Ohuchi N, Ohi R, Takahashi T, Kasai M
J Pediatr Surg. 1986 Jan;21(1):10-4. doi: 10.1016/s0022-3468(86)80641-8.
Intrahepatic portal veins were reconstructed from serial histologic sections of surgical liver specimens from patients with biliary atresia to explain the pathogenesis of portal hypertension, an important late postoperative complication of this disease. It was found that abnormal intrahepatic vasculature was already notable in an evidently reduced number of portal veins in the second month of age, and this was considered responsible for causing presinusoidal block that gave rise to the elevated resistance to intrahepatic portal flow. Reconstruction also revealed that restoration of ordinary portal structure was expectable if, after successful surgery, a patient was sufficiently protected from cholangitis and there was much room for portal veins to grow without being impeded by inflammatory and/or scarring processes. In contrast, the patient whose condition was complicated by cholangitis was shown to acquire a markedly hypoplastic portal system with apparently reduced vascularity.
通过对胆道闭锁患者手术肝脏标本的连续组织学切片重建肝内门静脉,以解释门静脉高压的发病机制,门静脉高压是该疾病术后重要的晚期并发症。研究发现,在2月龄时门静脉数量明显减少,肝内血管异常已很明显,这被认为是导致窦前阻塞的原因,进而引起肝内门静脉血流阻力升高。重建还显示,如果手术成功后患者能充分预防胆管炎,且门静脉有足够的生长空间而不受炎症和/或瘢痕形成过程的阻碍,那么恢复正常门静脉结构是可以预期的。相比之下,并发胆管炎的患者门静脉系统明显发育不全,血管明显减少。