Dental anomalies in the primary dentition and their repetition in the permanent dentition: a diagnostic performance study

This study investigated the recurrence in the permanent dentition of dental anomalies of the primary dentition. A sample of 189 subjects (100 males, 89 females, mean age of 5 years and 7 months) with anomalies of primary teeth (tooth hypodontia, supernumerary teeth, geminated teeth, and fused teeth) was selected and re-analyzed at a mean age of 11 years and 2 months for the recurrence of the dental anomalies in the permanent dentition. As a control group, 271 subjects (123 males, 148 females) without dental anomalies in the primary dentition were selected. The recurrence in the permanent dentition of the dental anomalies in the primary dentition was evaluated by measures of diagnostic performance. The results showed high values for the repetition of hypodontia (positive likelihood ratio = 102.0); low score for the repetition of hyperdontia (positive likelihood ratio = 6.5); low positive likelihood ratio (9.1) for gemination of primary teeth resulting in supernumerary permanent teeth; high positive likelihood ratio (47.0) for fusion of primary teeth followed by missing permanent teeth. Dental anomalies in the primary dentition are associated with an increased likelihood of anomalies of the succedaneous permanent.     

Dental anomalies and associated factors in 2- to 5-year-old Brazilian children

Background. Dental anomalies in primary teeth may lead to functional and aesthetic disorders, and their recognition contributes to early diagnosis and long-term treatment planning. Objective. This study investigated the prevalence of dental anomalies in primary dentition and associated factors in Brazilian preschoolers. Methods. The study population of this cross-sectional study comprised 1260 2- to 5-year-old children from public nurseries in Canoas, southern Brazil. Dental anomalies were recorded by five trained examiners according to Kreiborg criteria; classification included double teeth, hypodontia, supernumerary, and microdontia. Results. Dental anomalies as a group were found in 2.5% of children, although no significant difference occurred between genders and races. All the anomalies were observed in the anterior region, with no significant differences between the arches. However, supernumerary teeth were significantly more frequent among the non-white racial group (Fisher; P = 0.025) and double teeth on the lower arch (McNemar; P = 0.020). Individual anomaly frequencies were: double teeth, 1.3%; hypodontia, 0.6%; supernumerary, 0.3%; and microdontia, 0.3%. Conclusions. The frequency of primary dentition anomalies as a group was greater than that reported in other populations; the findings of this study provide a clear vision of the distribution of this oral condition and may well contribute to early detection and treatment planning.     

Epidemiology and genetics of hypodontia and microdontia: A study of twin families

Objective:To identify genetic and environmental factors contributing to hypodontia and microdontia by using Korean twin family data.Materials and Methods:A total of 1267 individuals (525 men and 742 women; 180 monozygotic twins [MZ] and 43 dizygotic twins [DZ] from 282 families) underwent an oral examination as part of the Healthy Twin Study in Korea. Dental anomalies classified as hypodontia or microdontia were diagnosed using radiographs and clinical examinations. In order to estimate genetic contributions to dental anomalies, we estimated the pairwise concordance rate (PCR), recurrence risk ratio (RRR), and heritability (h2).Results:The prevalence of hypodontia and microdontia was 3.55% and 3.00%, respectively. MZ had the highest PCR and RRR (13.0–15.3). The PCR and RRR values for both anomalies were much higher for DZ (5.0–11.9) than for siblings (1.4–2.6), despite the fact that DZ pairs and sibling pairs share 50% genetic identity. Further genetic analysis revealed both an additive genetic effect (0.38 when hypodontia and microdontia were pooled) and a strong “twin effect” (0.52 when hypodontia and microdontia were pooled).Conclusions:This twin-based study revealed that the formation of dental anomalies is affected by both genetic and environmental factors, and that the impact of these factors varies according to the specific dental anomaly.     

