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 narrowing of palpebral fissure, and significant upshoot or

Duane syndrome (DS) is a rare, congenital eye movement downshoot[5].Successful surgery results in a straighter
disorder most commonly characterized by the inability of the head position, a lessening of enophthalmos and upshoot and
eye to turn out. The syndrome was first described by Jakob downshoot, and better alignment in primary position.
Stilling (1887) and Siegmund Türk (1896), and subsequently
named for Alexander Duane who discussed the disorder in Surgical Options
more detail in 1905[1]. Various surgical techniques have been employed for the
treatment of upshoot and downshoot. Eisenbaum and Parks
Clinical Features [6] reported performing posterior fixation suture (Faden
DS is a miswiring of the eye muscles that causes some eye operation) on vertical and horizontal muscles to treat upshoot
muscles to contract when they should not and other eye and downshoot in patients with Duane retraction syndrome.
muscles not to contract when they should. Stabilization of the lateral rectus muscle, however, did
People with DS have abduction deficiency, narrowing of produce satisfactory results with elimination of the upshoot.
the palpebral fissure with retraction of globe on attempted
adduction, and upshoot or downshoot, which can be the most Recession of both horizontal recti
prominent feature[2]. Recession of both horizontal rectus muscles has been shown
to decrease the elevation and depression of the adducted eye
Upshoot and Downshoot: Mechanism and improvement in retraction of the globe[7,8]. The amount
The upshoots and downshoots are seen in 25% to 39% of of recession of both horizontal rectus muscles, if balanced,
patients with Duane’s retraction syndrome[3]. Upshoots and will not adversely effect alignment or motility[7,8]. However,
downshoots are classified into two types: mechanical and it is necessary to consider the primary position deviation when
innervational[4]. deciding on the amount of surgical intervention. The medial
In the mechanical type upshoot and downshoot in DS is due rectus muscle should be recessed relatively more than the
to tight lateral rectus attached to the crest of the globe, which lateral rectus muscle in cases of esotropia in primary position
causes the muscle to slip off the globe when the eye is adducting. whereas in exotropia the recession of lateral rectus may be
This upshoot may look cosmetically very disfiguring even in proportionately increased. The following case illustrates
patients with orthtropic Duane’s syndrome. Removal of the improvement with this procedure.
muscle from the crest of the globe can be achieved either by
a very large recession of the lateral rectus muscle or by Y Case 1
splitting the lateral rectus muscle. (figure 1a and b). A 16-year-old female presented with squinting and an
In the innervational type, there is gradually increasing upshot enophthalmic appearance of left eye. Unaided visual acuity
or downshoot of the eye in the horizontal position as it moves was 6/6 in both eyes. She had normal head posture .Orthoptic
into adduction. This is due to co-innervation of the vertical examination showed she had exotropia of 12 prism diopters
rectus muscle with the lateral rectus muscle. The innervational with limitation of movement in adduction of left eye and
type can be improved with recession of the appropriate vertical severe upshoot on adduction.
rectus muscle[4].
Surgery
Management Large recession of the medial (10-mm) & Lateral rectus
Indications for surgery (12mm) was performed in the ipsilateral eye. Postoperatively
Not all patients of Duane’s Retraction Syndrome require a left hypertropia 2 prism diopters and residual exotropia of 8
surgery. Indications for surgery include a significant deviation prism diopters was recorded. The enophthalmic appearance of
in primary position, abnormal head posture, retraction and 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 Delhi Journal of Ophthalmology
since childhood. Unaided visual acuity was 6/6 OU. She did Case 3
not have abnormal head posture and there was no squint in A 25- year- old male came to SCEH with complaints of
the primary position. However, Version tests revealed that abnormal head posture since childhood. Unaided visual acuity
there was limitation of abduction of the left eye and there was was 6/6 in both eyes. He had face turn to the right side. He
severe upshoot and down shoot on adduction. had left exotropia of 20 prism diopters when his head was
straight. Version testing showed a limitation of abduction
Upshoot 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.
Primary

Downshoot

Figure 4A : Improvement in head posture after surgery

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 4B : Marked upshoot in dextroversion pre operatively

Figure 3 B: Improvement in Upshoot & Downshoot 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 6. Eisenbaum AM, Parks MM. A study of various surgical
The patient underwent lateral rectus recession of 8 mm approaches for the leash effect in Duane’s syndrome. Presented
combined with a Y splitting of the lateral rectus 20 mm apart. at the joint session of the American Association for Pediatric
Post- operatively his head posture had completely resolved Ophthalmology and Strabismus and the American Academy of
but he had some residual upshoot which was not cosmetically Ophthalmology, Chicago, IL, November 5, 1989
disturbing to patient. Figure 4 a, b & c 7. von Noorden GK. Recession of both horizontal recti muscles in
Duane’s retraction syndrome with elevation and depression of
References the adducted eye. Am J Ophthalmol. 1992;114:311–313.
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Ophthalmol Strabismus 10. Rogers GL, Bremmer DL. Surgical treatment of the upshoot and
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