Prevalence and morphological features of molar-root incisor malformation in children attending a specialist paediatric dental unit.

BACKGROUND: Molar-root incisor malformation (MRIM) is a rare dental anomaly featuring constricted cervical margins and tapered, narrow root and pulp morphology, often associated with severe toothache and infection. AIM: The aim of this study was to determine the prevalence of MRIM in children seen in a specialist paediatric dental unit of a tertiary referral hospital and to describe the characteristics of affected individuals. DESIGN: This study was an audit of children attending from November 2020 to November 2021. Radiographs were used to identify individuals with MRIM, and clinical data were collated. In addition, histology and microcomputed tomography (microCT) imaging were performed on teeth extracted from an affected individual. RESULTS: The prevalence of MRIM was five cases of 1054 children examined (0.47% or 1:210). The permanent first molars were affected in all five children and the primary second molars in two children; all children had medical comorbidities and multiple exposures to general anesthesia before 4 years of age. In addition, histological and microCT analyses displayed numerous microchannels connecting the pulp chamber to the external surface of the tooth at the furcation. CONCLUSIONS: Molar-root incisor malformation is an uncommon dental anomaly affecting paediatric patients with multiple comorbidities and is characterized by porosities extending from the pulp chamber to the external tooth surface, predisposing the risk of bacterial ingress from the oral cavity into the pulp chamber. Early detection may prevent atypical odontogenic facial pain and infection.

Molar-root incisor malformation - a systematic review of case reports and case series.

OBJECTIVES: Molar-root incisor malformation (MRIM) is a seldom reported condition characterised by disturbances in root development of first permanent molars. This systematic review aimed to collate the clinical characteristics of individuals diagnosed with MRIM. MATERIALS AND METHODS: A systematic search strategy using PubMed, Embase, Web of Science, and SCOPUS databases was performed through to March 2023. Inclusion criteria were case reports or case series including a diagnosis consistent with MRIM. Critical appraisal for all included studies utilised the Joanna Briggs Institute (JBI) critical appraisal checklist for case reports and case series and collation of clinical characteristics was performed in JBI System for the Unified Management, Assessment and Review of Information program. RESULTS: The search identified 157 studies from which 35 satisfied the inclusion criteria. After full-text review, a total of 23 papers described the MRIM dental anomaly and were included in this paper. A total of 130 reported cases were retrieved, with age ranging 3-32 years, and males affected 1.16:1 females. Presence of neurological conditions, premature birth history, medication, and surgery within first years of life were synthesised and described. CONCLUSIONS: The aetiology of MRIM is yet to be determined but epigenetic changes from significant medical history in the first years of life are likely to influence the development of this root malformation. First permanent molars were most commonly affected, but clinicians should be aware that permanent central incisors, primary teeth and other permanent teeth may also be affected.

Oral Cleft Related-Genes may be Involved in Root Curvature of Maxillary Lateral Incisors.

The maxilla is formed by the medial nasal and maxillary processes fusion. The dental lamina develops from 2 origins connecting in the lateral incisor. The maxillary lateral incisor region is often affected by dental anomalies and clefting. It is possible that genes involved in oral cleft could also be associated with a variety of phenotypic variations in the maxillary lateral incisor. In this phenotype-genotype study, we explored the association between polymorphisms in the oral-cleft-related genes BMP2 and BMP4 and root curvature of maxillary lateral incisors.Cross-sectional study.Universities and private clinics.Panoramic radiographs and DNA from 231 patients were analyzed.Schneider method (1971) was applied to estimate the degree of root curvature of the maxillary lateral incisors and to classify the root as straight (5° or less) or curved (higher than 5°). Genetic polymorphisms in BMP2 (rs235768 and rs1005464) and BMP4 (rs17563) were genotyped. Statistical analysis was performed.A total of 401 teeth (199 left and 202 right) were evaluated. Genetic analysis demonstrated trends toward association for the rs1005464 in BMP2 (P = .025) in co-dominant model and in dominant model (P = .026) for left incisors. The rs235768 in BMP2 showed trends toward association with the degree of root curvature in left incisors in the recessive model (P = .031). rs17563 in BMP4 also showed trends toward association with the degree of the root curvature in left incisors (P = .019).BMP2 (rs235768 and rs1005464) and BMP4 (rs17563) might be involved in maxillary lateral incisor root curvature.

