Donor bile duct evaluation with magnetic resonance cholangiography in living-donor liver transplantation: a novel anatomical classification for predicting surgical techniques.

PURPOSE: To propose a novel, inclusive classification that facilitates the selection of the appropriate donor and surgical technique in living-donor liver transplantation (LDLT). METHODS: The magnetic resonance cholangiography examinations of 201 healthy liver donors were retrospectively evaluated. The study group was classified according to the proposed classification. The findings were compared with the surgical technique used in 93 patients who underwent transplantation. The Couinaud, Huang, Karakas, Choi, and Ohkubo classifications were also applied to all cases. RESULTS: There were 118 right-lobe donors (58.7%) and 83 left-lateral-segment donors (41.3%). Fifty-six (28.8%) of the cases were classified as type 1, 136 (67.7%) as type 2, and 7 (3.5%) as type 3 in the proposed classification; all cases could be classified. The number of individuals able to become liver donors was 93. A total of 36 cases were type 1, 56 were type 2, and 1 was type 3. Of the type 1 donors, 83% required single anastomosis during transplantation, whereas six patients classified as type 1 required two anastomoses, all of which were caused by technical challenges during resection. Moreover, 51.8% of the cases classified as type 2 required additional anastomosis during transplantation. The type 3 patient required three anastomoses. The type 1 and type 2 donors required a different number of anastomoses (P < 0.001). CONCLUSION: The proposed classification in this study includes all anatomical variations. This inclusive classification accurately predicts the surgical technique for LDLT.

Living Donor-Initiated Domino Split-Liver Transplantation in Pediatric Setup: A Case Report With Literature Review.

BACKGROUND: In patients undergoing liver transplantation for metabolic diseases, removing the patient's liver for transplantation to another recipient is called "domino liver transplantation." The extracted liver can be divided and transplanted into 2 recipients, which is called domino split-liver transplantation in the literature. However, in our study, the domino liver was obtained from a pediatric patient. METHODS: A patient with maple syrup urine disease (MSUD) underwent a living donor liver transplant, and the explanted liver was divided in situ into right and left lobes and transplanted to 2 separate patients. Demographic data, surgical techniques, postoperative period, and patient follow-ups were evaluated. RESULTS: The father's left lobe liver graft was transplanted into a 12-year-old boy with MSUD. The removed liver was divided in situ into right and left lobes. The left lobe was transplanted to a 14-year-old male patient, whereas the right lobe was transplanted to a 67-year-old male patient. The donor and the first recipient were discharged on postoperative days 5 and 22. The second pediatric patient who underwent domino split-left lobe transplantation was discharged on postoperative day 23. The adult patient who underwent domino split-right lobe transplantation died on postoperative day 12 owing to massive esophageal variceal bleeding. CONCLUSION: Patients who underwent liver transplantation due to MSUD are among the best donor choices for domino liver transplantation. If the extracted liver has a sufficient volume and anatomic features for a split, it can be used in "selected cases."

Literature Review and 2 Cases of Isolated Chylothorax After Pediatric Living-Donor Liver Transplant.

OBJECTIVES: Chylothorax without chylous ascites after liver transplant is rare. We present 2 cases of isolated chylothorax after liver transplant and a literature review. MATERIALS AND METHODS: We compiled a literature review of chylothorax cases after abdominal surgery and analyzed the cases related to liver transplant. The demographic information, follow-up results, and treatment details of our 2 cases of chylothorax after living-donor pediatric livertransplant were discussed. RESULTS: An 8-month-old child and a 15-month-old child with cholestatic liver disease and urea cycle defect, respectively, underwent living-donor left lateral segment liver transplant. Patients who presented with chylothorax after discharge were treated conservatively. CONCLUSIONS: Isolated chylothorax is rare complication after abdominal surgery, which is mostly possible to treat with conservative methods. Interventional procedures and a surgical approach should only be performed in resistant cases when conservative treatment has failed.

Anesthesia Management in Laparoscopic Donor Hepatectomy: The First Report from Turkey.

