• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.4
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• pp.667-672
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• 2003

Rieger syndrome is a rare, autosomal dominant genetic disorder characterized by malformation of the anterior chamber of the eye(goniodysgenesis) coincident with hypodontia. It may also be accompanied by a spectrum of dental, craniofacial and somatic anomalies. Mutations in paired-like homeodomain transcription factor2(PITX2) are associated with the syndrome, and its frequency in the general population has been estimated to be 1 : 200,000. In the present case, the patient, 4 year 7 month-old female, had posterior embryotoxon and polycoria. The maxilla was retrusive in cephalometric radiography. She had congenital missing on #52, #62 and some tooth germs of permanent tooth were not detected in panoramic radiography. The purpose of this paper is to report the dental and craniofacial findings and review the pertinent literature through this case.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.20 no.2
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• pp.227-234
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• 1990

The purpose of this study was to evaluate the incidence and several radiographic features of dilacerated teeth in 2132 full month radiograms in Korean. The results were as follows: 1. The occurrence was revealed to 2.7% in total examined teeth, and these anomalies were occurred in maxilla (52.4%) more than in mandible (47.6%). 2. There was a predilection for occurrence of dilaceration in female, which included 12.8% of the female compared to 11.3% in male. 3. The frequency of dilaceration in male, which included 40.5% in maxilla and 59.5% in mandible. The frequency of dilaceration in female, which included 52.4% in maxilla and 47.6% in mandible. 4. The order of frequency of dilaceration was second premolar, first premolar, lateral incisor, canine in maxilla, and first premolar, second premolar, canine, lateral incisor, central incisor in mandible. 5. In classifying of dilacerated teeth into 3 types by following appearances such as root curvature. Distal dilaceration was by far most common containing 65.8% of the cases. The least frequent was mesial dilaceration, which included 6.5% of the cases.

• The korean journal of orthodontics
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• v.23 no.3 s.42
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• pp.319-326
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• 1993

The purpose of this study was to evaluate the frequency of congenital missing teeth and supernumerary teeth in cleft patients. The subjects were divided into bilateral cleft lip and palate(BCLP), unilateral cleft lip and palate(UCLP) and cleft palate alone(CP alone) groups. 97 cleft patients(BCLP 15, UCLP 70, CP alone 12) between 6-20 years old were evaluated. Panorama film, Orthodontic chart and initial intraoral photogram were employed for this research. The obtained results were as follows. 1. The incidence of congenital missing teeth in total cleft samples was $57.7\%$, and the incidence of supernumerary teeth was $26.8\%$. Each incidence was higher than non-cleft. 2. The incidence of congenital missing teeth was the highest in BCLP and the lowest in CP alone. 3. The number of congenital missing teeth per perso was usually one, and the frequency was higher in the maxillary lateral incisors$(67.8\%)$, and maxillary second premolar$(14.9\%)$ than other teeth. 4. Most of tooth number anomalies in cleft patients were found in maxilla, especially adjacent region to the cleft site.

• 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.

• The korean journal of orthodontics
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• v.52 no.6
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• pp.451-460
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• 2022

Moebius syndrome (MBS) is a congenital neurologic disorder that causes cranio-facial abnormalities. It involves paralysis of the VI and VII cranial nerves and causes bilateral or unilateral facial paralysis, eye movement disorder, and deformation of the upper and lower limbs. The orofacial dysfunctions include microstomia, micrognathia, hypotonic mimetic and lip muscles, dental enamel hypoplasia, tongue deformity, open bite or deep overbite, maxillary hypoplasia, high arched palate, mandibular hyperplasia or features indicating mandibular hypoplasia. This case report presents a 7-year-old male patient who was diagnosed with MBS at the age 2 years. The patient displayed typical clinical symptoms and was diagnosed with Class II malocclusion with a large overjet/overbite, tongue deformity and motion limitation, and lip closure incompetency. Treatment was initiated using a removable appliance for left scissor bite correction. After permanent tooth eruption, fixed appliance treatment was performed for correction of the arch width discrepancy and deep overbite. A self-ligation system and wide-width arch form wire were used during the treatment to expand the arch width. After 30 months of phase II treatment, the alignment of the dental arch and stable molar occlusion was achieved. Function and occlusion remained stable with a Class I canine and molar relationship, and a normal overjet/overbite was maintained after 9.4 years of retainer use. In MBS patients, it is important to achieve an accurate early diagnosis, and implement a multidisciplinary treatment approach and long-term retention and follow-up.

• 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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.94-98
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• 2010

Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.118-121
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• 2012

Down syndrome (DS) is a genetic disease known as trisomy 21. Congenital cardiac anomalies are present in about 40% of DS patients. Dental anomalies are also common among DS patients. In DS patients, canine impaction is 10 times more frequent and transposition of maxillary canine and first premolar is 50 times more common than in general population. A female DS patient with congenital heart disease was diagnosed as having a transposed impacted maxillary canine. Sectional fixed appliance with Nance holding arch was used for the orthodontic treatment. After space was regained for the eruption of the canine, orthodontic button was attached using flap operation with closed technique. Traction and alignment of the tooth followed. To prevent endocarditis, prophylactic antibiotics were prescribed for the recommended dental procedures. Total treatment time was 25 months and no complication was found.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.113-117
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• 2012

Axenfeld-Rieger syndrome (ARS) is a rare disorder with variable morphology characterized by malformations of the anterior segment of the eye such as iris hypoplasia, iridocorneal adhesions, corectopia and polycoria. Craniofacial and dental anomalies are also regularly reported in connection with ARS. Dental features include hypodontia in primary and permanent dentition, microdontia, short roots and abnormally shaped teeth. The patient in this case is a 14-year-old girl who has visited Seoul National Dental Hospital in 2002 for the first time. A panorama was taken on the day of her first visit, and total absence of maxillary anterior dentition and mandibular premolars were observed. The patient was on a treatment schedule by the department of ophthalmology at Seoul National Hospital for a morphological dyplasia of the pupil and she was diagnosed with ARS. Periodic panoramic observations were performed followingly, and she was finally diagnosed of congenital missing of maxillary anteriors and mandibular second premolars. In 2009, composite resin build-ups of maxillary primary central incisors were performed, and she has been on her regular follow-ups until now.