A case of missed ocular torticollis

Introduction

Torticollis is defined by head tilt toward the contractured muscle and chin rotation to the opposite direction, and can lead to secondary problems such as plagiocephaly, scoliosis, delayed cognitive development and impaired balance [Cooperman, 1997]. Torticollises are a common cause of referral to rehabilitation clinics in the pediatric setting. The etiologies of torticollis may be orthopedic, neurologic or ocular. Congenital muscular torticollis due to sternocleidomastoid contracture is the most common type in infants and may be treated with conservative treatment such as muscle stretching. Surgery is recommended in congenital muscular torticollis if conservative treatment is ineffective or if it results in progressive maxillofacial asymmetry [Suhr and Oledzka, 2015]. Other causes are ocular torticollis due to ocular muscle weakness, Sandifer’s syndrome due to gastroesophageal reflux, neural axis abnormalities and benign paroxysmal torticollis. Ocular torticollis usually occurs due to different ophthalmic conditions, including paralytic and restrictive disorders of ocular movement, nystagmus and defects of the visual field [Herman, 2006]. Here we report a missed ocular torticollis in a girl with presumed congenital muscular torticollis.

Case presentation

A 5-year-old girl with presumed congenital muscular torticollis underwent left sternocleidomastoid muscle-release surgery. As the patient’s symptoms were not improved after surgery, she was referred to a physical medicine and rehabilitation clinic for further physical treatment. The child had left-side head tilt and chin rotation to the right side with normal passive range of motion. There was no facial asymmetry. Musculoskeletal and neurologic evaluations and cervical vertebrae imaging were normal and there was no evidence of muscular mass or shortening in neck muscles. The patient underwent several sessions of physiotherapy, but despite the normal range of motion, head tilt was not corrected. For further evaluations, she was referred to an ophthalmologist. In ocular motility examination, there was right hypertropia and overaction of the right inferior oblique muscle with underaction of the right superior oblique muscle. Visual acuity was 10 out of 10. The patient was diagnosed with congenital right superior oblique palsy and underwent right inferior oblique muscle weakening. In follow-up examination 1 week after surgery, hypertropia was eliminated and head tilt was corrected.

Comment

Children with ocular torticollis do not usually have fibrotic congenital torticollis observed in congenital muscular torticollis. Ocular torticollis manifests later than muscular and skeletal torticollis, usually in the binocular-vision-development period. Binocular-vision development begins at about 4 months of age, peaks at 2 years, is well developed by 4 years of age and slowly declines to cease by 9 years of age [Bhola, 2006]. Common causes of ocular torticollis are congenital paralytic squint and congenital nystagmus. Superior oblique muscle weakness or palsy is the most common cause of ocular torticollis [Boricean and Bărar, 2011], and leads to head tilt far from the involved eye. Researchers have found significant correlation between facial asymmetry and side of head tilt in patients with congenital right superior oblique palsy [Rao et al. 1999]. It is possible the defective formation of face and skull due to persistent sleep in one direction in the first 6–12 months of life may be the possible mechanism for facial asymmetry in these patients. Primary strabismus surgery in order to correct the head tilt could help to prevent the facial asymmetry, but giving different sleeping positions for the infant may have a more important role.

This report notes that proper knowledge of the ocular torticollis signs and symptoms to differentiate them from other orthopedic or neurologic etiologies is important. It is hard to differentiate ocular from nonocular causes clinically; therefore, it is suggested that patients with torticollis with unclear orthopedic etiologies, especially those children at the age of binocular vision, be referred to the ophthalmologist for ocular examination [Herman, 2006].