title: Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report.
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.
  
  
  METHODS
  We describe a very rare case in which KTWS was associated with a cervical intramedullary cavernous angioma surgically treated.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurological Sciences--Neurosurgery, University of Rome "La Sapienza," Rome, Italy. angelopichierri@fastwebnet.it
authors.forename: Angelo
authors.initials: A
authors.lastname: Pichierri
authors.forename: Manolo
authors.initials: M
authors.lastname: Piccirilli
authors.forename: Emiliano
authors.initials: E
authors.lastname: Passacantilli
authors.forename: Alessandro
authors.initials: A
authors.lastname: Frati
authors.forename: Antonio
authors.initials: A
authors.lastname: Santoro
mesh_terms: D000328:Adult; D002574:Cervical Vertebrae; D005260:Female; D020786:Hemangioma, Cavernous, Central Nervous System / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy