Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases

Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder primarily affecting bones, which undergo membranous ossification. It commonly presents with significant dental and facial problems, including the hypoplastic maxilla, retained primary teeth, unerupted permanent teeth and s...
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Autor*in:	Shikha Gupta [verfasserIn] Sunita Gupta [verfasserIn] Khushboo Singh [verfasserIn] Prajat Arora [verfasserIn] Sinny Goel [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2015

Schlagwörter:	Cleidocranial dysostosis dysplasia Marie-Sainton

Übergeordnetes Werk:	In: Journal of Oral and Maxillofacial Radiology - Wolters Kluwer Medknow Publications, 2018, 3(2015), 2, Seite 58-62
Übergeordnetes Werk:	volume:3 ; year:2015 ; number:2 ; pages:58-62

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.4103/2321-3841.157525

Katalog-ID:	DOAJ076663469

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520			\|a Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder primarily affecting bones, which undergo membranous ossification. It commonly presents with significant dental and facial problems, including the hypoplastic maxilla, retained primary teeth, unerupted permanent teeth and supernumerary teeth. It is usually diagnosed based on clinical and radiographic findings, which are pathognomic. Here, we report clinical and radiographic findings of 7 patients with CCD who reported to our Institute during 2013-2014. Results: Age of patient ranged from 13 to 55 years, out of which 5 were males and 2 were females. Characteristic clinical findings, which were present in all cases include frontal and parietal bossing, brachycephalic skull, hypertelorism, broad nasal bridge and shoulder hypermobility. Radiographic findings seen were hypoplasia/aplasia of the clavicle, open skull suture, wide, open fontanel, and the presence of impacted permanent and supernumerary teeth. In one case, there was follicular cyst associated with impacted mandibular canine. Conclusion: Successful treatment involves a multidisciplinary approach and involves timely diagnosis at an early stage of development
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callnumber-first	R - Medicine
author	Shikha Gupta
spellingShingle	Shikha Gupta misc RK1-715 misc R895-920 misc Cleidocranial misc dysostosis misc dysplasia misc Marie-Sainton misc Dentistry misc Medical physics. Medical radiology. Nuclear medicine Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases
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topic_title	RK1-715 R895-920 Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases Cleidocranial dysostosis dysplasia Marie-Sainton
topic	misc RK1-715 misc R895-920 misc Cleidocranial misc dysostosis misc dysplasia misc Marie-Sainton misc Dentistry misc Medical physics. Medical radiology. Nuclear medicine
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title	Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases
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title_full	Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases
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title_sort	cleidocranial dysplasia: spectrum of clinical and radiological findings in seven cases
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title_auth	Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases
abstract	Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder primarily affecting bones, which undergo membranous ossification. It commonly presents with significant dental and facial problems, including the hypoplastic maxilla, retained primary teeth, unerupted permanent teeth and supernumerary teeth. It is usually diagnosed based on clinical and radiographic findings, which are pathognomic. Here, we report clinical and radiographic findings of 7 patients with CCD who reported to our Institute during 2013-2014. Results: Age of patient ranged from 13 to 55 years, out of which 5 were males and 2 were females. Characteristic clinical findings, which were present in all cases include frontal and parietal bossing, brachycephalic skull, hypertelorism, broad nasal bridge and shoulder hypermobility. Radiographic findings seen were hypoplasia/aplasia of the clavicle, open skull suture, wide, open fontanel, and the presence of impacted permanent and supernumerary teeth. In one case, there was follicular cyst associated with impacted mandibular canine. Conclusion: Successful treatment involves a multidisciplinary approach and involves timely diagnosis at an early stage of development
abstractGer	Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder primarily affecting bones, which undergo membranous ossification. It commonly presents with significant dental and facial problems, including the hypoplastic maxilla, retained primary teeth, unerupted permanent teeth and supernumerary teeth. It is usually diagnosed based on clinical and radiographic findings, which are pathognomic. Here, we report clinical and radiographic findings of 7 patients with CCD who reported to our Institute during 2013-2014. Results: Age of patient ranged from 13 to 55 years, out of which 5 were males and 2 were females. Characteristic clinical findings, which were present in all cases include frontal and parietal bossing, brachycephalic skull, hypertelorism, broad nasal bridge and shoulder hypermobility. Radiographic findings seen were hypoplasia/aplasia of the clavicle, open skull suture, wide, open fontanel, and the presence of impacted permanent and supernumerary teeth. In one case, there was follicular cyst associated with impacted mandibular canine. Conclusion: Successful treatment involves a multidisciplinary approach and involves timely diagnosis at an early stage of development
abstract_unstemmed	Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder primarily affecting bones, which undergo membranous ossification. It commonly presents with significant dental and facial problems, including the hypoplastic maxilla, retained primary teeth, unerupted permanent teeth and supernumerary teeth. It is usually diagnosed based on clinical and radiographic findings, which are pathognomic. Here, we report clinical and radiographic findings of 7 patients with CCD who reported to our Institute during 2013-2014. Results: Age of patient ranged from 13 to 55 years, out of which 5 were males and 2 were females. Characteristic clinical findings, which were present in all cases include frontal and parietal bossing, brachycephalic skull, hypertelorism, broad nasal bridge and shoulder hypermobility. Radiographic findings seen were hypoplasia/aplasia of the clavicle, open skull suture, wide, open fontanel, and the presence of impacted permanent and supernumerary teeth. In one case, there was follicular cyst associated with impacted mandibular canine. Conclusion: Successful treatment involves a multidisciplinary approach and involves timely diagnosis at an early stage of development
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title_short	Cleidocranial dysplasia: Spectrum of clinical and radiological findings in seven cases
url	https://doi.org/10.4103/2321-3841.157525 https://doaj.org/article/a74f488b4a5a45faa25a1bd8283c098c http://www.joomr.org/article.asp?issn=2321-3841;year=2015;volume=3;issue=2;spage=58;epage=62;aulast=Gupta https://doaj.org/toc/2321-385X
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