Long-Term Outcomes in Neonatal Surgery

  • Risto J. Rintala
  • Mikko P. Pakarinen


Paediatric surgery and neonatal surgery as a part of it started to develop to an independent surgical speciality after World War II simultaneously in many Western countries. The first paediatric intensive care units and neonatal surgical units were opened in 1950s. These factors lead to rapid change in the mortality of patients with congenital malformations. Specialised paediatric surgeons and, surgical wards and operation theatres dedicated to care for children enabled survival of increasing numbers of patients with congenital defects and acquired neonatal surgical problems.


Neonatal surgery Paediatric surgery Long term outcomes Quality of life 


  1. 1.
    Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, Pakarinen MP. Cancer after repair of esophageal atresia: population-based long-term follow-up. J Pediatr Surg. 2008;43:602–5.CrossRefGoogle Scholar
  2. 2.
    Killelea BK, Lazar EL, Vitale MG. Principles of outcome analysis. In: Stringer MD, Oldham KT, Mouriquand PDE, editors. Pediatric surgery and urology (Chap. 2). 2nd ed. Cambridge: Cambridge University Press; 2006. p. 17–28.CrossRefGoogle Scholar
  3. 3.
    Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992;30:473–83.CrossRefGoogle Scholar
  4. 4.
    Varni JM, Limbers CA, Burwinkle TM. Impaired health-related quality of life in children and adolescents with chronic conditions: a comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL 4.0 Generic Core Scales. Health Qual Life Outcomes. 2007;5:43–58.CrossRefGoogle Scholar
  5. 5.
    Eypasch E, Williams JI, Wood-Dauphinee S, et al. Gastrointestinal quality of life index: development, validation, and application of a new instrument. Br J Surg. 1995;82:216–22.CrossRefGoogle Scholar
  6. 6.
    Lemons JA, Bauer CR, Oh W, et al. Very low birth weight outcomes of the National Institute of Child health and human development neonatal research network, January 1995 through December 1996. NICHD Neonatal Research Network. Pediatrics. 2001;107:E1.CrossRefGoogle Scholar
  7. 7.
    Vincer MJ, Allen AC, Joseph KS, Stinson DA, Scott H, Wood E. Increasing prevalence of cerebral palsy among very preterm infants: a population-based study. Pediatrics. 2006;118:e1621–6.CrossRefGoogle Scholar
  8. 8.
    Marlow N, Wolke D, Bracewell MA, Samara M, EPICure Study Group. Neurologic and developmental disability at six years of age after extremely preterm birth. N Engl J Med. 2005;352:9–19.CrossRefGoogle Scholar
  9. 9.
    Jevtovic-Todorovic V, Hartman RE, Izumi Y, et al. Early exposure to common anesthetic agents causes widespread neurodegeneration in the developing rat brain and persistent learning deficits. J Neurosci. 2003;23:876–82.CrossRefGoogle Scholar
  10. 10.
    Loepke AW. Developmental neurotoxicity of sedatives and anesthetics: a concern for neonatal and pediatric critical care medicine? Pediatr Crit Care Med. 2010;11:217–26.CrossRefGoogle Scholar
  11. 11.
    Wilder RT, Flick RP, Sprung J, et al. Early exposure to anesthesia and learning disabilities in a population-based birth cohort. Anesthesiology. 2009;110:796–804.CrossRefGoogle Scholar
  12. 12.
    Bartels M, Althoff RR, Boomsma DI. Anesthesia and cognitive performance in children: no evidence for a causal relationship. Twin Res Hum Genet. 2009;12:246–53.CrossRefGoogle Scholar
  13. 13.
    Rees CM, Pierro A, Eaton S. Neurodevelopmental outcomes of neonates with medically and surgically treated necrotizing enterocolitis. Arch Dis Child Fetal Neonatal Ed. 2007;92:193–8.CrossRefGoogle Scholar
  14. 14.
    Ta BD, Roze E, van Braeckel KN, et al. Long-term neurodevelopmental impairment in neonates surgically treated for necrotizing enterocolitis: enterostomy associated with a worse outcome. Eur J Pediatr Surg. 2011;21:58–64.CrossRefGoogle Scholar
  15. 15.
    Ahmad A, Gangitano E, Odell RM, et al. Survival, intracranial lesions, and neurodevelopmental outcome in infants with congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. J Perinatol. 1999;19(6 Pt 1):436–40.CrossRefGoogle Scholar
  16. 16.
