• 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.25 no.3
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• pp.598-603
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• 1998

Amelogenesis imperfecta is a group of hereditary defects of enamel unassociated with any other generalized defects. The prevalence of this condition has been estimated to range from 1 in 14,000 to 1 in 16,000, depending on the population studied. It may be differentiated into three general types : hypoplastic, hypocalcified, and hypomaturation, depending on the clinical presentation of the defects and the likely stage of enamel formation that is primarily affected. The dentin and root form are usually normal, but the enamel may lack the normal prismatic structure, being laminated throughout its thickness or at the periphery, with the result that these teeth are more resistant to decay. This case is that of an six-year-old girl brought to the pediatric dentistry department by her parents for esthetic reasons and also because of slight dental sensitivity. Clinical and radiographic examinations confirmed amelogenesis imperfecta. The author has treated with the crowning of the primary molars, using prefomed NiCr crowns and periodic fluoride application on whole dentition.

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.2
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• pp.323-328
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• 2006

Dentinogenesis imperfecta is an inheritable disease of dentinal defect, generally is inherited as a single autosomal dominant trait. It has a prevalence of 1 in 8000 with the trait, and no significant difference between male and female, with involvement of the primary and permanent teeth. Shields proposed three types of Dentinogenesis imperfecta. Affected teeth have various discoloration, separation of enamel rapid destruction of underlying dentin, and severe attrition. Radiographically, the teeth have cervical constriction, bulbous crown, thin root, obliteration of the root canals and pulp chambers, and periapical lesions in a sound tooth. The objective of treatment is rehabilitation of the esthetics, the masticatory function, and the vertical dimension of occlusion. In these cases, two pediatric patients reported to the Kyungpook University Pediatric clinic, with a chief complaint of discolored teeth and severe attrition. As a result of clinical and radiographic exam, it was diagnosed as Dentinogenesis imperfecta. The posterior teeth were restored with Stainless Steel Crown, and the anterior teeth were restored with composite resin veneering.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.96-101
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• 2009

Regional odontodysplasia(ROD) is relatively rare localized developmental anomaly of tooth formation in which hard tissue is affected. The maxilla is typically affected than the mandible, and especially the maxillary left quadrant is the most commonly involved. Females are affected twice as often as males, and there is no association with race. Its etiology remains undetermined, but local circulatory disorders, somatic mutations, virus infections, local trauma, hyperpyrexia, irradiation, metabolic disturbances, and hereditary transmission are considered as possible etiologic factors. The affected teeth are likely to be small, hypoplastic, brown, and grooved. Eruption failure or delay is frequently seen as well as abscess or fistulae formation in absence of caries. Radiographically, there is a lack of contrast between the enamel and dentin, both of which are less radiopaque than unaffected counterparts. Moreover, enamel and dentin layers are thin, giving the teeth a “ghost-like appearance”. The pulp chambers and canals are large, the roots seem like to be short and indistinct. A 2-year-3-month old boy came to the department of pediatric dentistry, Yonsei University, with the chief complaint of delayed eruption and abnormal tooth shape on the lower left quadrant. He was diagnosed as regional odontodysplasia based on the clinical and radiographic findings.

• 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.31 no.4
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• pp.721-728
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• 2004

Velopharyngeal closure is a sphincter mechanism between the activities of the soft palate, lateral pharyngeal wall and the posterior pharyngeal wall, which divides the oral and nasal cavity. It participates in physiological activities such as swallowing, breathing and speech. It is called a velopharyngeal dysfunction when this mechanism malfunctions. The causes of this dysfunction are defects in (1) length, function, posture of the soft palate, (2) depth and width of the nasopharynx and (3) activity of the posterior and lateral pharyngeal wall. The purposes of this study are to analyze the nasopharynx of cleft palate patients using cephalometry and to evaluate the degree of hypernasality using nasometry to find its relationship with velopharyngeal dysfunction. The following results were obtained : 1. In cephalometry, there were significant differences in soft palate length, soft palate thickness, nasopharyngeal depth, nasopharyngeal area, and adequate ratio between two groups. 2. In nasometry, there were significant differences between two groups in vowel /o/ and sentences including oral consonants. 3. In cleft palate patients, though no general correlation was found between Anatomic VPI and nasalance scores, vowel /i/ and sentences including oral consonants were slightly correlated. In conclusion, cephalometry and nasometer results were significantly different between the two groups. Though in the cleft palate group, Anatomic VPI and nasalance scores, which are indices for velopharyngeal closure, excluding the vowel /i/ and sentences including oral consonants show generally no significance.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.2
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• pp.331-338
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• 1999

