10岁以内先天性脊柱侧凸的术式探讨

目的总结10岁以内先天性脊柱侧凸的不同手术方式及疗效,初步探讨该年龄段先天性脊柱侧凸患儿的手术原则。方法选择先天性脊柱侧凸患儿66例,男34例,女32例,年龄1岁1个月至9岁10个月,平均年龄3岁8个月。其中半椎体畸形22例,半椎体并楔形椎体28例,凹侧存在骨桥或同时存在半椎体l1例,椎体畸形伴多发肋骨畸形5例。行后路原位融合术3例;前路或前后路骺阻滞融合术10例;前路半椎体切除、后路Apofix内固定21例;前路半椎体切除、后路椎弓根钉内固定19例;前路半椎体及相应肋骨切除、植骨融合术2例;前路半椎体切除融合、石膏背心矫形固定3例,其中1例半年后改行后路椎弓根钉内固定术;脊柱凹侧截骨撑开2例;前路半椎体切除骺阻滞、后路凹侧皮下撑开术3例．胸腔扩大成形术3例。通过术前、术后、随诊时站立位全脊拄X线片测量冠状面Cobb角,比较不同手术方式的脊柱侧凸矫形效果。结果全部病例均获随访,随访时间6～28个月,平均13个月。冠状面Cobb角得到较大矫正,矫正率为49％．脊柱侧凸畸形获得有效控制。结论10岁以内先天性脊柱侧凸畸形复杂,不同年龄及不同类型患者采用不同的手术方式,可取得较好的临床疗效。     

婴幼儿先天性脊柱侧后凸畸形的手术治疗

目的总结婴幼儿先天性脊柱侧后凸畸形的手术治疗经验。方法回顾性分析1999年10月~2006年12月经手术治疗的20例先天性脊柱侧后凸畸形病例资料。其中男8例,女12例。年龄11个月~3岁8个月。根据Winter及MCmaster分型,前方和单侧形成不全(后外侧1/4椎体)9例,前方形成不全(后方半椎体)7例,凸侧半椎体凹侧骨桥4例。脊柱侧凸Cobb角30°~50°,后凸Cobb角36°~56°。手术方法:4例行单纯脊柱后路短段融合术;6例经脊柱后路凸侧椎弓根行半椎体半骺切除 融合术;2例行侧前路椎体骺板及半锥体切除术;8例行脊柱后路短段融合 钢丝襻及钩棒系统矫形固定术。结果全部病例术后脊柱侧凸及后凸畸形部分矫正或原位融合,无并发症。经1~7年随访,5例脊柱侧后凸减轻;8例脊柱侧后凸无明显变化;2例脊柱畸形加重半椎体复出;3例假关节形成;2例钩棒脱出,无钢丝断裂。结论婴幼儿脊柱柔韧,可塑性强,在畸形尚未加重之前可通过脊柱后路手术矫正并控制脊柱畸形的进展,用钢丝襻固定可及时矫正畸形,稳定脊柱。近期疗效满意,远期疗效有待继续随访。     

脊柱前后入路治疗先天性脊柱侧凸

为了提高手术治疗小儿先天性脊柱侧凸的矫正率，减少术后畸形复发和曲轴现象的发生，采用脊柱前后入路手术治疗小儿先天性脊柱侧凸１５例。平均手术年龄９岁，Ｃｏｂｂ角平均８０°，平均随访４年。与同期２５例因先天性脊柱侧凸行单纯脊柱后路矫形术患儿进行术后平均矫正率、矫正丢失度／年、有无曲轴现象等临床指标比较。结果显示脊柱前后入路组治疗效果明显优于单纯脊柱后路组。对患有严重脊柱侧凸、弧度僵硬或并发后凸的患儿，宜行脊柱前后入路手术，对已行单纯脊柱后路矫形的患儿，应定期随访，如手术失败或畸形复发，有必要再次行脊柱前后入路矫形融合术。     

先天性单侧肾发育不良的诊治体会(附23例报告)

