[Hepatectomy for Intrahepatic Combined Hepatocellular Carcinoma Recurrence in a Hemophilia a Patient with Human Immunodeficiency Virus and Hepatitis C Virus-A Case Report].

A 40's Japanese man had a history of blood transfusion and administration of treatment coagulation factors for hemophilia A since he was 6 years old. He has been on IFN treatment for hepatitis C since he was 14 years old. Lastly, he has been undergoing HAART therapy for human immunodeficiency virus infection since he was 18 years old. Three years ago, he underwent partial hepatectomy for a tumor located in segment 8 of his liver and was diagnosed with combined hepatocellular carcinoma(CHC). Two years and 7 months after the operation, 2 intrahepatic recurrences were detected in the left lobe. He was referred to our hospital to undergo curative resection, and we performed a left lobectomy of the liver for the CHC recurrences. Perioperatively, supplemental factor Ⅷ was administered via APTT. Its activity was used as an index. Postoperatively, the patient was well, was discharged 13 days after surgery, and remained recurrence-free for 4 months.

[A Resected Case of Metachronous Solitary Metastasis of Jejunum after Surgery of Triple Colon Cancers].

A 68-year-old man underwent partial colectomy and double-barrel colostomy for an obstructive colon cancer of the splenic flexure at another hospital 10 years before. He was referred to us with an examination of anemia pointed out in human dock. Lower gastrointestinal endoscopy revealed the tumor occupied the remnant descending colon. We performed remnant left hemicolectomy and diagnosed as triple colon cancers. Six months after the initial operation, he was admitted to us with the chief complaints of abdominal fulness and vomit. Abdominal CT and radiologic enteroclysis after decompression used the ileus tube revealed complete stenosis at the small intestine. We performed surgery with a suspicion of obstruction of the small intestine. The tumor, 5 cm in diameter, occupied the jejunum was detected, and partial resection of the jejunum was performed. Histologically, the tumor was diagnosed as solitary metastasis of jejunum.

[Radiofrequency Ablation for Colorectal Liver Metastases].

AIM: The significance of radiofrequency ablation(RFA)for colorectal liver metastases(CRLM)remains to be elucidated. Therefore, this retrospective study aimed to evaluate the therapeutic efficacy of RFA for local recurrence of CRLM. SUBJECTS: Between June 2005 and June 2017, we retrospectively examined 63 patients(137 nodules)with CRLM who underwent RFA. RESULTS: The local recurrence rate was 36.5%, and the median local recurrence free survival(LRFS)was 26.3 months. We compared treatment background between the 2 groups with(50 nodules)and without(87 nodules)local recurrence. In the multivariate analysis, tumor size of the ablated lesion and method for ablation(direct tumor puncture)were independent risk factors for local recurrence. Receiver operating characteristic curve for tumor size of the ablated lesion showed an optimal cutoff value for tumor size of 1.8 cm(AUC=0.734, 95%CI: 0.612-0.855, p<0.0001). CONCLUSIONS: RFA for effective control of local recurrence of CRLM might be suitable for selected patients with tumor size of ablated lesion ofC1.8 cm and no touch ablation method.

[A Resected Case of Hilar Cholangiocarcinoma Confirmed as Pathological Complete Response Following Neoadjuvant S-1 Monotherapy].

A 74-year-old man was admitted to a local hospital with liver dysfunction. Imaging modalities revealed bile duct stenosis at the bifurcation of the anterior and posterior trunk. Exfoliative cytology of the bile and brushing cytology of the bile duct both revealed Class Ⅴ, and biopsy from the stenotic bile duct showed well differentiated adenocarcinoma. We diagnosed the patient with hilar cholangiocarcinoma and performed extended right bisectionectomy and biliary reconstruction after percuta- neous transhepatic right portal vein embolization(PTPE). Preoperatively, he was administered S-1(80mg/body weight/day) orally for 19 days. Histopathological assessment of the resected specimen revealed hemosiderin-laden macrophages without viable cancer cells, confirmingpatholog ical complete response(pCR).

Basiliximab treatment for steroid-resistant rejection in pediatric patients following liver transplantation for acute liver failure.

An IL-2 receptor antagonist, basiliximab, decreases the frequency of ACR in liver transplant (LT) recipients as induction therapy. The aim of this study was to evaluate the effectiveness of basiliximab against SRR as rescue therapy in pediatric LT patients with ALF. Forty pediatric ALF patients underwent LT between November 2005 and July 2013. Among them, seven patients suffering from SRR were enrolled in this study. The median age at LT was 10 months (6-12 months). SRR was defined as the occurrence of refractory rejection after more than two courses of steroid pulse therapy. Basiliximab was administered to all patients. The withdrawal of steroids without deterioration of the liver function was achieved in six patients treated with basiliximab therapy without patient mortality, although one patient developed graft loss and required retransplantation for veno-occlusive disease. The pathological examinations of liver biopsies in the patients suffering from SRR revealed severe centrilobular injuries, particularly fibrosis within one month after LT. We demonstrated the effectiveness and safety of rescue therapy consisting of basiliximab for SRR in pediatric LT recipients with ALF.

