Klippel-Trénaunay syndrome manifesting as gingival overgrowth and teeth agenesis.
OBJECTIVE
Klippel-Trénaunay syndrome (KTS) is characterized by triad of venous varicosity, naevus flammeus, and soft/hard tissue hypertrophy. Manifestations of the syndrome in the head and neck region are rare, but in some cases hemangioma of the lips, tongue, and gums, open bite and cross bite, and early tooth eruption are associated with the disease.


METHODS
We report a 12-year-old KTS patient with gingival hyperplasia, congenital missing teeth, and increased mucosal vascularization as oral manifestations.


CONCLUSIONS
All manifestations of the KTS were observed on the same side of the head, except bilateral missing teeth. Histological examination showed several vascular enlargements in enlarged gingiva. It is suggested that oral manifestations of the syndrome are generally related to the severity of the disease, but they do not always present in the same pattern.
Ozdemir
Hakan
H
Department of Periodontology, Cumhuriyet University, Sivas, Turkey. hozdemir@cumhuriyet.edu.tr
Marakoglu
Ismail
I
Akyol
Melih
M
Goze
O Fahrettin
OF
Gursoy
Ulvi Kahraman
UK
D000848:Anodontia / Q000473:pathology*; D001641:Bicuspid / Q000002:abnormalities; D002196:Capillaries / Q000473:pathology; D002648:Child; D004847:Epithelial Cells / Q000473:pathology; D005881:Gingiva / Q000098:blood supply; D019214:Gingival Overgrowth / Q000473:pathology*; D006801:Humans; D006984:Hypertrophy; D007715:Klippel-Trenaunay-Weber Syndrome / Q000473:pathology*; D008046:Lip / Q000098:blood supply / Q000473:pathology; D008297:Male; D010950:Plasma Cells / Q000473:pathology