Agenesia e fatores associados em pacientes portadores de fissura labiopalatina: uma revisão de literatura / Agenesis and associated factors in patients with cleft lip and palate: a review of the literature

Objetivo: verificar as principais agenesias dentárias e os fatores associados em portadores de fissura labiopalatina por meio de uma revisão de literatura. Métodos: foi realizada uma revisão de literatura utilizando as bases de dados PubMed, SciELO e Google Acadêmico. Revisão de literatura: foram incluídos 34 artigos, sendo 2 casos clínicos, 15 estudos retrospectivos, 10 revisões de literatura, 4 estudos de caso-controle e 5 estudos transversais; além de 3 referências escolhidas através de outras buscas para complementar a revisão. A partir dos estudos incluídos no trabalho, foi possível verificar que a maior prevalência ocorre no sexo masculino. E a fissura que mais acomete a população é a transforame incisivo. Pôde-se analisar também que não há causa exata, porém, existem fatores predisponentes, como os ambientais e os hereditários. Considerações finais: esta revisão de literatura poderá auxiliar os profissionais de saúde para a realização de diagnóstico e planejamento precoces, a fim de encontrar o melhor e mais eficiente tratamento para cada caso. O tratamento precoce, por uma equipe multidisciplinar, certamente garantirá uma melhor qualidade de vida aos pacientes.(AU)

Objective: to verify the main dental agenesis and associated factors in cleft lip and palate through a literature review. Methods: a literature review was carried out using PubMed, SciELO and Google Scholar databases. Literature review: 34 studies were included, 2 clinical studies, 1 retrospective study, 10 literature reviews, 4 case-control studies and 5 cross-sectional studies. In addition to 3 references chosen through other searches complementary to the review. From the included studies, it was verified a higher frequency of cleft lift and palate for the male sex, and a higher frequency of more severe clefts, such as the incisive transformation, in comparison to the labiopalatine. It can also be analyzed that there is no exact cause; however, there are environmental and hereditary predisposing factors. Final considerations: this literature review may help health professionals to carry out early diagnosis and treatment planning, in order to find the best and most efficient treatment for each case. Early treatment by a multidisciplinary team will certainly guarantee a better quality of life for patients.(AU)

Novel truncating mutations in CTNND1 cause a dominant craniofacial and cardiac syndrome.

CTNND1 encodes the p120-catenin (p120) protein, which has a wide range of functions, including the maintenance of cell-cell junctions, regulation of the epithelial-mesenchymal transition and transcriptional signalling. Due to advances in next-generation sequencing, CTNND1 has been implicated in human diseases including cleft palate and blepharocheilodontic (BCD) syndrome albeit only recently. In this study, we identify eight novel protein-truncating variants, six de novo, in 13 participants from nine families presenting with craniofacial dysmorphisms including cleft palate and hypodontia, as well as congenital cardiac anomalies, limb dysmorphologies and neurodevelopmental disorders. Using conditional deletions in mice as well as CRISPR/Cas9 approaches to target CTNND1 in Xenopus, we identified a subset of phenotypes that can be linked to p120-catenin in epithelial integrity and turnover, and additional phenotypes that suggest mesenchymal roles of CTNND1. We propose that CTNND1 variants have a wider developmental role than previously described and that variations in this gene underlie not only cleft palate and BCD but may be expanded to a broader velocardiofacial-like syndrome.

Study of rs12532, rs8670 Polymorphism of Msh Homeobox 1 (MSX1), rs61754301, rs4904155 Polymorphism of Paired Box Gene 9 (PAX9), and rs2240308 Polymorphism of Axis Inhibitor Protein 2 (AXIN2) Genes in Nonsyndromic Hypodontia.

