CAJ | 학술논문

【目的】探讨婴儿肠神经发育异常病例的诊疗流程及手术方案选择。【方法】对本院2011年1月至2014年1月间收治的79例经手术﹑病理证实为肠神经发育异常的病例资料进行回顾性分析。患儿年龄4 d至3个月，平均16．9 d。一期手术79例，术后56例获得随诊，行二期手术处理。【结果】一期手术79例术后病理检查均有结肠或回肠末端肠壁神经元发育不良证据；二期手术56例中，40例患儿，距一期手术3个月左右予以关瘘处理；4例先天性巨结肠症患儿，行病变结肠切除＋结肠肛门吻合的巨结肠根治术（二期未关瘘）；12例钡灌肠表现异常但无特异性表现患儿，予以定期复查6～12个月不等，延期关瘘。【结论】新生儿期肠神经发育不良较常见。随年龄增长，肠神经发育有逐渐成熟的趋势，并且能够维持正常的肠道功能。钡灌肠检查对于婴儿结肠旷置的肠神经异常的病例在二期处理前的初步评估有重要意义。对于诊断困难病例，适当延期观察以及再次手术时多处肠壁浆肌层活检快速冰冻切片检查很有必要。
【목적】탐토영인장신경발육이상병례적진료류정급수술방안선택。【방법】대본원2011년1월지2014년1월간수치적79례경수술﹑병리증실위장신경발육이상적병례자료진행회고성분석。환인년령4 d지3개월，평균16．9 d。일기수술79례，술후56례획득수진，행이기수술처리。【결과】일기수술79례술후병리검사균유결장혹회장말단장벽신경원발육불량증거；이기수술56례중，40례환인，거일기수술3개월좌우여이관루처리；4례선천성거결장증환인，행병변결장절제＋결장항문문합적거결장근치술（이기미관루）；12례패관장표현이상단무특이성표현환인，여이정기복사6～12개월불등，연기관루。【결론】신생인기장신경발육불량교상견。수년령증장，장신경발육유축점성숙적추세，병차능구유지정상적장도공능。패관장검사대우영인결장광치적장신경이상적병례재이기처리전적초보평고유중요의의。대우진단곤난병례，괄당연기관찰이급재차수술시다처장벽장기층활검쾌속빙동절편검사흔유필요。
[Objective] To explore the treatment procedures and operative schemes for children with intes‐tinal neuronal dysplasia .[Methods] Retrospective analysis was conducted for 79 cases of intestinal neuronal dysplasia from January 2011 to January 2014 .Their average age was 16 .9 (4~120) days .And 56 of them were followed up and re‐operated .[Results]Dysplasia of intestinal wall neurons was present in colon or termi‐nal ileum .Among 56 re‐operated cases ,40 underwent fistula closure at around 3 months after initial opera‐tions .Four cases of congenital megacolon underwent radical macrosigmoid operation (without closing fistula) . And 12 cases with unusual and yet non‐specific manifestations on barium enema received regular reexamination within 6~12 months and closure became delayed .[Conclusion] Neuronal intestinal dysplasia is commonly found in neonates .With advancing age ,enteric nerves grow more mature and assume normal functions .Bari‐um enema is of vital importance for preliminary assessments before re‐operation .For difficult‐to‐diagnose ca‐ses ,delayed observations and multiple‐site biopsy of wall sero‐muscular layer for rapid frozen slices during re‐operations are necessary .