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Tran TT, Truong TYN, Nguyen HVK, Luu NAT, Than TT, Bui HT, Ho PD, Pirotte T, Reding R. Liver Transplantation for Children With Budd-Chiari Syndrome: A Case Report From Vietnam and Literature Review. Pediatr Transplant 2025; 29:e70038. [PMID: 39900470 DOI: 10.1111/petr.70038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2024] [Revised: 01/10/2025] [Accepted: 01/18/2025] [Indexed: 02/05/2025]
Abstract
Budd-Chiari syndrome (BCS) presents with various degrees of liver damage, and the choice of treatment depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the final treatment option when other interventions are not feasible and when the liver injury is irreversible. We report a case of a pediatric patient with BCS who underwent liver transplantation from a living donor in the context of thrombophilic disorder. CASE PRESENTATION A 14-month-old girl was admitted to the hospital with ascites. She was malnourished, and an abdominal CT scan confirmed significant ascites with no visualization of the hepatic veins and retrohepatic inferior vena cava (IVC). A liver biopsy revealed fibrosis, necrosis, and parenchymal hemorrhage. Patient's portal hypertension was managed with prophylactic beta-blocker Propranolol and endoscopic esophageal variceal ligation. However, she was hospitalized four times due to gastrointestinal bleeding from ruptured esophageal varices. Protein C deficiency was found as probable etiology of BCS. The patient underwent liver transplantation at 3 years and 8 months old with a liver from a parental living donor. The surgery and postoperative course were uneventful, and the patient was discharged 25 days after the transplant. CLINICAL DISCUSSION Hypercoagulability is often the underlying cause of BCS. Maintaining anticoagulation/thrombophilic balance postoperatively contributed to the successful liver transplantation in this pediatric patient. CONCLUSION Liver transplantation is a safe and effective treatment for pediatric patients with BCS who meet the criteria for the procedure.
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Affiliation(s)
- Thanh Tri Tran
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Thi Yen Nhi Truong
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Hong Van Khanh Nguyen
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Nguyen An Thuan Luu
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | | | - Hai Trung Bui
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Phi Duy Ho
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Thierry Pirotte
- Department of Anesthesiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Raymond Reding
- Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Brussels, Belgium
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Tekbaş A, Schilling K, Fahrner R, Morath O, Malessa C, Bauschke A, Settmacher U, Rauchfuß F. Liver Transplantation for Budd-Chiari Syndrome From Myeloproliferative Neoplasms - Management and Long-Term Results. Transplant Proc 2024; 56:1759-1765. [PMID: 39237386 DOI: 10.1016/j.transproceed.2024.08.040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/08/2024] [Accepted: 08/24/2024] [Indexed: 09/07/2024]
Abstract
Myeloproliferative neoplasms can cause primary Budd-Chiari-Syndrome with acute or chronic liver failure necessitating liver transplantation. However, preventing the recurrence remains challenging and the need for post-transplant anticoagulant and cytoreductive treatment is not sufficiently clear. We analyzed the treatment regimens for all patients who presented to our department with PBCS from MPN between 2004 and 2021. Eight patients underwent liver transplantation - 6 of them due to an acute liver failure. Post-transplant, all patients received anticoagulant and 7 patients cytoreductive medication. The mean survival after transplantation was 13.25 years. Liver transplantation shows favorable long-term outcome when combined with post-transplant anticoagulant and cytoreductive treatment.
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Affiliation(s)
- Aysun Tekbaş
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Friedrich Schiller University, Jena, Germany; Research Programme "Advanced Clinician Scientist Programme" by the Interdisciplinary Center of Clinical Research of the Medical Faculty Jena, Jena University Hospital, Friedrich Schiller University, Jena, Germany.
| | - Kristina Schilling
- Department of Hematology and Medical Oncology, Jena University Hospital, Friedrich Schiller University, Jena, Germany
| | - René Fahrner
- Spital Thun, Department of General and Visceral Surgery, Thun, Switzerland
| | - Olga Morath
- Department of Hematology and Medical Oncology, Jena University Hospital, Friedrich Schiller University, Jena, Germany; Research Programme "DFG Clinician Scientist Programme Organ Age" by the Interdisciplinary Center of Clinical Research of the Medical Faculty Jena, Jena University Hospital, Friedrich Schiller University, Jena, Germany
| | - Christina Malessa
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Friedrich Schiller University, Jena, Germany
| | - Astrid Bauschke
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Friedrich Schiller University, Jena, Germany
| | - Utz Settmacher
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Friedrich Schiller University, Jena, Germany
| | - Falk Rauchfuß
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Friedrich Schiller University, Jena, Germany
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Yang XY, Le-Nguyen A, Alvarez F, Rong ZX, Borsuk D, Piché N, Lallier M, Beaunoyer M. Pediatric living donor liver transplant for Budd-Chiari syndrome using a cryopreserved pulmonary vein graft for retro-hepatic vena cava reconstruction: A case report. Pediatr Transplant 2024; 28:e14674. [PMID: 38054589 DOI: 10.1111/petr.14674] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/07/2023] [Revised: 11/10/2023] [Accepted: 11/25/2023] [Indexed: 12/07/2023]
Abstract
INTRODUCTION In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure. CONCLUSION IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.
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Affiliation(s)
- Xin Yu Yang
- Department of General Surgery, Université de Montréal, Montréal, Québec, Canada
| | - Annie Le-Nguyen
- Department of Pediatric Surgery, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Fernando Alvarez
- Department of Pediatric Gastroenterology, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Zhi Xia Rong
- Department of Hepato-pancreato-biliary Surgery, Centre hospitalier de l'Université de Montréal, Montréal, Québec, Canada
| | - Daniel Borsuk
- Department of Pediatric Plastic Surgery, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Nelson Piché
- Department of Pediatric Surgery, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Michel Lallier
- Department of Pediatric Surgery, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
| | - Mona Beaunoyer
- Department of Pediatric Surgery, Centre hospitalier universitaire Sainte-Justine, Montréal, Québec, Canada
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Samanta A, Sen Sarma M, Yadav R. Budd-Chiari syndrome in children: Challenges and outcome. World J Hepatol 2023; 15:1174-1187. [DOI: 10.4254/wjh.v15.i11.1174] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2023] [Revised: 10/08/2023] [Accepted: 10/30/2023] [Indexed: 11/24/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.
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Affiliation(s)
- Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Rajanikant Yadav
- Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
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Sanada Y, Sakuma Y, Onishi Y, Okada N, Hirata Y, Horiuchi T, Omameuda T, Lefor AK, Sata N. Surgical approach to supradiaphragmatic inferior vena cava in patients with metallic stent in the hepatic vein during repeat liver transplantation. Pediatr Transplant 2022; 26:e14402. [PMID: 36237167 DOI: 10.1111/petr.14402] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2022] [Revised: 08/05/2022] [Accepted: 09/18/2022] [Indexed: 12/01/2022]
Abstract
BACKGROUND Repeat liver transplantation (LT) for patients with the liver graft failure who underwent metallic stent placement in the previous graft hepatic vein (HV) for HV complications can be very difficult. We retrospectively reviewed the safer surgical procedures during repeat LT for patients with a metallic stent in the graft HV. CASE REPORTS Patient 1 with biliary atresia who was treated with metallic stent placement for HV stenosis underwent a third LT form a deceased donor at the age 17 years. Patient 2 with ornithine transcarbamylase deficiency who was treated with metallic stent placement for refractory HV stenosis underwent a second LT form a deceased donor at age 9 years. In both patients, transection of the previous graft HV through an intraabdominal approach was difficult during repeat LT, and a supradiaphragmatic inferior vena cava (IVC) approach was introduced. Using a midline incision of the diaphragm, the pericardium was incised and the supradiaphragmatic IVC was encircled. After clamping the supradiaphragmatic IVC, graft hepatectomy was performed. The metallic stent was successfully removed breaking, and HV reconstruction was performed on the suprahepatic IVC. Both patients did well without serious HV complications after repeat LT. CONCLUSIONS The surgical technique for the supradiaphragmatic IVC approach is useful to decrease the risk of fatal operative complications during repeat LT for patients with a metallic stent in the graft HV.
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Affiliation(s)
- Yukihiro Sanada
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Yasunaru Sakuma
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Yasuharu Onishi
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Noriki Okada
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Yuta Hirata
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Toshio Horiuchi
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Takahiko Omameuda
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Alan Kawarai Lefor
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
| | - Naohiro Sata
- Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Shimotsuke, Japan
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Living Donor Liver Transplantation for Budd-Chiari Syndrome: A Propensity Score-Matched Analysis. World J Surg 2022; 46:2806-2816. [PMID: 36071288 DOI: 10.1007/s00268-022-06709-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/14/2022] [Indexed: 10/14/2022]
Abstract
INTRODUCTION There are unique technical and management challenges associated with living donor liver transplantation (LDLT) for Budd-Chiari Syndrome (BCS). The outcomes of LDLT for BCS in comparison to other indications remain unclear and warrant elucidation. METHODS Data of 24 BCS patients who underwent LDLT between January 2012 and June 2019 were analyzed. There were 20 adults and 4 children. The early and long-term outcomes of adult LDLT BCS patients were compared to a control group of LDLT patients for other indications and matched using propensity scoring methodology. RESULTS Primary BCS was observed in 18 (90%) patients. Caval replacement was performed in 7 (35%) patients. Early and late hepatic venous outflow tract (HVOT) complications were seen in 1 (5%) and 3 (15%) patients. Preoperative acute kidney injury was identified as a risk factor for mortality in the BCS cohort (p = 0.013). On comparison, BCS recipients were younger with fewer comorbidities, more large volume ascites and higher rates of PVT. They also had longer cold ischemia time, increased blood loss and transfusion requirements, increased hospital stay, and higher late outflow complications. The 1-year and 3-year survivals were similar to non-BCS cohort (84.2% vs. 94% and 71.3% vs. 91.9%, respectively, log rank test p = 0.09). CONCLUSION LDLT is a good option for symptomatic BCS who have failed non-transplant interventions. The clinical and risk factor profile of BCS recipients is distinct from non-BCS recipients. By following an algorithmic management protocol, we show on propensity-score matched analysis that outcomes of LDLT for BCS are similar to non-BCS indications.
