Syringocephaly

Abstract

Syringomyelia is associated with Arnold-Chiari Type I malformations. Syringobulbia describes the phenomenon of syrinx extension into the brain stem. Syringocephaly is the further dissection of the fluid-filled cavity into the cerebral peduncles and cerebrum. In this case report, we describe a patient who presented with bulbar, sensory, motor, and coordination deficits both ipsilateral and contralateral to the lesion. This is most likely attributable to the wandering course the syrinx takes as it dissects through the spinal cord and into the internal capsule. This ill-defined syrinx disrupts various nuclei and fasciculi, both pre- and post-decussation, thus explaining the multiple deficits on each side. We initially treated this patient with a suboccipital craniectomy, C1 laminectomy, and duraplasty, which mildly improved his deficits. During follow-up, the patient was then found to have an exacerbation of his symptoms, at which time we performed a VP shunt revision (the patient had a history of hydrocephalus treated by a functioning VP shunt). Approximately 2 weeks after revision of the VP shunt, the patient had worsening of his symptoms, which we treated with a syringopleural shunt. This proved to be the most effective treatment with the greatest clinical improvement. Several months later, however, the patient died secondary to pulmonary disease exacerbated by VP shunt infection. In this paper, we also review the literature regarding the formation and treatment of syringocephaly, a rare and poorly understood entity.