Management of a patient with Mobius syndrome: a case report

Mobius syndrome is a rare congenital disorder with the primary diagnostic criteria of congenital facial and abducent nerve palsy. Orofacial anomalies and limb malformations may be associated with the disorder. Involvement of other cranial nerves also is common. Occasionally, the V, X, XI, and XII cranial nerves are involved, resulting in difficulty of chewing, swallowing, and coughing, which often leads to respiratory complications. Mental retardation and autism have been reported in some cases. An 18–year-old Hispanic male came to the General Practice Residency clinic at the University of Illinois at Chicago for dental treatment. The patient had a history of Mobius syndrome, mental retardation, and behavioral problems. Because of acute situational anxiety and violent behavior, we arranged for the patient to have general anesthesia while we provided complete oral rehabilitation. This article discusses the treatment of a patient, including special considerations taken during anesthesia and dental management. A review of the special challenges concerning patients with such a condition are reviewed.     

Early dental management of patients with Mobius syndrome

OBJECTIVE(S): The aim of this study was to evaluate the orofacial manifestations in patients with Mobius syndrome (MS), establish an early adequate dental treatment and discuss the possible etiology of all cases examined based on information about the gestational intercurrences. DESIGN: Prospective study. SETTING: Special Care Dentistry Center, School of Dentistry, University of Sao Paulo, Brazil. Subject(s) and methods: Twenty-nine patients with MS aged 0 to 4 underwent prospective dental examination as well as early orthodontic treatment. RESULTS: All patients presented micrognathia, lack of lip seal, high arched palate and weak soft palate. The use of orthopedic appliances was recommended to all 29 patients, but only 13 adhered to treatment and were monitored for at least 24 months. We observed that, after 24 months of treatment, the palate was expanded and micrognathia became less severe in the majority of the cases. Pregnancy-related complications were reported by 27 (97%) of the 29 mothers. CONCLUSION(S): The early use of orthopedic appliances was important to prevent malocclusion and glossoptosis. Attempted abortion with misoprostol is associated with an increased risk of MS in infants.     

Service-learning and dental hygiene: a literature review

Dental hygienists should be aware of the concept of service-learning and how it can enhance dental hygiene education. A look at national documents and other relevant historical literature will be reviewed, in addition to more recent books and articles to define service-learning. Although perceived as a new teaching strategy, service-learning has its roots in experiential education. Several definitions of service-learning have emerged, which take into consideration the reciprocal needs of the student and community, bridging academic theory with community service, and instilling civic responsibility through a reflective component. Considering the advantages and disadvantages, service-learning seems to be a good methodology for dental hygiene education. Recommendations include the expansion of the ADA Accreditation Standards to include service-learning, and incorporation of an operational definition of community-based oral health programs, as well as the term service-learning in its Definition of Terms.     

Larsen syndrome: a review of the literature and case report

Larsen syndrome is a rare genetic disorder characterized by multiple dislocations of the large joints and characteristic craniofacial abnormalities. It exists in both a severe autosomal recessive form and a mild autosomal dominant variety. To date, only three authors have reported oral findings for this syndrome. This paper describes an 8‐year‐old Chinese boy with Larsen syndrome who had advanced periodontitis. The need for periodontal therapy and regular monitoring of such patients has been highlighted.     

Schwartz-Jampel syndrome: a review of the literature and case report

Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive skeletal dysplasia associated with myotonia. The manifestations of SJS include short stature, blepharophimosis, and skeletal anomalies. The combination of skeletal and muscular abnormalities may result in oro-dental manifestations such as atypical facies, with micrognathia, microstomia, pursed lips, crossbite, cleft palate, as well as mandibular hypoplasia, the risk of dentigerous cysts, and impacted teeth. The use of general anesthesia in patients with SJS is dangerous, as there is a risk of malignant hyperthermia. The purpose of this paper is to describe a 3-year-old Chinese boy diagnosed with SJS at birth. His intra-oral examination revealed significant dental findings such as dentin defects with generalized attrition and hypodontia of the permanent dentition. Comprehensive dental treatment was provided at the same time as lower right limb surgery to reduce the potential complications with general anesthesia.     

