[Case report on the Ehlers-Danlos syndrome].
The Ehlers-Danlos syndrome is a hereditary disease of the connective tissue, characterized by tendencies of hyperextensibility and fragility of the skin, and hypermobility of the joints. We are reporting in this paper on a 4 year, 4 month old boy with the Ehlers-Danlos syndrome, Type III. The following findings were obtained: 1) In addition to the three main symptoms the complications of exotropia, low position of the auricle, defect of the right musculuspectoralis major, right double thumbs, dislocation of hip joint, right inguinal hernia, right undescended testis and pes planovalgus were found. 2) Based on histopathological tissue examination, hypoplasia of the collagen fibers and the elastic fibers was found. 3) The occlusion was edge to edge, the plate was high arched and the upper and the lower dental arches were contracted. 4) The mesiodistal lengths of all primary teeth and the lengths and widths of the dental arches were smaller than the standard size. 5) The orthopantomo and dental x-rays revealed a tendency toward late calcification of the deciduous teeth and permanent teeth germs, and the stricture of the root canals. 6) According to x-ray cephalometric analysis, retardation of the growth of the upper face as well as the maxilla and mandibula was found.
Fukao
T
T
Yanagida
E
E
Funakoshi
Y
Y
Shimoda
Y
Y
Kawamura
H
H
Hieda
T
T
D002675:Child, Preschool; D003768:Dental Occlusion, Centric; D004535:Ehlers-Danlos Syndrome; D006801:Humans; D008297:Male; D008445:Maxillofacial Development