半侧颜面发育不全畸形的综合治疗

目的探讨治疗半侧颜面发育不全畸形的各种正颌外科与其他手术方法组合,为提高治疗这种畸形的效果提供临床参考。方法根据11例半侧颜面发育不全患者的畸形程度,分别采用上颌LeFortⅠ型截骨术,下颌升支矢状劈开截骨术,下颌角成形术和颏成形术,其中有4例患者采用了腓骨肌皮瓣修复,3例患者采用了肩胛游离皮瓣修复,2例患者采用了腹部游离真皮脂肪瓣修复。并就该类患者颌面结构特征、手术方式的选择及疗效进行了回顾性分析。结果11例患者均顺利完成治疗,游离皮瓣均获得成功,所有患者面部偏斜畸形及咬合关系得到明显改善,5例患者咬合平面完全摆正,仍有6例患者咬合平面角维持在1°～4°。无长期不良并发症发生,随访期间无明显复发,患者对治疗结果感到非常满意。结论①畸形同时具有骨性畸形和软组织畸形,根据畸形的不同类型运用相应的手术方案,配合术前术后的正畸治疗,可获得咬合关系和容貌均满意的临床效果。②游离皮瓣的应用扩大了手术的适应证,使严重发育不全畸形得以较好矫正。     

Demographic and reproductive factors associated with hemifacial microsomia.

OBJECTIVE: To identify demographic and reproductive risk factors for hemifacial microsomia in offspring. DESIGN: In a case-control study, maternal interviews were conducted within 3 years after delivery. Cases with hemifacial microsomia were ascertained from craniofacial centers in 26 cities in the United States and Canada. Controls were patients of the cases' pediatricians. Two hundred thirty-nine cases were compared with 854 controls. Odds ratios for various infant and maternal factors were estimated. RESULTS: Cases had lower birth weights, were more often male or a twin, and had more relatives with craniofacial malformations or hearing loss than controls. Case mothers had lower family incomes, had a lower body mass index, had more vaginal bleeding in the second trimester, and were more likely to have had a spontaneous abortion in a previous pregnancy. CONCLUSIONS: Nonmodifiable factors (age and parity) were not associated with hemifacial microsomia risk. Factors that are related to poverty (low family income, late recognition of pregnancy, and low body mass index) are associated with an increase in risk. High risk estimates for multiple pregnancies and second-trimester vaginal bleeding suggest a vascular etiology.     

An association between hemifacial microsomia and facial clefting.

PURPOSE: Hemifacial microsomia (HFM) is a relatively uncommon malformation, a far second to cleft lip/palate (CL/P). Transverse oral cleft ("macrostomia") is known to be associated with HFM, but there are also reports of concurrent CL/P. We undertook a retrospective study of patients with HFM to document the prevalence of facial clefting. The hypothesis is that the coexistence of facial clefts and HFM suggests a common pathogenic mechanism. MATERIALS AND METHODS: Records of 198 HFM patients from the database of the Craniofacial Centre at Boston Children's Hospital were reviewed. The age, gender, predominant side of HFM, OMENS classification, and presence, size, and type of cleft (CL/P, transverse oral cleft, or oro-ocular cleft) were documented. We performed chi 2 , t test, and Fishers exact analyses to evaluate the association of facial clefting with HFM. RESULTS: There were 100 males and 98 females in the HFM study group, with a mean age of 15.3 years (range, 1 to 50 years); 90 were right-sided (45%), 79 were left-sided (40%), and 29 were bilateral (15%). Twenty patients had a CL/P (10%), and of these 15 had unilateral CL/P (UCL/P), equally distributed between right and left sides, and 5 had bilateral CL/P (BCL/P). There were 9 males and 11 females with combined HFM and CL/P. Cleft of the oral commissure (macrostomia) was present in 46 (23.0%) of the HFM patients with a left-to-right-to-bilateral ratio of 5:6:1. There was a significant association between the predominant side of HFM and the side of CL/P ( P < .001) and macrostomia ( P 相似文献   

半侧颜面发育不全的临床研究进展

半侧颜面发育不全（HFM）是来源于第一、第二鳃弓或与之有密切联系的颅颌面组织结构在胚胎期出现发育异常,继而形成的受累广泛的先天性畸形。病变主要累及眼、耳、下颌骨、颅神经和相关软组织,同时伴有颅颌面以外广泛畸形。病因及发病机制尚不明确,目前有许多纠正畸形的方法。本文对HFM的病因机制、临床表现、分类及治疗方法等进行综述。     

半侧颜面短小症牵张成骨治疗进展

半侧颜面短小症是仅次于唇腭裂的最常见的先天性颌面部畸形,临床表现多样,累及上下颌骨、颧骨、颞骨等多个颅颌面骨骼结构,同时伴有颜面软组织发育不全,常合并附耳、耳郭畸形等。牵张成骨技术治疗半侧颜面短小症,延长下颌骨,改善面部不对称畸形,疗效良好。该文就半侧颜面短小症牵张成骨治疗的进展进行综述。     

Intraoral distraction osteogenesis of the mandible in hemifacial microsomia.

