Microsomia Research Articles

Efficacy of distraction osteogenesis of mandible in adult patients with hemifacial deformity

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. Key words: Adult; Hemifacial microsomia; Distraction osteogenesis; Three-dimensional measurement; Recrudescence

Sagittal split ramus osteotomy combined with intraoral distraction osteogenesis for hemifacial microsomia

To investigate the effect of sagittal split ramus osteotomy(SSRO) combined with distraction osteogenesis(DO) for hemifacial microsomia (HFM). From January 2013 to September 2015,7 cases of unilateral HFM were included in the study. The 3-dimensional reconstruction images were used for the operation design. The location of the germs and inferior alveolar nerve was marked to avoid injury. The distractor was fixed after SSRO. The distractor was distracted 2-4 mm intraoperatively and began enlongation 3-5 days after operation,1-2 mm every day until the pre-designed length attained. The patients were followed up for 3-D CT and measurement of the height of bilateral ramus. The enlongation length was 7 mm to 20 mm(mean,14.6 mm).The facial symmetry and occlusion plane was improved a lot. No complication occurred. The mean follow-up period was 22 months (range 17-32 months) with almost symmetrical appearance and occlusion function. SSRO combined with DO has a good therapeutic effect and low complication for patients with HFM. The distraction rate is relatively high with a short treatment period. The ramus width, even thickness, are not decreased, or even can be increased after DO.

Mandibular distraction combined with orthognathic techniques for the correction of adult hemifacial microsomia

To study the combination of Mandibular distraction and orthognathic techniques for the reconstruction of adult hemifacial microsomia. The three-dimensional CT reconstruction data was used with Mimics for preoperation design. The osteotomy location, distraction vector, distraction distance were decided before operation with a surgical guider. At the first stage, internal distractor was implanted after ostetomy through an extra-oral approach. The distraction begun 5-7 days after operation with a frequency of 1 mm/day. After distraction, the distractor was maintained for 3-6 months. At the second stage, the distractor was removed. Le Fort I osteotomy was performed in order to correct the cross-bite and improve the facial contour. Usually, bone graft was inserted into the gap after Le Fort I osteotomy. The genioplasty was also performed if necessary. 9 cases of adult hemifacial microsomia with severe mandibular deviation were treated. The facial asymmetry were improved greatly. 1 patient suffered an wound infection in the maxillary region after Le Fort I osteotomy and healed uneventfully with wound irrigation. Mandibular distraction combined with orthognathic surgery is an effective procedure for adult hemifacial microsomia with complicated mandibular hypoplasia.

The oculoauriculovertebral spectrum: Refining the estimate of birth prevalence.

The oculoauriculovertebral spectrum (OAVS) is a well-described pattern of congenital malformations primarily characterized by hemifacial microsomia and/or auricular dysplasia. However, the birth prevalence of OAVS is poorly characterized. Figures ranging from 1 in 150,000 through to 1 in 5,600 can be found in the literature - the latter figure being the most frequently quoted. This study aims to evaluate the reasons behind such discrepant figures and to refine the estimated birth prevalence of OAVS. Published reports on the incidence and prevalence of OAVS were systematically sought after. This evidence was critically reviewed. Data from appropriate studies was amalgamated to refine the estimate of the birth prevalence for OAVS. Two main reasons were identified why birth prevalence figures for OAVS are so highly discrepant: differing methods of case ascertainment and the lack of a formal definition for OAVS. This study refines the estimate of birth prevalence for OAVS to between 1 in 40,000 and 1 in 30,000. This number needs to be confirmed in a large well-designed prospective study using a formally agreed-upon definition for OAVS.

Correlation between birth weight and cesarean section rate of term singleton live-birth newborns in recent 10 years

Objective To investigate the trend in birth weight of term infants delivered in Miyun Hospital in recent ten years, and its relationship with cesarean section rate, to provide evidence for health care during pregnancy and lowering the cesarean section rate. Methods Singleton term live-birth newborns in Beijing Miyun Hospital from January 1, 2000 to December 31, 2009 were admitted into this study. Trends of birth weight, microsomia rate and cesarean section rate were analyzed. Results (1) There were 14 716 singleton term live-birth newborns delivered in the tenyear period, among which 7642 males and 7074 females with the proportion of male and female of 1.08 : 1. One thousand seven hundred and seventy-three (12.0％) microsomia were delivered, and the number of low birth weight infants was 180 (1.2％). (2) The birth weight of term infants was from 1200 g to 5850 g and the average birth weight was (3422±447) g. The average birth weight of each year increased, and the differences among each year showed statistical significance (F= 15. 337,P＜0.01). (3) The percentage of microsomia of each year increased, and the differences among each year also showed statistical significance (x2= 125. 285, P＜0. 01). (4) The cesarean section rate increased from 49.9％ in 2000 to 57.1％ in 2009 with statistical difference (x2 =180. 883, P＜0.01).(5) The cesarean section rate increased with the average birth weight of the singleton live-birth term infants in the 10 years from 52.2 ％ in those ＜2500 g to 85.4 ％ in those 4500 g～, and the differences had statistical significance (x2 =518. 519, P＜0. 01). Conclusions Cesarean section rate had close correlation with birth weight in term birth infants. It is suggested that prenatal care should be strengthened and nutrition guidance during pregnancy should be paid more attention. Key words: Birth weight ; Infant,newborn; Cesarean section

