APMPPE - KSU



WHITE DOT SYNDROME

DDX

INFLAMMATORY

1. ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY. APMPPE

2. MULITPLE EVANSCENT WHITE DOT SYNDROME MEWDS

3. SERPIGINOUS CHORODITIS.

4. BIRDSHOT RETINOCHOROIDOPATHY.

5. MULTIFOCAL CHORODITIS AND PANUVEITIS.

6. PROGRESSIVE SUBRETINAL FIBROSIS AND UVEITIS.

7. PUNCTATE INNER CHOROIDOPATHY.

8. AIDS RELATED RETINAL MICROVASCULARITY.

9. BEHCET DISEASE.

10. VKH.

11. SYMPATHATIC OPHTHALMIA.

12. SARCOIDODSIS.

INFECIOUS

13. SYPHILIS.

14. TUBERCULOSIS.

15. FUNGAL CHORIORETINITIS.

16. CHOROIDAL PNEUMOCYSTOSIS.

17. PRESUMED OCULAR HISTOPLASMOSIS.

18. TOXOPLASMOSIS.

19. TOXOCARIASIS.

20. HSV/HZV.

21. CMV.

APMPPE

AGE: 20 -40

GENDER PREDOMINANCE: FEMALE

UNILATERAL OR BILATERAL: B>U

CELLS: RARELY MIGHT HAVE FEW CELLS IN AC OR VITREOUS.

RETINAL FINDINGS: MULTIPLE CREAMY COLORED PLACOID LESIONS, SCATTERED THROUGHOUT THE POSTERIOR POLE, FOVEA MAY OR MAYNOT INVOLVED, CAN HAVE GEOGRAPHIC SHAPE.

LEVEL OF RETINAL INVOLVEMENT: OUTER RETINA +/- RPE

IVFA: INITIAL HYPOFLUORESCENCE AND THEN LATE STAINING. ICG DOES SHO HYPOFLUORESCENT.

ERG: NORMAL

PRESENTATION: PAINLESS DECREASE VA.

OCULAR FINDINGS: RARELY SCLERITIS, IRITIS, VITRITIS, DISC SWELLING, VASCULAR SHEATHING.

SYSTEMIC FINDINGS: OFTEN PRECEEDED BY FLULIKE ILLNESS, RARELY SYSTEMIC VASCULITIS, MENINGEAL SYMPTOMS. LAB: INCREASE CELLS AND PROTEINS IN CSF, HEMATURIA.

TREATMENT: USUALLY NO TREATMENT NECESSARY, SYSTEMIC STEROID HAVE BEEN TRIED BUT NO STRONG EVIDENCE OF BENEFIT.

OUTCOME: RECOVERY WITHIN 4 TO 6 WEEKS, SOMETIMES LONGER, WITH REISDUAL PIGMENTS CHANGES, VA USUAL RECOVER EVEN IF FOVEA INVOLVED.

RECURRENCES OR EXACERBATIONS: RARE.

MEWDS

AGE: YOUNG FAMLE, 20-50

GENDER PREDOMINANCE: FEMALE

UNILATERAL OR BILATERAL:U>B

CELLS: RARE

RETINAL FINDINGS: MULTIPLE SMALL (100 TO 200 MICRON) WHITE DOTS, USUALLY APPEAR IN PERIMACULAR AREA AND BECOME LESS PROMINENT OUTISDE VASCULAR ARCADES. IN SEVERE CASES LESIONS CAN BE CONFLUENT. COMMON FINDING IS GRANULARITY OF THE MACULA (MULTIPLE SMALL YELLOW, ORANGE, OR WHITE GRANULES IN FOVEA

LEVEL OF RETINAL INVOLVEMENT: OUTER RETINA AND RPE.

IVFA: EARLY PHASE SHOWES PUNCTATE HYPERFLUORESCENT WITH LATE STAINING. ICG SHOWES HYPOFLUORESCENCE.

