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Liver transplantation (LT) for small infants6 months old is rare but becoming common as perioperative care improves. In Taiwan, living donor LT (LDLT) has expanded indications but is rarely performed for this age group because of unfavorable outcomes in the literature. We evaluated LDLT outcomes of patients6 months old.We identified infants6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed.Nine patients were identified. Indications for LT were biliary atresia (n = 2), Alagille syndrome (n = 1), protein C deficiency (n = 1), and acute liver failure (n = 5), including two patients with neonatal hemochromatosis, one with herpes simplex hepatitis, one with giant cell hepatitis with autoimmune hemolytic anemia, and one with hemophagocytic lymphohistiocytosis. Median age and weight at LT were 129 days and 4.8 kg, respectively. Graft types included left lateral segment (LLS, n = 4), hyper-reduced LLS (n = 4), and monosegment (n = 1). The median graft-to-recipient weight ratio was 4%. The median follow-up period was 14 months (range, 8 days to 127 months) with two mortalities, and two patients were totally weaned off immunosuppressants. Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. Preoperative reconstructive imaging for estimating graft thickness facilitated surgical planning.Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.

Total laparoscopic donor right hepatectomy (TLDRH) for adult living liver donors has been reported by a few experienced centers, but with limited cases, its safety and feasibility remain controversial. We report our experience initiating TLDRH using a stepwise approach to gradually convert laparoscopy-assisted donor right hepatectomy (LADRH) to TLDRH. We retrospectively analyzed the data of 61 LADRHs, 56 conventional open donor right hepatectomies (CODRHs), and 3 TLDRHs performed between March 2014 and June 2018. There were no significant differences in perioperative outcomes between donors undergoing LADRH and CODRH, except for a slight elevations in the operative time (436.5 vs 392.9 min, p

Background Most patients with isolated methylmalonic acidemia (MMA) /propionic acidemia (PA) presenting during the neonatal period with acute metabolic distress are at risk for death and significant neurodevelopmental disability. The nationwide newborn screening for MMA/PA has been in place in Taiwan from January, 2000 and data was collected until December, 2016. Results During the study period, 3,155,263 newborns were screened. The overall incidence of MMA mutase type cases was 1/121,356 (n = 26), 1 cobalamin B was detected and that for PA cases (n = 4) was 1/788,816. The time of referral is 8.8 days for MMA patients, and 7.5 days for PA patients. The MMA mutase type patients have higher AST, ALT, and NH3 values as well as a lower pH value (p

Background Fulminant liver failure (FLF) is a life-threatening disease. Methods Lethal FLF was induced by ischemia-reperfusion (I-R) injury in mini-pigs, and MSCs were infused via splenic vein after reperfusion. Results Accumulated survival within 28 days was significantly improved by MSCs (P = 0.0348). Notably, MSCs maintained blood-gas homeostasis in the first 24 hours and prevented FLF-induced elevation of prothrombin time, international normalized ratio, and creatinine and ammonia levels in the first 3 days. With MSCs, serum levels of liver enzymes gradually decreased after 3 days, and platelet count was back to normal at 1 week of FLF. MSCs promoted liver regeneration within 2 weeks and differentiated into functional hepatocytes at 2-4 weeks after transplantation, evidenced by increase in Ki67-positive cells, detectable human hepatocyte growth factor, human vascular endothelial growth factor, human hepatocyte-specific antigen, and human albumin-expressing cells in the liver at different time points. Reactive oxidative species (ROS) were accumulated after FLF and eliminated at 4 weeks after MSC transplantation. Conclusions Together, MSCs prolong the survival and prevent lethal sequelae of I-R injury-induced FLF by maintenance of liver-function homeostasis and rescue of ROS in the acute stage and by homing and differentiation into hepatocytes in the subacute stage.

Background Antifibrosis therapy may prevent progressive liver fibrosis after successful Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. The aim of this study is to evaluate the efficacy of antifibrosis therapy in a rat model of BA and KPE. Methods BA model was created on three-week-old Sprague–Dawley rats by intrabiliary alcohol injection as previously described, and KPE was performed at postoperative week (POW) 5 by cystoenterostomy. Liver biopsies were performed at the time of BA creation, during KPE, POW 9, and at sacrifice (POW 17). Prednisolone (0.1 mg/100 g/day, group 1, n = 20), Vitamin A (0.5 mg/100 g/day, group 2, n = 20), and ursodeoxycholic acid (UDCA, 1.5 mg/100 g/day, group 3, n = 20) were respectively given to three groups after KPE and continued daily until sacrifice. Histological evaluation of fibrosis and immunohistochemistry stains for 8 fibrosis markers were compared to the control group (without medication, n = 10). Results Among the four markers, namely ɑ-smooth muscle actin (ɑ-SMA), glial fibrillary acidic protein (GFAP), tumor growth factor β1 (TGFβ1) receptors 1 and 2, which showed persistently high expression after successful KPE in the examined 8 markers, only the expression of ɑ-SMA was significantly reduced in all treatment groups at POW17. However, the fibrosis grade at POW 17 was only significantly reduced in group 2 in comparison with the control group (Vitamin A vs. control group, Ishak score 3 vs. 1.8, p Conclusion In our rat model of BA with KPE, Vitamin A was effective in reducing liver fibrosis, and the mechanisms deserve further study. Level of evidence Basic science.

