• The korean journal of orthodontics
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• v.43 no.5
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• pp.248-260
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• 2013

Cleidocranial dysplasia (CCD), an autosomal dominant disorder with a prevalence of 1 in 1,000,000 individuals, is mainly caused by mutations in Runx2, a gene required for osteoblastic differentiation. It is generally characterized by hypoplastic clavicles, narrow thorax, and delayed or absent fontanel closure. Importantly, its orofacial manifestations, including midfacial hypoplasia, retained primary teeth, and impacted permanent and supernumerary teeth, severely impede the well-being of affected individuals. Successful treatment of the orofacial problems requires the combined efforts of dental specialists. However, only a few successfully treated cases have been reported because of the rarity of CCD and complexity of the treatment. This article presents the University of California, San Francisco (UCSF) treatment protocol for the dentofacial manifestations of CCD based on two treated and 17 diagnosed cases. The records of two patients with CCD who had been treated at the UCSF School of Dentistry and the treatment options reported in the literature were reviewed. The UCSF treatment protocol produced a successful case and a partially successful one (inadequate oral hygiene in the retention stage resulted in decay and loss of teeth). It provides general guidelines for successfully treating the orofacial manifestations of CCD.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.66-71
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• 2016

Intellectual disability is accompanied by a high incidence of congenitally absent teeth and supernumerary teeth, and is observed more frequently than are disorders of location and order during delayed eruption, when accompanied by other symptoms. Furthermore, it is associated with a higher occurrence of dental anomalies such as conical teeth, microdontia, and amelogenesis imperfecta. As it is difficult to obtain adequate cooperation from patients with intellectual disabilities, physical restraint and conscious sedation using medication and general anesthesia can be considered. Reshaping of conical teeth with resin composite may be helpful to rehabilitate patients with oligodontia and a conical tooth shape. Diagnostic wax-up and a silicone matrix formed the basis for the successful reconstruction of the anterior teeth. This case describes the treatment of a patient with intellectual disability who had oligodontia and conical-shaped incisors. Under general anesthesia, the patient was treated using direct composite resin restoration.

• Journal of the Korean Academy of Esthetic Dentistry
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• v.29 no.1
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• pp.13-24
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• 2020

Autotransplantation is the surgical repositioning of an autogenous erupted or unerupted tooth from one site to another in the same individual. This treatment is indicated in traumatic tooth loss, teeth with severe caries, congenitally missing teeth, teeth with bad prognosis and in case of developmental anomalies of teeth. The following 2 cases describe the potential to utilize autotransplantation to replace hopeless teeth with sound wisdom teeth.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.2
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• pp.331-338
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• 1999

Chemoradiation therapy used on pediatric oncology patients often causes dental developmental anomalies that affect future dental care. Defects noted include tooth and root agenesis, root thining and shortening, and localized enamel defects. The effect of radiotherapy usually are confined to the radiation site, but the effects of chemotherapy may be more wide spread because of its systemic distribution and structures and organs unrelated to the primary tumor may be affected. Many pediatric cancers are treated with a combination of radiation and multiagent chemotherapy to create synergic and additive effects. Dental treatment affected by chemoradiation damage to developing teeth includes orthodontic tooth movement, prosthetic abutment considerations, periodontal health, space maintenance, requirements for home fluoride regimens to protect hypomineralized areas, restoration options for hypoplastic/hypomineralized teeth, and endodontic procedures. The following case demonstrate chemoradiation therapy effects on the dental development.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.17-21
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• 2018

Williams syndrome (WS) is a rare congenital disorder which is caused by microdeletion of approximately 1.6 MBP from the long arm of chromosome 7 at 7q11.23. It is characterized by cardiovascular anomalies, elfin face and mental retardation. The most typical oral signs in patient with WS are hypodontia, reduced mesio-distal dimensions both in the primary and permanent teeth, macroglossia, excessive interdental spacing, enamel hypoplasia and enamel hypomineralization. The majority of children with WS have mild to moderate mental retardation, generalized anxiety disorder, hyperactivity disorder and sensitivity to sounds. The purpose of this presentation is to describe dental treatment for a child with WS. A 9-year-old boy diagnosed with WS had caries on his first permanent molars. Because of the poor cooperation, these teeth were filled temporarily with glass ionomer, and treatment under general anesthesia was planned. Under general anesthesia, caries treatment of first permanent molar and extraction of primary molar was successfully performed and there was no postoperative complications related to general anesthesia. Open bite, hypodontia, excessive dental space, enamel hypoplasia, enamel hypomineralization were observed which were characteristic in WS.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.119-125
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• 2009

