6岁以上先天性肌性斜颈患儿48例诊疗体会

目的探讨6岁以上大龄儿童先天性肌性斜颈治疗的体会。方法采用胸锁乳突肌双极松解和肌腱部分切除术,术后行枕颌吊带牵引,配戴胸颈联合支具联合治疗,随访3-10 a、平均6.13 a。结果疗效优良者45例（93.75%）;1例出现一过性乳突切口周围皮肤麻木感。结论对6岁以上大龄先天性肌性斜颈患者,行双极松解结合术后有效的枕颌吊带牵引及配戴矫形支具是较有效的治疗方法。     

先天性上斜肌麻痹致眼性斜颈的手术治疗

对38例有代偿头位的先天性上斜肌麻痹致眼性斜颈患儿行下斜肌减弱术或配偶肌的下直肌后徙术及麻痹眼上直肌后徙术.术后28例代偿头位消失,8例代偿头位好转,2例无效.认为先天性上斜肌麻痹致眼性斜颈手术治疗效果可靠.     

非手术疗法治疗斜颈22例疗效观察

小儿先天性肌性斜颈单纯手法治疗效果不甚理想。笔者近两年来采用保守疗法治疗22例,经随访观察,疗效满意。一、临床资料本组男14例,女8例,半岁以内12例,7个月～1岁8例,1～2岁2例。过期妊     

交变电震磁治疗先天性肌性斜颈

1980～1992年,我们采用改良电磁治疗机治疗75例先天性肌性斜颈患儿,疗效显著。1.临床资料:本组年龄7天至6个月,有难产史43例,臂位及产钳助产各8例。患儿出生后头颈均偏向一侧,其中左侧31例,右侧44例;胸锁乳突肌中下部触及肿块68例,直径1.5～4.5cm不等。肌挛缩5例。     

先天性眼外肌麻痹误诊为斜颈3例分析

先天性眼外肌麻痹误诊为斜颈３例分析山东电力中心医院（２５０００１）弭波笔者遇有３例在外院误诊为斜颈的先天性眼外肌麻痹患儿。男２例，女１例；年龄５～６岁。患儿分别在出生后６月～１岁时家长发现其头部歪向一侧，在当地医院诊断为先天性斜颈，经保守治疗无效，均...     

早期伸展运动治疗婴儿先天性肌性斜颈126例

先天性肌性斜颈（CMT）是常见的婴儿颈部畸形。其病因尚不完全清楚。2000年1月～2004年12月，我们对126例CMT患儿早期行被动伸展运动法（PSE）治疗，疗效满意。现报告如下。     

经腹直肌旁腹膜外单切口肾输尿管及膀胱袖套状切除术33例观察

目的 评价经腹直肌旁腹膜外单切口行肾输尿管及膀胱袖套状切除术的可行性及临床疗效.方法 回顾性分析33例经腹直肌旁腹膜外单切口行肾输尿管及膀胱袖套状切除术治疗上尿路移行细胞癌患者的手术经过与临床资料.结果 33例手术均成功,手术时间90～120 min,术中出血150～260 ml;术后胃肠功能恢复时间为16～48 h,术后止痛剂用量0～100 mg,引流量120～240 ml,术后住院7～10 d,无腹腔脏器损伤等并发症.结论 经腹直肌旁腹膜外单切口行肾输尿管及膀胱袖套状切除术,具有切开缝合简单、手术时间短、创伤小、恢复快等优点.     

先天性心脏病右腋下小切口心内直视术围术期护理

目的分析先天性心脏病右腋下小切口心内直视术的围术期护理。方法选择我院2012年1月~2015年1月收治的行右腋下小切口心内直视术治疗的先天性心脏病患儿50例,治疗期间全部患儿均给予科学有效的围手术期护理干预,观察其护理效果。结果所有患儿的手术均顺利实施,病情保持稳定,有效脱离呼吸机患儿出血量少、疼痛显著缓解,未出现肺部感染,切口愈合情况理想,患儿经检查后完全正常,无并发症出现。结论加强右腋下小切口心内直视术的围手术期护理,能有效提高手术治疗的成功率,降低术后并发症发生率,促进患者更好康复。     

经肛门先天性巨结肠根治术并直肠肌鞘部分切除术的临床应用观察

目的观察经肛门先天性巨结肠根治术并直肠肌鞘部分切除的疗效。方法 43例先天性巨结肠患儿,在行经肛门根治术的同时行直肠后壁肌鞘部分切除。结果本组手术均顺利完成。39例获半年以上随访,4例失访。术后1年优16例、良15例、差8例,2年优23例、良10例、差2例,2年以上优29例、良4例、差0例。术后1、2、3、6、9个月肛管静息压分别为（20.0±6.4）、（21.0±8.3）、（22.0±10.4）、（24.8±9.9）、（24.9±10.3）mmHg,均低于术前的（37.6±12.3）mmHg,P均〈0.05。结论经肛门先天性巨结肠根治术并直肠肌鞘部分切除治疗先天性巨结肠效果满意。     

