Extracorporeal Membrane Oxygenation for Congenital Diaphragmatic Hernia: A Single Center Experience in Turkey

Objective: Extracorporeal membrane oxygenation is a temporary life-support modality offered for stabilizing neonates with congenital diaphragmatic hernia who are in a critical condition and unresponsive to optimal medical therapy. The aim of this study was to examine our institutional outcomes of early congenital diaphragmatic hernia repair on extracorporeal membrane oxygenation. Materials and Methods: A total of 17 extracorporeal membrane oxygenation-supported patients with congenital diaphragmatic hernia were evaluated and the demographic, diagnostic, laboratory, clinical data, complications and outcome of the patients were reported. Results: The study consisted of 7 females and 10 males. Mean birth weight was 3107 g (range, 2360–3840 g). Median age of extracorporeal membrane oxygenation initiation was 2.1 days. In total four patients received venovenous extracorporeal membrane oxygenation. Other patients received venoarterial extracorporeal membrane oxygenation via aortic and right atrial cannulation. The mean duration of extracorporeal membrane oxygenation was 25 days (range: 1-140 days). Six patients (35.2%) could be weaned from extracorporeal membrane oxygenation. The most common extracorporeal membrane oxygenation related complications were hemorrhage, disseminated intravascular coagulation and limb ischemia (64.7%, 41.1% and 29.4%, respectively). Diaphragmatic defect was repaired via a subcostal incision in 12 of 13 patients. In 88.2% of patients diaphragmatic repair was performed with polytetrafluoroethylene patch. In 77% of patients early congenital diaphragmatic hernia repair was performed concomitantly with extracorporeal membrane oxygenation insertion. The survival rate was 17.6%. Conclusion: Early repair of congenital diaphragmatic hernia in neonates on extracorporeal membrane oxygenation can be associated with high rates of morbidity and mortality. However, extracorporeal membrane oxygenation may be considered a life-saving measure for patients with congenital diaphragmatic hernia who would have otherwise not been salvageable.

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[1] Langham MR Jr, Kays DW, Ledbetter DJ, et al. Congenital diaphragmatic hernia. Epidemiology and outcome. Clin Perinatol 1996; 23: 671-88.

[2] Sinha CK, Islam S, Patel S, et al. Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era. J Pediatr Surg 2009; 44: 312-6.

[3] Partridge EA, Peranteau WH, Rintoul NE, et al. Timing of repair of congenital diaphragmatic hernia in patients supported by extracorporeal membrane oxygenation (ECMO). J Pediatr Surg 2015; 50: 260-2.

[4] Khan AM, Lally KP. The role of extracorporeal membrane oxygenation in the management of infants with congenital diaphragmatic hernia. Semin Perinatol 2005; 29: 118-22.

[5] McHoney M, Hammond P. Role of ECMO in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed 2018; 103: F178-F181.

[6] 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: 1691-701.

[7] Hoffman SB, Massaro AN, Gingalewski C, et al. Predictors of survival in congenital diaphragmatic hernia patients requiring extracorporeal membrane oxygenation: CNMC 15-year experience. J Perinatol 2010; 30: 546-52.

[8] Fallon SC, Cass DL, Olutoye OO, et al. Repair of congenital diaphragmatic hernias on Extracorporeal Membrane Oxygenation (ECMO): does early repair improve patient survival? J Pediatr Surg 2013; 48: 1172-6.

[9] Inamura N, Usui N, Okuyama H, et al. Extracorporeal membrane oxygenation for congenital diaphragmatic hernia in Japan. Pediatr Int 2015; 57: 682-6.

[10] Mugford M, Elbourne D, Field D. Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database Syst Rev 2008; 3: CD001340.

[11] Hung WT, Huang SC, Mazloum DE, et al. Extracorporeal membrane oxygenation for neonatal congenital diaphragmatic hernia: The initial single-center experience in Taiwan. J Formos Med Assoc 2017; 116: 333-9.

[12] Okulu E, Atasay FB, Tunç G, et al. Extracorporeal membrane oxygenation support in neonates: a single center experience in Turkey. Turk J Med Sci 2018; 48: 223-30.

[13] Rothenbach P, Lange P, Powell D. The use of extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia. Semin Perinatol 2005; 29: 40-4.

[14] Stolar CJ, Dillon PW, Stalcup SA. Extracorporeal membrane oxygenation and congenital diaphragmatic hernia: modification of the pulmonary vasoactive profile. J Pediatr Surg 1985; 20: 681-3.

[15] Neonatal Inhaled Nitric Oxide Study Group. Inhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failure. N Engl J Med 1997; 336: 597-604.

[16] Hintz SR, Suttner DM, Sheehan AM, et al. Decreased use of neonatal extracorporeal membrane oxygenation (ECMO): how new treatment modalities have affected ECMO utilization. Pediatrics 2000; 106: 1339-43.

[17] Paden ML, Conrad SA, Rycus PT, et al; ELSO Registry. Extracorporeal Life Support Organization Registry Report 2012. ASAIO J 2013; 59: 202-10.

[18] Logan JW, Rice HE, Goldberg RN, et al. Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol 2007; 27: 535-49.

[19] Bartlett RH. Esperanza. Presidential address. Trans Am Soc Artif Intern Organs 1985; 31: 723-6.

[20] Seetharamaiah R, Younger JG, Bartlett RH, et al; Congenital Diaphragmatic Hernia Study Group. Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 2009; 44: 1315-21.

[21] Vaja R, Bakr A, Sharkey A, et al. The use of extracorporeal membrane oxygenation in neonates with severe congenital diaphragmatic hernia: a 26-year experience from a tertiary centre. Eur J Cardiothorac Surg 2017; 52: 552-7.

[22] Davis PJ, Firmin RK, Manktelow B, et al. Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience. J Pediatr 2004; 144: 309-15.

[23] Elbourne D, Field D, Mugford M. Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database Syst Rev 2002; 1: CD001340.

[24] Morini F, Goldman A, Pierro A. Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence. Eur J Pediatr Surg 2006; 16: 385-91.

[25] Garcia A, Stolar CJ. Congenital diaphragmatic hernia and protective ventilation strategies in pediatric surgery. Surg Clin North Am 2012; 92: 659-68, ix.