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Bowel Atresia and Stenosis

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Neonatal Surgery

Abstract

Congenital anomalies of the bowel can affect any portion of the gastrointestinal tract. Bowel atresia and stenosis concern abnormal closure, discontinuity or narrowing from the duodenum to sigmoid colon, and the incidence is about 2/10,000 live births. The pathogenesis was previously believed to be an embryologic trouble of bowel recanalization or a disruption compromising small bowel vascular supply, but new genetic hypothesis emerges since a few years to explain the occurrence of bowel atresia and stenosis.

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References

  1. Hemming V, Rankin J. Small intestinal atresia in a defined population: occurrence, prenatal diagnosis and survival. Prenat Diagn. 2007;27:1205–11.

    Article  PubMed  Google Scholar 

  2. Nichol PF, Reeder A, Botham R. Humans, mice, and mechanisms of intestinal atresias: a window into understanding early intestinal development. J Gastrointest Surg. 2011;15:694–700.

    Article  PubMed  Google Scholar 

  3. Best KE, Tennant PWG, Addor MC, et al. Epidemiology of small intestinal atresia in Europe: a register-based study. Arch Dis Child Fetal Neonatal Ed. 2012;97:F353–8.

    Article  PubMed  Google Scholar 

  4. Tandler J. Zur Entwicklungsgeschichte des Menschlichen Duodenum in Frühen Embryonal Stadien. Gegenbaurs Morphol Jahrb. 1900;29:187–216.

    Google Scholar 

  5. Santulli TV, Blanc WA. Congenital atresia of the intestine: pathogenesis and treatment. Ann Surg. 1961;154:939–48.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Clogg HS. Congenital intestinal atresia. Lancet. 1904;164:P1770–4.

    Article  Google Scholar 

  7. Louw JH, Barnard CN. Congenital intestinal atresia: observations on its origin. Lancet. 1955;2:1065–7.

    Article  Google Scholar 

  8. Baglaj M, Carachi R, Lawther S. Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg. 2008;18:13–8.

    Article  CAS  PubMed  Google Scholar 

  9. Pavri DR, Marshall DG, Armstrong RF, et al. Intrauterine intussusception: case report and literature review. Can J Surg. 1983;26:376–8.

    CAS  PubMed  Google Scholar 

  10. Romeo C, Arena F, Impellizzeri G. Intrauterine intussusception, rare cause of intestinal atresia: case report. It J Ped Surg Sci. 1994;8:157–60.

    Google Scholar 

  11. Kilic N, Kiristioglu I, Kirkpinar A, et al. A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel's diverticulum. Acta Paediatr. 2003;92:756–7.

    Article  CAS  PubMed  Google Scholar 

  12. Pueyo C, Maldonado J, Royo Y, et al. Intrauterine intussusception: a rare cause of intestinal atresia. J Pediatr Surg. 2009;44:2028–30.

    Article  PubMed  Google Scholar 

  13. Lewis MP, Emberton M, Owen ER, et al. Delayed presentation of intestinal atresia and intussusception. a case report and literature review. Eur J Pediatr Surg. 1993;3:296–8.

    Article  CAS  PubMed  Google Scholar 

  14. Chiari H. Über eine intrauterin entstandene und von Darmatresie gefolgte Intussusception des lleums. Prag Med Wschr. 1888;13:399–401.

    Google Scholar 

  15. Guttman FM, Braun P, Garance PH, et al. Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg. 1973;8:633–40.

    Article  CAS  PubMed  Google Scholar 

  16. Puri P, Fujimoto T. New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg. 1988;23:221–5.

    Article  CAS  PubMed  Google Scholar 

  17. Cole C, Freitas A, Clifton MS, et al. Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 2010;45:E21–4.

    PubMed  Google Scholar 

  18. Bilodeau A, Prasil P, Cloutier R, et al. Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg. 2004;39:726–30.

    Article  PubMed  Google Scholar 

  19. Robinson I, Gill H, Ng LY, et al. Familial distal foregut atresia in a family with likely autosomal dominant inheritance pattern. Pediatr Surg Int. 2012;28:1151–5.

