title: One-stage sequential bilateral thoracic expansion for asphyxiating thoracic dystrophy (Jeune syndrome).
abstract: OBJECTIVE
  Jeune syndrome (asphyxiating thoracic dystrophy) is a rare disorder characterized by skeletal dysplasia, reduced diameter of the thoracic cage and extrathoracic organ involvement. Fatal, early respiratory insufficiency may occur. Two-stage lateral thoracic expansion has been reported, addressing each side sequentially over 3-12 months. While staged repair theoretically provides less invasive surgery in a small child with respiratory distress, we utilized a single stage, bilateral procedure aiming to rapidly maximize lung development. Combined bilateral surgery also offered the chance of rapid recovery, and reduced hospital stay. We present our early experience of this modification of existing surgical treatment for an extremely rare condition, thought to be generally fatal in early childhood.
  
  
  METHODS
  Nine children (6 males, 3 females; median age 30 months [3.5-75]) underwent thoracic expansion for Jeune syndrome in our centre. All patients required preoperative respiratory support (5 with tracheostomy, 8 requiring positive pressure ventilation regularly within each day/night cycle). Two children underwent sequential unilateral (2-month interval between stages) and 7 children bilateral thoracic expansion by means of staggered osteotomies of third to eighth ribs and plate fixation of fourth to fifth rib and sixth to seventh rib, leaving the remaining ribs floating.
  
  
  RESULTS
  There was no operative mortality. There were 2 deaths within 3 months of surgery, due to pulmonary hypertension (1 following two-stage and 1 following single-stage thoracic expansion). At the median follow-up of 11 months (1-15), 3 children have been discharged home from their referring unit and 2 have significantly reduced respiratory support. One child remains on non-invasive ventilation and another is still ventilated with a high oxygen requirement.
  
  
  CONCLUSIONS
  Jeune syndrome is a difficult condition to manage, but bilateral thoracic expansion offers an effective reduction in ventilator requirements in these children. While two-stage repair has been described previously, this is the first report of single-stage bilateral thoracic expansion. Single-stage repair is feasible and may offer better resource management and significant cost savings by potentially reducing theatre usage and overall length of stay (intensive care and hospital) without compromising clinical outcomes.
authors.affiliation: Department of Cardiothoracic Surgery, Great Ormond Street Hospital NHS Trust, London, UK prathinaga@gmail.com.
authors.forename: Nagarajan
authors.initials: N
authors.lastname: Muthialu
authors.affiliation: Department of Cardiothoracic Surgery, Great Ormond Street Hospital NHS Trust, London, UK.
authors.forename: Shafi
authors.initials: S
authors.lastname: Mussa
authors.affiliation: Department of Radiology, Great Ormond Street Hospital NHS Trust, London, UK.
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Owens
authors.affiliation: Department of Plastic and Reconstructive Surgery, Great Ormond Street Hospital NHS Trust, London, UK.
authors.forename: Neil
authors.initials: N
authors.lastname: Bulstrode
authors.affiliation: Department of Cardiothoracic Surgery, Great Ormond Street Hospital NHS Trust, London, UK.
authors.forename: Martin J
authors.initials: MJ
authors.lastname: Elliott
mesh_terms: D002648:Child; D002675:Child, Preschool; D004613:Ellis-Van Creveld Syndrome / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013902:Radiography, Thoracic; D012189:Retrospective Studies; D013896:Thoracic Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D019616:Thoracic Surgical Procedures / Q000295:instrumentation* / Q000379:methods*; D035441:Thoracic Wall / Q000000981:diagnostic imaging / Q000601:surgery*
keywords: Chest expansion; Jeune syndrome; Thoracic dystrophy