Skip to main content

Advertisement

SpringerLink
Log in

Fetal surgery for spina bifida in Zurich: results from 150 cases

  • Original Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Purpose

Over the past 10 years, over 150 fetal spina bifida surgeries were performed at the Zurich Center for Fetal Diagnosis and Therapy. This study looks at surrogates for success and failure of this approach.

Methods

We focused on key outcome parameters including hydrocephalus shunt rate at one year, bladder control at 4, independent ambulation at 3 years, and maternal, fetal, and neonatal complications.

Results

From the first 150 patients undergoing fetal surgery for spina bifida, 148 (98.7%) were included in the study. Maternal–fetal surgery was uneventful in 143/148 (97%) cases. Intraoperative problems included resuscitation in 4/148 fetuses (2.7%). 1/148 fetuses (0.7%) died on postoperative day 4. Maternal complications included chorioamniotic membrane separation in 22/148 (15%), lung embolism in 3/148 (2.1%), chorioamnionitis in 2/148 (1.4%), AV-block III and uterine rupture in 1/148 each (0.7%). 1/148 (0.7%) newborn death was recorded. Hindbrain herniation was identified preoperatively in 132/148 (90%) fetuses and resolved completely in 119/132 (90%). At one year, 39/106 (37%) children had required a CSF diversion. At 4 years, 4/34 patients (12%) had normal bladder control. At 3 years, 48/57 (84%) walked independently.

Conclusion

A majority of patients benefitted from prenatal intervention, in that the shunt rate was lower and the rates of continent and walking patients were higher than reported with postnatal care.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Hutchins GM, Meuli M, Meuli-Simmen C, Jordan MA, Heffez DS, Blakemore KJ (1996) Acquired spinal cord injury in human fetuses with myelomeningocele. Pediatr Pathol Lab Med 16(5):701–712

    Article  CAS  Google Scholar 

  2. Meuli M, Meuli-Simmen C, Yingling CD, Hutchins GM, Hoffman KM, Harrison MR, Adzick NS (1995) Creation of myelomeningocele in utero: a model of functional damage from spinal cord exposure in fetal sheep. J Pediatr Surg 30(7):1028–1032

    Article  CAS  Google Scholar 

  3. Meuli M, Meuli-Simmen C, Hutchins GM, Yingling CD, Hoffman KM, Harrison MR, Adzick NS (1995) In utero surgery rescues neurological function at birth in sheep with spina bifida. NatMed 1(4):342–347

    CAS  Google Scholar 

  4. Meuli M, Meuli-Simmen C, Yingling CD, Hutchins GM, Timmel GB, Harrison MR, Adzick NS (1996) In utero repair of experimental myelomeningocele saves neurological function at birth. J Pediatr Surg 31(3):397–402

    Article  CAS  Google Scholar 

  5. Adzick NS, Sutton LN, Crombleholme TM, Flake AW (1998) Successful fetal surgery for spina bifida. Lancet 352(9141):1675–1676

    Article  CAS  Google Scholar 

  6. Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, Hernanz-Schulman M, Lowe LH, Reed GW (1999) Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282(19):1819–1825

    Article  CAS  Google Scholar 

  7. Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ, Hedrick HL, Wilson RD, Adzick NS (2003) Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 189(2):482–487

    Article  Google Scholar 

  8. Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282(19):1826–1831

    Article  CAS  Google Scholar 

  9. Adzick NS, Thom EA, Spong CY, Brock JW III, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364(11):993–1004

    Article  CAS  Google Scholar 

  10. Möhrlen U, Ochsenbein-Kölble N, Mazzone L, Kraehenmann F, Hüsler M, Casanova B, Biro P, Wille D, Latal B, Scheer I, Bernet V, Moehrlen T, Held L, Flake AW, Zimmermann R, Meuli M (2020) Benchmarking against the MOMS trial: Zurich results of open fetal surgery for spina bifida. Fetal Diagn Ther 47(2):91–97

    Article  Google Scholar 

  11. Meuli M, Moehrlen U, Flake A, Ochsenbein N, Huesler M, Biro P, Scheer I, Tharakan S, Durig P, Zimmermann R (2013) Fetal surgery in Zurich: key features of our first open in utero repair of myelomeningocele. Eur J Pediatr Surg 23(6):494–498

    PubMed  Google Scholar 

  12. Dindo D, Demartines N, Clavien PA (2004) Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 240(2):205–213

    Article  Google Scholar 

  13. Vonzun L, Kahr MK, Noll F, Mazzone L, Moehrlen U, Meuli M, Hüsler M, Krähenmann F, Zimmermann R, Ochsenbein-Kölble N (2020) Systematic classification of maternal and fetal intervention related complications following open fetal myelomeningocele repair—results from a large prospective cohort. BJOG 5(10):1471–1528

