Subramanian H, Badhe B A, Toi P C, Sambandan K
Department of Pathology (Histopathology section), Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
Department of Paediatric Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.
Neurogastroenterol Motil. 2017 Mar;29(3). doi: 10.1111/nmo.12939. Epub 2016 Sep 12.
To derive objective values for the diagnosis of Hirschsprung disease (HSCR) from a comparison of the morphometric profile of large intestinal neuronal plexuses in normal perinatal autopsies and surgical specimens of HSCR.
A cross-sectional comparative study with 40 subjects each in (i) non-HSCR perinatal group encompassing neonates and stillborn babies beyond 30 weeks of gestation on whom autopsies were conducted and (ii) HSCR group comprising all patients clinicoradiologically diagnosed as HSCR. The morphometric assessment was done on hematoxylin-and-eosin-stained sections.
The morphometric profile in terms of average number of ganglia/linear mm of colon, interganglion distance, number of ganglion cells/ganglion, average ganglion cell length, ganglion cell nuclear area, ganglion cell nuclear diameter, nerve trunk thickness, and density has been outlined. On comparison with the neuroanatomically normal zone of HSCR, the cut-offs to identify hypertrophic nerve trunks (nerve trunk thickness of >37.85 μm) and reduced number of ganglia (number of ganglia/linear mm of colon <2.05 and interganglion distance of >229 μm) were derived.
CONCLUSIONS & INFERENCES: The determined objective values, after testing on diagnostic rectal biopsies, may serve to formulate a diagnostic algorithm along with immunostaining for diagnosis of HSCR in colorectal specimens.
通过比较正常围产期尸检和先天性巨结肠症（HSCR）手术标本中大肠神经丛的形态计量学特征，得出用于诊断HSCR的客观指标。
进行一项横断面比较研究，每组40例受试者，其中（i）非HSCR围产期组包括妊娠30周以上进行尸检的新生儿和死产儿，（ii）HSCR组包括所有经临床放射学诊断为HSCR的患者。对苏木精-伊红染色切片进行形态计量学评估。
概述了结肠神经节平均数量/结肠线性毫米、神经节间距离、神经节神经细胞数量、神经节细胞平均长度、神经节细胞核面积、神经节细胞核直径、神经干厚度和密度等形态计量学特征。与HSCR神经解剖学正常区域相比，得出了识别肥厚性神经干（神经干厚度>37.85μm）和神经节数量减少（结肠神经节数量/结肠线性毫米<2.05且神经节间距离>229μm）的临界值。
在诊断性直肠活检中进行测试后，所确定的客观指标可用于制定诊断算法，并结合免疫染色用于诊断结直肠标本中的HSCR。
