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Determine the prevalence of pyramidal molars (PMs), associated tooth anomalies, and medical conditions (MC).Pantomograms from 10,168 subjects were searched for PMs.A total of 148 individuals (75.7 percent females), aged 9.7 to 18.9 years old, had PMs. Subjects with MCs (53.4 percent) had on average 17 percent more PMs (P=.0476) and 2.8 times greater odds of palatally displaced canines (P=.0309). The mean number of pyramidal and taurodont molars per subject was 2.9±1.4 (SD) and 3.1±1.7 (SD), respectively. In both sexes, 63.5 percent of all observed PMs were located in the maxilla and 78.5 percent of all PMs were second molars. Taurodont molars increased by 9.4 percent as other tooth anomalies increased by one, but decreased by 8.2 percent for each additional PM. Tooth anomalies associated with PMs were palatally displaced canines (17.6 percent) and short root anomaly of maxillary central incisors (6.1 percent). Delayed eruption was noted in 9.5 percent, tooth agenesis in 6.1 percent, and supernumerary teeth in 3.4 percent.The prevalence of pyramidal molars was 1.4 percent, and the female-to-male ratio was 3.1 to one. Pyramidal molars are often associated with medical conditions and other tooth anomalies.

The purpose of this study was to evaluate the association between gestational age status and the presence of dental anomalies in the permanent dentition.Dental and medical charts and panoramic radiographs of 340 seven- to 18-year-old subjects with a known history of premature birth were assessed retrospectively. The controls were 150 healthy children born at full term. The gestational ages of subjects were recorded and categorized as extremely premature, very premature, late premature, or full term. From the panoramic radiographs, dental anomalies were classified as shape, number, and positional. The total number of anomalies and the number of each type of anomaly was recorded.There was a statistically significant difference in the odds of having at least one anomaly according to gestational age (P=.04), with decreasing numbers of anomalies as gestational age increased. Subjects of younger gestational ages had significantly more shape anomalies (P.01); however, no such association occurred for number or positional types of anomalies (P.05).There was a significant association between gestational age status and shape anomalies in the permanent dentition. The number of anomalies increased as gestational age decreased. Subjects born at younger gestational ages had more shape anomalies compared to other types of anomalies.

The purpose of this study was to determine, from a large number of panoramic images taken in the clinic of a pediatric dentistry residency program, the prevalence of maxillary nine-year molars (9YM), associated medical conditions (MCs), and the presence of coexisting tooth anomalies (TAs).Pantomograms from 13,140 patients were searched for 9YM. Radiographs from identified subjects were examined for coexisting TAs, and charted MCs were noted. For identified 9YM, Nolla's stage of tooth formation was determined and the subjects' dental ages were calculated.Among 53 subjects (68 percent females), 21 unilateral and 32 bilateral cases of 9YM occurred. Females and males were of similar age (7.98 versus 7.91 years old). Overall dental and chronological ages were significantly different: dental development in bilateral cases was delayed by 4.1 months in females and 2.9 months in both sexes (P=.003). In unilateral cases, it was accelerated by 3.6 months in females and 3.5 months in males. The specific tooth age for all 9YM was 4.95 years for females and 5.56 years for males (P=.018). The average count of associated findings (e.g., missing teeth, peg laterals, other TAs, and other eruption delays) was 0.62 in unilateral and 1.44 in bilateral cases (P=.02). Commonly associated MCs included clefting (smallN/smallequals five), and Down syndrome (n equals three).The prevalence of nine-year molars was one in 248 (0.4 percent), and the average delay in dental development was 2.7 years.

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.

Dental transposition is the positional interchange of two adjacent teeth. Canine transpositions are usually accompanied by other dental anomalies, such as: impaction of the incisors; missing teeth; peg-shaped lateral incisors; severe rotation or malposition of adjacent teeth; dilacerations; and malformations. Local pathologic processes, such as tumors, cysts, retained primary canines, and supernumerary teeth, might be responsible for canine transposition. The purpose of this paper was to present a rare case of maxillary canine-to-maxillary incisor transposition in an 8-year-old girl. The patient presented with noneruption of the permanent maxillary left central incisor, and a radiographic examination revealed an impacted dilacerated incisor. The central incisor was extracted because the root was severely dilacerated. At the 3-year follow-up, an oral examination revealed that the canine had transposed to the extraction site. Through orthodontic traction, combined with reshaping of the tooth, the transposed canine was successfully positioned into the incisor position.

