ABO-incompatible liver transplantation for children under 2 years of age: A case report and a single-center review.

Desensitization with RTX has been broadly introduced in adult LT across the ABO blood type barrier. For pediatric LT, the prophylactic use of RTX has not been standardized, especially for children under 2 years of age. A 20-month-old girl with BA underwent living donor LT from her ABO-I mother. On POD 6, she developed combined T cell-mediated and AMRs. Steroid bolus injection was immediately introduced, followed by antibody-depleting therapy with PE and IVIG. Based on a peripheral blood lymphocyte analysis by fluorescence-activated cell sorting, ATG and RTX were introduced for refractory rejection. Although she recovered from the combined rejections, IHBCs were inevitable as a consequence. We recommend extending the desensitization protocol to cover children under 2 years of age in order to prevent life-threatening complications.

Impact of graft thickness reduction of left lateral segment on outcomes following pediatric living donor liver transplantation.

Reducing graft thickness is essential to prevent large-for-size graft problems in pediatric living donor liver transplantation (LDLT). However, long-term outcomes of LDLT using reduced-thickness left lateral segment (LLS) grafts are unclear. In 89 patients who underwent LDLT using reduced LLS grafts between 2005 and 2017, short-term and long-term outcomes were compared between a nonanatomically reduced LLS (NAR-LLS) graft group and a reduced-thickness LLS graft group. Estimated blood loss was lower and abdominal skin closure was less needed in the recipient operation in the reduced-thickness LLS graft group. Postoperatively, portal vein (PV) flow was significantly decreased in the NAR-LLS graft group, and there was shorter intensive care unit (ICU) stay and fewer postoperative complications, especially bacteremia, in the reduced-thickness LLS graft group. Graft survival at 1 and 3 years after LDLT using reduced-thickness LLS grafts was 95.2% and 92.4%, respectively, which was significantly better than for NAR-LLS grafts. Multivariate analysis revealed that fulminant liver failure, hepatofugal PV flow before LDLT, and NAR-LLS graft were associated with poor graft survival. In conclusion, LDLT using reduced-thickness LLS grafts is a safe and feasible option with better short- and long-term outcomes in comparison with NAR-LLS grafts.

A severely cholestatic liver graft can be successfully used in deceased donor liver transplantation.

The shortage of deceased organs is still a serious issue in Japan. A proactive approach to using liver grafts from extended criteria donors (ECDs) may be one way of expanding the donor pool; however, if it is recklessly attempted, a recipient receiving such a marginal graft can be at risk of mortality due to primary non-function or delayed graft function. We herein report the successful outcome of a recipient receiving a severely cholestatic graft that was considered transplantable because it lacked features characteristic of a long duration of "cholestasis" according to the precise interpretation of a donor biopsy. Plasma exchange was intentionally introduced to prevent toxic insult by hyperbilirubinemia immediately after transplant. Despite transient acute kidney injury immediately after transplant, the patient's renal impairment was well managed with a renal-sparing immunosuppressive regimen consisting of basiliximab and mycophenolate mofetil. Although the use of liver grafts from ECDs still needs to be discussed, especially regarding graft selection and allocation policies, efforts not to discard valuable grafts should be undertaken in our country.

Necrotizing enterocolitis in the setting of milk allergy after pediatric living donor liver transplantation.

NEC is an idiopathic intestinal mucosal injury that may progress to transmural bowel necrosis without mesenteric ischemia. NEC usually affects 7- to 10-day-old neonates following enteral feeding. A 10-month-old girl with no history of laparotomy underwent LDLT for acute liver failure. After starting enteral feeding on postoperative day 5, she developed abdominal distention. Diffuse PVG and PI were detected by radiologic modalities. Exploratory laparotomy revealed patchy necrosis of the intestine without perforation. The microscopic findings of a resected specimen revealed transmural coagulative necrosis with multiple small thromboses compatible with neonatal NEC features, and eosinophil infiltration was also observed. Subsequently, after the resumption of enteral feeding with cow's milk, she developed severe diarrhea, the symptoms of which were eliminated after the administration of cow's milk was stopped. These clinical and pathological findings support the speculation that NEC might have been induced by a CMA. Food allergies, which can be induced by immunosuppressive agents, should be considered as a potential cause of NEC in the setting of pediatric liver transplantation.

Modified triangular hepatic vein reconstruction for preventing hepatic venous outflow obstruction in pediatric living donor liver transplantation using left lateral segment grafts.

HVOO can be a critical complication in pediatric LDLT. The aim of this study was to evaluate a modified triangular technique of hepatic vein reconstruction for preventing HVOO in pediatric LDLT. A total of 298 pediatric LDLTs were performed using a left lateral segment graft by 2 methods for reconstruction of the hepatic vein. In 177 recipients, slit-shaped anastomosis was indicated with partial clamp of the IVC. A total of 121 recipients subjected to the modified triangular anastomosis with total clamp of the IVC. We compared the incidence of hepatic vein anastomotic complications between these 2 methods. Nine of the 177 cases (5.3%) treated with the conventional technique were diagnosed with outflow obstruction. All 9 cases underwent hepatic vein reconstruction with the slit-shaped hepatic vein anastomosis. In contrast, there were no cases of outflow obstruction in the 121 cases treated with the modified triangular anastomosis. The modified triangular technique of hepatic vein reconstruction with total clamping of the IVC was useful for preventing HVOO in pediatric LDLT.

Living donor liver transplantation during the first 3 months of life.

