外陰部に著変を呈したクリッペル・ウェーバー症候群の1例

Abstract

A case of Klippel-Weber's syndrome was reported. The patient was a 4-year-old girl who was admitted to our clinic because of enlarged phallus and tumors on the left thigh and lower leg. On physical examination, livid red enlarged phallus was found and there were tumors on the left side of the buttocks, left thigh and lower leg which had dark red pigmentation. All laboratory data were within normal limit and the result of sex chromatin test was positive, chromosomal karyotype proved 46'XX, female. A scout film of the pelvic region showed small roentgen flecks in according with tumor mass and the finding of arterioangiography suggested that there were arterio-venous fistula in the tumor mass on the phallus and the left lower extremity. The swollen phallus and surrounding tumorous tissues could be resected as a whole. Histological examination of the removed specimen revealed capillary hemangioma.