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Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome.
Oral Dis. 2008 Nov; 14(8):747-53.OD

Abstract

OBJECTIVE

The G/BBB syndrome is an X-linked recessive disorder characterized by eye anomalies, laryngotracheoesophageal cleft, congenital heart disease, genitourinary anomalies and gastrointestinal disorders. Patients may also present cleft lip and palate, high-arched palate and thin upper lip. This study aimed to investigate the occurrence of tooth abnormalities and soft tissue changes in patients with G/BBB syndrome.

DESIGN

Cross-sectional.

SUBJECTS AND METHODS

Twenty-one patients with G/BBB syndrome were analyzed as to the presence of tooth abnormalities and soft tissue alterations.

MAIN OUTCOME MEASURES

The prevalence of tooth agenesis and supernumerary teeth was compared to patients without morphofunctional alterations, matched for gender and age.

RESULTS

All patients had complete cleft lip and palate; 95.23% of patients presented tooth abnormalities, mainly hypoplastic alterations, with predominance of alterations of number, followed by alterations of structure, shape and position. The frequency of tooth agenesis and supernumerary teeth was significantly higher compared with the control group; 11 patients presented incisiform supernumerary teeth in the mandibular anterior region. Ankyloglossia was observed in 11 of 21 patients.

CONCLUSION

The presence of mandibular anterior supernumerary teeth and ankyloglossia should be investigated in the clinical evaluation of patients with suspected diagnosis of the G/BBB syndrome.

Authors+Show Affiliations

Paediatric and Community Dentistry Sector, Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru. gsdalben@usp.brNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

18627501

Citation

da Silva Dalben, G, et al. "Tooth Abnormalities and Soft Tissue Alterations in Patients With G/BBB Syndrome." Oral Diseases, vol. 14, no. 8, 2008, pp. 747-53.
da Silva Dalben G, Richieri-Costa A, de Assis Taveira LA. Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome. Oral Dis. 2008;14(8):747-53.
da Silva Dalben, G., Richieri-Costa, A., & de Assis Taveira, L. A. (2008). Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome. Oral Diseases, 14(8), 747-53. https://doi.org/10.1111/j.1601-0825.2008.01457.x
da Silva Dalben G, Richieri-Costa A, de Assis Taveira LA. Tooth Abnormalities and Soft Tissue Alterations in Patients With G/BBB Syndrome. Oral Dis. 2008;14(8):747-53. PubMed PMID: 18627501.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome. AU - da Silva Dalben,G, AU - Richieri-Costa,A, AU - de Assis Taveira,L A, Y1 - 2008/06/21/ PY - 2008/7/17/pubmed PY - 2009/4/2/medline PY - 2008/7/17/entrez SP - 747 EP - 53 JF - Oral diseases JO - Oral Dis VL - 14 IS - 8 N2 - OBJECTIVE: The G/BBB syndrome is an X-linked recessive disorder characterized by eye anomalies, laryngotracheoesophageal cleft, congenital heart disease, genitourinary anomalies and gastrointestinal disorders. Patients may also present cleft lip and palate, high-arched palate and thin upper lip. This study aimed to investigate the occurrence of tooth abnormalities and soft tissue changes in patients with G/BBB syndrome. DESIGN: Cross-sectional. SUBJECTS AND METHODS: Twenty-one patients with G/BBB syndrome were analyzed as to the presence of tooth abnormalities and soft tissue alterations. MAIN OUTCOME MEASURES: The prevalence of tooth agenesis and supernumerary teeth was compared to patients without morphofunctional alterations, matched for gender and age. RESULTS: All patients had complete cleft lip and palate; 95.23% of patients presented tooth abnormalities, mainly hypoplastic alterations, with predominance of alterations of number, followed by alterations of structure, shape and position. The frequency of tooth agenesis and supernumerary teeth was significantly higher compared with the control group; 11 patients presented incisiform supernumerary teeth in the mandibular anterior region. Ankyloglossia was observed in 11 of 21 patients. CONCLUSION: The presence of mandibular anterior supernumerary teeth and ankyloglossia should be investigated in the clinical evaluation of patients with suspected diagnosis of the G/BBB syndrome. SN - 1601-0825 UR - https://www.unboundmedicine.com/medline/citation/18627501/Tooth_abnormalities_and_soft_tissue_alterations_in_patients_with_G/BBB_syndrome_ L2 - https://doi.org/10.1111/j.1601-0825.2008.01457.x DB - PRIME DP - Unbound Medicine ER -