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Grisel's syndrome: a case report on this rare pediatric disease and its anesthetic challenges
Kavya N Reddy1, Shabaaz M Baig2, Meenu Batra2
1Saint Louis University, St. Louis, USA.
Insights
Grisel's syndrome, a non-traumatic atlantoaxial subluxation, can cause severe neck issues in children. This case highlights that while conservative treatment often works, surgical intervention may be necessary for persistent torticollis.
Area of Science:
- Pediatric Orthopedics
- Rheumatology
- Neurosurgery
Background:
- Grisel's syndrome involves non-traumatic atlantoaxial subluxation linked to head/neck inflammation, primarily affecting children.
- Pathogenesis involves increased ligamentous laxity during inflammation, leading to instability between the atlas and axis.
- Early diagnosis and treatment are crucial to prevent severe sequelae.
Observation:
- An 8-year-old child presented with torticollis following a two-week streptococcal throat infection.
- Conservative treatments including muscle relaxation, cervical halter traction, and Halo application were unsuccessful.
- The torticollis persisted, necessitating surgical correction via cervical spine fusion.
Findings:
- This case underscores that surgical intervention for Grisel's syndrome, though rare, may be required despite aggressive conservative management.
- The patient experienced no complications post-surgery and no recurrence of torticollis.
- Surgical management may involve additional anesthetic risks for diagnostic imaging and reduction procedures.
Implications:
- Highlights the importance of considering surgical options for refractory Grisel's syndrome cases.
- Emphasizes the need for careful anesthetic planning for interventions in pediatric patients with Grisel's syndrome.
- Contributes to the limited literature on surgical management of this condition.
Background:
Grisel's syndrome is a non-traumatic atlantoaxial subluxation associated with inflammatory conditions of the head and neck, which occurs primarily in children. Increased flexibility of the ligaments during inflammation is implicated in the pathogenesis of the subluxation between the axis and atlas. The potential sequelae may be severe, and early diagnosis and treatment of Grisel's syndrome can prevent tragic outcomes.
Case Presentation:
We present a case of torticollis in an 8-year-old child. She had a two-week history of a streptococcal throat infection. The patient was treated with several different methods of conservative care, including muscle relaxation, cervical halter traction, and Halo application. However, the torticollis persisted. The patient then required surgical correction involving cervical spine fusion. She had no complications and experienced no reoccurrence of the torticollis to date.
Conclusion:
Grisel's syndrome is a pathology for which conservative management is successful in most cases. Cases requiring surgical intervention are rarely documented in the literature. Our case is significant, as in spite of aggressive conservative management, the patient required surgical correction. Patients requiring surgical management of Grisel's syndrome may require additional anesthetic exposure for diagnostic interventions like magnetic resonance imaging or neck manipulations for closed reduction. We discuss the features of Grisel's syndrome and specific anesthetic management considerations for procedures such as magnetic resonance imaging, application of cervical traction, and surgical correction of torticollis.
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