• Journal of Chest Surgery
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• v.32 no.6
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• pp.584-587
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• 1999

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.251-255
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• 2005

Radiotherapy for head and neck tumors is a viable treatment modality. However, a wide range of potentially debilitating dental complications may be accompanied by this treatment. We report two cases of developmental disturbance of permanent tooth germs after radiotherapy. The one was that of a seven-year-old girl, who had congenitally missing teeth, and microdontia of permanent tooth germs. she had received radiotherapy for acute myelocytic leukemia at the age of 19 months. The other was that of a nine-year-old boy, in which congenitally missing teeth, microdontia, root hypoplasia, and enamel hypoplasia of permanent teeth were observed. He had undergone a course of radiotherapy for bilateral retinoblastoma at the age of 13 months.

• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.3
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• pp.447-453
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• 2007

The purpose of this study was to investigate the relationship between morphology and number of deciduous teeth and the occurrence of other dental anomalies in their successors, and to evaluate the necessity of early diagnosis of dental anomalies in the primary dentition. Prevalence of double teeth and congenital missing teeth was investigated in 254(134 boys, 120 girls) panoramic radiographic films, taken by 2 to 7-year-old children in Chonnam National University Hospital from 2000 to 2005. And then it was examined that relationship of anomalies of the primary dentition and their successors. Among them 11 children(6 boys, 5 girls) had double teeth or missing teeth. And prevalence of the double teeth was 1.6% and missing teeth was 3.1%. One subject had double teeth in in the mandible and missing teeth in the maxilla. Of the 11 cases of dental anomalies in primary dentition, 7 cases had congenital missing tooth in their successors. This study suggests that the dental anomalies in the primary dentition induced high prevalence of the congenital missing of permanent successors in the permanent dentition.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.180-187
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• 2015

Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

• Journal of the korean academy of Pediatric Dentistry
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• v.50 no.2
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• pp.179-191
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• 2023

The purpose of this study was to investigate the prevalence and patterns of congenitally missing teeth in permanent teeth excluding third molars, in patients aged 8 to 16 years who visited Pusan National University Dental Hospital from January 2010 to February 2021. This retrospective study evaluated tooth agenesis and the pattern of missing teeth represented by the tooth agenesis code by reviewing panoramic radiographs and electronic medical records of 11,759 patients, including 5,548 females and 6,211 males. The prevalence of congenitally missing teeth was 10.74% (females 11.95%, males 9.66%, p < 0.0001). Patients with tooth agenesis had an average of 2.22 missing teeth, and congenitally missing teeth occurred more frequently in the mandible (8.39%) than in the maxilla (4.52%, p < 0.0001). The mandibular second premolar (58.19%) was the most frequently missing tooth. The second premolar was the most frequently missing tooth in all quadrants (30.10%, 31.67%, 43.14%, and 35.59%) when a single tooth was absent, while the first and second premolars were the most commonly absent teeth (11.69%, 11.47%, 5.94%, and 5.24%) when two or more teeth were missing. In the relationship between maxillary-mandibular antagonistic quadrants and full mouth, the 1st to 4th place of the missing patterns were all involved with the 1st and 2nd premolars. This study can be clinically helpful in establishing a treatment plan for patients with missing teeth. In addition, it can be used as basic data for molecular biological research to find out the relationship between tooth agenesis and specific genes.

• The korean journal of orthodontics
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• v.31 no.1 s.84
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• pp.51-61
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• 2001

