Aim: This article describes the craniofacial and dental features of an individual with Gordon syndrome. The dental management implications and considerations of treating patients with Gordon Syndrome and similar conditions resulting in limited mouth opening are discussed. Methods: A 14-year-old South African male was referred to the Dental Genetics Clinic with the main complaint of carious teeth. His craniofacial characteristics included low set and posteriorly rotated ears, down-slanted palpebral fissures, sloping shoulders, and a broad neck. A prognathic mandible and mild facial asymmetry were noted. He had a significant limitation of mouth opening (2 cm at incisors). Radiographic examination revealed multiple carious teeth, missing mandibular premolars, impacted maxillary premolars, taurodontism of the 44 and 34, and enlarged coronoid processes of the mandible. Dental extractions and restorations have been performed under local anaesthesia. Conclusion: Gordon syndrome and similar conditions, may result in limited oral opening and impaired manual dexterity. The severity of limitation of mouth opening determines management. Dental management should focus on ensuring that the patient is able to maintain good oral hygiene by customising homecare for the individual and regular dental visits.
K E Y W O R D Sdistal arthrogryposis, Gordon syndrome, limited mouth opening
BACKGROUNDGordon syndrome (OMIM 114300), also known as Distal Arthrogryposis type 3, is a rare autosomal-dominant genetic disorder. 1,2 It falls within the spectrum of conditions known as Arthrogryposis Multiplex Congenita (AMC) which is used to describe congenital arthrogryposis affecting two or more areas of the body. The condition was first described by Gordon et al in 1969, in a South African family consisting of six affected individuals over three generations. 2 The condition is characterised by
