International Survey on the Management of Congenital Diaphragmatic Hernia

Abstract Aim This study aims to define patterns in the management of congenital diaphragmatic hernia (CDH). Methods A total of 180 delegates (77% senior surgeons) from 44 (26 European) countries completed a survey at the 2014 European Pediatric Surgeons' Association meeting. Results Overall, 34% of the surgeons work in centers that treat < 5 cases of CDH/y, 38% work in centers that treat 5 to 10 cases/y, and 28% work in centers that treat > 10 cases/y. Overall, 62% of the surgeons work in extra corporeal membrane oxygenation (ECMO) centers and 23% in fetal surgery centers. Prenatal work up and delivery: 47% surgeons request prenatal magnetic resonance imaging, 53% offer karyotyping, 22% perform a fetal intervention, 74% monitor head-to-lung ratio, and 55% administer maternal steroids. Delivery is via cesarean section for 47% surgeons, at 36 to 38 weeks for 71% surgeons, and in a tertiary care center for 94% of the surgeons. Postnatal Management A total of 76% surgeons report elective intubation, 65% start antibiotics preoperatively, and 45% administer surfactant. In case of refractory hypoxia, 66% surgeons consider ECMO with a variable course. Parenteral feeding is started preoperatively by 56% of the surgeons. Only 13% of the surgeons request contrast studies preoperatively to rule out malrotation.

[1]  M. Hayakawa,et al.  Growth Assessment and the Risk of Growth Retardation in Congenital Diaphragmatic Hernia: A Long-Term Follow-Up Study from the Japanese Congenital Diaphragmatic Hernia Study Group , 2015, European Journal of Pediatric Surgery.

[2]  Dionne A. Graham,et al.  Pulmonary support on day of life 30 is a strong predictor of increased 1 and 5-year morbidity in survivors of congenital diaphragmatic hernia. , 2015, Journal of pediatric surgery.

[3]  M. Davenport,et al.  Best Oxygenation Index on Day 1: A Reliable Marker for Outcome and Survival in Infants with Congenital Diaphragmatic Hernia , 2015, European Journal of Pediatric Surgery.

[4]  A. Pierro,et al.  Advances in the surgical approach to congenital diaphragmatic hernia. , 2014, Seminars in fetal & neonatal medicine.

[5]  Stephen S. Kim,et al.  Neurodevelopmental outcome in congenital diaphragmatic hernia: Evaluation, predictors and outcome. , 2014, World journal of clinical pediatrics.

[6]  J. Deprest,et al.  Antireflux surgery after congenital diaphragmatic hernia repair: a plea for a tailored approach. , 2013, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[7]  M. Bajpai,et al.  Presence of hernia sac in prediction of postoperative outcome in congenital diaphragmatic hernia , 2013, Indian Pediatrics.

[8]  E. Danzer,et al.  Use of ultrasound and MRI for evaluation of lung volumes in fetuses with isolated left congenital diaphragmatic hernia. , 2013, Seminars in pediatric surgery.

[9]  Sara C. Fallon,et al.  The presence of a hernia sac in congenital diaphragmatic hernia is associated with better fetal lung growth and outcomes. , 2012, Journal of pediatric surgery.

[10]  P. Chiu,et al.  Morbidity and Long-Term Follow-Up in CDH Patients , 2012, European journal of pediatric surgery.

[11]  S. Eaton,et al.  More patches or more lung in congenital diaphragmatic hernia? , 2012, Journal of pediatric surgery.

[12]  A. Synnes,et al.  Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice. , 2012, Journal of pediatric surgery.

[13]  C. Delacourt,et al.  Long term respiratory outcomes of congenital diaphragmatic hernia, esophageal atresia, and cardiovascular anomalies. , 2012, Seminars in fetal & neonatal medicine.

[14]  A. Flake,et al.  Patch repair for congenital diaphragmatic hernia: is it really a problem? , 2012, Journal of pediatric surgery.

[15]  D. Tibboel,et al.  Congenital diaphragmatic hernia: to repair on or off extracorporeal membrane oxygenation? , 2012, Journal of pediatric surgery.

[16]  L. Wessel,et al.  Preventive antireflux surgery in neonates with congenital diaphragmatic hernia: a single-blinded prospective study. , 2011, Journal of pediatric surgery.

[17]  E. Skarsgard,et al.  Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network. , 2011, Journal of pediatric surgery.

[18]  D. Tibboel,et al.  Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update , 2010, Neonatology.

[19]  S. Eaton,et al.  Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus , 2010, Neonatology.

[20]  D. Mullassery,et al.  Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta‐analysis , 2010, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[21]  M. Mugford,et al.  Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. , 2010, The Cochrane database of systematic reviews.

[22]  K. Nicolaides,et al.  Antenatal prediction of lung volume and in-utero treatment by fetal endoscopic tracheal occlusion in severe isolated congenital diaphragmatic hernia. , 2009, Seminars in fetal & neonatal medicine.

[23]  S. Dymarkowski,et al.  Quantification of intrathoracic liver herniation by magnetic resonance imaging and prediction of postnatal survival in fetuses with congenital diaphragmatic hernia , 2008, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[24]  F. Bargy,et al.  Preventive Antireflux Surgery in Patients with Congenital Diaphragmatic Hernia , 2008, World Journal of Surgery.

[25]  K. Lally,et al.  The Congenital Diaphragmatic Hernia Study Group: a voluntary international registry. , 2008, Seminars in pediatric surgery.

[26]  M. Pakarinen,et al.  The cumulative incidence of significant gastroesophageal reflux in patients with congenital diaphragmatic hernia-a systematic clinical, pH-metric, and endoscopic follow-up study. , 2008, Journal of pediatric surgery.

[27]  T. Schaible,et al.  Prediction of mortality and need for neonatal extracorporeal membrane oxygenation in fetuses with congenital diaphragmatic hernia: logistic regression analysis based on MRI fetal lung volume measurements. , 2007, AJR. American journal of roentgenology.

[28]  C. M. Cotten,et al.  Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies , 2007, Journal of Perinatology.

[29]  S. Hintz,et al.  Prenatal diagnosis of congenital diaphragmatic hernia: how should the babies be delivered? , 2007, Journal of Pediatric Surgery.

[30]  K. Nicolaides,et al.  Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia , 2007, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[31]  M. Harting,et al.  Surgical management of neonates with congenital diaphragmatic hernia. , 2007, Seminars in pediatric surgery.

[32]  P. Bagolan,et al.  Long-term follow up of infants with congenital diaphragmatic hernia. , 2007, Seminars in pediatric surgery.

[33]  O. Catalano,et al.  Pleural effusion requiring drainage in congenital diaphragmatic hernia: incidence, aetiology and treatment , 2006, Pediatric Surgery International.

[34]  J. Laudy,et al.  Prenatal prediction of survival in isolated left‐sided diaphragmatic hernia , 2005, Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology.

[35]  K. Meurs Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia , 2004 .

[36]  D. Tibboel,et al.  Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia. , 2004, Journal of pediatric surgery.

[37]  T. Hurme,et al.  Congenital diaphragmatic hernia: a survey of practice in Scandinavia , 2004, Pediatric Surgery International.

[38]  K. Azarow,et al.  Congenital diaphragmatic hernia--a tale of two cities: the Toronto experience. , 1997, Journal of pediatric surgery.

[39]  J. Vacanti,et al.  Congenital diaphragmatic hernia--a tale of two cities: the Boston experience. , 1997, Journal of pediatric surgery.

[40]  J. Tovar,et al.  Intestinal rotation in experimental congenital diaphragmatic hernia. , 1995, Journal of pediatric surgery.

[41]  C. Stolar,et al.  Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. , 1995, Journal of pediatric surgery.

[42]  J. Pichette,et al.  Estimation of lungs' hypoplasia on postoperative chest x-rays in congenital diaphragmatic hernia. , 1993, Journal of pediatric surgery.

[43]  A. Baraka,et al.  Congenital diaphragmatic hernia and eventration. , 1987, Middle East journal of anaesthesiology.

[44]  A. Bianchi,et al.  The reverse latissimus dorsi flap for congenital diaphragmatic hernia repair. , 1983, Journal of pediatric surgery.

[45]  A. Nasr,et al.  Outcomes after muscle flap vs prosthetic patch repair for large congenital diaphragmatic hernias. , 2010, Journal of pediatric surgery.

[46]  C. Barnewolt,et al.  Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia. , 2007, Journal of pediatric surgery.

[47]  E. Skarsgard Networks in Canadian paediatric surgery: Time to get connected. , 2006, Paediatrics & child health.

[48]  P. Dillon,et al.  Congenital Diaphragmatic Hernia and Eventration , 2006 .

[49]  R. Filly,et al.  Sonographic predictors of survival in fetal diaphragmatic hernia. , 1996, Journal of pediatric surgery.

[50]  M. Macken,et al.  Antenatal diagnosis of congenital diaphragmatic hernia. , 1993, Canadian Association of Radiologists journal = Journal l'Association canadienne des radiologistes.

[51]  D. Vane,et al.  Anomalies of intestinal rotation in childhood: analysis of 447 cases. , 1990, Surgery.