Publication: Oral and Craniofacial Anomalies of Fraser Syndrome: Prosthetic Management
Date
2022
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
ABSTRACT
Fraser syndrome (FS, MIM 219000) is a rare autosomal disorder characterized
by systemic and oro-facial malformation, usually comprising cryptophthalmos,
laryngeal malformations, syndactyly, and urogenital defects. We presented a
21-year-old FS case with partial missing teeth seeking aesthetic dental treatment.
Clinical examination revealed bilateral cryptophthalmos, extensive syndactyly
of hands and feet broad nose with the depressed nasal bridge, and surgically
corrected bilateral cleft lip. She presented class III jaw relation and reduced the
vertical height of the face. Prosthetic rehabilitation of the patient was done with
upper and lower overlay dentures made from acrylic resin (VIPI BLOCK TRILUX®,
VIPI Industria, Pirassununga, SP, Brazil) using computer-aided design (CAD)
and computer-aided manufacturing (CAM) process. At the follow-up visit, the
patient presented improved aesthetics and function. Proper management and
rehabilitation of FS patients are challenging, but standard guidelines for oral health
management are currently lacking. This article presents a case of Fraser syndrome
presenting oral and craniofacial anomalies, and prosthetic rehabilitation was
done. We also provided recommendations for the optimal oral health care for the
FS patients. Functional adaptation and rehabilitation have significant roles in the
various functions, survival, and quality of the life of FS patients. Integrated medical-
dental care is needed in such patients with support from family members, friends,
and colleagues.
KEY WORDS
Cryptophthalmos, Fraser syndrome, Overlay denture, Syndactyly
Description
Amornvit P,1 Rokaya D,2 Sapkota D,3 Sanohkan S4
1Maxillofacial Prosthetic Clinic
Golden Jubilee Medical Centre, Mahidol University
Salaya, Nakon Pathom 73170, Thailand
2Department of Clinical Dentistry
Walailak University International College of Dentistry
Walailak University, Phayathai, Bangkok 10400
Thailand
3Department of Oral Biology
Faculty of Dentistry, University of Oslo, Oslo, Norway
4Department of Prosthetic Dentistry
Faculty of Dentistry, Prince of Songkla University
Hat Yai, Songkhla 90110, Thailand
Keywords
Cryptophthalmos, Fraser syndrome, Overlay denture, Syndactyly