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DIAGNOSTIC CHALLENGES INDIAGNOSTIC CHALLENGES IN
RETINAL DISEASESRETINAL DISEASES
GEORGE KRANIAS, M.D.GEORGE KRANIAS, M.D.
Vitreoretinal Summer SchoolVitreoretinal Summer School
June 21, 2014June 21, 2014
OCULAR MANIFESTATIONS AS ANOCULAR MANIFESTATIONS AS AN
INITIAL SIGN OF SYSTEMIC DISEASEINITIAL SIGN OF SYSTEMIC DISEASE
BRIEF OVERVIEW OF 5 CASE HISTORIESBRIEF OVERVIEW OF 5 CASE HISTORIES
PATIENT HISTORYPATIENT HISTORY
PH: 43 yo WF c/o sudden onset of blurredPH: 43 yo WF c/o sudden onset of blurred
vision OS x1 day.vision OS x1 day.
PMH: Arthritis and multiple allergiesPMH: Arthritis and multiple allergies
EXAM: VA OD 20/30EXAM: VA OD 20/30
OS 20/400OS 20/400
IOP OD 18 mm HgIOP OD 18 mm Hg
OS 18 mm HgOS 18 mm Hg
BP 138/76 mm HgBP 138/76 mm Hg
LABORATORY FINDINGSLABORATORY FINDINGS
HGB: 6.5 LOWHGB: 6.5 LOW
HCT: 19.0 LOWHCT: 19.0 LOW
ANA: (+)ANA: (+)
SLE antibodiesSLE antibodies
NL 12.0-15.0NL 12.0-15.0
NL 35-42%NL 35-42%
TREATMENT COURSETREATMENT COURSE
BLOOD TRANSFUSIONBLOOD TRANSFUSION
PLAQUENIL RXPLAQUENIL RX
SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS
Autoimmune inflammatory diseaseAutoimmune inflammatory disease
Multiple organ involvementMultiple organ involvement
PolyarthritisPolyarthritis
Skin lesionsSkin lesions
Renal diseaseRenal disease
PericarditisPericarditis
HepatosplenomegalyHepatosplenomegaly
AnemiaAnemia
Neurological disorderNeurological disorder
SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS
OCULAR FINDINGSOCULAR FINDINGS::
Sjogren’s syndromeSjogren’s syndrome
ScleritisScleritis
RetinopathyRetinopathy
a. Retinal hemorrhagesa. Retinal hemorrhages
b. Cotton wool spotsb. Cotton wool spots
c. Retinal artery occlusionc. Retinal artery occlusion
d. Retinal vein occlusiond. Retinal vein occlusion
PATIENT HISTORYPATIENT HISTORY
PH: 69yr. W/M c/o increased difficultyPH: 69yr. W/M c/o increased difficulty
reading X1 moreading X1 mo
PMH: type II DM X12 yr, hypertension,PMH: type II DM X12 yr, hypertension,
prostate and testicular CAprostate and testicular CA
EXAM: VA OD 20/200 phniEXAM: VA OD 20/200 phni
VA OS 20/200 phniVA OS 20/200 phni
BP 130/70 mm HgBP 130/70 mm Hg
LABORATORYLABORATORY
INCREASED SERUMINCREASED SERUM
VISCOSITY 11.1VISCOSITY 11.1
HEMATOCRIT 15HEMATOCRIT 15
IgM PROTEIN 11,000IgM PROTEIN 11,000
NORMAL=2.0NORMAL=2.0
NORMAL=38-45NORMAL=38-45
TREATMENT COURSETREATMENT COURSE
CHEMOTHERAPY AGENTSCHEMOTHERAPY AGENTS
WALDENSTROM’S MACROGLOBULINEMIAWALDENSTROM’S MACROGLOBULINEMIA
LYMPHOPROLIFERATIVE DISORDERLYMPHOPROLIFERATIVE DISORDER
HIGH INTRAVASCULAR CONCENTRATION OFHIGH INTRAVASCULAR CONCENTRATION OF
ABNORMAL MONOCLONAL IgM PROTEINABNORMAL MONOCLONAL IgM PROTEIN
INCREASED BLOOD VISCOSITYINCREASED BLOOD VISCOSITY
INCREASED INTRAVASCULAR VOLUMEINCREASED INTRAVASCULAR VOLUME
RETINOPATHY IN 50% OF CASESRETINOPATHY IN 50% OF CASES
PATIENT HISTORYPATIENT HISTORY
CC: 27 yo WF c/o decreased VA x1CC: 27 yo WF c/o decreased VA x1
week, fatigue and not feeling well.week, fatigue and not feeling well.
PMH: Unremarkable.PMH: Unremarkable.
EXAM: VA OD CF @ 5 ft.EXAM: VA OD CF @ 5 ft.
OS 20/60OS 20/60
LABORATORY FINDINGSLABORATORY FINDINGS
WBC: 19.8 HIGHWBC: 19.8 HIGH
RBC: 1.13 LOWRBC: 1.13 LOW
HGB: 4.4 CRITICALHGB: 4.4 CRITICAL
HCT: 12.8 CRITICALHCT: 12.8 CRITICAL
PLT: 14.0 CRITICALPLT: 14.0 CRITICAL
NL 5.0-10.0 (X1000)NL 5.0-10.0 (X1000)
NL 4.2-5.4 (X10^6)NL 4.2-5.4 (X10^6)
NL 12.0-16.0NL 12.0-16.0
NL 36-46%NL 36-46%
NL 150-450 (X1000)NL 150-450 (X1000)
TREATMENT COURSETREATMENT COURSE
CHEMOTHERAPY AGENTSCHEMOTHERAPY AGENTS
PATIENT HISTORY
CC: 25 yo WF c/o “brown spot”CC: 25 yo WF c/o “brown spot”
centrally x2 weeks. Had similarcentrally x2 weeks. Had similar
episode 2 mo ago, attributed toepisode 2 mo ago, attributed to
optic neuritisoptic neuritis
PMH: UnremarkablePMH: Unremarkable
EXAM: VA OD CF @ 5 ft.EXAM: VA OD CF @ 5 ft.
OS 20/100OS 20/100
LABORATORY FINDINGSLABORATORY FINDINGS
WBC: 14.2WBC: 14.2
RBC: 2.72RBC: 2.72
HGB: 9.1HGB: 9.1
HCT: 26.5HCT: 26.5
BLASTS 81BLASTS 81
PATIENT HISTORYPATIENT HISTORY
CC:CC: 54 yo dermatologist developed54 yo dermatologist developed
scotoma OS while flying at 33,000scotoma OS while flying at 33,000
feet.feet.
PMH: Unremarkable.PMH: Unremarkable.
