We present the case of a 5-year-old boy presenting with a 54-degree scoliosis secondary to a Chiari I malformation with a holocord syringomyelia extending from C1 to T10. Neurosurgical treatment involved posterior fossa craniectomy with decompression, and partial C1 laminectomy. At follow-up 7 years later, at age 12, radiographs revealed only a 4-degree scoliosis, and follow-up MRI revealed a deflated syrinx. We report this case to reveal the most significant scoliosis regression seen in our experience that may occur in younger patients after neurosurgical syringomyelia decompression for Chiari I hindbrain herniation.
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