The incidence of radicular groove on maxillary lateral incisors of Saudi population: CBCT evaluation.

BACKGROUND: The radicular groove (RG) is one of the developmental anomalies that is commonly found in maxillary incisors. The formation of radicular groove is initiated around the cingulum and can reach the root at different levels. The incidence of radicular grooves was reported in different countries but there was no published data about the incidence of RG in Saudi Arabia. Therefore, this study aimed to evaluate the incidence of radicular grooves on maxillary lateral incisors in the Saudi population using cone-beam computed tomography (CBCT). METHODS: The dental records of 490 patients (N = 490) with CBCT images of maxillary anterior teeth were screened for inclusion criteria. Then 264 included cases were evaluated independently by two Endodontists. The evaluation was performed on CBCT images in the axial, sagittal, and coronal sections using Planmeca Romexis® software. The following data were recorded for each patient: Patients' age and gender, radicular groove presence or absence, and if it is bilateral or unilateral. The type of radicular groove was recorded according to Gu's classification (type I, II, or III). RESULTS: The incidence rate of radicular grooves in maxillary lateral incisors was 4.9%. RG was found to be unilateral in 61.5% and bilateral in 38.5%. The majority of RG were classified as type I in 69.2%, followed by type II in 15.4%, and type III was found in 15.4%. CONCLUSION: 4.9% of the Saudi population has RG in the upper lateral incisor. This anatomical variation is mostly present as type I on one side only (unilateral).

Association between dental agenesis and delay in dental development: a preliminary study in a Spanish paediatric population in relation with Dental Anomaly Pattern (DAP).

BACKGROUND: The association between dental anomalies has been studied, giving rise to the concept of Dental Anomaly Pattern (DAP). Tooth agenesis has been associated with alterations such as molar infracclusion, taurodontism and delayed dental development. The aim of this study was to evaluate the dental development pattern in patients with non-syndromic dental agenesis, in comparison with a control group. METHODS: Dental and chronological age was analysed in a sample size of 204 orthopantomographs divided into a study group (n = 104) and a control group (n = 100) with the Demirjian Method. Intra and intergroup differences in chronological and dental age, and the correlation between them were calculated by statistical analysis with a 95% confidence level (p < 0.05). RESULTS: Dental age exceeded chronological age both in the control group and in the study group. Statistically significant differences (p = 0.004) were found when comparing the difference between chronological and dental age in the study (-0.16 ± 1.12) and control group (-0.58 ± 0.90). Regarding sex and age intergroup differences, the results were only statistically significant in the girls' group (p = 0.017), and the age over 8 years old (p < 0.05). There were no significant differences in tooth development depending on the number of missing teeth or the affected tooth group, but there was a delay in the development of the homologous tooth contralateral to the absent one in 14.9% of patients. CONCLUSIONS: The difference between chronological and dental age in permanent dentition is significantly lower in Spanish children with non-syndromic agenesis compared to a control group, presenting a lower dental age than chronological age than children without non-syndromic agenesis.

Management of an infected immature tooth with a talon cusp using regenerative endodontic treatment: A case report.

