Freeman–Sheldon Syndrome (Freeman–Burian Syndrome): A Contemporary Literature Review

Abstract

Freeman–Sheldon syndrome (FSS), also known as Freeman–Burian syndrome (FBS), is a rare congenital disorder characterized by distinctive craniofacial abnormalities, musculoskeletal contractures, and variable systemic involvement. Recent evidence has redefined FSS as a congenital craniofacial myopathy rather than primarily a distal arthrogryposis syndrome, emphasizing the importance of characteristic facial features in diagnosis. This narrative review summarizes current evidence on the clinical presentation, pathogenesis, diagnosis, and multidisciplinary management of FSS, with particular emphasis on its dental manifestations and implications for dental practice. Common oral findings include microstomia, high-arched palate, malocclusion, hypodontia or oligodontia, ankyloglossia, and restricted mouth opening, all of which present significant challenges during dental treatment. Dentists play a crucial role in the early recognition of the syndrome and in planning comprehensive oral healthcare, including preventive, restorative, orthodontic, and anesthetic considerations. Although the available evidence is largely based on case reports and small case series, increasing awareness of the characteristic dental and craniofacial features can facilitate timely diagnosis, multidisciplinary management, and improved patient outcomes.