ERG: DIMINSHED A WAVE IN THE ACUTE PHASE, FOLLOWED BY RECOVERY LATER.

PRESENTATIONS: SCOTOMA, PHOTOPSIA OFTEN IN THE TEMPORAL VISUAL FIELD.

OCULAR FINDINGS: RETINAL VASCULAR SHEATHING, DISC EDEMA.

FEW REPORTS OF ASSOCIATION WITH ACUTE MACULAR NEURORETINOPATHY AND MULTIFOCAL CHORODITIS.

SYSTEMIC FINDINGS: FLULIKE ILLNESS PRESENT IN ABOUT ½ OF THE PATIENTS.

TREATMENT: NO TREATMENT. CYCLOSPORINE HAVE BEEN TRIED IN PATIENT WITH MULIPLE RECURRENCES WITH GOOD RESULTS

OUTCOME: RECOVERY WITHIN 2 TO 6 WEEKS.. MAY SHOW MILD PIGMENTRY CHANGES IN THE MACULA. CNV MAY DEVELOP AS A LATE COMPLICATION

RECURRENCES OR EXACERBATIONS: OCCASIONAL. REMARKABLE FEATURE OF MEWDS IS THE DRAMATIC RESOLUTION OF VA OVER PEROIDS OF WEEKS TO MONTHS

SERPIGINOUS CHORODITIS (GEOGRAPHIC, HELICOID)

AGE: 40-60

GENDER PREDOMINANCE: NONE, MORE IN CAUCASIANS.

UNILATERAL OR BILATERAL: B>U

CELLS: FINE PIGMENTS WITHIN THE VITREOUS IN 50% OF EYES. RARELY AC REACTION.

RETINAL FINDINGS: YELLOW TO GRAY IN COLOR RANGE IN SIZE FROM ONE TO SEVERAL DISC DIAMETERS AND HAVE VARIABLE DISTRIBUTION, LESIONS CLASSICALLY DEVELOP FIRST IN A PERIPAPILLARY LOCATION AND SPREAD CENTRIFUGALLY. NEW LESIONS MAY DEVELOP IN THE MACULA IN ABSENCE OF PERIPAPILLARY SCAR. NEW LESION APPEARS AT THE EDGE OF THE OLD ONES. THE OVERLYING RETINA USUALLY EDEMATOUS.

LEVEL OF RETINAL INVOLVEMENT: CHOROID, OUTER RETINA, RPE.

IVFA: EARLY HYPOFLUORESCNCE AND LATE HYPERFLUORESCENCE. ICG SHOWES HYPOFLUORESCENCE.

ERG: NORMAL, UNLESS IN ADVANCE CASES.

PRESENTATIONS: DECREASE VA, METAMORPHOPSIA, OR SCOTOMA

OCULAR FINDINGS: RETINAL VASCULITIS AND DISC EDEMA.

SYSTEMIC FINDINGS: SOME LINKED IT TO TUBERCULOSIS, ALTHOUGH ANTI TB MEDICATION MADE NO DIFFERENCES. INCREASE PREVALENCE OF HLA B7.

TREATMENT: CONTROVERSY, SOME REPORTS SHOWED BENEFIT OF USING CYCLOSPORINE, SOME USED COMBINATION OF STEROIDS, CYCLOSPORINE, AND CYTOTOXIC DRUGS.

OUTCOME: ACUTE LESION HEALS IN 2 TO 3 MONTHS WITH TIME ATROPHY OF THE RPE, CHORIOCAPILLAREIS, AND CHOROID. CNV MAY DEVELOP IN 13 TO 20%.. CENTRAL VA IS LOST IN 20% OF EYES.

RECURRENCES OR EXACERBATIONS: YES, WITH MONTHS TO YEARS OF REMISSION PERIODS.

