Living-Donor Liver Transplantation for Erythropoietic Protoporphyria: A Case Report and Literature Review.

Erythropoietic protoporphyria (EPP) is a very rare disease with an estimated prevalence of 1 in 200,000 individuals. Decreased ferrochelatase activity causes the accumulation of protoporphyrin in the body, and light exposure results in the generation of active oxygen, causing photosensitivity. Liver damage has the greatest influence on the prognosis, and liver transplantation is the only treatment option for patients with decompensated liver cirrhosis. We report a case of living-donor liver transplantation for decompensated liver cirrhosis associated with EPP. The patient was a 52-year-old male who led a normal life except for mild photosensitivity. When the patient was 37-year-old, hepatic dysfunction was noticed. At 48-year-old, high erythrocyte protoporphyrin levels, skin biopsy, and genetic tests resulted in a diagnosis of EPP. The patient underwent living- donor liver transplantation because of decompensated liver cirrhosis. In the operating room and intensive care unit, a special light-shielding film was applied to all light sources to block light with harmful wavelengths during treatment. Due to the need for special measures, a lecture on patients with EPP was given before surgery to deepen understanding among all medical professionals involved in the treatment. As a result, no adverse events occurred during the perioperative period, and the patient was discharged on the 46th post-operative day. Currently, the transplanted liver is functioning extremely well, and the patient is alive 3 years post-transplant. Herein, we describe a case of living donor liver transplantation for EPP with a brief literature review.

Novel technique for recanalization of severe hepaticojejunal obstruction using a transseptal needle in a pediatric liver transplant recipient.

BACKGROUND: Endoscopic and PTB interventions are common nonsurgical interventions for biliary anastomotic strictures that occur after liver transplantation. When these nonsurgical interventions fail, surgical re-anastomosis is considered; however, this is quite invasive and can cause additional injury that may lead to graft loss. We report a case in which conventional nonsurgical interventions failed, but a new method that involve the use of a transseptal needle-a device to create a transseptal left-heart access during cardiac catheter interventions-was successfully used in recanalization of the hepaticojejunal anastomotic obstruction. CASE: A 21-year-old man, who had received living-donor liver transplantation for biliary atresia at the age of 23 months presented with recurrent cholangitis and liver dysfunction due to a biliary anastomotic stricture of the hepaticojejunostomy. Therapeutic interventions for biliary stricture, including the PTB approach, double-balloon enteroscopic approach, and rendezvous approach failed. We then performed needle puncture of the anastomotic obstruction using a transseptal needle and succeeded in recanalizing the complete anastomotic obstruction. To perform the procedures safely, we evaluated the organ and needle positions using biplane fluoroscopy and placed a balloon in the afferent jejunal limb as a target for puncture. The 12 Fr catheter via the biliary route was removed 7 months after the procedure, without using a catheter, there was no recurrent stricture or cholangitis for 26 months. CONCLUSION: Using a transseptal needle to manage hepaticojejunal anastomotic obstruction can reduce the number of patients who need surgical re-anastomosis.

Cholesterol Granuloma of the Liver Mimicking Malignant Tumor: A Case Report.

Cholesterol granuloma is a benign, tumor-like lesion with an accumulation of cholesterol crystals in the tissue and is a consequence of a chronic inflammatory reaction. It commonly occurs in the middle ear but rarely in the liver. There is only one previous case report of cholesterol granuloma of the liver, which was caused by cholesterol hepatolithiasis. We report a case of cholesterol granuloma of the liver in a patient with no intrahepatic cholesterol stones; it was difficult to rule out malignant liver tumor preoperatively. The patient was a 79-year-old woman in whom a lesion in the liver was detected on abdominal ultrasonography. She was referred to our hospital for detailed examination and treatment. Abdominal contrast-enhanced computed tomography showed a 20 mm lesion with ring enhancement in the lateral segment of the liver during the arterial and delayed phases. Since a malignant tumor could not be ruled out radiologically, laparoscopic lateral segment hepatectomy was performed for definitive diagnosis and treatment. The resection specimen showed a yellowish-white lesion measuring 15 mm in diameter. Pathological examination showed a granulomatous lesion with cholesterol crystals surrounded by foreign body giant cells. The lesion was diagnosed as cholesterol granuloma of the liver. The postoperative course was good, and the patient was discharged on postoperative day 5. She was healthy, and no recurrence of the cholesterol granuloma was detected at the 5-month follow-up. This is the first case report of cholesterol granuloma of the liver mimicking a malignant liver tumor in a patient with no intrahepatic cholesterol stones.

