Klippel-feil: A syndrome in the occipital-cervical spine field and its dentofacial manifestations

Klippel-Feil syndrome (KFS) is defined by congenital cervical vertebral spine fusion and is seen with a wide spectrum of dental manifestations and craniofacial profiles. Previous studies on lateral cephalograms have documented an association between fusion of the cervical vertebrae and deviations in the craniofacial profile in non-syndromic patients with severe malocclusion. To our knowledge, no previous studies have described the craniofacial profile including the cranial base of KFS patients on lateral cephalograms. Therefore KFS and its craniofacial and dental manifestations were described according to existing literature and additionally the craniofacial profile and cranial base was analysed on lateral cephalograms of two patients with KFS. According to the literature the dental manifestations of KFS-patients included oligodontia, overjet, cross bite, open bite and deep bite. The craniofacial profile was clinically described as reduced lower facial height, midfacial hypoplasia, and mandibular prognathia. The analyses of the two lateral cephalograms showed increased mandibular inclination, increased vertical jaw-relationship, increased jaw angle and maxillary retrognathia. The cranial base was normal in both cases. The sagittal jaw relationship and mandibular prognathia varied between the two cases. The literature review and the analyses of the two lateral cephalograms have shown that deviations in the occipital and cervical spine field as KFS were associated with deviations in the teeth and craniofacial profile.     

Dental team management for a patient with Klippel–Feil syndrome: Case report

Klippel–Feil syndrome (KFS) is a rare congenital abnormality characterized by a short neck, a low posterior hairline, and limited head movement. Occasionally, patients with KFS may also show signs of deafness, intellectual disability, cardiac malformation, palpebral ptosis, facial nerve paralysis, cleft palate, and scoliosis. Although some researchers have documented this syndrome, scant attention has been paid to craniomaxillofacial manifestations and dental treatment of patients with KFS. The objective of this case report was to describe the planning and execution of dental treatment for a 10-year-old male patient with KFS.     

A case of klippel-feil and turner syndromes

The purpose of this paper was to describe the clinical case of a 12-year-old female patient with Klippel-Feil syndrome (KFS) combined with Turner syndrome (TS) and a submucous cleft palate (CP). The patient's general appearance was characterized by KFS, a clinical triad consisting of congenital fusion of at least 2 of 7 cervical vertebrae with a short neck, limited head motion, and a low posterior hairline. Three-dimensional images from cone-beam computed tomography (CBCT) revealed cervical vertebrae anomalies and submucous CP. It was reported that the patient had TS and has been administered growth hormone (GH) therapy. Due to a skeletal Class III pattern with a steep mandibular plane angle, facial asymmetry, and fused cervical vertebrae, GH's effects on the craniofacial complex should be considered before orthopedic/orthodontic treatment is started.     

Type III Klippel–Feil syndrome: case report and review of associated craniofacial anomalies

Klippel–Feil syndrome (KFS) is a complex syndrome of osseous and visceral anomalies that include the classical clinical triad of short neck, limitation of head and neck movements and low posterior hairline. It may also be associated with anomalies of the genitourinary, musculoskeletal, neurologic and cardiac systems. We report a case of type III KFS with associated rib anomalies such as cervical rib, fusion and bifid ribs, scoliosis and fused crossed renal ectopia. The aim of this paper was to summarize all craniofacial anomalies that occur in association with KFS, so that clinicians would be aware of them during diagnosis and treatment planning.     

