儿童结直肠血管畸形的诊治  

Diagnosis and treatment of colorectal vascular malformation in children

作　　者：彭春辉[1] 陈亚军[1] 张廷冲[1] 王大勇[1] 庞文博[1] 王增萌[1] 吴东阳[1] 

机构地区：[1]首都医科大学附属北京儿童医院普通外科,100045

出　　处：《中华小儿外科杂志》2015年第3期206-210,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的 总结儿童结直肠血管畸形的临床特点,探讨最佳手术方式.方法 回顾性分析2010年1月至2014年2月首都医科大学附属北京儿童医院普外科收治的14例结直肠血管畸形患儿临床资料,包括临床表现、影像学检查、手术和随访.本组男女之比为3.7∶1,平均发病年龄为1.5岁,平均误诊时间为4.1年.11例为独立的病变,3例为Klippel-Trenaunay综合征.主要表现为逐渐加重的便血、贫血.所有患儿术前均行结直肠超声、结肠镜检查,行CT血管成像（computed tomography angiography,CTA） 13例,行MR血管成像（magnetic resonance angiography,MRA）1例.结肠镜下可见病变肠管与正常肠管界限清晰,13例患儿可见受累肠管肠黏膜充血、水肿,血管网模糊；1例患儿可见散在大小不等的紫疱.12例患儿经腹肛门行Soave手术,1例仅行畸形重复血管结扎,1例腹腔镜探查、经肛门Soave手术.结果 本组术后住院时间4～25 d,平均15d.开始扩肛时间14～25 d,平均为20 d.除1例失访外,余者术后获门诊及电话随访,平均16个月.复查血常规,血红蛋白基本恢复正常.术后4例患儿有血便,其中2例患儿偶尔大便带血丝；1例术后4个月出现臀部感染,行抗感染和切开引流后治愈.结论 结直肠超声、CTA以及结肠镜的联合应用,可明确了解本病,降低误诊率；经腹肛门Soave手术,能安全完整地切除病变肠管,有效的治愈或改善便血.Objective To explore the clinical features and optimal treatments of colorectal vascular malformation in children.Methods From Jan.2010 to Feb.2014,a total of 14 patients with colorectal vascular malformation were recruited.Clinical manifestations,imaging examinations,surgical treatments and outcomes were analyzed.The ratio of boy and girl was 3.7 ∶ 1.The mean age of onset was 1.5 years.The average time of delay in diagnodis was 4.1 years.There were independent diseases （n =11） and Klippel-Trenaunay syndrome （n =3）.Aggravated rectal bleeding and anemia were typical manifestations.The preoperative daignosis was made by colorectal ultrasound and colonoscopy.And computed tomography angiography （CTA,n =13） and MR angiography （MRA,n =1） were performed.Colonoscopy revealed that there was a distinct boundary between lesion and normal tissue.Mucosal hyperemia,edema and fuzzy vascular network were present in 13 cases.And 1 case had purple swelling of various sizes.The procedures included transabdominal Soave （n =1 2）,ligation of abnormal repeated vessels （n =1） and laparoscopic exploration and transanal Soave （n =1）.Results The mean postoperative hospitalization stay was 15 （4-25） days.Anal dilation started from a mean time of 20 （14-25） days.Follow-ups were conducted by outpatient visits and telephone interviews during a mean period of 16 months.Four patients suffered hemafecia and two of them had bloodshot in stool occasionally.One case of hip infection was cured after antibiotics and local incision with drainage therapy.One patient became lost to follow-up.All blood routine tests showed normal hemoglobin level.Conclusions The combined use of colorectal ultrasound,CTA and colonoscopy can define the severity of vascular malformation and prevent its misdiagnosis.And transabdominal Soave procefure may completely and safely remove the lesion and cure or improve hemafecia effectively.

关 键 词：血管畸形 结肠 直肠 诊断 消化系统外科手术方法 

分 类 号：R726.5[医药卫生—儿科]