Tooth abnormalities and soft tissue alterations in patients with G/BBB syndrome.
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.


METHODS
Cross-sectional.


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


METHODS
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.


CONCLUSIONS
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.
da Silva Dalben
G
G
Paediatric and Community Dentistry Sector, Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru. gsdalben@usp.br
Richieri-Costa
A
A
de Assis Taveira
L A
LA
D000015:Abnormalities, Multiple; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000848:Anodontia / Q000453:epidemiology; D001641:Bicuspid / Q000473:pathology; D001938:Brazil / Q000453:epidemiology; D016022:Case-Control Studies; D002648:Child; D002971:Cleft Lip / Q000453:epidemiology; D002972:Cleft Palate / Q000453:epidemiology; D019465:Craniofacial Abnormalities / Q000453:epidemiology*; D003430:Cross-Sectional Studies; D003481:Cuspid / Q000473:pathology; D003744:Dental Enamel Hypoplasia / Q000473:pathology; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D008297:Male; D009056:Mouth Abnormalities / Q000453:epidemiology*; D015995:Prevalence; D012737:Sex Factors; D013577:Syndrome; D014060:Tongue Diseases / Q000453:epidemiology; D014071:Tooth Abnormalities / Q000453:epidemiology*; D014079:Tooth Eruption, Ectopic / Q000453:epidemiology; D014096:Tooth, Supernumerary / Q000453:epidemiology; D055815:Young Adult