Technical considerations of living donor hepatectomy of segment 2 grafts for infants.

BACKGROUND: The selection of an adequate graft to mitigate the problems associated with a large-for-size graft is essential to ensure the success of liver transplantation for smaller children. Reduced left lateral segment (LLS) grafts have been introduced to overcome this issue. METHODS: Five infants underwent living donor liver transplantation (LDLT) with segment 2 grafts. In the preoperative assessment, the graft-to-recipient weight ratio (GRWR) and the ratio of the thickness of the donor LLS were used as a reference index for graft size matching, and a 3-dimensional (3D) computer-generated model of the donor liver was used for the analysis of the intrahepatic vasculature. During the donor operation, the relevant portal vein branches feeding to the reduced part of segment 3 were first exposed and divided, and then the parenchymal transection was performed. RESULTS: Segment 2 grafts were selected in 3 cases and reduced segment 2 grafts were selected in the other 2 cases. The graft reduction was achieved with 46.6 ± 8.2% of the actual LLS, and thus the GRWR was reduced from 5.33 ± 2.09% to 2.70 ± 0.82%. The actual graft thickness was reduced by approximately half after the graft reduction. Primary abdominal closure was performed in all of the recipients. No surgical complications occurred in any of the donors or recipients. CONCLUSION: A segment 2 graft could be a valuable option for graft type selection in LDLT for smaller children. Precise planning using a 3D computer-generated model of the donor liver and meticulous operative procedures are necessary to obtain a viable graft.

Successful living donor liver transplantation for giant extensive venous malformation.

We report our success in employing LDLT as a course of treatment for extensive hepatic VM. A 14-yr-old pediatric patient presented at our hospital with nosebleed, fatigability, orthopnea, and abdominal distension. He had a history of right hemicolectomy with primary anastomosis due to VM of the transverse colon at age seven. Coagulation abnormalities were apparent, characterized by high international normalized ratio of prothrombin time, decreased fibrinogen level, increased FDPs, and D-dimer. T2-weighted magnetic resonance imaging revealed numerous, variable-sized high signal intensity nodules. Abdominal ultrasonography and CT scan showed hepatomegaly with multiple hypo-echogenic lesions and arteriovenous shunting in the liver. Doppler ultrasound showed hypokinetic flow in the hypo-echogenic lesions of liver. Immediate LDLT was performed to avoid spontaneous rupture and DIC. The right lobe of the liver was implanted with temporary portocaval shunt to prevent intestinal congestion and bleeding. Pathologic examination of the explanted liver confirmed the presence of an extensive hepatic VM. The postoperative course was uneventful, and the patient remained symptom-free with normal liver function throughout the 12-month follow-up period.

Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature.

CPM is one of the most serious neurological complications that can occur after OLT and is characterized by symmetrical demyelinization in the basis pontis. The etiology of CPM remains unclear, although the rapid correction of the serum sodium and CNI concentrations may be associated with the development of CPM. With recent advances in MRI technology, early diagnosis of CPM has become possible. Here, we present the case of a five-yr-old female who developed CNI-associated CPM after undergoing LDLT. A decreased level of consciousness and dysphasia was noted one wk after LDLT, and MRI revealed findings compatible with a diagnosis of CPM. The patient fully recovered from the neurological deficits related to CPM following the switch from the CNI to sirolimus. We propose MRI to be promptly considered for patients with abnormal neurological findings, together with the substitution of CNI with an mTOR inhibitor as a management regimen for CNI-related CPM.

Urgent living donor liver transplantation for biliary atresia complicated by a strangulated internal hernia at Roux-en Y limb: a case report.