OBJECTIVE: Regenerative endodontic treatment (RET) has been considered a successful approach to manage infected immature teeth; however, cases associated with dental anomalies, i.e., talon cusp, need to be further investigated. CASE REPORT: A 7-year-old girl with local swelling associated with the permanent maxillary right central incisor was referred; cone-beam computed tomography revealed a talon cusp, an immature root and two well-defined endodontic lesions. The treatment consisted of regenerative endodontic treatment (RET); the canal was chemically cleaned and a modified triple antibiotic paste was used as intracanal medication. In the next appointment, RET was performed through the creation of blood clot scaffold in the canal covered/sealed with calcium-enriched mixture (CEM) cement. RESULTS: In the 7-day recall session, clinical examination showed that the swelling had completely resolved. At 24-month recall, the treated tooth was asymptomatic and functional. CBCT images demonstrated evidence of maturation in the apical third of the root, healing of two large endodontic lesions and complete dentinal bridge formation beneath CEM cement. CONCLUSION: RET for an infected immature tooth with a dental anomaly, i.e., talon cusp, may be a desirable treatment option and result in the resolution of endodontic lesions as well as regeneration of new vital tissues; allowing continuous root maturation.

Oral health status of children and young adults with maple syrup urine disease in Turkey.

BACKGROUND: Maple syrup urine disease (MSUD) is an inherited disorder clinically characterized by ketoacidosis, seizures, coma, psychomotor delay, and intellectual disability. The treatment requires a life-long protein-restricted diet, rich in carbohydrates and fats, supplemented with a medical amino acid formula. Diet, oral health and general health influence each other in a vicious cycle. The aim of this study was to investigate the oral health status of children and young adults with MSUD in Turkey. METHODS: A descriptive study was conducted on patients with MSUD who applied for routine follow-up to the pediatric metabolic diseases clinic at Hacettepe University, Children's Hospital in Ankara, Turkey in a 12-month period. Patients with any other concomitant genetic diseases and acute infection were excluded. A total of twenty-five patients were enrolled and underwent oral examination including DMFT/S, dmft/s (decayed/missing/filled teeth/surfaces for deciduous and primary teeth, respectively), plaque and gingival indices. Panoramic radiographs were obtained in 12 cooperative patients. RESULTS: Mean age was 9.88 ± 5.68 s.d years. More than half of the parents had only primary school level education, and low income. Fourteen patients consumed medical formula during or right before sleep. Fourteen patients reported caries-associated pain. Gingival inflammation was present in all 15 patients who cooperated for evaluation. Seven out of twelve patients had at least one dental anomaly or alterations in mandibular morphology. Five patients had previously been treated for caries under general anesthesia. To our knowledge, this is the first study to document oral clinical and radiologic findings in patients with MSUD. CONCLUSIONS: Impaired oral health was observed in this rare disease population. Regular dental referral by physicians, preventive measures and dental treatments should be included in multidisciplinary management of maple syrup urine disease to promote oral health.

Dental Manifestations of Ehlers-Danlos Syndromes: A Systematic Review.

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twenty-four individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.

Clinical management of fusion in primary mandibular incisors: a systematic literature review.

Objective: Dental anomalies occurring in deciduous teeth can affect the eruption of the permanent dentition and the occlusion stability. The occurrence of dental anomalies such as double teeth during the primary dentition in the daily practice might be frequent. The study aimed to qualitatively summarize the therapeutic management of double teeth in primary incisors.Material and Methods: A systematic review regarding the therapy of primary fused incisors in the mandible was performed and the obtained data were assessed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The following electronic databases were screened from 1st January, 1996 until 30th July, 2019: PubMed, Scopus, EBSCO and the archives of paediatric dental journals. The search terms were grouped in anatomic entity: (tooth OR teeth OR incisor), pathological condition: (fused OR fusion OR geminated OR double), intervention: (treatment OR intervention OR therapy OR prevention OR control OR management OR restoration), observed parameters: (primary dentition OR primary tooth OR primary teeth).Results: Ten articles met all inclusion criteria. The data disclosed the occurrence of double teeth in mandibular incisors. The main management of this clinical condition is either preventive or surgical involving the extraction of fused teeth, based on the deciduous nature of the teeth, the degree of caries and malocclusion development risk.Conclusion: An early diagnosis of dental anomalies is fundamental for the application of proper preventive strategies to avoid a potential malocclusion in permanent dentition and to maintain these teeth sound and caries-free until the eruption of the permanent dentition.

