INTERNATIONAL MEDICAL JOURNAL ON DOWN SYNDROME Original Paper A study of visual quality in adults with Down´s syndrome Marina Castañé1, Mercè Boada2,3, Isabel Hernández3 1 Departament d’Òptica i Optometria. Universitat Politècnica de Catalunya 2 Servei de Neurologia. Hospital General Universitari Vall d’Hebron 3 Fundació ACE. Institut Català de Neurociències Aplicades Correspondence: Marina Castañé Escola Universitària d’Òptica i Optometria C/ Violinista Vellsolà, 27 08222 Terrassa, Spain e-mail: castane@oo.upc.es Abstract Introduction
A group of persons with Down syndrome aged
Individuals with cognitive impairment are
over 40 was studied to assess the presence of
at a greater risk for severe eyesight problems
Alzheimer’s disease and its clinical traits as well as
than non-disabled persons. Severe refractive error,
poor visual acuity, strabismus, and cataracts,
acetylcholinesterase inhibitor, for Alzheimer’s
among others, have all been described as issues
disease. Assessment was neurological, physical
and sensory, and also included the following
According to some studies, individuals with
additional examinations and cognitive scales:
accommodative disorders and visual acuity (VA)
psychopathological analysis, psychopathological
disorders (8-11), nystagmus (12), cataracts and
assessment scale, and an interview with each
person’s guardian or teacher. The present paper is
Similarly, individuals with cognitive disabilities
a part of the main study, and specifically looks at
have been found to have poor visual acuity, even
refractory status and visual quality. There were 49
with the best correction possible (4).
cases, aged 40 to 62. Testing included visual acuity
Some studies specifically highlight DS, citing
(VA) measurement, binocular vision and ocular
VA range values within the «low vision»
motility assessment, retinoscopy, subjective
range (14-16). Many individuals with disabilities
examination, and ocular health assessment. The
who ought to be wearing spectacles to counter
suitability of prescription lenses already worn by
patients and the visual performance they supplied
spectacles are worn, but are not optimally fitted
was also assessed, both for near and for distant
The purpose of this study was to assess the
visual quality of a group of adults with DS aged 40
Keywords:
and over, both in terms of refractive error and as
Optometric prescription. Down syndrome. INTERNATIONAL MEDICAL JOURNAL ON DOWN SYNDROME Subjects and method
cycloplegic drugs. Patients who used spectacleswere examined while wearing them.
The findings discussed below are part of a
Binocular integrity and ocular health were
general study of a group of individuals with DS
who were neurologically assessed for potential
Alzheimer’s disease (AD). Sensory testing of allparticipants included optometry to assess theirvisual status.
The 49 subjects included in the study (27 male
and 22 female) were patients with DS aged 40 to
Out of 49 subjects with Down syndrome, one
62, with a mean age of 47.59. Alzheimer’s disease
was excluded from the study due to acute viral
was diagnosed in 12 patients, who were treated
conjunctivitis at the time of assessment.
with donepezil; two patients diagnosed with AD
were unable to pursue their treatment for a variety
functional level thresholds: VA ≥ 0.5 was optimal,
of reasons, and four patients were borderline
AV< 0.5 ≥ 0.3 borderline low vision; VA < 0.3≥ 0.1
low vision, and VA < 0.1 legal blindness. Results
The study looks at the whole group of patients.
were rated using habitual correction lenses on the
Results comparing the two subgroups best eye. —individuals with Down Syndrome and patients
At the beginning of the study, 53% of the study
with DS and AD— were discussed in a different
population wore glasses habitually. In everyday
article (17). No statistically significant differences
viewing conditions, 54.2% of patients had low
were found between the two groups in terms of the
vision, or VA between 0.3 and 0.1; 25% were
between 0.3 and 0.5 VA, borderline low vision;
Distant-vision VA was assessed at 3 meters
2.2% had VA ≥ 0.5, which made them functionally
using the broken wheel test (BWT), which is based
normal, and one patient was a member of the
on the Landolt C test. Two cars are shown at a
Spanish National Organization for the Blind
time; one of them has whole wheels, and the other
(ONCE). In 18.7% of cases, assessment was
has incomplete wheels which are actually Landolt
C rings opening in different directions. Car size
varies according to a scale that assesses acuity up
retinoscopy and prescription update, 33.33% had
to 20/20. The BWT is designed for a 3-meter
VA between 0.3 and 0.1, 31.25% between 0.3 and
distance. It is easy to understand and encourages
0.5, 10.4% attained VA higher than 0.5, and VA
could not be assessed for 18.75% (Fig. 1).
