Successful Anesthetic Management of an Adult Patient With Glycogen Storage Disease Type 1 During Liver Transplant: A Case Report.

Glycogen storage disease type 1 is a congenital abnormality of metabolism caused by the deficiency of the glucose-6-phosphatase enzyme, essential in glucose homeostasis. Patients with this disease are at high risk of developing hypoglycemia, hyperlipidemia, lactic acidemia, growth retardation, neutropenia, inflammatory bowel disease, and many other severe complications, such as hepatic adenomas converting into hepatocellular carcinomas. To prevent these complications, a liver transplant is the ultimate method of treatment. We present the successful anesthesia management for a 21-year-old man who had gross hepatomegaly, severe hypoglycemia, and hyperlactatemia and who received a liver transplant from his mother, which is a substantial challenge for anesthesiologists. Anesthesiologists should know the underlying pathophysiological condition and perform a comprehensive preoperative evaluation to determine the correct anesthesia plan in patients with glycogen storage disease type 1 who will undergo an orthotopic liver transplant due to multiple system disorders. Successful perioperative management of patients with glycogen storage disease type 1 relies on effective communication and collaboration between specialists through a multidisciplinary team approach.

Perioperative management in pediatric domino liver transplantation for metabolic disorders: A narrative review.

Domino liver transplantation and domino-auxiliary partial orthotopic liver transplantation are emerging techniques that can expand the liver donor pool and provide hope for children with liver disease. The innovative technique of domino liver transplantation has emerged as a pioneering strategy, capitalizing on structurally preserved livers from donors exhibiting single enzymatic defects within a morphologically normal context, effectively broadening the donor pool. Concurrently, the increasingly prevalent domino-auxiliary partial orthotopic liver transplantation method assumes a critical role in bolstering available donor resources. These advanced transplantation methods present a unique opportunity for pediatric patients who, despite having structurally and functionally intact livers and lacking early signs of portal hypertension or extrahepatic involvement, do not attain priority on conventional transplant lists. Utilizing optimal clinical conditions enhances posttransplant outcomes, benefiting patients who would otherwise endure extended waiting periods for traditional transplantation. The perioperative management of children undergoing these procedures is complex and requires careful consideration of some factors, including clinical and metabolic conditions of the specific metabolic disorder, and the need for tailored perioperative management planning. Furthermore, the prudent consideration of de novo disease development in the recipient assumes paramount significance when selecting suitable donors for domino liver transplantation, as it profoundly influences prognosis, mortality, and morbidity. This narrative review of domino liver transplantation will discuss the pathophysiology, clinical evaluation, perioperative management, and prognostic expectations, focusing on perioperative anesthetic considerations for children undergoing domino liver transplantation.

A gross abdominal wall hematoma secondary to transversus abdominis plane block.

We aimed to share our experience with an abdominal wall hematoma that developed after an ultrasonography-guided TAP block performed for the palliation of chronic abdominal wall pain. Bleeding was successfully stopped with coil embolization.

Successful Anesthesia Management of Pediatric Living Donor Liver Transplant With Mild Bilateral Pulmonary Artery Stenosis Due to Alagille Syndrome: A Case Report.

Alagille syndrome is an autosomal-dominantinherited disease characterized by intrahepatic bile duct involvement, congenital heart disease, eye anomalies, skeletal and central nervous system involvement, kidney anomalies, and facial appearance. Liver transplant is the only treatment option for patients with end-stage liver disease and Alagille syndrome. Bilateral peripheral pulmonary artery stenosis is a contraindication for liver transplant due to high mortality, and the decision for liver transplant in patients with bilateral peripheral pulmonary artery stenosis is extremely challenging for anesthesiologists andtransplant surgeons.Wepresent a 2-year-oldfemale patient with successful anesthetic management of a pediatric living donor liver transplant with mild bilateral pulmonary artery stenosis, mild aortic stenosis, and mitral regurgitation due to Alagille syndrome. Anesthesiologists should know the underlying pathophysiological condition and perform a comprehensive preoperative evaluation to determine the correct anesthesia plan in patients with Alagille syndrome who will undergo liver transplants to treat multiple system disorders. Successful perioperative management of Alagille syndrome requires effective communication and collaboration between specialists through a multidisciplinary team approach.

