Liver transplantation in children with biliary atresia and vascular anomalies

Eight of 29 infants and children undergoing orthotopic liver transplantation for extrahepatic biliary atresia had associated major vascular anomalies. A distinctive and highly unusual vascular malformation consisting of absent inferior vena cava, anomalous origin of the hepatic artery, and preduodenal portal vein was encountered in three of these children. Although at times technically difficult, single anomalies of hepatic vasculature were satisfactorily handled. In contrast, transplantation attempts were lethal in all three infants having the complex vascular malformation. The suggestion is made that this specific subgroup of patients with biliary atresia be identified in advance and that, at the moment, children with this composite anomaly are highly questionable candidates for liver transplantation.     

Liver transplantation in children with biliary atresia and polysplenia syndrome

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.     

Orthotopic transplantation of the liver in children with biliary atresia and polysplenia syndrome: report of two cases

Biliary atresia is the most common indication for liver transplantation in infants and children, despite the advent of the Kasai operation. Coexisting anomalies, which have been noted in up to 27% of patients with biliary atresia, may form an association known as the "polysplenia syndrome," which includes (1) polysplenia, (2) midgut malrotation, (3) preduodenal portal vein, (4) absent prerenal inferior vena cava with azygos continuation, (5) situs inversus, (6) symmetric liver, (7) hepatic arterial anomalies, and (8) bilobed right lung with hyparterial bronchus. Two of 31 patients undergoing orthotopic liver transplantation for biliary atresia following failed portoenterostomy over the past 11 years manifested the polysplenia syndrome with absent prerenal inferior vena cava. The clinical course of these patients, constellation of anomalies, and technical adjustments required to perform liver transplantation are described. We do not believe that these complex congenital anomalies preclude liver transplantation.     

胆道闭锁患儿肝移植术后门静脉狭窄的血管腔内治疗

目的探讨血管腔内治疗在胆道闭锁患儿肝移植术后门静脉狭窄(PVS)治疗中的应用价值。方法收集因原发病为胆道闭锁接受肝移植、术后后发生PVS的患儿14例,均经门静脉造影证实,并接受经皮血管成形术和(或)经皮血管内支架成形术治疗。分析14例患儿血管腔内介入治疗的效果。结果 14例患儿共进行23次血管内腔内介入治疗,技术成功率82.61%(19/23)。10例患儿经1~2次球囊扩张治疗后治愈,4例患儿球囊扩张治疗后,行血管腔内支架成形术,支架植入后未发生狭窄。14例患儿均未出现治疗相关并发症。结论胆道闭锁患儿肝移植术后PVS的血管腔内介入治疗安全、有效。     

Gallbladder agenesis: An accidental finding during laparotomy for hepatic tumor

BackgroundAnomalies of the biliary system are frequently encountered. Agenesis of the gall bladder is a rare. The exact incidence is not known as most cases are asymptomatic and diagnosed incidentally during surgeries for unrelated conditions or at autopsy. This anomaly may occur alone or in association with other anomalies such as other biliary anomalies, portal vein anomalies and other vascular anomalies, or in some cases hepatic, gastrointestinal or anomalies in other body systems.Case presentationAn elderly man diagnoses with segment VI liver tumor and planned for surgical resection. During surgery we accidentally discovered gall bladder agenesis with slightly dilated common bile duct. The tumor resected successfully and the final diagnosis was well differentiated with hepatocellular carcinoma.ConclusionCareful search for the gall bladder must be done before diagnosing agenesis of the gall bladder. No conversion is required provided adequate visualization and search for the gall bladder is done during laparoscopy. Postoperative MCRP will define the biliary anatomy and to help the surgeons to have postoperative accurate decision.     

Anomalous gallbladder septum—A case report

IntroductionAnomalies and diseases of the biliary system are common with over 20 million cases of biliary disease and an estimated 1.8 million ambulatory visits each year in the United States. Congenital anomalies of the gallbladder are rare and include complete and partial duplications, floating gallbladders, and agenesis. Septations have also been reported in the literature. Case reports have typically described these as longitudinal. Transverse septa, when reported, are associated with inflammation or cholelithiasis. Variations in the cystic duct and vasculature in the portal triad have also been well described.Presentation of caseDuring the dissection of a 91-year-old female cadaver, an enlarged gallbladder with a partial transverse septum was observed. The gallbladder contained approximately 350 ml of bile, no stones, and had a partial transverse septum near the infundibulum. The hepatic, cystic, and common bile ducts were enlarged, but of normal configuration. Vascular anomalies were also present, including an accessory left hepatic artery from the left gastric artery and an anomalous origin of the right hepatic artery from the superior mesenteric artery.DiscussionThis is the first described case of a partial transverse septum with a markedly enlarged gallbladder, dilated duct system, and vascular anomalies in a patient with no evidence of gallstones, inflammation, or scarring.ConclusionWith the prevalence of biliary disease and frequent subsequent surgery it is essential to appreciate all anatomical variations to avoid iatrogenic injuries to these structures during surgery.     

Biliary atresia and the polysplenia syndrome

There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989, 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), aberrant hepatic artery (4), or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference). Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai's operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Acturial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation.     

First orthotopic liver transplantation in patient with biliary atresia and situs inversus in spain

Biliary atresia is the most common indication for liver transplantation in the pediatric age group. The Kasai portoenterostomy has become established as the primary treatment for biliary atresia. If portoenterostomy fails, death before 2 years of age is likely without liver transplantation. The most common multiple malformation syndrome associated with biliary atresia is polysplenia syndrome, which forms a constellation of defects of body symmetry, splenic development and vascular anomalies, including situs inversus, polysplenia and others. The situs inversus was formerly considered an absolute contraindication for liver transplantation. Recently however, several case reports have been published suggesting that neither situs inversus nor this particular subset of vascular abnormalities should be considered contraindications to liver transplantation. We present one case of liver transplantation performed in patient with biliary atresia, situs inversus and polysplenia. This is the first report described in Spain for a liver transplant in a child with biliary atresia plus situs inversus.     

HIF-1alpha-pathway activation in cholangiocytes of patients with biliary atresia: An immunohistochemical/molecular exploratory study

BackgroundBiliary atresia is a neonatal disease characterized by choledochal obstruction and progressive cholangiopathy requiring liver transplantation in most patients. Hypoxia-ischemia affecting the biliary epithelium may lead to biliary obstruction. We hypothesized that ischemic cholangiopathy involving disruption of the peribiliary vascular plexus could act as a triggering event in biliary atresia pathogenesis.MethodsLiver and porta hepatis paraffin-embedded samples of patients with biliary atresia or intrahepatic neonatal cholestasis (controls) were immunohistochemically evaluated for HIF-1alpha-nuclear signals. Frozen histological samples were analyzed for gene expression in molecular profiles associated with hypoxia-ischemia. Prospective clinical-laboratory and histopathological data of biliary atresia patients and controls were reviewed.ResultsImmunohistochemical HIF-1alpha signals localized to cholangiocytes were detected exclusively in liver specimens from biliary atresia patients. In 37.5% of liver specimens, HIF-1alpha signals were observed in biliary structures involving progenitor cell niches and peribiliary vascular plexus. HIF-1alpha signals were also detected in biliary remnants of 81.8% of porta hepatis specimens. Increased gene expression of molecules linked to REDOX status, biliary proliferation, and angiogenesis was identified in biliary atresia liver specimens. In addition, there was a trend towards decreased GSR expression levels in the HIF-1alpha-positive group compared to the HIF-1alpha-negative group.ConclusionActivation of the HIF-1alpha pathway may be associated with the pathogenesis of biliary atresia, and additional studies are necessary to confirm the significance of this finding. Ischemic cholangiopathy and REDOX status disturbance are putative explanations for HIF-1alpha activation. These findings may give rise to novel lines of clinical and therapeutic investigation in the BA field.     

Evaluation of Early and Late Effects of Surgical Treatment of Early Hepatic Artery Thrombosis After Liver Transplantation

