Múltiplos Dentes Supranumerários Inclusos: Uma Rara Manifestação Oral Associada à Síndrome de Beckwith-Wiedemann

Abstract

Introduction: Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder characterized by somatic overgrowth and a predisposition to embryonal tumors. The most frequently reported oral manifestation is macroglossia, while the presence of multiple included supernumerary teeth is an atypical. Objective: To report a clinical case of a patient with BWS, emphasizing clinical and radiographic findings, with a focus on the occurrence of multiple impacted supernumerary teeth as a potential expansion of the syndrome’s phenotypic spectrum. Case Report: A 17-year-old male patient was clinically diagnosed with BWS at 28 days of life, according to the criteria established by the International Consensus Statement. He presented four cardinal features (macroglossia, macrosomia, omphalocele, and lateralized overgrowth) and three suggestive features (ear creases/pits, transient hypoglycemia, and hepatomegaly), in addition to gigantism, splenomegaly, and non-obstructive nephrolithiasis. His medical history also included corrected cryptorchidism, seizure episodes, and neonatal sucking difficulties. Cone-beam computed tomography revealed eight included supernumerary teeth: three in the maxilla and five in the mandible. The proposed treatment included surgical extraction of the included supernumerary teeth, with no clinical indication for glossectomy. Conclusion: This report documents a rare oral manifestation in a patient with BWS and reinforces the importance of thorough dental evaluation, including imaging examinations, in the multidisciplinary management of these patients. The presence of multiple included supernumerary teeth may represent an expansion of the phenotypic spectrum of BWS, highlighting the role of dental assessment in early recognition and support of clinical management in such cases.