Diaphragm Disorders Treatment & Management

Patients with unilateral diaphragmatic paralysis are typically asymptomatic and do not need treatment. Patients may have some dyspnea in physiological situations associated with exertion or if they have an underlying pulmonary disease.

Treatment should be considered when dyspnea is out of proportion to the physical exertion. Medical care should be focused on the etiology of the dysfunction. In anatomic causes and defects, the only treatment option is surgical repair. Once an anatomic etiology is considered, the most important next step is to discover the cause. Neurologic processes, depending on the etiology, can generally be treated medically.

Supportive management

Many patients with severe diaphragmatic dysfunction require ventilatory support. Depending on the etiology of the disease, some patients only require short-term support, while others may require life-long ventilatory breathing.

Most patients with bilateral diaphragmatic paralysis eventually develop progressive ventilatory failure resulting from fatigue of the accessory muscles. Patients may have progressive disease with carbon dioxide retention and irreversible ventilatory failure. These patients require ventilatory assistance.

In one study, improvement was demonstrated after ventilatory support with external negative-pressure respirators in patients with overt carbon dioxide retention and decreased central ventilatory drive. Some patients who have decreased ventilatory strength as measured by PI-max and transdiaphragmatic pressures had these measures of respiratory function normalize after a period of negative-pressure ventilatory assistance.

Patients with cor pulmonale also may manifest improvement in function and correction of blood gas abnormalities with nighttime or intermittent daytime noninvasive ventilation.^[22]

If the patient does not respond to nasal or oral positive-pressure ventilation, alternative forms of therapy such as negative-pressure cuirass or pulmo-wrap, rocking bed, or positive-pressure pulmo-belt can be used.

Tracheotomy with positive-pressure intermittent or permanent ventilation is reserved for patients with life-threatening disease.

Patients should undergo a sleep study if he or she is considered for negative-pressure ventilation because it can precipitate or exacerbate preexisting upper airway obstruction. Positive-pressure ventilation can minimize the need for a sleep study.

If the phrenic nerve is intact and the problem lies in actually transmitting an impulse to the nerve, phrenic nerve or diaphragmatic pacing may be useful modalities in the treatment of this subset of patients.^[23]

Progressive reconditioning is recommended when using a diaphragmatic pacer. High stimulating frequencies and a prolonged period of pacing may lead to irreversible muscle dysfunction. Patients with diaphragmatic pacing require tracheotomies because pacer-induced breathing is not synchronized with the upper airway. Investigations with diaphragmatic pacers and upper airway sensors are ongoing.^{[24, 25]}

Neurologic

Once a diagnosis of neurologic dysfunction is made, ordering studies to determine the cause is vital. A number of neurologic etiologies can be managed medically, but discovering the cause often becomes a challenge.

Surgery in indicated in the management of anatomic defects in the diaphragm. The type of surgical intervention depends on the anatomic defect or problem.

Congenital defects

Manage congenital diaphragmatic defects through transabdominal primary surgical repair.

Acquired defects

Manage acquired diaphragmatic defects (ie, traumatic rupture, late-onset congenital diaphragmatic defect) by thoracoscopic plication of the hemidiaphragm. Plication results in improved lung function, exercise endurance, and dyspnea. Plication of the diseased diaphragm improves ventilation to the well-perfused lung and improves gas exchange, which improves static lung mechanics.

In a selected group of patients, plication of the diaphragm improved VC by 10-20% and improved PaO₂ by 10%.^[26]

A surgical series reported improvement in forced tidal volume from 216 mm/GHz to 415 mm/GHz postplication, and mechanical ventilatory support could be discontinued 2-12 days after plication. Plication can also be achieved by video-assisted thoracoscopy.^[27]

Phrenic nerve injury

Primary repair of phrenic nerve damage from trauma can be attempted but does not generally restore function. With expectant treatment, few patients regain phrenic nerve function. Manage injury from a tumor by resection of the tumor encasing the phrenic nerve. Most patients regain function of the nerve. Cold phrenic nerve injury during cardiac surgery generally resolves with expectant management.

Spinal cord injury or phrenic nerve injury

Diaphragmatic pacing is a technology that allows the placement electrodes within the diaphragm that stimulate the diaphragm to contract. This can be performed either transthoracically or transabdominally. The more recent studies support the use of laparoscopy and thoracoscopy.