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Crouzon Syndrome a Comprehensive Review

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Chrystalla Kyprianou1, Athina Chatzigianni2

1 Dental practice, Thessaloniki, Greece
2 School of Dentistry, Aristotle University of Thessaloniki, Thessaloniki, Greece


Crouzon syndrome is a rare genetic disorder with autosomal dominant inheritance. The underlying pathological process is premature synostosis of the cranial sutures with subsequent phenotypic alterations of the affected person. A review of the literature has been conducted in order to resume the overall characteristics of Crouzon syndrome such as craniomaxillofacial malformations, clinical features, dentoalveolar characteristics, aesthetic impairments, and psychological background, as well as, the different therapeutic procedures, which combine surgical and orthodontic interventions. Facial and functional malformations in individuals with Crouzon syndrome could be significantly improved after a series of surgical and orthodontic procedures in almost all cases. A multidisciplinary treatment approach would provide the best outcomes in affected patients.

Keywords: Crouzon; Crouzon Syndrome; Diagnosis; Multidisciplinary Approach; Orthodontic Management; Surgical Management



1. Kreiborg S. Crouzon syndrome. Scand J Plast Reconstr Surg, 1981;18:1-198.Google Scholar


2. Reardon W, Winter RM, Rutland P, Pulleyn LJ, Jones BM, Malcolm S. Mutations in the fibroblast growth receptor 2 gene cause Crouzon syndrome. Nat Genet, 1994;8:98-103.Google Scholar


3. Schulz C, Kress W, Schömig A, Wessley R. Endocardial cushion defect in a patient with Crouzon syndrome carrying a mutation in the fibroblast growth factor receptor (FGRF-2) gene. Clin Genet, 2005;72:305-307.Google Scholar


4. Chang CP, Wan L, Tsai CH, Lee CC, Tsai FJ. Mutation analysis of Crouzon’s syndrome In Taiwanese patients. J Clin Lab Anal, 2006;20:23-26.Google Scholar


5. Eswarakumar VP, Horowitz MC, Locklin R, Morriss- Kay GM, Lonai P. A gain-of function mutation of FGFR2c demonstrates the roles of this receptor variant in osteogenesis. Proc Natl Acad Sci USA, 2004;101:12555-12560.Google Scholar


6. Agochukwu NB, Solomon BD, Muenke M. Impact of genetics on the diagnosis and clinical management of syndromic craniosynostoses. Childs Nerv Syst, 2012;28:1447-1463.Google Scholar


7. Johnson D, Wilkie A O M. Craniosynostosis. Eur J Hum Genet, 2011;19:369-376.CrossrefGoogle Scholar


8. Ciurea AV, Toader C. Genetics of craniosynostosis: review of the literature. J Med Life, 2009;2:5-17.Google Scholar


9. WarrenSM, FongKD, ChenCM, LoboaEG, CowanCM, LorenzHP et al. The BMP antagonist noggin regulates cranial suture fusion. Nature, 2003;422:625-629.Google Scholar


10. Vagervik K, Rubin MS, Grayson BH, Figueroa AA, Kreiborg S, Shirley JC et al. Parameters of care for craniosynostosis: dental and orthodontic perspectives. Am J Orthod Dentofacial Orthop, 2012;141:S68-S73.Google Scholar


11. Cunningham ML, Seto ML, Rotisoontorn C, Heike L, Hing AV. Syndromic craniosynostosis: From history to hydrogen bonds. Orthod Craniofac Res, 2007;10:67-81.Google Scholar


12. Di Rocco F, Arnaud E, Renier D. Evolution in the frequency of nonsyndromic craniosynostosis. J Neurosurg Pediatr, 2009;4:21-25.Google Scholar


13. Tsuji M. Quantitative analysis of characteristic variations of the facial features of Crouzon syndrome. Kokubyop Gakkai Zasshi, 2006;73:125-138.Google Scholar


14. Forrest CR, Hopper RA. Craniofacial syndromes and surgery. Plast Reconstr Surg, 2013;131:86e-109e.Google Scholar


15. Kabbani H, Raghuveer TS. Craniosynostosis. Am Fam Physician, 2004;69:2863-2870.Google Scholar


16. Panchal J, Uttchin V. Management of craniosynostosis. Plast Reconstr Surg, 2003;111:2032-2048.Google Scholar


17. Kreiborg S, Cohen MM Jr. Is craniofacial morphology in Apert and Crouzon syndromes the same? Acta Odontol Scand, 1998;56:339-341.Google Scholar


18. Reitsma Jh, Ongkosuwito Em, Buschang Ph, Prahlandersen B. Facial growth in patients with apert and crouzon syndromes compared to normal children. Cleft Palate Craniofac J, 2012;49:185-193.Google Scholar


