2018 Northwest Residents Conference
Friday, June 8, 2018 1:00 pm‐6:30 pm (5.5 hours) 2018 Perspectives on Treatment & Management of Ocular Disease Posterior Segment (PS) |Part 1 • Bradley Jansen, OD: A Review of Choroidal Neovascularization. • Alina Pechko, OD: Late Onset Seropositive Neuromyelitis Optica: A Case Report and Review. • Dominic Lemas, OD: Macular Telangiectasia: A Condition with More Questions than Answers. • Lindsay Kleinschmit, OD: Clinical Applications of Fundus Auto fluorescence. • Zachary Vonderach, OD: Ocular Effects of Carotid Artery Disease; A Case Study. 2018 Perspectives on Glaucoma (GL) • Maria Jen, OD: The Chicken or the Egg: The Evolving Relationship Between Optic Nerve Structure and Function in Glaucoma. • Lindsey Rosencrans, OD: Future Considerations for Improved Detection of Glaucoma Progression with OCT.
2018 Perspectives on Treatment & Management of Ocular Disease Anterior Segment (AS) • Megan Mannen, OD: What Anterior Chamber!? Non‐traumatic Corneal Perforation Case Management.
• Christina Nguyen, OD: The Unusual Suspects: An Overview on Non‐typical Causes of Acute Intraocular Pressure Risers. 2018 Perspectives on Neuro‐Optometry (NO) • Sara George, OD: Perioptic Neuritis: A Case Study. • Kelsey Manescalco, OD: Papilledema vs. Pseudopapilledema in New Onset Exotropia.
1 Residents Conference Continuing Education Program
is supported by an education grant from Alcon Partners in Education
with additional support from Allergan Novartis
2 A REVIEW OF LEARNING OBJECTIVES Case presentation CHOROIDAL Types of CNV NEOVASCULARIZATION CNVM in AMD Other conditions with CNVM
BRADLEY JANSEN, OD No financial disclosures JUNE 8TH, 2018 SPOKANE VAMC
CASE PRESENTATION CASE PRESENTATION
77 YO WM presented for annual eye exam POHx: • Moderate senile cataracts OU CC: Distance and near vision were both blurry with his • Single stable choroidal nevus OD habitual specs, slow onset and constant for months to years ROS & Medications:
Last eye exam was ~ 1 year before • Extensive, yet non-contributory • Updated spec Rx, RTC 1 year for annual exam
CASE PRESENTATION CASE PRESENTATION
Entering DVA, CC: Pupils, EOMS, Confrontations, GAT • OD: 20/40- PH 20/30- • All WNL OU • OS: 20/30- PHNI Ant Seg: Refraction: • Moderate NS & CS cataracts OU • Stable low hyperopia & astigmatism OU • BCVA: Post Seg: • OD: 20/30- glare 20/50+ • ONH, macula, vitreous all WNL OU • OS: 20/30+ glare 20/50- • Choroidal nevus with drusen & MAs and hemes OD • And..
3 CASE PRESENTATION CASE PRESENTATION
What does this look like? Referred to retinal specialist, seen within 1 week
Assessment: • Peripheral CNVM OS • Choroidal nevus OD • Benign • MAs/dot hemes, suggesting occult BRVO
Plan • Avastin injection OS • Monitor OD
Acuity reducition was due to cataracts, asymptomatic for CNVM
4 CASE PRESENTATION
At retinal specialist, 5 months after initial visit photo • After 3 injections patient elected to discontinue treatment • Noted improvement in appaearance of lesion
At VA, 7 months after initial visit • Again visually significant cataracts (20/50 OD, 20/40 OS BCVA) • Resolving choroidal hemes and collections of HEs in the inf/temp mid-periphery OS, no neo or CNV seen • Still asymptomatic
PERIPHERAL EXUDATIVE HEMORRHAGIC CHORIORETINOPATHY photo Relatively uncommon disorder of the peripheral retina
Rarely described in literature
Many different names • Peripheral age-related degeneration • Peripheral choroidal neovascularization • Massive spontaneous retinal hemorrhage • Among others..
PEHCR PEHCR
Elderly, Caucasian, female Etiology unknown Inferior and temporal, usually in periphery Similarities to AMD Highly hemorrhagic and exudative in presentation Often misdiagnosed as malignant melanoma Episodic • Presence of large hemorrhage and exudation Possibly asymmetrical bilateral involvement with different sequence of events Usually asymptomatic, unless macular or vitreal involvement reaches visual axis • Pigment changes in fellow eye in unilateral cases
5 PEHCR TYPES OF CNVM
FA is gold standard for diagnosis Can be categorized histologically, angiographically
Can be self-limiting FA or ICGA • Hemorrhage, exudates resolve • gold standard • Leads to pigmentary changes and possible disciform scar • Literature states referral only if there is macular involvement OCT, OCT-A or the lesion is rapidly progressing
Anti-VEGF injection
Favorable prognosis
TYPE 1 - OCCULT TYPE 1 - OCCULT
CNV located below RPE OCT
TYPE 1 - OCCULT TYPE 1 - OCCULT
Fundus photo & FA OCT-A
6 TYPE 2 – CLASSIC TYPE 2 – CLASSIC
CNV located above RPE
TYPE 2 – CLASSIC TYPE 3 – INTRARETINAL RAP
Fundus photo & FA • Retinal angiomatous proliferation (RAP) • Intraretinal neovascular complex
• More on this in a moment
AMD AMD CNVM
Category 4 – Advanced AMD • Characterized by one or more of the following: • GA involving the fovea • Serous and/or hemorrhagic detachment of neurosensory retina or RPE • Disciform scar (subretinal fibrosis) • CNV
CNVM can be occult, classic, or both (mixed, “type 4”)
7 MYOPIC CHOROIDAL NEOVASCULAR AMD CNVM MEMBRANE
MYOPIC CNVM MYOPIC CNVM
Pathophysiology Retinal risk factors for CNV: • Progressive elongation of the globe • Tessellated fundus • Thinning of the chorioretinal tissue • Posterior staphyloma Patchy retinal atrophy • Abnormal choroidal vasculature • Choroidal thinning • Lacquer cracks Associated with high myopia • Patchy retinal atrophy • SE of -6D or greater, axial length of 26.5mm or greater
Pathologic myopia • SE of -8D or greater, axial length of 32.5mm or greater • High myopia with any posterior-specific pathology from axial elongation
MYOPIC CNVM MYOPIC CNVM
Tessellated fundus Posterior staphyloma
Posterior staphyloma
Fuchs spot
8 MYOPIC CNVM MYOPIC CNVM
Lacquer cracks
PERIPAPILLARY CHOROIDAL MYOPIC CNVM NEOVASCULAR MEMBRANE
Likely asymptomatic if extramacular If macular involvement: • Decreased vision, metamorphopsia, scotoma
FA is gold standard for diagnosis SD-OCT, OCTA
Anti-VEGF is standard treatment
Follow up: • Patients with high myopia, retinal risk factors, or known previous MCNV in one eye should be followed more closely
PERIPAPILLARY CNVM
9 PERIPAPILLARY CNVM PERIPAPILLARY CNVM
Likely asymptomatic if extramacular FA is gold standard • If macular involvement: • Blurred vision, distortion, scotoma, floaters • More likely to be symptomatic located temporal to nerve
Discolored subretinal lesion • Adjacent to nerve • Subretinal or sub-RPE hemorrhage may be present • Tend to grow toward the macula
PERIPAPILLARY CNVM PERIPAPILLARY CNVM
Lesion progression > 40 years old • Direct extension of membrane into macula • AMD, idiopathic • Serous or hemorrhagic detachment, lipid exudation • More commonly bilateral • Vitreous hemorrhage • Worse visual prognosis
Course of PCNVM Younger patients • Extremely variable • Extensive list of etiologies • Spontaneous resolution to significant macular involvement • More commonly unilateral • Poor prognosis if untreated • More favorable prognosis • Early treatment yields better prognosis
RETINAL ANGIOMATOUS PERIPAPILLARY CNVM PROLIFERATION
Treatment Unique form of AMD • Observation • Different than occult or classic CNVM • Anti-VEGF injection • “Type 3” • laser photocoagulation, steroid, PDT, surgery Early – intraretinal neovascularization • Intraretinal capillary proliferation • Small macular hemorrhages, sometimes punctiform • Associated with edema
Late – subretinal neovascularization & retinal-choroidal anastomosis • PED • CNVM
10 RAP RAP
RAP RAP Prevalence • Likely underreported • FA • OCT, OCT-A
• Estimated that 15-20% of wet AMD lesions in Caucasians are actually RAP
Treatment • Anti-VEGF
TAKE HOME REFERENCES
Luis Fernando Hernández-Zimbrón, Ruben Zamora-Alvarado, Lenin Ochoa-De la Paz, et al., “Age-Related Macular Degeneration: Many conditions with CNVM New Paradigms for Treatment and Management of AMD,” Oxidative Medicine and Cellular Longevity, vol. 2018, Article ID 8374647, 14 pages, 2018. doi:10.1155/2018/8374647 • Non-exhaustive list Cruickshanks KJ, Nondahl DM, Johnson LJ, Dalton DS, Fisher ME, Huang G, Klein BE, Klein R, Schubert CR. Generational Differences in the 5-Year Incidence of Age-Related Macular Degeneration. JAMA Ophthalmol.2017;135(12):1417–1423. doi:10.1001/jamaophthalmol.2017.5001
https://www.aao.org/preferred-practice-pattern/age-related-macular-degeneration-ppp-2015#references
FA is still gold standard for diagnosis Peripapillary subretinal neovascular membranes: A review • Fundoscopically Treating peripapillary choroidal neovascular membranes- a review of the evidence. pcv 1 Polypoidal Choroidal Vasculopathy Definition, Pathogenesis, Diagnosis, and Management
• OCT, likely OCT-A will continue to become more common Polypoidal Choroidal Vasculopathy: An Update on Therapeutic Approaches
pehcr 1 Peripheral Exudative Hemorrhagic Chorioretinopathy- A Clinical, Angiographic, and Histologic Study
Peripheral Exudative Hemorrhagic Chorioretinopathy in Patients With Nonexudative Age-Related Macular Degeneration
Anti-VEGF is mainstay treatment Treatment of peripheral exudative hemorrhagic chorioretinopathy by intravitreal injections of ranibizumab
Peripheral Exudative Hemorrhagic Chorioretinopathy- A Variant of Polypoidal Choroidal Vasculopathy?
Macular Post pole ppt
http://iovs.arvojournals.org/article.aspx?articleid=2667032
https://www.reviewofophthalmology.com/article/retinal-angiomatous-proliferation-in-amd
https://www.researchgate.net/figure/Classic-choroidal-neovascularisation-is-located-above-the-retinal-pigment- epithelium_fig1_264903506
http://eyewiki.aao.org/Myopic_CNVM
11 QUESTIONS?
Thank you!
12 + +
Optic Neuropathies of inflammatory etiologies affect primarily women between the ages of 20 and 60 years old
Late onset seropositive Alina V. Pechko, OD Neuromyelitis Optica: VA Puget Sound Health Care System a case report and review American Lake Division
+ + Case Report Personal Ocular History
71 year old Asian male Hx of optic neuritis OS in 2006 (likely OD too) CC: Woke up with sudden onset blurry Neurology work-up at the time was NEGATIVE per vision OS 2 days ago patient Moderate pain OS that is Optic nerve pallor OS > OD exacerbated by eye movement Unremarkable Family Ocular Hx
+ + HVF from 2015 (3 years ago) Personal Medical Hx
OS OD
Diabetes Mellitus Type 2
High cholesterol
Thalassemia
Hypothyrodism
13 + + Medications Entrance Testing
TEST OD OS Systemic Medications BCVA 20/20 20/25 Hydrochlorothiazide 25 mg po qd PUPILS PERRL (-)APD PERRL (+)trace APD Levothyroxine 0.05 mg po qd EOMs No restrictions No restrictions *pain on eye movement Losartan 100mg po qd FDT Clear Scattered non-specific defects RED CAP TEST Normal Desaturated ISHIHARA 14/14 plates 14/14 plates COLOR TEST
+ + Anterior Segment Posterior Segment
OD OS OD OS Lid/Lashes Unremarkable Unremarkable Lens 1+ NS 1+ NS Conjunctiva/sclera Clear/white Clear/white Vitreous Clear (-)cell Clear (-)cell Cornea Clear Clear Optic Nerve Pallor inferiorly Diffuse pallor Iris Flat and even Flat and even cup to disc ratio 0.35 0.30 Anterior chamber Deep and quiet Deep and quiet Macula Flat and even Flat and even Tonometry 08 mmHg 08 mmHg Vessels Normal Normal Periphery No breaks 360 No breaks 360
+ + Fundus Photos Macula OCT
14 + + RNFL OCT Tentative diagnosis:
Retrobulbar Optic Neuritis
PLAN: Patient was referred to ophthalmology same day
+ + While at ophthalmology office… HVF 30-2 #1 OS OD
Patient reported no pain on eye movement
Eye pain resolved with topical anesthetic
Patient’s symptoms were suspected to likely be a result of ocular surface issues and artificial tears were given to the patient
PLAN: Follow up in one month for HVF 30-2 and MRI to further investigate optic nerve pallor
+ MRI + MRI
Indeterminate for optic neuritis.
Some asymmetry in hyper-intensity of the posterior left optic nerve.
However, no other signs of inflammation and no optic nerve enhancement.
Axial plane T-1 weighted MRI with gadolinium enhancement
15 + + MRI – Optic Neuritis Reference Ophthalmology A/P
Optic Nerve Pallor OS>OD Inconsistent with Optic Neuritis Suspect pallor d/t repeated episodes of NAION
Refer to Neuroopthalmology for second opinion in 4 months
Bilateral optic neuritis
+ + Differential Dx for optic nerve 2 months later… appearance:
Inflammation While awaiting his neuroopthalmology Eg. Optic neuritis appointment patient returns to our Ischemia Ischemic optic neuropathy (NAION vs. AAION) clinic with sudden onset of blurry vision and inferior visual field loss Trauma in the left eye Compressive Lesion
Toxic/nutritional neuropathy
Infectious neuropathy
Congenital optic neuropathy
+ Second Presentation + HVF 30-2 2 months later… 2 months later
OD OS BCVA 20/20 20/40-2 PUPILS PERRL (-)APD PERRL (2+ APD) HRR COLOR Passed Failed all plates
Anterior and posterior segment findings are unchanged MD -12.58 DB P<0.5%
PSD 12.53 DB P<0.5%
16 Initial+ Presentation + VISUAL FIELD
Inferior altitudinal visual field defect is consistent with
Second Presentation (2 months later) ischemic optic neuropathy
+ + Lab orders to rule out GCA Assessment & Plan
Suspect non-arteritic ischemic optic neuropathy Test Result CBC w/ differential NORMAL Lack of optic nerve edema is likely due to already ESR NORMAL atrophic optic nerves CRP NORMAL
PLAN:
Contact neuroophthalmologist, request patient be seen sooner than his scheduled appt 2 months away.
