CONTINUING EDUCATION CERTIFICATION Tulalip Continuing Education
September 14, 2014 Tulalip Resort & Casino COPE EVENT #107876
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L Ryan Bulson, OD, MS Diabetic Retinopathy: An Evidence Based Approach (1 hr)
42551 SD
VERIFICATION STAMP
Dina Erickson, OD AMD Updates (2 Hrs)
42624 PS
VERIFICATION STAMP
Ryan Bulson, OD, MS The Effects of Smoking and the Eye (1 hr)
41520 PB
VERIFICATION STAMP
Ryan Bulson & Dina Erickson Grand Rounds (2 Hrs)
42625 PS
VERIFICATION STAMP
Therapeutic Hours: PB, SD, PS TOTAL HOURS OFFERED: 6 TOTAL HOURS ATTENDED:
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College of Optometry 2043 College Way Forest Grove, Oregon 97116 503-352-2202
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TULALIP CE FACULTY Ryan Bulson, OD, MS Dr. Bulson is a native of Barneveld, New York and he completed his OD and MS at State University of New York. His residency training in Hospital Based Primary Care Optometry at the Portland VA Medical Center brought him to the Pacific Northwest. He is an Assistant Professor at Pacific University where he teaches clinical procedures and is an attending optometric physician for 4th year optometry students.
Dina Erickson, OD Dr. Erickson has served as an adjunct faculty member since 1998 and assistant professor since 2004. Dr. Erickson is co-instructor in the Ocular Disease and Clinical Procedures courses and also advises third- and fourth-year students in the Pacific University EyeClinics. In addition to her teaching responsibilities at the college, Dr. Erickson is in an optometric physician in a primary care private practice and is an active member of the Portland Metro Optometric Society.
She earned her O.D. from Southern California College of Optometry and completed a residency in Hospital Based Optometry at the San Francisco VA Medical Center.
October 2 – 4, 2014 7th Annual Research Conference, Pacific University - 12 HOURS of Continuing Education January 10, 2015 Glaucoma Symposium, Willows Lodge, Woodinville, Washington 7 HOURS of Continuing Education. Information: [email protected] January 25 – 31, 2015 2015 Island Eyes Conference, Hilton Waikoloa Village, Hawaii Up to 29 HOURS of OD Continuing Education and 9 HOURS of Paraoptometric Education April 24 & 25, 2015 Coeur d’Alene CE, Coeur d’Alene Resort, Idaho - 10 HOURS of Continuing Education July 16 – 19, 2015 2015 Victoria Conference, Inn at Laurel Point, Victoria, BC, Canada 20 HOURS of Continuing Education and 4 HOURS of Paraoptometric Education
Can’t get away? Try our ONLINE CONTINUING EDUCATION http://www.pacificu.edu/current-graduate-professional/academics/areas-study/optometry
Our new mobile app will be introduced soon
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8/25/2014
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Diabetic Retinopathy: An Evidence Based
Approach Ryan Bulson, O.D., M.S., F.A.A.O.
Overview
• Review the impact of diabetes on the healthcare system
• Review the most common forms of diabetes and their risk factors
• Review standards for grading diabetic retinopathy
• Review newly revised (February 2014) AOA Clinical Practice Guidelines for managing diabetic retinopathy
The Problem
• Diabetes mellitus is a group of chronic metabolic diseases characterized
• Hyperglycemia
• Defects of insulin secretion
• Increased cellular resistance to insulin
• Microvascular and macrovascular disease
• Long term complications including diabetic nephropathy, neuropathy, and retinopathy
The Problem
• An estimated 25.8 million Americans (~8.3 percent of the population) have diabetes
• ~2 million adults newly diagnosed in 2010
• As many as 40 percent of people with diabetes don’t know they have the disease.
• If trends continue: one in three adults in the US will have diabetes by 2050
• Seventh leading cause of death in the United States
• Direct and indirect costs: $245 billion annually
• The number of people with diabetes worldwide increased from 153 million in 1980 to 347 million in 2008. This number is expected to grow to 429 million by 2030.
Diabetes Classification
Pre-Diabetes
• Blood glucose is higher than normal, but not high enough for diabetes
• Impaired glucose tolerance • On 75-g oral glucose tolerance test, the 2-hour plasma glucose
value is 140-199 mg/dl
• Impaired fasting glucose • Fasting glucose 100-125mg/dl
• Glycosolated Hemoglobin (A1C) • Measuring percentage of blood glucose attached to hemoglobin
• Average blood glucose level for the previous 2-3 months
• 5.7-6.4% is considered pre-diabetes
•
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Diabetes Classification
Diabetes
• HbA1C ≥ 6.5%
• Random (without regard to last meal) plasma glucose level ≥ 200 mg/dl in a person with classic symptoms of hyperglycemia (polyuria, polydipsia, and weight loss) or hyperglycemic crisis
• Fasting (no caloric intake for at least 8 hours) plasma glucose level ≥ 126 mg/dl
• Two-hour plasma glucose level ≥ 200 mg/dl on oral glucose tolerance test
Diabetes Classification
Type 1 Diabetes (formerly called insulin-dependent or juvenile diabetes)
• Body’s immune system attacks and destroys insulin-producing beta-cells in the pancreas • Rate of beta cell destruction varies
• In early stages may have sufficient beta cell function to prevent ketoacidosis, but will eventually require insulin
• Tend to be acutely symptomatic at onset, often complaining of polydipsia, polyphagia, polyuria, unexplained weight loss and dry mouth.
• Accounts for ~5-10 percent of diabetes in US
• Generally diagnosed in children and young adults, but can occur at any age • Likely genetic with possible environmental component
Diabetes Classification
Type 2 Diabetes (formerly termed non-insulin dependent or adult-onset diabetes) occurs due to
• Insulin deficiency (insufficient insulin production)
• Insulin resistance (inability to use insulin efficiently) • Initial compensatory mechanism involves increased insulin
production to normalize glucose levels, which eventually leads to insulin resistance
• As insulin resistance worsens, body has more difficulty producing insulin
• Process develops over decades • Initially asymptomatic
• Insulin resistance
• Hyperglycemia only following meals
• Diabetes
Diabetes Classification
Gestational Diabetes
• Glucose intolerance associated with onset during pregnancy
• Caused by hormones secreted during pregnancy and/or shortage of insulin
• Affects 5 to 10 percent of all pregnancies
• Usually diagnosed in 2nd or 3rd trimester
• Glucose tolerance typically returns to normal within 6 weeks after pregnancy ends
• Generally does not lead to retinopathy due to limited nature
• Women with GD has 35 to 60 percent chance of developing type 2 diabetes in 10-20 years
Diabetes Classification
Other Forms of Diabetes
• Secondary to:
• Genetic defects in beta-cell function or insulin action
• Pancreatic diseases
• Other endocrinopathies
• Medication
• Toxic chemicals
• Infections
• Uncommon (1-5% of all diabetes)
Risk Factors for Diabetes • Type 1
• Family history
• Viral exposure (Epstein-Barr, Coxsackie, Mumps, CMV)
• Autoimmune disease (Grave’s, Crohn’s, RA)
• Type 2 • Family history
• (BMI) ≥25 kg/m2
• Age >45 years
• Ethnic Background: American Indian, Alaska Native, Black, Hispanic/Latino, Asian American, Native Hawaiian and other Pacific Islander adults are nearly twice as likely as Caucasian adults to have type 2 diabetes
• H/o gestational diabetes or delivering baby > 9 lbs
• Blood pressure >140/90
• Abnormal cholesterol levels: HDL level < 35 mg/dl and/or a triglyceride level > 250 mg/dl
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Diabetic Retinopathy
• Diabetic retinopathy is leading cause of new blindness and visual impairment among Americans 20 to 74 years old
• Accounts for approximately 12 percent of all new cases of blindness each year
• Nearly 86 percent of individuals with type 1 diabetes mellitus and 40 percent of those with type 2 diabetes mellitus have some form of clinically evident diabetic retinopathy
• 20-40% of patients have signs of retinopathy at diagnosis
• 33-50% of patients with DM do not receive annual eye exams
Diabetic Retinopathy
• Diabetes duration and sustained hyperglycemia are among the primary risk factors for the development of diabetic retinopathy
• Intensive treatment to maintain blood glucose concentrations close to the normal range has been shown to decrease the risk of the development of diabetic retinopathy by as much as 76 percent.
