Grisel’s syndrome: A rare cause of torticollis with subluxation of the atlantoaxial joint secondary to a retropharyngeal abscess

• Main Authors: Michael D. White, David R. Hansberry, Arpan V. Prabhu, Nitin Agarwal, Vinay Kandula
• Format: Article
• Language: English
• Published: Elsevier 2020-12-01
• Series: Interdisciplinary Neurosurgery
• Subjects: Grisel’s syndrome; Torticollis; Retropharyngeal abscess; Subluxation; Grisel syndrome
• Online Access: http://www.sciencedirect.com/science/article/pii/S2214751920304333

Background: Atraumatic rotary subluxation of the atlantoaxial joint (Grisel’s syndrome) is a rare cause of torticollis. Although rare, prompt recognition and treatment of this disorder is essential in preventing permanent deformity.Case presentationA 4-year-old female with no significant past medical history presented to the emergency department (ED) after an outpatient positive streptococcus test with intermittent subjective fevers for one week. In the ED, the patient had a temperature of 103.0 F and was found to have a leukocytosis of 26,700 K/UL with a leftward shift of neutrophils. Initial radiograph of the neck found asymmetric soft tissue fullness and increased opacity in the right upper and mid cervical neck. Subsequently, contrast-enhanced computed tomography (CT) of the soft tissues of the neck found a right-sided retropharyngeal abscess measuring 1.5 cm. It was located medial to the carotid space with surrounding edema, inflammation, and fluid tracking inferiorly along the prevertebral soft tissues. There was associated lateral displacement of the right cervical internal carotid artery and jugular veins with regional stenosis. Additionally, there was atlantoaxial subluxation of C1 on C2. The patient was medically managed with intravenous antibiotics. The patient received dexamethasone for the edema and was admitted as an inpatient. She subsequently improved and had an uneventful hospital course with discharge to home. Conclusions: Grisel’s syndrome is an infrequent diagnosis, which is seen with atlantoaxial rotary subluxation accompanying infection or recent otolaryngolic surgery. Diagnosis typically requires cross-sectional imaging, often with CT. Without treatment with reduction and immobilization, there can be permanent fixed deformity.