A Case of Zimmermann‐Laband Syndrome with Supernumerary Teeth
- 1 August 2003
- journal article
- case report
- Published by Wiley in The Journal of Periodontology
- Vol. 74 (8), 1225-1230
- https://doi.org/10.1902/jop.2003.74.8.1225
Abstract
Zimmermann-Laband syndrome is a rare autosomal dominant disorder that is characterized by gingival fibromatosis, ear, nose, bone, and nail defects, and hepatosplenomegaly. This case report describes the clinical presentation and periodontal findings in a 13-year-old female patient with previously undiagnosed Zimmermann-Laband syndrome. Clinical and radiographic findings and genetic counseling confirmed the diagnosis of Zimmermann-Laband syndrome. The most striking oral findings were 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. 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 like Zimmermann-Laband syndrome. A comprehensive medical history and physical systemic evaluation are essential for correct diagnosis and treatment of these cases.Keywords
This publication has 15 references indexed in Scilit:
- A Mutation in the SOS1 Gene Causes Hereditary Gingival Fibromatosis Type 1American Journal of Human Genetics, 2002
- Laband syndrome: Report of two cases, review of the literature, and identification of additional manifestationsOral Surgery, Oral Medicine, Oral Pathology, 1994
- Hereditary gingival fibromatosis in a family with the Zimmermann Laband syndromeJournal of Oral Pathology & Medicine, 1991
- Laband syndrome: a case reportJournal of Oral Pathology & Medicine, 1990
- A new case of Zimmermann‐Laband syndrome with mild mental retardation, asymmetry of limbs, and hypertrichosisAmerican Journal of Medical Genetics, 1988
- “New syndromes,” part II: “European” syndromesAmerican Journal of Medical Genetics, 1988
- Zimmerman‐Laband syndrome and profound mental retardationAmerican Journal of Medical Genetics, 1986
- GROWTH RETARDATION, DYSMORPHIC FACIES AND MINOR MALFORMATIONS FOLLOWING MASSIVE EXPOSURE TO PHENOBARBITONE IN UTEROActa Paediatrica, 1976
- Congenital idiopathic gingival fibromatosis in the deciduous dentitionOral Surgery, Oral Medicine, Oral Pathology, 1967
- Hereditary gingival fibromatosis: Report of an affected family with associated splenomegaly and skeletal and soft-tissue abnormalitiesOral Surgery, Oral Medicine, Oral Pathology, 1964