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Tethered cord release in patients after open fetal myelomeningocele closure: intraoperative neuromonitoring data and patient outcomes

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Abstract

Purpose

The purpose of the study was to better understand the clinical course and impact of tethered cord release surgery on patients who have previously undergone open spinal dysraphism closure in utero.

Methods

This is a single-center retrospective observational study on patients undergoing tethered cord release after having previously had open fetal myelomeningocele (MMC) closure. All patients underwent tethered cord release surgery with a single neurosurgeon. A detailed analysis of the patients’ preoperative presentation, intraoperative neuromonitoring (IONM) data, and postoperative course was performed.

Results

From 2009 to 2021, 51 patients who had previously undergone fetal MMC closure had tethered cord release surgery performed. On both preoperative and postoperative manual motor testing, patients were found to have on average 2 levels better than would be expected from the determined anatomic level from fetal imaging. The electrophysiologic functional level was found on average to be 2.5 levels better than the anatomical fetal level. Postoperative motor levels when tested on average at 4 months were largely unchanged when compared to preoperative levels. Unlike the motor signals, 46 (90%) of patients had unreliable or undetectable lower extremity somatosensory evoked potentials (SSEPs) prior to the tethered cord release.

Conclusion

Tethered cord surgery can be safely performed in patients after open fetal MMC closure without clinical decline in manual motor testing. Patients often have functional nerve roots below the anatomic level. Sensory function appears to be more severely affected in patients leading to a consistent motor-sensory imbalance.

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Availability of data and material

The results/data/figures in this manuscript have not been published elsewhere, nor are they under consideration (from any of the authors) by another publisher.

Abbreviations

CHOP:

Children’s Hospital of Philadelphia

EEG:

Electroencephalogram

EMG:

Electromyography

IONM:

Intraoperative neuromonitoring

MOMS:

Management of Myelomeningocele Study

MMC:

Myelomeningocele

SSEPs:

Somatosensory evoked potentials

TCS:

Tethered cord syndrome

TIVA:

Total intravenous anesthetic

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Acknowledgements

The authors would like to acknowledge: Jesse A. Taylor, MD, and Jordan W. Swanson, MD, from the Division of Plastic Surgery at the Children’s Hospital of Philadelphia for complex wound closure in these patients; Beverly G. Coleman, MD, and Deborah M. Zarnow, MD, from the Department of Radiology at the Children’s Hospital of Philadelphia for their neuroradiological interpretation of the imaging in these patients; and Katie M. Schmidt, MSN, CRNP, from the Center for Fetal Diagnosis and Treatment as well as the Spina Bifida Program at the Children’s Hospital of Philadelphia for her invaluable coordination of fetal myelomeningocele patients.

Funding

The authors did not receive funding for this project.

Author information

Authors and Affiliations

  1. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Tracy M. Flanders, Kristen L. Lincul, Samuel R. Pierce, Edward R. Oliver, Julie S. Moldenhauer, N. Scott Adzick & Gregory G. Heuer

  2. SpecialtyCare, Nashville, TN, USA

    Alier J. Franco

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  1. Tracy M. Flanders
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Contributions

Tracy M. Flanders collected and analyzed the data, wrote the main manuscript text, prepared Tables 1–2 and Figs. 1–4, and prepared the manuscript for submission. Alier J.Franco collected and analyzed the intraoperative neuromonitoring data, wrote the manuscript text on intraoperative neuromonitoring, and prepared Fig. 5. Kristen L. Lincul and Samuel R. Pierce performed the preoperative and postoperative physical exams and collected the data. Edward R. Oliver reviewed the neuroradiologic images and collected the data. Julie S. Moldenhauer. and N. Scott Adzick. wrote the manuscript text. Gregory G. Heuer performed all the surgeries, collected the data, and wrote the manuscript text. All authors edited and reviewed the manuscript prior to submission.

Corresponding author

Correspondence to Tracy M. Flanders.

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The authors consent to publication in the journal Child’s Nervous System.

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The authors have no competing interests as defined by Springer, or other interests that might be perceived to influence the results and/or discussion reported in this paper.

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An abstract with preliminary data was presented at the AANS/CNS Section on Pediatric Neurosurgery’s 50th Annual Meeting in Arizona in December 2021. The abstract for this manuscript will be presented at the International Society of Pediatric Neurosurgery’s 48th Annual Meeting in Singapore this December 2022.

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Flanders, T.M., Franco, A.J., Lincul, K.L. et al. Tethered cord release in patients after open fetal myelomeningocele closure: intraoperative neuromonitoring data and patient outcomes. Childs Nerv Syst 39, 663–670 (2023). https://doi.org/10.1007/s00381-022-05756-5

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