Grand RoundsGrand Rounds

Terry J. Alexandrou, MDTerry J. Alexandrou, MDDepartment of Ophthalmology Department of Ophthalmology

and Visual Scienceand Visual ScienceThe University of ChicagoThe University of Chicago

9/7/059/7/05

August 9, 2003August 9, 2003

• HPI: W.A is a 60 y.o A.A. male who presents HPI: W.A is a 60 y.o A.A. male who presents with a 3 day history of decreased VA and with a 3 day history of decreased VA and pain in the right eye.pain in the right eye.

• Awoke with red, painful right eye.Awoke with red, painful right eye.

• Described decreased VA in right eye as Described decreased VA in right eye as “seeing only black.”“seeing only black.”

• C/O bifrontal headache.C/O bifrontal headache.

• Denied jaw pain, scalp tenderness, Denied jaw pain, scalp tenderness, discharge.discharge.

HistoryHistory

• PMH: None.PMH: None.• POH: s/p cataract extraction with POH: s/p cataract extraction with

PCIOL OU; “few years back”PCIOL OU; “few years back”• Meds: NoneMeds: None• All: NKDAAll: NKDA• FH: N/CFH: N/C• SH: Immigrant from Africa, No EtOH, SH: Immigrant from Africa, No EtOH,

tobacco or IV drug use.tobacco or IV drug use.

ExamExam• VA: Without correction: VA: Without correction:

OD- Light perception OS- 20/20 (-2) OD- Light perception OS- 20/20 (-2)• Pupils: + APD Pupils: + APD • EOM: fullEOM: fullAnterior Exam:Anterior Exam: OD: 3+ injection, severe corneal OD: 3+ injection, severe corneal

edema, +NVI, PCIOLedema, +NVI, PCIOL OS: mild blepharitis, PCIOLOS: mild blepharitis, PCIOL

IOP – any guesses?IOP – any guesses?

• 1515

• 2525

• 4646

• 7676

76!!!!!!!!!76!!!!!!!!!

Differential???Differential???

Differential…Differential…

• Diabetic RetinopathyDiabetic Retinopathy

• Hypertensive RetinopathyHypertensive Retinopathy

• Radiation RetinopathyRadiation Retinopathy

• Ocular Ischemic SyndromeOcular Ischemic Syndrome

• CRVOCRVO

• Hyperviscosity SyndromeHyperviscosity Syndrome

Next Step…Next Step…

• What do you want to do now?What do you want to do now?

Acute TreatmentAcute Treatment

• Alphagan x2Alphagan x2

• Timolol x2Timolol x2

• Diamox x1Diamox x1

• Pressure recheck 4 hours later:Pressure recheck 4 hours later:

OD – 45OD – 45

OS - 17OS - 17

• Based on the appearance of the Based on the appearance of the fundus photographs, what do you fundus photographs, what do you want to check for next?want to check for next?

Blood PressureBlood Pressure

• 202/102 202/102

-rechecked several times with -rechecked several times with

manual cuffmanual cuff

Admitted to Medicine ServiceAdmitted to Medicine Service

• Initial B.P in Emergency Department was 206/94Initial B.P in Emergency Department was 206/94

• Initially treated with Labetalol 5mg IV pushesInitially treated with Labetalol 5mg IV pushes

• Overnight, started on Coreg 6.25 mg bidOvernight, started on Coreg 6.25 mg bid

• B.P overnight ranged from 155-162/63-71B.P overnight ranged from 155-162/63-71

• Overnight pt. Received timolol x3, alphagan x3, diamox x2 Overnight pt. Received timolol x3, alphagan x3, diamox x2 (in addition to initial treatment)(in addition to initial treatment)

Labs and TestsLabs and Tests

• Chem 10 WNL (glucose of 105)Chem 10 WNL (glucose of 105)

• CBC WNLCBC WNL

• HgBA1C of 6.6HgBA1C of 6.6

• All other labs unremarkableAll other labs unremarkable

• Bilateral Carotid U/S - negativeBilateral Carotid U/S - negative

Day 2Day 2

• No painNo pain

Day 2Day 2

• VA: without correction:VA: without correction: OD – LP (but, temporal CF at 2 ft.)OD – LP (but, temporal CF at 2 ft.) OS – 20/20 (-2)OS – 20/20 (-2)

• IOP: IOP: OD – 48OD – 48 OS – 16OS – 16 (after total of timolol x4, alphagan x4, diamox (after total of timolol x4, alphagan x4, diamox

x3)x3)

• Diagnosis…….Diagnosis…….

