chondroectodermal dysplasia: a rare syndrome.

نویسندگان

dana tahririan resident, department of pediatric dentistry, faculty of dentistry, isfahan university of medical sciences, isfahan, iran.

alireza eshghi associate professor of pediatric dentistry, isfahan university of medical sciences, isfahan, iran.

pirooz givehchian resident, department of prosthodontics, faculty of dentistry, isfahan university of medical sciences, isfahan, iran.

mohammad ali tahririan assistant professor of orthopedics, isfahan university of medical science, isfahan, iran.

چکیده

chondroectodermal dysplasia (ellis-van creveld syndrome) is a rare autosomal recessive congenital abnormality. this syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. it is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. the case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Chondroectodermal Dysplasia: A Rare Syndrome

Chondroectodermal dysplasia (Ellis-Van Creveld syndrome) is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during den...

متن کامل

Chondroectodermal dysplasia (Ellis-Van Creveld syndrome: A case report

Ellis-Van Creveld syndrome is a very rare congenital disorder which its principal features are polysyndactyly, chondrodysplasia, cardiac abnormalities and ectodermal dysplasia. We report a 10-year-old girl with major manifestations of this syndrome who also had multiple brownish macules and patches on trunk and extremities with aortic and pulmonary stenosis in echocardiographic evaluations.

متن کامل

Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report

A case of chondroectodermal dysplasia (Ellis-van Creveld syndrome) with a remarkable number of the classic oral and dental changes is described. This syndrome involves all embryonic tissue layers and is polysymptomatic; yet some oral and dental manifestations are pathognomonic and must be considered in primary diagnostic criteria. However, in some patients, these oral and dental manifestations ...

متن کامل

Chondroectodermal dysplasia (Ellis-van Creveld syndrome): a case report.

Ellis-van Creveld (EVC) syndrome is a form of skeletal and chondroectodermal dysplasia, occurring with and without systemic involvement. Taurodontism of permanent and primary molars and upper posterior supernumerary teeth are rarely associated with this syndrome. A 5-year-old girl presented with early childhood caries and hypodontia. She had labiogingival adhesion, labiogingival frenulum hypert...

متن کامل

Ellis-van Creveld syndrome, (chondroectodermal dysplasia syndrome) in a Gurkha family.

The first reported case of Ellis--van Creveld syndrome in a Gurkha child is described, and the implications of the syndrome in this ethnic group are briefly considered.

متن کامل

Thanatophoric Dysplasia; a Rare Case Report on a Congenital Anomaly

The rare form of skeletal dysplasia is thanatophoric dysplasia. The meaning for thanatophoric dysplasia is death bearing which is derived from Greek word. It occurs 1in 20,000 to 50,000. It is mainly due to mutations in the fibroblast growth factor receptor 3gene. Features of thanatophoric dysplasia are frontal bossing, prominent eyes, narrow thorax, protruded abdomen and bowed legs. The knowle...

متن کامل

منابع من

با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید


عنوان ژورنال:
journal of dentistry, tehran university of medical sciences

جلد ۱۱، شماره ۳، صفحات ۳۶۱-۴

میزبانی شده توسط پلتفرم ابری doprax.com

copyright © 2015-2023