Prevalence and distribution of anomalies of permanent dentition in 9584 Japanese high school students

This study investigated the prevalence and distribution of anomalies of permanent dentition in the current Japanese population by examining an unbiased sample. We conducted a survey of dental anomalies by mass dental screening at eight high schools in 2012. Participants were all students with permanent dentition. Dental anomalies were classified as hypodontia, supernumerary teeth, peg-shaped teeth, fused teeth, and talon cusps. Students with one or more dental anomalies on oral examination were given a differential diagnosis by three specialists. The final sample comprised 9584 participants (5062 boys, 4522 girls). Hypodontia was present in 372 students (3.88 %) with no significant sex difference (191 boys, 181 girls). Frequent sites were the right or left mandibular second premolar, right or left maxillary second premolar, and right or left maxillary lateral incisor. Supernumerary teeth were observed in three boys (0.06 %) and one girl (0.02 %). Peg-shaped teeth were observed in 74 students (0.77 %; 27 boys, 47 girls), differing significantly between sexes; they were most prevalent among maxillary lateral incisors. Of affected students, 18 students (0.19 %) also had hypodontia (3 boys, 15 girls). Fused teeth were present in two boys (0.04 %) and three girls (0.07 %) (gemination in one boy and fusion in the remaining four students). Sites were limited to maxillary and mandibular central and lateral incisors. Talon cusps were observed in two boys (0.04 %) and four girls (0.09 %). The present survey of a large unbiased sample can be considered to reflect the prevalence and distribution of anomalies of permanent dentition in the current Japanese population.     

Dental findings of a child with Wolf-Hirschhorn syndrome

The case presented is that of a 5-year-old female with Wolf-Hirschhorn syndrome. Dental findings were severe hypodontia, late dental development, taurodontism of the primary molars, microdontia, and spacing. Hypodontia has previously been reported and therefore this case adds to the evidence that hypodontia may be a common feature of this syndrome. It also suggests that other dental anomalies could occur in children with Wolf-Hirschhorn syndrome.     

Pseudohypoparathyroidism: case report

A patient with pseudohypoparathyroidism is presented. The phenotypic appearance, known as Albright heredity osteodystrophy includes: short stature, round face, brachydactylia, and ectopic calcifications in the soft tissues. Dental manifestations reported in the literature are enamel hypoplasia, hypodontia, malformed roots, enlarged pulp chambers, microdontia, and pulp calcifications. Additional findings in this case are ankylosis and an enlarged frontal sinus. The delayed diagnosis of pseudohypoparathyroidism with the early presentation of multiple dental anomalies is discussed.     

Kabuki Syndrome with additional dental findings: a case report

Kabuki Syndrome (KS) is a multiple congenital anomalies/mental retardation syndrome of unknown etiology. It is characterized by a dysmorphic face, postnatal growth retardation, skeletal abnormalities, mental retardation, and unusual dermatoglyphic patterns. The characteristic dental findings include hypodontia, microdontia, absence of upper lateral and lower central incisors and upper molars, abnormal tooth shape, widely spaced teeth, ectopic upper molars and malocclusion. The purpose of this report is to describe common and additional dental findings and dental treatment of an 11-year old female patient with KS. This case report emphasises the importance of oral and dental manifestations for diagnosis.     

Hurler syndrome: a case report of a 5-year follow-up of dental findings after bone marrow transplantation

Hurler syndrome is a rare autosomal recessive disorder of mucopolysaccharide metabolism. It results from a deficiency in lysosomal enzymes responsible for the breakdown of glycosaminoglycans. Affected individuals may show progressive physical and mental deterioration as glycosaminoglycans are deposited in the organs of the body. Bone marrow transplantation (BMT) is effective in improving some of the clinical manifestations of Hurler syndrome. Death is caused by cardiorespiratory failure and usually occurs before the second decade of life.
In this case report, the course of dental development was followed over 5 years, from the primary dentition into the permanent dentition, of a child who was successfully treated with a bone marrow transplant in infancy. The timing of bone marrow therapy has significant and variable effect on the stages of tooth development with implications for the long-term maintenance of the dentition.     

