Surgical treatment for atlantoaxial subluxation with myelopathy in spondyloepiphyseal dysplasia congenita.

STUDY DESIGN: A retrospective review of 21 patients with spondyloepiphyseal dysplasia congenita (SEDC), including 7 operated patients for atlantoaxial subluxation.

OBJECTIVES: To clarify the morphological findings of atlantoaxial subluxation in SEDC patients and to evaluate the operative procedures based on these image findings.

SUMMARY AND BACKGROUND DATA: The presence of atlanto axial sublucation with hypoplasia of the odontoid and/or lax ligaments leads to myelopathy in patients with spondyloepiphyseal dysplasia congenita.

METHODS: We retropectively reviewed the physical and morphological findings on atlantoaxial images and the clinical findings of myelopathy in 21 patients with SEDC.

RESULTS: Myelopathy was found in 9 individuals with severe SEDC who presented with marked short stature and severe coxa vara; of these, 6 had gait disturbances. On the images of the 9 patients with myelopathy, the average sagittal canal diameter (SCD) at the level of the atlas was only 9.2 mm (range, 7-12 mm) with progressive atlantoaxial subluxation. The average atlantodental interval (ADI) was 3.5 mm (range, 2-6 mm) in the presence of a sagittal atlas diameter (SAD) of less than 27.1 mm (range, 22-36 mm). Surgery was performed for 6 of the patients with myelopathy. Since their SADs were small, and the average SCD, at 9.9 mm (8-14 mm), was narrow even at the position of extension (the position of reduction for atlantoaxial subluxation), C1 laminectomy was needed for all these patients, and occipital-cervical posterior fusion was performed. Stability was satisfactory in all cases and the operative outcome for myelopathy was excellent for 1 case, fine for 4, and fair for 2.

CONCLUSION: A small SAD may limit the effectiveness of reducing atlantoaxial subluxation. Persistent narrowing of the SCD may require concomitant C1 laminectomy and occipital-cervical fusion.