A 3-Year-Old Girl Whose Eyes Appear Misaligned

Zane D. Foster; Kimberly G. Yen, MD; Madhuri Chilakapati, MD

Disclosures

July 26, 2019

Case Diagnosis

This patient presented with an inability to elevate the left eye on adduction, the characteristic finding of Brown syndrome. In this syndrome, structural abnormalities involving the superior oblique muscle and trochlea limit elevation of the affected eye in adduction.

Duane syndrome, a congenital cause of strabismus, is divided into three types, with type 1 being the most common. Type 1 is characterized by a partial or complete inability to abduct the eye and is also commonly associated with an upshoot or downshoot of the eye in adduction. This patient had intact abduction and adduction yet was unable to elevate the left eye in adduction.

The fourth cranial nerve innervates the superior oblique muscle. A palsy of this nerve results in hypertropia that worsens with adduction of the affected eye. Torticollis may be noted when the head is tilted away from the affected eye to compensate for the hypertropia. This patient's left eye had an inability to elevate rather than an overaction in adduction, and she had a face turn as opposed to a head tilt.

Moebius syndrome is a genetic disorder characterized by paralysis of cranial nerves VI and VII; less often, other cranial nerves may be affected. This would manifest as an esotropia with an abduction deficit. Patients can have other signs and symptoms, including lagophthalmos, deafness, thoracic muscle weakness, tongue atrophy, limb abnormalities, mental developmental delay, and autism. This patient did not have esotropia or an abduction deficit and was otherwise healthy.

The third cranial nerve innervates all of the extraocular muscles except the superior oblique (cranial nerve IV) and the lateral rectus (cranial nerve VI), meaning it controls elevation, depression, adduction, and torsion of the eye. It also innervates the levator palpebrae superioris and carries parasympathetic fibers to the pupillary sphincter. A complete palsy will result in ptosis, deviation of the eye downward and outward, and dilation of the pupil if the parasympathetic fibers are involved. A partial third cranial nerve palsy presents with a variable degree of ptosis, motility limitation based upon the affected extraocular muscles, and possible pupil dysfunction. This patient did not have any of these characteristics.

Clinical Course

Given that this patient had a minimal face turn, no deviation in primary gaze, and equal vision between the two eyes, she was seen for follow-up appointments in the clinic.

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