title: Treatment of scoliosis in patients affected with Prader-Willi syndrome using various techniques.
abstract: BACKGROUND
  The incidence of spinal deformity in children with Prader-Willi syndrome (PWS) is high, with 86% of these patients found to have a significant structural scoliosis; however, there are very few case reports describing surgical treatment for this deformity.
  
  
  METHODS
  The authors reviewed a case series consisting of 6 patients who underwent spine surgery for scoliosis. Children's mean age at index surgery was 12 years and 10 months (range, 10 to 15 yrs). Clinical evaluation revealed the typical phenotypic features of the PWS in all of the patients; 4 subjects had a karyotype confirmation of PWS. Major structural curves showed preoperative mean Cobb angles of 80.8 degrees (range, 65 degrees to 96 degrees ). Hybrid instrumentation with sublaminar wires, hooks and screws was used in the first 2 patients, while the remaining 4 were treated with titanium pedicle screw constructs.
  
  
  RESULTS
  The mean clinical and radiological follow-up was 3 years and 10 months (range, 2 years to 9 years). Major complication rate was 50%. One patient who developed a major intraoperative complication (paraparesis) prevented spinal fusion to be obtained: the neurologic deficit resolved completely after instrumentation removal. Solid arthrodesis and deformity correction in both coronal and sagittal plane was, however, achieved in the other 5 cases and no significant curve progression was observed at follow-up. Another major short-term complication was encountered 3 months after surgery in a patient who experienced the detachment of a distally located rod and required correction through revision surgery and caudal extension by one level. Cervico-thoracic kyphosis was seen in 1 patient who did not require revision surgery.
  
  
  CONCLUSIONS
  Spine reconstructive surgery in patients with PWS is rare and highly demanding.The best method of reconstruction is posterior multilevel pedicle screw fixation. Moreover, even with modern techniques, the risk of complications is still high. These new techniques, however, have shown to improve the postoperative course by allowing for immediate mobilization without any brace or cast. The use of the growing rod techniques, requiring repeated surgeries, should be carefully evaluated in each single case.
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Tiziana
authors.initials: T
authors.lastname: Greggi
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Martikos
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Lolli
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Georgios
authors.initials: G
authors.lastname: Bakaloudis
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Di Silvestre
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Alfredo
authors.initials: A
authors.lastname: Cioni
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Giovanni Barbanti
authors.initials: GB
authors.lastname: Bròdano
authors.affiliation: Spinal Deformity Surgery Department, Rizzoli Orthopaedic Institute, Via Pupilli, 1, 40126 Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Giacomini