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Diaphragmatic dysfunction can result from nerve damage, primary muscle problems, or
problems with the muscle's interaction with the chest wall. The true incidence of

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diaphragmatic paralysis is unknown, since many patients are asymptomatic. Treatment for
diaphragmatic dysfunction usually consists of watchful waiting, addressing underlying
causes, with mechanical ventilation if respiratory failure develops.

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Causes of Diaphragmatic Weakness or Paralysis

Diaphragmatic paralysis is likely most often idiopathic and unilateral. When a cause for
diaphragmatic paralysis can be identified, it may be due to:

Update
Vasopressors for septic shock (Surviving
Sepsis Guidelines)
Surviving Sepsis Guidelines 2013 Review
& Update

Trauma or surgery causing cervical cord or phrenic nerve damage (high C-spine

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injuries involving C3-C5, phrenic nerve injury during cardiac surgery);

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Mechanical ventilation;

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COPD and other diseases that cause lung hyperinflation;

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Myopathies and neuropathies (myasthenia gravis; critical illness neuro/myopathy;

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amyotrophic lateral sclerosis, poliomyelitis, with a 35 year delay until diaphragmatic

Chronic obstructive pulmonary disease 2014

weakness);

update (COPD Review, Lancet)

Inflammatory disorders (e.g., sepsis);

New GOLD guidelines: Better than the old

Mediastinal masses.

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over time?
Diaphragmatic dysfunction and respiratory

Symptoms of Unilateral & Bilateral Diaphragmatic Paralysis

Unilateral diaphragmatic paralysis or weakness rarely causes symptomatic dyspnea at
rest, but may result in dyspnea on exertion or the patient's voluntary restriction of activity. It

illness (Review)

Surviving Sepsis Guidelines Update

can sometimes cause dyspnea when lying on one's back (supine). Often, unilateral
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diaphragmatic paralysis is detected incidentally on a chest X-ray obtained for other

purposes.
Bilateral diaphragmatic paralysis frequently causes dyspnea at rest, with exertion, when
supine (necessitating sleeping in a recliner), bending over, or when swimming with water
above waist level. Sleep disorders are also common in these patients, and symptoms thereof
(fatigue, somnolence, awakening during sleep) may be the first presentation of bilateral
diaphragmatic paralysis. Recurrent pneumonias (possibly due to basilar atelectasis) and
recurrent respiratory failure are also possible.

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Rather than causing problematic dyspnea or respiratory insufficiency on its own,

Update

diaphragmatic paralysis likely acts more often as a "co-conspirator" that reduces respiratory

Surviving Sepsis Guidelines 2013 Review

reserve and the threshold for respiratory failure. Many people with diaphragmatic paralysis

& Update

are well-compensated when at rest and not acutely ill, but an acute illness such as

Vasopressors for septic shock (Surviving

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pneumonia or an exacerbation of heart or lung disease may increase respiratory demand

Sepsis Guidelines)

beyond the ability of the paralyzed diaphragm, with resulting severe dyspnea or respiratory

Chronic obstructive pulmonary disease 2014

failure. Diaphragmatic dysfunction also likely makes it more difficult to escape from

update (COPD Review, Lancet)

dependence on mechanical ventilation.

Diaphragmatic dysfunction and respiratory

Diagnosis of Diaphragmatic Paralysis

illness (Review)
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A number of tests can help identify diaphragmatic dysfunction. Tests for diaphragmatic

Health)

paralysis include:

How dangerous are ground glass nodules

over time?

Chest X-rays in diaphragm paralysis may show elevated hemidiaphragms and basal

Are traditional protocols for goal directed

subsegmental atelectasis; insensitive in detecting bilateral paralysis as films may often be

therapy for sepsis dead? (ARISE trial)

interpreted as "poor effort" or "low lung volumes." Chest X-ray is 90% sensitive for

New GOLD guidelines: Better than the old

unilateral paralysis but only 44% specific (high false positive rate).

GOLD
Surviving Sepsis Guidelines: Early Goal

Fluoroscopy of the diaphragm (sniff test): the patient sniffs energetically during

Directed Therapy, Initial Fluid Resuscitation

fluoroscopy; descent of the diaphragm is the normal response. People with unilateral
diaphragmatic paralysis have a paradoxical upward movement of the weak hemidiaphragm,

