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There is no consensus about long-term outcomes in patients with biliary atresia. We retrospectively reviewed the long-term outcomes in pediatric patients who underwent living donor liver transplantation for biliary atresia.Between May 2001 and December 2020, 221 (73%) of 302 pediatric patients who underwent living donor liver transplantation had biliary atresia. The median age at living donor liver transplantation was 1.2 (range 0.2-16.5) years, and follow-up was 10.3 ± 5.5 years.The 10-year graft survival rates in patients with and without biliary atresia were 94% and 89%, respectively (P = .019). The 10-year graft survival was significantly poorer in patients ≥12 years of age (84%) versus those12 years of age at living donor liver transplantation (0-2 years: 95%; 2-12 years: 96%) (P = .016). The causes of graft failure in patients with biliary atresia included late-onset refractory rejection (n = 6), bowel perforation (n = 2), and acute encephalitis (n = 2), as well as cerebral hemorrhage, hepatic vein thrombosis, and sepsis (n = 1 for all). All 7 patients with graft failure due to refractory rejection and hepatic vein thrombosis underwent repeated liver transplantation and are alive in 2021. The rates of post-transplant portal vein complications and early-onset acute cellular rejection in patients with biliary atresia were higher than in those without biliary atresia (P = .042 and P = .022, respectively). In 2021, of 60 adolescents with biliary atresia, 14 (23%) reported medication nonadherence. The rate of liver dysfunction due to late-onset acute cellular rejection and graft failure due to late-onset refractory rejection in patients with nonadherence was higher than in patients with satisfactory adherence (P = .009).The long-term prognosis after living donor liver transplantation in pediatric patients with biliary atresia is quite good. However, long-term support to enhance medication adherence is required in adolescents with biliary atresia.

Background Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. Case Report We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing living-donor liver transplant, the patient showed no recurrence of peliosis hepatis. Conclusions Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

Graft inflow modulation (GIM) during adult-to-adult living donor liver transplantation (LDLT) is a common strategy to avoid small-for-size syndrome, and some transplant surgeons attempt small size graft strategy with frequent GIM procedures, which are mostly performed by splenectomy, in LDLT. However, splenectomy can cause serious complications such as portal vein thrombosis and overwhelming postsplenectomy infection.Forty-eight adult-to-adult LDLT recipients were enrolled in this study and retrospectively reviewed. We applied the graft selection criteria, which routinely fulfill graft-to-recipient weight ratio ≥ 0.8%, and consider GIM as a backup strategy for high portal venous pressure (PVP).In our current strategy of LDLT, splenectomy was performed mostly due to hepatitis C and splenic arterial aneurysms, but splenectomy for GIM was intended to only one patient (2.1%). The final PVP values ≤ 20 mmHg were achieved in all recipients, and no significant difference was observed in patient survival or postoperative clinical course based on whether splenectomy was performed or not. However, 6 of 18 patients with splenectomy (33.3%) developed postsplenectomy portal vein thrombosis (PVT), while none of the 30 patients without splenectomy developed PVT after LDLT. Splenectomy was identified as a risk factor of PVT in this study (P0.001). Our study revealed that a lower final PVP could be risk factor of postsplenectomy PVT.Using sufficient size grafts was one of the direct solutions to control PVP, and allowed GIM to be reserved as a backup procedure. Splenectomy should be avoided as much as possible during LDLT because splenectomy was found to be a definite risk factor of PVT. In splenectomy cases with a lower final PVP, a close follow-up is required for early detection and treatment of PVT.

Background Aspartate transaminase–to–platelet ratio index (APRI) and fibrosis-4 (FIB-4) are well known as representative indirect serum biomarkers related to liver fibrosis. The usefulness of these markers for the diagnosis of liver fibrosis after liver transplantation (LT) in hepatitis C virus (HCV)–infected patients and the influence of splenectomy were investigated. Methods From June 2003 to May 2014, 31 HCV-infected patients who underwent LT and postoperative follow-up liver biopsies were included in this study. The association between liver fibrosis and serum biomarkers and the influence of splenectomy on APRI and FIB-4 were also investigated. Results A total of 195 biopsy specimens were collected, and liver fibrosis was identified as: F0, 59.7%; F1, 34.1%; and F2, 6.3%. Both APRI and FIB-4 were significantly higher in patients who showed F1 and F2 in liver biopsy specimen than F0 (P values, .009 and .022, respectively); sensitivity and specificity of APRI were, respectively, 63.4% and 66.7%, and those of FIB-4 were 57.7% and 69.6%. In 11 patients (35.5%) who underwent splenectomy at the time of LT, the cutoff values for APRI and FIB-4 were 0.61 and 1.41, which were significantly lower than the corresponding values (1.00 and 3.64) of patients without splenectomy. Conclusions APRI and FIB-4 could effectively estimate liver fibrosis after LT for HCV-related liver disease. For LT patients with splenectomy, APRI and FIB-4 were also useful to estimate liver fibrosis, but the standard values should be adjusted lower than those for patients without splenectomy.

