Differential Diagnosis of ONH Edema Beth A. Steele, OD, FAAO

Transcription

1 Differential Diagnosis of ONH Edema Beth A. Steele, OD, FAAO Please silence all mobile devices and remove items from chairs so others can sit. Unauthorized recording of this session is prohibited.

2 Disclosure Statement: Optos Advisory Board

3 Some things make you look twice. Worrisome findings. Elevation Pallor Discoloration NFL defects Vascular changes Tools you have Stereoscopic DFE! Swinging flashlight test Pupil cycle time Red free filter SVP VF OCT FAF B scan

4 Terminology Disc Edema Papilledema Bilateral papilledema Pseudopapilledema Pseudotumor Benign Idiopathic Intractranial Hypertension

5 Disc characteristics: Tortuous vessels Blurred margins Elevated topography Disc hemorrhages Papilledema on a silver platter 5

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7 Other causes of disc edema Unilateral Vascular Diabetic Papillopathy Bilateral Toxic Inflammatory Infectious Compressive Hydrocephalus

8 Is it? And if so when why is it? Pseudopapilledema ONH drusen Anatomically crowded discs Hyperopic disc Myopic / tilted / obliquely inserted disc

9 Myopic / tilted discs? Be careful not to hide behind a comfortable label

10 Exam VF Photography Clinical Tool Exam Papilledema Hyperemia, Dilated capillaries, Vessel obscuration Buried ONH Drusen Absence of physiologic cup Anomalous vessel branching Anatomically Crowded Disc Absence of cup, elevated appearance SVP Absent Present in 80% normals Present in 80% normals Visual Field Enlarged blind spot, Various defects (e.g. Normal central defect arcuate, central, etc) FAF Normal Increased FAF Normal

11 Spontaneous Venous Pulsation (SVP) Occurs when IOP > Central Retinal Vein pressure 80% of normals have an SVP What if it is absent? 20% of normals don t have one But if once present, and now gone consider CRV is subject to external compression where it crosses optic nerve sheath SVP is absent if CRV pressure elevated (due to ICP) Increased venous pressure upstream

12 FAF in ONH Drusen Hyper AF Buried harder to visualize hyper EDI superior to AF Loft et al. J Neurophthalmology Optos.com

13 Spectral Domain OCT Clinical Tool Papilledema Buried ONH Drusen Anatomically Crowded Disc OCT disc topography Smooth, continuous elevation Non uniform elevation Smooth, continuous elevation OCT RNFL scan Thicker RNFL, especially nasal Thicker hypo reflective space between retina and RPE ( lazy V ) Increased in acutely damaged tissue, may be decreased over time Normal OCT/MRI Globe convexity Flattened / pushed forward Normal/concave Normal/increased concavity for hyperopes

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16 Key features disc edema on OCT: Smooth contour of elevation Nasal RNFL >86um 80% specificity Thick hyporeflective space adjacent to disc lazy V 90% specificity Johnson L. Archives of Ophth 2009.

17 Globe Convexity Increased ICP will push the globe anteriorly Easiest to appreciate with a 9mm scan With EDI, can see an anteriorly displaced Bruch s membrane

18 Ultrasonography B scan Clinical Tool Papilledema Buried ONH Drusen Anatomically Crowded Disc B scan reflectivity Normal Hyper reflectivity with reduced gain Normal B scan ON sheath Increased Normal Decreased diameter B scan crescent present absent absent shadow B scan 30 Test Positive Negative Negative

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20 B scan Ultrasonography Review of Tools ONH Sheath Diameter <5mm Elevation of ONH <1mm Crescent sign 30 degree test (+) when >15% in nerve sheath width following a 30 degree eccentric gaze highly sensitive (100%) but non specific (36.4%) Kimberly HH, et al. Acad Emerg Med

21 43 AA Female 20/15 140/90 DFE: drance heme OD and disc elevated nasally Brightness comparison equal Pupil Cycle Time equal and normal +SVP

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24 Optic Disc Drusen review and new Up to 2.4% of population Hereditary component 75% bilateral Benign? Retrobulbar axonal degeneration due to drusen! 24 87% have VF defects Most commonly nasal, arcuate or partial arcuate Worsen over time Degree of VF defect correlates with RNFL thinning around the disc Deficit and prognosis worse when lesions are more superficial Chan et al. Clin and Exp Ophth July Auw Haedrich C, et al. Surv Ophthalmol 2002 Malmqvist L et al. J Neuroophthalmol March, Duncan, et al. J AAOPS, Feb, Hamann et al. Acta Ophth 2018.

25 What about in kids? Around 20% have VF defects Up to 24% have CNVM associated! 50% with elevated discs due to IIHTN also had ONH Drusen! Gospe SM III. Br J Ophth Auw Haedrich C, et al. Surv Ophthalmol 2002 Malmqvist L et al. J Neuroophthalmol March, Duncan, et al. J AAOPS, Feb, 2016.