The etiology of hypodontia is complex, in which both genetic and environmental factors can be related. The main objective of our study was to contribute to elucidating the genetic background of nonsyndromic hypodontia (NSH). In this order, we selected 97 NSH subjects (70 females and 27 males) from patients referred to orthodontic treatment, and we matched to each NSH subject a control by age and sex. DNA was obtained from epithelial cells from the oral mucosa. Genotyping of the PAX9 (rs4904155 and rs61754301), MSX1 (rs8670 and rs12532), and AXIN2 (rs2240308) SNPs was performed by using TaqMan SNP Genotyping Assays on a real-time PCR system. Single-nucleotide polymorphisms (SNPs) were studied for the whole NSH group and for frontal and lateral agenesis NSH subjects separately. Our results showed that the variant genotype (p=0.0008, OR = 2.9, 95% CI = 1.58-5.3) and variant T allele (p=0.0002, OR = 2.65, 95% CI = 1.6-4.39) of the MSX1 rs8670 SNP increased the risk of hypodontia in the studied population when the whole NSH group was compared with controls. The variant genotype of the MSX1 rs8670 SNP was the most frequent in frontal agenesis; meanwhile in the lateral agenesis NSH group, the AXIN2 rs2240308 SNP showed a higher frequency of the variant genotype, with a trend towards statistical significance. In conclusion, the results of the present study showed that the variant genotype and variant T allele of the MSX1 rs8670 SNP increased the risk of hypodontia in the studied population. The presence of the variant A allele of AXIN2 rs2240308 is associated with frontal agenesis but not with lateral agenesis.

Rehabilitation of ectodermal dysplasia patients presenting with hypodontia: outcomes of implant rehabilitation part 1.

PURPOSE: This study has evaluated the pre and post perceptions of patients with ectodermal dysplasia (ED) who have been referred to Westmead Centre for Oral Health for treatment with dental implants. METHODS: Six patients with ED and hypodontia were treated with new maxillary complete dental prostheses or fixed implant prostheses, and a mandibular fixed dental prosthesis with implants. Patient perceptions were recorded with a 10cm visual analogue scale (VAS). All patients were asked to draw a line on the VAS which best described their feelings regarding aesthetics, chewing, diet and speech. RESULTS: Improvement in aesthetics was reported for all patients with change scores ranging from +9 to +4 points. Similarly, all patients reported an improvement in chewing ability with +6 point changes in 2 patients, and +8, +3, +7 and +1 in the other 4 patients. Five out of 6 patients reported less dietary restriction with 3 patients each indicating a change of +6 points, +4 points, +3 points, and 2 patients reporting +1 point change. Speech improved by +1 point for 3 patients, whilst 1 patient improved by +6 points, a further 2 patients scored no change. CONCLUSIONS: Patients with ED treated with dental implants reported encouraging outcomes post treatment after prostheses were fitted. Follow-up from 1.6 to 6.8 years has confirmed these improvements.

Tooth agenesis: A susceptible indicator for colorectal cancer?

CONTEXT/BACKGROUND: Tooth agenesis (excluding third molars) is a common congenital disorder that affects 2.2-10% of the general population. A number of different genes have been shown to be associated with cases of tooth agenesis including AXIN2, IRF6, FGFR1, MSX1, PAX9, and TGFA. Wingless/integration signaling gene, AXIN2, is linked to tooth agenesis and also to colorectal cancer (CRC). AIMS: To analyze the correlation between tooth agenesis and CRC. MATERIALS AND METHODS: The study included 50 individuals, who were divided into two groups. Group A: 25 individuals diagnosed with CRC and Group B: 25 individuals as a control group. The clinical details were recorded using preformed questionnaire, approved by ethical committee. Orthopantomogram was obtained for all the cases and controls. RESULTS: We observed that 16% of cases and 8% of controls reported having tooth agenesis and there was no statistical significance of difference between them (P = 0.384). Among the study group, 4% reported oligodontia and 12% cases reported hypodontia. In the control group 8% reported hypodontia, there was no incidence of oligodontia. Additional finding in the study group was that 24% cases had fissured tongue which was not seen in the control group. CONCLUSION: Individuals with tooth agenesis might have an increased risk for CRC. A larger epidemiological study along with genetic mapping and gene sequencing is necessary to rule out the risk and relationship between tooth agenesis and CRC.

WNT10B mutations associated with isolated dental anomalies.

Isolated hypodontia is the most common human malformation. It is caused by heterozygous variants in various genes, with heterozygous WNT10A variants being the most common cause. WNT10A and WNT10B are paralogs that likely evolved from a common ancestral gene after its duplication. Recently, an association of WNT10B variants with oligodontia (severe tooth agenesis) has been reported. We performed mutational analysis in our cohort of 256 unrelated Thai families with various kinds of isolated dental anomalies. In 7 families afflicted with dental anomalies we detected 4 heterozygous missense variants in WNT10B. We performed whole exome sequencing in the patients who had WNT10B mutations and found no mutations in other known hypodontia-associated genes, including WNT10A, MSX1, PAX9, EDA, AXIN2, EDAR, EDARADD, LPR6, TFAP2B, LPR6, NEMO, KRT17, and GREM2. Our findings indicate that the variants c.475G>C [p.(Ala159Pro)], found in 4 families, and c.1052G>A [p.(Arg351His)], found in 1 family, are most probably causative. They also show that WNT10B variants are associated not only with oligodontia and isolated tooth agenesis, but also with microdontia, short tooth roots, dental pulp stones, and taurodontism.

