Association of intracranial aneurysm and Loeys-Dietz syndrome: Case illustration, management, and literature review

Rudy J. Rahme, Joseph G. Adel, Bernard R. Bendok, John F. Bebawy, Dhanesh K. Gupta, H. Hunt Batjer

Research output: Contribution to journalArticle

22 Scopus citations


BACKGROUND AND IMPORTANCE: Loeys-Dietz syndrome (LDS) is a newly described connective tissue disease associated with aortic aneurysms. A strong association between LDS and intracranial aneurysms has not yet been documented in the literature. We present the first detailed report of an intracranial aneurysm finding in an LDS patient. CLINICAL PRESENTATION: The patient is a 20-year-old female recently diagnosed with LDS and found to harbor 2 incidental intracranial aneurysms on a screening magnetic resonance angiography: a 3-mm right carotid ophthalmic aneurysm and an 8-mm partially fusiform paraclinoid carotid artery aneurysm. A standard left pterional craniotomy was performed. Intraoperative adenosine was used instead of temporary clipping because her vessels were extremely friable. After reconstruction, an intraoperative indocyanine green angiogram was obtained, confirming complete aneurysmal obliteration and internal carotid artery patency. CONCLUSION: This is the first detailed report of a clear association between intracranial aneurysms and LDS. An association between LDS and intracranial aneurysms, if substantiated in a larger study, has implications for aneurysm screening in this population. Such an association may shed light on mechanisms of aneurysm formation, growth, and rupture.

Original languageEnglish (US)
Issue number2
Publication statusPublished - Aug 2011



  • Adenosine
  • Connective tissue disease
  • Intracranial aneurysm
  • Loeys-Dietz Syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery

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