Spontaneous intestinal perforation of the newborn
- Eugene S Kim, MD
Eugene S Kim, MD
- Associate Professor of Surgery, Division of Pediatric Surgery
- University of Southern California Keck School of Medicine
- Mary L Brandt, MD
Mary L Brandt, MD
- Professor of Surgery, Division of Pediatric Surgery
- Baylor College of Medicine
Spontaneous intestinal perforation (SIP) of the newborn, also referred to as isolated perforation of the newborn, is a single intestinal perforation that is typically found at the terminal ileum [1-5]. SIP occurs primarily in premature infants with very low birth weight (VLBW, birth weight <1500 g) and extremely low birth weight (ELBW, birth weight <1000 g). SIP is a separate clinical entity from necrotizing enterocolitis, the most severe gastrointestinal complication of premature infants. This differentiation is important because of management considerations. (See "Clinical features and diagnosis of necrotizing enterocolitis in newborns".)
The epidemiology, clinical features, diagnosis, and management of spontaneous intestinal perforation of the newborn will be reviewed here.
Spontaneous intestinal perforation (SIP) is most commonly found in very low birth weight (VLBW) and extremely low birth weight (ELBW) premature infants. The risk appears to be about 2 to 3 percent in VLBW infants [1,6,7] and about 5 percent in ELBW infants . The median gestational age varies from 25 to 27 weeks, and median birth weight from 670 to 973 g [1-3,9,10]. SIP occurs more frequently in male infants (ranging from 65 to 71 percent of affected infants) .
Risk factors — Prematurity is the only well established risk factor for SIP. Several reported antenatal and postnatal risk factors are based upon limited data that include findings from case series and a single data set from the Pediatrix Medical Group [1,11-13]. This data set is based upon clinical progress notes of premature infants (range of gestational age 23 to 34 weeks) who were born and cared for at 98 sites in the Pediatrix Medical Group between 1998 and 2000 .
Antenatal — Severe placental chorioamnionitis appears to be an antenatal risk factor for SIP. In one case-control study of 16 premature infants with SIP, infants with SIP versus matched controls were more likely to have severe placental chorioamnionitis with evidence of fetal vascular response based upon a blinded retrospective histologic examination of the placenta and umbilical vessels (40 versus 12 percent) . In this study, mothers of infants with SIP were more likely to have received antibiotics before or at delivery (93 versus 57 percent).
- Meyer CL, Payne NR, Roback SA. Spontaneous, isolated intestinal perforations in neonates with birth weight less than 1,000 g not associated with necrotizing enterocolitis. J Pediatr Surg 1991; 26:714.
- Holland AJ, Shun A, Martin HC, et al. Small bowel perforation in the premature neonate: congenital or acquired? Pediatr Surg Int 2003; 19:489.
- Drewett MS, Burge DM. Recurrent neonatal gastro-intestinal problems after spontaneous intestinal perforation. Pediatr Surg Int 2007; 23:1081.
- Pumberger W, Mayr M, Kohlhauser C, Weninger M. Spontaneous localized intestinal perforation in very-low-birth-weight infants: a distinct clinical entity different from necrotizing enterocolitis. J Am Coll Surg 2002; 195:796.
- Uceda JE, Laos CA, Kolni HW, Klein AM. Intestinal perforations in infants with a very low birth weight: a disease of increasing survival? J Pediatr Surg 1995; 30:1314.
- Alpan G, Eyal F, Vinograd I, et al. Localized intestinal perforations after enteral administration of indomethacin in premature infants. J Pediatr 1985; 106:277.
- Kawase Y, Ishii T, Arai H, Uga N. Gastrointestinal perforation in very low-birthweight infants. Pediatr Int 2006; 48:599.
- Blakely ML, Tyson JE, Lally KP, et al. Laparotomy versus peritoneal drainage for necrotizing enterocolitis or isolated intestinal perforation in extremely low birth weight infants: outcomes through 18 months adjusted age. Pediatrics 2006; 117:e680.
