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Spondylocarpotarsal synostosis syndrome (with a posterior midline unsegmented bar)
A Al Kaissi1, M Ben Ghachem, N Nassib
1Service d'orthopedie infantile, Hospital d'Enfants, Tunis, Tunisia.
Abstract:
Spondylocarpotarsal synostosis syndrome (SSS) is characterised by malsegmentation of the thoracic spine and carpal/tarsal fusions. A unilateral or bilateral unsegmented bar may be present in the thoracic spine. Presenting clinical signs are congenital scoliosis early in life, and shortening of the trunk with scoliosis and/or lordosis in older children. We report a 13-year-old girl with SSS and a midline unsegmented bar running along the spinal processes of T3 to L2 and extending into the posterior vertebral elements.
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