Segmental Liver Transplantation Research Articles

OHCCPredictor: an online risk stratification model for predicting survival duration of older patients with hepatocellular carcinoma

BackgroundAlthough the elderly constitute more than a third of hepatocellular carcinoma (HCC) patients, they have not been adequately represented in treatment and prognosis studies. Thus, there is not enough evidence to guide the treatment of such patients. The objective of this study is to identify the prognostic factors of older patients with HCC and to construct a new prognostic model for predicting their overall survival (OS).Methods2,721 HCC patients aged ≥ 65 were extracted from the public database-Surveillance, Epidemiology, and End Results (SEER) and randomly divided into a training set and an internal validation set with a ratio of 7:3. 101 patients diagnosed from 2008 to 2017 in the First Affiliated Hospital of Zhejiang University School of Medicine were identified as the external validation set. Univariate cox regression analyses and multivariate cox regression analyses were adopted to identify these independent prognostic factors. A predictive nomogram-based risk stratification model was proposed and evaluated using area under the receiver operating characteristic curve (AUC), calibration curves, and a decision curve analysis (DCA).ResultsThese attributes including age, sex, marital status, T stage, N stage, surgery, chemotherapy, tumor size, alpha-fetoprotein level, fibrosis score, bone metastasis, lung metastasis, and grade were the independent prognostic factors for older patients with HCC while predicting survival duration. We found that the nomogram provided a good assessment of OS at 1, 3, and 5 years in older patients with HCC (1-year OS: (training set: AUC = 0.823 (95%CI 0.803–0.845); internal validation set: AUC = 0.847 (95%CI 0.818–0.876); external validation set: AUC = 0.732 (95%CI 0.521–0.943)); 3-year OS: (training set: AUC = 0.813 (95%CI 0.790–0.837); internal validation set: AUC = 0.844 (95%CI 0.812–0.876); external validation set: AUC = 0.780 (95%CI 0.674–0.887)); 5-year OS: (training set: AUC = 0.839 (95%CI 0.806–0.872); internal validation set: AUC = 0.800 (95%CI 0.751–0.849); external validation set: AUC = 0.821 (95%CI 0.727–0.914)). The calibration curves showed that the nomogram was with strong calibration. The DCA indicated that the nomogram can be used as an effective tool in clinical practice. The risk stratification of all subgroups was statistically significant (p < 0.05). In the stratification analysis of surgery, larger resection (LR) achieved a better survival curve than local destruction (LD), but a worse one than segmental resection (SR) and liver transplantation (LT) (p < 0.0001). With the consideration of the friendship to clinicians, we further developed an online interface (OHCCPredictor) for such a predictive function (https://juntaotan.shinyapps.io/dynnomapp_hcc/). With such an easily obtained online tool, clinicians will be provided helpful assistance in formulating personalized therapy to assess the prognosis of older patients with HCC.ConclusionsAge, sex, marital status, T stage, N stage, surgery, chemotherapy, tumor size, AFP level, fibrosis score, bone metastasis, lung metastasis, and grade were independent prognostic factors for elderly patients with HCC. The constructed nomogram model based on the above factors could accurately predict the prognosis of such patients. Besides, the developed online web interface of the predictive model provide easily obtained access for clinicians.

Surgical therapy and survival in young patients with stage I-II hepatocellular carcinoma: a retrospective cohort study.

Hepatocellular carcinoma (HCC) is regarded as a high-mortality cancer, but the effectiveness of surgical strategies for young patients with early-stage HCC remains controversial. We aimed to analyze the survival in young patients with stage I-II HCC who underwent different kinds of surgical treatments. Overall survival (OS) and cancer-specific survival (CSS) were compared among patients aged 18-45 years with stage I-II HCC from the Surveillance, Epidemiology, and End Results (SEER) database (2004-2013) who underwent local tumor destruction (LTD), wedge or segmental resection (WSR), lobectomy resection (LR), liver transplantation (LT), or non-surgery. Univariate and multivariate analyses and Kaplan-Meier method were used to examine the OS and CSS of the patients. A stratification analysis of CSS was also conducted among the subgroups. Data from 664 patients were extracted. The median survival time was 46 months. In the multivariate analysis of OS, compared with non-surgery, LTD [hazard ratio (HR), 0.37; 95% confidence interval (CI): 0.25-0.54; P<0.0001], LR (HR, 0.29; 95% CI: 0.19-0.45; P<0.0001), and WSR (HR, 0.26; 95% CI: 0.17-0.39; P<0.0001) had better outcomes, and LT had the best survival benefit (HR, 0.24; 95% CI: 0.16-0.36; P<0.0001), which was similar to CSS. In the stratification analysis, compared with the non-surgery group, among patients with chemotherapy, LT reduced the risk of CSS by 64% (HR, 0.36; 95% CI: 0.19-0.66; P interaction=0.0004). Surgery offers a survival benefit compared with non-surgery for young patients with stage I-II HCC. LT is associated with better survival than WSR, LR, and LTD.