- Attridge JT, Herman AC, Gurka MJ, et al. Discharge outcomes of extremely low birth weight infants with spontaneous intestinal perforations. J Perinatol 2006; 26:49.
- Resch B, Mayr J, Kuttnig-Haim M, et al. Spontaneous gastrointestinal perforation in very-low-birth-weight infants--a rare complication in a neonatal intensive care unit. Pediatr Surg Int 1998; 13:165.
- Ragouilliaux CJ, Keeney SE, Hawkins HK, Rowen JL. Maternal factors in extremely low birth weight infants who develop spontaneous intestinal perforation. Pediatrics 2007; 120:e1458.
- Attridge JT, Clark R, Walker MW, Gordon PV. New insights into spontaneous intestinal perforation using a national data set: (1) SIP is associated with early indomethacin exposure. J Perinatol 2006; 26:93.
- Attridge JT, Clark R, Gordon PV. New insights into spontaneous intestinal perforation using a national data set (3): antenatal steroids have no adverse association with spontaneous intestinal perforation. J Perinatol 2006; 26:667.
- Guthrie SO, Gordon PV, Thomas V, et al. Necrotizing enterocolitis among neonates in the United States. J Perinatol 2003; 23:278.
- Wadhawan R, Oh W, Vohr BR, et al. Spontaneous intestinal perforation in extremely low birth weight infants: association with indometacin therapy and effects on neurodevelopmental outcomes at 18-22 months corrected age. Arch Dis Child Fetal Neonatal Ed 2013; 98:F127.
- Rattray BN, Kraus DM, Drinker LR, et al. Antenatal magnesium sulfate and spontaneous intestinal perforation in infants less than 25 weeks gestation. J Perinatol 2014; 34:819.
- Nakajima Y, Masaoka N, Yamamoto T. Obstetrical risk factors for focal intestinal perforation in very low birth weight infants. J Perinat Med 2011; 39:179.
- Gordon PV, Young ML, Marshall DD. Focal small bowel perforation: an adverse effect of early postnatal dexamethasone therapy in extremely low birth weight infants. J Perinatol 2001; 21:156.
- Stark AR, Carlo WA, Tyson JE, et al. Adverse effects of early dexamethasone in extremely-low-birth-weight infants. National Institute of Child Health and Human Development Neonatal Research Network. N Engl J Med 2001; 344:95.
- Gordon P, Rutledge J, Sawin R, et al. Early postnatal dexamethasone increases the risk of focal small bowel perforation in extremely low birth weight infants. J Perinatol 1999; 19:573.
- Kelleher J, Salas AA, Bhat R, et al. Prophylactic indomethacin and intestinal perforation in extremely low birth weight infants. Pediatrics 2014; 134:e1369.
- Schmidt B, Davis P, Moddemann D, et al. Long-term effects of indomethacin prophylaxis in extremely-low-birth-weight infants. N Engl J Med 2001; 344:1966.
- Paquette L, Friedlich P, Ramanathan R, Seri I. Concurrent use of indomethacin and dexamethasone increases the risk of spontaneous intestinal perforation in very low birth weight neonates. J Perinatol 2006; 26:486.
- Holland AJ. Comment on Kubota et al.: focal intestinal perforation in extremely-low-birth-weight neonates: etiological consideration from histological findings. Pediatr Surg Int 2008; 24:387.
- Aschner JL, Deluga KS, Metlay LA, et al. Spontaneous focal gastrointestinal perforation in very low birth weight infants. J Pediatr 1988; 113:364.
- Gordon PV. Understanding intestinal vulnerability to perforation in the extremely low birth weight infant. Pediatr Res 2009; 65:138.
- Lai S, Yu W, Wallace L, Sigalet D. Intestinal muscularis propria increases in thickness with corrected gestational age and is focally attenuated in patients with isolated intestinal perforations. J Pediatr Surg 2014; 49:114.
- Gordon PV, Herman AC, Marcinkiewicz M, et al. A neonatal mouse model of intestinal perforation: investigating the harmful synergism between glucocorticoids and indomethacin. J Pediatr Gastroenterol Nutr 2007; 45:509.
- Chan KY, Leung FW, Lam HS, et al. Immunoregulatory protein profiles of necrotizing enterocolitis versus spontaneous intestinal perforation in preterm infants. PLoS One 2012; 7:e36977.
- Chan KY, Leung KT, Tam YH, et al. Genome-wide expression profiles of necrotizing enterocolitis versus spontaneous intestinal perforation in human intestinal tissues: dysregulation of functional pathways. Ann Surg 2014; 260:1128.
- Adesanya OA, O'Shea TM, Turner CS, et al. Intestinal perforation in very low birth weight infants: growth and neurodevelopment at 1 year of age. J Perinatol 2005; 25:583.
- Adderson EE, Pappin A, Pavia AT. Spontaneous intestinal perforation in premature infants: a distinct clinical entity associated with systemic candidiasis. J Pediatr Surg 1998; 33:1463.
- Attridge JT, Clark R, Walker MW, Gordon PV. New insights into spontaneous intestinal perforation using a national data set: (2) two populations of patients with perforations. J Perinatol 2006; 26:185.
- Harms K, Lüdtke FE, Lepsien G, Speer CP. Idiopathic intestinal perforations in premature infants without evidence of necrotizing enterocolitis. Eur J Pediatr Surg 1995; 5:30.
- Fischer A, Vachon L, Durand M, Cayabyab RG. Ultrasound to diagnose spontaneous intestinal perforation in infants weighing ⩽1000 g at birth. J Perinatol 2015; 35:104.
- Baird R, Puligandla PS, St Vil D, et al. The role of laparotomy for intestinal perforation in very low birth weight infants. J Pediatr Surg 2006; 41:1522.
- Blakely ML, Gupta H, Lally KP. Surgical management of necrotizing enterocolitis and isolated intestinal perforation in premature neonates. Semin Perinatol 2008; 32:122.
- Cass DL, Brandt ML, Patel DL, et al. Peritoneal drainage as definitive treatment for neonates with isolated intestinal perforation. J Pediatr Surg 2000; 35:1531.
- Chiu B, Pillai SB, Almond PS, et al. To drain or not to drain: a single institution experience with neonatal intestinal perforation. J Perinat Med 2006; 34:338.
- Rao SC, Basani L, Simmer K, et al. Peritoneal drainage versus laparotomy as initial surgical treatment for perforated necrotizing enterocolitis or spontaneous intestinal perforation in preterm low birth weight infants. Cochrane Database Syst Rev 2011; :CD006182.
- Zamir O, Goldberg M, Udassin R, et al. Idiopathic gastrointestinal perforation in the neonate. J Pediatr Surg 1988; 23:335.
- Wadhawan R, Oh W, Hintz SR, et al. Neurodevelopmental outcomes of extremely low birth weight infants with spontaneous intestinal perforation or surgical necrotizing enterocolitis. J Perinatol 2014; 34:64.
- Fisher JG, Jones BA, Gutierrez IM, et al. Mortality associated with laparotomy-confirmed neonatal spontaneous intestinal perforation: a prospective 5-year multicenter analysis. J Pediatr Surg 2014; 49:1215.
- Roze E, Ta BD, van der Ree MH, et al. Functional impairments at school age of children with necrotizing enterocolitis or spontaneous intestinal perforation. Pediatr Res 2011; 70:619.
- Shah TA, Meinzen-Derr J, Gratton T, et al. Hospital and neurodevelopmental outcomes of extremely low-birth-weight infants with necrotizing enterocolitis and spontaneous intestinal perforation. J Perinatol 2012; 32:552.
- Risk factors
- - Antenatal
- - Postnatal
- Exposure to glucocorticoids
- Exposure to indomethacin
- PATHOLOGY AND PATHOGENESIS
- CLINICAL MANIFESTATIONS
- Age at presentation
- Physical findings
- Associated infections
- Laboratory tests
- Surgical treatment
- - PPD technique
- SUMMARY AND RECOMMENDATIONS