Ellis-van Creveld Syndrome

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Presentation transcript:

Ellis-van Creveld Syndrome Kaitlyn Scheid Date: 10/21/2010

Ellis-Van Creveld Syndrome (chondroectrodermal dysplasia) Name of Syndrome: Ellis-Van Creveld Syndrome (chondroectrodermal dysplasia) Patient: Age: 18 Gender: F Height: 4”0 Weight: 90lbs Vital Signs: HR:68 BPM irregular Respiratory rate: 12 RPM labored Blood Pressure: 113/75 mmHg Temperature: 97.2 Chief complaint Wants teeth reshaped. Medical Alert Needs pre-med due to cardiac defect. Cause of Syndrome - Medical History (if applicable) An autosomal-recessive inheritance.

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. 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. Radiographic Features of this Syndrome Thinning of enamel

This is a rare disease with only 150 cases world wide. 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 polydactyl 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 References (This should be more than your text book!) Picture 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.