先天性巨结肠根治术后医源性直肠阴道瘘的再手术治疗CSCD

Objective To explore the causes of iatrogenic rectovaginal fistula after pull-through in Hirschsprung’s disease (HD) and summarize the experiences and efficacies of reoperation. Methods From December 2007 to December 2020 , retrospective review was conducted for clinical data of 6 HD children with iatrogenic rectovaginal fistula after pull-through. The surgical procedures for repairing rectovaginal fistula included transabdominal and transanal Soave , transperineal or transanal surgery. Results A total of six girls were included. Clinical manifestation was vaginal excretion. Injuries occurred in initial surgery (n =4) and during redo pull-through (n =2). The reasons for re-operation were postoperative abdominal hemorrhage and aganglionic segment residue. Rectovaginal fistula with anastomotic retraction and stenosis were successfully repaired by transabdominal and transanal Soave procedure in single time(n =4). The remaining two cases underwent simple local repair of rectovaginal fistula , including fistula closure after anal repair twice (n =1) and failed closure after five local (perineal/anal) repairs (n =1). Conclusion Rectovaginal fistula after pull-through in HD is a serious iatrogenic injury. It should be separated close to rectal submucosa/rectal wall to avoid vaginal injury. Transabdominal and transanal Soave procedure has a high success rate for repairing rectovaginal fistula and managing anastomotic retraction and stenosis. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

胆道闭锁Kasai术后肝脏纤维化及生化指标变化趋势的横断面研究

目的 研究胆道闭锁患儿Kasai术后肝脏纤维化及生化指标的变化趋势.方法 采用横断面研究方法,对2013年1月至2013年12月于首都医科大学附属北京儿童医院普外科随访的胆道闭锁Kasai术后患儿进行复查资料收集,术后随访时间6个月以上,资料完整的121例纳入研究.按照Kasai术后胆红素降至正常的时间,以术后3个月、6个月为界分为优、良、差三组,获取患儿术时年龄、术前和术后Fibroscan肝脏硬度测量值及生化结果,绘制趋势图并进行统计学分析研究各组患儿Kasai术后肝脏纤维化及生化指标的变化趋势.结果 纳入本研究胆道闭锁患儿共121例,男69例(57.0％),女52例(43.0％);其中优组63例(52.1％),良组34例(28.1％),差组24例(19.8％).三组患儿术时年龄差异无统计学意义.三组患儿Fibroscan肝脏硬度测量值在术后1年内波动进展,1年后趋于稳定;单因素方差分析及秩和检验结果显示优良组差异无统计学意义,优良组与差组患儿Fibroscan肝脏硬度测量值在术后1年内差异均有统计学意义.优良组患儿总胆红素在术后前3个月下降较快,随后缓慢下降,至术后6个月稳定于正常值,而差组患儿总胆红素术后前2个月下降较快,但随后出现较大波动.三组患儿ALT趋势图均在术后1个月出现波峰,优良组术后1～6个月有所下降,但之后持续波动不能稳定,差组持续波动;三组患儿AST术后6个月内有所下降,但之后波动较大不能稳定.三组患儿CGT术后1个月达到最高峰,至术后6个月下降较快,随后逐渐趋于稳定小幅波动.结论 ①胆道闭锁患儿Kasai术后1年内肝脏纤维化波动性进展,自肝存活超过1年者肝纤维化情况进展缓慢趋于稳定,优组患儿在2年后更加稳定;②Kasai术后1个月内胆红素下降最快,3～6个月胆红素可降至正常的患儿,其胆红素水平可维持长期正常;③肝脏炎症、胆管反应、功能损害在术后仍继续进展,术后1个月开始减轻,术后6个月趋于稳定.     

7岁以上先天性肌性斜颈的单切口手术治疗

目的探讨7岁以上先天性肌性斜颈单切口手术治疗的疗效。方法15例先天性肌性斜颈患儿,年龄7-15岁、平均9.8岁。右侧10例,左侧5例。所有患儿均行单切口胸锁乳突肌远端松解;术后尽早活动锻炼同时结合佩带支具保持在矫枉过正姿势。结果术后外观及颈部活动受限取得明显改善,斜视和面部发育异常逐步近于正常,满意率达到93%。结论7岁以上先天性肌性斜颈患儿采用单切口手术治疗以及术后积极的康复锻炼也可获得满意的疗效。     

儿童脾脏良性占位性病变的外科诊治

目的 总结儿童脾脏良性占位性病变的临床特点.方法 回顾我院普外科自1988年1月至2011年12月收治的19例脾脏良性占位患儿(男12例,女7例,平均年龄10岁)的资料,分析其临床表现、诊断及治疗.结果 19例患儿中有8例为良性肿瘤,其中血管瘤居多.其余11例多为真性囊肿.14例行脾切除术,5例行部分脾切除术,患儿术后恢复良好.结论 儿童良性脾脏占位性病变主要依靠影像学诊断,主张行保脾为主的手术治疗,手术方式选择取决于肿物的性质及位置.     