    Article  PubMed  Google Scholar 

  20. Fourcade L, Shima H, Miyazaki E, et al. Multiple gastrointestinal atresias result from disturbed morphogenesis. Pediatr Surg Int. 2001;17:361–4.

    Article  CAS  PubMed  Google Scholar 

  21. Cheng W, Tam PK. Murine duodenum does not go through a “solid core” stage in its embryological development. Eur J Pediatr Surg. 1998;8:212–5.

    Article  CAS  PubMed  Google Scholar 

  22. Ornitz DM, Itoh N. Fibroblast growth factors. Genome Biol. 2001;2:1–9.

    Article  Google Scholar 

  23. Fairbanks TJ, Sala FG, Kanard R, et al. The fibroblast growth factor pathway serves a regulatory role in proliferation and apoptosis in the pathogenesis of intestinal atresia. J Pediatr Surg. 2006;41:132–6.

    Article  PubMed  Google Scholar 

  24. Ramalho-Santos M, Melton DA, McMahon AP. Hedgehog signals regulate multiple aspects of gastrointestinal development. Development. 2000;127:2763–72.

    CAS  PubMed  Google Scholar 

  25. Gao N, White P, Kaestner KH. Establishment of intestinal identity and epithelial-mesenchymal signaling by Cdx2. Dev Cell. 2009;16:588–99.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Johnson SM, Meyers RL. Inherited thrombophilia: a possible cause of in utero vascular thrombosis in children with intestinal atresia. J Pediatr Surg. 2001;36:1146–9.

    Article  CAS  PubMed  Google Scholar 

  27. Gupta T, Yang W, Iovannisci DM, et al. Considering the vascular hypothesis for the pathogenesis of small intestinal atresia: a case control study of genetic factors. Am J Med Genet A. 2013;161:702–10.

    Article  CAS  Google Scholar 

  28. Werler MM, Sheehan JE, Mitchell AA. Maternal medication use and risks of gastroschisis and small intestinal atresia. Am J Epidemiol. 2002;155:26–31.

    Article  PubMed  Google Scholar 

  29. Milla PJ. Feeding, tasting, and sucking. In: Walker-Smith WA, Watkins JB, editors. Pediatric gastrointestinal disease. Philadelphia: BC Decker; 1991. p. 217–23.

    Google Scholar 

  30. Gross RE. The surgery of infancy and childhood. Philadelphia: WB Saunders Co; 1953. p. 150–66.

    Google Scholar 

  31. Gray SW, Skandalakis JE. Embryology for surgeons. Philadelphia, PA: WB Saunders Co; 1972. p. 147–8.

    Google Scholar 

  32. Grosfeld JL, Ballantine TVN, Shoemaker R. Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg. 1979;14:368–75.

    Article  CAS  PubMed  Google Scholar 

  33. Escobar MA, Ladd AP, Grosfeld JL, et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg. 2004;39:867–71.

    Article  PubMed  Google Scholar 

  34. Dalla Vecchia LK, Grosfeld JL, West KW, et al. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 1998;133:490–7.

    Article  CAS  PubMed  Google Scholar 

  35. Nusinovich Y, Revenis M, Torres C. Long-term outcomes for infants with intestinal atresia studied at Children’s National Medical Center. J Pediatr Gastroenterol Nutr. 2013;57:324–9.

    Article  PubMed  Google Scholar 

  36. St. Peter SD, Little DC, Barsness KA, et al. Should we be concerned about jejunoileal atresia during repair of duodenal atresia? J Laparoendosc Adv Surg Tech A. 2010;20:773–5.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Kimura K. Diamond-shaped anastomosis for duodenal atresia: an experience with 44 patients over 15 years. J Pediatr Surg. 1990;25:977–8.

    Article  CAS  PubMed  Google Scholar 

  38. Bax NMA, Ure BM, Van der Zee DC, et al. Laparoscopic duodenoduodenostomy for duodenal atresia. Surg Endosc. 2001;15:217.

    Article  CAS  PubMed  Google Scholar 

  39. Kay S, Yoder S, Rothenberg S. Laparoscopic duodenoduodenostomy in the neonate. J Pediatr Surg. 2009;44:906–8.

    Article  PubMed  Google Scholar 

  40. Mentessidou A, Saxena AK. Laparoscopic repair of duodenal atresia: systematic review and meta-analysis. World J Surg. 2017;41:2178–84.

