Monica Verma, MD; Kimberly G. Yen, MD


March 19, 2010

Clinical Presentation

A 2-year-old boy presented to the clinic for evaluation of restricted ocular motility. The patient's mother had noticed that, since birth, the child's right eye did not move outward fully and that it also shot upward when he looked to the side. The patient also tended to tilt his head downward, but his mother had always attributed this to him being angry. She had not appreciated any noticeable crossing or drifting of the eyes.

On examination, the child had a slight chin-down head position with a slight right head tilt. His visual acuity was central, steady, and maintained in both eyes, with alternate fixation preference. In his preferred head position, he was orthotropic. In forced primary gaze, however, he was exotropic, with his exotropia measuring 12 prism diopters for both distance and near fixation (Figure 1). His versions were significant for severe limitation of both abduction and adduction in the right eye. With attempted adduction, he had palpebral fissure narrowing and globe retraction. He also had a large upshoot and a large downshoot with attempted adduction of the right eye, with the eye becoming buried under his eyelid (Figure 2). He had no nystagmus. The pupils were equal, round, and briskly reactive to light with no afferent pupillary defect. The slit lamp and dilated fundus examinations were normal for both eyes. His cycloplegic refraction revealed moderate hyperopia with a refractive error of +4.00 +0.50 x 090 in both eyes.

Figure 1. The patient is exotropic in forced primary position.
Figure 2. Upshoot of right eye and palpebral fissure narrowing in attempted adduction.

Question 1. What is the most likely diagnosis?

A. Brown syndrome
B. Duane syndrome, type II
C. Duane syndrome, type III
D. Cranial nerve VI palsy
E. Congenital fibrosis syndrome

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Question 2. What are the indications for surgery for this condition?

A. Primary position deviations
B. Torticollis
C. Marked globe retraction
D. Large upshoots or downshoots
E. All of the above

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