Dental Anomalies in Permanent Teeth Associated With Nonsyndromic Cleft Lip and Palate in a Group of Colombian Children.

OBJECTIVES: To assess the risk of dental anomaly presentation in permanent teeth in a group of Colombian children with nonsyndromic cleft lip and palate (NSCLP) and to determine the frequency of the anomalies according to the cleft type. METHODS: An analytical matched case-control study was conducted with 210 controls and 210 patients with NSCLP. The patients were classified into 3 groups: complete right unilateral cleft lip and palate (RCLP), complete left unilateral cleft lip and palate (LCLP), and complete bilateral cleft lip and palate (BCLP). Univariate and multivariate Poisson regression models were used to analyze paired samples (Bonferroni adjustment, P ≤ .002). RESULTS: A high risk of finding agenesis of the maxillary lateral incisors, supernumerary teeth, microdontia of the maxillary lateral incisors, and rotation of the maxillary central incisors adjacent to the cleft (P < .0001) was observed in the patients with NSCLP. One or more dental anomalies were found in 98% of patients with BCLP, in 96% of those with LCLP, and in 87% of those with RCLP. Most of the anomalies were located on the cleft area. The incidence relative risk (IRR) of anomalies was highest in patients with BCLP (IRR: 10.5; 95% confidence interval [CI]: 6.76-16.3), followed by in those with LCLP (IRR: 8.51; 95% CI: 5.64-12.8). CONCLUSIONS: Most dental anomalies were found in the cleft area; this was expected because the cleft area was the most affected in the patients included in this study.

Prevalence and Characteristics of Three-rooted Mandibular Molars in Saudi Population: A Retrospective Radiographic Analysis.

AIMS: To study the prevalence of three-rooted mandibular permanent first molars (PFM) among Saudi population in Al Zulfi. MATERIALS AND METHODS: Subjects were selected from the patients' records, College of Dentistry, Majmaah University, Al Zulfi, Kingdom of Saudi Arabia. Healthy patients with the presence of both mandibular PFM, orthopantomogram (OPG) and/or intraoral periapical (IOPA) radiographs were included in the study. All patients included were evaluated by a single examiner using OPG and IOPA radiographs. The occurrence of three roots, status of the tooth and associated anomalies were considered for analysis. Descriptive statistics performed using IBM SPSS (Version 21.0) at a 95% confidence interval (p < 0.05) while the Chi-square test and Fisher test were used for the incidence, and comparison of occurrence of the third root in PFM in the mandibular arch. RESULTS: A sample of 433 subjects was selected randomly based on our inclusion criteria. Only 24 (5.5%) subjects had evidence of three-rooted mandibular PFM with the mean age of 27.67 years. The frequency of bilateral and unilateral three-rooted mandibular PFM was 2.3% (10) and 3.2% (14), respectively. Overall 41.6% of subjects were witnessed with the bilateral occurrence and unilateral occurrence was 58.4% [(57% (8) were on the right and 42% (6) were left side] (p < 0.05). However, no significant relationship was observed between the left and right occurrence of three-rooted mandibular PFM (p < 0.05). Thirty-four (7.8%) PFM were presented with three roots and among them, 70% (24) received treatment. Dilacerations (12.5%), taurodontism (37%) and hypodontia (4%) were associated with three-rooted PFM in the mandibular arch. Kappa statistics showed excellent intra-examiner reliability (κ = 0.9). CONCLUSION: The prevalence of three-rooted PFM was 5.5% in the present study and unilateral occurrence is very common. Over 70% of these PFM received dental treatment in Saudi Arabia population. Always, IOPA radiographs are required to confirm the evidence of three-rooted mandibular PFM where three-dimensional imaging is not available. CLINICAL SIGNIFICANCE: An Eagle's eye of an endodontist on three-rooted PFM is of utmost importance in the treatment protocol when the tooth is planned for root canal therapy.

Does the Etiology of Pulp Necrosis Affect Regenerative Endodontic Treatment Outcomes? A Systematic Review and Meta-analyses.

