Extracorporeal Membrane Oxygenation for Neonates with Congenital Renal and Urological Anomalies and Pulmonary Hypoplasia: A Case Report and Review of the Extracorporeal Life Support Organization Registry

Dayanand Bagdure; Natalie Torres; L. Kyle Walker; Jaylyn Waddell; Adnan Bhutta; Jason W. Custer

doi:10.1055/s-0037-1598036

Journal of Pediatric Intensive Care, Table of Contents

J Pediatr Intensive Care 2017; 06(03): 188-193
DOI: 10.1055/s-0037-1598036

Original Article

Georg Thieme Verlag KG Stuttgart · New York

Extracorporeal Membrane Oxygenation for Neonates with Congenital Renal and Urological Anomalies and Pulmonary Hypoplasia: A Case Report and Review of the Extracorporeal Life Support Organization Registry

Dayanand Bagdure

¹Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, United States

,

Natalie Torres

²Department of Pediatrics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware, United States

,

L. Kyle Walker

¹Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, United States

,

Jaylyn Waddell

³Division of Neonatology, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, United States

,

Adnan Bhutta

¹Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, United States

,

Jason W. Custer

¹Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Maryland School of Medicine, Baltimore, Maryland, United States

› Author Affiliations

Abstract

Objective Congenital anomalies of the kidney and urinary tract constitute up to 30% of anomalies identified in the neonatal period. In utero oligohydramnios is often associated with pulmonary hypoplasia and respiratory failure in the neonate who may not be responsive to mechanical ventilation. Placement of these neonates on extracorporeal membrane oxygenation (ECMO) remains controversial and is considered in most centers to be a relative contraindication. The objective of this study is to use the Extracorporeal Life Support Organization (ELSO) database to describe the outcomes and complications of patients with congenital renal and urogenital anomalies with pulmonary hypoplasia who underwent ECMO in the neonatal period.

Data Sources Data from the ELSO registry were retrospectively reviewed for all patients with congenital renal and urogenital anomalies with pulmonary hypoplasia treated with ECMO support between 1990 and November 2014 using ICD-9 diagnosis codes.

Data Synthesis We identified 45 patients. The average age of the patient at the time of ECMO was 1.7 days (range: 0–14 days) and weight was 3.1 kg (interquartile range [IQR]: 2.5–3.3). Patients spent an average of 162 hours on ECMO (IQR: 81–207). The majority of patients were managed with venoarterial ECMO (60%), and the overall survival of this cohort was 42%. Survivors had higher weights (3.4 vs. 2.8 kg; p < 0.019) and were more likely to be male (90 vs. 44%; p < 0.002). Patients with obstructive urogenital lesions had an overall survival of 71 versus 16.6% in patients with a primary intrinsic renal diagnosis (p = 0.004). Renal replacement therapy was required in 51% of the patients during their ECMO support.

Conclusion Neonates with renal or urogenital disease and pulmonary hypoplasia have an overall survival rate of 42%. Patients with a diagnosis of urogenital obstruction have much more favorable outcomes when compared with those with intrinsic renal disease such as polycystic kidney disease.

Keywords

extracorporeal membrane oxygenation - Extracorporeal Life Support Organization - pulmonary hypoplasia - congenital renal anomalies - outcomes

Full Text

References

References
1 Kemper MJ, Mueller-Wiefel DE. Prognosis of antenatally diagnosed oligohydramnios of renal origin. Eur J Pediatr 2007; 166 (05) 393-398
2 Abrams ME, Ackerman VL, Engle WA. Primary unilateral pulmonary hypoplasia: neonate through early childhood - case report, radiographic diagnosis and review of the literature. J Perinatol 2004; 24 (10) 667-670
3 Wigglesworth JS, Desai R. Is fetal respiratory function a major determinant of perinatal survival?. Lancet 1982; 1 (8266): 264-267
4 Prodhan P, Stroud M, El-Hassan N. , et al. Prolonged extracorporeal membrane oxygenator support among neonates with acute respiratory failure: a review of the Extracorporeal Life Support Organization registry. ASAIO J 2014; 60 (01) 63-69
5 Bokman CL, Tashiro J, Perez EA, Lasko DS, Sola JE. Determinants of survival and resource utilization for pediatric extracorporeal membrane oxygenation in the United States 1997-2009. J Pediatr Surg 2015; 50 (05) 809-814
6 Rehder KJ, Turner DA, Cheifetz IM. Extracorporeal membrane oxygenation for neonatal and pediatric respiratory failure: an evidence-based review of the past decade (2002-2012). Pediatr Crit Care Med 2013; 14 (09) 851-861
7 Gupta P, McDonald R, Chipman CW. , et al. 20-year experience of prolonged extracorporeal membrane oxygenation in critically ill children with cardiac or pulmonary failure. Ann Thorac Surg 2012; 93 (05) 1584-1590
8 Koumbourlis AC, Wung JT, Stolar CJ. Lung function in infants after repair of congenital diaphragmatic hernia. J Pediatr Surg 2006; 41 (10) 1716-1721
9 Akinkuotu AC, Sheikh F, Cass DL. , et al. Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation. J Pediatr Surg 2015; 50 (01) 55-59
10 Seetharamaiah R, Younger JG, Bartlett RH, Hirschl RB. ; 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 (07) 1315-1321
11 Caesar RE, Packer MG, Kaplan GW. , et al. Extracorporeal membrane oxygenation in the neonate with congenital renal disease and pulmonary hypoplasia. J Pediatr Surg 1995; 30 (11) 1560-1563
12 Domico MB, Ridout DA, Bronicki R. , et al. The impact of mechanical ventilation time before initiation of extracorporeal life support on survival in pediatric respiratory failure: a review of the Extracorporeal Life Support Registry. Pediatr Crit Care Med 2012; 13 (01) 16-21
13 Zahraa JN, Moler FW, Annich GM, Maxvold NJ, Bartlett RH, Custer JR. Venovenous versus venoarterial extracorporeal life support for pediatric respiratory failure: are there differences in survival and acute complications?. Crit Care Med 2000; 28 (02) 521-525
14 Chen H, Yu RG, Yin NN, Zhou JX. Combination of extracorporeal membrane oxygenation and continuous renal replacement therapy in critically ill patients: a systematic review. Crit Care 2014; 18 (06) 675 . Doi: 10.1186/s13054-014-0675-x
15 Gallot D, Boda C, Ughetto S. , et al. Prenatal detection and outcome of congenital diaphragmatic hernia: a French registry-based study. Ultrasound Obstet Gynecol 2007; 29 (03) 276-283
16 Heiss K, Manning P, Oldham KT. , et al. Reversal of mortality for congenital diaphragmatic hernia with ECMO. Ann Surg 1989; 209 (02) 225-230
17 Zalla JM, Stoddard GJ, Yoder BA. Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15. year experience in a single institution. J Pediatr Surg 2015; 50 (04) 524-527
18 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?. J Pediatr Surg 2015; 50 (06) 893-897
19 Zamora IJ, Olutoye OO, Cass DL. , et al. Prenatal MRI fetal lung volumes and percent liver herniation predict pulmonary morbidity in congenital diaphragmatic hernia (CDH). J Pediatr Surg 2014; 49 (05) 688-693
20 Mallett TM, O'Hagan E, McKeever KG. Early bilateral nephrectomy in infantile autosomal recessive polycystic kidney disease. BMJ Case Rep 2015; ;2015.
21 Extracorporeal Life Support Organization. ECLS Registry Report. 2016 . Available at https://www.elso.org/Registry/Statistics/InternationalSummary.aspx . Accessed June 21, 2016