Surgical treatment for atlantoaxial subluxation with myelopathy in spondyloepiphyseal dysplasia congenita

Spine (Phila Pa 1976). 2004 Nov 1;29(21):E488-91. doi: 10.1097/01.brs.0000143621.37688.f3.


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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Atlanto-Occipital Joint / diagnostic imaging
  • Atlanto-Occipital Joint / surgery*
  • Bone Transplantation
  • Braces
  • Child
  • Child, Preschool
  • Decompression, Surgical
  • Female
  • Follow-Up Studies
  • Foramen Magnum / surgery
  • Humans
  • Joint Dislocations / complications
  • Joint Dislocations / diagnostic imaging
  • Joint Dislocations / surgery*
  • Laminectomy*
  • Male
  • Middle Aged
  • Odontoid Process / pathology
  • Osteochondrodysplasias / complications
  • Osteochondrodysplasias / congenital*
  • Preoperative Care
  • Radiography
  • Retrospective Studies
  • Severity of Illness Index
  • Spinal Cord Compression / etiology*
  • Spinal Fusion*
  • Suture Techniques
  • Traction
  • Treatment Outcome