Diabetic Retinopathy
Diabetic Retinopathy Findings
• Microaneurysms (MA) • Outpouching of retinal capillaries resulting from the loss of intramural
pericytes within the capillary walls • Face into areas of non-perfusion
• Retinal hemorrhages (H) • Ruptured or leaking microaneurysms or retinal capillaries • Typically occur within the inner nuclear and outer plexiform layers creating a
pinpoint or dot appearance • Hemorrhages >2A in all quadrants is associated with 48% chance of
developing neovascularization within 1 year
• Hard exudates • Lipoproteins from leaking retinal vasculature • Often associated with retinal edema
• Cotton wool spots • Localized infarct of the nerve fiber layer • Indicative of ischemia • CWS not associated with increased risk for neovascularization
• Image from Bresnick and Cuadros; EyePacs
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• Image from Bresnick and Cuadros; EyePacs • Image from Bresnick and Cuadros; EyePacs
• Image from Bresnick and Cuadros; EyePacs • Image from Bresnick and Cuadros; EyePacs
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Diabetic Retinopathy Findings
• Intraretinal microvascular anomalies (IRMA)
• New vessel growth within the retina or pre-existing vessels with endothelial cell proliferation that serve as “shunts” through areas of nonperfusion
• Indicates severe ischemic and neovascularization is likely to develop within a short time
• IRMA is associated with associated with 44% chance of developing neovascularization within 1 year
• Venous caliber abnormalities
• Venous dilation, venous beading (VB), or loop formation
• Large areas of nonperfusion can appear adjacent to these abnormal veins and are indicative of a substantial risk factor for neovascularization
• Also serve as strong indicators of severe retinal hypoxia
• VB associated with a 51% chance of developing neovascularization within 1 year
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Diabetic Retinopathy Findings
• Neovascularization (NV)
• The growth of new vessels, either at or near the optic disc (NVD), or elsewhere in the retina (NVE)
• Increased risk for vitreous hemorrhage or traction retinal detachment resulting in severe vision loss
• Diagnosis and treatment of PDR can prevent 50% of severe vision loss in advanced diabetic retinopathy
• EARLY diagnosis and treatment of PDR can prevent 90% of severe vision loss.
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Diabetic Retinopathy Findings • Diabetic Macular Edema (DME)
• Most common cause of vision loss in persons with diabetes
• May be present at any severity level of diabetic retinopathy
• Represents breakdown of blood-retina barrier when tight junctions of vascular endothelial cells break down
• Causes intraretinal fluid accumulation in the macula, causing photoreceptor disruption, and if untreated, increased risk of loss of vision
• 50% reduction in visual loss compared non-treatment after 1 year
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Image from Bresnick and Cuadros; EyePacs Image from Bresnick and Cuadros; EyePacs
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Clinical Practice Guidelines
• The AOA has developed the Evidence-Based Clinical Practice Guideline to provides ODs with examination and management recommendations designed to preserve vision and reduce the risk of vision loss in persons with diabetes, through timely diagnosis, appropriate management and referral.
• http://www.aoa.org/Documents/EBO/EyeCareOfThePatientWithDiabetesMellitus%20CPG3.pdf
Clinical Practice Guidelines
• Institute of Medicine:
“Clinical Practice Guidelines are statements that include recommendations intended to optimize patient care that are informed by a systematic review of the evidence and an assessment of the benefits and harms of alternative care options.”
Guidelines should:
• Be based on a systematic review of existing evidence
• Be developed by a knowledgeable, multidisciplinary panel of experts and key stakeholders.
• Be revised as appropriate when new evidence warrants modifications of recommendations
Case 1
• 40yo White Male
• Type 2 DM
• x 10 years
• Meds: none, diet controlled
• HbA1C: 7.2%
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No Retinopathy
• “Individuals with diabetes should receive at least annual dilated eye examinations. More frequent examination may be needed depending on changes in vision and the severity and progression of diabetic retinopathy.”
• “The individual’s primary care physician should be informed of eye examination results following each examination, even when retinopathy is minimal or not present.”
Case 2
• 35yo Hispanic Female
• Type 2 DM
• x 10 years
• Meds: none, diet controlled
• HbA1C: 6.8%
• Pregnant x 10 weeks
• Best corrected Distance VA
• OD 20/20 OS 20/20
• Full motilities, PERRL (-)APD
• Anterior segment unremarkable
• GAT 16/16 @ 3:15pm
• DFE
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• Diabetes and Pregnancy
• “Women with pre-existing diabetes who are planning pregnancy or who become pregnant should have a comprehensive eye examination prior to a planned pregnancy or during the first trimester, with follow-up during each trimester of pregnancy.”
• “Clinicians should use caution in administering topically applied drugs for pupillary dilation in pregnant women. Topically applied drugs for pupillary dilation, such as tropicamide, hydroxyamphetamine and phenylephrine are Pregnancy Category C drugs. When evaluation through a dilated pupil is necessary to assess diabetic retinal changes or unexplained decreased vision during pregnancy, the benefits of dilation may outweigh any potential risks. The use of digital punctual occlusion can minimize systemic absorption.”
Case 3
• 21yo White Male
• Type 1 DM
• x 16 years
• Meds: insulin
• HbA1C: 10.2%
• Best corrected Distance VA
• OD 20/20 OS 20/20
• Full motilities, PERRL (-)APD
• Anterior segment unremarkable
• GAT 17/18 @ 10:05am
• DFE
Case 1
ETDRS Classification
• Mild Non-Proliferative Diabetic Retinopathy
• At least 1 retinal microaneurysm
• Severity of hemorrhage or microaneurysm less than standard photo 2A
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Mild NPDR • “An annual dilated eye examination is generally sufficient
for monitoring the patient with mild NPDR, as long as there are neither DME nor coincident medical risk factors such as hypertension, renal disease or pregnancy, that may predispose patients to progression”
Case 4
• 65yo Hispanic Male
• Type 2 DM
• x 20 years
• Meds: orals
• HbA1C: 10.8%
• Best corrected Distance VA
• OD 20/20 OS 20/20
• Full motilities, PERRL (-)APD
• Anterior segment unremarkable
• GAT 15/16 @ 11:15am
• DFE
ETDRS Classification
• Moderate Non-Proliferative Diabetic Retinopathy
• Hemorrhage or microaneurysm greater than that depicted in standard photograph 2A in one to three retinal quadrants
• CWS, VB, or IRMA definitely present
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Moderate NPDR
• “For patients with moderate NPDR, fundus photography is strongly suggested, and repeat evaluation in 6 to 8 months is appropriate in the absence of DME or complicating medical or risk factors.”
• “If DME is present, but does not meet the criteria for CSME, follow-up every 2 to 4 months is advisable.”
