Fetal Surgery

Chapter

Abstract

Maternal fetal surgery for correction of an anatomic defect was first performed 30 years ago by Michael Harrison. At that time, the concept of the fetus as a patient was the subject of philosophical and ethical debate and the rationale and pre-requisites for prenatal surgical treatment were in evolution. Over the past three decades, the concept of the fetus as a patient has become commonly accepted and the ethical framework for maternal fetal intervention is now well developed. Improvements in prenatal diagnosis now provide certainty for the primary diagnosis and, in competent hands, can identify or exclude virtually all significant associated anomalies. Clinical experience with prenatally diagnosed fetuses has provided insight into the natural history of specific anomalies, improved our ability to predict the outcome for an individual fetus, and allowed more accurate selection of fetuses that will benefit from prenatal surgery. While application of open fetal surgery has remained limited to only a few anomalies, it is important to appreciate that the development of this field has accelerated technological progress in prenatal diagnosis and intervention, led to improved understanding of the pathophysiology and natural history of candidate disorders, allowed comprehensive counseling of prospective parents in centers with focused expertise in fetal anomalies, and driven the evolution of less invasive therapeutic approaches. In this chapter we will discuss the rationale and current indications for open fetal surgery, the evidence supporting its efficacy, and basic physiologic and technical considerations common to all fetal surgical interventions.

