对疑有先天性巨结肠症的６１例患儿行直肠粘膜吸引活检，标本应用神经细胞特异性烯醇化酶（ＮＳＥ）及Ｓ（１００）蛋白免疫组织化学法染色检查，诊断为先天性巨结肠症的３３例，全部经手术后全层病理检查证实，诊断符合率达１００％。研究结果表明，ＮＳＥ结合Ｓ（１００）蛋白的免疫组化染色对直肠粘膜下神经丛中神经节细胞不同侧面的特征性表达，对先天性巨结肠的诊断具有重要价值，尤其在新生儿期。 Totally 61 children with suspected Hirschsprung’s disease underwent colorectal mucosal aspiration biopsy. The specimens were examined by immunohistochemical staining of neuron-specific enolase (NSE) and S (100) protein and diagnosed as Hirschsprung’s disease 33 cases of disease, all confirmed by full-thickness pathological examination after surgery, the diagnostic coincidence rate of 100%. The results show that NSE combined with S (100) protein immunohistochemical staining of the rectal submucosal ganglion plexus on different sides of the characteristic expression of the diagnosis of Hirschsprung’s disease is of great value, especially in the neonatal period.