis common but mild. Active fundal grey white lesions may remain active for several months before becoming scalloped and atrophic. Diseases starts at optic disc and extend gradually. Recurrence contigious with or adjacent to existing areas resulting in extensive chorioretinal atrophy. MNV/ subretinal fibrosis/ preretinal NV are complications
170
What are some investigations of serpiginous choroidopathy and what do they show
TB should be excluded and test for syphilis
FAF- shows inflammation extent. Active lesion show hyperF halo on edges of areas hypoAF. Inactive lesions dark- good to monitor disease progression
OCTA- flow void at level of choriocapillaris
ICGA- active lesions show marked hypoF throughout all phases of angiogram
171
What is the treatment for serpiginous choroidopathy
PO/ IV steroid may control activitiy. Variety of immunosuppressives and infliximab may be effective alone or in combination.
172
What is idiopathic multifocal choroiditis
uncommon bilateral but asymmetrical chronic/recurrent disease affecting young/ middle aged adult women. Most people have flu like symptoms before ocular disease onset. Severity and prognosis variable. Unknown aetiology. condition diagnosed clinically after systemic and ID (TB/West nile virus choroiditis/candida) have been excluded
173
What are features of multifocal choroiditis
blurry vision, photopsia, floaters. AAU, Vitritis, fundus show multiple discrete ovoid yellowish grey lesions 50-350 microns diameter at posterior pole/ periphery with linear clusters/ streaks. Inactive lesions have sharply defined margins and pigmented borders resembling POHS. PPA may be seen. Course prolonged with development of new lesions and recurrent inflammatory episodes. MNV occurs in 25-25%, CMO and subretinal fibrosis resembling SFU can develop. Optic disc oedema and blind spot enlargement can be present
174
What investigations are useful in multifocal choroiditis and what do they show
VF- large defects not corresponding to exam findings. FA- early hypoF and late hyperF. Old inactive lesions show window defects. If MNV develops, hyperF seen due to leakage.
ICGA- hypoF acute lesions which may not be apparent. Old lesions remain hypoF
OCT- elevated RPE/ with subRPE material intermediate reflectivity and surrounding outer retinal disruption
OCTA- well demarcated areas of reduced flow at level of choriocapillaris
ERG remains normal until advanced retinal atrophy
175
What is the treatment for Multifocal choroiditis
systemic + local steroids. steroid resistant patients need immunosuppressive therapy (cyclosporin/ tacrolimus together with MMF). MNV treated with steroids and anti-VEGF agents
176
What is PIC
PIC affects young myopic women- both eyes involved in 80%. Involvement is asymmetrical. Likely to be a form of idiopathic MFC. involvement predominantly involves MACULA
177
What are the clinical features of PIC
Blurring, scotoma, floaters, photopsia. AAU/ vitritis absent/ mild. Fundus shows several yellow white macula spots with fuzzy borders at the level of inner choroid and retina sometimes with overlying serous sensory RD. These evolve into sharply demarcated atrophic scars with little pigmentation similar to histo spots in POHS. Central vision may be compromised by lesion at fovea or MNV. V/A improves to 6/7.5 in 50% cases
178
What are some investigations for PIC
VF- enlarged blind spot in 40% and central/paracentral scotoma
FAF- hypoAF spots and areas of hyperAF
FA- early hyperfluorescence and late staining lesions shows MNV
OCT/OCTA- submacula MNV and acute hyperreflective material with disruption of outer retina in acute stage
ICG- multiple mid phase hypoF lesions in posterior pole. Choroidal vessels show localised points of hyperF close to vessel walls.
