Epidural venous engorgement resulting in progressive cervical myelopathy from shunt-related intracranial hypotension: Case report and review of the literature

Journal Article

The authors report an unusual case of engorged epidural veins causing progressive cervical myelopathy after long-term cerebrospinal fluid (CSF) shunt therapy and intracranial hypotension. An 18-year-old woman, who had previously undergone shunt placement with a distal slit valve for a porencephalic cyst when 2 years of age, presented with progressive spastic quadriparesis, numbness, and gait difficulty. Postural headaches were absent and a lumbar puncture revealed low CSF pressure. Neuroimaging disclosed markedly engorged anterior epidural veins causing compression of the cervical spinal cord. The slit-valve shunt system was surgically removed and an external drain was placed. The patient's CSF pressure was gradually raised to clinically tolerable levels. Once the optimal pressure was identified, a programmable shunt was placed with the valve set at the same level. The patient's neurological status improved, and the epidural veins had returned to their normal size on follow-up imaging. The authors describe the unique treatment strategy used in this patient and review the literature on epidural venous engorgement as it relates to intracranial hypotension.

Duke Authors

Cited Authors

  • Liu, JK; Gottfried, ON; Brockmeyer, DL

Published Date

  • 2006

Published In

Volume / Issue

  • 105 PEDIATRICS / SUPPL. 6

Start / End Page

  • 499 - 503

International Standard Serial Number (ISSN)

  • 0022-3085