Disturbances of dental development distinguish patients with oligodontia-ectodermal dysplasia from isolated oligodontia.

OBJECTIVE: To investigate phenotypic differences in dental development between isolated oligodontia and oligodontia-ectodermal dysplasia (ED). SETTING AND SAMPLE POPULATION: A total of 129 patients diagnosed with isolated oligodontia and 22 patients with oligodontia as part of ED were eligible. METHODS: The phenotype of dental development was assessed for the frequency of missing a certain tooth, dental age, development of each tooth present, abnormal size and abnormal shape of teeth. The data were analysed building linear, ordinal and logistic regression models. RESULTS: Compared to patients with isolated oligodontia, patients with oligodontia-ED missed more frequently central incisors and second molars in both jaws, and lateral incisors in the mandible (P < .05). Oligodontia-ED was associated with delayed development of the permanent dentition (ß = -0.10; 95% CI: -0.17, -0.03). Specifically, the maxillary teeth: right central incisor, right lateral incisor, right second premolar and left second premolar were delayed approximately from 2 to 4 developmental stages. In addition, the left mandibular second premolar was 3 developmental stages delayed. Abnormal shape of teeth was 7 times more evident in patients with oligodontia-ED compared to patients with isolated oligodontia (OR = 6.54; 95% CI: 2.34, 18.28). The abnormal size of teeth was not a distinctive characteristic for oligodontia-ED. CONCLUSIONS: Oligodontia-ED distinguishes from isolated oligodontia by more disturbances in dental development. The abnormal shape of incisors and canines in a patient with oligodontia can raise suspicions for accompanying ectodermal abnormalities.

Cephalometric Soft Tissue Characteristics of Unilateral Cleft Lip and Palate Patients in Relation to Missing Teeth.

OBJECTIVE: This study aimed to evaluate cephalometric soft tissue characteristics in individuals with unilateral complete cleft lip and palate (UCCLP) both with and without missing teeth. DESIGN: A retrospective investigation of patient records, who are being treated at the cleft lip and palate (CLP) clinics at the College of Dentistry. Ninety-six consecutive records of nonsyndromic UCCLP subjects were recruited (33 subjects without missing teeth and 63 subjects with missing teeth). Linear and angular soft tissue measurements obtained from lateral cephalometric radiographs were evaluated and compared among the studied samples. RESULTS: Lower lip was significantly retruded and shorter (p = 0.037), p = 0.015, respectively; in addition to the fact that shallower mentolabial sulcus (p = 0.05) was found in the subjects with missing teeth, the rest of the soft tissue was not significantly different between the two groups. CONCLUSION: In subjects with a UCCLP anomaly, missing teeth have an effect on lower lip position and length, which influenced the mentolabial sulcus. Lower lip position and length differ between cleft patients who present with either multiple missing teeth or with no missing teeth, and this needs to be considered during orthodontic treatment planning and surgical management for the cleft defect.

A novel G to A transition at initiation codon and exon-intron boundary of PAX9 identified in association with familial isolated oligodontia.

Several studies on experimental animals indicate that the process of organogenesis crucially depends upon the spatiotemporal dose of certain critical bio-molecules. Tooth development is also not an exception. While most of the knowledge regarding the molecular mechanism of tooth development comes from the studies on mouse model, pathogenic variations identified in human tooth agenesis also provide valuable information on mammalian tooth development. Until now five major candidate genes have been identified for tooth agenesis in human. Among them, PAX9 plays the crucial role in tooth development and in non-syndromic congenital tooth agenesis. In this study, microsatellite and SNP based genotyping identifies a disease specific haplotype block, which includes PAX9 gene, segregates with autosomal dominant tooth agenesis phenotype. Direct sequencing of PAX9 identifies a novel heterozygous G to A transition at the third base (c.3G>A) of initiation codon leading to ATG to ATA shift in all affected individuals which is absent in all unaffected relatives and 200 control chromosomes. Further, in vitro functional analysis creating PAX9 minigene construct did apparently show no effect on the splice-site migration. It is therefore proposed that haploinsufficiency of PAX9 is the causal factor for tooth agenesis in this family.