• Hypertensive retinopathy with ass. Hypertensive retinopathy with ass. CRVO and secondary NVGCRVO and secondary NVG

Neovascular GlaucomaNeovascular Glaucoma• SYSTEMIC VASCULAR SYSTEMIC VASCULAR

DISEASESDISEASES*** - Carotid occlusive Disease *** - Carotid occlusive Disease

****** - Carotid artery ligation- Carotid artery ligation - Giant cell arteritis- Giant cell arteritis - Takayusu disease- Takayusu disease

• OCULAR VASCULAR DISEASEOCULAR VASCULAR DISEASE*** - Diabetic Retinopathy ****** - Diabetic Retinopathy ****** - CRVO ****** - CRVO *** - CRAO- CRAO - BRVO - BRVO - Sickle cell retinopathy- Sickle cell retinopathy - Coats Disease- Coats Disease - ROP- ROP

• OTHER OCULAR DISEASESOTHER OCULAR DISEASES - Chronic uveitis- Chronic uveitis - Chronic RD- Chronic RD - Endophthalmitis- Endophthalmitis - Retinoschisis- Retinoschisis

• INTRAOCULAR TUMORSINTRAOCULAR TUMORS - Uveal melanoma- Uveal melanoma - Metastatic Carcinoma- Metastatic Carcinoma - Retinoblastoma- Retinoblastoma

• OCULAR THERAPYOCULAR THERAPY - Radiation Therapy- Radiation Therapy

• TRAUMATRAUMA

Treatment…any thoughtsTreatment…any thoughts

• Elevated pressure OD (48) with no view….Elevated pressure OD (48) with no view….

CyclocyrotherapyCyclocyrotherapy

• 6 spots of cryo6 spots of cryo

Retinal CryotherapyRetinal Cryotherapy

• 12 spots of cryo12 spots of cryo

Day 3 (still an inpatient)Day 3 (still an inpatient)• POD # 1 s/p retinal and ciliary body cryotherapy for NVG 2/2 CRVOPOD # 1 s/p retinal and ciliary body cryotherapy for NVG 2/2 CRVO

• C/O pain in the right eyeC/O pain in the right eye

• VA: OD-HM OS: 20/30VA: OD-HM OS: 20/30• IOP: OD-18 OS-12IOP: OD-18 OS-12• Anterior Exam: OD - edematous cornea (poor view), Anterior Exam: OD - edematous cornea (poor view),

with hyphema and fibrinous reaction in the AC; NVIwith hyphema and fibrinous reaction in the AC; NVI

• Pt. started on PF q1 hour and atropine bid, along with Pt. started on PF q1 hour and atropine bid, along with pressure lowering medspressure lowering meds

Day 4 (outpatient)Day 4 (outpatient)

• POD # 2 s/p retinal and ciliary body cryotherapy for NVG 2/2 CRVOPOD # 2 s/p retinal and ciliary body cryotherapy for NVG 2/2 CRVO

• Still with pain in right eyeStill with pain in right eye

• VA: OD-HM OS: 20/30VA: OD-HM OS: 20/30• IOP: OD-21 OS-16IOP: OD-21 OS-16• Anterior Exam: OD- edematous cornea, slightly Anterior Exam: OD- edematous cornea, slightly

clearer, with 5% suspended hyphema and clearer, with 5% suspended hyphema and fibrinous reaction in the AC; fibrinous reaction in the AC;

• PF q2 hours, atropine bid, alphagan, timololPF q2 hours, atropine bid, alphagan, timolol

1 Week1 Week

• VA:VA:

OD-HMOD-HM

OS- 20/30OS- 20/30

IOP:IOP:

OD-12OD-12

OS-13OS-13

AC: resolving fibrinAC: resolving fibrin

2 weeks2 weeks

• VA:VA: OD- HMOD- HM OS – 20/30OS – 20/30AC: No fibrin, D+Q, No NVI; cornea AC: No fibrin, D+Q, No NVI; cornea

clear………..FINALLY!!!clear………..FINALLY!!!