+ +Visual Field at Neuroophthalmology office (2 days later)
Neuroophthalmology saw the patient in 2 days
Anterior & posterior exam findings unchanged MD -20.20DB P<0.5%
PSD 10.90DB P<0.5%
17 Initial+ Presentation + Ophthalmology A/P
Second Presentation (2 months later) ASSESSMENT:
Progressive optic neuropathy Acutely worsening on all parameters Lab work pending
2 DAYS LATER PLAN:
Consult neurology for steroid therapy given worsening VF
+ + At 2 week follow up
Neurology agrees After Steroid Initiation Tx Test OD OS BCVA 20/20 20/25 PUPILs No APD 2+ APD Patient was hospitalized and 3 day ISHIHARA 13/13 13/13 course of Solu-Medrol (IV) was started
+2 weeks after 3-day course of steroid Tx +Visual Field at Neuroophthalmology office (2 days later)
MD -6.16DB P<0.5% MD -20.20DB P<0.5%
PSD 8.68DB P<0.5% PSD 10.90DB P<0.5%
18 +2 weeks after 3-day course of steroid Tx Initial+ Presentation
Second Presentation (2 months later)
2 DAYS LATER
2 weeks after Steroid Tx
MD -6.16DB P<0.5%
PSD 8.68DB P<0.5%
+ MRI + Left retrobulbar optic neuritis MRI
Initial Presentation
Second Presentation
Axial plane T-1 weighted MRI with gadolinium enhancement
+ + Lab Results
Test Result CBC with diff Normal ESR Normal What is NMO Antibody? CRP Normal ACE Normal ANA Negative Syphilis panel Negative TB quant gold Positive NMO antibodies Strongly POSITIVE
19 + + Neuromyelitis Optica (NMO) History of NMO
Autoimmune inflammatory demyelination of Eugene Devic identified the disease in 1894 as: CNS but atypical for Multiple Sclerosis. Neuromyelitis Optica Associated with: (aka Devic’s Syndrome)
recurrent episodes of optic neuritis
transverse myelitis And for many years it has been considered as a variant of MS
+ + History of NMO Pathophysiology of NMO
The discovery of NMO antibody 1.2 million axons make up the optic nerve in 2004 led to recognition of Optic nerve axons are wrapped in myelin
Astrocytes are the housekeeping cell that are Neuromyelitis Optica as a abundant throughout the myelinated optic nerve distinct disease from MS Astrocytes also comprise a blood-brain-barrier with their foot processes
NMO MS
+ + Pathophysiology of NMO Pathophysiology of NMO
NMO patients produce antibodies that attack the AQP4 channels
AQP4 Antibody = NMO Antibody
Astrocytes express aquaporin-4 (AQP4) on cell membrane of astrocyte foot processes
AQP4 is bidirectional water channel present
20 + + Pathophysiology of NMO NMO Antibody
H2O
astrocyte
NORMAL NMO Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis optica.Prog Retin Eye Res,36, 159-171. doi:10.1016/j.preteyeres.2013.03.001
+ myelin + NMO Pathology astrocyte NMO-antibodies bind to aquaporin-4 AQP4 channel channels on Axon astrocytes
AQP4 Antibody Aka NMO Antibody
tight gap junctions
Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis optica.Prog Retin Eye Res,36, 159-171. optica.Prog Retin Eye Res,36, 159-171. doi:10.1016/j.preteyeres.2013.03.001 doi:10.1016/j.preteyeres.2013.03.001
Astrocyte disruption + +
Inflammatory response Astrocyte Death Gap Junction Axon Demyelination disruption
Neutrophils & macrophages eosinophils
Demyelinated axon Vacuolization of myelin
Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis optica.Prog Retin Eye Res,36, 159-171. Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis optica.Prog Retin Eye Res,36, 159-171. doi:10.1016/j.preteyeres.2013.03.001 doi:10.1016/j.preteyeres.2013.03.001
21 + + Pathophysiology of NMO Pathophysiology of NMO
NMO MS
AQP4-IgG binds to AQP4 on Primary: astrocyte destruction Primary: demyelination astrocyte Secondary: DEMYELINATION
Activates antibody effector Necrotic perivascular spinal Plaques of inflammatory function inflammation cord lesions demyelination within CNS
Astrocyte destruction Positive serum for NMO-IgG Negative serum for NMO-IgG
MRI: posterior ON involvement, Rarely involves chiasm and rarely Demyelination sometimes includes optic chiasm presents as bilateral optic or bilateral optic neuritis neuritis.
+ + Clinical features of NMO Clinical features
Optic Neuritis (often bilateral) NMO MS Transverse Myelitis Vision loss (bilaterally >20/200) Common (50% in 10 years) Uncommon (4.2% in 11 years) Symmetric paraparesis or quadriparesis Severe myelitis (complete paraplegia) Brainstem syndromes Common (30-70% at first attack) Rare Nausea and vomiting Rate of disability progression Hiccups (sometimes intractable) Fast (median 12 yrs to EDSS 6) Slow (median 23 yrs to EDSS 6) Mortality Acute neurogenic respiratory failure and death High (5-yr survival as low as 68%) Low (life expectancy reduced by 7- 14 years compared to general)
*** EDSS = expanded disability status scale *** EDSS 6 = walking aid required
+ + Epidemiology of NMO Diagnostic Criteria
Test for NMO-Ab NMO MS Prevalence Up to 10 per 100, 000 90 per 100,000 in US Approximately 25% of NMO across different studies
Demographics Asian, Indian, Black Caucasian patients are seronegative Test Gender predilection Up to 10 : 1 2.3 : 1 Indirect immunofluorescence (IIF) (women : men) (women : men) Enzyme-linked immunosorbent assay (ELISA) Age of onset 32 – 41 years old 24 years old Cell-based assay (CBA)
*Epidemiology of NMO is not clearly established due to high Flow cytometry assay (FACS-assay) rate of misdiagnosis as MS
22 + + NEW TERMINOLOGY Diagnostic Criteria
For NMO-IgG SEROPOSITIVE
Neuromyelitis Optica Spectrum Positive test for NMO-IgG (aka AQP4 IgG) Disorders (NMOSD) Exclusion of alternative diagnoses includes seronegative and seropositive patients At least 1 core clinical characteristic
Adapted from: Wingerchuk, DM et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015, 85:177-189
+ + Diagnostic Criteria OUR PATIENT Wingerchuk’s criteria
Core Clinical Characteristics Fits Diagnostic Criteria Differs from Diagnostic Criteria Optic Neuritis + Optic Neuritis - acute myelitis Acute myelitis + Strongly Seropositive for - brainstem syndrome Area postrema syndrome: hiccups/nausea/vomiting NMO-IgG Acute brainstem syndrome: diplopia/vertigo/imbalance + Posterior optic nerve - area postrema syndrome involvement on MRI Symptomatic narcolepsy or acute diencephalic clinical syndrome with NMOSD-typical diencephalic MRI lesions Symptomatic cerebral syndrome with NMOSD-typical brain lesions
Adapted from: Wingerchuk, DM et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015, 85:177-189
+ + What makes this case unique Treatment & Management
NMO Our Patient There is no curative treatment for NMO Vision loss (bilaterally >20/200) Recovered to 20/25 The goal in NMO is to: Common (50% in 10 years) + good visual field recovery keep patient in remission Severe myelitis (complete No myelitis Manage symptoms in relapse phase paraplegia) Long-term stabilization Common (30-70% at first attack) Mortality First reported ON in 2006 High (5-yr survival as low as 68%) ??onset of the disease?? Due to rarity of the disease, all treatment options and our Age of onset 32-41 years old 71 years old (questionable onset at understanding of it is based on case reports and retrospective 60 years old) studies Gender predilection Male women : men (10 : 1) No long term, controlled, prospective studies have been done to date
23 + + Treatment & Management Treatment & Management
Immunosuppressive agents Systemic Steroids (IV) in acute phase Azathioprine Methylprednisone Mycophenolate mofetin Solu-Medrol Rituximab Therapeutic plasma exchange in acute phase Methotrexate (as second line drug) Can be done if steroid Tx is not effective enough
Immunosuppressive Agents For chronic long-term management To prevent relapses or weaken the course of the disease relapse
+ + Treatment & Management Treatment & Management
Potentially harmful agents in NMO Immunosuppressive agents used in treatment of MS Fingolimod Fingolimod - Severe relapses Natalizumab Natalizumab - Severe relapses Interferon-B Interferon-B - Can exacerbate NMO Azathioprine Mycophenolate mofetin Rituximab And others…
+ + Treatment & Management Etiologies that can mimic NMO our patient
Due to positive TB, long-term immunosuppressive treatment Multiple sclerosis is delayed.
Patient is currently undergoing treatment with isoniazid for Inflammatory diseases TB. Malignancy Patient will begin immunosuppressive treatment for NMO with Rituximab after he is cleared by infectious disease Infection doctor. Vascular disease
24 + + Summary of NMO Acknowledgements
Severe vision loss Jeffrey A. Hiett, OD Fast disability progression Judith E. Oh, OD High mortality rate
Treatment may differ from MS
+ + References References
1. Alvarenga, M., Schimidt, S., & Alvarenga, R. P. (2017). Epidemiology of neuromyelitis optica in Latin 8. Jarius, S., Ruprecht, K., Wildemann, B., Kuempfel, T., Ringelstein, M., Geis, C., . . . Paul, F. (2012). America. Multiple Sclerosis Journal – Experimental, Translational and Clinical,3(3), 205521731773009. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre doi:10.1177/2055217317730098 study of 175 patients.Journal of Neuroinflammation,9(1). doi:10.1186/1742-2094-9-14
2. Barnett, M. H., & Sutton, I. (2012). Neuromyelitis optica.Current Opinion in Neurology,25(3), 215-220. 9. Jiao, Y., Fryer, J. P., Lennon, V. A., Jenkins, S. M., Quek, A. M., Smith, C. Y., . . . Pittock, S. J. (2013). Updated doi:10.1097/wco.0b013e3283533a3f estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica. Neurology,81(14), 1197-1204. doi:10.1212/wnl.0b013e3182a6cb5c 3. Bennett, J., Seze, J. D., Lana-Peixoto, M., Palace, J., Waldman, A., Schippling, S., . . . Paul, F. (2015). Neuromyelitis optica and multiple sclerosis: Seeing differences through optical coherence 10. Khanna, S., Sharma, A., Huecker, J., Gordon, M., Naismith, R. T., & Stavern, G. P. (2012). Magnetic tomography.Multiple Sclerosis Journal,21(6), 678-688. doi:10.1177/1352458514567216 Resonance Imaging of Optic Neuritis in Patients With Neuromyelitis Optica Versus Multiple Sclerosis. Journal of Neuro-Ophthalmology,32(3), 216-220. doi:10.1097/wno.0b013e318254c62d 4. Drori, T., & Chapman, J. (2014). Diagnosis and classification of neuromyelitis optica (Devics Syndrome).Autoimmunity Reviews,13(4-5), 531-533. doi:10.1016/j.autrev.2014.01.034 11. Levin, M. H., Bennett, J. L., & Verkman, A. S. (2013). Optic neuritis in neuromyelitis optica.Prog Retin Eye Res,36, 159-171. doi:10.1016/j.preteyeres.2013.03.001 5. Glisson, C. C., DO, MS. (2017, September). Neuromyelitis optica spectrum disorders (F. Gonzalez- Scarano MD, Ed.). Retrieved January 12, 2018, from https://www.uptodate.com/contents/neuromyelitis- 12. Mealy, M. A., Whetstone, A., Orman, G., Izbudak, I., Calabresi, P. A., & Levy, M. (2015). Longitudinally optica-spectrum-disorders extensive optic neuritis as an MRI biomarker distinguishes neuromyelitis optica from multiple sclerosis. Journal of the Neurological Sciences,355(1-2), 59-63. doi:10.1016/j.jns.2015.05.013 6. Jarius, S., Wildemann, B., & Paul, F. (2014). Neuromyelitis optica: Clinical features, immunopathogenesis and treatment.Clinical & Experimental Immunology,176(2), 149-164. doi:10.1111/cei.12271 13. Trebst, C., Jarius, S., Berthele, A., Paul, F., Schippling, S., Wildemann, B., . . . Kümpfel, T. (2013). Update on the diagnosis and treatment of neuromyelitis optica: Recommendations of the Neuromyelitis Optica 7. Jarius, S., Wildemann, B., & Paul, F. (2014). Neuromyelitis optica: Clinical features, immunopathogenesis Study Group (NEMOS).Journal of Neurology,261(1), 1-16. doi:10.1007/s00415-013-7169-7 and treatment.Clinical & Experimental Immunology,176(2), 149-164. doi:10.1111/cei.12271
+ + References
14. Wildéa Lice De Carvalho Jennings Pereira, Reiche, E. M., Kallaur, A. P., & Kaimen-Maciel, D. R. (2015). Epidemiological, clinical, and immunological characteristics of neuromyelitis optica: A review. Journal of the Neurological Sciences,355(1-2), 7-17. doi:10.1016/j.jns.2015.05.034
15. Wingerchuk, D. M., Hogancamp, W. F., Obrien, P. C., & Weinshenker, B. G. (1999). The clinical course of neuromyelitis optica (Devics syndrome).Neurology,53(5), 1107-1107. doi:10.1212/wnl.53.5.1107
16. Wingerchuk, D. M., Lennon, V. A., Pittock, S. J., Lucchinetti, C. F., & Weinshenker, B. G. (2006). Revised diagnostic criteria for neuromyelitis optica.Neurology,66(10), 1485-1489. Thank you for your attention! doi:10.1212/01.wnl.0000216139.44259.74
17. Wingerchuk, D. M. (2009). Neuromyelitis optica: Effect of gender.Journal of the Neurological Sciences,286(1-2), 18-23. doi:10.1016/j.jns.2009.08.045
18. Wingerchuk, D. M., Banwell, B., Bennett, J. L., Cabre, P., Carroll, W., Chitnis, T., . . . Weinshenker, B. G. (2015). International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology,85(2), 177-189. doi:10.1212/wnl.0000000000001729
19. Wingerchuk, D. M., & Weinshenker, B. G. (2017). Neuromyelitis optica spectrum disorder diagnostic criteria: Sensitivity and specificity are both important.Multiple Sclerosis Journal,23(2), 182-184. doi:10.1177/1352458516688352
20. Wu, G. F., & Alvarez, E. (2011). The Immunopathophysiology of Multiple Sclerosis.Neurologic Clinics,29(2), 257-278. doi:10.1016/j.ncl.2010.12.009
25 +
Questions?
26 44 YO M presents for CEE August 2017
Macular CC: Broke glasses and needs new ones. No concerns about vision or ocular health SCVA: OD: 20/50-2 PH: 20/40 Telangiectasia, OS: 20/60 PH: 20/40 Entrance Skills: Unremarkable Type 2 Refraction: OD: +0.50 DS 20/40-1 OS: +1.25-0.50 x 155 20/30 BCVA @ previous exam at Portland VA 2014 OD: 20/25 OS: 20/25 Anterior seg and IOP: Unremarkable BY DOMINIC LEMAS, OD Posterior seg: Small area of pigment hyperplasia temporal from fovea OU OCT: Disruption of RPE and overlying retinal layers temp to fovea OU Plan: Referred to retinal specialist for evaluation and recommendations
Day after exam with retinal specialist, patient presents to clinic stating he needs to discuss diagnosis Patient hands me sticky note that states “Macular Telangiectasia Type 2” and says that was the diagnosis Patient visually upset and very anxious. States he was told he would “slowly go blind” and there is no treatment Was told that cause is unknown, genetics are unknown, treatment is unknown. Has questions I can not answer
Has twin sister and a son. Wants to know risk of inheritance
Wants to know what the next step is
Wants to know what I have heard about treatment for this condition
Informed him I will look into the research about condition and call him with answers to his questions.