Expected Life Expectancy on Liver Transplantation and Aging Donor.

In this study, our aim was to show the life expectancy according to donor age groups at 1, 3, 5, 10, 15, and 20 years after liver transplant in liver transplant recipients. In this retrospective study, we analyzed the survival rate of 236 patients who had liver transplant procedures between 1988 and 2021. The 5-year life expectancy of recipients with donors over age 50 years in the literature has been shown to vary between 50% and 80%. Little information could be found on life expectancy after 10, 15, and 20 years in other studies. In the studies from Haberal and colleagues, life expectancy at 10, 15, and 20 years was 49%, 42%, and 42%, respectively. This study presents an evidence-based example of the use of elderly donors to enlarge the donor pool.

Relationship Between Perioperative Factors and Splenic Artery Steal Syndrome After Orthotopic Liver Transplant: A Retrospective Clinical Study.

OBJECTIVES: After orthotopic liver transplant, ischemia of biliary tract and graft loss may occur due to impaired hepatic arterial blood flow. This situation with hypersplenism and impaired hepatic arterial blood flowis defined as splenic artery steal syndrome.The aim of this study was to investigate the relationship between perioperative factors and splenic artery steal syndrome in orthotopic liver transplant patients. MATERIALS AND METHODS: Forty-five patients who underwent orthotopic liver transplant between 2014 and 2022 were included in the study. The data for the patients were obtained from the hospital database, including the intraoperative anesthesiology and postoperative intensive care records. RESULTS: Eleven patients were diagnosed with splenic artery steal syndrome. Patients with splenic artery steal syndrome had higher need for intraoperative vasopressor agents (P = .016) and exhibited lower intraoperative urine output (P = .031). In the postoperative intensive care follow-up, patients with splenic artery steal syndrome had higher levels of C-reactive protein during the first 48 hours (P = .030). CONCLUSIONS: Intraoperative administration of vasopressor drugs, low urine output, and early postoperative high C-reactive protein levels were associated with the development of splenic artery steal syndrome in patients undergoing orthotopic liver transplant. Future studies should focus on investigation of biomarkers associated systemic hypoperfusion that may contribute to the development of splenic artery steal syndrome.

Immediate Tracheal Extubation After Pediatric Liver Transplantation.

OBJECTIVES: We examined whether immediate tracheal extubation among pediatric liver transplant recipients was safe and feasible. MATERIALS AND METHODS: We retrospectively analyzed medical records of pediatric liver transplant recipients at Baskent University Hospital from January 2012 to December 2017. We grouped children who were extubated in the operating room versus those extubated in the intensive care unit. RESULTS: In our study group of 81 pediatric patients, median age was 4 years (range, 4 mo to 16 y) and 44 (54%) were male. Immediate tracheal extubation in the operating room was performed in 39 patients (48%). Children who remained intubated (n = 42) had more frequent massive hemorrhage (14% vs 0%; P = .015), received larger amounts of packed red blood cells (19.3 vs 10.2 mL/kg; P < .001), and had higher serum lactate levels (9.0 vs 6.9 mmol/L; P = .001) intraoperatively. All children with open abdomens postoperatively remained intubated (n = 7). Patients extubated in the operating room received less vasopressors (1 [3%] vs 12 [29%]; P = .002) and antibiotics (11 [28%] vs 22 [52%]; P = 0.041) and developed infections less frequently postoperatively (3.0 [8%] vs 15.0 [36%]; P = .003). Children extubated in the operating room had shorter mean stay in the intensive care unit (2.0 vs 4.5 days; P < .001). Hospital mortality was higher in children who remained intubated (12% vs 0%; P = .026). CONCLUSIONS: Immediate tracheal extubation was well tolerated in almost half of our patients and did not compromise their outcomes. Patients who remained intubated had longer intensive care unit stays and higher hospital mortalities. Therefore, we recommend immediate tracheal extubation in the operating room after pediatric liver transplant among those children without intraoperative requirements for massive blood transfusion, high-dose vasopressors, high serum lactate levels, and open abdomen.