Transplantation in Turkey and region

The article provides insight into significant milestones in the history of organ transplantation in Turkey and region.&#x0D; In 1975, we were the first in Turkey to perform living‑related kidney transplantation and, in 1978, deceased‑donor kidney transplantation, using an organ supplied by Eurotransplant. In 1979, the law on harvesting, storage, grafting, and transplantation of organs and tissues was enacted. The first local deceased‑donor kidney transplantation was performed by our team in 1979. The first successful deceased‑donor liver transplantation, which was a groundbreaking surgery procedure for Turkey, the Middle East and Northern Africa, was carried out by our team in 1988. In 1990, we were the first not only in Turkey but also in the Middle East region as well as Europe to perform pediatric living‑related segmental liver transplantation. One month later, an adult‑to‑adult living‑related liver transplant (a left lobe) was successfully performed for the first time in the history of organ transplantation. On May 16, 1992, we carried out the first combined liver‑kidney transplantation from a living‑related donor, which was the first operation of its kind in the world. Between November 1975 and October 2021, we performed 3,256 kidney transplantations at Hacettepe University Hospitals . Since 1988 to date, we have carried out 695 liver transplantations at Baskent University. According to the registry of the Ministry of Health, from 2002 to 2021, 46,115 kidney transplants were performed nationwide, as compared with 17,868 liver transplants, 1,153 heart transplants and 198 pancreas transplants. In 2001, the Ministry of Health established the National Coordination Center as an umbrella organization to promote transplantation activities, especially deceased‑donor organ procurement. Despite constantly increasing overall living‑donor transplant rate across the country, overall deceased‑donor transplant rate is still far below the desired level.&#x0D;

S3483 Recurrent Pyogenic Cholangitis

Introduction: Recurrent pyogenic cholangitis is a chronic infective process characterized by recurrent attacks of acute bacterial cholangitis, in the setting of intrahepatic biliary ductal dilation and strictures, traditionally seen in South East Asia. Case Description/Methods: We present a 39 year old female who immigrated to the United States from Burma referred to gastroenterology clinic for abnormal liver tests and computed topography (CT) scan. Patient also reported intermittent right upper quadrant abdominal pain, fatigue and decreased appetite. Labs were significant for mildly elevated aspartame transferase (AST), alanine transferase (ALT) and alkaline phosphatase (AP). CT and magnetic resonance cholangiopancreatography (MRCP) were notable for intrahepatic biliary ductal dilation with multiple non-enhancing filling defects suggestive of stones or sludge, most pronounced in the left lobe of the liver and left hepatic lobe atrophy. Common bile duct was non-dilated without stones, sludge, wall thickening or abnormal enhancement. Liver elastography was performed which revealed cirrhosis. Patient did not have evidence of acute infection at this time but overall clinical presentation was consistent with recurrent pyogenic cholangitis. Discussion: Treatment options for recurrent pyogenic cholangitis include antibiotic therapy with signs of sepsis, removal of extrahepatic biliary stones via endoscopic retrograde cholangiopancreatography, decompression of intrahepatic biliary system via percutaneous biliary drainage and less likely surgical removal of diseased liver segment and potential liver transplantation. After discussion between an advanced endoscopist, general gastroenterologists, interventional radiologists and surgeons, she was started on ursodeoxycholic acid with plan for antibiotic therapy if evidence of acute infection. She will continue surveillance MRCP every 6 months for evaluation of hepatocellular carcinoma and cholangiocarcinoma.Figure 1.: Patient's CT image demonstrating intrahepatic biliary ductal dilation with hyperdense material concerning for biliary sludge, chronic obstruction of the left intrahepatic biliary tree and left hepatic lobe atrophy.

