No abstract available.
Goldenhar Syndrome*

No abstract available.
Goldenhar Syndrome*

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.
Goldenhar Syndrome* ; Humans ; Mandible

Oculo-auriculo-vertebral spectrum (OAVS), also known as Goldenhar syndrome, hemifacial microsomia, oculo-auriculo-vertebral dysplasia and facio-auriculo-vertebral spectrum, is a developmental disorder associated with the first and second branchial arches. Most cases are sporadic, while some familial instances observed suggested that the etiology of OAVS heterogeneous. In this review, we summarize the OAVS epidemiology, classification and mainlyheterogeneous etiology.
Goldenhar Syndrome ; pathology ; Humans

No abstract available.
Autografts ; Goldenhar Syndrome

Mandibular distraction osteogenesis has gained popularity as a surgical technique for hemifacial microsomia. Although there was the general acceptance of mandibular distraction osteogenesis by surgeons, the standardization of techniques among surgeons has been limited due to short period of its history. So, many surgeons having make an effort for suggesting standard protocol, based on various clinical experiences. We found that corrected mandibular asymmetry of hemifacial microsomia by rapid distraction osteogensis has a tendency to relapse, in spite of external fixator during consolidation period. On this, we studied that which factors have influences on relapse of mandibular asymmetry during consolidation period. As a result, there were significant differences of the relapse during consolidation period in proportion to mandibular hypoplasia and the vertical distraction distance. But there was no significant difference of the relapse among the duration of the consolidation period groups. And there was significant difference of the vertical distraction distance in proportion to mandibular hypoplasia. The amount of the relapse during consolidation period has correlation with the severity of the mandibular hypoplasia. In conclusion, the severity of the mandibular hypoplasia is the important forecast index of relapse during consolidation period.
External Fixators ; Goldenhar Syndrome ; Osteogenesis, Distraction* ; Recurrence*

Hemifacial microsomia is a common congenital craniofacial deformity involving bone and soft-tissue. Mandibular hypoplasia is the most obvious skeletal manifestation of hemifacial microsomia. In the past, complete realignment of the skeleton was preferred to soft-tissue correction, which was clearly second choice. However, in this study, simultaneous correction of bone and soft tissue deformities were equally important in treatment of hemifacial microsomia. One-stage and simultaneous bone and soft tissue reconstruction is possible and staged operations of the skeleton and soft tissue are no longer necessary, except in special cases. Even in children and adolescents, good results and normal growth potential can be achieved with simultaneous correction of bone and soft tissue.
Adolescent ; Child ; Congenital Abnormalities* ; Goldenhar Syndrome ; Humans ; Skeleton

Bone-anchored auricular prosthesis have been proved that was another apporach to the treatment of the defective external ear. A 19years old woman with hemifacial microsomia was treated with osseointegrated implants for the support of craniofacial prosthesis. 3 implants were placed in temporal region and craniofacial prosthesis was retained with telescopic magnet retention system. The literatures and surgical technique are reviewed and our experience is presented.
Ear, External* ; Female ; Goldenhar Syndrome ; Humans ; Prostheses and Implants*

Hemifacial microsomia is a condition most frequently associated with facial asymmetry. Authors have experienced eighteen patients with mild hemifacial microsomia who were classified as type IA and IB. For the correction of mandibular asymmetry, mandibular augmentation was performed on the mandibular angle of the affected side using mandibule shaped porous high-density polyethylene(Medpor(R)) in thirteen patients. Reduction mandibuloplasty was performed on the mandibular angle of the normal side using lateral cortectomy in four patients, and genioplasties were used in 2 patients. Osseous genioplasty(n=10), buccal fat removal(n=4), augmentation rhinoplasty(n=2), and onlay bone graft(n=1) as ancillary procedures were simultaneously executed. Except for 4 patients in whom the implants were removed due to exposure and infection, all other patients were satisfied with a more symmetric contour of their face. The results were clinically evaluated through ordinary scale method and photogrammetric analysis. The mean score was rated 'good' as 12.5 points, and the mean bigonial distance index and the mean gonion-midsagittal distance index were 105.20%(p=0.035) and 100.65%(p=0.368), respectively, which meant a more symmetry of the lower face. In minor asymmetry of the mandible in cases of hemifacial microsomia, augmentation with cautions of the affected side, reduction with lateral cortectomy of the mandible in the non-affected side, and an even osseous genioplasty can provide the patient with a more symmetric lower face.
Facial Asymmetry ; Genioplasty* ; Goldenhar Syndrome* ; Humans ; Inlays ; Mandible ; Polyethylene*

Goldenhar's syndrome is a rare form of a hemifacial microsomia in which the facial and vertebral anomalies are frequently associated with cardiac, pulmonary and renal defects. Infants with Goldenhar's syndrome commonly have an airway that is difficulty to manage. A difficult tracheal intubation may be due to a combination of mandibular hypoplasia, macrognathia, palatal defects and vertebral anomalies. We report the successful anesthetic management of a 10-month-old girl with Goldenhar's syndrome for the excision of conjunctival lipodermoid. The use of remifentanil followed with an anesthetic induction dose of propofol provides adequate conditions for tracheal intubation without the use of neuromuscluar blocking agents in a patient with Goldenhar's syndrome.
Airway Management* ; Female ; Goldenhar Syndrome ; Humans ; Infant ; Intubation ; Propofol