Abstract
Background
Survival for severe (observed to expected lung-head ratio (O:E LHR) < 25%) congenital diaphragmatic hernia (CDH) remains a challenge (15–25%). Management strategies have focused on fetal endoscopic tracheal occlusion (FETO) and/or extracorporeal membrane oxygenation therapy (ECMO) utilization.
Objective(s)
Describe single center outcomes for infants with severe CDH.
Study design
Observational study of 13 severe CDH infants managed with ECMO, a protocolized DR algorithm, and early repair on ECMO with an innovative perioperative anticoagulation strategy.
Results
13/140 (9.3%) infants met criteria and were managed with ECMO. 77% survived ECMO and 69% survived to discharge. 22% underwent tracheostomy. Median days on mechanical ventilation was 39 days (IQR 22:107.5) and length of stay 135 days (IQR 62.5:211.5). All infants received a gastrostomy tube (GT) and were discharged home on oxygen and pulmonary hypertension (PH) meds.
Conclusion
Survival for infants with severe CDH can be optimized with early aggressive intervention and protocolized algorithms (149).
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Data availability
Data relevant to this manuscript is presented in the main manuscript and if requested will be made available to referees and to readers promptly upon request.
References
Boloker J, Bateman DA, Wung J-T, Stolar CJH. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnia, spontaneous respiration, and elective repair. J Pediatr Surg. 2002;37:357–66.
Doyle NM, Lally KP, Doyle NM, Lally KP. The CDH study group and advances in the clinical care of the patient with congenital diaphragmatic hernia. Semin Perinatol. 2004;28:174–84.
Downard CD, Jaksic T, Garza JJ, Dzakovic A, Nemes L, Jennings RW, et al. Analysis of an improved survival rate for congenital diaphragmatic hernia. J Pediatr Surg. 2003;38:729–32.
Jani J, Nicolaides KH, Keller RL, Benachi A, Peralta CFA, Favre R, et al. Antenatal CDH Registry Group. Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia. Ultrasound Obstet Gynecol. 2007;30:67–71.
Deprest JA, Nicolaides KH, Benachi A, Gratacos E, Ryan G, Persico N, et al. TOTAL Trial for severe hypoplasia investigators. Randomized trial of fetal surgery for severe left diaphragmatic hernia. N. Engl J Med 2021;385:107–18.
Dekoninck P, Gratacos E, Van Mieghem T, Richter J, Lewi P, Ancel AM, et al. Results of fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia and the set-up of the randomized controlled TOTAL trial. Early Hum Dev. 2011;87:619–24.
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? Pediatr Surg. 2015;50:893–7.
Stoffan AP, Wilson JM, Jennings RW, Wilkins-Haug LE, Buchmiller TL. Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia? J Pediatr Surg. 2012;47:1053–7.
Shieh HF, Wilson JM, Sheils CA, Smithers CJ, Kharasch VS, Becker RE, et al. Does the ex-utero intrapartum treatment to extracorporeal membrane oxygenation procedure change morbidity outcomes for high-risk congenital diaphragmatic hernia survivors? J Pediatr Surg. 2017;52:22–5.
Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW, Housley HT, Beech R, et al. Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. Pediatr Surg. 1997;32:1634–6.
Alfaraj MA, Shah PS, Bohn D, Pantazi S, O’Brien K, Chiu PP, et al. Congenital diaphragmatic hernia: lung-to-head ratio and lung volume for prediction of outcome. Am J Obstet Gynecol. 2011;205:43.e1–8.
Phillips RC, Shahi N, Leopold D, Levek C, Shirek G, Hilton S, et al. Thromboelastography-guided management of coagulopathy in neonates with congenital diaphragmatic hernia supported by extracorporeal membrane oxygenation. Pediatr Surg Int. 2020;36:1027–33.
Thiagarajan RR, Barbaro RP, Rycus PT, Mcmullan DM, Conrad SA, Fortenberry JD, et al. ELSO member centers Extracorporeal Life Support Organization Registry International Report 2016. ASAIO J. 2017;63:60–7.
Paden ML, Conrad SA, Rycus PT, Thiagarajan RR. ELSO Registry. Extracorporeal Life Support Organization Registry Report 2012. ASAIO J. 2013;59:202–10.
Davis JS, Ryan ML, Perez EA, Neville HL, Bronson SN, Sola JE, et al. ECMO hospital volume and survival in congenital diaphragmatic hernia repair. J Surg Res. 2012;178:791–6.
Bucher BT, Guth RM, Saito JM, Najaf T, Warner BW. Impact of hospital volume on in-hospital mortality of infants undergoing repair of congenital diaphragmatic hernia. Ann Surg. 2010;252:635–42.
Apfeld JC, Kastenberg ZJ, Gibbons AT, Carmichael SL, Lee HC, Sylvester KG. Treating Center Volume and Congenital Diaphragmatic Hernia Outcomes in California. J Pediatr. 2020;222:146–.e1.
