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.

Normal Primary Graft Function Despite Early Hepatic Artery Thrombosis After Living-Donor Liver Transplant.

OBJECTIVES: Early hepatic artery thrombosis is rare but devastating in living-donor liver transplant recipients. In this study, our aim was to evaluate the results of all patients with early hepatic artery thrombosis at our center. MATERIALS AND METHODS: Between October 2004 and November 2015, 841 patients underwent liver transplant for end-stage liver disease at our center. All recipients with early hepatic artery thrombosis were identified and retrospectively analyzed. Early hepatic artery thrombosis was defined in our study as its occurrence within the first postoperative week. RESULTS: Early hepatic artery thrombosis was seen in 12 patients (1.8%). Seven of these 12 patients developed hepatic artery thrombosis on postoperative day 1 after intraoperative dissection of the hepatic artery with necessity of repeated reconstruction. However, the primary liver function of these patients was excellent, with nearly normal serum liver panel results. The other 5 patients were diagnosed after sudden significant increases of liver values followed by graft dysfunction within the first postoperative week. All patients were listed as high urgent and underwent retransplant. Two patients died perioperatively due to primary nonfunction, and 2 other patients died due to liver cirrhosis based on recurrent autoimmune hepatitis 29 and 106 months after retransplant. The remaining patients were alive with good liver function after a median time of 18 months (interquartile range, 13-45 mo). CONCLUSIONS: Early hepatic artery thrombosis is not always associated with graft dysfunction. Retransplant is still necessary due to ischemic cholangiopathy in the long-term follow-up. However, an elective retransplant, which could mean better outcomes, should be preferred instead of an emergency retransplant.

Prognostic Factors of Liver Transplantation for HCC: Comparative Literature Review.

PURPOSE: The aim of the review study is investigation of the prognostic factors of the liver transplantation for hepatocellular carcinoma. METHODS: A literature review has been made, especially in countries where dominantly living donor liver transplantation is performed, such as Turkey. Liver transplantation from deceased donor and from living donor has been evaluated about as advantages and disadvantages, and their effects on prognosis have been compared. In addition, hepatocellular carcinoma series of Koç University Liver Transplantation center has been presented. RESULTS: Liver transplantation is still the best treatment option with 5-year 50% survival rate for hepatocellular carcinoma even in patient who has locally advanced tumor. The patient's survival is not only an important issue but also the living donor's safety is controversial particularly when expectation of recipient's 5-year survival is below 50% due to donor complication. CONCLUSION: Detailed preoperative examination, appropriate patient selection, and timing of surgery are seen the most important issues in liver transplant's patients with hepatocellular carcinoma.

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.

The advantage of early liver transplantation for Wilson's disease using living donors.

AIM: The aim of the study was to investigate the surgical timing, results, and advantages of living-donor liver transplantation in patients who underwent liver transplantation due to Wilson's disease. MATERIAL AND METHODS: The study included Wilson's patients who underwent liver transplantation and their live donors. Demographic information, preparations for surgery, liver transplant type, grafts used, results, and complications were examined. RESULTS: Between 2006 and 2020, 29 liver transplants were performed for 27 Wilson's patients in our clinic. The study included 11 female and 16 male patients, with a mean age of 20.8 ±11.1 years and a mean body mass index of 20.5 ±3.2 kg/m2. The mean MELD score of the adult patients was 16.5 ±6.3, and the mean PELD score of the paediatric patients was 19.6 ±17.2. Five patients underwent transplantation due to acute liver failure, and 22 patients with low MELD score had liver transplants due to chronic liver disease. Three patients who were referred with acute liver failure died in the perioperative period; no mortality was observed in the 22 elective patients. The overall survival was calculated as 88.8%. The 1-, 3-, and 5-year survival were 100% among elective early transplanted patients. CONCLUSIONS: Liver transplant is the most effective treatment for liver failure caused by Wilson' s disease. When performed promptly, living-donor liver transplantation results in high survival rates in cases of both acute liver failure and chronic liver failure, and it no deterioration of the patient's condition is evident.

Living Donor Liver Transplantation: The Optimal Curative Treatment for Hepatocellular Carcinoma Even Beyond Milan Criteria.

