• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.4
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• pp.274-282
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• 2013

The goal of this study was to analyze prevalence and distribution of congenitally missing teeth in 3302 patients from 7 to 15 years old, who had visited and taken panoramic radiographs at the Department of Pediatric Dentistry, from January 2008 to December 2012. The prevalence of hypodontia in the males (5.9%) was slightly higher than in the females (4.7%). In the affected patients, the mean number of congenitally missing teeth in the mandible was statistically higher than in the maxilla (p < 0.05). However, there was no significant difference in the number of congenitally missing teeth between left and right dentitions (p > 0.05). Excluding the Mn. third molars, the most frequent congenitally missing teeth site was the Mn. second premolars. In contrast, the most infrequent congenitally missing teeth sites were the Mx. and Mn. first premolars, and Mx. central incisors. The bilateral combination or antagonistic quadrants combination of congenitally missing teeth in hypodontia patients was 80.3%. The result of this study will contribute to clinicians to make accurate diagnosis and establish favorable treatment plans, by offering invaluable data about the number and distribution of congenitally missing teeth.

• 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.32 no.4
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• pp.682-686
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• 2005

Incontinentia pigmenti(IP), so called Block-Sulzberger syndrome is a rare genodermatosis that occurs almost in female infant; usually lethal in males, X-linked dominantly inherited disorder. IP is characterized by abnormalities of mesodermal and ectodermal tissues including eye, tooth, skin, nail, breast and hair as well as neurological deficiencies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape and so on. Here is a case of 6 year-old female with IP. She had congenital missing of primary and permanent teeth, delayed eruption, maxillary deficiency and extra cusps, resulting in unstable occlusion. Systemically, she had a history of operating eyes due to problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP.

• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.4
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• pp.658-665
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• 2007

Oligodontia is defined as the congenital absence of six or more teeth in dentition, excluding the third molars. The prevalence of congenital missing teeth is about 1.6 to 9.6% of population and the prevalence of oligidontia is about 0.08 to 1.1%. The mandibular second premolar is the most frequently absent after the third molar, followed by the maxillary lateral incisor and upper second premolar. Females seem to be affected slightly more than males. Oligodontia may occur either in isolation, or as a part of a syndrome such as ectodermal dysplasia. Different causes are possible for oligodontia: physical obstruction or distruction of the dental lamina, space limitation, functional abnormalities of the dental epithelium, failure of induction of the underlying mesenchyme, chemotherapy, radiotherapy or genetic factor. Because oligodontia would result in esthetic and functional problems, such as facial asymmetry or occlusal disharmony, early diagnosis from clinical and radiographic examination was necessary. And appropriate treatment plan should be followed. This case report was about oral conditions and treatment of the oligodontia patients who have no specific systemic disease.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.563-567
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• 2009

Incontinentia pigmenti(Bloch-Sulzberger syndrome) is a sex-linked hereditary disorder so girls are almost exclusively affected. The frequency rate is approximately 1:40,000 among girls. It is associated with skin, ocular, dental, skeletal and central nervous deformities. We reported 2 cases of medically diagnosed IP patients who were 4 and 5 years old girls. They had thin and sparse hair, and represented congenital missing of multiple primary and permanent teeth, accessory cusps and cone-shaped crowns. Therefore we report the dental manifestations and treatment progress.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.245-252
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• 2009

The congenital missing of teeth is common, which takes place since the proliferation and differentiation are not allowed in that tooth bud fail to start development. The purpose of this study is to research incidence rate, number, and missing part of congenital missing teeth, and to study whether a person who has missing teeth has other abnormality of teeth or not. For this study, 1,520 subjects(aged 2.9$\sim$17) who had visited pediatric dentist department of Chonbuk national university dental hospital within 2 years were examined with an panoramic radiograph; exempting third molar missing state. The obtained results are as follows. 1. 8.88% among total subjects show missing teeth; male 9.05%, female 8.64% 2. The most frequently missing permanent teeth were the mandibular second premolars(22.3%). The most frequently missing primary teeth are mandibular lateral incisors(50%). 3. 43.3% patients have one permanent missing tooth, 34.3% have two, and 10.4% have more than six, respectively. In primary teeth, 86.7% patients have one missing tooth, and 13.3% have two missing teeth. 4. 18 patients(13.3%) have missing teeth as well as hyperdontia, while some patients have microdont, ectopic eruption, and fusion teeth.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.2
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• pp.233-239
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• 2010

