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

Abstract

IntroductionEllis‐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.ObjectiveIn 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 ReportClinical 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.ConclusionIn 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.

Diode Laser Surgery of Peripheral Ossifying Fibroma in Children: A Case Report with Narrative Review of the Literature

“…Considering that 95% of POF cases occur in 10-19 years old pediatric patients, only few studies report of POF cases in children under 10 years [24]; for this reason, we decided to carry out a brief review of the relevant literature about POF in pediatric patients, and the results are presented as follows. Tomáz Ferraresso [25] described an interesting case of POF of the anterior mandible in a 6year-old patient, affected by Ellis-van Creveld (EVC) syndrome, an autosomal recessive disease consisting in skeletal and ectodermal dysplasia, characterized by short stature, postaxial polydactyly, ectodermal dysplasia, and congenital heart disease. The patient showed a small nodular neoformation on the edentulous alveolar ridge in the mandible, without radiological investigations, treated by conventional surgical excision, hemostasis and suture, without recurrence.…”

“…The most reported and performed surgery consists of scalpel, followed by bone debridement, tooth scaling and root planning, with an approach that is as conservative as possible [39]. Of the 12 studies conducted on pediatric patients that we considered in this review, 9 authors have preferred this conventional treatment strategy because of its wellestablished reliability [8,11,14,20,25,26,28,29,32]. The remaining 3 authors reported the surgical excision of POF in newborns using diode laser [27,30,31].…”