Cervical myelopathy due to chronic overshunting in a pediatric patient: case report and review of the literature

Abstract

We present a rare cause of cervical myelopathy produced by an engorged suboccipital epidural venous plexus due to chronic cerebrospinal fluid (CSF) overdrainage. A 17-year-old boy with obstructive hydrocephalus due to a retrocerebellar cyst and secondary implantation of a ventricloperitoneal shunt (VP-shunt) presented with progressive spastic tetraparesis. MRI imaging revealed myelopathy due to significant compression of the cervical spinal cord by engorged epidural veins. Further assessment at a low-pressure setting revealed a broken shunt valve. The VP-shunt valve was changed with an additional anti-siphon device leading to a gradual increase of the intracranial pressure (ICP). After intensive physiotherapy, the patient showed slight clinical improvement. Follow-up imaging within nine days showed distinct regression of the dilated venous plexus at the cranial-cervical junction (CCJ) with the resolution of cord compression. Engorgement of the epidural venous plexus should always be considered in the differential diagnosis of myelopathy in long-term shunt patients even when classical clinical and radiological signs of overshunting are missing.