Takahashi Seishiro, Yoshida Eriko, Sakanishi Yasushi, Tohyama Norihiro, Ayhan Ayse, Ogawa Hiroshi
Department of Pathology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
Department of Stroke Medicine, Seirei Mikatahara General Hospital Hamamatsu, Japan.
Int J Clin Exp Pathol. 2013 Dec 15;7(1):425-31. eCollection 2014.
Multiple intrahepatic portosystemic shunt (IPSS) without portal hypertension, now thought to be congenital in origin, is very rare. The presence of IPSS, unlike other congenital diseases, may not be recognized for several decades due to the time it takes to develop hepatic encephalopathy. In this article, we report an autopsy case of an 80-year-old Japanese woman with a one-month history of hyperammonemic encephalopathy. Radiological examination of the liver revealed some abnormal connections between the branches of the portal veins and the hepatic veins, but the cause of the aberrant blood flow was not found. The cause of death was extensive cerebral infarction due to thromboembolism. At postmortem examination, multiple anomalous blood vessels were identified histologically in both lobes of the non-cirrhotic liver. In comparison with the few similar cases existing in the literature, this case should be diagnosed as congenital IPSS. To our knowledge, this is the first detailed histological study of IPSS, as several autopsy case reports exist but their histological descriptions are poor. Unlike past reports, the shunt vessels were accompanied by clear elastic lamellae that were microscopically observed. In addition to shunt vessels, septal fibrosis, disorder of hepatic acinar structure, and sinusoidal dilatation and capillarization were observed in the liver. We suggest that these histological modifications observed in the circumference of the shunt vessels acted as secondary regenerative/hyperplastic changes based on blood-flow imbalance caused by the IPSS.
无门静脉高压的多发性肝内门体分流（IPSS）现被认为起源于先天性，极为罕见。与其他先天性疾病不同，由于发生肝性脑病所需的时间，IPSS的存在可能数十年都未被发现。在本文中，我们报告了一例80岁日本女性尸检病例，其有1个月的高氨血症性脑病病史。肝脏的放射学检查显示门静脉分支与肝静脉之间存在一些异常连接，但未发现血流异常的原因。死因是血栓栓塞导致的广泛脑梗死。尸检时，在非肝硬化肝脏的两叶均组织学鉴定出多条异常血管。与文献中现有的少数类似病例相比，该病例应诊断为先天性IPSS。据我们所知，这是对IPSS的首次详细组织学研究，因为虽然有几例尸检病例报告，但它们的组织学描述很差。与过去的报告不同，分流血管伴有在显微镜下可观察到的清晰弹性板。除分流血管外，肝脏还观察到间隔纤维化、肝腺泡结构紊乱以及窦状隙扩张和毛细血管化。我们认为，在分流血管周围观察到的这些组织学改变是基于IPSS引起的血流失衡而产生的继发性再生/增生性改变。
