Satoshi Egami

A case of successful enteroscopic balloon dilation for late anastomotic stricture of choledochojejunostomy after living donor liver transplantation.

Biliary complications remain a major concern after living donor liver transplantation. We describe a pediatric case who underwent a successful endoscopic balloon dilatation of biliary‐enteric stricture following living donor liver transplantation using a newly developed method of enteroscopy. The 7‐year‐old boy with late biliary stricture of choledochojejunostomy was admitted 6 years after transplantation. Since percutaneous transhepatic cholangiography was technically difficult in this case, endoscopic retrograde cholangiography was performed using a double‐balloon enteroscope under general anesthesia. The enteroscope was advanced retrograde through the duodenum, jejunum, and the leg of Roux‐Y by the double‐balloon method, and anastomotic stricture of choledochojejunostomy was clearly confirmed by endoscopic retrograde cholangiography and endoscopic direct vision. Balloon dilatation was performed and the anastomosis was expanded. Restenosis was not noted as of 2 years after the treatment. In conclusion, endoscopic balloon dilation of biliary‐enteric anastomotic stricture using a new enteroscopic method can be regarded as an alternative choice to percutaneous transhepatic management and surgical re‐anatomists. (Liver Transpl 2005;11:1608–1610.)

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 (≥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.

Rendezvous penetration method using double-balloon endoscopy for complete anastomosis obstruction of hepaticojejunostomy after pediatric living donor liver transplantation

A 12-year-old boy underwent a living donor liver transplantation at another facility in March 2006 after undergoing a failed Kasai operation. The left lobe from his mother was used for the graft with a single-orifice bile duct 4 mm in diameter. The biliary reconstruction was performed via a Roux-en-Y hepaticojejunostomy with an interrupted 6-0 absorbable monofilament suture material without a biliary stent. The patient was treated with tacrolimusand methylprednisolone-based immunosuppression. The postoperative course was uneventful, except for an episode of postoperative diabetes requiring a subcutaneous insulin injection. In September 2006, he was referred to our department because of intrahepatic bile duct dilatation. Image findings and laboratory data revealed biliary stricture with liver dysfunction. A percutaneous transhepatic cholangiodrainage (PTCD) was performed, with placement of a 7-French PTCD tube. Complete obstruction of the anastomosis was observed on cholangiography 2 weeks later. The PTCD tube was changed from a 7-French tube to a 9-French tube in order to observe the anastomosis with a 2.8-mm-diameter cholangioscope (CHF-CB30S, Olympus, Tokyo, Japan). This proved ineffective, however, as the guide wire could not be passed through the anastomosis. Because in our experience double-balloon endoscopy (DBE) can reveal the outline of a complete obstruction of hepaticojejunostomy, we were compelled to apply the rendezvous penetration method using DBE. RENDEZVOUS PENETRATION METHOD USING DBE

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.

Living Donor Liver Transplantation for Neonates Using Segment 2 Monosubsegment Graft

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.

Living-Donor Liver Transplantation in 126 Patients with Biliary Atresia: Single-Center Experience

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.

Living donor liver transplantation for ornithine transcarbamylase deficiency

Wakiya T, Sanada Y, Mizuta K, Umehara M, Urahasi T, Egami S, Hishikawa S, Fujiwara T, Sakuma Y, Hyodo M, Murayama K, Hakamada K, Yasuda Y, Kawarasaki H. Living donor liver transplantation for ornithine transcarbamylase deficiency.
Pediatr Transplantation 2011: 15: 390–395.

Living Donor Liver Transplantation for Congenital Absence of the Portal Vein

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 patients 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.

Living donor liver transplantation from an asymptomatic mother who was a carrier for ornithine transcarbamylase deficiency

Wakiya T, Sanada Y, Urahashi T, Ihara Y, Yamada N, Okada N, Egami S, Sakamoto K, Murayama K, Hakamada K, Yasuda Y, Mizuta K. Living donor liver transplantation from an asymptomatic mother who was a carrier for ornithine transcarbamylase deficiency.

The role of operative intervention in management of congenital extrahepatic portosystemic shunt

BACKGROUND AND AIMS Congenital extrahepatic portosystemic shunt (CEPS) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. It is still a matter of debate whether conservative or operative strategies should be used to treat symptomatic CEPS. The aim of this study was to evaluate the role of operative intervention in the management of CEPS. METHODS Between June 2004 and August 2010, 6 consecutive patients with symptomatic CEPS were treated in our department. There were 3 male and 3 female patients, with a median age of 3.5 years (range, 1-8). Their demographic, clinical, and laboratory data were analyzed. All patients were scheduled to undergo shunt ligation or liver transplantation (LT). RESULTS Living donor LT was carried out in 4 patients, and shunt ligation in 2. After a median follow-up of 25 months, all the patients are alive currently with marked relief of symptoms. CONCLUSION Shunt ligation or LT for symptomatic CEPS is potentially curative.