EXAM: VA OD 20/60EXAM: VA OD 20/60
OS 20/80OS 20/80
LABORATORY FINDINGSLABORATORY FINDINGS
WBC: 19.8WBC: 19.8
RBC: 1.13RBC: 1.13
HGB: 4.4HGB: 4.4
HCT: 12.8HCT: 12.8
PLT: 14.0PLT: 14.0
BLASTS: 80BLASTS: 80
LEUKEMIALEUKEMIA
Group of blood disorders with abnormal,Group of blood disorders with abnormal,
unregulated proliferation of WBC.unregulated proliferation of WBC.
Acute (80% children) or chronicAcute (80% children) or chronic
Lymphoid: B-cell and T-cellLymphoid: B-cell and T-cell
MyeloidMyeloid
Accounts for 3.7% of cancer deathsAccounts for 3.7% of cancer deaths
LEUKEMIALEUKEMIA
OCULAR MANIFESTATIONSOCULAR MANIFESTATIONS::
50-80% ocular involvement50-80% ocular involvement
Leukemic infiltratesLeukemic infiltrates
Secondary complications related to:Secondary complications related to:
-anemia-anemia
-thrombocytopenia-thrombocytopenia
-hyperviscosity-hyperviscosity
Opportunistic infectionsOpportunistic infections
RETINOPATHY AS AN INITIAL SIGN OF
SYSTEMIC DISEASE
SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS
WALDENSTROM’S MACROGLOBULINEMIAWALDENSTROM’S MACROGLOBULINEMIA
LEUKEMIALEUKEMIA
PATIENT HISTORYPATIENT HISTORY
60 yo WF60 yo WF
Loss of vision ODLoss of vision OD
Eyeball achinessEyeball achiness
Red and puffy ODRed and puffy OD
PMH: Hysterectomy, hernia, hypertension,PMH: Hysterectomy, hernia, hypertension,
skin Caskin Ca
PATIENT HISTORYPATIENT HISTORY contcont..
EXAMINATIONEXAMINATION
OD: 20/60, OS: 20/20OD: 20/60, OS: 20/20
Massive sclerochoroidal swelling withMassive sclerochoroidal swelling with
localizedlocalized orbital pseudotumor OD.orbital pseudotumor OD.
Proptosis OD.Proptosis OD.
Prominent, elevatedProminent, elevated
mass at themass at the
sclerochoroidal levelsclerochoroidal level
Acoustically solidAcoustically solid
Shallow retrobulbarShallow retrobulbar
echolucent spaceecholucent space
A-scan: high internalA-scan: high internal
reflectivityreflectivity
Pathology ReportPathology Report
Scleral tissue: mild toScleral tissue: mild to
moderately infiltratedmoderately infiltrated
with chronicwith chronic
inflammatory cells.inflammatory cells.
Evidence of scleralEvidence of scleral
necrosis.necrosis.
No evidence ofNo evidence of
tumor.tumor.
5 years later5 years later
GIANT NODULAR POSTERIORGIANT NODULAR POSTERIOR
SCLERITIS SIMULATINGSCLERITIS SIMULATING
CHOROIDAL MELANOMACHOROIDAL MELANOMA
Review of 400 patients with lesionsReview of 400 patients with lesions
clinically simulating choroidalclinically simulating choroidal
melanomamelanoma
Suspicious choroidal nevus 27%Suspicious choroidal nevus 27%
Age-related macular degeneration 13%Age-related macular degeneration 13%
Age-related extramacular degen. 11%Age-related extramacular degen. 11%
Congenital hypertrophy of RPE 10%Congenital hypertrophy of RPE 10%
Choroidal hemangioma 8%Choroidal hemangioma 8%
Nodular scleritisNodular scleritis 1.5%1.5%
Shields JA, Augsburger JJ, Brown GC,Shields JA, Augsburger JJ, Brown GC,
Stephens RF: The differential diagnosis ofStephens RF: The differential diagnosis of
posterior uveal malanoma. Ophthalmology,posterior uveal malanoma. Ophthalmology,
1980; 87: 518-5221980; 87: 518-522
NODULAR POSTERIOR SCLERITISNODULAR POSTERIOR SCLERITIS
Inflammatory sclerochoroidal massInflammatory sclerochoroidal mass
Systemic disease in 10% of patientsSystemic disease in 10% of patients
UnilateralUnilateral
AdultsAdults
Blurred vision with pain and red eyeBlurred vision with pain and red eye
Exudative retinal detachmentExudative retinal detachment
A/B scan, CT, MRI helpfulA/B scan, CT, MRI helpful
Systemic corticosteroid therapySystemic corticosteroid therapy
PATIENT HISTORYPATIENT HISTORY
27 yo, HEALTHY WM27 yo, HEALTHY WM
CC: BLURRED VA OD X6 WEEKSCC: BLURRED VA OD X6 WEEKS
PMH: BELL’S PALSYPMH: BELL’S PALSY
VA: OD 20/80, OS 20/40.VA: OD 20/80, OS 20/40.
LABORATORY FINDINGSLABORATORY FINDINGS
CBC: NLCBC: NL
ESR: 2ESR: 2
ACE: NLACE: NL
LYSOZYME: NLLYSOZYME: NL
LYME TITER: NLLYME TITER: NL
ANA: SL. POSITIVEANA: SL. POSITIVE
(1:80)(1:80)
HEPATITIS A,B,C: NEGHEPATITIS A,B,C: NEG
ANTICARDIOLIPIN:ANTICARDIOLIPIN:
NEGNEG
C-ANCA, P-ANCA: NEGC-ANCA, P-ANCA: NEG
SEROLOGY: NEGSEROLOGY: NEG
FOLLOW-UP (6 MONTHS)FOLLOW-UP (6 MONTHS)
CC: PROGRESSIVE LOSS OF VISIONCC: PROGRESSIVE LOSS OF VISION
VA: OD 20/400, OS 20/80VA: OD 20/400, OS 20/80
FUNDUS: SEVERE ONH EDEMAFUNDUS: SEVERE ONH EDEMA
PROGRESSIVE EXUDATIONPROGRESSIVE EXUDATION
DX: R/O OPTIC NEURITIS, MENINGIOMADX: R/O OPTIC NEURITIS, MENINGIOMA
RX: SYSTEMIC STEROIDSRX: SYSTEMIC STEROIDS
FOLLOW-UP (1 YEAR)FOLLOW-UP (1 YEAR)
VA: OD NLP, OS 20/40VA: OD NLP, OS 20/40
SLE: SEVERE RUBEOSIS IRIDIS ODSLE: SEVERE RUBEOSIS IRIDIS OD
FUNDUS: ONH EDEMA, EXUDATIONFUNDUS: ONH EDEMA, EXUDATION
RX: LASER PHOTOCOAGULATIONRX: LASER PHOTOCOAGULATION
NEURORADIOGRAPHIC FINDINGSNEURORADIOGRAPHIC FINDINGS
CHEST X-RAY:CHEST X-RAY:
NEGNEG
MRI: ENLARGEDMRI: ENLARGED
RIGHT OPTICRIGHT OPTIC
NERVENERVE
May 2001May 2001 August 2002August 2002
OPTIC NERVE BIOPSYOPTIC NERVE BIOPSY
Dense collagenous stroma with inflammatory infiltrates ofDense collagenous stroma with inflammatory infiltrates of
plasma cells, lymphocytes, histiocytes and neutrophilsplasma cells, lymphocytes, histiocytes and neutrophils
IDIOPATHIC SCLEROSINGIDIOPATHIC SCLEROSING
INFLAMMATION OF THE ORBITINFLAMMATION OF THE ORBIT
Autoimmune diseaseAutoimmune disease
Progressive infiltrative fibrosisProgressive infiltrative fibrosis
Chronic courseChronic course
Visually disableVisually disable
Treatment unsatisfactoryTreatment unsatisfactory
PATIENT HISTORYPATIENT HISTORY
50 y/o healthy, W/F50 y/o healthy, W/F
C/O: blurred and distorted VA OD x 2-3 yrs.C/O: blurred and distorted VA OD x 2-3 yrs.
