What is the management and treatment of diaphragmatic eventration?

Management and Treatment of Diaphragmatic Eventration

Symptomatic diaphragmatic eventration requires surgical plication, while asymptomatic cases can be managed conservatively with observation. 1

Distinguishing Eventration from Other Diaphragmatic Pathology

Diaphragmatic eventration is the abnormal elevation of an intact diaphragm due to paralysis or aplasia of muscular fibers, maintaining all anatomical attachments normally. 2 This differs from diaphragmatic hernias where there is an actual defect or tear in the diaphragm allowing organ herniation. 3

Diagnostic Approach

Start with chest X-ray (both anteroposterior and lateral views) as the initial diagnostic study in patients presenting with respiratory symptoms. 3, 4

• Chest X-ray will show abnormal diaphragmatic elevation but has limited sensitivity (2-60% for left-sided, 17-33% for right-sided abnormalities). 4
• Normal chest X-rays do NOT exclude diaphragmatic pathology, with false negatives in 11-62% of cases. 4, 5
• Proceed to fluoroscopy to assess diaphragmatic motion and detect paradoxical movement, which confirms eventration versus simple elevation. 1
• CT scan with contrast is the gold standard if diagnosis remains uncertain, with sensitivity of 14-82% and specificity of 87%. 5

Indications for Surgical Intervention

Surgery is indicated ONLY when patients have significant symptoms that fail conservative management. 1, 6

Specific surgical indications include:

• Severe dyspnea or orthopnea affecting quality of life 7
• Recurrent pneumonia 1
• Failure to thrive (in children) 1
• Positional dyspnea 6
• Cardiac or gastrointestinal symptoms causing distress 6, 8
• Pain related to the eventration 6

Asymptomatic eventration should be managed conservatively with observation, as surgical intervention provides no benefit without symptoms. 1, 8

Surgical Technique: Diaphragmatic Plication

The standard surgical approach is diaphragmatic plication performed via lateral thoracotomy or video-assisted thoracoscopic surgery (VATS). 6

Technical approach:

• Minimally invasive approaches (VATS or uniportal VATS) should be attempted first as they offer equivalent outcomes with reduced morbidity. 7
• The procedure involves reinforcing the diaphragm to reduce abnormal ascent and restore normal pulmonary parenchymal volume. 1
• Stapled resection via uniportal VATS is a technically simple and feasible alternative to traditional plication. 7
• Use non-absorbable sutures for plication to ensure durability. 6

Expected outcomes:

• Immediate symptom remission occurs in most patients, with gradual improvement continuing for up to one year. 1
• The operated hemidiaphragm typically becomes immobile without paradoxical motion postoperatively. 1
• Functional improvement is sustained long-term in approximately 100% of appropriately selected patients. 6
• Significant improvement in dyspnea grade, FEV1, and FVC values at 6-month follow-up. 2

Critical Pitfalls and Caveats

Do not operate on asymptomatic patients, as 5 out of 7 patients in one series experienced gradual diaphragmatic rise or relapse of respiratory symptoms after repair despite initial improvement. 8 This suggests that surgery without clear symptomatic indication may not provide durable benefit.

Rigorous preoperative patient selection is essential for surgical success. 6 This requires:

• Thorough morphological and functional investigation of neuromuscular and respiratory components 6
• Documentation that symptoms are truly attributable to the eventration 8
• Confirmation that conservative management has failed 6

The diaphragm will be immobile after plication - this is expected and does not indicate treatment failure as long as symptoms resolve. 1

Bilateral eventration is rare but has been successfully treated with bilateral plication in selected cases. 6

Special Consideration: Central vs Peripheral Dysfunction

Eventration of peripheral origin (most common) is treated with plication only when symptomatic. 6 Central causes of diaphragmatic paralysis may require phrenic nerve pacing in highly selected cases (tetraplegia above C3, central alveolar hypoventilation) rather than plication. 6