    Danzer E, Gerdes M, Bernbaum J, et al. Neurodevelopmental outcome of infants with congenital diaphragmatic hernia prospectively enrolled in an interdisciplinary follow-up program. J Pediatr Surg. 2010;45:1759–66.CrossRefGoogle Scholar
  17. 17.
    Calderon J, Bonnet D, Courtin C, Concordet S, Plumet MH, Angeard N. Executive function and theory of mind in school-aged children after neonatal corrective cardiac surgery for transposition of the great arteries. Dev Med Child Neurol. 2010;52:1139–44.CrossRefGoogle Scholar
  18. 18.
    Vahsen N, Kavsek M, Toussaint-Götz N, Schneider K, Urban AE, Schneider M. Cognitive and motor abilities and behavioural outcome in children after neonatal operation with cardiopulmonary bypass. Klin Padiatr. 2009;221:19–24.CrossRefGoogle Scholar
  19. 19.
    Ludman L, Spitz L, Lansdown R. Developmental progress of newborns undergoing neonatal surgery. J Pediatr Surg. 1990;25:469–71.CrossRefGoogle Scholar
  20. 20.
    Ludman L, Spitz L, Lansdown R. Intellectual development at 3 years of age of children who underwent major neonatal surgery. J Pediatr Surg. 1993;28:130–4.CrossRefGoogle Scholar
  21. 21.
    Rescorla FJ, Grosfeld JL. Inguinal hernia repair in the perinatal period and early infancy: clinical considerations. J Pediatr Surg. 1984;19:832–7.CrossRefGoogle Scholar
  22. 22.
    Steinau G, Treutner KH, Feeken G, et al. Recurrent inguinal hernias in infants and children. World J Surg. 1995;19:303–6.CrossRefGoogle Scholar
  23. 23.
    Benson CD, Lloyd JR. Infantile pyloric stenosis. A review of 1,120 cases. Am J Surg. 1964;107:429–33.CrossRefGoogle Scholar
  24. 24.
    Dietl KH, Borowski U, Menzel J, Wissing C, Senninger N, Brockmann J. Long-term investigations after pyloromyotomy for infantile pyloric stenosis. Eur J Pediatr Surg. 2000;10:365–7.CrossRefGoogle Scholar
  25. 25.
    Carter CO, Evans KA. Inheritance of congenital pyloric stenosis. J Med Genet. 1969;6:233–54.CrossRefGoogle Scholar
  26. 26.
    Sistonen SJ, Koivusalo A, Nieminen U, Lindahl H, Lohi J, Kero M, Kärkkäinen P, Färkkilä MA, Sarna S, Rintala RJ, Pakarinen MP. Esophageal morbidity and function in adults with repaired esophageal atresia: A population-based long-term follow-up. Ann Surg. 2010;251:1167–73.CrossRefGoogle Scholar
  27. 27.
    Deurloo JA, van Lanschot JJ, Drillenburg P, et al. Esophageal squamous cell carcinoma 38 years after primary repair of esophageal atresia. J Pediatr Surg. 2001;36:629–30.CrossRefGoogle Scholar
  28. 28.
    Sistonen SJ, Koivusalo A, Lindahl H, Pukkala E, Rintala RJ, Pakarinen MP. Cancer after repair of esophageal atresia: Population-based long-term follow-up. J Pediatr Surg. 2008;43:602–5.CrossRefGoogle Scholar
  29. 29.
    Deurloo JA, Ekkelkamp S, Taminiau JA, Kneepkens CM, Ten Kate FW, Bartelsman JF, Legemate DA, Aronson DC. Esophagitis and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg. 2005;40:1227–31.CrossRefGoogle Scholar
  30. 30.
    Taylor AC, Breen KJ, Auldist A, et al. Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: A long-term follow-up study. Clin Gastroenterol Hepatol. 2007;5:702–6.CrossRefGoogle Scholar
  31. 31.
    Krug E, Bergmeijer JH, Dees J, et al. Gastroesophageal reflux and Barrett’s esophagus in adults born with esophageal atresia. Am J Gastroenterol. 1999;94:2825–8.CrossRefGoogle Scholar
  32. 32.
    Chetcuti P, Phelan PD. Gastrointestinal morbidity and growth after repair of oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child. 1993;68:163–6.CrossRefGoogle Scholar
  33. 33.