目的总结先天性单侧肾发育不良的诊治经验。方法回顾性分析23例先天性单侧肾发育不良的临床资料。其中女20例,男3例。年龄2～14岁。合并肾位置异常2例,输尿管异位开口15例,双侧输尿管囊肿1例,输尿管积水3例,膀胱输尿管返流(VUR)1例。对侧重复肾并输尿管异位开口2例,膀胱输尿管返流1例。B超检查21例,IVP检查22例,输尿管逆行造影4例,膀胱镜检查10例,CT检查13例,VCU检查2例。均行手术治疗,其中肾切除19例;输尿管囊肿电切开窗1例;对侧重复肾切除2例;膀胱输尿管再吻合1例。结果全部病例随访6个月～3年,漏尿、发热及尿路感染等症状消失。结论临床症状结合影像学检查是诊断先天性单侧肾发育不良的有效方法,单侧肾发育不良常合并输尿管末端及对侧肾脏病变。治疗上主要是切除发育不良的肾。     

一期手术矫治肾输尿管重复畸形合并异位输尿管囊肿

目的 探讨分析完全性肾输尿管重复畸形合并同侧上输尿管异位囊肿的病人，治疗时是否需要同时切除病变肾和输尿管囊肿。方法 对近8年来我院收治的10名完全性肾输尿臂重复畸形合并同侧上输尿管异位囊肿的病例予以总结。结果 发现其中6名初期治疗中仅切除患侧上半肾输尿管的儿童，平均术后1年都因为膀胱内囊肿增大而再次接受了输尿管囊肿切除术。另3例病人一期同时施行了上半肾输尿管切除术、膀胱内输尿管囊肿切除术和下半肾输尿管再植术，术后随访疗效良好。结论 我们认为对类似疾病应该在切除肾输尿管的同时一并切除异位输尿管囊肿。     

腹腔镜输尿管端侧吻合联合膀胱再植术治疗儿童复杂重复肾畸形

目的探讨采用腹腔镜下输尿管端侧吻合联合输尿管膀胱再植术治疗儿童复杂重复肾畸形的效果及预后情况。方法收集郑州大学第一附属医院2020年6月至2022年6月手术治疗重复肾患儿的临床资料, 选取同侧双输尿管病变的复杂重复肾畸形的病例患儿11例, 其中男1例, 女10例;平均月龄为33.5个月, 范围为3～73个月;重复肾左侧7例, 右侧2例, 双侧2例。术前完善泌尿系B型超声、泌尿系CT血管造影/ CT尿路造影、肾动态核素显像、磁共振泌尿系水成像、排泄性膀胱尿路X线造影等影像学检查, 采用腹腔镜下输尿管端侧吻合联合输尿管膀胱再植术治疗。分析手术时长、出血量、术后住院天数、术前术后肾盂前后径变化、输尿管管径粗细、肾实质厚度、患肾部分肾功能、术后并发症等指标。数据采用配对t检验。结果 11例患儿中上位输尿管末端囊肿5例, 上位输尿管末端梗阻6例(含输尿管开口异位3例), 下位肾积水伴输尿管反流8例, 下位肾积水伴输尿管末端梗阻3例。所有患儿均顺利行腹腔镜下输尿管端侧吻合联合输尿管膀胱再植术, 手术时长为(242.22±93.15) min, 均未中转开放, 术中出血0～20 ml, 均未输血, ...     

严重脊柱侧凸与肠系膜上动脉综合征一例

我科自 1 985年以来 ,手术矫形治疗脊柱侧凸近 40 0余例 ,其中 1例确诊为肠系膜上动脉综合征 ,现报告如下 :患儿 :女 ,1 4岁。因先天性脊柱侧凸 1 4年 ,畸形逐渐加重。近 2个月出现腰痛 ,伴有左下肢疼痛 ,且活动后呼吸困难而收入院。体检 :患儿发育差 ,体质瘦弱 ,身高仅 1 .31m ,脊柱胸段明显左侧凸 ,并呈严重剃刀背畸形 ,脊柱僵硬 ,右侧胸廓塌陷。右下腹触觉迟钝。双下肢病理反射阴性。X线片显示 :先天性脊柱侧凸 ,主弯为T3 T1 2 ,Cobb’s角为1 4 5°。MRI显示 :胸段脊髓空洞。拟行Ⅱ期手术矫形、植骨固定。Ⅰ期经胸前路软组织松解、凸…     

