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Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report.

Publication ,  Journal Article
Hughes, BD; Powers, CJ; Zomorodi, AR
Published in: Neurosurgery
September 2011

BACKGROUND AND IMPORTANCE: his is the first case report of clipping a cerebral aneurysm in a patient with Loeys-Dietz syndrome (LDS). LDS is a newly described autosomal dominant connective tissue disease with systemic vascular involvement. Unique to this syndrome is the development of aneurysms at a young age with the propensity of dissection or rupture at a stage that is earlier than when surgical intervention is typically indicated. We describe the nuances in intraoperative and postoperative management. CLINICAL PRESENTATION: A 31-year-old woman who recently received a diagnosis of with LDS type II presented to neurosurgical attention for management of an unruptured right ophthalmic artery aneurysm. The patient underwent a right pterional craniotomy for clipping of the aneurysm, with lumbar drain placement before the procedure. Papaverine had to be used several times to counteract vasospasm of the vessels during arachnoid dissection. Because of vascular reactivity, temporary clipping was not used, and the aneurysm was clipped successfully. CONCLUSION: LDS is a newly described disorder that warrants awareness in the neurosurgical community because of its association with intracerebral aneurysms as well as craniosynostosis (19%), scoliosis (20%), cervical spine instability (7%), hydrocephalus, and Arnold-Chiari malformation. When clipping aneurysms in these patients, the surgeon should be aware of the potential for severe vascular reactivity during dissection and avoid temporary clipping when possible. Avoidance of lumbar drainage intraoperatively reduces the risk of intracranial hypotension after removal.

Duke Scholars

Published In

Neurosurgery

DOI

EISSN

1524-4040

Publication Date

September 2011

Volume

69

Issue

3

Start / End Page

E746 / E755

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Surgical Instruments
  • Recovery of Function
  • Ophthalmic Artery
  • Neurosurgical Procedures
  • Neurology & Neurosurgery
  • Magnetic Resonance Imaging
  • Loeys-Dietz Syndrome
  • Intracranial Aneurysm
  • Image Processing, Computer-Assisted
 

Citation

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MLA
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Hughes, B. D., Powers, C. J., & Zomorodi, A. R. (2011). Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report. Neurosurgery, 69(3), E746–E755. https://doi.org/10.1227/NEU.0b013e31821964a3
Hughes, Betsy D., Ciaran J. Powers, and Ali R. Zomorodi. “Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report.Neurosurgery 69, no. 3 (September 2011): E746–55. https://doi.org/10.1227/NEU.0b013e31821964a3.
Hughes BD, Powers CJ, Zomorodi AR. Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report. Neurosurgery. 2011 Sep;69(3):E746–55.
Hughes, Betsy D., et al. “Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report.Neurosurgery, vol. 69, no. 3, Sept. 2011, pp. E746–55. Pubmed, doi:10.1227/NEU.0b013e31821964a3.
Hughes BD, Powers CJ, Zomorodi AR. Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report. Neurosurgery. 2011 Sep;69(3):E746–E755.
Journal cover image

Published In

Neurosurgery

DOI

EISSN

1524-4040

Publication Date

September 2011

Volume

69

Issue

3

Start / End Page

E746 / E755

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Surgical Instruments
  • Recovery of Function
  • Ophthalmic Artery
  • Neurosurgical Procedures
  • Neurology & Neurosurgery
  • Magnetic Resonance Imaging
  • Loeys-Dietz Syndrome
  • Intracranial Aneurysm
  • Image Processing, Computer-Assisted