BIRDSHOT RETINOCHOROIDOPATHY (VITILIGINOUS CHORIORETINITIS) because of the depigmented nature of the fundus lesion and the association in some patients of Cutaneous vitiligo (SALMON PATCH CHOROIDOPATHY)

AGE: 30 -60, AVERAGE AGE IS 50 (older than previous entities)

GENDER PREDOMINANCE: NONE ?FEMALE (70% of reported cases were female), MORE IN CAUCASIANS OF NORTHERN EUROPE

UNILATERAL OR BILATERAL: B>U

CELLS: MINIMUM AC REACTION, VITRITIS IN ALL PATIETNS.

RETINAL FINDINGS: CREAMY LESION LESS THAN ONE DISC DIAMETER SCATTERED THROUGHOUT THE FUNDUS,

LEVEL OF RETINAL INVOLVEMENT: CHOROID, RPE, OUTER RETINA

IVFA: NO CHANGES IN EARLY PHASES, BUT LATER HYPERFLUORESCENCE DOES OCCUR. MAIN FEATURE IS THAT IT IS MORE PROMINENT OPHTHALMOSCOPICALLY THAN ANGIOGRAPHICALLY.

ERG: OFTEN DECREASE B WAVE, WHILE A WAVE IS MAINTAINED..

PRESENTATIONS: BLURRED VISION, FLOATERS, PHOTOPSIA, LATER NYCTALOPIA, AND COLOR BLINDNESS.

OCULAR FINDINGS: QUITE NON PAINFUL EYE. AC/VITREOUS CELLS, DISC EDEMA, NARROWED RETINAL VESSELES, VASCULITIS, CME IN 62%, ERM IN 10%, CNVM IN 6%, NVE, NVD IN 7%, OPTIC NERVE ATROPHY.

SYSTEMIC FINDINGS: HLAB29 IS POSITIVE IN 80-90%. ( while 7% in control group) ( if u r HLA B29 + then the risk is 50 to 240 fold more then HLA B29 -). ? PATIENTS WITH BIRDSHOT HAVE HIGHER INCIDENCE OF HTN, CVA, CAD, RETINAL VEIN OCCLUSION, OAG)

PATHOLGY: GRANULOMATOUS INFLAMMATION OF THE OUTER RETINA WITH GIANT CELLS, EPITHELIOID CELLS, AND PLASMA CELLS, THE CHOROID WAS LESS INFLAMMED, MODERATE FOCAL INFLAMMATION OF IRIS AND CILIARY BODY.

PATHOGENESIS: ? GRANULOMATOUS INFLAMMATION RELATED RETINAL S ANTIGEN, CHANGES IN BIRDSHOT ARE SIMILAR TO THE CHANGES OBSERVED IN EXPERMINTAL AUTIMMUNE UVEORETINITIS.

WORK UP: U NEED TO R/O SARCOIDOSIS BEFOR CALLING IT BIRDSHOT, WORK UP SHOWED INCLUDE ACE, X RAY, SYPHILIS, MRI FOR LYMPHOMA.

TREATMENT: STEROID HAS LIMITED SUCCESS, IT CAN BE BENEFICIAL FOR CME WHEN GIVEN PERIOCULAR OR SYSTEMIC. FOR CONTROLLING THE DISEASE AND LIMITING RECURRENCE CYCLOSPORINE 2.5 MG/KG/DAY ALONE OR IN COMBINATION WITH AZATHIOPRINE 1.5 TO 2 MG/KG/DAY.

OUTCOME: CHRONIC SLOWLY PROGRESSIVE DISEASE WITH CHRONIC COURSE OF EXACERBATION AND REMISSION. MAIN CAUSE OF DECREASE VA IS CME, OPTIC ATROPHY. 30 TO 45% OF EYES WOULD END WITH VA U

CELLS: AC REACTION AND VITRITIS

RETINAL FINDINGS: YELLOWISH TO GRAY CHOROIDAL LESION WHICH VARY IN SIZE FROM 50-350MICRON , VARY IN NUMBER, AND THEY CAN OCCUR IN LINEAR CLUSTER OR AS STREAK LESIONS. OLD LESIONS APPEAR ATROPHIC AND PUNCHED-OUT WITH VARIABLE AMOUNT OF PIGMENTS.