膀胱颈折叠成形术治疗女童短尿道畸形所致尿失禁8例

目的探讨膀胱颈折叠成形术治疗女童短尿道畸形所致压力性尿失禁的临床经验和疗效。方法对本院从2008年5月至2011年2月因尿失禁收治的8例女童施行膀胱颈折叠成形术。8例患儿中,包括先天性短尿道畸形6例,1例合并先天性直肠肛门畸形术后,尿道开口发育差,位置偏后汐p伤性尿道阴道瘘、尿道会师术后尿失禁1例,先天性短尿道畸形合并右侧异位发育不良肾、异位输尿管口1例。结果8例患儿手术顺利,恢复良好,术后12～14d出院。术后1个月、3个月、6个月门诊随访,6例控尿满意,元尿失禁、排尿困难、尿路感染等并发症,1例（先天性短尿道畸形合并右侧异位发育不良肾、异位输尿管E1）术后存在尿失禁,1例（先天性短尿道畸形合并先天性直肠肛门畸形术后）存在部分性尿失禁。8例术后彩超提示膀胱元残余尿、无肾盂积水、膀胱结石等。结论短尿道畸形是女性尿失禁的原因之一,严重影响患儿日常生活,膀胱颈折叠术对短尿道畸形所致尿失禁疗效满意,术式简单安全,并发症少,值得临床推广。     

不同腹腔镜微创术式处理儿童重复肾畸形的研究

目的探讨儿童重复肾畸形的不同腹腔镜微创处理方法。方法 2018年1月至2019年6月,郑州大学第一附属医院小儿外科共收治儿童各类重复肾畸形患儿30例,其中反复泌尿道感染13例,排尿疼痛5例,肾区疼痛5例,非排尿期持续滴尿5例,无症状7例。经术前进行泌尿系彩色多普勒超声、磁共振泌尿系水成像、泌尿系CTU、肾动态显像、排尿性膀胱尿道造影等检查,诊断上位肾积水并输尿管扩张18例,上位肾发育不良伴上位输尿管全程扩张开口异位4例,上下位肾积水伴上下位输尿管均全程扩张3例,下位肾盂输尿管交界处梗阻5例,30例患儿中有输尿管囊肿8例。所有患儿均行腹腔镜微创手术,根据不同情况选用腹腔镜上位肾脏及输尿管切除术、腹腔镜上下位输尿管端侧吻合术、腹腔镜下位肾盂输尿管端侧吻合术、腹腔镜下位肾切除联合上位输尿管膀胱再植术、气膀胱下双根输尿管再植术、膀胱镜下输尿管囊肿基底部电切术等。结果本组所有手术均未中转开放,历时20～280 min,术中出血0～20 ml,均未输血。所有患儿随访6～12个月,11例上位肾切除术后下位肾血供好,滴尿症状完全消失,泌尿道感染无复发;5例上下位肾盂输尿管和输尿管之间端侧吻合术后肾积水...     

Appplication of end-to-side ureteroureweterostomy via incision of inguinal striae in children with complete duplication anomalies

目的 评价经腹股沟皮纹切口输尿管端侧吻合术治疗儿童完全性重肾畸形的手术疗效.方法 从2008年1月至2010年12月间用同侧输尿管端侧吻合术治疗完全性重肾畸形患儿16例(17侧),女14例,男2例,年龄为4个月至12岁.患儿主要表现发热性尿路感染或正常排尿间歇滴尿等症状.经术前影像学检查明确输尿管囊肿8例,膀胱输尿管反流8例,输尿管开口异位5例(1例为双侧).其中上输尿管囊肿同时存在反流3例,上输尿管囊肿伴下输尿管反流2例,单纯输尿管囊肿3例,单纯下输尿管反流2例,1例上输尿管曾作过再植手术后仍存在反流.结果 所有患儿手术过程顺利,平均手术操作时间60 min,术后平均住院5d.术后患儿发热性尿路感染或滴尿等症状消失,经术后3～6个月超声、同位素肾图等检查,患肾功能改善,反流消失,肾积水缩小,吻合口无梗阻.结论 经腹股沟皮纹切口输尿管端侧吻合术治疗重肾畸形输尿管囊肿、反流及开口异位是一种合适方法.手术操作过程简单、省时,腹股沟皮纹切口美观,此外,手术创伤小,并发症少,缩短了住院时间,还可作为重肾输尿管手术后再次手术的方法.     