Treatment – PRP right eyeTreatment – PRP right eye

F/U Next weekF/U Next week

Hypertensive RetinopathyHypertensive Retinopathy

• JNC lists retinopathy as 1 of several markers of JNC lists retinopathy as 1 of several markers of target-organ damage in hypertensiontarget-organ damage in hypertension

• First described by Marcus GunnFirst described by Marcus Gunn

Gunn noticed:Gunn noticed: - - arteriolar narrowingarteriolar narrowing

- arteriovenous nicking- arteriovenous nicking

- flame-shaped and blot-shaped retinal - flame-shaped and blot-shaped retinal hemorrageshemorrages

- cotton-wool spots- cotton-wool spots

- swelling of the optic disc- swelling of the optic disc

PathophysiologyPathophysiology

• 1) Vasoconstrictive Stage – initially, seen as 1) Vasoconstrictive Stage – initially, seen as generalized narrowing of retinal arteriolesgeneralized narrowing of retinal arterioles

• 2) Persistently Elevated BP – more severe 2) Persistently Elevated BP – more severe generalized and focal areas of arteriolar generalized and focal areas of arteriolar narrowing; AV nicking, and alterations in narrowing; AV nicking, and alterations in arteriolar light reflex (copper wiring)arteriolar light reflex (copper wiring)

• 3) Exudative Stage – microaneurysms, 3) Exudative Stage – microaneurysms, hemorrhages, hard exudates, cotton-wool spots, hemorrhages, hard exudates, cotton-wool spots, swelling of the optic disc (if severely elevated swelling of the optic disc (if severely elevated blood pressure)blood pressure)

EpidemiologyEpidemiology

• > 50 million people in the US affected by > 50 million people in the US affected by hypertensionhypertension

• Prevalence of 2-15 % for various signs of Prevalence of 2-15 % for various signs of retinopathyretinopathy

Classification of Hypertensive Classification of Hypertensive RetinopathyRetinopathy

Grade 0 No changesGrade 0 No changes

Grade 1 Barely detectable arterial narrowingGrade 1 Barely detectable arterial narrowing

Grade 2 Obvious arterial narrowing with focal Grade 2 Obvious arterial narrowing with focal irregularitiesirregularities

Grade 3 Grade 2 plus retinal hemorrhages and/or Grade 3 Grade 2 plus retinal hemorrhages and/or exudateexudate

Grade 4 Grade 3 plus disc swellingGrade 4 Grade 3 plus disc swelling

TreatmentTreatment

• Blood pressure ControlBlood pressure Control

• Chronic hypertensive retinopathy Chronic hypertensive retinopathy alone rarely, if ever, results in alone rarely, if ever, results in significant vision loss.significant vision loss.

• Evidence of hypertensive retinopathy Evidence of hypertensive retinopathy can be used for risk stratification of can be used for risk stratification of other systemic diseases associated other systemic diseases associated with hypertensionwith hypertension

Atherosclerosis Risk in Communities Atherosclerosis Risk in Communities StudyStudy

• Multisite cohort studyMultisite cohort study

• 5 year risk of stroke of participants with both 5 year risk of stroke of participants with both hypertensive retinopathy and cerebral lesions on hypertensive retinopathy and cerebral lesions on MRI (compared to those with neither of these 2 MRI (compared to those with neither of these 2 findings) was 18.1.findings) was 18.1.

- Among participants with only cerebral lesions - Among participants with only cerebral lesions on MRI only, the RR of stroke was 3.4.on MRI only, the RR of stroke was 3.4.

CRVOCRVO

PathogenesisPathogenesis

• Thrombus in the central retinal vein Thrombus in the central retinal vein at the level of the lamina cribosa, at the level of the lamina cribosa, often secondary to atherosclerosis of often secondary to atherosclerosis of the neighboring central retinal the neighboring central retinal artery.artery.

Risk FactorsRisk Factors

• Age > 50 Age > 50

• DMDM

• HtnHtn

• Hyperviscosity syndromesHyperviscosity syndromes

• GlaucomaGlaucoma

Classification of CRVOClassification of CRVO

• 1) Non-ischemic – ~75-80% have this 1) Non-ischemic – ~75-80% have this milder formmilder form

• 2) Ischemic - ~20-25% have this severe 2) Ischemic - ~20-25% have this severe formform

Non-Ischemic CRVONon-Ischemic CRVO

• Symptoms – Symptoms – 1) mild to moderate decrease in VA 1) mild to moderate decrease in VA (can range from normal to CF however); 2) (can range from normal to CF however); 2) intermittent blurring; 2 pain is rareintermittent blurring; 2 pain is rare

• Signs – Signs – 1) APD is rare; 2) dot and flame 1) APD is rare; 2) dot and flame hemorrhages in all 4 quadrants; 3) optic nerve hemorrhages in all 4 quadrants; 3) optic nerve head swelling; 4) engorgement and tortuosity of head swelling; 4) engorgement and tortuosity of retinal veins; 5) macular hemorrhage or edema; retinal veins; 5) macular hemorrhage or edema; 6) rarely is there neovascularization of either the 6) rarely is there neovascularization of either the anterior or posterior segment (<2%)anterior or posterior segment (<2%)