Case 5
70yo White Male
• Type 2 DM
• x 40 years
• Meds: insulin and orals
• HbA1C: 12.1%
• Best corrected Distance VA
• OD 20/30 OS 20/40
• Full motilities, PERRL (-)APD
• Anterior segment: grade 1 NSC OU
• GAT 18/19 @ 12:45pm
• DFE
ETDRS Classification
• Severe Non-Proliferative Diabetic Retinopathy • Hemorrhage or MA ≥ standard photograph 2A in four quadrants • VB (standard photograph 6B) in two or more quadrants • Prominent IRMA ( ≥than standard photograph 8A) in at least one
quadrant. • Sometimes referred to as “4-2-1” rule • Hemorrhages >2A in all quadrants is associated with 48% chance of
developing neovascularization within 1 year • VB in 2 quadrants associated with a 51% chance of developing
neovascularization within 1 year • IRMA is associated with associated with 44% chance of developing
neovascularization within 1 year • CWS not associated with increased risk for neovascularization
• Very Severe Non-Proliferative Diabetic Retinopathy • Two or more criteria for severe NPDR are met, in the absence of
frank neovascularization
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ETDRS Classification
• Diabetic Macular Edema (DME)
• Retinal thickening within two disk diameters (DD) of the center of the macula
• Clinically Significant Macular Edema (CSME)
• Thickening of the retina ≤ 500 microns (1/3 DD) from the center of the macula
• Hard exudates ≤ 500 microns (1/3 DD) from the center of the macula with thickening of the adjacent retina.
• A zone or zones of retinal thickening ≥1 DA in size, any portion of which is ≤ 1 DD from the center of the macula.
• Hard exudate within one disc diameter (1DD) (1500 microns) of the center of the macula will identify CSME with 94% sensitivity
• CSME associated with 10 fold increased risk for “moderate visual loss” (defined as doubling of the visual angle, e.g. 20/4020/80)
Severe/Very Severe NPDR
• “Follow-up every 2 to 3 months in consultation with an ophthalmologist experienced in the management of diabetic retinal disease is advisable for patients with severe or very severe NPDR.”
CSME
• “Consultation with an ophthalmologist experienced in the management of diabetic retinal disease is indicated if PDR or DME is suspected or if there is an unexplained loss of visual acuity.”
Case 6
35yo White Male
• Type 1 DM
• x ~30 years
• Meds: insulin
• HbA1C: 15.3%
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ETDRS Classification
• Proliferative Diabetic Retinopathy (PDR)
• New vessels on or within one disc diameter of the disc (NVD) or new vessels elsewhere on the retina (NVE) < standard photo 10A
• Fibrous proliferation on or within one disc diameter of the optic disc (FPD) or elsewhere on the retina (FPE)
• Preretinal hemorrhage (PRH) or NVE < ½ disc area without NVD
• High Risk Proliferative Diabetic Retinopathy
• NVD > one-fourth to one-third disc area in size (standard photograph 10A)
• NVD < one-fourth disc area in size with fresh vitreous hemorrhage (VH) or preretinal hemorrhage (PRH) present
• NVE > one-half disc area in size with VH or PRH present.
• Without treatment 50% of eyes with PDR are blind within 5 years
PDR
• “Consultation with an ophthalmologist experienced in the management of diabetic retinal disease is indicated if PDR or DME is suspected or if there is an unexplained loss of visual acuity.”
• “Prompt referral to a vitreo-retinal surgeon is indicated when a vitreous hemorrhage, a retinal detachment or other evidence of proliferative diabetic retinopathy is present.”
Conclusions
• Diabetes and management of diabetic retinopathy will continue to be a major issue in healthcare
• ODs should continue to be at the forefront of screening for and monitoring diabetic retinopathy
• When in doubt, don’t be afraid to refer anything outside your comfort level
• Stay current with the AOA’s Clinical Practice Guidelines http://www.aoa.org/Documents/EBO/EyeCareOfThePatientWithDiabetesMellitus%20CPG3.pdf
Thank You and Questions?
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AMD Update Dina Erickson, OD, FAAO
Outline: Background:
• Geographic atrophy and neovascular age related macular degeneration Treatment options: Dry AMD:
• AREDS II o Study criteria o Nutritional changes from AREDS I formula o Major outcomes o Possible study weaknesses o What have we learned and how should we advise our patients based
on AREDS II outcomes
Wet AMD: • Current FDA approved treatments • Photocoagulation • PDT • Anti-VEGF tx:
o Macugen o Lucentis o Avastin? o Eylea o Patient education regarding Anti VEGF treatment o Potential side effects
• Case presentation:
o 75 year old Caucasian male seeing “white lines on the road as sheep jumping” with OD for 3 weeks.
• Recent Studies and their outcomes: o CATT, o IVAN o View I and View II
• Current therapy strategy:
o Monthly treatment o Monthly then as needed
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o Treat and extend
Potential new strategies: o Extended delivery methods for anti VEGF Tx o Anti PDGF:
Fovista o Combination Tx: VEGF/PDGF
Injections Potential oral combination on the horizon How may this affect the practice of optometry
• Eye Drops to treat wet AMD:
o Pazopanib Eye Drops A Randomised Trial in Neovascular Age-related Macular
Degeneration
• Genetics and AMD: o Is AMD in our DNA? o Genetic testing:
Macular Risk RetnaGene
o To test or not to test? o Genetic therapy
Genzyme gene therapy for AMD Future of genetic Tx
• Stem Cell therapy
o What is stem cell therapy o Where we are and where we are headed with AMD and stem cell
therapy
• How to get reimbursed for your AMD patients o ICD-9 for insured patients
What to tell your patients about:
o AMD and aspirin To use or not to use
o AMD and homocysteine level Association of Homocysteine levels to AMD progression
o Blue light and link to AMD
FDA approved Implantable miniature telescope for end stage AMD • When is it used • How is it used • How does it work
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• Who can benefit from this device
Final thoughts on the care and education of your AMD patient • When recommending treatment consider the patient’s:
o Age o Level of activity o Transportation issues o General health o Visual goals
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THE EFFECTS OF SMOKING ON
THE EYE
Ryan Bulson, O.D., M.S., F.A.A.O.
LEARNING OBJECTIVES
Be able to describe the most common ocular
effects of smoking
Be able to discuss management options for the
most common ocular conditions found in smokers
Be able to provide a list of resources for patients
interested in quitting
Be able to discuss the most common smoking
cessation strategies with patients
THE PROBLEM
Tobacco smoke is known to have 4,000 active
compounds, including 40 known chemical
carcinogen
Cigarette smoking is the primary preventable
cause of disease, disability, and premature death
in the United States
According to the CDC, ~20% of Americans are
smokers
~15% of Canadians over age 15 are smokers
Nearly 70% of smokers report a desire to quit
THE PROBLEM
Kennedy et al (2014)
Survey sent to 4528 optometrists in Canada regarding their knowledge and practices for patients who smoke
98% believed that smoking cigarettes was a risk factor for AMD, however, knowledge levels of associations were lower for other eye diseases
Only 55% assessed the smoking status of patients during their initial visit
33% reported that they always or regularly assess their patients’ interest in quitting smoking.
7% reported that they discussed the benefits of tobacco use prevention with patients younger than 19 years
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WHY ARE WE NOT EDUCATING PATIENTS
ON THE EFFECTS OF SMOKING?
WHY YOU ARE HERE!
90% were interested in a continuing education
program about the impact of smoking on vision
and eye health as well as strategies for
discussing tobacco cessation and prevention
Study Conclusion: “Opportunities for continuing
education around cessation--including training
specifically for optometrists--need to continue to
increase.”
