Craniofacial and oral features in a case of Tatton-Brown-Rahaman syndrome

TATTON-BROWN-RAHAMAN Syndrome is also known as the DMNT3A overgrowth syndrome, that was firstly described in 2014 on a series of 13 patients, characterized both by intellectual disability and autistic spectrum, and by physical abnormalities such as: overgrowth and tall stature, facial dismorphism, joint hypermobility, dolicocephaly with macrodontia of upper incisors. heavy horizontal eyebrows. Case report: A 14-year-old male patient attended the Meyer's Special Dental Care Unit as affected by mental disability, behavioral difficulties including parent's abili- ties in mantaining an adeguate oral hygene. Facial aspect was of a long, dolicocephaly cranial growth subject, with protrusion of the upper incisors and difficulties in man- teining a proper labial seal. Upper dental arch revealed to be narrow, and the lower arch was relatively retruded. Radiographically upper left cuspid resulted palatally im- pacted. Heavy horizontal eyebrows were also observed. Discussion: few cases of such arare entity are reported; we compoared all other clinical data of patients affected by the same ewntity. Conclusion: We describe craniofacial characteristics, oral and facial aspects of a boy with a rare "de novo" DNMT3A variant, so far unreported.