Petrous Apex Cephaloceles: Radiology Features and Surgical Management of a Rare Entity.

OBJECTIVE: To characterize the radiologic findings of petrous apex cephalocele (PAC) in a patient cohort, and report the surgical management for three symptomatic PAC patients and cerebrospinal fluid (CSF) leak via the middle cranial fossa approach.

STUDY DESIGN: Retrospective case series.

SETTING: Academic center.

PATIENTS: Thirty-five patients with PAC were identified by review of the imaging archive between 2008 and 2019 (29 females; mean, 55 yrs; range, 4-86 yrs). All patients underwent magnetic resonance imaging of the skull base and/or computed tomography examination.

INTERVENTIONS: Surgical repair of PAC.

MAIN OUTCOME MEASURES: Radiologic features of PAC.

RESULTS: Radiological features of PAC: 25.7% bilateral; partial or expanded empty sella in 82.9%; arachnoid pits in 14.2%; and enlarged CSF space of optic nerve sheath in 20.0%. Coexisting pathology included temporal, sphenoid, and bilateral jugular foramen meningocele; as well as cribriform, middle crania fossa, and right temporal defect. Three case studies describing the surgical course of spontaneous CSF leak secondary to PAC were managed with the middle cranial fossa approach.

CONCLUSIONS: PAC is an exceedingly unusual cause for CSF leak in the adult and pediatric population. A middle fossa approach may be used to treat CSF leak as a result of PAC. Typically, CSF otorrhea originates from an encephalocele that extends via a bony defect in the tegmen tympani or tegmen mastoideum. However, on occasion the source of the CSF otorrhea is not via the tegmen, instead defects in the middle fossa floor, medial to the ridge for the gasserian ganglion (tubercle of Princeteau), need to be considered.