Lowe syndrome: literature review and case report

Abstract

This paper is the first to describe dental findings and orthodontic characteristics of a young adult patient with Lowe syndrome. This syndrome is a rare genetic disorder inherited by a sex-linked pattern, involving primarily the kidneys, eyes and nervous system, which also present oral manifestations. This paper also present the results of careful extra and intra-oral evaluations and the findings of panoramic and cephalometric radiographs of an 18-year-old male with Lowe syndrome.     

Marfan syndrome: a review of the literature and case report

Marfan syndrome (MFS) is a connective tissue disorder of variable inheritance that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Orofacially, MFS patients typically exhibit skeletal class II malocclusion, dolichofacial growth pattern, mandibular retrognathia, malar hypoplasia, high arched palate, dental crowding, and root anomalies. The purpose of this paper is to provide a review of the literature, as well as describe an 11‐year‐old female with MFS diagnosed at the age of 10.5 years. This report emphasizes the orofacial findings in MFS and highlights particularities of dental treatment when social deficits and intellectual disabilities are also implicated.     

Mentoring of dental and dental hygiene faculty: a case study

Given the predicted shortages of dental faculty in the United States, it is important to retain faculty members. Mentoring could play a crucial role in this context. The objectives of this case study were to explore how a six-year mentoring program in the Department of Periodontics and Oral Medicine at the University of Michigan School of Dentistry changed faculty members' perceptions of support from colleagues and their mentoring expectations. In addition, we sought to determine how junior versus senior faculty members and mentors versus mentees differed in their perceptions of faculty roles, their self-perceived competence, and their awareness of departmental expectations at the end of the program. Data were collected with self-administered surveys from twenty-five of the thirty-six faculty members in this department in 2002 (response rate: 69 percent) and from thirty-seven of the fifty-four faculty members in 2008 (response rate: 69 percent). The results showed that the perceptions of support from colleagues improved significantly over the six-year period. During the same time period, mentoring expectations increased. In 2008, junior faculty members as compared to senior faculty members remained less positive about their role as faculty members, felt less well prepared for their professional life, and were less aware of departmental expectations. In conclusion, a departmental mentoring program resulted in improved support from colleagues and increased expectations concerning mentoring experiences. However, future targeted interventions are needed to address the identified differences between junior and senior faculty members. Recommendations for faculty mentoring efforts are discussed.     

AEC综合征1例报告及文献复习

AEC综合征是一种罕见的以睑缘黏连、外胚层发育不全及面裂为主要临床表征的基因病。目前,国内尚未见该病的报道。本文报告1例AEC综合征病例,根据患者的临床表现,制订相应的治疗方案,手术修复左侧唇裂,术后患者外形有一定改善。通过文献复习,对AEC综合征的病因、临床表现、鉴别诊断及序列治疗进行了讨论。由于AEC综合征临床表现多样,严重影响患者面容及生长发育,因此,应增强对本病的认识,并进行系统序列治疗。     

Facial palsy in the dental surgery. Case report and review

Facial palsy is a relatively uncommon condition with a variety of causes including dental intervention. A brief history of two cases of facial palsy with dental implications is presented. The aetiology of facial palsy is reviewed and the management of such cases is discussed. The option of immediate treatment of Bell's palsy with prednisone is stressed.     

Large intraparotid facial nerve schwannoma: case report and review of the literature

Here is reported an extremely rare case of a large intraparotid facial nerve schwannoma in a 32-year-old female who presented with a parotid mass. There had been a long clinical course and sudden onset of facial weakness. Diagnostic evaluation and surgical management are discussed along with a brief review of the literature.     

