Diaphragmatic Pacing in Pediatrics, Professional Edition

Mechanical ventilation is a life saving measure. Provision of respiration to produce respiratory effects in the body is a necessary treatment but it can have complications. In a 2010 research study by Principi, et al., 150 pediatric patients with varying diagnoses were followed over ten months. In these patients, 40% or 60 children had complications. These included:

• Atelectasis 16.7% (left lower lobe 36%, right upper lobe 26%)
• Atelectasis in children under one year old, 12%, over one year old 18%
• Post-extubation stridor 13.3%
• Failed extubation 9.3%
• Accidental extubation 3.3%
• Nasal or perioral tissue damage, 2.7%
• Pneumothorax, 2%
• Ventilatory associated pneumonia 1.9%

Long term mechanical ventilation in children with spinal cord injury is associated with complications that are physiological and mechanical. Over time, the diaphragm, which is a muscle, becomes atrophied from lack of use or ventilator induced diaphragm dysfunction (VIDD). Pneumothorax is a common complication for long term mechanical ventilation. Other physical complications include bronchopleural fistula, tracheal wound expansion, pneumonia, and loss of taste. Speaking and speech development is affected by the movement of air by the ventilator. Concerns about power outages require home generators to be used in emergencies or manual ventilation with an ambu bag. Quality of life is affected by concerns about accidental disconnections which can occur while doing activities of daily living or play. It can be challenging to find individuals who will be willing to provide care.

An alternative to mechanical ventilation can be found with diaphragmatic pacing. This process is being used in the pediatric population for infants and children that lack or have diminished breathing. Diaphragmatic pacing is used in pediatric patients who have Congenital Central Hypoventilation Syndrome (CCHS), a neurological condition that affects breathing, high level spinal cord injury, brain injury, amyotrophic lateral sclerosis, acute flaccid myelitis, COVID, and other conditions that affect the ability to breathe. It is used both temporarily and long term. If a child is currently utilizing mechanical ventilation, they can be evaluated to switch to diaphragmatic pacing. Quality of life improves. However, services such as night care may be eliminated by the payor.

Completely different mechanisms of operation are the foundations between mechanical ventilation and diaphragmatic pacing. In mechanical ventilation, air is forced into the lungs by use of an external machine. In diaphragmatic pacing, electrodes are placed on the diaphragm (occasional on the phrenic nerve) using minimally invasive surgical technique. The diaphragm is electrically stimulated to bring air into the body. This takes advantage of the natural human function of breathing by using the anatomy and physiology of the body that are designed to perform these functions. It carefully stimulates natural breathing.

There are two types of diaphragmatic pacers. In the conventional type, an internal electrode is attached to the phrenic nerve in the neck, chest, or diaphragm. Under the skin is a receiver that is controlled by an external transmitter. The Diaphragmatic Pacing System (DPS) has four electrodes which are placed directly on the diaphragm. A fifth electrode is implanted under the skin for grounding. A socket exiting the skin is used as an external pulse generator. It connects to an external, removeable cable.

There are distinct advantages to diaphragmatic pacing in the pediatric population. Depending on the situation, some of the pre-testing is not needed which makes implantation faster without extended use of mechanical ventilation. There are fewer physical complications as air is not forced into the body but rather the diaphragm draws air in. A tracheostomy is often not necessary. Speech has a regular flow. The child can usually feed, with a sense of taste, using breast, bottle, or solid food depending on their development. There is no ‘tube’ to work around that restricts movement which can benefit development. Children with diaphragmatic pacers do not have alterations to their appearance as with a tracheostomy tube.

If the phrenic nerve is not functioning as in the case of injury to the lower motor neurons, nerve transfers can assist with the stimulation by the diaphragmatic pacer. An example of this is use of an intercostal nerve to phrenic nerve transfer that was performed in a five-year-old child with tetraplegia. This nerve transfer enabled innervation for diaphragmatic pacing (Latreille, et al, 2021).

There is evidence that in some cases, the phrenic nerve can enhance recovery through stimulation of the phrenic nerve over time (Edmiston, et al, 2019). As the diaphragm is working in a physiological way and there is no artificial body opening to the airway, life span may be enhanced.

Surgical complications of diaphragmatic pacing include usual surgical issues of infection, nerve damage, bleeding or poor healing. The equipment can fail, become dislodged, internal wires could possibly break which would require a correction surgery. But these complications have been few.

Some individuals may have concerns about multiple stimulations devices being implanted or utilized with one implanted and another on the surface of the skin. However, an example of overcoming this issue was demonstrated in a study led by Khan, et al, 2022 where a cardiac pacer and diaphragmatic pacer were both used in a 17-year-old individual. The devices were successfully adjusted by changing modes during adjustments creating the ability for use of both pacers.

Diaphragmatic pacers are FDA approved. These devices are no longer classified as experimental however, research continues to refine and improve both the device and surgical techniques as with all medical procedures and equipment. The devices are available anywhere in the U.S. and in many other countries. They are being implanted by pulmonary surgeons but have been extensively used by trauma surgeons who are implanting as soon as possible because the complications are so much less than mechanical ventilation. This allows children to be moved from intensive care to rehabilitation to home much quicker. Temporary use is also assisting in faster recoveries.