    Google Scholar 

  14. Perotto L, Zimmermann R, Quack Lötscher KC (2020) Maternal mortality in Switzerland 2005–2014. Swiss Med Weekly 150:w20345

    Google Scholar 

  15. Moise KJ Jr, Moldenhauer JS, Bennett KA, Goodnight W, Luks FI, Emery SP, Tsao K, Moon-Grady AJ, Moore RC, Treadwell MC, Vlastos EJ, Wetjen NM (2016) Current selection criteria and perioperative therapy used for fetal myelomeningocele surgery. Obstet Gynecol 127(3):593–597

    Article  Google Scholar 

  16. Golombeck K, Ball RH, Lee H, Farrell JA, Farmer DL, Jacobs VR, Rosen MA, Filly RA, Harrison MR (2006) Maternal morbidity after maternal-fetal surgery. Am J Obstet Gynecol 194(3):834–839

    Article  Google Scholar 

  17. Bennett KA, Carroll MA, Shannon CN, Braun SA, Dabrowiak ME, Crum AK, Paschall RL, Kavanaugh-McHugh AL, Wellons JC 3rd, Tulipan NB (2014) Reducing perinatal complications and preterm delivery for patients undergoing in utero closure of fetal myelomeningocele: further modifications to the multidisciplinary surgical technique. J Neurosurg Pediatr 14(1):108–114

    Article  Google Scholar 

  18. Danzer E, Thomas NH, Thomas A, Friedman KB, Gerdes M, Koh J, Adzick NS, Johnson MP (2016) Long-term neurofunctional outcome, executive functioning, and behavioral adaptive skills following fetal myelomeningocele surgery. Am J Obstet Gynecol 214(2):269.e261–269.e268

    Article  Google Scholar 

  19. Moldenhauer JS, Soni S, Rintoul NE, Spinner SS, Khalek N, Martinez-Poyer J, Flake AW, Hedrick HL, Peranteau WH, Rendon N, Koh J, Howell LJ, Heuer GG, Sutton LN, Johnson MP, Adzick NS (2015) Fetal myelomeningocele repair: the post-MOMS experience at the Children’s Hospital of Philadelphia. Fetal Diagn Ther 37(3):235–240

    Article  Google Scholar 

  20. Zamłyński J, Olejek A, Koszutski T, Ziomek G, Horzelska E, Gajewska-Kucharek A, Maruniak-Chudek I, Herman-Sucharska I, Kluczewska E, Horak S, Bodzek P, Zamłyński M, Kowalik J, Horzelski T, Bohosiewicz J (2014) Comparison of prenatal and postnatal treatments of spina bifida in Poland–a non-randomized, single-center study. J Mater Fetal Neonatal Med 27(14):1409–1417

    Article  Google Scholar 

  21. Elbabaa SK, Gildehaus AM, Pierson MJ, Albers JA, Vlastos EJ (2017) First 60 fetal in-utero myelomeningocele repairs at Saint Louis Fetal Care Institute in the post-MOMS trial era: hydrocephalus treatment outcomes (endoscopic third ventriculostomy versus ventriculo-peritoneal shunt). Child’s Nervous System ChNS 33(7):1157–1168

    Article  Google Scholar 

  22. Heye P, Moehrlen U, Mazzone L, Weil R, Altermatt S, Wille DA, Scheer I, Meuli M, Horst M (2019) Inclusion cysts after fetal spina bifida repair: a third hit? Fetal Diagn Ther 46(1):38–44

    Article  Google Scholar 

  23. Horst M, Mazzone L, Schraner T, Bodmer C, Möhrlen U, Meuli M, Gobet R (2017) Prenatal myelomeningocele repair: do bladders better? Neurourol Urodyn 36(6):1651–1658

    Article  CAS  Google Scholar 

  24. Brock JW 3rd, Carr MC, Adzick NS, Burrows PK, Thomas JC, Thom EA, Howell LJ, Farrell JA, Dabrowiak ME, Farmer DL, Cheng EY, Kropp BP, Caldamone AA, Bulas DI, Tolivaisa S, Baskin LS (2015) Bladder function after fetal surgery for myelomeningocele. Pediatrics 136(4):e906-913

    Article  Google Scholar 

  25. Brock JW 3rd, Thomas JC, Baskin LS, Zderic SA, Thom EA, Burrows PK, Lee H, Houtrow AJ, MacPherson C, Adzick NS (2019) Effect of prenatal repair of myelomeningocele on urological outcomes at school age. J Urol 202(4):812–818

    Article  Google Scholar 

  26. Carr MC (2015) Urological results after fetal myelomeningocele repair in pre-MOMS trial patients at the Children’s Hospital of Philadelphia. Fetal Diagn Ther 37(3):211–218