Hemifacial hyperplasia: a case series and review of the literature

Condylar hyperplasia is known to result in facial asymmetries and constitutes a well-recognized group of unilateral mandibular enlargements. Condylar hyperplasia has been sub-classified into hemimandibular hyperplasia and hemimandibular elongation. A much rarer disorder, hemifacial hyperplasia (or hemifacial hypertrophy) is a congenital malformation characterized by prominent unilateral overdevelopment of the hard and soft tissues of the face. The affected side grows at a faster rate than the non-affected side, creating a marked asymmetry that potentially involves the skeleton and teeth, as well as all components of the associated soft tissues. Hemifacial hyperplasia is usually identified at birth and progresses towards puberty, but is not thought to alter throughout the lifetime of affected individuals. A case series of five patients clinically diagnosed with hemifacial hyperplasia is presented, with the aim of reviewing the clinical features, discussing their individual surgical management, and summarizing the more recent identification of possible genetic mutations that may be responsible for hemifacial hyperplasia and related overgrowth disorders. It is speculated that depending on the genetic factors, the disorder may be progressive in specific cases.     

基底细胞痣综合征1例报告及文献复习

基底细胞痣综合征属常染色体显性遗传性疾病,系由多发性颌骨角化囊肿、皮肤基底细胞痣(癌)、骨骼系统异常以及各种其他缺陷所组成的一种复杂罕见的综合征,本文报告1例典型病例,对其主要发病机制、临床特点、诊断和治疗等进行回顾分析。     

Oculocerebrorenal Lowe syndrome: a literature review and two case reports

The present paper reviews the dental findings in oculocerebrorenal Lowe syndrome and presents two case histories. Reports of different patients are useful in order to enhance knowledge about the syndrome, because there are so many different oral manifestations.     

SAPHO syndrome with TMJ involvement: review of the literature and case presentation

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin manifestations. The etiology is unknown but probably involves genetic, infectious, and immunological components. The characteristic feature of the disease is found in the bone lesions, which typically involve the anterior chest wall and axial skeleton. In the literature review, six case reports discussed involvement of the TMJ. Treatment of SAPHO is geared toward symptom management as there is no cure. Surgery for mandibular lesions is usually a last resort as results are reported to be temporary with symptoms recurring within a year. Surgery appears to be performed early after diagnosis of TMJ related pathology; probably because lesions affecting the TMJ involve some limitation in mouth opening with varying degrees of ankylosis. The authors provide a literature review and describe a case of SAPHO syndrome with ankylosis of the left TMJ. The patient was treated with joint reconstruction using a patient-fitted total joint prosthesis (TMJ Concepts Inc., Ventura CA) in single stage surgery. This paper is the first to report maxillary involvement in SAPHO syndrome.     

歌舞伎综合征伴先天性膈疝一例及相关文献回顾

歌舞伎综合征（KMS）首次由日本报道,发病率约1/32 000。该疾病常伴发多种先天畸形,但目前没有明确的诊断标准。KMS主要通过5个临床表现来诊断：（1）特殊面容;（2）骨骼异常;（3）皮纹异常;（4）轻中度智力发育落后;（5）身材短小。除此之外,其他的特殊表现也有助于诊断该疾病,但是该综合征伴发先天性膈疝（CDH）较罕见。本文报道了1例12月龄KMS患儿伴发CDH和腭裂。该病例提示对于KMS患者有必要进行全面的体格检查,同时全面的麻醉管理也非常重要。     

Sturge-Weber综合征1例报告及文献复习

目的探讨SturgeWeber综合征的临床特点、诊断方法及治疗手段。方法对1例不典型Sturge-Weber综合征的临床资料进行总结,复习文献以比较各种治疗方法的临床疗效。结果对症治疗效果良好。促脑代谢药物可减轻神经症状,激光治疗皮肤血管痣疗效确切,动脉栓塞治疗颊部血管瘤体积缩小60%以上,随访半年未见增大。结论该病综合治疗的效果明显好于单一疗法。