Background
Methods
Protocol development
Review question
Eligibility criteria
Search strategy and study selection
PubMed | (“Mobius Syndrome/classification“[Mesh] OR “Mobius Syndrome/complications“[Mesh] OR “Mobius Syndrome/diagnosis“[Mesh] OR “Mobius Syndrome/embryology“[Mesh] OR “Mobius Syndrome/epidemiology“[Mesh] OR “Mobius Syndrome/etiology“[Mesh] OR “Mobius Syndrome/genetics“[Mesh] OR “Mobius Syndrome/history“[Mesh] OR “Mobius Syndrome/immunology“[Mesh] OR “Mobius Syndrome/microbiology“[Mesh] OR “Mobius Syndrome/pathology“[Mesh] OR “Mobius Syndrome/physiopathology“[Mesh] OR “Mobius Syndrome/prevention and control“[Mesh] OR “Mobius Syndrome/rehabilitation“[Mesh] OR “Mobius Syndrome/surgery“[Mesh] OR “Mobius Syndrome/therapy“[Mesh] ) |
Scopus and Web of Science | TITLE-ABS-KEY (Moebius syndrome AND Oral manifestations AND Dental treatment OR Oral Health) |
Quality assessment, data extraction, and statistical analysis
Results
Study selection
Study characteristics
No. | Authors/ Year/ Country | Type of Study/Cases No. | A/G | Extraoral Manifestations | Oral Manifestations |
---|---|---|---|---|---|
1 | Aren G, (2002), Turkey [30] | Case Report 1 | 7/M | Bilateral facial paralysis, lack of facial expression, blinking, visual problems, speech difficulties, and malformations of the upper and lower limbs | Tongue deformities and dental caries |
2 | De Serpa Pinto et al., (2002), Brazil [15] | Case Series 12 | 9 = M, 3 = F, Mean age = 6 | 12 = Bilateral and unilateral facial paralysis and malformations of the upper and lower limbs | 12 = Micrognathia, microstomia, cleft palate, gothic palate, tongue deformities anterior open bite |
3 | Chungyoon and Zakaria (2003), USA [20] | Case Report 1 | 18/M | Lack of facial expression, blinking, and visual problems | Periodontal disease, dental caries, and odontogenic abscesses |
4 | Sensat et al., (2003), USA [21] | Case Report 1 | 40/M | Bilateral facial paralysis, lack of facial expression, dysphagia, blinking, and visual problems | Microdontia, microstomia, periodontal disease, and dental caries |
5 | Magalhães et al., (2006), Brazil [16] | Case Series 29 | 18 = M, 11 = F, Mean age = 1,5 | 29 = Blinking, visual problems, and malformations of the upper and lower limbs | 29 = Micrognathia, tongue deformities, cleft palate, and gothic palate |
6 | Lima et al., (2008), Brazil [17] | Case Report 1 | 5/M | Unilateral facial paralysis, lack of facial expression, blinking, and visual problems, speech difficulties, and compromised breathing | Tongue deformities and anterior open bite |
7 | Scarpelli et al., (2008), Brazil [18] | Case Report 1 | 5/M | Bilateral facial paralysis and speech difficulties | Micrognathia, gothic palate, tongue deformities, dental caries, Class II malocclusion, and anterior open bite |
8 | Escoda-Francolí et al., (2009), Spain [23] | Case Report 1 | 49/F | Malformations of the upper and lower limbs | Microstomia, total edentulism, and cleft palate |
9 | Cai et al., (2012), China [25] | Case Series 3 | 2 = M, 1 = F, Mean age = 19,3 | 2 = Bilateral facial paralysis, blinking, and visual problems 1 = Unilateral facial paralysis, blinking, and visual problems | 3 = Labial incompetence, anterior open bite with mandibular hyperplasia, crowded dentition, tongue deformities |
10 | Guijarro-Martínez et al., (2012), Spain [24] | Case Report 1 | 15/F | Lack of facial expression and speech difficulties | Labial incompetence, anterior open bite tongue deformities, reduced vestibular depth in the upper and low lip, and periodontal disease |
11 | Bianchi et al., (2013), Italy [26] | Case Report 1 | 23/M | Bilateral facial paralysis | Micrognathia, and Class II malocclusion |
12 | Greene et al., (2015), United Kingdom [31] | Care Report 1 | 12/M | Speech difficulties | Velopharyngeal dysfunction |
13 | Pradhan et al., (2015), Australia [32] | Case Report 1 | 19/F | Lack of facial expression | Dental caries |
14 | Magnifico et al., (2017) Italy [27] | Case Report 1 | 23/M | Bilateral facial paralysis, blinking, and visual problems | Micrognathia, labial incompetence, and crowded dentition |
15 | Magnifico et al., (2017) Italy [36] | Case series 58 | NI / Mean age = 10 | 50 = Strabismus, absence of blinking 24 = Malformations of the upper and lower limbs 28 = Reduced TMJ movements | 42 = Tongue deformities 8 = Lip and palatal cleft 40 = Labial incompetence |
16 | Mahrous et al., (2018), USA [22] | Case Report 1 | 40/F | Bilateral facial paralysis, blinking, visual problems, and malformations of the upper and lower limbs | Micrognathia, complete edentulism, reduced vestibular depth, microstomia, tongue deformities, |
