Dental Considerations in Patients with Loeys-Dietz Syndrome: A Review of the Literature and Case Report.

Abstract

Loeys-Dietz Syndrome (LDS) is a rare connective tissue disorder with an autosomal dominant pattern of inheritance, linked to heterozygous mutations in six genes from the transforming growth factor beta receptor complex. The classical syndrome characteristics include aortic aneurisms with generalized arterial tortuosity, hypertelorism and cleft palate or bifid/broad uvula. LDS is also associated with a wide range of skeletal, craniofacial, cutaneous and ocular abnormalities, as well as allergic, atopic and inflammatory diseases. Common oral findings include high arched and/or narrow palate, enamel defects and class II skeletal malocclusion. Dental management of patients with LDS is complex and includes approaches to prevent medical complications, as well as considerations for safe delivery of dental care. The purpose of this report, reviews the literature related to LDS oral manifestations as well as to describe the comprehensive dental management of an adolescent patient with LDS and discuss the challenges that dental practitioners may face when providing treatment for these patients. LDS is a newly described syndrome and the literature reviewing its oral manifestations is limited. Patients are reported to have lower oral health-related quality of life as a result of tooth sensitivity and malocclusions. Dental management is compounded by the complexity of medical factors that should be taken into consideration for the safe delivery of care. The presented case is an example of the challenges that dental practitioners may face when providing treatment for LDS patients.