Single stage reduction and stabilization of basilar invagination after failed prior fusion surgery in children with Down's syndrome.
METHODS
We describe an innovative single-stage reduction and stabilization technique using modern cervical instrumentation.


OBJECTIVE
We hypothesis modern instrumentation has made more aggressive surgical corrections possible and has reduced the need for transoral resection of the odontoid and traction reduction in children with basilar invagination.


BACKGROUND
Craniocervical junction abnormalities, including atlantoaxial instability and progressive basilar invagination, are relatively common phenomenon in Down's syndrome patients, and can lead to chronic progressive neurologic deficits, catastrophic injury, and death. This patient population also can be a difficult one in which to perform successful stabilization and fusion.


METHODS
We reviewed the records and films on 2 children with Down's syndrome and atlantoaxial instability who had undergone prior occipital-cervical fusion and then presented with symptomatic progressive basilar invagination due to atlantoaxial displacement. In both cases, the children had progressive symptoms of spinal cord and brain stem compression. Multiple approaches for surgical correction, including preoperative traction and transoral odontoid resection, were considered, but ultimately it was elected to perform a single stage posterior operation. In both patients, we performed fusion takedown, intraoperative realignment with reduction of the basilar invagination, and stabilization using modern occipito-cervical instrumentation.


RESULTS
In both children, excellent cranio-cervical realignment was achieved; along with successful fusion and improvement in clinical symptoms.


CONCLUSIONS
In this article we will discuss the clinical cases and review the background of craniocervical junction abnormalities in Down's syndrome patients. We hypothesis modern instrumentation has made more aggressive surgical corrections possible and has reduced the need for transoral resection of the odontoid and traction reduction in children with basilar invagination.
Hedequist
Daniel
D
Department of Orthopedic Surgery, Children's Hospital Boston, Boston, MA, USA.
Bekelis
Kimon
K
Emans
John
J
Proctor
Mark R
MR
D001268:Atlanto-Axial Joint / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D001915:Braces; D002648:Child; D004314:Down Syndrome / Q000503:physiopathology / Q000601:surgery*; D005684:Gait; D006801:Humans; D007103:Immobilization / Q000295:instrumentation; D007593:Joint Instability / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D007796:Laminectomy / Q000009:adverse effects* / Q000295:instrumentation; D008279:Magnetic Resonance Imaging; D008297:Male; D011542:Pseudarthrosis / Q000209:etiology / Q000601:surgery; D020127:Recovery of Function; D012086:Reoperation; D013117:Spinal Cord Compression / Q000209:etiology / Q000601:surgery; D013123:Spinal Fusion / Q000009:adverse effects* / Q000295:instrumentation; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D014143:Traction; D017211:Treatment Failure