17 | Cudzilo and Brzozowska, (2019), Poland [33] | Case Report 1 | 9/F | Bilateral facial paralysis and lack of facial expression | Crowded dentition, hypoplastic enamel, and periodontal disease |
18 | Freire et al., (2019), Brazil [19] | Case Report 1 | 5 months/F | Lack of facial expression, blinking, and visual problems | Micrognathia, microstomia, and tongue deformities |
19 | Hassib et al., (2020), Egypt [34] | Case Series 4 | 4 = M Mean age = 3,8 | 4 = Lack of facial expression, blinking, visual problems, and malformations of the upper and lower limbs | 4 = Gothic palate, dental caries, periodontal disease, tongue deformities, microdontia, enamel hypoplasia, premature eruption |
20 | Chen et al., (2021), China [12] | Case report 1 | 21/M | Lack of facial expression, blinking, and visual problems | Microstomia, crowded dentition, periodontal disease, dental caries, and gothic palate |
21 | Lee and Moon, (2022). Korea [35] | Case Report 1 | 7/M | Blinking, visual problems, and malformations of the upper and lower limbs | Microstomia, tongue deformities, anterior deep bite |
22 | Duggal et al., (2023), India [28] | Case Report 1 | 9/F | Bilateral facial paralysis, lack of facial expression, blinking, and visual problems | Anterior open bite, crowded dentition |
23 | Mittal et al., (2023), India [29] | Case Report 1 | 5/M | Hypotonia, malformations of the upper and lower limbs, lack of facial expression, blinking and visual problems, and compromised breathing | Gothic palate, dental caries, periodontal disease, hypoplastic enamel, microstomia, and micrognathia |
Oral and extraoral manifestations of patients with MS
Comprehensive dental management of patients with MS
No. | Authors | Treatment | Dental Discipline | Follow-up (years) |
---|---|---|---|---|
1 | Aren G [30] | Dental extractions Dental restorations | Pediatric dentistry Restorative dentistry Oral and maxillofacial surgery | NI |
2 | De Serpa Pinto [15] | Periodontal treatment Dental restorations | Pediatric dentistry Restorative dentistry | NI |
3 | Chungyoon and Zakaria [20] | Periodontal treatment Dental restorations Dental restorations | Pediatric dentistry Periodontics Restorative dentistry Oral and maxillofacial surgery | NI |
4 | Sensat et al., [21] | Periodontal treatment Dental restorations Dental prostheses | Periodontics Restorative dentistry Prosthodontics | NI |
5 | Magalhães et al., [16] | Orthopedic appliances | Pediatric dentistry Orthodontic and maxillary orthopedics | 8 |
6 | Lima et al., [17] | Frenectomy Removable orthodontic appliance | Oral and maxillofacial surgery Orthodontic and maxillary orthopedic Pediatric dentistry | 1 |
7 | Scarpelli et al., [18] | Behavioral therapy Dental restorations | Restorative dentistry Pediatric dentistry | NI |
8 | Escoda-Francolí et al., [23] | Dental implants | Oral implantology | 8 |
9 | Cai et al., [25] | Orthognathic surgery | Oral and maxillofacial surgery | 4 |
10 | Guijarro-Martínez et al. [24], | Pre-surgical orthodontics Orthognathic surgery | Orthodontic and maxillary orthopedic Oral and maxillofacial surgery Pediatric dentistry | 0.5 |
11 | Bianchi et al., [26] | Dental extractions Orthodontic appliance Orthognathic surgery Smile surgery | Orthodontic and maxillary orthopedic Oral and maxillofacial surgery | 5 |
12 | Greene et al., [31] | Orthopedic appliances | Orthodontic and maxillary orthopedic Oral and maxillofacial surgery Pediatric dentistry | 1,3 |
13 | Pradhan et al., [32] | Dental extractions Dental restorations | Restorative dentistry Oral and maxillofacial surgery | 7 |
14 | Magnifico et al., [27] | Orthodontic appliance Orthognathic surgery Smile surgery | Orthodontic and maxillary orthopedic | 4 |
15 | Magnifico et al., [36] | Removable/ fixed orthodontic appliance | Orthodontic and maxillary orthopedic | NI |
16 | Mahrous et al., [22] | Implant-supported dentures | Oral implantology Prosthodontics | 0.6 |
17 | Cudzilo and Brzozowska, [33] | Dental extractions Removable / fixed orthodontic appliance | Orthodontic and maxillary orthopedic Oral and maxillofacial surgery Pediatric dentistry | NI |
18 | Freire et al., [19] | Frenectomy | Pediatric dentistry Oral and maxillofacial surgery | The patient had died before reaching one year of age of a reported cardiac arrest. |
19 | Hassib et al., [34] | Dental extractions Periodontal treatment Dental restorations Root canal treatment Dentures | Pediatric dentistry Periodontics Restorative dentistry Oral and maxillofacial surgery Prosthodontics | NI |
20 | Chen et al., [12] | Dental extractions Periodontal treatment Dental restorations Root canal treatment Periapical surgery | Periodontics Restorative dentistry Oral and maxillofacial surgery | 2 |
