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ORIGINAL RESEARCH Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 3  |  Page : 253-256
Cementum analysis in cleidocranial dysostosis


1 Department of Oral and Maxillo-Facial Pathology, Meenakshi Ammal Dental College and Hospitals, Chennai - 600 095, India
2 Department of Oral and Maxillo-Facial Surgery, Meenakshi Ammal Dental College and Hospitals, Chennai - 600 095, India

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Date of Submission14-Dec-2007
Date of Decision27-Feb-2008
Date of Acceptance29-Feb-2008
 

   Abstract 

Objective: Cleidocranial dysostosis (CCD) is a skeletal disorder associated with dental anomalies such as failure or delayed eruption of permanent teeth and multiple impacted supernumerary or permanent teeth. Absence of cellular cementum at the root apex is presumed to be one of the factors for failure or delayed eruption. The aim of this study was to analyze the root cementum of supernumerary(S) and permanent teeth (P) of a CCD patient and to compare the findings with those of normal individuals with special emphasis on delayed eruption.
Materials and Methods: Ground sections of 12 extracted teeth (9S + 3P) of a CCD patient and 12 teeth of normal individuals (5S + 7P) were taken for the study. The sections were longitudinal and 50 m thick. Root characteristics were recorded with the aid of a light microscope.
Results: The absence of apical cellular cementum, increased root resorption and increased percentage of the gap type of cemento-enamel (C-E) junctions were significant findings noted both in the supernumerary and permanent teeth of the CCD patient.
Conclusion: The presumption of cellular cementum is essential for tooth eruption is not supported by the findings of the present study. Delayed exception could be attributed to various other factors like mechanical obstruction, reduced serum alkaline phosphatase levels etc. Also, the significance of the increased percentage of the gap type of C-E junctions and its role in tooth eruption has to be further evaluated.

Keywords: Acellular cementum, cellular cementum, cleidocranial dysostosis, gap type C-E junction, root analysis

How to cite this article:
Manjunath K, Kavitha B, Saraswathi T R, Sivapathasundharam B, Manikandhan R. Cementum analysis in cleidocranial dysostosis. Indian J Dent Res 2008;19:253-6

How to cite this URL:
Manjunath K, Kavitha B, Saraswathi T R, Sivapathasundharam B, Manikandhan R. Cementum analysis in cleidocranial dysostosis. Indian J Dent Res [serial online] 2008 [cited 2014 Nov 28];19:253-6. Available from: http://www.ijdr.in/text.asp?2008/19/3/253/42960
Cleidocranial dysostosis (CCD) is an autosomal dominant skeletal disorder with a wide range of expressivity. It primarily affects the bones undergoing intramembranous ossification. [1]

CCD is a skeletal disorder caused by both dominant and recessive patterns of inheritance with variable expressivity. The disorder was originally thought to involve bones of intramembranous origin only, namely the bones of the skull, clavicles and flat bones, hence the term, "cleidocranial." Later it was known that bones of endochondral ossification are also affected and it is a generalized disorder of many skeletal structures. [2]

It is characterized by clavicular aplasia or hypoplasia, (which results in hypermobility of the shoulders), retarded cranial ossification (open sagittal sutures and patent fontanelles resulting in wormian bones), short stature, a characteristic craniofacial appearance showing parietal and frontal bossing, hypoplastic maxilla and zygomas, relative mandibular prognathism and a variety of other skeletal abnormalities. [1]

It is a disorder that involves a mutation in the transcription factor, Runx2/Cbfa1 (Core Binding Factor A1,a member of the Runt family of transcription factors) located on chromosome 6p21. [3],[4],[5],[6],[7],[8] The CBFA1 gene controls the differentiation of precursor cells into osteoblasts, which is essential for membranous as well as endochondral ossification. [9] Studies on the developing tooth germs of Runx2/Cbfa1-/- mice show severely hypoplastic, misshapen and arrested formation at the Cap stage. [10]

Dental anomalies include prolonged retention of deciduous dentition and failure of eruption of permanent dentition associated with multiple impacted and unerupted supernumerary teeth. [11] Absence of cellular cementum at the root apex is presumed to be one of the factors for failure or delayed eruption. The aim of this study was to analyze the root cementum of supernumerary (S) and permanent teeth (P) of a CCD patient and to compare the findings with those of normal individuals with special emphasis on delayed eruption.


   Materials and Methods Top


Our patient was a 60 year-old male patient affected by cleidocranial dysostosis, who complained of an ill-fitting, upper complete denture and a lower partial removable denture due to the eruption of new teeth at the previous extraction site. Twelve erupted teeth (9S + 3P) extracted from this patient and 12 (5S + 7P) age- and sex-matched teeth of normal individuals were taken for this study.

