Subscribe to RSS
Does the Presence of a Hernia Sac Improve Survival in Newborns with Congenital Diaphragmatic Hernia? A United Kingdom Single-Center Experience
Introduction A developing body of literature suggests that the presence of a hernia sac in fetuses with congenital diaphragmatic hernia (CDH) may indicate improved prognosis. By examining a large cohort of CDH newborns admitted to a single United Kingdom specialist center, we aimed to establish if presence of hernia sac is a robust predictor of improved survival.
Materials and Methods All CDH patients admitted to a single center were recruited. Postneonatal presentations and Morgagni hernias were excluded. Demographics, defect type, laterality, survival, and hernia recurrence were recorded.
Results In this study, 192 CDH newborns were managed from 1997 to 2017; 39 were excluded (10 Morgagni and 29 postneonatal); 22 (14%) neonates had a hernia sac. Survival in patients with a hernia sac was 21/22 (95%) versus 107/124 (86%) in cases without hernia sac (p = 0.2). There was no difference in hernia sac proportion by gender (male:female 15 vs. 13.2%, p = 0.8).
Conclusion In contrast to studies showing a survival advantage, albeit with smaller patient numbers, we report a statistical nonsignificant benefit of hernia sac. Better survival outcomes at this specialist center with CDH patients without a hernia sac than reported in other published studies are likely responsible for the lack of statistical significance observed, despite a larger cohort. National and international CDH registries yielding “big data” may provide further answers on the utility of a CDH hernia sac as a new prognostic scoring tool.
Received: 15 February 2020
Accepted: 08 May 2020
11 June 2020 (online)
© 2020. Thieme. All rights reserved.
Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany
- 1 Ramakrishnan R, Salemi JL, Stuart AL. et al. Trends, correlates, and survival of infants with congenital diaphragmatic hernia and its subtypes. Birth Defects Res 2018; 110 (14) 1107-1117
- 2 Zani-Ruttenstock E, Zani A, Eaton S, Fecteau A. First population-based report of infants with congenital diaphragmatic hernia: 30-Day outcomes from the American College of Surgeons National Quality Improvement Program. Eur J Pediatr Surg 2019; 29 (01) 62-67
- 3 Wright JC, Budd JL, Field DJ, Draper ES. Epidemiology and outcome of congenital diaphragmatic hernia: a 9-year experience. Paediatr Perinat Epidemiol 2011; 25 (02) 144-149
- 4 Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 2003; 112 (3 Pt 1): 532-535
- 5 Burgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP. Congenital Diaphragmatic Hernia Study Group. Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - side, stage, and outcome. J Pediatr Surg 2019; 54 (04) 651-655
- 6 Long A-M, Bunch KJ, Knight M, Kurinczuk JJ, Losty PD. BAPS-CASS. Early population-based outcomes of infants born with congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed 2018; 103 (06) F517-F522
- 7 Deprest J, Gratacos E, Nicolaides KH. FETO Task Group. Fetoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia: evolution of a technique and preliminary results. Ultrasound Obstet Gynecol 2004; 24 (02) 121-126
- 8 Harrison MR, Keller RL, Hawgood SB. et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med 2003; 349 (20) 1916-1924
- 9 Ba'ath ME, Jesudason EC, Losty PD. How useful is the lung-to-head ratio in predicting outcome in the fetus with congenital diaphragmatic hernia? A systematic review and meta-analysis. Ultrasound Obstet Gynecol 2007; 30 (06) 897-906
- 10 Mullassery D, Ba'ath ME, Jesudason EC, Losty PD. Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis. Ultrasound Obstet Gynecol 2010; 35 (05) 609-614
- 11 Cohen MS, Rychik J, Bush DM. et al. Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia. J Pediatr 2002; 141 (01) 25-30
- 12 Spaggiari E, Stirnemann J, Bernard J-P, De Saint Blanquat L, Beaudoin S, Ville Y. Prognostic value of a hernia sac in congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 2013; 41 (03) 286-290
- 13 Panda SS, Bajpai M, Srinivas M. Presence of hernia sac in prediction of postoperative outcome in congenital diaphragmatic hernia. Indian Pediatr 2013; 50 (11) 1041-1043
- 14 Zamora IJ, Cass DL, Lee TC. et al. The presence of a hernia sac in congenital diaphragmatic hernia is associated with better fetal lung growth and outcomes. J Pediatr Surg 2013; 48 (06) 1165-1171
- 15 Zamora IJ, Mehollin-Ray AR, Sheikh F. et al. Predictive value of MRI findings for the identification of a hernia sac in fetuses with congenital diaphragmatic hernia. Am J Roentgenol 2015; 205 (05) 1121-1125
- 16 Grizelj R, Bojanić K, Vuković J. et al. Hernia sac presence portends better survivability of isolated congenital diaphragmatic hernia with “liver-up”. Am J Perinatol 2017; 34 (05) 515-519
- 17 Bouchghoul H, Marty O, Fouquet V. et al. Congenital diaphragmatic hernia has a better prognosis when associated with a hernia sac. Prenat Diagn 2018; 38 (09) 638-644
- 18 Oliver ER, DeBari SE, Adams SE. et al. Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes. Pediatr Radiol 2019; 49 (05) 593-599
- 19 Jawaid WB, Qasem E, Jones MO, Shaw NJ, Losty PD. Outcomes following prosthetic patch repair in newborns with congenital diaphragmatic hernia. Br J Surg 2013; 100 (13) 1833-1837
- 20 Levesque M, Derraugh G, Schantz D. et al. The presence of a hernia sac in isolated congenital diaphragmatic hernia is associated with less disease severity: a retrospective cohort study. J Pediatr Surg 2019; 54 (05) 899-902
- 21 Skarsgard ED, Harrison MR. Congenital diaphragmatic hernia: the surgeon's perspective. Pediatr Rev 1999; 20 (10) e71-e78