INTRODUCTION: Liver transplantation offers the most reasonable expectation for curative treatment for hepatocellular carcinoma. Living-donor liver transplantation represents a treatment option, even in patients with extended Milan criteria. This study aimed to evaluate the outcomes of hepatocellular carcinoma patients, particularly those extended Milan criteria. MATERIALS AND PATIENTS: All HCC patients who received liver transplant for HCC were included in this retrospective study. Clinical characteristics including perioperative data and survival data (graft and patient) were extracted from records. Univariate and multivariate analyses was performed to identify significant prognostic factors for survival, postoperative complications and recurrence. RESULTS: Two-hundred and two patients were included. The median age was 54.8 years (IQR 53-61). Fifty-one patients (25.3%) underwent deceased donors liver transplantation and 151 patients (74.7%) underwent living donor liver transplantation. Perioperative mortality rate was 5.9% (12 patients). Recurrent disease occurred in 43 patients (21.2%). The overall 1-year and 5-year survival rates were 90.7% and 75.6%, respectively. Significant differences between patients beyond Milan criteria compared to those within Milan criteria were not found. Alpha-fetoprotein level >300 ng/mL, vascular invasion, and bilobar tumor lesions were independent negative prognostic factors for survival. CONCLUSION: Liver transplantation is the preferred treatment for hepatocellular carcinoma and it has demonstrated an excellent potential to cure even in patients with beyond Milan criteria. This study shows that the Milan criteria alone are not sufficient to predict survival after transplantation. The independent parameters for survival prediction are Alpha-Fetoprotein-value and status of vascular invasion.

Splenic Artery Transposition for Arterial Reconstruction in Living Donor Liver Transplantation.

PURPOSE: In living donor liver transplantation, poor compatibility of the recipient hepatic artery remains a technical challenge. Here, we analyzed our 14 years of experience with extra-anatomic hepatic artery reconstruction. METHODS: Between July 2004 and December 2018, there were 1063 liver transplantations at our center. All patients with an extra-anatomic hepatic artery reconstruction were identified. The gastroduodenal artery and the transposed splenic artery were the primary options for extra-anatomic arterial reconstruction. Patient characteristics, operative data, and post-transplant outcome were reviewed retrospectively. RESULTS: There were 22 patients with extra-anatomic hepatic artery reconstruction, 6 with gastroduodenal artery, and 16 with splenic artery. There were 2 major complications: 1 patient underwent early reoperation due to bleeding from the splenic artery trunk and another had an iatrogenic injury to the transposed splenic artery during conversion hepaticojejunostomy. Both were treated successfully with surgery. One patient died perioperatively due to sepsis. The 1- and 3-year graft survival rates of these 16 patients were 93.7% and 87.5%. CONCLUSION: If the hepatic arteries are not suitable for anastomosis, then we consider the gastroduodenal artery and the splenic artery to be the conduits of choice for extra-anatomic arterial reconstruction. The transposed splenic artery is very consistent, easily accessible, and offers adequate length and diameter for successful arterial anastomosis.

Giant Infantile Hepatic Hemangioma: Case Report and Surgical Technique.

Infantile hepatic hemangioma is the most common liver tumor in children. The most common symptoms are mass in her stomach, anemia and heart failure. According to the findings of the patient, the treatment may vary from the clinical follow-up to liver transplantation. In our study, the details of the surgical technique were presented. An 11-day-old newborn case with giant hepatic hemangioma causing postnatal respiratory arrest was presented in this study. Large and symptomatic patients with infantile hepatic hemangiomas who face us with different clinical behaviors are operated. It will be useful to share the surgical technique for these rarely seen cases for surgeons.

Comorbidity Index as a Selection Tool for Living Donor Liver Transplantation in Elderly Patients.

BACKGROUND: Previously published data have shown that age alone is not a contraindication for living donor liver transplantation (LDLT). However, careful evaluation is needed to identify the patients who are unlikely to benefit from LDLT. We hypothesized that the Charlson Comorbidity Index (CCI) could be used as one of the criteria for risk stratification in elderly patients undergoing LDLT. PATIENTS AND METHODS: There were 951 patients who underwent LDLT between October 2004 and February 2018. All recipients who were older than 60 years of age at the time of transplantation were identified. The comorbidity score was retrospectively assessed for each elderly patient according to the Charlson Comorbidity Index. Univariate and multivariate Cox regression analyses were performed to identify independent predictive factors for survival in elderly patients after LDLT. RESULTS: There were 96 patients (10.1%) in the age of > 60 years. All patients received the right lobe of their donor. Out of these patients, 18 (18.7%) died in the median time of 4 months. The remaining 78 patients (81.2%) are alive, with a median survival of 33 months. The CCI of these patients was significantly lower compared to the other 18 patients (2 versus 4). None of the patients with a CCI above 4 survived longer than 12 months. The results of the multivariate Cox regression analyses have shown that pulmonary disease, renal disease, and CCI are independent negative predictive factors for survival. CONCLUSION: The results of our study show clearly that the CCI has a significant influence on survival after LDLT in elderly patients and can be used as one of the selection criteria for LDLT in elderly patients.

Graft-to-recipient weight ratio threshold adjusted to the model for end-stage liver disease score for living donor liver transplantation.