The extracted teeth were washed well to remove blood stains. Longitudinal ground sections of 50 μm thickness were prepared using a laboratory lathe with a coarse and fine abrasive lathe wheel. The ground sections were polished with fine Arkansas stone, dehydrated and mounted in dibutyl phthate xyelene (DPX). Root characteristics were recorded using an ocular micrometer in a light microscope. The parameters recorded were apical root resorption, type of cementum in the apical third of the root and the type of cemento-enamel junction.


   Results Top


Type of cementum in the apical third [Figure 1] and [Figure 2]

Cellular cementum was seen at the apices of all 12 teeth (100%) of normal individuals whereas it was seen at the apex of only one supernumerary tooth (8%) of the CCD patient while all the other 11 teeth (92%) showed acellular cementum at their apices.

Root resorption at the apex [Figure 3]

None of the 12 teeth of normal individuals showed any apical root resorption. Among the nine supernumerary teeth of the CCD patient, one (11.2%), three (33.3%) and five (55.5%) teeth showed no, total and partial apical root resorption, respectively. Among the three permanent teeth of the CCD patient, two (66.7%) showed total and one (33.3%) showed partial apical root resorption.

Cemento-enamel junction [Figure 4]

For each ground section, two cemento-enamel (C-E) junctions were considered - one on the facial and the other on the lingual/palatal aspect. The incidence of gap junctions, edge-to-edge and cementum-overlapping enamel type junctions in the supernumerary teeth of the CCD patient was 64, 28 and 8% respectively whereas the permanent teeth of the CCD patient showed 100% gap junctions. In normal individuals, the incidence of gap junctions, edge-to-edge and cementum-overlapping enamel type junctions in supernumerary and permanent teeth was 20, 60 and 20%, and 27, 64, 9% respectively.

The average length of the gap junctions in normal permanent teeth was 209.1 μm whereas it was 109.4 μm and 108 μm in supernumerary teeth and permanent teeth respectively, in our CCD patient.


   Discussion Top


Tooth formation and eruption is a complex and highly regulated process that involves the cells of the tooth organ and the surrounding alveolus. The causes of unerupted teeth are attributed to the disturbance of bone resorption, [12] the early loss of the gubernacular cord or canal, [13],[14],[15],[16] lack of cellular cementum [14] or lack of union between the dental follicle and the mucosa due to interposed fibrous tissue acting as a barrier to eruption. [13]

The lack of cellular cementum is considered to be one of the factors for uneruption of teeth in CCD. [17] In the present case, the patient complained of eruption of new teeth at the previous extraction site, in spite of the absence of cellular cementum. Therefore, absence of cellular cementum is considered unlikely to have any influence on the eruption process.

It is also suggested that remnants of the dental lamina are activated to form supernumerary teeth in CCD patients when mineralization of the crowns of the permanent teeth is complete. [18] They are also thought to be evolved by hyperplasia or by the segmentation of tooth germs due to loss of genetic control. [13] The presence of multiple supernumerary teeth may cause mechanical obstruction and may be the chief factor for the impaction of permanent teeth observed in CCD. [14]

Serum alkaline phosphatase activity has been observed to be consistently reduced in patients suffering from CCD. [18] Alkaline phosphatase-deficient animals present delayed tooth eruption and onset of mineralization of the root dentine, [19] thus suggesting that decreased alkaline phosphatase levels could be one of the factors for delayed eruption in CCD.

In this study, we observed apical root resorption in 100% of the permanent teeth and in 88.8% of supernumerary teeth in our CCD patient while teeth of normal individuals failed to show any resorption. It is reported that the bone obtained from the alveolous overlying unerupted teeth in CCD is dense and shows reversal lines suggesting that this represents an abnormal resoption pattern. [20] A slow rate of bone resorption may result in prolonged stress that is concentrated in the root of the tooth, which in turn could trigger a cascade of fatigue-related events leading to root resorption.

Eruption of the tooth starts after the completion of crown formation and activation of Hertwig's epithelial root sheath. This is followed by radicular dentin formation and disintegration of Hertwig's epithelial root sheath allowing cementum deposition on dentine. [16] Failure of disintegration of this root sheath at the appropriate time leads to the formation of the gap type of C-E junction. In the absence of cementum formation at the crucial period of time, the lack of attachment of periodontal fibers may probably lead to delayed eruption. Increased percentage of the gap type of C-E junctions seen in supernumerary and permanent teeth of our CCD patient (64 and 100% respectively) may be considered to be one of the factors responsible for the delayed eruption of teeth in CCD. However, further advanced studies are required to support this contention.


   Conclusion Top


Cellular cementum formation may not be the only essential factor involved in normal tooth eruption. Mechanical obstruction may be the chief factor for the impaction of teeth seen in CCD and our observation of the increased percentage of the gap type of C-E junctions could be an additional factor explaining the delayed eruption in CCD.

