Received 02.10.2020
DOI: 10.35556/idr-2021-1(94)50-57
Clinical case of orthopaedic dental rehabilitation of a patient with Scheuthauer-Marie-Sainton syndrome
Galonsky V.G.¹, ², Tarasova N.V.¹, Surdo E.S.¹, Gradoboev A.V.³
¹ V.F. Voino-Yasenetsky Krasnoyarsk State Medical University
660036, Russia, Krasnoyarskiy kray, Krasnoyarsk, St. Partizana Zheleznyaka, 1
² Krasnoyarsk Science Center of the Siberian Branch of the Russian Academy of Sciences, Scientific Research Institute of Medical Problems of the North (SRI MPN)
660022, Russia, Krasnoyarskiy kray, Krasnoyarsk, St. Partizana Zheleznyaka, 3G
³ Dental clinic “Gelios” LLC
662313, Russia, Krasnoyarskiy kray, Sharypovo, 6 Mikrorayon, 25

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The article presents results of content analysis of Russian and foreign reference materials, scientific and educational literature regarding a rare pathology: the Scheuthauer-Marie-Sainton syndrome (cleidocranial dysplasia). The results are provided in the form of an explicit diagnostic algorithm for dental practitioners revealing this disease in patients in orthopaedic dentistry and orthodontics practice being a guideline for involvement of allied general profile specialists in consulting of such a patient.
The clinical case reflected in the paper describes orthopaedic dental treatment provided to a 19-years-old female patient with cleidocranial dysplasia as well as a complex and ambiguous clinical picture of the oral cavity. The case demonstrates possibilities in efficacy of dental rehabilitation procedures for this category of patients using minimally invasive methods as the most justified and predictable approach providing a wide spectrum of furtherclinical decisions in short- and long-term patient management after treatment.
Keywords: Scheuthauer-Marie-Sainton syndrome, cleidocranial dysplasia, cleidocranial dysostosis, congenital maxillofacial pathology, tooth retention, supernumerary teeth, exfoliation delay of teeth.
For citation: Galonsky V.G., Tarasova N.V., Surdo E.S., Gradoboev A.V. A clinical case of orthopaedic dental rehabilitation of a patient with Scheuthauer-Marie-Sainton syndrome. Stomatology for All / Int. Dental Review. 2021, no.1(94): 50-57 (In Russian). doi: 10.35556/idr-2021-1(94)50-57

1. Belyakov Yu.A. Hereditary diseases and syndromes in dental practice. Ed. 2nd, re-work. and add. Moscow: JSC “Publishing house “Medicine “, 2008, 240 p. (In Russian).
2. Vinogradova T.F. Atlas of dental diseases in children. Tutorial. M .: MEDpress-inform, 2007, 168 p. (In Russian).
3. Glukhova Yu.M., Shpak N.S., Sinyakov V.P. Analysis of the causes and clinical manifestations of combined pathology (retention of teeth and malocclusion) in adolescents and adults according to the data of cone-beam computed tomography. Institute of Stomatology. 2016, no.1(70): 72—73 (In Russian).
4. Kenneth L. Jones. David Smith hereditary syndromes. Atlas-reference. Moscow: Praktika, 2011, 1024 p. (In Russian).
5. Paltseva A.I., Keda L.N., Ponomarenko S.M., Motyuk I.N., Kosarenkova Zh.A. Clinical observation of kleidocranial dysplasia. Sat. materials of the interregional scientific and practical conference with international participation. Topical issues of pediatrics. 2016: 86—90 (In Russian).
6. Petrov R.S., Kurilo L.F., Demikova N.S., Kozlova S.I. Hereditary syndromes, including developmental disorders of the oral cavity. Clinical and experimental morphology. 2014, no.4(12): 4—13 (In Russian).
7. Kozlova S.I., Demikova N.S., Semanova E., Blinnikova O.E. Hereditary syndromes and medical genetic counseling. Atlas-reference. Ed. 2nd, rev. and add. Moscow: Practice, 1996, 416 p. (In Russian).
8. Ramm N.L., Zakirov T.V., Shutova T.O., Shishma-reva A.S. The use of surgical methods in the complex treatment of retention of the permanent incisors of the upper jaw. Dentistry problems. 2011, no.2: 51—54 (In Russian).
9. Sleptsov A.R. Prenatal diagnosis of rare congenital malformations and syndromes. LV. Cleidocranial dysplasia (Marie-Sainton syndrome). Prenatal diagnostics. 2012, Vol.11, no.3: 226—229 (In Russian).
