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.

[Tongue disfunction and abnormal development]. / Dysfonctions linguales et dysmorphies.

Almost all the disorders seen in the mixed and adult dentitions were already present in the temporary dentition. We should ask ourselves what postural and dynamic role the tongue plays in defective development of the dental arches.

[Dynamic and postural changes in the tongue: influence on facial growth]. / Modificazioni dinamiche e posturali della lingua: influenza sulla crescita facciale.

The Authors examine the effect of the tongue's postural and dynamic modifications on facial grow. Some clinical cases are presented.

[Cauhépé-Fieux syndrome. Interception in the deciduous dentition of mid-face hypoplasia]. / A propos du "syndrome de Cauhépé-Fieux". L'interception en denture lactéale des hypoplasies de l'étage moyen.

Inadequate transverse development of the milk dentition alveolar arcade (Cauhepe-Fieux syndrome) may have important effects on the constitution of facial architecture and in the medium term require orthognathic surgery. The acquisition of dental arcade contact on deglutition is only possible after normalization of the dimensions of the arch of the palate. The recommended suture inducing prosthesis should obligatorily include intercalating molar plates designed to overcome inadequate occlusion during treatment (on average 9 months). Both stimulation of growth at the level of the intermaxillary suture in this age group, and the stability of the results obtained, have been demonstrated after a period of follow up of more than 10 years taking into account the cybernetic aspects of the studies carried out by Professor Petrovic.

[Mandibular angle and orthodontic treatment. Apropos of the possible therapeutic inversion of the direction of rotation of mandibular growth]. / Angle mandibulaire et traitement orthopédique. A propos de l'inversion thérapeutique possible du sens de la rotation de croissance mandibulaire.

The spontaneous and therapeutic modifications of the mandibular angle suggest that the rotations of growth described by Björk are not inevitable but are due to lingual dysfunction, particularly involving the pharyngeal part. Adequate treatment can modify the widening and narrowing of this angle.

[Facial profile--psychological profile]. / Profil facial--profil psychologique.

The morphological organisation of the face reflects the individual's social context and heredity and also reveal certain personality traits. Emotional facial expression is a very special form of communication. This functional and behavioural dynamic leads to the formation of a certain anatomical structure as a result of repetitive functions. The psychological examination of nineteen retrognathic patients and seven prognathic patients revealed the timid, inhibited of even anxious personality of the first group of patients in contrast with the extraverted and frequently aggressive personality of prognathic patients.

[Lingual vascular malformations, their consequences on the growth of the inferior facial plane. The integration of orthodontic embolization]. / Les malformations vasculaires linguales, leurs conséquences sur la croissance de l'étage inférieur. Le complément orthopédique de l'embolisation.

Lingual vascular malformations are usually benign, but have repercussions on the growth of the maxillo-mandibular unit in the child. The improvement in the vascular problems at puberty leads the authors to prefer a conservative form of treatment, hence the important role of embolization techniques. These techniques should be followed by the fitting of a lingual guide appliance, similar to the "mono-block" designed by Pierre Robin. This orthesis forces the child to modify incorrect movements due to the volume of the angiomatous tongue and gradually teaches him to automatize the new motor images created based on the dynamics of a virtually normal tongue, within the 6 months following embolization.

[Scanning radiographic evaluation of cleft lip and palate]. / Bilan scannographique dans les fentes labio-maxillaires.

The works of Paul Tessier, M.D., have shown the frequent association of facial and cranial clefts. After studying eleven cases of cleft palate which could be associated to encephalic anomalies, we conclude that we cannot systematize an evaluation by CAT-scan for low and isolated cleft palates. On the other hand, as soon as an association exists with: a psychomotor deficit (even slight); neurologic symptoms; a participation of the eye or the socket, the scanner evaluation becomes necessary.