European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis.

BACKGROUND: The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care. METHOD: A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations. RESULTS: The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection. RECOMMENDATIONS: The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi's approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.

Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center.

BACKGROUND: Recurrent tracheoesophageal fistula (rTEF) is a well-known complication after surgery of EA, occurring in roughly 3-10% of the patients. Recent studies have highlighted safety and efficacy of endoscopic management of recurrent TEF. The aim of this study was to evaluate the efficacy of chemocauterization with trichloroacetic acid (TCA) in rTEF and congenital tracheoesophageal fistula (cTEF). METHODS: Retrospective chart review of consecutive patients with recurrent or congenital TEF who underwent endoscopic chemo-cauterization between 2018 and 2022 at a tertiary center. Children diagnosed with TEF who underwent primary or secondary endoscopic treatment were included. Median follow up time was 19 months for rTEF and 33 months for cTEF. RESULTS: During the study period, 18 patients were treated endoscopically by chemocatuerization with TCA at our institution. Treatment of recurrent TEF was successful in 13 of 14 patients (93%) Treatment of congenital TEF was successful in 2 of 4 patients (50%). In 14 patients, closure was seen after 1-2 treatments. There were no serious adverse reactions or complications to the endoscopic treatment of TEF. CONCLUSION: Endoscopic chemocauterization is a minimal invasive technique with low morbidity and high success rate and may be considered as primary treatment for recurrent TEF. LEVEL OF EVIDENCE: III.

Transitional Care for Patients with Congenital Colorectal Diseases: An EUPSA Network Office, ERNICA, and eUROGEN Joint Venture.

BACKGROUND: Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD. METHODS: A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported. RESULTS: In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs. CONCLUSION: Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future. LEVEL OF EVIDENCE: III.

Syndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry.

BACKGROUND: The aim of this study was to describe the incidence of Congenital Diaphragmatic Hernia, CDH, associated with known or clinically suspected syndromes, and the postnatal outcomes from a large database for CDH. METHODS: Data from the multicenter, multinational database on infants with CDH (Congenital Diaphragmatic Hernia Study Group Registry) born from 1996 to 2020 were analyzed. Patients with known or suspected syndromes were grouped and outcome data were analyzed and compared to those without syndromic features. RESULTS: A total of 12,553 patients were entered in the registry during the study period, and 421 had reported known syndromes, representing 3.4% of all CDH cases in the registry. A total of 50 different associated syndromes were reported. In addition to those with clinically suspected genetic conditions, a total rate of genetic syndromes with CDH was 8.2%. The overall survival to discharge for syndromic CDH was 34% and for non-syndromic CDH was 76.7%. The most common were syndromes Fryns syndrome (19.7% of all syndromes, 17% survival), trisomy 18 or Edward syndrome (17.5%, 9% survival), trisomy 21 or Down syndrome (9%, 47% survival), trisomy 13 or Patau syndrome (6.7%, 14% survival), Cornelia de Lange syndrome (6.4% of all syndromes, 22% survival) and Pallister-Killian syndrome (5.5% of all syndromes, 39.1% survival). In addition, 379 cases had reported chromosomal anomalies and 233 cases had clinically suspected syndromes, based on two more dysmorphic features or malformations in addition to CDH, but without molecular diagnosis. The syndromic CDH group had lower birth weight and gestational age at birth and increased incidence of bilateral CDH (2.9%) and rates of non-repair (53%). The length of hospital stay was longer, and larger number of patients needed O2 at 30 days. Extracorporeal life support was used only in 15% of the cases. Those who underwent surgical repair had survival to discharge rates of 73%. CONCLUSION: Syndromic CDH is rare and only 3.4% of the reported cases of CDH have a known syndrome or association, but, if including patients with two dysmorphic features malformations, in addition to CDH, altogether as many as 8.2% have a diagnosed or suspected genetic condition. These children have with lower survival rates. Given higher rates of non-repair and decreased extracorporeal life support use, along with a high early mortality, decision-making regarding goals of care clearly influences outcomes. Survival varies depending on the genetic cause. Early genetic diagnosis is important and may influence the decision-making.

Expanding neonatal ECMO criteria: When is the premature neonate too premature.

