巨结肠类缘性疾病肛管测压、直肠黏膜活检和钡灌肠检查的意义

目的　根据病理学的诊断依据 ,评价先天性巨结肠类缘性疾病 (HAD)中直肠肛管测压、直肠黏膜活检AchE组化染色和钡灌肠检查的特点 ,了解其在诊断上的意义。方法　 1999～ 2 0 0 1年间 92例临床诊断为先天性巨结肠 (HD)或巨结肠类缘性疾病并施行手术的患儿 ,根据病理诊断排除HD和HAD共存病变病例 ,筛选出HD 4 0例 ,其中男 36例 ,女 4例 ,平均年龄 2 1个月 ;HAD 34例 ,其中男 2 1例 ,女 13例 ,平均年龄 6 3个月。研究二组的临床征候指标以及在经典的测压、组化以及钡灌肠三项检查上的差别。结果　二组病例相比 ,三项检查每项指标间都存在着极显著差异 (P <0 .0 1) :在HAD组患儿 ,85 %都存在有直肠肛管抑制反射 ,其反射波出现特征性的“W”、“U”波形 ;直肠黏膜AchE组化染色 79%为阴性 ;钡灌肠常不能发现明显的狭窄、移行段 ,但有明显的结肠扩张和2 4h钡滞留。结论　HD和HAD是都以便秘为主要表现的两种不同的疾病。经典的三联检查在HAD的诊断和鉴别上也具有重要意义

Anorectal manometry,AchE histochemistry and barium enema for the diagnosis of the allied diseases of Hirschsprung's disease

Objective To evaluate the value of anorectal manometry, AchE histochemical staining of rectal mucosal biopsy and barium enema in the diagnosis of allied diseases of Hirschsprung's disease. Methods During the period of 1999 2001, 92 patients underwent surgery for Hirschsprung's disease (HD) or allied diseases of Hirschsprung's disease (HAD). Based on the postoperative pathological examinations, the subjects with coexistence of HD and HAD were excluded from this study. Forty cases with HD (36 males and 4 females, a mean age of 21 months) and thirty four cases with HAD (21 males and 13 females, a mean age of 63 months) were selected. The comparisons were made in terms of the findings from their preoperative anorectal manometry, AChE histochemical staining of rectal mucosal biopsy and barium enema between HD and HAD groups. Results There were very significant differences between HD and HAD patients in these three examinations ( P < 0.01 ): In HAD group, 85% had recto anal inhibition reflex (RAIR) and a reflex wave with a shape of "W" or "U". 79% of HAD patients were negative in AChE histochemical staining of rectal mucosal biopsy. The barium enema of the most HAD patients showed no obvious narrow and shift segments, but dilatation and barium stagnation over 24 hours had been observed usually in their colon. Conclusions HD and HAD are two kinds of difference diseases. Though they may present a similar constipation, the classic triad of examinations for HD seems to have an important role in their differential diagnosis.