Identifying Preoperative Clinical Characteristics of Unexpected Gastrointestinal Perforation in Infants-A Retrospective Cohort Study.

BACKGROUND: Infants presenting with unexpected pneumoperitoneum upon abdominal X-ray, indicating a gastrointestinal perforation (GIP), have a surgical emergency with potential morbidity and mortality. Preoperative determination of the location of perforation is challenging but will aid the surgeon in optimizing the surgical strategy, as colon perforations are more challenging than small bowel perforations. Therefore, the aim of this study is to provide an overview of preoperative patient characteristics, determine the differences between the small bowel and colon, and determine underlying causes in a cohort of infants with unexpected GIP. METHODS: All infants (age ≤ 6 months) who presented at our center with unexpected pneumoperitoneum (no signs of pneumatosis before) undergoing surgery between 1996 and 2024 were retrospectively included. The differences between the location of perforation were analyzed using chi-squared and t-tests. Bonferroni correction was used to adjust for multiple tests. RESULTS: In total, 51 infants presented with unexpected pneumoperitoneum at our center, predominantly male (N = 36/51) and premature (N = 40/51). Among them, twenty-six had small bowel, twenty-two colon, and three stomach perforations. Prematurity (p = 0.001), birthweight < 1000 g (p = 0.001), respiratory support (p = 0.001), and lower median arterial pH levels (p = 0.001) were more present in patients with small bowel perforation compared with colon perforations. Pneumatosis intestinalis was more present in patients with colon perforation (p = 0.004). All patients with Hirschsprung disease and cystic fibrosis had colon perforation. The final diagnoses were mainly focal intestinal perforations (N = 27/51) and necrotizing enterocolitis (N = 9/51). CONCLUSIONS: Infants with unexpected GIP, birthweight < 1000 g, and prematurity have more risk for small bowel perforation. In case of colon perforation, additional screening (for Hirschsprung and cystic fibrosis) should be considered.

Development of an international core outcome set for treatment trials in paediatric inguinal hernia: protocol for a three-phase study including a systematic review and Delphi survey.

INTRODUCTION: In children, open inguinal hernia repair has been the gold standard for treatment, but with recent technical advancements in laparoscopy, laparoscopic hernia repair is gaining popularity. Despite available results from comparative studies, there is still no consensus regarding the superiority of open versus laparoscopic treatment strategy. An important reason for lack of consensus is the large heterogeneity in the trials' reported outcomes and outcome definitions, which limits comparisons between studies and precludes conclusions regarding the superiority of treatment strategies. The development and implementation of a core outcome set (COS) is a solution for this heterogeneity in the selection, measurement and reporting of trial outcome measures across studies. Currently, there is no COS for the treatment of paediatric inguinal hernia. METHODS AND ANALYSIS: The aim of this project is to reach international consensus on a minimal set of outcomes that should be measured and reported in all future clinical trials investigating inguinal hernia repair in children. The development process comprises three phases. First, we identify outcome domains associated with paediatric inguinal hernia repair from a patient perspective and through a systematic review of the literature using EMBASE, MEDLINE and the Cochrane Library databases. Second, we conduct a three-step Delphi study to identify and prioritise 'core' outcomes for the eventual minimal set. In the third phase, an expert meeting is held to establish the final COS and develop implementation strategies with participants from all stakeholder groups: healthcare professionals, parents and patients' representatives. The final COS will be reported in accordance with the COS-Standards for Reporting statement. ETHICS AND DISSEMINATION: The medical research ethics committee of the Amsterdam UMC confirmed that the Dutch Medical Research Involving Human Subjects Act (WMO) does not apply to this study and that full approval by the committee is not required. Electronic informed consent will be obtained from all participants. Results will be presented in peer-reviewed academic journals and at relevant conferences. PROSPERO REGISTRATION NUMBER: CRD42021281422.

Appendicitis and its associated mortality and morbidity in infants up to 3 months of age: A systematic review.