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.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.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.Fused primary teeth are highly correlated with the absence of permanent teeth, and the prevalence depends on the combination of fused primary teeth.

This paper presents the diagnosis and management of a rare case of bulbous exophytic malformation of three permanent mandibular incisors. An 8-year-old Caucasian girl presented with concerns on the appearance of these teeth. Medical history was noncontributory, and there was no recall of relevant past trauma. Upon clinical examination, 3 permanent mandibular incisors exhibited hard bulbous protuberances in the crown's middle and cervical areas. Radiographic examination assisted by dental computed tomography revealed that these were continuous with the tooth structure, contained no pulpal tissue, and the teeth displayed normal root development. They were excised and the teeth were restored with a thin composite facing. They remained asymptomatic with acceptable esthetics for 4 years. Scanning electron microscopy and histology of the biopsied hard tissue revealed a nonhomogeneous structure resembling anomalously intertwined enamel with predentin and dentin. The etiology of this disturbance of dental development remains unknown.

Radiotherapy treatment options can cause adverse dental sequelae, including xerostomia, dental radiation caries, abnormal tooth development, and osteoradionecrosis (ORN). Hyperbaric oxygen (HBO) therapy can be used prophylactically or therapeutically to treat or reduce the risk of ORN. The purpose of this paper was to describe a case involving a 5-year-old male with rhabdomyosarcoma of the left temporal fossa and a history of radiation therapy who presented with gross radiation caries and xerostomia. Full-mouth extractions of all primary teeth were performed under general anesthesia, with the patient receiving HBO therapy before and after the surgery. The child was monitored postoperatively, and healing occurred with minimal post-operative complications. Based on his results, it can be concluded that hyperbaric oxygen therapy is an effective supplement to consider when treating children who have undergone radiation therapy and require dental care.

The purpose of this report was to: summarize the care of a child between the ages of 12 to 16 years old born with Noonan-like central giant cell syndrome and unrelated common variable immune deficiency; provide information on the dental management of patients with Noonan's syndrome; and present a brief discussion of the recent associated genetic findings. A review of the common features of Noonan syndrome and Noonan-like central giant cell syndrome is also provided.

Axenfeld-Rieger syndrome is a rare genetic disorder characterized by ocular and dental abnormalities. This case demonstrates a comprehensive review of the syndrome's clinical and dental features and describes a case of a 13-year-old boy presenting with it. The patient showed severe hypodontia, microdontia, and short roots. Different treatment options are discussed. Early diagnosis and an interdisciplinary approach are necessary to provide the best short- and long-term treatment plans, as well as treatment and follow-up for individuals with the syndrome.

The purpose of this study was to investigate the prevalence of congenital anomolies in the primary dentition in preschool Taiwanese children compared to Caucasion populations.The study group comprised 2,611 children (1,442 boys ond 1,169 girls) between 2 and 6 years-old in the primary dentition phase using a random sampling technique. The presence of hypodontia, hyperdontia, and double teeth (fusion ond gemination) were recorded ond analyzed using Fisher's exact test.The prevalence of hypodontia was approximately 2%. Unilaterol missing teeth were more frequently observed than bilateral missing teeth. The primary mandibular right lateral incisor was the most frequently missing tooth. The prevalence af hyperdontia was less than 1%. All of the supernumerary teeth were located in the primary maxillary incisor area. The prevalence of double teeth was approximately 3%. All double teeth were located in the anterior region, and most of them were found in the mandibular lateral incisor and canine area. There were no differences in the prevalence of anomalies between the sexes.The prevalence of hypodontia and double teeth, but not hyperdontia, was significantly higher in Taiwanese children vs Caucasian children. Early detection of primary dental anomalies may help prevent oral disease in children.

4H syndrome/ADDH, a disease of the cerebral white matter, seems to be associated with delayed tooth eruption and other dental abnormalities, which so far could not be assessed conclusively-mainly because patients were too young. The aim of this study was to characterize these abnormalities in a sample of patients old enough for a reliable assessment.Three children, all diagnosed with 4H syndrome/ADDH, were followed from approximately 4 to 10 years of age and examined clinically and radiographically. In one case, a histopathological analysis supplemented these records.All 3 patients showed a generalized delay in eruption of the primary and permanent teeth, which culminated in complete retention of all primary maxillary central incisors. Permanent mandibular second premolars were missing in all children and permanent maxillary central incisors of 2 individuals exhibited a concave labial surface, while agenesis of the permanent maxillary lateral incisors and natal or neonatal teeth were observed in one patient.4H syndrome/ADDH seems to be associated with a delay in primary tooth eruption, complete retention of the primary maxillary central incisors, and shape abnormalities of the permanent maxillary central incisors, which otherwise are very rare. Therefore, a neurological examination would appear warranted when these peculiarities are encountered.