Lewit RA, Jancelewicz T. Sources of regional and center-level variability in survival and cost of care for congenital diaphragmatic hernia (CDH). J Pediatr Surg. 2021;56:130–5.
Kim AG, Norwitz G, Karmakar M, Ladino-Torres M, Berman DR, Kreutzman J, et al. Discordant prenatal ultrasound and fetal MRI in CDH: wherein lies the truth? Pediatr Surg. 2020;55:1879–84.
Barnhart DC, Jacques E, Scaife ER, Yoder BA, Meyers RL, Harman A, et al. Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias. J Pediatr Surg. 2012;47:81–6.
Dewberry L, Hilton S, Gien J, Liechty KW, Marwan AI. Flap repair in congenital diaphragmatic hernia leads to lower rates of recurrence. J Pediatr Surg 2019;54:2487–249.
Jancelewicz T, Langham MR Jr, Brindle ME, Stiles ZE, Lally PA, Dong L, et al. Survival Benefit Associated with the Use of Extracorporeal Life Support for Neonates With Congenital Diaphragmatic Hernia. Ann Surg. 2022;275:e256–63.
Golden J, Jones N, Zagory J, Castle S, Bliss D. Outcomes of congenital diaphragmatic hernia repair on extracorporeal life support. Pediatr Surg Int. 2017;33:125–31.
Robertson JO, Criss CN, Hsieh LB, Matsuko N, Gish JS, Mon RA, et al. Comparison of early versus delayed strategies for repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg. 2018;53:629–34.
Bryner BS, West BT, Hirschl RB, Drongowski RA, Lally KP, Lally P, et al. Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter? J Pediatr Surg. 2009;44:1165–71.
Delaplain PT, Harting MT, Jancelewicz T, Zhang L, Yu PT, Di Nardo M, et al. Potential survival benefit with repair of congenital diaphragmatic hernia (CDH) after extracorporeal membrane oxygenation (ECMO) in select patients: Study by ELSO CDH Interest Group. J Pediatr Surg. 2019;54:1132–7.
Partridge EA, Peranteau WH, Rintoul NE, Herkert LM, Flake AW, Adzick NS, et al. Timing of repair of congenital diaphragmatic hernia in patients supported by extracorporeal membrane oxygenation (ECMO). J Pediatr Surg. 2015;50:260–2.
Fallon SC, Cass DL, Olutoye OO, Zamora IJ, Lazar DA, Larimer EL, 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.
Dao DT, Burgos CM, Harting MT, Lally KP, Lally PA, Nguyen HT, et al. Surgical repair of congenital diaphragmatic hernia after extracorporeal membrane oxygenation cannulation: early repair improves survival. Ann Surg. 2021;274:186–94.
Dassinger MS, Copeland DR, Gossett J, Little DC, Jackson RJ, Smith SD, et al. Early repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg. 2010;45:693–7.
Steen EH, Lee TC, Vogel AM, Fallon SC, Fernandes CJ, Style CC, et al. Congenital diaphragmatic hernia repair in patients on extracorporeal membrane oxygenation: How early can we repair? J Pediatr Surg. 2019;54:50–4.
Ferguson DM, Gupta VS, Lally PA, Luco M, Tsao K, Lally KP, et al. Congenital Diaphragmatic Hernia Study Group. Early postnatal pulmonary hypertension severity predicts inpatient outcomes in congenital diaphragmatic hernia. Neonatology. 2021;118:147–54.
Ameis D, Khoshgoo N, Keijzer R. Abnormal lung development in congenital diaphragmatic hernia. Semin Pediatr Surg. 2017;26:123–8.
Harting MT, Lally KP. The congenital diaphragmatic hernia study group registry update. Semin Fetal Neonatal Med. 2014;19:370–5.
Acknowledgements
The patients who trusted the Colorado Fetal Care Center and CDH Team at Children’s Hospital Colorado with their care.
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JG was responsible for conception, acquisition, analysis and interpretation of data for the work. JG wrote the first draft of the manuscript, is agreeable to be accountable for all aspects of the work related to the accuracy or integrity of the work and gave approval for the final version to be published. JPK was responsible for conception and interpretation of data for the work. JK reviewed the initial manuscript draft and provided feedback revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. NJB was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. MVZ was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. HLG was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. KWL was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published.
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Colorado Institutional review board (COMIRB) approval was obtained with a waiver of consent. The study was performed in accordance with the Declaration of Helsinki.
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Gien, J., Kinsella, J.P., Behrendt, N.J. et al. Improved survival for infants with severe congenital diaphragmatic hernia. J Perinatol 42, 1189–1194 (2022). https://doi.org/10.1038/s41372-022-01397-3
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DOI: https://doi.org/10.1038/s41372-022-01397-3
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