Cleft lip and/or palate (CLP) is one of the most common congenital craniofacial anomalies and occurs more frequently in Asian people. Dental abnormalities in number, size, shape, and eruption of teeth are frequently associated with CLP. The purposes of this study were to investigate the effects of CLP on number, size, shape and eruption of teeth and to provide basic clinical data for diagnosis and treatment of the CLP patients. With the orthodontic and cleft charts, diagnostic models, orthopantomograms and intraoral x-ray films from 241 CLP patients who visited Dept. of Orthodontics, Seoul National University Dental Hospital, we evaluated the frequency of congenital missing teeth, supernumerary teeth, Impacted teeth, and microdontia. The results were as fellows ; 1. Frequency of congenital missing was relatively high up to $56.8\%$. Congenital missing occurred frequently in the maxillary lateral incisor and the maxillary second premolar. Among the CLP types, frequencies of congenital missing in cleft lip and Palate group and cleft lip and alveolus group were higher than those of cleft lip group and cleft palate group. And bilateral cleft showed higher frequencies than unilateral ones. 2. Supernumerary tooth was shown in $11.2\%$ of CLP patients. It occurred frequently in the area between the maxillary lateral Incisors and the maxillary canine. Among the CLP types, cleft lip group showed relatively most highest frequency. 3. Impaction was shown in $18.3\%$ of CLP patients. It occurred most frequently In the maxillary lateral incisor and the maxillary canine than other teeth. Among the CLP types, cleft lip group and cleft lip and palate group showed most highest frequencies. 4. Microdontia was shown in $15.8\%$ of CLP patients. It occurred the most frequently In the maxillary lateral incisors and maxillary canines. Among the CLP types, cleft lip and alveolus group and cleft lip and palate group showed relatively higher frequencies. There was no microdontia in cleft palate group.

• Journal of the korean academy of Pediatric Dentistry
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• v.2 no.1
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• pp.53-56
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• 1975

We observed the orthopantomography taken by 800 children who came to our pedodontic department, School of Dentistry, S.N.U. and also got the incidence of supernumerary and congenital missing teeth as follows. (1) The children who had supernumerary or congenital missing teeth were 9.75% of all. (2) In incidence of supernumerary teeth, male was 0.50% higher than female. (3) Incidence of conginital missing teeth was 7.0% and male was 1.0% higher than female. (4) Incidence of congeintal missing teeth was mainly arised from mandible.

• The korean journal of orthodontics
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• v.28 no.6 s.71
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• pp.981-989
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• 1998

Positions, angulation and mesiodistal dimension of lower incisors are important in esthetics, occlusion and post-treatment stability of tower arch. When lower incisor is congenitally missing, problems such as increased overjet and overbite, closing in of adjacent teeth and size/space discrepancies may occur. When creating treatment plans, incisor position and angulation, lip support, anteroposterior skeletal relationship canine-molar relationship, overjet overbite, remaining growth potential, crowding and anterior tooth ratio have to be considered. For an accurate analysis of incisal size discrepancy, diagnostic model set-up may be helpful. The two patients in this presentation both had two lower incisor missing, but the degree of crowding, skeletal relationship, lip support, molar relationship are different and therefore treatment plan was different as well. Long term follow-up may be necessary for stability and retention.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.88-91
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• 1997

Congenital long-segment tracheal stenosis with complex cardiac anomaly has generally been regarded as a fatal disease This report described the successful concomitant repair of unexpected congenital tracheal stenosis and complex cardiac anomaly with the use of edrdiopulmonary bypass. The patient was a 3-month-old girl with coarctation of aorta, V D, and PDA. The presence of tracheal stenosis was not discovered until when difficulty with endotracheal intubation was encountered at operating room. Thus, we decided concomitant repair of both lesions and performed anterior pericardial tracheoplasty combined with one stage repair of coarctation of aorta, VSD, and PDA under the cardiopulmonary bypass. The patient is doing well without any signs of complication at present, 2 years and 1 month after the operation.

• The Journal of the Korean dental association
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• v.40 no.9 s.400
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• pp.667-679
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• 2002

일반 특수신체 정신장애자나 연소연로자의 병약 문제에 대해서는 일단 복지차원으로 국가가 배려하고 있으나 치과분야에서의 절대 무치악자, 선천성 기형 구강암 수술 후의 다량의 조직결손 등의 특수질환자에 대해서는 조명되지 않고 간과돼 온 것이 사실이다. 기술적인 면에서나 경제적인 면에서 일반적인 방법으로는 치료가 불가능한 치과 특수장애자를 위한 특수진료원을 국가가 정책적으로 설립하는 것이 당연하다는 것을 객관화할 수 있는 근거를 본 연구에서는 제시하고 있다. 치협의 학술국 연구사업으로서 국립구강악안면특수장애 수복진료원 설립을 위한 기초자료 조사를 완료하여 지난 4월 보고한 내용을 게재한다.