Eye (2015) 29, 184–190

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Pattern strabismus R Kekunnaya1, T Mendonca1 and V Sachdeva2

CAMBRIDGE OPHTHALMOLOGICAL SYMPOSIUM

and torsion needs

special surgical
attention

Abstract deviation is minimal. However, AHP may be

absent if there is a large deviation in primary
Pattern strabismus is relatively common in
position. Patients often complain of asthenopia
strabismus practice. Although it is classically
and double vision, especially if the deviation is
used to include A and V patterns, the term
more in downgaze.
has been expanded to include additional
Clinical workup of patients with pattern
vertically incomitant horizontal strabismus.
strabismus includes: (1) measurement of
This article reviews the clinical features,
deviation in nine gaze with accommodation
etiopathogenesis, and surgical options for the
being controlled; (2) measurement of deviation
patients with pattern strabismus.
in 251 of upgaze (chin depression) and 351 of
Eye (2015) 29, 184–190; doi:10.1038/eye.2014.270;
downgaze (chin elevation); (3) careful
published online 21 November 2014
assessment of overelevation and overdepression
in adduction; and (4) objective assessment of
Introduction fundus torsion with indirect ophthalmoscopy or
The term pattern strabismus denotes a fundus photography.
significant difference in the size of horizontal
deviations in defined positions of upgaze and
Uncommon subtypes of pattern strabismus
downgaze. Classically, it includes A and V
1
Jasti V Ramanamma patterns, where by convention the difference Y pattern exists when the change in deviation is
Children’s Eye Care Center,
between upgaze and downgaze must be 410 minimal between primary and downgaze, and
L V Prasad Eye Institute, KAR
campus, Hyderabad, India prism diopters (PD) and 15 PD for A and V occurs only in upgaze. It is highly characteristic
patterns, respectively. However, in recent years, of bilateral inferior oblique overaction seen in
2
Nimmagadda Prasad this term has been expanded to include the congenital esotropia or exotropia. It can also be
Children’s Eye Care Center, other patterns with significant differences in the seen in Duane retraction syndrome (DRS) and
L V Prasad Eye Institute, primary gaze and up/downgaze such as Y, X, Brown syndrome (Figures 1a and b).1 This
GMRV Campus,
arrow, and lambda patterns. A lesser degree of pattern of strabismus offers advantage for
Visakhapatnam, India
difference is accepted for A pattern, because binocular vision, especially if the primary
Correspondence: convergence is more essential in downgaze. The position deviation is minimal.
R Kekunnaya, Head, Jasti V exact mechanism of pattern strabismus is poorly X pattern is seen in long-standing exotropia and
Ramanamma Children’s Eye understood even today. Cyclotorsion is a type 3 DRS patients. In both situations, a tight
Care Center, Consultant, common feature of pattern strabismus and is lateral rectus muscle causes a leash effect and leads
Pediatric Ophthalmology,
also proposed to be one of the etiological factors to increased divergence in both up and downgaze.
Strabismus and
Neuroophthalomology, for A and V patterns. Various surgical options Overaction of all four oblique muscles was shown
L V Prasad Eye Institue, for pattern strabismus include procedures on to be the cause for X pattern by Brandner et al2
Kallam Anji Reddy Campus, superior/inferior oblique muscles if there is using computer-based simulation model.
L V Prasad Marg, Banjara associated oblique dysfunction or transposition Arrow pattern occurs when maximum
Hills, Hyderabad 500034,
procedures on the rectus muscles in its absence. convergence occurs in between the primary and
India
Tel: +91 40 3061 2632; downgaze. This is seen in cases of bilateral
Fax: +91 40 2354 8271. superior oblique palsy.
E-mail: rameshak@lvpei.org Clinical features
Lambda pattern is the opposite of Y pattern
or drrk123@gmail.com Presence of pattern strabismus may result in an wherein, maximum divergence occurs between
abnormal head posture (AHP) to maintain primary and downgaze. Such a pattern is seen
Received: 1 October 2014
fusion. For example, a patient with V pattern with bilateral superior oblique overaction3 or
Accepted: 6 October 2014
Published online: 21 exotropia may adopt a chin elevation (Table 1) inferior rectus underaction (over-recessed or
November 2014 to keep the eye in downgaze where the slipped muscle).
 Pattern strabismus, A pattern, V pattern
R Kekunnaya et al
185

