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Hypophosphatasia affecting the permanent dentition.

Reports on dental abnormalities in connection with hypophosphatasia almost exclusively describe changes in primary teeth. A 23-year-old man with hypophosphatasia, first diagnosed at the age of 8 months, is described; histologically and radiographically verified signs of the condition were present in the permanent dentition. The findings included a reduced level of the marginal alveolar bone supporting the upper central incisors, which had to be extracted. The molars displayed large coronal pulp chambers. Histologically, the upper incisors demonstrated abnormal root cementum, with areas of dentin resorption, as well as disturbances of the mineralization of the coronal dentin. The patient also had signs of abnormal root resorption of molars. The potential involvement of permanent teeth puts children with hypophosphatasia at risk of developing oral complications during adolescent and adult life.

Adult↗

Enamel defects in a case of Menkes' syndrome.

A patient affected by Menkes' syndrome presents some dental abnormalities, such as enamel defects, delayed eruption and a biconically shaped incisor. The deficient action of a cuproenzyme in this syndrome leads us to hypothesize an explanation of defective enamel formation.

Brain Diseases, Metabolic↗

Short root anomaly in a patient with severe short-limbed dwarfism.

A 17-year-old youth was referred complaining of missing mandibular incisors and mobile canine teeth. He was extremely short (99 cm), with shortening of both trunk and limbs. A radiological skeletal survey showed abnormal and unusual findings, and his teeth were found to have short, conical roots and obliterated pulp chambers. Despite extensive biochemical and endocrinological investigation, it was concluded that the findings were not consistent with any recognized skeletal dysplasia nor with any recognized syndrome affecting the oral and dental tissues.

Adolescent↗

Dento-alveolar abnormalities in oculomandibulodyscephaly (Hallermann-Streiff syndrome).

Oculomandibulodyscephaly (OMD) is characterized by anomalies of skull, mandible, skin and eyes and also by a proportionate nanism. Dental abnormalities are often observed but poorly defined. Examination of a 2-month-old infant that suffered from OMD, having died from an unrelated cause revealed premature eruption of several deciduous teeth, crowding of unerupted deciduous teeth and agenesis of all permanent teeth excepting the first permanent molars.

Hallermann's Syndrome↗

Displacement of maxillary premolar teeth in a filly.

Abnormalities of dental development such as hypoplasia of cementum, oligodontia, polydontia, enamel hypoplasia, brachygnathia, and prognathia are quite common in the horse. Abnormalities of eruption are less common and often associated with trauma. This case report describes the diagnosis and therapy for pre-eruption displacement of the maxillary left third and fourth premolar teeth resulting from previous facial trauma in a 1.5-year-old filly.

Animals↗

Dental abnormalities in children preparing for pediatric bone marrow transplantation.

The purpose of our study was to describe the types and frequencies of altered dental development in pediatric patients preparing for bone marrow transplantation (BMT). Retrospective review of the medical records and panoramic radiographs of all patients who underwent BMT at St Jude Children's Research Hospital between 1990 and 2000 for whom pre-BMT dental examination and panoramic radiography records were available. All patients were treated on institutional protocols. We recorded patient demographics and radiographic evidence of microdontia, hypodontia, taurodontia, root stunting, caries, enamel pearls, and pulpal calcifications. The 259 patients identified (150 male and 109 female) had a median age of 12.82 years (range, 3.18-25.93 years) at the time of BMT. In total, 203 were Caucasian, 38 were African-American, and 18 were of other races. In all, 150 (57.9%) had abnormal dentition. The most common dental abnormalities were caries (n=84), pulpal calcifications (n = 34), and dental extractions (n = 33). Developmental abnormalities occurred less frequently: taurodontia (n = 8), hypodontia (n = 10), microdontia (n = 11), and root stunting (n = 11). Dental abnormalities are prevalent in children undergoing BMT. Pre-transplant oral hygiene and dental examination should be standard care in order to minimize potential sites of infection.

Adolescent↗

Dental abnormalities and ectopic eruption in patients with isolated cleft palate.