Percutaneous Transhepatic Treatment for Obstructing Retained Surgical Biliary Stents After Pediatric Liver Transplantation Without Placement of Biliary Drain

Percutaneous Transhepatic Treatment for Obstructing Retained Surgical Biliary Stents After Pediatric Liver Transplantation Without Placement of Biliary Drain

A220 AN UNEXPECTED CAUSE OF ACUTE JAUNDICE IN A POST-TRANSPLANT PATIENT

Abstract Background Complications post liver transplantation are common. These may include thromboses, primary graft nonfunction, biliary complications, chylothorax, infection, and rejection. Hepaticojejunostomy with Roux-en-Y limb is a common technique for pediatric liver transplantation. Complications of hepaticojejunostomy include small bowel obstruction due to incarceration of the Roux-en-Y limb within an internal hernia. Acquired diaphragmatic hernia has previously been reported in liver transplant patients. Here we present a unique case of acute biliary obstruction secondary to incarceration of a Roux limb within an acquired diaphragmatic hernia. Aims To describe a unique case of incarcerated Roux-en-Y limb in a diaphragmatic hernia leading to acute jaundice in a pediatric liver transplant patient. Methods Retrospective chart review and case review with the surgical and medical teams. Results A 14 month old girl with unresectable hepatoblastoma received a living donor, left lateral segment liver transplant using standard venous reconstruction, microvascular plastics arterial reconstruction, and the recipient common hepatic duct was anastomosed to the donor left hepatic duct. Serial ultrasounds showed persistent intrahepatic duct dilatation with cholestasis. Given the concern of biliary obstruction, 10 days post-transplant, she underwent repeat laparotomy and was found to have a dilated common bile duct with a proximal obstruction. A Roux-en-Y hepaticojejunostomy was performed to establish adequate biliary drainage. Three months post-transplant she developed lung metastases that required wedge resection and chemotherapy. 7 months post-transplant, while admitted for routine chemotherapy she acutely developed jaundice, pruritus, and transaminitis. Liver ultrasound showed an apparent fluid collection at the cut surface of the liver and biliary dilatation. Extrinsic compression of the biliary tree was suspected, and so a percutaneous biliary drain was placed to drain the collection and decompress the biliary system. Despite improvement in the transaminitis and cholestasis, several days later, she developed severe abdominal pain and associated grunting respirations. An MRI with MRCP to reassess the previous findings and rule out a new intraabdominal cause for her symptoms was completed and unexpectedly showed herniation of her Roux-en-Y limb into a diaphragmatic hernia. She had urgent diaphragmatic hernia repair with reduction of the incarcerated bowel loop, with resolution of her pain and jaundice. Conclusions Post liver transplant Roux-en-Y herniation is uncommon, but can occur with internal hernias and in diaphragmatic hernias. This uncommon complication should be considered for the post liver transplant patient with hepaticojejunostomy in the setting of acute jaundice, particularly if accompanied by acute onset abdominal pain and/or increased work of breathing. Funding Agencies None

A Novel Surgical Technique to Assure Donor and Recipient Safety: Pyloric to Segment 4 Arterial Reconstruction.

Background.Strategies to extend the pool of organs include and promote the use of segmental liver grafts. While performing a living donor left lateral segment (LLS) liver transplant and in split procedures, the hepatic artery´s division becomes critical when a dominant segment 4 artery (S4A) emerges from the left hepatic artery (LHA). We aim to describe a novel technique that consists of performing microsurgical reconstruction from the pyloric artery (PA) to S4A.Case Reports.A 45-y-old living donor was evaluated to use his LLS as a graft for a pediatric recipient. During the procedure, a dominant S4A born from the LHA was dissected. To obtain an appropriate LHA length and diameter for the recipient, it was necessary to transect it. An extended right lobe split graft was used in a 61-y-old patient. The S4A born from LHA had to be sectioned during the split procedure. In both cases, segment 4 remained incompletely perfused. The PA was dissected with enough length to be rotated, to perform a microsurgical anastomosis to the S4A, recovering parenchyma’s color and Doppler signal while vascular permeability was demonstrated using CT scan. There was no biliary or cut surface complication.Conclusions.PA to S4A reconstruction is a simple and novel technique that can be used for LLS and extended right lobe split graft and might contribute to increase donor selection and reduce living donor and recipient S4A-related complications.

Pediatric living donor left lateral segment liver transplantation for biliary atresia: Doppler ultrasound findings in early postoperative period.

