• Archives of Plastic Surgery
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• v.36 no.4
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• pp.481-484
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• 2009

Purpose: Cleidocranial dysostosis is a rare hereditary disorder affecting bones that develop by intramembranous formation. The typical features include excessive growth of transverse diameter of the skull, hypoplastic clavicles, low height and characteristic facial features. Methods: A 28 years old female patient visited by frontal area depression. The diagnosis was performed by computed tomographic study and radiographic imaging. The patient has widely opened anterior fontanelle, partial fused metopic suture, multiple wormian bone and supernumenary impacted teeth. Under the coronal incision, we exposed depressed frontal area and corrected with Medpor block carving. Results: Postoperatively, frontoparietal skull was aestheticlly improved and she was satisfied with the results. Conclusion: Authors report a case of cleiodocranial dysostosis who has been done correction of abnormal skull shape by Medpor$^{(R)}$ insertion.

• The Journal of the Korean dental association
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• v.13 no.12
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• pp.1149-1156
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• 1975

• Imaging Science in Dentistry
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• v.30 no.3
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• pp.229-234
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• 2000

Cleidocranial dysplasia (previously known as cleidocranial dysostosis) is a well-known, rare and hereditary skeletal disorder characterized by a variety of dental abnormalities and as its name implies, striking involvement of the cranial vaults and clavicles. A 17-year-old female who presented with short stature and prolonged retention of deciduous teeth, subsequent delay in eruption of permanent teeth is described. She could touch her shoulders together at the midline anteriorly. Diagnostic procedures showed hypoplasia of the maxillary and zygomatic bones, open fontanelles and sutures, and aplasia of the clavicles. The paranasal sinuses were absent or underdeveloped. Characteristically, she had near parallel-sided borders in the ascending ramus of the mandible and abnormal-shaped, the slender pointed coronoid process. The zygomatic arches had a downward bend and discontinuity at the zygomaticotemporal suture area. Radiographic and clinical investigations of her cranial and skeletal abnormalities revealed features of cleidocranial dysplasia.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.37
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• pp.41.1-41.6
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• 2015

Cleidocranial dysplasia is an autosomal dominant heritable skeletal disorder. The characteristic features of cleidocranial dysplasia (CCD) may include hypoplasia of the clavicle, delayed closure of frontanelles, late tooth eruption, and other skeletal disorders. This case report describes clinical and radiographic manifestations at the age of 11 and 29 of a CCD patient, investigates the mutation of core-binding factor A1 (CBFA1) based on gene analysis, and illustrates successful oral reconstruction with fixed prosthesis and dental implant after the extraction of multiple teeth.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.3 no.2
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• pp.91-96
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• 2007

Cleidocranial dysplasia(CCD) is a congenital genetic disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs, CCD was reported by Morand at first in 1766, And later, it was named cleidocranial dysostosis, cleidocranial dysplasia, Marie-sainton syndrome and mutational dysostosis. It is autosomal dominant disorder and there is no prevalence between man and woman. Until recent days, mutation of Runx2 in chromosome6p21 has known to be a main factor causing CCD. The specific clinical features of CCD are aplasia or hypoplasia of one or both clavicles and incomplete closing of fontanels and cranial sutures. Dental manifestations include retention of deciduous teeth, delayed eruption of permanent teeth, supernumerary teeth and cyst. Because there is no mental retardation and physical disability in CCD patients, they usually can not recognize their dental abnormality by the time of abolescence. So, after exfoliation of deciduous teeth, they usually live with edentulous status. It usually drives CCD patients to suffer from esthetic and functional problem. For this reason, CCD patients must be early diagnosed and improved in their appearance as well as masticatory function. So, surgical removal of supernumerary teeth and orthodontic eruption of the natural permanent teeth at adequate time is necessary.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.7 no.1
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• pp.49-52
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• 1977

The authors had observed a case of cleidocranial dysostosis of 42 years old unmarried woman. The observation was founded upon roentgenogram of skull P-A view, orthopantomograph, cephalometric view, chest P-A view and wrist X-ray view. The patient gave a dwarfic impression with 145㎝ body height and concaved mandibular prognastic facial feature. The palate was narrowed and extremely high arched. The eruption state of teeth were extremely poor, only four teeth were erupted, composed of upper third molar and lower three deformed teeth. The skull P-A view of roentgenogram reveals overlying metopic suture, extends from the nasion to the sagittal suture, and the suture are delayed up to date and many wormian bones are formed. On the cephalometric view the maxilla is underdeveloped and produce the false mandibular prognathism. Twenty four impacted teeth can be detected by orthopantmograph, twelve in upper jaw and twelve in lower jaw. There are many supernumerary teeth, one in upper jaw and seven in lower jaw. On the chest P-A view, the clavicles are totally aplastic, but a vestige of clavicle is visible in the right side. On the wrist roentgenogram, the epiphyseal ossification of distal phalanx, mesial phalanx and proximal phalanx is delayed.

• The Journal of the Korean dental association
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• v.23 no.6 s.193
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• pp.499-508
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• 1985

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.1
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• pp.12-15
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• 2001

Dilaceration is a tooth abnormaly occuring in the histo-morphodifferention stage resulting from disturbance between the uncalcified and already calcified portion that affects both the crown and root. The involved tooth is usually the maxillary central incisor and it also shows high prevalence of impaction. The cause of dilaceration can be either from the trauma of the primary tooth, ectopic development of the tooth germ or from cysts. And it is also found in some cases of Otodental syndrome, Hurler syndrome, Cleidocranial dysostosis. The purpose of this study is to show that periapical lesions caused by dental caries can be another factor in causing dilaceration.

• 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.25 no.1
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• pp.127-133
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• 1998

An impacted tooth is defined pathologically as a tooth that remains under the mucosa of inside bone without eruption of the crown after a specific period of eruption. Clinically, the term includes those teeth, even before eruption period, that are not expected to erupt due to shape, position and alignment of tooth and lack of space. Canine is prone to impaction more than other teeth because it has the longest time to develop and a complex route from the place of formation to the site of eruption. The impaction incidence of maxillary canine is repoted 0.92$\sim$3.3% (Ferguson, 1990). In 1995 Orton reported that the incidence was 0.92$\sim$2.2% and palatal impaction was more frequent than labial impaction(85%:15%). In 1969 Johnston presented it was more common to woman than to man(3:1). The etiology includes systemic disease such as endocrine disorder, cleidocranial dysostosis, irradiation, Crouzon syndrome, ricketts, facial hemihypertrophy and hereditary and local problems such as ectopic position of the tooth, distance of tooth from its place of eruption, malformation of the tooth, presence of supernumerary teeth, trauma of tooth germ, infection of tooth germ, displacement of tooth germ or tooth by a neoplasm, ankylosis, overretention of deciduous predecessor, lack of space for the tooth in the dental arch and mucosal barrier due to gingival fibrosis. The maxillary canine is especially important as it has the longest root, provides guidance for lateral movement of the mandible and masticatory function and assumes an important role esthetically as it is located at mouth angle. If left untreated, it may cause migration and external, internal resorption of adjacent teeth, loss of arch length, formation of dentigerous cyst or tumors, infection and referred pain as well as malposition of the tooth. Therefore, periodic examination of the development and eruption of the maxillary canine is especially important in a growing child. This case study presents the results of treatment of palatally impacted maxillary canine utilizing surgical exposure and orthodontic tooth movement on patients visiting SNUDH dept. of pediatric dentistry.