Chiari malformation with syringocephaly. Case report.

A 69-year-old white woman presented with a left hemiparesis which progressed to quadriparesis and encephalopathy. Computerized tomography and magnetic resonance imaging revealed a Chiari I malformation and a hydromyelic cavity extending from C-2 to T-6. Rostrally, the cavity extended through the ventral medulla, pons, and right cerebral peduncle into the right cerebral hemisphere, where the cavity enlarged and was associated with mass effect. The patient has made a dramatic neurological recovery following suboccipital craniectomy with insertion of a dural graft to decompress the Chiari malformation and upper cervical laminectomy and dorsal root entry zone myelotomy to decompress the hydromyelia.