Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

RETINA 01 > RYAN VOLUME 2 - White Spot Syndromes and Related Diseases #77 > Flashcards

RYAN VOLUME 2 - White Spot Syndromes and Related Diseases #77 Flashcards

1
Q

What are the White Spot Syndromes?

A

Group of diseases likely driven by autoimmune dysfuction and characterized by disruption of the outer retina, RPE and the choroid or a combination of these.
Shared characteristics: blurred vision, photopsias, visual field changes, floaters, changes in contrast sensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Is there any evidence of autoimmunity?

A

Although there is not an anti-retina antibody, an increased prevalence of systemic autoimmunity exist in both patients with WSS and their first- and second-degrees relatives.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Birdshot Chorioretinopathy -
Definition

A

Multiple small, cream colored lesions scattered around the optic disc radiating to the equator in a shotgun pattern with histopathologic evidence that the primary lesion is in the choroid. Bilateral, chronic process with vitritis, retinal vasculitis and CME.
Strong link with HLA-A29.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Birdshot Chorioretinopathy -
Symptons

A

Blurred vision, floaters, photopsias.
Most have 20/40 or better.
Eye generally not red or painful.
Severe nyctalopia may occur.
VF and color vision changes may occur.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Birdshot Chorioretinopathy -
Epidemiology

A

6 - 7.9% with patients with posterior uveitis.
Slight female preponderance.
Mean reported age at the onset: 53 years.
White people / Northern European descent
HLA-A29 (present in 7% of white people).
>90% of patients with BCR are HLA-A29 positive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Birdshot Chorioretinopathy -
How many subtypes are there for the HLA-A29

A

11 subtypes.
HLA-A2902 is most commonly associated with BCR.
HLA-A2902 us 20 times more prevalent in white people than the HLA-A29*01.
The rest subtypes are exceedingly rare.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Birdshot Chorioretinopathy -
Fundus findings

A

Oval/round lesions, 1/2 - 1/4 DD in size, deep retina/choroid.
Can be subtle and asymmetric. Can coalesce.
Tend to cluster close to the disc.
Linear radiation away from the disc.
Align with choroidal blood vessels.
RPE and overlying retina appear intact but in chronic stages there may be RPE atrophy.
Minimal AS inflammation.
Optic disc edema, retinal vasculitis, CME, ERM.
Retinal / CNV.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Birdshot Chorioretinopathy -
2006 consensus on BCR diagnosis

A
  • Bilateral
  • At least 3 peripapillary lesions inferior or nasal to the disc
  • Low grade AS inflammation (< or = 1+ cells)
  • Low grade vitreous inflammation (< or = 2+ vitreous haze)

Supportive findings
- HLA-A29 +
- Retinal vasculitis
- CME

Exclusion criteria
- Significant keratic precipitates
- Posterior synechiae
- Infectious, neoplasic or inflammatory disease that may cause choroidal lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Birdshot Chorioretinopathy -
Clinical course and prognosis

A

Chronic disease. Does NOT regress.
Symptoms may be present for years prior to the onset of fundus lesions.
VA may be good despite the symptoms.
VA may decrease slowly despite the treatment.
20/40 or better in the best eye in 75%.
CME in 50.5% - most common cause of vision loss.
CNV in 5% (near the disc and bilateral).
Optic disc edema leading to atrophy may occur.
Preservation of Arden ratio (EOG) may be a positive factor for better outcome.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Birdshot Chorioretinopathy -
Fluorescein angiography

A

Early hypofluorescence.
Late diffuse HYPERfluorescence.
Increased transit time, leakage with CME, optic disc hyperfluorescence, disc/retinal neovascularization
Vessel wall staining

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Birdshot Chorioretinopathy -
Indocyanine Green Angiography

A

Hypofluorescent lesions during intermediate and late phases(active disease). Lesions appear to align with medium to large vessels.
Hypofluorescent lesions may occur prior to clinical lesions.
Choroidal vessels appear indistinct.
Chronic disease may appear as isofluorescent lesions.

