Non-traumatic Atlanto-axial Rotatory Subluxation- Grisel’s Syndrome Case Report and Literature Review

Abstract

Study design: Case report of conservatively treated non-traumatic Atlanto-axial rotatory sublaxation.Objective: To report our case with this rare condition (non-traumatic Atlanto-axial rotatory subluxation-Grisel Syndrome) in a 9-year old girl presenting with torticollis and neck pain and literature review. Summary of background data:Atlanto-axial rotatory fixation or non-traumatic atlanto-axial subluxation which is usually secondary to an infection or an inflammation at the head and neck region is a rare subtype of subluxation in which a pathologic fixation of the atlas on the axis is present. It is most common in paediatric patients and it can be managed conservatively in the acute stage. In contrast, cases of chronic non-traumatic atlanto-axial rotatory subluxation are usually treated with operative reduction, according to literature data. Although high success rates have been achieved with operative reduction in chronic cases, a significant reduction in neck motion occurs due to the atlanto-axial fusion.Methods: A 9-year-old girl developed torticollis established longer than 10 weeks as a result of an upper respiratory tract infection. Computed tomography showed a type 1 rotatory subluxation of the Atlanto-axial joint according to the classification of Fielding and Hawkins. The patient was treated by manual reduction according to Jeszenszky (Jeszenszky transoral Citation Nr 22) under general anaesthesia and fluoroscopy control, and then a halo vest had been applied for 6 weeks. Results:We successfully treated this patient with chronic non-traumatic Atlanto-axial rotatory subluxation with manual reduction and immobilisation by applying a halo-body-jacket. She showed full recovery of neck motion and normal Atlanto-axial angle on clinical and radiological follow-up after 1 month and 3 months respectively. Conclusion:Chronic non-traumatic Atlanto-axial rotatory subluxation is a rare condition which can easily escape the attention of physicians especially in children. Conservative treatment with complete reduction under anaesthesia and subsequent immobilisation via halo-body-jacket for at least 6 weeks is a viable option as presented in this case report. Surgical treatment should be reserved only for cases of failure of conservative management (recurrence or irreducible subluxation).

Diagnostic Approach and Treatment Options for Pediatric Cases of Grisel’s Syndrome Post Otolaryngology Procedure: A Systematic Review

“…Radiological findings eliminate potential diagnoses such as para pharyngeal abscess, still-active ear, nose throat infection, and neurological and traumatic causes. Neurologic complications are more likely to occur with a delayed diagnosis [33][34][35]. While Beyazal et al reported that the patient's medical history and clinical symptoms are crucial indicators of the diagnosis, neck pain is the commonly reported symptom that worsens with attempted motion.…”

Grisel syndrome presenting as hemiplegia in a patient with multifocal staphylococcal sepsis

“…Karkos et al conducted a systematic review of 71 articles published between 1950 and 2006, which included 96 patients with C1–C2 subluxation with no history of previous trauma. Of these, 48% had a recent history of infection mostly affecting the upper airways and 40% were in the postoperative period after head and neck procedures9,10,11. The Karkos et al review demonstrates how rare the condition is.…”

“…Another theory proposed by Tedesco et al suggests that cervical lymphadenitis caused by a nasopharyngeal infection may lead to irritative spastic contraction of sub-occipital and paravertebral muscles, causing torticollis11.…”

“…According to this protocol: Conservative treatment for type 1 is a soft collar, for type 2 a rigid collar, and a halo-vest for type 3 lesions. For type 4 lesions, they recommend surgical treatment11,12. This however poses controversy as far as type 3 pathology is concerned.…”