Crouzon’s Syndrome: Review of the literature and case report
Crouzon syndrome craniosynostosis proptosis facial asymetry
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Crouzon’s Syndrome: Congenital craniosynostosis characterised by the premature in-utero closure of the coronal, sagittal, and lambdoid sutures. It produces an abnormal growth of the cranium, hypoplasia of the jaw, and facial asimmetry. The case of a five year old girl with Crouzon Syndrome is presented; she consulted the service of Paediatric Dentistry at School of Dentistry of Universidad del Valle (Cali,Colombia).
This work highlights the multidisciplinary approach and the orthopaedic pre-surgical management performed, it involved an intraoral fixed device for controlled expansion with a Hyrax type screw and a facial mask for maxillary protraction as treatment before surgery. The surgery was performed in mono-block and advancement with an osteogenic distactor was done.
Key words: Crouzon syndrome, craniosynostosis, proptosis, facial Asymmetry
Díaz, P.-A., & Hernández, J.-A. (2016). Crouzon’s Syndrome: Review of the literature and case report. Revista Estomatología, 24(2). https://doi.org/10.25100/re.v24i2.5799
1. Ponce de León FC. Craneoestenosis. II. Análisis de las craneoestenosis sindromáticas y diferentes tipos de tratamiento. Bol Med Hosp Infant Mex. 2011; 68(6):409-18.
2. Dicus BC, Golden BA, Turvey TA. Craniosynostosis syndromes. Atlas Oral Maxillofac Surg Clin North Am. 2014; 22(2):103-10.
3. Tahiri Y, Paliga JT, Vossough A, Bartlett SP, Taylor JA. The spheno-occipital synchondrosis fuses prematurely in patients with Crouzon syndrome and midface hypoplasia compared with ageand gender-matched controls. J Oral Maxillofac Surg. 2014; 72(6):1173-9.
4. Pal US, Gupta C, Chellappa AA. Crouzon syndrome with primary optic nerve atrophy and normal brain functions: A case report. J Oral Biol Craniofac Res. 2012; 2(2):116-8.
5. Opperman LA. Cranial sutures as intramembranous bone growth sites. Dev Dyn. 2000; 219(4):472-85. 6. Hoyos SM. Sindrome De Crouzon. Rev Actual Clin. 2014; 46:2457.
7. Kirmi O, Lo SJ, Johnson D, Anslow P. Craniosynostosis: a radiological and surgical perspective. Semin Ultrasound CT. 2009; 30(6):492-512.
8. Mahima G, Karthikeya P, Ekta P, Prasannasrinivas. Deshpande Crouzon Syndrome. Int J Contemp Dent. 2011; 2(3):80-3.
9. Katsianou MA, Adamopoulos C, Vastardis H, Basdra EK. Signaling mechanisms implicated in cranial sutures pathophysiology: Craniosynostosis. BBA Clin 2016; 6:165-176.
10. Proffit WR, Fields HW. Ortodoncia Contemporánea. 4 ed. Barcelona; 2008.
11. KabbaniH , Raghuveer TS . Craniosynostosis. Am Fam Physician. 2004; 69(12):2863-70.
12. Pournima G, Monica Y, Meghna S,. Crouzon Syndrome: A Case Report. Eur J Dent Med. 2012; 4(1):8-13.
13. Bowling EL, Burstein FD. Crouzon syndrome. Optometry. 2006; 77(5):217- 22.
14. 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(4):373-9.
15. Doerga PN, Spruijt B, Mathijssen IM, Wolvius EB, Joosten KF, van der Schroeff MP. Upper airway endoscopy to optimize obstructive sleep apnea treatment in Apert and Crouzon syndromes. J Craniomaxillofac Surg. 2016; 44(2):191-6.
16. Pagnoni M, Fadda MT, Spalice A, Amodeo G, Ursitti F, Mitro V, et al. Surgical timing of craniosynostosis: what to do and when. J Craniomaxillofac Surg. 2014; 42(5): 513-9.
17. Robin NH, Falk MJ, Haldeman-Englert CR. FGFR-Related Craniosynostosis Syndromes. Gene Reviews (R). Seattle (WA) 1993.
18. Galvin BD, Hart KC, Meyer AN, Webster MK, Donoghue DJ. Constitutive receptor activation by Crouzon syndrome mutations in fibroblast growth factor receptor (FGFR)2 and FGFR2/Neu chimeras. Proc Natl Acad Sci USA. 1996; 93(15):7894-9.
19. Mansukhani A, Bellosta P, Sahni M, Basilico C. Signaling by fibroblast growth factors (FGF) and fibroblast growth factor receptor 2 (FGFR2)-activating mutations blocks mineralization and induces apoptosis in osteoblasts. J Cell Biol. 2000; 149(6):1297-308.
20. Cohen MM, Jr, Kreiborg S. Birth prevalence studies of the Crouzon syndrome: comparison of direct and indirect methods. Clin Genet.1992; 41(1): 12-5.
21. Vidal Sanahuja R, Gean Molins E, Sánchez Garré C, Quilis Esquerra J, García Fructuoso G, Costa Clara JM. Síndrome de Crouzon: a propósito de 2 casos. Entidades craneoestenóticas alélicas de los genes FGFR. An Pediatr. 2012; 77(4):272-8.
22. Nurko C, Quinones R. Dental and orthodontic management of patients with Apert and Crouzon syndromes. Oral Maxillofac Surg Clin North Am. 2004; 16(4):541-53.
23. Hernández CP. Terapia miofuncional en los síndromes craneofaciales. Bol AELFA. 2012; 12(2):54-60.
24. Vargervik 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(4 Suppl):S68-73.
25. Liou EJ, Tsai WC. A new protocol for maxillary protraction in cleft patients: repetitive weekly protocol of alternate rapid maxillary expansions and constrictions. Cleft Palate-Cran. J 2005; 42(2):121-7.
26. Abu-Sittah GS, Jeelani O, Dunaway D, Hayward R. Raised intracranial pressure in Crouzon syndrome: incidence, causes,and management. J Neurosurg Pediatr. 2016; 17(4):469-75.
2. Dicus BC, Golden BA, Turvey TA. Craniosynostosis syndromes. Atlas Oral Maxillofac Surg Clin North Am. 2014; 22(2):103-10.
3. Tahiri Y, Paliga JT, Vossough A, Bartlett SP, Taylor JA. The spheno-occipital synchondrosis fuses prematurely in patients with Crouzon syndrome and midface hypoplasia compared with ageand gender-matched controls. J Oral Maxillofac Surg. 2014; 72(6):1173-9.
4. Pal US, Gupta C, Chellappa AA. Crouzon syndrome with primary optic nerve atrophy and normal brain functions: A case report. J Oral Biol Craniofac Res. 2012; 2(2):116-8.
5. Opperman LA. Cranial sutures as intramembranous bone growth sites. Dev Dyn. 2000; 219(4):472-85. 6. Hoyos SM. Sindrome De Crouzon. Rev Actual Clin. 2014; 46:2457.
7. Kirmi O, Lo SJ, Johnson D, Anslow P. Craniosynostosis: a radiological and surgical perspective. Semin Ultrasound CT. 2009; 30(6):492-512.
8. Mahima G, Karthikeya P, Ekta P, Prasannasrinivas. Deshpande Crouzon Syndrome. Int J Contemp Dent. 2011; 2(3):80-3.
9. Katsianou MA, Adamopoulos C, Vastardis H, Basdra EK. Signaling mechanisms implicated in cranial sutures pathophysiology: Craniosynostosis. BBA Clin 2016; 6:165-176.
10. Proffit WR, Fields HW. Ortodoncia Contemporánea. 4 ed. Barcelona; 2008.
11. KabbaniH , Raghuveer TS . Craniosynostosis. Am Fam Physician. 2004; 69(12):2863-70.
12. Pournima G, Monica Y, Meghna S,. Crouzon Syndrome: A Case Report. Eur J Dent Med. 2012; 4(1):8-13.
13. Bowling EL, Burstein FD. Crouzon syndrome. Optometry. 2006; 77(5):217- 22.
14. 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(4):373-9.
15. Doerga PN, Spruijt B, Mathijssen IM, Wolvius EB, Joosten KF, van der Schroeff MP. Upper airway endoscopy to optimize obstructive sleep apnea treatment in Apert and Crouzon syndromes. J Craniomaxillofac Surg. 2016; 44(2):191-6.
16. Pagnoni M, Fadda MT, Spalice A, Amodeo G, Ursitti F, Mitro V, et al. Surgical timing of craniosynostosis: what to do and when. J Craniomaxillofac Surg. 2014; 42(5): 513-9.
17. Robin NH, Falk MJ, Haldeman-Englert CR. FGFR-Related Craniosynostosis Syndromes. Gene Reviews (R). Seattle (WA) 1993.
18. Galvin BD, Hart KC, Meyer AN, Webster MK, Donoghue DJ. Constitutive receptor activation by Crouzon syndrome mutations in fibroblast growth factor receptor (FGFR)2 and FGFR2/Neu chimeras. Proc Natl Acad Sci USA. 1996; 93(15):7894-9.
19. Mansukhani A, Bellosta P, Sahni M, Basilico C. Signaling by fibroblast growth factors (FGF) and fibroblast growth factor receptor 2 (FGFR2)-activating mutations blocks mineralization and induces apoptosis in osteoblasts. J Cell Biol. 2000; 149(6):1297-308.
20. Cohen MM, Jr, Kreiborg S. Birth prevalence studies of the Crouzon syndrome: comparison of direct and indirect methods. Clin Genet.1992; 41(1): 12-5.
21. Vidal Sanahuja R, Gean Molins E, Sánchez Garré C, Quilis Esquerra J, García Fructuoso G, Costa Clara JM. Síndrome de Crouzon: a propósito de 2 casos. Entidades craneoestenóticas alélicas de los genes FGFR. An Pediatr. 2012; 77(4):272-8.
22. Nurko C, Quinones R. Dental and orthodontic management of patients with Apert and Crouzon syndromes. Oral Maxillofac Surg Clin North Am. 2004; 16(4):541-53.
23. Hernández CP. Terapia miofuncional en los síndromes craneofaciales. Bol AELFA. 2012; 12(2):54-60.
24. Vargervik 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(4 Suppl):S68-73.
25. Liou EJ, Tsai WC. A new protocol for maxillary protraction in cleft patients: repetitive weekly protocol of alternate rapid maxillary expansions and constrictions. Cleft Palate-Cran. J 2005; 42(2):121-7.
26. Abu-Sittah GS, Jeelani O, Dunaway D, Hayward R. Raised intracranial pressure in Crouzon syndrome: incidence, causes,and management. J Neurosurg Pediatr. 2016; 17(4):469-75.
- María-Alejandra Cardozo, José-Manuel Martínez, Natalia Molina, Jesús-Alberto Hernández, Effects of functional maxillary orthopedics on the dimensions of the dental arches in patients with deep bite in primary dentition , Revista Estomatología: Vol. 24 No. 1 (2016)
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