Atypical S-curve scoliosis associated with a Chiari 1 malformation and secondary syrinx causing arm numbness while playing Volleyball.
Surgical decompression of the skull and shunt placed to drain the syrinx.
Bracing for scoliosis and plan for surgical correction if brace fails.
Patient is doing well. She has some residual nausea from the surgical decompression which may subside with time.
This case illustrates many important clinical nuances regarding the presentation of thoracic-based scoliotic curves with subtle neurologic symptoms and associated central nervous system lesions.
Thoracic curves in children should always raise suspicion for underlying neurologic lesions. Scoliosis itself is a complex problem whose pathogenesis is still being understood and described (Kouwenhoven, Spine 2007).
Chari I malformation can often present asymptomatically and are discovered as the result of serendipitous imaging. When symptoms are present, they usually present late in childhood or early adulthood, most often with headache precipitated by sudden coughing, sneezing or straining. Patients with Chiari Type I Malformation can also experience:
• Neck pain (running down the shoulders at times)
• Unsteady gait (problems with balance)
• Poor hand coordination (fine motor skills)
• Numbness and tingling of the hands and feet
• Difficulty swallowing (sometimes accompanied by gagging, choking and vomiting)
• Vision problems (blurred or double vision)
• Slurred speech
Scoliosis, and in particular a thoracic curve, is quite common with Chiari I Malformations, though they seem to appear more often as right-sided thoracic curves. Syringomyelia is also often associated with Chiari I Malformations. This was well-described by Eule et al. (Spine, 2002) and Aitken et al. (Pediatr Neurol, 2009.) The PCP and authors did an excellent job recognizing the potential significance of an atypical thoracic curve in 12 year-old patient who ended up having a complicated problem.
Aitken LA et al. Chiari Type I Malformation in the Pediatric Population. Pediatr Neurol. 2009; 40(6):449-54.
Eule JM et al. Chiari I Malformation Associated with Syringomyelia and Scoliosis: A Twenty-Year Review of Surgical and Nonsurgical Treatment in a Pediatric Population. Spine. 2002; 27(13):1451-55.
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