The application of the multi-slice CT based OMENS classification in diagnosing pediatric hemifacial microsomia

Objective To assess the value of the multi-slice CT based OMENS classification in diagnosing pediatric hemifacial microsomia. Methods Eleven patients including 9 boys and 2 girls with suspicion of hemifacial microsomia underwent MSCT. Multi-planar reformation (MPR), maximum intensity projection (MIP) and volume-rendered image (VR) reconstruction were performed at the workstation. The malformations of the facial structures were analyzed respectively with the OMENS classification system.Results On CT reconstruction images, hemifacial microsomia in all 11 patients was demonstrated clearly.Nine patients were found to have unilateral lesion, including 6 on the left side and 3 on the right side. Two patients presented bilateral involvement with various severity degree. The OMENS classification system can be perfectly used to analyze the deformities. Respectively, the orbit and zygomatic arch deformity involved 8 sides in 8 cases, the mandible deformity involved 10 sides in 9 cases, the auricular deformity involved 13 sides in 11 cases, and the soft tissue deformity involved 12 sides in 11 cases. Conclusion MSCT based OMENS classification system can make accurate and comprehensive evaluation of the morphological changes in hemifacial microsomia, which provide strong imaging evidence to clinical evaluation. Key words: Facial asymmetry; Tomography, X-ray computed; Child

The influence of environmental pollutants to children microsomia

儿童身高发育受到某些不良因素的影响会偏离正常的生长曲线.传统的观点认为遗传和营养是造成儿童身高发育迟缓的主要因素,但在环境污染日益严重的今天,不断有研究发现环境污染物质也是造成儿童身高发育迟缓的重要因素,环境污染物质通过各种途径和机制影响儿童身高发育并可能引起身高发育迟缓。

Pituitary hyperplasia in children during preadolescence: three cases report and review of Iiteratures

Objective Study the mechanism and treatment of pituitary hyperplasia in preadolescence of children. Method To report three pituitary hyperplasia in children during preadolescence and review literatures. Results All three children during preadolescence present mass in saddle area in MRI, present microsomia and chilly in clinical manifestation. The enlarged pituitary combined with both elevated thyrotrophin stimulating hormone(TSH) and prolactin(PRL) and low thyroid hormone value. All of three children during preadolescence was cured by thyroxine replacement therapy for about a half year. The pituitary size was normal in MRI examination and the clinical symptom had obviously improved and presented normal value in the endocrine examination. Conclusions The diagnosis of pituitary hyperplasia with primary hypothyroidism should be considered when the children daring preadolescence present with the enlargement of pituitary gland combined with both elevated TSH and PRL and low thyroid hormone value, and the pituitary hyperplasia only can be cured by thyroxine replacement therapy,and needn't make a operation. Key words: Hypothyroidism; Pituitary hyperplasia; Thyroxine

Surgical treatment of hemifacial microsomia

To summarize the methods and experiences in surgical treatment of hemifacial microsomia. Fifty-eight cases of hemifacial microsomia that have been treated by plastic and reconstructive surgery and followed-up in our department during last 20 years have been reviewed. Every patient's characteristic was assessed by physical examination, photography, craniofacial cephalometry before planned the method of surgery. Different surgical treatments were chosen according to the side and the structures involved in the abnormalities and the severity of hemifacial microsomia, and the cases were followed up since 3 months after the treatments. The follow-up lean of midline of the lower 1/3 face with that before treatment is compared. The degrees of patients' and surgeons' satisfactions with the treatments were evaluated respectively. The average angle of lean of midline of skeletal and soft tissue of the lower 1/3 face decreased 4.2 degrees and 2.9 degrees respectively after treatment. Fifty (82.6%) cases satisfied with the outcome of the surgical treatment and surgeons satisfied with 84.5% of the outcome of all the cases. Individual surgical treatment based on the side and the structures involved in the abnormalities can effectively correct facial asymmetry of hemifacial microsomia.

Goldenhar syndrome (oculoauriculovertebral dysplasia): report of one case.

Goldenhar syndrome, also known as oculoauriculovertebral dysplasia, is an uncommon condition, characterized by a combination of anomalies: epibulbar dermoids or lipodermoids, preauricular appendices, malformation of the ears, hemifacial microsomia, vertebral anomalies, and others. The etiology of this disease has remained unclear; factors including chromosomal abnormalities, maternal diabetes mellitus or drug use, and influence of environment during pregnancy have been proposed. Here, we describe a case of Goldenhar syndrome in a 1-day-old female newborn, who presented with right external ear atresia, left preauricular appendices, cleft-like extension of the right oral angle, mandibular hypoplasia and relatively small hands. The literature on Goldenhar syndrome is briefly reviewed.