To analyze hepatic hemodynamic parameters detected by Doppler ultrasound (DU) of uncomplicated children with biliary atresia who underwent left lateral segment living donor liver transplantation (LLS-LDLT), explore its normal change trend over time and determine the normal reference interval. We retrospectively involved the data from 227 biliary atresia patients (100 Males,127 Females). Hemodynamic parameters include peak systolic velocity (PSV), end-diastolic velocity (EDV), resistivity index (RI), and pulsation index (PI) of the hepatic artery (HA), portal vein velocity (PVV), portal vein flow (PVF) and hepatic vein velocity (HVV) during intra-operative and on the 1st, 3rd, 5th and 7th day after operation were collected. Repeated measures analysis of the variance and Friedman test were used to analyze the changing trend of hemodynamic parameters over time in the first week after the operation. PSVHA and EDVHA showed a similar changing tendency at one week after surgery, with an overall decrease-rise trend;RIHA and PIHAalso changed similarly with an overallrise-decrease trend. The HVV and PVV at surgery were lower than at all time points after surgery. As for PVF, the value of POD5 was the highest and then decreased. Additionally, this study provided the normal reference interval of hemodynamic parameters for LLS-LDLT patients, which were PSVHA: 18.4-98.3cm/s, EDVHA: 0-43.3cm/s, RIHA: 0.41-1.0, PIHA: 0.51-2.0, PVV: 19.0-83.7cm/s, HVV: 19.4-68.0cm/s, and PVF:99.5-500.0ml/min/100g at intraoperation. Within the first postoperative week: PSVHA: 21.0-97.7cm/s, EDVHA: 0-32.7cm/s, RIHA: 0.47-1.0, PIHA: 0.62-2.0, PVV: 23.0-92.0cm/s, HVV: 19.7-86.0cm/s, and PVF: 100.0-513.0ml/min/100g. The hepatic hemodynamic of post-transplanted children detected by DU had specific changing trends and normal ranges, which provides valuable reference values for ultrasonologists and pediatric transplant clinicians.

A prospective observational study of branching pattern of middle hepatic vein on contrast enhanced computed tomography

Background: The main purpose of pre-operative imaging in liver transplantation is to expose the arterial and venous vascular map. Prior to transplantation it is necessary to image the vascular structures property due to complex nature of liver vascular anatomy and its frequent variations. CT is a useful method not only in the determination of hepatic arterial anatomy, hepatic venous anatomy, accessory hepatic veins and portal veins variations.Methods: This was a prospective observational study performed at the department of surgery, Gandhi Medical College Bhopal and study of CECT was carried out in the department of radiodiagnosis with approval from college ethical committee on 100 patients during a period of 2 years from 2017 to 2019.Results: In study of 100 cases were studied. The study mainly done on the MHV mainly its length, width draining segments of liver by MHV and accessory hepatic veins. In overall cases maximum cases had drainage from segment IVb, V and segment VIII. The segment 5 accessory vein is the most common single accessory vein found in the study.Conclusions: Many parameters of MHV have studied in the study and understanding of these parameters is undoubtedly important for operating surgeon its anatomical draining patterns, draining liver segments and has got the presence of the various accessory veins in the liver. Recognition of these accessory hepatic veins is important because some of these accessory veins may become useful in segmental liver transplantation.

Biliary restoration using a combined endoscopic-percutaneous approach following “orphan duct syndrome” after pediatric liver transplantation

A 2-year-old male with arginosuccinate lyase deficiency underwent left lateral segment liver transplantation complicated by “orphan duct syndrome (Celik et al., 2019) [1]” and biliary leak. After revision of the Roux-en-Y anastomosis, biliary drainage was still impaired. The excluded bile duct was diagnosed and a biliary restoration (or neo-duct) using a combined endoscopic and percutaneous approach was created by Gastroenterology and Interventional Radiology. To our knowledge, this case report represents the first combined endoscopic-percutaneous biliary restoration procedure performed in a pediatric patient.

Identification of predictive factors in hepatocellular carcinoma outcome: A longitudinal study.