VA :VA :
OD: 20/80OD: 20/80
OS: 20/20OS: 20/20
CHOROIDAL OSTEOMACHOROIDAL OSTEOMA
Mostly womenMostly women
Mature boneMature bone
Well defined round or ovalWell defined round or oval
Orange, white or white-yellowOrange, white or white-yellow
Juxtapapillary and macular areaJuxtapapillary and macular area
Bilateral 20%, most often young womenBilateral 20%, most often young women
Ultrasonography: calcificationUltrasonography: calcification
Patient HistoryPatient History
14 y/o w/f, mother noticed that pupil was14 y/o w/f, mother noticed that pupil was
turning white over the past few months.turning white over the past few months.
PMH : unremarkablePMH : unremarkable
VA : OD CFVA : OD CF
OS 20/20OS 20/20
COATS’ DISEASECOATS’ DISEASE
Retinal telangiectasis with exudative RDRetinal telangiectasis with exudative RD
Unilateral, male to female 3 to 1Unilateral, male to female 3 to 1
Young children under age 16Young children under age 16
Poor vision, strabismus or leukocoriaPoor vision, strabismus or leukocoria
Subretinal yellowish to greenish exudationSubretinal yellowish to greenish exudation
Retinal telangiectasisRetinal telangiectasis
Management: photocoagulation,Management: photocoagulation,
cryotherapy, vitrectomycryotherapy, vitrectomy
PATIENT HISTORYPATIENT HISTORY
PH: 19 WM college student who reports decreasedPH: 19 WM college student who reports decreased
vision OU x 3 wks. Recent cold/flu-likevision OU x 3 wks. Recent cold/flu-like
symptoms.symptoms.
PMH: UnremarkablePMH: Unremarkable
Exam:Exam: ODOD 20/200 phni20/200 phni IOPIOP 1313
OSOS 20/200 phni20/200 phni 1313
BPBP 132/78132/78
2 Week F/U at Univ. of Kentucky2 Week F/U at Univ. of Kentucky
Medical CenterMedical Center
Complains of headachesComplains of headaches
Blood pressure 240/150 on multipleBlood pressure 240/150 on multiple
checkschecks
Positive ANAPositive ANA
SED rate 55SED rate 55
MRI (head and orbits) NormalMRI (head and orbits) Normal
Hypertensive RetinopathyHypertensive Retinopathy
ESSENTIAL HYPERTENSIONESSENTIAL HYPERTENSION
(no known cause)(no known cause)
SECONDARY HYPERTENSIONSECONDARY HYPERTENSION
Preeclampsia/eclampsiaPreeclampsia/eclampsia
kidney diseasekidney disease
adrenal diseaseadrenal disease
coarctation of the aortacoarctation of the aorta
PATIENT HISTORYPATIENT HISTORY
PH: 12 yr. WM c/o decreased vision OD x 5PH: 12 yr. WM c/o decreased vision OD x 5
days. Flu-like symptoms 1 week prior.days. Flu-like symptoms 1 week prior.
PMH: HealthyPMH: Healthy
EXAM:EXAM: ODOD CF @ 4 ft.CF @ 4 ft. IOPIOP 1212
OSOS 20/2020/20 1212
Serologic Work UpSerologic Work Up
HLA B27HLA B27 NEGATIVENEGATIVE
FTA-ABSFTA-ABS NON-REACTIVENON-REACTIVE
ANAANA NEGATIVENEGATIVE
RA quant.RA quant. NORMALNORMAL
RPRRPR NON-REACTIVENON-REACTIVE
HIVHIV NEGATIVENEGATIVE
ACEACE NORMALNORMAL
CAT SCRATCH AB-RCAT SCRATCH AB-R POSITIVEPOSITIVE
SED RATESED RATE 6161
CXRCXR NORMALNORMAL
Acute Neuroretinitis Secondary toAcute Neuroretinitis Secondary to
Cat Scratch DiseaseCat Scratch Disease
Bartonella henselaeBartonella henselae is the causativeis the causative
organismorganism
VA loss may be mild or severeVA loss may be mild or severe
33% of cases may be bilateral33% of cases may be bilateral
> 90% improve to 20/40 within 8 weeks> 90% improve to 20/40 within 8 weeks
and 20/20 within 6 monthsand 20/20 within 6 months
Small subgroup left with severe visual lossSmall subgroup left with severe visual loss
and associated optic atrophyand associated optic atrophy
TreatmentTreatment
No proven treatmentNo proven treatment
Periocular and systemic corticosteriodsPeriocular and systemic corticosteriods
Antibiotic therapyAntibiotic therapy
No difference in treated vs. untreatedNo difference in treated vs. untreated
patientspatients
PATIENT HISTORYPATIENT HISTORY
PH: 50yr. WF who reports distortion andPH: 50yr. WF who reports distortion and
occasional blurred vision OU x 6 yrs.occasional blurred vision OU x 6 yrs.
PMH: Breast lumpectomy (benign), traumaPMH: Breast lumpectomy (benign), trauma
(auto accident) and Bell’s palsy.(auto accident) and Bell’s palsy.
POH: Glaucoma OU.POH: Glaucoma OU.