Horizontal osteotomy combined with augmentation mentoplasty for serious chin microsomia

目的 介绍一种治疗严重小颏畸形,重塑颏部外形的有效方法.方法采用口内切口颏部水平截骨前移固定后,再联合利用在颏部置入合适假体的隆颏术,以进一步增加颏突度和颏长度,改善颏部形态.结果共治疗9例,术后患者颏部形态均得到明显改善,重塑的颏部外形符合美学标准,达到患者术前的预期要求.随访6个月至2年,效果稳定满意,无明显并发症出现.结论利用颏水平截骨联合置入假体的颏成形术治疗严重小颏畸形,使重塑的颏部形态达到了美学标准,面部轮廓比例协调,效果显著。

Diagnóstico e tratamento das assimetrias dentofaciais

A assimetria facial é uma característica humana comum, que muitas vezes não é notada pelo próprio paciente nem pelas pessoas com quem ele convive. Entretanto, ela se torna relevante quando o próprio paciente relata alguma alteração. A avaliação profissional deve ser requisitada para que a etiologia seja estabelecida através de diversos métodos de diagnóstico. A deformidade poderá decorrer de fatores genéticos, como encontrado em pacientes portadores de microssomia hemifacial, ou adquirida em traumas e patologias. O tratamento das assimetrias faciais tem como objetivo um resultado estético satisfatório e, principalmente, estabilidade oclusal e funcional. O plano de tratamento é elaborado de acordo com a etiologia, a severidade da deformidade, a idade do paciente e as áreas afetadas, corrigindo a deformidade instalada ou impedindo sua evolução. O propósito deste trabalho é revisar a literatura no que diz respeito à etiologia, métodos de diagnóstico e às formas de tratamento das assimetrias faciais, exemplificando com casos clínicos as diferentes formas de abordagem das assimetrias.

Common craniofacial anomalies: the facial dysostoses.

After studying this article, the participant should be able to: 1. Understand the etiology and pathogenesis of facial dysostosis syndromes. 2. Recognize and classify common facial dysostoses. 3. Understand the different management plans for the reconstruction of facial dysostoses. The wide spectrum of craniofacial malformations makes classification difficult. A simple classification system allows an overview of the current understanding of the etiology, assessment, and treatment of the most frequently encountered craniofacial anomalies. Facial dysostoses are reviewed on the basis of their diverse etiology, pathogenesis, anatomy, and treatment. Conditions discussed include craniofacial microsomia, Goldenhar syndrome, Treacher Collins syndrome, Nager syndrome, Binder syndrome, and Pierre Robin sequence. Approaches to the surgical management of these conditions are reviewed.

訂正

訂正

Dental and facial asymmetries: a review.

Asymmetry in the face and dentition is a naturally occurring phenomenon. In most cases facial asymmetry can only be detected by comparing homologous parts of the face. The etiology of asymmetry includes: a) Genetic or congenital malformations e.g. hemifacial microsomia and unilateral clefts of the lip and palate; b) Environmental factors, e.g. habits and trauma; c) Functional deviations, e.g. mandibular shifts as a result of tooth interferences. Dental asymmetries and a variety of functional deviations can be treated orthodontically. On the other hand, significant structural facial asymmetries are not easily amenable to orthodontic treatment. These problems may require orthopedic correction during the growth period and/or surgical management at a later point. Patient complaints and desires need to be addressed since they may vary from unrealistic expectations to a lack of concern even in the presence of large deviations. With mild dental, skeletal and soft tissue deviations the advisability of treatment should be carefully considered.

Mandibulofacial dysostosis or bilateral hemifacial microsomia with hearing loss, telecanthus, tetramelic postaxial hexadactyly, congenital hypotonia and lymphedema with joint hypermobility, and pigmentary dysplasia: A new syndrome?

Ce syndrome ne semble pas avoir ete decrit anterieurement. Il ne s'agit pas d'un syndrome d'aneuploidie: 1) les chromosomes sont normaux, 2) il n'existe pas d'anomalies mineures, 3) malgre la lenteur du developpement moteur (hypotonie congenitale) et cognitif (perte de l'audition) le nourrisson donne une impression «brillante»

Induced condylar growth in a patient with hemifacial microsomia.

Hemifacial microsomia is a congenital abnormality which results in progressive, three-dimensional facial asymmetry in the growing child. Functional appliance therapy was instituted in an 11 year, 7 month old boy with hemifacial microsomia in order to determine if symmetry could be re-established by promoting masticatory muscle function on the affected side, thereby stimulating bone growth in the affected condyle over and above what would occur without any treatment intervention. Removable functional appliance therapy, and later, unilateral Herbst appliance therapy, produced a dramatic change in the condylar growth on the affected side. Herbst therapy has the advantage over a removable functional appliance in that patient acceptance is much greater. Lack of patient compliance may be the primary cause of the variable results obtained with functional appliances in hemifacial microsomia cases as reported in the literature.

The corrective treatment of the skeletal asymmetry in hemifacial microsomia.

The corrective treatment of the skeletal asymmetry in hemifacial microsomia.