Two-level thoracic pedicle subtraction osteotomy for progressive post-laminectomy kyphotic deformity following resection of an unusual thoracolumbar intradural extramedullary tumor

Case report

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  • 1 Departments of Neurosurgery,
  • 2 Neuroradiology,
  • 3 Neuropathology, and
  • 4 Pediatric Orthopedic Surgery, Yale University School of Medicine, New Haven, Connecticut
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The authors report a case in which multilevel thoracic pedicle subtraction osteotomy (PSO) was performed to correct post-laminectomy kyphotic deformity in a 9-year-old boy presenting with worsening lower-extremity neurological deficits. Five years prior to presentation, the patient underwent multilevel thoracolumbar laminectomies for resection of an atypical teratoid/rhabdoid tumor (AT/RT), a rare lesion that typically occurs intracranially and has a poor prognosis, making this particular presentation unusual and the patient's subsequent postoperative course remarkable. No fusion was undertaken at the time of resection, given the patient's age and presumptive poor prognosis. Over the next 5 years, the patient developed progressive thoracolumbar kyphotic deformity, with a Cobb angle greater than 110°, despite bracing, and bilateral lower-extremity weakness requiring ankle-foot orthotics for continued ambulation due to progressive foot drop. Worsening gait and the onset of respiratory issues prompted surgical intervention. Multilevel thoracic PSO and thoracolumbar fusion were performed, resulting in improved lower-extremity function and correction of the kyphotic deformity to approximately 65°. This report outlines an unusual AT/RT presentation and postoperative course and also discusses literature related to PSO within the context of pediatric kyphotic deformity. The authors' experience supports the use of multilevel PSO with fusion as a potential treatment option for significant pediatric thoracolumbar kyphotic deformity requiring surgical correction.

Abbreviations used in this paper:AT/RT = atypical teratoid/rhabdoid tumor; EMA = epithelial membrane antigen; PSO = pedicle subtraction osteotomy; SMA = smooth muscle actin.

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Contributor Notes

Address correspondence to: Brian J. Kelley M.D., Ph.D., Yale Spine Institute–Yale University School of Medicine, P.O. Box 208082, New Haven, Connecticut 06520-8082. email: brian.kelley@yale.edu.

Please include this information when citing this paper: published online August 3, 2012; DOI: 10.3171/2012.7.PEDS11526.

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