Seckel syndrome is a rare form of primordial dwarfism that is characterized by short stature, skeletal defects, mental retardation, and characteristic facial features such as microcephaly, micrognathia, and a bird-head appearance. Dental findings include hypodontia, enamel hypoplasia, crowding, and Class II malocclusion. The purpose of this paper was to report the case of a female patient with Seckel syndrome type II and describe her orodental manifestations. She presented with interesting dental findings, including gingival hyperplasia, recession and ulceration, significant crowding, and early exfoliation of the primary dentition with accelerated eruption of the permanent dentition. The patient received comprehensive dental care under general anesthesia, and hard and soft tissue samples were collected for histologic analysis. The patient was followed for over 3 years.

Solitary median maxillary central incisor syndrome (SMMCIS) is a rare anomaly that affects 1 in 50,000 live births. Of unknown etiology, SMMCIS is characterized by the presence of a single central incisor located on the maxillary midline and may be associated with developmental defects and systemic alterations. SMMCIS also is associated with short stature, mild forms of deviation in craniofacial morphology, and intellectual disability. The purposes of this paper were to: describe the clinical case of an 8-year-old boy with a permanent central incisor located at the midline in association with holoprosencephaly; and highlight the most important aspects related to diagnosis and treatment of solitary median maxillary central incisor syndrome.

This investigation sought to identify the common manifestations of KBG syndrome in the literature and to determine the major oral abnormalities in a 12-year-old Chinese boy.PUBMED search, using the keyword phrase "KBG syndrome," produced 20 articles. Due to the disparities in the quality of evaluations in the reports, any physical feature that was not discussed was assumed to be absent.Of the 54 cases, including the present case, 36 (67%) were males. Mental retardation or global developmental delay was reported in 42 (78%) cases, while 46 (85%) exhibited shortness of stature. Oral features occurred in 53 (98%) cases; macrodontia was present in 49 (96%) cases; the present case was the only one with hyperdontia and a talon cusp. Other features were craniofacial anomalies, followed by abnormalities of the nose, hand, mouth, eyes, eyebrows, philtrum, costovertebrae, ears, and, less frequently, low hairline and lower extremity abnormalities.Cardinal features of KBG syndrome include facial dysmorphism, short stature, skeletal anomalies, and mild developmental delay; intraorally, macrodontia of the maxillary central incisors occurs in most cases. This is the first known report of KBG syndrome in a Chinese subject and the only case with hyperdontia and a talon cusp.

Craniometaphysial dysplasia (CMD) is a rare disorder that mainly affects craniofacial bones. It is caused by mutations within a region of human homolog (Ankh) of the mouse progressive ankylosis (Ank) gene. ANK, together with other factors, regulates intracellular and extracellular levels of pyrophosphate/inorganic phosphate critical for maintaining mineral homeostasis. The systemic manifestations noted in CMD patients have been reported previously. The dental anomalies in CMD patients, however, have been minimally described in the dental literature. The purpose of this case report was to describe both systemic and dental manifestations of a 3 1/2-year-old child with craniometaphysial dysplasia. At the gross level, enamel discoloration and tooth malformations were observed in multiple primary teeth without obvious defects in the roots. Radiographic evidence of excess mineralization was noted on the primary maxillary second molars, limited to the mesial region of the crowns. The genetic and molecular effects of Ank/Ankh mutations are also discussed.

