Spinal correction of scoliosis in Jeune syndrome: a report of two cases.

BACKGROUND

Jeune syndrome (asphyxiating thoracic dystrophy) is an autosomal recessive disorder with constriction and narrowing of the thorax. To our knowledge, there are no reports regarding spinal deformity and correction in Jeune syndrome. Herein, we report two cases of spinal correction in patients with Jeune syndrome, and review the literature.

CASE PRESENTATION

We experienced cases of spinal scoliosis in an adolescent boy and a young adult woman, both with Jeune syndrome. Their spinal deformities had progressed by the time they came to our hospital for surgical correction. After preoperative evaluation of their general condition, including respiratory function in detail to confirm that they could undergo surgery, we treated both cases with posterior spinal correction and fusion. Spinal correction was performed safely and there were no severe complications, including respiratory depression, associated with surgery, and relatively satisfactory correction was obtained in both cases. In case 1, coronal deformity was corrected from 70° to 36° and from 82° to 42°, respectively. In case 2, Cobb angle was corrected from 52° to 20° and from 55° to 21°.

CONCLUSIONS

Posterior spinal correction can be performed safely in young patients with Jeune syndrome who have survived their infant stage and matured without a severe general condition.