New treatment concept for children with thoracic insufficiency syndrome due to congenital spine deformity

BACKGROUND: Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will inevitably develop thoracic insufficiency syndrome and curve progression with hemithorax compression without treatment. It is assumed that the concave side of such curves and their unilateral unsegmented bars do not grow. In the past early spinal fusion was performed with consecutive short thoracic spines and loss of lung volume. Little attention has been paid to lung function. These patients often suffered from lung failure and early death due to a small thorax. METHOD: A new surgical technique is based on an indirect deformity correction and enlargement of the thorax due to a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The spine is not fused, thus promoting growth of the spine, the thorax and the lungs. Elongation of the implant is done every six months. Since 2002 this method has been performed on fifteen children in Basel as the first European center. RESULTS: Patients (mean age 6 years; 11 months to 12 years) were suffering from thoracic insufficiency syndrome due to unilateral unsegmented bars with fused ribs (n = 4), absent ribs (n = 2), bilaterally fused ribs (n = 2), hemivertebrae (n = 3) or neuromuscular scoliosis (n = 6). Doing fifteen primarily implantations and thirteen elongations there were three complications (two hook dislocations, one skin breakage). All patients improved cosmetically, functionally and radiologically which was shown on X-rays as a reduction of the Cobb angle from an average of 76 degrees (40-110 degrees ) to 55 degrees (30-67 degrees ). CONCLUSIONS: Expansion thoracoplasty and VEPTR implantation is a new treatment concept for children with thoracic insufficiency syndrome due to spinal deformities, which is based on distraction and expansion of the thorax thus allowing growth of the spine, the thorax and probably lungs. Presently it seems to be superior to any other method for the treatment of small children with progressive scoliosis and thoracic insufficiency syndrome.     

小儿脊柱侧弯并发剃刀背畸形的矫治

目的 矫正脊柱侧弯患儿的肋骨剃刀背畸形。方法 在实行后路脊柱侧弯矫形及脊柱融合时,采用凸侧多节短段肋骨切除术同时矫治剃刀背及术后佩带矫形石膏或支具背心,更为有利。结果 1997年9月至1999年6月共有49例脊柱侧弯患儿施行剃刀背矫形术,未发生气胸。胸膜破裂等并发症,有3例患儿遗有残余剃刀背,其余患儿矫形效果均满意。结论 多节短段肋骨切除术是一种简便有效、安全的方法。在矫形剃刀背的同时,还可提供脊柱融合的植骨材料,免去髂骨取骨。     

纵向可延长钛金属肋骨假体技术治疗先天性脊柱侧弯合并胸廓畸形

目的 报道我们采用纵向可延长钛金属肋骨假体(VEPTR)技术治疗先天性脊柱侧弯合并胸廓畸形的初步体会.方法 5例先天性脊柱侧弯合并胸廓畸形的患儿,男3例,女3例,平均年龄8.3岁(4.7～12.2岁);均自出生就发现脊柱畸形并呈进行性加重.除1例曾经接受过弧度段脊柱张力钢丝捆绑治疗外,其余病例均无过去手术史;所有病例体格矮小、躯体平衡失调并倒向凸侧;肺功能检测明显低下,其中最大肺活量(VCMAX)和最大自主通气量(MVV)分别只有同年龄、同身高正常预测值的24.8%～48.1%(平均32.8%)和20.8%～54.4%(平均34.5%);胸段脊柱混合型分节、分化不良,3例存在凹侧肋骨融合畸形;术前Cobb's角平均为77.6°(63°～106°);CT及CT三维重建测量,脊柱旋转(SR)平均为19.6°(11.4°～26.8°),胸廓旋转(TR)平均为61.4°(34.2°～477.2°),后半胸廓对称度(PHSR)平均为2.4(1.2～3.6);MRI脊髓扫描未发现异常.手术在神经监护仪下进行,所有病例均I期完成手术,4例进行了肋骨开放截骨,术中4例胸膜壁层破裂,所有病例胸膜脏层均保持完整.所有病例均各安置了肋骨-肋骨、肋骨-脊柱钛肋各一组.结果 所有病例均获得随访,平均随访时间14个月(12～18个月),所有病例均已完成了2次延长.术后所有病例躯体平衡获得明显改善,凹侧胸廓扩大,骨盆倾斜平衡恢复,脊柱高度增加,身高平均增长4.4 cm(3～7 cm).所有患儿肺功能检查均较术前有不同程度提高,VCMAX和MVV平均增加35 7%(10.4%～78.4%)和51.7%(8.2%～84.9%).呼吸系统易患感染、活动后气急等现象明显改善.脊柱畸形均有所矫正,Cobb's角测量平均57°(44°～69°),家长对患儿外观改变均表示满意.结论 VEPTR技术对于治疗先天性脊柱侧弯合并胸廓畸形所致的胸腔功能不全综合症显示了一定的效果,肺功能改善,脊柱畸形控制,脊柱生长继续,躯体平衡恢复.其中长期功效和并发症尚待观察.     

