Chiari type I malformation (CM) and syringomyelia (SM) are closely associated but incompletely understood disorders of the craniovertebral junction and spinal cord. Children with CM+SM often suffer chronic pain, including headaches, spinal deformity, and neurological deficits such as sensory loss or weakness. When untreated, these conditions may progress and result in substantial, lifelong disability. Although neurosurgical intervention can halt or even reverse disease progression, the optimal surgical approach remains unclear. The traditional gold standard treatment, posterior fossa decompression with duraplasty (PFDD), involves surgical decompression of the craniovertebral junction along with opening of the dura and an expansile closure with graft material. PFDD is associated with significant morbidity (20% of patients experience clinically significant complications), prompting many physicians and patients to opt for a less invasive posterior fossa decompression without dural opening (PFD). Preliminary data suggest that PFD is associated with less harm to patients than PFDD, but whether it is as effective at halting symptomatic progression, reducing SM, or improving quality of life (QOL) remains unknown.