    Sistonen SJ, Helenius I, Peltonen J, Sarna S, Rintala RJ, Pakarinen MP. Natural history of spinal anomalies and scoliosis associated with esophageal atresia. Pediatrics. 2009;124:e1198–204. Epub 2009 Nov 9CrossRefGoogle Scholar
  34. 34.
    Jaureguizar E, Vazquez J, Murcia J, et al. Morbid musculoskeletal sequelae of the thoracotomy for esophageal fistula. J Pediatr Surg. 1985;20:511–4.CrossRefGoogle Scholar
  35. 35.
    Hall JE, Simmons ED, Danylchuk K, Barnes PD. Instability of the cervical spine and neurological involvement in Klippel-Feil syndrome. A case report. J Bone Joint Surg Am. 1990;72(3):460–2.CrossRefGoogle Scholar
  36. 36.
    Koivusalo A, Pakarinen MP, Turunen P, et al. Health-related quality of life in adult patients with esophageal atresia: a questionnaire study. J Pediatr Surg. 2005;40:307–12.CrossRefGoogle Scholar
  37. 37.
    Ure BM, Slany E, Eypasch EP, et al. Quality of life more than 20 years after repair of esophageal atresia. J Pediatr Surg. 1998;33:511–5.CrossRefGoogle Scholar
  38. 38.
    Sistonen SJ, Pakarinen MP, Rintala RJ. Long-term results of esophageal atresia: Helsinki experience and review of literature. Pediatr Surg Int. 2011;27:1141–9.CrossRefGoogle Scholar
  39. 39.
    Yanchar NL, Soucy P. Long-term outcome after Hirschsprung’s disease: patients’ perspectives. J Pediatr Surg. 1999;34:1152–60.CrossRefGoogle Scholar
  40. 40.
    Reding R, de Ville de Goyet J, Gosseye S, et al. Hirschsprung’s disease: a 20-year experience. J Pediatr Surg. 1997; 32:1221–5.CrossRefGoogle Scholar
  41. 41.
    Catto-Smith AG, Coffey CM, Nolan TM, Hutson JM. Fecal incontinence after the surgical treatment of Hirschsprung disease. J Pediatr. 1995;127:954–7.CrossRefGoogle Scholar
  42. 42.
    Bai Y, Chen H, Hao J, et al. Long-term outcome and quality of life after the Swenson procedure for Hirschsprung’s disease. J Pediatr Surg. 2002;37:639–42.CrossRefGoogle Scholar
  43. 43.
    Jarvi K, Laitakari EM, Koivusalo A, et al. Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg. 2010;252:977–81.CrossRefGoogle Scholar
  44. 44.
    Heikkinen M, Rintala RJ, Louhimo I. Bowel function and quality of life in adult patients with operated Hirschsprung’s disease. Pediatr Surg Int. 1995;10:342–4.CrossRefGoogle Scholar
  45. 45.
    Baillie CT, Kenny SE, Rintala RJ, et al. Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung disease. J Pediatr Surg. 1999;34:325–9.CrossRefGoogle Scholar
  46. 46.
    Diseth TH, Bjornland K, Novik TS, et al. Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung’s disease. Arch Dis Child. 1997;76:100–6.CrossRefGoogle Scholar
  47. 47.
    Pakarinen MP, Rintala RJ, Koivusalo A, et al. Increased incidence of medullary thyroid carcinoma in patients treated for Hirschsprung’s disease. J Pediatr Surg. 2005;40:1532–4.CrossRefGoogle Scholar
  48. 48.
    Moore SW, Zaahl MG. Multiple endocrine neoplasia syndromes, children, Hirschsprung’s disease and RET. Pediatr Surg Int. 2008;24:521–30.CrossRefGoogle Scholar
  49. 49.
    Rintala RJ, Pakarinen MP. Imperforate anus: long- and short-term outcome. Semin Pediatr Surg. 2008;17:79–89.CrossRefGoogle Scholar
  50. 50.
    Hassink EA, Rieu PN, Severijnen RS, et al. Are adults content or continent after repair for high anal atresia? A long-term follow-up study in patients 18 years of age and older. Ann Surg. 1993;218:196–200.CrossRefGoogle Scholar
  51. 51.
    Stephens FD, Smith ED. Classification, identification and assessment of surgical treatment of anorectal anomalies. Pediatr Surg Int. 1986;1:200–5.CrossRefGoogle Scholar
  52. 52.
    Stephens FD, Smith ED. Ano-rectal malformations in children. Chicago: Year Book Medical; 1971.Google Scholar
  53. 53.
