title: Association of intracranial aneurysm and Loeys-Dietz syndrome: case illustration, management, and literature review.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA.
authors.forename: Rudy J
authors.initials: RJ
authors.lastname: Rahme
authors.forename: Joseph G
authors.initials: JG
authors.lastname: Adel
authors.forename: Bernard R
authors.initials: BR
authors.lastname: Bendok
authors.forename: John F
authors.initials: JF
authors.lastname: Bebawy
authors.forename: Dhanesh K
authors.initials: DK
authors.lastname: Gupta
authors.forename: H Hunt
authors.initials: HH
authors.lastname: Batjer
mesh_terms: D002340:Carotid Artery Diseases / Q000150:complications / Q000473:pathology / Q000601:surgery; D003399:Craniotomy; D005260:Female; D006801:Humans; D002532:Intracranial Aneurysm / Q000150:complications* / Q000473:pathology / Q000601:surgery; D055947:Loeys-Dietz Syndrome / Q000150:complications* / Q000473:pathology / Q000601:surgery; D018810:Magnetic Resonance Angiography; D055815:Young Adult