Preoperative vascular evaluation in living donor liver transplantation for biliary atresia

Background

Liver transplantation is an important treatment option in the management of end-stage liver disease. Preoperative vascular evaluation plays an important role for a safe and successful operation, especially in pediatric patients undergoing living donor liver transplantation (LDLT).

Purpose

The purpose of this study is to assess the usefulness and accuracy of Doppler ultrasound (US), computed tomographic angiography (CTA), and magnetic resonance angiography (MRA) in evaluating vascular anomalies in patients with biliary atresia (BA) undergoing LDLT.

Methods and Materials

Images of Doppler US, CTA, and MRA for preoperative vascular evaluation in 55 patients with BA undergoing LDLT were reviewed with the operative findings.

Results

All patients underwent preoperative US, CTA, and MRA. Pathologic portal vein (n = 18), interruption of the retrohepatic vena cava (n = 1), and aberrant right hepatic artery from the superior mesenteric artery (n = 2) were confirmed during the transplantation. The success rates of CTA and MRA in identifying vascular anomalies were 96% and 82%, respectively (P = .01). The sensitivity, specificity, and accuracy of Doppler US were 89%, 94%, and 92%, respectively. For CTA, it was 94%, 97%, and 96%, respectively; for MRA (including technical failure), it was 75%, 97%, and 89%, respectively.

Conclusion

Doppler US serves as an initial assessment for vascular evaluation and has the advantage in determining vascular flow quantities. CTA and MRA are used for precise surgical planning. However, MRA has lower success and accuracy rates when compared with CTA (P = .01). Doppler US with CTA can provide accurate preoperative vascular imaging in patients with BA undergoing LDLT.     

Outcome of living donor liver transplantation for post-Kasai biliary atresia in adults.

Previous reports described the effectiveness of living donor liver transplantation (LDLT) for post-Kasai biliary atresia (BA) in the pediatric population. Information on the outcome of LDLT in patients that have reached adulthood after the Kasai procedure, however, is limited. A recent report postulated a poorer long-term outcome of LDLT in these adults. We reviewed our experience to evaluate the validity of this hypothesis. Between January 1996 and October 2006, 385 LDLTs were performed at our institution. There were 80 post-Kasai BA cases in the series; 60 (75%) were pediatric, and 20 (25%) were adults. There were no ABO blood type-incompatible cases. None were complicated with severe hepatopulmonary syndrome, portopulmonary hypertension, or hepatocellular carcinoma. The 5-year overall survival rates were 90% for the adults and 90% for the children (P > 0.99). The median follow-up period was 7 years in the adults and 11 years in the children. There was no donor mortality. The outcome of LDLT in adult post-Kasai BA patients in the present series was satisfactory; that is, adult and pediatric patient survival rates were not different. This finding suggests that for post-Kasai BA patients without serious comorbidity at the time of transplantation, LDLT can be performed safely in all age groups.     

Biliary complications and management in pediatric living donor liver transplantation for underlying biliary atresia

Background

Biliary complications are a major problem in pediatric liver transplantation. The aim of this study was to evaluate the management and outcomes of biliary complication after pediatric living donor liver transplantation (LDLT).

Methods

From 1994 to 2010, 157 pediatric LDLT due to biliary atresia were performed in our center. Doppler ultrasound was initially performed daily for 2 weeks postoperatively to evaluate biliary and vascular complications. Computed tomography and or magnetic resonance cholangiography were performed when complications were suspected. They were treated using radiological or surgical interventions.

Results

Among the 157 cases, we observed 10 (6.3%) biliary complications, which were divided into three groups: bile leakage (n = 3); biliary stricture without vascular complication (n = 4); and biliary stricture with vascular complication (n = 3). The three cases bile leakages recovered after interventional procedures. The seven biliary strictures underwent percutaneous transhepatic cholangial drainage (PTCD). All cases without vascular complications were completely cured after PTCD or a subsequent surgical re-anastomosis. In the vascular complication group, early recorrection of the HA occlusion with successful PTCD treatment were performed in two cases, but one other case with diffuse ischemic biliary destruction had a poor result.

Conclusion

Successful interventional radiographic approaches are effective for anastomotic biliary complications but with poor results in diffuse ischemic biliary destruction.     

Duct-to-duct biliary reconstruction for living donor liver transplantation: experience of 92 cases

Bile duct-to duct reconstruction is now performed in living donor liver transplantation (LDLT) for adult patients. To confirm the feasibility, the results after the reconstruction were retrospectively analyzed. The subjects were 92 adult patients who underwent LDLT at the University of Tokyo Hospital. During the observation period (median 546 days), biliary complications were observed in 28 cases (30%). The complications included bile juice leakage in 11, stenosis at the anastomotic site in 9, and tube trouble in 8. Of these, 20 patients required surgical revision. The results suggest that duct-to-duct reconstruction provides satisfactory results, although long-term observation will be necessary.     