ICGA may prove useful to monitor efficacy of treatment because lesions may disappear with treatment.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Birdshot Chorioretinopathy -
OCT

A

Thinning of the retina and choroid in periphery.
Discrete outer retinal and choroidal hypereflective foci.
Hyporeflective in the suprachoroidal space - correlates to the presence of symptoms.
Perivascular retinal thickness correlates to the activity of vasculitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Birdshot Chorioretinopathy -
OCT Angiography

A

Decreased vessel density (specially deep outer retinal plexus).
Decreased choroidal flow.
Larger choroidal vessels on the edges of the lesions.
Retinal capillary loops and ioncreased intercapillary spaces.
Thinning of the retina and hyperreflective foci in the outer retina.
Generalized thinning of the choroid and loss of Sattler’s layer.
Hyperreflective foci in the choroid.
Flow voids in the choroid.
— findings suggest that the the focal lesions develop in Haller’s layer eraly on and expand anteriorly if untreated.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Birdshot Chorioretinopathy -
Fundus autofluorescence

A

Hypoautofluorescent lesions. Usually, there are more hypoFAF lesions than clinical lesions
Diffuse hypoautofluorescence was correlated with chronicity.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Birdshot Chorioretinopathy -
Electroretinogram

A

Early stage disease abnormal ERG x Late stage disease abnormal ERG

Early - electronegative ERG pattern
- Supernormal ERG amplitudes
- Decrease in b-wave amplitude versus a-wave amplitude - inflammation
- Rod b-wave may be affected prior to the photopic b-wave and flicker

Late
- Progressive decrease in a-wave and b-wave amplitudes.
- 30 Hz flicker implicit time is abnormal in 70% of patients at baseline

ERG findings may improve with treatment.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Birdshot Chorioretinopathy -
Visual field

A

Peripheral constriction
Enlarged blind spot
Central / paracentral scotomas
Generalized diminished sensitivity

It is unlikely that the birdshots lesions themselves cause scotomas.

The changes may be reversible with immunosuppression - MONITORING component.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Birdshot Chorioretinopathy -
Adaptive optics

A

Patchy loss of photoreceptors on AO-SLO that colocalized with areas of photoreceptor disruption on OCT.
Reduced cone density.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Birdshot Chorioretinopathy -
Systemic associations

A

No definitive systemic associations.
Hearing loss / cutaneous vitiligo/ vascular disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Birdshot Chorioretinopathy -
Pathogenesis

A

Inflammation as a primary feature.
Lesions consist of an accumulation of lymphocytes (CD8+T that secret IL-17) in the choroid at multiple levels, occasionally associated with hemorrhage.
Nongranulomatous nodular choroidal infiltration was reported.
Increased IL-17 levels in the aqueous humor.
HLA-A29 - ERAP2 - IL-17

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Birdshot Chorioretinopathy -
Diferential diagnosis

A

Sarcoidosis.
Sympathetic ophthalmia.
Pars planitis.
Syphilitic chorioretinitis.
Intraocular B-cell lymphoma.
MFC.
Panuveitis sd.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Birdshot Chorioretinopathy -
Management

A

Corticosteroids (oral, sub-tenon, intraocular)- reduce CME, inflammation and ON edema, improve symptoms.
Immunosuppressive therapy - consider when low dose steroids are not sufficient at controlling disease and for long-term refractory cases.
- Cyclosporine (inhibits T lymphocytes and prevents S-Ag-induced experimental uveitis). Nephrotoxicity / hypertension.
- Azathioprine / Mycophenolate mofetil / Methotrexate (antimetabolites) - bone marrow suppression and hepatotoxicity.
- Cyclophosphamide and chlorambucil (alkylating agents). bone marrow suppression / malignancies.
- Daclizumab (monoclonal antibody against alpha-subunit of the IL-2 receptor of T-cells)
-Infliximab / Adalimumab
- Secukinumab (Targeted IL-17 human monoclonal antibody) - was NOT found to be effective in early trials.
- Tocilizumab (IL-6 receptor antagonist) - beneficial in patients with refractory CME

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Placoid Diseases

A
  • Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)
  • Serpiginous choroiditis (SC)
  • Relentless placoid chorioretinitis (RPC)
  • Persistent placoid maculopathy (PPM)

Placoid lesions due to choroidal flow deficits
OCT: opacification of the Henle’s layer and variable loss in the ONL.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Symptoms

