Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas

Area of Science:

• Neurosurgery
• Otolaryngology
• Skull Base Surgery

Background:

• Spontaneous congenital cerebrospinal fluid (CSF) fistulae present diagnostic and management challenges.
• Recurrent meningitis can indicate underlying CSF leaks, often linked to lateral skull base anomalies.

Observation:

• A case of recurrent meningitis in an 11-year-old female revealed a patent Hyrtl's fissure and internal auditory canal (IAC) diverticulum.
• The fistula connected the middle ear to the jugular bulb, with evidence of demineralized bone.

Findings:

• Initial repair of the Hyrtl's fissure failed, with fistula recurrence noted.
• Re-exploration identified multifocal CSF egress, leading to middle ear obliteration and external auditory canal (EAC) overclosure.
• Literature review showed 19 cases of spontaneous congenital perilabyrinthine CSF leaks, with recurrence common in complex cases.

Implications:

• Complex congenital CSF leaks, especially with multiple egress points or intracranial hypertension, demand cautious surgical planning.
• Middle ear obliteration with EAC overclosure appears to be a successful strategy for recalcitrant congenital CSF fistulae.