Background: Delayed Chiari malformation with syringomyelia is an uncommon and sparsely reported complication after lumbar cisternal shunting. A number of treatments have been implemented with varying degrees of success. After review of these modalities, a treatment plan was devised and implemented.

Case Description: A 15-year-old girl was diagnosed with idiopathic intracranial hypertension (pseudotumor cerebri) and had placement of a Medtronic Spetzler lumboperitoneal shunt. She did well for 10 years and then developed a 3-week history of headache, balance problems, and sensory loss. Workup demonstrated a new Chiari malformation and cervical syrinx. She underwent initial placement of a ventriculoperitoneal shunt, followed by ligation of the lumboperitoneal shunt. She then had a suboccipital decompression and duraplasty. Follow-up at 6 months showed relief of her symptoms and MRI demonstrated resolution of the Chiari malformation and syrinx.

Conclusion: Delayed Chiari malformation and/or syringomyelia after lumbar shunting is best treated with placement of a ventricular device and ligation of the lumbar shunt. The Chiari malformation and/or syrinx should be surgically addressed for persistent symptoms or progression of the structural abnormality.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3463835PMC
http://dx.doi.org/10.4103/2152-7806.100369DOI Listing

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