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which gets "sucked up" by the negative pleural pressure created by the working
hemidiaphragm. The sniff test is not considered to be accurate in diagnosing bilateral
diaphragmatic paralysis, with a ~6% false positive rate and a higher false negative
rate.
Pulmonary function tests show restriction, which may be moderate to severe (30-50%
predicted total lung capacity) in bilateral diaphragmatic paralysis. The restriction worsens
when supine, evidenced by a drop in vital capacity of 30 to 50% in bilateral diaphragm
paralysis. This test is sensitive and has a high negative predictive value: if there is no
reduction in FVC when supine, there is probably no significant diaphragmatic paralysis.
Maximal static inspiratory pressure (MIP) and sniff nasal inspiratory pressure are
reduced to ~60% predicted in people with unilateral diaphragmatic paralysis and to ~30%
predicted in bilateral diaphragmatic paralysis. However, these tests are effort-dependent
and less reproducible than lung volumes; with a high false positive rate for respiratory
weakness.
Ultrasound can be extremely useful in measuring diaphragmatic function. The point of
contact between the diaphragm and the rib cage should be viewed. The diaphragm should
thicken with inspiration, indicating shortening; if the diaphragm does not thicken,
paralysis is present. As a fast, inexpensive and noninvasive test, ultrasound offers many
advantages and can also be used serially to assess recovery of a paralyzed diaphragm. A
2011 study among 88 mechanically ventilated patients suggested that diaphragmatic
weakness on ultrasound could help predict extubation failure and inability to wean from
mechanical ventilation.
Electromyography of the diaphragm is technically difficult and its results can therefore
be hard to interpret or rely upon. It can potentially help differentiate between a myopathy
and neuropathy, if one of these is strongly believed to be the cause of diaphragmatic
dysfunction.

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Physical Examination Findings in Diaphragmatic Dysfunction

Generally speaking, physical findings are more likely in people with bilateral diaphragmatic
paralysis. Some, all, or none of these physical examination findings may be present in
people with diaphragmatic dysfunction.

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Abdominal paradox, with the abdomen moving inward as the thorax expands during

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inspiration; this is the "classic" sign of diaphragmatic dysfunction, caused by the accessory
muscles creating negative pleural pressure that "sucks up" the flaccid diaphragm into the

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chest during inspiration. Abdominal paradoxical breathing is almost exclusively found in

people with bilateral diaphragmatic paralysis; if present in unilateral paralysis, it means the

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respiratory muscles in general are weak.

Other possible physical examination findings in diaphragmatic paralysis include:
Tachypnea
Use of accessory muscles during quiet breathing; detectable by contraction of the
sternocleidomastoid muscles during neck palpation
Decreased diaphragmatic excursion (percussing the lower rib cage at end
inspiration and end expiration; the change in resonance should span at least 3 - 5 cm )

Treatments for Diaphragmatic Paralysis

Most people with diaphragmatic paralysis do not require treatment, other than watchful
waiting, potentially with serial examinations. Many or most people with diaphragmatic
paralysis have other likely contributing causes for dyspnea (obesity and deconditioning, lung
and heart disease, etc.), making a determination of the contribution of diaphragmatic
paralysis to dyspnea extremely difficult.
A common-sense approach to treatment of diaphragmatic paralysis can include:
Remove/treat any obvious contributing factors (hypokalemia, hypophosphatemia,
high-dose steroids, neurotoxic drugs, neuromuscular blockers).
Nocturnal noninvasive ventilation for people with an awake pCO2 of 45+ mm Hg;
nocturnal hypoxemia (SaO2 < 88% of >5 consecutive min); or progressive
neuromuscular disease and a maximal static inspiratory pressure (MIP) < 60 cm H2O
or forced vital capacity (FVC) < 50% predicted.
Treat sleep-disordered breathing, if present, with continuous positive airway
pressure (CPAP) or nocturnal noninvasive ventilation.
Surgical plication of the hemidiaphragm involves "tightening" the loose, paralyzed
hemidiaphragm by oversewing its center. This therapy improved lung function and dyspnea
in retrospective, uncontrolled trials. It is of no use in bilateral diaphragmatic paralysis, and
is relatively contraindicated in progressive neuromuscular disease and in severely obese
people. Because unilateral paralysis is usually either minimally symptomatic or improves
with time, plication should be considered only after a long period of watchful waiting.
Phrenic pacing is only appropriate for ventilator dependent patients, mainly quadriplegics
with cervical spine injuries at C3-C5 or above ("high quads").
Read more: McCool FD, Tzelepis GE. Dysfunction of the Diaphragm. N Engl J Med
2012;366:932-942.

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One Response to Diaphragmatic dysfunction and respiratory illness

(Review)

1.

Wooley says:
October 16, 2012 at 11:10 pm
Good review
Add new etiology: pulmonary vein ablation therapy for atrial fibrillation. This can occur with
electrical or cryotherapy. I have seen two cases this year.
M. Wooley
Reply

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