Keywords

Fetal surgery Birth defects Fetus Outcomes 

References

  1. 1.
    Harrison MR, Golbus MS, Filly RA, Callen PW, Katz M, de Lorimier AA, et al. Fetal surgery for congenital hydronephrosis. N Engl J Med. 1982;306(10):591–3.CrossRefGoogle Scholar
  2. 2.
    Harrison MR. Unborn: historical perspective of the fetus as a patient. Pharos Alpha Omega Alpha Honor Med Soc. 1982;45(1):19–24.Google Scholar
  3. 3.
    Flake AW. Prenatal intervention: ethical considerations for life-threatening and non-life-threatening anomalies. Semin Pediatr Surg. 2001;10(4):212–21.CrossRefGoogle Scholar
  4. 4.
    Glick PL, Harrison MR, Adzick NS, Noall RA, Villa RL. Correction of congenital hydronephrosis in utero IV: in utero decompression prevents renal dysplasia. J Pediatr Surg. 1984;19(6):649–57.CrossRefGoogle Scholar
  5. 5.
    Harrison MR, Ross NA, de Lorimier AA. Correction of congenital diaphragmatic hernia in utero. III. Development of a successful surgical technique using abdominoplasty to avoid compromise of umbilical blood flow. J Pediatr Surg. 1981;16(6):934–42.CrossRefGoogle Scholar
  6. 6.
    Adzick NS, Harrison MR, Glick PL, Flake AW. Fetal urinary tract obstruction: experimental pathophysiology. Semin Perinatol. 1985;9(2):79–90.Google Scholar
  7. 7.
    Adzick N, Harrison M, Flake A, Glick P, Bottles K. Automatic uterine stapling devices in fetal surgery: experience in a primate model. Surg Forum. 1985;XXXVI:479–81.Google Scholar
  8. 8.
    Adzick NS, Harrison MR, Glick PL, Anderson J, Villa RL, Flake AW, et al. Fetal surgery in the primate. III. Maternal outcome after fetal surgery. J Pediatr Surg. 1986;21(6):477–80.CrossRefGoogle Scholar
  9. 9.
    Harrison MR, Anderson J, Rosen MA, Ross NA, Hendrickx AG. Fetal surgery in the primate I. Anesthetic, surgical, and tocolytic management to maximize fetal-neonatal survival. J Pediatr Surg. 1982;17(2):115–22.CrossRefGoogle Scholar
  10. 10.
    Nakayama DK, Harrison MR, Seron-Ferre M, Villa RL. Fetal surgery in the primate II. Uterine electromyographic response to operative procedures and pharmacologic agents. J Pediatr Surg. 1984;19(4):333–9.CrossRefGoogle Scholar
  11. 11.
    Adzick NS. Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med. 2010;15(1):1–8.CrossRefGoogle Scholar
  12. 12.
    Johnson MP. The North American Fetal Therapy Network (NAFTNet): a new approach to collaborative research in fetal diagnosis and therapy. Semin Fetal Neonatal Med. 2010;15(1):52–7.CrossRefGoogle Scholar
  13. 13.
    Adzick NS. Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med. 2010;15(1):9–14.CrossRefGoogle Scholar
  14. 14.
    Chervenak FA, McCullough LB. Ethics of maternal-fetal surgery. Semin Fetal Neonatal Med. 2007;12(6):426–31.CrossRefGoogle Scholar
  15. 15.
    Gonzaga S, Henriques-Coelho T, Davey M, Zoltick PW, Leite-Moreira AF, Correia-Pinto J, et al. Cystic adenomatoid malformations are induced by localized FGF10 overexpression in fetal rat lung. Am J Respir Cell Mol Biol. 2008;39(3):346–55.CrossRefGoogle Scholar
  16. 16.
    d’Agostino S, Bonoldi E, Dante S, Meli S, Cappellari F, Musi L. Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature. J Pediatr Surg. 1997;32(9):1381–3.CrossRefGoogle Scholar
  17. 17.
    Granata C, Gambini C, Balducci T, Toma P, Michelazzi A, Conte M, et al. Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation. Pediatr Pulmonol. 1998;25(1):62–6.CrossRefGoogle Scholar
  18. 18.
    Sudou M, Sugi K, Murakami T. Bronchioloalveolar carcinoma arising from a congenital cystic adenomatoid malformation in an adolescent: the first case report from the orient. J Thorac Cardiovasc Surg. 2003;126(3):902–3.CrossRefGoogle Scholar
  19. 19.
    Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998;179(4):884–9.CrossRefGoogle Scholar
  20. 20.
    MacGillivray TE, Harrison MR, Goldstein RB, Adzick NS. Disappearing fetal lung lesions. J Pediatr Surg. 1993;28(10):1321–4. discussion 4–5.CrossRefGoogle Scholar
  21. 21.
    Crombleholme TM, Coleman B, Hedrick H, Liechty K, Howell L, Flake AW, et al. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg. 2002;37(3):331–8.CrossRefGoogle Scholar
  22. 22.
    Tsai AY, Liechty KW, Hedrick HL, Bebbington M, Wilson RD, Johnson MP, et al. Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions. J Pediatr Surg. 2008;43(3):513–7.CrossRefGoogle Scholar
  23. 23.
    Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, et al. Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther. 2007;22(5):365–71.CrossRefGoogle Scholar
  24. 24.
