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