Risk Factors for Postoperative Prolonged Mechanical Ventilation After Pediatric Liver Transplantation.

OBJECTIVES: Duration of postoperative mechanical ventilation after pediatric liver transplant may influence pulmonary functions, and postoperative prolonged mechanical ventilation is associated with higher morbidity and mortality. Here, we determined its incidence and risk factors after pediatric liver transplant at our center. MATERIALS AND METHODS: We retrospectively analyzed the records of 121 children who underwent liver transplant between April 2007 and April 2017 (305 total liver transplant procedures were performed during this period). Prolonged mechanical ventilation was defined as postoperative tracheal extubation after 24 hours. RESULTS: Mean age at transplant was 6.2 ± 5.4 years and 71/121 children (58.7%) were male. Immediate tracheal extubation was achieved in 68 children (56.2%). Postoperative prolonged mechanical ventilation was needed in 12 children (9.9%), with mean extubation time of 78.0 ± 83.4 hours. Reintubation was required in 13.4%. Logistic regression analysis revealed that presence of preoperative hepatic encephalopathy (odds ratio of 0.130; 95% confidence interval, 0.027-0.615; P = .01), high aspartate amino transferase levels (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P = .02), intraoperative usage of more packed red blood cells (odds ratio of 1.001; 95% confidence interval, 1.000-1.002; P = .04), and longer surgery duration (odds ratio of 0.723; 95% confidence interval, 0.555-0.940, P = .01) were independent risk factors for postoperative prolonged mechanical venti-lation. Although mean length of intensive care unit stay was significantly longer (12.6 ± 13.6 vs 6.0 ± 0.6 days; P = .001), mortality was similar in children with and without postoperative prolonged mechanical ventilation. CONCLUSIONS: Our results indicate that postoperative prolonged mechanical ventilation was needed in 9.9% of our children. Predictors of postoperative prolonged mechanical ventilation after pediatric liver transplant at our center were preoperative presence of hepatic encephalopathy, high aspartate amino transferase levels, intraoperative usage of more packed red blood cells, and longer surgery duration.

The New Anterior Less Invasive Crescentic Incision for Living Donor Nephrectomy.

OBJECTIVES: Living-donor nephrectomy is a devoted procedure performed in a healthy individual; for these procedures, it is essential to complete the surgery with the lowest possible risk and morbidity and allow donors to regain their normal daily activity. To minimize anatomic and physiologic damage, we modified a surgical technique. Here, we report our experiences with the new anterior less invasive crescentic donor nephrectomy technique. MATERIALS AND METHODS: We retrospectively evaluated 728 donor nephrectomy patients who had the new anterior less invasive cresentic incision (n = 224), the classic open (n = 431), or the laparoscopic living-donor nephrectomy (n = 73) procedures. Demographic characteristics, preoperative and postoperative parameters, acute renal graft dysfunction, and firstyear graft and patient survival rates were compared between groups. RESULTS: During the operation, the new cresentic incision living-donor nephrectomy allowed a safe and comfortable position for the patient and the anesthesiologist. Also, it procures safe access especially for grefts with multiple vessels. Patients had lower pain scores (P = .010), shorter hospital stays (2.25 vs 3.49 days) than those who received the classic open living-donor nephrectomy. Patients who received laparoscopic living-donor nephrectomy had significantly longer mean operation time (P = .016) and warm ischemia time (P ≤ .001) than those who had the new cresentic incision technique. All groups showed similar rates of first-year survival and delayed graft dysfunction. CONCLUSIONS: The new anterior less invasive cresentic incision open-donor nephrectomy approach is a safe, comfortable, effective, and less invasive modification of the living donor nephrectomy. Also, it procures safe access for grefts with multiple vessels.

Liver and Kidney Transplant During a 6-Month Period in the COVID-19 Pandemic: A Single-Center Experience.