乌鲁木齐地区青少年恒牙形态及数目异常的分析

目的探讨乌鲁木齐地区青少年先天缺失牙、多生牙、过小牙的发生率及好发部位。方法本文观察和分析了620例患者的全颌曲面体层X线片．均无恒牙拔牙史或牙齿损伤史。结果先天缺失牙（包括第三磨牙）的发生率为45．48％,第三磨牙的缺失发生率为32．58％,缺失牙的好发部位依次为上颌第三磨牙、下颌第三磨牙、下颌侧切牙、下颌中切牙、上颌第二双尖牙等;多生牙发生率为2．58％,好发部位是上颌切牙区。过小牙的发生率为6．30％．其主要为上颌侧切牙。结论牙齿先天缺失在人群中的发生率明显高于多生牙．缺失牙主要发生在功能相对弱的牙位上：缺失牙与过小牙之间存在一定联系．     

Seckel syndrome associated with oligodontia,microdontia, enamel hypoplasia,delayed eruption,and dentin dysmineralization: a new variant?

Seckel syndrome (SCKL) [OMIM Entry 210600] is a rare, autosomal recessive syndrome, characterized by severe intrauterine and postnatal growth retardation, microcephaly, mental retardation, and typical facial appearance with beaklike protrusion of the midface (bird-headed). Associated findings may include limb anomalies, dislocation of femoral heads, scoliosis, and gastrointestinal malformation. A 14-year-old boy is presented with brain hypoplasia, pachygyria, hydrocephaly, enamel hypoplasia and root dysplasia in the temporary dentition, and oligodontia, severe microdontia, and delayed eruption of the permanent dentition. The association of SCKL with the above unusual dental findings may represent a new phenotype.     

Dental and oral anomalies in incontinentia pigmenti: a systematic review

Objectives

Incontinentia pigmenti (IP) is an X-linked genodermatosis caused by a mutation of the IKBKG gene. The objective of this study was to present a systematic review of the dental and oral types of anomalies, to determine the total number and sex distribution of the anomalies, and to analyze possible therapies.

Materials and methods

We analyzed the literature data from 1,286 IP cases from the period 1993–2010.

Results

Dental and/or oral anomalies were diagnosed for 54.38% of the investigated IP patients. Most of the anomaly types were dental, and the most frequent of these were dental shape anomalies, hypodontia, and delayed dentition. The most frequent oral anomaly types were cleft palate and high arched palate. IKBKG exon 4–10 deletion was present in 86.36% of genetically confirmed IP patients.

Conclusions

According to the frequency, dental and/or oral anomalies represent the most frequent and important IP minor criteria. The most frequent mutation was IKBKG exon 4–10 deletion. The majority of dental anomalies and some of the oral anomalies could be corrected.

Clinical relevance

Because of the presence of cleft palate and high arched palate in IP patients, these two anomalies may be considered as diagnostic IP minor criteria as well.     

Isolated soft tissue cleft lip: epidemiology and associated dental anomalies

Oral Diseases (2011) 17 , 221–231 Objectives: The aim of this investigation was to study the epidemiology of the isolated soft tissue cleft lip (ICL) population and to evaluate the dental anomalies associated with permanent dentition. Methods: The study included 19 children aged 9–13 years presenting ICL selected from 657 cleft lip‐affected patients treated during the last 10 years in two craniofacial centers. Only 17 patients could be included for dental anomaly evaluation: Hyperdontia, Hypodontia, Gemination, Talon tooth, Microdontia, and Macrodontia. These were compared with cleft lip and palate (CLP) and cleft lip and alveolus (CLA)‐affected populations and with normal populations. Results: The prevalence of ICL was 2.8%. All types of tooth abnormalities were found to be higher and mainly significant for the cleft side of ICL compared with the normal population. On the side opposite the cleft, the prevalence of dental anomalies reduced toward the normal individuals and was not significantly different. The significant differences found between CLP, CLA, and ICL‐affected populations were mostly depicted by lateral incisors and second pre‐molar hypodontia. Conclusions: Isolated cleft lip is a rare phenomenon among the spectrum of the cleft‐affected population. The prevalence of the dental anomalies in ICL maintains the proportional trend according to clefting severity.     

