佐久間 康成

症例は69歳，男性．CT，MRIで肝右葉に早期濃染される最大径15 cmの巨大腫瘍と外側区域と尾状葉に数個の小腫瘍を認め，PIVKAIIの高値と画像所見から肝細胞癌と診断した．総コレステロール855 mg/dl ，トリグリセリド753 mg/dl と著明な高脂血症も認めた．2年前の総コレステロールは250 mg/dl であった．胆汁うっ滞所見がなく，高脂血症の家族歴もなく，急激な上昇を認めていることから腫瘍随伴症候群による高脂血症と考えられた．右肝の主腫瘍切除後，総コレステロール・トリグリセリド値は急速に改善したが，術後早期に肝内再発をきたし，肝動脈化学塞栓療法を行うも著効なく，骨転移により急速な転帰をたどった．<br>

Bilioenteric anastomotic stricture after liver transplantation is still frequent and early detection and treatment is important. We established the management using double-balloon enteroscopy (DBE) and evaluated the intractability for bilioenteric anastomotic stricture after pediatric living donor liver transplantation (LDLT). We underwent DBE at Jichi Medical University from May 2003 to July 2009 for 25 patients who developed bilioenteric anastomotic stricture after pediatric LDLT. The patients were divided into two types according to the degree of dilatation of the anastomotic sites before and after interventional radiology (IVR) using DBE. Type I is an anastomotic site macroscopically dilated to five times or more, and Type II is an anastomotic site dilated to less than five times. The rate of DBE reaching the bilioenteric anastomotic sites was 68.0% (17/25), and the success rate of IVR was 88.2% (15/17). There were three cases of Type I and 12 cases of Type II. Type II had a significantly longer cold ischemic time and higher recurrence rate than Type I (P = 0.005 and P = 0.006). In conclusion, DBE is a less invasive and safe treatment method that is capable of reaching the bilioenteric anastomotic site after pediatric LDLT and enables IVR to be performed on strictures, and its treatment outcomes are improving. Type II and long cold ischemic time are risk factors for intractable bilioenteric anastomotic stricture.

PURPOSE: Bowel perforation after liver transplantation (LT) is a rare, but highly lethal complication with a poor prognosis. Here, we report the outcome of cases of bowel perforation after pediatric LT in our department. PATIENTS AND METHODS: The study subjects were 148 patients who underwent pediatric living donor liver transplantation. The 114 with biliary atresia (BA) were divided into two groups: those with associated bowel perforation (Group A) and those without (Group B). RESULTS: Four patients in all (2.5%) suffered bowel perforation. Their original disease was BA and emergency surgery was performed in all cases, with a mortality rate of 50.0%. Comparison of Groups A and B revealed significant differences in the patient age, body weight, duration of surgery, cold ischemic time, and blood loss volume. The survival rates in Groups A and B were 50.0 and 99.1%, respectively (p < 0.01). Duration of surgery was an independent risk factor (p = 0.05). CONCLUSION: Bowel perforation after LT is a potentially fatal complication. LT is a procedure that requires care and precision, and the possibility of bowel perforation should always be borne in mind during post-operative management, when the duration of surgery has been long.

OBJECTIVES: To describe our experience with 126 consecutive living-donor liver transplantation (LDLT) procedures performed because of biliary atresia and to evaluate the optimal timing of the operation. PATIENTS AND METHODS: Between May 2001 and January 2010,126 patients with biliary atresia underwent 130 LDLT procedures. Mean (SD) patient age was 3.3 (4.2) years, and body weight was 13.8 (10.7) kg. Donors included 64 fathers, 63 mothers, and 3 other individuals. The left lateral segment was the most commonly used graft (75%). Patients were divided into 3 groups according to body weight: group 1, less than 8 kg (n = 40); group 2,8 to 20 kg (n = 63); and group 3, more than 20 kg (n = 23). Medical records were reviewed retrospectively. Follow up was 4.5 (2.7) years. RESULTS: All group 3 donors underwent left lobectomy, and all group 1 donors underwent left lateral segmentectomy. No donors required a second operation or died. Comparison of the 3 groups demonstrated that recipient Pediatric End-Stage Liver Disease score in group 1 was highest, operative blood loss in group 2 was lowest (78 mL/kg), and operative time in group 3 was longest (1201 minutes). Hepatic artery complications occurred more frequently in group 1 (17.9%), and biliary stenosis (43.5%) and gastrointestinal perforation (8.7%) occurred more frequently in group 3. The overall patient survival rates at 1, 5, and 9 years was 98%, 97%, and 97%, respectively. Five-year patient survival rate in groups 1,2, and 3 were 92.5%, 100%, and 95.7%, respectively. Gastrointestinal perforation (n = 2) was the primary cause of death. CONCLUSIONS: Living-donor liver transplantation is an effective treatment of biliary atresia, with good long-term outcome. It seems that the most suitable time to perform LDLT to treat biliary atresia is when the patient weighs 8 to 20 kg.