Near vision was assessed at a 25-cm distance.
Although most standard near-vision tests involve
refractive correction had been optimized in the
text or letters, the test chosen in this case was one
low-vision borderline range. The rate of low vision
that involved numbers, as illiteracy was fairly
due to poor VA was also lowered with prescription
updates. To summarize, correct lens prescription
VA was measured monocularly and binocularly,
improved distant vision for 39.6% of patients,
as well as under normal conditions for the subject
failed to improve it for 14.6%, required a change
(i.e., wearing spectacles if prescribed). After
of prescription for 16.7%. No lenses were required
refractive status was assessed, VA was measured
For near vision, the cut-off point was set at
Refractive status was assessed using Mohindra
VA≥ 0.4: this is the threshold for low vision, and it
retinoscopy, which requires total darkness in order
is also the level for typical typeface size on most
to control for accommodation (18,19) to ensure
printed matter (books, magazines, newspaper
accurate correction of refractive error and avoid
articles, etc.). Only 4.2% of the population —that
is, 2 patients— had been using spectacles for near
undercorrection of hyperopia, as well as the use of
vision before the study. VA values obtained in
INTERNATIONAL MEDICAL JOURNAL ON DOWN SYNDROME
and one patient received spectacles for the firsttime. VA improved in 26.7% of cases and did notchange in 13.3%. VA figures obtained with newrefractive correction were: VA ≥ 0.5 in 13.3%; VA< 0.5 ≥ 0.3 in 13.3%; finally, assessment wasimpossible in a single case (Fig. 3).
Once the prescription was updated, visual
Figure 1. - A comparison of distant-vision VA under everyday refraction
(Rx hab.) and induced refraction (Rx ind.), in the best eye.
functionally acceptable in 13.3% of cases, asopposed to none in prior everyday conditions.
normal viewing conditions were as follows:
Degree of refractive error was analyzed for the
VA≥ 0.4 in 6.25% of patients; VA< 0.4 in 46% of
patients. Assessment at this distance level was
hyperopia were established: low (0 to 2.75
impossible for 48% of patients due to lack of
diopters), medium (3 to 6 diopters), and high (>6
cooperation or failure to recognize numbers diopters). The purely astigmatic component(Fig. 2).
Following refractive assessment, VA testing
was repeated using the correct prescription for
independently and also sorted into three
near vision. The following values were obtained:
levels: low (0 to 0.75 cylinder diopters), medium
VA ≥ 0.4 in 18.75%; VA < 0.4 in 6.25%;
(1 to 2 cylinder diopters) and high ( > 2 cylinder
prescription in 2.1% of cases (Fig. 2).
Additional correction for near vision was
follows: low myopia (0-2.75D) in 14.6% of eyes,
prescribed for 29.2% of patients: 25% received
medium myopia (3-6D) in 25% of eyes, high
myopia (>6D) in 21.9% of eyes (Fig. 4). At the
The age range suggests that presbyopia may be
time of assessment, 42.7% were using prescription
present in every one of the study subjects. The DS
lenses for myopia; 12.5% were advised to change
population has low accommodative capacity (2-4),
their prescription, 28.1% required no change, and
a fact which also explains near-vision difficulties,
spectacles were prescribed for the first time for
14.6%; VA improved in 24.6% of cases with the
Where binocular vision is concerned, the rate of
strabismus and motility disorders was 66.7%.
Ocular health assessment turned up the following
hyperopia (0-2.75D), 6.25% medium (3-6D), and
results: among other findings, 59.4% had lens
none had high hyperopia (>6D) (Fig. 4); 7.3% had
opacities, 25% had nystagmus, 15.6% had had
been wearing spectacles, changes in prescription
were advised for 5.2%, no changes were required
abnormalities, and 6.2% had keratoconus.
in 2.1%, and spectacles were prescribed for the
The group of individuals who had undergone
first time in 8.3%. Prescription changes were
cataract surgery was specifically studied and theirVA was analyzed using the same ranges as above:no patient had VA ≥ 0.5; 20% had VA < 0.5 ≥ 0.3;26.7% had VA < 0.3 ≥ 0.1 and VA could not beassessed in 6.7% of cases (Fig. 3).
