Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of six patients.

PURPOSE: When localised scleroderma occurs in the face, neck and scalp area, it is called scleroderma en coup de sabre (SCS) for its resemblance to the stroke of a sabre. Most observed characteristics: abnormal skin and dental development, facial atrophy and neurological complications. The aim was to evaluate the extent of SCS in the underlying subcutis, including teeth/bone tissues. The goal was to solve, how far the external visual skin abnormality extends in depth, and if the condition appears within and limited to craniofacial neural crest fields. METHODS: Photographic and radiographic materials from six patients (one male, five females, aged 5-39 years) were included. The cases were divided in three groups, two in each, according to similarity in location of SCS in the skin. Dentition and gingiva were analysed clinically and from intra-oral photos, dental radiographs and orthopantomograms. Agenesis, dental maturity stage (root length), deviation in crown and root morphology (size and shape), and eruption disturbances were registered. Profile and frontal radiographs were analysed cephalometrically for jaw relationships and bone structures. RESULTS: In SCS, skin affection corresponds to the neural crest regions/fields. A close spatial association between skin, teeth and bone affections within neural crest fields was found. No common traits in profile analyses were observed. Asymmetry from minor to severe appears in the frontal analyses. A malformation in planum sphenoidale was observed in two individuals with the same location of skin affections. CONCLUSION: SCS conditions seem to extend from the skin in the depth to the sella turcica area within neural crest fields.

Primary failure of eruption of teeth in two siblings with a novel mutation in the PTH1R gene.

BACKGROUND: Primary failure of tooth eruption (PFE) is a rare non-syndromic disorder with prematurely ceased eruption of the posterior teeth, despite clearance by bone resorption of the eruption path. It is generally agreed that most of the impacted teeth are second molars that are deeply seated, and without symptoms. Traditionally, patients with failure of tooth eruption undergo surgical and/or orthodontic treatment. However, patients with PTH1R mutations have no beneficial effect of such a regime and PFE is therefore important to diagnose. CASE REPORT AND FOLLOW-UP: A family with three PFE affected members in two generations, involving both the primary and permanent dentitions, and a novel mutation in the PTH1R gene are reported. Furthermore, the treatment of the eruption failure was documented in one of the cases. CONCLUSION: In the present study, the proband initially only had a minor clinical problem, lack of eruption of the primary second left mandibular molar. However, over time several problems appeared in the permanent dentition. Clinical signs of PFE should lead one to look for similar dental problems in related family members and to molecular DNA testing. Confirmation of the diagnosis PFE in young children has the advantage that unnecessary treatment can be avoided, since early orthodontic intervention for these patients is futile. Once growth is complete, several multidisciplinary treatment strategies can partially solve the posterior open bite malocclusion that is characteristic of this disorder. Treatment should be planned in cooperation with specialists who are used to treating PFE patients.

Upper cervical spine and craniofacial morphology in hypohidrotic ectodermal dysplasia.

AIM: Morphological deviations in the upper cervical spine and craniofacial morphology in patients with X-linked hypohidrotic ectodermal dysplasia (XLHED) were compared to non-syndromic controls. METHODS: All children and adolescents with genetically verified XLHED, registered at the Resource Centres for Oral Health in Rare Diseases, who met the inclusion criteria, were included. The group thus comprised 15 XLHED patients (3 girls and 12 boys, aged 8-16 years, mean 11.2 years). The control group comprised 22 non-syndromic pre-orthodontic children (14 girls and 8 boys aged 9-16 years, mean 11.9 years) with agenesis of one tooth, neutral occlusion and normal craniofacial morphology. The craniofacial and upper spine morphology was analysed on lateral cephalograms by standard methods. Differences between XLHED patients and controls were tested and adjusted for age and gender by multiple regression analyses. RESULTS: Morphological deviations in the upper spine occur significantly more often in XLHED patients compared to controls (60 vs. 9.1%; p < 0.01). The cranial base angle (n-s-ar, p < 0.05), sagittal jaw relationship (ss-n-pg, p < 0.001), maxillary inclination (NSL/NL, p < 0.001) and mandibular inclination (NSL/ML, p < 0.01) were significantly smaller in XLHED patients compared to controls. The mandibular prognathia (s-n-pg) was significantly larger in XLHED patients compared to controls (p < 0.05). CONCLUSIONS: The upper spine and the craniofacial morphology were different in XLHED patients compared to controls. The results of this study may contribute to a further understanding of the craniofacial and spinal phenotypic spectrum in patients with XLHED and thus have implications for diagnosis and treatment planning of these patients.

Linear scleroderma en coup de sabre including abnormal dental development.

