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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
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
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
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
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
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.
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.
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
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
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
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?
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