Unusual presentation of an unusual disease: A very delayed diagnosis of Grisel’s syndrome

Grisel’s syndrome (GS) is a rare syndrome which refers only to non-traumatic atlantoaxial subluxation. This syndrome predominantly occurs in young children following an upper respiratory infection or otolaryngologic procedures. An eight-year-old girl with a delayed diagnosis of GS was admitted to our outpatient clinic with complaints of painful torticollis and neck stiffness. Three-dimensional computed tomography revealed rotatory atlantoaxial subluxation. After consulting with the neurosurgery department, the patient underwent surgery. The significance of this patient was that she was unable to be diagnosed early and atlantoaxial subluxation remained hidden for five years without any complications. In conclusion, this rare case highlights the importance of delayed diagnosis of GS and clinicians should be aware of this syndrome.

Grisel’s syndrome – case report

Introduction: By definition from the literature, Grisel’s syndrome is described as non-traumatic rotational atlantoaxial instability between C1 and C2 vertebrae. It can occur during an infection of a soft tissue in the cervicocranial region or after an operation in the ENT region. Because of the frequent occurrence after operations, we inclined to the definition which includes a traumatic subluxation as a cause of origin, and it’s not defined as non-traumatic only. The instability manifests itself with abnormal head posture that is called torticollis. Increased incidence in adolescence is more common because of a greater ligamentous laxity of the joint capsules, increased perfusion of antlantoaxial regions and longer alar ligaments. In this article, the case of a child with Grisel’s syndrome after adenotomy is described. The pathophysiology, symptomatology, diagnostic management and treatment are discussed. Keywords: Grisel’s syndrome – torticollis – atlantoaxial instability – adenotomy

Retropharyngeal abscess causing grisel’s syndrome secondary to tuberculosis of cervical spine, a rare form of torticollis: case report

<p>Grisel’s syndrome is a rare type of non-traumatic subluxation of an atlantoaxial joint characterized by torticollis, neck pain, and reduced neck movement. The common causes of Grisel’s syndrome are head and neck infection or post-otorhinolaryngology (ORL) procedures. We are reporting a case of a 3-year-old boy with a gradual worsening of neck stiffness, neck pain, and restricted neck movement for more than one month. The patient had no history of trauma. The computerized tomographic (CT) showed a retropharyngeal abscess with a bony erosion causing atlantoaxial subluxation. The management and progress of this patient are discussed. The objective of this case report is to emphasize that Grisel’s syndrome should be considered a differential in a painful torticollis to prompt an early diagnosis and treatment to prevent serious neurological complications.</p>

Grisel’s syndrome in adults: A case report

Background: The non traumatic, post inflammatory atlantoaxial rotatory instability, also known as Grisel’s syndrome is a relatively rare condition usually affecting children. Adult cases are rare and even less frequently reported with separate case reports describing a single patient. Although antibiotic treatment and close neurological monitoring seem to be the gold standard of care, there is no general consensus on the optimal timing and extent of the surgical treatment. Case Description: We present a case of C1-C2 spondylitis, secondary to retropharyngeal abscess, without atlantoaxial instability on initial evaluation that progressed to C1-C2 subluxation with rapidly developing myelopathy 3 months after optimal antibiotic therapy and complete clinical and biochemical remission. Conclusion: Grisel’s syndrome is a rare condition in adults with secondary instability in spite of successful antibacterial treatment, which requires decompression and delayed surgical fixation in our case.

Grisel’s Syndrome in Children: Two Case Reports and Systematic Review of the Literature

Background and Objective. Grisel’s syndrome is a rare syndrome characterized by nontraumatic rotatory subluxation of the atlantoaxial joint. It usually affects children and typically presents with torticollis after ear, nose, and throat (ENT) surgery or head and neck infections. In the pediatric literature, there is only a small amount of available data; moreover, no systematic review has been previously done with focus on the pediatric population. We report our experience of two cases, and we provide a systematic review on Grisel’s syndrome in children in order to offer a deeper insight about its clinical presentation, its current diagnosis, and principles of treatment. Case Reports and Review. We describe two boys of 9 and 8 years old, who developed atlantoaxial subluxation after adenoidectomy. Considering the early diagnosis, a conservative treatment was chosen, with no recurrence and no sequelae at follow-up. We identified 114 case reports, of which 90 describe children, for a total of 171 pediatric patients. Of the 154 cases in which cause was reported, 59.7% presented a head and neck infection and 35.7% had previous head and neck surgery. There is no sex prevalence (49.7% males versus 50.2% females). Mean delay in diagnosis is 33 days. Eight % of the patients had neurological impairment of the 165 cases which mentioned treatment, 96% underwent a conservative treatment, of whom the 8.8% recurred with the need of surgery. As a whole, 12% underwent surgery as a first- or second-line treatment. 3 6% of the patients whose follow-up was reported developed a sequela, minor limitation of neck movement being the most frequent. Conclusion. Grisel’s syndrome should be suspected in children with painful unresponsive torticollis following ENT procedures or head and neck inflammation. CT scan with 3D reconstruction is the gold standard for diagnosis, allowing the identification of the subluxation and the classification according to the Fielding–Hawkins grading system. Surgical treatment is indicated in case of high-grade instability or failure of conservative treatment. Review of the literature shows how early diagnosis based on clinical and radiological evaluation is crucial in order to avoid surgical treatment and neurologic sequelae.