Early use of an implantable diaphragm pacing stimulator for a child with severe acute flaccid myelitis—a case report

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.

Acute flaccid myelitis: cause, diagnosis, and management

“…21,22,85 Muscle or tendon transfer, or both, has been reported in a few cases, with generally positive outcomes for restoration of elbow or hand function. 21,85 Anecdotal reports indicate that lower limb nerve transfers, nerve transfer to the phrenic nerve, and diaphragmatic pacing have been undertaken in individual cases; 86 however, there are few published data regarding outcomes in these cases.…”

Acute Flaccid Myelitis: Review of Clinical Features, Diagnosis, and Management with Nerve Transfers

“…45 Diaphragm pacing stimulator placement has demonstrated some benefit for patient respiratory function in terms of decreased ventilator settings and ventilator dependence but has yet to be successful in weaning patients off mechanical ventilation. 22,64…”

“…After removal of duplicates and initial screening, 500 were reviewed and assessed for eligibility. After application of inclusion and exclusion criteria, 99 articles were included in this study (55 case reports/series, 1,4–7,9–57 33 retrospective cohort studies, 2,3,58–88 seven cross-sectional studies, 89–95 and four case-control studies 96–99 ) (Fig. 1).…”

Diaphragm pacing and independent breathing in individuals with severe Pompe disease

“…Additionally, DP for SCI is associated with a reduced risk of respiratory infections ( 47 ) and improved quality of life ( 32 ). Beyond SCI, reports in congenital central hypoventilation syndrome ( 48 ), acute flaccid myelitis ( 49 ), and other neurological conditions ( 50 ) indicate off-label clinical use of DP can be implemented safely and associated with relatively few adverse effects ( 31 ).…”