How is bilateral diaphragmatic paralysis treated?

Updated: Oct 10, 2018
  • Author: Justina Gamache, MD; Chief Editor: Guy W Soo Hoo, MD, MPH  more...
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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 used in patients with central respiratory paralysis and upper cervical spinal cord injury (lesions above C3) to wean them off the ventilators. [24] 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. [25] 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. [24] 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. [26] 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.

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