活体肝移植治疗儿童胆道闭锁44例

目的 观察活体肝移植治疗儿童胆道闭锁的效果,总结其临床经验.方法 2006年10月至2010年12月间共有44例胆道闭锁患儿接受了活体肝移植,其中男性26例,女性18例,年龄(12.1±9.0)个月,中位数为9个月(6～60个月);44名供者全部为患者直系亲属,年龄(32.7±8.0)岁,中位数为31岁(20～54岁).供、受者ABO血型相容,供肝均为供者肝脏的左外叶.对供、受者术前评估过程、手术方法、术后管理以及预后等临床资料进行总结.结果 术后对全部供者进行系统随访,随访时间(17.5±13.3)个月,无供者发生严重并发症和死亡,所有供者均恢复健康.44例受者中,死亡9例,死因分别为门静脉栓塞3例、肝动脉栓塞1例、胆道并发症2例、切口感染1例、腹腔出血1例及肺部感染1例,其余35例健康存活.术后1年和2年累积存活率分别为81.2%和76.1%,无一例受者接受再次肝移植.术后主要并发症有门静脉血栓、肝动脉血栓、胆汁漏及逆行性胆管炎、肺部感染、切口感染及急性排斥反应等.结论 活体肝移植是治疗儿童胆道闭锁的有效方法,预后良好.完善缜密的术前评估,熟练精细的手术操作以及精心的术后管理是改善受者预后的关键因素.

Abstract：

Objective To observe the outcomes of living donor liver transplantation (LDLT) for children with biliary atresia (BA) and to summarize the clinical experiences. Methods Forty-four BA patients (26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean (SD) and median (range) age at operation was (12.1 ± 9.0) months and 9 (6-60) months,respectively. The 44 donors were lineal relatives to the consorted recipients. Their mean (SD) and median (range) age at operation was (32. 7 ± 8. 0) months and 31 (20～54) years, respectively. All donor graft types were the left lateral segments with compatible ABO blood groups. Clinical data,including pre-operative evaluations, surgical technique, postoperative management and outcomes in all donors and recipients were retrospectively analyzed. Results All donors were followed up for (17. 5 ± 13. 3) months. No donor mortality was encountered, with a minimal morbidity and no long-term sequelae. Nine out of 44 recipients died. Three patients died of portal vein thrombosis (PVT), one of hepatic artery thrombosis (HAT), two of biliary complications, one of surgical site infections, one of abdominal bleeding and one of pulmonary infection. The overall 1-year and 2-year cumulative survival rate in recipients was 81. 2% and 76. 1 %, respectively. No re-transplantation was done. Postoperative complications included PVT, HAT, biliary leakage and refluxing cholangitis, pulmonary infections,surgical site infections and acute rejection. Conclusion LDLT has been the effective treatment for pediatric recipients with BA and provides favorable prognosis. To improve prognosis of recipients, the key points are pre-operative evaluations, surgical technique, and postoperative management     

Progress in living donor liver transplantation

Living donor liver transplantation (LDLT) has the capacity to reduce the current discrepancy between the number of patients on the transplant waiting list and the number of available organ donors. For pediatric patients, LDLT has clearly reduced the number of waiting list deaths, providing compelling evidence for an increase in LDLT programs. This review discusses many of the recent advances in LDLT.     

The effects of Kasai procedure on living donor liver transplantation for children with biliary atresia

ObjectiveTo evaluate the effects of Kasai procedure (hepatic portoenterostomy) on living donor liver transplantation (LDLT) for children with biliary atresia (BA).MethodsFrom January 2006 to January 2014, 150 children with BA were treated with LDLT in China. The children were categorized into pre-Kasai and non-Kasai groups, based on whether they had previously undergone Kasai procedure. Clinical data were retrospectively analyzed, and the difference in postoperative survival was compared between the groups. Preoperative data, including height, weight, serum bilirubin, and pediatric end-stage liver disease score, and perioperative blood loss, operation duration, incidence of postoperative surgical complications including vascular complications, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were compared between the groups.ResultsIn total, 89 and 61 children were categorized in the pre-Kasai and non-Kasai groups, respectively. The 1-, 6-, and 12-month survival was 97.8%, 95.4%, and 95.4% for the Kasai group, and 98.4%, 96.7%, and 96.7% for the non-Kasai group, respectively (P > 0.05). The differences in mean operation duration and mean blood loss, and the incidences of outflow tract obstruction, portal vein stenosis, hepatic artery thrombosis, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were not statistically significant between the groups (P > 0.05).ConclusionKasai procedure could effectively delay the requirement of liver transplantation. In light of previous findings that Kasai procedure could significantly improve the liver transplantation-free survival of children with BA, we suggest that Kasai procedure should be used as a first-line treatment method for this condition.Type of studyTreatment Study.Level of evidenceLevel III.     

