Document Detail

Klippel-Trénaunay syndrome manifesting as gingival overgrowth and teeth agenesis.
MedLine Citation:
PMID:  20831140     Owner:  NLM     Status:  MEDLINE    
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. 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.
Hakan Ozdemir; Ismail Marakoglu; Melih Akyol; O Fahrettin Goze; Ulvi Kahraman Gursoy
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of clinical pediatric dentistry     Volume:  34     ISSN:  1053-4628     ISO Abbreviation:  J Clin Pediatr Dent     Publication Date:  2010  
Date Detail:
Created Date:  2010-09-13     Completed Date:  2010-11-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9100079     Medline TA:  J Clin Pediatr Dent     Country:  United States    
Other Details:
Languages:  eng     Pagination:  351-4     Citation Subset:  D    
Department of Periodontology, Cumhuriyet University, Sivas, Turkey.
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MeSH Terms
Anodontia / pathology*
Bicuspid / abnormalities
Capillaries / pathology
Epithelial Cells / pathology
Gingiva / blood supply
Gingival Overgrowth / pathology*
Klippel-Trenaunay-Weber Syndrome / pathology*
Lip / blood supply,  pathology
Plasma Cells / pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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