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Giant syringobulbia associated with cerebellopontine angle arachnoid cyst and hydrocephalus

Ai Muroi1, Nigel Peter Syms, Shizuo Oi

  • 1Division of Pediatric Neurosurgery, Jikei Women's and Children's Medical Center, The Jikei School of Medicine, Tokyo, Japan. muroiai@aol.com

Journal of Neurosurgery. Pediatrics
|July 5, 2011
PubMed

Insights

This case study highlights successful surgical intervention for a rare infant condition involving a giant syringobulbia and cerebellopontine angle arachnoid cyst, resolving noncommunicating hydrocephalus and ensuring normal development.

Area of Science:

  • Pediatric Neurosurgery
  • Neuroscience
  • Medical Imaging

Background:

  • Giant syringobulbia and cerebellopontine angle (CPA) arachnoid cysts are rare congenital conditions.
  • Noncommunicating hydrocephalus in infants can result from brainstem or posterior fossa abnormalities.

Observation:

  • A 7-month-old infant presented with delayed milestones and rapidly increasing head circumference.
  • Brain imaging revealed a large brainstem syrinx and a right CPA arachnoid cyst obstructing CSF flow.
  • The cyst and syrinx were confirmed to be in communication.

Findings:

  • Surgical cerebrospinal fluid (CSF) diversion from the syringobulbia and lateral ventricle successfully treated the hydrocephalus.
  • Postoperative imaging showed reduced syrinx and ventricular size, with restored CSF pathways.
  • The infant achieved normal developmental milestones by age 3.

Implications:

  • Early surgical intervention is crucial for managing giant syringobulbia with associated CPA cysts.
  • Tailoring surgical approaches based on specific pathophysiology is essential for optimal outcomes.
  • This case demonstrates the efficacy of CSF diversion in resolving complex pediatric hydrocephalus.

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