What is the management approach for congenital diaphragmatic hernia?

Management of Congenital Diaphragmatic Hernia

For congenital diaphragmatic hernia presenting acutely, perform CT scan for diagnosis, stabilize the patient with immediate intubation and bowel decompression, and proceed with laparoscopic repair in hemodynamically stable patients or open repair in unstable patients, using biological or bioabsorbable mesh for large defects. 1

Diagnostic Approach

CT scan of the chest and abdomen is the gold standard for diagnosing CDH and evaluating defect size, herniated organs, and degree of pulmonary hypoplasia 1, 2. This imaging modality is essential for surgical planning and risk stratification.

Critical Differential Diagnosis

• Distinguish CDH from persistent pulmonary hypertension of the newborn (PPHN), which coexists in 63% of CDH cases 2
• Chest radiography in isolated PPHN shows lung fields without abdominal viscera, whereas CDH demonstrates abdominal organs in the thoracic cavity 2
• Echocardiography is mandatory to evaluate pulmonary hypertension severity, which determines survival (mortality reaches 45% when present) 2

Initial Stabilization

Immediate Delivery Room Management

When CDH is diagnosed prenatally, the newborn should be delivered at a tertiary center with plans for immediate intubation in the delivery room 2. The initial resuscitation includes:

• Bowel decompression via nasogastric tube placement 3
• Avoid mask ventilation to prevent gastric distension and further lung compression 3
• Endotracheal intubation if respiratory distress is present 3

Ventilation Strategy

• Gentle ventilation is the cornerstone to prevent ventilator-induced lung injury in hypoplastic lungs 3
• For mild-to-moderate disease severity, conventional mechanical ventilation (CMV) is preferred as it requires less frequent mode switching, shorter mechanical ventilation time, and reduced inhaled nitric oxide (iNO) use 4
• For severe cases, high-frequency oscillatory ventilation (HFO) may be more advantageous, associated with less frequent ventilation mode switching and reduced ECMO need 4

Surgical Management Algorithm

Patient Stratification by Hemodynamic Status

Hemodynamically Stable Patients:

• Laparoscopic repair is the preferred technique for stable patients without significant comorbidities 1
• This approach has an excellent safety profile with in-hospital mortality of only 0.14% 2
• Laparoscopy facilitates early diagnosis and reduces postoperative complications 1

Hemodynamically Unstable Patients:

• Open surgical repair via laparotomy is necessary 1, 2
• Damage Control Surgery can be life-saving in critically unstable patients 1, 2
• Open surgery is also indicated when laparoscopic skills/equipment are unavailable or exploratory laparotomy is needed 1

Mesh Reinforcement Considerations

• For large defects (>8 cm or >20 cm²), mesh reinforcement is recommended 2
• Use biological or bioabsorbable meshes, which have proven to reduce recurrence rates 1
• Avoid tackers near the pericardium due to risk of cardiac complications 2

Timing of Surgery

• Prompt surgical intervention is necessary but not always as an emergency procedure 2
• Appropriate preoperative assessment and stabilization before surgical intervention is recommended 1, 2
• Surgery should be performed after hemodynamic stabilization and optimization of pulmonary hypertension management 3

Management of Pulmonary Hypertension

• Hemodynamic monitoring and treatment of pulmonary hypertension precede surgery 3
• Although inhaled nitric oxide is not FDA-approved for CDH-induced PPHN, it is commonly used in clinical practice 3
• ECMO is considered after failure of conventional medical management for infants ≥34 weeks' gestation or weight >2 kg with CDH and no associated major lethal anomalies 3

Common Pitfalls and Caveats

Avoid These Critical Errors:

• Never use mask ventilation in CDH patients, as this causes gastric distension and worsens respiratory compromise 3
• Do not delay bowel decompression, as abdominal organ distension in the thorax further compromises pulmonary function 3
• Avoid complete diaphragmatic unloading with ventilator settings, as this accelerates diaphragmatic atrophy at 6% reduction in thickness per day 5
• Right-sided CDH requires specialized approaches due to liver herniation 2

Postoperative Complications

• Gastroesophageal reflux occurs in up to 62% of patients after repair and requires vigilant monitoring 2
• The chronicity of the condition is a primary factor in determining surgical approach and outcomes 2

Prognostic Factors

Multiple factors affect survival including prematurity, associated abnormalities, severity of PPHN, type of repair, and need for ECMO 3. With current management strategies, overall survival is 70-90% in non-ECMO infants and up to 50% in infants requiring ECMO 3.