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Incontinentia pigmenti: seven cases with dental manifestations.

Incontinentia Pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis that usually affects female infants. The condition is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. A large case series of seven patients, all female, who presented to the Department of Paediatric Dentistry at the Eastman Dental Hospital over the last 16 years is reported. The dental features of these cases were typical and included missing teeth, microdontia and delayed eruption. In two of the seven cases, both maxillary canines were palatally impacted.

Anodontia↗

Mandibular talon cusp: report of two rare cases.

Two rare cases of talon cusps in the mandibular incisors were observed during a prevalence study on talon cusp. One of them was seen on a mandibular primary lateral incisor. The other one was observed on the mandibular left permanent central incisor, and the right mandibular permanent central incisor was congenitally missing. There were no associated developmental syndromes with either of the cases reported. Of the 4770 children examined, nine cases had talon cusp with only two rare cases of mandibular talon cusp, one in the primary dentition and the other in the permanent dentition.

Adolescent↗

Surviving male with incontinentia pigmenti: a case report.

Incontinentia pigmenti, or Block-Sulzberger Syndrome, is an X-linked dominant disorder with characteristic skin, hair, eye and tooth abnormalities. It is classically considered a male-lethal disorder with recurrent miscarriages of male foetuses. A few cases of surviving males with incontinentia pigmenti have been reported in the medical literature. This article reports the medical and dental findings of a boy diagnosed with incontinentia pigmenti.

Anodontia↗

Meningococcal septicaemia and dental complications: a literature review and two case reports.

The present report describes two cases of dental complications of meningococcemia at an early age. The meningococcal infection in these young children resulted in limb amputation and significant plastic surgery. Dental treatment and psychological considerations are described in both cases. Similar dental complications, especially in the premaxilla, were found. Some of the severely affected teeth were extracted.

Bacteremia↗

Dental abscess in a tooth with intact dens evaginatus.

This article reports a case of dental abscess in a mandibular premolar with intact dens evaginatus. Dentists are advised to critically evaluate those teeth with dens evaginatus, both clinically and radiographically, before attempting prophylactic treatments. This is particularly important medicolegally in case the tooth develops symptoms shortly after the prophylactic treatment. Dentists practising in Western countries should also be aware of this dental anomaly because of the increasing global migration of people from Asia.

Asian People↗

A case of Coffin-Lowry syndrome with premature exfoliation of primary teeth.

We present a case of a 5-year-old boy with premature exfoliation of primary teeth. All eight primary incisors had exfoliated by the age of 3 years, and three canines and one primary first molar were subsequently lost when he was 4 years old. None of the exfoliated teeth exhibited caries. The boy also showed characteristic facial changes, tapering of the fingers, and mental and motor retardation. Based on these findings, he was diagnosed as having Coffin-Lowry syndrome. Premature exfoliation of primary teeth in Coffin-Lowry syndrome has been described in a few reports. This manifestation of the disease would be helpful for diagnosis at an early stage as those previous reports suggested.

Child, Preschool↗

Concomitant developmental dental anomalies in Chinese children with dens evaginatus.

AIM: To determine the prevalence of concomitant developmental dental anomalies in a group of Chinese children with dens evaginatus. METHODS: The dental records and orthopantomograms of 10-15 year old children and adolescents who had been diagnosed with dens evaginatus in a school dental clinic were reviewed retrospectively. RESULTS: Four hundred and forty-eight of 7102 (6.3%) children were found to have dens evaginatus. Concomitant developmental dental anomalies were found in 77 children (17.2%). The most commonly seen dental anomalies in the study population were hypodontia, hyperdontia, microdontic maxillary lateral incisor, and dental impaction. The prevalence of these anomalies did not differ significantly to that found in the general Chinese population. When analysed separately, however, the prevalence of supernumerary premolars was found to be higher in children with dens evaginatus than in the general population, and the difference was statistically significant (P < 0.01). CONCLUSION: Supernumerary premolars appeared to be more prevalent in Chinese children with dens evaginatus than in the general population. There may be an association of supernumerary premolars with dens evaginatus in this study population.

Adolescent↗

Anomalies of tooth formation in hypohidrotic ectodermal dysplasia.

OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study is the first to elaborate on anomalies of tooth formation found in a group of hemizygous males and heterozygous females with known ED1 mutations. These tooth anomalies may be used as dental biomarkers for heterozygous females, enabling an earlier diagnosis, and therefore, better treatment and genetic counselling. METHODS: Anomalies of tooth formation were examined using panoramic radiographs, dental casts and oral photographs in hemizygous males and heterozygous females who were identified by molecular genetic analysis. The results were compared to existing controls and normative data. RESULTS: All affected males had multiple missing permanent teeth and tooth malformations. The heterozygous females had a significantly higher frequency of agenesis of permanent teeth compared to normative data. The heterozygous females had an increased prevalence of tooth malformations and reduced tooth size, especially in the mesiodistal dimension. CONCLUSIONS: We conclude that observed anomalies of tooth formation may be used as dental biomarkers in the clinical identification of potentially heterozygous females.

Adolescent↗

Tricho-dento-osseous syndrome: a scanning electron microscopic analysis.

A large kindred of which multiple members have the Tricho-dento-osseous syndrome is presented. This is an autosomal dominant disorder characterized by defective enamel, taurodontia, unusually curly hair and occasionally mild to moderate skeletal osteosclerosis. Histologic investigation of teeth (by both LM and SEM) demonstrated that there is a uniformly thin enamel covering with randomly distributed depression and pits. The mineral content of this enamel is closer to that of the underlying dentin, which accounts for its lack of radiographic contrast. The dentin was normal. A bizarre finding is that of a periradicular sheath or membrane that enclosed the open apices and extended partway up the root. It was composed of collagen fiber bundles. The anatomical position of this membrane suggested that it may represent the developing peridontal ligament seen in early tooth formation. Recent embryologic evidence provides support for mesenchymal culpability for all reported features of the syndrome.

Adolescent↗

The Nance-Horan syndrome: a rare X-linked ocular-dental trait with expression in heterozygous females.

This report describes two families with the Nance-Horan syndrome, an X-linked trait featuring lenticular cataracts and anomalies of tooth shape and number. Previous reports have described blindness in affected males but posterior sutural cataracts with normal vision as the primary ocular expression in heterozygous females. In one of these two families, the affected female is not only blind in one eye but reportedly had supernumerary central incisors (mesiodens) removed. This constitutes the most severe ocular and dental expression of this gene in heterozygous females yet reported.

Adult↗

Analyzing the etiology of an extremely worn dentition.

Patients requiring extensive restorative care frequently exhibit significant loss of tooth structure. Specific clinical findings in an extremely worn dentition may vary widely and are often confusing. Severe wear can result from a mechanical cause, a chemical cause, or a combination of causes. The location of the wear, the accompanying symptoms and signs, and information gained from the patient interview are essential components in determining the etiology. A diagnostic decision tree facilitates a systematic analysis and diagnosis of dental wear.

Decision Trees↗

Studies on root enamel (2). Enamel pearls. A review of their morphology, localization, nomenclature, occurrence, classification, histogenesis and incidence.

Enamel pearls are one of a number of different enamel structures that can be found on the roots of deciduous and permanent teeth. They have a distinct predilection for the furcation areas of molar teeth, particularly the maxillary third and second molars. They can consist primarily of enamel, but in most instances, a core of dentin is contained within them. On rare occasions, even pulpal tissues can be found. Enamel pearls usually occur singularly, but up to 4 enamel pearls have been observed on the same tooth. Depending on the study, enamel pearls on permanent molar teeth have an incidence rate of between 1.1%-9.7% with distinct differences among racial and national groups. The incidence of enamel pearls increases greatly in histological studies, suggesting that they are often obscured by a covering of cementum.

Ameloblasts↗

The morphology of root fusion in Chinese adults (I). Grades, types, location and distribution.

The purpose of the present study was to investigate the morphology, extent, and distribution of root fusions in 1st and 2nd permanent molars in a Chinese population. The sample consisted of 158 maxillary and 151 mandibular extracted 1st and 2nd molars. Root fusion in molars was assessed by direct viewing and measuring the length of the root and root trunk with stereoscopic microscopy. Root fusion was graded by the extent of fusion and the number of affected surfaces. The main results were as follows: (1) the majority of root fusions affected upper and lower 2nd molars (39.7% and 28.1%, respectively); (2) the prevalence of maxillary roots with 1, 2 and 3 fusions was 55.2%, 31.3% and 13.4% respectively, and most of the root fusions affected the maxillary 2nd molars (65.7%); (3) the majority of mandibular root fusions affected the 2nd molars (89.2%), and were of grade III (48.7%); (4) in order of decreased prevalence, the grades of root fusion in 2nd molars were grades III, II and I; whereas in 1st molars they were grades I, II and III; (5) there were 44 (41.5%) and 19 (51.4%) complete root fusions (grade III) among 106 fused roots in maxillary molars and 37 fused roots in mandibular molars, respectively.

