手术治疗先天性肌性斜颈35例

目的 探讨先天性肌性斜颈的手术方式。方法 依据胸锁乳突肌挛缩程度分为Ⅲ级，结合年龄大小分别采用单纯胸锁乳突肌起点切断术，部分起始部切除术，周围松解术乃至胸锁乳突肌切除术，达到彻底松解，疗效分为优、良、差三级。结果 疗效优28例，良6例，差1例，优良率达97．1％(34／35)。结论 对先天性肌性斜颈根据挛缩程度和不同年龄段采用相应的手术方式可提高疗效。     

先天性肌性斜颈的综合治疗

目的 探讨小儿先天性肌性斜颈的治疗方法。方法 对我院1993-2003年收治的先天性肌性斜颈871例进行回顾性分析,其中288例采用单纯胸锁乳交肌切断术,583例采用以胸锁乳突肌部分切除术为主的综合治疗。结果 单纯胸锁乳突肌切断术满意率80.56％,综合治疗满意率93.14％,明显高于前者(P<0.005)。在综合治疗组中1-2岁年龄组满意率97.12％,明显高于其他年龄组(P<0.005)。结论 先天性肌性斜颈采用以胸锁乳突肌部分切除术为主的综合治疗,疗效很好,推荐手术年龄为1～2岁。     

先天性肌性斜颈病因的实验研究

目的先天性肌性斜颈(Congenital Muscular Torticollis,CMT)在治疗上已得到解决,但在病因上学术界观点不一,本实验旨在探讨静脉受阻是否能导致CMT类似的病理变化。方法用32只本地小狗(约生后一月左右),随机分4组,经手术结扎一侧颈外静脉,观察颈部有否肿块出现,测胸头肌(SCM)、肌电图(EMG)的变化和取SCM标本通过HE染色,Mallory三色法和Bielschdiwsky foot氏法作病理检查及电镜观察。分成四组,手术1个月后;2个月后;3个月后和对照组。 结果实验组胸头肌静息状态EMG节律和形态不规则,频率变慢,低振幅,肌肉强收缩,出现异常EMG(P<0.05),HE染色和特殊染色示实验组出现不同程度间质增生,肌组织纤维化,电镜发现间质增生,纤维化明显且出现成纤维细胞,肌细胞内线粒体减少。结论本实验进一步确认了先天性肌性斜颈的发病与胸头肌(相当于人的胸锁乳头肌)静脉受阻有直接的关系。     

小儿先天性肌性斜颈术后的护理

先天性肌性斜颈是小儿常见的先天性疾病之一。多是由于产伤等原因造成颈部胸锁乳突肌血肿、机化，以致胸锁乳突肌发生挛缩。患儿面部出现畸形，甚至斜视，造成生理和心理上的损害。胸锁乳突肌及软组织松解是治疗该病的常用方法，术后的护理配合对患儿的治愈亦有很重要的作用。我科2001年1月～2003年12月共收治了先天性肌性斜颈患儿20例，取得较好的效果，现报告如下。     

先天性肌性斜颈的治疗进展

先天性肌性斜颈（Congential Muscular Torticol．1is,CMT）是一种小儿常见畸形,临床表现为胸锁乳突肌纤维性挛缩,头面部向一侧偏斜,颅面脊柱畸形。治疗目标是解除活动受限,消除肿块,防止头颈部歪斜,防止面部及头颅畸形。治疗方法分非手术治疗和手术治疗两大类。现就其治疗进展综述如下。     

关于先天性肌性斜颈治疗方法的讨论

先天性肌性斜颈是新生儿常见病。既往其治疗方法,无疑是手法按摩、推拿、支具矫形及手术治疗。可近读人民卫生出版社的《实用儿科学》第7版,在下册2354页,斜颈一节支持这一观点,而上册493页,金百祥教授编写的先天性斜颈一节中认为“当前的治疗观点是期待自愈,手法矫正和按摩是有害无益的”。在患者法律意识和维权意识日渐增强的今天,这种相左的观点出现在国内公认的儿科经典权威专著《实用儿科学》同一版本的不同章节,让读者无所适从。如若按照既往的方法去做,现代观点认为是有害无益;如若按照现代观点静待自愈而不干预,患方又将会拿前面的观点告医生不作为,延误治疗时机,要求赔偿。     