Congenital mandibular hypoplasia: analysis and classification

Mandibular hypoplasia is a frequently encountered craniofacial difference and can be classified into three groups: congenital, developmental, and acquired. The focus of this article is on the congenital group, the majority of which is associated with syndromes. There have been numerous publications on patients with syndromic congenital mandibular hypoplasia; however, there has been no investigation and differentiation of the "nonsyndromic" patients. The purpose of this study was to analyze this subgroup of patients with nonsyndromic congenital mandibular hypoplasia to determine incidence, clinical presentation, and treatment.A retrospective analysis of all children treated for congenital mandibular hypoplasia at the Children's Hospital of Philadelphia between 1975 and 2003 was performed. Two hundred sixty-six patients were identified during this 27-year period. Of these 266 patients, 148 presented with oculo-auriculo-vertebral (OAV) spectrum, 52 with mandibulofacial dysostosis, 31 with Pierre Robin sequence, and 17 with miscellaneous syndromes. The remaining 18 patients were identified as having congenital mandibular hypoplasia without any known syndrome.Of the 18 patients with nonsyndromic congenital mandibular hypoplasia, 17 had primary bilateral growth anomalies and 1 had a primary unilateral growth disturbance resulting in bilateral deformity. Seven patients were products of a complicated pregnancy, 10 patients required tracheotomy or prolonged intubations, and 7 required gastric tube feedings. Associated anomalies included temporomandibular joint ankylosis in five patients, aglossia/microglossia in three patients, and rare craniofacial clefts in three patients. The average number of procedures required to treat the mandibular deformity for each patient was six.Although mandibular hypoplasia is a common craniofacial anomaly, patients manifesting nonsyndromic congenital mandibular hypoplasia are a rare subgroup. Case reports illustrating the range of mandibular deformities are presented.     

Feeding and swallowing outcomes following mandibular distraction osteogenesis: an analysis of 22 non-isolated paediatric cases

Patients with mandibular hypoplasia and upper airway obstruction are at an increased risk of feeding and swallowing difficulties. Little has been described regarding these outcomes following mandibular distraction. The aim of this study was to evaluate the effect of mandibular distraction on feeding and swallowing function. A retrospective study was performed on 22 patients with non-isolated mandibular hypoplasia and severe upper airway obstruction treated with mandibular distraction. Median age at first mandibular distraction was 3.1 years (interquartile range 2.3–6.0 years) and the median follow-up time was 3.5 years (interquartile range 2.0–9.4 years). Prior to mandibular distraction, feeding difficulties were present in 18 patients. Swallowing difficulties were present in 20 patients, all of whom had problems in the oral phase of swallowing, while 11 patients had additional problems in the pharyngeal phase. Following mandibular distraction, at the time of follow-up, feeding difficulties persisted in 13 patients. Swallowing difficulties in the oral phase remained present in all 20 patients, while pharyngeal phase problems persisted in seven patients. In conclusion, feeding and swallowing difficulties are highly prevalent in non-isolated patients and often persist following mandibular distraction. Moreover, these can be the reason that decannulation cannot be accomplished. Hence, awareness and close follow-up by a specialized speech therapist is of paramount importance.     

骨性安氏Ⅲ类错牙合伴偏颌患者下颌骨偏斜程度与下颌骨形态和肌功能不对称指数相关性研究

目的    探讨骨性安氏Ⅲ类错牙合伴偏颌患者下颌骨偏斜程度与下颌骨形态和肌功能不对称指数（asymmetry index,AI）的相关关系。方法    选择2007年 1 月至2012 年12 月中国医科大学附属口腔医院正畸科收治的骨性安氏Ⅲ类错牙合伴偏颌患者30例,通过测量和计算得到其下颌骨形态学各指标的AI值和相关肌肉在下颌骨不同运动状态时的肌电AI值,采用 Pearson相关分析法分析下颌骨偏斜程度与上述AI值之间的相关关系。结果    下颌骨形态学指标中,髁状突长度AI值和下颌升支后缘长度AI值与下颌骨偏斜的距离呈显著的正相关关系（相关系数r分别为0.392和0.366,P值分别为0.031和0.043）。相关肌肉中,颞肌前束在下颌前伸牙合位和牙尖交错位紧咬时的肌电AI值和胸锁乳突肌在息止牙合位的肌电AI值均与下颌骨偏斜距离的具有显著的正相关关系（相关系数r分别为0.379、0.351、0.339,P值分别为0.034、0.043、0.040）。结论    骨性安氏Ⅲ类错牙合伴偏颌患者,既存在下颌骨形态不对称,也存在相关肌肉功能活动的不对称。     

