Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV

Wouter I. Schievink, Michael J. Link, David G. Piepgras, Robert F. Spetzler, Robert A. Solomon, Daniel L. Barrow, Christopher S. Ogilvy, H. Hunt Batjer, Issam A. Awad

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

OBJECTIVE: Ehlers-Danlos syndrome Type IV is a heritable connective tissue disorder with frequent neurovascular manifestations, such as intracranial aneurysms. Patients with this syndrome have notoriously fragile blood vessels, and the reported mortality rate for any type of vascular surgical procedure is 40%. This syndrome is rare, however, and the complication rate of aneurysm surgery may have been overestimated. METHODS: We reviewed our experience with aneurysm surgery in a group of patients with Ehlers-Danlos syndrome Type IV. RESULTS: The patient population consisted of three women and one man with a mean age of 44 years (age range, 20-57 yr). One patient, who had a ruptured anterior circulation aneurysm, died as a direct result of surgery because of marked vascular fragility. Three patients underwent successful surgery, consisting of a craniotomy and clipping of a ruptured anterior circulation aneurysm in two patients and a craniotomy and clip ligation of the parent artery in one patient with a ruptured dissecting vertebral artery aneurysm. Intraoperatively, mild vascular or connective tissue fragility was commonly observed. Postoperative complications (e.g., spontaneous pneumothorax and vertebral artery dissection) also were common but did not result in permanent morbidity. CONCLUSION: The risk of neurovascular surgery is high in patients with Ehlers-Danlos syndrome Type IV and intra- and postoperative complications are common. However, most patients tolerate the operation without permanent morbidity.

Original languageEnglish (US)
Pages (from-to)607-613
Number of pages7
JournalNeurosurgery
Volume51
Issue number3
DOIs
StatePublished - Sep 1 2002

Fingerprint

Ehlers-Danlos Syndrome
Intracranial Aneurysm
Aneurysm
Vertebral Artery Dissection
Blood Vessels
Craniotomy
Connective Tissue
Vascular Surgical Procedures
Morbidity
Intraoperative Complications
Pneumothorax
Surgical Instruments
Ligation
Arteries

Keywords

  • Cerebral aneurysm
  • Dissection
  • Ehlers-Danlos syndrome Type IV
  • Subarachnoid hemorrhage

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery

Cite this

Schievink, W. I., Link, M. J., Piepgras, D. G., Spetzler, R. F., Solomon, R. A., Barrow, D. L., ... Awad, I. A. (2002). Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV. Neurosurgery, 51(3), 607-613. https://doi.org/10.1097/00006123-200209000-00004

Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV. / Schievink, Wouter I.; Link, Michael J.; Piepgras, David G.; Spetzler, Robert F.; Solomon, Robert A.; Barrow, Daniel L.; Ogilvy, Christopher S.; Batjer, H. Hunt; Awad, Issam A.

In: Neurosurgery, Vol. 51, No. 3, 01.09.2002, p. 607-613.

Research output: Contribution to journalArticle

Schievink, WI, Link, MJ, Piepgras, DG, Spetzler, RF, Solomon, RA, Barrow, DL, Ogilvy, CS, Batjer, HH & Awad, IA 2002, 'Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV', Neurosurgery, vol. 51, no. 3, pp. 607-613. https://doi.org/10.1097/00006123-200209000-00004
Schievink WI, Link MJ, Piepgras DG, Spetzler RF, Solomon RA, Barrow DL et al. Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV. Neurosurgery. 2002 Sep 1;51(3):607-613. https://doi.org/10.1097/00006123-200209000-00004
Schievink, Wouter I. ; Link, Michael J. ; Piepgras, David G. ; Spetzler, Robert F. ; Solomon, Robert A. ; Barrow, Daniel L. ; Ogilvy, Christopher S. ; Batjer, H. Hunt ; Awad, Issam A. / Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV. In: Neurosurgery. 2002 ; Vol. 51, No. 3. pp. 607-613.
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