LEVEL OF RETINAL INVOLVEMENT: CHOROID, RPE

IVFA: EARLY HYPOFLUORESCENCE, LATE HYPERFLUORESCENCE.

ERG: NORMAL

PRESENTATIONS: DECREASE VA, PHOTOPSIA,

OCULAR FINDINGS: CME, CNVM.

SYSTEMIC FINDINGS: ONE STUDY SHOWED LINKAGE WITH EPV.

TREATMENT: STEROID.

OUTCOME: LESIONS HEAL. CME AND CNVM POSSIBLE COMPLICATION.

RECURRENCES OR EXACERBATIONS: YES

PROGRESSIVE SUBRETINAL FIBROSIS AND UVEITIS SYNDROME

CONSIDER TO BE SEVERE VARIANT OF MFC

AGE: 20-40

GENDER PREDOMINANCE:

UNILATERAL OR BILATERAL:

CELLS: CHRONIC VITREOUS CELLS

RETINAL FINDINGS: WHITE FIBROTIC SUBRETINAL LESION, WHICH ENLARGE AND COALESCE IN A PROGRESSIVE MANNER TO INVOLVE MOST OF THE RETINA AND CHOROID

LEVEL OF RETINAL INVOLVEMENT:

IVFA:

ERG:

PRESENTATIONS:

OCULAR FINDINGS:

SYSTEMIC FINDINGS:

TREATMENT: USUALLY NO RESPONSE TO TX AS THERE IS USUALLY PROGRESSION TO SUBRETINAL FIBROSIS OF THE MACULA. SOME TRIED SYSTEMIC STEROID OR CYTOTOXIC.

OUTCOME:

RECURRENCES OR EXACERBATIONS

PUNCTATE INNER CHOROIDOPATHY (PIC)

CONSIDER TO BE MILDER FORM OF MFC

AGE: YOUNG HEALTHY MYOPIC WOMAN

GENDER PREDOMINANCE: FEMALE

UNILATERAL OR BILATERAL: B>U

CELLS: LITTLE AC AND VITREOUS REACTION

RETINAL FINDINGS: SMALL YELLOW WHITE LESION, MAINLT IN POSTERIOR POLE

LEVEL OF RETINAL INVOLVEMENT: CHOROID, RPE

IVFA: EARLY HPOFLOURESCENCE THEN LATE HYPER

ERG:

PRESENTATIONS: BLURRED VISION, PHOTOPSIA, PARACENTRAL SCOTOMA

OCULAR FINDINGS: SEROUS RETINAL DETACHMENT, LESION HEAL TO FORM ATROPHIC SCAR OF VARIABLE PIGMENTATION. CNVM CAN OCCUR.

SYSTEMIC FINDINGS:

TREATMENT: UNNECESSARY UNLESS CNVM DEVELOP WHICH MAY NEED LASER OR SURGICAL TX.

OUTCOME:

RECURRENCES OR EXACERBATIONS: RECURRENCE CAN OCCUR.

POSTERIOR UVEITIS OF UNKNOWN CAUSES

ACUTE RETINAL PIGMENT EPITHELIALITIS (KJAR)

AGE: 20-50

GENDER PREDOMINANCE:

UNILATERAL OR BILATERAL: U>B

CELLS: NO

RETINAL FINDINGS: SMAL BROWN TO GRAY SPOTS IN THE INVOLVED MACULA , USUALLY BETWEEN 2 TO 4 SPOTS, OCCASIONALLY WHITE TO YELLOW HALO MAY SURROUND THE SPOTS

LEVEL OF RETINAL INVOLVEMENT: OUTER RETINA, RPE

IVFA: RING OF HYPER FLUORESCENCE SUROUNDING HYPOFLOURESCENCE LESION IN EARLY PHASE.

