1/27. Simultaneous occurrence of unusual odontodysplasia and oligodontia in the permanent dentition: report of a case.odontodysplasia is an uncommon clinicopathological condition with a variety of expressions. Although it is generally recognized as a localized disorder of dental tissue, its aetiology has not yet been well explained. In the present case, odontodysplasia with oligodontia in the permanent dentition is reported. The patient was in good health with normal stature and no other physical abnormalities. His parents and siblings were dentally and medically normal. The primary teeth appeared to be normal except for the primary second molars, where the enamel was malformed. However, the permanent incisors that had erupted into the oral cavity showed rough and hypoplastic enamel. An orthopantomogram showed 17 congenitally missing permanent teeth and malformation of the other 11 permanent teeth and tooth-germs. Because these findings were caused by developmental disturbances of both the mesodermal and ectodermal dental components, we diagnosed the present case as odontodysplasia accompanied by oligodontia in the permanent dentition.- - - - - - - - - - ranking = 1keywords = dysplasia (Clic here for more details about this article) |
2/27. Seckel syndrome: report of a case.An interesting case of a seven years old boy with a combination of clinical, genetic, radiological, pathologic and dental findings is presented in view of Seckel syndrome literature. General appearance of the patient was characterized by small forehead, posteriorly slanted ears, slightly beaked nose, midfacial hypoplasia very stunted stature with microcephaly. He had borderline mental retardation with normal motor development. Class II dentoskeletal pattern with mild overjet and open bite, congenitally missing permanent teeth, microdontia, enamel hypoplasia, taurodontism and dentinal dysplasia was observed according to the clinical and radiographic examination. In conclusion, Seckel syndrome is not encountered routinely in dental clinics, this case illustrates the importance of dental care in such a rare condition.- - - - - - - - - - ranking = 0.14285714285714keywords = dysplasia (Clic here for more details about this article) |
3/27. Segmental odontomaxillary dysplasia. Report of two cases and review of the literature.Segmental odontomaxillary dysplasia is a rare childhood disorder. The condition affects bone growth and maturation and causes maldevelopment of the ipsilateral teeth and gingiva. Lips and skin also may be affected. The purpose of this article is to describe two cases of segmental odontomaillary dysplasia and summarize the clinical and radiographic findings if the entity after a thorough review of the English literature.- - - - - - - - - - ranking = 0.85714285714286keywords = dysplasia (Clic here for more details about this article) |
4/27. Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl.Terminal osseous dysplasia and pigmentary defects is a rare X-linked dominant disorder with prenatal male lethality. Affected females display multiple systemic abnormalities such as limb deformities and pigmented lesions of the face and scalp. Phenotypic expression of the syndrome varies among the affected individuals. In this case report, we describe the syndromic dental and oral abnormalities in a female child aged 3 and 1/2 years. A widened bigonial width of the mandible and a brachyfacial pattern are observed. Intraoral findings include multiple frenulae, shallow mucobuccal fold, hypodontia, conical incisors, and other developmental structural defects.- - - - - - - - - - ranking = 0.71428571428571keywords = dysplasia (Clic here for more details about this article) |
5/27. Oculodentodigital dysplasia: study of ophthalmological and clinical manifestations in three boys with probably autosomal recessive inheritance.Oculodentodigital dysplasia (ODDD) is a rare inherited disorder affecting the development of the face, eyes, teeth, and limbs. The majority of cases of ODDD are inherited as an autosomal dominant condition. There are few reports of probable autosomal recessive transmission. Affected patients exhibit a distinctive physiognomy with a narrow nose, hypoplastic alae nasi, and anteverted nostrils, bilateral microphthalmos, and microcornea. Sometimes iris anomalies and secondary glaucoma are present. There are malformations of the distal extremities such as syndactyly. In addition, there are defects in the dental enamel with hypoplasia and yellow discoloration of the teeth. Less common features include hypotrichosis, intracranial calcifications, and conductive deafness secondary to recurrent otitis media. We describe three brothers with ODDD. Their parents are first cousins and present no features of ODDD. These data are in favor of autosomal recessive inheritance and suggest genetic heterogeneity for this entity.- - - - - - - - - - ranking = 0.71428571428571keywords = dysplasia (Clic here for more details about this article) |