Living donor liver transplantation (LDLT) is now an established technique for treating children with end-stage liver disease. Few data exist about liver transplantation (LT) for exclusively young infants, especially infants of <3 months of age. We report our single-center experience with 12 patients in which LDLT was performed during the first 3 months of life and compare the results with those of older infants who underwent LT. All of the patients were treated at the National Center of Child Health and Development, Tokyo, Japan. Between November 2005 to November 2016, 436 children underwent LT. Twelve of these patients underwent LT in the first 3 months of life (median age, 41 days; median weight, 4.0 kg). The indications for transplantation were fulminant hepatic failure (n = 11) and metabolic liver disease (n = 1). All the patients received the left lateral segment (LLS) in situ to mitigate the problem of graft-to-recipient size discrepancy. A reduced LLS graft was used in 11 patients and a segment 2 monosegment graft was used in 1 patient. We compared the results with those of infants who were 4-6 months of age (n = 67) and 7-12 months of age (n = 110) who were treated in the same study period. There were significant differences in the Pediatric End-Stage Liver Disease score and the conversion rate of tacrolimus to cyclosporine in younger infants. Furthermore, the incidence of biliary complications, bloodstream infection, and cytomegalovirus infection tended to be higher, whereas the incidence of acute cellular rejection tended to be lower in younger infants. The overall cumulative 10-year patient and graft survival rates in recipients of <3 months of age were both 90.9%. LDLT during the first 3 months of life appears to be a feasible option with excellent patient and graft survival. Liver Transplantation 23 1051-1057 2017 AASLD.

Living donor liver transplantation for post-Kasai biliary atresia: Analysis of pretransplant predictors of outcomes in infants.

After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into 3 groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants. Outcome was relatively worse in infants than older children, with overall survival at 1 and 5 years of 94.5% and 93.2%, respectively, and graft survival at 1 and 5 years of 91.1% each. Incidence of vascular complications was not significantly higher in infants. High Pediatric End-Stage Liver Disease (PELD) score (odds ratio [OR], 3.72; 95% confidence interval [CI], 1.30-10.67; P = 0.02) and portal vein (PV) hypoplasia (OR, 3.23; 95% CI, 1.10-9.52; P = 0.03) were independent risk factors for morbidity. Low weight-for-age z score (hazard ratio, 5.76; 95% CI, 1.05-31.47; P = 0.03) was identified as a significant risk factor for mortality after LDLT, but not age or absolute body weight (BW). Infants with BW deficit had a significantly smaller PV diameter (P = 0.005), greater blood loss (P = 0.001), and higher incidence of postoperative bacteremia (P = 0.01). In conclusion, high PELD score and PV hypoplasia were independent risk factors for morbidity, and BW deficit was associated with poor survival in infants with post-Kasai BA after LDLT. However, LDLT in these infants at the earliest possible time after referral is a feasible option with excellent patient survival in an experienced center. Liver Transplantation 23 1199-1209 2017 AASLD.

Optimizing hepatic venous outflow reconstruction for hepatic vein stenosis with indwelling stent in living donor liver retransplantation.

The patient was a boy of 7 years and 5 months of age, who underwent LDLT for acute liver failure at 10 months of age. HV stent placement was performed 8 months after LDLT because of intractable HV stenosis. At 7 years of age, his liver function deteriorated due to chronic rejection. The patient therefore underwent living donor liver retransplantation from his father. The HV was transected with the stent in situ. The IVC was resected due to stenosis. The pericardial cavity was opened and detached around the IVC to elongate the IVC. The divided ends of the IVC were joined by suturing to the posterior wall of the IVC. A new triangular orifice was made by adding an incision on the anterior wall of the IVC. The graft HV was then anastomosed to the new orifice with continuous sutures in the posterior wall and interrupted sutures in the anterior wall using 5-0 non-absorbable sutures. Doppler ultrasound showed a triphasic waveform. We successfully performed HV reconstruction without a vascular graft. This is a feasible procedure for overcoming HV stenosis in LDLT patients with an indwelling stent.

Anatomical Resection of the Bile Duct: A Hidden Door to the Bile Duct.

BACKGROUND/AIMS: Pancreas-preserving resection of the bile duct has been attempted as an organ preserving procedures for the treatment of low-grade malignant neoplasms of the bile duct. The fact that the lower bile duct penetrates pancreas head to join the duodenum, makes those attempts one of the challenging procedures in biliary tract surgery. Here we present a novel and unique surgical technique for anatomically resecting lower bile duct, focusing on the anatomy of the pancreas head. METHODOLOGY: A patient with middle bile duct cancer underwent this procedure. Subsequent to Kocher's maneuver, pancreas head was dissected from the posterior side of the duodenum that was a key step to recognize the embryological fusion plane between the anterior and the posterior pancreatic segments. Along this fusion plane pancreas head was able to be divided and the covering pancreatic parenchyma was split open to expose the whole intrapancreatic bile duct. RESULTS: The patient had no signs of pancreatic fistula and post-operative course was uneventful. Negative surgical margins were obtained thanks to the presented technique. CONCLUSIONS: This procedure might be applicable for the treatment of bile duct neoplasms, in case of lesions spread to the lower bile duct. Otherwise that might require pancreatoduodenectomy.

[OPERATIONAL EXPERIENCE OF AUTOMATIC POSTING SYSTEM TO THE NATIONAL CLINICAL DATABASE USING FILEMAKER PRO].

We would like to introduce a semi-automated registration system for the National Clinical Database. Japan Surgery Society runs the National Clinical Database since January 2011. And automated registration system is long-awaited due to the number of cases and diverse items to register. Well-known database management system, FileMakerPro enabled collecting and registering the items to the web-site automatically. Still some items need to copy one by one, but after filling the data, all data would be input to the web page for registration. Merit for this automated system is increasing not only the efficiency of administration work but the quality and the usefulness of this database. This paper is to report the outline of the registration system to the NCD database.