Case report

Progressive non-infectious anterior vertebral fusion, split cord malformation and situs inversus visceralis

Ali Al Kaissi^1,2 , Farid Ben Chehida³ , Maher Ben Ghachem² , Franz Grill⁴ and Klaus Klaushofer¹

¹Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 4th Medical Department, Hanusch Hospital. Heinrich Collins Str. 30 A-1140, Vienna, Austria

²Department of Paediatric Orthopaedic Surgery-Children Hospital of Tunis, Jabari, 1007, Tunisia

³Center of Radiology-Department of Imaging Studies-Ibn Zohr Institute, Tunis, City Khadra 1003, Tunisia

⁴Orthopaedic Hospital of Speising, Paediatric Department, Speisinger Str. 109, Vienna-1130, Austria

author email corresponding author email

BMC Musculoskeletal Disorders 2006, 7:94doi:10.1186/1471-2474-7-94

Published:	5 December 2006

Abstract

Background

Progressive non-infectious anterior vertebral fusion is a unique spinal disorder with distinctive radiological features. Early radiographic findings consist of narrowing of the anterior aspect of the intervertebral disk with adjacent end plate erosions. There is a specific pattern of progression. The management needs a multi-disciplinary approach with major input from the orthopaedic surgeon.

Case report

We report a 12-year-old-female with progressive anterior vertebral fusion. This occurred at three vertebral levels. In the cervical spine there was progressive fusion of the lateral masses of the Axis with C3. Secondly, at the cervico-thoracic level, a severe, progressive, anterior thoracic vertebral fusion (C7-T5) and (T6-T7) resulted in the development of a thick anterior bony ridge and massive sclerosis and thirdly; progressive anterior fusion at L5-S1. Whereas at the level of the upper lumbar spines (L1) a split cord malformation was encountered. Situs inversus visceralis was an additional malformation. The role of the CT scan in detecting the details of the vertebral malformations was important. To our knowledge, neither this malformation complex and nor the role of the CT scan in evaluating these patients, have previously been described.

Conclusion

The constellations of the skeletal abnormalities in our patient do not resemble any previously reported conditions with progressive anterior vertebral fusion. We also emphasise the important role of computerized tomography in the investigation of these patients in order to improve our understanding of the underlying pathology, and to comprehend the various stages of the progressive fusion process. 3D-CT scan was performed to improve assessment of the spinal changes and to further evaluate the catastrophic complications if fracture of the ankylosed vertebrae does occur. We believe that prompt management cannot be accomplished, unless the nature of these bony malformations is clarified.