Macular telangiectasia Three types of MacTel (MacTel)
Classified into three types
Type 1: Unilateral and mostly affecting middle age men with classic AKA: idiopathic juxtafoveolar retinal telangiectasia signs of hard exudates and macular edema Group of rare retinal vascular disorders first described in 1982 Type 2: Most common of the three. Bilateral w/ no gender predilection Identified by J. Donald Gass, an American-Canadian retinal specialist causing slow degeneration of macular layers and appearance of refractile deposits. Vision loss is slow unless NVM develops which speeds Gass and Blondi updated the classification of MacTel in 1993 resulting in loss of vision three separate types Type 3: Extremely rare, no gender predilection causing capillary In 2006, Yunuzzi, an American ophthalmologist, further refined the obliteration and slow, progressive vision loss. Sometimes associated with classification of these conditions based on results from new imaging central nervous system symptoms technology
27 Prevalence Macular Telangiectasia Project
Rare condition making accurate representative studies difficult Beaver Dam Eye Study International research effort to better understand the clinical Study of population in Beaver Dam, WI that analyzed colored fundus presentation of Mac Tel and to research risk factors and treatment photos for signs of MacTel 2. methods Subjects 99% Caucasian More than 30 centers around the world participate in the MacTel Prevalence: 0.1% Project Melbourne Collaborative Cohort Study Originally started in 2005 Australian study analyzing colored fundus photos for signs of MacTel 2 Largest population of Macular Telangiectasia of any study to date Subjects 100% Caucasian Over 1000 participants so far Prevalence 0.0045% to 0.022% Ability to analyze genetics of condition with statistical evidence African Study
Assessment of colored fundus photos from subjects in Kenya and Nigeria
Prevalence 0.06%
Patient Characteristics No evidence of gender predilection Symptoms Recent evidence suggests slight female predilection (64% F MacTel Project)
No evidence of racial predilection Typically asymptomatic in early stages Possible Caucasian predilection (MacTel Project) First complaints: blurred vision, metamorphopsia, paracentral scotomas presenting in mid to late 50s Mean age of diagnosis is 57 YO (MacTel Project) Patients often present with complaints of reading Risk Factors difficulty due to paracentral scotomas (Scotomas always present temporal from fixation) No statistical evidence of genetic factor Progression often slow so patient can be unaware of changes in vision Hypertension? (MacTel Project) (52%)
Diabetes? (MacTel Project) (28%)
Fundus findings
Findings are often bilateral and usually found temporal to fovea Early findings:
subtle loss of retinal transparency in parafoveal area (Earliest finding)
Blunted, dilated venules that may make right angle turn
Pigment hyperplasia and crystalline deposits around abnormal vessels
Some develop yellow pseudovitelliform lesion of maculas Advanced stage findings:
33% of patients develop choroidal neovascular membrane
Disciform scar can develop at end stage of condition
28 OCT Findings
All findings commonly seen temporal to fovea Loss and disruption of photoreceptor layer (ellipsoid zone)
If photoreceptor layer intact, VA is often preserved
Intra-retinal cysts
Internal limiting membrane drape
Foveolar atrophy and thinning
May mimic a lamellar macular hole
Not to mistaken for choroidal leakage
Fluorescein Angiography Prognosis Findings Loss of VA is slow but progressive Originally the only diagnostic tool of choice 1.2 lines of VA loss by 3 year for condition. Now require either FA or 3.4 lines of VA loss by 10 years OCT angiography for confirmed diagnosis *First long term study of MacTel
Telangiectatic vessels (Hallmark finding) VA loss often bilateral but asymmetric Seen as early hyperfluorescence with late phase leakage 1/3 of people get secondary CNVM which causes increased rate of vision loss and less favorable outcomes Findings are often parafoveal and temporal to fovea
Pathogenesis Treatment and Management
No agreed upon treatment modality for non-CNVM form Unknown Usually close observation until choroidal neovascularization forms Anti- VEGF, focal grid laser photocoagulation or photodynamic No genetic factor established (studies disagree on influence of genetics) therapy were treatments in the past
Possible genetic association based on monozygotic twin evidence (MacTel Proj) Studies show no beneficial outcome unless treating CNVM Most popular theory is dysfunctional Muller cells Managed/monitored by retinal specialist Death of Muller cells lead to death of endothelial cells and subsequent retinal Once diagnosed, retinal specialists recommend observation every atrophy 1-3 months to monitor for neovascularization Crystalline deposits postulated to be remnants of Muller cells No genetic factor established, so genetic counseling not endorsed Evidence found in histological studies of MacTel project donated eyes Slow loss of vision so get patient connected with occupational Unsure of cause of Muller Cell death therapists and Low Vision services early on
29 Differential Diagnosis The unknowns
Rare disease so rule out other conditions before diagnosis Pathogenesis
Coats disease
Diabetic macular edema Racial predilection Crystalline retinopathy
Lamellar macular hole Risk factors/ systemic associations Wet macular degeneration
Genetics and inheritance
Key difference is parafoveal telangiectasia temporal to fovea Most appropriate management other than monitoring for neovascularization
References Management of case Chen, John J, et al. “Idiopathic Juxtafoveal Telangiectasia Type II (Macular Telangiectasia Type 2).” A Primer on Ptosis, University of Iowa, 17 Feb. 2014, webeye.ophth.uiowa.edu/eyeforum/cases/185-JXT.htm.
Chew, Emily Y, and Lawrence A Yannuzzi. “Macular Telangiectasia.” Retinal Vascular Disease, 2018. Patient not satisfied with lack of information about condition Clinicalkey.com, macular telangiectasia.
Requested second opinion→ referred to retina clinic at Portland VA Cooper, Erin S, et al. “Exam Reveals Subtle Macular Disease.” Review Of Optometry, 12 Mar. 2015, www.reviewofoptometry.com/article/exam-reveals-subtle-macular-disease.
Gass, J. Donald M., and Barbara A. Blodi. “Idiopathic Juxtafoveolar Retinal Telangiectasis.” Ophthalmology, vol. 100, no. 10, 1993, pp. 1536–1546., doi:10.1016/s0161-6420(93)31447-8.
Referred patient to Visual Impairment Services Team at SORCC Mehta, Hemal, et al. “Natural History and Effect of Therapeutic Interventions on Subretinal Fluid Causing Foveal Detachment in Macular Telangiectasia Type 2.” British Journal of Ophthalmology, vol. 101, no. 7, 2016, pp. 955–959., doi:10.1136/bjophthalmol-2016-309237.
Meyer-Ter-Vehn, Tobias, et al. “Long-Term Course in Type 2 Idiopathic Macular Telangiectasia.” Graefe's Archive for Clinical and Experimental Ophthalmology, vol. 251, no. 11, 2013, pp. 2513–2520., doi:10.1007/s00417-013-2346-z.
Most Recent Follow-up Peto, Tunde, et al. “Correlation Of Clinical And Structural Progression With Visual Acuity Loss In Macular Telangiectasia Type 2.” Retina, vol. 38, no. 1, 2017, pp. 8–13., doi:10.1097/iae.0000000000001697. LEE: April 2018 at Portland VA Klein, Ronald, et al. “The Prevalence of Macular Telangiectasia Type 2 in the Beaver Dam Eye Study.” American BCVA OD: 20/150 OS: 20/100-2 Journal of Ophthalmology, vol. 150, no. 1, 2010, doi:10.1016/j.ajo.2010.02.013. No neovascularization Tan, Anna C.s., et al. “Treatment of Macular Telangiectasia Type 2 With Carotenoid Supplements Containing Meso- Zeaxanthin: A Pilot Study.” Ophthalmic Surgery, Lasers and Imaging Retina, vol. 47, no. 6, 19 Apr. 2016, pp. 528–535., OCT: ILM draping OD ; cystic spaces OS doi:10.3928/23258160-20160601-04. Vujosevic, Stela, et al. “Scotoma Characteristics In Macular Telangiectasia Type 2.” Retina, vol. 38, no. 1, 2018, pp. 14–19., doi:10.1097/iae.0000000000001693. Patient unable to read without magnifiers Yannuzzi, Lawrence A. “Idiopathic Macular Telangiectasia.” Archives of Ophthalmology, vol. 124, no. 4, 2006, pp. Patient no longer legal to drive 450–460., doi:10.1001/archopht.124.4.450.
30 Objectives: By the end of the presentation, attendees will be able to describe the principles behind fundus autofluorescence, as well as recognize certain patterns and presentations of common retinal diseases Clinical Applications of imaged with FAF. Fundus Autofluorescence No disclosures
Lindsay Kleinschmit, OD Mann-Grandstaff VAMC Spokane, WA
Fluorophores Fundus Autofluorescence Lipofuscin Primary fluorophore Allows a different view of the fundus Accumulates in the RPE Fluorescence: molecules (fluorophores) absorb light at a specific Often early marker of disease/degeneration wavelength, and release it at a longer, less energetic wavelength Decreased fluorescence Signals emitted between 500-800nm Melanin Overview of metabolic and overall health of RPE and photoreceptors Luteal pigment (lutein + zeaxanthin) Rhodopsin Concentrated in photoreceptor outer segments
Normal Autofluorescence Normal Autofluorescence
Optic nerve head Healthy fundus Dark Mild diffuse hyperfluorescence Absence of lipofuscin/RPE Due to normal levels of lipofuscin in the RPE Macula Retinal vessels are dark due to blocked fluorescence Reduced fluorescence Signal absorption from blood Luteal pigment (absorbs light)
31 The Lens and cornea
Emit natural autofluorescence Auto-fluorescence from the cornea increases in diabetic patients Build up of cellular waste Increase in glucose, proteins, and collagen Cataracts Absorb light Produce poor contrast Fundus cameras – filters with red shifted wavelengths Helps filter out lens AF
Stargardt’s Lipofuscin- opathies Increased Pattern Reduced FAF lipofuscin Dystrophies Drusen RPE loss or atrophy Intra-retinal fluid Best’s Disease Fibrosis Vitelliform Dystrophy Adult Onset Sub-retinal Areas of dense hyperpigmentation Vitelliform material Dystrophy Blood (blockage by material anterior to the RPE/photoreceptors) CSCR
Increased FAF Hyper- fluorescence Large soft drusen MEWDS White Dot Lipofuscin accumulation Syndromes Multifocal Old hemorrhages may have fluorophore buildup choroiditis Window CSCR Window defect Defect
MacTel Type 2
Optic Disc Other Drusen
Yung, M., Klufas, M. A., & Sarraf, D. (2016). Clinical applications of fundus autofluorescence in retinal disease. International Journal of Retina and Vitreous, 2, 12. http://doi.org/10.1186/s40942-016-0035-x
Geographic Atrophy RPE atrophy Ways to Image Reduced CSCR Lipofuscin cSLO RPE tear Uses a laser to scan the retina Typically a series of images (9-16, averaged)
Media Clearer images Hypo- Opacities fluorescence Cannot proceed FA Lutein Fundus camera Macular pigments One image / much quicker Blockage Zeaxanthin Reduced motion artifact (material More reflected and scattered light anterior to RPE) Intra- or Sub- Yung, M., Klufas, M. A., & Sarraf, D. (2016). Clinical applications of fundus New filters help reduce cataract artifact autofluorescence in retinal disease. International Journal of Retina and Vitreous, 2, 12. retinal Pigment http://doi.org/10.1186/s40942-016-0035-x hemorrhage ONH does not appear as dark Wide field - Optos Fibrosis and scar tissue
Yung, M., Klufas, M. A., & Sarraf, D. (2016). Clinical applications of fundus autofluorescence in retinal disease. International Journal of Retina and Vitreous, 2, 12. http://doi.org/10.1186/s40942-016-0035-x
32 Age Related Macular Degeneration
FAF abnormalities and fundoscopy do not always correspond May appear more widespread on FAF Can be used to detect conversion to neovascular ARMD as well as risk of progression 8 patterns associated with ARMD Patchy pattern Strongest correlation with conversion 30.4% in 30 months Linear and reticular patterns also associated
Drusen
Variable appearance on FAF Small hard drusen may be iso-autofluorescent Not visible on FAF Large soft drusen typically hyper-FAF
Dominant Drusen Geographic Atrophy
Decreased fluorescence due to the absence of lipofuscin and atrophy of the RPE Junctional zone Border between unaffected retina and GA Increased AF = Extension area More hyper fluorescent = high extension rate Most predictive factor of progression GA classified into 5 categories Higher risk of disease progression Diffuse (Esp diffuse trickling) Banded
33 FAF in Geographic Atrophy
Fritsche, L. G., Fleckenstein, M., Fiebig, B. S., Schmitz-Valckenberg, S., Bindewald-Wittich, A., Keilhauer, CN, Weber, B. H. (2012). A Subgroup of Age-Related Macular Degeneration is Associated With Mono-Allelic Sequence Variants in theABCA4Gene. Investigative Opthalmology & Visual Science, 53(4), 2112. doi:10.1167/iovs.11-8785
Choroidal Neovascularization Resolving CNVM
Early neo often not detectable on FAF Intact RPE and photoreceptor layer Classic CNV Typically reduced FAF Blocked by neo vessels or fibrovascularization Occult CNV Variable More studies need to be done!
Fibrous scarring Diabetic Retinopathy
Minimal information at this time Primarily associated with DME Overall increased FAF in photos due to increased corneal AF DME/CSME Typically increased macular FAF Correspond to cystoid fluid spaces
34 Optic Disc Drusen
Drusen are hyper-autofluorescent Typically superficial drusen, mildly buried drusen FAF associated with the mitochondria
Incidental Disc Drusen
Plaquenil Choroidal Nevi & Melanomas
Early damage: increased parafoveal autofluorescent ring Associated with photoreceptor loss/scotomas Orange pigment = lipofuscin Asian populations tend to show photoreceptor loss occurring further from the Bright! hyperfluorescence fovea Helpful in amelanotic lesions (harder to see orange pigment) FAF may show when OCT does not Study done showed Hyper-FAF and LF were found in 90% of melanomas Nevi Often do not FAF May hypo-AF RPE atrophy/melanin
http://imagebank.asrs.org/file/6155/plaquenil-toxicity
35 Vitelliform and Nevus Vitelliform lesions
Consist of extracellular fluorophores Hyper-autofluorescent Best’s, Vitelliform macular dystrophy
Central Serous Chorioretinopathy
Various FAF patterns Initial presentation Hypo or Hyper-AF Hypo due to blockage by subretinal fluid Hyper corresponds to area of serous fluid Eventually will show granular hyper- autofluorescence
Gabai, A., Veritti, D., & Lanzetta, P. (2015). Fundus autofluorescence applications in retinal imaging. Indian Journal of Ophthalmology, 63(5), 406–415. http://doi.org/10.4103/0301-4738.159868
36 POHS
FAF can also be used for White dot syndromes Other drug toxicity Glaucoma? Artery/Vein Occlusions Retinitis Pigmentosa Stargardt’s Macular holes Commonly increased due to enhanced visibility of RPE
Retinitis Pigmentosa MEWDS
https://journalretinavitreous.biomedcentral.com/articles/10.1186/s40942-016-0035-x https://journalretinavitreous.biomedcentral.com/articles/10.1186/s40942-016-0035-x
Peripapillary CNVM Cone – Rod Dystrophy
37 References – APA Q?
Shahid, K. (2013, January). Fundamentals of Fundus Autofluorescence Imaging. Retrieved April 24, 2018, from https://www.reviewofoptometry.com/ce/fundamentals-of-fundus-autofluorescence-imaging Ly, A., Nivison-Smith, L., Assaad, N., & Kalloniatis, M. (2017). Fundus Autofluorescence in Age-related Macular Degeneration. Optometry and Vision Science, 94(2), 246–259. http://doi.org/10.1097/OPX.0000000000000997 Sepah, Y., Akhtar, A., Hafeez, Y., Nasir, H., Perez, B., Mawji, N., Nguyen, Q. (2014). Fundus Autofluorescence Imaging: Fundamentals and Clinical Relevance. Ophthalmological Imaging and Applications, 28(2), 117-126. doi:10.1201/b17026- 7Gündüz, K., Pulido, J. S., Bakri, S. J., & Petit-Fond, E. (2007). Fundus Autofluorescence In Choroidal Melanocytic Lesions. Retina, 27(6), 681-687. doi:10.1097/iae.0b013e31812565d2 Gabai, A., Veritti, D., & Lanzetta, P. (2015). Fundus autofluorescence applications in retinal imaging. Indian Journal of Ophthalmology, 63(5), 406–415. http://doi.org/10.4103/0301-4738.159868 Schmidt, J. , Peters, S. , Sauer, L. , Schweitzer, D. , Klemm, M. , Augsten, R. , Müller, N. and Hammer, M. (2017), Fundus autofluorescence lifetimes are increased in non‐proliferative diabetic retinopathy. Acta Ophthalmol, 95: 33-40. doi:10.1111/aos.13174 Frampton GK, Kalita N, Payne L, Colquitt J, Loveman E. Accuracy of fundus autofluorescence imaging for the diagnosis and monitoring of retinal conditions: a systematic review. Health Technol Assess 2016;20(31) Yung, M., Klufas, M. A., & Sarraf, D. (2016). Clinical applications of fundus autofluorescence in retinal disease. International Journal of Retina and Vitreous, 2, 12. http://doi.org/10.1186/s40942-016-0035-x Ly, A., Nivison-Smith, L., Assaad, N., & Kalloniatis, M. (2017). Fundus Autofluorescence in Age-related Macular Degeneration. Optometry and Vision Science, 94(2), 246–259. http://doi.org/10.1097/OPX.0000000000000997 Shahid, K. (2013, January 13). Fundamentals of Fundus Autofluorescence Imaging. Retrieved April 13, 2018, from https://www.reviewofoptometry.com/ce/fundamentals-of-fundus-autofluorescence-imaging#faf3
38 6/6/2018
Stroke • Stroke is the fourth leading cause of mortality. Ocular Effects of Carotid Artery • 750,000 Americans experience a new or recurrent stroke annually. Disease • 80% of strokes are ischemic due to carotid artery stenosis. ZACHARY VONDERACH, O.D. • The economic burden in North America is astounding, costing the PUGET SOUND VA –AMERICAN LAKE DIVISION healthcare system more than 50 billion dollars annually in the United JUNE 2018 States.
