Treatment of Cavernous Transformation of Portal Vein Caused by Hepatic Cystic Echinococcosis Using Ex Vivo Liver Resection and Autotransplantation.

BACKGROUND Hepatic cystic echinococcosis (HCE) is a frequently overlooked parasitic liver disease, for which the commonly recommended treatment is radical resection. However, this approach is often associated with severe comorbidities such as HBV/HCV, cirrhosis, and hepatic carcinoma, among others. CASE REPORT In this report, we present a case successfully managed by ex vivo liver resection and autologous liver transplantation (ELRA). In the described case, ex vivo resection was not feasible due to recurrent lesions and infections invading the portal vein, which resulted in portal vein cavernous transformation. CONCLUSIONS Through this paper, we aim to detail the treatment process, showcasing the feasibility and advantages of ELRA. Additionally, we propose a novel approach for the treatment of this disease, while emphasizing the importance of radical resection surgery to prevent long-term complications.

TGF-ß1 signaling activates hepatic stellate cells through Notch pathway.

Hepatic stellate cells (HSCs), as the most important stromal cells in the liver microenvironment, play crucial roles in hepatic fibrosis, hepatocellular carcinoma, liver regeneration and fetal liver development after transdifferentiating into myofibroblasts (MFs). Transforming growth factor ß1 (TGF-ß1), as an important polyergic cytokine, is involved in HSCs activation process. However, the specific mechanisms of HSCs transdifferentiation process are not clearly demonstrated. Here we added exogenous recombinant TGF-ß1 protein and transforming growth factor ß receptor 1 (TGF-ßR1) inhibitor SB431542 into mouse HSCs to detect the detailed impact of TGF-ß1 signaling on HSCs activation. TGF-ß1 signaling significantly increased phosphorylated (P)-Smad2/3 level and promoted Smad2/3 translocation from the cytoplasm to the nucleus, which also caused transdifferentiation of HSCs into MFs. Importantly, TGF-ß1 signaling also resulted in high expression of Notch pathway markers Notch1, Jagged1, Hes1 in HSCs. In contrast, expression of those above markers in mouse HSCs were obviously decreased after hampering TGF-ß1 signaling via TGF-ßR1 inhibitor SB431542. To further examine the effect of Notch pathway on HSCs activation process, TGF-ß1-stimulated HSCs and control HSCs were treated with or without LY450139, a specific inhibitor of Notch pathway. LY450139 evidently decreased the expression of Notch1 and MFs marker α-smooth muscle actin (α-SMA) expression in HSCs. These above results may provide a novel insight that TGF-ß1 signaling controls HSCs activation process through regulating the expression of Notch pathway markers.

[The clinical practice of improvement the "Volume and Quality" of functional liver in autologous liver transplantation for the patients with alveolar echinococcosis].

Objective: To investigate the clinical significance of accurate assessment of "volume and quality" of functional liver in Autologous liver transplantation (ALT) in the treatment of the advanced hepatic alveolar echinococcosis (HAE). Methods: The clinical data of 12 patients with advanced HAE who underwent ALT at the First Affiliated Hospital of Xinjiang Medical University from May 2015 to July 2016 were retrospectively analyzed. Results: The preoperative hepatic functions of 12 patients were 8 Child-Pugh Grade A, 1 Grade B, and 3 Grade C. Three of the patients had moderate or severe jaundice. Three of the patients calculated functional liver graft volume (GV) and standard liver volume (SLV) ratio (GV/SLV) were <30%. After the protection of liver function, anti-infection, percutaneous transhepatic cholangiography drainage (PTCD), selective portal vein embolization (PVE), and staging liver resection, liver function Child-Pugh grade of 11 patients was raised to A grade, and the other patient was B grade, meanwhile the bilirubin was reduced to 2 times the normal value. The GV/SLV ratios of 3 patients with low GV/SLV ratio had reached 44.4%, 47.2% and 56.2% respectively. In this study, the GV/SLV ratios of the 12 patients were between 73.2% and 40.8% with an average of 55.6%. Operation time was 11.5-20.5 h, with an average of 12.3 h. Anhepatic phase time was 193-375 min with median 253.5 min. The red blood cell suspension was 0-6 U during the operation. The average hospitalization was 10-42 d, with the average 22.7 d. Total hospital costs were 121 600-434 800 Yuan, with the median cost of 174 400 Yuan. One patient died of septic shock a week after surgery. Conclusion: (1)ALT may provide feasibility for the advanced HAE. (2)Accurate assessment of functional liver "volume and quality" appeared as the key points to the ALT. (3)Precise surgery and individualized treatment could improve and protect the functional liver "volume and quality" .

[Application of three-dimensional visualization technology in surgical treatment for patients with hepatic alveolar echinococcosis].

