Spinal arteriovenous malformation associated with Schimmelpenning syndrome

The authors report the presentation and management of a 13-year-old girl with Schimmelpenning syndrome, a rare neurocutaneous syndrome; this patient suffered hemorrhage of a spinal arteriovenous malformation. This is the first case of a spinal arteriovenous malformation reported in association with Schimmelpenning syndrome. Neurosurgeons should be aware of this rare phacomatosis as well as of the various neurological disorders associated with this diagnosis. The threshold for imaging the neuraxis in these patients should be low.