BACKGROUND: Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknown. A paediatric dentist may be the first medical person to identify SCS by its involvement in dentition. It is assumed that the malformation of a dentition could be associated with the severity of the skin deviation. CASE REPORT: A 6-year and 10-month-old Turkish girl with a history of SCS was referred for dental diagnostics and treatment. The SCS skin lesion affected the left side of her hairline over the forehead and nose, involving the left orbit proceeding towards the left oral region. Dental clinical/radiographic examination revealed malformed left maxillary incisors with short roots and lack of eruption. FOLLOW-UP: The patient has been regularly controlled and treated since she was first diagnosed. A surgical and orthodontic treatment was performed to ensure optimal occlusion, space and alveolar bone development. The present age of the patient is 14 years and 10 months. CONCLUSION: This case demonstrated a patient with a left-sided skin defect (SCS) and a left-sided local malformation in her dentition. It is possible that there is a developmental connection between these two left-sided defects, both with an ectodermal origin.

Interrelation between fusions in the primary dentition and agenesis in the succedaneous permanent dentition seen from an embryological point of view.

The purpose of the present study was to elucidate the relationship between fusions in the primary dentition and the occurrence of agenesis in the succedaneous permanent dentition in a Danish child population and to elucidate this relationship from the recently described normal embryological development of the anterior parts of the human maxilla and mandible. The material included radiographs, either as intraoral film or as orthopantomograms from a total of 19 primary dentitions with a total of 21 fusions. Radiographs of the permanent dentition in the fusion regions were available for all 19 dentitions. Of 21 fusions, a total of 20 were in the mandible and one in the maxilla. In 15 cases, the fusions were between primary incisors and in six cases between lateral incisors and canines. Agenesis of a permanent lateral incisor always occurred when there had been fusion of a primary lateral incisor and a canine in the primary dentition. When fusion had been between primary incisors, there was only agenesis of an incisor in the permanent dentition in a few cases. The degree of fusion between the involved teeth was not related to the occurrence of agenesis. It is suggested that the intra-jaw differences are related to the recently reported prenatal developmental patterns of the alveoli of the incisors and canines. Moreover, it is suggested that neural crest developmental field differences between the developing maxilla and mandible may explain the inter-jaw differences in phenotypic abnormalities.

Pattern of agenesis in the primary dentition: a radiographic study of 193 cases.

The purpose of this study was to examine radiographs of a large sample of children who had congenital absence of primary teeth and to determine the number and distribution of the missing teeth. Radiographs collected within the Danish Municipal Child Oral Health Care System were available of 193 children, all of whom had congenital absence of one or more primary teeth but no other abnormalities in the jaws or dentition. More than half of the children (54.9%) had agenesis of only one primary tooth, and 7.8% of more than two primary teeth. Agenesis was found twice as frequently in the maxillary lateral incisor region (119 children) than in the mandibular lateral incisor region (53 children). Congenital absence of primary molars, canines and maxillary central incisors was extremely rare. However, agenesis of one maxillary primary central incisor was found in two cases. A follow-up study will compare the agenesis patterns recorded with the pattern of agenesis in the permanent dentition of the same group of children.

Comparison of the pattern of agenesis in the primary and permanent dentitions in a population characterized by agenesis in the primary dentition.

The first aim of this study was to collect a large sample of radiographs from children with congenitally missing teeth (CMT) in the primary dentition and to analyse the local relationship between agenesis of a primary tooth and the presence/absence of its permanent successor. The second aim was to compare, in the same sample, the pattern of agenesis in the primary dentition with the developmental pattern seen in the permanent dentition. 124 dentists from 72 municipalities contributed to the investigation of a total of 213 children. The dentists were asked to lend existing radiographic material from patients with agenesis in the primary dentition. The analysis of the local occurrence of agenesis showed that agenesis of a primary incisor was often but not always followed by agenesis of the succedaneous tooth. In the molar region, agenesis of a primary tooth was in all cases but one followed by agenesis of the succedaneous tooth. Comparison of the pattern of CMT in the primary dentition with the pattern of tooth presence/absence in the permanent dentition in a group of 33 patients, for whom complete radiographic material was available showed that agenesis always occurred in the permanent dentition and that the pattern of agenesis in the permanent dentition differed from that in the primary dentition. Incisors were most frequently missing in the primary dentition and premolars in the permanent dentition. The number of congenitally missing teeth was substantially larger in the permanent dentition that in the primary dentition. Also, permanent teeth that are very rarely congenitally missing were missing in this sample, characterized by the occurrence of agenesis in the primary dentition.

Palate structure in human holoprosencephaly correlates with the facial malformation and demonstrates a new palatal developmental field.

In this study we analyzed palate structure in holoprosencephaly and correlated it with the facial malformations. Eleven human holoprosencephalic fetuses (three cyclopic, two ethmocephalic, one cebocephalic, four with median cleft lip, and one with short philtrum) at 17-23 weeks of gestation and three children (age 2 1/2, 6 and 7 years) with a single central incisor were studied. Photographic and radiographic methods were used. We found that in holoprosencephaly palate structure is abnormal. The severity of this malformation decreases with decreasing severity of facial malformation. Thus, the study shows a close relationship between the facial and the palatal malformation. In all phenotypes the premaxillary area is malformed. From this region, a fan-shaped field along the midpalatal suture is involved in all facial phenotypes, the fan being broadest in cyclopia and narrowest in the short philtrum malformation. A similar fan-shaped field can be discerned in the face, where the broadest fan also indicates the greatest severity with cyclopia, and the narrowest fan the least severe median lip malformation. In the palate field, the anteroposterior furrows seemingly demarcate the field. The findings may be of importance for the future evaluation of palatal malformations in children.