下切牙先天缺失与牙颌畸形关系的分析研究

目的：探讨下恒切牙先天缺失情况及其与牙颌畸形的关系以及下尖牙近远中径的变化。方法：从962名门诊正畸病人中选取下颌恒切牙缺失的病例27名，并测量下前牙牙冠的近远中径。结果：下颌恒切牙先天缺失96％是中切牙，92％伴有前牙深覆He，下尖牙无显著性增大。结论：下颌中切牙先天缺失与前牙深覆He的形成有密切关系，下尖牙无代偿性增大。     

���԰��Ϣ�������ϰ�ƫ򢻼������ƫб�̶���������̬�ͼ����ܲ��Գ�ָ��������о�

?? Objective    To evaluate the correlationship between mandibular asymmetry and the Asymmetry Index ??AI?? of mandibular profile and muscle functions in patients with skeletal Angle Class?? malocclusion combined with mandibular asymmetry. Methods    Totally 30 patients with both mandibular prognathism and asymmetry from 2007 to 2012 in the Department of Orthodontics??Hospital of Stomatology?? China Medical University?? were included in this study. We measured the distance and the angle of different parts of mandible which could indicate the profile of the mandible to some extent and electromyographic parameters for masticatory activities?? and calculated Asymmetry Index of them. The Pearson's correlation analysis was used to find the correlationship between mandibular asymmetry and this Asymmetry Index. Results    The condyle length and the ramal height had a significant  positive correlation with mandibular asymmetry?? meanwhile?? to the masticatory activity?? the same was shown for anterior Temporalis in protrusive occlusion and ICP biting and sternocleidomastoid muscle in resting position. Conclusion    There are asymmetries of both mandibular profile and functional activity in patients with skeletal Angle Class?? malocclusion combined with mandibular asymmetry.     

Biodegradable osteosynthesis in mandibular advancement: a pilot study

In this pilot study four patients are presented who underwent bilateral sagittal split osteotomies of the mandibular ramus for mandibular advancement. Biodegradable osteosyntheses were used for internal fixation. All patients were from the same dentofacial category: mandibular retrognathia with a low to normal mandibular plane angle. In two patients cylindrical self-reinforced polyglycolide (PGA) rods were used for internal fixation of the osteotomy segments. Long-term evaluation showed a complete ossification at the sites of biodegradable rods. In two other patients, self-reinforced poly(L-lactide) (PLLA) screws with a diameter of 2 mm were used to stabilize osteotomy segments. The two different techniques of osteosynthesis and the problems encountered are discussed.     

下颌骨骨折的临床分析（附98例报告）

目的探讨下颌骨骨折的特点及治疗方法。方法收集整理了我院口腔科1995～2000年间98例下颌骨骨折住院患者,对其致伤因素、骨折部位、骨折合并伤及治疗方法进行了整理分析。结果本组病例中,车祸为主要致伤原因,占57.14％;下颌骨体部骨折居首位,占40.54％,最常见的合并伤为面中份骨折,其次分别为胸、腹、四肢伤,颅脑损伤;近年来,坚固内固定在下颌骨骨折治疗中取得了满意临床疗效。结论下颌骨骨折以下颌骨体部、颏部最多;坚固内固定为今后治疗下颌骨骨折的主要发展方向。     