Indications and outcomes of an endoscopic approach under laparotomy for the treatment of bilioenteric anastomotic strictures.

BAS is a potentially life-threatening complication of LDLT. The aim of this study was to report on the indications and outcomes of an endoscopic approach under laparotomy being used in our institution to treat BAS after LDLT, using hepaticojejunostomy, for a small case series. Eighty-three patients underwent an LDLT in our institution between 1991 and 2014. Retrospective chart review indicated that 10 of these patients developed BAS and were included in our analysis. The endoscopic approach under laparotomy was used in three patients who developed BAS 10 yr or more after their LDLT and in whom a percutaneous transhepatic approach and an endoscopic approach had failed. The course of recovery post-operatively was unremarkable for two of the three patients who underwent the endoscopic approach under laparotomy. One patient required follow-up laparotomy to treat a perforation of the bowel causing acute peritonitis. At follow-up one yr post-operatively, the stent tube was removed in two patients who recovered fully. The other patient had full recovery with the stent remaining in situ. The endoscopic approach under laparotomy could be a safe and promising option in the treatment of BAS to avoid surgical re-anastomosis.

Soluble Thrombomodulin Ameliorates Ischemia-Reperfusion Injury of Liver Grafts by Modulating the Proinflammatory Role of High-Mobility Group Box 1.

Transplantation using grafts obtained after cardiac death (CD) is considered a promising solution for graft shortages. However, no standard criteria for organ preservation have been established for CD donors. High-mobility group box 1 (HMGB1) is a DNA-binding protein that is released from dying hepatocytes as an early mediator of inflammation and organ tissue damage. HMGB1 stimulates immunocytes to produce inflammatory cytokines, thereby amplifying the inflammatory response. Thrombomodulin is an integral membrane protein that functions as an endothelial anticoagulant cofactor, and it binds HMGB1 through the extracellular domain. We investigated the effects of ART-123, recombinant human soluble thrombomodulin, on warm ischemia-reperfusion injury in liver grafts. Male Wistar rats were divided into four ex vivo groups: heart-beating (HB) group, in which livers were isolated from HB donors; CD group, in which livers were isolated from CD donors exposed to apnea-induced conditions and warm ischemic conditions for 30 min after cardiac arrest; and two CD groups pretreated with ART-123 (1 or 5 mg/kg). Each isolated liver was reperfused for 1 h after cold preservation for 6 h. The perfusate levels of HMGB1, LDH, TNF-α, and IL-6 were significantly lower in the CD group pretreated with ART-123 (5 mg/kg) than in the CD group. Bile production was significantly higher in the CD group pretreated with ART-123 (5 mg/kg) than in the CD group. The sinusoidal spaces were significantly narrower in the CD group than in the other groups. We propose that ART-123 maintains sinusoidal microcirculation by reducing endothelial cell damage during warm ischemia-reperfusion injury.

Time to symptom onset and manual reduction outcomes as predictors of bowel viability in incarcerated obturator hernias.

The current study aimed to identify the indications for manual reduction in incarcerated obturator hernias (OH). Further, whether time to symptom onset and manual reduction outcomes can be predictors of bowel viability and the need for bowel resection in incarcerated OH were examined. This retrospective study included 26 patients with incarcerated OH who underwent surgery. All patients underwent manual reduction, and computed tomography scan after manual reduction confirmed hernia release. Multivariate analyses were performed to determine the predictors of bowel resection. The bowel resection group had a significantly longer average time to symptom onset than the nonbowel resection group (88 vs 36 h). Further, the bowel resection group was more likely to have failed manual reduction than the nonbowel resection group. A time to symptom onset of ≥ 72 h and failed manual reduction were significant predictors of bowel viability. Age, sex, hernia localization, American Society of Anesthesiologists physical status score, and laboratory findings did not differ significantly between the bowel resection and nonbowel resection groups. Time to symptom onset and manual reduction outcomes are significant predictors of bowel viability in incarcerated OH. Patients with a time to symptom onset of ≥ 72 h and failed manual reduction require surgical evaluation due to a high risk of bowel nonviability. Therefore, a cautious approach is required in the management of OH, and further research on optimized treatment protocols should be conducted.