先天性心脏病术后并发膈肌麻痹26例护理

目的 总结先天性心脏病矫治手术后膈肌麻痹患儿的护理方法 .方法 对26例先天性心脏病矫治手术后出现膈肌麻痹的患儿密切观察病情变化,防止水、电解质、酸解平衡紊乱,做好呼吸道的护理和膈肌折叠术后的护理.加强营养支持等护理工作.结果 26例均安全撤离呼吸机,治愈出院,其中6例行膈肌折叠术,术后经过合理治疗、护理,最后顺利撤机.结论 密切病情观察、及时有效的护理对先性心脏病手术后并发膈肌麻痹的患儿的康复非常重要.     

Sternocleidomastoid size and upper trapezius muscle thickness in congenital torticollis patients: A retrospective observational study

The purpose of this study was to investigate the upper trapezius muscle thickness (UTMT) in congenital muscular torticollis (CMT) patients and determine the correlation among sternocleidomastoid muscle thickness (SCMT), accessory nerve (AN) cross-sectional area (CSA), and UTMT in CMT.This retrospective study consisted of 2 participant groups: Group 1 (SCM mass CMT, n = 20) and Group 2 (Postural CMT, n = 22). For both groups, B-mode ultrasound was performed by a physiatrist to measure the SCMT and UTMT and calculate the CSA of the AN. The correlation among SCMT, CSA of the AN, and UTMT in both groups was evaluated.The between-group comparison revealed that Group 1 had significantly greater SCMT, UTMT, and CSA of the AN on the affected side than Group 2 (P < .05). The intragroup comparison between the affected and unaffected sides also revealed that, in Group 1, the SCMT, UTMT, and CSA of the AN were significantly higher on the affected side than on the unaffected side (P < .05), whereas no significant differences were observed in Group 2. In Group 1, a positive correlation (r = 0.55) was observed between the UTMT and CSA of the AN on the affected side, but not observed between the SCMT and CSA of the AN.The findings of the study indicate that sternocleidomastoid muscle size may impact the thickness of the upper trapezius muscle via the accessory nerve in patients with congenital torticollis.     

心内直视下镶嵌治疗肌部室间隔缺损

目的 探讨心内直视下镶嵌治疗肌部室间隔缺损（mVSD）的手术方法及临床疗效.方法 2008年1月至2013年7月,在体外循环心内直视下镶嵌治疗肌部室间隔缺损29例,男19例,女10例,年龄2个月～7岁;其中单个肌部室间隔缺损7例,多发室间隔缺损22例.合并法洛四联症5例,完全性大血管转位1例,肺动脉瓣狭窄3例,房间隔缺损6例,主动脉缩窄3例.合并心脏畸形均同期手术纠治.结果 死亡2例,死亡率6.9％.术后随访3个月～3年,无远期死亡,封堵器边缘少量残余分流3例,无封堵器偏移、二尖瓣反流、主动脉瓣反流、Ⅲ度房室传导阻滞.结论 心内直视下镶嵌治疗肌部室间隔缺损是一种安全、简便、有效的方法.     

Muscular ventricular septal defects repaired with left ventriculotomy

Between 1974 and 1979 nine patients, aged 10 months to 4 years, underwent left ventriculotomy for closure of single or multiple defects in the muscular ventricular septum. The vertical incision paralleled the anterior descending branch of the left coronary artery near the apex of the left ventricle and ranged from 2.5 to 3.5 cm in length. Four patients also had a right ventriculotomy with closure of a high perimembranous ventricular defect in two. Serial electrocardiograms indicated changes of myocardial ischemia or necrosis. Left bundle branch block did not develop in any patient.Three patients died in the early postoperative period. The six surviving patients are living and well 2 to 7 years later. There is apparent complete closure of the ventricular defects, which was documented by cardiac catheterization in four cases. Two patients had cardiomegaly and left ventricular dysfunction as assessed with echocardiographic and angiographic study, whereas four displayed good cardiac function. In three of the latter patients, cardioplegia or deep hypothermia techniques were utilized intraoperatively. The observations indicate that left ventriculotomy of limited size is an acceptable approach to the difficult problem of repair of muscular ventricular defects but may involve some risk of compromise of the coronary circulation.     

