Fatal acute portal vein thrombosis associated with hepatic cysts in a patient with autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) often involves polycystic liver disease (PLD). In severe cases, PLD can develop various complications. However, fatal acute portal vein thrombosis (APVT) associated with PLD has not been reported. A 64-year-old male reported mild consciousness dis...

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Bibliographic Details
Published in:CEN case reports 2024-02, Vol.13 (1), p.32-36
Main Authors: Tsuchida, Yohei, Tsubata, Yutaka, Nozawa, Ryosuke, Maruyama, Shuntaro, Ikarashi, Kouzo, Saito, Noriko, Morioka, Tetsuo, Kamura, Takeshi, Shimada, Hisaki, Narita, Ichiei
Format: Article
Language:English
Subjects:
Case Report
Consciousness Disorders - complications
Cysts - complications
Humans
Liver Diseases
Liver Failure - complications
Male
Medicine
Medicine & Public Health
Middle Aged
Nephrology
Polycystic Kidney, Autosomal Dominant - complications
Portal Vein
Thrombosis - complications
Urology
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Summary:Autosomal dominant polycystic kidney disease (ADPKD) often involves polycystic liver disease (PLD). In severe cases, PLD can develop various complications. However, fatal acute portal vein thrombosis (APVT) associated with PLD has not been reported. A 64-year-old male reported mild consciousness disorder. He had been under maintenance hemodialysis for end-stage renal disease due to ADPKD with PLD. Because of recurring hepatic cyst infections, he had sustained high levels of C-reactive protein. Regarding the mild consciousness disorder, a diagnosis of hepatic encephalopathy was made based on an elevation of serum ammonia without any other abnormal liver function tests. Several days after his admission, hepatobiliary enzymes elevated, and acute liver failure progressed. Enhanced abdominal computed tomography suggested the possibility of complete occlusion of the portal vein by a thrombus. Based on an absence of obvious portosystemic collaterals, a diagnosis of APVT was made. The patient died 19 days after admission. Patients with PLD with repeated cystic infections have been seen to develop liver failure, and APVT formation may be one cause of the rapid progression of fatal liver failure. In conclusion, this is the first paper to report on the involvement of APVT in patients with PLD.
ISSN:2192-4449
2192-4449
DOI:10.1007/s13730-023-00795-6