Author/Authors
Aladağ, Buket Uysal Selcuk University - Faculty of Medicine - Department of Pediatrics, Turkey , Yılmaz, Fatma Hilal Selcuk University - Faculty of Medicine - Department of Pediatrics, Turkey , Koçak, Nadir Selcuk University - Faculty of Medicine - Department of Genetics, Turkey , Annagür, Ali Selcuk University - Faculty of Medicine - Department of Neonatology, Turkey
Title Of Article
A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly
شماره ركورد
23720
Abstract
Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC) is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate). A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atrophy), hair (sparse hair and eye brows), teeth (small, absent or dysplastic teeth), nails (nail dystrophy) and exocrine glands (reduction/ absence of sweat, sebaceous and salivary glands). A multidisciplinary approach for treatment is needed which is coordinated by orthopedic, plastic, dental surgeons, ophthalmologist, dermatologists and speech therapists, psychologists. We presented EEC syndrome case with hydrocephaly in the literature.
From Page
531
NaturalLanguageKeyword
Ectrodactyly , ectodermal dysplasia , cleft palate and lip , hydrocephaly
JournalTitle
Cukurova Medical Journal
To Page
535
JournalTitle
Cukurova Medical Journal
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