Extracorporeal membrane oxygenation (ECMO) is a universally accepted and life-saving therapy for neonates with respiratory or cardiac failure that is refractory to maximal medical management. Early studies found unacceptable risks of mortality and morbidities such as intracranial hemorrhage among premature and low birthweight neonates, leading to widely accepted ECMO inclusion criteria of gestational age ≥34 weeks and birthweight >2 kg. Although contemporary data is lacking, the most recent literature demonstrates increased survival and decreased rates of intracranial hemorrhage in premature neonates who are supported with ECMO. As such, it seems like the right time to push the boundaries of ECMO on a case-by-case basis beginning with neonates 32-34 weeks GA in large volume centers with careful neurodevelopmental follow-up to better inform practices changes on this select population.

Hidden cardiovascular morbidity in children and young adults born with congenital diaphragmatic hernia: A population-based study.

INTRODUCTION: Congenital diaphragmatic (CDH) hernia is a rare congenital malformation with considerable mortality and morbidity in the neonatal period. The majority of the children today survive but little is known about long term cardiovascular morbidity. MATERIAL AND METHODS: This was a nationwide population-based prospective case-control study within a cohort of Swedish children with CDH, born 1982-2015. Five controls for each patient were randomly sampled from the population. The outcomes were the corresponding International Statistical Classification of Disease (ICD) codes for cardiovascular diagnoses according to ICD 9 and 10. RESULTS: There was an overrepresentation of cardiovascular diagnoses in the CDH group after one year of age compared to the control group, 8.0vs 0.5% (n = 53 versus n = 16). The risk of having a cardiovascular diagnosis in this CDH group was 15 times higher compared to the control group (HR 15.8, 95% CI: 9-27.6, p < 0.005). The diagnoses of cardiac arrhythmias and systemic hypertension were less common in the CDH group before the age of one year compared to the CDH group beyond the age of one year. Arrhythmia 3.7 vs 15.1%, systemic hypertension 3.7 vs 7.5%. CONCLUSION: CDH survivors have increased cardiovascular morbidity during childhood and young adulthood. This implies that structured follow up programs, covering cardiovascular morbidity, needs to be developed and should be offered in pediatric and adult care. Being born with CDH seems to be a risk factor for future cardiovascular diagnoses. LEVEL OF EVIDENCE: Level 3: Case-Control Study.

The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations.

BACKGROUND: The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery. MATERIAL AND METHODS: A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed. RESULTS: Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery. CONCLUSION: Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms. LEVEL OF EVIDENCE: III.

Premature and Extracorporeal Life Support: Is it Time? A Systematic Review.

Early preterm birth < 34 gestational weeks (GA) and birth weight (BW) <2 kg are relative contraindications for extracorporeal membrane oxygenation (ECMO). However, with improved technology, ECMO is presently managed more safely and with decreasing complications. Thus, these relative contraindications may no longer apply. We performed a systematic review to evaluate the existing literature on ECMO in early and late (34-37 GA) prematurity focusing on survival to hospital discharge and the complication intracranial hemorrhage (ICH). Data sources: MEDLINE, PubMed, Web of Science, Embase, and the Cochrane Database. Only publications in the English language were evaluated. Of the 36 included studies, 23 were related to ECMO support for respiratory failure, 10 for cardiac causes, and four for congenital diaphragmatic hernia (CDH). Over the past decades, the frequency of ICH has declined (89-21%); survival has increased in both early prematurity (25-76%), and in CDH (33-75%), with outcome similar to late prematurity (48%). The study was limited by an inherent risk of bias from overlapping single-center and registry data. Both the risk of ICH and death have decreased in prematurely born treated with ECMO. We challenge the 34 week GA time limit for ECMO and propose an international task force to revise current guidelines. At present, gestational age < 34 weeks might no longer be considered a contraindication for ECMO in premature neonates.

Transport on extracorporeal membrane oxygenation for congenital diaphragmatic hernia: A unique center experience.