BACKGROUND: We aimed to report a single-center experience in laparoscopic donor left-side and right-side hepatectomy cases regarding preoperative evaluation, perioperative and anesthetic management protocols, and postoperative follow-up. METHODS: Laparoscopic donor left-side and right-side hepatectomy cases were included in the study because of their excessive transection area and bleeding potential. Medical records of living donors were reviewed in terms of age, sex, body mass index (BMI), presence of consanguinity with the recipient, perioperative and early postoperative biochemical parameters, hemodynamic changes during surgery, duration of surgery, the ratio of liver volume to total liver volume, perioperative complications, and length of hospital stay. RESULTS: Eighty-one laparoscopic living-donor hepatectomy procedures were performed in our unit between 2018 and 2022. Six laparoscopic donor right-side cases and two left-side cases were retrospectively reviewed. Donors' mean age and BMI were 29.6 ± 8.6 years and 23.1 ± 4.3, respectively. The average weights of the right and left lobe liver grafts were 727 g and 279 g, respectively, constituting 65.8% and 22.7% of the total liver volume, respectively. The mean operation time was 593 ± 94 minutes, and the mean volume of blood loss was 437 ± 294 mL. A major complication, namely portal vein stenosis, developed in 1 donor (1/8), and portal vein patency was achieved postoperatively. CONCLUSIONS: Anesthesia management and teamwork between surgeons and anesthesiologists are the most important building blocks for donor safety, which is of the utmost priority. Effective communication and cooperation in the operating room may prevent potential donor complications and improve postoperative recovery time.

Hepatic Artery Intimal Dissection During Living Donor Liver Transplantation: A New Modified Approach.

Ensuring optimal arterial flow in solid organ transplantation is very important. Insufficient flow causes important problems such as bile duct problems, intrahepatic abscess formation, and organ loss. Arterial intimal dissection is an important factor that negatively affects organ blood flow. In this study, hepatic artery dissections that we detected in patients who underwent living donor liver transplantation in our clinic were defined, and the microvascular intima-adventitial fixation technique, which can be considered a new approach, was described.

Using Pericholedochal Varix Inflow for Complete Portal Vein Thrombosis in Living Donor Liver Transplantation: A Case Report.

One of the crucial steps of liver transplantation is to provide the portal inflow. Portal vein thrombosis is the most challenging factor to achieve. Using a pericholedochal varix for portal inflow in a patient with complete portal vein thrombosis in living donor liver transplantation (LDLT) is a rare technique. We present our experience of a LDLT with PVT.

Evaluation of pulmonary arterial stiffness and comparison with right ventricular functions in patients with cirrhosis preparing for liver transplantation.

OBJECTIVE: Pulmonary complications are common in patients with liver cirrhosis. Devolopment of pulmonary hypertension (PH) is associated with a poor prognosis in these patients. Pulmonary arterial stiffness (PAS) is considered an early sign of pulmonary vascular remodeling. The aim of this study is to investigate PAS and compare it with right ventricular (RV) functions in patients with cirrhosis who are scheduled for liver transplantation. METHODS: The study included 52 cirrhosis patients (mean age 51.01 ± 12.18 years, male gender 76.9%) who were prepared for liver transplantation and 59 age and sex matched (mean age 51.28 ± 13.63 years, male gender 62.7%) healthy individuals. Patients with left ventricular ejection fraction (LVEF) less than 55%, ischemic heart disease, more than mild valvular heart disease, chronic pulmonary disease, congenital heart disease, rheumatic disease, moderate to high echocardiographic PH probability, rhythm or conduction disorders on electrocardiography were excluded from the study. In addition to conventional echocardiographic parameters, PAS value, pulmonary vascular resistance (PVR) and RV ejection efficiency was calculated by the related formulas with transthoracic echocardiography (TTE). RESULTS: Demographic characteristics and cardiovascular risk factors of the groups were similar. PAS, PVR, and sPAP values were found to be significantly higher in the patient group (20.52 ± 6.52 and 13.73 ± 2.05; 1.43 ± 0.15 and 1.27 ± 0.14; 27.69 ± 3.91 and 23.37 ± 3.81 p < 0.001, respectively). RV FAC and RV Ee were significantly lower and RV MPI was significantly higher in the patient group (45.31 ± 3.85 and 49.66 ± 3.62, p < 0.001; 1.69 ± 0.35 and 1.85 ± 0.23, p = 0.005; 0.39 ± 0.07 and 0.33 ± 0.09, p = 0.001, respectively). PAS was significantly correlated with RV FAC and MPI (r = -0.423, p < 0.001; r = 0.301, p = 0.001, respectively). CONCLUSIONS: Increased PAS in cirrhosis patients may be associated with early pulmonary vascular involvement. Evaluation of RV functions is important to determine the prognosis in these patients. FAC, MPI, and RV Ee measurements instead of TAPSE or RV S' may be more useful in demonstrating subclinical dysfunction. The correlation of PAS with RV FAC and MPI may indicate that RV subclinical dysfunction is associated with early pulmonary vascular remodeling in patients with liver cirrhosis.