OBJECTIVES: With the declaration of COVID-19 as a pandemic, many studies have indicated that elective surgeries should be postponed. However, postponement of transplants may cause diseases to get worse and increase the number in wait lists. We believe that, with precautions, transplant does not pose a risk during pandemic. Here, we aimed to evaluate our transplant results, which we safely performed during a 6-month pandemic period. MATERIALS AND METHODS: Until September 2020, 3140 kidney and 667 liver transplants have been performed in our centers. We evaluated 38 kidney transplants and 9 liver transplants procedures performed during the pandemic (March 1 to September 2, 2020). Recipient and donor candidates were screened for COVID-19 with polymerase chain reaction and thoracic computed tomography. All recipients had routine immunosuppressive protocol. During hospitalization at our COVID-19-free transplant facility, we restricted the interactions during multidisciplinary rounds. RESULTS: During the pandemic, 38 kidney transplants with an average length of hospital stay of 8.1 days were performed. Mean serum creatinine values of recipients were 0.91, 0.86, and 0.74 mg/dL on postoperative days 7, 30, and 90, respectively. During the pandemic, 9 living donor liver transplants (1 adult, 8 pediatric) were performed with an average length of hospital stay of 17.1 days. Mean serum total bilirubin levels were 0.9, 0.5, and 0.4 mg/dL on postoperative days 7, 30, and 90, respectively. Mean serum aspartate aminotransferase levels were 38.1, 28.3, and 22.3 U/L on postoperative days 7, 30, and 90, respectively. All recipients and donors were successfully discharged. Only 1 liver recipient died (on day 55 after discharge as a result of oxalosis-induced heart failure). CONCLUSIONS: According to our results, when precautions are taken, transplant does not pose a risk to patients during the pandemic period. We attribute the safety and success shown to our newly developed protocol in response to the COVID-19 pandemic.

Predictors of Immediate Tracheal Extubation in the Operating Room After Pediatric Liver Transplantation.

OBJECTIVES: Pediatric orthotopic liver transplant recipients frequently need mechanical ventilation during the immediate posttransplant period. However, intensive care unit beds are costly and scarce; therefore, anticipating which patients will require postoperative mechanical ventilation support is important. In addition, immediate postoperative extubation may reduce the incidence of postoperative respiratory complications and improve patient outcomes after orthotopic liver transplant. Here, we aimed to determine the predictors of need for mechanical ventilation after orthotopic liver transplant in pediatric patients. MATERIALS AND METHODS: We retrospectively analyzed the records of 57 pediatric patients who underwent orthotopic liver transplant (performed by the same team at Baskent University Hospital from April 1996 to August 2009). Patients were divided into 2 groups according to whether they required postoperative mechanical ventilation or not. Collected data included demographic features; comorbidities; cause of liver failure; perioperative laboratory values; intraoperative hemodynamic parameters; use and volume of crystalloids, colloids, and blood products; albumin levels; portal vein clamping time, requirement of inotropes/vasopressors; and anesthesia duration. RESULTS: Mean age and body weight of patients were 25.0 ± 23.1 months and 10.8 ± 5.3 kg, respectively. Of 57 patients, 26 (46%) needed postoperative mechanical ventilation. Compared with those who did not require postoperative mechanical ventilation, patients who required mechanical ventilation had growth failure (P = .03), higher mean intraoperative lactate level (P = .03), and higher mean intraoperative fresh frozen plasma/erythrocyte suspension (P = .049) and intraoperative vasopressor (P = .022) requirements. Multivariate logistic regression analysis revealed that growth failure (odds ratio = 37; P = .03) and higher intraoperative lactate level (odds ratio = 1.5; P = .03) were predictors of the need for mechanical ventilation. CONCLUSIONS: About 46% of our pediatric orthotopic liver transplant recipients required mechanical ventilation postoperatively. Growth failure and higher intraoperative lactate levels were associated with need for postoperative mechanical ventilation.

Temporary Abdominal Closure Technique After Pediatric Liver Transplant: Single-Center Experience.

