Ehlers-Danlos Syndrome (Classic Type): Report of a Case Presenting with an Unusual Dental Anomaly

Abstract

Ehlers-Danlos syndrome (EDS) comprises a group of generalised connective tissue disorders. Deficiency or alteration of the collagen present in the tissues results in some classic signs such as skin hyper extensibility, joint hypermobility, and vascular fragility. Multiple supernumerary teeth, congenitally missing teeth, and odontogenic keratocysts have been reported in some patients with EDS. To the author's knowledge, transposition of permanent canines has not previously been reported in any case of EDS. This case report presents the dental findings of a sporadic case of classic-type EDS in a 14-year-old Chinese male who had transposition of a permanent maxillary canine as well as the presence of two supernumerary premolars in the mandible.

The role of the dental practitioner in diagnosing connective tissue and other systemic disorders: a case report of a patient diagnosed with type III Ehlers–Danlos syndrome

“…EDS could be as common as 1 in 5000 live births and affects males and females equally. The classic symptoms are skin hyperelasticity, joint hypermobility and vascular fragility. There are at least 15 phenotypes and six classifications of EDS; however, only types I–IV are discussed in this case report as the others are considered very rare with only a handful of cases being reported worldwide.…”

“…Some of the presenting oral symptoms of EDS as well as other general connective tissue disorders include dislocations of the TMJ joint, mucosal fragility, severe periodontitis leading to premature tooth loss, enamel and dentine defects, caries and supernumerary teeth.…”

Dental Manifestations of Ehlers-Danlos Syndromes: A Systematic Review

“…Two of 17 patients with cEDS showed 2–5 supernumerary teeth ( 42 , 45 ). None showed crown malformations, tooth discoloration or hypodontia, the latter was validated by inspection of the published orthopantomographs.…”

Prepubertal Periodontitis in a Patient with Combined Classical and Periodontal Ehlers–Danlos Syndrome

“…The usually cited reference for periodontitis in classical EDS is the paper by Pope et al (1992), where the authors describe three patients with defective dentinogenesis affecting the mandibular incisors [18]. Some similar cases were described [19,20]. In one of these individuals, two mandibular incisors with hypoplastic roots presented with severe periodontal breakdown [18].…”