Summer 2010 - School of Optometry

Transcription

Summer 2010
Volume 13, Numbers 1/2
Assessing the Vision Readiness of Indiana School Children
– An Analysis of P.L. 140-1986
Clinical Diagnosis and Management of Streff Syndrome: A
Case Report
Stickler Syndrome
Bridging a Gap in the Informed Consent Process
Making Small Visual Displays Accessible to People with
Vision Loss
A Description of the Visual Aura in Migraine Aura
Without Headache
Book review: Fixing My Gaze: A Scientist’s Journey into
Seeing Three Dimensions
In This Issue
This is the twenty-third issue of the Indiana Journal of Optometry since its beginning in
1998. A variety of topics are addressed in this 2010 issue. Authors for this issue include
Indiana University School of Optometry faculty members, as well as alumni of the Doctor
of Optometry program, the physiological optics/vision science graduate program, and the
residency program at Indiana University. We hope that you will find it to be of interest.
David A. Goss
Editor
ON THE COVER: The new Atwater Eye Care Center, across the street from the IU School
of Optometry building on the IU Bloomington Campus.
Correspondence and manuscripts submitted for publication should be sent to the Editor: David A.
Goss, School of Optometry, Indiana University, Bloomington, IN 47405 USA (or
dgoss@indiana.edu). Business correspondence should be addressed to the Production Manager:
J. Craig Combs, School of Optometry, Indiana University, Bloomington, IN 47405 USA (or jocombs
@indiana.edu). Address changes or subscription requests should be sent to Sue Gilmore, School
of Optometry, Indiana University, Bloomington, IN 47405 USA (or sgilmore@indiana.edu).
Our appreciation is extended to Essilor of America for
financial support of this publication.
Varilux® is a registered trademark of Essilor International, S.A
Summer 2010
Volume 13, Numbers 1/2
Table of Contents
Indiana University School of
Optometry Administration:
Joseph A. Bonanno, O.D., Ph.D.,
Dean
Clifford W. Brooks, O.D.,
Executive Associate Dean of
Academic Affairs and Student
Administration
Rowan Candy, Ph.D.,
Associate Dean for Research
Sandra L. Pickel, B.G.S., A.S.,
Opt.T.R., Director,
Optician/Technician Program
Cindy Vance,
Director of Student
Administration
Don Lyon, OD, Chief of Clinical
Services
Indiana Journal of Optometry
Editor:
David A. Goss, O.D., Ph.D.
Editorial Board:
Arthur Bradley, Ph.D.
Clifford W. Brooks, O.D.
Daniel R. Gerstman, O.D., M.S.
Victor E. Malinovsky, O.D.
Neil A. Pence, O.D.
Production and Layout
J. Craig Combs, M.H.A.
TABLE OF CONTENTS
Assessing the Vision Readiness of Indiana School Children –
An Analysis of P.L. 140-1986,
Edwin C. Marshall, Richard E. Meetz, and L’erin Garner ... 1
Clinical Diagnosis and Management of Streff Syndrome:
A Case Report, Scott J. Caughell …………………………… 8
Stickler Syndrome, Craig Andrews ……………………...… 12
Bridging a Gap in the Informed Consent Process,
John W. Potter ..….................................................…….… 14
Making Small Visual Displays Accessible to People with
Vision Loss, Mark Uslan .................................................... 16
A Description of the Visual Aura in Migraine Aura Without
Headache, David A. Goss…………………………………... 17
Book review: Fixing My Gaze: A Scientist’s Journey into
Seeing Three Dimensions,
Reviewed by David A. Goss ..................…………………... 19
Statement of Purpose: The Indiana Journal of Optometry is published by the
Indiana University School of Optometry to provide members of the Indiana
Optometric Association, Alumni of the Indiana University School of Optometry, and
other interested persons with information on the research and clinical expertise at
the Indiana University School of Optometry, and on new developments in
optometry/vision care.
The Indiana Journal of Optometry and Indiana University are not responsible for
the opinions and statements of the contributors to this journal. The authors and
Indiana University have taken care that the information and recommendations
contained herein are accurate and compatible with the standards generally
accepted at the time of publication. Nevertheless, it is impossible to ensure that all
the information given is entirely applicable for all circumstances. Indiana
University disclaims any liability, loss, or damage incurred as a consequence,
directly or indirectly, of the use and application of any of the contents of this
journal. This journal is also available on the world wide web at:
http://www.opt.indiana.edu/IndJOpt/home.html
Assessing the Vision Readiness of Indiana
School Children – An Analysis of P.L. 140-1986
Edwin C. Marshall, O.D., M.S., M.P.H., Richard E. Meetz, O.D., M.S.,
L’erin L. Garner, O.D., M.P.H.
BY
Abstract
Background. Vision screening of school children is a widely accepted procedure to detect vision problems that can
interfere with learning. The Indiana Department of Education requires the annual vision screening with the Modified
Clinical Technique (MCT) of all children upon their enrollment in either kindergarten or the first grade.
Methods. Indiana University School of Optometry conducted an analysis of the Indiana State Department of Health’s
statewide school screening data on 36,967 Grade 1 children from 139 of the 294 Indiana school corporations that
submitted data for the 2000-2001 school year to examine differences in referral rate by screening method, the
socioeconomic status of children(SES) screened, and academic performance.
Results. Significant differences were seen when comparing the mean referral rates of school corporations that conduct the
MCT compared to those school corporations that do not conduct the MCT (p = 0.001) and in the rate of referral by median
family income of the children screened (p = 0.050). A median family income of $46,500 was identified as the level at
which the income-specific difference in referral rates ceased to be significant (p = 0.074). A weak non-significant trend (p
= 0.116) was seen comparing the percentage of Grade 1 children referred in 2000-2001 to their percentages of passing both
the English/Language Arts and Mathematics components of the 2002-2003 ISTEP+ exam by SES.
Discussion/Conclusion. Schools using the highly sensitive and specific MCT identified more visually at-risk children
than schools using alternative, less sensitive vision charts techniques, and the percentage of Grade 1 children referred to an
eye care provider was higher for school corporations with lower median family incomes. Although statistically
insignificant, the results trend support that students who fail the vision screening in Grade 1 tend to be more at-risk for
poorer academic performance on standardized testing in Grade 3.
As a state, Indiana is challenged by the health and wellbeing of its residents. Indiana ranks 34th in relative
overall healthiness and 31st in overall child wellbeing.1,2 Indiana falls below the national averages
($60,374 and 18.3% respectively) in median family
income ($57,602) and percent of children (17.3%)
between the ages of 0-17 years living in poverty.3
Among Indiana’s 92 counties, 17 have child poverty
rates equal to 20% or more of the county’s child
population. For the 2007-2008 school year, 31.0% of
public elementary school children in Indiana were
eligible for the free lunch program, indicating a family
income of equal to or less than 130% of the Federal
Poverty Level.4,5 Based on its “Chance-for-Success
Index” of 13 indicators (family income, parent
education, parental employment, linguistic integration,
preschool enrollment, kindergarten enrollment,
elementary reading, middle school mathematics, high
school graduation, postsecondary participation, adult
educational attainment, annual income and steady
employment), Education Week’s Quality Counts 2007
report places Indiana 30th in terms of the probability of
students achieving academic success.6
In Indiana a total of 722,380 nonelderly individuals
are uninsured; of these 113,580 (15.7%) are children.7
Over 535,000 Indiana children are insured by either
Medicaid or by the State Children’s Health Insurance
Program (SCHIP), with enrollment densities for
Medicaid and SCHIP being highest in urban counties
and rural counties respectively.8-10 However,
enrollment in Medicaid or SCHIP does not necessarily
translate into enrollee utilization; it only can be assumed
that enrolled children are properly utilizing their
benefits and receiving appropriate eye and vision care
services in a timely manner. The 2007 National Survey
of Children’s Health revealed that 61.7% of Indiana
children “received care within a medical home” and that
86.3% had “a preventive medical care visit in the past
year; 79.4% had “a preventive dental visit in the past
year.”11
Good vision and ocular health are necessary
components to general well-being, including social,
educational, and economic well-being. The prevalence
of undetected vision problems in preschool children is
estimated to be 5% to 10%, or possibly higher in certain
select populations.12-15 The 2008 population estimates
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indicate that Indiana has 443,089 preschool (0 to 4
years) children and 1,141,592 school age (5 to 17 years)
children.16 Using an estimated prevalence of 10%,
approximately 44,310 preschool and 114,160 school-age
children in Indiana are at risk for undetected and
untreated vision problems and their consequences,
including the increased probability of a low level of
academic readiness due to uncorrected vision problems.
Approximately 3% of preschoolers and school children
(13,293 and 34,248 respectively) are at risk for
amblyopia with 4% (17,724 and 45,664 respectively) at
risk for strabismus and 30% (132,927 preschool and
342,478 school children) at risk for refractive error. In
2002 Vision Service Plan (VSP) partnered with the
Indiana Optometric Association, the Indiana University
School of Optometry, and the Indianapolis Indians
minor league baseball team to conduct one of its seven
city-specific “Kids Get Focused” campaign events of the
summer. The campaign included the vision screening of
local school children with the goal of educating parents
about the need for regular eye examinations and the role
good vision plays in academic and athletic performance.
