Syndrome of Hajdu-Cheney: three case reports of orofacial interest.
Hajdu-Cheney syndrome is a rare, probably autosomal dominant connective tissue disorder with a variable expressivity. It is characterized by an osteoporotic skeleton, acro-osteolysis, a proportionate short stature, and distinctive orofacial anomalies. The aim of this article is to focus on the orofacial manifestations in two sporadic cases and one familial case with Hajdu-Cheney syndrome. Several common dental and craniofacial features are described. In contrast to earlier proposed diagnostic features, these patients show persisting deciduous teeth, problematic tooth eruption, and tendency toward a Class III malocclusion.
Vingerhoedt
E
E
Department of Orthodontics, School of Dentistry, Oral Pathology and Maxillo-Facial Surgery, Leuven, Belgium. elisa_vingerhoedt@hotmail.com
Bailleul-Forestier
I
I
Fellus
P
P
Schoenaers
J
J
Frijns
J-P
JP
Carels
C
C
D002508:Cephalometry; D002648:Child; D005147:Facial Bones / Q000002:abnormalities*; D019066:Facies; D005260:Female; D031845:Hajdu-Cheney Syndrome / Q000473:pathology*; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000209:etiology* / Q000628:therapy; D008313:Malocclusion, Angle Class III / Q000209:etiology; D008334:Mandible / Q000002:abnormalities; D063173:Retrognathia / Q000209:etiology; D014071:Tooth Abnormalities / Q000209:etiology*; D014097:Tooth, Unerupted / Q000601:surgery