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@#Objective To explore the indications and clinical effects of simultaneous mandibular angleplasty on the unaffected side, performed using preformed titanium mesh combined with mandibular angle bone graft, for the treatment of hemifacial microsomia (HFM). Methods The study group included 11 patients between 2013 and 2021 with HFM who underwent simultaneous mandibular angle reduction of the unaffected side and augmentation of the affected side using resected bone scaffolds within the titanium mesh. A series of mandibular linear measurements such as ramus height(RH)and mandibular body length(BL), angular measurements, and asymmetry index combined with clinical photographs and postoperative complications were used to assess the therapeutic effect. Results Postoperatively, all patients were satisfied. Significant improvement in mandibular contour was shown, with no obvious complications. Postoperative data, such as BL and angular measurements, showed no statistically significant differences(P>0.05) between the affected and unaffected sides with improvement in symmetry, except for RH(P<0.05). The preoperative and postoperative asymmetry index were (17.11±4.56)% and (4.81±1.73)%, respectively, with significant improvement(P=0.002). Conclusion This approach provides satisfactory results and significantly improves facial asymmetries for unilateral HFM with hypertrophy of the contralateral mandibular angle.
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Abstract Introduction: goldenhar syndrome is a rare congenital syndrome that affects the craniofacial morphogenesis. It is a complex syndrome, with heterogeneous presentation which the diagnosis can still be performed in the intrauterine through morphological ultrasound. Description: a case report of a 4-year-old male patient diagnosed with Goldenhar syndrome, along with its clinical presentation, diagnostic investigation and follow-up. Discussion: the follow-up on these patients remains a challenge, since it can affect different systems and with different presentations. The earlier the diagnosis is performed, the greater the patient's chances of having a favorable prognosis with multidisciplinary stimulation. The objective of this article is to contribute to the medical literature, in order to assist in the diagnosis and management of future cases.
Resumo Introdução: a síndrome de Goldenhar é uma síndrome congênita rara que afeta a morfogênese craniofacial. Trata-se de uma síndrome complexa, de apresentação heterogênea, cujo diagnóstico pode ser realizado ainda intra-útero através do ultrassom morfológico. Descrição: relato de caso de um paciente do sexo masculino de quatro anos, com diagnóstico de síndrome de Goldenhar, sua apresentação clínica, a investigação diagnóstica e seguimento. Discussão: o acompanhamento desses pacientes continua sendo um desafio, já que pode acometer diversos sistemas e com apresentação diversa. O diagnóstico e a estimulação multiprofissional precoce, podem levar a maiores chances de um prognóstico favorável. O objetivo deste trabalho é contribuir para a literatura médica, de forma a auxiliar no diagnóstico e conduta perante futuros casos.
Subject(s)
Humans , Male , Child, Preschool , Prenatal Care , Prenatal Diagnosis , Goldenhar Syndrome/diagnosis , Goldenhar Syndrome/diagnostic imagingABSTRACT
OBJECTIVE@#To preliminarily verify the effectiveness of self-designed artificial condyle-mandibular distraction (AC-MD) complex in the treatment of Pruzansky type ⅡB and Ⅲ hemifacial microsomia (HFM) through model test.@*METHODS@#Five children with Pruzansky type ⅡB and Ⅲ HFM who were treated with mandibular distraction osteogenesis (MDO) between December 2016 and December 2021 were selected as the subjects. There were 3 boys and 2 girls wih an average age of 8.4 years (range, 6-10 years). Virtual surgery and model test of AC-MD complex were performed according to preoperative skull CT of children. The model was obtained by three-dimensional (3D) printing according to the children's CT data at a ratio of 1∶1. The occlusal guide plate was designed and 3D printed according to the children's toothpaste model. The results of the model test and the virtual surgery were matched in three dimensions to calculate the error of the residual condyle on the affected side, and the model test was matched with the actual skull CT after MDO to measure and compare the inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible.@*RESULTS@#The error of residual condyle was (1.07±0.78) mm. The inclination rotation of the mandible, the distance between the condylar of the healthy side and the residual condyle of the affected side, and the lengthening length of the mandible after 3D printing model test were significantly larger than those after MDO ( P<0.05).@*CONCLUSION@#In the model test, the implantation of AC-MD complex can immediately rotate the mandible to the horizontal position and improve facial symmetry, and the residual condyle segment can be guided close to the articular fossa or the preset pseudoarticular position of the skull base after operation.