Of the seven cities in which the campaign was
conducted (Birmingham, AL, Columbus, OH, Grand
Rapids, MI, Indianapolis, IN, Memphis, TN, Portland,
OR, Sacramento, CA), Indianapolis had the third highest
percentage (30%) of children to fail the screening.17
The percentages of failure ranged from a high of 34% in
Columbus to a low of 15% in Sacramento.
Indiana Vision Screening Legislation
Early in the 1980s the State of Indiana recognized that
good vision was essential to the educational success of
its children. The Indiana Superintendent of Public
Instruction further recognized that any program that
would reduce the academic failure rate in Indiana
schools would save the state money. In 1986, the
Indiana General Assembly enacted P.L. 140-1986 to
require the annual vision screening with the Modified
Clinical Technique (MCT) of all children upon their
enrollment in either kindergarten or the first grade, and
the annual screening of visual acuity of all children
enrolled in the third and eighth grades, and all other
school children suspected of having a visual defect.
However, if a school corporation could not find
qualified screeners to conduct the MCT vision
screening, the school corporation could apply to the
Superintendent of Public Instruction for a waiver to
conduct only a Snellen chart screening of visual acuity
at 20 feet. In the absence of an approved waiver or a
written request from an eye care provider that a child not
be tested, the Modified Clinical Technique is to be used
to screen kindergarten and first grade children. The
distance Snellen visual acuity test is to be used for
children enrolled in grades 3 and 8. The local school
system has the discretion to use, on the recommendation
of its professional health advisors, testing equipment
that is equivalent to or more elaborate than the Snellen
chart. It is the responsibility of the school
administration to secure the services of local eye care
providers to conduct parts of the MCT battery and to
make recommendations regarding the need for followup.
Each Indiana public school corporation is required
to submit the School Corporation Vision Screening
Report on the results of the annual vision testing to the
Indiana Department of Education by June 1 of each year
(prior to 2007, the annual reports were to be submitted
to the Indiana Department of Health). Private schools
are not required by law to provide vision screenings or
to submit an annual report. Each annual report is to
include the total number of children screened in grades
K, 1, 3, and 8 along with the actions taken as a result of
the screening (i.e., number referred from positive
findings, number receiving professional treatment,
number who saw a doctor but treatment was not needed,
number of referrals not yet completed and number of
referrals completed from the previous year). The report
also must indicate the screening method and whether a
waiver was received if the MCT was not used. To
increase the efficiency of the reporting process and
starting with the 2000-2001 school year, school nurses
could access and submit the report electronically via the
Indiana State Department of Health web page.
A significant omission of the Indiana vision screening
regulation is the absence of a strong follow-up
provision. The rules do call upon schools to recommend
for further examination students who: 1) are unable to
read the 20/40 line with either eye (unable to read with
each eye the 20/30 line in grade 3, unable to read with
each eye the 20/20 line in grade 8); 2) with one eye can
read a line that is two or more lines higher or lower on
the chart than the line that can be read with the other
eye; 3) are unable to read the 20/30 line at 14 inches
using both eyes; 4) demonstrate a manifest deviation of
any size; 5) demonstrate a latent deviation of 10 prism
diopters of exodeviation; 6) demonstrate a latent
deviation of 8 prism diopters of esodeviation; or 7)
demonstrate a lack of stereoacuity. However, these rules
only provide for the recommendation and not the
.................................................... Indiana Journal of Optometry ... Summer 2010 ... Vol. 13, Nos. 1/2... page 2
requirement of further examination in response to a
failed screening.
The Indiana School Vision Screening Guidelines
states that Indiana’s vision screening law “supports the
concept of local participation and cooperation of . . .
school nurses, teachers, parents and eye care
professionals, as well as interested volunteers, service
clubs and civic groups” for the improved eye care of
Indiana’s children.18 While stating that “we cannot
afford to let any youngster go through the educational
process with vision defects or problems undetected,” Dr.
Suellen Reed, former Indiana Superintendent of Public
Instruction, realized that vision screenings serve only as
an “early warning system” and “should not be confused
with more comprehensive full eye examinations, which
take place in the office of any eye care professional.”18
Assessing the Vision Readiness of Indiana School
Children
In 2001, the Indiana State Department of Health
requested the Indiana University School of Optometry
to conduct an analysis of the statewide school screening
data submitted by the Indiana public schools. The
purpose of the request was to answer a series of key
questions:
• Do any areas of the state show higher than average
referral rates?
• Are there differences in the referral rates between
schools utilizing the MCT and schools utilizing the
Snellen chart only?
• What are the referral rate differences between
schools using different screening techniques?
• What are the referral rate differences between
different socioeconomic groups?
• What are the reasons for the existence of any
differences?
• Are there any factors that could be identified as
indicators of greater need?
• What is the impact of any differences on the
education and productive future of affected students?
• What can be concluded about the screening
program’s success and areas of unmet need?
Although five years of data (2000-2001 to 20042005 school years) were made available for analysis,
only the 2000-2001 school year had a response rate
sufficient for statistical analysis (i.e., greater than 40%).
For the 2000-2001 school year 45.6% of Indiana public
school corporations complied with the reporting
requirement. The sample of Grade 1 students from
which data were extracted represented 41% of the
state’s first grade student enrollment in 2000-2001. The
2000-2001 Grade 1 sample comprised the same cohort
of children analyzed in the 2002-2003 Grade 3 sample.
During the 2000-2001 school year, 36,967 public
school children were screened in 139 (47.3%) of the 294
Indiana public school corporations and, of those
screened, 3,540 (9.6%) were referred for follow-up.
The MCT was used by 125 school corporations; some
other technique was used by 14 school corporations.
The percentage of Grade 1 children referred to an eye
care provider was found to be significantly different (p
= 0.001) between the MCT and non-MCT vision
screening techniques. [Figure 1] Only 9.3% of the
children who failed the state’s vision screening by
whatever method actually had their eyes examined
within the year in which the screening failure occurred.
If the findings from the 2000-2001 cohort of first grade
students are projected to the 2008-2009 cohort of 80,932
first grade students using the calculated sample referral
rates, the MCT would refer 8,983 (11.1%) children for
comprehensive eye examinations, compared to the
projected Snellen E chart referral of 4,613 (5.7%)
children. The difference of 4,370 (5.4%) indicates the
number of children who would not be referred by the
Snellen E chart – children whose vision problems would
go undetected as a result of the under-referral.
The median family income of students enrolled in
the 294 Indiana public school corporations ranged from
a low of
$31,778 to a
high of
$96,747.2
The average
median
family
income for
the school
corporations
in the study
sample was
$50,521,
with 80.4%
of the
school
corporations
comprised
of students
from
families
with median
incomes
between
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$40,000 and $59,999. The rate of referral by income
class was found to be significant (p = 0.050), with a
graduated decrease in the rate of referral as the median
family income increased. [Figure 2] An income of
$46,500 was identified as the level at which the incomespecific difference in referral rates ceased to be
significant (p = 0.074), and 31.9% of the sample
corporations had median family incomes below
$46,500. The school corporations with lower median
family incomes are located in either extremely rural or
metropolitan areas of the state.
Studies have demonstrated that populations with
great income disparity between the rich and poor have
worse health status than populations with economic
homogeneity and high SES.19 Health status can be a
function of several factors, including income, parental
education, parental employment, and health
insurance.20,21 The health status disparity function
within Indiana is not implicit in the results of this study.
However, research supports the premise that children
whose families live in poverty and without health
insurance must overcome greater barriers in access to
health care than children whose families live
comfortably with health insurance.20 It can be
assumed, therefore, that children living under these
conditions need greater access to primary eye care.
The Indiana Statewide Testing for Educational
Progress Plus (ISTEP+) exam is administered as a
measure of academic achievement to Indiana school
children each year beginning in Grade 3 and continuing
through Grade 10. The test assesses English/Language
Arts and Mathematics in grades 3 through 10 and
Science in grades 5 and 7. The mean pass rate for the
combined English/Language Arts and Mathematics
components of the 2002-2003 ISTEP+ for Grade 3
students was 59.9 – 72% of Grade 3 public school
students passed English/ Language Arts and 66% passed
Mathematics. The research literature contains evidence
of significant associations between MCT failure and
decreased reading skill in children. 22 However, the
results of this study found a non-significant (p = 0.116)
relationship between the percent of Grade 1 children
referred to an eye care provider in 2000-2001 and the
percent of the same cohort subsequently passing both
the English/Language Arts and Mathematics
components of 2002-2003 ISTEP+ exam two years
later. The greatest percentage of students referred for an
eye examination came from the population of students
with below average ISTEP+ results.
[Figure 3] The downward trend in ISTEP+ performance
among students most likely to have been referred is
2000-2001
implies an
increased risk
of poorer
ISTEP+
performance
in Grade 3
among
students who
fail the vision
screening in
Grade 1.
