Ocular torticollis is an abnormal head posture (AHP) adopted as a compensatory mechanism for eye abnormalities. It refers to a condition where a specific head position is preferred to optimize vision or maintain binocular vision.
Abnormal head posture is broadly classified into the following components2).
Combinations of these can also occur. The incidence in pediatric ophthalmology practice is reported to be about 3%. It usually appears early after birth and may become more noticeable as the visual system matures.
The causes of torticollis are broadly divided into three categories: ocular, neurological, and orthopedic. Among 63 cases of abnormal head posture identified during routine pediatric checkups, ocular causes were confirmed in 25 cases (39.7%), highlighting the importance of ophthalmologic evaluation.
The incidence in pediatric ophthalmology practice is about 3%. Approximately 40% of children with abnormal head posture are found to have an ocular cause.
Children with ocular torticollis compensate for visual function by adopting a specific head posture, so they are often unaware of the symptoms. It is usually the parent who notices the abnormal head position.
The pattern of abnormal head posture varies depending on the underlying disease. The main abnormal head postures and their associated eye diseases are shown below.
| Pattern of Abnormal Head Posture | Main Underlying Disease |
|---|---|
| Head tilt | Superior oblique palsy, DVD, Inferior oblique palsy |
| Facial turn | Duane syndrome, nystagmus, A-V pattern strabismus |
| Chin up/chin down | Brown syndrome, superior oblique palsy, monocular elevation deficiency |
In superior oblique palsy, patients often adopt an abnormal head posture with the chin down, face turned toward the unaffected side, and head tilted toward the unaffected side. In patients with hypertropia, head tilt accounts for 62.8%, facial turn 19.3%, and chin up 16.4% 2).
Facial asymmetry is closely related to long-standing ocular torticollis. It presents a pattern where the line connecting both eyes and the line connecting the mouth corners intersect on the unaffected side. It also serves as an indicator to differentiate congenital from acquired superior oblique palsy.
In studies of patients with ocular torticollis, facial compression (compression or mass reduction of the frontal, maxillary, and mandibular regions) ipsilateral to the head tilt/rotation was confirmed in most cases where photographic evaluation was possible2).
Long-term abnormal head posture carries a risk of causing facial asymmetry and scoliosis in growing children, in addition to neck muscle tension. Early diagnosis and treatment to improve head posture are important.
The causes of ocular torticollis are diverse, but the most common are incomitant strabismus and nystagmus. In a prospective study of 188 cases, incomitant strabismus accounted for 62.7% and nystagmus for 20.2%. A large study of 630 cases confirmed a similar trend, with 330 cases (52.4%) of incomitant strabismus and 120 cases (19%) of nystagmus. Among incomitant strabismus, A-V pattern strabismus was the most common with 116 cases (35.2%), followed by superior oblique palsy with 59 cases (17.9%).
Ocular Causes
For visual improvement: nystagmus (compensation to null point), ptosis (chin elevation to secure visual axis), refractive error (especially oblique astigmatism), homonymous hemianopia (placing residual visual field in front)
Ocular motility disorders: superior oblique palsy, Duane syndrome, A-V pattern strabismus, Brown syndrome, third and sixth cranial nerve palsy, thyroid eye disease, dissociated vertical deviation (DVD), ocular tilt reaction
Non-ocular causes
Orthopedic: congenital muscular torticollis (CMT), trauma, inflammatory myositis, skeletal abnormalities (Klippel-Feil syndrome, plagiocephaly, cervical subluxation)
Neurological: syringomyelia, focal dystonia, brain tumor, post-inflammatory sequelae of central nervous system, unilateral hearing loss
This is one of the most important causes of ocular torticollis. It is classified into three types: congenital, idiopathic, and acquired.
When CMT is confirmed by ultrasound, other causes tend to be excluded, but ocular torticollis may coexist 1).
Kim et al. reported a case of a 3-month-old boy diagnosed with CMT whose abnormal head posture did not improve after 7 months of physical therapy. Ophthalmic examination at 10 months revealed left fourth cranial nerve aplasia and left superior oblique hypoplasia, and left inferior oblique myectomy at 15 months completely resolved the torticollis 1).
Incomitant strabismus accounts for 52–63% of cases, followed by nystagmus at 19–20%. Among incomitant strabismus, A-V pattern strabismus and superior oblique palsy are most frequent.
