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Meconium Ileus

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Neonatal Surgery
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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].

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References

  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.

    Article  CAS  PubMed  Google Scholar 

  2. Fakhoury K, Durie PR, Levinson H, Canny GJ. Meconium ileus in the absence of cystic fibrosis. Arch Dis Child. 1992;67:1204–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  5. Wilmott RW, Tyson SL, Dinwiddie R, Matthew DJ. Survival rates in cystic fibrosis. Arch Dis Child. 1983;58(10):835–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  10. Committee on Genetics. ACOG Committee Opinion No. 486: update on carrier screening for cystic fibrosis. Obstet Gynecol. 2011;117:1028–31.

    Article  Google Scholar 

  11. Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989;245:1073–80.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Guilbault C, Saeed Z, Downey GP, et al. Cystic fibrosis mouse models. Am J Respir Cell Mol Biol. 2007;36:1–7.

    Article  CAS  PubMed  Google Scholar 

  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. 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.

    Article  CAS  PubMed  Google Scholar 

  17. Cystic Fibrosis Foundation. Cystic fibrosis foundation patient registry annual data report 2010. Bethesda, MD: Cystic Fibrosis Foundation; 2010.

    Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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. 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.

    Article  Google Scholar 

  21. Bahado-Singh R, Morotti R, Copel JA, et al. Hyperechoic fetal bowel: the perinatal consequences. Prenat Diagn. 1994;14:981–7.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  23. Noblett HR. Treatment of uncomplicated meconium ileus by Gastrografin enema: a preliminary report. J Pediatr Surg. 1969;4(2):190–7.

    Article  CAS  PubMed  Google Scholar 

  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. http://www.mhra.gov.uk/home/groups/spcpil/documents/spcpil/con1484285048304.pdf.

  26. Caniano DA, Beaver BL. Meconium ileus: a fifteen-year experience with forty-two neonates. Surgery. 1987;102(4):699–703.

    CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  30. Lutzger LG, Factor SM. Effects of some water-soluble contrast media on the colonic mucosa 1. Radiology. 1976;118(3):545–8.

    Article  CAS  PubMed  Google Scholar 

  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.

    CAS  PubMed  Google Scholar 

  32. Rescorla FR, Grosfeld J. Meconium, ileus. Oper Pediatr Surg. 2013;1:433.

    Google Scholar 

  33. Rescorla FJ, Grosfeld JL. Contemporary management of meconium ileus. World J Surg. 1993;17(3):318–25.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  35. Meeker IA. Acetylcysteine used to liquefy inspissated meconium causing intestinal obstruction in the newborn. Surgery. 1964;56:419–25.

    PubMed  Google Scholar 

  36. Rubin BK. Mucolytics, expectorants, and mucokinetic medications. Respir Care. 2007;52(7):859–65.

    PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  39. Gross RE. The surgery of infancy and childhood: its principles and techniques. Philadelphia: Saunders; 1953.

    Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Santulli TV, Blanc WA. Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg. 1961;154(6):939.

    CAS  PubMed  PubMed Central  Google Scholar 

  42. D'Agostino S, Musi L, Fabbro MA, Belloli G. Uncomplicated meconium ileus. Pediatr Surg Int. 1995;10(5):329–31.

    Google Scholar 

  43. Nagar H. Meconium Ileus–is a single surgical procedure adequate? Asian J Surg. 2006;29(3):161–4.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Sawyer SM, Taylor R, MacMahon R, Robertson CF. Meconium ileus in cystic fibrosis. Pediatr Surg Int. 1994;9(3):180–4.

    Article  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  48. Caty MG, Escobar MA. Meconium disease. In:Ashcraft's pediatric surgery, vol. 33. Philadelphia: Saunders/Elsevier; 2010. p. 425–38.

    Chapter  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  50. British National Formulary. Royal Pharmaceutical Society of Great Britain, and RCPCH Publications Ltd; 2017.

    Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    PubMed  Google Scholar 

  67. McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest J. 2006;129(1_suppl):250S–9S.

    Article  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  74. Curry MP, Hegarty JE. The gallbladder and biliary tract in cystic fibrosis. Curr Gastroenterol Rep. 2005;7(2):147–53.

    Article  PubMed  Google Scholar 

  75. Ahmad A, Ahmed A, Patrizio P. Cystic fibrosis and fertility. Curr Opin Obstet Gynecol. 2013;25(3):167–72.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

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Corbett, P., Saxena, A. (2019). Meconium Ileus. In: Lima, M., Reinberg, O. (eds) Neonatal Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-93534-8_19

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