胸锁乳突肌巨微解剖与先天性肌性斜颈病因的关系

目的　观察胸锁乳突肌的血供来源及婴幼儿胸锁乳突肌假性肿瘤的病变特征,探索先天性肌性斜颈的病因与两者之间的关系。方法　用巨微解剖的方法,研究6例小儿尸体胸锁乳突肌的血供。对25例婴幼儿胸锁乳突肌假性肿瘤标本,行大体观察及光镜检查。结果　胸锁乳突肌的血供有多个起源,而且来自不同的解剖平面及方向,平均由4条动脉供给。婴幼儿胸锁乳突肌假性肿瘤大体标本为纤维瘤样,混杂有不同程度的肌组织。光镜显示间质增生,无钙化、出血及炎症反应。结论　从胸锁乳突肌解剖特征,难以解释头颈前曲、侧弯和外旋会造成胸锁乳突肌缺血。结合婴幼儿胸锁乳突肌假性肿瘤病理变化,我们推测婴幼儿胸锁乳突肌假性肿瘤是由于胸锁乳突肌发育紊乱引起,而不是创伤。     

先天性斜颈的病理研究

目的：进一步探讨先天性斜颈的病理变化特征。方法：采用ＨＥ染色、电镜、免疫组化技术进行观察。结果：ＨＥ染色示病变不同程度的间质增生。电镜发现在增生的间质中不仅有成纤维细胞，而且有肌母细胞、间充质样细胞和肌成纤维细胞。在有肿块的患儿，结蛋白、α－平滑肌肌动蛋白和肌动蛋白的阳性率较高。波形纤维蛋白示间质有不同程度的增生。结论：进一步确认间质中有肌母细胞存在，肿块具有分化成熟能力，肌成纤维细胞在病理变化中有重要作用。     

120例先天性肌性斜颈术后的长期随访

目的 通过长期随访手术治疗先天性肌性斜颈的疗效,探讨最佳手术时机及影响疗效的因素.方法 手术治疗120例先天性肌性斜颈患儿,手术时年龄为3～142个月,平均随访4.9年（2～12.5年）,按年龄、术前是否曾行理疗及术式进行分组,根据改良Cheng and Tang疗效评分标准,使用头颈部旋转角、侧屈角、瘢痕、颅面部畸形、肌束情况、头斜及主观评价等指标评估其疗效.结果 总评分为优79例（65.8%）,良37例（30.9%）,中1例（0.8%）,差3例（2.5%）,优良率为96.7%.按生长发育特点分为Ⅰ组（≤1岁）、Ⅱ组（1～3岁）、Ⅲ组（＞3岁）,之后对各指标优率进行x2检验,在瘢痕评分方面无统计学差异（P＞0.05）;但在总评分、旋转角、侧屈角、头斜及肌束挛缩方面,Ⅰ组和Ⅱ组分别与Ⅲ组比较,P＜0.05,提示3岁以内优率更高;在颅面部畸形方面,Ⅱ组和Ⅲ组分别与Ⅰ组比较,P＜0.05,提示1岁以内优率更高;在医生的主观评价方面,3组之间相互比较,P＜0.05,提示年龄越小,优率越高.结论 先天性肌性斜颈3岁以内手术能够获得较好的手术疗效,1岁以内手术疗效最佳.对于1岁以内接受正规理疗6个月无明显效果者应及早手术治疗.     

Conservative Management of Congenital Muscular Torticollis: An Evidence-Based Algorithm and Preliminary Treatment Parameter Recommendations

ABSTRACT

Purpose: To present an algorithm with accompanying treatment parameters for the management of congenital muscular torticollis (CMT) based on the best available literature. Methods: A systematic search of PubMed, MEDLINE, CINHAL, and Cochrane databases was conducted to identify evidence to guide the conservative management of CMT. Results: An evidence-based algorithm was created based on three prognostic factors that influence treatment duration and outcome, including a sternocleidomastoid fibrotic mass, passive range of motion rotation deficit, and age at initiation of treatment. Preliminary treatment parameter recommendations for clinic and home programming accompany the algorithm. Conclusion: Use of the proposed evidence-based algorithm with accompanying preliminary treatment parameter recommendations may improve consistency of care and outcomes for infants with CMT. While a higher level of evidence supports the three prognostic factors utilized in the algorithm, research gaps continue to exist with regards to treatment parameters.     

