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A molar-incisor malformation (MIM) is a newly discovered type of dental anomaly of the permanent first molars, deciduous second molars, and permanent maxillary central incisors. MIM anomalies of the permanent first molars and deciduous second molars may include normal crowns with a constricted cervical region and thin, narrow, and short roots, whereas the affected maxillary central incisors may exhibit a hypoplastic enamel notch near the cervical third of the clinical crown. Although the etiology of MIM remains to be determined, it is thought to be attributable to an epigenetic factor linked to brain- and central nervous system-related systemic diseases at around age 1 to 2 years. MIM teeth are associated with clinical problems such as impaction, early exfoliation, space loss, spontaneous pain, periapical abscess, and poor incisor esthetics. Children with MIM teeth should be observed closely with respect to their medical history, and dentists should formulate a wider-ranging treatment plan., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The clinical importance of variations of tooth number, size and shape is seen in many dental disciplines. Early diagnosis allows optimal patient management and treatment planning, with intervention at an appropriate time to prevent complications in development and so reduce later treatment need. Understanding the process of dental morphogenesis and the variations in outcomes is an important contribution to the multidisciplinary clinical team approach to treatment. Tooth number, size and shape are determined during the initiation and morphogenetic stages of odontogenesis. The molecular evidence of repetitive signalling throughout initiation and morphogenesis is reflected clinically in the association of anomalies of number, size and shape. This association has been statistically modelled from epidemiological evidence and confirmed by 2D and 3D measurement of human dental study casts. In individuals with hypodontia, the teeth that are formed are smaller than the population mean and often show reduced and simplified shape. In contrast, in individuals with supernumerary teeth, the other teeth are larger than average and may show an enhanced shape. Clinical observations in humans and studies of laboratory animals gave rise to the concept of morphogenetic fields within the dentition. The findings, which can also be considered as reflecting gene expression territories, have been developed to incorporate field, clone and homeobox theories. The clinical distribution of developmental anomalies tends to follow the pattern of these fields or territories. Improved care for patients with these anomalies will come not only from utilizing a multidisciplinary clinical team but also by expanding the approach to include other relevant scientific disciplines., (© 2014 Australian Dental Association.)

The purpose of this paper was to present the case of a two-year-old male diagnosed with Timothy syndrome who presented with generalized enamel defects in the primary dentition. Timothy syndrome is an autosomal dominant condition characterized by a de novo missense mutation in the Cav1.2 L-type calcium channel CACNA1C. Timothy syndrome patients present with multiple clinical manifestations, including: cardiac arrhythmias; syndactyly; immune deficiency; intermittent hypoglycemia; and neurologic issues, including seizures, mental retardation, hypotonia, and autism. Craniofacial abnormalities reported include: low-set ears; flat nasal bridge; small upper jaw; thin upper lip; round face; and baldness at birth. Abnormalities in the dentition have been reported, including small, misplaced teeth with poor enamel and severe caries. At present, there is no thorough description of the dental abnormalities seen in a patient with Timothy syndrome.

Tooth agenesis affects 20% of the world population, and maxillary lateral incisors agenesis (MLIA) is one of the most frequent subtypes, characterized by the absence of formation of deciduous or permanent lateral incisors. Odontogenesis is a complex mechanism regulated by sequential and reciprocal epithelial-mesenchymal interactions, controlled by activators and inhibitors involved in several pathways. Disturbances in these signaling cascades can lead to abnormalities in odontogenesis, resulting in alterations in the formation of the normal teeth number. Our aim was to study a large number of genes encoding either transcription factors or key components in signaling pathways shown to be involved in tooth odontogenesis. We selected 8 genes-MSX1, PAX9, AXIN2, EDA, SPRY2, TGFA, SPRY4, and WNT10A-and performed one of the largest case-control studies taking into account the number of genes and variants assessed, aiming at the identification of MLIA susceptibility factors. We show the involvement of PAX9, EDA, SPRY2, SPRY4, and WNT10A as risk factors for MLIA. Additionally, we uncovered 3 strong synergistic interactions between MLIA liability and MSX1-TGFA, AXIN2-TGFA, and SPRY2-SPRY4 gene pairs. We report the first evidence of the involvement of sprouty genes in MLIA susceptibility. This large study results in a better understanding of the genetic components and mechanisms underlying this trait.