Background and Aims: Although appendicitis is rare in young infants, the reported mortality is high. Primary aim of this systematic review was to provide updated insights in the mortality and morbidity (postoperative complications, Clavien-Dindo grades I-IV) of appendicitis in infants ≤3 months of age. Secondary aims comprised the evaluation of patient characteristics, diagnostic work-up, treatment strategies, comorbidity, and factors associated with poor outcome. Methods: This systematic review was reported according to the PRISMA statement with a search performed in Pubmed, Embase and Web of Science (up to September 5th 2022). Original articles (published in English ≥1980) reporting on infants ≤3 months of age with appendicitis were included. Both patients with abdominal appendicitis and herniated appendicitis (such as Amyand's hernia) were considered. Data were provided descriptively. Results: In total, 131 articles were included encompassing 242 cases after identification of 4294 records. Overall, 184 (76%) of the 242 patients had abdominal and 58 (24%) had herniated appendicitis. Two-hundred (83%) of the patients were newborns (≤28 days) and 42 (17%) were infants between 29 days and ≤3 months of age. Either immediate, or after initial conservative treatment, 236 (98%) patients underwent surgical treatment. Some 168 (69%) patients had perforated appendicitis. Mortality was reported in 20 (8%) patients and morbidity in an additional 18 (8%). All fatal cases had abdominal appendicitis and fatal outcome was relatively more often reported in newborns, term patients, patients with relevant comorbidity, nonperforated appendicitis and those presented from home. Conclusion: Mortality was reported in 20 (8%) infants ≤3 months of age and additional morbidity in 18 (8%). All patients with fatal outcome had abdominal appendicitis. Several patient characteristics were relatively more often reported in infants with poor outcome and adequate monitoring, early recognition and prompt treatment may favour the outcome.

Cardiac anomalies in children with congenital duodenal obstruction: a systematic review with meta-analysis.

BACKGROUND: Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain unknown. Therefore, the aim of this systematic review is to aggregate the available literatures on cardiac anomalies in patients with DO. METHODS: In July 2022, a search was performed in PubMed and Embase.com. Studies describing cardiac anomalies in patients with congenital DO were considered eligible. Primary outcome was the pooled percentage of cardiac anomalies in patients with DO. Secondary outcomes were the pooled percentages of the types of cardiac anomalies, type of DO, and trisomy 21. A meta-analysis was performed to pool the reported data. RESULTS: In total, 99 publications met our eligibility data, representing 6725 patients. The pooled percentage of cardiac anomalies was 29% (95% CI 0.26-0.32). The most common cardiac anomalies were persistent foramen ovale 35% (95% CI 0.20-0.54), ventricular septal defect 33% (95% CI 0.24-0.43), and atrial septal defect 33% (95% CI 0.26-0.41). The most prevalent type of obstruction was type 3 (complete atresias), with a pooled percentage of 54% (95% CI 0.48-0.60). The pooled percentage of Trisomy 21 in patients with DO was 28% (95% CI 0.26-0.31). CONCLUSION: This review shows cardiac anomalies are found in one-third of the patients with DO regardless of the presence of trisomy 21. Therefore, we recommend that patients with DO should receive preoperative cardiac screening. LEVEL OF EVIDENCE: II.

Variability in the Reporting of Baseline Characteristics, Treatment, and Outcomes in Esophageal Atresia Publications: A Systematic Review.

INTRODUCTION: As survival rates of infants born with esophageal atresia (EA) have improved considerably, research interests are shifting from viability to morbidity and longer-term outcomes. This review aims to identify all parameters studied in recent EA research and determine variability in their reporting, utilization, and definition. MATERIALS AND METHODS: Following PRISMA guidelines, we performed a systematic review of literature regarding the main EA care process, published between 2015 and 2021, combining the search term "esophageal atresia" with "morbidity," "mortality," "survival," "outcome," or "complication." Described outcomes were extracted from included publications, along with study and baseline characteristics. RESULTS: From 209 publications that met the inclusion criteria, 731 studied parameters were extracted and categorized into patient characteristics (n = 128), treatment and care process characteristics (n = 338), and outcomes (n = 265). Ninety-two of these were reported in more than 5% of included publications. Most frequently reported characteristics were sex (85%), EA type (74%), and repair type (60%). Most frequently reported outcomes were anastomotic stricture (72%), anastomotic leakage (68%), and mortality (66%). CONCLUSION: This study demonstrates considerable heterogeneity of studied parameters in EA research, emphasizing the need for standardized reporting to compare results of EA research. Additionally, the identified items may help develop an informed, evidence-based consensus on outcome measurement in esophageal atresia research and standardized data collection in registries or clinical audits, thereby enabling benchmarking and comparing care between centers, regions, and countries.

