Rene Wijnen

Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe

Congenital diaphragmatic hernia (CDH) is associated with high mortality and morbidity. To date, there are no standardized protocols for the treatment of infants with this anomaly. However, protocols based on the literature and expert opinion might improve outcome. This paper is a consensus statement from the CDH EURO Consortium prepared with the aim of achieving standardized postnatal treatment in European countries. During a consensus meeting between high-volume centers with expertise in the treatment of CDH in Europe (CDH EURO Consortium), the most recent literature on CDH was discussed. Thereafter, 5 experts graded the studies according to the Scottish Intercollegiate Guidelines Network (SIGN) Criteria. Differences in opinion were discussed until full consensus was reached. The final consensus statement, therefore, represents the opinion of all consortium members. Multicenter randomized controlled trials on CDH are lacking. Use of a standardized protocol, however, may contribute to more valid comparisons of patient data in multicenter studies and identification of areas for further research.

Cultural Bias in the AAP’s 2012 Technical Report and Policy Statement on Male Circumcision

The American Academy of Pediatrics recently released its new Technical Report and Policy Statement on male circumcision, concluding that current evidence indicates that the health benefits of newborn male circumcision outweigh the risks. The technical report is based on the scrutiny of a large number of complex scientific articles. Therefore, while striving for objectivity, the conclusions drawn by the 8 task force members reflect what these individual physicians perceived as trustworthy evidence. Seen from the outside, cultural bias reflecting the normality of nontherapeutic male circumcision in the United States seems obvious, and the report’s conclusions are different from those reached by physicians in other parts of the Western world, including Europe, Canada, and Australia. In this commentary, a different view is presented by non–US-based physicians and representatives of general medical associations and societies for pediatrics, pediatric surgery, and pediatric urology in Northern Europe. To these authors, only 1 of the arguments put forward by the American Academy of Pediatrics has some theoretical relevance in relation to infant male circumcision; namely, the possible protection against urinary tract infections in infant boys, which can easily be treated with antibiotics without tissue loss. The other claimed health benefits, including protection against HIV/AIDS, genital herpes, genital warts, and penile cancer, are questionable, weak, and likely to have little public health relevance in a Western context, and they do not represent compelling reasons for surgery before boys are old enough to decide for themselves.

The incidence and morbidity of adhesions after treatment of neonates with gastroschisis and omphalocele: a 30-year review

BACKGROUND/PURPOSE Adhesive small bowel obstruction (SBO) is a feared complication after correction of abdominal wall defects in neonates. Knowledge of its incidence and potential risk factors in a well-documented group with strict follow-up is needed to guide preventive measures. METHODS Records of 170 neonates with abdominal wall defects, 59 gastroschisis (GS) and 111 omphalocele (OC), were reviewed focusing on SBO. Risk of SBO was calculated, and potential risk factors were analyzed. Long-term complaints possibly associated with adhesions were assessed through questionnaire. RESULTS One hundred forty-seven neonates were operated on, 12 were treated nonoperatively, and 11 patients died shortly after birth. Defects were primarily closed in 128, 7 neonates needed prosthetic mesh, and 12 had a silastic sac inserted. Twenty-six (18%) neonates had SBO, 14 (25%) of 55 with GS, and 12 (13%) of 92 with OC (P = .06). Of the 26 with SBO, 26 (88%) needed laparotomy. Four patients died because of SBO. Most episodes (85%) were in the first year. Sepsis and fascia dehiscence were predicting risk factors for SBO. Abdominal pain and constipation were frequent long-term complaints not significantly associated with SBO. CONCLUSIONS Adhesive SBO is a frequent and serious complication in the first year after treatment of congenital abdominal wall defects. Sepsis and fascial dehiscence are predictive factors.

In utero Repair of an Experimental Neural Tube Defect in a Chronic Sheep Model Using Biomatrices

Objective: Persistent exposure of the unprotected spinal cord to amniotic fluid and the uterine wall can lead to progressive damage of neural tissue in case of a myelomeningocele (two-hit hypothesis). The aim of this study was to evaluate whether in utero repair of an experimental neural tube defect in a fetal lamb could protect neural tissue from secondary injury and save neurologic functions after birth. Methods:In 19 fetal lambs, a neural tube defect was created at 79 days’ gestation. In 12 lambs the defect was covered either with a novel, molecular defined collagen-based biocompatible and biodegradable matrix (UMC) or with a small intestinal submucosa (SIS) biomatrix (Cook®) or by closing the skin over the defect. Results: All lambs with the defect covered showed no or minor neurologic morbidity in contrast to the lambs with the defect uncovered in which major neurologic morbidity was seen. Conclusions:These results demonstrate that long-term exposure of the open spinal cord to the intrauterine environment can lead to damage of neural tissue and, consequently loss of neurologic functions and that coverage of the defect can lead to a better neurologic outcome. Furthermore, we could show that a UMC biomatrix and an SIS biomatrix are useful for in utero coverage of a surgically created neural tube defect in our model.