Case 78 YOM
CASE 78 YOM presents for 24‐2 HVF Previous Assessment and Plan • Sclerosed arteriole distal to possible embolitic lesion LE, suspect branch retinal artery occlusion, cardiovascular risk factors including carotid disease R+L , stable appearance. Possible progression of LE inf optic nerve RNFL thinning on OCT today// Pt ed on findings. Continue carotid monitoring, patient reports that Vet will schedule f/u with outside provider. Currently monitor every 12 months. RTC for HVF 24‐2 R+L in 6 weeks. • Macular atrophy RE, suspect branch artery/vein occlusion, likely stable and longstanding. Cardiovascular risk factors . // Monitor Annually. • DM2 – controlled without retinopathy. • Pseudophakia OD –s/p YAG //stable monitor • Cataract OS –not visually significant // monitor • Corneal scars R+L – secondary to trauma, longstanding, stable// Monitor • Choroidal Nevi OS – longstanding stable//Monitor
39 1 6/6/2018
Medical History Medications Case Report • DM2 –since 2001 • Insulin BID • CC: No visual or ocular complaints. Vision has been stable. A1C 7.9; Avg BS 100 • Metformin HCL 500mg TID • Carotid Occlusive Disease • Losartan 50mg QD • 50‐79% stenosis Test OD OS • Metoprolol Succinate 50mg • Coronary Arteriosclerosis Visual acuity (cc) 20/25 20/20 • Heart attack 1990 • Rosuvastatin 40mg • Hypertension ‐135/84 • Aspirin 81mg QD EOMs Full Full • Mixed Hyperlipidemia Pupils REL, (‐)APD REL, (‐)APD • Dyslipidemia IOP 14 15 • Smoker from 1973‐1983
Clinical Findings OD OS Lens PCIOL, Open Capsule 1+NS Fundus Photo Vitreous (+)PVD (‐)Cell (‐)PVD (‐)Cell Optic Nerve Distinct Margins Distinct Margins 0.30R 0.35R Macula Flat and Dry, retinal atrophy superior nasal Flat and Dry, Even Pigment
Vessels Copper wiring, A/V nicking, crossing changes Copper wiring, A/V nicking, crossing changes. HHP at ONH , (+) multiple hhp. Arterial ghost vessel Periphery No breaks 360 No breaks 360 1DD nevus superior temporal –flat (‐) lipofuscin Pinpoint RPE disturbance inf temp
Multicolor Fundus Photo Imaging
Heidelberg Spectralis OCT
40 2 6/6/2018
Multicolor Multicolor Imaging Imaging
Heidelberg Spectralis OCT Heidelberg Spectralis OCT
IR Wide Field IR Wide Field
Macula OCT Macula OCT
41 3 6/6/2018
OCT‐A •Motion contrast imaging to high‐resolution volumetric blood flow information generating angiographic images. •The en‐face images can be scrolled from ILM to choroid in order to visualize the individual vascular plexus and segment the inner retina, outer retina, choriocapillaris, or other area of interest.
•Artery Occlusions • Wedge –shaped areas of capillary non perfusion that correlate to areas of abnormalities on retinal thickness map.
42 4 6/6/2018
RIGHT
LEFT
Assessment and Plan BRAO RE –chronic phase with significant inner retinal thinning in macular region. Reduced BCVA 20/25, longstanding and corresponding VF defect today on Baseline 24‐2. Vet has known carotid artery and heart disease and is being monitored closely in the community. Last carotid duplex revealed 50‐79% stenosis R+L and was advised to continue medical management. Reviewed with patient the signs and symptoms of stroke and to go to the emergency room stat if they occur. BRAO LEFT EYE –chronic phase significantly affecting rNFL with correlating visual Assessmentfield loss. and See Plan #1. Fluctuating changes to rNFL OCT LE. Monitor in 6 months. BRAO Right Eye –chronic phase with significant inner retinal thinning in macular region. Reduced BCVA 20/25, longstanding and corresponding VF defect today on baseline 24‐2. Vet has known carotid artery and heart disease Assessment and Plan and is being monitored closely in the community. Last carotid duplex revealed 50‐79% stenosis R+L and was advised BRAO LEFT EYE –chronic phase significantly affecting rNFL with correlating visual field loss. See #1. Fluctuating to continue medical management. Reviewed with patient the signs and symptoms of stroke and to go to the changes to rNFL OCT LE. Monitor in 6 months. emergency room stat if they occur.
43 5 6/6/2018
Retinal Blood Supply • Internal Carotid • Acts as a nonthrombogenic conduit for blood flow to the brain. Carotid Artery Disease • Ophthalmic Artery • Central Retinal Artery
Carotid Artery Disease Carotid Artery Disease • Alteration in permeability • Around 5 % of the general population over 65yo has an if arterial wall endothelium. asymptomatic CAS of 50% or greater. • CAS 7% in women and 9% in men. • Atherosclerosis ‐ buildup of fats, cholesterol and • The prevalence of CAS below the age of 60 is 0.5% and increases to other substances in and on 10% above the age of 80. your artery walls, which can • Native American and Caucasian individuals have the highest restrict blood flow. prevalence of CAS, whereas African American males and Asian • Stenosis females appear to have the lowest prevalence.
Ocular Implications
Risk Factors
44 6 6/6/2018
Amaurosis Fugax Hollenhorst Plaques • 60‐75% of patients with AF have significant carotid stenosis. • Highly refractive plaques in the retinal • The EXPRESS study found that starting proven medications and therapies within arterioles. 24 hours of symptoms of TIA reduces the risk of having stroke within three • Tend to lodge at bifurcations of arterioles months by 80%. where they rarely obstruct the vessel. • Risk of developing stroke after TIAs is 20% within the 1st month. Risk of stroke • Persist longer than platelet‐ fibrin plugs events remains high for 10 to 15 years after TIAs. • In asymptomatic patients, retinal emboli • Only 8.2% Americans know TIA definition, only 8.6% can identify symptom; have poor predictive power for further elderly who are most at risk at least knowledgeable. hemispheric events and are not highly correlated with significant carotid artery disease.
Work Up Carotid Duplex •Blood Work • Conventional ultrasound and Doppler ultrasound. • ESR CRP and platelets to rule out GCA (CRAO) • Identify patients at risk for stroke and document • Fasting blood sugar & HA1C • CBC progressive or recurrent disease in patients already known • Prothrombin time/Activated partial thromboplastin time to be at risk. •Blood Pressure •Carotid Bruit • Duplex evidence of arterial stenosis: •Carotid Artery Duplex • Elevated velocities. •Cardiac Evaluation • Diameter reduction. • ECG • Turbulence
Treatment • In general, medical treatment is provided for asymptomatic patients while invasive treatment is considered for symptomatic patients with CAS greater than 50% and for asymptomatic patients with CAS greater than 60%. Control Risk factors • Antiplatelet • Significantly reduces the incidence of stroke in high risk patients with a resultant 25% reduction in strokes overall. • Statin Medications • Lowers LDL‐ A reduction in cholesterol levels with statins may be associated with plaque regression • Antihypertensives
45 7 6/6/2018
Differentiation of Occult Branch Retinal Artery Occlusion from Glaucoma Clinical findings in occult branch retinal artery occlusion (BRAO) can mimic those of primary open‐angle glaucoma (POAG). Because management of these conditions substantially differs, accurate diagnosis is crucial.
Purpose Defining Occult BRAO • All BRAO subjects were asymptomatic and unaware of any prior retinal artery occlusion episodes. The aim of this study was to identify clinical • Presence of regional inner retinal thinning with lack of inner‐layer stratification on macular SD‐OCT b‐scan images that spatially factors that reliably and efficiently identify corresponded with arteriolar distribution and visual field loss location. occult BRAO masquerading as POAG. • Pattern of inner retinal thinning following course of a specific retinal artery. • Spatial correspondence between location of inner retinal layer thinning and location of repeatable and non progressive visual field loss. • No other retinal or optic nerve disease.
Methods Results • Enrolled patients who were initially suspected of having POAG but were • BRAO eyes exhibited: subsequently diagnosed with occult BRAO when their SD‐OCT macular scans • Shorter axial length demonstrated severe inner retinal layer loss that spatially corresponded with • Smaller C/D ratios arteriolar distribution and visual field loss. • Higher intraeye macular thickness asymmetry • POAG and control (NML) subjects were selected from a longitudinal study at • Higher intereye macular thickness asymmetry our institution and matched to the BRAO group for age and by field loss • Higher frequency of thinner macular thickness blocks of <200 severity. • All subjects had comprehensive eye examinations including SD‐OCT macular • Most diagnostically accurate parameters for differentiating asymmetry and RNFL scans, and systemic health profiles were recorded. Both BRAO from POAG: eyes were studied and asymmetry parameters were calculated. • Intraeye macular thickness asymmetry >25um and 2 macular thickness blocks of <200um.
46 8 6/6/2018
Considerations Results Table here • High individual variability associated with C/D ratio and axial length limits clinical utility. • All occult branch retinal artery occlusion subjects demonstrated intraeye macular thickness asymmetry of greater than 25um AND three or more thin macular thickness blocks, whereas none of the POAG subjects met both criteria. • 100% sensitivity & 100% specificity in this sample.
Conclusion Acknowledgements • Although an argument can be made that occult branch retinal Dr. Judy Oh & Dr. Jeffery Hiett artery occlusion may be a rare condition, the prevalence of this disorder remains unknown and is likely underestimated given its diagnostic challenges. • Not rare in this study: 11/437 2.5% Thank you for the wealth of knowledge you have shared with me this year. I will forever be grateful. • Given the remarkably disparate management approaches for BRAO and POAG and the lack of current knowledge regrading BRAO epidemiology, macular scan acquisition may be important to use as a routine test within baseline glaucoma evaluation.
References References Arruga et al. Ophthalmic Findings in 70 Patients with Evidence of Retinal Embolism. Ophthalmology Hackam, DG. Prognosis of asymptomatic carotid artery occlusion: systematic review and meta‐analysis. 89: 1336‐1347, 1982. Stroke. 2016;47(5):1253–1257 Johnston SC, Fayad PB, Gorelick PB, et al. Prevalence and knowledge of TIA among US Adults. Neurololgy 2003; Bruno et al. Concomitants of asymptomatic retinal cholesterol emboli. Stroke. 1992 June; 23 (6): 900‐ 60: 1429‐1434. 902. Klein R, et al. Retinal emboli and stroke: The Beaver Dam Eye Study. Arch Ophthalmol. 1999: 117: 1063‐1068. Chang et al. RAO and the 3‐year risk of Stroke in Taiwan: A Nationwide Population Based Study. Am J Petty GW, Brow RD, Jr., Whisnant JP, Sicks JD, O’Fallon WM, Wiebers DO. Ischemic stroke subtypes: a population‐ Ophthalmol 2012; 154: 645‐652 based study of incidence and risk factors. Stroke 1999;30:2513–2516. Filho et al. OCT Angiography in RAO. Retina 35: 2339‐2346, 2015. Remington, L. A. (2012). Clinical anatomy and physiology of the visual system. St. Louis: Elsevier/Butterworth‐ Heinemann. Giles MF, Flossman E, Rothwell PM. Patient Behavior Immediately after TIA according to Clinical Rothwell PM, Giles MF, Chandrateva A, Marquardt L, Geraghty O, Redgrave JNE, Lovelock CE, Binney LE, Bull LM, Characteristics, Perception of the Event, and Predicted Risk of Stroke. Stroke 2006; 37: 1254‐1260 Cuthbertson FC, Welch SJV, Bossc S, Carasco‐Alexnder F, Silver LE, Gutnikov SA, Mehta Z. Effect of urgent treatment of transient ischaemic attack and minor stroke on early recurrent stroke (EXPRESS study): a prospective Hayreh et al. Fundus Changes in BRAO. Retina 35: 2060‐2066, 2015. population‐based sequential comparison. Lancet. 2007; 370: 1432–1442. Hayreh et al. Ocular Arterial Occlusive Disorders and Carotid Artery Disease. Ophthalmology Retina Sullivan‐Mee M et al. Differentiating Occult Branch Retinal Artery Occlusion from Primary Open‐angle Glaucoma. 2017; 1: 12‐18. Optom Vis Sci 2018, 95(2): 106‐112 Wong TY, Klein R. Retinal arteriolar emboli: epidemiology and risk of stroke. Curr Opin Oph 2002, 13: 142‐146
47 9 The Chicken or The Egg: Learning Objectives The Evolving Relationship between Optic Nerve Structure and Function in Glaucoma Understand current methods of measuring structural and functional glaucomatous progression Discuss previous landmark glaucoma studies Identify limitations of current imaging & visual field testing measurements Maria Jen, OD VA Portland Health Care System
Financial disclosure Glaucoma
No financial interests or relationships to American Glaucoma Society disclose “Characteristic deterioration of the optic nerve leading to progressive loss of the field of vision” At least 3 million Americans suffer from glaucoma One of the leading causes of adult blindness in the world Leading cause of preventable blindness in the world
What is Glaucoma? History of Structure vs. Function in Glaucoma
First visual field? 1945: Hans Goldmann Late 1960s/early 1970s: Automated perimetry Octopus, Humphrey Visual Field Late 1980s: Structure- From early 1880s until 1970s: “pressure within the function relationship by Quigley et al2 eye higher than the statistical normal of the population” Visual fields & histology 20% RGC 5dB loss of sensitivity
48 What is Glaucoma? History of Structure vs. Function in Glaucoma
1996: Optical Coherence Tomography 2001: OCT3/Stratus TD-OCT 2006: Cirrus HD-OCT, Spectralis SD OCT 2000s: “nowadays generally accepted that POAG is an optic neuropathy characterized by cupping 2012: Swept-source OCT of the optic nerve head with corresponding nerve fiber loss and visual field defects…” 2014: OCT angiography 2008: Structure-function relationship in detecting glaucoma by Bowd et al4
American Glaucoma Society: Categories Which comes first? Structure vs. of Glaucoma Function
Published February 2015 Ocular Hypertension Treatment Study (OHTS) Phase I: At 72 months follow-up5, conversion to glaucoma had been seen In the optic disc first in 55% of eyes In the visual field in 35% of eyes In both simultaneously in 10% of eyes At 8 years follow-up6, among those with both structural and functional progression 41% showed visual field progression first 29% showed optic disc progression first
Which comes first? Structure vs. Glaucomatous Progression Function Increasing functional Structural and Functional Progression in the Early loss in a series of visual Manifest Glaucoma Trial (2016)7: fields OR In eyes with manifest glaucoma, progression in Increasing structural the visual field was detected first more than change in the optic four times as often as progression in the optic nerve head or the disc. retinal nerve fiber Among fellow eyes without visual field loss at layer baseline, progression was detected first as frequently in the optic disc as in the visual Structural change field. functional change?
49 Glaucomatous Progression: OCT or VF? Glaucomatous Progression: OCT or VF?
OCT (RNFL & GCC) VF Early stages: peripapillary retinal nerve fiber (RNFL) Early: GS & PPG 59.8% 27.3% thickness and macular ganglion cell complex (GCC) Late: PG 62.1% 41.8% were together more sensitive than VF in detecting progression After stratifying by severity in perimetric glaucoma eyes, OCT had significantly higher Late stages: RNFL is less sensitive, GCC is equivalent detection rate than VF in MILD PG (63.1% vs. to VF in detecting progression 38.7%).
