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Cano-Rosás M, Benito-Cano J, Benito-Cano J, Diosdado-Cano JM, Benito-Duque P, Curto A. Multidisciplinary Treatment of Hemifacial Microsomia: Several Clinical Cases. Clin Pract 2024; 14:2410-2418. [PMID: 39585016 PMCID: PMC11587098 DOI: 10.3390/clinpract14060188] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2024] [Revised: 08/30/2024] [Accepted: 10/06/2024] [Indexed: 11/26/2024] Open
Abstract
Hemifacial microsomia is the second most common congenital anomaly of the craniofacial region. Hemifacial microsomia is characterised by unilateral hypoplasia of the ear. Treatment of this condition depends on the severity of the lesion. The treatment of hemifacial microsomia must be carried out by a multidisciplinary group of professionals familiar with this pathology, including plastic surgeons, parapsychologists, orthodontists, and paediatricians. In hemifacial microsomia, microtia is usually accompanied by alterations of the middle ear. Since the ear develops embryonically from the first and second branchial arches, the facial areas that also develop from these embryonic origins are usually affected to a greater or lesser degree, including through hypoplasia of the jaw, maxilla, zygomatic bones, and temporal bone, among others. Although jaw hypoplasia is the most evident deformity in craniofacial microsomia, microtia is the alteration that often has the greatest aesthetic impact on patients. Alterations in dentition are also common, typically presenting as a cephalad inclination of the anterior occlusal plane of the maxilla and mandible on the affected side. This study aims to review the surgical approach and evaluate the results of a paediatric case of hemifacial microsomia. Hemifacial microsomia is present at birth, and successful reconstruction is essential for the correct integration of such infantile patients into society. Multiple facial asymmetries as well as neonatal onset are a challenge for reconstructive surgery, and the importance of multidisciplinary treatment in these patients must be emphasised.
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Affiliation(s)
- Mónica Cano-Rosás
- Department of Surgery, Faculty of Medicine, University of Salamanca, Alfonso X El Sabio Avenue s/n, 37007 Salamanca, Spain;
| | | | | | | | - Pablo Benito-Duque
- Servicio de Cirugía Plástica y Reparadora del Hospital Ramón y Cajal, 28034 Madrid, Spain;
| | - Adrián Curto
- Department of Surgery, Faculty of Medicine, University of Salamanca, Alfonso X El Sabio Avenue s/n, 37007 Salamanca, Spain;
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Huh J, Park JS, Sodnom-Ish B, Yang HJ. Growth characteristics and classification systems of hemifacial microsomia: a literature review. Maxillofac Plast Reconstr Surg 2024; 46:18. [PMID: 38733452 PMCID: PMC11088588 DOI: 10.1186/s40902-024-00427-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2024] [Accepted: 04/25/2024] [Indexed: 05/13/2024] Open
Abstract
BACKGROUND Hemifacial microsomia is characterized by the hypoplasia of the mandible and temporomandibular joint, involving a variety of abnormalities of the craniofacial area. Since it gradually worsens as patients grow, it is necessary to understand the characteristics of facial bone growth and facial deformity in hemifacial microsomia patients in order to determine appropriate treatment timing and treatment methods. MAIN BODY Appropriate classification of hemifacial microsomia would facilitate accurate diagnosis, selection of treatment methods, and prognosis prediction. Therefore, in this article, we review previously published hemifacial microsomia classification and provide an overview of the growth of the facial skeleton and the characteristics of hemifacial microsomia-related facial deformities. The OMENS system is the most comprehensive classification method based on the characteristics of hemifacial microsomia deformity, but it needs to be improved to include malar/midface abnormalities and nerve involvement. In hemifacial microsomia, growth is progressing on the affected side, but to a lesser degree than the unaffected side. Therefore, surgical intervention in growing patients should be performed selectively according to the severity of deformity. CONCLUSION Understanding growth patterns is important to develop appropriate treatment protocols for correcting asymmetry in adult patients and to minimize secondary anomalies in growing patients.
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Affiliation(s)
- Joonyoung Huh
- Department of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, 101, Daehak-Ro, Jongno-Gu, Seoul, 03080, Korea
| | - Ji-Song Park
- Department of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, 101, Daehak-Ro, Jongno-Gu, Seoul, 03080, Korea
| | - Buyanbileg Sodnom-Ish
- Department of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, 101, Daehak-Ro, Jongno-Gu, Seoul, 03080, Korea
| | - Hoon Joo Yang
- Department of Oral and Maxillofacial Surgery, School of Dentistry, Seoul National University, 101, Daehak-Ro, Jongno-Gu, Seoul, 03080, Korea.