BACKGROUND: Support on Extracorporeal oxygenation membrane (ECMO) represents the last therapeutic option in the management of respiratory failure and pulmonary hypertension refractory to treatment in patients with congenital diaphragmatic hernia (CDH). AIM: The objective of this work was to present our experience of all the cases of CDH that we have transported on ECMO. MATERIAL AND METHODS: Medical records of patients, national and international, with CDH transported by our service on ECMO from 1997 to 2018 were reviewed. RESULTS: During 22 years, we performed 40 ECMO transports of newborns with CDH, 39 primary and one secondary. In 10% (4/40) we transferred patients from their primary hospital after the implantation of cannulae and commencement of ECMO to another center abroad owing to the lack of beds in our unit. Twenty (50%) of the transports were from a foreign country. Median transport distance was 560 (428-1381) km and the median transport time was 4.5 (4.2-6.3) h. The mode of transport was ground ambulance in 20%, helicopter in 10%, fixed wing aircraft in 62.5% and ground ambulance in Freight aircraft in 7.5%. In 40% of the transports, 20 complications occurred. In one of every four transports with complications, more than one event occurred. Most frequent complication was loss of tidal volumes (35%) and in 30% of the complications another patient related event was recorded. Equipment failure occurred in 20%, and climate problems and transport vehicle problems in 15%. No deaths occurred during transport. Venoarterial ECMO was used in 39 of the 40 cases. Survival to discharge was 87% for the entire period and long-term survival was 77%. CONCLUSIONS: Long and short distance interhospital transports of CDH patients on ECMO can be performed safely. Despite occurrence of adverse events, the risk of mortality is very low. The personnel involved must be highly competent in intensive care, physiology and physics of ECMO, cannulation, intensive care transport and air transport medicine. They must also be trained to recognize risk factors in these patients. LEVEL OF EVIDENCE: III Retrospective cohort study.

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome.

AIM: To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH. MATERIAL AND METHODS: We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position. RESULTS: 3746 cases of CDH were entered in the registry between 2007 and 2015, with an overall survival of 71%. Of those, 68% had a prenatal diagnosis. Survival rates were significantly better in the postnatally diagnosed group, 83 vs 65%. There was a higher proportion of bigger defect sizes, C and D, in the prenatally diagnosed group, but the survival rates were similar when patients were stratified by defect size. The rate of ECMO utilization was higher overall in the prenatally diagnosed group, 33 vs 22%, but it was similar within similar defect sizes. Right-sided defects are more commonly missed at prenatal screening than left-sided CDH, 53 vs 35% (p < 0.0001). CONCLUSIONS: Prenatally diagnosed CDH is associated with larger defect sizes compared to those with a postnatal diagnosis, and consequently have higher morbidity and mortality. Right-sided CDH are more often missed at prenatal ultrasound. The increasing rate of prenatal detection requires a clear understanding of accurate risk stratification, in order to counsel families and to provide appropriate perinatal management. LEVEL OF EVIDENCE: I for a Prognosis Study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).

Correction to: The Met Needs for Pediatric Surgical Conditions in Sierra Leone: Estimating the Gap.

In the original article, Johan von Schreeb's last name was spelled incorrectly. It is correct as reflected here.

Closer location of the tracheoesophageal fistula to the carina in newborns with esophageal atresia and VACTERL association.

BACKGROUND: VACTERL is a complicated syndrome with an unknown etiology where many studies have failed to identify the cause. In esophageal atresia (EA) roughly 10%-23% also have concurrent anomalies that align with VACTERL disorder. The aim of this study is to investigate if there is a difference regarding localization of the tracheoesophageal fistula between patients with VACTERL and non-VACTERL patients. METHODS: Retrospective chart review of newborn operated for esophageal atresia between 2006 and 2016 at our Institution was performed. Children with a C-type fistula according to Gross and reliable preoperative tracheoesophageal fistula to carina distance measurement at rigid tracheoscopy were included in the study. RESULTS: A total of 90 patients were included in the study. Fifteen of those were diagnosed with VACTERL. Before and after adjusting for weight and gestational week patients with VACTERL had significantly shorter carina to fistula distance at perioperative rigid tracheoscopy (p = <0.001 nonadjusted, p = 0.016 adjusted). CONCLUSION: Patients with VACTERL born with EA type C had shorter carina to fistula distance as shown at perioperative rigid tracheoscopy. The significantly shorter distance may not only present surgical difficulties but may also suggest a structural or molecular difference in the development of the esophageal atresia between the two groups. LEVEL OF EVIDENCE: III.

Behavioral, emotional and social functioning in children born with congenital diaphragmatic hernia.

PURPOSE: The aim was to investigate social competence and behavioral and emotional problems in children and adolescents born with CDH. METHODS: All children born with CDH, treated in Stockholm 1990-2009, were invited to participate. After written consent, the Child Behavior Checklist or Adult Self-Report questionnaires were sent to participants. Of the 145 long-term survivors, 51% returned a completed questionnaire. Both the syndrome and competence scales were used and open-ended questions were analyzed with manifest content analysis. RESULTS: All parents of children aged 1.5-5 years and 90% of parents of children aged 6-18 years reported a normal range on the syndrome scale. Five parents indicated internalizing, but none externalizing behavior. All young adults achieved a normal score on the syndrome scale. Eighty-five percent had normal school achievement, 79% had normal social scores and 40% had normal activity levels. Significantly fewer boys (23%) were in the normal activity range compared with 67% of girls. CONCLUSIONS: The vast majority of all parents of children born with CDH scored no behavioral or emotional problems, furthermore, they reported normal social and school competence. However, the activity levels seemed to be reduced in children born with CDH.