ERG: NORMAL

PRESENTATIONS: DECREASE VA WHICH CAN BE MINIMUM TO SEVERE

OCULAR FINDINGS:

SYSTEMIC FINDINGS: NON

TREATMENT: NOT NECESSARY

OUTCOME: RESOLUTION IS GRADUAL AND COMPLETE IN 6-12/52

RECURRENCES OR EXACERBATIONS: NO

ACUTE MACULAR NEURORETINOPATHY

AGE: 20-40

GENDER PREDOMINANCE: FEMALE

UNILATERAL OR BILATERAL: U OR B

CELLS: NO

RETINAL FINDINGS: RED OR BROWN WEDGE SHAPE OR CIRCULAR LESION

LEVEL OF RETINAL INVOLVEMENT: CONTROVERSIAL

IVFA:. NORMAL

ERG: NORMAL

PRESENTATIONS: DECREASE VA OR PARACENTRAL SCOTOMA

OCULAR FINDINGS: NONE

SYSTEMIC FINDINGS: POSSIBLE PRECEEDED BY VIRAL ILLNESS

TREATMENT: NOT NECESSARY

OUTCOME: RECOVERY WITH RESIDUAL PIGMENT CHANGES, MIGHT TAKE MONTHS

RECURRENCES OR EXACERBATIONS: RARE

UNILATERAL ACUTE IDIOPATHIC MACULOPATHY (UAIM)

AGE: 20 -50

GENDER PREDOMINANCE: NONE

UNILATERAL OR BILATERAL: U

CELLS: FEW VITREOUS CELLS

RETINAL FINDINGS: NEUROSENSORY DETACHMENT OF THE MACULAR AREA WITH RPE THICKENING, AND INTRARETINAL HEMORRHAGE.

LEVEL OF RETINAL INVOLVEMENT: RPE

IVFA: EARLY ALTERNATING AREA OF HYPER AND HYPO, LATE INTENSE HPERFLOURESCENCE DUE TO STAINING AND POOLING

ERG: NORMAL

PRESENTATIONS: SUDDEN SEVERE VISIAL LOSS

OCULAR FINDINGS:

SYSTEMIC FINDINGS: MIGHT PRECEEDED BY VIRAL ILLNESS.

TREATMENT: NOT NECESSARY

OUTCOME: EXCELLENT, COMPLETE RECOVERY, WITH REISDUAL BUUL’S EYE MACULOPATHY. RARELY CNVM CAN DEVELOP.

RECURRENCES OR EXACERBATIONS: NO

ACUTE ZONAL OCCULT OUTERRETINOPATHY (AZOOR)

AGE: 20-50

GENDER PREDOMINANCE: FEMALE

UNILATERAL OR BILATERAL: U AND B

CELLS: FEW VITREOUS CELLS

RETINAL FINDINGS: USUALLY FOUND LATER, AND CHARACTRISE BY NARROWING OF THE BLOOD VESSELES, AND DEPIGMETATION OF THE ZONE INVOLOVED.

LEVEL OF RETINAL INVOLVEMENT: OUTER RETINA, CHOROID.

IVFA: NORMAL IN ACUTE PHASE, IN CHRONIC STAGE IT SHOWES WINDOW DEFECT.

ERG: DECREASE A WAVE.

PRESENTATIONS: PHOTOPSIA, SCOTOMA CORRESPONDING TO AREA INVOLVED

OCULAR FINDINGS:

SYSTEMIC FINDINGS:

TREATMENT: STEROID AND ANTIVIRAL HAVE BEEN TRIED BUT DIDN’T CHANGE THE FIELD LOST.

OUTCOME: PATIENT USUALY END WITH VISUAL FIELD LOSS.

RECURRENCES OR EXACERBATIONS: ?

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