Routine contrast enema prior to stoma reversal seems only required following treatment for necrotizing enterocolitis: An evaluation of the diagnostic accuracy of the contrast enema.

INTRODUCTION: Contrast enemas are often made prior to stoma reversal in order to detect distal intestinal strictures distal of the stoma. If untreated these strictures can cause obstruction which might necessitate redo-surgery. However, the value of contrast enemas is unclear. Therefore, we aim to evaluate the contrast enema's diagnostic accuracy in detecting strictures in children with a stoma. METHODS: Young children (≤3 years) treated with a stoma between 1998 and 2018 were retrospectively included. The STARD criteria were followed. Patients treated for anorectal malformations and those that died before stoma reversal were excluded. Surgical identification of strictures during reversal or redo-surgery within three months was used as gold standard. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the curve (AUC) reflected diagnostic accuracy. RESULTS: In 224 included children, strictures were found during reversal in 10% of which 95% in patients treated for necrotizing enterocolitis. Contrast enema was performed in 68% of all patients and detected 92% of the strictures. In the overall cohort, the sensitivity was 100%, specificity 98%, PPV 88% and NPV 100% whilst the AUC was 0.98. In patients treated for NEC, the sensitivity was 100%, specificity 97%, PPV 88% and NPV 100% whilst the AUC was 0.98. CONCLUSION: Strictures prior to stoma reversal seem to be mainly identified in patients treated for NEC and not in other diseases necessitating a stoma. Moreover, the contrast enema shows excellent diagnostic accuracy in detecting these strictures. For this reason we advise to only perform contrast enemas in patients treated for NEC. LEVEL OF EVIDENCE: II.

The Incidence of Associated Anomalies in Children with Congenital Duodenal Obstruction-A Retrospective Cohort Study of 112 Patients.

BACKGROUND: Duodenal obstruction (DO) is a congenital anomaly that is highly associated with other anomalies, such as cardiac anomalies and trisomy 21. However, an overview of additional anomalies and patient-specific risk factors for cardiac anomalies is lacking. Potential association with the vertebral, anorectal, cardiac, trachea-esophageal, renal and limb anomalies (VACTERL) spectrum remains unknown. Therefore, we aim to examine the incidence of associated anomalies, a VACTERL-spectrum association and patient-specific risk factors for cardiac anomalies in patients with DO. METHODS: A retrospective cohort study was performed between 1996 and 2021. Outcomes were the presence of any additional anomalies. Risk factors for cardiac anomalies were analyzed using multivariate logistic regression. RESULTS: Of 112 neonates with DO, 47% (N = 53/112) had one associated anomaly and 38% (N = 20/53) had multiple anomalies. Cardiac anomalies (N = 35/112) and trisomy 21 (N = 35/112) were present in 31%. In four patients, VACTERL-spectrum was discovered, all with cardiac anomalies. Trisomy 21 was found to be a risk factor for cardiac anomalies (OR:6.5; CI-95%2.6-16.1). CONCLUSION: Associated anomalies were present in half of patients with DO, of which cardiac anomalies and trisomy 21 occurred most often, and the VACTERL-spectrum was present in four patients. Trisomy 21 was a significant risk factor for cardiac anomalies. Therefore, we recommend a preoperative echocardiogram in patients with DO. In case a cardiac anomaly is found without trisomy 21, VACTERL-screening should be performed.

Identification of the optimal treatment strategy for complex appendicitis in the paediatric population: a protocol for a multicentre prospective cohort study (CAPP study).

