Hernia Sac Presence Portends Better Survivability of Isolated Congenital Diaphragmatic Hernia with “Liver-Up”

Abstract

Objective The objective of this study was to investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH) with intrathoracic liver herniation (“liver-up”).

Study Design A retrospective study from the single tertiary center. Isolated “liver-up” CDH neonates referred to our institution between 2000 and 2015 were reviewed for the presence or absence of a hernia sac. Association between the presence of a hernia sac and survival was assessed.

Results Over the study period, there were 29 isolated CDH patients with “liver-up” who were treated, 7 (24%) had a sac, and 22 (76%) did not. Demographics were similar between groups. However, disease acuity, assessed from lower Apgar scores (p = 0.044), lower probability of survival (p = 0.037), and lower admission oxygenation (p = 0.027), was higher in neonates without a sac. Hospital survival was significantly higher for those with sac compared with those without (7/7, 100 vs. 7/22, 32%, p = 0.002).

Conclusion The presence of a hernia sac may be associated with better survival for isolated “liver-up” CDH. As the presence of sac can be prenatally detected, it may be a useful marker to aid perinatal decision making.

Keywords

congenital diaphragmatic hernia - hernia sac - liver-up - mortality - outcome - risk assessment - survival

^* These authors contributed equally to this article.

• References

• 1 Bojanić K, Pritišanac E, Luetić T , et al. Survival of outborns with congenital diaphragmatic hernia: the role of protective ventilation, early presentation and transport distance: a retrospective cohort study. BMC Pediatr 2015; 15: 155
• 2 Grizelj R, Bojanić K, Vuković J , et al; Croatian Diaphragmatic Hernia Study Group. Epidemiology and outcomes of congenital diaphragmatic hernia in Croatia: a population-based study. Paediatr Perinat Epidemiol 2016; 30 (4) 336-345
• 3 Albanese CT, Lopoo J, Goldstein RB , et al. Fetal liver position and perinatal outcome for congenital diaphragmatic hernia. Prenat Diagn 1998; 18 (11) 1138-1142
• 4 Hedrick HL, Danzer E, Merchant A , et al. Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol 2007; 197 (4) 422.e1-422.e4
• 5 Mayer S, Klaritsch P, Petersen S , et al. The correlation between lung volume and liver herniation measurements by fetal MRI in isolated congenital diaphragmatic hernia: a systematic review and meta-analysis of observational studies. Prenat Diagn 2011; 31 (11) 1086-1096
• 6 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 (5) 609-614
• 7 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 (6) 1165-1171
• 8 Kitano Y, Nakagawa S, Kuroda T , et al. Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy. J Pediatr Surg 2005; 40 (12) 1827-1832
• 9 Bebbington M, Victoria T, Danzer E , et al. Comparison of ultrasound and magnetic resonance imaging parameters in predicting survival in isolated left-sided congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 2014; 43 (6) 670-674
• 10 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 (5) 1121-1125
• 11 Spaggiari E, Stirnemann J, Bernard JP, De Saint Blanquat L, Beaudoin S, Ville Y. Prognostic value of a hernia sac in congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 2013; 41 (3) 286-290
• 12 Congenital Diaphragmatic Hernia Study Group. Estimating disease severity of congenital diaphragmatic hernia in the first 5 minutes of life. J Pediatr Surg 2001; 36 (1) 141-145
• 13 Sinha CK, Islam S, Patel S, Nicolaides K, Greenough A, Davenport M. Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era. J Pediatr Surg 2009; 44 (2) 312-316
• 14 Salas AA, Bhat R, Dabrowska K , et al. The value of Pa(CO2) in relation to outcome in congenital diaphragmatic hernia. Am J Perinatol 2014; 31 (11) 939-946
• 15 Kays DW, Islam S, Perkins JM, Larson SD, Taylor JA, Talbert JL. Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: whom should we treat?. J Pediatr Surg 2015; 50 (6) 893-897
• 16 Lazar DA, Ruano R, Cass DL , et al. Defining “liver-up”: does the volume of liver herniation predict outcome for fetuses with isolated left-sided congenital diaphragmatic hernia?. J Pediatr Surg 2012; 47 (6) 1058-1062
• 17 Walsh DS, Hubbard AM, Olutoye OO , et al. Assessment of fetal lung volumes and liver herniation with magnetic resonance imaging in congenital diaphragmatic hernia. Am J Obstet Gynecol 2000; 183 (5) 1067-1069
• 18 Zavorsky GS, Cao J, Mayo NE, Gabbay R, Murias JM. Arterial versus capillary blood gases: a meta-analysis. Respir Physiol Neurobiol 2007; 155 (3) 268-279