Subject(s)
Male , Child , Female , Humans , Goldenhar Syndrome/surgery , Mandible/surgery , Osteogenesis, Distraction/methods , Printing, Three-Dimensional , Facial Asymmetry/surgeryABSTRACT
Hemifacial Microsomia (HFM) is a congenital anomaly involving embryological derivatives of the first and second branchial arches and characterized mainly by mandibular hypoplasia and unilateral or bilateral microtia; although, other facial structures may be affected. It may have long-term effects on psychological development and social well-being, due to unaesthetic facial appearance, functional disturbances and complex medical treatments
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Franceschetti-Goldenhar syndrome or Goldenhar syndrome, also known as facio-auricular-spectrum (FAV), first and second branchial arch syndrome, or Oculo-Auriculo-Vertebral (OAV) spectrum is a rare congenital malformation which encompasses various morphological and functional abnormalities. The incidence of Goldenhar syndrome has been reported between 1:3500 to 1:5600 children, with a male: female ratio of 3:23. The incidence is higher, about 1 in 1000 children with congenital deafness. The exact etiology is not known. However, it is possible that abnormal embryonic vascular supply, disrupted mesodermal migration or some other factors leads to defective formation of the brachial and vertebral system. Most of the cases have been sporadic. Autosomal dominant, autosomal recessive and multifactorial modes of inheritance have also been suggested. Chromosomal studies have not revealed any abnormality. Authors report a case of a neonate with hemifacial microsomia, bilateral cleft lip and cleft palate, right deformed pinna, right facial palsy, single umbilical artery and congenital heart disease.
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Hemifacial microsomia involves a broad spectrum of facial malformations of varying severity. Prenatal diagnosis rarely occurs and is usually related to severe cases or associated with other body malformations. We present a case of severe hemifacial microsomia with multiple gastrointestinal and cerebral malformations.
La macrosomía hemifacial involucra un amplio espectro de malformaciones faciales de diversa severidad. El diagnóstico prenatal es hecho excepcionalmente y habitualmente está relacionado a casos severos o con asociación a otras malformaciones corporales. Presentamos un caso de macrosomía hemifacial severa con malformaciones gastrointestinales múltiples y cerebrales.
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Objective @#To explore the clinical effect of simultaneous distraction osteogenesis of the maxilla and affected mandible combined with second-stage orthognathic surgery for the treatment of adult hemifacial microsomia to provide a reference for clinical practice.@*Methods@#Twelve adult patients with hemifacial microsomia who underwent simultaneous distraction osteogenesis and second-stage orthognathic surgery from 2006 to 2013 were enrolled. Distraction osteogenesis of the maxilla and malformed lateral mandible and second-stage orthognathic surgery were performed according to each patient’s specific conditions. The height ratio of the mandibular ramus of the affected side to the contralateral side, the shift distance of the chin point and the inclination angle of the screw plane were measured before and after the operation, and the therapeutic effect was evaluated with clinical photographs.@*Results @#No patients had obvious complications after the operation, the postoperative aesthetic effect was good, and the relationship between the facial shape and occlusion was significantly improved. The cephalometric measurement showed that the height ratio of the mandibular ramus of the affected side to the contralateral side was increased by 23.83% (t=11.658, P<0.001), the deviation distance of the chin was corrected, and the chin point moved back to the midline by 6.63 mm (t=13.042, P<0.001) on average, and the inclination angle of the occlusal plane was improved and returned to the horizontal plane by 8.83° (t=15.358, P<0.001) on average.@*Conclusion @#The application of simultaneous distraction osteogenesis of the maxilla and mandible combined with secondary orthognathic surgery for the treatment of adult hemifacial microsomia can obtain satisfactory clinical and aesthetic results and has clinical value.