Role of GCC OCT Role of GCC OCT
Ganglion Cell Complex includes the 3 innermost retinal layers: -RNFL“axons” -GCL“body” - IPL “dendrites”
Ganglion Cell Complex includes the 3 innermost retinal layers: -RNFL“axons” -GCL“body” - IPL “dendrites”
Glaucomatous Progression: OCT or VF? Glaucomatous Progression: OCT or VF?
CASE 1 CASE 2
50 Gold Standard for Tracking Relationship of Structure & Function Progression?
Advantages Disadvantages Visual Field Essential for function Subjective, poor reproducibility Optic nerve photos Useful for structural Subjective, requires high degree changes of clinical expertise, poor agreement between clinicians RNFL OCT Objective, precise Less useful in advanced glaucoma due to “floor effect” of RNFL
Depends highly on the stage of the disease! Early: RNFL OCT + GCC OCT + HVF (24-2) Late: GCC OCT + HVF (10-2?)
Relationship of Structure & Function Relationship of Structure & Function
Examine if the functional correlation between points is related to the relative proximity of the points at the ONH and retinal periphery Spatial filtering to help reduce variability/“noise”? When used on 50 patients, filter helped reduce variability (improved both specificity and sensitivity) WITHOUT reducing detection of loss by total deviation maps
Other Considerations Questions?
Limitations? Photos & OCT: structure only (subjective, inter-clinician variability, “floor effect”) HVF: function only (subjective) Future considerations Role of RNFL OCT? Changes to current VF testing? Possibilities of testing both structure and function simultaneously?
51 References
Thank you! 1. Casson, Robert J., et al. "Definition of glaucoma: clinical and experimental concepts." Clinical & experimental ophthalmology 40.4 (2012): 341-349.
2. Quigley HA, Dunkelberger GR, Green WR. Retinal ganglion cell atrophy correlated with automated perimetry in human eyes with glaucoma. Am J Ophthalmol. 1989;107:453-464.
3. Wolfs, Roger CW, et al. "Changing views on open-angle glaucoma: definitions and prevalences—The Rotterdam Study." Investigative ophthalmology & visual science 41.11 (2000): 3309-3321.
4. Bowd C, Hao J, Tavares IM, et al. Bayesian machine learning classifiers for combining structural and functional measurements to classify healthy and glaucomatous eyes. Invest Ophthalmol Vis Sci. 2008;49:945-953.
5. Kass MA, Heuer DK, Higginbotham EJ, et al. The ocular hypertension treatment study. A randomized trial determines that topical ocular hypotensive medication delays or prevents the onset of primary open-angle glaucoma. Arch Ophthalmol. 2002; 120:701–13.
6. Keltner JL, Johnson CA, Anderson DR, et al. The association between glaucomatous visual fields and optic nerve head features in the ocular hypertension treatment study. Ophthalmol. 2006; 113:1603–12.
7. HannaMaria Ö, Anders H, Lena B, Anderson H, Boel B. Structural and functional progression in the Early Manifest Glaucoma Trial. Ophthalmology. 2016;123(6):1173-1180.
8. Jia, Yali, et al. "Optical coherence tomography angiography of optic disc perfusion in glaucoma." Ophthalmology 121.7 (2014): 1322-1332.
9. Shin, Hye-Young, et al. "Comparative study of macular ganglion cell–inner plexiform layer and peripapillary retinal nerve fiber layer measurement: structure–function analysis." Investigative ophthalmology & visual science 54.12 (2013): 7344-7353.
10. Garway-Heath DF, Poinoosawmy D, Fitzke FW, Hitchings RA. Mapping the visual field to the optic disc in normal tension glaucoma eyes. Ophthalmology. 2000;107:1809-1815.
11. Asaoka R, Russell RA, Malik R, et al. A novel distribution of visual field test points to improve the correlation between structure-function measurements. Invest Ophthalmol Vis Sci. 2012;53:8396-8404.
12. Strouthidis NG, Vinciotti V, Tucker AJ, et al. Structure and function in glaucoma: the relationship between a functional visual field map and an anatomic retinal map. Invest Ophthalmol Vis Sci. 2006;47:5356-5362.
52 FINANCIAL DISCLOSURE
• No financial interests or relationships to disclose
LEARNING OBJECTIVES
• Distinguish between various OCT scan techniques, and understand their implications and utilization as well as their limitations for the monitoring and detection of glaucoma. • Describe the application of Bruch’s membrane opening measurements.
• Discuss the benefits of accounting for the angle from the fovea to the basement membrane opening when acquiring and analyzing scan data • Recognize the importance of the analysis of the macula in monitoring glaucoma
• Identify which regions of the optic nerve and retinal tissue are most susceptible to damage from glaucoma.
53 DETECTION
OCT PARAMETERS: NORMAL CHANGE PER OCT FLOOR: AVG FLOOR +/- STD DEV YEAR ON AVERAGE
• 38 um (± 4.2 um), Bowd et al cpRNFL • 49.9 um for Spectralis, 70.6 um for RTVue, cpRNFL •- 0.36 um/year Mwanza et al
GC-IPL •38 um (± 3.4 um) GC-IPL •- 0.21 um/year
Bowd, C., Zangwill, L. M., Weinreb, R. N., Medeiros, F. A., & Belghith, A. (2017). Estimating optical Bowd, C., Zangwill, L. M., Weinreb, R. N., Medeiros, F. A., & Belghith, A. (2017). Estimating optical coherence tomography structural measurement floors to improve detection of progression in advanced coherence tomography structural measurement floors to improve detection of progression in advanced glaucoma. American journal of ophthalmology, 175, 37-44. glaucoma. American journal of ophthalmology, 175, 37-44.
PROGRESSION PROGRESSION
Types of Progressive GCIPL VF progression along with GCIPL Evidence supporting use of GCIPL and RNFL for early thinning thinning in 57% detection and monitoring disease progression • Widening of defects 58%, most • GCIPL thinning first: 61% common • Longitudinal Data • Deepening defects 26% • Concomitantly with VF progression: • Integrates macula and peripapillary region • New GCIPL defects 21% 22% • Demonstrates relevance of both regions
54 Hou, H. W., Lin, C., & Leung, C. K. S. (2018). Integrating Macular Ganglion Cell Inner Plexiform Layer and Parapapillary Retinal Nerve Fiber Layer Measurements to Detect Glaucoma Progression. Ophthalmology, 125(6), 822-831.
Hou, H. W., Lin, C., & Leung, C. K. S. (2018). Integrating Macular Ganglion Cell Inner Plexiform Layer and Parapapillary Retinal Hou, H. W., Lin, C., & Leung, C. K. S. (2018). Integrating Macular Ganglion Cell Inner Plexiform Layer and Parapapillary Retinal Nerve Fiber Layer Measurements to Detect Glaucoma Progression. Ophthalmology, 125(6), 822-831. Nerve Fiber Layer Measurements to Detect Glaucoma Progression. Ophthalmology, 125(6), 822-831.
55 For each 1 mm2 increase in the area of progression RNFL = 2.26 fold incr risk of VF progression GCIPL = 4.70 fold incr risk of VF progression Hou, H. W., Lin, C., & Leung, C. K. S. (2018). Integrating Macular Ganglion Cell Inner Plexiform Layer and Parapapillary Retinal Hou, H. W., Lin, C., & Leung, C. K. S. (2018). Integrating Macular Ganglion Cell Inner Plexiform Layer and Parapapillary Retinal Nerve Fiber Layer Measurements to Detect Glaucoma Progression. Ophthalmology, 125(6), 822-831. Nerve Fiber Layer Measurements to Detect Glaucoma Progression. Ophthalmology, 125(6), 822-831.
TRADITIONAL TWO DIMENSIONAL OCT SCANNING
RNFL
Rim Area Macula GCIPL Rim Thickness
Three Dimensional OCT THREE DIMENSIONAL OCT SCANNING: Scanning • Termination of the Bruch’s membrane- BMO : retinal pigment epithelium complex within the ONH Bruch’s membrane opening • The maximum aperture within ONH through which retinal ganglion cell axons can pass
• BMO-MRW: • Minimum distance between the ILM and
Fan, K. C., Tsikata, E., Khoueir, Z., Simavli, H., Guo, R., de Luna, R. A., ... & Chen, T. C. (2017). Enhanced Bruch’s membrane opening BMO, irrespective of the plane diagnostic capability for glaucoma of 3-dimensional versus 2-dimensional neuroretinal rim parameters using minimum rim width spectral domain optical coherence tomography. Journal of glaucoma, 26(5), 450-458.
56 3-D SCANNING: BMO-MRW MINIMUM RIM WIDTH : MRW
Ing, E., Ivers, K. M., Yang, H., Gardiner, S. K., Reynaud, J., Cull, G., ... & Burgoyne, C. F. (2016). Cupping in the Fan, K. C., Tsikata, E., Khoueir, Z., Simavli, H., Guo, R., de Luna, R. A., ... & Chen, T. C. (2017). Enhanced diagnostic monkey optic nerve transection model consists of prelaminar tissue thinning in the absence of posterior laminar capability for glaucoma of 3-dimensional versus 2-dimensional neuroretinal rim parameters using spectral domain deformation. Investigative ophthalmology & visual science, 57(6), 2914-2927. optical coherence tomography. Journal of glaucoma, 26(5), 450-458.
3-D SCANNING: MINIMUM DISTANCE BAND 3-D SCANNING: RIM VOLUME, THICKNESS, AREA
Fan, K. C., Tsikata, E., Khoueir, Z., Simavli, H., Guo, R., de Luna, R. A., ... & Chen, T. C. (2017). Enhanced diagnostic capability for glaucoma of 3-dimensional versus 2-dimensional neuroretinal rim parameters Shieh, E., Lee, R., Que, C., Srinivasan, V., Guo, R., DeLuna, R., ... & De Boer, J. (2016). Diagnostic performance of a novel using spectral domain optical coherence tomography. Journal of glaucoma, 26(5), 450-458. three-dimensional neuroretinal rim parameter for glaucoma using high-density volume scans. American journal of ophthalmology, 169, 168-178.
Stereo Disc Photographs (green) & Projected BMO
Fan, K. C., Tsikata, E., Khoueir, Z., Simavli, H., (red) positions Guo, R., de Luna, R. A., ... & Chen, T. C. (2017). Enhanced diagnostic capability for glaucoma of 3-dimensional versus 2-dimensional neuroretinal rim parameters using spectral domain optical coherence tomography. Journal Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of clinically invisible, but optical of glaucoma, 26(5), 450-458. coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative ophthalmology & visual science, 53(4), 1852-1860.
57 Stereo Disc Photographs (green) & Projected BMO (red) positions
CD
Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of clinically invisible, but optical Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of clinically invisible, but coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative ophthalmology & visual science, 53(4), optical coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative ophthalmology & visual 1852-1860. science, 53(4), 1852-1860.
A
B
Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of clinically Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of clinically invisible, invisible, but optical coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative but optical coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative ophthalmology & ophthalmology & visual science, 53(4), 1852-1860. visual science, 53(4), 1852-1860.
Orientation of the foBMO FOBMO impacts : • Accuracy of sector-based rim measurements of the ONH • Peripapillary RNFL scans • Macular retinal nerve fiber layer thickness
• Average FoBMO is apx - 7 degrees • Range: +2.5 to -17.5 among individuals • Current system of assigning sectors results in artificially high variability among individuals.
58 CONVENTIONAL FOBMO ALIGNED FOVEA
Chauhan, B. C., Danthurebandara, V. M., Sharpe, G. P., Demirel, S., Girkin, C. A., Mardin, C. Y., ... & Burgoyne, C. F. (2015). Bruch's membrane opening minimum rim width and retinal nerve fiber layer thickness in a normal white population: a multicenter study. Ophthalmology, 122(9), Huang, G., Gast, T. J., & Burns, S. A. (2014). In vivo adaptive optics imaging of the temporal raphe and its relationship to 1786-1794. the optic disc and fovea in the human retina. Investigative ophthalmology & visual science, 55(9), 5952-5961.
Huang, G., Gast, T. J., & Burns, S. A. (2014). In vivo adaptive optics imaging of the temporal raphe and its Huang, G., Gast, T. J., & Burns, S. A. (2014). In vivo adaptive optics imaging of the temporal raphe and its relationship to the optic disc and fovea in the human retina. Investigative ophthalmology & visual relationship to the optic disc and fovea in the human retina. Investigative ophthalmology & visual science, 55(9), 5952-5961. science, 55(9), 5952-5961.
A DEEPER ANATOMICAL UNDERSTANDING OF WHERE RETINAL GANGLION CELLS EXTEND FROM THE NERVE
INFERIOR SUPERIOR
Hood, D. C. (2017). Improving our understanding, and detection, of glaucomatous damage: an approach based upon optical coherence Jansonius, N. M., Schiefer, J., Nevalainen, J., Paetzold, J., & Schiefer, U. (2012). A mathematical model for describing the retinal nerve fiber bundle trajectories in the human eye: average course, variability, and influence of refraction, optic disc tomography (OCT). Progress in retinal Hood, D. C. (2017). Improving our understanding, and detection, of glaucomatous damage: an approach based upon optical and eye research, 57, 46-75. size and optic disc position. Experimental eye research, 105, 70-78. coherence tomography (OCT). Progress in retinal and eye research, 57, 46-75.
59 FUTURE OCT TECHNOLOGY
• Swept source OCT • future implications as it does not use a standardized ring for RNFL but rather maps the entire posterior pole. • Polarization-sensitive OCT • Visible light OCT
Image: Yang, Z., Tatham, A. J., Zangwill, L. M., Weinreb, R. N., Zhang, C., & Medeiros, F. A. (2015). Diagnostic ability of retinal nerve fiber layer imaging by swept-source optical coherence tomography in glaucoma. American journal of ophthalmology, 159(1), 193-201.
GLAUCOMA MODULE PREMIUM EDITION SPECTRALIS
• https://business- ANATOMICAL lounge.heidelbergengineering.com/us/ POSITIONING SYSTEM en/products/spectralis/glaucoma- – FOBMO ALIGNMENT module/ • BMO - MRW
REFERENCES SUMMARY • Jansonius, N. M., Schiefer, J., Nevalainen, J., Paetzold, J., & Schiefer, U. (2012). A mathematical model for describing the retinal nerve fiber bundle trajectories in the human eye: average course, variability, and influence of refraction, optic disc size and optic disc position. Experimental eye research, 105, 70-78. • Looking at sectors or flagged • Reis, A. S., O'Leary, N., Yang, H., Sharpe, G. P., Nicolela, M. T., Burgoyne, C. F., & Chauhan, B. C. (2012). Influence of quadrants alone on RNFL OCT is not clinically invisible, but optical coherence tomography detected, optic disc margin anatomy on neuroretinal rim evaluation. Investigative ophthalmology & visual science, 53(4), 1852-1860. enough • Fan, K. C., Tsikata, E., Khoueir, Z., Simavli, H., Guo, R., de Luna, R. A., ... & Chen, T. C. (2017). Enhanced diagnostic capability for glaucoma of 3-dimensional versus 2-dimensional neuroretinal rim parameters using spectral domain • More detailed testing and improved optical coherence tomography. Journal of glaucoma, 26(5), 450-458. analysis is necessary in order to better • Shieh, E., Lee, R., Que, C., Srinivasan, V., Guo, R., DeLuna, R., ... & De Boer, J. (2016). Diagnostic performance of a detect early glaucoma and glaucoma novel three-dimensional neuroretinal rim parameter for glaucoma using high-density volume scans. American journal of ophthalmology, 169, 168-178. progression • Reis, A. S., Zangalli, C. E. S., Abe, R. Y., Silva, A. L., Vianna, J. R., Vasconcellos, J. P. C., & Costa, V. P. (2017). Intra‐and interobserver reproducibility of Bruch's membrane opening minimum rim width measurements with spectral domain optical coherence tomography. Acta ophthalmologica, 95(7), e548-e555.
