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Severe scoliosis associated with Costello syndrome: a case report
Authors:Motosuneya T  Asazuma T  Tsuji T  Watanabe H  Nakayama Y  Nemoto K
Affiliation:Department of Orthopaedic Surgery, National Defense Medical College, Namiki, Tokorozawa, Saitama, Japan. tmotosuneya@yahoo.co.jp
Abstract:Costello syndrome is characterised by dwarfism, unique cutaneous lesions, a distinct facial gestalt, and mental retardation. There have been no detailed reports of severe spinal deformities requiring surgical treatment as a complication of Costello syndrome. We report a case of a 10-year-old girl with progressive scoliosis associated with Costello syndrome. She underwent anterior release and posterior surgical correction and fusion from T5 to L2 using a third generation hook and rod system plus spinous process wiring. Congenital portal vein deficiency and coagulopathy were other major complications. At 15-month follow-up, the patient had good balance and no evidence of instrumentation failure.
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