Grisel's syndrome: the two-hit hypothesis-A case report and literature review

Ear, Nose & Throat Journal, August, 2004 by Andrew P. Battiata, George Pazos

Abstract

Grisel's syndrome is a rare but well-documented clinical entity. It is a nontraumatic, fixed rotary subluxation of C1 on C2 (atlantoaxial). Although first described in 1830, the exact mechanism of Grisel's syndrome remains unclear. We present a postoperative case of Grisel's syndrome and an extensive literature review, and we propose a mechanism for its pathogenesis. In addition, we propose a treatment algorithm for Grisel's syndrome.

Introduction

Grisel's syndrome, previously described as a nontraumatic rotary subluxation of C1 on C2 (atlantoaxial subluxation) without any prior history of osteopathy, was first described by Bell in 1830 as a consequence of a syphilitic ulceration of the pharynx, (1) but since has assumed the name of Grisel, a French otolaryngologist who described a similar syndrome following a course of nasopharyngitis in 1930. (2) Grisel's syndrome has been described in the otolaryngologic, neurosurgical, and orthopedic literature as a rare consequence of inflammatory, infectious, and/or postsurgical complication in the head and neck. Since its first description, the exact pathogenesis of Grisel's syndrome has been the subject of much debate. No universally accepted mechanism for its occurrence exists, primarily because of the rarity of its presentation and its relative predilection for the pediatric population.

In this case report and literature review, we describe a case of Grisel's syndrome and provide a critique of several proposed explanations for its pathogenesis. Finally, we provide a logical treatment approach based on our literature review.

Case report

An otherwise healthy 11-year-old boy underwent an uncomplicated tonsillectomy for recurrent tonsillitis. He had no history of trauma or preexisting bone disease. On postoperative day 3, the patient presented with increasing neck pain. There was no history of fever, chills, sweats, nausea, vomiting, or neurologic symptoms. The patient was evaluated and on physical examination was found to have a torticollis (chin down and to the left). Cervical range of motion was severely limited. There was no trismus. Intraoral examination revealed a normally healing surgical field. There was significant muscular spasm. A positive Sudeek's sign was noted (spinous process of C2 displaced to the same side toward which the head is turned). Contrast computed tomography (CT) of the neck revealed a significant C1-C2 rotary subluxation (figure 1), consistent with a Fielding's type 2 rotary subluxation. No abscess or significant lymphadenopathy was present.

[FIGURE 1 OMITTED]

The patient was immediately placed in a soft cervical collar, and a neurosurgical consult was obtained. He was treated with appropriate oral antibiotics, intravenous muscle relaxants, and bed rest. Three-dimensional computer-generated reconstructions derived from the CT scans of this patient were obtained, which further illustrate the C1-C2 subluxation (figure 2). Within 24 hours the patient's torticollis resolved, as did the muscle spasm. CT and MRI scans performed on postoperative day 5 revealed complete resolution of the subluxation. The patient was maintained in soft cervical immobilization for 2 weeks, after which flexion-extension radiographs were obtained and found to be within normal limits. The collar was removed, and the patient did well with no lasting limitation or deficits.

[FIGURE 2 OMITTED]

Discussion

As previously noted, Grisel's syndrome is a rare clinical phenomenon, primarily affecting the pediatric population, with 68% of patients under the age of 12 years (3) and 90% under the age of 21. (4) Depending upon how cases are categorized, they have been reported most commonly following surgical procedures in the head and neck (14 of 62, 22.6%), (4) most often after mastoidectomy, tonsillectomy, and adenoidectomy, in that order. Upper respiratory infection was the second most commonly associated syndrome (12 of 62, 19.4%). (4) Gourin et al reported 21 of 78 (26.9%) cases as postoperative complications, and 14 of 78 (18%) secondary to upper respiratory infection. (3) There are no reports of predilection based on gender (5) or predominance of the side affected.

Patients typically present with a painful torticollis, possible history of fever, and other nonspecific signs of infection. On physical examination, significant muscle spasm is evident, and patients typically have a fixed torticollis. A positive Sudeck's sign is commonly observed. Although diagnosis of Grisel's syndrome is clinical, confirmation of the diagnosis is radiographic. We feel that CT of the neck is the gold standard for diagnosis. The scan should be performed with contrast enhancement to rule out abscess formation. Although rotary subluxation can be diagnosed with plain radiographs, CT is more precise and is essential to rule out deep-space infection and abscess formation.

Grisel's syndrome has been previously described, but its etiology is debated. Although several theories have been proposed, no mechanism for its pathogenesis has been generally accepted.