Incidence and clinicopathological analysis of portal vein and inferior vena cava thrombosis in autopsy cases of autosomal dominant polycystic kidney disease.

BACKGROUND

With regard to complications of portal vein (PV) and inferior vena cava (IVC) thrombosis in autosomal dominant polycystic kidney disease (ADPKD), several cases have been reported based on imaging findings. However, only one autopsy case has been described and no systematic analysis has been conducted to date. This retrospective study aimed to review autopsy cases from our department over the past 37 years to clarify the frequency and background factors of thrombosis formation in ADPKD.

METHODS

Among 4001 autopsies performed at our institution from 1987 to 2023, 10 ADPKD cases were identified. We examined the presence of thrombus in these 10 cases and compared pleural effusion and ascites volumes, major organ weights, and clinicopathological factors between cases with thrombus and those without.

RESULTS

Among 10 ADPKD cases, thrombi were identified in four cases in which autopsies were performed relatively recently. These thrombi were distributed in the PV, IVC, and their branches. Compared to non-thrombotic cases, those with thrombi showed a statistically significant increase in kidney weight and tended to have a higher frequency of complications such as sepsis and severe aortic atherosclerosis.

CONCLUSION

This study reports the first systematic autopsy-based investigation of PV and IVC thrombosis in ADPKD. Thrombosis was found at a high frequency of 40% and appears to have increased in recent years. Increased kidney weight was associated with thrombosis formation, and blood stasis due to compression by enlarged kidneys is considered the primary cause. Further case accumulation and elucidation of the pathophysiology involved are anticipated.