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At least 109 records · Page 6Linked to original sources

Development of the dentition in cleidocranial dysplasia.

The purpose of the present investigation was to describe the formation, maturation and eruption of the dentition, including supernumerary teeth in a sample of patients with cleidocranial dysplasia. The dentition was evaluated from orthopantomograms, intraoral radiographs, cephalometric films, surgically removed teeth and intraoral photographs in 19 patients (9 men, 10 women), aged 3.5 to 34 years. Formation of primary teeth was normal, whereas all patients but one had supernumerary permanent teeth. Frequency of supernumerary teeth ranged from 22% in the maxillary incisor region to 5% in the molar regions. Supernumerary teeth were formed lingually and occlusally to the normal teeth. Maturation of the primary dentition was normal, while permanent teeth were delayed from 1 to 4 yr. Supernumerary teeth were delayed about 4 years in relation to normal permanent teeth. Eruption of primary teeth was normal, whereas all patients had severe eruption problems of permanent teeth. It was hypothesized that the dental lamina for both primary and permanent dentition is normal, but does not resolve completely and therefore may form supernumerary teeth. Abnormalities of tooth morphology is related to inadequate space and arrested eruption. Delayed or arrested eruption is probably caused by diminished resorption of bone and of primary teeth and to the presence of multiple supernumerary teeth.

Adolescent↗

Oral manifestations in Ellis-van Creveld syndrome: report of five cases.

Ellis-van Creveld syndrome, or chondroectodermal dysplasia, is an autosomal recessive disorder with characteristic clinical manifestations. Its incidence in the general population is low. The oral manifestations of Ellis-van Creveld are found in soft tissues and teeth, but the dental literature on the subject is scarce. In the last 20 years, 5 cases of Ellis-van Creveld syndrome have been followed at the Pediatric Dentistry Service of the Hospital Sant Joan de Déu, Barcelona. The present study describes the constant and variable oral findings in these patients, which play an important role in the diagnosis criteria for the syndrome. The presence of a great variety of oral manifestations such as fusion of the upper lip to the gingival margin, presence of multiple frenula, abnormally shaped and microdontic teeth, and congenitally missing teeth requires multidisciplinary dental treatment, with consideration for the high incidence of cardiac defects in these patients.

Anodontia↗

Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: brief review and role of scanning electron microscopy in diagnosis.

Uncombable hair syndrome was first described some 3 decades ago as "cheveux incoiffables" and is also known as spun-glass hair and pili trianguli et canaliculi. Both inherited (autosomal dominant and recessive with variable levels of penetrance) and sporadic forms of uncombable hair syndrome have been described, both being characterized by scalp hair that is impossible to comb due to the haphazard arrangement of the hair bundles. A characteristic morphologic feature of hair in this syndrome is a triangular to reniform to heart shape on cross-sections, and a groove, canal or flattening along the entire length of the hair in at least 50% of hairs examined by scanning electron microscopy. Most individuals are affected early in childhood and the hair takes on a spun-glass appearance with the hair becoming dry, curly, glossy, lighter in color, and progressively uncombable. Only the scalp hair is affected. Several conditions are associated with uncombable hair, such as ectodermal dysplasia, retinal dysplasia/pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia. Other syndromes with hair abnormalities may also mimic uncombable hair syndrome clinically and these include, Rapp-Hodgkin ectodermal dysplasia; loose anagen hair syndrome; ectodermal dysplasia, ectrodatyly, cleft lip/palate (EEC) syndrome; and familial tricho-odonto-onchyial ectodermal dysplasia with syndactyly. Unlike other conditions with an uncombable hair component, uncombable hair syndrome alone (cheveux incoiffables, pili trianguli et canaliculi) is not associated with physical, neurologic, or mental abnormalities. In most cases of uncombable hair syndrome, the hair is grossly abnormal in infancy and early childhood, but may have improved manageability later in life. Scanning electron microscopy of hair samples provides definitive evidence for diagnosis of clinically suspected uncombable hair syndrome and eliminates other hair abnormalities from the differential diagnosis.

Chemical Phenomena↗

Interobserver variability in radiographic interpretation of pediatric dental diseases: a pilot study.

Interobserver variability in radiographic interpretation of dental disease may be a result of clinical bias, education, training, experience or other factors, and may potentially lead to misdiagnosis of disease. Few studies have investigated variability related to dental specialty. The purpose of this pilot study was to determine interob-server variability in radiographic interpretation of pediatric dental diseases using a small number of observers, consisting of a pedodontist, an orthodontist and an oral radiologist. These specialists interpreted initial radiographic surveys of 200 pediatric patients (4.5 to 15.5 years old). The results showed no differences in interpretations of radiographic signs of dental abnormalities except for caries, abnormal primary root resorption due to the eruptive path of a permanent tooth and presence of a congenital/developmental tooth abnormality. These findings will be used to design future studies which will include a larger number of observers from different dental specialties.

Adolescent↗

Surgical exposure, orthodontic movement, and final tooth position as factors in periodontal breakdown of treated palatally impacted canines.

Twenty-three patients who had completed orthodontic treatment for the resolution of unilateral palatal canine impactions were examined 2.3 years (mean) after all appliances were discarded. The patients were divided into two groups on the basis of whether the surgical exposure was "light" or "heavy." The whole group was also divided according to the type of orthodontic movement that was carried out--"light" for tipping, extrusive, and rotating movements and "heavy" for root movements. Final position of the teeth was classified as ideal or incomplete if rotations or spaces were present. The results showed marked deteriorative changes where the surgery had been more radical and where the tooth movement involved active alteration of root position. No change due to abnormal tooth position was seen. It is suggested that, in these cases, surgical procedures be limited in scope and that exposure of the cementoenamel junction be avoided.

Alveolar Process↗

The blood-vessel thrust theory of tooth eruption and migration.

The Blood-Vessel Thrust Theory is a new hypothesis regarding the forces which produce the normal eruption of teeth, and the movement of 'nonerupted' teeth through bone away from their normal position in the jaws. It points out that the flow of blood through the vessels of the dental pulp, and of the tissues surrounding the tooth, must produce hydrodynamic and hydrostatic forces within the blood vessels, and that these forces have a resultant towards the tooth crown, thus causing the tooth to move, crown first, through the bone during normal eruption or abnormal tooth migration.

Humans↗

Dental phenotypes associated with novel PHEX variants in X-linked hypophosphatemia.

OBJECTIVES: X-linked hypophosphatemia (XLH) is a genetic disorder related to bone, mainly due to the mutations in PHEX gene. Previous studies have reported that XLH patients had various tooth phenotypes. It is unclear whether there are any rules about these abnormal tooth phenotypes, especially in those XLH cases with PHEX mutations. The objectives of this study were to find the most representative dental characteristics of XLH and the possible phenotype-genotype correlation. DESIGN: Two unrelated patients with XLH underwent clinical, radiographic, biochemical, and genetic evaluation. Whole-exome sequencing and whole-genome sequencing were used to identify pathogenic variants. The ultrastructure of extracted teeth was analyzed using a stereomicroscope, micro-CT, and scanning electron microscopy. In addition, a PubMed search (up to January 2026) identified 22 articles involving 366 patients for descriptive phenotype comparison. RESULTS: Two novel PHEX variants were identified: a novel complex structural variant (NC_000023.11, g.22035649-22041668delins) and a novel heterozygous splice-site variant (NM_000444.6, c.850-1 G>A). Radiographic examination showed enlarged pulp chambers and irregular pulp morphology. Ultrastructural analysis revealed dentin defects, including globular dentin, irregular interglobular dentin, disrupted dentinal tubules, and exposed collagen fibrils. Literature-based analysis indicated prevalent clinical manifestations (pulp necrosis, tooth loss, periodontitis) and radiographic findings (enlarged pulp chamber, and prominent pulp horn). CONCLUSION: In these two patients, novel PHEX variants were associated with a recurrent dentin-pulp phenotype. Integrated clinical, radiographic, ultrastructural, and literature evidence supports dentin defects as a central component of the dental phenotype in XLH and underscores the importance of early dental assessment.

Humans↗

Bizarre pathologic tooth migration.

An extreme example of pathologic tooth migration associated with bruxism and severe periodontitis is presented. The prognosis of the tooth was deemed hopeless; it subsequently was extracted. Various etiological factors of abnormal tooth displacement are discussed.

Bruxism↗

Vectored upper cervical manipulation for chronic sleep bruxism, headache, and cervical spine pain in a child.

OBJECTIVE: To discuss the management of chronic sleep bruxism in a 6-year old girl. Clinical features The patient had morning headaches and cervical spine pain. Due to abnormal tooth wear, bruxism had been previously diagnosed and was verified by observation during sleep. She also had abnormal postural and palpatory findings, indicating upper cervical joint dysfunction. Intervention and outcome Bilateral rotary cervical stretching/mobilization and a vectored high-velocity, low-amplitude adjustment were performed in the upper cervical spine, using the atlas transverse process as the contact point. There was complete relief of the chronic subjective symptoms concomitant with remission of the objective signs of joint dysfunction. CONCLUSIONS: Cervical, particularly upper cervical, spine muscle-joint dysfunction should be considered as a potential etiology in chronic childhood sleep bruxism.

Bruxism↗