Invasive cerebrospinal fluid cysts and cephaloceles of the petrous apex

OBJECTIVE: To describe the presentation, diagnostic evaluation, and surgical management of petrous apex cerebrospinal fluid (CSF) cysts and cephaloceles. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Six patients with symptomatic CSF cysts or cephaloceles. INTERVENTION(S): All patients underwent operative intervention. MAIN OUTCOME MEASURE(S): Presentation, imaging characteristics, operative findings, surgical approach, resolution of symptoms, and complications. RESULTS: Six patients presented with various neurotologic symptoms including vertigo, otalgia, diplopia, meningitis, hearing loss, and retroorbital headaches. Four lesions were centered within the anterior petrous apex and were classified as a cephalocele originating from Meckel's cave. The remaining two lesions were arachnoid cysts that involved the posterior petrous apex. Cysts and cephaloceles both demonstrated bone erosion on computed tomography and were hyperintense on T2-weighted magnetic resonance imaging and isointense or hypointense on T1-weighted magnetic resonance imaging. A variety of surgical approaches was used to treat these lesions. Preoperative symptoms were improved in five of six cases. One patient developed a postoperative CSF leak that resolved with conservative measures. CONCLUSION: Petrous apex CSF cysts and cephaloceles may present with a variety of neurotologic symptoms. Imaging often helps narrow the differential diagnosis, but these lesions can still be confused with other erosive skull base lesions such as cholesterol granulomas, epidermoids, or tumors. Optimal treatment of symptomatic posterior petrous apex CSF cysts is marsupialization via a posterior fossa approach (i.e., retrosigmoid or retrolabyrinthine). A middle fossa approach with obliteration of the anterior petrous apex may be used to treat symptomatic CSF cephaloceles arising from Meckel's cave.