Abstract
Introduction
Acute Flaccid Myelitis (AFM) is a recently recognized, polio-like illness of children that can be functionally devastating. Severe cases can lead to ventilatory failure. Incomplete phrenic nerve injuries in other populations has been shown to respond to diaphragmatic stimulation. We therefore proposed an early assessment for incomplete denervation by laparoscopic direct stimulation of the diaphragm and placement of a diaphragmatic pacing system to enhance diaphragm function.
Case presentation
A 3 year-old girl presented with AFM with clinically and electrodiagnostically severe involvement of all four limbs and muscles of respiration. Direct stimulation of the diaphragm demonstrated contraction and a diaphragmatic stimulator was placed at 3 weeks post presentation. The patient was immediately able to tolerate short bouts of reduced ventilation settings. Electromyography via the pacing wires demonstrated intact motor units consistent with partial denervation/reinnervation in the left hemidiaphragm, and no motor units in the right hemidiaphragm. At three months, she tolerated 6 h of pacing on pressure support setting. At 5 months she demonstrated larger tidal volumes with active pacing than without.
Discussion
In our experience, AFM patients who require chronic ventilator support are rarely able to be weaned. Despite clinical and surface electrodiagnostic evidence of complete phrenic nerve involvement, the patient’s diaphragm responded to direct stimulation. The patient preferred pacing over non-pacing times and showed improved ventilatory ability with pacing as opposed to without, though remains ventilator-dependent. These findings support augmentation of diaphragm function and possible enhanced recovery of spontaneous function.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Gordon-Lipkin E, Muñoz LS, Klein JL, Dean J, Izbudak I, Pardo CA. Comparative quantitative clinical, neuroimaging, and functional profiles in children with acute flaccid myelitis at acute and convalescent stages of disease. Dev Med Child Neurol. 2018. https://doi.org/10.1111/dmcn.14030.
CDC. Acute Flaccid Myelitis | AFM Surveillance |. 2019. https://www.cdc.gov/acute-flaccid-myelitis/afm-surveillance.html.
Onders RP, Elmo MJ, Ignagni AR. Diaphragm pacing stimulation system for tetraplegia in individuals injured during childhood or adolescence. J Spinal Cord Med. 2007;30(Suppl 1):S25–9.
Dean JM, Onders RP, Elmo MJ. Diaphragm pacers in pediatric patients with cervical spinal cord injury: a review and implications for inpatient rehabilitation. Curr Phys Med Rehabil Rep 2018;6:257–63.
Onders RP, Elmo M, Kaplan C, Katirji B, Schilz R. Extended use of diaphragm pacing in patients with unilateral or bilateral diaphragm dysfunction: a new therapeutic option. Surgery. 2014;156:776–84.
Onders RP, Elmo M, Kaplan C, Schilz R, Katirji B, Tinkoff G. Long-term experience with diaphragm pacing for traumatic spinal cord injury: early implantation should be considered. Surgery. 2018;164:705–11.
Kerwin AJ, Yorkgitis BK, Ebler DJ, Madbak FG, Hsu AT, Crandall ML. Use of diaphragm pacing in the management of acute cervical spinal cord injury. J Trauma Acute Care Surg. 2018;85:928–31.
Ayscue P, Van Haren K, Sheriff H, Waubant E, Waldron P, Yagi S, et al. Acute flaccid paralysis with anterior myelitis—California, June 2012–June 2014. MMWR Morb Mortal Wkly Rep. 2014;63:903–6. 10
Onders RP, Ponsky TA, Elmo M, Lidsky K, Barksdale E. First reported experience with intramuscular diaphragm pacing in replacing positive pressure mechanical ventilators in children. J Pedia Surg. 2011;46:72–6.
Al-Majed AA, Neumann CM, Brushart TM, Gordon T. Brief electrical stimulation promotes the speed and accuracy of motor axonal regeneration. J Neurosci. 2000;20:2602–8.
Langer JC, Filler RM, Coles J, Edmonds JF. Plication of the diaphragm for infants and young children with phrenic nerve palsy. J Pedia Surg. 1988;23:749–51.
Baker CJ, Boulom V, Reemtsen BL, Rollins RC, Starnes VA, Wells WJ. Hemidiaphragm plication after repair of congenital heart defects in children: quantitative return of diaphragm function over time. J Thorac Cardiovasc Surg. 2008;135:56–61.
Latreille J, Lindholm EB, Zlotolow DA, Grewal H. Thoracoscopic intercostal to phrenic nerve transfer for diaphragmatic reanimation in a child with tetraplegia. J Spinal Cord Med. 2019. https://doi.org/10.1080/10790268.2019.1585706 [Epub ahead of print].
Acknowledgements
Janet M. Dean, MS, RN, CRRN, CRNP for expertise and clinical management. Suzanne Venoski Rybczynski, MD for expertise and clinical management. Michelle Melicosta, MD, MPH, FAAP for expertise and clinical management. Thomas Owen Crawford, M.D. for expertise and electrodiagnostics
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher’s note: Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Edmiston, T.L., Elrick, M.J., Kovler, M.L. et al. Early use of an implantable diaphragm pacing stimulator for a child with severe acute flaccid myelitis—a case report. Spinal Cord Ser Cases 5, 67 (2019). https://doi.org/10.1038/s41394-019-0207-7
Received:
Revised:
Accepted:
Published:
DOI: https://doi.org/10.1038/s41394-019-0207-7
