BACKGROUND: To present the first known reported case of late onset idiopathic scoliosis with concomitant neural anomalies, treated with sequential distraction using magnetic growth rod, had significant improvement in both cranio-cervical and intraspinal anomaly. METHODS: A caucasian, growing female child (at the age of ten) presented with moderately progressive late onset right thoracic scoliosis. She was found to have Chiari type I malformation and a cervicothoracic syrinx on routine pre-operative MRI scanning. We treated this child by inserting magnetic growing rod (MGR) system. After 48 months of follow up with serial distractions, the metalwork (MGR) was removed due to aseptic wound breakdown and granuloma formation. Subsequently due to the progression of deformity, a definitive posterior instrumented spinal fusion was done. A repeat MRI Scan of the Spine was done prior to this definitive procedure to assess for any residual neural anomalies. RESULTS: The Chiari type I malformation appeared to have completely resolved, with no cerebellar tonsillar herniation seen, and a significant improvement in the size of the cervicothoracic syringomyelia effectively downgrading it to a prominent central canal. CONCLUSIONS: This is a unique case of progressive late onset idiopathic scoliosis with associated Chiari malformation and syringomyelia, showing an improvement in these neural anomalies after gradual and protracted distractive lengthening of the spine with MGR.
BACKGROUND: To present the first known reported case of late onset idiopathic scoliosis with concomitant neural anomalies, treated with sequential distraction using magnetic growth rod, had significant improvement in both cranio-cervical and intraspinal anomaly. METHODS: A caucasian, growing female child (at the age of ten) presented with moderately progressive late onset right thoracic scoliosis. She was found to have Chiari type I malformation and a cervicothoracic syrinx on routine pre-operative MRI scanning. We treated this child by inserting magnetic growing rod (MGR) system. After 48 months of follow up with serial distractions, the metalwork (MGR) was removed due to aseptic wound breakdown and granuloma formation. Subsequently due to the progression of deformity, a definitive posterior instrumented spinal fusion was done. A repeat MRI Scan of the Spine was done prior to this definitive procedure to assess for any residual neural anomalies. RESULTS: The Chiari type I malformation appeared to have completely resolved, with no cerebellar tonsillar herniation seen, and a significant improvement in the size of the cervicothoracic syringomyelia effectively downgrading it to a prominent central canal. CONCLUSIONS: This is a unique case of progressive late onset idiopathic scoliosis with associated Chiari malformation and syringomyelia, showing an improvement in these neural anomalies after gradual and protracted distractive lengthening of the spine with MGR.
Entities:
Keywords:
Chiari malformation; idiopathic scoliosis; magnetic growing rod (MGR); syringomyelia
Authors: Steven W Hwang; Amer F Samdani; Andrew Jea; Ami Raval; John P Gaughan; Randal R Betz; Patrick J Cahill Journal: Childs Nerv Syst Date: 2012-04-18 Impact factor: 1.475
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