Space loss of dental arch can appear when the proper position of teeth within the dental arch changes by a certain cause, because the balance of force makes changes about tooth position as well as alignment. The causes of space loss include proximal caries, early extraction, congenital missing of a tooth and hypodontia, etc. Among those causes of space loss, congenital missing of a tooth is more rarely observed in the primary dentition than in the permanent dentition. Congenital missing in the primary dentition is associated with that in the permanent dentition. Furthermore, it can cause space problem, such as mesial tilting or drift of adjacent teeth, space loss for permanent successors and dental arch constriction, etc. Primary lateral incisors is the most commonly involved, in the maxilla rather than in the mandible, but primary canine is rarely reported. In this patient, who visited the department of pediatric dentistry at Yonsei university dental hospital, it was observed that the maxillary right primary canine was congenitally missing and an odontoma was found insteadly. However, neither the space loss for the congenitally missing primary canine nor midline deviation is remarkable during the 2-year-10-month observation period. In addition, any clinical or radiographical symptom did not occur in spite of odontoma. Therefore, surgical enucleation of odontoma is planned according to the eruption of permanent lateral incisor or canine, unless eruption failure of permanent lateral incisor or canine nor cystic change around the odontoma is occurred. Through further evaluation, space maintainer or orthodontic treatment may be necessary.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.654-660
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• 2001

Dentinogenesis imperfecta is an example of an inheritable dentinal defect originating during the histodifferentiation stage of tooth development, with involvement of the primary and permanent teeth. Shields, Bixler and El-Kafrawy proposed three types of Dentinogenesis imperfecta : Type I, II, III. Witkop reported a prevalence of 1 in 8000 with the trait, and no significant difference between male and female. Affected teeth have red-brown discoloration often with distinctive wearness of occlusal surface of posterior teeth and incisal surface of anterior teeth. Once enamel seperated from underlying defective dentin, the dentin demonstrates significantly acclerated attrision. Radiographically, the teeth have thin roots, bulbous crown, cervical constriction, and obliteration of the root canals and pulp chambers. In primary dentition periapical lesions or multiple root fractures are often observed. In successive generations the phenotypes of discoloration and wearness of teeth occurred, and one of the patient's subships, 10 year-old sister, showed general discoloration of her teeth and mild wearness. In this case, a 4 year-old male reported to the Yonsei University Pedodontics clinic, with a chief complaint of discolored teeth. The teeth showed generally yellowish-brown discoloration and moderate wearness. In radiographic features, obliteration of pulp, bulbous crown, and short roots were observed. It was diagnosed as Dentinogenesis imperfecta. The posterior teeth were restored with Stainless Steel Crown, and defective incisors including left upper primary central incisor which was extracted due to a root fracture with Open-faced Stainless Steel crown.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.1
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• pp.124-129
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• 2010

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine growth retardation and post-natally by dwarfism, severe microcephaly, bird-headed profile with receding chin, prominent nose, joint defects, clubfoot, sparse hair, malformation of genitourinary tract and rectum, mental retardation and hematological disorders. There is also a reduction in the number of blood cells. Dental anomalies of Seckel syndrome are crowded teeth with malocclusion, enamel hypoplasia, absence of some teeth and taurodontism. This report described the oral and maxillofacial manifestations of children associated with Seckel syndrome. Children with Seckel syndrome have several dental and skeletal irregularities. The purpose of this study was to report the dental and medical characteristics of the patient and review the literatures of Seckel syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.3
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• pp.241-246
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• 2014

The purpose of this study was to find out prevalence of peg-laterals and to evaluate the frequency of dental anomalies in Korean children with peg-laterals. For this study, panoramic radiographs of 3,278 patients (aged 7 to 15 years) from the Department of Pediatric Dentistry admitted from January, 2008 to January, 2013 were selected for an investigation on peg-laterals distribution. The prevalence of peg-laterals was 2.62% (86 subjects). Among the peg-laterals children, the distribution of associated dental anomalies were as follows: congenitally missing teeth (29.1%), dens invaginatus (19.8%), impacted teeth (12.8%), supernumerary teeth (9.3%), and transposition (4.7%). Due to this study showing frequent occurrences of peg-laterals with other dental anomalies, one suggestion is to consider such relationships before deciding on a diagnosis and treatment plan.