Case 2: A pain in the neck

A 10-year-old African-Canadian girl experienced a sudden onset of neck stiffness. There was no history of preceding trauma, but she had reported a sore throat and hoarse voice three weeks earlier. Over the next year, she developed progressively worsening torticollis with associated neck pain, daily headaches and intermittent diplopia. She was taking ibuprofen two to three times daily for head and neck pain with partial improvement.

She presented to the emergency department 13 months after onset because her head was now locked in position. There was no history of fevers, weight loss, skin rash, joint pain, muscle weakness, sensory deficits or gait abnormalities. On physical examination, the patient’s head was fixed in position with her chin rotated toward the right and her head tilted toward the left. There was minimal tenderness over her left sternocleidomastoid muscle and no palpable neck mass. Her neurological examination, including cranial nerve testing, was normal. She had no active or effused joints, enthesitis or tenosynovitis. An ophthalmological assessment, including visual acuity, was also unremarkable.

Laboratory investigations revealed an increased erythrocyte sedimentation rate of 35 mm/h, but her C-reactive protein, complete blood count, creatine kinase and transaminase levels were normal. Plain radiographs showed a loss of cervical lordosis and abnormal alignment of C1–C2. Further imaging revealed the underlying diagnosis.

CASE 2 DIAGNOSIS: GRISEL’S SYNDROME

A computed tomography (CT) scan of the child’s cervical spine demonstrated atlanto-axial rotatory subluxation and magnetic resonance imaging (MRI) found inflammation of the C1–C2 articular joint and surrounding soft tissues (Figure 1). Her clinical presentation and the finding of atlanto-axial rotatory subluxation led to a diagnosis of Grisel’s syndrome. She was admitted to hospital for a trial of traction, using a halter-chin apparatus, and anti-inflammatory therapy with naproxen. Unfortunately, this did not reduce the subluxation or inflammation and she subsequently underwent cervical traction with a halo and oral corticosteroid therapy. She is now asymptomatic with full range of movement of her neck. A recent MRI scan showed resolution of the rotatory subluxation and inflammatory changes but a persistently widened atlanto-odontoid space.

Figure 1).

Computed tomography reconstruction of the posterior cervical spine shows rotatory subluxation of C1 over C2

Grisel’s syndrome is characterized by nontraumatic, atlanto-axial rotatory subluxation. It is an uncommon condition that has been reported in more than 100 patients in the literature, mostly in case reports and case series (1,2). Typically, it is recognized following head and neck infections, such as pharyngitis, otitis media, retropharyngeal abscess or tonsillar abscess (1). It has also been reported in the context of tonsillectomy and other otolaryngological procedures and rarely as an idiopathic process (1). The pathogenesis of Grisel’s syndrome remains unclear. However, a proposed anatomical mechanism involves the spread of infection- or injury-related inflammation through a plexus of pharyngovertebral veins next to the anterior transverse ligament of C1–C2 (2,3).

Grisel’s syndrome leads to neck pain, loss of cervical mobility and torticollis. Tenderness and deviation of the spinous process of C2 in the same direction as head rotation (Sudeck’s sign) may also be present (1,3). Neurological complications occur in approximately 15% of cases and range from mild radiculopathy to severe myelopathy (1,3).

The differential diagnosis for new onset of neck pain and torticollis during childhood is extensive (4). It includes benign paroxysmal torticollis, traumatic neck injury, infections, calcification of the cervical disc, congenital abnormalities of the cervical vertebrae such as hemiatlas, Arnold-Chiari malformation, central nervous system tumours, neurogenic or drug-induced dystonias, gastroesophageal reflux with torsional neck spasms (Sandifer syndrome), inflammatory arthritis and myositis (4). A careful clinical assessment is necessary to differentiate benign causes from more serious causes of neck pain and torticollis (5).

Benign paroxysmal torticollis is a self-limiting condition that usually presents in children younger than five years of age. It has been described as a migraine equivalent with recurrent episodes of head tilt associated with vomiting, irritability, ataxia and drowsiness. Traumatic injuries may involve the vertebrae, muscles or ligaments, or may result in a spinal epidural hematoma. Unusual or extreme posturing of the neck, gait abnormalities and neurological deficits are concerning for possible posterior fossa tumours, spinal cord tumours, spinal cord compression and discitis (4). These conditions may also be associated with headaches, night pain, vertigo, visual disturbances and vomiting (5). A child with a retropharyngeal abscess, cervical adenitis, osteomyelitis or discitis involving the cervical spine may present with fever, neck pain and limited range of motion (5). Medications and herbal supplements with extrapyramidal side effects may lead to neck pain and torticollis. Finally, ocular motility disorders may also lead to postural torticollis when children tilt their heads to avoid diplopia (4).

Children with acute-onset torticollis who present with a history of trauma, persistent neck symptoms, increasing severity of neck pain, worsening neck deformity, neurological deficits or gait abnormalities should undergo imaging (5). Conventional radiographs and CT imaging are often useful to identify traumatic neck injury or congenital abnormalities of the cervical vertebrae. In Grisel’s syndrome, plain radiographs may show asymmetry of the facet joints and a larger predental space (1,3). CT imaging is currently the gold standard to identify atlanto-axial rotatory subluxation (1–3). MRI of the head and spine are indicated to assess for a central nervous system tumour and is also a helpful tool to evaluate the condition of the vertebrae and supporting ligaments, to demonstrate inflammation of the cervical spine and surrounding structures, and to look for spinal cord or nerve root compression (1,3).

The present patient’s diagnosis of Grisel’s syndrome was based on her clinical assessment and imaging. An acute infectious process was unlikely because she was afebrile, systemically well and had chronic neck symptoms. The presence of atlanto-axial rotatory subluxation has not been reported in children with chronic osteomyelitis. She had no history of trauma or medication use. Given the absence of neurological, gait and vision abnormalities, a central nervous system tumour was not expected or identified on MRI. Her imaging excluded any congenital anomalies of the cervical spine and any muscle or disc abnormalities. An inflammatory arthropathy was unlikely because she had no peripheral arthritis long after the onset of neck involvement.

The key to successful management of Grisel’s syndrome is early diagnosis and initiation of therapy (1,3). Failure of reduction, recurrence and complications are more likely with a prolonged delay before starting treatment (2). Inappropriate treatment may lead to permanent deformity and neurological deficits (2). Antibiotics are required when there is an underlying head and neck infection. A trial of immobilization with a cervical collar and anti-inflammatory medications may be successful if initiated early after the onset of subluxation (1–3). More significant C1–C2 rotatory subluxation may require immobilization and traction, which alleviates pain and reduces the fixation (3).

Grisel’s syndrome is a rare but potentially serious condition that often presents as torticollis in children and young adults. Given the strong association with head and neck infections and procedures, this syndrome has mostly been described and identified by otolaryngologists. However, paediatricians and paediatric emergency physicians should also be aware of this condition because they frequently evaluate children with neck pain and torticollis.

CLINICAL PEARLS

• Children who present with new onset of neck pain and torticollis need a careful history and physical examination to identify the underlying cause.

• Imaging should be arranged for children presenting with a history of trauma, persistent or recurrent symptoms, increasing severity of neck pain, worsening neck deformity, neurological deficits or gait abnormalities.

• Vigilant follow-up should be arranged if the cause of neck pain and torticollis is not initially apparent.