Oral and craniofacial manifestations of Ellis–van Creveld syndrome: Case series

Tuna, Elif Bahar; Koruyucu, Mine; Kürklü, Esma; Çifter, Muhsin; Gençay, Koray; Seymen, Figen; Tüysüz, Beyhan

doi:10.1016/j.jcms.2016.04.025

Cited by 11 publications

(13 citation statements)

References 19 publications

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“…It has previously been reported that polydactyly occurs commonly in the hands of EVC patients but occurs in the feet of only 10% of patients with EVC (4). Oral findings such as multiple hyperplastic frenula, diastemas, taurodontism, and hypodontia (mandibular and maxillary anterior region) observed in our patient are also observed in patients with EVC (8). However, natal and supernumerary teeth are seen in 25-30% of patients with EVC (8) and were not observed in our patient.…”

Section: Discussionsupporting

confidence: 53%

Oro-dental and Radiographic Findings of Weyers Acrofacial Dysostosis: Report of a Rare Case

Öz¹,

Kırzıoğlu²

2020

Bezmialem Science

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Section: Discussionsupporting

confidence: 53%

Oro-dental and Radiographic Findings of Weyers Acrofacial Dysostosis: Report of a Rare Case

Öz¹,

Kırzıoğlu²

2020

Bezmialem Science

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“…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].…”

Section: Case Reportmentioning

confidence: 99%

“…Oral aspects include labiogingival adherences, accessory labiogingival frenula, labiogingival frenulum hypertrophy, conical teeth, enamel hypoplasia and hypodontia, natal and neonatal teeth, changes in eruption and exfoliation dental patterns, malocclusion and some others abnormalities [1,5,6].…”

Section: Introductionmentioning

confidence: 99%

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

Delgado

Couto

Marcato

et al. 2021

RGO, Rev. Gaúch. Odontol.

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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.

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“…In the international literature, it is characteristic a tetrad, present in our patient, consisting of: dwarfism, bilateral polydactyly of the hands, ectodermal dysplasia (disorder in fingernails, teeth) and cardiac congenital malformations (2,4-6,10).…”

Section: Discussionmentioning

confidence: 71%

“…It is the result of a genetic defect located in chromosome 4p16. (4) The prevalence varies from 1/1000000 in general population to 7/1500000 in amish population (Lancaster, Pennnsylvania, USA) (2,5).…”

Section: Introductionmentioning

confidence: 99%

Oral manifestations of ellis-van creveld syndrome. A rare case report

et al. 2019

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Ellis-van Creveld syndrome (EVC) or chondroectodermal dysplasia is an autosomal recessive disorder, characterized by dwarfism, polydactyly, hypoplastic fingernails and congenital heart defects, finding in most of the cases orofacial anomalies. We describe a clinical case of a 9 year old male patient diagnosed with EVC who visited our Maxillofacial private consultation at Alcorcon Southern Hospital, presenting typical oral manifestations such as dental agenesis, delayed eruption, hypoplasia of the enamel, dental dysmorphism, taurodontism and supernumerary teeth. EVC syndrome is a rare disease and requires a multidisciplinary approach. Oral features are constant and requires the jointly performance of Odontologist and Maxillofacial surgeon aiming to get an appropriate treatment sequence surgery-orthodontics in order to achieve a suitable functional result to improve the quality of life of these patients. Key words: Ellis-Van creveld syndrome, chondroectodermal dysplasia, oral manifestations, craniofacial manifestations.

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Oral and craniofacial manifestations of Ellis–van Creveld syndrome: Case series

Cited by 11 publications

References 19 publications

Oro-dental and Radiographic Findings of Weyers Acrofacial Dysostosis: Report of a Rare Case

Oro-dental and Radiographic Findings of Weyers Acrofacial Dysostosis: Report of a Rare Case

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

Oral manifestations of ellis-van creveld syndrome. A rare case report

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