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

Preoperative Evaluation

Before proceeding to surgery, conduct thorough morphological and functional investigation including:

• Baseline dyspnea grading (MRC scale) 2
• Pulmonary function tests: FEV1 and FVC measurements 2
• Fluoroscopic assessment of diaphragmatic motion 1
• Evaluation for comorbid conditions that increase surgical risk 6

Surgical Technique

Diaphragmatic plication is the standard surgical procedure for eventration. 1, 2, 6

Surgical approach options:

• Video-assisted thoracoscopic surgery (VATS) or uniportal VATS is preferred as a minimally invasive approach with excellent outcomes. 6, 7
• Lateral thoracotomy remains an alternative for complex cases or when minimally invasive approach is not feasible. 6
• The procedure involves reinforcing the diaphragm to restore normal pulmonary parenchymal volume by replacing the diaphragm in its normal anatomical position. 1

Stapled resection via uniportal VATS provides a technically simple and feasible alternative to traditional plication. 7

Expected Outcomes

Surgical plication is safe and effective with low morbidity and mortality. 6

• Immediate symptom remission occurs in most patients, with gradual improvement continuing for up to one year. 1
• Statistically significant improvement in dyspnea grade, FEV1, and FVC values at 6-month follow-up. 2
• Long-lasting functional benefit approaching 100% in properly selected patients. 6
• The operated hemidiaphragm typically becomes immobile without paradoxical motion postoperatively. 1

Critical Pitfalls and Caveats

Beware of symptom relapse: 5 out of 7 patients in one series experienced gradual diaphragmatic rise or relapse of respiratory symptoms after repair, despite initial improvement. 8 This underscores the importance of:

• Careful patient selection based on severity of symptoms
• Long-term follow-up with repeat fluoroscopy at 1-5 years postoperatively 1
• Setting realistic expectations that the diaphragm will be immobile but stabilized 1

Do not operate on asymptomatic patients or those responsive to medical therapy, as surgical benefit is limited to those with persistent, significant symptoms. 8

Bilateral eventration is rare and requires specialized consideration, with only occasional case reports of bilateral plication. 6

Special Consideration: Central vs Peripheral Dysfunction

Distinguish between peripheral and central causes of diaphragmatic dysfunction, as management differs:

• Peripheral eventration (most common): Managed with plication as described above 6
• Central causes with ventilator dependence: May require phrenic nerve pacing in highly selected cases (tetraplegia above C3, central alveolar hypoventilation) 6