Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort.

BACKGROUND: Biliary atresia (BA) is the most common indication for pediatric liver trans plantation, although portoenterostomy is usually performed first. However, due to the high failure rate of portoenterostomy, liver transplantation has been advocated as the primary procedure for patients with BA. It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes. AIM: To investigate the effect of prior portoenterostomy in infants un dergoing liver transplantation for BA. METHODS: This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil. Patients with BA were divided into two groups: Those under going primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy. Continuous variables were compared using the Student's t-test or the Kruskal-Wallis test, and categorical variables were compared using the χ2 or Fisher's exact test, as appropriate. Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis. Patient and graft survival analyses were conducted with the Kaplan-Meier product-limit estimator, and patient subgroups were compared using the two-sided log-rank test. RESULTS: Forty-two patients were included in the study (25 [60%] girls), 23 undergoing liver transplantation without prior portoenterostomy, and 19 undergoing liver transplantation with prior portoenterostomy. Patients with prior portoenterostomy were older (12 vs 8 months; P = 0.02) at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores (13.2 vs 21.4; P = 0.01). The majority of the patients (35/42, 83%) underwent living-donor liver transplantation. The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis (39 vs 11%), but this result did not reach statistical significance. Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation, graft-to-recipient weight ratio, and use of vascular grafts. Finally, the groups did not significantly differ in terms of post-transplant survival. CONCLUSION: In our study, prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

Hepatic venous reconstruction of the left lateral segment with emphasis on anomalous hepatic vein in pediatric liver transplantation.

Left lateral segment grafts have become a suitable option in pediatric liver transplantation (PLT). The correlation between hepatic vein (HV) reconstruction and outcome is relevant when assessing the safe use of these grafts. We retrospectively reviewed the medical records prospectively collected from a pediatric living donor liver transplantation database and conducted a comparative analysis of the different left lateral segment graft types according to HV reconstruction. Donor, recipient, and intraoperative variables were analyzed. Post-transplant outcomes included vascular complications such as hepatic vein outflow obstruction, early (≤30 d) and late (>30 d) PVT, hepatic artery thrombosis, and graft survival. From February 2017 to August 2021, 303 PLTs were performed. According to venous anatomy, the distribution of the left lateral segment was as follows: single HV (type I) in 174 (57.4%), close HVs, simple venoplasty for reconstruction (type II) in 97 (32.01%), anomalous hepatic vein (AHV) with a distance between the HVs orifices that allowed simple venoplasty (type IIIA) in 25 (8.26%) and AHV with a distance between the HVs orifices requiring homologous venous graft interposition (type IIIB) in 07 (2.31%) grafts. Type IIIB grafts came from male donors ( p =0.04) and had a higher mean donor height ( p =0.008), a higher mean graft weight, and a higher graft-to-recipient weight ratio, both p =0.002. The median follow-up time was 41.4 months. The overall cumulative graft survival was 96.3%, and comparative graft survival showed no difference (log-rank p =0.61). No hepatic vein outflow obstructions were observed in this cohort study. There was no statistically significant difference in the post-transplant outcomes between the graft types. The venous reconstruction of the AHV with homologous venous graft interposition had similar outcomes in the short and long term.

A comparison between ultrasonography and single-phase computed tomography for preoperative assessment of solid abdominal tumors in children

Abstract Objective: This study aimed to estimate the performance of single-phase-enhanced computed tomography and ultrasonography examinations in the preoperative evaluation of solid abdominal tumors and their relationship with relevant adjacent structures in children. Methods: This retrospective study included 50 pediatric patients with malignant solid abdominal tumors treated with surgical resection between 2009-2017. Preoperative computed tomography and ultrasonography were compared to operative findings (gold standard) in the diagnosis of invasion or encasement of adjacent structures. Accuracy, sensitivity, specificity, and positive and negative predictive values were evaluated. Results: Renal (20.4%) and neuroblastic (19.4%) tumors were the most common. Complete surgical resection with negative margins was achieved in 44 (88%) patients. The comparison between single-phase-enhanced computed tomography and ultrasonography findings showed the following results: sensitivity = 90.3% vs 86.6%, specificity = 86.8% vs 94.6%, negative predictive value = 95.3% vs 94.4%, positive predictive value = 75.3% vs 86.9%, and accuracy = 87.9% vs 92.2%. The correlation (kappa index) between computed tomography and ultrasonography examinations was 0.72 (p < 0.001). In 14% (7/50) of the patients, the invasion of adjacent structures was diagnosed by ultrasonography but not by computed tomography (1 patient had 2 invaded structures).