OBJECTIVE: To evaluate if there is a connection between the causes of pulp necrosis (eg, caries, trauma, dental anomaly) and the success of regenerative endodontic treatment. METHODS: Electronic databases (PubMed, Scopus, Web of Science, Cochrane Central Register of Controlled Trials, Embase) were searched for studies on regenerative endodontic treatment, which used both clinical and radiographic evaluation of root maturation after at least 6 months of follow-up. The search terms "necrotic pulp", "regenerative endodontic treatment", "revascularization", and "revitalization" were combined using Boolean operators. The main journals on endodontics and dental traumatology were additionally hand-searched. Studies were included if they specified the causes of pulp necrosis. The primary question under review was, "Does the cause of pulp necrosis affect the outcome of regenerative endodontic treatment?" Other factors such as tooth type, intracanal medicament, irrigation protocol, use of a collagen matrix, and the type of scaffold were evaluated for possible relation with the outcome. The risk-of-bias assessment for randomized and nonrandomized studies was performed separately, using a modified Cochrane Collaboration's tool and risk of bias in non-randomized studies of interventions-I tool, respectively. Meta-analysis was performed, when possible, between studies comparing treatment outcomes of teeth whose pulp necrosis had different etiology. The search strategy yielded 1197 items. After screening, 18 studies reporting 445 regenerative endodontic treatment cases were included. RESULTS: The overall success rate for 274 teeth with trauma etiology was 94.8%, for 95 teeth with dens evaginatus etiology was 93.1%, and for 24 teeth with caries etiology was 96%. No significant difference was found between the results of regenerative endodontic treatment among teeth with trauma, dens evaginatus, and caries etiology (P = .055). Meta-analysis of studies comparing teeth with caries vs dens evaginatus and those with trauma vs caries confirmed that there was no evidence for difference in outcomes. CONCLUSION: Further randomized studies specifically testing such hypothesis are needed to confirm the preliminary results of this review.

Coincidence of Fusion and Concrescence in Mandibular Deciduous Incisors: A Case Report.

AIM: The aim of this paper is to report a rare case in which two dental anomalies in primary dentition coexisted-fusion and concrescence in a 4-year-old boy. This highlights the significance of the accurate early diagnosis of these dental anomalies. BACKGROUND: Odontogenic anomalies are frequently encountered in dental practice. These anomalies can occur due to abnormalities during the differentiation stage and lead to abnormalities in the hard tissue formation. Dental anomalies in number and form include fusion, gemination, and concrescence. CASE DESCRIPTION: Primary dentition with fusion between the right central and lateral primary incisors with increased mesiodistal width. Concrescence between the left central and lateral primary incisors with a groove through the incisal edge. Clinical observation along with radiographic evaluation using intraoral periapical (IOPA) radiographs and orthopantomogram (OPG) were used to arrive at a diagnosis. CONCLUSION: Early diagnosis of concrescence will reduce the risk of possible complications associated with any need of extraction later on. Monitoring the patient and long-term follow-up is required to manage the case with coexisting anomalies of true fusion and acquired concrescence. CLINICAL SIGNIFICANCE: A thorough history taking and clinical and radiographic evaluation of fusion and concrescence at an early stage result in an accurate diagnosis. A careful monitoring plan is a key to reduce the risk of possible complications later on.

Patients with SATB2-associated syndrome exhibiting multiple odontomas.

