反射亢进型神经源性尿失禁的外科治疗及康复训练

目的：探讨反射亢进型神经源性尿失禁的外科治疗及康复训练方法。方法：于1992年1月至2000年10月治疗81例反射亢进型神经源性尿失禁患儿，男54例，女27例，年龄4～14岁，术前检查均证实盆底肌松弛，膀胱容量较小。采用双侧髂腰肌加强盆底肌，回肠去粘膜带血管蒂浆肌层补片的基础上，行膀胱扩大术进行治疗，其中5例行锤状肌膀胱颈悬吊。结果：81例随访6个月～7年，尿失禁症状明显改善63例，术后经过严格的训练有改善的18例。但其中2例效果较差。结论：双侧髂腰肌加强盆底肌，回肠去粘膜带血管蒂浆肌层补片加强膀胱逼尿肌基础上行膀胱扩大术是治疗小儿反射亢进型神经源性尿失禁的较好方法，术后必须进行正规的康复功能训练。     

腰骶部脊髓脊膜膨出并发神经源性膀胱的临床研究

随访，复查腰骶部囊性脊柱裂术后患儿，观察神经源性膀胱发病情况。方法：对３８例行尿流动力学，排尿性光膀胱尿道造影，Ｂ超及静脉尿路造影检查。结果；１．脊髓脊膜膨出占囊性脊柱裂的６２％，脊髓脊膜同并发神经源性膀胱发病率为９６％；２．骨质缺损≥１．５ｃｍ×１．５ｃｍ多为脊髓脊膜膨出。３．共有８例上尿路功能受损，残余尿量均≥６０ｍｌ，其中４例充盈期膀胱内压力≥１．９６ｋＰａ，而３例膀胱逼尿肌－尿道括约肌     

神经源性膀胱患儿回肠浆肌层膀胱扩大术后长期随访

目的 探讨回肠浆肌层膀胱扩大术治疗神经源性膀胱患儿的远期疗效.方法 应用回肠浆肌层膀胱扩大术治疗神经源性膀胱患儿32例.男23例,女9例;8例术前合并双肾输尿管积水及双侧输尿管扩张,3例单侧膀胱输尿管返流,2例有轻度肾功能损害.对比手术前后主观症状[国际尿失禁咨询委员会问卷简表(ICI-Q-SF问卷)]、尿流动力学检查、泌尿系超声及逆行膀胱造影,评价术后疗效.术后所有患儿定期复查血电解质、肾功能及泌尿系超声,监测并发症.结果 随访5～12 a,26例(81.25%)临床症状好转或痊愈,6例(18.75%)无明显改善.术前ICI-Q-SF问卷评分为(18.1±1.0)分,随访结束为(7.8±2.5)分,二者比较差异有统计学意义(t=14.688,P=0.000).尿流动力学检查显示术后最大膀胱容量、膀胱顺应性较术前明显增加,充盈末逼尿肌压较术前降低.术后电解质、肾功能均正常.远期并发症5例:4例并症状性泌尿系感染,1例并膀胱结石.结论 回肠浆肌层膀胱扩大术治疗神经源性膀胱患儿并发症少,远期疗效比较理想.     

小儿神经源性膀胱诊断与治疗

神经源性膀胱是指与排尿功能有关的中枢或周围神经受到损伤引起的排尿困难。可表现为逼尿肌和括约肌过度活动和(或)功能低下,导致尿失禁,膀胱出口梗阻、膀胱输尿管反流,反复泌尿系感染,甚至肾功能衰竭。对于神经源性膀胱患儿首选保守治疗,清洁间歇导尿是治疗神经源性膀胱的重要方法。另外还有膀胱功能训练、药物治疗及电刺激神经调控等;对于保守治疗无效的患儿,如有适应证可考虑手术治疗。     