The purpose of this study was to investigate the oral and dental findings in children with Fanconi anemia (FA).The study included 26 FA patients who came to the hospital (Hacettepe University Faculty of Medicine, Pediatric Hematology Unit) from the central region of Anatolia (17 [65%] mole, 9 [35%] female; mean age = 10.0 +/- 5.2 years (range = 2-18; median = 9 years]). Oral and radiological examinations and salivary collection were performed at the Department of Pediatric Dentistry of Hacettepe University Faculty of Dentistry.Among 26 FA children: (a) 16 (62%) had never visited a dentist; (b) 6 (23%) had visited a dentist once; and (c) 4 (15%) had visited a dentist regularly. Furthermore: (a) only 5 children (19%) brushed their teeth regularly; (b) 7 (27%) had never brushed their teeth previously; and (c) the other 14 (54%) had brushed their teeth rarely. The prevalence of dental caries was 35% in this study's patients. Gingival examination revealed that 9 (35%) children had gingivitis and the other 17 (65%) had normal gingival health status. Examination of the oral cavity revealed that: (a) 3 children (12%) had a coated tongue; and (b) 1 (4%) had papillary atrophy. No leukoplakia or other precancerous lesion was detected in this patient group. Salivary flow rate was less than 0.7 ml/minute in 56% of the patients. No patients had a salivary pH less than 5. Salivary buffering capacity of less than 5, however, was detected in 5 patients (33%). Radiological evaluation revealed that the most common congenital dental abnormalities were: (1) microdontia (44%); (2) congenitally missing teeth (26%); (3) transposition (9%); and (4) supernumerary teeth (4%).These results demonstrate that poor oral hygiene, dental decay, gingivitis, and congenital dental abnormalities--including generalized microdontia, supernumerary teeth, transposition, and congenitally missing teeth--are common oral and dental findings in this group of Turkish children with Fanconi anemia.

Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in her face, trunk, back, legs, and fingers as well as abnormal hair distribution, alopecia on the vertex, and hypoplasia of eyebrows. During the dental examination, conical shaped-teeth and delayed dental eruption was evidenced. The microanalytical examination showed dentin without significant alterations in the mineralization except for hypermineralization in focal areas. In addition, a decrease in the enamel mineralization was observed.

The purpose of this article is to describe the clinical features of otodental syndrome. A 9-year-old boy presented with dental abnormalities that have been described for otodental syndrome. The characteristic findings included large bulbous crowns in canine and molar teeth of both dentitions, deep vertical enamel fissures separating the cusps of affected molars, and hypoplastic yellow areas on the labial surfaces of the canines. Radiographs revealed the abnormal molars to possibly be the product of fusion of multiple tooth buds. The pulp chambers appeared to be duplicated, and possibly a supernumerary tooth or complex odontoma is present.

The purpose of this descriptive clinical study was to determine the prevalence rates of taurodontism and learning disabilities in a sample of patients with Klinefelter syndrome.Questionnaires and dental radiographs of Klinefelter syndrome patients were obtained and analyzed using previously published methods. Prevalence rates were determined for taurodontism and learning disabilities in the sample population and compared to the general population. Statistical analyses included a Fisher's exact 2-sided test to compare the prevalence rates to that found in the general population and subsequent determination of the positive predictive value.Taurodontism was found in 75% of the 24 participants. Eighty-three percent of the participants reported having a learning disability. These rates are significantly higher than the general population, as reported in the literature.The positive predictive value for Klinefelter syndrome, given a male patient with taurodontism and a learning disability, is 84%. In this case, the dentist should recommend karyotyping to the patient, parent, or physician. This demonstrates how important it is for dentists to understand and assist physicians in the diagnosis of genetic disorders.

Mutations in the genes encoding fibrillin, an extracellular matrix protein involved in providing elastic properties to the connective tissues, may result in specific craniofacial and oral anomalies. A number of craniofacial (retrognathia, dolichocephaly, high palate) and dental (root deformity, pulp calcification) manifestations are considered pathognomic for the Marfan syndrome (MFS), a condition caused by congenital fibrillin-1 deficiency. Reports on similar features in congenital contractural arachnodactyly (CCA), caused by fibrillin-2 deficiency, support the hypothesis that fibrillin deficiency might result in a number of morphological anomalies by influencing tissue interaction during growth and development. Hence, clinical manifestations can be related to specific aspects of fibrillin deficiency pathogenesis, and may be adopted as diagnostic tools in the outlook for affected individuals.

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

Congenital contractural arachnodactyly (CCA) is an inherited disorder of connective tissue similar to Marfan's syndrome. The craniofacial and oral features of a young girl with CCA are described. The patient has the typical features of CCA as well as some additional dental anomalies which have not previously been reported with this syndrome. These include banded pitted enamel hypoplasia and hypomineralization, long, spindly tapered roots, and pulp canal obliteration with multiple pulp stones. Dentists must be aware of the clinical features of a patient's syndrome to determine whether there are implications for dental treatment such as a need for antibiotic prophylaxis. It is important to exclude Marfan's syndrome as a differential diagnosis for CCA because the former has more associated complications and a less favorable prognosis.