Noncardiac comorbidities of congenital heart disease in children

BACKGROUND: Noncardiac conditions may complicate heart disease care, contribute to the progression of the disease and alter the response to treatment. The aim of this prospective study was to evaluate the proportion of cardiac children with such significant conditions. METHODS: Of the 1058 children with congenital heart disease primarily diagnosed during a 10-year-period in one hospital, we identified those patients who had significant congenital and acquired comorbidities. RESULTS: Associated problems were diagnosed in 224 children (21.2%). Among them, 118 children (11.2%) had genetic or syndromic conditions, of which 38 had Trisomy 21. Six subspecialty areas accounted for the vast majority of the cases: neurology (n = 140), pulmonology (n = 36), orthopaedics (n = 26), nephro-urology (n = 19), gastroenterology (n = 14) and endocrinology (n = 13). The most frequent associated conditions were mental retardation, asthma, epilepsy and scoliosis. During the study period, death occurred in 46 of the children (4.3%) and was not related to the cardiac disease in 20 cases (1.9%). CONCLUSION: A substantial proportion of children with congenital heart disease have significant noncardiac comorbidities. Close collaboration between paediatric cardiologists and paediatricians of other subspecialties is imperative to optimize care for these children.     

Congenital Anomalies of the Elbow Joint: Clinical Features and Classification

In order to examine the distribution of different types of congenital anomalies of the elbow joint and discuss their classification, a total of 94 patients, 96 congenital anomalies of the elbow joint, were analyzed. They were classified according to the elbow deformity itself as follows; radio-ulnar synostosis in 59 cases (61%), radial head dislocation in 23 cases (24%), ankylosis of the elbow joint in eight cases (8%), severe flexion contracture of the elbow joint in three cases (3%), and complete or partial aplasia of the elbow joint in three cases (3%). On the other hand, congenital elbow deformities can be classified into three types according to the associated anomalies. In 78 patients, in which congenital elbow deformity appeared as an independent deformity, there were radio-ulnar synostosis in 56 patients, radial head dislocation in 19 patients, ankylosis of the elbow joint in two patients, and hypoplasia of trocholea in one patient. In the 13 patients, which congenital elbow deformity appeared as a part of congenital upper limb anomalies, the underlying congenital anomalies of the upper limb were ulnar deficiency in eight patients, radial deficiency in two patients, transverse deficiency in two patients, and phocomelia in one patient. In five patients, elbow deformity appeared as a part of congenital syndromes. Classification according to associated anomalies seems to be more suitable in order to examine the real distribution of different types of congenital deformities of the elbow joint as a part of the upper limb.     

Lung function in congenital and idiopathic scoliosis

The vital capacity (VC) in patients with congential scoliosis was compared with the VC in idiopathic scoliosis. For any given Cobb angle (a measure of spinal angulation) the loss in VC was approximately 15% greater in congenital scoliosis than in idiopathic scoliosis. The greater impairment of lung function in congenital scoliosis may be due to the associated rib deformity or to an underlying lung anomaly.     