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Objective@#To investigate the correlation between mandibular condyle volume and external ear volume in M2a type of hemifacial microsomia.@*Methods@#19 patients with M2a type of hemifacial microsomia diagnosed by CT scan in the Shanghai Ninth People′s Hospital from August 2017 to December 2018 were included, and the head CT data were obtained. At the same time, 19 healthy people were recruited as volunteers and obtain CT data of their heads as control. The Mimics 15.0 software was used for 3D reconstruction of CT data, measure the volume of mandibular condyle and external ear. SPSS 25.0 software was used to analyze the correlation between mandibular volume and external ear volume, as well as the difference between bilateral mandibular condyle volume (healthy side mandibular condyle volume-affected side mandibular condyle volume) and bilateral external ear volume difference (healthy side external ear volume-affected side external ear volume), and the correlation was obtained by spearman correlation coefficient analysis. P<0.05 indicates that the difference was statistically significant.@*Results@#The data of 19 cases of M2a type of hemifacial microsomia and 19 healthy volunteers were analyzed. The left and right volume of bilateral mandibular condyle in healthy volunteers was (1 309.23±420.63) mm3, (1 325.93±425.60) mm3(P=0.904), the left and right volume of external ear was (7 854.18±2 005.77) mm3, (7 862.63±1 994.02) mm3(P=0.990), bilateral development was synchronous, there was no statistical difference. There was a significant positive correlation between mandibular condyle volume and external ear volume in healthy volunteers (P=0.004, rs=0.772). The volume of healthy and affected mandibular condyle in the patients with M2a type of hemifacial microsomia was (1 160.89±549.07) mm3, (509.55±303.88) mm3 (P=0.006), and the volume of healthy and affected external ear was (7 418.19±2 434.93) mm3and (2 029.99±1 080.37) mm3 (P=0.007). The development is not synchronized in the mandibular condyle and external ear in M2a type of hemifacial microsomia, but the mandibular condyle volume is significantly positively correlated with the external ear volume(healthy side: P=0.008, rs=0.740; affected side: P=0.004, rs=0.709), and the affected part of the mandibular condyle and the external ear development (the volume difference between the mandibular condyle or the external ear volume) also has the same performance(P=0.006, rs=0.753).@*Conclusions@#There is a significant positive correlation between mandibular condyle volume and external ear volume in patients with M2a type of hemifacial microsomia.
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Objective@#To summarize the effect of simultaneous orthognathic surgery along with mandibular ramus reconstruction using costochondral graft, for adult M3 hemifacial macrosomia.@*Methods@#From November 2015 to October 2017, 5 adults diagnosed with M3 hemifacial macrosomia were treated. There were 3 males and 2 females, aged from 19 to 26 years. Le Fort Ⅰ osteotomy and SSRO with simultaneous mandibular ramus reconstruction using contralateral sixth or seventh costochondral graft was performed to correct the facial asymmetry and occluding relation. The data of clinical examination and CTs were collected at the time point of immediately postoperative, 1, 3, 6, 12 months after surgery. The facial symmetry, joint function, occlusion and 3D measurements in CT image reconstruction were analysed to evaluate the surgery outcome.@*Results@#The length of rib and costal cartilage ranged from 47 mm to 67 mm. All the costal cartilage grafts survived, and 4 patients got primary healing. All patients were followed for 2-13 months (with the mean follow-up of 8 months). The ratio of ramus length of affected side to normal side was over 80%. The occlusion was stable. The facial structures were satisfactory after 6 months.@*Conclusions@#Orthognathic surgery with simultaneous mandibular ramus reconstruction using costochondral graft is suitable for the adult severe hemifacial macrosomia, with satisfactory cosmetic and functional results. This method is easily performed with reliable graft survival rate, aesthetic facial structure and stable occlusion.