Highlights • We report a case in which a brain death donor, supported by a left ventricular assist device, with significant congestive fibrosis of the liver. • This case serves as a note of caution when addressing organ procurement from brain death donor with left ventricular assist device. • Exposure of the surgical fields was restricted by the device and drive lines. • It also should be noted that chronic heart failure can lead to subclinical advanced liver fibrosis., Instruction Organ transplantation from a brain death donor on mechanical circulatory support is rare. We report a case in which a brain death donor, supported by a left ventricular assist device (LVAD), unexpectedly displayed significant congestive fibrosis of the liver. Presentation of case The potential organ donor was diagnosed 23 years previously as having dilated-phase of hypertrophic cardiomyopathy. He had undergone implantation of an LVAD as a bridge to heart transplantation. Laboratory tests and imaging studies performed during the follow-up for his cardiac disease and donor evaluation confirmed that he was suitable for donation of liver. During organ procurement, special attention was paid to preserving LVAD and its device’s drive lines and the exposure of the surgical fields was restricted by those devices. Thoracotomy and laparotomy were performed, and the aorta and inferior vena cava were encircled successfully. The gross appearance of liver, however, suggested significant fibrosis. Therefore, the decision was made not to use this liver. Subsequent trichrome-stained permanent sections revealed advanced fibrosis (stage F3–4). Discussion As previously reported, organ procurement from donors with LVAD was thought to be demanding procedure because of the limited exposure of surgical field. In addition, it would be difficult to predict severe liver fibrosis in patients with an LVAD without a pathological examination. Conclusion Donors with mechanical circulatory support systems can be candidate to expand the donor pool, but technical difficulty should be expected owing limited exposure during the donor operation. For liver transplantation, subclinical advanced liver fibrosis should be noted.

A 39-year-old man was diagnosed with allopurinol-induced hepatic injury. He did not show any sign of hepatic encephalopathy, but his serum total bilirubin level was >40 mg/dL when he visited the local hospital. The therapeutic effects of initial medical treatments were transient, and both renal function and coagulation ability were gradually deteriorated. Four months after the onset of hepatic injury, he was referred to our hospital for the purpose of liver transplantation (LT). Although he was wasting and severely jaundiced, his consciousness level was not disturbed at all, with normal serum ammonia blood concentration before LT. Owing to allopurinol-induced severe cholestatic liver failure, living-donor LT (LDLT) was performed with the use of a right lobe graft from his younger brother. The explanted liver was extremely enlarged, with a weight of 2,480 g, and severely cholestatic. Microscopic findings were also compatible with drug-induced cholestatic liver injury. He was discharged from hospital 55 days after LDLT, whereas his renal dysfunction remained at 6 months after LT. There are 3 types of pathophysiology of drug-induced hepatotoxicity: hepatocellular, cholestatic, and mixed liver injury. Although allopurinol hepatotoxicity is rare, it can be severe and even fatal. This is the 1st case report of successful LDLT for a patient who had developed allopurinol-induced cholestatic liver failure.

Background There are few reports on the short- and long-term follow-up of endoscopic retrograde cholangiography (ERC) in adult patients with hepaticojejunostomy (HJS) stricture after living-donor liver transplantation (LDLT). Methods Nine LDLT recipients underwent ERC with the use of double-balloon endoscopy (DBE) for HJS stricture at Nagoya University Hospital. We assessed the rate of reaching biliary anastomosis, procedure success rate, procedure duration, complications, improvement in liver function test results, and biliary anastomosis patency. Results In total, 19 ERC procedures with the use of DBE were performed for 9 adult LDLT recipients with HJS stricture from June 2006 to September 2014. Balloon dilation with the use of DBE was successfully performed in 5 of the 9 patients during the 1st procedure. Of the 4 patients in whom DBE-ERC failed to be completed, 3 patients underwent 2nd procedures successfully. Liver function test results were significantly improved in the successful cases. Four patients underwent 2nd DBE-ERC for stricture recurrence at a mean time of 2.3 years after the 1st successful procedure. Of those, 2 patients required 3rd procedures for stricture recurrence after the 2nd procedure. Conclusions DBE-ERC is promising as a treatment for HJS stricture in adult LDLT recipients in the short term. However, the DBE-ERC procedure may have a considerable risk of restenosis.