Various surgical methods impact the prognosis of patients with hepatocellular carcinoma (HCC) differently. However, clinical guidelines remain inconsistent and the relative importance of predictors of survival outcomes requires further evaluation. The present study aimed to rank the importance of predictive factors that impact the survival outcomes of patients with HCC and to compare the prognosis associated with different surgical methods based on data obtained from the Surveillance, Epidemiology and End Results database. To achieve these aims, the present study used a random forest (RF) model to detect important predictive factors associated with survival outcomes in patients with HCC. Cox regression analysis was used to compare different surgery methods. The variables included in the Cox regression model were selected based on the Gini index calculated by the RF model. Using the RF model, the present study demonstrated that surgery method, tumor size and age were the first, second and third most important factors associated with HCC prognosis, respectively. Overall, patients who underwent local tumor destruction [(hazard ratio (HR)=0.48; 95% confidence interval (CI), 0.45–0.51; P<0.001)], wedge or segmental resection (HR, 0.31; 95% CI, 0.29–0.33; P<0.001), lobectomy (HR, 0.29, 95% CI, 0.27–0.31; P<0.001) or liver transplantation (HR, 0.16; 95% CI, 0.14–0.17; P<0.001) demonstrated improved overall survival time compared with those treated with surgery, with a gradual decreasing trend observed in HRs. The present study demonstrated that the surgical method used is the most important predictor of the survival outcomes of patients with HCC. Liver transplantation resulted in the best prognosis for patients with HCC, except for those with undifferentiated tumors or distant metastasis.

AIUM Practice Parameter for the Performance of an Ultrasound Examination of Solid-Organ Transplants.

AIUM Practice Parameter for the Performance of an Ultrasound Examination of Solid-Organ Transplants.

A97 TACROLIMUS-ASSOCIATED HEMOLYTIC UREMIC SYNDROME IN A PEDIATRIC LIVER TRANSPLANT RECIPIENT

Abstract Background Tacrolimus associated hemolytic uremic syndrome (HUS) has been reported in renal and pediatric cardiac transplantation. Furthermore, in adults receiving liver transplant, there are reports of multiorgan failure, death, and end stage renal failure secondary to tacrolimus induced HUS [J NEPHROL 2003; 16: 580–585]. Although tacrolimus is commonly used in pediatric liver transplantation, there are no reports of the same entity. Aims To describe a case of tacrolimus induced HUS in a pediatric liver transplant patient and subsequent re-initiation of tacrolimus. Methods The patient was followed during his hospital admission and after discharge. Results An 8-year-old male with Progressive Familial Intrahepatic Cholestasis (PFIC) Type III, diagnosed at age 2, presents for living unrelated donor liver transplantation. He was previously well compensated but began to deteriorate in the months leading up to transplant with rising liver enzymes, ascites, and mild encephalopathy after presumed viral gastroenteritis. He receives a left lateral segment liver transplantation and is started on tacrolimus immunosuppression post liver transplant as per protocol. On initiation of tacrolimus, the patient’s hemoglobin trends downward to a trough of 59 g/L four days post transplantation. Concurrently, his haptoglobin is &amp;lt;0.10g/L, total bilirubin peaks at 708 umol/L, creatinine rises to a peak of 71 umol/L, urea peaks at 23.0 mmol/L. Platelets reach a nadir at 27x10^9/L, seven days post transplantation. Direct antibody test was negative, and blood cultures remained negative. Given the constellation of hemolytic anemia, thrombocytopenia, and acute kidney injury following initiation of tacrolimus, a diagnosis of atypical HUS is made. Tacrolimus is discontinued, intravenous methylprednisolone is started and the patient is switched to cyclosporine immunosuppression. Both the hemolysis and kidney injury resolve gradually. The patient develops side effects from cyclosporine including hypertension and excessive hair growth. One month post discontinuation of tacrolimus, he is started on sirolimus, while weaning steroids and cyclosporine. Seven weeks post discontinuation of tacrolimus, he is restarted on tacrolimus and is currently tolerating dual immunosuppression with no signs of HUS recurrence. Conclusions Atypical HUS secondary to tacrolimus post liver transplant in children is a rare entity. In the acute period, discontinuation of tacrolimus is essential to preserve renal function given potentially devastating outcomes seen in adults. Cyclosporine is an alternative immunosuppressive agent used in pediatric solid organ transplantation, although it has also been implicated in causing HUS. Funding Agencies None

Transjugular Liver Biopsy Following Left Lobe or Lateral Segment Transplantation in Pediatric Patients