无肛术后残留直肠尿道瘘手术方法的选择和治疗效果

目的 总结治疗无肛术后残留直肠尿道瘘手术方法 的选择和临床效果.方法 对28例无肛术后残留直肠尿道瘘,按肛门外观和功能不同,采取两种手术方法 :肛门外观和功能良好的22例采用肛门前会阴矢状入路直肠尿道瘘修补、肛门成形术,肛门外观和功能较差(直肠回复严重5例及肛门开口前移1例)的6例采用后矢状入路直肠尿道瘘修补、肛门成形术,修补直肠尿道瘘,同时肛门成形.结果 无肛术后残留直肠尿道瘘存在明显的瘘管样结构,管长约(0.8±0.3)cm,瘘管的尿道开口大都位于尿道膜部.27例尿道瘘修补Ⅰ期愈合.25例获得随访,尿道无狭窄,无憩室;肛门功能临床评分21例优,4例良,与术前比较意义有差异统计学意义(P＜0.05).两种手术的手术时间分别为(72.8±11.2)min, (105.6±14.6)min(P＜0.05). 结论 通过肛门前会阴矢状入路和后矢状入路修补无肛术后残留的直肠尿道瘘,成功率高,肛门功能明显提高.肛门前会阴矢状入路尿道瘘修补术术野清楚,操作方便.     

儿童终末期肝病评分在胆道闭锁患儿肝移植中的应用

儿童终末期肝病(pediatric end-stage liver disease,PELD)评分系统是根据客观的实验室检查数据评估儿童肝脏疾病严重程度的模型,国际上用它来分配日益减少的供肝给儿童肝移植受者.对于Kasai手术效果不佳的胆道闭锁(biliary atresia,BA)患儿来说,肝移植成为唯一的选择.本文通过分析PELD评分系统与经典的临床肝功能Child分级、BA肝移植手术时机的选择、活体肝移植(living donor liver transplantation,LDLT)、肝纤维化、葛西手术(Kasai procedure,KP)以及肝移植手术预后之间可能存在的关联,旨在较全面的了解PELD评分在BA患儿肝移植中所发挥的作用,力求使其应用更加科学、合理.     

肝脏硬度测量在胆道闭锁与其他婴儿胆汁淤积鉴别诊断中的应用价值

目的 评价肝脏硬度测量在鉴别胆道闭锁与其他婴儿胆汁淤积时的应用价值,并与生化指标做对比研究.方法 选取2015年1月至2015年12月收治于首都医科大学附属北京儿童医院行手术探查及术后肝脏病理确诊的婴儿胆汁淤积患儿62例,男30例(48.4%),女32例(51.6%),平均手术时年龄(72.7±20.1)d;入组其他婴儿胆汁淤积患儿22例,男21例(95.5%),女1例(4.5%),平均手术时年龄(84.6±41.7)d.回顾性研究患儿术前3 d内的肝脏硬度测量值及生化指标,采用独立样本t检验、ROC曲线分析及配对卡方检验,对比评价肝脏硬度测量在鉴别胆道闭锁与其他婴儿胆汁淤积患儿时的应用价值.结果 比较两组患儿的肝脏硬度测量值、谷丙转氨酶(ALT)、谷草转氨酶(AST)、γ-谷氨酰转肽酶(GGT)、总胆红素(TBIL)、总胆汁酸(TBA),仅肝脏硬度测量值(12.29±6.99比8.05±2.97,P=0.007)和GGT(376.79±346.70比641.70±474.29,P=0.008)差异有统计学意义.对这两项指标进行ROC曲线分析,曲线下面积分别为AUROC(肝脏硬度测量)=0.743,AUROC(GGT)=0.696.取肝脏硬度测量界值8.1kPa时可获得最大约登指数0.427,超过此值提示患儿为胆道闭锁的敏感度0.79,特异度0.64,阳性预测值0.86,阴性预测值0.52;取GGT界值344.15μmol/dl时可获得最大约登指数0.359,超过此值提示患儿为胆道闭锁的敏感度0.68,特异度0.68,阳性预测值0.86,阴性预测值0.43.以手术探查实际结果为标准,分别对肝脏硬度测量(界值8.1kPa)和GGT(界值344.15μmol/dl)进行配对卡方分析(McNemar法),结果肝脏硬度测量8.1kPa(P=0.383),GGT344.15μmol/dl(P=0.019),提示当取肝脏硬度测量界值8.1kPa时对胆道闭锁和其他婴儿胆汁淤积的鉴别判断与实际情况无差异,而取GTT界值344.15μmol/dl时则与实际情况有差异.结论 肝脏硬度测量可较好的应用于胆道闭锁与其他婴儿胆汁淤积的术前鉴别,超过界值8.1kPa时能有效提示患儿为胆道闭锁.     