骶神经调节治疗青少年神经源性膀胱二例报告并文献复习

目的 探讨骶神经调节对青少年神经源性膀胱的疗效及安全性.方法 回顾性分析2013年6月至2013年11月收治的2例青少年神经源性膀胱应用骶神经调节技术治疗的临床资料,并结合文献复习讨论.2例患儿均表现为排尿费力,伴尿频及便秘,1例残余尿量120 ml,另1例残余尿量360 ml;尿动力学检查结果为逼尿肌收缩乏力.分别进行骶3神经电极植入体外测试4周,经排尿日记及尿动力学参数评估,均获得明显改善,随后行刺激器永久性植入术.结果 术后患儿排尿费力、尿频及便秘症状明显减轻,2例患儿残余尿量分别降至20 ml和50 ml.排尿日记及尿动力学参数评估(排尿量、最大尿流率、逼尿肌收缩压)较术前好转.分别随访6个月和11个月,疗效稳定,未见不良反应.结论 骶神经调节可以改善青少年神经源性膀胱的排尿及便秘症状,安全性高,但仍需大样本随机对照研究来确定此技术对儿童神经源性膀胱的长期疗效及安全性.     

神经源性膀胱扩大术远期疗效观察

目的 评价保留膀胱黏膜的双层肠管浆肌层膀胱扩大术的远期疗效.方法 病例选择条件:术前有明确支配膀胱的神经性损害,膀胱容量明显小于同龄儿童的正常值,同时伴有Ⅳ度以上膀胱输尿管反流,经过一段时间的口服抗胆碱能药物、清洁间歇导尿等保守治疗后,膀胱内压仍然较高,输尿管反流无明显改善,我们对符合上述条件的75例神经源性膀胱患儿行切除部分逼尿肌保留膀胱黏膜的双层肠浆肌层膀胱扩大术,同时根据患儿的具体情况选择性联合应用膀胱输尿管移植抗反流、膀胱颈紧缩、膀胱颈悬吊等手术方式,术后对患儿进行长期随访,对手术前后临床症状、肾脏功能、尿流动力学(膀胱容量、残余尿、逼尿肌压和顺应性)等方面进行评价.结果 75例手术患儿中68例获得随访,随访时间平均4.3年,术后无一例发生肾脏功能衰竭,45例获得一定的临床治疗改善.23例术后在尿流动力学、临床症状方面无明显改善.结论 术后膀胱容量不能有效扩大是导致术后疗效不理想的重要原因,保留膀胱黏膜的双层肠浆肌层膀胱扩大术是治疗神经源性膀胱的一种方法,但该方法有待改进.     

尿动力学检查对小儿神经源性膀胱手术方式选择的意义

目的 探讨尿动力学检查对小儿神经源性膀胱手术方式选择的意义.方法 回顾性分析我院手术治疗的神经源性膀胱患儿的资料41例.所有患儿术前完成B型超声、排尿性膀胱尿道造影(voiding cystourethrogram,VCUG)、99mTc-DTPA肾动态显像(DTPA)、尿动力学检查.其中34例行储尿期+排尿期膀胱压力描记.结果 尿动力学检查提示逼尿肌过度活动23例,活动低下11例;括约肌过度活动21例,活动低下13例.根据患儿不同的临床症状,参考尿动力学检查结果,选用不同的手术方式.具体术式包括:回肠代膀胱扩大+阑尾代可控性膀胱流出道(Mitrofanoff)+阑尾代顺行结肠灌洗造瘘(Malone)+膀胱颈悬吊(Sling)2例,回肠代膀胱扩大+Mitrofanoff+ Sling 5例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff+ Sling 7例,回肠代膀胱扩大+Mitrofanoff+右肾切除+Sling 1例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff 18例,回肠代膀胱扩大+Mitrofanoff 2例,输尿管再植+Mitrofanoff 3例,膀胱颈封闭+回肠代膀胱扩大+Malone+回肠卷管代可控性膀胱流出道(Monti)1例,Mitrofanoff 2例.术后39例患儿获得随访,随访时限3个月～5年,7例出现术后并发症,大部分患儿术后恢复满意.结论 小儿神经源性膀胱的手术方式选择主要依据其临床症状以及超声、影像、同位素检查结果,尿动力学检查可明确膀胱尿道功能障碍的类型,对于手术的方案制定也有重要参考意义.     

