Klippel-Trenaunay-Weber syndrome and intramedullary cervical cavernoma: a very rare association. Case report.
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.
Pichierri
Angelo
A
Department of Neurological Sciences--Neurosurgery, University of Rome "La Sapienza," Rome, Italy. angelopichierri@fastwebnet.it
Piccirilli
Manolo
M
Passacantilli
Emiliano
E
Frati
Alessandro
A
Santoro
Antonio
A
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