• Jean-Charles Aurégan, Thierry Odent, Ryan M Coyle, Lotfi Miladi, Philippe Wicart, Jean Dubousset, Martine Le Merrer, Jean-Paul Padovani, and Christophe Glorion.
• *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.
• Spine. 2014 Apr 20;39(9):E564-75.

Study DesignA review of clinical publications, current knowledge, and recent developments regarding the etiology of ischiovertebral dysplasia was combined with a clinical review of the condition.ObjectiveTo 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.Summary Of Background DataIschiovertebral 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.MethodsThirty 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.ResultsAll 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).ConclusionRecognizing 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.Level Of Evidence4.

25 keywords...

hide…