P D Sponseller1, M Bhimani, D Solacoff, J P Dormans. 1. Johns Hopkins Hospital, Baltimore, Maryland; and the Children's Hospital of Philadelphia, Pennsylvania, USA. psponse@jhmi.edu
Abstract
STUDY DESIGN: 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 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 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 degrees 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 degrees or less and final curve remaining 45 degrees or less. Failure was a final curve of more than 45 degrees. 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 degrees 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 degrees +/- 8 degrees per year, for a final mean curve of 49 degrees. 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 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.
STUDY DESIGN: 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 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 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 degrees 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 degrees or less and final curve remaining 45 degrees or less. Failure was a final curve of more than 45 degrees. 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 degrees 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 degrees +/- 8 degrees per year, for a final mean curve of 49 degrees. 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 degrees and a Risser sign of 2 or less will reach the surgical range, even with brace treatment.
Authors: Samuel B Rudnick; Hannah Zabriskie; Justin Ho; Christina A Gurnett; Matthew B Dobbs Journal: J Pediatr Orthop B Date: 2018-03 Impact factor: 1.041
Authors: Konstantinos C Soultanis; Alexandros H Payatakes; Vasilios T Chouliaras; Georgios C Mandellos; Nikolaos E Pyrovolou; Fani M Pliarchopoulou; Panayotis N Soucacos Journal: Scoliosis Date: 2007-10-23
Authors: Jillian G Buchan; David M Alvarado; Gabe E Haller; Carlos Cruchaga; Matthew B Harms; Tianxiao Zhang; Marcia C Willing; Dorothy K Grange; Alan C Braverman; Nancy H Miller; Jose A Morcuende; Nelson Leung-Sang Tang; Tsz-Ping Lam; Bobby Kin-Wah Ng; Jack Chun-Yiu Cheng; Matthew B Dobbs; Christina A Gurnett Journal: Hum Mol Genet Date: 2014-05-15 Impact factor: 6.150