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Aktas H, Ozer A, Yilmaz TU, Keceoglu S, Can MG, Emiroglu R. Liver transplantation for Budd-Chiari syndrome: A challenging but handable procedure. Asian J Surg 2021; 45:1396-1402. [PMID: 34688524 DOI: 10.1016/j.asjsur.2021.09.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2021] [Revised: 08/26/2021] [Accepted: 09/26/2021] [Indexed: 11/25/2022] Open
Abstract
BACKROUND Establishing venous outflow in liver transplantation for patients with Budd-Chiari syndrome is crucial and requires various surgical techniques. The outcomes of these patients exibits distinct problems including vasculary thrombosis and biliary complications. METHODS In this single center study, the outcomes and surgical features of 33 patients with Budd-Chiari syndrome who were carried out liver translantation (27 patients from living donor). Another group was formed among patients underwent liver transplantation due to other etiologies and the outcomes were compared. RESULTS The most-seen type was the classical type of Budd-Chiari syndrome (25, 75.8%). For twenty-six patients inherited or acquired prothrombotic disorders were identified (78.2%) in Budd-Chiari group. Average follow-up was 29.7 ± 15.5 months. We have observed no recurrence of disease in our BCS patients. When the two groups was compared in terms of thrombotic complications, there was a significantly increased risk in BCS group (p = 0.014). Our 1 and 3-year survival rates for the BCS group were 81.8% and 78.8%, respectively. In the control group, 1 and 3-year survival rates were 93.3% and 88.9%, respectively. Log-rank test analyses showed no statistically significant results. CONCLUSION Liver transplantation with individual surgical and postoperative treatment strategy for patients with Budd-Chiari syndrome provides comparable outcomes.
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Affiliation(s)
- Hikmet Aktas
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Ali Ozer
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey.
| | - Tonguc Utku Yilmaz
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Selim Keceoglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Meltem Guner Can
- Department of Anaesthesiology and Reanimation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Remzi Emiroglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
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Wang C, Qiu Y, Wang W. Application of ex vivo liver resection and autotransplantation in treating Budd-Chiari syndrome secondary to end-stage hepatic alveolar echinococcosis: A case series. Medicine (Baltimore) 2021; 100:e27075. [PMID: 34449508 PMCID: PMC8389916 DOI: 10.1097/md.0000000000027075] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/23/2021] [Accepted: 08/12/2021] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Secondary Budd-Chiari syndrome (BCS) occurs due to a blockage in the liver caused by invasion or compression by a large lesion. Conventional treatments for BCS do not solve practical problems, wherease liver transplantation has been only applied as a last-resort therapy and as the only opportunity for a radical cure. We explored the feasibility of applying ex vivo liver resection and autotransplantation (ELRA) for the new indications of treating patients with end-stage hepatic alveolar echinococcosis (HAE). Our center has firstly proposed the idea and successfully treated the 49 patients with HAE. This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE. METHODS According to the degree of lesion invasion and surgical options, 11 patients were divided into 4 types. These 11 patients had large lesions that invaded the second and third hilum of the caudate lobe and involved the confluence of the hepatic vein and the inferior vena cava, suprahepatic vena cava, or at least 2 hepatic veins and led to secondary BCS. The aim of the present work was to report 11 patients with life-threatening diseases who underwent ELRA (ex vivo liver resection and autotransplantation) for secondary BCS, to propose a classification system for secondary BCS, and to suggest that secondary BCS is an indication for ELRA. RESULTS Eleven patients successfully underwent ELRA without intraoperative mortality. The median autograft weight was 690 g (440-950 g); operative time was 12.5 hours (9.4-16.5 hours); Postoperative hospital stay was 15 days (7-21 days). Clavien-Dindo grade IIIa or higher postoperative complications occurred in only 5 patients. CONCLUSIONS This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE, not only provides new ideas for alternative treatments of secondary BCS, but also provides a classification system for secondary BCS. This article describes the technical process of outflow tract reconstruction and the experience for expanding the indications for ELRA. Our study demonstrated that ELRA is well feasible for treatment of BCS secondary to advanced HAE.
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Affiliation(s)
- Cong Wang
- Department of Liver Surgery, Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, Sichuan Province
- Department of Hepatopancreatobiliary Surgery, Affiliated Hospital of Qinghai University, Xining, Qinghai Province, China
| | - Yiwen Qiu
- Department of Liver Surgery, Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, Sichuan Province
| | - WenTao Wang
- Department of Liver Surgery, Liver Transplantation Center, West China Hospital of Sichuan University, Chengdu, Sichuan Province
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Une N, Tokodai K, Kanai N, Saitoh Y, Ohta M, Sasaki K, Miyazawa K, Kashiwadate T, Fujio A, Nakanishi W, Miyagi S, Unno M, Kamei T. Living donor liver transplantation for Budd‒Chiari syndrome with right posterior segment graft and patch plasty using the superficial femoral vein: a case report. Surg Case Rep 2021; 7:136. [PMID: 34086114 PMCID: PMC8178427 DOI: 10.1186/s40792-021-01224-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2021] [Accepted: 05/31/2021] [Indexed: 11/23/2022] Open
Abstract
Background In living donor liver transplantation (LDLT) for patients with Budd‒Chiari syndrome (BCS), there are several concerns about reconstruction of the inferior vena cava (IVC) and hepatic veins. Herein, we report the case of a patient with BCS who underwent LDLT with right posterior segment graft (RPSG) and patch plasty for reconstruction of the hepatic venous outflow, using the patient’s own superficial femoral vein (SFV). Case presentation A 19-year-old man, who was diagnosed with primary BCS, underwent LDLT. His main hepatic veins were totally obstructed, and membranous stenosis was seen in the IVC. The LDLT donor was his mother; however, liver volumetric analysis showed that only her RPSG was appropriate. In the recipient surgery, 16 cm of the left SFV was harvested and was cut longitudinally and opened. The right hepatic vein (RHV) of the RPSG was anastomosed to the sidewall of the SFV graft. After explantation of native diseased liver was completed, the stenotic and thickened wall of the IVC was widely resected, and a large anastomotic orifice was created. Patch cavoplasty was performed with the RHV‒SFV graft patch. After portal reperfusion started, hepatic venous outflow was satisfactory, and there was no venous graft congestion. Both his postoperative course and his long-term course after discharge were uneventful. Conclusions In LDLT for BCS patients, ingenuity is required for the reconstruction of venous outflow. The SFV patch can be safely harvested from liver transplant recipients and is suitable for venous reconstruction. In addition, RPSG is an alternative type of liver graft for LDLT if a conventional right- or left-lobe graft cannot be used.
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Affiliation(s)
- Norikazu Une
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan.
| | - Kazuaki Tokodai
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Norifumi Kanai
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Yoshikatsu Saitoh
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Mineto Ohta
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Kengo Sasaki
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Koji Miyazawa
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Toshiaki Kashiwadate
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Atsushi Fujio
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Wataru Nakanishi
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Shigehito Miyagi
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Michiaki Unno
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
| | - Takashi Kamei
- Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, Japan
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Leal-Leyte P, Valamparampil J, Thambithurai R, Shanmugam NP, Reddy MS, Rela M. Non-standard hepatic venous reconstruction in the setting of absent inferior vena cava in partial graft pediatric liver transplantation-A matched retrospective cohort study. Pediatr Transplant 2021; 25:e13834. [PMID: 32959953 DOI: 10.1111/petr.13834] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2020] [Revised: 07/20/2020] [Accepted: 08/08/2020] [Indexed: 12/11/2022]
Abstract
Recipient cava may be unavailable for outflow reconstruction in some children undergoing liver transplantation (PLT) due to caval agenesis, tumor, or fibrotic caval occlusion. Non-standard hepatic venous reconstruction (NHVR) with a direct veno-caval anastomosis or neo-cava reconstruction is necessary in such cases. Retrospective review of all PLT needing NHVR performed in our unit from January 2010 to September 2019 was performed. Outcomes of this group were compared to a 2:1 matched control group who underwent transplantation with standard piggyback technique. Fifteen children (4.9%) of 304 PLT recipients underwent NHVR. Caval agenesis in biliary atresia (n = 5, 33%) and hepatoblastoma infiltrating the cava (n = 4, 27%) were the commonest indications. Ten children had neo-cava reconstruction, while 5 had direct anastomosis to the supra-hepatic caval cuff or right atrium. One child had developed neo-cava thrombosis without graft venous outflow obstruction in the post-operative period. There was no significant difference in major morbidity, need for re-operation (20% vs 16.7%; P = 1.00), hospital stay (24 days, vs 21 days; P = .32), graft & patient survival among the study and control groups. Absent or inadequate recipient cava during PLT with a partial liver graft can be safely managed with technical modifications. Results equivalent to standard piggyback implantation can be achieved.