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Objective@#To discuss the establishment of three-dimensional mirror image plane ofhemifacial microsomia.@*Methods@#32 cases of hemifacial microsomia from Jan. 2006 to Jan. 2017 were collected in our hospital. CT scanning was performed for all patients. Then, the deformity characteristics were summarized through three steps: 3D reconstruction, mirror and discrepancy analysis.@*Results@#After statistical analysis, the stability of these three points was good. Clinical result showed that the patient′s appearance was greatly improved.@*Conclusions@#Using these three points to determine the mirror image plane is helpful to analyze the patient's facial features and facilitate the surgical design.
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Objective@#To investigate the influence of mandibular distraction osteogenesis on masticatory function by analyzing the maximal bite force before and after surgery.@*Methods@#From May 2014 to November 2015, 22 hemifacial microsomia cases, ageing from 6 to 10 years old, underwent mandibular distraction osteogenesis and their biting force data were collected. The maximal bite forces in incisor area, bilateral premolar area and molar area were measured before surgery and 1 year after surgery.@*Results@#The average maximum bite forces in the molar area of the affected side, premolar area of the affected side, incisor area, premolar area of the unaffected side, molar area of the unaffected side were (170.6±42.8)N, (141.9±34.9)N, (89.7±18.8)N, (169.3±29.0)N and (230.2± 56.4)N respectively before surgery. Statistics indicated that most patients had uneven distribution of bite force between the affected side and unaffected side before surgery. And they changed to (198.8±44.0)N, (151.8±31.1)N, (88.9±18.0) N, (167.8±26.1)N and (234.3±52.5)N respectively 1 year after surgery, showing that the bite force in the molar area of the affected side was significantly improved by the surgery(P<0.05). Regression analysis showed that the postoperative bite force distribution had a tendency to become more symmetrical in hemifacial microsomia patients.@*Conclusions@#Mandibular distraction osteogenesis can improve the bite force of the affected side in hemifacial microsomia patients and enhance the balance of masticatory in the long-term postoperatively.
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Objective To evaluate recurrence after mandibular distraction osteogenesis in adult patients with hemifacial microsomia using the digital three dimensional measurement.Methods Nine adult hemifacial microsomia patients were evaluated using the method of digital measurements with three dimensional computed tomography.Three observation points were studied:pre-operation (T0),post-operation (T1) and follow-up (T2).The length of ramus (H for normal side and h for affected side),the length of body (L for normal side and l for affected side) and distance between pogonion and facial midline (D) were measured and the recurrence rate (R) was calculated.Results Percentages of h/H at T0,T1,T2 were (58.19±12.06)％,(87.09±17.92)％and (81.13±30.57)％,which had statistical significance respectively.2.Percentage of l/L at T0,T1,T2 were (87.70±7.86)％,(90.69± 5.77) ％ and (90.11±5.62)％,respectively,which had statistical significance between T0 and T1.3.Lengths of D at T0,T1,T2 were (13.66±3.28) mm,(4.926±1.75) mm and (6.616±1.82) mm,respectively,which had statistical significance between T0,T1 and T0,T1.4.Average R was (22.80± 9.73) ％.Conclusions Varying degrees of recurrence do happen in both ramus and body of mandibular after distraction osteogenesis.While it is demonstrated that the mandible grows obviously after operation compared to that pre-operation,Meanwhile,the facial symmetry is also improved significiantly.
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ABSTRACT Hemifacial microsomia is the second congenital malformation in prevalence, after cleft lip and palate, and is described as a congenital alteration of the first and second branchial arches. As a condition of wide spectrum, its characteristics are expressed in many different ways and therefore treatments are usually individualized. This topic review discusses its etiology, classification, characteristics, and treatment with mandibular surgery.
RESUMEN La microsomía hemifacial corresponde a la segunda malformación congénita en prevalencia, luego de la fisura labiopalatina, y se describe como una alteración congénita del primer y el segundo arcos branquiales. Al ser una entidad en espectro, presenta características de expresión variable y por tanto los tratamientos son acordes a su individualidad. En esta revisión de tema se analizan su etiología, clasificaciones, características y tratamiento quirúrgico mandibular.