Adult↗

Prevalance of malocclusion in young Finnish Skolt-Lapps.

Two hundred Skolt Lapps aged 8 to 16 years were examined for malocclusion with the aid of intraoral radiography and dental casts. The frequency of loss of permanent teeth was high, particularly of the first molars. Also, many teeth were severely defective because of caries. Hypodontia was very common. The frequency of tipped and rotated teeth was high, probably as a consequence of the frequent loss of neighboring teeth. Space and occlusal anomalies were roughly as common as in urbanized Swedish and Danish materials. The need for orthodontic treatment, judged on a 5-grade scale by one examiner, was found to be greater than in Swedish materials.

Adolescent↗

Epidemiologic study of joined primary teeth in Finnish children.

The frequency of germinated and fused primary teeth was studied in a sample of 1141 Finnish children aged 3-4 years. The prevalence was 0.7%. All the joined teeth were found in the anterior area of the mouth. In seven cases the diagnosis was a fusion of two separate teeth, and in one case it was a germination of a single tooth. A correlation between joined teeth in primary dentition and numerical variations of teeth in permanent dentition was noted in three cases.

Child, Preschool↗

Panoramic radiographic examination of 704 Danish children aged 9--10 years.

Panoramic radiographs of 704 apparently health children aged 9--10 years were examined regarding anomalies and pathologic conditions in the maxillofacial area. Almost all findings were mesial to the molars. 1.7% of the children had a supernumerary tooth, 7.7% had hypodontia. Malposition of 631 permanent teeth was recorded. 42 dentigerous cysts were detected. Malformed crown or root was seen on 60 permanent teeth. Caries was found on 224 primary and 32 permanent teeth. 257 primary teeth showed atypical resorption, and 53 had a periapical inflammatory radiolucency. 20 retained roots of primary teeth were detected. 31 maxillary sinuses had an opacity. There were very few other findings.

Child↗

Prevalence of selected dental anomalies in Saudi children from Gizan region.

The present report gives prevalence values for a selected group of developmental dental anomalies in 2393 Saudi children aged 4-12 yr from Gizan region, Saudi Arabia. The anomalies selected in this study were those involving the number and shape of the dentition. The following anomalies were identified: congenitally missing teeth (2.2%), supernumerary teeth (0.50%), gemination (0.08%), and peg-shaped lateral incisors (0.37%).

Anodontia↗

Prevalence and distribution of developmental enamel defects in primary dentition of Chinese children 3-5 years old.

A total of 1344 children, 3-5 yr old, from two rural counties, Haidian and Miyun, close to Beijing, China, were examined in 1992. A modified DDE Index was employed in this study, and a pre-designed formula was used to calculate an enamel defect score (EDS) for each individual in the study. Oral examination was performed by one dentist under natural light using a standard mouth mirror and dental probe. Developmental enamel lesions were diagnosed without drying or cleaning the teeth prior to examination. Results from this study showed that primary teeth with defective enamel were seen in 23.9% of the children examined, opacity in 1.6%, and hypoplasia in 22.2%. Among the teeth, maxillary central and lateral incisors were affected by enamel hypoplasia most often (40.8% and 39.2%), followed by maxillary canines (25.7%), maxillary 1st molars (22.1%), and mandibular 1st molars (18.5%). The enamel defects occurred more frequently on the buccal surfaces of teeth than on any other surface. The study did not find a significant association of the children's age, family socioeconomic status, and anthropometric measurements with the distribution of enamel defects. However, there was a significantly higher prevalence of teeth with defects in males compared to females (P < 0.001), as well as mean tooth surfaces with defects (P < 0.05), and mean EDS (P < 0.05). Children born prematurely were shown to have four times more enamel lesions than children who were full term (P < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