SMOKING AND THE EYE
Toxic effects of smoking related to
Cell damage from free radicals
Decrease blood flow to ocular vasculature
Increased formation of clots with ocular vasculature
THE DOCTOR’S REPORT
http://www.youtube.com/watch?v=X0tWONdhyO
0&feature=related
CORNEA AND TEAR FILM
Very thin (~0.5mm) transparent front surface layer of the eye
Responsible for ~2/3 of the eye’s refractive power
No vasculature
Receives oxygen passively from environment
Receives nutrition/waste removal via aqueous humor
One of the body’s most densely innervated tissues
Tear film coats the most anterior layer of the cornea (epithelium) and maintains moisture
Lipid layer (prevent evaporation)
Aqueous layer (maintain moisture)
Mucin layer (adherence to the cornea)
SMOKING AND THE CORNEA/TEAR FILM
Increases risk of dry eye syndrome
Symptoms include:
Burning
Itching
Redness
Tearing
Foreign body sensation
Intermittent blurred vision
Second hand smoke increases dry eyes in children
Mechanism: free radicals and other pro-oxidants in
smoke/tar cause lipid peroxidation (break down of lipid
layer) and alteration of tear proteins
Results in a reduced tear break up time
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SMOKING AND THE CORNEA/TEAR FILM
Treatment:
Eliminate offending agent
Lubrication (artificial tears, ophthalmic ointments)
Restasis
Punctal occlusion
Omega-3s
Mechanism
Anti-inflammatory?
Enhancement of lipid layer of tear film?
How much?
No FDA recommendation (1g-6g/day)
EXTRAOCULAR MUSCLES
Six muscles responsible for movement and
alignment of the eyes
EXTRAOCULAR MUSCLES
Cigarette smoking is a risk factor
for Graves’ disease
Development
Progression
Severity (increased risk of diplopia
and exophthalmos)
Poorer outcomes with immunosuppressive therapies
in the active phase
Increases risk of progression following radiotherapy
The relationship between smoking and effect may
be dose dependent
EXTRAOCULAR MUSCLES
Graves’ Disease Signs/Symptoms
Ocular irritation, redness, tearing, photophobia
Diplopia
“Stare” due to lid retraction
Exophthalmos
EOM restriction
Dry eyes/exposure keratopathy
Lagophthalmos
Elevated IOP
Compressive optic neuropathy (~5%)
Management
Copious lubrication, nocturnal lid taping
Systemic steroids, radiotherapy, surgery
CONJUNCTIVA AND SCLERA
Conjunctiva is the thin cellophane-like layer that
lines the sclera and inner eyelids
Responsible for maintaining moisture of the ocular
surface
Sclera is the white/opaque, fibrous, layer of the
eye located directly posterior to the conjunctiva
Primary role is protective in nature
Smoking increases the risk
of conjunctivitis
Bacterial
Viral
Allergic
CONJUNCTIVA AND SCLERA
Conjunctivitis symptoms:
“Pink eye” (cosmesis)
Burning
Itching
Redness
Tearing
Foreign body sensation
Swollen eyelids
Increased sensitivity to light
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CONJUNCTIVA AND SCLERA
Mechanism: toxins and irritants in smoke causes
allergic response that results in inflammation
Higher amount of squamous metaplasia in the
conjunctival epithelium
Reduction or absence of conjunctival growth factors
Treatment:
Eliminate offending agent
Lubrication
ANTERIOR CHAMBER
Space between cornea and iris that houses
aqueous
Plays a nutritional role with posterior cornea,
trabecular meshwork, lens, and anterior vitreous
Maintains intraocular pressure
Smoking doubles the risk for infectious and
inflammatory uveitis
Symptoms
Eye pain
Redness
Photophobia
Tearing
ANTERIOR CHAMBER
Mechanism: pro-inflammatory components of
tobacco smoke cause vascular inflammation (via
hydrogen peroxide and upregulation of
cytokines), which promotes organism entry to
intraocular tissue
Tobacco smoke may also enhance the response of
inflammatory cells to the microorganism
Treatment
Topical steroids
Topical cycloplegic
Systemic work up as indicated
LENS
Crystalline lens is a transparent refractive
medium that provides ~1/3 of the eye’s power
In young patients (<45), changes shape to change
focus (accommodation)
Cloudiness of the lens is called a cataract
LENS
Cataract symptoms
Blurred vision (distance and near)
Difficulty night driving (pupil dilates)
Glare/haloes around lights
Poor glare recovery (oncoming headlights)
Mechanism:
Systemic absorptions of smoke constituents produces
oxidative stress
Accumulation of metals such as Cd and Fe and the
reduction in levels of vitamin C in the lens and blood
LENS
Treatment:
Eliminate offending agent
Cataract surgery
Vitamins/Supplements are inconclusive
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OPTIC NERVE
Responsible for conduction of sensory signal from
the retina to the visual cortex
Cranial nerve II
“Blind spot”
http://drugster.info/ail/pathography/2765/
http://drugster.info/ail/pathography/2765/
OPTIC NERVE
Smoking has been associated with increased risk of
Optic neuritis
Poor recovery than non-smokers (more R/G color defects)
May speed progression and/or increase relapses
Ischemic optic neuropathy
Toxic optic neuropathy
OPTIC NERVE
Symptoms of optic neuritis/neuropathy
Blurred vision (minorprofound)
Visual field defect
Pupillary abnormality
Color deficiency
Pain (in acute inflammatory phase)
Treatment of optic neuritis/neuropathy
Remove offending agent
IV/Oral steroids in acute inflammatory phase
Speeds resolution but does not improve outcome
Low vision rehabilitation
OPTIC NERVE Glaucoma is a bilateral, progressive death of the optic
nerve
2nd leading causes of blindness worldwide
Affects 2-3 million in the US; ~10% of blindness in the US
Exact pathogenesis is unclear
Treatment involves controlling the disease there is no cure
Smoking increases the risk and progression of glaucoma
and worsens surgical outcomes
OPTIC NERVE
Mechanisms
Smoking produces ischemia and oxidative stress
Smoking increases risk of vascular disease and glaucoma
likely has an underlying vascular perfusion component
Toxic substances that increase in free radicals and
decrease in antioxidants
Mitochondrial dysfunction
Treatment
Remove offending agent
Reduce intraocular pressure
Topical medication
Surgery
RETINA
Sensory portion of the eye
Contains rod and cone photoreceptors
Transmits visual sensory information to the
brain via optic nerve
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RETINA Smoking is associated with increased risk of
Diabetic retinopathy (DR) and Diabetic Macular Edema (DME)
Age related macular degeneration (ARMD/AMD)
RETINA Diabetic retinopathy and diabetic macular edema
Leading cause of blindness in people <65
10% of world will be diabetic by 2035; 11% of US
DME is the most frequent cause of severe vision
impairment in diabetic patients
Vision loss is secondary to the accumulation fluid
within the macula (most sensitive part of vision)
Treatment
Glycemic control
Laser Photocoagulation
Anti-VegF injection
RETINA
Age related macular degeneration
Affects ~10 million in US
Accounts for 50% of severe and irreversible vision
loss in the US, particularly those >65
Expected to rise to nearly ~20 million by 2050
Population attributable risk percent for smoking
and AMD is 13.8%
Dry AMD Wet AMD
RETINA
Mechanism:
Oxidative damage to retinal pigment epithelial cells
Depression of serum antioxidant levels
Alterations of choroidal blood flow and of retinal pigment
epithelium drug detoxification pathways
Decrease luteal pigments to increase, increasing damage to
the macula by light and oxidative damage,
RETINA
Treatment:
Early Dry AMD-No treatment
Intermediate/Late Dry AMD-AREDS formula
vitamins
500mg Vitamin C
400mg Vitamin E
15mg Beta carotene*
80mg Zinc
2mg Copper
Reduces risk of progression to advanced (exudative/wet) by
25% in patients with intermediate/late dry AMD
Wet AMD-Anti-VegF (Lucentis/Avastin) injections
REFRACTIVE CONSIDERATIONS
Smoking does not appear to influence refractive
error of smokers, but can second hand smoke
influence children’s refractive error?