A

Onset of central vision loss, blurred vision, paracentral scotoma, metamorphopsia, “spots” in the vision and photopsias.
Bilateral 75%.
If unilateral, fellow eye involved in few days or weeks.
Headaches, stiff neck and malaise may accompany ocular symptoms.
Recent viral sd/ vaccination.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Epidemiology

A

Male = female
20 - 50 y.o.
Older individuals may have worse outcome with moderate or severe vision loss due to GA or CNV.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Fundus findings

A

Multiple round flat confluent cream-colored lesions with indistinct margins scattered in PP and periphery. Lesions of different ages can be visible.
The lesions tend to resolve centrally with hypopigmentation.
Later, mild pigment mottling and, finally, increasing coarse pigment clumping may ensue.
Lesions can enlarge.
Localized serous detachments may occur.
Vasculitis. Subhyaloid hemorrhage. CNV. Disc edema.
Variable uveitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Clinical course and prognosis

A

Improvement of the visual symptoms within 2 - 4 weeks.
60% of the eyes have residual visual symptoms.
Foveal involvemente - important prognostic marker
70% of the eyes present with foveal involvement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Fluorescein angiography

A

Early hypofluorescence.
Late HYPERflourescence - staining (progressive/irregular)

As the process becomes inactive, HYPERfluorescence due to window defects in the mottled RPE develops

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Indocyanine Green Angiography

A

Early hypofluorescence. Late hypofluorescence.
As lesions heal, late hypofluorescence becomes smaller and less defined (supports choroidal ischemia).
More hypofluorescence in acute fase due to swollen outer retina and RPE.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
OCT

A

Serous detachment - bacillary detachment (splitting of the photoreceptor layer).
– presence of reflective material in the fluid
Mild hyperreflective area above the RPE in the photoreceptor layer corresponding to the placoid lesions.
Hyperreflective lesions radiating in the Henle fiber layer and widened choriocapillaris - acute phase
Drusen-like abnormalities and RPE disruption.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
OCT Angiography

A

Flow deficits. Improve as lesions heal.
Areas of deficits may extend beyond clinical lesions .

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Fundus autofluorescence

A

As lesions heal:
Clinically - lesions become pigmented centrally and have a halo of depigmentation
FAF - central intense HYPERautofluorescence and hypoautofluorescence corresponding to the depigmented halo.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Electrophisiology

A

ERG normal or minimally subnormal.
Abnormal Arden ration reported.
ERG and EOG may normalize

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Systemic associations

A

CNS - cerebral vasculitis / meningoencephalitis / stroke / sixth CN palsy / hearing loss.
– CSF: pleocytosis
MRI and CSF analysis are important!
Systemic vasculitis.
Erythema nodosum, ulcerative colitis, thyroiditis, nephritis, juvenile rheumatoid arthritis.
Granulomatosis with polyangiitis, pulmonary tuberculosis, sarcoidosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Pathogenesis

A

Vascular insult - choroidal ischemia - RPE and photoreceptor disruption.
Viral illness: mumps, adenovirus, coxsckievirus B.
Bacteria - Lyme, GAS.
Vaccine - influenza, varicella, menigoC conjugate, hep B.
Lymph T.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Differential diagnosis

A

Acute syphilitic posterior placoid chorioretinopathy - FA, ICGA and OCTA do not show inner choroidal ischemia.
SC, RPC, PPM.
Systemic vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) -
Management

A

Steroids - if CNS involvement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Idiopathic Serpiginous Choroiditis

A

Helicoid peripapillary chorioretinal degeneration
Bilateral, chronic, progressive, assymetric
Outer retina, RPE, choriocapillaris and large choroidal vessels

38
Q

Idiopathic Serpiginous Choroiditis
Clinical course

A

Asymptomatic until fovea affected,
Blurred vision. Paracentral scotomas (absolute in acute stage, relative when healing)

39
Q

Idiopathic Serpiginous Choroiditis
Epidemiology

A

Healthy individuals
30 - 70 yo.
Slight male predilection.
Higher HLA-B7 - Finnish study.

