title: Congenital kypho-scoliosis: a case of thoracic insufficiency syndrome and the limitations of treatment.
abstract: BACKGROUND
  Congenital spinal vertebral anomalies may present with deformity resulting in congenital scoliosis and kyphosis. This leads to abnormal spinal growth. The latter when combined with associated rib fusions may impair normal thoracic cage development and resultant pulmonary hypoplasia. Most congenital scoliosis can be detected in utero by ultrasound scan or recognized in the neonatal period, but a few spinal defects can remain undetected.
  
  
  METHODS
  In this Grand Round, we present the case of a 7-year-old girl with a severe scoliosis and thoracic insufficiency syndrome (TIS). 3D CT reconstruction imaging demonstrated a mixed picture of fusion and segmentation abnormalities. A marked kyphoscoliosis was demonstrated at the thoraco-lumbar junction. Via a left thoracotomy, anterior excision of intervertebral discs was performed together with, interbody fusion, and in situ stabilisation of the kyphosis with double allograft (femur) strut grafts.
  
  
  CONCLUSIONS
  This article highlights the features of congenital kypho-scoliosis and TIS. The difficulties of treating kyphosis when combined with TIS are discussed together with the limitations of current surgical techniques.
authors: Centre for Spinal Studies and Surgery, Queens Medical Centre, Derby Road, Nottingham, UK. deanchatterjee@doctors.net.uk
authors: A D
authors: AD
authors: Chatterjee
authors: K
authors: K
authors: Hassan
authors: M P
authors: MP
authors: Grevitt
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D007738:Kyphosis / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D009139:Musculoskeletal Abnormalities / Q000000981:diagnostic imaging / Q000601:surgery; D012272:Ribs / Q000002:abnormalities; D012600:Scoliosis / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D013123:Spinal Fusion; D013131:Spine / Q000002:abnormalities*; D013577:Syndrome; D056164:Teaching Rounds; D013896:Thoracic Diseases / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed