• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.492-498
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• 1999

Oculodentodigital syndrome(ODD) was first reported by Lohmann in 1920 and termed by Meyer Schwicketath, which they called "dysplasia oculo-dento-digitalis" in 1957. It is somewhat rare heritable disease. ODD is generally inherited in an autosomal dominant pattern with a complex phenotype. The characteristic features are : (1) unique facial features, (2) microphthalmos, (3) syndactyly and camptodactyly of 4th and 5th fingers, (4) osseous anomalies of the middle phalanges of 5th fingers and toes, (5) enamel hypoplasia, (6) dry lusterless hair. We found several occlusal wearing and yellow discoloration of succedaneous teeth, multiple caries lesions, premature loss and pulpal involvement of primary teeth with associated enamel abnormalities caused by generalized enamel hypoplasia in a fairly constant oral finding. Occasionally partial anodontia, microdontia and cleft lip and palate can be manifested. This case, a 9-year-old female with repaired bilateral syndactyly was referred to pediatric dental clinic, Pusan National University Hospital for evaluation of severe attrition of teeth and caries lesions. She had most of the above mentioned typical manifestations of the syndrome. Dental treatment including caries control, stainless steel crown were performed.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.510-514
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• 2003

The Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular features include partial or complete bilateral hypoplasia of the iris stroma, abnormalities of the angle structures with congenital iris adhesions, and anterior displacement of Schwalbe's corpuscles. Common oral findings are hypodontia(especially in anterior maxillary segment), microdontia, misshaped teeth, delayed eruption of the teeth. Additionally, other systemic symptoms can be seen and early detection by the pedodontist through dental diagnosis should prevent visual impairment.

• The korean journal of orthodontics
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• v.50 no.6
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• pp.407-417
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• 2020

Objective: To investigate the dental phenotypes and treatment modalities (Tx-Mod) in Korean patients with Parry-Romberg syndrome (PRS) using longitudinal data. Methods: The samples consisted of 10 PRS patients, who were treated and/or followed-up at Seoul National University Dental Hospital between 1998 and 2019. Using a novel PRS severity index based on the numbers of the atrophy-involved area and asymmetry-involved item, we classified them into mild (n = 3), moderate (n = 2), and severe (n = 5). Dental phenotypes, including congenitally missing tooth (Con-Missing-Tooth), microdontia, tooth with short root (Short-Root), tooth with dilacerated root, and delayed eruption/impacted tooth, were investigated along with Tx-Mod. Results: The side of occurrence of all dental phenotypes showed 100% concordance with the side of PRS involvement. The most two common dental phenotypes were Con-Missing-Tooth and Short-Root (n = 29 and n = 17 in six patients). The sums of the average number of Con-Missing-Tooth and Short-Root increased from mild PRS to moderate PRS and severe PRS cases (1.0, 6.0, and 6.2). In terms of Tx-Mod, growth observation due to mild atrophy, fixed orthodontic treatment, and grafting were used for mild PRS cases. Tx-Mod for moderate PRS cases involved growth observation for surgery due to an early age at the initial visit. For severe PRS cases, diverse Tx-Mod combinations including unilateral functional appliance, fixed orthodontic treatment, growth observation, grafting, and orthognathic surgery were used. Conclusions: The novel PRS severity index may be useful to provide primary data for individualized diagnosis and treatment planning for PRS patients.

• The korean journal of orthodontics
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• v.45 no.4
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• pp.190-197
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• 2015

Objective: The aim of the study was to analyze the prevalence and distribution of ectopic eruption of the permanent maxillary first molar (EEM) in individuals scheduled for orthodontic treatment and to investigate the association of EEM with dental characteristics, maxillary skeletal features, crowding, and other dental anomalies. Methods: A total of 1,317 individuals were included and randomly divided into two groups. The first 265 subjects were included as controls, while the remaining 1,052 subjects included the sample from which the final experimental EEM group was derived. The mesiodistal (M-D) crown width of the deciduous maxillary second molar and permanent maxillary first molar, maxillary arch length (A-PML), maxillomandibular transverse skeletal relationships (anterior and posterior transverse interarch discrepancies, ATID and PTID), maxillary and mandibular tooth crowding, and the presence of dental anomalies were recorded for each subject, and the statistical significance of differences in these parameters between the EEM and control groups was determined using independent sample t -tests. Chi-square tests were used to compare the prevalence of other dental anomalies between the two groups. Results: The prevalence of maxillary EEM was 2.5%. The M-D crown widths, ATID and PTID, and tooth crowding were significantly greater, while A-PML was significantly smaller, in the EEM group than in the control group. Only two subjects showed an association between EEM and maxillary lateral incisor anomalies, which included agenesis in one and microdontia in the other. Conclusions: EEM may be a risk factor for maxillary arch constriction and severe tooth crowding.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.1
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• pp.51-56
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• 2002