小儿神经源性下尿路功能障碍的表现及外科治疗现状

神经源性膀胱的治疗是历来己久的临床难题,小儿神经源性膀胱绝大多数是脊髓发育不良(主要是骶髓)和脊髓栓系,常表现为神经源性大小便失禁.     

儿童神经源性膀胱手术治疗进展

儿童神经源性膀胱可以引起尿失禁、尿路感染、上尿路功能损害, 甚至肾衰竭等。主要治疗方针是保护上尿路功能和改善尿失禁症状。对于保守治疗效果不佳以及膀胱功能严重受损的患儿, 以膀胱扩大为主的手术能够有效扩大膀胱容量, 提高生活质量, 保护上尿路功能, 但围手术期和远期并发症较多。为此, 人们尝试过多种方法扩大膀胱容量, 每种方法各有其优点和缺点。本文就儿童神经源性膀胱的手术治疗进展进行综述。     

脊髓栓系综合征合并脊柱、脊髓畸形临床研究

目的探讨脊髓栓系综合征的特征及与脊柱、脊髓畸形的相关性和治疗适应证。方法1989年10月～2003年7月收治脊髓栓系综合征321例。应用Kendall等级相关检验分析手术年龄与术后功能改善的关系。比较临床表现、影像学所见、术中所见和随访结果。结果321例患者中，294例合并有腰骶段脊柱裂，85例合并先天性脊柱侧弯，114例合并硬膜内或外脂肪瘤，76例合并脊髓空洞，63例合并脊髓纵裂，4例合并双重脊髓。主要术式包括：椎板切除、脊髓及神经根粘连松解、终丝切断、脂肪瘤切除、纵隔切除、硬膜重建。对先天性脊柱畸形同时行脊柱后路植骨融合加内固定78例，未行内固定而单纯行植骨融合34例。结论神经症状改善程度与手术年龄相关，年龄越小，改善越明显；肢体运动感觉障碍、上神经源性膀胱和脊柱变形手术治疗效果明显；而下神经源性膀胱、直肠功能障碍和足部畸形改善不明显。     

小儿神经原性膀胱手术前后尿流动力学检查评价

目的　基于手术前后尿流动力学检查结果的分析,对小儿神经原性膀胱逼尿肌和括约肌不协调的成因进行讨论,并对外科治疗进行评价。方法　55例行盆底肌加强及膀胱颈悬吊的神经原性膀胱患儿于术前和术后4～10个月行尿流动力学对照检查,然后分析其结果并行统计学处理。结果　34例逼尿肌反射亢进型中,30例术后逼尿肌和括约肌不协调减轻或较协调,4例无变化。最大膀胱容量增加（89.0±17.2）ml,最大尿道压力下降（2.7±0.37）kPa,最大尿道闭合压下降（3.1±0.6）kPa;21例术后逼尿肌无反射或反射低下型,逼尿肌反射明显改善11例,改善10例,最大膀胱容量增加（55.6±10.3）ml,最大尿道压力增加（2.9±0.7）kPa,最大尿道闭合压增加（2.6±0.7）kPa。结论　盆底肌加强和膀胱颈悬吊手术是治疗小儿神经原性膀胱的良好术式。因腰骶部脊膜膨出所致的小儿神经原性膀胱逼尿肌与外括约肌协同失调本质上可能是尿道外括约肌对漏斗状膀胱颈代偿性收缩的一个表现。     

Catheterisable continent urinary diversion (Mitrofanoff principle)--clinical experience and psychological aspects.

