Long-term outcome of combined radiologic and surgical strategy for the management of biliary complications after pediatric liver transplantation.

OBJECTIVES: We aimed to analyze the risk factors for management failure of BC after pediatric liver transplantation (pLT) by retrospectively analyzing primary pLT performed between 1997 and 2018 (n = 620 patients). RESULTS: In all, 117/620 patients (19%) developed BC. The median (range) follow-up was 9 (1.4-21) years. Patient survival at 1, 5 and 10 years was 88.9%, 85.7%, 84.4% and liver graft survival was 82.4%, 77.4%, and 74.3% respectively. Graft not patient survival was impaired by BC (p = 0.01). Multivariate analysis identified the number of dilatation courses > 2 (p = 0.008), prolonged cold ischemia time (p = 0.004), anastomosed multiple biliary ducts (p = 0.019) and hepatic artery thrombosis (p = 0.01) as factors associated with impaired graft survival. The number of dilatation courses > 2 (p < 0.001) and intrahepatic vs anastomotic stricture (p = 0.014) were associated with management failure. Thus, repeated (> 2) radiologic dilatation courses are associated with impaired graft survival and management failure. Overall, graft but not patient survival was impaired by BC.

Biliary drainage surgery before or after 3 months of life versus primary liver transplantation in children with biliary atresia: comparative cohort study.

BACKGROUND: Early biliary drainage surgery (BDS; Kasai) is associated with longer transplant-free survival in biliary atresia. However, evidence is lacking on whether an age limit can be established at which liver transplantation should be performed as first-line treatment for children with a delayed diagnosis of biliary atresia. The aim of the current study was to compare the outcome of a large cohort of children with biliary atresia who underwent BDS after 90 days of life with those who underwent early BDS (before 90 days) and those who did not receive BDS and were directly referred for primary liver transplantation. METHODS: All patients with biliary atresia treated at Bicêtre, Paris-Saclay University Hospital between 1995 and 2017 were analysed in this STROBE-compliant study. Three groups were defined: BDS before 90 days of life (early BDS); BDS after 90 days of life (late BDS); and patients without BDS who were referred for primary liver transplantation (no BDS). Patient characteristics, overall survival, and native liver survival were compared. RESULTS: Of 424 children with biliary atresia, 69 patients (16 per cent) were older than 90 days when they underwent BDS. Twenty-five patients had no BDS and were referred for primary liver transplantation (6 per cent). The main reason for not performing BDS was manifest portal hypertension (18/25). Two- and 5-year transplant-free survival were significantly higher in patients with late BDS compared with no BDS (53.5 versus 12.0 per cent respectively for 2-year data and 30.4 versus 4.0 per cent respectively for 5-year data, P < 0.001). Five- and 10-year overall survival did not differ between early BDS (92 and 91 per cent respectively), late BDS (88 and 83 per cent respectively) and no BDS (80 and 80 per cent respectively, P = 0.061). CONCLUSION: Age alone should not routinely be considered a contraindication to BDS in patients older than 90 days. Liver transplantation in infancy (less than 12 months) could be avoided in 88 per cent of cases with late diagnosis of biliary atresia and is delayed significantly even when BDS is performed after 3 months. Overall survival is at least equal to patients who are referred for primary liver transplantation.

Epidemiology and complications of anaesthesia in the French centres that participated to NECTARINE: A secondary analysis.

INTRODUCTION: Neonatal and infant anaesthesia are associated with a high risk of perioperative complications. The aim of the current study was to describe those risks in France using the French data from the NECTARINE study. MATERIAL AND METHODS: Data from the French centres that participated to the NECTARINE study were analysed. The primary goal of the study was the description of patients' characteristics, procedures and perioperative management and their comparison with the results of the European NECTARINE study. Secondary outcomes were the description of major perioperative complications and death. RESULTS: Overall, 926 procedures collected in 15 centres (all teaching hospitals) were analysed. Comparison between the French and European NECTARINE cohorts found few differences related to patients' characteristics and procedures. The rate of interventions for critical events (respiratory, haemodynamic, and metabolic) was similar between the two cohorts. Near-infrared spectroscopy monitoring was used in 12% of procedures. Nearly none of the thresholds for these interventions met the published standards. By day 30, complications (respiratory, haemodynamic, metabolic, renal, and liver failure) and death were observed in 14.4% [95% CI 11.6-16.4]% and 1.8% [95% CI 1.1-2.9] of cases, respectively. DISCUSSION: Although the health status of the patients in the French cohort was less severe, procedures, management and postoperative complications and mortality rates were similar to the European cohort. However, thresholds for interventions were often inadequate in both cohorts. Efforts should be undertaken to improve the knowledge and use of new monitoring devices in this population.