    Templeton JM, Ditesheim JA. High imperforate anus—quantitative result of long-term fecal continence. J Pediatr Surg. 1985;20:645–52.CrossRefGoogle Scholar
  54. 54.
    Holschneider A, Hutson J, Peña A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg. 2005;40:1521–6.CrossRefGoogle Scholar
  55. 55.
    Peña A. Anorectal malformations. Semin Pediatr Surg. 1995;4:35–47.Google Scholar
  56. 56.
    Rintala R, Mildh L, Lindahl H. Fecal continence and quality of life in adult patients with an operated high or intermediate anorectal malformation. J Pediatr Surg. 1994;29:777–80.CrossRefGoogle Scholar
  57. 57.
    Rintala R, Lindahl H, Sariola H, et al. The rectourogenital connection in anorectal malformations is an ectopic anal canal. J Pediatr Surg. 1990;25:665–8.CrossRefGoogle Scholar
  58. 58.
    Husberg B, Lindahl H, Rintala R, et al. High and intermediate imperforate anus: Results after surgical correction with special respect to internal sphincter function. J Pediatr Surg. 1992;27:185–9.CrossRefGoogle Scholar
  59. 59.
    Iwai N, Hashimoto K, Goto Y, et al. Long term results after surgical correction of anorectal malformations. Z Kinderchir. 1984;39:35–9.CrossRefGoogle Scholar
  60. 60.
    Mollard P, Meunier P, Mouriquand P, et al. High and intermediate imperforate anus: functional results and postoperative manometric assessment. Eur J Pediatr Surg. 1991;1:282–6.CrossRefGoogle Scholar
  61. 61.
    Hedlund H, Peña A, Rodriquez G, et al. Long-term anorectal function in imperforate anus treated by a posterior sagittal anorectoplasty: manometric investigation. J Pediatr Surg. 1992;27:906–9.CrossRefGoogle Scholar
  62. 62.
    Holschneider AM, Pfrommer W. Gerresheim B, Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur J Pediatr Surg. 1994;4:303–9.CrossRefGoogle Scholar
  63. 63.
    Rintala R, Lindahl H, Marttinen E, et al. Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations. J Pediatr Surg. 1993;28:1054–8.CrossRefGoogle Scholar
  64. 64.
    Langemeijer RATM, Molenaar JC. Continence after posterior sagittal anorectoplasty. J Pediatr Surg. 1991;26:587–90.CrossRefGoogle Scholar
  65. 65.
    Rintala R, Marttinen E, Virkola K, et al. Segmental colonic motility in patients with anorectal malformations. J Pediatr Surg. 1997;32:453–6.CrossRefGoogle Scholar
  66. 66.
    Mulder W, de Jong E, Wauters I, et al. Posterior sagittal anorectoplasty: functional results of primary and secondary operations in comparison to the pull-through method in anorectal malformations. Eur J Pediatr Surg. 1995;5:170–3.CrossRefGoogle Scholar
  67. 67.
    Tuuha SE, Aziz D, Drake J, et al. Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients? J Pediatr Surg. 2004;39:773–7.CrossRefGoogle Scholar
  68. 68.
    Breech L. Gynecologic concerns in patients with anorectal malformations. Semin Pediatr Surg. 2010;19:139–45.CrossRefGoogle Scholar
  69. 69.
    Rintala R, Mildh L, Lindahl H. Fecal continence and quality of life in adult patients with an operated low anorectal malformation. J Pediatr Surg. 1992;27:902–5.CrossRefGoogle Scholar
  70. 70.
    Hassink EA, Rieu PN, Brugman AT, Festen C. Quality of life after operatively corrected high anorectal malformation: a long-term follow-up study of patients aged 18 years and older. J Pediatr Surg. 1994;29:773–6.CrossRefGoogle Scholar
  71. 71.
    Hartman EE, Oort FJ, Aronson DC, et al. Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung’s disease. Am J Gastroenterol. 2004;99:907–13.CrossRefGoogle Scholar
  72. 72.
    Hartman EE, Oort FJ, Aronson DC, et al. Explaining change in quality of life of children and adolescents with anorectal malformations or Hirschsprung disease. Pediatrics. 2007;119:e374–83.CrossRefGoogle Scholar
  73. 73.
    Hartman EE, Oort FJ, Sprangers MA, et al. Factors affecting quality of life of children and adolescents with anorectal malformations or Hirschsprung disease. J Pediatr Gastroenterol Nutr. 2008;47:463–71.CrossRefGoogle Scholar
  74. 74.
    Diseth TH, Emblem R. Somatic function, mental health, and psychosocial adjustment of adolescents with anorectal anomalies. J Pediatr Surg. 1996;31:638–43.CrossRefGoogle Scholar
  75. 75.
    Hanneman MJ, Sprangers MA, De Mik EL, et al. Quality of life in patients with anorectal malformation or Hirschsprung’s disease: development of a disease-specific questionnaire. Dis Colon Rectum. 2001;44:1650–60.CrossRefGoogle Scholar
  76. 76.
    Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 2001;34:114–20.CrossRefGoogle Scholar
  77. 77.
    Dillon CM, Davis BE, Duguay S, et al. Longevity of patients born with myelomeningocele. Eur J Pediatr Surg. 2000;10(Suppl 1):33–4.CrossRefGoogle Scholar
  78. 78.
    Shinkai M, Ohhama Y, Take H, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children’s hospital. J Pediatr Gastroenterol Nutr. 2009;48:443–50.CrossRefGoogle Scholar
  79. 79.
    Lykavieris P, Chardot C, Sokhn M, et al. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005;41:366–71.CrossRefGoogle Scholar
  80. 80.
    Hutson JM, Balic A, Nation T, Southwell B. Cryptorchidism. Semin Pediatr Surg. 2010;19:215–24.CrossRefGoogle Scholar
  81. 81.
    Nasr A, Himidan S, Pastor AC, et al. Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma? J Pediatr Surg. 2010;45:1086–9.CrossRefGoogle Scholar
  82. 82.
    Homsy YL, Anderson JH, Oudjhane K, Russo P. Wilms tumor and multicystic dysplastic kidney disease. J Urol. 1997;158:2256–9.CrossRefGoogle Scholar
  83. 83.
    Lee MY, Jensen E, Kwak S, Larson RA. Metastatic adenocarcinoma arising in a congenital foregut cyst of the esophagus: a case report with review of the literature. Am J Clin Oncol. 1998;21:64–6.CrossRefGoogle Scholar
  84. 84.
    Mathieu A, Chamlou R, Le Moine F, et al. Tailgut cyst associated with a carcinoid tumor: case report and review of the literature. Histol Histopathol. 2005;20(4):1065–9.Google Scholar
  85. 85.
    Michael D, Cohen CR, Northover JM. Adenocarcinoma within a rectal duplication cyst: case report and literature review. Ann R Coll Surg Engl. 1999;81:205–6.PubMedPubMedCentralGoogle Scholar
  86. 86.
    Kuraoka K, Nakayama H, Kagawa T, et al. Adenocarcinoma arising from a gastric duplication cyst with invasion to the stomach: a case report with literature review. J Clin Pathol. 2004;57:428–31.CrossRefGoogle Scholar
  87. 87.
    Yoshida A, Maoate K, Blakelock R, et al. Long-term functional outcomes in children with Currarino syndrome. Pediatr Surg Int. 2010;26:677–81.CrossRefGoogle Scholar
  88. 88.
    Benjamin IS. Biliary cystic disease: the risk of cancer. J Hepatobiliary Pancreat Surg. 2003;10:335–9.CrossRefGoogle Scholar
  89. 89.
    Diseth TH, Egeland T, Emblem R. Effects of anal invasive treatment and incontinence on mental health and psychosocial functioning of adolescents with Hirschsprung’s disease and low anorectal anomalies. J Pediatr Surg. 1998;33:468–75.CrossRefGoogle Scholar
  90. 90.
    Bau MO, Younes S, Aupy A, et al. The Malone antegrade colonic enema isolated or associated with urological incontinence procedures: evaluation from patient point of view. J Urol. 2001;165(6 Pt 2):2399–403.CrossRefGoogle Scholar
  91. 91.
    Wilkins BM, Spitz L. Incidence of postoperative adhesion obstruction following neonatal laparotomy. Br J Surg. 1986;73:762–4.CrossRefGoogle Scholar
  92. 92.
    Vanamo K, Rintala RJ, Lindahl H, Louhimo I. Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects. J Pediatr Surg. 1996;31:551–4.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag London Ltd., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Section of Paediatric SurgeryChildren’s Hospital, Helsinki University Central HospitalHelsinkiFinland

Personalised recommendations