Background Endovascular intervention with stent placement to treat portal vein (PV) and hepatic vein (HV) stenosis after pediatric liver transplantation (LT) is still controversial in small children owing to the potential risk of functional stenosis after growth. The aim of this study is to evaluate the safety and efficacy of stent placement in this population. Methods Between 2004 and 2016, 6 children (all n = 2) and PV ( n = 4) stents placement among 46 pediatric LT patients at our institution. The clinical outcome and patency rate were followed. Morphologic changes of stents were assessed from plain films by a new index: the stent diameter ratio (SDR). Results The median age of the patients at LT was 8.9 months. The patency rate was 100% without functional stenosis during a median follow-up period of 65.5 months. The "stent growth" phenomenon was demonstrated by SDR with significant resolution of hourglass deformity 2 years after stent placement ( p for trend Conclusion Vascular stent placement is a safe and effective method for the management of PV and HV stenosis following pediatric LT because these stents will enlarge as children grow. Type of study Case Series with no Comparison Group Level of evidence Level IV.

Hepatic-based metabolic disorders are characterized by an enzyme deficiency expressed solely or mainly in the liver. They are divided into cirrhotic or non-cirrhotic metabolic liver diseases (NCMLDs), and most of them can be treated by liver transplantation. Because the livers with NCMLDs are usually structurally and functionally normal, the primary aim of the liver graft is to support the deficient enzymes rather than maintaining liver functions. Hence, we hypothesize that the exchange of partial liver grafts by the technique of auxiliary partial orthotopic liver transplantation (APOLT) between patients with 2 different NCMLDs may be feasible to replace the deficient enzymes in each patient. This hypothesis is based on the following conditions: (i) the patients have no chance of undergoing timely liver transplantation, (ii) the symptoms of each NCMLD may be alleviated after exchanging partial liver grafts, and (iii) each graft is anatomically appropriate for APOLT. In addition, we evaluate it with a focus on selection of cases, designing of graft sizes, and surgical techniques for reciprocal APOLT.

A 24-yr-old man was diagnosed with HCU during neonatal screening and remained on a pyridoxine, vitamin B12, folic acid, and betaine regimen with dietary methionine restriction for more than 10 yr. He had normal mental development, marfanoid appearance, myopia because of lens dislocation, and recurrent ankle subluxation during adolescence. Thereafter, he was a poor adherent to the conventional diet-restrictive therapy, and LT was considered when he developed hypertension and multiple infarctions over the right cerebellum early in the second decade of his life despite taking aspirin as a prophylaxis from 17 yr of age. In November 2009, he received a deceased whole LT from a blood group compatible donor. Along with the success of the transplantation, he was completely disease free without dietary or nutritional control. To the best of our knowledge, this is the first case of LT intended to cure HCU, and with promising results. This case provides an insight into the role of LT for this congenital metabolic disease, for which the decision should be made by judging between the severity of the disease and the risk of the operation, as well as the life quality of the patient.

SummaryCholedochoduodenal fistula (CDF) complicated by peptic diseases or following surgical or endoscopic approaches of the common bile duct is not uncommon. However, it usually occurs without significant symptoms and can be well controlled with conservative treatment in normal immunized patients. Here we report a case involving a 58-year-old male patient with diabetic nephropathy, who received a choledocholithotomy for choledocholithiasis in November 2007 and renal transplantation in March 2008. The patient had recurring cholangitis during the 5 months following his renal transplantation. Cholangiography and liver biopsy revealed sclerosing cholangitis. The patient underwent liver transplantation (LT) in May 2009 because radiological and endoscopic procedures failed to control his jaundice. A proximal CDF was found during the LT procedures. We considered that the patient's advanced secondary sclerosing cholangitis was induced by this fistula. At the 16 months' follow-up, the patient was surviving well and the graft remained intact. To our knowledge, this is the first report of a renal transplantation recipient receiving LT because of uncontrolled cholangitis caused by a CDF.