Progression of Non-Ischemic to Ischemic Progression of Non-Ischemic to Ischemic CRVOCRVO

• Central Vein Occlusion Study Central Vein Occlusion Study

- 34 % of eyes initially diagnosed - 34 % of eyes initially diagnosed with non-ishemic CRVO progressed with non-ishemic CRVO progressed to ischemic variant within 3 years; to ischemic variant within 3 years; (15% converted within first 4 (15% converted within first 4 months)months)

Ischemic CRVOIschemic CRVO• 1) Symptoms – 1) Symptoms – 1) Acute, markedly decreased visual 1) Acute, markedly decreased visual

acuity (usual presenting complaint, VA ranges from 20/200 acuity (usual presenting complaint, VA ranges from 20/200 to LP) ; 2) Pain (if neovascular glaucoma has already to LP) ; 2) Pain (if neovascular glaucoma has already developed)developed)

• 2) Signs – 2) Signs – 1) APD is common; 2) Extensive 4 – quadrant 1) APD is common; 2) Extensive 4 – quadrant retinal hemorrhages and edema; 3) Marked venous retinal hemorrhages and edema; 3) Marked venous Dilation; 4) Anterior Segment Neovascularization (60 % or Dilation; 4) Anterior Segment Neovascularization (60 % or higher) 5) Neovascular glaucoma – can occur within 3 higher) 5) Neovascular glaucoma – can occur within 3 months of disease onset (90 day glaucoma), often resulting months of disease onset (90 day glaucoma), often resulting in intractably elevated IOP’s; 6) Neovascularization of optic in intractably elevated IOP’s; 6) Neovascularization of optic disc and retina may be seen, but not as common;disc and retina may be seen, but not as common;

DiagnosisDiagnosis

• 1) Characteristic fundus photos – 1) Characteristic fundus photos – • 2) FANG in ischemic CRVO– most useful ancillary test; Eyes 2) FANG in ischemic CRVO– most useful ancillary test; Eyes

with 10 disc areas or greater of non-perfusion are classified as with 10 disc areas or greater of non-perfusion are classified as ischemicischemic

- demonstrates marked hypofluorescence – secondary to - demonstrates marked hypofluorescence – secondary to blockage from extensive retinal hemorrhages or to capillary blockage from extensive retinal hemorrhages or to capillary non-perfusionnon-perfusion

- optic nerve head leakage- optic nerve head leakage - macular edema- macular edema• 2) FANG in non-ischemic CRVO – 2) FANG in non-ischemic CRVO – - staining along retinal veins- staining along retinal veins - microaneurysms - microaneurysms - dilated optic nerve head capillaries- dilated optic nerve head capillaries - minimal or absent retinal capillary non-perfusion- minimal or absent retinal capillary non-perfusion

TreatmentTreatment

• If Neovascular Glaucoma – CVOS demonstrated PRP was If Neovascular Glaucoma – CVOS demonstrated PRP was recommended in eyes where iris neovascularization was recommended in eyes where iris neovascularization was present, however prophylactic PRP did not show statistical present, however prophylactic PRP did not show statistical significance in prevent neovascularization.significance in prevent neovascularization.

• If Macular Edema – CVOS showed that macular grid laser If Macular Edema – CVOS showed that macular grid laser reduced angiographic evidence of macular edema, however reduced angiographic evidence of macular edema, however there was no difference in VA of treated vs. untreated eyes; there was no difference in VA of treated vs. untreated eyes; Recent study demonstrated intravitreal kenalog effective in Recent study demonstrated intravitreal kenalog effective in reversing CME and improving VA in recent-onset non-reversing CME and improving VA in recent-onset non-ischemic CRVO at 6 months, but results not maintained at 1 ischemic CRVO at 6 months, but results not maintained at 1 yearyear

• Laser – induce Chorioretinal Venous AnastomosisLaser – induce Chorioretinal Venous Anastomosis

• Radial neurotomyRadial neurotomy

Typical Course of CRVOTypical Course of CRVO

• 50 % of non-ischemic CRVO patients deteriorate 50 % of non-ischemic CRVO patients deteriorate to 20/200 or worseto 20/200 or worse

• 90 % of ischemic CRVO patients have VA of 90 % of ischemic CRVO patients have VA of 20/200 or worse20/200 or worse

• 7% of CRVO patients develop a venous occulsion 7% of CRVO patients develop a venous occulsion in the fellow eye within 2 years (risk ~ .9 % per in the fellow eye within 2 years (risk ~ .9 % per year)year)