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Virginia C. Karlsson, CO, COMT
Certifed Orthoptist
Mayo Clinic
Rochester, Minnesota
Series Editors:
Karlsson, Virginia.
A systematic approach to strabismus / Virginia Karlsson. -- 2nd ed.
p. ; cm.
Includes bibliographical references and index.
ISBN-13: 9781556427947 (alk. paper)
1. Strabismus. 2. Eye--Examination. 3. Ophthalmic assistants. I. Title.
[DNLM: 1. Strabismus. 2. Strabismus--diagnosis. WW 415 K18s 2008]
RE771.H36 2008
617.7'62--dc22
2008029541
DOI: 10.1201/9781003526681
Dedication
In memory of
Steven William Salevouris
1956 – 2006
Dedication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v
Acknowledgments. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . viii
About the Author . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
Foreword by Jonathan M. Holmes, MD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . x
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi
Chapter 6. Binocularity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107
Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Acknowledgments
Another 10 or so years have gone by since A Systematic Approach to Strabismus was first
written for SLACK in response to their success with the original 12-volume series for ophthalmic
personnel. Now, we have this second edition: my third book, but probably not the last.
Again, I’ve learned a lot in the past 10 years and am grateful to the pediatric ophthalmologists
at the Mayo Clinic with whom I’ve had the pleasure to work. They have encouraged me to have
the best of both worlds: seeing real patients within a team approach and teaching and academics.
Drs. Jonathan Holmes, Michael Brodsky, Brian Mohney, and George Hohberger all have shared
a common joy in the care of our patients and a wonderful variety in styles of doing so!
I blinked, and it happened: my children have grown up considerably. Thank you Dory for
proving that the impossible can happen. Thank you Lars for taking on an impossible role. Thank
you Carlson for fitting in impossible places. Thank you Greta for knowing that everything is pos-
sible, and thank you Colby for reminding me daily that today might just possibly be the best.
About the Author
Virginia Carlson’s orthoptic career began at the age of 2 when she climbed over into the front
seat of her parents’ 1956 Chevy Bel Air and gave her mother a corneal abrasion with her finger-
nail. Unfortunately, this abrasion was to her mother’s NON-amblyopic eye and required 3 days of
pressure patching to heal. From that point onward, however, her mother always claimed that her
amblyopic eye could see just a little bit better after all that albeit unintentional, occlusive therapy
that had been overlooked in her childhood.
Virginia Karlsson, now spelling her last name the way her paternal grandfather had before
immigrating to the United States, celebrated her 30th year as a certified orthoptist in 2008.
Colby-Sawyer College in New London, New Hampshire, provided her introduction to orthop-
tics as a career, providing an excellent undergraduate experience. The University of Florida
orthoptic training program in Gainesville provided something unique 32 years ago—simultane-
ous training as a tech and as an orthoptist. After graduating, Carlson now Hansen returned to
New England where she took two part-time jobs: one as a tech to support her second part-time
job as an orthoptist in western Massachusetts. Two years later, she became the director of the
orthoptic training program at Tufts-New England Medical Center in Boston. After the birth of
her first child, she filled in (twice each) for other orthoptists out on maternity leave themselves
at the University of Massachusetts in Worcester; the Lions Orthoptic Clinic in Springfield, Mas-
sachusetts; and the Newington Children’s Hospital in Newington, Connecticut.
She returned to work full-time in 1990 when her family moved to Minneapolis; she worked
for a large health system and then a private practice. In 2005, Karlsson took the orthoptic posi-
tion at the Mayo Clinic and has thoroughly enjoyed the extraordinarily challenging patients, the
completely ordinary patients, the academic environment, and the peaceful commute from Min-
neapolis to Rochester, Minnesota.
Five children with varied interests don’t leave much time for Mom, but most recently she
has remembered how to ski, speak Swedish, play kubb (poorly), and definitely laugh more.
“Don’t blink!” is the advice she tells new parents about their baby’s first year of life. Maybe she
shouldn’t have blinked so much over the past 30 years of being an orthoptist!