Special AT-rich sequence-binding protein 2 (SATB2)-associated syndrome (SAS) is characterized by alterations of SATB2. Its clinical features include intellectual disability and craniofacial abnormalities, such as cleft palate, dysmorphic features, and dental abnormalities. Here, we describe three previously undiagnosed, unrelated patients with SAS who exhibited dental abnormalities, including multiple odontomas. Although isolated odontomas are common, multiple odontomas are rare. Individuals in families 1 and 3 underwent whole-exome sequencing. Patient 2 and parents underwent targeted amplicon sequencing. On the basis of the hg19/GRCh37 reference and the RefSeq mRNA NM_001172517, respective heterozygous mutations were found and validated in Patients 1, 2, and 3: a splice-site mutation (chr2:g.200137396C > T, c.1741-1G > A), a nonsense mutation (chr2:g.200213750G > A, c.847C > T, p.R283*), and a frame-shift mutations (chr2:g.200188589_200188590del, c.1478_1479del, p.Q493Rfs*19). All mutations occurred de novo. The mutations in Patients 1 and 3 were novel; the mutation in Patient 2 has been described previously. Tooth mesenchymal cells derived from Patient 2 showed diminished SATB2 expression. Multiple odontomas were evident in the patients in this report; however, this has not been recognized previously as a SAS-associated phenotype. We propose that multiple odontomas be considered as an occasional manifestation of SAS.

Prevalence of an unusual hypoplastic defect of the permanent maxillary lateral incisor in great apes.

In this article, I describe a previously unreported maxillary lateral incisor defect (MLID) of the enamel in great apes and evaluate potential general causes (genetic, systemic stress, or localized disturbance), as well as examine differences in prevalence among the represented taxa. This defect occurred only on the labial surface of the maxillary lateral incisor and extended from the cervical-mesial quarter of the crown to the mesial edge of the cementoenamel junction (CEJ). The study sample consisted of 136 great ape specimens, including 41 gorillas, 25 chimpanzees, and 70 orangutans from the Smithsonian's National Museum of Natural History great ape collection. I used logistic regression to assess the prevalence of this defect in the sample and a binomial probability test for bilaterality. This defect of the maxillary lateral incisor is the second most common defect I observed in the study sample (30.1% of individuals affected), and was more likely to occur in individuals with linear enamel hypoplasia (LEH) and pit defects than those without these defects. Among specimens with both maxillary lateral incisors present, the defect was mostly bilateral. Pan and Pongo were significantly more likely to exhibit the defect than Gorilla. Between Pongo species, Pongo pygmaeus was significantly more likely to exhibit the defect than Pongo abelii. Between subspecies of Gorilla, although Gorilla gorilla gorilla exhibited the defect and Gorilla gorilla beringei did not, the difference was not significant. No sex differences were evident in this sample. The prevalence of this defect indicates it is not hereditary. The bilateral trend indicates a systemic cause, although the high inter-tooth specificity suggests a local disturbance and a combination of both is possible.

A Longitudinal Study of the Presence of Dental Anomalies in the Primary and Permanent Dentitions of Cleft Lip and/or Palate Patients.

OBJECTIVE: The aim is to survey primary and permanent dental anomalies: hypodontia, microdontia, a supernumerary tooth, and fused teeth in patients with cleft lip and/or palate. DESIGN: Retrospective longitudinal study Subjects : The subjects were selected from all 1724 patients with cleft lip and/or palate who were registered at the orthodontic clinic of Kyushu University Hospital, Fukuoka, Japan, from 1970 to 2009. Finally, 994 subjects were evaluated for primary dentition, 1352 for permanent dentition, and 871 for the longitudinal changes from primary to permanent dentition. METHODS: The prevalence of dental anomalies was compared for each tooth type, among various cleft types, between males and females, and between the alveolar cleft area and the noncleft area. RESULTS: The prevalence of hypodontia was 16.2% for primary dentition and 52.7% for permanent dentition in the subjects with cleft lip and/or palate. Hypodontia increased with the severity of the cleft type. Multiple hypodontia was found more frequently in the subjects with bilateral cleft lip and palate and the subjects with unilateral cleft lip and palate. Microformed lateral incisors were found in 22.7% of permanent lateral incisors but not in primary dentition. Supernumerary teeth were found in 17.7% of the subjects with cleft lip and/or palate for primary maxillary dentition and in 5.7% for permanent maxillary dentition. CONCLUSION: The prevalence of hypodontia was greater in permanent dentition than in primary dentition; although, it was not much different between males and females or between the right and left sides. The prevalence of dental anomalies was significantly different among four groups by cleft type: cleft lip, cleft lip and alveolus, cleft lip and palate, and cleft palate.