Highlights • Donor organ scarcity is the major problem in the field of liver transplantation. • Extended criteria donor for living donor liver transplantation (LDLT) is crucial. • This is the first adult-to-adult LDLT using liver allograft with benign hemangioma. • Liver allograft with hemangioma is a remarkable strategy to resolve organ shortage. • This case is so suggestive for a safe expansion of living donor pool in LDLT., Introduction Hepatic hemangioma is one of the most common benign liver tumors. There are few published reports regarding liver transplantation using liver allografts with hemangioma. Presentation of case A 45-year-old man was evaluated as a living donor for 19-year-old son with cirrhosis due to hepatic fibrosis. Preoperative investigations revealed 20 and 7 mm hemangiomas, at segment 2 (S2) and 4 (S4) respectively. Considering the anatomical relation of S2 hemangioma and Glisson 2, liver graft was designed as left lobe excluded S2 hemangioma by partial resection. Estimated graft recipient weight ratio (GRWR) even after partial resection of hemangioma was reasonable. During the donor operation, a partial hepatic resection of S2 hemangioma was performed. Intraoperative pathologic findings revealed a cavernous hemangioma, and then, the left hepatic graft with the caudate lobe was harvested. Actual GRWR was 0.90%. Donor's postoperative course was uneventful. Recipient’s post-operative course was almost uneventful. Postoperative computed tomography of the recipient showed the graft regeneration without increase or recurrence of hemangioma. Discussion Organ shortage is a major concern in the field of liver transplantation. A novel donor source with a further option is extremely crucial for a guarantee of liver transplantation. We experienced the first case of adult-to-adult living donor liver transplantation using liver allograft after the resection of hemangioma. Conclusion We advocate that the use of liver allograft with hemangiomas in adult-to-adult LDLT settings can be remarkable strategy to reduce the problem of organ shortage without any unfavorable consequences in both living donor and recipient.

Living donor liver transplantation (LDLT) using a right liver graft with additional vein reconstructions has not been previously reported in a situs inversus (SI) patient. A 60-year-old man with SI was referred for LDLT for end-stage cirrhosis secondary to hepatitis B. The calculated regional volumes of the individual hepatic vein territories in the right liver graft suggested that the middle hepatic vein (MHV) tributaries and the inferior right hepatic veins (IRHVs) should be reconstructed in addition to the right hepatic vein (RHV). On the back-table, the recipient's recanalized umbilical vein graft was anastomosed to the V5 opening, and the other side of vein graft was anastomosed to the RHV and V8 opening to create a large single orifice. After total hepatectomy, the right liver graft was placed in the left subphrenic space at the reversed position. The common orifice of hepatic venous drainage from RHV, V8 and V5 was anastomosed to the anatomical RHV conduit of the recipient, followed by IRHV anastomosis to the inferior vena cava. Postoperative course was almost uneventful, and no vascular complications were experienced. Even for SI patients, LDLT using a right liver graft with reconstructions of the MHV tributaries and the IRHVs is feasible.

Objective To elucidate the effect of liver transplantation (LT) on brain dysfunctions in cirrhotic patients who had no clinical evidence of hepatic encephalopathy (HE), we performed a prospective study of voxel-based diffusion tensor imaging (DTI) and detailed cognitive examination. Methods We assessed 12 consecutive patients as transplant candidates by DTI, with neurological and cognitive examinations just before and at 6 months after LT. Results After LT, cirrhotic patients showed significant improvement in visual reproduction, digit symbol, digit span, Stroop test, and Trail-making test scores, suggesting recovery of frontal-temporal function. As for voxel-based DTI, increased mean diffusivity (MD) and reduced fractional anisotropy (FA) values were found before LT in the frontal and temporal lobes of cirrhotic patients. After LT, the unusual FA and MD values observed in the frontal and temporal lobes preoperatively were significantly reduced. Conclusion End-stage cirrhotic patients without clinical evidence of HE showed increased MD and decreased FA values in both frontal and temporal lobes. These parameters improved after LT, in line with cognitive function. MD and FA values might be of value as a biomarker in end-stage cirrhotic patients for investigating brain tissue dysfunctions and evaluating the efficacy of LT.