title: Thoracic elongation in type III osteogenesis imperfecta patients with thoracic insufficiency syndrome.
abstract: METHODS
  Prospective patient series.
  
  
  OBJECTIVE
  To evaluate outcome of children experiencing severe osteogenesis imperfecta (OI) with thoracic insufficiency syndrome (TIS), who were treated with a novel expandable spinothoracic fixation device.
  
  
  BACKGROUND
  Current treatment options for TIS in patients with OI are limited. As in other patients with TIS, thoracic elongation is the treatment of choice; however, the use of vertical expandable prosthetic titanium rib device is contraindicated for patients with poor bone quality.
  
  
  METHODS
  From 2007 to 2009, 4 patients with type III OI with TIS, aged 8 to 12 years, were treated with a novel expandable spinothoracic fixation device. Pre- and postoperative mobility, Cobb angle, PO2, PCO2, forced vital capacity, forced expiratory flow, use of accessory muscles when breathing, body weight, complication rates, and patient satisfaction were assessed.
  
  
  RESULTS
  Prior to surgery, 2 patients were wheelchair bound and 2 walked with assistive devices. Average follow-up was 24 months (range, 14-36 mo). Mean Cobb angle improved by up to 32% in the coronal plane. Pulmonary function improved in all patients, with mean increases of 45% in forced vital capacity, 93% in forced expiratory flow, and 43% in PO2. PCO2 declined 30% on average, returning to normal values. Mean patient weight at 6-month follow-up increased by 10.9%. Patients moved from less than 3rd weight percentile per age group to the 3rd to 7th percentiles at the 6-month follow-up, with continuing weight gain. There were no major complications. All patients and their caregivers were satisfied that the procedure led to improved self-image and functional level.
  
  
  CONCLUSIONS
  Thoracic elongation improved pulmonary function, facilitated weight gain, and made an indirect correction of spinal deformities (Cobb angle) by more than 30%, with no significant complications. Because of the rarity of this condition, a multicenter study to gain experience in a larger number of patients is recommended.
authors.affiliation: Spine Unit, Department of Orthopaedic Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. kapileon@netvision.net.il
authors.forename: Leon
authors.initials: L
authors.lastname: Kaplan
authors.forename: Yair
authors.initials: Y
authors.lastname: Barzilay
authors.forename: Amir
authors.initials: A
authors.lastname: Hashroni
authors.forename: Eyal
authors.initials: E
authors.lastname: Itshayek
authors.forename: Josh E
authors.initials: JE
authors.lastname: Schroeder
mesh_terms: D002648:Child; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D010013:Osteogenesis Imperfecta / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D011183:Postoperative Complications; D011446:Prospective Studies; D012123:Pulmonary Ventilation / Q000502:physiology*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D012272:Ribs / Q000601:surgery; D012600:Scoliosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D019616:Thoracic Surgical Procedures; D013909:Thorax; D015625:Tissue Expansion Devices; D016896:Treatment Outcome; D014797:Vital Capacity; D015430:Weight Gain