BACKGROUND: There have been few reports on the management of intra-abdominal drains after living donor liver transplantation (LDLT). We retrospectively investigated changes in ascitic data related to management of an intra-abdominal drain. PATIENTS AND METHODS: Between March 2008 and June 2009, we performed 28 LDLT. On the first and the fifth postoperative day (POD) after LDLT, we examined the number of ascites cells and cell fractions as well as performed biochemical examination and cultures. RESULTS: The day of removal of the drain for massive ascites (10 mL/kg/d or more) was 14.2 ± 5.4 POD; for less than 10 mL/kg/d it was 8.7 ± 1.9 POD (P < .001). Nine patients were ascites culture positive; long-term placement of the drain caused an infection in two patients. CONCLUSIONS: When the amount of ascitic fluid on the fifth POD after LDLT was small, it was important to assess the properties of the ascitic fluid because of the possibility of a drain infection or of poor drainage. If the ascitic neutrophil count is less than 250/mm(3) or the examined ascites is normal, intra-abdominal drains should be removed.

Recent studies suggest that the overall survival and risk of end-stage renal disease among renal transplant donors are similar to those of the general population, but few studies focused on elderly donors. Among 88 donors who underwent retroperitoneoscopic live donor nephrectomies; 20 (22.7%) were elderly, namely, older than 65 years. Perioperative characteristics, such as sex, donor kidney side (left or right), body mass index, operative time, blood loss, and complication rate were not significantly different among groups classified by age: young (<50), middle (50-65), or elderly (>65). One month after kidney donation, the serum, creatinine values in the young, middle, and elderly groups increased to 1.05 ± 0.25, 0.96 ± 0.24, and 1.06 ± 0.15 mg/dL (P = .103) and the estimated glomerular filtration rate (eGFR) decrease to 63 ± 10, 63 ± 14, 56 ± 8 mL/min/1.73 m(2), respectively (P = .037). At three months and at three years after donation these parameters showed the same degree of improvement in all groups. Percentage of eGFR (% eGFR) of its pre-donation value in the young and middle groups improved up to 21% and up to 9%, respectively, until four years after donation, whereas that of the elderly group remained unchanged below 1%. In conclusion residual renal function after retroperitoneoscopic kidney donation in elderly donors was stable and acceptable during mid-term observation. Our retroperitoneoscopic approach was safe.

The prognosis of liver transplantation for neonates with fulminant hepatic failure (FHF) continues to be extremely poor, especially in patients whose body weight is less than 3 kg. To address this problem, we have developed a safe living donor liver transplantation (LDLT) modality for neonates. We performed LDLTs with segment 2 monosubsegment (S2) grafts for three neonatal FHF. The recipient age and body weight at LDLT were 13-27 days, 2.59-2.84 kg, respectively. S2 or reduced S2 grafts (93-98 g) obtained from their fathers were implanted using temporary portacaval shunt. The recipient portal vein was reconstructed at a more distal site, such as the umbilical portion, to have the graft liver move freely during hepatic artery (HA) reconstruction. The recipient operation time and bleeding were 11 h 58 min-15 h 27 min and 200-395 mL, respectively. The graft-to-recipient weight ratio was 3.3-3.8% and primary abdominal wall closure was possible in all cases. Although hepatic artery thrombosis occurred in one case, all cases survived with normal growth. Emergency LDLT with S2 grafts weighing less than 100 g can save neonates with FHF whose body weight is less than 3 kg. This LDLT modality using S2 grafts could become a new option for neonates and very small infants requiring LT.