48 Mitrofanoff principles (MTR) were performed on 46 patients (male : female ratio, 30 : 16) with a mean age of 9.1 years (range 2.5 to 24 years). The primary diagnoses were neurogenic bladder in 11, infravesical obstruction in 7 and bladder exstrophy-epispadias complex in 28 patients. The most common type of conduit was appendix (38 cases); other conduits were constructed from the ileum (seven) and ileocaecum (one). In two cases with bladder substitution the uterine tube and tubularised bladder stump were used as perineal MTR. 33 of the 46 children underwent augmentation cystoplasty in conjunction with the MTR procedure. The Malone procedure for antegrade colonic enema (ACE) was performed at the same stage with MTR in eight cases. To achieve continence, bladder neck reconstruction was performed in 32 patients and the bladder neck was closed in four patients during the same operation and MTR procedure. The mean follow-up period was 28.7 months (range one month to 57 months). To assess the psychological aspects of the MTR procedure, the Rosenberg Self-Esteem Scales were completed by 12 children older than eight years of age. Nine patients had problems with the MTR (19.5 %). Three appendiceal MTR had strictures at the skin level, 2 of which needed minor surgical revisions. A mucocele formation at the skin level of an appendix was removed successfully. We did not observe any complaints among the other appendiceal conduits. All the tapered ileum conduits were difficult to catheterise, and 1 of them had a leakage from the stoma. None of the three transversely tubularised ileum MTRs had problems with catheterisation or leakage. A stricture of the conduit from the uterine tube was observed. 36 of the 42 patients are now continent, giving a ratio of 86 %. The results of the Rosenberg Self-Esteem Scales revealed that there was an increase in the percentage of patients with high self-esteem, and a decrease in depressive feelings after the MTR procedure. We conclude that the MTR procedure provides excellent continence, offers good prospects of a socially acceptable life with increased self-esteem, and the appendix seems to be the ideal organ for continent urinary diversion, with the transverse tubularised ileal tube as a second choice.     

腹部神经母细胞瘤切除的标准化方法

目的探讨一种有效切除腹部神经母细胞瘤(NB)的方法和程序。方法收集2005年3月-2011年8月本院小儿外科收治且手术的腹部NB患儿40例。年龄6个月～13岁2个月。国际NB系列研究组织(INSS)分期:Ⅰ期4例,Ⅱ期3例,Ⅲ期13例,Ⅳ期20例。对所有患儿进行术前CT影像评估、一致的肿瘤切除方法即骨骼化血管和网格样切除、腹膜后淋巴结清扫3个程序,对其结果进行分析。结果肿瘤的完全切除率为97.5%,10例患儿左肾或右肾被切除,手术持续时间1 h 5 min～10 h 50 min,平均4 h 22 min;术中出血量为50～1 200 mL,平均400 mL;术中输血0～1 200 mL,平均280 mL。术后7例患儿发生并发症,其中1例患儿死亡,1例患儿右上腹出现少量包裹性积液,2例出现乳糜腹水,3例出现腹泻。无肾萎缩、血管破裂、肠梗阻、切口感染等并发症。结论通过建立标准化的程序,能使大部分患者肿瘤安全、快速和完全地切除,并发症少。腹部NB手术的程序化有利于临床医师更容易掌握这门技术。     

神经母细胞瘤并骨转移患儿临床特征及预后相关因素的单中心分析CSCD

目的分析伴骨转移神经母细胞瘤(neuroblastoma,NB)患儿的临床特征及预后相关因素,总结临床诊疗经验,以提高伴骨转移NB患儿的生存率。方法以2013年12月至2020年12月重庆医科大学附属儿童医院肿瘤外科收治的伴骨转移NB患儿为研究对象,收集并总结患儿临床资料及预后情况。随访时间截至2021年3月31日。结果共收集97例NB患儿,男68例,女29例,男女比例为2.4∶1;中位年龄为49.4个月。首发症状:发热47例(48.5%),骨痛38例(39.2%),腹痛或腹胀29例(29.9%),咳嗽15例(15.5%);伴骨转移的NB患儿存在多类骨转移(62.9%),且合并骨髓转移(73.2%),整体预后差,1年生存率为93.6%,5年生存率仅20.2%。经生存分析发现,女性、伴多类骨转移、肿瘤位于腹部、首诊时LDH测定值大于660 U/L以及术中肿瘤残留是预后不佳的影响因素,其中手术切除范围是独立预后影响因素。结论伴骨转移的NB患儿临床表现多样,其预后受诸多因素影响,肿瘤复发或进展是主要的致死原因。对于此类患儿,建议术中尽可能完全切除肿瘤,以改善预后。     

神经母细胞瘤并骨转移患儿临床特征及预后相关因素的单中心分析

目的 分析伴骨转移神经母细胞瘤(neuroblastoma,NB)患儿的临床特征及预后相关因素,总结临床诊疗经验,以提高伴骨转移NB患儿的生存率.方法 以2013年12月至2020年12月重庆医科大学附属儿童医院肿瘤外科收治的伴骨转移NB患儿为研究对象,收集并总结患儿临床资料及预后情况.随访时间截至2021年3月31日...     