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Affiliation(s)
- Pilar Leal-Leyte
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India.,Liver Transplant Study Group Mexico, Hospital Angeles Acoxpa, Mexico City, Mexico
| | - Joseph Valamparampil
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India
| | - Ravikumar Thambithurai
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India.,Bharath Institute of Higher Education & Research, Chennai, India
| | - Naresh P Shanmugam
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India.,Bharath Institute of Higher Education & Research, Chennai, India
| | - Mettu Srinivas Reddy
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India.,Bharath Institute of Higher Education & Research, Chennai, India
| | - Mohamed Rela
- Institute of Liver Disease and Transplantation, Dr. Rela Institute & Medical Centre, Chennai, India.,Bharath Institute of Higher Education & Research, Chennai, India
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Rocha-Santos V, Waisberg DR, Pinheiro RS, Nacif LS, Arantes RM, Ducatti L, Martino RB, Haddad LB, Galvao FH, Andraus W, Carneiro-D'Alburquerque LA. Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis: A case report and review of the literature. World J Hepatol 2021; 13:151-161. [PMID: 33584994 PMCID: PMC7856862 DOI: 10.4254/wjh.v13.i1.151] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2020] [Revised: 11/12/2020] [Accepted: 11/29/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is a challenging indication for liver transplantation (LT) due to a combination of massive liver, increased bleeding, retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava (IVC). Occasionally, it may be totally thrombosed, increasing the complexity of the procedure, as it should also be resected. The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC; thus, it may be necessary to reconstruct it.
CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction. It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed, up to almost the right atrium. A right-lobe graft was retrieved from his sister, with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein. Owing to massive subcutaneous collaterals in the abdominal wall, venovenous bypass was implemented before incising the skin. The right atrium was reached via a transdiaphragramatic approach. Hepatectomy was performed en bloc with the retrohepatic vena cava. It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor. The patient remains well on outpatient clinic follow-up 25 mo after the procedure, under an anticoagulation protocol with warfarin.
CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.
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Affiliation(s)
- Vinicius Rocha-Santos
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Daniel Reis Waisberg
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Rafael Soares Pinheiro
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Lucas Souto Nacif
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Rubens Macedo Arantes
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Liliana Ducatti
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Rodrigo Bronze Martino
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Luciana Bertocco Haddad
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
| | - Flavio Henrique Galvao
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
- Department of Gastroenterology, Laboratory of Medical Investigation 37 (LIM-37), Faculdade de Medicina da Universidade de Sao Paulo (FMUSP), Sao Paulo 01246903, Brazil
| | - Wellington Andraus
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
- Department of Gastroenterology, Laboratory of Medical Investigation 37 (LIM-37), Faculdade de Medicina da Universidade de Sao Paulo (FMUSP), Sao Paulo 01246903, Brazil
| | - Luiz Augusto Carneiro-D'Alburquerque
- Department of Gastroenterology, Abdominal Organs Transplantation Division, Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo (HC-FMUSP), Sao Paulo 05403900, SP, Brazil
- Department of Gastroenterology, Laboratory of Medical Investigation 37 (LIM-37), Faculdade de Medicina da Universidade de Sao Paulo (FMUSP), Sao Paulo 01246903, Brazil
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Obed M, Othman MI, Hammoudi S, Chattab MA, Jarrad A, Bashir A, Obed A. Living Donor Liver Transplant in Patients With Budd-Chiari Syndrome: A Single-Center Experience at Our University Hospital. EXP CLIN TRANSPLANT 2020; 18:796-802. [PMID: 33349208 DOI: 10.6002/ect.2020.0331] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
OBJECTIVES Budd-Chiari syndrome is an infrequent, but potentially fatal, hepatic condition with the clinical manifestation of obstructed venous drainage. This may lead to progressive hepatic congestion, portal hypertension, and, ultimately, liver failure. If medical, interventional, and surgical approaches are not effective, liver transplant offers a rescue modality. The primary objective of this study was to report the perioperative and, above all, the vascular challenges associated with living donor liver transplant in patients with Budd-Chiari syndrome. MATERIALS AND METHODS We retrospectively reviewed demographic and clinical characteristics of 6 patients with Budd-Chiari syndrome who underwent living donor liver transplant at our transplant center from April 2004 to July 2020. We also evaluated all data regarding perioperative course, surgical outcome, and the postoperative follow-up period. RESULTS All patients displayed advanced liver disease with a Child-Pugh score C. The mean calculated Model for End-Stage Liver Disease score was 32. The causes of Budd-Chiari syndrome were factor V Leiden thrombophilia in 1 patient, myeloproliferative disorder in 3 patients, antiphospholipid antibody syndrome in 1 patient, and a protein C deficiency in 1 patient. The mean age of patients was 40 years. One of the 6 patients was female. All patients had living donor liver transplant from immediate kin according to Jordanian allocation rules. The mean graft-to-recipient weight ratio was 0.9, and the median follow-up period was 89 months. Cumulative 1-, 3-, and 5-year-survival rates were 84%, 67%, and 67%, respectively. CONCLUSIONS Good survival rates are achievable with living donor liver transplant for patients with advanced Budd-Chiari syndrome, particularly by means of posterior cavoplasty for enlargement of the cava orifice. Therefore, in countries with insufficient deceased donor programs, such as Jordan, living donor liver transplant may be a lifesaving therapeutic possibility.
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Affiliation(s)
- Mikal Obed
- From the Hepatology, Gastroenterology, and Hepatobiliary/Transplant Unit Jordan Hospital, Amman, Jordan
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13
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Gonultas F, Akbulut S, Barut B, Usta S, Kutluturk K, Kutlu R, Yilmaz S. Usability of Inferior Vena Cava Interposition Graft During Living Donor Liver Transplantation: Is This Approach Always Necessary? J Gastrointest Surg 2020; 24:1540-1551. [PMID: 31385171 DOI: 10.1007/s11605-019-04342-6] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2019] [Accepted: 07/22/2019] [Indexed: 02/07/2023]
Abstract
PURPOSE To share the outcome of caval reconstruction technique in patients who underwent living donor liver transplantation (LDLT) with inferior vena cava (IVC) interposition grafting. METHODS Between January 2009 and December 2018, an artificial or homologous interposition vascular graft was used for the continuity of resected native (IVC) due to various reasons in 29 of 1740 patients who underwent LDLT at our institute. Demographic, clinical, and radiological data were prospectively collected and retrospectively analyzed. RESULTS Sixteen female and 13 male patients ranging 6-67 years of age were included. Right, left, and left lobe lateral segments were used in 22, 5, and 2 patients, respectively. The three leading LDLT indications were primary or idiopathic Budd-Chiari syndrome (BCS) (n = 12), alveolar echinococcosis (n = 7), and secondary BCS (n = 5). The three leading indications for IVC interposition grafting were thrombosis, dense fibrosis, and IVC invasion caused by tumor or echinococcosis. Homologous IVC graft was used in 17, homologous aortic graft in 7, and Dacron graft in 5 patients. Throughout the follow-up period, ascites ± pleural effusion and elevated liver enzymes were detected in 12 and 4 patients, respectively. Stenosis and/or thrombosis requiring one or more procedures such as 1-6 sessions balloon angioplasty, stent, and thrombus aspiration were observed in half of the patients. CONCLUSION Retrohepatic IVC damages are not a contraindication for LDLT. The presence or absence of venous collateral circulation is an important indicator of the need for IVC interposition graft use.
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Affiliation(s)
- Fatih Gonultas
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey
| | - Sami Akbulut
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey.
| | - Bora Barut
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey
| | - Sertac Usta
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey
| | - Koray Kutluturk
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey
| | - Ramazan Kutlu
- Department of Radiology, Faculty of Medicine, Inonu University, 44280, Malatya, Turkey
| | - Sezai Yilmaz
- Liver Transplant Institute, Inonu University, Elazig Yolu 10. Km, 44280, Malatya, Turkey
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14
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Zhang Y, Xie P, Yang C, Yang H, Liu J, Zhou G, Deng S, Lau WY. Percutaneous stenting of left hepatic vein followed by Ex vivo Liver Resection and Autotransplantation in a patient with hepatic alveolar echinococcosis with Budd-Chiari syndrome. Int J Surg Case Rep 2020; 68:251-256. [PMID: 32199250 PMCID: PMC7082604 DOI: 10.1016/j.ijscr.2020.03.004] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2020] [Accepted: 03/02/2020] [Indexed: 12/21/2022] Open
Abstract
A metal mesh stent was placed in the left hepatic vein of a 45-year-old man who presented with Budd-Chiari syndrome in stage 1. After disappearance of ascites and improvement in liver function, ELRA was performed in stage 2. Follow-up examination at 6 months showed normal liver function and no evidence of recurrence. In selected patients with Budd-Chiari syndreme, percutaneous stenting followed by ELRA represent an curative treatment option. Background and aims Infiltration of hepatic venous outflow in hepatic alveolar echinococcosis can lead to development of Budd-Chiari syndrome. Medical treatment of this condition is generally unsatisfactory. Radical hepatic resection is impossible for extensive parasitic involvement of liver. This is a case report on a patient who was successfully treated with percutaneous stenting of left hepatic vein followed by Ex vivo Liver Resection and Autotransplantation (ELRA). Methods Using the transjugular approach, a metal mesh stent was placed in the left hepatic vein of a 45-year-old man who presented with Budd-Chiari syndrome. After disappearance of ascites and improvement in liver function, Ex vivo Liver Resection and Autotransplantation were performed. Results The patient underwent left hepatic vein stenting for Budd-Chiari syndrome without complication. Three months later, liver biopsy showed fibrous proliferation of interlobular portal areas and normal hepatic lobules. After successful Ex vivo Liver Resection and Autotransplantation, follow-up examination at 6 months showed normal liver function and no evidence of recurrence. Conclusions Allotransplantation is an accepted treatment for advanced hepatic alveolar echinococcosis with Budd-Chiari syndrome. However, shortage of organ donors and need for immunosuppression are challenging problems. In selected patients with hepatic vein stenoses presenting as Budd-Chiari syndreme, percutaneous stenting of hepatic veins followed by ex vivo liver resection and autotransplantation represent an alternative curative treatment option.
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Affiliation(s)
- Yu Zhang
- Organ Transplantation Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Ping Xie
- Organ Transplantation Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Chong Yang
- Organ Transplantation Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Hongji Yang
- Organ Transplantation Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Jun Liu
- Ultrasonography Department, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Guo Zhou
- Ultrasonography Department, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China
| | - Shaoping Deng
- Organ Transplantation Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Clinical Medicine, University of Electronic Science and Technology of China, Chengdu 610072, Sichuan, China.
| | - Wan Yee Lau
- Faculty of Medicine, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, N.T., Hong Kong Special Administrative Region.
