Bone Abstracts

Background: Torticollis in toddlers is most frequently a manifestation of traumatic atlantoaxial rotatory displacement or oropharyngeal inflammation, and rarely due to retropharyngeal abscesses and pyogenic cervical spondylitis. Rarely, intermittent torticollis may be caused by posterior fossa tumors. A very rare case of chronic intermittent torticollis due to upper spine Ewing sarcoma is presented.

Presenting problem: A 2.2 year-old girl suffered four episodes of painful torticollis, over a 9 month period. Anti-inflammatory drugs helped relieving the symptoms. Three months later, a very painful torticollis episode lasted for 15 days and then proceeded to full immobilization of the neck in torticollis. Following evaluation by a pediatric orthopedic at age 3.4 years, a MRI scan was suggested and revealed a solid mass occupying lesion arising from the body of the C2 vertebra, extending posteriorly, encasing the neck and the pedals of the axis (C2) and extending to the spinous process. The mass extended to the superior and inferior joint and the spinal canal, resulting in spinal cord compression. Maximal length of the intracanal mass was 3.2 cm and was causing spinal cord displacement and compression by imaging, interestingly without significant neurologic deficits clinically.

Clinical management: The patient was transferred to our neurosurgery service, where steroids were initiated. CT guided biopsies were suggestive of Ewing Sarcoma, the diagnosis of which was established with an open biopsy. Full staging confirmed only local pathology, without metastases. The patient was started on treatment according to the EURO-Ewing Protocol, while taking extreme care for the stability of the upper cervical area.

Discussion: Chronic intermittent, relapsing remitting torticollis is rare in toddlers, as acute torticollis is caused by trauma or inflammation. Rare causes to be considered are early childhood myopathies and myasthenia, juvenile cerebral palsy, phenothiazine-induced acute dystonic reactions, juvenile-onset Wilson or Huntington disease, gastroesophageal reflux, anterior-horn disease, radiculopathy and C1-C2 pathologies. This patient had intermittent chronic torticollis, without neurological deficits yet, despite the significant spinal cord compression, due to a very lingering chronic course. Early appropriate imaging could have revealed the exact pathology to the patient’s benefit.

Disclosure: The authors declared no competing interests.