40
Q

Idiopathic Serpiginous Choroiditis
Fundus findings

A

Classic presentation
- One/several geographic patches of gray or creamy yellow placoid outer retinal and RPE lesions in the peripapilllary region.
- Progresses with recurrences in a centrifugal manner.
- Edematous outer retina
- Serous retinal detachmente.
- Lesions resolve with or without treatment in weeks.
- Extensive RPE and choriocapillaris atrophy.
- Recurrences at the edge of old scars but not always .

Macular serpiginous choroiditis
- Same, except for the location.
- Poorer prognosis.
- Misdiagnosed as AMD, macular dystrohpies or toxoplasmosis.

41
Q

Idiopathic Serpiginous Choroiditis
Other ocular findings

A

Nongranulomatous anterior uveitis.
Fine vitreous cells.
CNV, vasculitis, ON edema, bilateral FTMH.

42
Q

Idiopathic Serpiginous Choroiditis
Clinical course / prognosis

A

Recurrences with months / years intervals. Usually one eye is active at a time.
Healing in 2 - 8 weeks
Vision loss correlates to the foveal involvement

43
Q

Idiopathic Serpiginous Choroiditis
Fluorescein angiography

A

Early phase hypofluorescence. Borders may be HYPER (intact choriocapillaris or leakage when old lesion).
Late phase HYPERfluorescence due to staining.

44
Q

Idiopathic Serpiginous Choroiditis
Indocyanine green angiography

A

Early phase hypofluorescence.
Late phase hypofluorescence.
Better to assess the choroid / active lesions.

More extensive than FA and clinical evaluation.

45
Q

Idiopathic Serpiginous Choroiditis
OCT

A

Retinal atrophy and disruption of photoreceptor layers.
RPE thinning, intraretinal fluid, cystic changes.
Choroid / deeper retinal layers increased reflectance.
Hyperreflective of ONL and Henle’s layer (OPL).

46
Q

Idiopathic Serpiginous Choroiditis
OCT angiography

A

Decrease choriocapillaris flow.
Improves with treatment.

47
Q

Idiopathic Serpiginous Choroiditis
Fundus autofluorescence

A

Active/inactive areas.
New lesions: HYPERautofluorescent.
Old lesions: hypoautofluorescent.
Recurrence: HYPERautofluorescence ant the border of older lesions.
Lag behind OCTA findings.

48
Q

Idiopathic Serpiginous Choroiditis
Electrophisiology

A

ERG normal,
EOG abnormal if extense lesions.
Perimetry: scotomas may not be permanent.
Microperimetry may be able to detect subclinical lesions.

49
Q

Systemic associations

A

Systemic lupus erythematosus, Crohn’s disease, celiac disease, extrapyramidal dystonia

50
Q

Idiopathic Serpiginous Choroiditis
Pathogenesis

A

Unknown.
Inner choroid ischemia.
Lymphocytic infiltrate.
Virus (herpes family) / Candida.
Elevated factor VIII - occlusive mechanism
Degenerative etiology

51
Q

Idiopathic Serpiginous Choroiditis
Diferential diagnosis

A

Tuberculosis related SC.
APMPPE.
WSS in general.
Sarcoidosis, systemic non-Hodgkin lymphoma, AAS, toxo, syphilis, posterior scleritis.
Lupus, toxemia of pregnancy, DIVC, thrombotic thrombocytopenic purpura, malignat hypertension

52
Q

Idiopathic Serpiginous Choroiditis
Management

A

TB skin test, chest radiography, quantiferonTB gold
ACE, VDRL. FTA Abs, toxoplasma, viral screen.
AC tap if cells.
Steroids - agressive if foveal involvement.
When tapered, lesions may recur.
Immunosuppresive agents - same as BSC.

53
Q

Tuberculous Serpiginous Choroiditis

A

More vitritis.
More multifocal lesions involvind the periphery.
Smaller lesions.
Similar FA.
FAF may distinguish
– Stippled autofluorescence of tuberculous disease
Choroidal infiltration and RPE elevation may be visible (OCT).
Francisella tularensis and Bartonella hanselae are also associated to SC

54
Q

Relentless Placoid Chorioretinitis
Clinical symptoms
Epidemiology

A

Sudden painless vision loss.
Metamorphopsia and floaters.
17 - 51 yo.
Male = female

55
Q

Relentless Placoid Chorioretinitis
Fundus findings

A

Bilateral posterior creamy-white lesions at the level of the RPE.
Lesions smaller than APMPPE.
As they heal, pigmented chorioretinal atrohpy develops.
Lesions can persist, grow and recur.
Hallmark: presence of numerous (>50 to hundreds) lesions with involvement anterior and posterior to the equator (periphery first, posterior pole later).
Mild vitritis.
SRF.
Disc swelling.
Iritis with KP’s.