OBJECTIVES: Primary abdominal wound closure is performed at the completion of liver transplant in most patients. However, this is not always possible in pediatric recipients. The shortage of size-matched donor organs for pediatric patients means that occasionally it is necessary to use whole livers that are larger than can be accommodated comfortably in the child's abdomen. The present report outlines our experience with temporary patch closure after pediatric liver transplant of the abdominal wall. MATERIALS AND METHODS: Our team performed the first liver transplant in Turkey in 1988. Since 1988, we have performed 629 liver transplant (336 adult and 293 pediatric) procedures at our center. We evaluated data of 191 liver transplants performed in recipients who were under 10 years of age. Left lateral lobe grafts were used in 169 patients (88%), and whole grafts were used in 22 patients (12%). RESULTS: Temporary closure with the Bogota bag patch was necessary in 31 transplant procedures (16.2%), 3 of which involved whole livers and 28 of which involved left lateral lobe grafts. The age range of recipients was 5 months to 10 years (median, 30 mo). The temporary abdominal closure technique was preferred in 22 patients because the abdomen could not be closed during surgery. CONCLUSIONS: In pediatric patients with difficult abdominal closure after liver transplant, temporary patch closure is the treatment of choice. Our preference has been reinforced silicone sheeting, which allows minimal adhesion formation between the patch and abdominal viscera; in addition, the transparent nature of the material provides a window for inspection of the donor liver.

Efficacy of Levetiracetam for Epilepsy in Pediatric Liver Transplant Recipients With Posterior Reversible Encephalopathy Syndrome.

OBJECTIVES: Liver transplant is currently the most effective option for patients with end-stage liver disease. Seizures are the most common neurologic complication after liver transplant. Posterior reversible encephalopathy syndrome is a neurologic syndrome characterized by lethargy, seizures, visual disturbances, and radiologic findings of edema in the posterior regions of the cerebral hemispheres. Levetiracetam is prescribed for a broad spectrum of seizure types but does not have a specific indication for epilepsy in children after solid-organ transplant. Our aim was to investigate the efficacy and tolerability of levetiracetam in pediatric transplant recipients with posterior reversible encephalopathy syndrome and epilepsy. MATERIALS AND METHODS: We reviewed records of patients treated for epilepsy due to posterior reversible encephalopathy syndrome after liver transplant seen at our pediatric neurology clinic between January 2010 and March 2019. Patients were assessed clinically and by neurologic examination, electroencephalography, and cerebral magnetic resonance imaging. RESULTS: Among 134 children who had undergone liver transplant between 2010 and 2019, 10 patients (6 males, 4 females; age range,7-19 y) who were diag-nosed with posterior reversible encephalopathy syndrome and epilepsy were included in the study. All patients received levetiracetam at 20 mg/kg/day. After a mean follow-up of 28.9 months (range, 24-40 mo), 9 patients (90%) attained complete seizure freedom. One patient who had an underlying neurodegenerative disease (hemophagocytic syndrome) other than posterior reversible encephalopathy syndrome continued to have seizures under levetiracetam treatment. One patient had a mild adverse reaction (irritability) due to levetiracetam but did not require drug discontinuation. CONCLUSIONS: In this study, 90% of patients with posterior reversible encephalopathy syndrome became seizure free with levetiracetam treatment. Our findings suggest that levetiracetam has a favorable efficacy for epilepsy due to posterior reversible encephalopathy syndrome in pediatric liver transplant recipients with tolerable adverse effects.

Incidence of Urinary Complications With Double J Stents in Kidney Transplantation.

OBJECTIVES: Ureteral complications remain a major source of morbidity and occasional mortality in renal transplant. Among all ureteral complications, leaks are the most frequently encountered in the early posttransplant period. The routine use of a double-J ureteric stent remains controversial, with reported increased incidence of urinary tract infection. Here, we retrospectively compared the efficacy of a double J stent in kidney transplant patients to investigate ureteral complication incidence in our center. MATERIALS AND METHODS: Our study included 382 kidney transplant patients. At 5 weeks after transplant, the double J stent was removed under sedation. Patients were divided into 2 groups: 125 patients with double J stent placement (group 1) and 257 patients without double J stent placement (group 2). RESULTS: We observed no significant demographic differences between the 2 groups with regard to patient age (median patient age of 30 y [range, 2-73 y] for group 1; median patient age of 33 y [range, 4-69 y] for group 2), patient sex (30.2% females in group 1, 32.4% females in group 2), and body mass index (median of 25.1 vs 24.9 kg/m2 in groups 1 and 2, respectively). Cold and warm ischemia time for donor organ, delayed graft function, and episodes of acute rejection did not differ significantly between the groups. Urinary tract infection was observed in 25/125 (20.4%) and 50/257 patients (19.2%) in groups 1 and 2, respectively. Urinary leak was present in 8/125 group 1 (6.4%) and 6/257 group 2 patients (2.3%). CONCLUSIONS: A double J stent in ureteral anastomosis was not likely to decrease the frequency of leakage but is likely to reduce the gravity of the complication and the need for reoperation. In addition, the use of a double J stent was not associated with increased urinary tract infections in renal transplant recipients.