Retrograde arterial flush (RGAF) of liver grafts, which prevents arterial intima injuries in living donor liver transplantation (LDLT), seemed to reduce post-transplantational cholestasis by our preliminary study. The aim of this study was to further confirm this effect of RGAF of grafts in LDLT by a prospective randomized study. The enrolled cases were randomly divided into two groups, RGAF (portal flush plus RGAF) and non-RGAF (portal flush only). The outcome measures included intra-operative hemodynamic changes, one-month post-transplantational liver function tests and acute cellular rejection, vascular and biliary complications, the length of postoperative hospital stay, and graft and patient survivals. The significant findings of results were lower postoperative serum bilirubin in the RGAF group until three wk after transplantation (days 1, 3, 7, 14, and 21 serum total bilirubin, mg/dL: 5.82±5.46, 3.95±4.28, 4.87±5.01, 3.62±5.32, and 3.63±6.91 vs. 8.41±5.49, 7.47±5.61, 10.0±10.1, 9.06±11.6, and 6.02±9.84, respectively, in RGAF and non-RGAF, p=0.017, 0.002, 0.014, 0.011, 0.109) and shorter postoperative hospital stay in the RGAF group (31.1±17.6 vs. 44.8±32.3 d, respectively, in RGAF and non-RGAF, p=0.035). In conclusion, the RGAF of liver grafts on a back table may ameliorate postoperative functional cholestasis in LDLT, which possibly reduces postoperative hospital stay.

Liu C, Niu D-M, Hsia C-Y, Loong C-C, Lin N-C, Tsai H-L, Tsou M-Y, Chin T. Living donor liver transplantation using a graft from a donor with Dubin–Johnson syndrome. Pediatr Transplantation 2012: 16: E25–E29. © 2010 John Wiley & Sons A/S. Abstract: DJS is an autosomal recessive disorder that causes an increase in conjugated bilirubin without elevation of liver enzymes. Most patients are asymptomatic and have normal life spans, but to the best of our knowledge, their livers have never been reported to be grafts in liver transplantation. Herein, we report an infant patient with MMA that received a partial liver graft from his mother, who had DJS. A biliary anastomosis stricture was noted five months after transplantation and was successfully treated with radiological interventions. Otherwise, the patient’s liver functions were normal, and a liver biopsy showed a pathognomonic picture of DJS nine months after the transplantation. The patient was followed for one yr, and the results were satisfactory for an increase in oral intake and protein uptake, no recurrence of metabolic stroke and there was a gradual catch-up with regard to physical development despite having a persistently abnormal profile of amino acid analysis. From the experience of our case, we suggest that a liver from a donor with DJS can be used as a graft for liver transplantation, although long-term follow-up is mandatory to examine the grafted liver under the use of immunosuppressive medications.

We aimed to minimize the dose of tacrolimus in pediatric patients undergoing liver transplantation prospectively.Pediatric liver transplant recipients with stable graft function1year (transplant at1year of age), or 2years (transplant at1year of age) post transplant were screened. After baseline graft biopsy, patients were enrolled into our protocol for elective tacrolimus dose reduction. Patients were assessed by liver function test and protocol biopsy during and after tacrolimus dose reduction.From January 2011 to December 2012, 16 patients were recruited, of whom 15 completed follow-up at a mean 40.75±5.98months. Six patients were preliminarily weaned off tacrolimus, and five remained tacrolimus-free for more than 2years. Of the 10 patients who were not weaned off tacrolimus, six experienced seven episodes of clinical rejection. Five patients had a reduction in tacrolimus dosage to an undetectable trough level, another five to a trough level4ng/ml, including one patient who was off the study. At the last patient visit, all of the patients had normal liver function test results with no graft loss. Three patients had low-grade graft fibrosis. The patients with metabolic liver disease (p=0.039) and who were recruited earlier after transplantation (p=0.028) were more likely to be weaned off tacrolimus.Tacrolimus withdrawal is feasible in select pediatric liver transplant recipients, and long-term follow-up for these patients is suggested.

Delayed PV complications are not rare in pediatric liver transplantation. Although PTPV offers a treatment and minimizes surgical revision, in case of complete PV thrombosis (PVT), the failure rate of PTPV is high. Herein, we report a successful technique of PTPV in a case of complete PVT with a stent placement using a bidirectional approach in a child with living donor liver transplantation.