The aim of this study was to evaluate the influence of cleft size and surgical treatment on the development of permanent teeth in patients with isolated cleft palate. The series comprised 109 children with isolated cleft palate, 70 girls and 39 boys, including 14 patients with Robin sequence. The patients were grouped according to the sagittal extent of the palatal cleft, measured on dental casts obtained before the primary palate surgery. Forty-six children were treated by one-stage palatoplasty, and 63 in two stages. The dentition was studied on orthopantomograms taken at 5, 8, 11, and 14 years of age. Congenitally missing permanent teeth (third molars excluded) were found in 33 subjects (30%). Children with large clefts had significantly more missing teeth than children with small clefts. The tooth most usually missing was the mandibular second premolar, followed by the maxillary lateral incisor, and the upper second premolar. The incidence of dental malformation was 23%, mostly mild forms. Ectopic eruption of the upper first permanent molars was seen in 23 (45%) of the subjects with large clefts, and in 18 (31%) of those with small clefts. The surgical method did not significantly affect the direction of the eruption. There is a correlation between cleft size and hypodontia, dental deformity, and ectopic eruption. Children with Robin sequence had almost the same incidence of hypodontia, malformed teeth, and ectopic eruption as children with large clefts. There was no correlation between surgical method and ectopic eruption of the maxillary first permanent molars.

Adolescent↗

Oro-maxillofacial development in patients with GH deficiency and in normal short children.

Detailed oro-maxillofacial studies using dental cast, pantomogram and cephalogram were performed in 43 patients with GH deficiency aged 7-17 years and compared statistically to the results from 62 short children with normal GH secretion. The dental age was retarded as compared to the chronological age in patients with GH deficiency by a mean of 2.0 +/- 1.3 years. This value did not differ statistically from that observed in normal short children (1.7 +/- 0.8 years). However the bone age was significantly retarded in patients with GH deficiency (3.2 +/- 1.7 yrs vs 1.5 +/- 1.1 yrs, p less than 0.001). There was no difference between tooth size or cephalogram analysis results in the children in the two groups. The coronal arch length, basal arch width and basal arch length were shorter in patients with GH deficiency. These data indicate underdevelopment of the maxilla in patients with GH deficiency.

Adolescent↗

Otodental syndrome.

Otodental dysplasia, a form of ectodermal dysplasia, is characterized by abnormal dental crown morphology and sensorineural hearing loss. A variety of dental abnormalities are prominent features of this syndrome--most notably gigantic, bulbous posterior teeth. In this case, a sibling is identifies in a family with otodental syndrome that had been diagnosed earlier. A unique opportunity is provided to view the intact adult dentition and possible complications for dental treatment.

Adolescent↗

Oral findings in the Morquio syndrome (mucopolysaccharidosis IV).

The Morquio syndrome is characterized by a specific pattern of platyspondylia, corneal opacities, keratosulfate excretion in the urine, and dental abnormalities. Oral examinations were performed on twelve patients with the condition. The maxillary anterior teeth were widely spaced and flared. The posterior teeth were tapered and had pointed cusp tips. The enamel was of normal hardness, and in some patients the surface was pitted. In roentgenograms, the enamel was less than one fourth its normal thickness but was of normal radiodensity. The prevalence of caries may have been reduced. The hard palates were broad and flat. The dental abnormalities in the Morquio syndrome are of a type that is unique among the group of genetic mucopolysaccharidoses. Our findings therefore support the conjecture that the biochemical defect in this condition is different from that which occurs in the other mucopolysaccharidoses.

Adolescent↗

Dental anomalies in familial adenomatous polyposis coli.

Forty-seven Danish and 50 Finnish patients with familial adenomatous polyposis coli (FPC) were studied by panoramic tomography (PTG) of the mandible, which showed dental abnormalities in 17% of the cases. Eleven patients (11%) had supernumerary teeth and/or compound osteomas, and nine patients (9%) had impacted permanent teeth. The frequency of the dental anomalies was statistically significantly higher in FPC patients than in the normal population; it is therefore concluded that this dental abnormality should be included in the list of extracolonic manifestations that may occur in any FPC patient. The frequency of dental anomalies was higher in Finnish than in Danish patients, probably owing to a higher frequency of extracolonic manifestations in the Finnish series.

Adenomatous Polyposis Coli↗

Incontinentia pigmenti: a case report.

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.

Child↗

Dental abnormalities associated with a chromosome 2 deletion.

