BACKGROUND: Bardet-Biedl syndrome (BBS) is a rare, heterogeneous, autosomal recessive condition, primarily characterized by polydactyly, obesity, mental retardation, hypogonadism, retinopathy, and renal failure. Dental anomalies, regarded as secondary manifestations, include hypodontia, microdontia, short roots, and deep palate. Few reports in the literature have described the oral manifestations of BBS. This article reports a case of BBS in a boy who presented some typical oral manifestations added to a generalized gingival overgrowth, an anomaly that had not been reported previously in patients with this syndrome. METHODS: A 12-year-old white male presented with a diagnosis of BBS and chief complaint of gingival enlargement in the anterior segment of both arcades. The treatment plan included surgical removal of the overgrown gingiva followed by orthodontic therapy. The excised tissues were submitted to histologic analysis. RESULTS: There was no sign of recurrence 1 year after gingivectomy. Histopathology revealed a dense connective tissue with a mild inflammatory infiltrate, irregularly arranged fiber bundles, and epithelial acanthosis, which is characteristic of gingival overgrowth. CONCLUSIONS: The gingival overgrowth was treated successfully by gingivectomy. The periodontal surgery minimized the functional, social, and emotional consequences of the oral manifestation associated with the syndrome.
BACKGROUND:Bardet-Biedl syndrome (BBS) is a rare, heterogeneous, autosomal recessive condition, primarily characterized by polydactyly, obesity, mental retardation, hypogonadism, retinopathy, and renal failure. Dental anomalies, regarded as secondary manifestations, include hypodontia, microdontia, short roots, and deep palate. Few reports in the literature have described the oral manifestations of BBS. This article reports a case of BBS in a boy who presented some typical oral manifestations added to a generalized gingival overgrowth, an anomaly that had not been reported previously in patients with this syndrome. METHODS: A 12-year-old white male presented with a diagnosis of BBS and chief complaint of gingival enlargement in the anterior segment of both arcades. The treatment plan included surgical removal of the overgrown gingiva followed by orthodontic therapy. The excised tissues were submitted to histologic analysis. RESULTS: There was no sign of recurrence 1 year after gingivectomy. Histopathology revealed a dense connective tissue with a mild inflammatory infiltrate, irregularly arranged fiber bundles, and epithelial acanthosis, which is characteristic of gingival overgrowth. CONCLUSIONS: The gingival overgrowth was treated successfully by gingivectomy. The periodontal surgery minimized the functional, social, and emotional consequences of the oral manifestation associated with the syndrome.
Authors: Katarzyna Gawron; Katarzyna Łazarz-Bartyzel; Jan Potempa; Maria Chomyszyn-Gajewska Journal: Orphanet J Rare Dis Date: 2016-01-27 Impact factor: 4.123