Cervical Spine Subluxation in Marfan Syndrome

Abstract

Subluxation of the cervical spine may occur in patients secondary to trauma. Patients with Down syndrome and rheumatoid arthritis can have spontaneous cervical spine subluxation. Although cervical spine abnormalities are common in patients with Marfan syndrome, clinical problems are rare1. Lumbar subluxation is rarely seen in patients with this condition2 and, in an extensive review of cervical spine involvement in patients with Marfan syndrome, Hobbs et al. noted no cases of cervical subluxation1. We describe the clinical features and surgical treatment of a patient with Marfan syndrome who had progressive multilevel cervical subluxation. The patient was informed that information concerning this case would be submitted for publication. Athirteen-year-and-four-month-old boy presented to the Spine Service with right-sided neck pain associated with decreased range of motion. There was no history of trauma or recent illness. The medical history was unremarkable. The range of neck motion was flexion to 45°, extension to 10°, left axial rotation to 60°, and right axial rotation to 20°. The patient had mild upper cervical spine tenderness and had normal findings on neurological examination. Initial radiographic examination revealed anterior subluxation of C2 on C3, with marked segmental kyphosis (Fig. 1). Magnetic resonance imaging revealed disc-space changes at C2-C3 that were consistent with infection. The facet joints were aligned. Given the patient's complaints of pain and the finding of cervical subluxation on plain radiographs, the patient was admitted for evaluation and treatment. Computed tomography revealed no evidence of fracture. A bone scan with SPECT (single photon emission computed tomography) imaging showed slight posterior uptake at C2-C3 consistent with trauma. The findings of the laboratory evaluation were unremarkable and included a normal white blood-cell count, erythrocyte sedimentation rate, and C-reactive protein level. The patient was managed with skeletal traction with use of tongs, with easy …