Results of brace treatment of scoliosis in Marfan syndrome

Study Design. Retrospective review of a defined Marfan population with trad itional indications for bracing. Objectives. To determine the success rate of brace treatment in keeping cur ves from progressing by more than 5 degrees or exceeding 45 degrees. Summary of Background Data. Few studies exist regarding brace treatment of Marfan syndrome, and they include many patients with curves of more than 45 degrees, as well as some who are near maturity. All of the prior studies r isk the possibility of some selection bias. Methods. Patients were selected from support groups and several institution s. Inclusion criteria were: Definite diagnosis of Marfan syndrome, curve of 45 degrees or less, Risser sign 2,1, or 0 at inception of bracing, recomme nded wear of 18 hours or more per day, and follow-up until maturity or surg ery (minimum, 2 years). Success was defined as curve progression of 5 degre es or less and final curve remaining 45 degrees or less. Failure was a fina l curve of more than 45 degrees. 1 Twenty-four patients met the criteria. T here were 15 girls and 9 boys. Twenty-two patients wore a brace as recommen ded. Two additional patients were unable to tolerate it. Results. Mean age at inception of bracing was 8.7 I,years (range, 4-12 year s). There were 14 double major, 6 thoracic, and 4 thoracolumbar curves with a mean size of 29 degrees at the beginning of bracing. The stated wearing time averaged 21 hours per day. Five patients had significant ii-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 cons idered a failure. Mean progression was 6 degrees +/- 8 degrees per year, fo r a final mean curve of 49 degrees. Sixteen of the patients had, or were ad vised to have, surgical correction. The difference in age an degree of curv ature were not statistically significant 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 rea sons include increased progressive forces, altered transmission of correcti ve pressure to the spine?, and younger age at inception of bracing. Because there was no control group, it is unknown whether bracing slowed curve pro gression. Physicians should understand that most patients with Marfan syndr ome who have a curve of more than 25 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.