Survival and Neurodevelopmental Outcomes in Congenital Diaphragmatic Hernia Patients with Single versus Repeat Extracorporeal Membrane Oxygenation Runs

Abstract

Objective We describe the survival and neurodevelopmental outcomes of congenital diaphragmatic hernia (CDH) patients who received single and repeat extracorporeal membrane oxygenation (ECMO).

Study Design This is a retrospective single-center study comparing neurodevelopmental outcomes in CDH patients who were managed without ECMO, who received one ECMO run, and those who received two ECMO runs. Neurodevelopmental testing was performed utilizing the Bayley Scales of Infant Development-III.

Results There were 68 neonates identified with CDH from January 2011 to June 2019: 30 did not receive ECMO, 29 received single ECMO run, and 9 received two ECMO runs. Survival of ECMO patients was 50%, with 48% of single run and 57% of repeat run patients surviving to discharge. Second-run ECMO patients had increased median ventilator days (60 vs. 33, p = 0.04) and increased median length of hospital stay (159 vs. 89, p = 0.01). Neurodevelopmental testing via Bayley Scales of Infant Development-III was performed on 74% of survivors at the mean age of 24 months. CDH neonates who underwent ECMO (single or repeat runs) were more likely to have lower cognitive, language, and motor composite scores as compared with CDH neonates who had not required ECMO. Motor composite scores were significantly lower in repeat ECMO run neonates as compared with single ECMO run (72 + 6 vs. 85 + 4, p = 0.0003), but there were no further deficits noted in language or cognitive domains.

Conclusion Survival after a second ECMO run in CDH is possible, although with increased ventilator days and increased length of hospitalization. We also find further deficits in motor outcomes in the second-run ECMO group compared with single-run ECMO. Our findings do not preclude a second ECMO run, but rather inform our counseling to families and reinforce the need for close neurodevelopmental follow-up for these patients.

Key Points

• A repeat ECMO run is associated with further neurodevelopmental deficits in the motor domain.

• Survival is possible after repeat ECMO and is associated with increased length of hospital stay.

• Neurodevelopmental follow-up is critical for all CDH ECMO patients.

Keywords

congenital diaphragmatic hernia - ECMO - repeat ECMO support - neurodevelopment - neurodevelopmental outcome - mortality

^* These authors contributed equally to this work.

Publication History

Received: 07 March 2022

Accepted: 10 June 2022

Accepted Manuscript online:
16 June 2022

Article published online:
25 August 2022

• References

• 1 Burgos CM, Frenckner B. Addressing the hidden mortality in CDH: a population-based study. J Pediatr Surg 2017; 52 (04) 522-525
• 2 Putnam LR, Harting MT, Tsao K. et al; Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia defect size and infant morbidity at discharge. Pediatrics 2016; 138 (05) e20162043
• 3 Zalla JM, Stoddard GJ, Yoder BA. Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15 year experience in a single institution. J Pediatr Surg 2015; 50 (04) 524-527
• 4 Harting MT, Lally KP. The Congenital Diaphragmatic Hernia Study Group registry update. Semin Fetal Neonatal Med 2014; 19 (06) 370-375
• 5 Guner YS, Khemani RG, Qureshi FG. et al. Outcome analysis of neonates with congenital diaphragmatic hernia treated with venovenous vs venoarterial extracorporeal membrane oxygenation. J Pediatr Surg 2009; 44 (09) 1691-1701
• 6 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 (06) 893-897
• 7 Russo FM, Eastwood MP, Keijzer R. et al. Lung size and liver herniation predict need for extracorporeal membrane oxygenation but not pulmonary hypertension in isolated congenital diaphragmatic hernia: systematic review and meta-analysis. Ultrasound Obstet Gynecol 2017; 49 (06) 704-713
• 8 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
• 9 Mesas Burgos C, Öst E, Frenckner B. Re-ECMO for congenital diaphragmatic hernia: Is it worth the effort?. J Pediatr Surg 2020; 55 (11) 2289-2292
• 10 Church JT, Mon R, Wright T. et al. Neurodevelopmental outcomes in CDH survivors: a single institution's experience. J Pediatr Surg 2018; 53 (06) 1087-1091
• 11 Montalva L, Raffler G, Riccio A, Lauriti G, Zani A. Neurodevelopmental impairment in children with congenital diaphragmatic hernia: not an uncommon complication for survivors. J Pediatr Surg 2020; 55 (04) 625-634
• 12 Bouman NH, Koot HM, Tibboel D, Hazebroek FW. Children with congenital diaphragmatic hernia are at risk for lower levels of cognitive functioning and increased emotional and behavioral problems. Eur J Pediatr Surg 2000; 10 (01) 3-7
• 13 Meehan JJ, Haney BM, Snyder CL, Sharp RJ, Acosta JM, Holcomb III GW. Outcome after recannulation and a second course of extracorporeal membrane oxygenation. J Pediatr Surg 2002; 37 (06) 845-850
• 14 Danzer E, Gerdes M, Bernbaum J. et al. Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program. J Pediatr Surg 2010; 45 (09) 1759-1766
• 15 Danzer E, Hedrick HL. Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia. Early Hum Dev 2011; 87 (09) 625-632
• 16 Danzer E, Gerdes M, D'Agostino JA. et al. Preschool neurological assessment in congenital diaphragmatic hernia survivors: outcome and perinatal factors associated with neurodevelopmental impairment. Early Hum Dev 2013; 89 (06) 393-400
• 17 Friedman S, Chen C, Chapman JS. et al. Neurodevelopmental outcomes of congenital diaphragmatic hernia survivors followed in a multidisciplinary clinic at ages 1 and 3. J Pediatr Surg 2008; 43 (06) 1035-1043
• 18 Danzer E, Hoffman C, D'Agostino JA. et al. Short-term neurodevelopmental outcome in congenital diaphragmatic hernia: the impact of extracorporeal membrane oxygenation and timing of repair. Pediatr Crit Care Med 2018; 19 (01) 64-74
• 19 Jaillard SM, Pierrat V, Dubois A. et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study. Ann Thorac Surg 2003; 75 (01) 250-256
• 20 Wynn J, Aspelund G, Zygmunt A. et al. Developmental outcomes of children with congenital diaphragmatic hernia: a multicenter prospective study. J Pediatr Surg 2013; 48 (10) 1995-2004
• 21 Patel N, Massolo AC, Kipfmueller F. Congenital diaphragmatic hernia-associated cardiac dysfunction. Semin Perinatol 2020; 44 (01) 151168
• 22 Wild KT, Rintoul N, Kattan J, Gray B. Extracorporeal Life Support Organization (ELSO): guidelines for neonatal respiratory failure. ASAIO J 2020; 66 (05) 463-470