Dental Anomalies in a Brazilian Cleft Population.

OBJECTIVE: The aim of this study was to radiographically investigate the prevalence of dental anomalies outside the cleft area in a group of Brazilian patients with nonsyndromic cleft lip and/or palate (NSCL/P). DESIGN, PARTICIPANTS, AND SETTING: A retrospective analysis of 207 panoramic radiographs of patients with NSCL/P aged 12 to 45 years without history of tooth extraction and orthodontic treatment was performed. RESULTS: Dental anomalies were found in 75.4% of the patients, and tooth agenesis (29.2%) and supernumerary tooth (2.6%) were the most common anomalies. The risk of agenesis was higher among the individuals with cleft palate (CP) compared with individuals with cleft lip (CL) and cleft lip and palate (CLP) (agenesis: CP versus CL: odds ratio 6.27, 95% confidence interval 2.21-17.8, P = .0003; CP versus CLP: odds ratio 2.94; 95% confidence interval 1.27-6.81, P = .01). The frequency of dental agenesis was higher in patients with unilateral complete CLP (agenesis: P < .0001), incomplete bilateral CLP (agenesis: P = .0013), complete CP (agenesis: P < .0001), and incomplete CP (agenesis: P < .0001). The frequency of supernumerary teeth was higher in patients with bilateral complete CLP (P < .0001). The frequency of dental agenesis (P < .0001) and ectopic tooth (P = .009) was higher than the frequency estimated for general population. CONCLUSIONS: The prevalence of dental anomalies in patients with NSCL/P was higher than that reported in overall population. This study found preferential associations between dental anomalies and specific extensions of NSCL/P, suggesting that dental agenesis and ectopic tooth may be part of oral cleft subphenotypes.

Rare dental manifestation in Simpson-Golabi-Behmel syndrome.

Simpson-Golabi-Behmel syndrome (SGBS) is a rare X-linked recessive overgrowth disorder with prominent craniofacial manifestations. Macrodontia is also an uncommon dental anomaly that can be an isolated finding and has been associated with numerous systemic conditions and syndromes. This case report describes this previously unreported dental anomaly, macrodontia, in a patient with SGBS, which may broaden the phenotype of this syndrome. A brief review of the literature on orofacial findings associated with SGBS is also presented.

Maxillary dental anomalies in patients with cleft lip and palate: a cone beam computed tomography study.

OBJECTIVE: To compare the frequency of maxillary dental anomalies in patients affected by unilateral (UCLP) and bilateral (BCLP) cleft lip with palate and to determine whether statistical differences were present or not between cleft and normal sides in UCLP group by using cone beam computed tomography (CBCT). In addition, the frequency of those dental anomalies was compared with previous studies presenting the same population without cleft Study Design: Fifty non-syndromic patients affected by UCLP (28 patients) and BCLP (22 patients) were selected for analysis of dental anomalies by means of CBCT. The frequency of maxillary dental anomalies including tooth agenesis, microdontia of lateral incisor, ectopic eruption and impaction of canine and supernumerary tooth were examined. Pearson chi-square and Fisher's exact tests were performed for statistical comparisons. RESULTS: All patients affected by UCLP and BCLP were found to have at least one maxillary dental anomaly. The most frequently observed dental anomaly was tooth agenesis (92.5% and 86.4%, respectively) in UCLP and BCLP groups. Tooth agenesis and canine impaction were observed more commonly in the cleft side (75.0% and 35.7%, respectively) than in the normal side (57.1% and 14.3%, respectively) in UCLP group (p≯0.05). All dental anomalies were found to be higher in both cleft groups than in general populations not affected by cleft. CONCLUSION: Since patients affected by UCLP and BCLP had at least one dental anomaly and higher dental anomaly frequency as compared to patients without cleft, those patients should be examined carefully prior to orthodontic treatment.

PHACES syndrome and multi-regional odontodysplasia: a case report.

PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as "ghost teeth", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child's dentition as it develops.