title: Congenital multiple intrahepatic portosystemic shunt: an autopsy case.
abstract: 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.
authors.affiliation: Department of Pathology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Seishiro
authors.initials: S
authors.lastname: Takahashi
authors.affiliation: Department of Stroke Medicine, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Eriko
authors.initials: E
authors.lastname: Yoshida
authors.affiliation: Department of Hepatology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Sakanishi
authors.affiliation: Department of Radiology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Norihiro
authors.initials: N
authors.lastname: Tohyama
authors.affiliation: Department of Pathology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Ayse
authors.initials: A
authors.lastname: Ayhan
authors.affiliation: Department of Pathology, Seirei Mikatahara General Hospital Hamamatsu, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ogawa
mesh_terms: D000369:Aged, 80 and over; D001344:Autopsy; D005260:Female; D006501:Hepatic Encephalopathy / Q000473:pathology; D006801:Humans; D011169:Portal Vein / Q000002:abnormalities* / Q000473:pathology; D054079:Vascular Malformations / Q000473:pathology*
keywords: Congenital intrahepatic portosystemic shunt; autopsy; hepatic encephalopathy