Liver Transplant in a Patient With Hemophagocytic Lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis is a rare and life-threatening systemic disease that can cause hepatic infiltration and present as acute liver failure. Here, we report a case of a 3-year-old pediatric patient who presented with acute liver failure and hepatic encephalopathy secondary to hemophagocytic lymphohistiocytosis. She had left lateral segment liver transplant from her father. After 27 months, she had bone marrow transplant from her sister. At the time of reporting (36 months after liver transplant), she showed normal liver function and blood peripheral counts. We found that liver transplant can be a curative treatment for this type of rare disorder, not only to improve the quality of life but also to prolong survival.

Technique of Ileobladder and Kidney Transplant in Rats and Pigs.

OBJECTIVES: Kidney transplant is the best choice for treatment of patients with advanced chronic renal disease. However, small, poorly compliant, and unstable bladders can result in major problems for patients. Here, we aimed to develop and evaluate a new ileobladder model. MATERIALS AND METHODS: Fifteen rats (250-300 g) and 5 pigs (~100 kg) were cared for according to institutional and published guidelines. After general anesthesia, laparotomy was done through midline incision. Ileal loops were prepared for ileobladder. After cystectomy (0.5 cm above the trigone in rats, 1 cm above the trigone in pigs), anastomoses were done between antimesenteric sides of ileal loops and bladder remnant with 6/0 Prolene suture. Three other pigs received simultaneous renal transplant. RESULTS: One rat died on day 1 postsurgery from multiorgan hemorrhage. Two rats survived for 5 days, 3 rats for 7 days, and 3 rats for 11 days; 6 rats were killed for pathologic evaluation after 3 months. One pig survived for 22 days and 1 for 9 days. Of the 3 pigs that received a simultaneous renal transplant, 2 pigs were alive and doing well 80 and 72 days after surgery with normal urinary discharge (1 pig was killed for pathologic evaluation after 3 days). When ileobladder was opened, complete recovery of the anastomosis line was observed. Pathologic examination of the anastomosis sites reported a normal healing process with moderate inflammation and the muscular wall of the intestine showed hypertrophia that nearly reached the size of the bladder muscularis propria. CONCLUSIONS: Although we had some complications because no draining procedure was used, in terms of technique, our new ileobladder model is promising for providing functional bladder volume. A larger scale series in the clinical setting is planned. This technique can be useful for small bladders and bladder physiology disorders.

Effect of Graft Weight to Recipient Body Weight Ratio on Hemodynamic and Metabolic Parameters in Pediatric Liver Transplant: A Retrospective Analysis.