Russell-Silver syndrome is a type of intrauterine growth retardation, characterized by short stature noted at birth, hemiatrophy or asymmetry, variation in sexual development and other abnormalities, including cafe-aulait pigmentation and clinodactyly. Facial features commonly associated with this syndrome are a small triangular face, decreased facial height, down-turned corners of the mouth(shark's mouth), a small mandible, and occasionally asymmetry. The major intra-oral features of the syndrome that have been reported are a high-arched palate, delayed tooth eruption, microdontia, hypodontia, and crowding. These cases were diagnosed at birth as Russell-Silver syndrome by clinical features such as prenatal growth retardation, short stature, low body weight, et al., and have been treated with growth hormone. The purpose of this paper is to report the dental findings of two patients and review the pertinent literature through the two cases.

• The Journal of Korean Academy of Prosthodontics
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• v.59 no.1
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• pp.63-70
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• 2021

Using computer-aided design and manufacturing technique improve quality of treatment in many aspect. This case reports the complete mouth rehabilitation of a patient with amelogenesis imperfecta utilizing digital technology. Clinical examination revealed loss of mastication due to insufficient occlusal stop, missing teeth, interdental spacing due to microdontia, insufficient overbite, and etc. Full veneer crowns for teeth were selected, followed by a fixed partial denture and implant placement was done using CAD-CAM guide template with bone graft for partially edentulous space. Definitive restorations were duplicated by double scanning provisional restorations and successfully delivered to the patient. These full mouth rehabilitation procedures resulted in satisfactory outcomes for the patient functionally and aesthetically.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.486-491
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• 1999

Hypoparathyroidism has abnormally decreased secretion of parathyroid hormon which responds to the blood calcium level. Wherease, Pseudohypoparathyroidism has normal activity of thyroid hormon, but end-organs, such as urinary tract and osteoclast, do not respond to parathyroid hormon. The cause of this disease is due to the mutation of Guanine stimulating(Gs) protein regulating Gs gene, which is the receptor to this hormon. Pseudohypoparathyroidism is usually noted before 20 years old on average of 8-9 years old. The clinical features of this disease includes delayed growth and development, round face, obesity, soft tissue calcification, ectopic ossification, shortening of metacarpals and metatarsals by epiphyseal closure in advance of age. The mutation of Gs gene which are found in brain, endocrine organs, and chondrocytes is the cause of those features. Reaction to Glucagon, gonadal hormon, and thyroid stimulating hormon is not expected in both cases. The common dental manifestations include enamel hypoplasia, delayed eruption, agenesis of tooth, hypodontia, dysplastic short roots, widened pulpal space, microdontia, intrapulpal calcification, and malocclusion are also often reported. This case which is diagnosed to Pseudohypoparathyroidism showed short and under-developed root of permanent troth, delayed eruption, and non-eruption of premolars and molars. And morphogenesis imperfecta of first and second premolars were also found.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.139-144
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• 2009

Laron syndrome was first described by Dr. Laron. Administration of exogenous growth hormone failed to stimulate insulin-like growth factor-I(IGF-I) production which was related to postnatal growth, because these patients lacked receptors in the liver for this hormone. The diagnosis of this syndrome is based on the typical features of GH resistance such as normal or elevated serum GH, low serum IGF-I, and impaired IGF-I response to hGH. Laron syndrome patients showed characteristically severe postnatal growth failure and markedly reduced adult height. This report describes the oral and maxillofacial manifestations of children associated with Laron syndrome. Children with Laron syndrome have several dental and skeletal irregularities. Relatively little is known of the direct effect of Laron syndrome on dental development. Further research should be needed.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.1
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• pp.144-150
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• 2008

Multimodal cancer therapy including surgery, chemotherapy, and radiotherapy could not only improve the prognosis of malignancy but also reduce the dosage and toxicity of cancer drug for treatment of malignant tumor. The effects of radiotherapy are generally localized, additive, and accumulative, and depend on dosage, site and cell sensitivity. However, in growing individuals, the dental and skeletal sequelae to radiotherapy result in dental or facial abnormalities that are irreversible : arrested root development, disturbances in enamel formation, microdontia, anodontia, altered tooth eruption and mandibular or maxillary hypoplasia. Especially, the teeth which are developing is affected according to the stage. We report three cases of developmental disturbance of permanent teeth after radiotherapy. These children had received radiotherapy for malignant tumor at the age of 3 to 4 years, in which root hypoplasia, short tapered root and early apex closure were observed. For the management of radiation caries and radiotherapy-related teeth, periodic recall check and oral hygiene instruction are required.

• 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.