The acronym CHARGE refers to a non-random clustering of congenital malformations whose cause remains unknown. To date, the dental features of CHARGE association are not well known. A brief review of the literature and a case in a 10-year old boy with the CHARGE association are presented. The patient had multiple dental anomalies including congenital absence of teeth, ectopic eruption, submergence of primary molars and an odontogenic fibroma associated with an impacted permanent molar.

This investigation stemmed from preliminary clinical observations from a school dental clinic, which suggested that a proportion of clinically undetected, radiolucent lesions on radiographs may originate as pre-eruptive intracoronal dentin defects. This study investigated the prevalence of such defects in orthopantomograms from a group of children and young adults.A total of 1281 orthopantomograms with 11,767 unerupted permanent teeth were examined.The prevalence of intracoronal dentin defects was 3% by subjects, and 0.5% by teeth; the highest prevalence being noted in the maxillary and mandibular first permanent molars. Most of the lesions occurred singly, and nearly half had extended to greater than two-thirds the width of dentin thickness. Ectopic positioning was significantly associated with this lesion.Pre-eruptive intracoronal dentin defects occur at a significant prevalence rate. Increased awareness of this entity may improve diagnosis and allow early treatment.

Dens evaginatus is a disturbance in tooth formation that appears clinically as an accessory occlusal tuberculated cusp composed of enamel and dentin, usually enclosing pulp tissue. The tubercle often fractures or is worn away, with subsequent pulp exposure leading to pulpal inflammation, necrosis, and periapical inflammation. When this happens to an immature tooth, diagnosis may be complicated by the similarity in radiographic appearance of the periapical lesion and the dental follicle. The management of such teeth may be complicated because of their immaturity. A case report of multiple abraded dens evaginatus with pulp necrosis is presented that illustrates 1) the difficulty of diagnosing otherwise apparently healthy teeth associated with periapical lesions and sinus tracts, and 2) their management and treatment complications including recurrent infections and root fractures.

Facial, oral, and dental findings of an 11-year-old girl with XXX syndrome are reported. Clinical examination reveals midfacial hypoplasia, congenital absence of teeth, and solitary maxillary central incisors both in primary and permanent dentitions.

Kozlowski syndrome is the most common type of spondylometaphyseal dysplasia (SMD). It is characterized by short stature (130 to 150 cm), pectus carinatum, limited elbow and hip movement, mild bowleg deformity, and curvature of the spinal column. Children with Kozlowski dwarfism usually are not recognized at birth, since they have normal clinical features, weight, and size. This article reports the dental treatment and oral findings of a 14-year-old female patient with Kozlowski dwarfism.

The purpose of this study was to determine the prevalence of an uncommon lesion of the primary mandibular canines (primary canine hypoplasia), and its association with age, race, gender, dental caries, fluoridation status of the drinking water, breast-feeding, and geographic location. The study population comprised 2686 randomly selected children, black and white, 4 and 8 years old, who were enrolled in accredited private and public preschools and elementary schools in Mississippi. There were 1318 males and 1368 females, 1289 white children and 1397 black children, 1353 4-year-olds and 1333 8-year-olds. Results indicate that an association exists for race (P = 0.0001), gender (P = 0.01), and dental caries (P = 0.0001), but not for age (P = 0.07), geographic location (P = 0.73), fluoridation status of the water supply (P = 0.145), or breast-feeding (P = 0.392). The prevalence rate of primary canine hypoplasia was 33.2% for black children and 17.2% for white children. These data indicate that this lesion is significantly more prevalent in blacks than whites, and that teeth with this lesion have a greater probability of developing dental caries.

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

A two-year, 2-month old girl with Ehlers-Danlos syndrome Type VII is described. The patient presented with bleeding after tooth brushing, and exhibited microdontia and yellow discoloration of teeth. She had carious teeth, but did not exhibit periodontal disease, poor wound healing after extraction, radiographic evidences of pulp stones, or malformed roots. Microscopic examination of a decalcified section of an extracted tooth stained with HE demonstrated some inclusions in the dentin, around which collagen was either missing or scanty, as confirmed by counterstaining with van Gieson's solution. The sections of pulp stained with van Gieson's solution showed an abnormal pattern of fibrous tissue. Furthermore, the radiopacity of the dentin of this patient was significantly higher than that of the control dentin.