Etiopathogenesis An imbalance of the cyclovertical muscles was also

proposed to be one of the mechanisms for pattern
In 1897, Duane described V pattern in a patient with
strabismus. It was thought that, while an A pattern
bilateral superior oblique palsy.4 Measurements of
occurs when inferior rectus underacts and yoke superior
deviation in up and downgaze was popularised by
oblique overacts, a V pattern is noted when superior
Urrets-Zavalia5 in 1948. The worldwide accepted term of
rectus underacts and yoke inferior oblique overacts.10
A and V patterns was coined by Urist.6 The role of
This theory has also been abandoned due to lack of
oblique muscle dysfunction was described by Knapp7 in
adequate evidence.
1959. He also recommended surgeries on oblique
muscles to treat A and V patterns.
About 12.5–50% of the patients with horizontal
strabismus may have coexisting A and V patterns.3–5 Oblique muscle overaction or underaction
Spina bifida was associated with A pattern in 31% of the
This theory is most popular and widely accepted and
cases.8
was put forward by Knapp7 in 1959. It is based on the
Etiology of pattern strabismus has evolved through
fact that abduction is the tertiary action of oblique
many theories. Though there are various schools of
muscles. Thus a primary or secondary overaction of the
thought, there is no unanimity regarding the exact
inferior oblique muscle leads to increased abduction,
pathophysiology of pattern strabismus. Proposed
especially when the eye is in elevation. This leads to a V
etiological mechanisms can be mechanical (peripheral)
pattern. Vice versa is seen when the superior oblique
and neural (central).9
muscles are overacting. In clinical practice, usually this
type of oblique muscle dysfunction is observed to be
Mechanical factors associated with pattern strabismus.
Classification of eye movement abnormalities and
According to the theory of horizontal muscle overaction,6
strabismus (CEMAS)—a national Eye Institute sponsored
an imbalance between the actions of the rectus muscles
workshop referred superior oblique overaction as
leads to pattern strabismus, for example the medial
overdepression in adduction and inferior oblique
rectus muscles overact in downgaze and lateral rectus
overaction as overelevation in adduction.Therefore,
muscles overact in upgaze giving rise to a V esotropia.
‘overelevation/overdepression in adduction’ is a
Conversely, an A pattern arises when the medial rectus
generalized term where oblique dysfuntion is one of the
muscle underacts in downgaze and lateral rectus muscle
causes for the same.
underacts in upgaze. However, this theory has been
disproved by electromyographic studies that showed
that electrical activity of horizontal rectus is similar in
pattern strabismus and other incomitant strabismus. Anomalies of orbital structures
Anatomical factors like craniofacial anomalies,
Table 1 Showing AHP in A and V pattern
hydrocephalus, and heterotopy of muscle pulleys
Chin up posture A esotropia, V exotropia
demonstrate pattern strabismus owing to abnormal
Chin down posture A exotropia, V esotropia
vector force. Patients with mongoloid slant are seen to

Figure 1 Preoperative (a) and postoperative (b) photographs of a patient who had Y pattern due to Brown syndrome, who underwent
superior oblique tenotomy.

Eye
 Pattern strabismus, A pattern, V pattern
R Kekunnaya et al
186

have A esotropia and V exotropia, whereas patients with pattern strabismus and dissociated vertical deviation
antimongoloid slant have V esotropia and A exotropia.11 (DVD).
The ‘theory of sagittalisation of oblique muscles’ as a
cause of pattern strabismus was put forward by Gobin12
Could A pattern strabismus be a special form of skew
According to this theory, reduction in angle between
deviation?
visual axis and muscle axis results in cyclophoria that is
compensated by contraction of respective cyclovertical Donahue and Itharat17 reported 13 cases of A pattern
muscles, thereby resulting in overelevation or strabismus with neurologic abnormalities like
overdepression in adduction. hydrocephalus, spina bifida, perinatal stroke, and global
Pseudoparalysis of superior oblique and V pattern developmental delay. In all, 11 out of 13 patients had no
may be seen in plagiocephaly owing to shallow orbit on difference in vertical misalignment during right and left
affected side.13 Similarly pseudo A pattern may be seen head tilts. Hence, they proposed that in A pattern
in patients with hydrocephalus.8 strabismus, damage to utricular pathways resulted in
decrease in anterior semicircular canal input and increase
Abnormalilties of extraocular muscle (EOM) pulleys in posterior semicircular canal input that causes
increased bilateral depressor tonus of superior oblique
EOM pulleys are condensations of posterior tenon’s
and inferior rectus muscles. However, the superior
capsule composed of collagen, elastin, and smooth
oblique muscle is an incyclotorter and the inferior rectus
muscles, which act as the functional origin of EOMs.
muscle is an excyclotorter, which should nullify the
Muscle pulleys are thought to minimize sideslip relative
torsion effects of each other when there is an increase in
to orbit during globe rotations. Clark et al14 have studied
bilateral depressor tonus.
EOM pulley positions using high-resolution MRI. They
have shown that small mislocations (o2 mm) of rectus
muscle pulleys can result in incomitance in vertical gaze. Ocular torsion as a cause of pattern strabismus
They found inferior displacement of the lateral rectus
Association of excyclotorsion with V pattern and
pulley is associated with apparent overelevation in
incyclotorsion with A pattern was first reported by
adduction, whereas superior displacement of the same
Piper18 in 1963. Weiss19 used campimetric measurements
causing apparent overdepression in adduction. They
of position of blind spot to confirm ocular torsion. He
concluded that pulley heterotopy can itself cause
postulated that apparent rotation of insertion of rectus
incomitance without dysfunctional oblique muscles,
muscles due to excyclotorsion of the globe is the
hence resulting in A or V pattern strabismus.
etiological factor for pattern strabismus. For example, if a
patient has a V pattern with inferior oblique muscle
Neural mechanisms
overaction, each eye will be extorted. This will rotate the
Several neural mechanisms have been proposed to insertions of the rectus muscles counterclockwise in the
explain pattern strabismus. Miller and Guyton15 studied right eye and clockwise in the left eye. An abducting
pre- and postoperative courses of patients who were force will be created from superior rectus in upgaze and
overcorrected after surgery for intermittent exotropia. adducting force from inferior oblique in downgaze,
About 43% patients with consecutive esotropia, vs only which will result in a V pattern. In addition, the medial
5% controls, developed A or V pattern strabismus at 28 rectus muscle acts like a partial elevator and lateral
months after surgery. The authors say that loss of rectus muscle acts like a partial depressor in abduction,
fusional control leads to torsional drift, which is similar which will exacerbate the V pattern. This complex action
to exotropic drift seen in sensory strabismus. Torsional of all EOMs was also supported by an investigation
drift may cause altered vector forces of vertical rectus conducted by Kushner20
muscles, which may lead to pattern strabismus. Guyton21 proposed that loss of fusion predisposes the
Abnormal supranuclear circuits have been postulated oculomotor system to cyclodeviations of the eyes, which
as one of the neural causes for pattern strabismus. In an in turn causes A and V patterns according to the
animal study, Das and Mustari16 have studied burst mechanism proposed by Weiss. Ocular torsion has been
neuron activity from oculomotor nucleus in three attributed as a major etiological factor for pattern
juvenile rhesus monkeys with A pattern exotropia as strabismus by the author. He also introduced the term
they performed horizontal or vertical smooth pursuit sensory torsion.22 Similarly, Miller and Guyton15 showed
during monocular viewing. The authors concluded that occurrence of sensory torsion in patients with subnormal
in animal models with sensory-induced strabismus, binocularity resulting in alteration of vector forces of
innervation to EOMs from motor nuclei produce the horizontal rectus muscles, which in turn results in
inappropriate cross-axis eye movements, resulting in pattern strabismus.

