title: Correction of the axial and appendicular deformities in a patient with Silver-Russel syndrome.
abstract: BACKGROUND
  Scoliosis and limb length discrepancy are the major orthopaedic abnormalities in patients with Silver-Russel syndrome (SRS). In this paper, we describe a series of orthopaedic interventions in an attempt to overcome the progressive pathologic mechanism in a 7-year-old girl who manifested the full phenotypic features of SRS.
  
  
  METHODS
  Unilateral hip dislocation, progressive scoliosis and limb length discrepancy have been dealt with through Pemberton osteotomy, spinal fusion and Taylor-Spatial-Frame respectively.
  
  
  RESULTS
  In order to correct the axial and the appendicular deformities a sum of seven operations were performed (between the age of 7 years and 13 years). Pemberton osteotomy was performed to treat dislocation of her right hip because of developmental dysplasia of the hip. Spinal fusion (spondylodesis) of segments Th3-L5 was done to correct her scoliosis. And, to overcome the limb length discrepancy of 15-cm we used Taylor-Spatial-Frame with percutaneous distal corticotomy of the femur, and the proximal tibia, as well as the foot, were performed. We were able to minimize the limb length discrepancy to 5 cm. The girl became able to walk with the aid of a below knee orthosis and through lifting the left limb with 5-cm height shoe.
  
  
  CONCLUSIONS
  Limb lengthening surgery in patients with multiple malformation complex as in SRS is associated with high recurrence risk because of; muscular hypotonia, overtubulation of the long bones, and the poor bone regenerative quality. Our interventions were principally directed towards improving the cosmetic outlook, functions and the biomechanics.
authors: Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital; Department of Paediatric, Orthopaedic Hospital of Speising, Vienna, Austria.
authors: Ali
authors: A
authors: Al Kaissi
authors: Rudolf
authors: R
authors: Ganger
authors: Gabriel
authors: G
authors: Mindler
authors: Christoph
authors: C
authors: Karner
authors: Klaus
authors: K
authors: Klaushofer
authors: Franz
authors: F
authors: Grill
mesh_terms: D001852:Bone Lengthening; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D019637:Orthopedic Procedures / Q000379:methods*; D010027:Osteotomy; D056730:Silver-Russell Syndrome / Q000000981:diagnostic imaging / Q000601:surgery*; D013123:Spinal Fusion; D013997:Time Factors; D016896:Treatment Outcome