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CASE REPORT |
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| Year : 2008 | Volume
: 26
| Issue : 1 | Page : 40-43 |
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Cleidocranial dysplasia: A case report
R Hemalatha1, MR Balasubramaniam2
1 Department of Pedodontics, SRM Dental College, Ramapuram, Chennai - 600 089, Tamil Nadu, India
2 Department of Orthodontics and Pedodontics, SRM Dental College, Ramapuram, Chennai - 600 089, Tamil Nadu, India
Correspondence Address:
R Hemalatha
Department of Pedodontics, SRM Dental College, Ramapuram, Chennai - 600 089, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-4388.40322
 Abstract | | |
Cleidocranial dysplasias is an autosomal dominant disorder that presents with skeletal dysplasia. The dental manifestations are mainly delayed exfoliation of primary teeth and delayed eruption of permanent teeth, with multiple impacted supernumeraries. This report addresses the complex nature of the treatment modalities. In our patient, surgical exposure of unerupted teeth was done with orthodontic traction. Post-surgical follow-up was uneventful.
Keywords: Autosomal dominant, cleidocranial dysostosis, delayed eruption, impacted supernumerary, skeletal dysplasia
How to cite this article:
Hemalatha R, Balasubramaniam M R. Cleidocranial dysplasia: A case report. J Indian Soc Pedod Prev Dent 2008;26:40-3 |
| Introduction | |  |
Cleidocranial dysplasias shows an autosomal dominant inheritance pattern. [1] It is associated with a spontaneous mutation in the gene coding for osteoblast transcription factor Runx2, which is essential for osteoblast and dental cell differentiation as well as for bone and tooth formation. [2] There is a mutation in Cbfa1 (Core binding factor); the affected molecule is the transcription factor and the phenotype shows abnormal clavicles, wormian bones, and supernumerary teeth. The gene has been mapped to chromosome 6p21 in the region containing Cbfa1, which controls the differentiation of precursor cells into osteoblasts. [3],[4] Runx2, also known as OSH2, Cbfa1, PEBP2aA, AML-3 belongs to runt domain gene family. [4]
In this article, we present a case report of a child with the classical features of cleidocranial dysplasia.
The pathology is due to an early developmental disorder of mesenchyme or connective tissue, causing retarded ossification of bone precursors, especially at junctions, which can lead to defective ossification or even failure of ossification of portions of skeletal structures. The syndesmosis between cranial bones and the symphysis of other bones are basically connective tissue junctions. The medial and lateral centers of ossification of the clavicles are separated by a fibro-cellular structure. (The distribution follows a pattern in which syndesmosis or suture lines of connective tissue and bone in which nuclei grow into proliferative cartilage by direct apposition of fibro cellular layer). [1]
Counts et al . [5] reported that cleidocranial dysplasia is a systemic disorder that manifests itself as a condition in which teeth fail to erupt. It is logical to assume that the formation and maintenance of root cementum could be affected.
The anomaly manifests as almost complete absence of cellular cementum and an increase in the amount of acellular cementum of the roots of the affected teeth. This was thought to be the probable cause for failure of eruption of a significant number of teeth in patients affected with cleidocranial dysplasia. [2]
| Case Report | | |
A 12-year-old male child reported with his parents to our department with the complaint of unerupted upper and lower anterior teeth for the past 6 months. The patient had a moderate build, clubbing, slurred speech, and shrugged shoulders. He was diagnosed as a case of cleidocranial dysplasia with absence of clavicles [Figure - 1].
The medical history revealed ventricular septal defect closure 2 years back. Oral and dental examination revealed competent lips and retained deciduous teeth 52, 62, and 71 and unerupted 11, 12, 21, 22, 31, 41, and 42 [Figure - 2].
Orthopantomographs, occlusal and full mouth intraoral periapical radiographs were taken, a) for detection of impacted supernumeraries and unerupted teeth, b) to reconfirm the position of the impacted supernumeraries, and c) for periapical assessment. The orthopantomographs revealed impacted permanent teeth and multiple supernumeraries both in the maxilla and the mandible (six in number).
Extraction of 52, 62, and 71 was done under local anesthesia and with prophylactic antibiotic cover after obtaining consent from the parents. Since the patient had undergone ventricular septal defect closure 2 years back, prophylactic antibiotics were given prior to extraction according to the regimen recommended by the Institute of Cardiovascular Disease (a unit of Madras Medical Mission) in order to protect the patient from bacterial endocarditis. Healing of extraction wounds was satisfactory [Figure - 2].
Wound healing was satisfactory. Upper and lower impressions were then made to fabricate temporary partial denture [Figure - 3] for esthetic rehabilitation. The patient used the denture for 2 months, after which he discontinued its use.
This patient had also developed a tongue thrusting habit; a Nance palatal arch space maintainer with a fixed tongue crib was given to interrupt the habit [Figure - 4].
