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Aged Budd-Chiari syndrome attributed to chronic deep venous thrombosis with alcoholic liver cirrhosis
Y Hoshida1, S Yamakado, K Shinoki
1Division of Gastroenterology, Tokyo Metropolitan Tama Geriatric Hospital, Higashimurayama, Japan.
Insights
Budd-Chiari syndrome, a rare condition, can be acquired, often due to thrombosis in elderly patients. This case highlights a 74-year-old man with Budd-Chiari syndrome linked to chronic deep venous thrombosis and liver cirrhosis.
Area of Science:
- Vascular Medicine
- Hepatology
- Thrombosis Research
Background:
- Budd-Chiari syndrome is a rare hepatic vascular disease characterized by hepatic venous outflow obstruction.
- Etiologies are diverse, but acquired lesions from thrombosis are increasingly recognized, particularly in elderly populations.
- Alcoholic liver cirrhosis is a known risk factor for various thrombotic complications.
Observation:
- A 74-year-old male presented with Budd-Chiari syndrome.
- The patient had a history of stasis ulcers at age 45, cerebral infarction and left hemiparesis at age 71.
- Imaging revealed inferior vena cava obstruction with extensive thrombi and collateral vessel formation.
Findings:
- Budd-Chiari syndrome was attributed to chronic deep venous thrombosis and alcoholic liver cirrhosis.
- Potential contributing factors included chronic disseminated intravascular coagulation and hereditary coagulopathy, suggested by family history and low clotting inhibitors.
- The patient survived approximately 30 years from the suspected onset, contrasting with the typically high mortality of untreated cases.
Implications:
- This case underscores the possibility of long-term survival in Budd-Chiari syndrome, even with extensive thrombosis.
- Investigating underlying coagulopathies is crucial for understanding disease progression and management.
- The interplay between liver cirrhosis and acquired thrombosis warrants further investigation in clinical practice.
Abstract:
Budd-Chiari syndrome is a rare disease, but there are many known causes. Recent studies showed that it can be an acquired lesion resulting from thrombosis in some elderly patients. We report a 74-year-old man with Budd-Chiari syndrome attributed to chronic deep venous thrombosis and alcoholic liver cirrhosis. When he was aged 45 years, stasis ulcers of the lower extremities appeared. Cerebral infarction and left hemiparesis occurred at age 71. Ultrasonography, venacavography, and three-dimensional-magnetic resonance imaging on admission demonstrated total obstruction of the inferior vena cava with several massive thrombi and developed collateral vessels. Although the etiology of the thrombosis remained obscure, we made some speculative assumptions that chronic disseminated intravascular coagulation (which is frequently observed in cirrhosis) or hereditary coagulopathy could be involved, from his familial history of thrombotic phenomena and a severe deficiency of clotting inhibitors. Despite the high mortality of untreated Budd-Chiari syndrome reported in previous studies, this patient had been alive for about 30 years from the suspected onset.