INTRODUCTION: In daily practice large heterogeneity in the treatment of children with complex appendicitis exists. Complex appendicitis can be divided into two subtypes; complex appendicitis with and without appendiceal mass and/or abscess. As complex appendicitis is associated with high morbidity and costs, identification of the optimal treatment strategy is essential. In this article, we present the study protocol for the CAPP (Complex Appendicitis in the Pediatric Population) study. METHODS AND ANALYSIS: This nation-wide, multi-centre, comparative, non-randomised prospective cohort study includes all children <18 years old with a preoperative suspicion of complex appendicitis, which is based on imaging confirmed acute appendicitis and predefined criteria regarding the severity of appendicitis. Eligible patients are recruited in more than 30 hospitals. Open appendectomy will be compared with laparoscopic appendectomy for children without appendiceal mass and/or abscess and initial non-operative treatment (ie, intravenous antibiotics with or without percutaneous drainage) to direct appendectomy for children with appendiceal mass and/or abscess. Based on historical data supplied by the participating hospitals and an inclusion period of 2 years and 9 months, a sample size of 1308 patients is aimed. Primary outcome is the proportion of patients experiencing any complication at 3 months follow-up. Reported complications will be assessed by an independent adjudication committee. Secondary outcomes include, but are not limited to, quality of life, and (in)direct costs. To adjust for baseline differences and selection bias, outcomes will be compared after propensity score analysis (inverse probability weighting and stratification). ETHICS AND DISSEMINATION: The Medical Ethics Review Committee of the Amsterdam UMC, location AMC, declared that the Medical Research involving Human Subjects Act (WMO) did not apply to this study. Therefore, no official approval was required by national law. Study results will be presented in peer-reviewed scientific journals and at (inter)national conferences. TRIAL REGISTRATION NUMBERS: NCT04755179; NL9371.

Outcomes after appendectomy in children with acute appendicitis treated at a tertiary paediatric centre: results from a retrospective cohort study.

PURPOSE: In general, an appendectomy is presumed to have a limited burden of disease. However, in current literature, reported complication rates vary. This study aims to provide additional insights in the incidence of post-appendectomy complications in children with acute appendicitis. METHODS: This retrospective cohort study included children (0-17 years old) that underwent appendectomy at our tertiary referral centre for suspected acute appendicitis (January 2011-December 2018). Children referred to our centre, and those that underwent non-operative treatment were excluded. Post-appendectomy complications were recorded from electronic medical charts using predefined definitions and classified as severe (Clavien-Dindo III-IV) or less severe (Clavien-Dindo I-II). RESULTS: A total of 131 children were included. Simple and complex appendicitis was diagnosed in 66 (50%) and 60 (46%) children, respectively. A non-inflamed appendix was seen in five (4%) children. One or more complications were identified in 33 (25%) patients. Eight (12%) children with simple appendicitis developed a complication, three of these were severe. In children with complex appendicitis, 23 (38%) children developed a complication, 14 of these were severe. CONCLUSION: This study shows a high rate of complications compared with current literature, both in children with simple and complex appendicitis. This is probably the result of our definition of complications and being a tertiary referral centre receiving more severe appendicitis cases. However, these results still show that appendectomy is not always a routine procedure with only few complications. Substantiating the need to keep optimizing treatment for children with appendicitis.

The Dutch Incidence of Infantile Hypertrophic Pyloric Stenosis and the Influence of Seasons.

INTRODUCTION: Studies report contradicting results on the incidence of infantile hypertrophic pyloric stenosis (IHPS) and its association with seasons. We aim to assess the IHPS incidence in the Netherlands and to determine whether seasonal variation is present in a nationwide cohort. MATERIALS AND METHODS: All infants with IHPS hospitalized in the Netherlands between 2007 and 2017 were included in this retrospective cohort study. Incidence rates per 1,000 livebirths (LB) were calculated using total number of LB during the matched month, season, or year, respectively. Seasonal variation based on month of birth and month of surgery was analyzed using linear mixed model and one-way ANOVA, respectively. RESULTS: A total of 2,479 infants were included, of which the majority was male (75.9%). Median (interquartile range) age at surgery was 34 (18) days. The average IHPS incidence rate was 1.28 per 1,000 LB (variation: 1.09-1.47 per 1,000 LB). We did not find a conclusive trend over time in IHPS incidence. Differences in incidence between season of birth and season of surgery were not significant (p = 0.677 and p = 0.206, respectively). CONCLUSION: We found an average IHPS incidence of 1.28 per 1,000 LB in the Netherlands. Our results showed no changing trend in incidence and no seasonal variation.

Current Opinions and Practices of Bariatric Surgery in Adolescents: A Survey among Pediatric Surgeons.

INTRODUCTION: Bariatric surgery is performed at increasing rate in severely obese adolescents who do not respond to conservative treatment. In the United States, this treatment is generally accepted, yet in Europe, surgeons are more reluctant because of concerns regarding safety and (long-term) efficacy. We evaluated in which (European) countries bariatric surgery is allowed and performed, and the opinion of the members of the European Paediatric Surgeons' Association (EUPSA) regarding bariatric surgery in adolescents. MATERIALS AND METHODS: Information was obtained with an online questionnaire sent to all EUPSA members. RESULTS: A total of 108 pediatric surgeons (PSs) from 33 countries (of which 25 European) completed the survey. Sixty-two PSs (57.4%) from 22 countries stated that bariatric surgery in adolescents was allowed in their country. In only 14 countries, the costs were (partially) covered by health care insurances. Overall, 65.7% of the surgeons (n = 71) believed bariatric surgery may offer a valuable contribution to obtain substantial long-term weight loss in severely obese adolescents. Fifty-one (47.2%) reported that these procedures should be performed by a combination of a bariatric and a PS, while 20 (18.5%) and 16 (14.8%) preferred that these procedures should be performed solely by a PS or bariatric surgeon, respectively. CONCLUSION: Although allowed in most European countries, bariatric surgery in adolescents is only practiced on a small scale, and the treatment is often excluded from regular health care reimbursement. The majority of PSs acknowledge the value of bariatric surgery, which should be performed by a combination of PS and bariatric surgeon.

Long-term results after transplantation of pediatric liver grafts from donation after circulatory death donors.

BACKGROUND: Liver grafts from donation after circulatory death (DCD) donors are increasingly accepted as an extension of the organ pool for transplantation. There is little data on the outcome of liver transplantation with DCD grafts from a pediatric donor. The objective of this study was to assess the outcome of liver transplantation with pediatric DCD grafts and to compare this with the outcome after transplantation of livers from pediatric donation after brain death (DBD) donors. METHOD: All transplantations performed with a liver from a pediatric donor (≤16 years) in the Netherlands between 2002 and 2015 were included. Patient survival, graft survival, and complication rates were compared between DCD and DBD liver transplantation. RESULTS: In total, 74 liver transplantations with pediatric grafts were performed; twenty (27%) DCD and 54 (73%) DBD. The median donor warm ischemia time (DWIT) was 24 min (range 15-43 min). Patient survival rate at 10 years was 78% for recipients of DCD grafts and 89% for DBD grafts (p = 0.32). Graft survival rate at 10 years was 65% in recipients of DCD versus 76% in DBD grafts (p = 0.20). If donor livers in this study would have been rejected for transplantation when the DWIT ≥30 min (n = 4), the 10-year graft survival rate would have been 81% after DCD transplantation. The rate of non-anastomotic biliary strictures was 5% in DCD and 4% in DBD grafts (p = 1.00). Other complication rates were also similar between both groups. CONCLUSIONS: Transplantation of livers from pediatric DCD donors results in good long-term outcome especially when the DWIT is kept ≤30 min. Patient and graft survival rates are not significantly different between recipients of a pediatric DCD or DBD liver. Moreover, the incidence of non-anastomotic biliary strictures after transplantation of pediatric DCD livers is remarkably low.

Esophageal mucosal integrity improves after laparoscopic antireflux surgery in children with gastroesophageal reflux disease.