179
What is the treatment for PIC
No treatment unless evidence of MNV
AntiVEGF- used for choroidal MNV
Steroids/ systemic immunosuppression can be helpful in recurrent CNV
180
What is Birdshot retinochoroidopathy
uncommon idiopathic inflammatory disease usually affecting middle aged women. Chronic/ bilateral and manifest with severe retinitis and stromal choroiditis that are independent inflammatory events. Nearly all patients HLA-A29 positive
181
What are the clinical features of Birdshot retinochoroidopathy
insiduous impairment of central vision with floaters/ nyctalopia
vitritis in either or both eyes
fundus shows multiple ill defined ovoid cream coloured choroidal patches <1DD size in posterior pole and mid periphery. Lesions often radiate out from disc, spare macula. More in inferotemporal area. Inactive lesions are well delinieated atrophic spots. CMO/ ERM/ MNV develop.
VF testing is appropriate functional test for monitoring disease evolution and treatment
182
What is the prognosis for Birdshot retinochoroidopathy
1/3rd eventual BCVA <6/60. Improving results with immuno treatment. Choroiditis treated but retina less so
183
What are the investigations for Birdshot retinochoroidopathy
HLA- A29- over 95% positive
OCT- CMO
Autofluorescence - numerous hypoAF lesions
FA- extensive vascular leakage iwth disc and vessel staining. Diffuse CMO. Fovea spared and true CMO less common
ICGA- lesions more numerous. Early HypoF which persist into late phases and do not correspond to retinal lesions
OCTA- areas of flow void in choroidal stroma with initial sparing of choriocapillaris. As disease progresses this is affected.
ETG- normal in early disease but with time rod/cone abnormalities
184
What is the treatment for birdshot retinochoroiditis
first step- subtenon triamcinolone in patients with monocular disease
Upto 90% need systemic treatment. Systemic steroids initially but immuno treatment often needed (cyclosporin + MMF + infliximab)
185
What is AMN
Rare self limited condition affecting healthy young women. May affect one - both eyes preceeded by flu like illness. Incidence increased since COVID.
186
What are the clinical features of AMN
Dereased vision + paracentral scotomas. Red brown wedge shaped lesions in flower petal arrangement around macula centre corresponding to scotoma. FA normal or shows faint hypoF. Acute aMN shown by wedge shaped parafoveal lesion on IR imaging. OCT shows hyperreflectivity in OPL. Over time retinal layers thin and disruption to outer segment + RPE. Syptoms signs fade but scotoma remains
187
What is AZOOR (Acute zonal occult outer retinopathy)
group of rare conditions with visual symptoms in 1-2 eyes of young/ middle aged women some have antecedent viral illness. AZOOR commonest of AZOR syndromes. Characterised by minimal fundal signs in disease course
188
What are the clinical features of AZOOR
Photopsia/ mild vitritis freqeutn with subtle vasculitis.
VF- acute onset 1 or more zones of VF loss. Field loss progress with limited recovery
Later findings- RPE clumping + vascular attenuation in involved areas and peripapillary region. Fundus may look normal
189
What investigations are performed in AZOOR and what do they show
FAF- abnormalities and helps define areas of retinal involvement.
OCT + FAF- similar signs to MEWDS but choriocapillaris preserved and no evidence of hypoF on ICGA.
OCT- loss of outer retina in 1 or more zones. Chronic atrophy of RPE + thinning of outer retinal layersfollowed by thinning of inner retinal layers
ERG- a and b wave reduction in amplitude + delayed 30Hz flicker. (cones affected more than rods). EOG shows absence or severerly reduced light rise
190
What is the treatment for AZOOR
No treatment proven - some reports of systemic steroids useful
191
Part 2
flashcards
Decks in class (20)
# Cards
-
Exam Techniques68
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Eyelids183
-
Lacrimal Drainage System41
-
Orbit167
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Dry Eye34
-
Conjunctiva80
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Cornea183
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Corneal and Refractive Surgery49
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Episclera and Sclera35
-
Lens101
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Glaucoma236
-
Uveitis191
-
Retinal Vascular Disease147
-
Acquired Macula Disorders163
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Inherited Retinal Degenerations127
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Retinal Detachment99
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Vitreous Opacities10
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Strabismus201
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Neuro-ophthalmology352
-
Ocular Tumours164