先天性肌性斜颈患儿胸锁乳突肌组织病理改变和整合素β1表达

目的 研究先天性肌性斜颈(CMT)患儿胸锁乳突肌的组织病理学改变及整合素β1的表达,探讨CMT的病变过程及整合素β1的作用机制及意义.方法 采用HE染色、Gomori银染观察45例CMT患儿胸锁乳突肌标本组织病理学改变,采用免疫组织化学方法检测其整合素β1表达,利用图像分析系统定量分析整合素β1表达量和分布特点.结果 1.光镜观察 HE染色标本显示胸锁乳突肌纤维化改变可以归纳为2类:A类改变表现为肌细胞萎缩变小,周围有大量结缔组织纤维增生,纤维排列紊乱,且伴许多血管和神经增生,最后肌细胞萎缩消失;B类肌细胞首先表现为肌原纤维结构消失(横纹或正常肌节结构消失),在纤维化改变前保持细胞轮廓,细胞膜完整,然后肌细胞原位发生纤维性病理改变,在纤维束之间有成纤维样细胞,细胞有较多突起.Gomori银染标本显示胸锁乳突肌主要改变以大量胶原纤维增生为主,A类胶原纤维排列紊乱,B类胶原纤维排列较整齐.2.免疫组织化学染色显示整合素β1在健康对照组表达为弱阳性(125.7±5.167).病变标本表达程度分3种,A类肌细胞质中表达呈强阳性(30.15±6.543),与正常组比较有显著性差异(P＜0.01);B类肌细胞表达呈阴性(177.15±13.45),与正常组比较有显著性差异(P＜0.01),另外一些肌细胞肌原纤维结构基本正常,表达呈弱阳性(114.6±17.23),与正常组比较无显著性差异(P＞0.05)).结论 胸锁乳突肌纤维化是一个复杂渐变过程,纤维化病理过程可能有不同方式发生,整合素β1可能参与疾病的发生过程.     

Congenital nephrosis: Ultrasonographic features

The striking ultrasonographic features in a case of Congenital Nephrosis are described. The kidneys are enlarged and markedly echogenic. There is loss of distinction between the cortex, medulla and renal sinus. The differential diagnosis and histological findings are discussed.     

先天性马蹄内翻足病因学研究

为先天性马蹄内翻足的神经肌肉病变学说提供科学依据。对手术后随访的９２例摄腰骶部Ｘ线片，７７例作直肠肛门测压检查，并对２３例患肢腓肠肌、胫前肌和腓骨肌４２个标本进行了运动神经支配的观察，测量了轴突终末支配比率，观察了运动终板形态，还通过琥珀酸脱氢酶组化染色检查了４８个标本不同肌群的组化分型变化。另外还对１４个标本进行了肌肉超微结构的研究。结果：９２例中发现７８．３％合并隐性能椎裂；肛管静息压和直肠肛管压差明显高于对照组；三组肌群中运动终板存在着退变或再生者占４２．９％～５４．６％；轴突终末支配比率（ＴＩＲ）增高者占６２％；组化分型提示有红肌纤维增高与集聚，肌纤维大小不等，形态各异，尤以腓肠肌和胫前肌突出，其超微结构也呈现去神经肌萎缩改变。这些改变提示本病病因系神经肌肉病变使三组肌力不平衡而产生马蹄内翻足，但是脊髓神经病变尚不清楚，肌肉改变是否为继发性病变也难定论。     

Congenital Muscular Dystrophies -Problems of Classification

The classification of congenital muscular dystrophies (CMD), based on perceived clinical and morphological similarities or differences, is controversial. CMD without cerebral involvement has sometimes been divided into a mild and a severe form. This distinction is, however, arbitrary and not uncontested. Whether Ullrich's disease, formerly called atonic-sclerotic dystrophy, is a disease entity and if so, whether it is a primary muscle disorder, is uncertain. CMD without cerebral involvement is inherited in an autosomal recessive fashion in the great majority of cases. CMDs with cerebral involvement are usually classified into at least three forms: the Fukuyama type of CMD, occurring almost exclusively in Japanese patients; CMD with hypomyelination, sometimes also called the occidental type of cerebromuscular dystrophy; and Walker-Warburg syndrome. Muscle-eye-brain disease, described in a number of Finnish patients, may or may not belong in this last category. In CMD with cerebral involvement inheritance is also autosomal recessive. It is possible that single sporadic cases are pheno-copies due to infectious or other exogenous causes. Reports of clinical and morphological findings from an increasing number of patients show a high degree of variability within and, on the other hand, certain similarities between the forms of CMD with cerebral involvement. In addition, neuroradiological changes are also found with increasing frequency in CMD patients without clinical neuropsychological abnormalities. It is not unreasonable to speculate that molecular genetic techniques will reveal in the near future a variable defect in one gene locus or defects in a few gene loci as the cause of the various clinical forms of CMDs.     

Ultrasonographic evaluation of abdominal masses in children

Ultrasonography is a recently acquired diagnostic modality in our sub-continent. The initial experiences with this technique in 55 children with abdominal masses are presented. In 15 children ultrasonography merely confirmed the diagnosis which was arrived at by other means; a positive contribution towards the final diagnosis was made in 33 children, while in 7, ultrasonography did not contribute towards the diagnosis. The scope, advantages and pitfalls of ultrasonography are briefly discussed.