When we think about breathing difficulties we typically think of asthma, lung infections, or heart disease to name a few. Eventration is one of the many issues that could cause the muscle responsible for most of our breathing (the diaphragm) to provide insufficient respiratory support leading to chronic shortness of breath and negative impact on daily quality of life.
As a thoracic surgeon Dr. Kamran Ali sees many patients with unexplained breathlessness. Too often these patients are shocked that their breathlessness is due to some issue with the diaphragm which they never associated with the breathing issues they have.
This article will explain how diaphragm problems (such as diaphragmatic eventration) can cause breathlessness, what symptoms to look for, how they are diagnosed, and what the treatments are.
What Is the Diaphragm and Why is it Important?
The diaphragm is a large dome-shaped muscle underneath the lungs, which separates the heart and lungs in the chest cavity from the abdominal cavity. Every breath we take relies on the diaphragm; it contracts and flattens so that our lungs can fully expand and fill with air, while elastic recoil (breathing out) results from the diaphragm relaxing, and our lungs can then expel carbon dioxide.
In short:
- Healthy diaphragm = smooth breathing
- Weak or abnormal diaphragm = limited breathing capacity
Therefore, even though the lungs themselves may be normal if the diaphragm is abnormal, the diaphragm has the potential to cause breathlessness or fatigue or poor exercise tolerance.
What is Diaphragmatic Eventration?
Diaphragmatic eventration is when part of the diaphragm or the diaphragm is abnormally elevated due to a thinning or weakness of the muscle. In contrast to a diaphragmatic hernia, in which an actual defect or hole is present, the diaphragm is intact with eventration but is just not functioning well.
Characteristics of eventration are:
- The diaphragm appears to be higher than normal on chest X-rays
- Eventration can be unilateral, but rarely bilateral
- Areas of muscle fibers are occupied by thin fibrous tissue with decrease or complete loss of movement.
The decreased movement means that there is less room for the ipsilateral lung to expand, meaning that the individual will perceive shortness of breath to a greater extent in situations requiring exertion.
Causes of Diaphragm Eventration
Eventration of the diaphragm can either be congenital (present at birth) or acquired (after birth).
1. Congenital Causes
•Incomplete development of the diaphragm muscle while in utero.
•Often found in children but can lay latent until found in adulthood.
2. Acquired Causes
•Fracture of the phrenic nerve (the nerve that controls movement of the diaphragm) which can occur following:
•Surgery (notably heart surgery, thoracic or chest surgery).
•Trauma or accident.
•Tumors found that press on the nerve.
– Infections : polio, viral, and neuropathy.
– Idiopathic : no cause.
What Does Eventration Do to Breathing?
Breathing changes (bio-medical term: Dyspnea) come about as your diaphragm can no longer contract and/or relax.
Mechanism:
- The diaphragm becomes weak and cannot produce enough negative pressure.
- The lung on the affected side remains partially compressed.
- The patient will feel short of breath, especially going up stairs, brisk walking, or exercising.
- As time passes, the likelihood of obtaining enough oxygen intake becomes increasingly less and the patient may feel fatigue or not be able to obtain oxygen enough to sustain stamina.
In patients with severe chest wall difficulties, you may also find that they now experience orthopnea (difficult breathing when lying flat), if chest wall mechanics is affected so severely, that lung expansion is poor and the patient has repeated bouts of chest infections.
Signs of Diaphragmatic Eventration
While not everyone will experience symptoms (especially those with mild cases), many people have clear symptoms, including:
•Persistent shortness of breath (especially on exertion)
•Difficulty lying flat or requiring extra pillows to sleep
•Tight or discomfort in the chest
•Repeated respiratory infections
•Loss of physical capacity
•Fatigue and inattentiveness due to low oxygen readings
•In children, symptoms may include: failure to thrive, repeated pneumonia, or noisy breaths.
How does diagnosis of Eventration occur?
Proper diagnosis is crucial, as symptoms can resemble a lung or heart issue. Thoracic surgeon, Dr. Kamran Ali, will likely use some or all of the following tests:
1. Chest X-Ray
– This will show an abnormal rise of one side of the diaphragm.
2. CT Scan or MRI
– Provides detail for ruling out hernia, tumour, or other lung pathology.
3. Fluoroscopy with Sniff Test
– Observes the diaphragm in real-time.