BACKGROUND: When BA patients with end-stage liver dysfunction have bowel obstruction, especially strangulated internal hernia, selecting optimal surgical therapeutic options is crucial. CASE REPORT: An 11-month-old female with end-stage biliary atresia (BA) was admitted for a strangulated internal hernia at the Roux-en Y limb and frequent episodes of gastrointestinal bleeding requiring blood transfusion. She was scheduled within a month to receive a portion of the liver from her blood-type identical mother. Despite intensive care, her clinical condition obviously needed a prompt surgical intervention. The operative findings at laparotomy revealed exudative moderate ascites and a dilated and ischemic afferent loop that was strangulated by a band extending from the mesentery to the transverse mesocolon. The attachment of the band was released, and gangrenous changes were recognized in the incarcerated bowel, although there were no obvious findings of intestinal perforation. After the gangrenous afferent loop was resected, the remnant afferent loop was too short to anastomose again. Following these procedures, as the patient's vital signs remained stable, we decided to simultaneously perform living donor liver transplantation (LDLT). She successfully underwent LDLT and her post-transplant course was uneventful. CONCLUSIONS: When faced with candidates for LT as an urgent life-saving surgery, determining whether LDLT should be performed simultaneously during perioperative management is necessary to save the life of the patient.

[Deceased donor liver transplantation receiving a liver from a child under six years old: an experience of organ retrieval from a pediatric donor in Japan].

We report a first pediatric liver transplantation from a brain-dead donor under 6-year-old in Japan. A 9-month-old girl suffered from graft failure after living donor liver transplantation for biliary atresia. The deceased donor liver transplantation (DDLT) from under 6-year-old was performed. It was important to select appropriate surgical devices for the pediatric organ recovery based on the preoperative evaluation. Sufficient vessel grafts were procured because of difficult vascular reconstruction. She discharged on 172 days after DDLT. There have been few cases of pediatric organ donation in Japan. Therefore we should make careful preparation for both of donor and recipient.

Impact of the current organ allocation system for deceased donor liver transplantation on the outcomes of pediatric recipients: a single center experience in Japan.

PURPOSE: The aim of this study was to analyze the outcomes of children added to the waiting list for deceased donor liver transplantation (DDLT) and the results of DDLT in a single Japanese center. METHODS: Forty-seven children were listed on the organ allocation system for DDLT. The priority points related to the medical status of each patient were evaluated and stratified into four categories; 10, 8, 6, and 3 points. The clinical data were collected from the medical records, and the outcomes were analyzed. RESULTS: There were 10 priority points in 25 patients, 6 points in 13 and 3 points in 9. Ten recipients (21.3 %); 7 patients with 10 points and 3 patients with 6 points, underwent DDLT. Seven out of the 10 recipients received split/reduced liver grafts. The surgical complications consisted of biliary stricture, hepatic venous outflow obstruction, intraabdominal abscess and intraabdominal bleeding. Two recipients, who were critically-ill before DDLT, died due to sepsis. The one-year graft survival rate was 70.0 %, with a median follow-up period of 6.4 months. CONCLUSION: The initial experience with pediatric DDLT in our series was satisfactory. Split LT of deceased donor organs may have the potential to resolve the serious organ shortage in Japan.

Living-donor liver transplantation with hyperreduced left lateral segment grafts: a single-center experience.

BACKGROUND: In the setting of liver transplantation in small infants who receive left lateral segment (LLS) grafts, problems are encountered related to graft-size mismatching in the form of so-called "large-for-size" grafts. To address these problems, the feasibility of further reducing the size of LLS grafts to form hyperreduced LLS (HRLLS) grafts was investigated. METHODS: Of the 175 pediatric living-donor liver transplantations performed between November 2005 and December 2011 at our institute, 31 cases were performed using HRLLS grafts. The medical records were reviewed and data were collected retrospectively. RESULTS: The graft-to-recipient body weight ratio was successfully reduced from 5.2% ± 2.0% to 2.9% ± 0.5%. Portal vein thrombosis was observed in one case, and biliary stenosis was seen in two cases. No hepatic artery thrombosis was encountered. The graft and patient 2-year survival rate was 87%. When the results categorized according to the original disease were verified, patients with fulminant hepatic failure (FHF) weighed less and had smaller abdominal cavities compared with patients with cholestatic or metabolic disease. Patients with FHF frequently required skin or partial skin closure to avoid graft compression. For this reason, the anteroposterior diameters in the recipients' abdominal cavities were not adequately large to accommodate the graft thickness, especially in patients with FHF. CONCLUSIONS: In conclusion, living-donor liver transplantation using HRLLS grafts offers a safe and useful option for treating smaller infants.

Living donor liver transplantation with alternative porto-left gastric vein anastomosis in patients with post-Kasai extrahepatic portal vein obstruction.

EPVO is a common cause of prehepatic portal hypertension in pediatric patients and sometimes results in cavernous transformation of the PV. We herein present the cases of two patients who underwent LDLT for EPVO with post-Kasai biliary atresia. PV reconstruction was performed with a porto-left gastric vein anastomosis. The patient who underwent PV reconstruction using an interposition vein graft is doing well without surgical complications, whereas PV anastomotic stenosis was detected three months after LDLT in the patient who did not receive an interposition vein graft. The availability of vein grafts is limited in the LDLT setting. In such cases, performing PV reconstruction with varicose veins using interposition vein grafts is a feasible and valuable alternative option for obtaining a sufficient portal blood flow. Our experiences suggest that using interposition vein grafts may be appropriate for preventing the anastomotic stenosis caused by the fragility of varicose veins.