右腋下纵行直切口在未成年先天性心脏病患者手术中的临床应用

目的总结和分析我科在右腋下纵行直切口体外循环下,矫治房间隔缺损、室间隔缺损及肺动脉瓣狭窄未成年患者的临床资料,并对该径路的优缺点进行评价。方法：回顾性分析我科2011年1月-2012年10月,经右腋下纵行直切口治疗的280例未成年先天性心脏病（CHD）患者的临床资料,包括：体外循环时间、主动脉阻断时间和主要并发症。结果：280例全部手术患者体外循环时间平均为52．35 min,主动脉阻断时间为22．36 min,无死亡及其它严重并发症,但术后80例有不同的并发症：心包积液19例、心包积气2例、右下肺感染27例、右肺感染2例、双肺感染1例、右侧胸膜增厚及黏连1例、右侧气液胸（胸腔积液）1l例、伴肺组织压缩3例、右侧气胸4例、右上叶不张3例、右侧胸壁皮下气肿2例、左侧胸腔积液3例、双侧胸腔积液1例及右侧肋骨骨折伴胸腔积液1例。结论：采用右腋下纵行直切口径路治疗CHD具有创伤小、出血少、关胸快,术后胸腔及心包引流量少、胸廓稳定性好、可避免术后胸骨裂开、鸡胸等并发症及美观效果好等优点。在充分判断患者体质量、年龄的基础上明确诊断,并可预见性地预防术后相关肺部并发症的发生,值得广泛应用。     

Camptocormia: a sign of axial myopathy. Report of 7 cases

PURPOSE: Camptocormia or progressive lumbar kyphosis is an anterior bend of the trunk. It appears in orthostatism or while walking and is reducible in the decubitus position. It concerns patients older than 60 years of age. It is due to a fatty degeneration of the paravertebral muscles, although the physiopathology remains unclear. METHODS: We report seven cases of camptocormia revealing authentic myopathies. RESULTS: Our observations concern five women and two men of 55 to 72 years of age. All patients present lumbar kyphosis and had a fatty involution of the paraspinal muscles on the muscular MRI. Four patients fulfilled the Bohan and Peter criteria of polymyositis and dermatomyositis. In the other cases paravertebral muscular biopsies led to the diagnosis of a congenital myopathy, a mitochondrial myopathy and an amyloid myopathy. Four patients received a corticosteroid-immunoglobulins or cyclosporin regimen. An improvement in the camptocormia was observed in three cases. In the other cases the treatment consisted of chemotherapy on account of severe nephrotic syndrome, a coenzyme-Q treatment for the patient with mitochondrial myopathy and only physiotherapy in the case of congenital myopathy, but without positive effect on camptocormia. CONCLUSION: Camptocormia appears as a muscular symptom that may reveal an axial myopathy due to multiple and varied pathologies. Thus, the discovery of camptocormia requires an aetiological investigation in order to propose an adequate treatment, which should be associated with physiotherapy.     

Assessment of left ventricular systolic and diastolic functions in children with merosin-positive congenital muscular dystrophy

Cardiopathy is an expected finding in X-linked Duchenne and Becker muscular dystrophies. This holds true for some other forms such as autosomal recessive limb-girdle dystrophies. However, data on early-onset and usually severe congenital muscular dystrophies are limited. The purpose of this study was to investigate the presence of cardiac involvement in children with merosin-positive congenital muscular dystrophy. A total of 42 patients and 22 healthy subjects were evaluated by M-mode, 2D, and Doppler echocardiography. Cardiac anatomy, left ventricular dimensions, wall thickness and systolic and diastolic functions were investigated in patients and compared with those of healthy control subjects. Mean left ventricular ejection fraction and shortening fraction were significantly lower in the patient group (P<0.05 and P<0.001, respectively) and in three patients ejection fraction was below 55%. Although some impairments in left ventricular inflow indexes which were suggestive of left ventricular diastolic dysfunction were detected in patients with merosin-positive congenital muscular dystrophy they were not statistically significant. Our results suggest that left ventricular systolic abnormalities may occur in children with merosin-positive congenital muscular dystrophy.     

介入技术和外科手术同期"复合"治疗先天性心脏病的临床研究

目的结合介入器械和外科手术,实施“复合（Hybrid）”技术对先天性心脏病实施治疗。方法2005年3-10月,20例先天性心脏病患者接受了术中Hybrid技术治疗。球囊扩张组包括3例室间隔完整型肺动脉闭锁的新生儿和4例婴儿重度肺动脉瓣狭窄。缺损封堵组13例,包括10例房间隔缺损和3例多发室间隔缺损。正中或右侧腋下小切口进胸,在超声引导下经右室流出道置入球囊扩张管扩张肺动脉瓣或经右心房置入封堵器。2例多发室间隔缺损于体外循环下经三尖瓣置入封堵器。合并的其他心脏病变同期常规外科矫正,术式包括部分性肺静脉异位引流矫正、动脉导管结扎、冠状动脉旁路移植术等。术后心脏超声随访。结果患者全部顺利出院。1例婴儿重度肺动脉瓣狭窄术后2个月接受常规右室流出道成形术,1例多发肌部室间隔缺损因无法置入封堵器而转为常规术式。其余患者均顺利实施Hybrid术式。随访期内未发现介入器材相关的并发症。结论“复合”Hybrid技术可以避免体外循环,减少手术创伤,对于提高先天性心脏病的疗效具有重要的意受．     

