Cerebral syrinx with Chiari I malformation

Area of Science:

• Neurology
• Pediatric Neurology
• Neurosurgery

Background:

• Hydrosyringomyelia (HSM) is a rare condition characterized by fluid-filled cavities within the spinal cord.
• Syringobulbia, the extension of a syrinx into the brainstem, is even rarer and often associated with Chiari I malformation.
• Altered cerebrospinal fluid (CSF) dynamics are implicated in the pathogenesis of HSM and related conditions.

Observation:

• A 13-year-old female presented with acute left gaze paralysis, indicating brainstem or cranial nerve involvement.
• Magnetic Resonance Imaging (MRI) revealed extensive hydrosyringomyelia (HSM) with syringobulbia extending from the pons into the centrum semiovale.
• Surgical exploration identified an imperforate foramen of Magendie, a key outlet for CSF from the fourth ventricle.

Findings:

• Surgical decompression via suboccipital craniotomy and addressing the imperforate foramen of Magendie led to significant clinical improvement.
• Postoperative MRI confirmed the resolution of syringobulbia and the cerebral syrinx, with diminished HSM.
• The patient's ocular movements normalized within two months, demonstrating the reversibility of neurological deficits.

Implications:

• This case underscores the critical role of adequate cerebrospinal fluid (CSF) flow in preventing complex syringomyelia and syringobulbia.
• It highlights the potential for surgical intervention to correct congenital CSF flow abnormalities, even in severe presentations.
• The findings contribute to understanding the spectrum of Chiari I malformation and its impact on pediatric neurological health.