The patient was posted for surgery after almost a year, the delay being due to some personal need of the patient. Unerupted impacted teeth 11, 12, and 21 were exposed under local anesthesia by crevicular incisions, a mucoperiosteal flap was raised, orthodontic brackets were placed, and traction was applied by attaching tie wires to the habit-breaking appliance [Figure - 5],[Figure - 6],[Figure - 7].
The case was followed up. The right upper central incisor (11) erupted after 3 months and, simultaneously, an eruption bulge appeared at the site of the left upper central incisor (21) [Figure - 8]. The treatment plan included surgical exposure of the lower anteriors and, at a later date, removal of the supernumeraries [Figure - 9].
| Discussion | | |
An anomaly in the eruption of the anterior teeth can interfere with facial esthetics and lead to other clinical problems. If the impacted tooth is extracted, loss of alveolar bone can be anticipated; following the healing period, the alveolar ridge becomes thin and deficient. To overcome these problems, in this case, orthodontic treatment was chosen to facilitate eruption of natural teeth. Forced eruption of impacted teeth must always be considered in young patients since it can lead to favorable results from a periodontal, occlusal, and esthetic perspective.
The treatment objectives were to redistribute the space in the maxillary anterior region, to surgically expose the impacted maxillary central incisors and guide them into position, and to expand the maxillary arch to gain additional space for tooth alignment. [6]
Crevicular incision of oral mucosa immediately overlying the impacted tooth was done, after which orthodontic brackets were bonded to the labial surface of the impacted incisors (11, 12, and 21). Orthodontic traction was applied with ligature wires attached to the fixed tongue crib.
Long-term monitoring of the stability and periodontal health of impacted incisors is necessary following orthodontic traction. [7] Bayram [7] reported a case of bilateral impacted maxillary central incisors that was corrected by surgical exposure and orthodontic treatment. Similar cases were reported by Tsai, [8] Garvey et al . [9] Thosar and Vibhute, [10] Uematsu et al ., [11] Tanaka et al ., [6] and Ibricevic et al . [12]
| Conclusion | | |
For patients with compromised esthetics, surgical treatment with orthodontic traction is a convenient and viable alternative. However, strict aseptic protocol should be followed in order to perform safe and successful surgical procedures.
For eruption of impacted teeth, especially in young patients, surgical exposure is preferred as it can be completed in one or two sessions. Orthodontic occlusal movement of the tooth will restore the height of the alveolar ridge to that compatible with normal dental and skeletal growth. Normal periodontal attachment and gingival margins can be achieved, thus eliminating the need for additional periodontal therapy. [8]
| References | | |
| 1. | Feldmann VB. Cleidocranial dysplasian: A case report. JCCA J Can Chiropr Assoc 2002;46:185-91  |
| 2. | Chen S, Santos L, Wu Y, Vuous R, Gay I, Schulze J, et al . Altered gene expression in human cleidocranial dysplasia dental pulp cells. Arch Oral Biol 2005;50:227-36 |
| 3. | Furuuchi T, Kochi S, Sasano T, Likubo M, Komai S, Igari K, et al . Morphological characteristics of masseter muscle in cleidocranial dysplasia: A report of three cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:185-90 |
| 4. | Markovic MD. At the crossroads of facial genetics. Eur J Orthodont 1992;14:469-81 |
| 5. | Counts AL, Rohrer MD, Prasad H, Bolen P, et al . An assessment of root cementum in cleidocranial dysplasia. Angle Orthodont 2001;71:293-8 |
| 6. | Tanaka E, Watanabe M, Nagaoka K, Yamaguchi K, Tanne K, et al . Orthodontic traction of an impacted maxillary central incisor. J Clin Orthod 2001;35:375-8 |
| 7. | Bayram M, Ozer M, Sener I, et al . Bilaterally impacted maxillary central incisors. J Contemp Dent Pract 2002;7:98-105 |
| 8. | Tsai TP. Surgical repositioning of an impacted dilacerated incisor in mixed dentition. JADA 2002;133:61-6 [PUBMED] [FULLTEXT] |
| 9. | Garvey TM, Barry HJ, Blake M, et al . 2 nd . Supernumerary teeth: An overview of classification, diagnosis and management. J Can Dent Assoc 1999;65:612-22 |
| 10. | Thosar NR, Vibhute P, et al . Surgical and orthodontic treatment of an impacted permanent central incisor. J Indian Soc Pedod Prev Dent 2006;24:100-3 |
| 11. | Uematsu S, Uematsu T, Furusawa K, Deguchi T, et al . Orthodontic treatment of an impacted dilacerated maxillary central incisor combined with surgical exposure and apicoectomy. Angle Orthod 2004;74:132-6 [PUBMED] [FULLTEXT] |
| 12. | Ibriceive H, al-Mesad S, Mustag Rudic D, Al-Zohejry N, et al. Supernumerary teeth causing impaction of permanent maxillary incisors. J Clin Pediatr Dent 2003;27:327-32 |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]
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