Phrenic Nerve Reconstruction in Pediatric Diaphragm Paralysis: Outcomes and Techniques

Abstract

Background

Symptomatic diaphragm paralysis in the pediatric patient is an uncommon condition. This study aims to evaluate the outcomes and technique of phrenic nerve reconstruction and its application to pediatric patients with unilateral and bilateral diaphragm paralysis at a single institution. The objective of this study is to demonstrate the application of a well-studied reconstructive technique in a population of patients not previously studied.

Methods

A retrospective review of pediatric patients between 2012 and 2022 with symptomatic diaphragm paralysis treated with phrenic nerve reconstruction. Nine patients with a median age of 10 years were offered surgical treatment. The etiology of their paralysis included: birth trauma, congenital cervical anomaly, mediastinal neoplastic disease, cervical spinal cord injury, and acute flaccid myelitis. Measures of postoperative improvement include: fluoroscopic sniff testing, pulmonary function testing, electromyography/nerve conduction testing, ultrasound evaluation, and ventilator requirements.

Results

One hundred percent of patients with unilateral paralysis demonstrated improvement, defined as improvements in dyspnea, orthopnea, fatigability, and decreased respiratory infections. This was corroborated by sniff testing, pulmonary function testing, and electrodiagnostic evaluation. In 80% of patients, there was recovery of diaphragm excursion on the chest fluoroscopy, and a 10% or greater improvement in pulmonary spirometry (forced expiratory volume in 1 second, FEV1, and forced vital capacity, FVC) percent predicted values. In patients with bilateral diaphragm paralysis, 75% demonstrated improvement in sniff testing, ultrasound findings, and ventilator requirements. One of four patients with bilateral paralysis and chronic ventilator dependency did not improve. There were no postoperative complications defined as hematoma, wound infection, pleural effusion, pneumonia, sepsis, nerve injury, or hardware malfunction seen during follow-up.

Conclusion

Phrenic nerve reconstruction in pediatric patients demonstrates potential as a safe and effective surgical option for symptomatic diaphragm paralysis. In patients with unilateral paralysis, this intervention consistently improved respiratory function. In patients with bilateral paralysis, the results were variable but showed promise in facilitating ventilator weaning when performed early. These findings underscore the importance of early surgical intervention. Larger, multicenter studies are needed to validate its long-term potential.

Keywords

diaphragm paralysis - phrenic nerve injury - diaphragm pacemaker - cervical tetraplegia - spinal cord injury

Publication History

Received: 23 May 2024

Accepted: 09 February 2025

Accepted Manuscript online:
11 February 2025

Article published online:
21 March 2025

• References

• 1 Brouillette RT, Hahn YS, Noah ZL, Ilbawi MN, Wessel HU. Successful reinnervation of the diaphragm after phrenic nerve transection. J Pediatr Surg 1986; 21 (01) 63-65
• 2 Akay TH, Ozkan S, Gultekin B. et al. Diaphragmatic paralysis after cardiac surgery in children: incidence, prognosis and surgical management. Pediatr Surg Int 2006; 22 (04) 341-346
• 3 Singh B, Arora S, Sandhu N. Emerging trends and insights in acute flaccid myelitis: a comprehensive review of neurologic manifestations. Infect Dis (Lond) 2023; 55 (10) 653-663
• 4 Massery M. Multisystem consequences of impaired breathing mechanics and/or postural control. In: Frownfelter D, Dean E. eds. Cardiovascular and Pulmonary Physical Therapy Evidence and practice. 4th ed.. St. Louis: Elsevier Health Sciences; 2006: 695-717
• 5 Janssens L, Brumagne S, Polspoel K, Troosters T, McConnell A. The effect of inspiratory muscles fatigue on postural control in people with and without recurrent low back pain. Spine 2010; 35 (10) 1088-1094
• 6 Schoeller T, Ohlbauer M, Wechselberger G, Piza-Katzer H, Margreiter R. Successful immediate phrenic nerve reconstruction during mediastinal tumor resection. J Thorac Cardiovasc Surg 2001; 122 (06) 1235-1237
• 7 Kaufman MR, Elkwood AI, Rose MI. et al. Reinnervation of the paralyzed diaphragm: application of nerve surgery techniques following unilateral phrenic nerve injury. Chest 2011; 140 (01) 191-197
• 8 Kaufman MR, Elkwood AI, Colicchio AR. et al. Functional restoration of diaphragmatic paralysis: an evaluation of phrenic nerve reconstruction. Ann Thorac Surg 2014; 97 (01) 260-266
• 9 Kaufman MR, Elkwood AI, Brown D. et al. Long-term follow-up after phrenic nerve reconstruction for diaphragmatic paralysis: a review of 180 patients. J Reconstr Microsurg 2017; 33 (01) 63-69
• 10 Kaufman MR, Chang EI, Bauer T. et al. Phrenic nerve reconstruction for effective surgical treatment of diaphragmatic paralysis. Ann Plast Surg 2021; 87 (03) 310-315
• 11 Gordon T, Tyreman N, Raji MA. The basis for diminished functional recovery after delayed peripheral nerve repair. J Neurosci 2011; 31 (14) 5325-5334
• 12 Krieger LM, Krieger AJ. The intercostal to phrenic nerve transfer: an effective means of reanimating the diaphragm in patients with high cervical spine injury. Plast Reconstr Surg 2000; 105 (04) 1255-1261
• 13 Kaufman MR, Elkwood AI, Aboharb F. et al. Diaphragmatic reinnervation in ventilator-dependent patients with cervical spinal cord injury and concomitant phrenic nerve lesions using simultaneous nerve transfers and implantable neurostimulators. J Reconstr Microsurg 2015; 31 (05) 391-395
• 14 Kaufman MR, Bauer T, Campbell S, Rossi K, Elkwood A, Jarrahy R. Prospective analysis of a surgical algorithm to achieve ventilator weaning in cervical tetraplegia. J Spinal Cord Med 2022; 45 (04) 531-535
• 15 Nath RK, Mackinnon SE. Nerve transfers in the upper extremity. Hand Clin 2000; 16 (01) 131-139 , ix
• 16 Lu GN, Flynn J. Facial nerve reconstruction. Otolaryngol Clin North Am 2023; 56 (04) 757-767
• 17 Millesi H. [Nerve transplantation for reconstruction of injured peripheral nerves by the use of microsurgical techniques]. Minerva Chir 1967; 22 (17) 950-951
• 18 Fraser III CD, Ravekes W, Thibault D. et al. Diaphragm paralysis after pediatric cardiac surgery: an STS congenital heart surgery database study. Ann Thorac Surg 2021; 112 (01) 139-146
• 19 Massery M. Physical therapy following phrenic nerve graft surgery: implications far beyond breathing. In: Elkwood A, Kaufman M, Schneider L. eds. Rehabilitative Surgery: A Comprehensive Text for an Emerging Field. Switzerland: Springer; 2017: 129-137
• 20 Callegari A, Neidenbach R, Milanesi O. et al. A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. Congenit Heart Dis 2019; 14 (02) 147-155