Congenital anodontia with abnormities of the dermal system

Abstract

Summary.A case is described of a boy, aged 5, where X ray examination showed that only a singular dental «Anlage» was present in the right, upper jaw. Abnormities of the dermal system are observed, similar to those described in the few hitherto published cases in the litterature. The patient has a characteristic appearance with saddle nose and typical foetal hairgrowth exclusively localized to the face and the head. Sweat glands and sebaceous glands are lacking as proved by histologic examination of tissue excissions from the skin of the axilla and the scalp. Ozaena is pronounced. To a certain extent mental inferiority is possibly present.A syndrome or complexity of anomalies is present, expressing ectodermal abnormities. Organic abnormities are not observed, neither are signs of endocrine dysfunctioning. The patient has never gone through any disease of importance.The formative and causal genesis is discussed. Heridity is finally considered as the most probable causative factor in this congenital condition, although similar anomalies have not been demonstrated among the family members. These have unfortunately not been examined so thouroughly as desireable.The teratogenetic period of termination is thought to be about 9–10 embryonic week in the present case, as the primary germs of the sweat‐ and sebaceous glands are formed about this time; at the same time the dental lamina are formed in the primitive oral pit, from them the dental «Aniage» originate.The authors stress the relatively reduced development of the lower part of the skeleton of the face. They think that this phenomenon is due to the lack of teeth; the normal irritants for the development of the jaws are abscent and atrofy will result from inactivity. By X ray examination the structural bony arrangement characteristic of normal jaws, is not observed in the lower jaw, as trajectorium dentale is lacking. X ray examination shows furthermore that the upper corticalis plate in the frontal part of the lower jaw is partly resorbed, while the rest is smooth. The authors are of the opinion that this is due to the heavy pressure in this place during mastication. When roentgenologic measurements are performed on the patient and on a normally developed 5 years child, we find that the height of the corpus mandibula is markedly reduced on the patients roentgenogram. This reduction applies particularly to the part between foramen mentale and the upper margin of the lower jaw.The lack of teeth will also influence the physiognomi of the individual. The markedly compressed lower face is thought to originate in the lack of a fixed height of the bite, and this is also the cause of the «snoutlike» position of the lips.The macroglossus is regarded as compensatory hypertrophy, as the tongue, when the teeth are lacking, is involved in the act of mastication to quite another extent than in normal individuals. The authors can not share the opinion of certain american and english investigators that macroglossus should be an expression of a mongoloid condition.The lack of teeth must also be regarded as a concomitant cause of the poorly developed ability of speech.