BACKGROUND: Esophageal intraluminal baseline impedance reflects the conductivity of the esophageal mucosa and may be an instrument for in vivo evaluation of mucosal integrity in children with gastroesophageal reflux disease (GERD). Laparoscopic antireflux surgery (LARS) is a well-established treatment option for children with proton pump inhibitory (PPI) therapy resistant GERD. The effect of LARS in children on baseline impedance has not been studied in detail. The aim of this study was to evaluate the effect of LARS on baseline impedance in children with GERD. METHODS: This is a prospective, multicenter, nationwide cohort study (Dutch national trial registry: NTR2934) including 25 patients [12 males, median age 6 (range 2-18) years] with PPI-resistant GERD scheduled to undergo LARS. Twenty-four hour multichannel intraluminal impedance pH monitoring (MII-pH monitoring) was performed before and 3 months after LARS. Baseline impedance was evaluated during consecutive 2-h intervals in the 24-h tracings. RESULTS: LARS reduced acid exposure time from 8.5 % (6.0-16.2 %) to 0.8 % (0.2-2.8 %), p < 0.001. Distal baseline impedance increased after LARS from 2445 Ω (1147-3277 Ω) to 3792 Ω (3087-4700 Ω), p < 0.001. Preoperative baseline impedance strongly correlated with acid exposure time (r -0.76, p < 0.001); however, no association between symptomatic outcome and baseline impedance was identified. CONCLUSIONS: LARS significantly increased baseline impedance likely reflecting recovery of mucosal integrity. As the change in baseline impedance was not associated with the clinical outcome of LARS, other factors besides mucosal integrity may contribute to symptom perception in children with GERD.

Patient-Reported Defecation and Micturition Problems Among Adults Treated for Sacrococcygeal Teratoma During Childhood--The Need for New Surveillance Strategies.

BACKGROUND: To evaluate defecation and micturition complaints in adults treated for sacrococcygeal teratoma (SCT) during childhood and to identify risk factors for soiling, urinary incontinence, and constipation beyond childhood. PROCEDURE: Records of patients aged ≥18 treated for SCT during infancy in the Netherlands were retrospectively reviewed. Frequency and severity of soiling, constipation, and urinary incontinence were evaluated using questionnaires designed in line with the Krickenbeck classification. Problems during childhood were compared to outcomes at adult age in part of the cohort. Associations between patient- and disease-related factors with complaints beyond childhood were analyzed with the chi-square test or Fisher's exact test, when appropriate. RESULTS: Of 47 included patients (mean age 26.2 years, SD ±6.5), 49% reported at least one defecation or micturition complaint. Urinary incontinence was present in 30% and had a greater negative impact than soiling (24%). Ten patients (21%) reported constipation; five found this severely bothering. Three patients reported social restrictions due to defecation or micturition complaints (6.4%). While sex and tumor histology were not identified as risk factors, a tumor diameter of >10 cm and Altman type I or type II SCT were associated with constipation during adulthood. CONCLUSIONS: One-third of the patients treated for SCT during childhood reported urinary and defecation problems beyond childhood. In only a minority of cases, these led to social restrictions. A greater tumor diameter was associated with a higher risk of constipation during adulthood. Prolonged surveillance strategies are advised for all patients with SCT.

Evaluation of pregnancy and delivery in 13 women who underwent resection of a sacrococcygeal teratoma during early childhood.

BACKGROUND: Sacrococcygeal teratoma resection often brings changes in pelvic anatomy and physiology with possible consequences for defecation, micturition and sexual function. It is unknown, whether these changes have any gynecological and obstetric sequelae. Until now four pregnancies after sacrococcygeal teratoma resection have been described and cesarean section has been suggested to be the method of choice for delivery. We evaluated the pregnancy course and mode of delivery in women previously treated for a sacrococcygeal teratoma. METHODS: The records of all patients who underwent sacrococcygeal teratoma resection after 1970 in one of the six pediatric surgical centers in the Netherlands were reviewed retrospectively. Women aged 18 years and older were eligible for participation. Patient characteristics, details about the performed operation and tumor histology were retrieved from the records. Consenting participants completed a questionnaire addressing fertility, pregnancy and delivery details. RESULTS: Eighty-nine women were eligible for participation; 20 could not be traced. Informed consent was received from 41, of whom 38 returned the completed questionnaire (92.7%). Thirteen of these 38 women conceived, all but one spontaneously. In total 20 infants were born, 17 by vaginal delivery and 3 by cesarean section, in one necessitated by previous intra-abdominal surgery as a consequence of sacrococcygeal teratoma resection. Conversion to a cesarean section was never necessary. None of the 25 women without offspring reported involuntary childlessness. CONCLUSIONS: There are no indications that resection of a sacrococcygeal teratoma in female patients is associated with reduced fertility: spontaneous pregnancy is possible and vaginal delivery is safe for mother and child, irrespective of the sacrococcygeal teratoma classification or tumor histology.

Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease.

OBJECTIVES: A nonrelaxing internal anal sphincter is present in a relatively large proportion of children with surgically treated Hirschsprung disease (HD) and can cause obstructive gastrointestinal symptoms. The short- and long-term outcome and adverse effects of intrasphincteric botulinum toxin (Botox) injections in children with obstruction after surgically treated HD are evaluated. METHODS: The outcome of children with surgically treated HD treated with intrasphincteric Botox injections for obstructive symptoms was analyzed with a retrospective chart review between 2002 and 2013 in the University Medical Centers of Maastricht and Nijmegen. RESULTS: A total of 33 patients were included. The median time of follow-up was 7.3 years (range 1-24). A median of 2 (range 1-5) injections were given. Initial improvement was achieved in 76%, with a median duration of 4.1 months (range 1.7-58.8). Proportion of children hospitalized for enterocolitis decreased after treatment from 19 to 7. A good long-term response was found in 49%. Two children experienced complications: transient pelvic muscle paresis with impairment of walking. In both children symptoms resolved within 4 months without treatment. CONCLUSIONS: Intrasphincteric Botox injections in surgically treated HD are an effective long-term therapy in approximately half of our patients with obstructive symptoms. The possibility of adverse effects should be noticed.

Variability in protocols on donation after circulatory death in Europe.

INTRODUCTION: Organ donation after circulatory death (DCD) has become an accepted strategy to reduce the shortage of organs for transplantation in many European countries. The use and number of DCD donors varies between countries. The purpose of this study was to evaluate the available protocols for DCD in Europe. METHODS: We contacted national transplant societies and responsible transplant co-ordinators in the countries that perform DCD to obtain DCD protocols. We compared information on the protocols and additional data including: inclusion and exclusion criteria for donation, legislation, determination of death and preservation methods. RESULTS: In ten European countries DCD is performed, eight of which describe the methods in protocols. There are large differences in used DCD categories, legislation and the way death is determined. Protocols differ in the detail in which DCD procedures are described and the way methods are supported by additional consensus statements and ethical frameworks. CONCLUSIONS: Although DCD is an established strategy to enlarge the donor pool and to contribute to the reduction of the waiting list for transplantation, its potential has not been fully utilized yet. To further promote DCD transplantation, it is important to share expertise and obtain consensus, so that this can be translated into more uniform and solid protocols supported by the competent authorities, transplant and intensive care professionals, which may eventually result in a further promotion of DCD transplantation in Europe.

Transplantation of kidneys from paediatric DCD donors: a comparison with DBD donors.

