Atlantoaxial rotatory subluxation in patients with Marfan syndrome. A report of three cases.

METHODS

This is a retrospective case series of three patients, ages 9 1/2, 13, and 20 years old, with Marfan syndrome treated for atlantoaxial rotatory subluxation. In the first two cases, acute torticollis was noted postoperatively, following pectus excavatum repair. The diagnosis was made in the third patient after she presented to the emergency room with a week-long history of unresolved neck pain following minor trauma.

OBJECTIVE

To report and discuss the courses and clinical sequelae of atlantoaxial subluxation in patients with Marfan syndrome.

BACKGROUND

Radiographic analysis of patients with Marfan syndrome has shown that increased atlantoaxial translation, larger odontoid height, and basilar impression are more prevalent in this population compared to age-matched controls. Despite these findings, there are sparse data on injuries secondary to cervical spine instability or abnormalities in this population. To the authors' best knowledge, no report of atlantoaxial rotatory subluxation in patients with Marfan syndrome exists in the literature.

METHODS

Case records of rotatory instability of the atlanto-axial level were reviewed and are presented in the following report.

RESULTS

The first two patients described in this report were noted to have "cock robin" posturing of their necks following pectus excavatum repairs. The first patient's subluxation was partially reduced with halo traction, and he subsequently underwent posterior spinal fusion of C1-C2 with internal fixation. The patient was well aligned postoperatively, and had no neurologic deficits. The second patient's subluxation reduced after 20 days of halter and traction; he was immobilized in a collar following discharge and reduction was maintained. The third patient's subluxation failed to reduce with halo traction; further imaging studies revealed odontoid prominence in the foramen magnum. She underwent posterior spinal fusion, occiput to C3, with satisfactory result.

CONCLUSIONS

The cervical bony and ligamentous abnormalities seen in patients with Marfan syndrome may slightly increase their risk for atlantoaxial rotatory instability. Special attention to intubation and positioning, both intraoperatively and postoperatively, may be necessary in patients with Marfan syndrome. Additionally, rotatory subluxation should be included in the differential diagnosis for Marfan patients with neck pain after injury.