神经源性膀胱伴输尿管反流的尿动力学研究

目的 探讨有输尿管反流的神经源性膀胱(NB)患儿有或没有逼尿肌过度活动(DO)时的尿动力学差异,为临床治疗此类患儿提供理论参考依据.方法 选取2013～2015年就诊并经影像尿动力学检查发现膀胱输尿管反流的NB患儿68例,男30例,女38例,年龄4～12岁,平均7.5岁.按照充盈期有DO,将其分为DO组(n=20)与无DO组(n=48).观察记录两组发生膀胱输尿管反流时的膀胱灌注量、逼尿肌压并计算发生反流时的膀胱顺应性;记录两组充盈结束时最大膀胱测压容量、最大逼尿肌压、并计算充盈期膀胱顺应性.结果 DO组发生膀胱输尿管反流时的膀胱容量与顺应性分别为(98.7±16.1)ml和(5.2±1.9) ml/cmH2O,无DO组发生膀胱输尿管反流时的膀胱容量与顺应性分别为(127.3±36.3)ml,(7.1±2.1)ml/cmH2O,差异均有统计学意义(P＜0.05);两组的逼尿肌压分别为(21.6±9.2)cmH2O、(19.2±7.4)cmH2O,差异没有统计学意义;DO组充盈结束时的膀胱容量与顺应性分别为(182.7±31.2)ml、(5.4±1.7) ml/cmH2O,与无DO组充盈结束时的膀胱容量(230.6±34.6)ml与顺应性(6.5±1.1)ml/cmH2O相比,差异有统计学意义;两组尿动力学检查结束时逼尿肌压分别为(33.8±7.8)cmH2O、(36.4±8.1)cmH2O,差异没有统计学意义.结论 膀胱容量小,膀胱顺应性差是有输尿管反流的NB患儿伴发DO时的尿动力学特征.     

关于神经母细胞瘤外科治疗的几点思考CSCD

神经母细胞瘤手术复杂危险,极具挑战。尽管外科手术的水平高低难以被科学定量评估,且这种艰苦手术对于患儿生存获益的贡献还有不同的意见,但就神经母细胞瘤治疗而言,外科手术仍然是必须的,而且要努力做到更好。临床应努力创新各种技术方法,拓展微创手术,提高神经母细胞瘤的外科治疗效果。     

Outcomes of renal transplantation in children with and without urinary tract abnormalities: is there any difference?

PurposeTo evaluate if children with urinary tract abnormalities, particularly bladder disorders, have different long-term outcome after renal transplantation, compared to those with non-urological cause of ESRD.Material and Methods211 children (age <19 years) were submitted to 226 renal transplantations between 1989 and 2005. They were separated in three groups: 136 children with ESRD due to non-urological cause (GROUP 1), 56 children with urological disorders but with adequate bladders (GROUP 2) and 19 children with lower urinary tract dysfunction associated or not to inadequate bladder emptying (GROUP 3). In group 3, 15 children were submitted previously to a bladder augmentation (ureterocystoplasty in 6 and enterocystoplasty in 9), one to a bladder autoaugmentation, two children to a continent urinary diversion, and one to an appendical Mitrofanoff procedure. The renal transplantation was performed in a classical, extraperitoneal access, and the ureter implanted, whenever possible, with an antireflux technique.ResultsAfter a mean follow-up of 75 months, 13 children died, 59 grafts were lost, and 15 children received a second transplant. Two patients of GROUP 3 required a complementary urological procedure to preserve renal function (one enterocystoplasty and one vesicostomy). There was a total of 12 (5.3%) major surgical complications, with an equal incidence in the three groups. The overall graft survivals at 5 years were 75% (GROUP 1), 74% (GROUP 2) and 84% (GROUP 3).ConclusionsWith an individualized treatment, children with urological abnormalities, including those with lower urinary tract disorders, have the same long-term outcome as those with non-urological cause of ESRD.     

