Advertisement

Meconium Ileus

  • Philip Corbett
  • Amulya SaxenaEmail author
Chapter

Abstract

Meconium ileus (MI) is neonatal intestinal obstruction caused by protein-rich, inspissated meconium in the terminal ileum. This viscid meconium adheres to the bowel wall, causing an intra-luminal obstruction (sometimes referred to as obturator-type obstruction). Its incidence is approximately 1:10,000 live births [1] within the white population but lower in other races. Historically, MI has been almost exclusively associated with cystic fibrosis (CF); however this dogma has been challenged by some published series that report 21–46% of their cases did not have CF [2, 3]. 10–20% of children born with CF present as MI [4, 5].

References

  1. 1.
    Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947–2003. Eur Respir J. 2007;29:522–6.CrossRefGoogle Scholar
  2. 2.
    Fakhoury K, Durie PR, Levinson H, Canny GJ. Meconium ileus in the absence of cystic fibrosis. Arch Dis Child. 1992;67:1204–6.CrossRefGoogle Scholar
  3. 3.
    Gorter RR, Karimi A, Sleebom C, Kneepkens CMF, Heij HA. Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50(5):569–72.PubMedGoogle Scholar
  4. 4.
    Donnison AB, Shwachman H, Gross RE. A review of 164 children with meconium ileus seen at the Children's Hospital Medical Center, Boston. Pediatrics. 1966;37(5):833–50.PubMedGoogle Scholar
  5. 5.
    Wilmott RW, Tyson SL, Dinwiddie R, Matthew DJ. Survival rates in cystic fibrosis. Arch Dis Child. 1983;58(10):835–6.CrossRefGoogle Scholar
  6. 6.
    Evans AK, Fitzgerald DA, Mc Kay KO. The impact of meconium ileus on the clinical course of children with cystic fibrosis. Eur Respir J. 2001;18:784–9.CrossRefGoogle Scholar
  7. 7.
    Lai HC, Kosorok MR, Laxova A, et al. Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening. Pediatrics. 2000;105:53–61.CrossRefGoogle Scholar
  8. 8.
    Munck A, Gerardin M, Alberti C, et al. Clinical outcome of cystic fibrosis presenting with or without meconium ileus: a matched cohort study. J Pediatr Surg. 2006;41:1556–60.CrossRefGoogle Scholar
  9. 9.
    Efrati O, Nir J, Fraser D, et al. Meconium ileus in patients with cystic fibrosis is not a risk factor for clinical deterioration and survival: the Israeli Multicenter Study. J Pediatr Gastroenterol Nutr. 2010;50:173–8.CrossRefGoogle Scholar
  10. 10.
    Committee on Genetics. ACOG Committee Opinion No. 486: update on carrier screening for cystic fibrosis. Obstet Gynecol. 2011;117:1028–31.CrossRefGoogle Scholar
  11. 11.
    Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245:1073–80.CrossRefGoogle Scholar
  12. 12.
    Chen EY, Yang N, Quinton PM, Chin WC. A new role for bicarbonate in mucus formation. Am J Phys Lung Cell Mol Phys. 2010;299(4):L542–9.Google Scholar
  13. 13.
    Garcia M, Abigail S, Ning Y, Quinton PM. Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator–dependent bicarbonate secretion. J Clin Invest. 2009;119(9):2613–22.CrossRefGoogle Scholar
  14. 14.
    Guilbault C, Saeed Z, Downey GP, et al. Cystic fibrosis mouse models. Am J Respir Cell Mol Biol. 2007;36:1–7.CrossRefGoogle Scholar
  15. 15.
    Cystic fibrosis mutation database. Cystic Fibrosis Centre at the hospital for sick children in Toronto http://www.genet.sickkids.on.ca/StatisticsPage.html.
  16. 16.
    Bobadilla JL, Macek M Jr, Fine JP, et al. Cystic fibrosis: a worldwide analysis of CFTR mutations—correlation with incidence data and application to screening. Hum Mutat. 2002;19:575–606.CrossRefGoogle Scholar
  17. 17.
    Cystic Fibrosis Foundation. Cystic fibrosis foundation patient registry annual data report 2010. Bethesda, MD: Cystic Fibrosis Foundation; 2010.Google Scholar