Trans-Arterial Radioembolization with Yttrium-90 of Unresectable and Systemic Chemotherapy Resistant Hepatoblastoma in Three Toddlers.

The aim of this short communication was to report the results of transarterial radioembolization (TARE) with Yttrium-90 (Y90) loaded resin microspheres in three toddlers with unresectable and systemic chemotherapy-resistant HB hepatoblastoma (HB). Six TARE procedures were performed on the patients. The dose required for treatment was calculated using partition model. Administered doses of Y90 were 1.369, 0.851, and 1.147 GBq. Complete radiological response in two patients and partial response enabling liver resection in one patient were achieved. Neither life-threatening nor minor complications developed after the treatment. These results demonstrates that HB is a radiosensitive neoplasm, and TARE-Y90 can be used as the primary, neoadjuvant and palliative treatment method in patients with unresectable and systemic chemotherapy-resistant HBs. However, studies with higher number of patients and long-term results are required.

Liver Transplant and Improvements in Cholesterol Biosynthesis Defects: A Case Report of Smith-Lemli-Opitz Syndrome.

Smith-Lemli-Opitz syndrome is an autosomal recessive metabolic disease characterized by mental retardation and multiple congenital anomalies. The main pathology is the lack of the enzyme 3ß-hydroxysterol Δ7-reductase, which is the last enzymatic step in cholesterol synthesis, ending with a low cholesterol level. Cholesterol is vitally important in cell membranes and myelination of the nervous system. The cholesterol level affects many systems of the body, especially the nervous system. The cause of liver involvement in Smith-Lemli-Opitz syndrome is unclear, and many hypotheses have been suggested. Here, we present the early results of a patient with Smith-Lemli-Opitz syndrome who underwent living-donor liver transplant due to cirrhosis. As a result of liver transplant, normal cholesterol levels were shown, as well as improvements in the patient's neurodevelopment and behavior. Early liver transplant may be considered for patients with a defect of cholesterol biosynthesis, even in the absence of cirrhosis, and may be a future treatment option to prevent risks of neurologic deterioration.

Examining the Hepatic Immune System in Children With Liver Disease With Fine Needle Aspiration.

OBJECTIVES: Liver biopsy is the standard in diagnosing liver diseases. Yet, it provides little space to perform comprehensive immune profiling of the liver. Hence, we explored whether fine needle aspirates (FNAs) could be used to elucidate the hepatic immunity in children. METHODS: We enrolled 74 children undergoing diagnostic (n = 17) or protocol biopsy (n = 57) following liver transplantation (LT). Matched blood and FNAs were obtained. Additionally, explant liver tissue was collected from children (n = 14) undergoing LT. Immune cells were isolated from peripheral blood, FNAs and explanted livers. Immune-phenotypical profiling was done by flow cytometry. RESULTS: Biopsied patients (58% female) were at a median age of 46 months (interquartile range [IQR]: 12-118) and LT patients (71% female) were 48 months (IQR: 21-134, P = 0.78) old. CD69+, a hallmark of tissue-resident immune cells was expressed in 1.3% of CD3+ T cells from blood being higher in FNA (20%) and tissue (49%, P < 0.001). CD4+ T-cell frequencies in tissue (13%) and FNAs (20%) were lower compared to blood (35%, P < 0.001) whereas CD8+ T cells in tissue (33.5%) and FNA (32%) were higher than in blood (25%, P < 0.01). Mucosal associated invariant T cells were enriched in liver tissue (8.8%) and in the FNA (4.4%) compared to blood (1.7%, P < 0.001). Whereas the percentage of total Tregs (CD4+CD25+FOXP3+CD127low/-) decreased, the proportion of activated Tregs (CD4+CD45RA-FOXP3high) increased in FNA and explant. Breg (CD19+CD20+CD24highCD38high) frequencies were similar in all groups. CONCLUSION: FNA is a practical method to sample the liver immune system collecting even small cell subsets such as regulatory T/B cells.

Patient With Hemophilia A Undergoing Consecutive Coronary Bypass and Live Donor Liver Transplantation: A Case Report.

Liver transplantation surgery due to cirrhosis in patients with hemophilia is rare and there are limited cases in the literature. We present a case of a patient with hemophilia A who underwent 2 consecutive surgeries owing to coronary artery disease and cryptogenic cirrhosis with hepatocellular carcinoma. After these surgeries, no bleeding or other complications were seen, and the factor VIII levels have not changed since liver transplantation in the follow-up.

Cardiac Intervention Before Liver Transplantation.

BACKGROUND: Cardiovascular complication is one of the leading causes of mortality after liver transplantation (LT). Thus, a thorough cardiac evaluation is a must before proceeding to a liver transplant surgery. Percutaneous coronary intervention (PCI) with stent and to a lesser extent coronary artery bypass grafting (CABG) are both valuable treatment options for patients with coronary artery disease. METHODS: A retrospective, single-center study that included patients who underwent cardiac intervention and subsequent LT for end-stage liver disease. All patients who had PCI or CABG were included in the study. RESULTS: Twenty-nine adult patients out of 51 had a cardiac intervention before liver transplantation. Twenty-four patients had a diagnostic PCI, 3 patients had therapeutic PCI with stent, and 2 had failed PCI and proceeded to CABG before liver transplant. The mean age of the patients was 60.5 years. There were 24 men. All patients had cirrhosis. The 2 CABG cases were done during the same admission with a 13- and 18-day interval between the CABG and the transplantation. Both cases were live-related liver transplantation. No mortality was reported. CONCLUSION: In case of PCI failure, CABG may be a valuable and safe treatment option for cirrhotic patients as a preparation for liver transplantation. Live donor liver transplantation may be a good back-up for those patients in case they develop hepatic decompensation.

Are the criteria always right? Assessment of hepatocellular carcinoma cases in living donor liver transplantation at a high-volume center

Background/aim: With the increased experience in living donor liver transplantation (LDLT), it has been adopted for the treatment of hepatocellular carcinoma (HCC), with emerging discussions of criteria beyond tumor size and number. In contrast to deceased donor liver transplantation (DDLT), recipient selection for LDLT is not limited by organ allocation systems. We discuss herein the assessment, criteria, and experience with liver transplantation (LT) in HCC cases at a high-volume LDLT center. Material and methods: Between August 2006 and December 2017, 191 adult LT HCC recipients with at least one-year follow-up were retrospectively analyzed. Results: In 191 patients, one-, three- and five-year survival rates were 87.2%, 81.6%, and 76.2%, respectively, including early postoperative mortality. In 174 patients with long-term follow-up, one-, three- and five-year disease-free survival rates were 91.6%, 87.7%, and 84.4%, respectively. When multivariate analysis was utilized, tumor differentiation was the only factor which statistically affected survival (p = 0.025). Conclusion: LDLT allows us to push the limits forward and the question "Are the criteria always right?" is always on the table. We can conclude that, with the advantage of LDLT, every HCC patient deserves a case-by-case basis discussion for LT under scientific literature support. In borderline cases, tumor biopsy might help determine the decision for LT.

Extreme living donation: A single center simultaneous and sequential living liver-kidney donor experience with long-term outcomes under literature review.

