CAJ | 학술논문

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.
목적 총결선천성항문직장기형술후완고성편비합병거결장적림상특점화치료효과.방법 회고성분석2005년8월지2010년7월수치적완고성편비합병거결장23례림상자료,남10례,녀13례.수차수술년령3 d지6세,술전진단균위중저위항문직장기형.23례환인균이항문성형술후편비취진.하소화도기패조영현시하단결장급직장확장,무이행단,배패연지.본차수술년령1세2개월지15세8개월.균채용Soave거결장근치술.결과 술전견항문외관대치정상13례,외관정상합병직장뇨도루1례,항문개구위치전이6례,반흔회축2례,항문외구협착1례.술중진입분강후즉위확장장관,계막증생증후,장벽혈관증생조대.13례단순경회음수술,10례경복회음수술,기중1례동시회장말단조루.9례거결장절제동시,재차행항문성형술,1례거결장절제동시행전시상입로직장뇨도루수보술.병리검사15례절제장관원근단,가견신경절세포자,8례장관원단무신경절세포자,23례균견기층증생비후,기섬유변성,기층배렬문란.이차술후수진항문공능리씨평분5～6분.결론 선천성항문직장기형술후완고성편비왕왕병발거결장개변,영상현시위장관국한성확장、강경화동력문란.채취수술치료,절제병변장관,주필요적항문해부결함수복,술후효과량호.
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.