[Figure 4]
The
school
corporations
can be
identified as
being
located in
one of six
geographic
regions
(Northwest, Northeast, East Central, West Central,
Southwest, and Southeast). When analyzing the
regional percentages of Grade 1 children referred to an
eye care provider in 2000-2001 by the 2002-2003
regional below average/above average Grade 3 ISTEP+
exam performance, the results were found to be
................................................... Indiana Journal of Optometry ... Summer 2010 ... Vol. 13, Nos. 1/2... page 4
insignificant
(Northwest: p =
0.417; Northeast: p
= 0.177; East
Central: p = 0.437;
West Central: p =
0.114; Southwest: p
= 0.237; and
Southeast: p =
0.156). [Table 1]
However, within
every region of the
state, except one
(East Central),
students whose
performance was
below average on the
ISTEP+ exam had a
higher rate of vision
screening failure and
referral for a
comprehensive eye
examination than
students whose
performance was
above the ISTEP+
state average.
[Figure 5] Students
enrolled in the East
Central Indiana
schools were found
to have a higher
vision screening
referral rate among
students with above
average ISTEP+
results (10.0%)
compared to students
with below average
results (8.3%).
These findings could
imply that the East
Central grade 1
students were more
likely to have
received the
appropriate followup for their referral
two years earlier and
no longer manifested
an uncorrected
vision problem in Grade 3. The East Central region
includes an economically diverse mixture of school
corporations ranging from some with median family
incomes of less that $40,000 to one with a median
family income in excess of $90,000. [Figures 6-7]
Approximately 58% of the East Central Indiana school
corporations are comprised of students from families
with median incomes of $50,000 or higher, well above
the $46,500 income threshold for finding a referral rate
difference by income. Therefore, the findings for the
East Central Indiana schools also could imply that,
because of their higher SES position relative to other
regional schools, they have greater resources to ensure
that the more diagnostically sensitive MCT test is
conducted – potentially yielding higher rates of referral
(true positives) than the less sensitive Snellen chart and
positioning students to be more academically ready
through the early intervention of proper vision care.
A review of the sample and mean referral rates for
the schools in the Southwest region shows a
contradiction in ISTEP+ exam performance with the
sample referral rate (10.2%) being higher for students
with above average performance and the mean referral
rate (15.0%) being higher for students with below
average performance. The contradiction may be due, at
least partially, to the “weighting” effect on the small
screening populations of primarily rural school districts
when grouped with the screening populations of larger
metropolitan school corporations. In addition to the
sample size differences existent within the region,
differences also exist with respect to the sensitivity of
the screening measures adopted by large and small
schools.
The greatest intra-regional referral rate disparities
between below average and above average ISTEP+
performance were found in the Southeast and Northeast
Indiana school populations. The magnitude of the
difference in the below average/above average mean
referral rates was 4.1% for the Southeast regional
schools and 3.8% for the Northeast regional schools.
[Figure 8] When compared to children with above
average performance on the Grade 3 ISTEP+ exam, the
regional findings indicate that children in the Southwest
(15.0%), West Central (13.2%), and Northeast (13.1%)
school corporations had the highest likelihood (although
not to the level of statistical significance) of performing
poorly on the ISTEP+ exam when failing the Grade 1
vision screening two years earlier. Nonetheless, the
mean referral rates for students performing below
average were in the double digit range for all regions,
except the East Central region (8.3%). As discussed
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earlier, students in the
East Central Indiana
schools demonstrated
a higher vision
screening referral rate
for students with
above average
ISTEP+ results
compared to students
with below average
ISTEP+ results.
However, the East
Central region also
demonstrated the
smallest intra-regional
referral rate disparity (1.7%) between the below average
and above average ISTEP+ performance population.
These findings indicate that children who performed
below the state average on the ISTEP+ exams were
more likely to have failed the vision screening exam in
Grade 1, placing increased emphasis on the importance
of follow-up care for students who do not pass the
vision screening.
Unfortunately, the data provided by the school
corporations were not as complete and robust as desired.
Consequently, the low reliability of the data set
contributes to results that are of insufficient strength to
conclude a significant association between referral rate
and academic performance. Although school personnel
are required to report on the Vision Screening Report
Form the number of referrals completed and not
completed, it is unknown if students initially referred for
an eye examination actually received professional eye
care services during the two year period between when
the vision screening was held in Grade 1 and when the
same student cohort took the ISTEP+ exam in Grade 3.
Future efforts should investigate in greater detail the
relationship between referral, follow-up examination,
and academic performance on the ISTEP+. Students
referred to an eye care provider should be followed
administratively to ascertain the completion of a
comprehensive follow-up eye examination, and the
differences in ISTEP+ performance between students
who received professional care against those students
who did not receive professional care should be
examined with greater understanding and accuracy.
References
1. United Health Foundation. America’s Health
Rankings™ 2008: A Call to Action for Individuals &
Their Communities. 2008 Edition. Available at:
http://www.americashealthrankings.org/2008/index.html
. Accessed August 28, 2009.
2. The Annie E. Casey Foundation. Kids Count Data
Center. Available at:
http://datacenter.kidscount.org/Default.aspx. Accessed
August 28, 2009.
3. U.S. Census Bureau. 2005-2007 American
Community Survey 3-Year Estimates. Available at:
http://factfinder.census.gov. Accessed August 28, 2009.
4. Indiana Department of Education. Indiana K – 12
Education Data. Available at:
http://www.doe.in.gov/data/. Accessed August 28, 2009.
5. United States Department of Agriculture. National
School Lunch Program. Available at:
http://www.fns.usda.gov/cnd/Lunch/. Accessed August
28, 2009.
6. Education Week. Quality Counts 2007: From
Cradle to Career, Connecting American Education From
Birth to Adulthood. Available at:
http://www.edweek.org/ew/toc/2007/01/04/index.html.
Accessed January 30, 2007.
7. The Kaiser Family Foundation. Statehealthfacts.org.
Available at: http://www.statehealthfacts.org/. Accessed
May 24, 2007.
8. EP&P Consulting, Inc. Indiana’s Children’s Health
Insurance Program Annual Evaluation Report. April 1,
2006. Available at:
http://www.in.gov/fssa/programs/chip/pdf/chipannualre
port2006.pdf. Accessed September 19, 2006.
9. The Kaiser Commission on Medicaid and the
Uninsured. Medicaid Enrollment in 50 States:
December 2002 Data Update. Menlo Park, CA: The
Henry J. Kaiser Family Foundation, 2003. Available at:
http://www.kff.org/medicaid/upload/MedicaidEnrollment-in-50-States-December-2002-Update.pdf.
Accessed September 19, 2006.
10. The Kaiser Commission on Medicaid and the
Uninsured. SCHIP Enrollment in 50 States: December
2004 Data Update. Menlo Park, CA: The Henry J.
Kaiser Family Foundation, 2005. Available at:
http://www.kff.org/medicaid/upload/7348.pdf. Accessed
September 19, 2006.
11. The Child and Adolescent Health Measurement
Initiative. National Survey of Children’s Health, 2007.
Data Resource Center. Available at:
http://www.nschdata.org. Accessed August 28, 2009.
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12. U.S. Preventive Services Task Force. Guide to
Clinical Preventive Services. 2nd Edition. Washington,
DC: U.S. Department of Health and Human Services,
Office of Disease Prevention and Health Promotion,
1996.
13. Scheiman M, Gallaway M, Coulter R, et al.
Prevalence of vision and ocular disease conditions in a
clinical pediatric population. J Am Optom Assoc 1996;
67:193-202.
14. Ciner EB, Schmidt PP, Orel-Bixler D, et al. Vision
screening of preschool children: evaluating the past,
looking toward the future. Optom Vis Sci 1998; 75:571584.
15. The Vision in Preschoolers Study Group.
Comparison of preschool vision screening tests as
administered by licensed eye care professionals in the
Vision in Preschoolers Study. Ophthalmology 2004;
111:637-650.
16. U.S. Census Bureau. Population Estimates.
Available at:
http://www.census.gov/popest/estimates.html. Accessed
August 28, 2009.
17. Vision Service Plan. VSP Program Finds 28% of
Children Have Vision-Related Problems. Press Release,
August 26, 2002.
18. Indiana Optometric Association. Indiana School
Vision Screening Guidelines. Third Edition.
Indianapolis, IN: Indiana Optometric Association, 2000.
19. Wilkinson RG. Comment: income, inequality, and
social cohesion. Am J Public Health 1997; 87:1504-6.
20. Newacheck PW, Hughes DC, Stoddard J.
Children’s access to primary care: differences by race,
income, and insurance status. Pediatrics 1996; 97: 2632.
21. Shi L, Starfield B, Kennedy B, Kawachi I. Income,
inequality, primary care, and health indicators. J Fam
Pract 1999; 48: 275-284.
22. Kulp MT, Schmidt PP. Visual predictors of reading
performance in kindergarten and first grade children.
Optom Vis Sci 1996; 73:255-262.
Edwin Marshall is Vice President for Diversity, Equity, and Multicultural Affairs and Professor of Optometry at
Indiana University. He is also Adjunct Professor of Public Health in the Indiana University School of Medicine.
