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Original Research

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Klippel-Trénaunay Syndrome Manifesting as Gingival Overgrowth and Teeth Agenesis

  • Hakan Ozdemir1,*,
  • Ismail Marakoglu2
  • Melih Akyol3
  • O Fahrettin Goze4
  • Ulvi Kahraman Gursoy5

1Department of Periodontology, Cumhuriyet University, Sivas, Turkey

2Department of Periodontology, Selcuk University, Konya, Turkey

3Department of Dermatology, Cumhuriyet University, Sivas, Turkey

4Department of Pathology Cumhuriyet University, Sivas, Turkey

5Institute of Dentistry, Helsinki University, Helsinki, Finland

DOI: 10.17796/jcpd.34.4.l41414372l4332m1 Vol.34,Issue 4,July 2010 pp.351-354

Published: 01 July 2010

*Corresponding Author(s): Hakan Ozdemir E-mail: hozdemir@cumhuriyet.edu.tr

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Abstract

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.

Keywords

Klippel-Trenaunay syndrome, congenital missing teeth, gingival overgrowth

Cite and Share

Hakan Ozdemir,Ismail Marakoglu,Melih Akyol,O Fahrettin Goze,Ulvi Kahraman Gursoy. Klippel-Trénaunay Syndrome Manifesting as Gingival Overgrowth and Teeth Agenesis. Journal of Clinical Pediatric Dentistry. 2010. 34(4);351-354.

URL:

https://www.jocpd.com/articles/10.17796/jcpd.34.4.l41414372l4332m1

References

1. Auluck A, Suhas S, Pai KM. Klippel-Trenaunay syndrome. Oral Dis, 11: 255–258, 2005.

2. Steiner M, Gould AR, Graves SM, Kuerschner TW. Klippel-Trenaunay-Weber syndrome. Oral Surg Oral Med Oral Pathol, 63: 208–215, 1987.

3. Bathi RJ, Agarwal N, Burde KN. Klippel-Trenaunay syndrome (angio osteohypertrophy syndrome): A report of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 93: 276–80, 2002.

4. Wiedemann HR, Kunze J. Klippel-Trenaunay syndrome. In: Atlas der Klinishen Syndrome, 4th ed. New York: Schattauer, 1995.

5. Kuhl SRJM, Schulze RKW, Kreft A, d’Hoedt B. Epulis granulomatosa as an oral manifestation of Klippel-Trenaunay syndrome. J Oral Pathol Med, 35: 576–578, 2006.

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