Pathological study of idiopathic portal hypertension with an emphasis on cause of death based on records of Annuals of Pathological Autopsy Cases in Japan
Article first published online: 2 JUN 2006
Journal of Gastroenterology and Hepatology
Volume 22, Issue 2, pages 204–209, February 2007
How to Cite
Sawada, S., Sato, Y., Aoyama, H., Harada, K. and Nakanuma, Y. (2007), Pathological study of idiopathic portal hypertension with an emphasis on cause of death based on records of Annuals of Pathological Autopsy Cases in Japan. Journal of Gastroenterology and Hepatology, 22: 204–209. doi: 10.1111/j.1440-1746.2006.04492.x
- Issue published online: 29 JAN 2007
- Article first published online: 2 JUN 2006
- Accepted for publication 18 January 2006.
- hepatic failure;
- idiopathic portal hypertension;
- intestinal infarction;
- portal venous thrombosis
Background and Aim: Idiopathic portal hypertension (IPH) is thought to be benign if bleeding gastroesophageal varices can be controlled or prevented. A recent autopsy of a woman with IPH who died of hemorrhagic intestinal infarction related to mesenteric thrombosis prompted the authors to examine the terminal antemortem features and causes of death of IPH.
Methods: Autopsy cases registered as IPH from 1986 to 1997 were surveyed in the records of the Annuals of Pathological Autopsy Cases in Japan, with permission from the Japanese Society of Pathology. The records of 65 of these cases were collected and examined pathologically.
Results: It was found that the most frequent cause of death in these cases was (i) bacterial infection (20 cases). The next three causes of death were directly or indirectly related to hepatic disease or its altered portal hemodynamics as follows: (ii) progressive hepatic failure (16 cases); (iii) massive hemorrhage from ruptured gastroesophageal varices (11 cases); and (iv) hemorrhagic intestinal infarction due to mesenteric venous thrombosis (5 cases). Although portal venous thrombosis was closely associated with (iv), (ii) and (iii) seemed not to be associated with portal venous thrombosis. In addition, intracranial hemorrhage and other heterogeneous factors were identified as the cause of death in five cases and eight cases, respectively.
Conclusion: These results suggest that progressive hepatic failure and intestinal hemorrhagic infarction should be considered in addition to rupture of gastroesophageal varices when monitoring patients with IPH. Clinicians should be also aware of severe bacterial infection and intracranial hemorrhage as a fatal complication of IPH.