Fusion and gemination are irregularities in tooth development. It is often difficult to differentiate between gemination and fusion and it is common to refer to these anomalies as 'double teeth'. The deciduous teeth are most commonly involved, but in 0.1% of cases permanent teeth are affected, in which case aesthetic, funtional and periodontal problems can result. Double teeth present great difficulties in management and required a multidisciplinary approach. The central groove on the labial and palatal surfaces of a double tooth is extremely prone to caries, therefore early 'fissure sealing' is essential. In permanent dentition, surgical separation of fused teeth may be possible with subsequent orthodontic alignment and restorative treatment as needed to reshape the crown. Reshaping or reduction of a double tooth with a single canal may be attempted by modifying the appearance of the labial groove and the use of composite tints but is often impossible and extraction may be the only alternative. Orthodontic treatment and prosthetic replacement is then required. Implants may be an option for adolescents. The present study describes three clinical cases of double teeth in the position of the maxillary permanent incisors. The first case demonstrates an example of multidisciplinary care including surgical intraoral hemisection, root canal therapy, restorative and orthodontic treatment. The second and third cases describe the external and internal morphology of the two fused teeth by means of three dimensional dental computer tomography.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.3
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• pp.374-380
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• 2010

Treacher Collins syndrome(TCS) is a rare, incurable condition occurring in approximately 1 of 25,000 to 50,000 births. It may occur as a spontaneous mutation out of genetically normal parents or it may be manifested as an autosomal dominant pattern. TCS is characterized by facial deformities such as, underdevelopment of the maxilla, mandible and zygoma, malocclusion, bilateral deformities of auricles, antimongoloid slant of the palpebral fissures. The syndrome is often associated with cleft lip and palate, ear malformations and hearing loss, short stature, and anomalies of the heart and skeleton. Respiratory difficulty associated with air way obstruction may also be observed, and there is considerable difficulty in airway management during general anesthesia. It is necessary that dentists provide safe dental treatments and guidelines to TCS patients by providing adequate understanding about the characteristics of the syndrome and proper ways of managements. The purpose of this study is to report the dental and medical characteristics of the patient who visited the Department of Pediatric Dentistry, Yonsei University for multiple dental caries treatment and to review the literatures of TCS.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.5 no.2
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• pp.92-95
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• 2009

In 1923, Pierre Robin, a French stomatologist, first reported the association of micrognathia with glossoptosis. As more than 80% of all Pierre Robin cases accompanied with other syndrome such as Stickler syndrome, velocardiofacial syndrome, fetal alcohol syndrome, Treacher Collins syndrome, etc, it is called Pierre Robin sequence(PRS). PRS is described in the literature as a triad of anomalies characterized by micrognathia, glossoptosis and cleft palate. Clinically, the triad consists of airway obstruction and feeding difficulty, which are more frequent and severe in the neonatal period. In this case, a 14-month old boy with PRS was referred to the department of pediatric dentistry, Yonsei university dental hospital, who resolved airway obstruction by lateral positioning and feeding problem by percutaneous endocutaneous gastrotomy insertion. PRS is a developmental disorder as well as an anatomic obstructive disorder, therefore it should be dealt with by a multidisciplinary team.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.1
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• pp.46-51
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• 2004

Talon cusp is cusp-like structure that develops additionally in crown of anterior tooth. And it is one of dental anomalies. Talon cusp can cause various diagnostic, functional, aesthetic problems depending on the size and configuration of cusp. there is a tendency for caries to occur in the developmental grooves, and advanced attrition, periodontal problems, irritation the tongue and temporomandibular pain, occlusal interference and displacement of affected tooth may result. Therefore, early diagnosis and appropriate treatment of each case is important so that it minimize local problems, such as caries, periodontal diseases, and malocclusion. These cases which are in permanent and primary anterior teeth are about gradual reduction forming reparative dentine and complete reduction of talon cusp and root canal therapy which is an alternative and effective form of treatment when gradual reduction of talon cusp may not be possible.