[Initial experience of split liver transplantation in National Center for Child Health and Development, Japan].

PURPOSE: We reviewed our initial experience of deceased split liver transplantation in National Center for Child Health and Development and report the results herein. SUBJECTS AND METHODS: We have listed 50 patients with end-stage liver disease for deceased liver transplantation, and done 6 cases of split liver transplantation during October 2010 through October 2012. Variables including indication of liver transplantation outcome were reviewed. RESULTS: The indication for split liver transplantation was acute liver failure in 4, re-transplantation in 1 and primary sclerosing cholangitis in 1. The age of recipient ranged 17 days-15 yeras and body weight ranged 2.4 kg-55 kg. The split grafts were hyper-reduced left lateral segment in 1, left lateral segment in 3, left lobe in 1 and right lobe in 1. Although case #2 received retransplantation with living donor, all the patients are currently doing well with acceptable liver function with minimum follow-up of 2 months. CONCLUSION: Satisfactory result can be achieved on initial experience of split liver transplantation in National Center for Child Health and Development. The follow-up period was too short to make definitive conclusion, however, split liver transplantation could be expand potential donor pool and may reduce the waiting list mortality without alterations of the results.

Incidentally detected cholangiocarcinoma in an explanted liver with biliary atresia after Kasai operation.

This report presents the case of a 30-yr-old woman with BA who developed incidental cholangiocarcinoma following the Kasai operation. She showed progressive liver dysfunction and cirrhosis at the age of 30 yr and underwent LDLT. A 4-cm-diameter liver tumor in the anastomotic site of portoenterostomy was incidentally found as a result of a pathological examination of the explanted native liver. The tumor was pathologically diagnosed to be intrahepatic cholangiocarcinoma. Although cholangiocarcinoma in patients with BA has been previously reported in only three cases, it should be nevertheless always considered in the differential diagnosis of hepatic tumors during a long follow-up course in patients with BA.

Reducing the thickness of left lateral segment grafts in neonatal living donor liver transplantation.

Liver transplantation is now an established treatment for children with end-stage liver disease. Left lateral segment (LLS) grafts are most commonly used in split and living donor liver transplantation in children. In very small children, LLS grafts can be too large, and further nonanatomical reduction has recently been introduced to mitigate the problem of large-for-size grafts. However, the implantation of LLS grafts can be a problem in infants and very small children because of the thickness of the grafts, and these techniques do not address problems related to thickness. We herein describe a technique for reducing the thickness of living donor left lateral grafts and successful transplantation in a 2.8-kg infant with acute liver failure.

Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein.

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. A 14-year-old male with CAPV underwent living donor liver transplantation with a left lobe graft from his father. The portal vein reconstruction was achieved with a renoportal anastomosis using an interpositional graft from the native collateral vein, because portal venous system directly drains to the left renal vein without constructing the confluence of superior mesenteric vein and splenic vein. The patient is doing well with a normal liver function and mild hypoxemia.

Living donor liver transplantation using grafts with hepatic cysts.

Cystic lesions in the liver are often found through the evaluation of liver donors. Multiple cysts are worrisome, and donor candidates with multiple cysts may be unacceptable as liver donors, especially when their recipients have fibrocystic disease (FCD), which is an inherited disorder. This study reviewed 183 cases of living donor liver transplantation. We collected clinical and radiological data associated with donors with cystic lesions and with donors without cystic lesions, and we evaluated the outcomes of these donors and their recipients. As part of the preoperative radiological assessment of grafts, magnetic resonance cholangiography (MRC) was performed to evaluate the biliary anatomy of donor candidates with multiple cysts. Thirty-four donors (18.6%) had 1 or more cystic lesions in the liver, and 6 of these donors had multiple cysts (ie, >10). Donors with multiple cysts were older and heavier, and there was a significant relationship between these donors and recipients whose original disease was FCD. During the follow-up (median = 3.1 years), all donors with cystic lesions were found to be doing well without any major postoperative complications. Fifteen recipients who received grafts with cystic lesions (12 left-sided lobes and 3 right-sided lobes) had no complications related to the cystic lesions. In conclusion, donors with cystic lesions may be acceptable as liver donors, although our data are limited mostly to left-sided lobe donation with a short follow-up period. MRC should be preoperatively performed to rule out any biliary anomalies, especially in donor candidates whose recipients have FCD.