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Nardini LG, Val M, Colonna A, Cagidiaco EF, Ferrari M, Manfredini D. Treatment of Condylar Hypoplasia in Alagille Syndrome - A Case Report. Ann Maxillofac Surg 2024; 14:85-88. [PMID: 39184415 PMCID: PMC11340841 DOI: 10.4103/ams.ams_220_23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2023] [Revised: 02/11/2024] [Accepted: 02/19/2024] [Indexed: 08/27/2024] Open
Abstract
Rationale Alagille syndrome is a rare genetic disorder with dental and facial abnormalities in the head-and-neck area. It is autosomal dominant and occurs in approximately 1 in 100,000 people. No cases of Alagille Syndrome (ALGS) with mandibular hypoplasia and temporomandibular joint ankyloses (TMJa) have been reported to date. Patient Concerns and Diagnosis A 3-year-old female patient suffering from ALGS came to our hospital affected by unilateral mandibular hypoplasia and TMJa with severe limitation of mouth opening (maximal interincisal distance [MID] of 2 mm). Treatment A two-phase surgical management approach was undertaken based on computed tomography scans and the patient's age. The first phase involved mandibular distraction, followed by arthroplasty with amniotic human membrane placement. Outcomes After immediate post-surgery rehabilitation, the patient showed good mandibular function with no complications after 15 months. MID was 20 mm. Take-away Lessons This is a rare and interesting case with no previous literature reports. The use of amniotic membranes in surgical management adds further significance.
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Affiliation(s)
- Luca Guarda Nardini
- Unit of Oral and Maxillofacial Surgery, Ca’ Foncello Hospital, Treviso, Italy
| | - Matteo Val
- Unit of Oral and Maxillofacial Surgery, Ca’ Foncello Hospital, Treviso, Italy
- Department of Biomedical Technologies, School of Dentistry, University of Siena, Siena, Italy
| | - Anna Colonna
- Department of Biomedical Technologies, School of Dentistry, University of Siena, Siena, Italy
| | | | - Marco Ferrari
- Department of Biomedical Technologies, School of Dentistry, University of Siena, Siena, Italy
| | - Daniele Manfredini
- Department of Biomedical Technologies, School of Dentistry, University of Siena, Siena, Italy
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Herrmann S, Küchler EC, Reis CLB, Paddenberg E, Zbidat N, Mattos NHR, Schröder A, Proff P, Kirschneck C. Association of third molar agenesis and microdontia with genetic polymorphisms in vitamin-D-related genes. Ann Anat 2022; 244:151972. [PMID: 35738313 DOI: 10.1016/j.aanat.2022.151972] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2021] [Revised: 05/24/2022] [Accepted: 05/25/2022] [Indexed: 10/18/2022]
Abstract
The present study aimed to evaluate if functional genetic polymorphisms in vitamin-D-related genes are associated with third molar agenesis and third molar microdontia in German orthodontic patients. Pre-orthodontic and follow-up treatment records were evaluated for phenotype definition. Saliva samples were collected for DNA extraction. Eight potential functional genetic polymorphisms in VDR [rs731236 (TaqI), rs7975232 (ApaI), rs2228570 (FokI), and rs1544410 (BsmI)], CYP27B1 (rs4646536), CYP24A1 (rs927650), GC (rs4588), and SEC23A (rs8018720) were evaluated using real-time PCR. Comparison among the groups were performed (third molar anomaly vs. control; third molar agenesis vs. control; and third molar microdontia vs. control) with an alpha of 5%. A total of 164 patients were analyzed. Forty-nine (29.9%) patients had at least one third molar anomaly. In the haplotype analysis, genetic polymorphisms in VDR and CYP27B1 were associated with third molar anomalies (p<0.05). The G allele in rs8018720 (SEC23A) was more frequent in microdontia cases. In the genotype distribution analysis, rs8018720 in SEC23A was associated with third molar microdontia in the co-dominant (p=0.034; Prevalence Ratio [PR]=5.91, 95% Confidence Interval [CI]= 1.14-30.66) and in the recessive (p=0.038; PR=5.29; 95% CI= 1.09-25.65) models. In conclusion, vitamin D-related genes could be involved in third molar anomalies.
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Affiliation(s)
- Susann Herrmann
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
| | - Erika Calvano Küchler
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany; School of Dentistry, Tuiuti University from Paraná, Curitiba, Paraná, Brazil
| | - Caio Luiz Bitencourt Reis
- Department of Pediatric Dentistry, School of Dentistry of Ribeirão Preto, University of São Paulo. Av. do Café S/N, 14040-904 Ribeirão Preto, Brazil
| | - Eva Paddenberg
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
| | - Nermien Zbidat
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
| | | | - Agnes Schröder
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
| | - Peter Proff
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany
| | - Christian Kirschneck
- Department of Orthodontics, University of Regensburg. Franz-Josef-Strauss-Allee 11, 93053 Regensburg, Germany.
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