Perventricular Device Closure of Residual Muscular Ventricular Septal Defects after Repair of Complex Congenital Heart Defects in Pediatric Patients

Residual muscular ventricular septal defects are surgical challenges, especially after the repair of complex congenital heart defects. We investigated perventricular device closure as a salvage technique in pediatric patients who had postoperative residual muscular ventricular septal defects.From February 2009 through June 2011, 14 pediatric patients at our hospital had residual muscular ventricular septal defects after undergoing surgical repair of complex congenital heart defects. Ten patients met our criteria for perventricular device closure of the residual defects: significant left-to-right shunting (Qp/Qs >1.5) or substantial hemodynamic instability (a defect ≥2 mm in size). The patients'' mean age was 20.4 ± 13.5 months, and their mean body weight was 10 ± 3.1 kg. The median diameter of the residual defects was 4.2 mm (range, 2.5–5.1 mm).We deployed a total of 11 SQFDQ-II Muscular VSD Occluders (Shanghai Shape Memory Alloy Co., Ltd.; Shanghai, China) in the 10 patients, in accord with conventional techniques of perventricular device closure. The mean procedural duration was 31.1 ±9.1 min. We recorded the closure and complication rates perioperatively and during a 12-month follow-up period. Complete closure was achieved in 8 patients; 2 patients had persistent trivial residual shunts. No deaths, conduction block, device embolism, or other complications occurred throughout the study period.We conclude that perventricular device closure is a safe, effective salvage treatment for postoperative residual muscular ventricular septal defects in pediatric patients. Long-term studies with larger cohorts might further confirm this method''s feasibility.Key words: Cardiac surgical procedures/instrumentation/methods, equipment design, heart defects, congenital/adverse effects, heart septal defects, ventricular/complications/pathology/therapy, hemodynamics, postoperative complications, prostheses and implants, surgical procedures, minimally invasive/methods, treatment outcomeMuscular ventricular septal defects (VSDs) constitute approximately 20% of all VSDs¹ and are possibly associated with other complex congenital heart conditions, such as tetralogy of Fallot and double-outlet right ventricle.² Surgical closure is the mainstay treatment for most VSDs; however, muscular VSDs cannot be approached adequately without a ventricular incision, especially when associated with a large left-to-right shunt, pulmonary hypertension, or interventricular septal hypertrophy.^3–5 After complex congenital heart defects with severe left-to-right shunting are repaired, residual muscular VSDs can significantly increase the patient''s risk of death.^4,5 Particularly in pediatric patients, postoperative residual muscular VSD poses a surgical challenge. Open surgical repair is associated with repeat cardiopulmonary bypass (CPB), the risk of ventriculotomy because of an inadequate operative field of vision, and postoperative ventricular dysfunction.^5,6 Fluoroscopically guided transcatheter device closure is another treatment option; however, restricted vascular access and the technical difficulty of this procedure are well documented in pediatric patients. Radiation exposure and hemodynamic instability during device positioning can also compromise the prognosis.^2,7 Perventricular device closure of VSDs with transesophageal echocardiographic (TEE) guidance was first reported by Amin and colleagues.⁸ This method enables direct access, which is especially suitable in closing apical muscular VSDs.⁹ We investigated the feasibility of perventricular device closure as a salvage procedure in pediatric patients who had residual muscular VSDs after having undergone repair of complex congenital heart defects.     

Sustained monomorphic ventricular tachycardia associated with unrepaired double-chambered right ventricle.

Double-chambered right ventricle (DCRV) is a congenital anomaly characterized by the division of the ventricular cavity into two chambers separated by an abnormal hypertrophied muscular band or by severe hypertrophy of the muscle wall. Two adult patients with a diagnosis of DCRV presented sustained monomorphic ventricular tachycardia. In both cases, the clinical tachycardia was induced with programmed stimulation. After surgically resecting, the muscular band tachycardia could no longer be induced in the patient who underwent follow-up electrophysiological study. The outcome was favourable; there was no clinical recurrence of ventricular tachycardia in the two patients at 48 and 36 months, respectively.