EXAM:EXAM: 20/40 phni20/40 phni IOPIOP 1717
20/60 phni20/60 phni 1717
BPBP 140/78140/78
Geographic Helicoid PeripapillaryGeographic Helicoid Peripapillary
ChoroiditisChoroiditis
(Serpiginous choroiditis)(Serpiginous choroiditis)
• CHRONIC RECURRING CHOROIDITISCHRONIC RECURRING CHOROIDITIS
• MULTIFOCALMULTIFOCAL
• INNER CHOROID & RPEINNER CHOROID & RPE
• INFLAMMATION vs. SRNVINFLAMMATION vs. SRNV
TreatmentTreatment
Steroids (oral or periocular injection)Steroids (oral or periocular injection)
Photocoagulation for SRNVMPhotocoagulation for SRNVM
Anti - VEGFAnti - VEGF
PATIENT HISTORYPATIENT HISTORY
25 y/o , w/f was seen for the 125 y/o , w/f was seen for the 1stst
time on Feb.time on Feb.
1616thth
20102010
c/o intermittent blurring of central visionc/o intermittent blurring of central vision
which started 2 mo ago and last about 4-5which started 2 mo ago and last about 4-5
hours.hours.
Her visual symptoms increased recentlyHer visual symptoms increased recently
and last longer.and last longer.
PMH: unremarkable.PMH: unremarkable.
Case 4
ExaminationExamination
Visual acuityVisual acuity
– OD 20/60OD 20/60 OD 16 mm HgOD 16 mm Hg
– OS 20/40OS 20/40 OS 14 mm HgOS 14 mm Hg
SLE:SLE: a few vitreous cells.a few vitreous cells.
Fundus:Fundus:
Hyperemic optic nerves, OUHyperemic optic nerves, OU
Cystoid macular edema, OUCystoid macular edema, OU
FANG:FANG: CME with ON leakage, OUCME with ON leakage, OU
OCT: Thickened neuroepithelium with largeOCT: Thickened neuroepithelium with large
cysticcystic changes, OUchanges, OU
Feb. 16Feb. 16thth
20102010
Feb. 16Feb. 16thth
20102010
OD
OS
Laboratory Work-upLaboratory Work-up
CBCCBC NormalNormal
ESRESR NormalNormal
CRPCRP NormalNormal
RFRF NormalNormal
ANAANA NormalNormal
ACEACE NormalNormal
RPRRPR Non-reactiveNon-reactive
FTA-AbsFTA-Abs Non-reactiveNon-reactive
CXRCXR NormalNormal
MRIMRI NormalNormal
Feb 23Feb 23rdrd
20102010
No visual complaintsNo visual complaints
VAVA
– ODOD 20/2020/20
– OSOS 20/2020/20
Fundus:Fundus:
– Macula and optic nerve flat OUMacula and optic nerve flat OU
OCT:OCT: NormalNormal
Feb. 23Feb. 23rdrd
20102010
OD
OS
March 16March 16thth
20102010
C/O blurred vision since March 3C/O blurred vision since March 3rdrd
OUOU
On further questioning, she reported that allOn further questioning, she reported that all
episodes of blurred vision would start with eachepisodes of blurred vision would start with each
menstrual period (21 days), last the first half ofmenstrual period (21 days), last the first half of
each cycle, and resolve at the end ofeach cycle, and resolve at the end of
menstruation. No oral contraceptionmenstruation. No oral contraception
VA: 20/80 OUVA: 20/80 OU
Fundus: CME with congested ON, OU.Fundus: CME with congested ON, OU.
OCT: CMEOCT: CME
BibliographyBibliography
Cyclic Presentation of CME: A. Assi, J. Hickman Casey,Cyclic Presentation of CME: A. Assi, J. Hickman Casey,
et al.: Acta Ophthalmol. Scand. 1998: 76: 245-246et al.: Acta Ophthalmol. Scand. 1998: 76: 245-246
– Presented the case of a 28 y/o lady with unilateral pars planitisPresented the case of a 28 y/o lady with unilateral pars planitis
and associated CME in whom visual symptoms fluctuatedand associated CME in whom visual symptoms fluctuated
regularly during menstrual cycles.regularly during menstrual cycles.
Cyclical central serous chorloretinopathy associatedCyclical central serous chorloretinopathy associated
with cystoid macular oedema.with cystoid macular oedema. Birchall W, Charles SJ,Birchall W, Charles SJ,
Buckler HM. BJO 2001 Jun;85(6):756-8.Buckler HM. BJO 2001 Jun;85(6):756-8.
– CME resolved on oral contraceptives.CME resolved on oral contraceptives.
Consultations and Work-upConsultations and Work-up
GynecologicalGynecological NormalNormal
EndocrineEndocrine NormalNormal
ImmunologicalImmunological NormalNormal
ClottingClotting APC, R 0.6APC, R 0.6
TreatmentTreatment
August 20August 20thth
, 2010., 2010.
– Oral contraceptive pill Yasminelle.Oral contraceptive pill Yasminelle.
– Her cycle from 21 days changed to 28 daysHer cycle from 21 days changed to 28 days
with initial improvement of her visualwith initial improvement of her visual
symptoms for 2-3 months.symptoms for 2-3 months.
November 11November 11thth
, 2010, 2010
– Started on stronger contraceptive pill, Yasmin,Started on stronger contraceptive pill, Yasmin,
with subjective visual improvement.with subjective visual improvement.
Dec 20Dec 20thth
20102010
Reports visual improvementReports visual improvement
On oral contraceptives for 4 monthsOn oral contraceptives for 4 months
VAVA
– OD 20/60OD 20/60
– OS 20/40OS 20/40
Dec 20Dec 20thth
20102010
OD
OS
Beginning of Jan. 2011 developed progressiveBeginning of Jan. 2011 developed progressive
painful ophthalmoplegia OD which was attributed topainful ophthalmoplegia OD which was attributed to
a large brain mass. It was not present at her initiala large brain mass. It was not present at her initial
evaluation by MRI.evaluation by MRI.
Biopsy revealed inflammatory compounds.Biopsy revealed inflammatory compounds.
Was treated with IV antibiotics with completeWas treated with IV antibiotics with complete
regression of her ophthalmoplegiaregression of her ophthalmoplegia..
10/02/2011
27/05/2013
26/04/2011
Happy EndingHappy Ending
Patient got married and is expectingPatient got married and is expecting
a baby soon.a baby soon.
She is on no medications.She is on no medications.
No visual complaints.No visual complaints.
VA: 20/20 OU.VA: 20/20 OU.
QuestionsQuestions
Etiology of Cyclic Presentation of CME.Etiology of Cyclic Presentation of CME.
Treatment?Treatment?
If anyone in the audience has comeIf anyone in the audience has come
across with a similar case, please shareacross with a similar case, please share..