Study of ~4000 children age 1-6 years (STARS)
Inverse relationship between child myopia and
H/o maternal smoking
Smoking during child’s life
Smoking during pregnancy
H/o paternal smoking
Previous study of older children (mean 8.7)in the
US found similar inverse relationship
Mechanism: nicotinic acetylcholine receptors?
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MARIJUANA
23 States and DC have legalized medical use of
marijuana
2 States have legal recreational marijuana
THC is the primary compound marijuana’s
neurogenic and psychotropic effects
Stimulate specific dopamine receptors in the central
nervous system, resulting in an induced state of
euphoria or relaxation Kabat and Sowka
MARIJUANA
Initial studies in the 1970s (Hepler and Frank) showed IOP lowering effect peaking at 60-90 minutes and lasting only 3-4 hours
Prostaglandin analog may have IOP lowering effect 80+ hours
Side effects
Conjunctival hyperemia
Diminished tear production (leading to dry eye)
Pupillary mydriasis
Alteration of blood pressure
Cardiac arrhythmias
Psychogenic effects: disruption of short-term memory, cognitive impairment, a sense of time distortion, reduced motor coordination and sleepiness
MARIJUANA
Mariol-synthetic version of THC available by Rx
in 2.5mg, 5mg or 10mg capsules
Topical routes are challenging due to the
hydrophobic/ lipophilic nature of cannabinoids,
which make them insoluble in water.
Canasol-topical glaucoma medication marketed in
Jamaica
MARIJUANA
Position from American Academy of
Ophthalmology:
“Based on analysis by the National Eye Institute
and the Institute of Medicine, the Academy finds
no scientific evidence that marijuana is an effective
long-term treatment for glaucoma, particularly
when compared to the wide variety of prescription
medication and surgical treatments available.”
SMOKING CESSATION
Nearly 70% of smokers report a desire to quit, but many lack access to resources to help
Physician involvement greatly increases success rates, but physicians are not being as effective as they could be in helping patients quit
Long term abstinence alone: 7%
Long term abstinence with help of physician: 30%
Less than 3 minutes of counseling by health care providers has been shown to increase quit rates by 30% (US DHS)
Ophthalmologists are more likely to counsel on smoking cessation than Optometrists (Lawrenson, 2013)
SMOKING CESSATION BY EYE CARE PROVIDERS
Caban-Martinez et al
Cessation counseling for patients with AMD
Most patients who smoke reported never being
advised to quit smoking
Most eye care providers reported that they had
advised smokers to quit.
Two-thirds of providers expressed a desire for
additional training and resources to support
patient quit attempts, indicating the need for the
integration of smoking cessation opportunities in
the clinic setting.
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SMOKING CESSATION BY PCPS (AAMC)
All physicians surveyed believe it is their role to help
patients quit smoking.
86% ask patients who smoke about their smoking status
and advise them to stop
13% usually refer smokers to others for appropriate
treatment
17% arrange for follow-up visits to address smoking.
Five factors cited most often by physicians as
significant barriers to successful intervention:
(1) lack of patient motivation (63%)
(2) limited coverage for interventions (54%)
(3) limited reimbursement for a physician’s time (52%)
(4) time with patients is limited (41%)
(5) too few available cessation programs (39%)
SMOKING CESSATION OPTIONS
According to PCPs, “highly effective”
interventions include
Bupropion with nicotine replacement (29%)
Nicotine replacement with counseling (21%)
Family support (19%)
Evidence based studies have suggested these
interventions have success rates (long term
abstinence) up to 38%
NICOTINE REPLACEMENT
Designed to wean the body off cigarettes
Supply nicotine in controlled amounts without chemicals found in cigarettes
OTC nicotine replacement products include
Skin patches-transdermal nicotine patches
brand names Habitrol and Nicoderm
Chewing gum
Brand name Nicorette
Lozenges
Brand name Commit
Rx only product
Nasal spray or inhaler
E-cigarettes-controversial
NON-NICOTINE MEDICAL THERAPY
Chantix (varenicline tartrate)
Acts at sites in the brain affected by nicotine. It
provides some nicotine effects to ease withdrawal
symptoms and blocks the effects of nicotine from
cigarettes if users resume smoking.
Side effects include
Nausea
Constipation
Gas
Vomiting
Trouble sleeping
Vivid, unusual, or strange dreams.
Changes in behavior, depressed mood, hostility, and
suicidal thoughts
NON-NICOTINE MEDICAL THERAPY
Zyban (buproprion)
Aids in cessation; precise mechanism unknown
Same active ingredient as the antidepressant
Wellbutrin
Side effects include
Insomnia
Dry mouth
Changes in behavior, depressed mood, hostility, and
suicidal thoughts
COUNSELING & FAMILY SUPPORT
Mentoring programs and smoking counselors
Support groups
Helplines
Smokefree Text
Encouragement, tips, and advice via text
Apps QuitSTART-free-tracks cravings and moods, monitors and
milestones, identifies smoking triggers, personalized "pick me ups" for challenging times to
QuitPal-not yet available-integrates calendar to set date goals, tracks monetary savings and financial goals, personalized messages from loved ones, social networking
QuitGuide-designed to help prepare quitting and provides support in the days and weeks after quitting via information and social networking
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ONLINE RESOURCES
Government Resources
National Quit line: 1-800-QUITNOW
SmokeFree.gov http://www.smokefree.gov
National Cancer Institute http://www.cancer.gov/cancertopics/factsheet/Tobacco/cessation Telephone Hotline: 1-877-44U-QUIT (448-7848)
National Institutes of Health Smoking Cessation http://health.nih.gov/topic/SmokingCessation
Health Resources and Services Administration http://www.hrsa.gov/stopsmoking/
1-800-QUITNOW http://1800quitnow.cancer.gov/
Centers for Disease Control and Prevention http://www.cdc.gov/tobacco/quit_smoking/how_to_quit/index.htm
Agency for Healthcare Research and Quality http://www.ahrq.gov/consumer/tobacco/lowlit.htm
Womenshealth.gov http://www.womenshealth.gov/quit-smoking/
Healthfinder Tobacco Resources Page http://healthfinder.gov/scripts/SearchContext.asp?topic=860
MedlinePlus Tobacco/Smoking http://www.nlm.nih.gov/medlineplus/smoking.html
ONLINE RESOURCES
Outside Organizations
American Lung Association http://www.lungusa.org/site/c.dvLUK9O0E/b.22931/k.8550/Smoking_Cessation_Support.htm Telephone Hotline: 800-LUNG-USA (586-4872)
American Cancer Society http://www.cancer.org/docroot/subsite/greatamericans/content/Guide_to_Quitting_Smoking.asp Telephone Hotline: 800-ACS-2345 (228-2345)
American Heart Association http://www.americanheart.org/presenter.jhtml?identifier=3048036
Become an Ex (project of the National Alliance for Tobacco Cessation) http://www.becomeanex.org/
American Legacy Foundation http://women.americanlegacy.org/quit/index.cfm Telephone Hotline: 866-66-START (667-8278) [for pregnant smokers]
Campaign for Tobacco Free Kids http://www.tobaccofreekids.org/index.php
American Association for Respiratory Care http://www.aarc.org/resources/tobacco/index.asp
CONCLUSIONS AND THE GOOD NEWS!
