腹腔镜辅助治疗Currarino综合征的研究及中期功能评估  

Mid-term outcomes of laparoscopic-assisted pullthrough for Currarino syndrome

作　　者：李颀[1] 张震[1] 姜茜[2] 闫淯淳 肖萍[4] 马亚 李龙[1] Li Qi;Zhang Zhen;Jiang Qian;Yan Yuchun;Xiao Ping;Ma Ya;Li Long(Department of General Surgery,Capital Institute of Pediatrics,Beijing 100020,China;Department of Medical Genetics,Capital Institute of Pediatrics,Beijing 100020,China;Department of Radiology,Capital Institute of Pediatrics,Beijing 100020,China;Department of Pathology,Capital Institute of Pediatrics,Beijing 100020,China;Department of Ultrasound,Capital Institute of Pediatrics,Beijing 100020,China)

机构地区：[1]首都儿科研究所附属儿童医院普通外科,100020 [2]首都儿科研究所遗传研究室,100020 [3]首都儿科研究所附属儿童医院影像科,100020 [4]首都儿科研究所附属儿童医院病理科,100020 [5]首都儿科研究所附属儿童医院超声科,100020

出　　处：《中华小儿外科杂志》2020年第10期906-913,共8页Chinese Journal of Pediatric Surgery

基　　金：国家自然科学基金(81700451)。

摘　　要：目的探讨腹腔镜治疗Currarino综合征(Currarino syndrome,CS)的可行性和安全性,并对其术后肛门直肠功能进行中期评估。方法收集2016年1月至2019年1月于首都儿科研究所附属儿童医院普通外科采用腹腔镜辅助治疗的CS患儿14例,作为腹腔镜组。其中,男6例,女8例;手术年龄为(20.7±13.9)个月,范围为3~48个月;完全型13例,轻型1例。所有患儿均存在排便困难,均伴有肛门畸形,包括肛门直肠狭窄8例,女性直肠会阴瘘3例,直肠阴道瘘1例,男性中高位肛门闭锁直肠尿道瘘1例、不伴瘘1例。既往手术史包括肛门成形术4例;脊髓栓系松解+脊膜修补术3例;骶前肿物切除+脊髓栓系松解+脊膜修补术1例;单纯肿物切除术4例;腹腔镜辅助巨结肠根治术1例。共4例术后出现瘘管,其中肿物皮肤瘘1例,直肠皮肤瘘2例,吻合口漏1例。既往行结肠造瘘术4例。伴有明确家族史5例。辅助检查结果:14例患儿全部可见骶骨畸形;直肠末端扩张9例,直肠骶部皮肤瘘2例;骶前肿物12例,神经管畸形8例。选择2013年1月至2015年5月于本中心接受单纯经肛门手术的10例患儿作为对照组。采用t检验、χ2检验比较两组手术结果。结果腹腔镜组1例中转开腹手术,其余患儿均于腹腔镜下完成手术。手术时间为(120±25)min,范围为90~150 min;术中出血量范围为5~50 ml。腹腔镜组术后早期并发症发生例数(0例)少于对照组(3例)。剔除随访不足1年的患儿,腹腔镜组10例患儿术后随访满1年,术后随访时间为(15.9±3.3)个月,范围为12~22个月;自主排便者大便次数为(3.3±1.5)次/d,范围为1~6次/d,4例有偶发污粪(<3次/周),1例有频繁污粪(≥3次/周),2例便秘,无尿失禁及尿潴留,1例患儿术后半年发生直肠黏膜脱垂,无肿物或瘘管复发。两组在术后排便次数和便秘发生例数方面的差异无统计学意义,但频繁污粪的发生例数在腹腔镜组(1例)少于对照组(5例),差异具有统计学Objective To evaluate the mid-term outcomes of laparoscopic-assisted procedure for Currarino syndrome (CS).Methods From 2016 to 2019, 14 CS children underwent laparoscopic assisted procedure. There were 6 boys and 8 girls. Another 10 CS children undergoing a transanal approach procedure from 2013 to 2015 were adopted as control group. All cases had constipation and 4 children underwent colostomy. Anal malformations included anorectal stenosis (n=8), female rectoperineal fistula (n=3), rectovaginal fistula (n=1), male anal atresia without fistula (n=1) and male rectourethral fistula (n=1). The procedures included anoplasty (n=4), median posterior sacral detethering & duraplasty (n=3), median posterior sacral tumor enucleation, detethering & duraplasty (n=1). Among four cases of median posterior sacral tumor enucleation, two children developed rectocutaneous fistula in sacrococcygeal region postoperatively. One child underwent laparoscopic assisted pull-through without enucleation and suffered from amostomosis leak postoperatively. Four cases underwent colostomy and there were 5 familial cases. Radiograph and computed tomography (CT) demonstrated a variety of anomaly sacrum. Constrast imaging indicated dilated rectum (n=9) and rectocutaneous fistula (n=2). Pelvic magnetic resonance imaging revealed presacral masses (n=12). And lumbosacral magnetic resonance imaging depicted nervous system malformations (n=8).Results All children successfully underwent laparoscopic operation except for one case of convertion. The mean operative duration was (90-150) min and volume of blood loss (5-50) ml. All recovered uneventfully without early complication. In control group, three children (30%) suffered from early complication. The incidence of early complication was significantly lower in laparoscopic group than that in control group (P<0.05). Bowel function was assessed for those treated for over 1 years in laparoscopic group (n=10). The mean follow-up period was (15.9±3.3)(12-22) months in laparoscopic group. In laparoscopic

关 键 词：腹腔镜 CURRARINO综合征 

分 类 号：R726.5[医药卫生—儿科]