Dentition Patterns in Different Unilateral Cleft Lip Subphenotypes.

Oral clefts play an essential role in disturbed odontogenesis of the deciduous and permanent dentition, yet little is known about this relationship. We investigated, within the categories cleft lip with or without alveolus (CL ± A) and cleft lip, alveolus and palate (CLAP), whether different CL subphenotypes based on morphological severity of the cleft show different dentition patterns and whether a more detailed subdivision of the incomplete CL has clinical relevance. In this retrospective study, 345 children with nonsyndromic unilateral CL ± A and CLAP from the Dutch Association for Cleft Palate and Craniofacial Anomalies (NVSCA) registry were included to assess the association between the CL subphenotypes and lateral incisor patterns. Five different deciduous and permanent patterns of the lateral incisor were distinguished: located in normal position (pattern z/Z), in the anterior segment (pattern x/X) or in the posterior segment of the cleft (pattern y/Y), one in each segment of the cleft (pattern xy/XY), and agenesis of the lateral incisor (pattern ab/AB). Analyses were performed by using multinomial logistic regression models. Children born with a vermillion notch or a one-third to two-thirds CL were most likely to have a deciduous pattern x and a permanent pattern X, while children born with a two-thirds to subtotal CL were most likely to have deciduous pattern xy and a permanent pattern X compared to children with a complete CL that predominantly had deciduous pattern y and a permanent pattern AB. Based on the relationship of the CL morphology with the deciduous dentition, subdivision of the CL morphology into vermillion notch to two-thirds CL, two-thirds to subtotal CL, and complete CL appears to be an optimal subdivision. Our results indicate that a more detailed subdivision of the CL has clinical relevance and that critical factors in the pathogenesis of the CL are also critical for the odontogenesis.

Dental Age and Tooth Development in Orthodontic Patients with Agenesis of Permanent Teeth.

Objective. To compare the development of permanent teeth in a group of children with the congenitally missing permanent teeth (CMPT) and corresponding nonaffected group. Methods. The formation stages of all developing permanent teeth were determined on 345 panoramic radiographs (OPTs) by the method of Haavikko (1970), and dental age was calculated. The paired samples t-test was used to compare the differences between dental age (DA) and chronological age (CA) in those with CMPT and those not affected. Spearman test was used to evaluate the correlation between DA-CA and the number of missing teeth. The Wilcoxon signed rank test was used to compare the development of the teeth adjacent to the place of the agenesis with matched pair in corresponding nonaffected group. Results. Dental age was significantly delayed in CMPT children compared to the nonaffected group (p < 0.001). The mean differences were -0.57 ± 1.20 years and -0.61 ± 1.23 years in males and females, without difference between sexes (p = 0.763). The number of missing teeth affected the delay only in females (p = 0.024). Only mesial teeth in females were significantly delayed in development when compared to the nonaffected group (p = 0.007). Conclusion. Our findings show that the development of the permanent teeth is delayed when compared to the nonaffected group of the same sex and age.

Dental development in Down syndrome and healthy children: a comparative study using the Demirjian method.

OBJECTIVE: In children with Down syndrome, the timing of dental eruption is important for orthodontics treatment planning. Aim of this study was to determine whether tooth eruption and development of the dentition in children with Down syndrome are impaired. MATERIAL AND METHODS: Dental development was scored on orthopantomograms (OPTs) of 95 children with Down syndrome. The dental age was determined at the left mandibular side according to the Demirjian method and by converting the assigned scores to the dental maturity score. Dental development scores of control children and DS children were compared with a mixed model linear regression analysis. RESULTS: The model showed statistically significant changes relating to increasing age (P<0.001) and gender (P<0.05). In this comparison, the total DS group (with and without hypodontia) was not statistically significantly different from the control group. There was also no significant difference between the total sample of DS children and the control group after using the Nyström imputation (with and without hypodontia). CONCLUSION: The findings showed that dental development in DS children is similar to the development of control children and that a relationship exists between hypodontia and dental development. The clinically observed late eruption is probably not due to late dental development but due to the other processes that take place during eruption, such as the possible impaired processes at the apical side and the occlusal side of an erupting element.

Tooth agenesis and orofacial clefting: genetic brothers in arms?