A new liver graft preparation method for uncontrolled non-heart-beating donors, combining short oxygenated warm perfusion and prostaglandin E1.

BACKGROUND: To resolve the shortage of donors associated with liver transplantation, the potential uncontrolled non-heart-beating donor (UNHBD) pool is expected to increase. However, warm ischemia-reperfusion injury leads to inferior survival in transplantation using the grafts from UNHBD compared with those from heart-beating donors. To overcome this problem, we developed a new method for preparation of liver grafts from UNHBDs consisting of a combination of short oxygenated warm perfusion (SOWP) and prostaglandin E1 (PGE1). METHODS: Using an ex vivo perfusion rat model, we examined the effectiveness of this new method. RESULTS: Using SOWP and PGE1 treatment, the total amount of bile production during reperfusion in UNHBD grafts was increased to the same level as that in the heart-beating donor grafts. The addition of PGE1 to SOWP buffer decreased aspartate aminotransferase/alanine aminotransferase and tumor necrosis factor α levels during 1 h of reperfusion. Necrosis and apoptosis were significantly decreased by SOWP + PGE1 treatment. SOWP + PGE1 ameliorated induction of mitochondrial permeability transition, and the total amount of mitochondrial cytochrome c in the SOWP + PGE1 group after reperfusion was kept significantly higher than that in the no treatment group. Cytosolic c-Jun N-terminal protein kinase activation was significantly suppressed by SOWP + PGE1. Decrease in mitochondrial Bcl-2 was suppressed by SOWP alone and SOWP + PGE1 treatment, and Bax in the mitochondria was significantly suppressed by SOWP + PGE1. CONCLUSION: SOWP and PGE1 prior to cold preservation significantly improved the function of liver grafts that underwent warm ischemia-reperfusion injury. Therefore, this method might be useful in liver transplantation using UNHBD grafts.

Clinical Significance of the Mac-2 Binding Protein Glycosylated Isomer as a Surrogate Marker of Graft Fibrosis After Pediatric Liver Transplantation.

BACKGROUND: After pediatric liver transplantation, liver fibrosis may occur during long-term follow-up. Noninvasive markers for assessing this liver fibrosis are desired. Mac-2 binding protein glycosylated isomer (M2BPGi) has recently been reported as a useful biomarker for liver fibrosis. However, its usefulness in the pediatric population is yet to be established. This study investigated the clinical significance of M2BPGi levels as a surrogate marker of graft fibrosis after pediatric liver transplantation. METHODS: We retrospectively identified 96 patients who underwent pediatric liver transplantation at our institution between 1991 and 2015. The association between M2BPGi levels and other fibrosis markers was analyzed in 60 patients in whom fibrosis markers were measured. The association between fibrosis marker levels and graft fibrosis was assessed in 42 patients who underwent biopsies between 2016 and 2022. RESULTS: The M2BPGi levels were statistically correlated with the hyaluronic acid and type-IV collagen levels. None of the fibrosis markers were significantly associated with liver graft fibrosis, although the levels of these markers were slightly higher in patients with severe liver fibrosis than in those with mild fibrosis. CONCLUSIONS: The M2BPGi levels had a limited ability to assess liver graft fibrosis after pediatric liver transplantation, similar to other fibrosis markers. Further studies with larger cohorts are required to validate these findings externally.

Functional Assessment of Cardiac Arrest Hepatocytes and Effect of Mechanical Perfusion on Function in a Rat Model.

BACKGROUND: Hepatocyte transplantation has been reported to be useful for metabolic diseases and acute liver failure. However, the shortage of donors limits its widespread use. The use of livers from donors after circulatory death, which are currently unavailable for liver transplantation, may alleviate donor shortage. In this study, we investigated the effects of mechanical perfusion on cardiac arrest hepatocytes in a rat model using cardiac arrest donor livers, and we evaluated the function of cardiac arrest hepatocytes. METHODS: F344 rat hepatocytes isolated from livers removed during cardiac pulsation were compared with those isolated from livers removed after 30 minutes of warm ischemia after cardiac arrest. We then compared hepatocytes isolated from livers removed after 30 minutes of warm ischemia with those isolated after 30 minutes of mechanical perfusion before isolation. The yield per liver weight, ammonia removal capacity, and adenosine diphosphate/adenosine triphosphate ratio were evaluated. RESULTS: Thirty minutes of warm inhibition reduced hepatocyte yield but did not alter ammonia removal capacity and energy status. Mechanical perfusion increased hepatocyte yield and improved the adenosine diphosphate/adenosine triphosphate ratio after 30 minutes of warm inhibition. CONCLUSION: Thirty minutes of warm ischemic time may decrease isolated hepatocyte yield without degrading their function. If increased yields are obtained, livers from donors dying of cardiac arrest could be used for hepatocyte transplantation. The results also suggest that mechanical perfusion may positively affect the energy status of hepatocytes.