Chemoradiation therapy used on pediatric oncology patients often causes dental developmental anomalies that affect future dental care. Defects noted include tooth and root agenesis, root thining and shortening, and localized enamel defects. The effect of radiotherapy usually are confined to the radiation site, but the effects of chemotherapy may be more wide spread because of its systemic distribution and structures and organs unrelated to the primary tumor may be affected. Many pediatric cancers are treated with a combination of radiation and multiagent chemotherapy to create synergic and additive effects. Dental treatment affected by chemoradiation damage to developing teeth includes orthodontic tooth movement, prosthetic abutment considerations, periodontal health, space maintenance, requirements for home fluoride regimens to protect hypomineralized areas, restoration options for hypoplastic/hypomineralized teeth, and endodontic procedures. The following case demonstrate chemoradiation therapy effects on the dental development.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.3
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• pp.481-488
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• 2009

Buckley's formocresol was first introduced as a pulp medicament in 1904, and since 1930, it has been the treatment of choice for primary molar pulpotomies. Formocresol has fixation effect of pulp tissue and high clinical success rate. But side effect such as displacement and loss of permanent successor, amelogenesis imperfecta, cyst formation, mutation by general absorption, possibility of cancer induction have been reported. Of those, dentigerous cyst can form in the periradicular region after formocresol pulpotomy caused by an alteration of the reduced enamel epithelium, which result in fluid accumulation between the epithelium and the tooth crown. The present case describes a 6-year-old girl who had accidentally discovered in the panoramic radiograph a single, well-defined, radioluscent area enclosing the second unerupted mandibular premolar. The second left primary molar had been pulpotomizied 3 year before. Surgical treatment was carried out, the primary molar was extracted and cystectomy was performed under local anesthesia. In the extracted second primary molar, formocresol cotton pellet was left in the pulp chamber. Histologic study confirmed the suspected diagnosis of dentigerous cyst. This report present a case of dentigerous cyst associated with inadequate formocresol pulpotomized deciduous molar.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.224-228
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• 2005

The effect of primary tooth infection on permanent teeth was reported by many previous study. It is seen histologic change in the enamel forming organ and in the surrounding sac, thus produce the defect as either hypoplasia or hypocalcification and alter the eruption of the permanent teeth. The periapical inflammation cause permanent tooth displacement in various direction and can be classified into the three categories : rotation, deflection inversion. This study reported case which displacement of succedaneous teeth may be caused by periapical infection of primary dentition and concluded that treatment procedure of infected primary tooth is selected and performed by importance to retain the tooth for space maintain, possibility to successfully restored, evidence of abnormal development of the succedaneous tooth.

• Journal of the korean academy of Pediatric Dentistry
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• v.38 no.1
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• pp.82-87
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• 2011

The pulp infection of primary tooth is often caused by dental caries or trauma. But, if it is not managed properly, it can be produce the periapical lesion. The periapical lesion can cause some complications such as enamel hypoplasia, displacement, root dilaceration and impaction. Treatment options of displaced permanent successor are clinical and radiological follow-up after extraction of primary teeth, surgical opening, orthodontic traction, transplantation and extraction. In these cases, the premolars with abnormal eruption path caused by periapical lesion of the primary tooth have shown successful spontaneous eruption just as a result of extraction of infected primary tooth and space maintenance.