Ellis-van Creveld Syndrome

Kaitlyn ScheidDate: 10/21/2010

Patient:

Age: 18

Gender: F

Height: 4”0

Weight: 90lbs

Vital Signs: HR:68 bpmRespiratory rate: 12 Blood Pressure: 113/75Temperature:97.2

Chiefcomplaint

Wants teeth reshaped.

MedicalAlert

Needs pre-med due to cardiac defect.

Name of Syndrome:

Ellis-Van Creveld Syndrome (chondroectrodermal dysplasia)

Cause of Syndrome - Medical History (if applicable) An autosomal-recessive inheritance.

Orofacial Clinical Features

Absent upper vestibule, serrated lower alveolar ridge, polydactyly. malocclusion, labiogingival adherences and gingival hypertrophy, labiogingival frenulum hypertrophy, accessory labiogingival frenula, serrated incisal margins, dental transposition, diastrema, conical teeth, enamel hypoplasia and hypodontia. Teeth may be prematurely erupted, at birth, or exfolliate prematurely.

Age/race/sex predilections and Systemic Clinical Features:

Age- From Birth. As common in males as females. More common in the Amish community. Systemic clinical features include small stature due to shortening of limbs, ectodermal dysplasia affecting hair, nails and teeth, extra phalanges, and congenital heart malformations.

Radiographic Features of this Syndrome

Thinning of enamel

Special considerations in Treatment of this patient? How is it Diagnosed?How common is it? Is it a horse or zebra?

Patient must take a pre-med due to cardiac defects. Patient should probably be treated in sub-supine position because of heart malformations. Ellis-van Creveld Syndrome is diagnosed by clinical features such as skeletal features, oral manifestations (including hare lip), short stature, and ploydactyl affecting hands. Differential diagnosis includes supernumerary digits, and ectodermal dysplasia. This is a rare disease with only 150 cases world wide.

Pictures of Ellis-van Creveld Syndrome

Intraoral Pictures

List Sources and ReferencesPicture sources:http://www.ijdr.in/articles/2007/18/1/images/IndianJDentRes_2007_18_1_31_30920_11.jpg

http://www.jisppd.com/articles/2008/26/5/images/JIndianSocPedodPrevDent_2008_26_5_19_41749_11.jpg

www.mdconsult.com http://www.netterimages.com/images/vtn/000/000/000/383-150x150.jpg

http://www.jisppd.com/articles/2007/25/5/images/JIndianSocPedodPrevDent_2007_25_5_5_34737_4.jpg

http://www.jisppd.com/articles/2008/26/5/images/JIndianSocPedodPrevDent_2008_26_5_19_41749_5.jpg

http://health.allrefer.com/health/ellis-van-creveld-syndrome-skin-cancer-melanoma-on-the-fingernail.html Informational sources: http://www.ojrd.com/content/2/1/27

Oral Pathology for the Dental Hygienist text book. Pg. 212 & 226.