Necrotizing Enterocolitis Incidence, Characteristics, and Outcomes in Neonatal Down Syndrome Patients

Abstract

Introduction Neonates with Down syndrome (nDS) have multiple medical issues that may place them at an increased risk of necrotizing enterocolitis (NEC). The goal of this study was to determine the incidence, characteristics, and outcomes of nDS patients that developed NEC.

Methods Data from the Pediatric Health Information Systems database on all nDS were reviewed. Demographics, medical conditions, development of NEC, and mortality were recorded. Patients were divided into nDS patients who developed NEC (nDS-NEC) versus nDS patients who did not develop NEC (nDS-nNEC).

Results Incidence of NEC in nDS patients was 6.6% (381/5,737). Baseline demographic data indicated nDS-NEC patients were more likely to be born earlier, have a diagnosis of congenital diaphragmatic hernia, ventricular septal defect, patent ductus arteriosus, Ebstein's anomaly, or a left-sided obstructive lesion versus nDS-nNEC patients. The odds ratio for death in the nDS-NEC patients was 2.5 (95% confidence interval, 1.8–3.3) versus the nDS-NEC patients.

Conclusion The incidence of NEC in nDS patients requiring admission to a children's hospital after birth is much higher than that reported for term infants. Baseline characteristics differ in nDS patients that may place them at a higher risk for NEC. nDS-NEC patients have an increased morbidity and a significantly higher mortality compared with the nDS-nNEC patients.

