Imaging in Acute Torticollis

Division of Neuroradiology Department of Radiology University of North Carolina at Chapel Hill

Overview of This Presentation

I. II. III. Introduction Imaging algorithm for acute torticollis Causes of torticollis
A. B. C. D. Trauma Infection/Inflammation Neoplasm Other/Idiopathic

IV. V.

Atlantoaxial rotatory fixation Selected references

At the Conclusion of this Exhibit One Should Be Able To:

Define torticollis Describe an algorithm for imaging patients presenting with torticollis List several potential causes of torticollis and describe their typical imaging features Discuss the concept of atlanto-axial rotatory fixation and its diagnosis

Introduction: What is Torticollis?

Derived from the Latin tortus (twisted) + collis (neck or collar) Torticollis is defined as abnormal twisting of the neck which causes the head to be held in a rotated or tilted position.

Introduction: Clinical Aspects of Acute Torticollis

Torticollis refers to a symptom rather than a distinct disease process It can be caused by a wide variety of conditions (over 80 causes have been described) which range from relatively innocuous to lifethreatening May be congenital or acquired Occurs more frequently in children than in adults The right side is affected in 75% of patients

Introduction: Chronic Sequelae of Torticollis

Physical Positional plagiocephaly Facial deformities Cervical spine degeneration Radiculopathies and myelopathies Psychiatric Major depression Agoraphobia Substance abuse OCD

Imaging of Patients with Torticollis

Choice of imaging studies depends on age and if history of trauma is present. In newborn infants with congenital muscular torticollis, ultrasound is preferred and often diagnostic. In older children and adults with post trauma torticollis, CT of neck/cervical spine is needed to exclude fracture or malalignment. If CT is positive, MRI and MRA of the neck should be considered to evaluate for associated cord, ligamentous, or arterial injuries. In older children and adults presenting with torticollis without trauma, neck/cervical spine CT is the initial imaging study; if negative, then brain and cervical spine MRI is performed to exclude a CNS cause of torticollis.

Imaging Algorithm for Acute Torticollis

Patient with torticollis Newborn infant Older child or adult Trauma CT neck and/or cervical spine Negative Stop Positive MRI C-Spine MRA neck No trauma CT neck and/or cervical spine Positive Stop Negative MRI C-spine MRI brain

Ultrasound

Negative CT or MRI of neck

Positive

Stop

Causes of Torticollis

Traumatic Causes of Torticollis

Muscular
Fibromatosis colli Muscle spasm following trauma

Skeletal
Unilateral interfacetal dislocation (UID) Occipital condyle fractures Atlanto-axial rotatory fixation (? truly traumatic)

CNS related
Subarachnoid hemorrhage Spinal epidural hematoma

Traumatic Causes of Torticollis: Fibromatosis Colli

Rare form of infantile fibromatosis affecting sternocleidomastoid muscle (SCM) Accounts for >80% of childhood cases of torticollis Due to traumatic delivery or possibly abnormal head position in utero Infants usually appear normal at birth, torticollis develops in the 2-3rd weeks of life More common in males and in right side Sonographic findings are typical

Traumatic Causes of Torticollis: Fibromatosis Colli

Longitudinal US views of the right (top) and left (bottom) SCMs in an infant with torticollis. The right SCM is enlarged and of heterogeneous echotexture. The left SCM is normal. There are mildly enlarged lymph nodes posterior to the left SCM

Traumatic Causes of Torticollis: Fibromatosis Colli

Axial contrast CT in an infant with fibromatosis colli. The right SCM is enlarged and has faint central enhancement (arrowhead).

Traumatic Causes of Torticollis: Unilateral Interfacetal Dislocation

Axial CT image and a saggital reformatted imagedemonstrate right facet dislocation (arrows).

Traumatic Causes of Torticollis: Occipital Condyle Fracture

Axial and coronal reformatted CT images show a right occipital condyle fracture (type III) in a patient presenting with acute torticollis after trauma.

Occipital Condyle Fractures

Classified into 3 types by Anderson and Montesano
I Axial loading fracture limited to the occipital condyle without displacement into foramen magnum II Fracture of basiocciput extending into occipital condyle III Small fragment arising from medial surface of condyle avulsed by an intact alar ligament and distracted towards dens

Infectious and Inflammatory Causes of Torticollis

CNS related
Meningitis

Head and Neck related

Upper respiratory infections Otitis media Mastoiditis/Bezolds abscess Cervical adenitis Retropharyngeal abscess

Spine related
Vertebral osteomyelitis and/or discitis Epidural abscess Rheumatoid arthritis

Infectious Causes of Torticollis: Mastoiditis/Bezolds Abscess

Unenhanced (right) and enhanced (left) axial CT images in a patient with acute torticollis and right ear pain demonstrate coalescing mastoiditis eroding medial surface of mastoid (arrow). Inferior to this is an abscess involving the right SCM (arrowhead).