THANK YOUTHANK YOU

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Kranias diagnostic challenges in retinal diseases 06 20 14

  • 1. DIAGNOSTIC CHALLENGES INDIAGNOSTIC CHALLENGES IN RETINAL DISEASESRETINAL DISEASES GEORGE KRANIAS, M.D.GEORGE KRANIAS, M.D. Vitreoretinal Summer SchoolVitreoretinal Summer School June 21, 2014June 21, 2014
  • 2. OCULAR MANIFESTATIONS AS ANOCULAR MANIFESTATIONS AS AN INITIAL SIGN OF SYSTEMIC DISEASEINITIAL SIGN OF SYSTEMIC DISEASE BRIEF OVERVIEW OF 5 CASE HISTORIESBRIEF OVERVIEW OF 5 CASE HISTORIES
  • 3. PATIENT HISTORYPATIENT HISTORY PH: 43 yo WF c/o sudden onset of blurredPH: 43 yo WF c/o sudden onset of blurred vision OS x1 day.vision OS x1 day. PMH: Arthritis and multiple allergiesPMH: Arthritis and multiple allergies EXAM: VA OD 20/30EXAM: VA OD 20/30 OS 20/400OS 20/400 IOP OD 18 mm HgIOP OD 18 mm Hg OS 18 mm HgOS 18 mm Hg BP 138/76 mm HgBP 138/76 mm Hg
  • 4.
  • 5.
  • 6. LABORATORY FINDINGSLABORATORY FINDINGS HGB: 6.5 LOWHGB: 6.5 LOW HCT: 19.0 LOWHCT: 19.0 LOW ANA: (+)ANA: (+) SLE antibodiesSLE antibodies NL 12.0-15.0NL 12.0-15.0 NL 35-42%NL 35-42%
  • 7. TREATMENT COURSETREATMENT COURSE BLOOD TRANSFUSIONBLOOD TRANSFUSION PLAQUENIL RXPLAQUENIL RX
  • 8.
  • 9. SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS Autoimmune inflammatory diseaseAutoimmune inflammatory disease Multiple organ involvementMultiple organ involvement PolyarthritisPolyarthritis Skin lesionsSkin lesions Renal diseaseRenal disease PericarditisPericarditis HepatosplenomegalyHepatosplenomegaly AnemiaAnemia Neurological disorderNeurological disorder
  • 10. SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS OCULAR FINDINGSOCULAR FINDINGS:: Sjogren’s syndromeSjogren’s syndrome ScleritisScleritis RetinopathyRetinopathy a. Retinal hemorrhagesa. Retinal hemorrhages b. Cotton wool spotsb. Cotton wool spots c. Retinal artery occlusionc. Retinal artery occlusion d. Retinal vein occlusiond. Retinal vein occlusion
  • 11. PATIENT HISTORYPATIENT HISTORY PH: 69yr. W/M c/o increased difficultyPH: 69yr. W/M c/o increased difficulty reading X1 moreading X1 mo PMH: type II DM X12 yr, hypertension,PMH: type II DM X12 yr, hypertension, prostate and testicular CAprostate and testicular CA EXAM: VA OD 20/200 phniEXAM: VA OD 20/200 phni VA OS 20/200 phniVA OS 20/200 phni BP 130/70 mm HgBP 130/70 mm Hg
  • 12.
  • 13.
  • 14. LABORATORYLABORATORY INCREASED SERUMINCREASED SERUM VISCOSITY 11.1VISCOSITY 11.1 HEMATOCRIT 15HEMATOCRIT 15 IgM PROTEIN 11,000IgM PROTEIN 11,000 NORMAL=2.0NORMAL=2.0 NORMAL=38-45NORMAL=38-45
  • 16.
  • 17. WALDENSTROM’S MACROGLOBULINEMIAWALDENSTROM’S MACROGLOBULINEMIA LYMPHOPROLIFERATIVE DISORDERLYMPHOPROLIFERATIVE DISORDER HIGH INTRAVASCULAR CONCENTRATION OFHIGH INTRAVASCULAR CONCENTRATION OF ABNORMAL MONOCLONAL IgM PROTEINABNORMAL MONOCLONAL IgM PROTEIN INCREASED BLOOD VISCOSITYINCREASED BLOOD VISCOSITY INCREASED INTRAVASCULAR VOLUMEINCREASED INTRAVASCULAR VOLUME RETINOPATHY IN 50% OF CASESRETINOPATHY IN 50% OF CASES
  • 18. PATIENT HISTORYPATIENT HISTORY CC: 27 yo WF c/o decreased VA x1CC: 27 yo WF c/o decreased VA x1 week, fatigue and not feeling well.week, fatigue and not feeling well. PMH: Unremarkable.PMH: Unremarkable. EXAM: VA OD CF @ 5 ft.EXAM: VA OD CF @ 5 ft. OS 20/60OS 20/60
  • 19.
  • 20.
  • 21. LABORATORY FINDINGSLABORATORY FINDINGS WBC: 19.8 HIGHWBC: 19.8 HIGH RBC: 1.13 LOWRBC: 1.13 LOW HGB: 4.4 CRITICALHGB: 4.4 CRITICAL HCT: 12.8 CRITICALHCT: 12.8 CRITICAL PLT: 14.0 CRITICALPLT: 14.0 CRITICAL NL 5.0-10.0 (X1000)NL 5.0-10.0 (X1000) NL 4.2-5.4 (X10^6)NL 4.2-5.4 (X10^6) NL 12.0-16.0NL 12.0-16.0 NL 36-46%NL 36-46% NL 150-450 (X1000)NL 150-450 (X1000)
  • 23.
  • 24.
  • 25. PATIENT HISTORY CC: 25 yo WF c/o “brown spot”CC: 25 yo WF c/o “brown spot” centrally x2 weeks. Had similarcentrally x2 weeks. Had similar episode 2 mo ago, attributed toepisode 2 mo ago, attributed to optic neuritisoptic neuritis PMH: UnremarkablePMH: Unremarkable EXAM: VA OD CF @ 5 ft.EXAM: VA OD CF @ 5 ft. OS 20/100OS 20/100
  • 26.
  • 27.
  • 28.
  • 29. LABORATORY FINDINGSLABORATORY FINDINGS WBC: 14.2WBC: 14.2 RBC: 2.72RBC: 2.72 HGB: 9.1HGB: 9.1 HCT: 26.5HCT: 26.5 BLASTS 81BLASTS 81
  • 30. PATIENT HISTORYPATIENT HISTORY CC:CC: 54 yo dermatologist developed54 yo dermatologist developed scotoma OS while flying at 33,000scotoma OS while flying at 33,000 feet.feet. PMH: Unremarkable.PMH: Unremarkable. EXAM: VA OD 20/60EXAM: VA OD 20/60 OS 20/80OS 20/80
  • 31.
  • 32.
  • 33.
  • 34.