BACKGROUND: Although acceptable outcomes have been reported in kidney transplantation from donation after cardiac death (DCD), little is known about kidney transplantation from paediatric DCD. The objective of this study was to compare the outcome of kidney transplantation using paediatric DCD with the outcome of paediatric donation after brain death (DBD). METHODS: Recipients from DCD and DBD donors <18 years of age transplanted in the Netherlands between January 1981 and July 2006 were included in this study. Ninety-one patients were transplanted with kidneys from paediatric DCD donors and 405 patients received grafts from paediatric DBD donors. RESULTS: Grafts from DCD donors were associated with higher percentage of primary non-function (9 versus 2%, P < 0.01) and delayed graft function (48 versus 8%, P < 0.001) compared with DBD donor grafts. Estimated glomerular filtration rate did not differ between groups (57 ± 17 versus 58 ± 21 mL/min at 1 year and 62 ± 14 versus 57 ± 22 mL/min at 5 years, respectively). After correction for confounding variables, the risk of graft failure was higher in the DCD group [hazard ratio 2.440 (95% confidence interval (CI) 1.280-4.650; P = 0.007]. Patient survival, however, was similar between groups [hazard ratio 1.559 (95% CI 0.848-2.867; P = 0.153)]. CONCLUSIONS: Paediatric DCD kidneys represent a valuable source of donor kidneys that has not been fully utilized. Although transplantation of paediatric DCD kidneys is associated with a higher risk of graft failure than transplantation of paediatric DBD kidneys, results are comparable with adult donors. We therefore conclude that paediatric DCD kidneys can be safely added to the donor pool.

A pilot study assessing the feasibility of a short period of normothermic preservation in an experimental model of non heart beating donor kidneys.

BACKGROUND: Restoring metabolism to an organ after hypothermic storage and before transplantation could reverse some of the detrimental effects of ischemic injury. This may be particularly beneficial for kidneys from non-heart-beating (NHBD) donors that sustain significant periods of warm and cold ischemic injury. This pilot study assessed the feasibility of a short period of normothermic preservation (NP) in a porcine autotransplant model. METHODS: Kidneys were subjected to 30 min of warm ischemia, then preserved by hypothermic machine perfusion (HMP) for 22 h or 20 h HMP followed by 2 h of NP using autologous blood. Kidneys were then re-implanted, a contralateral nephrectomy performed, and renal function measured over 10 d. RESULTS: Post-transplant, 4/6 animals survived in the NP group compared with 5/6 in the HMP group (P = 1.00). Creatinine levels fell below 250 µmol/L in all four of the surviving animals in the NP group compared with 2/5 in the HMP group (P = 0.608). There was no difference in levels of renal function (peak creatinine, HMP = 1736 ± 866 versus NP = 1553 ± 516 µmol/L; P ≥ 0.990). Levels of lipid peroxidation were significantly lower 60 min post-transplant in the NP group (NP = 477 ± 118.0 versus HMP = 671 ± 99.4 pg/mL; P = 0.026). CONCLUSION: A period of NP at the end of the renal preservation period in NHB kidneys is a feasible method of kidney preservation. NP could prove to be a useful technique to predetermine graft function and allow pre-transplant modification of organs.

A pilot study on potential new plasma markers for diagnosis of acute appendicitis.

BACKGROUND: Diagnosis of acute appendicitis (AA) remains a surgical dilemma, with negative appendectomy rates of 5% to 40% and perforation suggestive for late operative intervention in 5% to 30%. The aim of this study is to evaluate new plasma markers, representing early neutrophil activation, to improve diagnostic accuracy in patients suspected for AA. MATERIALS AND METHODS: Fifty-one patients who underwent surgery for AA were included (male-female = 28:23), and blood was sampled. Plasma concentrations of 2 neutrophil proteins were measured: lactoferrin (LF) and calprotectin (CP). Controls consisted of 27 healthy volunteers. C-reactive protein (CRP) and white blood cell count (WBC) concentrations were measured for routine patient care. RESULTS: Median plasma concentrations for LF and CP were significantly higher in 51 patients with proven AA (665 and 766 ng/mL, respectively) than in 27 healthy volunteers (198 and 239 ng/mL, respectively, P < .001). No clinically relevant correlation exists between the plasma levels of LF and CP and the conventional laboratory tests for CRP and WBC. CONCLUSIONS: Circulating LF and CP levels are significantly elevated in patients with appendicitis and are detectable in plasma using relatively simple and low-cost enzyme-linked immunosorbent assays. Furthermore, plasma levels of LF and CP give additional information to conventional markers WBC and CRP, making them potential new markers for AA diagnosis.