1/50. Dental and craniofacial features of Aarskog syndrome: report of a case and review of literature.Aarskog syndrome is a rare syndrome with a typical triad of facial, digital and genital characteristics. The characteristic cephalometric finding in this patient was the unusually large upward slant of SN plane and a steep Ba-N plane. Though the patient presented with a class I skeletal pattern, both the maxilla and mandible were hypoplastic and retruded with respect to the cranial base. Other characteristic features regarding the mandibular morphology were a large FMA (37 degrees) and Sn-GoGn (44 degrees) angles, a large gonial angle (138 degrees), an increase in total anterior facial and lower anterior facial height.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/50. Malformation in the primary and permanent dentitions following trauma prior to tooth eruption: a case report.Dento-facial injuries that occur prior to the eruption of teeth in the primary dentition are rare, but can result in damage to the primary dentition. We report a rare case where an injury to the anterior maxilla and mandible of an infant prior to primary tooth eruption resulted in hypoplasia, displacement and impaction of the primary dentition and damage to a developing permanent tooth.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
3/50. Rieger syndrome is associated with PAX6 deletion.PURPOSE: Rieger syndrome is an autosomal dominant condition defined by anterior segment dysgenesis in combination with facial, dental, skeletal and umbilical abnormalities. To date Rieger syndrome has been associated with mutations in the PITX2 gene at chromosome 4q25 and a second locus has been found at chromosome 13q14. methods: We describe a Rieger syndrome case with all the typical dysmorphic features and the molecular genetic finding by use of FISH analysis of the PAX6 gene. RESULTS: An eight-year-old girl had iris stroma hypoplasia, corectopia and iridogoniodysgenesis. She had an underdeveloped premaxilla and a congenital absence of nine teeth in the maxilla. The front teeth in the mandible were peg-shaped and all teeth were small. There was failure of involution of the periumbilical skin. FISH analysis using probes for the PAX6 gene showed a small deletion for the PAX6 gene on one homologue of chromosome 11. CONCLUSION: Rieger syndrome can -- in addition to PITX2 gene mutations and abnormalities at chromosome 13q14 -- be associated with PAX6 gene abnormalities.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
4/50. The dentofacial manifestations of XXXXY syndrome: a case report.This paper presents a six-year-old patient with XXXXY syndrome, whose oral findings included a cleft soft palate, hyper- or meso-taurodontism in eight primary molars and in the mandibular permanent first molars, five congenitally missing premolars, and delayed development of the permanent tooth germs. The maxillary and mandibular primary central incisors were in a cross-bite relationship. Cephalometric findings showed a short ramus of the mandible and a short maxilla in the anterioposterior plane. The anteroposterior jaw relationship was in harmony. The cross-bite was considered to be due to the retroinclination of the maxillary primary incisors. This case emphasises the importance of regular dental care, and monitoring of facial growth and dental development in children with XXXXY syndrome.- - - - - - - - - - ranking = 1.1545010592708keywords = mandible, jaw (Clic here for more details about this article) |
5/50. A newly recognized syndrome of skeletal dysplasia with opalescent and rootless teeth.A Thai girl with skeletal dysplasia and dental anomalies was seen. Her anomalies consisted of disproportionately short stature, short neck, broad and depressed nasal bridge, broad chest in the anteroposterior dimension, kyphosis, widely spaced nipples, and protruded abdomen. Radiographic testing indicated that she had a large sella turcica, platyspondyly, hypoplastic acetabulum, and a small body of mandible. Both her deciduous and permanent teeth were equally opalescent, and most were rootless, with root development of the mandibular teeth more severely affected. Some maxillary roots were extremely short and tapered. Hypodontia was also observed. These findings represent a unique and hitherto undescribed syndrome of skeletal dysplasia with concomitant dental anomalies.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
6/50. A case of multiple AOT-like jawbone lesions in a young patient--a new odontogenic entity?We assessed the immunohistochemical profile of an unusual case of multiple similarly looking tumors in the jawbone of a young patient. Histologically, the tumors exhibited features of adenomatoid odontogenic tumor (AOT) and adenomatoid dentinoma but showed no resemblance to any other defined odontogenic tumor entities. They expressed high amounts of cytokeratin (CK) 8 and 14 together with some vimentin. A small rim of peripheral cells showed CK 5, 17, and 19 reactivity. Also, these lesions expressed some bcl-2 as well as p53 and Ki67. Histologically and immunohistochemically, the unusual multiple lesions differed in details from a simultaneously examined group of 24 classical AOT cases, suggesting that they may represent a hitherto less well-defined odontogenic tumor entity.- - - - - - - - - - ranking = 0.77250529635418keywords = jaw (Clic here for more details about this article) |
7/50. Use of dental implants in patients with down syndrome: a case report.down syndrome is caused by trisomy of the 21st chromosome and is associated with well-described physical and systemic problems. Most people with down syndrome have some degree of mental retardation as well as malformation of head and neck. Oral structures that are commonly affected include the tongue (macroglossia), abnormalities in the number and shape of teeth, and poor quality (osteoporotic-like) of alveolar bone and jaw. These oral malformations as well as a tendency toward poor cooperation in the dental office contribute to the belief among dentists that people with down syndrome are not good candidates for oral rehabilitation with dental implants. This article describes the use of dental implants in the oral rehabilitation of a 16-year-old boy with down syndrome. Although more experience is needed before dental implants can be considered a suitable option during oral rehabilitation in people with down syndrome, this case report shows a promising beginning.- - - - - - - - - - ranking = 0.15450105927084keywords = jaw (Clic here for more details about this article) |
8/50. Report of a patient with hypoglossia-hypodactylia syndrome and a review of the literature.OBJECTIVE: To present the morphology of the extremities, craniofacial structures, and the oral cavity based on roentgencephalometry and three-dimensional computed tomography and magnetic resonance imaging scanning in a patient with hypoglossia-hypodactylia syndrome, discuss the orthodontic treatment method, and review the literature for the syndrome. PATIENT: The patient was a 6-year-old boy diagnosed with hypoglossia-hypodactylia syndrome at birth. He had hypodactylia as well as micrognathia with steep inclination of the anterior surface of the mandible in relation to the lower mandibular plane. He had missing mandibular incisors with concomitant bone defect limited to the associated alveolar ridge and an absence of any malformations in the mandibular ramus and condylar head. The patient had a bilateral scissors bite with an extremely constricted mandibular dental arch, skeletal Class II jaw relationship with an average mandibular plane angle and maxillary incisors inclined palatally. He had extremely reduced tongue size and hypertrophy of the floor of the mouth. Anomalies of the central nervous system were not observed. There was no evidence of hearing loss.- - - - - - - - - - ranking = 1.1545010592708keywords = mandible, jaw (Clic here for more details about this article) |
9/50. The Kabuki syndrome: four patients with oral abnormalities.The aim of this paper is to report the oral signs and symptoms of four patients with Kabuki syndrome. All had oral abnormalities affecting the palate, teeth, and/or lips, including wide spacing between the teeth, screwdriver-shaped upper incisors, hypodontia, delayed tooth eruption, narrow spacing in the upper canine area, large pulp chambers of the upper incisors and permanent molars, external root resorption of the upper central incisors and permanent molars, a division of the lower third of the root canal in normally one-rooted teeth, tooth retention, retrognathia of the upper jaw, a high palate or cleft lip/palate, and microforms of lower lip fistula.- - - - - - - - - - ranking = 0.15450105927084keywords = jaw (Clic here for more details about this article) |
10/50. 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 = 1keywords = mandible (Clic here for more details about this article) |
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