Delhi Journal of Ophthalmology
Major Review
Upshoot And Downshoot In Duane’s Retraction
Syndrome: Mechanism And Treatment
Suma Ganesh
Department of Pediatric Ophthalmology and Strabismus, Dr Shroffs Charity Eye Hospital, New Delhi
Definition
Duane syndrome (DS) is a rare, congenital eye movement
disorder most commonly characterized by the inability of the
eye to turn out. The syndrome was first described by Jakob
Stilling (1887) and Siegmund Türk (1896), and subsequently
named for Alexander Duane who discussed the disorder in
more detail in 1905[1].
Clinical Features
DS is a miswiring of the eye muscles that causes some eye
muscles to contract when they should not and other eye
muscles not to contract when they should.
People with DS have abduction deficiency, narrowing of
the palpebral fissure with retraction of globe on attempted
adduction, and upshoot or downshoot, which can be the most
prominent feature[2].
Upshoot and Downshoot: Mechanism
The upshoots and downshoots are seen in 25% to 39% of
patients with Duane’s retraction syndrome[3]. Upshoots and
downshoots are classified into two types: mechanical and
innervational[4].
In the mechanical type upshoot and downshoot in DS is due
to tight lateral rectus attached to the crest of the globe, which
causes the muscle to slip off the globe when the eye is adducting.
This upshoot may look cosmetically very disfiguring even in
patients with orthtropic Duane’s syndrome. Removal of the
muscle from the crest of the globe can be achieved either by
a very large recession of the lateral rectus muscle or by Y
splitting the lateral rectus muscle. (figure 1a and b).
In the innervational type, there is gradually increasing upshot
or downshoot of the eye in the horizontal position as it moves
into adduction. This is due to co-innervation of the vertical
rectus muscle with the lateral rectus muscle. The innervational
type can be improved with recession of the appropriate vertical
rectus muscle[4].
Management
Indications for surgery
Not all patients of Duane’s Retraction Syndrome require
surgery. Indications for surgery include a significant deviation
in primary position, abnormal head posture, retraction and
Vol. 21, No. 3, January-March, 2011
narrowing of palpebral fissure, and significant upshoot or
downshoot[5].Successful surgery results in a straighter
head position, a lessening of enophthalmos and upshoot and
downshoot, and better alignment in primary position.
Surgical Options
Various surgical techniques have been employed for the
treatment of upshoot and downshoot. Eisenbaum and Parks
[6] reported performing posterior fixation suture (Faden
operation) on vertical and horizontal muscles to treat upshoot
and downshoot in patients with Duane retraction syndrome.
Stabilization of the lateral rectus muscle, however, did
produce satisfactory results with elimination of the upshoot.
Recession of both horizontal recti
Recession of both horizontal rectus muscles has been shown
to decrease the elevation and depression of the adducted eye
and improvement in retraction of the globe[7,8]. The amount
of recession of both horizontal rectus muscles, if balanced,
will not adversely effect alignment or motility[7,8]. However,
it is necessary to consider the primary position deviation when
deciding on the amount of surgical intervention. The medial
rectus muscle should be recessed relatively more than the
lateral rectus muscle in cases of esotropia in primary position
whereas in exotropia the recession of lateral rectus may be
proportionately increased. The following case illustrates
improvement with this procedure.
Case 1
A 16-year-old female presented with squinting and an
enophthalmic appearance of left eye. Unaided visual acuity
was 6/6 in both eyes. She had normal head posture .Orthoptic
examination showed she had exotropia of 12 prism diopters
with limitation of movement in adduction of left eye and
severe upshoot on adduction.
Surgery
Large recession of the medial (10-mm) & Lateral rectus
(12mm) was performed in the ipsilateral eye. Postoperatively
a left hypertropia 2 prism diopters and residual exotropia of 8
prism diopters was recorded. The enophthalmic appearance of
the eye improved and there was no upshoot during adduction.
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 Delhi Journal of Ophthalmology Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment
syndrome. The patients showed a marked decrease in upshoot
Upgaze and downshoot, without vertical deviation, after surgery.
Das et al reported marked amelioration of upshoot and
downshoot with Y-splitting of the lateral rectus muscle at the
insertion in a patient with type-I Duane retraction syndrome
with cosmetically unacceptable upshoot in adduction. The
following two cases are examples to show an improvement in
upshots and down shoot after Y split surgery.
Primary

