Diaphragmatic dysfunction and respiratory illness (Review, NEJM) - PulmCCM
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Mar 152012
 

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 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.

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:

  • Trauma or surgery causing cervical cord or phrenic nerve damage (high C-spine injuries involving C3-C5, phrenic nerve injury during cardiac surgery);
  • Mechanical ventilation;
  • COPD and other diseases that cause lung hyperinflation;
  • Myopathies and neuropathies (myasthenia gravis; critical illness neuro/myopathy; amyotrophic lateral sclerosis, poliomyelitis, with a 35 year delay until diaphragmatic weakness);
  • Inflammatory disorders (e.g., sepsis);
  • Mediastinal masses.

 

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 can sometimes cause dyspnea when lying on one’s back (supine). Often, unilateral 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.

Rather than causing problematic dyspnea or respiratory insufficiency on its own, diaphragmatic paralysis likely acts more often as a “co-conspirator” that reduces respiratory reserve and the threshold for respiratory failure. Many people with diaphragmatic paralysis are well-compensated when at rest and not acutely ill, but an acute illness such as pneumonia or an exacerbation of heart or lung disease may increase respiratory demand beyond the ability of the paralyzed diaphragm, with resulting severe dyspnea or respiratory failure. Diaphragmatic dysfunction also likely makes it more difficult to escape from dependence on mechanical ventilation.

Diagnosis of Diaphragmatic Paralysis

A number of tests can help identify diaphragmatic dysfunction. Tests for diaphragmatic paralysis include:

Chest X-rays in diaphragm paralysis may show elevated hemidiaphragms and basal subsegmental atelectasis; insensitive in detecting bilateral paralysis as films may often be interpreted as “poor effort” or “low lung volumes.” Chest X-ray is 90% sensitive for unilateral paralysis but only 44% specific (high false positive rate).

Fluoroscopy of the diaphragm (“sniff test”): the patient sniffs energetically during fluoroscopy; descent of the diaphragm is the normal response. People with unilateral diaphragmatic paralysis have a paradoxical upward movement of the weak hemidiaphragm, 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.

 

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.

Abdominal paradox, with the abdomen moving inward as the thorax expands during 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 chest during inspiration. Abdominal paradoxical breathing is almost exclusively found in people with bilateral diaphragmatic paralysis; if present in unilateral paralysis, it means the 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”).

McCool FD, Tzelepis GE. Dysfunction of the Diaphragm. N Engl J Med 2012;366:932-942.

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  33 Responses to “Diaphragmatic dysfunction and respiratory illness (Review, NEJM)”

  1. 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

    • Daughter had ablation in December 2013 for atypical atrial flutter and in late January 2014 has discovered she has a paralyzed right hemidiaphragm.

  2. I think i might have a ruptured diaphragm for the past year they have dignosed me with a paradoxical left diaphragm paralisis , but I did have a have my apendix removed last summer and people noticed i was breathing hard.I have vomited once and felt very sick in my chest and abdomen .My palminaligest have also have not experienced such a complicated case any help?Sorry about the misspelled words .

  3. Or a diaphragm hernia

  4. ive been diagnosed with diapragmatic disfunction, it is ‘weak’ and only moves a little im looking for information on how to strengthn and get more movement in it??

    • My dr. has me using a spirometer. It is a device they have patients use after surgery. I could only reach 500 when I began. Now I can reach 2000. My Dr. was amazed that I could do this. He thought if I could keep it at 500 I would bee doing good. I have CMT- a neurological disorder that destroys nerves and then affects muscle. I go to Vanderbilt CMT clinic Nov. 14 and hope I can find other things that will help. Will get back to you if find other things. God bless. Peggy

  5. I’ve got a paralized hemi diaphragm due to my Phrenic nerve being damaged whilst I was on life support in a coma due to having contracted meningitis septacemia. I get very breathless on any exertion does anyone have any suggestions on how to stay well clearly my immune system is compromised?

