Surgical management of Grisel syndrome in the adult patient: illustrative case

Abstract

Background: Grisel syndrome describes an infectious soft tissue process that destabilizes the cervical bony elements and ligamentous complexes. This nontraumatic atlantoaxial rotary subluxation occurs in children primarily. This case illustrates a rare case presentation of an adult with Grisel syndrome: infectious destruction of the right atlantoaxial facet joint caused the occiput-C1 vertebra (head) to rotate rightward with lateral horizontal displacement off the C2 vertebra.

Observations: Because the infection destroyed the C1 bony arch and atlantoaxial facet joints with epidural extension, the rotated head and atlas pulled the brainstem-cervical spinal cord junction against a fixed odontoid process, resulting in a cord contusion. Because of the highly unstable craniocervical junction, the patient presented with torticollis and left upper extremity weakness.

Lessons: Treatment entailed closed reduction under general anesthesia followed by occipitocervical fusion with an occipital plate, C1 lateral mass screws, and C2-C5 pedicle screws. This case describes the unique surgical pearls necessary for occipitocervical fusion of an unstable craniocervical junction, including tips with neuronavigation, trajectories of the cervical pedicle screws, aligning the lateral mass and pedicle screws with the occipital plate, and nuances with occipitocervical distraction.

Keywords: Grisel syndrome; atlantoaxial; cervical; pedicle; rotary; subluxation; surgery.

FIG. 1.

Illustration of the surgical correction for Grisel syndrome in the adult patient. A: Preoperative view: nontraumatic atlantoaxial rotary subluxation with the head rotated toward the right. The inset demonstrates the kinking at the left brainstem–spinal cord junction because of rightward rotation and lateral displacement of the head. B: Postoperative fixation: the distractors on the occipital plate and C2 pedicle screws (trajectory of cervical pedicle screws shown in the inset) allow for correction of the cervical deformity. Notice the inset reveals realignment of the atlantoaxial segment and brainstem–spinal cord junction. Illustration by Chris Gralapp.

FIG. 2.

Preoperative CT. Sagittal CT demonstrates (A) destruction of the joint between the right occipital condyle and C1 superior articulation and (B) migration of the left C1 lateral mass anterior to the midline dens. Coronal CT demonstrates (C) the right lateral bending along the coronal plane, which caused the left C1 posterior arch to erode through the dens and C2 vertebral body. D and E: C4 vertebrae reveal generous pedicle sizes, well-suited for subaxial pedicle screws.

FIG. 3.

Preoperative magnetic resonance imaging (MRI). A: Notice epidural extension of the abscess on the preoperative MRI. Axial (B) and sagittal (C) MRI reveals a left-sided contusion at the cervicomedullary junction. Compression occurs because both the head and C1 are rotated and laterally displaced against the dens (at midline position). This rightward displacement kinks and contuses the left junction of the brainstem–spinal cord.

FIG. 4.

A: Intraoperative fluoroscopy confirms that the hard palate (dotted line) is perpendicular to the floor (straight line), which ensures a neutral gaze in occipitocervical fusions. B: Postoperative radiograph illustrating realignment of the head with the cervical spine. The lateral image reveals the surgeon’s preference for rod contouring. Also, notice that the superior direction of the C1 lateral mass and C2 pedicle screws shifts to an inferior direction of the C4 and C5 pedicle screws.

FIG. 5.

The proud occipital screw without the occipital plate serves as an anchor for the navigated reference frame.