Diaphragmatic Paralysis Treatment & Management
- Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Ryland P Byrd, Jr, MD more...
Unilateral diaphragmatic paralysis
Most patients with unilateral diaphragmatic paralysis are asymptomatic and do not require treatment. If the underlying causes are discivered, they can be treated. Even when the etiology is not known, many times paralysis resolves on its own, albeit slowly over a period of months to more than a year. In a select group of patients with unilateral diaphragmatic paralysis who have severe dyspnea upon excursion, surgical treatment has been shown to be beneficial.
Stabilization from surgical plication of the paralyzed diaphragm provides good results in selected patients. Following plication, the paralyzed diaphragm does not paradoxically move cephalad into the thorax during inspiration and, therefore, improves ventilation to the affected site. Furthermore, the procedure also favors the healthy diaphragm, which now performs less work.
In a select group of patients, diaphragmatic plication decreased breathlessness, improved vital capacity by 10-20%, and improved PaO2 by 10%. In one surgical series, the mean forced tidal volume improved dramatically from 216 mL to 415 mL after plication and it was possible to discontinue mechanical ventilation within 2-12 days of plication. Functional and physiologic results of diaphragm plication have been shown to endure over long-term follow-up. In another study, 41 patients underwent plication of the hemidiaphragm. Patients were followed up for at least 48 months. Mean forced vital capacity, forced expiratory volume at 1 second, functional residual capacity, and total lung capacity all improved by 17%, 21%, 20%, and 20% (P < .005), respectively, at 48 months. These mean values had remained constant when compared with the 6-month follow-up.
Plication of the diaphragm can be performed using a number of techniques through a thoracotomy, video-assisted thoracoscopic surgery (VATS), or laparoscopy. The VATS approach can have similar results as the thoracotomy series, with fewer complications.
A common relative contraindication to plication is morbid obesity, as surgical plication is technically more difficult in these patients. This group of patients should be evaluated for bariatric surgery and may be able to avoid plication with improvement of pulmonary function after significant weight loss. Patients with certain neuromuscular disorders (ie, amyotrophic lateral sclerosis and muscular dystrophy) should be approached with caution as plication provides only modest benefit with more complications.
Bilateral diaphragmatic paralysis
The treatment of bilateral diaphragmatic paralysis mainly depends on the etiology and severity of the paralysis. Invasive ventilation was historically the main treatment for patients who developed respiratory failure as a result of bilateral diaphragmatic paralysis. Later, a subset of these patients who did not have intrinsic lung pathology became candidates for noninvasive ventilation.
Currently phrenic pacing is increasingly being employed in patients with central respiratory paralysis and upper cervical spinal cord injury (lesions above C3) to wean them off the ventilators. These patients ideally should not have any intrinsic lung disease. Electrodes can be implanted intrathoracically via thoracotomy and, more recently, with VATS. Alternatively, electrodes can be placed intramuscularly via a laparoscopic approach. In this approach, intramuscular electrodes are placed near the entrance points of the phrenic nerves using motor-point mapping techniques.
Diaphragm pacing allows patients to speak again and use their olfaction system. It reduces the occurrence of respiratory infections, provides more natural breathing, and avoids dependency on a mechanical ventilator. The phrenic nerve should be tested with a phrenic nerve conduction study before planning for diaphragmatic pacing. Deconditioning and atrophy of the diaphragm prior to pacing is the main limiting factor in weaning patients off the ventilators.
Negative-pressure systems may induce obstruction of the upper airway, particularly if the upper airway dilators are weak and unable to counteract the negative pressure generated by the ventilator. Therefore, sleep studies are required for patients who are being considered for negative-pressure ventilation. Consideration of positive-pressure ventilation lessens the need for screening sleep studies.
Most patients with mild-to-moderate diaphragmatic weakness maintain daytime gas exchange but worsen during sleep. Sleep studies and ventilatory-assist device treatments may identify this condition. Nighttime noninvasive ventilation could be used in this group of patients.
Patients in whom nasal or oral positive-pressure ventilation is unsuccessful may need other forms of noninvasive ventilation (eg, negative-pressure cuirass, pulmonary wrap, rocking bed, positive-pressure pneumobelt).
Tracheostomy with positive-pressure intermittent or permanent ventilation is reserved for patients who are not candidates for less invasive methods or in whom less invasive methods fail.
Nerve reconstruction techniques
In a select group of patients, nerve surgery may be used to restore function to the paralyzed hemidiaphragm.[18, 19] Neurolysis, nerve grafting, and neurotization have demonstrated promise in returning function to unilateral phrenic nerve injury that occurred as a result of anesthetic procedures and operative and nonoperative trauma to the neck. With microscopic neurolysis, fibrous tissue from the compressed portion of the phrenic nerve is removed.
Inspiratory muscle strength and endurance training
Daily inspiratory muscle strength and endurance training can lead to increased nondiaphragmatic inspiratory muscle recruitment and help those with mild symptoms from diaphragmatic paralysis.
Kumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clin Proc. 2004 Dec. 79(12):1563-5. [Medline].
Maish MS. The diaphragm. Surg Clin North Am. 2010 Oct. 90(5):955-68. [Medline].
Summerhill EM, El-Sameed YA, Glidden TJ, McCool FD. Monitoring recovery from diaphragm paralysis with ultrasound. Chest. 2008 Mar. 133(3):737-43. [Medline].
Groth SS, Andrade RS. Diaphragm plication for eventration or paralysis: a review of the literature. Ann Thorac Surg. 2010 Jun. 89(6):S2146-50. [Medline].
Han KY, Bang HJ. Exercise therapy for a patient with persistent dyspnea after combined traumatic diaphragmatic rupture and phrenic nerve injury: A case report. PM R. 2014 Nov 20. [Medline].
Weiss C, Witt T, Grau S, Tonn JC. Hemidiaphragmatic paralysis with recurrent lung infections due to degenerative motor root compression of C3 and C4. Acta Neurochir (Wien). 2011 Mar. 153(3):597-9. [Medline].
Ulku R, Onat S, Balci A, Eren N. Phrenic nerve injury after blunt trauma. Int Surg. 2005 Apr-Jun. 90(2):93-5. [Medline].
Gierada DS, Slone RM, Fleishman MJ. Imaging evaluation of the diaphragm. Chest Surg Clin N Am. 1998 May. 8(2):237-80. [Medline].
Miller JM, Moxham J, Green M. The maximal sniff in the assessment of diaphragm function in man. Clin Sci (Lond). 1985 Jul. 69(1):91-6. [Medline].
Lloyd T, Tang YM, Benson MD, King S. Diaphragmatic paralysis: the use of M mode ultrasound for diagnosis in adults. Spinal Cord. 2006 Aug. 44(8):505-8. [Medline].
Gottesman E, McCool FD. Ultrasound evaluation of the paralyzed diaphragm. Am J Respir Crit Care Med. 1997 May. 155(5):1570-4. [Medline].
Versteegh MI, Braun J, Voigt PG, Bosman DB, Stolk J, Rabe KF. Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea. Eur J Cardiothorac Surg. 2007 Sep. 32(3):449-56. [Medline].
Freeman RK, Van Woerkom J, Vyverberg A, Ascioti AJ. Long-term follow-up of the functional and physiologic results of diaphragm plication in adults with unilateral diaphragm paralysis. Ann Thorac Surg. 2009 Oct. 88(4):1112-7. [Medline].
Gazala S, Hunt I, Bedard EL. Diaphragmatic plication offers functional improvement in dyspnoea and better pulmonary function with low morbidity. Interact Cardiovasc Thorac Surg. 2012 Sep. 15(3):505-8. [Medline]. [Full Text].
Le Pimpec-Barthes F, Gonzalez-Bermejo J, Hubsch JP, Duguet A, Morelot-Panzini C, Riquet M, et al. Intrathoracic phrenic pacing: a 10-year experience in France. J Thorac Cardiovasc Surg. 2011 Aug. 142(2):378-83. [Medline].
DiMarco AF, Onders RP, Ignagni A, Kowalski KE, Mortimer JT. Phrenic nerve pacing via intramuscular diaphragm electrodes in tetraplegic subjects. Chest. 2005 Feb. 127(2):671-8. [Medline].
Bach JR, Penek J. Obstructive sleep apnea complicating negative-pressure ventilatory support in patients with chronic paralytic/restrictive ventilatory dysfunction. Chest. 1991 Jun. 99(6):1386-93. [Medline].
Kaufman MR, Elkwood AI, Rose MI, Patel T, Ashinoff R, Saad A, et al. Reinnervation of the paralyzed diaphragm: application of nerve surgery techniques following unilateral phrenic nerve injury. Chest. 2011 Jul. 140(1):191-7. [Medline].
Kaufman MR, Elkwood AI, Colicchio AR, CeCe J, Jarrahy R, Willekes LJ, et al. Functional restoration of diaphragmatic paralysis: an evaluation of phrenic nerve reconstruction. Ann Thorac Surg. 2014 Jan. 97(1):260-6. [Medline].
Petrovic M, Lahrmann H, Pohl W, Wanke T. Idiopathic diaphragmatic paralysis--satisfactory improvement of inspiratory muscle function by inspiratory muscle training. Respir Physiol Neurobiol. 2009 Feb 28. 165(2-3):266-7. [Medline].
Easton PA, Fleetham JA, de la Rocha A, Anthonisen NR. Respiratory function after paralysis of the right hemidiaphragm. Am Rev Respir Dis. 1983 Jan. 127(1):125-8. [Medline].
Xu WD, Gu YD, Lu JB, Yu C, Zhang CG, Xu JG. Pulmonary function after complete unilateral phrenic nerve transection. J Neurosurg. 2005 Sep. 103(3):464-7. [Medline].
Respiratory management following spinal cord injury: a clinical practice guideline for health-care professionals. J Spinal Cord Med. 2005. 28(3):259-93. [Medline].