Comparison of monitoring performance of bioreactance versus esophageal Doppler in pediatric patients.

BACKGROUND AND AIMS: Cardiac output (CO) monitoring and goal-directed therapy during major abdominal surgery is currently used to decrease postoperative complications. However, few monitors are currently available for pediatric patients. Nicom(®) is a noninvasive CO monitoring technique based on the bioreactance principle (analysis of frequency variations of a delivered oscillating current traversing the thoracic cavity). Nicom(®) may be a useful monitor for pediatric patients. SUBJECTS AND METHODS: Pediatric patients undergoing major abdominal surgery under general anesthesia with cardiac monitoring by transesophageal Doppler (TED) were included. Continuously recorded hemodynamic variables obtained from both bioreactance and TED were compared. Data were analyzed using the Bland-Altman method. RESULTS: A total of 113 pairs of cardiac index (CI) measurments from 16 patients were analyzed. Mean age was 59 months (95% CI: 42-75) and mean weight was 17 kg (95% CI: 15-20). In the overall population, Bland-Altman analysis revealed a bias of 0.4 L/min/m(2), precision of 1.55 L/min/m(2), limits of agreement of -1.1 to 1.9 L/min/m(2) and a percentage error of 47%. For children weighing >15 kg, results were: Bias 0.51 L/min/m(2), precision 1.17 L/min/m(2), limits of agreement -0.64 to 1.66 L/min/m(2) and percentage error 34%. CONCLUSION: Simultaneous CI estimations made by bioreactance and TED showed high percentage of errors that is not clinically acceptable. Bioreactance cannot be considered suitable for monitoring pediatric patients.

Development and validation of a risk score to predict the probability of postoperative vomiting in pediatric patients: the VPOP score.

BACKGROUND: Few data are available in the literature on risk factors for postoperative vomiting (POV) in children. OBJECTIVE: The aim of the study was to establish independent risk factors for POV and to construct a pediatric specific risk score to predict POV in children. METHODS: Characteristics of 2392 children operated under general anesthesia were recorded. The dataset was randomly split into an evaluation set (n = 1761), analyzed with a multivariate analysis including logistic regression and backward stepwise procedure, and a validation set (n = 450), used to confirm the accuracy of prediction using the area under the receiver operating characteristic curve (ROCAUC ), to optimize sensitivity and specificity. RESULTS: The overall incidence of POV was 24.1%. Five independent risk factors were identified: stratified age (>3 and <6 or >13 years: adjusted OR 2.46 [95% CI 1.75-3.45]; ≥6 and ≤13 years: aOR 3.09 [95% CI 2.23-4.29]), duration of anesthesia (aOR 1.44 [95% IC 1.06-1.96]), surgery at risk (aOR 2.13 [95% IC 1.49-3.06]), predisposition to POV (aOR 1.81 [95% CI 1.43-2.31]), and multiple opioids doses (aOR 2.76 [95% CI 2.06-3.70], P < 0.001). A simplified score was created, ranging from 0 to 6 points. Respective incidences of POV were 5%, 6%, 13%, 21%, 36%, 48%, and 52% when the risk score ranged from 0 to 6. The model yielded a ROCAUC of 0.73 [95% CI 0.67-0.78] when applied to the validation dataset. CONCLUSIONS: Independent risk factors for POV were identified and used to create a new score to predict which children are at high risk of POV.

Experience with endoscopic management of high-risk gastroesophageal varices, with and without bleeding, in children with biliary atresia.