PURPOSE: Lengthening of the mandible by distraction osteogenesis is the preferred method for treatment of hemifacial microsomia in children. Use of an intraoral distraction technique and horizontal oblique ramus osteotomy in such patients is presented. PATIENTS AND METHODS: Mandibular ramus lengthening was performed in 11 patients aged 6 to 12 years with hemifacial microsomia. During the age of mixed dentition in hemifacial microsomia patients with a hypoplastic mandible, the unerupted molars buds are located high in the retromolar region and are in danger of being damaged by the osteotomy. Therefore, an intraoral approach exposing the mandibular ramus and angle was performed, and a horizontal oblique ramus osteotomy was made, preserving the inferior alveolar nerve. An intraoral device was placed along the ramus, and distraction was started on the third postoperative day at the rate of 1 mm/d and continued for 2 to 3 weeks or as long as necessary. The device was maintained for retention an additional 6 weeks and was then removed. RESULTS: Clinically, the face became more symmetric. The postdistraction posteroanterior cephalometric radiographs demonstrated elongation of the affected ramus and improvement in facial symmetry. CONCLUSIONS: The advantages of this method are that it allows device placement along the ramus, permitting the ramus elongation necessary in treatment of hemifacial microsomia, that it prevents damage to the tooth buds which, during the age of mixed dentition, are in a higher position in the retromolar area, and that it prevents injury of the inferior alveolar nerve.     

Evaluation of temporomandibular joint in patients with hemifacial microsomia.

OBJECTIVE: The purpose of this study was to elucidate positional relationships between temporomandibular joint (TMJ) components, including the articular discs, using magnetic resonance imaging (MRI) in patients with hemifacial microsomia (HFM). SUBJECTS AND METHODS: Twenty TMJs in 10 patients with HFM were examined at closed- and open-mouth positions using an MRI scanner. The condyle-fossa and disc-condyle relationships, disc configuration at the closed-mouth position, and the reduction of the disc at the open-mouth position were evaluated. RESULTS: On the unaffected side, the condyle-fossa and disc-condyle relationships appeared fairly normal at the closed mouth position. The disc-condyle relationship at the open-mouth position was also normal. The TMJ disc showed normal biconcave configurations at both closed- and open-mouth positions. On the affected side, there was considerable variation in the state of the TMJ. At the closed-mouth position, 5 of the 10 patients revealed fairly normal disc-condyle relationships, one patient showed anterior displacement of the disc, and four patients had no disc. Two patients appeared biconcave, three patients appeared biplanar, and one patient was hemiconvex. At the open-mouth position, the condyle and disc moved in harmony in five patients with normal disc-condyle relationships, but the disc was reduced in a patients with anterior disc displacement. The degree of the TMJ disc dysplasia did not necessarily correspond with the degree of mandibular dysplasia. CONCLUSIONS: The present study contributes to an improved understanding of TMJ pathology in patients with HFM. The results suggest that, in HFM patients, the examination of the TMJ using MRI is helpful for determining treatment procedures in mandibular distraction osteogenesis.     

Microvascular groin flaps in the treatment of hemifacial microsomia.

The use of free microvascular groin flaps, both myocutaneous and osteocutaneous, is described in five patients with hemifacial microsomia. Flaps were designed according to the degree of soft tissue and skeletal abnormality. Long-term results are described. Though the majority of patients with hemifacial microsomia do not require free flap grafts, the procedure does present as a viable approach to severe tissue deficiency with excellent results.     

Application of custom-made TMJ prosthesis in hemifacial microsomia

A case of hemifacial microsomia in an adult female is presented. The ascending ramus and condyle was primarily reconstructed using an iliac crest free bone graft. Following resorption of the bone graft, a custom-made total TMJ device (TMJ Concepts, USA) was used to correct the deformity. The alternative available reconstructive techniques are discussed and the advantages and disadvantages of the method selected.     

Familial hemifacial microsomia due to autosomal dominant inheritance. Case reports

Hemifacial microsomia is a rare dentofacial anomaly which is regarded as a separate entity to Goldenhar syndrome and primarily affects the structures of the first branchial arch. It has a heterogeneous aetiology and tends to occur sporadically, though positive family histories have been reported. This paper reports on individuals in two generations of a family that has overlapping features of hemifacial micro-somia and Goldenhar syndrome segregating as an autosomal dominant condition.     

Psychosocial adjustment in twin pairs with and without hemifacial microsomia.