    Morris LM, Lim FY, Livingston JC, Polzin WJ, Crombleholme TM. High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg. 2009;44(1):60–5.CrossRefGoogle Scholar
  25. 25.
    Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, et al. Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg. 2010;45(1):145–50.CrossRefGoogle Scholar
  26. 26.
    Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, et al. The ex utero intrapartum therapy procedure for high-risk fetal lung lesions. J Pediatr Surg. 2005;40(6):1038–43. discussion 44.CrossRefGoogle Scholar
  27. 27.
    Flake AW. Fetal sacrococcygeal teratoma. Semin Pediatr Surg. 1993;2(2):113–20.Google Scholar
  28. 28.
    Roybal JL, Moldenhauer JS, Khalek N, Bebbington MW, Johnson MP, Hedrick HL, et al. Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas. J Pediatr Surg. 2011;46(7):1325–32.CrossRefGoogle Scholar
  29. 29.
    Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg. 1974;9(3):389–98.CrossRefGoogle Scholar
  30. 30.
    Flake AW, Harrison MR, Adzick NS, Laberge JM, Warsof SL. Fetal sacrococcygeal teratoma. J Pediatr Surg. 1986;21(7):563–6.CrossRefGoogle Scholar
  31. 31.
    Bond SJ, Harrison MR, Schmidt KG, Silverman NH, Flake AW, Slotnick RN, et al. Death due to high-output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg. 1990;25(12):1287–91.CrossRefGoogle Scholar
  32. 32.
    Schmidt KG, Silverman NH, Harison MR, Callen PW. High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and Doppler ultrasound. J Pediatr. 1989;114(6):1023–8.CrossRefGoogle Scholar
  33. 33.
    Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, et al. Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome. J Pediatr Surg. 2004;39(3):430–8. discussion -8.CrossRefGoogle Scholar
  34. 34.
    Mitchell LE, Adzick NS, Melchionne J, Pasquariello PS, Sutton LN, Whitehead AS. Spina bifida. Lancet. 2004;364(9448):1885–95.CrossRefGoogle Scholar
  35. 35.
    Botto LD, Moore CA, Khoury MJ, Erickson JD. Neural-tube defects. N Engl J Med. 1999;341(20):1509–19.CrossRefGoogle Scholar
  36. 36.
    Velie EM, Shaw GM. Impact of prenatal diagnosis and elective termination on prevalence and risk estimates of neural tube defects in California, 1989–1991. Am J Epidemiol. 1996;144(5):473–9.CrossRefGoogle Scholar
  37. 37.
    Edmonds LD, James LM. Temporal trends in the prevalence of congenital malformations at birth based on the birth defects monitoring program, United States, 1979–1987. MMWR CDC Surveill Summ. 1990;39(4):19–23.PubMedPubMedCentralGoogle Scholar
  38. 38.
    Garne E, Dolk H, Loane M, Boyd PA. EUROCAT website data on prenatal detection rates of congenital anomalies. J Med Screen. 2010;17(2):97–8.CrossRefGoogle Scholar
  39. 39.
    Oakeshott P, Hunt GM. Long-term outcome in open spina bifida. Br J Gen Pract. 2003;53(493):632–6.PubMedPubMedCentralGoogle Scholar
  40. 40.
    Hunt GM. Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol. 1990;32(2):108–18.CrossRefGoogle Scholar
  41. 41.
    Bruner JP, Tulipan NE, Richards WO. Endoscopic coverage of fetal open myelomeningocele in utero. Am J Obstet Gynecol. 1997;176(1 Pt 1):256–7.CrossRefGoogle Scholar
  42. 42.
    Bruner JP, Tulipan NB, Richards WO, Walsh WF, Boehm FH, Vrabcak EK. In utero repair of myelomeningocele: a comparison of endoscopy and hysterotomy. Fetal Diagn Ther. 2000;15(2):83–8.CrossRefGoogle Scholar
  43. 43.
    Adzick NS, Sutton LN, Crombleholme TM, Flake AW. Successful fetal surgery for spina bifida. Lancet. 1998;352(9141):1675–6.CrossRefGoogle Scholar
  44. 44.
    Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW. Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA. 1999;282(19):1826–31.CrossRefGoogle Scholar
  45. 45.
    Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, et al. Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol. 2003;189(2):482–7.CrossRefGoogle Scholar
  46. 46.
    Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993–1004.CrossRefGoogle Scholar
  47. 47.
    Danzer E, Gerdes M, Bebbington MW, Koh J, Adzick SN, Johnson MP. Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM). Childs Nerv Syst. 2011;27(7):1083–8.CrossRefGoogle Scholar
  48. 48.
    Labbe A, Coste K, Dechelotte PJ. Congenital diaphragmatic hernia—mechanisms of pulmonary hypoplasia. Rev Mal Respir. 2011;28(4):463–74.CrossRefGoogle Scholar
  49. 49.
    Kitagawa M, Hislop A, Boyden EA, Reid L. Lung hypoplasia in congenital diaphragmatic hernia. A quantitative study of airway, artery, and alveolar development. Br J Surg. 1971;58(5):342–6.CrossRefGoogle Scholar
  50. 50.