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15
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Surgical Techniques and Long-term Outcomes of Living-donor Liver Transplantation With Inferior Vena Cava Replacement Using Atriocaval Synthetic Interposition Graft for Budd-Chiari Syndrome. Ann Surg 2020; 269:e43-e45. [PMID: 30080720 DOI: 10.1097/sla.0000000000002847] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
OBJECTIVE We aimed to describe our living-donor liver transplantation (LDLT) surgical technique and its long-term patency for patients with Budd-Chiari syndrome (BCS) and retrohepatic inferior vena cava (IVC) obstruction that extends up to the atrium. BACKGROUND From a technical perspective, LDLT for BCS with an IVC obstruction up to the right atrium is one of the most challenging surgical procedures. Consequently, the optimal surgical technique for patients with BCS has not yet been elucidated. METHODS A durable LDLT technique without piggy-back hepatectomy was designed using a large-caliber synthetic interposition vascular graft between the right atrium and the infrahepatic IVC for reconstructing the hepatic outflow tract in patients with BCS. RESULTS Between May 2006 and May 2017, 5 of 17 BCS patients who underwent LDLT required the described technique. All patients with a median follow-up of 10.5 years (range, 9.2-11.5 years) demonstrated the patent IVC grafts and no recurrence of BCS. CONCLUSIONS Our refined technique does not require unnecessary and dangerous dissection of the diseased IVC, and eliminates the residual suprahepatic vena cava with the possibility of BCS recurrence by connecting the graft to the healthy atrium.
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16
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Karaca CA, Yilmaz C, Farajov R, Iakobadze Z, Kilic K, Buket S, Kilic M. Hepatic Venous Outflow Reconstruction Directly Into the Right Atrium. EXP CLIN TRANSPLANT 2019. [PMID: 31801445 DOI: 10.6002/ect.2019.0299] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
OBJECTIVES Living-donor liver transplant for BuddChiari syndrome is particularly challenging because of the need for venous outflow reconstruction as grafts from living donors lack vena cava. In addition, recipient vena cava may be thrombotic and fibrotic to such an extent that it would not allow graft venous outflow reconstruction. Under these circumstances, the right atrium provides an easily accessible alternative for venous outflow reconstruction, omitting the need for vena cava replacement. MATERIALS AND METHODS Data from 3 patients who were treated using this technique were collected and evaluated with regard to surgical technique and outcomes. RESULTS All patients were alive without vascular complications after a mean follow-up of 67 months. The applied surgicaltechnique was similar except with regard to vena cava preservation. CONCLUSIONS During the natural course of the disease, venous collaterals form as chronic thrombosis extends into the vena cava. The vena cava can be safely resected in these patients to facilitate hepatectomy through dense adhesions, which is another common clinical problem in this disease. Consequently, venous outflow reconstruction to the right atrium creates the feasible opportunity of draining the graftliver without having to replace the vena cava.
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Affiliation(s)
- Can A Karaca
- From the Izmir University of Economics, Faculty of Medicine, Izmir, Turkey
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17
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Karaca C, Yilmaz C, Ferecov R, Iakobadze Z, Kilic K, Caglayan L, Aydogdu S, Kilic M. Living-Donor Liver Transplantation for Budd-Chiari Syndrome: Case Series. Transplant Proc 2018; 49:1841-1847. [PMID: 28923635 DOI: 10.1016/j.transproceed.2017.04.028] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2017] [Accepted: 04/08/2017] [Indexed: 12/14/2022]
Abstract
BACKGROUND Venous reconstruction in living-donor liver transplantation for Budd-Chiari syndrome (BCS) has challenges because the grafts from living donors lack vena cava, and hepatic venous anastomosis must be performed on an already-thrombosed and/or stenosed inferior vena cava. Several techniques are described to overcome this problem, and we represent our experience with 22 patients. METHODS Medical recordings of 22 patients were retrospectively collected, and disease-specific data as well as recordings about surgical technique were analyzed. RESULTS Creation of a wide, triangular de novo orifice was the main method used for venous drainage, which was used in 19 patients. The remaining 3 patients had totally thrombosed vena cava; thus, direct anastomosis to the supra-hepatic portion of the vena cava was used in 2 patients and an anastomosis to the right atrium was used in 1 patient. CONCLUSIONS Venous reconstruction in BCS can be achieved without the use of patch-plasty, and the inferior vena cava can be safely resected in selected patients. Living-donor liver transplantation is a feasible option for the treatment of BCS, considering the scarcity of cavaderic donors.
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Affiliation(s)
- C Karaca
- Department of General Surgery, Izmir University of Economics, Faculty of Medicine, Izmir, Turkey
| | - C Yilmaz
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey
| | - R Ferecov
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey
| | - Z Iakobadze
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey
| | - K Kilic
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey
| | - L Caglayan
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey
| | - S Aydogdu
- Department of Pediatrics, Ege University Faculty of Medicine, Izmir, Turkey
| | - M Kilic
- Department of Liver Transplantation, Izmir Kent Hospital, Izmir, Turkey.
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18
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Living donor liver transplantation for adult Budd Chiari syndrome - Resection without replacement of retrohepatic IVC: A case report. Int J Surg Case Rep 2017; 42:50-54. [PMID: 29216531 PMCID: PMC5724988 DOI: 10.1016/j.ijscr.2017.11.050] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2017] [Revised: 11/23/2017] [Accepted: 11/28/2017] [Indexed: 01/03/2023] Open
Abstract
Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction. Liver transplantation is a definitive treatment of BCS, indicated in failure of other treatment modalities or end stage liver diseases. The Budd-Chiari syndrome patients with IVC obstruction can receive liver transplantation with IVC removal and without IVC replacement. Living donor liver transplant with refined techniques can be performed safety with stable hemodynamics peri-and post-liver transplantation. Introduction Suprahepatic caval resection and replacement of inferior vena cava (IVC) is standard procedure in deceased donor liver transplantation for patients with Budd-Chiari syndrome (BCS). However, replacement of IVC in living donor liver transplantation (LDLT) is difficult. We report a case of BCS successfully treated by LDLT without replacement of IVC. Presentation of case A 52-years-old female with a primary BCS due to IVC thrombosis. A vena cava (VC) stent placed after angioplasty without improvement of the hepatic, portal venous flow and liver functions, Transjugular intrahepatic portosystemic shunt was considered and the patient had a rapid deterioration and increased ascites. The patient was scheduled for living donor liver transplantation (LDLT). Her Child-Paugh and MELD scores were 11, 18, respectively at time of transplantation. Left lobe was obtained from her son. Preservation of the native suprarenal IVC was impossible due to massive fibrosis and thrombosed. The suprahepatic IVC was also fibrotic and unsuitable for anastomosis with hepatic vein. The retrohepatic IVC resected include suprahepatic IVC together with the liver. The supradiaphragmatic IVC was reached and encircled through opening the diaphragm around the IVC and a vascular clamp applied on the right atrium with subsequent anastomosis with hepatic vein of the graft. The hemodynamic stability of the patient was maintained throughout the operation without IVC replacement due to developed collateral vessels. Conclusion Patients with Budd-Chiari syndrome with obstructive IVC are successfully treated with living donor liver transplantation without replacement of IVC.
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Pahari H, Chaudhary RJ, Thiagarajan S, Raut V, Babu R, Bhangui P, Goja S, Rastogi A, Vohra V, Soin AS. Hepatic Venous and Inferior Vena Cava Morphology No Longer a Barrier to Living Donor Liver Transplantation for Budd-Chiari Syndrome: Surgical Techniques and Outcomes. Transplant Proc 2017; 48:2732-2737. [PMID: 27788809 DOI: 10.1016/j.transproceed.2016.08.009] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2016] [Accepted: 08/03/2016] [Indexed: 12/26/2022]
Abstract
BACKGROUND Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) has been reported with <10 inferior vena cava (IVC) replacements with vascular/synthetic graft. The goal of this study was to review outcomes of LDLT for BCS at our center, with an emphasis on surgical techniques and postoperative anticoagulation therapy. METHODS Between October 2011 and December 2015, a total of 1027 LDLTs were performed. Nine of these patients had BCS. We analyzed their etiologies, operative details, postoperative complications, and outcomes. RESULTS The indication was chronic liver disease for all patients. Two patients required retrohepatic IVC replacement with a polytetrafluoroethylene graft due to severe adhesions and thrombosis, respectively. One patient required V-Y plasty for suprahepatic IVC narrowing. Five patients had portal venous thrombosis, 3 treated by thrombectomy, and 1 by renoportal anastomosis. The mean follow-up time was 18 ± 16 months. Only 1 early death occurred due to sepsis. The anticoagulation therapy involved heparin infusion from postoperative day 1, conversion to low-molecular-weight-heparin on postoperative days 3 to 6, followed by warfarin (postoperative days 9-16 to maintain an international normalized ratio of 2-3 long term), along with low-dose aspirin for 6 months. There was no recurrence of thrombosis. CONCLUSIONS LDLT for BCS is well documented in literature. Prevention of recurrent thrombosis depends on meticulous surgical technique, perfect and wide outflow anastomoses, and a strict anticoagulation protocol. A synthetic (polytetrafluoroethylene) graft for IVC interposition is a safe and feasible option for reconstruction with good results. Low-dose aspirin with low-molecular-weight-heparin later converted to warfarin provides excellent results and prevents recurrence of thrombosis.