OBJECTIVE: To compare the psychosocial adjustment of children with and without hemifacial microsomia (HFM). DESIGN: This cross-sectional pilot study investigated psychosocial adjustment of twin pairs in which one twin was affected with HFM and the other was unimpaired. Data were analyzed using paired t tests. SETTING: The dental medicine clinic of a large urban children's hospital, serving a multistate area. PARTICIPANTS: Participants were six twin pairs aged 9 to 15 years and their parents. Data were also collected from classroom teachers. MAIN OUTCOME MEASURES: Outcome measures included: the Child Behavior Checklist (CBCL), the Teacher Report Form (TRF), the Coopersmith Self-Esteem Inventory (SEI), and a structured self-concept interview (the Self-Interview). RESULTS: Children with HFM had significantly higher CBCL and TRF total behavior problem scores and lower SEI general self-esteem scores than their unimpaired twins. No differences were found in CBCL competence scores. Interview data revealed concerns among the HFM group related to appearance, negative social responses, and fear of hospital procedures. Children also reported positive aspects of coping with HFM. CONCLUSIONS: The results of this study suggest that, similar to other craniofacial conditions, HFM is associated with an elevated risk for childhood psychosocial difficulties. Replication of specific findings with a larger sample is needed. Multicenter, collaborative studies are a crucial next step for this field.     

Orthopedically induced condylar growth in a patient with hemifacial microsomia.

OBJECTIVE: Hemifacial microsomia is a congenital abnormality that causes three-dimensional facial asymmetry, affection of the dental occlusion, and failure of growth of the midface in the growing child. This report outlines orthopedically induced condylar growth in a patient with hemifacial microsomia. PATIENT: A 7-year-old girl with hemifacial microsomia, complete absence of the left mandibular condyle, and severe facial asymmetry was treated orthopedically in an early stage by means of a functional appliance. Functional therapy was instituted to stretch the deficient soft tissues to guide and promote skeletal growth and stimulate the affected areas. The treatment was completed with fixed appliances. RESULTS: The effect of the therapy was an excessive change in condylar growth in the affected side. Facial asymmetry was corrected and a symmetric mouth opening was established. CONCLUSIONS: The treatment of patients with hemifacial microsomia should be initiated early enough so that the stimulus could in some degree normalize the deficient tissues and induce bone apposition, and in some cases surgical intervention could be avoided.     

Recipient vessels for microvascular transplants in patients with hemifacial microsomia.

Hemifacial microsomia is a developmental abnormality involving structures derived from the first and second branchial arches. Microvascular transplants are increasingly being used to improve facial contour in patients with this condition. We have reviewed 9 patients with this abnormality to determine which recipient vessels have been used. In 6 patients, the facial vessels were located and used for flap revascularization. In 3 patients, the facial vessels could not be identified intraoperatively, and the occipital artery and a branch of the external jugular vein were used as recipient vessels. In this series of hemifacial microsomia patients, therefore, the facial vessels could not be located in 3 patients. We recommend that surgeons performing microsurgical transplants in cases of hemifacial microsomia be prepared to explore the external carotid and external jugular systems for recipient vessels.     

Volume and shape of masticatory muscles in patients with hemifacial microsomia.

OBJECTIVE: To test the following hypotheses in hemifacial microsomia (HFM): (1) the volumes of the masseter, lateral and medial pterygoid, and temporal muscles are reduced on the affected versus unaffected side; (2) significant differences exist between the degrees of right-left disproportion in these four masticatory muscles; (3) circumferential shapes of the masticatory muscles are more irregular on the affected versus unaffected side; and (4) the degree of masticatory muscle right-left disproportion can be judged by the degree of ear, mandibular, or dental anomalies. SUBJECTS AND METHODS: Ten preadolescent patients with HFM were studied using facial photographs, dental casts, cephalometric and panoramic radiographs, and helical computed tomography scanning and three-dimensional reconstruction technique. Volumes of masseter, lateral and medial pterygoid, and temporal muscles on both sides were measured. Muscle volume disproportion was expressed as the affected/unaffected ratio. Muscle circumferential irregularity was expressed as the ratio between the total circumferential length and corresponding cross-sectional area. RESULTS: Masticatory muscle volumes were significantly smaller on the affected versus unaffected side. No significant differences were observed between the degrees of disproportion of the four masticatory muscles examined. Circumferential shapes of masticatory muscles were significantly more irregular on the affected versus unaffected side. There were no significant relationships of the degree of ear, mandibular, or dental anomalies in relation to masticatory muscle disproportion. CONCLUSIONS: In HFM the masseter, lateral and medial pterygoid, and temporal muscles all have a significantly smaller volume on the affected versus unaffected side, and specific muscles were not severely affected in the present subjects. Furthermore, all four muscles showed a significantly more irregular shape on the affected versus unaffected side. Finally, the severity of masticatory muscle disproportion can probably not be judged by the degree of ear, mandibular, and dental anomalies in preadolescent patients with HFM.