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A Case of Zimmermann-Laband Syndrome with Supernumerary Teeth.

Authors :
Holzhausen, Marinella
Gonçalves, Daniela
Bello Corrêa, Fernanda De Oliveira
Spolidorio, Luis Carlos
Rodrigues, Valter Curi
Orrico, Silvana Regina Perez
Source :
Journal of Periodontology; Aug2003, Vol. 74 Issue 8, p1225-1230, 6p, 5 Color Photographs, 3 Black and White Photographs
Publication Year :
2003

Abstract

Background: Zimmermann-Laband syndrome b a rare autosomal dominant disorder that is characterized by gingival fibromatosis, ear, nose, bone, and nail defects, and hepatosplenomegaly. Methods: This case report describes the clinical presentation and periodontal findings in a 13-year-old female patient with previously undiagnosed Zimmermann-Laband syndrome. Results: Clinical and radiographic findings and genetic counseling confirmed the diagnosis of Zimmermann-Laband syndrome. The most striking oral findings mere the presence of gingival enlargement involving both the maxillary and mandibular arches, anterior open bite, non-erupted teeth, and two supernumerary teeth. Periodontal treatment consisted of gingivectomy in four quadrants. Histopathologic evaluation of excised tissue supported the diagnosis of gingival fibromatosis. The patient was referred for appropriate orthodontic treatment and genetic counseling, and has been closely followed for the earliest signs of hepatosplenomegaly. Conclusions: Dental practitioners should be alert for developmental abnormalities that may occur in patients with gingival fibromatosis as this may indicate the presence of a rare disorder tike Zimmermann-Laband syndrome. A comprehensive medical history and physical systemic evaluation are essential for correct diagnosis and treatment of these cases. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00223492
Volume :
74
Issue :
8
Database :
Complementary Index
Journal :
Journal of Periodontology
Publication Type :
Academic Journal
Accession number :
51007414
Full Text :
https://doi.org/10.1902/jop.2003.74.8.1225