This case report describes the dental and physical anomalies observed in a young female patient who had a chromosome abnormality involving deletion of the q 33-q 35 region of chromosome 2. The dental dysplasia observed in this patient is proposed to be of genetic origin but the complication of anoxia at birth makes it difficult to assign the intellectual and physical impairments solely to the genetic deletion.

Child, Preschool↗

Prenatal anticonvulsant drug exposure: teratogenic effect on the dentition.

Prenatal exposure to anticonvulsant medication has been shown to cause craniofacial dysmorphology, prenatal growth retardation, hypoplastic nails and phalanges, and visceral abnormalities. In this study we examined maxillary and mandibular stone dental casts (45) and panoramic radiographs (39) from 45 individuals with ages 4.5 to 22.0 years for changes in mesiodistal crown size of deciduous and permanent teeth, and the presence of dental anomalies. These individuals had been exposed prenatally to antiepileptic drugs (AEDs). Mesiodistal crown diameters were measured from the dental casts and converted into standard scores (Z), using published normative data from the University of Michigan Longitudinal Craniofacial Growth Series. A significant increase in mesiodistal crown dimensions of the posterior maxillary teeth was observed, specifically in primary molars and their permanent premolar successors, as well as permanent molars. Changes in tooth size were more common in females than in males. Dental maturity, assessed using the panoramic radiographs, was equal to chronologic age. An increased frequency of hypodontia was the only notable dental anomaly.

Adolescent↗

"Otodental" dysplasia.

The case of a 3 11/12-year-old Chinese boy with the dental abnormalities of "otodental" dysplasia is reported. Hearing was normal. Dental anomalies consisted of delayed eruption of globe-shaped molars, bulbous deciduous canines, and double pulp chambers in the molars. Radiographs taken 4 years later showed taurodontic molars, supernumerary microdontic teeth, retarded formation of premolars, and probable aplasia of the mandibular second premolars.

Child↗

Diagnostic accuracy and observer performance in the diagnosis of abnormalities in the anterior maxilla: a comparison of panoramic with intraoral radiography.

An investigation was undertaken to assess the reliability of radiographic diagnosis of abnormalities of orthodontic significance in the anterior region of the maxilla from intraoral and dental panoramic tomograph (DPT) views. DPT films of 200 patients were scrutinised by two observers on two separate occasions, who also examined intraoral radiographs of the same patients. Sixty-three of these patients were selected because they had previously been diagnosed as presenting with a defined abnormality of orthodontic relevance, such as supernumerary teeth, absent incisors, etc. The remaining cases had been assessed as depicting normal radiographic appearances. Each radiograph was allocated a unique, randomly selected code number as the only means of identification. It was not possible for the observers to match the DPT to the corresponding intraoral films, and this information was only available to the principal investigator (JWF). Both observers were asked to record the presence or otherwise of any abnormalities at each observation of each radiograph, and to record their findings. The overall level of diagnostic accuracy and reproducibility was fairly high, and in general terms the DPT views were as reliable as the intraoral films with regard to the detection of abnormalities; however, there were significant differences between the observers. It was concluded that in many instances supplementary intraoral films would not contribute any additional information compared to the DPT and that selection criteria should be defined for the former.

Chi-Square Distribution↗

Otodental syndrome. A case report.

A 12-year-old boy with characteristic findings of otodental syndrome is presented. Abnormalities of deciduous and permanent dentition included bulbous canines, globe-shaped posterior teeth and agenesis of maxillary premolars. High-frequency sensorineural hearing loss was demonstrated, and results of genetic studies indicated an autosomal dominant trait. This patient presented previously unreported findings, including generalized macrodontia, delayed mineralization of the mandibular premolars, and supplementary permanent maxillary canines.

Child↗

Dental and facial bone abnormalities in pyknodysostosis: CT findings.

Pyknodysostosis is an autosomal-recessive disorder of osteoclast dysfunction causing osteosclerosis, with associated maxillofacial anomalies. Multidetector CT with multiplanar and 3D reconstruction illustrated the pathologic findings in this case. Abnormalities included multiple retained deciduous teeth, unerupted teeth with associated follicles, an irregularly expanded alveolus and body of the mandible, and an obtuse mandibular angle. Volume-rendered imaging better delineated the irregular dentition, with crowding and retention of deciduous teeth.

Adult↗