title: Pulmonary and Radiographic Outcomes of VEPTR (Vertical Expandable Prosthetic Titanium Rib) Treatment in Early-Onset Scoliosis.
abstract: BACKGROUND
  VEPTR (vertical expandable prosthetic titanium rib) expansion thoracoplasty is used to manage thoracic insufficiency syndrome in early-onset scoliosis. Literature regarding the effects of this technique on pulmonary function is scarce. The aim of this study was to report the intermediate-term results of VEPTR expansion thoracoplasty.
  
  
  METHODS
  Twenty-one children with thoracic insufficiency syndrome underwent VEPTR expansion thoracoplasty from 2002 to 2012 and had complete chart data, preoperative and follow-up radiographs, and pulmonary function tests performed at the index implantation, first expansion, and last expansion. Pulmonary function tests with forced and passive deflation techniques developed for children under general anesthesia were performed prior to the index implantation and each expansion surgery under the same anesthetic conditions. Pulmonary and radiographic parameters were analyzed longitudinally.
  
  
  RESULTS
  Mean follow-up was six years, and mean age at implantation was 4.8 years. The mean number of expansion procedures per patient was eleven, and the mean number of pulmonary function tests was ten. The mean interval between surgical procedures was 6.4 months. Mean forced vital capacity (FVC) increased from 0.65 to 0.96 L (p < 0.0001). However, the percentage of the predicted FVC decreased from 77% to 58%. Respiratory system compliance normalized on the basis of body weight, Crs/kg, decreased by 39%, from 1.4 to 0.86 mL/cm H2O/kg. The mean Cobb angle before treatment was 80°, and the mean maximum thoracic kyphosis angle was 57° (range, 7° to 107°). The initial coronal correction was maintained at the time of final follow-up (67°); however, there was a trend toward a decrease in the maximum thoracic kyphosis angle (to 66°, p = 0.08). Clinically apparent proximal thoracic kyphosis occurred in four patients, and spinal imbalance occurred in seven. The mean gain in T1-T12 height during the treatment period was 18 mm (2.9 mm/year).
  
  
  CONCLUSIONS
  FVC improved over time; however, this increase in lung volume did not keep up with the growth of the child, as the percentage of the predicted FVC decreased, and the chest wall stiffness increased. Coronal correction was maintained, but the increase in proximal thoracic kyphosis is concerning.
  
  
  METHODS
  Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Ozgur
authors.initials: O
authors.lastname: Dede
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Etsuro K
authors.initials: EK
authors.lastname: Motoyama
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Charles I
authors.initials: CI
authors.lastname: Yang
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Rebecca L
authors.initials: RL
authors.lastname: Mutich
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Stephen A
authors.initials: SA
authors.lastname: Walczak
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Austin J
authors.initials: AJ
authors.lastname: Bowles
authors.affiliation: Department of Orthopaedic Surgery (O.D., A.J.B., and V.F.D.), Department of Anesthesiology (E.K.M. and C.I.Y.), and Division of Pediatric Pulmonology, Allergy and Immunology (E.K.M., R.L.M. and S.A.W.), Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center (UPMC), 4401 Penn Avenue, Pittsburgh, PA 15224. E-mail address for V.F. Deeney: Vincent.deeney@chp.edu.
authors.forename: Vincent F
authors.initials: VF
authors.lastname: Deeney
mesh_terms: D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D019736:Prostheses and Implants; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology; D012189:Retrospective Studies; D012272:Ribs / Q000601:surgery*; D012600:Scoliosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000601:surgery*; D013577:Syndrome; D013905:Thoracoplasty; D014025:Titanium; D016896:Treatment Outcome
chemical_list: D014025:Titanium