Taguchi T, Tanaka K, Ikeda K
Gastroenterology. 1985 May;88(5 Pt 1):1099-103. doi: 10.1016/s0016-5085(85)80066-4.
Abnormal arteries were demonstrated in the intestines of 8 of 25 children with Hirschsprung's disease. The histologic findings were similar to those seen in fibromuscular dysplasia with proliferation of smooth muscle cells in the thickened adventitia. We propose a new pathological entity, "adventitial fibromuscular dysplasia." The abnormal arteries were located only in the transitional zone, which includes the proximal aganglionic, the oligoganglionic, and the distal normoganglionic segments. These findings are compatible with the suggestion that the craniocaudal migration of ganglion cells might be interrupted by intestinal ischemia in the presence of abnormal arteries in the embryo.
在25例患有先天性巨结肠症的儿童中，有8例在肠道中发现了异常动脉。组织学检查结果与纤维肌性发育异常相似，增厚的外膜中有平滑肌细胞增生。我们提出了一种新的病理实体，即“外膜纤维肌性发育异常”。异常动脉仅位于过渡区，该区域包括近端无神经节、少神经节和远端正常神经节段。这些发现与以下观点相符：在胚胎期存在异常动脉的情况下，神经节细胞的头尾迁移可能会因肠道缺血而中断。
