Normal Tension Glaucoma. - School of Medicine · IOP • CNTGS has 24 mm Hg as cut off. • Most...
Transcript of Normal Tension Glaucoma. - School of Medicine · IOP • CNTGS has 24 mm Hg as cut off. • Most...
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Normal Tension Glaucoma.Normal Tension Glaucoma.
Jorge L. Fernandez Bahamonde, MD.
Jorge L. Fernandez Bahamonde, MD.
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Definition & Epidemiology.Definition & Epidemiology.• Progressive optic neuropathy that mimics
POAG without elevated IOP.• 33% of POAG.
• Close to 2/3 in Japan.• More common in females? (questionable)
• Live longer.
• POAG and NTG coexist in same families.
• Progressive optic neuropathy that mimics POAG without elevated IOP.
• 33% of POAG.• Close to 2/3 in Japan.• More common in females? (questionable)
• Live longer.
• POAG and NTG coexist in same families.
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IOPIOP• CNTGS has 24 mm Hg as cut off.• Most studies use 21 mm Hg or less.• Many patients have asymmetric damage
with symmetric IOP.• Drop in IOP may slow but not halt
disease 1.• 30 % drop from baseline for treated.• Deterioration ratio. Control vs. Treated, 3:1.
• CNTGS has 24 mm Hg as cut off.• Most studies use 21 mm Hg or less.• Many patients have asymmetric damage
with symmetric IOP.• Drop in IOP may slow but not halt
disease 1.• 30 % drop from baseline for treated.• Deterioration ratio. Control vs. Treated, 3:1.
1 Am J Ophthalmol 1998 Oct;126(4):487-97
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Blood FlowBlood Flow• Increase incidence of migraine and
vasospasms.• Ca +2 channel blockers may help.
• Nocturnal hypotension.• Sleep apnea (OSAS)• Lower pulsatile ocular blood flow.• Reduced peripapillary blood flow.
• Increase incidence of migraine andvasospasms.• Ca +2 channel blockers may help.
• Nocturnal hypotension.• Sleep apnea (OSAS)• Lower pulsatile ocular blood flow.• Reduced peripapillary blood flow.
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Immune related?Immune related?• Elevated antibodies to retinal proteins.• 12% of NTG show increase level of
monoclonal gammopathies.• Similar to progressive peripheral
neuropathies with monoclonal paraproteinemia.
• 30% prevalence of autoimmunedisorders.
• Elevated antibodies to retinal proteins.• 12% of NTG show increase level of
monoclonal gammopathies.• Similar to progressive peripheral
neuropathies with monoclonal paraproteinemia.
• 30% prevalence of autoimmunedisorders.
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Natural History of NTGNatural History of NTG
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Natural History of NTGNatural History of NTG
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OverlapSyndromes
OverlapSyndromes
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Differential Diagnosis of cuppingDifferential Diagnosis of cupping
• Compressive optic neuropathy• Congenital disc anomalies - Pit, Coloboma,Morning glory
syndrome, Tilted disc, Macrodisc, Papillorenal syndrome• A-AION >>>>>> NA-AION• Hereditary optic neuropathies - LHON, DOA, ROA,DIDMOAD• Post-inflammatory optic neuropathy - MS• Toxic optic neuropathy - Methanol, Paint thinner, Nutritional• Traumatic optic neuropathy• Periventricular leukomalacia - Prematures• Other - Paraneoplastic, Advanced ARMD, etc.
• Compressive optic neuropathy• Congenital disc anomalies - Pit, Coloboma,Morning glory
syndrome, Tilted disc, Macrodisc, Papillorenal syndrome• A-AION >>>>>> NA-AION• Hereditary optic neuropathies - LHON, DOA, ROA,DIDMOAD• Post-inflammatory optic neuropathy - MS• Toxic optic neuropathy - Methanol, Paint thinner, Nutritional• Traumatic optic neuropathy• Periventricular leukomalacia - Prematures• Other - Paraneoplastic, Advanced ARMD, etc.