60 REFERENCES CONTINUED
• Zhang, X., Dastiridou, A., Francis, B. A., Tan, O., Varma, R., Greenfield, D. S., ... & Huang, D. (2017). Comparison of THANK YOU, QUESTIONS? Glaucoma Progression Detection by Optical Coherence Tomography and Visual Field. American journal of ophthalmology, 184, 63-74. • Mwanza JC, Durbin MK, Budenz DL, et al. Glaucoma diagnostic accuracy of ganglion cell-inner plexiform layer thickness: comparison with nerve fiber layer and optic nerve head. Ophthalmology 2012;119(6):1151–1158. • Hammel, N., Belghith, A., Weinreb, R. N., Medeiros, F. A., Mendoza, N., & Zangwill, L. M. (2017). Comparing the rates of retinal nerve fiber layer and ganglion cell–inner plexiform layer loss in healthy eyes and in glaucoma eyes. American journal of ophthalmology, 178, 38-50. • Bowd, C., Zangwill, L. M., Weinreb, R. N., Medeiros, F. A., & Belghith, A. (2017). Estimating optical coherence tomography structural measurement floors to improve detection of progression in advanced glaucoma. American journal of ophthalmology, 175, 37-44. • Ing, E., Ivers, K. M., Yang, H., Gardiner, S. K., Reynaud, J., Cull, G., ... & Burgoyne, C. F. (2016). Cupping in the monkey optic nerve transection model consists of prelaminar tissue thinning in the absence of posterior laminar deformation. Investigative ophthalmology & visual science, 57(6), 2914-2927. • Hood, D. C. (2017). Improving our understanding, and detection, of glaucomatous damage: an approach based upon optical coherence tomography (OCT). Progress in retinal and eye research, 57, 46-75.
61 Learning Objectives
• Explain the mechanism of corneal perforation WHAT ANTERIOR CHAMBER!? NON-TRAUMATIC CORNEAL • Differentiate non-traumatic etiologies PERFORATION CASE MANAGEMENT • Recognize signs and symptoms
Megan Mannen, O.D. • Distinguish and document findings Resident Jonathan M. Wainwright Memorial VAMC Pacific Cataract and Laser Institute [email protected] 503-396-6320 • Understand how to effectively manage corneal perforation
• Describe complications in perforation patients
Case Presentation Medical History:
• 51 year old Caucasian female presents to clinic after hours • Current Conditions: • Current Systemic Medications: • Seizure Disorder • Calcium Carbonate • CC: • Degenerative disc disease • Hyudrocodone-acetaminophen • Progressively poor vision in the left eye for the last 2 months • Chronic back pain • Levetiracetam • Patient seen in the ED on July 8th for other medical problems; physician noticed ocular redness and when questioned, patient reported pain OS • Migraines • Lorazepam • Patient was started on diclofenac and neomycin-bacitracin-polymyxin-hydrocortisone 1% OS • Anemia • Magnesium oxide • Patient states the redness and pain improved but her vision began to worsen • Polyethylene glycol • Patient woke up with her left eye stuck shut; she pried the eye open and felt a gush of clear • Sumatriptan fluid • Current Ocular Medications: • Thiamine • The left eye is no longer uncomfortable • Bacitracin-Polymyxin B- • The patient reports had been in the hospital up until presentation neomycin-hydrocortisone ophth • Timolol maleate solution TID OS • Tizanidine • Diclofenac OS
Findings OS: • DVA OS: Hand motion @ 1 foot • Cornea: large perforated corneal ulcer including inferior portion of the visual axis with collapsed chamber and iris prolapse • Conjunctiva: trace temporal limbal bulbar injection • AC: extremely shallow with iris prolapse • Lens: dense white cataract
OD OS
62 Assessment and Plan Review of Corneal Anatomy
Assessment: • Corneal epithelium: 50 um • Large corneal perforationCulture: OS with iris prolapse NEGATIVE • Bowman’s layer: ~10 um • Likely secondary to neurotrophic ulcer • Corneal stroma: 500 um • Other secondary cataractFor any OS microorganism growth • Descemet’s membrane: 5-10 um Plan: Assuming neutrotrophic; however, the patient has been on • Corneal endothelium: 5 um • Requires emergency penetratinga topical keratoplasty antibiotic OS for two months • Patient educated that the goal is to save the left eye • Which layers of the cornea are cellular? • Recommend PK at OHSU as it will be complicated and may require general anesthesia • Epithelium: surface, wing, and basal cells • Stroma: fibroblasts/keratocytes • Emergency PK performed at OHSU on 08/11/17 along with culture • Endothelium: single cell layer advancedvisionsurgery.com/corneal-collagen-crosslinking/
1 Mechanism of Corneal Perforation Non-traumatic Etiologies
Ocular Surface Disease • Dry Eye Syndrome, Neurotrophic Keratitis
Autoimmune Disease • Rheumatoid arthritis: collagen vascular disease characterized by inflammation and collagenase activity Protease production Breakdown of Epithelial lamellae/Stromal Descemetocele Rupture Breakdown (via lacrimal gland, bacteria, tissue lysis Infectious Keratitis WBC) • Bacterial, viral or fungal: Alters epithelial integrity, released pro-inflammatory cytokines from the epithelium and stroma • Most common infectious agent: Staph epidermidis
Medicamentosa • NSAIDS, Steroids, Anesthetics
2,3 Effects of Topical NSAIDS on Perforation Effects of Steroids on Perforation
Prostaglandins 4,5 COX • Delay epithelial and stromal wound healing by decreasing stromal tensile strength PATHWAY • Increased risk of corneal stromal melting and perforation by upregulating collagenases6-10 Lipoxygenase Neutrophil infiltration Collagenase • Increased risk of infection especially herpetic disease due to immunocompromise Pathway and degranulation
EXTREMELY CONTROVERSIAL IN PERFORATION: • Diclofenac inhibits keratocyte proliferation • If you absolutely must use a steroid, combine with a preservative free antibiotic (Levofloxacin, • Possible induction of neurotrophy similar to topical anesthetic abuse Cefuroxime, Ceftazidime) • May increase MMPs which up-regulate stromal degradation and decrease re-epithelialization and tissue remodeling • Contraindications: • MMPs found to be elevated in ulcerated corneas • Exposure keratitis • Neurotrophic keratitis • Caution in those with at risk corneas: • Epithelial defects • Epithelial irregularity post operatively • In conjunction with topical steroids
63 Classic Signs: Classic Symptoms: • Vision Loss •Pain: • Precursors: • Beware: neurotrophic cases may not experience any pain • Progressive corneal thinning • Folds in Descemet’s membrane • Leakage mistaken as excessive tearing • Descemetocele
• Perforation Site • Determine depth and extent of corneal stromal involvement •Location: • Autoimmune disease is usually peripheral: look for overhanging edges • Adjacent AC shallowing • Brown pigment from the iris in the wound, due to the iris moving forward • Aqueous Leakage: Test for Seidel sign • beware of pressure-dependent leaks • Look for infected tissue: necrosis/edema/etc. Descemetocele Sterile corneal ulcer with inferior Marked flattening of the temporal perforation anterior chamber
Management Strategies
Based on cause
Based on size Based on location TREATMENT BASED Based on degree of stromal involvement ON ETIOLOGY Based on age
Based on vision potential
Etiology1,11 Treatment
Autoimmune Disease • Order systemic workup: CBC, RF, ANA, anti-CCP, anti-MCV, ESR, CRP • Rheumatologic referral • Manage systemic condition, often with high dose steroids or immuno-modulators
Infectious Keratitis • Culture (gram stain, calcoflour white, chocolate agar, Sabouraud, dextrose, and thyoglycollate broth) TREATMENT BASED • Treat aggressively with fluoroquinolones or topical fortified antibiotics, antivirals, or antifungals Ocular Surface Disease • Punctal occlusion (plugs or even thermal cautery) ON SIZE • Frequent PF artificial tears • Xiidra or Restasis • Autologous serum drops • BCL or amniotic membrane • Tarsorrhaphy, botox into the levator, or palpebral spring
64 Micro-perforations <2 mm or Impending Perforations12-17 • Amniotic Membrane Transplant:
• May seal spontaneously 1. Increases corneal epithelialization, repair, and remodeling • decreases MMPs and thus inflammation, vascularization and scarring12,13 • Bandage Contact Lens: 2. Promotes healing of persistent corneal defects13 • Kept in place for 4-7 days, closing off the leak to allow epithelialization and scarring 3. Contains multiple growth factors • Can be kept in place longer in conjunction with topical antibiotics and aqueous • fibroblastic growth factor, hepatocyte growth factor, and transforming growth factor β), inhibits suppressants cytokines, inhibits proteases13 • Indications: 4. Can be secured with sutures or fibrin adhesive15 • non-infectious impending or small corneal perforations
• no iris prolapse Sutured amniotic membrane Double amniotic membrane 3 weeks later: AC formed, graft • Pressure patch adhered with Tissucol perforation closed • To protect the globe and contain contents • Drawbacks: Increased risk of secondary infection • Aqueous suppressants (CAI, beta blockers) • Decrease outflow of aqueous from the site of defect and encourage healing in cases where the anterior chamber is formed12
MM1
• Single layer can be used for persistent epithelial defects 11, 17 • Multilayered used for corneal thinning and corneal melt Lamellar Keratoplasty: Epithelium, Bowman’s Membrane, Stroma • AMT has success in 73% of cases for perforations <1.5 mm16
Paracentesis Viscoelastic • Lower rejection rate than traditional KP Advantages • Lower risk of endothelial decompensation • Lower risk of endophthalmitis and secondary glaucoma
• Intralamellar neovascularization Disadvantages • Incomplete removal of pathogens: It is recommended to irrigate the corneal bed with antibacterials/antifungals to decrease the load before suturing the graft Tissucol into AC Placing double AM
• Conjunctival Flap: Small Perforations (2-3 mm) • Cyanoacrylate Glue and Fibrin Tissue Adhesive • Controls pain and promotes healing by allowing fibrovascular tissue to cover area and providing serum growth factors Advantages Disadvantages • Reserved for chronic ulcers that do not heal (i.e. neurotrophic corneals) or descemetoceles • Provides tectonic support arresting stromal lysis • Increased risk for infection10 • Reserved for eyes with poor visual potential • via inhibiting polymorphonuclear lymphocytes and collagenases1,11,19 • The tissue vascularizes limiting acuity • Can temporize the cornea prior to transplant • Can dislodge13 • A thin flap of conjunctiva is pulled over the affected cornea and secured into place using • Provides better outcomes in cases of active sutures inflammation or infection11 • Contraindicated in perforation because leaks can continue below11,18 • Better results prior to PK in perforations secondary to RA10 Insert Photos • Decreased enucleation in perforation • Complications18: • 6% vs. 19% w/ the introduction of adhesives20 • Corneal neovascularization • GPC • elevated IOP/glaucoma • worsening of infectious keratitis
Success rates for perforations <3 mm with glue alone: • ~30%-86% with cyanoacrylate glue19,21 • ~44-79% with fibrin glue22 • Re-application of glue required in ~30%23
65 11,18 Cyanoacrylate Glue versus Fibrin
Cyanoacrylate Glue Fibrin Tissue Adhesive Case Example: Before and After • Bacteriostatic • Biologic • Lasts longer (weeks to months) • Lasts a few days before resorption Day 1 OD • Solidifies quickly • Day 1: Day 1 OS • Easier to apply • Application of • Less discomfort Cyanoacrylate glue • Less neovascularization • BCL • Gatifloxacin 0.3% QID Recommendations for Success • Restasis BID • PF AT q2hours • Use smallest amount possible necessary • Day 2: • Sterile plastic sheet and Day 7 OD Day 7 OS reapplication of glue OS • Check eye for evidence of leakage after • Day 7: application, then place BCL over the top • Sealed perforation and well formed AC OU • Antibiotic QID and protective shield
Large Perforations >3mm Penetrating Keratoplasty Continued
• Rejection rates for PK estimated at 20% • Patch graft • Higher rejection in peripherally placed grafts due to their proximity to limbal blood • For cases that do not fully require keratoplasty vessels and lymphatics. • If the graft edges will likely interfere with vision, keratoplasty is • Exclusively neurotrophic and autoimmune etiologies which persist recommended24 • Better survival if transplantation can be delayed days to weeks11 • Temporary fix for central corneal perforations • If the leak cannot be stopped, the graft should be performed immediately • Permanent fix for peripheral corneal perforations or descemetoceles11,18 • Most common cause of PK failure in RA perforation: recurrent melt at the graft-host • Debridement is recommended prior to placement to remove necrotic tissues junction10 and epithelium
• Penetrating Keratoplasty • Reserved for large corneal perforations (> 3 mm), flat anterior chamber with iris prolapse, or failure of other treatments • In a study by Jonas et al. improvement in post-operative BCVA was attained in 90% of eyes (mean follow-up 14.5 months) 25 • approximately 17% requiring a second transplant for recurrent corneal ulcer
Adjunct Oral and Topical Therapy1,11,24
• Collagenase Inhibitors: inhibit collagenase, MMPs, and oral vitamin C to facilitate collagen synthesis • Topical N-acetylcysteine • Oral Doxycycline: • Clinical trial for chronic corneal wound healing: Dosage: 50 mg bid x 2 weeks, 50 mg qd for 10 weeks • Reminders: Avoid in pregnancy, No one under 8 years old ADJUNCT THERAPY • Adverse effects: Photosensitivity, teeth discoloration, GI upset • Oral Medroxyprogesterone
• Immunosuppressants: control the underlying disease process in inflammatory diseases • Cyclosporine (oral or topical) • systemic methotrexate • cyclophosphamide • rituximab or infliximab
66 Complications of Perforation21 How to Manage Non-Healing Epithelial Defects1,11,24: • Despite therapy, perforation patients are likely to have long term non-healing epithelial defects Persistent epithelial defects (63%)
Ocular hypertension/glaucoma (40.1%) PF Artificial Tears or Autologous Serum
Recurrence of infection (20.4%) Bandage CL Persistent anterior chamber leakage (20.4%) Amniotic Membrane: Prokera or Amnio-dry Choroidal detachment (12.5%) Tarsorrhaphy Endophthalmitis (4.7%) Botox Injection into the Levator Retinal Detachment (3.9%)
Irregular Astigmatism Palpebral Spring
Back to the Case Take Home Points
• Visual Acuity OS: 20/400 PH 20/80-20/200 • Medications throughout treatment: • Size of perforation is a key to proper management • Ocular medications: throughout treatment course • make sure you measure, document, and use these findings to guide your clinical decision making • Treatments: • PA 1% • Emergency PK (August 2017) • Ofloxacin • Failure to diagnose can result in: • Repeat PK with cataract extraction IOL (August • Besivance • permanent corneal damage 2017) • Systemic medications: • loss of AC integrity • Multiple Prokera membranes (August and • 250 mg Cephalexin BID po • endophthalmitis September 2017) • Hydrocodone/APAP PRN for pain • secondary glaucoma (due to formation of peripheral anterior synechiae) • Multiple Tarsorrhaphies (August and September • Acyclovir 400 mg 5x/day for prophylaxis • cataract development 2017) • profound vision loss • AMT (October 2017) • enucleation
• ULTIMATE GOAL: to restore ocular integrity and preserve visual function • Patient passed away on 01/02/2018 from a large stomach ulcer