河南漯河地区儿童先天性脊柱侧弯患病率调查

目的 了解河南省漯河地区儿童先天性脊柱侧弯患病率及治疗现状.方法 采用分层抽样的方法从漯河地区36所公立幼儿园及小学3～10岁84 325儿童中随机选出15 000名,在其父母签署知情同意书后进行体检,初步筛选出疑似病例.再对疑似病例进行影像学检查确诊为先天性脊柱侧弯.结果 最终有14 326名儿童参与调查,应答率为95.51％.其中,236例儿童被物理体检出脊柱侧弯,233例进行了全脊柱X线正、侧位片检查,38例进行了MRI和CT检查,确诊先天性脊柱侧弯患儿29例(女21例、男8例).先天性脊柱侧弯患病率为2‰(29/14 326),其中女童患病率为2.9‰(21/7 095),男童患病率为1.1‰(8/7 231),两者比较,差异有统计学意义(P＜0.05).筛查出先天性脊柱侧弯的年龄主要集中在3～4岁(58.62％).畸形类型:半椎体畸形15例、单侧骨桥畸形9例、一侧骨桥合并对侧多节段半椎体畸形4例、L1-4椎体分节不全1例,以半椎体畸形最为多见(51.72％).畸形部位:中上胸椎(T1-8)5例、胸腰段(T9～L2)21例、腰段(L3-5)3例,畸形主要位于胸腰结合部72.41％(21/29).侧凸Cobb角:10～19度16例,20～39度8例,≥40度5例,脊柱侧弯Cobb角以10～19度最多(55.17％).该人群先天性脊柱侧弯知晓率31.03％(9/29),有效治疗率6.90％(2/29).结论 漯河地区儿童先天性脊柱侧弯发病率为0.2％,高于目前报道的全国平均水平(0.1％),女童发病显著高于男童,畸形类型以半椎体多见,部位以胸腰段最多.知晓率、治疗率偏低,先天性脊柱侧弯得到正确治疗的情况不容乐观.     

Long-term follow-up of lung function and standing height following scoliosis surgery in children and adolescents

Previous investigations have reported conflicting results on the development of lung function and body height after operative correction of scoliosis at young age. Partly the number of patients or the duration of observation were relatively small. 120 patients were followed for at least 10 years (10-14, average 12 years) after surgical correction of scoliosis at the age of 18.2 +/- 6.8 years. Standing height and vital capacity were measured 1, 2, 5 and 10 years after operation. Height was expressed as percentile for age and vital capacity as % expected for actual height. The primary diagnoses were idiopathic scoliosis (n = 84, average Cobb angle 90 degrees), congenital scoliosis (n = 20, 83 degrees), poliomyelitis (n = 10, 128 degrees), neurofibromatosis (n = 6, 101 degrees). We found an initial decrease in lung function after 1 year, which was probably due to post-operative immobilization and increased by the gain in body height achieved by the operation. During the rest of the observation period a significant (p less than 0.05) increase of vital capacity (% of expected for actual height) was observed. The gain was maximal for idiopathic scoliosis (+6%). In congenital scoliosis body height %ile and relative vital capacity appeared fixed at the preoperative level. Interestingly patients with an initial vital capacity below 50% expected (n = 29) or preoperative Cobb angle above 90 degrees showed the best results. They also lacked the initial deterioration of lung function in the first postoperative year and had a significant gain of vital capacity (+14%) over 10 years.(ABSTRACT TRUNCATED AT 250 WORDS)     

先天性脊柱畸形合并泌尿生殖系统畸形发病情况及相关因素分析

目的 了解先天性脊柱畸形患儿中泌尿生殖系统畸形的发生率并探讨其与多种因素之间的联系.方法 选取2003年3月至2008年11月入院治疗的425例先天性脊柱畸形患儿,术前均行腹部B超了解泌尿生殖系统畸形情况、行脊柱CT了解脊柱畸形及脊柱内神经畸形、行心电图除外心脏异常,出现阳性结果行MRI及超声心动网以确诊.结果 先天性脊柱畸形患儿中泌尿生殖系统的发生率为11.8%(50/425),其发病与胎次、母亲年龄、出生地差异、脊柱畸形的分类、侧弯部位、侧弯方向以及是否合并心血管畸形、椎管内神经系统畸形均无统计学关系.泌尿生殖系统畸形的患儿出生时父亲年龄较无泌尿生殖畸形的患儿父亲年龄大(P=0.018),合并泌尿生殖系统畸形的患儿较易合并肋骨畸形(P=0.011).结论 先天性脊柱畸形患儿合并泌尿生殖系统畸形的发生率较高,需引起临床高度重视,从而给予适当处理.