Abstract
Congenital diaphragmatic hernia (CDH) in the newborn poses challenges to the multi-disciplinary teams involved in its management. Mortality remains significantly high, despite growing understanding and treatment options. Early intubation of antenatally diagnosed cases is crucial in preventing deterioration and persistent pulmonary hypertension. Early recognition of cases not diagnosed on antenatal scan, with appreciation of differential diagnosis, requires an index of suspicion and imaging. Increasing options and modalities are available, with only modest, if any, survival advantage. Permissive hypercapnea and minimal ventilation have made the most significant impact on survival in modern era. High-frequency oscillatory ventilation (HFOV), inhaled nitric oxide (iNO), treatment of pulmonary hypertension, and ECMO are used in a somewhat stepwise manner for stabilisation. Delayed surgery has become established later in management plan. The impact of individual therapies (e.g. HFOV, iNO, ECMO) on outcome is difficult to ascertain. Little level 1 or 2 evidence exists. Randomised studies and reviews on the role of ECMO have not yet proven any long-term survival benefit. One pilot randomised study of thoracoscopic repair suggests increased acidosis; intraoperative blood gases and CO2 levels should be closely monitored. Monitoring tissue oxygenation should be considered. There is no evidence to suggest the best patch material.
Similar content being viewed by others
References
Abbas PI, Cass DL, Olutoye OO, Zamora IJ, Akinkuotu AC, Sheikh F, Welty SE, Lee TC (2015) Persistent hypercarbia after resuscitation is associated with increased mortality in congenital diaphragmatic hernia patients. J Pediatr Surg 50:739–743
Salas AA, Bhat R, Dabrowska K, Leadford A, Anderson S, Harmon CM, Ambalavanan N, El-Ferzli GT (2014) The value of Pa(CO2) in relation to outcome in congenital diaphragmatic hernia. Am J Perinatol 31:939–946
Ruttenstock E, Wright N, Barrena S, Krickhahn A, Castellani C, Desai AP, Rintala R, Tovar J, Till H, Zani A, Saxena A, Davenport M (2015) Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia. Eur J Pediatr Surg 25:3–8
Park HW, Lee BS, Lim G, Choi YS, Kim EA, Kim KS (2013) A simplified formula using early blood gas analysis can predict survival outcomes and the requirements for extracorporeal membrane oxygenation in congenital diaphragmatic hernia. J Korean Med Sci 28:924–928
Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJ (1995) Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 30:406–409
Wilson JM, Lund DP, Lillehei CW, Vacanti JP (1997) Congenital diaphragmatic hernia—a tale of two cities: the Boston experience. J Pediatr Surg 32:401–405
Boloker J, Bateman DA, Wung JT, Stolar CJ (2002) Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg 37:357–366
Logan JW, Rice HE, Goldberg RN, Cotten CM (2007) Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol 27:535–549
Finer NN, Tierney A, Etches PC, Peliowski A, Ainsworth W (1998) Congenital diaphragmatic hernia: developing a protocolized approach. J Pediatr Surg 33:1331–1337
Kays DW, Langham MR Jr, Ledbetter DJ, Talbert JL (1999) Detrimental effects of standard medical therapy in congenital diaphragmatic hernia. Ann Surg 230:340–348
Muratore CS, Kharasch V, Lund DP, Sheils C, Friedman S, Brown C, Utter S, Jaksic T, Wilson JM (2001) Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic. J Pediatr Surg 36:133–140
Guidry CA, Hranjec T, Rodgers BM, Kane B, McGahren ED (2012) Permissive hypercapnia in the management of congenital diaphragmatic hernia: our institutional experience. J Am Coll Surg 214(640–645):647
Henderson-Smart DJ, De Paoli AG, Clark RH, Bhuta T (2009) High frequency oscillatory ventilation versus conventional ventilation for infants with severe pulmonary dysfunction born at or near term. Cochrane Database Syst Rev (13):CD002974. doi: 10.1002/14651858
Kinsella JP, Truog WE, Walsh WF, Goldberg RN, Bancalari E, Mayock DE, Redding GJ, deLemos RA, Sardesai S, McCurnin DC, Moreland SG, Cutter GR, Abman SH (1997) Randomized, multicenter trial of inhaled nitric oxide and high-frequency oscillatory ventilation in severe, persistent pulmonary hypertension of the newborn. J Pediatr 131:55–62
van den Hout L, Tibboel D, Vijfhuize S, te Beest H, Hop W, Reiss I (2011) The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial. BMC Pediatr 11:98
Oliveira CA, Troster EJ, Pereira CR (2000) Inhaled nitric oxide in the management of persistent pulmonary hypertension of the newborn: a meta-analysis. Rev Hosp Clin Fac Med Sao Paulo 55:145–154
Bialkowski A, Moenkemeyer F, Patel N (2015) Intravenous sildenafil in the management of pulmonary hypertension associated with congenital diaphragmatic hernia. Eur J Pediatr Surg 25:171–176
Rocha G, Baptista MJ, Correia-Pinto J, Guimaraes H (2013) Congenital diaphragmatic hernia: experience of 14 years. Minerva Pediatr 65:271–278
Malowitz JR, Hornik CP, Laughon MM, Testoni D, Cotten CM, Clark RH, Smith PB (2015) Management practice and mortality for infants with congenital diaphragmatic hernia. Am J Perinatol 32:887–894
McNamara PJ, Laique F, Muang-In S, Whyte HE (2006) Milrinone improves oxygenation in neonates with severe persistent pulmonary hypertension of the newborn. J Crit Care 21:217–222
Patel N (2012) Use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients. Neonatology 102:130–136
Mohseni-Bod H, Bohn D (2007) Pulmonary hypertension in congenital diaphragmatic hernia. Semin Pediatr Surg 16:126–133
Hofmann SR, Stadler K, Heilmann A, Hausler HJ, Fitze G, Kamin G, Nitzsche KI, Hahn G, Dinger J (2012) Stabilisation of cardiopulmonary function in newborns with congenital diaphragmatic hernia using lung function parameters and hemodynamic management. Klin Padiatr 224:e1–e10
Skarda DE, Yoder BA, Anstadt EE, Lally PA, Greene T, McFadden M, Rollins MD (2014) Epoprostenol does not affect mortality in neonates with congenital diaphragmatic hernia. Eur J Pediatr Surg. doi: 10.1055/s-0034-1389096
Kays DW, Islam S, Perkins JM, Larson SD, Taylor JA, Talbert JL (2015) Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: whom should we treat? J Pediatr Surg 50:893–897
Prodhan P, Stroud M, El-Hassan N, Peeples S, Rycus P, Brogan TV, Tang X (2014) Prolonged extracorporeal membrane oxygenator support among neonates with acute respiratory failure: a review of the Extracorporeal Life Support Organization registry. ASAIO J 60:63–69
Davis PJ, Firmin RK, Manktelow B, Goldman AP, Davis CF, Smith JH, Cassidy JV, Shekerdemian LS (2004) Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience. J Pediatr 144:309–315
Morini F, Goldman A, Pierro A (2006) Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence. Eur J Pediatr Surg 16:385–391
Mugford M, Elbourne D, Field D (2008) Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database Syst Rev (3):CD001340. doi: 10.1002/14651858.CD001340.pub2
Ijsselstijn H, van Heijst AF (2014) Long-term outcome of children treated with neonatal extracorporeal membrane oxygenation: increasing problems with increasing age. Semin Perinatol 38:114–121
Azarow K, Messineo A, Pearl R, Filler R, Barker G, Bohn D (1997) Congenital diaphragmatic hernia–a tale of two cities: the Toronto experience. J Pediatr Surg 32:395–400
Fallon SC, Cass DL, Olutoye OO, Zamora IJ, Lazar DA, Larimer EL, Welty SE, Moise AA, Demny AB, Lee TC (2013) Repair of congenital diaphragmatic hernias on Extracorporeal Membrane Oxygenation (ECMO): does early repair improve patient survival? J Pediatr Surg 48:1172–1176
Zalla JM, Stoddard GJ, Yoder BA (2015) Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15 year experience in a single institution. J Pediatr Surg 50:524–527
Garcia A, Stolar CJ (2012) Congenital diaphragmatic hernia and protective ventilation strategies in pediatric surgery. Surg Clin N Am 92:659–68, ix
Kays DW, Islam S, Richards DS, Larson SD, Perkins JM, Talbert JL (2014) Extracorporeal life support in patients with congenital diaphragmatic hernia: how long should we treat? J Am Coll Surg 218:808–817
Desai AA, Ostlie DJ, Juang D (2015) Optimal timing of congenital diaphragmatic hernia repair in infants on extracorporeal membrane oxygenation. Semin Pediatr Surg 24:17–19
Partridge EA, Peranteau WH, Rintoul NE, Herkert LM, Flake AW, Adzick NS, Hedrick HL (2015) Timing of repair of congenital diaphragmatic hernia in patients supported by extracorporeal membrane oxygenation (ECMO). J Pediatr Surg 50:260–262
Grant H, Rode H, Cywes S (1994) Potential danger of ‘trial of life’ approach to congenital diaphragmatic hernia. J Pediatr Surg 29:399
Garriboli M, Duess JW, Ruttenstock E, Bishay M, Eaton S, De CP, Puri P, Hollwarth ME, Pierro A (2012) Trends in the treatment and outcome of congenital diaphragmatic hernia over the last decade. Pediatr Surg Int 28:1177–1181
Hollinger LE, Lally PA, Tsao K, Wray CJ, Lally KP (2014) A risk-stratified analysis of delayed congenital diaphragmatic hernia repair: does timing of operation matter? Surgery 156:475–482
Rozmiarek AJ, Qureshi FG, Cassidy L, Ford HR, Hackam DJ (2004) Factors influencing survival in newborns with congenital diaphragmatic hernia: the relative role of timing of surgery. J Pediatr Surg 39:821–824
Kays DW, Islam S, Larson SD, Perkins J, Talbert JL (2013) Long-term maturation of congenital diaphragmatic hernia treatment results: toward development of a severity-specific treatment algorithm. Ann Surg 258:638–644
Moyer V, Moya F, Tibboel R, Losty P, Nagaya M, Lally KP (2002) Late versus early surgical correction for congenital diaphragmatic hernia in newborn infants. Cochrane Database Syst Rev:CD001695
Pacilli M, Pierro A, Kingsley C, Curry JI, Herod J, Eaton S (2006) Absorption of carbon dioxide during laparoscopy in children measured using a novel mass spectrometric technique. Br J Anaesth 97:215–219
Bishay M, Giacomello L, Retrosi G, Thyoka M, Nah SA, McHoney M, De CP, Brierley J, Scuplak S, Kiely EM, Curry JI, Drake DP, Cross KM, Eaton S, Pierro A (2011) Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants. J Pediatr Surg 46:47–51
McHoney M, Mackinlay G, Munro F, Capek A, Aldridge L (2008) Effect of patient weight and anesthetic technique on CO(2) excretion during thoracoscopy in children assessed by end-tidal CO(2). J Laparoendosc Adv Surg Tech A 18:147–151
Mortellaro VE, Fike FB, Adibe OO, Juang D, Aguayo P, Ostlie DJ, Holcomb GW (2011) St Peter SD. The use of high-frequency oscillating ventilation to facilitate stability during neonatal thoracoscopic operations. J Laparoendosc Adv Surg Tech A 21:877–879
McHoney M, Giacomello L, Nah SA, De CP, Kiely EM, Curry JI, Drake DP, Eaton S, Pierro A (2010) Thoracoscopic repair of congenital diaphragmatic hernia: intraoperative ventilation and recurrence. J Pediatr Surg 45:355–359
