Bilateral sixth nerve paresis, divergence insufficiency, and bilateral

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PRIMARY AUTHOR: Marcus Myers OD, Vision Therapy and Rehabilitation Resident, SUNY
SECONDARY AUTHOR: Briana Larson OD, Vision Therapy and Rehabilitation Resident, SUNY
ABSTRACT
Bilateral type I Duane’s syndrome may present similar clinical findings to divergence insufficiency and
more ominous disorders. We discuss differential diagnosis and management of an atypical case of bilateral
type I Duane’s syndrome.
TITLE
Atypical Bilateral Type I Duane’s Syndrome: A Case Report
CASE HISTORY
A 34 year-old male complains of frequent intermittent diplopia at distance. Symptoms begin at about 18
years of age coincident with initial spectacle use. Onset has no association with trauma or illness, and
symptoms remain stable. Neurological evaluation identifies no pathology. Ocular history is significant for
ptosis surgery in the right eye several years ago. Medical history is otherwise insignificant, and the patient
reports taking no medications.
PERTINENT FINDINGS
Ocular motilities exhibit esophoria at distance and orthophoria at near. The left eye demonstrates apparent
underaction of the inferior oblique. Versions appear grossly full including abduction, although reports of
uncrossed diplopia in all cardinal positions except primary gaze indicate slight misalignment of the visual
axes. Cover test at distance finds two prism diopters of esophoria in primary gaze and four to six prism
diopters of esotropia in the other eight cardinal positions of gaze. Palpebral fissures narrow on adduction
bilaterally. Other examination findings are normal including no nystagmus.
DIFFERENTIAL DIAGNOSIS
-Lateral Rectus Paresis
Vasculopathic
Demyelinating
Compressive
Infiltrative
Inflammatory
Infectious
-Divergence Insufficiency
-Divergence Paralysis
-Duane’s Syndrome
DIAGNOSIS AND DISCUSSION
Any presentation involving esotropia or esophoria greater with distance fixation than with near fixation
indicates consideration of the myriad pathological etiologies of lateral rectus paresis. Divergence paralysis
also warrants consideration, although we agree with Jampolsky (as reviewed by Schieman et al) that many
cases of divergence paralysis may actually represent bilateral lateral rectus paresis. Divergence
insufficiency is previously diagnosed in this case. Narrowing of the palpebral apertures on adduction,
however, suggests Duane’s syndrome. This case of Duane’s syndrome is atypical in that the abduction
deficit is difficult to detect. Diplopia is also unusual, since suppression is common. Apparent underaction
of the left inferior oblique represents a form of upshooting/downshooting, a common finding in Duane’s
syndrome. Interestingly, the presentation resembles Brown’s Syndrome with Duane’s Syndrome, a
combination reported in the literature at least once. Corresponding to our case, a recent retrospective study
indicates that bilateral Duane’s syndrome may be more common in males than females. The case
presentation briefly reviews current genetic and imaging studies illuminating Duane’s syndrome.
TREATMENT/MANAGEMENT
Surgical intervention may benefit patients with Duane’s syndrome in cases of significant strabismic
deviation in primary gaze, bothersome abnormal head posture, significant upshoots or downshoots, or
severe globe retraction on adduction. Prism may relieve abnormal head posture and allow fusion in
primary gaze. Since this patient maintains fusion and exhibits near-orthophoria in primary gaze without
abnormal head posture, we consider surgery to be contra-indicated and prism to be of little benefit. The
only available treatment is vision therapy (orthoptics). We begin weekly 45-minute sessions of vision
therapy with home activities emphasizing fusional vergence in primary, secondary, and tertiary positions of
gaze. Outcomes of vision therapy are available by the case presentation in October. An abbreviated
bibliography follows.
CONCLUSION
Diagnosis of bilateral type I Duane’s syndrome requires consideration of various pathological etiologies.
This atypical case resembles bilateral lateral rectus paresis, divergence paralysis, and divergence
insufficiency. Particularly when surgery and prism are inappropriate or unhelpful, orthoptics may be
utilized as the only potential means to improve a symptomatic condition.
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