Article

Retrospective review of a defined Marfan population with traditional indications for bracing. Objectives. To determine the success rate of brace treatment in keeping curves from progressing by more than 5°or exceeding 45°. Summary of Background Data. Few studies exist regarding brace treatment of Marfan syndrome, and they include many patients with curves of more than 45°, as well as some who are near maturity. All of the prior studies risk the possibility of some selection bias. Methods. Patients were selected from support groups and several institutions. Inclusion criteria were: Definite diagnosis of Marfan syndrome, curve of 45°or less, Risser sign 2, 1, or 0 at inception of bracing, recommended wear of 18 hours or more per day, and follow-up until maturity or surgery (minimum, 2 years). Success was defined as curve progression of 5°or less and final curve remaining 45°or less. Failure was a final curve of more than 45°. Twenty-four patients met the criteria. There were 15 girls and 9 boys. Twenty-two patients wore a brace as recommended. Two additional patients were unable to tolerate it. Results. Mean age at inception of bracing was 8.7 years (range, 4-12 years). There were 14 double major, 6 thoracic, and 4 thoracolumbar curves with a mean size of 29°at the beginning of bracing. The stated wearing time averaged 21 hours per day. Five patients had significant pain over bony prominences. Although correction of the curve in brace was good (45%), only 4 of the patients had success, and in 20 of the 24 treatment was considered a failure. Mean progression was 6°± 8°per year, for a final mean curve of 49°. Sixteen of the patients had, or were advised to have, surgical correction. The difference in age and degree of curvature were not statistically significant between the success and nonsuccess groups. Conclusions. The success rate for brace treatment of Marfan scoliosis is 17%, which is lower than that reported for idiopathic scoliosis. Possible reasons include increased progressive forces, altered transmission of corrective pressure to the spine, and younger age at inception of bracing. Because there was no control group, it is unknown whether bracing slowed curve progression. Physicians should understand that most patients with Marfan syndrome who have a curve of more than 25°and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.

Langue : ANGLAIS

Identifiant basis : 2000213412