    Article  PubMed  Google Scholar 

  41. Chiarenza SF, Bucci V, Conighi ML, et al. Duodenal atresia: open versus MIS repair-analysis of our experience over the last 12 years. Biomed Res Int. 2017;2017:7.

    Article  Google Scholar 

  42. Van der Zee DC. Laparoscopic repair of duodenal atresia: revisited. World J Surg. 2011;35:1781–4.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Valusek PA, Spilde TL, Tsao K, et al. Laparoscopic duodenal atresia repair using surgical U-clips: a novel technique. Surg Endosc. 2007;21:1023–4.

    Article  PubMed  Google Scholar 

  44. Parmentier B, Peycelon M, Muller CO, et al. Laparoscopic management of congenital duodenal atresia or stenosis: a single center early experience. J Pediatr Surg. 2015;50:1833–6.

    Article  PubMed  Google Scholar 

  45. Spilde TL, St. Peter SD, Keckler SJ, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Pediatr Surg. 2008;43:1002–5.

    Article  PubMed  Google Scholar 

  46. Hill S, Koontz CS, Langness SM, et al. Laparoscopic versus open repair of congenital duodenal obstruction in infant. J Laparoendosc Adv Surg Tech A. 2011;21:961–3.

    Article  PubMed  Google Scholar 

  47. Jensen AR, Short SS, Anselmo DM, et al. Laparoscopic versus open treatment of congenital duodenal obstruction: multicenter short-term outcomes analysis. J Laparoendosc Adv Surg Tech A. 2013;23:876–80.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Okamatsu T, Arai K, Yatsuzuka M, et al. Endoscopic membranectomy for congenital duodenal stenosis in an infant. J Pediatr Surg. 1989;24:367–8.

    Article  CAS  PubMed  Google Scholar 

  49. Ziegler K, Schier F, Waldschmidt J. Endoscopic laser resection of a duodenal membrane. J Pediatr Surg. 1992;27:1582–3.

    Article  CAS  PubMed  Google Scholar 

  50. Van Rijn RR, Van Lienden KP, Fortuna TL, et al. Membranous duodenal stenosis: initial experience with balloon dilatation in four children. Eur J Radiol. 2006;59:29–32.

    Article  PubMed  Google Scholar 

  51. Torroni F, De Angelis P, Caldaro T, et al. Endoscopic membranectomy of duodenal diaphragm: pediatric experience. Gastrointest Endosc. 2006;63:530–1.

    Article  PubMed  Google Scholar 

  52. Barabino A, Gandullia P, Arrigo S, et al. Successful endoscopic treatment of a double duodenal web in an infant. Gastrointest Endosc. 2011;73:401–2.

    Article  PubMed  Google Scholar 

  53. Bittencourt PFS, Malheiros RS, Ferreira AR, et al. Endoscopic treatment of congenital duodenal membrane. Gastrointest Endosc. 2012;76:1273–5.

    Article  PubMed  Google Scholar 

  54. Di Maio CJ, Kamal N, Hogan CM, et al. Pediatric therapeutic endoscopy: endoscopic management of a congenital duodenal web. Gastrointest Endosc. 2014;80:166–7.

    Article  Google Scholar 

  55. Bleve C, Costa L, Bertoncello V, et al. Endoscopic resection of a duodenal web in an 11-month-old infant with multiple malformations. Endoscopy. 2015;47:E210–1.

    Article  PubMed  Google Scholar 

  56. Huang MH, Bian HQ, Liang C, et al. Gastroscopic treatment of membranous duodenal stenosis in infants and children: report of 6 cases. J Pediatr Surg. 2015;50:413–6.

    Article  PubMed  Google Scholar 

  57. Poddar U, Jain V, Yachha SK, et al. Congenital duodenal web: successful management with endoscopic dilatation. Endosc Int Open. 2016;4:E238–41.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Virgone C, D’Antonio F, Khalil A, et al. Accuracy of prenatal ultrasound in detecting jejunal and ileal atresia: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2015;45:523–9.

    Article  CAS  PubMed  Google Scholar 

  59. John R, D’Antonio F, Khalil A, et al. Diagnostic accuracy of prenatal ultrasound in identifying jejunal and ileal atresia. Fetal Diagn Ther. 2015;38:142–6.

    Article  PubMed  Google Scholar 

  60. Goruppi I, Arevalo S, Gander R, et al. Role of intraluminal bowel echogenicity on prenatal ultrasounds to determine the anatomical level of intestinal atresia. J Matern Fetal Neonatal Med. 2017;30:103–8.

    Article  CAS  PubMed  Google Scholar 

  61. Sammour RN, Leibovitz Z, Degani S, et al. Prenatal diagnosis of small-bowel volvulus using 3-dimensional doppler sonography. J Ultrasound Med. 2008;27:1655–61.

    Article  PubMed  Google Scholar 

  62. Ozguner IF, Savas C, Ozguner M, et al. Intestinal atresia with segmental musculature and neural defect. J Pediatr Surg. 2005;40:1232–7.

    Article  PubMed  Google Scholar 

  63. Gfroerer S, Metzger R, Fiegel H, et al. Changes of smooth muscle contractile filaments in small bowel atresia. World J Gastroenterol. 2010;16:5716–21.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Wang X, Yuan C, Xiang L, et al. The clinical significance of pathological studies of congenital intestinal atresia. J Pediatr Surg. 2013;48:2084–91.

    Article  PubMed  Google Scholar 

  65. Louw JH. Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery. 1967;62:940–50.

    CAS  PubMed  Google Scholar 

  66. Martin LW, Zerella JT. Jejunoileal atresia: a proposed classification. J Pediatr Surg. 1976;11:399–403.

    Article  CAS  PubMed  Google Scholar 

  67. Stollman TH, de Blaauw I, Wijnen MHWA, et al. Decreased mortality but increased morbidity in neonates with jejunoileal atresia; a study of 114 cases over a 34-year period. J Pediatr Surg. 2009;44:217–21.

    Article  PubMed  Google Scholar 

  68. Tsuji Y, Maeda K, Ono S, et al. A new paradigm of scarless abdominal surgery in children: transumbilical minimal incision surgery. J Pediatr Surg. 2014;49:1605–9.

    Article  PubMed  Google Scholar 

  69. Sawai T, Yonekura T, Yamauchi K, et al. A novel approach to neonatal abdominal surgery via a circular incision around the umbilical cord. Pediatr Surg Int. 2016;32:1009–11.

    Article  PubMed  Google Scholar 

  70. Lima M, Ruggieri G, Domini M, et al. Evolution of the surgical management of bowel atresia in newborn: laparoscopically assisted treatment. Pediatr Med Chir. 2009;31:215–9.

    CAS  PubMed  Google Scholar 

  71. Abhyankar A, Mukhtar Z. Laparoscopy-assisted surgery for neonatal intestinal atresia: single-center experience. Asian J Endosc Surg. 2011;4:90–3.

    Article  CAS  PubMed  Google Scholar 

  72. Li B, Chen WB. Laparoscopy-assisted surgery for neonatal intestinal atresia and stenosis: a report of 35 cases. Pediatr Surg Int. 2012;28:1225–8.

    Article  CAS  PubMed  Google Scholar 

  73. Fockens P. Ein operativ geheilter Fall von Kongenitaler Dünndarmatresie. Zentralbl Chir. 1911;38:532–5.

    Google Scholar 

  74. von Mikulicz J. Chirurgische Erfahrungen über das Darmcarcinom. Arch Klin Chir. 1903;69:28–47.

    Google Scholar 

  75. Willital GH. Atlas der Kinderchirurgie. Indikationen und Operationstechnik. Stuttgart: Schattauer Verlag; 1981. p. 115.

    Google Scholar 

  76. Bishop HC, Koop CE. Management of meconium ileus: resection, Roux-en-y anastomosis and ileostomy irrigation with pancreatic enzymes. Ann Surg. 1957;145:410–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Kumaran N, Shankar KR, Lloyd DA, et al. Trends in the management and outcome of jejuno-ileal atresia. Eur J Pediatr Surg. 2002;12:163–7.

