Dental abnormalities in the tricho-dento-osseous syndrome.
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Four patients, three in the same family, with cleidocranial dysplasia, are reported. The birth of a male newborn with cleidocranial dysplasia has allowed to investigate his mother and grandmother with the same condition. An other 30-year-old patient with cleidocranial dysplasia is described. All the adult patients showed the main clinical manifestations of the disease with the peculiar abnormalities of the dentition.
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Stomatological examinations of infants aged 6 to 36 months, whose mothers suffer from heart diseases, have revealed that the number of infants with deciduous dental tissue abnormalities grows with the age of the infants, reaching the maximum in those aged 3 years: 63.88 percent. Caries and caries-complicated diseases were recorded in 3-year-old children. This permits regarding the presence of a heart disease in a mother as a risk factor in respect of developing deciduous teeth abnormalities and caries.
A case of VACTERL association has been presented in conjunction with possible hemifacial microsomia and fused teeth. Although the etiology of VACTERL association and hemifacial microsomia is unclear, the similarity of symptoms would suggest a common cause. A retrospective study of patients previously diagnosed with VACTERL association might reveal additional symptoms of dental and/or facial anomalies and warrant changing the acronym to VACTERL-DF in recognition of these dentofacial findings.
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Seventy-three children with submucous cleft palate (38 girls and 35 boys), mean age 8.2 years (range 7.7-9.5), were studied retrospectively from orthopantomograms. Dental abnormalities in permanent dentition were found in 26 patients (36%). Missing teeth, mainly lower 2nd premolars, upper lateral incisors, and upper 2nd premolars, were found in 12 patients (16%). Most of the patients had 1 or 2 missing teeth, 2 had 3 missing teeth. In 5 patients hypodontia was associated with another dental abnormality. Other dental abnormalities included peg-shaped lateral incisors in 7 patients (10%), ectopic eruption of upper 1st molars in 6 patients (8%), transposition of upper canines and 1st premolars in 3 patients (4%), supernumerary teeth in 2 patients (3%), and palatally displaced upper canines in 1 patient (1%). As children with submucous cleft palate have a tendency towards increased frequency of missing teeth and other dental abnormalities, the need for thorough clinical and radiological dental examination is emphasized.
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This clinicopathologic case report documents severe gingival recession in the primary dentition of a trisomy 18 patient. Primary molar and canine teeth exhibited recession extending beyond the midpoint of the buccal aspect of the root, occasionally reaching the root apex. Radiographic examination revealed taurodontism in both primary and permanent teeth. Clinical and histopathologic findings, along with case history, eliminated the possibility of prepubertal periodontitis and suggested a diagnosis of self-inflicted injury associated with mental retardation. Histologic examination of the primary teeth revealed normal cementum and dentin structure. Taurodontism, histologic structure of the dentition, and severe attachment loss in the primary dentition have not been described previously in trisomy 18.
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Skulls (n = 301) of adult feral cats from Marion Island, a sub-Antarctic island, were examined macroscopically. Congenital anomalies, which were rare, included a few supernumerary premolars, located mesially to those normally present. Supernumerary roots, mainly of the maxillary third premolar, were found in just over 10% of cases. The maxillary second premolar was absent in 16.8% of skulls; dichotomous and double-formed roots of this tooth were present in 20.1% and 1.9% of cases, respectively. Enamel hypoplasia, which is most unusual in this species, was noted in 24.6% of cases, and persistent deciduous teeth in 2.0%. Dental abrasion, which was noted in 19.3% of cats, affected only 2.3% of teeth, most commonly the lower fourth and upper third premolars. Dental fractures, mainly complicated crown fractures and root fractures, were noted in 54.8% of cats and 7.0% of teeth. Fractures were found most commonly in the canine and carnassial teeth, as also were periapical lesions, which were often severe. Mandibular fractures in various stages of healing were found in 11 cats (3.7%); such fractures most frequently affected the body of the mandible and resulted in malunion. It was concluded that the high prevalence of dental fractures and associated periapical lesions probably exerted a significant adverse effect on health and survival in this population of feral cats.