Bezolds Abscess
Rare complication of suppurative mastoiditis occuring when infection erodes the mastoid tip into the neck, forming an abscess May cause spasm of the SCM, resulting in torticollis Abscess may spread down the plane of the sternocleidomastoid muscle into the lower neck Also associated with cholesteatomas

Infectious Causes of Torticollis: Suppurative Adenitis

Enhanced axial fat suppressed T1 MR image demonstrates a necrotic retropharyngeal lymph node (arrowhead) in a child with suppurative adenitis presenting as acute torticollis.

Infectious Causes of Torticollis: Discitis and Osteomyelitis

T2

T1 post-Gd

Inflammatory Causes of Torticollis: Rheumatoid Arthritis

Unenhanced sagittal T1 MR in a patient with rheumatoid arthritis and torticollis. There is pannus destroying the dens and compressing the lower brainstem and medulla.

Neoplastic Causes of Torticollis

CNS tumors
Spinal cord or brainstem tumors Posterior fossa tumors and cysts Vestibular schwannoma Metastases

Bone tumors
Vertebral eosinophilic granuloma Osteoid osteoma/osteoblastoma Metastases (spine or skull base)

Neoplastic Causes of Torticollis: Spinal Cord Tumor

Sagittal enhanced T1 MRI of the cervical spine demonstrates an enhancing, expansile ganglioglioma in a 10year-old female presenting with acute torticollis.

Neoplastic Causes of Torticollis: Skull Base Tumor

Axial enhanced T1 MRI in an adult with acute torticollis demonstrates a metastasis from renal cell carcinoma (arrowheads) involving the left occipital condyle.

Other Causes of Torticollis

Dystonic syndromes (idiopathic spasmodic torticollis) Chiari 1 malformation Syringomyelia Neuroleptic drug reactions Congenital vertebral anomalies (e.g. congenital scoliosis, cervical segmentation anomalies, Klippel-Feil syndrome) Hemifacial microsomia Oculomotor nerve palsies/Strabismus Gastroesophageal reflux (Sandifers syndrome) Vascular abnormalities (craniocervical AV fistula; congenital hypoplasia of the internal carotid artery) Pseudotumor cerebri

Other Causes of Torticollis: Chiari I Malformation

Unenhanced midsagittal T1 weighted MR image shows significant downward displacement of pegshaped cerebellar tonsils (arrowhead) through foramen magnum (type I Chiari malformation).

Other Causes of Torticollis: Chiari I Malformation with a Syrinx

Unenhanced sagittal T1 weighted image demonstrates a large, expansile, multiseptated cyst in the cervical cord of a patient with a Chiari I malformation and torticollis.

Chiari I Malformation
Defined as greater than 5 mm of displacement of triangular-shaped cerebellar tonsils below the foramen magnum Believed to be due to an abnormality of expression of spinal segmentation genes that lead to varying degrees of hypoplasia of the skull base Unclear if torticollis is due to associated skeletal abnormalities or due to compression of brainstem and lower cranial nerves Torticollis may be caused by syringohydromyelia even in absence of a Chiari malformation

Other Causes of Torticollis: Klippel-Feil Syndrome

Lateral radiograph of the cervical spine shows hypoplasia and fusion of lower cervical vertebrae in a patient with Klippel-Feil syndrome and torticollis

Klippel-Feil Syndrome
Heterogeneous group of conditions unified by presence of congenital synostosis of some or all cervical vertebrae Classic triad described by Klippel and Feil consisting of short neck, low posterior hairline, and limited range of motion of neck (seen in <50% of patients) Commonly associated abnormalities include congenital scoliosis, rib abnormalities, deafness, genitourinary abnormalities, Sprengels deformity, and cardiac abnormalities Along with congenital scoliosis, accounts for nearly 1/3 of nonmuscular causes of torticollis in children Cervical anomalies are well characterized by CT

Idiopathic Spasmodic Torticollis (IST)