Keywords

necrotizing enterocolitis - Down syndrome - neonatal

• References

• 1 Wójkowska-Mach J, Różańska A, Borszewska-Kornacka M. , et al. Necrotising enterocolitis in preterm infants: epidemiology and antibiotic consumption in the Polish neonatology network neonatal intensive care units in 2009. PLoS One 2014; 9 (03) e92865 . Doi: 10.1371/journal.pone.0092865
• 2 Yee WH, Soraisham AS, Shah VS, Aziz K, Yoon W, Lee SK. ; Canadian Neonatal Network. Incidence and timing of presentation of necrotizing enterocolitis in preterm infants. Pediatrics 2012; 129 (02) e298-e304
• 3 Viswanathan S, McNelis K, Super D, Einstadter D, Groh-Wargo S, Collin M. Standardized slow enteral feeding protocol and the incidence of necrotizing enterocolitis in extremely low birth weight infants. JPEN J Parenter Enteral Nutr 2015; 39 (06) 644-654
• 4 Heida FH, Stolwijk L, Loos MH. , et al. Increased incidence of necrotizing enterocolitis in the Netherlands after implementation of the new Dutch guideline for active treatment in extremely preterm infants: Results from three academic referral centers. J Pediatr Surg 2017; 52 (02) 273-276
• 5 McElhinney DB, Hedrick HL, Bush DM. , et al. Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes. Pediatrics 2000; 106 (05) 1080-1087
• 6 Polin RA, Pollack PF, Barlow B. , et al. Necrotizing enterocolitis in term infants. J Pediatr 1976; 89 (03) 460-462
• 7 Frost BL, Modi BP, Jaksic T, Caplan MS. New medical and surgical insights into neonatal necrotizing enterocolitis: a review. JAMA Pediatr 2017; 171 (01) 83-88
• 8 Guthrie SO, Gordon PV, Thomas V, Thorp JA, Peabody J, Clark RH. Necrotizing enterocolitis among neonates in the United States. J Perinatol 2003; 23 (04) 278-285
• 9 Seeman SM, Mehal JM, Haberling DL, Holman RC, Stoll BJ. Infant and maternal risk factors related to necrotising enterocolitis-associated infant death in the United States. Acta Paediatr 2016; 105 (06) e240-e246
• 10 Kitsantas P, Kang E, Yang L. Root cause analysis and Bayesian networks of Down syndrome and necrotizing enterocolitis. Qual Manag Health Care 2007; 16 (04) 328-335
• 11 Boghossian NS, Hansen NI, Bell EF. , et al; Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network. Survival and morbidity outcomes for very low birth weight infants with Down syndrome. Pediatrics 2010; 126 (06) 1132-1140
• 12 Canfield MA, Honein MA, Yuskiv N. , et al. National estimates and race/ethnic-specific variation of selected birth defects in the United States, 1999-2001. Birth Defects Res A Clin Mol Teratol 2006; 76 (11) 747-756
• 13 Aynaci FM, Orhan F, Celep F, Karagüzel A. Frequency of cardiovascular and gastrointestinal malformations, leukemia and hypothyroidism in children with Down syndrome in Trabzon, Turkey. Turk J Pediatr 1998; 40 (01) 103-109
• 14 Weijerman ME, de Winter JP. Clinical practice. The care of children with Down syndrome. Eur J Pediatr 2010; 169 (12) 1445-1452
• 15 Zachor DA, Mroczek-Musulman E, Brown P. Prevalence of celiac disease in Down syndrome in the United States. J Pediatr Gastroenterol Nutr 2000; 31 (03) 275-279
• 16 Freeman SB, Taft LF, Dooley KJ. , et al. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet 1998; 80 (03) 213-217
• 17 Cua CL, Blankenship A, North AL, Hayes J, Nelin LD. Increased incidence of idiopathic persistent pulmonary hypertension in Down syndrome neonates. Pediatr Cardiol 2007; 28 (04) 250-254
• 18 Weijerman ME, van Furth AM, van der Mooren MD. , et al. Prevalence of congenital heart defects and persistent pulmonary hypertension of the neonate with Down syndrome. Eur J Pediatr 2010; 169 (10) 1195-1199
• 19 Cua CL, Rogers LK, Chicoine LG. , et al. Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine. Eur J Pediatr 2011; 170 (07) 859-863
• 20 Lambert DK, Christensen RD, Henry E. , et al. Necrotizing enterocolitis in term neonates: data from a multihospital health-care system. J Perinatol 2007; 27 (07) 437-443
• 21 Battersby C, Longford N, Costeloe K, Modi N. ; UK Neonatal Collaborative Necrotising Enterocolitis Study Group. Development of a gestational age-specific case definition for neonatal necrotizing enterocolitis. JAMA Pediatr 2017; 171 (03) 256-263
• 22 Carter BM, Holditch-Davis D. Risk factors for necrotizing enterocolitis in preterm infants: how race, gender, and health status contribute. Adv Neonatal Care 2008; 8 (05) 285-290
• 23 Kessler U, Schulte F, Cholewa D. , et al. Outcome in neonates with necrotizing enterocolitis and patent ductus arteriosus. World J Pediatr 2016; 12 (01) 55-59
• 24 Kort EJ. Patent ductus arteriosus in the preterm infant: an update on morbidity and mortality. Curr Pediatr Rev 2016; 12 (02) 98-105
• 25 Bain J, Benjamin Jr DK, Hornik CP, Benjamin DK, Clark R, Smith PB. Risk of necrotizing enterocolitis in very-low-birth-weight infants with isolated atrial and ventricular septal defects. J Perinatol 2014; 34 (04) 319-321
• 26 Freeman SB, Torfs CP, Romitti PA. , et al. Congenital gastrointestinal defects in Down syndrome: a report from the Atlanta and National Down Syndrome Projects. Clin Genet 2009; 75 (02) 180-184
• 27 Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int 2009; 25 (08) 727-730
• 28 Friedmacher F, Puri P. Hirschsprung's disease associated with Down syndrome: a meta-analysis of incidence, functional outcomes and mortality. Pediatr Surg Int 2013; 29 (09) 937-946
• 29 Källén B, Mastroiacovo P, Robert E. Major congenital malformations in Down syndrome. Am J Med Genet 1996; 65 (02) 160-166
• 30 Best KE, Addor MC, Arriola L. , et al. Hirschsprung's disease prevalence in Europe: a register based study. Birth Defects Res A Clin Mol Teratol 2014; 100 (09) 695-702
• 31 Kwendakwema N, Al-Dulaimi R, Presson AP. , et al. Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21. J Pediatr Surg 2016; 51 (12) 2001-2004
• 32 Knott EM, Gasior A, St Peter S, Snyder C. Mortality of necrotizing enterocolitis and isolated ileal perforation at a single institution over the past 40 years. Eur J Pediatr Surg 2014; 24 (05) 394-397
• 33 Neu J, Walker WA. Necrotizing enterocolitis. N Engl J Med 2011; 364 (03) 255-264