OBJECTIVES: Living liver and kidney donor surgeries are major surgical procedures applied to healthy people with mortality and morbidity risks not providing any direct therapeutic advantage to the donor. In this study, we aimed to share our simultaneous and sequential living liver-kidney donor experience under literature review in this worldwide rare practice. MATERIAL AND METHODS: Between January 2007 and February 2018, a total of 1109 living donor nephrectomies and 867 living liver donor hepatectomies were performed with no mortality to living-related donors. Eight donors who were simultaneous or sequential living liver-kidney donors in this time period were retrospectively reviewed and presented with their minimum 2- year follow-up. RESULTS: Of the 8 donors, 3 of them were simultaneous and 5 of them were sequential liver-kidney donation. All of them were close relatives. Mean age was 39 (26-61) years and mean BMI was 25.7 (17.7-40). In 3 donors, right lobe, in 4 donors, left lateral sector, and in 1 donor, left lobe hepatectomy were performed. Median hospital stay was 9 (7-13) days. Two donors experienced early and late postoperative complications (Grade 3b and Grade 1). No mortality and no other long-term complication occurred. CONCLUSION: Expansion of the donor pool by utilizing grafts from living donors is a globally-accepted proposition since it provides safety and successful outcomes. Simultaneous or sequential liver and kidney donation from the same donor seems to be a reasonable option for combined liver-kidney transplant recipients in special circumstances with acceptable outcomes.

A comparison of rates and severity of chronic kidney disease in deceased-donor and living-donor liver transplant recipients: times matter

Background/aim: The progression of chronic kidney disease (CKD) in recipients of living-donor liver transplant (LDLT) compared to deceased-donor liver transplant (DDLT) has not been studied in the literature. We hypothesize that CKD stage progression in LDLT recipients is reduced compared to that of their DDLT counterparts. Materials and methods: A retrospective study was undertaken including 999 adult, single-organ, primary liver transplant recipients (218 LDLT and 781 DDLT) at 2 centers between January 2003 and December 2012, in which CKD progression and regression were evaluated within the first 3 years after transplantation. Results: Waiting time from evaluation to transplantation was significantly lower in LDLT patients compared to recipients of DDLT. CKD stage progression from preoperative transplant evaluation to transplantation was significantly greater in DDLT. Deceased-donor liver transplant recipients continued to have higher rates of clinically significant renal disease progression (from stage I­II to stage III­V) across multiple time points over the first 3 years posttransplant. Furthermore, a greater degree of CKD regression was observed in recipients of LDLT. Conclusion: It can be concluded that LDLT provides excellent graft and patient survival, significantly reducing the overall incidence of clinically significant CKD stage progression when compared to DDLT. Moreover, there is a significantly higher incidence of CKD stage regression in LDLT compared to DDLT. These observations were maintained in both high and low model for end-stage liver disease(MELD)populations. This observation likely reflects earlier access to transplantation in LDLT as one of the contributing factors to preventing CKD progression.

Non-occlusive hepatic artery hypoperfusion syndrome and imaging findings: a systematic review.

Non-occlusive hepatic artery hypoperfusion syndrome (NHAHS), in other words, splenic steal, is a rare disorder that can arise following liver transplantation. After liver transplantation, its frequency has been defined as between 0.6 and 10.1%. The diversion of flow from hepatic to splenic arteries results in low perfused hepatic artery which causes elevated liver enzymes, hyperbilirubinemia, and graft dysfunction. This may result from a high resistance in the hepatic arteries, enlarged splenic arteries, a limited hepatic arterial flow due to high portal flow, or a discordance of the graft size and hepatic arterial flow. There may be a need for some prophylactic and/or posttransplant treatment procedures. We aimed to describe pre and post-treatment imaging findings of NHAHS.

Complications and outcomes of 890 living liver donor hepatectomies at a single center: risks of saving loved one's life.

