Introduction: Cleidocranial dysplasia (CCD) is an autosomal dominant disorder, also referred to ascleidocranial dysostosis. The diagnosis is made on the basis of clinical and radiological findings and is confirmed through a genetic analysis. Cleidocranial dysplasia manifestations in the skeletal system include aplasia or hypoplasia of the clavicles, leading to shoulder joint hypermobility, a cone-shaped chest with short ribs, a wide skull with prominent frontal bone, etc. The dental status of CCD patients is commonly marked by a persistent primary dentition, delayed eruption of permanent teeth, supernumerary teeth, an incorrect proportion between the two jaws, impaired masticatory function and aesthetics.
Aim: The aim of the present study is to show the clinical and radiologic findings of two CCD patients and describe the multidisciplinary approach necessary for their treatment. Early diagnosis and awareness of the condition help to determine the optimal treatment and duration as well as to improve patients’ quality of life.
Case Report: The subject of this clinical study are two closely related patients who sought professional dental help due to persistent primary teeth, delayed eruption of permanent dentition beyond the mean eruption age and resultant occlusal and aesthetics problems.
Results: Correction of the dental status in CCD patients is a long process. Timely combination of orthodontic and surgical treatments is crucial for optimal and long-lasting results.
Conclusion: Dental treatment of CCD patients is a complex endeavor. The type of approach in the orthodontic, surgical and prosthetic treatment is largely determined by the patients’ age, their general health as well as their intraoral status (present deciduous teeth, existing permanent teeth and occlusal contacts).
Carvalho CCS, Heimlich FV, Freire NA, Ramos MEB, Rosemiro MM, Israel M. Cleidocranial dysplasia: 2 case reports. Oral Surg oral Med oral Pathol Oral Radiol. 2020;129:e-41. doi: 10.1016/j.oooo.2019.06.131.
Jensen B. Somatic development in cleidocranial dysostosis. Am J Med Genet. 1990;35(1):69-74. doi: 10.1002/ajmg.1320350113.
Butterworth C. Cleidocranial dysplasia: modern concepts of treatment and report of an orthodontic resistant case requiring a restorative solution. Dent Update. 1999;26(10):458-62. doi: 10.12968/denu.1999.26.10.458.
Cooper SC, Flaitz CM, Johnston DA, Lee B, Hecht JT. A natural history of cleidocranial dysplasia. Am J Med Genet. 2001;104(1):1-6. doi: 10.1002/ajmg.10024.
D’Alessandro G, Tagariello T, Piana G. Craniofacial changes and treatment of the stomatognathic system in subjects with cleidocranial dysplasia. Eur J Paediatr Dent. 2010;11(1):39-43.
Jensen B.L., Kreiborg S. Dental treatment strategies in cleidocranial dysplasia. Br Dent J. 1992;172(6):243-7. doi: 10.1038/sj.bdj.4807836.
Haese K, Le Toux G. Surgical strategy for patient with late-diagnosed form of cleidocranial dysplasia: three cases. J Oral Med Oral Surg, 2020;26:4. doi: 10.1051/mbcb/2019033.
Mabrouk Y, Ammar S, Labidi A, Mansour L, Ghoul S. Dental prosthetic treatment in cleidocranial dysplasia: case report and literature review. Case Rep Dent. 2020;2020:8910798. doi: 10.1155/2020/8910798.
Jensen BL, Kreiborg S. Development of the dentition in cleidocranial dysplasia. J Oral Pathol Med. 1990;19(2):89-93. doi: 10.1111/j.1600-0714.1990.tb00803.x.
Leite PB, Carvalho CCS, Heimlich FV, Freire NA, Ramos MEB, Israel MS. The role of dentistry in the diagnosis of cleidocranial dysplasia: reports of two cases. J Oral Diag. 2020;05:e20200019. doi: 10.5935/2525-5711.20200019.
Roberts T, Stephen L, Beighton P. Cleidocranial dysplasia: a review of the dental, histological, and practical implications with an overview of the South African experience. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;115(1):46-55. doi: 10.1016/j.oooo.2012.07.435.
Arun SP, Simon SS, Karthik AK, Chacko RK, Salitha S. A review of clinical and radiological features of cleidocranial dysplasia with a report of two cases and dental treatment protocol. J Pharm Bioall Sci. 2015;7(Supp 2):428-32. doi: 10.4103/0975-7406.163490.
Gupta S, Gupta S, Singh K, Arora P, Goel S. Cleidocranial dysplasia: spectrum of clinical and radiological findings in seven cases. J Oral Maxillofac Radiol. 2015;3(2)58-62. doi:10.4103/2321-3841.157525.
Vij R, Batra P, Vij H. Cleidocranial dysplasia: complete clinical, radiological and histological profiles. BMJ Case Rep. 2013;2013:bcr2013009015. doi: 10.1136/bcr-2013-009015.
Lukimnaa PL, Jensen BL, Thesleff I, Andreasen JO, Kreisborg S. Histological observations of teeth and peridental tissues in cleidocranial dysplasia imply increased activity of odontogenic epithelium and abnormal bone remodeling. J Craniofac Genet Dev Biol. 1995;15(4):212-21.
Daskalogiannakis J, Piedale L, Lindholm TC, Sẚndor GKB, Carmichael RP. Cleidocranial dysplasia: Two generations of management. J Can Dent Assoc. 2006; 72(4):337-42.
Ayub NFAM, Hamzah SH, Hussein AS, Rajali A, Ahmad MS. A case report of cleidocranial dysplasia: A noninvasive approach. Spec Care Dentist. 2021;41(1):111-7. doi: 10.1111/scd.12532.
Petropoulos VC, Balshi TJ, Blshi SF , Wolfinger GT. Treatment of patient with cleidocranial dysplasia using ossteointegrated implants: a patient report. Int J Oral Maxillofac Implants. 2004;19(2):282-7.
Schnutenhaus S, Gӧtz W, Luthardt RG. Prosthetic rehabilitation of a patient with cleidocranial dysplasia using dental implants – a clinical report. Int J Implant Dent. 2021;7(1):5. doi: 10.1186/s40729-020-00287-7
Schnutenhaus S, Luthardt RG, Rudolph H, Gӧtz W. Histological examination and clinical evaluation of the jawbone of on adult patient with cleidocranial dysplasia: a case report. Int J Clin Pathol 2015;8(7):8521-31.
Angle AD, Rebellato J. Dental team management for a patient with cleidocranial dysostosis. Am J Orthod Dentofacial Orthop. 2005;128(1):110-7. doi: 10.1016/j.ajodo.2004.05.019.
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(1):28-33. doi: 10.1016/s0889-5406(97)70298-1.
Becker A, Sheteyer A, Bimstein E, Lustman J. Cleidocranial dysplasia: Part 2 - Treatment protocol for the orthodontic and surgical modality. Am J Orthod Dentofacial Orthop 1997;111(1):173-83. doi: 10.1016/s0889-5406(97)70213-0.
Berg RW, Kurtz KS, Watanabe I, Lambrakos A. Interim prosthetic phase of multidisciplinary management of cleidocranial dysplasia: ,,the Bronx Approach’’. J Proshtodont 2011;20:S20-5. doi: 10.1111/j.1532-849X.2011.00786.x.
Chang H, Wei J, Wang Y, Jia J, Gao X, Li X, Feng H. Restorative treatment strategies for patient with cleidocranial dysplasia. Acta Odontologica Scandinavica 2015;73(6):447-53. doi: 10.3109/00016357.2014.983541.
Email this article (Login required) 