Subject(s)
Craniofacial Abnormalities , Facial AsymmetryABSTRACT
Goldenhar syndrome (GS) is a poly-malformation syndrome, also defined as oculo-auricolo-vertebral dysplasia with hemifacial microsomia. It is a rare congenital defect involving first and second branchial arches. The aetiology is not known. The most supported hypothesis is based on the abnormal embryonic vascular supply after mesodermal migration. Autosomal dominant, autosomal recessive and multifactorial modes of inheritance have been reported. We report the case of a female neonate affected by hemifacial microsomia and presence of pre-auricular tragi. Patients were subjected to computed tomography scan and MRI that revealed a mandibular unilateral hypoplasia without association of skeleton, brain and ocular alteration. The purpose of our study was to define the important role of the CT and MRI in the diagnosis of this poly-marformation syndrome.
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La reconstrucción de la articulación temporomandibular (ATM) es una de las cirugías más demandantes para el cirujano maxilofacial y su equipo. Sus objetivos no sólo incluyen la rehabilitación del complejo mecanismo normal de la articulación, sino también la restauración de la simetría facial, mejorar la oclusión y a la vez la masticación. Las funciones de la ATM pueden verse afectadas por diferentes en enfermedades: tumorales, infecciosas, traumáticas y displasias. Éstas son las que constituyen la mayor indicación para la reconstrucción de la ATM, siendo las congénitas asociadas a síndromes las más difíciles de tratar. La microsomía hemifacial es el segundo defecto craneofacial más común, después de la fisura labio palatina. Es un síndrome que, a diferencia de otros, presenta una gran diversidad de opciones de tratamiento, los cuales van a ser aplicados con un enfoque multidisciplinario, ya que los tratamientos pueden ir desde los conservadores o no quirúrgicos como los de ortopedia funcional hasta los quirúrgicos que comprenden la reconstrucción de la ATM y rama mandibular, los cuales en su mayoría se realizan con injertos libres costocondrales. Se presenta el caso de un paciente femenino de cinco años de edad, la cual acude referida al Hospital Central ®Dr. Ignacio Morones Prieto¼ con diagnóstico de microsomía hemifacial severa. Este trabajo se enfoca en describir el protocolo utilizado en nuestra institución para la reconstrucción mandibular de este síndrome desde edades tempranas, lo que beneficiará tanto la función articular como la estética facial.
Temporomandibular joint (TMJ) reconstruction is one of the most demanding surgical procedures performed by maxillofacial surgeons and their teams, whose aim is not only to rehabilitate the complex normal mechanism of the joint but also to restore facial symmetry, and to improve occlusion and chewing. TMJ functions can be affected by various types of maladies: tumors, infectious diseases, trauma, and dysplasias, which constitute the main indicators for TMJ reconstruction, being those associated with congenital syndromes the most difficult to treat. Hemi-facial microsomia is the second most common craniofacial defect after cleft lip and palate. Unlike other syndromes, it presents a wide range of treatment options, all involving a multidisciplinary approach as that they can range from conservative or nonsurgical procedures to functional orthopedics, and surgical procedures in which the TMJ and the mandibular ramus are reconstructed...
Subject(s)
Humans , Female , Child, Preschool , Temporomandibular Joint/surgery , Goldenhar Syndrome/surgery , Goldenhar Syndrome/rehabilitation , Bone Transplantation/methods , Age Factors , Clinical Protocols , Dental Service, Hospital , Mexico , Oral Surgical Procedures/methodsABSTRACT
Hemifacial microsomia (HFM) is the most common craniofacial anomaly after cleft lip and cleft palate; this deformity primarily involves the facial skeleton and ear, with either underdevelopment or absence of both components. In patients with HFM, the management of the asymmetries requires a series of treatment phases that focus on their interception and correction, such as distraction osteogenesis or functional appliance treatment during growth and presurgical orthodontic treatment followed by mandibular and maxillary surgery. Satisfactory results were obtained in a 9-year-old girl with HFM who was treated with distraction osteogenesis. At the age of 19, genioplasty and mandible body augmentation with a porous polyethylene implant (PPE, Medpor(R), Porex) was sequentially performed for the functional and esthetic reconstruction of the face. We report a case of HFM with a review of the literature.