BackgroundHepatic artery thrombosis (HAT) is the most severe vascular complication after liver transplantation and one of the major causes of early graft loss and mortality after transplantation. The number of retransplantations and recipient deaths can be decreased with an urgent thrombectomy of the hepatic artery.The aim of the study was to analyze the early and long-term outcomes of the surgical revascularization of early hepatic artery thrombosis after liver transplantation.MethodsFour hundred eleven orthotopic liver transplantations in 380 patients were performed at our center between 2005 and 2020. A Doppler evaluation of the graft vessels patency was performed daily for the first 5 days after transplantation in all recipients. After angio–computed tomography confirmation, most of the cases of HAT qualified for surgical revascularization.ResultsEarly HAT was diagnosed in 20 cases (4.9%), occurring most frequently between the first and third day after transplantation. Sixteen patients underwent revascularization surgery. Among them, in the early post-transplantation period, 4 died and 2 more had retransplantation. Of the remaining 10 recipients, 2 had no biliary complications, 1 had bile leakage, and 7 had common bile duct stenosis, all treated endoscopically. Among 4 nonoperated patients, 1 died and the other 2 had retransplantation in the early post-transplantation period; the last of these 4 recipients had bile duct stenosis.ConclusionsThe urgent surgical revascularization in liver recipients with early HAT allows the avoidance of early retransplantation. However, these patients require intensified surveillance owing to the high risk of biliary complications that may affect shortened graft and patient survival.     

The Factors Affecting Mortality After Pediatric Liver Transplantation and Long-Term Survival Outcomes: A Single Center Experience

BackgroundLiver transplantation (LT) is a treatment modality in the pediatric population for several diseases like biliary atresia, metabolic liver disease, hepatoblastoma, and so on. According to the Organ Procurement and Transplantation Network, 5-year survival was reported as 85.4% to 93.5% by age after pediatric liver transplantation (PLT). This study aimed to evaluate our single-center experience of PLT by analyzing long-term results, comparing the outcomes with the literature, and identifying predictors of patient survival.MethodsThe data of 40 patients who underwent LT at <18 years of age between June 2015 and June 2021 were studied retrospectively. Recipient characteristics such as age, sex, etiology of liver disease follow-up time, postoperative vascular and biliary complications, and donor characteristics were evaluated.ResultsThere were 20 (50%) girls and 20 (50%) boys, and the median age was 42 (IQR = 9-117) months. The most common indications of LT were biliary disorders (45%). A whole liver graft was used in 7 (17%), a right lobe graft in 9 (23%), a left lobe graft in 4 (10%), and a left lateral lobe graft in 20 (50%) of the recipients. The 1-, 3-, 5-, and 7-year survival rates were 85%, 82.1%, 82.1%, and 82.1%, respectively. The multivariate survival analysis revealed that the pediatric end-stage liver disease score, hepatic artery thrombosis, and portal vein thrombosis are associated with overall mortality.ConclusionIn conclusion, our long-term survival is similar to the literature, with satisfactory results. However, reducing the vascular complication rates can provide superior results on PLT.     

Successful hepatic transplantation in congenital absence of recipient portal vein

The first case of hepatic transplantation in a patient with congenital absence of the portal vein (CAPV) is reported. A 10-year-old girl with biliary atresia and CAPV underwent successful hepatic transplantation and has normal liver function 9 months after transplantation. This case is only the seventh reported case of CAPV. Patients with CAPV commonly have additional liver anomalies, cardiac and inferior vena cava anomalies, and polysplenia. Surprisingly, hepatic encephalopathy is not a prominent feature in patients with CAPV despite systemic drainage of mesenteric venous blood. CAPV should not be considered a contraindication to hepatic transplantation.     

Feasibility of morphologic assessment of vascular and biliary anatomy in pediatric liver transplantation: all-in-one protocol with breath-hold magnetic resonance

Background/Purpose

Noninvasive imaging for children with liver transplantation for possible sites of vascular and biliary complication remains a challenge. The aim of this study was to investigate the feasibility of magnetic resonance (MR) imaging as a comprehensive noninvasive test for the above purpose.

Methods

Thirteen children (age, 8-16 years) with biliary atresia and who received liver transplantation underwent a comprehensive MR study including MR cholangiography and gadolinium-enhanced MR angiography. Images were interpreted by 3 radiologists for liver parenchymal abnormalities; definition of hepatic arterial and venous, portal venous, and biliary anatomy; and detection of any complications. Findings were correlated with surgical records. Conventional angiography and percutaneous cholangiography were obtained for correlation in 2 patients. Confidence level scores (1-5) for depiction of anatomy were given for source, multiplanar, and 3-dimensional images.

Results

Hepatic artery anastomosis was visualized in 12 patients (92%) and the intrahepatic arteries were demonstrated in 10 (77%). The portal, hepatic venous, and biliary anastomoses were clearly demonstrated in all patients. Stenosis of hepatic artery anastomosis and multiple biliary strictures were detected in 1 patient each and confirmed by conventional imaging. High confidence scores (higher than 4) were obtained for all kinds of MR images.

