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In Utero Surgery for Spina Bifida Aperta

  • Jan DeprestEmail author
  • Roland Devlieger
  • Frank Van Calenbergh
  • Sarah Devroe
  • Hannes Van der Merwe
  • Liesbeth Lewi
  • Luc De Catte
  • Luc Joyeux
Chapter

Abstract

Spina bifida aperta (SBA) can cause severe lifelong physical and neurodevelopmental disabilities. Experimental and clinical studies have shown that the neurological deficits associated with SBA are caused by a combination of incomplete neurulation (first hit) and additional damage caused by prolonged exposure of the spinal cord and nerves to the intrauterine environment, as well as a suction gradient due to cerebrospinal fluid leakage, in turn leading to progressive downward displacement of the hindbrain. The natural history can be reversed by prenatal repair. A randomized controlled trial demonstrated that mid-gestational maternal-fetal surgery for SBA decreases the need for ventriculoperitoneal shunting and hindbrain herniation at 12 months and improves neurological motor function at 30 months of age. The downside is that this operation has maternal and fetal risks, the most relevant ones being increased prematurity and a persistent uterine corporeal scar. Recently, minimally invasive fetal approaches have been introduced clinically, yet at present they lack extensive experimental or clinical trials. This chapter describes the operative technique of open prenatal repair and the short-term, 5- and 10-year outcomes with that approach.

Keywords

Fetal surgery Spina bifida Myelomeningocele Myeloschisis Hysterotomy fetoscopy Spina bifida aperta Uterine dehiscence Prenatal prelabor rupture of the membranes Preterm labor 

Supplementary material

Video 32.1

This video displays the steps of open fetal surgery for spina bifida aperta during a surgery performed at the University Hospital of Leuven, Belgium. Copyright UZ Leuven, Leuven, Belgium (MP4 170530 kb)

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Jan Deprest
    • 1
    Email author
  • Roland Devlieger
    • 1
  • Frank Van Calenbergh
    • 2
  • Sarah Devroe
    • 3
  • Hannes Van der Merwe
    • 4
  • Liesbeth Lewi
    • 1
  • Luc De Catte
    • 1
  • Luc Joyeux
    • 4
  1. 1.Department of Obstetrics and GynaecologyUniversity Hospitals Leuven, Campus GasthuisbergLeuvenBelgium
  2. 2.Department of NeurosurgeryUniversity Hospitals Leuven, Campus GasthuisbergLeuvenBelgium
  3. 3.Department of AnesthesiologyUniversity Hospitals Leuven, Campus GasthuisbergLeuvenBelgium
  4. 4.Department of Development and Regeneration, Cluster Woman and ChildKatholieke Universiteit LeuvenLeuvenBelgium

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