| Dental and maxillofacial characteristics in six Japanese individuals with ectrodactyly-ectodermal dysplasia-clefting (EEC)syndrome. | |
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MedLine Citation:
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PMID: 22236363 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Abstract Objective: Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is a congenital anomaly characterized by ectodermal dysplasia, ectrodactyly, cleft lip and palate, and lacrimal duct anomalies. Because this syndrome is frequently accompanied by a congenital lack of teeth, narrow palate, and malocclusion, comprehensive orthodontic intervention is required. Design: To highlight the specific dental and maxillofacial characteristics of EEC syndrome, 6 Japanese individuals diagnosed with EEC are described here. Patients: The subjects consisted of 2 boys and 4 girls (age range, 6.0-13.9 years) diagnosed with EEC syndrome by medical and dental specialists. Their conditions included ectodermal dysplasia (hypodontia, microdontia, enamel hypoplasia, and abnormalities in hair and nails), cleft lip and/or palate, and ectrodactyly. Cephalograms, panoramic X-rays, and dental casts were taken; systemic complications were recorded at the first visit to our dental hospital. Results: All individuals had severe oligodontia with 9 to 18 missing teeth. The missing teeth were mainly maxillary and mandibular incisors and second bicuspids, arranged in a symmetrical manner. Cephalometric analysis showed retruded and short maxilla due to cleft lip and/or palate. Interestingly, all individuals showed a characteristically shaped mandibular symphysis with a retruded point B. It is likely that this unusual symphyseal morphology is due to the lack of mandibular incisors. Conclusions: This study demonstrates the presence of severe oligodontia in the incisal and premolar regions and describes a characteristic maxillary and mandibular structure in Japanese individuals with EEC syndrome. Key Words: EEC syndrome, dental maxillofacial anomalies. |
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Authors:
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Erika Okamura; Naoto Suda; Yoshiyuki Baba; Hiroki Fukuoka; Takuya Ogawa; Mizue Ohkuma; Nozomi Ahiko; Akihiro Yasue; Toshimoto Tengan; Momotoshi Shiga; Michiko Tsuji; Keiji Moriyama |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-1-11 |
Journal Detail:
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Title: The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Volume: - ISSN: 1545-1569 ISO Abbreviation: - Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2012-1-12 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9102566 Medline TA: Cleft Palate Craniofac J Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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a Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School, Tokyo Medical and Dental University. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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