Natal teeth erupt in utero, whereas neonatal teeth present during the first month of life. The lower central incisors are the most frequently affected teeth. The etiology is unknown, and the choice of treatment depends on several factors. The purpose of this paper is to review 23 cases of children with natal or neonatal teeth who attended a dental care program in a university maternal and infant oral health clinic. There was no gender predilection. Most were natal teeth (83 percent) and presented a mild degree of mobility (64 percent). Only two patients had breast-feeding difficulties (nine percent). Tooth maintenance was the most common treatment of choice (64 percent), and only two patients presented Riga-Fede disease (nine percent).

A comprehensive understanding of root and root canal morphology of primary teeth is valuable for successful endodontic therapy. A supernumerary root is a developmental anomaly which can affect any tooth. An extra root placed lingually (radix entomolaris) or buccally (radix paramolaris) can be seen in mandibular molars nonetheless; bilateral association of an additional root in deciduous mandibular molars is rare. This report aimed to describe the diagnosis, significance and management of an extra root in deciduous mandibular molars bilaterally in perspective of modern clinical paediatric endodontics.

Objective: To determine the prevalence of mesiodens in deciduous and mixed dentitions and its association with other dental anomalies., Material and Methods: Panoramic radiographs of 1,995 orthodontic patients were analyzed retrospectively, obtaining a final sample of 30 patients with mesiodens. The following aspects were analyzed: gender; number of mesiodens; proportion between erupted and non-erupted mesiodens; initial position of the supernumerary tooth; related complications; treatment plan accomplished; and associated dental anomalies. The frequency of dental anomalies in the sample was compared to reference values for the general population using the chi-square test, with a significance level set at 5%., Results: The prevalence of mesiodens was 1.5% more common among males (1.5:1). Most of the mesiodens were non-erupted (75%) and in a vertical position, facing the oral cavity. Extraction of the mesiodens was the most common treatment. The main complications associated with mesiodens were: delayed eruption of permanent incisors (34.28%) and midline diastema (28.57%). From all the dental anomalies analyzed, only the prevalence of maxillary lateral incisor agenesis was higher in comparison to the general population., Conclusions: There was a low prevalence of mesiodens (1.5%) in deciduous and mixed dentition and the condition was not associated with other dental anomalies, except for the maxillary lateral incisor agenesis.

Dentofacial injuries that occur prior to the eruption of primary teeth can result in developmental disturbances not only in the primary but also in permanent dentition. Here we report a rare case of long term sequelae of trauma in a female child of 4 to 5 months of age which resulted in dilaceration and impaction of maxillary primary central incisors and subsequent enamel hypoplasia of the permanent maxillary central incisors.

Purpose: The purpose of this study was to examine inter-relations between infraocclusion of primary mandibular molars, tipping of adjacent teeth, and alveolar bone height of infraoccluded teeth., Methods: A total of 402 periapical radiographs of infraoccluded primary mandibular molars were reviewed. Infraocclusion, alveolar bone height, and tipping of adjacent teeth were measured., Results: Infraocclusion was most prevalent among primary mandibular first molars. The amount of infraocclusion was most profound among primary mandibular second molars (2.79±1.25). The shortest distal alveolar crest (reflected by the highest distance between the cementoenamel junction and bone crest) was observed among infraoccluded primary mandibular first molars (0.9±0.92). The permanent mandibular first molar and the primary mandibular first molar demonstrated the maximum tipping mesially and distally., Conclusions: Infraocclusion of primary mandibular molars, tipping of adjacent teeth, and alveolar bone height of the infraoccluded teeth are inter-related.

The present study was designed to evaluate associations between developmental defects of enamel (DDE) in the primary dentition and aspects related to mothers and preschoolers in the city of Diamantina, Brazil. A case-control study was carried out involving children aged three to five years. The case group was composed of 104 children with at least one dental surface affected by DDE. The control group comprised 105 children without DDE, matched for gender and age. The diagnosis of enamel defects was performed using the Developmental Defects of Enamel Index. Information was collected through interviews investigating socio-demographic aspects, gestation, birth weight, prematurity and breastfeeding. Simple and multiple regression analyses were performed, providing unadjusted and adjusted prevalence ratios (Poisson regression). DDE were more prevalent among children who had not been breastfed (PR=1.57; 95% CI: 1.1-2.2) and those whose mothers were under 24 years of age at the birth of the child (PR=1.41; 95% CI: 1.1-1.9). The prevalence of DDE in the primary dentition was higher among children who had not been breastfed and those whose mothers were under 24 years of age at the birth of the child.

Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathy, characterized by the disturbances in calcium and phosphorous metabolism, owing to deficiency in parathyroid hormone, which leads to tetanic manifestations. Onset of the clinical features occurs early in life, and the severity depends on the extent of chemical imbalance. This article describes a case of 22-year-old patient undiagnosed for 12 years with this endocrinopathy (IHP). Over retained deciduous teeth, delayed eruption, impacted tooth and short roots probably resulting from untreated hypocalcemia during the developmental phase of dentition enabled us to unearth this endocrinopathy through a series of investigations. Thus the article emphasizes the importance of dental findings of this endocrinopathy.

Developmental enamel defects, presenting as enamel hypoplasia or opacities are caused by damage or disruption to the developing enamel organ as a result of inherited and acquired systemic conditions. The high prevalence of these defects in the primary dentition demonstrates the vulnerability of the teeth to changes in the pre- and postnatal environment. The presence of enamel hypoplasia increases the risk of primary teeth to early childhood caries and tooth wear as the defective enamel is thinner, more plaque retentive and less resistant to dissolution in acid compared to normal enamel. The purpose of this paper was to critically review the aetiology and clinical complications of developmental enamel defects in the primary dentition and propose recommendations for the clinical management of affected teeth., (© 2013 Australian Dental Association.)

Aim: This study compared enamel defects in children born prematurely and at term., Method: 96 children born at term (G1), and with 96 children born prematurely (G2) were studied., Results: A higher prevalence of enamel defects was found in the premature group, with a predominance of hypoplasia. In G1, 64 teeth displayed enamel defects (51 opacities and 13 hypoplasias). In G2, 110 defective teeth were found, (29 opacities and 81 hypoplasias). A significant correlation was found between very low birth weight (VLBW) and the presence of these defects (p < or = 0.001). The teeth most affected were the incisors, canines and molars., Conclusion: Prematurity, in conjunction with other factors, can predispose children to enamel defects.

BACKGROUND.  Hypophosphatasia (HP) is characterized by defective mineralization of bone and teeth because of deficient alkaline phosphatase activity. There are generally six recognized clinical forms, of which the most severe is often lethal prenatally or early in life. In milder forms, such as odontohypophosphatasia (OHP), premature exfoliation of primary teeth may be the only clinical manifestation. CASE REPORT.  A 20-month-old girl was referred to the Specialist Paediatric Salaried Dental Service within the Harrogate and District NHS Foundation Trust with mobility of tooth numbers 71 and 81. Clinical examination revealed grade III mobile 71 and 81, with minimal gingival inflammation and plaque deposits. There were no other dental findings and no significant medical history. Tooth numbers 71 and 81 exfoliated prematurely with no evidence of root resorption, shortly after presentation. Haematological and urinary investigations showed no abnormalities. Histological examination showed a complete absence of cementum. A diagnosis of OHP was made. After 10 months of dental follow-up, no further teeth have increased mobility. CONCLUSION.  Odontohypophosphatasia should be included as a differential diagnosis in children presenting with early loss of primary teeth. The dentist may be the first health care professional to whom the patient presents., (© 2012 John Wiley & Sons Ltd, BSPD and IAPD.)

