• The korean journal of orthodontics
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• v.30 no.6 s.83
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• pp.739-745
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• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or mote ectodermal structures. Characteristic manifestations Include scanty hair and eyebrows, pigmented and hyperkeratinized skin around the eyes and mouth, frontal bossing with prominent supraorbital ridges, nasal bridge depression and dental anomalies. Hyperthermia or unexplained high fever as a result of the deficiency of sweat glands is common medical history. Findings of intraoral structures are anodontia or oligodontia with conical crowns. Consequently, generalized spacing and loss of vertical dimension of occlusion. Interdisciplinary approach has been performed to treat a 10-year old boy with ectodermal dysplasia. Orthodontists and a prosthodontist worked together on this case, and the result was satisfactory.

• The Journal of Korean Academy of Prosthodontics
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• v.54 no.1
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• pp.28-34
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• 2016

Implant has been an effective treatment option for the patients with oligodontia. However, it still remains unclear when the implant should be placed. Skeletal growth that can appear even after the growth period can lead to infraocclusion of the implant which can cause functional or esthetic complications. In order to minimize these problems, definitive restorations should be placed after the functional and esthetic rehabilitation is achieved through the use of provisional restorations. Definitive restorations made with monolithic zirconia were created by replicating provisional restorations by using the latest CAD/CAM technology. These definitive restorations were delivered to the patient and clinical observation after the treatment showed satisfactory result.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.626-632
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• 2001

Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).

• Journal of the korean academy of Pediatric Dentistry
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• v.24 no.1
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• pp.179-185
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• 1997

The prevalence of congenital teeth missing is about 2-10% of population and it is more common in permanent dentition than primary dentition. In the patients with congenital missing teeth symptoms such as conical tooth, prolonged retention of primary tooth, tooth attrition. and deficient alveolar bone growth can be accompanied. In this paper the results of clinical observation of the congenital teeth missing brothers and their familial history are presented.

• The Journal of Korean Academy of Prosthodontics
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• v.51 no.4
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• pp.339-346
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• 2013

In case of missing of permanent teeth by trauma or innate defect, the decision of treatment modalities and application timing have an important effect on the prognosis of oral rehabilitation. In this case report, interdisciplinary approach between the orthodontic and prosthodontic treatment, the way to re-establish the collapsed occlusal vertical dimension, and implant prosthetic considerations will be discussed. Proper diagnosis on teeth and craniofacial skeleton was made prior to treatment and provisional restorations were used in regard of growth patterns of the patient. Finally, the edentulous areas were restored with fixed implant prostheses. Diagnosis, treatment rationale and prognosis will be discussed thoroughly.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.606-609
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• 2003

Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida occulta may be present, also ocular and dental abnormalities are reported. Radiologic findings are the osteopathy and striation of the long bone. We experienced a case of Goltz syndrome in a 9-year old female who was presented with right side hypotrophy, focal dermal hypoplasia, ocular(anidria, microcornea), dental(oligodontia, amelogenesis) and skeletal(syndactyly) abnormalities. Skin biopsy was performed and showed decreased expression of type I collagen gene with Northern blotting.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.11 no.1
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• pp.5-8
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• 2015

Neuroblastoma is a common malignant tumor of the sympathetic nervous system in childhood, arises from embryonic neural crest cells. The period of tooth development is matched with peak times of diagnosis and treatment of neuroblastoma. The intensive multimodality treatment including radiotherapy and chemotherapy is used in patients with neuroblastoma has been shown to have late adverse effects and disturbances in dental development like tooth agenesis, microdontia, enamel hypoplasia and short roots. A 8-year old girl had been on medication and radiotherapy for neuroblastoma since she was 15 months old at Department Pediatrics, Chonnam National University Hospital. Oligodontia, microdontia, and short root formation was notable in clinical and radiological examination. Mobility of lower permanent incisor was detected and measured at about degree 2. Resin wire splint using mini-screw implantation on buccal alveolar bone was conducted for maintenance of mandibular incisors and alveolar bone. Excessive mobility has been eliminated and maintained well so far. Further treatment is planned for re-evaluation of mobility, preventing dental caries and regular oral hygiene management. Although we need further evaluation, this treatment could be one of alternative therapy for those who have similar dental anomalies.

• Imaging Science in Dentistry
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• v.44 no.1
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• pp.7-13
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• 2014

Purpose: This study was performed to evaluate the prevalence of all types and subtypes of dental anomalies among 7- to 35-year-old patients by using panoramic radiographs. Materials and Methods: This cross-sectional study was conducted on 1649 people in Hamadan City, in 2012-2013. The prevalence of four types and 12 subtypes of dental anomalies was evaluated by two observers separately by using panoramic radiography. Dental anomalies were divided into four types: (a) shape (including fusion, taurodontism, and dens invagination); (b) number (including hypodontia, oligodontia, and hyperdontia); (c) structure (including amelogenesis imperfecta, dentinogenesis imperfecta, and dentin dysplasia); and (d) position (including displacement, impaction, and dilacerations). Results: The reliability between the two observers was 79.56% according to the Kappa statistics. The prevalence of dental anomalies diagnosed by panoramic radiographs was 29%. Anomalies of position and number were the most common types of abnormalities, and anomalies of shape and structure were the least in both genders. Anomalies of impaction (44.76%), dilacerations (21.11%), hypodontia (15.88%), taurodontism (9.29%), and hyperdontia (6.76%) were the most common subtypes of dental anomalies. The anomalies of shape and number were more common in the age groups of 7-12 years and 13-15 years, respectively, while the anomalies of structure and position were more common among the other age groups. Conclusion: Anomalies of tooth position were the most common type of dental anomalies, and structure anomalies were the least in this Iranian population. The frequency and type of dental anomalies vary within and between populations, confirming the role of racial factors in the prevalence of dental anomalies.

• The Journal of Korean Academy of Prosthodontics
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• v.50 no.4
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• pp.330-335
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• 2012

Shortened dental arch (SDA) as a treatment goal is the concept that stable occlusion and enough masticatory force can be achieved by restoration to the second premolars when the situation is not favorable. SDA could be applied both natural teeth and implant supported fixed prostheses. This case dealt with a patient who has grade 2 intellectual disability and a lot of missing teeth. Because of intellectual disability, patient cooperation during treatment could not be expected. Therefore every treatment should be done under general anesthesia. In addition to that, ridge resorption around molar area was severe and there were maxillary sinus pneumatization and maxillary sinusitis which increased failure probability. SDA concept was adopted to reduce risk factor and minimize general anesthesia. After the treatment, functional and esthetic improvement was achieved and oral hygiene was fortified by periodic recall check and education.

• 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.