56
Q

Relentless Placoid Chorioretinitis
Clinical course

A

Prolonged.
Multiple relapses and recurrences (both eyes simultaneously)
Pigemented chorioretinal atrophy in weeks.
Central vision affected in untreated cases.Relentless Placoid Chorioretinitis

57
Q

Relentless Placoid Chorioretinitis
Fluorescein angiography

A

Early hypofluorescence.
Late HYPERfluorescence (staining).

58
Q

Relentless Placoid Chorioretinitis
ICGA

A

Early hypo.
Late hypo.

59
Q

Relentless Placoid Chorioretinitis
OCT

A

SRF.
PED.
Central (zone 1): dome-shaped elevation in IS/OS junction. Loss of ellipsoid zone and RPE thinning with SRF.
Peripheral to this (zone 2): IS/OS thickened and SRF.
Outermost region (zone 3): normal IS/OS band and mild hyperreflective outer retinal layers.

60
Q

Relentless Placoid Chorioretinitis
Fundus autofluorescence

A

Marked hypoautofluorescence in areas of widespread chorioretinal atrophy.
Zone 1: hypoauto
Zone 2: HYPERauto
Zone 3: hypoauto but less than in zone 1

61
Q

Relentless Placoid Chorioretinitis
Electrophisiology

A

EOG and ERG may be reduced.

62
Q

Relentless Placoid Chorioretinitis
Systemic associations

A

Hashimoto thyroiditis / aseptic meningitis

63
Q

Relentless Placoid Chorioretinitis
Pathogenesis

A

Choroidal ischemia. Similar to APMPPE/ SC

64
Q

Relentless Placoid Chorioretinitis
Diferential diagnosis

A

APMPPE and SC…

65
Q

Relentless Placoid Chorioretinitis
Management

A

Steroids +/- immunosuppression
Adalimumab may be beneficial

66
Q

Compare APMPPE, SC and RPC - photo on the phone

A
67
Q

Persistent Placoid Maculopathy
Clinical Symptoms

A

Gradual decreased vision
No pain
More commonly in one eye
Photopsias and decline in color vision can occur

68
Q

Persistent Placoid Maculopathy
Epidemiology

A

50 - 68 yo
Vision preserved until atrophy of RPE or CNV (both common)
Scarring occurs in the region of CNV

69
Q

Persistent Placoid Maculopathy
Fundus findings

A

Bilateral, almost symmetric large whitish plaque-like lesions at the level of the outer retina and RPE.
Centered in the fovea.
Not contiguous to the optic disc.
Jigsaw pattern to the margins.
Gradual fading of the whitish lesions occurs (months - years).
Satellite lesions can occur.
RPE clumping and mottling can occur.
CNV is common.
No cells in the AS.
Vitreous cells may be present.

70
Q

Persistent Placoid Maculopathy
Fluorescein angiography

A

Early hypofluorescence
Late HYPERfluorescence

71
Q

Persistent Placoid Maculopathy
ICGA

A

Persistent hypofluorescence

72
Q

Persistent Placoid Maculopathy
OCT

A

Hyperreflectivity of the ONL
Disruption of the ELM
Complete restoration of the outer retina could occur

73
Q

Persistent Placoid Maculopathy
OCTA

A

Decreased flow of the choriocapillaris corresponding to the ICGA hypofluorescence
Apparently, the flux is impaired in the choriocapillaris only.