At the time of examination, 26.7% of surgery
patients were using spectacles, 20% were not, and20% had received an intraocular lens implant inthe course of surgery.
Following refractive examination, 26.7% of
Figure 2. - A comparison of near-vision VA under everyday refraction (Rx
patients had changes in their refractive prescription
hab.) and induced refraction (Rx ind.), in the best eye. INTERNATIONAL MEDICAL JOURNAL ON DOWN SYNDROME
Figure 3. - A comparison of distant-vision VA under everyday refraction(Rx hab.) and induced refraction (Rx ind.), in the best eye of patients who
Figure 5. - Distribution of spectacle wearers and of proposed prescription
found to have improved VA in 12.5% of cases
Our group had a 53% rate of spectacle wearers,
a fairly high rate compared to other studies.
As to astigmatism, 22.9% of prescriptions had a
Another finding was that individuals who benefit
cylindrical component, 7.3% had low astigmatism
from the use of spectacles for distant vision
(0-0.75DC), 29.2% medium (1-2DC), and 7.3%
achieve greater visual quality, though still below
high (>2DC) (Fig. 6). A change in cylindrical
what is considered normal. These findings are
power was prescribed for 7.3%, change was
similar to those of other studies (3,6).
Another point worth highlighting is that,
component was prescribed for the first time in
although many prescriptions ought to be reviewed
and modified, the challenges posed by thesepatients and the high rate of severe ametropia maycall for partial prescriptions (but we are not aware
Discussion
of whether this had been the case of the patientswho were already wearing spectacles before the
These results support the statement that the
study). VA for near vision was within the expected
visual quality of individuals with DS is clearly
range for this group, given both their age and the
compromised, particularly where distant vision is
reduced accommodative capacity apparently
concerned. Results obtained in this study are
linked to Down syndrome, which would further
similar to the findings of earlier studies of groups
explain loss of vision at all distances, especially
with learning disabilities (4,5) and DS specifically
(8,9,11), as well as studies involving age groups
A point that must not be forgotten is the number
of subjects for whom VA could not be measured at
this distance range, probably because the test
percentage of individuals with low VA in everyday
employed was not ideally suited to their learning
Figure 4. - Myopic and hyperopic eye distribution.
Figure 6. - Distribution of astigmatic eyes. INTERNATIONAL MEDICAL JOURNAL ON DOWN SYNDROME
changes in prescription were not very frequentbecause vision did not improve with better fit. Where prescriptions were changed or lenses wereprescribed for the first time, VA did increase; thesechanges were therefore deemed favorable.
significance of securing appropriate visual care forthese individuals. We also point out that in ourexperience they can benefit significantly through
Figure 7. - Distribution of spectacle wearers with cylindrical prescriptions
improved visual quality by the use of spectacles
for both near and distant vision. In most cases, useof spectacles does not require a great effort by
After adding a prescription for near vision,
relatives or other caregivers: the obvious
visual quality clearly improved. The fact that high
improvement in vision, quality of life and
reading performance is not required of these
individuals does not mean that their near-distance
visual needs should be ignored. After all, manymembers of the DS population are in shelteredworkshops, where activities often involve near
Acknowledgments
vision and precision is frequently required. Specific lenses for near vision, whether monofocal
We wish to thank all the volunteers who took
or bifocal, may therefore improve performance
part in this study, as well as their relatives and
and level of comfort. This is borne out by our own
findings in this study and those of Woodhouse (6)
This study was carried out with the institutional
and financial support of Fundació Catalana
Also worth noting is the number of subjects
Síndrome de Down, Fundació ACE, Institut Català
de Neurociències, Universitat Politècnica de
nevertheless had very low visual acuity in
Catalunya, Pfizer SA, and the Department of
everyday conditions. Once refractive correction
Health and Social Security of the Government of
was updated, VA improved and a significant
number of patients achieved levels consideredfunctionally acceptable.
Appropriate treatment for cataracts should
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