A 35-year-old mother was scheduled to be the living donor for liver transplantation to her second son, who suffered from biliary atresia complicated with biliary cirrhosis at the age of 2 years. The operative plan was to recover the left lateral segment of the mother's liver for living donor transplantation. With the use of cholangiography at the time of surgery, we found the right anterior segmental duct (RASD) emptying directly into the cystic duct, and the catheter passed into the RASD. After repairing the incision in the cystic duct, transplantation was successfully performed. Her postoperative course was uneventful. Biliary anatomical variations were frequently encountered, however, this variation has very rarely been reported. If the RASD was divided, the repair would be very difficult because the duct will not dilate sufficiently in an otherwise healthy donor. Meticulous preoperative evaluation of the living donor's biliary anatomy, especially using magnetic resonance cholangiography and careful intraoperative techniques, is important to prevent bile duct injury and avoid the risk to the healthy donor.

OBJECTIVES: Recent studies demonstrated that prolactin (PRL) has beneficial effects on beta cells for islet transplantation. We examined the effect of human recombinant PRL (rhPRL) supplementation to the culture media to determine its potential use in the context of clinical islet transplantation. MATERIALS AND METHODS: Each human islet isolated from 14 deceased multiorgan donors was cultured in Miami modified media-1 supplemented with or without rhPRL (500 microg/L) for 48 hr. beta-Cell survival and proliferation (BrdU and Ki-67) were determined by laser scanning cytometry. The cytoprotective effects of rhPRL against noxious stimuli were assessed by flow cytometry (tetramethylrhodamine ethyl ester). Cytokine/chemokine and tissue factor productions were measured in vitro, and islet potency was assessed in vivo in diabetic immunodeficient mice. RESULTS: beta-Cell survival during culture was 37% higher in the rhPRL group than in control (P=0.029). rhPRL protected beta cells in vitro from cytokines, Nitric oxide donor, and H2O2. The exposure to rhPRL did not affect human beta-cell proliferation with our protocol. rhPRL treatment did not alter cytokine/chemokine and tissue factor production in vitro or affected human islet functionality in vivo: recipient mice achieved normoglycemia with a comparable tempo, whereas loss of graft function was observed in two of the seven mice in the control group and in none of the rhPRL group (p=n.s.). CONCLUSION: rhPRL supplementation to islet culture media improved human beta-cell-specific survival without altering islet quality. Addition of rhPRL to cultured islets may grant a more viable beta-cell mass in culture. The development of beta-cell cytoprotective strategies will be of assistance in improving islet transplantation outcomes.

8歳女児。胆道閉鎖症術後胆汁うっ滞性肝硬変に対し、1歳時に父親をドナーとする生体肝移植術を施行した。術後経過は良好で、第65病日に退院となった。外来フォロー中に門脈吻合部の狭窄を合併し、2歳時と4歳時にバルーン拡張術を施行した。しかし、8歳頃から再々狭窄を認めるようになった。全身麻酔下にバルーン拡張術を施行し、門脈の狭窄を解除後、プロトコール肝生検を超音波ガイド下に実施した。手技中のトラブルはなかった。また、術直前と術後8時間時に、予防的に抗生を投与した。40℃台の発熱が出現し、肝胆道系酵素の上昇を認めた。敗血症と診断し抗生剤、γグロブリン製剤の投与を開始した。敗血症の原因は肝生検による胆管炎が考えられた。術後3日目には解熱傾向に転じ、炎症反応や黄疸の改善もみられ、その後は速やかに状態の改善を得て、術後10日目に退院となった。

