Syringomyelia-associated scoliosis with and without the Chiari I malformation

Although there may be a hereditary component to true idiopathic scoliosis, the condition has no known cause and is not associated with dysraphism. However, scoliosis with an associated syrinx, with or without the Chiari I malformation, sometimes presents as an idiopathic-type curve. Physical examination findings and subtle clues on diagnostic imaging may help the orthopaedic surgeon diagnose scoliosis associated with syringomyelia. Examination findings include asymmetric reflexes and presentation at ages younger than those of patients who present with adolescent idiopathic curves (i.e., 10 to 14 years). Radiologic findings include kyphosis at the apex of the curve. Indications for surgical decompression include progressive neurologic deficits, weakness, pain, and progressive curves. Most orthopaedic surgeons agree that a syrinx should be evaluated neurosurgically before any planned spinal arthrodesis to decrease the risk of neurologic injury connected with surgical correction. The indications for arthrodesis in these patients compared with those with idiopathic curves are evolving.