First Multicenter Clinical Trial on Machine Perfusion Preservation for Marginal Donor Kidney Transplantation in Japan.

BACKGROUND: Machine perfusion has not been widely used because of its low demand in Japan; however, we believe its advantages may increase the number of organ transplants. METHODS: Here, we report the first clinical trial of machine perfusion for kidney transplantation in Japan. We used the CMP-X08 perfusion device (Chuo-Seiko Co, Ltd, Asahikawa, Hokkaido, Japan) to preserve the donated organs. The flow rate, perfusion pressure, renal resistance, and temperature were monitored during continuous hypothermic perfusion. RESULTS: From August 2020 to the present, 13 cases of perfusion-preserved kidney transplantation have been performed. Of these, ten and 3 cases were performed using organs donated after brain death (DBD) and cardiac death (DCD), respectively. The average age of the recipients was 55.9 ± 7.3 (45-66) years. The average dialysis period was 14.8 ± 8.4 (0-26) years. The donor's final creatinine level before organ retrieval was 1.58 ± 1.0 (0.46-3.07) mg/dL. The warm ischemic times of the 3 DCD donors were 3, 12, and 18 minutes. The average total ischemic time was 12.0 ± 3.7 (7.17-19.88) hours. The average MP time was 140 (60-240) minutes. A total of 7 cases had delayed graft function. The best creatinine level during hospitalization was 1.17 ± 0.43 (0.71-1.85) mg/dL. There were no primary non-functional cases, and perfusion preservation was safely performed in all cases. CONCLUSIONS: Therefore, we present this report as the first clinical trial on machine perfusion for kidney transplantation from marginal donors with DBD and DCD in Japan.

A case of spontaneously ruptured hepatic angiosarcoma resected after transcatheter arterial embolization.

Hepatic angiosarcoma is a very rare disease, but it has a poor prognosis. Here, we report the case of a 77-year-old man who was referred to our hospital for suspicion of hepatocellular carcinoma and cancerous peritonitis. Based on the imaging findings, a diagnosis of spontaneously ruptured hepatic hemangioma was made. Six days later, the patient was transported to the hospital in a state of shock and an emergency transarterial embolization was performed. He underwent lateral hepatic segmentectomy 7 days later. Histopathologically, he was diagnosed with hepatic angiosarcoma. Fever was observed 21 days after surgery, and computed tomography was performed. Multiple liver masses, which ware increasing rapidly, were found, and hepatic angiosarcoma recurrence was confirmed. He requested home medical care and died at home 36 days after surgery. When a tumor diagnosed as a hepatic hemangioma by imaging has ruptured, the possibility of hepatic angiosarcoma should be considered.

Long-term survival of an adult patient with undifferentiated embryonal sarcoma of the liver with multidisciplinary treatment: a case report and literature review.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) primarily occurs in children; it is rarely seen in adults and appears to have a poor prognosis. However, in recent years, some cases indicated that long-term survival was possible due to a combination of multiple surgeries, chemotherapy, and liver transplantation. CASE PRESENTATION: A 33-year-old female patient presented with a complaint of epigastric pain, for which she underwent a medical examination. Computed tomography (CT) and magnetic resonance imaging showed a cystic tumor in the right hepatic lobe, approximately 10 cm in size. During observation, the abdominal pain worsened, and a contrast-enhanced CT revealed that the tumor's peripheral solid components increased in size and volume, suggesting a malignant tumor threatening hepatic rupture. Subsequently, transcatheter arterial embolization of the anterior and posterior segmental branches of the hepatic artery was performed, followed by right trisectionectomy. Histopathological and immunohistochemical examinations of the lesion revealed UESL. Two months after the surgery, we initiated sarcoma-directed chemotherapy with doxorubicin because of multiple metastases to the liver. After initiating the chemotherapy, she received another regimen using gemcitabine/docetaxel, eribulin, trabectedin, ifosfamide/mesna, pazopanib, and cisplatin. During the chemotherapy, she underwent palliative surgery twice due to the progressive disease. She lived for 49 months after the initial operation. CONCLUSIONS: Improved long-term survival was achieved in an adult patient with UESL after multidisciplinary therapy, involving a combination of three surgical procedures and several chemotherapies.