Accuracy and impact of prenatal diagnosis in infants with omphalocele.

BACKGROUND: Associated anomalies in omphalocele are common, but to which extent these anomalies are diagnosed before or after birth is less well documented. AIM: To investigate the different types of associated anomalies, long-term survival and the extent whether these are diagnosed pre- or postnatally in children with a prenatal diagnosis of omphalocele at a single institution. MATERIALS AND METHODS: Retrospective review of all pregnancies with omphalocele managed and/or born at our institution between 2006 and 2016. RESULTS: A total of 42 cases with prenatally diagnosed omphalocele were identified. Of those 14 (31%) decided to terminate the pregnancy (TOP). Of the remaining 28 that continued, 12 were giant omphaloceles. The overall mortality rate was 18, 25% for giant and 12% for non-giant omphaloceles. 64% had associated anomalies. Only 1/3 of these anomalies is diagnosed prenatally. CONCLUSION: The rate of associated malformations that are diagnosed postnatally is high, but the majority was malformations with a minor clinical significance or impact on future health. Beckwith-Wiedemann syndrome was present only in cases of non-giant omphalocele in our cohort.

Health-related quality of life in children born with congenital diaphragmatic hernia.

PURPOSE: The aim of this study was to examine health-related quality of life (HRQoL) in children born with congenital diaphragmatic hernia (CDH). METHODS: Between 1993 and 2003, a total of 102 children born with CDH were treated at Astrid Lindgren Children's hospital in Stockholm. In 2012, long-term survivors (n = 77) were asked to participate in the present study, which resulted in a 46% (n = 35) response rate. The KIDSCREEN-52 questionnaire was used for measuring HRQoL and a detailed review of medical records was performed. RESULTS: The study participants did not differ from the non-participants in terms of prenatal diagnosis, gender, side of lesion, method of surgical repair, time to intubation, need for ECMO support, or way of discharge from the hospital. Children born with CDH considered themselves to have a good HRQoL, as good as healthy Swedish children. There were only a few significant HRQoL differences within the group of children with CDH, although several median scores in ECMO-treated patients were somewhat lower. Correlations between child and parent scores on HRQoL were low. CONCLUSIONS: Health-related quality of life in children born with CDH is good overall, however, a correlation between the severity of the malformation and HRQoL cannot be excluded.

Lung function and pulmonary artery blood flow following prenatal maternal retinoic acid and imatinib in the nitrofen model of congenital diaphragmatic hernia.

BACKGROUND: Lung and pulmonary vascular maldevelopment in congenital diaphragmatic hernia (CDH) results in significant morbidity and mortality. Retinoic acid (RA) and imatinib have been shown to improve pulmonary morphology following prenatal administration in the rat nitrofen-induced CDH model. It remains unclear if these changes translate into improved function. We evaluated the effect of prenatal RA and imatinib on postnatal lung function, structure, and pulmonary artery (PA) blood flow in the rat CDH model. METHODS: Olive oil or nitrofen was administered alone or in combination with RA or imatinib to pregnant rats. Pups were assessed for PA blood flow by ultrasound and pulmonary function/morphology following delivery, intubation, and short-term ventilation. RESULTS: Neither RA nor imatinib had a negative effect on lung and body growth. RA accelerated lung maturation indicated by increased alveoli number and thinner interalveolar septa and was associated with decreased PA resistance and improved oxygenation. With the exception of a decreased PA pulsatility index, no significant changes in morphology and pulmonary function were noted following imatinib. CONCLUSION: Prenatal treatment with RA but not imatinib was associated with improved pulmonary morphology and function, and decreased pulmonary vascular resistance. This study highlights the potential of prenatal pharmacologic therapies, such as RA, for management of CDH.

The Met Needs for Pediatric Surgical Conditions in Sierra Leone: Estimating the Gap.

