Singh Sanjeev K, Gupta Umesh K, Aggarwal Roopak, Rahman Rafey A, Gupta Nand K, Verma Vandana
Pathology, Uttar Pradesh University of Medical Sciences, Saifai, Etawah, IND.
Pediatric Surgery, Uttar Pradesh University of Medical Sciences, Saifai, Etawah, IND.
Cureus. 2021 Feb 16;13(2):e13373. doi: 10.7759/cureus.13373.
Background Hirschsprung's disease (HD) is a developmental disorder of the intrinsic component of the enteric nervous system. It is characterized by the absence of ganglion cells in the myenteric and submucosal plexus. Histopathological diagnosis becomes difficult many times due to submucosal ganglionic cells are not easily identifiable. Aims and objective The aim of this study was to examine the clinical and histopathological features of HD and to establish the utility of calretinin staining to diagnose the case of suspicious HD. Materials and methods After taking necessary informed consent, we studied 41 cases in which clinical suspicion of HD had been made, in a study duration of three years (July 2017-June 2020). Open biopsies were taken from spastic segment, transition zone and dilated segment. Histopathological diagnosis had been made in three categories: HD, no Hirschsprung's disease (NHD) and suspicion of HD. Post histopathological diagnosis calretinin immunohistochemistry (IHC) was applied to all cases and interpretations were noted. Results On the basis of histopathological findings, 25 cases were diagnosed as HD, nine cases were marked for suspicion for HD and seven cases as NHD. After evaluating calretinin IHC on the suspicious case, total of 30 cases were confirmed as HD while the remaining 11 cases were confirmed as NHD. Twenty-four patients of HD were males while the remaining six cases were females. The age of patients ranged from four days to 10 years. Median age six days while 22 patients were in the neonatal period. Conclusion Calretinin immunostaining is a useful modality in diagnosing suspicious cases of HD. Its results are easy to interpret by less experienced pathologist with accuracy.
背景 先天性巨结肠症(HD)是一种肠神经系统内在组成部分的发育障碍性疾病。其特征是肌间神经丛和黏膜下神经丛中缺乏神经节细胞。由于黏膜下神经节细胞不易识别,组织病理学诊断多次变得困难。
目的 本研究的目的是检查HD的临床和组织病理学特征,并确定钙视网膜蛋白染色在诊断可疑HD病例中的效用。
材料和方法 在获得必要的知情同意后,我们在三年(2017年7月至2020年6月)的研究期间研究了41例临床怀疑为HD的病例。从痉挛段、过渡区和扩张段进行开放性活检。组织病理学诊断分为三类:HD、非先天性巨结肠症(NHD)和可疑HD。组织病理学诊断后,对所有病例进行钙视网膜蛋白免疫组织化学(IHC)检测并记录结果。
结果 根据组织病理学结果,25例被诊断为HD,9例被标记为可疑HD,7例为NHD。对可疑病例进行钙视网膜蛋白IHC评估后,共有30例被确诊为HD,其余11例被确诊为NHD。HD患者中24例为男性,其余6例为女性。患者年龄从4天到10岁不等。中位年龄为6天,22例患者处于新生儿期。
结论 钙视网膜蛋白免疫染色是诊断可疑HD病例的一种有用方法。其结果易于经验不足的病理学家准确解读。