• Maxillofacial Plastic and Reconstructive Surgery
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• v.13 no.1
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• pp.69-76
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• 1991

A 9-year old boy suffering from cleidocranial dysplasia associated with impacted 4 supernumerary teeth and unerupted all permanent teeth is presented with his mother. The pedigree showed autosomal dominant pattern of inheritance, and the raiographic features of them were very similar in clavicle, skull, vertebrae, peivis and extremities. Although almost of the skeleton was involved with this syndrome, they did not recognize any other problem but except dental problem. In mother, who was wearing removable partial dentures leaving 24 impacted teeth in her jaws, the radiographic abnormalities like cystic lesion were not detected. And in the son, who showed impacted 4 supernumerary and all permanent teeth, we have attempted surgical extraction of the supernumerary teeth and periodic surgical opening of the alveolar bone covering the permanent dentition to induce the eruption of permanent teeth at the proper position, Orthodontic treatment has also been combined to correct class III malocclusion state.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• Imaging Science in Dentistry
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• v.42 no.1
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• pp.41-45
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• 2012

Hyperdontia or supernumerary teeth without associated syndrome is a rare phenomenon, as supernumerary teeth are usually associated with cleft lip and palate or other syndromes such as Gardner's syndrome, cleidocranial dysplasia, and so on. Five patients with supernumerary teeth visited our department. They had no familial history or other pathology, certain treatment protocols was modified due to the presence of supernumerary teeth. Non-syndromic supernumerary teeth, if asymptomatic, need to have periodical radiographic observation. If they showed no variation as they impacted in the jaw, careful examination is necessary because they may develop into pathological status such as dentigerous cysts. The importance of a precise clinical history and radiographic examination for patients with multiple supernumerary teeth should be emphasized.

• The Korean Journal of Oral and Maxillofacial Pathology
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• v.42 no.5
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• pp.129-133
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• 2018

Dentigerous cysts, the most commonly occurring developmental cysts of the jaw, develop in association with impacted teeth. Most dentigerous cysts are solitary. Multiple dentigerous cysts are rare and generally occur in association with a developmental syndrome or systemic disease, such as mucopolysaccharidosis and cleidocranial dysplasia. However, in the absence of a syndrome, occurrence of multiple dentigerous cysts is rare. Development of multiple dentigerous cysts on first molars extremely rare. The purpose of this paper was to report on a nonsyndromic, 8-year-old boy who presented multiple dentigerous cysts on first molars.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.644-648
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• 2005

Supernumerary tooth was resulted from excessive proliferation of dental lamina and associated with familial tendency and a congenital syndrome such as Cleidocranial dysostosis or Gardner's syndrome. Incidence reports identify a range of $0.3{\sim}0.8%$ in primary dentition, $1.0{\sim}3.5%$ in permanent dentition with males being affected twice as frequently as females, maxilla nine times as frequently as mandible. The most common supernumerary tooth is the mesiodens, which located between the maxillary central incisors, and the next common site is the fourth molar and lateral incisors. Supernumerary teeth are uncommon in the mandible, but premolars are the most common supernumerary teeth and occurrence is very rare in the incisor region of the mandible and the incidence is 2%. We need a early diagnosis and appropriate treatment plan because of possiblilty of diastema and eruption failure displacement, rotation of the associated permanent teeth, root resorption and dentigerous cyst with presence of the supernumerary teeth. In this two case, one supernumerary tooth located in the mandibular incisor region, the other supernumerary tooth located in premolar region. We could get normal alignment of mandibular dentition by extraction and orthodontic treatment.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.2
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• pp.196-203
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• 2002

Most dentigerous cysts are solitary. Bilateral and multiple dentigerous cysts are rare and occur typically in association with a number of syndromes such as Maroteaux-Lamy syndrome, Hunter's syndrome, Basal cell nevus syndrome, Marfan syndrome, cleidocranial dysplasia. The presented case is of bilateral nonsyndromic, dentigerous cysts associated with mandibular right and left first premolars. A marsupialization procedure may be a choice of treatment for a large sized dentigerous cyst rather than an enucleation. The marsupialization procedure is recommended during the age when the erupting force of the teeth is still strong. We can expect the unerupted tooth to erupt normally. Although most of bilateral or multiple dentigerous cysts which are not associated with syndromes are rare, a bilateral dentigerous cyst without syndrome is seen. Therefore, it is wise to explain a possibility of development of new one to patient / parents in advance.

