An alternative method of arterial reconstruction in pediatric living donor liver transplantation with the recipient right gastroepiploic artery

The classical method for arterial reconstruction in pediatric living donor liver transplantation using left lateral segment consists of end-to-end anastomosis between the donor left hepatic artery and the recipient right hepatic artery. In the present case, an intra-operative hepatic artery thrombosis occurred because of extensive intima wall dissection of the recipient hepatic artery. The patient was a 6-yr-old boy with fulminant hepatic failure, who underwent living donor partial liver transplantation with left lateral segment from his father. The graft was irrigated by a left hepatic artery and an accessory left hepatic artery from gastric artery, both arteries with diameter of <2 mm. These arteries were anastomosed to the recipient right and left hepatic arteries, respectively. Before performing the bile duct reconstruction it was noted that these anastomoses were occluded by clots of blood. An extensive subintimal dissection of the recipient hepatic artery was the cause of this problem. The creation of a new anastomosis by using a more proximal part of this artery without subintimal dissection was judged impossible. Then, the right gastroepiploic artery was mobilized and an anastomosis was performed with the donor left hepatic artery in an end-to-end fashion. Arterial blood flow to the graft was established successfully and the patient's postoperative recovery was excellent. Fifteen days after the transplantation, an angiotomography demonstrated a good hepatic arterial blood flow. The patient is now alive and well, 4 months after the transplantation. In conclusion, the method of hepatic graft arterialization described here is an important option for patients who undergo living donor or split liver transplantation.     

Arterial anastomosis in a pediatric patient receiving a right extended split liver transplant: a case report

Abstract:  We report a case of a pediatric patient who received a right-extended liver transplant. The size of the recipient hepatic artery did not match with the donor right hepatic arterial stump. Moreover, recipient arterial anatomy made the direct anastomosis difficult or at increased risk for complications. The recipient's splenic artery was then mobilized, divided and anastomosed to the donor's right hepatic artery. The spleen was preserved and revascularization through collaterals is demonstrated by Angio CT Scan. Doppler US of the transplanted liver demonstrated good flow through the liver and the patient was discharged with perfect liver function. Splenic artery is perfectly suited for hepatic artery anastomosis. The use of splenic artery is favored in particular situations as in the case of a pediatric recipient receiving a right-extended liver graft with small caliber artery.     

Nonsystemic treatment of metastatic tumors of the liver--a review.

Hepatic metastasis is usually quite resistant to conventional systemic chemotherapy. Nonsystemic treatment of metastatic tumors of the liver include surgical resection; infusion of chemotherapeutic agent(s), either via hepatic artery or portal vein; ligation of hepatic artery; radiotherapy; and other more investigative approaches (isolation perfusion, cryosurgery, liver transplantation, etc). The relative applicability, results, and limitations of each of these therapeutic modalities are reviewed.     

Outcome of pediatric liver transplant in grafts with multiple arteries

Abstract:  Young age, small diameter of the hepatic artery, presence of multiple arteries, and the selected technique are risk factors for hepatic arterial complications. We report the outcomes of pediatric liver transplant (LT) patients who had received a liver graft with multiple arteries. Since April 2001, 89 pediatric LTs have been performed at our center. Twenty-six liver grafts (29.2%) that had multiple hepatic arteries were analyzed retrospectively. Twenty-five grafts had 2, and 1 graft had 3, hepatic arteries. In 17 grafts with double arteries, and in 1 graft with 3 arteries, the adjacent edges of the hepatic arteries were sutured together at the back table to create a single opening, and the recipient's common hepatic artery was then anastomosed to that orifice. In 8 grafts with double arteries, 2 separate anastomoses were performed between the graft hepatic arteries and the recipient's hepatic artery branches. The mean age of the recipients was 7.9 ± 5.4 years (range, 0.6–17 years). During the early postoperative period, hepatic arterial thromboses occurred in 3 recipients. Two of these 3 recipients were treated with transcatheter arterial thrombolysis using streptokinase or recombinant plasminogen and intraluminal stent placement, and the remaining recipient was treated with a re-anastomosis using a polytetrafluoroethylene graft. Three recipients died during follow-up, which ranged from 1 to 71 months. In conclusion, multiple graft arteries did not increase the incidence of hepatic arterial complications in our series.     

Diagnosis of liver cirrhosis in children based on colour Doppler ultrasonography with histopathological correlation

Background. Changes in hepatic architecture in cirrhosis and chronic active hepatitis affect liver vascular haemodynamics. Objective. To determine the criteria for the diagnosis of liver cirrhosis using Doppler US. Materials and methods. Twenty-two children with liver disease of unknown histology were prospectively examined and compared with eight normal children. Doppler US of portal vein velocity, arterio-portal velocity ratio, loss of reverse flow component in the hepatic vein and hepatic artery visualisation were examined prior to liver biopsy. Doppler results were compared with histological activity indices. Twelve patients had cirrhosis and ten had chronic active hepatitis. Results. The most sensitive method (83 %) for the assessment of cirrhosis was portal vein velocity less than 20 cm/sec. Arterio-portal velocity ratio (greater than 3) and hepatic artery visualisation were less sensitive (75 % and 33 % respectively) but specificity was 100 % for all three methods. When these three methods were evaluated together, sensitivity increased to 91 % and accuracy to 96 %. Loss of reverse flow component was less specific (77 %) but was sensitive (75 %). Conclusions. Portal vein velocity, arterio-portal vein ratio and hepatic artery visualisation together were reliable in diagnosis of cirrhosis in the paediatric age group. Received: 11 August 1997 Accepted: 17 June 1998     

Recurrence of hepatic artery thrombosis following acute tacrolimus overdose in pediatric liver transplant recipient

Acute overdose of tacrolimus appears to cause no or minimal adverse clinical consequences. We encountered a pediatric case who underwent liver transplantation associated with hepatic artery thrombosis (HAT), which recurred following acute tacrolimus overdose. A 10-month-old girl underwent living-related liver transplantation because of biliary atresia. To reconstruct the hepatic artery, the right gastroepiploic artery of the donor was interposed between the right hepatic artery of the recipient (2.5 mm in diameter) and the left hepatic graft artery (1 mm in diameter) under microscopy. On postoperative day 4, Doppler ultrasonography showed a remarkable reduction in hepatic arterial flow, which was consistent with HAT. The patient underwent immediate hepatic arteriography and balloon angioplasty. The stenotic sites were dilated by the procedure. Tacrolimus was infused intravenously after transplantation and the infusion rate was adjusted to achieve a target concentration of 18-22 ng/mL, which remained stable until the morning of day 6. An unexpectedly high blood concentration of tacrolimus (57.4 ng/mL) was detected at 6:00 PM on day 6, and tacrolimus was discontinued at 9:00 PM; however, the tacrolimus level reached 119.5 ng/mL at 0:00 h on day 7. While the concentration decreased to 55.2 ng/mL on the morning of day 7, the hepatic arterial flow could not be observed by Doppler ultrasonography. Emergent hepatic arteriography showed stenosis of the artery at the proximal site of the anastomosis. Balloon angioplasty was again performed and the stenotic site was successfully dilated. High level of tacrolimus exposure to the hepatic artery with injured endothelium by preceding angioplasty may have been related to the recurrence of HAT in the present case.     

Evaluation of Neoadjuvant Chemotherapy Effects on Liver Parenchyma in Resected Pediatric Malignancies

Neoadjuvant chemotherapy for colorectal liver metastases in adults is responsible for chemotherapy-associated liver injury (CALI), characterized by steatosis, steatohepatitis, and sinusoidal obstruction syndrome. These alterations cause delayed operation to reduce the risk of hemorrhage, portal hypertension, and hepatic failure. Children with hepatic malignancies usually receive neoadjuvant chemotherapy prior to surgery. The aim of this study was to evaluate retrospectively whether the CALI occurs in this pediatric population. This study evaluated patients referred since 1996 for hepatic malignancies who received hepatectomy after chemotherapy. Liver resection material was reviewed, in order to investigate the presence of morphological changes compatible with the CALI in the peritumoral hepatic tissue. Twelve patients were recruited. All patients satisfied the inclusion criteria except one who did not receive neoadjuvant chemotherapy. Eleven children underwent surgery 1 month after the last chemotherapy cycle. All are alive disease-free. Histological examination of specimen revealed only mild changes such as diffuse swelling of hepatocytes and focal, mild portal inflammation. Severe hepatic changes such as steatosis, necrosis, or fibrosis were not identified. CALI-related morphological changes were not found in our patients. The absence of the CALI could be attributed to the younger age of patients (possible different response to stress) and/or to the different chemotherapy schedules compared to those in use for adults patients.     

History of the infantile hepatic hemangioma: From imaging to generating a differential diagnosis

We aim to provide an up-to-date summary of infantile hepatic hemangioma (IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver. Eligible peer-reviewed articles on hepatic infantile hemangiomas, published between 2000 and 2015, were reviewed for this study. IHH is the most common hepatic vascular tumor in children. Once a liver mass is identified in an infant, the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma, hepatoblastoma, metastatic neuroblastoma, so careful physical examination, imaging studies, and, if indicated, tumor markers and biopsy, are of pivotal importance to ascertain the correct diagnosis. Despite the benign nature of IHHs, some of these lesions may demand medical and/or surgical intervention, especially for multiple and diffuse IHH. Complications can include hepatomegaly, hypothyroidism and cardiac failure. Therefore, a close follow-up is required until complete involution of the lesions. We propose an algorithm to guide the physicians towards the proper management of hepatic lesions.     

CT三维重建及肝脏体积测定在小儿肝脏肿瘤手术中的应用

目的探讨螺旋CT三维成像及肝脏体积测定在小儿巨大肝脏肿瘤和位于肝门部位肝脏肿瘤手术治疗中的价值。方法2002年1月至2009年1月本院诊治巨大和位于肝门部位的小儿肝脏肿瘤25例,均使用螺旋CT增强扫描,并在CT工作站行肝动脉、门静脉及肝静脉的三维影像重建,测量全肝体积,预测切除肝脏体积,并计算残余肝脏体积及残余肝脏体积与标准化肝脏体积之比。结果25例经CT三维重建后均获得清晰的肿瘤瘤体及与血管毗邻关系的图像。其中手术患儿21例,未手术患儿4例。21例手术患儿中,行右半肝切除6例,左半肝切除5例,右三叶切除5例,左二三叶切除1例,肝中叶切除4例,手术患儿术后平均残余肝脏体积为182．55cm^3,最小残余肝脏体积为62．26m^3,平均残余肝脏体积与标准化肝脏体积之比（平均残余肝脏体积比）为44．69％,最小残余肝脏体积比为20．16％,所有患儿同手术期均未发生严重肝功能衰竭。结论通过CT三维重建的画面,可以旋转地动态观察肝脏血管的走行、位置及与肿瘤的关系,并可计算出术后残余肝脏体积及残余肝脏体积与标准化肝脏体积之比,为判断手术的可行性、手术方案的选择与制定及评估术后残余肝脏功能提供了很大的帮助。     

Hyperbaric oxygen therapy for hepatic artery thrombosis following liver transplantation: current concepts

This article presents the case of an infant who underwent an orthotopic liver transplant and then developed hepatic artery thrombosis that was detected on routine post-operative right upper quadrant ultrasound. Alteplase (TPA) failed to open the artery, so the child received systemic heparin and hyperbaric oxygen (HBO) therapy. After six HBO treatments, the hepatic artery had recanalized and his liver function tests had returned to normal or near normal. There were no complications to the HBO therapy, and 1 yr after the transplant, the child's liver is functioning well. The present study discusses the beneficial effects of HBO therapy and the proposed mechanisms for its favorable results. In our patient, systemic heparin and HBO therapy prevented liver failure and need for retransplantation.     

Hepatic artery embolisation; successful treatment of multinodular haemangiomatosis of the liver

A patient with multinodular haemangiomatosis of the liver, rapidly deteriorating in the first weeks of life due to severe progressive congestive heart failure, was successfully treated by hepatic artery catheter embolisation at the age of 6 weeks.Abbreviations MHL multinodular haemangiomatosis of the liver - CHF congestive heart failure Corresponding author     

Biliary strictures and hepatic artery flow abnormalities in split liver transplants

O'Loughlin EV, Stormon MO, Shun A, Verran D, Jermyn V, Wong C, Lord D. Biliary strictures and hepatic artery flow abnormalities in split liver transplants.
Pediatr Transplantation 2010: 14: 121–125. © 2009 John Wiley & Sons A/S.
Abstract:  The aim of this study was to examine the role of HA flow abnormalities in the development of biliary strictures following split liver transplants. Data was obtained from a prospective data base of all patients undergoing split liver transplants from 2000–2008 with a follow up time of at least six months. Forty-six transplants were performed in 44 patients. Fourteen of 46 developed strictures of whom four were intrahepatic and 10 anastomotic. Nine of 14 with strictures had either hepatic artery thromobosis (HAT, four) or abnormalities of HA flow identified by routine Doppler ultrasound (5) compared with two of 32 without strictures (p < 0.02, (one temporary loss of flow and one HA aneurysm). There were no differences between the stricture and non stricture group with regard to age or weight at transplant, donor age, cold and warm ischemia times or intraoperative portal vein flow though there was a significant decrease in intraoperative HA flow in the stricture group. In conclusion, both HAT and hepatic artery flow abnormalities are associated with biliary strictures in the majority of split liver transplants. However, unrecognised abnormalities in HA flow and or other factors are likely to contribute.     

Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases

Abstract:  The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.     

Interventional radiology for hepatic artery complications soon after living donor liver transplantation in a neonate

Early hepatic artery complications after liver transplantation in children, having undergone LDLT, can directly affect graft and recipient outcomes, making early diagnosis and treatment essential. In the past, laparotomy (thrombectomy or reanastomosis) was generally employed to treat early hepatic artery complications. Recently, favorable outcomes of IR have been reported. In children, however, the number of such reports is small. To the best of our knowledge, there is no published report on IR applied to neonates with early hepatic artery complications. We recently succeeded in safely using IR for a neonate with early hepatic artery complications after LDLT and obtained a favorable outcome. This case is presented herein.     