26 At what point do you lower IOP in patient with drusen? 71 year old with longstanding ONH drusen Progressive VF loss OD>OS IOP averages 21mmHg OU

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28 Decrease in IOP : of retinal ganglion cell function Stabilizes RNFL thickness May delay the progression of optic neuropathy Podja Wilczek et al. Ophthalmic Res, 2017.

29 34 WF 20/20, pupils normal, CF normal 7.00 OU BMI 31 Denies H/A Elevated ONH.1/.1 CD +SVP

30 Normal RNFL thickness No subretinal cavitation

31 Globe convexity is normal Vs

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33 Measuring the ONH Sheath Diameter Axial images of the optic nerve (V and H) 3 mm behind the posterior eye wall Hyperechogenic borders

34 In summary ( ) hyperreflectivity consistent with drusen ( ) crescent sign Normal ONH sheath diameter Normal RNFL thickness +SVP Normal globe convexity Stability. Presumed crowded discs but careful follow up

35 32 AA FM Severe headaches, BMI 42

36 Disc characteristics: Tortuous vessels? Mildly indistinct margins Elevated topography Paton s folds? SVP absent (-) disc hemes

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38 Papilledema Suspected? Now what Brain Imaging MRI rule out space occupying mass MRV rule out cerebral venous thrombosis Lumbar Puncture Higher than 25/30 cm H2O is abnormal Referral? Determine underlying cause/association if any Weight Associated medications

39 MRI normal This patient: opening pressure = 28cm H 2 0

40 Idiopathic Intracranial HTN 90 98% complain of headache Nausea/vomiting/dizziness 40% Pulsatile tinnitus 16 60% Visual disturbances 30% Mollan SP, et al. Pract Neurol 2018

41 Etiologies Idiopathic But what causes the papilledema? Axoplasmic flow stasis swollen nerve fibers compression of venules in the area and so venous stasis/leakage accumula on of extracellular fluid Associated Medications Oral contraceptives Steroids Tamoxifen Vitamin A Nitrofurantoin Tetracyclines

42 IIH Treatment Trial JAMA, April 2014 acetazolamide + a low sodium weight reduction diet vs. diet alone modest improvement in visual field function OCT Substudy of the IITT, Ophthalmology, Sept 2015 Better RNFL thickness, TRT, and ONH volume swelling measurements RNFL and Total Retinal Thickness (TRT) useful in following and monitoring response to treatment

43 Acetazolamide + Low Sodium/Wt Loss Diet The Idiopathic Intracranial Hypertension Treatment Trial. JAMA ,250mg tablets, and 500mg SR capsules (Diamox Sequels, Duramed Pharmaceuticals) Contraindications: Sulfa allergy? chemical structure different than antibiotics little evidence of cross sensitivity consider avoiding if hx of severe reaction Long term: liver, kidney disease, severe COPD Caution with sickle cell Caution with low potassium paresthesia metallic taste fatigue malaise gastrointestinal disturbances Common Side Effects decreased libido metabolic acidosis electrolyte imbalance (including hypokalemia) renal calculi blood dyscrasias Than T, Smith H. must have oral meds.

44 Treatment Goals Visual Fields OCT Labs Referral? Mollan SP, et al. Pract Neurol 2018

45 Following Tx Diamox 250mg BID x 2 years

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47 Those tools can miss subtle elevation.. 3 years earlier was consistent with anatomical elevation; then lost to f/u x 3 years 3 years later patient decides to come back

48 21 AAF High BMI MRI clear Initial opening pressure of 52

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50 After 1 month of 250mg Diamox BID still (+) H/A

51 2 mos, then 5 mos (now TID Diamox) Denies H/A

52 Watching VF carefully H/A s improved RNFL thickness reduced Total volume reduced

53 Repeated Lumbar Puncture for Recalcitrant Cases? Not well reported Procedure causes anxiety, local discomfort, complications, headache LP induced reduction of ICP is only short lived Yiangou et al. Hoffman J. Cephalagia 2018.

54 4:30 on a Friday. 41 year old male has never had an eye exam LPE: 11 years ago No medications C/o: severe headache and blurred vision x 2 weeks BCVA: 20/30, 20/60 Pupils: appeared normal CFs: reduced OD, OS EOM: normal

55 Large meningioma at base of skull

56 Pre/post surgical RNFL scans can drive decisions for surgery (i.e. urgency) ing: become permanent Stable: may wait even if VF shows loss Comparison of pre vs. post surgical scans in neuro cases Meningioma can grow back, so useful for monitoring progression

57 26 WF, blurry vision Hx hydracephalous, multiple previous surgeries VA 20/40 OD, OS H/A developing with more intensity MRI all clear. ICP was borderline

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