73
Q

Persistent Placoid Maculopathy
Fundus autofluorescence

A

Hypoautofluorescence correlating with RPE damage
Healed lesions show hyperauto and hypoauto

74
Q

Persistent Placoid Maculopathy
Eletrophysiology

A

Normal

75
Q

Persistent Placoid Maculopathy
Systemic associations

A

Nothing definitive.
Hypertension is common

76
Q

Persistent Placoid Maculopathy
Pathogenesis

A

Choriocapillaris hypoperfusion

77
Q

Persistent Placoid Maculopathy
Differential diagnosis

A

SC, APMPPE, RPC, Syphilitic posterior placoid chorioretinitis.

78
Q

Persistent Placoid Maculopathy
Treatment

A

Oral / periocular corticosteroids

79
Q

Multifocal choroiditis / Punctate inner choroidopathy
Symptoms

A

Decreased central vision, photopsias, floaters, metamorphopsia, paracentral/temporal scotomas, ocular discomfort, photophobia (peripapillary lesions - activity of the disease).

More than half have 20/100 or less in the worse eye.

80
Q

Multifocal choroiditis / Punctate inner choroidopathy
Epidemiology

A

White women with myopia (mean -4.6 D)
Second to sixth decades of life (most around 30s)

81
Q

Multifocal choroiditis / Punctate inner choroidopathy
Findings

A

Acute phase: round/oval yellow lesions (1 to several hundred) in the outer retina and RPE. Size 50 to 1000 micra.
Posterior pole, peripapillary region and midperiphery.
Nasal retina often shows clustering.
When limited to the posterior pole, an occasional linear pattern forms.
Initial lesions: sub-RPE, neurosensory detachment may appear.
Lesions erupt into the overlying retina.
With time, the lesions evolve into chorioretinal scars.
After 2-3 years, they become more distinct and pigmented, similar to the punched-out lesions of POHS.
CNV is common. (25 - 30%).
If peripheral linear scars parallel to the ora: Schlaegel lines.
ON edema and atrophy may apper.
Peripapillary subretinal fibrosis: napkin ring.

Quiescent or mild to moderate anterior uveitis.
Non-granulomatous KP and posterior synechiae can be present.
Mild to moderate vitritis.
Findings may be asymmetric.

82
Q

Multifocal choroiditis / Punctate inner choroidopathy
Fluorescein angiography

A

Early hypo
Late HYPER (stain)
Healed lesions: window defect
Dumbbell-shapped pattern of subretinal fibrosis

83
Q

Multifocal choroiditis / Punctate inner choroidopathy
ICGA

A

Hypo (early/mid/late) round spots (more than FA and fundus exam)
Large choroidal vessels may cross the lesion areas
HYPERfluorescence of the wall of choroidal vessels was noted (possible vasculitis)

84
Q

Multifocal choroiditis / Punctate inner choroidopathy
OCT

A

Nodular collections below the RPE that erupted into the subretinal space and outer retina (drusen like material).
Choroidal hyperreflectivity below the infiltrates.

Stages (Zhang)
Choroidal infiltrates - sub-RPE nodules - chorioretinal nodules - regression - retina herniation

Foveal outer retinal hyperreflectivity (FORH): hyperreflectivity finger-like projections extending from the RPE into the ONL associated with discontinuous interdigitation and EZ and extensions of the hyperreflective foci to the internal limiting membrane.

84
Q

Multifocal choroiditis / Punctate inner choroidopathy
Fundus Autofluorescence

A

Hypoautofluor exceeding those that were clinically detected.
Many resolved with immunosuppression

HYPERauto borders or entire lesions can appear in active lesions

85
Q

Multifocal choroiditis / Punctate inner choroidopathy
Electrophysiology

A

Cone and rod depression in MFC with retinal and choroidal atrophy.

86
Q

Multifocal choroiditis / Punctate inner choroidopathy
VF

A

Blind spot enlargement (if normal optic nerve - Acute Idiopatic Blind Spot Enlargement [AIBSE] - MEWDS and MFC)
Peripapillary disfunction rather than optic nerve problem.

Scotomas in areas of PR loss, scarring or serous detachment.

Import to monitor the disease.

87
Q
A
88
Q

Multifocal choroiditis / Punctate inner choroidopathy
Systemic associations

A

EBV - not proven
– IgM against viral capsid antigen or the Epstein-Barr early antigen were present in patients with MFC.
Sarcoidosis

89
Q
A
90
Q
A

RETINA 01 (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions