Download PDF
Am J Perinatol 2015; 32(11): 1038-1044
DOI: 10.1055/s-0035-1548729
Original Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Short-Term Outcomes and Medical and Surgical Interventions in Infants with Congenital Diaphragmatic Hernia

Theresa R. Grover
1   Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado
,
Karna Murthy   *
2   Ann and Robert H. Lurie Children's Hospital and Feinberg School of Medicine, Northwestern University, Chicago, Illinois
,
Beverly Brozanski
3   Children's Hospital of Pittsburgh and University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
,
Jason Gien
1   Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado
,
Natalie Rintoul
4   Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
,
Sarah Keene
5   Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, Georgia
,
Tasnim Najaf
6   Washington University, St. Louis, Missouri
,
Louis Chicoine
7   Nationwide Children's Hospital, Columbus, Ohio
,
Nicolas Porta
2   Ann and Robert H. Lurie Children's Hospital and Feinberg School of Medicine, Northwestern University, Chicago, Illinois
,
Isabella Zaniletti
8   Children's Hospital Association, Overland Park, Kansas
,
Eugenia K. Pallotto   *
9   Children's Mercy Hospital and University of Missouri, Kansas City, Missouri
,
and the Children's Hospitals Neonatal Consortium › Author Affiliations
Further Information

Publication History

14 October 2014

20 January 2015

Publication Date:
31 March 2015 (online)

Also available at
   Permissions and Reprints

Abstract

Objective The aim of this study is to characterize medical and surgical therapies and short-term outcomes in infants with congenital diaphragmatic hernia (CDH).

Study Design Retrospective analysis of CDH infants admitted to 27 children's hospitals submitting data to Children's Hospital Neonatal Database (CHND) from 2010 to 2013, stratified by gestational age, birth weight, and survival.

Results A total of 572 infants were identified, 508 (89%) born ≥ 34 weeks' gestation and ≥ 2 kg. More mature infants had higher APGAR scores, shorter duration of mechanical ventilation, and were more likely to receive extracorporeal membrane oxygenation (ECMO). Overall, mortality for the cohort was 29%, with mortality lower in infants born ≥ 34 weeks' gestation and ≥ 2 kg (26 vs. 50%, p < 0.01). Nonsurvivors were more likely to receive treatment with high-frequency oscillatory ventilation (HFOV), vasopressors, pulmonary vasodilators, and ECMO, and to have associated major congenital anomalies than survivors. In hospital morbidity and complications were relatively uncommon among survivors.

Conclusion Infants with CDH have a high risk of morbidity and mortality, and for preterm infants with CDH those risks are amplified. Patterns of respiratory and circulatory support appeared to be different for survivors. In addition to established data registries, this consortium of regional neonatal intensive care units provides a new collaborative effort to describe short-term outcomes for infants referred with CDH.

Keywords

congenital diaphragmatic hernia - neonatal - pulmonary hypertension - prematurity - NICU - pulmonary hypoplasia - respiratory failure

* Michael A Padula, Jeanette M Asselin, David J Durand, Billie L Short, Francine D Dykes, Kristina M Reber, and Jacquelyn R Evans.


 

  • References

  • 1 Langham Jr MR, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS. Congenital diaphragmatic hernia. Epidemiology and outcome. Clin Perinatol 1996; 23 (4) 671-688
    CrossrefPubMedGoogle Scholar
  • 2 Bohn D. Congenital diaphragmatic hernia. Am J Respir Crit Care Med 2002; 166 (7) 911-915
    CrossrefPubMedGoogle Scholar
  • 3 Keijzer R, Puri P. Congenital diaphragmatic hernia. Semin Pediatr Surg 2010; 19 (3) 180-185
    CrossrefPubMedGoogle Scholar
  • 4 Wynn J, Krishnan U, Aspelund G , et al. Outcomes of congenital diaphragmatic hernia in the modern era of management. J Pediatr 2013; 163 (1) 114-9.e1
    CrossrefPubMedGoogle Scholar
  • 5 Tsao K, Lally KP The Congenital Diaphragmatic Hernia Study Group. a voluntary international registry. Semin Pediatr Surg 2008; 17 (2) 90-97
    CrossrefPubMedGoogle Scholar
  • 6 Skarsgard ED. Networks in Canadian paediatric surgery: Time to get connected. Paediatr Child Health (Oxford) 2006; 11 (1) 15-18
    PubMedGoogle Scholar
  • 7 Harting MT, Lally KP. The Congenital Diaphragmatic Hernia Study Group registry update. Semin Fetal Neonatal Med 2014; 19 (6) 370-375
    CrossrefPubMedGoogle Scholar
  • 8 Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP ; Congenital Diaphragmatic Hernia Study Group. A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatrics 2014; 134 (2) e413-e419
    CrossrefPubMedGoogle Scholar
  • 9 Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000; 35 (8) 1187-1197
    CrossrefPubMedGoogle Scholar
  • 10 Schultz CM, DiGeronimo RJ, Yoder BA ; Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome. J Pediatr Surg 2007; 42 (3) 510-516
    CrossrefPubMedGoogle Scholar
  • 11 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
    CrossrefPubMedGoogle Scholar
  • 12 Baird R, Eeson G, Safavi A, Puligandla P, Laberge JM, Skarsgard ED ; Canadian Pediatric Surgery Network. Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network. J Pediatr Surg 2011; 46 (5) 801-807
    CrossrefPubMedGoogle Scholar
  • 13 Murthy K, Dykes FD, Padula MA , et al. The Children's Hospitals Neonatal Database: an overview of patient complexity, outcomes and variation in care. J Perinatol 2014; 34 (8) 582-586
    Google Scholar
  • 14 American Academy of Pediatrics Committee on Fetus And Newborn. Levels of neonatal care. Pediatrics 2012; 130 (3) 587-597
    CrossrefPubMedGoogle Scholar
  • 15 Van Meurs K, Lally KP, Peek G, Zwischenberger JB , eds. ECMO Extracorporeal Cardiopulmonary Support in Critical Care. In: Extracorporeal Life Support Organization (ELSO) 3rd ed. Ann Arbor, MI: 2005
    Google Scholar
  • 16 Olsen IE, Groveman SA, Lawson ML, Clark RH, Zemel BS. New intrauterine growth curves based on United States data. Pediatrics 2010; 125 (2) e214-e224
    CrossrefPubMedGoogle Scholar
  • 17 Tsao K, Allison ND, Harting MT, Lally PA, Lally KP. Congenital diaphragmatic hernia in the preterm infant. Surgery 2010; 148 (2) 404-410
    CrossrefPubMedGoogle Scholar
  • 18 Levison J, Halliday R, Holland AJ , et al; Neonatal Intensive Care Units Study of the NSW Pregnancy and Newborn Services Network. A population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001. J Pediatr Surg 2006; 41 (6) 1049-1053
    CrossrefPubMedGoogle Scholar
  • 19 Jaffray B, MacKinlay GA. Real and apparent mortality from congenital diaphragmatic hernia. Br J Surg 1996; 83 (1) 79-82
    CrossrefPubMedGoogle Scholar
  • 20 Harrison MR, Bjordal RI, Langmark F, Knutrud O. Congenital diaphragmatic hernia: the hidden mortality. J Pediatr Surg 1978; 13 (3) 227-230
    CrossrefPubMedGoogle Scholar
  • 21 Campbell BT, Herbst KW, Briden KE, Neff S, Ruscher KA, Hagadorn JI. Inhaled nitric oxide use in neonates with congenital diaphragmatic hernia. Pediatrics 2014; 134 (2) e420-e426
    CrossrefPubMedGoogle Scholar
  • 22 Hunter L, Richens T, Davis C, Walker G, Simpson JH. Sildenafil use in congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal Ed 2009; 94 (6) F467
    PubMedGoogle Scholar
  • 23 Su W, Berry M, Puligandla PS, Aspirot A, Flageole H, Laberge JM. Predictors of gastroesophageal reflux in neonates with congenital diaphragmatic hernia. J Pediatr Surg 2007; 42 (10) 1639-1643
    CrossrefPubMedGoogle Scholar
  • 24 Clark RH, Kueser TJ, Walker MW , et al; Clinical Inhaled Nitric Oxide Research Group. Low-dose nitric oxide therapy for persistent pulmonary hypertension of the newborn. N Engl J Med 2000; 342 (7) 469-474
    CrossrefPubMedGoogle Scholar
  • 25 The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics 1997; 99 (6) 838-845
    CrossrefPubMedGoogle Scholar
  • 26 Porta NF, Steinhorn RH. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin Perinatol 2012; 39 (1) 149-164
    CrossrefPubMedGoogle Scholar