A variant of the Klippel-Trenaunay-Weber syndrome with temporal lobe astrocytoma.

An 8-year-old boy with a variant of the Klippel-Trenaunay-Weber syndrome (KTW syndrome) is described. The hemangiomatous tissue located to the right half of his trunk and extremities was hypotrophic. On the same side, on his face and gingivae the tissue appeared hypertrophic and dental abnormalities were present. Moreover, the patient suffered from psychomotor epilepsy caused by a right-sided temporal astrocytoma. The connection between the KTW syndrome and the neurocutaneous syndromes is discussed.