Eye
 Pattern strabismus, A pattern, V pattern
R Kekunnaya et al
187

Fundus torsion is also described as a marker for palsy and extorsion in a study conducted by Clark et al.28
subsequent development of inferior oblique overaction in However, there was no displacement of other EOM
cases of infantile esotropia by Eustis and Nussdorf.23 The pulleys that suggests torsion may not be the primary
authors say that inferior oblique overaction developed in cause of pulley heterotopy.28
every child who showed fundus extorsion and it is
suggestive of a 100% positive predicitve value for the same.
Surgical implications
Good correlations were found between fundus
intorsion, superior oblique muscle overaction, and A It is important to correct pattern deviations along with
patterns; and between fundus extorsion, inferior oblique correction of horizontal deviations if it is clinically
muscle overaction, and V patterns in a study conducted significant.
by Deng et al.24 The authors also say that correlations Apart from measurement of deviation in all diagnostic
increased in the absence of stereopsis. positions of gazes, workup of patient should also include
Early onset strabismus may lead to sensorial measurement of vertical deviation (hyper/hypotropia in
adapatations such as suppression, cylcofusion, or adduction) and careful search for the muscle insertion
anomalous retinal correspondence that may not reveal any during slit lamp examination. Accomodative esotropia
torsion on subjective methods of assessment. Correlation and increased AC/A ratio may simulate pseudo V
between subjective and objective torsion was studied by pattern. Imaging of the orbit may be considered in
Kushner and Hariharan25 and the authors claim that better selected cases where pattern cannot be attributed to any
correlation exists in patients with late onset strabismus or of the ocular motility findings and also in unusual cases.
with good stereopsis. They also observed that patients with The most popular surgical modalities to treat pattern
early onset strabismus with absent binocularity may not strabismus include weakening of oblique muscles and
experience subjective torsion.25 vertical transpositions of horizontal muscles. Other
An experimental study published by Kushner26 surgical modalities described in literature are insertion
evaluated whether ocular torsion is a primary factor for slanting procedures and horizontal transposition of
the development of pattern strabismus, or is just a vertical rectus muscles. Introperatively, one may inspect
contributing factor. He studied trajectory of eye the course of horizontal rectus muscle course, which may
movements in five patients with oblique muscle provide additional clues for the surgical management.
overaction and found that for all five patients elevation
or depression of the eye was curvilinear as the eye was
Oblique muscle surgery
moved from abduction to adduction owing to primary
oblique muscle overaction. The second investigation was When vertical deviation is greatest in adduction
on two patients with unilateral superior oblique palsy (overelevation in adduction), primary oblique muscle
who underwent Harada–Ito procedure for torsional overaction could be the commonest cause. Primary
diplopia, which was abolished after surgery. However, inferior oblique overaction is usually associated with V
patients persisted to have significant inferior oblique pattern and extorsion. Similarly, primary superior
overaction. The third investigation quantified increase in oblique muscle overaction is associated with A pattern
fundus torsion after horizontal muscle transposition for and intortion. The amount of anatomic torsion usually
pattern strabismus in five patients whereas the pattern correlates with the amount of oblique muscle overaction.
collapsed after surgery. The mean increase in fundus Ocular torsion is said to be a more reliable indicator of
torsion was 6.41 with a range of 5–71. Hence the authors oblique muscle overaction than versions.29
concluded that ocular torsion may not be the primary Oblique muscle weakening is appropriate in cases of
etiological factor for pattern strabismus. pattern deviations with oblique muscle overaction as it
Sharma et al27 studied pre- and postoperative torsion decreases torsion, which may contribute to the pattern.29
subjectively by synoptophore and objectively by fundus Primary position deviation does not get affected by
photography in 21 patients who underwent monocular surgery on oblique muscle. Hence, horizontal muscle
surgery with horizontal rectus transposition for pattern surgery should be planned according to horizontal
strabismus. They also demonstrated worsening of torsion deviation in primary position. Thus, V esotropia or
as well as occurrence of new onset torsion after exotropia with overelevation in adduction requires
horizontal muscle surgery. inferior oblique weakening with horizontal muscle
It has been argued that ocular torsion may lead to surgery. Similarly, horizontal muscle surgery with
heterotopy of orbital pulleys and displacement of pulleys posterior tenotomy of superior oblique can be done for A
may result in altered vector forces in the presence of pattern with overdepression in abduction. The decision
cyclotorsion.22 Upward displacement of medial rectus of posterior tenotomy can be titrated based on forced
pulley alone was found in patients with superior oblique traction test for superior oblique.