胆总管囊肿根治术后胆道梗阻的原因分析和治疗策略

目的 本研究旨在分析胆总管囊肿根治术后胆道梗阻的原因, 提出相应的治疗策略.方法 回顾2006年1月至2016年1月间47例在外院接受胆总管囊肿根治术后胆道梗阻,转入我院成功行腹腔镜二次手术的患儿,对其病因进行观察,分析及治疗.结果 出现胆道梗阻症状,即反复胆管炎或持续肝功能异常距离第一次手术的时间为术后2周~10.8年(平均1.7年).在外院接受抗炎保肝利胆PTD治疗时间2周~10年(平均2.7年),未见明显好转.接受二次手术的间隔时间为1个月~11年(平均4.5年).病理显示57.4% (27/47)患儿存在I~IV级肝纤维化.本研究发现术后胆道梗阻的原因如下:①异位肝右动脉前置压迫肝管空肠吻合口近端导致梗阻占27.6% (13/47).术中将异位肝右动脉重置于肝管空肠吻合口后方解除梗阻;②初次手术未解决伴发的单一或多处肝管狭窄占29.8% (14/47).二次手术中行肝管扩大成型+肝管空肠再吻合;③吻合口狭窄占42.6% (20/47).二次手术中行肝管空肠再吻合.中位随访时间为48个月(1~120个月),无1例发生胆道再梗阻或胆管炎.肝功能于二次术后1个月~2年恢复正常.结论 异位前置肝右动脉压迫、合并单一或多处肝管狭窄、吻合技术是胆总管囊肿术后胆道梗阻的原因.应尽早外科手术解除梗阻避免肝功能损伤及肝硬化.     

Minimally invasive endovascular and biliary treatments of children with acute hepatic artery thrombosis following liver transplantation

Hepatic artery thrombosis is a major problem after pediatric liver transplantation. Ischemia caused by hepatic artery thrombosis results in severe biliary and parenchymal damage and is associated with high rates of graft loss and mortality. We present a case-based pictorial essay to illustrate the role of minimally invasive treatment in the prompt management of acute hepatic artery thrombosis, and the associated biliary complications.     

Microvascular hepatic artery reconstruction in pediatric abdominal transplantation: pearls and pitfalls

The "back-wall first" hepatic artery microanastomosis in pediatric liver transplantation saves lives and reduces morbidity. We describe our technique of hepatic artery reconstruction and offer a systematic and multi-team collaborative approach that optimizes surgical outcomes.     

Undifferentiated embryonal sarcoma of the liver in a child complicated by haemorrhage

Undifferentiated embryonal sarcoma (UES) of the liver is a rare malignant neoplasm that mostly affects children younger than 15 years of age. We report a patient with UES in the right lobe that was complicated by haemorrhage after needle biopsy. The tumour was managed by embolization of the right hepatic artery and treated successfully with chemotherapy and surgical resection. Prophylactic embolization of the feeding artery should be undertaken before a biopsy procedure if there is the possibility of tumour rupture, in the presence of signs of intratumoral or peritumoral bleeding, or in the presence of a vascular liver mass.     

Liver transplantation before 1 year of age

Since 1981, 20 infants younger than 1 year of age received 26 orthotopic liver transplants. Immunosuppression was with cyclosporine and corticosteroids. Thirteen (65%) of the recipients were discharged from the hospital. To date, 12 (60%) of the 20 recipients are surviving, with follow-up of 1 to 56 months (average 14 months). The 5-year actuarial survival is 53.8%. The allograft liver function in the majority of surviving infants is excellent. The predominant causes of mortality were primary nonfunction of the allograft (three patients) and sepsis (three). Major morbidity was caused by hepatic artery thrombosis (five patients), gastrointestinal complications (six), biliary tract complications (five), and bacterial and viral infections (13). Six patients underwent retransplantation; three of these six survived. Results could be improved by prevention of hepatic artery thrombosis, by decreasing the incidence of sepsis, and by procurement of more and better suited pediatric donors.     

Nodular regenerative hyperplasia of the liver: Description of two cases

Nodular regenerative hyperplasia (NRH) of the liver is a condition of unknown origin, rarely occurring in children, usually accidentally discovered, described in association with a variety of clinical conditions and drugs. Confusion with other types of hepatic masses may pose a problem and for this reason NRH is considered a “tumor-like lesion.” Histologically it consists of single or multiple regenerative foci. Hepatic failure and rupture of the liver have been rarely described as complications in adults, and not in children, and malignant transformation has not been demonstrated. Neither surgical removal nor other treatment is needed. These features are described as they were found in two patients we encountered. © 1996 Wiley-Liss, Inc.