Ellis‐Van Creveld syndrome: dental management considerations and description of a new oral finding

Hassona, Yazan; Hamdan, M. A.; Shqaidef, Abedalrahman; Karaky, Ashraf Abu; Scully, Crispian

doi:10.1111/scd.12116

Cited by 5 publications

(14 citation statements)

References 18 publications

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“…Although oral characteristics of EVCS are widely documented, there is a lack of reports about the oral condition of EVCS infants. Knowledge about EVCS infants presenting natal/neonatal tooth(s) is mostly based on case reports about EVCS children, adolescents or adults that presented them, according to parents report (memories) [4,12]. A bibliographic search revealed only three reports of NNT in EVCS infants [12,13].…”

Section: Discussionmentioning

confidence: 99%

“…It presents multisystemic features, signs and symptoms ranging from bone and cardiac abnormalities until stomatologic ones [2]. The general occurrence is low, with a prevalence of 7:10 births [3,4] affecting both genders and without race predilection [1]. Congenital heart disease may support the diagnosis and appears to be the main determinant of longevity [1], 50% of childhood deaths are associated with cardiac and respiratory changes that patients may present [3].…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

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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“…Oligodontia and small conical teeth with enamel hypoplasia are also present . The differential diagnosis from similar chondrodystrophies, such as Jeune dystrophy, McKusick‐Kaufman syndrome, includes oro‐facial‐digital syndrome, and Weyers acrodental dysostosis . The oral dental management is quite complex and often requires a multidisciplinary approach to correct the dental defects.…”

Section: Genetic Disordersmentioning

confidence: 99%

“…The oral dental management is quite complex and often requires a multidisciplinary approach to correct the dental defects. Orthodontics and maxillofacial oral surgery are often involved in the treatment; moreover, comprehensive restorative treatment is fundamental to manage enamel hypoplasia and to replace missing teeth …”

Section: Genetic Disordersmentioning

confidence: 99%

Genetic and developmental disorders of the oral mucosa: Epidemiology; molecular mechanisms; diagnostic criteria; management

Pinna

Cocco

Campus

et al. 2019

Periodontology 2000

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A large number of disorders may affect the oral cavity, including genetic diseases, infections, cancers, blood diseases, skin diseases, endocrine and metabolic disorders, autoimmune and rheumatologic diseases, local lesions, to name a few. Oral mucosa shows a considerable variation in its normal structure and a wide range of conditions may affect it. Such conditions are often harmless or minor and could be primary or secondary to systemic disease. Several of them are quite rare and, hence, the diagnosis is not easy. Clinically, lesions may appear as ulcers, discoloration of the oral mucosa and alterations in size and configuration of oral anatomy. Genetic disorders have specific manifestations and can be caused by a derangement of one or more components of the tissue. Many of them follow the skin or systemic signs of the underlying genetic disease, but in a few cases oral signs could be the first manifestation of the disorder. Among them genodermatoses are prominent. They are inherited disorders characterized by a multisystem involvement. This review describes chondro‐ectodermal dysplasia, dyskeratosis congenita, Ehlers‐Danlos syndrome, hereditary benign intraepithelial dyskeratosis, keratosis follicularis, lipoid proteinosis, multiple hamartoma syndrome, pachyonychia congenita, Peutz‐Jeghers syndrome, tuberous sclerosis and white sponge nevus. Other genetic disorders not included in the genodermatosis group and reported in the present review are: acanthosis nigricans, angio‐osteo‐hypertrophic syndrome, encephalotrigeminal angiomatosis, familial adenomatous polyposis, focal dermal hypoplasia, focal palmoplantar and oral mucosa hyperkeratosis syndrome, gingival fibromatosis, Maffucci's syndrome, neurofibromatosis (type 1) and oro‐facial‐digital syndrome (type 1). Disorders during embryonic development might lead to a wide range of abnormalities in the oral cavity; some of them are quite common but of negligible concern, whereas others are rare but serious, affecting not only the oral mucosa, but also other structures of the oral cavity (ie palate, tongue and gingiva). Fordyce′s granules, leukoedema, cysts of the oral mucosa in newborns, retrocuspid papilla, geographic tongue, fissured tongue, median rhomboid glossitis, hairy tongue, lingual varices and lingual thyroid nodule are described. This review may help dentists, dental hygienists, but also general internists and pediatricians to diagnose different disorders of the oral mucosa, to understand the pathogenesis and to schedule a treatment plan.

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“…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.…”

mentioning

confidence: 99%

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

Ferraresso

Fagundes

Padovese

et al. 2023

Special Care in Dentistry

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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.

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Ellis‐Van Creveld syndrome: dental management considerations and description of a new oral finding

Cited by 5 publications

References 18 publications

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

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

Genetic and developmental disorders of the oral mucosa: Epidemiology; molecular mechanisms; diagnostic criteria; management

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

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