title: Orthopedic Management of Scoliosis by Garches Brace and Spinal Fusion in SMA Type 2 Children.
abstract: BACKGROUND
  Scoliosis is the most debilitating issue in SMA type 2 patients. No evidence confirms the efficacy of Garches braces (GB) to delay definitive spinal fusion.
  
  
  OBJECTIVE
  Compare orthopedic and pulmonary outcomes in children with SMA type 2 function to management.
  
  
  METHODS
  We carried out a monocentric retrospective study on 29 SMA type 2 children who had spinal fusion between 1999 and 2009. Patients were divided in 3 groups: group 1-French patients (12 children) with a preventive use of GB; group 2-French patients (10 children) with use of GB after the beginning of the scoliosis curve; and group 3-Italian patients (7 children) with use of GB after the beginning of the scoliosis curve referred to our centre to perform orthopedic preoperative management.
  
  
  RESULTS
  Mean preoperative and postoperative Cobb angle were significantly lower in the group 1 of proactively braced than in group 2 or 3 (Anova p = 0.03; Kruskal Wallis test p = 0.05). Better surgical results were observed in patients with a minor preoperative Cobb angle (r = 0.92 p <  0.0001). Fewer patients in the group 1 proactively braced required trunk casts and/or halo traction and an additional anterior fusion in comparison with patients in the group 2 and 3. Moreover, major complications tend to be less in the group 1 proactively braced. No significant differences were found between groups in pulmonary outcome measures.
  
  
  CONCLUSIONS
  A proactive orthotic management may improve orthopedic outcome in SMA type 2. Further prospective studies comparing SMA management are needed to confirm these results.
  
  
  METHODS
  Therapeutic Level III. See Instructions to Authors on jbjs.org for a complete description of levels of evidence (Retrospective comparative study).
authors.affiliation: Unit of Neuromuscular and Neurodegenerative Disorders, Laboratory of molecular Medicine, Department of Neurosciences and Neurorehabilitation, Bambino Gesù Children's Research Hospital, IRCCS, Rome, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Catteruccia
authors.affiliation: Hospices Civils de Lyon,Hôpital Femme-Mère-Enfant, L'Escale, Service de Médecine Physique et de Réadaptation Pédiatrique, Bron, F-69500, France, Université de Lyon, Lyon, F-69000, France, CNRS UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, Equipe Biostatistique Santé, Pierre-Bénite, F-69310, France.|FILNEMUS, Réseau National Français de la Filière Neuromusculaire, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Vuillerot
authors.affiliation: AP-HP, Raymond Poincaré Hôpital, INSERM CIC 14.29, 92380 Garches, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Vaugier
authors.affiliation: Assistance Publique des Hôpitaux de Paris (AP-HP), Service de Pédiatrie, Hôpital Raymond Poincaré, Garches, Hôpitaux Universitaires Paris-Ile-de-France Ouest, France.|Centre de Référence de Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), France.
authors.forename: Danielle
authors.initials: D
authors.lastname: Leclair
authors.affiliation: Assistance Publique des Hôpitaux de Paris (AP-HP), Service de Pédiatrie, Hôpital Raymond Poincaré, Garches, Hôpitaux Universitaires Paris-Ile-de-France Ouest, France.|Centre de Référence de Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), France.
authors.forename: Viviane
authors.initials: V
authors.lastname: Azzi
authors.affiliation: Assistance Publique des Hôpitaux de Paris (AP-HP), Service de Pédiatrie, Hôpital Raymond Poincaré, Garches, Hôpitaux Universitaires Paris-Ile-de-France Ouest, France.|Centre de Référence de Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), France.
authors.forename: Louis
authors.initials: L
authors.lastname: Viollet
authors.affiliation: Assistance Publique des Hôpitaux de Paris (AP-HP), Service de Pédiatrie, Hôpital Raymond Poincaré, Garches, Hôpitaux Universitaires Paris-Ile-de-France Ouest, France.|Centre de Référence de Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), France.|FILNEMUS, Réseau National Français de la Filière Neuromusculaire, France.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Estournet
authors.affiliation: Unit of Neuromuscular and Neurodegenerative Disorders, Laboratory of molecular Medicine, Department of Neurosciences and Neurorehabilitation, Bambino Gesù Children's Research Hospital, IRCCS, Rome, Italy.
authors.forename: Enrico
authors.initials: E
authors.lastname: Bertini
authors.affiliation: Assistance Publique des Hôpitaux de Paris (AP-HP), Service de Pédiatrie, Hôpital Raymond Poincaré, Garches, Hôpitaux Universitaires Paris-Ile-de-France Ouest, France.|Centre de Référence de Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), France.|Université de Versailles-St Quentin, U1179 UVSQ - INSERM, Montigny, France.|FILNEMUS, Réseau National Français de la Filière Neuromusculaire, France.
authors.forename: Susana
authors.initials: S
authors.lastname: Quijano-Roy
keywords: Spinal muscular atrophy; orthotic device; scoliosis; spinal fusion