19. Reitsma J H, Balk-Leurs I H, Ongkosuwito E M, Wattel E, Prahl-Andersen B. Dental maturation in children with the syndrome of crouzon and apert. Cleft Palate Craniofac J, 2013;51:639-644.Google Scholar


20. Reitsma J H, Elmi P, Ongkosuwito E M, Buschang P H, Prahl-Andersen B. A longitudinal study of dental arch morphology in children with the syndrome of Crouzon or Apert. Eur J Oral Sci, 2013;121:319-332.Google Scholar


21. Bjerklin K, Kurol J. Ectopic eruption of the maxillary first permanent molar: etiologic factors. Am J Orth, 1983;84:147-155.Google Scholar


22. Stavropoulos D, Bartzela T, Tarnow P, Mohlin B, Kahnberg K, Hagberg C. Dental agenesis patterns in Crouzon syndrome. Swed Dent J, 2011;35:195-201.Google Scholar


23. Stavropoulos D, Bartzela T, Bronkhorst E, Mohlin b, Hagberg C. Dental agenesis patterns of permanent teeth in Apert syndrome. Eur J Oral Sci, 2011;119:198-203.Google Scholar


24. Bowling EL, Burstein FD. Crouzon syndrome. Optometry, 2006;77:217-222.Google Scholar


25. Gray TL, Casey T, Selva D, Anderson PJ, David DJ. Ophthalmic sequelae of Crouzon syndrome. Ophthalmology, 2005;112:1129-1134.Google Scholar


26. Akram A, McKnight MM, Bellardie H, Beale V, Evans RD. Craniofacial malformations and the orthodontist. Br Dent J, 2015;218:129-141.Google Scholar


27. Fearon JA. Halo distraction of the Le Fort III in syndromic craniosynostosis: A long-term assessment. Plast Reconstr Surg, 2005;115:1524.Google Scholar


28. Farole A, Mundenar MJ, Braitman LE. Posterior airway changes associated with mandibular advancement surgery: implications for patients with obstructive sleep apnea. Int J Adult Orthodon Orthognath Surg, 1990;5:255-258.Google Scholar


29. Cohen MM. Craniosynostosis and syndromes with craniosynostosis: incidence, genetics, penetrance, variability and new syndrome updating. Birth Defects Orig Artic Ser, 1979;15:13-63.Google Scholar


30. Gorlin RJ, Hennekam RCM. Syndromes with craniosynostosis: general aspects and well-known syndromes. In: Gorlin RJ, Cohen MM, Hennekam RCM, eds. Syndromes of the Head and Neck. Oxford, UK: Oxford University Press, Inc.; 2001.Google Scholar


31. Nur BG, Pehlivanoğlu S, Mıhçı E, Calışkan M, Demir D, Alper OM et al. Clinicogenetic study of Turkish patients with syndromic craniosynostosis and literature review. Pediatr Neurol, 2014;50:482-490.Google Scholar


32. Micheletti-Helfer T, Borges-Peixoto A, Tonni G, Araujo JE. Craniosynostosis: prenatal diagnosis by 2D/3D ultrasound, magnetic resonance imaging and computed tomography. Med Ultrason, 2016;18:378-385.CrossrefGoogle Scholar


33. Tonni G, Panteghini M, Rossi A, Baldi M, Magnani C, Ferrari B et al. Craniosynostosis: prenatal diagnosis by means of ultrasound and SSSE-MRI. Family series with report of neurodevelopmental outcome and review of the literature. Arch Gynecol Obstet, 2011;283:909-916.Google Scholar


34. Faro C, Benoit B, Wegrzyn P, Chaoui R, Nicolaides KH. Three-dimensional sonographic description of the fetal frontal bones and metopic suture. Ultrasound Obstet Gynecol, 2005;26:618-621.Google Scholar


35. Nørgaard P, Hagen CP, Hove H, Dunoe M, Nissen KR, Kreiborg S et al. Crouzon syndrome associated with acanthosis nigricans: prenatal 2D and 3D ultrasound findings and postnatal 3D CT findings. Acta Radiol short reports, 2012;1:1-5.Google Scholar


36. Fjørtoft MI, Sevely A, Boetto S, Kessler S, Sarramon MF, Rolland M. Prenatal diagnosis of craniosynostosis: value of MR imaging. Neuroradiology, 2007;49:515-521.Google Scholar


37. Kimonis V, Gold JA, Hoffman TL, Panchal J, Boyadjiev SA. Genetics of craniosynostosis. Semin Pediatr Neurol, 2007;14:150-161.Google Scholar


38. Prenatal Imaging of Craniosynostosis Syndromes. Ketwaroo PD, Robson CD, Estroff JA. Semin Ultrasound CT MR, 2015;36:453-464.Google Scholar


39. Carinci F, Avantaggiato A, Curioni C. Crouzon syndrome: Cephalometric analysis and evaluation of pathogenesis. Cleft Palate Craniofac J, 1994;31:201-209.Google Scholar


