肠神经发育不良症的诊断与手术治疗

The diagnosis and surgical treatment of intestinal neuronal hypogenesis

目的探讨肠神经发育不良症的诊断与治疗效果。方法对我科收治的19例肠神经发育不良症患儿进行回顾性分析及随访。结果本组19例肠神经发育不良症术前全部行钡灌肠X线检查,共有9例可见狭窄段、扩张段,其中2例可见明显移行段。19例行直肠肛管测压有7例出现直肠肛管抑制反射,19例行乙酰胆碱酯酶测定8例阳性;均术后病理明确诊断,术后4例发生小肠结肠炎,经保守治疗痊愈。2例术后仍腹胀、便秘,二次手术将回肠末端拖下与直肠吻合而治愈,其他患儿痊愈出院。结论肠神经发育不良症的术前诊断困难,术中快速病理有助明确诊断,手术应尽量切除病变肠管,术后疗效满意。

Objective To investigate the diagnostic methods and the therapeutic results of intestinal neuronal hy- pogenesis in children. Methods Nineteen cases with intestinal neuronal hypogenesis were retrospectively analyzed. The data included clinical presentations, barium clysis, anorectal manometry , histochemical staining for acetyl- cholinesterase（AchE） before operation, the pathological examination after operation, and postoperative complications were recorded and analyzed. Results Out of all 19 cases,9 patients showed the typical narrow and distention segment of colon by barium clysis. And 7 patients showed rectoanal inhibitory reflex（RAIR） by underwent anorectal manome- try. In 19 specimens for histochemical staining for AchE, 8 cases showed positive results. Neuronal hypogenesis could be finally confirmed by postoperative biopsies. There were 4 cases suffered from enterocolitis after operation and recov- ered by expectant treatment, and 2 cases were cured by second operation of ileum - rectum anastomosis for still tympa- nites and constipation. Other patients were recovered without complications. Conclusions It is helpful to diagnose the intestinal neuronal hypogenesis by pathological diagnosis in operation. Resection of the pathological segment of bowels and colon - rectum anastomosis to treat intestinal neuronal hypogenesis can get the satisfactory curative effect.