    Article  CAS  PubMed  Google Scholar 

  78. Wit J, Sellin S, Degenhardt P, et al. Is the Bishop-Koop anastomosis in treatment of neonatal ileus still current? Chirurg. 2000;71:307–10.

    Article  CAS  PubMed  Google Scholar 

  79. Levy E, Palmer DL, Frileux P, et al. Inhibition of upper gastrointestinal secretions by reinfusion of succus entericus into the distal small bowel. A clinical study of 30 patients with peritonitis and temporary enterostomy. Ann Surg. 1983;198:596–600.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Richardson L, Banerjee S, Rabe H. What is the evidence on the practice of mucous fistula refeeding in neonates with short bowel syndrome? J Pediatr Gastroenterol Nutr. 2006;43:267–70.

    Article  PubMed  Google Scholar 

  81. Wong KKY, Lan LCL, Lin SCL, et al. Mucous fistula refeeding in premature neonates with enterostomies. J Pediatr Gastroenterol Nutr. 2004;39:43–5.

    Article  PubMed  Google Scholar 

  82. Sato S, Nishijima E, Muraji T, et al. Jejunoileal atresia: a 27-year experience. J Pediatr Surg. 1998;33:1633–5.

    Article  CAS  PubMed  Google Scholar 

  83. Wang J, Du L, Cai W, et al. Prolonged feeding difficulties after surgical correction of intestinal atresia: a 13-year experience. J Pediatr Surg. 2014;49:1593–7.

    Article  PubMed  Google Scholar 

  84. Webb CH, Wangsteen OH. Congenital intestinal atresia. Am J Dis Child. 1931;41:262–84.

    Google Scholar 

  85. England RJ, Scammel S, Murthy GV. Proximal colonic atresia: is right hemicolectomy inevitable? Pediatr Surg Int. 2011;27:1059–62.

    Article  PubMed  Google Scholar 

  86. Etensel B, Temir G, Karkiner A, et al. Atresia of colon. J Pediatr Surg. 2005;40:1258–68.

    Article  PubMed  Google Scholar 

  87. Haxhija EQ, Schalamon J, Höllwarth ME. Management of isolated and associated colonic atresia. Pediatr Surg Int. 2011;27:411–6.

    Article  PubMed  Google Scholar 

  88. Mirza B, Iqbal S, Ijaz L. Colonic atresia and stenosis: our experience. J Neonatal Surg. 2012;1(1):4.

    PubMed  PubMed Central  Google Scholar 

  89. Ruggieri G, Libri M, Gargano T, et al. Congenital colonic stenosis: a case of late-onset. Pediatr Med Chir. 2009;31:130–3.

    Google Scholar 

  90. Guo JB, Li ZZ. A case report of congenital colonic stenosis. Chin J Contemp Pediatr. 2009;11:699–700.

    Google Scholar 

  91. Saha N, Talukder SA, Alam S. Congenital stenosis in the descending colon causing intestinal obstruction in a one and half years male child. Mymensingh Med J. 2013;22:574–7.

    CAS  PubMed  Google Scholar 

  92. Zambaiti E, Chiaramonte C, Salemo S, et al. Multiple congenital colonic stenosis: a rare gastrointestinal malformation. Case Rep Pediatr. 2016;2016:4.

    Google Scholar 

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Acknowledgements

Thank you to Olivier Reinberg for reviewing this chapter.

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Authors and Affiliations

  1. Department of Pediatric Surgery, University Hospital Centre, Saint-Etienne, 42055, Cedex 2, France

    François Varlet, Sophie Vermersch & Aurélien Scalabre

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  1. François Varlet
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Correspondence to François Varlet .

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  1. Pediatric Surgery, S.Orsola Hospital, University of Bologna, Bologna, Italy

    Mario Lima

  2. Pediatric Surgeon FMH EBPS, Lausanne-Pully, Switzerland

    Olivier Reinberg

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Varlet, F., Vermersch, S., Scalabre, A. (2019). Bowel Atresia and Stenosis. In: Lima, M., Reinberg, O. (eds) Neonatal Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-93534-8_18

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