BACKGROUND: In low- and middle-income countries, there is a gap between the need for surgery and its equitable provision, and a lack of proxy indicators to estimate this gap. Sierra Leone is a West African country with close to three million children. It is unknown to what extent the surgical needs of these children are met. AIM: To describe a nationwide provision of pediatric surgical procedures and to assess pediatric hernia repair as a proxy indicator for the shortage of surgical care in the pediatric population in Sierra Leone. METHODS: We analyzed results from a nationwide facility survey in Sierra Leone that collected data on surgical procedures from operation and anesthesia logbooks in all facilities performing surgery. We included data on all patients under the age of 16 years undergoing surgery. Primary outcomes were rate and volume of surgical procedures. We calculated the expected number of inguinal hernia in children and estimated the unmet need for hernia repair. RESULTS: In 2012, a total of 2381 pediatric surgical procedures were performed in Sierra Leone. The rate of pediatric surgical procedures was 84 per 100,000 children 0-15 years of age. The most common pediatric surgical procedure was hernia repair (18%), corresponding to a rate of 16 per 100,000 children 0-15 years of age. The estimated unmet need for inguinal hernia repair was 88%. CONCLUSIONS: The rate of pediatric surgery in Sierra Leone was very low, and inguinal hernia was the single most common procedure noted among children in Sierra Leone.

Surgical excision is the treatment of choice for cervical lymphatic malformations with mediastinal expansion.

BACKGROUND: Lymphatic malformations (LMs) in the mediastinum are uncommon. However, cervical LMs may expand into the mediastinum. The aim of this study was to review our experience with the management of patients with LMs involving the mediastinum and to propose a treatment algorithm to guide the management of these rare malformations. MATERIAL AND METHODS: This was a descriptive retrospective chart review of all patients with LMs involving the mediastinum treated at our Institution between 2009 and 2015. We collected demographic data, data on investigations, management, and complications of the treatment, as well as outcomes at follow-up. Complications were defined and described according to the Clavien-Dindo classification. The clinical outcome was assessed using a clinical assessment scale. RESULTS: The cohort consisted of seven patients. Airway compromise at the time of diagnosis was seen in 4 patients. Three patients had anatomical compression of the trachea and two patients had sub-total compression of the right lung. All three patients with tracheoscopy-verified compression of the trachea had compromise of the distal trachea, and a tracheostomy would not have been protective. All patients received sclerotherapy. The median time with mechanical ventilation at the neonatal intensive care unit after each sclerotherapy was eleven days (range 8-31). Each patient received sclerotherapy in median three times (range 1-9). Five of the patients (71%) were operated with excision of the LM in the mediastinum. Two of the patients were operated primarily and three patients were operated after major complications to sclerotherapy. The patients treated with sclerotherapy and the operated patients had comparable amount of mild complications, Clavien-Dindo grade I-II complications. Severe complications, Clavien-Dindo grade III-IV, were seen five times more commonly after sclerotherapy than after surgery. The clinical outcome was excellent for the operated patients and fair to good for the patients receiving only sclerotherapy. CONCLUSION: Patients with cervical LM involving the mediastinum represent a high-risk group with respect to the severity of complications following sclerotherapy. The swelling is unpredictable and requires extended observation at an intensive care unit with ventilation support. Tracheostomy does not prevent tracheal compression in mediastinal LM, as the malformation may compress trachea distal to the stoma. Surgical resection of the LM in the mediastinum is recommended, with the possibility of intra-operative sclerotherapy as an adjunctive. LEVEL OF EVIDENCE: IV.

Right versus left congenital diaphragmatic hernia - What's the difference?

BACKGROUND: Right-sided congenital diaphragmatic hernias (CDH) and bigger defect sizes have been associated with poorer outcomes. AIM: The aim of this study was to evaluate right- and left-sided CDH in terms of size, survival, associated anomalies, and morbidity. MATERIAL AND METHODS: We used information from a multicenter, multinational database including patients with CDH born between 2007 and 2015. All infants with data on defect side were included for this analysis. We compared differences in outcomes between right- and left-sided CDH. Further analysis on the association between side, size of the defect, and outcome was performed. RESULTS: A total of 3754 cases of CDH were entered in the registry between January 2007 and September 2015, with an overall survival of 71%. Of those, 598 (16%) were right-sided and 3156 left-sided, with a survival rate of 67% and 72%, respectively. Right-sided CDH had a larger proportion of C and D defects (p<0.001 and 0.04, respectively). Survival rates for the same size defect were similar, independent of the side of the defect. Multivariable logistic regression analysis with survival as dependent variable identified a significant correlation with defect size, but not side. CONCLUSIONS: The higher proportion of large defects (C & D) in right-sided CDH, not the side itself, accounts for the reported poorer survival in right-sided CDH. LEVEL OF EVIDENCE: Level I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge).