Ernica Clinical Consensus Statements on Total Colonic and Intestinal Aganglionosis.

BACKGROUND: Hirschsprung disease is a congenital intestinal motility disorder characterized by an absence of enteric ganglion cells. Total colonic aganglionosis and near total or total intestinal aganglionosis, defined as absence of ganglion cells in the entire colon and with variable length of small bowel involved, are life-threatening conditions which affect less than 10 % of all patients with Hirschsprung disease. The aim of this project was to develop clinical consensus statements within ERNICA, the European Reference Network for rare congenital digestive diseases, on four major topics: Surgical treatment of total colonic aganglionosis, surgical treatment of total intestinal aganglionosis, management of poor bowel function in total colonic and/or intestinal aganglionosis and long-term management in total colonic and or intestinal aganglionosis. METHODS: A multidisciplinary panel of representatives from ERNICA centers was invited to participate. Literature was searched, using specified search terms, in Medline (ALL), Embase and Google Scholar. Abstracts were screened and full text publications were selected. The panel was divided in four groups that extracted data from the full text publications and suggested draft statements for each of the major topics. A modified Delphi process was used to refine and agree on the statements. RESULTS: The consensus statement was conducted by a multidisciplinary panel of 24 participants from 10 European countries, 45 statements reached consensus after 3 Delphi-rounds. The availability of high-quality clinical evidence was limited, and most statements were based on expert opinion. Another 25 statements did not reach consensus. CONCLUSIONS: Total colonic and total intestinal aganglionosis are rare variants of Hirschsprung disease, with very limited availability of high-quality clinical evidence. This consensus statement provides statements on the surgical treatment, management of poor bowel function and long-term management for these rare patients. The expert panel agreed that patients benefit from multidisciplinary and personalized care, preferably in an expert center. TYPE OF STUDY: Clinical consensus statement. LEVEL OF EVIDENCE: 3a.

Native liver survival in bile salt export pump deficiency: results of a retrospective cohort study.

BACKGROUND: Bile salt export pump (ABCB11) deficiency [Progressive familial intrahepatic cholestasis (PFIC2)] is the most common genetic cause of PFIC and is associated with pruritus and progressive liver disease. Surgical biliary diversion or pharmacological [ileal bile acid transporter inhibitor (IBATi)] approaches can be used to block the recirculation of bile acids to the liver. There is a paucity of detailed data on the natural history and, in particular, the longitudinal evolution of bile acid levels to predict treatment response. Cross-sectional data from large international consortia suggested a maximum cutoff value of bile acids after the intervention to predict a successful outcome. METHODS: This retrospective, single-center, cohort study included all patients with confirmed biallelic pathogenic ABCB11 genotype PFIC2 treated at our institution with ≥2 years follow-up. The outcomes of interventions and predictors of long-term health were analyzed. RESULTS: Forty-eight cases were identified with PFIC2. Eighteen received partial external biliary diversion (PEBD) surgery, and 22 patients underwent liver transplantation. Two patients developed HCC and 2 died. Improved survival with native liver was closely associated with genotype, complete normalization of serum bile acids following PEBD, and alleviation of pruritus. Persistence of mild-to-moderate elevation of bile acids or a secondary rise following normalization was associated with liver disease progression and led to transplantation, suggesting that any prolonged elevation of bile acids worsens the chance of native liver survival. Higher-grade fibrosis at the time of PEBD was not associated with reduced long-term native liver survival. Patients with PFIC2 benefit from PEBD even at a stage of advanced fibrosis. CONCLUSION: Serum bile acid levels are an early predictor of treatment response and might serve as the gold standard in the evaluation of novel therapies including IBATi.

Laparoscopic versus Open Inguinal Hernia Repair Is Feasible in Infants with Caudal Anesthesia and Spontaneous Respiration.

INTRODUCTION: Minimally invasive surgery (i.e., laparoscopy) and minimally invasive anesthesia (i.e., caudal anesthesia with spontaneous respiration) have separately shown benefits for inguinal hernia repair in infants, yet to what degree these techniques can be combined remains unknown. This study investigated whether laparoscopy impacts the feasibility of performing caudal anesthesia with spontaneous respiration in infants. METHODS: Prospectively collected data of all infants less than 12 months old and over 3 kg weight who underwent laparoscopic indirect hernia repair (LAP) at our department from 2019 to 2021 were compared with a historical control-matched group of infants who underwent open repair (OPEN) from 2017 to 2021. We assessed the patients' characteristics, anesthesia, and surgical data as well as intra- and postoperative complications. RESULTS: A total of 87 infants were included (LAP n = 29, OPEN n = 58). Caudal anesthesia with spontaneous respiration was feasible in 62.1% of cases (LAP n = 55.2%, OPEN n = 65.5%; nonsignificant). Neither group registered anesthetic intra- or postoperative complications. Sedatives were utilized in 97% of LAP patients versus 56.9% of OPEN patients (p < 0.00001). The airway was secured with a laryngeal mask in 89.7% of patients during LAP versus 41.4% during OPEN (p < 0.00001). No significant differences were found regarding the use frequency of opioids (48.3% LAP vs. 34.5% OPEN; nonsignificant) or neuromuscular blockers (6.9% LAP vs. 5.2% OPEN; nonsignificant). CONCLUSION: This is the first comparative study on caudal anesthesia and spontaneous respiration in infants undergoing laparoscopic versus open inguinal hernia surgery. Laparoscopy increased the need for ventilatory support and sedatives but did not significantly impair the feasibility of caudal anesthesia and spontaneous respiration.

Pleural CD14+ monocytes/macrophages of healthy adolescents show a high expression of metallothionein family genes.

Nowadays laparoscopic interventions enable the collection of resident macrophage populations out of the human cavities. We employed this technique to isolate pleural monocytes/macrophages from healthy young adults who underwent a correction of pectus excavatum. High quality CD14+ monocytes/macrophages (plMo/Mφ) were used for RNA-sequencing (RNA-seq) in comparison with human monocyte-derived macrophages (MDM) natural (MDM-0) or IL-4-polarized (MDM-IL4). Transcriptome analysis revealed 7166 and 7076 differentially expressed genes (DEGs) in plMo/Mφ relative to natural MDM-0 and polarized MDM-IL4, respectively. The gene set enrichment analysis, which was used to compare RNA-seq data from plMo/Mφ with single-cell (scRNA-seq) data online from human bronchial lavage macrophages, showed that plMo/Mφs are characterized by a high expression of genes belonging to the metallothionein (MT) family, and that the expression of these genes is significantly higher in plMo/Mφ than in MDM-0 or MDM-IL4. Our results provide additional insights on high MTs-expressing macrophage subsets, which seem to be present not only in bronchial lavage of healthy adults or in pleural exudates of lung cancer patients but also in pleural fluid of healthy young adults. Macrophage subsets expressing high MTs may have specific roles in lung defense, repair, and homeostasis, and require further investigations.

The Role of Laparoscopic Treatment of Choledochal Malformation in Europe: A Single-Center Experience and Review of the Literature.

BACKGROUND: Numerous studies from Asian countries, including large collectives, have reported excellent results after laparoscopic resection of choledochal malformation (CM). However, the role of laparoscopic CM resection is still controversial outside Asia. We aimed to analyze the outcome of laparoscopic CM resection in our institution and to compare our outcome with the data reported in the literature. METHODS: All patients who underwent laparoscopic CM resection in our pediatric surgical department from 2002 to 2019 were retrospectively analyzed for surgical details and postoperative complications, which were graded according to the Clavien-Dindo classification. A systematic literature search identified all reports on over 10 cases of laparoscopic pediatric CM resection and surgical details, follow-up, and complication rates were extracted. RESULTS: Fifty-seven patients (72% female) with a mean age of 3.6 + 4.1 years underwent laparoscopic CM resection in our department. Conversion rate was 30%. Total complication rate was 28%. The rate of major complications (Clavien-Dindo grade III or more) was 16% and included stricture of the biliodigestive or enteric anastomosis (n = 4), adhesive ileus (n = 3), portal vein thrombosis (n = 1), and recurrent cholangitis with consecutive liver transplantation (n = 1). With increasing experience, complication rates decreased. The majority of publications on laparoscopic CM resections originated from Asia (n = 36) and reported on low complication rates. In contrast, publications originating from non-Asian countries (n = 5) reported on higher complications following laparoscopic CM resection. CONCLUSION: Our data indicate that laparoscopic CM resection can be safely performed. The learning curve in combination with the low incidence calls for a centralization of patients who undergo laparoscopic CM resection. There seems to be a discrepancy on complications rates reported from Asian and non-Asian countries following laparoscopic CM resection.

Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists.

BACKGROUND: While congenital choledochal malformation (CCM) is relatively well known within the pediatric surgical and pediatric gastroenterological communities, many controversies and questions remain. METHODS: In this paper, we will discuss the results of an international Delphi survey among members of the European Reference Network RARE-LIVER and of the faculty of the Biliary Atresia and Related Diseases (BARD) network to identify the most common practices as well as controversies regarding diagnosis, treatment and follow-up of this still enigmatic disease. RESULTS: Twenty-two individual respondents completed the survey. While there seems to be agreement on the definitions of CCM, preoperative workup, surgical approach and follow-up still vary considerably. The mainstay of treatment remains the removal of the entire extrahepatic biliary tract, clearance of debris both proximally and distally, followed by reconstruction with (according to 86% of respondents) a Roux-en-Y hepaticojejunostomy. Nonetheless, both laparoscopic and robotic-assisted resections are gaining ground with the suggestion that this might be facilitated by concentration of care and resources in specialized centers. However, long-term outcomes are still lacking. CONCLUSIONS: As even post-surgical CCM has to be considered as having premalignant potential, follow-up should be well-organized and continued into adulthood. This seems to be lacking in many centers. International cooperation for both benchmarking and research is paramount to improving care for this rare disease.

Analysis of Pulmonary Surgery in Children and Adolescents in Germany: Who Is doing What?

INTRODUCTION: In Germany, pediatric surgery is organized in a decentralized manner. A nationwide clinical registry does not exist. The aim of this research is to analyze the current status of pulmonary surgery in the country in respect of children and adolescents. MATERIALS AND METHODS: Claims data have been extracted from two health insurance databases representing 34% of the German population. Operative diagnoses and procedures, as well as patients' characteristics, were recorded from 2016 to 2019. RESULTS: The incidence of the four index diagnoses investigated matched the current literature in three of four entities. The main indications <8 years were congenital lung malformations (61%) and spontaneous pneumothorax at age 8 to 17 years (77%). Furthermore, 1,668 pediatric pulmonary procedures (0-17 years) were identified (668 pulmonary resections). Two age peaks were identified: 13% of patients were operated on before 1 year old and 50% of patients were between 15 and 17 years old. Video-assisted-thoracoscopic-surgery (VATS) for pulmonary resections was applied in 62% of cases. 96% of pulmonary resections in patients <1 year and 70% of pulmonary resections in children between 1 and 14 years were performed by pediatric surgeons. Of patients between 15 and 17 years old, only 14% were operated on in pediatric surgery. The estimated average pulmonary resection caseload was 3.2 cases per year and per institution for pediatric surgery and 1.5 for adult surgery. CONCLUSION: Indications for pulmonary surgery in children arise from rare diagnoses. In total, 66% of lung resections are performed in patients <1 year and >14 years. The majority of lung resections are performed as VATS. Patients ≤14 years are predominantly operated on by pediatric surgeons. The accessibility of pediatric pulmonary surgery is acceptable, but there is a low caseload per center.

Laparoscopic versus Open Pediatric Surgery: Three Decades of Comparative Studies.

INTRODUCTION: Despite its wide acceptance, the superiority of laparoscopic versus open pediatric surgery has remained controversial. There is still a call for well-founded evidence. We reviewed the literature on studies published in the last three decades and dealing with advantages and disadvantages of laparoscopy compared to open surgery. MATERIALS AND METHODS: Studies comparing laparoscopic versus open abdominal procedures in children were searched in PubMed/MEDLINE. Reports on upper and lower gastrointestinal as hepatobiliary surgery and on surgery of pancreas and spleen were included. Advantages and disadvantages of laparoscopic surgery were analyzed for different types of procedures. Complications were categorized using the Clavien-Dindo classification. RESULTS: A total of 239 studies dealing with 19 types of procedures and outcomes in 929,157 patients were analyzed. We identified 26 randomized controlled trials (10.8%) and 213 comparative studies (89.2%). The most frequently reported advantage of laparoscopy was shorter hospital stay in 60.4% of studies. Longer operative time was the most frequently reported disadvantage of laparoscopy in 52.7% of studies. Clavien-Dindo grade I to III complications (mild-moderate) were less frequently identified in laparoscopic compared to open procedures (80.3% of studies). Grade-IV complications (severe) were less frequently reported after laparoscopic versus open appendectomy for perforated appendicitis and more frequently after laparoscopic Kasai's portoenterostomy. We identified a decreased frequency of reporting on advantages after laparoscopy and increased reporting on disadvantages for all surgery types over the decades. CONCLUSION: Laparoscopic compared with open pediatric surgery seems to be beneficial in most types of procedures. The number of randomized controlled trials (RCTs) remains limited. However, the number of reports on disadvantages increased during the past decades.

Kasai Procedure in Patients Older Than 90 Days: Worth a Cut.

INTRODUCTION: Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE. MATERIALS AND METHODS: A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90-133 days). RESULTS: Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57-2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old. CONCLUSION: Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.

Phenotypic Switch of Human Peritoneal Macrophages during Childhood.

INTRODUCTION: Human peritoneal macrophages are resident in the abdominal cavity where they support the specific microenvironmental regulation. We have previously observed a phenotypic switch of murine macrophages during infancy that was associated with a functional development. To investigate the age related changes in human peritoneal macrophages, we analyzed peritoneal macrophages of children undergoing laparoscopic procedures. MATERIALS AND METHODS: Immunologically healthy children who received minimally invasive surgery in our department were included in this study. In all cases, the written consent was obtained. At the beginning of laparoscopy, physiologic NaCl-solution was instilled and manually removed through the umbilical trocar to gain macrophages. Lavage cells were processed for flow cytometry analysis. CD14+ myeloid cells were monitored for specific lineage marker expression. RESULTS: A total of 21 donors (age: 7 days-18 years) were included and divided into three groups. In all age groups, 97% of myeloid cells expressed CD11b. 70% of these expressed CD14. Three subsets of CD14 cells were detected on the basis of CD14/CD16 expression (CD14 + CD16dim, CD14 + CD16inter, and CD14 + CD16high). In neonates, >80% belonged to the CD14 + CD16high subset, reducing to 30% in adolescents. In none of the cases, the M2 markers CD23 and CD25 were expressed. CONCLUSION: This is the first study showing that lineage marker expression of peritoneal macrophages in neonates differs from that in adults. The knowledge about neonatal tissue resident macrophages might help to understand their complex interaction and to use specific macrophage properties for therapeutic approaches.

Video-Assisted Thoracoscopic or Conventional Thoracic Surgery in Infants and Children: Current Evidence.

