Treatment of Orthognathic Surgical Class III Patient with Coffin–Siris Syndrome: A Case Report
Source abstract
Abstract
We present a case report of a 26-year-old female suffering from Coffin–Siris Syndrome, who underwent orthodontic treatment and surgery to solve her malocclusion and to improve her aesthetics and functional occlusion. Methods: The presurgical phase involved multibracket self-ligating attachments, namely a Damon prescription. The patient underwent maxillofacial surgery to correct the severe skeletal malocclusion and to relocate the bone bases to the right position. Post-surgical orthodontic treatment was performed to complete the alignment. Results: The patient’s aesthetics and functional abilities improved. Conclusions: Syndromic patients can undergo orthodontic treatment if comorbidities and collaboration allow it. The support and collaboration of families and psychotherapists must be considered, but clinical cases of syndromic patients can be faced and solved. Obviously, each syndromic patient is considered unique, and the risk–benefit ratio must be correctly assessed for each one.
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Research context
OpenAlex topics: Chromatin Remodeling and Cancer · Cancer Mechanisms and Therapy · Neurofibromatosis and Schwannoma Cases
SciVX discovery tags are terminology-based. OpenAlex topics are source-provided classifications.
Affiliations
Department of Innovative Technologies in Medicine & Dentistry, University “G. D’Annunzio” of Chieti-Pescara, 66100 Chieti, Italy
Department of Anatomic, Histologic, Medical-Legal Sciences and of Locomotor System, Rome University Sapienza, 00185 Rome, Italy
Department of Oral and Maxillofacial Sciences, Sapienza University, 00161 Rome, Italy
References (27)
Show source-provided references
1. Wang, X., Wu, H., Sun, H., Wang, L., and Chen, L. (2022). ARID2, a Rare Cause of Coffin-Siris Syndrome: A Clinical Description of Two Cases. Front. Pediatr., 10.10.3389/fped.2022.911954 ↗
2. Vergano, S.S., Santen, G., Wieczorek, D., Wollnik, B., Matsumoto, N., and Deardorff, M.A. (1993). Coffin-Siris Syndrome Synonym: Fifth Digit Syndrome. GeneReviews, University of Washington.
3. Numerous BAF complex genes are mutated in Coffin-Siris syndrome10.1002/ajmg.c.31406 ↗
4. Coffin-Siris syndrome and related disorders involving components of the BAF (mSWI/SNF) complex: Historical review and recent advances using next generation sequencing10.1002/ajmg.c.31415 ↗
5. Vasko, A., Drivas, T.G., and Schrier Vergano, S.A. (2021). Genotype-Phenotype Correlations in 208 Individuals with Coffin-Siris Syndrome. Genes, 12.10.3390/genes12060937 ↗
6. Coffin-Siris syndrome: Review and presentation of new cases from a questionnaire study10.1002/1096-8628(20010215)99:1<1::AID-AJMG1127>3.0.CO;2-A ↗
7. Successful difficult airway management of a child with Coffin-siris syndrome10.1002/ccr3.1045 ↗
8. Navarro-Bielsa, A., Ruiz-de-Larramendiz, D.R., Abenia-Usón, P., Gracia-Cazaña, T., and Gilaberte, Y. (An. Bras. Dermatol., 2024). Nail dysplasia and digital hypoplasia—Coffin-Siris syndrome, An. Bras. Dermatol., in press.10.1016/j.abd.2022.11.010 ↗
9. Haploinsufficiency of ARID1B, a member of the SWI/SNF-a chromatin-remodeling complex, is a frequent cause of intellectual disability10.1016/j.ajhg.2012.02.007 ↗
10. Genotype-phenotype correlation of Coffin-Siris syndrome caused by mutations in SMARCB1, SMARCA4, SMARCE1, and ARID1A10.1002/ajmg.c.31407 ↗
11. The ARID1B phenotype: What we have learned so far10.1002/ajmg.c.31414 ↗
12. Clinical correlations of mutations affecting six components of the SWI/SNF complex: Detailed description of 21 patients and a review of the literature10.1002/ajmg.a.35933 ↗
13. The Coffin–Siris syndrome: A proposed diagnostic approach and assessment of 15 overlapping cases10.1002/ajmg.a.35415 ↗
14. Oral findings in Coffin-Siris syndrome: A case report
15. Efficacy of a Newly Designed Cephalometric Analysis Software for McNamara Analysis in Comparison with Dolphin Software
16. Cone beam computed tomography for dental and maxillofacial imaging: Technique improvement and low-dose protocols10.1007/s11547-017-0758-2 ↗
17. Orthodontic camouflage versus orthodontic-orthognathic surgical treatment in borderline class III malocclusion: A systematic review10.1007/s00784-022-04685-6 ↗
18. Macrì, M., D’Albis, G., D’Albis, V., Timeo, S., and Festa, F. (2023). Augmented Reality-Assisted Surgical Exposure of an Impacted Tooth: A Pilot Study. Appl. Sci., 13.10.3390/app131911097 ↗
19. Orthognathic surgery: A hierarchy of stability
20. Comprehensive post orthognathic surgery orthodontics: Complications, misconceptions, and management10.1016/j.coms.2019.09.003 ↗
21. Le Fort I Osteotomy10.1016/j.cxom.2015.10.001 ↗
22. Mandibular reduction10.1016/j.jemermed.2007.06.037 ↗
23. Rigid skeletal fixation of fractures10.1016/S0278-2391(10)80016-3 ↗
24. Coffin Siris Syndrome: A Rare Case Report
25. Macrì, M., Toniato, E., Murmura, G., Varvara, G., and Festa, F. (2022). Midpalatal suture density as a function of sex and growth-pattern-related variability via CBCT evaluations of 392 adolescents treated with a rapid maxillary expander appliance. Appl. Sci., 12.10.20944/preprints202201.0167.v1 ↗
26. Macrì, M., Rendina, F., Feragalli, B., Pegreffi, F., and Festa, F. (2023). Prevalence of Ponticulus Posticus and Migraine in 220 Orthodontic Patients: A Cross-Sectional Study. Biology, 12.10.3390/biology12030471 ↗
27. Macrì, M., and Festa, F. (2022). Three-dimensional evaluation using CBCT of the mandibular asymmetry and the compensation mechanism in a growing patient: A case report. Front. Public Health, 10.10.3389/fpubh.2022.921413 ↗