Subject(s)
Child , Female , Humans , Cleft Lip , Cleft Palate , Congenital Abnormalities , Ear , Follow-Up Studies , Genioplasty , Goldenhar Syndrome , Mandible , Osteogenesis, Distraction , Polyethylene , SkeletonABSTRACT
BACKGROUND: The aim of this study was to present three-dimensional (3D) structural characteristics of the mandible in the hemifacial microsomia. The mandible has six distinct functional units, and its architecture is the sum of balanced growth of each functional unit and surrounding matrix. METHODS: In order to characterize the mandibular 3D architecture of hemifacial microsomia, we analyzed the mandibular functional units of four hemifacial microsomia patients using the 3D reconstructed computed tomography (CT) images. And we compared the functional unit size between affected and non-affected side. RESULTS: The length of condyle and angle showed significant differences between affected and non-affected sides. However, the length of mandibular body showed insignificant differences. The size differences between affected and non-affected side were observed at the condyle, angle, and body in descending order. CONCLUSIONS: This preliminary study suggests that the main etiopathogenic units are condyle and angle in the hemifacial microsomia mandible. Further investigation with the increased number of subjects will be helpful to establish treatment modality by etiopathogenic targeting of hemifacial microsomia.
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Humans , Goldenhar Syndrome , MandibleABSTRACT
The five major areas of concern in craniofacial surgery are the cleft lip and palate, hemifacial microsomia, craniosynostosis and its accompanying craniofacial syndromes, maxillofacial surgery, and breakthrough research on the each of these. Furlow's double opposing Z-plasty and 2-flap palatoplasty are often used to correct the cleft palate. Hereafter, the most appropriate surgical timing and methods must be determined through a prospective randomized control study. Currently, Millard's rotation advancement flap technique is generally used to correct the cleft lip. The repair of a cleft lip and especially a bilateral cleft lip is supplemented by presurgical orthodontics. Effort toward mastering the simultaneous repair of the nose continues. For hemifacial microsomia, distraction osteogenesis, orthognathic surgery, and fat injection is employed to correct facial asymmetry. Tissue engineering will soon be introduced as a treatment option. Currently, craniosynostosis is treated with methods such as total calvarial vault remodeling, endoscopic suturectomy, and distraction osteogenesis. In the future, a simpler and less invasive surgical technique must be developed. Maxillofacial (orthognathic) surgery generally refers to the bilateral sagittal split ramus osteotomy and LeFort I osteotomy. In the future, minimally invasive surgical techniques using endoscopes or robots will be introduced. Through the development of recombinant DNA technology, genetic research of craniofacial anomalies has identified many relevant genes. In the future, gene therapy will be plausible. Through advancements in tissue engineering, regeneration of tissue to correct congenital craniofacial deformities through autologous stem cells and scaffolds will be conceivable in the near future.
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Cleft Lip , Cleft Palate , Congenital Abnormalities , Craniosynostoses , DNA, Recombinant , Endoscopes , Facial Asymmetry , Genetic Research , Genetic Therapy , Korea , Nose , Orthodontics , Orthognathic Surgery , Osteogenesis, Distraction , Osteotomy , Osteotomy, Sagittal Split Ramus , Palate , Prospective Studies , Regeneration , Stem Cells , Surgery, Oral , Tissue EngineeringABSTRACT
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Humans , Congenital Abnormalities , Contracture , Dental Occlusion , Ear, External , Facial Asymmetry , Imidazoles , Jaw , Malocclusion , Mandible , Maxilla , Muscles , Nitro Compounds , Osteogenesis, Distraction , Osteotomy , Palatal Expansion Technique , Skeleton , TransplantsABSTRACT