Smoking increases the risk for a variety of ocular
conditions
These ocular conditions range from nuisances to sight
threatening
BUT, studies suggest there is a dose dependent
relationship between tobacco and tobacco use–
related eye diseases
THEREFORE: risk of tobacco use–related eye
disease will likely decrease substantially after
smoking cessation
We play a valuable role in providing resources to
assist patients in quitting
Please complete your session evaluation using EyeMAP™ online at
http://eyemap.cistems.net
Tweet about this session using the official meeting hashtag #aaoptom14
REFERENCES
Age-Related Eye Disease Study 2 Research Group. Lutein + zeaxanthin and omega-3 fatty acids for age-related macular degeneration: the Age-Related Eye Disease Study 2 (AREDS2) randomized clinical trial. JAMA. 2013 May 15;309(19):2005-15.
Asensio-Sánchez VM, Gómez-Ramírez V, Morales-Gómez I, Rodríguez-Vaca I. Clinically significant diabetic macular edema: systemic risk factors. Arch Soc Esp Oftalmol. 2008 Mar;83(3):173-6.
Caban-Martinez AJ, Davila EP, Lam BL, Dubovy SR, McCollister KE, Fleming LE, Zheng DD, Lee DJ. Age-related macular degeneration and smoking cessation advice by eye care providers: a pilot study. Prev Chronic Dis. 2011 Nov;8(6):A147.
Krishnaiah S, Vilas K, Shamanna BR, Rao GN, Thomas R, Balasubramanian D. Smoking and its association with cataract: results of the Andhra Pradesh eye disease study from India. Invest Ophthalmol Vis Sci. 2005 Jan;46(1):58-65.
Iyer JV, Low WC, Dirani M, Saw SM. Parental smoking and childhood refractive error: the STARS study. Eye (Lond). 2012 Oct;26(10):1324-8
Lawrenson JG, Evans JR. Advice about diet and smoking for people with or at risk of age-related macular degeneration: a cross-sectional survey of eye care professionals in the UK. BMC Public Health 2013;13:564.
Lin P, Loh AR, Margolis TP, Acharya NR. Cigarette smoking as a risk factor for uveitis. Ophthalmology 2010;117:585-90.
Macsai MS. The role of omega-3 dietary supplementation in blepharitis and meibomian gland dysfunction (an AOS thesis). Trans Am Ophthalmol Soc. 2008;106:336-56.
Physician behavior and practice patterns related to smoking cessation summary report. Washington (DC): American Association of Medical Colleges; May 2007. http://www.aamc.org/workforce/smoking-cessation-summary.pdf.
Timothy CO, Nneli RO. The effects of cigarette smoking onintraocular pressure and arterial blood pressure ofnormotensive young Nigerian male adults. Niger J PhysiolSci 2007; 22:31–35
US Department of Health and Human Services. Agency for
Healthcare Research and Quality. Clinical Care Guideline. Treating
Tobacco Use and Dependence: 2008 Update. Available at: http://www.ahrq.gov/professionals/clinicians-providers/guidelines-recommendations/ tobacco/clinicians/update/treating_tobacco_use08.pdf.
Vingerling JR, Hofman A, Grobbee DE, de Jong PT. Age-related macular degeneration and smoking. The Rotterdam Study. Arch Ophthalmol. 1996 Oct;114(10):1193-6.
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Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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31 of 46
8/29/2014
2
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyesFOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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32 of 46
8/29/2014
3
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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33 of 46
8/29/2014
4
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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34 of 46
8/29/2014
5
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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35 of 46
8/29/2014
6
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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36 of 46
8/29/2014
7
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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37 of 46
8/29/2014
8
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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38 of 46
8/29/2014
9
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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8/29/2014
10
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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40 of 46
8/29/2014
11
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyesFOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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41 of 46
8/29/2014
12
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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42 of 46
8/29/2014
13
Grand Rounds
Ryan Bulson, O.D., M.S., [email protected]
Dina Erickson, O.D., [email protected]
Pacific University College of Optometry
Case #1
27 yo Caucasian female CC: Possible visual field defect detected on confrontation testing
Unaware of defects in daily lifeDenied neurological symptoms
Ocular History: UnremarkableROS: UnremarkableMedication: NoneFamily Ocular History: Glaucoma (maternal aunt)
Case #1
Uncorrected distance visual acuityOD 20/15 OS 20/15
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fieldsSuperior nasal constriction OD and OS
Refraction: +1.00 DS OUAnterior Segment: UnremarkableGAT (@2:32pm): 24mmHg OD/OSPosterior Segment:
Follow up 1 week later
No changes in vision, no new visual/ocular/neurological complaintsUncorrected distance acuity20/15 OD/OS
Motilities full and comitantPERRL (-)APD Ishihara Color Testing10/10 plates correct OD/OS
GAT @ 1:29pm: 22/23 mmHgPachymetry: 586/581 micronsGonioscopy: flat approach, open to CB 360 OU, tr pigment
http://www.westcoastretina.com/WestCoastRetina/Nov-2010.html
Follow up over ~ 3 years
No new ocular/visual/neurological complaintsNo treatment has been electedUncorrected visual acuityOD 20/15 OS 20/15
Tmax 25/25, range: 22-25
Optic Nerve Head Drusen
Congenital, acellular, calcific bodies commonly often found bilaterally and associated with small, crowded optic nerves
It is believed that the formation of ONHD is associated with axonal transport alteration and degeneration.
The prevalence of ONHD in the general population has been estimated at approximately 0.4%; however, histological studies have reported prevalence as high as 2.4%
Optic Nerve Head Drusen
In young patients, drusen typically “buried”With age, “buried” drusen enlarge due to calcium apposition and extend anteriorlyAs the drusen expand, the nerve fiber layer is disrupted, resulting in visual field loss
in 24-87% of adults (Auw-Haedrich et al)Central visual acuity is generally spared, and thus this condition is often
asymptomatic
Pseudopapilledema
Critical to differentiate pseudopapilledema from drusen from true optic nerve edemaBlurring of disc marginsObscuration of retinal vesselsPeripapillary hemorrhages Retinal/choroidal folds
B-scan continues to be the gold standard for detecting drusenOCT (and AF) gaining popularity
Optic Nerve Edema or Optic Nerve Drusen?
Lee et al
Treatment
No universally accepted treatment protocolContrasting views on whether nerve fiber layer loss occurs more rapidly in presence
of ocular hypertension (Moussalli et al, Grippo et al)Some evidence that treatment with topical alpha-2 adrenergic agonist brimonidine
may provide neuroprotection in addition to it’s anti-hypertensive effect
Brimonidine
Believed to upregulate intrinsic cell survival signaling pathways, as well as antiapoptotic genes.
In rat models, has been shown to reduce levels of intravitreal glutamate associated with optic nerve degeneration secondary to ischemia.
Elevates neurotrophic factors important in preserving retinal ganglion cells after insult.
Brimonidine
In laboratory studies, has demonstrated three out of four criteria required to demonstrate neuroprotective effectsReceptors on target tissueAdequate penetration to the retinaInduction of intracellular changes that enhance neuronal resistance to insult in
animals studiesTo date, has yet to satisfy the fourth criterion: demonstrated efficacy in human
clinical trials.
Pregnancy and Glaucoma
Conventional wisdom is to defer treatment during pregnancyThere is a statistically significant drop in IOP during pregnancy (from 1st to 3rd
trimester) (Dinh; Marris)Mechanisms?Increase in outflow facilityDecrease in episcleral venous pressureDevelopment of a mild metabolic acidosis
Pregnancy Categories-Glaucoma
Category B: BrimonidineCategory C: Beta blockers, prostaglandin analogues, CAIs, cholinergics, apraclonidine
Pregnancy Categories
Category A: Studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
Category B: Animal studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
Category C: Animal studies have shown an adverse effect on the fetus and there are no studies in humans, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category D: There is positive evidence of human fetal risk based on human studies, but potential benefits may warrant use of the drug in pregnant women despite potential risks.