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Affiliation(s)
- H Pahari
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India.
| | - R J Chaudhary
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - S Thiagarajan
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - V Raut
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - R Babu
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - P Bhangui
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - S Goja
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - A Rastogi
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
| | - V Vohra
- Department of Liver Transplant, GI Anaesthesia and Intensive Care, Medanta The Medicity, Gurgaon, India
| | - A S Soin
- Medanta Liver Institute, Medanta The Medicity, Gurgaon, India
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20
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Hidaka M, Eguchi S. Budd-Chiari syndrome: Focus on surgical treatment. Hepatol Res 2017; 47:142-148. [PMID: 27249222 DOI: 10.1111/hepr.12752] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/09/2016] [Revised: 05/11/2016] [Accepted: 05/24/2016] [Indexed: 12/28/2022]
Abstract
Budd-Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC. Liver transplantation (LT) is usually indicated as a treatment for liver failure despite various treatments. The outcomes of LT are good, with a 5-year survival after LT of nearly 70%.
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Affiliation(s)
- Masaaki Hidaka
- Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
| | - Susumu Eguchi
- Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
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21
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Ara C, Akbulut S, Ince V, Karakas S, Baskiran A, Yilmaz S. Living donor liver transplantation for Budd-Chiari syndrome: Overcoming a troublesome situation. Medicine (Baltimore) 2016; 95:e5136. [PMID: 27787368 PMCID: PMC5089097 DOI: 10.1097/md.0000000000005136] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND The aim of the study was to report the detailed surgical techniques of living donor liver transplantation (LDLT) in patients with Budd-Chiari syndrome (BCS). METHODS Demographic and surgical techniques characteristics of 39 patients with BCS who underwent LDLT were retrospectively reviewed. Thirty-two of them had native vena cava inferior (VCI) preservation and 6 had retrohepatic VCI resection with venous continuity established by cryopreserved VCI (n = 4) or aortic graft (n = 2). In 1 patient, the anastomosis was established between the graft hepatic vein (HV) and the suprahepatic VCI. For preservation of the native VCI, immediately before the graft implantation, the thickened anterior, and right/left lateral walls of the recipient VCI were resected caudally and cranially until the intact vein wall was reached, and then an anastomosis was created between the (HV) of the graft reconstructed as a circumferential fence and the reconstructed recipient VCI. For resection of the retrohepatic VCI, the anastomosis was created with the same technique in all 6 patients in whom VCI was reformed by using a vascular graft. RESULTS Post-LT complications developed in 19 of the patients. Complications related to the biliary anastomosis accounted for 12 of these cases, with 11 treated by PTC and/or ERCP, and 1 by hepaticojejunostomy. Two of the 39 patients developed recurrent BCS and were treated by interventional radiological methods. Thirteen patients died and none were related to the BCS recurrence. CONCLUSION Favorable outcomes are achievable with LDLT treatment of patients with BCS, which carries important implications for countries with inadequate cadaveric donor pools.
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Affiliation(s)
| | - Sami Akbulut
- Department of Surgery and Liver Transplant Institute, Inonu University Faculty of Medicine, Malatya, Turkey
- Correspondence: Sami Akbulut, Department of Surgery and Liver Transplant Institute, Inonu University Faculty of Medicine, Turgut Ozal Medical Center, Malatya, Turkey (e-mail: )
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Alnajjar A, Al-Hussaini H, Al Sebayel M, Al-Kattan W, Elsiesy H. Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocythemia: Case Report. [Corrected]. Transplant Proc 2016; 47:2282-6. [PMID: 26361700 DOI: 10.1016/j.transproceed.2015.05.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2015] [Accepted: 05/14/2015] [Indexed: 02/01/2023]
Abstract
Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)-like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, and abnormal liver function tests and coagulation profile. Imaging revealed hepatic vein thrombosis, confirming Budd-Chiari syndrome, and a 6.2 × 6.1 × 6.8 cm lesion in segment 8 of the liver. Primary cause of Budd-Chiari syndrome was essential thrombocythemia according to bone marrow biopsy and molecular testing results. The patient was treated medically and underwent transjugular intrahepatic portosystemic shunt insertion. The lesion in segment 8 continued to enlarge. Cadaveric liver transplantation was carried out. On gross and histologic examination of the explanted liver, the lesion was found to be a true FNH.
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Affiliation(s)
- A Alnajjar
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
| | - H Al-Hussaini
- Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - M Al Sebayel
- Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
| | - W Al-Kattan
- College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
| | - H Elsiesy
- Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
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Park GC, Song GW, Moon DB, Lee SG. A review of current status of living donor liver transplantation. Hepatobiliary Surg Nutr 2016; 5:107-17. [PMID: 27115004 DOI: 10.3978/j.issn.2304-3881.2015.08.04] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Living donor liver transplantation (LDLT) has become an inevitable procedure in Asia due to its shortage of deceased donor under the influence of the religion and native cultures. Through a broad variety of experience, LDLT has been evolved and extended its indication. Although there have been many surgical and ethical efforts to prevent donor risk, concerns of donor's safety still are remaining questions due to its strict selection criteria. Therefore, dual grafts LDLT or ABO incompatible (ABO-I) LDLT may be effective means in its application and safety aspect. Many Asian LDLT centers have pointed out the useful extended criteria of LDLT for hepatocellular carcinoma (HCC), but the applicability of extended criteria should be validated and standardized by worldwide prospective studies based on the Milan criteria. Recent struggling efforts have been reported to surmount extensive portal vein thrombosis and Budd-Chiari syndrome which were previously contraindicated to LDLT. There is no doubt that LDLT is a surely complicated therapy to be performed successfully and requires devoted efforts by surgeons and co-workers. Nonetheless, comprehensive increasing understandings of partial graft LT and improvements of surgical techniques with challenges to obstacles in LDLT will make its prosperity with satisfactory outcomes.
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Affiliation(s)
- Gil-Chun Park
- Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gi-Won Song
- Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Deok-Bog Moon
- Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Sung-Gyu Lee
- Division of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Abstract
OBJECTIVES This study sought to evaluate the indications, techniques, and results of inferior vena cava (IVC) replacement at living donor liver transplantation (LDLT). MATERIALS AND METHODS We performed 821 LDLTs and 11 (1.3%) patients required concomitant IVC replacement. We analyzed the indications, replacement materials, and outcomes. RESULTS Right, left, and left lateral liver lobes were transplanted in 7, 2, and 2 patients, respectively. The indications for IVC replacement were thrombosis/fibrosis in 7 patients (Budd-Chiari 4, hereditary tyrosinemia 1, congenital hepatic fibrosis 1, cryptogenic 1), involvement with mass in 3 patients (Echinococcus alveolaris 2, hepatoblastoma 1) and iatrogenic narrowing at IVC in 1 patient. Cryopreserved grafts (aorta n = 5, IVC n = 4, iliac vein n = 1) or synthetic graft (n = 1) were used for replacements. In 1 patient, hepatic outflow obstruction developed at 39 days and was treated successfully by interventional radiology. There was only 1 hospital mortality (8.9%) that was unrelated to caval replacement (subarachnoid hemorrhage). Of the remaining patients, the caval grafts were patent after a mean 7.7 months of follow-up (range 1 to 17 months). CONCLUSIONS Although rare, IVC replacement can be necessary at LDLT. Budd-Chiari and E. alveolaris are the main underlying diseases for replacement requirements. Caval replacement with cryopreserved vascular grafts can provide successful short-term and long-term patency.
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Cetinkunar S, Ince V, Ozdemir F, Ersan V, Yaylak F, Unal B, Yilmaz S. Living-Donor Liver Transplantation for Budd-Chiari Syndrome--Resection and Reconstruction of the Suprahepatic Inferior Vena Cava With the Use of Cadaveric Aortic Allograft: Case Report. Transplant Proc 2016; 47:1537-9. [PMID: 26093762 DOI: 10.1016/j.transproceed.2015.04.043] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Living-donor liver transplantation with inferior vena cava resection and reconstruction is rarely indicated for Budd-Chiari syndrome. The aim of this case presentation was to present and discuss the inferior vena cava reconstruction with the use of cadaveric aortic allograft after resection of the suprahepatic inferior vena cava in a patient with Budd-Chiari syndrome who was treated with living-donor liver transplantation. CASE REPORT A 29-year-old male patient with end-stage liver disease and suprahepatic inferior vena cava obstruction was referred to our center. He was scheduled for living-donor liver transplantation. The suprahepatic inferior vena cava was resected and reconstruction was achieved by means of interposition of the cadaveric aortic allograft between the right atrium and inferior vena cava. Postoperative course was uneventful. DISCUSSION Liver transplantation and vena cava reconstruction is indicated in some patients with end-stage liver disease and Budd-Chiari syndrome. Limitations in cadaveric organ donation may be compensated for with the use of living-donor liver. In this condition, various aspects of inferior vena cava reconstruction may be discussed. CONCLUSIONS Budd-Chiari syndrome due to suprahepatic inferior vena cava obstruction close to the right atrium may be treated with vascular reconstruction with the use of a cadaveric aortic allograft.
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Affiliation(s)
- S Cetinkunar
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey.
| | - V Ince
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
| | - F Ozdemir
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
| | - V Ersan
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
| | - F Yaylak
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
| | - B Unal
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
| | - S Yilmaz
- Department of Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey
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26
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Akamatsu N, Sugawara Y, Kokudo N. Budd-Chiari syndrome and liver transplantation. Intractable Rare Dis Res 2015; 4:24-32. [PMID: 25674385 PMCID: PMC4322592 DOI: 10.5582/irdr.2014.01031] [Citation(s) in RCA: 42] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/10/2014] [Revised: 12/23/2014] [Accepted: 12/25/2015] [Indexed: 12/13/2022] Open
Abstract
Budd-Chiari syndrome involves obstruction of hepatic venous outflow tracts at various levels from small hepatic veins to the inferior vena cava and is the result of thrombosis or its fibrous sequelae. There is a conspicuous difference in its etiology in the West and the East. Myeloproliferative disease predominates in the West and obstruction of the vena cava predominates in the East. The clinical presentation and clinical manifestations are so varied that it should be suspected in any patient with acute or chronic liver dysfunction. It should be treated with step-wise management. First-line therapy should be anticoagulation with medical treatment of the underlying illness, and interventional revascularization and TIPS are indicated in the event of a lack of response to medical therapy. Liver transplantation may be indicated as a rescue treatment or for fulminant cases with promising results. This step-by-step strategy has achieved a 5-year transplant-free survival rate of 70% and a 5-year overall survival rate of 90%. Living donor liver transplantation can also be used for patients with Budd-Chiari syndrome if deceased donor livers are scarce, but it requires a difficult procedure particularly with regard to venous outflow reconstruction.
