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Fusion involving both maxillary central incisors.
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Oral findings in osteogenesis imperfecta.
The dentitions of twenty-eight patients, each of whom had either an autosomal dominant or a sporadic osteogenesis imperfecta (OI) syndrome, were evaluated. The diagnosis of dentinogenesis imperfecta (DI) could be established in all seven patients with dominantly inherited OI in three families, while all eight persons with dominant OI in three other families had normal teeth. Of the thirteen remaining patients with OI, twelve had no family history of the disorder; four had DI and eight had normal teeth. One person had a family history of OI and DI. All patients with abnormal tooth wear and spontaneous tooth fractures had DI. The DMF ratio increased with age in all patients with OI type I and was higher among the patients with OI type III and DI. Class III malocclusions were found in 66% of the patients. A statistically significant high incidence of impacted first and second molars was noted.
Management of a supernumerary tooth fused to a permanent maxillary central incisor.
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Bilateral fusion and gemination: a literature analysis and case report.
The dental literature concerning the bilateral presentation of fusion and gemination is reviewed and analyzed. Tables are presented to show the prevalence and distribution of these anomalies in both the primary and permanent dentitions. In addition, the first prevalence statistics for the bilateral occurrence of fusion and gemination in the primary and permanent dentitions are determined. The first case of bilateral fusion of mandibular primary incisors to be reported in 13 years is presented. The presence of all four succedaneous incisors and the diagnosis of bilateral fusion are confirmed radiographically. The developmental concerns and possible sequelae of bilateral fusion and gemination are also discussed.
Dental anomalies in the midline.
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Comparison of dental findings in patients with isolated growth hormone deficiency treated with human growth hormone (hGH) and in untreated patients with Laron-type dwarfism.
The effect of human growth hormone (hGH) on dental structures was investigated by comparing two groups of patients with hypopituitarism. One group of 19 patients with isolated growth hormone deficiency who received hGH replacement therapy was compared with a group of 13 patients with high immunoreactive growth hormone and IGF-I deficiency, Laron-type dwarfism, who could not benefit from hGH treatment. Plaster casts of the dentitions and panoramic radiographs were obtained from all patients and analyzed for dental age, arch length, arch circumference, and width of teeth. the main findings were that (1) hGH therapy did not accelerate dental age but enhanced bone age; (2) the mandible was significantly smaller in the untreated group but no significant difference was found between the groups in the maxilla; (3) third molars were missing in more than 90% of the patients with Laron-type dwarfism whereas hypodontia of other teeth was found in 30% of all the patients; and (4) crowding was less prevalent than expected and the mesiodistal width of the teeth seemed to be smaller.
Oral clinicopathologic manifestations in maple syrup urine disease.
Maple syrup urine disease is a rare inborn error of metabolism that manifests mainly by neurologic deterioration, coma, and death in early childhood. Oral manifestations described in this article were mostly gingival enlargement and rampant caries, while histopathologic findings revealed a picture compatible osteomyelitis.
Dental findings in mucopolysaccharidosis type IV A (Morquio's disease type A).
Dental examinations on nine patients with mucopolysaccharidosis type IV A (MPS IV A, Morquio's disease type A) were carried out. Detailed medical, radiologic, and biochemical studies of each case were also performed independently. Dental changes were present in all cases, although the severity varied. The severity of the dental changes did not correlate with the clinical or biochemical findings in all cases. These dental changes are seen only in MPS IV A (N-acetylgalactosamine-6-sulphate sulphatase deficiency) and are not found in MPS IV B (beta-galactosidase deficiency) or the recently delineated MPS IV C (enzyme defect unknown). The dental changes can aid in the diagnosis of patients affected by MPS IV A and are especially useful in mild atypical cases.
Contiguous enlarged dental follicles with histologic features resembling the WHO type of odontogenic fibroma.
Defective odontogenesis and/or retarded eruption of teeth can be associated with histologic features akin to odontogenic fibroma in the dental follicles. Unerupted mandibular premolar and molar teeth of a 24-year-old man were surgically exposed, yet the teeth failed to erupt. About a year and a half later, radiographs indicated further enlargement of the follicle of the premolar, and both teeth were subsequently surgically removed. Histologically, the follicles were composed of mature collagenous tissue among which epithelial islands and numerous clusters of calcified bodies were present. Indirect immunofluorescence showed positive staining for type I and type III collagen, which exhibited a sparse distribution, but not for the aminoterminal propeptide of type III procollagen. The hamartomatous nature of the lesions is discussed with emphasis on their histologic resemblance to the WHO type of odontogenic fibroma.
Anomalous morphologic formation of deciduous and permanent teeth in a 5-year-old 15th century child: a variant of the Ekman-Westborg-Julin syndrome.
A gross morphologic anomaly affecting both the primary and secondary teeth of unknown cause is presented. A 5-year-old American Indian child exhibited macrodontia, extreme shovel-shaping, agenesis, three-rooted deciduous molars, dens invaginatus, and other less striking dental features. This case represents the earliest example of a variant of the Ekman-Westborg-Julin syndrome reported in the New World.
Fusion and gemination in alternate dentitions.
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Three-rooted permanent mandibular first molars of Asian and black groups in the Middle East.
The occurrence of supplementary roots of the lower first permanent molars is 2.33% for a Saudi group of Asiatic descendants and 0.65% for an Egyptian group of African descendants. The most prominent finding demonstrated in this study is the lower percentage of occurrence of the third root among the Saudi samples compared to other Asiatic samples in other studies. Furthermore, this study confirmed the occurrence of the anomaly in our African samples, and the clinical situation illustrates the importance of early diagnosis to avoid complications for such molars by dental practitioners.
Microdontia, hypodontia, short bulbous roots and root canals with strabismus, short stature, and borderline mentality.
A case of unusual dental findings associated with mild growth and mental retardation is presented. The patient, a 15-year-old girl, manifests small teeth with peculiar short bulbous roots and roundly widened root canals, congenital absence of permanent teeth, strabismus, short stature, and borderline mentality.