Peripheral odontogenic fibroma in a child with Ellis-van Creveld syndrome: Case report.

Autor: Ferraresso LFOT; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Fagundes FAU; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Padovese M; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Singi P; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Paiva MF; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Inagaki-Nomura LT; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Dezan-Garbelini CC; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil., Boer FAC; Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil.
Jazyk: angličtina
Zdroj: Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry [Spec Care Dentist] 2024 Jan-Feb; Vol. 44 (1), pp. 103-108. Date of Electronic Publication: 2023 Apr 11.
DOI: 10.1111/scd.12855
Abstrakt: Introduction: Ellis-van Creveld (EVC) syndrome is an autosomal recessive disorder predominantly characterized by a disproportionate dwarfism, ectodermal dysplasia, postaxial polydactyly, and congenital heart malformations and pulmonary hypoplasia.
Objective: In this article, we hereby present a case of a 6-year-old Brazilian boy with EVC syndrome who presented a rare oral lesion as well as a remarkable number of classical and uncommon oral and dental features.
Case Report: Clinical and radiographic examination revealed multiple enamel hypoplasia, teeth agenesis, conical teeth, lower canine rotation, bilateral posterior crossbite, taurodontism of deciduous and permanent molars and delayed tooth eruption, dental caries, and absent vestibular sulcus. Additionally, a whitish lobulated nodule located in the alveolar ridge in the anterior region of the mandible was noted. Anatomopathological examination was compatible with the diagnosis of peripheral odontogenic fibroma (POF). In a 10-month clinical follow-up, no signs of recurrence were observed.
Conclusion: In view of the characteristic oral findings of EVC syndrome and the potential for recurrence of POF, the pediatric dentist plays an essential role in clinical follow-up, planning and preventive, and rehabilitative treatment.
(© 2023 Special Care Dentistry Association and Wiley Periodicals LLC.)
Databáze: MEDLINE
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