The clinical pathological analysis of intestinal neuronal dysplasia type B / 中国小儿急救医学

ABSTRACT

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.