title: Developments in the Orthopaedic Management of Children With Stüve-Wiedemann Syndrome: Use of the Fassier-Duval Telescopic Rod to Maintain Correction of Deformity.
abstract: BACKGROUND
  Stüve-Wiedemann syndrome is a rare inherited condition, which is frequently fatal in infancy. Those patients who survive into childhood demonstrate a complex progressive deformity of the long bones, with high rates of recurrence after initial successful correction. Because of the rarity of the condition there is, at present, limited evidence on the most appropriate treatment.
  
  
  METHODS
  We describe our experience in the management of 4 patients, who underwent correction of deformity and fixation with Fassier-Duval telescopic rods.
  
  
  RESULTS
  In this series we have seen good correction of deformity and maintenance of alignment, with improvement in the walking ability of children treated with this technique.
  
  
  CONCLUSIONS
  Fassier-Duval rodding has a role in the prevention of recurrence of deformity and should be considered as a means to reduce the number of operative procedures required.
  
  
  METHODS
  Level IV-therapeutic.
authors.affiliation: Great Ormond Street Hospital for Sick Children, London, UK.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Wright
authors.forename: Sarmad
authors.initials: S
authors.lastname: Kazzaz
authors.forename: Robert A
authors.initials: RA
authors.lastname: Hill
mesh_terms: D002648:Child; D005097:Exostoses, Multiple Hereditary / Q000601:surgery*; D005260:Female; D005269:Femur / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D008297:Male; D009989:Orthotic Devices; D010009:Osteochondrodysplasias / Q000601:surgery*; D011859:Radiography; D013977:Tibia / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*