Background:   Studies on the prevalence of enamel defects in the primary dentition as a whole are scarce, as most investigations examine specific population groups., Objectives:   The aim of this study was to evaluate the prevalence of enamel defects in primary teeth and determine whether prematurity, birthweight, and socio-demographic variables are associated with such defects., Design:   A cross-sectional study was carried out with 381 children aged 3-5 years. Data were collected through clinical examinations and interviews with parents during the National Immunisation Day. The diagnosis of enamel defects was performed using the Developmental Defects of Enamel (DDE) Index. Through interviews, information was collected on socio-demographic aspects, pregnancy, birthweight, prematurity, and breastfeeding. Statistical analysis was performed using the SPSS program for Windows and involved descriptive analysis, Fisher's exact test, the chi-square test, and Poisson regression., Results:   The prevalence of developmental defects of enamel was 29.9%. Demarcated opacity was the most frequent type of defect. Children with a history of very low birthweight had a greater prevalence of enamels defects (PR, 2.7; 95% CI, 1.66-4.61). Prematurity and socio-demographic variables were not associated with enamel defects., Conclusion:   Children with a history of very low birthweight had a greater frequency of enamel defects in primary teeth., (© 2012 John Wiley & Sons Ltd, BSPD and IAPD.)

Objective: The mandibular molars typically have two roots placed mesiodistally, but they occasionally have an additional distolingual (DL) root. This study was to determine the morphological characteristics of the crown of such mandibular molars., Design: Radiographic records and study models were collected from a Korean population (n=86 patients). Each molar was assigned to either the experimental group (i.e. with a DL root) or the control group (i.e. without a DL root; n=41 patients) based on the radiographic findings. The intercuspal distances of the first permanent molars (total, n=100; control/experimental, n=50/50) and primary second molars (46, 23/23), and the largest buccolingual/mesiodistal widths of those molars and primary first molars (42, 21/21) were measured for molars with and without a DL root. In addition, the correlation between the existence of a sixth cusp and a DL root was examined., Results: The crowns of first permanent and primary second molars with DL roots had significantly larger intercuspal distances between the distobuccal-distolingual cusp tips and a larger distal-area buccolingual width than those without the DL root (t-test; p<0.05). There was no significant correlation between the existence of a sixth cusp and the presence of a DL root., Conclusions: The existence of a DL root was associated with larger buccolingual dimensions, especially in the distal area., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Fusion has been described as a developmental anomaly characterized by the union of two adjacent teeth. Few cases of this rare anomaly in primary dentition have been reported in Indian population. A rare case of bilateral fusion between primary mandibular lateral incisors and canines is presented in this report.

Purpose: The fusion of primary teeth may be associated with the absence of 1 of the 2 permanent successors. Moreover, even if both successors erupt, developmental disturbances such as microdontia or delayed tooth formation may occur. The purpose of this study was to elucidate the effects of different combinations of fused primary teeth on the eruption of permanent successors., Methods: One hundred ninety-seven children with 247 fused primary teeth were examined. Combinations of primary teeth involved in the fusion were identified, and the effects of these different combinations on the presence, morphology, and eruption of the permanent successors were determined., Results: Three types of fusion in the primary teeth were identified: (1) between the maxillary central and lateral incisors (UCI/LI); (2) between the mandibular central and lateral incisors (LCI/LI); and (3) between the mandibular lateral incisor and canine (LLI/C). The results revealed an absence of the successional lateral incisor in 65% of UCI/LI cases and 74% of LLI/C cases, whereas only 16% of LCI/LI cases resulted in a missing successor., Conclusions: Fused primary teeth are highly correlated with the absence of permanent teeth, and the prevalence depends on the combination of fused primary teeth.

The term double tooth is often used to describe fusion and gemination. The development of isolated large or joined teeth is not rare, but the literature is confusing when the appropriate terminology is presented. The objective of this paper is to present a case of a primary double tooth in a 5-year-old girl with a history of trauma. The tooth was endodontically treated and esthetic management was carried out using a silicone putty guide.

Tooth agenesis is one of the most intriguing phenomena, because it is frequently associated with other oral anomalies, structural variations and malformations of other teeth, late eruption, transposition and crowding. The diagnosis can be quite challenging as radiographic examination is critical for the diagnosis but not always possible and the late developing teeth may be sometimes scored developmentally missing. Accurate diagnosis therefore requires radiographic, clinical, and dental cast examinations. It is an important clinical and public health problem. Patients with missing permanent teeth may suffer from a reduced chewing ability, inarticulate pronunciation, and an unfavorable aesthetic appearance. Clinically, early diagnosis of a dental anomaly can alert the clinician to the possible development of other associated dental anomalies in the same patient or family, and avoid the possible sequelae. Understanding of tooth agenesis patterns and their impact on diagnosis, prevention, and eventually therapeutics are becoming integral parts of comprehensive dental care. Dental examination with radiographic screening of hypodontia in early childhood should be emphasized as part of public oral health policy to allow early diagnosis and timely intervention.

