Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

Shivaprakash, P K; Joshi, Hrishikesh V; Noorani, Hina; Reddy, Venugopal

doi:10.4103/0976-237x.95120

Cited by 7 publications

(22 citation statements)

References 6 publications

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“…The improvement in our case was reflected on the patient's acceptance to continue the prosthetic treatment and space management on the dental chair. This was further confirmed by the reduced overall scale scores of the (CPQ [11][12][13][14] after treatment. The positive behavior was also noticeable in taking care of the space management appliances, complying with the oral hygiene instructions and dietary recommendations as well as attending the recall visits on regular basis.…”

Section: Discussionmentioning

confidence: 80%

“…Proper oral hygiene instructions and dietary recommendations were given to the child and the mother. Oral health-related quality of life (OHRQoL) was assessed using a validated Arabic version 8 of the (16-items short-form) child perceptions questionnaire (CPQ [11][12][13][14] ). 9,10 The questionnaire consisted of four domains: oral symptoms (n = 4), functional limitations (n = 4), emotional wellbeing (n = 4), and social well-being (n = 4).…”

Section: Managementmentioning

confidence: 99%

“…The child's quality of life was as well improved. This was evidenced by the reduction in the overall scale scores of the (CPQ [11][12][13][14] ) after oral rehabilitation ( Table 1).…”

Section: Managementmentioning

confidence: 99%

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Dental management of a child with ectrodactyly ectodermal dysplasia cleft lip/palate syndrome: A case report

Elhamouly

Dowidar

2019

Special Care in Dentistry

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Ectrodactyly ectodermal dysplasia with clefting is a rare syndrome resulting from TP63 gene mutations. It is inherited in autosomal dominant manner or as a de novo transfiguration. It is characterized by a triad of ectodermal dysplasia, ectrodactyly, and facial clefts. This report represents a clinical case of 5 years and 6 months‐old male child with ectrodactyly ectodermal dysplasia cleft lip and palate syndrome requiring treatment of his carious teeth. After history taking and clinical examination, the necessary treatment was provided under general anesthesia due to the definitely negative behavior of the child. The treatment outcomes had a positive impact on the behavior and acceptance to dental treatment. This was evidenced by completion of the prosthetic and space management appliances on the dental chair. The child's quality of life was consequently improved. This was evidenced by the reduced response scores of the child perception questionnaire (CPQ11‐14) after treatment. This report highlighted the value of proper diagnosis and fulfillment of the unmet dental needs for patients with orofacial syndromes to improve their quality of life.

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Section: Discussionmentioning

confidence: 80%

Section: Managementmentioning

confidence: 99%

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Dental management of a child with ectrodactyly ectodermal dysplasia cleft lip/palate syndrome: A case report

Elhamouly

Dowidar

2019

Special Care in Dentistry

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“…I) [7][8][9]. En effet, l'anomalie détectable à partir de la 16 e semaine de vie intra-utérine à l'échographie nécessite une recherche de pathologie associée, comme par exemple le syndrome de Pierre Robin qui regroupe une hypoplasie congénitale de la mandibule, une glossoptose et une fente palatine.…”

Section: Commentairesunclassified

Forme fruste de fente labio-palatine : présentation d’un cas clinique

Devisse¹,

Hacquard²,

Lelièvre

et al. 2012

Med Buccale Chir Buccale

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Résumé -Les fentes labio-palatines sont les dysmorphoses buccales congénitales les plus fréquentes (1/600 naissances). Elles peuvent être associées à des syndromes polymalformatifs. Malgré une étiologie inconnue, l'hérédité serait un facteur prédominant et il existerait des facteurs prédisposants comme la toxicomanie maternelle, l'alcoolisme parental, la prise de certains médicaments pendant la grossesse. Ces fentes résultent d'une absence ou d'une insuffisance de fusion des différents bourgeons faciaux se déroulant de la 5 e à la 7 e semaine de vie intra-utérine, aboutissant à diverses formes de fentes plus ou moins marquées : les fentes labiales, labio-alvéolaires, palatovélaires, unilatérales ou bilatérales, complètes ou incomplètes. Les fentes labio-palatines de forme fruste, également appelées mineures ou abortives, n'ont pas d'incidence sur l'esthétique : moins visibles, elles sont moins connues. Elles se caractérisent par une luette bifide mais elles peuvent également comporter des anomalies dentaires comme par exemple des dents gémellées ; la dent la plus concernée par ces anomalies étant l'incisive latérale. Dans les formes mineures, il existe également la fente palatine sous-muqueuse qui est une sous-catégorie de fente palatine pour laquelle la plupart des individus atteints ne décrivent aucun symptôme. Elle touche le palais mou et peut entrainer une insuffisance vélo-pharyngée qui affecte la qualité et l'intelligibilité de l'élocution. Le cas rapporté évoque ces formes frustes. L'odontologiste a un rôle dans la prise en charge de ces patients d'une part pour établir un diagnostic précoce et d'autre part pour réaliser un traitement approprié permettant d'éviter des malpositions, des interférences occlusales, des rétentions provoquant des complications infectieuses. L'approche de ce type de fentes est multidisciplinaire associant chirurgien-dentiste, médecin ORL, orthodontiste. Abstract -Minor form of clef lip and palat: a clinical case report.The cleft lip and palate are congenital oral dysmorphoses most common (1/600 births). They can be associated with multiple malformation syndromes. Despite an unknown etiology, heredity is a dominant factor and there would be predisposing factors such as maternal drug use, parental alcoholism, medications during pregnancy. It result from a lack or insufficiency of fusion of different facial buds unfolding of the 5th to the 7th week of intrauterine life, leading to various forms of more or less marked slots: cleft lip, lip and alveolar, palato-velar, unilateral or bilateral, complete or incomplete. The cleft lip and palate in crude form, also called minor or abortive forms, are having no impact on aesthetics: less visible, they are less known. They are characterized by a bifid uvula and also have consequences for dental example geminated teeth, the teeth most affected by the anomalies being the lateral incisor along the slit. In mild forms, there is also the submucosal cleft palate is a subcategory of cleft palate for which most affected individuals do not describe ...

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“…To date, about 200 cases have been described [26]. Some patients affected by EEC show a form of "incomplete syndrome," lacking, for example, the ectodermal components [27].…”

Section: Ectrodactyly Ectodermal Dysplasia Cleft Lip and Palate (Eementioning

confidence: 99%

Genetic Factors in Selected Complex Congenital Malformations with Cleft Defect

Wójcicki¹,

Koźlik²,

Wójcicka³

2016

Adv Clin Exp Med

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Ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome: A case report of "Incomplete syndrome"

Cited by 7 publications

References 6 publications

Dental management of a child with ectrodactyly ectodermal dysplasia cleft lip/palate syndrome: A case report

Dental management of a child with ectrodactyly ectodermal dysplasia cleft lip/palate syndrome: A case report

Forme fruste de fente labio-palatine : présentation d’un cas clinique

Genetic Factors in Selected Complex Congenital Malformations with Cleft Defect

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