Treatment of traumatic dental injuries in children.

The major emphasis of this review should rest on articles written within the past 12 months. Many of these papers comprise comprehensive surveys of treatment of various aspects of dental trauma in the primary and permanent dentition, as well as epidemiological studies. There have also been some very good experimental studies which have attempted to standardize luxation injuries as well as tooth replantation in order to study pulpal and periodontal healing in reproducible animal models. However, it can be seen from the reference list that one year's production in dental traumatology would not suffice to cover the scope of the treatment needs nor to adequately orient the reader on the progress made with respect to our present knowledge of wound healing following injury and the most recent developments in the restoration of the traumatized dentition. To present an overall view of the philosophy which has evolved concerning wound healing in the dental pulp and periodontium following injury as well as innovations in the treatment of acute dental trauma, this review must of necessity delve back into the mid-80's and probe forward into publications which are on the way in 1991. In 1984, in Dallas, Texas, the American Association of Endodontists' Endowment and Memorial Foundation convened the first International Conference on Oral Trauma. In 1989, in Stockholm, Sweden, this was followed up with the second International Conference on Dental Trauma. The one-day program summarized the state of the art of dental traumatology with respect to various aspects of epidemiology, diagnosis, treatment and prevention as well as the biological principles which form a basis for our present knowledge about healing and healing complications in the pulp and periodontium after traumatic dental injuries. The proceedings from this conference have been compiled in a newly published volume. Where applicable, these presentations have been cited as current reviews. It could also be mentioned that at the conclusion of the second conference, in recognition of the need to promote research and propagate knowledge in the area of dental traumatology, the International Association of Dental Trauma (IADT) was founded. The third International Conference on Dental Trauma was convened in Copenhagen, Denmark, June 1991. The major theme was restoration of the traumatized dentition. Proceedings from this conference are to be published. However, new information from that program is also cited in the following where applicable. Finally, the second Charlotte Conference on Pathobiology of the Dentin/Pulp Complex was held in Charlotte, North Carolina in May 1991.(ABSTRACT TRUNCATED AT 400 WORDS)

Treatment of crown fractured incisors with laminate veneer restorations. An experimental study.

A method is described by which crown fractured incisors are restored with cast ceramic (Dicor) laminate veneers after initial treatment with either reattachment of the original crown fragment with a dentin bonding agent, with a composite resin build-up or no treatment (i.e. the veneer alone is used to restore the incisal edge). In order to elucidate the effect of the fragment/composite-tooth bonding interface on fracture strength of the restored teeth, the fracture strengths of the various treatment groups were compared to that of intact teeth supplied with Dicor laminate veneers. In an experimental investigation using central and lateral incisors from sheep, it was found that fracture strength (16.6 +/- 4.2 MPa) equal to that of intact incisors (16.1 +/- 2.6 MPa) could be achieved using laminate veneers made of porcelain on fractured teeth whose crown fragments were reattached using a dentin bonding agent (5). In the present investigation, using the same experimental model but using cast ceramic (Dicor) laminate veneers, the fracture strength of the restored incisors was significantly increased (21.0 +/- 3.7 MPa), exceeding that of intact teeth. The fracture strength of intact teeth was also exceeded in veneered incisors which were initially restored with a conventional composite resin build-up (20.2 +/- 5.6 MPa). However, the greatest fracture strength (28.2 +/- 8.9 MPa) was achieved when a Dicor laminate veneer alone was used to restore the fractured incisal edge.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of traumatic dental injuries in children.

The major emphasis of this review rests on articles written within the past 12 months. Many of these papers comprise comprehensive surveys of treatment of various aspects of dental trauma in the primary and permanent dentition, as well as epidemiologic studies. There have also been some very good experimental studies that have attempted to standardize luxation injuries as well as tooth replantation in order to study pulpal and periodontal healing in reproducible animal models. However, it can been seen from the reference list that 1 year's production in dental traumatology would not suffice to cover the scope of the treatment needs nor to adequately orient the reader to the progress made with respect to our present knowledge of wound healing following injury and the most recent developments in the restoration of the traumatized dentition. To present an overall view of the philosophy that has evolved concerning wound healing in the dental pulp and periodontium following injury as well as innovations in the treatment of acute dental trauma, this review must of necessity delve back into the mid-1980s and probe forward into publications that are on the way in 1991.

Reinforcement of bonded crown fractured incisors with porcelain veneers.

A method is described by which porcelain laminate veneers are used to reinforce crown-fractured incisors which have been restored by reattachment of enamel-dentin fragments using enamel etching and a dentin bonding system. In an experimental model using sheep incisors, it was found that fracture strength equal to that of intact incisors could be achieved by employing this method. This is in contrast to fracture strengths of reattached enamel-dentin tooth fragments without porcelain laminates which were only 50% of intact incisors. It is suggested that porcelain laminate veneers may be used to supplement fragment bonding, thereby enhancing dental esthetics and function.