Ocular torticollis is diagnosed through medical history taking and a detailed neuro-ophthalmological examination. It is essential to perform ocular alignment tests with the head in the correct position, as abnormal head posture can mask strabismus. In infants, asking parents about the child’s usual head position preference can also be helpful.
| Test method | Purpose |
|---|---|
| Parks three-step test | Identification of the paretic muscle |
| Hess red-green test | Quantitative assessment of ocular motility abnormalities |
| Large synoptophore | Evaluation of nine gaze positions |
| MRI/CT | Differentiation between congenital and acquired |
| Forced duction test | Assessment of superior oblique muscle laxity |
| Stereopsis test | Evaluation of binocular function |
Parks 3-step test (Parks-Bielschowsky three-step test) is a basic procedure for identifying the paretic muscle in vertical strabismus.
In mild superior oblique palsy, excyclotorsion in the tertiary gaze position on the major amblyoscope may be the key to diagnosis. In congenital superior oblique palsy, MRI shows more severe abnormalities of muscle insertion or hypoplasia than in acquired cases.
If the following findings are present, actively investigate non-muscular causes1).
If the abnormal head posture disappears when one eye is covered, an ocular cause is suggested. Additionally, worsening of symptoms when gazing at an object while seated may also indicate an ocular cause. Lack of response to physical therapy is an important red flag for non-muscular causes, including ocular causes.
The basic treatment is intervention for the underlying disease causing ocular torticollis. Early diagnosis and correction of the underlying condition are important to prevent secondary musculoskeletal changes such as asymmetry of the neck and face.
In cases where binocular vision is maintained by ocular torticollis, binocular function is often good. However, long-term neglect can lead to facial asymmetry and scoliosis, so surgery is performed to improve head posture.
In addition to horizontal rectus muscle surgery, vertical transposition of the horizontal rectus muscle insertion (Trick procedure) is performed concurrently.
Surgery is considered when vertical deviation is noticeable in daily life. Surgical options include superior rectus recession and anterior transposition of the inferior oblique, but a definitive procedure has not been established at present.
The main purpose of nystagmus surgery is to improve abnormal head posture by moving the null point (the gaze position where nystagmus is minimized) to the front. Identifying the fixation eye and the null point is key to preoperative evaluation.
Currently, there is no treatment that can completely stop nystagmus.
Ocular torticollis occurs as a central compensatory response to visual system disorders. The purpose of compensation varies depending on the underlying disease.
In congenital motor nystagmus, the intensity (amplitude and frequency) of nystagmus changes with eye position (positional nystagmus). The eye position where nystagmus is least intense is called the null point, and patients adjust their head position to bring this null point to the front. For example, if nystagmus worsens when looking to the right, the patient prefers looking to the left and turns the face to the right.
In some cases of congenital motor nystagmus, convergence reduces the nystagmus. In such cases, the patient fixates in an extreme adducted position, resulting in apparent esotropia (nystagmus blockage syndrome).
In incomitant strabismus, there is a gaze direction where the ocular deviation is minimal. To eliminate diplopia and maintain binocular vision, the patient adopts a head posture that brings that direction to the front. In superior oblique palsy, the deviation is maximal in adduction, so the patient turns the face toward the healthy side and tilts the head toward the healthy side to minimize vertical deviation.
In AHP analysis of patients with hypertropia, head tilt was most common at 62.8%, followed by face turn at 19.3% and chin elevation at 16.4%. In hypotropia, face turn was 41.5% and head tilt 37%, showing a different pattern from hypertropia 2).
Al-Dabet et al. (2025) systematically analyzed the causal relationship between abnormal head posture (AHP) and ocular diseases. Using Cramer’s V statistic, they quantified the strength of associations among AHP, ocular diseases, and ocular misalignment, identifying strong associations between Duane syndrome and head tilt plus exotropia/esotropia, superior oblique palsy and face turn plus hypertropia, and inferior oblique palsy and head tilt plus hypertropia/hypotropia. These findings are expected to improve diagnostic accuracy for ocular torticollis and enhance rehabilitation strategies2).
In a retrospective study by Goodman et al. (119 cases, 1981–1991), among 59 patients with long-term head tilt since infancy, 45 had superior oblique palsy and 14 had alternating sursumduction. Significant facial asymmetry was observed in the congenital superior oblique palsy group but not in the alternating sursumduction group. The significance of early surgical intervention to prevent facial asymmetry is being investigated2).
Kim et al. (2021) reported two cases of congenital muscular torticollis (CMT) coexisting with ocular torticollis or bony torticollis. They propose that when CMT does not respond to treatment, not only differential diagnosis but also coexistence with other causes should be considered1).
