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

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.

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

“…Classic oral and dental characteristics found in this patient corroborate those described by several authors: Macroglossia and high-arched palate, teeth agenesis, 2,[5][6][7]9,11,12 conical teeth, 2,5,6,8,9,11,12 enamel hypoplasia, 8,9,11 natal/neonatal teeth, 10,11 bilateral posterior crossbite 7,11,12 presence of multiples accessory labial frenula. 2,6,7,9,11,12 However, in the literature, there are reports of unusual findings, such as taurodontism, 2,6,9,11 supernumerary tooth, 9 microdontia, dens in dente, talons cusp, 2,5 single-rooted deciduous molars, 6 impacted tooth, 2 and dental transposition.…”

“…Parental consanguinity is extensively reported in the literature 2,6,8,10,11,16 and it is present in about 30% of EVC syndrome cases. 4 The patient has a history of consanguineous marriage in the family, a fact that supports the syndrome recessive character.…”

“…Ellis‐van Creveld (EVC) syndrome also known as chondroectodermal dysplasia is a rare autosomal recessive congenital disorder 1,2 caused by mutations in the two genes EVC1 and EVC2 localized in locus 16 on the short arm of chromosome 4 3 . Short limb dwarfism 1,2,4,5–13 postaxial polydactyly of the hands, 1,2,4–14 ectodermal dysplasia may affect hair, teeth and nails 1,2,4–14 and congenital heart malformations 1,4,6,7,10,13 and pulmonary hypoplasia are attributions that characterize the EVC syndrome. Congenital heart defects occur in 50%–60% of cases 4,15,16 …”

“…The prevalence is 7 in 1 million births 4,11 with no gender or race predilection; however, it is commonly found in the Amish community in the state of Pennsylvania, United States 17,18 . Parental consanguinity may be present 2,10,11 in about 30% of the cases 4 …”

“…The prevalence is 7 in 1 million births 4,11 with no gender or race predilection; however, it is commonly found in the Amish community in the state of Pennsylvania, United States. 17,18 Parental consanguinity may be present 2,10,11 in about 30% of the cases. 4 Classic oral manifestations may include tooth agenesis 2,[5][6][7]9,11,12 , conical teeth, 2,5,6,8,9,11,12 taurodontism , 2,6,9,11 enamel hypoplasia, 8,9,11 malocclusion, 7,11,12 presence of multiples and accessory labial frenula, 2,6,7,9,11,12 lower rotated canine, midline notch in the upper lip anterior portion to the maxillary gingival mucosal margin.…”