title: VEPTR: Are We Reducing Respiratory Assistance Requirements?
abstract: BACKGROUND
  The assisted ventilation rating (AVR) indicates the degree of external respiratory support required in children with thoracic insufficiency syndrome (TIS) and early onset scoliosis. For skeletally immature patients with TIS, the vertical expandable prosthetic titanium rib (VEPTR) device can be used to improve lung volume and growth. We hypothesized that patients who underwent early thoracic reconstruction by VEPTR treatment had an improved respiratory status.
  
  
  METHODS
  Preoperative and postoperative AVR ratings were prospectively collected in a multicenter study group and compared to determine change after VEPTR treatment. Patients under 10 years of age at initial implant with minimum of 2-year follow-up data were included. Patients were excluded if there were incomplete data or if initial AVR was normal (breathing on room air). Statistical analysis was performed on groups which had stable, declined, and improved AVR at final follow-up.
  
  
  RESULTS
  Database search yielded 77 patients with initial abnormal AVR. Average follow-up was 5.6 years. The most frequent primary diagnoses were congenital scoliosis (n=14) and spinal muscular atrophy (n=14). In total, 19 (24%) demonstrated improvement, 9 (12%) patients deteriorated, and 49 (64%) remained at the same level. The average preoperative major curve in those with improvement (58.4 degrees) and those with no change (63.5 degrees) was less than in those with deterioration (85.5 degrees) (P=0.014). The average age in years at implant of those with improvement (4) was less than those declined (6.7) and those with no change (5.5). In total, 16 (84.2%) of those that improved had a normal AVR and did not require respiratory support at last follow-up.
  
  
  CONCLUSIONS
  There is evidence that a subset of patients with early onset scoliosis and TIS who received early thoracic reconstruction with VEPTR treatment show complete resolution of pulmonary support at final follow-up. In total, 89% of 79 patients did not experience respiratory deterioration. A total of 24% (n=19) had a positive change with over 84% (n=16) of this group no longer requiring support.
  
  
  METHODS
  Level III-prognostic.
authors.affiliation: Shriners Hospital for Children, Philadelphia, PA.
authors.forename: Sarah B
authors.initials: SB
authors.lastname: Nossov
authors.affiliation: Monmouth Medical Center, Long Branch, NJ.
authors.forename: Evan
authors.initials: E
authors.lastname: Curatolo
authors.affiliation: Children's Hospital of Philadelphia (CHOP).
authors.forename: Robert M
authors.initials: RM
authors.lastname: Campbell
authors.affiliation: Children's Hospital of Philadelphia (CHOP).
authors.forename: Oscar H
authors.initials: OH
authors.lastname: Mayer
authors.affiliation: Children's Hospital Colorado, University of Colorado.
authors.forename: Sumeet
authors.initials: S
authors.lastname: Garg
authors.affiliation: Children's Hospital of Philadelphia (CHOP).
authors.forename: And Patrick J
authors.initials: APJ
authors.lastname: Cahill
mesh_terms: D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008168:Lung / Q000254:growth & development; D019736:Prostheses and Implants; D012121:Respiration, Artificial; D012272:Ribs / Q000601:surgery*; D012600:Scoliosis / Q000151:congenital / Q000601:surgery*; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology / Q000601:surgery*; D013577:Syndrome; D035441:Thoracic Wall / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D014025:Titanium; D016896:Treatment Outcome
chemical_list: D014025:Titanium