References 1. Portnoy SL, Insler MS, and HE Kaufman. Surgical management of corneal ulceration and perforation. Survey of Ophthalmology. 1989:34:1 2. Guidera AC, Luchs JI, and IJ Udell. Keratitis, ulceration, and perforation associated with topical nonsteroidal anti-inflammatory drugs. Ophthalmology. 2001; 108: 936-944 3. Hsu JKW, TW Johnston, et. Al. Histopathology of corneal melting associated with diclofenac use after refractive surgery. Journal of Cataract & Refractive Surgery. 2003 29:2;250-256 4. Hersh, PS, Rice BA, Baer JC, et al. Topical nonsteroidal agents and corneal wound healing. Ophthalmology. (1990): 180: 577-583. 5. Petroutsos G, Guimaraes R, Giraud JP, and Y Pouliquen. Corticosteroids and corneal epithelial wound healing. British Jounral of Ophthalmology, 1982, 66, 705-708 6. Lambiase A, Rama P, Aloe L, and S Bonini. Management of neurotrophic keratopathy. Current Opinion in Ophthalmology. 1999, 10:270-276 7. Sacchetti M and A Lambiase. Diagnosis and management of neurotrophic keratitis. Clinical Ophthalmology 2014.8 571-579 8. Semeraro F, Forbice E, Romano V, et. Al. Neurotrophic keratitis. Ophthalmologica 2014;231:191-197 9. Bonini S, Rama, P, Olzi D, and A Lambiase. Neurotrophic keratitis. Eye (2003) 17, 989-995 10. Bernauer W, Ficker LA, Watson PG, Dart JKG. The management of corneal perforations associated with rheumatoid arthritis. Ophthalmology. 1995; 102:1325-1337 11. Jhanji V, Young AL, et. Al. Management of Corneal Perforation. Survey of Ophthalmology. 2011: 56:6; 522-537 12. Kaufman SC. Anterior segment complications of herpes zoster ophthalmicus. Ophthalmology 2008;115:S24-S32 13. Solomon A, Meller, D, Prabhasawat P. et. Al. Amniotic membrane grafts for nontraumatic corneal perforations, descemetoceles, and deep ulcers. Ophthalmology 2002;109:694-703 14. Kitagawa K, Yanagisawa S, Watanabe K, et. Al. A hyperdry amniotic membrane patch using a tissue adhesive for corneal perforations and bleb leaks. American Journal of Ophthalmology. 2009 148:3; 383-389. 15. Hick S, Demers PE, Brunette I, et al. Amniotic membrane transplantation and fibrin glue in the management of corneal ulcers and perforations: a review of 33 cases. Cornea. 2005;24:369-377 16. Rodriguez-Ares MT, Tourino R, Lopez-Valladares MJ, et al. Multilayer amniotic membrane transplantation in the treatment of corneal perforations. Cornea. 2004;23:577-83 QUESTIONS? 17. Krysik K, Dobrowolski D, Lyssek-Boron A, et al. Differences in surgical management of corneal perforations, measured over six years. Journal of Ophthalmology. 2017. 18. Fynn-Thompson N and Goldstein MH. Management of corneal thinning, melting, and perforation. In: Yanoff M, Duker J. Ophthalmology. 4th ed. Saunders. 2014: Ch 2, pp 325-327 19. Setlik, DE, Seldomridge, DL, Adelman, RA, Semchyshyn, TM, and NA Afshari. The effectiveness of isobutyl cyanoacrylate tissue adhesive for the treatment of corneal perforations. American Journal of Ophthalmology (2005). 20. Hirst Fraco, LW, BA, WES and E. De Juan. Tissue adhesive therapy for corneal perforations. Australian Journal of Opthalmology. 1983(11): 113–118. doi:10.1111/j.1442- 9071.1983.tb01060.x 21. Loya-Garcia D, Serna-Ojedo, JC, Pedro-Aguilar L, Jimenez-Corona, A, Olivo-Payne, A, and EO Graue-Hernandez. Non-traumatic corneal perforations: aetiology, treatment, and outcomes. Br J Ophthalmol. Rev Bras Reumatol. 2015;55(4)387-389. 22. Weiss JL, Williams P, Lindstrom RL, Doughman DJ. The use of tissue adhesive in corneal perforations. Ophthalmology. 1983 Jun;90(6):610-5. 23. Sharma A, Kaur R, Kumar R, Kumar S, Gupta P, Pandav S, Patnaik B, Gupta A. Fibrin glue versus N-butyl-2-cyanoacrylate in corneal perforations. Ophthalmology. 2003;110:291-298 24. Chang BH and Groos EB. Neurotrophic Keratitis. In: Cornea: Fundamentals, Diagnosis, and Management. Elsevier. 2017: 1035-1042. 25. Jonas JB, Rank RM, Budde WM. Tectonic sclerokeratoplasty and tectonic penetrating keratoplasty as treatment for perforated or predescemetal corneal ulcers. Am J Ophthalmol. 2001 Jul;132(1):14-8. 26. Nobe JR, Moura BT, Robin JB, et al. Results of therapeutic penetrating keratoplasty. Jpn J Ophthalmol. 2004;48:368-71 27. Dong N, Li C, Chen WS, et al. Fibrin glue-assisted for the treatment of corneal perforations using glycerin-cryopreserved corneal tissue. Int J Ophthalmol 2014;7(1):62-65
67 Learning Objectives The Unusual Suspects: An Overview of Non-Typical Understand Understand the pathophysiology of non-typical intraocular Causes of Acute Intraocular pressure elevation
Pressure Rises Recognize clinical signs and diagnose the etiology of acute Recognize pressure spikes CHRISTINA NGUYEN, O.D. JONATHAN M. WAINWRIGHT VAMC & PACIFIC CATARACT AND LASER INSTITUTE OF KENNEWICK JUNE 8TH, 2018 [email protected] Determine Determine appropriate treatment and management plans
67 YO Caucasian Male
Returning for follow up of acute non- granulomatous anterior uveitis with secondary development of herpes simplex virus OS
Case #1 Initial diagnoses: Acute non-granulomatous CASE #1 anterior uveitis Placed on pred acetate
Developed herpes simplex virus OS discontinued pred acetate
Case #1 Case #1: History
Medical Hx Type 2 Diabetes Hyperlipidemia Medications Hypertension Transient cerebral ischemia Timolol 0.5% BID OS Dorzolamide BID OS Benign Prostate Kidney Cancer Hyperplasia Zirgan 5x/day Valacyclovir 500 mg TID PO
Diltiazem Furosemide Ocular Hx Primary Open Angle Acute non- Potassium Chloride Pravastatin Glaucoma suspect granulotmatous anterior uveitis OS with secondary Sertraline HCL Brimonidine – d/c due to development of anomalous pressure spike herpes simplex virus
68 Case #1: Clinical findings Case #1: Assessment and Plan
Entrance Testing Anterior segment Assessment Acute non-granulomatous anterior uveitis secondary to herpes simplex virus with ocular Test OD OS Test OS hypertension OS Visual Acuity (CC) 20/20 20/30 PH NI Cornea Epithelium: Resolved dendrites with 3+ diffuse PEEs, epi defect temporally with corneal thinning near the Pupils ERRL, (-)APD ERRL, (-)APD limbus Stroma: steamy haze Endothelium: few small KPs > inferiorly Plan Confrontation VF FTFC FTFC Educated patient. Unable to control pressure in office. Latanoprost contraindicated by EOM Full Full Conjunctiva 1+ diffuse injection > inferiorly herpes simplex and brimonidine contraindicated due to history of anomalous pressure Anterior Chamber (-)cells/flare appreciated though likely present spike. Continue Valacyclovir 500mg TID PO, and Zirgan 5x/day OS. Continue Timolol BID Intraocular pressures (OD/OS): Iris Normal, (-)NVI, (-)synechiae and Dorzolamide BID OS and acetazolamide 250 mg BID PO until further notice. Referral 16/52 mmHg to glaucoma specialist for consult. 250 mg Acetazolamide given orally 16/42 mmHg 1 gtt Cosopt 47 mmHg OS
Case #1 Conclusion
Patient continued REVIEW follow ups for next 3 No surgical intervention weeks with very slow was recommended by tapering of Pred Glaucoma specialist Acetate from q1hr to TID
Primary vs Anatomy of Secondary causes of acute IOP rise the Angle
Primary Cause Elevated IOP is commonly attributed to an increase Acute Angle Closure in resistance to outflow but other factors may be involved Secondary Cause Aqueous humor secreted into posterior chamber Inflammation/Uveitis anterior chamber drains into the angle via two Posner Schlossman routes Steroid induced
Post Operative
69 Anatomy of Trabecular the Angle Meshwork
Trabecular Outflow Trabecular meshwork is a triangular shaped tissue Trabecular meshwork schelmm’s canal that is divided into the collector channels uveal and corneoscleral episcleral veins meshworks with the insert region being the Uveoscleral Outflow juxtacanalicular tissue Starts with the ciliary (JCT) muscle flows in many directions including: The meshworks are across the sclera, within comprised of the supraciliary/ fenestrated beams of suprachoroidal spaces, lamellae with large emissarial canals, into intertrabecular spaces uveal vessels, and between adjacent vortex veins sheets
Trabecular CAN BE CAUSED BY: Inflammatory debris such as White blood cell proteins, fibrin or normal Meshwork accumulation serum components following (macrophages and blood-aqueous barrier activated T-lymphocytes) breakdown increases aqueous viscosity JCT is composed of loosely arranged extracellular Trabecular matrix (ECM) ECM is highly dynamic Meshwork Overwhelming the and composed of Swelling of trabecular trabecular endothelial many bioactive Obstruction lamellae and endothelial phagocytic and clearing molecules that cells processes by a severe influence outflow inflammation resistance TM cells within the JCT region sense IOP changes as a stretch/distortion and respond by activating and Irreversible damage or loss releasing MMPs to focally to trabecular endothelial degrade the ECM to allow cells leading to permanent greater outflow reduction in outflow
Inflammation, Uveitis and Non-typical Etiologies of Effects on Intraocular Pressure Spikes Pressure Rise
70 Inflammation and Uveitis Inflammation and Uveitis
•IOP often drops in acute uveitis due to reduced Role of inflammatory aqueous production when ciliary body is mediators inflamed and increased uveloscleral outflow due to prostaglandin release •Posterior synechia can be extensive and cause angle closure •IOP increase often occurs following an episode of uveitis increased trabecular resistance •Scarring of the TM can also result from following TM hypoperfusion in severe iritis chronic inflammation and cause persistent increase in IOP Certain cytokines are •Pressure elevation results from TM compromised IL-1 activity in TM has been chemoattractant to trabeculocytes Cytokines (IL-1 and TNF-a) are implicated in IOP elevation in POAG and possible that damaged TM outflow due exposure from filtered inflammatory expressed in ocular tissues in structure may occur from chronic cells, cytokines, and iris pigment experimental uveitis Thought to be a result of IOP-related cytokine exposure stress to the trabeculocytes Loss of JCT TM cells outflow obstruction
Etiologies include: Uveitis, trauma or intraocular surgery
Inflammation and Uveitis
Clinical Presentation Treatment and Management Signs Elevated IOP Management is directed towards controlling Posner-Schlossman Unilateral Significant cells and flare inflammation Peripheral anterior synechiae/posterior Topical steroids – Dosing depends on severity of synechiae uveitis Syndrome Keratic precipitates Mydriatics/cycloplegics Conjunctival injection Ciliary flush Initiate IOP lowering medications Increased TM pigmentation Corneal edema Consider glaucoma filtering surgery if IOP remains Symptoms Pain elevated after maximal medical therapy Photophobia Decrease vision Halos around lights
Posner-Schlossman Posner-Schlossman Syndrome Syndrome Clinical Presentation Treatment and Management Signs Typically unilateral • Aggressive IOP control and long term monitoring is strongly Also known as Glaucomatocyclitic Crisis Mild ciliary flush recommended Corneal epithelial edema • Aim to reduce inflammation and control IOP Characterized by unilateral recurrent attacks Scant keratic precipitates • Combined regimen of topical steroids and topical and Mild iridocyclitis with IOP range of 40-60 mmHg systemic antiglaucoma drugs is usually successful to of mild nongranulomatous anterior uveitis with Lacks posterior synechiae or peripheral anterior control condition elevations of IOP synechiae • Topical NSAIDs can be used to reduce inflammation Open angle on gonioscopy • Surgical treatment can be considered in cases with Typically affects individuals between 30-60 Between attacks, AC and IOP will return to progressive cupping and visual field for IOP management years old normal Symptoms Slight discomfort Inflammation of trabecular meshwork; Slight blurring of vision or halo IOP pressure typically out of proportion of the underlying cause unknown – possibly degree of pain and AC inflammation associated with some infection
Trabeculitis
71 Steroid Induced
Degree of IOP elevation depends on potency and dosing of medication used Approximately 33-40% of general population Steroid Induced are steroid responders Pressure spikes have been observed in patients with glaucoma or glaucoma suspects in higher frequency Steroids affect the trabecular meshwork by inducing microstructural changes
Steroid Induced Steroid Induced
Thickened trabecular Clinical Presentation Treatment and Management beams Proliferation Phagocytosis Inhibited inhibited • Elevation in IOP typically seen after 2 weeks of • Damage to aqueous outflow is typically reversible initiation with discontinuation of steroid therapy Decreased Thickened • Clinical presentation is similar to primary open intertrabecular juxtacanalicular • Similar to treatment of Primary Open Angle spaces tissues angle glaucoma Glaucoma • Affected individuals are unaware that they have • Prevention and early detection are key ocular hypertension because of painless IOP • Baseline IOP measurement should be performed increased Deposition of elevation Cell shape extracellular prior to start of steroid therapy accumulation • Causes very similar irreversible optic nerve head • IOP should be rechecked every 2 weeks for the first and and size matrix cupping and visual field loss month, monthly for 2-3 months and then every 3-6 decreased decreased material clearance of months for long term steroid therapy debris increased
Brimonidine Associated
Common ocular side effects of brimonidine 0.2% include hyperemia, burning and conjunctival follicles Anterior uveitis has been reported as a late ocular side effect in several cases Theorized that patients who are predisposed to uveitis develop Brimonidine Associated this side effect Although mechanism is still unclear, it is thought to be immune mediated rather than direct toxicity due to timeline of symptom onset Several case reports suggests that brimonidine causes anterior granulomatous uveitis typically after 12 months of use Rare due to the fact that most therapies are discontinued in eyes with allergic reactions predisposes patients to development of uveitis
72 Brimonidine Associated
Clinical Presentation Treatment and Management Signs Typically presents in eye treated • Signs and symptoms respond rapidly to cessation of Mutton fat keratic percipitates brimonindine Redness • Course of steroids should be administered and Anterior chamber cells and flare patients should be followed closely for resolution Post-Operative Corneal edema Increased IOP
Symptoms Pain Photophobia Blurred vision
Post-operative Pressure Post-operative Pressure Elevation: Elevation: Cataract Surgery Cataract Surgery
Elevation of IOP following cataract surgery can occur as early as 2 hours after surgery and typically lasts about 24 hours Clinical Presentation Treatment and Management
IOP spike is attributed to Signs Increased IOP • Depending on how significant IOP elevation is, use Decreased Visual Acuities topical beta-blockers, CAIs, alpha-agonists, oral Post-op inflammation and disruption of the blood-aqueous Corneal edema acetazolamide as necessary barrier Inflammation Deformation of outflow channels • If IOP is approaching 40mmHg or greater, consider “burping” the paracentesis port Release of prostaglandins Symptoms Pain Blockage of TM by inflammatory cells, red blood cells, Nausea pigmented iris debris, retained viscoelastic Vomiting
Patients with preexisting glaucoma and ocular hypertension are commonly at risk for post-op IOP elevation
Post-operative Pressure Post-operative Pressure Elevation: Elevation: Aqueous Aqueous Misdirection Misdirection
A form of secondary glaucoma related to pressure build Clinical Presentation Treatment and Management up from trapping of aqueous within the vitreous Signs Diffuse shallowing of the anterior chamber • First line therapy is cycloplegics to tighten the lens Typically occurs after correctional surgery for angle Anterior displacement of the lens-iris diaphragm zonules and pull the displaced lens posteriorly closure glaucoma Posterior segment free of • Initiation of pressure lowering medications hemorrhage/masses/vein occlusion May occurs days to months postoperatively • Up to 50% will resolve after 5 days of treatment Patent iridotomy/iridectomy taper medications Aqueous passes posteriorly to the posterior chamber No pupillary block instead of anteriorly due to obstruction to flow caused by • Laser Therapy: YAG Capsulotomy or pars plana anterior rotation of ciliary processes Accumulation of vitrectomy removal of anterior hyaloid is most aqueous in the posterior segment causes an anterior Symptoms Pain critical to prevent recurrence displacement of the lens-iris diaphragm Blurry vision/haloes Headache/brow ache Nausea or vomiting
73 74 YO Caucasian Female
CC:
Intermittent reduced VA and superficial discomfort followed with deeper discomfort and further reduction in VA over the last few weeks. Patient attributes this to changing to Simbrinza. H/o Case #2 Cataract extraction was complicated by capsule CASE #2 tear, requiring ACIOL. Patient presented to referring OD with pressure of 74 mmHg OS, after instillation of: 1gtt latanoprost, 1gtt Alphagan, 1 gtt of Timolol and 500 mg Acetazolamide PO @4:20pm, patient left referring OD office with IOP of 69 mmHg.