A comparison between ultrasonography and single-phase computed tomography for preoperative assessment of solid abdominal tumors in children.

OBJECTIVE: This study aimed to estimate the performance of single-phase-enhanced computed tomography and ultrasonography examinations in the preoperative evaluation of solid abdominal tumors and their relationship with relevant adjacent structures in children. METHODS: This retrospective study included 50 pediatric patients with malignant solid abdominal tumors treated with surgical resection between 2009-2017. Preoperative computed tomography and ultrasonography were compared to operative findings (gold standard) in the diagnosis of invasion or encasement of adjacent structures. Accuracy, sensitivity, specificity, and positive and negative predictive values were evaluated. RESULTS: Renal (20.4%) and neuroblastic (19.4%) tumors were the most common. Complete surgical resection with negative margins was achieved in 44 (88%) patients. The comparison between single-phase-enhanced computed tomography and ultrasonography findings showed the following results: sensitivity = 90.3% vs 86.6%, specificity = 86.8% vs 94.6%, negative predictive value = 95.3% vs 94.4%, positive predictive value = 75.3% vs 86.9%, and accuracy = 87.9% vs 92.2%. The correlation (kappa index) between computed tomography and ultrasonography examinations was 0.72 (p < 0.001). In 14% (7/50) of the patients, the invasion of adjacent structures was diagnosed by ultrasonography but not by computed tomography (1 patient had 2 invaded structures). CONCLUSION: Ultrasonography can be considered a complementary method to single-phase-enhanced computed tomography in the preoperative evaluation of children with an abdominal tumor. The present study showed that ultrasonography and single-phase-enhanced computed tomography each possess a high accuracy in the preoperative planning of resection of solid abdominal tumors in children. Thus, it seems that the combination of both imaging methods would be enough for the evaluation of most abdominal tumors in the pediatric population.

Liver volumetric and anatomic assessment in living donor liver transplantation: The role of modern imaging and artificial intelligence.

The shortage of deceased donor organs has prompted the development of alternative liver grafts for transplantation. Living-donor liver transplantation (LDLT) has emerged as a viable option, expanding the donor pool and enabling timely transplantation with favorable graft function and improved long-term outcomes. An accurate evaluation of the donor liver's volumetry (LV) and anatomical study is crucial to ensure adequate future liver remnant, graft volume and precise liver resection. Thus, ensuring donor safety and an appropriate graft-to-recipient weight ratio. Manual LV (MLV) using computed tomography has traditionally been considered the gold standard for assessing liver volume. However, the method has been limited by cost, subjectivity, and variability. Automated LV techniques employing advanced segmentation algorithms offer improved reproducibility, reduced variability, and enhanced efficiency compared to manual measurements. However, the accuracy of automated LV requires further investigation. The study provides a comprehensive review of traditional and emerging LV methods, including semi-automated image processing, automated LV techniques, and machine learning-based approaches. Additionally, the study discusses the respective strengths and weaknesses of each of the aforementioned techniques. The use of artificial intelligence (AI) technologies, including machine learning and deep learning, is expected to become a routine part of surgical planning in the near future. The implementation of AI is expected to enable faster and more accurate image study interpretations, improve workflow efficiency, and enhance the safety, speed, and cost-effectiveness of the procedures. Accurate preoperative assessment of the liver plays a crucial role in ensuring safe donor selection and improved outcomes in LDLT. MLV has inherent limitations that have led to the adoption of semi-automated and automated software solutions. Moreover, AI has tremendous potential for LV and segmentation; however, its widespread use is hindered by cost and availability. Therefore, the integration of multiple specialties is necessary to embrace technology and explore its possibilities, ranging from patient counseling to intraoperative decision-making through automation and AI.

EARLY INCISIONAL HERNIA AFTER LIVER TRANSPLANTATION: RISK FACTORS AND HERNIA REPAIR RESULTS.

