title: Ischiovertebral dysplasia: a retrospective analysis of 30 consecutive cases pointing out the specifics and risks of the spine management.
abstract: METHODS
  A review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition.
  
  
  OBJECTIVE
  To acquaint orthopedic spine surgeons with identification patterns of ischiovertebral dysplasia in order to provide them with guidelines about spine management and which complications to expect.
  
  
  BACKGROUND
  Ischiovertebral dysplasia is a rare skeletal dysplasia that may appear in a sporadic fashion or be inherited with an autosomal dominant inheritance pattern. It is defined by the association of an ischiopubic ramus hypoplasia and a vertebral dysplasia. It leads to a specific spine deformity whose management and complications should be clarified.
  
  
  METHODS
  Thirty consecutive patients from 0 to 31 years of age with ischiovertebral dysplasia were included from 5 centers specialized in congenital spinal deformities. Frontal and sagittal Cobb angles before treatment, natural history of the curves, therapeutic options, and their complications were systematically analyzed.
  
  
  RESULTS
  All the patients had a vertebral dysplasia and 28 of them developed a spinal deformity. This deformity was an extremely severe thoracic kyphoscoliosis in 25 cases. The other deformities were a thoracolumbar scoliosis in 1 case and a thoracolumbar kyphosis in 2 cases. The management of the thoracic kyphoscoliosis was always challenging and complications included death by respiratory failure (3 cases) and neurological impairment (9 cases).
  
  
  CONCLUSIONS
  Recognizing the occurrence of ischioverterbral dysplasia is very important to allow for dedicated treatment. The authors advocate preoperative distraction and circumferential fusion to prevent progression of the curve and to avoid the potentially fatal sequelae associated with this disorder.
  
  
  METHODS
  4.
authors: *Department of Pediatric Orthopedics, Necker Enfants Malades Hospital, AP-HP, University Paris Descartes, Paris, France †Department of Orthopedic Surgery and Traumatology, Columbia University Medical Center, New York, NY ‡Department of Pediatric Orthopedics, Saint Vincent de Paul Hospital, AP-HP, University Paris Descartes, Paris, France; and §Department of Genetics, Necker Enfants Malades Hospital, AP-HP, University Paris Descartes, Paris, France.
authors: Jean-Charles
authors: JC
authors: Aurégan
authors: Thierry
authors: T
authors: Odent
authors: Ryan M
authors: RM
authors: Coyle
authors: Lotfi
authors: L
authors: Miladi
authors: Philippe
authors: P
authors: Wicart
authors: Jean
authors: J
authors: Dubousset
authors: Martine
authors: M
authors: Le Merrer
authors: Jean-Paul
authors: JP
authors: Padovani
authors: Christophe
authors: C
authors: Glorion
mesh_terms: D000293:Adolescent; D000328:Adult; D001848:Bone Diseases, Developmental / Q000151:congenital / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007512:Ischium / Q000002:abnormalities* / Q000601:surgery; D008297:Male; D013121:Spinal Curvatures / Q000151:congenital / Q000601:surgery*; D013123:Spinal Fusion / Q000009:adverse effects*; D013131:Spine / Q000002:abnormalities* / Q000601:surgery; D055815:Young Adult