    Article  Google Scholar 

  27. Mazzone L, Hölscher AC, Moehrlen U, Gobet R, Meuli M, Horst M (2020) Urological outcome after fetal spina bifida repair: data from the Zurich cohort. Fetal Diagn Ther 47(12):882–888

    Article  Google Scholar 

  28. Pastuszka A, Bohosiewicz J, Koszutski T (2018) Prenatal myelomeningocele repair improves urinary continence and reduces the risk of constipation. Neurourol Urodyn 37(8):2792–2798

    Article  Google Scholar 

  29. Snodgrass WT, Adams R (2004) Initial urologic management of myelomeningocele. Urolo Clin North Am 31(3):427–434

    Article  Google Scholar 

  30. Houtrow AJ, Thom EA, Fletcher JM, Burrows PK, Adzick NS, Thomas NH, Brock JW 3rd, Cooper T, Lee H, Bilaniuk L, Glenn OA, Pruthi S, MacPherson C, Farmer DL, Johnson MP, Howell LJ, Gupta N, Walker WO (2020) Prenatal repair of myelomeningocele and school-age functional outcomes. Pediatrics 145(2):e20191544

    Article  Google Scholar 

  31. Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS, Wilson RD, Johnson MP (2009) Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 25(1):47–53

    Article  Google Scholar 

  32. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA (2001) Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34(3):114–120

    Article  CAS  Google Scholar 

Download references

Acknowledgements

We thank the Clinical Trial Center, University Hospital Zurich, for hosting the Redcap® Database; we thank the following Foundations for their generous financial support for this study: Zwillenberg Foundation, Berne, Switzerland and Uniscientia, Zurich, Switzerland.

Author information

Author notes

  1. Roland Zimmermann and Martin Meuli Shared last authorship.

Authors and Affiliations

  1. Department of Pediatric Surgery, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Ueli Moehrlen, Luca Mazzone, Maya Horst, Sonja Schauer, Barbara Casanova, Theres Moehrlen, Sasha Tharakan & Martin Meuli

  2. The Zurich Center for Fetal Diagnosis and Therapy, Zurich, Switzerland

    Ueli Moehrlen, Nicole Ochsenbein, Ladina Vonzun, Luca Mazzone, Barbara Casanova, Nele Strübing, Theres Moehrlen, Sasha Tharakan, Roland Zimmermann & Martin Meuli

  3. Department of Obstetrics, University Hospital Zurich, Zurich, Switzerland

    Nicole Ochsenbein, Ladina Vonzun, Nele Strübing & Roland Zimmermann

  4. Department of Neonatology, University Hospital Zurich, Zurich, Switzerland

    Dirk Bassler

  5. Division of Pediatric Neurology, University Children’s Hospital Zurich, Zurich, Switzerland

    David Alexander Wille

  6. Division of Pediatric Rehabilitation, University Children’s Hospital Zurich, Zurich, Switzerland

    Beth Padden

  7. Division of Diagnostic Imaging, MR-Center, University Children’s Hospital Zurich, Zurich, Switzerland

    Raimund Kottke & Patrice Grehten

  8. Division of Intensive Care and Neonatology, University Children’ Hospital Zurich, Zurich, Switzerland

    Cornelia Hagmann

  9. Zurich Center for Spina Bifida, University Children’s Hospital Zurich, Zurich, Switzerland

    Luca Mazzone, Maya Horst, Sonja Schauer, David Alexander Wille & Beth Padden

  10. Children’s Research Center, University Children’s Hospital of Zurich, University of Zurich, Zurich, Switzerland

    Ueli Moehrlen, Luca Mazzone, Maya Horst, Sonja Schauer, David Alexander Wille, Cornelia Hagmann, Raimund Kottke, Patrice Grehten, Barbara Casanova, Theres Moehrlen, Sasha Tharakan, Beth Padden & Martin Meuli

  11. University of Zurich, Zurich, Switzerland

    Ueli Moehrlen, Nicole Ochsenbein, Dirk Bassler, Roland Zimmermann & Martin Meuli

Authors
  1. Ueli Moehrlen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nicole Ochsenbein
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ladina Vonzun
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Luca Mazzone
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Maya Horst
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Sonja Schauer
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. David Alexander Wille
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Cornelia Hagmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Raimund Kottke
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Patrice Grehten
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Barbara Casanova
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Nele Strübing
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Theres Moehrlen
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Sasha Tharakan
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Beth Padden
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Dirk Bassler
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Roland Zimmermann
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Martin Meuli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ueli Moehrlen.

Ethics declarations

Conflict of interest

None of the authors has any conflict of interest to declare.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moehrlen, U., Ochsenbein, N., Vonzun, L. et al. Fetal surgery for spina bifida in Zurich: results from 150 cases. Pediatr Surg Int 37, 311–316 (2021). https://doi.org/10.1007/s00383-020-04824-8

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-020-04824-8

Keywords

Search

Navigation