  6. Great article. I live in the UK and was perfectly fit and well until 6 months ago. In October I experienced a severe pain in my right shoulder and have suffered from breathlessness ever since. I received anti- inflammatory drugs for the shoulder but nothing for the breathlesssness. I went to the swimming pool last week and immediately I was up to chest height in the water I was extremely breathless and had to get out of the pool. This recurs every time I go into the pool, whether I swim or not.
    I can run for a short time, but if I bend down or over exert, I become breathless.
    I have had an XRay and it showed some abnormailty, I beleive that I have got Diaphragmatic Paralysis and am booked for an Ultrascan tomorrow.
    Can the problem resolve itself or will I need surgery?

    Bill

    • Dear Bill,

      I have had breathing issues for 9 weeks. 6 weeks ago I was diagnosed with the “sniff” test with UDP. I am highly symptomatic. I, now after 9 weeks, have confirmed the cause of my UDP as have Parsonage-Turner Syndrome. I have had 5 MRI’s, 2 CT’s, 2 Neuroconduction, echo, lung capacity testing, and more. I finally ended up with the head of the University of Miami Neurology department (Dr. David Adams).

      Here’s how it began. I had a slight cold – but pain in my back was increasing and my orthopedic prescribed a Medrodose pack (corticosteroids). took them for 7 days – then about 3 days later experience extensive pains in my right should (a key symptom for PTS). Basically the corticosteroids suppressed my immune system and a virus attacked my bracial plexus (which is one place all these nerves come together).

      Today at UM I had an extensive EMG (which is required to fully support the diagnosis). Good news — the nerves are damaged – but communicating and will heal in time. I am now working with a series of doctors to reduce my inflammation, increase my immune processes and add in some supplements that promote nerve repair.

      Doctors estimate 4-6 month recovery.

      It was the anxiety that was killing me. Now I have a better idea WHAT it is — and WHAT to do.

      Sincerely,

      • Hi Patrick
        It’s good news that your nerves are still communicating, looks like there’s a good chance of full recovery. I feel on top of the world at the moment, I’m no longer breathless and can immerse in water. I’ve lost weight and feel much fitter. My doctor has stopped one of my blood pressure tablets as my blood pressure has reduced.I took fucosamine supplement for 3 months to help with nerve repair, and it seemed to help. I have an appointment with the specialist next week to see how I am progressing, but without an ulttra sound scan I don’t know if my hemi-diaphragm is still paralysed.
        I’ll keep you informed of the result.
        Bill

      • Your symptoms sounds alot like what I have been going thru for several years without any support or finding a doctor that will listen. I had PTS back in 2009 and was told my phrenic nerve was damaged. What or type of doctor would you recommend?

  7. Had ultrascan today and it has been confirmed that my right hemdiaphragm is paralysed, doctor says that there is nothing that can be done, and I will have to live with it. Can’t accept that, there must be some alternative, surely??

  8. Hello Bill I was told the same thing that I would have to live with it and that there is nothing they can do as my left hemidiaphragm is completely paralized. I understand that there is a procedure that can be done for those whose diaphragm are not completely paralized called ‘plication’. I don’t know if that’s any help.
    Denise Waterton

  9. My husband was diagnosed last year, and after having a bronchoscopy (his lower lung was slightly collapsed) to resolve the lung, felt much better for the better part of a year. He’s now back where he was, and he also was told there’s nothing that can be done. If anyone knows of any therapies to alleviate this, please share

  10. Ive had bilateral paralyzed diaphragms for the last 5 years. Seems to be some type of demyleination of the phrenic nerves. Have to use vent to lie down and very breathless all the time. On O2 24/7

    Also some neuropath in the feet and ankles, so some nerve issue is going on. Just dont know what.

    Did nerve biopsy and see no inflammation so Dr. said not likely CIDP. So looks to be no treatment. If it had been inflammatory , then the treatment is IvIg. To counteract some of the anti bodies.

    Ive even had the phrenic nerve stimulators implanted….but with the phrenic nerves compromised, they dont work either. There is no nerve pathway from the stimulator link to the diaphragm since the nerve is compromised. So looks like Im stuck with it.

    But just sent all my records to Mayo to see if anyone wants to use me for guinea pig.

    • Hi. I am reading about your case. My left phrenic nerve was cut during a surgery, was a malpractice, . I get very Thank you!

      • Hi Wendy,
        I think the same thing happened to me during lung surgery .How did you find a lawyer? Did you win? Lawyers shy away from these cases as I think they are hard to prove. what tests did you have and symptoms? Are you in US?
        Thanks
        There is a cutting edge :) specialist in phrenic nerve surgery in Shrewsbury NJ Dr Kaufman and I’m seeing him soon.

        • Dr. Kaufman is a great doctor. I have a paralysed left diaphragm due to a damaged phrenic nerve that happened during surgery to remove a bone spur in my left shoulder. I was also told that nothing could be done and I would have to live with it. Well I kept searching the Internet any found Dr. Kaufman. My surgery was in 2005 and I located him in 2010. He tried to clean the scar tissue from my phrenic nerve but it still would not work so he did a nerve graft around the damaged area. My diaphragm is still paralysed. I keep hoping and praying that the graft will work. Don’t let this message deter you from going to see Dr. Kaufman he may be able to help you. I am 73 so my age may have something to do with my results but that’s just my guess.

  11. I have Charcot-Marie-Tooth Type 1A and have been diagnosed with failure of right diaphragm. I am on a vpap sleep machine. What do I do if other side phrenic nerve is affected as quickly as right side.

    • I also have CMT, with bilateral diaphragm paralysis. I am using bipap with supplemental oxygen at night. Hopefully, this will slow the rate of progression down. Try to sleep with your head elevated(I use a foam wedge). Have regular appointments with a pulmonologist/sleep specialist in order to assess progression and ventilation need adjustment. Best of luck to you.

      • Glad to hear from someone else with CMT, but sad we have the similar diaphragm problems, I didn’t think someone could live with both sides affected, but I read on this site others with bilateral paralysis. My left side is so far ok. Thanks for the information about the wedge. I am having problems getting use to it , but I will. I go to Vanderbilt CMT Clinic Nov. 16. I’ll get back if I found out any thing. Saying a prayer for you now.

  12. After being diagnosed with a paralysed right diaphragm in October 2012 with 69% lung capacity and unable to immerse in water, I was told that my condition was unlikely to improve. I decided to get fit and loose weight. I also had 6 sessions with an osteopath to free the phrenic nerve and lost 2.5 stone (14.5 to 12 stone) After 8 months I can now run 2 miles per day, swim a full length of a swimming pool underwater and walk without being breathless. I beleive that my body has adapted and repaired itself. I have an appointment with my specialist in 3 months time and am looking forward to hisresponse. Hope that this gives some hope and reassurance to fellow sufferers.

    Bill

  13. I am 34 and have a complicated medical history. I have APLS (antiphospholid syndrome) and Lupus among many other things. I have been hospitalized 3 times in the last year with a collapsed lung its always the left one. Finally this last hospitalization they realized after thinking my diaphragm was ruptured that it is actually paralyzed. They have me on a cough o later 2x a day to help with sputum and to inflate the lungs, Im not sure if that therapy will help anyone. They did say it wont prevent future collapses but hopefully diminish the amount of them.

    • My dr. has be on a v-pap machine at night. You get them from a sleep center. I have a rare neurological disorder CMT God bless. Peggy

  14. Had my 6 monthly consultants review this morning, had spirometer tests and the results are staggering! From a lung capacity of 69% in May, it has gone to normal (95% both sitting and in the supine position.
    The consultant doesn’t want to see me for a year unless I have problems, he is then going to do another ultra-sound scan to see if the right hemi-diaphragm has some movement.
    Very pleased, feel much better.
    Hope that this gives others some hope of improvement,

  15. Bill……new to this forum and struggling with my GP to recognise that my increasingly poor breathing is not down to stress(it’s causing me stress!). I was running miles this time 12 months ago but following back injuries (and associated stress) by the time lower back healing just before christmas, tension in ribcage and upper back intense – it’s like my abs and diaphragm just won’t switch off and relax. Breathing is now so difficult on standing and short walks with severe lactic acid build up in shoulders, mid back and neck. Where did you go for tests and what kind of tests did you have. I will self fund but just want to get on with it as getting very concerned that not being taken seriously. Any info you can help with Bill would be much appreciated.