To evaluate the efficacy and safety of transjugular liver biopsy in pediatric patients with left lobe or lateral segment liver transplant. This was a retrospective study of data from 104 transjugular liver biopsies (57 children) using an 18- or 19-G biopsy needle. Transjugular liver biopsy was selected due to coagulopathy and/or ascites in 56 (54%) procedures. The median patient age, body weight, and interval from liver transplantation were 56months, 16kg, and 2months, respectively. Technical success was defined as adequate liver specimens yield determined by the operator at the time of procedure. Complications, the longest length of obtained liver specimens according to needle type, and adequacy for histological diagnosis were analyzed. Biopsy using a biopsy needle was successfully achieved in 103 procedures (99%). In one procedure, liver samples were obtained using biopsy forceps following unsuccessful attempts with a biopsy needle. Major complication occurred in one procedure following biopsies using both a biopsy needle and biopsy forceps. The patient received transfusion to manage decreased blood pressure and hemoglobin levels although post-biopsy venography did not show extravasation. The median longest length of liver specimens using 18-G (n = 63) and 19-G (n = 40) needles were 10mm (range, 5-20) and 10mm (range, 5-20), respectively (p = 0.704). Liver specimens were adequate for histological diagnosis in all procedures. Transjugular liver biopsy using a biopsy needle appears to be a safe and effective method to obtain liver specimens for histological diagnosis in pediatric patients with left lobe or lateral segment liver transplant. Level 4, case series.

Diagnosis and treatment of intraductal papillary neoplasm of the bile duct

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare tumor arising from epithelia of bile duct, with a better prognosis compared with common cholangiocarcinoma. IPNB can occur anywhere along the biliary tree, and cause the dilation of the bile duct and acute cholangitis. Recent large case series of this peculiar disease in the far eastern countries showed greater incidences of hepatolithiasis and clonorchiasis compared with Western series. According to the immunochemistry features, IPNB can be classified into four subtypes, pancreaticobiliary, intestinal, gastric and oncocytic types. The pancreaticobiliary type is the most common and is usually associated with invasive lesions. Patients without distant metastasis can undergo surgical resection, surgical procedures include pancreaticoduodenectomy, hemihepatectomy, segmental liver resection and liver transplantation, and regional lymphectomy is necessary. Key words: Bile tract neoplams; Papillary neoplasms; Diagnosis; Therapy

A reliable scoring system after major liver resection in mice

BackgroundPosthepatectomy liver failure and its transplant counterpart, small-for-size syndrome, remain significant limitations for liver resections and segmental liver transplantation. Partial hepatectomy in mice is one of the most commonly used models to study liver regeneration, but blood and tissue sampling necessary to collect data can affect outcomes or even require euthanasia. We therefore developed a quantitative observational system to predict death from hepatectomy during the first 24 postoperative hours. Materials and methodsA total of 100 female, 10 to 12-week-old C57BL/6 mice underwent two-thirds hepatectomy and were monitored for up to 7 d. Our scoring system was based on five categories, each assigned 0-2 points: activity level, body posture, fur condition, respiratory status, and eye appearance. Seventy-five mice were scored 6 h, 12 h, 24 h, 2 d, 3 d, 5 d, and 7 d after surgery. The remaining 25 mice were scored similarly, but underwent, in addition, blood sampling for serum alanine aminotransferase, total bilirubin, interleukin-6, tumor necrosis factor-alpha, or euthanasia with liver sampling for conventional hematoxylin-eosin and Ki-67 staining. ResultsRetrospective analysis indicated that body condition scores ≤5 on two consecutive time points within the first 24 postoperative hours accurately predicted eventual death. Animals in the low scoring group also had significantly higher serum alanine aminotransferase, total bilirubin, interleukin-6, tumor necrosis factor-alpha, more hepatocyte necrosis in hematoxylin-eosin, and fewer Ki-67 positive hepatocytes. ConclusionsOur scoring system accurately predicts survival, hepatocyte damage, liver regeneration, and systemic inflammation in a mouse hepatectomy model, within the first 24 hours of surgery. This could be useful in evaluating posthepatectomy interventions for their effect on survival and liver regeneration.

