American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: Editor-in-chief: Samy, I. McFarlane
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American Journal of Medical Case Reports. 2018, 6(10), 193-201
DOI: 10.12691/ajmcr-6-10-1
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A Dento – Surgical Management of a Cleidocranial Dysplasia Case. A New Approach for Better Oral Health Quality of Life Part II to: (A 9-year-old Saudi Boy with Cliedocranial Dysplasia)

Rabab A. Al Buainain1, Tarek Ezzeldin2, Hoda Abougareeb3, Faisal Al Sineedi4, Basma A. Almaghlouth5, Yousef H. Almoumen6 and Abdullah Faraj Alshammari7,

1Senior Resident in Saudi Board Pediatric Dentistry Program, Dammam Medical Complex, Ministry of Health, Kingdom of Saudi Arabia

2Consultant in Pediatric Dentistry, (French Board Certified) Dammam Medical Complex, Ministry of Health, Kingdom of Saudi Arabia

3Consultant in Pediatric Dentistry, Dammam Medical Complex, Ministry of Health, Kingdom of Saudi Arabia

4Consultant in Pediatric Dentistry, King Fahad Military Medical Complex, Ministry of Defense, Kingdom of Saudi Arabia

5SBO Consultant in Orthodontics, Dammam Medical Complex, Ministry of Health, Kingdom of Saudi Arabia

6Consultant in Oral & Maxillo Facial Surgery, BDS, SBOMFS, AAAM (American Academy of Aesthetic Medicine Board Certified)

7College of Dentistry, University of Hail, Hail, KSA

Pub. Date: October 23, 2018

Cite this paper:
Rabab A. Al Buainain, Tarek Ezzeldin, Hoda Abougareeb, Faisal Al Sineedi, Basma A. Almaghlouth, Yousef H. Almoumen and Abdullah Faraj Alshammari. A Dento – Surgical Management of a Cleidocranial Dysplasia Case. A New Approach for Better Oral Health Quality of Life Part II to: (A 9-year-old Saudi Boy with Cliedocranial Dysplasia). American Journal of Medical Case Reports. 2018; 6(10):193-201. doi: 10.12691/ajmcr-6-10-1


Background: Cleidocranial dysplasia is a rare congenital defect of autosomal dominant heritable disease with prevalence of 1 per 1,000000 individuals. It is primarily affects bones which undergo intramembranous ossification [1,2,3,4]. Case presentation: A 9 years old Saudi male with CCD attended Pediatric Dental Clinics in Dammam Medical Complex. Chief complaint was delay eruption of permanent teeth and multiple caries. Based on clinical examination, radiographic investigations including CBCT & with coordination between Pediatric Dentistry, maxillofacial surgery and Orthodontic departments, a treatment plan has been concluded taking into consideration the wellbeing and the oral health related life style of the patient by addressing a new approach to preserve all the primary teeth, surgically removing all supernumerary teeth, applying removal or fixed modified Gropers’ appliance to improve speech, esthetic & function than observing the eruption of the permanent teeth to intervene orthodontically only when needed. Conclusion: Team work involving multi-disciplinary departments helped to select a preeminent & new dental management approach for CCD patients including all aspects needed, Dental rehabilitation, Surgical & Orthodontic treatment to provide better oral health quality of life for the young patient.

cliedocranial dysplasia dammam approach oral health quality of life dento-surgical management

