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Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI

  • Magnetic Resonance
  • Published:
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Abstract

Objectives

To describe characteristics of foetuses undergoing in utero repair of open neural tube defects (ONTD) and assess postoperative evolution of posterior fossa and brain morphology.

Methods

Analysis of pre- and postoperative foetal as well as neonatal MRI of 27 foetuses who underwent in utero repair of ONTD. Type and level of ONTD, hindbrain configuration, posterior fossa and liquor space dimensions, and detection of associated findings were compared between MRI studies and to age-matched controls.

Results

Level of bony spinal defect was defined with exactness of ± one vertebral body. Of surgically confirmed 18 myelomeningoceles (MMC) and 9 myeloschisis (MS), 3 MMC were misdiagnosed as MS due to non-visualisation of a flat membrane on MRI. Hindbrain herniation was more severe in MS than MMC (p < 0.001). After repair, hindbrain herniation resolved in 25/27 cases at 4 weeks and liquor spaces increased. While posterior fossa remained small (p < 0.001), its configuration normalised. Lateral ventricle diameter indexed to cerebral width decreased in 48% and increased in 12% of cases, implying a low rate of progressive obstructive hydrocephalus. Neonatally evident subependymal heterotopias were detected in 33% at preoperative and 50% at postoperative foetal MRI.

Conclusion

MRI demonstrates change of Chiari malformation type II (CM-II) features.

Key Points

• Hindbrain herniation is significantly more pronounced in myeloschisis than in myelomeningocele

• Resolution of hindbrain herniation 4 weeks after in utero closure of ONTD

• MRI is valuable for preoperative assessment and postoperative evaluation following in utero repair

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Abbreviations

bBPD:

Bony biparietal diameter

cBPD:

Cerebral biparietal diameter

CM-II:

Chiari malformation type II

CSF:

Cerebrospinal fluid

CSOA:

Clivus-supraocciput angle

GA:

Gestational age

MMC:

Myelomeningocele

MOMS:

Management of myelomeningocele study

MRI:

Magnetic resonance imaging

MS:

Myeloschisis

ONTD:

Open neural tube defect

PF:

Posterior fossa

PFA:

Posterior fossa area

PFV:

Posterior fossa volume

TDPF:

Transverse diameter of posterior fossa

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Authors and Affiliations

  1. Department of Diagnostic Imaging, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Christin Rethmann, Ianina Scheer & Christian Johannes Kellenberger

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

    Christin Rethmann, Ianina Scheer, Martin Meuli, Luca Mazzone, Ueli Moehrlen & Christian Johannes Kellenberger

  3. Children’s Research Center (CRC), Zurich, Switzerland

    Christin Rethmann, Ianina Scheer, Martin Meuli, Luca Mazzone, Ueli Moehrlen & Christian Johannes Kellenberger

  4. Department of Pediatric Surgery, University Children’s Hospital Zurich, Zurich, Switzerland

    Martin Meuli, Luca Mazzone & Ueli Moehrlen

Authors
  1. Christin Rethmann
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  2. Ianina Scheer
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  3. Martin Meuli
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  4. Luca Mazzone
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  5. Ueli Moehrlen
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  6. Christian Johannes Kellenberger
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Corresponding author

Correspondence to Christin Rethmann.

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Guarantor

The scientific guarantor of this publication is Christin Rethmann.

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Funding

The authors state that this work has not received any funding.

Statistics and biometry

One of the authors has significant statistical expertise.

No complex statistical methods were necessary for this paper.

Ethical approval

Institutional review board approval was obtained.

Informed consent

Written informed consent was obtained from all subjects (patients) in this study.

Study subjects or cohorts overlap

Some study subjects or cohorts have been previously reported in

1. Premiere use of Integra™ artificial skin to close an extensive fetal skin defect during open in utero repair of myelomeningocele. Meuli M, Meuli-Simmen C, Flake AW, Zimmermann R, Ochsenbein N, Scheer I, Mazzone L, Moehrlen U. Pediatr Surg Int. 2013 Dec;29(12):1321–6. doi: 10.1007/s00383-013-3412-7. (one patient, case report)

2. Fetal surgery in Zurich: key features of our first open in utero repair of myelomeningocele. Meuli M, Moehrlen U, Flake A, Ochsenbein N, Huesler M, Biro P, Scheer I, Tharakan S, Dürig P, Zimmermann R. Eur J Pediatr Surg. 2013 Dec;23(6):494–8. doi: 10.1055/s-0032-1329700. No abstract available. (1 patient, case report)

3. Prenatal myelomeningocele repair: Do bladders better? Horst M, Mazzone L, Schraner T, Bodmer C, Möhrlen U, Meuli M, Gobet R. Neurourol Urodyn. 2016 Nov 15. doi: 10.1002/nau.23174. [Epub ahead of print] PMID: 27862250 (all 27 patients)

Methodology

retrospective

diagnostic or prognostic study / observational

performed at one institution

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Rethmann, C., Scheer, I., Meuli, M. et al. Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI. Eur Radiol 27, 4571–4580 (2017). https://doi.org/10.1007/s00330-017-4807-y

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  • DOI: https://doi.org/10.1007/s00330-017-4807-y

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