Closure of giant omphaloceles by the abdominal wall component separation technique in infants

BACKGROUND/PURPOSE Several techniques have been described to repair giant omphaloceles. There is no procedure considered to be the criterion standard worldwide. The aim of the present prospective study was to analyze the early and late results of secondary closure of giant omphaloceles using the component separation technique (CST) in infants. METHODS From January 2004 to January 2007, 10 consecutive pediatric patients with a giant omphalocele were treated at our department. Initially, patients were treated conservatively. After epithelialization of the omphalocele, the abdominal wall was reconstructed using CST. Patients were monitored for complications during admission, and all patients were seen for follow-up. RESULTS Component separation technique was performed at median age of 6.5 months (range, 5-69 months). The median diameter of the hernia was 8 cm (range, 6-9 cm). There was no mortality. The postoperative course was uneventful in 7 patients. Complications were seen in 3 patients (infection, skin necrosis, and hematoma). Median hospital stay was 7 days. After median follow-up of 23.5 months (range, 3-39 month), no reherniations were found. CONCLUSIONS The CST is a safe 1-stage procedure for secondary closure in children with a giant omphalocele without the need for prosthetic material and with good clinical outcome.

International survey on the management of esophageal atresia

INTRODUCTION Because many aspects of the management of esophageal atresia (EA) are still controversial, we evaluated the practice patterns of this condition across Europe. METHODS A survey was completed by 178 delegates (from 45 [27 European] countries; 88% senior respondents) at the EUPSA-BAPS 2012. RESULTS Approximately 66% of respondents work in centers where more than five EA repairs are performed per year. Preoperatively, 81% of respondents request an echocardiogram, and only 43% of respondents routinely perform preoperative bronchoscopy. Approximately 94% of respondents prefer an open approach, which is extrapleural in 71% of respondents. There were no differences in use of thoracoscopy between Europeans (10%) and non-Europeans (11%, p = nonsignificant). Approximately 60% of respondents measure the gap intraoperatively. A transanastomotic tube (90%) and chest drain (69%) are left in situ. Elective paralysis is adopted by 56% of respondents mainly for anastomosis tension (65%). About 72% of respondents routinely request a contrast study on postoperative day 7 (2-14). Approximately 54% of respondents use parenteral nutrition, 40% of respondents start transanastomotic feeds on postoperative day 1, and 89% of respondents start oral feeds after postoperative day 5. Pure EA: 46% of respondents work in centers that repair two or more than two pure EA a year. About 60% of respondents opt for delayed primary anastomosis at 3 months (1-12 months) with gastrostomy formation without esophagostomy. Anastomosis is achieved with open approach by 85% of respondents. About 47% of respondents attempt elongation of esophageal ends via Foker technique (43%) or with serial dilations with bougies (41%). Approximately 67% of respondents always attempt an anastomosis. Gastric interposition is the commonest esophageal substitution. CONCLUSION Many aspects of EA management are lacking consensus. Minimally invasive repair is still sporadic. We recommend establishment of an EA registry.

Pancreatic injury in children: good outcome of nonoperative treatment.

PURPOSE Treatment of blunt injury of the pancreas in children remains controversial. Some prefer nonoperative treatment, whereas others prefer operative management in selected cases. This report reviews the treatment of patients with blunt pancreatic trauma admitted to a level I pediatric trauma center in The Netherlands. METHODS Medical records of all children less than 15 years with blunt pancreatic trauma admitted to the University Medical Center St Radboud in the period 1975 to 2003 were retrospectively analyzed. RESULTS Thirty-four children were included, age 3 to 14 years. Most injuries were because of bicycle accidents (58%). On admission, amylase was raised in 90% of the patients. Five patients had pancreatic duct injuries identified by imaging (endoscopic retrograde cholangiopancreaticography was used once, magnetic resonance cholangiopancreaticography twice) or at surgery. Thirty-one children were initially managed nonoperatively. Pancreatic surgery was performed in 3 children (1 Roux-Y, 2 drainage only). Mean hospital stay was 29 days in the operative group and 24 days in the nonoperative group. Fluid collections developed in 2 operated patients. Both resolved spontaneously. In 14 of the 31 nonoperated patients, a pseudocyst developed. Only 6 of these needed secondary intervention. Of these, 3 were drained percutaneously. There was no mortality and no long-term morbidity in both groups. CONCLUSIONS Nonoperative management of pancreatic injury in children has good clinical outcome. Only 10% need secondary surgery. In 50%, pseudocysts develop of which half can be managed nonoperatively. The reliability of computed tomographic scan grading is of limited value to decide whether to operate primarily. There is little to gain with ERCP and stenting. The place of MRCP as a noninvasive diagnostic tool remains to be determined.

