The importance and effectiveness of conservative treatment in Grisel's syndrome.

Dear Sir,

Nontraumatic atlo-axial subluxation (AAS), known as well as Grisel's syndrome, is a rare condition occurring mainly in children as a result of otolaryngology procedures or, less commonly, upper neck's inflammatory processes. For its typical symptoms, Grisel's syndrome has an important role in differential diagnosis of torticollis due to its low incidence and its necessity for early treatment.[1]

Current treatment is based on Fielding's classification[2] (type I-IV), introduced to assess the degree of instability of C1-C2 displacement, including advanced stages which may require halo fixation or surgical intervention [Table 1].

Table 1

Stage-related therapy of Grisel's syndrome

A 5-year-old girl referred to our Emergency Department reporting a 2 weeks-persistent right torticollis. Her recent clinical history revealed cough and fever from the day before the neck stiffness’ onset, which spontaneously regressed in 2-3 days; no trauma was reported. She had already been treated with nonsteroidal anti-inflammatory drug (NSAID) and physiotherapy reporting no benefits.

On admission she presented, at physical examination, with right torticollis, sore throat, tonsillar hypertrophy and hyperemia; neurological examination reported no deficits.

Laboratory analysis showed elevated C-reactive protein (2.3 mg/dl) and mild leukocytosis. The child was admitted for further investigation: Cerebral and column magnetic resonance imaging (MRI) was performed, it showed incidental syringomyelia at C6-C7 and D6-D7 levels and an AAS with ventral dislocation of C1 entailing a significant reversal of cervical lordosis ascribable to a type III Grisel's syndrome. These results were confirmed by a cerebral and cervical computed tomography (CT).

The patient, after manual reduction performed under conscious sedation, was treated with cervical brace (Aspen Vista): We attempted a less aggressive approach before considering a surgical solution. After that procedure she continued medical therapy with myorelaxant agents and NSAID.

At 1 month follow-up the patient showed benefit from the treatment and torticollis resolution.

A MRI was performed after 5 weeks: It showed a resolution of subluxation and a complete normalization of cervical lordosis, syringomyelia was slightly reduced at dorsal level. Considering the good clinical and radiological outcome we interrupted the cervical brace prescription and the patient performed a rehabilitation cycle, no further neurological symptoms were reported.

Nontraumatic atlo-axial subluxation, known as well as Grisel's syndrome, is a rare condition occurring mainly in children, with 68% of cases occurring in patients under the age of 12 and 90% under the age of 21, no gender-specific predisposition has been shown and manifestation in adults is exceptionally rare. The syndrome has been reported to be associated with a head and neck surgical operation most commonly, followed by upper respiratory infection as the second most common.[1] The reason why this pathology is almost solely confined to the pediatric population remains unclear. A hypermobility of C1 on C2 with an increased dens-atlas interval of up to 4.5 mm has been observed in children (adults range from 2.5 to 3 mm).[3] Thus, Battiata and Pazos postulated a combination of ligament laxity and an accompanying precipitator (e.g., infectious process, surgery) as a “two-hit” hypothesis.[4]

The presenting symptoms are typically persisting torticollis with the head in an oblique position, elevated ipsilateral muscular tension and stiffness of the sternocleidomastoid muscle.

Diagnosis is better based on clinical examination supplemented with high resolution CT.[2] Each case is assigned a stage from Fielding's classification (type I-IV), introduced to assess the degree of instability of C1-C2 displacement and to deal with the treatment with type-related recommendations, including advanced stages (III and IV) which may require halo fixation or surgical intervention (arthrodesis or C1-C2 fusion) and minor types (I and II) for which a conservative treatment (immobilization, muscle relaxants) is recommended.

Despite the stage-related recommendations, our patient with Fielding III AAS benefited from a conservative treatment with manual reduction and cervical brace which produced a complete regression of the symptoms and a normalization of cervical axis showed by MRI.

A less invasive approach in children should be preferred due to the side effects linked to halo fixation and surgical intervention. Extensive fusion and stabilization with instrumentation should be avoided in those patients due to problems of future axial skeleton growth[5] and the risk of postoperative deformity.

In children it is usually recommended to use an orthesis until skeletal maturity before performing an arthrodesis.[6] After surgical stabilization, persistent neurological complications occur in up to 15% of all patients[7] and even fatal outcomes with tetraplegia have been described[8] in the literature.

Surgical treatment may be considered in case of persistent deformities or recurrences but the first line approach should be conservative whenever possible: Early diagnosed patients benefit from nonsurgical treatment, as recently suggested in Pilge's work[4] (five young patients with various Grisel's syndrome types as a result of otolaryngology surgery being treated with manual reduction under general anesthesia and Minerva cast, achieving a good outcome) and confirmed in our case.

Grisel's syndrome has to be considered in the differential diagnosis for torticollis, despite its rarity, because when early diagnosed with MRI, or better with CT, the majority of patients can be successfully treated with reduction and stabilization in a nonoperative setting.

A less aggressive approach is recommended whenever possible: We attempted a manual reduction and a cervical brace treatment associated with rehabilitation achieving a complete clinical-radiological normalization despite the high. Fielding stage (III) and the 2 week interval between symptoms’ onset and diagnosis. Due to its possible complications, a surgical solution should be considered only as a second level approach whenever possible.