Left Trisectionectomy and Supra-Hepatic Caval Reconstruction with Vascular Prosthesis for Chronic Budd-Chiari Syndrome Caused by Hepatic Alveolar Echinococcosis
Hepatic alveolar echinococcosis (AE) is a severe and often fatal infectious disease caused by the larval stage of Echinococcus multilocularis. This disease is characterized by its low prevalence in endemic areas but high morbidity and mortality rates. AE lesions can invade critical structures such as the hepato-caval confluence, including the main hepatic veins and the retro-hepatic inferior vena cava (IVC). This invasion can lead to Budd-Chiari Syndrome (BCS), a severe complication presenting with symptoms such as abdominal pain, ascites, and hepatomegaly. This article details a case of chronic BCS caused by hepatic AE and outlines the therapeutic approach involving left trisectionectomy and supra-hepatic IVC replacement with a vascular prosthesis.
The patient, a 57-year-old man from an E. multilocularis endemic area, was admitted with complaints of right upper quadrant pain and ascites. Pre-operative imaging studies, including abdominal computed tomography (CT) and magnetic resonance imaging (MRI), revealed a 10 x 10 x 8 cm AE lesion in the left and right anterior liver lobes. The lesion had invaded the supra-hepatic IVC, as confirmed by venous angiography, which also showed a cramped section of the supra-hepatic IVC extending into the right atrium and the establishment of collateral circulation. According to the World Health Organization Informal Working Group on Echinococcosis PNM classification system, the patient was staged as P4N0M0, with liver function classified as Child grade B.
A multidisciplinary team devised a step-by-step resection strategy. The first step involved a left trisectionectomy to remove the majority of the AE lesion, leaving only the portion invading the IVC. The IVC was then blocked on both sides, and due to adequate collateral circulation, hemodynamics remained stable. The invaded section of the IVC was resected and reconstructed using a vascular prosthesis. Finally, the diaphragm was repaired with a patch, which was not completely closed to prevent pericardial effusion. The entire surgical procedure lasted 8 hours, with vascular reconstruction taking 40 minutes and an estimated blood loss of 500 mL, without the need for blood transfusion.
Post-operative pathological examination confirmed the diagnosis of AE through hematoxylin and eosin staining. The patient recovered well, with no signs of vascular thrombosis or other major complications during a 4-year follow-up period. Post-operative imaging confirmed the patency of the IVC and the absence of AE recurrence.
BCS caused by hepatic AE is a rare clinical condition, and treatment options have included hepato-caval reconstruction, transjugular intra-hepatic portosystemic shunt placement, and liver transplantation. In this case, the strategy of hepatectomy combined with artificial blood vessel replacement was employed. The reconstruction of the supra-hepatic IVC presents significant challenges, particularly when the vessel is fibrotic and stenotic. In this instance, the defect area was too extensive for repair with an autologous vessel graft, and the use of allogeneic blood vessels would have necessitated lifelong immunosuppressants, increasing the risk of AE recurrence. Therefore, an artificial vascular graft was deemed the most suitable option, despite potential concerns such as thrombosis or graft obstruction. Fortunately, post-operative monitoring showed no evidence of these complications.
In conclusion, BCS caused by hepatic AE is an extremely rare clinical phenomenon. Radical resection with an R0 margin offers the best chance for long-term disease-free survival in AE patients. This case highlights the effectiveness of a combined approach involving hepatectomy and artificial vascular graft replacement for managing this complex condition. The experience gained from this case may provide valuable insights and treatment modalities for managing secondary BCS caused by hepatic AE.
doi.org/10.1097/CM9.0000000000000521
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