Rassouli-Kirchmeier Roxana, Lok Maarten Janssen, Kusters Benno, Nagtegaal Iris, Köster Nils, van der Steeg Herjan, Wijnen Marc, de Blaauw Ivo
Department of Surgery-Pediatric Surgery, Radboudumc, Nijmegen, The Netherlands,
Pediatr Surg Int. 2014 Aug;30(8):809-13. doi: 10.1007/s00383-014-3541-7. Epub 2014 Jul 5.
The diagnosis of Hirschsprung's disease (HD) remains challenging. The identification of ganglion cells is difficult and acetycholine esterase (AChE) staining can be subject to a great variability, particularly in the neonatal period (<8 weeks). Nerve trunks greater than 40 µm are considered to be predictive for HD. The aim of this study was to evaluate the usefulness of measuring nerve trunk size in the newborn with HD.
Out of 292 biopsies 69 could be reanalyzed by three independent researchers. 40 µm was used as cutoff point for nerve trunk size. They were subdivided into three groups: (a) diagnosis of HD certain at the first biopsy, (b) no HD and (c) diagnosis of HD remains doubtful and re-biopsy taken.
In 87 % of group A nerve trunk size was ≥ 40 µm (SD 13.8). In 84 % of group B trunk size was < 40 µm (SD 16.2). In group C only 60 % of the patients showed a positive correlation between final diagnosis and nerve trunk size.
Using 40 µm as the cutoff point gave 13 % false-negative and 16 % false-positive cases. Measurement of the nerve trunk in the neonatal period does not seem to be a reliable method for detecting HD.
先天性巨结肠(HD)的诊断仍然具有挑战性。神经节细胞的识别困难,乙酰胆碱酯酶(AChE)染色可能存在很大差异,尤其是在新生儿期(<8周)。直径大于40 µm的神经干被认为是HD的预测指标。本研究的目的是评估测量HD新生儿神经干大小的实用性。
在292例活检中,69例可由三位独立研究人员重新分析。以40 µm作为神经干大小的截断点。将它们分为三组:(a)首次活检时确诊为HD,(b)无HD,(c)HD诊断仍存疑问并进行了再次活检。
A组中87%的神经干大小≥40 µm(标准差13.8)。B组中84%的神经干大小<40 µm(标准差16.2)。C组中只有60%的患者最终诊断与神经干大小呈正相关。
以40 µm作为截断点会出现13%的假阴性和16%的假阳性病例。在新生儿期测量神经干似乎不是检测HD的可靠方法。