Portal vein complications after liver transplantation (LT) are serious complications that can lead to graft liver failure. Although the treatment of interventional radiology (IVR) by means of balloon dilatation for portal vein stenosis (PVS) after LT is an effective method, the high rate of recurrent PVS is an agonizing problem. Anticoagulant therapy for PVS is an important factor for preventing short-term recurrence following IVR, but no established regimen has been reported for the prevention of recurrent PVS following IVR. In our population of 197 pediatric patients who underwent living donor liver transplantation (LDLT), 22 patients (22/197, 11.2%) suffered PVS. In the 9 earliest patients, unfractionated heparin was the only anticoagulant therapy given following IVR. In the 13 more recent patients, 3-agent anticoagulant therapy using low-molecular-weight heparin, warfarin, and aspirin was employed. In the initial group of 9 patients, 5 patients (55.6%) suffered recurrent PVS and required repeat balloon dilatation. Among the 13 more recent patients, none experienced recurrent PVS (P = 0.002). In conclusion, our 3-agent anticoagulant therapy following IVR for PVS in pediatric LDLT can be an effective therapeutic strategy for preventing recurrent PVS.

CONTEXT: Although surgical resection is the only curative therapeutic option for recurrent or metachronous pancreatic carcinomas, most such cancers are beyond surgical curability. We herein report on two rare cases of remnant pancreatectomy used to treat recurrent or metachronous pancreatic carcinomas. CASE REPORTS: CASE#1 A 65-year-old male developed weight loss and diabetes mellitus 83 months after a pylorus-preserving pancreaticoduodenectomy followed by two years of adjuvant chemotherapy (5-fluorouracil plus leucovorin plus mitomycin C) for a pancreatic carcinoma in the head of the pancreas (stage IA). An abdominal CT scan revealed a 3 cm tumor in the remnant pancreas which appeared as a 'hot' nodule on FDG-PET. A remnant distal pancreatectomy was performed and a pancreatic carcinoma similar in profile to the primary lesion (stage IIB) was confirmed pathologically. CASE#2 A 67-year-old male showed increased CA 19-9 levels 25 months after a distal pancreatectomy for a pancreatic carcinoma in the body of the pancreas (stage IA). An abdominal CT scan revealed a cystic lesion in the cut end of the pancreas which appeared as a 'hot' nodule on FDG-PET. A remnant proximal pancreatectomy with duodenectomy was performed and a metachronous pancreatic carcinoma (stage III) was confirmed pathologically. CONCLUSION: Remnant pancreatectomy can be considered a treatment option for recurrent or metachronous pancreatic carcinomas. FDG-PET can play a key role in detecting remnant pancreatic carcinomas.

A 70-year-old female with a long history of progressive leukocytopenia and giant splenomegaly is described. She had no clinically manifested rheumatoid arthritis, although she complained of slight arthralgia in the digital joints, wrists and ankles at irregular intervals. Repeated bone marrow aspirations showed no cellular atypism, chromosomal abnormalities, or phenotypical abnormalities. Just before splenectomy, both anti-neutrophil antibody positivity and anti-cyclic citrullinated peptide antibody positivity were shown. Histology of the splenectomized spleen showed follicular hyperplasia with plasmacyte infiltration and extramedullary hematopoeisis. After splenectomy, leukocyte counts returned to normal with normal leukocyte differentials and anti-neutrophil antibodies disappeared. She was almost free of arthralgia one year after splenectomy, although the anti-cyclic citrullinated peptide antibody titers remained high.

The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.

Portal vein stenosis (PVS) after living donor liver transplantation (LDLT) is a serious complication that can lead to graft failure. Few studies of the diagnosis and treatment of late-onset (> or = 3 months after liver transplantation) PVS have been reported. One hundred thirty-three pediatric (median age 7.6 years, range 1.3-26.8 years) LDLT recipients were studied. The patients were followed by Doppler ultrasound (every 3 months) and multidetector helical computed tomography (once a year). Twelve patients were diagnosed with late-onset PVS 0.5-6.9 years after LDLT. All cases were successfully treated with balloon dilatation. Five cases required multiple treatments. Early diagnosis of late-onset PVS and interventional radiology therapy treatment may prevent graft loss.