Prevalence of temporal bone tegmen defects among patients with Marfan syndrome

Background: Marfan syndrome (MFS) is a genetic disorder affecting connective tissue. The composition of the dura can change. Consequently, lumbo-sacral dural herniations and cerebrospinal fluid (CSF) leaks are encountered, however, they have yet to been described in the temporal bone. Aims/Objectives: To define the prevalence of temporal bone meningocele or encephalocele among patients with MFS. Materials and methods: Reviewed medical records of all adult patients, diagnosed with MFS, who were treated between 1993 and 2018 at a single academic referral institute. Head targeted CT scans were analyzed. The presence of an anterior or lateral skull base defect was recorded. Results: One-hundred and one patients diagnosed with MFS were identified. Twelve of which had suitable CT scans and were enrolled in the study. The median age of patients with defects was 65 years (range 41–71). Five of the twelve patients (41.6%) had tegmen defect. Of the seven defects found, the median size of the defects was 3 mm (range 2–5 mm). All defects were in the temporal bone, none in the anterior skull base. Conclusions and significance: The prevalence of radiological evidence of a temporal bone defect among patients with MFS is high. This is a new, important, and potentially life-threatening association with the syndrome.