Conclusions

Comprehensive MR imaging can be used in long-term follow-up of pediatric liver transplant recipients for depiction of hepatic structures and possible complications.     

Resection of metastatic liver cancer in a patient with a left-sided gallbladder and intrahepatic portal vein and bile duct anomalies: A case report

INTRODUCTIONThe presence of left-sided gallbladder is closely associated with multiple combined anomalies of the portal vein, hepatic vein, hepatic artery, and bile duct. This requires special attention for preoperative evaluation for the purpose of preventing postoperative complications.PRESENTATION OF CASEA 70-year-old woman with metastatic liver cancer and intrahepatic portal vein, biliary system and hepatic artery anomalies with left-sided gallbladder is reported. On computed tomography (CT), a solitary low density mass occupied from the right anterior to the posterior segment of the liver. The gallbladder bed was on the left of the hepatic fissure. On drip-infusion-cholangiography (DIC) CT three-dimensional (3D) reconstruction, the left medial bile duct arose from the right umbilical portion after arising from the left lateral bile duct. Following a right hepatectomy and lymph node dissection of the hepatoduodenal ligament, hepaticojejunostomy was conducted separately to the left medial and left lateral bile duct.DISCUSSIONThe left-sided gallbladder accompanies with several anomalies of hepatic vascular and bile duct anomalies in a frequent manner. A safe hepatectomy needs accurate operative plans to ascertain the range of hepatectomy, because it often has the diversity of a combined anomaly.CONCLUSIONPreoperative DIC-CT 3D reconstruction was extremely useful because it provided an important information that could not be obtained with 2D-DIC-CT. 3D imaging has the ability to demonstrate complex anatomical relationships, this devise is a effective new tool for making appropriate preoperative strategy.     

胆道闭锁的外科诊治进展

胆道闭锁是一种病因不明、以胆道梗阻为特点的儿童先天性罕见疾病,主要表现为肝内外胆道系统的进行性炎症以及纤维性梗阻,进而发展为胆汁淤积性肝硬化、肝衰竭。胆道闭锁虽然罕见,但它是婴幼儿胆汁淤积性疾病的最常见原因,如不经手术治疗（如Kasai手术、肝移植手术）,患儿通常可在短期内死亡。Kasai手术可改善胆道引流,但不能改变胆道闭锁患儿的结局,大多数仍会因为胆汁淤积而遭受持续的肝损伤,最终需要进行肝移植。而目前关于肝移植术前是否行Kasai手术尚未达成一致意见。本文就胆道闭锁的诊断、外科治疗现状等进行综述,以期为临床胆道闭锁的诊断和治疗提供参考,改善胆道闭锁患儿的生存。     

彩色多普勒超声在诊断肝移植术后并发症中的价值

目的探讨彩色多普勒超声在肝移植术后并发症的诊断价值。方法回顾分析彩色多普勒超声对95例肝移植患者术后并发症的诊断结果。结果95例中发现肝动脉血栓形成3例（3.15%）,肝动脉狭窄2例（2.10%）,门静脉血栓形成1例（1.05%）,门静脉狭窄1例（1.05%）,下腔静脉狭窄2例（2.10%）,胆道并发症10例（10.52%）,排异反应7例（7.36%）,浆膜腔积液93例（97.89%）,肝周血肿7例（7.36%）,肿瘤复发5例（29.41%）。结论彩色多普勒超声在肝移植术后并发症诊断中具有重要的实用价值。     

Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study

IntroductionKasai hepatoportoenterostomy is the standard of care for children with biliary atresia, but a majority of patients progress to end-stage liver disease and require a salvage liver transplant. Given the high failure rates of the hepatoportoenterostomy operation, some have advocated for primary liver transplantation as a superior treatment approach. The aim of this study was to compare outcomes of pediatric candidates with biliary atresia listed for primary vs. salvage liver transplantation.MethodsThe SRTR/OPTN database was retrospectively reviewed for all children with biliary atresia listed for liver transplant between March 2002 and February 2021. Candidates were categorized as primary liver transplant if they had not undergone previous abdominal surgery prior to listing and salvage liver transplant if they had. Salvage transplants were further categorized as early failure if listed within the first year of life or late failure if listed at an older age.Results3438 children with biliary atresia were listed for transplant during the study period, with 15% of them listed for a primary transplant, 17% for salvage transplant after early failure, and 67% after late failure. Recipients of salvage liver transplant with late failure had lower bilirubin levels and were less critically ill as demonstrated by MELD/PELD scores and hospitalization status. Correspondingly, these recipients had higher waiting list and graft survival, though this did not remain statistically significant after adjustment in multivariable models. There were no differences in waiting list, recipient, or graft survival with primary vs. salvage liver transplant after early failure.ConclusionKasai hepatoportoenterostomy should remain the standard of care in biliary atresia as it may delay need for transplant beyond the first year of life in a subset of recipients and does not jeopardize subsequent transplant outcomes, even with early failure.Levels of evidenceRetrospective cohort study (Level III)     

