Holocord syrinx associated with shunt malfunction in tetraventricular hydrocephalus.

We present an uncommon case of holocord, "rosary bead-like," multiloculated syringomyelia consistent with a fourth ventricle outlet obstruction in a 2-month-old infant who was previously shunted for posthemorrhagic hydrocephalus of prematurity. The shunt malfunction resulted in a syringomyelia because of the simultaneous obstruction of the 3 outlets of the fourth ventricle, resulting in an enlargement of the central canal of the spinal cord. The syringomyelia resolved with shunt revision, avoiding further complex surgical procedures.