Category X: Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based human studies, and the risks involved in use of the drug in pregnant women clearly outweigh potential benefits
Case #2
16yo HFCC: blurred distance vision x 1-2 monthsEstablished patient, LEE 2 years ago: unremarkableROS: unremarkableMedication: NoneOcular history: unremarkableFamily ocular history: unremarkable
Case #2
Uncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields: Generalized constriction OD/OSRefraction: OD: -1.25 DS 20/50 OS: -1.00-0.75x005 20/50
Anterior Segment: UnremarkableGAT (@2:55pm): 12 mmHg OD/OSPosterior Segment:
Follow up 1 week later
No new visual/neurological complaintsUncorrected distance visual acuityOD 20/150 PH 20/50 OS 20/200 PH 20/50
PERRL (-)APDMotilities full and comitant without pain/diplopiaColor Vision: OD/OS: test plate correct, 7/11 correctBlood Pressure: 104/50
Ordered labs, f/u 1-2 weeks
CBCSerum levels of B1 (thiamine), B9 (folate), B12 (cobalamin) If normal, may considerFTA-ABS/VDRLHeavy metal screen (e.g. Lead)MRI
Follow up
Patient placed on liquid multivitamin supplement Follow up 1 month
1 month follow up
Vision improving, feeling better and has more energyCorrected distance visual acuityOD 20/20- OS 20/20-
Color vision: 17/17 OD/OS
Common Causes of Anemia
Common Causes of Vision Loss in Teenagers
Anemia
Condition in which the body does not possess enough healthy red blood cellsVarious classification typesMorphological-Based on size (mean corpuscular volume)Microcytic-MCV <80 fLMacrocytic-MCV >100 fL
Pathogenic-Based on etiology
Anemia
Adolescent females are 10 times more likely to develop anemia than adolescent boys (CDC)
Mexican heritage and poor socioeconomic status have been shown to increase risk for developing the condition
While anemia is a rare cause of vision loss, it can produce optic nerve ischemia, particularly in the setting of hypotension.
Ischemic optic neuropathies related to anemia have been reported in cases of repeated gastrointestinal bleeding, trauma involving excessive blood loss, spinal and cardiac bypass surgeries, nasal/sinus surgery, and spontaneous abortion.
Iron deficiency anemia has been associated with other optic neuropathies, including NAION and papilledema.
Case #3
9 yo HF Previous Records:
Subjective:OD: +0.75-1.75x180 OS: -0.50sph
BCVA OD/OS/OU: 20/60SLE/DFE: unremarkableA/P: New spectacle Rx given, RTO 1 month.
F/U visit 1 month later: No change in vision-BCVA remains 20/60.Ishihara: (?)Red-Green and Blue-Yellow Deficiencies
A/P: Reduced BCVA/color. Refer to University
Case #3
9 yo HF Case Hx (per parents):
Difficulty localizing objects Excessive sensitivity to sunlight. Avoids outdoor activities.Difficulty functioning at nightAll visual difficulties began around age 5
Mild speech impairment and learning disabilityMildly overweightNormal pregnancy and birth. (+) PolydactylNo FHx of any visual/ocular conditions
Patient K.H.
Corrected Distance Visual AcuityOD/OS/OU: 20/60 PH:NI
Motilities full and comitantPERRL (-)APDRefraction: OD: +0.75-1.75x180 OS: -0.50sph20/60 OD/OS/OU
Ishihara: Test plate correct, all others missedAnterior segment: unremarkableGAT: 12/12 @ 10:30amPosterior Segment:
Mom
Mom
Dad
Dad
K.H.
Dad
GDX
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of PIL centrally and peripherally
Patient K.H.
A/PPresumed Early Cone-Rod dystrophy.Educated on condition, potential for future progression of vision loss/visual
impairment, sunglasses whenever outside. Parents encouraged to pursue educational services in school.
Defer ERG at this time
Do We Need an ERG?
Microstructural retinal changes are commonly observed in patients with inherited retinal dystrophies using high definition OCT. (Gerth et al).
Absence or interruption of the photoreceptor integrity line, as seen in inherited retinal dystrophies, is associated with reduced visual function.
High resolution OCT images can be used to predict visual function through the presence, size, and integrity of the photoreceptor integrity line (Aizawa et al).
Patient K.H.
Summary of relevant findings:Reduced BCVA (20/60)Reduced color visionh/o excessive sunlight sensitivity and difficulty functioning at night, polydactyl, LD,
overweightClinically excessive retinal sheen confirmed by thickened RNFL, possible arteriolar
attenuationAbsence of junction centrally and peripherally
Patient K.H.
Is there more to this case than a cone-rod dystrophy?
Laurence-Moon-Bardet-BiedlSyndrome
Primary Features
Rod-Cone DystrophyPolydactylyObesity Learning DisabilitiesHypogonadism in MalesRenal Anomalies
Secondary Features
Speech DisorderBrachydactylyDevelopmental DelayPolyuriaAtaxia
Secondary Features
Poor CoordinationDM Left Ventricle HypertrophyHepatic FibrosisSpasticity
Diagnosis
Must exhibit 4 primary characteristics or 3 primary and 2 secondary. Primary:Rod-Cone DystrophyPolydactylyObesityLearning Disabilities
Secondary:Speech DisorderDevelopmental DelayAtaxiaPoor Coordination
Treatment
The only treatment is the management of symptoms. Vision- Low Vision ServicesObesity- DieticianKidney Problems-Medication/TransplantPolydactyly- surgical removal
Demographics
Prevalence rate in North America/Europe ranges from 1:14,000 to 1:16,000 live births (Sharifian et al)
However, in some regions such as Kuwait, the rate is higher, with an estimated incidence of 1:13,500 due to a high frequency of consanguine marriages.
While this syndrome is considered rare, it is suspected that there is a major problem with its under-diagnosis
Genetics and Types
Type 1 11q13-- Most CommonType 2 16q21Type 3 3p12-- Least CommonType 4 15q22There are patients with the disease that do not have any of the genes listed above.
Case 4
53 yo WFCC: blurred distance and near vision OUOHx: glaucoma suspect x many years, h/o corneal abrasion OD age 20, h/o dry eyes FOHx: strabismus (sister), cataracts (parents)PMx: asthma (albuterol), bipolar/depression (tegretol,wellbutrin), h/o astrocytoma
right side s/p surgery, radiation, and chemotherapy (~20 years ago)
Case 4
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaConfrontation fields?superior restriction OD/OSFDT 30-5 screener: scattered non-specific defects superior>inferior
Refraction: -0.50DS to 20/20 OD/OSAnterior Segment: UnremarkableGAT (@10:53am): 15mmHg OD/OSPosterior Segment:
A/P
Low risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Possible superior constriction on confrontation fields OD/OS; FDT demonstrates
non-specific defects superior>inferiorOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 2 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@10:44am): 17mmHg OD/OSPachymetry: 527/529Gonioscopy: flat approach, open to CB 360 OU Visual Field
Moderate risk normotensive glaucoma suspect Moderate physiological cupping with healthy appearance Suspicious glaucomatous field defects OS>ODOCT demonstrates thinning superior OD, no thinning OSH/o right side astrocytoma status post radiation, surgery, and chemotherapy that
may confound visual fieldNo known family history of glaucoma
Follow up 3-4 weeks later
Uncorrected distance visual acuityOD 20/25 OS 20/25
PERRL (-)APDMotilities full and comitant without pain/diplopiaAnterior Segment: UnremarkableGAT (@11:21am): 16/13mmHg Visual Field
Astrocytoma
Tumor arising from star shaped astrocytes in the brainAlong with oligodendrocytes and ependymal cells, serve as glial tissue that
provides support to brainAstrocytomas are a form of glioma
More common in men than womenTend to affect cerebral hemispheres in adultsGraded on a scale from I-IVI-slow growing, non-invasive (more common in children)IV-rapid growing, infiltrative (more common in adults)
Astrocytoma
Grade I Generally non-infiltratingThe most common type is pilocytic astrocytoma, which grows slowly but can grow
largeMost common in the cerebellum, cerebrum, optic nerve pathway and brainstem. Occurs mostly in children and teenagersAccounts for 2% of all brain tumors.
