Non-traumatic Atlantoaxial Subluxation Following Pharyngitis in Healthy Children: A Case Series

Grisel syndrome is a rare condition characterized by non-traumatic rotary subluxation of the atlantoaxial joint. It is commonly seen in pediatric patients, but can also be seen in adults. Although rare and with only a few reported cases, a high index of suspicion is required for prompt recognition and early treatment of this condition, as it is essential in preventing permanent deformity and neurological deficits. We report two pediatric cases; the first was a 13-year-old girl presenting to the pediatric emergency department with neck pain and head tilt for two weeks. She had a history of pharyngitis but no trauma. On examination, there was neck pain on movement with torticollis but no midline tenderness. CT confirmed Atlantoaxial subluxation. The second case was a three-year-old boy who presented with an acute onset of neck pain. He had a history of fever with throat pain one week before presentation, and no history of trauma. On examination, there was neck pain on movement with torticollis. CT of the cervical spine in both cases showed evidence of Atlantoaxial subluxation. The first case was offered conservative management with excellent clinical improvement and complete resolution of symptoms and signs. However, the second case was offered conservative management, antibiotics, and analgesia, but unfortunately, no prognosis was known for this patient as he was lost to follow-up. Children presenting with torticollis with a recent history of fever or pharyngitis and without a history of trauma should raise suspicion of Grisel's syndrome. Surgical intervention is indicated only in cases of high-grade instability. Early diagnosis allows for treatment without the need for surgery in most patients; surgical intervention is only indicated in the presence of severe instability.