Abnormalities in tooth morphology, structure and dentition in two children with chromosome aberrations. Translocation trisomy 13 and trisomy 21.

PURPOSE: Dental malformations due to chromosomal trisomies are rarely described and need an intensive cooperation between pediatricians, orthodonticians and human geneticists to enable the collection of data and to extend the investigations on specific parameters of the teeth. RESULTS: Here we present tooth studies of two children with trisomies 13 (Pätau-syndrome) and 21 (Down-syndrome): the dentition, the tooth morphology and the structure as well as the composition were investigated over a period of six years. Both male patients showed a delayed and abnormal dentition. Morphologic and structural changes compared to the general population were also detectable; whereas, the composition of the teeth was unchanged in enamel, dentin, and the border between them. CONCLUSIONS: The abnormalities in all parameters investigated were more pronounced in the patient with Pätau-syndrome than in the child with Down-syndrome.

[The effect of presurgical orthodontic treatment on the development of cleft children]. / Die Bedeutung der präoperativen kieferorthopädischen Behandlung für die Entwicklung des Spaltkindes.

The implications of immediate postnatal early orthodontic obturation for the development of cleft children and parental acceptance of the deformity are discussed and the practical procedure is explained.

[A solitary central maxillary incisor and holoprosencephaly in siblings]. / Ein solitärer mittlerer oberer Schneidezahn und Holoprosencephalie bei Geschwistern.

In families in which holoprosencephaly seems to be inherited by a rare dominant autosomal mutation, some individuals exhibit a single central maxillary incisor (SCMI). This anomaly was considered a minimal manifestation of the gene for holoprosencephaly. We report on a family, in which one child suffered from median cleft lip and palate and alobar holoprosencephaly, whereas the other child only had a SCMI. In the father and his sister lateral upper incisors were absent. Therefore the question is raised as to whether this anomaly may be related to the same condition.

[The eruption of deciduous teeth in children with various forms of Down's syndrome and congenital heart defects]. / Milchzahndurchbruch bei unterschiedlichen Formen des Down-Syndroms und kongenitalen Herzfehlern.

This study demonstrated that both congenital cardiovascular defects and the cytogenetic form of trisomy 21 have considerable effects on the chronology of the first dentition. The delay in the eruption of the deciduous teeth was even greater in children with congenital heart defects than in those with free trisomy 21. So-called mosaic-types of Down's syndrome, on the other hand, were associated with almost regular times of eruption.

[Early orthodontic treatment measures in infants with the EMG syndrome]. / Frühe kieferorthopädische Behandlungsmassnahmen bei Säuglingen mit EMG-Syndrom.

In 1963 Beckwith and Wiedemann reported a syndrome, which was characterized by umbilical hernia, macroglossia and somatic gigantism. In the literature, early intervention by tongue reduction is recommended in order to prevent both mandibular prognathism and open bite. In this paper seven cases were presented showing that early functional treatment of the stomatognatic system leads to pleasing results, both functionally and aesthetically. Therefore it is suggested, that functional treatment should replace surgical management of macroglossia in the Beckwith-Wiedemann-syndrome.

[Potentials of the interdisciplinary care of children with ectodermal dysplasia]. / Möglichkeiten der interdisziplinären Versorgung von Kindern mit Ektodermaldysplasie.

Children with ectodermal dysplasia represent a group of patients demanding highly sophisticated dental care. Characteristic morphological and functional oral problems lead to special technical difficulties and additionally, the altered psychology of these children requires empathy in treatment. With respect to these problems, children with ectodermal dysplasia are treated in our clinics, multidepartmental cooperation. The interplay between stimulative, restorative and prosthodontic therapy has been shown to be of great benefit for these children.

[Consultation for patients with cheilognathopalatoschisis and their family members. Cooperation between orthodontists and human geneticists]. / Die Beratung von Patienten mit Lippen-Kiefer-Gaumenspalten und ihren Familienangehörigen. Zusammenarbeit von Kieferorthopäden und Humangenetikern.

In children with a cleft lip and palate the question of aetiology always arises. This is the case in particular when the neonatologist discovers or suspects additional malformations. Since newborn children with cleft lip and palate are now usually brought for initial treatment to an orthodontist, it is his responsibility to inform the parents about the possibility and necessity of genetic counselling and possibly, to initiate contact with the appropriate institute. This is always the case when the wish for further children is expressed. If the orthodontist recognizes microsymptoms in the parents or finds indications of their occurrence in the family, he will strongly recommend such a consultation, with cleft lip and palate patients, the orthodontist should discuss a genetic consultation as part of his confidential relationship with the patient so that the young patients understand the possible risk for the next generation. This consultation can bring enormous psychological relief for the patient if there is only a slight risk of recurrence.

Sagittal craniostenosis, congenital heart disease, mental deficiency and various dysmorphies in two sibs--a "new" syndrome?

This is a report on two mentally retarded sibs with similar anomalies (sagittal craniosynostosis, complex cardiovascular malformations, mandibular ankylosis) and various dysmorphic features. They could represent a "new" probably monogenic syndrome.