Tooth agenesis and orofacial clefts represent the most common developmental anomalies and their co-occurrence is often reported in patients as well in animal models. The aim of the present systematic review is to thoroughly investigate the current literature (PubMed, EMBASE) to identify the genes and genomic loci contributing to syndromic or non-syndromic co-occurrence of tooth agenesis and orofacial clefts, to gain insight into the molecular mechanisms underlying their dual involvement in the development of teeth and facial primordia. Altogether, 84 articles including phenotype and genotype description provided 9 genomic loci and 26 gene candidates underlying the co-occurrence of the two congenital defects: MSX1, PAX9, IRF6, TP63, KMT2D, KDM6A, SATB2, TBX22, TGFα, TGFß3, TGFßR1, TGFßR2, FGF8, FGFR1, KISS1R, WNT3, WNT5A, CDH1, CHD7, AXIN2, TWIST1, BCOR, OFD1, PTCH1, PITX2, and PVRL1. The molecular pathways, cellular functions, tissue-specific expression and disease association were investigated using publicly accessible databases (EntrezGene, UniProt, OMIM). The Gene Ontology terms of the biological processes mediated by the candidate genes were used to cluster them using the GOTermMapper (Lewis-Sigler Institute, Princeton University), speculating on six super-clusters: (a) anatomical development, (b) cell division, growth and motility, (c) cell metabolism and catabolism, (d) cell transport, (e) cell structure organization and (f) organ/system-specific processes. This review aims to increase the knowledge on the mechanisms underlying the co-occurrence of tooth agenesis and orofacial clefts, to pave the way for improving targeted (prenatal) molecular diagnosis and finally to reflect on therapeutic or ultimately preventive strategies for these disabling conditions in the future.

Alteration of palatine ruga pattern in subjects with oligodontia: A pilot study.

INTRODUCTION: The aim of this study was to compare the palatal ruga patterns in subjects with oligodontia and normal tooth numbers. METHODS: An observational investigation was conducted by using maxillary dental study casts to compare ruga numbers, lengths, and shapes in subjects with diagnosed oligodontia or normal tooth numbers. RESULTS: A total of 32 subjects comprised both the oligodontia (mean age, 14.0 years; SD, 5.0 years) and the control (mean age, 14.5 years; SD, 5.1 years) groups. The mean number of missing teeth in the oligodontia group was 8.7. The mean number of rugae in the whole sample was 7.36 (SD, 1.16), with no significant difference between the groups (P = 0.264). For ruga pattern, no differences were found for right-sided rugae; however, on the left side, a significant difference existed in shape frequency associated with ruga 2. Specifically, a curved shape was seen more frequently in ruga 2 of the oligodontia group (P = 0.01). CONCLUSIONS: The identification of subtle differences in ruga patterns between subjects with oligodontia and normal tooth numbers suggests potentially shared pathways during the development of these oral structures. Further large-scale investigations are warranted.

Radiographic Assessment of Dental Maturation in Children With Dental Agenesis.

BACKGROUND: Dental agenesis is the most common developmental anomaly in humans, frequently associated with disorders in dental development and maturation. AIM: The purpose of this study is to determine radiographic variations in dental maturation in a group of Venezuelan children with dental agenesis. STUDY DESIGN: 1,188 panoramic radiographs, from healthy patients ages 5 to 12 years old were studied for agenesis of permanent teeth. Dental maturation was assessed by relative eruption and dental age according to Nolla, comparing children affected with dental agenesis to a stratified control group selected from the same population, excluding children with premature loss of primary teeth in the left quadrants and unclear radiographs. Descriptive analysis, and differences between means and medians (Student t test, Kruskall-Wallis p=0.05) were performed. RESULTS: Medians for Nolla stages were similar between groups, with delay in tooth formation in the agenesis group for second molars (p<0.05) and maxillary lateral incisors and second premolars. Dental age was significantly underestimated for both groups, -0.89 (±0.78) for the control group and -1.20 (±0.95) for the study group. Tooth eruption was similar between groups. CONCLUSION: Dental age was significantly delayed in Venezuelan children with dental agenesis, with variable significance for tooth formation of studied teeth.

Effect of malocclusion severity on oral health-related quality of life and food intake ability in a Korean population.

