title: The efficacy of rib-based distraction with VEPTR in the treatment of early-onset scoliosis in patients with arthrogryposis.
abstract: BACKGROUND
  Although arthrogryposis multiplex congenital (AMC) is a rare condition, rapidly progressive scoliosis is common in children with AMC. Only a limited number of studies characterize the nature of these curves, and even fewer describe surgical outcomes. To determine efficacy or rib-based distraction in these patients, we reviewed the outcomes of the use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device in what we believe is the first study to report this.
  
  
  METHODS
  Search of the Chest Wall Spinal Deformity Study Group database identified 10 children with AMC and early-onset scoliosis who were treated with the VEPTR device at 6 different pediatric health centers. The 7 female and 3 male patients had their initial surgery at an average age of 5 years. Mean follow-up was 4.2 years.
  
  
  RESULTS
  The most common curve was from T5 to L2. After initial VEPTR insertion, the scoliosis decreased from a mean of 67 to 43 degrees (37% correction) and kyphosis from 65 to 48 degrees (29% correction). The mean proximal junctional kyphosis after initial insertion was 33 degrees. At final follow-up, scoliosis and kyphosis were 55 degrees (17% correction) and 62 degrees (8% correction), respectively. Spinal growth during the treatment period showed a mean T1-S1 increase of 4.2 cm or approximately 1 cm/y. In the 62 procedures performed over the course of the study period, 6 complications occurred in 4 patients: 3 infections, 2 rib failures, and 1 implant failure. Six patients had proximal junctional kyphosis of ≥45 degrees at the last follow-up.
  
  
  CONCLUSIONS
  In children with AMC, rib-based distraction using the VEPTR is an effective treatment method for controlling scoliosis and kyphosis and maintaining thoracic growth, but proximal junctional kyphosis remains a problem.
authors: *Campbell Clinic, Le Bonheur Children's Hospital, Memphis, TN †Children's Hospital ∥Shriners Hospital, Philadelphia, PA ‡Hospital de la Concepcion, San German, PR §Boston Children's Hospital, Boston, MA ¶Shriners Hospital, Portland, OR.
authors: Nelson
authors: N
authors: Astur
authors: John M
authors: JM
authors: Flynn
authors: John M
authors: JM
authors: Flynn
authors: Norman
authors: N
authors: Ramirez
authors: Michael
authors: M
authors: Glotzbecker
authors: Harold J
authors: HJ
authors: van Bosse
authors: Jane S
authors: JS
authors: Hoashi
authors: Charles R
authors: CR
authors: d'Amato
authors: Derek M
authors: DM
authors: Kelly
authors: William C
authors: WC
authors: Warner
authors: Jeffrey R
authors: JR
authors: Sawyer
mesh_terms: D000367:Age Factors; D017668:Age of Onset; D001176:Arthrogryposis / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D019857:Osteogenesis, Distraction / Q000295:instrumentation* / Q000379:methods; D019736:Prostheses and Implants; D011474:Prosthesis Design; D019919:Prosthesis Implantation / Q000379:methods*; D012272:Ribs / Q000000981:diagnostic imaging / Q000601:surgery*; D018570:Risk Assessment; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000601:surgery*; D012720:Severity of Illness Index; D013131:Spine / Q000254:growth & development; D013997:Time Factors; D014025:Titanium; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D014025:Titanium