Adult Living Donor Liver Transplantation for the Carryover Patients After Kasai Operation Who Have a History of Multiple Laparotomies.

OBJECTIVE: Approximately 50 years have passed since the Kasai operation announcement for biliary atresia. In adult liver failure cases, the so-called "the carryover cases after Kasai operation" have increased. These patients often underwent polysurgery. In such cases, adult living-donor liver transplantation (LDLT) is occasionally difficult. Many complications have been reported to be caused by severe cholangitis, hepatic portal regional inflammation, and adhesion. We investigated the complications of adult LDLT in post-Kasai biliary atresia cases with polysurgery. METHODS: Between 1991 and 2021, we performed 205 LDLT cases. We investigated the outcome of adult LDLT for post-Kasai biliary atresia cases (transplanted over 16 years old) (n = 20) and the risk factors for complications after LDLT. RESULTS: On 5 years overall survival, there were no significant differences between "adult LDLT for post-Kasai" group and the others (81.8% vs 81.2%). Adult LDLT for post-Kasai was not found to be a risk factor for complications. However, polysurgery before LDLT was an independent risk factor for biliary stenosis and portal stenosis, as identified in our univariate and multivariate analysis. We analyzed the relationship between biliary stenosis and the frequency of laparotomies using a receiver operating characteristic curve. The analysis showed that the cutoff point (maximum point of sensitivity plus specificity) was more than 3 times that of laparotomies before LDLT. CONCLUSIONS: In our study, adult LDLT for post-Kasai cases was not a risk factor for any complications. However, polysurgery before LDLT has been identified as a risk factor for biliary stenosis and portal vein stenosis.

Optimal Conditions for Oxygenated Subnormothermic Machine Perfusion for Liver Grafts Using a Novel Perfusion Device.

BACKGROUND: Liver transplantation from donors after cardiac death (DCD) resolves donor shortages. PURPOSE: We investigated the optimal time for subnormothermic oxygenated perfusion in DCD liver transplantation. METHODS: Ten F1 pigs (body weight: 27-32 kg) were allocated to 2 groups: the heart beating group (n = 6), from which livers were retrieved while the heart was beating, and the donation after cardiac death (DCD) group (n = 4), in which liver retrieval was performed on pigs under apnea-induced cardiac arrest for 20 minutes. In both groups, the livers were kept in cold storage for 2 hours after retrieval and perfused with a subnormothermic oxygenated Krebs-Henseleit buffer for 120 minutes. We used a novel perfusion device, which can set maximum perfusion pressures of arteries and portal vein, developed by Asahikawa Medical University and Chuo Seiko Co. Bile production, liver enzymes, and inflammatory cytokines were measured and the sinusoidal space, using tissue specimens taken from liver grafts, was measured at 30, 60, 90, and 120 minutes after the start of perfusion. RESULTS: Bile production peaked at 90 minutes. Significantly higher levels of liver enzymes and inflammatory cytokines were found in the DCD group (P < .05). The release of liver enzymes peaked at 60 minutes and that of inflammatory cytokines peaked at 90 minutes. The hepatic sinusoidal space was wide at 90 minutes and narrowed after 120 minutes. CONCLUSIONS: The results suggest that subnormothermic oxygenation perfusion may maintain optimal graft condition until around 90 minutes and perfusion for more than 120 minutes may be counterproductive.

Oxygenated Hypothermic Machine Perfusion of Kidney Transplantation from Donors After Cardiac Death Due to Long-Term Low Blood Pressure and Hypoxia: The First Case Report of a Clinical Trial Using a New Japanese Perfusion System.

