Langer-Giedion syndrome (LGS) and trichorhinophalangeal syndrome (TRPS) type II are contiguous gene syndromes caused by loss of functional copies of the
TRPS1 and
EXT1 genes associated with 8 q chromosome deletion.
TRPS1 gene deletion causes craniofacial dysmorphism and skeletal abnormalities, while deletion of the
EXT1 gene causes multiple cartilaginous exostoses. Patients with LGS have clinical variability, depending on the loss of additional genes associated with the variability of the size of the deleted chromosomes. However, dental abnormalities have not been extensively reportedinLGS,incontrasttoTRPStypeI,whichiscausedbylossoffunctionofthe
TRPS1 gene.Here, we describe dental anomalies including an impacted supernumerary tooth, delayed tooth eruption, and crowded teeth in an 11-year-old boy with LGS and mild mental retardation. A large number of carious teeth were found along with poor oral hygiene. The chief complaint was tooth pain caused by dental caries as well as crowding in the maxillary anterior teeth. Radiographic findings also revealed an impacted supernumerary tooth in the left mandibular premolar region. In the present case,there were several risk factors associated with LGS in regard to dental treatment and management,such as mental retardation, hyperflexible joints, and redundant skin. With behavior modification therapy to promote cooperation for dental treatment at our outpatient clinic, both treatment of the dental caries and extraction of the impacted supernumerary tooth were successfully performed under local anesthesia. We are continuing follow-up examinations every 3−6 months to maintain oral hygiene and observe eruption of permanent teeth.
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