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Affiliation(s)
- Nobuhisa Akamatsu
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Yasuhiko Sugawara
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
- Address correspondence to: Dr. Yasuhiko Sugawara, Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. E-mail:
| | - Norihiro Kokudo
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Saleh Y, Eldeen FZ, Kamel Y, Kabbani M, Al Sebayel M, Broering D. Liver transplant in Budd-Chiari syndrome: a single-center experience in Saudi Arabia. EXP CLIN TRANSPLANT 2015; 12:52-4. [PMID: 24471724 DOI: 10.6002/ect.2013.0153] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
OBJECTIVES If they do not respond to other treatments, patients with Budd-Chiari syndrome are potential candidates for a liver transplant. Timing for transplant is controversial; however, before other systems deteriorate, early intervention in relatively stable patient may improve the outcome and survival of these patients. MATERIALS AND METHODS Six patients (2 women and 4 men) had Budd-Chiari syndrome (1.2%) among 475 patients who had undergone a liver transplant at our center between 2001 and 2012. Imaging modalities including duplex ultrasound, abdominal computed tomography angiography, and hematologic evaluation were part of our routine diagnostic work-up. Although we perform mostly living-donor liver transplants, these patients received a liver transplant from a deceased donor, because there was not enough evidence to justify a living-donor liver transplant. We thought that not replacing the caval vein might negatively influence the outcome. Postoperatively, these recipients were started on a heparin infusion and triple therapy immunosuppression; only then was warfarin introduced as long-term anticoagulant. RESULTS Two patients died, 1 from uncontrollable bleeding and disseminated intravascular coagulopathy, and the other died in the intensive care unit after 5 months because of multiorgan failure and sepsis. One patient had portal vein thrombosis 9 months after the liver transplant; the other patient needed a liver retransplant after 5 years owing to liver failure, secondary to chronic rejection. Graft survival rate was 75%, and patient survival rate was 66.6%. CONCLUSIONS This is the first article from Saudi Arabia to describe the outcome of a liver transplant in this subgroup of patients with Budd-Chiari syndrome. Treatment of Budd-Chiari syndrome follows a therapeutic algorithm that should start with anticoagulation and may end up with liver transplant; however, it should be considered early if other treatments fail.
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Affiliation(s)
- Yahia Saleh
- King Faisal Specialist Hospital and Research Hospital, Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, Riyadh, Saudi Arabia
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Abstract
PURPOSE OF REVIEW Living donor liver transplantation (LDLT) continues to evolve, generating interesting issues on the applicability and safety of new techniques. RECENT FINDINGS Specific selection criteria and standardized surgical techniques with high ethical and medical standards are needed to minimize donor risk. In this aspect, minimally invasive donor hepatectomy has caused controversies. The reproducibility and safety of pure laparoscopic major hepatectomy in LDLT remains uncertain. Therefore, a stepwise approach is needed to avoid unnecessary donor risk. To expand the living donor pool, dual graft and ABO-incompatible LDLT have emerged as well tolerated and effective methods. The extended selection criteria for hepatocellular carcinoma in LDLT appear acceptable to balance donor risk and recipient outcome. However, these criteria should be validated based on the risk-benefit ratio. Despite technical advances, technical challenges persist such as Budd-Chiari syndrome and portal vein thrombosis. To address these issues, several innovative surgical techniques have been proposed and have shown promising results. SUMMARY LDLT is associated with donor safety concerns, technical complexity, and small-for-size issues. Nonetheless, accumulated experience and technical know-how from large-volume Asian LDLT centers have led to progress in LDLT. Further technical refinement and investigation to overcome the disadvantages of partial grafts will broaden the applicability of LDLT.
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30
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Sakçak I, Eriş C, Ölmez A, Kayaalp C, Yılmaz S. Replacement of the vena cava with aortic graft for living donor liver transplantation in Budd-Chiari syndrome associated with hydatid cyst surgery: a case report. Transplant Proc 2013; 44:1757-8. [PMID: 22841264 DOI: 10.1016/j.transproceed.2012.04.023] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
A 12-year-old girl, operated because of a hydatid cyst of the liver, with Budd-Chiari syndrome was evaluated for postoperative development of ascites and paraumbilical varicose veins. A vena caval stent was placed for the relief of inferior vena caval obstruction. The patient was admitted because of progressive deterioration in ascites and liver functions. Imaging techniques showed degeneration adjacent to the right hepatic vein in liver segments 7 to 8, a partially calcified 5-cm hydatid cyst, and a thrombosis in the inferior vena cava was that addressed with a 10-cm metal stent. A living donor segments 2 to 3 liver transplantation was obtained from the patient's mother. After completion of the donor operation without complications, the vena caval stent was removed following the recipient hepatectomy. Suprarenal flow continued after resection of the fibrotic vena cava and placement of a cadaveric cryopreserved aortic graft for the vena cava, anastomosed between the suprarenal and subdiaphragmatic segments of the vena cava. An end-to-side anastomosis was performed between the left hepatic vein of the donor liver and the aortic graft. There was no complication and the patient was discharged on postoperative day 19. Follow-up Doppler ultrasonography showed the aortic vena caval graft to be open, along with the hepatic/portal vein and hepatic artery. This case demonstrated that operations for liver hydatid cyst surgeries can iatrogenically induce Budd-Chiari syndrome; a cryopreserved aortic graft can be an alternative to ensure the continuity of the vena cava in living donor liver transplantation.
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Affiliation(s)
- I Sakçak
- Department of General Surgery, University of Inönü, Malatya, Turkey.
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31
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Gomes AC, Rubino G, Pinto C, Cipriano A, Furtado E, Gonçalves I. Budd-Chiari syndrome in children and outcome after liver transplant. Pediatr Transplant 2012; 16:E338-41. [PMID: 22452639 DOI: 10.1111/j.1399-3046.2012.01683.x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
BCS is a rare form of portal hypertension in children. The authors describe two cases of BCS with differing presentations. Case 1: Previously healthy four-yr-old girl. BCS was diagnosed during the course of an episode of acute gastroenteritis with dehydration. Despite conservative therapy for two months, the condition was progressive resulting in liver failure leading ultimately to LT. Molecular studies showed that she was heterozygous for the Factor (F) V Leiden. At follow-up, six yr post-LT (two yr without anticoagulation therapy), no thromboembolic/bleeding events were apparent. Case 2: Three-yr-old boy with IgA deficiency and liver disease. Following a febrile episode, he developed fulminant liver failure requiring urgent LT from a living donor (father). Molecular studies disclosed MTHFR C677T homozygosity and FV Leiden heterozygosity. The father was homozygous for the MTHFR mutation. Three months post-LT, persistent graft dysfunction was associated with stenosis of the IVC, which improved upon stent placement. He received dipyridamole and aspirin for five yr, after which time dipyridamole was discontinued. Evidence is sparse on the follow-up of BCS cases with liver transplant. The authors discuss their findings, particularly the need for long-term anticoagulation.
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Affiliation(s)
- Ana Cristina Gomes
- Hospital Pediátrico de Coimbra, Centro Hospitalar da Universidade de Coimbra, Coimbra, Portugal.
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32
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Fukuda A, Ogura Y, Kanazawa H, Mori A, Kawaguchi M, Takada Y, Uemoto S. Living donor liver transplantation for Budd-Chiari syndrome with hepatic inferior vena cava obstruction after open pericardial procedures. Surg Today 2012. [PMID: 23188387 DOI: 10.1007/s00595-012-0440-1] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
Living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) presents a unique challenge as it does not involve replacement of the hepatic inferior vena cava (IVC). We report a case of successful LDLT in a patient with BCS associated with occlusion of the hepatic veins as well as the IVC. A 34-year-old woman with a history of two open pericardial procedures had decompensated liver failure and portal hypertension. Venography showed complete obstruction of the hepatic IVC and well-developed collateral vessels. We performed LDLT via sternotomy and laparotomy, with an end-to-end anastomosis between the left hepatic vein of the donor and the patient's suprahepatic vena cava in the pericardium. The patient recovered uneventfully and has been doing well for 5 years. LDLT without caval replacement for BCS in a patient with hepatic IVC obstruction is feasible if the patient has good functional collaterals before liver transplantation.
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Affiliation(s)
- Akinari Fukuda
- Division of Hepato-Biliary-Pancreatic Surgery and Transplantation, Department of Surgery, Kyoto University Hospital, Kyoto, Japan,
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Ogura Y, Kanazawa H, Yoshizawa A, Nitta T, Ikeda T, Uemoto S. Supradiaphragmatic approach for Budd-Chiari syndrome with transjugular intrahepatic portosystemic shunt stent in combination with inferior vena cava reconstruction during living donor liver transplantation: a case report. Transplant Proc 2011; 43:2093-6. [PMID: 21693334 DOI: 10.1016/j.transproceed.2011.03.046] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2011] [Accepted: 03/15/2011] [Indexed: 02/08/2023]
Abstract
Treatment of Budd-Chiari syndrome consists of medical management, surgical shunt, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation. Liver transplantation is indicated only when other treatments have failed. A 36-year-old Japanese man underwent living-donor liver transplantation after radiologic intervention procedures. Because of the position of the TIPS stent and the damaged vascular lesion of Budd-Chiari syndrome, a supradiaphragmatic approach was employed to achieve a safe total hepatectomy. Moreover, after resection of damaged portion of the inferior vena cava (IVC), an artificial vascular graft was utilized to fill the IVC gap. The postoperative course was uneventful; no serious complications were experienced within 2 years after liver transplantation. This supradiaphragmatic IVC approach and IVC reconstruction technique emphasized the option of surgical techniques to decrease the operative risk during liver transplantation for Budd-Chiari syndrome.