Use of diaphragmatic pacers is becoming mainstream. Eventually, they will likely take the place of mechanical ventilation. The use of these devices is changing healthcare.

Written by Linda M. Schultz, PhD, CRRN.

References and Further Reading

Edmiston TL, Elrick MJ, Kovler ML, Jelin EB, Onders RP, Sadowsky CL. Early Use of an Implantable Diaphragm Pacing Stimulator for a Child with Severe Acute Flaccid Myelitis–A Case Report. Spinal Cord Ser Cases. 2019 Jul 17;5: 67. doi: 10.1038/s41394-019-0207-7. PMID: 31632725; PMCID: PMC6786381.

Khan MS, Hoyt W Jr, Snyder C. Minimizing Device-Device Interactions Using Bipolar Pacemaker Leads in a Pediatric Patient. Pediatr Cardiol. 2022 Jan 13. doi: 10.1007/s00246-022-02816-0. Epub ahead of print. PMID: 35024901.

Kasi AS, Perez IA, Kun SS, Keens TG. Congenital Central Hypoventilation Syndrome: Diagnostic and Management Challenges. Pediatric Health Med Ther. 2016 Aug 18;7: 99-107. doi: 10.2147/PHMT.S95054. PMID: 29388615; PMCID: PMC5683295.

Principi T, Fraser DD, Morrison GC, Farsi SA, Carrelas JF, Maurice EA, Kornecki A. Complications of Mechanical Ventilation in the Pediatric Population. Pediatr Pulmonol. 2011 May;46(5):452-7. doi: 10.1002/ppul.21389. Epub 2010 Dec 30. PMID: 21194139.

Hazwani TR, Alotaibi B, Alqahtani W, Awadalla A, Shehri AA. Pediatric Diaphragmatic Pacing. Pediatr Rep. 2019;11(1):7973. Published 2019 Mar 11. doi:10.4081/pr.2019.7973

Kaufman MR, Bauer T, Onders RP, Brown DP, Chang EI, Rossi K, Elkwood AI, Paulin E, Jarrahy R. Treatment for Bilateral Diaphragmatic Dysfunction Using Phrenic Nerve Reconstruction and Diaphragm Pacemakers. Interact Cardiovasc Thorac Surg. 2021 May 10;32(5):753-760. doi: 10.1093/icvts/ivaa324. PMID: 33432336; PMCID: PMC8691533.

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. 2021 May;44(3):425-428. doi: 10.1080/10790268.2019.1585706. Epub 2019 Mar 18. PMID: 30883296; PMCID: PMC8081323.

Onders RP Diaphragm Pacing in Pediatric SCI and Patients with COVID-19. Oral presentation at: Steel Assembly, Annual Meeting, Orlando, Florida, 2021.

Onders RP. Functional Electrical Stimulation: Restoration of Respiratory Function. Handb Clin Neurol. 2012;109:275-82. doi: 10.1016/B978-0-444-52137-8.00017-6. PMID: 23098719.

Onders RP, Elmo M, Stepien C, Katirji B. Spinal Cord Injury Level and Phrenic Nerve Conduction Studies Do Not Predict Diaphragm Pacing Success or Failure–All Patients Should Undergo Diagnostic Laparoscopy. Am J Surg. 2021 Mar;221(3):585-588. doi: 10.1016/j.amjsurg.2020.11.042. Epub 2020 Nov 22. PMID: 33243416.

Onders RP, Khansarinia S, Ingvarsson PE, Road J, Yee J, Dunkin B, Ignagni AR. Diaphragm Pacing in Spinal Cord Injury Can Significantly Decrease Mechanical Ventilation in Multicenter Prospective Evaluation. Artif Organs. 2022 Feb 28. doi: 10.1111/aor.14221. Epub ahead of print. PMID: 35226374.

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 Oct;164(4):705-711. doi: 10.1016/j.surg.2018.06.050. Epub 2018 Sep 6. PMID: 30195400.

Smith BK, Fuller DD, Martin AD, Lottenberg L, Islam S, Lawson LA, Onders RP, Byrne BJ. Diaphragm Pacing as a Rehabilitative Tool for Patients with Pompe Disease Who Are Ventilator-Dependent: Case Series. Phys Ther. 2016 May;96(5):696-703. doi: 10.2522/ptj.20150122. Epub 2016 Feb 18. PMID: 26893511; PMCID: PMC4858660.

Weese-Mayer DE, Silvestri JM, Kenny AS, Ilbawi MN, Hauptman SA, Lipton JW, Talonen PP, Garcia HG, Watt JW, Exner G, Baer GA, Elefteriades JA, Peruzzi WT, Alex CG, Harlid R, Vincken W, Davis GM, Decramer M, Kuenzle C, Saeterhaug A, Schöber JG. Diaphragm Pacing with a Quadripolar Phrenic Nerve Electrode: An International Study. Pacing Clin Electrophysiol. 1996 Sep;19(9):1311-9. doi: 10.1111/j.1540-8159.1996.tb04209.x. PMID: 8880794.