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

2. 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.

3. 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

4. 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.

5. Pictures of Ellis-van Creveld Syndrome

6. Intraoral Pictures

7. List Sources and References (This should be more than your text book!)
Picture sources:    
Informational sources:  
Oral Pathology for the Dental Hygienist text book. Pg. 212 & 226.