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Trichodentoosseous (TDO) syndrome is an autosomal dominant condition characterized by dysplastic nails, curly hair, bone sclerosis of the long bones and calvarium, taurodontism, and enamel hypoplasia that occurs with hypomaturation/hypocalcification defects. To date, nine previous case reports of TDO in the dental literature have shown that while taurodontism and enamel defects are consistently present in TDO, the changes in the nails, hair, and bones may be variably expressed and occasionally not noted. Because of this, a few previous cases of TDO syndrome had been diagnosed simply as amelogenesis imperfecta. This case report highlights the typical clinical findings, diagnostic problems, and the clues to diagnosing this interesting condition. Clinical management of TDO is centered on preventing dental abscesses with stainless steel crowns and improving appearance with esthetic restorations.
The dental status of 16 children who had been treated with bone marrow transplantation (BMT) for serious bone marrow diseases was followed for up to 6 years. Several types of disturbances in dental development were observed in children who had been conditioned with total body irradiation (TBI) at 10 Gy before BMT. Thus, impaired root development that caused short V-shaped roots was found in all patients, a complete failure of root development and premature apical closure were found in five patients, enamel hypoplasia was observed in four patients, and microdontia was observed in three patients conditioned with TBI. Patients younger than 6 years of age at BMT exhibited the most severe and extensive dental aberrations. The TBI at 10 Gy appeared to be the major cause of the disturbances found.
c-src knockout and op/op mice develop osteopetrosis as a result of defective osteoclast function and osteoclast formation, respectively. The mutant mice can be distinguished readily from their wild-type littermates around 10-12 days after birth because their incisors do not erupt, but the morphology of their teeth and surrounding bone has not been reported previously in detail. Histologic examination of jaws of src-mutant mice reveals unerupted, abnormal incisors within their bony crypts. The tooth roots are distorted by foci of haphazard proliferation of odontogenic epithelium associated with primitive tooth structures that strongly resemble the tumor-like lesions in humans, known as odontomas. The crowns of the incisors are fused to the adjacent bone, and the developing periodontal ligament is disordered and hypocellular. Osteoclasts are present in the bone surrounding the distorted teeth, but as in other bones in these mice they lack ruffled borders and thus do not resorb effectively. Similar odontogenic proliferation is present around unerupted incisors in op/op mice which form very few osteoclasts, but the amount is significantly less than in src mutant mice. Molars fail to erupt in both types of mutant mice, but they are not accompanied by aberrant odontogenic proliferation. These findings and previous reports of similar abnormalities in jaws from op/op rats suggest that failure of incisor eruption and associated proliferation of odontogenic epithelium in osteopetrotic rodents are a direct result of defective osteoclastic bone resorption.
An 11 year old child presented to our department with an isolated abnormality of the left superior median incisor which was bigeminate. The existence of two distinct roots allowed the separation of 21 and 21 b with extraction of 21 b and preservation of 21, whose crown was reconstituted by means of a composite. There was a favourable outcome with conservation of the vitality of the tooth and no signs of ankylosis.
Ellis-van Creveld syndrome (EvC), also called chondroectodermal dysplasia, is a rare occurrence inherited as an autosomal recessive disease. Despite the fact that oral manifestations play an important role in the diagnosis criteria for EvC, few detailed reports have been published in the dental literature. This articles presents two siblings with EvC, a boy aged 9 years and a girl aged 7 1/2 years, a product of unaffected first cousin parents. The patients manifests: chondrodysplasia of tubular bones resulting in disproportionate dwarfism, polydactyly and syndactyly of hands and feet, severe dystrophic nails, multiple broad labial frenula with abnormal attachments, congenital missing incisors, anomalous teeth, bilateral partial clefts of the alveolar bone, and malocclusion. Other features noted in either cases are: congenital heart defect, median notch of the upper lip, shovel-shaped incisors and taurodontism. Of the unusual dental findings observed in our patients are talon cusp, reduced crown size, supernumerary tooth, and early eruption of teeth. Because half of the cases with EvC have cardiac malformation, dental treatment must be performed under prophylactic antibiotic coverage. Dentists play an important role in early diagnosis and control of dental problem of this condition.