What is the recommended treatment for Congenital Diaphragmatic Hernia (CDH) in adults?

Treatment of Congenital Diaphragmatic Hernia in Adults

Surgical repair is strongly recommended for all adult patients with congenital diaphragmatic hernia (CDH), regardless of symptoms, to prevent serious complications such as bowel obstruction, strangulation, and incarceration. 1

Diagnostic Approach

• CT scan of the chest and abdomen is the gold standard for diagnosing diaphragmatic hernia 2
• CDH in adults may present with nonspecific respiratory and gastrointestinal symptoms, or may be discovered incidentally on imaging 2

Surgical Management Algorithm

1. Patient Stability Assessment

• For unstable patients with complicated CDH:

  • Laparotomy (open surgical approach) is recommended 2
  • Damage Control Surgery may be life-saving in critically unstable patients 2

• For stable patients with complicated CDH:

  • Minimally invasive approach (laparoscopic) is preferred 2
  • This approach has excellent safety profile with reported in-hospital mortality rate of only 0.14% 2

2. Surgical Technique Selection

• Primary repair:

  • Should always be attempted when possible using non-absorbable sutures 2
  • Appropriate for smaller defects with minimal tension 2

• Mesh reinforcement:

  • Recommended for defects larger than 8 cm or area greater than 20 cm² 2
  • Mesh should overlap the defect edge by 1.5-2.5 cm 2
  • Can be fixed using tackers or transfascial sutures 2
  • Avoid tackers near pericardium due to risk of cardiac complications 2

• For right-sided diaphragmatic hernias:

  • May require combined or thoracic approach due to presence of liver 2
  • Repair can be more technically challenging 2

3. Additional Procedures to Consider

• Fundoplication:

  • Should be performed during congenital hernia repair 2
  • Incidence of gastroesophageal reflux after CDH repair is high (up to 62%) 2
  • Nissen or Toupet fundoplication can be performed with similar effectiveness 2

• For high-risk elderly patients:

  • Gastrostomy or PEG may be considered 2
  • Provides fixation of anterior stomach to abdominal wall 2
  • Well-tolerated with low morbidity 2

Surgical Approach Based on Hernia Type

• Bochdalek hernia (most common, 95% of CDH cases):

  • Occurs predominantly on posterior left side of diaphragm (85% left-sided vs. 15% right-sided) 3
  • Minimally invasive repair is preferred in stable patients 3

• Morgagni hernia (2-4% of diaphragmatic hernias):

  • Surgical repair recommended even when asymptomatic 4
  • Can be repaired via laparoscopic approach with excellent outcomes 4

Outcomes and Complications

• Survival rate in adults with delayed complicated CDH varies between 97-100% 2
• Recurrence rates are relatively low when appropriate surgical technique is used 2
• Potential complications include:
  • Postoperative pulmonary complications (atelectasis) 2
  • Surgical infection, bleeding, respiratory failure 2
  • Gastroesophageal reflux (up to 62% incidence) 2
  • Chronic pain and hernia recurrence 2

Special Considerations

• Thoracoscopic repair may be considered for selected patients with specific countermeasures to prevent complications 5
• For giant hernias, extended procedures may be necessary to avoid abdominal compartment syndrome 6
• Robotic surgery is emerging but currently has limited data 7