  18. 18.
    Blackman SM, Deering-Brose R, McWilliams R, et al. Relative contribution of genetic and non-genetic modifiers to intestinal obstruction in cystic fibrosis. Gastroenterology. 2006;131:1030–9.CrossRefGoogle Scholar
  19. 19.
    Guidelines for the performance of the sweat test for the investigation of cystic fibrosis in the UK v.2. Dr Sarah Heap. Royal College of Paediatrics and Child Health; 2014.Google Scholar
  20. 20.
    Scotet V, Dugueperoux I, Audrezet MP, et al. Focus on cystic fibrosis and other disorders evidenced in fetuses with sonographic finding of echogenic bowel: 16-year report from Brittany, France. Am J Obstet Gynecol. 2010;203:592.e1–6.CrossRefGoogle Scholar
  21. 21.
    Bahado-Singh R, Morotti R, Copel JA, et al. Hyperechoic fetal bowel: the perinatal consequences. Prenat Diagn. 1994;14:981–7.CrossRefGoogle Scholar
  22. 22.
    Lang I, Daneman A, Cutz E, Hagen P, Shandling B. Abdominal calcification in cystic fibrosis with meconium ileus: radiologic-pathologic correlation. Pediatr Radiol. 1997;27(6):523–7.CrossRefGoogle Scholar
  23. 23.
    Noblett HR. Treatment of uncomplicated meconium ileus by Gastrografin enema: a preliminary report. J Pediatr Surg. 1969;4(2):190–7.CrossRefGoogle Scholar
  24. 24.
    Boyd A, Carachi R, Azmy AF, Raine PA, Young DG. Gastrografin enema in meconium ileus: the persistent approach. Pediatr Surg Int. 1988;3(2-3):139–40.Google Scholar
  25. 25.
  26. 26.
    Caniano DA, Beaver BL. Meconium ileus: a fifteen-year experience with forty-two neonates. Surgery. 1987;102(4):699–703.PubMedGoogle Scholar
  27. 27.
    Copeland DR, Peter SD, Sharp SW, Islam S, Cuenca A, Tolleson JS, Dassinger MS, Little DC, Jackson RJ, Kokoska ER, Smith SD. Diminishing role of contrast enema in simple meconium ileus. J Pediatr Surg. 2009;44(11):2130–2.CrossRefGoogle Scholar
  28. 28.
    Kao SC, Franken EA. Nonoperative treatment of simple meconium ileus: a survey of the Society for Pediatric Radiology. Pediatr Radiol. 1995;25(2):97–100.CrossRefGoogle Scholar
  29. 29.
    Burke MS, Ragi JM, Karamanoukian HL, Kotter M, Brisseau GF, Borowitz DS, Ryan ME, Irish MS, Glick PL. New strategies in nonoperative management of meconium ileus. J Pediatr Surg. 2002;37(5):760–4.CrossRefGoogle Scholar
  30. 30.
    Lutzger LG, Factor SM. Effects of some water-soluble contrast media on the colonic mucosa 1. Radiology. 1976;118(3):545–8.CrossRefGoogle Scholar
  31. 31.
    Hiatt RB, Wilson PE. Celiac syndrome; therapy of meconium ileus, report of eight cases with a review of the literature. Surg Gynecol Obstet. 1948;87(3):317–27.PubMedGoogle Scholar
  32. 32.
    Rescorla FR, Grosfeld J. Meconium, ileus. Oper Pediatr Surg. 2013;1:433.Google Scholar
  33. 33.
    Rescorla FJ, Grosfeld JL. Contemporary management of meconium ileus. World J Surg. 1993;17(3):318–25.CrossRefGoogle Scholar
  34. 34.
    Kalayoglu M, Sieber WK, Rodnan JB, Kiesewetter WB. Meconium ileus: a critical review of treatment and eventual prognosis. J Pediatr Surg. 1971;6(3):290–300.CrossRefGoogle Scholar
  35. 35.
    Meeker IA. Acetylcysteine used to liquefy inspissated meconium causing intestinal obstruction in the newborn. Surgery. 1964;56:419–25.PubMedGoogle Scholar
  36. 36.
    Rubin BK. Mucolytics, expectorants, and mucokinetic medications. Respir Care. 2007;52(7):859–65.PubMedGoogle Scholar
  37. 37.
    Mushtaq I, Wright VM, Drake DP, Mearns MB, Wood CB. Meconium ileus secondary to cystic fibrosis The East London experience. Pediatr Surg Int. 1998;13(5):365–9.CrossRefGoogle Scholar
  38. 38.
    Steiner Z, Mogilner J, Siplovich L, Eldar S. T-tubes in the management of meconium ileus. Pediatr Surg Int. 1997;12(2):140–1.CrossRefGoogle Scholar
  39. 39.
    Gross RE. The surgery of infancy and childhood: its principles and techniques. Philadelphia: Saunders; 1953.Google Scholar
  40. 40.
    Bishop HC, Koop CE. Management of meconium ileus: resection, Roux-en-Y anastomosis and ileostomy irrigation with pancreatic enzymes. Ann Surg. 1957;145(3):410.CrossRefGoogle Scholar
  41. 41.
    Santulli TV, Blanc WA. Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg. 1961;154(6):939.PubMedPubMedCentralGoogle Scholar
  42. 42.
    D'Agostino S, Musi L, Fabbro MA, Belloli G. Uncomplicated meconium ileus. Pediatr Surg Int. 1995;10(5):329–31.Google Scholar
  43. 43.
    Nagar H. Meconium Ileus–is a single surgical procedure adequate? Asian J Surg. 2006;29(3):161–4.CrossRefGoogle Scholar
  44. 44.
    Karimi A, Gorter RR, Sleeboom C, Kneepkens CM, Heij HA. Issues in the management of simple and complex meconium ileus. Pediatr Surg Int. 2011;27(9):963–8.CrossRefGoogle Scholar
  45. 45.
    Sawyer SM, Taylor R, MacMahon R, Robertson CF. Meconium ileus in cystic fibrosis. Pediatr Surg Int. 1994;9(3):180–4.CrossRefGoogle Scholar
  46. 46.
    Del Pin CA, Czyrko C, Ziegler MM, Scanlin TF, Bishop HC. Management and survival of meconium ileus. A 30-year review. Ann Surg. 1992;215(2):179.CrossRefGoogle Scholar
  47. 47.
    Docherty JG, Zaki A, Coutts JA, Evans TJ, Carachi R. Meconium ileus: a review 1972–1990. Br J Surg. 1992;79(6):571–3.CrossRefGoogle Scholar
  48. 48.
    Caty MG, Escobar MA. Meconium disease. In:Ashcraft's pediatric surgery, vol. 33. Philadelphia: Saunders/Elsevier; 2010. p. 425–38.CrossRefGoogle Scholar
  49. 49.
    Jawaheer J, Khalil B, Plummer T, Bianchi A, Morecroft J, Rakoczy G, Bruce J, Bowen J, Morabito A. Primary resection and anastomosis for complicated meconium ileus: a safe procedure? Pediatr Surg Int. 2007;23(11):1091–3.CrossRefGoogle Scholar
  50. 50.
    British National Formulary. Royal Pharmaceutical Society of Great Britain, and RCPCH Publications Ltd; 2017.Google Scholar
  51. 51.
    Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988;41:583–91.CrossRefGoogle Scholar
  52. 52.
    Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530–5.CrossRefGoogle Scholar
  53. 53.
    Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, Spear SL, Michel SH, Parad RB, White TB, Farrell PM, Marshall BC. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(6):S73–93.CrossRefGoogle Scholar
  54. 54.
    Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002;35:246–59.CrossRefGoogle Scholar
  55. 55.
    Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman HG, Robberecht E, Döring G. Nutrition in patients with cystic fibrosis: a European consensus. J Cyst Fibros. 2002;1(2):51–75.CrossRefGoogle Scholar
  56. 56.
    Holliday KE, Allen JR, Waters DL, Gruca MA, Thompson SM, Gaskin KJ. Growth of human milk-fed and formula-fed infants with cystic fibrosis. J Pediatr. 1991;118(1):77–9.CrossRefGoogle Scholar
  57. 57.
    Walkowiak J, Nousia-Arvanitakis S, Henker J, Stern M, Sinaasappel M, Dodge JA. Indirect pancreatic function tests in children. J Pediatr Gastroenterol Nutr. 2005;40(2):107–14.CrossRefGoogle Scholar
  58. 58.
    Durie PR, Newth CJ, Forstner GG, Gall DG. Malabsorption of medium-chain triglycerides in infants with cystic fibrosis: correction with pancreatic enzyme supplement. J Pediatr. 1980;96:862–4.CrossRefGoogle Scholar
  59. 59.
    Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, on Growth CP, Ad Hoc Working Group. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832–9.CrossRefGoogle Scholar
  60. 60.
    Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy: consensus committee. J Pediatr. 1995;127:681–4.CrossRefGoogle Scholar
  61. 61.
    Smyth RL, van Velzen D, Smyth AR, Lloyd DA, Heaf DP. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet. 1994;343:85–6.CrossRefGoogle Scholar
  62. 62.
    Schwarzenberg SJ, Wielinski CL, Shamieh I, Carpenter BL, Jessurun J, Weisdorf SA, Warwick WJ, Sharp HL. Cystic fibrosis–associated colitis and fibrosing colonopathy. J Pediatr. 1995;127(4):565–70.CrossRefGoogle Scholar
  63. 63.
    FitzSimmons SC, Burkhart GA, Borowitz D, Grand RJ, Hammerstrom T, Durie PR, Lloyd-Still JD, Lowenfels AB. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med. 1997;336(18):1283–9.CrossRefGoogle Scholar
  64. 64.
    Ozcelik U, Goeman A, Niper N, Coskun T, Yilmaz E, Ozgue M. Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr. 1994;153:829–31.CrossRefGoogle Scholar
  65. 65.
    Heine RG, Button BM, Olinsky A, Phelan PD, Catto-Smith AG. Gastro-oesophageal reflux in infants under 6 months with cystic fibrosis. Arch Dis Child. 1998;78(1):44–8.CrossRefGoogle Scholar
  66. 66.
    Flume PA, Robinson KA, O'Sullivan BP, Finder JD, Vender RL, Willey-Courand DB, White TB, Marshall BC, Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009;54(4):522–37.PubMedGoogle Scholar
  67. 67.
    McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest J. 2006;129(1_suppl):250S–9S.CrossRefGoogle Scholar
  68. 68.
    Hardy KA, Schidlow DV, Wolfson MR, Shaffer TH. Mechanics and energetics of breathing in newly diagnosed infants with cystic fibrosis: effect of combined bronchodilator and chest physical therapy. Pediatr Pulmonol. 1989;6(2):103–8.CrossRefGoogle Scholar
  69. 69.
    Maayan CH, Bar-Yishay E, Yaacobi T, Marcus Y, Katznelson D, Yahav Y, Godfrey S. Immediate effect of various treatments on lung function in infants with cystic fibrosis. Respiration. 1989;55(3):144–51.CrossRefGoogle Scholar
  70. 70.
    Phillips GE, Pike SE, Rosenthal M, Bush A. Holding the baby: head downwards positioning for physiotherapy does not cause gastro-oesophageal reflux. Eur Respir J. 1998;12(4):954–7.CrossRefGoogle Scholar
  71. 71.
    Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis. Arch Dis Child. 1997;76(2):148–50.CrossRefGoogle Scholar
  72. 72.
    Colombo C, Ellemunter H, Houwen R, Munck A, Taylor C, Wilschanski M. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10:S24–8.CrossRefGoogle Scholar
  73. 73.
    El-Chammas KI, Rumman N, Goh VL, Quintero D, Goday PS. Rectal prolapse and cystic fibrosis. J Pediatr Gastroenterol Nutr. 2015;60(1):110–2.CrossRefGoogle Scholar
  74. 74.
    Curry MP, Hegarty JE. The gallbladder and biliary tract in cystic fibrosis. Curr Gastroenterol Rep. 2005;7(2):147–53.CrossRefGoogle Scholar
  75. 75.
    Ahmad A, Ahmed A, Patrizio P. Cystic fibrosis and fertility. Curr Opin Obstet Gynecol. 2013;25(3):167–72.CrossRefGoogle Scholar
  76. 76.
    Escobar MA, Grosfeld JL, Burdick JJ, Powell RL, Jay CL, Wait AD, West KW, Billmire DF, Scherer LR, Engum SA, Rouse TM. Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes. Surgery. 2005;138(4):560–72.CrossRefGoogle Scholar
  77. 77.
    Carlyle BE, Borowitz DS, Glick PL. A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg. 2012;47(4):772–81.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryChelsea Children’s Hospital, Chelsea and Westminster NHS Foundation Trust, Imperial College LondonLondonUK

Personalised recommendations