Downgaze

Figure 1A : Severe upshoot and downshoot on adduction

Upgaze

Primary

Downgaze

Figure 2 : The bifurcation and recession of the lateral rectus

(top) for upshoot and downshoot acts by removing the muscle
from the crest of the globe, with the two recessed muscle
arms spread apart. Each muscle arms are vertically transposed
Figure 1B : Postop pictures showing improvement after Y split such that the two arms are spread apart a total of 20 mm and
each arm recessed 5-10 mm as appropriate for each case. This
Y – split of lateral rectus
ameliorates both the globe retraction and the upshoot and or
The splitting of the ends of the lateral rectus muscle into a
downshoot.
Y-configuration (figure 2a & b) is a unique idea first advocated
This figure is adapted from: Jampolsky A. Duane Syndrome
by Jampolsky[9]. The bifurcation of the muscle decreases
in Rosenbaum A L. Santiago A P. Clinical Strabismus
the upward or downward rotation of the globe because the
Management: Principles and Techniques 1999. W B Saunders
halves are positioned to stabilize the muscle’s position on
Company: 335-336.
the eye. Subsequently, Rogers and Bremer[10] performed
Y-splitting of the lateral rectus muscle along with medial
Case 2
rectus muscle recession in five patients with Duane retraction
A 12-year-old girl presented with complaints of squinting

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 Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment
since childhood. Unaided visual acuity was 6/6 OU. She did
not have abnormal head posture and there was no squint in
the primary position. However, Version tests revealed that
there was limitation of abduction of the left eye and there was
severe upshoot and down shoot on adduction.
Upshoot
Primary
Downshoot
Figure 3 A: Preop photos
Surgery
She underwent 5 mm MR recession combined with 7 mm
lateral rectus recession combined with lateral rectus Y split.
After surgery her upshoot and downshoot were corrected and
she looked much better cosmetically. Figure 3a & b
Figure 3 B: Improvement in Upshoot & Downshoot
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Delhi Journal of Ophthalmology
Case 3
A 25- year- old male came to SCEH with complaints of
abnormal head posture since childhood. Unaided visual acuity
was 6/6 in both eyes. He had face turn to the right side. He
had left exotropia of 20 prism diopters when his head was
straight. Version testing showed a limitation of abduction
of his left eye and retraction of the globe and narrowing of
the palpebral fissure on adduction was noted. A significant
upshoot and downshoot was noted in elevation and depression
in the adducted position. Fusion and stereopsis was present
with abnormal head posture and he had stereopsis of 400 arcs
second on the Titmus test.
Figure 4A : Improvement in head posture after surgery
Figure 4B : Marked upshoot in dextroversion pre operatively
Figure 4C : Improvement in upshoot post operatively
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 Delhi Journal of Ophthalmology Upshoot And Downshoot In Duane’s Retraction Syndrome: Mechanism And Treatment
Preop
The patient underwent lateral rectus recession of 8 mm
combined with a Y splitting of the lateral rectus 20 mm apart.
Post- operatively his head posture had completely resolved
but he had some residual upshoot which was not cosmetically
disturbing to patient. Figure 4 a, b & c
References
1 Duane A. Congenital deficiency of abduction associated with
impairment of adduction, retraction movement, contraction of
the palpebral fissure and oblique movements of the eye. Arch
Ophthalmol. 1905;34:133–159.
2. Das JC, Chaudhuri Z, Bhomaj S, Sharma P. Lateral rectus split
in the management of Duane’s retraction syndrome. Ophthalmic
Surgery and Lasers 2000;31:499-501
3. Mohan K, Saroha V, Sharma A. Factors predicting upshoots
an downshoots in duane retraction syndrome. J Pediatric
Ophthalmol Strabismus
4. Kraft SP. Surgical approach for Duane syndrome. J Pediatric
Ophthalmol strabismus1988;25 : 119- 130
5. Gobin MH. Surgical management of Duane’s syndrome. Br J
Ophthalmol. 1974;58:301–306.
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6. Eisenbaum AM, Parks MM. A study of various surgical
approaches for the leash effect in Duane’s syndrome. Presented
at the joint session of the American Association for Pediatric
Ophthalmology and Strabismus and the American Academy of
Ophthalmology, Chicago, IL, November 5, 1989
7. von Noorden GK. Recession of both horizontal recti muscles in
Duane’s retraction syndrome with elevation and depression of
the adducted eye. Am J Ophthalmol. 1992;114:311–313.
8. Sprunger DT. Recession of both horizontal rectus muscles in
Duane’s syndrome with globe retraction in primary position. J
Pediatr Ophthalmol Strabismus. 1997;1:31–33.
9. Jampolsky A. Discussion of Eisenbaum AM, Parks MM. A
study of various surgical approach to the leash effect in Duane’s
syndrome. Presented at the joint session of the American
Association for pediatric ophthalmology and strabismus and the
American Academy of Ophthalmology, Chicago, IL, November
5, 1980
10. Rogers GL, Bremmer DL. Surgical treatment of the upshoot and
downshoot in Duane’s retraction syndrome. Ophthalmology.
1984;91:1380–1382
11. Feretis D. Papastratigakis B, Tsanparlakis J. Planning surgery in
Duane’s retraction syndrome. Ophthalmologica. 1981;183:148–
153.
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