BACKGROUND: Liver transplantation is a complex and valuable therapy. However, complications that burden postoperative quality of life, such as incisional hernia, are to be better elucidated, such as risk factors and prophylactic measures. AIM: This study aimed to define the rate of incisional hernia in patients who underwent liver transplantation in a population in southern Brazil and to assess the related risk factors in order to establish measures for prior optimization and specific prophylactic care in the future. METHODS: Patients undergoing adult Liver transplantation from January 2004 to November 2020 were retrospectively analyzed, assessing demographic features, surgical outcomes, and predisposing factors. RESULTS: Among 261 liver transplantation patients included, incisional hernia was diagnosed in 71 (27.2%). Of the 71 incisional hernia patients, 28 (39.4%) developed IH during the first post-transplant. Majority of the patients were male (52/71, 73.2%); of the 71 patients, 52 had hepatitis C virus (HCV) and 33 (46.5%) had hepatocellular carcinoma (HCC). Male gender (p=0.044), diabetes mellitus (p=0.008), and acute cellular rejection (p<0.001) were risk factors for IH. In all, 28 (39.4%) patients were submitted for hernia repair with mesh, with a recurrence rate of 17.8%. CONCLUSION: Incisional hernia after liver transplantation is a relatively common problem associated with male gender, diabetes, and acute cellular rejection. This is a problem that should not be trivialized in view of the complexity of liver transplantation, as it can lead to a reduction in quality of life as well as jeopardize late liver transplantation results and lead to incarceration and strangulation.

Severe hepatopulmonary syndrome with hypoxemia refractory to liver transplant: Recovery after 67 days of ECMO support.

Hepatopulmonary syndrome (HPS) is a complication of end stage liver disease (ESLD) and is manifested by severe hypoxemia, which usually responds to liver transplantation (LT). As compared to patients undergoing LT for other etiologies, patients with HPS present an increased risk of postoperative morbidity and mortality. There is no effective treatment for patients whose hypoxemia does not respond to LT. This subset of patients is at a highly increased risk of death. There are very few reports on the use of extracorporeal membrane oxygenation (ECMO) in this setting with rapid response. However, there is no prior report of ECMO utilization for longer than 4 weeks. We present the case of a 17 year-old male patient who underwent LT for ESLD secondary to chronic portal vein thrombosis and HPS. He received a liver from a deceased donor and presented with severe HPS after LT, requiring ECMO support for 67 days. The patient was discharged home and is breathing in ambient air. He is currently asymptomatic and has a normal liver function.

Early incisional hernia after liver transplantation: risk factors and hernia repair results / Hérnia incisional precoce após transplante hepático: fatores de risco e resultados do reparo cirúrgico

ABSTRACT BACKGROUND: Liver transplantation is a complex and valuable therapy. However, complications that burden postoperative quality of life, such as incisional hernia, are to be better elucidated, such as risk factors and prophylactic measures. AIM: This study aimed to define the rate of incisional hernia in patients who underwent liver transplantation in a population in southern Brazil and to assess the related risk factors in order to establish measures for prior optimization and specific prophylactic care in the future. METHODS: Patients undergoing adult Liver transplantation from January 2004 to November 2020 were retrospectively analyzed, assessing demographic features, surgical outcomes, and predisposing factors. RESULTS: Among 261 liver transplantation patients included, incisional hernia was diagnosed in 71 (27.2%). Of the 71 incisional hernia patients, 28 (39.4%) developed IH during the first post-transplant. Majority of the patients were male (52/71, 73.2%); of the 71 patients, 52 had hepatitis C virus (HCV) and 33 (46.5%) had hepatocellular carcinoma (HCC). Male gender (p=0.044), diabetes mellitus (p=0.008), and acute cellular rejection (p<0.001) were risk factors for IH. In all, 28 (39.4%) patients were submitted for hernia repair with mesh, with a recurrence rate of 17.8%. CONCLUSION: Incisional hernia after liver transplantation is a relatively common problem associated with male gender, diabetes, and acute cellular rejection. This is a problem that should not be trivialized in view of the complexity of liver transplantation, as it can lead to a reduction in quality of life as well as jeopardize late liver transplantation results and lead to incarceration and strangulation.