INTRODUCTION: The pros and cons of video-assisted thoracoscopic versus conventional thoracic surgery in infants and children are still under debate. We assessed reported advantages and disadvantages of video-assisted thoracoscopy in pediatric surgical procedures, as well as the evidence level of the available data. MATERIALS AND METHODS: A systematic literature search was performed to identify manuscripts comparing video-assisted thoracoscopic and the respective conventional thoracic approach in classic operative indications of pediatric surgery. Outcome parameters were analyzed and graded for level of evidence (according to the Oxford Centre of Evidence-Based Medicine). RESULTS: A total of 48 comparative studies reporting on 12,709 patients, 11 meta-analyses, and one pilot randomized controlled trial including 20 patients were identified. More than 15 different types of advantages for video-assisted thoracoscopic surgery were described, mostly with a level of evidence 3b or 3a. Most frequently video-assisted thoracoscopic surgery was associated with shorter hospital stay, shorter postoperative ventilation, and shorter time to chest drain removal. Mortality rate and severe complications did not differ between thoracoscopic and conventional thoracic pediatric surgery, except for congenital diaphragmatic hernia repair with a lower mortality and higher recurrence rate after thoracoscopic repair. The most frequently reported disadvantage for video-assisted thoracoscopic surgery was longer operative time. CONCLUSION: The available data point toward improved recovery in pediatric video-assisted thoracoscopic surgery despite longer operative times. Further randomized controlled trials are needed to justify the widespread use of video assisted thoracoscopy in pediatric surgery.

The Intestinal Flora at Kasai Procedure in Children with Biliary Atresia Appears Not to Affect Postoperative Cholangitis.

INTRODUCTION: Evidence supports long-term oral antibiotic prophylaxis to prevent cholangitis after Kasai procedure. Data regarding perioperative intravenous prophylaxis are lacking. Ascending pathogens from the intestine are made responsible for recurrent cholangitis. Therefore, we analyzed the flora in the upper jejunum during the Kasai procedure and their potential impact on postoperative cholangitis. MATERIALS AND METHODS: In 26 patients, swabs were taken at the bowel prepared for the Roux-en-Y-loop. Our postoperative protocol includes intravenous third-generation cephalosporins for 2 weeks and rectal steroids starting at day 4. Cholangitis was defined as the postoperative reappearance of acholic stools or increase of serum bilirubin in combination with fevers or increase of inflammatory parameters. In this scenario, Tazocin was administered for another 2 weeks. RESULTS: Swabs remained sterile in nine patients (34.6%). In 17 patients (65.4%), gram-positive and gram-negative pathogens were identified; all belonging to physiological intestinal flora. A total of 96.2% pathogens were covered by the antibiotic prophylaxis. The cholangitis incidence was 55.6% in the sterile cohort, and 23.5% in the gram-positive and gram-negative cohort (p = 0.06). In the cholangitis cohort, no significant differences were detected for the age at Kasai and the pre- and postoperative total bilirubin. CONCLUSION: We found that our antibiotic regiment covered bacteria in the upper gastrointestinal (GI) tract in the majority of our patients at the time of Kasai. Nonetheless, a significant proportion of patients developed signs of cholangitis. There was no higher rate of cholangitis in patients with resistant bacteria. Thus, our data do not support the hypothesis of extended postoperative intravenous antibiotics to prevent ascending cholangitis.

Choledochal Cysts Resected during Childhood Show No Mutations of KRAS and BRAF as Early Markers of Malignancy in Cholangiocytes.

INTRODUCTION: In patients with choledochal cysts (CDC), a hyperplasia-dysplasia-carcinoma sequence can lead to biliary tract malignancy. The limited data available suggest that the risk decreases considerably after excision in childhood. We analyzed samples of resected CDC from pediatric patients histologically and performed mutational analysis of the proto-oncogenes KRAS and BRAF as early markers of malignant alteration in cholangiocytes. MATERIALS AND METHODS: After institutional review board approval, patients undergoing resection for CDC in our center from 2011 to 2019 were retrospectively identified. Histopathological reports were searched for inflammation and endothelial alteration. Cases with sufficient tissue specimen were tested for KRAS codon 12/13 and BRAF codon 600 mutations by pyrosequencing. RESULTS: In total, 42 patients underwent resection for choledochal cyst in the study period. Median age at surgery was 2.4 years (range = 18 days-18 years). Histopathological analysis showed no malignancy, but various degrees of inflammation or fibrosis in approximately 50% of the patients and in all age groups. Sufficient tissue for mutation analysis was available for 22 cases, all of which tested negative for KRAS or BRAF mutation. CONCLUSION: In our series, chronic inflammatory changes were frequently present in CDC of infants and children. However, the lack of KRAS and BRAF mutations suggests that no malignant changes have been initiated in this group of European patients undergoing early resection.

Low gestational age is associated with less anastomotic complications after open primary repair of esophageal atresia with tracheoesophageal fistula.

BACKGROUND: The aim of this study was to evaluate anastomotic complications after primary one-staged esophageal atresia (EA) repair relating to the patients` gestational age (GA). METHODS: Retrospective data analyses of patients who underwent closure of tracheoesophageal fistula (TEF) and primary esophageal anastomosis from 01/2007 to 12/2018 in two pediatric surgical centers. Exclusion of EA other than Gross type C, long-gap EA, minimal invasive or staged approach. Postoperative complications during the first year of life were assessed. Associated malformations, the incidence of infant respiratory distress syndrome (IRDS) and intraventricular bleeding were analyzed. RESULTS: Inclusion of 75 patients who underwent primary EA repair. Low GA was associated with significantly lower incidence of anastomotic complications (p = 0.019, r = 0.596, 95% CI 0.10-0.85). Incidence of anastomotic leakage (0% vs. 5.5%; p = 0.0416), recurrent TEF (0% vs. 5.5%; p = 0.0416) und anastomotic stricture (0% vs. 14.5%; p = 0.0019) was significantly lower in patients < 34 gestational weeks. Incidence of IRDS (55% vs. 0%; p < 0.0001) and intraventricular bleeding (25% vs. 3.6%; p = 0.0299) was significantly higher in patients < 34 gestational weeks. CONCLUSIONS: Despite prematurity-related morbidity, low GA did not adversely affect surgical outcome after primary EA repair. Low GA was even associated with a better anastomotic outcome indicating feasibility and safety of primary esophageal reconstruction.

Minimally Invasive Pediatric Surgery: The Learning Curve.

The use of minimally invasive surgery (MIS) in pediatric patients has increased over the past decades. The process of mastering a new procedure is termed the learning curve, during which the ability to operate increases but poorer outcomes are produced. We aim to analyze the current evidence on learning curves in pediatric MIS and evaluate its impact on patient's clinical outcomes. A systematic literature search was performed for studies listed on PubMed that reported on the learning curve for MIS surgical procedures. Studies were included if they stated the number of procedures required to reach a consistency in outcomes or if they compared outcomes between early and late period of MIS experience regarding the endpoints operative time, conversions, and intra-/postoperative complications. A total of 22 articles reporting on 11 surgical procedures were included in the study. Most authors reported a significant decrease in operative time as well as peri- and postoperative complications with increasing experience of the surgeon. Complications ranged from minor to major, the latter being especially severe for patients receiving pyloromyotomy (5-7% higher risk of mucosal perforation), esophageal atresia repair (15% higher leakage rate and 19-77% higher stenosis rate), or Kasai portoenterostomy (26-35% more liver transplants in the first year after surgery) during the learning curve period. Pediatric MIS comes with a considerable learning curve that may have a significant impact on the patient's clinical outcomes. Efforts should be made to minimize the effect of the learning curve on the patients.

Thirty Years of Minimally Invasive Surgery in Children: Analysis of Meta-Analyses.

INTRODUCTION: In the last three decades, minimally invasive surgery (MIS) has been widely used in pediatric surgery. Meta-analyses (MAs) showed that studies comparing minimally invasive with the corresponding open operations are available only for selected procedures. We evaluated all available MAs comparing MIS with the corresponding open procedure in pediatric surgery. MATERIALS AND METHODS: A literature search was performed on all MAs listed on PubMed. All analyses published in English, comparing pediatric minimally invasive with the corresponding open procedures, were included. End points were advantages and disadvantages of MIS. Results of 43 manuscripts were included. MAs evaluating the minimally invasive with the corresponding open procedures were available for 11 visceral, 4 urologic, and 3 thoracoscopic types of procedures. Studies included 34 randomized controlled trials. In 77% of MAs, at least one advantage of MIS was identified. The most common advantages of MIS were a shorter hospital stay in 20, a shorter time to feeding in 11, and a lower complication rate in 7 MAs. In 53% of MAs, at least one disadvantage of MIS was found. The most common disadvantages were longer operation duration in 16, a higher recurrence rate of diaphragmatic hernia in 4, and gastroesophageal reflux in 2 MAs. A lower native liver survival rate after laparoscopic Kasai-portoenterostomy was reported in one MA. CONCLUSION: In the available MAs, the advantages of MIS seem to outnumber the disadvantages. However, for some types of procedures, MIS may have considerable disadvantages. More randomized controlled trials are required to confirm the advantage of MIS for most procedures.

Impact of Capnoperitoneum on Renal Perfusion and Urine Production in Infant and Adolescent Pigs: Crystalloid versus Colloid Fluid Resuscitation.

BACKGROUND: Infants are likely to develop anuria during laparoscopy which is uncommon in older patients. The reason for this susceptibility remains unknown. We compared the impact of CO2 pneumoperitoneum on renal perfusion and urine production in piglets compared with adolescent pigs. We furthermore investigated the effects of different resuscitation strategies. MATERIALS AND METHODS: Male piglets (n = 21) were divided into four groups: (a) infant controls (n = 5), (b) infants with crystalloid restitution (n = 6), (c) infants with colloidal restitution (n = 5), and (d) adolescents with crystalloid restitution (n = 5). Animals were ventilated, the central vessels and ureters were cannulated, and the animals were subjected to a 3-hour, 10 mm Hg CO2 pneumoperitoneum followed by 2-hour resuscitation. Renal perfusion was assessed by fluorescent microspheres and the rate of urine flow was measured. RESULTS: Urine production significantly decreased after insufflation only in the infant crystalloid and adolescent group, but not in controls or infants treated with colloids. In the infant crystalloid group, urine production remained at levels below 20% of baseline throughout the experiment. In this group, the renal perfusion dropped significantly after the beginning of the capnoperitoneum and remained significantly reduced throughout the experiment. CONCLUSION: Our data indicates that capnoperitoneum impairs renal perfusion and urine production in infants. In moderate-pressure capnoperitoneum, this effect cannot be compensated by application of crystalloids but with colloids.

Quality of Life in Patients With Progressive Familial Intrahepatic Cholestasis: No Difference Between Post-liver Transplantation and Post-partial External Biliary Diversion.

OBJECTIVES: In patients with progressive familial intrahepatic cholestasis (PFIC), partial external biliary diversion (PEBD), which is associated with a permanent stoma, is recommended as first-line therapy, whereas primary liver transplantation (LTx) is restricted to those with cirrhosis. Our aim was to quantify the health-related quality of life (HRQOL) in patients with PFIC and to evaluate whether there is a difference in their HRQOL depending on the surgical approach. METHODS: A prospective HRQOL study on a consecutive series of PFIC was conducted using Pediatric Quality of Life Inventory 4.0 child-self and parent-proxy reports. Patients with PFIC after PEBD who still lived with their native livers were compared to those after LTx. Both groups were compared to healthy children. RESULTS: A total of 32 patients (53% girls) patients with a mean age of 17.7 ±â€Š7.3 years were studied. Twenty-two had undergone LTx at a mean age of 7.8 ±â€Š3.8 years and 10 had undergone PEBD at a mean age of 4.1 ±â€Š3.9 years. At the time of HRQOL assessment, the mean age was 18.9 ±â€Š7.5 years in the LTx group and 15.3 ±â€Š6.5 years in the PEBD group. Child-self and parent-proxy reports showed no significant difference in HRQOL between patients with PFIC after LTx and those after PEBD except for marginal difference in physical functioning/health (P = 0.07). Except for a lower score in patient school functioning of patients after LTx (P = 0.01), HRQOL-results showed no difference from healthy children in any group. CONCLUSIONS: The HRQOL of patients with PFIC after PEBD was similar to those after LTx. The HRQOL in both groups was also similar to that of healthy children. Thus, our data support the current policy of PEBD as primary surgical treatment for patients with PFIC without cirrhosis.

Diagnostic accuracy and therapeutic relevance of thoracoscopic lung biopsies in children.

INTRODUCTION: Histopathological assessment of lung biopsies does play an important diagnostic role in children's interstitial lung disease (ChILD). Thoracoscopic lung biopsy has been shown to be safe and effective. The aim of this study was to evaluate the diagnostic accuracy of thoracoscopic lung biopsies in children with ChILD. Furthermore, therapeutic relevance of the procedure, operative details, and perioperative complications of our series were investigated. METHODS: We retrospectively reviewed all consecutive thoracoscopic lung biopsies taken from children with suspected ChILD in our institution over an 11-year period. Feasibility and complications were evaluated as well as histopathological diagnoses according to the recent ChILD classification and relevance of the procedure for medical treatment. RESULTS: Fifty-nine patients (54.2% male, median age 7 years [8 d-18 y]) underwent 112 thoracoscopic lung biopsies. An endostapler (27%), endoloop ligature (63%), or cut and suture technique (10%) were used. A chest tube was placed in 54% of the cases. Complications occurred in 15% of cases and mainly consisted of pneumothoraces or bleedings. Adequate tissue was obtained in all but one case and the biopsy led to a specific diagnosis according to the ChILD classification in 98%. Medical treatment of the underlying disease was changed due to the results of the biopsy in 86%. CONCLUSIONS: The diagnostic accuracy of thoracoscopic lung biopsies in children with suspected ChILD is high. The histopathologic results lead to a disease-specific treatment in the majority of the cases. Thoracoscopic lung biopsy is a safe and effective procedure with a low complication rate.

Computed tomography donor liver volumetry before liver transplantation in infants ≤10 kg: does the estimated graft diameter affect the outcome?

AIM OF THE STUDY: Living donor liver transplantation (LDLT) is regularly performed in small-sized infants. Computed tomography (CT)-based donor liver volumetry is used to estimate the graft size. The aim of our study was to assess the results of CT liver volumetry and their impact on the clinical outcome after LDLT in extremely small-sized infants. PATIENTS AND METHODS: In this study, we included all patients with a body weight of ≤10 kg who underwent living related liver transplantation at our centre between January 2004 and December 2014. In all cases of LDLT, a preoperative CT scan of the donor liver was performed, and the total liver and graft volumes were calculated. The graft shape was estimated by measuring the ventro-dorsal (thickness), cranio-caudal, and transversal (width) diameter of segment II/III. We assessed the impact of CT donor liver volumetry and other risk factors on the outcome, defined as patient and graft survival. RESULTS: In the study period, a total of 48 living related liver transplantations were performed at our centre in infants ≤10 kg [20 male (42%), 28 female (58%)]. The mean weight was 7.3 kg (range 4.4-10 kg). Among the recipients, 33 (69%) received primary abdominal closure and 15 (31%) had temporary abdominal closure. The patient and graft survival rates were 85% and 81%, respectively. In CT volumetry, the mean estimated graft volume was 255 mL (range 140-485 mL) and the actual measured mean graft weight was 307 g (range 127-463 g). The mean ventro-dorsal diameter of segment II/III was 6.9 cm (range 4.3-11.2 cm), the mean cranio-caudal diameter was 9 cm (range 5-14 cm), and the mean width was 10.5 cm (range 6-14.7 cm). The mean graft-body weight ratio (GBWR) was 4.38% (range 1.41-8.04%). A high graft weight, a GBWR >4%, and a large ventro-dorsal diameter of segment II/III were risk factors for poorer patient survival. CONCLUSION: Preoperative assessment of the graft size is a crucial investigation before LDLT. For extremely small-sized recipients, not only the graft weight but also the graft shape seems to affect the outcome.