Foreword
Many of us can remember the sense of inadequacy we first felt when we tried to approach a
young child to glean any information from his or her eyes. Pediatric ophthalmology and strabis-
mus are “lore unto themselves”; it seems that nothing we learn from examining adults applies to
these hypermobile, hyperdistractable bundles of joy. In this book, Ginny Karlsson shares with us
many superbly practical tips for “making it happen”; acquiring that information from the child;
allowing us to discover, treat, and help; while still having fun. Fun for them and fun for us.
For beginning orthoptists, ophthalmic technicians, and ophthalmology residents, this book is
crammed full of helpful pearls and insights based on the many years of Ginny’s experience. It has
been a joy to work with Ginny taking care of children. May you also find a sense of joy as you
embark on the adventure of taking care of children who have eye problems.
Jonathan M. Holmes, MD
Professor and Chair of Ophthalmology
Mayo Clinic
Rochester, Minnesota
Introduction
As an orthoptic student I remember staring at my big, blank exam sheet and wondering what
the patient in the exam chair had. Worse was staring at my big filled-in exam sheet and wondering
what the patient in the exam chair had. Now I’m looking at a computerized chart note with drop-
down boxes of cookie-cutter choices and realize that no matter how much gets filled in, we still
have to think about how to put our whole exam together and be able to make sense of the infor-
mation. Over time (up to the present and beyond), the examination of the patient with strabismus
has become clearer to me, and it is that exam that I have tried to put into this book.
Many other texts cover pediatric ophthalmology and strabismus in immense detail. My only
intention here is to get the beginner started turning that big, blank exam sheet; computer monitor;
or some yet-to-be-invented tool into a skillfully diagnosed patient with a treatment plan that the
patient and parents will work with.
The Systematic
Approach to
Strabismus
K E Y P O I N T S
DOI: 10.1201/9781003526681-1
2 Chapter 1
Congratulations! You have bravely entered the world of strabismus and ocular motility. This is
the same world that has terrified residents and technicians, brought experienced ophthalmologists
to their knees, and placed a smile on the orthoptist’s face. This book is designed to get you past
the common core of knowledge that you possess and move you boldly into the exam room face-
to-face with a 3-year-old child. You know how to test vision and you have read how to measure
stereopsis. The technique for doing a prism and cover test really did not sound that difficult. So
why the terror when your next chart reads, “3 year old with RET”?
Two Eyes
Strabismus and its related subjects bring the added dimension of a second eye to the eye
exam. Although you are always testing two eyes when testing vision, refracting, or testing pres-
sure, each eye is treated as a separate entity. Start thinking of the two eyes as a pair, a combination
to be reckoned with as a single unit. Despite familiarity with the basic tests required to complete
a motility exam, performing these tests gracefully, accurately, and in an orderly and timely fash-
ion on that 3-year-old child with the right esotropia may be a sticking point in your repertoire of
techniques.
exam, which puts many children on guard for a frightening experience. If you really want
to get the child’s attention, speak softly. That will get everyone’s attention.
3. Not every test needs to be done on every patient. Do only those that are necessary. Assum-
ing that your young patient’s cooperation will only last a finite period of time, plan on
doing that part of the exam that is most necessary while the child is still cooperative. For
instance, if a child is destined to be cyclopleged with a postcyclo refraction, do not waste
time trying to get a manifest refraction. The tables in Chapter 7 will help you put together
an exam strategy.
4. Try to limit the level of exam pollution. Exam pollution happens when performing one
test alters the outcome of a subsequent test. An example of this in general ophthalmology
would be to anesthetize the cornea for a pressure check before checking corneal sensitivity.
Testing order is critical. A strabismus exam has four key parts: history, vision, alignment
measurements, and fusion. But those tests are never done in that order!
Fusion is most easily disturbed by measuring alignment or vision, so fusion is usually mea-
sured first. Since the most sensitive fusion test should be done initially, stereopsis is measured
first. This would be followed by Worth four-dot testing if desired and then the single cover–
uncover test (which does not measure alignment but tests for motor fusion control; for more on
this, see Chapter 2).