    Mohseni-Bod H, Bohn D. Pulmonary hypertension in congenital diaphragmatic hernia. Semin Pediatr Surg. 2007;16(2):126–33.CrossRefGoogle Scholar
  51. 51.
    Hedrick HL. Management of prenatally diagnosed congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2010;15(1):21–7.CrossRefGoogle Scholar
  52. 52.
    Mullassery D, Ba’ath ME, Jesudason EC, Losty PD. Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2010;35(5):609–14.CrossRefGoogle Scholar
  53. 53.
    Hedrick HL, Danzer E, Merchant A, Bebbington MW, Flake AW, Johnson MP, et al. Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol. 2007;197:422e1–4.CrossRefGoogle Scholar
  54. 54.
    Jani JC, Benachi A, Nicolaides KH, Allegaert K, Gratacos E, Mazkereth R, et al. Prenatal prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia: a multicenter study. Ultrasound Obstet Gynecol. 2009;33(1):64–9.CrossRefGoogle Scholar
  55. 55.
    Harrison MR, Adzick NS, Flake AW, Jennings RW, Estes JM, MacGillivray TE, et al. Correction of congenital diaphragmatic hernia in utero: VI. Hard-earned lessons. J Pediatr Surg. 1993;28(10):1411–7. discussion 7–8.CrossRefGoogle Scholar
  56. 56.
    DiFiore JW, Wilson JM. Lung development. [Review]. Semin Pediatr Surg. 1994;3(4):221–32.Google Scholar
  57. 57.
    DiFiore JW, Fauza DO, Slavin R, Peters CA, Fackler JC, Wilson JM. Experimental fetal tracheal ligation reverses the structural and physiological effects of pulmonary hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg. 1994;29(2):248–56. discussion 56–7.CrossRefGoogle Scholar
  58. 58.
    Hedrick MH, Estes JM, Sullivan KM, Bealer JF, Kitterman JA, Flake AW, et al. Plug the lung until it grows (PLUG): a new method to treat congenital diaphragmatic hernia in utero. J Pediatr Surg. 1994;29(5):612–7.CrossRefGoogle Scholar
  59. 59.
    O’Toole SJ, Karamanoukian HL, Irish MS, Sharma A, Holm BA, Glick PL. Tracheal ligation: the dark side of in utero congenital diaphragmatic hernia treatment. J Pediatr Surg. 1997;32(3):407–10.CrossRefGoogle Scholar
  60. 60.
    Harrison MR, Mychaliska GB, Albanese CT, Jennings RW, Farrell JA, Hawgood S, et al. Correction of congenital diaphragmatic hernia in utero IX: fetuses with poor prognosis (liver herniation and low lung-to-head ratio) can be saved by fetoscopic temporary tracheal occlusion. J Pediatr Surg. 1998;33(7):1017–22. discussion 22–3.CrossRefGoogle Scholar
  61. 61.
    Harrison MR, Keller RL, Hawgood SB, Kitterman JA, Sandberg PL, Farmer DL, et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med. 2003;349(20):1916–24.CrossRefGoogle Scholar
  62. 62.
    Flake AW, Crombleholme TM, Johnson MP, Howell LJ, Adzick NS. Treatment of severe congenital diaphragmatic hernia by fetal tracheal occlusion: clinical experience with fifteen cases. Am J Obstet Gynecol. 2000;183(5):1059–66.CrossRefGoogle Scholar
  63. 63.
    Jani JC, Nicolaides KH, Gratacos E, Valencia CM, Done E, Martinez JM, et al. Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion. Ultrasound Obstet Gynecol. 2009;34(3):304–10.CrossRefGoogle Scholar
  64. 64.
    Deprest JA, Gratacos E, Nicolaides K, Done E, Van Mieghem T, Gucciardo L, et al. Changing perspectives on the perinatal management of isolated congenital diaphragmatic hernia in Europe. Clin Perinatol. 2009;36(2):329–47. ix.CrossRefGoogle Scholar
  65. 65.
    Senat MV, Deprest J, Boulvain M, Paupe A, Winer N, Ville Y. Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med. 2004;351(2):136–44.CrossRefGoogle Scholar
  66. 66.
    Harrison MR, Adzick NS, Flake AW, VanderWall KJ, Bealer JF, Howell LJ, et al. Correction of congenital diaphragmatic hernia in utero VIII: Response of the hypoplastic lung to tracheal occlusion. J Pediatr Surg. 1996;31(10):1339–48.CrossRefGoogle Scholar
  67. 67.
    Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Adzick NS, et al. The EXIT procedure: experience and outcome in 31 cases. J Pediatr Surg. 2002;37(3):418–26.CrossRefGoogle Scholar
  68. 68.
    Hirose S, Sydorak RM, Tsao K, Cauldwell CB, Newman KD, Mychaliska GB, et al. Spectrum of intrapartum management strategies for giant fetal cervical teratoma. J Pediatr Surg. 2003;38(3):446–50. discussion -50.CrossRefGoogle Scholar

Copyright information

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

Authors and Affiliations

  1. 1.Department of Pediatric SurgeryUniversity of Pennsylvania Perelman School of Medicine, Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Department of SurgeryAbramson Research CenterPhiladelphiaUSA

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