BACKGROUND: Machine perfusion of marginal kidney grafts obtained from donors after cardiac death (DCD) has become a standard therapy worldwide. However, the use of grafts from DCD due to long-term low blood pressure is associated with a high incidence of primary graft nonfunction. Furthermore, the importance of oxygenation in machine perfusion remains unclear. We report the first case of a clinical trial of a kidney transplant obtained from a DCD using a Japanese oxygenated hypothermic perfusion system (CMX-08W, Chuo Seiko Co Ltd, Asahikawa, Japan). PATIENTS AND METHODS: The donor was a 61-year-old man with amyotrophic lateral sclerosis. His SpO2 decreased to 80% to 90%, his blood pressure remained consistently low for 4 hours and 30 minutes, and he suffered a cardiac arrest. Subsequently, we carried him to the operating room. The warm ischemic time was 12 minutes, and the cold ischemic time was 418 minutes. The recipient was a 58-year-old man who had been undergoing hemodialysis for 26 years. He was diagnosed with nephrosclerosis and multiple renal cysts. Oxygenated hypothermic machine perfusion was used on the kidney transplant obtained from the DCD. RESULTS: The recipient gradually recovered and was withdrawn from hemodialysis therapy 14 days post transplantation. His renal function improved, and he was discharged on postoperative day 36. Currently, his renal function remains good (phosphocreatine, 1.7). CONCLUSIONS: Oxygenated machine perfusion is used to preserve organs and determine if an organ is suitable for transplantation. This may provide the possibility of perfusion preservation and expand the criteria for cardiac arrest-associated renal transplantation.

Usefulness of Thiel-Embalmed Cadavers for Training in Organ Procurement.

BACKGROUND: The number of brain-dead donors has been increasing; however, the opportunity for young surgeons to experience deceased donor surgeries is extremely limited, especially in many Asian countries including Japan. Deceased donor surgeries require unique surgical skills and knowledge; however, it is difficult to provide on-the-job guidance and education. Therefore, cadaver training is meaningful and suitable for the training of deceased donor surgeries. Thiel's embalming method (TEM) provides natural coloration, flexibility, and tissue plasticity, and is widely used for cadaver surgical training. In this study, we evaluated the usefulness of Thiel's embalmed cadaver training for organ procurement surgery. MATERIAL AND METHODS: Each trainee performed hepatectomy, pancreatectomy, and nephrectomy using conventional open techniques. Faculty experts of transplantation surgery and organ procurement took attendees through surgical steps. After the procedure, all participants were asked to complete a voluntary, anonymous survey, consisting of a 10-point satisfaction scale, to evaluate their perceptions of the training. RESULTS: A total of 33 gastrointestinal surgeons participated in the training program for procuring the liver, pancreas, and kidneys. In the questionnaire administered to the participants, the evaluation was generally satisfactory, with an average of 9.1 points on the 10-point scales. Some participants expressed that Thiel-embalmed cadavers are more suitable for training on organ procurement compared with animals used in wet-lab training. CONCLUSION: We conclude that organ procurement training in human cadavers preserved by TEM is useful and suitable for practicing deceased donor organ procurement, especially in countries where deceased donors are not common, as in Japan.

Probable posttransplant lymphoproliferative disorder after pediatric living donor liver transplantation: Is a biopsy still needed?

Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein-Barr virus (EBV). Recently, EBV-related PTLD was defined as probable PTLD or proven PTLD. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly, or other end-organ manifestations, without a histological diagnosis, together with significant EBV DNAemia. Proven PTLD is the detection of EBV-encoded proteins in a tissue specimen, together with symptoms and/or signs originating from the affected organ. Probable PTLD after pediatric liver transplantation has not been well documented. Therefore, here, we aimed to describe cases of five pediatric patients with probable PTLD after liver transplantation, who were successfully treated with preemptive immunosuppression reduction with or without rituximab. All five patients (age range, 1-4 years; two girls and three boys) had EBV DNAemia. Three patients developed probable PTLD within 12 months of transplantation. Further, three patients had a significantly high EBV viral load, but the other two patients with lymphadenopathy and end-organ manifestation had a relatively low EBV viral load. Early onset pediatric PTLD with significant EBV DNAemia is almost universally EBV-related. Biopsy was not performed in any patient due to the relative inaccessibility of the lesion and young age of the patients. If the patient's symptoms are too mild, if excisional biopsy is too difficult to perform, or if the patient is too sick to undergo an invasive procedure, initiating preemptive treatment without a histological diagnosis could be the treatment option.

A New Strategy of Liver Transplantation for Locally Advanced Unresectable Perihilar Cholangiocarcinoma Using Living Grafts With Simultaneous Resection of Recipients' Hepatic Artery and Portal Vein Without Neoadjuvant Radiation: A Case Report.