  • 35. LABORATORY FINDINGSLABORATORY FINDINGS WBC: 19.8WBC: 19.8 RBC: 1.13RBC: 1.13 HGB: 4.4HGB: 4.4 HCT: 12.8HCT: 12.8 PLT: 14.0PLT: 14.0 BLASTS: 80BLASTS: 80
  • 36.
  • 37. LEUKEMIALEUKEMIA Group of blood disorders with abnormal,Group of blood disorders with abnormal, unregulated proliferation of WBC.unregulated proliferation of WBC. Acute (80% children) or chronicAcute (80% children) or chronic Lymphoid: B-cell and T-cellLymphoid: B-cell and T-cell MyeloidMyeloid Accounts for 3.7% of cancer deathsAccounts for 3.7% of cancer deaths
  • 38. LEUKEMIALEUKEMIA OCULAR MANIFESTATIONSOCULAR MANIFESTATIONS:: 50-80% ocular involvement50-80% ocular involvement Leukemic infiltratesLeukemic infiltrates Secondary complications related to:Secondary complications related to: -anemia-anemia -thrombocytopenia-thrombocytopenia -hyperviscosity-hyperviscosity Opportunistic infectionsOpportunistic infections
  • 39. RETINOPATHY AS AN INITIAL SIGN OF SYSTEMIC DISEASE SYSTEMIC LUPUS ERYTHEMATOSUSSYSTEMIC LUPUS ERYTHEMATOSUS WALDENSTROM’S MACROGLOBULINEMIAWALDENSTROM’S MACROGLOBULINEMIA LEUKEMIALEUKEMIA
  • 40. PATIENT HISTORYPATIENT HISTORY 60 yo WF60 yo WF Loss of vision ODLoss of vision OD Eyeball achinessEyeball achiness Red and puffy ODRed and puffy OD PMH: Hysterectomy, hernia, hypertension,PMH: Hysterectomy, hernia, hypertension, skin Caskin Ca
  • 41.
  • 42. PATIENT HISTORYPATIENT HISTORY contcont.. EXAMINATIONEXAMINATION OD: 20/60, OS: 20/20OD: 20/60, OS: 20/20 Massive sclerochoroidal swelling withMassive sclerochoroidal swelling with localizedlocalized orbital pseudotumor OD.orbital pseudotumor OD. Proptosis OD.Proptosis OD.
  • 43.
  • 44.
  • 45. Prominent, elevatedProminent, elevated mass at themass at the sclerochoroidal levelsclerochoroidal level Acoustically solidAcoustically solid Shallow retrobulbarShallow retrobulbar echolucent spaceecholucent space A-scan: high internalA-scan: high internal reflectivityreflectivity
  • 46.
  • 47. Pathology ReportPathology Report Scleral tissue: mild toScleral tissue: mild to moderately infiltratedmoderately infiltrated with chronicwith chronic inflammatory cells.inflammatory cells. Evidence of scleralEvidence of scleral necrosis.necrosis. No evidence ofNo evidence of tumor.tumor.
  • 48.
  • 49. 5 years later5 years later
  • 50. GIANT NODULAR POSTERIORGIANT NODULAR POSTERIOR SCLERITIS SIMULATINGSCLERITIS SIMULATING CHOROIDAL MELANOMACHOROIDAL MELANOMA
  • 51. Review of 400 patients with lesionsReview of 400 patients with lesions clinically simulating choroidalclinically simulating choroidal melanomamelanoma Suspicious choroidal nevus 27%Suspicious choroidal nevus 27% Age-related macular degeneration 13%Age-related macular degeneration 13% Age-related extramacular degen. 11%Age-related extramacular degen. 11% Congenital hypertrophy of RPE 10%Congenital hypertrophy of RPE 10% Choroidal hemangioma 8%Choroidal hemangioma 8% Nodular scleritisNodular scleritis 1.5%1.5% Shields JA, Augsburger JJ, Brown GC,Shields JA, Augsburger JJ, Brown GC, Stephens RF: The differential diagnosis ofStephens RF: The differential diagnosis of posterior uveal malanoma. Ophthalmology,posterior uveal malanoma. Ophthalmology, 1980; 87: 518-5221980; 87: 518-522
  • 52. NODULAR POSTERIOR SCLERITISNODULAR POSTERIOR SCLERITIS Inflammatory sclerochoroidal massInflammatory sclerochoroidal mass Systemic disease in 10% of patientsSystemic disease in 10% of patients UnilateralUnilateral AdultsAdults Blurred vision with pain and red eyeBlurred vision with pain and red eye Exudative retinal detachmentExudative retinal detachment A/B scan, CT, MRI helpfulA/B scan, CT, MRI helpful Systemic corticosteroid therapySystemic corticosteroid therapy
  • 53. PATIENT HISTORYPATIENT HISTORY 27 yo, HEALTHY WM27 yo, HEALTHY WM CC: BLURRED VA OD X6 WEEKSCC: BLURRED VA OD X6 WEEKS PMH: BELL’S PALSYPMH: BELL’S PALSY VA: OD 20/80, OS 20/40.VA: OD 20/80, OS 20/40.
  • 54.
  • 55. LABORATORY FINDINGSLABORATORY FINDINGS CBC: NLCBC: NL ESR: 2ESR: 2 ACE: NLACE: NL LYSOZYME: NLLYSOZYME: NL LYME TITER: NLLYME TITER: NL ANA: SL. POSITIVEANA: SL. POSITIVE (1:80)(1:80) HEPATITIS A,B,C: NEGHEPATITIS A,B,C: NEG ANTICARDIOLIPIN:ANTICARDIOLIPIN: NEGNEG C-ANCA, P-ANCA: NEGC-ANCA, P-ANCA: NEG SEROLOGY: NEGSEROLOGY: NEG
  • 56. FOLLOW-UP (6 MONTHS)FOLLOW-UP (6 MONTHS) CC: PROGRESSIVE LOSS OF VISIONCC: PROGRESSIVE LOSS OF VISION VA: OD 20/400, OS 20/80VA: OD 20/400, OS 20/80 FUNDUS: SEVERE ONH EDEMAFUNDUS: SEVERE ONH EDEMA PROGRESSIVE EXUDATIONPROGRESSIVE EXUDATION DX: R/O OPTIC NEURITIS, MENINGIOMADX: R/O OPTIC NEURITIS, MENINGIOMA RX: SYSTEMIC STEROIDSRX: SYSTEMIC STEROIDS
  • 57.