RESUMO RACIONAL: O transplante de fígado é uma terapia complexa e valiosa. Entretanto, complicações que prejudicam a qualidade de vida pós-operatória, como a hérnia incisional, devem ser mais bem elucidadas, analisando os fatores de risco e medidas profiláticas. OBJETIVOS: Definir a taxa de hérnia incisional em pacientes submetidos a transplante de fígado em uma população do sul do Brasil, avaliar os fatores de risco relacionados, a fim de estabelecer futuramente medidas de otimização prévia e cuidados profiláticos específicos. MÉTODOS: Foram analisados, retrospectivamente, pacientes submetidos a transplante de fígado adultos, de janeiro de 2004 a novembro de 2020, avaliando suas características demográficas, resultados cirúrgicos e fatores predisponentes. RESULTADOS: Dentre os 261 pacientes transplantados hepáticos incluídos, a hérnia incisional foi diagnosticada em 71 (27,2%). Vinte e oito do total de 71 pacientes com hérnia incisional (39,4%) desenvolveram hérnia incisional durante o primeiro ano pós-transplante. A maioria era do sexo masculino [n=52, (73,2%)]; 52/71 (73,2%) apresentavam cirrose secundária ao vírus da hepatite C; 33/72 (46,5%) foram portadores de carcinoma hepatocelular. Sexo masculino (p=0,044), diabetes mellitus (p=0,008) e rejeição celular aguda (p<0,001) foram fatores de risco estatisticamente significantes para hérnia incisional. Vinte e oito pacientes (39,4%) foram submetidos à hernioplastia incisional com tela, com taxa de recidiva de 17,8%. CONCLUSÕES: Hérnia incisional após transplante de fígado é um problema relativamente comum, associado ao sexo masculino, diabetes e também a rejeição celular aguda. Este é um problema que não deve ser banalizado, já que pode levar à redução da qualidade de vida, comprometer os resultados tardios do transplante de fígado e pode levar a encarceramento ou estrangulamento.

Giant hepatic extra-gastrointestinal stromal tumor treated with cytoreductive surgery and adjuvant systemic therapy: A case report and review of literature.

BACKGROUND: Primary extra-gastrointestinal stromal tumors (E-GIST) of the liver are rare. The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect. Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care. However, under specific circumstances, a cytoreductive approach may represent a therapeutic option. We describe herein the case of an 84-year-old woman who presented with a tender, protruding epigastric mass. Abdominal computed tomography scan revealed a large, heterogeneous mass located across segments III, IV, V, and VIII of the liver. The initial approach was transarterial embolization of the tumor, which elicited no appreciable response. Considering the large size and central location of the tumor and the advanced age of the patient, non-anatomic complete resection was indicated. Due to substantial intraoperative bleeding and hemodynamic instability, only a near-complete resection could be achieved. Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver. Considering the risk/benefit ratio for therapeutic options, debulking surgery may represent a strategy to control pain and prolong survival. CASE SUMMARY: Here, we present a case report of a patient diagnosed with E-GIST primary of the liver, which was indicated a cytoreductive surgery and adjuvant therapy with imatinib. CONCLUSION: E-GIST primary of the liver is a rare conditional, the treatment is with systemic therapy and total resection surgery. However, a cytoreductive surgery will be necessary when a complete resection is no possible.

Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome.

BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.

Frequency of and factors associated with vascular complications after pediatric liver transplantation / Frequência e fatores relacionados a complicações vasculares após transplante hepático pediátrico

OBJECTIVE: to evaluate the frequency and factors associated with vascular complications after pediatric liver transplantation. METHOD: risk factors were evaluated in 99 patients under 18 years of age with chronic liver disease who underwent deceased donor liver transplantation (DDLT) between March of 1995 and November of 2009 at the Hospital de Clínicas de Porto Alegre, Brazil. The variables analyzed included donor and recipient age, gender, and weight; indication for transplant; PELD/MELD scores; technical aspects; postoperative vascular complications; and survival. RESULTS: vascular complications occurred in 19 patients (19%). Arterial events were most common, occurred earlier in the postoperative period, and were associated with high graft loss and mortality rates. In the multivariate analysis, the following factors were identified: portal vein diameter < 3 mm, donor-to-recipient body weight ratio (DRWR), prolonged ischemic time, and use of arterial grafts. CONCLUSION: the choice of treatment depends on the timing of diagnosis; however, in this study, surgical revision or correction produced worse outcomes than percutaneous angioplasty. The reduction of risk factors and early detection of vascular complications are key elements to a successful transplantation. .

