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In spina bifida aperta, muscle ultrasound can quantify the “second hit of damage”

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Abstract

Purpose

In spina bifida aperta (SBA), the “second-hit hypothesis” addresses consequences by delayed neurological damage superimposed upon the congenital myelomeningocele (MMC). This secondary damage is postulated to underlie the disappearance of leg movements shortly after birth. Innovative fetal surgery might prevent this, but results are methodologically hard to prove in small and heterogeneous treatment groups. We reasoned that delayed postnatal alterations in muscle ultrasound density (MUD = muscle echogenicity) could quantitatively reflect consequences by “the second hit” of damage. In the present study, we investigated whether delayed postnatal leg-MUD alterations are associated with postnatal muscle function loss.

Methods

We cross-sectionally assessed leg-MUD in 16 postnatally operated SBA children (MMC-L5; at 0, 6, and 12 months; in n = 11/16; 11/16, and 15/16 children, respectively) and compared outcomes with 13 healthy control children. Additionally, we assessed SBA MUD caudal and cranial to the MMC and calculated MMC-L5 impact by: dMUD(MMC-L5) = [MUDcalf muscle/S1–2] − [MUDquadriceps muscle/L2–4] and associated outcomes with leg muscle function caudal to the MMC.

Results

At 0 month, clinically discernible dMUD was more often increased in SBA than in control newborns (p < .05), but a relationship between absolute quantitative differences and leg muscle dysfunction was still lacking. At 6–12 months, additionally increased dMUD outcomes coincided with SBA leg muscle dysfunction (p < .05).

Conclusions

In post-neonatal SBA, secondarily increased dMUD (i.e., MMC impact) coincides with leg muscle dysfunction. This may implicate that muscle ultrasound could provide a quantitative tool to assess the neuromuscular impact by the second hit of damage.

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Notes

  1. Leg-dMUD in SBA vs controls [0 months, 3 (−15 to 29) vs 0 (−5 to 10); 6 months, 22 (−6 to 40) vs 11 (−6 to 19); and 12 months, 18 (1–39) vs 8 (−6–13); medians (ranges)]

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Acknowledgments

The authors would like to thank all infants and parents who cooperated in this study. We wish to thank P.B. Mulder (muscle ultrasonographer) for recruiting healthy pregnant women. We thank A.L. Staal-Schreinemacher for the clinical information and K.M. Sollie for her help and support at the obstetrics department. We thank M. Gremmer for the availability of the ultrasound equipment and H. Kunst, J. Bijmolt, J. Sikkema, G. Oosterhof, H. Hooijsma, and M. Luursema for their administrative help.

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Neurology, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700RB, Groningen, the Netherlands

    R. J. Verbeek, J. H. van der Hoeven, N. M. Maurits & O. F. Brouwer

  2. Department of Neurosurgery, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700RB, Groningen, the Netherlands

    E. W. Hoving

  3. Department of Pediatrics, Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700RB, Groningen, the Netherlands

    D. A. Sival

Authors
  1. R. J. Verbeek
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  2. J. H. van der Hoeven
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  3. N. M. Maurits
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  4. O. F. Brouwer
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  5. E. W. Hoving
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  6. D. A. Sival
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Corresponding author

Correspondence to D. A. Sival.

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Verbeek, R.J., van der Hoeven, J.H., Maurits, N.M. et al. In spina bifida aperta, muscle ultrasound can quantify the “second hit of damage”. Childs Nerv Syst 29, 469–474 (2013). https://doi.org/10.1007/s00381-012-1947-x

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  • DOI: https://doi.org/10.1007/s00381-012-1947-x

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