  • 58. FOLLOW-UP (1 YEAR)FOLLOW-UP (1 YEAR) VA: OD NLP, OS 20/40VA: OD NLP, OS 20/40 SLE: SEVERE RUBEOSIS IRIDIS ODSLE: SEVERE RUBEOSIS IRIDIS OD FUNDUS: ONH EDEMA, EXUDATIONFUNDUS: ONH EDEMA, EXUDATION RX: LASER PHOTOCOAGULATIONRX: LASER PHOTOCOAGULATION
  • 59. NEURORADIOGRAPHIC FINDINGSNEURORADIOGRAPHIC FINDINGS CHEST X-RAY:CHEST X-RAY: NEGNEG MRI: ENLARGEDMRI: ENLARGED RIGHT OPTICRIGHT OPTIC NERVENERVE
  • 60.
  • 61. May 2001May 2001 August 2002August 2002
  • 62. OPTIC NERVE BIOPSYOPTIC NERVE BIOPSY Dense collagenous stroma with inflammatory infiltrates ofDense collagenous stroma with inflammatory infiltrates of plasma cells, lymphocytes, histiocytes and neutrophilsplasma cells, lymphocytes, histiocytes and neutrophils
  • 63. IDIOPATHIC SCLEROSINGIDIOPATHIC SCLEROSING INFLAMMATION OF THE ORBITINFLAMMATION OF THE ORBIT Autoimmune diseaseAutoimmune disease Progressive infiltrative fibrosisProgressive infiltrative fibrosis Chronic courseChronic course Visually disableVisually disable Treatment unsatisfactoryTreatment unsatisfactory
  • 64. PATIENT HISTORYPATIENT HISTORY 50 y/o healthy, W/F50 y/o healthy, W/F C/O: blurred and distorted VA OD x 2-3 yrs.C/O: blurred and distorted VA OD x 2-3 yrs. VA :VA : OD: 20/80OD: 20/80 OS: 20/20OS: 20/20
  • 65.
  • 66.
  • 67.
  • 68.
  • 69.
  • 70. CHOROIDAL OSTEOMACHOROIDAL OSTEOMA Mostly womenMostly women Mature boneMature bone Well defined round or ovalWell defined round or oval Orange, white or white-yellowOrange, white or white-yellow Juxtapapillary and macular areaJuxtapapillary and macular area Bilateral 20%, most often young womenBilateral 20%, most often young women Ultrasonography: calcificationUltrasonography: calcification
  • 71. Patient HistoryPatient History 14 y/o w/f, mother noticed that pupil was14 y/o w/f, mother noticed that pupil was turning white over the past few months.turning white over the past few months. PMH : unremarkablePMH : unremarkable VA : OD CFVA : OD CF OS 20/20OS 20/20
  • 72.
  • 73.
  • 74.
  • 75.
  • 76.
  • 77. COATS’ DISEASECOATS’ DISEASE Retinal telangiectasis with exudative RDRetinal telangiectasis with exudative RD Unilateral, male to female 3 to 1Unilateral, male to female 3 to 1 Young children under age 16Young children under age 16 Poor vision, strabismus or leukocoriaPoor vision, strabismus or leukocoria Subretinal yellowish to greenish exudationSubretinal yellowish to greenish exudation Retinal telangiectasisRetinal telangiectasis Management: photocoagulation,Management: photocoagulation, cryotherapy, vitrectomycryotherapy, vitrectomy
  • 78. PATIENT HISTORYPATIENT HISTORY PH: 19 WM college student who reports decreasedPH: 19 WM college student who reports decreased vision OU x 3 wks. Recent cold/flu-likevision OU x 3 wks. Recent cold/flu-like symptoms.symptoms. PMH: UnremarkablePMH: Unremarkable Exam:Exam: ODOD 20/200 phni20/200 phni IOPIOP 1313 OSOS 20/200 phni20/200 phni 1313 BPBP 132/78132/78
  • 79.
  • 80.
  • 81.
  • 82.
  • 83.
  • 84.
  • 85. 2 Week F/U at Univ. of Kentucky2 Week F/U at Univ. of Kentucky Medical CenterMedical Center Complains of headachesComplains of headaches Blood pressure 240/150 on multipleBlood pressure 240/150 on multiple checkschecks Positive ANAPositive ANA SED rate 55SED rate 55 MRI (head and orbits) NormalMRI (head and orbits) Normal
  • 86. Hypertensive RetinopathyHypertensive Retinopathy ESSENTIAL HYPERTENSIONESSENTIAL HYPERTENSION (no known cause)(no known cause) SECONDARY HYPERTENSIONSECONDARY HYPERTENSION Preeclampsia/eclampsiaPreeclampsia/eclampsia kidney diseasekidney disease adrenal diseaseadrenal disease coarctation of the aortacoarctation of the aorta
  • 87. PATIENT HISTORYPATIENT HISTORY PH: 12 yr. WM c/o decreased vision OD x 5PH: 12 yr. WM c/o decreased vision OD x 5 days. Flu-like symptoms 1 week prior.days. Flu-like symptoms 1 week prior. PMH: HealthyPMH: Healthy EXAM:EXAM: ODOD CF @ 4 ft.CF @ 4 ft. IOPIOP 1212 OSOS 20/2020/20 1212
  • 88.
  • 89.
  • 90.
  • 91.
  • 92.
  • 93.
  • 94. Serologic Work UpSerologic Work Up HLA B27HLA B27 NEGATIVENEGATIVE FTA-ABSFTA-ABS NON-REACTIVENON-REACTIVE ANAANA NEGATIVENEGATIVE RA quant.RA quant. NORMALNORMAL RPRRPR NON-REACTIVENON-REACTIVE HIVHIV NEGATIVENEGATIVE ACEACE NORMALNORMAL CAT SCRATCH AB-RCAT SCRATCH AB-R POSITIVEPOSITIVE SED RATESED RATE 6161 CXRCXR NORMALNORMAL
  • 95. Acute Neuroretinitis Secondary toAcute Neuroretinitis Secondary to Cat Scratch DiseaseCat Scratch Disease Bartonella henselaeBartonella henselae is the causativeis the causative organismorganism VA loss may be mild or severeVA loss may be mild or severe 33% of cases may be bilateral33% of cases may be bilateral > 90% improve to 20/40 within 8 weeks> 90% improve to 20/40 within 8 weeks and 20/20 within 6 monthsand 20/20 within 6 months Small subgroup left with severe visual lossSmall subgroup left with severe visual loss and associated optic atrophyand associated optic atrophy
  • 96. TreatmentTreatment No proven treatmentNo proven treatment Periocular and systemic corticosteriodsPeriocular and systemic corticosteriods Antibiotic therapyAntibiotic therapy No difference in treated vs. untreatedNo difference in treated vs. untreated patientspatients
  • 97.
  • 98. PATIENT HISTORYPATIENT HISTORY PH: 50yr. WF who reports distortion andPH: 50yr. WF who reports distortion and occasional blurred vision OU x 6 yrs.occasional blurred vision OU x 6 yrs. PMH: Breast lumpectomy (benign), traumaPMH: Breast lumpectomy (benign), trauma (auto accident) and Bell’s palsy.(auto accident) and Bell’s palsy. POH: Glaucoma OU.POH: Glaucoma OU. EXAM:EXAM: 20/40 phni20/40 phni IOPIOP 1717 20/60 phni20/60 phni 1717 BPBP 140/78140/78
  • 99.
