Intraoperative neurophysiological monitoring in patients undergoing tethered cord surgery after fetal myelomeningocele repair

Clinical article

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  • 1 Department of Neurosurgery, Johns Hopkins University, Baltimore, Maryland;
  • | 2 Department of Neurosurgery, Drexel University, Philadelphia;
  • | 4 Department of Radiology,
  • | 7 Division of Neurosurgery, and
  • | 5 The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia;
  • | 6 Department of Neurosurgery, University of Pennsylvania, Philadelphia; and
  • | 3 Surgical Monitoring Associates, Bala Cynwyd, Pennsylvania
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Object

Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring.

Methods

The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord.

Results

During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level.

Conclusions

All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

Abbreviations used in this paper:

CMAP = compound muscle action potential; EMG = electromyography; IONM = intraoperative neurophysiological monitoring; MOMS = Management of Myelomeningocele Study; SSEP = somatosensory evoked potential; tcMEP = transcranial motor evoked potential.

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Contributor Notes

Address correspondence to: Gregory Heuer, M.D., Ph.D., Division of Neurosurgery, Children's Hospital of Philadelphia, Wood Center, 6th Floor, 34th and Civic Center Blvd., Philadelphia, PA 19104. email: heuerg@email.chop.edu.

Please include this information when citing this paper: published online February 7, 2014; DOI: 10.3171/2014.1.PEDS11336.

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