BACKGROUND & AIMS: Biliary atresia, the most common cause of childhood cirrhosis, increases the risks for portal hypertension and gastrointestinal bleeding. We report the results from a single-center study of primary and secondary prophylaxis of bleeding in children with portal hypertension and high-risk varices. METHODS: We collected data from 66 children with major endoscopic signs of portal hypertension, including grade 3 esophageal varices or grade 2 varices with red wale markings and/or gastric varices, treated consecutively from February 2001 through May 2011. Thirty-six children (mean age, 22 mo) underwent primary prophylaxis (sclerotherapy and/or banding, depending on age and weight). Thirty children (mean age, 24 mo) who presented with gastrointestinal bleeding received endoscopic treatment to prevent a relapse of bleeding (secondary prophylaxis). RESULTS: In the primary prophylaxis group, a mean number of 4.2 sessions were needed to eradicate varices; no bleeding from gastroesophageal varices was observed after eradication. Varices reappeared in 37% of children, and 97% survived for 3 years. In the secondary prophylaxis group, a mean number of 4.6 sessions was needed to eradicate varices. Varices reappeared in 45%, and 10% had breakthrough bleeding; 84% survived for 3 years. There were no or only minor complications of either form of prophylaxis. CONCLUSIONS: Endoscopic therapy as primary or secondary prophylaxis of bleeding appears to be well tolerated and greatly reduces the risk of variceal bleeding in children with biliary atresia and high-risk gastroesophageal varices. However, there is a risk that varices will recur, therefore continued endoscopic surveillance is needed.

General anesthesia for intussusception reduction by enema.

OBJECTIVES: Intussusception is the most frequent cause of bowel obstruction in children. Although enema is usually used as the initial treatment, surgery may be required in more than 50% of patients. General anesthesia (GA) has been suggested to increase the rate of enema success. The purpose of this study was to evaluate whether GA increases the success rate of reduction by air enema. METHODS: In this retrospective single-center study from 1989 to the end of June 2008, patients receiving air enema for intussusception reduction were studied. Multivariable analysis using propensity score was performed to compare the success rate between patients receiving sedation or GA. RESULTS: The success rate of air enema increased from 72% in 1989 to the current rate of 90%. When time elapsed between first symptoms and enema was >12 h, the success rate decreased significantly (Odds Ratio 0.67 [0.56-0.81], P < 0.0001). When patients were matched by propensity score, GA significantly increased the likelihood of success (OR 5.66 [2.85-12.89], P = 0.013). CONCLUSIONS: Air enema performed under GA allows intussusception reduction in more than 90% of patients.

Outcome of central hepatectomy for hepatoblastomas.

BACKGROUND/PURPOSE: Central hepatoblastomas (CHBL) involving liver segments (IV + V) or (IV + V + VIII) are in contact with the portal bifurcation. Their resection may be achieved by central hepatectomy (CH) with thin resection margins on both sides of the liver pedicle, by extended right or left hepatectomy with thin resection margins on one side, or by liver transplantation with thick free margins. The aim of this study is to assess the operative and postoperative outcome of CH for hepatoblastoma. METHODS: This was a retrospective monocentric study of 9 patients who underwent CH for CHBL between 1996 and 2008. RESULTS: The operative time was 4 hours 50 minutes (2 hours 20 minutes to 7 hours), vascular clamping lasted 30 minutes (0-90 minutes), and the amount of blood cell transfusion was 250 mL (0-1800 mL). Two patients had biliary leakage requiring percutaneous drainage. Median follow-up time was 27 months (14-120 months). All of 8 nonmetastatic patients are alive and disease-free; 1 metastatic patient died of recurrent metastases at last follow-up. Although 3 of 9 patients had surgical margins less than 1 mm, none, including the patients who died from metastases, had local recurrence. CONCLUSIONS: Our study demonstrates the feasibility of CH for CHBL without operative mortality or local recurrence. Central hepatectomy is an alternative to extensive liver resections in selected patients.

Human herpesvirus-6 infection: a prospective study evaluating HHV-6 DNA levels in liver from children with acute liver failure.

The aim of this prospective study was to investigate the role of HHV-6 infection in children with acute onset of liver failure using real-time quantitative PCR. Twenty-three children (median age, 24 months) were included: 6 cases of fulminant hepatic failure of undetermined cause (group 1); 4 cases of fulminant hepatic failure of recognized cause (group 2); 3 cases of acute decompensation of chronic liver disease (group 3); and 10 cases of chronic liver disease (group 4). HHV-6 genomic DNA was detected and quantified using real-time PCR in plasma and livers obtained at the time of transplantation. HHV6-DNA detection rate was significantly higher among groups 1, 2, and 3 compared to group 4 (76.9% vs. 20% P = 0.02). Viral loads ranged from 6 to 32,500 copies/106 cells. Significantly higher viral loads were found in 4 of 9 children with acute onset of liver failure of unknown origin (group 1, n = 3; group 3, n = 1) and 1 child with fulminant autoimmune hepatitis (group 2) (P = 0.03). These results strongly support the hypothesis that HHV-6 may cause fulminant hepatic failure and acute decompensation of chronic liver disease in children. Nevertheless, a threshold viral load value still remains to be determined.

