Spontaneous Congenital Perilabyrinthine Cerebrospinal Fluid Fistulas

Abstract

Objectives: To report a recalcitrant spontaneous cerebrospinal fluid (CSF) fistula arising from multiple, anatomically-linked lateral skull base defects, and to review the available literature to determine optimal techniques for operative repair of congenital CSF fistulae. Methods: A patient with recurrent episodes of otologic meningitis was found to have a patent tympanomeningeal fissure, also known as a Hyrtl’s fissure, and internal auditory canal (IAC) diverticulum that communicated with the jugular bulb. A systematic review of the literature characterized all reports of spontaneous congenital perilabyrinthine CSF leaks, and all cases of Hyrtl’s fissures. Results: An 11-year-old female was referred for recurrent meningitis. Imaging demonstrated a fistulous connection between the middle ear and IAC diverticulum via the jugular foramen. Specifically, a Hyrtl’s fissure was identified, as well as demineralized bone around the jugular bulb. Obliteration of the fissure was initially performed, and a fistula reformed 4 months later. Multifocal CSF egress in the hypotympanum was identified on re-exploration, and middle ear obliteration with external auditory canal (EAC) overclosure was performed. A systematic review of the literature demonstrated 19 cases of spontaneous congenital perilabyrinthine CSF leaks. In total, 6 cases had multiple sources of CSF leak and 2 had history suggestive of intracranial hypertension. All of these noted cases demonstrated leak recurrence. Middle ear obliteration with EAC overclosure was successful in 4 recalcitrant cases. Conclusions: Repair of spontaneous congenital perilabyrinthine CSF leaks in cases demonstrating multiple sources of egress or signs of intracranial hypertension should be approached with caution. Middle ear obliteration with EAC overclosure may provide the most definitive management option for these patients, particularly if initial attempt at primary repair is unsuccessful.

Jugular Foramen Cerebrospinal Fluid Leak Repaired Via the Infralaybryinthine Approach With Adjunct Use of Intrathecal Fluorescein and Stereotactic Navigation

“…Spontaneous CSF leaks appear most commonly in the tegmen tympani or tegmen mastoideum (2). Only one other case has been described in the literature of a CSF leak associated with a defect at the jugular foramen (4). This occurred as a congenital leak associated with a Hyrtl's fissure.…”

Enterothecal fistula as a rare cause of adult pneumocephalus and meningitis: a case report

“…One such example is a fistulous connection between the subarachnoid space and paraspinal veins termed CSF venous fistula ( 1 , 2 ). Other fistulous connections, such as those occurring between the dura and the middle ear, can lead to pneumocephalus and intracranial abscesses ( 3 ).…”