This review assesses the current status of laparoscopic liver resection.The trend in laparoscopic liver resection has been moving from limited resections toward major hepatectomy. The surgical techniques for laparoscopic major hepatectomy include pure laparoscopic, hand-assisted laparoscopic, and laparoscopy-assisted methods. We performed a literature search and systematic review to assess the current status of laparoscopic major hepatectomy.Our literature review was conducted in Medline using the keywords "laparoscopy" or "laparoscopic" combined with "liver resection" or "hepatectomy." Articles written in English containing more than 10 cases of laparoscopic major hepatectomy were selected.Twenty-nine articles were selected for this review. The laparoscopic major hepatectomies achieved similar patient and economic outcomes compared with open liver resections in selected (noncirrhotic) patients. Surgeon experience with the techniques affected the results; thus, a learning period is mandatory. Of these 3 techniques, the pure laparoscopic method is suitable for experienced surgeons to achieve better cosmetic outcomes, whereas the hand-assisted laparoscopic method was associated with better perioperative outcomes; the laparoscopy-assisted method is used by surgeons for unique resections such as resection of cirrhotic livers, laparoscopic resection of tumors in unfavorable locations, and living donor hepatectomies. In addition, the laparoscopic major hepatectomy-specific, long-term oncologic outcomes remain to be addressed in future publications.

Although the lateral segment (LS) from the split-liver of a deceased donor or a live donor can increase the organ pool of pediatric patients awaiting liver transplantation, the shortage of organ donation in Asia countries is still serious and results in high death rates of pediatric patients. The medial segment (MS) of the liver is sacrificed during the standard technique of splitting a whole liver into an LS and an extended right liver because the cutting sites of portal vein, hepatic artery and bile duct are all in the bifurcation of the liver hilum to have adequate length of vascular and biliary pedicles for easier grafting. However, the surgical techniques of vascular and biliary reconstructions for liver transplantation, particularly from the experiences of living donor liver transplantation, have been much improved in the last decade. Therefore it may be possible for an additional MS of the liver to be an isolated graft for a small recipient on the premise that grafts of right lobe (RL) and LS are minimally injured. In light of detailed reviews of anatomies of hepatic arteries, hepatic veins, portal veins and bile ducts, the dissection and reconstruction of vessels and bile ducts for the MS can possibly be performed if the extra-hepatic length of the artery to the MS is long enough. As the artery for the MS, middle hepatic artery (MHA), usually derives from a branch of the left hepatic artery and often in the liver parenchyma, the length is usually too short to be reconstructed. If the MHA for the MS is isolated and its extra-hepatic length is more than 1cm, triple liver grafts from a deceased whole liver, consisting of the RL, MS and LS may be possible. The anatomies of the hepatic artery in abdominal computed tomography (CT) or magnetic resonance imaging (MRI) for live liver donors in our institution were retrospectively analyzed. The results showed that three types of hepatic arterial anatomies could be considered for possible recovery of triple segments: type I is an accessory left gastric artery to feed the lateral segment; type II is an isolated MHA; type III is an early bifurcation of the left hepatic artery and MHA.

Background: Liver transplantation is a challenging procedure that is associated with perioperative morbidity and mortality, so it is justifiable to perform such a procedure in high-volume procedure centers. Organ shortage remains a major issue in Taiwan. Due to the difficulty in establishing a high-volume procedure center, it is important to review the overall outcome of patients undergoing liver transplantation at a small-volume procedure center to determine if performing such a procedure is justified. Methods: Between April 2001 and May 2005, 26 adults underwent deceased donor liver transplantation at Taipei Veterans General Hospital. The overall outcomes were reviewed in terms of 90-day mortality, 1-year and 3-year survival rates. In addition, the patients were divided into a hepatocellular carcinoma (HCC) group (n = 12) and a benign end-stage liver disease (ESLD) group (n = 14). The clinical demographics, 90-day mortality, 1-year and 3-year survival rates were reviewed and compared between the 2 groups. Results: The 90-day mortality was 15.3% in the whole series, 8.3% in the HCC group and 18.7% in the ESLD group. The overall 1-year and 3-year survival rates were 76.9% and 63.5%, respectively, for the whole series. For the 2 groups, the respective 1-year and 3-year survival rates were 83.3% and 71.4% in the HCC group, and 71.4% and 57.1% in the ESLD group. The survival difference was not significant (p = 0.319) between the 2 groups. In the HCC group, the 1-year and 3-year disease-free survival rates were 88.9% and 71.1%, respectively. Conclusion: The survival rates between ESLD and HCC patients undergoing liver transplantation at a small-volume procedure center were comparable. The results of the whole series were not satisfactory, but the results for the HCC group were acceptable. [J Chin Med Assoc 2008;71(4):186–190]