Bishay M, Giacomello L, Retrosi G, Thyoka M, Garriboli M, Brierley J, Harding L, Scuplak S, Cross KM, Curry JI, Kiely EM, De CP, Eaton S, Pierro A (2013) Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial. Ann Surg 258:895–900
Szavay PO, Obermayr F, Maas C, Luenig H, Blumenstock G, Fuchs J (2012) Perioperative outcome of patients with congenital diaphragmatic hernia undergoing open versus minimally invasive surgery. J Laparoendosc Adv Surg Tech A 22:285–289
Inoue M, Uchida K, Otake K, Nagano Y, Mori K, Hashimoto K, Matsushita K, Koike Y, Uemura A, Kusunoki M (2015) Thoracoscopic repair of congenital diaphragmatic hernia with countermeasures against reported complications for safe outcomes comparable to laparotomy. Surg Endosc
Garriboli M, Bishay M, Kiely EM, Drake DP, Curry JI, Cross KM, Eaton S, De CP, Pierro A (2013) Recurrence rate of Morgagni diaphragmatic hernia following laparoscopic repair. Pediatr Surg Int 29:185–189
Tsao K, Lally PA, Lally KP (2011) Minimally invasive repair of congenital diaphragmatic hernia. J Pediatr Surg 46:1158–1164
Lansdale N, Alam S, Losty PD, Jesudason EC (2010) Neonatal endosurgical congenital diaphragmatic hernia repair: a systematic review and meta-analysis. Ann Surg 252:20–26
Fishman JR, Blackburn SC, Jones NJ, Madden N, De CD, Haddad MJ, Clarke SA (2011) Does thoracoscopic congenital diaphragmatic hernia repair cause a significant intraoperative acidosis when compared to an open abdominal approach? J Pediatr Surg 46:458–461
Liem NT, Nhat LQ, Tuan TM (2011) Dung lA, Ung NQ, Dien TM. Thoracoscopic repair for congenital diaphragmatic hernia: experience with 139 cases. J Laparoendosc Adv Surg Tech A 21:267–270
Gomes FC, Kuhn P, Lacreuse I, Kasleas C, Philippe P, Podevin G, Bonnard A, Lopez M, de Lagausie P, Petit T, Lardy H, Becmeur F (2013) Congenital diaphragmatic hernia: an evaluation of risk factors for failure of thoracoscopic primary repair in neonates. J Pediatr Surg 48:488–495
Yang EY, Allmendinger N, Johnson SM, Chen C, Wilson JM, Fishman SJ (2005) Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome. J Pediatr Surg 40:1369–1375
Nam SH, Cho MJ, Kim DY, Kim SC (2013) Shifting from laparotomy to thoracoscopic repair of congenital diaphragmatic hernia in neonates: early experience. World J Surg 37:2711–2716
Vijfhuize S, Deden AC, Costerus SA, Sloots CE, Wijnen RM (2012) Minimal access surgery for repair of congenital diaphragmatic hernia: is it advantageous? An open review. Eur J Pediatr Surg 22:364–373
Mitchell IC, Garcia NM, Barber R, Ahmad N, Hicks BA, Fischer AC (2008) Permacol: a potential biologic patch alternative in congenital diaphragmatic hernia repair. J Pediatr Surg 43:2161–2164
Laituri CA, Garey CL, Valusek PA, Fike FB, Kaye AJ, Ostlie DJ, Snyder CL (2010) St Peter SD. Outcome of congenital diaphragmatic hernia repair depending on patch type. Eur J Pediatr Surg 20:363–365
Jawaid WB, Qasem E, Jones MO, Shaw NJ, Losty PD (2013) Outcomes following prosthetic patch repair in newborns with congenital diaphragmatic hernia. Br J Surg 100:1833–1837
Romao RL, Nasr A, Chiu PP, Langer JC (2012) What is the best prosthetic material for patch repair of congenital diaphragmatic hernia? Comparison and meta-analysis of porcine small intestinal submucosa and polytetrafluoroethylene. J Pediatr Surg 47:1496–1500
Ricci KB, Higgins R, Daniels VC, Kilic A (2014) Bovine pericardial reconstruction of the diaphragm after a heart transplant. Exp Clin Transplant 12:277–278
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
McHoney, M. Congenital diaphragmatic hernia, management in the newborn. Pediatr Surg Int 31, 1005–1013 (2015). https://doi.org/10.1007/s00383-015-3794-9
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-015-3794-9