Maxillofacial Prosthetic Clinic, Golden Jubilee Medical Centre, Mahidol University, Salaya, Nakon Pathom 73170, Thailand.
Department of Clinical Dentistry, Walailak University International College of Dentistry, Walailak University, Phayathai, Bangkok 10400, Thailand.
Kathmandu Univ Med J (KUMJ). 2022 Jul-Sep;20(79):391-395.
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 medicaldental care is needed in such patients with support from family members, friends, and colleagues.
弗雷泽综合征（FS，MIM 219000）是一种罕见的常染色体疾病，其特征为全身和口面畸形，通常包括隐眼、喉畸形、并指和泌尿生殖缺陷。我们报告了一个 21 岁 FS 患者，她有部分缺牙，寻求美容牙科治疗。临床检查发现双侧隐眼、手和脚广泛的并指、宽鼻伴鼻梁凹陷，以及双侧唇裂已手术矫正。她表现为 III 类颌关系，面下部垂直高度降低。使用计算机辅助设计（CAD）和计算机辅助制造（CAM）工艺，由丙烯酸树脂（VIPI BLOCK TRILUX ® ，VIPI Industria，Pirassununga，SP，巴西）制成的上、下覆盖义齿对患者进行了修复。在随访时，患者的美观和功能均得到改善。FS 患者的适当管理和康复具有挑战性，但目前缺乏口腔健康管理的标准指南。本文介绍了一例 FS 患者，表现出口腔和颅面异常，并进行了修复。我们还为 FS 患者提供了最佳口腔保健建议。功能适应和康复在 FS 患者的各种功能、生存和生活质量方面发挥着重要作用。此类患者需要整合医疗和牙科护理，并得到家庭成员、朋友和同事的支持。