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Affiliation(s)
- Y Ogura
- Department of Surgery, HPB and Transplantation Surgery, Kyoto University Hospital, Kyoto City, Japan.
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Soyama A, Eguchi S, Yanaga K, Takatsuki M, Hidaka M, Kanematsu T. Living donor liver transplantation with extensive caval thrombectomy for acute-on-chronic Budd-Chiari syndrome. Surg Today 2011; 41:1026-8. [PMID: 21748628 DOI: 10.1007/s00595-010-4376-z] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2009] [Accepted: 02/18/2010] [Indexed: 11/25/2022]
Abstract
The key consideration when performing living donor liver transplantation (LDLT) in patients with Budd-Chiari syndrome (BCS) is careful management of a stenotic or occluded inferior vena cava (IVC), because it is not possible to replace the recipient stenotic or occluded IVC with donor IVC as in cadaver donor transplantation. We describe how we performed LDLT with extensive thrombectomy in a patient with acute-on-chronic BCS with a totally thrombosed retrohepatic IVC. The operation was successful and the patient remains well, with follow-up images showing a patent IVC and hepatic veins. To our knowledge, LDLT for a BCS patient with severe extensive caval thrombus has never been reported before. We consider that the successful outcome of this patient clearly demonstrates the feasibility of our technique of extensive thrombectomy, without a vessel graft, to manage a stenotic or occluded IVC in LDLT in patients with BCS.
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Affiliation(s)
- Akihiko Soyama
- Department of Surgery, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan
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35
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Shirai Y, Yoshiji H, Ko S, Yamazaki M, Ikenaka Y, Noguchi R, Morioka C, Kaji K, Aihara Y, Nakanishi K, Yamao J, Toyohara M, Mitoro A, Sawai M, Yoshida M, Fujimoto M, Uemura M, Nakajima Y, Fukui H. Salvage living donor liver transplantation after percutaneous transluminal angioplasty for recurrent Budd-Chiari syndrome: a case report. J Med Case Rep 2011; 5:124. [PMID: 21447168 PMCID: PMC3078858 DOI: 10.1186/1752-1947-5-124] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2010] [Accepted: 03/29/2011] [Indexed: 01/25/2023] Open
Abstract
INTRODUCTION Budd-Chiari syndrome is a very rare pathological entity that ultimately leads to liver failure. Several therapeutic modalities, including percutaneous transluminal angioplasty, have been attempted to save the life of patients with Budd-Chiari syndrome. Few reports have described a salvage living donor liver transplantation performed after percutaneous transluminal angioplasty in a patient with acute Budd-Chiari syndrome. CASE PRESENTATION A 26-year-old Japanese man developed severe progressive manifestations, such as massive ascites and hematemesis due to rupture of esophageal varices. After making several investigations, we diagnosed the case as Budd-Chiari syndrome. We first performed percutaneous transluminal angioplasty to dilate a short-segment stenosis of his inferior vena cava. The first percutaneous transluminal angioplasty greatly improved the clinical manifestations. However, after a year, re-stenosis was detected, and a second percutaneous transluminal angioplasty failed to open the severe stricture of his inferior vena cava. Since our patient had manifestations of acute liver failure, we decided to perform salvage living donor liver transplantation from his brother. The transplantation was successfully performed and all clinical manifestations were remarkably alleviated. CONCLUSION In cases of recurrent Budd-Chiari syndrome, the blocked hepatic venous outflow is not always relieved, even with invasive therapies. We have to take into account the possibility of adopting alternative salvage therapies if the first therapeutic modalities fail. When invasive therapy such as percutaneous transluminal angioplasty fails, liver transplantation should be considered as an alternative option.
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Affiliation(s)
- Yusaku Shirai
- Third Department of Internal Medicine, Nara Medical University, Nara, Japan.
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36
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Choi GS, Park JB, Jung GO, Chun JM, Kim JM, Moon JI, Kwon CHD, Kim SJ, Joh JW, Lee SK. Living donor liver transplantation in Budd-Chiari syndrome: a single-center experience. Transplant Proc 2010; 42:839-42. [PMID: 20430186 DOI: 10.1016/j.transproceed.2010.02.045] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Budd-Chiari syndrome (BCS), which is characterized by hepatic venous outflow obstruction due to occlusion of the major hepatic vein and/or the inferior vena cava (IVC), is rare. Traditionally, a caval resection is advocated for these patients; however, such a maneuver renders living donor liver transplantation (LDLT) impossible. We encountered BCS in 4/377 LDLT patients during a 5-year period (January 2003 to December 2007). This report examine the various surgical modifications in these 4 patients, who underwent to LDLT for BCS. Resection of right hepatic vein (RHV) with an adjacent fibrotic part of the IVC with direct anastomosis of the graft RHV to the IVC was performed in 2 patients. One patient underwent retrohepatic IVC excision and reconstruction with a cryopreserved autologous IVC graft. The fourth patient, with a preexisting mesoatrial shunt for BCS, underwent conversion of this to a RHV atrial shunt. Graft and patient survivals were 100%. There were few complications in either donors or recipients. LDLT for BCS can be performed safely with adequate venous drainage techniques and with anticoagulant therapy and good follow-up for early diagnosis and treatment of recurrence leading to excellent long-term results.
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Affiliation(s)
- G S Choi
- Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
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Zahn A, Gotthardt D, Weiss KH, Richter G, Schmidt J, Stremmel W, Sauer P. Budd-Chiari syndrome: long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt. BMC Gastroenterol 2010; 10:25. [PMID: 20193077 PMCID: PMC2838758 DOI: 10.1186/1471-230x-10-25] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2009] [Accepted: 03/01/2010] [Indexed: 12/24/2022] Open
Abstract
Background Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic porto-systemic shunt (TIPS) and surgical interventions including orthotopic liver transplantation (OLT). Controlled trials or reports on larger cohorts are limited due to rare disease frequency. The aim of this study was to report our single centre long term results of patients with BCS receiving one of three treatment options i.e. medication only, TIPS or OLT on an individually based decision of our local expert group. Methods 20 patients with acute, subacute or chronic BCS were treated between 1988 and 2008. Clinical records were analysed with respect to underlying disease, therapeutic interventions, complications and overall outcome. Results 16 women and 4 men with a mean age of 34 ± 12 years (range: 14-60 years) at time of diagnosis were included. Myeloproliferative disorders or a plasmatic coagulopathy were identified as underlying disease in 13 patients, in the other patients the cause of BCS remained unclear. 12 patients presented with an acute BCS, 8 with a subacute or chronic disease. 13 patients underwent TIPS, 4 patients OLT as initial therapy, 2 patients required only symptomatic therapy, and one patient died from liver failure before any specific treatment could be initiated. Eleven of 13 TIPS patients required 2.5 ± 2.4 revisions (range: 0-8). One patient died from his underlying hematologic disease. The residual 12 patients still have stable liver function not requiring OLT. All 4 patients who underwent OLT as initial treatment, required re-OLT due to thrombembolic complications of the graft. Survival in the TIPS group was 92.3% and in the OLT group 75% during a median follow-up of 4 and 11.5 years, respectively. Conclusion Our results confirm the role of TIPS in the management of patients with acute, subacute and chronic BCS. The limited number of patients with OLT does not allow to draw a meaningful conclusion. However, the underlying disease may generate major complications, a reason why OLT should be limited to patients who cannot be managed by TIPS.
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Affiliation(s)
- Alexandra Zahn
- Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany.
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Kazimi M, Karaca C, Ozsoy M, Ozdemir M, Apaydin AZ, Ulukaya S, Zeytunlu M, Kilic M. Live donor liver transplantation for Budd-Chiari syndrome: anastomosis of the right hepatic vein to the right atrium. Liver Transpl 2009; 15:1374-7. [PMID: 19790160 DOI: 10.1002/lt.21815] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Affiliation(s)
- Mircelal Kazimi
- Department of Surgery, Ege University Hospital, Izmir, Turkey
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Kawaguchi Y, Tashiro H, Amano H, Kobayashi T, Irei T, Igarashi Y, Ide K, Oshita A, Itamoto T, Asahara T, Ohdan H. ABO-blood type incompatible living donor liver transplantation in a patient with Budd-Chiari Syndrome secondary to essential thrombocythemia. Hepatol Res 2009; 39:520-4. [PMID: 20849569 DOI: 10.1111/j.1872-034x.2008.00470.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/01/2023]
Abstract
Budd-Chiari syndrome (BCS) results from diverse causative factors. Myeloproliferative disorders (MPDs) including essential thrombocythemia (ET) account for a minority of BCS cases in Japan. ABO-blood-type incompatible living donor liver transplantation (LDLT) in adults has become an acceptable procedure owing to the development of new strategies for preventing antibody-mediated rejection. This report presents a rare case of BCS secondary to ET, which was cured by an ABO-incompatible (AB to A) LDLT. In this case, prostaglandin E(1) and gabexate mesilate were administered into portal vein and rituximab prophylaxis was applied. No splenectomy was performed as it is in most ABO-incompatible cases, since a flow cytometry showed no anti-B antibodies in the splenocytes collected by a wedge biopsy during the LDLT. The postoperative course was uneventful. Anti-coagulation therapy was initiated with aspirin and warfarin instead of hydroxyurea. This report describes an ABO-incompatible LDLT without a splenectomy for BCS secondary to ET.
