The Infant Who Keeps Turning His Face Leftwards

Kimberly G. Yen, MD; Ariel Chen

Disclosures

March 14, 2018

Discussion

Children can present with an anomalous head posture (AHP) due to ocular, orthopedic, or neurologic etiologies.[1] AHP may present as a head tilt, face turn, chin up, chin down, or any other combination thereof, which are adopted in an attempt to improve visual acuity or binocularity.[2]

The most common cause of ocular AHP is incomitance from such ocular motility problems as superior oblique muscle palsy, sixth nerve palsy, and Duane syndrome.[1] These patients commonly use an anomalous head position to place the eyes in a position where they are best aligned or to prevent diplopia.[2]

One of the most common causes of incomitance in young children is superior oblique muscle palsies,[3,4] which are either acquired or congenital. Acquired cases may occur due to head trauma, vascular lesions, or tumors. Patients often present with a contralateral head tilt toward the side of the unaffected eye or hypertropia of the affected eye. Clinicians should be aware that superior oblique palsies may be bilateral when examining patients. To diagnose superior oblique palsies, a Bielschowsky test can be performed; the patients will have increased hypertropia when the head is tilted to the affected side. In bilateral cases, patients have right hypertropia when the head is tilted to the right and left hypertropia when the head is tilted to the left.[2]

Sixth nerve palsy also may be congenital or acquired. These cases may occur due to intracranial lesions, trauma, postinfectious, or immunologic etiologies, and patients present with a face turn toward the side of the affected eye.[2]

Duane syndrome is congenital and occurs due to poor development of the motor neurons innervating the lateral rectus muscle.[5] It may be associated with palpebral fissure narrowing and globe retraction on adduction, as well as a compensating face turn.[2]

If a patient has an AHP due to ocular motility problems, occlusion of one eye briefly in clinic, also known as the patch test, can be performed to help determine if a strabismus is the cause. If the AHP improves with the patch, the patient has a higher likelihood of an ocular etiology. Nonocular causes of AHP such as congenital shortening of neck muscles, cerebral palsy, or occipitocervical synostosis would not improve with patching of either eye. However, no improvement with patching does not exclude ocular causes.[6]

Another cause of ocular AHP is a compensating face turn due to the presence of a null point in patients with nystagmus. These patients may turn their head to obtain a position of gaze where there is the least amount of eye movement and the vision is usually the best.[2,7]

Refractive error should be considered as a cause of ocular AHP,[3,8,9] although the mechanism for this is not well understood and may be due to an attempt to align with the meridian of astigmatism or a pinhole/squinting effect.[2,3] A cycloplegic refraction should always be performed when evaluating children presenting with AHP.

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