Ideally, alignment measurement should be done before vision testing, since prolonged patch-
ing of the eye for vision testing could alter the everyday ocular alignment and will certainly
disrupt fusion. Alignment measurements require the proper fixation target, appropriate measuring
test, and your polished technique.
Vision testing on children requires your utmost patience. The ability of the patient must be
considered and the appropriate test selected long before the patch goes on the child’s eye. An
adhesive patch should always be used to prevent cheating and peeking, unintentional or other-
wise. Finally, while the patch is still on, a dry retinoscopy or manifest refraction (to be balanced
later) should be done, and vision rechecked for improvement, with that correction in place.
You can learn how to do all of this by keeping the guidelines in order and by mastering your
skills practicing on cooperative adults.
Chapter 2
The Four-Part
Exam
K E Y P O I N T S
DOI: 10.1201/9781003526681-2
6 Chapter 2
The ocular motility exam generally has four parts, which are performed in the following order:
history clues (so you will hopefully know what you are looking for), fusion testing, alignment
assessment, and vision. This order of testing is essential to reduce pollution of the test results.
problem: “What makes it better/worse? When did it start? Who notices it?” (Grandparents can
be exceedingly accurate regarding grandchildren who they see sporadically with a fresh, albeit
scrutinizing eye.)
A child who was able to articulate a visual complaint to his or her parents certainly should be
able to intelligently respond to questions put forth by a professional: “When you see the double
vision, is it side-to-side like this (demonstrate with your hands) or up and down like this? Is it
blurry when you read a book or when you sit at the back of the room and try to read the board or
overhead projector? Is it blurry to everyone else in class (because of glare, angle, poor penman-
ship) or just to you?”
It is often difficult for patients/parents to verbalize medical problems. A parent may be
reluctant to blurt out the details of the child’s problem(s) in front of the child. A patient is often
kept unaware of the diagnosis until it has been confirmed. If you get nowhere when you ask the
patient if there are any ongoing health problems, ask what other doctors he or she has seen. The
patient may have no idea that the reason he or she is suddenly seeing double, has lost weight,
has been sent to see an endocrinologist who did blood work, and now needs a motility/eye exam
is because someone thinks that he or she probably has Graves’ disease or a thyroid problem. We
often encounter patients who deny that they have high blood pressure because they are on medi-
cation that controls it. Ask what medications the patient is on, but find out if it is important to
know how much medication and what time of day it is taken. You need to know when the patient
last took his or her glaucoma drops, but does it really matter when his or her last antihistamine
was taken? Knowing which doctors are currently following the patient and what medications the
patient is on will help complete the patient’s history.
Record the patient’s allergies to medication, food, and the environment. Patients often do not
realize that an eye drop can actually affect their heart or that an allergy to peanuts could be fatal
in a child given the eye drop phospholine iodide.
As far as the parents are concerned, visual history can be divided into two categories: the
child needs glasses in order to see properly, or more seriously, the child might have uncorrectable
vision and be blind. Amblyopia, which frequently requires occlusion therapy along with glasses,
falls halfway between those two categories since although it is only one eye, it results in per-
manent loss of vision if left untreated. Ask the parents, “How do you think Gus sees?” followed
by, “Who wonders if he sees okay?” This will usually get the answers necessary to determine
if blindness is a true concern or if the visit is merely due to a failed vision screening in a 4 year
old who otherwise appears normal. These questions will usually elicit the parents’ real concerns,
which range from their genuine fear that their baby is blind to disappointment that their child
will be wearing spectacles.
Symptomatic history also has two categories: behavior that is observed (by the parents, teach-
ers, or mother-in-law) and symptoms that the child complains about (cannot see, does not want
to go to school, headaches, does not want to go to school, does not want glasses, does not want
to go to school, sees double, does not want to go to school). The child’s complaints may be real
or fictitious. Table 2-1 lists observed behavior and the potential diagnoses. Table 2-2 lists typical
childhood complaints.
Cosmetic history immediately implies a surgical correction in the minds of parents and chil-
dren. An honest response to “How do you think the eyes look?” may be tainted by their fear of
surgery. When strabismus is obviously present, ask the child if his or her friends ever say anything
about his or her eye alignment. Ask if people who are not the child’s friends ever comment on
it and if that bothers him or her. Ask the parents who else notices the child’s misalignment. The
8 Chapter 2
Table 2-1
Observed Behavior Potential Diagnosis
Holding objects close/sitting near the TV Myopia
Intermittent crossing, especially at near Hyperopia/accommodative esotropia
Tearing/discharge in infant/baby Nasolacrimal duct obstruction
Tearing/photophobia/large eyes Congenital glaucoma
Head positioning Null point for nystagmus
Turning face to one side Lateral incomitance (Duane syndrome/VI nerve palsy)
Tilting head to one side IV nerve palsy
Tipping chin up Avoiding upgaze (Brown syndrome/double elevator
palsy/A or V pattern)
Tipping chin down Avoiding downgaze (A or V pattern)
Headache, diplopia, tires when reading Convergence insufficiency
Table 2-2
Typical Childhood Complaints Potential Cause
Blurry: Distance only Myopia
Near only Extreme hyperopia or convergence insufficiency
Both Astigmatism, malingering, or amblyopia
Headache Any phoria with poor fusional amplitudes
Convergence insufficiency
Diplopia Any intermittent deviation with poor control
Recent appreciation of physiologic diplopia
Strabismus without diplopia Any tropic or intermittent deviation with suppression
Eyes hurt Any of the above plus foreign body
Eyes tear while reading Convergence insufficiency
observational powers of loving parents (and spouses) is remarkably accurate or blind, with some
parents noticing a microtropia and others being able to ignore a 40 prism diopter tropia. This is
why it is so important to ask the parents who else notices the deviation. The parent who thinks
his or her child looks just fine with a 20 prism diopter exotropia (XT) may still consider surgery
if other people are constantly noticing it and commenting on it.
Table 2-3
Worth Four-Dot (Red Lens Over Right Eye)
Response Means Record as
4 lights: 1 red/3 green, 2 red/2 green Fusion in ortho patient Fuse
ARC in tropic patient
2 red lights only Left green eye suppressing OS suppress
3 green lights only Right red eye suppressing OD suppress
2 reds, then 3 greens not at same moment Alternate suppression Alt suppress
2 reds seen to the right of the 3 greens Uncrossed (homonymous) diplopia Unc diplopia
NRC in ET patient
ARC if not ET
2 reds seen to the left of the 3 greens Crossed (heteronymous) diplopia X ed diplopia
NRC in XT patient
ARC if not XT
ARC = anomalous retinal correspondence; NRC = normal retinal correspondence; ET = esotropia; XT = exotro-
pia
for me. While holding the test booklet and ready to start asking about the animals, I inform the
child that “we” will be putting on the very cool glasses (and I have got the glasses on the child
by now) so that the child can show Mommy or Daddy just how cool her or she is. (I am rambling
in whispers by now, but that keeps the child from thinking about taking off the glasses.) “And
now, you can see the animals. Does one of them (without taking a breath) look like it’s sticking
up at you, like it’s sticking up off of the page? (Do not wait for an answer.) Push it down for me,
push him back down on the page. (Ignore the parent’s questions about the test.) That’s great. How
about on this row? Now which circle in this first group looks like a doorbell?” Imitating doorbell
chimes adds to the exclusive relationship that you are developing with the patient, and the testing
continues until you have completed the test. If the child takes the glasses off, say very seriously,
“Oh I don’t want you to touch my special equipment. Let’s have fun instead,” and proceed on to
the Worth four-dot testing, getting the red-green glasses on the same way. Some time during this
process, I mutter to the parents that I will explain everything I am doing later, once I am through
with everything.