OBJECTIVES: To analyze how graft-weight-to-bodyweight ratio in pediatric liver transplant affects intraoperative and early postoperative hemodynamic and metabolic parameters. MATERIALS AND METHODS: We reviewed data from 130 children who underwent liver transplant between 2005 and 2015. Recipients were divided into 2 groups: those with a graft weight to body weight ratio > 4% (large for size) and those with a ratio ≤ 4% (normal for size). Data included demographics, preoperative laboratory findings, intraoperative metabolic and hemodynamic parameters, and intensive care follow-up parameters. RESULTS: Patients in the large-graft-for-size group (>4%) received more colloid solution (57.7 ± 20.1 mL/kg vs 45.1 ± 21.9 mL/kg; P = .08) and higher doses of furosemide (0.7 ± 0.6 mg/kg vs 0.4 ± 0.7 mg/kg; P = .018). They had lower mean pH (7.1 ± 0.1 vs 7.2 ± 0.1; P = .004) and PO2 (115.4 ± 44.6 mm Hg vs 147.6 ± 49.3 mm Hg; P = .004) values, higher blood glucose values (352.8 ± 96.9 mg/dL vs 262.8 ± 88.2 mg/dL; P < .001), and lower mean body temperature (34.8 ± 0.7°C vs 35.2 ± 0.6°C; P = .016) during the neohepatic phase. They received more blood transfusions during both the anhepatic (30.3 ± 24.3 mL/kg vs 18.8 ± 21.8 mL/kg; P = .013) and neohepatic (17.7 ± 20.4 mL/kg vs 10.3 ± 15.5 mL/kg; P = .031) phases and more fresh frozen plasma (13.6 ± 17.6 mL/kg vs 6.2 ± 10.2 mL/kg; P = .012) during the neohepatic phase. They also were more likely to be hypotensive (P < .05) and to receive norepinephrine infusion more often (44% vs 22%; P < .05) intraoperatively. More patients in this group were mechanically ventilated in the intensive care unit (56% vs 31%; P = .035). There were no significant differences between the groups in postoperative acute renal dysfunction, graft rejection or loss, infections, length of intensive care stay, and mortality (P > .05). CONCLUSIONS: High graft weight-to-body-weight ratio is associated with adverse metabolic and hemodynamic changes during the intraoperative and early postoperative periods. These results emphasize the importance of using an appropriately sized graft in liver transplant.

Results of Biliary Reconstruction Using a Polytetrafluoroethylene Graft in Liver Transplant Patients.

OBJECTIVES: Biliary complications after liver transplant are a major concern with their high incidence, the need for repeated and long-term treatment, and their potential effects on graft and patient survival. We report our experience with biliary anastomosis using a spiral polytetrafluoroethylene graft. MATERIALS AND METHODS: Between December 8, 1988, and July 2016, we performed 538 liver transplant procedures. We used a spiral polytetrafluoroethylene graft for biliary anastomosis in 10 patients: for biliary stricture reconstruction after liver transplant in 4 patients and during the primary liver transplant in 6 patients. RESULTS: Four patients who underwent biliary stricture reconstruction are doing well, with normal liver function. Of the 6 patients who received the graft during primary liver transplant, 2 died from sepsis, although they maintained normal liver function. Of the 4 living patients, 1 had a biliary complication that was reconstructed surgically. The 4 living patients are currently doing well, with normal liver function. CONCLUSIONS: Our small series of patients shows that the use of a spiral polytetrafluoroethylene graft is effective at reducing biliary complications in transplant patients.

Auxiliary Partial Orthotopic Living Liver Transplant for Wilson Disease.

Wilson disease is a genetic disease involving copper metabolism disturbances that result in copper accumulations, especially in the liver and brain. Wilson disease can be treated with pharmacologic agents, such as chelators that induce urinary excretion of copper or zinc salts that inhibit copper absorption in the digestive tract. Liver transplant is the only treatment option for Wilson disease when liver failure has occurred. In some patients, that is, in those with Child-Pugh A score, neurologic disease can be seen without hepatic failure. Our recommendation is for these patients to have auxiliary partial orthotopic liver transplant. Here, we present a 36-year-old male patient with neurologic disease associated with Wilson disease who had successful related living-donor auxiliary partial orthotopic liver transplant using a left lobe. The patient, as a result of neurologic symptoms that included tremor walking and speaking problems and low serum ceruloplasmin level of 7 mg/dL, was diagnosed with Wilson disease, and a liver biopsy was performed. Chronic necroinflammatory disease activity was 4/18, and the patient received chelation treatment. His hepatic functions were normal. The donor was the patient's 57-year-old father whose liver function tests were also normal. The graft-to-recipient weight ratio was 1% using a left lobe graft. After transplant, serum ceruloplasmin levels on day 15 and month 1 were 14 and 19 mg/dL. At month 1, liver function tests were normal. Doppler ultrasonography showed normal vascular flow of the native liver and the graft. The patient's neurologic symptoms were progressively reduced. Progressive neurologic deterioration with no hepatic insufficiency is considered a suitable indication for auxiliary partial orthotopic liver transplant; this procedure is suggested before the neurologic and liver failure symptoms of Wilson disease occur.