Orthotopic liver transplantation in biliary atresia: a single-center experience

Introduction

Biliary atresia (BA) is the leading indication for orthotopic liver transplantation (OLT) among children. However, there are technical difficulties, including the limited dimensions of anatomical structures, hypoplasia and/or thrombosis of the portal vein and previous portoenterostomy procedures.

Objective

The objective of this study was to present our experience of 239 children with BA who underwent OLT between September 1989 and June 2010 compared with OLT performed for other causes.

Methods

We performed a retrospective analysis of patient charts and analysis of complications and survival.

Results

BA was the most common indication for OLT (207/409; 50.6%). The median age of subjects was 26 months (range, 7-192). Their median weight was 11 kg (range, 5-63) with 110 children (53.1%) weighing ≤10 kg. We performed 126 transplantations from cadaveric donors (60.8%) and 81 from living-related donors (LRD) (39.2%). Retransplantation was required for 31 recipients (14.9%), primarily due to hepatic artery thrombosis (HAT; 64.5%). Other complications included the following: portal vein thrombosis (PVT; 13.0%), biliary stenosis and/or fistula (22.2%), bowel perforation (7.0%), and posttransplantation lymphoproliferative disorder (PTLD; 5.3%). Among the cases of OLT for other causes, the median age of recipients was 81 months (range, 11-17 years), which was higher than that for children with BA. Retransplantation was required in 3.5% of these patients (P < .05), mostly due to HAT. The incidences of PVT, bowel perforation, and PTLD were significantly lower (P < .05). There was no significant difference between biliary complications in the 2 groups. The overall survival rates at 1 versus 5 years were 79.7% versus 68.1% for BA, and 81.2% versus 75.7% for other causes, respectively.

Conclusions

Children who undergo OLT for BA are younger than those engrafted for other causes, displaying a higher risk of complications and retransplantations.     

The efficacy of Kasai operation for biliary atresia: a single institutional experience.

To assess the efficacy of Kasai operation for biliary atresia, 60 cases during a 14-year period (1976 to 1989) at this institution were reviewed. Thirty-four (56.7%) underwent operation within 90 days of life, 11 (18.3%) between 90 and 120 days and 15 (25.0%) beyond 120 days. Fifty-six underwent hepaticoportojejunostomy and 4 underwent hepaticoportocholecystostomy. Follow-up was 1 to 14 years (mean, 7.2 years). The postoperative follow-up of these 60 patients was categorized into five groups: group A (16 patients, 26.7%) was characterized by survival greater than 3 years and without jaundice; group B (3 patients, 5.0%) was defined as survival greater than 3 years, but with jaundice; group C (11 patients, 18.3%) was defined by survival but follow-up less than 3 years, (this group was further divided into C1 [7 patients, 11.7%] if anicteric and C2 [4 patients, 6.7%] if icteric); group D (2 patients, 3.3%) was patients who underwent liver transplantation; and group E (28 patients, 46.7%) was the group of patients who died at time of review. Group A and group C1 were interpreted as success after Kasai operation; hence, the potential success rate in this series was 38.3% (23 patients). They were long-term survivers, and most of them performed normal activities appropriate for their ages. We conclude that approximately one third of patients with biliary atresia would benefit from or be cured by this operation, which should be the first attempt in the treatment of this disease entity.     

Duct-to-duct biliary reconstruction in adult-to-adult living donor liver transplantation

In living donor liver transplantation (LDLT), Roux-en-Y hepaticojejunostomy has been a standard technique for biliary reconstruction because the majority had been pediatric patients with biliary atresia. Adult-to-adult LDLT using the right lobe graft has recently been developed and we introduced duct-to-duct biliary reconstruction (hepaticohepaticostomy) in such cases. The aim of this study is to evaluate the feasibility of this procedure in adult-to-adult LDLT. From August 2000 to October 2001, five patients underwent adult-to-adult LDLT using the right hepatic lobe and were followed for more than 6 months at our institution. All patients underwent duct-to-duct biliary reconstruction (single hepaticohepaticostomy in one, multiple in four). For the grafts with multiple bile ducts, various techniques were used for reconstruction. In all patients, oral intake could be started early after the operation, and biliary leakage was not encountered. One patient developed two episodes of acute cholangitis who later developed biliary anastomosis stricture which required percutaneous dilatation at 11 months postop. However, otherwise, there were no infectious complications postoperatively. We conclude duct-to-duct biliary reconstruction is feasible and effective in adult-to-adult LDLT.     

