ارزش تشخیصی رنگ آمیزی انولاز اختصاصی نرون در بیوپسی های رکتوم بیماران مشکوک به بیماری هیرشپرونگ

Background and purpose: Hirschsprung’s disease (HD) is the most common etiology of neonatal intestinal obstruction. Definite diagnosis is based on demonstration of aganglionosis in seromuscular and full tickness rectal biopsy. Because of problems and complications of full tickness and seromuscular rectal biopsies, mucosal-submucosal rectal biopsy is prefered,but interpretation of these biopsies with Hematoxylin-Ëosin (H&Ë) staining and even with usual Ïmmunohistochemical staining (acetylcholinesterase) is difficult. Because neuron-specific enolase (NSË) for diagnosis of Hirschsprung’s disease is an available marker with performance on paraffin blocks and lack of data about its accuracy, this study was done to determine the accuracy of NSË staining on mucosal- submucosal rectal biopsies in diagnosis of HD and allied disorders. Materials and methods : We studied 65 mucosal- submucosal and 65 seromuscular biopsies of patients suspected of HD or allied disorders in Shafa and Booali hospitals Sari,Ïran (from 23 Sep. 2003 until 22 Ôct. 2004). For each patient both seromuscular and Mucosal-submucosal biopsiess were prepared. Mucosal-submucosal slides were stained by NSË and seromucular slides stained by H&Ë then the slides examined double blindly and the results compared with each other. Results : Sensitivity, specificity, efficeincy, positive and negative predictive value in diagnosis of HD are 100% , %84.2,%89.1, %81.8, 100% respectively (p<0.05) , showing statistically significant difference with standard. Ôn evaluation of Hypogangliononsis, we had one false – negative and nine false – positive. Çonclusion: Ïn NSË staining, finding of ganglion cell definitively role out HD but lack of ganglion cell in %81.8 confirms H.D. Thus NSË Staining on mucosal-submucosal specimens is possibly adequate for establishing the presence or absence of ganglion cells but ganglion cells should be quantified in the myenteric plexus.