INTRODUCTION: The aim of this study was to evaluate the effect of malocclusion severity on oral health-related quality of life and food intake ability in adult patients, controlling for sex, age, and the type of dental clinic visited. METHODS: The sample consisted of 472 Korean patients (156 male, 316 female) with a mean age of 21.1 (SD, 8.6) years in a dental hospital and a private clinic. The correlations between the Korean version of the Oral Health Impact Profile-14 (OHIP-14K), subjective food intake ability (FIA) for 5 key foods, and Index of Orthodontic Treatment Need-Dental Health Component (IOTN-DHC) were investigated. RESULTS: The mean IOTN-DHC and OHIP-14K scores were significantly higher for the dental hospital patients than for the private clinic patients (IOTN-DHC, P <0.001; OHIP-14K, P <0.05). Malocclusion severity was significantly higher in male than in female subjects (P <0.001). Older patients perceived their oral health-related quality of life more negatively than did the teens (P <0.001). As the severity of the malocclusion increased, oral health-related quality of life and masticatory function worsened (OHIP-14K, P <0.001; FIA, P <0.05). CONCLUSIONS: As the severity of the malocclusion and the age of the patients increased, oral health-related quality of life and masticatory function relatively deteriorated. This finding provides evidence that severe malocclusions are associated with lower quality of life and less masticatory efficiency in older patients.

Prosthetic treatment outcome in patients with severe hypodontia: a systematic review.

Severe hypodontia (≥6 missing teeth) is associated with aesthetic and functional problems. Its presentation is heterogenic, and a variety of treatment modalities are used resulting in different treatment outcomes. As there is currently no standard treatment approach for patients with severe hypodontia, the literature was systematically reviewed with the focus on treatment outcomes. Medline, Embase and The Cochrane Central Register of Controlled Trials were searched (last search 24 August 2015). This was completed with a manual search of the reference lists of the selected studies. To be included, studies had to describe dental treatment outcome measure(s) in patients with severe hypodontia; there were no language restrictions. The methodological quality was assessed using MINORS criteria. Twenty-one studies were eligible, but the diversity in type and quality did not allow for a meta-analysis; seventeen studies had a retrospective design; sixteen studies described the results of implant treatment. Treatment with (partial) dentures, orthodontics, fixed crowns or bridges was sparsely presented in the eligible studies. Implant survival, the most frequently reported treatment outcome, ranging from 35·7% to 98·7%, was influenced by 'location' and 'bone volume'. The results of implant treatment in severe hypodontia patients are promising, but due to its heterogenic presentation, its low prevalence and the poor quality of the studies, evidence-based decision-making in the treatment of severe hypodontia is not yet feasible, thus prompting further research.

The relationship between craniofacial development and hypodontia in patients with Down syndrome.

BACKGROUND/OBJECTIVE: Hypodontia is often seen in people with Down syndrome (DS). In the normal population, persons with hypodontia have a shorter cranial base and a hypoplastic maxilla, leading to a skeletal Class III tendency and a reduced face height. The purpose of this study was to examine craniofacial morphology in patients with DS at different ages and the influence of hypodontia on their craniofacial morphology. MATERIALS AND METHODS: A comparative cross-sectional study was conducted in 63 children with DS (6-19 years old; 28 males and 35 females) at a Centre for Special Care Dentistry in Rotterdam, the Netherlands (CBT Rijnmond). Digital lateral cephalograms were obtained from all subjects and a cephalometric analysis was performed. The subjects were divided into a group with hypodontia (13 males and 25 females) and a group without hypodontia (15 males and 10 females). RESULTS: Significant results included a decrease in antero-posterior relationship of upper and lower jaw (ANB angle -0.331° per year, P = 0.044) and a decrease in vertical dimension (S-N_Go-Gn angle -0.72° per year, P = 0.039) over the years in subjects with hypodontia compared to subjects without hypodontia. CONCLUSION: The process of growth in DS patients is towards a reversed overjet. Hypodontia seems to have an additional effect on this development. The management of hypodontia as part of the complete treatment of dental development in DS children is important because it strongly influences the jaw relationship.

Familial Hypodontia: A Case Series.

The phenomenon of congenitally missing teeth has been described with different names: hypodontia, oligodontia, anodontia, congenitally missing teeth and dental agenesis. The present paper reports familial hypodontia, involving two siblings from a single family. Case I reported the absence of 18, 12, 22 and 31 teeth whereas case II reported the absence of 31 and 41. On the evaluation of the two reported cases that were offspring of same parents and absence of any clinical features associated with any syndrome the final diagnosis of non- syndromic familial hypodontia was made. Both the cases presented agenesis of the teeth leading to malocclusion and aesthetic disturbances. Cases were offered a treatment of interdisciplinary dental approach.