10. Koperchak A.V., Milyukov A.Yu., Gilev Ya.Kh., Konev S.V., Malev V.A. A case of Sheithauer-Marie-Senton syndrome. Polytrauma. 2007, no.1: 67—69 (In Russian).
11. Shulkina N.M., Dunaevskaya I.I., Zharkova L.A. Dentofacial anomaly as part of a rare general somatic disease. Institute of Stomatology. 2006, no.4(33): 80—81 (In Russian).
12. Tripathi S., Singh R.D., Singh S.V. et al. A case of Cleidocranial Dysostosis: Dilemma for a Prosthodontist. The journal of the Indian Prosthodontic Society. 2012, V.12, Issue: 252—255.doi: 10.1007/s13191-012-0135-8
13. Abdelnasir G., Osman M., Awadalkreem F. Full-mouth rehabilitation of a patient with cleidocranial dysplasia using immediately loaded basal implant-supported fixed prosthesis: A case report. International Journal of Surgery Case Reports. 2019, 65: 344—348. doi:
10.1016/j.ijscr.2019.11.0514. Ambard A.J., Clemens S., Phillips D.S. Multidisciplinary implant rehabilitation of a patient with cleidocranial dysostosis: A journey from Age13 to 21. Journal of Prosthodontics. 2019, 28(4): 361— 364. doi: 10.1111/jopr.13039
15. Bharti K., Goswami M. Cleidocranial dysplasia: A report of two cases with brief review. Intractable Rare Dis. Res. 2016, 5(6): 117—120. doi: 10.5582/ irdr.2016.01022
16. Singh S., Sharma S., Singh H., Wazir N.D. Cleidocranial dysplasia: A Case Report Illustrating Diagnostic Clinical and Radiological Findings. J. Clin. Diagn. Res. 2014, 8(6): 19—20.
17. Zhu Y., Zou Y., Yu Q., Sun H., Mou S., Xu Sh., Zhu M. Combined surgical-orthodontic treatment of patients with cleidocranial dysplasia: case report and review of the literature. Orphanet J. Rare Dis. 2018, 13(1): 217. doi:
18. Lu H., Zeng B., Yu D., Jing X., Hu B., Zhao W., Wang Y. Complex dental anomalies in a belatedly diagnosed cleidocranial dysplasia patient. Imaging Sci. Dent. 2015, 45(3): 187—192. doi: 10.5624/isd.2015.45.3.187
19. Lee C., Jung H., Baek J., Leem D., Ko S. Manifestation and treatment in a cleidocranial dysplasia patient with a RUNX2 (T420I) mutation. Maxillofacial plastic and reconstructive surgery. 2015, 37(1): 41. doi:
10.1186/s40902-015-0042-020. Omami G. Multiple unerupted and supernumerary teeth in a patient with cleidocranial dysplasia. Radiol. Case Rep. 2018, 13(1): 118— 120. doi: 10.1016/j.radcr.2017.11.009
21. Li Z., Wang J., Gao M., Wu D., Chang X. Orthodontic treatment of a patient with cleidocranial dysplasia: A case report. Exp. Ther. Med. 2016, 12(2): 690—694. 22. Farronato G., Maspero C., Farronato D., Gioventu S. Orthodontic treatment in a patient with Cleidocranial Dysostosis. The Angle of Orthodontics. 2009, 79(1): 178—185. doi: 10.2319/111307-393.1
23. Roberts T., Stephen L., Beighton P. Cleidocranial dysplasia: a review of the dental, hystorical, and practical implications with overview of the South African experience. Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. 2013, 115(1): 46—55. doi: 10.1016/j.oooo.2012.07.435
24. Greene S., Kau C.H., Sittitavornwong S., Powell K., Childers N.K., MacDougall M., Lamani E. Surgical management and evaluation of the craniofacial growth and morphology in Cleidocranial dysplasia. J. Craniofacial Surg. 2018, 29(4): 959—965. doi: 10.1097/SCS.0000000000004334
25. Petropoulos V.C., Balshi T.J., Wolfinger G.J., Balshi S.F. Treatment of a patient with cleidocranial dysplasia using a single-stage implant protocol. Journal of prosthodontics. 2011, 20(2): 26—31. doi: 10.1002/ 9781119274605.ch17
26. Petropoulos V.C., Balshi T.J., Balshi S.F., Wolfinger G.J. Treatment of a patient with cleidocranial dysplasia using osseointegrated implants: а patient report. Int. J. Oral Max. Impl. 2004, 19(2): 282—287. doi: 10.1002/ 9781119274605.ch17