Grade IIUsually infiltrative, but grows slowlyMost common in adults 20-40 years old
Grade IIIInfiltrative and grows more quickly, aka malignant astrocytoma astrocytomaMost common in adults 30-50 years oldAccounts for 4% of all brain tumors.
Grade IV Most aggressive nervous system tumor, aka glioblastomaMost common in adults 50-80Accounts for 23% of all primary brain tumors.
References
Aizawa S, Mitamura Y, Baba T, Hagiwara A, Ogata K, Yamamoto S. 2008. Correlation between visual function and photoreceptor inner/outer segment junction in patients with retinitis pigmentosa. Eye.
Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol 2002; 47: 515-532.
Biousse V, Rucker JC, Vignal C, et al. Anemia and papilledema. Am J Ophthalmol2003;135(4):437-46.
Bunn HF. Approach to the anemias. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:Chap 161.
Centers for Disease Control and Prevention. Recommendations to Prevent and Control Iron Deficiency in the United States. MWR 1998; 47(No. RR-3):25
Chopra KB, Banka NH, Chandalia HB. Bilateral optic nerve infarction following acute systemic hypotension and anemia--a case report. Indian J Ophthalmol1992;40(2):66-9.
Dinn RB, Harris A, Marcus PS. Ocular changes in pregnancy. Obstet Gynecol Surv. 2003;58(2):137-144.
Frith-Terhune AL, Cogswell ME, Khan LK, Will JC, Ramakrishnan U. Iron deficiency anemia: higher prevalence in Mexican American than in non-Hispanic white females in the third National Health and Nutrition Examination Survey, 1988-1994. Am J ClinNutr. 2000 Oct;72(4):963-68
Gerth C, Zawadzki RJ, Werner JS, Héon E. 2008 Retinal morphology in patients with BBS1 and BBS10 related Bardet-Biedl Syndrome evaluated by Fourier-domain optical coherence tomography. Vision Res. 48, 392-9.
Grippo TM, Shihadeh WA, Schargus M, Gramer E, Tello C, Liebmann JM, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17:100-104.
Green J.S. et al. 1989. The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome. NEJM. 321, 002-9.
References
Kacer B, Hattenbach LO, Horle S, et al. Central retinal vein occlusion and nonarteriticischemic optic neuropathy in 2 patients with mild iron deficiency anemia. Ophthalmologica 2001;215(2):128-31.
Kahlon N, Gandhi A, Mondal S, et al. Effect of iron deficiency anemia on audiovisual reaction time in adolescent girls. Indian Journal of Physiology & Pharmacology 2011;55(1):53-9.
Lee KM, Woo SJ, Hwang JM. Differentiation of optic nerve head drusen and optic disc edema with spectral-domain optical coherence tomography. Ophthalmol2011;118:971-977.
Maris Jr. PJG, Mandal AK, Netland PA. Medical therapy of pediatric glaucoma and glaucoma in pregnancy. Ophthalmol Clin N Am. 2005;18:461-468.
Matsuoka Y, Hayasaka S, Yamada K. Incomplete occlusion of central retinal artery in a girl with iron deficiency anemia. Ophthalmologica 1996;210(6):358-60.
Moussalli MA, Sanseau A, Ebner R. Papillary drusen and ocular hypertension. IntOphthalmol 2001;23:275-278.
Onaran Z, Tan FU, Yılmazbaş P, Onaran Y. Bilateral non-arteritic anterior ischemic optic neuropathy following second-trimester spontaneous abortion-related haemorrhage. J Clin Neurosci. 2012 Aug 13.
Sharifian M, Dadkhah-Chimeh M, Einollahi B, Nafar M, Simforoush N, Basiri A, Otukesh H. 2007 Renal transplantation in patients with Bardet-Biedl syndrome. Arch. Iran Med. 10, 339-42
Trethowan BA, Gilliland H, Popov AF, Varadarajan B, Phillips SA, McWhirter L, Ghent R. A case report and brief review of the literature on bilateral retinal infarction following cardiopulmonary bypass for coronary artery bypass grafting. J CardiothoracSurg. 2011; 21(6):154.
http://www.webmd.com/cancer/brain-cancer/astrocytomaVillegas RB, Ilsen PF. Functional vision loss: a diagnosis of exclusion. Optometry
2007;78(10):523-33.
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Grand Round Cases Dina Erickson, OD, FAAO
Outline: Central Retinal Vein Occlusion: Case #1:
• 59 year old Caucasian male presented to the office with visual disturbances after a car accident March 2014
o Post concussion • Findings • Scheduled for a F/U in 1 month for glaucoma F/U & DFE
o Fundus findings. • What is your ddx? • Sent to PCP to for blood work up
o Blood work up results • Returned for a F/U in 1 month.
o Fundus findings o What is your Dx?
• What does the OCT show • What would you do next?
CRVO Review:
• Risk Factors o Age o Systemic
The metabolic syndrome HTN DM Hyperlipidemia HLD Atherosclerosis associated Dz Others Drug Therapy
o Severity of systemic Dz: o Socioeconomic factors
AA 58% increased risk of CRVO compared to non-Hispanic whites.
o Smoking o Ocular Risk factors:
Glaucoma Drug treatments for CME secondary to CRVO:
• What are the treatment options?
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• Anti VEGF therapy • Studies to support the above:
o COPERNICUS 2012 Aflibercept
o GALILEO 2012 Aflibercept
o Cruise 2010 Ranibizumab
o EPSTEIN 2012 Bevacizumab
• Steroid therapy: • Studies to support the above:
o GENEVA 2010 Dexamethasone
o SCORE 2009 Triamcinolone
• How do the above two therapy options compare? • Weaknesses of the studies • What are retina specialists doing? • What may work best for the patient • Further research.
Case #2: Age Related Macular Degeneration:
• A 77 year old Caucasian male presented with the chief complaint of “ white lines in middle of road appear as “sheep jumping”.
• Case history: • Entering VAs
o Funuds exam o TD OCT o DDx
• Referral • FA & PHP • Treatment • Progression and follow ups over the past 4 years
Current AMD Treatment:
• Avastin • Lucentis
o Long term potential side effects • Eyelea
o FDA approval vs clinical experience •
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Case #3 Macular Hole: Case report:
• A 39 year old Caucasian female presented with sudden painless vision loss in her left eye.
• No improvement with refraction • Photos • OCT
Macular hole review:
• Pathophysiology • Stages of macular hole • Risk factors
o Age o Gender o Elevated plasma fibrinogen
• Impact on quality of life • Treatment options:
o Observation o Vitrectomy
Advantages Disadvantages
o Ocriplasmin: Jetrea FDA approved Oct 2012 for the Tx of VMT How it works Patient selection Simulates surgery Clinical efficacy Safety Future potential
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