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Prevalence of temporal bone tegmen defects among patients with Marfan syndrome

Uri Chavkin1,2, Adi Brenner-Ullman3, Omer Jacob Ungar4

  • 1a Department of Otolaryngology, Head and Neck, and Maxillofacial Surgery , Tel-Aviv Sourasky Medical Center , Tel-Aviv , Israel.

Acta Oto-Laryngologica
|March 13, 2019
PubMed
Abstract

Insights

Temporal bone defects are common in Marfan syndrome (MFS), a connective tissue disorder. This study found a high prevalence of these defects, which may be a new, serious complication of MFS.

Area of Science:

  • Neurosurgery
  • Radiology
  • Genetics

Background:

  • Marfan syndrome (MFS) is a genetic connective tissue disorder.
  • Dural changes in MFS can lead to cerebrospinal fluid (CSF) leaks, but temporal bone involvement was previously undescribed.
  • This study investigates previously undocumented temporal bone defects in MFS patients.

Purpose of the Study:

  • To determine the prevalence of temporal bone meningocele or encephalocele in patients with Marfan syndrome.
  • To identify potential associations between MFS and skull base defects.

Main Methods:

  • Retrospective review of adult MFS patients treated between 1993 and 2018.
  • Analysis of head CT scans to identify anterior or lateral skull base defects.
  • Inclusion criteria focused on adult patients diagnosed with MFS.

Main Results:

  • 101 MFS patients identified; 12 with suitable CT scans were analyzed.
  • A high prevalence of temporal bone defects was observed (41.6% of analyzed patients).
  • Seven defects were found, all located in the temporal bone, with a median size of 3mm.

Conclusions:

  • Radiological evidence of temporal bone defects is highly prevalent in Marfan syndrome patients.
  • This finding represents a novel and potentially serious association with MFS.
  • Further research is warranted to understand the clinical implications of these defects.
Keywords:
CSF leakMarfan syndromemeningoceleotorrheategmen defecttemporal bone

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