Case #2: History Case #2
Medical Hx Hypertension Asthma Medications Hyperlipidemia Hearing loss Albuterol Aspirin Thyroid disease Depression
Atorvastatin Liothyronine Family Hx Cardiovascular Diabetes – Mother Losartan Paxil disease –father
Glaucoma - mother Pulmicort Flexhaler Latanprost QHS OU
Ocular Hx Simbrinza BID OS Cataract Extraction w/ Glaucoma OU capsule tear OS
Case #2: Clinical findings Case #2: Assessment and Plan
ASSESSMENT
Entrance Testing Anterior segment 1. Glaucoma secondary to severe stage inflammation, mechanical in nature OS Test OD OS Test OS 2. Other mechanical complication of intraocular lens OS Visual Acuity (CC) 20/20 20/60 PH 20/40 Anterior ACIOL, trace white cell, mild flare, clear 3. Iridocyclitis OS Chamber space between AC IOL and pupil space – Pupils RRL, (-)APD Horizontally peaked, no pupil block, obvious chafing and 4. Presence of intraocular lens; ACIOL at root of problem but reactive, (-)APD atrophy at 3 & 9 where haptics touch, Confrontation VF FTFC Difficult but appears peaked at 3 & 9, looks like pt may have PLAN full had superior PI but that appears fibrosed and sealed 1-3. Continue with Acetazolamide 500 mg BID, add pred forte q2h. Continue all EOM Full Full glaucoma meds and add Timolol QD Intraocular pressures 69 mmHg OS Cornea fine/small granulomatous KPs 1-2+, no edema 4. IOL exchange at some point, but will focus on acute IOP spike at this time Posterior pole: OD 0.75V/0.70, OS 0.85/0.85
74 Case #2: Follow Up
CC: Patient reports vision is improved from last Entrance Testing Anterior segment night and comfort improved OS from last night. Test OD OS Test OS Good compliance with gtts and pills. OD doing well, no concerns. Visual Acuity 20/25+2 20/20-2 Anterior deep and quiet, no cells visible; Case #2: (CC) Chamber ACIOL atrophy from chafing at 3 Current medications: Pupils RRL, (-)APD Horizontally & 9, no PI above 500 mg Acetazolamide x2 this morning peaked, but 1 Day follow reactive, (-)APD Pred forte Q2hrs OS (LD: 7AM) Confrontation FTFC FTFC Cornea More KP present today, still no up Istalol BID OS (LD 10PM) VF edema EOM Full Full Latanoprost QHS OU (LD 10:30AM)
Simbrinza BID OS (LD 7AM) Intraocular pressures 17 mmHg OS Posterior pole: OD 0.75V/0.70, OS 0.85/0.85
Assessment and Plan, Visit #2 Take Home Points
ASSESSMENT Conduct a careful evaluation to determine the etiology of the 1. Glaucoma secondary to severe stage inflammation, mechanical chaffing OS pressure elevation 2. Mechanical complication of intraocular lens OS In cases of steroid induced pressure spikes Consider what is 3. Primary open angle glaucoma OS, severe stage dictating the steroid dosage and if discontinuing the steroid is truly 4. Uveitis – IOP spike has an inflammatory component OS needed 5. Presence of intraocular lens Key is to lower the IOP and keep it lowered during the duration of IOP spike PLAN 1-5. Discussed findings with patient. Consult with cataract surgeon: recommended replacing Consider switching to ester-based steroids such as loteprednol lens after pressure is under control; possibility of glaucoma surgery in the future. Appropriate monitoring and follow up timelines should be Continue pred forte q2h OS. Istalol BID OS, latanoprost QHS OU. Simbrinza BID OS. RTC in 4 established to insure timely and effective resolution days.
References
1. Byles, Daniel B, et al. “Anterior uveitis as a side effect of topical brimonidine.” American Journal of Ophthalmology, vol. 130, no. 3, 2000, pp. 287–291., doi:10.1016/s0002-9394(00)00491-8.
2. Clark, Abbot F. , et al. “Chapter 19 - Steroid-Induced glaucoma.” Math Calculations for Pharmacy Technicians, Second ed., Elsevier, 2013, pp. 146–152.
3. Gery, Igal, and Chi-Chao Chan. “Mechanisms of Uveitis.” Ophthalmology, Fourth ed., Elsevier, 2014, pp. 690–693.
4. Lim, Ridia, and Ivan Goldberg. “10.16 - Inflammatory and Corticosteroid-Induced Glaucoma.” Ophthalmology, Fourth ed., Elsevier, 2014, pp. 1080–1083.
5. Kulkarni, Avinash, et al. “36 - Uveitic Glaucoma.” Glaucoma, Second ed., Elsevier, 2015, pp. 410–424.
6. Nazm, Nazneen, et al. “Outcome of Raised Intraocular Pressure in Uveitic Eyes With and Without a Corticosteroid-Induced Hypertensive Response.” American Journal of Ophthalmology, vol. Thank you! 149, no. 3, 2010, pp. 525–526., doi:10.1016/j.ajo.2009.10.013. 7. Piltz-Seymour, Jody, and Tak Yee Tania Tai. “Other Secondary Glaucomas.” Glaucoma, 2015, pp. 434–445., doi:10.1016/b978-0-7020-5193-7.00038-8.
8. Rojanapongpun, Prin, and Orathai Suwanpimolkul. “Acute Intraocular Pressure Rise.” Glaucoma, 2nd ed., Elsevier Limited, 2015, pp. 598–608. QUESTIONS? 9. Saeedi, Osamah Jawaid, et al. “Epidemiology of Glaucoma.” Ophthalmology, Fourth ed., Elsevier, 2014, pp. 1001–1006. 10. Sung, Velota C.t., and Keith Barton. “Management of inflammatory glaucomas.” Current Opinion in Ophthalmology, vol. 15, no. 2, 2004, pp. 136–140., doi:10.1097/00055735-200404000-00014.
11. Toris, Carol B. “Aqueous Humor Dynamics and Intraocular Pressure Elevation.” Glaucoma, 2015, pp. 47–56., doi:10.1016/b978-0-7020-5193-7.00006-6.
12. Keller KE, Acott TS. The Juxtacanalicular Region of Ocular Trabecular Meshwork: A Tissue with a Unique Extracellular Matrix and Specialized Function. J Ocul Biol. 2013;1(1):3.
13. Abu-Hassan DW, Acott TS, Kelley MJ. The Trabecular Meshwork: A Basic Review of Form and Function. J Ocul Biol. 2014;2(1). pmid:25356439; PubMed Central PMCID: PMCPMC4209746.
14. Rhee DJ, Deramo VA, Connolly BP, Blecher MH. Intraocular pressure trends after supranormal pressurization to aid closure of sutureless cataract wounds. J Cataract Refract Surg. 1999 Apr;25(4):546-9.
15. Tripathi RC, Parapuram SK, Tripathi BJ, et al. Corticosteroids and glaucoma risk. Drugs Aging. 1999;15(6):439-50.
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19. Greaves MW. Anti-inflammatory action of corticosteroids. Post Graduate Medical Journal. 1976;52:63.
75 Sara George, OD Perioptic Neuritis: A Case Study
76 77 78 79 80 81 COURSE DESCRIPTION
● This presentation explores differential diagnosis of papilledema using fundus examination and popular technologies as seen in a case report of a 15 year-old female with exotropia of questionable onset with accompanying neurological symptoms.
PAPILLEDEMA VS Kelsey Manescalco, O.D. PSEUDOPAPILLEDEMA IN NEW ONSET EXOTROPIA
LEARNING OBJECTIVES PRACTICE GAP
● By then end of the presentation attendees will be able to: ● Technology is ever changing in the practice of optometry. In order to provide standard of care Clinically differentiate papilledema from pseudopapilledema using fundus examination and optical for our patients we must adapt to the technology available to us in order to take advantage coherence tomography of their diagnostic techniques. However, the learning curve can be difficult as many of these Differentiate long standing from aquired exotropia Identify and rule out pathologic causes of exotropia complex technologies can emerge post-graduation for a practitioner. ● Optometric Education can allot more time to new technologies beneficial to patient care so that practitioners/optometrists not only know how to use the equipment, but fully understand the capabilities of the instrument.
CASE STUDY -- HISTORY CASE STUDY -- PRE-TESTING
● LD 15 YO Asian American Female ● Confrontation VF ● CC: OD: FTFC Neurological symptoms beginning 1-2 years ago OS: FTFC Balance issues ● Pupils: Daily HA Dizziness while moving, PERRL-APD Light sensitivity ● IOP Secondary complaints of trouble with school work and reading OD: 18 mmHg ● No pertinent personal medical hx or family medical hx OS: 20 mmHg ● No pertinent personal ocular hx or ocular hx ● EOMs FROM LEE: 1 year ago ● Stereo ● Medications Randot 5/10 Oral Contraceptive Random Dot 250 Sec Arc Aleve prn
82 CASE STUDY -- BINOCULAR TESTING EXOTROPIA
● Cover Test ● Vergences ● Classifications of Strabismus Distance - Ortho BI 18/16 Age of Onset Near -- 25pd RXT BO: suppression Constancy ● NPC ● Worth 4 Dot Laterality OD out 10 cm Distance – Alternating OS Suppression Directionality ● Pursuits: Smooth, but symptoms of Near- 4 Dots Magnitude dizziness ● Maddox Rod Testing -- UTT Comitancy ● Saccades: Smooth suppression Organicity ● MEM: +0.50
EXOTROPIA -- CAUSES OF ACQUIRED ONSET CASE STUDY -- VISUAL TESTING
● Duane Syndrome Type 2 ● Habitual Rx: ● Neuromuscular Issues -1.50 -0.75 x 175 1.0 BU 20/20- Palsy -1.75 -1.00 x 003 1.0 BU 20/25 Myasthenia Gravis ● Retinoscopy INO OD: -1.50 -0.50x 180 Thyroid Disease OS: -2.25 -0.75 x 180 ● Orbital Disease ● Final Refraction OD: -1.75 -0.75 x175 20/20 OS: -2.50 -075 x180 20/20 ● Accommodation NRA +3.00 PRA -3.00
CASE STUDY -- OCULAR HEALTH PAPILLEDMA VS PSEUDOPAPILLEDMA
● Anterior Segment: ● Bilateral optic disc swelling Normal edema, elevation, blurred vessels, and blurred margins from increased intracranial pressure ● Posterior Pole: Pseudopapilledema C/D: 0.4 OU Vessels not obscured Elevated temporally OU, distinct margins and vessels ONH Drusen ● Symptoms Headache Transient vision loss Double Nausea ● Can see hemorrhages or loss of SVP ● Various Etiologies
https://jamanetwork.com/journals/jamaophthalmology/fullarticle/424713
83 PAPILLEDEMA VS PSEUDOPAPILLEDEMA OCT
CASE STUDY -- ASSESSMENT AND PLAN PATIENT PROGRESS
● H52.13 - Myopia ● 12 Therapy Sessions (Estimated 6-8 months of VT) ● H52.223 - Astigmatism Patient still symptomatic but has noticed improvement in frequency and severity ● H47.393 - Other disorders of Optic Disc 20 pd Intermittent RXT at near (<25%) No longer suppressing BO, W4D, or NPC ● H50.111 - Monocular Exotropia Randot improved to 8/10 ● H53.34 -- Suppression CPM +/- 2.00 w/ suppression checks = 14
84 TAKE AWAY REFERENCES
● Important to differentiate between strabismus and pathology Bassi, Shikha, and Kuppuswamy Mohana. "Optical Coherence Tomography in Papilledema and Pseudopapilledema with and without Optic Nerve Head Drusen." Indian Journal of Ophthalmology, vol. 62, no. 12, 2014, pp. 1146-1151, ProQuest, http://proxy.lib.pacificu.edu:2048/login?url=https://search-proquest- Case history com.proxy.lib.pacificu.edu:2443/docview/1649167228?accountid=13047, doi:http://dx.doi.org.proxy.lib.pacificu.edu:2048/10.4103/0301- 4738.149136 Bagheri, Nika, et al. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Wolters Kluwer Health/Lippincott Sensorimotor Evaluation Williams & Wilkins, 2017. Fard, Masoud A., et al. "Quantification of Peripapillary Total Retinal Volume in Pseudopapilledema and Mild Papilledema using Spectral-Domain Optical Coherence Tomography." American Journal of Ophthalmology, vol. 158, no. 1, 2014, pp. 136-43, ProQuest, Dilated Fundus Examination http://proxy.lib.pacificu.edu:2048/login?url=https://search-proquest- com.proxy.lib.pacificu.edu:2443/docview/1535066476?accountid=13047, Diagnostic Equipment doi:http://dx.doi.org.proxy.lib.pacificu.edu:2048/10.1016/j.ajo.2014.03.008. Grosvenor, Theodore P. Primary Care Optometry. Butterworth-Heinemann/Elsevier, 2007. ● Rule out any underlying treatments before initiating VT How, FRCS Alicia C. S. “Population Prevalence of Tilted and Torted Optic Discs Among an Adult Chinese Population in SingaporeThe Tanjong Pagar Study.” Archives of Ophthalmology, American Medical Association, 13 July 2009, jamanetwork.com/journals/jamaophthalmology/fullarticle/423628. Martinez, Michael R., M.D., and Avinoam Ophir M.D. "Optical Coherence Tomography as an Adjunctive Tool for Diagnosing Papilledema in Young Patients." Journal of Pediatric Ophthalmology and Strabismus, vol. 48, no. 3, 2011, pp. 174-181, ProQuest, http://proxy.lib.pacificu.edu:2048/login?url=https://search-proquest-com.proxy.lib.pacificu.edu:2443/docview/867784860?accountid=13047, doi:http://dx.doi.org.proxy.lib.pacificu.edu:2048/10.3928/01913913-20100719-05. Press, Leonard J. Applied Concepts in VisioÌn Therapy. Optometric Extension Program Foundation, 2008. Shin, Hye-Young, Hae-Young L. Park, and Chan K. Park. "The Effect of Myopic Optic Disc Tilt on Measurement of Spectral-Domain Optical Coherence Tomography Parameters." British Journal of Ophthalmology, vol. 99, no. 1, 2015, pp. 69, ProQuest, http://proxy.lib.pacificu.edu:2048/login?url=https://search-proquest- com.proxy.lib.pacificu.edu:2443/docview/1778959685?accountid=13047, doi:http://dx.doi.org.proxy.lib.pacificu.edu:2048/10.1136/bjophthalmol-2014-305259.
85 Mark your calendar – UPCOMING CE EVENTS
Northwest Residents Conference Pacific University Campus June 8-9, 2018 11.5 hours of continuing education with clinical case presentations from 2017-2018 Optometric Residents $100
2018 Victoria Conference Hotel Grand Pacific July 12 – 15, 2018 Up to 20 hours of Continuing Education featuring: Lee Carr, Anthony DeWilde, Beth Kinoshita, James Kundart and Cathy Evans $575
2019 Island Eyes Conference January 20 – 26, 2019 Fairmont Orchid, Kohala Coast, Hawaii This winter classic offers up to 30 hours of CE with a family-friendly schedule of lectures in the mornings and afternoons and evenings free. Invited speakers include: Steve Laukaitis, MD; Nate Lighthizer, OD; Brian Mathie, OD; Stuart Richer, OD, PhD; Stan Teplick, MD; and Maria Walker, OD, MS. Early registration: $700
No time to get away? Explore our online CE options: https://online-ce.opt.pacificu.edu/
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