OBJECTIVE: To determine the clinical value of three-dimensional(3D) visualization technology in pre-operative assessment and surgical planning for patients with hepatic alveolar echinococcosis. METHODS: Eighty-five hepatic alveolar echinococcosis patients received surgical treatment in the First Affiliated Hospital of Xinjiang Medical University between May 2011 and May 2015.3D reconstruction and virtual surgeries were performed on diseased livers using a 3D visualization reconstruction system for liver, which based on the data set of 64-slice CT from those patients and indicated the feasibility and safety of liver resection. The pre-operative measurement results were compared with intra-operative conditions to verify the accuracy of pre-operative evaluation. RESULTS: All surgical strategies of patients underwent surgical treatment(59 of 85 received traditional liver resection and 26 of 85 received liver auto-transplantation)were consistent with pre-operative surgical planning in 3D reconstruction. Furthermore, the pre-operative resection liver volume((751±510)cm(3)) estimated by 3D calculation method was positively correlated with the actual weight((777±567)g) after the surgery(r=0.990), and the error rate was 4.7%; the pre-operative remaining liver volume((829±157)cm(3)) estimated by 3D calculation method was positively correlated with the actual weight((770±206) g) after the surgery(r=0.978). Patients were followed for 6-46 months after the surgery, and 3 post-operative death and 2 recurrence (one case received secondary surgery and one case received drug therapy) were reported during the follow-up period. CONCLUSIONS: A liver 3D visualization technology has application value in the pre-assessment and surgical planning.When it combined with ultrasound, CT and MRI, traditional examinations, the liver 3D visualization technology can effectively improve the success rate of operation, reduce the risks of surgery.

[Living donor liver transplantation for the treatment complicated hepatic cystic echinococcosis: first case report from China].

OBJECTIVE: To report the first clinical experience of living donor liver transplantation(LDLT) in patient with complicated hepatic cystic echinococcosis in China. METHODS: First Affiliated Hospital of Xinjiang Medical University successfully implemented LDLT in the treatment of one patient with complicated hepatic cystic echinococcosis after four times liver surgery and secondary congestive cirrhosis on May 9, 2015. The clinical data of the patient has been retrospectively analyzed. RESULTS: LDLT procedure was successfully performed in a patient with four previous liver intervention due to his recurrence. The patient was lack of right lobe, while with significant hypertrophy in left lobe. The reoccurred lesion compressed the retrohepatic vena cava, left hepatic vein, portal vein, that led to the stricture of retrohepatic vena cava, left hepatic vein orifice. The phlebography displayed remarkable stricture in retrohepatic vena cava and rich collateral circulation by which the venous blood flow back to the right atrium via paravertebral vein. The orifice of left hepatic vein shaped as hair-like so that the stenting was impossibly. After the multi-disciplinary team discussion, the liver transplantation was proposed, while the autotransplantation was considered as first line option, and with the backup of living related donor. The laparotomy showed the hugely enlarged liver with nodular changes. The frozen section was tested twice and showed as spotty necrosis with pseudo-lobe formation. Although the enlarged left lobe about 2 000 g weight, the liver colored as grey with cotton-like texture, the plan for autotransplantation was abandoned regarding the high risk for post-operative liver failure. Therefore, LDLT was performed. The donor was her brother who donated right lobe with 685 g weight. The operation time was 1 005 min with anhepatic time 335 min. Total of 12 units red blood cell suspension was transfused. The coagulating function was back to normal in 3 days after operation. The patient was discharged at 30 days after operation, while the routine blood test and biochemical markers had back to normal levels. The patient had taken tacrolimus and methylprednisolone with normal life. The liver functions of transplanted liver were normal in 90 days after surgery. The patient was able to self-activity and regular outpatient follow-up. CONCLUSION: Hepatic CE is an infectious disease, and liver transplantation could be the last choice for complicated case, especially in combination with severe complications, like Budd-Chiari syndrome.

[Research Achievements and Challenges for Echinococcosis Control].

Echinococcosis is an important communicable disease that has remarkable impacts on the global health. The disease is highly endemic in western China. In the last decades, achievements were obtained for the surgery and drug therapies for echinococcosis, as well as for studies on genomics, signaling pathways, and liver proliferation and injury of the intermediate hosts. Although steps have entered vaccine development, challenges remainin immunodiagnosis and drug treatment for intermediate hosts, and in vaccine development for definitive hosts. This paper gives an overview on the current achievements and challenges for echinococcosis control.

Surgical treatment for hepatic alveolar echinococcosis: report of 50 cases.

BACKGROUND/AIMS: The purpose of this study is to present the surgical treatment of hepatic alveolar echinococcosis and evaluate the treatment outcomes. METHODOLOGY: A retrospective analysis was made on 50 patients admitted between 2000 and 2008. All the patients were divided into two groups, 26 patients for the radical surgery group and 24 for the palliative surgery group. RESULTS: In the palliative surgery group, 10 patients died during the follow-up period, 2 patients presented with brain metastasis and 4 patients with lung metastasis, 1 patient had multiple lesions in the liver, 1 patient who received left hepatectomy had a new lesion in caudate lobe of the liver, 4 patients had postoperative jaundice, 3 had cholangitis and 4 patients had biliary leaks. There were also 3 patients lost in the follow-up period. In the radical surgery group, 22 patients were alive and asymptomatic without recurrence during the follow-up period, 3 patients had recurrent cholangitis and 4 patients had biliary leaks. Three patients were lost during the followup period in this group. CONCLUSIONS: Radical resection appears to be best treatment of hepatic alveolar echinococcosis and palliative operation may cause a series of severe complications leading to death.