 
   References Top

1.Kanda M, Kabe S, Kanki T, Sato J, Hasegawa Y. Cleidocranial dysplasia: A case report. No Shinkei Geka (1997);25:1109-13.  Back to cited text no. 1  [PUBMED]  
2.McNamara CM, Riordan BC, Blake M, Sandy JR. Cleidicranial dysplasia: Radiological appearances on dental panoramic radiolography. Dent Maxillofac Radiol 1999;28:89-97.  Back to cited text no. 2    
3.Nienhaus H, Mau U, Zang KD, Henn W. Pericentric inversion of chromosome 6 in a patient with cleidocranial dysplasia. Am J Med Genet 1993;46:630-1.  Back to cited text no. 3  [PUBMED]  
4.Gelb BD, Cooper E, Shevell M, Desnick RJ. Genetic mapping of the cleidocranial dysplasia (CCD) locus on chromosome band 6p21 to include a microdeletion. Am J Med Genet 1995;58:200-5.  Back to cited text no. 4  [PUBMED]  
5.Feldman GJ, Robin NH, Brueton LA, Robertson E, Thompson EM, Siegel-Bartelt J. A gene for cleidocranial dysplasia maps to the short arm of chromosome 6. Am J Hum Genet 1995;56:938-43.  Back to cited text no. 5    
6.Mundlos S, Mulliken JB, Abramson DL, Warman ML, Knoll JH, Olsen BR. Genetic mapping of cleidocranial dysplasia and evidence of a microdeletion in one family. Hum Mol Genet 1995;4:71-5.  Back to cited text no. 6  [PUBMED]  
7.Mundlos S, Otto F, Mundlos C, Mulliken JB, Aylsworth AS, Albright S , et al . Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia. Cell 1997;89:773-9.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Machuca-Tzili L, Monroy-Jaramillo N, Gonzalez del Angel A, Kofman-Alfaro S. New mutations in the CBFA1 gene in the two Mexican patient with cleidocranial dysplasia. Clin Genet 2002;61:349-53.  Back to cited text no. 8    
9.Mundlos S. Cleidocranial dysplasia: Clinical and molecular genetics. J Med Genet 1999;36:177-82.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.D'Souza RN, Aberg T, Gaikwad J, Cavender A, Owen M, Karsenty G, et al . Cbfa1 is required for epithelial-mesenchymal interaction regulating tooth development in mice. Development 2000;126:2911-20.  Back to cited text no. 10    
11.Shaikh R, Shusterman S. Delayed dental maturation in cleidocranial dysplasia. ASDC J Dent Child 1998;65:325-9.  Back to cited text no. 11  [PUBMED]  
12.Migliorisi JA, Blenkinsopp PT. Oral surgical management of cleidocranial dysostosis. Br J Oral Surg 1980;18:212-20.  Back to cited text no. 12  [PUBMED]  
13.Stewart RE, Prescott GH, editors. Oral facial genetics. St Louis; The CV Mosby Co; 1976. p. 282-4,566-7.  Back to cited text no. 13    
14.Yamamoto H, Sakae T, Davies JE. Cleidocranial dysplasia: A light microscope, electron microscope, and crystallographic study. Oral Surg Oral Med Oral Pathol 1989;68:195-200.  Back to cited text no. 14  [PUBMED]  
15.Tanaka JL, Ono E, Filho EM, Castilho JC, Moraes LC, Moraes ME. Cleidocranial dysplasia: Importance of radiographic images in diagnosis of the condition. J Oral Sci 2006;48:161-6.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Tencate RA. Oral histology. 6 th ed. St louis: The CV Mosby Company; 2003. p. 278, 107, 202-205, 250.  Back to cited text no. 16    
17.Counts AL, Rohrer MD, Prasad H, Bolen P. An assessment of root cementum in cleidocranial dysplasia. Angle Orthodont 2001;71:293-8.  Back to cited text no. 17    
18.Unger S, Mornet E, Mundlos S, Blaser S, Cole DE. Severe cleidocranial dysplasia can mimic hypophosphatasia. Eur J Pediatr 2002;161:623-6.  Back to cited text no. 18    
19.Zeichner-David M, Oishi K, Su Z, Zakartchenko V, Chen LS, Arzate H, et al . Role of Hertwig's epithelial root sheath cells in tooth root development. Dev Dyn 2003;228:651-63.  Back to cited text no. 19    
20.Hitchin AD, Faily JM. Dental management in cleidocranial dysostosis. Br J Oral Surg 1974;12:46-55.  Back to cited text no. 20    

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Correspondence Address:
T R Saraswathi
Department of Oral and Maxillo-Facial Pathology, Meenakshi Ammal Dental College and Hospitals, Chennai - 600 095
India
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DOI: 10.4103/0970-9290.42960

PMID: 18797104

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    Figures

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