OBJETIVO: avaliar a frequência e os fatores associados a complicações vasculares após transplante hepático pediátrico. MÉTODO: os fatores de risco foram avaliados em 99 pacientes com mais de 18 anos de idade comdoença hepática crônica submetidos a transplante hepático cadavérico (THC) entre marc¸o de1995 e novembro de 2009 no Hospital de Clínicas de Porto Alegre, Brasil. As variáveis analisadasincluíram: idade, sexo e peso dos doadores e receptores; indicac¸ão de transplante; escores PELD/MELD; aspectos técnicos; complicações vasculares pós-operatórias; e sobrevida. RESULTADOS: ocorreram complicações vasculares em 19 pacientes (19%). Os eventos arteriais foram mais comuns, tendo ocorrido precocemente no pós-operatório, e foram associados a altas taxas de perda do enxerto e mortalidade. Em uma análise multivariada, foram identificadosos seguintes fatores: diâmetro da veia porta < 3 mm, proporc¸ão de peso do doador/receptor (DRWR), tempo de isquemia prolongado e uso de enxertos arteriais. CONCLUSÃO: A escolha do tratamento depende do momento do diagnóstico; contudo, nessa série, a cirurgia de revisão, ou correc¸ão cirúrgica, produziu resultados piores que a angioplastia per-cutânea. A redução dos fatores de risco e a detecção precoce de complicações vasculares sãofundamentais para um transplante bem-sucedido. .

Frequency of and factors associated with vascular complications after pediatric liver transplantation.

OBJECTIVE: to evaluate the frequency and factors associated with vascular complications after pediatric liver transplantation. METHOD: risk factors were evaluated in 99 patients under 18 years of age with chronic liver disease who underwent deceased donor liver transplantation (DDLT) between March of 1995 and November of 2009 at the Hospital de Clínicas de Porto Alegre, Brazil. The variables analyzed included donor and recipient age, gender, and weight; indication for transplant; PELD/MELD scores; technical aspects; postoperative vascular complications; and survival. RESULTS: vascular complications occurred in 19 patients (19%). Arterial events were most common, occurred earlier in the postoperative period, and were associated with high graft loss and mortality rates. In the multivariate analysis, the following factors were identified: portal vein diameter ≤ 3mm, donor-to-recipient body weight ratio (DRWR), prolonged ischemic time, and use of arterial grafts. CONCLUSION: the choice of treatment depends on the timing of diagnosis; however, in this study, surgical revision or correction produced worse outcomes than percutaneous angioplasty. The reduction of risk factors and early detection of vascular complications are key elements to a successful transplantation.

Modificacões no perfil do paciente submetido à operacão de revascularizacão do miocárdio / Changes in profile of patients submitted to coronary bypass graft surgery

INTRODUÇAO: Indicacões da operacão de revascularizacão miocárdica (RM) foram modificadas pela introducão de novas drogas e da angioplastia coronária transluminal percutânea (ACTP), sendo o procedimento cada vez considerado em pacientes com doenca multiarterial coronária e de condicão clínica mais grave. OBJETIVO: Comparar perfil clínico e cirúrgico entre dois grupos de pacientes submetidos a RM com intervalo de 10 anos, bem como observar sua influência na mortalidade hospitalar. MÉTODO: Estudo de coorte retrospectivo, envolvendo 307 pacientes submetidos a RM em 1991/92 (grupo INICIAL, n=153) ou 2001/02 (grupo ATUAL, n=154). Para cada grupo foram identificadas características demográficas, doencas cardíacas, co-morbidades e eventos operatórios, visando comparacão e determinacão dos fatores relacionados à mortalidade hospitalar aumentada. RESULTADOS: Grupo recente tinha idade mais avancada, condicão cardíaca mais grave(classe funcional, prevalência de insuficiência cardíaca e número de vasos com lesão grave) e maior prevalência de co-morbidades. Pacientes iniciais mostraram maior prevalência na indicacão cirúrgica de urgência. Não ocorreu diferenca na mortalidade hospitalar (respectivamente 3,3 por cento e 1,9 por cento para grupos INICIAL e ATUAL). CONCLUSÕES: Pacientes atualmente submetidos a RM são mais idosos e de condicão clínica mais grave (cardíaca e sistêmica) que os operados há 10 anos, embora isto não tenha influenciado de modo significativo a mortalidade hospitalar, que é menor recentemente.