Microdontia and hypodontia of premolars and permanent molars in childhood cancer survivors after chemotherapy

International Journal of Paediatric Dentistry 2012; 22: 239–243 Background. Adverse long‐term general and dental health effects of cancer and cancer therapy during childhood have been reported. Aim. To examine the association between chemotherapy before the age of 8 years and (1): microdontia; (2): hypodontia of premolars and permanent molars. Material and methods. In The Danish Registry of Childhood Cancer (DBCR), we identified 203 children who met the following inclusion criteria: (1) age below 8 years at the start of treatment; (2) age between 12 to 18 years upon dental examination; (3) had received chemotherapy The exclusion criterion was radiotherapy to the head and neck. A total of 150 children fulfilled the inclusion criteria. As controls, a random sample of 193 age‐matched unexposed children was included. Results. Microdontia was found in a total of 88 teeth in 29 (19.3%) of the 150 children who had been exposed to chemotherapy, while none of the controls had microdontia of premolars or permanent molars (difference: 19.3%; 95% CL: 13.5%; 26.4%). The earlier the exposure, the more frequent was microdontia. We found a total of 27 missing premolars and permanent molars in 14 (9.3%) of the exposed children and a total of 18 missing premolars and permanent molars in 8 (4.1%) of the controls (difference: 5.2%; 95% CL: ?0.1%; 11.3%). Conclusion. The present study confirms findings from previous studies that chemotherapy, especially in very young children, causes microdontia and hypodontia of premolars and permanent molars.     

Pfeiffer syndrome: oral healthcare management and description of new dental findings in a craniosynostosis

Pfeiffer syndrome is a rare fibroblast growth factor receptor‐related craniosynostosis with variable clinical presentations. We describe new dental findings of hypodontia, microdontia, dilacerations, and radicular dentin dysplasia in a 19‐year‐old girl, and discuss the oral health management.     

Prevalence and distribution of selected developmental dental anomalies in an Indian population

The purpose of this study was to determine the prevalence of developmental dental anomalies in an Indian population and to statistically analyze the distribution of these anomalies. The study was based on clinical examination, evaluation of dental casts, and panoramic radiographs of 1123 Indian subjects (572 males, 551 females), who visited the outpatient clinic at Government Dental College, Indore between November 2009 and September 2010, after obtaining their informed consent. These patients were examined for the following developmental dental anomalies: shape anomalies (microdontia, talon cusp, dens evaginatus, fusion, taurodontism), number anomalies (hypodontia, oligodontia, anodontia), structural anomalies (amelogenesis imperfecta, dentinogenesis imperfecta) and positional anomalies (ectopic eruption, rotation, impaction). The percentages of these anomalies were assessed for the whole group and compared using statistical analysis. Among the 1123 subjects, a total of 385 individuals (34.28%) presented with the selected developmental dental anomalies. The distribution by sex was 197 males (34.44%), and 188 females (34.06%). Out of the total 1123 individuals, 351 (31.26%) exhibited at least one anomaly, 28 (2.49 %) showed two anomalies and 6 (0.53%) displayed more than two anomalies. P values indicated that the dental anomalies were statistically independent of sex. On intergroup comparison, positional anomalies were significantly most prevalent (P < 0.05) in the Indian population. The most common developmental dental anomaly was rotation (10.24%), followed by ectopic eruption (7.93%). The next common group was number anomalies. The most common number anomaly was hypodontia (4.19%), which had a higher frequency than hyperdontia (2.40%). Analyzing the next prevalent group of shape anomalies, microdontia (2.58%) was found to be the most common, followed by taurodontism (2.49%), dens evaginatus (2.40%) and talon cusp (0.97%). Dentinogenesis imperfecta (0.09%) was the rarest, followed by amelogenesis imperfecta (0.27%) and fusion (0.27%).     