A case of inflammatory pseudotumor of the liver hilum successfully treated with aggressive hepatectomy

Inflammatory pseudotumor (IPT) of the liver is a rare, benign tumor. When the tumor involves the hepatic hilum, however, the clinical course of the patient becomes problematic because of obstructive jaundice and portal hypertension. Complete removal of the tumor sometimes is difficult when the hepatic hilum is extensively involved, and liver transplantation becomes necessary for such an unresectable tumor. This report concerns a 7-year-old boy who presented with obstructive jaundice and portal hypertension owing to an inflammatory pseudotumor of the hepatic hilum and was treated successfully with aggressive hepatectomy, vascular reconstructions of both the portal vein and the hepatic artery, and biliary reconstruction. The patient was discharged after 39 days of hospitalization. Eight months later, portal vein obstruction was detected and treated successfully with percutaneous transhepatic balloon dilatation of the obstructed site. The liver has continued to function well for 11 months after the tumor resection.     

Multislice computed tomography angiography in pediatric liver transplantation

BACKGROUND: Preoperative delineation of any vascular anomalies offers planning for possible alteration of surgical procedures, especially in pediatric recipients undergoing living-related liver transplantation. PURPOSE: We assess the efficacy of three-dimensional (3D) multislice computed tomography (CT) angiography in the hope of replacing conventional angiography as the pretransplant evaluation of the hepatic vascular system for potential recipients of liver transplantation. METHODS: 3D CT angiography was performed in 38 children with biliary atresia. Conventional angiography was also performed in the first 15 patients. Twelve patients underwent living-related liver transplantation. The findings on 3D CT angiography were compared with conventional angiography and operative findings. RESULTS: 3D CT angiography was successfully performed in 37 pediatric patients. All findings of 3D CT angiography on hepatic artery, portal vein, and inferior vena cava paralleled those of catheter angiography and operative findings. Four patients were unsuitable to receive living grafts because of pathologic insults of the hepatic artery (one patient) and the portal vein (three patients). Three patients were advised to undergo a venous graft for portal anastomoses. Eight patients demonstrated portosystemic shunts that may require closure. CONCLUSION: 3D CT angiography proves to be a better tool in the demonstration of the vascular system and identification of pathologic insults in pediatric patients. It is superior to conventional angiography because it is less invasive, more convenient, and more efficient in providing thorough preoperative information that would have a major impact on patient selection and surgical planning.     

Liver Transplantation in Children Weighing Less Than 10 kg: Chilean Experience

BackgroundOrthotopic liver transplantation is the treatment of choice for most terminal liver diseases in children. In small children (≤10 kg), this procedure is challenging and has special considerations. The aim of this study is to describe the experience of a Chilean liver transplantation program in this subgroup of patients.MethodsThe liver transplant database of Hospital Luis Calvo Mackenna and Clinica Las Condes was reviewed. All children less than 10 kg undergoing liver transplantation between January 1994 and July 2011 were included. Patient and graft outcomes and main complications were analyzed.ResultsWe have performed 230 pediatric liver transplantations, 49 of them in 41 patients weighing less than 10 kg. The first indication for transplantation was biliary atresia in 25 patients (61%). A living related donor was used in 23 cases (51%). Actuarial survival was 75.7% at 1 year and 67.1% at 5 years. The main cause of death was infection, and the leading cause of graft loss was vascular complication.DiscussionOur transplant program includes 2 centers that perform more than 90% of pediatric liver transplantations in Chile, including public health pediatric patients from all around the country. Patients weighing less than 10 kg represent the most challenging group in pediatric liver transplantation due to higher rates of vascular and biliary complications and postoperative infections.