Background: Prune belly syndrome is a rare condition produced by an early mesodermal defect that causes abdominal abnormalities. However, the literature indicates that disturbances related to ectodermal development may also be present. This is the first case report in the literature to suggest that dental abnormalities are part of the broad spectrum of clinical features of prune belly syndrome. Because the syndrome causes many serious medical problems, early diagnosis of abnormalities involving the primary and permanent dentitions are encouraged., Case Presentation: The authors report the clinical case of a 4-year-old Caucasian boy with prune belly syndrome. In addition to the triad of abdominal muscle deficiency, abnormalities of the gastrointestinal and urinary tracts, and cryptorchidism, a geminated mandibular right central incisor, agenesis of a mandibular permanent left incisor, and congenitally missing primary teeth (namely, the mandibular right and left lateral incisors) were noted., Conclusion: This original case report about prune belly syndrome highlights the possibility that dental abnormalities are a part of the broad spectrum of clinical features of the syndrome. Therefore, an accurate intra-oral clinical examination and radiographic evaluation are required for patients with this syndrome in order to provide an early diagnosis of abnormalities involving the primary and permanent dentitions.

Mucopolysaccharidosis type IIIB (MPS IIIB) is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme a-N-acetylglucosaminidase. Affected subjects present developmental delay, attention deficit disorder, uncontrollable hyperactivity, and aggressive behavior, followed by progressive dementia and death in late adolescence. The purpose of this paper is to report the dental findings and treatment in a child with MPS IIIB. His primary molars and permanent mandibular incisors presented obliterated pulp chambers and root canals, which may be a clinical manifestation of this disorder.

Concomitant hypo-hyperdontia is a rare condition of coexisting missing teeth and supernumerary teeth in the same subject. Its etiology is still not exactly known. Permanent dentition is usually affected. Presented here are two uncommon cases of bimaxillary concomitant hypo-hyperdontia during the mixed dentition period. Early diagnosis of the condition and multidisciplinary approach for management of such cases is recommended.

Congenital Rubella Syndrome is a rare disorder comprised of a constellation of physical abnormalities that develop in infants as a result of maternal infection and subsequent fetal infection with rubella virus. The congenital lesions involve vital organs such as heart, eye, ear, brain and endocrine system and less frequently, teeth. The severity of systemic involvement depends on the stage of gestation at which maternal rubella infection occurs. With the implementation of immunization programs worldwide, its incidence has been dramatically reduced during the past half century. This article provides an insight into the prolonged effect of the virus on ameloblasts by highlighting the presence of hypoplastic enamel in primary teeth and erupting permanent teeth in a female child diagnosed with congenital rubella syndrome.

The dental literature contains a small number of reports of primary multi-rooted anomalies. Among these reports the majority are regarding three-rooted primary mandibular second molar anomalies. The presence of bilateral three-rooted primary mandibular first and second molars is uncommon. There have been no reports of three-rooted primary molars from India. Here, we present a case of an 8-year-old boy with bilateral three-rooted primary mandibular first and second molars.

Impaction of primary anterior teeth is very rare. A tooth that fails to erupt into normal functional position by the time it normally should is considered impacted. A rare case of impacted dilacerated maxillary primary incisors in a 3-year-old child is presented. The diagnostic position of impacted primary incisors was determined using conical beam computed tomography (CBCT) to assist in surgical intervention with least surgical trauma to the tissues.

Described for the first time in 1971, Schimke immuno-osseous dysplasia (SIOD) is an autosomal-recessive multisystem disorder that is caused by bi-allelic mutations of SMARCAL1, which encodes a DNA annealing helicase. To define better the dental anomalies of SIOD, we reviewed the records from SIOD patients with identified bi-allelic SMARCAL1 mutations, and we found that 66.0% had microdontia, hypodontia, or malformed deciduous and permanent molars. Immunohistochemical analyses showed expression of SMARCAL1 in all developing teeth, raising the possibility that the malformations are cell-autonomous consequences of SMARCAL1 deficiency. We also found that stimulation of cultured skin fibroblasts from SIOD patients with the tooth morphogens WNT3A, BMP4, and TGFβ1 identified altered transcriptional responses, raising the hypothesis that the dental malformations arise in part from altered responses to developmental morphogens. To the best of our knowledge, this is the first systematic study of the dental anomalies associated with SIOD.

Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive skeletal dysplasia associated with myotonia. The manifestations of SJS include short stature, blepharophimosis, and skeletal anomalies. The combination of skeletal and muscular abnormalities may result in oro-dental manifestations such as atypical facies, with micrognathia, microstomia, pursed lips, crossbite, cleft palate, as well as mandibular hypoplasia, the risk of dentigerous cysts, and impacted teeth. The use of general anesthesia in patients with SJS is dangerous, as there is a risk of malignant hyperthermia. The purpose of this paper is to describe a 3-year-old Chinese boy diagnosed with SJS at birth. His intra-oral examination revealed significant dental findings such as dentin defects with generalized attrition and hypodontia of the permanent dentition. Comprehensive dental treatment was provided at the same time as lower right limb surgery to reduce the potential complications with general anesthesia., (© 2012 Special Care Dentistry Association and Wiley Periodicals, Inc.)

Aim: The purpose of this article is to report some unusual characteristics related to gender, location and manifestations of severe Regional Odontodysplasia detected in three subjects. Regional Odontodysplasia is a rare disturbance of dental development whose aetiology is still unknown. Anomalies involve enamel, dentin, pulp and dental follicle causing atypical structure, colour, shape, size and eruptive disturbances of the affected teeth. Its early onset may lead to craniofacial development disturbances., Cases Reports: This article reports three cases with unusual characteristics observed in male children who were assisted by a paediatric dentist. Generally the disease affects one hemiarch and it is very rare that it crosses the midline as in one of the presented cases. Regional Odontodysplasia has been predominantly described in the maxilla and in women, however these three cases are in boys and two of them occured in the mandible., Conclusion: Regional Odontodysplasia is a rare disease, causing severe dental, growing and craniofacial development anomalies. Treatment needs to be personalised, aiming at preservation of the affected teeth taking into account their risk to develop severe infections. Parents should be made aware of the need for an extensive follow-up.

The purpose of this article is to report the orthodontic treatment of a patient with extremely delayed development of the maxillary lateral incisors. At 7 years of age, the boy's permanent maxillary lateral incisors had not erupted. A radiograph showed no tooth germs in place, although well-defined radiolucent areas were evident. Removal of the radiolucent areas was contemplated, but it was rejected in favor of a conservative approach. At age 13, peg-shaped maxillary lateral incisors erupted; they were positioned during orthodontic treatment and reshaped with composite restorations, providing good esthetics and function., (Copyright © 2012 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.)

Infantile Refsum disease (IRD) is a peroxisome biogenesis disorder (PBD), and is part of a larger group of diseases called leukodystrophies, which are inherited conditions that damage the white matter of the brain and affect motor movements. Multiple signs and symptoms of IRD begin in infancy and progress through early childhood, including hearing and visual impairment, intellectual and growth impairment, seizures, liver involvement, and orofacial and dental abnormalities. This paper presents a case history of a 12-year-old female patient with IRD who underwent dental rehabilitation in the operating room under general anesthesia and includes a 2-year follow-up. Medical, dental, and management considerations in the care of this child's condition are presented. This paper also discusses the importance of a multidisciplinary approach in the management of children with special needs., (© 2012 Special Care Dentistry Association and Wiley Periodicals, Inc.)

This study was embedded in the Generation R Study, a population-based prospective cohort study from fetal life until young adulthood. This study focused on the relationship between Deciduous Molar Hypomineralization (DMH) and Molar Incisor Hypomineralization (MIH). First permanent molars develop during a period similar to that of second primary molars, with possible comparable risk factors for hypomineralization. Children with DMH have a greater risk of developing MIH. Clinical photographs of clean, moist teeth were taken with an intra-oral camera in 6,161 children (49.8% girls; mean age 74.3 mos, SD ± 5.8). First permanent molars and second primary molars were scored with respect to DMH or MIH. The prevalence of DMH and MIH was 9.0% and 8.7% at child level, and 4.0% and 5.4% at tooth level. The Odds Ratio for MIH based on DMH was 4.4 (95% CI, 3.1-6.4). The relationship between the occurrence of DMH and MIH suggests a shared cause and indicates that, clinically, DMH can be used as a predictor for MIH.