A controlled study of the association of various dental anomalies with hypodontia of permanent teeth

Although hypodontia, or oligodontia, is one of the most common human dental anomalies observed, there have been few studies on the association of other anomalies occurring with it. The present investigation of 1032 patient records found that 65.7% of patients with hypodontia showed ankylosis of primary molars compared to only 1.5% of control children (P less than 0.001). In addition, taurodontism of the mandibular first permanent molar was observed in 34.3% of hypodontia cases compared to 7.1% in the controls (P less than 0.001). Other dental anomalies significantly associated with hypodontia include enamel hypoplasia (11.9%, P less than 0.01) and conical incisors (8.9%, P less than 0.01). In contrast, there were significantly more impacted teeth in control children compared to the hypodontia group. The results indicate that for patients with missing permanent teeth, clinicians should be alert to the possibility of these associated anomalies and their accompanying clinical implications.     

Anomalies of deciduous teeth and findings in permanent dentition]

Prevalence of anomalies of deciduous teeth has been analyzed in the general population of Zagreb preschool children. The sample comprised 2,987 children from 3 to 6 years of age (1,582 boys and 1,405 girls). Hypodontia was found in 0.47%, hyperdontia in 0.10%, and double teeth in 0.43% of total sample. Total prevalence of all anomalies in the sample was 1.0%. The sample for the analysis of permanent dentition has been enlarged with the clinical sample of children having the same anomalies and comprised 48 children. In all children with anomalies of primary teeth the orthopantomograms were taken and the status of permanent dentition was analyzed. In children with hypodontia in primary dentition hypodontia of permanent teeth was found in 100% of cases. Patients with hyperdontia of primary teeth displayed anomalies in permanent dentition in 85.7%, while in children with primary double teeth, anomalies of permanent teeth were present in 61.1% of cases. Missing deciduous teeth were found in both jaws in only 8.7% of cases, and in 34.8% in permanent dentition. Symmetrical occurrence of hypodontia of primary teeth (i.e. in both sides of jaws) was significantly higher in boys (56.3%) than in girls (28.6%). In permanent dentition symmetrical occurrence of hypodontia was significantly higher than in primary dentition (81.3% in boys and 57.1% in girls). The most frequently missing primary teeth were maxillary lateral incisors (48.8%) followed by mandibular central incisors (34.9%). Hyperdontia of primary teeth has been found only in boys, and it related only to the maxillary lateral incisors. Anomalies of deciduous teeth show a high degree of association with the finding in the permanent dentition.(ABSTRACT TRUNCATED AT 250 WORDS)     

Delayed eruption of permanent teeth in hyperimmunoglobulinemia E recurrent infection syndrome

OBJECTIVE: To determine the incidence of abnormal tooth eruption in patients with hyperimmunoglobulinemia E (hyper-IgE) syndrome. STUDY DESIGN: This study evaluated 34 individuals with hyper-IgE syndrome (age range, 2-40 years). A comprehensive dental history and a head and neck evaluation were performed on all patients. Dental age was assessed in patients younger than 17 years by 2 methods: (1) clinical assessment of tooth eruption and (2) a radiographic method. Relationships between the chronologic age, dental developmental age, and age at tooth eruption were determined. Other oral or dental anomalies were recorded. RESULTS: Of patients older than 7 years, 75% reported problems with permanent tooth eruption, as evidenced by retained primary teeth or the need for elective extractions of primary teeth to allow eruption of permanent teeth. None of the patients experienced problems with eruption of primary teeth. Eruption of the first and second permanent molars also occurred on time. Dental maturity scores were established for 14 patients 17 years of age or younger. In each case, the difference between chronologic age and the estimated dental developmental age was less than 12 months; however, we found a significant discrepancy between the chronologic age and the mean age of tooth eruption in 80% of these patients when using a particular set of standardized values. Persistence of Hertwig's epithelial root sheath was observed on histologic examination. Chronic multifocal oral candidiasis was a consistent feature in patients with hyper-IgE recurrent infection syndrome. Other oral anomalies were also noted. CONCLUSION: We confirmed that a disorder of tooth eruption is part of the hyper-IgE syndrome. This problem occurs because of delayed primary tooth exfoliation rather than a developmental delay in the formation of the permanent dentition. The persistence of Hertwig's epithelial root sheath is unusual and may be associated with the lack of resorption of the primary teeth. Dentists should be aware of this feature of hyper-IgE syndrome because timely intervention will allow normal eruption to occur.