肠神经元性发育不良病理学特征临床意义的探讨

Clinical Significance of the Pathologic Features in Intestinal Neuronal Dysplasia

目的探讨肠神经发育不良(IND)病理学特征的临床意义。方法回顾分析我院1990年～2002年间269例临床诊断为“HD”(小儿258例,成人11例)病人的临床和病理资料。选取HD病变段和近端各12例,节细胞减少症3例,IND8例,以及对照21例的全层结肠标本行SY免疫组化染色。光镜下观察肠神经丛的分布,肠壁肌层SY免疫活性纤维的分布。结果269例中单纯IND15例(6%),HD+IND6例(2%),两者各有1例因近端肠壁IND病变症状复发而再手术。对照中2例见纵肌异位神经丛,1例同时见粘膜下巨大神经丛;先天性肛门闭锁的横结肠和结肠肿瘤各2例,肠肌间见丰富的巨大神经丛。肠壁SY免疫活性在HD和节细胞减少症的病变段,有不同程度的明显减低;对照组和HD近端功能正常的肠壁多表现丰富;IND病变段表现正常和轻度减低的各一半。结论IND的病理特征缺乏特异性,是IND研究结果不一致的重要原因之一。IND与“具有IND特征改变的继发性现象”可能仅是组织学现象上的相似,应有不同的病理基础。IND的诊断应结合病人的年龄、合并的其它病理因素及临床症状的情况,量化IND的病变更有诊断意义。

Objective To evaluate the clinical significance of the pathologic features in intestinal neuronal dysplasia(IND).Methods The clinical and pathological data of269cases(258children,11adults),diagnosed as'HD'in clinic from1990to2002,were studied retrospectively.The full thickness colonic specimens(12ganglionosis and their proximal,8IND,3hypoganglionosis,and21controls)were investigated by immunohistochemically(SY antibodies).SY-immunoreactive nerve fibers in the bowel layers were graded under optical microscope.Results There were15simple IND and6HD plus IND in the269cases,and each group had one receiving re -operation because the proximal pathological changes of IND leads to reoccur constipation.In the controls,2had heterotopic ganglia in longitudinal muscle layers,of which one still had giant submucous plexuses.Meanwhile,abundant giant myenteric plexuses were found in the transverse colonic walls of2congenital anal atresia and the colonic tumor lesion segments.SY-immunoreactivity was markedly decreased in a ganglionoic segments,mildly in hypoganglionosis and half of IND.In contrast,abundant in the most controls and the proximal segments of HD,and the other half of IND.Conclusions The disagreement about IND studies may result from the lower specificity of its pathologic features.Those whose histological changes similar to IND's may have different pathophysiology from IND,though they resemble each other morphologically.Therefore,the age,any other associated pathologic conditions,and the degree of constipation of a patient should be considered to make a diagnosis of IND.Quantitative analysis of the pathological changes of IND can make a more reliable diagnosis.[