Only perform Worth four-dot testing if it is necessary, although it is a great way of demon-
strating your mind-reading powers to children. It is easy to not-so-subtly convince them that they
better not lie about anything. You know just what they see. As you cover the green eye say, “Now
you see two reds, don’t you?” and nod your head in an all-knowing, positive, authoritative way.
In order to complete Worth four-dot testing on a very young child, I invite the child to touch
the lights while I count. In an attempt to get positive answers, I ask an older child, “Do you see
some lights? Any red ones? How many?” The key question if the patient claims to see five lights
is to differentiate between diplopia (and what kind of diplopia) and rapid alternation. “Do you
see reds and greens at the same time, or first one, then the other?” I still have not made eye con-
tact with the parent as I mutter, “No, this isn’t a color vision test.” Hopefully by now, the parent
should realize he or she merely provides a lap for the child thus keeping interruptions to a mini-
mum. Table 2-3 indicates the possible response and method for recording Worth four-dot testing.
The Worth four-dot test is performed at near (one-third of a meter), which tests more peripherally,
and at distance (6 meters), which tests more centrally.
10 Chapter 2
Haploscopic devices are not readily available in most offices. These instruments, such as the
synoptophore or troposcope, allow each eye to be presented with a different target so that assess-
ment of the patient’s retinal correspondence and sensory and motor fusion can be made.
Motor fusion is tested when control of the patient’s alignment is assessed. It is, therefore,
covered in the next section.
Part 3. Alignment
A patient’s strabismic deviation has three components: control, direction, and size.
Control
Control is tested by the cover–uncover test. This is generally done during a routine eye exam
by the single cover test, which determines the presence of a phoria, tropia, or intermittent tropia.
The patient must steadily maintain fixation on a target that requires and stimulates his or her
accommodation. Most patients will not accommodate on a fixation light, and therefore, a pen-
light should not be used for fixation. Many patients will not accommodate on the 20/200 E or
on the stuffed dog at the end of the room. To make a patient fixate and accommodate, you must
force the patient to attempt to identify small objects. While you perform the cover test, ask the
patient, “How many whiskers does the dog have? Is he sitting or walking? Read me the 20/40 line
backward. Forward. What letter is above the Z?” This causes the patient to maintain accommoda-
tion and fixation while you assess eye alignment. The patient should have a cover–uncover test
performed at distance fixation, preferably 20 feet or 6 meters, and at near fixation at 14 inches or
one-third meter. As one eye is covered, the opposite eye is observed for movement. As the eye is
uncovered, it is observed for movement. Table 2-4 breaks down the possible movement responses
when the right eye is cover–uncovered describing a phoria, tropia, or intermittent deviation. Table
2-5 is a similar algorithm for the left eye as it is cover–uncovered.
Vergence amplitudes of a fusing patient are another method of judging control. However,
these measurements should not be taken until all other alignment measurements are done. Gener-
ally, the examiner would want to determine how well a patient with an exodeviation can converge
or how well a patient with an esodeviation can diverge. The horizontal prism bar is gradually
introduced starting with the one and moving up to larger prisms using base-In to measure dIver-
gence, and base-Out to measure cOnvergence. The patient maintains fixation and fusion as the
amount of prism is increased and then is instructed to report when diplopia is appreciated. The
measurement is performed first at distance and then again at near. Seeing two images is the signal
that the vergence amplitude has been exhausted. This is the break point. Reducing the prism until
fusion is regained is a signal of the patient’s ability to recover fusion and, normally, should be
only one or two incremental steps down from the break point. This is the recovery point. While
measuring convergence, a patient should also be instructed to report when the single image blurs,
as this is a signal of the inappropriate use of accommodative convergence to maintain binocular-
ity. This is the blur point. The patient may be able to converge a lot, but the image is blurry. Their
pupils may overconstrict at the moment they overaccommodate and blur. This is a classic finding
in patients with symptomatic convergence insufficiency who will complain of blurred vision after
a few minutes of reading. The print may never actually double; it just blurs.
