Abstract | November 18, 2023
Case of Budd Chiari Syndrome Secondary to Tumor Thrombosis
Learning Objectives
- Budd Chiari is due to obstruction of hepatic veins and adjacent structures, such as the IVC, which is a rare occurrence with estimated prevalence of 1 to 4 per million in western countries. The major cause are hypercoagulable states, and these predisposed to formation of thrombosis in these vascular structures, leading to obstruction of the outflow. Clinical features primarily include those of outflow obstruction such as abdominal pain, hepatomegaly and ascites, which is a classical presentation. Other features such as nausea, vomiting, weight loss, and jaundice with biochemical liver dysfunction may occur. Diagnostic imaging with ultrasound is the first line modality, followed by CT or MRI, which has a higher specificity. Additionally hepatic venography can visualize filling defects and screening for hypercoagulable states would be recommended. Usual treatment is treating the cause, for example tumor causing obstruction with surgery/chemo/radiotherapy, and first line approach is anticoagulation with low molecular weight heparin. Surgical procedures such as IVC shunting, dilation, TIPS is recommended when there is a reversible cause, or anticoagulation fails or is contraindicated.
BCS is a rare constellation of conditions due to obstruction of venous flow from levels ranging from the hepatic veins to the confluence of the IVC and right atrium. The resulting retrograde flow of blood leads to hepatomegaly, ascites and a myriad of clinical features. Our case highlights the clinical features, diagnostic challenges and management of a patient.
This is a 67 year old male with prostate adenocarcinoma and aortic valve replacement on warfarin anticoagulation who presented to us for complaints of abdominal pain. Patient has had low grade back and abdominal pain for years, however it was worsening over the last hours and associated with bloating which warranted him to come to the ED. Physical exam with massively distended and tense abdomen. Initial evaluation of the patient was significant for hypotension, urine suggestive of UTI, CT with IVC thrombus, hepatomegaly and possible cirrhosis. CT also showed bony lumbar spine ostepenia and elevated ALP suggested metastasis to the spine. He follows a urologist and oncologist for the prostate cancer, and was scheduled to start radiation therapy next week.
Vascular was consulted who recommended to continue warfarin, he was given albumin for low BP and started on appropriate treatment and general surgery was consulted for paracentesis. He was discharged after stabilization on appropriate medical therapy.
He was subsequently seen by oncology who planned to start patient on leuprolide and during this visit his abdominal pain and distention had worsened to the point that he was unable to lie flat. A PET scan showed increased uptake in prostate, lumbar and thoracic spine and uptake in the IVC suggestive of a tumor thrombus.
He was started on palliative radiation for the prostate cancer, and patient had a stent placed in the IVC and continued back on warfarin. Additional considerations for TIPS, anticoagulation and symptomatic management are usually fundamental in BCS.
References and Resources
- Advancing Liver Therapeutic Approaches (ALTA) practice-based recommendations on transjugular intrahepatic portosystemic shunts in portal hypertension can be found in Clin Gastroenterol Hepatol 2022 Aug;20(8):1636full-text, commentary can be found in Clin Gastroenterol Hepatol 2023 Jun;21(6):1673
- Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease can be found in Hepatology 2021 Jan;73(1):366, commentary can be found in Hepatobiliary Surg Nutr 2023 Feb 28;12(1):105full-text
- Disorders of hepatic and mesenteric circulation can be found in Am J Gastroenterol 2020 Jan;115(1):18, commentary can be found in Am J Gastroenterol 2020 Jun;115(6):952
