Microsurgery of Arnold-Chiari malformation in adults with and without hydromyelia.

Microsurgical exploration of 15 adults with Arnold-Chiari malformation with and without hydromyelia using 3 to 20 X magnification has led to the following conclusions. Hydromyelia, associated with Arnold-Chiari malformation, is a progressive mechanical disorder that causes spinal cord deficits by pressure distention of the cord. Arnold-Chiari malformation causes slowly or suddenly progressive bulbar dysfunction by impaction of the malformation in the foramen magnum. Decompression of both can be achieved by a suboccipital carniectomy, upper cervical laminectomy, establishing an outlet from the fourth ventricle, and opening the distended cord in the thinnest exposed area, which is usually along the dorsal root entry zone. If Pantopaque myelography in patients in the supine position shows the Arnold-Chiari malformation, hydromyelia can be established as a cause of central cord deficit even if myelography shows the cord size to be normal. Syringomyelia, traditionally considered a degenerative disease, is a less common cause of a slowly progressive central cord deficit than either hydromyelia or intramedullary tumor.