Aim: Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the cranium and face. It is not known whether the centrally located permanent incisor is always preceded by a centrally located primary incisor. The aim was to analyse whether a permanent single central incisor in SMMCI is always preceded by a primary single central incisor and to study extra- and intraoral phenotypic traits of the condition., Study Design: cross-sectional radiographic study of 11 children, visual analysis of photos and dental and panoramic radiographs., Results: Nine of the 11 cases exhibited a primary SMMCI with one symmetrical crown and root. Two cases exhibited two separate primary central incisor crowns with fused roots. The phenotypical traits (indistinct philtrum, lack of normal upper lip contour, missing superior labial frenulum and distinct mid-palatal ridge) were findings observed in young children with a primary SMMCI., Conclusion: The present study concludes and stresses the necessity of diagnosing of the SMMCI condition early in life. Furthermore, paediatric dentists are recommended to be aware of the condition and to refer these patients to interdisciplinary diagnostics and treatment.

In the primary dentition, traumatic injuries affecting the tooth-supporting structures are common due to increased bone resilience in children. Crown-root fracture, defined as a fracture involving enamel, dentin, and cementum, is uncommon in the primary dentition, comprising only 2% of dental traumas. This article reports the treatment and follow-up of a 2-year-old boy who suffered a traumatic crown-root fracture involving a primary anterior incisor that was fused to a supernumerary tooth.

Knowledge regarding the morphological variations in roots and root canals aids in successful dental treatment. However, literature regarding dysmorphology of roots in primary dentition is sparse. The purpose of this article is to present a case of bilateral primary mandibular first molars with an unusual morphology of single root. Review of literature regarding variations in root number in primary dentition has been discussed in this article. The etiology for occurrence of such variations has also been briefly reviewed.

Purpose: Preterm children may not be prepared for extra-uterine life and thus need neonatal intensive care, such as artificial ventilation through orotracheal intubation. Oral tissue development changes as a result of the use of an orotracheal tube and its mechanical influence in preterm neonates has not been intensively studied. The purpose of the present study was to determine the association of orotracheal intubation with incidence of oral alterations in preterm infants., Materials and Methods: A retrospective cohort of 117 subjects who were born during the period 2002-2003 in Brazil were evaluated and the data were analysed using the Fisher and Mann-Whitney exact tests. A 95% confidence interval was calculated for observed oral conditions., Results: The cohort revealed that the most frequent oral conditions included anterior crossbite, superior alveolar contour and palatal deformation., Conclusions: The association between the intubated-infant group and the non-intubated-infant group suggested that mechanical trauma had an influence on the oral structure development.

Fusion occurs infrequently, but could cause esthetic, spacing, and periodontal problems. An understanding of fused teeth and their associated problems is important to provide prophylactic measures, thereby preventing or minimizing possible complications. The purpose of this case report was to highlight the infrequency of fused primary teeth by virtue of the number of such cases reported in the literature. Six cases of asymptomatic dental twinning anomalies in the primary dentition are reported in this study, and a clinical and radiographic presentation of the cases of fusion of primary incisor teeth is illustrated. This clinical entity has been found to appear with varied clinical and radiographic appearances. All cases are different from each other. This study's fourth patient is of particular interest because he is the second known case of bilateral fusion of the primary mandibular central and lateral incisors to be reported in the literature. An association of fusion of primary incisors with the number of succedaneous teeth was observed.

This article reports the case of a boy born with 11 natal teeth who had many alterations in his permanent dentition. In this case, 22 teeth were missing and a shape anomaly was detected in eight teeth. The treatment consisted of prosthetic rehabilitation and follow-up of teeth eruption and jaw growth.

