The impact of rituximab in ABO-incompatible pediatric living donor liver transplantation: the experience of a single center.

Previous studies have demonstrated the safety of ABO-incompatible pediatric LDLT using preoperative plasmapheresis and rituximab; however, no reports have described the timing and dosage of rituximab administration for pediatric LDLT. This study aimed to describe a safe and effective dosage and timing of rituximab for patients undergoing pediatric ABO-incompatible LDLT based on the experience of our single center. A total of 192 LDLTs in 187 patients were examined. These cases included 29 ABO-incompatible LDLTs in 28 patients. Rituximab was used beginning in January 2004 in recipients older than two yr of age (first period: 375 mg/m(2) in two cases; second period: 50 mg/m(2) in two cases; and 200 mg/m(2) in eight cases). Two patients who received 375 mg/m(2) rituximab died of Pneumocystis carinii pneumonia and hemophagocytic syndrome. One patient who received 50 mg/m(2) rituximab required retransplantation as a consequence of antibody-mediated complications. All eight patients administered 200 mg/m(2) survived, and the mean CD20(+) lymphocyte count was 0.1% at the time of LDLT. In the preoperative management of patients undergoing pediatric ABO-incompatible LDLT, the administration of 200 mg/m(2) rituximab three wk prior to LDLT was safe and effective.

Living donor liver transplantation from an asymptomatic donor with mild coagulation factor IX deficiency: report of a case.

The use of donors with coagulation FIX deficiency is controversial, and there are no current protocols for peri-transplant management. We herein describe the first reported case of a pediatric LDLT from an asymptomatic donor with mild coagulation FIX deficiency. A 32-yr-old female was evaluated as a donor for her 12-month-old daughter with biliary atresia. The donor's pretransplant coagulation tests revealed asymptomatic mild coagulation FIX deficiency (FIX activity 60.8%). Freeze-dried human blood coagulation FIX concentrate was administered before the dissection of the liver and 12 h afterwards by bolus infusion (40 U/kg) and was continued on POD 1. The bleeding volume at LDLT was 590 mL. On POD 1, 3, 5, and 13, the coagulation FIX activity of the donor was 121.3%, 130.6%, 114.6%, and 50.2%, respectively. The donor's post-transplant course was uneventful, and the recipient is currently doing well at 18 months after LDLT. The FIX activity of the donor and recipient at nine months after LDLT was 39.2% and 58.0%, respectively. LDLT from donors with mild coagulation FIX deficiency could be performed effectively and safely using peri-transplant short-term coagulation FIX replacement and long-term monitoring of the plasma FIX level in the donor.

Double-balloon enteroscopy for bilioenteric anastomotic stricture after pediatric living donor liver transplantation.

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.

Pediatric living donor liver transplantation for biliary atresia with hepatopulmonary syndrome: the gift of a second wind.

PURPOSE: Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13-47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe hypoxemia before transplantation, but the course of HPS after living donor liver transplantation (LDLT), especially for biliary atresia (BA) patients is not well established. PATIENTS AND METHODS: The present study evaluated 122 patients who received an LDLT for BA and of these, 3 patients had HPS at the time of LDLT in a single-center series. RESULTS: Two patients of the HPS patients them had biliary and/or vascular complications, but they recovered uneventfully with interventional treatment. None of the patients required supplemental oxygen and had no residual cardiopulmonary abnormalities at a follow-up of more than 24 months. CONCLUSION: Although a series of three patients is too small for definitive conclusion and further investigations must be conducted, pediatric LDLT can be a favorable therapeutic option for HPS.

Bowel perforation after pediatric living donor liver transplantation.

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.

Strategy to prevent recurrent portal vein stenosis following interventional radiology in pediatric liver transplantation.

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.

Diagnosis and treatment of pediatric patients with late-onset portal vein stenosis after living donor liver transplantation.

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.

Lymphocytotoxic crossmatch in pediatric living donor liver transplantation.

To investigate the relationship between the pretransplant LCT results and the outcome after pediatric LDLT in a single center. The clinical data of 76 children undergoing 79 LDLTs including three retransplantations from May 2001 to January 2006 were retrospectively analyzed. All of the children had end-stage liver disease, and their median age was 1.4 yr (range, six months to 16.5 yr). Immunosuppressive therapy consisted of cyclosporine- or FK-based regimens with steroids. The children were classified into two groups (positive or negative) according to the pretransplant LCT results. The incidences of post-transplant surgical complications and of rejection episodes were compared. The relationship between the pretransplant LCT results and patient and graft survival rates was also analyzed. Seventy-nine pretransplant crossmatch tests were done; 13 (16.5%) were positive, and 66 (83.5%) were negative. No significant difference was found in the pretransplant clinical factors between two crossmatch groups. There was no significant difference between the groups in the incidence of vascular and biliary tract complications, in the rate of early or steroid-resistant cellular rejections, or in one- and three-yr patient (91.7%, 91.7%, respectively, in the positive group, 93.5%, 93.5%, respectively, in the negative group, p = 0.80) and graft (92.3%, 92.3%, respectively, in the positive group, 88.8%, 86.4%, respectively, in the negative group, p = 0.63) survival. The present study demonstrates that there is no reason to do pretransplant LCT to select the living donor for pediatric LDLT.

Retroperitoneoscopic hand-assisted live-donor nephrectomy according to the basic principle of transplantation in donor kidney selection.

PURPOSE: We assessed the feasibility of retroperitoneoscopic hand-assisted live-donor nephrectomy according to the basic principle of transplantation in kidney selection, namely, leaving the better-functioning kidney in the donor. PATIENTS AND METHODS: Thirty consecutive live-donor nephrectomies, including 10 right-sided and 20 left-sided procedures, were evaluated. The surgery was started endoscopically using three ports, followed by hand assistance for dissecting the renal pedicles through the extended inner-port incision. A vascular Endostapler and polymer clips were used to transect the renal vessels. RESULTS: Two right-sided cases required open conversion because of multiple renal vessels and uncontrollable bleeding. The median operative time, warm ischemia time (WIT), blood loss, and renal vein length were 244 minutes (upper and lower quartile 215 and 274 minutes), 186 seconds (134, 239 seconds), 175 mL (45, 305 mL), and 22 mm (19, 26 mm), respectively. The operative time and WIT were longer, and the renal vein was shorter, in the right-sided than in the left-sided procedures (P < 0.05), but no difference was found in the other perioperative data for the two sides. No delayed graft function was observed, and the kidney function 1 month postoperatively was acceptable in all donors and all recipients. CONCLUSION: Our technical devices, such as the site and timing of hand assistance and control of the renal vessels, seem feasible. Although we could not draw a conclusion about the safety of the right-sided procedure, this alternative procedure should be applicable for laparoscopic donor nephrectomy uninfluenced by the side of the donor kidney provided the surgical team has sufficient expertise.

Recurrent cholangitis by biliary stasis due to non-obstructive afferent loop syndrome after pylorus-preserving pancreatoduodenectomy: report of a case.

We report a 71-year-old man who had undergone pylorus-preserving pancreatoduodenectomy (PPPD) using PPPD-IV reconstruction for cholangiocarcinoma. For 6 years thereafter, he had suffered recurrent cholangitis, and also a right liver abscess (S5/8), which required percutaneous drainage at 9 years after PPPD. At 16 years after PPPD, he had been admitted to the other hospital because of acute purulent cholangitis. Although medical treatment resolved the cholangitis, the patient was referred to our hospital because of dilatation of the intrahepatic biliary duct (B2). Peroral double-balloon enteroscopy revealed that the diameter of the hepaticojejunostomy anastomosis was 12 mm, and cholangiography detected intrahepatic stones. Lithotripsy was performed using a basket catheter. At 1 year after lithotripsy procedure, the patient is doing well. Hepatobiliary scintigraphy at 60 minutes after intravenous injection demonstrated that deposit of the tracer still remained in the upper afferent loop jejunum. Therefore, we considered that the recurrent cholangitis, liver abscess, and intrahepatic lithiasis have been caused by biliary stasis due to nonobstructive afferent loop syndrome. Biliary retention due to nonobstructive afferent loop syndrome may cause recurrent cholangitis or liver abscess after hepaticojejunostomy, and double-balloon enteroscopy and hepatobiliary scintigraphy are useful for the diagnosis of nonobstructive afferent loop syndrome.

Participation of surgical residents does not adversely affect the outcome of inguinal hernia repair in an integrated teaching program.

OBJECTIVE: The Lichtenstein inguinal hernia repair is commonly performed and suitable for teaching basic surgical skills. The objective of this study is to evaluate the feasibility of this procedure for surgical training, particularly in regard to patient outcomes. DESIGN: Retrospective case review after introduction of an integrated teaching program. SETTING: University teaching hospital. PARTICIPANTS: The Lichtenstein inguinal hernia repair is the standard procedure for adult primary unilateral inguinal hernia since 2003 at Jichi Medical University. We introduced an integrated teaching system of lectures, skill training. and videos to teach the skills for Lichtenstein inguinal hernia repair to residents and junior faculty in 2003. Cases were retrospectively divided into 4 groups based on the experience of the operating surgeon; junior residents (PGY 1-2, group A), senior residents (PGY 3-5, group B), junior faculty (PGY 6-10, group C), and senior faculty (PGY 11 or more, group D). Background, perioperative factors, and outcomes were evaluated among the groups. RESULTS: A total of 246 elective inguinal hernia repairs (group A: 136, group B: 49, group C: 42, group D: 19) were performed. There was a significant difference in the frequency of concomitant diseases (p = 0.012) and anticoagulant therapy (p = 0.031). Average operating time was 80.7 ± 24.9, 72.6 ± 20.8, 63.5 ± 22.0, and 54.7 ± 27.9 (min ± SD) in groups A, B, C, and D, respectively, with a significant difference between groups A and D (p < 0.001). No significant differences were observed in estimated blood loss (p = 0.216) or morbidity (p = 0.294). CONCLUSIONS: The Lichtenstein inguinal hernia repair can be safely performed by residents and junior faculty with the appropriate supervision of senior faculty without any disadvantage to patients. This integrated teaching program for Lichtenstein inguinal hernia repair is effective and feasible for training residents and junior faculty.

