title: Spinal Deformity in Bethlem Myopathy.
abstract: METHODS
  Retrospective review.
  
  
  OBJECTIVE
  To report the characteristics of spinal deformity in a series of 3 patients with Bethlem myopathy.
  
  
  BACKGROUND
  Bethlem myopathy presents with mild muscular weakness and typically has a benign course. Severe scoliosis in patients affected with Bethlem myopathy has not been previously reported.
  
  
  METHODS
  Clinical records of 3 brothers with Bethlem myopathy were reviewed. Clinical and radiographic features of the spinal deformity are presented.
  
  
  RESULTS
  All 3 patients had progressive scoliosis with coronal and sagittal imbalance. At a minimum of 26 months of follow-up after posterior instrumented fusion, there were no complications and deformity correction was maintained. Posterior instrumentation and fusion did not negatively affect the pulmonary function in this group of patients with Bethlem myopathy.
  
  
  CONCLUSIONS
  Bethlem myopathy may present with severe scoliosis along with proximal muscle weakness. This condition should be included in the differential diagnosis of adolescent patients with progressive spinal deformity.
authors.affiliation: Department of Orthopaedic Surgery, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA. Electronic address: ozgurdede@gmail.com.
authors.forename: Ozgur
authors.initials: O
authors.lastname: Dede
authors.affiliation: Department of Pediatrics, Division of Child Neurology, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.
authors.forename: Hoda Z
authors.initials: HZ
authors.lastname: Abdel-Hamid
authors.affiliation: Department of Orthopaedic Surgery, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.
authors.forename: Vincent F
authors.initials: VF
authors.lastname: Deeney
keywords: Muscular dystrophy; Scoliosis; Syndromic