The graft-to-recipient weight ratio (GRWR) is an important selection criterion for living donor liver transplantation (LDLT). The generally accepted threshold is known to be 0.8%. We believe that this threshold can be reduced under certain conditions. The aim of this study was to evaluate the results of these patients with GRWR < 0.8%. Between 2004 and 2015, 649 patients underwent right lobe LDLT for end-stage liver disease in adult patients. All recipients who had GRWR < 0.8% were identified. The data of these patients were retrospectively analyzed and compared to patients with GRWR ≥ 0.8%. There were 43 patients with GRWR < 0.8%. Out of these patients, 7 (16%) had GRWR of 0.6%. The median Model for End-Stage Liver Disease (MELD) score was 15, and the median donor age was 30 years. Anterior segment drainage was ensured. Portal inflow modulation was performed by splenic artery ligation according to the portal flow. Postoperative complications were seen in 6 (14%) patients. Of all 43 patients, 3 (7%) died perioperatively within 1 month, and 1 (2%) patient underwent retransplantation due to graft failure. The mean hospital stay was 18 days. The 1-year survival rate was 93%. None of the patients had a laboratory MELD score above 20. The comparison of the results with the patients who had GRWR ≥ 0.8% has shown no significant difference, except MELD score, body mass index (BMI), and rate of anterior segment drainage. The GRWR can be decreased even to 0.6% if the MELD score is below 20, donor age is below 45 years, and there are no signs for any hepatosteatosis of the donor graft. In these patients, it is essential that the anterior segment drainage is secured and the portal inflow modulation is performed according to the portal flow. Liver Transplantation 22 1643-1648 2016 AASLD.

Liver transplantation for homozygote familial hypercholesterolemia: the only curative treatment.

FH is an autosomal dominant genetic disorder characterized by increased TC and LDL level, which leads to xanthomas, atherosclerosis, and cardiac complications even in childhood. The treatment options are diet, medical treatment, lipid apheresis, and LT. The aim of our study was to analyze our data of patients with FH. Between 2004 and 2015, there were 51 patients who underwent pediatric LT at our center. All patients with FH were identified, and the data were retrospectively analyzed. There were eight patients with homozygous FH in the median age of 10 years (IQR 6-12) who underwent LT. The median pre-operative TC and LDL levels were 611 mg/dL (IQR: 460-844) and 574 mg/dL (IQR: 398-728) and decreased to normal levels 1 week after LT (TC: 193 mg/dL and LDL: 141 mg/dL). Two patients died two and 18 months after LT due to sudden cardiac arrest. Both patients were diagnosed with cardiovascular disease pre-operatively. The LT is the only curative treatment for this disease. To achieve an excellent outcome, it should be performed before the development of cardiovascular disease, because the regression of severe cardiovascular disease after transplantation is limited.

Total laparoscopic donor nephrectomy in situs inversus totalis: a case report.

Situs inversus totalis is a rare anomaly characterized by the total inversion of all abdominal and thoracic organs. For the first time, we report a case of a donor nephrectomy in a patient with situs inversus totalis, completed with a full laparoscopic approach. At the time of this writing, the donor and the recipient are doing well after 6 month's follow-up. Our experience shows that patients with situs inversus totalis may be eligible candidates for laparoscopic donor nephrectomy, provided that detailed preoperative imaging studies as well as precise preoperative planning are performed before the transplant.

Cystic pancreatic lymphangioma.

Lymphangioma of the pancreas is a rare benign tumor of lymphatic origin. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas. Herein, we report a case of cystic pancreatic lymphangioma diagnosed in 34 year-old female patient who was hospitalized for a slight pain in the epigastrium and vomiting. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum. During the laparoscopic surgery, a well circumscribed polycystic tumor was completely excised preserving the pancreatic duct. The patient made a complete recovery and is disease-free 12 months postoperatively.

Management of esophageal perforation secondary to caustic esophageal injury in children.

PURPOSE: To review our management of esophageal perforation in children with caustic esophageal injury. METHOD: We reviewed the medical records of 22 children treated for esophageal perforations that occurred secondary to caustic esophageal injury. RESULTS: There were 18 boys and 4 girls (mean age, 5 years; range, 2-12 years). Three children were treated for perforation during diagnostic endoscopy and 19 were treated for a collective 21 episodes of perforation during balloon dilatation. One child died after undergoing emergency surgery for tracheoesophageal fistula and pneumoperitoneum. Another patient underwent esophagostomy and gastrostomy. Twenty patients were treated conservatively with a nasogastric tube, broad spectrum antibiotics, and tube thoracostomy, 16 of whom responded but 4 required esophagostomy and gastrostomy. Although the perforation healed in 21 patients, 20 were left with a stricture. Two children were lost to follow-up, 8 underwent colonic interposition, and 10 continued to receive periodic balloon dilatations. Two of these 10 patients underwent colonic interposition after a second perforation. The other 8 became resistant to dilatations: 4 were treated by colon interposition; 2, by resection and anastomosis; and 2, by an esophageal stent. CONCLUSIONS: Esophageal perforation can be managed conservatively. Because strictures tend to become resistant to balloon dilatation, resection and anastomosis is preferred if they are up to 1 cm in length, otherwise colonic interposition is indicated.