成人活体肝移植的早期经验

目的 总结成人活体肝移植的早期经验,提高活体肝移植效果.方法 回顾性分析解放军总医院肝移植中心2006年6月至2008年2月31例成人活体肝移植资料.结果 31例中慢性乙型肝炎肝硬化失代偿期8例,急性肝功能衰竭7例,肝细胞肝癌12例,肝脏紫癜病2例,肝门部胆管癌1例和Wilson 病1例.含肝中静脉(middle hepatic vein,MHV)的右半肝移植25例,不含MHV的右半肝移植3例,双供肝为含MHV的右半肝+左外叶和含MHV的右半肝+左半肝各1例,左半肝辅助性原位肝移植1例.33例供者中5例发生并发症6例次.9例受者发生并发症11例次,其中胆道并发症4例,血管并发症3例,感染性并发症3例,切口延迟愈合1例.2例肺部感染和1例全身多处曲霉菌感染者死亡.结论 活体肝移植已成为拓展供肝来源的有效途径,合理的供、受者评估,手术方式和术后处理是关键.     

T-tube drainage for biliary stenosis after living donor liver transplantation

The optimal strategies for bile duct stenosis after living donor liver transplantation with duct-to-duct biliary reconstruction remain unclear. Patients who underwent liver transplantation with duct-to-duct bile duct reconstruction (n=182) and were complicated with biliary stenosis (n=34) were analyzed. Treatment of biliary stenosis was attempted using an endoscopic approach or transhepatic biliary drainage. When this failed, the T-tube drainage technique was indicated. T-tube placement was performed in 14 patients. Intraoperative ultrasonography was performed to identify the bile duct. The common bile duct was cut open, a Kelly clamp was inserted and the stenotic portion was dilated, and a T-tube was inserted. The patients were scheduled to have the tube removed 1 year after insertion. Complications following T-tube placement or T-tube removal were negligible. The present technique can be an effective therapeutic option when endoscopic treatment is unsuccessful.     

Case of living donor liver transplantation in a patient with biliary atresia combined with situs inversus

Until recently, situs inversus was considered to be an absolute contraindication for liver transplantation. However, recent reports have suggested that situs inversus should not be considered a contraindication. This study presents a successful living donor liver transplantation performed in a 4-month-old male infant with biliary atresia and situs inversus. The surgical findings revealed abdominal situs inversus with polysplenia and an absent retrohepatic inferior vena cava and intestinal malrotation.     

活体肝移植的胆道重建与胆道并发症

To systematically summarize the current status of surgical techniques in biliary reconstruction and biliary complications following living donor liver transplantation and analyze the biliary reconstruction techniques and difficulties in the prevention of biliary complications.The refinements of surgical techniques and successful prevention and therapeutic strategies for reducing biliary complications after living donor liver transplantation are discussed.     

Clinical implications of serum autotoxin in regular follow up after pediatric living donor liver transplantation for biliary atresia

Background: Pediatric patients sometimes develop graft fibrosis after living donor liver transplant (LDLT). Autotaxin is a recently developed serum marker for hepatic fibrosis. We studied the relationship between serum autotaxin levels and histological findings in patients after LDLT for biliary atresia (BA). Methods: Information on patients aged <19 years who received LDLT for BA and were followed for at least 1 year after LDLT was gathered. Autotaxin levels were compared with pathological fibrosis scores. Results: The study included 52 patients, of whom 4 patients had no fibrosis (F0), 36 patients had F1 fibrosis, and 12 patients had F2. The median serum autotaxin level was 0.89 mg/L. In patients with portal vein (PV) complications such as stenosis or thrombosis (n = 7), the mean autotoxin level was 1.25 mg/L compared with 0.95 mg/L in patients without PV complications (p = 0.004). Among patients without PV complications, the mean autotaxin level was 0.90, 0.88, and 1.18 mg/L in F0, F1, and F2 fibrosis, respectively. The mean autotaxin was higher in F2 fibrosis than in F0 or F1 fibrosis (p<0.05). Autotoxin had a high area under the curve (0.86) with the cut-off level of 0.897 mg/L. Conclusion: Serum autotaxin is a novel marker for liver fibrosis in patients after pediatric LDLT for BA.Type of study: Study of Diagnostic Test.Level of evidence: Level II.