First Population-Based Report of Infants with Congenital Diaphragmatic Hernia: 30-Day Outcomes from the American College of Surgeons National Quality Improvement Program

Elke Zani-Ruttenstock; Augusto Zani; Simon Eaton; Annie Fecteau

doi:10.1055/s-0038-1668563

European Journal of Pediatric Surgery, Table of Contents

Eur J Pediatr Surg 2019; 29(01): 062-067
DOI: 10.1055/s-0038-1668563

Original Article

Georg Thieme Verlag KG Stuttgart · New York

First Population-Based Report of Infants with Congenital Diaphragmatic Hernia: 30-Day Outcomes from the American College of Surgeons National Quality Improvement Program

Authors

Elke Zani-Ruttenstock

¹Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

²Department of Surgery, University of Toronto, Toronto, Ontario, Canada
Augusto Zani

¹Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

²Department of Surgery, University of Toronto, Toronto, Ontario, Canada
Simon Eaton

³UCL Great Ormond Street Institute of Child Health, London, United Kingdom
Annie Fecteau

¹Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

²Department of Surgery, University of Toronto, Toronto, Ontario, Canada

Abstract

Aim The American College of Surgeons has developed a registry, the National Quality Improvement Program Pediatric (NSQIP-P), that provides participating centers with high-quality surgical outcome data for children. Herein, we aimed to analyze for the first time the short-term outcomes of live-born infants with congenital diaphragmatic hernia (CDH) registered on this large North American database.

Methods During 2015 to 2016, up to 101 participating centers uploaded 95 perioperative data points on the NSQIP-P database for patients that underwent surgical repair of CDH. The demographics, peri-, and post-operative data (up to 30 days following surgical repair) of infants with CDH were reviewed. Binary logistic regression was performed to test associations between risk factors and mortality.

Main Results There were 432 (61% male) infants, who underwent CDH surgical repair during the study period. The prematurity rate (gestational age < 37 weeks) was 17%. The majority of infants (82%) had cardiac risk factors identified (72% were reported as major/severe). Extracorporeal membrane oxygenation (ECMO) was employed in 13% of patients prior to surgery. The majority of infants (83%) were ventilated preoperatively, and 34% received inotropes. Median age at surgery was 5 (0–74) days. CDH repair was attempted via thoracoscopy in 18% (n = 79) infants, but with a high rate of conversion to open surgery (n = 32, 41%). The postoperative 30-day mortality rate was 9%. At binary logistic regression, major cardiac risk factors (odds ratio [OR], 1.7 [0.9–3.2], p = 0.095), Appearance, Pulse, Grimace, Activity, and Respiration at 1 minute (OR, 0.7 per unit [0.5–0.8], p < 0.005), and birth weight (OR, 0.5 per kg [0.2–1.0], p < 0.05) were retained in the final model as significantly associated with mortality.

Conclusion This is the first report on CDH outcomes from the NSQIP-P database. Utilization of ECMO was low compared with single-center studies from North America. The early postoperative mortality rate of babies with CDH considered suitable for surgery remains high.

Keywords

congenital diaphragmatic hernia - short-term outcome - Bochdalek hernia - NSQIP - epidemiology

Full Text

References

References
1 Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis 2012; 7: 1
2 Zani A, Zani-Ruttenstock E, Pierro A. Advances in the surgical approach to congenital diaphragmatic hernia. Semin Fetal Neonatal Med 2014; 19 (06) 364-369
3 Ruttenstock E, Wright N, Barrena S. , et al. Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia. Eur J Pediatr Surg 2015; 25 (01) 3-8
4 Morini F, Lally PA, Lally KP, Bagolan P. The Congenital Diaphragmatic Hernia Study Group Registry. Eur J Pediatr Surg 2015; 25 (06) 488-496
5 Dyamenahalli U, Morris M, Rycus P, Bhutta AT, Tweddell JS, Prodhan P. Short-term outcome of neonates with congenital heart disease and diaphragmatic hernia treated with extracorporeal membrane oxygenation. Ann Thorac Surg 2013; 95 (04) 1373-1376
6 Grover TR, Murthy K, Brozanski B. , et al; Children's Hospitals Neonatal Consortium. Short-term outcomes and medical and surgical interventions in infants with congenital diaphragmatic hernia. Am J Perinatol 2015; 32 (11) 1038-1044
7 Goonasekera C, Ali K, Hickey A. , et al. Mortality following congenital diaphragmatic hernia repair: the role of anesthesia. Paediatr Anaesth 2016; 26 (12) 1197-1201
8 Barroso C, Correia-Pinto J. Perioperative complications of congenital diaphragmatic hernia repair. Eur J Pediatr Surg 2018; 28 (02) 141-147
9 Long AM, Bunch KJ, Knight M. , et al. Early population-based outcomes of infants born with congenital diaphragmatic hernia. Arch Dis Child Fetal Neonatal 2018; 103 (06) F517-F522
10 Raval MV, Dillon PW, Bruny JL. , et al; ACS NSQIP Pediatric Steering Committee. American College of Surgeons national surgical quality improvement program pediatric: a phase 1 report. J Am Coll Surg 2011; 212 (01) 1-11
11 Daodu O, Brindle ME. Predicting outcomes in congenital diaphragmatic hernia. Semin Pediatr Surg 2017; 26 (03) 136-139
12 Knox E, Lissauer D, Khan K, Kilby M. Prenatal detection of pulmonary hypoplasia in fetuses with congenital diaphragmatic hernia: a systematic review and meta-analysis of diagnostic studies. J Matern Fetal Neonatal Med 2010; 23 (07) 579-588
13 Debus A, Hagelstein C, Kilian AK. , et al. Fetal lung volume in congenital diaphragmatic hernia: association of prenatal MR imaging findings with postnatal chronic lung disease. Radiology 2013; 266 (03) 887-895
14 Coughlin MA, Werner NL, Gajarski R. , et al. Prenatally diagnosed severe CDH: mortality and morbidity remain high. J Pediatr Surg 2016; 51 (07) 1091-1095
15 Akinkuotu AC, Cruz SM, Abbas PI. , et al. Risk-stratification of severity for infants with CDH: prenatal versus postnatal predictors of outcome. J Pediatr Surg 2016; 51 (01) 44-48
16 Congenital Diaphragmatic Hernia Study Group. Estimating disease severity of congenital diaphragmatic hernia in the first 5 minutes of life. J Pediatr Surg 2001; 36 (01) 141-145
17 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 (02) e413-e419
18 Skarsgard ED, MacNab YC, Qiu Z, Little R, Lee SK. ; Canadian Neonatal Network. SNAP-II predicts mortality among infants with congenital diaphragmatic hernia. J Perinatol 2005; 25 (05) 315-319
19 Chiu LW, Desai J, Shanti C. , et al. SNAPPE II score as a predictor of survival in neonates with congenital diaphragmatic hernia: a single center experience. Eur J Pediatr Surg 2016; 26 (04) 316-321
20 Menon SC, Tani LY, Weng HY, Lally PA, Lally KP, Yoder BA. ; Congenital Diaphragmatic Hernia Study Group. Clinical characteristics and outcomes of patients with cardiac defects and congenital diaphragmatic hernia. J Pediatr 2013; 162 (01) 114-119
21 Pierro M, Thébaud B. Understanding and treating pulmonary hypertension in congenital diaphragmatic hernia. Semin Fetal Neonatal Med 2014; 19 (06) 357-363
22 Burgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP. ; Congenital Diaphragmatic Hernia Study Group. Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - side, stage, and outcome. J Pediatr Surg 2018 Doi: 10.1016/j.jpedsurg.2018.04.008
23 Abdullah F, Zhang Y, Sciortino C. , et al. Congenital diaphragmatic hernia: outcome review of 2,173 surgical repairs in US infants. Pediatr Surg Int 2009; 25 (12) 1059-1064
24 American Heart Association. 2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric advanced life support. Pediatrics 2006; 117 (05) e1005-e1028
25 Reiss I, Schaible T, van den Hout L. , et al; CDH EURO Consortium. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010; 98 (04) 354-364
26 Canadian Congenital Diaphragmatic Hernia Collaborative. Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline. CMAJ 2018; 190 (04) E103-E112
27 Zani A, Eaton S, Puri P. , et al; EUPSA Network Office. International survey on the management of congenital diaphragmatic hernia. Eur J Pediatr Surg 2016; 26 (01) 38-46
28 Putnam LR, Tsao K, Lally KP. , et al; Congenital Diaphragmatic Hernia Study Group and the Pediatric Surgery Research Collaborative. Minimally invasive vs open congenital diaphragmatic hernia repair: is there a superior approach?. J Am Coll Surg 2017; 224 (04) 416-422
29 Vijfhuize S, Deden AC, Costerus SA, Sloots CE, Wijnen RM. Minimal access surgery for repair of congenital diaphragmatic hernia: is it advantageous?--an open review. Eur J Pediatr Surg 2012; 22 (05) 364-373
30 Putnam LR, Gupta V, Tsao K. , et al; Congenital Diaphragmatic Hernia Study Group. Factors associated with early recurrence after congenital diaphragmatic hernia repair. J Pediatr Surg 2017; 52 (06) 928-932
31 Nagata K, Usui N, Terui K. , et al. Risk factors for the recurrence of the congenital diaphragmatic hernia-report from the long-term follow-up study of Japanese CDH study group. Eur J Pediatr Surg 2015; 25 (01) 9-14
32 Alluri RK, Leland H, Heckmann N. Surgical research using national databases. Ann Transl Med 2016; 4 (20) 393