He received O.D. (1971) and M.S. (1979) degrees from Indiana University and an M.P.H. from University of
North Carolina in 1982. Richard Meetz is Clinical Associate Professor of Optometry at Indiana University. He
received his O.D. degree from Indiana University in 1976 and an M.S. in biostatistics from the University of
Michigan in 1988. L’erin Garner received her M.P.H. degree from Indiana University in 2005 and is a 2009
graduate of the Indiana University School of Optometry. She is in practice with Midwest Eye Consultants in
Marion, Indiana.
Page 7 ... Vol. 13, Nos. 1/2 ... Summer 2010 ... Indiana Journal of Optometry ......................................................
Clincal Diagnosis and Management of Streff
Syndrome: A Case Report
BY
Scott J. Caughell, O.D.
Abstract: Streff syndrome is a controversial clinical entity associated with multiple thoughts on treatment and
etiology. Some authors suggest that this could be a common clinical presentation that is often misdiagnosed or
ignored. Patients with these symptoms may undergo unnecessary medical or psychological testing. Presenting
symptoms may include blurred vision, headaches, difficulty with reading, tunnel vision, and trouble judging
distances. Because the symptoms can be significant and greatly affect activities of daily living, eye care providers
should be familiar with the signs and symptoms of Streff syndrome and know how to initiate treatment or referral.
A typical case of Streff syndrome with treatment and background of the syndrome is presented here.
Key Words: Streff syndrome, juvenile amblyopia, non-malingering syndrome, early adaptive syndrome,
hysterical amblyopia
Introduction
Streff syndrome, also known as juvenile bilateral
functional amblyopia, non-malingering syndrome, and
early adaptive syndrome, is a bilateral functional loss of
vision with a relatively rapid onset. This disorder is
most common in the school age population and tends to
affect girls more than boys 2:1, although it has been
reported in adults.1 Age of onset appears to peak
between 7 and 18 years old.2 A study in Finland
showed that of 2280 children out of 14,000 that were
referred for an ophthalmological exam, 1.75% exhibited
“psychogenic amblyopia.”3 Most others place the
prevalence at around 1% of cases.2 Streff syndrome
patients exhibit decreases in visual acuity at both
distance and near. This decrease in acuity presents with
no signs of organic pathology and little if any refractive
error (typically between plano and 1.00 diopter of
hyperopia). Many believe the syndrome is an entity
distinct from hysterical amblyopia and visual
convergence disorders, although they are somewhat
similar. In the case of Streff syndrome, it appears that
visual stress is a significant component that can be
amplified by other emotionally or physically stressful
situations or events.
Several authors have created diagnostic lists for the
diagnosis of Streff syndrome. Streff’s introduction to
the “non-malingering syndrome” in 1961 listed three
specific criteria that were later expanded upon by
others.4 The original three were: (1) reduced distance
acuity of 20/25 and worse, (2) refractive posture of
plano to +1.00 diopters, and (3) no change in distance
acuity with corrective lenses. Kowalski2 developed a
more expansive set of criteria to better help the clinician
identify these cases. This author’s experiences (n=4)
have all involved young girls under 15 years old with
decreased visual acuity (worse than 20/40) distance and
near OD/OS/OU, low hyperopic refractive states of
around 0.50 diopters, decreased Randot stereopsis,
constricted confrontational fields, color vision deficits,
and reported stressful events. It is this author’s opinion
that the final diagnosis is confirmed when treatment is
successful.
Case Report
Patient EW, a 10 year old white female presented to
our office with complaints of near blur and frequent
severe headaches associated with reading. The
frequency of the headaches was “almost daily” and they
were located around the eyes mostly in the afternoons.
She never woke up with headaches or was awakened by
them. EW had noticed the blur and headaches for
approximately two months. EW’s medical history was
unremarkable except for an appendectomy two years
prior. No medications or OTC products were being
administered.
Entering unaided acuities were 20/40+2 OD and
20/30 OS at six meters and 20/60 OD/OS at 40 cm.
Pupils were equal and reactive with no afferent papillary
defect, versions were smooth and full, cover test showed
ortho posture distance and near, and the near point of
convergence was at the nose. Monocular confrontation
fields were significantly constricted OD and OS.
Ishihara color recognition was reduced with EW
correctly identifying 2/10 OD and 4/10 OS after
significant effort. Near point of accommodation was
greater than 20 cm or worse than 5 diopters. Near
retinoscopy with the monocular estimation method was
+0.50 OD/OS and the patient had zero Randot
stereopsis. Worth dot test showed the normal response
of 4 lights.
Initial non-cycloplegic retinoscopy was -0.250.75x005 OD, and -0.25-0.50x016 OS with no
improvement in visual acuity. At this point, I was
beginning to suspect a possible case of Streff Syndrome,
...............................................................Indiana Journal of Optometry ... Summer 2010 ... Vol. 13, Nos. 1/2 ... page 8
so I trial framed +0.50 lenses OU. The patient
immediately commented that her vision seemed much
better. Refraction after 20 minutes of installation of one
drop of 1% cyclopentolate OU was +0.25-0.25x002
20/40+ OD and pl-0.25x030 OS 20/30 OS. Thus the
refractive status did not explain the decreased visual
acuities at this time and an ocular health assessment was
required to rule out organic etiologies.
Prior to instillation of cyclopentolate, anterior ocular
health was assessed by slit lamp biomicroscopy. All
structures including the lids, lashes, tear film, cornea,
conjunctiva, anterior chamber and aqueous were
unremarkable. Dilated fundus examination showed
clear lenses OU, clear vitreous, and normal retinal
health. The disc margins were clear and distinct with no
signs of papilledema. There were no signs of macular
disruption or graininess.
At the conclusion of the exam I had the mother join
me outside the exam room and asked her if EW was
undergoing any new stressful events. She sadly
admitted that EW’s father and she were going through a
divorce for the past few months and that it has been a
very stressful living situation for EW. Though such an
emotional event may not be present in all cases it helped
support my diagnosis of Streff syndrome.
To relieve the concomitant near point stress, EW
was prescribed +0.50 DS / 0.75 prism diopters base in
OD/OS. There is significant clinical evidence that low
plus is very effective in treating Streff syndrome.4,5
EW was scheduled to return in two days for automated
perimetry to further reassure the parents and myself that
no other neurological etiology for the headaches was
present.
Visit #2
EW presented two days after the initial examination
for visual field testing to rule out neurological defects.
A 30 degree 40 point threshold exam was completed
OD/OS. The results show only a few sporadic
reductions in sensitivity. It was assessed as not
consistent with any organic neurological problems and
EW was scheduled to return for a progress report.
Visit #3
EW returned to the clinic in three weeks for a
progress report. EW stated that she was “doing much
better with her glasses on” and that she had not
experienced a single headache. Visual acuities with the
low plus/prism spectacle prescription were 20/30
OD/OS at distance and 20/40 OD/OS at near.
Confrontation fields had returned to normal and EW
scored much better on the Ishihara color plates correctly
identifying 7/10 OD and 8/10 OS. She was assessed as
having improving Streff syndrome and was scheduled to
return in two months for a progress report.
Visit #4
EW returned for a progress report in two months
stating that the headaches had not returned and her
vision seemed even more improved. Visual acuity at 6
meters without the spectacle Rx was now 20/25 OD/OS
and 20/30 at near with the prescribed Rx. Ishihara color
testing now showed a normal 10/10 response OD/OS
with the correction at near. EW was instructed to return
for a second visual field test and final progress report.
Visit #5
At the final visit, one month after the previous visit,
EW reported that her symptoms were still gone.
Interestingly, since the symptoms had subsided she tried
to go without her Rx for approximately a week. During
this time the patient and her mother reported that the
headaches returned and once again were gone after she
resumed spectacle wear. Visual acuities at 6 meters
without Rx were 20/25 OD/OS and she attained 20/25
OU at 40 cm with the correction on. Confrontation
fields were full to examiner’s fields, and near point of
accommodation was now age normal at 6 cm or
approximately 15 diopters of accommodation with the
Rx on. A final threshold field was still unremarkable.
EW and her parents were instructed to return in 6
months to re-evaluate her visual status. She was to
continue full time wear of the Rx and return to the office
if the symptoms returned.
Visit #6
EW returned five months later for a final progress
evaluation. Headaches and other symptoms were still
extinguished. She noted having to use the glasses less
often at this point in time and primarily used them for
near tasks. Unaided visual acuities were 20/20 OD,
20/25 OS. OS was correctable to 20/20 with a refractive
error of -0.50. NPA was age normal at 6 cm. Ishihara
color plates were all correctly identified OD and OS.
Confrontation fields were also full as was the final
automated perimetry.
At this point I deemed EW successfully treated
functionally and symptomatically. She was educated to
return in six months for her annual exam and to let me
know if the symptoms returned before that time.
Discussion
EW represents a classic case of Streff syndrome.
She was a school age female with recent onset of
symptoms that coincided with an emotionally stressful
event. The symptoms of near blur and headaches seem
to be prevalent with the Syndrome.5,6 The clinician
must be careful to rule out other pathological etiologies
of the presenting symptoms and findings.
Differential diagnoses to be considered in these
cases may include the following: (1) Stargardt’s disease,
(2) hysterical ambylopia / visual convergence disorder,
(3) latent hyperopia, (4) aneurysm or mass impinging on
the visual pathway or cortex, (5) malingering, or (6)
optic neuritis.
Stargardt’s disease and Streff syndrome can both
present with bilateral vision loss.7 Stargardt’s disease is
a recessively inherited bilateral macular dystrophy that
Page 9 ... Vol. 13, Nos. 1/2 ... Summer 2010 ... Indiana Journal of Optometry ..........................................
usually affects patients of the same age group. While
Streff syndrome affects females more than males,
Stargardt’s disease has no gender predilection. The
significant differences are that patients with Stargardt’s
disease have normal accommodation, confrontation
fields (perimetry might show central scotoma), and don’t
commonly report headaches. More advanced cases of
Stargardt’s disease show depigmented maculae with a
“beaten metal appearance” and surrounding yellow
flecks termed fundus flavimaculatus. Flourescein
angiography and photostress, while normal in Streff
cases, can be positive in Stargardt’s disease.
Hysterical amblyopia and convergence disorders are
sometimes lumped together with Streff syndrome.8
Further investigation shows that there is a distinct
difference. Patients with hysterical amblyopia have a
primary psychological etiology with secondary visual
symptoms. A good history will usually point to this
diagnosis which is best treated psychologically. For this
explanation it is important to differentiate between stress
and significant psychosis.5
Latent hyperopia can display bilateral reduced
distance and near visual acuity, headaches, and difficulty
with near work. Standard non-cycloplegic distance
retinoscopy and manifest refraction may not reveal the
latent hyperopia at first glance. Careful refraction with
the Humphriss blur technique or cycloplegic refraction
can expose the hyperopia and yield normal distance
visual acuity. Color vision, stereopsis, and confrontation
fields will also be normal in cases of latent hyperopia or
accommodative spasm.
Cranial space occupying lesions and aneurysms can
also cause headaches and blurred vision. These cases
may also show normal refractive posture. Despite the
fact that these etiologies are rare in young patients visual
fields would not show 360 degree constriction and
accommodation would not be affected. Threshold visual
fields would also be more characteristic with identifiable
quadrantanopsias, heteronopsias, and respects to the
midlines. A space occupying lesion may also
demonstrate papilledema or optic atrophy. The
headaches in these cases would also not correspond to
near tasks or could occur at night or early in the
morning. If a patient does not quickly respond to
optometric therapy in suspected Streff syndrome cases, a
CT or MRI would be prudent. This is the most
significant differential diagnosis.
Malingering can cause any number visual signs or
symptoms. The clinician usually can reveal malingering
in the younger age groups without difficulty.
Malingering is actually considered quite rare and usually
presents monocularly.9
Optic neuritis usually presents in a slightly advanced
age group of 18-45 years.10 Optic neuritis (ON) may
occur bilaterally, but most commonly is seen
monocularly. Streff syndrome patients also do not
exhibit pain with ocular motility as do 90% of optic
neuritis cases.10 A relative afferent papillary defect
(RAPD) may also present in ON and swollen discs or
papillitis may be evident in younger optic neuritis
patients.
Once a strong case for Streff syndrome has been
made, a treatment course must be initiated. Unlike cases
of hysterical amblyopia which may be best treated
psychologically, Streff syndrome cases are best treated
optometrically. Optometric treatment usually involves
stress-relieving low plus lenses with or without
optometric vision therapy. Streff showed that low plus
or bifocal lenses were significantly more effective than
placebo and the effect was evident on EOG.4,5,11
Some other optometric colleagues believe that no
treatment is necessary,12 as do many ophthalmologists.9
Since low plus lenses are economically practical and
Streff and others proved their effectiveness, it appears an
excellent place to start.13
It seems that the low power plus lens is an effective
means to break the stress-induced disconnect between
the ambient and focal visual processing streams. In this
case, the patient had a very positive response to the low
plus therapy. If some of the patient’s symptoms did not
subside, vision therapy would have been initiated.
Vision therapy would have included standard
accommodative and vergence work and later integrating
central and peripheral visual skills.
The etiology of Streff syndrome seems to be related
to a present visual stress that becomes magnified by
other physical or emotional stresses. Examples may be
parental divorce, death of a family member, illness,
problems with other children, or school stress.8 The
major mediators of the stress response in the autonomic
nervous system involves the antagonistic interaction
between the sympathetic and parasympathetic response.
The stress response in Streff syndrome appears to trigger
the visual system to get stuck or disorganized in the
processing of focal and ambient visual information. You
could think of this as a central peripheral organization
where the ambient system determines the space at large
(ground) and the focal system processes the details
(figure).14
Trevarthen15 explained the focal visual system
(parvo-cellular) as slower and answering the question:
What is it? Here information is sent to the cerebral
cortex from the foveal area. The ambient vision system
is the big picture, responds quickly, and puts the content
in perspective. Input from posture, balance, and
movement is combined after fibers from the retina
outside of the fovea are sent through the superior
colliculus in the midbrain. This also explains why the
magnocellular pathway assessed with frequency
doubling technology may aid diagnosis.16 Streff
syndrome patients are thought to use focal vision to
compensate for a functional loss of the ambient system
................................................. Indiana Journal of Optometry ... Summer 2010 ... Vol. 13, Nos. 1/2... page 10
thus showing the collapsed fields and loss of visual
function due to the fact that the slower focal system is
overwhelmed. Thus in a way you could functionally
consider both systems as partially inhibited; explaining
the visual acuity, decreased color sensitivity,17
constricted confrontation fields, and loss of fine
orientation (stereopsis).
Conclusion
Streff syndrome is an interesting clinical entity that
must be diagnosed carefully. Organic causes of
binocular vision loss must be ruled out before beginning
treatment. A clinician can greatly help these patients
and avoid expensive and stressful medical testing by
correctly identifying the etiology of the vision loss and
other symptoms. There is very little, if any, mention of
psychological treatment for these patients so it seems
prudent to treat optometrically. The patient and parents
should be reassured that outcomes are usually very good
and that it may take a little time to “get back to normal.”
It is often also helpful to identify the stressors in the
patient’s life in order to reduce that impact as well.
Most patients never have a return of the syndrome, but if
the visual and physical/emotional stress is not corrected
or returns there have been a few cases of recurrence.18
Streff syndrome is a reasonable diagnosis to keep in the
back of your mind when a younger patient comes in
with bilateral functional vision loss.
References
1. Streff JW. A visual syndrome-a review. Eastern
Seaboard Conference on Visual Training, Washington,
DC, 1969:84-88.
2. Kowalski PM. Streff syndrome: a retrospective study
of patterns in clinical examination. J Optom Vis Dev
1994;25:29-32.
3. Mantyjarvi MI. The amblyopic schoolgirl syndrome. J
Ped Ophthalmol Strab 1981; 18:30-33.
4. Streff JW. Juvenile bilateral functional amblyopia: the
Streff syndrome. Behav Aspects Vis Care 2001; 42
(1):1-28.
5. Scott C. Streff syndrome and emotional factors – the
effect of lens therapy. J Optom Vis Dev 1994; 25:101106.
6. Gilman GP. Optometric or psychological problem? J
Am Optom Assoc 1981; 52:609-610.
7. Bruce AS, Swann PG, Livanes A. Psychogenic
amblyopia and Stargardt’s disease – the differential
diagnosis. Clin Exp Optom 1993; 76:61-66.
8. Erickson GB, Griffin JR, Kurihara JI. Streff syndrome
– a literature review. J Optom Vis Dev 1994; 25:64-69.
9. Sletteberg O, Bertelsen T, Hovding G. The prognosis
of patients with hysterical visual impairment. Acta
Ophthalmol 1989; 67:159-163.
10. Ehlers JP, Shah CP. The Wills Eye Manual.
Boston: Lippincott Williams Wilkins 2008.
11. Harris P. Visual evoked potentials as an aid in
diagnosis of a case of Streff nonmalingering syndrome.
J Optom Vis Dev 1994; 25:97-100.
12. Flax N. The nonmalingering syndrome revisited. J
Optom Vis Dev 1994; 25:79-83.
13. Birnbaum MH. Optometric Management of
Nearpoint Vision Disorders. Stoneham: ButterworthHeinemann, 1993:193-196.
14. Forrest EB. Stress and Vision. Santa Ana:
Optometric Extension Program, 1988.
15. Trevarthen CB. Two vision mechanisms in primates.
Psychol Rev 1968;31:299-337.
16. Patel N. The use of frequency doubling techonology
to determine magnocellular pathway deficiencies. J
Behav Optom 2004; 15:31-36.
17. Schwartz I. Color deficiency in the nonmalingering
syndrome. J Optom Vis Dev 1994; 25:84.
18. Harris P. The non-malingering syndrome – catching
it twice. J Optom Vis Dev 1999; 30:142-149.
Scott Caughell practices in Warsaw, Indiana. After
graduating from the Indiana University School of
Optometry, he completed the Pediatrics and Binocular
Vision residency at IU. He can be reached at
drc@warsaweye.com. This case report is one of the
case reports he submitted for his Fellowship in the
American Academy of Optometry.
Page 11 ... Vol. 13, Nos. 1/2 ... Summer 2010 ... Indiana Journal of Optometry ........................................
Stickler Syndrome
BY
Craig Andrews, O.D.
The most common cause of childhood retinal
detachments is a syndrome most optometrists have
never heard of--Stickler syndrome. I think that it is
important to make optometrists aware so that vision loss
may be prevented. Stickler syndrome was named after
the German born pediatrician who put the pieces of the
puzzle together in 1965. He originally called it
“hereditary progressive arthro-ophthalmopathy” because
he associated severe sight deterioration with joint
changes in several generations of one family.1 I think
you can see why his name is now used as the label.
Prominent points concerning Stickler syndrome are
the following:
1. It is a genetic autosomal dominant disorder caused by
a collagen gene mutation.
2. It is the leading cause of retinal detachment in
children and the most common cause of inherited
rhegmatogenous retinal detachment.
3. It may possibly be the most common connective
tissue disorder in North America and Europe but is
frequently undiagnosed or misdiagnosed.
4. It is a progressive disorder with incomplete
penetrance, varied expression, and severity affects not
only the eyes, but also causes auditory, skeletal and/or
craniofacial abnormalities.
5. It is often confused with other syndromes such as
Wagner, Marshall, or Marfan syndromes.
6. It does not affect lifespan or mental capacity.
Three gene mutation locations are currently
known— Stickler Type I (COL2A1), Stickler Type II
(COL11A1) and Stickler Type III (COL11A2). Types I
and II have distinctive ocular characteristics, with Type
III affecting joints and hearing, but not vision. In
addition, there is at least one other type of Stickler
syndrome with an unidentified gene mutation location.
The prevalence of Stickler syndrome is 1 in 7,500.
Early identification is critical, especially due to the high
instance of retinal detachment in children (20% before
age 10 years2), and the inability of children to identify
and verbalize symptoms.
Stickler syndrome may be diagnosed by either
genetic testing or diagnostic criteria. The former is
complicated by the genetic heterogeneity of the
mutation. The diagnostic criteria evaluate characteristic
ocular, auditory and skeletal abnormalities, molecular
data, and family history.3 Typical facial features
include flat cheeks, flat nasal bridge, small upper jaw,
small lower jaw, and palate abnormalities.1 Figures 1-4
illustrate the typical facial features.
Common ocular features of
Stickler syndrome include:
high myopia (–5 to –18 diopters)
often present at birth, spontaneous
retinal detachment, usually a giant
tear, congenital abnormality of the
vitreous, pre-senile cataracts
(wedge or fleck), open-angle
glaucoma (less common),
chorioretinal degeneration—
lattice formation, holes, tears, and
Figure 1. Facial features
strabismus/astigmatism.
typical of Stickler syndrome.
Patients with Stickler
Photo provided by Stickler
syndrome are likely to have the
Involved People.
following needs related to eye
care: corrective lenses, frequent
(at least annual) eye exams,
including “evaluation under
anesthesia” for infants and young
children, prophylactic laser or
cryo treatment,
regular eye pressure readings and
education of the patient as to
normal range, and avoidance of
contact sports.
Prophylactic cryotherapy may
reduce the risk of retinal
detachment.4 Prophylactic
treatment is recommended early, Figure 2. Facial features
because Stickler syndrome infants typical of Stickler syndrome.
have been known to experience a Involved People.
giant tear by 18 months of age.
Further patient and clinician
information is available from
Stickler Involved People (SIP), a
not-for-profit volunteer
organization with a mission to
support those affected by the
syndrome. SIP also strives to
raise the awareness of health care
providers and increase their
knowledge about this disorder. To
that end, SIP sponsors an annual
three-day conference with
physician speakers who discuss the typical of Stickler syndrome.
latest knowledge and treatment of Photo provided by Stickler
Involved People.
the syndrome. Information,
including a brochure, a slide
presentation, reference lists, and instructions to obtain a
30-minute DVD, can be found at the Stickler Involved
................................................. Indiana Journal of Optometry ... Summer 2010 ... Vol. 13, Nos. 1/2... page 12
People website.5
As optometrists, we
need to be aware and on
the lookout for this sight
threatening disease. It
is not a well known
disease, so we also may
be able to educate our
local ophthalmologists
and pediatricians.
typical of Stickler syndrome.
Involved People.
References
1. Houchin B. About Stickler Syndrome.
http://www.sticklers.org/sip2/content/view/16/17/, 2007.
Accessed April 12, 2010.
2. Stickler GB, Hughes W, Houchin P. Clinical features
of hereditary progressive arthro-ophthalmopathy
(Stickler syndrome): a survey. Genet Med 2001;3:192196.
3. Rose PS, Levy HP, Liberfarb RM, Davis J, et al.
Stickler syndrome: clinical characteristics and diagnostic
criteria. Am J Med Genet 2005;138A:199-207.
4. Ang A, Poulson AV, Goodburn SF, Richards AJ, Scott
JD, Snead MP. Retinal detachment and prophylaxis in
type 1 Stickler syndrome. Ophthalmol 2008;115:164168.
5. http://www.sticklers.org/sip2/.
Craig Andrews graduated from the Indiana University
School of Optometry in 1979. He started the Salem Eye
Clinic in Salem, Illinois, which is now a five doctor
office. He is also President of Bernell Corporation
(Mishawaka, Indiana; 800-348-2225). He and his
optometry school classmate, Charlie Shearer, of
Mishawaka, resurrected the company in 1997 when a
bank was going to close its doors. He can be reached at
bernell@charter.net.
Page 13... Vol. 13, Nos. 1/2 ... Summer 2010 ... Indiana Journal of Optometry ........................................
Bridging a Gap in the Informed Consent
Process
BY JOHN W. POTTER, O.D., M.A.
It is everyone’s role to participate in the informed
consent process. By way of background, the legal
doctrine of informed consent has been one of the
primary instruments used by legislatures and courts to
evaluate the scope and nature of information doctors
must document and disclose to patients undergoing
treatment. The failure of a doctor to disclose all the
risks associated with treatment can be a breach of
professional duty and, perhaps, a negligent act.1
Furthermore, juridical interpretations of the
responsibilities of doctors in the informed consent
process influence the daily practice of physicians and
surgeons, too. Clearly, the doctrine of informed consent
is evolving, and most experts in the field suggest that
physicians and surgeons constantly monitor changes in
the approach and elaboration of the informed consent,
which sounds ideal but impractical for most physicians
and surgeons.2 Furthermore, it is not perfectly clear
what role a referring optometrist has in the informed
consent process either. And, it is no comfort to learn
that the history of the informed consent in research is a
direct result of the tragic events revealed at the
Nuremberg trials or through the Tuskegee research.3
Finally, in the era of greater cost consciousness in health
care, the time and objective financial costs of the
process are relevant, also.4 It is enough to make the
strongest minds in optometry pause.
Having spent the last six years of my professional
life devoted to disputes and conflict resulting from
refractive surgery, I have begun to see the informed
consent process differently. I don’t pretend to offer
legal advice about the informed consent process, but I
can offer you some insight into a dimension that is
rarely considered. My hope is that it may offer you
insight to better help your patients. That dimension is
“the middle ground” in reducing risk of a dispute or
conflict concerning the informed consent process that
most lawyers, legislators, jury members, and few
physicians and surgeons interact with on any regular
basis.
So, let me begin to move directly into the middle
ground by making this observation. I have rarely
spoken with a patient in a dispute or conflict who has
not said, “I was not adequately informed.” Nor, have I
spoken with a patient’s surgeon who has not said, “I am
certain I provided informed consent, and I have the
signed and properly executed document in the patient’s
record to prove it.” How does this gap occur and what is
the context? Simply put, the culture and values of the
patient and doctor may be quite different. Patients are
understandably concerned about their individual needs
and well-being. Doctors frequently take a more “macroapproach.” Informed consent documents reflect this
idea and you see it in the documents as they often focus
more on the percentages of a particular problem in a
group of patients. In my experience, and the experience
of others, this gap can lead to what appears to be “a
cross-cultural interaction,” and therefore it is no wonder
that patients and their doctors may not understand each
other and disputes and conflict can turn into hostility.5
Can we bridge this gap easily? A big question, but
for the sake of our discussion, is there a way a doctor
can use a few sentences in the informed consent process
begin to bridge the gap. I think so and I will try to
summarize an idea for you. At some point in the
informed consent process a doctor might say something
like, “The informed consent document is perhaps a bit
daunting, and it might not address all of your concerns
as well as you or I might like. The fact is that problems
are inevitable in the treatment of patients, and no
document can address them all. In the end, what matters
to me is that you understand what I will do for you if
you have a problem associated with your treatment. I
will do what I can to help you get better, and if I cannot
help, your surgeon and I will trust your care to someone
else who we think might be better able to help you.” In
my experience, this is fundamentally what the patient
needs to hear from the referring optometrist and the
refractive surgeon. It is my modest effort to try to use
the middle ground to address the micro-issues of the
patient and their concern about their well-being as well
as the macro-issues the caring doctor wants their patient
to understand about their treatment.
In addition, it sets the tone for an important idea in
surgery. A referring optometrist and/or a refractive
surgeon may find that a patient has a problem that
someone else might be better able to treat, and using this
language lets the patient know that there are limits to
what the optometrist and/or refractive surgeon may be
able to do to help a patient get better. Often patients
expect more from their doctors than they are able to
provide with certainty, so it is best to set that tone early
in the doctor-patient relationship, and not when the
patient has an unexpected result or a problem following
surgery.
However, before embarking on such a strategy to
help your patients, it would be best for the referring
optometrist and the refractive surgeon to have a
discussion about the informed consent process and the
language each will use with the patient. It is surprising
how little time and narrative is invested in this language,
but it is all the same very meaningful for the patient and
their doctors. Again, the idea is to be able to express
your respect for the patient as an individual, outline
what you will do to help the patient get better if there
are problems following surgery, while at the same time
setting a boundary on what you may be able to do.
References
1. LeBlang TR. Informed consent and disclosure in the
physician-patient relationship: expanding obligations for
physicians in the United States. Med Law 1995;14:429444.
2. Levine EG, Brandt LJ, Plumeri P. Informed consent:
a survey of physician outcomes and practices.
Gastrointestinal Endoscopy 1995;41:448-452.
3. Miller FG, Joffe S. Limits of research risks. J Med
Ethics 2009;35: 445-449.
4. Fukuda H, Imanaka Y, Kobuse H, Hayashida K,
Murakami G. The subjective incremental cost of
informed consent and documentation in hospital care: a
multicenter questionnaire survey in Japan. J Evaluation
Clin Pract 2009;15: 234-241.
5. Lebed MR, McCauley JJ. Mediation within the health
care industry: hurdles and opportunities. Georgia State
Univ Law Rev 2005;21: 911-929.
John W Potter, OD, MA, is Vice President for
Patient Services for TLC Vision Corporation and a
faculty member in Dispute Resolution at the Annette
Caldwell Simmons School of Education and Human
Development at Southern Methodist University,
Dallas, TX. Dr. Potter is an alumnus of the IU School
of Optometry (1973).
Making Small Visual Displays Accessible to
People with Vision Loss
BY MARK USLAN, M.S., M.ED.
The ability to read small visual displays (SVDs)
affects successful functioning at home and in the
workplace. SVDs can be found in products as diverse as
cell phones, personal digital assistants, photocopiers, fax
machines, kitchen and laundry appliances, home
entertainment devices, exercise equipment, and diabetes
self-management technology. Individuals with vision
loss face severe limitations in using such products safely
and effectively because the visual displays lack
accessibility features. Although vision and ergonomics
experts do agree on the most important optical
characteristics of an SVD, many current SVDs utilize
inexpensive and commonplace LCD technology, which
is far less than optimal. A usable SVD is extremely
important to the more than 20 million who report having
vision loss because they prefer getting device output
through reading the visual display.
The "readability" of an SVD depends on two
aspects – the ability of the visually impaired person to
discern details and the ability of the screen to generate
them. These two aspects can be quantified. The
American Foundation for the Blind (AFB) has been
creating means to assess SVDs (a) by developing optical
instrumentation to measure these displays and (b) by
conducting a study to correlate display measures with
display recognition ability of persons with vision loss in
conjunction with the Palo Alto VA.
Building on this past research and with support
from the Department of Education’s National Institute
on Disability and Rehabilitation Research, AFB has
undertaken a project with three sets of outcomes: (1) a
replicable, and potentially commercializable, Optics Lab
for measuring SVDs; (2) a set of standards for the
design of SVDs relative to human contrast sensitivity
function which will be commercially valuable for
product developers; and (3) a searchable, tabular
analysis of the accessibility and usability of SVDs used
in approximately 250 products with informative
guidance for consumers to use in inquiring about
accessibility of products with SVDs.
The AFB TECH Optics Lab is designed to measure
display qualities of all types of small visual displays.
The lab uses a custom-made integrating sphere to
illuminate the display and a digital camera to take a high
resolution image of it. The picture is then sent to the
dedicated PC, which uses image analysis software to
determine the contrast, modulation transfer function,
Square-Root Integral, and reflection of the display. This
information will be put into a format for consumers and
manufacturers to easily compare the low-vision
accessibility of hundreds of
devices currently on the
market. The AFB TECH
Optics Lab equipment and
some key AFB personnel are
shown in Figure 1.
This is a development
project and more. It is a
systems change project, an
accessibility project, an
Figure 1. The AFB TECH Optics Lab
advocacy project, and an
equipment and some key AFB personnel,
information dissemination
from left to right: Lee Huffman, AFB
project. The intent is to
Editor of AccessWorld; Caesar Eghtesadi,
establish a market
Ph.D., Technical Consultant; Morgan
environment in which
Blubaugh, AFB research Intern; Paul
manufacturers – now and in Schroeder, AFB V.P.; Mark Uslan,
the future – compete to
Director, AFB TECH; Ron Schuchard,
improve accessibility and
Ph.D., Palo Alto VA/Stanford University.
give consumers a choice of
SVDs that best match their visual abilities. The project
will build on AFB’s strong history of technology
awareness and expertise, mission synchronicity, and
reputation for collaboration and advocacy. AFB's
approach is to inform consumers and promote change
directly with mainstream manufacturers. The standards
and guidelines created will be shared with the national
community of technology experts, national standards
bodies, researchers, consumers, educators, and
rehabilitation professionals -- practitioners who are
shaping policy and practices for provision of accessible
SVDs. Through its AccessWorld e-zine,
www.afb/or/acessworld, AFB transmits results of
specific product testing directly to 8,000 readers who, as
sophisticated consumers, are access technology
advocates. AFB has many successes in transmitting
similar information to manufacturers and getting product
modifications in next-generation products. With this
project, we are also testing our ability to affect national
electronics standards bodies by having our standards
adopted.
Mark Uslan earned an M.S. in physiological optics from
Indiana University in 1977. He also holds an M.Ed. degree.
He is Director of AFB TECH at the American Foundation for
the Blind. He has more than 25 years of experience working in
the field of vision loss. In the 1990s he helped develop AFB's
Product Evaluation Laboratory and AFB's technology journal,
AccessWorld®. He has published over twenty journal articles
and two books.
A DESCRIPTION OF THE VISUAL AURA IN MIGRAINE
AURA WITHOUT HEADACHE
BY
DAVID A. GOSS, O.D., PHD.
I first experienced the visual aura of migraine aura
without headache in my mid 30s. It took a few minutes to
figure out that strange visual sensation, but having read a
good description of the aura associated with migraine in
Diagnosis and Management in Vision Care,1 I braced
myself for the coming headache. Thankfully, the
headache never came. Since then, I have often had
migraine aura without headache, sometimes as often as
two or three times a week, but other times going for
months without one. I have not been able to identify any
associations with them, other than that they seem more
likely in stressful times.
Migraine aura without headache was previously
known as acephalgic migraine or migraine equivalent.2,3
In a survey of one thousand patients, 18 years of age and
older, at the University of Alabama Birmingham School of
Optometry, 6.5% reported having visual sensations
consistent with migraine aura without headache.3 That
survey identified a history of migraine headaches and a
history of childhood motion sickness as significant risk
factors. The age at which the first migraine aura without
headache occurred was variable, with a mean of 39.3 years
and a standard deviation of 18.4 years. Fatigue was
reported to be a precipitating factor in 45.8% of cases.
The visual aura in migraine aura without headache is
thought to be “very similar – if not identical” to the aura
preceding a migraine headache.2 Scintillating scotoma
and photopsia are the terms used most frequently to
describe the visual aura in migraine aura without
headache.2,4,5 The aura is usually described as
homonymous and as enlarging and moving across the
visual field over a period of ten to thirty minutes.
When I experience migraine aura without headache,
the first thing I see is a tiny pinpoint flashing light at the
fixation point. Then it slowly moves to one side and
enlarges slightly, becoming a tiny kidney-bean shaped
flashing light. The concave side of the “kidney bean” is
just temporal to the fixation point. Next it enlarges into a
crescent shaped object, similar in shape to the crescent
moon. The top and bottom points of the crescent are
directly above and below the fixation point, with the top
point of the crescent closer to the fixation point than the
bottom point. Then the crescent elongates into a C-shaped
object. As the Cenlarges, it moves away from the fixation
point, and finally disappears into the periphery, with the
top of the C moving out of the superior visual field, the
bottom of the C moving out of the inferior visual field, and
the body of the C disappearing temporally.
The aura is seen with both eyes and is homonymous.
The area within the crescent and C seems to be a mix of a
shimmering area and an area of zig-zag lines, somewhat
like a chain-link fence, but is somewhat variable from
episode to episode. Sometimes it is mostly shimmering
area and sometimes it is mostly zig-zag lines, and the
shimmering area can sometimes be very colorful and other
times less colorful. The time from the first appearance of
the flashing light at the fixation point to the disappearance
of the aura into the periphery is usually twenty to twentyfive minutes. I have noticed the aura in both the left visual
field and the right visual field, perhaps a little more often
in the right visual field. Drawings which are similar in
appearanace to the aura I experience have been
published.1,6
Amos and Fleming2 have reported that the visual
aura in migraine aura without headache can be
accompanied by somato-sensory, motor, speech, olfactory,
auditory, vestibular, or cognitive symptoms. I have
experienced none of those symptoms, but sometimes I feel
more comfortable in quiet, dimly-lit surroundings during
and immediately after the aura.
Kunkel5 notes that migraine aura without headache
is benign, but that it is a diagnosis of exclusion.
Differential diagnosis includes transient ischemic attack,
retinal disease, partial seizures, and recurrent emboli to the
brain. The visual sensations in transient ischemic attack
are usually of shorter duration, do not move across the
visual field, and generally cause a dimming of vision.5
Flashes of light can occur in retinal disease, but they tend
to last longer and are monocular. Visual symptoms can
occur in recurrent emboli to the brain, but multiple
repetition of the same symptom pattern is unlikely.5
Kunkel5 suggests that “if a patient has had recurring
symptoms for some time that are typical of migraine aura
but has no deficits found on physical or neurological
examination, a complete workup with laboratory and
imaging tests is probably not necessary.” Testing to rule
out other conditions could include tests such as magnetic
resonance imaging, magnetic resonance angiography,
vascular ultrasonography, electroencephalography,
electrocardiogram, and laboratory tests.2,5 The references
listed below can be consulted for more information on
migraine aura without headache.
References
1. Stelmack TR. Headache. In: Amos JF, ed. Diagnosis
and Management in Vision Care. Boston: Butterworths,
1987:9-42.
2. Amos JF, Fleming JB. Clinical description and
review of migraine aura without headache. Optom – J Am
Optom Assoc 2000;71:372-380.
3. Amos JF. Migraine aura without headache:
prevalence and risk factors in a primary eye care
population. Optom – J Am Optom Assoc 2000;71:381389.
4. Kunkel RS. Acephalgic migraine.
Headache1986;26:198-201.
5. Kunkel RS. Migraine aura without headache:
benign, but a diagnosis of exclusion. Cleveland Clin J
Med 2005;72:529-534.
6. Richards W. The fortification illusions of
migraines. Sci Am 1971; 224:88-95.
David Goss is Professor of Optometry at Indiana
University. He holds an O.D. degree from Pacific
University and a Ph.D. in physiological optics from
Indiana University.
BOOK REVIEW: FIXING MY GAZE: A SCIENTIST’S
JOURNEY INTO SEEING THREE DIMENSIONS
REVIEWED BY DAVID A. GOSS, O.D., PHD.
Fixing My Gaze: A Scientist’s Journey into Seeing in
Three Dimensions. Susan R. Barry. New York: Basic
Books, 2009. xvi + 249 pages. ISBN: 978-0-46500913-8. Hardcover, $26.00.
This noteworthy book is
written at a popular science level,
but it should also be enlightening
for eye and vision care
practitioners and for vision
scientists. The author not only
tells a remarkable story as an
optometry patient, but she also
enriches the story with knowledge
coming from her background as a
neurobiologist.
Sue Barry was diagnosed
with congenital alternating
esotropia as a small child and had
three strabismus surgeries, the first
at 28 months of age. The surgeries
reduced the esotropia and resulted in her eyes appearing
straight much of the time, particularly in upward gaze.
She developed a habit of tilting her head down to keep
her eyes straight. She never had stereopsis as a child or
young adult. She was unaware of her lack of stereopsis
until a lecture in college in which stereopsis and its
absence in strabismus were discussed. She did not
know what she was missing.
Her childhood vision problems led to poor
performance on placement examinations in third grade
and she found herself in the lowest level classroom.
Her mother worked very hard with her to teach her to
read and to get her to appreciate reading. By fifth
grade, she made it to a regular classroom. She
described herself at that point as “a competent, if slow,
reader.” (p. 37) One classroom reading exercise was
particularly difficult for her. The words of a story were
moved across a projector screen. She was unable to
follow the words across the screen due to her poor eye
movement skills. Later she had considerable difficulty
learning to drive.
At 40 years of age, she was having problems
because “the world appeared jittery” (p. 46). Reading
road signs when driving was difficult due to her
inability to keep her eyes on the words on the signs.
She had an eye exam and was told that she had nothing
wrong with her eyes that glasses for nearsightedness
didn’t correct.
Her visual world appeared unstable due to the
alternation of fixation between her two eyes. Her
unstable visual environment and her uncomfortable
vision led her to try another eye doctor. She saw Dr.
Steven Markow, an optometrist who referred her to Dr.
Theresa Ruggiero, an optometrist specializing in vision
therapy. Dr. Ruggerio found an esotropia which was
greater at near than distance and a vertical tropia at all
distances, along with a lack of binocular fusion at any
distance. The author reported that Ruggerio gave her a
more complete vision examination than she had
experienced before. Ruggerio prescribed vertical prism
and vision therapy.
At first, the author thought that the exercises, like
Brock string, seemed simplistic, but she quickly
realized how much trouble she had trying to perform
them. She devoted two chapters to how her visual
system and her perceptions were changing as a result of
her work in vision therapy and what she was learning
about vision through the experience. She speaks
movingly of her first experience of stereovision. Her
description is something that those of us who take
binocular vision for granted probably couldn’t
anticipate. She said that the emergence of stereovision
brought her “moments of absolute wonder and delight.
The most ordinary objects looked so beautiful. A large
sink faucet reached out toward me, and I thought I had
never seen such a lovely arc as the arc of the faucet.
The grape in my lunchtime salad was rounder and more
solid than any grape I had ever seen before. I could see,
not just infer, the volume of space between tree limbs,
and I loved looking at, and even immersing myself in,
those inviting pockets of space.” (p. 95)
She also wrote that it had been impossible for her
to imagine what having stereopsis was like until she
experienced it herself. “Stereopsis provides a
distinctive, subjective sensation…While I could infer
indirectly a sense of depth through cues like perspective
and shading, I could not synthesize stereoscopic depth
from other visual attributes…The sensation provided by
stereopsis of empty space and things projecting or
receding into that space is unique.” (pp. 101-102) She
said that she “was completely unprepared for my new
appreciation of space and for the deep feelings of joy
and wonder, the enormous emotional high, that these
novel sights gave me.” (p. 111) And when she sought
out other persons who had gained stereopsis for the first
time in adulthood, she found that they had experienced
the same feelings of awe and amazement.
Barry noted that it is often assumed that strabismic
children can learn to judge depth through motion
parallax, but studies have shown that individuals who
have been strabismic since early childhood are not good
at using motion parallax for judging depth. A normal
experience of depth through stereopsis is needed to
learn effective use of motion parallax. It has also been
found that pursuit eye movements are important in
motion parallax. The author found that as she
developed stereopsis and better pursuit eye movements
through vision therapy, she was able to “make better
use of motion parallax.” (p. 126)
There are many insights in the book about vision
that we should be reminded about from time to time.
For example, Barry’s experience in gaining stereovision
led her to observe that an optimally functioning visual
system is essential for being able to move confidently
and accurately through our surroundings. And her
childhood experiences and those of other persons that
she corresponded with led her to write that “Examples
abound of children who have visual problems
misdiagnosed as learning disorders.” (p. 40)
Changing concepts of critical periods in visual
development are consistent with the author’s ability to
attain binocular fusion for the first time in her 40s. The
very young nervous system may undergo changes due
to passive stimuli. The adult nervous system will not
respond to those same passive stimuli. Active learning
is necessary to change the adult brain. It was vision
therapy that provided the active learning necessary for
Barry to develop binocular fusion as an adult.
The book is, as the subtitle states, the author’s
“journey into seeing in three dimensions,” a fascinating
and insightful story in itself. But it is also a well
researched book with important lessons for clinicians
and scientists. The book includes a 58 page section of
reference notes that can be used for further reading and
study.
In the foreword to the book, the noted neurologist
Oliver Sacks calls Fixing My Gaze “an ode to the
fascination and wonder of the visual world, even those
parts of it which many of us take for granted.” (p. xvi)
It has gotten significant attention from various circles.
Favorable reviews appeared in such journals as Nature
Neuroscience, New England Journal of Medicine, and
Optometry and Vision Science. Two Nobel Prize
winners, David Hubel and Eric Kandel, were quoted in
dust jacket endorsements. Amazon.com listed it at
fourth in the list of top science books for 2009
(http://www.amazon.com/gp/feature.html?ie=UTF8&pl
group=1&docId=1000446551). Fixing My Gaze should
be read by all optometrists and all vision scientists.
More information about the book can be found at
http://www.stereosue.com.
Non-Profit Org.
U.S. Postage
PAID
Bloomington, IN
Permit #2
Indiana Journal of Optometry
Indiana University School of Optometry
800 East Atwater Avenue
Bloomington, IN 47405

Summer 2010 - School of Optometry

Transcription

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