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Affiliation(s)
- Yasuo Kawaguchi
- Department of Gastroenterological Surgery, Hiroshima University Hospital, Hiroshima, Japan
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Abstract
The technical success of cadaveric whole-size liver transplantation and better immunosuppressive drugs has extended the application of this life-saving procedure to include patients with irreversible acute and chronic liver diseases. However, because of the scarcity of cadaveric liver grafts, living-donor liver transplantation (LDLT) has emerged as an alternative to cadaveric-donor liver transplantation (CDLT), especially in Asia. In Korea, 8% of the population are hepatitis B virus (HBV) carriers, and the resultant HBV cirrhosis, with or without hepatocellular carcinoma (HCC), is common in the 40- to 60-year-old generation. Accordingly, many patients require orthotopic liver transplantation (OLT). In 1992, we started performing CDLTs in the Asan Medical Center. In 1994, the first successful pediatric LDLT was performed in Korea, on a 9-monthold infant with biliary atresia. In 1997, the first successful adult LDLT was performed in our department, using a left lobe, on a 37-year-old patient with HBV cirrhosis associated with HCC. Even after the first successful right-lobe LDLT, we faced the obstacle of anterior segment congestion of a right-lobe graft, and initiated reconstruction of the middle hepatic venous tributaries of a right-lobe graft in 1998. In 1999, we performed more than 100 OLTs a year. Insufficient graft size has hindered the expansion of adult LDLT, when the remaining left-lobe of potential donors is too small to assure donor safety. Dual two-left-lobe graft LDLT (transplanting from two donors into one recipient) was developed in 2000 to solve graft-size insufficiency and minimize donor risk. More than 200 OLTs a year have been performed since 2004, while broadening the indications for adult LDLT to near complete obstruction of the portal vein, with the application of intraoperative portography (IOP) and portal vein stenting. In 2007, 320 LTs were performed, including 276 adult LDLTs, 10 pediatric LDLTs, and 34 CDLTs (including 7 adult and 1 pediatric split-liver transplant). There has been no donor mortality in LDLT. With technical refinement and advanced perioperative care, the in-hospital mortality of recipients has dropped to 4%: attributed to the dedication of our liver transplantation team members.
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41
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Living donor liver transplantation with vena cava reconstruction using a cryopreserved allograft for a pediatric patient with Budd-Chiari syndrome. Transplantation 2009; 87:304-5. [PMID: 19155990 DOI: 10.1097/tp.0b013e3181938b10] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
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Liu C, Hsia CY, Loong CC, Perng CK, Huang CH, Tsai HL, Tsou MY, Wei C. A technique of diamond-shape venoplasty to reconstruct the hepatic venous outflow in living donor liver transplantation for a case of Budd-Chiari syndrome. Pediatr Transplant 2009; 13:35-8. [PMID: 18992058 DOI: 10.1111/j.1399-3046.2008.01059.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
It is difficult to reconstruct the hepatic venous outflow in LDLT for the case of BCS, especially when the thrombosis extends to the retro-hepatic and supra-hepatic vena cava. We reported on a technique of diamond-shape venoplasty which successfully treated such a case. The venoplasty used autologous vein grafts to overcome the size discrepancy between the orifices of supra-diaphragmatic inferior vena cava and hepatic vein of the liver graft.
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Affiliation(s)
- Chinsu Liu
- Division of Pediatric Surgery, Department of Surgery, Taipei Veterans General Hospital, Taipei, Taiwan.
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Jeng LB, Li PC, Yang MD, Lee CC, Chang CL, Wu RC. Artificial Vascular Graft for Inferior Vena Cava Reconstruction in Living Donor Liver Transplantation: A Case Report. Transplant Proc 2008; 40:2527-8. [DOI: 10.1016/j.transproceed.2008.07.024] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
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Horton JD, San Miguel FL, Membreno F, Wright F, Paima J, Foster P, Ortiz JA. Budd-Chiari syndrome: illustrated review of current management. Liver Int 2008; 28:455-66. [PMID: 18339072 DOI: 10.1111/j.1478-3231.2008.01684.x] [Citation(s) in RCA: 62] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.
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Affiliation(s)
- John D Horton
- William Beaumont Army Medical Center, Department of Surgery, El Paso, TX 79920-5001, USA.
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Jones VS, Sundaraj AP, O'Loughlin EV, Stormon M, Lord DJE, Shun A. Late-onset inferior vena cava obstruction in a shunted patient--a unique cause of rebleeding in children with portal hypertension. J Pediatr Surg 2007; 42:1953-6. [PMID: 18022456 DOI: 10.1016/j.jpedsurg.2007.07.048] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2007] [Revised: 07/11/2007] [Accepted: 07/14/2007] [Indexed: 11/28/2022]
Abstract
BACKGROUND Rebleeding in the presence of an adequate patent portosystemic shunt in a patient with portal hypertension (PHT) is uncommon. Inferior vena cava (IVC) obstruction as the cause of rebleeding in this situation has not been reported in the literature. METHODS Records from a pediatric tertiary care center were reviewed over a 15-year period. Portosystemic shunt procedures for bleeding esophageal varices were done in 39 children. Patients who, after a shunt surgery for PHT, developed a rebleed because of IVC obstruction in the presence of a patent shunt were identified. RESULTS AND CONCLUSIONS Late IVC obstruction in the presence of a patent shunt was identified in 2 patients. The etiology included adhesions, caudate lobe hypertrophy, and macronodular cirrhosis. Diagnosis was by angiography, and treatment included angioplasty and liver transplantation. Awareness of this condition helps direct treatment appropriately in the clinical scenario of a rebleed in a shunted patient with PHT.
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Affiliation(s)
- Vinci S Jones
- Department of Surgery, The Children's Hospital at Westmead, Sydney 2145, NSW, Australia.
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Yang F, Takada Y, Sakamoto S, Okamoto S, Uemoto S. Pseudotumoral azygos and paraesophageal varices of posterior mediastinum in a 15-month-old infant: a case report. Pediatr Transplant 2007; 11:804-6. [PMID: 17910662 DOI: 10.1111/j.1399-3046.2007.00775.x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
A case of azygos and paraesophageal varices presenting as a posterior mediastinal mass in a 15-month-old infant with biliary atresia is described. The patient was evaluated for living donor liver transplantation because of repeated cholangitis after Kasai operation, and plain CT scan demonstrated a mass in posterior mediastinum. The operation of mediastinal tumor resection was planned before liver transplantation in order to exclude malignant disease, however, possibility of paraesophageal varices remained. Contrast-enhanced magnetic resonance imaging clearly demonstrated azygos and paraesophageal varices in posterior mediastinum. Living donor liver transplantation was performed successfully without ligation of paraesophagogastric varices. Contrast-enhanced CT demonstrated distinctly decreased mediastinal mass one month after transplantation.
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Affiliation(s)
- Fuchun Yang
- Department of HPB and Transplant Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan
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Segev DL, Nguyen GC, Locke JE, Simpkins CE, Montgomery RA, Maley WR, Thuluvath PJ. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl 2007; 13:1285-94. [PMID: 17763380 DOI: 10.1002/lt.21220] [Citation(s) in RCA: 73] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Several treatment options exist for the management of Budd-Chiari syndrome (BCS), yet the relative role and timing of liver transplantation (LT) remain poorly defined. Small case series published to date have not been able to delineate the impact of comorbidities and thromboembolic complications of BCS on survival after LT. To better understand the outcomes after LT for BCS, we analyzed 510 liver transplants performed for this disease in the United States between 1987 and 2006. Risk factors predicting graft loss or patient death included increased recipient age, hyperbilirubinemia, elevated creatinine, life support or hospitalization at the time of transplantation, prior transplantation, prior abdominal surgery, increased donor age, and prolonged cold ischemic time (CIT). Prior transjugular intrahepatic portosystemic shunt (TIPS) was not associated with worse outcomes. Transplantation in the Model for End-Stage Liver Disease (MELD) era was associated with significantly lower risk of graft loss (hazard ratio [HR], 0.50; 95% confidence interval [CI], 0.30-0.86; P = 0.012) and death (HR, 0.52; 95% CI, 0.29-0.93; P = 0.027). Similarly, MELD era was associated with significantly lower risk of early graft loss (odds ratio [OR], 0.35; 95% CI, 0.16-0.79, P = 0.012) and early death (odds ratio, 0.37; 95% CI, 0.14-0.95; P = 0.040). However, patients with BCS transplanted in the MELD era were less likely to have life support, hospitalization, prior transplants, and prolonged cold ischemia times. In conclusion, outcomes of LT for BCS are excellent, with further improvements since 2002 associated with a selection shift imposed by MELD-based organ allocation.
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Affiliation(s)
- Dorry L Segev
- Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
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Shimoda M, Marubashi S, Dono K, Miyamoto A, Takeda Y, Nagano H, Umeshita K, Monden M. Utilization of autologous vein graft for replacement of the inferior vena cava in living-donor liver transplantation for obliterative hepatocavopathy. Transpl Int 2007; 20:804-7. [PMID: 17623051 DOI: 10.1111/j.1432-2277.2007.00519.x] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Obliterative hepatocavopathy (OHC) is a subtype of Budd-Chiari syndrome in which stenosis or obstruction of the retrohepatic inferior vena cava (IVC) is observed. Although IVC replacement is necessary in OHC patients, there are hardly any graft vessels available for IVC reconstruction during living-donor liver transplantation (LDLT). Here, we describe a novel technique of IVC reconstruction using only the autologous blood vessels in an OHC patient during LDLT. In this case, sufficient drainage of the hepatic outflow and reconstruction of the venous return from the lower half of the body were simultaneously required. Therefore, we substituted the retrohepatic IVC with the suprarenal IVC of the recipient, and we reconstructed the IVC continuity by using the autologous internal jugular vein and external iliac vein. The operation was safe, and the postoperative venous drainage from the hepatic tributaries was in good condition. This procedure might be an option for IVC replacement during LDLT.
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Affiliation(s)
- Masafumi Shimoda
- Department of Surgery, Osaka University Graduate School of Medicine, Osaka, Japan
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