MANAGEMENT OF ORAL MANIFESTATIONS OF ELLIS VAN CREVELD SYNDROME: A CASE REPORT

Prof. Divya S. Sharma, *Dr. Ashik Mohamed VN and Dr. Pranshu Varshney

ABSTRACT

A rare autosomal-recessive disorder called Ellis-van Creveld syndrome is characterized by short limbs, post-axial polydactyly, ectodermal dysplasia, an edentulous mandibular incisor region, the absence of a mucobuccal fold, congenitally missing teeth, light alveolar ridge serrations, and numerous small alveolar notches. This clinical report includes findings such as polydactyly, clinodactyly, hypoplastic nails, congenitally missing multiple teeth, serrations in the incisors and alveolar notches. The case report also emphasizes on patients' preventive and therapeutic oral health care as well as differential diagnosis. Management of Ellis-van Creveld syndrome involves dentist, nutritionist, radiologist, pediatrician, cardiologist, orthopaedician, psychologist, geneticist for the positive outcome of the disorder. Recognizing the oral findings is important for early diagnosis and correct treatment planning of the syndrome. Various treatment strategies include oral prophylaxis, preventive measures for dental caries, restoration of dental caries, prosthodontic rehabilitation, orthodontic correction of malocclusion, and surgical corrections of soft tissue and skeletal abnormalities which must be followed up regularly by a pedodontist.

Keywords: Ellis-van Creveld syndrome, Chondroectodermal dysplasia, Dental anomalies.