Gonzalez-Vasquez R, Heiss W H
Kinderchirurgische Abteilung, Mutterhaus der Borromäerinnen, Trier.
Z Kinderchir. 1988 Dec;43(6):424-6. doi: 10.1055/s-2008-1043501.
Reports on zonal aganglionosis of the bowel are very rare and the few cases reported concern colon and rectum. We present a case of segmental aganglionosis of the jejunum in a 2-months-old boy. He had to be operated upon twice because of an ileus of the short bowel. At the second operation, a 10 cm long narrow segment of the jejunum was resected. The histological examination revealed a segmental aganglionosis of the plexus myentericus. Possible causes of this rare zonal aganglionosis could be: 1. Local damage of the plexus myentericus through anoxia. 2. Primary abnormity in the gastrointestinal anlage. 3. An intrauterine inflammation. 4. A double migration and development gradient of the precursors of the neuroblasts, from each end to the middle of the gut. A defect in the microenvironment of the enteric neurons would result in their failure in differentiation. This possible explanation would challenge the accepted theory of pathogenesis of colonic aganglionosis.
关于肠道节段性无神经节症的报道极为罕见，少数已报道的病例涉及结肠和直肠。我们报告一例2个月大男婴空肠节段性无神经节症的病例。由于短肠肠梗阻，他不得不接受两次手术。在第二次手术中，切除了一段10厘米长的狭窄空肠。组织学检查显示肌间神经丛节段性无神经节症。这种罕见的节段性无神经节症的可能原因可能是：1. 肌间神经丛因缺氧而局部受损。2. 胃肠道原基的原发性异常。3. 宫内炎症。4. 神经母细胞前体从肠道两端向中间的双重迁移和发育梯度。肠神经元微环境中的缺陷会导致它们分化失败。这种可能的解释将挑战目前公认的结肠无神经节症发病机制理论。
