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Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report.

Publication ,  Journal Article
Alexander, MJ; Grossi, PM; Spetzler, RF; McDougall, CG
Published in: Neurosurgery
November 2002

OBJECTIVE AND IMPORTANCE: Spinal cord involvement in Klippel-Trenaunay-Weber (KTW) syndrome is rare. Cases of intradural spinal cord arteriovenous malformations (AVMs) have been associated with this syndrome. Likewise, cases of epidural hemangioma and angiomyolipoma have been reported to occur at the same segmental level as cutaneous hemangioma in KTW syndrome. This report details a rare case of an extradural thoracic AVM in a patient with KTW syndrome. CLINICAL PRESENTATION: A 30-year-old man presented with a 10-month history of progressive myelopathy, bilateral lower-extremity weakness, and numbness, with the right side affected more than the left. His symptoms had progressed to the point that he was unable to walk. The patient had the characteristic manifestations of KTW syndrome, including numerous cutaneous angiomas and cavernomas, limb hypertrophy and syndactyly, and limb venous malformations. A magnetic resonance imaging scan and subsequent angiogram demonstrated a large extradural AVM causing cord compression at the T3-T4 levels. INTERVENTION: The patient underwent two separate endovascular procedures, including embolization of upper thoracic and thyrocervical trunk feeders. Subsequently, he underwent T1-T4 laminectomy and microsurgical excision of the AVM. Clinically, the patient improved such that he could walk without assistance. CONCLUSION: KTW syndrome represents a spectrum of clinical presentations. Although involvement of the spinal cord is uncommon, the manifestations of this syndrome may include both intradural and extradural AVMs in addition to various tumors.

Duke Scholars

Published In

Neurosurgery

DOI

ISSN

0148-396X

Publication Date

November 2002

Volume

51

Issue

5

Start / End Page

1275 / 1278

Location

United States

Related Subject Headings

  • Thorax
  • Spinal Cord Compression
  • Neurology & Neurosurgery
  • Male
  • Laminectomy
  • Klippel-Trenaunay-Weber Syndrome
  • Humans
  • Embolization, Therapeutic
  • Arteriovenous Malformations
  • Adult
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Alexander, M. J., Grossi, P. M., Spetzler, R. F., & McDougall, C. G. (2002). Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report. Neurosurgery, 51(5), 1275–1278. https://doi.org/10.1097/00006123-200211000-00025
Alexander, Michael J., Peter M. Grossi, Robert F. Spetzler, and Cameron G. McDougall. “Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report.Neurosurgery 51, no. 5 (November 2002): 1275–78. https://doi.org/10.1097/00006123-200211000-00025.
Alexander MJ, Grossi PM, Spetzler RF, McDougall CG. Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report. Neurosurgery. 2002 Nov;51(5):1275–8.
Alexander, Michael J., et al. “Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report.Neurosurgery, vol. 51, no. 5, Nov. 2002, pp. 1275–78. Pubmed, doi:10.1097/00006123-200211000-00025.
Alexander MJ, Grossi PM, Spetzler RF, McDougall CG. Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report. Neurosurgery. 2002 Nov;51(5):1275–1278.
Journal cover image

Published In

Neurosurgery

DOI

ISSN

0148-396X

Publication Date

November 2002

Volume

51

Issue

5

Start / End Page

1275 / 1278

Location

United States

Related Subject Headings

  • Thorax
  • Spinal Cord Compression
  • Neurology & Neurosurgery
  • Male
  • Laminectomy
  • Klippel-Trenaunay-Weber Syndrome
  • Humans
  • Embolization, Therapeutic
  • Arteriovenous Malformations
  • Adult