– If the diaphragm was not moving correctly, or moves in a paradoxical fashion (i.e. opposite direction during breathing), this would indicate suspicion for eventration.
4. Ultrasound of the Diaphragm
– Is non-invasive test looking at diaphragm thickness and movement.
5. Pulmonary Function Tests (PFTs)
– Measures lung capacity, which is usually decreased in eventration.
6. Electromyography (EMG) in selected cases (neurogenic causes?)
– Tests nerve supply to the diaphragm.
No, not all diaphragm problems can cause breathlessness.
• A mild eventration may be without symptoms and found incidentally on x-ray.
• Severe eventration or bilateral involvement will almost always cause breathlessness.
• Other diaphragm related conditions that can cause breathing problems include:
- Diaphragm paralysis (due to injury of the phrenic nerve)
- Diaphragm hernia
- Diaphragm rupture (after trauma)
So generally, not all elevations of the diaphragm cause problems for the patient, but if an eventration is diagnosed, then if any symptoms develop the patient should seek medical attention.
Treatment Considerations for Diaphragmatic Eventration
Treatment is guided by symptom severity and the underlying cause.
- Conservative (or Non-Surgical) Measures
•Surgery is not needed right away if the eventration is mild and the patient has no symptoms.
•Changes in activity and lifestyle:
- Breathing exercises (e.g., diaphragmatic breathing)
- Pulmonary rehabilitation
- Weight loss
- Treatment of co-existing disorders (e.g. asthma, infection)
- Surgical Management – Diaphragmatic Plication
Surgical treatment may be appropriate for patients that have marked breathlessness or recurrent infections.
Diaphragmatic plication is the procedure most commonly performed:
•The paralyzed diaphragm is folded and stitched to itself, shortening it.
•Lowering the elevated diaphragm results in the lung having more room to expand.
•Surgery is then performed using one of two approaches: either open surgery or minimally invasive thoracoscopic (VATS).
Benefits of surgery:
- Improved lung function
- Improved breathlessness
- Reduced frequency of infections
- Improved quality of life
Life After Treatment – Expectations for Patients
Most patients notice a quick improvement in their symptoms, and are able to:
•Breathe easier
•Continue with their daily activiites
•Exercise with less fatigue
Recovery includes:
•Short hospital length of stay (3-5 days, depending on the type of surgical procedure)
•Physio-assisted breathing exercises
•Follow-up with the surgeon during regular intervals
Frequently Asked Questions (FAQ)
1. Is diaphragmatic eventration fatal?
Mild cases are not fatal, however if serious there may also be a secondary respiratory disease that could develop and make the patient susceptible to infections or respiratory failure that would be untreated.
2. Can children also have eventration?
Yes, congenitally. We see this in infants and children born with eventration; this is a recognized entity in radiology and tends to be diagnosed early due to infants and children having recurrent problems related to affliction of the lungs.
3. Does eventration always require surgery?
No. Only symptomatic cases that, by severity, cause significant breathlessness or may contribute to lung compromise require surgery.
4. Can I exercise to solve eventration?
Some breathing exercises will improve your overall lung function (strengthening the function of the lungs) but will not fix the diaphragm muscle that is weak; surgery is the only way to cure eventration when required.
5. How is eventration different from hernia?
Eventration = intact but weakened diaphragm muscle. o Hernia = defect or hole in diaphragm allowing abdominal organs to migrate through and into chest cavity.
When to Consult a Thoracic Surgeon?
You should seek a medical consultation if you are experiencing:
•Persistent breathlessness for no reason – not asthma, heart disease or infection.
•Difficulty lying flat without breathlessness.
•Unexplained Chest X-ray evidence of an elevated diaphragm.
•Recurrent respiratory infections despite treatment.
A thoracic surgeon such as Dr. Kamran Ali can provide a complete evaluation and can discuss whether conservative or surgical management is appropriate for your specific situation.
Conclusion
The diaphragm is a crucial component of every breath we take. When the diaphragm is weakened or elevated abnormally, as with a condition of diaphragmatic eventration, chronic and often distressing breathlessness will be the characteristic result.
Fortunately, with advances in diagnostics and minimally invasive techniques, patient outcomes result in excellent outcomes today. When diagnosed early and treatment is initiated in a timely manner, quality of life can significantly improve.
If you or someone you know suffers unexplained breathlessness, it is worthwhile taking the time to consult an experienced thoracic surgeon to see if the problem is related to a diaphragm condition that is neglected.