  16. By the way Bill, I am in the UK – Wiltshire.

  17. I have had a long history of Brachial Neuritis starting when i was 17 i’ve just turned 47 and have just had the:-) worse episode yet. I was on morphine for 6 weeks and my breathing was terrible. Over the 30 years i have learnt to live with the BN which has sudden shoulder arm and neck pain, which I’ve had on both shoulders but only effecting one side at a time.

    My GP set me for X-rays just before Christmas 2013 the results revealed that I had a raise hemi-diaphragm on the left side (same are the current pain). The day after i got my results i had severe abdominal pains from the groin up into my abdomen. I was admitted to hospital and had more X-rays and ultrasound scans. It was noted in my scan that i had bilateral paralysis of the hemi-diaphragm, this was after i asked the doctor to check as i knew that my other X-rays mentioned the diaphragm. As soon as I lay flat for my scan that something wasn’t right (I could hardly breath).

    I was discharged from hospital as no hernias were found in my lower abdomen? I asked the Dr about my diaphragm and what that meant he just said that wasn’t his field and that i was admitted with abdominal pain and that’s what he was interested in and they found nothing that was causing it. To be fair the pain was undercontrol.

    My Gp was mad that I wasn’t passed over to the neurologists.

    So here I am with a bilateral paralysed diaphragm and still alive and kicking. My GP has managed to get a MRI scan sorted and has spoken directly to the Neurologists who have taken on my case. I am also seeing an Orthopedic surgeon on Monday through my private medical so I’ll have 2 teams looking at it. (He is unaware of my diaphragm yet)

    Bill if your still checking this site drop me an email as i would love to chat as we seem to have a couple of things in common. Email is [email protected] I live up in Perth Scotland.

    I’ll post more once I know more.

  18. Hi Bill and the others on this site. I was well till November 2013, when following neck manipulation by a Physiotherapist I experienced severe pain in my neck and right shoulder blade, which lasted about 24 hours. After this my breathing became very difficult especially when lying down, leaning forward and on exertion. I was perfectly fit before this. My x-ray showed a markedly raised right hemidiaphragm, and I was diagnosed with Phrenic nerve Palsy. However, my consultant has offered no help and left me to cope. I have no idea how long this will last or if indeed it will ever get better. I cannot walk far, and sleep is difficult. It is now April 2014, so I have had it for nearly five months and I am feeling alone with this. I live in the South of England by the way. Why do doctors know so little and seem to have no idea of prognosis? Any help would be so appreciated. I am 65 by the way.

    • Hello Janet I have the same condition as yourself following contracting Meninngitis Septaceamia and being on a life support machine in an induced coma in ITU for just over 2 weeks back in 2007. Then 3 years ago it became clear that my hemidiaphragm was weakened/parilized and then started a very long journey trying to get my local respiratory team to help me but they didn’t seem to have a clue -then last September I decided I needed to take control of my condition so after much investigating I asked my GP to refer me to the Royal Brompton & Harefield hospital to see a Professesor Mike Polkey and his team who specialised in diaphragmatic problems. Since then Janet I have been seen 4 times and been given enormous help and advice so for me, at long last I feel someone knows how to help me with my condition.i hope this is of help to you – I am a 62 year old woman living in Buckinghamshire.
      Kind regards Dee Waterton

      • Hello Dee, Thank you for your helpful reply. The whole thing has been such a shock as I was healthy and fit before November last year. Sorry to hear about your terrible time in ITU. Do you think the Phrenic Nerve Palsy was caused at that time or later? I have made a note of the Royal Brompton and Professor Mike Polkey. I will tell my GP about him and ask if I can be referred. It seems to be something that is little known here in the UK. I live south of Southampton by the way.
        Kind regards Jan

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