• Molecules and Cells
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• v.43 no.2
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• pp.168-175
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• 2020

Runx2 is an essential transcription factor for skeletal development. It is expressed in multipotent mesenchymal cells, osteoblast-lineage cells, and chondrocytes. Runx2 plays a major role in chondrocyte maturation, and Runx3 is partly involved. Runx2 regulates chondrocyte proliferation by directly regulating Ihh expression. It also determines whether chondrocytes become those that form transient cartilage or permanent cartilage, and functions in the pathogenesis of osteoarthritis. Runx2 is essential for osteoblast differentiation and is required for the proliferation of osteoprogenitors. Ihh is required for Runx2 expression in osteoprogenitors, and hedgehog signaling and Runx2 induce the differentiation of osteoprogenitors to preosteoblasts in endochondral bone. Runx2 induces Sp7 expression, and Runx2, Sp7, and canonical Wnt signaling are required for the differentiation of preosteoblasts to immature osteoblasts. It also induces the proliferation of osteoprogenitors by directly regulating the expression of Fgfr2 and Fgfr3. Furthermore, Runx2 induces the proliferation of mesenchymal cells and their commitment into osteoblast-lineage cells through the induction of hedgehog (Gli1, Ptch1, Ihh), Fgf (Fgfr2, Fgfr3), Wnt (Tcf7, Wnt10b), and Pthlh (Pth1r) signaling pathway gene expression in calvaria, and more than a half-dosage of Runx2 is required for their expression. This is a major cause of cleidocranial dysplasia, which is caused by heterozygous mutation of RUNX2. Cbfb, which is a co-transcription factor that forms a heterodimer with Runx2, enhances DNA binding of Runx2 and stabilizes Runx2 protein by inhibiting its ubiquitination. Thus, Runx2/Cbfb regulates the proliferation and differentiation of chondrocytes and osteoblast-lineage cells by activating multiple signaling pathways and via their reciprocal regulation.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.651-658
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• 2004

Runx2 is a transcription factor in homologous with Drosophila runt gene and it is essential for bone formation during embryogenesis and a critical gene for osteoblast differentiation and osteoblast function. Runx2-haploinsufficency causes cleidocranial dysplasia (CCD). CCD is an autosomal-dominant inherited disorder characterized by hypoplastic clevicle and delayed ossification in fontanelles and wormian bones. Dental defects are possibly shown to CCD patients : multiple supernumerary teeth, irregular and compressed permanent tooth crowns, hypoplastic and hypomineralized defects in enamel and dentin, an excess of epithelial root remnants, the absence of cellular cementum, and abnormally shaped roots. In addition, delayed eruption of the secondary dentition is a constant finding. The aim of this study is to evaluate the role of Runx2 in the tooth development and eruption through analyzing the expression pattern of Runx2 by in situ hybridization during crown (late bell stage) and root formation of tooth, using postnatal day 1, 4, 7, 14 and 21 mice mandibular molar teeth. mRNA of Runx2-full length is expressed in dental follicle and surrounding tissue at postnatal day1 and 4. At postnatal day 7, it is expressed in ameloblasts of occlusal surface of enamel and bone area surrounding the tooth. In comparison with previous stage, at postnatal day 14, it is expressed in ameloblasts of proximal surface of enamel. At postnatal day 21 it's expression is observed only in bone area. mRNA of Runx2-typeII is not expressed. At postnatal day 1 and 7. At postnatal day 14 and 21, it's expression is observed in the bone area. In this study, we suggest that Runx2 have a relation of ameloblasts differentiation and an important role to tooth eruption made by dental follicle during intraosseous eruption stage. Also we can confirm that Runx2 has a role to bone formation.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.270-274
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• 2009

Dentigerous cysts generally encompass the crown of an unerupted tooth. These cysts are usually solitary. They are the second most common odontogenic type of cysts following radicular cysts, and are frequently associated with impacted mandibular third molars or maxillary canines. Most multiple cysts found in the jaw are odontogenic keratocysts associated with the nevoid basal cell carcinoma syndrome, mucopolysaccharidoses and cleidocranial dysplasia. Although a single dentigerous cyst is well documented in the medical literature, including the prevalence, treatment and prognosis, multiple dentigerous cysts without any systemic symptoms is unusual. Furthermore, cases involving both the maxilla and mandible are especially rare. We present the case of an 11-year-old boy with nonsyndromic multiple dentigerous cysts associated with a mandibular second premolar and a maxillary canine. The treatment was conservative and included marsupialization and eruption guidance. Further follow up is planned to rule out additional problems and the possible identification of a syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.3
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• pp.444-449
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• 2002

The term 'impaction' is used to designate a tooth which remains unerupted in the jaw beyond the time at which it should normally be erupted. The main causal factors are local (lack of space, ectopic positions of teeth, supernumerary teeth, cyst, the occurrence of infectious process in the eruption path, traumatic facial injury etc.). Systemic and genetic disorders, however, may have primary failure of eruption and retarded eruption as additional symptoms (cleidocranial dysplasia, osteopetrosis etc.). Most cases of impacted teeth reported in the literature are of permanent teeth. The absence of primary teeth occur rarely whereas impaction of second primary molars is more numerous than all other impactions. Impaction due to primary failure of eruption must be distinguished from the secondary infraocclusion. The etiology of impaction of primary teeth is probably related to early ankylosis of primary teeth, but it is not clear. Failure of eruption of primary teeth may cause a number of complications, such as interference with development and eruption of succedaneous teeth, formation of cyst, and damage to adjacent teeth. This study is to report cases of primary failure of eruption in the primary dentition.