The Four-Part Exam 11
Table 2-4
Cover–Uncover Right Eye—Test Requires Central Vision in Each Eye
If, when covering OD: This means: If, when uncovering This means:
OD:
1. OS doesn’t move OS was fixing A. OD doesn’t move Either ortho or R tropia
either (confirms with Table
2-5, Step 1)
B. OD moves in OD was out—exophoria
C. OD moves out OD was in—esophoria
D. OD moves down OD was up—
hyperphoria or DVD
E. OD moves up OD was down—
hypophoria
F. Combination
2. OS moves in OS was out (LXT) A. OD doesn’t move Alternating XT
now
B. OD moves back in LXT, prefers OD
to fix
3. OS moves out OS was in (LET) A. OD doesn’t move Alternating ET
now
B. OD moves back out LET, prefers OD
to fix
4. OS moves down OS was up (LHT) A. OD doesn’t move LHT, which alternates
now
B. OD moves back up LHT, prefers OD
to fix
5. OS moves up OS was down A. OD doesn’t move
RHT, which alternates
(L hypo T) now
B. OD moves back down RHT, prefers OD
to fix
6. Combination of moves Combined horizon- A. Combination
tal/vertical
7. OS has unsteady Latent nystagmus
fixation
Direction
Direction of the strabismus is determined by the direction of movement of the eye during
cover measurements. Essentially, an eye that moves out to fixate must have been in (eso) under
that cover. An eye that moves in must have been out (exo), up must have been down (hypo), and
down must have been up (hyper). Table 2-6 lists the commonly used abbreviations for strabismic
deviations.
Size
The size of the existing deviation is measured with prisms. The more sophisticated the test,
the more accurate it will be. Table 2-7 lists methods of measuring strabismus from the least
sophisticated test to the most sophisticated. While the Maddox rod can give you very accurate
measurements, it also has the most limitations and, therefore, should not be used on most patients.
12 Chapter 2
Table 2-5
Cover–Uncover Left Eye—Test Requires Central Vision in Each Eye
If, when covering This means: If, when uncovering This means:
OS: OS:
1. OD doesn’t move OD was fixing A. OS doesn’t move Either ortho or L tropia
either (confirm with Table 2-4,
Step 1)
B. OS moves in OS was out—exophoria
C. OS moves out OS was in—esophoria
D. OS moves down OS was up—hyperphoria
or DVD
E. OS moves up OS was down—hypophoria
F. Combination
2. OD moves in OD was out (RXT) A. OS doesn’t move now Alternating XT
B. OS moves back in to RXT, prefers OS
fix
3. OD moves out OD was in (RET) A. OS doesn’t move now Alternating ET
B. OS moves back out RET, prefers OS
to fix
4. OD moves downs OD was up (RHT) A. OS doesn’t move RHT, which alternates now
B. OS moves back up to RHT, prefers OS
fix
5. OD moves up OD was down (R A. OS doesn’t move LHT, which alternates now
hypo T)
B. OS moves back down LHT, prefers OS
to fix
6. Combination of Combined horizon- A. Combination
moves tal/vertical
7. OD has unsteady Latent nystagmus
fixation
Table 2-6
Strabismus Abbreviations
Tropia T
Intermittent tropia, add () (T)
Phoria, drop T
Esodeviation E
Exodeviation X
Hyperdeviation, add R or L eye H RH or LH
Hypodeviation, add R or L hypo R hypo or L hypo
Dissociated vertical deviation DVD
Add R or L eye R DVD or L DVD
Near, add prime’ ET’
Examples:
Left exotropia LXT
Right hypertropia RHT
Left dissociated vertical deviation L DVD
Esophoria at near E’
Right hypotropia at distance R hypo T
Intermittent exotropia at near X(T)’
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Three items have been added to the Contents of the First Volume; they are
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found has been given, again marked off by ~ ~, and linked to the relevant
text. Any infelicities in these and other internal links are the transcriber’s
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