小儿肛门直肠损伤诊治体会

目的 探讨小儿肛门直肠损伤的诊治及影响预后的相关因素。方法 总结近10年小儿肛门直肠损伤32例的诊治体会，着重对手术时机、手术方式选择等影响疗效的相关因素进行分析。结果 本组32例中，男18例、女14例，平均年龄6．5岁(0．5岁～12岁)。车祸伤17例、意外伤8例、医原性损伤5例、烧伤2例。单纯肛门直肠损伤15例，合并伤17例，其中骨盆骨折7例、尿道损伤10例、阴道损伤2例、膀胱损伤2例、脾破裂2例。伤后直接或及时转入我科17例，并接受了急诊手术，在院外已接受手术处理或延期来院15例。接受3次以上手术5例，2例遗留永久性人工肛门，2例轻度污粪，其余排便功能正常。结论 小儿肛门直肠损伤在临床上并非少见，病情较复杂，常有合并伤。其预后除与致伤原因及受伤程度有关外，与早期诊断、及时处理及手术方法的选择密切相关。     

The impact of surgical radicality on outcome in childhood neuroblastoma.

AIM: Improvement of treatment results for neuroblastoma (NB) has been achieved during recent years, especially by intensifying therapy for advanced NB. Surgery, however, has not contributed very much to this progress and there is still controversy regarding the best approach for high-risk NB. We therefore attempted to find criteria for a differentiated strategy for tumour resection in NB. METHODS: We retrospectively analysed the data of 2251 NB patients treated in the German Cooperative NB Studies NB79 - NB97 (1979 - 1999) including patients' age, tumour stage, MYCN oncogene status, surgical intervention, completeness of resection, surgical complications and outcome. RESULTS: 1148 patients had a localised NB (stage 1 - 3), 878 had stage 4, and 225 had stage 4 S disease. 2112 patients underwent surgery. Tumour resection as the final result of primary or delayed operation was complete in 1403 (66.4 %), incomplete in 449 (21.3 %), and only a biopsy was carried out in 260 (12.3 %) cases. Complete resection was performed most often in localised NB (73.5 %), less often in stage 4 NB (59.2 %) and in stage 4 S (54.5 %). Clinically relevant complications occurred in 19.2 % of all operations independent of the time of resection during treatment, patients' age, tumour stage, tumour site and MYCN status. For 1787 patients from the studies NB79 - NB90 the probability of 5-year event-free survival (EFS) could be analysed and correlated with surgical radicality. In localised NB of patients aged > 1 year there was a significant difference in 5-year EFS between complete, incomplete, and no substantial resection ( P < 0.0001), while this was not the case in infants < 1 year. In the early study period (NB79 - NB85) patients with stage 4 NB had a significantly better 5-year EFS after complete excision of the primary tumour. In contrast, this difference was not confirmed during the later period (NB90) with intensified drug therapy. Timing of surgery did not influence prognosis. For infants with stage 4 S NB there was no difference in outcome after complete or incomplete resection of the primary tumour. MYCN status did not alter the significant relevance of tumour resection for outcome in localised NB. In contrast to MYCN non-amplified stage 4 NB, however, stage 4 NB with MYCN amplification had a significantly better outcome if the primary tumour could be completely or incompletely resected, compared to a biopsy only. CONCLUSIONS: Radical tumour resections with the risk of surgical complications are only justified in children > 1 year with a localised NB. In stage 4 NB, the primary tumour may be resected during intensive multimodal treatment without risky complications. Complete or incomplete resection of the primary tumour improves prognosis only in MYCN-amplified stage 4 NB. Stage 4 S NB with non-amplified MYCN are subject to spontaneous regression; a resection after chemotherapy may be indicated in cases of non-sufficient regression or growth of the primary tumour.