Also referred to as cervical dystonia Nontraumatic, acquired form of torticollis presenting as spasms or jerks of SCMs Females more commonly affected by 4.5:1 Typically occurs in adults over age 30 Diagnosis requires exclusion of other potential causes of torticollis and that symptoms be present for at least 6 months Conventional neuroimaging studies usually negative

Idiopathic Spasmodic Torticollis (IST)

Although pathophysiology of IST is not understood, the interstitial nucleus in the brainstem has been implicated as a probable site of abnormality IST may be due to abnormalities of the basal ganglia, vestibular systems, or spinal accessory nerves Proton MR spectroscopy in IST patients may demonstrate diminished n-acetyl-aspartate (NAA) levels in basal ganglia when compared with normal controls

Proton MR Spectroscopy in Idiopathic Spasmodic Torticollis

Long echo time proton MRS at level of left basal ganglia (left) demonstrates low level of n-acetylaspartate relative to normal right basal ganglia (right).

Atlanto-axial Rotatory Fixation

Atlanto-axial rotatory fixation (AARF) is a controversial entity - Is it the result of or the cause of torticollis? True atlanto-axial subluxation or dislocation is rare 75-80% of reported cases occur in children Compression of spinal cord may occur if there is anterior or posterior displacement Vertebral artery kinking or stretching may occur and cause posterior circulation ischemic symptoms

Atlanto-axial Rotatory Fixation

Frequently, there is an antecedent history of trauma or upper respiratory infection Grisels syndrome = non-traumatic atlanto-axial subluxation secondary to ligamentous laxity and inflammation following infection or surgery in the head and neck region It has been postulated that swollen capsular and synovial tissues and muscle spasm prevent reduction early on and that ligament and capsular contractures develop later, ultimately causing fixation

Types of Atlanto-axial Rotatory Fixation (Fielding classification)

Type 1 Rotatory fixation w/o anterior displacement of atlas (intact transverse and alar ligaments) most common type Rotatory fixation with 3-5 mm of anterior displacement of atlas (implies deficiency of transverse ligament) Rotatory fixation with >5 mm of anterior displacement of atlas (implies deficiency of both transverse and alar ligaments) Rotatory fixation with posterior displacement of atlas (implies deficiency of odontoid process)

Type 2

Type 3

Type 4

Types of Atlanto-axial Rotatory Fixation (Fielding classification)

From Lustrin ES, Karakas SP, Ortiz AO, et al. Pediatric cervical spine: Normal anatomy, variants, and trauma. Radiographics 2003; 23:539-60. (Used with permission)

Radiographic Diagnosis of Atlantoaxial Rotatory Fixation

CT is essential for imaging of AARF When rotation is accompanied by anterior or posterior displacement (Fielding types 2-4), CT is diagnostic Type 1 rotatory fixation appears identical to other causes of torticollis when patients are imaged at rest
Thus, patients with suspected type 1 AARF should be scanned at rest and with maximal voluntary contralateral head rotatation CT in patients with AARF shows little or no change in position of atlas with respect to axis

Type 1 Atlanto-axial Rotatory Fixation

Axial CT image with head rotated to left shows widened space between dens and right C1 lateral mass which persists with rotation of head to right (arrowheads) compatible with AARF. The atlanto-dental interval is normal making this a type 1 AARF.

Selected References
Anderson PA, Montesano PX. Morphology and treatment of occipital condyle fractures. Spine 1988; 13:731-6. Ballock RT, Song KM. The prevalence of nonmuscular causes of torticollis in children. J Pediatr Orthop 1996; 16:500-4. Castillo M, Albernaz VS, Mukherji SK, Smith MM, et al. Imaging of Bezolds abscess. AJR Am J Roentgenol 1998; 171:1491-5. Federico F, Lucivero V, Simone IL, Defazio G, et al. Proton MR spectroscopy in idiopathic spasmodic torticollis. Neuroradiology 2001; 43:532-6. Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation (fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977; 59:3744. Kraus R, Han BK, Babcock DS, Oestreich AE. Sonography of neck masses in children. AJR Am J Roentgenol 1986; 146:609-13. Roche CJ, OMalley M, Dorgan JC, Carty HM. A Pictorial Review of Atlantoaxial Rotatory Fixation: Key points for the radiologist. Radiographics 2001; 56:947-58. Tracy MR, Dormans JP, Kusumi K. Klippel-Feil Syndrome: Clinical features and current understanding of etiology. Clin Orthop Relat Res 2004; 424:183-90.