骨性Ⅲ类错(牙合)患者咀嚼时下颌运动特点的初步研究

目的:研究成人骨性Ⅲ类错患者咀嚼不同食物时的下颌运动.方法: 对成人骨性Ⅲ类错伴下颌偏斜患者14 例,不伴有下颌偏斜患者10 例,以及10 例正常对照组,利用下颌定位仪测量咀嚼运动时双侧髁突运动中心点以及切牙点在矢状向、冠状向、水平向的移动范围的面积比值.结果: 咀嚼软食时,从矢状方向和水平方向看,偏斜组双侧髁突点的运动轨迹与非偏斜组和正常组差异显著(P<0.05),而3 组样本切牙点运动轨迹的差异无统计学意义.咀嚼脆食时, 3 组样本在三个方向上的差异更加明显.咀嚼韧食时,在矢状方向上偏斜组偏斜侧髁突点和切牙点运动面积比值明显高于非偏斜组和正常组(P<0.01),而非偏斜侧髁突点运动面积比值仅明显高于正常组(P<0.05).结论: 成人骨性Ⅲ类错患者下颌运动受食物形态和质地的影响相对较大,下颌偏斜患者尤为明显.     

Mandibular trauma treatment: A comparison of two protocols

Objectives: The aim of this study was to evaluate the treatment of mandibular fractures treated in two European centre in 10 years. Study Design: This study is based on 2 systematic computer-assisted databases that have continuously recorded patients hospitalized with maxillofacial fractures in two centers in Turin, Italy and in Amsterdam, the Netherlands for ten years. Only patients who were admitted for mandibular fractures were considered for this study. Results: Between 2001 and 2010, a total of 752 patients were admitted at Turin hospital with a total of 1167 mandibular fractures not associated with further maxillofacial fractures, whereas 245 patients were admitted at Amsterdam hospital with a total of 434 mandibular fractures. At Amsterdam center, a total of 457 plates (1.5 - 2.7 mm) were used for the 434 mandibular fracture lines, whereas at Turin center 1232 plates (1.5 – 2.5 mm) were used for the management of the 1167 mandibular fracture lines. At Turin center, 190 patients were treated primarily with IMF, whereas 35 patients were treated with such treatment option at Amsterdam center. Conclusions: Current protocols for the management of mandibular fractures are quite efficient. It is difficult to obtain a uniform protocol, because of the difference of course of each occurring fracture and because of surgeons’ experiences and preferences. Several techniques can still be used for each peculiar fracture of the mandible. Key words:Mandibular fracture, facial trauma, maxillofacial, treatment, multicentre, database.     

Maxillary anterior and mandibular posterior residual ridge resorption in patients wearing a mandibular implant-retained overdenture

The mandibular implant-retained overdenture could improve masticatory function compared to the conventional complete denture. However, increased forces exerted by the overdenture could increase residual ridge resorption of the maxillary anterior and mandibular posterior areas. The aim of this study was to compare the effect of the mandibular implant-retained overdenture using two or four dental implants, or the conventional complete denture on resorption of the residual ridge of the maxillary anterior and mandibular posterior areas over a period of 10 years. In total, 120 patients, 30 patients treated with an overdenture on two implants (two-implant group), 30 patients with an overdenture on four implants (four-implant group) and 60 patients treated with a conventional full denture (conventional group), participated in this study. On panoramic radiographs, made before and 10 years after treatment, proportional area measurements were applied to determine changes in bone height. After 10 years, a statistically significant amount of bone resorption had occurred in the anterior maxilla in the two-implant group and in the four-implant group. A significant amount of bone resorption had occurred in the posterior mandible in all three groups. There were no statistically significant differences between the groups in both areas. Patients presented large individual differences. It is concluded that patients rehabilitated with implant-retained mandibular overdentures are not subjected to more residual ridge resorption in the anterior maxilla when compared to patients wearing a conventional full denture. Regarding the mandibular posterior residual ridge, resorption was irrespective of wearing an implant-retained mandibular overdenture or a conventional mandibular denture.     