Eye
 Pattern strabismus, A pattern, V pattern
R Kekunnaya et al
188

The various weakening procedures available for inferior horizontal muscle is transposed, its primary action is
oblique are recession, myectomy and anterior decreased while it gains a new action in the direction of
transposition. Anterior transposition is preferred in the transposition. Thus when a medial rectus muscle is
presence of a DVD or in very severe overaction. The shifted downwards it gains a depressor action, which is
choice between myectomy and recession varies between maximum in the downgaze and less in the upgaze
surgeons. Most of these procedures reduce the exotropia (Figures 2a and b). The anticipated decrease in the
and increase esotropia in upgaze to an extent of 15–25 PD. horizontal action of the muscle determines the direction
Many surgeons prefer posterior tenotomy of superior of the transposition of the muscle. Thus, the insertion of a
oblique muscle as it preserves the anterior fibers that are muscle should be moved in the direction in which it is
responsible for intorsion and thereby prevents unwanted desirable to most decrease its horizontal action and in the
postoperative cyclotorsion, especially in patients with direction opposite that in which one wishes its horizontal
good fusion. action to be more effective. Hence in the case of a V
A study by Yu et al30 compared fundus extorsion before esotropia, an inferior transposition of the medial rectus
and after inferior oblique myectomy using fundus corrects increase in the horizontal deviation in
photography combined with horizontal muscle surgery downgaze. Table 2 shows various types of pattern
for horizontal deviations. They found significant strabismus and possible combination of surgeries for the
reduction in excyclotorsion after inferior oblique treatment and Figure 3 shows direction of transposition
myectomy. Similarly, Wu et al31 showed significant of horizontal rectus muscles.
reduction in intorsion after weakening of superior oblique. Kushner,32 in a retrospective case series, illustrated
occurrence of ocular torsion after transposition of
horizontal muscles for pattern strabismus in three
Vertical offset of horizontal rectus muscles
patients and development of pattern strabismus after
In cases where the oblique muscles are not overacting, vertical rectus transposition for ocular torsion in five
transposition of horizontal rectus muscles is another patients. Hence, he states that horizontal muscle
effective surgery to collapse pattern strabismus. When a transposition surgery can have adverse effect on torsion

Figure 2 Preoperative (a) and postoperative (b) photographs of a patient who had A esotropia, who underwent bilateral medial
rectus recession with superior transposition.

Eye
 Pattern strabismus, A pattern, V pattern
R Kekunnaya et al
189

Table 2 Shows various types of pattern strabismus and possible surgeries

Pattern Surgery

V esotropia/exotropia with inferior oblique overaction Horizontal muscle surgery with inferior oblique weakening
A esotropia/exotropia with superior oblique overaction Horizontal muscle surgery with Superior oblique weakening
V esotropia without oblique muscle overaction Bimedial recession with downshift
V exotropia without oblique muscle overaction Bilateral lateral rectus recession with upshift
A esotropia without oblique muscle overaction Bimedial recession with upshift
A exotropia without oblique muscle overaction Bilateral lateral rectus recession with downshift

Conclusions
MR MR In conclusion, pattern strabismus is a relatively common
condition. The exact pathophysiology behind occurrence
MR MR
LR LR LR
of pattern strabismus is still an enigma. In most cases
LR where an associated oblique overaction can be clinically
determined, it may be believed to be causative. Careful
Figure 3 Direction of transposition of horizontal rectus
muscles.
attention should be paid to associated craniofacial
abnormality or orbital asymmetry, which may be
contributory in specific cases. Table 2 summarizes the
if it is done for pattern and vice versa. As five out of
possible surgical options. Although many options are
eight patients had Grave’s orbitopathy, the author
described, surgical management depends on presence or
hypothesizes that torsion or alphabet pattern could have
absence of oblique overaction. However, in the author’s
been more owing to tight muscles. Also, patients who
experience, weakening of oblique muscles usually gives
underwent surgery for torsion had bilateral surgery,
satisfactory results in cases of oblique muscle overaction
which could be having an added effect on the
with pattern strabismus.
development of torsion.
A monocular recession/resection procedure with
Conflict of interest
vertical transposition is also commonly practiced for
pattern strabismus. Worsening of existing torsion or The authors declare no conflict of interest.
occurrence of new torsional changes has been studied by
Sharma et al.27 They compared pre- and postoperative
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