40. David LR, Velotta E, Weaver RG, Wilson JA, Argenta LC. Clinical findings precede objective diagnostic testing in the identification of increased intracranial pressure in syndromic craniosynostosis. J Craniofac Surgery, 2002;13:67680.Google Scholar


41. Hayward R, Jones B, Dunaway D, Evans R (eds). The clinical management of craniosynostosis. Clinics in Developmental Medicine No. 163. London: Mac Keith Press, 2004.Google Scholar


42. Evans R D. Highly specialised services. J Orthod, 2013:40:181-182.Google Scholar


43. Kahnberg KE, Hagberg C. Orthognathic surgery in patients with craniofacial syndrome. I. A 5-year overview of combined orthodontic and surgical correction. J Plast Surg Hand Surg, 2010;44:282-288.Google Scholar


44. Pagnoni M, Fadda MT, Spalice A et al. Surgical timing of craniosynostosis what to do and when. J Cranio-Maxillo- Facial Surg, 2014;42:513-519.CrossrefGoogle Scholar


45. Renier D, Sainte-Rose C, Marchac D, Hirsch JF. Intracranial pressure in craniostenosis. J Neurosurg, 1982;57:370-377.CrossrefGoogle Scholar


46. Dobbs TD, Wall SA, Richards PG, Johnson D. A novel technique to secure the rigid external distraction (RED) frame in a thin skull allowing sutural mid-face distraction. J CranioMaxillofacial Surg, 2014;42:1048-1051.Google Scholar


47. Gosain AK, Santoro TD, Havlik RJ, Cohen SR, Holmes RE. Midface distraction following Le Fort III and monobloc osteotomies: Problems and solutions. Plast Reconstr Surg, 2002;109:1797-1808.Google Scholar


48. Sakamoto Y, Nakajima H, Tamada I, Sakamoto T. Le Fort IV+I distraction osteogenesis using an internal device for syndromic craniosynostosis. J Oral Maxillofac Surg, 2014;72:788-795.Google Scholar


49. Meling TR, Hogevold HE, Due-Tonnessen BJ, Skjelbred P. Comparison of perioperative morbidity after LeFort III and monobloc distraction osteogenesis. Br J Oral Maxillofac Surg, 2011;49:131-134.Google Scholar


50. Fearon JA, Whitaker LA. Complications with facial advancement: a comparison between the Le Fort III and monobloc advancements. Plast Reconstr Surg, 1993;91:990-995.CrossrefGoogle Scholar


51. McCarthy JG, Warren SM, Bernstein J, Burnett W, Cunningham ML, Edmond JC et al. Craniosynostosis Working Group. Parameters of care for craniosynostosis. Cleft Palate Craniofac J, 2012;49:1S-24S.Google Scholar


52. Lee DW, Ham KW, Kwon SM, Lew DH, Cho EJ. Dual midfacial distraction osteogenesis for Crouzon syndrome: long-term follow-up study for relapse and growth. J Oral Maxillofac Surg, 2012;70:e242-e251.Google Scholar


53. Mtihlbauer W, Anderl H, Heeckt P, Schmidt A, Zenker J, Hopner F et al. Early Operation in Craniofacial Dysostosis. World J Surg, 1989;13:366-372.Google Scholar


54. Allam KA, Wan DC, Khwanngern K, Kawamoto HK, Tanna N, Perry A et al. Treatment of Apertsyndrome: a long-term follow-up study. Plast Reconstr Surg, 2011;127:1601-1611.Google Scholar


55. Helman SN, Badhey A, Kadakia S, Myers E. Revisiting Crouzon syndrome: reviewing the background and management of a multifaceted disease. Oral Maxillofac Surg, 2014;18:373-379.Google Scholar


56. Nurko C, Quinones R. Dental and orthodontic management of patients with Apert and Crouzon syndromes. Oral Maxillofac Surg Clin North Am, 2004;16:541-553.Google Scholar


57. Kreiborg S. Postnatal growth and development of the craniofacial complex in premature craniosynostosis. In: Cohen MM Jr, MacLean RE, editors. Craniosynostosis: diagnosis, evaluation, and management. 2nd ed. New York: Oxford University Press; 2000. p. 158-174.Google Scholar


58. Kreiborg S, Aduss H, Cohen MM Jr. Cephalometric study of the Apert syndrome in adolescence and adulthood. J Craniofac Genet Dev Biol, 1999;19:1-11.Google Scholar


59. Welch TB. Stability in the correction of dentofacial deformities:a comprehensive review. J OraL Maxillofac Surg, 1989;47:1142-1149.Google Scholar


60. Múfalo PS, Kaizer R de OR, Dalben G de S, de Almeida ALPF. Comparison of periodontal parameters in individuals with syndromic craniosynostosis. J Appl Oral Sci, 2009;17:13-20.CrossrefGoogle Scholar