Conventional Mechanical Ventilation Versus High-frequency Oscillatory Ventilation for Congenital Diaphragmatic Hernia: A Randomized Clinical Trial (The VICI-trial).

Objectives:To determine the optimal initial ventilation mode in congenital diaphragmatic hernia. Background:Congenital diaphragmatic hernia is a life-threatening anomaly with significant mortality and morbidity. The maldeveloped lungs have a high susceptibility for oxygen and ventilation damage resulting in a high incidence of bronchopulmonary dysplasia (BPD) and chronic respiratory morbidity. Methods:An international, multicenter study (NTR 1310), the VICI-trial was performed in prenatally diagnosed congenital diaphragmatic hernia infants (n = 171) born between November 2008 and December 2013, who were randomized for initial ventilation strategy. Results:Ninety-one (53.2%) patients initially received conventional mechanical ventilation and 80 (46.8%) high-frequency oscillation. Forty-one patients (45.1%) randomized to conventional mechanical ventilation died/ had BPD compared with 43 patients (53.8%) in the high-frequency oscillation group. An odds ratio of 0.62 [95% confidence interval (95% CI) 0.25–1.55] (P = 0.31) for death/BPD for conventional mechanical ventilation vs high-frequency oscillation was demonstrated, after adjustment for center, head-lung ratio, side of the defect, and liver position. Patients initially ventilated by conventional mechanical ventilation were ventilated for fewer days (P = 0.03), less often needed extracorporeal membrane oxygenation support (P = 0.007), inhaled nitric oxide (P = 0.045), sildenafil (P = 0.004), had a shorter duration of vasoactive drugs (P = 0.02), and less often failed treatment (P = 0.01) as compared with infants initially ventilated by high-frequency oscillation. Conclusions:Our results show no statistically significant difference in the combined outcome of mortality or BPD between the 2 ventilation groups in prenatally diagnosed congenital diaphragmatic hernia infants. Other outcomes, including shorter ventilation time and lesser need of extracorporeal membrane oxygenation, favored conventional ventilation.

Congenital diaphragmatic hernia with(out) ECMO: impaired development at 8 years.

Objective To evaluate developmental and social-emotional outcomes at 8 years of age for children with congenital diaphragmatic hernia (CDH), treated with or without neonatal extracorporeal membrane oxygenation (ECMO) between January 1999 and December 2003. Design Cohort study with structural prospective follow-up. Setting Level III University Hospital. Patients 35 children (ECMO: n=16; non-ECMO: n=19) were assessed at 8 years of age. Interventions None. Main outcome measures Intelligence and motor function. Concentration, behaviour, school performance, competence and health status were also analysed. Results Mean (SD) intelligence for the ECMO group was 91.7 (19.5) versus 111.6 (20.9) for the non-ECMO group (p=0.015). Motor problems were apparent in 16% of all participants and differed significantly from the norm (p=0.015) without differences between treatment groups. For all participants, problems with concentration (68%, p<0.001) and with behavioural attention (33%, p=0.021) occurred more frequently than in reference groups, with no difference between treatment groups. School performance and competence were not affected. Conclusions Children with CDH—whether or not treated with neonatal ECMO—are at risk for long-term morbidity especially in the areas of motor function and concentration. Despite their impairment, children with CDH have a well-developed feeling of self-competence.

Research perspectives in the etiology of congenital anorectal malformations using data of the International Consortium on Anorectal Malformations: evidence for risk factors across different populations

PurposeThe recently established International Consortium on Anorectal Malformations aims to identify genetic and environmental risk factors in the etiology of syndromic and nonsyndromic anorectal malformations (ARM) by promoting collaboration through data sharing and combined research activities.MethodsThe consortium attempts to recruit at least 1,000 ARM cases. DNA samples are collected from case–parent triads to identify genetic factors involved in ARM. Several genetic techniques will be applied, including SNP arrays, gene and whole exome sequencing, and a genome-wide association study. Questionnaires inquiring about circumstances before and during pregnancy will be used to obtain environmental risk factor data.ResultsCurrently, 701 ARM cases have been recruited throughout Europe. Clinical data are available from all cases, and DNA samples and questionnaire data mainly from the Dutch and German cases. Preliminary analyses on environmental risk factors in the Dutch and German cohort found associations between ARM and family history of ARM, fever during first trimester of pregnancy and maternal job exposure to cleaning agents and solvents.ConclusionFirst results show that both genetic and environmental factors may contribute to the multifactorial etiology of ARM. The International Consortium on Anorectal Malformations will provide possibilities to study and detect important genes and environmental risk factors for ARM, ultimately resulting in better genetic counseling, improved therapies, and primary prevention.