BACKGROUND: Perihilar cholangiocarcinoma (pCCA) is often unresectable, because it includes crucial blood vessels in portal area. The prognosis of locally advanced unresectable cholangiocarcinomas is extremely poor. Recently, there have been several reports of the prognosis improving drastically with transplantation and combined chemoradiation therapy. However, liver transplantation for pCCA has 2 big problems. The first is that pCCA is located at a lethal position and its progress is sometimes rapid; therefore, the optimal timing of transplantation is sometimes lost. The second is vascular complications associated with neoadjuvant radiation, especially in living donor liver transplantation (LDLT). To overcome these problems, we performed conversion surgery using LDLT with simultaneous resection of the hepatic artery and portal vein, instead of neoadjuvant radiation. Herein, we report our experience of interposition reconstruction. METHODS: A 31-year-old man with primary sclerosing cholangitis (PSC) was diagnosed with locally advanced unresectable pCCA. The patient underwent radical chemotherapy (gemcitabine/cisplatin/S-1) and avoided radiation because of PSC. After 6 months, positron emission tomography-computed tomography revealed no lymph node metastasis. There was no time to wait. We immediately performed LDLT with simultaneous resection of hepatic artery and portal vein, and microsurgical reconstruction using auto-vessel grafts. RESULTS: The recipient recovered and was discharged 31 days posttransplant. His liver function improved, and he has had no recurrence after LDLT. CONCLUSION: LDLT with neoadjuvant radiation is associated with high risk of vascular complications. In some cases, conversion surgery after radical chemotherapy using good timing LDLT without radiation may increase chances of transplantation for locally advanced pCCA.

Recurrence of Adult Granulosa Cell Tumor in the Greater Omentum 11 Years after Surgery.

Adult-type ovarian granulosa cell tumors (AGCTs) are very rare tumors that account for <5% of all ovarian carcinomas. AGCTs have low malignancy potential and rarely metastasize 5-30 years after the initial diagnosis. Because time has passed from the first surgery and because recurrence develops in various locations, the differential diagnosis is difficult. In particular, tumors developing in the greater omentum are encountered rarely, and it is necessary to carefully consider the differential diagnosis, including primary and secondary neoplasms. Although CT is useful to detect omental tumors, the diagnosis requires invasive procedures. We report a case of AGCT recurrence in the greater omentum that was resected during laparoscopic cholecystectomy. A patient visited our hospital with right-sided abdominal pain. The CT revealed gallbladder stones, a ureteral stone, and a right abdominal mass. The diagnosis of the abdominal tumor was difficult on the basis of blood biochemical testing, gastrointestinal endoscopy, or image inspection. Although the patient underwent several previous surgeries and there were no findings of malignancy with positron emission tomography, we chose to resect the tumor for combined diagnosis and treatment during laparoscopic cholecystectomy. Intraoperative findings showed that the tumor originated from the greater omentum, and the tumor was diagnosed as AGCT recurrence by pathology. A recurrence of AGCT in the greater omentum is very rare, and laparoscopic surgery was safe and useful for resection, in our case.

Bleeding from jejunal varices formed at the Roux-en-Y jejunum site caused by the compression of the left renal vein after living donor liver transplantation with renoportal anastomosis.

BACKGROUND: Renoportal anastomosis is an option for the portal vein reconstruction of a liver transplantation with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report the case of gastrointestinal bleeding who underwent living donor liver transplantation (LDLT) with renoportal anastomosis. CASE PRESENTATION: Six-year-old female patient who underwent LDLT with renoportal anastomosis at 1 year of age had severe anemia with normal liver function during the follow-up period. The varices at the Roux-en-Y jejunum were considered the source of bleeding, and the compression of the left renal vein, which is known as a cause of Nutcracker syndrome, seemed to induce venous hypertension through the splenorenal shunt, which might induce the formation of the varices. She underwent percutaneous transhepatic sclerotherapy of the varices, and the anemia improved at her last follow-up, 6 months after sclerotherapy. This is the first reported case of Roux-en-Y jejunal varices bleeding related to the compression of the left renal vein after LDLT was performed with renoportal anastomosis. CONCLUSIONS: Although renoportal anastomosis should be cautiously performed when there are no options for severe portal vein thrombosis, the status of the left renal vein and new collateral formation should be observed carefully during the follow-up period in pediatric cases of renoportal anastomosis.