Ellis-Van Creveld Syndrome, neonatal teeth and breastfeeding impairment: a case report

Danielle PORTINHO,Romulo Andrade MARCATO,Wanda Terezinha Garbelini FROSSARD,Renata Zoraida Rizental DELGADO,Ana Carolina Fernandes COUTO,Cássia Cilene Dezan GARBELINI

doi:10.1590/1981-863720210004620200048

Danielle PORTINHO, Romulo Andrade MARCATO + Show 4 more

Open Access

https://doi.org/10.1590/1981-863720210004620200048

Copy DOI

Abstract

ABSTRACT Because of multisystemic impairment in patients with Ellis-van Creveld syndrome, multidisciplinary care may be demanded since birth to assure breastfeeding. This report presents a case of an Ellis-van Creveld infant that was facing breastfeeding difficulties because of maxillary neonatal teeth. A 3 months old male infant with Ellis-van Creveld syndrome was referred to Pediatric Dentistry Department because of two upper neonatal teeth causing breastfeeding difficulties. Clinical examination revealed that teeth position was compatible to 51 and 61, and both presented uncommon ectopic soft tissue placement, conical crown and hypoplastic enamel covered by a large amount of dental biofilm. Radiography indicated they were of normal series and had 2/3 of crown completion. Due to teeth mobility that impaired breastfeeding, treatment option was exodontia. Early tooth eruption, such as in natal and neonatal teeth, by itself can’t be considered a reason for exodontia. But exodontia must be considered when an early erupted tooth(s) impairs breastfeeding, especially in systemically compromised infants. In this present case report, after teeth extraction, the infant was able to breastfeed and gain weight properly.

Highlights

Ellis Van-Creveld Syndrome (EVCS), known as chondroectodermal dysplasia, is a complex genetic disorder [1]
The aim of this study is to report a case of an infant with EVCS that was facing breastfeeding difficulties due to rare maxillary neonatal teeth
EVCS infants present a diversity of oral problems that can affect breastfeeding - i.e. labiogingival adherences, accessory labiogingival frenula, labiogingival frenulum hypertrophy, natal and neonatal teeth, change in dental eruption timing [1,5,6]

Summary

Introduction

Ellis Van-Creveld Syndrome (EVCS), known as chondroectodermal dysplasia, is a complex genetic disorder [1]. It presents multisystemic features, signs and symptoms ranging from bone and cardiac abnormalities until stomatologic ones [2]. Pediatric dentistry follow-up is important for the prompt diagnosis and treatment of oral disorders, especially in newborns and infants who present conditions that may compromise breastfeeding and may increase the risk of bronchoaspiration [7]. The aim of this study is to report a case of an infant with EVCS that was facing breastfeeding difficulties due to rare maxillary neonatal teeth. The care plan developed to ensure continued breastfeeding is presented

Objectives

Findings

Discussion

Conclusion