Carcinoid tumor of the gallbladder: report of two cases.

We report two cases of carcinoid tumor of the gallbladder. Case 1 was a 59-year-old woman who presented with epigastric pain. Abdominal ultrasonography and computed tomography (CT) revealed a 16 mm polypoid lesion in the neck of the gallbladder. Tumor markers were within normal limits. Open cholecystectomy was performed with a preoperative diagnosis of early cancer of the gallbladder. Case 2 was a 45-year-old man. A polyp in the gallbladder was incidentally detected on annual checkup. Ultrasound and CT showed an 18 mm protruding lesion in the neck of the gallbladder. Laparoscopic cholecystectomy was performed and the tumor diagnosed as a carcinoid tumor based on the findings of funicular and tubular cells in the lamina propria mucosa, homogeneous nuclei, basophilic cytoplasm, and positive staining with chromogranin A and synaptophysin. The postoperative course of both patients was uneventful, with no recurrence at 44 and 41 months after surgery. In this literature review of 39 cases, classical carcinoid of the gallbladder has a favorable postoperative outcome. Of cases reviewed, 60% are located in the neck of the gallbladder and 50% have a polypoid shape.

Pediatric living donor liver transplantation using liver allograft with hemangioma.

BACKGROUND: Although liver transplantation using liver allograft with hemangiomas has been previously reported, little is known about the fate of hemangiomas in the transplanted liver. We herein describe a case of pediatric living donor liver transplantation (LDLT) using living donor liver allograft with a hemangioma which is considered to the first reported case performing in vivo hemangioma resection. CASE REPORT: A 27-year-old female was evaluated as a donor for her 2-year-old son with cholestatic cirrhosis due to biliary atresia. Preoperative ultrasonography and computed tomography revealed a 20-mm hemangioma located at lateral side of segment 3. During LDLT, an in vivo partial hepatic resection of the hemangioma of segment 3 was performed without the Pringle maneuver using intraoperative ultrasonography to keep the main portal triad of segment 3 before the donor liver resection, and the left lateral segment graft without the hemangioma, which underwent an intraoperative pathologic diagnosis, was transplanted into the recipient. The donor's postoperative course was uneventful and the recipient course was not observed subsequent liver necrosis, bleeding or bile leakage from the resection site. CONCLUSIONS: Liver allografts with hemangiomas can be accepted as potential liver allografts, and such hemangiomas should undergo be performed in vivo resection during LDLT irrespective of tumor size.

Right anterior segmental hepatic duct emptying directly into the cystic duct in a living donor.

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.

Growth curves of pediatric patients with biliary atresia following living donor liver transplantation: factors that influence post-transplantation growth.

We evaluated the growth curves of children with BA after LDLT, and identified factors influencing growth velocity one-yr after LDLT (DeltaZ). The clinical data of 51 children with BA, who had an LDLT at our center from 2001 to 2005, were retrospectively reviewed. The Z scores for height and weight, and DeltaZ were studied. The correlation between DeltaZ and various clinical factors was evaluated statistically. Multivariate stepwise analyses were performed for DeltaZ. The average height and weight Z scores at the time of LDLT were -1.34 +/- 1.36 (+/-s.d.) and -0.78 +/- 1.15, respectively. Among 30 BA recipients with stable liver function after transplant, weight returned to normal one-yr post-transplantation. However, height did not return to normal even by the third post-transplantation year. On multivariate analyses, 73% of the variance in height DeltaZ could be accounted for by factors such as standardized height at the time of LDLT (proportion of variance: 38%), number of steroid pulse treatments (17%), donor age (10%), and the presence of HVS (9%). Fifty-four percentage of the variance in weight DeltaZ could be accounted for by factors such as standardized weight at the time of LDLT (37%) and the total steroid dose given (17%). Height and weight status at the time of LDLT likely have the strongest impact on DeltaZ. Additional factors include steroid exposure, age of the living donor, and presence of HVS, all of which should be considered to improve post-transplantation growth.

Successful pediatric living donor liver transplantation from carrier to carrier of hereditary butyrylcholinesterase variant.

Hypocholinesterasemia is often observed clinically, especially in various liver diseases. Not well known, however, is the fact that some patients have a hereditary BChE variant. Little has been reported on liver transplants associated with this hereditary BChE variant. Furthermore, no cases have been reported of a LDLT involving hereditary BChE variant that had been diagnosed preoperatively. A 23-month-old girl who had had a failed Kasai operation for biliary atresia underwent a liver transplant using as a graft her father's lateral segment. Preoperatively, she had been diagnosed with hypocholinesterasemia. As the donor, her father had undergone a preoperative examination, during which he was found to also have hypocholinesterasemia. DNA sequencing revealed that both had the hereditary BChE variant. The unique mutation caused a frame-shift mutation. Variant K was also detected. The patient was discharged 143 days after the operation and has had no problems with immunosuppression since. In conclusion, we report that the hereditary BChE variant is not a contraindication for either transplantation or living liver donation.