B型肠神经元发育不良的临床病理分析

目的：探讨B型肠神经元发育不良的临床病理特征及诊断。方法对9例B型肠神经元发育不良患儿的临床病理特征进行分析，患儿年龄3个月～1岁，平均7.8个月，男女比为5∶4，均表现为便秘及腹胀进行性加重；标本进行全层肠段取材，常规制片，行HE和免疫组织化学染色，观察肠神经元及神经节细胞形态和数量的变化。结果本组病例狭窄肠段肠壁肌间神经节细胞数明显增多，神经节细胞密度增高，可见巨神经丛及孤立或异位神经节细胞，近端肠段肠壁肌间神经节细胞数量和密度正常，未见巨神经丛及孤立或异位神经节细胞，形态符合B型肠神经元发育不良的诊断标准，结合临床表现，可诊断为B型肠神经元发育不良。结论对B型肠神经元发育不良的诊断标准应严格掌握，临床上必须有典型巨结肠类缘病的表现，病理形态上具有巨神经节、孤立或异位神经节细胞、肠壁神经节细胞数目增多，并排除其他原发性因素后，才能诊断为B型肠神经元发育不良。

The clinical pathological analysis of intestinal neuronal dysplasia type B

Objective To investigate the clinicopathological features and diagnosis of intestinal neu-ronal dysplasia type B.Methods Between January 2004 and August 2014 , 9 patients ( 5 males and 4 females) were treated for constipation and abdominal distention,and in all of them an intestinal neuronal dys-plasia type B was confirmed histopta holoig cally.The age of 9 patients ranged from 3 months to 1 year old ( mean 7.8 months) .The specimen of 9 patients was routinely takne by paraffin-embedded full-circumference sections of lesional bowel,hematoxylin and eosin and immunohits ochemical stainign were carried out on the specimen.The patholgo ical morphology and quantitative of inet stinal en urons and ganglia were retrospectively analyzed.Results Total of the 9 patients,the number and density of myenteric ganglia increased significant-ly increased in the lesional bowel,the pathological findings included giant nerve plexus,isolated and ectopic ganglia.In the proximal bowel,the number and density of myenteric ganglia were observed abnormal on giant nerev plexus, isoal ted and ectopci ganglia was osb erved.Combinated these pathological findings and symp-toms,intestinal neuronal dysplasia type B was diagnosed.Conclusion The diagnosis of ni testinal neuronal dysplasia type B relies on typical presentations of allied disorders of Hirschsprung′s disease,giant ganglia, isolated and ectopic ganglia,and increasing the density of giant submucosal ganglia of pathologic morpholo-gy,meanwhile,excludingo thers prima ry etiologies.