Top

Published in:

01-03-2021 | Spina Bifida | Original Article

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

Authors: Ueli Moehrlen, Nicole Ochsenbein, Ladina Vonzun, Luca Mazzone, Maya Horst, Sonja Schauer, David Alexander Wille, Cornelia Hagmann, Raimund Kottke, Patrice Grehten, Barbara Casanova, Nele Strübing, Theres Moehrlen, Sasha Tharakan, Beth Padden, Dirk Bassler, Roland Zimmermann, Martin Meuli

Published in: Pediatric Surgery International | Issue 3/2021

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.

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):701–712CrossRef

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):1028–1032CrossRef

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

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):397–402CrossRef

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

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):1819–1825CrossRef

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):482–487CrossRef

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):1826–1831CrossRef

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):993–1004CrossRef

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):91–97CrossRef

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):494–498PubMed

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):205–213CrossRef

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

14.

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

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):593–597CrossRef

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):834–839CrossRef

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):108–114CrossRef

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.e268CrossRef

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):235–240CrossRef

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):1409–1417CrossRef

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):1157–1168CrossRef

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):38–44CrossRef

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):1651–1658CrossRef

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):e906-913CrossRef

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):812–818CrossRef

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):211–218CrossRef

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):882–888CrossRef

28.

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

29.

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

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):e20191544CrossRef

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):47–53CrossRef

32.

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

Title: Fetal surgery for spina bifida in Zurich: results from 150 cases
Authors: Ueli Moehrlen
Nicole Ochsenbein
Ladina Vonzun
Luca Mazzone
Maya Horst
Sonja Schauer
David Alexander Wille
Cornelia Hagmann
Raimund Kottke
Patrice Grehten
Barbara Casanova
Nele Strübing
Theres Moehrlen
Sasha Tharakan
Beth Padden
Dirk Bassler
Roland Zimmermann
Martin Meuli
Publication date: 01-03-2021
Publisher: Springer Berlin Heidelberg
Keywords: Spina Bifida
Spina Bifida
Published in: Pediatric Surgery International / Issue 3/2021
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-020-04824-8

At a glance: The STEP trials

Springer Medicine

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

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 3/2021

Telemedicine in the pediatric surgery in Germany during the COVID-19 pandemic

Doppler ultrasound assessment of splanchnic perfusion and heart rate for the detection of necrotizing enterocolitis

The 33rd international symposium on pediatric surgical research

Thoracoscopic oesophageal atresia/tracheo-oesophageal fistula (OA/TOF) repair is associated with a higher stricture rate: a single institution’s experience

Remote ischemic conditioning avoids the development of intestinal damage after ischemia reperfusion by reducing intestinal inflammation and increasing intestinal regeneration

Early enteral feeding after intestinal anastomosis in children: a systematic review and meta-analysis of randomized controlled trials