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Cleft lip and palate: developmental abnormalities of the face, mouth and jaws
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Dental anomalies are common findings in children with cleft lip and/or palate. Various phenomena including delayed tooth development, delayed eruption of teeth and morphological abnormalities are well documented. The number of teeth may be reduced (hypodontia) or increased (hyperdontia), occurring most commonly in the region of the cleft alveolus involving the maxillary lateral incisor tooth. These abnormalities can occur in both primary and secondary dentition.
Individual conditions grouped according to the international nosology and classification of genetic skeletal disorders*
Published in Christine M Hall, Amaka C Offiah, Francesca Forzano, Mario Lituania, Michelle Fink, Deborah Krakow, Fetal and Perinatal Skeletal Dysplasias, 2012
Christine M Hall, Amaka C Offiah, Francesca Forzano, Mario Lituania, Michelle Fink, Deborah Krakow
Prognosis: viable – life expectancy is reduced only in cases with significant malformations. Affected individuals develop progressive short stature, microcephaly, moderate to severe intellectual impairment and obesity. Behavioural problems are common, particularly short attention span, impulsivity, autistic behaviour, self-injurious and aggressive behaviour. Childhood problems include respiratory difficulties, sleep apnoea, feeding problems, recurrent infections, severe constipation. Joint hypermobility is common, in particular dislocations of the patellae. Visual problems include retinal dysfunction, strabismus, refractory errors, cataracts, coloboma and glaucoma. Dental problems are frequent, especially malocclusion and susceptibility to dental caries, typical findings comprise hypodontia, hyperdontia, natal teeth, and talon cusps on the upper incisors of the secondary dentition. Cutaneous findings include keloids following minimal trauma and pilomatrixomas. Individuals with RSTS have an increased risk of tumour development; among the reports are meningioma, pilomatrixoma, rhabdomyosarcoma, phaeochromocytoma, neuroblastoma, medulloblastoma, oli-godendroglioma, leiomyosarcoma, seminoma, odontoma, choristoma and leukaemia.
Rubinstein−Taybi Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Other anomalies may affect the eye (e.g., strabismus, refractory errors, ptosis, nasolacrimal duct obstruction, cataract, unilateral or bilateral iris/retinal/optic nerve coloboma, nystagmus, glaucoma, and corneal dysfunction), ear (e.g., conductive and/or sensorineural deafness, recurrent middle ear infections), heart (e.g., nonspecific abnormalities of electroencephalography, and congenital heart defects including atrial septal defect, ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, pulmonic stenosis, bicuspid aortic valve, pseudotruncus, aortic stenosis, dextrocardia, vascular rings, and conduction disorders), genitourinary organs (e.g., renal abnormalities, and cryptorchidism/undescended testes in boys), orthopedic, skeletal, and spinal tissues (e.g., dislocated patellae, ligamentous laxity, spine curvatures, Legg−Perthes disease, slipped capital femoral epiphysis, craniospinal and posterior fossa abnormalities, Chiari malformation, syringomyelia, os odontoideum, cervical cord compression, and cervical vertebral abnormalities), respiratory organs (e.g., respiratory difficulties, obstructive sleep apnea due to narrow palate, micrognathia, hypotonia, obesity, easy collapsibility of the laryngeal walls, and recurrent respiratory infections), skin (e.g., keloids nevus, café-au-lait spots, keratoses pilaris, ingrown toenails, and paronychia), teeth (e.g., overcrowding of teeth, enamel hypoplasia, malocclusion, multiple caries, hypodontia, hyperdontia, natal teeth, and talon cusps on the upper incisors of the secondary dentition), gastrointestinal tract (e.g., gastroesophageal reflux, constipation, and megacolon/Hirschsprung disease), and endocrine organs (e.g., congenital hypothyroidism, thyroid hypoplasia, GH deficiency, and pituitary hypoplasia) [18–23].
Molecular Genetics of Cleidocranial Dysplasia
Published in Fetal and Pediatric Pathology, 2021
Jamshid Motaei, Arash Salmaninejad, Ebrahim Jamali, Imaneh Khorsand, Mohammad Ahmadvand, Sasan Shabani, Farshid Karimi, Mohammad Sadegh Nazari, Golsa Ketabchi, Fatemeh Naqipour
Human have two teeth series, 20 teeth in the deciduous dentition and 32 teeth in the permanent dentition. Supernumerary teeth (ST), or hyperdontia are defined as additional teeth to the normal dental components. Supernumerary teeth (ST) are one of the most common human anomalies [52]. They may occur anywhere in the mandible or in the maxilla, singly or in multiples, unilaterally or bilaterally, erupted or unerupted. The etiology of ST is still unknown. They may be associated with a syndrome or non-syndrome patients. The prevalence of ST in the general population is 0.2% to 0.8% in the deciduous dentition and 0.5% to 5.3% in the permanent dentition [52] . The presence of one, two and multiple ST for non-syndromic cases has been reported to be 76–86%, 12–23%, and 1%, respectively [53]. Multiple supernumerary teeth have been reported in normal individuals, but most are associated with other disorders or syndromes [52, 54].
Reduced mesiodistal tooth dimension in individuals with osteogenesis imperfecta: a cross-sectional study
Published in Acta Odontologica Scandinavica, 2021
L. Staun Larsen, K. J. Thuesen, H. Gjørup, J. D. Hald, M. Væth, M. Dalstra, D. Haubek
The present results on mesiodistal tooth dimension in healthy 20-yr-old controls corroborate Townsend’s results from 1983 [22]. The age of twenty was chosen to match Townsend’s control group comprising 265 children and young adults [22]. Considering gender dimorphism, both studies show that women’s teeth in general are smaller than men’s teeth. This finding is in line with men exhibiting a higher frequency of hyperdontia and macrodontia than women, and women having a higher prevalence of microdontia and hypodontia than men [20]. The present gender difference (Table 3) is in the same order of magnitude as Townsend found [22], with women’s teeth being in average 2% smaller in the mesiodistal dimension compared to men’s teeth. Townsend made measurements on plaster models using a dial calliper [22] as compared to the present digital measurements on 3 D-models. A digital approach was chosen since a recent study has shown that measurements on digital models present with less variation than measurements on plaster models [23].
Testing the London atlas for age estimation in Thai population
Published in Acta Odontologica Scandinavica, 2020
Wanwisah Namwong, Scheila Mânica
Ethical approval was granted from the Ethic Review Committee for Human research, Police General Hospital, Bangkok, Thailand (COA no. 94/2016). The London atlas for age estimation was tested in 111 digital panoramic radiographs from the General Police Hospital, Bangkok, Thailand. The sample was composed of children (57 ♂ and 54 ♀) aged between 4.00 and 15.99 years. The CA of each subject was calculated by subtracting the date of birth from the date of radiographic examination. Inclusion criteria included good quality panoramic radiographs of healthy children with no medical history of systemic diseases/disorders. Children who presented hypodontia, hyperdontia, gross pathology and previous orthodontic treatment or severe malocclusion were excluded. The distribution between female and male was almost equal in order to avoid age mimicry as seen in Table 1. The radiographs were assessed by the main author using the sex-specific application software to determine the developmental and eruption stages of all teeth in the left side, both upper and lower jaws, according to AlQahtani et al. [8].