A1 Refereed original research article in a scientific journal

Klippel-Trenaunay Syndrome Manifesting as Gingival Overgrowth and Teeth Agenesis




AuthorsOzdemir H, Marakoglu I, Akyol M, Goze OF, Gursoy UK

PublisherJOURNAL PEDODONTICS INC

Publication year2010

JournalJournal of Clinical Pediatric Dentistry

Journal name in sourceJOURNAL OF CLINICAL PEDIATRIC DENTISTRY

Journal acronymJ CLIN PEDIATR DENT

Volume34

Issue4

First page 351

Last page354

Number of pages4

ISSN1053-4628

DOIhttps://doi.org/10.17796/jcpd.34.4.l41414372l4332m1


Abstract
Objective: Klippel-Trenaunay 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. Study design: We report a 12-year-old KTS patient with gingival hyperplasia, congenital missing teeth, and increased mucosal vascularization as oral manifestations. Results and conclusion: 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.



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