Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report

Angelo Pichierri, Manolo Piccirilli, Emiliano Passacantilli, Alessandro Frati, Antonio Santoro

Research output: Contribution to journalArticle

Abstract

Background: Klippel-Trenaunay-Weber syndrome is a rare mesodermal phakomatosis characterized by cutaneous hemangiomata, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. As the pathologic aspect of KTWS arises from the site in which malformations occur, the clinical picture varies widely from patients who complain for cosmetic reasons to patients with life-threatening lesions. Case Description: We describe a very rare case in which KTWS was associated with a cervical intramedullary cavernous angioma surgically treated. Conclusion: This report confirms the wide range of expression of vascular abnormalities in neurocutaneous developmental diseases and the need of a careful multisystemic evaluation of these patients.

Original languageEnglish
Pages (from-to)203-206
Number of pages4
JournalSurgical Neurology
Volume66
Issue number2
DOIs
Publication statusPublished - Aug 2006

Keywords

  • Intramedullary cavernous angioma
  • Klippel-Trenaunay-Weber
  • Neurocutaneous
  • Neurosurgery
  • Phakomatosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery

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