6/27. Clinical features of tricho-dento-osseous syndrome and presentation of three new cases: an addition to clinical heterogeneity.OBJECTIVE: Tricho-dento-osseous dysplasia is a rare autosomal dominant disorder which involves increased bone density, enamel hypoplasia, enlarged pulp chambers, and molar taurodontism. This study discusses the phenotypic variability of this condition and describes 3 new cases from a large family. STUDY DESIGN: Three affected females and 1 unaffected female from the same family were clinically and radiographically evaluated. mutation analysis was performed in the candidate gene DLX3. Phenotypes of affected individuals from 3 generations were compared to an unaffected control. RESULTS: All affected subjects show increased bone density in long bones and increased thickness and bone density in the skull, especially the skull base. Mandibles are within the upper normal size limits and display increased trabeculation and bone density. No bone loss or regression of the alveolar ridge occurs in older subjects after teeth have been lost. CONCLUSION: The mutation in DLX3 has positive effects on bone density throughout life. Although the mutation in this family is identical with mutations found in other families it results in clinical and phenotypic variability.- - - - - - - - - - ranking = 0.14285714285714keywords = dysplasia (Clic here for more details about this article) |
7/27. focal dermal hypoplasia: oral and dental findings.focal dermal hypoplasia/Goltz Gorlin syndrome is a rare syndrome characterized by widespread dysplasia affecting tissues of mesodermal and ectodermal origin with cutaneous, osseous, ocular, oral and dental defects. Enamel hypoplasia is the most commonly reported dental manifestation and has recently been described as a possible manifestation of Lyonisation. This article reviews the reported dental findings and reports a new case with typical findings of focal dermal hypoplasia, which has been under review on our department for 10 years. It discusses the differential diagnosis as well as newer concepts of aetiology and pathogenesis in relation to dental anomalies. Enamel hypoplasia may make plaque control difficult, resulting in generalized gingivitis. hand anomalies may limit dexterity and exacerbate this. From the dental standpoint we emphasize the implementation of timely preventive and/or therapeutic strategies. Since there are periods of exacerbation during the course of this syndrome, regular surveillance from an early age with the frequency of visits increasing during and after adolescence is indicated. The role of the dentist in improving aesthetics and function can have tremendous psychological impact to enhance self-esteem of such patients.- - - - - - - - - - ranking = 0.14285714285714keywords = dysplasia (Clic here for more details about this article) |
8/27. Enamel dysplasia with odontogenic fibroma-like hamartomas: review of the literature and report of a case.This article reports on a case presenting with a rare syndrome characterized by enamel dysplasia and multiple unerupted teeth with large solid fibrous pericoronal lesions manifesting with odontogenic fibroma-like features. Our case shows in addition to these findings an anterior open bite malocclusion and gingival overgrowths. These overgrowths exhibit the microscopic features of the multiple pericoronal odontogenic fibroma-like lesions that appear to be the hallmark of this syndrome. This unusual case brings the total number documented in the literature to 5, all of which were reported from south africa.- - - - - - - - - - ranking = 0.71428571428571keywords = dysplasia (Clic here for more details about this article) |
9/27. A syndrome of widened medullary cavities of bone, aortic calcification, abnormal dentition, and muscular weakness (the Singleton-Merten syndrome).Two patients with clinical and radiological features similar to those of Singleton and Merten's patients are described. These patients exhibit features of a unique clinical syndrome of unknown etiology: generalized muscular weakness with secondary hip and foot deformities, progressive calcification of the thoracic aorta beginning in childhood, calcific aortic stenosis leading to heart failure, dysplasia of the teeth, poor physical development, osteoporosis, expanded medullary cavities of the metacarpal and metatarsal bones, and chronic psoriaform skin lesions.- - - - - - - - - - ranking = 0.14285714285714keywords = dysplasia (Clic here for more details about this article) |
10/27. Oculodentodigital syndrome: report of a case.The clinical and radiographic changes in a case of oculodentodigital syndrome are presented. The characteristic features of this rare developmental disorder, microphthalmus with microcornea and iris anomalies, hypertelorism, thin nose with hypoplastic alae and anteverted nostrils, syndactyly with camptodactyly and clinodactyly of the fourth and fifth fingers associated with bony anomalies of the middle phalanges of the fifth fingers and toes, were all present. In addition, histological examination of a lateral incisor showed the enamel dysplasia to be due to enamel hypoplasia; the dentine also showed marked hypocalcification. There were widespread pulp denticles and hypercementosis throughout the dentition. Both the patient and his mother had coronoid hypoplasia.- - - - - - - - - - ranking = 0.14285714285714keywords = dysplasia (Clic here for more details about this article) |
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