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Cmprssv Optc Nr-pty - AcromegalyCmprssv Optc Nr-pty - Acromegaly
Growth-Hormone-SecretingPituitaryAdenoma
24 y/oAA male
VAR- 20/20
L- CF
Nerve-fieldmismatch
in OS.Disc shape
& cupasymmetry
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Cmprssv Optic Nr-pthy - AneurysmCmprssv Optic Nr-pthy - Aneurysm
47 y/o WFVA : R - 20/40 - cupped
L - 20/20DX: Right Internal Carotid Artery Aneurysm
Cupping OD
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Cmprssv Optic Nr-pthy - GliomaCmprssv Optic Nr-pthy - Glioma
DX:ChiasmalGlioma
9 y/o WMNo NF
BandONA,Pseudo
Cup
MRI:Fat
Chiasm
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Congenital - ColobomaCongenital - Coloboma
VF - NormalIOP - Stable & normal
Course - No deterioration or change
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Congenital - Morning GloryCongenital - Morning Glory
Morning Glory FlowerCentral & Anomalous Vessels. Cuppedcenter. White central tissues. Unilateral.Pigmentary changes around Disc.
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Congenital - PapilloRenal SyndromeCongenital - PapilloRenal Syndrome
No central vessels.Multiple cilio-retinal vessels.
Cupped center.Associated Renal disease.
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Congenital - Tilted DiscCongenital - Tilted Disc
Common in myopes.Difficult to tell from glaucoma.
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Congenital - Macro DiscsCongenital - Macro DiscsNormal-sized disc.
Large-sized disc,Horizontal cup.
PROBLEM - Determining disc size from a simple disc exam,since normal discs vary so much.
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NA-AION - FundusNA-AION - Fundus
Acute phase photo
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Hereditary optc nr-pthy - ADOAHereditary optc nr-pthy - ADOA
Autosomal Dominant Optic Atrophy (Kjer type)
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Hereditary optc nr-pthy - LHONHereditary optc nr-pthy - LHON
Leber’s Hereditary Optic Neuropathy
Acute Phase Chronic Phase
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Hereditaryoptic nr-pthy
DIDMOAD
Hereditaryoptic nr-pthy
DIDMOAD
Diabetes Insipidus,Diabetes Mellitus,Optic Atrophy,
Deafness(Wolfram’s Syndrome)Autosomal Recessive
WFS1 gene defect
19 y/o WM20/200 OU
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Post-Optic Neuritis optic neuropathyPost-Optic Neuritis optic neuropathy
30 y/wM,ON-IS-OD:
Rcrr:23 yrs later:OD-20/50,OS - 20/20
1998 1998
2003 2003
After MS optic
neuritis
After MS optic
neuritis
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Periventricular leukomalaciaPeriventricular leukomalacia
Perinatal hypoxic brain damage.Associated neurologic deficits.
Perinatal hypoxic brain damage.Associated neurologic deficits.
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Traumatic Optic NeuropathyTraumatic Optic Neuropathy
16 y maleFell from bike & hit forehead.
V- HM. Rx: Megadose steroids. V-200
Acute - 16 days. Chronic - 90 days.
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Paraneoplastic Optic NeuropathyParaneoplastic Optic Neuropathy
Teratoma-associated Paraneoplastic Retinopathy
VA:OD - 20/16OS - 20/25
TOD - 15OS - 13
Vitritis - cellsCups
Thin vessels
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Paraneoplastic Optic NeuropathyParaneoplastic Optic Neuropathy
Multicystic AnteriorMediastinal Teratoma
OS OD
ODOS
Initial VF’s
Post-Rx periocular steroids
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Diagnosis & DifferentialDiagnosis & Differential
• Presentation.• Optic nerve cupping similar to POAG
without elevated IOP.• Peripapillary atrophy.• In general quite similar to POAG.
• Disk hemorrhage no longer exclusive of NTG, it is only a predictor of future damage.
• Presentation.• Optic nerve cupping similar to POAG
without elevated IOP.• Peripapillary atrophy.• In general quite similar to POAG.
• Disk hemorrhage no longer exclusive of NTG, it is only a predictor of future damage.
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Diagnosis & DifferentialDiagnosis & Differential• VFs.
• Similar to POAG but appears more advance that optic nerve status.
• Deeper, steeper & closer to fixation?, more often superior?
• VFs.• Similar to POAG
but appears more advance that optic nerve status.
• Deeper, steeper & closer to fixation?, more often superior?