61. Pawlicki R, Knychalska-Karwan Z, Darczuk D, Nowak T. Crouzon’s syndrome: tooth morphological and microanalytical evaluation. Eur Arch Paediatr Dent, 2008;9:232-235.CrossrefGoogle Scholar


62. Dalben Gda S, Costa B, Gomide MR. Oral health status of children with syndromic craniosynostosis. Oral Health Prev Dent, 2006;4:173-179.Google Scholar

Balkan Journal of Dental Medicine, Volume 22, Issue 1, Pages 1–6, ISSN (Online) 2335-0245,DOI:

Welcome message

Welcome message


Dear colleagues,

For the last twenty years, the Balkan Journal of Stomatology (now the Balkan Journal of Dental Medicine) has succeeded in spreading and encouraging scientific thinking among dentists in the Balkans. The Balkan Stomatological Society lies on strong foundations built with the professional cooperation and communication among peers, while the Balkan Journal of Dental Medicine in its written form bears testimony to the persistence, development and evolution of the scientific thinking in this region.

The aim of The Balkan Journal of Dental Medicine is to continue the tradition of the Balkan Journal of Stomatology by promoting dentists’ efforts aimed at professional development and research throughout the region. In addition, the Journal seeks to introduce the world’s latest findings and achievements in dentistry to the Balkan region.  So as to accomplish our mission, I am sure we will enjoy a great support from numerous eminent lecturers from the BaSS Congress and the assistance of the distinguished Editorial Board members, whose reputation and work guarantee high quality and respect.

It is our ongoing mission to advance dental medicine and promote science by publishing high-quality papers relevant to everyday dental practice. The previous XIX volumes, numerous authors who have published their work in them and the bright future we strive for compel us to regard the Evidence-based Dentistry as both our goal and responsibility.

To move with the times, we have joined the large community of open access journals, upgraded the design and prepared an online edition. Thus we have provided scientists and professionals with an easy access to scientific papers, as well as with the opportunity to contribute to creating the new future of the Journal.

We are going to pay a special attention to young researchers, since we are eager to support their efforts in making the-first steps in the world of scientific publications so that they can become the great future of dentistry.

Science has no borders and it spreads across nations with an intention to broaden one’s horizons, assisting one’s need for success and arousing one’s curiosity. With its positive energy, scientific thinking occupies human mind purging it from everyday worries and leading it towards the light. This is why it is essential for ideas and achievements to be written down in order that they can become one’s incentive and challenge while being a part of the collective memory rather than a mere recollection or some unfulfilled plans of an individual.


  24.03.2016.                                                  Prof. Dr Dejan Markovic, DDS, MSc, PhD,     Belgrade                                         Editor in Chief of  Balkan Journal of Dental Medicine

Welcome Message


Dear distinguished colleagues, dear ladies and gentlemen,

It is my honor and privilege to be at your service as the president of the Balkan Stomatological Society following in the footsteps of many inspirational leaders of this Society.

Twenty years ago, a group of enthusiasts founded the Balkan Stomatological Society at the 1st Balkan Dental Congress in Thessaloniki, Greece, in 1996. The main idea of establishing such an association in the Balkans was to bring general practitioners and specialists in different fields of dental medicine closer together and enable the exchange of personal experience, scientific data and information concerning contemporary preservation of oral health, diagnosing and dental treatment.

I have witnessed the Society evolve over the last decade to become a vibrant and ever growing association, embracing change yet keeping core values, above all, those of friendship and cooperation. These were the initial reasons that attracted me to the Society and subsequently to become more involved.

With the Society’s ultimate aim of promoting high quality dental care, in recent years we have broadened the scope of BaSS activities, especially during the Congresses (lectures, hands-on training, exhibitions) and a modernized, on-line with open access, Balkan Journal of Dental Medicine. None of this could have taken place without the dedication and commitment of the Council and its members, whose exceptional work will surely be continued.

I’m very happy and proud to be a part of the BaSS, which lies on strong foundations built with sincere enthusiasm and efforts of my predecessors. The Society is now strong, sustainable and sure to achieve new horizons.

However, without the support of its membership, there cannot be true progress. We would therefore like to hear from you, and for this, all lines of communication are open. So, please visit our website as often as you can. We would also encourage you to volunteer to be part of any of the committees and working groups, and will continue to update you on all of our activities through our BJDM as well as the website.

The BaSS welcomes to its membership all who are engaged in the dental profession and are concerned with the latest achievements in dentistry. We are open to all suggestions on improvement, and of course welcome topics related to annual congresses.

With kindest regards, I wish you a lot of success in all your endeavors!

24.03.2016.                                    Professor Dragoslav Stamenković, DDM, MSc, PhD Belgrade                                           President of the BaSS