Aim: To determine if the presence of developmental enamel defects (DED) in the primary dentition is a risk indicator for the presence of DED in the permanent dentition in children with mixed dentition, as well as others factors., Materials and Methods: A cross-sectional study was undertaken in 1296 school children ages six to 72 years. The DED [FDI; 1982] in both dentitions were identified by means of an oral exam scoring enamel opacities [classified as demarcated or diffused], and enamel hypoplasia. Sociodemographic and socioeconomic variables were collected through a questionnaire. Socioeconomic status (SES) was determined based on the occupation and maximum level of education of parents. Statistical analysis included logistic regression., Results: Mean age of participants was 8.40 +/- 1.68; 51.6% were boys. DED prevalence was 7.5% in the permanent dentition and 10.0% in the primary dentition. The logistic regression model, adjusting for sociodemographic and socioeconomic variables, showed that for each primary tooth with DED, the odds of observing DED in the permanent dentition increased 7.38 times [95% CI = 1.17-1.64; p < 0.001]., Conclusion: An association between DED presence in both permanent and primary dentitions was observed. Further studies are necessary to fully characterise such relationship.

This case report describes the presence of a talon cusp in a right primary incisor in a 6-year-old girl. It is a rare entity with only three cases having been reported from the Indian population. The talon cusp showed presence of wear facets along with altered morphology of the involved tooth. No treatment was done apart from sealing the cusp-tooth interface with fissure sealant.

Regional odontodysplasia (RO) is an uncommon, nonhereditary, odontogenic developmental disturbance characterized by hypoplasia and hypocalcification of the dental tissues that produce so-called "ghost teeth." This report describes a case of a 2.5-year-old girl who came to the clinic with RO affecting her right maxillary arch. The distinguishing characteristics of this case were the involvement of both the primary and permanent dentitions and the early occurrence of odontogenic abscesses that required the patient's hospitalization. Ultrastructural analysis revealed dental tissue failures that compromised the integrity of the involved teeth, justifying the high susceptibility to caries that was clinically observed. Follow-up was characterized by periodic prosthetic adjustments to maintain the patient's ability to masticate and for social interaction, beyond allowing normal development of her maxillofacial complex. Patients with RO require individualized treatment planning and close follow-up with a multidisciplinary approach.

Purpose: The purpose of this study was to compare developmental defects of enamel (DDE) in the primary and permanent dentitions of children from a low-fluoride district., Methods: A total of 517 healthy schoolchildren were examined using the modified DDE criteria., Results: The prevalence of DDE in the primary and permanent dentition was 25% and 58%, respectively (P<.001). The mean number of teeth with enamel opacity per subject was approximately threefold compared to that affected by enamel hypoplasia (3.1±3.8 vs 0.8±1.4, P<.001 in the primary dentition and 3.6±4.7 vs 1.2±2.2, P<.001 in the permanent dentition). Demarcated opacities (83%) were predominant compared to diffuse opacities (17%), while missing enamel was the most common type of enamel hypoplasia (50%), followed by grooves (31%) and enamel pits (19%) (P=.04). In the permanent dentition, diffuse and demarcated opacities were equally frequent, while enamel grooves were the commonest type of hypoplasia (52%), followed by missing enamel (35%) and enamel pits (5%; P<.001)., Conclusions: In a low-fluoride community, developmental defects of enamel were twice as common in the permanent dentition vs the primary dentition. In the primary dentition, the predominant defects were demarcated opacities and missing enamel, while in the permanent dentition, the defects were more variable.

The purpose of this paper was to present the case of a 15-year-old female diagnosed with infantile Refsum disease (IRD) that presented with generalized enamel defects in the primary and permanent dentition. IRD is an inherited autosomal recessive disorder characterized by aberrant peroxisome function. IRD patients present with multiple clinical manifestations, including: retinitis pigmentosa; nystagmus; sensorineural hearing loss; mental and developmental delays; neuromotor defects; and cerebral ataxia. Craniofacial abnormalities reported include: high forehead; hypoplastic supraorbital ridges; epicanthal folds; midface hypoplasia; and large anterior fontanelle. At present, there is only one known report of dental anomaly associated with this syndrome. This represents the first known reported case in the pediatric dental literature.

Odontomas are usually associated with permanent teeth and rarely with primary dentition. The consequences of their presence are several clinical complications. Presented here is a case where diagnosis and management of an odontome causing impaction of primary tooth is discussed thus emphasising need for alertness in a clinician using the aid of radiological examination for early detection and subsequent intervention.