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[1]  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(3):161-166.
[2]  Brueton LA, Reeve A, Ellis R, Husband P, Thomson EM, Kingston HM: Apparent cleidocranial dysplasia associated with abnormalities of 8q22 in three individuals. Am J Med Genet 1992, 43(3):612-618.
[3]  Dard M: Histology of alveolar bone and primary tooth roots in a case of cleidocranial dysplasia. Bull Group Int Rech Sci Stomatol Odontol 1993, 36(3-4): 101-107.
[4]  Mohan, R.P., Suma, G.N., Vashishth, S. and Goel, S. (2010) Cleidocranial dysplasia: Clinico-radiological il- lustration of a rare case. Journal of Oral Science, 52, 161-166.
[5]  Marie P, Sainton P: Sur la dysostose cleido-cranienne hereditaire. Rev Neurol 1898, 6:835-838.
[6]  Mundlos S. Cleidocranial dysplasia: clinical and molecular genetics. J Med Genet 1999; 36: 177-182.
[7]  T. P. Croll and R. R. Cavanaugh, “Enamel color modi cation by controlled hydrochloric acid-pumice abrasion. I. technique and examples,” Quintessence International, vol. 17, no. 2, pp. 81-87, 1986.
[8]  Richardson A and Deussen FF: Facial and dental anomalies in cleidocranial dysplasia: A study of 17 cases. Int J Paediatr Dent 4: 225‐231, 1994.
[9]  Cooper SC, Flaitz CM, Johnston DA, Lee B and Hecht JT: A natural history of cleidocranial dysplasia. Am J Med Genet 104: 1-6, 2001.
[10]  Lee B, Thirunavukkarasu K, Zhou L, Pastore L, Baldini A, Hecht J, Geoffroy V, Ducy P and Karsenty G: Missense mutations abolishing DNA binding of the osteoblast‐speci c transcription factor OSF2/CBFA1 in cleidocranial dysplasia. Nat Genet 16: 307‐310, 1997.
[11]  Mundlos S, Otto F, Mundlos C, Mulliken JB, Aylsworth AS, Albright S, Lindhout D, Cole WG, Henn W, Knoll JH, et al: Mutations involving the transcription factor CBFA1 cause cleido- cranial dysplasia. Cell 89: 773‐779, 1997.
[12]  Otto F, Thornell AP, Crompton T, Denzel A, Gilmour KC, Rosewell IR, Stamp GW, Beddington RS, Mundlos S, Olsen BR, et al: Cbfa1, a candidate gene for cleidocranial dysplasia syndrome, is essential for osteoblast differentiation and bone devel- opment. Cell 89: 765‐771, 1997.
[13]  Komori T, Yagi H, Nomura S, Yamaguchi A, Sasaki K, Deguchi K, Shimizu Y, Bronson RT, Gao YH, Inada M, et al: Targeted disruption of Cbfa1 results in a complete lack of bone formation owing to maturational arrest of osteoblasts. Cell 89: 755‐764, 1997.
[14]  Smylski, P.T., Woodside, D.G. and Harnett, B.E. (1974) Surgical and orthodontic treatment of cleidocranial dysostosis. International Journal of Oral Surgery, 3, 380-385.
[15]  Becker A, Lustman J, Shteyer A. Cleidocranial dysplasia: part 1-General principles of the orthodontic and surgical treatment modality. Am J Orthod Dentofacial Orthop 1997; 111: 28-33.
[16]  Becker A, Shteyer A, Bimstein E, Lustmann J. Cleidocranial dysplasia: part 2-treatment protocol for the orthodontic and surgical modality. Am J Orthod Dentofacial Orthop 1997; 111: 178-83.
[17]  Kelly, E. and Nakamoto, R.Y. (). Cleidocranial dy-sostosis-A prosthodontic problem. Journal of Prosthetic Dentistry, 1974, 31, 518-526.
[18]  Behlfelt K. Cleidocranial dysplasia: diagnosis and treatment con-cept. Trans Eur Orthod Soc 1987; 63: 25.
[19]  Berg RW, Kurtz KS, Watanabe I, Lambrakos I. Interim prosthetic phase of multidisciplinary managementof cleidocranial dysplasia: “the Bronx approach.” J Prosthodont 2011; 20: S20-5.
[20]  Kalliala E, Taskinen PJ: Cleidocranial dysostosis: report of six typical cases and one atypical case. Oral Surg Oral Med Oral Pathol 1962, 14:808-822.
[21]  Ahmet Ercan Sekerci, Burhan Balta, Oğuzhan Bahadir, Yildiray Sisman, Munis Dundar, Turgut Tursem Tokmak, Stefan Mundlos Cleidocranial dysplasia with a rare mutation: Study of a family with review of literature. Journal of Stomatology, 2013, 3, 402-410.
[22]  Tina Roberts, BChD, MChD, Lawrence Stephen, BChD, MSc, PhD, and Peter Beighton, MD, PhD, FRCP, Cape Town, South Africa. Cleidocranial dysplasia: a review of the dental, historical, and practical implications with an overview of the South African experience. Oral Surg Oral Med Oral Pathol Oral Radiol 2013; 115: 46-55.
[23]  Sischo L, Broder HL. Oral Health-related Quality of Life: What, Why, How, and Future Implications .J Dent Res. 2011 Nov; 90(11): 1264-70.
[24]  American Association of Pediatric Dentistry (Policies & Guidelines). Policy on the role of Pediatric dentists as both primary and Specialty Care Providers.V39/NO 6 17/18.