How to manage a paralyzed diaphragm?

Management of Paralyzed Diaphragm

Diaphragmatic paralysis requires surgical intervention for symptomatic cases, with plication being the primary treatment option for unilateral paralysis, while bilateral paralysis often necessitates ventilatory support. 1, 2

Diagnosis

• Initial evaluation should include chest radiography, which can assess the relative position of the diaphragm and provide clues to diaphragmatic paralysis 3
• Fluoroscopy allows for more accurate assessment of diaphragmatic motion and is considered a reference standard for diagnosis 3
• Ultrasound of the chest is highly recommended as it:
  • Shows concordant results with fluoroscopic imaging 3
  • Can evaluate diaphragmatic excursion amplitude, thickness, and contraction 3
  • Can identify paralysis as paradoxical movement during respiration 3
  • Has high sensitivity and specificity for diagnosing neuromuscular disorders of the diaphragm 3
• CT scan is the gold standard for diagnosing diaphragmatic hernia but has limited value in assessing diaphragmatic dysfunction 3
• Cine dynamic MRI sequences, although not widely practiced, allow for direct visualization of diaphragm motion and comprehensive analysis of both diaphragm and chest wall muscle movement 3

Management Approach Based on Type of Paralysis

Unilateral Diaphragmatic Paralysis

• Usually well-tolerated in the absence of underlying lung disease 2
• For symptomatic cases with significant dyspnea or hypoxemia:
  • Surgical plication of the diaphragm is recommended 2, 4
  • Plication prevents paradoxical motion of the affected diaphragm 4
  • This technique does not prevent return of diaphragmatic function if nerve recovery occurs 4

Bilateral Diaphragmatic Paralysis

• Typically more symptomatic and may result in ventilatory failure 1, 2
• Management options include:
  • Continuous positive airway pressure (CPAP) for less severe cases 1
  • Noninvasive ventilation for moderate cases 2
  • Mechanical ventilation and tracheostomy for severe cases with ventilatory failure 1
  • Diaphragmatic pacing for select cases 1
  • Patient positioning at 30 degrees or higher can significantly improve oxygenation 5

Surgical Management Considerations

• For diaphragmatic hernia associated with paralysis:
  • Primary repair should be attempted when possible using non-absorbable sutures 3
  • For larger defects (>3 cm), mesh reinforcement is recommended to prevent recurrence 3
  • Biosynthetic, biologic, or composite meshes are preferred due to lower recurrence rates, higher resistance to infections, and lower risk of displacement 3
  • Minimally invasive approach (laparoscopic) is preferred for stable patients 6
  • Open laparotomy approach is recommended for unstable patients 3

Special Considerations

• For patients with congenital central hypoventilation syndrome using diaphragm pacing:

  • Spare antennae should be available at home 3
  • A backup diaphragm pacer transmitter is mandatory 3
  • Diaphragm pacers should only be implanted by surgeons with extensive expertise 3
  • Biannual then annual comprehensive evaluation is required 3

• Patients with oral intake difficulties may benefit from percutaneous endoscopic gastrostomy (PEG), gastrostomy, or jejunostomy 3, 6

• For patients with ventilatory failure, positioning is crucial:

  • Symptoms, oxygenation, and vital capacity typically worsen in supine position 1
  • Elevation to 30 degrees or higher can significantly improve oxygenation 5

Prognosis

• Prognosis is generally good in unilateral paralysis, especially in the absence of underlying neurological or pulmonary disease 1, 7
• Prognosis is usually poorer in patients with:
  • Advanced lung disease 1
  • Bilateral paralysis 1
  • Chronic demyelinating conditions 1
• Diaphragmatic function may return spontaneously in some patients, but usually over prolonged periods (up to nine months) 2, 5