  • 100.
  • 101.
  • 102.
  • 103.
  • 104.
  • 105.
  • 106.
  • 107.
  • 108. Geographic Helicoid PeripapillaryGeographic Helicoid Peripapillary ChoroiditisChoroiditis (Serpiginous choroiditis)(Serpiginous choroiditis) • CHRONIC RECURRING CHOROIDITISCHRONIC RECURRING CHOROIDITIS • MULTIFOCALMULTIFOCAL • INNER CHOROID & RPEINNER CHOROID & RPE • INFLAMMATION vs. SRNVINFLAMMATION vs. SRNV
  • 109. TreatmentTreatment Steroids (oral or periocular injection)Steroids (oral or periocular injection) Photocoagulation for SRNVMPhotocoagulation for SRNVM Anti - VEGFAnti - VEGF
  • 110. PATIENT HISTORYPATIENT HISTORY 25 y/o , w/f was seen for the 125 y/o , w/f was seen for the 1stst time on Feb.time on Feb. 1616thth 20102010 c/o intermittent blurring of central visionc/o intermittent blurring of central vision which started 2 mo ago and last about 4-5which started 2 mo ago and last about 4-5 hours.hours. Her visual symptoms increased recentlyHer visual symptoms increased recently and last longer.and last longer. PMH: unremarkable.PMH: unremarkable. Case 4
  • 111. ExaminationExamination Visual acuityVisual acuity – OD 20/60OD 20/60 OD 16 mm HgOD 16 mm Hg – OS 20/40OS 20/40 OS 14 mm HgOS 14 mm Hg SLE:SLE: a few vitreous cells.a few vitreous cells. Fundus:Fundus: Hyperemic optic nerves, OUHyperemic optic nerves, OU Cystoid macular edema, OUCystoid macular edema, OU FANG:FANG: CME with ON leakage, OUCME with ON leakage, OU OCT: Thickened neuroepithelium with largeOCT: Thickened neuroepithelium with large cysticcystic changes, OUchanges, OU
  • 114. Laboratory Work-upLaboratory Work-up CBCCBC NormalNormal ESRESR NormalNormal CRPCRP NormalNormal RFRF NormalNormal ANAANA NormalNormal ACEACE NormalNormal RPRRPR Non-reactiveNon-reactive FTA-AbsFTA-Abs Non-reactiveNon-reactive CXRCXR NormalNormal MRIMRI NormalNormal
  • 115. Feb 23Feb 23rdrd 20102010 No visual complaintsNo visual complaints VAVA – ODOD 20/2020/20 – OSOS 20/2020/20 Fundus:Fundus: – Macula and optic nerve flat OUMacula and optic nerve flat OU OCT:OCT: NormalNormal
  • 117. March 16March 16thth 20102010 C/O blurred vision since March 3C/O blurred vision since March 3rdrd OUOU On further questioning, she reported that allOn further questioning, she reported that all episodes of blurred vision would start with eachepisodes of blurred vision would start with each menstrual period (21 days), last the first half ofmenstrual period (21 days), last the first half of each cycle, and resolve at the end ofeach cycle, and resolve at the end of menstruation. No oral contraceptionmenstruation. No oral contraception VA: 20/80 OUVA: 20/80 OU Fundus: CME with congested ON, OU.Fundus: CME with congested ON, OU. OCT: CMEOCT: CME
  • 118. BibliographyBibliography Cyclic Presentation of CME: A. Assi, J. Hickman Casey,Cyclic Presentation of CME: A. Assi, J. Hickman Casey, et al.: Acta Ophthalmol. Scand. 1998: 76: 245-246et al.: Acta Ophthalmol. Scand. 1998: 76: 245-246 – Presented the case of a 28 y/o lady with unilateral pars planitisPresented the case of a 28 y/o lady with unilateral pars planitis and associated CME in whom visual symptoms fluctuatedand associated CME in whom visual symptoms fluctuated regularly during menstrual cycles.regularly during menstrual cycles. Cyclical central serous chorloretinopathy associatedCyclical central serous chorloretinopathy associated with cystoid macular oedema.with cystoid macular oedema. Birchall W, Charles SJ,Birchall W, Charles SJ, Buckler HM. BJO 2001 Jun;85(6):756-8.Buckler HM. BJO 2001 Jun;85(6):756-8. – CME resolved on oral contraceptives.CME resolved on oral contraceptives.
  • 119. Consultations and Work-upConsultations and Work-up GynecologicalGynecological NormalNormal EndocrineEndocrine NormalNormal ImmunologicalImmunological NormalNormal ClottingClotting APC, R 0.6APC, R 0.6
  • 120. TreatmentTreatment August 20August 20thth , 2010., 2010. – Oral contraceptive pill Yasminelle.Oral contraceptive pill Yasminelle. – Her cycle from 21 days changed to 28 daysHer cycle from 21 days changed to 28 days with initial improvement of her visualwith initial improvement of her visual symptoms for 2-3 months.symptoms for 2-3 months. November 11November 11thth , 2010, 2010 – Started on stronger contraceptive pill, Yasmin,Started on stronger contraceptive pill, Yasmin, with subjective visual improvement.with subjective visual improvement.
  • 121. Dec 20Dec 20thth 20102010 Reports visual improvementReports visual improvement On oral contraceptives for 4 monthsOn oral contraceptives for 4 months VAVA – OD 20/60OD 20/60 – OS 20/40OS 20/40
  • 123. Beginning of Jan. 2011 developed progressiveBeginning of Jan. 2011 developed progressive painful ophthalmoplegia OD which was attributed topainful ophthalmoplegia OD which was attributed to a large brain mass. It was not present at her initiala large brain mass. It was not present at her initial evaluation by MRI.evaluation by MRI. Biopsy revealed inflammatory compounds.Biopsy revealed inflammatory compounds. Was treated with IV antibiotics with completeWas treated with IV antibiotics with complete regression of her ophthalmoplegiaregression of her ophthalmoplegia..
  • 125. Happy EndingHappy Ending Patient got married and is expectingPatient got married and is expecting a baby soon.a baby soon. She is on no medications.She is on no medications. No visual complaints.No visual complaints. VA: 20/20 OU.VA: 20/20 OU.
  • 126. QuestionsQuestions Etiology of Cyclic Presentation of CME.Etiology of Cyclic Presentation of CME. Treatment?Treatment? If anyone in the audience has comeIf anyone in the audience has come across with a similar case, please shareacross with a similar case, please share..