小儿脾性腺融合二例报告

脾性腺融合是一种罕见的先天性畸形,临床上较易误诊.部分表现为睾丸肿大患者常因考虑为睾丸肿瘤而接受不必要的睾丸切除术.我们收治2例,报告如下.     

先天性肛门直肠畸形术后便秘合并巨结肠的临床特点及处理

目的 总结先天性肛门直肠畸形术后顽固性便秘合并巨结肠的临床特点和治疗效果.方法 回顾性分析2005年8月至2010年7月收治的顽固性便秘合并巨结肠23例临床资料,男10例,女13例.首次手术年龄3 d至6岁,术前诊断均为中低位肛门直肠畸形.23例患儿均以肛门成形术后便秘就诊.下消化道气钡造影显示下端结肠及直肠扩张,无移行段,排钡延迟.本次手术年龄1岁2个月至15岁8个月.均采用Soave巨结肠根治术.结果 术前见肛门外观大致正常13例,外观正常合并直肠尿道瘘1例,肛门开口位置前移6例,瘢痕回缩2例,肛门外口狭窄1例.术中进入盆腔后即为扩张肠管,系膜增生增厚,肠壁血管增生粗大.13例单纯经会阴手术,10例经腹会阴手术,其中1例同时回肠末端造瘘.9例巨结肠切除同时,再次行肛门成形术,1例巨结肠切除同时行前矢状入路直肠尿道瘘修补术.病理检查15例切除肠管远近段,可见神经节细胞者,8例肠管远端无神经节细胞者,23例均见肌层增生肥厚,肌纤维变性,肌层排列紊乱.二次术后随诊肛门功能李氏评分5～6分.结论 先天性肛门直肠畸形术后顽固性便秘往往并发巨结肠改变,影像显示为肠管局限性扩张、僵硬和动力紊乱.采取手术治疗,切除病变肠管,做必要的肛门解剖缺陷修复,术后效果良好.

Abstract：

Objective To summarize the experience of diagnosis and treatment of intractable constipation in children with repaired anorectal malformation.Methods Between August 2005 and July 2010,23 children with a history of anorectal malformation (ARM) repair were diagnosed with intractable constipation,and underwent surgical treatment at this center.The patients,including 10 boys and 13 girls,underwent primary surgeries to repair low or intermediate type of ARMs.Their ages at the primary surgery ranged from 3 days to 6 years old.Physical examinations found normal appearance of anus on 14 children including 1 with rectourethral fistula,anus antelocation on 6,cicatricial retraction of anus on 2,and anal stricture on 1.The positive barium enema revealed the dilated distal colon and rectum without transition zones,and delayed barium transit time.The patients' age at the second surgery ranged from 1 year and 2 months to 15 years and 8 months old.Results Dilated distal colon and rectum,with increased intestinal wall and mesentery thickness as well as proliferated blood vessels,was found during surgery on all patients.However,no transition zone was observed.To remove the dilated intestine,transanal Soave procedure was performed on 13 children,and the other 10 underwent abdomino-perineal Soave procedure including 1 had additional ileostomy.Besides the megacolon resection,9 patients also underwent anoplasty,and rectourethral fistula was also repaired on 1 patient.At the distal end of the removed intestine,pathological examination revealed ganglion cells in 15 patients,and no ganglion cells in 8 patients.Hypertrophy,degeneration and derangement of the intestinal smooth muscle cells were also observed.After reoperation,patients' anal function was graded as 5 or 6 according to Lizheng's anal function scoring criteria.Conclusions Intractable constipation after ARM repair is usually caused by acquired megacolon.It can be cured by megacolon resection.