title: Computer navigation-assisted spinal fusion with segmental pedicle screw instrumentation for scoliosis with Rett syndrome: a case report.
abstract: Scoliosis is a common clinical manifestation of Rett syndrome, a neurodevelopmental disorder that almost exclusively affects females. The spinal curve in patients with Rett syndrome is typically a long C curve of a neuromuscular type. As the onset of the scoliosis is very early and shows rapid progression, early surgical intervention has been recommended to prevent a life-threatening collapsing spine syndrome. However, there are high perioperative risks in Rett syndrome patients who undergo spinal surgery, such as neurological compromise and respiratory dysfunction due to rigid spinal curve. We herein report the surgical result of treating severe rapid progressive thoracic scoliosis in a 16-year-old girl with Rett syndrome. Posterior segmental pedicle screw fixation was performed from T1 to L3 using a computer-assisted technique. Post-operative radiography demonstrated a good correction of the curve in both the sagittal and coronal alignment. There were no postoperative complications such as neurological compromise. The patient had maintained satisfactory spinal balance as of the 3-year follow-up examination.
authors.affiliation: Department of Orthopaedic Sugery, Okayama University Hospital, Okayama, Japan. tanakam@md.okayama-u.ac.jp
authors.forename: Masato
authors.initials: M
authors.lastname: Tanaka
authors.forename: Kazuo
authors.initials: K
authors.lastname: Nakanishi
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Sugimoto
authors.forename: Haruo
authors.initials: H
authors.lastname: Misawa
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Takigawa
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Nishida
authors.forename: Toshifumi
authors.initials: T
authors.lastname: Ozaki
mesh_terms: D000293:Adolescent; D001863:Bone Screws; D005260:Female; D006801:Humans; D015518:Rett Syndrome / Q000150:complications / Q000601:surgery; D012600:Scoliosis / Q000209:etiology / Q000601:surgery; D013123:Spinal Fusion / Q000295:instrumentation / Q000379:methods; D025321:Surgery, Computer-Assisted; D016896:Treatment Outcome