Gordon syndrome: Dental implications and a case report

Abstract

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

Congenital mandibular coronoid process hyperplasia and associated diseases

“…Gordon syndrome (OMIM 114300), also known as distal arthrogryposis type 3, is a rare autosomal‐dominant genetic disorder characterized by cleft palate and multiple congenital contractures of both hands and feet. Roomaney et al (2021) reported a 14‐year‐old male patient with Gordon syndrome and bilateral CPH.…”

Study evaluating prevalence of Taurodonts in patients using Cone Beam Computed Tomography

“…Ti=a/bX100 where as in the past only 1 variable was used to diagnose taurodonts which is distance from top of pulp chamber to floor. 17 Also there are differences in different races regarding prevalence of taurodont. Jabali AH et al found prevalence of taurodonts to be 0.1 % in Saudi Arabia.…”

Comparação da eficácia do fio dental convencional com o Flosser na remoção do biofilme dental em crianças

“…Os autores Roomaney et al, (2021) e Quinlan (2016), concordam que o flosser é um fio dental eficaz, e capaz de promover uma adequação de meio, pois a maioria dos pacientes apresentaram resultados satisfatórios ao uso deste, em relação a boa remoção da placa interdental. É válido ressaltar que, não há indícios que apenas o fio dental seja suficiente para a higienização bucal, e por isso, é sempre aconselhável utilizar também a escova e creme dental.…”