Attenuation of Portal Hypertension by Continuous Portal Infusion of PGE1 and Immunologic Impact in Adult-to-Adult Living-Donor Liver Transplantation

Small-for-size syndrome remains the greatest limiting factor of expanding segmental liver transplantation from living donors. Portal hyperperfusion is considered to substantially contribute to small-for-size syndrome. We investigated the impact of continuous portal infusion of prostaglandin E1 (PGE1) on small-for-size grafts (SFSGs) in adult-to-adult living-donor liver transplantation (LDLT). From July 2003 to December 2009, LDLT was performed in 122 patients. We introduced continuous portal infusion of PGE1 to five SFSG patients (PG group) from November 2007 to December 2009 and retrospectively compared them with a historical control group of eight relevant SFSG patients without PGE1 infusion (non-PG group) from July 2003 to October 2007 to determine the safety and efficacy of continuous PGE1 portal infusion for SFSGs. Splenectomy cases were excluded from analysis. The PG group demonstrated significantly lower postoperative portal pressure than the non-PG group. Moreover, the PG group demonstrated significantly improved liver function in the early posttransplantation period and significantly better recovery from hyperammonemia at 1 week after transplantation and from hyperbilirubinemia in the late posttransplantation period. Overall survival was significantly better in the PG group than in the non-PG group. Three patients in the non-PG group died of rejection-related reasons. Interestingly, immunomonitoring assay revealed that antidonor immune responses were significantly accelerated in the non-PG group compared with the PG group after LDLT. In contrast, the PG group showed well-suppressed antidonor immune responses. Continuous portal infusion of PGE1 for SFSG attenuated portal hypertension, improved graft function, and suppressed antidonor immune responses, resulting in better survival.

Right diaphragmatic hernia after liver transplant in pediatrics: A case report and review of the literature

Diaphragmatic hernias (DH) are an unusual complication after pediatric liver transplantation; however, they have been reported with increased frequency in the past few years. DHs are responsible for nearly half of the small bowel obstructions requiring surgical intervention in this patient population. It has been suggested that the use of a left lobe liver graft, surgical trauma, malnourishment, elevated intra-abdominal pressures, and mTor inhibitors may predispose to development of DH. The use of a segmental graft may increase the recognition of diaphragmatic hernia because the surgically damaged right hemi-diaphragm often remains exposed to underlying viscera, instead of being covered by the right hepatic lobe. Treatment is surgical reduction, with up to 20% of the patients requiring resection of the herniated intestine. Herein we describe a case of DH after left segmental liver transplant in a two- yr-old boy that presented one month post left lobe split liver transplant with abdominal pain, anorexia, and respiratory distress. Just like in the majority of the reported cases, an urgent laparotomy with primary repair was performed. No resection of the herniated segment of intestine was required. For pediatric patients with otherwise unexplained respiratory or gastrointestinal symptoms after a left lateral segment liver transplant, right-sided diaphragmatic hernias should always be high in the differential diagnosis.

Diagnosis and management of polycystic liver disease

Polycystic liver disease (PLD) is arbitrarily defined as a liver that contains >20 cysts. The condition is associated with two genetically distinct diseases: as a primary phenotype in isolated polycystic liver disease (PCLD) and as an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD). Processes involved in hepatic cystogenesis include ductal plate malformation with concomitant abnormal fluid secretion, altered cell-matrix interaction and cholangiocyte hyperproliferation. PLD is usually a benign disease, but can cause debilitating abdominal symptoms in some patients. The main risk factors for growth of liver cysts are female sex, exogenous oestrogen use and multiple pregnancies. Ultrasonography is very useful for achieving a correct diagnosis of a polycystic liver and to differentiate between ADPKD and PCLD. Current radiological and surgical therapies for symptomatic patients include aspiration-sclerotherapy, fenestration, segmental hepatic resection and liver transplantation. Medical therapies that interact with regulatory mechanisms controlling expansion and growth of liver cysts are under investigation. Somatostatin analogues are promising; several clinical trials have shown that these drugs can reduce the volume of polycystic livers. The purpose of this Review is to provide an update on the diagnosis and management of PLD with a focus on literature published in the past 4 years.

Extrahepatic portal vein aneurysm after liver transplantation in a child: Case report

Portal vein aneurysms are very rare and represent <3% of all venous aneurysms. They can be congenital or acquired. Most patients do not have liver disease at diagnosis. Although uncommon, portal vein aneurysm has been described after liver transplant. We report the case of a six-yr-old girl who presented with an aneurysm of the extrahepatic portal vein after segmental liver transplantation. Because the patient was asymptomatic and owing to its extrahepatic location, this aneurysm has been successfully followed by clinical exam and imaging for four yr.