Mandibular reconstruction in the growing patient with unilateral craniofacial microsomia: a systematic review

The purpose of this systematic review is to provide an overview of the surgical correction of the mandible in unilateral craniofacial microsomia (UCM) performed in the growing patient, and its long-term outcome and stability. The following databases were searched: PubMed, Embase, Cochrane, and Web of Science. Articles reporting prospective and retrospective studies of patients not older than 16 years (N ≥ 4) who had undergone surgical correction of a craniofacial microsomia spectrum condition using grafts, osteotomies, distraction, or combinations of these, were reviewed. The period of follow-up was selected to be ≥1 year. After inclusion, the articles were evaluated on short- and long-term outcomes, relapse, and any increase in asymmetry following treatment. Thirty of 1611 articles were included in the qualitative synthesis. Analysis of the surgical mandibular correction of UCM showed that the outcome is not so much treatment-dependent, but patient-dependent, i.e. deformity gradation-dependent. The type I–IIa Pruzansky–Kaban patient had the best results with regard to minimal relapse and/or minimal increase in asymmetry. Single-stage correction of the asymmetry should be postponed until the permanent dentition stage. It can be concluded that in the treatment of the severely hypoplastic mandible, the patient will benefit from a multi-stage treatment protocol if indicated for functional or psychological problems.     

Rokitansky sequence in association with the facio-auriculo-vertebral sequence: Part of a mesodermal malformation spectrum?

We report on a 4-year-old first-born mono-zygotic twin girl with a hypoplastic left face, mandible and zygoma, left microtia without an external auditory canal, a U-shaped cleft palate, right ectopic-fused kidneys, a blindly ending vaginal pouch, and absent uterus. We review 3 other cases with the manifestations of the Rokitansky and the facio-auriculo-vertebral sequence. The anomalies in these disorders can be thought of as deriving from an early defect or disruption in fetal mesoderm or its progenitor tissue at the time of primitive streak formation. They are frequently associated with other mesodermally derived defects or sequences and may together represent an extended polytopic field defect. While such speculation on how these spatially separated anomalies develop is probably simplistic, the concept of a mesodermal “malformation” spectrum is helpful in reminding the clinician to look for other mesodermal defects when one mesodermally derived defect or sequence is detected.     

电针疗法联合手法灸治疗Meige综合征1例并文献复习

目的 观察电针疗法联合手法灸治疗Meige综合征的疗效及优势。方法 整理1例Meige综合征患者资料,辨证取穴,针刺得气后连接电针仪。留针30 min后出针施以手法灸10～15 min。1次/d, 7 d为1疗程,5个疗程结束后观察患者症状改善情况。结果 5个疗程结束后患者症状基本消失,无复发。结论 电针联合手法灸治疗Meige综合征疗效及优势显著。     

Ⅱ型Crigler-Najjar综合征1例报告

正Crigler-Najjar综合征(CNS)是由Crigler和Najjar于1952年首次报道而得名,是由尿苷二磷酸葡萄糖醛酸基转移酶(uridine 5'-diphosphate glucuronosyltransferase,UGT)1A1基因突变使其酶活性完全或部分丧失而导致的遗传性胆红素代谢障碍性疾病,分为Ⅰ型和Ⅱ型,发病率为百万分之一[1-3]。为常染色体     

6年随访1例伴染色体1,11核型异常演变的骨髓增生异常综合征

本研究探讨骨髓增生异常综合症-原始细胞增多型(MDS-RAEB)患者的染色体核型演变与疾病进展的关系。通过对1例MDS-RAEB-II患者长达6年的随访,动态检测染色体核型、骨髓以及血常规,并采用荧光原位杂交技术(FISH)对患者的异常核型进行验证。结果表明,随访72个月中发现急性髓系白血病型化疗对患者无效,而全反式维甲酸、6-巯基嘌呤的诱导及支持治疗对患者有效。在初诊时为正常男性核型,病程第48月出现核型演变46,XY,2q+[1]/46,XY[19],第56月演变为46,XY,dup(1q)[3]/46,XY[7],第68月出现以dup(1)和der(11)为主的复杂染色体异常,并有血小板进行性下降。结论:染色体核型的演变与骨髓增生异常综合症疾病的进展可能有相关性。     

微血管减压术治疗面肌痉挛患者的围术期护理

目的探讨微血管减压术治疗面肌痉挛术后出现的并发症及围术期护理措施。方法统计微血管减压术治疗面肌痉挛的患者例数以及并发症发生情况,回顾性分析住院期间发生并发症的患者病历资料。结果 28例患者中,术后出现面瘫8例、听力下降3例、后组颅神经损伤1例、小脑损伤1例、脑脊液漏1例,通过有效的护理评估和干预,患者均顺利度过术后并发症高危期。结论精心的护理能减少并发症给患者造成的伤害和促进患者康复。     

同步视频监测侧方扩散反应在面肌痉挛手术中的应用

目的 探讨显微血管减压术(microvascular decompression,MVD)治疗面肌痉挛术中应用同步视频监测侧方扩散反应(lateral spread response,LSR)的意义.方法 回顾性分析105例行MVD且术中实施同步视频LSR监测的面肌痉挛病人的临床资料.分别将依据术中LSR监测及同步视频...     

局部注射A型肉毒毒素治疗Meige综合征

目的 观察A型肉毒毒素(BTX—A)治疗Meige综合征，(眼睑痉挛-口颌肌张力障碍综合征)的疗效。方法 用A型肉毒毒素对17例Meige综合征行面部肌肉局部多点注射，分析其治疗结果。结果 17例中完全缓解者9例，明显缓解者5例，部分缓解者2例，无效1例。总有效率94％。起效时间数小时至3天，疗效持续时间3～6个月。局部副反应轻微、短暂，无全身反应及过敏反应。结论 A型肉毒毒素是治疗Meige综合征最有效的方法。     

臀筋膜间室综合征

目的:探讨臀筋膜间室综合征的发病机理,临床特点和手术治疗。方法:对4例臀筋膜室综合征病人进行减压治疗。结果:2例6小时内减压术后无后遗症,2例超过6小时减压遗留臀肌挛缩症、足下垂。结论:醉酒后臀部较长时间受压是GCS的主要病因,临床表现为臀部张力性包块、臀和髋关节疼痛并向小腿放射、踝反射和伸长肌肌力伸减弱,应尽早手术、彻底减压。     

显微血管减压术后面肌痉挛症状的演变特点

目的分析面肌痉挛行显微血管减压手术后症状的演变特点,探讨其可能机制。方法回顾性分析90例面肌痉挛病人的临床资料,均采用显微血管减压手术治疗。根据Cohen分级对病人术后1年内不同时间段的面肌痉挛程度进行评分,得到症状评分-时间曲线,分析面肌痉挛症状改善的演变特点。结果显微血管减压术后,将病人分为术后顷刻和出院时症状均消失组(A组)、术后顷刻症状消失而出院时又再现组(B组)、术后顷刻和出院时症状均减轻组(C组),3组病人长期随访症状消失比例分别为90.1%、83.3%、66.7%。术后症状评分-时间曲线表明:总体演变趋势先急剧下降,后低幅波动,并逐渐下降,术后9个月趋于稳定。结论显微血管减压术后,面肌痉挛症状有逐渐缓解趋势。根据术后即刻至出院前的症状恢复情况将病人分组,有助于预测显微血管减压手术的长期疗效。