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NTG BRAIN TUMOR Atypical
Hx Slow Fast or ± ±
Age > 60 < 50 ±
Color wnl abn ±
VA wnl Poor or N ±
IOP wnl wnl BDL
VFNasal, Arcuate, Paracentral
ScotomasVertical, Central,
JunctionalMismatch, Atypical
ONHPPCC, Notch, Flame, NRR-
N, Cup > PallorPallor > Cup, Pale
NRRMismatch, Atypical
Basic Diff DxBasic Diff Dx
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Work up stepsWork up steps1- History, Age2- Visual Acuity and Color vision !!!3- Pupils4- Clinical course5- Visual fields - Essential & Basic6- Optic nerve head7- NeuroImaGing = (NIG)8- Other tests : Lab, X-R, CSF, AG7- NeuroImaGing = NIG
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Diagnosis & DifferentialDiagnosis & Differential• Work-up.
• Diurnal curve.• At least 3 readings.
• Optic nerve documentation.• Photograph, drawing.• GDx, HRT-II or OCT.
• VF.• SWAP?
• Work-up.• Diurnal curve.
• At least 3 readings.• Optic nerve documentation.
• Photograph, drawing.• GDx, HRT-II or OCT.
• VF.• SWAP?
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Work-Up: MRIWork-Up: MRIMRIMRIMRI low yield in asymptomatic patients (2/53)2
• INDICATIONS FOR MRI:
• HISTORY - Headaches,Eye pains, Progressive visual loss, Mental changes, Diplopia, Endocrine changes, Neurologic deficits, Sudden onset.
• OTHER - Age < 50 yrs, VA < 20/40, Dyschromatopsia,
• VFs - Vertical step, Junction scotoma.
• COURSE - Progression after 25-30% IOP drop.
• ONH - NRR pallor, Pallor > cupping.
• ONH & VF - Mismatch of findings.
MRI low yield in asymptomatic patients (2/53)2
• INDICATIONS FOR MRI:
• HISTORY - Headaches,Eye pains, Progressive visual loss, Mental changes, Diplopia, Endocrine changes, Neurologic deficits, Sudden onset.
• OTHER - Age < 50 yrs, VA < 20/40, Dyschromatopsia,
• VFs - Vertical step, Junction scotoma.
• COURSE - Progression after 25-30% IOP drop.
• ONH - NRR pallor, Pallor > cupping.
• ONH & VF - Mismatch of findings.
2 AAO: Optic nerve head and RNFL. Oph 1999;106:1414-1424
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Diagnosis & DifferentialDiagnosis & Differential• Work-up.
• Labs.• CBC, r/o anemia.• VDRL/FTA.• Sed rate & C-reactive protein.
• Temporal Arteritis.
• Carotid doppler.• Sleep studies.
• Benefits of CPAP?
• Work-up.• Labs.
• CBC, r/o anemia.• VDRL/FTA.• Sed rate & C-reactive protein.
• Temporal Arteritis.
• Carotid doppler.• Sleep studies.
• Benefits of CPAP?
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Waiting &observation areoptions in some
glaucomas:NTG, OHT
The ThinkerSculpture - Rodin
PEARLS FOR OBSERVATION:1- Watch out for rapid progressors.2- Do IF: few &/or low risk factors.3- Close FU required:
1st 2 years, do VF’s q 3-4 mos.,after 2 yrs, do VF’s 2x/year.
Treatment: Observation & FU optionTreatment: Observation & FU option
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Indications for Treating NTGIndications for Treating NTGConsider when there are Risk Factors as:--Females, Migraine.--Vasospastic phenomena: Raynaud’s, Cold
hands & feet, Prinsmetal angina.--Severe VFD’s at presentation.--Subjective worsening of vision.--Disc hemorrhages.
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Treatment.Treatment.• Initial target.
• 30% from baseline.• Adjust with frequent VFs and imaging.
• Medical Therapy.• PFG.• Topical CAIs• Alpha 2 agonists.
• ALT/SLT.• Sx.
• Initial target.• 30% from baseline.• Adjust with frequent VFs and imaging.
• Medical Therapy.• PFG.• Topical CAIs• Alpha 2 agonists.
• ALT/SLT.• Sx.