The transesophageal Doppler and hemodynamic effects of epidural anesthesia in infants anesthetized with sevoflurane and sufentanil.

BACKGROUND: It is thought that pediatric epidural anesthesia (EA) provides hemodynamic stability in children. However, when compared with information relating to adults, little is known about the hemodynamic effects of epidural EA on cardiac output (CO) in infants. METHODS: Using transesophageal Doppler to monitor CO, we prospectively studied 14 infants <10 kg who were scheduled for abdominal surgery. During sevoflurane general anesthesia, CO transesophageal Doppler monitoring was performed before and after lumbar EA with 0.75 mL/kg of 0.25% bupivacaine and 1:200,000 adrenaline. CO, arterial blood pressure, and heart rate were measured before and 5, 15, and 20 min after performance of EA. RESULTS: In patients anesthetized with sevoflurane and sufentanil, EA resulted in an increase in stroke volume by 29% (P < 0.0001) and a decrease in heart rate by 13% (P < 0.0001). EA also induced a significant decrease in systolic, diastolic, mean arterial blood pressure, and systemic vascular resistance by 11%, 18%, 15%, and 25%, respectively. Conversely, CO remained unchanged. CONCLUSIONS: The increase in stroke volume observed is probably explained by optimization of afterload because of the sympathetic blockade induced by EA. These results confirm that EA provides hemodynamic stability in infants weighing <10 kg and supports the use of EA in this pediatric population.

Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center.

The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome. We reviewed 332 OLTs performed in 280 children between the years 1986 and 2000. Univariate and multivariate analysis were performed on patient and graft survivals according to recipients' and donors' characteristics as well as intraoperative data. The long-term outcome among the 80 children living at 10 years after OLT was studied according to growth, immunosuppressive therapy, and liver and renal functions. Liver graft status was eventually documented by liver biopsy. Status of rehabilitation was assessed by reviewing school performance and employment. Overall patient survival rates at 1, 5, and 10 years were 85, 82, and 82%, respectively, and the corresponding overall graft survival rates were 77, 73, and 71%. In the multivariate analysis, we identified 4 independent prognostic factors: polysplenia syndrome (P = .03), United Network for Organ Sharing (UNOS) status (P = .05), donor's age (P = .01), and perioperative surgical complications (P = .03). At 10 years after transplant, 80 children were alive and had normal growth rates. Liver histology was abnormal in 73% of these long-term survivors, mainly due to chronic rejection and centrilobular fibrosis. A total of 63 of the 80 children attended normal school and in 55 children (69%) school performance was not delayed. In conclusion, we discovered that a good long-term survival could be achieved after liver transplantation for BA, with a 82% survival rate at 10 years with normal scholastic studies in the majority of recipients.

Paediatric caudal and epidural analgesia.

PURPOSE OF REVIEW: Epidural blocks (caudal, lumbar or thoracic) are of common use in paediatric patients for special procedures such as open fundoplication as well as for postoperative analgesia. However, because neonates and infants have lower metabolic capacities and specific anatomy as compared to adults, the dose of local anaesthetics and the way of their administration need careful attention. RECENT FINDINGS: Recent pharmacokinetic studies have more precisely identified the differences in pharmacokinetics between age groups, and between local anaesthetic agents. In that respect, ropivacaine exhibits a unique pharmacokinetic profile. Among all adjuvant drugs proposed so far, S-ketamine is promising; however, complete safety in case of injection into the cerebrospinal fluid must be checked before routine use. Thoracic epidural anaesthesia is technically difficult, and carries a potential risk of spinal cord puncture. Numerous techniques have been proposed to avoid this risk. The advancement of a catheter into the epidural space after caudal insertion has long been proposed by German and Italian authors, but for anatomical reasons, this technique is only possible in young infants. Some improvement of the original technique using lumbar insertion at the L4-L5 interspace has recently been suggested; the possibility of catheter guidance by means of electric stimulation has also been proposed. SUMMARY: Pharmacokinetic studies allowing safer dosing, especially with ropivacaine have recently been published. New techniques of thoracic epidural with puncture at the lumbar level are also proposed. However, all these new techniques need to pass the test of time.