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

The authors report an unusual case of engorged epidural veins causing progressive cervical myelopathy after longterm 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.