球帽式和Locator式下颌种植覆盖总义齿的临床效果观察

目的：评价球帽式和Locator式下颌种植覆盖总义齿的临床应用效果。方法：共完成2颗种植体支持的球帽下颌覆盖总义齿9例,Locator下颌覆盖总义齿11例,上颌均制作可摘局部义齿。修复完成后于3、6、12、24个月进行随访,对各组种植体周围边缘骨吸收（MBL）进行评估,同时进行义齿满意度调查。结果：Locator组、球帽组在咀嚼功能、固位功能和舒适程度的满意度均高于治疗前,有显著性差异（P〈0.05）;Locator组与球帽组患者满意度、种植体周牙槽骨吸收量差异无统计学意义。结论：使用球帽、Locator附着体种植覆盖义齿在下颌无牙颌的应用中临床效果明显。     

下颌骨前突患者下颌管走行及临床价值探讨

目的：：运用锥形束CT(cone-beam CT,CBCT)分析正常人群和下颌骨前突患者下颌神经管轨迹,为临床手术方案的制订和操作时避开重要神经血管提供帮助。方法：选取28例骨性Ⅲ类下颌骨前突患者（男10例,女18例）、20例正常对照组患者（男12例,女8例）,使用CBCT拍摄下颌管影像,从下颌孔至第一磨牙区分为5个平面,在每个层面分别测量下颌管内外径、下颌骨厚度、下颌管至颊舌侧骨皮质及下颌骨下缘的距离。采用SPSS 17.0软件包对测量结果进行统计学分析。结果：下颌骨前突患者自下颌支到下颌角前部逐渐增厚,继续至下颌第一磨牙区逐渐变薄。下颌神经管的内外径自下颌支到下颌体部逐渐变细。颊侧骨皮质厚度自下颌支区域逐渐增厚,到下颌体部继续变薄。下颌骨前突患者与对照组患者下颌管的对比分析显示,骨髓腔宽度与下颌支厚度有显著差异,且具有显著相关性。结论：CBCT能够精确显示下颌神经管的走行及与周边结构的关系,下颌骨前突患者与对照组患者下颌支厚度的差异主要由颊侧骨髓腔宽度造成。     

The surgical-orthodontic correction of mandibular deficiency. Part II

As discussed in Part I of this study, persons with mandibular deficiency and Class II malocclusions exhibit a wide spectrum of esthetic, cephalometric, and occlusal characteristics. In many such patients optimal overall results are best obtained via a combined orthodontic-surgical approach. In such patients a critical evaluation is essential to decide (1) the optimal operation and (2) the appropriate orthodontic-surgical sequencing. Our method of making these two basic decisions was presented in Part I. In this article the indications for mandibular advancement with or without genioplasty, total subapical mandibular advancement, superior repositioning of the maxilla, and inferior repositioning of the maxilla are discussed in detail. These alternatives are illustrated with representative cases, and the results are discussed. The intention of these two presentations is to illustrate an orthodontic-surgical approach to the correction of mandibular deficiency and Class II malocclusion that is predicated on a systematic evaluation of the individual patient rather than a standard approach.     

Mandibular manipulation of anterior disc displacement without reduction.

The history and clinical symptoms of anterior temporomandibular joint disc displacement without reduction are characteristic, and include limitation of mandibular movement and mandibular deviation on opening of the mouth. Twelve consecutive patients attending a clinic with such symptoms were treated by mandibular manipulation. An immediate increase in the range of mandibular movement was obtained for all patients, with a mean increase in interincisal opening of 8 mm. The method of manipulation is described, and the implications of the results for the treatment of TMJ patients are discussed.     

CAD/CAM制作个性化钛网联合髂骨移植重建单侧下颌骨大型轮廓缺损

目的 探讨CAD/CAM技术联合个性化预成型钛网及游离髂骨移植修复单侧下颌骨大型轮廓缺损的可行性及有效性.方法 选取四川大学华西口腔医院正颌及关节外科2015年11月—2019年12月的15例单侧下颌骨大型轮廓缺损的患者.采用基于逆向工程的计算机辅助设计/计算机辅助制作(CAD/CAM)技术,镜像重建患侧下颌骨轮廓并制...