OBJECTIVES: Living liver donor surgery is a major surgical procedure applied to healthy people with mortality and morbidity risks and does not provide any direct therapeutic advantage to the donor. We retrospectively analyzed the postoperative complication of our living liver donors to figure out the risks of donation. MATERIAL AND METHODS: Between November, 2006 and December, 2018, a total of 939 living liver donor hepatectomies were performed with no mortality to the living-related donors. Eight hundred and ninety donors with a minimum 1-year follow-up were analyzed retrospectively. RESULTS: Of the 890 donors, 519 (58.3%) were males and 371 (41.7%) were females. Mean age was 35 years (18-64) and mean body mass index was 25.7 kg/m2 (17.7-40). Right donor hepatectomy was performed to 601 (67.5%), left donor hepatectomy to 28 (3.2%) and left lateral sector hepatectomy to 261 (29.3%) of the donors. Of the 890 donors, 174 (19.5%) donors experienced a total of 204 early and late complications including life- threatening and nearly life- threatening complications in 26 (2.9%) of them. Intraoperative complication occurred in 4 (0.5%) donors. Right donors hepatectomy complication rate (23.3%) was higher than left donor (14.3%) and left lateral sector donor hepatectomy (11.5%). CONCLUSION: All donor candidates should be well-informed not only on the details of early and late complications of living liver donation, also possible outcomes of the recipient. In addition to detailed physical evaluation, preoperative psychosocial evaluation is also mandatory. Comprehensive donor evaluation, surgical experience, surgical technique, close postoperative follow-up and establishing a good dialog with the donor allows better outcomes.

Liver Transplant in Children with Hepatoblastoma.

OBJECTIVES: In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Sisli Memorial Hospital Transplantation Center are presented. MATERIALS AND METHODS: We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information. RESULTS: The median age of the 10 patients included in our study was 13.5 months (range, 8-120 mo), and the median weight was 10 kg (range, 6.5-30 kg). Two of the patients were twins. Five patients had pretreatment extent of disease III (centrally placed cases), and five had pretreatment extent of disease IV hepatoblastoma. Preoperative chemotherapy was given to 7 patients as cisplatin plus doxorubicin and to 3 patients per the International Childhood Liver Tumors Strategy Group 3 High-Risk Protocol at external centers. These protocols were administered according to treatment center preference. Nine patients received transplants from living donors. Two grafts were right lobes, and 7 were left lateral segments. In the remaining patient, a whole liver was received from a deceased donor. The histopathologic subgroups were epithelial in 5 patients, with others being of mixed type. Postoperative complications occurred in 3 patients as infection, intra-abdominal fluid collection, and acute rejection. The median follow-up was 32 months. One patient died because of lung metastasis within 9 months after transplant. CONCLUSIONS: Centers should offer liver transplant to patients with centrally located tumors. For centers that have an insufficient number of deceased donors, living-donor liver transplant with optimal planning and early treatment can be performed.

Diaphragmatic Hernia After Liver Transplant in Children: Report of 2 Cases.

OBJECTIVES: Diaphragmatic hernia is a rare complication after pediatric liver transplant. This report presents occurrences of diaphragmatic hernia after living-donor liver transplants in 2 children. MATERIALS AND METHODS: In 1 of the 2 patients, a right-sided diaphragmatic hernia developed after a living-donor liver transplant due to progressive familial intrahepatic cholestasis where a left lateral segment graft was used. In the other patient, a left-sided diaphragmatic hernia developed after a living-donor liver transplant due to biliary atresia following Kasai portoenterostomy where a left lateral segment graft was used. RESULTS: After diaphragm repair, the postoperative course was uneventful and there were no recurrences. CONCLUSIONS: A literature review identified nearly 30 cases of diaphragmatic hernia following liver transplants; diaphragmatic hernia should be considered a potential surgical complication after liver transplant.

Argininosuccinic Aciduria-A Rare Indication for Liver Transplant: Report of Two Cases.

Argininosuccinic aciduria is a urea cycle disorder caused by an argininosuccinate lyase enzyme deficiency that ends with nitrogen accumulation as ammonia. Argininosuccinic aciduria patients are at risk for long-term complications including poor neurocognitive outcome, hepatic disease, and systemic hypertension despite strict pharmacologic and dietary therapy. As the liver is the principle site of activity of the urea cycle, it is logical that a liver transplant should be an option, with careful patient selection, even in the absence of cirrhosis. We present 2 pediatric argininosuccinic aciduria patients who underwent a living-donor liver transplant from their mothers. After the liver transplant, the general well-being of the patients and their quality of life improved significantly. Liver transplant should be an option for argininosuccinic aciduria patients to prevent further neurologic deterioration and improve the patient's quality of life.