21 | Lee and Moon, [35] | Removable / fixed orthodontic appliance | Orthodontic and maxillary orthopedic Pediatric dentistry | 9,4 |
22 | Duggal et al. [28], | Dental extractions Orthodontic camouflage | Pediatric dentistry Orthodontic and maxillary orthopedic | 1 |
23 | Mittal et al., [29] | Dental extractions Periodontal treatment Dental restorations Root canal treatment Dentures | Pediatric dentistry Periodontics Restorative dentistry Oral and maxillofacial surgery Prosthodontics | 0.6 |
Variables | Values | % |
---|---|---|
Articles | 23 | |
Total Cases | 124 | |
Age (years) | ||
< 15 ≥ 15 | 112 12 | 90.32 10.68 |
Mean ± SD Range (Min-Max) | 14.92 ± 12.80 0.5–49 | |
Gender | ||
Male Female | 42 24 | 64.00 36.00 |
Extraoral Manifestations | ||
Hypotonia | 1 | 0.80 |
Speech difficulty Bilateral facial paralysis Unilateral facial paralysis Compromised breathing Lack of facial expression Blinking and visual problems Malformations of the upper and lower limbs | 5 16 8 2 15 97 73 | 4.03 12.90 6.45 1.61 12.09 78.22 58.87 |
Oral Manifestations | ||
Cleft palate Dental caries Microstomia Microdontia Micrognathia Dental crowding Anterior open bite Gothic palate Periodontal disease Tongue deformities Premature eruption Hypoplastic enamel Labial incompetence Class II Malocclusion Total/partial edentulism Reduced vestibular depth Mandibular/Maxilla hyperplasia | 42 11 19 2 47 7 19 20 10 97 4 2 45 2 2 2 1 | 33.87 8.87 15.32 1.61 37.90 5.64 15.32 16.12 8.06 78.22 3.22 1.61 36.29 1.61 1.61 1.61 0.80 |
Dental Discipline | n(23) | |
Periodontics Prosthodontics Pediatric dentistry Oral implantology Restorative dentistry Oral and maxillofacial surgery Orthodontic and maxillary orthopedic | 5 4 14 2 9 13 10 | 21.73 17.39 60.86 8.69 39.13 56.52 43.47 |
Follow-up (years) | ||
Mean ± SD | 3.74 ± 3.22 |
Evaluation of the methodological quality of the selected studies
No. | Authors | Q1 | Q2 | Q3 | Q4 | Q5 | Q6 | Q7 | Q8 | Overall Score and Quality |
---|---|---|---|---|---|---|---|---|---|---|
1 | Aren G [30] | Y | Y | Y | Y | Y | N | Y | Y | 87.5 |
2 | Chungyoon and Zakaria [20] | Y | Y | Y | Y | Y | N | Y | Y | 87.5 |
3 | Sensat et al., [21] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
4 | Lima et al., [17] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
5 | Scarpelli et al., [18] | Y | Y | Y | Y | U | N | Y | Y | 75 |
6 | Escoda-Francolí et al., [23] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
7 | Guijarro-Martínez et al., [24] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
8 | Bianchi et al., [26] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
9 | Greene et al., [31] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
10 | Pradhan et al., [32] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
11 | Magnifico et al., [27] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
12 | Mahrous et al., [22] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
13 | Cudzilo and Brzozowska, [33] | Y | Y | Y | Y | Y | N | Y | Y | 87.5 |
14 | Freire et al., [19] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
15 | Chen et al., [12] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
16 | Lee and Moon, [35] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
17 | Duggal et al., [28] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
18 | Mittal et al., [29] | Y | Y | Y | Y | Y | Y | Y | Y | 100 |
Discussion
Surgical, orthodontic, and orthopedic treatment of MS
Restorative, periodontal, and prosthetic treatment of MS
Conclusions
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The most frequent extraoral findings of MS are blinking and visual problems, malformations of the upper and lower limbs, bilateral facial paralysis, lack of facial expression, and unilateral facial paralysis.
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The most representative intraoral findings of MS are tongue deformities, micrognathia, labial incompetence, cleft palate, gothic palate, microstomia, anterior open bite, dental caries, and periodontal disease, which have become a great challenge for management by the dentist since it involves the participation of different specialists in the area.
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Thus, patients with MS are subjected to different surgical procedures, orthodontic and orthopedic treatments, and restorative procedures to resolve their problems.
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A comprehensive dental treatment plan adapted to each patient is required, covering both the resolution of the problem and the maintenance of oral health.