Ghose S I, Squire B R, Stringer M D, Batcup G, Crabbe D C
Departments of Paediatric Surgery and Pathology, Clarendon Wing, Leeds General Infirmary, Leeds, UK.
J Pediatr Surg. 2000 Dec;35(12):1805-9. doi: 10.1053/jpsu.2000.19263.
BACKGROUND/PURPOSE: It is generally accepted that if surgery for Hirschsprung's disease is to be successful, ganglionic bowel must be anastomosed to the lower rectum or anal canal. Above the aganglionic distal bowel lies a transition zone (TZ) where more subtle abnormalities of innervation are apparent. The significance of this transition zone in respect to the functional outcome of surgery has received little attention. The aim of this study was to identify the incidence of transition zone pull-through (TZPT) in a cohort of children who underwent surgery for Hirschsprung's disease, to identify the reasons why TZPTs occurred, and to identify the functional consequences. The authors report the long-term outcome of these children with emphasis on bowel function and the results of subsequent surgery.
A Retrospective study was conducted of children treated at a single institution from 1979 through 1994. TZPT patients were subject to detailed review of surgical records and histopathologic material.
Thirteen children were identified with a TZPT. In 12 cases, histopathologic errors contributed to the TZPT: in 5 cases this was caused by single point biopsies missing an asymmetrical TZ, whereas in 7 cases the histopathologic features of the TZ were not recognized. In 1 case the TZPT was caused by surgical error. As a consequence of the TZPT 7 children underwent repeat pull-through. One child is fully continent, one has daytime fecal continence, and 2 others are incontinent. Two children have permanent stomas. One child is clean with antegrade colonic washouts. Repeat pull-throughs were not attempted in 6 children. Two children have achieved full continence, 2 have permanent stomas, 1 is clean with antegrade colonic washouts, and 1 child receives regular suppositories.
Transition zone pull-throughs occurred because of a combination of surgical and histopathologic errors. The transition zone may follow an asymmetric course around the circumference of the bowel and may be missed if single-point extramucosal biopsy specimens are taken. Recognition of the subtle histologic features of the transition zone requires an experienced pathologist. The functional consequences of a TZPT are severe, with symptoms of constipation, diarrhea, and incontinence. The results of revisional pull-through were disappointing. Serious consideration should be given to alternative procedures such as the antegrade continence enema operation.
背景/目的：人们普遍认为，若要使先天性巨结肠症手术成功，必须将有神经节的肠段与直肠下段或肛管进行吻合。在无神经节的远端肠段上方存在一个过渡区（TZ），此处神经支配存在更为细微的异常。该过渡区对于手术功能结果的意义鲜受关注。本研究的目的是确定一组接受先天性巨结肠症手术的儿童中过渡区拖出术（TZPT）的发生率，找出发生TZPT的原因，并确定其功能后果。作者报告了这些儿童的长期结果，重点是肠道功能及后续手术结果。
对1979年至1994年在单一机构接受治疗的儿童进行回顾性研究。对发生TZPT的患者的手术记录和组织病理学材料进行详细审查。
确定了13例发生TZPT的儿童。12例中，组织病理学错误导致了TZPT：5例是由于单点活检遗漏了不对称的TZ，而7例是未识别出TZ的组织病理学特征。1例TZPT是由手术失误导致的。由于TZPT，7名儿童接受了再次拖出术。1名儿童完全能自主控制排便，1名儿童白天能控制排便，另外2名儿童大小便失禁。2名儿童有永久性造口。1名儿童通过顺行结肠灌洗保持清洁。6名儿童未尝试再次拖出术。2名儿童已完全能自主控制排便，2名儿童有永久性造口，1名儿童通过顺行结肠灌洗保持清洁，1名儿童定期使用栓剂。
过渡区拖出术的发生是手术和组织病理学错误共同作用的结果。过渡区可能在肠周呈不对称分布，如果采用单点黏膜外活检标本可能会遗漏。识别过渡区细微的组织学特征需要经验丰富的病理学家。TZPT的功能后果严重，会出现便秘、腹泻和大小便失禁等症状。再次拖出术的结果令人失望。应认真考虑替代手术，如顺行可控灌肠术。
